10 Flashcards
bilious emesis due to intestinal obstruction, abdominal distension, blood per rectum, and lethargy in infant, think
malrotation with volvulus
vs intussusception: colicky abdominal pain and currant jelly stools
COMMON ETIOLOGIES OF ACUTE ABDOMINAL PAIN IN INFANTS AND YOUNG CHILDREN
http://casefiles.mhmedical.com.mwu.idm.oclc.org/ViewLarge.aspx?figid=140632540&gbosContainerID=75&gbosid=219808
if unable to get a peripheral IV in a decompensating pt, next step?
interosseous IV, quicker than central line
classic triad of situs inverses, recurrent sinusitis, and bronchiectasis
Kartagener syndrome
galactosemia caused by deficiency of ?
galactose-1-phosphate uridyle transferase
-cataracts, lethargy, hepsplenmeg, hypoglycemia, convulsions, poor weight gain, jaundice
iron def. vs thalassemia
anemia in iron def will have an increased RDW
thalassemia will have normal RDW and blood count, both will have low MCV
levels in Turners
what are these girls at risk for?
the swelling in these girls is due to?
low estrogen and high FSH due to lack of negative feedback
- osteoporosis due to estrogen deficiency
- lymphedema
Tourettes meds
haloperidol, pimozide, risperidone
angular cheilosis (lips), stomatitis, glossitis, normocytic anemia, seborrheic dermatitis
vitamin B2 (riboflavin) deficiency (similar to B6 pyridoxine def. but that should have mental changes)
polycythemia risk factors
symptoms?
delayed cord clamping, maternal HTN, DM
resp. distress, hypoglycemia, neuro manifestations
lactic vs renal tubular acidosis
RTA will have a normal anion gap, lactic acidosis will have an elevated anion gap
metabolic/e-lyte abnormalities in IDM
hypoglycemia, hypocalcemia, hypomagnesemia
vaccines to give at 2 months
HepB, Rotavirus, DTaP, Hib, IPV, PCV (pneumococcal)
pts with ataxia-telangiectasia will have what changes to immunglobulins
low IgA, IgG, IgE, elevated IgM
Abdominal radiographs may be normal or have nonspecific findings in cases of volvulus; thus, an ? is the test of choice
upper gastrointestinal contrast series
characteristic finding in cases of volvulus on upper GI series
a “corkscrew” pattern of the duodenum or “bird’s beak” of the second or third portions of the duodenum
management of an unstable pt with malrotation before emergent surgical intervention
evaluate fluid status
place a nasogastric tube to aid GI decompression
initiate IV abx
Exploratory laparotomy to assess bowel viability
Areas of necrotic bowel are removed and Ladd procedure of disengaging bowel with anomalous fixation and appendectomy is performed
distension, vomiting, bloody stools, and systemic signs such as temperature instability, thrombocytopenia, poor feeding, apnea, and respiratory failure.
necrotizing enterocolitis (NEC)
how to diagnose posterior urethral valves (PUV)
renal US or VCUG
can also see on prenatal US
? are the most common cause of severe urinary tract obstruction in boys
Posterior urethral valves
25% to 30% ultimately have end-stage renal disease or chronic renal insufficiency
Neonates with PUVs may present with
respiratory distress secondary to lung hypoplasia from oligohydramnios, distended bladders, poor or dribbling urinary streams, palpable kidneys, reduced renal function, or UTI
Immediate relief of PUV obstruction includes ?
labs?
- bladder catheterization through the urethra with a small feeding tube
- start abx if thinking UTI
- Serum electrolytes, BUN and creatinine levels are measured with correction as needed
- Hemodynamic status is monitored because sepsis or renal failure can lead to cardiovascular collapse
PUV tx once stabilized
endoscopic transurethral valve ablation
If the serum creatinine remains elevated, the urethral lumen is too narrow, or the UTI does not respond to antibiotics, ? may be necessary for PUV instead of endoscopic transurethral valve ablation
emergent vesicostomy
what kids should undergo evaluation for vesicoureteral reflux in addition to being treated with antimicrobials
2 to 24 months, children with recurrent UTIs, febrile UTIs, or pyelonephritis
renal US, then VCUG, then cystoscopy
how to tx nursemaid’s elbow
supinating the child’s forearm with the elbow in flexed position while applying pressure over the radial head
HUS
A syndrome of nephropathy, thrombocytopenia, and microangiopathic hemolytic anemia. It is associated with E.coli 0157:H7, Shigella, and Salmonella. A prodrome of bloody diarrhea is common
TTP
Pentad of fever, microangiopathic hemolytic anemia, thrombocytopenia, abnormal renal function, and CNS changes
-more common in adolescents than younger kids
ITP
A condition of increased platelet destruction by circulating antiplatelet antibodies, most frequently antiglycoprotein IIb/IIIa.
Laboratory findings of ITP
thrombocytopenia, which can be severe (<20,000/mm3), but the platelet size is normal or increased.
WBC count and hemoglobin level are normal (unless excessive bleeding has occurred)
PT and aPTT are normal
when to do BM eval in ITP
typically unnecessary, do if peripheral blood smear is concerning, the WBC count is abnormal, or adenopathy or organomegaly is present
-will show ncreased number of megakaryocytes in ITP
most serious ITP complication
intracranial hemorrhage
ITP tx
most are self-limiting
ITP with significant bleeding: IVIG for 1-2 ds, IV anti-D therapy, or a 2- to 3-week course of systemic corticosteroids
meds that cause immune-mediated thrombocytopenia
PCN, TMP-SMX, igoxin, quinine, quinidine, cimetidine, benzodiazepine, and heparin
MMR vaccine
Approximately ? of children with ITP have spontaneous resolution within 6 months
70% to 80%
peripheral blood smear of HUS
helmet cells, burr cells, and fragmented RBCs
A fracture of the long bones that is considered pathognomonic for inflicted injury
classic metaphyseal lesion
“corner fracture,” the “bucket handle fracture,” and the “metaphyseal fragmentation fracture.”
-occurs at the primary spongiosa region of the ends of the long bones and has the appearance of a bone fragment.
Bacterial diseases that share similar features of fever and exanthem to Kawasaki
group A streptococcal disease (scarlet fever, toxic-shock syndrome), Staphylococcus aureus toxic shock syndrome, Rocky Mountain Spotted fever, and leptospirosis
Noninfectious causes of similar symptoms of Kawasaki
drug hypersensitivity reactions or systemic-onset juvenile idiopathic arthritis (JIA)
lab findings in Kawasaki
elevated ESR and CRP normocytic anemia, leukocytosis, thrombocytosis, hypoalbuminemia, elevated ALT, and sterile pyuria. CSF pleocytosis and mildly elevated hepatic transaminase levels
associated Kawasaki symptoms
painful and frequent urination, meningismus, vomiting, or right upper quadrant pain
Klinefelter syndrome
syndrome comprised of behavioral problems (immaturity, insecurity), developmental delay (speech, language, lower IQ), and physical abnormalities (gynecomastia, hypogonadism, long limbs) caused by an extra X chromosome in males
-caused by nondisjunction
etiologies of ID (intellectual disability)
preconception and early embryonic disruptions, fetal brain insults, perinatal difficulties, postnatal brain inures, postnatal family difficulties
Physical findings to be considered in patients with suspected ID
the size of the occiput, unusual hair color or distribution, eye shape and placement, malformed ears or nose, and abnormalities in jaw size, mouth shape, or palate height
short metacarpals or metatarsals, overlapping or supernumerary digits, abnormal palmar creases, or nail changes
afé au lait spots or depigmented nevi, and the genitalia may be abnormally sized or ambiguous
tall and thin with long extremities
small testes/penis, gynecomastia, sparse facial hair, and azoospermia
chromosomal analysis of Klinfelter syndrome
one extra X chromosome (47,XXY) but may show additional X chromosomes (48,XXXY) or mosaicism (46,XY/47,XXY)
lab and imaging for intellectual disability
urine and serum amino and organic acids, serum levels of ammonia, lead, zinc, and copper, and serum titers for congenital infections
cranial CT, MRI, EEG
XYY males presentation (NOT XXY: Klinefelter)
explosive (often antisocial) behavior, weakness with poor fine motor control, accelerated growth in mid-childhood, large teeth, prominent glabella and asymmetrical ears, and severe acne at puberty
Girls with Turner syndrome (45,XO) present how
short stature, amenorrhea, excessive nuchal skin, low posterior hairline, broad chests with widely spaced nipples, edema of the hands and feet in the newborn period, cubitus valgus, coarctation of the aorta, hypothyroidism, and decreased hearing. Hypertension is common, possibly due to renal abnormalities (horseshoe kidney)
Intellectual development is typically normal
Fragile X syndrome
the most common form of inherited intellectual disability, is seen primarily in boys and can be diagnosed in patients with intellectual disability who have macrocephaly, long face, high arched palate, large ears, and macroorchidism after puberty.
