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PATH STORE > Pediatric Diseases > Flashcards

Pediatric Diseases Flashcards

1
Q

A 17-year-old primigravida gives birth at 34 weeks’ gestation to a male infant of low birth weight. The infant is given exogenous surfactant and does not develop respiratory distress. On the third day of life, physical examination reveals hypotension, abdominal distention, and absent bowel sounds, and there is bloody stool in the diaper. A radiograph shows pneumatosis intestinalis and abdominal free air. What do you think of the diagnosis?

A

Necrotizing enterocolitis

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2
Q

An 18-year-old woman gives birth to a term infant after an uncomplicated pregnancy and delivery. Over the first 2 days of life, the infant becomes mildly icteric. On physical examination, there are no morphologic abnormalities. Laboratory studies show a neonatal bilirubin concentration of 4.9 mg/dL. The direct Coombs test of the infant’s RBCs yields a positive result. The infant’s blood type is A negative, and the mother’s blood type is O positive. What type of bilirubin is present in child’s blood?

A

Unconjugated

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3
Q

Amniotic Band Syndrome is a congenital birth defect believed to be caused by entrapment of fetal parts (usually a limb or digits) in________________ while in utero

A

fibrous amniotic bands

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4
Q

Potter sequence: typical physical appearance and associated _______________(1) of a neonate as a direct result of ____________(2) and compression while in utero

A
  1. pulmonary hypoplasia 2. oligohydramnios
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5
Q

Oligohydramnios (decreased amniotic fluid) is caused by___________ and ___________

A

Renal Agenesis Amniotic leak

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6
Q

The position of delivery of the child in potters is_____________

A

Breech Presentation

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7
Q

An infant born prematurely at 28 weeks gestation is immediately noticed to be tachypneic and cyanotic following birth. A chest CT scan is obtained, which is shown in Figure A. A presumptive diagnosis is made based on the clinical picture and chest CT, and treatment is initiated. Which of the following organelles and cell types are involved in this clinical picture?

A
  1. Lamellar bodies - type I pneumocytes 2. Mitochondria - type II pneumocytes 3. Lamellar bodies - type II pneumocytes 4. Endoplasmic reticulum - type I pneumocytes 5. Plasma membrane - type II pneumocytes
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8
Q

An infant born prematurely at 28 weeks gestation is immediately noticed to be tachypneic and cyanotic following birth. A chest CT scan is obtained, which is shown in Figure A. A presumptive diagnosis is made based on the clinical picture and chest CT, and treatment is initiated. Which of the following organelles and cell types are involved in this clinical picture?

A

Lamellar bodies - type II pneumocytes

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9
Q

A P2G1 diabetic woman is at risk of delivering at 29 weeks gestation. Her obstetrician counsels her that there is a risk the baby could have significant pulmonary distress after it is born. However, she states she will give the mother corticosteroids, which will help prevent this from occurring. Additionally, the obstetrician states she will perform a test on the amniotic fluid which will indicate the likelihood of the infant being affected by this syndrome. Which of the following ratios would be most predictive of the infant having pulmonary distress?

A

Lecithin: sphingomyelin ratio < 1.5

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10
Q

A cyanotic neonate. Following is the blood gas following 100% oxygen therapy. Whats the likely cause/mechanism? Before: PO2=30% After: PO2=35%

A

Hyalin membrane disease/diffusion defect likely

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11
Q

A cyanotic neonate. Following is the blood gas following 100% oxygen therapy. What’s the likely cause/mechanism? Before: PO2=30% After: PO2=50%

X-Ray chest attached.

A

Pulmonary stenosis/perfusion defect likely

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12
Q

1.Retrolental fibroplasia is due to___________________

A

(high concentration of O2 produces free radicals)

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13
Q

Necrotizing enterocolitis (NEC), which typically occurs in the ________________of life in ___________, _________-fed infants.

A

second to the third Week
premature
formula

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14
Q

NEC, The Underlying pathology is_______

A

intestinal ischemia

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15
Q

In NEC septic and shock is caused by_______

A

1.Platelet-activating factor (PAF) → by promoting enterocyte apoptosis → sepsis and shock

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16
Q

NEC:

Gangrene of the intestine

-Involves________

A

terminal ileum, cecum and right colon

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17
Q

Fetal hydrops refers to the ____________in the

fetus during intrauterine growth.

A

accumulation of edema fluid (transudate)

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18
Q

Fetal Hemolysis: Mother forms _____ antibodies which cross the placenta and destroy fetal RBCs (______ hypersensitivity)

A

IgG

Type 2

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19
Q

A 19-year-old woman is G2 P1. Her previous gestation resulted in a normal term birth at home. Her current pregnancy results in the birth of a 2990 gm baby at 35 weeks. At birth, the infant appears hydropic and icteric, but no congenital anomalies are present. The baby’s hemoglobin is 8.5 g/dL. The placenta is also hydropic, but microscopic examination of the placenta shows no inflammation of either fetal membranes or placental villi, and there is no meconium staining.

Which of the following mechanisms is the most likely explanation for these events?

A Maternal rubella infection in the first trimester

B A maternal chromosomal abnormality

C Maternal antibodies crossing the placenta

D Elevated maternal serum glucose

E Increased maternal phenylalanine levels

A

C Maternal antibodies crossing the placenta

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20
Q

Cystic hygromas are characteristically seen, but not limited to, constitutional chromosomal anomalies such as ————karyotypes

A

45,X0

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21
Q

Anemia →——————- → increased hydrostatic pressure → edema and anasarca, →hydrops fetalis

A

high output Cardiac failures

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22
Q

Anemia → ————— → decreased plasma oncotic pressure → edema and anasarca, →hydrops fetalis

A

Liver hypoxia

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23
Q

CF=The carrier frequency in the United States is ———–among Caucasians but significantly lower in African Americans, Asians, and Hispanics.

A

1 in 20

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24
Q

Even heterozygote carriers (__________) have a higher incidence of respiratory and pancreatic diseases

A

2-4%

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25
Q

CYSTIC FIBROSIS IN SKIN: Chloride channel defect in the sweat duct causes increased _____________________concentration in sweat

A

chloride and sodium

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26
Q
A
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27
Q

In the airway, cystic fibrosis patients have__________chloride secretion and __________sodium and water reabsorption leading to _______ of the mucus layer coating epithelial cells

A

decreased

increased

DEHYDRATION

28
Q

CF= Bronchiectasis •Infections by common

A

Pseudomonas

29
Q

CF PANCREAS:

  • _________ of ducts leads to cystic dilatation, AND
  • _____________ of exocrine pancreas acini.
A

Mucous plugging

Atrophy

30
Q

Pancreatic insufficiency, a feature of classic cystic fibrosis, is virtually always present when there are CFTR mutations with abnormal ______________conductance.

A

bicarbonate

31
Q

Deficiency of fat-soluble vitamins A would lead to__________

A

Sq metaplasia
decreased night vision

32
Q

Deficiency of fat-soluble vitamins D would lead to__________

A

osteomalacia

fracture

33
Q

Deficiency of fat-soluble vitamins D in renal failure is also known as __________

A

Renal osteodystrophy (osteomalacia)

34
Q

Deficiency of fat-soluble vitamins K would lead to__________

A

bleeding, deficiency of clotting factors- 2,7,9 and 10.

35
Q

CF. Chronic inflammation with scarring leads to a metabolic disease __________.

A

Diabetes mellitus

36
Q

Meconium ileus: infants present with abdominal distension, failure to pass ___________, and emesis.

A

stools

37
Q

cystic fibrosis has higher levels of salt than normal -more than ——

A

60 mmol/L

38
Q

Pyloric stenosis- ———–vomiting, no stool

A

projectile

39
Q

–Duodenal Artesia seen in- ———— vomiting +

A

Downs syndrome

40
Q

–Intussusceptions - constipation ( < 12 months) is caused by

A

Diarrhea

Swollen payers patches

41
Q

the sudden death of an infant under ___________of age which remains unexplained after a thorough case investigation, including a performance of a complete autopsy, examination of the death scene, and review of the clinical history

A

1 year

42
Q

1=

2=

3=

Please see the attached image and answer

A

2-4 months

Preterm, maternal smoking

Prone, overheating, bed sharing, head covering

43
Q

SCID•Non-specific autopsy findings

–_______________

A

Multiple petechiae, Pulmonary congestion ± pulmonary edema

44
Q

A 27-year-old male presents with an unprovoked convulsive episode. Medical history is noncontributory. Family history is significant for clear cell renal cell carcinoma in his father, resulting in death. The exact cause of his paternal grandfather’s death is unclear, but is thought to be due to a “bleed in the brain.” Head CT is performed.

What do you think?

A

Think VHL

45
Q

A 21-year-old female presents to the family physician with 3 weeks of headaches, sweating, and palpitations. Her BP was 160/125 mmHg, and a 24-hour urine test revealed elevated vanillymandelic acid (VMA) and normetanephrine. Past medical history is notable for bilateral retinal hemangioblastomas, and family history is significant for three generations (patient, mother, and maternal grandfather) with similar symptoms. Genetic analysis revealed a mutation of a gene on chromosome 3p. Which of the following is the patient at risk of developing?

A

CCC (RCC)

46
Q

Lack of VHL activity prevents ubiquitination and degradation of ___________and is associated with increased levels of ___________.

A

HIF-1

angiogenic growth factors (VEGF)

47
Q

•Germline mutations of the VHL gene is associated with

A

–Hereditary bilateral renal cell cancers

–Pheochromocytomas

–Hemangioblastomas of the central nervous system

–Retinal angiomas

–Renal Cysts

48
Q

•Glomus tumor

–benign, ____________, red-blue tumor formed from arteriovenous shunts

•found under the fingernails

–derived from ______________of glomus body

A

painful

smooth muscle cells

49
Q

Lynphangioma_ Histology: Numerous dilated channels lined by _____________and contain ____________cells.

A

flattened endothelium

lymphoid

50
Q

Small Blue Round Cell Tumor because of _____________ratio. Microscopically similar cells with certain differences. Always malignant.

A

high N/C

51
Q

A 4-year-old girl is brought to the emergency room after her parents felt an abdominal mass during her bath. She complains of headaches. On physical exam, her eyes jerk around erratically. Her feet also twitch occasionally. The abdominal mass is irregular, firm, and clearly crosses the midline. An MRI of her head shows potential metastasis to the skull. What do you think?

A

Neuroblastoma

52
Q

Neuroblastoma-If arise from sympathetic ganglia (neural crest origin)-

A

paraganglioma

53
Q

Larger cells having more abundant cytoplasm with large vesicular nuclei & prominent nucleoli, representing ganglion cells in various stages of maturation admixed with neuroblasts- diagnosis?

A

Ganglioneuroblastoma

54
Q

Neuroblastoma staging

•Stage 4S (“S” = special): Localized primary tumor (as defined for stages 1, 2A, or 2B) with dissemination limited to ____, _____, and/or _________; stage 4S is limited to infants younger than 1 year.

A

skin

liver

bone marrow

55
Q

Neuroblastoma- Imaging131I-MIBG body scan to detect__________

A

metastasis

56
Q

A 15-month-old male is brought in to his pediatrician for a routine exam. On exam, his pediatrician notices that he has no irises, consistent with aniridia. He is also found with a palpable, non-tender mass on the left side of his abdomen. On further questioning, his mother reveals that her cousin had a similar abdominal mass at a young age. The patient and his mom are referred to pediatric surgeons for further management. An abdominal CT scan is obtained. Diagnosis?

A

Wilms

57
Q

A 2-year-old girl is brought to the emergency room after her mother discovers a mass in the girl’s abdomen while bathing. The girl occasionally cries when urinating, but is otherwise asymptomatic, without any abdominal pain. On exam, she is found to be hypertensive. There is a palpable, non-tender mass on the right side, which does not cross the midline. A chest radiograph is ordered to assess for lung involvement. Diagnosis?

A

Nephroblastoma

58
Q

WAGR syndrome:_______________

WT 1 is a tumor suppressor, is deleted

A

Wilm’s Tumor, Aniridia, Genital anomalies, Mental Retardation

59
Q

Denys-Drash syndrome:

Characterized by________________

A

gonadal dysgenesis and early onset nephropathy

60
Q

Beckwith-Weidemann syndrome

  • ____________________________
  • WT 2, second Wilms’ tumor locus is deleted neonatal hypoglycemia.
A

Enlargement of body organs: macroglossia, hemihypertrophy, omphalocele, adrenal cytomegaly

61
Q

Wilms histology

A

Mixed tumor: (blastemal, epithelial, and stromal cells) Various tissue components and all derive from one germ cell

62
Q

A newborn male is diagnosed with unilateral retinoblastoma. Molecular analysis of enucleated tumor cells reveals loss of both normal alleles of Rb gene. In comparison, skin fibroblasts of the patient do not show any abnormality at the Rb locus.

Familial or Sporadic?

A

Sporadic- both mutation in retinoblast

63
Q

Normal P53

–prevents a cell from entering ____ phase

–leads to inhibition of kinase activity of Cdk4 via _______

–can induce apoptosis if cell damage is severe

•via the __________gene (proapoptotic)

–inhibits __________antiapoptosis gene

–stimulates the release of _____________from mitochondria

A

S

p21

bax

BCL2

cytochrome c

64
Q

•Rb

–prevents cell from entering S phase

–binds __________to block its function as a transcription factor

A

E2F

65
Q

Histology of Flexner-Wintersteiner rosettes

A

•Rosette with a central lumen and pink fibrillary back ground

PATH STORE (24 decks)

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