Enalapril reduces the albuminuria of patients with sickle cell disease.

PURPOSE: To evaluate the effects of enalapril, an angiotensin-converting enzyme inhibitor, on albuminuria associated with sickle cell anemia. PATIENTS AND METHODS: Two males and 6 females, mean age 22.8 +/- 5.5 years, with sickle cell anemia and albuminuria, received enalapril for 6 months. Before entry into the study, all had a urinary albumin concentration above 30 mg/L as determined by radioimmunoassay documented on three separate occasions at intervals of 15 to 30 days. Samples were collected before 10 AM after an oral water load of 10 mL/kg. RESULTS: Enalapril reduced 6 patients' pretreatment hyperalbuminuria to normal. One patient whose levels did not reach normal values experienced a reduction of 70%. Fractional excretion of sodium, potassium, and lithium did not change during the treatment. Mean arterial pressure decreased by 8.6 +/- 0.42 mm Hg. Two years after enalapril was discontinued, there were no changes in sodium, potassium, or creatinine levels of 7 patients who had received enalapril or in their mean arterial pressures. Urinary albumin concentration increased relative to pretreatment levels in 2 individuals, returned to pretreatment levels in 2, and remained below 30 mg/L in 2. CONCLUSION: Our results demonstrate that enalapril reduces albuminuria in patients with sickle cell anemia. After discontinuation of the drug, however, the albuminuria may increase to pretreatment levels or higher. Whether the reduction in urinary albumin concentration by angiotensin-converting enzyme inhibitors can delay the development of progressive renal failure in sickle cell anemia patients remains to be established.

Acute Megakaryoblastic Leukemia: Importance of Bone Marrow Biopsy in Diagnosis.

Five cases of megakaryoblastic leukemia, presenting as "de novo" acute leukemias are reported. They represented 7.2% of all cases of adult acute non-lymphoblastic leukemias (ANLL) seen in our Hematology Service during the past 30 months. These cases showed features of predominantly blast proliferation with a marked increase of reticulin fibres in the bone marrow. One case had features of acute leukemia with trilineage myelodysplasia and one had a more chronic evolution with splenomegaly initially resembling a myeloproliferative syndrome. In all cases, the definitive diagnosis was made on bone marrow histology as cytology was poor and the blast cells were positive for factor VIII with the immunoperoxidase technique. The importance of bone marrow histology is emphasized.

Microalbuminuria in sickle cell disease.

The kidney is involved in virtually all individuals who inherit the sickle cell form of hemoglobin. Though asymptomatic and relatively common, proteinuria in patients with sickle cell anemia (SS) over 40 years old is associated with reduced creatinine clearance. The subclinical increase in urinary albumin is termed microalbuminuria and is a marker of preclinical glomerular damage. The aim of the present study was to determine the presence of microalbuminuria measured by radioimmunoassay in patients with sickle cell disease. The study included 41 patients with SS, 11 patients with hemoglobin SC disease, 4 subjects with S beta-thalassemia and 10 normal controls. All subjects were teenagers or adults. Sixteen SS patients (40%) and 1 SC (9%) and 1 S beta (25%) patient presented mean urinary albumin excretion (UAE) above normal values (30 mg/l. No correlation was observed between UAE and age, creatinine clearance, hemoglobin level or %HbF. These parameters, as well as the presence of leg ulcers, were not significantly different between SS patients with and without UAE above 30 mg/dl. The high prevalence of microalbuminuria in patients with sickle cell anemia indicates that glomerular damage is common. The connection between microalbuminuria and clinical nephropathy has been demonstrated in diabetes and may indicate a sign of early disease rather than a marker for susceptibility. Thus, microalbuminuria may be an early indicator of glomerular damage for patients with sickle cell disease.

Microalbuminuria in sickle cell disease

The kidney is involved in virtually all individuals who inherit the suckle cell form of hemoglobin. though asymptomatic and relatively common, proteinuria in patients with sickle cell anemia (SS) over 40 years old is associated with reduced creatinine clearance. The subclinical incrase in urinary albumin is termed microalbuminuria and is a marker of preclinical glomerular damage. The aim of the present study was to determine the presence of microalbuminuria measured by radioimmunoassay in patients with sickle cell disease. The study inclused 41 patients with SS, 11 patients with hemoglobin SC disease, 4 subjects with Sß-thalassemia and 10 normal controls. All subjects were teenagers or adults. Sixteen SS patients (40%) and 1 SC (9%) and 1 Sß (25%) patient presented mean urinary albumin excretion (UAE) above normal values (30 mg/l). No correlation was observed between UAE and age, creatinine clearance, hemoglobin level or %HbF. These parameters, as well as the presence of ulcers, were not significantly differente between SS patients with and without UAE above 30 mg/dl. The high prevalence of microalbuminuria in patient with sickle cell anemia indicates that glomerular damage is common. The connection between microalbuminuria and clinical neplhropathy has been demonstrated in diabetes and may indicate a sign of early disease rather than a marker for susceptibility. Thus, microalbuminuria may be an early indicator of flomerular damage for patients with sickle cell disease