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)

The natural history of brittle diabetes is unknown. We have followed up 13 patients with disabling brittle diabetes unresponsive to continuous subcutaneous insulin infusion (CSII) for 3-6 yr. All were young, C-peptide deficient females. One patient has died (of hypoglycaemia). In the others, disruption of life has generally lessened, but only one patient is currently considered metabolically stable. Insulin treatment regimens have included long-term intravenous insulin infusions and intraperitoneal insulin, but all but four have now reverted to subcutaneous injections. Eleven patients intermittently required greater than 200 U/day of insulin and two have needed greater than 1,000 U/day. Insulin dosages have fallen significantly during follow-up (from 6.8 +/- 3.1 to 1.4 +/- 0.3 U/kg/day). Diabetic complications, initially present in only 2 cases (1 cataract, 1 proliferative retinopathy), have now developed in 5 others (2 background retinopathy, 1 proliferative retinopathy, 1 mixed peripheral neuropathy and 1 intermittent proteinuria). Psychosocial disturbance and non-compliance were common. We conclude that brittleness generally seems to improve, which probably explains the scarcity of older brittle patients. However, these patients are at considerable risk from diabetes, its complications and its treatment.
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PMID:The natural history of brittle diabetes. 304 51

Preemptive simultaneous kidney-pancreas transplantation (SKPT) was performed in two patients with Type 1 diabetes and nephrotic syndrome due to diabetic nephropathy, although the native kidneys exhibited near-normal function. Before and 3 months as well as 12 months after SKPT S-creatinine, creatinine clearance (Cr-Cl) and urinary protein excretion were measured. Additionally, 99mTC scintigraphic examinations of the kidneys were performed 3 and 12 months after SKPT. Thereby, the injected 99mTC activities were assessed in the kidney graft as well as in the patient's native kidneys. Aim of the study was to find out the impact of successful SKPT on proteinuria and further functioning of the patient's own kidneys after transplantation. Indication for pancreas transplantation was severe diabetic autonomic neuropathy and Brittle diabetes, respectively. In Patient 1, we registered 3 months after SKPT a near-normal protein excretion of mean 0.20 g/24-h urine at a Cr-Cl of 82 ml/ min. The scintigraphic examinations showed 60% of the radioactivity in the kidney graft and 40% in the patient's own kidneys (22% right and 18% left). Data 1 year after SKPT were: mean protein excretion 0.28 g/24-h urine, Cr-Cl 78 ml/min and in the scan, furthermore, 30% of the activity in the patient's native kidneys (16% right and 14% left). In Patient 2 after 3 months we obtained a mean protein excretion of 0.18 g/24-h urine at a Cr-Cl of 80 ml/min. Scintigram of the kidneys: 58% of the injected activity were measured in the kidney graft and 42% in the patient's own kidneys (22% right and 20% left). After 12 months of SKPT we measured a mean protein of 0.26 g/24-h urine and Cr-Cl 78 ml/min. Scintigram of the kidneys: 36% of the activity was in the patient's native kidneys (18% right and left). We conclude that in diabetic patients with nephrotic syndrome and near-normal function of the native kidneys SKPT leads to rapid and nearly complete diminution of proteinuria although the residual function of the patient's native kidneys was about 40% at 3 months after transplantation and slightly lower at 12 months after SKPT.
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PMID:Proteinuria disappears promptly after simultaneous kidney-pancreas transplantation in nephrotic diabetic patients with near-normal GFR. 1804 67