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Pediatrics > BRS #4 > Flashcards

BRS #4 Flashcards

0
Q

how many AAs are essential

A

9

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1
Q

what are essential nutrients

A

stuff that cannot be made by the body

you have to take it in from your diet

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2
Q

infants require more _____ in their diet

A

protein

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3
Q

_______ play an important role in infant brain development

A

essential fatty acids

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4
Q

most common energy depletion state

  • near starvation from protein and nonprotein deficiencies
  • very thin from loss of muscle and body fat
A

marasmus

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5
Q
  • protein deficient state characterized by generalized edema, abdominal distension, changes in skin pigmentation, and thin sparse hair
  • common in areas where starches are the main dietary staple
A

kwashiorkor

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6
Q

vitamin and mineral deficiency

-night blindness, xerophthalmia (dry conjunctiva and cornea)

A

vitamin A

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7
Q

vitamin and mineral deficiency

rickets/osteomalacia, dental caries, hypocalcemia, hypophosphatemia

A

vitamin D

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8
Q

vitamin and mineral deficiency

anemia/hemolysis, neurologic deficits, altered prostaglandin synthesis

A

vitamin E

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9
Q

vitamin and mineral deficiency

coagulopathy/prolonged PT, abnormal bone matrix synthesis

A

vitamin K

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10
Q

vitamin and mineral deficiency

berberi (cardiac failure, peripheral neuropathy, hoarseness or aphonia, wernicke’s encephalopathy)

A

vitamin B1 (thiamine)

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11
Q

vitamin and mineral deficiency

dermatitis, cheilosis, glossitis, microcytic anemia, peripheral neuritis

A

vitamin B6 (pyridoxine)

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12
Q

vitamin and mineral deficiency

megaloblastic anemia, demyelination, methymalonic acidemia

A

vitamin B12 (cobalamin)

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13
Q

vitamin and mineral deficiency

scurvy (hematologic abnormalities, edema, spongy swelling of gums, poor wound healing, impaired collagen synthesis)

A

vitamin C

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14
Q

vitamin and mineral deficiency

megaloblastic anemia, neutropenia, impaired growth, diarrhea

A

folic acid

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15
Q

vitamin and mineral deficiency

pellagra (diarrhea, dermatitis, dementia), glossitis, stomatitis

A

niacin

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16
Q

vitamin and mineral deficiency

skin lesions, poor wound healing, immune dysfunction, diarrhea, growth failure

A

zinc

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17
Q

what do you find in the stool if there carbohydrate malabsorption

A
  • watery
  • acidic (pH < 5.6)
  • reducing substances detected via a positive clinitest reaction
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18
Q

congenital enterokinase deficiency is a rare cause of _____ and ______ loss in the stool
what results?

A

protein and nitrogen

hypoproteinemia –> edema and growth impairment

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19
Q

what happens in protein losing enteropathies

A

transudation of protein from inflamed intestinal mucosa

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20
Q

what test to look for protein in the stool

A

fecal alpha-1-antitrypsin levels

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21
Q

decreased _____ activity results in steatorrhea, decreased absorption of fat-soluble vitamins (A, D, E, K)

A

lipase

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22
Q

acanthocytosis of erythrocytes is seen in _________

A

abetalipoproteinemia

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23
Q

AR syndrome

pancreatic exocrine insufficiency, FTT, short stature, neutropenia, sometimes pancytopenia

A

Schwachman-Diamond syndrome

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24
Q

most common protein intolerance in kids is ______
sxs include abdominal pain, diarrhea, vomiting, mucus in stool, rectal bleeding after exposure to protein
sxs resolve within a few days after withdrawal of suspected antigen
prognosis??

A

cow’s milk protein

most protein intolerance is transitory and resolves by 1-2 years of age

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25
Q

celiac disease is an autoimmune disease in the _________ 2/2 intolerance to gluten

A

proximal small intestine

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26
Q

how to dx celiac disease

A

small bowel biopsy: short flat villi, deep crypts, vacuolated epithelium with lymphocytes

  • clinical response after removal of gluten
  • antibodies: IgA-endomysial, TTG, *antigliadin IgG for IgA deficient pts
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27
Q

noncompliance with gluten free diet in adolescence can cause:

A

growth failure and delayed sexual maturity

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28
Q

after gut resection, ________ and ______ malabsorption with steatorrhea are common
distal small bowel (ex. ileum) limits _____ and ______ resorption

A

carbohydrate and fat

vitamin B12 and bile acid

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29
Q

how to manage short bowel syndrome

A
  • TPN
  • early enteral feedings
  • small bowel transplant only with life-threatening TPN-associated liver disease
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30
Q

complications of short gut syndrome

A 
TPN cholestasis 
intestinal bacterial overgrowth 
nutritional deficiencies
poor bone mineralization
renal stones
secretory diarrhea
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31
Q

GER vs. GERD

A

GER- normal physiologic state, happy spitters, emesis is benign
GERD- pathologic state associated with GI or pulm sxs and sequelae

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32
Q

sandifer syndrome

A

torticollis with arching of the back caused by painful esophagitis

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33
Q

if you have sxs of GERD past 1 year of age, it is unlikely to resolve by itself (T/F)

A

T

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34
Q

how to dx GERD

A
  • UGI- high sensitivity, low specificity
  • scintigraphy- look for rate of gastric emptying, also if anything is in the lungs
  • pH probe measurement
  • endoscopy with biopsy if diagnosis is uncertain
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35
Q

how to tx GERD

A

sit up when eating
frequent small meals that are thickened
H2 blockers, PPIs
motility agents like metoclopramide but side effects are high
may need surgery like nissen fundoplication (+ G tube in infants)

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36
Q
  • first born Caucasian male with projectile vomiting of nonbilious milky fluid in weeks 2-3
  • irritable but hungry
A

pyloric stenosis

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37
Q

physical and lab exam of pyloric stenosis

A
  • olive (hypertrophied pyloric muscle just above and to the right of the umbilicus), may see peristaltic waves after feeding
  • hypokalemic, hypochloremic metabolic alkalosis from vomiting
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38
Q

how to dx pyloric stenosis

A
  • *US

- can also see string sign on UGI

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39
Q

how to tx pyloric stenosis

A

correct electrolytes and dehydration then partial pyloromyotomy

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40
Q

midgut volvulus involves the midgut twisting around the ______

A

superior mesenteric vessels –> ischemic and obstruction

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41
Q

malrotation is more common in _____ (boys/girls)

A

boys

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42
Q

lack of small bowel fixation can result in these compressing the duodenum and causing mechanical obstruction

A

peritoneal bands (Ladd’s bands)

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43
Q

classic presentation of volvulus

A

sudden onset of abdominal pain and bilious vomiting in an otherwise healthy infant

  • older children: intermittent crampy abdominal pain and vomiting
  • anorexia, distension, blood tinged stools
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44
Q

how to evaluate for volvulus

A
  • xrays: proximal intestinal distention and obstruction
  • UGI is the diagnostic tool of choice
  • lower intestinal contrast studies sometimes
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45
Q

how to tx volvulus

A

surgical emergency!

fluids, NG suction, abx

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46
Q

________ is the MCC obstruction in the neonatal period

it happens more in ______ (boys/girls)

A

intestinal atresia

boys

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47
Q

duodenal atresia is sometimes associated with ______

A

Down syndrome

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48
Q

duodenal atresia

  • prenatal US may show:
  • PE may show:
A

gastric dilation with polyhydramnios

scaphoid abdomen with epigastric distention

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49
Q

xray finding in duodenal atresia

what else can you do?

A

double bubble sign

contrast study

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50
Q

how to tx duodenal atresia?

A

NG tube, hydration, correct electrolytes

duodenoduodenostomy

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51
Q

jejunoileal atresia is like duodenal atresia except….

A

they are caused by mesenteric vascular accidents and are not associated with other anomalies

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52
Q

what is intussusception
optimal age
most common location

A

telescoping of a proximal portion of intestine into a more distal portion
ages 5-9 months
ileocolic

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53
Q

_____ is the MCC bowel obstruction after the neonatal period but less than 2 years of age

A

intussusception

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54
Q
  • sudden onset of crampy or colicky abdominal pain that occurs in intervals
  • infants may draw legs toward the chest
A

intussusception

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55
Q

PE findings of intussusception

A

currant jelly stool

sausage shaped mass palpated in the RUQ

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56
Q

how to dx and tx intussusception

what’s a classic sign that you see?

A

air or contrast enema to dx and tx- you see coil spring sign
if that doesn’t work, you can operatively reduce it

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57
Q

most common pediatric emergency operation

A

apendectomy

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58
Q

peak age of appendicitis

A

10-12 years

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59
Q

appendicitis classically presents with peri-umbilical pain that migrates to _________

A

McBurney’s point

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60
Q

how to dx and tx appendicitis

A

US or CT but sometimes might go straight to surgery

fluids, periop abx, appendectomy

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61
Q

Gray Turner and Cullen signs and what they are assoc with

A

Grey Turner- bluish discoloration of flanks
Cullen- bluish discoloration of the periumbilical area
assoc with severe cases of pancreatitis

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62
Q

pancreatitis is ______ (common/uncommon) in children and is often caused by _______ and ______

A

uncommon

trauma (most common), infection, idiopathic (2nd most common)

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63
Q

how to evaluate pancreatitis

A
  • amylase- rises within hours of pain onset and remains elevated 4-5 days
  • lipase- more specific indicator and remains elevated for longer
  • abdominal US for diagnosing and monitoring
  • CT to look for complications: necrosis, pseudocyst, abscess, etc.
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64
Q

how to manage pancreatitis

A

supportive care
TPN if needed
abx for necrotizing pancreatitis
+/- surgery but only when the inflammation has calmed down

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65
Q

2 complications of pancreatitis

A

ARDS

pseudocyst

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66
Q

acute cholecystitis is ______ (common/uncommon) in children and can be caused by ______, _______, or ________

A

uncommon

sickle cell disease, CR, or prolonged TPN therapy

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67
Q

what’s the biggest cause of acute acalculous cholecystitis

A

infection with salmonella, shigella, E. coli

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68
Q

cholecystitis PE shows ______

A

Murphy’s sign and RUQ pain

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69
Q

how to dx cholecystitis

A

RUQ ultrasound

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70
Q

how to tx cholecystitis

A

fluids, abx, analgesia, cholecystectomy once stabilized

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71
Q

chronic abdominal pain is pain that occurs each month for ____

A

3 consecutive months

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72
Q

chronic epigastric pain

A

belching, bloating, nausea, vomiting, early satiety

like adult nonulcer dyspepsia

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73
Q

chronic periumbilical pain

age and characteristics

A

classic functional abdominal pain (FAP)
age > 5 years
pain is varied in character and does not interfere with pleasurable activities

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74
Q

chronic infraumbilical pain

A

abdominal cramping, bloating, alterations in stool

like adult IBS

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75
Q

how to evaluate chronic abdominal pain

A

if you think it’s functional based on hx, you don’t have to do much
t/c screening labs and lactose breath hydrogen testing for lactose intolerance
**don’t screen for H pylori b/c many kids are asymptomatic carriers

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76
Q

how to treat functional abdominal pain

what’s the prognosis

A

normalize child’s activities, educate the parents
counseling
symptomatic meds don’t really work
prognosis is poor– many have chronic abdominal pain as adults

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77
Q

encopresis is almost always associated with ______

A

severe constipation

liquid stool leaks around the hard retained stool

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78
Q

initially, ______ (breastfed/formula-fed) infants defecate more frequently

A

breastfed

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79
Q

_______ is the most common form of constipation during childhood and is usually due to ______

A

functional fecal retention (FFR)

some sort of traumatic event

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80
Q

MCC organic constipation in an otherwise healthy child

A

Hirschsprung’s disease

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81
Q

signs that point towards organic constipation

A

delayed passage of meconium (>48 hours), onset in infant, h/o pelvic surgery, encopresis before age 3 years, inability to toilet train

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82
Q

how to manage constipation in kids

A

most of the time, don’t need to do diagnostic tests

  • increase soluble fibers and sorbitol containing juices
  • clean out the fecal mass
  • soften the stool with mineral oil
  • educate the patient and family
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83
Q

systemic causes of organic constipation

A

dehydration, hypothyroidism, cystic fibrosis

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84
Q

IBD age of onset is _____

A

bimodal (teens and then 50s)

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85
Q

in ulcerative colitis, inflammation is limited to the ___ and localized to the _____
it starts in the ______ and extends proximally in a continuous fashion

A

mucosa, colon

rectum

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86
Q

complications of UC

A

toxic megacolon- fever, abdominal distention, septic shock, perf
increased risk of colon cancer

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87
Q

extraintestinal manifestations of UC

A

uveitis, arthropathy, *pyoderma gangrenosum, sclerosing cholangitis

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88
Q

serologic antibody in UC

A

antineutrophil cytoplasmic antibody

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89
Q

inflammation of any segment of the GI tract

  • skip lesions
  • transmural inflammation
A

Crohn’s disease

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90
Q

most common area for Crohn’s in kids

A

terminal ileum

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91
Q

some intestinal sxs of Crohn’s

A
  • pain, decreased growth, cramping, diarrhea
  • small bowel disease –> malabsorption of B12, iron, zinc, folate
  • perianal disease
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92
Q

extraintestinal manifestations of Crohn’s disease

-more common in Crohn’s vs. UC

A 
FTT
delayed sexual development
oral aphthous ulcers
erythema nodosum
arthritis 
renal stones
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93
Q

serologic antibody in Crohn’s

A

anti-saccharomyces cerevisiae antibody

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94
Q

complications of Crohn’s disease

A

fistula, stricture, abscess

risk for colon cancer is less than for UC

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95
Q

how to work up IBDD

A 
CBC: anemia, leukocytosis
ESR: high 
albumin, LFTs, antibody tests
US or CT or UGI
colonoscopy with biopsies confirm the diagnosis
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96
Q

treatment for IBD

A

sulfasalazine- mild dz esp for UC, prevents relapses too
steroids- for acute exacerbations and inducing remission
immunosuppressive agents- induces long term remission
flagyl- treatment of UC with peri-anal involvement

97
Q

surgery for IBD

A
  • proctocolectomy is curative for UC but not first line

- surgery only when really needed for CD b/c recurrence rate is high after bowel resection

98
Q

_________ for dx and tx for active UGI bleeding with hemodynamic changes

A

upper endoscopy

99
Q

managing UGI bleed

A
  • fluid resuscitation +/- transfusion
  • octreotide/vasopressin for variceal bleeding
  • abx if H Pylori ulcer or H2 blocker/PPI for ulcers, esophagitis, gastritis
  • endoscopic therapy for active bleeding or if rebleeding is very likely
  • arterial embolization for serious bleeding from vascular malformations
  • surgery for duodenal ulcers with active arterial bleeding, perforation, or varices
100
Q

premie with rectal bleeding, feeding intolerance, abdominal distention

A

necrotizing enterocolitis

101
Q

MCC significant lower GI bleeding beyond infancy
-painless, intermittent, streaky bleeding

what is it and how to tx

A

juvenile polyps

colonoscopy with polypectomy

102
Q

painless acute rectal bleeding in an otherwise healthy child
what is it and how to tx?

A

meckel’s diverticulum- ectopic gastric mucosa

surgical resection

103
Q

MAHA, thrombocytopenia, acute renal failure

intestinal ulceration and infarction of the bowel cause bleeding

A

HUS

104
Q

palpable purpuric rash on butt and LE
large joint arthralgias
renal involvement
GI bleeding

A

HSP (IgA mediated vasculitis)

105
Q

other causes of LGIB in kids

A

Hirschsprung’s disease
allergic colitis
infectious enterocolitis
IBD

106
Q

hepatocellular enzymes… which one is most specific for liver injury?

A

AST is sensitive but not specific
LDH is nonspecific
**ALT is very specific for liver disease

107
Q

2 biliary enzymes

A
  • alkaline phosphatase- also can be elevated with rapid growth, bone, kidney, intestinal disease
  • GGT and 5NT (this is more specific)
108
Q

define infant jaundice

when does jaundice become clinically evident

A

elevated bili after neonate period and within the first year of life
when bili is > 3mg/dL

109
Q

as many as ____ of all neonates or infants experience transient jaundice, the majorty of whom have unconjugated hyperbilirubinemia

A

50%

110
Q

jaundice begins _____ and spreads ______

A

cranially and spreads caudally

111
Q

hemolysis (ex. ABO incompatibility) or very large hematoma –> too much bili production –> early on unconjugated hyperbili then later conjugated bili as the liver catches up

A

inspissated bile syndrome

112
Q

50% activity of UDP-glucuronyl transferase deficiency

mild unconjugated hyperbili with stress or poor nutrition

A

Gilbert’s

113
Q

AR disorder
100% UDP-glucuronyl transferase deficiency
extremely high unconjugated bili –> kernicterus almost universally

A

Crigler-Najjar type 1

114
Q

AD disorder
90% UDP-glucuronyl transferase deficiency
lower bili levels lead to kernicterus sometimes

A

Crigler-Najjar type 2

115
Q

MCC cholestasis in the newborn

what is it, what’s the tx and prog?

A
  • neonatal hepatitis- idiopathic hepatic inflammation during the neonatal period
  • course is generally self-limited and 70% resolve during infancy
  • dx of exclusion
  • tx with nutritional support (concentrated calories, MCT-containing formulas, ADEK vitamins, +/- TPN), ursodeoxycholic acid to enhance bile flow, +/- liver transplant in cases of severe liver failure
116
Q

infant age 4-6 weeks presents with jaundice, dark urine, and pale/acholic stools
-bilirubin levels are moderately elevated

A

biliary atresia- progressive fibrosclerotic dz that affects the extrahepatic biliary tree
-progresses quickly with bile duct obliteration and cirrhosis by 4 months

117
Q

how to dx biliary atresia

A
  • US, HIDA, and liver bx to r/o other causes

- intraop cholangiogram to confirm the diagnosis

118
Q

how to tx biliary atresia

A

Kasai portoenterostomy by age 50-70 days at the latest (success decreases with age)- cholangitis is a complication

  • liver transplant for liver failure
  • supportive care
119
Q
  • debilitating pruritus
  • broad forehead, deep set and wide spaced eyes, saddle nose, pointed chin, large ears
  • pulmonary outflow obstruction
  • renal disease
  • posterior embryotoxon of the eye
  • butterfly vertebrae, broad thumbs
  • growth failure, short stature
  • pancreatic insufficiency
  • hypercholesterolemia
  • cholestatic liver disease
A

Alagille syndrome- AD disorder characterized by paucity of intrahepatic bile ducts and multi-organ involvement

  • perhaps something on chromosome 20
  • dx based on clinical features
  • management is supportive
120
Q

MC viral hepatitis infections in kids

A

hep A and B

-most infections in childhood are asymptomatic

121
Q

MC hepatitis virus causing infection
how is it transmitted
clinical presentation

A

hep A
fecal oral
most kids are asymptomatic; teens and adults more likely to have sxs

122
Q

how to dx and tx hep A (HAV)

A

dx with serology
-IgM anti-HAV: early in infection-6months
-IgG anti-HAV: early in infection-lifelong immunity
tx with supportive care

123
Q

transmission of HBV

A

perinatal vertical

IVDU, tattoos, blood products, sex

124
Q

chronic hep B infection is more likely in _______

what are the sxs?

A

younger infants

sxs: wide range ranging from asymptomatic to fulminant liver failure

125
Q 
hep B serology
HBsAg
HBsAb
HBcAb
HBeAg
HBeAb
A

HBsAg- pathognomonic for active disease
HBsAb- protective, can result from vaccine or natural infection
HBcAb- results from natural infection (not vaccine) and persists lifelong
HBeAg- rises early in active infection, useful for diagnosing acute ifxn
HBeAb- rises late in infection

126
Q

tx for HBV infection

A

supportive care for acute infection

interferon and antivirals for chronic infection

127
Q

HCV transmission

A

perinatal vertical

IV exposure- accounts for 90% of transfusion-associated hepatitis

128
Q

Although acute infection is rarely symptomatic (esp in children), HCV is more likely than the other hepatitis viruses to cause chronic infection (T/F)

A

T

80% end up being chronically infected

129
Q

how to dx HCV

A

HCV antibody or PCR

130
Q

Hepatitis D requires ______ for replication

A

HBsAg

131
Q

Hepatitis E is transmitted ______ and is especially dangerous in ______

A

fecal-oral

pregnant women

132
Q

progressive liver dz characterized by elevated serum transaminases, hypergammaglobulinemia, and circulating autoantibodies
-whats the classic demographic

A

autoimmune hepatitis

females with presentation before puberty

133
Q

2 types of autoimmune hepatitis

A

type 1- ANA or ASMA- this type is more common

type 2- anti-LKMA or anti-liver cytosol type 1 antibody

134
Q

how does autoimmune hepatitis first present

A

50% acute hepatitis

50% chronic liver disease

135
Q

how to tx autoimmune hepatitis

A

steroids for acute control of inflammation

imunosuppressives for long term control: azathioprine, 6-mercaptopurine

136
Q

heterotaxy puts you at risk for malrotation (T/F)

A

T

137
Q

maintenance water requirements

A

100 mL/kg/day for first 10kg
50 mL/kg/day for second 10kg
20 mL/kg/day for each kg above the first 20kg

138
Q

maintenance Na requirement

A

2-3 mEq/kg/day

139
Q

maintenance K requirement

A

2 mEq/kg/day during infancy but decreases with age

140
Q

what to give for emergency phase of treating dehydration

A

20 mL/kg boluses of 0.9% NaCl or LR

this is the same for all pts, regardless of initial serum Na levels

141
Q

define hematuria

microscopic hematuria

A

presence of RBCs in the urine

> 6 RBCs/hpf is microscopic hematuria

142
Q

Urine dipstick also detects hemoglobin or myoglobin in the urine (T/F)
what might cause a false negative for blood in the urine

A 
T
vitamin C (ascorbic acid) ingestion
143
Q

RBC casts indicate bleeding from the _____

A

glomerulus

144
Q

acute hemorrhagic cystitis can be caused by bacterial infections, viral infections like _____, and drugs like ______

A

adenovirus

cyclophosphamide

145
Q

proteinuria > ______ is considered pathologic

A

100 mg/m2/day

146
Q

urine dipstick tests for this type of protein

A

albumin

147
Q

normal urine TP/CR

A

infants 6-24 months: < 0.5

children > 2 years: < 0.2

148
Q

kids who have increased urinary protein excretion while upright but not while supine
what is it and how to dx for sure

A

orthostatic proteinuria

  • morning urine protein should be normal, afternoon urine protein should be high
  • this is benign
149
Q

two origins of pathologic proteinuria and which one is more common?

A

glomerular- increased permeability, this is more common

tubular- decreased reabsorption

150
Q

lab findings in tubular proteinuria

A

urinary beta2-microglobulin (normally freely filtered at the glomerulus and then completely reabsorbed)
glucosuria
aminoaciduria

151
Q

lab finding in glomerular proteinuria

A

increased urinary microalbumin

152
Q

primary vs. secondary glomerulonephritis

A

primary- limited to kidney

secondary- 2/2 a systemic disease

153
Q

gross hematuria, HTN, signs of fluid overload from renal insufficiency

A

nephritic syndrome

154
Q

heavy proteinuria, hypercholesteremia, edema

A

nephrotic syndrome

155
Q

most common form of acute GN in school age children

A

poststrep GN

156
Q

timeline of post strep GN

A

8-14 days after skin or pharynx infection

21-28 days after impetigo

157
Q

clinical presentation of post strep GN

A

hematuria (often gross), proteinuria (but not as much as nephrotic syndrome), HTN with signs of fluid overload like edema

158
Q

labs in post strep GN

A

low serum complement (C3) which normalizes in 8-12 weeks
ASO titer- sensitive after pharyngitis but not impetigo
ADB titer- good for after respiratory or skin infections

159
Q

you don’t routinely bx in post strep GN, but if you did, what would you see

A

mesangial cell proliferation and increased mesangial matrix

160
Q

tx for post strep GN

prognosis

A
  • supportive care: fluid restriction, anti-HTN, dietary restriction of protein/Na/K/phosphorus
  • prognosis is excellent
161
Q

treating strep infections with abx prevents post strep GN from occurring (T/F)

A

F

it only prevent rheumatic fever and PANDAS

162
Q

most common type of chronic GN worldwide

-recurrent bouts of gross hematuria associated with respiratory infections

A

IgA nephropathy

163
Q

renal bx results of IgA nephropathy

A
  • mesangial proliferation and increased mesangial matrix

- mesangial deposition of IgA

164
Q

how to tx IgA nephropathy

what’s the prog

A
  • supportive
  • meds (steroids, ACEI) for pts with pathologic proteinuria or renal insufficiency
  • prognosis: variable… 20-40% end up with ESRD
165
Q

nonthrombocytopenic palpable purpura on the buttocks and thighs, abdominal pain, arthritis and arthralgias, gross or microscopic hematuria

A

Henoch-Schonlein purpura (HSP) nephritis- IgA mediated vasculitis

166
Q

HSP nephritis prognosis

A

in most cases, renal features are self limited with complete recovery within 3 months
in 1-5%, pts develop chronic renal failure

167
Q

common types of GN in children

A 
post strep 
IgA nephropathy (Berger's disease)
HSP nephritis 
membranoproliferative GN (MPGN)
membranous nephropathy (MN)
SLE nephritis
168
Q

lobular mesangial hypercellularity and thickening of the GBM

  • nephritis or nephrotic syndrome with hematuria
  • HTN
  • low serum complement
  • most pts develop ESRD
A

membranoproliferative GN (MPGN)

169
Q

how to tx MPGN

A

no definitive tx

can try steroids and ACEI

170
Q

rare form of GN in young children….heavy proteinuria which progresses to renal insufficiency

A

membranous nephropathy (MN)

171
Q

heavy proteinuria (> 50mg/kg/24 hours)
hypoalbuminemia
hypercholesterolemia
edema

A

nephrotic syndrome

172
Q

3 categories of nephrotic syndrome

A
  1. primary NS- not due to systemic disease, 90% of childhood NS
  2. NS from other primary glomerular disease (IgA nephropathy, MPGN, PSGN)
  3. NS from systemic diseases like SLE and HSP
173
Q

MCC primary nephrotic syndrome

A

minimal change disease (MCD)

174
Q

typical presentation of nephrotic syndrome in kids

A

most present with edema following a URI

175
Q

2 things you need to watch out for in kids with nephrotic syndrome (besides the usual stuff)

A
  1. predisposition to thrombosis 2/2 hypercoagulability –> stroke, DVT, renal vein thrombosis, sagittal sinus thrombosis
  2. increased risk of infection with encapsulated organisms like streo pneumo –> SBP, pneumonia, over whelming sepsis
176
Q

UA in nephrotic syndrome would show:

A

3+ to 4+ protein

elevated urinary TP/CR

177
Q

CBC in nephrotic syndrome shows:

A

elevated hematocrit and/or elevated plts 2/2 hemoconcentration resulting from hypoproteinemia

178
Q

management of nephrotic syndrome

A
  • most children are hospitalized for initial treatment
  • if widespread edema, hypotension, or symptomatic pleural effusion –> IV 25% albumin to achieve diuresis and maintain intravascular volume
  • no added salt diet
  • most kids with MCD respond to steroids; if not, try cyclophosphamide or cyclosporine
  • be vigilant about encapsulated infections
179
Q

prognosis of nephrotic syndrome

what typically causes mortality

A
  • depends on underlying etiology
  • mortality usually 2/2 infection or thrombosis
  • if steroid sensitivie, most end up ok
  • if steroid resisant, most develop FSGS and ESRD
180
Q

acute renal failure with MAHA and thrombocytopenia

A

HUS

181
Q

2 types of HUS… which is more common

A

shiga toxin-associated HUS –> more typical

atypical HUS

182
Q

most common pathogen that causes HUS

A

E Coli 0157:H7

others include E Coli other strains and shigella dysenteriae type 1

183
Q

classic presentation of shiga toxin-associated HUS

A

bloody diarrhea followed by sudden onset of hemolytic anemia, thrombocytopenia, and acute renal failure

184
Q

how to tx shiga toxin-associated HUS

A
  • transfusions as needed for anemia and thrombocytopenia
  • do not give abx… in fact, if you give abx for E. coli hemorrhagic colitis, you may increase likelihood of getting HUS
185
Q

prognosis of shiga toxin-associated HUS

what are some complications

A

prognosis is generally favorable
poor prog signs: high WBC on admission, prolonged oliguria
complications may be toxic megacolon, cerebral infarctions, etc

186
Q

2 causes of atypical HUS

A

drugs (OCP, cyclosporine, tac, OKT3)

inherited

187
Q

presentation, management, and prog of atypical HUS

A
  • no diarrhea; severe proteinuria and HTN are common
  • tx is supportive
  • prog: some pts have recurrent HUS… compared to shiga toxin-associated HUS, these pts have a higher risk of progressing to HUS
188
Q

progressive hereditary nephritis that’s secondary to defects in the side chains of type IV collagen within the GBM

A

Alport’s syndrome- X linked dominant

189
Q

clinical features of Alport’s

A

HTN, hematuria, ESRD
hearing loss
ocular problems involving the lens and retina

190
Q

how to manage Alport’s

A

treatment of HTN
ACEI
eventual kidney transplant

191
Q

MCC renal mass in the newborn

A

multicystic renal dysplasia

192
Q
  • maternal h/o oligohydramnios secondary to nonfunctioning or poorly functioning kidneys
  • pulmonary hypoplasia
  • greatly enlarged cystic kidneys
  • severe HTN
  • usually has some liver involvement
A

autosomal recessive polycystic kidney disease (ARPKD) or infantile polycystic kidney disease
-it is progressive and will eventually require transplant

193
Q

-abdominal pain, flank masses, UTI, hematuria, severe HTN, renal insufficiency in an adult

A

ADPKD

194
Q

prognosis of ADPKD

A

most develop severe HTN and require transplant

watch out for cerebral aneurysms

195
Q

this inherited kidney disease has 2 forms

A

nephronophthisis-medullary cystic disease complex (NPH-MCD)
juvenile form- AR, ESRD in childhood
adult form- AD, ESRD later in life

196
Q

medullary sponge kidney is caused by ______

A

inheritance

197
Q 
definitions:
normal HTN
significant HTN
severe HTN
malignant HTN
A

normal HTN: 90-95th percentile
significant HTN: >95% percentile
severe HTN: >99% percentile
malignant: assoc with evidence of end-organ damage

198
Q

most HTN in childhood is _____ (primary/secondary)

A

secondary

199
Q

common causes of HTN based on age

  • neonates and young infants
  • age 1-10 years
  • adolescents
A
  • renal artery embolus after umbilical artery catheter placement, coarctation of aorta, congenital renal disease, renal artery stenosis
  • renal dz, coarctation of aorta
  • renal disease, essential HTN
200
Q

what to look for on PE of kids with HTN

A

four limb BP to look for coarctation
funduscopic exam
look for CHF, cafe au lait (neurofibromatosis), abdominal masses, abdominal bruits, ambiguous genitalia

201
Q

goal of chronic HTN management

A

be below 90th percentile

202
Q

clinical presentation of RTA in kids

A

infants and young children- growth failure, vomiting

older children and adults- recurrent calculi, muscle weakness, bone pain, myalgias

203
Q

RTA: inability of the distal renal tubular cells to excrete H+
-vomiting, growth failure, acidosis, nephrocalcinosis and nephrolithiasis

A 
distal RTA (type I)
tx with small doses of oral alkali
204
Q

RTA: impaired bicarb reabsorption by the proximal rental tubular cells
-vomiting, growth failure, acidosis, muscle weakness

A 
proximal RTA (type II)
tx with large doses of oral alkali
205
Q

RTA: transient acidosis in infants and children, hyperkalemia is hallmark
-may be asymptomatic, can present with FTT

A

type IV RTA

-tx with furosemide to get rid of K and also oral alkali

206
Q

proximal RTA, hyperphosphaturia, aminoaciduria, glucosuria, K wasting

A

Fanconi syndrome

207
Q

classic electrolyte finding in RTA

what’s the urine anion gap like in distal RTA

A

non-anion gap hyperchloremic metabolic acidosis

+ urine anion gap in distal RTA

208
Q

oliguris in kids is defined as

A

< 1mL/kg/hr

209
Q

management of renal failure

A
  • restore intravascular volume first
  • then restrict fluid intake to patient’s insensible losses plus urine and stool output
  • restrict Na, K, phosphorus, protein intake
  • give oral phosphate binders and vitamin D analogs
  • tx anemia with iron and EPO
  • may need dialysis or transplant
210
Q

FeNa formula

A

FeNa = (urine Na/serum Na)/(urine Cr/serum Cr) x 100%

211
Q

when to consider dialysis or renal xplant

which dialysis to use

A

when GFR is 5-10% of normal
peritoneal dialysis preferred in kids
overall, xplant is the preferred tx for kids with ESRD

212
Q

causes of bladder outlet obstruction

A

posterior urethral valves in males
polyps
prune belly syndrome (absence of rectus muscles, bladder outlet obstruction, cryptorchidism in males)

213
Q

renal agenesis is 2/2 failure of development of _____ or _____

A

mesonephric duct or the metanephric blastema

214
Q

_________ is associated with altered structural organization of the kidney
functionally, it’s assoc with concentrating defects, RTA, and renal insufficiency

A

renal dysplasia

215
Q

fusion of lower poles of kidney

A

horseshoe kidney

216
Q

kidney located outside of renal fossa

A

renal ectopia

217
Q

_______ is identified in 30-50% of infants and young children with UTIs

A

vesicoureteral reflux

218
Q

how is VUR inherited

A

AD with variable expression

219
Q

VUR, if prolonged, may lead to ______ and ______

A
  • pyelonephritis

- reflux nephropathy- ESRD, HTN, renal scars, contraction, interstitial nephritis

220
Q

children with reflux usually need surgery to correct it (T/F)

A

F

most kids wit lower grades (1-2/5) eventually have spontaneous resolution of reflux

221
Q

how to dx VUR

A

voiding cystourethrogram

222
Q

how to manage VUR

A

ppx abx until kid outgrows the VUR

if grade 4-5, consider surgical re-implantation of the ureters

223
Q

renal stones are common in children and you can just treat with hydration (T/F)

A

F- they are uncommon in children and you should seek out predisposing metabolic disorders

224
Q

if renal stone is due to hypercalciuria, look for these conditions

A

hypercalcemia
familial hypercalciuria
furosemide use

225
Q

if renal stone is due to hyperoxaluria, look for these

A 
inherited d/o
enteric malabsorption (ex. IBD)
226
Q

if renal stone is due to hyperuricosuria, look for these

A

tx of leukemia or lymphoma
Lesch-Nyhan syndrome
primary gout

227
Q

if renal stone is due to cystinuria

A

look for cystinuria- AR disorder leading to radiopaque stones

228
Q

if renal stone caused by UTI, look for this organism

A

proteus mirabilis

229
Q

tx of renal stones

A

hydration
relief of obstruction
tx UTI if resent
specific therapy depends on which type of stone it is

230
Q

demographics of those at risk for UTI

A

< 6 months: uncircumcised boys

> 6 months: girls

231
Q

common bacteria causing UTI

A

E Coli

klebsiella, pseudomonas, staph saprophyticus (teen girls), serratia, proteus (alkaline urine), enterococcus

232
Q

how to collect urine for UA and culture

A

neonates and infants: suprapubic aspiration or via sterile urethral cath
older children: clean catch

233
Q

UA of UTI

A

leukocytes (>5-10 WBCs/hpf)

+ nitrite or leukocyte esterase

234
Q

urine culture for UTI

what are the cutoffs for + result?

A

any growth if suprapubic aspiration
> 10,000 colonies if sterile urethral catherization
> 50,000 - 100,000 if clean catch

235
Q

when should you image (renal US and VCUG) for UTI

A

recurrent UTI
all males
girls < 4 years of age

236
Q

how to tx UTI

A
  • empiric abx while cx is pending: bactrim or keflex (cephalexin)
  • neonates or toxic-looking children with UTI are admitted for IV abx: ampicillin and gentamicin
  • duration: 7-10 days for cystitis, 14 days for pyelo
237
Q

2/2 risk of renal scarring after pyelo in infants, give ppx abx for ______ after an episode of pyelo

A

3 months

238
Q

microscopic hematuria is usually > ______

A

6 RBCS/hpf for 3 or more consecutive urine samples

239
Q

maintenance fluid should be increased by 12% for every degree above 38 degrees C (T/F)

A

T

240
Q

_______ have a greatly increased risk of renal vein thrombosis

A

infants of diabetic mothers

241
Q

maternal SLE may result in infant ______

A

heart block

Pediatrics (14 decks)

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