Gene/Protein Disease Symptom Drug Enzyme Compound
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 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Intrahepatic cholestasis is characterized by a decrease in bile flow in the absence of overt bile duct obstruction, resulting in the accumulation of bile constituents in the liver and blood. Various etiological factors have been incriminated including drugs, total parenteral nutrition, sepsis, pregnancy, graft-versus-host disease and systemic disorders such as sarcoidosis, amyloidosis and Hodgkin's disease. The pathogenesis of cholestasis is unclear and several mechanisms have been hypothesized, without convincing evidence that any of these play a role in clinical cholestasis. Despite the uncertainty about the pathophysiology of intrahepatic cholestasis, several forms of therapy have been employed. Ursodeoxycholic acid may relieve pruritus and lethargy, and in some cases may modify disease progression. If cholestasis persists, supportive therapy is important to maintain optimal physical and nutritional well-being. In patients with advanced liver disease associated with hepatocellular failure, liver transplantation is the only viable option.
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PMID:Intrahepatic cholestatic syndromes: pathogenesis, clinical features and management. 1043 57

Although involvement of the liver is common in systemic amyloidosis, clinical manifestations of hepatic dysfunction and liver biochemical abnormalities are often absent or only mild. Here we report on a patient with primary amyloidosis and rapid development of liver failure, who was successfully treated by liver transplantation. The patient is a 61-year-old Swedish man who was admitted to the local hospital for spontaneous rupture of the spleen. Before admission, he had suffered from diffuse upper abdominal discomfort, diminished appetite, and had lost 15 kg in 6 months. Shortly after splenectomy, he developed cholestatic liver failure with moderate hepatomegaly, jaundice, ascites and hyponatremia. Over a period of 3 weeks his liver failure progressed, renal function deteriorated rapidly, and he developed encephalopathy. Liver transplantation was performed on the 35th day after splenic rupture. Histological examination revealed extensive deposits of amyloid in the spleen and liver. N-terminal amino acid sequence analysis of the amyloid protein, purified from the patient's native liver, revealed an AL protein of kappa I-type origin. The postoperative course was uncomplicated, apart from one episode of sepsis and one course of treatment for acute rejection. He was discharged from hospital with normal liver function and good kidney function. One year after surgery, he was in good condition, with normal liver function. However, a liver biopsy taken at the same time showed de novo amyloid deposits in the grafted liver. We conclude that liver transplantation may be indicated as a life-saving procedure in rapidly progressing hepatic amyloidosis with cholestatic jaundice, although the underlying disease has not changed.
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PMID:Liver transplantation as rescue treatment in a patient with primary AL kappa amyloidosis. 1083 44

Renal involvement in 204 cases with multiple myeloma admitted over a 10-year period to this tertiary care center in north India was retrospectively examined. Renal involvement occurred in 55 cases (27%); the vast majority of whom (94.5%) had presented with renal failure and 7.3% had nephrotic syndrome. The diagnosis of multiple myeloma was made after admission in 51 of the 55 (92.7%) cases. Oliguria was seen in 23.6% and two-third patients required dialysis. Factors precipitating renal failure were identified in 53% and included dehydration (33%), hypercalcemia (24%), nephrotoxic drugs (16%), sepsis (9%), recent surgery (5%) and contrast media (2%), Severe anemia, hypercalcemia, Bence Jones proteinuria and skeletal abnormalities were more frequent in those with renal involvement. Patients with renal involvement were more likely to have a high tumor burden. The myeloma was of light chain type in 68% of those with renal involvement whereas IgG myeloma was commonest (57%) in those without evidence of renal disease. Renal histology was studied in 27 cases with myeloma cast nephropathy seen in over 60%. Tubulointerstitial nephritis was seen in 14% cases, 11% had amyloidosis, 7% had acute tubular necrosis and 3.6% each had nodular glomerulosclerosis and plasma cell infiltration. In 8 cases (14.6%), renal biopsy provided the first clue to the diagnosis of myeloma. Renal function improved in 33% cases. Only 22% of patients on dialysis survived over 6 months. Median survival in those with renal involvement was only 4 months. Development of unexplained renal failure in an elderly individual with normal sized kidneys, in association with disproportionate anemia even in the absence of skeletal lesions should alert the physician to the diagnosis of multiple myeloma.
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PMID:Renal involvement in multiple myeloma: a 10-year study. 1090 Nov 84

Familial amyloid polyneuropathy type I (FAP-I) is an inherited amyloidosis secondary to systemic deposition of amyloid fibrils containing mutant transthyretin (TTR) variants. The disease has a progressive clinical course and is usually fatal 10 years after its onset. TTR is mainly produced in hepatocytes, and liver transplantation (LT) has been proposed as an effective treatment for FAP-I. The aim of this study is to evaluate the results of LT for FAP-I in Brazil and analyze prognostic factors associated with survival after surgery. Twenty-four patients (median age, 36 years; range, 25 to 52 years) who underwent LT with the diagnosis of FAP-I were evaluated. Surgery was uneventful in all but six patients who died of complications of primary liver nonfunction (n = 1), cardiogenic shock (n = 1), sepsis (n = 3), and hepatic artery thrombosis (n = 3). Overall 1- and 5-year survival rates were 70% and 58%, respectively. Most patients had stabilization or improvement of symptoms after a median follow-up of 36 months (range, 14 to 82 months). Survivors had a shorter disease duration before LT (median, 6 years; range, 2 to 17 years v 9 years; range, 7 to 12 years; P =.02), greater albumin levels (median, 4 g/dL; range, 3 to 4.7 g/dL v 3.6 g/dL; range, 2.6 to 4.1 g/dL; P =.03), and greater modified body mass index scores (median, 735; range, 502 to 1,432 v 659; range, 411 to 803; P =.04) compared with nonsurvivors. However, only disease duration and albumin levels were independently associated with survival in multivariate analysis. In conclusion, LT is an effective therapy for FAP-I. Mortality after surgery is associated with poor nutritional status and long-standing disease before LT. Thus, LT should be performed as early as possible after the onset of FAP-I symptoms to avoid major disability and improve survival.
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PMID:Results of liver transplantation for familial amyloid polyneuropathy type I in Brazil. 1179 83

Bronchiectasis is defined as acquired and permanent abnormal dilation and destruction of the bronchial walls. Secondary amyloidosis is a disorder characterized by the deposition of amyloid A (AA) in multiple organs and tissues in the body. End-stage renal disease (ESRD) secondary to bronchiectasis-related amyloidosis has only been mentioned in case reports. Little is known about the complications of bronchiectasis-related amyloidosis and the outcomes in patients who develop ESRD due to amyloid deposition in the kidneys. The aim of this study was to identify the clinical characteristics of this patient group, and to report the outcomes of these cases relative to bronchiechtasis type. We assessed the records of 40 patients with AA-type amyloid nephropathy and ESRD who were on hemodialysis (HD) at Baskent University Hospital between 1997 and 2000. The diagnosis of amyloidosis was based on histopathological findings in kidney, rectum, bone marrow, lymph node, thyroid, bladder, liver, and stomach biopsies. Bronchiectasis was diagnosed on the basis of history and findings on physical examination, chest X-ray, and thoracic high-resolution computerized tomography (HRCT). The patients' records were retrospectively evaluated for cause of secondary amyloidosis, and cases with causes other than bronchiectasis were excluded. Secondary amyloidosis due to bronchiectasis and recurrent pulmonary infection was identified in 40% (16 patients) of the 40 patients. For each of these 16 cases, we recorded patient age, duration of bronchiectasis, duration of HD, amount of lung involvement, and biopsy site(s). The means for age, duration of bronchiectasis, and duration of HD in the 16 patients were 50.6 +/- 13.5 years, 22.18 +/- 12.02 years (range, 6-42 years), and 30.81 +/- 36.94 months (range, 4-144 months), respectively. The most common biopsy site was the rectum (n = 8). Thoracic HRCT revealed cystic bronchiectasis in 8 cases (50%). Four of these 8 patients (25% of the group of 16) died from sepsis within 3-year follow-up period. Two of the four patients who died had bilateral cystic bronchiectasis, and the other two had unilateral cystic bronchiectasis. In the other eight patients in the group, thoracic HRCT showed chronic fibrotic changes in the pulmonary parenchyma and minimal traction bronchiectasis. Four of these patients exhibited apical fibrosis and bronchiectasis (25% of the group of 16), and these radiological findings were considered sequelae of previous tuberculosis infections. In conclusion, chronic respiratory infections and associated bronchiectasis remain a serious problem in Turkey due to insufficient prevention, diagnosis, and treatment. It is important that patients with progressive cystic and diffuse bronchiectasis be followed carefully, as they may develop amyloidosis and ESRD in time. Also, the clinical course in patients with cystic bronchiectasis may be worse than that in other types of bronchiectasis due to complicating pulmonary infections.
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PMID:Bronchiectasis-related amyloidosis as a cause of chronic renal failure. 1247 3

Few cases of combined heart and liver transplantation (CHLT) for familial amyloidotic polyneuropathy have been reported, and the technique for the operation is far from being consolidated. Three patients with amyloidogenic transthyretin (ATTR)-related (variant Glu89Gln to ATTR Glu89Gln) cardiomyopathy underwent CHLT at our institution. Patient 1 had no serious involvement of other organs, whereas patients 2 and 3 had evident peripheral neuropathy and gastrointestinal motility alterations. Patient 3 also had high-grade orthostatic hypotension. All three patients underwent cardiac and sequential hepatic transplantation using the piggyback technique with organs procured from the same donor. Venovenous bypass (VVB) was used only in patient 1, with an uncomplicated procedure. After CHLT, his cardiac performance remained normal, and no progression of amyloidosis was observed. Patient 2 had no intraoperative complications, but experienced postoperative bleeding, renal failure, sepsis, and heart failure and eventually died of multiorgan failure 2 months after transplantation. In patient 3, right hemicolectomy was required intraoperatively because of intestinal ischemia without significant hemodynamic perturbations, whereas extracardiac symptoms of amyloidosis gradually worsened postoperatively. Two patients (no. 1 and 3) currently are alive after 38 and 18 months, respectively. CHLT for ATTR Glu89Gln can be performed successfully, even in patients with advanced disease. However, the most compromised patients are more exposed to intraoperative risks, postoperative complications, and worsening of extracardiac and extrahepatic symptoms. The need for VVB remains to be evaluated.
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PMID:Combined heart and liver transplantation for familial amyloidotic neuropathy: considerations from the hepatic point of view. 1294 63

Hepatic involvement in primary amyloidosis (AL type) is not rare but is often clinically silent. However, presentation with jaundice in AL-type amyloidosis is rare, with an incidence of less than 5% reported in the literature. It is considered to be a preterminal sign. We herein report on a case of primary hepatic amyloidosis presenting with severe intrahepatic cholestasis. Viral, drug, alcohol, and autoimmune etiologies were all excluded. A liver biopsy was performed because of unexplained cholestatic jaundice for 3 months. The pathology showed hepatic amyloidosis with extensive amyloid deposition in the expanded portal tracts and sinusoidal space. The patient received supportive treatment only, because of persistent jaundice, coexistent colon cancer with para-aortic lymph node metastasis, and possibly peritoneal carcinomatosis. Unfortunately, the patient died of sepsis 10 months after the onset of jaundice. We suggest that hepatic amyloidosis must be considered in the differential diagnosis of unexplained cholestatic jaundice.
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PMID:Severe intrahepatic cholestasis in an elderly patient with primary amyloidosis and colon adenocarcinoma. 1507 94

There are few reports of combined heart and liver transplantation (CHLT) for familial amyloidotic polyneuropathy (FAP). The technique for the operation remains to be defined. Four CHLTs were performed for amyloidogenic transthyretin-related (variant Glu89Gln-ATTR Glu89Gln) cardiomyopathy in our center. Patients 1 and 4 had no serious involvement of other organs, whereas patients 2 and 3 had evident peripheral neuropathy and gastrointestinal motility alterations. Patient 3 also had high-grade orthostatic hypotension. All four patients underwent cardiac and sequential hepatic transplantation with organs procured from the same donor. Venovenous bypass was used in patients 1 and 4 who experienced uncomplicated procedures. The amyloidotic liver of patient 4 was successfully utilized for a domino procedure to treat a patient with hepatocellular carcinoma on cirrhosis. The cardiac performance of patients 1 and 4 remains normal; there has been no progression of amyloidosis at 42 and 1 months after transplantation. Patient 2 had no intraoperative complications but experienced postoperative bleeding, renal failure, sepsis, and heart failure, and finally died of multiorgan failure 2 months after transplant. In patient 3, right hemicolectomy was required intraoperatively due to intestinal ischemia, without significant hemodynamic instability, while extracardiac symptoms of amyloidosis gradually worsened postoperatively. In conclusion, CHLT for ATTR Glu89Gln may be performed even in patients with advanced disease. However, the most compromised patients are more likely to display intraoperative risks, postoperative complications, and worsening of extracardiac, extrahepatic symptoms.
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PMID:Combined heart and liver transplantation in four adults with familial amyloidosis: experience of a single center. 1511 Jun 20

A young female with Fisher-Evans' syndrome and a previous melanoma developed acute renal failure with generalized lymphoadenopathy and fever. The appearance of renal lesions is common in the course of several hematological disorders, but is unusual in Fisher-Evans' syndrome. Fisher-Evans' syndrome, defined as Coombs' positive hemolytic anemia and immune thrombocytopenia, is more frequently associated with the other autoimmune diseases, but not with renal involvement. In our case report, having excluded amyloidosis, myeloma, interstitial nephritis and sepsis, the rapid involvement of renal function with enlarged renal size seemed to suggest renal lymphoma. However, the lack of a monoclonal T-lymphocyte population in the renal tissue and peripheral blood, along with a clinical course characterized by a rapid reversibility of acute renal failure made this diagnosis rather an unlikely one. Polyclonal lymphocyte infiltration in a patient with a persistent autoimmune disease made us suspect a hyperimmune reaction. This syndrome is a non-neoplastic proliferation of B-cells involving an exaggeration of lymphocyte transformation. However, the clinical course is progressive and fatal, and can trigger a lymphoproliferative systemic disease. In our patient, two elements led us to suspect it was not a typical hyperimmune syndrome: first, polyclonal lymphocytes had massively infiltrated the kidney and, secondly, the clinical outcome was extremely favorable. Therefore, we were faced with an "atypical" and "singular" hyperimmune reaction with renal involvement, polyclonal proliferation of T-lymphocytes that had exhausted itself over time. Infective or toxic agents or drugs such as cyprofloxacin could have triggered the phenomenon, in the presence of a favorable condition such as Fisher-Evans' syndrome.
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PMID:Acute renal failure in a young woman with Fisher-Evans' syndrome. 1559 44

Compared to young patients with Takayasu's arteritis (TA), little information about elderly patients with TA has been reported. Additionally, no reports were found regarding TA cases with complications of intestinal amyloidosis. This is a case report of an elderly female, who developed intestinal amyloidosis, during late-stage TA. After years of outpatient management, she developed sudden severe dyspnea with pulmonary effusion, requiring hospitalization. After this event, betamethasone was replaced by methotrexate (MTX) for the next 34 months, but it seemed ineffective. After 1.5 years, she developed intractable diarrhea, followed by increases in BUN and serum creatinine (Cr), requiring several courses of hemodialysis. Colonoscopy revealed the presence of amyloid in her intestine, although she died of complicated sepsis caused by MRSA infection. This may be the first paper describing intestinal amyloidosis in a TA patient. Additionally, her case is rare in that she lived more than 30 years after the onset and diagnosis of TA.
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PMID:An elderly female who survived more than 30 years following a diagnosis of Takayasu's arteritis, complicated by fatal intestinal amyloidosis. 1629 68


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