End-stage renal disease in two pediatric patients with Fechtner syndrome.

Fechtner syndrome, a disease in the spectrum of the hereditary nephridites, is a macrothrombocytopenia associated with sensorineural hearing loss, cataracts, nephritis, and characteristic leukocyte inclusions. Renal biopsy findings are consistent with those of Alport syndrome, and the associated renal disease is said to be unusual before mid to late adulthood. Here, we review the available literature on this disease and report two African-American pediatric patients with Fechtner syndrome who rapidly progressed to end-stage renal disease during adolescence. We conclude that chronic renal failure can occur at a young age in patients with Fechtner syndrome, with a possible relation to race/ethnicity. Fechtner syndrome, or other variants of Alport syndrome, need to be considered in patients presenting with proteinuria and thrombocytopenia.

Increased incidence of insulin-dependent diabetes mellitus in pediatric renal transplant patients receiving tacrolimus (FK506)

BACKGROUND: Use of tacrolimus (FK506), a potent immunosuppressive agent, has been reported to have a 10-20% incidence of insulin-dependent diabetes mellitus (IDDM) in adults, but the incidence of IDDM in pediatric renal transplant recipients treated with this agent is unknown. In this article, we report our single-center experience with FK506-induced IDDM in children. METHODS: Five consecutive living related donor pediatric renal transplants were reviewed retrospectively. RESULTS: All five patients developed IDDM lasting longer than 6 months. Mean follow-up time was 18.6 months. CONCLUSIONS: Pediatric patients may be at high risk for developing FK506-induced IDDM.

A typical hemolytic uremic syndrome in human immunodeficiency virus-1-infected children.

We describe the clinical and pathological findings of the hemolytic uremic syndrome (HUS) in two children with human immunodeficiency virus (HIV) infection. Both patients presented with microangiopathic hemolytic anemia, thrombocytopenia, and subsequently developed renal failure. The diagnosis of HUS was confirmed by renal histopathology in both patients. None of these children presented with bloody diarrhea, evidence of circulating antibody response to Escherichia coli O157 lipopolysaccharide, or other known risk factors for HUS, except for the presence of HIV infection. Each patient was treated with intravenous plasma infusion and renal replacement therapy. Their clinical course was characterized by non-oliguria and lack of significant hypertension throughout the acute phase of the disease. Despite these favorable clinical parameters, both patients developed end-stage renal failure. The etiology of this atypical HUS characterized by poor renal survival remains unknown and the role of HIV infection in its pathogenesis, although possible, is unclear.

PREVALENCE OF ANTIBODIES TO HEPATITIS C VIRUS IN HOSPITALIZED POPULATION SUBSETS.

Prevalence of antibodies to hepatitis C virus (anti-HCV antibody) was ascertained in different subsets of hospitalized patients. Anti-HCV antibody testing was done using a third generation test The anti-HCV positivity in the sera of 308 tuberculosis patients, who had been given streptomycin, was 12.3 per cent It was 7.1 percent among 84 cardiac patients undergoing invasive cardiac procedures, 15.6 per cent in 77 patients on haemodialysis and 41.2 per cent patients with surgical jaundice. It is surmised that hospitalization, particularly parenteral therapy and invasive procedures during hospitalization, puts patients at higher risk of contracting HCV infecticn.

Gentamicin-induced Bartter-like syndrome.

Gentamicin is well known to be associated with nephrotoxicity, including acute renal failure and renal tubular dysfunction. A Bartter-like syndrome has also been described as a toxic manifestation of gentamicin therapy in adults, but this nephrotoxic syndrome has not been well characterized in children. In this report we describe the clinical course of four patients with gentamicin-associated Bartter-like syndrome. These patients ranged in age from 4 months to 17 years; they all demonstrated evidence of renal tubulopathy, primarily affecting the distal nephron. Hypocalcemia, hypomagnesemia, alkalosis, and hypokalemia were the main manifestations in these patients. After discontinuation of gentamicin, recovery of the renal tubular functions and resolution of the electrolyte abnormalities were complete in all patients.

The implementation of a continuous quality improvement program in a pediatric peritoneal dialysis unit.

Continuous quality improvement (CQI) principles and practices were utilized to evaluate the outpatient peritoneal dialysis follow-up process and the routine monthly laboratory testing of chronic dialysis patients. CQI enabled us to reduce total clinic visit time from a mean of 161 min to 90.8 min. Waiting was decreased from a mean of 51.6 min to 15.8 min. The number of routine monthly laboratory tests performed on patients undergoing chronic dialysis was also reduced, resulting in decreased charges of at least $429.50 per patient per month.

Cavitating pulmonary infarction in nephrotic syndrome.

A 6-year-old boy was admitted with anasarca due to relapse of nephrotic syndrome, non-productive cough, and dyspnea on exertion. On the 8th hospital day he manifested severe right shoulder and chest pain. A nodular opacity in the right lung that transformed into a cavitating lesion was documented by computerized axial tomography. Surgical biopsy of the affected area of the lung documented infarction due to pulmonary embolism. This case highlights the need to consider pulmonary embolism in the evaluation of nephrotic children with a cavitating pulmonary lesion.

Kinetics and mechanics of stem gravitropism in Coprinus cinereus.

Using video recordings we have completed the first kinetic analysis of mushroom stem gravitropism. The stem became gravireceptive after completion of meiosis, beginning to bend within 30 minutes of being placed horizontal. Stem bending first occurred in the apical 15% of its length, then the position of the bend moved rapidly towards the base, traversing 40% of stem length in 2.5 h. Meanwhile, the stem elongated by 25%, mostly in its upper half but also in basal regions. If the apex was pinned horizontally the stem base was elevated but overshot the vertical, often curling through more than 300 degrees. When the base was pinned to the horizontal (considered analogous to the normal situation), 90% of the initial bend was compensated as the stem brought its apex accurately upright, rarely overshooting the vertical. The apex had to be free to move for this curvature compensation to occur. Stems transferred to a clinostat after some minutes gravistimulation showed curvature which increased with the length of initial gravistimulation, indicating that continued exposure to the unilateral gravity vector was necessary for continued bending. Such gravistimulated stems which bent on the clinostat subsequently relaxed back towards their original orientation. Reaction kinetics were unaffected by submergence in water, suggesting that mechanical events do not contribute, but submerged stems bent first at the base rather than apex. In air, the gravitropic bend appeared first near the apex and then moved towards the base, suggesting basipetal movement of a signal. In water, the pattern of initial bending was changed (from apex to base) without effect on kinetics. Taken together these results suggest that bending is induced by a diffusing chemical growth factor (whose extracellular propagation is enhanced under water) which emanates from the apical zone of the stem. The apex is also responsible for regulating compensation of the bend so as to bring the tip to the vertical. The nature of this latter stimulus is unknown but it is polarized (the apex must be free to move for the compensation to occur) and it may not require reference to the unilateral gravity vector.

Absent intestinal response to calciferols in hereditary resistance to 1,25-dihydroxyvitamin D: documentation and effective therapy with high dose intravenous calcium infusions.

We describe a patient with an absent intestinal response to 1,25-dihydroxyvitamin D [1,25-(OH)2D] and the beneficial effects of treatment with high dose iv calcium infusion. The patient presented with severe rickets despite therapy with extraordinarily high doses of 1 alpha-hydroxyvitamin D3 or 1,25-(OH)2D3. Unidirectional intestinal fractional calcium absorption when he was not treated with any calciferol was 14% (normal, 20-70%), as measured with stable calcium isotopes; no increase in calcium absorption occurred when serum 1,25-(OH)2D levels were more than 50-fold elevated. Cultured skin fibroblasts contained no detectable 25-hydroxyvitamin D3-24-hydroxylase activity in response to 1,25-(OH)2D3 (10(-9)-10(-6) mol/L). High dose iv calcium infusions and oral phosphorus supplementation for 135 days improved or normalized biochemical parameters and resulted in radiographic healing of the rachitic lesions. We conclude that 1) this patient had no response to 1,25-(OH)2D3 in vivo and in vitro; 2) long term parenteral calcium infusions were effective therapy in managing the patient's severe resistance to 1,25-(OH)2D; and 3) stable calcium isotopes are useful for measuring low levels of fractional calcium absorption.

Anion gap in nephrotic syndrome.

Low anion gap occurs in a variety of disorders including hypoproteinemia. We evaluated anion gap in 28 children - 18 boys and 10 girls with minimal change nephrotic syndrome during 55 separate episodes of exacerbations. To assess the contribution of serum total proteins, albumin, cholesterol and immunoglobulin levels to low anion gap, a coefficient of correlation was calculated. Anion gap was significantly low (p less than 0.01) during exacerbations in nephrotic. The low anion gap correlated with low serum total proteins (less than 4 g/dl) and serum albumin levels (less than 1.5 g/dl) but not to serum cholesterol or immunoglobulin levels. Low total serum protein at patients blood pH, contributed fewer negative charges to anion gap thereby lowering the anion gap. The major cause of low anion gap in nephrotic syndrome is hypoproteinemia especially hypoalbuminemia.

Stenosing ureteritis in Henoch-Schönlein purpura.

We report on a boy with Henoch-Schönlein purpura in whom flank pain and gross hematuria developed during the early phase of the disease. Urologic investigations revealed hydronephrosis and ureteral stenosis. Pyeloplasty was done to relieve ureteropelvic junction obstruction. Severe hemorrhagic ureteritis and vasculitis were noted on the ureteral biopsy. Recovery was slow and residual hydronephrosis persisted. Although abdominal pain usually accompanies Henoch-Schönlein purpura, colicky flank pain associated with hematuria should alert the physician to the presence of ureteritis. Recognition and early surgical treatment of this urologic complication of Henoch-Schönlein purpura may prevent a potentially serious outcome.