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1

Most devastating, acute complication that is likely to occur with DKA

cerebral edema

2

clues to DKA

N/V, severe abdominal pain, fatigue
recent history of polyuria, polydipsia, and polyphagia
signs of dehydration: increased pulse, decreased BP, and increased capillary refill time. also labored breathing, consistent with Kussmaul respirations
"fruity breath," caused by acetone formation

3

DKA

A severe insulin deficiency that leads to decreased peripheral glucose utilization.

4

Resultant complications of DKA include ?

hypertonic dehydration, ketonuria, and metabolic disturbances including increased serum anion gap, decreased serum bicarbonate, decreased serum pH.

5

Kussmaul respirations

Rapid, deep respirations associated with the compensatory respiratory alkalosis of DKA in response to the body's metabolic acidosis

6

DKA lab evaluation

serum glucose, serum electrolytes including blood urea nitrogen (BUN) and creatinine, serum pH, and urinary ketones
often: CBC and bld/ur cultures, as may be preceded by infection

7

DKA serum findings

elevated glucose (usually 400-800 mg/dL) and metabolic acidosis (decreased bicarbonate level with increased anion gap), hyperkalemia (although total body potassium is invariably low), hyponatremia (dilutional from increased serum glucose)

8

true serum sodium concentration can be calculated how?

by adding 1.6 mEq/L for every 100 mg/dL of serum glucose above the normal range

9

initial treatment of DKA (after ABCs)

dehydration correction (5-10%)
bolus of 10 mL/kg of isotonic fluids is given prior to treatment with insulin
remainder of the calculated fluid deficit should be replaced over the next 48 hours
-potassium and phosphate added to IVF

10

after initial fluid bolus given in DKA

IV insulin infusion should also be initiated after the initial bolus at a rate of 0.05 to 0.1 units/kg/h with the infusion titrated based on the patient's hourly glucose concentration

11

when to stop insulin therapy in DKA?

when anion gap has closed (last thing)
also: glucose has normalized, bicarbonate level is greater than 18 mEq/L, and her serum pH is greater than 7.3
transition to subQ insulin

12

how to prevent inadvertent hypoglycemia during the therapy phase of DKA

dextrose is added to the IV fluids once serum glucose levels reach 250 to 300 mg/dL

13

s/s of cerebral edema

severe headache, sudden deterioration of mental status, bradycardia, hypertension, and incontinence

14

if signs of cerebral edema, treat immediately with ?

IV mannitol and hyperventilation

15

management of Turner's syndrome

Monitor for cardiac and renal abnormalities, growth hormone for short stature, and laboratory work to check for hypothyroidism and dyslipidemia

16

initial diagnostic tests for Turner's

karyotype and ECHO (coarctation)

17

Turner's features

webbed neck, lymphedema (swollen hands and feet), low set ears, broad chest with wide spaced nipples, drooping eyes, and a higher incidence of hip dysplasia, cubitus valgus, and low posterior hairline, frequent OME-->hearing loss, strabismus, congenital glaucoma and anterior chamber deformities
later findings: short stature or primary amenorrhea

18

cubitus valgus

Physical finding in which the angle between the shaft of the ulna and humerus is increased greater than 15% in females.

19

Turner's chromosome analysis

single X chromosome with absence of all or part of the second sex chromosome (45 X). Mosaicism can be seen and can ameliorate expression of some of the clinical findings
caused by nondisjunction, not inherited

20

other cardiac conditions seen in Turner's

aortic root dilation, bicuspid aortic valve, MVP, and hypoplastic left heart syndrome

21

autoimmune conditions associated with Turner's

Hashimoto thyroiditis, celiac disease, IBD, glucose intolerance

22

consider treating Turner's with

GH and estrogen

23

other Turner findings

pigmented nevi, osteoporosis, inflammatory bowel disease, neuroblastoma, and liver disease.
scoliosis, kyphosis, lordosis

24

Prenatal diagnosis of Turner syndrome is suspected in the female fetus with ?

nuchal cysts and severe lymphedema.

25

syndrome that may be confused with Turner's but has a normal karyotype

Noonan syndrome
- seen in both genders, also presents with lymphedema at birth and with neck webbing, results in short stature and developmental delay

26

Beckwith-Wiedemann syndrome

macrosomic, macroglossic, and often hypoglycemic at birth; they have a higher incidence of Wilms tumor.

27

Features of trisomy 18 include ?

severe mental retardation, microcephaly, microphthalmia, micrognathia, clenched fingers and toes, malformed ears, high incidence of VSD, omphalocele, cryptorchidism, and thyroid hypoplasia.

28

A toddler with fatigue, decreased appetite, periorbital discoloration, and a multiquadrant abdominal mass, think?

neuroblastoma
an embryonal cancer of the peripheral sympathetic nervous system composed of primitive neuroendocrine tissue (3rd most common ped. cancer)
-most in adrenal gland, others: intrathoracic and paraspinal neuronal ganglia

29

opsoclonus-myoclonus syndrome

Characterized by chaotic eye movements and myoclonic jerks; described as "dancing eyes, dancing feet" related to autoantibodies produced against neuronal elements.
-seen with neuroblastoma (and small cell lung cancer in adults)

30

Metastatic disease from neuroblastoma typically involves ?
Findings may include ?

the long bones and skull, lymph nodes, liver, and skin.
fever, irritability, failure to thrive, and lymphadenopathy.

31

what happens if the orbital bones are involved in neuroblastoma ?

proptosis and bluish periorbital discoloration, described as "raccoon eyes," may be noted.

32

major ddx of neuroblastoma

Wilms tumor
typically associated with hematuria, HTN, and a localized abdominal mass that is smooth, well-defined, and rarely crosses the midline. In general, patients with neuroblastoma are slightly younger and sicker than patients with Wilms tumor

33

neuroblastoma imaging

CT/MRI to identify and assess the extent of neuroblastoma
Characteristic findings may include calcifications or hemorrhage

34

Laboratory markers of neuroblastoma include ?

elevated urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels (catecholamine metabolites), enolase, ferritin, LDH

35

Pathologic diagnosis of neuroblastoma usually is achieved via ?

tissue analysis from tumor biopsy or resection

36

neuroblastoma treatment

surgical excision of the tumor, usually after chemotherapy and/or radiotherapy to decrease tumor size

37

what patients have poorer prognosis in neuroblastoma

older children typically have a worse prognosis than infants
-skeletal metastases or N-myc oncogene amplification at the cellular level

38

labs in Wilms tumor workup

checking a urinalysis for hematuria, metabolic panel for renal or hepatic dysfunction, and CBC for anemia

39

Crohn disease

Involves the entire GI tract from mouth to anus. Transmural inflammatory process. Tendency for strictures, fistulas, and abscesses. May include skip lesions.

40

Ulcerative colitis

Only affects the colon and rectum. Characterized by crypt abscesses.

41

toxic megacolon

Complication characterized by fever, abdominal distention and pain, dilated colon, anemia, and hypoalbuminemia. The condition is life threatening.

42

common deficiencies seen in IBD

vitamin B12, folate, and iron secondary to malabsorption, anorexia, and chronic inflammation

43

Extraintestinal manifestations of Crohn disease

erythema nodosum, pyoderma gangrenosum, arthritis, digital clubbing*, arthralgias, and uveitis.

44

Extraintestinal manifestations of UC

primary sclerosing cholangitis*, arthritis, uveitis, pyoderma gangrenosum, arthritis of large joints, and erythema nodosum.

45

diagnose IBD with ?

physical examination, serum and stool laboratory tests, imaging, and colonoscopy (sometimes esophagogastroduodenoscopy (EGD) as well)

46

Characteristic findings of mucosa during colonoscopy of Crohn disease

inflammation with deep fissures, cobblestoning, pseudopolyp formation, skip lesions, and aphthous ulcers. Noncaseating granulomas are present in about 50% of pts

47

CT scans may be performed if no evidence of disease is seen on other studies for Crohn disease, may show ?

thickening of the bowel wall or abscesses

48

Barium enema in UC pt may reveal ?

a “lead pipe” appearance, caused by a loss of haustral markings; or “thumb-printing,” indicating inflammation

49

Characteristic findings of mucosa during colonoscopy of UC pt

protrusions of granulation tissue and regenerating epithelium called pseudo polyps, cryptitis and crypt abscesses. UC may result in shortening of the colon or postinflammatory colonic strictures.

50

what goes deeper, Crohn disease or UC?

Crohn
UC inflammation involves mucosal and submucosal infiltration by inflammatory cells, which do not often extend beyond the muscularis layer (unlike CD which involved the full thickness of the bowels)
in severe cases may extend beyond the muscularis mucosae into the submucosa.

51

erum laboratory values of patients with IBD may reveal ?

leukocytosis, hypoalbuminemia, anemia, and elevated ESR

52

about 10% to 25% of patients with CD have this Ab positivity
0% to 70% of patients with CD are positive for ?

perinuclear neutrophil cytoplasmic antibody (p-ANCA)

anti–Saccharomyces cerevisiae antibody (ASCA)

53

about 50% to 80% of patients with UC are positive for ?

pANCA
about 5% for ASCA

54

treatment of Crohn disease

azathioprine, 6-mercaptopurine, or methotrexate (immune modifying drugs), TNF-alpha (biologics), antibiotics
surgery may be required

55

treatment of UC

aminosalicylate drugs: sulfasalazine, olsalazine, or balsalazide; immune modifying drugs: azathioprine, 6-mercaptopurine, and methotrexate antibiotics, Surgical colectomy is also an option if symptoms are uncontrolled with medication

56

complications of CD

strictures; fibrosis; fistulas between bowel, bladder, or vagina; strictures; stenosis; and abscesses, small increased risk of colon cancer

57

complications of UC

toxic megacolon, often accompanied by fever, tachycardia, hypokalemia, hypomagnesemia, hypoalbuminemia, and dehydrate, (major risks: colonic perforation, massive hemorrhage)
colon cancer risk (more than CD)

58

The most common causes of rectal bleeding in the infant period are ?

a milk protein allergy and an anal fissure