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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 49 year old West Indian man with sickle cell disease and chronic renal failure was maintained on hemodialysis for 10 months before receiving a cadaveric renal transplant. Nine months post-transplant his renal function is good. His main problem has been high HbS levels needing repeated exchange transfusions. We conclude that hemodialysis and transplantation may be use successfully performed in patients with sickle cell disease with end-stage renal failure.
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PMID:Renal transplantation in sickle cell disease. 675 98

Erythropoietin responsible for the hormonal regulation of red blood cell production. Its formation is largely controlled by the kidneys. A number of assay methods for erythropoietin are available. Asymptomatic patients with sickle cell disease have elevated erythropoietin levels, as expected with chronic hemolysis. When complicated by chronic renal failure, erythropoietin levels do not rise appropriately. Chronic infection has not been studied, but the erythropoietin response in acute infection does not seem to conform to a pattern. Aplastic crises are characterized by very high levels of erythropoietin, suggesting bone marrow suppression, but events that trigger the crises remain obscure. In vaso-occlusive crises, there is also some suggestion of mild and transient lack of bone marrow response. Patients with sickle cell disease, with their chronic high erythropoietin anemia and susceptibility to altered states, are uniquely suited for investigating the physiology of erythropoietin, especially under the constraints of present assay methods.
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PMID:Erythropoietin in sickle cell disease: relation of erythropoietin levels to crisis and other complications. 711

A 25-year-old black male with known sickle cell disease developed progressive chronic renal failure. Eight months after the initiation of chronic hemodialysis, he developed a rapidly-increasing transfusion requirement. The requirement was one unit of blood per week in order to maintain a hematocrit of 16%. His spleen was found, at that time, to be enlarged both on physical examination and radioisotopic scan. A radiochromium study disclosed a red blood cell half-life of 17 days. No evidence of splenic sequestration could be demonstrated isotopically. Splenectomy produced a marked decrease in the patient's transfusion need. Presently, this patient needs one unit of blood every 10 to 12 weeks to maintain a hematocrit of 18%.
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PMID:A beneficial effect of splenectomy in sickle cell anemia and chronic renal failure. 742 63

Between 1989 and 1995, 32 patients underwent combined kidney-pancreas transplantation for diabetic chronic renal failure. Only one of these patients received an isolated pancreas following cessation of function of a previously implanted segmental pancreas. The surgical technique always consisted of pure retroperitoneal transplantation into the right iliac fossa of a total pancreas transplant with duodenovesical anastomosis. The postoperative complications included one death on D10 from pulmonary vein thrombosis in a patient with sickle cell anaemia and early loss of the transplanted pancreas due to venous thrombosis. Nine patients underwent at least one surgical revision, due to a leaking duodenovesical anastomosis in 8 cases. With a mean follow-up of 33 +/- 20 months, the results demonstrate, apart from the early death indicated above, another death at 50 months of a patient who had lost his pancreas due to early venous thrombosis and who died with a functioning kidney. 23 of the 30 surviving patients have a functioning kidney and pancreas (79%), i.e. 74% of the total population of 32 patients. Loss of pancreatic function was surgical in two cases (one case of infection of the transplant site, one case of thrombosis), vascular in one case due to rupture of a mycotic aneurysm into the duodenum and immunological in three cases: two of these pancreases retained partial function allowing perfect blood glucose control with less than 10 units of ordinary insulin per day. Lastly, a perfectly functioning pancreas was removed 13 months after transplantation because of renal rejection not controlled by reinforced immunosuppression. Compared to the data of the international registry, these results demonstrate the value of the retroperitoneal approach used in this series and the improvement of the results obtained with increasing experience of the transplant team.
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PMID:[Combined renal-pancreas transplantation in the treatment for chronic renal insufficiency of diabetic origin . Results from the Pitie Urology Clinic]. 862 26

Healthy DHCWs do not seem to be at significantly higher risk for occupationally acquired diseases when compared with other HCWs. Special attention should be paid to DHCWs who are more susceptible to diseases potentially transmitted in a dental setting. These DHCWs include pregnant women, due to their immunologic changes, and the developing fetus; DHCWs; those with habits such as excessive intake of alcohol; DHCWs following splenectomy, radiotherapy, and long-term corticosteroid therapy; and DHCWs suffering from diseases that have an impact on the first and secondary defense against infections, such as diabetes mellitus, chronic renal failure, sickle cell anemia, leukemia, lymphoma, or HIV.
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PMID:Dental health care workers at risk. 864 21

We performed a cross sectional analysis of glomerular function in 34 adult patients with sickle cell anemia (SSA). Patients were divided according to GFR and albumin excretion rate (AER): SSA controls (normal GFR and AER, N = 10), albuminuria (increased AER, but normal GFR, N = 7) and chronic renal failure (CRF, low GFR, N = 17). GFR did not correlate with age (that is, duration of disease), but was inversely related to AER and IgG excretion rates (r = -0.61 and -0.69, respectively, P < 0.001) and directly related to the hematocrit (r = 0.56, P < 0.001). Renal plasma flow was disproportionately higher than GFR, so that filtration fraction was low in all groups. Albuminuria was accompanied, even in patients with normal GFR, by a reduction in ultrafiltration coefficient (16 +/- 3 in albuminuria vs. 25 +/- 3 in controls, P < 0.05). A more severe loss of ultrafiltration coefficient and glomerular permselectivity occurred in CRF. We conclude that renal failure in SSA occurs because of glomerular injury with loss of ultrafiltration coefficient and glomerular permselectivity. The earliest clinically detectable abnormality is an increase in albumin and IgG excretion. When albuminuria is present, the ultrafiltration coefficient is already diminished even if GFR is preserved. Detection of albuminuria can identify established glomerular injury in SSA.
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PMID:Early detection and the course of glomerular injury in patients with sickle cell anemia. 864 21

Anemia with a relatively low erythropoietin level has been described in several medical conditions associated with chronic inflammatory diseases such as rheumatoid arthritis, cancer, sickle cell disease, chronic renal failure, acquired immunodeficiency syndrome, and severe autonomic nervous system failure. This case report describes the development of anemia with a relatively low erythropoietin level in a 65-year-old man with non-insulin-dependent diabetes mellitus, normal renal function, and negative hematologic, thyroid, and autoimmune disease work-ups. The serum erythropoietin level was 14 mU/mL (N: 10-20 mU/mL). The hemoglobin was 7.5 g/dL and the hematocrit was 24%. The patient was treated with recombinant erythropoietin at 50 U/kg subcutaneously three times weekly. The hemoglobin level increased over a 4-week period. When erythropoietin was stopped, the anemia recurred in 2 months. We conclude that the patient's anemia was caused by a relative lack of endogenous erythropoietin release. The exact mechanism of this anemia is unknown. We recommend including a test for erythropoietin level in the evaluation of any unexplained anemia.
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PMID:Anemia secondary to low erythropoietin in a patient with normal renal function. 936 36

Two hundred and seventy two children with the nephrotic syndrome were seen and followed up at the Paediatric Renal Unit of the University of Nigeria Teaching Hospital, Enugu over 12 1/2 year period, between June 1983 and December 1995. Of these, there were nine (3.3%) children with homozygous sickle cell disease (SS). This latter group had a mean age of 9.6 +/- 3.2 years, male to female ratio of 1:2 and serum cholesterol of 3.13 +/- 1.48 mmol/l at the time of diagnosis. The mortality rate was 55.6% in these sicklers, death occurring within one and a half to five years of diagnosis. Causes of death were attributable to sickle cell disease in 60%, renal diseases in 20%, and other causes in 20%. Renal biopsy in two of the sicklers showed membrano-proliferative glomerulonephritis (MPGN) and focal glomerulosclerosis (FGS) respectively. Homozygous sickle cell disease does seem to predispose to the development of the nephrotic syndrome and those that do develop nephrotic syndrome exhibit some special characteristics, when compared to non-sicklers with nephrotic syndrome. These include older age of onset of the nephrotic syndrome, reverse sex ratios, lower serum cholesterol, higher mortality rate and sickle cell complications rather than chronic renal failure as the major cause of death.
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PMID:Nephrotic syndrome in Nigerian children with homozygous sickle cell disease. 955 31

The correction of anemia in patients with chronic renal failure (CRF) has become the most important application of recombinant human erythropoietin (rHuEpo). The merits of rHuEpo therapy in patients with CRF are overt. Firstly, patients with CRF have an absolute deficiency in endogenous erythropoietin production and a relatively low maintenance dose of rHuEpo (often less than 100 IU/kg body weight per week) is effective in avoiding regular transfusions in the majority of the patients with CRF. Secondly, rHuEpo is able to avoid long-term complications of frequent transfusions (hemochromatosis, transfusion-transmissible diseases). Thirdly, patients with uremia notice a considerable improvement in quality of life (QOL) after initiation of rHuEpo. These advantages justify administration of this costly drug in CRF patients. The use of rHuEpo outside the setting of uremia do, however, not cover the complete spectrum of beneficial effects as compared to its use in (pre)dialysis patients. The aim of this overview is to provide some annotations on recently approved (cisplatin-induced anemia, preoperative anemia, zidovudine-related anemia) and possibly future (several types of malignancy and inflammation) indications for rHuEpo in non-uremic patients, leaving out the correction of anemia due to relatively uncommon disorders in the Dutch population (such as sickle cell anemia and thalassemia).
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PMID:Erythropoietin treatment for non-uremic patients: a personal view. 1004 90

In a prospective study spanning 12 1/2 years (July 1983 to December 1995), 272 children with nephrotic syndrome seen at the University of Nigeria Teaching Hospital Enugu, Nigeria, were followed up and reviewed at the end of the study period. The demographic, clinical and laboratory features, response to treatment and prognosis were documented. Nephrotic syndrome made up 1.34% of all paediatric admissions. There were 164 males and 108 females giving a male to female ratio of 1.5:1. The ages ranged from 2 to 16 years, with a mean of 7.9 +/- 3.4 years and peak age of 5-7 years. The major clinical features were generalized oedema (100%), hypertension (23%), fever (20%), oliguria (10%) and cough (7%). Haematuria was present in 26%, mean serum albumin was 16 +/- 5, 1 gm/L, serum cholesterol 9.53 +/- 1.6 mmol/L Malaria parasitaemia was present in 38.7% and 9 patients (3.3%) had sickle cell disease (SS). Treatment with diuretics, pooled plasma, prednisolone or cyclophosphamide in various combination achieved 63.9% remission. Mortality was 5.5% being mainly due to chronic renal failure, hypertension and infections. The study calls for more trials in the use of steroids and cyclophosphamide in the treatment of childhood nephrotic syndrome in the tropics.
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PMID:Childhood nephrotic syndrome in Enugu, Nigeria. 1107 Jul 50


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