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)

Allogeneic stem cell transplantation (allo-SCT) using related or unrelated donor could eradicate paroxysmal nocturnal hemoglobinuria (PNH) clones and may cure the disease. Chronic graft-versus host disease (GVHD) is a major complication of patients who have undergone allo-SCT. Nephrotic syndrome has been described as one of the rare manifestations of chronic GVHD following the usual myeloablative allo-SCT. We report a case of nephrotic syndrome that developed 25 months after non-myeloablative allo-SCT for PNH. The patient had grade II acute GVHD and extensive chronic GVHD after non-myeloablative allo-SCT. Typically the patient presented with preserved renal function and full nephrotic syndrome including generalized edema, proteinuria, hypoalbuminemia, and hypercholesterolemia. Renal biopsy revealed findings of membranous glomerulopathy (MG). The patient is alive with a stable engraftment and full donor chimerism under the administration of tacrolimus for control of chronic GVHD and MG without refractory hemolysis and cytopenia.
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PMID:Membranous glomerulopathy as a manifestation of chronic graft-versus-host-disease after non-myeloablative stem cell transplantation in a patient with paroxysmal nocturnal hemoglobinuria. 1467 53

BACKGROUND: Black and African patients with type 2 diabetes have a greater frequency and more severe vascular complications of the disease, even after correction for socioeconomic factors. Asymptomatic sickle cell trait (SCT; hemoglobin AS) is also common among black Africans and may independently cause endothelial damage, manifested as isolated target organ complication or infarction. We examined the possibility that patients with concurrent type 2 diabetes and SCT may be predisposed to more frequent or severe diabetic macro- or microvascular complications than those without SCT. METHODS: Fifty-two type 2 diabetic patients were divided into four groups, according to gender and hemoglobin genotype (normal: AA or SCT: AS). The groups were well matched for age and for clinical and demographic parameters. Diabetic complications were assessed in each patient and scored. Hemoglobin genotype was determined by hemoglobin-gel electrophoresis. Statistical comparisons were made between the groups. RESULTS: The composite complication score for vascular disease differed significantly according to gender and genotype (p<0.027 ANOVA). Male diabetics with SCT had a higher risk ratio (RR 1.6, p<0.02) for complications than those with normal hemoglobin; however, this was not the case with female diabetics. Among the male diabetics with SCT, there was a significantly greater proportion with proteinuria (p<0.02) or retinopathy (p<0.05) than among those with a normal hemoglobin genotype. Multiple regression analysis showed that gender and SCT were independent predictors of the vascular complication severity score and that exclusion of hemoglobin genotype weakened the predictability of the regression. A significantly higher proportion of male than female diabetics had at least one detectable complication. Systolic or diastolic blood pressure had no significant impact on the regressions. CONCLUSION: Male gender and SCT may adversely affect the expression of microvascular diabetic complications in Africans. Diabetic patients from populations predisposed to the sickle gene should be screened for the trait as part of their initial risk assessment. Large-scale studies on the impact of hemoglobin genotype on diabetic complications are clearly indicated.
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PMID:Sickle cell trait and gender influence type 2 diabetic complications in African patients. 1545 Sep 89

High-dose melphalan followed by stem cell transplantation (HDM-SCT) has become the treatment of choice for patients with immunoglobulin light chain amyloidosis (AL). Unfortunately, treatment mortality can be excessive in certain subpopulations. We have noted that patients who gained more than 2% body weight during mobilization had a poorer outcome following HDM-SCT. Excluding 2 patients for lack of weight record and denial of consent, 126 patients between July 1997 and June 2003 were retrospectively studied. Weight increased more than 2.0% during mobilization in 51.6% of the patients. Patients who accumulated more than 2.0% tended to have more proteinuria, more organs involved, lower serum albumin, more diuretic use, and dosage adjustment during mobilization. First-year mortality was significantly higher in those with more than 2% weight gain (33.9% versus 9.8%, P = .002). Multivariate analysis showed weight gain, glomerular filtration rate, and septal thickness to be independent predictors of first-year mortality. The increase in mortality was noted even after the excess weight was diuresed prior to conditioning. The impact on mortality dissipated after the first year. Weight gain during mobilization appears to be a new marker of adverse outcome following HDM-SCT. Better prognostication may improve the treatment mortality rate of these patients.
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PMID:Excessive fluid accumulation during stem cell mobilization: a novel prognostic factor of first-year survival after stem cell transplantation in AL amyloidosis patients. 1603 90

A 55-year-old woman with primary Immunoglobulin light chain (AL) systemic amyloidosis died due to spontaneous rupture of her liver following treatment with high-dose melphalan and autologous stem cell transplant (HDM/SCT). She was first diagnosed after developing nephrotic-range proteinuria. Spontaneous rupture of her liver occurred 10 days after treatment with HDM/SCT and was complicated by septic shock. She was not eligible for surgical intervention and died shortly after. Amyloid fibrils were extracted from the autopsied liver sample (05-135L) and the biochemical nature of the fibrils was analyzed using electrophoretic and immunohistochemical techniques. Our testing showed that the fibrils were composed of immunoglobulin lambda light chains that were not glycosylated. While the liver is often involved in AL amyloidosis, this is the first documented case of a spontaneous hepatic rupture in a patient during treatment with HDM/SCT. A literature review of spontaneous liver rupture in patients with amyloidosis is presented.
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PMID:Spontaneous rupture of the liver in a patient with systemic AL amyloidosis undergoing treatment with high-dose melphalan and autologous stem cell transplantation: a case report with literature review. 2053 4

Patients undergoing auto-SCT for neuroblastoma present a unique population to study transplant-associated thrombotic microangiopathy (TA-TMA), due to standardized chemotherapy and later exposure to radiation and cis-retinoic acid (cis-RA). We retrospectively analyzed 20 patients after auto-SCT to evaluate early clinical indicators of TA-TMA. A total of 6 patients developing TA-TMA (30% prevalence) were compared with 14 controls. Four of six patients were diagnosed with TA-TMA by 25 days after auto-SCT. Compared with controls, TA-TMA patients had higher average systolic and diastolic blood pressure levels during high-dose chemotherapy and developed hypertension by day 13 after auto-SCT. Proteinuria was a significant marker for TA-TMA, whereas blood and platelet transfusion requirements were not. Serum creatinine did not differ between groups post transplant. However, patients with TA-TMA had a 60% decrease in renal function from baseline by nuclear glomerular filtration rate, compared with a 25% decrease in those without TA-TMA (P=0.001). There was no TA-TMA-related mortality. Significant complications included end-stage renal disease (n=1) and polyserositis (n=3). Patients with TA-TMA were unable to complete cis-RA therapy after auto-SCT. We suggest that careful attention to blood pressure and urinalysis will assist in the early diagnosis of TA-TMA, whereas serum creatinine seems to be an insensitive marker for this condition.
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PMID:Early clinical indicators of transplant-associated thrombotic microangiopathy in pediatric neuroblastoma patients undergoing auto-SCT. 2069 72

Our previous data have revealed that proteinuria, antinuclear antibodies and anti-dsDNA antibodies in refractory systemic lupus erythematosus (SLE) reduced after MSC infusion. This study focused on the roles of mesenchymal SCT (MSCT) in SLE patients with refractory cytopenia. Thirty-five SLE patients with refractory cytopenia were enrolled in a MSCT trial. Hematological changes of pre- and post-transplantation were evaluated. Mechanisms for MSCT effects focused on the analysis of percentage of regulatory T cells (Treg) and Th17. The results showed that in 35 SLE patients, 20 patients had leukopenia, 24 with anemia or thrombocytopenia. The average follow-up period after MSCT was 21 months (range 6-45 months). Significant improvements in blood cell count were found after MSCT for most patients, in parallel with the decline of disease activity. Clinical remission was accompanied by increased Treg and decreased Th17. Two patients died of uncontrolled disease recurrence after infection, whereas no adverse events related to transplantation was observed. The result suggested that MSCT could reverse hematological aberration in SLE patients with refractory cytopenia, which might be associated with reconstitution of Treg and Th17. Longer follow-up and clinical larger-scale controlled study, as well as the exact mechanism exploration, will need further investigations.
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PMID:Mesenchymal SCT ameliorates refractory cytopenia in patients with systemic lupus erythematosus. 2306 40