Internal Medicine

0521779407-19 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:21

Short Bowel Syndrome Sickle Cell Syndromes 1345

follow-up

■Weekly body weights, monthly examinations, nutritional assessment

to include vitamin assays [(B12), folate, vitamins A, D, and prothrom-

bin time (INR)], magnesium, calcium

complications and prognosis

■Varies with underlying disease with potential of recurrence (Crohn’s,

intestinal ischemia, collagen vascular disease)

SICKLE CELL SYNDROMES

ORAH S. PLATT, MD

history & physical

History

■Family history of sickle syndrome (common syndromes include

sickle trait, disease, SC disease, sickle cell anemia, S/thalasse-

mia)

■Most states in the US screen newborns for homozygous disease

(SS=sickle cell anemia)

■History of anemia – no anemia in sickle trait, no or mild anemia in

SC disease and some patients with S/thalassemia

■History of pain crises, acute chest syndrome, priapism, stroke – not

found in sickle trait, less common in SC disease than SS

Signs & Symptoms

■Pallor, icterus, mild tachycardia only in syndromes with anemia – ie

NOT in sickle trait

■Splenomegaly absent in sickle trait and most adults with SS (because

of autoinfarction)

■Splenomegaly common in children with SS, and adults and children

with SC disease and S/thalassemia

■Hepatomegaly common in SS (increased RE system)

■Flow murmur found in essentially all anemic patients

■Signs of hemiparesis found in the <20% of adults with SS who have

had a stroke

■Leg ulcers found in <5% of adults with SS, even rarer in childhood or

in other sickle syndromes

■Retinal lesions (proliferative and non-proliferative) most common

in adults with SC disease