Pediatrics Must-Knows Flashcards

1
Q

Adenohypophysis arises from

Neurohypophysis arises from

A

Rathke’s pouch; neural ectoderm or nervous tissue

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2
Q

Most common type of tumor areas in the Rathke’s pouch in children/adult

A

Craniopharyngoma; prolactinoma

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3
Q

Other name of ADH

A

Vasopressin or Arginine VP (AVP)

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4
Q

Definitive diagnosis of hypopituitarism

A

Absent or low levels of GH

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5
Q

Vasopressin deficiency?

A

Central DI

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6
Q

Vasopressin insensitive kidneys?

A

Nephrogenic DI

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7
Q

Drug that causes Nephrogenic DI

A

Lithium

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8
Q

Diabetes Insipidus is manifested by polyuria (__cc/kg/hr) and polydipsia, _______mOsm, spgr _______

A

5cc/kg/hr; 50-200 mOsm; spgr 1.001-1.005

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9
Q

Electrolyte imbalance for DI

A

Hypernatremia and Hyperchloremia

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10
Q

Etiology of DI for 50% of cases

Other causes:

A
Brain tumor
Idiopathic
Head trauma
CNs infection
Histiocytosis
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11
Q

Precocious puberty is defined as premature appearance of secondary sexual characteristics
Breast bud before __y/o in females
Testicular enlargement ___y/o in males

A

8; 9

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12
Q

Most common cause of gonadotropin dependent precocious puberty?

A

Hypothalamic hamartomas

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13
Q

Triad of McCune Albright syndrome

A

Precocious puberty, polyostotic fibrous dysplasia, unilateral café-au-lait spots

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14
Q

Treatment for precocious puberty

A

Leuprolide acetate

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15
Q

Delayed puberty is the absence of any sign of puberty.
Boys?
Girls?

A

14 y/o

13 y/o

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16
Q

Structures that arise from mullerian duct

A

FT, prostatic utricle, uterus, cervix, vagina

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17
Q

Homologues of prostatic utricle

A

Uterus, cervix, vagina

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18
Q

Structures that arise from Wolffian duct

A

Rete testis, rete ovarii, epididymis, gartner’s duct, bas deferens, seminal vesicle

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19
Q

Congenital absence of uterus and vagina

A

Rokitansky-Meyer-Kuster-Hauser syndrome

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20
Q

Deficiency in CAH

A

21-hydroxylase

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21
Q

Deficiency in CAH tested in the Philippines

A

17-hydroxylase

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22
Q

Most common manifestation of CAH

A

Ambiguous genitalia

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23
Q

Treatment of CAH

A

Cortisol

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24
Q

CAH pathogenesis

A

NB at risk for neonatal adrenal crisis due to sodium loss due to absence of aldosterone

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25
Q

Most common cause of genital ambiguity

A

CAH

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26
Q

Most common cause of congenital hypothyroidism

A

Thyroid dysgenesis (85%)

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27
Q

Most common location of ectopic thyroid tissue

A

Lingual pr base of the tongue

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28
Q

Pathogenesis of Congenital Hypothyroidism

A

Inborn of errors of thyroxine synthesis and maternal transfer of TRBAb

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29
Q

Manifestations of Congenital Hypothyroidism

A

Prolonged jaundice, large tongue (due to ectopic thyroid tissue, MR, umbilical hernia, sluggisu

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30
Q

Syndrome that is characterized with intractable neonatal hypoglycemia, macrosomia, midline abdominal wall defects (omphalocele, umbilical hernia, diastasis recti), facial nevus flammeus, a characteristic earlobe creases (5 common features)

A

Beckwith-Wiedemann Syndrome

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31
Q

3 embryonal tumors in BWS

A

Wilms tumor, hepatoblastoma, rhabdomyosarcoma

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32
Q

Most common cause of thyroid disease in children

A

Thyroiditis

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33
Q

Most common cause of acquired hypothyroidism

A

Thyroiditis

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34
Q

Other names of thyroiditis

A

Lymphocytic thyroiditis, hashimoto’s thyroiditis, autoimmune thyroiditis

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35
Q

Thyroiditis with diffuse fibrosis that makes thyroid hard and woody apperance

A

Reidel’s thyroiditis

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36
Q

Other names of subacute thyroiditis

A

Viral thyroiditis, De Quervain thyroiditis

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37
Q

Catecholamine-secreting tumor of the chromaffin cells

A

Pheochromocytoma

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38
Q

Metabolite of pheochromocytoma

A

Urine VMA

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39
Q

DOC of pheochromocytoma

A

Phenoxybenzamine

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40
Q

Adrenal medulla tumors in children will be most likely be

A

Neuroblastoma

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41
Q

Locations of neuroblastoma

A

Abdomen, either from adrenals or retroperitoneal ganglia

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42
Q

Histopathological feature of neuroblastoma

A

Homer-Wright pseudorosettes

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43
Q

Retinoblastoma features

A

Cat’s eye
Leukocoria
Flexner-Wintersteiner bodies/rossettes

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44
Q

Components of Triple screen test

A

AFP, beta HCG, estriol

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45
Q

Other names of Triple screen test

A

Kettering or Bart’s test

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46
Q

Basic defect of PKU

A

Deficiency of phenylalanine hydroxylase or its cofactor tetrahydrobiopterin (BH4)

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47
Q

Cofactor that required to convert phenylalanine to tyrosine

A

Tetrahydrobiopterin

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48
Q

Physical examination findings of PKU

A

Seborrheic or eczematoid rash, mousy or musty odor

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49
Q

Histopathologic findings of G6PD deficiency

A

Heinz bodies, reticulocytosis

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50
Q

Enzyme that is responsible for decarboxylation of leucine, isoleucine and valine

A

Alpha ketoacid dehydrogenase

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51
Q

Inborn error of metabolism that has deficiency in the branched chain alpha ketoacid dehydrogenase

A

MSUD

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52
Q

Purely ketogenic amino acid

A

Leucine and isoleucine

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53
Q

Odor of MSUD

A

Caramel odor

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54
Q

Physical examination findings of MSUD

A

Hypertonicity, muscular rigidity and opisthotonus

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55
Q

Syndrome deficient of 7-dehydrocholesterol reductase deficiency characterized by pre- and postnatal growth retardation, MR, facial dysmorphic features, hypospadia, syndactyly between toes 2

A

Smith-Lemli-Opitz syndrome

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56
Q

Deficiency of hexosaminidase A deficiency, GM2 gangliosidosis characterized with cherry red spot with optic atrophy

A

Tay-Sachs Disease

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57
Q

Deficiency of both hexosaminidase A and B characterized by moderate hepatosplenomegaly and coarse granulations in BM histiocytes

A

Sandhoff disease

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58
Q

Deficiency in glucocerebrosidase characterized by hypotonia, crumpled tissue appearance

A

Infantile Gautcher disease

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59
Q

Disease with characteristic histiocytes (Gaucher cells) in marrow smears and liver and spleen biopsies

A

Infantile Gaucher Disease

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60
Q

Deficiency in Sphingomyelinase characterized by marked enlargement of liver with histopathologic finding of vacuolated histiocytes in the BM

A

Infantile Niemann-Pick Disease

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61
Q

Deficiency of ceramidase characterized by hoarse cry due to fixation of laryngeal cartilage with histopathologic finding of periarticular and subcutaneous swellings and progressive arthropathy

A

Faber disease or Lipogranulomatosis

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62
Q

Peroxisomal disorder in abnormal very long chain fatty acid metabolism manifests at birth, IUGR, developmental delay, lim contractuee, hepatomegalu, floppy baby, abnormal facies

A

Cerebrohepatorenal (Zellweger) disease

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63
Q

Enzyme deficient in Zellweger disease

A

Pipecolote oxidase

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64
Q

Deficiencies of phytanoyl-CoA hydroxyladse (chromosome 10) or peroxin-7 (chromosome 6) characterized by neurologic damage, cerebellar degeneration, and peripheral neuropathy

Think of Referee from fighting

A

Refsum disease

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65
Q

Autosomal recessive disease characterized by intermittent red, scaly rash over the face, neck, hands and legs resembling that of pellagra (niacin deficiency) and cerebellar ataxia

A

Hartnup disease

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66
Q

X-linked recessive disorder deficient in Cu-dependent enzymes, characterized by pili torti

A

Menkes disease (Kinky- or Steeply hair disease; trichopoliodystrophy)

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67
Q

Hepatolenticular degeneration due to decreased levels of ceruloplasmin

A

Wilson’s disease

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68
Q

3 organs that accumulate copper in Wilson’s disease

A

Cornea (Kayser-Fleishner rings)
Liver (cirrhosis)
Brain (in basal ganglia)

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69
Q

ABCD of Wilson’s disease

A

Asterixis
Basal ganglia ceruloplasmin
Ceruloplasmin decreased, cirrhosis, corneal deposits), Cu accumulation, carcinoma, chorea
Dementia

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70
Q

Most common cause of Charcot-Marie tooth in 70-80% of cases

A

Duplication of a large region on the short arm of chromosome 16 that includes PMP22 (Peripheral Myelin)

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71
Q

Manifestations of Charcot-Marie tooth disease

A
Peroneal muscular atrophy
Foot drop
High arched foot
Stork legs
Hammer toes
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72
Q

Vasculitis of small vessels with IgA and C3 deposition in small vessels of the skin and glomeruli typically URTI

A

HSP or Anaphylactoid purpura

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73
Q

Hallmark finding of HSP

A

Palpable purpura

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74
Q

Biopsy result of HSP

A

Leukoclastic angiitis

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75
Q

Most common vasculitis in children

A

HSP

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76
Q

Cardiac complication of Kawasaki disease

A

Coronary artery aneurysm

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77
Q

Other name of Kawasaki disease

A

Febrile mucocutaneous lymphadenopathy

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78
Q

Pulseless disease, vasculitis of large vessels, chronic, inflammatory infiltrate, preference for aorta and its branches, pulseless and claudication

A

Takayasu arteritis

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79
Q

Necrotizing vasculitis of small and medium vessels, bx: fibrinoid necrosis with thrombosis and infarction, ssx of HPN, hematuria, purpura, CVA, TIA, myocarditis, bone pain and blindness

A

Panarteritis or polyarteritis nodosa

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80
Q

Classic presentation of TEF

A

Choking, coughing, cyanosis occurs with an attempt at feeding

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81
Q

VACTERL in TEF

A

Vertebral, anal anomalies, cardiac, tracheo-esophageal fistula, renal anomalies, limb anomalies

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82
Q

2 other treatment aside from surgery for Achalasia

A

Nifedipine

Botulinum toxin

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83
Q

Surgical procedure for the treatment of achalasia

A

Heller myotomy

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84
Q

Radiologic finding of Hypertrophic pyloric stenosis

A

Shoulder sign, double tract sign, string sign

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85
Q

Surgical procedure for Hypertrophic pyloric stenosis

A

Ramstedt pyloromyotomy

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86
Q

Triad of volvulus

A

Severe epigastic pain
Intractable retching with emesis
Inability to pass a tube into the stomach

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87
Q

Duodenal atresia is associated with:

A

Down syndrome

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88
Q

Most common cause of intestinal obstruction between 3 months and 6 years of age

A

Intussuseption

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89
Q

2 types of intussuseption

A

Ileocolic or ileocecal

Ileoileocolic

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90
Q

Most common type of intussuseption

A

Ileocolic or ileocecal

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91
Q

Investigation of the RLQ for retraction which can be an indication of intussuseption

A

Dance’s sign

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92
Q

Radiologic finding of intussuseption

A

Coiled spring sign

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93
Q

Condition with prolonged elevated levels of conjugated bilirubin after 14th DOL

A

Neonatal cholestasis

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94
Q

Surgical procedure for biliary atresia

A

Kasai or hepatoportoenterostomy performed before 8 weeks

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95
Q

Emergency medical condition primarily seen in premature infants where portions of the bowel undergo necrosis

A

Necrotizing enterocolitis (NEC)

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96
Q

Radiologic finding of NEC

A

Pneumatosis intestinalis

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97
Q

Most common sites of NEC

A

Distal ileum and proximal colon

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98
Q

Physiologic jaundice presents ________

Resolves in ____week (term), ____weeks (preterm)

A

After 48th hour of life
1 week
2 weeks

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99
Q

Pathologic jaundice presents ________

Persists beyond in ____week (term), ____weeks (preterm)

A

1st 24 hours of life
1 week
2 weeks

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100
Q

Breastfeeding jaundice occurs at ____DOL
Accentuated ___________________
Factors: ______________
Duration: ______

A

3-4 DOL
Unconjugated hyperbilirubinemia
Decreased milk intake with dehydration
Few days

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101
Q
Breast milk jaundice occurs at \_\_\_\_DOL
Accentuated \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
As high as \_\_\_\_mg/dL during the 2nd-3rd week
Factors: \_\_\_\_\_\_\_\_\_\_\_\_\_\_
Duration: \_\_\_\_\_\_
A

After 7th DOL
Increased B1 in 2% of breastfed term infants
10-30
Presence of glucuronidase in some breast milk
3 weeks to 3 months

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102
Q

Level of bilirubin for icteric sclerae

A

2-3 mg/dL

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103
Q

Level of bilirubin requiring phototheraphy

A

15 mg/dL

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104
Q

Level of bilirubin in kernicterus or bilirubin encephalopathy

A

20mg/dL

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105
Q

Early signs of kernicterus (3-4days)

A

Lethargy
Poor feeding
High pitched cry
Hypotonia

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106
Q

Late signs of kernicterus (>1week)

A
Irritability
Seizure
Apnea
Hypertonia
Fever
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107
Q

Mechanisms of phototherapy

A

Geometric photoisomerization of conjugated bilirubin
Structural isomerization: lumirubin
Photooxidation

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108
Q

Chronic signs of kernicterus (3 years of age)

A
Athetpid cerebral palsy
High frequency hearing loss
Paralysis of upward gaze
Dental dysplasia
Mild MR
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109
Q

Pharmacologic therapy for kernicterus

A

Phenobarbital: induction of glucoronyl transferase activity, enhances hepatic uptake
Metalloporphyrins: inhibits heme oxygenase

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110
Q

If phototherapy and pharmacologic treatment does not work

A

Exchange transfusion

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111
Q

Most common congenital anomaly of the respiratory tract

A

Choanal atersia

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112
Q

Most common type of chonal atresia

A

Bony (90%)

Membranous (10%)

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113
Q

Most common manifestation of choanal atresia

Others

A
  1. Nasal obstruction (if unilateral, asymptomatic)
  2. Cyanosis improved with crying if bilareral nasal obstruction
  3. Inability to insert NGT
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114
Q

CHARGE syndrome is _________

A

Coloboma
Heart disease
Atresia choanae
Retarded growth and development or CNS anomalies
Genital anomalies or hypogonadism or both
Ear anomalies or deadness or both

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115
Q

Most common congenital laryngeal anomaly

A

Laryngomalacia

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116
Q

Most frequent cause of strudor in infants and children

A

Larnyngomalacia

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117
Q

2nd most common cause of stridor

A

Congenital subglottic stenosis

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118
Q

Congenital anomaly of the larynx characterized by biohadic stridor, recurrent or persistent croup

A

Congenital subglottic stridor

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119
Q

Most frequent cause of airway obstruction requiring trachepstomy in infants

A

Larngotracheal stenosis

120
Q

Acquired laryngotracheal stenosis is related to ___________

A

ET intubation

121
Q

The most common respiratory problem of a premature infant

A

Hyaline membrane disease

122
Q

DOC that decreasea the incidence and severity of HMD

A

Corticosteroid

123
Q

When the transient phenomenon of TTN lasts?

A

6-24 hours

124
Q

Radiologic finding of TTN

A

Hyperaeration, prominent vascular markings (sunburst pattern), prominent minor fissure

125
Q

AVP acts at what part of the kidney

A

Distal tubules

126
Q

Most common etiology of rhinosinusitis

A

Rhinovirus

127
Q

Most common complication of rhinosinusitis

A

Otitis media

128
Q

Enzyme for conversion of testosterone to DHT

A

Alpha reductase

129
Q

Peak incidence of acute pharyngitis

A

4-7 years old

130
Q

Most common cause of acute pharyngitis

A

Viral

131
Q

Bacterial causes of acute pharyngitis

A

Group A beta hemolytic strep (strep pyogenes)
Mycoplasma
Arcanobacterium haemolyticum

132
Q

Reapiratory condition that presenta as sore theoat and fever worse at nigr, seal-like cough, copl night air usually helps

A

Croup or laryngotracheobronchitis

133
Q

Condition that involves prtial teatis determination in the presence of a Y chromosome

A

Incomplete gonadal dysgenesis

134
Q

Etiologic agent of croup

A

Parainfluenza

135
Q

Radiologic sign of croup

A

Steeple sign or subglottic narrowing

136
Q

Condition seen as dysmorphic syndrome that has defect in testosterone biosynthesis

A

Male pseudohermaphroditism

137
Q

Radiologic finding of epiglotitis

A

Thumb print sign

138
Q

What is Wolfram syndrome?

A

DI, DM, optic atropht, deafness

139
Q

Most common cause of bacterial tracheitis

A

S. Aureus

140
Q

Etiologic agent for pneumatocele pneumonia

A

S. Aureus

141
Q

Common disease of the LRT of infants; etiologic agent RSV

A

Bronchiolitis

142
Q

Most common hematologic disease of infancy in childhood

A

IDA

143
Q

When should exclusively breastfed infants must be supplemented with Iron?

A

4 months

144
Q

What diagnostic test should be measured for idiopathic or unexplained DI in addition to serial MRI scan?

A

Beta HCG

145
Q

Test for Vitamin B12 absorption

A

Schilling test

146
Q

What vitamin has excessive methymalonic acid in the urine?

A

Vitamin B12/cobalamin

147
Q

Most common familial and congenital abnormality of the red cell membrane

A

Hereditary spherocytosis

148
Q

Hematologic disease that has defect on the cytoskeleton protein, specifically ankyrin and spectrin

A

Hereditary spherocytosis

149
Q

Test for hereditary spherocytosis

A

Osmotic fragility test

150
Q

Blood coagulation derangement in Factor VIII

A

Increased PTT; normal BT, PT

151
Q

Painful bump below the knee is the manifestation of what condition

A

Osgood Schlatter

152
Q

Blood coagulation derangement in Factor IX

A

Increased PTT; normal BT, PT

153
Q

Cause of idiopathic central DI in 50% of cases

A

Hypophysitis

154
Q

Most common hereditary bleeding disordee

A

VWF disease

155
Q

Blood coagulation derangement in VWF disease

A

Prolonged BT and PTT

156
Q

Hematologic condition with deficiency in Vitamin K, 1972, more common in breastfed infants due to lack of free K, absence of bacteria responsible for Vitamin K synthesis in the intestines

A

Hemorrhagic disease in the newborn

157
Q

Drugs associated with the inhibition of vasopressin release

A
Halothane
Opiate antagonist
Phenytoin
Ethanol
Alpha-adrenergic agent
158
Q

Mainstay treatment for DI

A

Fluid therapy

159
Q

Total fluid intake is limited to how much

A

1L/m2/24 hours

160
Q

Most common childhood CA

A

Leukemia

161
Q

Most common leukemia as seen also in Down Syndrome, ataxia-telangiectasia, fanconinsyndrome, NF type

A

ALL (77%)

162
Q

Peak incidence of ALL

A

4 years old

163
Q

Environmental factors associated with ALL

A

Ionizing radiation
Drugs
Alkylating agents
Benzene

164
Q

Medical treatment for ALL

A

Vincristine, dexamethasone or prednisone, L-asparaginase

165
Q

Drugs associated with acquired nephrogenic diabetes insipidus

A
Lithium
Demeclocycline
Foscarnet
Clozapine
Amphotericin
Methicillin
Rifampin
166
Q

Remission of ALL is defined as

A

<5% blast in marrow and return of neutrophil and platelet count to near normal

167
Q

Leukemia predominant neonatal/congenital leukemia

A

AML

168
Q

Diagnostic finding for AML

A

Myeloperoxidase and Auer rods

169
Q

Higher incidence of conditions in AML

A

Down syndrome, fanconi anemia, diamond-blackfan anemia

170
Q

Leukemia uncommon in children with only 3% under the age of 20; more common in 55-65years

A

CML

171
Q

Pathogenesis of CML

A

t (9,22) philadelphia chromosome

172
Q

Pathogenesis of Burkitt lymphoma

A

t (8,14)

173
Q

Most common congenital anomaly of the respiratory tract

A

Choanal atersia

174
Q

Most common type of chonal atresia

A

Bony (90%)

Membranous (10%)

175
Q

Most common manifestation of choanal atresia

Others

A
  1. Nasal obstruction (if unilateral, asymptomatic)
  2. Cyanosis improved with crying if bilareral nasal obstruction
  3. Inability to insert NGT
176
Q

CHARGE syndrome is _________

A

Coloboma
Heart disease
Atresia choanae
Retarded growth and development or CNS anomalies
Genital anomalies or hypogonadism or both
Ear anomalies or deadness or both

177
Q

Most common congenital laryngeal anomaly

A

Laryngomalacia

178
Q

Most frequent cause of strudor in infants and children

A

Larnyngomalacia

179
Q

2nd most common cause of stridor

A

Congenital subglottic stenosis

180
Q

Congenital anomaly of the larynx characterized by biohadic stridor, recurrent or persistent croup

A

Congenital subglottic stridor

181
Q

Most frequent cause of airway obstruction requiring trachepstomy in infants

A

Larngotracheal stenosis

182
Q

Acquired laryngotracheal stenosis is related to ___________

A

ET intubation

183
Q

The most common respiratory problem of a premature infant

A

Hyaline membrane disease

184
Q

DOC that decreasea the incidence and severity of HMD

A

Corticosteroid

185
Q

When the transient phenomenon of TTN lasts?

A

6-24 hours

186
Q

Radiologic finding of TTN

A

Hyperaeration, prominent vascular markings (sunburst pattern), prominent minor fissure

187
Q

AVP acts at what part of the kidney

A

Distal tubules

188
Q

Most common etiology of rhinosinusitis

A

Rhinovirus

189
Q

Most common complication of rhinosinusitis

A

Otitis media

190
Q

Enzyme for conversion of testosterone to DHT

A

Alpha reductase

191
Q

Peak incidence of acute pharyngitis

A

4-7 years old

192
Q

Most common cause of acute pharyngitis

A

Viral

193
Q

Bacterial causes of acute pharyngitis

A

Group A beta hemolytic strep (strep pyogenes)
Mycoplasma
Arcanobacterium haemolyticum

194
Q

Reapiratory condition that presenta as sore theoat and fever worse at nigr, seal-like cough, copl night air usually helps

A

Croup or laryngotracheobronchitis

195
Q

Condition that involves prtial teatis determination in the presence of a Y chromosome

A

Incomplete gonadal dysgenesis

196
Q

Etiologic agent of croup

A

Parainfluenza

197
Q

Radiologic sign of croup

A

Steeple sign or subglottic narrowing

198
Q

Condition seen as dysmorphic syndrome that has defect in testosterone biosynthesis

A

Male pseudohermaphroditism

199
Q

Radiologic finding of epiglotitis

A

Thumb print sign

200
Q

What is Wolfram syndrome?

A

DI, DM, optic atropht, deafness

201
Q

Most common cause of bacterial tracheitis

A

S. Aureus

202
Q

Etiologic agent for pneumatocele pneumonia

A

S. Aureus

203
Q

Common disease of the LRT of infants; etiologic agent RSV

A

Bronchiolitis

204
Q

Most common hematologic disease of infancy in childhood

A

IDA

205
Q

When should exclusively breastfed infants must be supplemented with Iron?

A

4 months

206
Q

What diagnostic test should be measured for idiopathic or unexplained DI in addition to serial MRI scan?

A

Beta HCG

207
Q

Test for Vitamin B12 absorption

A

Schilling test

208
Q

What vitamin has excessive methymalonic acid in the urine?

A

Vitamin B12/cobalamin

209
Q

Most common familial and congenital abnormality of the red cell membrane

A

Hereditary spherocytosis

210
Q

Hematologic disease that has defect on the cytoskeleton protein, specifically ankyrin and spectrin

A

Hereditary spherocytosis

211
Q

Test for hereditary spherocytosis

A

Osmotic fragility test

212
Q

Blood coagulation derangement in Factor VIII

A

Increased PTT; normal BT, PT

213
Q

Painful bump below the knee is the manifestation of what condition

A

Osgood Schlatter

214
Q

Blood coagulation derangement in Factor IX

A

Increased PTT; normal BT, PT

215
Q

Cause of idiopathic central DI in 50% of cases

A

Hypophysitis

216
Q

Most common hereditary bleeding disordee

A

VWF disease

217
Q

Blood coagulation derangement in VWF disease

A

Prolonged BT and PTT

218
Q

Hematologic condition with deficiency in Vitamin K, 1972, more common in breastfed infants due to lack of free K, absence of bacteria responsible for Vitamin K synthesis in the intestines

A

Hemorrhagic disease in the newborn

219
Q

Drugs associated with the inhibition of vasopressin release

A
Halothane
Opiate antagonist
Phenytoin
Ethanol
Alpha-adrenergic agent
220
Q

Mainstay treatment for DI

A

Fluid therapy

221
Q

Total fluid intake is limited to how much

A

1L/m2/24 hours

222
Q

Most common childhood CA

A

Leukemia

223
Q

Most common leukemia as seen also in Down Syndrome, ataxia-telangiectasia, fanconinsyndrome, NF type

A

ALL (77%)

224
Q

Peak incidence of ALL

A

4 years old

225
Q

Environmental factors associated with ALL

A

Ionizing radiation
Drugs
Alkylating agents
Benzene

226
Q

Medical treatment for ALL

A

Vincristine, dexamethasone or prednisone, L-asparaginase

227
Q

Drugs associated with acquired nephrogenic diabetes insipidus

A
Lithium
Demeclocycline
Foscarnet
Clozapine
Amphotericin
Methicillin
Rifampin
228
Q

Remission of ALL is defined as

A

<5% blast in marrow and return of neutrophil and platelet count to near normal

229
Q

Leukemia predominant neonatal/congenital leukemia

A

AML

230
Q

Diagnostic finding for AML

A

Myeloperoxidase and Auer rods

231
Q

Higher incidence of conditions in AML

A

Down syndrome, fanconi anemia, diamond-blackfan anemia

232
Q

Leukemia uncommon in children with only 3% under the age of 20; more common in 55-65years

A

CML

233
Q

Pathogenesis of CML

A

t (9,22) philadelphia chromosome

234
Q

Pathogenesis of Burkitt lymphoma

A

t (8,14)

235
Q

Most common congenital anomaly of the respiratory tract

A

Choanal atersia

236
Q

Most common type of chonal atresia

A

Bony (90%)

Membranous (10%)

237
Q

Most common manifestation of choanal atresia

Others

A
  1. Nasal obstruction (if unilateral, asymptomatic)
  2. Cyanosis improved with crying if bilareral nasal obstruction
  3. Inability to insert NGT
238
Q

CHARGE syndrome is _________

A

Coloboma
Heart disease
Atresia choanae
Retarded growth and development or CNS anomalies
Genital anomalies or hypogonadism or both
Ear anomalies or deadness or both

239
Q

Most common congenital laryngeal anomaly

A

Laryngomalacia

240
Q

Most frequent cause of strudor in infants and children

A

Larnyngomalacia

241
Q

2nd most common cause of stridor

A

Congenital subglottic stenosis

242
Q

Congenital anomaly of the larynx characterized by biohadic stridor, recurrent or persistent croup

A

Congenital subglottic stridor

243
Q

Most frequent cause of airway obstruction requiring trachepstomy in infants

A

Larngotracheal stenosis

244
Q

Acquired laryngotracheal stenosis is related to ___________

A

ET intubation

245
Q

The most common respiratory problem of a premature infant

A

Hyaline membrane disease

246
Q

DOC that decreasea the incidence and severity of HMD

A

Corticosteroid

247
Q

When the transient phenomenon of TTN lasts?

A

6-24 hours

248
Q

Radiologic finding of TTN

A

Hyperaeration, prominent vascular markings (sunburst pattern), prominent minor fissure

249
Q

AVP acts at what part of the kidney

A

Distal tubules

250
Q

Most common etiology of rhinosinusitis

A

Rhinovirus

251
Q

Most common complication of rhinosinusitis

A

Otitis media

252
Q

Enzyme for conversion of testosterone to DHT

A

Alpha reductase

253
Q

Peak incidence of acute pharyngitis

A

4-7 years old

254
Q

Most common cause of acute pharyngitis

A

Viral

255
Q

Bacterial causes of acute pharyngitis

A

Group A beta hemolytic strep (strep pyogenes)
Mycoplasma
Arcanobacterium haemolyticum

256
Q

Reapiratory condition that presenta as sore theoat and fever worse at nigr, seal-like cough, copl night air usually helps

A

Croup or laryngotracheobronchitis

257
Q

Condition that involves prtial teatis determination in the presence of a Y chromosome

A

Incomplete gonadal dysgenesis

258
Q

Etiologic agent of croup

A

Parainfluenza

259
Q

Radiologic sign of croup

A

Steeple sign or subglottic narrowing

260
Q

Condition seen as dysmorphic syndrome that has defect in testosterone biosynthesis

A

Male pseudohermaphroditism

261
Q

Radiologic finding of epiglotitis

A

Thumb print sign

262
Q

What is Wolfram syndrome?

A

DI, DM, optic atropht, deafness

263
Q

Most common cause of bacterial tracheitis

A

S. Aureus

264
Q

Etiologic agent for pneumatocele pneumonia

A

S. Aureus

265
Q

Common disease of the LRT of infants; etiologic agent RSV

A

Bronchiolitis

266
Q

Most common hematologic disease of infancy in childhood

A

IDA

267
Q

When should exclusively breastfed infants must be supplemented with Iron?

A

4 months

268
Q

What diagnostic test should be measured for idiopathic or unexplained DI in addition to serial MRI scan?

A

Beta HCG

269
Q

Test for Vitamin B12 absorption

A

Schilling test

270
Q

What vitamin has excessive methymalonic acid in the urine?

A

Vitamin B12/cobalamin

271
Q

Most common familial and congenital abnormality of the red cell membrane

A

Hereditary spherocytosis

272
Q

Hematologic disease that has defect on the cytoskeleton protein, specifically ankyrin and spectrin

A

Hereditary spherocytosis

273
Q

Test for hereditary spherocytosis

A

Osmotic fragility test

274
Q

Blood coagulation derangement in Factor VIII

A

Increased PTT; normal BT, PT

275
Q

Painful bump below the knee is the manifestation of what condition

A

Osgood Schlatter

276
Q

Blood coagulation derangement in Factor IX

A

Increased PTT; normal BT, PT

277
Q

Cause of idiopathic central DI in 50% of cases

A

Hypophysitis

278
Q

Most common hereditary bleeding disordee

A

VWF disease

279
Q

Blood coagulation derangement in VWF disease

A

Prolonged BT and PTT

280
Q

Hematologic condition with deficiency in Vitamin K, 1972, more common in breastfed infants due to lack of free K, absence of bacteria responsible for Vitamin K synthesis in the intestines

A

Hemorrhagic disease in the newborn

281
Q

Drugs associated with the inhibition of vasopressin release

A
Halothane
Opiate antagonist
Phenytoin
Ethanol
Alpha-adrenergic agent
282
Q

Mainstay treatment for DI

A

Fluid therapy

283
Q

Total fluid intake is limited to how much

A

1L/m2/24 hours

284
Q

Most common childhood CA

A

Leukemia

285
Q

Most common leukemia as seen also in Down Syndrome, ataxia-telangiectasia, fanconinsyndrome, NF type

A

ALL (77%)

286
Q

Peak incidence of ALL

A

4 years old

287
Q

Environmental factors associated with ALL

A

Ionizing radiation
Drugs
Alkylating agents
Benzene

288
Q

Medical treatment for ALL

A

Vincristine, dexamethasone or prednisone, L-asparaginase

289
Q

Drugs associated with acquired nephrogenic diabetes insipidus

A
Lithium
Demeclocycline
Foscarnet
Clozapine
Amphotericin
Methicillin
Rifampin
290
Q

Remission of ALL is defined as

A

<5% blast in marrow and return of neutrophil and platelet count to near normal

291
Q

Leukemia predominant neonatal/congenital leukemia

A

AML

292
Q

Diagnostic finding for AML

A

Myeloperoxidase and Auer rods

293
Q

Higher incidence of conditions in AML

A

Down syndrome, fanconi anemia, diamond-blackfan anemia

294
Q

Leukemia uncommon in children with only 3% under the age of 20; more common in 55-65years

A

CML

295
Q

Pathogenesis of CML

A

t (9,22) philadelphia chromosome

296
Q

Pathogenesis of Burkitt lymphoma

A

t (8,14)