Robert G. Schacht

The Long-Term Course of Poststreptococcal Glomerulonephritis

Abstract Clinical, pathologic, and functional observations have been made in 126 patients (89 adults and 37 children) with poststreptococcal glomerulonephritis, 60 of whom were followed for 2 to 15...

Renal failure in minimal change nephrotic syndrome

Renal insufficiency, with serum creatinines ranging from 2.3 to 13.4 mg/dl, was observed in 15 patients with the minimal change nephrotic syndrome. Recovery of renal function occurred in association with diuretic therapy in 13, eight of whom subsequently underwent steroid-induced remission of the nephrotic syndrome. Two patients failed to undergo diuresis or to have a remission of the nephrotic syndrome and died with persistent renal failure. Glomerular filtration rate (Cinulin) was reduced out of proportion to renal plasma flow (CPAH) as evidenced by remarkably low filtration fractions ranging from 0.03 to 0.095. The invariable association between diuresis and recovery of renal function, the recurrence of renal failure when fluid reaccumulated and the finding of markedly depressed filtration fractions lead us to postulate that renal failure in minimal change nephrotic syndrome may be due to a reversible alteration in glomerular hemodynamics which is related to fluid retention and associated intrarenal edema.

Nephrotoxicity of nitrosoureas

Irreversible and progressive renal parenchymal damage and functional impairment occurred in the majority of patients receiving at least six courses (200 mg/m2 of BCNU and/or methyl CCNU at eight‐week intervals) of nitrosoureas for therapy of malignant brain tumors. Seventeen of 18 patients who received at least six courses and all nine patients who received more than ten courses developed impaired renal function as judged by elevation of blood urea nitrogen and/or serum creatinine or decrease in filtration rate as determined by inulin clearance. Four patients have developed uremia. Renal tissue obtained from seven patients demonstrated tubular atrophy, interstitial fibrosis and glomerular sclerosis. This remarkably high incidence of renal damage occurred without a phase of acute renal failure and in the absence of significant urinary abnormalities, while producing an insidiously progressive interstitial renal lesion.

Progression to Uremia after Remission of Acute Poststreptococcal Glomerulonephritis

Whether or not poststreptococcal glomerulonephritis advances over the long term to uremia remains an unsettled issue. We documented ultimate progression to renal failure in six patients who were observed at onset with the typical clinical and morphologic features of acute nephritis after proved beta-hemolytic streptococcal infection. Within one year, renal function returned essentially to normal in all, as the proliferative changes in glomeruli subsided, Subsequently, increasing renal failure developed in these patients over periods ranging from two to 12 years, during which all were hypertensive. As the renal disease progressed, glomeruli showed increasing sclerosis in the absence of proliferation, and fibrohyaline thickening of renal arterioles appeared.

Exaggerated Natriuresis in the Course of Poststreptococcal Glomerulonephritis

The response to rapid infusion of 2.5% saline solution was examined in 21 patients 1–15 years after an episode of acute poststreptococcal glomerulonephritis. 12 were normotensive and 9 hypertensive; 11 had minimal proteinuria; renal biopsies showed varying degrees of glomerular sclerosis in 9 and were normal in the remainder; filtration rates ranged from 151 to 26 ml/min. Exaggerated natriuresis during the saline load occurred in 19 of the 21. The occurrence or magnitude of natriuresis was not dependent on the level of filtration rate or the presence of hypertension. The renal mechanism, which could not be attributed to increase in filtration rate, decrease in peritubular oncotic pressure, or increase in intrarenal pressure, is unexplained. It is suggested that this remarkably altered reactivity to saline loading reflects the presence of diseased nephrons and constitutes further evidence that irreversible renal damage occurs commonly following acute poststreptococcal glomerulonephritis.

Lymphocyte subpopulations in minimal change nephrotic syndrome

Abstract Minimal change nephrotic syndrome (MCNS) was characterized by a relative lymphocytopenia, a normal T-cell count, and a discordance between B cells enumerated by the presence of surface immunoglobulins (Bsig) and by C3B receptors (Beac). By either technique of B-cell count, the total number of T and B lymphocytes in MCNS exceeded that of controls, suggesting the presence of “double-marked” cells or decreased null cell population. This alteration in surface receptor characteristics of lymphocytes which was observed both in relapse and in remission, documents an abnormality, in MCNS, possibly of cell-mediated immunity, which may be relevant to its pathogenesis.

Irreversible disease following acute poststreptococcal glomerulonephritis in children

Abstract We report on the clinical, pathologic and functional features of sporadic poststreptococcal glomerulonephritis in 83 children (ages 2–16 yr) who were hospitalized at onset and followed subsequently for periods extending up to 17 yr. Beyond 2 yr from onset, one or more features of irreversible renal disease, i.e. hypertension, proteinuria, decreased renal function or sclerosis of glomeruli, were demonstrable in 40% of these children.

A case of Ménétrier disease in a child.

Ménétrier disease is a protein-losing gastroenteropathy often misdiagnosed in the pediatric population. The disease is characterized by hypoalbuminemia secondary to protein loss through the gastrointestinal mucosa and resultant peripheral edema. It is important for emergency department practitioners to consider this diagnosis in the differential diagnosis for edema and low albumin levels in pediatric patients. We present a case report of Ménétrier disease in an edematous child and a brief review.

Fetal and Postnatal Magnetic Resonance Imaging of Unilateral Cystic Renal Dysplasia in a Neonate with Tuberous Sclerosis.

Tuberous sclerosis (TS) is an autosomal dominant condition associated with mutations in the TSC1 and/or TSC2 genes. Clinical manifestations are multisystemic, and they often include lesions in the brain, skin, heart, kidneys, and bones. TSC2 gene mutations can be seen concomitantly with autosomal dominant polycystic kidney disease gene mutations. We present a case of a fetus with prenatal diagnosis of TS that had unique asymmetrical distribution of renal cystic disease. We describe the extensive work up with both fetal and neonatal magnetic resonance imaging with correlating images of the unilateral polycystic renal disease in addition to typical TS brain findings.

Acute Poststreptococcal Glomerulonephritis: A Manifestation of Immune Reconstitution Inflammatory Syndrome

Immune reconstitution inflammatory syndrome (IRIS) is a well-described complication of initiation of highly active antiretroviral therapy in HIV-infected patients. As the immune system recovers, an inappropriate inflammatory response often occurs that causes significant disease. It is most commonly seen in patients naïve to therapy with CD4+ T-lymphocyte counts <100 cells/cmm and usually presents as a flare of mycobacterial, cytomegalovirus, or herpes zoster infections. Less commonly, this syndrome occurs in response to noninfectious triggers and results in autoimmune or malignant disease. Here we present the first case of acute poststreptococcal glomerulonephritis associated with varicella zoster virus and IRIS in an adolescent with perinatally acquired HIV and hepatitis C virus infections. Our patient was not naïve to therapy but was starting a new regimen of therapy because of virologic failure and had a relatively high CD4+ T-lymphocyte count. This case report indicates that IRIS remains a concern after initiation of a new highly active antiretroviral therapy regimen in HIV-infected patients with high viral loads, even in the presence of CD4+ T-lymphocyte counts >100 cells/cmm. It may present as infectious, malignant, or autoimmune conditions including poststreptococcal glomerulonephritis.