Disease presentations and explanations Flashcards by Melanie Bray

Cause of blood in the stool in HSP

These occur in approx 50% of patients, about 1 week after dx.
Edema of the GI tract can cause damage to the vasculature and result in colicky abdominal pain and bleeding. Ultimate cause: vasculitis causing enlarged mesenteric lymph nodes, bowel edema and hemorrhage

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causes of secondary immunodeficiency

HIV infection, DM, malnutrition, hepatic disease, autoimmune disease, aging, stress

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primary immunodeficiencies

severe combined immunodeficiency
chronic granulomatous disease (phagocytic cell def)
complement deficiency

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reduces vertical transmission of HIV

Zidovudine (antiretroviral) given in 2nd trimester then given to infant through 6 weeks reduces transmission to < 10%

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preferred testing for HIV in neonate

HIV DNA PCR -> must be done this way due to maternal antibodies
** use ELISA followed by Western blot in 18 mos or older
Disease status eval: HIV RNA activity, CD4 count, clinical findings
“severe”: CD4 < 200
“normal”: CD4 >500

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used to prevent PCP infection in HIV+ infants

TMP-SMX prophylaxis started at 6 weeks

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Pediatric antiretroviral regimen for HIV+

2 nucleoside reverse transcriptase inhibitors and 1 protease inhibitor
Nucleoside reverse transcriptase inhibitors:
1. didanosine
2. stavudine
3. zidovudine
Nonnucleoside reverse transcriptase inhibitors:
1. efavirenz
2. nevirapine
Protease inhibitors:
1. indinavir
2. nelfinavir

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erythematous macular rash with satellite lesions

cutaneous candidiasis

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recurrent oropharyngeal and cutaneous infections with delayed wound healing
recurrent sinopulmonary infections

leukocyte adhesion deficiency (LAD)

be suspicious if have birth wounds that aren’t healing and attached umbilical cord at 6 weeks

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diminished/absent T cells
diminished/absent serum immunoglobulins
thymic dysgenesis
recurrent cutaneous, GI or pulm infections (CMV, PCP)

SCID

AR or X-linked

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Thymic aplasia
Boot-shaped heart on XR
atypical facies (wide-set eyes, prominent nose, small mandible)
cleft palate

DiGeorge Syndrome

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hypogonadism
gynecomastia
long limbs
learning difficulties (spelling, reading, math)
often not dx until puberty

Klinefelter syndrome
(extra X in males) -> the more X’s the more MR -> check IQ
**Note increased breast cancer risk which approaches that for females

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males display explosive temper and aggressive or defiant behavior
long asymmetrical ears

XYY

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Child is exclusively fed goat’s milk. What are the complications?

megaloblastic anemia (from folate or B12 def); also is iron-poor
brucellosis (if not pasteurized)

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congenital condition which the liver’s bile ducts are blocked and fibrotic, resulting in reduced bile flow into the bowel (since less bile salts have fat-soluble vitamin deficiency, ex: vit D)

biliary atresia
Surgical correction: Kasai procedure
Note: patient’s with liver failure are often treated with diuretics for ascites and this results in calcium loss in the urine -> higher risk for rickets)

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Dental differences between rickets and familial hypophosphatemia

Rickets: enamel defects

Familial hypophosphatemia: intraglobular dentin deformities

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lab value seen in all rickets cases

elevated serum alkaline phosphatase level

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labs typical of low Vit D

decreased serum calcium and phosphorus

increased serum alkaline phosphatase and urine amino acids

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caused by dystrophin gene deletion, resulting in proximal lower extremity weakness

Duchenne muscular dystrophy

-calf muscles show fat and fibrosis with absent dystrophin on immunochemistry staining

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urinary tract anomaly
anuria/oliguria in utero 
oligohydramnios
pulmonary hypoplasia
flat facies
limb deformities

Potter sequence

decreased amniotic fluid can cause physical anomalies

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most common cause of urinary tract obstruction in newborn boys

posterior urethral valves

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Kawasaki disease vs Scarlet fever

Both have history of strep pharyngitis, but in scarlet fever it is “untreated strep pharyngitis”
Kawasaki has a blanching rash that includes the palms and soles. Swelling and erythema of the palms/soles.
Kawasaki has cervical lymphadenopathy of at least one lymph node.
Scarlet fever has a sandpaper rash that spares the palms and soles. It also has tonsillar exudates.

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How much weight loss equals 1 L fluid loss?

1 kg

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cool, clammy skin
capillary refill > 3s
cracked lips
dry mucous membranes
sunken eyes
sunken fontanelle
tachycardia
lethargy

severe dehydration (10-15% volume loss)

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``` decreased skin turgor dry mucous membranes tachycardia irritability capillary refill 2-3 s ```

moderate dehydration (6-9%)

sign on dehydration in diaper

brick-red urate crystals

Blanching rash vs nonblanching rash

Blanching rashes: blanching suggests that the erythema is due to vasodilation and is seen with viral illnesses, drug eruptions, Kawasaki disease, roseola and scarlet fever. Nonblanching rashes are from dermal hemorrhage: meningococcal disease and late in Rocky Mountain spotted fever

starts with "pins and needles" then ascending weakness | flaccid paralysis with absent DTRs and nerve conduction abnormalities

Guillain-Barre

edema, fatigue, abdominal pain and proteinuria

minimal change disease

doll-like face, thin extremities, protuberant abdomen from enlarged liver and kidneys, short stature seizures from hypoglycemia

glucose-6-phosphatase deficiency

50,000 colony-forming units/mL in catheter-obtained urine specimen

bacteriuria

greater than or equal to 5 WBCs in urine

pyuria

stridor that improves with neck extension | associated with cardiac abnormalities

vascular ring

``` metabolic acidosis with AG hyperketonemia hyponatremia Elevated BUN & creatinine may see elevated WBCs from infection causing exacerbation ```

DKA

hemoglobin A1c goals for diabetics

6-9% | > 12% suggests poor control

Other than DKA, what might cause glycosuria?

stress response to infection

Special management of sickle cell disease (SCD)

1. daily PCN by 2 months age 2. daily folate by 6 months age 3. pneumococcal and meningococcal vaccines by age 2 4. watch for evidence of stroke -> treat with exchange transfusion to reduce the number of circulating sickle cells. 5. routine CBC with retic count starting at diagnosis or 2 months

complication of splenic sequestration

cardiovascular collapse

coughing spells with quiet intervals, often heard with pertussis and chlamydial pnemonia

staccato cough

causes of pleural effusion

1. CHF 2. mycobacterial pneumonia 3. malignancy (lymphoma)

most frequent cause of pediatric bacterial pneumonia

pneumococcus

cause of "belly pain" in a child with pneumonia

irritation of the diaphragm by pulm infection

``` diarrhea/defecation urination miosis bradycardia bronchorrhea emesis/excitation of muscles lacrimation salivation ```

``` cholinergic excess (organophosphate poisoning) Tx: atropine and pralidoxime ```

symptoms of arsenic ingestion

nausea, vomiting, abdominal pain, diarrhea

red or maroon-colored stools

hematochezia

black tarry stools produced by oxidation of heme by intestinal flora

melena

causes 50% of lower GI bleeds in children less than 2 yr

Meckel diverticulum

most common cause of hematochezia in infants, children and adolescents

anal fissure

treatment of AOM

amoxicillin 80-90 mg/kg/d for 7-10 days if clinical failure after 3d, change to augmentin, cefuroxime axetil (Ceftin), azithromycin, cefixime, ceftriaxone, or tympanocentesis

Wheezing vs stridor

wheezing is polyphonic, and stridor is monophonic

drooling, preference to sit in a tripod position, muffled vocalizations, inspiratory stridor, absence of cough

epiglottitis

vaccine associated with thrombocytopenia

MMR | give with caution to those with ITP

most common infectious cause of congenital sensorineural hearing loss (SNHL)

prenatal CMV Other infectious causes: toxoplasmosis, rubella, syphilis Postnatal causes: GBS sepsis and S. pneumoniae meningitis

partial albinism (white forelock), SNHL, lateral inner canthi displacement, eyebrow confluence, broad nasal bridge and mandible

Waardenburg syndrome

absence of the lens

aphakia

reflux with intermittent torticollis

Sandifer syndrome | These have abnormal head posturing associated with reflux

Drugs that caused dystonia that may present like torticollis

phenothiazine, metaclopramide, haloperidol

most common cause of severe urinary tract obstruction in boys

posterior urethral valves | Risk: end stage renal disease

Conditions associated with undescended testis

Hernia, testicular torsion, testicular cancer, infertility

elevated urinary vanillylmandelic acid and homovanillic acid levels elevated enolase, ferritin, lactate dehydrogenase

Neuroblastoma Presentation: abdominal mass, pallor, proptosis, "raccoon eyes" Tumor is typically found in the adrenal gland Poor prognosis: older children with bony mets, N-myc oncogene amplification

Structures at risk with retropharyngeal abscess

Mediastinal