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    • fileName "Pediatrics"
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    • Daily fluid requirement of infants/children weighing 10-20kg:
    • 1000mL/day
    • + 50mL/kg/day for every kg over 10kg
  1. Daily protein requirement in infants =
  2. DDX vomiting by age:
    • Duodenal atresia - newborns, bilious
    • Pyloric stenosis - 3-12 weeks, nonbilious
    • Malrotation - first few weeks of life, bilious
    • Midgut volvulus - first few weeks of life, bilious
    • Intussusception - 3-18 mo
  3. Daily fluid requirements of premature infant (< 1.5kg)
  4. Daily fluid requirements of neonates/infants (1.5-10kg)
  5. Daily fluid requirements of children (> 20kg)
    1500mL + 20mL/kg over 20kg
  6. Daily glucose requirement
    4-6 mg/kg/min
  7. Daily fat requirement
    Start infusion at 0.5 g/kg/day and advance to 2.5-3 g/kg/day
  8. Daily sodium requirement
    2-5 mEq/kg
  9. Daily potassium requirement
    2-3 mEq/kg
  10. What is intralobar pulmonary sequestration?
    A congenital pulmonary airway malformation (CPAM) that has a systemic arterial blood supply and a variable venous drainage

    Arterial flow can originate anywhere along the descending aorta inlcuding subdiaphragmatic locations

    A true sequestration has no tracheobronchial communication
  11. What is paradoxical aciduria?
    Urinary loss of acid (H+) and potassium to retain sodium and water in order to preserve fluid volume
  12. What electolyte imbalances are seen with gastric outlet obstruction (ex: hypertrophic pyloric stenosis)?
    • Hypokalemia
    • Hypochloremic metabolic alkalosis (contraction alkalosis)
    • Paradoxical aciduria
  13. Indications for ECMO:
    • Meconium aspiration syndrome (most common)
    • Respiratory distress syndrome
    • Persistent pulmonary hypertension
    • Sepsis
    • Congenital diaphragmatic hernia

    • Selection criteria:
    • - failure of conventional therapy with A-a gradient >620 for 12h or 6h in a pt with extensive barotrauma requiring high inotropic support
    • - oxygen index > 40%
  14. Exclusion criteria for ECMO:
    • Gestational age < 34weeks
    • Birth weight < 2kg
    • Irreversible pulmonary disease
    • Uncorrectable cyanotic congenital heart disease
    • Intractable coagulopathy/hemorrhage
    • Intracranial hemorrhage
    • Hx of > 10-14 days of high pressure mechanical ventilation
  15. Labs to follow while on ECMO:
    • Hematocrit
    • Platelet count
    • Fibrinogen
    • Activated clotting time values
    • Daily cranial ultrasound
  16. Leading cause of death in children 1-15yo is
    Trauma (MVA's, falls, bicycle accidents, child abuse)
  17. Indications for CT in pediatric trauma:
    • Painful distracting injury
    • Significant head injury
    • Unreliable exam
  18. Surgical airway in kids < 12yo =
    Needle cricothyroidotomy with percutaneous transtracheal ventilation
  19. Infant blood volume =
    80 mL/kg
  20. Blood products in infants:
    • PRBC's 10-20 mL/kg
    • FFP 20 mL/kg
    • Platelets 10-20 mL/kg
  21. Testicular torsion diagnosis:
    Doppler ultrasound
  22. Bell clapper deformity =
    Horizontal lie of a testicle as a result of the failure of normal posterior anchoring of the gubernaculum, epididymis, and testis to the tunica vaginalis thereby predisposing the testis to torsion
  23. Testicular torsion treatment:
    Surgical repair within 6hrs of symptom onset and contralateral orchiopexy to prevent future torsion
  24. Management of noncommunicating hydrocele:

    If hydrocele persists past 1yo peritoneal communication is likely and hydrocelectomy with ligation of the patent processus vaginalis is indicated.
  25. Most common incarcerated organs in an inguinal hernia in a girl:
  26. Location of first branchial remnants:
    In the front or back of the ear in the region of the mandible
  27. Location of second branchial remnants:
    Along anterior border of the sternocleidomastoid

    Tract emrges superior to cranial nerve IX and crosses anterior to IX and XII. The cyst lies anterior to X and XII

    Most common
  28. Location of third branchial remnants:
    At the sternal notch or supraclavicular region
  29. Location of Type I first arch anomalies:
    Duplications of external auditory canal
  30. Location of Type II first arch anomalies:
    Angle of the mandible and pass up throuh the parotid gland
  31. Location of second arch anomalies:
    Along anterior boarder of sternocleidomastoid deep to external carotid but superficial to the internal carotid, ending at he tonsillar fossa

    Most common
  32. Location of third arch anomalies:
    Lower neck, enter pharynx at piriform sinus. Pass deep to internal carotid and glossopharyngeal nerve but superficial to vagus nerve

  33. Location of Meckel diverticulum:
    On the antimesenteric boarder of the ileum between 4-60 cm from the ileocecal valve
  34. Cause of Meckel diverticulum:
    Incomplete closure of the omphalomesenteric (vitelline) duct
  35. Tissue found in Meckel diverticulum:
    • Gastric mucosa (50%) - can lead to ulcer formation causing painless
    • lower GI bleeding = most common presenting symptom
    • Ectopic pancreatic tissue (5%)
  36. Most common childhood malignancy:
    Leukemia (40%)
  37. Most common solid tumor of children:
    • < 2yo = Neuroblastoma
    • > 2yo = Wilms tumor
  38. Hepatocellular necrosis & giant cell transformation =
    Neonatal hepatitis
  39. Pathologic findings of biliary atresia =
    • Severe cholestasis
    • Bile duct proliferation
    • Inflammatory cell infiltration
  40. What is biliary atresia?
    Progressive fibrosis of the extrahepatic and intrahepatic bile ducts leading to biliary cirrhosis, portal hypertension, and death by 2yo without surgical correction

    Most common indication for pediatric liver transplant
  41. Work up for suspected biliary atresia includes:
    • US of liver - extrahepatic bile ducts not seen
    • US gallbladder - diminutive/absent
    • HIDA scan
    • Percutaneous liver biopsy
  42. Biliary atresia diagnosis:
    Intraoperative cholangiogram demonstrating lack of opacification of the intrahepatic biliary tree
  43. Describe Kasai procedure.
    Surgical treatment of biliary atresia = Resection of the gallbladder, extrahepatic bile ducts, and fibrotic portal plate followed by Roux-en-Y portojejunostomy to restore biliary flow

    Long-term successful outcome low if performed aftee 90 days of life
  44. Common complications of Kasai procedure =
    • Cholangitis (most common)
    • Biliary cirrhosis
    • Hepatic failure
    • Portal hypertension
  45. Hirschsprung disease diagnosis =
    Rectal biopsy demonstrating submucosal hypertrophied nerve endings, abscent ganglion cells in the Meissner and Aeurbach plexuses, and acetylcholinesterase staining
  46. Malrotation vs Midgut volvulus on upper GI study
    Midgut volvulus = abrupt cutoff from failure of contrast material to pass beyond the distal duodenum or corkscrew pattern of partial obstruction

    Malrotation = aberrant course of the duodenum and duodenal-jejunal junction
  47. Midgut volvulus twists in a ________ direction and needs to be untwisted in a ________ manner.
    • Clockwise
    • Counterclockwise
  48. What are Ladd bands?
    Present in malrotation, they are peritoneal bands between the ascending colon and the posterior abdominal wall in the rigt upper quadrant that must be divided so the duodenum can be mobilized and the small bowel can be positioned in the right side of the abdomen with the colon in the left side
  49. Type A tracheoesophageal malformation:
    True esophageal atresia without tracheoesophageal fistula (incidence = 6%)
  50. Type B tracheoesophageal malformation:
    Distal esophageal atresia with a proximal tracheoesophageal fistula (incidence = 2%)
  51. Type C tracheoesophageal malformation:
    Proximal esophageal atresia with distal tracheoesophageal fistula

    Most common (incidence = 85%)
  52. Type D tracheoesophageal fistula:
    Proximal and distal tracheoesophageal fistula (incidence = 1%)
  53. Type H tracheoesophageal malformation:
    Intact esophagus with a single tracheoesophageal fistula (incidence = 2%)
  54. When are umbilical hernias repaired?
    • Defect > 2cm
    • History of incarceration
    • Large skin proboscis
    • Presence of ventriculoperitoneal shunt
  55. Most common cause of duodenal obstruction at birth =
    Duodenal atresia

    S/S: bilious vomiting within first 24hrs of life without abdominal distention
  56. Cause of intestinal atresia =
    In utero vascular accident

    S/S: bilious vomiting with abdominal distention

    • 10% of pts with jejunoileal atresia have cystic fibrosis
    • Most common & significant morbidity a/w jejunoileal atresia = short gut syndrome
  57. VACTERL association includes:
    • Vertebral anomalies
    • Imperforate Anus
    • Cardiac defects
    • Tracheoesophageal fistula
    • Radial & Renal malformation
    • Limb defects
  58. Treatment of esophageal atresia and tracheoesophageal fistula in medically stable infant weighing > 2500g =
    Primary repair with division of the fistula, closure of its tracheal end, and end-to-end anastomosis of the esophageal segments
  59. Treatment of esophageal atresia and tracheoesophageal fistula in unstable infants =
    Gastrostomy and sump drainage of the blind proximal pouch

    If respiratory problems, may benefit from primary fistula ligation with delayed esophageal repair
  60. Common complications following EA/TE fistula repair:
    • Esophageal strictures
    • Anastomotic leak
    • Recurrent fistula
    • Gastroesophageal reflux
  61. Risk factors for necrotizing enterocolitis:
    • Perinatal stress
    • Maternal cocaine use
    • Prematurity
    • Enteral feeding
  62. Initial injury in NEC:
    Intestinal mucosa

    Spectrum of severity ranges from isolated mucosal injury to transmural bowel necrosis
  63. Most commonly affected sites in NEC:
    Terminal ileum and right colon

    But disease can be segmental or affect entire GI tract
  64. Necrotizing enterocolitis (NEC) treatment:
    • Initial: nasogastric decompression, bowel rest, broad-spectrum antibiotics, optimal fluid management, parental nutrition
    • Close monitoring with physical exam, serial radiographs, & biochemical assessment for signs of deterioration

    • Operative: bowel resection with proximal enterostomy
    • Indicated to treat acute complications including:
    • - intestinal perforation
    • - persistent necrosis evidenced by progressive clinical deterioration
    • - persistent bleeding
    • - obstruction

    Operative intervention does not prevent progression of disease
  65. What is pneumatosis intestinalis?
    Pathognomonic radiographic finding of NEC thst indicates invasion of the bowel wall by gas-forming organisms

    Not an absolute indication for surgery
  66. Best way to clinically monitor cardiac perfusion in a newborn =
    Capillary refill. Should be < 1sec
  67. Cardiac output in newborns is primarily dependent on ________.
    Heart rate.

    Ability to increase cardiac output depends on increase in heart rate, not stroke volume
  68. Why are newborns immunodeficient?
    Low immunoglobulin and C3b levels
  69. Incidence of congenital diaphragmatic hernia =
    1 in 2000-5000 live births
  70. Most common diaphragmatic defect leading to herniation is ______.

    This is called:
    Posterolateral location

    Known as Bochdalek hernia.
  71. What is a Morgagni hernia?
    Anteromedial (retrosternal) defect in the diaphragm leading to herniation
  72. Congenital diaphragmatic hernia diagnosis:
    Prenatal ultrasound.

    Plain CXR demonstrating intrathoracic location of gastric air bubbles, nasogastric tube, or intestines
  73. Treatment of congenital diaphragmatic hernia:
    Wait 24-72 hours for cardiorespiratory stabilization followed by surgical repair. (Emergent repair does not improve outcomes)

    Survival rate 70-90%
  74. Cause of morbidity and mortality in infants with congenital diaphragmatic hernia =
    Pulmonary hypoplasia & pulmonary HTN (due to pulmonary arteriolar vasoconstriction)

    High pulmonary vascular resistance produces right --> left shunting via the patent ductus arteriosus
  75. Initial resussitation of infants with respiratory distress caused by congenital diaphragmatic hernia:
    Goals= correction of hypoxia, metabolic acidosis, & hypothermia to relieve pulmonary vasoconstriction

    • Endotracheal intubation with high-frequency oscillatory ventilation or inhaled nitric oxide (pulm vascular dilator) to avoid barotrauma
    • - high pressure ventilation may cause PTX requiring chest tube
    • Orogastric tube placement
    • IV fluids
    • ECMO in infants who remain critically ill despite conventional support
  76. Bronchopulmonary malformations:
    • Congenital Pulmonary Airway Malformation (CPAM)
    • -Pulmonary sequestrations (intralobar or extralobar) - associated with congenital cardiac defects
    • -Congenital cystic adenomatoid malformation (CCAM)
    • Congenital lobar emphysema
  77. Cause of congenital lobar emphysema:
    Overdistention of a histologically normal lung because of air trapping secondary to abnormal cartlaginous support of the feeding bronchus
  78. Describe the difference between gastroschisis & omphalocele
    • Omphalocele - umbilical cord arises from the layers of peritoneum and amnion covering the abdominal wall contents
    • 50% of infants born with omphalocele have other malformations including cardiac and chromosomal

    Gastroschisis - a complete abdominal wall defect located to the right of the umbilical ring
  79. Treatment of abdominal wall defects:
    Initial: nasogastric decompression, IV fluids, broad-spectrum antibiotics, protection of abdominal wall contents

    • Omphalocele - cover sac with non-occlusive dressing
    • Gastroschisis - place silo bag over exposed intestines

    Later surgical correction
  80. How to distinguish Wilms tumor from neuroblastoma in a 3yo with an asymptomatic palpable abdominal mass:
    Urine vanillylmandelic acid (VMA) level - elevated in neuroblastoma

    CT/MRI - neuroblastoma = displacement of intact kidney, Wilms = tumor of renal origin
  81. Wilms tumor is associated with what syndromes?
    • Denys-Drash syndrome
    • WAGR syndrome (wilms, aniridia, GU abnormalities, mental retardation)
    • Beckwith-Wiedemann syndrome
  82. Third most common pediatric malignancy:

    • #1 - leukemia
    • #2 -
  83. Colicky abdominal pain and guiac-positive stools in a child between 3-18 months
    Ileocolic intussusception
  84. Complications of cystic hygroma:
    • Infection (most common)
    • Hemorrhage
    • Resporatory distress
    • Malignant degeneration (rare)
  85. What is a cystic hygroma?
    Congenital lymphangiomatous malformation that commonly occurs in the posterior neck, axilla, groin, or mediastinum
  86. Newborn with abdominal distention, bilious vomiting, and radiographic finding of "soap bubbles"
    Meconium ileus
  87. Meconium ileus treatment:
    • Gastrografin enema
    • The detergent and hyperosmolar effects may loosen the thick meconium and relieve the obstruction

    Surgery if no response to gastrografin enema or if peritonitis/perforation present
  88. Ileocolic intussusception treatment:
    Barium or air enema for diagnosis & possible reduction (successful in 80%) - do not attempt if perforation/peritonitis

    Operative manual reduction with appendectomy

    Surgical ressection with primary ileocolic anastomosis (primary tx if nonviable bowel is present)
  89. Indications for surgical treatment of ileocecal intussusception:
    • Perforation/peritonitis
    • Nonviable bowel
    • Multiple recurrences
    • Suspected lead point - intestinal polyp, Meckel diverticulum, tumor (lymphoma)
  90. Ileocecal recurrence rate:
    5-10% regardless of radiographical or operative reduction
  91. "Double-bubble" sign on abdominal radiographs indicates:
    • Duodenal atresia
    • Normal finding (air takes 6-12hrs to reach distal colon)
    • Malrotation - air present in distal intestines
    • Midgut volvulus - air present in distal intestines
  92. Duodenal atresia treatment:

    Also pass foley catheter through distal part of duodenum to assess for intraluminal windsock diaphragm or partial webs
  93. Most reliable test for diagnosing gastroesophageal reflux in pediatric patients:
    12-24hr esophageal pH study is the most sensitive & specific
  94. What is a teratoma?
    Tumors arising from more than one of the three embryonic germ layers containing tissue that is foreign to the anatomic site in which they are found
  95. Most common site of teratoma in neonates?
    In adolescents?

  96. Hepatoblastoma is associated with:
    • Hemihypertrophy
    • Very low birth weight
    • Familial adenomatous polyposis
    • Beckwith-Wiedemann syndrome
  97. Hepatocellular carcinoma associated with:
    • Perinatally acquired Hep B & C
    • Mutations in c-met
    • Tyrosinemia
    • Biliary cirrhosis
    • Alpha-1-antitrypsin deficiency
  98. Overall survival rate in hepatoblastoma:
  99. Overall survival rate for hepatocellular carcinoma:
  100. Regarding hepatoblastoma vs HCC, chemotherapy is more efdective for:
  101. Regarding hepatoblastoma vs HCC:
    - age affected:
    - focality:
    - alpha-fetoprotein levels:
    • Hepatoblastoma
    • - under 3yo
    • - unifocal
    • - alpha-fetoprotein level parallels disease activity

    • HCC
    • - children and adults
    • - multicentric and invasive
    • - alpha-fetoprotein level parallels disease activity
  102. DDX pediatric midline neck mass:
    • Thyroglossal duct cyst
    • Dermoid cyst
    • Lymphadenopathy
    • Ectopic thyroid
    • Thymic cyst
    • Ranula
  103. Most common location for thyroglossal duct cyst:
    Hyoid bone
  104. Thyroglossal duct cyst work up:
    Ultrasound and thyroid function tests to confirm thyroid tissue in normal anatomic location so patient is not rendered hypothyroid post-operatively
  105. Thyroglossal duct treatment:
    Sistrunk procedure = removal of cyst tract along with central hyoid bone
  106. Thyroglossal duct cyst recurrence rate after Sistrunk procedure:
    < 5%
  107. Best indicators of sufficient fluid intake in infants:
    • Urine output (1-2mL/kg/day)
    • Osmolarity (infant can only concentrate urine to max 700 mOsm/kg vs. 1200 in adult)
  108. Daily enteral caloric requirement for neonate:
    120 calories/kg to achieve ideal growth of 25-35 g/kg/day weight gain
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