@article{c3836b43dd494ca2b5ece0fc078f8c4b,
title = "Cholestasis in sickle cell anemia",
abstract = "A patient with sickle cell anemia who presented with marked cholestatic jaundice is described, with emphasis upon the difficulties encountered in differentiating this symptom complex from extrahepatic obstruction due to stone formation. This patient had extraordinarily high serum bilirubin levels, apparently the result of increased bilirubin formation, decreased hepatic excretion due to dysfunction of the bile secretory apparatus and, possibly, decreased urinary excretion of the bile pigment.",
author = "Klion, {Franklin M.} and Weiner, {Martin J.} and Fenton Schaffner",
note = "Funding Information: A twenty-four year old Negro man was admitted to the Mount Sinai Hospital for the eighth time in August 1963, with the complaint of abdominal pain and {"}yellow eyes{"} of four days duration . The patient was first seen at The Mount Sinai Hospital in 1955 for evaluation of cardiomegaly and anernia . A diagnosis of sickle cell anemia was established by hemoglobin electrophoresis . He was readmitted in 1956 because of back pain, at which time the edge of the liver was felt 2 fingerbreadths below the right costal margin . The serum bilirubin was 7 .8 ing . per cent, with 1 mg . per cent direct-reacting . On two occasions in 1959 he was treated for skirt ulcers over both medial malleoli, and urinalysis during this period indicated isosthenuria . In February 1960, and again in 1961, he was readmitted because of severe pain in the lower abdomen accompanied by nausea and vomiting . X-ray examination of the upper gastrointestinal tract revealed a deformed duodenal bulb . Oral cholecystography demonstrated a normal-functioning gallbladder without calculi . Recurrent abdominal pain associated with emesis, bilirubinuria and jaundice precipitated readmission in August 1962 . Physical examination at this time revealed a nontender liver extending 3 hngerbreadths below the right costal margin and generalized rebound tenderness of the abdomen . Laboratory values included a white blood cell count of 37,000 per cu . mm ., serum bilirubin 5 .3 rag . per cent with 2 .4 mg . per cent direct-reacting serum alkaline phosphatasc 7 .2 King-Armstrong units, serum glutamic oxalacetic transaminase 78 units, serum cholesterol 107 mg, per cent with 85 ing . per cent esterified, cephalin flocculation test 4 plus, serum albumin 3.3 gin. per cent and globulin 3.9 gnr. per cent . Urinalysis revealed 3 plus bilirubinuria and urobilinogen present in a dilution of 1 :80 . Oral cholecystography again demonstrated a well functioning gallbladder, although the cystic and common ducts were not visualized . Free air was seen under both diaphragms in a chest roentgenogram . A presumptive diagnosis of a perforated duodenal ulcer was made but the patient was treated conservatively with suction and antibiotics because of his poor clinical condition . He made an uneventful recovery . The result of a sulfbro-mophthalein test performed just prior to discharge was 3 per cent retention in 45 minutes (dose . 5 mg . per kilogram body weight) . Subsequent examination of the upper intestine demonstrated an active duodenal ulcer . The patient remained well until three days prior to the August 1963 admission when he experienced postprandial cramps . Although these subsided on the following day, he noted scleral icterus, dark urine and light stools, in addition to mild anorexia and distaste for cigarettes . -there was no history of exposure to hepatotoxins or injections, alcohol ingestion, chills, fever or peen itus . Physical examination revealed a thin Negro mar, in no acute distress . The blood pressure was 110/70 mm . Hg, pulse rate 86 per minute and respiration rate 16 per minute . The temperature was 98 .8°r . The skin was markedly icteric ; the mucous membranes were prominently yellow-orange in color . The lungs were clear to percussion and auscultation . The heart was slightly enlarged, with the point oftnaximal * From the Departments of Medicine and Pathology . The Mount Sinai Hospital, New York . Neww York . This work was supported in part by Pathology Grant No . AM03846 and Grant No . 2i-5126 from the National Institute of Arthritis and Metabolic Diseases . National Institutes of Health . Manuscript received lanuary 21, 1964 . i Present address : The Memorial Hospital, New York . New York .",
year = "1964",
month = nov,
doi = "10.1016/0002-9343(64)90031-2",
language = "English",
volume = "37",
pages = "829--832",
journal = "American Journal of Medicine",
issn = "0002-9343",
publisher = "Elsevier Inc.",
number = "5",
}