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Pediatrics Blueprints

Pediatrics Blueprints

by jblustin9, Mar. 2015

Subjects: Pediatrics, Blueprints

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327 Cards in this Set

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	What are the two developmental screens most commonly used by pediatricians?	Denver II (0-6 years of age)- divides stream of development into gross motor, fine motor-adaptive, language, and personal-social Clinical Adaptive Test/Clinical Linguistic and Auditory Milestone Scale (CAT/CLAMS)- between birth and 36 months- rates problem-solving/visual motor ability and language development
	Developmental Delay	performance in a certain developmental area lags significantly compared with average attainment in a given skill area
	Developmental Quotient	development age/chronological age x 100 -- this gives an idea of the child's developmental status
	Developmental dissociation	When there is a substantial difference in the rates of development between two skill areas
	What is the best indicator of future intellectual potential?	language- both receptive and expressive
	What is the most common developmental delay in preschool children?	language delay
	Mental Retardation	as defined by the DSM IV-TR 1) IQ 2) onset prior to age 18 3) impaired adaptive functioning (in at least two of the following areas: communication, self-care, home living, social/interpersonal skills, work, leisure, health, and safety) * the cause of MR is only identified in about 1/2 of cases
	What are the most commonly used IQ tests in the Pediatric population?	1. Wechsler - preschool and school age 2. Stanford-Binet - school age
	Mild, Moderate, Severe and Profound Mental Retardation by IQ	Mild - 50-70 Moderate- 35-55 Severe - 20-40 Profound- below 20-25
	How common in language delay in preschoolers?	up to 15% of preschoolers have some sort of speech/language delay at one time or another. In most cases there is underlying biologic abnormality such as genetic syndrome etc
	Language disorders	inability to understand or acquire vocabulary, grammatical rules, or conversation patterns of language (expressive and receptive disorder)
	Speech disorders	difficulty producing sounds and rhythms of speech (expressive disorder)
	Phoentic disorders	problems with articulation (expressive disorder)
	Dysfluency	interruptions in the flow of speech - this is observed in many preschoolers and usually resolves by age 4 - true dysfluency = stuttering and is characterized by signs of tension and struggle when speaking, sound repetition, or complete speech blockage and significant impedes the ability to communicate
	What should any child with a suspected language delay receive?	a full audiologic (hearing) assessment followed be referral to a speech pathologist * early and intensive speech therapy often results in significant and sustained improvement in communication skills over time
	What is the most common cause of mild-to-moderate hearing loss in young children	otitis media with effusion
	Attention-deficity/hyperactivity disorder (ADHD)	- syndrome characterized by inattention, hyperactivity and impulsivity, which are inconsistent with the developmental stage of the child and manifested through maladaptive behaviors - the classic form is more common in boys and is usually diagnosed during elementary school - school performance and peer relationships often suffer, placing the child at risk for low self-esteem - a form that is solely characterized by inattentiveness is more common in girls, and is often diagnosed later - symptoms persist into adulthood in the majority of patients
	Diagnosis of ADHD	This is a clinical diagnosis and the inattention, hyperactivity and impulsivity must be present by age 7, persist for at least 6 months, and be observed in multiple environments - initial assessment relies firmly on history obtained from parents and teachers - age appropriate rating scales are available and standardized (Conner's Parent and Teacher Rating Scale)
	Management of ADHD	a multidisciplinary approach is required, including emotional support and development of a behavioral management plan for positive and negative reinforcement evaluate for learning disorder as this is concomittant in up to 25% of patients Psychostimulants such as methylphenidate, dextroamphetamine, mixed amphetamine salts should be used not only during school hours, but also on weekends and during vacations to encourage good function in all environments
	Autistic spectrum disorder - definition - prevalence - gender distribution - age of typical diagnosis	A collection of chronic, nonprogressive disabilities characterized by impairments in social interaction, communication, and behavior. Both autism and asperger syndrome fall under this umbrella reported prevalence has been rising for the past 20 years more common in males usually diagnosed between the ages of 18 months and 3 years of age long-term studies have NOT found any association between the MMR vaccine or thimerosal and the development of autism
	Autism - characteristics - what differentiates this condition from Asperger's syndrome	Children with classic autism have significant language and communication abnormalities, which helps differentiate this condition from Asperger's in which language difficulties are not present children with this condition: avoid eye contact, exhibit little or no reciprocal communication, and do not engage in pretend play patients usually display stereotypic and/or repetitive behavior patterns (e.g. rocking or spinning) and may have attachment to or fascination with unusual objects
	Asperger's Syndrome - characteristics	difficulty forming relationships/relating to others and development of intense interest in very specific topics (e.g. dinosaurs, mummies, speakers, etc) generally do not have language impairment, however patients often do not understand abstract forms of language such as metaphors and sarcasm unable to pick up on nonverbal cues, which it makes it difficult for affected individuals to form friendships even though they usually want to
	Management of Autistic Spectrum Disorder	combination of intensive behavioral and sensory integration therapy, speech and language training, social modeling, family support, and pharmacotherapy for anxiety, hyperactivity and perseverative behaviors early intervention is important so all children should be screened before age 2
	What is the best prognostic indicator for patients with Autistic Spectrum Disorder?	The extent of language development during the preschool years
	Hand-foot-and mouth disease	common acute disease of young children during the spring and summer caused by Coxsakie A viruses there is usually a prodrome of fever, anorexia, and oral pain followed by crops of ulcers on the tongue and oral mucosa, as well as a vesicular rash on the hands, feet, and occassionally buttocks the individual vesicles often have a "football" shape and are surrounded by erythema treatment is supportive
	Giannoti-Crosti Syndrome (aka papular acrodermatitis of childhood)	asymptomatic erythematous papular eruption occurring commonly from one to six years of age following an URI the rash is symmetrically distributed on the face, extensor surfaces of the arms, legs, and buttocks and SPARES THE TRUNK papules may coalesce into larger areas of edematous plaques or become purpuric associated with several viruses including Hep B and EBV tx: supportive- rash usually resolves within 8 weeks
	Varicella	highly contagious disease caused by primary infection with varicella zoster virus can be fatal in immunocompromised kids incubation period = 10-21 days prodrome of fever, malaise, anorexia, and occasionally scarlatinaform or morbilliform rash followed by the characteristic pruritic rash that occurs the next day. characteristic rash consists of red papules that evolve into clear vesicles that are 1-2 mm in diameter ("dew drop on a rose petal"). These vesicles then become cloudy, rupture and form crusts. The lesions begin on the trunk and then spread peripherally. often occur in "crops" across the body patients are infectious from 24 hours BEFORE the rash to until the last lesion crusts over, which is usually one week after rash onset
	Herpes Zoster/Shingles - characteristics	Reactivation of latent VZV infection that is latent in the dorsal root ganglion of the nerves. primarily occurs in adults, however can occur in children who experienced an initial infection very early in life attack begins with pain and/or pruritus in a dermatomal dermatomal distribution accompanied by fever and malaise. This is followed by a rash in this same distribution- which consists of crops of vesicles clears within 7-14 days although the rash can last as long as 4 weeks. The pain may persist for weeks to months
	Management of herpes Zoster/shingles	Pain management - narcotics are sometimes needed For immunocompromised patients, patients over 12 yo, children with chronic diseases, and those who have received system steroids then systemic acyclovir should be considered
	Molluscum contagiosum	cutaneous viral infection caused by poxvirus very common in childhood small flesh-colored, pearly, dome shaped papules with central umbilication that usually occurs in moist areas such as the axillae, buttocks, and groin region the papules spread via touching, auto-inoculation, and scratching often seen in wrestlers and sauna users
	Treatment of Molluscum Contagiosum	Lesions usually resolve spontaneously over 2 years, however most patients do not want to wait that long Treatment depends on the experience level of the practioner Options include: curettage, cryotherapy, cantharidin (an extract from the blister beetle that causing blistering of the epidermis), oral cimetidine, and imiquimod cream
	Verrucae- characteristics and types	"warts" Caused by HPV typically benign in otherwise healthy individuals spread by skin-to-skin contact or fomites may develop at the site of trauma four common types: 1) verruca vulgaris (common wart), verruca plantaris (plantar wart), verruca plana (flat wart), and condyloma accuminata (genital warts)
	Management of warts/verruca	Treatment depends on the type of wart and location Options include: topical salicylic acid, liquid nitrogen, imiquimod cream, oral cimetidine, intralesional Candida antigen, and squaric acid dibutylester Patients may require multiple treatments
	Pityriasis Rosea	exanthem of unknown etiology often follows a viral prodrome, such as a URI rash has a distinct morphology that begins with a "herald patch" - a 2-10 cm oval salmon-pink plaque on the trunk, neck, upper extremities or thigh. This is followed by several smaller lesions distributed in a christmas tree pattern over the trunk and upper extremities that develops over days to weeks some patients, especially young children and African Americans develop a rash with a more papular appearance rash fades over 4-12 weeks and is typically asymptomatic but may be pruritic usually self-limited and no treatment is needed, although topical antihistamines as well as a mild topical steroid can be used for pruritus sunlight hastens the resolution
		Pityriasis Rosea- herald patch
	unilateral thoracic exanthem	asymmetric periflexural exanthem of childhood rash with varying morphologies that occurs in children ages 1-5 years and begins with an exanthem on one side of the trunk that spreads centripetally rash is seen more commonly in winter and spring months and may follow symptoms or low-grade fever, lymphadenopathy, respiratory or GI complaints often confused with contact dermatitis lesions vary from erythematous macules or papules with surrounding halo to morbilliform, eczematous, scarlatinaform or reticulate configurations that may spread to the opposite side from initial involvement pruritus is common and can be treated with oral antihistamines or mild topical steroids lesions resolve over 6-8 weeks without any treatment and may desquamate or leave post-inflammatory pigment changes
	What are the most common cause of bacterial infections of the skin in children?	group A beta-hemolytic streptococus or S. aureus
	Bullous impetigo	caused by a toxin-producing strain of S. aureus begins as red macules that progress to bullous (fluid-filled) eruptions on an erythematous base lesions range from a few millimeters to a few centimeters in diameter after the bullae rupture, a clear, think varnish-like coating forms over the denuded area these lesions may be mistaken for cigarette burns, making child abuse considered
	Nonbullous impetigo- causes and characteristics	caused by both beta-hemolytic streptocccus and s. aureus begins as papules that progress to vesciles and then to pustules measuring approximately 5 mm in diameter with a thin erythemaous rim the papules rupture, leaving a honey-colored crust thin exudate that then forms a crust over a shallow ulcerated base local lymphadenopathy is common with streptococcal impetigo, however fever is uncommon
	Treatment of non-bullous impetigo	can be treated with topical antibiotics such as mupirocin If there are many bullous or nonbullous lesions than oral antibiotics such as first generation cephalosporin (cephalexin) can be used- effective against staph and strep If MRSA is suspected then clindamycin or trimethoprim sulfamethoxazole (Bactrim) should be used the honey-colored crusts can be removed twice daily with cool compresses
	Staphylococcal Scalded Skin Syndrome	caused by exfoliative toxin-producing isolates of S. aureus most common in infancy and rarely occurs beyond age 5 onset is abrupt, with diffuse erythema, marked skin tenderness, irritability and fever within 12-24 hours of onset superficial flaccid bullae develop and then rupture almost immediately, leaving beefy red, weeping surface although widespread areas ma be affected there is often more on the perioral areas and neck, axillae and inguinal creases positive Nikolsky sign unruptured bullae contain sterile fluid
	Treatment of staphyloccal scalded skin syndrome	Mild to moderate cases are treated with an oral antistaphylococcal medication severe cases should be treated as second degree burns, with fluid management and IV oxacillin or clindamycin
	Folliculitis	infection of the shaft of the hair follicle usually caused by s. aureus the buttocks and lower legs in girls who shave are common sites of infection deep forms of this infection include furuncles (boils) and carbuncles
	furuncles and carbuncles	begin as superficial folliculitis and are most frequently found in areas of hair-bearing skin that are subject to friction and maceration, especially the scalp, buttocks, and axillae carbuncles are larger accumulations of furuncles
	Treatment of superficial folliculitis	Aggressive hygeine with antiseptic and topical mupirocin folliculitis of the male beard is usually recalcitrant and requires oral anti-staph drugs
	Treatment of furuncles and carbuncles	simple furuncles are treated with moist heat larger and deeper furuncles may to be I&D'ed
	What is the cause of "hot tub folliculitis" and how is it managed?	Pseudomonas aeruginosa - These lesions are self-limited so long as exposure is discontinued
	What are the two most common superficial fungal infections?	Tricophyton and Microsporum
	What is the most common cause of tinea capitis in the US? How does this manifest? Treatment?	Tricophyton tonsurans (although microsporum canis can be spread from animals) this manifests as patches of scaling and hair loss, broken off hairs known as "black dots" and boggy, pustular masses known as kerions the kerions may be itchy and painful or may be associated with morbilliform eruption and LAD Treatment: Oral griseofulvin for 4-6 weeks. Selenium sulfide shampoo kills the spores but does not eradicate the infection
	Tinea corporis- presentation and treatment	$"ring worm"annular plaques with peripheral scaling, giving the appearance of ringsTreatment: topical antifungals (clotrimazole) for at least 4 weeks, oral griseofulvin if refractory$ "ring worm" annular plaques with peripheral scaling, giving the appearance of rings Treatment: topical antifungals (clotrimazole) for at least 4 weeks, oral griseofulvin if refractory
	Tinea pedis- cause, presentation, and treatment	usually an infection with Microsporum rubrum classically presents as scaling in the "moccassin" distribution and frequently also involves the interdigital spaces of the toes Treatment: topical antifungals (clotrimazole) for at least 4 weeks, oral griseofulvin if refractory. Plus good foot hygeine
	Tinea cruris- cause, presentation, and treatment	"jock itch" caused by Microsporum rubrum (which also causes tinea pedis) presents with erythema, scaling, and maceration in the inguinal creases
	When are systemic antifungal drugs needed for superificial fungal infections?	When these infections affect the hair or nails
	Tinea (pityriasis) versicolor- characteristics and treatment	caused by infection with a yeast, known as malassezia furfur characterized by superficial tan or hypopigmented oval scaly patches on the neck, upper part of the back, chest and uppers arms dark-skinned individuals tend to have hypopigmented lesions during the sumer when uninfected skin tans from sunlight exposure, however patients may have both dark and light colored lesions at the same time, which is why the condition is called "versicolor" Treatment: selenium sulfide shampoo and other topical or systemic antifungals. Recurrence in the summer-time is common
	Diaper rash - causes, characteristics and treatment	can be caused by atopic dermatitis, primary irritant dermatitis, or primary or secondary Candida albicans infection 80% of diaper rashes lasting longer than 4 days are colonized with candida appears as fiery red papular lesions with peripheral papules, pustules, and scales in the skin folds and satellite lesions Treatment: barrier creams along with topical antifungal or nystatin creams - first-line
	Acne vulgaris	very common, self-limited, multifactorial disorder of the sebaceous follicles noted mostly during the teenage years lesions may begin as early as 8-10 yo prevalence increases steadily throughout adolescence and then decreases in adulthood girls often develop acne at younger ages, however severe disease is ten times more common in boys because of higher androgen levels 15% of all teenage boys have severe acne
	Pathogenesis of acne vulgaris	adrogen stimulation of the sebaceous glands follicular plugging proliferation of Propionibaterium acnes inflammatory changes (closed comedones- white heads and open comedones- black heads are non-inflammatory and non-scarring, however, papules, pustules and nodules/cysts are inflammatory and can cause hypertrophic or pitted scarring
	Risk factors for acne vulgaris	family history puberty PCOS in girls rarely cushing disease another condition that leads to excess androgens some medications can cause or exacerbate acne poor hygiene and food intake are NOT risk factors
	Treatment of acne vulgaris	Treatment should be individualized based on gender, severity, type and distribution of the lesions Mild acne- generally responds to topical therapy without any scarring. Benzoyl peroxide works by decreasing the colonization of P. acnes Topical retinoids (tretinoin, adapalene, and tazarotene) have strong anticomedogenic activity but side effects including dryness, burning and photosensitivity limit their use - need to use SPF > 15 topical antibiotic such as clindamycin and erythromycin are used to prevent and decrease skin colonization of p. acnes, however when used alone this promotes antibacterial resistance so they should be used with benzoyl peroxide oral antibiotics should be reserved for severely affected patients who do not respond to topical therapy (tetracycline, doxycycline, minocycline, and erythromycin) for females, OCPs may be helpful in suppressing androgen production and are now approved for acne therapy Oral isotretinoin is very effective for acne, but it is a teratogen and has a high SE profile so strict monitoring is required-- usually only prescribed by a dermatologist
	Psoriasis - characteristics, epidemiology	common but often diagnosed childhood disease, with 10% of cases beginning before age 10 and 35% beginning before age 20 Often there is a family history and HLA inheritance is part of the mode of transmission non-pruritic, papulosquamous eruption consisting of erythematous papules that coalesce to form dry plaques with sharply demarcated borders and silvery scales these scales tend to build up into layers and their removal may cause pinpoint bleeding known as the Auspitz sign often appears at the sites of physical, thermal or mechanical trauma = Koebner phenomenon usually symmetric with plaques appearing on the knees, elbows, and childhood, the scalp, periocular and genital areas the nails often demonstrate punctate stipling or pitting and there can be dettachment of the distal nail plate (onycholysis) and accumulation of subungual debris the palms and soles often have scaling and fissuring there are remissions and exacerbations
	eczema vs psoriasis	Eczema generally affects flexural creases and is pruritic, while psoriasis generally affects extensor surfaces and is non-pruritic
	What bacterial commonly causes an exacerbation of psoriasis?	Group A beta hemolytic streptococcus (S. pyogenes)
	Management of psoriasis	Most importantly- educate patient and family about the chronic nature of this disease it cannot be cured but it can be controlled no matter where the rash is-- the goal of therapy is to keep the skin well-hydrated topical steroids are a mainstay of treatment, however the least potent effective dose should be used in order to prevent adrenal suppression topical vitamin D cream and tar can also help for severe cases, natural sunlight or UVB light is helpful in severe cases methotrexate and immunosuppressant such as entanercept can be used under the supervision of a dermatologist
	Erythema multiforme	acute, self-limited, hypersensitivity reaction that is uncommon in children the most frequent etiologic agents = viral infection such as herpesvirus, adenovirus, and EBV characteristics: symmetric distribution of lesions that evolves through multiple morphologic stages: erythematous macules, papules, plaques, vesicles and target lesions the lesions evolve over days, not hours and often have target-like appearance it is often confused with polycyclic urticaria which can also appear targetoid, however urticarial rashes are not fixed and do not have necrotic centers. These urticarial lesions tend to have edematous, erythematous borders with central clearing and the individual lesions resolve in 12-24 hours EM is typically fixed and develops dusky, necrotic centers. It also tends to occur over the dorsum of the hands and feets, palms, soles, extensor surfaces of extremities and may spread to the trunk burning and itching are common fever, malaise any myalgias may also be present
	What is the most common cause of Erythema Multiforme in children??	Herpes Virus type I
	Stevens-Johnson Syndrome (SJS)	a distinct hypersensitivity reaction that is a variant of toxic epidermal necrolysis (TEN) there is prodrome of fever, malaise, yalgias, arthralgias, arthritis, headache, emesis and diarrhea that lasts for 1-14 days this is followed by the sudden onset of a high fever, erythematous and purpuric macules with dusky centers, and inflammator bullae of two or more mucous membranes (oral mucosa, lips, bulbar conjunctiva, and anogenital area) In the most severe cases there is involvement of the GI, respiratory and GU tracts If left untreated, there is a 10% mortality rate The most common cause = includes drugs (NSAIDS, penicillins sulfonamides, and many anti-epileptics), myoplasma infections, and rarely immunizations
	Toxic Epideral Necrolysis (TEN)	the most severe form of cutaneous hypersensitivity - likely a more severe variant of SJS rare in children, but the mortality rate is about 30% etiology is likely related to upregulated Fas ligand, which is a mediator of apoptosis onset is acute, with high fever, burning sensation of the skin and mucous membranes and/or oral oral and conjuctival erythema and erosions the presentation of the skin resembles staphylococcal scalded skin syndrome with widespread erythemaa, tenderness, blister formation, and full dettachment of the epidermis causing denudation (positive Nikolsky sign) Mucous membrane involvement is severe and the nails may shed systemic complication include elevate liver enzymes, renal failure and fluid and electrolyte imbalance sepsis and shock are frequent causes of death
	Treatment of Erythema Multiforme (EM)	if uncomplicated- symptomatic treatment and reassurance. Oral antihistamines, moist compresses and oatmeal baths are helpful the lesions resolve over a 1-3 week period, with some hyperpigmentation the use of corticosteroids is controversial
	treatment of SJS	HOSPITALIZATION with barrier isolation fluid and electrolyte support treatment of secondary infections of the skin, moist compresses on bullae, colloidal baths for oral mucosal lesions, mouthwashes with vicous lidocaine, diphenhydramine, and Maaloz are comforting because corneal uleration, keratitis, uveitis and panophthalmitis are possible and ophthalmology consult is recommended
	Treatment of TEN	treat as though there is a full body second degree burn fluid therapy and reverse barrier isolation are critical for survival many patent require treatment in an ICU or burn center IVIG has been used with some success- most likely due to binding or modulating fas ligand
	Allergic reactions to drugs	These typically present as urticarial or morbilliform exanthems which can develop within 1-2 weeks of starting a new medication The risk of allergic reaction may be increased if the patient has a concurrent viral infection similar to patients with mononucleosis treated with ampicillin The eruption may clear afterthe inciting agent is removed, but it may take several days to weeks Following the acute eruption, many patients tend to desquamate Treatment is based on the symptoms Decision to discontinue the inciting medication depends on the risks vs benefits (risk from the primary illness and if alternative treatments are available)
	Risk factors for melanoma and non-melanoma skin cancer in children	fair skin excessive sun exposure multiple nevi
	Congenital nevi vs acquired nevi	Congenital nevi tend to be larger and can vary in color and shape. They tend to get darker, thicker, and hairier with time although giant nevi will often become lighter congenital nevi are classified by their size (Large/giant = >20 cm2, small is < 2 cm2 with intermediate between these). The larger nevi have increased malignant potential follow these annually as changes may require excision The increased lifetime risk of melanoma in giant nevi is between 5-15% There is also an association with neurocutaneous melanosis-- if lesions on head or spine then do an MRI
	Acquired nevi	These often develop in infancy and childhood and reach there maximum number in early adulthood patients with more than 15 common acquired moles may be at increased risk for melanoma Look for ABCDE- asymmetry, borders, color, diameter > 6mm, evolution
	Spitz nevus	smooth pink to brown to jet black dome-shaped papule that usually enlarges rapidly after it appears these are generally benign, although they may need to be excised if there is rapid growth because rarely malignant forms do occur
	Halo nevus	moles which develop up to 1 cm of depigmentation surrounding them the depigmentation is an autoimmune reaction again the pigment cells these nevi may completely regress leave a white macule that eventually fills in the lesions themselves are generally benign in children but may be associated with the presence of melanoma or vitiligo at other sites
	Prevention of skin cancers in children	sun protection with a sunblock of SPF 15 or more that protects against UVA AND UVB re-application of sunblock is essential avoiding prolong sun exposure in mid-day ad sun-protective clothing are equally important
	Infantile hemangiomas	vascular tumors which are common in infancy occur in 1-2% of neonates more common in females, caucasians, and premature infants classified as superficial, deep, or mixed generally not present at birth can be found in any location, but are most common on the head or neck generally have a predictable evolution- appear during the first month of life and maintain growth for several months to one year, then begin to slowly involute. Most lesions resolve by age 10, although there may be residual textural changes in the skin or superficial telangiectasias *Image shows superficial infantile hemangioma
	Superficial vs deep hemangiomas	Superficial hemagiomas are bright red and non-compressible, whereas deep hemangiomas are subcutaneous, compressible and often have a bluish hue and superficial telangiectasias * Image shows deep infantile hemangioma
	Management of infantile hemagiomas	rule out other conditions such as more aggressive vascular tumors and vascular malformations provide anticipatory guidance and observe changes in the lesion intervention is required for life-threatening or functional risks. Large lesions may cause heart failure, lesions in the airway may cause obstruction treatment of periocular lesions is mandatory so as to prevent astigmatism and/or blindness multiple lesions (>5) may be associated with live and other internal hemangiomas local or systemic corticosteroids, antibiotics and dressings and excision may be required depending on the situation vincristine and alpha interferon can be considered for severe cases
	Type I Diabetes Mellitus - definition, symptoms, risk factors, epidemiology	chronic metabolic disorder characterized by autoimmune destruction of the insulin-producing beta islet cells of the pancreas this leads to hyperglycemia and abnormal energy metabolism symptoms include polydipisia, polyphagia, polyuria, weight loss, vision changes, and possible DKA peak age of onset is 10-12 yo equal in males and females main risk factor is positive family history (50% concordance rate among identical twins) 85% of newly diagnosed patients have anti-islet cell antibodies suggesting an autoimmune etiology, however it is not clear what triggers this reaction no particular virus has been determined to be directly responsible
	At what blood glucose level does glucose start appearing in the urine and leading to osmotic diuresis?	180 mg/dL
	What is the most common endocrine disease in childhood and what is the prevalence of this disease?	Type I Diabetes Mellitus - 0.25-0.5% of the population (1 in 400 children)
	If a child is suspected of being in DKA, what questions should be asked during the history?	usual insulin dose last insulin dose child's diet over the previous day whether the child has been ill or emotionally or physically stressed lately
	Presentation of a child with DKA	moderate to profound dehydration polyuria polydipsia fatigue headache nausea emesis abdominal pain AMS: confused to comatose On physical exam: tachycardia, hyperpnea (Kussmaul's respirations), +/- fruity odor to the breath from the ketones, +/- hypotension, AMS
	Symptoms of hypoglycemia	trembling diaphoresis flushing tachycardia sleepiness confusion mood changes seizures coma
	Causes of secondary diabetes	Secondary diabetes can be caused by insulin antagonism from excess glucocorticoids (Cushing syndrome or iatrogenic), hyperthyroidism, pheochromocytoma, GH excess, or medications such as thiazide diuretics
	Cystic Fibrosis Related Diabetes (CFRD)	beta cell destruction from auto-digestion of the pancreas and inflammation
	Maturity-onset diabetes of the young (MODY)	clinically heterogeneous group of disorders characterized by nonketotic diabetes inherited in an autosomal dominant fashion with onset before age 25 defect in one of the six genes involved in insulin secretion
	Diagnosis of Type I Diabetes Mellitus	glucosuria, ketonuria and random plasma glucose > 200 mg/dL fasting blood glucose > 126 mg/dL 2 hour post-prandial glucose > 200 mg/dL anti-islet cell antibodies in 85% high Hgb A1c if DM present for some time
	Blood chemistry in DKA	primary metabolic acidosis with respiratory compensation - decreased HCO3 and decreased PCO2 Low pH BUN increased decreased phosphate, potassium, calcium
	Potassium in DKA	Serum potassium may appear normal or high due to intracellular shifting of potassium out of the cells, however total body potassium is always low and needs to be repleted
	Treatment of new-onset DM	reversal of the catabolic state through exogenous insulin administration and reversal of electrolyte abnormalities diet exercise psychological support regular medical follow-up carbohydrate counting
	What is the starting insulin dose in a newly diagnosed type I diabetic?	0.5-1 units per kg per day (40 kg child--> 20-40 units of insulin per day) divided in 2-3 doses - often a long acting insulin is given in the morning and then fast acting insulin/sliding scale is given with meals
	Treatment of DKA	fluid resuscitation with normal saline or lactated ringer solution of 10 mL/kg IV bolus calculate total fluid deficit and replace this over 48 hours assess the level of hyperglycemia and start an insulin drip at 0.1 unit/kg/hour with the goal being to decrease the blood glucose by 50-100 mg/dL/hour a glucose level that falls to quickly can precipitate cerebral edema when serum glucose approaches 250-300 mg/dL or the anion gap closes, then dextrose should be added to the insulin drip to avoid hypoglycemia frequent measurements of blood glucose, electrolytes and acid-base status
	Annual screening for diabetic children	annual urine evaluation for microalbuminuria ophthalmology exam hyperlipidemia screening
	Pathogenesis of Type II Diabetes Mellitus	Type II DM is a polygenic condition that results from relative insulin resistance and beta cell dysfunction. The insulin resistance initially causes a compensatory increase in insulin secretion, however, with time there is a progressive decline in the glucose-stimulated insulin secretion
	Epidemiology of Type II DM	This accounts for 10-40% of newly diagnosed diabetes in adolescents, which is related to the increase in childhood obesity most cases are diagnosed around the time of puberty there is a higher prevalence in native americans, african americans and hispanics
	Acanthosis nigricans	skin condition involving hyperpigmentation and thickening of the skin folds, found primarily on the back of the neck and flexor areas, which may indicate DM
	What is the only oral diabetes medication approved for use in children?	Metformin-- and only in children > 10 yo
	What is the definition of hypoglycemia - at what plasma and whole blood glucose level does it occur?	plasma glucose < 50 mg/dL whole blood glucose < 60 mg/dL
	Causes of hypoglycemia	hyperinsulinism (congenital, insulinoma, exogenous administration of insulin) ketotic hypoglycemia hormone deficiency (ACTH with or without growth hormone deficiency) glycogen storage disorder (glucose-6-phosphatase deficiency) disorders of gluconeogenesis (hereditary fructose intolerance, fructose 1,6-diphosphatase deficiency) defects in fatty acid oxidation
	Clinical manifestations of hypoglycemia	These are divided into two categories: 1) activation of the autonomic nervous system and release of epinepherine, 2) neuroglycopenia ANS activation- sweating, shaing, tachycardia, anxiety neuroglycopenia- HA, visual disturbances, lethargy, irritability, mental confusion, loss of consciousness, coma
	Evaluation of hypoglycemia	Need to obtain a "critical sample" when the blood glucose is > 50 mg/dL. This should include: chem panel with bicarbonate, insulin, c-peptide, cortisol, GH, FFA, beta-hydroxybutyrate, acetoacetate, lactate and ammonia in a normal fasting state you would expect glycogen stores to be depleted and levels of gluconeogenic substrates, FFA, beta hydroxybutyrate (the major ketone body) to rise significantly. GH and cortisol should be upregulated and insulin levels should be undetectable
	Diabetes Insipidus - definition and causes	Loss of ADH/vasopressin release from the posterior pituitary which results in the inability to concentrate urine. This may occur after head trauma or with a brain tumor or CNS infection surgical removal of a craniopharyngioma often causes this by interrupting the pituitary stalk it is rare for DI to be an isolated idiopathic disorder
	Clinical manifestations of diabetes insipidus	abrupt onset of polydipsia and polyuria if water intake is inadequate then there can be severe dehydration and hypernatremia there may be focal neurological deficits depending on the cause of DI increased urine output may reach 5-10 L/day with a urine specific gravity and urine osmolality that are quite low serum sodium and serum osmolality increase over time leading to hemoconcentration
	Diagnosis of DI	water deprivation test- in normal individuals urine will become concentrated with deprived of water, however in patients with DI the urine will continue to be dilute with a urine osm < 300 mOsm/kg in the setting of hypertonicity (hypernatremia and plasma osm > 295 mOsm/kg) ADH levels are necessary to determine primary from secondary causes in secondary causes there is renal resistance to ADH (nephrogenic DI)- this may be from a rare x-linked condition or lithium use
	Treatment of DI	Administration of Desmopressin acetate (DDAVP), an ADH analogue that can be given intranasally, subQ, or orally
	SIADH	Syndrome of Inappropriate ADH secretion-- excess ADH is released, resulting in: normovolemic hyponatremia relatively concentrated urine normal renal, thyroid, and adrenal function symptoms are related to how fast the hyponatremia develops and are not expected to occur with Na+ levels > 125 mEq/L. Headache, nausea, and lethargy may occur when the sodium falls < 125 mEq/L This is a diagnosis of exclusion. Other causes such as hyperglycemia, increased serum lipids or protein must be ruled out A serum osm <280mOsm/kg combined with a urine osm >200 mOsm/k and a urine sodium concentration > 20 mEq/L are consistent with SIADH The patient should appear euvolemic
	Treatment of SIADH	most cases can be managed with fluid restriction to <1000 mL/day In chronic cases Demeclocycline- a drug that causes reversible nephrogenic DI may be used treatment for acute symptomatic hyponatremia can be conducted with hypertonic saline with a goal of increasing the serum sodium by 0.5 mEq/hour and NO more than 12 mEq/day. Serum sodium should be monitored every 3-4 hours
	What are the two most common causes of short stature (causing 80% of cases)?	familial/genetic short stature and constitutional delay. These both cause proportionate short stature, which is most commonly the case in pathologic causes as well
	What disorders can cause a disproportionate short stature?	Rickets (vitamin D deficiency) and Achondroplasia (dwarfism)
	Causes of proportionate short stature	These need to be divided into prenatal and postnatal causes Prenatal: IUGR, placental dysfunction intrauterine infections, teratogens, chromosomal abnormalities. The most common chromosomal causes are Trisomy 21 and Turner syndrome (45 X,0) Postnatal: malnutrition, chronic systemic diseases, psychosocial deprivation, drugs, endocrine defects, such as hypothryoidism, GH deficiency, glucocorticoid excess, and precocious puberty
	What is the affect of precocious puberty on height?	Initially there is a growth acceleration prior to their peers, however the patient then stops growing sooner and their final adult height is shorter than it would otherwise have been
	By what age to children with familial short stature manifest this condition?	children with familial causes of short stature manifest growth curves at or below the 5%ile by 2 years of age. They are otherwise completely healthy and have normal bone ages and puberty occurs at the normal time
	Constitutional delay	children with constitutional delay develop at or below the 5th percentile however their growth curve is parallel to other children, indicating normal growth velocity puberty is significantly delayed, which results in a delay in bone age family member are usually of average height and these children will likely mature to the height expected for their family, just later than their peers
	Growth hormone deficiency	children with this condition have a diminished growth velocity (less than 5 cm/yr) Findings that suggest idiopathic GH deficiency are history of birth asphyxia orneonatal hypoglycemia, or physical findings of microphallus, cleft palate, or midline defects
	When should you suspect a tumor as the cause of growth problems? What tests should you order?	older children with a recent onset of subnormal growth CNS deficits IGF-1 or IGF-BP3 levels should be checked as well as a timed sampling of GH itself TSH, T4 and T3 should also be measured
	What questions are important to ask in taking the history of someone who presents for evaluation of short stature in a child?	prenatal and birth history pattern of growth presence of chronic disease long-term medication use (methylphenidate and dextroamphetamine can cause growth deficits achievement of developmental milestones growth and pubertal patterns of patient's parents evaluate child's growth charts-- proportionate, disproportionate, changes in growth velocity etc feeding history
	In failure to thrive, what is the head circumference like generally compared with the height and weight?	Height and weight are generally normal, whereas head circumference is normally spared
	What is the most common cause of hyperthyroidism in children?	Grave's disease- an autoimmune disorder caused by circulating thyroid stimulating antibodies binding to throptropin receptors on thyroid cells, which results in diffuse hyperplasia and increased levels of T4. TSH is suppressed
	Clinical manifestations of hyperthroidism	increased appetite without weight gain or loss heat intolerance emotional lability restlessness excessive sweating frequent loose stools poor sleep exopthalmos- uncommon in children palpitations in older children change in behavior and/or school performance
	Physical exam findings consistent with Grave's disease/hyperthyroidism	flushed appearance fidgety warm proptosis hyperactive precordium resting tachycardia widened pulse pressure thyroid gland is enlarged, smooth, firm but not hard and non-tender with a bruit on auscultation often there is a fine tremor noted proximal muscle weakness
	Symptoms of malignant hyperthyroidism	acute onset of tachycardia, hyperthermia, diaphoresis, fever, nausea, and vomiting = thyroid storm which can be life-threatening but is fortunately rare in children
	clinical manifestations in infants with neonatal Grave's disease	staring, jittery, hyperactive, increased appetite but poor weight gain. Tachycardia is usually present and the thyroid may feel enlarged on palpation. There may also be evidence of CHF as the CV system is very sensitive to increased thyroid levels
	Treatment of neonatal Grave's disease	Neonatal grave's disease generally resolves over the first several months of life as maternal antibodies are cleared. In infant who are not hemodynamically stable, IV fluids, digoxin, propanolol, and antithyroid meds may be needed
	Management of Grave's disease in children	Radioiodine ablation is usually delayed until adolescence to avoid radiation exposure in young children. Overdosing with resulting hypothyroidism is preferred over underdosing and then requiring a second dose/exposure to radiation In children anti-thyroid drugs like methimazole and PTU can be used, but this requires a prolonged period of treatment- usually 2-5 years and less than 60% of children with achieve remission with drug therapy alone PTU is associated with idiopathic hepatic toxicity and agranulocytosis
	What is the most common cause of juvenile or acquired hypothyroidism? What are the characteristics of this disease?	Hashimoto's thyroiditis- chronic lymphocytic thyroiditis that results in autoimmune destruction of the thyroid gland The incidence of Hashimotos is 4 times higher in women than men and there is often a history of grave's or hashimoto's in the family This generally presents during adolescence
	Clinical manifestations of hypothyroidism	cold intolerance diminished appetite lethargy constipation slow linear growth delayed puberty immature body proportions coarse puffy facies dry thin hair DTRs with delayed relaxation
	Diagnostic evaluation of hypothyroidism	Thyroid function tests- decreased T4 and T3, depending on the cause there is either increased or decreased TSH. If the pituitary gland or the hypothalamus is at fault then it will be low, whereas other causes will result in increased TSH
	Treatment of hypothyroidism in children	thyroid hormone replacement with Levothyroxine is required Free T4 and TSH need to be monitored frequently upon starting therapy and yearly once normal levels are attained failure to reach adequate thyroid levels for longer than 6-9 month results in diminished linear growth and may decrease the final adult height achieved
	What is the most common type of congenital adrenal hyperplasia? What is elevated in this condition? What is decreased?	21-hydroxylase deficiency. Lack of this enzyme leads to build up of the precursor 17-hydroxyprogesterone. There is decreased production of both cortisol and aldosterone. - this is an autosomal recessive condition
	What is elevated in 11-hydroxylase deficiency? What is decreased?	11-deoxycortisol builds up and cortisol and aldosterone are both decreased - this is an autosomal recessive condition
	Clinical manifestations of 21-hydroxylase deficiency	female infants are born with ambigious genitalia, while males are born with normal-appearing genitalia in females, there is normal ovarian development and the internal genital structures are female male infants tend to present with poor feeding, failure to thrive, lethargy, dehydration, hypotension, hyponatremia, and hyperkalemia within the first 2-4 weeks of life, symptoms of emesis, salt wasting, dehydration and shock develop There is hyponatremia and hyperkalemia from lack of aldosterone and hypoglycemia from lack of cortisol
	How is 21-hydroxylase deficiency diagnosed?	Elevated serum levels of 17-hydroxyprogesterone > 5,000 ng/dL
	How does 21-hydroxylase deficiency differ from 11-hydroxylase deficiency in terms of electrolyte levels?	In 11-hydroxylase deficiency there is a build up of deoxycorticosterone which has mineralocorticoid activity. This leads to hypernatremia, hypokalemia and hypertension-- the opposite of what is seen in 21-hydroxylase deficiency where there is decreased mineralocorticoids
	What is the treatment for congenital adrenal hyperplasia?	Cortisol therapy reduces ACTH secretion and overproduction of androgens Mineralocorticoid administration depends on the type of CAH and is given to normalize renin levels If salt loss occurs then fludrocortisone can be given Stress dosing of cortisol is needed whenever the patient has physiological stress, such as: fever >101F, vomiting, illness, trauma, surgical procedures
	Addison disease	Primary adrenal hyperplasia- this may be congenital or acquired, but results in decreased cortisol secretion. Depending on the disease process there may also be concomitant decrease in aldosterone release. Symptoms: weakness, nausea, vomiting, weight loss, headache, emotional lability, salt craving, postural hypotension, increased pigmentation over joints an on scar tissue, lips, nipples, and oral mucosa due to increased ACTH secretion-- melanocyte stimulating hormone is a byproduct of this pathway adrenal crisis is possible- fever, vomiting, dehydration, and shock that may be precipitated by intercurrent illness, trauma or surgery
	What are the causes of primary adrenal insufficiency in the newborn?	Adrenal hypoplasia ACTH unresponsiveness adrenal hemorrhage adrenal ischemic infarction with sepsis (waterhouse-friderichsen syndrome)
	What is the most common cause of primary adrenal insufficiency in older children and adolescents?	Autoimmune adrenal insufficiency - other potential causes are tuberculosis, hemorrhage, fungal infection, neoplastic infiltration and HIV infection
	Adrenoleukodystrophy	X-linked recessive disorder of long-chain fatty acid metabolism that results in adrenal insufficiency and progressive neurologic dysfunction
	What is the most common cause of ACTH deficiency (secondary adrenal insufficiency)?	Chronic steroid therapy that results in pituitary suppression -- decreased ACTH * congenital hypopituitarism or pituitary tumors (craniopharyngioma) are also possible causes
	Adrenal crisis	A medical emergency resulting from inadequate cortisol production that is characterized by fever, vomiting, dehydration and shock. This may be precipitated by intercurrent illness, trauma or surgery, especially if a stress dose of cortisol is not given in these situations
	What electrolyte abnormalities would you expect to see in untreated Addison's disease?	hyponatremia, hyperkalemia, and hypoglycemia. There may be also a mild metabolic acidosis from dehydration. There should be elevated ACTH levels with decreased cortisol
	Treatment of adrenal crisis? Addison's disease long-term management?	Adrenal crisis- this is an emergency. Correct the electrolyte abnormalities and dehydration by giving 5% dextrose in normal saline and a stress dose of IV glucocorticoids Long-term management of adrenal insufficiency- maintenance doses of oral glucocorticoids and mineralocorticoids and stress doses as needed.
	Cushing syndrome - cause and symptoms	a group of symptoms caused by excess cortisol either from exogenous or endogenous sources symptoms include: slow growth with pubertal arrest, "moon" facies, central obesity, abdominal striae, acne, hirsuitism, facial flushing, hyperpigmentation, hypertension, fatigue, muscle weakness, buffalo hump, emotional changes
	What is the most common cause of cushing syndrome in children older than 7 yo?	Cushing disease- bilateral adrenal hyperplasia due to a pituitary overproduction of ACTH (usually due to a pituitary adenoma)
	What is the most common cause of cushing syndrome in children younger than 7 yo?	adrenal tumors
	How do you diagnose cushing syndrome?	First you need to document increased cortisol levels via a serum cortisol then a 24 urine free cortisol. Second- you need to conduct a dexamethasone suppression test. If dexamethasone does not suppress cortisol levels then this is consistent with cushing syndrome
	Treatment of cushing disease	Surgical removal of the pituitary adenoma- usually with a transsphenoidal approach. Patients may have cortisol and mineralocorticoid deficiency post op- watch for these.
	Precocious puberty- definition	Secondary sex characteristics presenting in a girl before the age of 7 or a boy before the age of 9. This may be either gonadotropin dependent or independent. gonadotropin dependent is much more common.
	What is the difference in the etiology of precocious puberty in females vs males?	In females, true precocious puberty is usually idiopathic, whereas in boys there is a greater incidence of CNS pathology. Tumors that cause gonadotropin dependent precocious puberty include: hamartomas, gliomas, embryonic germ cell tumors. Other CNS causes include: hydrocephalus, head injury, and CNS infection
	McCune-Albright syndrome	a genetic disease that consists of precocious puberty, cafe au lait spots, and polyostotic fibrous dysplasia
	Precocious telarche	Isolated early breast development in females. The usual age of onset for this is 12-24 months. It occurs due to small transient bursts of estrogen from the prepubertal ovary of from increased sensitivity to low levels of estrogen in the prepubertal female.
	Premature adrenarche	early appearance of sexual hair before 8 yo in girls and 9 yo in boys this is a benign condition caused by early maturation of adrenal androgen secretion it is an isolated finding in children with normal growth rate and bone age
	How can you tell the difference between precocious puberty and precocious thelarche or premature adrenarche?	In precocious thelarche, gonadotropin and estrogen levels are in the prepubertal range and their is no accelerated growth or advanced skeletal maturation. In premature adrenarche, adrenal androgens are elevated above norms for chronological age but are normal for the pubertal stage. The bone age is slightly advanced but there is a normal growth rate. In gonadotropin dependent precocious puberty there is development of secondary sex characteristics in combination with a growth spurt. There is advanced bone age and pubertal levels of LH, FSH, estrogen or testosterone.
	Treatment of precocious puberty	If the cause if gonadotropin dependent, then GnRH (Leuprolide) is given. This suppresses the release of endogenous GnRH and decreases secondary sex characteristics, slows skeletal growth and prevents premature fusion of the epiphyseal plates
	Pubertal delay- definition	delay in the onset of puberty or the rate at which is progresses in females this refers to the absence of secondary sex characteristics at 13 years of age or the absence of menarche 3 years after onset of sexual development in males, this refers to absence of secondary sex characteristics at 14 years of age or failure to complete genital growth 5 years from the onset of puberty
	What is the most common cause for pubertal delay?	Constitutional delay- this account for 90-95% of cases and there is usually a family history of it.
	Differential diagnosis for pubertal delay	systemic disease turner syndrome 45 X,0 Klinefelter syndrome 47 X,X,Y Kallman Syndrome- hypogonadotrophic hypogonadism isolated gonadotropic deficiency hypothalamic and pituitary tumors hypopituitarism anorexia nervosa hypothyroidism
	What should the evaluation of pubertal delay include?	examination of growth trends timing of puberty in other family members assessment of the patient's Tanner staging Labs- bone age, testosterone and estradiol levels, gonadotropins, FSH, LH, prolactin, thyroid function tests screen for systemic disease
	Treatment of pubertal delay	For constitutional delay- short course of sex steroids to initiate puberty psychosocial support if there is permanent hypogonadism then sex steroid replacement should be initiated at the time of puberty and continued for life
	What are the body's two main regulators of calcium homeostasis?	Parathyroid hormone (PTH) Vitamin D
	How does PTH act to increase serum calcium levels?	releases calcium stored in bone increases renal retention of calcium increases the production of activated Vitamin D (1,25 OH2 vitamin D), which then increases calcium absorption in the gut
	What is the differential diagnosis of hypocalcemia?	inadequate PTH secretion (hypoparathyroidism) or action of PTH (pseudohypoparathyroidism) vitamin D deficiency or resistence other disorders such as hypomagnesemia, hyperphosphatemia, hypoproteinemia and/or drug toxicity Bartter syndrome renal tubular acidosis (RTA) drug side effects (furosemide, antineoplastics) * magnesium is required for PTH secretion
	What is high risk for vitamin D deficiency and secondary hypocalcemia?	infants that are exclusively breastfed highly pigmented individuals people who take medications that rapidly metabolize vitamin D people who live in areas with limited sun exposure (e.g. Minnesota)
	Clinical manifestations of hypocalcemia	carpo-pedal spasm facial twitching jitteriness tetany seizures prolonged QTc interval
	What are serum calcium and phosphate levels like in hypoparathyroidism? Vitamin D deficiency?	Hypoparathyroidism: low calcium, high phosphate as PTH is not there to stimulate its excretion Vitamin D Deficiency: low calcium, low phosphate. The low calcium stimulates very high PTH levels, which then causes the excessive excretion of phosphate
	Treatment of functional hypoparathyroidism	oral calcium supplements and an active metabolite of vitamin D (calcitriol)
	Differential diagnosis for hypercalcemia	hyperparathyroidism hypervitaminosis D immobilization neoplasia familial hypocalciuric hypocalcemia William syndrome MEN- hyperparathyroidism
	Clinical manifestations of hypercalcemia	may be asymptomatic vomiting lethargy seizures polyuria hypertension psychiatric disturbances kidney stones pathological fractures short QT interval
	Treatment of hypercalcemia	hyrdation with IV saline furosemide (1 mg/kg) in 6-8 hour intervals bisphosphonate infusions to inhibit osteoclasts If the causes is vitamin D excess then glucocorticoids or ketoconazole can be given to suppress the renal activation of vitamin D familial hypocalciuric hypocalcemia is a benign condition, however diagnosis is important so other treatments for the hypercalcemia are not sought
	How do you calculate the amount of maintenance fluid needed for a child?	Holliday-Segar Method- used to calculate the total DAILY requirement: 100 mL/kg/day for the first 10 kg, 50 mL/kg/day for the second 10 kg, and 25 mL/kg/day for every kg thereafter. To calculate the hourly rate the 4-2-1 method can be used. 4 mL/kg/hr for the first 10 kg, 2 mL/kg/hr for the second 10 kg, and 1 mL/kg/hr for every 1 kg thereafter
	What is the best type of maintenance IV fluid for children?	1/4 Normal saline with 5% dextrose (10% dextrose in infants) and 20 mEq/L KCl 1/2 Normal Saline with KCl for adolescents and adults
	Why are infants and toddlers particularly susceptible to dehydration?	The immature kidneys have limited ability to conserve water and electrolytes. And because the child is dependent on their caretakers to supply them with hydration
	What are important benchmarks of the degree of dehydration in children?	weight loss and decreased urine output
	Physical examination findings that are suggestive of dehydration?	The primary mechanism of compensation for dehydration in children is tachycardia. IMPORTANTLY, while hypotension is sensitive early indicator of dehydration in adults, it is a very late and ominous finding in children!
	What is the definition of hypotonic/hyponatremia dehydration?	Serum sodium less than 130 mEq/L -- this may happen in children who electrolytes or supplement with free water excessively
	Hypertonic (hypernatremic) dehydration	serum sodium greater than 150 mEq/L. This is very uncommon in children, but can occur when there are excessive free water losses, such as in diabetes insipidus.
	What is the preferred treatment for mild-to-moderate dehydration in children?	Oral rehydration therapy (ORT) with 90 mEq/L sodium, 20 mEq/L potassium, and 20 g/L of glucose. This is labor intensive as it requires small volumes of fluid to be given very frequently
	When there are fluid deficits, what timeline should be used to replace them?	Most deficits should be replaced over 24 hours, with half of the deficit replaced within the first 8 hours and the rest given over the next 16 hours. If hypovolemic shocks is suspected then a bolus of 20 mL/kg of IV isotonic fluid should be given (normal saline or ringer's lactate) When replacing fluids, maintenance and replacement fluids should NOT contain potassium until a serum K+ level is checked Ongoing GI losses are usually replaced with 1/2 normal saline
	Hyponatremia 1. definition 2. signs and symptoms 3. when does this most often occur?	serum sodium < 130 mEq/L anorexia, nausea, confusion, lethargy, and decreased tendon reflexes. Seizures and respiratory arrest are late findings in children, this most often occurs during dehydration. Clinical findings are more common and severe when sodium level decreases quickly
	What should the lab workup for hyponatremia entail?	serum electrolytes glucose BUN and Cr serum osmolality LFTs protein lipid levels urine sodium concentration and specific gravity
	How does hyperglycemia affect measured serum sodium levels? How can it be corrected?	hyperglycemia makes the serum sodium level appear lower than it is for every 100 mg/dL rise in glucose (above normal - 100 mg/dL) add 1.6 to the measured value to get the corrected value
	What is the treatment of hyponatremia due to either SIADH or renal failure?	Fluid restriction and treatment of the underlying disorder.
	When should hypertonic saline (3%) be used in the setting of hyponatremia?	Its use should be reserved for life-threatening situations, such as intractable seizures
	Hypernatremia 1. definition 2. signs and symptoms 3. rate of correction	serum sodium > 150 mEq/L muscle weakness, irritability, and lethargy. Seizures and coma are major complications the rate of correction should not exceed 1-2 mEq/L per hour in order to prevent cerebral edema
	Hyperkalemia 1. definition 2. most common cause in children 3. differential diagnosis 4. signs and symptoms	serum potassium > 5.8 mEq/L artifact- hemolysis of RBCs during sample collection acidosis, severe dehydration, potassium sparing diuretics, excessive parenteral nutrition, renal failure, addison disease, RTA, massive crush injury with rhabdomyolysis paresthesias, weakness, flaccid paralysis, tetany, ECG changes including peaked T waves
	What are the progressive ECG changes seen in the setting of hyperkalemia?	T-wave elevation - peaked T wave loss of p waves widened QRS complex ST depression Vfib arrest at levels > 9 mEq/L
	Treatment of hyperkalemia	calcium gluconate to stabilize the cardiac myocyte membranes Infusion of sodium bicarbonate or insulin and glucose to push the K+ intracellularly. Hyperventilation may also prove helpful via the same mechanism The only ways to actually remove potassium from the body are with cation exchange resins (Kaexylate) and hemodialysis
	Hypokalemia 1. definition 2. signs and symptoms 3. causes	K+ levels < 3.5 mEq/L weakness, tetany, constipation, polyuria, polydipsia Usually seen in the setting of alkalosis secondary to vomiting, administration of loop diuretics or DKA
	Progressive ECG changes seen in the setting of HYPOkalemia?	prolonged QT interval T-wave flattening (U waves) cardiac arrhythmias such VTach and Vfib- most often occur in patients on digitalis
	What is the differential diagnosis for patients with hypokalemia and elevated blood pressure?	If the urine potassium is elevated then consider: renovascular disease excess renin congenita adrenal hyperplasia cushing syndrome excess mineralocorticoid
	What is the differential diagnosis for patients with hypokalemia, normal blood pressure, and increased urine potassium?	renal tubular acidosis Fanconi syndrome Bartter syndrome Antibiotics diuretics alkalosis increased insulin
	What is the differential diagnosis for patients with hypokalemia, normal blood pressure, and decreased urine potassium?	skin losses GI losses high carbohydrate diet enema/laxative abuse anorexia nervosa
	What is the most consistent physical exam finding in children with metabolic acidosis?	Hypernea
	What is the most common and acid-base disorder and children and what is its definition?	Metabolic acidosis- pH= 7.35 that results from either a loss of HCO3- or increased H+ in the extracellular fluid.
	How do you determine the expected PCO2 for adequate respiratory compensation in the setting of metabolic acidosis?	Winter formula PaCO2 = 1.5 x HCO3- +8 (+/- 2) If the PaCO2 is higher than expected then there is concurrent primary respiratory acidosis If the PaCO2 is lower than expected then there is concurrent primary respiratory alkalosis
	What clinical manifestations are seen in metabolic acidosis?	cardiac contractility is impaired cardiac output is decreased heart is vulnerable to arrhythmias protein breakdown is accelerated and mental status changes occur
	What lab workup should be conducted for metabolic acidosis?	serum electrolytes BUN and Cr glucose venous or arterial blood gas urine dipstick for pH and glucose calculation of the anion gap (cations - anions) = (Na+ + K+) - (Cl- + HCO3-). Normal gap = 12 +/- 4
	How do you calculate an anion gap and what is the normal range?	(cations - anions) = (Na+ + K+) - (Cl- + HCO3-). Normal gap = 12 +/- 4
	What are some causes of anion gap metabolic acidosis?	Methanol Uremia (renal failure) DKA Paraldehyde Isoniazid, Iron ingestion, Inborn errors or metabolism Lactic acidosis Ethanol ingestion, ethylene glycol ingestion Salicyclate ingestion hypokalemia hypocalcemia hypomagnesemia hyperphosphatemia
	What is the differential diagnosis for a non-anion gap metabolic acidosis?	diarrhea renal tubular acidosis hyperalimentation hypoaldosteronism lithium ingestion
	What is the differential diagnosis for a decreased anion gap metabolic acidosis?	hyperkalemia hypercalcemia hypermagnesemia hypoalbuminemia
	What is the treatment of metabolic acidosis?	Treat the underlying condition IV sodium bicarbonate should be reserved for cases in which the pH is less than 7.0 and the cause is unknown or slow to reverse If alkali therapy is given then there must be frequent monitoring of blood pH, sodium, potassium, and calcium
	Metabolic alkalosis 1. definition 2. potential causes 3. potential complications 4. treatment	pH >/= 7.45 (much less common than acidosis in children) "Contraction" alkalosis results from loss of fluid high in H+ or Cl- such as in protracted vomiting or chronic thiazide or loop diuretic treatment reduction in coronary blood flow and arrhythmias, hypoventilation, seizures, and decreased potassium, magnesium, and phospate levels volume expansion with chloride replacement unless the disorder is a resut of mineralocorticoid excess- from renal artery stenosis, adrenal disorders etc as K+ supplements are also necessary in these situations
	Respiratory acidosis 1. Definition 2. Causes	Increase in PaCO2 to above 45 mmHg due to a respiratory cause CNS depression, chest wall muscle weakness, pulmonary or cardiopulmonary diseases
	Respiratory alkalosis 1. definition 2. causes	1. decrease in PaCO2 below 35 mmHg from a respiratory cause 2. lung disease-hyperventilation, mechanical ventilation or any cause (metabolic or neurological) that results in an increased respiratory rate
	Chronic abdominal pain 1. definition 2. most frequent cause in children	1. at least three bouts of abdominal pain severe enough to affect activities over a period of at least 3 months 2. If there is no objective evidence of an underlying organic disorder, the abdominal pain is most often functional
	Differential diagnosis for acute abdominal pain in children	mesenteric lymphadenitis UTIs lower lobe pneumonias PID mononucleosis gall bladder diseases (cholecystitis, choledocholithiasis, biliary colic) pancreatitis PUD Henoch Scholein Purpura (HSP) Kawasaki disease polyarteritis nodosa SLE appendicitis ectopic pregnancy urologic obstruction intussusception
	Differential diagnosis for chronic abdominal pain in children	constipation functional dyspepsia IBS abdominal migraine lactase deficiency celiac disease Inflammatory Bowel Disease (Crohn's disease, ulcerative colitis)
	What should you consider in the setting of abdominal pain, anemia, and poor growth?	Celiac disease
	What is the most common surgical cause of abdominal pain in children?	appendicitis
	What should you think of in a young child with intermittent severe bouts of abdominal pain which may also involve lethargy?	intussusception
	What is the classic presentation of appendicitis in a child?	Fever, emesis, anorexia, diffuse periumbilical pain which then localizes to the RLQ. There is also commonly guarding, rebound tenderness, obturator, and psoas signs.
	At what time from pain onset does the appendix typically perforate if it is going to?	36 hours after the pain begins
	Treatment of appendicitis in children	if uncomplicated- appendectomy if complicated by perforation, then broad spectrum antibiotics such as ampicillin, gentamycin, and metronidazole to treat peritonitis from intestinal flora
	What is one of the most common causes of intestinal obstruction in infancy?	intussusception
	What are some potential lead points in intussusception?	Meckel diverticulum, intestinal polyp, lymphoma, or a foreign body
	Clinical presentation of intussusception	violent episodes of irritability, colicky pain, and emesis that are interspersed with relatively normal periods rectal bleeding occurs in 80% of patients but is less commonly the classic "currant jelly" stool the degree of lethargy in the child may be striking there may be evidence of obstruction on Xray with air-fluid levels A contrast or air enema may demonstrate a characteristic "coiled spring" appearance.
	Treatment of intussusception	fluid resuscitation with normal saline or LR hydrostatic reduction with barium enema or air enema is successful in 75% of cases if performed within the first 48 hours If air and/or barium enema is unsuccessful then direct reduction via laporotomy must be done
	Complications of persistent vomiting in children	dehydration hypochloremic, hypokalemic metabolic alkalosis mallory-weiss tear
	What is important to determine when taking the history regarding a vomiting child?	is the vomiting forceful (actual vomiting) or just spitting up (reflux) Is the vomiting projectile as in pyloric stenosis taking any medications such as antibiotics that might cause it signs of increased ICP diarrhea present too -- gastroenteritis fever and abdominal pain -- appendicitis vaginal discharge -- PID sore throat -- infectious pharyngitis bilious emesis with abdominal pain - obstruction emesis and syncope in female of childbearing age -- consider pregnancy
	Physical exam findings that suggest increased ICP as a cause for vomiting?	Bulging fontanelle, papilledema
	Diagnostic evaluation of vomiting in a child	cultures maybe CBC BMP lipase and amylase CXR for PNA UA and urine cx -- assess degree of dehydration
	Pyloric stenosis	important cause of gastric outlet obstruction and vomiting in the first 2-3 weeks of life Peak incidence occurs at 2-4 weeks, 1:500 infants Males are affected at a rate of 4:1 over females family history is a risk factor clinical presentation: projectile, non-bilious vomiting. Dehydration and poor weight gain are common when the diagnosis is delayed. hypochloremic, hypokalemic metabolic alkalosis olive-sized mass in the epigastric area, but this may be difficult to palpate US reveals hypertrophic pylorus Upper GI study shows classic string sign tx: NG tube to correct dehydration, alkalosis and electrolyte abnormalities. Pylorotomy ASAP
	Malrotation and volvulus	Malrotation- occurs when the small intestines rotate abnormally in utero, resulting in malposition in the abdomen. When this improperly attached intestine twists on its own vascular supply, this is volvulus
	What is the most common age for volvulus to present?	less than 1 month
	Clinical manifestations of volvulus	bilious emesis blood-stained emesis or stool abdominal XRay shows gas in the stomach and a paucity of air in the intestine upper GI series with small bowel follow-through confirms the diagnosis by illustrating the abnormal position of the ligament of Treitz and the cecum unexplained lactic acidosis from intestinal ischemia tx: operative correction of the malrotation ASAP to prevent sepsis
	Gastroesophageal reflux disease (GERD) 1. definition 2. diagnosis 3. clinical manifestations	passage of gastric contents into the esophagus clinical manifestations: vomiting, poor weight gain, dysphagia, abdominal or substernal pain, esophagitis and respiratory disorders if there is forceful or projectile vomiting then reflux is NOT the cause an upper GI series is not sensitive or specific for the diagnosis of GERD, but it is useful for the presence of anatomic abnormalities, such as pyloric stenosis, malrotation, annular pancreas, hiatal hernia, and esophageal stricture esophageal pH monitoring is a valuable tool for diagnosis endoscopy with biopsy can assess the severity of the disease and whether metaplasia (Barretts) is occurring
	Treatment of GERD in children	After 12 months when the risk of SIDS decreases then the child can sleep on the left side and elevate the head of the bed avoid food triggers: caffeine, spicy foods, high fat foods if obese, lose weight. avoid tobacco and etoh PPIs are the most effective medication. If this is not effective then can consider histamine 2 receptor blockers If not responding to treatment, reconsider diagnosis -- might be eosinophilic esophagitis if definitely GERD and not responding to tx- consider fundoplication
	Diarrhea vs enteritis vs colitis	1. Diarrhea- increase in the frequency and the water content of stools 2. Enteritis- small bowel inflammation 3. Colitis- large bowel inflammation
	What is the most common cause of acute diarrhea illness in the world?	Viral gastroenteritis
	What findings on physical exam are consistent with diarrhea as a cause for abdominal pain?	hyperactive bowel sounds abdominal mass plus diarrhea- intussusception or malignancy look for signs of dehydration
	Diagnostic evaluation of diarrhea in children	If there is a history of blood or mucus in the stool, the child needs hospitalization, or the child is less than 3 months old then bacterial stool cultures need to be taken serum electrolytes should be considered for persistent diarrhea - CBC and stool tests (c. diff, cryptosporidium, parasites), immunodeficiency testing, pancreatic insufficiency testing, celiac disease etc
	Treatment of diarrhea in children	Acute gastroenteritis is usually self-limited and supportive care (e.g. avoidance of dehydration) is all that is needed immunization against rotavirus if the child has mild to moderate diarrhea, their normal/preferred diet should be continued avoid clear liquids as a substitute for oral rehydration solution if the child is unable to keep hydrated or has altered mental status, then IV fluids or NG oral rehydration solution is warranted antidiarrheal agents and antiemetics are not recommended as routine management of children with acute gastroenteritis- although zofran is safe and effective antibiotics should be reserved for children with special risks or evidence of a serious bacterial infection
	Treatment of a child with a positive stool culture and a fever 1. younger than 3 months 2. infant older than 3 months 3. any infant who looks toxic or has a positive blood culture	1. admit to the hospital, get blood culture and start IV abx. A LP and UA should be considered 2. admit to hospital, get blood culture, but wait on abx until results of the blood culture is known 3. admit for IV abx. Evaluate for pyelonephritis, meningitis, pneumonia, and osteomyelitis
	Treatment of C. difficile colitis	discontinue concurrent antimicrobial therapy oral metronidazole and vancomyocin consider probiotics
	Constipation 1. definition 2. complications	1. infrequent passage of hard, dry stools 2. impaction, abdominal pain, overflow diarrhea resulting from leakage around the fecal mass, anal fissure, rectal bleeding, UTI due to extrinsic pressure on the urethra, encopresis - accidental soiling/stooling
	What is the most common cause of constipation after the neonatal period?	Functional constipation- voluntary withholding of stool - 90-95%. This may be due to pain associated with defecating, which becomes worse as the stool becomes more hard with increasing constipation
	When should an organic cause for constipation be sought out?	in any patient who fails to pass meconium in the first 24-48 hours of life (e.g. cystic fibrosis, hirshsprung disease)
	Treatment of constipation during childhood	If functional constipation is suspected- determine whether there is a fecal impaction and treat if present. Initiate treatment with oral medication. Provide parental education and follow-up (soiling due to overflow incontinence is NOT a willful or deviant act)
	What is the preferred agent for disimpaction in children? for daily medication/prophylaxis?	Polyethylene glycol (PEG) 3350 for both. The goal is to achieve a soft (mashed potato consistency) stool on a daily or more frequent basis
	Hirschsprung Disease- definition, characteristics and epidemiology	congential aganglionic megacolon occurs in 1 in 5000 children results from failure of the ganglion cells of the myenteric plexus to migrate down to the developing colon there is abnormally innervated distal colon that remains tonically contracted and obstructs the flow of feces 3 times more common in boys this should be suspected in any infant who fails to pass meconium in the first 24-48 hours of life or who requires rectal stimulation to induce bowel movements
	Diagnosis of Hirschsprung Disease	stool that is palpable throughout the abdomen with an empty rectum on exam is suggestive of this disease abdominal xray may show stool and distention throughout the proximal abdomen with no gas or feces in the rectum an anal manometry that demonstrates failure of the internal sphincter to relax with balloon dilation of the rectum is consistent with the diagnosis rectal biopsy showing an absence of ganglion cells, abnormal AChE staining, and hypertrophied nerve trunks is the diagnostic study of choice
	Treatment of Hirschsprung Disease	Treatment is generally surgical in two stages 1. creation of a diverting colostomy 2. resection of the aganglionic segment of the of the bowel and anastamosis of the ganglionic section with the rectum * This procedure is often postponed until the infant is 12 months old or 3-6 months after diagnosis in an older child
	GI bleeding in children- types	hematemesis -emesis of fresh or old blood from the GI tract (fresh blood becomes chemically altered to a coffee-ground appearance within the first 5 minutes of exposure to gastric acid) hematochezia- passage of bright red blood or dark maroon blood from the rectum. The source is usually the colon, although very rapid upper GI bleeding can be the cause melena- passage of shiny, jet black, tarry stools that are positive for occult blood. Usually this comes from an upper GI source
	Differential diagnosis of upper GI (proximal to the ligament of Treitz) bleeds in children	usually seen in critically ill children, as most GI bleeding in children is from lower GI sources esophagitis, gastritis, esophageal varices secondary to portal hypertension, medication (NSAIDs), PUD
	What is the most common cause of minor rectal bleeding in children (stool streaked with blood)?	Anal fissure of polyp
	Differential diagnosis of lower GI bleeds in children (distal to the ligament of Treitz)	fissure- painful, bright red blood on the surface of the stool chronic polyps- painless rectal bleeding on surface of stool, may have some mucus mixed in cow's milk-soy entercolitis- blood mixed with stool, may have diarrhea, hypoalbuminemia and edema meckel's diverticulum- painless, often a large amount of blood inflammatory bowel disease (IBD)- diarrhea, fever, abdominal pain, poor growth, associated systemic signs and symptoms bacterial colitis- diarrhea, abdominal pain, antecedent watery diarrhea (c. diff, E. coli O157:H7) henoch shonlein purpura (HSP)- joint pain, abdominal pain, purpura Intussusception- intermittent abdominal pain, pallor, currant jelly stools, right sided abdominal mass
	What is the immediate priority in the assessment of a child with GI bleeding?	Determine if hypovolemia secondary to an acute bleed is present. First sign will be increased resting heart rate. a drop in blood pressure is not seen until at least 40% of the intravascular volume is depleted assess capillary refill
	Diagnostic evaluation of a child with GI bleeding	CBC Platelet count coag studies if source of bleeding is unclear, then perform a gastric lavage should be considered to determine whether the source of the bleeding is from the upper GI tract proximal to the ligament of Treitz if there is bloody diarrhea, a stool should be sent for culture (bloody diarrhea following several days of non-bloody diarrhea is common in E. coli O157:H7, which can be complicated by HUS in 10-15% of cases consider Meckel's diverticulum if there is a large amount of painless rectal bleeding
	Treatment of GI bleeding in children 1. what if there is a normal hgb or hct? 2. what should guide fluid replacement?	1. a normal hgb or hct does not rule out severe acute bleeding as full hemodilution takes up to 12 hours in the acutely bleeding patient 2. IV normal saline or LR should be given in 20 mL/kg boluses until the vitals improve. Tachycardia should guide fluid replacement as hypotension is a late finding
	Meckel Diverticulum 1. definition 2. peak age of incidence	1. vestigial remnant of the omphalomesenteric duct that is the most common anomaly of the GI tract 2. 2 years of age
	Clinical manifestations of meckel diverticulum	the most common presentation is painless rectal bleeding In symptomatic cases, heterotopic tissue (usually gastric) is ten times more likely to be present because of acid secretion and ulceration of the nearby ileal mucosa 85% have hematocheezia, 10% have intestinal obstruction from intussusception or volvulus, 5% have painful diverticulitis mimicking appendicitis
	Diagnosis and Treatment of Meckel Diverticulum	Meckel scan- Technetium-99-pertechne scan to identify ectopic acid secretion that lead to hemorrhage. Definitive treatment is surgical resection.
	Inflammatory Bowel Disease- types	Crohn's disease and ulcerative colitis Ulcerative colitis- only affects the colon, produces superficial colonic ulceration and crypt abscesses Crohn's disease- transmural inflammation in discontinuous pattern (skip lesions) that may affect any part of the GI tract from the mouth to the anus. Often affects the terminal ileum and may lead to Vitamin B12 deficiency. There may be fistula formation, perforation, or fibrostenotic disease with stricture formation
	Clinical manifestations of IBD	crampy abdominal pain recurrent fever weight loss diarrhea is common, but not universal rectal bleeding is only seen in 35% of Crohn's disease cases abdominal pain tends to be more severe in crohn's disease perianal disease may lead to skin tags, fissures, fistulas, or abscesses anorexia, poor weight gain and delayed growth in 40% of children ulcerative colitis- bloody diarrhea with mucopus, abdominal pain, and tenesmus toxic megacolon and intestinal perforation are rare complications
	Definition of mild, moderate and severe disease in IBD	mild- less than six stools per day, no fever, no anemia, and no hypoalbuminemia moderate- greater than six stools per day, fever, anemia and hypoalbuminemia severe - may be fulminant with high fever, abdominal tenderness, distention, tachycardia, leukocytosis, hemorrhage, severe anemia, and more than eight stools per day
	What is the risk of developing colon cancer in ulcerative colitis, and how should this risk be managed?	After 10 years of disease, there is a cumulative risk of 1-2% per year. After ten years, patients should have annual colonoscopy with biopsy.
	What are some extraintestinal manifestations of IBD?	polyarticular arthritis ankylosing spondylitis primary sclerosing cholangitis chronic active hepatitis sacroiliitis pyoderma gangrenosum erythema nodosum aphthous stomatitis (crohns) episcleritis recurrent iritis uveitis increased risk for nephrolithiaisis secondary to ileal disease and abdominal absorption of oxalate (crohn's disease)
	What does Upper GI studies of patients with crohn's disease often reveal?	ileal or proximal small bowel disease with segmental narrowing of the ileum ("string sign") and longitudinal ulcers
	What type of anemia is common in IBD and why?	Megaloblastic anemia due to folate or B12 deficiency ( In Crohn's disease, the terminal ileum is often involved and this is where B12 is absorbed in the gut)
	Treatment of IBD in children	control of the inflammation and suppression of the immune system 5-aminosalicylic acid (ASA) compounds are the mainstay of anti-inflammatory treatment antibiotics may be used in crohns aggressive nutritional support including tube feedings may be needed in crohns disease early management is often with corticosteroids as these decrease inflammation and the immune response steroid-sparing agents include: mercaptopurine, azathioprine, and methotrexate vitamin and mineral supplements such as folate and B12 may be necessary surgery is eventually needed in 25% with UC and 70% with crohns. Surgery should be limited with crohn's however, as it is not curative and 50% will have a recurrence colectomy is curative in UC as the disease is limited to the colon
	What is the period of childhood when the mortality rate is the highest?	The late fetal and early and neonatal period
	What does the perinatal mortality rate refer to?	fetal deaths occurring from the 20th week of gestation until the 7th day after birth. Intrauterine death represents 40-50% of the perinatal mortality rate.
	What does the neonatal mortality rate refer to?	infants who die between birth and 28 days of life
	What does the postneonatal mortality rate refer to?	death between 28 days to the first year of life. Modern NICUs have allowed children with life-threatening diseases to live longer, leading to an increase in the postneonatal mortality rate when some of these children eventually die
	What is the definition of infant mortality rate and how does it differ with respect to race?	The infant mortality rate includes both the neonatal and postneonatal periods (from 7 days of life to one year). The infant mortality rate is 6.8 per 1000 for all Americans, yet 13.2 per 1000 for African Americans
	APGAR Scoring	means of measuring newborn's health taken at 1 and 5 minutes of life
	What does the APGAR at 1 minute reflect vs that at 5 minutes?	1 minute- intrauterine environment and birth process 5 minute- neonate's success at transitioning from life inside the uterus to outside the uterus
	What does an APGAR score of 8-10 represent? 0-3?	8-10: normal cardiopulmonary transition 0-3: cardiorespiratory arrest or a condition resulting from metabolic acidosis, hypoventilation, and/or CNS depression
	What is assessed in APGAR measurements and how many points are awarded for what?	A- Appearance - Pale or cyanotic = 0 pts, cyanotic extremities = 1 pt, pink throughout = 2 pts P- Pulse - absent = 0 pts, <100 = 1 pt, > 100 = 2 pts G- Grimace/reflex irritability- absent = 0 pts, grimace suction or aggressive stimulation = 1 pt, active cry and avoidance with stimulation A- Activity/ muscle tone- absent = 0 pts, weak, slightly flexed extremities = 1 pt, flexed arms and legs that resist extension = 2 pts R- Respiration - absent = 0 pts, irregular with weak cry, gasping = 1 pt, vigorous cry = 2 pts
	Cephalohematoma 1. definition 2. predisposing factors 3. course 4. complications	type of birth trauma - traumatic subperiosteal hemorrhage (usually the parietal bone) that does NOT cross suture lines. This is fluctuant without discoloration of the overlying skin. May not be apparent until hours to days after delivery large head size, prolonged labor, vacuum extraction, forceps delivery there is generally spontaneous resolution over several weeks there may be indirect hyperbilirubinemia requiring phototherapy, especially in a premature infant, or depression of the calvarium from calcification of the hematoma (2%)
	Caput Succedaneum 1. definition 2. how does it form and when is it usually seen?	diffuse, edematous, and often dark swelling of the soft tissue of the scalp that extends across the midline and/or suture lines this is found in children born vaginally in the occiput anterior position. There is pressure placed on the overriding parietal and frontal sutures that causes head molding, especially in cases of prolonged labor.
	Clavicle Fractures in Neonates 1. prevalence 2. Which clavicle is more likely to be fractured? 3. Predisposing factorss 4. How is this usually diagnosed and what is the appropriate management?	2-3% of vaginal deliveries the right clavicle is 2x more likely to be fractured because the right shoulder must move beneath the pubic symphysis during normal delivery Predisposing factors = large size, shoulder dystocia, and traumatic delivery 80% of affected neonates have no symptoms and only minimal physical findings. Findings that might be seen include: swelling and fullness over the fracture site, crepitus, and decreased arm movement. The injury is often diagnosed a 3-6 weeks of age when a callus is seen at the affected site. Xrays are not needed. Parents should be told to avoid tension on the affected arm.
	Erb Palsy in Neonates 1. definition 2. When should this be expected? 3. Management?	1. Injury to the nerves of the brachial plexus (C5 and C6) that results from excessive traction on the neck, leading to paresis. The infant's arm will be held in the waiter's tip position, with the arm extended and the wrist flexed. 2. If there is an absent Moro reflex in the right arm and the right hand grasp is intact, then Erb palsy should be suspected. 3. 90% of these lesions will resolve spontaneously by 4 months of age. If the deficit persists ten nerve grafting should be considered.
	Low Birth Weight (LBW) vs Very Low Birth Weight (VLBW) 1. definitions 2. associated risks/complications 3. incidence over time	1. LBW = infants weighing less than 2,500 g, VLBW = infants weighing less than 1,500 g 2. LBW infants make up only 7% of all births, but account for up to 2/3 of all neonatal deaths, whereas VLBW infants make up only 1% of all births but represent 50% of all neonatal deaths 3. Although the overall infant mortality rate has declined, there has NOT been an improvement/decrease in the number of LBW births. This is one of the reasons that the infant mortality rate in the US is the worst for any large, industrialized, modern country. However, the survival rate of LBWs s the highest in the US
	Causes of LBW	1. Premature Birth 2. Intrauterine Growth Retardation
	Causes of Premature Birth by category 1. fetal 2. placental 3. uterine 4. maternal 5. other	fetal- fetal distress, multiple gestations, erythroblastosis fetalis, nonimmune hydrops fetalis, congenital anomalies placenta previa, abruptio placenta bicorunate uterus, incompetent uterus pre-eclampsia, chronic medical illness, infection (chorionamnionitis), drug abuse (esp. cocaine) PROM, polyhydramnios, trauma, DES exposure
	Causes of Intrauterine Growth Retardation (IUGR) by category 1. Fetal 2. Placental 3. Maternal	multiple gestations, congenital viral infection, chromosomal abnormalities (trisomies or Turner syndrome), congenital malformation syndromes (esp CNS) Placental - chorionic villitis, chronic placental abruption, twin-twin transfusion, placental tumor, placental insufficiency secondary to maternal vascular disease Maternal- Severe peripheral vascular diseases the reduce uterine blood flow, chronic htn, diabetic vasculopathy, preeclampsia, sickle cell anemia, cardiac disease, renal disease, reduced nutrional intake, alcohol or drug abuse, cigarette smoking, uterine anomalies, uterine constraint (mothers of small stature and reduced weight gain during pregnancy)
	What are maternal factors associated with birth of a LBW infant?	low SES low level of education lack of prenatal care maternal age <16 or >35 short time interval in between pregnancies unmarried status low prepregnancy weight (<100 lbs) poor weight gain during pregnancy (less than 10 lbs) maternal substance abuse African American race
	Postmaturity 1. definition 2. cause 3. Clinical manifestations 4. Treatment	gestation > 42 week unknown in most cases Thy syndrome of postmaturity is characterized by normal length and head circumference but decreased weight. SGA babies are different, as postmature infants were doing well prior to going past 42 weeks when placental insufficiency arose. Symptoms- dry, cracked, peeling, loose, wrinkled skin and malnourished appearance with decreased subcutaneous tissues. Meconium aspiration, persistent pulmonary hypertension of the newborn, hypoglycemia, hypocalcemia and polycythemia are all more common in postmature infants Monitor fetal well-being with US, BPP, and NST. Induce labor for decreased fetal well-being. Early feeding to prevent hypoglycemia. Take caution for perinatal respiratory depression and meconium aspiration
	Small for Gestational Age (SGA) 1. Definition 2. Incidence 3. Pathogenesis - (early onset and late onset)	Birth weights below the 10th percentile for gestational age- includes normal infants that followed a stable growth curve but are small, and infants that suffered from IUGR at some point in utero. 1/3 of LBW neonates-- infants weighing less than 2500 g are SGA Early onset IUGR- symmetrical, thought to arise from an insult that begins before 28 weeks gestation. Head circumference and height are proportionate and weight:height ration is relatively normal. Late onset IUGR- starts after 28 weeks gestation, normal head circumference with reduced length and weight. The weight:height ratio is low and the infant appears long and emaciated. The neonate initially follows a normal percentile growth but the falls off the curve late in gestation when placental function decreases
	Factors to consider in delivery of SGA neonates	delivery should take place in a facility with a NICU delivery team should be prepared for perinatal asphyxia and/or respiratory depression, and hypothermia examination of the placenta may help determine the cause of the IUGR monitor the neonate for hypothermia, hypoglycemia, hypocalcemia, hyponatremia, polycythema, pulmonary hemorrhage, and persistent pulmonary hypertension start feedings ASAP to decrease risk of hypoglycemia
	Large for Gestational Age (LGA) 1. definition 2. risk factors 3. complications and perinatal management	birth weight > 2 standard deviations above the mean or above the 90th percentile maternal diabetes, postmaturity, neonates with transposition of the great vessels, erythroblastosis fetalis, Beckwith-Wiedemann syndrome. Most LGA infants are constitutionall large- large parents or family with predilection for large infants) Check blood sugar, watch for hypoglycemia and feed early. Check Hct as there is an increased risk for polycythemia
	Macrosomia 1. definition 2. macrosomia vs LGA 3. complications 4. risk factors	1. Birth Weight > 4000 g 2. All macrosomic infants are LGA, but not all LGA infants are macrosomic. 3. increased risk of shoulder dystocia and other birth trauma 4. maternal diabetes, obesity, and postmaturity
	Polyhydramnios 1. definition 2. causes of/associations with acute polyhydramnions 3. causes of chronic polyhydramnios	amniotic fluid volume > 2 L (1 in 1000 births) acute- premature labor, maternal discomfort, and respiratory compromise chronic- GDM, immune and nonimmune hydrops fetalis, abdominal wall defect (omphalocele and gastroschisis), multiple gestations, trisomy 18 and 21, neural tube defects (anencephaly and meningomyelocele impair fetal swallowing), esophageal or duodenal atresia, diaphragmatic hernia, and cleft palate interfere with swallowing
	Oligohydramnios 1. associations 2. complications 3. intrapartum management	Decreased amniotic fluid volume 1. IUGR, postmaturity, congenital anomalies of the fetal kidneys 2. bilateral renal agenesis results in Potter syndrome - clubbed feet, compressed facies, low-set ears, scaphoid abdomen, and diminished chest wall size accompanied by pulmonary hypoplasia. Uterine compression in the absence of amniotic fluid retards lung growth and patients with this condition often die from respiratory failure or renal failure. 3. The risk for fetal distress during labor can be reduced by normal saline amnioinfusion
	Neonatal sepsis 1. early-onset sepsis 2. late-onset sepsis 3. nosocomial sepsis	1. birth to 3 days- caused by bacteria residing in the mother's GU tract. Responsible organisms include: group B Strep, E. coli, Klebsiella, and Listeria. Administration of penicillin to mother's with GBS colonization can decrease this risk. Risk factors include prolonged ROM (>24 hours), chorioamnionitis, maternal fever or leukocytosis, fetal tachycardia, preterm birth, African American race, and male sex 2. late-onset sepsis- occurs between 3-28 days, and often happens in a full-term baby who was discharged healthy from the hospital. Bacteremia can lead to meningitis (GBS or E.coli), osteomyelitis (GBS or S. aureus), arthritis (N. gonorrhea, S. aureus, Candida albicans, or gram neg bacteremia) and UTI (gram neg bacteremia) 3. Nocosomially acquired sepsis- day 3- discharge- usually occurs in premature infants in the NICU as many of these infants are colonized by multidrug resistant bacteria indigenous to the NICU. Multiple indwelling devices increases the risk of this. Main culprits include: S. aureus, S. epidermidis, gram neg bacteria and candida albicans
	About what % of women in the US are positive for GBS at that time of delivery?	About 20% -- need penicillin intrapartum
	Clinical manifestations of early-onset sepsis	grunting, tachypnea and cyanosis can be present at birth, which it makes it difficult to distinguish sepsis from RDS in neonates-- so broad spectrum abx are often given to infants with RDS poor-feeding emesis lethargy apnea ileus abdominal distention petechia and purpura if DIC is present
	Clinical manifestations of late-onset sepsis	lethargy poor feeding hypotonia apathy seizures bulging fontanelle fever direct hyperbilirubinemia
	Evaluation of early-onset sepsis	Send blood and CSF for culture CSF gram stain, cell count and differential, protein and glucose levels WBC <5,000 or > 40,000 correlate with increased risk of bacterial infection CXR to look for pneumonia ABGs may be indicated if there is hypoxia or metabolic acidosis associated with shock blood pressure, UOP, CVP, and peripheral perfusion are monitored to determine the need to treat septic shock with fluids and vasomotor agents
	Evaluation of late-onset sepsis	Very similar to the evaluation of early-onset sepsis, except more attention is given to the bones, lab values and urine culture (via suprapubic aspiration or urethral catheterization)
	Treatment of early-onset or late-onset sepsis	A combination of ampicillin and gentamycin for 10 - 14 days once an organism is identified and drug sensitivities are known, then abx coverage can be narrowed/tailored if meningitis is present then treatment is extended and a 3rd generation cephalosporin is added for better BBB penetration
	Treatment of nosocomial sepsis	Depends on indigenous pathogens of the NICU and their antibiotic sensitivities MRSA, coagulase negative staph and gram negative pathogens are the most common causes so vancomycin and gentamycin are usually recommended persistent signs of infection despite antibiotic treatment suggest candidal sepsis and amphoteracin B should be started
	Risk of Chlamydia infection in the newborn	transmitted from the genital tract of the infected mother during delivery 50% of infants born to infected mothers will acquire the infection the risk of conjunctivitis is 25-50% the risk of pneumonia is 5-20% the nasopharynx is the most commonly infected site infected children may have symptoms for more than 2 years!
	Clinical manifestations of chlamydia infection in the newborn	conjuctivitis- ocular congestion, edema and discharge develop a few days to several wees after birth and last for 1-2 weeks pneumonia- usually afebrile illness presenting at 3-19 weeks after birth. Repetitive staccato cough and tachypnea are common. +/- crackles hyperinflation on CXR is prominent
	Treatment of chlamydial infection in the newborn	14 days of oral erythromycin, as topical treatment of the conjunctivitis is often ineffective and does not treat or prevent pneumonia. Test mother and treat her as well as all partners.
	What is the most common cause of respiratory failure in newborn infants?	Respiratory Distress Syndrome (hyaline membrane disease)
	Respiratory Distress Syndrome - pathophysiology	most common cause of respiratory failure in a newborn occurs predominantly in premature infants who are bone with immature lungs in the average child, lung maturity is attained at 32-43 weeks gestation, when pulmonary surfactant is produced by type II pneumocytes the function of surfactant is to decrease the alverolar surface tension and increase lung compliance, preventing alveolar collapse RDS is caused by deficiency of surfactant do to surfactant deficiency, there is poor compliance leading to progressive atelectasis, intrapulmonary shunting, hypoxemia, and cyanosis the forces of mechanical ventilation, oxygen exposure, and alveolar capillary leak then leak to formation of a hyaline membrane that blocks gas exchange
	How can fetal lung maturity be predicted in utero?	Measurement of amniotic fluid lethicin to sphingomyelin ratio (>2 if mature) and phosphatidylglycerol content (present if mature).
	How can the production of surfactant be accelerated when delivery is expected to be pre-term?	Administration of steroids. The following have also been noted to accelerate fetal lung maturity: prolonged ROM maternal narcotic addiction preeclampsia chronic fetal stress maternal hyperthyroidism theophylline
	What maternal factors have been noted to increase the risk of Respiratory Distress Syndrome	hyperglycemia and hyperinsulinemia as in maternal diabetes
	Clinical manifestations of Respiratory Distress Syndrome	within the first few hours of life: tachypnea grunting nasal flaring chest wall retractions cyanosis poor air entry on auscultation CXR shows uniform reticulonodular or ground-glass pattern and air bronchograms that are consistent with diffuse atelectasis
	What is the natural course of neonatal Respiratory Distress Syndrome	progressive worsening over the first 24-48 hours of life after the initial insult, the airway is repopulated with type II pneumocytes that do produce surfactant and there is a sufficient quantity by about 72 hours of life, at which time there is improved lung compliance and decreased respiratory distress along with increased urine output
	Acute complications of neonatal RDS	pulmonary interstitial emphysema pneumothorax pneumomediastinum pneumopericardium intraventricular hemorrhage necrotizing entercolitis
	Treatment of neonatal RDS	artificial surfactant is the primary therapy, although respiratory support is often also needed. Wean vent settings as allowed to prevent injury administration of corticosteroids to mother 48 hours before delivery if preterm delivery is expected risk of lung injury increases as the duration of mechanical ventilation, mean airway pressure, and intermittent mandatory ventilation rate increase evaluate for sepsis and pneumonia which may mimic RDS--- antibiotics are recommended until blood culture results are back
	Bronchopulmonary dysplasia	chronic lung disease of prematurity most common chronic lung disease of childhood commonly affects those infants with very low birth weight born at less than 26 weeks gestation criteria for diagnosis: require O2 therapy for at least 28 days and fail a room air challenge test at 36 weeks postmenstrual age major cause is arrested alveolar development-- decreased number of alveoli -- alveolar hypoplasia complications include: chronic respiratory insufficiency, requiring home O2, diuretics, bronchodilators, right sided CHF secondary to pulmonary htn, and pneumothorax it can take several months to wean the infant off of oxygen and they will often have long-lasting reactive airway disease SIDS is more common in children with this condition lower respiratory infections that are benign in healthy kids, may cause severe respiratory distress in kids with this condition some infants recover fully, but it takes years
	Meconium production, incidence, and risk factors for intrauterine passage	meconium is present in the fetal intestine by the 2nd trimester, however the smooth muscles of the intestines do not typically mature until the 3rd trimester, so meconium passage before 36 weeks is rare. It does not usually occur until several days after preterm delivery. The risk of intrauterine passage of meconium increases with each week thereafter. Fetuses are more likely to pass mec if they are stressed. Overall incidence is 12-15% of all deliveries Risk Factors- post-term gestation and IUGR -- fetal hypoxia has been implicated due to placental insufficiency More common in African Americans
	Meconium Aspiration Syndrome	clinical diagnosis- delivery through meconium-stained amniotic fluid along with respiratory distress and characteristic chest Xray (air trapping and patchy atelectasis) Ranges in severity. In severe disease, positive pressure ventilation is required, which may lead to pulmonary hypertension Complications of severe disease include air leak, chronic lung disease and developmental delay
	Clinical manifestations of Meconium Aspiration Syndrome	tachypnea, hypoxia, hypercapnia meconium in the tracheal of amniotic fluid combined with symptoms of respiratory distress and a CXR that reveals a pattern of diffuse infiltrates with hyperinflation 10% of infants with severe disease will develop pneumothoraces
	Treatment of Meconium Aspiration Syndrome	In pregnancies where uteroplacental insufficiency is documented or suspected, tests of fetal well-being should be conducted to identify high-risk infants When meconium is noted in the amniotic fluid, then the OB should suction the oropharynx before delivering the thorax and the first breath after delivery, if the infant appears vigorous, then intubation is not required, but should have routine suctioning of the oropharynx if the infant has poor respiratory effort, then after suctioning a BVM should be applied and intubation considered Meconium inactivates endogenous surfactant so surfactant administration may help for persistent hypoxia (PaO2 < 50 mmHg) or severe hypercapnia (PCO2 > 60 mmHg), intubation and mechanical ventilation are indicated in cases of persistent pulmonary hypertension of the newborn (PPHN)- ECMO should be considered pulmonary vasodilators (e.g. inhaled nitric oxide) have been shown to be beneficial
	Persistent Pulmonary Hypertension of the Newborn	aka persistent fetal circulation disorder of term and post-term infants who have experienced acute or chronic hypoxia in utero there is a failure of pulmonary vascular resistance to fall with post-natal lung expansion If pulmonary vascular resistance exceeds SVR then there will be right to left shunting of deoxygenated blood either through the foramen ovale or ductus arteriosus the hypoxemia and acidosis that result from the right to left shunting worsens the pulmonary htn -- vicious cycle
	Risk Factors for Pulmonary Hypertension of the Newborn (PPHN)	meconium aspiration severe RDS diaphragmatic hernia pulmonary hypoplasia neonatal pneumonia sepsis Down syndrome
	Clinical manifestations of PPHN	often the severity of the respiratory distress is greater than what could be explained on cxray echo reveals absence of structural heart disease, evidence of increased pulmonary vascular resistance and presence of right to left shunting
	Treatment of persistent pulmonary hypertension of the newborn	pulmonary htn usually resolves within 3-7 days of birth conditions that potentiate pphn include: hypoxia, acidosis, hypoglycemia, hyperviscocity, anemia, and systemic HTN- therapy is designed to combat these supplemental oxygen, hyperventilation, administration of sodium bicarbonate, pulmonary vasodilators, and support of systemic BP inhaled nitric oxide is the cornerstone of treatment sedation assists with not only relaxation of the infant but also pulmonary vasodilation the mortality is 25% in term infants
	Manifestations of hyperbilirubinemia and at what level of bilirubin is it noticeable in a) neonates and b) older children?	jaundice- yellowing the of the skin, mucous membranes, and sclera clinically apparent at total bilirubin of 5 mg/dL in neonates but 2 mg/dL in older children
	What are the potential dangers associated with neonatal hyperbilirubinemia?	Kernicterus - yellow staining of the basal ganglia and hippocampus, resulting in widespread cerebral dysfunction. Clinical features include lethargy, irritability, hypotonia, opisthotonos (state of severe hyperextension and spasticity), seizures, mental retardation, cerebral palsy, and hearing loss
	Physiologic jaundice	elevated bilirubin levels that occur normally after birth secondary to increased RBC volume, decreased RBC survival time, inadequate conjugation, reduced bilirubin excretion, etc This begins at 24 hours of life and is associated with a peak of 12-15 mg/dL at 3-5 days of life and returns to normal by 10-14 days risk factors for developing more severe physiologic jaundice include: prematurity, matrenal diabetes, Asian or Native American ancestry, male gender, exclusive breastfeeding and diminished intake
	breast milk jaundice	although the physiology of why this occurs is not completely understood, the infant's peak bilirubin level tends to be higher and lasts longer than with physiologic jaundice
	When should intervention be considered in neonatal jaundice	infants who develop jaundice within the first 24 hours of life have an increase in serum bilirubin > 0.5 mg/dL/hr have risk factors such as: prematurity, matrenal diabetes, Asian or Native American ancestry, male gender, exclusive breastfeeding and diminished intake have prolonged jaundice (more than 1 week in a full-term infant and more than 2 weeks in a premature neonate) have conjugated hyperbilirubinemia
	What history is essential to gather in the setting of neonatal hyperbilirubinemia?	feeding history, including breast or formula feeding, volumes taken per feed and duration of feeding family history of red cell structural defects, hemoglobinopathies, enzyme deficiencies or a previous child with ABO incompatibility length of time the jaundice has been present, whether it is worsening or improving change in stool or urine color (light or dark)
	How might you determine the extent of hyperbilirubinemia/jaundice in a neonate by physical exam?	jaundice tends to progress in a cephalopedal sequence, so infants with clinically apparent jaundice below the umbilicus are likely to have higher levels than those with only facial jaundice
	Diagnostic evaluation of neonatal jaundice	serum bilirubin, total, conjugated (direct) and unconjugated (indirect) determination of maternal and neonatal blood types if a hemolytic process is suspected then a Coombs test, reticulocyte count and peripheral smear should be ordered bilirubin normograms can be used to help guide decision-making
	Treatment of neonatal jaundice	Two modalities include phototherapy and exchange transfusion. When to use which depends on the birth weight and the bilirubin level of the infant The AAP recommends testing bilirubin levels in ALL infants before hospital discharge Phototherapy converts unconjugated bilirubin into water-soluble photoisomers that can be excreted without conjugation (hydration is important) Exchange transfusion directly removes the bilirubin from the intravascular space as well as the maternal immunoglobulins that may be contributing to a hemolytic process exchange transfusion is usually reserved for bilirubin levels > 25 in the setting of hemolytic disease Phototherapy is not effective in conjugated hyperbilirubinemia

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