Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033687 (proteinuria)
 24,015 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Therapy-related leukemia and myelodysplastic syndrome (TRL/MDS) in Japan were analysed in a multi-institution study to assess clinical, cytogenetic aspects, and prognostic factor. From 1985 to 1994, 405 cases of adult TRL/MDS were diagnosed and overall percentage of TRL/MDS in leukemia and MDS was 1.9%. Median age was 61 years old. The median latency from primary malignancies was 53.4 months, which latency was significantly shorter in the patients treated with chemotherapy. Primary malignancies were hematologic in 39%. Common symptoms were fatigue/ weakness and anemia. Chromosome 7,5, and 11 were frequently involved. MLL gene rearrangement were detected in 12 of 64 analysed cases. Overall median survival was 10.0 months. Body weight loss, neurologic abnormality, hypoproteinemia, hypofibrinogenemia, proteinuria, lack of Auer rods, and 5q-were prognostic factors in TRL/MDS. This large population study documented some datas useful for the prevention of TRL/MDS.
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PMID:[Therapy-related leukemia and myelodysplastic syndrome: a multi-institution study in Japan]. 946 95

Objective: Acute fatty liver of pregnancy (AFLP) is an uncommon, potentially fatal disorder that usually occurs in the late third trimester of pregnancy. We present the first reported case of acute fatty liver in the second trimester of pregnancy.Methods: We report the clinical and laboratory findings in a patient with AFLP who presented in the second trimester of pregnancy.Results: A 37-year-old G5P4 woman presented at 22 weeks gestation (by 18 weeks ultrasound) with nausea and vomiting. She was normotensive, had no proteinuria, had elevated SGOT and SGPT (266 and 261, respectively), negative hepatitis studies and a normal platelet count. She was managed conservatively for presumed cholelithiasis until 24 weeks gestation when she was transferred to our facility because of worsening SGPT and SGPT (368 and 505, respectively), jaundice (total bilirubin of 8.9 mg/dL), hypoglycemia, and laboratory evidence of disseminated intravascular coagulation (DIC) (PT = 18.6, PTT = 56, hypofibrinogenemia and presence of fibrin split products). Ultrasound showed singleton fetus (EFW 450 g) with total placenta previa. Computed tomography scan of the abdomen revealed decreased hepatic density consistent with AFLP. Delivery of a nonviable fetus was effected after transfusion of fresh frozen plasma. Postoperatively, the patient had rapid resolution of DIC, jaundice, and hypoglycemia; liver transaminases normalized 5 days postoperatively and the patient was discharged home in good condition 5 days later.Conclusion: It has been traditionally stated that AFLP occurs in the late third trimester of pregnancy. This case demonstrates that, even in the second trimester of pregnancy, the diagnosis of AFLP should be considered as a cause of deteriorating liver function, jaundice, and DIC.
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PMID:Acute fatty liver in the second trimester of pregnancy. 1083 61

Hemophagocytic lymphohistiocytosis (HLH) is a heterogeneous disorder characterized by excessive activation and proliferation of nonmalignant histiocytes, which are commonly found in bone marrow, lymph nodes, spleen and liver in affected patients. Here, we report the presence of glomerular macrophages, including one showing erythrophagocytosis, on renal biopsy in a 25-year-old patient with clinical presentation and laboratory changes consistent with HLH. The clinical course was marked by persistent fever for 2 months, pleural and pericardial effusion, splenomegaly, lymphadenopathy, pancytopenia, cardiac arrhythmias, multiple organ dysfunction, and proteinuria, with demise after a 2-month hospitalization. Positive assay for Epstein-Barr virus (EBV), marked hyperferritinemia, hypofibrinogenemia, hypertriglyceridemia, elevated anti-nuclear antibody, proteinuria, and decreased circulating NK cells by flow cytometry were compatible with the diagnosis of HLH. We suggest that the glomerular hemophagocytic macrophages, which have not heretofore been described in the kidney of a patient with HLH, may have contributed to renal dysfunction manifest as proteinuria.
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PMID:Glomerular hemophagocytic macrophages in a patient with proteinuria and clinical and laboratory features of hemophagocytic lymphohistiocytosis (HLH). 2203 68

To investigate the clinical features of adult patients with hemophagocytic lymphohistiocytosis (HLH) and to explore possible risk factors for death, we retrospectively reviewed the medical records of 103 adult HLH patients hospitalized from 1997 to 2012. We analyzed the underlying diseases, clinical characteristics, laboratory findings, outcomes, and prognostic factors. The most common cause of HLH was hematologic malignancies (n = 49), followed by infectious diseases (n = 24) and autoimmune disorders (n = 14); 24 cases were of unknown etiology. Eight patients had a combination of underlying diseases. HLH was clinically characterized by high fever (96.1%), splenomegaly (79.6%), hepatomegaly (65.0%), lymphadenopathy (53.4%), proteinuria (31.1%), skin rash (25.2%), gastrointestinal hemorrhage (14.6%), disseminated intravascular coagulation (13.6%), increased creatinine (7.8%), and central nervous system involvement (12.6%) including altered mental status (9.7%) and cranial hemorrhage (2.9%). Laboratory abnormalities included cytopenia (99.0%), serum ferritin >500 ug/L (98.4%), liver dysfunction (98.1%), hypertriglyceridemia (88.5%), hemophagocytosis in bone marrow smear (87.4%), and hypofibrinogenemia (60.9%).In addition to the treatment they received for the underlying causes, patients received therapy for HLH consisting of corticosteroids, immunosuppressive drugs, and intravenous immunoglobulin. Twenty-six patients (25.2%) recovered after treatment, and 19 of them achieved long-term remission during follow-up. Seventy-seven patients (74.8%) died because of tumor, sepsis, multiple organ failure, or HLH-related organ hemorrhage and coagulopathy. The deceased patients were more likely to be older at disease onset, male, and to present with splenomegaly and thrombocytopenia, compared to the survivors. Treatment for the underlying diseases combined with corticosteroids, immunosuppressive agents, and immunoglobulin therapy may improve the prognosis of HLH. More attention should be paid to high-risk patients to prevent the development of serious complications associated with HLH.
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PMID:Hemophagocytic lymphohistiocytosis: clinical analysis of 103 adult patients. 2464 66