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206 Cards in this Set
- Front
- Back
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What is esophageal atresia associated with
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VACTERL
vertebral, anorectal, cardiac, tracheal, esophageal, renal, limb anomalies |
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Suspect esophageal atresia in which neonates
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drooling and excessive oral secretions
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Inability to pass a rigid NG from the mouth to the stomach is diagnostic of what
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esophageal atresia
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Definition of esophageal atresia
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the esophagus ends blindly
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Signs of esophageal atresia
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-Hx of maternal polyhydramnios
-newborn w/ increase oral secretions -choking, cyanosis, coughing during feeding -esophageal atresia w/ fistula -aspiration of gastric contents via distal fistula -tympanitic distended abdomen -airless abdomen on abdominal x-ray |
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Dx of esophageal atresia
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-usually made at delivery
-unable to pass NG tube into stomach -chest x-ray demonstrates air in upper esophagus |
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The most common site of esophageal impactation
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thoracic outlet
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Esophageal foreign body signs
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-gagging.choking
-difficulty with secretions -dysphagia/food refusal -throat pain or chest pain -emesis/hematemesis |
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Dx of esophageal foreign body
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x ray
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Define GERD
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passive reflux of gastric contents due to incompetent lower esophageal sphincter
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Risk factors of GERD
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-prematurity
-neurologic disorders -incompetence of LES due to prematurity, asthma -medications (theophylline, calcium channel blockers, Beta blockers) |
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Signs of GERD
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-excessive spitting up in first week of life
-symptoms resolve without Tx by age 2 -Forceful vomiting -aspiration pneumonia -Chronic cough, wheezing, and recurrent pneumonia -regurgitation |
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Dx of GERD
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-esophageal pH probe studies and barium esophagography
-esophagoscopy w/ biopsy for Dx of esophagitis |
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Tx of GERD
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-positioning following feeds (keep infant upright up to an hour after feeds)
-mealtime more than 2 hrs before sleep and sleeping wiht head elevated -thickening formula w/ rice cereal -Mediations: Antacids, H2 blockers (rantidine), proton pump inhibitors (omeprazole), motility agents (metoclopramide and erythromycin), surgery (Nissen fundoplication) |
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Primary Signs of Peptic ulcers
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- pain, vomiting, and acute and chronic GI blood loss
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Secondary signs of Peptic ulcers
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-stress ulcers secondary to sepsis, respiratory or cardiac insufficiency, trauma or dehydration
-stress ulcers and erosions associated with burns (Curling ulcers) -Ulcers associated with head trauma or surgery (Cushing ulcer) -drug related- NSAIDS or steroids -infections- H pylori |
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Dx of peptic ulcer
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-endoscopy
-Hydrogen breath test for H pylori |
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Tx for Peptic ulcer
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-antibiotic for H pylori: Triple therapy- PPI + 2 antibiotics (amoxicillin, clarthromycin, PPI)
-Antacids, sucralfate, misoprostol -H2 blockers and PPIs |
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How long do you give antimicorbials and PPIs for peptic ulcers
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antimicrobial-14 days
PPIs- 1 mth |
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Define Colic
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Rule of 3's
crying >3 hrs/day, >3 days /wk for >3 wks btw ages of 3 wks and 3 mths |
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Etiology of Colic
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unknown but can be related to
-under or overfedding -milk protein allergy -parental stress -smoking |
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Signs of colic
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-sudden onset of loud crying
-facial flushing -distended, tense abdomen -legs drawn up on abdomen |
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Tx of colic
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-improve feeding method (burping)
-avoid over and underfeeding -resolves spontaneous with time |
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Define Pyloric stenosis
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-most common etiology is idiopathic
-first born male |
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Pyloric stenosis associations
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erythromycin, eosinophilic gastroenteritis, epidermolysis bullosa, trisomy 18, Turners syndrome
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Signs of pyloric stenosis
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-projective vomiting, palpable mass and peristalsis
- nonbilious vomiting - progressive after eating |
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Dx of pyloric stenosis
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-ultrasound
-string sign |
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Tx of pyloric stenosis
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-surgery-Pyloromyotomy
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define Duodenal atresia
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failure to recanalize lumen after solid phase of intestinal development
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Signs of dueodenal atresia
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-bilious vomiting w/o abdominal distension
-Hx of polyhydramnios in 50% of pregnancies -Down syndrome seen in 20-30% |
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Associated anomalies with duodenal atresia
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-malrotation
-esophageal atresia -congenital heart disease |
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Dx of duodenal atresia
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-x-ray: double bubble sign
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Tx of duodenal atresia
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-NG and orogastric compression with IV fluids
-Surgery |
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Define volvulus
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-
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cause of volvulus
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consequence of intestinal malrotation- obstruction is complete, and compromise to the blood supply of the midgut has started
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Signs of volvulus
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-vomiting in infancy
-emesis -abdominal pain-> acute abdomen -early satiety -blood stained stools -distension |
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Dx of volvulus
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-plain abdominal films (bird beak appearance)
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Complications of volvulus
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-perforation
-peritonitis |
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Tx for volvulus
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-surgical correction
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Define intussusception
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invagination of one portion of the bowel into itself
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Most common site of intussusception
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ileocolic (90%)
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Most common cause of intestinal obstruction under 2 yrs old
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intussusception
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Cause of intussusception
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-MC- idiopathic
-viral (enterovirus in summer, rotavirus in winter) -Meckel's -Cystic fibrosis |
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Intussusception triad
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-intermittent colicky abdominal pain
-bilious vomiting -currant jelly stool |
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Dx of intussusception
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-ultrasound (test of choice)
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Tx of intussusception
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- correct dehydration
-NG tube for compression -Barium air/enema |
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Define Meckel's diverticulum
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-persistence of the omphalomesenteric (vitelline) duct (should disappear by 7th wk gestation)
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Meckel's rules of 2
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-2% of population
-2 inches long -2 ft from ileocecal junction -under 2 yrs of age -2% are symptomatic |
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What is Meckel's diverticulum similar to
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- mimic acute appendicitis and also act as lead point for intussusception
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Tx for Meckels diverticulum
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surgical-diverticular resection w/ transverse closure of the enterotomy
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Dx of Meckel's diverticulum
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-meckels scan
-most common mucosa is gastric |
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Signs of Merkel diverticulum
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-intermittent painless rectal bleeding (hematochezia- most common presenting sign)
-intestinal obstruction -diverticulitis |
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Define appendicitis
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-most common cause of emergent surgery in childhood
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Cause of appendicitis
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occurs secondary to obstruction of lumen of appendix
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phases of appendicitis
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1. luminal obstruction, venous congestion progresses to mucosal ischemia, necrosis, and ulceration
2. bacterial invasion w/ inflammatory infiltrate through all layers 3. Necrosis of wall results in perforation and contamination |
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Signs of appendicitis
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-pain, vomiting and fever
-periumbilical pain; emesis infrequent -anorexia -low grade fever -pain radiates to right lower quadrants |
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Dx of appendicitis
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-Hx and physical exam
-CT scan |
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Tx for appendicitis
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-surgery ASAP
-broad spectrum antibiotics needed for perforation (amp, gentamicin, clindamycin, or metronidazole x 7 days) |
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causes of constipation in neonates
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-Hirschsprung
-intestinal pseudo-obstruction -hypothyroidism -organic and inorganic (cow's milk protein intolerance, drugs) -metabolic (dehydration, hypothyroidism, hypokalemia, hypercalcemia, psychaitric) |
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Define constipation
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passage of bulky or hard stool at infrequent times
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Tx for constipation
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-increase fluids and fiber intake
-stool softeners -Glycerin suppositories -Cathartics such as senna or docusate -Nonabsorable osmotic agents |
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Define Hirschsprung megacolon
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-abnormal innervation of bowel (absence of ganglion cells in bowel)
-associated with Down's |
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Signs of Hirschsprung megacolon
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-delayed passage of meconium at birth
-increase abdominal distension-> decrease blood flow-> deterioration of mucosal barrier->bacterial proliferation ->enterocolitis -Chronic constipation and abdominal distension in older children |
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Dx of Hirschsprung's megacolon
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-Rectal manometry- measure pressure of anal sphincter
-rectal suction biopsy- must obtain submucosa to evaluate for ganglionic cells |
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Tx of Hirschsprung's megacolon
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surgery
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Define Imperforate anus
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-absence of normal anal opening
-rectum is blind -can be assoc w/ VACTERL and Downs |
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Signs of Imperforate anus
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-failure to pass meconium
-abdominal distension |
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Dx of Imperforate anus
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-physical exam
-abdominal ultrasonography -sacral radiography -Spinal ultrasound |
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Tx for Imperforate anus
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surgery
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Define Anal fissure
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-painful linear tear in the anal mucosa below the dentate line induced by constipation or excessive diarrhea
-tear of squamous epithelium of anal canal between anocutaneous junction and dentate line |
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Signs of Anal fissure
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-pain w/ defecating/crying during bowel movements
-increase sphincter tone |
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Dx of anal fissure
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anal inspection
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Tx of anal fissure
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sitz bath, fiber supplements, increase fluid intake
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define inflammatory bowel dz
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idiopathic chronic dz including Crohns, and UC
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Signs of inflammatory bowel disease
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-crampy abdominal pain
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Signs of Crohns
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Perianal fistula
sclerosing cholangitis chronic active hepatitis pyoderma gangrenosum ankylosing spondylitis erythema nodosum |
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Signs of UC
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bloody diarrhea
anorexia wt loss pyoderma gangrenosum sclerosing cholangitis marked by flare-ups |
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Tx of Crohns
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-corticosteroids
-aminosalicylates -methotrexate -azathioprine -cyclosporine -metronidazole -sitz baths |
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Tx for UC
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-Aminosalicylates
-oral corticosteroids -colectomy |
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Epidemiology of inflammatory bowel disease
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-common onset in adolescence and young adulthood
-bimodal pattern of patients 15-25 and 50-80 |
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Define irritable bowel syndrome
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abdominal pain associated with intermittent diarrhea and constipation without organic basis
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Signs of irritable bowel syndrome
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-abdominal pain
-diarrhea alternating with constipation |
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Diagnosis of irritable bowel syndrome
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-difficult to make, exclude other pahtologies
-obtain CBC, ESR, stool occult blood |
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Tx of irritable bowel syndrome
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supportive with reinforcement and reassurance
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Define acute gastroenteritis
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inflammation of the entire GI tract and thus involves both vomiting and diarrhea
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define diarrhea
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-excessive loss of of fluid and electrolytes in stool, usually secondary to disturbed solute transport. technically limited to the lower GI tract
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Most common cause of diarrhea in children
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viral - 1. rotavirus 2. enteric adenovirus 3. Norwalk virus
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bacterial causes of diarrhea
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1) campylobacter
2)salmonella and shigella, enterohemorrhagic e.coli |
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what is the difference in the cause of acute and chronic diarrhea
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-acute diarrhea is usually caused by infectious agent whereas chronic persistent diarrhea may be secondary to infectious agents, infection of immunocompromised host, or residual symptoms due to intestinal damage
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Systemic manifestation of diarrhea
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fever, malaise and seizures
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Inflammatory diarrhea symptoms
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fever, severe abdominal pain, tenemus, may have blood/mucus in stool
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noninflammatory diarrhea symptoms
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emesis, fever usually absent, crampy abdominal pain, watery diarrhea
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Dx of diarrhea and gastroenteritis
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-examine stool for blood, mucus and leukocytes
-fecal leukocyte -stool cultures -clostridium toxin |
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Tx of diarrhea and gastroenteritis
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-rehydration
-oral electrolyte solution (pedialyte) - |
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what should someone with diarrhea eat
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the BRAT diet (bananas, Rice, applesauce, toast)
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define pseudomembranous colitis
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-antibiotics disrupts normal flora and predisposes to C difficile diarrhea
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signs of pseudomembranous colitis
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-blood and mucus with fever, cramps, abdominal pain, nausea, and vomiting days and weeks after antibiotics
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Dx of pseudomembranous colitis
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-recent Hx of antibiotic use
-C difficile toxin in stool -sigmoidoscopy or colonoscopy |
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Tx of pseudomembranous colitis
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-discontinue antibiotics
-oral metronidazole or vancomycin 7-10 days |
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Define umbilical hernia
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-imperfect closure of umbilical ring -common in low birth wt, female, and african american infants
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Tx for umbilical hernias
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-Most disappear within yr
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Define inguinal hernia
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-processus vaginalis herniates through abdominal wall with hydrocele into canal
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Signs of inguinal hernia
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infant with scrotal/inguinal bulge on staining or crying
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Tx of inguinal hernia
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surgery
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Complication of inguinal hernia
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incarceration
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Define Peutz-Jeghers syndrome
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-mucosa pigmentation of lips and gums w/ hamartomas of stomach, small intestine and colon
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Signs of Peutz Jeghers
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-deeply pigmented freckles on lips and buccal mucosa at birth
-bleeding and crampy abdominal pain |
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Dx of Peutz-Jeghers
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genetic and family studies may reveal Hx
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Tx of peutz-Jeghers
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excise intestinal lesions
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Define Gardner syndrome
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multiple intestinal polyps, tumors of soft tissue and bone
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Signs of gardner syndrome
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-dental abnormalities
-pigmented lesions in ocular fundus -intestinal polyps w/ high malignant potential |
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Tx of Gardner syndrome
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-aggressive surgical removal of polyps
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Define Carcinoid tumors
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tumors of enterochromaffin cells in intestine -usually appendix
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Signs of carcinoid tumors
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-may cause appendicitis
-may cause carcinoid syndrome if mets to liver |
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Tx of carcinoid tumors
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-surgical excision
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define Familial Polyposis
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-large number of polyps on colon
-secondary to germline mutation in APC gene |
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Signs of Familial Polyposis
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-hematochezia
-cramps -diarrhea |
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Dx of familial Polyposis
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- consider family Hx (strong)
-colonoscopy with biopsy (screening every yr after 10 yrs of age) |
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Tx of Familial Polyposis
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surgical resection of affected colonic mucosa
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Define Juvenile polyposis coli
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-most common childhood bowel tumor
-mucus filled cystic glands |
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Signs of Juvenile polyposis coli
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-bright red painless bleeding w/ bowel movements
-iron deficiency |
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Dx of Juvenile polyposis coli
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-colonoscopy
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Tx of Juvenile polyposis coli
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surgical removal of polyp
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Define short bowel syndrome
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decrease absorptive surface and bowel function
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Cause of Short Bowel syndrome
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-may be congential (malrotation or atresia)
-most commonly secondary to surgerical resection |
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Signs of short bowel syndrome
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-malabsorption and diarrhea
-steatorrhea (fatty stools) -dehydration -decrease sodium and potassium -acidosis (secondary to loss of bicarb) |
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Define Celiac dz
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sensitivity to gluten
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cause of Celiac dz
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-cereals, genetics, environment
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Signs of Celiac disease
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diarrhea
failure to thrive vomiting pallor abdominal distension large bulky stools |
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Dx of Celiac Dz
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-anti-endomysial and anti-tissue transglutaminase antibodies
-biopsy: most reliable test |
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Tx of celiac dz
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-dietary restriction of gluten (must avoid barley, ryes, oats, and wheat)
-Corticosteroids |
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Define Tropical Sprue
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-generalized malabsorption associated with diffuse lesions of small bowel mucosa
-seen in people who live or have traveled to certain tropical regions- Caribbean countries, South America, Africa, parts of Asia |
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Signs of Tropical Sprue
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- fever, malaise, watery diarrhea
-after 1 wk, chronic malabsorption and signs of malnutrition including night blindness, glissitis, stomatitis, cheolosis, muscle wasting |
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Dx of Tropical Sprue
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biopsy shows villous shortening, increase crypt depth, and increase chronic inflammatory cells in lamina propria of small bowel
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Tx of Tropical Sprue
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-Antibiotics x 3-4 wks
-folate -Vitamin B12 |
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Definition of Lactase Deficiency
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decrease of absent enzyme that breaks down lactose in the intestinal border
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Causes of Lactase deficiency
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-congenital absence reported in few cases
-decrease because of diffuse mucosal disease |
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Signs of lactase deficiency
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-explosive watery diarrhea with abdominal distension, borborygmi and flatulence
-reccurent, vague abdominal pain -espisodic midabdominal pain |
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Tx of Lactase deficiency
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- eliminate milk from diet
-oral lactose supplement -yogurt |
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define hyperbilirubinemia
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elevated serum bilirubin
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Signs of hyperbilirubinemia
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-jaundice at birth or in neonatal period
-may be lethargic and feedback |
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Dx of hyperbilirubinema
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-direct and indirect bilirubin fractions
-Hb -reticulocyte count -blood type -examine peripheral smear |
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Tx of hyperbilirubinemia
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-Phototherapy
-exchange transfusion |
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Children with cholestatic hepatic disease need which replacements
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vitamins A, D,E, K (fat soluble vitamins)
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What are differentials for hemolysis
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-indirect hyperbilirubinemia
-reticulosis -red cell destruction |
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What does direct hyperbilirubinemia indicate
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-hepatitis, cholestasis, inborn errors of metabolism, cystic fibrosis or sepsis
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What are the differentials if reticulocyte, Coombs and direct bilirubin are normal
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physiologic or pathologic indirect hyperbilirubinemia
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What is Gilbert syndrome
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-benign condition caused by missense mutation in transferase gene resulting in low enzyme levels w/ unconjugated hyperbilirubinemia
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define Crigler -Najjar I Syndrome
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-autosomal recessive, secondary to mutations in glucuronyl transferase
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signs of Crigler Najjar I
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-in homozygous infants -umconjugated hyperbilirubinemia in first 3 days of life
-Kernicterus - pale yellow stool -increase levels of indirect bilirubin after first week after first week in absence of hemolysis |
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Dx Crigler Najjar I
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-measure glucuronyl transferase activity in liver biopsy specimen
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Tx for Crigler Najjar I
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-maintain serum bilirubin <20 for first 2-4 wks of life
-repeated exchange transfusion -phototherapy -hepatic transplant |
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Define Crigler Najjar II
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-homozygous mutation in glucuronyl transferase isoform I activity
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Signs of Crigler Najjar II
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-unconjugated hyperbilirubinemia in first 3 days of life
-concentration remains increased after third week of life -unusual kernicterus -stool normal -infants asymptomatic |
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Dx of Crigler Najjar II
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-concentration of bilirubin nearly normal
-decrease bilirubin after 7-10 day treatment w/ phenobarbital may be diagnostic |
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Tx for Crigler Najjar II
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-Phenobarbital for 7-10 days
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Define Alagille syndrome
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-absence of reduction in number of bile ducts
-results from preogressive destruction of the ducts |
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Signs of Alagille syndrome
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-unusual facies (broad forehead, wide-set eyes, underdeveloped mandible)
-ocular abnormalities -CV abnormalities -Tubulointerstitial nephropathy -vertebral defect |
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Define Zellweger Syndrome
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-caused by progressive degeneration of liver and kidneys
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Signs of Zellweger Syndrome
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-usually fatal within 6-12 mths
-Severe generalized hypotonia -impaired neurologic fxn w/ psychomotor retardation -hepatomegaly -renal cortical cyst -ocular abnormalities -congenital diaphragmatic hernia |
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Dx of Zellweger syndrome
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-absence of peroxisomes in hepatic cells (on biopsy)
-genetic testing available |
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Define Extrahepatic Biliary atresia
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distal segmental bile duct obliteration with patent extrahepatic ducts up to portal hepatis
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Signs of Extrahepatic Biliary atresia
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-alcoholic stools (stools are very light in color)
-increase incidence of polysplenia syndrome with heterotaxia, malrotation, levocardia and intra-abdominal vascular anomalies |
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Dx of Extrahepatic Biliary atresia
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-ultrasound
-hepatobiliary scintigraphy -liver biopsy |
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Tx of Extrahepatic Biliary atresia
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-Exploratory laparotomy and direct cholangiography to determine presence and site of obstruction
-direct drainage if lesion is correctable -surgery if lesion is not correctable |
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Define hepatoblastoma
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-associated with Beckwith-Wiedmann syndrome
-arises from the right lobe of the liver and is unifocal -2 types - epithelial and mixed |
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Signs of hepatoblastoma
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-present in first 18 mths
-Lg asymptomatic abdominal mass -abdominal distension and increase liver size -weight loss, anorexia, vomiting, and abdominal pain |
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Dx of hepatoblastoma
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-alpha fetoprotein level
-Ultrasound to detect mass -CT -MRI |
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Tx of hepatoblastoma
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-complete resection of tumor
-cisplastin and doxorubicin |
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Define Echinococcus
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-transmitted from domestic and wild canine animals
-Hosts are dogs, wolves, coyotes, and foxes -humans are infected by ingesting contaminated food or water |
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signs of Echinococcus infection
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-majority of cyst in liver
-increase abdominal girth, hepatomegaly, vomiting, or abdominal pain - second most common site is lungs; symptoms include chest pain and coughing or hemoptysis |
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Dx of Echinococcus infection
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-clinical
-ultrasound |
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Tx for Echinococcus infection
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-surgery
-albendazole |
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Signs of Amebic Abscess
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-abdominal pain, distension, and liver enlargement w/ tenderness
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Dx of Amebic Abscess
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-increase ESR
-nonspecific ALT increase -stool exam negative in>50% of patients -CT or MRI |
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Tx of Ambic Abscess
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-Metronidazole
-Chloroquine -Aspiration of left lobe abscesses if rupture is imminent |
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Reye Syndrome definition
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-acute encephalopathy and fatty degeneration
-hepatic dysfunction -secondary to aspirin |
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Signs of Reye syndrome
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-URI, influenza or varicella chickenpox followed by recovery, then abrupt onset of protracted vomiting 5-7 days after illness onset
-liver enlargement -first neurological -delirium, combative behavior and stupor -manifestation-Lethargy -neurological symptoms including seizures, coma and death - |
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Dx of Reye syndrome
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-based on clinical staging
-liver biopsy may show yellow to white color because of high triglyceride content |
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Tx of Reye syndrome
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-Airway, breathing, circulation is the priority
-Bedside glucose (provide dextrose to manage hypoglycemia) -control intracranial pressure secondary to cerebral edema |
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Define alpha1 antitrypsin deficiency
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-alpha1 antitrypsin is a major protease inhibitor
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the most likely clinical manifestation of alpha1 antitrypsin deficiency
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newborn jaundice (neonatal cholestasis)
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signs of alpha1 antitrypsin deficiency
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-jaundice, alcholic stools, hepatomegaly in first week of life,
jaundice clears by second to fourth month -may have complete resolution, persistent liver dz, or cirrhosis |
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Dx of alpha1 antitrypsin
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liver biopsy
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Tx of alpha1 antitrypsin
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liver transplant
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define Wilson Dz
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-excessive copper deposition in brain and liver
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Signs of Wilson Dz
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-jaundice, abdominal pain
-hepatomegaly, subacute/chronic hepatitis or fulminant liver failure -portal HTN, ascites, edema, esophageal bleeding -delayed puberty, amenorrhea, coagulation defect -psychosis -tremors -Kayser Fleischer rings |
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Dx of Wilson Dx
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-low serum ceruloplasmin
-high serum copper level -liver biopsy -genetic testing including siblings |
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Tx of Wilson Dx
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-Zinc (blocks absorption of copper in GI tract)
-Copper chelating agents to decrease deposition -pencillamine and trientine |
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which hepatitis are DNA virus
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HBV
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which hepatitis cause the most illnesses in children
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HAV
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Which hepatitis viruses are acute only
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HAV and HEV
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Tx for hepatitis A
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-careful hand washing
-vaccine |
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Dx of Hep A
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-Hx of jaundice in family contacts, or child care playmates or travel to endemic region
-serologic criteria- IgM present at onset and disappears at 4 mths. IgG is detectable after 6 mths -increase alanine transaminase, aspartate transaminase, bilirubin, gamma-glutamyl transpeptidase |
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signs of Hep A
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-abrupt fever, malaise, nausea, emesis, anorexia, abdominal discomfort
-diarrhea -jaundice -all recover |
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risk factors for Hep A
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-daycare, contaminated food and water, endemic travels
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How is Hep A spread
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person to person
fecal-oral |
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Risk factors for Hep B
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-perinatal exposure to hep B surface antigen from mother
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signs of Hep B
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-increase ALT prior to lethargy, anorexia, malaise
-extrahepatic conditions, polyarteritis, glomerulonephritis, aplastic anemia -jaundice: icteric skin and mucous membranes -hepatosplenomegaly and lymphadenopathy |
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Chronic Hep B is a risk factor for what
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hepatocellular carcinoma
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What is seen for someone vaccinated for Hep B and someone with resolved infection
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-vaccinated-antiHbsAg
-resolved infection- antiHbsAg and antiHbcAg |
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Hep B prevention
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-screen blood donors
-screen pregnant women to prevent vertical transmission |
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Tx for Hep B
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-interferon alpha
-liver transplant |
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Tx for Hep C
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-interferon alpha
-ribavirin |
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Signs of Hep C
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-mild and insidious onset
-cryoglobinemia, vasculitides, peripheral neuropathy |
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Dx of Hep C
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-PCR
-increase ALT -confirmed by liver biopsy |
