Internal Medicine

0521779407-09 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:13

700 Hereditary Spherocytosis Hernia

■splenectomy “failure” due to accessory spleens missed at surgery or

another superimposed RBC disorder

■limited follow-up data available for partial splenectomy

follow-up

■follow for symptomatic anemia (exercise tolerance, dyspnea, tachy-

cardia)

■monitor growth and development

■observe for hematologic decompensation during acute illnesses

■interval ultrasounds for cholelithiasis

complications and prognosis

■symptomatic hemolytic anemia

■megaloblastic anemia due to folate deficiency

■cholelithiasis –∼50% patients, presents in adolescents and young

adults

■hemolytic crisis – jaundice, splenomegaly, and decreased hemat-

ocrit, associated with viral infection,

■aplastic crisis – fever, lethargy, nausea, vomiting, abdominal pain,

due to parvovirus B19 infection; Severe anemia may be life-

threatening; may be first manifestation of HS.

■megaloblastic crisis – patients with increased folate demands – e.g.

pregnant patient, growing child, recovering from aplastic crisis, or

elderly

■Rare: Chronic leg dermatitis/ulceration, gout, cardiomyopathy, neu-

romuscular abnormalities, “tumors” due to extramedullary erythro-

poiesis

Hernia............................................

ARTHUR I. SAGALOWSKY, MD

history & physical

■bulge in scrotum; may extend into inguinal canal

■size may change with activity

■asymptomatic or vague pressure, pain on exertion

■possible acute pain if incarcerated (non-reducible) or strangulated

tests

■Physical exam alone usually diagnostic

■acute tenderness, fever, elevated WBC suggest acute incarceration,

strangulation, impending infarction, and is a surgical emergency