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Board Review > GI and Nutrition > Flashcards

GI and Nutrition Flashcards

1
Q

what is marasmus?

A

severe calorie malnutrition

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2
Q

what is kwashiorkor?

A

severe protein deficiency (will have pitting edema, fatty liver)

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3
Q

what are common manifestations of vitamin C deficiency?

A

follicular hyperkeratosis, “corkscrew-coiled” hairs, gingival bleeding, brittle bones, anemia

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4
Q
A

white lines of frankl - associated with vit c deficiency (scurvy)

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5
Q

what are characteristics of vitamin A deficiency?

A

Vitamin A Night blindness, xerophthalmia, Bitot spots, follicular hyperkeratosis

if corneal clouding present - emergency, needs large doses of IV vitamin A

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6
Q

what causes scaly skin, pseduotumor cerebri and hepatomegaly?

A

vitamin A overdose

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7
Q

what is seen with vitamin D deficiency?

A

weight-bearing age and results in poor growth, curvature of weight-bearing bones (Fig. 10-9), widening of epiphyses, and costochondral “rachitic rosary” beading (Fig. 10-10). Craniotabes, or softening of the skull, may be seen in infants

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8
Q

lab findings in rickets?

A

high PTH

low 25-OH D3

high 1,25-OH2

low calcium

low phos

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9
Q

before and after of what disease?

A

Rickets. Radiograph of the wrist of a patient with rickets. A, Irregularity and widening of the epiphyses in the distal radius and ulna. B, With appropriate therapy, remineralization and healing occur.

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10
Q

what is the other name for vitamin E?

A

tocopherol

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11
Q

manifestations of vitamin E deficiency?

A

neurologic dysfunction, neuroaxonal degernation and loss of reflexes, hemolytic anemia, peripheral neuropathy

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12
Q

what vitamin deficiency causes:

Beriberi: parasthesias, foot and wrist drop

Wernicke encephalopathy: opthalmoplegia, ataxia, confusion

A

thiamine (b1)

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13
Q

what vitamin deficiency causes:

cheilosis and sore tongue?

A

riboflavin (b2)

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14
Q

what vitamin deficiency causes:

pellagra: dermatitis, dementia and diarrhea

A

niacin B3

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15
Q

what vitamin deficiency causes:

megaloblastic anemia?

A

cobalamin B12

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16
Q

what vitamin deficiency causes:

diarrhea, rash, hypoguesia

A

zinc

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17
Q

in what syndromes can esophageal perforation occur spontaneously?

A

marfan and ehlers danlos

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18
Q

what metabolic derrangement occurs with pyloric stenosis?

A

hypochloremic hypokalemic metabolic alkalosis

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19
Q

what is menetrier disease?

A

protein-losing gastropathy secondary to CMV

swollen fundus and body

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20
Q

what is zollinger-ellison syndrome?

A

gastrin-secreting tumor (gastrinoma)

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21
Q

what is associated with gastrinomas?

A

MEN-1

so look for hyperparathyroidism and adrenal tumors

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22
Q

at what age do kids start to develop lactase deficiency?

A

2yrs and up

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23
Q

what is abetalipoprotinemia?

A

autosomal recessive

absence of apo B

inability to synthesize chylomicrons

maldorous steatorrhea, FTT

need fat soluble vitamins, limit fat intake

24
Q

what is hartnup disease?

A

defect in transport of free neutral amino acids

25
Q

what is defective in blue diaper syndrome?

A

tryptophan (malabsorption of it)

26
Q

how do children with abnormal zinc absorption present?

A

bullous and pustular dermatits

alopecia

blepharitis

conjunctivitis

diarrhea

FTT

27
Q

what is absorbed in the ileum (important for short gut kids)

A

bile salts

vitamin B12

28
Q

name some things associated with UC

A

clubbing

ankylosis spondylitis

erythema nodosum

pyoderma gangrenosum

primary sclerosing cholangitis

dvt/pulm embolism

29
Q

name some things associated with chron’s disease

A

apthous ulcers

fissures

anal fistulas

abscesses

weight loss

growth failure

30
Q

which responds to bowel rest: UC or chrons

A

chrons

31
Q

how does peutz-jeghers present

A

autosomal dominant

GI harmartomatous polyps and mucocutaneous hyperpigmentation

lip and buccal freckles!

intermittent colicky abdominal pain

increased risk of cancers

32
Q

what is proteus syndrome?

A

hemihypertrophy, hamartomatous polyps, gigantism of extremities, angiomas (PTEN related)

33
Q

what is Gardner syndrome?

A

familial adenomatous polyposis with extraintestinal tumors

desmoid tumors, epidermoid cysts, osteomas,

need screening colonoscopies, thryoids studiesn, and upper GI studies to monitor for cancer

34
Q

what type of lymphoma is common in the small intestine

A

burkitt’s lymphoma (non-hodgkins)

35
Q

what are omphaloceles associated with?

A

beckwith-wiedemann, pentalogy of cantrell, trisomies

36
Q

which is located on the midline - omphalocele or gastroschisis?

A

omphalocele - umbilical cord connects to it

gastroschisis is to the right of the umbilicus

37
Q

what are the top three causes of rectal prolapse?

A
  1. constipation
  2. diarrhea
  3. CF
38
Q

what are the top two causes of exocrine pancreatic insufficiency?

A
  1. CF
  2. Shwachman-Diamond syndrome
39
Q

what makes up shwachman-diamond syndrome?

A

pancreatic insufficiency, short stature, neutropenia, skeletal abnormalities

autosomal recessive

higher risk of myelproliferative disorders

40
Q

what is the cullen sign? the grey turner sign?

A

cullen: bluish discoloration around/near umbilicus

grey turner: bluish discoloration of the flanks

both associated with acute hemorrhagic pancreatitis

41
Q

what is the triad for choledocal cyst? how do you image it?

A

abdominal pain, jaundice, RUQ mass

ultrasound will show extra and intrahepatic dilitation

ERCP can show anatomy

42
Q

what is associated with congenital hepatic fibrosis?

A

autosomal recessive polycystic kidney disease

43
Q

what is caroli disease?

A

abnormality of the hepatic ductal plate - congenital dilation of the larger, segmental intrahepatic bile ducts

can occur with congential hepatic fibrosis (dilated bile duct structuresand portal tracts without interlobular ducts in the center)

44
Q

what is alagille syndrome?

A

autsomal dominant

associated with peripheral pulmonary stenosis and neonatal cholestasis

“paucity” of small intrahepatic ducts, butterfly vertebrae, abnormal radius/ulna, and facies including pointed chin and prominent forehead

45
Q

what do you use for hepatitis A prophylaxis in case of exposure?

A

immunoglobulin

also give to household contacts, needlesharing partners, daycare, nursing home

46
Q

does gilbert cause conjugated or unconjugated hyperbili?

A

unconjugated

alteration in UDP-GT gene

prominent during times of illness or fasting

47
Q

what is difference between crigler-najjar type 1 and type 2?

A

type 1: no conjugated bilirubin (complete absence of UDP-GT activity), need exchange transfusion as neonate

type 2: partial activity, hyperbili <10 is common, resolves with phenobarb

48
Q

what is dubin-johnson syndrome?

A

deficiency of MOAT/MRP2 gene - mild conjugated hyperbili, no hepatocellular injury

49
Q

how does a1-antitrypsin present?

A

neonatal cholestasis, juvenile cirrhosis, chronic hepatitis, hepatocellular cancer

50
Q

what are the screening tests for wilson disease?

A

ceruloplasmin (will be low)

24-hr urinary copper (high)

liver biopsy

can give D-penicillamine (will increase urinary excretion even higher)

51
Q

what is the triad for hemochromatosis?

A

elevated iron levels, bronzed skin, diabetes

52
Q

what are teh antibodies anti-sla and anti-lkm associated with?

A

autoimmune hepatitis

(look for other autoimmune diseases)

53
Q

what will you see on cholangiogram in primary sclerosing cholangitis?

A

“beading” - alternating normal and strictured portions of biliary tree

54
Q

what is fitz-hugh-curtis syndrome?

A

gonococcal perihepatitis

55
Q

what all is hydrops of the gallbladders associated with?

A

kawasaki, strep pharyngitis, prolonged fasting, tpn, HSP

noninflammatory enlargement of gallbladder

56
Q

what is the most common malignant liver tumor in children?

A

hepatoblastomas

elevated afp

Board Review (11 decks)

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