BRS #4 Flashcards
how many AAs are essential
9
what are essential nutrients
stuff that cannot be made by the body
you have to take it in from your diet
infants require more _____ in their diet
protein
_______ play an important role in infant brain development
essential fatty acids
most common energy depletion state
- near starvation from protein and nonprotein deficiencies
- very thin from loss of muscle and body fat
marasmus
- 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
kwashiorkor
vitamin and mineral deficiency
-night blindness, xerophthalmia (dry conjunctiva and cornea)
vitamin A
vitamin and mineral deficiency
rickets/osteomalacia, dental caries, hypocalcemia, hypophosphatemia
vitamin D
vitamin and mineral deficiency
anemia/hemolysis, neurologic deficits, altered prostaglandin synthesis
vitamin E
vitamin and mineral deficiency
coagulopathy/prolonged PT, abnormal bone matrix synthesis
vitamin K
vitamin and mineral deficiency
berberi (cardiac failure, peripheral neuropathy, hoarseness or aphonia, wernicke’s encephalopathy)
vitamin B1 (thiamine)
vitamin and mineral deficiency
dermatitis, cheilosis, glossitis, microcytic anemia, peripheral neuritis
vitamin B6 (pyridoxine)
vitamin and mineral deficiency
megaloblastic anemia, demyelination, methymalonic acidemia
vitamin B12 (cobalamin)
vitamin and mineral deficiency
scurvy (hematologic abnormalities, edema, spongy swelling of gums, poor wound healing, impaired collagen synthesis)
vitamin C
vitamin and mineral deficiency
megaloblastic anemia, neutropenia, impaired growth, diarrhea
folic acid
vitamin and mineral deficiency
pellagra (diarrhea, dermatitis, dementia), glossitis, stomatitis
niacin
vitamin and mineral deficiency
skin lesions, poor wound healing, immune dysfunction, diarrhea, growth failure
zinc
what do you find in the stool if there carbohydrate malabsorption
- watery
- acidic (pH < 5.6)
- reducing substances detected via a positive clinitest reaction
congenital enterokinase deficiency is a rare cause of _____ and ______ loss in the stool
what results?
protein and nitrogen
hypoproteinemia –> edema and growth impairment
what happens in protein losing enteropathies
transudation of protein from inflamed intestinal mucosa
what test to look for protein in the stool
fecal alpha-1-antitrypsin levels
decreased _____ activity results in steatorrhea, decreased absorption of fat-soluble vitamins (A, D, E, K)
lipase
acanthocytosis of erythrocytes is seen in _________
abetalipoproteinemia
AR syndrome
pancreatic exocrine insufficiency, FTT, short stature, neutropenia, sometimes pancytopenia
Schwachman-Diamond syndrome
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??
cow’s milk protein
most protein intolerance is transitory and resolves by 1-2 years of age
celiac disease is an autoimmune disease in the _________ 2/2 intolerance to gluten
proximal small intestine
how to dx celiac disease
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
noncompliance with gluten free diet in adolescence can cause:
growth failure and delayed sexual maturity
after gut resection, ________ and ______ malabsorption with steatorrhea are common
distal small bowel (ex. ileum) limits _____ and ______ resorption
carbohydrate and fat
vitamin B12 and bile acid
how to manage short bowel syndrome
- TPN
- early enteral feedings
- small bowel transplant only with life-threatening TPN-associated liver disease
complications of short gut syndrome
TPN cholestasis intestinal bacterial overgrowth nutritional deficiencies poor bone mineralization renal stones secretory diarrhea
GER vs. GERD
GER- normal physiologic state, happy spitters, emesis is benign
GERD- pathologic state associated with GI or pulm sxs and sequelae
sandifer syndrome
torticollis with arching of the back caused by painful esophagitis
if you have sxs of GERD past 1 year of age, it is unlikely to resolve by itself (T/F)
T
how to dx GERD
- 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
how to tx GERD
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)
- first born Caucasian male with projectile vomiting of nonbilious milky fluid in weeks 2-3
- irritable but hungry
pyloric stenosis
physical and lab exam of pyloric stenosis
- 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
how to dx pyloric stenosis
- *US
- can also see string sign on UGI
how to tx pyloric stenosis
correct electrolytes and dehydration then partial pyloromyotomy
midgut volvulus involves the midgut twisting around the ______
superior mesenteric vessels –> ischemic and obstruction
malrotation is more common in _____ (boys/girls)
boys
lack of small bowel fixation can result in these compressing the duodenum and causing mechanical obstruction
peritoneal bands (Ladd’s bands)
classic presentation of volvulus
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
how to evaluate for volvulus
- xrays: proximal intestinal distention and obstruction
- UGI is the diagnostic tool of choice
- lower intestinal contrast studies sometimes
how to tx volvulus
surgical emergency!
fluids, NG suction, abx
________ is the MCC obstruction in the neonatal period
it happens more in ______ (boys/girls)
intestinal atresia
boys
duodenal atresia is sometimes associated with ______
Down syndrome
duodenal atresia
- prenatal US may show:
- PE may show:
gastric dilation with polyhydramnios
scaphoid abdomen with epigastric distention
xray finding in duodenal atresia
what else can you do?
double bubble sign
contrast study
how to tx duodenal atresia?
NG tube, hydration, correct electrolytes
duodenoduodenostomy
jejunoileal atresia is like duodenal atresia except….
they are caused by mesenteric vascular accidents and are not associated with other anomalies
what is intussusception
optimal age
most common location
telescoping of a proximal portion of intestine into a more distal portion
ages 5-9 months
ileocolic
_____ is the MCC bowel obstruction after the neonatal period but less than 2 years of age
intussusception
- sudden onset of crampy or colicky abdominal pain that occurs in intervals
- infants may draw legs toward the chest
intussusception
PE findings of intussusception
currant jelly stool
sausage shaped mass palpated in the RUQ
how to dx and tx intussusception
what’s a classic sign that you see?
air or contrast enema to dx and tx- you see coil spring sign
if that doesn’t work, you can operatively reduce it
most common pediatric emergency operation
apendectomy
peak age of appendicitis
10-12 years
appendicitis classically presents with peri-umbilical pain that migrates to _________
McBurney’s point
how to dx and tx appendicitis
US or CT but sometimes might go straight to surgery
fluids, periop abx, appendectomy
Gray Turner and Cullen signs and what they are assoc with
Grey Turner- bluish discoloration of flanks
Cullen- bluish discoloration of the periumbilical area
assoc with severe cases of pancreatitis
pancreatitis is ______ (common/uncommon) in children and is often caused by _______ and ______
uncommon
trauma (most common), infection, idiopathic (2nd most common)
how to evaluate pancreatitis
- 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.
how to manage pancreatitis
supportive care
TPN if needed
abx for necrotizing pancreatitis
+/- surgery but only when the inflammation has calmed down
2 complications of pancreatitis
ARDS
pseudocyst
acute cholecystitis is ______ (common/uncommon) in children and can be caused by ______, _______, or ________
uncommon
sickle cell disease, CR, or prolonged TPN therapy
what’s the biggest cause of acute acalculous cholecystitis
infection with salmonella, shigella, E. coli
cholecystitis PE shows ______
Murphy’s sign and RUQ pain
how to dx cholecystitis
RUQ ultrasound
how to tx cholecystitis
fluids, abx, analgesia, cholecystectomy once stabilized
chronic abdominal pain is pain that occurs each month for ____
3 consecutive months
chronic epigastric pain
belching, bloating, nausea, vomiting, early satiety
like adult nonulcer dyspepsia
chronic periumbilical pain
age and characteristics
classic functional abdominal pain (FAP)
age > 5 years
pain is varied in character and does not interfere with pleasurable activities
chronic infraumbilical pain
abdominal cramping, bloating, alterations in stool
like adult IBS
how to evaluate chronic abdominal pain
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
how to treat functional abdominal pain
what’s the prognosis
normalize child’s activities, educate the parents
counseling
symptomatic meds don’t really work
prognosis is poor– many have chronic abdominal pain as adults
encopresis is almost always associated with ______
severe constipation
liquid stool leaks around the hard retained stool
initially, ______ (breastfed/formula-fed) infants defecate more frequently
breastfed
_______ is the most common form of constipation during childhood and is usually due to ______
functional fecal retention (FFR)
some sort of traumatic event
MCC organic constipation in an otherwise healthy child
Hirschsprung’s disease
signs that point towards organic constipation
delayed passage of meconium (>48 hours), onset in infant, h/o pelvic surgery, encopresis before age 3 years, inability to toilet train
how to manage constipation in kids
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
systemic causes of organic constipation
dehydration, hypothyroidism, cystic fibrosis
IBD age of onset is _____
bimodal (teens and then 50s)
in ulcerative colitis, inflammation is limited to the ___ and localized to the _____
it starts in the ______ and extends proximally in a continuous fashion
mucosa, colon
rectum
complications of UC
toxic megacolon- fever, abdominal distention, septic shock, perf
increased risk of colon cancer
extraintestinal manifestations of UC
uveitis, arthropathy, *pyoderma gangrenosum, sclerosing cholangitis
serologic antibody in UC
antineutrophil cytoplasmic antibody
inflammation of any segment of the GI tract
- skip lesions
- transmural inflammation
Crohn’s disease
most common area for Crohn’s in kids
terminal ileum
some intestinal sxs of Crohn’s
- pain, decreased growth, cramping, diarrhea
- small bowel disease –> malabsorption of B12, iron, zinc, folate
- perianal disease
extraintestinal manifestations of Crohn’s disease
-more common in Crohn’s vs. UC
FTT delayed sexual development oral aphthous ulcers erythema nodosum arthritis renal stones
serologic antibody in Crohn’s
anti-saccharomyces cerevisiae antibody
complications of Crohn’s disease
fistula, stricture, abscess
risk for colon cancer is less than for UC
how to work up IBDD
CBC: anemia, leukocytosis ESR: high albumin, LFTs, antibody tests US or CT or UGI colonoscopy with biopsies confirm the diagnosis
treatment for IBD
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
surgery for IBD
- 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
_________ for dx and tx for active UGI bleeding with hemodynamic changes
upper endoscopy
managing UGI bleed
- 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
premie with rectal bleeding, feeding intolerance, abdominal distention
necrotizing enterocolitis
MCC significant lower GI bleeding beyond infancy
-painless, intermittent, streaky bleeding
what is it and how to tx
juvenile polyps
colonoscopy with polypectomy
painless acute rectal bleeding in an otherwise healthy child
what is it and how to tx?
meckel’s diverticulum- ectopic gastric mucosa
surgical resection
MAHA, thrombocytopenia, acute renal failure
intestinal ulceration and infarction of the bowel cause bleeding
HUS
palpable purpuric rash on butt and LE
large joint arthralgias
renal involvement
GI bleeding
HSP (IgA mediated vasculitis)
other causes of LGIB in kids
Hirschsprung’s disease
allergic colitis
infectious enterocolitis
IBD
hepatocellular enzymes… which one is most specific for liver injury?
AST is sensitive but not specific
LDH is nonspecific
**ALT is very specific for liver disease
2 biliary enzymes
- alkaline phosphatase- also can be elevated with rapid growth, bone, kidney, intestinal disease
- GGT and 5NT (this is more specific)
define infant jaundice
when does jaundice become clinically evident
elevated bili after neonate period and within the first year of life
when bili is > 3mg/dL
as many as ____ of all neonates or infants experience transient jaundice, the majorty of whom have unconjugated hyperbilirubinemia
50%
jaundice begins _____ and spreads ______
cranially and spreads caudally
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
inspissated bile syndrome
50% activity of UDP-glucuronyl transferase deficiency
mild unconjugated hyperbili with stress or poor nutrition
Gilbert’s
AR disorder
100% UDP-glucuronyl transferase deficiency
extremely high unconjugated bili –> kernicterus almost universally
Crigler-Najjar type 1
AD disorder
90% UDP-glucuronyl transferase deficiency
lower bili levels lead to kernicterus sometimes
Crigler-Najjar type 2
MCC cholestasis in the newborn
what is it, what’s the tx and prog?
- 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
infant age 4-6 weeks presents with jaundice, dark urine, and pale/acholic stools
-bilirubin levels are moderately elevated
biliary atresia- progressive fibrosclerotic dz that affects the extrahepatic biliary tree
-progresses quickly with bile duct obliteration and cirrhosis by 4 months
how to dx biliary atresia
- US, HIDA, and liver bx to r/o other causes
- intraop cholangiogram to confirm the diagnosis
how to tx biliary atresia
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
- 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
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
MC viral hepatitis infections in kids
hep A and B
-most infections in childhood are asymptomatic
MC hepatitis virus causing infection
how is it transmitted
clinical presentation
hep A
fecal oral
most kids are asymptomatic; teens and adults more likely to have sxs
how to dx and tx hep A (HAV)
dx with serology
-IgM anti-HAV: early in infection-6months
-IgG anti-HAV: early in infection-lifelong immunity
tx with supportive care
transmission of HBV
perinatal vertical
IVDU, tattoos, blood products, sex
chronic hep B infection is more likely in _______
what are the sxs?
younger infants
sxs: wide range ranging from asymptomatic to fulminant liver failure
hep B serology HBsAg HBsAb HBcAb HBeAg HBeAb
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
tx for HBV infection
supportive care for acute infection
interferon and antivirals for chronic infection
HCV transmission
perinatal vertical
IV exposure- accounts for 90% of transfusion-associated hepatitis
Although acute infection is rarely symptomatic (esp in children), HCV is more likely than the other hepatitis viruses to cause chronic infection (T/F)
T
80% end up being chronically infected
how to dx HCV
HCV antibody or PCR
Hepatitis D requires ______ for replication
HBsAg
Hepatitis E is transmitted ______ and is especially dangerous in ______
fecal-oral
pregnant women
progressive liver dz characterized by elevated serum transaminases, hypergammaglobulinemia, and circulating autoantibodies
-whats the classic demographic
autoimmune hepatitis
females with presentation before puberty
2 types of autoimmune hepatitis
type 1- ANA or ASMA- this type is more common
type 2- anti-LKMA or anti-liver cytosol type 1 antibody
how does autoimmune hepatitis first present
50% acute hepatitis
50% chronic liver disease
how to tx autoimmune hepatitis
steroids for acute control of inflammation
imunosuppressives for long term control: azathioprine, 6-mercaptopurine
heterotaxy puts you at risk for malrotation (T/F)
T
maintenance water requirements
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
maintenance Na requirement
2-3 mEq/kg/day
maintenance K requirement
2 mEq/kg/day during infancy but decreases with age
what to give for emergency phase of treating dehydration
20 mL/kg boluses of 0.9% NaCl or LR
this is the same for all pts, regardless of initial serum Na levels
define hematuria
microscopic hematuria
presence of RBCs in the urine
> 6 RBCs/hpf is microscopic hematuria
Urine dipstick also detects hemoglobin or myoglobin in the urine (T/F)
what might cause a false negative for blood in the urine
T vitamin C (ascorbic acid) ingestion
RBC casts indicate bleeding from the _____
glomerulus
acute hemorrhagic cystitis can be caused by bacterial infections, viral infections like _____, and drugs like ______
adenovirus
cyclophosphamide
proteinuria > ______ is considered pathologic
100 mg/m2/day
urine dipstick tests for this type of protein
albumin
normal urine TP/CR
infants 6-24 months: < 0.5
children > 2 years: < 0.2
kids who have increased urinary protein excretion while upright but not while supine
what is it and how to dx for sure
orthostatic proteinuria
- morning urine protein should be normal, afternoon urine protein should be high
- this is benign
two origins of pathologic proteinuria and which one is more common?
glomerular- increased permeability, this is more common
tubular- decreased reabsorption
lab findings in tubular proteinuria
urinary beta2-microglobulin (normally freely filtered at the glomerulus and then completely reabsorbed)
glucosuria
aminoaciduria
lab finding in glomerular proteinuria
increased urinary microalbumin
primary vs. secondary glomerulonephritis
primary- limited to kidney
secondary- 2/2 a systemic disease
gross hematuria, HTN, signs of fluid overload from renal insufficiency
nephritic syndrome
heavy proteinuria, hypercholesteremia, edema
nephrotic syndrome
most common form of acute GN in school age children
poststrep GN
timeline of post strep GN
8-14 days after skin or pharynx infection
21-28 days after impetigo
clinical presentation of post strep GN
hematuria (often gross), proteinuria (but not as much as nephrotic syndrome), HTN with signs of fluid overload like edema
labs in post strep GN
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
you don’t routinely bx in post strep GN, but if you did, what would you see
mesangial cell proliferation and increased mesangial matrix
tx for post strep GN
prognosis
- supportive care: fluid restriction, anti-HTN, dietary restriction of protein/Na/K/phosphorus
- prognosis is excellent
treating strep infections with abx prevents post strep GN from occurring (T/F)
F
it only prevent rheumatic fever and PANDAS
most common type of chronic GN worldwide
-recurrent bouts of gross hematuria associated with respiratory infections
IgA nephropathy
renal bx results of IgA nephropathy
- mesangial proliferation and increased mesangial matrix
- mesangial deposition of IgA
how to tx IgA nephropathy
what’s the prog
- supportive
- meds (steroids, ACEI) for pts with pathologic proteinuria or renal insufficiency
- prognosis: variable… 20-40% end up with ESRD
nonthrombocytopenic palpable purpura on the buttocks and thighs, abdominal pain, arthritis and arthralgias, gross or microscopic hematuria
Henoch-Schonlein purpura (HSP) nephritis- IgA mediated vasculitis
HSP nephritis prognosis
in most cases, renal features are self limited with complete recovery within 3 months
in 1-5%, pts develop chronic renal failure
common types of GN in children
post strep IgA nephropathy (Berger's disease) HSP nephritis membranoproliferative GN (MPGN) membranous nephropathy (MN) SLE nephritis
lobular mesangial hypercellularity and thickening of the GBM
- nephritis or nephrotic syndrome with hematuria
- HTN
- low serum complement
- most pts develop ESRD
membranoproliferative GN (MPGN)
how to tx MPGN
no definitive tx
can try steroids and ACEI
rare form of GN in young children….heavy proteinuria which progresses to renal insufficiency
membranous nephropathy (MN)
heavy proteinuria (> 50mg/kg/24 hours)
hypoalbuminemia
hypercholesterolemia
edema
nephrotic syndrome
3 categories of nephrotic syndrome
- primary NS- not due to systemic disease, 90% of childhood NS
- NS from other primary glomerular disease (IgA nephropathy, MPGN, PSGN)
- NS from systemic diseases like SLE and HSP
MCC primary nephrotic syndrome
minimal change disease (MCD)
typical presentation of nephrotic syndrome in kids
most present with edema following a URI
2 things you need to watch out for in kids with nephrotic syndrome (besides the usual stuff)
- predisposition to thrombosis 2/2 hypercoagulability –> stroke, DVT, renal vein thrombosis, sagittal sinus thrombosis
- increased risk of infection with encapsulated organisms like streo pneumo –> SBP, pneumonia, over whelming sepsis
UA in nephrotic syndrome would show:
3+ to 4+ protein
elevated urinary TP/CR
CBC in nephrotic syndrome shows:
elevated hematocrit and/or elevated plts 2/2 hemoconcentration resulting from hypoproteinemia
management of nephrotic syndrome
- 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
prognosis of nephrotic syndrome
what typically causes mortality
- 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
acute renal failure with MAHA and thrombocytopenia
HUS
2 types of HUS… which is more common
shiga toxin-associated HUS –> more typical
atypical HUS
most common pathogen that causes HUS
E Coli 0157:H7
others include E Coli other strains and shigella dysenteriae type 1
classic presentation of shiga toxin-associated HUS
bloody diarrhea followed by sudden onset of hemolytic anemia, thrombocytopenia, and acute renal failure
how to tx shiga toxin-associated HUS
- 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
prognosis of shiga toxin-associated HUS
what are some complications
prognosis is generally favorable
poor prog signs: high WBC on admission, prolonged oliguria
complications may be toxic megacolon, cerebral infarctions, etc
2 causes of atypical HUS
drugs (OCP, cyclosporine, tac, OKT3)
inherited
presentation, management, and prog of atypical HUS
- 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
progressive hereditary nephritis that’s secondary to defects in the side chains of type IV collagen within the GBM
Alport’s syndrome- X linked dominant
clinical features of Alport’s
HTN, hematuria, ESRD
hearing loss
ocular problems involving the lens and retina
how to manage Alport’s
treatment of HTN
ACEI
eventual kidney transplant
MCC renal mass in the newborn
multicystic renal dysplasia
- maternal h/o oligohydramnios secondary to nonfunctioning or poorly functioning kidneys
- pulmonary hypoplasia
- greatly enlarged cystic kidneys
- severe HTN
- usually has some liver involvement
autosomal recessive polycystic kidney disease (ARPKD) or infantile polycystic kidney disease
-it is progressive and will eventually require transplant
-abdominal pain, flank masses, UTI, hematuria, severe HTN, renal insufficiency in an adult
ADPKD
prognosis of ADPKD
most develop severe HTN and require transplant
watch out for cerebral aneurysms
this inherited kidney disease has 2 forms
nephronophthisis-medullary cystic disease complex (NPH-MCD)
juvenile form- AR, ESRD in childhood
adult form- AD, ESRD later in life
medullary sponge kidney is caused by ______
inheritance
definitions: normal HTN significant HTN severe HTN malignant HTN
normal HTN: 90-95th percentile
significant HTN: >95% percentile
severe HTN: >99% percentile
malignant: assoc with evidence of end-organ damage
most HTN in childhood is _____ (primary/secondary)
secondary
common causes of HTN based on age
- neonates and young infants
- age 1-10 years
- adolescents
- 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
what to look for on PE of kids with HTN
four limb BP to look for coarctation
funduscopic exam
look for CHF, cafe au lait (neurofibromatosis), abdominal masses, abdominal bruits, ambiguous genitalia
goal of chronic HTN management
be below 90th percentile
clinical presentation of RTA in kids
infants and young children- growth failure, vomiting
older children and adults- recurrent calculi, muscle weakness, bone pain, myalgias
RTA: inability of the distal renal tubular cells to excrete H+
-vomiting, growth failure, acidosis, nephrocalcinosis and nephrolithiasis
distal RTA (type I) tx with small doses of oral alkali
RTA: impaired bicarb reabsorption by the proximal rental tubular cells
-vomiting, growth failure, acidosis, muscle weakness
proximal RTA (type II) tx with large doses of oral alkali
RTA: transient acidosis in infants and children, hyperkalemia is hallmark
-may be asymptomatic, can present with FTT
type IV RTA
-tx with furosemide to get rid of K and also oral alkali
proximal RTA, hyperphosphaturia, aminoaciduria, glucosuria, K wasting
Fanconi syndrome
classic electrolyte finding in RTA
what’s the urine anion gap like in distal RTA
non-anion gap hyperchloremic metabolic acidosis
+ urine anion gap in distal RTA
oliguris in kids is defined as
< 1mL/kg/hr
management of renal failure
- 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
FeNa formula
FeNa = (urine Na/serum Na)/(urine Cr/serum Cr) x 100%
when to consider dialysis or renal xplant
which dialysis to use
when GFR is 5-10% of normal
peritoneal dialysis preferred in kids
overall, xplant is the preferred tx for kids with ESRD
causes of bladder outlet obstruction
posterior urethral valves in males
polyps
prune belly syndrome (absence of rectus muscles, bladder outlet obstruction, cryptorchidism in males)
renal agenesis is 2/2 failure of development of _____ or _____
mesonephric duct or the metanephric blastema
_________ is associated with altered structural organization of the kidney
functionally, it’s assoc with concentrating defects, RTA, and renal insufficiency
renal dysplasia
fusion of lower poles of kidney
horseshoe kidney
kidney located outside of renal fossa
renal ectopia
_______ is identified in 30-50% of infants and young children with UTIs
vesicoureteral reflux
how is VUR inherited
AD with variable expression
VUR, if prolonged, may lead to ______ and ______
- pyelonephritis
- reflux nephropathy- ESRD, HTN, renal scars, contraction, interstitial nephritis
children with reflux usually need surgery to correct it (T/F)
F
most kids wit lower grades (1-2/5) eventually have spontaneous resolution of reflux
how to dx VUR
voiding cystourethrogram
how to manage VUR
ppx abx until kid outgrows the VUR
if grade 4-5, consider surgical re-implantation of the ureters
renal stones are common in children and you can just treat with hydration (T/F)
F- they are uncommon in children and you should seek out predisposing metabolic disorders
if renal stone is due to hypercalciuria, look for these conditions
hypercalcemia
familial hypercalciuria
furosemide use
if renal stone is due to hyperoxaluria, look for these
inherited d/o enteric malabsorption (ex. IBD)
if renal stone is due to hyperuricosuria, look for these
tx of leukemia or lymphoma
Lesch-Nyhan syndrome
primary gout
if renal stone is due to cystinuria
look for cystinuria- AR disorder leading to radiopaque stones
if renal stone caused by UTI, look for this organism
proteus mirabilis
tx of renal stones
hydration
relief of obstruction
tx UTI if resent
specific therapy depends on which type of stone it is
demographics of those at risk for UTI
< 6 months: uncircumcised boys
> 6 months: girls
common bacteria causing UTI
E Coli
klebsiella, pseudomonas, staph saprophyticus (teen girls), serratia, proteus (alkaline urine), enterococcus
how to collect urine for UA and culture
neonates and infants: suprapubic aspiration or via sterile urethral cath
older children: clean catch
UA of UTI
leukocytes (>5-10 WBCs/hpf)
+ nitrite or leukocyte esterase
urine culture for UTI
what are the cutoffs for + result?
any growth if suprapubic aspiration
> 10,000 colonies if sterile urethral catherization
> 50,000 - 100,000 if clean catch
when should you image (renal US and VCUG) for UTI
recurrent UTI
all males
girls < 4 years of age
how to tx UTI
- 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
2/2 risk of renal scarring after pyelo in infants, give ppx abx for ______ after an episode of pyelo
3 months
microscopic hematuria is usually > ______
6 RBCS/hpf for 3 or more consecutive urine samples
maintenance fluid should be increased by 12% for every degree above 38 degrees C (T/F)
T
_______ have a greatly increased risk of renal vein thrombosis
infants of diabetic mothers
maternal SLE may result in infant ______
heart block
