peds34 Flashcards Preview

Peds > peds34 > Flashcards

Flashcards in peds34 Deck (100):
1

how does hypercholesterolemia happen in NS?

reduced plasma oncotic pressure induces increased hepatic production of plasma proteins, including lipoproteins; plasma lipid clearance is reduced because decr lipoprotein lipase in adipose tissue

2

clinical features of NS

edema following an URI; patients are predisposed to thrombosis;

3

patients with NS at risk for what?

thrombosis and infection with encapsulated organisms (strep pnumona) so may present with bacterial peritonitis, pneumonia, or sepsis

4

what does CBC show in NS

elevated hematocrit as a resut of hemoconcentration due to decr protein; platelet count may be elevated

5

why would you get a metabolic acidosis in NS?

renal tubular acidiosis

6

NS on renal u/s

enlarged kidneys

7

management of NS?

IV infusions of albumin to treat edema; no salt diet; steroids (or cyclophosphamide or cyclosporine if don't respond); if child is febrile, do blood and urine culture and IV antibiotic if needed

8

prognosis of NS

mortality about 5%, usually from infection or thrombosis; mortality in kids who are steroid-resistant;

9

ESRD in NS kids?

the majority of kids who are steroid-resis develop but the majority of kids who are steroid sens do not develop

10

Hemolytic uremic syndrome

acute renal failure in the presence of microangiopathic hemolytic anemia and thrombocytopenia

11

two diff subtiypes of HUS

shiga toxin asociated and atypical HUS

12

most common HUS subtype seen in kids

shiga-toxin associated

13

most common pathogen to cause shiga-txin HUS

e coli

14

how does shiga toxin cause HUS

vascular endothelial injury, leads to platelet thrombi formation and renal ischemia

15

clinical features of shiga toxin hus?

diarrheal prodrome (often bloody and may be severe) followed by suden onset of hemolytic anemia, thrombocytopenia and acute renal failure

16

management of shiga toxin HUS

mostly supportive; transfusion for severe anemia and thrombocytopenia; anitbiotics not indicated

17

prognosis for shiga toxin HUS

generally good but poor prognostic indicators are high WBCs and prolonged oliguria;

18

if patients do die from shiga HUS, what do they die of?

complications of colitis lke toxic megacolon or from CNS complications like cerebral infarction

19

causes of atypical HUS

drugs (OCPs, cyclosporine, tacrolmus); inherited

20

clinical features of atypical HUS

same as shiga HUS but no diarrhe

21

treatment of atypical HUS

supportive

22

prognosis of atypical HUS

some patients have a chronic relapsing course; all patients with atypical HUS have a higher risk of progression to ESRD than shiga HUS

23

alport's syndrome

progressive nephritis secondary to defects in type 4 collagen within the glomerular basement membrane

24

inheritance of alport

x linked dome

25

other non-renal manifestations of alports

hearing loss that begins in childhood and is progressive; ocular abnormalities

26

management of alport

ace inhib initially; eventually renal transplant

27

multcystic renal dysplasia

most common cause of renal mass in the newborn; most often unilateral

28

autosomal recess polycystic idney disease or infantile polycystic kidney disease

uncommon; cystic kidneys with severe htn; liver involvement (cirrhosis) w portal htn can be seen

29

infants with ARPKD may have in utero hx of

oligo hydramnios, leading to pulm hypoplasia

30

prognosis of ARPKD

progressive and ultimately all patients require renal transplant

31

Aut dom PKF or adult polycystic disease

common; presents in adulthood

32

prognosis for ADPKD

sever htn and renal insuff; require transplant

33

medullary sponge kidney

occurs sporadically or may have aut dom inheritance; patient maybe symp or asymptomatic

34

nephronophthisis-medullary cystic disease complex

juvenile form is aut recessive and leads to ESRD in childhood

35

malig htn

htn associated with evidence of end organ damage

36

essential htn

htn without a clear etiology

37

most htn in kids is secondary htn or essential htn?

secondary

38

most common causes of htn in neonates

renal artery embolus after umbilical artery catheter placement; coarctation of the aorta; congenital renal disease, renal artery stenosis

39

most common causes of htn in ages 1-12 yrs

renal disease and coarctation of aorta

40

most common cause of htn in adolescents

renal disease and essential htn

41

what fundoscopic findings indicate htn?

retinal hemorrhages, papilledema, arterial-venous nicking

42

how to identify renal artery stenosis

radioisotope renal scan w administration of captopril or renal angiography

43

captopril

ACE-inhib

44

renal tubular acidosis

inability of the kidney to maintain normal acid/base balance because of defects in biacrb conservation or bc of defects in excretion of H ions

45

younger kids RTA presentation

growth failure and vomitting, and sometimes w life threatening metabolic acidosis

46

older kids RTA

recurrent calculi, muscle weakness, bone pain, and myalgias

47

how can you get polyuria in RTA

some forms of RTA lead to nephrocalcinosis which gives you urinary concentrating defects

48

electrolyte presentation in RTA

hyperchloremic metabolic acidosis with a normal serum anion gap

49

calculate anion gap

Na+ K+- Cl

50

when is a pos URINE anion gap seen?

distal RTA

51

distal RTA (type 1)

inability of distal renal tubules to excrete H+

52

proximal RTA (type 2)

impaired bicarb reabsoprtion y the proximal renal tubules

53

RTA type 3

variant of type 1, complicated by proximal tubular bicarb wasting during infancy

54

type 4 RTA

transient acidosis in infants and kids; hyperkalemia is the hall mark

55

treatment for RTA

type 1- small doses of oral alkali; types 2 and 3- large doses of oral alkali

56

treamtnet for type 4 RTA

furosemid to lower serum potassium, oral alkali

57

diffuse tubular disorder

manifested by hypokalemia, hypophosphatemia, and aminoacidduria, think Fanconi syndrome

58

oliguria

less than 1 mL/kg/hr

59

renal osteodystrophy

bone disease secondary to renal failure

60

nutritional management in chronic kidney disease

avoid high phosphorous, high sodium, and high potassium foods; patients need oral phosphate binder and vit D analogs to prevent renal osteodystrophy

61

when is dialysis or transplant indicated?

when GFR is 5-10% of normal

62

preferred dialysis in kids

peritoneal dialysis; but kidney transplant is the preferred treatment in kids with esrd

63

most common cause of transplant loss

acute and chronic rejection, noncompliance with meds, technical problems during surgery, and recurrent disease

64

causes of bladder outlet obstruction?

posterior urethral valves (in males), polyps, or prune belly syndrome

65

prune belly syndrome

absence of rectus muscles, bladder outlet obstruction, and, in males, cryptochoridsm

66

most common abdominal mass in newborns

multicystic dysplastic kidney, which is usually associated with atretic ureter

67

vesicoureteral refleux

urine refluxing from the urinary bladder into the ureters and the renal collecting system

68

VUR is most commonly associated with what condition?

UTIs

69

VUR genetics?

aut dom inheritance with varibale expression

70

grades of VUR

grade 1- reflux into distal ureter; grade 2- into renal pelvis and calyces w/o dilation; grade 3 is into calyces w dilateion; grade 4 clubbed calyces; grae 5 gross dilation of entire system

71

diagnosis of VUR

voiding cystourethrogram, in which contrast is introduced into the urinary bladder and bladder and kidneys are imaged during voiding

72

management of VUR

low dose prophylactic antibiotics until kid outgrows the VUR; kids with grade 4 or 5 reflux should be referred to urologist

73

urolithiasis

uncommon in kids; but most common stones seen in childhood are calcium, uirc acid, cystein, and mag ammonium phosphate (struvite)

74

what conditions are associated with renal stones

hypercaliuria, hyperoxaluria, distal RTA, hyperuricosuria, cystinuria, UTI, hyperPTH

75

what can cause hyperoxaluria

can be inherited or secondary to enteric malabsorption (inflamm bowel disease)

76

how can hyperuriosuria occur?

during the treatment of leukemia or lymphoma, with Lesch-nyhan syndrome, or primary gout

77

lesch-nyhan syndrome

def in an enzyme that affects how your body breaks down purines

78

UTIs and gender

more common in boys until 6 months of age, then more common in girls

79

circumcison and UTIs

before 6 months, UTIs are 10x more common in uncircumcised boys than those who are circumcised

80

proteus causign URI

associated w high urinary pH

81

urinalysis findings suggestive of UTI

leukocytes (>5-10 WBC/hpf) and a pos nitirite or leukocyte esterase on dipstick

82

who should have an imaging eval with UTI? (renal u/s and voiding cystourethrogram)

pyelonephriitis, recurrent UTI, all males, all girls less than 4 with cystitis,

83

management for uti

empiric antibiotic therapy; neonatesadmitted to hospital for IV managemen; neonates alaso get low-dose prophylactic antibitoics for at least 3 months

84

duration of treatment of UTI

10 daysl but 14 days for pyelonephritis

85

renal vein thrombosis

unilateral flank mass

86

central vs peripheral hypotonia

central is UMN; peripheral is LMN

87

associated with peripheral hypotonia

decreased fetal movements and breech presentation

88

associated with central hypotonia

seizures in the neonatal period

89

consciousness in central vs peripheral hypotonia

central has an altered level of consciousness and incr DTRs, often w ankle clonus; perip- consciousness not affected but decr DTRs and muscle bulk

90

causes of hypotonia

systemic pathology (sepsis, etc.) or neural pathology

91

what imaging do you do when you suspect central hypotonia?

head CT

92

spinal muscular atrophy

anterior horn cell degeneration and infiltration of microglia and astrocytes; presents with hypotonia, weakness, and tongue fasciculations

93

second most common hereditary neuromuscular disorder

SMA

94

most common inherited neuromusc disorder

duchenne muscular dystrophy

95

types of spinal muscular atrophy

type 1 is infantile form with onset less than 6 mos; type 2 or intermediate form is onset 6-12 mos; type 3 or juvnile form with onset greater than 3 years

96

inheritance of spinal muscular atrophy

aut recessive

97

genetics of SMA

aut recessive, all three types caused by mutation in survival motor neuron gene (SMN1) on chrom 5

98

clinical features of SMA

weak cry, tongue fasciculations, and difficulty sucking and swallowing; bell shaped chest; frog leg poster; normal extraocular movements and norman sensory exam

99

what does muscle biopsy of kid with SMA show?

atrophy of muscle fibers that were innervated by the damaged azons

100

management of SMA

supportive