Omphalocele + Gastroschisis both need staged reduction (لا تتحمس)
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Amniotic Fluid
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Oligohydroamnios
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Polyhydroamnios
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?
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Baby can’t pee
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Baby cant swallow
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DDx
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Weak abd muscles (tx w/ serial foley)
Renal agenesis
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Wardeng-hoffman
Intestinal atresia
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Congenital Umbilical Hernia
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Path
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Incomplete closure of abdominal muscles
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Clx
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· Soft, nontender bulge at umbilicus
· Protrudes with increased abdominal pressure
· Typically reducible
· Non tender
· Dx CLINICALLY (no need for dxic tests)
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Tx
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· Observe (spontaneous closure)
· Elective surgery around age 5
· Do US if it’s ACQUIRED umbilical hernia.
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Coanal Atresia
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CHARGE syndrome is a set of congenital defects which are seen in conjunctions.
C: coloboma of the eye, central nervous system anomalies
H: heart defects
A: atresia of the choanae
R: retardation of growth and/or development
G: genital and/or urinary defects (hypogonadism)
E: ear anomalies and/or deafness
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S/S
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· Baby turns blue while feeding & pink while crying
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Dx
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Clx → CT
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Tx
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Surgery
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Hirshsprung dz
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?
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· Associated w/ Down
· Failure of neurons to migrate
· ø aurbach / myenteric
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S/S
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Can presents in 2 ways:
· FTPM (on DRE → diarrhea)
· Toddler w/ overflow incontinence
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Dx
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· 1˚ X-Ray
· 2˚ Barium
· 3˚ Manometry (⤴️ Pressure)
· BEST: Bx
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Tx
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3-stage Surgery
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Doudenal Atresia
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?
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Associated w/ Down,
Annular pancreas
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S/S
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First 12 hours: Billous Vomiting
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Dx
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X-ray: double-bouble
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Tx
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NGT
Surgery
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Biliary atresia
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Path
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· Idiopathic
· Progressive obliteration of extrahepatic bile ducts
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Clx
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· Asymptomatic at birth
· Age 1-8 weeks:
Jaundice
Acholic stools &/or dark urine
Hepatomegaly
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Dx
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· Conjugated hyperbilirubinemia
· Liver biopsy: bile duct plugs & proliferation, portal tract edema, fibrosis
· Intraoperative cholangiogram (gold standard): biliary obstruction
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Tx
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· Hepatoportoenterostomy (Kasai procedure)
· Liver transplant
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Intususseption
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?
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Associated with previously used Rotavirus vaccine and HSP.
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S/S
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colicky abdominal pain, bilious vomiting, and currant jelly stool.
On PEx: right quadrant sausage-shaped mass
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Dx
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US
Contrast enema “Target sign”
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Tx
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1˚: IVF + Electrolytes
2˚ NGT
Barium Enema (both dx & Tx)
· contraindicated if the child has signs of peritonitis, shock, or perforation.
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Intussusception
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RF
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· Recent viral illness or rotavirus vaccination
· Pathological lead point
Congenital malformation of the intestines (eg, Meckel
diverticulum)
Henoch-Schönlein purpura
Celiac disease
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Clx
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· Sudden, intermittent abdominal pain
· “Currant jelly” stools
· Sausage-shaped abdominal mass
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Dx
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· “Target sign” on ultrasound
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Tx
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· Air or saline enema
· Surgery
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VACTERL syndrome
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· V: vertebral anomalies
· A: anal atresia
· C: cardiovascular anomalies
· T: tracheoesophageal fistula
· E: esophageal atresia
· R: renal anomalies
· L: limb anomalies
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Diarrhea (Gastroenteritis)
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?
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Can be Infectious / non-infectious
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S/S
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Diarrhea → Dehydration
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Dx
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Look for an organism
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Tx
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Fluid Resuscitation
Antidiarrheals such as loperamide are always the wrong answer.
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Necrotizing enterocolitis (NEC)
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RF
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Prematurity (but not only to premature!)
Very low birth weight (<1.5 kg [3.3 1b])
Enteral feeding (formula > breast milk)
Pt w/ CHD -> ⤵️ BF to intestines
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Clx
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Feeding intolerance / Lethargy
Unstable VS (⤵️ T°)
Bilious emesis, bloody stools, abdominal distention
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X-Ray
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Pneumatosis intestinalis
Portal venous gas
Pneumoperitoneum
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Tx
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Bowel rest; parenteral nutrition
Broad-spectrum IV Abx
± Surgery
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Wilms
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Neuroblastoma
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?
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· Semi-hypertrophy of 1 kidney
· (Wilms tumor, aniridia, GU malformations, and mental retardation is referred to as WAGR syndrome. The a deletion on chromosome 11)
· metanephros
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adrenal medulla tumor
(neural crest)
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S/S
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Abdominal mass (does not cross midline)
Anirdia (no iris)
Constipation
N/V
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Abdominal mass
Hypsarrythmia (dancing eyes) and opsoclonus (dancing feet) are hallmarks.
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Dx
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· 1˚: US
· Best˚: Contrast CT
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· ⤴️ URINE VMA and metanephrines
· Bx: small round blue/purple cells
· N-myc gene amplification
· 131I-MIBG body scan to detect metastasis
· MRI
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Tx
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Total nephrectomy + chemo + radiation
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Surgical resection
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Neuroblastoma
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Path
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· Neural crest origin
· Involves adrenal medulla, sympathetic chain
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Clx
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· Median age <2
· Abdominal mass
· Periorbital ecchymoses (orbital metastases)
· Spinal cord compression from epidural invasion (“dumbbell tumor”)
· Opsoclonus-myoclonus syndrome
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Dx
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· Elevated catecholamine metabolites
· Small, round blue cells on histology
· N-myc gene amplification
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Meckel diverticulum
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#
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Rule of 2s:
· 2% prevalence
· Age 2
· 2:1 male/female ratio
· Location within 2 feet of ileocecal valve
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Clx
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· May be asx, incidental finding
· Painless lower gastrointestinal bleeding
· ± Anemia
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Comp
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· Intussusception
· Volvulus
· Intestinal obstruction
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Dx
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· Technetium-99m pertechnetate scan
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Evaluation of neonatal hydration
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Signs
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Decreased wet diapers
Absence of tears
Sunken fontanelle
Dry mucous membranes
Decreased skin turgor
Delayed capillary refill
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Tx
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<7%
· Continue exclusive breastfeeding
· Follow-up at age 10-14 days to check that infant has regained birth weight
≥7%
· Assess for oromotor dysfunction
· Assess for lactation failure
· Dally weights
· Consider formula supplementation
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Differential diagnosis of regurgitation & vomiting in infants
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Dx
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Clx
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Tx
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GERD
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Physiologic
· Asymptomatic
· Happy spittter
Pathologic
· FTT
· Significant irritability
· Sandifer syndrome
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· Reassurance
· Positioning therapy
· Thickened feeds
· Antacid therapy
· If severe, esophageal pH probe monitoring & upper endoscopy
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Milk protein allergy
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· Regurgitation/vomiting
· Eczema
· Bloody stools
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· Elimination of dairy & soy protein from diet
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Pyloric stenosis
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· Projectile nonbilious vomiting
· Olive-shaped abdominal mass
· Dehydration, weight loss
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· Abdominal U/S
· Pyloromyotomy
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HUS
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Path
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Shiga toxin (infection w/ E.coli or Shigella)
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Clx
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· Hx of bloody diarrhea
· Fatigue, pallor
· Bruising, petechiae
· Oliguria, edema
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Dx
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· Hemolytic anemia (schistocytes, ⤴️bilirubin)
· ⤵️PLT
· Acute kidney injury (t BUN, T creatinine)
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Tx
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· Fluid & electrolyte management
· Blood transfusions
· If severe → Dialysis
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PED constipation
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RF
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· Initiation of solid food & cows milk
· Toilet training
· School entry
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Clx
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· Painful/hard bowel movements
· Stool withholding
· Encopresis (feces in underwear >4ys)
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Complications
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· Anal fissures
· Hemorrhoids
· Enuresis/urinary tract infections
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Tx
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· ⤴️Dietary fiber & water intake
· Limit cow’s milk intake to <24 oz
· Laxatives
· ± Suppositories, enema
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Food protein—induced allergic proctocolitis
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RF
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Family history of allergies, eczema, or asthma
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Clx
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· Young infant
· Painless, bloody stools
· ± Spit-up
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Tx
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· Elimination of milk & soy from maternal diet in breastfed infants
· Hydrolyzed formula in formula-fed infants
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Prognosis
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resolution by 1 year
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