Focal segmental glomerulosclerosis. A common entity in nephrotic black adults.

We studied 100 renal biopsy specimens from adults with the primary nephrotic syndrome in an inner city hospital serving mostly black patients and found that 47 had focal segmental glomerulosclerosis. Most of the men presented in the third decade of life, a peak distribution not seen in women. Half of the patients were hypertensive at presentation. Two thirds of the patients had not used intravenous drugs. The addicts were younger than nonaddicts (mean +/- SD age, 27 +/- 4 years vs 35 +/- 13 years), had greater proteinuria (10 +/- 5 g/d vs 6.3 +/- 5 g/d), and exhibited more glomerulosclerosis and tubulointerstitial fibrosis on biopsy. Of the 18 patients (8 addicts) remaining under our care, 4 addicts and 4 nonaddicts became uremic within 3 years. We conclude that even in the absence of drug addiction, focal segmental glomerulosclerosis is a common cause of primary glomerular disease in black adults, in whom it may represent a nonspecific glomerular reaction to injury. The prognosis in the nonaddict may not be different from that in the addict, but more patients need to be studied.

Exchange transfusion in the neonate: a controlled study using frozen-stored erythrocytes resuspended in plasma.

During a nine-month period 43 consecutive infants who needed exchange transfusions for the management of their hyperbilirubinemia randomly received whole blood less than 5 days old (control group) or frozen erythrocytes diluted in plasma (experimental groups). There was no different prior to and after exchange transfusions in total protein, albumin, globulin, immunoglobulins, bilirubin, and hematocrit in the control group versus the experimental group. The efficacies of bilirubin removal, as measured by delta bilirubin, were the same in the two groups. Advantages of exchange transfusion with freeze-preserved erythrocytes suspended in type-specific plasma are discussed.

Nonamyloidotic fibrillar glomerulopathy and recurrent gestational anasarca.

Congo-red-negative microfibrils have been described in various glomerular diseases, some of which have no known etiology. We report the unusual case of a young woman who, over a period of 17 years, developed recurrent gestational anasarca but was asymptomatic between pregnancies except for proteinuria. Her blood pressure and renal function have remained normal over the years. A renal biopsy done 5 years after her third pregnancy showed diffuse mesangial expansion and irregular thickening of the glomerular basement membrane, both caused by the deposition of nonamyloidotic microfibrils. We discuss the differential diagnosis of this case and review the pertinent literature.

[Identification of antinuclear, nucleolar and SS-A antibodies, using an immunoassay system]. / Identificación de anticuerpos antinucleares, nucleolares y SS-A, con el uso de un sistema de inmunoensayo.

In this paper we report a solid phase ELISA for screening of antinuclear, nucleolar, and SS-A antibodies. The test system was evaluated with 158 sera that were positive by immunofluorescence (IF), some of which were immunologically characterized as containing antibodies to Sm, RNP, SS-A, SS-B, nucleolar, and DNA, and 247 IF negative that were used as a control group. Ninety eight per cent of serum samples with an antibody of known specificity and/or positive IF, were positive by the ELISA system including antinucleolar samples. Reproducibility of the assay was demonstrated by using five sequentially prepared antigenic extracts. The lineal regression from the last experiment was between 0.75 and 0.91. With this system, positive and negative ANA can be identified as the screening procedure without the need of additional studies. To our knowledge this is the first ELISA system report for the screening of antinucleolar antibodies.

Immunoglobulins in submassive hepatic necrosis.

The presence of submassive hepatic necrosis in acute liver injury of viral or toxic etiology in liver biopsy specimens is of great diagnostic and prognostic significance. Liver biopsy, however, cannot be performed in some of these patients due to an abnormal coagulation profile. Hence, other noninvasive methods for diagnosis of submassive hepatic necrosis would be helpful. We compared the serum immunoglobulin-G levels in 15 patients with typical acute viral hepatitis and 19 patients with severe acute viral hepatitis with submassive hepatic necrosis. The difference of the Ig-G levels in both groups was statistically significant. By using discriminant analysis we found that it was possible to make the correct diagnosis of submassive hepatic necrosis in 89.5% of patients with acute viral hepatitis when only Ig-G levels were utilized.

Prognosis of the nephrotic syndrome in sickle glomerulopathy. A retrospective study.

Of 240 adults with sickle cell anemia seen over 11 years, 12 had the nephrotic syndrome. In 9 (75%) the glomerular lesion, sickle glomerulopathy, consisted of mesangial expansion and basement membrane duplication. Six patients had type IV renal tubular acidosis. Four of the 9 Patients died within 24 months (17 +/- 5; mean +/- SD), while 5 survived 36 months or longer (80 +/- 49); no significant differences were seen between the former and the latter in age, admission serum creatinine and C3 levels, urinary protein excretion, or the frequency of renal tubular acidosis. Chronic azotemia developed in 3 and acute renal shutdown in another 2. Of 22 patients with sickle glomerulopathy (our 9 added to 13 from the literature) 11 died within 2 years. Ten of these (91%) had developed renal failure, compared to only 5 of the 11 (45%) who survived longer than 2 years (p less than 0.05). The 5-year mortality in the general population of sickle cell anemia is 3.75%, and 75% of patients aged 15 years or older survive 18 years or longer. The nephrotic syndrome, most often caused by sickle glomerulopathy, occurs in 4% of patients with sickle cell anemia, leading to renal failure in two-thirds and death in 2 years in half the patients. The development of chronic azotemia correlates strongly with early mortality. The prognosis is much worse than that in the general population of sickle cell anemia.