Internal Medicine

0521779407-17 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:19

1214 Primary Hyperparathyroidism Primary Sclerosing Cholangitis

➣Reduced creat clearance (<70% of normal)

➣Renal stones

➣Reduced bone mass (>–2.5 SD below sex-, and race-matched

normals)

■Small clinical trials show skeletal benefit with po alendronate

follow-up

■Twice-yearly or annual monitoring of symptoms, Ca, PTH, renal

function, annual bone mineral density

complications and prognosis

■Renal stones, deterioration of renal function, fractures, changes in

mental status, depression, neurologic and myopathic changes

■Prognosis: recent 10 year prospective study – no skeletal or func-

tional deterioration in majority of patients with mild, asymptomatic

disease

■Symptomatic and severe disease – excellent response to parathy-

roidectomy (>95% curative) and gains in bone mass

PRIMARY SCLEROSING CHOLANGITIS

JOANNE C. IMPERIAL, MD

history & physical

History

■frequently a disease of men; onset usually below age 45

■approximately 70% have IBD (usually ulcerative colitis), although

only 4–5% of ulcerative colitis patients have PSC

■often associated with other autoimmune diseases (Sjogren’s, thy-

roiditis) and certain HLA haplotypes (HLA-B8)

■must exclude other causes of chronic cholestasis in order to confirm

the diagnosis

Signs and Symptoms

■two potential patterns of disease progression have been described:

■insidious development of abdominal discomfort, pruritus, diarrhea,

malnutrition, osteoporosis

■fluctuating course of fever, RUQ pain and jaundice associated with

development of large dominant ductal strictures (possible increased

association with cholangiocarcinoma)