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Pediatrics Flashcards

1
Q

Most common heart defect in Edwards syndrome (trisomy 18)

A

VSD

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2
Q

Cya optic baby with decrease pulm markings on CXR and left axis deviation on EKG

A

Tricuspid atresia. Normal babies have a right axis deviation because right heart is larger than left heart in new born

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3
Q

Gastroschisis vs omphacele associated defects

A

Gastroschisis is usually an isolated defect lateral to the umbilicus with uncovered hernia ting bowel. It requires immediate surgical closure in single stage closure.
Other malformations such as cardiac defects, neural tube defects etc occur in about half do patients with omphalocele. Patients require surgical closure in stage closure with silastic silo

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4
Q

Treatment for pin worm (enterobius vermicularis)

A

Albendazole or pyrantel pamoate

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5
Q

Cyanotic heart defects

Which presents with severe cyanosis a few hours after birth.

A

The five Ts that have right to left shunts

  1. Truncus arteriosus = 1 arterial vessel overriding ventricles
  2. Transposition of the great vessels = 2 arteries switched
  3. Tricuspid atresia = 3
  4. Tetralogy of Fallot = 4
  5. Total anomalous pulmonary venous return = 5 words

Transposition of the great vessels is the only one the presents a few hours after birth

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6
Q

Noncyanotic heart defects

A

The three D’s

VSD, ASD, PDA

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7
Q

Common conditions associated with the VSD

A

VSD is the most common cause of congenital heart disease.
Apert’s syndrome ( cranial deformities, fusion fingers and toes)
Down syndrome, fetal alcohol syndrome, TORCH syndrome, cri du chat syndrome and trisomy 13 and 18

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8
Q

Murmur of VSD

A

Holosystolic murmur that can be accompanied by systolic thrill crackle hepatomegaly and a narrow S2 with an increased T2 and a mild diastolic apical rambling reflecting increase flow across the mitral valve

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9
Q

ASD’s Ostium premium versus ostium secundum

A

Ostium premium = early childhood; murmur or fatigue with exertion; associated with down syndrome.
Ostium secundum = late childhood or early adulthood

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10
Q

Holt Oram syndrome

A

Absent radii, ASD, first degree heart block. remember this association with ASD

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11
Q

When is surgical closure of ASD indicated

A

90% of defects close spontaneously. Surgical or Catheter closures indicated in infants with CHF and in patients with more than 2:1 ratio of pulmonary to systemic blood flow

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12
Q

What heart defects can present with recurrent respiratory infections

A

VSD, AST, PDA

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13
Q

PDA cardiac exam

A

Continuous machinery murmur at the second left intercostal space at the sternal border. A loud S2, wide pulse pressure, and bounding peripheral pulses

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14
Q

PDA closure

A

Give indomethacin.
come in and close the door

Keep patent with prostaglandin E1

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15
Q

Coarctation of the aorta location

A

Occurs just below the left subclavian artery in 98% of patients

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16
Q

Coarctation facts

A
  1. Associated with turner syndrome
  2. Coarctation requires a patent PDA for survival.
  3. Can present as a shock likr state when the PDA closes a few weeks after life.
  4. Can’t present with lower extremity claudication, syncope, epistaxes, and headache
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17
Q

Risk factors for transposition of the great vessels

A

Diabetic mothers and rarely DiGeorge syndrome

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18
Q

Most common cyanotic heart disease

A

Transposition of the great vessels is the most common cyanotic heart disease of newborns
Tetralogy of Fallot is the most common cyanotic heart disease of childhood

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19
Q

Tetralogy of Fallot cardiac exam

A

Systolic ejection murmur at the left upper sternal border (right ventricular outflow obstruction), right ventricular heave, and a single S2

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20
Q

What are tet spells and what our treatments

A

Acute cyanosis seen in children with tetralogy of Fallot.
Improves of placing knees to chest
Medical treatment involves oxygen, propranolol, phenylephrine (increases after load), fluids and morphine

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21
Q

Infant growth

A

Newborns can lose 5 to 10% of body weight in the first week but we did buy the second week
Infants are expected to double their birth weight by 4 to 5 months triple by one year and quadruple by two years

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22
Q

Disorders associated with down syndrome

A

Duodenal atresia, Hirschsprung’s disease and congenital heart disease in infancy.
Increases the risk of acute lymphocytic leukemia (ALL)hypothyroidism, early onset Alzheimer’s

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23
Q

Most common congenital cardiac disease in down syndrome

A

AV Canal malformation

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24
Q

Rocker bottom feet, low-set ears, micrognathia, clenched hands (overlapping fourth and fifth digits close), and a prominent occiput

A

Edwards syndrome trisomy 18 (election)

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25
Q

Microcephaly, cleft lip/palate, holoprosencephaly, punched out scalp lesions, polydactyly, Omphacele. Microphthalmia

A

Pataus syndrome

Focus on the P’s

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26
Q

Fair hair and skin, eczema, blonde hair, blue eyes and a musty urine odor

A

Phenylketonuria ; decreased phenylalanine hydroxylase.

Phenylalanine accumulates and tyrosine becomes essential

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27
Q

Deficiency of alpha galactosidase A

A

Fabry disease
Leads to the accumulation of ceramide trihexoside in the heart, brain and kidney
First sign is severe neuropathic limb pain.
Angiokeratoma and telengecticia can be seen on the skin
Findings include renal failure and an increase risk of stroke and MI

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28
Q

Galactosylceramide deficiency

A

Krabbe disease
Leads to accumulation of galactocerebroside in the brain.
Characterized by progressive CNS degeneration, optic atrophy, spasticity, and death within the first three years of life
Autosomal recessive

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29
Q

Glucocerebrosidase deficiency

A

Gaucher’s disease
Leads to the accumulation of glucereboside in the brain, liver, spleen and bone marrow.
May present with anemia and thrombocytopenia
Gaucher’s cells have a characteristic crinkled paper and parents with an large cytoplasm
Infantile form results in early rapid neurologic decline. Adult form which is more common is compatible with normal lifespan does not affect the brain

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30
Q

Sphingomyelinase deficiency

A

Niemann pick disease
Leads to build up of sphingomyelin cholesterol and reticuloendothelial and parenchymal cells and tissues
Presents with cherry red spot and hepatosplenomegaly
No man picks his nose with his sphinger

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31
Q

Hexosamindase deficiency

A

Tay-Sachs disease
Leads to GM2 ganglioside accumulation
Cherry red spots but no hepatosplenomegaly
Normal development until 3 to 6 months of age when weakness begins. An. exaggerated startle response maybe seem
Tay-saX lacks heXosaminidase

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32
Q

Arylsulfatase A deficiency

A

Meta-chromatic leukodystrophy
Accumulation of sulfatide in the brain, kidney, liver and peripheral nerves
Demyelination leads to progress ataxia and dementia

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33
Q

Alpha L iduronidase deficiency

A

Hurlers syndrome
Leads to corneal cloudy, mental retardation and gargoylism
Autosomal recessive

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34
Q

Iduronidate sulfate deficiency

A

Hunters syndrome
A milder form of hurler syndrome with no corneal cloudin and mild mental retardation
Hunters need to see (no corneal clouding) to aim for the X
X-linked recessive

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35
Q

Bowel obstruction by age

A

first month of life = malrotation with volvulus
~3 weeks= Pyloric stenosis
3 months - 3 years of age = intussusception

36
Q

Treatments for intussusception

A

Air/contrast barium enema. Diagnostic and greater than 95% of cases and cared even greater than 80%

37
Q

Sudden intermittent pain with rectal bleeding and a two-year-old

A

Think Merkel’s diverticulum

38
Q

Definitive diagnosis of Hirschsprung’s disease

A

Rectal biopsy of myenteric(Auerbach) plexus and submucosal (Meissner’s) plexus

39
Q

Treatment of malrotation with volvulus

A

NG tube decompression

Emergency surgical repair when Volvulus is gastric, routine surgery or endoscopy when volvulus is gastric

40
Q

Pneumatosis intestinalis more air in Bowel wall

A

Pathognomonic for necrotizing enterocolitis in neonates

41
Q

Treatment for IgA deficiency

A

Do not give IVIG because this may lead to the production of anti-IGA antibodies. Treat infection

42
Q

Progressive cellular ataxia and oculocutaneous telangiectasia

A

Ataxia-telangiectasia

Caused by DNA repair defect

43
Q

Severe lack of B and Tcells #bubble boy disease

A

Severe combined immunodeficiency (SCID)

Caused by adenosine deaminase deficiency requires PCP prophylaxis

44
Q

thrombocytopenia, eczema (atopiclike dermatitis), and recurrent pyogenic infections

A

Wiskott-Aldrich syndrome
X-linked recessive
High IgE/IgA, low IgM
Mnemonic WIPE: WAS; infection, purpura (thrombocytopenic); Eczema

45
Q

Recurrent skin abscesses

Nitroblue tetrazolium test positive

A

Chronic granulomatous disease
Deficient superoxidase production
Treat with daily TMP-SMX
INF-gamma can reduce the incidence of serious infection

46
Q

Delayed separation of the umbilical cord

Recurrent skin mucosal and Pulmonary infections

A

Leukocyte adhesion deficiency
A defect in the chemotaxis of leukocyte
no plus with minimal information and wounds
High white blood cell count in blood. Bone marrow transplant is curative

47
Q

Very White baby. Oculocutaneous Albinism, peripheral neuropathy, and neutropenia

A

Chediak Higashi syndrome
Defect in neutrophil chemotaxis/ micro tubule polymerization
Giant granules in neutrophils

48
Q

Coarse facies, abscesses, retain primary teeth, hyper IGE (hypereosinophilia), dermatologic (severe eczema)

A

FATED
Job syndrome
Defect in neutrophil chemotaxis
Recurrent s.aureus infection and abscess

49
Q

Recurrent episodes of angioedema

A

C1 esterase deficency (hereditary angioedema)
Toto hemolytic complement (CH50) to assess the quantity and function of complement
Purified C1 esterase and FFP can be use prior to surgery

50
Q

Recurrent neissera infections, meningococcal or gonococcal

A

Terminal complement deficiency (C5-C9)

Inability to form membrane attack complex (MAC)

51
Q

Crash and burn

A

Kawasaki

Conjunctivitis, rash, Adenopathy, strawberry tongue, hand and foot, burn (fever greater than 104)

52
Q

DiGeorge syndrome

A

Catch 22

Cardiac abnormalities, abnormal faces, thymic aplasia, cleft palate, hypocalcemia, chromosome 22

53
Q

Juvenile idiopathic arthritis serology

A

ANA +. RF -

Uveitis is common

54
Q

Top three causes of acute otitis media

Treatment

A

Bacterial: Strep pneumoniae, nontypable H. Influenzae, Moraxella catarrhalis
Virus: influenza A, RSV and parainfluenza virus
High dose Amoxicillin (Amoxicillin / clav for resistant)

55
Q

Most common cause of bronchiolitis

A

RSV

56
Q

Cause of croup
Presentation
Radiologic sign

A

Parainfluenza virus
Barking cough
Steeple sign from subglottic narrowing

57
Q

Treatment for severe croup

A

Nebulizer race mix epinephrine

If failure then intubation

58
Q

Epiglottitis presentation
Posturing
Radiologic sign

A

Dysphagia, drooling, high fevers
Neck hyperextended and Chin protruding (sniffing dog position)
Swollen epiglottis obliterating the vallaculae (his thumb sign)
Expected true emergency, intubate early

59
Q

Causes of meningitis in infants versus children

A

GEL in neonates. GBS, E.coli, listeria
SHiN in children: strep pneumonia, H.influ, Nesseiria

Treat with ampicillin and cefotaxime (or gentamicin) in the neonates, add acyclovir if concerned for herpes encephalitis
Treat with ceftriaxone and vancomycin in children

60
Q

Why avoid ceftriaxone in neonates

A

Ceftriaxone displaces bilirubin from albumin. Causes increased risk of biliary sledging and kernicterus

61
Q

Cause and treatment for whooping cough

A

Caused by Bordetella pertussis

Treat with 14 days of erythromycin

62
Q 
Fifth disease (erythema infectiosum)
Presentation in children vs presentation in adults
A

Slapped cheek disease in children. Can develop aplastic crisis (red cell)
Arthropathy in children and adults
Caused by parvovirus B19

63
Q

Koplik spots

A

Red spots with central gray speck on buccal mucosa

Measles!

64
Q

Complications of measles

A

3 Cs
Cough, coryza, conjunctivitis
Rare subacute sclerosing panencephalitis

65
Q

Rubella versus measles(rubeola)

A

Children with Rubella often have only a low-grade fever and do not appear ill.
Rash appears in spreads in one day in rubella, in measles last longer.

66
Q

Cause of transients tachypnea of the newborn

A

Retained amniotic fluid results in prominent peri hilar streaking in interlobar fissures resolved O2 administration.
Higher incidence in babies with C-sections due to no squeeze of chest during vaginal birth

67
Q

treatment of Lead poisoning by level

A

70: chelation therapy (inpatient EDTA + BAL [IM dimercaprol])

68
Q

what cell like does neuroblastoma arise from

A

Neural crest cells

69
Q

What cell like does wilms tumor arise from

A

metanephros (embryonic precursor to renal parenchymal)

70
Q

Imaging in pediatric bacterial meningitic

A

Low risk of herniation in children who are not comatose.

Administer abx then lumbar puncture

71
Q

children with a family history of migranes comes in with idopathic episodes of nausea and vomitting once a month

A

suspect cyclic vomiting syndrome due to abdominal migrane

72
Q

treament for unilateral cervical lymphadenitis

A

Incision and drainage

Clindamycin (coverage for staph and strep; amoxicillin has no staph coverage and thus is inappropriate)

73
Q

Jaundice, light colored stools and hepatomegaly that begins a few weeks after birth.

A

Biliary atresia.

New borns are initially well but develop conjugated hyperbilirubinemia

74
Q

Gilbert vs Crigler Najjar syndrome

A

Both result in unconjugated hyperbilirubinemia caused by UDP-glucuronl transferase deficiency. Gilbert’s is the less severe form (enzyme deficiency). C-N is more severe (absent enzyme)

75
Q

Location of medulloblastoma

A

cerebellar vermis

76
Q

Left axis deviation in neonate ad small or absent R wave in precordial leads

A

Tricuspid atresia. Lack of communication between R atria and R ventricle leads to hypoplastic right ventricle. thus no rigth axis deviation on ECG. Association with ASD or VSD is necessary for survival (thus VSD systolic murmur or ASD fixed split may be heard)

77
Q

An adolescent with nasal obstruction, visible nasal mass and frequent epitaxis. Has evidence of adjacent cartilage erosion

A

Angiofibroma

78
Q

Congenital cmv vs rubella

A

Rubella is bilateral deafness vs unilateral deafness; blindness on CMV is from chorioretinitis vs cataracts in rubella

79
Q

Neonatal vaccination time

A

Vaccinations should be given according to chronological age not gestational age.
Hep B vaccine should be given at birth (unless

80
Q

Precocious puberty, cafe au lait spots, and bony abnormalities

A

McCune Albright syndrome

The 3 Ps pigmentation (cafe au lait), precocious puberty, polyostotic fibrous dysplasia

81
Q

Non blanching skin rash on lower extremities after URI or GI bug. Extensive deposition of IgA in skin rash (on biopsy).

A

Henoch schonlein purpura which is a leukocytoclastic vasculitis.

82
Q

Inspiratory strider in infants that improves with neck extension concern for

A

Vascular ring

83
Q

Inspiratory strider an infant that is loudest and supine position and improves when fences held upright or prone

A

Laryngomalacia

84
Q

Three-month-old with hypoglycemia, lactic acidosis, hyperuricemia, hyperlipidemia. Appearance = doll like faces (fat cheeks), thin extremities, short stature, and protuberant belly (due to large liver and kidney)

A

Glucose-6-phosphatase deficiency = type 1 glycogen storage disease = von gierks disease

85
Q

Glycogen storage disease that presents with floppy baby with feeding difficulties, macroglossia, and heart failure (due to hypertrophic cardiomyopathy)

A

Type 2 glycogen storage disease = acid maltase deficiency = Pompe’sdisease

86
Q

Similar clinical manifestations as Type 1 glycogen storage disease. However laboratory findings differ patients with elevated liver transaminases, fasting ketosis, and normal blood lactate and uric acid concentration. Also normal kidneys but splenomegaly.

A

Type 3 glycogen storage disease = Glycogen debranching enzyme deficiency

87
Q

Infant with hepatosplenomegaly and failure to thrive as well as progressive cirrhosis of the liver in first 18 months of life. Glycogen storage disease

A

Type 4 glycogen storage disease = deficiency of branching enzyme activity = amylopectinosis

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