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  1. In Thalassemia, who has to be a carrier to produce children with the disease?"
    Both parents
  2. What is Thalassemia?
    An inherited blood disorder of Hgb
  3. In a-Thalassemia the a-chains are affected. Which races does this affect?"
    Chinese Thai, African, Mediterraneans
  4. In B-Thalassemia the B-chains are affected and comes in 4 forms. Which races are affected?"
    Greeks Italians Syrians
  5. In B-Thalassemia the B-chains are affected and comes in 4 forms. Which is the most common form?"
    Thalassemia Major
  6. Blood transfusions can lead to Hemosiderosis. What is the definition of Hemosiderosis?"
    The accumulation of iron in the liver, heart and/or endocrine organs. Usually seen in patients who receive frequent transfusions (thalassemia, sickle cell, aplastic anemia, myelodysplastic syndrome)"
  7. Aplastic Anemia results in "Pancytopenia". What is the definition of Pancytopenia?"
    Depressed RBC, WBC, & Platelets
  8. Is Aplastic Anemia acquired or congenital?
  9. Hypoplastic anemia results in??
    Profound depression of RBC's but normal WBC's and platelets
  10. Is Hypoplastic anemia acquired or congenital?
  11. What is the definition of Hemophilia?
    A group of hereditary bleeding disorders that result in deficiencies of specific clotting factors
  12. What percentage of hemophilia is X-linked recessive?
  13. Hemophilia A is also called?
    Classic Hemophilia
  14. Hemophilia A is a deficiency of WHAT factor??
  15. Hemophila A accounts for what percentage of cases?
  16. Hemophilia A occurs in males at the rate of
    1 in 5000
  17. Hemophilia B is also known as WHAT disease?
    Christmas disease
  18. Hemophilia B is caused by a deficiency of WHAT factor?
  19. Hemophilia B accounts for what percentage of cases?
  20. In Hemophilia A, females may be??
  21. What is Hemarthrosis?"
    Bleeding into joint spaces of knee, ankle, elbow leading to impaired mobility
  22. Why do you want to use NSAIDS with caution in hemophilia?"
  23. DDAVP nasal spray or IV is used to increase which factor in hemophilia?
    Factor VIII (in mild hemophilia)
  24. What is the prognosis for hemophilia?
    • No cure
    • Historically most died by age 5yr
    • Now mild-moderate live near normal lives
  25. What is the definition of von Willebrand's disease?
    It is a hereditary bleeding disorder involving deficiency of von Willebrand's factor (a plasma protein which is the carrier for factor VIII)
  26. von Willebrand's factor is necessary for adhesion of WHAT??
  27. Is von willebrand's disease transmitted as an autosomal (dominant, recessive) trait?
  28. Who does von Willebrand's disease affect?
  29. The gene for von Willebrand's disease is located on WHAT chromosome??
  30. What are the S/S of von Willebrand's disease?
    • Easy bruising
    • Epistaxis
    • Gingival bleeding
    • Excessive bleeding with lacerations or surgeries Menorrhagia
  31. The most common form of childhood cancer is ???
  32. Leukemia occurs most frequently in which children?
    • Males > 1 yr old
    • Peak onset 2-5 years
  33. What is the survivability of leukemia?
    Approx. 80%
  34. Which children are at 20 times the normal risk for developing Acute lymphoid leukemia (ALL)?"
    Children with trisomy 21 (Down's syndrome)
  35. Leukemia is an unrestricted proliferation of immature ____________in the blood forming tissues of the body.
  36. Which organs are the most severely affected in leukemia?
    • Liver
    • Spleen
  37. Growth Hormone (GH) deficiency is called???
  38. GH deficiency is diminished or deficient secretion of???
    Pituitary Hormones
  39. S/S of dehydration
    • Poor skin turgor/ cool skin
    • Weight loss
    • Lethargy/Irritability
    • Tachycardia
    • Tachypnea
    • Orthostatic BP
    • Thirst
    • Dry mucous membranes
    • Decreased tears
    • Sunken fontanel
  40. What labs would you expect to see for dehydration?"
    • Electrolyte imbalances
    • CO2
    • Specific gravity (1.020-1.028 is normal)
  41. Gastroenteritis involves which organs?
    • Stomach
    • Intestines
  42. Enteritis involves which organs?
    Small Intestines
  43. Colitis involves which organs?
  44. Rotavirus is spread by which route?
  45. S/S of Rotavirus
    • Fever
    • Diarrhea Followed by vomiting
  46. E.Coli or "Shiga" toxin is what type of toxin?
  47. S/S of E. Coli
    • Cramps
    • Watery diarrhea - -Followed by bloody diarrhea
  48. What medication is used for C.diff?
  49. S/S of C.diff
    • Mild to prolonged diarrha
    • May have High fever
    • leukocytosis
    • pseudomembranous colitis
  50. Which IV fluids are used for rehydration?
    • NS or LR to replace volume
    • Saline solution with 5% dextrose in ¼ NS or ½ NS with potassium chloride for maintenance (Replace slowly)
  51. First meconium should be passed within what time frame?
    24-36 hours of life
  52. The inappropriate passage of feces (often with soiling) is called???
  53. What is Hirschsprung Disease?
    • A mechanical obstruction of the bowel from inadequate motility of the intestine caused by an absence of ganglion cells in the colon.
    • Babies are born with it, most commonly males/down's syndrome
  54. S/S of Hirschsprung Disease
    • Bilious emesis
    • Ribbonlike stools
    • Gas
    • Constipation
    • Fussiness
  55. S/S of GERD (gastroesophageal reflux) in infants
    • Spitting-up/vomiting
    • Excessive crying/irritability
    • Arching of the back/stiffening
    • Wheezing, stridor, gagging, choking
    • Hematemesis
    • Apnea/Apparent life threatening event (ALTE)
  56. Possible complications of GERD in infants are???
    • Esophagitis
    • Esophageal stricture Laryngitis
    • Recurrent PNA
    • Anemia
  57. Therapeutic management of GERD includes???
    • Thickening feeds: 1t. to 1T. rice cereal per 1- 2 oz. formula
    • Positioning upright for 1 hour after feeds
    • Medication: H2 receptor antagonists- Zantac, Pepcid: Proton pump inhibitors- Prevacid, Prilosec, Nexium, Protonix, Aciphex, Prokinetic drugs controversial- Reglan Nissen fundoplication if severe
  58. What are the two most common types of intestinal parasites in the U.S???
    • Giardiasis
    • Pinworms
  59. What is the cycle of the pinworm?
    • Eggs Are ingested or inhaled (eggs float in the air)
    • Hatch in upper intestine then female migrate out the anus to lay eggs
    • Everything is contaminated in the environment-clothes, bedding, toilet seats, door knobs
    • Main symptom: intense itching
  60. What medication is used for pinworms?
    Vermox (not for children under 2 yrs)
  61. Cleft lip and/or cleft palate occur at what point in embryonic
    Between the 3-12 week
  62. Failure of the esophagus to develop as a continuous passage during pregnancy is called???
    Esophageal Atresia with Tracheoesophageal Fistula (TEF)
  63. Constriction of pyloric sphincter with obstruction of gastric outlet is called
    Hypertrophic Pyloric Stenosis (HPS)
  64. S/S of Hypertrophic Pyloric Stenosis (HPS)
    • Projectile vomiting 30-60 minutes after feeding
    • Dehydration
    • Metabolic alkalosis
    • Growth failure
    • Olive shaped mass in the RUQ
    • May be palpated easily
  65. The pathophysiology of HPS includes
    • Hypertrophy & hyperplasia of muscle of the pyloric sphincter
    • Inflammation & edema results in complete obstruction
    • More common in FT, Caucasian infants
    • Genetic predisposition
  66. Telescoping or invagination of one portion of the intestine into another, pulling the mesentary with it is called
  67. Major symptom of Intussusception is
    • Bright red bleeding and mucus
    • Described as "red currant jelly" stool"
  68. Intussusception is diagnosed by:
  69. What is the antidote for APAP overdose?
    • Mucomyst given PO or IV
    • Tastes bad, smells of rotten eggs
  70. What is the antidote for Iron overdose?"
    Chelation therapy - Deferoxamine
  71. Pediatric urine output is
    1-2 mL/kg/hr
  72. 80% of UTI's are caused by which organism?
    E. Coli
  73. Clinical manifestations of a UTI in a neonate are:
    • Poor feeding,
    • vomiting
    • Failure to gain weight
    • Respiratory distress
    • Frequent urination
    • Screaming on urination
    • Jaundice,
    • dehydration
  74. Clinical manifestations of a UTI in infancy are:
    • Poor feeding,
    • vomiting
    • Failure to thrive
    • Excessive thirst
    • Frequent urination
    • Foul-smelling urine Pallor,
    • fever
    • Persistent diaper rash
  75. Clinical manifestations of a UTI in childhood are:
    • Poor appetite,
    • vomiting, pallor Fatigue
    • Growth failure
    • Excessive thirst
    • Enuresis, incontinence
    • Swelling of face,
    • abdominal or back pain
    • Blood in urine
  76. Nephrotic Syndrome (Nephrosis) is???
    an abnormality in the urinary stem that causes urine backup
  77. Clinical manifestations in Nephrotic Syndrome are:
    • Proteinuria
    • Hypoalbuminemia
    • Hyperlipidemia
    • Edema
    • Massive urinary protein loss
  78. Common S/S of Nephrotic Syndrome are:
    • Weight gain over that expected from usual pattern
    • Puffiness of face
    • Parent observation that child’s clothes fit tightly
    • Decreased urine output
    • Frothy urine
    • Pallor, fatigue
    • Generalized edema
    • Irritability
  79. Smoky, tea colored urine occurs in
  80. The most common malignant renal and intraabdominal tumor of childhood is
    Wilms Tumor
  81. Wilms Tumor is three times more common in which race?
    African Americans, especially boys
  82. Wilms Tumor is most commonly seen in which kidney?
  83. TX of Wilms Tumor includes???
    • Surgical removal of the affected kidney
    • Chemotherapy and/or radiation
  84. What is Uremic Frost?
    deposits of urea crystals on the skin
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