Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.1.3.1 (alkaline phosphatase)
 47,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Stauffer's syndrome represents a paraneoplastic liver disorder associated with renal cell carcinoma and is characterized by elevation of the serum alkaline phosphatase, increased bromsulphthalein retention, hypalbuminaemia, elevation of alpha-2-globulin and hypoprothrombinaemia, as well as hepatosplenomegaly. Two cases are reported in which this syndrome was the presenting feature and operation was undertaken on the basis of suspected primary biliary tract disease. The aetiology of the typical findings of Stauffer's syndrome are discussed. As they may be the only symptoms of an otherwise occult renal cell carcinoma, their presence should guide the diagnostic efforts in the right direction. Moreover, the possibility of predicting the postoperative course by follow-up control of the liver function tests is stressed.
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PMID:[Stauffer's syndrome. Reversible hepatic dysfunction in renal cell carcinoma (author's transl)]. 63 40

Biochemical changes, especially isolated rise in alkaline phosphatase and increased thromboplastin time, which have been described as "reversible hepatic dysfunction" (Stauffer's syndrome) were found in a 47-year-old patient with hypernephroma and hepatomegaly without liver metastases at post-mortem examination. The alkaline phosphatase could not be distinguished from the placental isoenzyme (Regan's enzyme). Increased thromboplastin time was due to circulating fibrinogen degradation products.
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PMID:[Contribution to the aetiology of "reversible hepatic dysfunction" (Stauffer's syndrome) associated with renal tumours]. 111

Malignant diseases may cause cholestatic jaundice through either main bile duct obstruction or widespread hepatic metastasis. Renal cell carcinoma (hypernephroma, RCC) can cause a variety of paraneoplastic manifestations which can be the main presenting symptoms. Cholestasis, as a paraneoplastic syndrome, has been well described in patients with malignant lymphohyperplastic diseases. Non-metastatic nephrogenic hepatic dysfunction syndrome without jaundice has often been described in patients with hypernephroma (Stauffer's syndrome). Paraneoplastic cholestatic jaundice has not yet been described. We report, for the first time, two patients who presented with pruritus and cholestatic jaundice. During the diagnostic work-up, RCC was diagnosed. The renal tumour was an unexpected finding during computed tomographic (CT) scan. No clinical manifestations of hypernephroma, short of microscopic haematuria, were detected. Conjugated bilirubin, alkaline phosphatase and gamma-glutamyltranspeptidase were markedly increased. No hepatic metastasis or main bile duct obstruction were detected by appropriate investigations. After radical nephrectomy, liver abnormalities disappeared rapidly. We conclude that RCC should be included among neoplasms causing not only anicteric intrahepatic cholestasis but also frank jaundice as part of a paraneoplastic syndrome. The differential diagnosis from hepatic metastasis, main bile duct obstruction or other causes of jaundice is of clinical importance and of prognostic value. Patients with unexplained cholestasis should be investigated for malignant diseases including hypernephroma.
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PMID:Cholestatic jaundice as a paraneoplastic manifestation of renal cell carcinoma. 909 37

Malignancies may cause cholestatic jaundice through well-recognized mechanisms (e.g., bile duct obstruction or widespread hepatic infiltration). Paraneoplastic syndromes associated with malignancy, particularly with renal cell carcinoma (Stauffer's syndrome) and malignant lymphoproliferative diseases, can induce a reversible form of cholestasis through an unclear pathogenetic mechanism. Prostate cancer presenting initially with cholestatic jaundice without any obvious cause (i.e., obstruction or infiltration) has been reported in 2 cases in the medical literature. We report a patient who presented with pruritus and cholestatic jaundice. During the diagnostic work-up, prostate cancer was diagnosed. Conjugated bilirubin and alkaline phosphatase levels were increased markedly with modest increases of gamma-glutamyltranspeptidase and transaminase levels. The results of appropriate investigations performed during the patient's hospitalizations indicated no evidence of hepatic metastases or extrahepatic biliary obstruction. After treatment with flutamide and leuprolide, the patient's symptoms and the laboratory abnormalities reversed rapidly. We regard the cholestatic jaundice of this patient as part of a paraneoplastic syndrome; the cause of cholestasis remains an enigma. Patients with unexplained cholestasis should be investigated for malignancies, including prostate cancer.
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PMID:Cholestatic jaundice as a paraneoplastic manifestation of prostate adenocarcinoma. 1501 48

Cholestasis is a common feature of several malignant diseases, including pancreatic, hepatic, gallbladder, and ampullary carcinomas. It is usually secondary to main bile duct obstruction or widespread hepatic metastasis, but it can also be a paraneoplastic syndrome of other underlying malignancies. Stauffer's syndrome is a rare paraneoplastic manifestation of renal cell carcinoma (RCC) that is characterized by elevated alkaline phosphatase, erythrocyte sedimentation rate, alpha-2-globulin, and gamma-glutamyl transferase, thrombocytosis, prolongation of prothrombin time, and hepatosplenomegaly, in the absence of hepatic metastasis and jaundice. A rare variant of this syndrome with jaundice has recently been described in 3 cases in the literature. We report a patient who presented with abdominal pain and cholestatic jaundice in whom RCC was incidentally found during initial workup. Jaundice and liver dysfunction resolved completely after surgical resection of the tumor. This case illustrates the protean manifestations of RCC, and the importance of considering Stauffer's syndrome and its variant in the differential diagnosis of anicteric and icteric cholestasis, which may allow early recognition and treatment of an underlying malignancy.
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PMID:Stauffer's syndrome variant with cholestatic jaundice: a case report. 1680 61

Nonmetastatic hepatic dysfunction known as Stauffer's syndrome is a rare entity. It is commonly associated with clear cell carcinoma of kidney. Rarely it has been reported in cases of prostatic carcinoma, bronchogenic tumor and lymphoproliferative diseases. Stauffer s syndrome presents as elevated alkaline phosphatase, aminotransferases, and prolonged prothrombin time without jaundice. However a very rare variant of Stauffer's syndrome presenting with jaundice has been reported in few case reports of clear cell carcinoma of kidney. But such a presentation in cases of renal sarcomas has not been reported so far. Here we report a unique case of primary synovial sarcoma of kidney presenting as nonmetastatic cholestatic jaundice.
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PMID:Paraneoplastic hepatic dysfunction with jaundice in a case of primary renal synovial sarcoma: A very rare scenario. 3121 Oct 52