peds22 Flashcards
pityriasis alba
hypopigmented dry scaly patches, most commonly on the cheeks; treat with moisturizers and mild steroids
vitiligo
complete loss of skin pigment in patchy area; caused by melanocyte desruction; no treatment but psoralen with UV light might help
oculocutaneous albinism
genetic defect in melanin synthesis. White skin and hair, blue eyess and other eye findings; no treatment
tuberous sclerosis
ash-leaf spots (hypopigmented macules under woods light); adenoma sebaceum; shagreen patch; ungual fibromas
neurofibromatosis type 1
cafee-au-lait spot, axillary or inguinal freckling, plexiform neurofibroma or skin neurofibroma
CNS findings in tuberous sclerosis
seizures, infantile spasms, intracranial calcifications
CNS findings in NF-1
optic glioma, intracranial calcifications, CNS neurfibromas
systemic findings in TS
renal cysts, cardiac rhabdomyomas (number 1 cause of neonatal cardiac tumors), retinal astrocytomas or hamartoma; mental retardation
systemic findings in NF-1
oseeous lesions; sphenoid dysplasia; scoliosis, hypertension; learning problems
congenital nevi
first detected before 6 months of age; all have increased risk of malignancy but giant nevi have a 6-7% ifetime risk of maig melanoma; you excise giant nevi
acquired nevi
aka moles; peak ages 2-3 yo and 11-18 yo incr in size and number after puberty and pregnancy or in sun; most are junctional nevi; risk of malignancy is lower
alopecia
autoimmune lymphocyte mediated injury to the hair follicle; complete hair loss in certain areas without scalp inflamm;
alopecia can be associated with what nail finding
pitting of nails
alopecia totalis vs alopecia universalis
totalis is loss of all scalp hair; universalis is loss of all scalp and body hair
management of alopecia
most patients regrow hair in 1 year without treatment; topical or injected corticosteroids may help accelerate growth
tinea capitis is a common cause of hair loss
right
trichotillomania
hair loss due to conscious or unconscious pulling or twisting of the hair
traction alopecia
hair loss caused by constant traction (tight braids); thinned, small hairs but few broken hais
telogen effluvium
acutely stressful event (pregnancy, surgery) that converts hairs from a growing phase (anagen) to final resting phase (telogen); excessive hair loss 2 months after event
most common skin disease
acne
noninflamm acne
open comedones (black heads) and closed comedones (white heads)
inflamm acne
erythematous papules, pustules, nodules and cysts
systemic isotretinoin (Accutane)
highly effective for all kinds of acne (inflamm and noninflam); women must be tested for pregnancy before treatment and use BC
lisch nodules
nf1
murphys sign
palpation of the RUQ during inspir elcits pain; means inflamed gall bladde
when is cholecystectomy indicated?
not in all cases of cholecystitis; only if disease progresses or peritonitis develops
chronic abdominal pain
abdominal pain that occurs each month for at least 3 consec months
h pylori screening should be reserved for kids with sx of dyspepsia
because the asymp carrier rate is very hgih
lactose breath hydrogen testing
to rule out lactose intolerance
long term prognosis of fuctional abdom pain
poor; only 50% complete symptom resolution during childhood; 25% still have sx into adulthood
encopresis
developmentally inapp release of stool; it almost always asso with severe constip; seen predom in males
stool frequency during first week of life
4x/day
stool freq by 1 year of life
2x/day
stool freq by 4 years
1x/day
breastfed infants defecate more frequently during the first month
but by 4 mos, they and formula fed are at about the same rate
most common form of fecal retention in kids
functional fecal retention; results from inapprop constriction of the anal sphincter
organic causes of fecal retention
hirschsprung (most common)
management of FFR
stool evacuation, mineral oil to soften the stool, eduaction
age of onset is bimodeal
peak at 15-20 years and a second pekak after 50 year
inflamm in UC
diffuse, limited to mucosa, localized to the colon; begins in the rectum and extends proximally
toxic megacolon
complication of severe UC in which disruption of the mucosal barrier allows bacteria to enter and you get colonic dilation and fever and septic shock
UC gives you incr risk for what type of cancer
colon cancer
crohns disease inflamm
any segment of the GI tract but usually distal ileum; skip lesions; transmural inflamm (may get fistulas, sinus tracts)
perianal disease in crohns?
yes, often preceds the development of intestinal disease and presents with skin tags, fissures, fistulas, and abscesses
drugs for IBD
sulfasalazine (for mild disease), corticosteroids, immunosupp agents, metrondazole (for CD, esp perianal involvement)
serologic testing for UC
antineutrophil cytoplasmic antibody pos in 80%
serologic testing for crohns
anti-saccharaomyces cerevisiae pos in 70%
hematochezia
bright red blood passed per rectum
melena
dark, tarry stools; often indicates upper GI bleed proximal to ligament of treitz
ligament of treitz
suspensory muscle that marks the division between duodenum and jejunum;
false pos guiac test
ingested iron, rare red meats, beets, and foods w high peroxidase content
flase neg guiac test
large ingested doses of vit C
mallory-weiss tear
mechanical injury to the mucosa from vomitting
varices
as a result of portal hypertension or vascular malformations are uncommon but possible causes of upper GI bleeds
how to assess ongoing upper GI bleeding
nasogastric tube aspirate
elevated BUN suggests what
GI bleed
octreotide
vasopressin; can be used to vasoconstrict varices
newborn with rectal bleeding, feeding intolerance, or abdom distension
consider NEC
hemolytic uremic syndrome
infection triggers hemolytic anemia, kidney failure, thrombocytopenia; most common in kids
henoch-schonlein purpura
preceded by infection; palpable purpura on skin and kidney involvement; triad is purpura, athritis and abdominal pain
most common cause of signif lower Gi bleed beyond infancy
juvenile polyps; bleeding is painless, intermittent, and often streaky
allergic collitis
from sensitization to protein antigens in milk
infectious enterocolitis caused by what
salmonella, shigella, campylobacter, yersinia, and e coli
meckels diverticulum
outpouching of the bowel in the terminal ileum; important cause of lwer GI bleeding in infants and kids; the diverticulum contains ectopic gastric mucosa that produces acid
painless acute rectal bleeding
classic for meckels diverticulum
treatment for meckels diverticulum
use nuclear scan to identify the ectopic gastic mucosa and then resect it surgically
hemolytic uremic syndrome
vasculitis characterized by microangiopathic hemolytic anemia, trombocytopenia, and acute renal failure; intestinal ulceration and infarction of the bowel causes bleeding
henoch-schonlein purpura
igA mediated vasculitis with palpable purpura on the butt and loweer extremities, large joint arthralgias, renal involvement, and GI bleeding from complications like intussusception and bowel perforation
how does H-S purpura cause GI bleeding
intussusception can be a complication, as can GI perforation
how can a gall bladder issue cause increased liver enzymes?
damage to the biliary system causes retention of bile enzymes that damage the bilary tree and hepatocytes
AST vs ALT
AST is sens but nonspecific, since also found in muscle, RBCs, and heart; ALT is very specific
LDH
elevation is nonspec for liver disease
alk phos
can be elevated in biliary disease and for lots of different reasons like rapid growth in a kid
GGTP and 5NT
elevated in biliarydisease. 5NT is more specific than GGTP for biliary tract disease
how is bilirubin conjugated in the liver
unconjugated bili is combined with glucuronide by the enzyme UDP-glucuronyl transferase in the liver to form mono and di conjugates
how is synthetic function of the liver assessed
evaluating protein production (prealbumin, albumin, and prothrombin time), serum chemistries (glucose, cholesterol), and toxic clearance (lactate, ammonia)
cholestatic jaundice
retention of bile within the liver; occurs when the direct component of bilirubin is greater than 2 mg/dl or 15% of total bili
cholestasis gives you direct or indirect hyperbilirubinemea?
direct
conjugated (direct) hyperbilirubnemia most commonly caused by what?
neonatal hepatitis or biliary atresia
how does jaundice appear
cranially, then extends caudally
inspissated bile syndrome
cause of indirect hyperbilirubinema; associated with hemolysis or large hematoma; conjugated hyperbilirubinemia happens later as liver meets demand
UDP-glucuronyl trasnsferase def
def of enzyme that conjugates the bilirubin; seen in Gilberts syndorme, crigler-najjar syndrome type 1 and type 2
gilberts syndrome
50% of enzyme activity of UDP-glucuronyl transferase is absent; mild unconjugated bilirubinemia
crigler-najjar type 1
aut recessive; 100% of enzyme activity of UDP-glucuronyl transferase activity is absent; kernicterus occurs
crigler-najjar type 2
aut dom; 90% of enzyme activity of UDP-glucuronyl transferase act is absent; lower likelihood of kernicterus
causes of cholestasis (retention of direct bili in the liver)
infections, extrahep obstruction, intrahep obstruction, a1-antitrypsin def, TPN-associated disease
clinical features of cholesstasis
jaundice, acholic or light stools, dark urine, hepatomegaly, bleeding, FTT
why do you get bleeding in cholestasis
prolongation of prothrombin time as a result of diminished hepatic synthetic function
most common cause of cholestasis in the newborn
neonatal hepatitis; it is idopathic; male predisposition
presenting features in the first week of life in babies with neonatal hepatitis
jaundice and hepatomegaly; FTT occurs later
course of neonatal jaundice
slef-liited, with full recovery in 70%
management of neonatal hepatitis
nutritional support with fat soluble vitamins; TPN may be needed; also give ursodeoxycholic acid (bile acid) ; liver transplant in severe failure
biliary atresia
progressive fibrosclerotic disease that affects the EXTRAhepatic bilary tree; etiology is unknown
clinical features of biliary atresia
most present between the ages of 4 and 6 weeks with jaundice, dark urine, and pale stools; bili is slightly elevated; progression of disease is rapid; hepatosplenomegaly
kasai portoenterostomy
roux en y intestinal loop attached directly to the porta hepatis treatment of choice to establish bile flow in biliary atresia; works best if performed by 50-70 days of age
worrisome complication of kasai
cholangitis
only give urso acid when?
once bile flow is re-established
alagille syndrome
aut dom; paucity of intrahep bile ducts and multiorgan involvement
facial features in alagille sydnrome
broad forehead, deepset wide spaced eyes, saddle nose with bulbous tip; pointed chin and large ears
