Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
197 Cards in this Set
- Front
- Back
|
What are contraindications to breast feeding?
|
• mother receiving chemotherapy or radioactive compounds • mother with HIV/AIDS, active untreated TB, herpes in breast region
• mother using >0.5 glkgld alcohol and/or illicit drugs (decrease milk production and/or directly toxic to baby) • mother taking certain medications e.g. antimetabolites, bromocriptine, chloramphenicol, high dose diazepam, ergots, gold, metronidazole, tetracycline, lithium, cyclophosphamide • Note: oral contraceptive pills (OCP) not a contraindication to breast feeding. (estrogen may decrease lactation but is not dangerous to infant) |
|
|
When can children start eating veggies?
|
4-7 mo
|
|
|
When can fruits and juices be introduced?
|
6-9 mo
|
|
|
When can finger foods and milk be introduced?
|
9-12 mo
• do not delay introduction of solid foods beyond 9 months |
|
|
How to differentiate a pathologic hear murmur?
|
Symptoms and signs of cardiac disease 1m exercise intolerancel
All diastolic, pansystolic, or continuous (except venous hum) >3I6Ipalpable thrill) Fixed split or single S2 extra heart sounds unchanged with positioning |
|
|
How it fetal belood flow before birth?
|
• shunting deoxygenated blood
• ductus arteriosus: connection between pulmonary artery and aorta • shunting oxygenated blood • foramen ovalI.': connection between R and L atria • ductus venosus: connecting between umbilical vein and lVC • circulation: • placenta (oxygenated blood) > umbilical vein> ductus venosus> lVC> R atrium> oxygenated blood shunted through foramen ovalI.' > L atrium> L ventricle> aorta> brain/myocardium/upper extremities • deoxygenated blood returns via SVC to R atrium> 1/3rd of blood entering R atrium does not flow through foramen ovalI.' and flows to the R ventricle> pulmonary arteries> ductus arteriosus> aorta> systemic circulation> placenta for reoxygenation |
|
|
How is fetal blood flow at birth?
|
• with first breath, lungs open up and pulmonary resistance decreases allowing
pulmonic blood flow • with separation of low resistance placenta, systemic circulation becomes a high resistance system • with closure of the fetal shunts and changes in pulmonic/systemic resistance, infant circulation assumes normal adult flow • increasing pulmonic How increases left atrial pressures leading to foramen ovalI.' closure • increased oxygen concentration in blood after first breath leads to decreased prostaglandins leading to closure of the ductus arteriosus • as the umbilical cord is clamped, the umbilical vein closes, systemic vascular resistance increases and the ductus venosus closes |
|
|
What is the difference b/w cyanotic heart disease and acyanotic heart disease?
|
• cyanotic heart disease: (i.e. R> L shunt) blood bypasses the lungs> no oxygenation occurs> high levels of deoxygenated hemoglobin enters the systemic circulation> cyanosis
• acyanotic heart disease: (i.e. L> R shunt, obstruction occurring beyond lungs) blood passes through pulmonic circulation> oxygenation takes place> low levels of deoxygenated blood in systemic circulation> no cyanosis |
|
|
What is the pathophys of left to right shunts?
|
• extra blood is displaced through a communication from the left to the right side of the heart --> increased pulmonary blood flow --> increased pulmonary pressures
• shunt volume dependent upon three factors: size of defect, pressure gradient between chambers or vessels, peripheral outflow resistance • untreated shunts can result in pulmonary vascular disease, right ventricular hypertension and hypertrophy (RVH), and eventually R-->L shunts |
|
|
What are the types of ASDs? What is their clinical and physical presentation? How to treat?
|
• three types: ostium primum (common in Down syndrome), ostium secundum (most common type, 50-70%), sinus venosus (defect located at entry of superior vena cava (5VC) into right atrium)
• epidemiology: 6-8% of congenital heart lesions • natural history: 80-100% spontaneous closure rate if A5D diameter <8 mm • if remains patent, congestive heart failure (CHF) and pulmonary hypertension can develop in adult life • history: often asymptomatic in childhood • physical exam: grade 2-3/6 pulmonic outflow murmur (5EM), a mid-diastolic rumble at the left lower sternal border, and a widely split and fixed 52 • investigations: • ECC: right axis deviation (RAD), mild RVH, right bundle branch block (RBBB) • CXR: increased pulmonary vasculature • treatment: elective surgical or catheter closure between 2-5 years of age |
|
|
How does a small VSD present? How to treat?
|
• history: asymptomatic, normal growth and development • physical exam: early systolic to holosystolic murmur, best heard at left lower sternal
border (LL5B) • investigations: ECC and CXR are normal • treatment: most close spontaneously, do not need surgical closure even if remains patent |
|
|
How does a moderate/large VSD present? What is the physical presentation? How to treat?
|
• natural history: secondary pulmonary hypertension, CHF by 2 months of age • history: delayed growth and development, decreased exercise tolerance, recurrent
URTIs or "asthma" episodes, CHF • physical exam: holosystolic murmur at LLSB with thrill, mid-diastolic rumble at apex, size of VSD is inversely related to intensity of murmur • investigations: • ECG: left ventricular hypertrophy (LVH), left atrial hypertrophy (LAH), RVH • CXR: increased pulmonary vasculature, cardiomegaly, CHF • treatment: treatment of CHF and surgical closure by I year of age Size of VSD is inverserly related intensity of murmur. |
|
|
How does PDA present? How to investigate and treat?
|
• patent vessel between descending aorta and left pulmonary artery • epidemiology
• functional closure within first 15 hours of life, anatomical closure within first days of life • 5-10% of all congenital heart defects • common in premature infants (113 of infants <1750 grams) natural history: spontaneous closure common in premature infants, less common in term infants • history: may be asymptomatic or have apneic or bradycardic spells, poor feeding, accessory muscle use • physical exam: tachycardia, bounding pulses, hyperactive precordium, wide pulse pressure, continuous "machinery" murmur, best heard at left infraclavicular area • investigations: • ECG: may show LAH, LVH, BVH • CXR: normal to mildly enlarged heart, increased pulmonary vasculature, prominent pulmonary artery • diagnosis by echocardiography (Echo) • treatment: • indomethacin (Indocid™) - PGEI antagonist (PGEI maintains ductus arteriosus patency) in premature infants if necessary • catheter or surgical closure if PDA is contributing to respiratory compromise or persists beyond 3rd month of life |
|
|
What is endocardial cushion defect? How to reat?
|
• spectrum from endocardial cushion VSD and ostium primum ASD to complete AV canal with common AV valve
• commonly associated with Down syndrome • treatment: • natural history depends on size of defect and valvular involvement, and should be repaired by age 6 months to prevent development of pulmonary hypertension • complete AV canal requires early complete surgical repair, preferably before 3 months of age |
|
|
What is coarctation of the aorta? How does it present? Prognosis and treatment? Associations?
|
• narrowing of aorta almost always at the level of the ductus arteriosus • commonly associated with bicuspid aortic valve (50%); Turner syndrome (35%) • few have high BP in infancy (160-200 mmHg systolic) but this decreases as collaterals
develop • if severe, presents with shock in the neonatal period when the ductus closes • history: often asymptomatic • physical exam: upper extremity systolic pressures of 140-145 mmHg, decreased blood pressure and weak/absent pulses in lower extremities, radial-femoral delay, absent or systolic murmur with late peak at apex, left axilla, and left back • investigations: ECG: RVH early in infancy, LVH later in childhood • prognosis and treatment • if associated with other lesions (e.g. PDA, VSD) can cause CHF • complications: hypertension • management: balloon arterioplasty or surgical correction in symptomatic neonate, give prostaglandins to open up PDA for stabilization |
|
|
What is aortic stenosis? How does it present? How to treat?
|
• valvular (75%), subvalvular (20%), supravalvular and idiopathic hypertrophic subaortic stenosis (IHSS) (5%)
• history: often asymptomatic but may be associated with CHF, exertional chest pain, syncope or sudden death • physical exam: SEM at upper right sternal border (URSB) with aortic ejection click at the apex • treatment • surgical repair if infant with critical aortic stenosis or older child with symptoms or peak gradient >50 mmHg • exercise restriction required |
|
|
What is pulmonic stenosis? How does it present? How to treat?
|
• valvular (90%), subvalvular, or supravalvular • usually part of other congenital heart lesions (e.g. Tetralogy of Fallot) or in association
with other syndromes (e.g. congenital rubella, Noonan syndrome) • critical pulmonic stenosis: inadequate pulmonary blood flow, dependent on ductus for oxygenation, progressive hypoxia and cyanosis • history: spectrum from asymptomatic to CHF • physical exam: wide split S2 on expiration, SEM at ULSB, pulmonary ejection click • investigations: • ECG: RVH • CXR: dilated post-stenotic pulmonary artery • treatment: surgical repair if critically ill or severe PS, or if presence of symptoms in older infants/children |
|
|
How do differentiate cyanosis due to cardiac or dut to resp causes?
|
• systemic venous return re-enters systemic circulation directly • most prominent feature is cyanosis (0
sat <75%) • differentiate between cardiac and other causes of cyanosis with hyperoxic test 2 • obtain preductal, right radial ABC in room air, repeat ABC after the child inspires 100% oxygen • if Pa0 improves to greater than 150 mmHg, cyanosis less likely cardiac in origin 2 • survival depends on mixing via shunts (e.g. ASD, VSD, PDA) |
|
|
What is the most common cyanotic heart defect beyond infancy? What is it a/w? How does it present? What are complications? How to treat?
|
• 10% of all CHD, most common cyanotic heart defect diagnosed beyond infancy • embryologically, a single defect with hypoplasia of the conus causing:
• VSD • right ventricle (RV) outflow tract obstruction (RVOTO) • overriding aorta • RVH • degree of RVOTO directly determines the direction and degree of shunt and therefore the extent of clinical cyanosis and degree of RVH • infants may initially have a L· ... R shunt and therefore are not cyanotic but the RVOTO is progressive, resulting in increasing R ->L shunting with hypoxemia and cyanosis • history: hypoxic "tet" spells • primary pathophysiology is hypoxia, leading to increased pulmonary vascular resistance (PVR) and decreased systemic resistance, occurring in exertional states (e.g. crying, exercise) • paroxysm of rapid and deep breathing, irritability and crying hyperpnea, increasing cyanosis often leading to deep sleep and decreased intensity of murmur (decreased flow across RVOTO) • peak incidence at 2-4 months of age • if severe may lead to seizures, loss of consciousness, death (rare) • management: O 2 , knee-chest position, fluid bolus, morphine sulfate, propanolol • physical exam: single loud S2 due to severe pulmonary stenosis (i.e RVOTO) • investigations: • ECC: RAD, RVH • CXR: boot shaped heart (small PA, RVH), decreased pulmonary vasculature, right aortic arch (in 20%) • treatment: surgical repair within first two years of life, or earlier if marked cyanosis, "tet" spells, or severe RV outflow tract obstruction |
|
|
What is Ebstein's anomaly? What is the cause? How it is treated?
|
• congenital defect of the tricuspid valve in which the septal and posterior leaflets are malformed and displaced into the RV leading to variable degrees of RV
dysfunction, TS, TR or functional pulmonary atresia if RV w1able to open pulmonic valves • RA massively enlarged, interatrial commW1ication (PFO) often exists allowing R --> L shunting • TR and accessory conduction pathways (WPW) are often present • cause: unknown, associated with maternal lithium and benzo use in 1 trimester • treatment: • in newborns, consider closure of tricuspid valve + aortopulmonary shunt, or transplantation • in older children, tricuspid valve repair or valve replacement + ASD closure |
|
|
How does TGA present? How to treat?
|
• 3-5% of all congenital cardiac lesions • parallel pulmonary and systemic circulations
• systemic: body --> RA -+ RV --> aorta -+ body • pulmonary: lungs --> LA --> LV --> pulmonary artery --> lungs • physical exam: • no murmur if no VSD • newborn presents with progressive cyanosis unresponsive to oxygen therapy as the ductus arteriosus closes and mixing between the two circulations diminishes; severe hypoxemia, acidosis, and death can occur rapidly • if VSD present, cyanosis is not prominent and infant presents with CHF after a few weeks of life • investigations: • ECG: RAD, RVH • CXR: egg-shaped heart with narrow mediastinum ("egg on a string") • treatment: • prostaglandin EJ. (Prostin VRTM) infusion to keep ductus open until septostomy or surgery (artenal switch procedure) • infants without VSD must be repaired within 2 weeks to avoid weak LV muscle |
|
|
What is hypoplastic left heart syndrome? How to treat?
|
• 1-3% of all congenital cardiac lesions • a spectrum of hypoplasia of left ventricle, atretic mitral and/or aortic valves, small
ascending aorta, coarctation of the aorta with resultant systemic hypoperfusion • most common cause of death from congenital heart disease in first month of life • systemic circulation is dependent on ductus patency; upon closure of the ductus, infant presents with circulatory shock and metabolic acidosis • treatment • intubate and correct metabolic acidosis • IV infusion of PGE to keep ductus open • surgical correction (overall survival 50% to late childhood) or heart transplant |
|
|
What is truncus arteriosis? How to treat?
|
• a single great vessel arising from the heart which gives rise to the aorta, PA, and coronary arteries
• the truncal valve overlies a large VSD • treatment: surgical repair within first 6 months of life to prevent development of pulmonary vascular disease |
|
|
What are the 4 key features of CHF?
|
Tachycardia Tachypnea Cardiomegaly Hepatomegaly
Physical Findings • four key features: tachycardia, tachypnea, cardiomegaly, hepatomegaly • failure to thrive (FIT) • respiratory distress, gallop rhythm, wheezing, crackles, cyanosis, clubbing (with CHD) • alterations in peripheral pulses, four limb blood pressures (in some CHDs) • dysmorphic features associated with congenital syndromes • CXR - cardiomegaly, pulmonary venous congestion |
|
|
What are the etiologies of CHF in children? Symptoms?
|
Etiology
• congenital heart disease (CHD) • acute hypertension • arteriovenous malformations (AVMs) • anemia • cardiomyopathy • cor pulmonale • arrhythmias • myocarditis Symptoms • infant: feeding difficulties, easy fatiguability, exertional dyspnea, diaphoresis when sleeping or eating, respiratory distress, lethargy, cyanosis, FIT • child: decreased exercise tolerance, fatigue, decreased appetite, failure to thrive, respiratory distress, frequent URTls or "asthma" episodes • orthopnea, paroxysmal nocturnal dyspnea, pedal/dependant edema are all uncommon in children |
|
|
How to treat CHF in children ?
|
Management
• correction of underlying cause • general: sitting up, 02' sodium and water restriction, increased caloric intake • pharmacologic: diuretics, digoxin, afterload reducers |
|
|
What is the most frequent sustained dysrhythmia in children?
|
• most frequent sustained dysrhythmia in children
• not life-threatening but can lead to symptoms • caused by re-entry via accessory cormection (atrioventricular (A V) node most common site) • characterized by a rate of greater than 210 bpm • treatment: vagal maneuver, adenosine, digoxin (except in Wolfe-Parkinson-White (WPW) because the opportunity for re-entry increases with slowing of the AV node) or ~-blockers, or synchronized cardioversioin if child unstable |
|
|
What are the criteria for FAS?
|
a) growth deficiency -low birth weight and/or length at birth that continues
through childhood b) abnormal craniofacial features - small head, short palpebral fissures, long smooth philtrum, thin upper lip c) central nervous system dysfunction - microcephaly and/or neurobehavioural dysfunction (e.g. hyperactivity, fine motor problems, attention deficits, learning disabilities, cognitive disabilities) d) strong evidence of maternal drinking during pregnancy |
|
|
What are the diagnostic criteria for DM?
|
1200 mg/dLI
OR Symptoms Ipolyuria, polydipsia, weight loss) + random glucose ~ 11.1 mmoVL Fasting glucose ~7.0 mmoVL 1126 mgldLI OR 2hr glucose during OGTT ~11.1 mmoVL 1200 mgldL) OGTT=oral glucose tolerance test |
|
|
What is DI? What are the different types? How to diagnose and manage?
|
• Dl is the inability of the kidneys to concentrate urine • central:
• due to decreased ADH production from the brain (genetic, due to trauma, surgery, radiation, neoplasm, meningitis) • presents with polyuria, polydipsia, enuresis • nephrogenic: • renal unresponsiveness to ADH (genetic, drug-induced) • x-linked recessive condition that affects males in early infancy • polyuria, FIT, hyperpyrexia, vomiting, hypematremic dehydration Diagnosis • symptoms: polyuria, enuresis, nocturia, polydipsia, dehydration • labs: dilute urine (SG<1.010), hypematremia, elevated serum osmolality, low urine osmolality • water deprivation test; central cause if >50% change in urine osmolality after ADH administration Management • central: DDAVP intranasally, SC, or PO • nephrogenic: low-solutE' diet, thiazide diuretics |
|
|
What is SIADH? What do labs show?
|
• etiology: intracranial, malignancy, pulmonary disease, psychiatric disease, drugs • symptoms: asymptomatic, oliguria, volume expansion, or hyponatremic symptoms
(nausea, vomiting, H/A, seizure, coma) • labs: hyponatremia, urine Osm > plasma Osm, urine Na > 20mmol/L • management: fluid restriction, 3% NaCi for symptomatic hyponatremia |
|
|
What are features of congenital hypothryoidism? What is the prognosis?
|
• usually asymptomatic in neonatal period because maternal T4 crosses the placenta
but may have the following symptoms: • prolonged jaundice • constipation • sluggish, hoarse cry, lethargy, poor feeding • macroglossia, coarse facial features, large fontanelles, umbilical hernia • prognosis • excellent if treatment started within 1-2 months of birth • if treatment started after 3-6 months of age may result in permanent developmental delay and/or mental retardation (mild to profound) • management: thyroxine replacement |
|
|
How does acquired hypothyroidism present in a child?
|
• most commonly Hashimoto's thyroiditis (autoimmune destruction of the thyroid) • signs and symptoms similar to hypothyroidism in adults, but also:
• delayed bone age, decline in growth velocity, short stature, goiter • sexual pseudoprecocity: early sexual development with short stature and delayed bone age • does not cause permanent developmental delay • treated with L-thyroxine 10 f-lg/kg/day |
|
|
What is CAH? What do labs show?
|
• occurs in 1/15000 live births and is the most common cause of ambiguous genitalia • autosomal dominant condition causing rartial or total enzyme defect
• 21-hydroxylase deficiency causes 95% 0 CAH cases; this causes decreased cortisol and aldosterone with shunting toward overproduction of androgens • cortisol deficiency leads to elevated ACTH, which causes adrenal hyperplasia • clinical presentation depends on the specific deficiency and the cause • high ACTH, increased 17-0H progesterone, increased testosterone, DHEAS, urinary 17-ketosteroids, advanced bone age |
|
|
What is late onset 21 hydroxylase deficiency? How does it present/ How is it diagnosed and treated?
|
• allelic variant of classic 21-hydroxylase deficiency - mild enzymatic defect • girls present with amenorrhea
• boys present with precocious puberty with early adrenarche, dehydration • accelerated linear growth in early puberty but early fusion of epiphyses leading to decreased adult height • diagnosis • increased plasma 17-0H-progesterone after ACTH stimulation test • treatment • dexamethasone, spironolactone (anti-androgen) • mineralocorticoid replacement is not needed |
|
|
Causes of true precocious puberty?
|
• hypergonadotropic hypergonadism, hormone levels as in normal puberty • premature activation of the hypothalamic-pituitary-gonadal axis • much more common in females than males 9:1
• differential diagnosis • idiopathic or constitutional (most common, especially females) • CNS disturbances: tumours, hamartomas, post-meningitis, increased rcp, radiotherapy • neurofibromatosis (NF), primary severe hypothyroidism |
|
|
Causes of pseudoprecocious puberty?
|
• hypogonadotropic hypergonadism • differential diagnosis
• adrenal disorders: CAH, adrenal neoplasm • testicular/ovarian tumour • gonadotropin secreting tumour: hepatoblastoma, intracranial teratoma, germinoma • exogenous steroid administration • McCune-Albright syndrome: endocrine dysfunction resulting in precocious puberty, cafe-au-lait spots, and fibrous dysplasia of skeletal system |
|
|
What are the investigations and treatments for precocious puberty?
|
Investigations
• history: symptoms of puberty, family history of puberty onset, medical illness • physical exam: growth velocity, Tanner staging, neurological exam • estradiol, testosterone, LH, FSH, TSH, GnRH test • bone age (often advanced) • consider CT or MRI of head, D/S of adrenals, pelvis Treatment • GnRH analogs, GnRH agonist (LupronTM) - negative feedback to downregulate GnRH receptors • medroxyprogesterone - slows breast and genital development • treat underlying cause |
|
|
Short Stature DD. What are the 4 questions to ask when evaluating short stature?
|
Alone (neglected infant)
Bone dysplasias (rickets, scoliosis, mucopolysaccharidoses) ChromosomallTurner, Down) Delayed growth Endocrine (low growth hormone, Cushing, hypothyroid) Familial GI malabsorption (celiac, Crohn) 1. was there IUGR? 2. is the growth proportional? 3. is the growth velocity normal? 4. is bone age delayed? |
|
|
Short stature with normal growth velocity?
|
• Constitutional Growth Oalay - delayed puberty - may have family history of delayed puberty - may require shon-term therapy with androgens/estrogens
- delayed bone age - often mid-parental height is normal • Familial - normal bone age -treatment not indicated - family Hx of short stature |
|
|
Short stature with decreased growth velocity?
|
• Primordiallheight. weight, and HC are affected) - chromosomalle,g, Turner, Down syndrome, dysmorphic featuresl -skeletal dysplasias - intrauterine growth restriction (IUGRllteratogen, placental insufficiency, infectionl
• Endocrine (height affected more than weightl- "short and fat" - GH deficiency Islow growth velocity, decreased bone age, delayed pubertyl - hypothyroidism - hypercortisolism ICushing syndromellexogenous and endogenousl - hypopituttarism • Chronic disease Iweight affected more than heightl- "shon and skinny" - cyanotic congenital heart disease - celiac disease, inflammatory bowel disease, cystic fibrosis - chronic infections - chronic renal failure loften height more affectedl • Psychosocial naglect [psychosocial dwarfisml - usually decreased height and weight (decreased head circumference if severel |
|
|
What hormones affect growth in a fetus or child?
|
• GH important for chondrocyte proliferation and IGF-I release • GH has little effect on fetal growth (maternal IGF-I, uterine factors more important) • IGF-I acts at long bones, liver, negative feedback
|
|
|
What is the clinical presentation of a TEF? How to manage? What are comlications?
|
• clinical presentation (vary with type of fistula)
• may have history of maternal polyhydramnios • may present after several months, if no associated esophageal atresia, with vomiting, coughing, and gagging • cyanosis with feeds, respiratory distress, recurrent pneumonia • frothy bubbles of mucus in mouth and nose that return after suctioning • associated anomalies in 50%: VACTERL association (see Pediatric Genetics, Dysmorphisms, and Metabolism, P42) • x-ray: anatomic abnormalities, NG tube curled in pouch • management • investigate for other congenital anomalies • early repair by surgical ligation to prevent lung damage and maintain nutrition and growth • complications • pneumonia, sepsis, reactive airways disease • following repair: esophageal stenosis and strictures at repair site, gastroesophageal reflux and poor swallowing (i.e. dysphagia, regurgitation) |
|
|
Vomiting in newborn period
|
TEF
duodenal atresia pyloric stenosis malrotation of intestine |
|
|
Duodenal atresia
|
• incidence: 1:10 000, 50% are born prematurely • clinical features
• bile-stained vomiting if atresia distal to bile duct • no abdominal distention • dehydration • associated with Down syndrome, prematurity • may have history of maternal polyhydramnios • abdominal x-ray: air-fluid levels on upright film; "double bubble" sign (dilated stomach and duodenum) • differential diagnosis: annular pancreas, aberrant mesenteric vessels, pyloric stenosis • treatment • decompression with NG tube • correction of metabolic abnormalities • surgical correction |
|
|
Malroation
|
• incidence: 1:500 • 80% experience symptoms in first two months of life
• 3 presentations • recurrent vomiting (bilious intermittently) • FIT with vomiting • sudden onset abdominal pain and then shock (if vomiting with bilious material, malrotation with volvulus until proven otherwise) • clinical features • distended abdomen • vomiting due to volvulus and bands across duodenum • diagnosis: abdominal U/S and upper GI series • treatment: NG tube decompression and surgery • complications: volvulus is a surgical emergency as it can result in bloody stools, perforation, and peritonitis |
|
|
Vomiting after newborn period
|
infectious and inflammatory
anatomic GER CNS • metabolic/endocrine: DKA, inborn errors of metabolism, liver failure • poisons/drugs: lead, digoxin, erythromycin, theophylline • psychogenic: rumination syndrome, anorexialbulimia • food allergy • overfeeding |
|
|
Infctious causes of vomiting
|
• Gl causes: gastroenteritis, peritonitis, appendicitis, hepatitis, ulcers, pancreatitis, cholecystitis
• non-Gl causes: urinary tract infection (UTI), pyelonephritis, nephrolithiasis, otitis media, labyrinthitis, meningitis, pneumonia |
|
|
Anatomic causes of vomiting
|
• Gl tract obstruction • intussusception, volvulus • foreign body (e.g. bezoar)
|
|
|
How does GER present? How to manage? How to treat? What are complications?
|
• extremely common in infancy (up to 50%): thriving baby requires no investigation • vomiting typically soon after feeding, non-bilious, rarely contains blood, small volume
«loz) • investigations required if: FIT, remrrent cough, pneumonia or bronchospasm, Gl blood loss, symptoms persist after 18 months • 24-hour pH probe, VGl series to rule out anatomical cause, upper endoscopy and esophageal biopsy for suspected esophagitis • management • conservative: thickened feeds, frequent and smaller feeds, elevate bed to 45° • medical: • short-term parenteral feeding to enhance weight gain • ranitidine, omeprazole: to decrease gastric acidity, decrease esophageal irritation • domperidone: to improve gastric emptying and Gl motility • surgical: indicated for failure of medical therapy (Nissen fundoplication) • complications: esophagitis, strictures, Barrett's esophagus, FIT, aspiration |
|
|
What are cns cases of vomiting in infant?
|
• increased intracranial pressure (ICP) (e.g. hydrocephalus, neoplasm) • drugs/toxins
• migraine, cyclic vomiting |
|
|
What are clinical associations with viral diarrhea?
|
• assoLiated with URTls • resolves in 3-7 days
• slight fever, malaise, vomiting, vague abdominal pain |
|
|
Causes of chronic diarrhea by age?
|
|
|
|
What is Toddler's diarrhea? How does it present? How to manage?
|
• epidemiology • most common cause of chronic diarrhea during infancy
• onset between 6-36 months of age, ceases spontaneously between 2-4 years • clinical presentation • diagnosis of exclusion in thriving child (no weight loss/FTT, no fluid or electrolyte abnormalities) • diet history: too much juice overwhelms small bowel resulting in disaccharide malabsorption • stool may contain undigested food particles, 4-6 bowel movements (BM's)/day • excoriated diaper rash • management • reassurance, self-limiting • four F's (adequate fibre, normal fluid intake, 35-40% fat, discourage excess fruit juice) |
|
|
How to diagose lactase deficiency?
|
• clinical features • chronic, watery diarrhea
• abdominal pain, bloating associated with dairy intake • primary lactose intolerance: crampy abdominal pain with loose stool (older children, usually of East Asian and African descent) • secondary lactose intolerance: older infant, persistent diarrhea (post viral/bacterial infection, celiac disease, or IBD) • diagnosis • trial of lactose-free diet • watery stool, acid pH, positive reducing sugars • positive breath hyarogen test if >6 years • management • lactose-free diet, soy formula • lactase-containing tablets/capsules/drops (e.g. Lacteeze™, Lactaid™) |
|
|
What is celiac disease? What are the clinical features? How to Dx? What is the treatment?
|
• incidence 1:10 000 • also known as "gluten-sensitive enteropathy"
• defect in mucosa: immune-mediated inflammation and destruction of absorptive villi • clinical features • presents at any age, usually 6-18 months with the introduction of gluten in the diet • • FIT with poor appetite, irritability, apathy • anorexia, nausea, vomiting, edema, anemia, abdominal pain • wasted muscles, distended abdomen, flat buttocks, clubbing of fingers • rickets • diagnosis • anti-transglutaminase, antigliadin, antiendomysial antibodies, low D-xylose absorption • fat malabsorption studies • small bowel biopsy: villous atrophy with resolution after trial of gluten-free diet • treatment • gluten-free diet for life • complications if untreated • small bowel lymphoma • malnutrition, FIT |
|
|
What is Schwachman-Diamond Syndrome? How does it present?
|
• incidence: 1:20,000, autosomal recessive • pancreatic insufficiency, cyclic neutropenia, and anemia
• skeletal abnormalities (metaphyseal dysostosis leading to short stature) • recurrent pyogenic infections • distinguished from CF by normal sweat chloride test, characteristic metaphyseal lesions, fatty pancreas on cr |
|
|
What are clinical manifestations that could signify a metabolic disease?
|
• vomiting and acidosis after feeding initiation (amino acid or carbohydrate metabolic disorder)
• hepatosplenomegaly (metabolites accumulate in the liver) • neurologic syndrome: acute and chronic encephalopathy, MR, megalencephaly (mucopolysaccharide disorders) • severe acidosis (aminoaciduria), hyperarnmonemia (urea cycle and organic acid disorders) • growth retardation, seizures, coma, hypoglycemia • autonomic manifestations (e.g. pallor, sweating, tremor) |
|
|
What are initial investigations undertaken in suspected metabolic disorder?
|
• electrolytes, ABGs (calculate anion gap, rule out acidosis) • CBC with differential and smear • blood glucose (hypoglycemia seen with organic acidemia, fatty acid oxidation defects
and glycogen storage diseases) • lactate, ammonium (hyperammonemia with urea cycle defects), plasma Ca and Mg • routine urinalysis: ketonuria must be investigated • others: urate, urine nitroprusside, amino acid screen, CSF glycine, free fatty acids (3-~-hydroxybutyrate ratio >4 in fatty acid oxidation defect) • storage diseases: urine mucopolysaccharide and oligosaccharide screen |
|
|
What is PKU? How does it present? What is the treatment?
|
• incidence: 1 in 10,000 • screened in all newborns
• etiology: deficiency of phenylalanine hydroxylase prevents conversion of phenylalanine to tyrosine leading to build up of toxic metabolites • mothers who have PKU may have infants with congenital abnormalities • presentation • baby is normal at birth then develops a musty odour, eczema, hypertonia, tremors, and mental retardation • hypopigmentation due to low tyrosine (fair hair, blue eyes) • treatment • PKU screening at birth • dietary restriction of phenylalanine starting within the first 10 days of life • duration of dietary restriction controversial-lifelong or until end of puberty, recommended during pregnancy |
|
|
What is galactosemia? How does it present? What is the treatment?
|
• incidence: 1 in 60,000, autosomal recessive disease • most commonly due to deficiency of galactose-I-phosphate uridyltransferase leading to
an inability to process lactose/galactose • increased risk of sepsis • if the diagnosis is not made at birth, liver and brain damage may become irreversible • features: neonate who ingests lactose/galactose exhibits signs of liver and renal failure, FIT and cataracts • treatment • elimination of galactose from the diet (Le. dairy) |
|
|
What is physiologic anemia?
|
• elevated hemoglobin (>170 gIL) and reticulocyte count at birth as a result of relatively hypoxic environment in utero
• after birth, levels start to fall due to shorter fetal RBC lifespan, decreased RBC production (during first 6-8 weeks of life virtually no erythropoiesis due to new 02 rich environment) and increasing blood volume secondary to growth • lowest levels about 100 gIL at 6-12 weeks age (earlier and more exaggerated in premature infants); levels rise spontaneously with activation of erythropoiesis • no treatment usually required |
|
|
What are risk factors for iron-deficiency anemia in a baby?
|
• dietary risk factors • "milk baby" - bottle-fed infants (9-24 months) receiving large volumes (>20 oz.
per day) of cow's milk leading to poor intake of iron-rich foods • formula without iron • delayed introduction of iron fortified infant cereal • blood loss • iatrogenic: repeated blood sampling (especially in hospitalized neonates) • true cow's milk allergy: occult bleeding and protein-losing enteropathy secondary to GI inflammation |
|
|
What is the pathophys of sickle cell disease?
|
• caused by a genetic defect in ~-globin genes • red blood cells sickle under conditions of stress (low p02' dehydration, fever, acidosis) • acute intravascular sickling results in infarction of tissue (capillary occlusion and
thrombosis - spleen, lungs, bones, brain, digits) • hemolysis causes chronic, well-compensated normochromic normocytic anemia (Hb 60-90 gIL) • increased incidence in people of African and Mediterranean heritage, felt to be protective in malaria endemic countries • greatest cause of mortality is infection |
|
|
What are the types of crises in SCA?
|
• vasa-occlusive crisis • due to obstruction of blood vessels by rigid, sickled cells -> tissue hypoxia -->
cell death; presents as fever and pain in any organ; most commonly in long bones of arms and legs, chest, abdomen, CNS (stroke), dactylitis (in young children), priapism • acute chest crisis: fever, chest pain, progressive respiratory distress, increased WBC count, pulmonary infiltrates • aplastic crisis - depression of erythropoiesis (decreased reticulocyte count to <1 %, decreased Hb), generally associated with infection (especially parvovirus B19) • acute splenic sequestration -- sudden massive pooling of red cells in spleen, splenomegaly, tender spleen, acute fall in hemoglobin, shock, increased reticulocyte count |
|
|
What are complications of SCA?
|
Functional Asplenia
• splenic dysfunction usually by 5 years of age secondary to autoinfarction • susceptible to infection by encapsulated organisms (especially S. pneumoniae) • requires prophylactic antibiotics, pneumococcal/meningococcal/H. influenzae type b vaccination, and immediate evaluation of any fever Other Manifestations • increased incidence of osteomyelitis (especially due to Salmonella) • growth delay, bony abnormalities - e.g. avascular necrosis (AVN) of femoral head, gallstones, retinopathy, restrictive lung disease |
|
|
How to manage a sickle cell patient?
|
• acute crises • admit for supportive and symptomatic treatment
• fluids (l.5x maintenance; Ix maintenance only if in chest crisis), analgesia (morphine), antibiotics (e.g. 3 gen. cephalosporins), incentive spirometry to decrease risk of chest crisis rd • straight transfusions for symptomatic/significant anemia (e.g. aplastic crisis), evolving chest crisis • RBC exchange transfusion for impending stroke, severe chest crisis, persistent priarism • O? i respiratory distress or chest crisis (with incentive spirometry) cu1tures and CBC if febrile, reticulocyte counts, CXR or LP if indicated • chronic • early aggressive treatment of infections, prophylactic antibiotics (daily oral penicilfin) pneumococcal, meningococcal, hepatitis B, Bib, and influenza vaccines folate supplementation if folate deficient • hydroxyurea if frequent crises (raises HbF level) transcranial doppler ultrasound to assess risk of stroke or abnonnal transcranial doppler • chronic transfusion program if history of stroke or abdominal transcranial doppler • genetic counselling and education annual ophthalmologic exam (after 10 years old) referral to hematology |
|
|
How does G6PD manifest?
|
• X-linked recessive; thf' most common enzyme deficiency worldwide • higher prevalence in Mediterraneans, African Americans, Asians • enzyme-deficient RBC unable to J.efenJ. against oxidative stress (infection, drugs) ->
form Heinz boJ.ies (Hb precipitates within RBCs) -, phagocytosed by splenic macrophages creating "bite cells" (RBCs that appear to have bites taken out of them, also known as "cookie cells") • presents with acute hemolytic anemia (hemoglobinuria, decreased haptoglobin, increased lDH, and elevated indirect bilirubin) with jaundice, pallor and dark urine (rarely causes chronic anemia) • management: supportive, hydration, transfusion, phototherapy • prevention: avoid known oxidants (e.g. fava beans, antimalarials (primiquine), suI fonamides) |
|
|
What is ITP? What is its presentation, course, and management?
|
• most common cause of thrombocytopenia in childhood • peak age: 2-6 years, M=F
• caused by antibodies that bind to platelet membranes - .. splenic uptake ---.. destruction of platelets • presentation and course • typically presents 1-3 weeks after viral illness (URTI, chicken pox) • sudden onset of petechiae, purpura, epistaxis in an otherwise well child • no lymphadenopathy, no hepatosplenomegaly • labs: thrombocytopenia with normal RBC, WEC • if atypical presentation (~1 cell line abnormal, hepatosplenomegaly), do bone marrow to rule out leukemia • differential diagnosis: leukemia, drug-induced thrombocytopenia, HN, infection (viral), SLE • management • self-limited in children; spontaneous recovery in >80% of cases • usually choose to treat because spontaneous recovery takes a few months, and risk of bleeding (especially intracranial hemorrhage with platelets <..-20) • IVIG or oral prednisone (mainstays of treatment), IV anti-D (if blood group Rh positive) • splenectomy (only for life-threatening bleeding) |
|
|
Approach to neonatal fever
|
|
|
|
What are th etiologis of AOM?
|
• bacterial (70%) - S. pneumoniae (35%), H. inJIuenzae (25%), M. catarrhalis (10-20%),
Group A Streptococcus (3%) • viral (20-30%) - commonly RSV, influenza, parainfluenza, adenovirus |
|
|
How to manage AOM?
|
• 60-80% of AOM in children self-resolve without antibiotic therapy, therefore controversy exists surrounding antibiotic use in AOM management
• antibiotics: • non-severe: amoxicillin, 80-90 mg/kg/day divided tid • severe: amoxicillin 90 mg/kg/day + c1avulin 6.4 mg/kg/day • treahnent failure 48-72 hr. after initial antibiotic use in severe AOM: ceftriaxone 1-3 days • analgesics: acetaminophen or ibuprofen for pain management |
|
|
Etiologis of meningitis? Pathophysi?
|
• 0-3 months: Group B Strep., E. coli, L. monocytogenes, viral (HSV, enteroviruses) • 3 months-3 years: S. pneumoniae, N. meningitidis, TB, viral (enteroviruses, herpes
virus 6, HSV) • 3-18 years: S. pneumonia/?, N. mt'ningitidis, viral (enteroviruses, adenoviruses, herpes viruses) URTI ---> compromise in integrity of mucosa ---> blood stream invasion from respiratory tract ---> hematogenous seeding of meninges ---> meningeal and CNS inflammation |
|
|
CLinical presentation of meingitis?
|
• toxic
• ± URTI prodrome • fever, lethargy, irritability, photophobia, nausea/vomiting, headache, stiff/sore neck • younger infants: may not demonstrate localizing signs, may have non-specific symptoms (poor feeding, irritability, lethargy), bulging fontanelle, increasing head circumference • signs of meningismus • Brudzinski's sign: reflex flexion of hips and knees upon active flexion of the neck • Kernig's sign: reflex contraction and pain in hamstrings upon extension of leg that is flexed at the hip • opisthotonos: spasm in which head and heels are bent backward and body bowed forward • nuchal rigidity • signs of increased Iep: headache, diplopia, ptosis, CN VI palsy, bradycardia with hypertension, a~nea, papilledema is uncommon • seizure in 20-30 Yo of patients with bacterial meningitis • petechial rash: (meningococcemia) associated with poor prognosis |
|
|
CSF values in meningitis
|
|
|
|
What are the complications of meningitis?
|
• mortality: neonate 15-20%, children <1-8%; pnewnococcus > meningococcus> Hib • morbidity: up to 50% may have neurobchavioural morbidity, severe
neurodevelopmental sequelae in 10-20% • acute • SIADH -> hyponatremia -> brain edema seizures • subdural effusion/empyema • brain abscess, disseminated infection (osteomyelitis, septic arthritis, abscess) • shock/DIC • chronic • hearing loss • mental retardation, learning disability • neurological deficit, seizure disorder • hydrocephalus |
|
|
How to treat meningitis?
|
• isolation with appropriate infection control procedures until24hr after culture-sensitive antibiotic therapy
• bacterial: empiric antibiotics: vancomycin 60 mglkglday IV divided q6h x 7-14 days + cefotaxime 200 mglkglday IV divided q6h • if C&S indicates Gram negatives then d/c vancomycin and replace with an aminoglycoside (Canadian Pediatric Society Position Statement, 2001) • viral: supportive, acyclovir for HSV meningitis • most cases of viral meningitis can be sent home (except HIV) • if neonatal, use high dose ampicillin as part of regimen until GBS and Listeria ruled out • monitor: glucose, acid-base and volume status • anticonvulsants may be needed to treat seizures • prophylaxis • H. influenzae type b vaccine - routine • meningococcal vaccine - asplenism, complement deficiency, for outbreaks, routine in some provinces • pneumococcal vaccine - irnmunocompromised, asplenism, routine in some provinces • BCG vaccine - if born in TB-endemic area • antibiotic prophylaxis for contacts and index case • H. influenzae - rifampin • N. meningitidis - rifampin, ceftriaxone or ciprofloxacin • report to public health: acute meningitis (bacterial, viral, other) |
|
|
What are clinical features of AIDS in infants and children?
|
• signs and symptoms occur often within the first year, most within two years of age • encephalopathy
• recurrent/persistent thrush • chronic interstitial pneumonitis (relatively common); Pneumocystis Carinii Pneumonia (PCP) infection • hepatomegaly • FIT, opportunistic infections, lymphadenopathy |
|
|
What are the etiologies of pharyngitis and tonsillitis? Which ones resent with exudate? Which ones prsent with soft palate petechiae?
|
• viral (adenoviruses, enteroviruses, EBV virus) - 80% • bacterial (Group A Streptococcus - GAS) - 20%
• others: fungal (candida), Kawasaki's, retropharyngeal/peritonsillar abscess, epiglottitis, bacterial tracteitis • cannot be reliably distinguished on clinical features alone • exudative tonsillitis: GAS, adenovirus, EBV, diphtheria • soft palate petechiae: GAS, EBV |
|
|
Clinical manifestions of pharyngitis: viral vs bacterial
|
Viral:
- under 3 - gradual - low-grade fever - sore throat, rhinorrhea/cough, conjunctivits, hoarsenss, rash Bacterial: - over 3 abrupt > 38 fver sore throat, no rhinitis/cough, nausea, ab discomfort red pharynx, tender cervical nods, tonsillar exudats, palatal petechaie |
|
|
What are the clinccal features of GAS infection? How to diagnose?
|
• Group A Streptococcus (GAS) infection • most commonly school aged (4-13 yrs), uncommon in children <3 yrs
• McIsaac Criteria: no cough, tender anterior cervical lymphadenopathy, erythematous tonsils with exudate, fever >38°C, age 3-14 • score 0-1 (no culture, no antibiotic); 2-3 (culture, treat if positive); 4 (antibiotics) Fever >38°C Lymphadenopathy- anterior, tender, cervical Age 3-14 No Cough Erythematous, exudative tonsils >2 years old, culture before treatment or do rapid Strep antigen test • rapid Strep test only 70-90% sensitive (pick up 20% of carriers of GAS), culture if negative (throat swab for culture is gold standard, sensitivity 90-95%) |
|
|
How to treat symptomatic Step pharyn? WHy?
|
• symptomatic
• if 1 symptom, no culture or antibiotics • if>1 symptom, culture --+ antibiotics • penicillin V or amoxil 40 mglkglday PO divided bid x 10 days • erythromycin 40 mg/kglday PO divided tid x 10 days if allergic to penicillin • acetaminophen for discomfort • can prevent rheumatic fever if treated within 9-10 days • antibiotics do not alter the risk of post-streptococcal glomerulonephritis • tonsillectomy for proven, recurrent streptococcal tonsillitis • complications • if untreated, can lead to • suppurative complications: otitis media, sinusitis, cervical adenitis, pneumonia, mastoiditis • direct extension: retropharyngeal/ peritonsillar abscess • scarlet fever, rheumatIC fever • hematogenous spread: bone/joint infection, meningitis, SBE • acute glomerulonephritis (irrespective of antibiotic treatment) • invasive GAS disease: illness associated with isolation of GAS from normally sterile sites (blood, CSF, or pleural fluid) • treatment of invasive GAS disease • admit • N clindamycin 40 mglkg divided into 3-4 doses + rv penicillin 250000 - 400000 U/kglday divided into 6 doses |
|
|
What are other illnesses caused by GAS?
|
strep: impetigo, cellulitis, bacteremia, vaginitis, toxic shock syndrome
• streptococcal toxic shock: illness associated with isolation of GAS from normally sterile sites (blood, CSF, or pleural fluid) + hypotension, renal impairment, coagulopathy, liver impairment, RDS, rash, soft tissue necrosis (necrotizing fasciitis, myositis, or gangrene) |
|
|
What is scarlett fever?
|
• erythrogenic strain of Group A Streptococcus
• acute onset of fever, sore throat, strawberry tongue • 24-48 hours after ,pharyngitis, rash begins m the groin, axillae, neck, antecubital fossa • within 24 hours, sandpaper" rash becomes generalized with perioral sparing, non-pruritic, non-painful • rash fades after 3-4 days, may be followed by peeling • treatment: penicillin, amoxicillin or erythromycin (if penicillin allergic) x 10 days 4Sand4P Sore throat Strawberry tongue Sandpaper rash Perioral Sparing Non-Pruritic Non-Painful Peeling |
|
|
What is rheumatic fever? How to diagnose?
|
• Jones Criteria (revised) • requires 2 major OR 1 major and 2 minor PLUS evidence of preceding Strep
infection (history of scarlet fever, group A streptococcal pharyngitis culture, rapid Ag detection test (only useful if positive), anti-streptolysin 0 titers (ASOT) • major criteria: "SPACE" • subcutaneous nodules, pea-sized firm, non-tender nodules typically on extensor surfaces • pancarditis involving pericardium, myocardium, endocardium • arthritis (migratory): very tender, red, warm, swollen joints, affects mostly large joints • chorea (Sydenham's): may be characterized by clumsiness, difficulty with handwriting • erythema marginatum: begins as pink macules on trunk with central blanching; non-pruritic • mmor criteria • previous history of rheumatic fever or rheumatic heart disease • polyarthralgia • fever • elevated ESR or C-reactive protein or leukocytosis • prolonged PR interval |
|
|
How to treat RF? What are complications?
|
• treatment • penicillin or erythromycin for acute course x 10 days
• ASA for arthritis • prednisone if severe carditis • secondary prophylaxis with daily penicillin or erythromycin; course depends on: • without carditis: 5 years or until 21 years old, whichever is longer • with carditis but no residual heart disease (no valvular disease): 10 years or longer • carditis and residual heart disease (persistant valvular disease): at least 10 years since last episode, sometimes life long prophylaxis • complications • acute: myocarditis, conduction system (sinus tachycardia, atrial fibrillation), valvulitis (acute MR), pericarditis • chronic: rheumatic vafvular heart disease - mitral and/or aortic insufficiency/stenosis, increased risk of infectious endocarditis ± thromboembolic phenomenon • onset of symptoms usually after 10-20 year latency from acute carditis of rheumatic fever |
|
|
What are the clinical features and complications of mono?
|
Clinical Features
• prodrome: 2-3 days of malaise, anorexia • infants and young children: often asymptomatic or mild disease • older children and young adults: may develop typical infectious mononucleosis syndrome • fever, tonsillar exudate, generalized lymphadenopathy, pharyngitis ± hepatosplenomegaly ± rash (rash more frequent with patients treated with amoxicillin/arnpicillin) • any "-itis" (including arthritis, hepatitis, nephritis, myocarditis) chronic fatigue • resolves over 2-3 weeks although fatigue may persist for several months • administration of amoxiciIlin results in rash in >90% of cases Complications • aseptic meningitis, encephalitis, Guillain-Barn~, splenic rupture, agranulocytosis, myocarditis (rare) |
|
|
How to Dx and Tx mono?
|
Diagnosis
• heterophil antibody test (Monospot™ test) 85% sensitive in adults and older children, only 50% sensitive <4 yrs of age • false positive results with HN, SLE, lymphoma, rubella, parvovirus • EBV titres • CBC + differential: atypical lymphocytes, lymphocytosiS, Downey cells, ± anemia, ± thrombocytopenia Treatment • throat culture to rule out streptococcal pharyngitis • supportive care (bed rest, fluids, saline gargles for sore throat, acetaminophen) • if airway obstructed secondary to node and/or tonsillar enlargement, admit to hospital, steroids • patients with splenic enlargement often not apparent clinically so all patients should avoid contact sports for 6-8 weeks • acyclovir not useful |
|
|
What is the clinical presentation of pertussis? How to Dx and treat?
|
Clinical Presentation
• prodromal catarrhal stage • 1-2 weeks, most contagious • coryza, mild cough • paroxysmal stage • 2-4 weeks • paroxysms of cough, sometimes followed by inspiratory whoop (whoop may be absent in children <6 months or adults) • infants may present with apnea • ± vomiting with coughing spells onset of attacks precipitated by yawning, sneezing, eating, physical exertion • can have severe symptoms for 6 weeks, cough for 6 months • pressure effect - subconjunctival hemorrhage, rectal prolapse, hernias, epistaxis • convalescent stage • 1-2 weeks, noninfectious • occasional paroxysms of cough, but decreased frequency and severity, lasts up to 6 months Diagnosis • clinical: URTI symptoms followed by paroxysms of cough in an afebrile child • lymphocytosis • PCR of nasopharyngeal swab or aspirate Treatment • supportive care • hospitalize if paroxysms of cough are associated with cyanosis and/or apnea, (give 02) • erythromycin 40 mglkg/day x 10 days started within 3 weeks after onset of cough • isolate until 5 days of treatment • treatment will decrease infectivity but not change course of illness • shortens period of communicability • antibiotic prophylaxis: erythromycin for all household contacts • prevention: acellular pertussis vaccine (PentaceI™) in infants and children, and pertussis booster (AdaceFM) in adolescents and adults |
|
|
What are complications of pertussis?
|
• otitis media • respiratory complications
• sinusitis, secondary pneumonia, atelectasis, pneumomediastinum, pneumothorax, interstitial or subcutaneous emphysema secondary to ruptured alveoli • neurological complications • seizures, encephalopathy (1:100,000), intracranial hemorrhage |
|
|
What is the clinical presentation of chicken pox? What are complications?
|
Clinical Presentation
• 1-3 day prodrome: fever and respiratory symptoms • characteristic polymorphous rash • very pruritic crops of red macules which quickly become vesicles surrounded by erythema "dewdrop on erythematous base" • vesicles burst and lesions crust over • on trunk, face, scalp, conjunctivae, vagina, oral mucosa, palms and soles new crops usually stop forming after 5-7 days Complications • secondary bacterial infection (most common) • infection with Staph, GAS • presents as impetigo, abscesses, cellulitis, necrotizing fasciitis, sepsis • cerebellar ataxia, pneumonia, hepatitis, encephalitis • immunocompromised patients: varicella may be life-threatening • neonates born to mothers who develop varicella from 5 days before to 2 days after delivery are considered high risk • must administer varicella-zoster immune globulin (VZ1G) and follow for signs of infection/sepsis • virus latent in sensory ganglia and reappears as herpes zoster in 68/100,000 individuals • incidence is increased in immunocompromised patients 2ndary bacterial infection Hepatitis Cerebellar Ataxia Pneumonia Encephalitis |
|
|
What is roseola? What causes it? How does it present? How to treat? COmplications?
|
• human herpes virus 6
• incubation: 5-15 days; infectivity and spread: unknown • typically affects children <3 years Clinical Presentation • high fever (>39.5) lasting 3-5 days, cough, respiratory symptoms, nasal congestion • pharynx, tonsils and tympanic membranes are erythematous • cervical, posterior cervical lymphadenopathy, bulging anterior fontanelle (if eNS involvement) • fever ceases before rash appears • pink non-pruritic macules and maculopapules • macules coalesce and disappear in 1-2 days Treatment • supportive (acetaminophen) Complications • febrile seizures • encephalitis |
|
|
What is measles? What causes it? How does it present? How to treat? COmplications?
|
• morbiLLivirus
• incubation: 10-14 days; infectivity: 4 days pre-rash, spread by airborne route Clinical Presentation • prodrome: "3 C's" - cough, coryza, conjunctivitis, fever, eyelid edema • Koplik spots (1-2 days before and after rash): small white papules on red base on buccal mucosa • maculopapular rash spreads over face and hairline spreading in a descending fashion over the body over 3 days Diagnosis • clinical examination and positive serology for measles 19M Treatment • supportive and symptomatic (appropriate treatment of secondary bacterial infection) • prophylactic immlilloglobulin to prevent disease if administered within 6 days of exposure • vitamin A supplementation in selected children Complications • secondary bacterial infection (laryngotracheobronchitis, otitis media, sinusitis), bronchopneumonia, croup • encephalitis (1:1,000): ataxia, vomiting, seizures, coma • subacute sclerosing panencephalitis (1:100,000): slow measles virus infection of brain manifesting years later, characterized by progressive cerebral deterioration with myoclonic jerks, fatal within 6-12 months |
|
|
What is mumps? What causes it? How does it present? How to treat? COmplications?
|
• paramyxovirus
• incubation: 12-25 days; infectivity: 7 days pre-parotitis to 7 days post-parotitis, spread by droplets • diagnosis: urine or saliva for viral serology Clinical Presentation • fever, headache, parotitis (bilateral; pushes earlobes up and out), myalgia, malaise • 30-40% of cases are subclinical with minimal symptoms Treatment • supportive Complications • meningoencephalomyelitis: over 10% of patient with parotitis • orchitis, epididymitis, infertility • pancreatitis: may see elevated serum amylase without symptoms • other: ocular complications, thyroiditis, hearing impairment, myocarditis, arthritis, thrombocytopenia, cerebellar ataxia, glomerulonephritis |
|
|
What is rubella? What causes it? How does it present? How to treat? COmplications?
|
• rubivirus
• incubation:14-21 days; infectivlty: 7 days pre-rash to 5 days post-rash, spread by droplets • diagnosis: serology for rubella IgM; may not be detected 4-5 days after rash onset Clinical Presentation • prodrome of nonspecific respiratory symptoms and adenopathy (suboccipital) • rash • maculopapular, initially on face, then spreading to entire body • pruritic, disappearing by fourth day • congenital rubella syndrome (CRS) • mother infected in first 4 months of pregnancy • infection in utero, failure of rubella vaccine is <5% and rarely results in CRS • cataracts/congenital glaucoma, congenital heart disease, hearing impairment (common), purpura ("blueberry muffin baby), hepatosplenomegaly, jaundice, microcephaly, developmental delay, radiolucent bone disease • prevention: routine childhood immunization, assure immunity of women of childbearing age with vaccination Treatment • symptomatic Prognosis • excellent prognosis in patients with acquired disease • irreversible defects in congenitally infected patients Complications • arthritis/arthralgia: polyarticular (fingers, wrists, knees), lasts days to weeks • encephalitis |
|
|
What is Reye syndrome?
|
• acute hepatic encephalopathy and noninfIammatory fatty infiltration of liver and kidney • mitochondrial injury of unknown etiology results in reduction of hepatic
mitochondrial enzymes, diagnosis by liver biopsy • associated with aspirin ingestion by children with varicella or influenza infection • 40% mortality Clinical Presentation • vomiting • hyperventilation, tachycardia, decerebrate posturing • respiratory failure • agitated delirium, coma, death Treatment • should be tailored based on severity of presentation • IV glucose (to counteract effects of glycogen depletion) • fluid restriction, mannitol (if cerebral edema) • prevention: avoid aspirin with viral illness |
|
|
What is erythema infectiosum? How does to present? How to treat? COmplications?
|
• parvovirus B19, "fifth disease" • incubation: 4-14 days; infectivity: prior to onset of rash
Clinical Presentation • initial 7-10 days: flu-like illness with fever • day 10-17: rash appears (immune response) • raised maculopapular lesions on cheeks ("slapped cheek" appearance), forehead, chin, circumoral sparing • warm, nontender, may be pruritic, may also appear on extensor surfaces, trunk, neck, buttocks • days to weeks: rash fades, may reappear with local irritation (heat, sunlight) Treatment • supportive • blood transfusions for some with aplastic crisis Complications • arthritis (10%, pain and stiffness in peripheral joints), vasculitis, fetal loss in pregnancy • aplastic crisis: reticulocytopenia occurs for 1 week during illness, unnoticed in normal individuals, but severe anemia in patients with chronic hemolytic anemia |
|
|
Neonatal classifications by birth weight?
|
• small for gestational age (SGA): 2 SO < mean weight for GA or <10
percentile • appropriate for gestational age (AGA): within 2 SO of mean weight for GA • large for gestational age (LGA): 2 SO > mean weight for GA or >90 percentile th th |
|
|
Go through the steps of initial resuscitation
|
• anticipation - know maternal history, history of pregnancy, labour, and delivery • all infants ("before ABC's")
• provide warmth: warm (radiant heater, warm towels), dry (remove wet towels) • position & clear airway: "sniffing" position • stimulate infant (if needed): rub back gently or flick soles of feet EXCEPT if meconium present • Airway • gentle suction of mouth then nose • if meconium and baby vigorous (strong respiratory effort, good muscle tone, HR >100): suction nasopharynx after delivery of head • if meconium and baby not vigorous: free flow 0" intubate and suction trachea • Breathing • if HR <100 or apneic apply positive pressure ventilation (PPV) • PPV at rate of 40-60/min 100% 02 with enough pressure to see visible chest expansion • Circulation • if HR <60, apply chest compressions ("60 or less, compress") • chest compressions at lower 1/3 of the sternum at 1/3 of the AP depth at a rate of 120 events/min (3 compressions: 1 ventilation = 90 compressions/min: 30 breaths/min) |
|
|
What are signs of sepsis in a neonate?
|
• no reliable absolute indicator of occult bacteremia in infants <3 months, most consistent result has been WBC >15
• temperature instability (hypo/hyperthermia) • respiratory distress, cyanosis, apnea • tachycardialbradycardia • lethargy, irritability • poor feeding, vomiting, abdominal distention, diarrhea • hypotonia, seizures, confusion, lethargy, coma • jaundice, hepatomegaly, petechiae, purpura |
|
|
Causes and management of cyanosis?
|
• peripheral cyanosis • can be normal transiently but may indicate sepsis, temperature instability
• central cyanosis • due to poor oxygenation - decreased Sa02' decreased Pa0 • secondary to • respiratory insufficiency • cardiac (congenital heart disease [CHD], PPHN) • CNS (asphyxia) • hematologIc (polycythemia) • sepsis Management • ABGs or capillary blood gas • hyperoxic test (to rule out CHD): get baseline p02 in room air, then p02 on 100% 02 x 10-15 min • p02 <150 mmHg: suggests congenital heart disease 2 (see Pediatric Cardiology, PIS) pO. >150 mmHg: suggests respiratory (airway, chest, lungs), brain or blood problems |
|
|
What is apnea? DD? Managemtn?
|
• absence of respiratory gas flow for more than 15-20 seconds (less if associated with bradycardia or cyanosis)
• central: no chest wall movement • obstructive: chest wall movement continues • mixed: combination of central and obstructive apnea Differential Diagnosis • in term infants, apnea always requires full work-up • apnea <24 hrs - strongly associated with sepsis • apnea >24 hrs • CNS: • apnea of prematurity: combination of CNS prematurity and obstructive apnea, resolves by 36 weeks GA, diagnosis of exclusion • seizures • intracranial hemorrhage (ICH) • hypoxic injury • infectious: sepsis, meningitis, necrotizing enterocolitis • GI: gastroesophageal reflux disease (GERD), aspiration with feeding • metabolic: hypoglycemia, hyponatremia, hypocalcemia • cardiovascular: low and high blood pressure, anemia, hypovolemia, PDA, heart failure • drugs: morphine Management • correct underlying cause, monitor • 02' continuous positive airway pressure (CPAP), ventilation • tactile stimulation • medications • methylxanthines (caffeine) stimulate the CNS and diaphragm |
|
|
DD of res distress in neonate
|
• pulmonary • respiratory distress syndrome (RDS)
• transient tachypnea of the newborn (TIN) • meconium aspiration syndrome (MAS) • pleural effusions, pneumothorax • congenital lung malformations • infectious • sepsis • pneumonia (GBS + others) • cardiac • congenital heart disease (cyanotic, acyanotic) • persistent pulmonary hypertension (PPHN) • hematologic • blood loss • polycythemia • anatomic • tracheoesophageal fistula • congenital diaphragmatic hernia • upper airway obstruction (see Otolaryngology. OT43) • choana 1atresia • Pierre-Robin sequence (retrognathia and/or micrognathia plus cleft palate, and glossoptosis) • laryngeal (malacia) • tracheal (malacia, vascular ring) • mucous plug • cleft palate • metabolic • hypoglycemia • inborn errors of metabolism (amino acidemia, organic acidemia, urea cycle disturbance, galactosemia, 1 lactic acidosis) • neurologic 0 • CNS damage (trauma, hemorrhage) • drug withdrawal syndromes |
|
|
What is the pathophys of RDS? Risk factors?
|
Pathophysiology
• surfactant deficiency --> poor lung compliance due to high alveolar surface tension and atelectasis --> decreased surface area for gas exchange --> hypoxia + acidosis --> respiratory distress • surfactant decreases alveolar surface tension, improves lung compliance and maintains functional residual capacity Risk Factors • premature babies: rare at term, risk is inversely proportional to birth weight and GA; sufficient surfactant production usually by 36 weeks • infants of diabetic mothers (IDM): insulin inhibits the cortisol surge necessary for surfactant synthesis • C-section without labour • asphyxia, acidosis, sepsis, meconium aspiration • males> females • hypothermia |
|
|
Clinical presentation of RDS? How to prevent or treat?
|
Clinical Presentation
• symptoms of respiratory distress • onset within first few hours of life, worsens over next 24-72 hours • infants may develop respiratory failure and require ventilation ---_..... .... ' , .'}-~~~~~~~~--, RDS is the most common cause of res piratory distress in the pre-term infant. .... ' , .'}-----------, • CXR: decreased aeration and lung volumes, reticulonodular pattern throughout lung fields with air bronchograms, atelectasis; may resemble pneumonia, if severe can see 'Ground glass' appearance of lungs is white-out pathognomonic of RDS. Prevention • steroid therapy (e.g. Celestone™) for mothers (12 mg q24h x 2 doses) prior to delivery of premature infants • monitor lecithin:sphingomyelin (LIS) ratio with amniocentesis, L/S >2:1 indicates lung maturity Treatment • supportive • O~, assisted ventilation (PEEP, CPAP, or intubation), and nutrition • administer fluids cautiously to avoid pulmonary edema • endotracheal surfactant administration (4 ml/kg/dose, can repeat q6-12h x 2 doses) |
|
|
What prognoisis and complications in RDS?
|
Prognosis
• in severe prematurity and/or prolonged ventilation, increased risk of bronchopulmonary dysplasia (BPD) Complications • bronchopulmonary dysplasia (BPD) • pulmonary air leaks (pneumothorax) |
|
|
What is TTN? How to is presnt? HOw treated?
|
• also known as "wet lung syndrome" and respiratory distress syndrome type II
Pathophysiology • delayed resorption of fetal lung fluid -> accumulation of fluid in peribronchial lymphatics and vascular spaces -, tachypnea Risk Factors • full term or slightly premature infant • no labour/short labour (hypothesized lack of catecholamine release) • C-section (lungs are not compressed during passage through pelvic floor) • diabetic mother Clinical Presentation • tachypnea within the first few hours of life, mild retractions, grunting, nasal flaring, without signs of severe respiratory distress • usually resolves in :!4-n hours • CXR: fluid in fissures, increased vascularity, slight cardiomegaly Treatment • supportive: 02' nutrition, careful fluid administration • full recovery expected within 2-5 days |
|
|
What is mconium aspiration syndrome? Presntation? Complications? Treatmnt? Prvention?
|
• 10-15% of an infants are meconium stained at birth, -5% of meconium stained infants get MAS (higher incidence with thick meconium)
• usually associated with fetal distress in utero, or post-term infant Clinical Presentation • respiratory distress within hours of birth • small airway obstruction, chemical pneumonitis -> tachypnea, barrel chest with audible crackles • Dill: hyperinflation, streaky atelectasis, patchy infiltrates • 10-20% have pneumothorax Complications • hypoxemia, hypercapnea, acidosis, PPHN, pneumothorax, pneumomediastinum, pneumonia, sepsis, respiratory failure, death Treatment • supportive care and ventilation • may benefit from surfactant replacement (surfactant function is inhibited by meconium irritation) • inhaled NO, extracorporeal membrane oxygenation Prevention • in utero: careful monitoring • after delivery of the head: suction nas%ropharynx • at birth: intubate and suction below cords |
|
|
Clinical presntation of diaphragmatic hernia?
|
• if resuscitation required at birth, DO NOT bag because air will enter stomach and compress lungs
Clinical Presentation • respiratory distress, cyanosis • scaphoid abdomen and barrel-shaped chest • affected side dull to percussion and breath sounds absent; may hear bowel sounds instead • asymmetric chest movements, trachea deviated away from affected side • resultant pulmonary hypoplasia • may present outside of neonatal period • often associated with other anomalies (cardiovascular, CNS lesions) • CXR: portion of GI tract in thorax (usually left side), displaced mediastinum |
|
|
What is the pathophys of perisitent pulmonary hypertnsion of the newbown? What are risk factors? How to investigate? How to treat?
|
Pathophysiology
• severe hypoxemia due to persistence of fetal circulation • R to L shunt through PDA, foramen ovale, intrapulmonary channels --> decreased pulmonary blood flow and hypoxemia --> further pulmonary vasoconstriction Risk Factors • asphyxia, MAS, RDS, sepsis, structural abnormalities (e.g. diaphragmatic hernia) Investigations • echocardiogram reveals increased pulmonary artery pressure and a R --> L shunt, also used to rule out other cardiac defects Treatment • a, given early and tapered slowly, minimize stress and hypoxia, alkalinization, inotropes (to make systemic pressure greater than pulmonary pressure) • mechanical ventilation, high frequency oscillation (BFa), extracorporeal membrane oxygenation (ECMO) |
|
|
What is BPD? How to treat?
|
• usually after prolonged intubation/ventilation with high pressures and high O
concentration (often after ventilation for RDS) • a, requirement at 28 days/36 wks GA and abnormal CXR findings (lung opacification, then cysts with sites of over distention and atelectasis, appears spongy) • chronic respiratory distress leads to pulmonary hypertension, poor growth, right-sided heart failure Treatment • gradual wean from ventilator, optimize nutrition, stress avoidance, diuretics, bron chodilators • dexamethasone may help decrease inflammation and encourage weaning 2 |
|
|
How to classify hypoglycemia in a baby? Etiology? CLinical findings?
|
• glucose <2.2 mmol/L (40 mgldL) in full term infant; <2.6-2.8 mmol/L (47-51 mg/dL) in preterm
Etiology • decreased carbohydrate stores (premature, illeR) • infant of a diabetic mother (IDM): maternal hyperglycemia --> fetal hyper~lycernia and hyperinsulinism --> hypoglycemia in the newborn infant because of high msulin levels • sepsis • endocrine: hyperinsulinism due to islet cell hyperplasia (e.g. Beckwith-Wiedemann syndrome), panhypopituitarism • inborn errors of metabolism: fatty acid oxidation defects, galactosemia Clinical Findings • signs often non-specific and subtle: lethargy, poor feeding, irritability, tremors, apnea, cyanosis, seizures |
|
|
How to manage hypoglyemia in infant?
|
• identify and monitor infants at risk, (pre-feed blood glucose checks)
• begin oral feeds within first few hours of birth • if hypoglycemic, provide glucose IV (DlO, D12.5) • if persistent hypoglycemia (past day 3), hypoglycemia unresponsive to IV glucose, and/or no predisposing cause for hypoglycemia, send the following "critical bloodwork" during an episode of hypoglycemia • insulin • cortisol • growth hormone (GIl) ~-hydroxybutyrate • lactate • ammonia • free fatty acids (FFA's) • ABG • treat hyperinsulinism with diazoxide, glucagon |
|
|
When is jaundice visible?
|
serum bilirubin levels of 85-120 flll10lIL (5-6 mgldL)
|
|
|
Classifications of jaundice
|
|
|
|
When must jaundice be investigated in an infant?
|
• jaundice at <24 hours of age • serum unconjugated bilirubin rises rapidly or is excessive for patient's age and
weight (>85 flmollL per day or >220 flmollL before 4 days of age) • conjugated bilirubin >35 flmollL (2.0 mg/dL) • persistent jaundice lasting beyond 1-2 weeks of age |
|
|
What investigations must be made for pathologic jaundice?
|
• hemolytic work-up: CBC, blood group (mother and infant), peripheral blood
smear, Coombs test, bilirubin (conjugated, unconjugated) • if baby is unwell or has fever, septic work-up: CSC + differential, blood and urine cultures, CXR ± LP • other: TSH, G6PD screen (in males) • conjugated hyperbilirubinemia: consider liver enzymes (AST, ALT), coagulation studies (IT, PTT), serum albumin, ammonia, TSH, TORCH screen, septic work-up, galactosemia screen (erythrocyte galactose-I-phosphate uridyltransferase levels), metabolic screen, abdominal VIS, HlDA scan, sweat chloride |
|
|
treatment of unconjugated hyprbilirubinemia
|
• to prevent kernicterus (see below) • breast feeding does not need to be discontinued, ensure adequate feeds and hydration
• get lactation consultant support, mother to pump after feeds • treat underlying causes (e.g. sepsis) • phototherapy • insoluble unconjugated bilirubin is converted to excretable form via photoisomerization • serum bilirubin should be monitored during and immediately after therapy (risk of rebound because photoisomerization reversible when phototherapy discontinued) • contraindicated in conjugated hyperbilirubinemia: results in "bronzed" baby side effects: hypematremic dehydration, eye damage, skin rash, diarrhea • exchange transfusion • prevents toxic effects of bilirubin by removal from body • mdications: depending on level and rate of rise of bilirubin • most commonIy performed for hemolytic disease (G6PD) |
|
|
What is kernicterus? Why is it dangerous?
|
• unconjugated bilirubin concentrations exceed albumin binding capacity and bilirubin enters and is deposited in the brain resulting in permanent damage (often basal
ganglia or brainstem) • incidence increases as serum bilirubin levels increase above 340 flmollL (19.8 mg/dL) • can occur at lower levels in presence of sepsis, meningitis, hemolysis, hypoxia, hypothermia, hypoglycemia and prematurity • up to 15% of infants have no obvious neurologic symptoms • acute form • first 1-2 days: lethargy, hypotonia, poor feeding, high-pitched cry, emesis, seizures • middle of first week: hypertonia, opisthotonic posturing, fever, bulging fontanelle, pulmonary hemorrhage • chronic form (first year and beyond) • hypotonia, delayed motor skills, extrapyramidal abnormalities (choreoathetoid cerebral palsy), gaze palsy, MR, sensorineural hearing loss • treatment: exchange transfusion • complications: sensorineural deafness, choreoathetoid cerebral palsy (CP), gaze palsy, mental retardation |
|
|
Biliary atresia
|
BILIARY ATRESIA
• atresia of the extrahepatic bile ducts • cholestasis and l' conjugated bilirubin after the first week of life • incidence: 1/10,000-15,000 live births Clinical Presentation • dark urine, pale stool, jaundice (persisting for >2 weeks), abdominal distention, hepatomegaly Diagnosis • HillA scan Treatment • surgical drainage procedure • hepatoportoenterostomy (Kasai procedure most successful if before 8 weeks of age) • usually requires liver transplantation • Vitamins A, D, E, and K. Diet should be enriched with medium-chain triglycerides to ensure adequate fat ingestion |
|
|
DD of bleeding disorders in a newbown
|
• increased platelet destruction: maternallTP, neonatal alloimmune thrombocytopenia purpura (NATP), infection, DIC, drugs, extensive localized
thrombosis, critically ill infants, giant hemangiomas, maternal lUpus • decreased platelet production: bone marrow replacement, pancytopenia, Fanconi anemia, trisomy 13 & 18 • mechanism undetermined: inborn error of metabolism, congenital thyrotoxicosis • hemorrhage disease of the newborn |
|
|
NEC
|
• intestinal inflammation associated with focal or diffuse ulceration and necrosis • primaril)' affecting terminal ileum and colon
• affects 1-5% of all newborns admitted to NICU Pathophysiology • postulated mechanism of bowel ischemia --> mucosal damage, and enteral feeding providing a substrate for bacterial growth and mucosal invasion, leading to bowel necrosis or gangrene and perforation Risk Factors • prematurity (immature defenses) • asphyxia, shock (poor bowel (Jerfusion) • enteral feeding WIth formula (breast milk can be protective) • sepsis Clinical Presentation • distended abdomen • increased amount + bile stained gastric aspirate/vomitus • frank or occult blood in stool • feeding intolerance • diminished bowel sounds • signs of bowel perforation - sepsis, shock, peritonitis, DIC Investigations • abdominal x-ray: intramural air ("train tracks"), free air, fixed loops, thickened bowel wall, portal venous gas • CBC, ABG, blood culture (25% will be positive at time of diagnosis) • high WBC, low platelets, electrolyte imbalance, acidosis, hypoxia, hypercapnea Treatment • ~PO (minimum 2-3 weeks), vigorous IV fluid resuscitation, NG decompression • TPN • antibiotics for infection (triple therapy given empirically: ampicillin, gentamicin, metronidazole x 7-10 days) • serial abdominal x-rays detect early perforation • surgical resection of necrotic bowel and surgery for complications (e.g. perforation, strictures) |
|
|
Differential diagnosis: pneumatosis imestinalis
|
NEe
Hirschsprung disease Pseudomembranous enterocolitis Neonatal ulcerative colitis Ischemic bowel disease |
|
|
What is IVH? What are the risk factors?
|
NEe
Hirschsprung disease Pseudomembranous enterocolitis Neonatal ulcerative colitis Ischemic bowel disease |
|
|
What are the classifications of IVH?
|
• screening and cliagnosis by U/S on first day of life if risk factors fresent (50% of IVH occurs during the first 6-12 hours of life) ± crIMRl at 4-7 days 0 life (detects 90-100%
of all hemorrhages) • grade I: GM hemorrhage • grade II: IVH without ventricular dilatation • grade III: IVH with ventricular dilatation • grade IV: GM hemorrhage or IVH with parenchymal involvement |
|
|
How to manage IVH? What is the prognosis?
|
Management of Acute Hemorrhage
• supportive care to maintain blood volume and acid-base status • avoid fluctuations in blood pressure and cerebral blood flow • follow-up with serial imaging Prognosis • short-term outcomes: mortality and posthemorrhagic hydrocephalus (PHH) • long-term major neurological sequelae: cerebral palsy, cognitive deficits, motor deficits, visual and hearing impairment Prognosis of IVH Classification Mortality PHHrate Neurological sequelae mild to moderate IVH 5-10% 5-20% 5-15% severe IVH 20% 55% 30-40% severe IVH and parenchymal involvement -50% -80% as high as 100% |
|
|
What is retinopathy of prematuriy? What is pathophys? Risk factors?
|
• interruption in the progression of developing retinal vessels
Pathophysiology ----------' • early vasoconstriction and obliteration of the capillary bed . ~ neovascularization -> detachment of the retina blindness Risk Factors • association with period of high oxygen concentrations is now not so clear • extreme prematurity is the most significant risk factor • maternal complications, apnea, sepsis, hyper- and hypocapnea, vitamin E deficiency, intraventricular hemorrhage, anemia, exchange transfusion, hypoxia, lactic acidosis, and bright light |
|
|
How to assess and manage ROP?
|
Assessment
• ophthalmoscopic examination • infants weighing :<;:1500 g or $18 weeks GA: starting at 4-6 weeks cf chronologie age or at 32 weeKS corrected age (whichever is later) with exams g2-3 weeks until retinal maturity with no disease • infants with ROP or very immature vessels: exams ql-2 weeks Management • cryotherapy or laser photocoagulation • follow-up eye examinations for myopia, strabismus, amblyopia, glaucoma, and late detachment Prognosis • stage I and II: 90% spontaneous regression • stage III+: -50% spontaneous regression; with treatment, incidence of severe visual impairment reduces by -50% |
|
|
How to assess dehydration?
|
Capillary refill
BP Anterior fontanelle Skin turgor Eyes sunken HR Oral mucosa Output of urine |
|
|
What are the electrolyte requiremwnts/
|
Na 3meq/kg/d
K 2 Cl 3 |
|
|
Hematuria in neonate
|
Asphyxia, malformation, trauma, renal vein thrombosis
|
|
|
Cola/red-coloured urine
|
Hemoglobinuria (hemolysis) Myoglobinuria Irhabdomyolysis) Hematuria (e.g, glomerulonephritis), pigmenturia
|
|
|
Edema
|
Nephrotic syndrome, nephritis, acute/chronic renal failure
(also consider cardiac or liver disease) |
|
|
Hypertension
|
Acute glomerulonephritis, renal failure, dysplasia (also consider coarctation of aorta, drugs,
endocrine causes) |
|
|
Oliguria
|
Dehydration, acute tubular necrosis (ATN), interstitial nephritis
|
|
|
Hematuria key points
|
• 0.5-2% prevalence of asymptomatic microscopic hematuria in school-aged children • history of prior acute infection (upper respiratory, skin or GI) • family history: dialysis, transplant, SLE, familial hematuria
• physical exam: SP, edema, rashes, arthritis |
|
|
What are the etiologies of hematuria? Invest?
|
• nephrologic • glomerular disease:
• recurrent gross hematuria: IgA nephropathy, benign familial hematuria, Alport syndrome • post-streptococcal GN, lupus nephritis, HSP, HUS, Goodpasture disease (rare in childhood) • tubulointersitial: ATN, interstitial nephritis, pyelonephritis, hypercalciuria • infection: bacterial, TB, viral, UTI, pyelonephritis • hematologic: coagulopathies, thrombocytopenia, sickle cell disease or trait, renal vein thrombosis • nephrolithiasis • anatomic abnormalities: congenital, trauma, polycystic kidneys, vascular abnormalities, tumours (Wilms) • other: exercise, drugs • CBC, urine dip and culture, creatinine, BUN, 24hr urine collection for creatinine, protein, Ca, serum C3 and C4level • other if suspected: antistreptolysin 0 titer, ANA, throat swab • if above do not yield a diagnosIs, consider D/S ± Doppler to rule out structural anomalies • plain abdominal x-ray if nephrolithiasis is suspected |
|
|
What is asymptomatic hematura?
|
• definition: 5-10 RBC/HPF of centrifuged urine • usually found on routine screening
• dipsticks are very sensitive, but have a high false positive rate • 5% of school-aged children on single test but <1 % on repeated testing • benign familial hematuria in 2/3 of cases • sporadic or familial • no associated proteinuria |
|
|
Pathophys of HUS?
|
E. coli 0157:H7 verotoxin ("hamburger disease") or shiga toxin • toxin binds, invades and destroys colonic epithelial cells, causing bloody diarrhea • toxin enters the systemic circulation, attaches and injures endothelial cells
(especially in kidney) causing a release of endothelial products (e.g. von Willebrand factor, platelet aggregating factor) form platelet/fibrin thrombi in multiple organ systems (e.g. renal, pancreas, brain) resulting in thrombocytopenia • RBCs are forced through occluded vessels resulting in fragmented RBC (schistocytes) and removed by the reticuloendothelial system (hemolytic anemia) • other, rare forms of I illS in childhood are due to bacteria (e.g. S. pneumoniae), viruses, familial inheritance, or drugs |
|
|
Clinical pres of HUS?
|
• triad: acute renal failure, thrombocytopenia, rnicroangiopathic hemolytic anemia • initial presentation of abdominal pain and diarrhea, followed by bloody diarrhea • within 5-7 days the patient begins to show signs of anemia, thrombocytopenia and
renal insufficiency • history: weakness, lethargy, oliguria • physical exam: pallor, jaundice (hemolysis), edema, petechiae, hypertension • investigations: CBC, platelets, blood smear, urinalysis, BUN, creatinine • prognosis: 5-10% mortality, 10-30% kidney damage • supportive treatment; nutritional support; monitor electrolytes; dialysis if electrolyte abnormality cannot be corrected, fluid overload, or BUN >100 mgldL; PRBC for symptomatic anemia • steroids not helpful; antibiotics not indicated |
|
|
nephritic syndrome?
|
Proteinuria (<50 mg/kgldl Hematuria
Azotemia RBC casts Oliguria Hypertension acute, subacute or chronic • hematuria with RBC casts, proteinuria (<50 mglkglday, not nephrotic-range), azotemia, hypertension • renal failure (oliguria) |
|
|
Post-Streptococcal Glomerulonephritis
|
antigen-antibody mediated complement activation • most common in children, especially in 4-8 year olds, M > F
• occurs 1-3 weeks following group A ~-hemolytic streptococcal infection of skin or throat • diffuse, proliferative glomerulonephritis • diagnosed by elevated serum antibody titres against strep antigens (ASOT, anti-D~Ase B) • 95% of children recover completely within 1-2 weeks • 5-10% have persistent hematuria • management: • symptomatic treatment: fluid restriction, antihypertensives, diuretics • in severe cases: hemodialysis or peritoneal dialysis may be necessary • eradication of inlection (penicillin or erythromycin) |
|
|
Major Causes of Acute Glomerulonephritis with depressed C3
|
Renal Post-infectious GMN
Membranoproliferative Type I (50-80%) Type II (>80%) Systemic SLE SBE Shunt nephritis Cryoglobulinemia |
|
|
Major Causes of Acute Glomerulonephritis with normal C3
|
IgA Nephropathy
Idiopathic rapidly progressive GMN Anti-GBM disease Polyarteritis nodosa Wegener's granulomatosis Goodpasture's syndrome Henoch-Schiinlein purpura (HSP) |
|
|
Ne~hrotic Syndrome
Clinical Presentation |
• severe proteinuria (>50 mg/kg/day or > 40 mg/m
/hr) • hypoalbuminemia <20 gIL «2.0 g/dL) • edema (usually first sign, initially see facial swelling, especially periorbital, and pretibial edema) • hyperlipidemia >5.17 mmol/L (200 mg/dL) • secondary findings: hypocalcemia, hyperkalemia, hyponatremia (likely a dilutional effect), hypercoagulability (decreased PTT) Proteinuria (>50 mg/kg/dl hypoAlbuminemia «20g/Lt hyperUpidemia Edema |
|
|
minimal change disease (MCD) (76%)
|
peak occurrence between 2-6 years of age, more common in boys than girls
(2:1 ) • often treated empirically with steroids without kidney biopsy, 90% steroid responsive • membranous glomerulonephritis (8%) • focal segmental glomerular sclerosis (FSGS) (7%) • membranoproliferative glomerulonephritis (5%) |
|
|
Secondary NS
|
• vasculitis
• infections (e.g. hepatitis B & C, syphilis, mV) • medications (e.g. captopril, penicillamine, NSAIDs, anticonvulsants) • malignancy • hereditary (e.g. sickle cell disease, Alport syndrome) • metabolic, inflammatory (e.g. lupus nephropathy, rheumatoid arthritis) |
|
|
Complications & invest
GN |
• risk of infections (e.g. spontaneous peritonitis, cellulitis, sepsis) • hypercoagulability due to decreased intravascular volume and antithrombin III
depletion (pulmonary embolism, renal vein thrombosis) • side effects of drugs (diuretics, steroids, immunosuppressants) • hypotension, shock, renal failure Investigations • to rule out secondary causes of NS: serum complement levels, BUN, Cr, serum chemistries, ANA, antistreptolysin 0 titre, in certain cases HIV, Hep B/C and syphilis titers • consider kidney biopsy if: • HTN (higher risk of FSGS), steroid resistant, frequent relapses (>2 relapses in 6 month period), low serum complement, decreased renal function • presentation before first year of life (high likelihood of congenital nephrotic syndrome) • presentation after 10 years of age to rule out more serious renal pathology than MCD |
|
|
Management GN
|
• salt and water restriction, diuretic may be required • optimal nutrition, including high-quality protein • daily weights to assess therapeutic progress • varicella antibody titre if not immune • pneumococcal vaccine after remission (avoid live vaccines)
• initial treatment of MCD: • oral prednisone (or equivalent) 60 mg/m /day in divided doses (max. dose 80 mg/day) for up to 12 weeks 2 • a negative tuberculin skin test should be performed before starting steroid medications • a measurable decrease in protein excretion may take at least 7 to 10 days following initiation of treatment, and proteinuria clears by third week of oral prednisone • up to 2/3 of patients experience relapses • if unresponsive to steroids, frequent relapses or steroid-resistant (proteinuria continues beyond 3 months): • consider renal biopsy or treat with cytotoxic agent (i.e. cyclophosphamide or chlorambucil), immunomodulating agents such as levamisole and cyclosporine A, and high-dose pulse corticosteroid with guidance of a pediatric nephrologist |
|
|
Hypertension in Childhood
Etiology |
• infants: renal artery/vein thrombosis, congenital renal disease, coarctation of the aorta, bronchopulmonary dysplasia, hypercalcemia
• 1-6 years old: renal artery stenosis, renal parenchymal disease, Wilms' tumor, neuroblastoma, coarctation of the aorta • 7-12 years old: renal parenchymal disease, abnormalities of the renal vasculature, endocrine cause (hyperthyroidism, hyperparathyroidism, Cushing, primary hyperaldosteronism), essential hypertension • adolescents: essential hypertension, renal parenchymal disease, endocrine cause • white coat hypertension |
|
|
Approach to the Child with a Suspected Convulsive Disorder
|
Did the child have a seizure?
NO~ Breath holding Night terror Benign paroxysmal vertigo Narcolepsy Pseudoseizures Syncope Tics Hypoglycemia TIA YES Investigations: electrolytes, calcium, magnesium, glucose, eBe EEG*, eSF, CT, tox screen, LP may be indicated Benign febrile seizures (most common) Hypoxic ischemic encephalopathy ("asphyxia") Intracranial hemorrhage, trauma (e.g. shaken baby syndrome) Ingestions/drug withdrawal Metabolic causes le.g. hypoglycemia, hypocalcemia, hyponatremia) eNS infections Idiopathic epilepsy Neurocutaneous syndromes Tumour/arteriovenous malformation Heart problems such as long QT syndrome and hypertrophic car diomyopathy are often misdiag nosed as epilepsy. Include cardiac causes of syncope in your differential diagnosis. particularly when the episodes occur during physical activit |
|
|
Generalized and Partial Seizures
|
• generalized tonic-clonic is the most common form of non-febrile seizures • absence seizures occur in 6-12% of epileptic children, uncommon <4 years or
>25 years of age • partial seizures constitute up to 40% of epileptic activity |
|
|
Recurrent Headache
|
Assessment
• if unremarkable history, and neurological and general phYSical exam is negative, likely diagnosis is migraine or tension-type headache • obtain CT or MRl if history or physical reveals red flags • inquire about level of disability, academic performance, after-school activities Differential Diagnosis • migraine, cluster • psychogenic factors or stress • organic causes with or without increased rcp • others: refractive errors, strabismus, sinusitis, malocclusion of the teeth Red Flags Abnormal neurological finding Seizuras Acute onset of severe headache Change in the type of headache |
|
|
ORGANIC HEADACHES
ICP? tumor? |
• organic etiology often suggested with occipital headache • with increased ICP
• etiology: brain tumours, hydrocephalus, meningitis, encephalitis, cerebral abscess, pseudotumour cerebri, subdural hematoma • characteristics: diffuse early morning headaches, early morning vomiting, headache worsened by increased Iep (cough, sneeze, Valsalva); as ICP increases, headache is constant and child is lethargic and irritable • without increased ICP • etiology: cerebral arteriovenous malformation (AVM), aneurysm, collagen vascular diseases, subarachnoid hemorrhage, stroke Suspect aspace occupying lesion if 1. Headache duration <1 month 2. No family history of migraine 3. Abnormal neurological findings 4. Gait abnormalities 5. Seizures |
|
|
Approach to hypotonic baby?
|
• decreased resistance to movement - "floppy baby" • proper assessment of tone requires accurate determination of gestational age
• history - obstetrical/perinatal, family exposures, regression in milestones • evaluate: • spontaneous posture (spontaneous movement, movement against gravity, frog leg position) important in evaluation of muscle weakness • joint mobility (hyperextensibility) • muscle bulk, presence of fasiculations • postural maneuvers • traction response - pull to sit, look for flexion of arms to counteract traction and head lag • axillary suspension - suspend infant by holding at axilla and lifting; hypotonic babies will slip through grasp because of low shoulder girdle tone • ventral suspension - infant is prone and supported under the abdomen by one hand; infant should be able to hold up extremities; inverted "U" posturing demonstrates hypotonia, i.e. baby will drape self over examiner's arms • investigations will depend on history and physical exam • rule out systemic disorders • blood glucose • enhanced CT of brain • peripheral CK, EMG, muscle biopsy • chromosome analYSis, genetic testing • treatment: counsel parents on prognosis and genetic implications; refer patients for specialized care, refer for rehabilitation, OT, PT, assess feeding abiHty |
|
|
Causes of hypotonia that respond to rapid treatment
|
treatment: hypokalemia, hypermagnesemia, acidemia,
toxins, drugs, hypoglycemia, seizure, infection, intracranial bleeding, hydrocephalus. |
|
|
DD of central hypotonia?
|
• chromosomal (e.g. Down syndrome, Prader-Willi, Fragile X)
• metabolic (e.g. hypoglycemia, kernicterus) • perinatal problems (e.g. asphyxia, ICH) • endocrine (e.g. hypothyroidism, hypopituitarism) • infections (e.g. TORCH) • CNS malformations • dysmorphic syndromes |
|
|
DD of peripheral hypotonia?
|
• motor neuron (e.g. spinal muscular atrophy, polio)
• peripheral nerve (e.g. Charcot-Marie-Tooth syndrome) • neuromuscular junction (e.g. myasthenia gravis) • muscle fibres (e.g. mitochondrial myopathy, muscular dystrophy, myotonic dystrophy) |
|
|
Neurofibromatosis (NF-1)Type I
• autosomal dominant but 50% are the resu |
autosomal dominant but 50% are the result of new mutations also known as von Recklinghausen disease incidence 1:3000, mutation in NFl gene on 17qll.2 (codes for neurofibromin protein)
learning disorders, abnormal speech development, and seizures are common diagnosis of NF-1 requires 2 or more of: • ~6 cafe-au-lait spots (>5 mm if prepubertal, >1.5 em if postpubertal) ~ neurofibromas of any type or one plexiform neurofibroma • ~ Lisch nodules (hamartomas of the iris) • optic glioma • freckling in the axillary or inguinal region • a distinctive bony lesion (e.g. sphenoid dysplasia, cortical thinning of long bones) • a first degree relative with confirmed NF-1 |
|
|
Neurofibromatosis (NF-2)Type II
|
autosomal dominant • incidence 1:33 000
• characterized by predisposition to form intracranial, spinal tumours • diagnosed when either bilateral CNVIII masses or a first-degree relative with NF-2 and either a neurofibroma, meningioma, glioma, or schwannoma • also associated with posterior subcapsular cataracts • treatment consists of monitoring for tumour development and surgery |
|
|
Sturge-Weber Syndrome
|
port-wine nevus syndrome in VI distribution with associated angiomatous malformations of brain causing contralateral hemiparesis and hemiatrophy,
also associated with seizure, glaucoma and mental retardation |
|
|
Tuberous Sclerosis
|
• autosomal dominant inheritance; 50% new mutations • adenoma sebaceum (angiokeratomas on face, often in malar distribution), Shagreen
patch (isolated raised plaque over lower back, buttocks), "ash leaf" hypopigrnentation seen with Wood's lamp (UV light) • cardiac rhabdomyomas, kidney angiomyolipoma, mental retardation and seizures • cerebral cortex tubers (areas of cerebral dysplasia); subependymal nodules (SEN) may evolve into giant cell astrocytomas (may cause obstructive hydrocephalus) • calcifications within the SEN are seen on CT, MRl (especially around the foramen of Munroe); these may obstruct the foramen and cause hydrocephalus |
|
|
What is the clinical presentation of leukemia?
|
• infiltration of leukemic cells into bone marrow results in bone pain, and subsequent bone marrow failure (anemia, neutropenia, thrombocytopenia, purpura, petechiae)
• infiltration into tissues results in: lymphadenopathy, hepatosplenomegaly, CNS manifestations • fever, fatigue, weight loss |
|
|
Clinical Presentation
Brain Tumours |
• infratentorial: increased ICP (obstruction of 4th ventricle), vomiting, morning headache, increased head circumference, VI nerve palsy, eyes "sunsetting", ataxia, cramal nerve palsies
• supratentoriai: focal deficits, seizures, long tract signs, visual field defects • evaluation • history, physical exam including complete neurological exam • CT and/or MRl of head |
|
|
Wilms'Tumour (Nephroblastoma)
|
• usually diagnosed between 2 and 5 years of age • most common primary renal neoplasm of childhood
• M=F • 5-10% of cases both kidneys are affected (simultaneously or in sequence) • differential diagnosis • hydronephrosis, polycystic kidney disease, renal cell carcinoma, neuroblastoma Clinical Presentation • 80% present with asymptomatic, unilateral abdominal mass • may also present with hypertension, gross hematuria, abdominal pain, vomiting • may have pulmonary metastases at time of primary diagnosis (respiratory symptoms) Associated Congenital Abnormalities • WAGR syndrome (Wilms' tumour, Aniridia, Genital anomalies, mental Retardation) with llp13 deletion • Beckwith-Wiedemann syndrome • characterized by enlargement of body organs, hemihypertrophy, renal medullary cysts, and adrenal cytomegaly • also at increased risk for developing hepatoblastoma, adrenocortical tumours, rhabdomyosarcomas, and pancreatic tumours • Denys-Drash syndrome • characterized by gonadal dysgenesis and nephropathy leading to renal failure Management • nephrectomy • staging, chemotherapy, radiation Prognosis • 90% long-term survival |
|
|
Neuroblastoma
|
Epidemiology
• most common cancer occurring in first year of life • neural crest cell tumour arising from sympathetic tissues (neuroblasts) • adrenal medulla (45%) • sympathetic chain (25% retroperitoneal, 20% posterior mediastinal, 4% pelvis, 4% neck) Clinical Presentation • can originate from any site in sympathetic nervous system, presenting as mass in neck, chest or abdominal mass (most common site is adrenal gland) • signs and symptoms of disease vary with location of tumour • thoracic: dyspnea, Homer's syndrome • abdomen: pa1pable mass • metastases are common at presentation (>50% present with advanced stage disease) • usually to bone or bone marrow (presents as bone pain, limp) • can also present with periorbital ecchymoses, abdominal pain, emesis, fever, weight loss, anorexia, bepatomegaly, "blueberry muffin" skin nodules • paraneoplastic: hypertension, palpitations, sweating (from excessive catecholamines), diarrhea, FIT (from VIP secretion), opsomyoclonus |
|
|
What are the good prognostic factors of neuroblastoma?
|
Investigations
• CBC, electrolytes, LFTs, renal function tests, LDH, Ca, Mg, serum ferritin • urine VMA, HVA levels • CT scan of chest, abdomen and pelvis, bone scan • bone marrow analysis - neuroblastoma cells in "rosettes" • tissue biopsy Good Prognostic Factors • "age and stage" are important determinants of better outcome • <1 year old • stage I, II, IV-5 disease • primary site: posterior mediastinum and neck • low serum ferritin • specific histology • tumour cell markers • aneuploidy • absent N-myc oncogene amplification Management • surgery, radiation, chemotherapy ± bone marrow transplantation • prognosis is often poor as it is found at an advanced stage |
|
|
BACTERIALTRACHEITIS
|
Etiology
• S. aureus is most common • H. influenzae, alpha-hemolytic strep, pneumococcus, Moraxella catarrhalis Clinical Presentation • initially similar to croup, but then rapid deterioration, with high fever • does not respond to croup treatments Investigations • clinical diagnosis • endoscopy: definitive diagnosis Management • start therapy for croup • usually requires intubation and antibiotics |
|
|
Differential Diagnosis of Wheezing
|
• common • asthma: recurrent wheezing episodes, identifiable triggers
• bronchiolitis: first episode of wheezing • recurrent aspiration: often neurological impairment • pneumonia: fever, cough, malaise • uncommon • foreign body: acute wheezing and coughing • cystic fibrosis: prolonged wheezing, unresponsive to therapy • bronchopulmonary dysplasia: often develops after prolonged ventilation in the newborn • rare • congestive heart failure • mediastinal mass • bronchiolitis obliterans • tracheobronchial anomalies |
|
|
When is diagnostic testing for bronchiolitis desirablee?
|
• CXR (only needed in severe disease, poor response to therapy, chronic episode) • air trapping, peribronchial thickening, atelectasis, increased linear markings
• nasopharyngeal swab • direct detection of viral antigen (immunofluorescence) • WBC usually normal |
|
|
Treatmnt for bronchiolitis
|
• mild distress • supportive: oral or IV hydration, antipyretics for fever • humidified ° (maintain 02 sat >92%)
inhaled bronchodilator (Ventolin™) 0.03 cc in 3 ml NS by mask, q20 min, and then ql hour - stop if no response • moderate to severe distress • as above - rarely, intubation and ventilation • ipratropium (Atrovent™) and steroids are not effective • consider rebetol (Ribavirin TM) in high risk groups: bronchopulmonary dysplasia, CHD, congenital lung disease, immunodeficient • monthly RSV-Ig or palivizumab (monoclonal antibody against the F-glycoprotein of RSV) may offer some protection against severe disease in high risk groups • case fatality rate <1 % • antibiotics have no therapeutic value unless there is secondary bacterial pneumonia • indications for hospitalization saturation <92% on initial presentation persistent resting tachypnea >60/minute and retractions after several • hypoxia: O 2 Salbutamol (Ventolin™) masks • past history of chronic lung disease, hemodynamically Significant cardiac disease, neuromuscular problem, immunocompromised • young infants <6 months old (unless extremely mild) • Significant feeding problems • social problem (e.g. inadequate care at home) |
|
|
CF presenting signs
|
Chronic cough and wheezing Failure to thrive Pancreatic insufficiency (symptoms of malabsorption like steatorrhea) Alkalosis and hypotonic dehydration
Neonatal intestinal obstruction (meconium i1eusl! Nasal polyps Clubbing of fingers! Chest radiograph with characteristic changes Rectal prolapse Electrolyte elevation in swea~ salty skin Absence or congenital atresia of vas deferens Sputum with Staph or Pseudomonas (mucoidl |
|
|
Clinical presentation of CF by age
|
• neonatal • meconium ileus
• prolonged jaundice • antenatal bowel perforation • infancy • pancreatic insufficiency with steatorrhea and FIT (despite voracious appetite) • childhood • anemia, hypoproteinemia, hyponatremia heat intolerance • wheezing or chronic cough recurrent chest infections (5. aureus, P. aeruginosa, H. influenzae) hemoptysis • nasal polyps (associated with milder disease) distal mtestinal obstruction syndrome, rectal prolapse • clubbing of fingers • older patients • chronic obstructiVE' pulmonary disease (COPD) • infertility |
|
|
Pathophys of CF and investigations
|
• mutation in transmembrane conductance regulator of chloride - causes cells to be impermeable to Cl which increases the reabsorption of Na
• leads to relative dehydration of airway secretions, resulting in impaired mucociliary transport and airway ohstruction • sweat chloride test x 2 (>60 mEq/L) • false positive tests: malnutrition, celiac disease, adrenal insufficiency, anorexia nervosa, hypothyroidism, nephrogenic diabetes insipidus, nephrotic syndrome • false negatiw tests: peripheral edema, cloxacillin, glycogen storage disease, hypoparathyroidism, atopic dermatitis, Klinefelter syndrome, hypogammaglobulinemia • pancreatic dysfunction - determined by 3-day fecal fat collection • genetics - useful where sweat chloride test is equivocal |
|
|
How to manage CF?
|
• nutritional counselling • high calorie diet
• pancreatic enzyme replacements • fat soluble vitamin supplements • management of chest disease • physiotherapy, poshlral drainage • exercise . • bronchodilators • aerosolized DNAase • antibiotics: depends on spuhlm C&S (e.g. cephalosporin, cloxacillin, ciprofloxacin, inhaled tODramycin) • lung transplantation • genetic counselling |
|
|
Complications of CF?
|
• respiratory failure • pneumothorax (poor prognostic sign)
• cor pulmonale (rate) • pancreatic fibrosis with diabetes mellitus • gallstones • cirrhosis with portal hypertension • infertility • early death (current median survival is 30 years) |
|
|
Evaluation of Limb Pain
History |
• pain: onset, duration, location, character, intensity, frequency, aggravating/alleviating factors, limitations in daily activity
• trauma, injury • morning stiffness, limp, swelling/redness/warmth of joints • general: fever, rash, fatigue, weight loss, cough, chest pain, back pain, hair loss, medications, voiding or stooling problems, iritis • farrtily history: arthritis, psoriasis, IBO, bleeding disorders, sickle cell anemia |
|
|
Evaluation of Limb Pain
Physical Exam Investigations |
Physical Exam
• complete physical exam; always examine joint above and below joint involved • all joints: inspection, palpation, range of motion • gait, leg length discrepancy • tenderness over tendons or tendon insertion sites, swelling or nodularity • muscle weakness or atrophy • entheses- swelling, tenderness Investigations • CBC, differential, blood smear, ESR • x-rays of painful joints/limbs • as indicated: blood C& S, ANA, RF, PIT, sickle cell prep, viral serology, immunoglobulins, complement, urinalysis, synovial analysis and culture, TB test, ASOT, slit lamp |
|
|
Physical Exam
• complete physical exam; always examine joint above and below joint involved • all joints: inspection, palpation, range of motion • gait, leg length discrepancy • tenderness over tendons or tendon insertion sites, swelling or nodularity • muscle weakness or atrophy • entheses- swelling, tenderness Investigations • CBC, differential, blood smear, ESR • x-rays of painful joints/limbs • as indicated: blood C& S, ANA, RF, PIT, sickle cell prep, viral serology, immunoglobulins, complement, urinalysis, synovial analysis and culture, TB test, ASOT, slit lamp |
• medical emergency • hematogenous osteomyelitic spread seen most commonly in neonates and infants
• clinical presentation: acute monoarthritis with erythema, warmth, swelling, intense pain on passive movement (pain may be so severe that it causes pseudoparalysis of involved limb), fever & chills • definitive test: joint aspirate and culture • management: proper antibiotic selection requires knowledge of likely bacterial pathogen at various age |
|
|
Growing Pains
|
• age 2-12 years, M=F • diagnosis
• poorly localized pain in the legs • usually bilateral • occurs in evening or awakens child at night • responds to reassurance, massage or analgesics • resolves completely in the morning • no associated systemic symptoms (e.g. fever) • possible family history of growing pains • normal physical examination • lab investigations not necessary if typical presentation |
|
|
Transient Synovitis
|
• age 3-10 years, M>F • benign, self limited disorder, usually occurs after upper respiratory tract infection,
pharyngitis, bronchitis, otitis media • clinical presentation - afebrile or low-grade fever, pain typically occurs in hips, knees, painful limp but still capable of ambulating • symptoms resolve over 7-10 days • lab investigations (ESR, WBC) within normal limits • x-ray is typically normal • U/S may show joint effusion • must exclude septic arthritis, osteomyelitis, AVN, SCFE • treatment: symptomatic and anti-inflammatory medications |
|
|
Juvenile Idiopathic Arthritis (JIA)
|
a heterogenous group of conditions characterized by persistent arthritis in children under 16 years
• diagnosis: arthritis in >/= I joint lasting >6 weeks in child <16 years with exclusion of other causes of arthritis • classification defined by features/number of joints affected in the first 6 months of onset: • systemic: arthritis, fewr and other systemic features • oligoarticular: S4 joints (most common) • polyarticular:::::5 joints • enthesitis-related arthritis • psoriatic arthritis • unclassified |
|
|
Systemic Arthritis (Still's disease)
|
• high spiking fever (38.5
C) for at least 2 weeks • extra-articular features: erythematous "salmon-coloured" maculopapular rash, u lymphadenopathy, hepatosplenomegaly, leukocytosis, thrombocytosis, anemia, serositis (pericarditis, pleuritis) • onset at any age, M=F • arthritis may occur weeks to months later • high ESR, CRP, WBC, platelet count |
|
|
Oligoarticular Arthritis
|
Arthritis (arthritis of 14 joints in the first 6 months)
• persistent - affects no more than 4 joints during the disease course • extended - affects more than 4 joints after the first six months • onset 1-3 years of age, F > M • typically affects large joints knees> ankles, elbows, wrists, hip involvement unusual • ANA positive -80%, rheumatoid factor (RF) negative • screening eye exams for asymptomatic anterior uveitis (occurs in -20%) • complications: knee flexion contracture, quadriceps atrophy, leg-length discrepancy, growth disturbances |
|
|
Polyarticular Arthritis
|
Arthritis (arthritis of 5 or more joints)
• RF negative • often involves large and small joints of hands and feet, temporomandibular joint, cervical spine • patients, especially those who are ANA positive, are prone to chronic uveitis • RF positive • similar to the aggressive form of adult rheumatoid arthritis • severe, rapidly destructive, symmetrical arthritis of large and small joints • may have rheumatoid nodules at pressure points (elbows, knees) • unremitting disease, persists into adulthood |
|
|
Enthesitis-Related Arthritis
|
• arthritis or enthesitis or both with at least two of: • sacroiliac tenderness and/or inflammatory spinal pain • HLA 827 positive
degree relative • family history of confirmed HLA 827 -associated disease in a 1 • symptomatic (acute) anterior uveitis • onset of arthritis in a boy >8 years st or 2 nd |
|
|
Management of JIA
|
children may complain very little about their pain and disability • exercise to maintain range of motion (ROM) and muscle strength • multidisciplinary approach with OT/PT, social work, orthopaedics, ophthalmology,
rheumatology • first line drug therapy: NSAIDs, intra-articular corticosteroids • 2nd line: • DMARDs - methotrexate, sulfasalazine st • other corticosteroids - intra-articular, systemic for systemic onset of JIA, topical eye drops for uveitis • biologic agents - etanercept (EnbreJTM): binds TNF and blocks its interaction with cell surface receptors |
|
|
Associated Features
Kawasaki |
• acute phase (as long as fever persists, about 10 days) • most of diagnostic criteria present
• irritability, aseptic meningitis, myocarditis, pericarditis, CHF • diarrhea, gallbladder hydrops, pancreatitis, urethritis, arthritis • subacute phase (resolution of fever, peeling of skin, elevated ESR and platelets, usually days 11-21) • arthritis • convalescent phase (lasts until ESR and platelets normalize, >21 days) • coronary artery aneurysms, aneurysm rupture, myocardial infarction (MI), CHF |
|
|
Management of kawasaki
|
• high (anti-inflammatory) dose of ASA while febrile • low (anti-platelet) dose of ASA in subacute phase until platelets normalize or longer if
coronary artery involvement • IV immunoglobulin (2 g/kg) reduces risk of coronary aneurysm formation • baseline 2D-echo and follow up periodic 2D-echocardiograms (usually at 6 weeks) |
|
|
Diagnostic criteria of Kawasaki
|
• fever persisting 5 days or more AND • 4 of the following features:
1. bilateral nonpurulent conjunctivitis 2. red fissured lips, strawberry tongue, erythema of oropharynx 3. changes of the peripheral extremities • acute phase: erythema, edema of hands and feet • subacute phase: peeling from tips of fingers and toes 4. polymorphous rash 5. cervical lymphadenopathy >1.5 em in diameter • exclusion of other diseases (e.g. scarlet fever, measles) • atypical Kawasaki disease: less than 5 of 6 diagnostic features but coronary artery involvement "Warm CREAM" Fever ~5 days Conjunctivitis Rash EdemalErythema (hands and feetl Adenopathy Mucosal involvement |
|
|
Complications
Kawasaki |
• coronary artery vasculitis with aneurysm formation occurs in 20-25% of untreated children, <5% if receive MG within 10 days of fever
• 50% of aneurysms regress within 2 years • anticoagulation for multiple or large coronary aneurysms • risk factors for coronary disease: male, age <lor >9 years, fever >10 days |
|
|
At what age should aphysician be concerned
if a child is not walking or talking? |
18 months
|
|
|
Name the adverse reactions to al PentacellDPTPHibl, b) MMR
|
a) 24-48 hr fever, local erythema/swelling, seizure (rare), hypotonic hyporesponsive episode (rare)
b) 7-14 dfever, measles-like rash, lymphadenopathy (rarel. arthritis !rare), parotitis (rare), |
|
|
How do you differentiate irritant diaper dermatoses from candidiasis on exam?
|
Candida involves the creases and has satellite lesions
|
|
|
What is the mean presenting age for leukemia?
|
2-5 years
|
|
|
What is the most common cancer in children <lyear?
|
Neuroblastoma
|
|
|
What further investigations are appropriate for a 6month old presenting with first febrile
UTI? |
U/S, VCUG; if abnormal, DMSA
|
