PubMed Health⌕ Search

Biomedical subjects

B S Kaplan

Publications and source records attributed to B S Kaplan.

At least 37 records · Page 2Linked to original sources
PubMed1999

Nephrotic syndrome at 5 months: no definitive treatment or complications for 12 years.

We describe a patient who developed nephrotic syndrome at 5 months, with extensive glomerular and tubular damage on biopsy. The patient was treated with diuretics and was asymptomatic for a decade despite unremitting proteinuria. A repeat biopsy at 13 years of age showed remarkable healing with histopathological features consistent with "minimal change" nephrotic syndrome. This patient illustrates a favorable clinical outcome, without specific treatment, of nephrotic syndrome of long duration.

Biopsy↗
PubMed1998

Further evidence that the Hajdu-Cheney syndrome and the "serpentine fibula-polycystic kidney syndrome" are a single entity.

The Hajdu-Cheney syndrome (HCS) is a rare autosomal dominant disorder. It comprises a coarse face, short neck, hirsutism, joint laxity, and normal intelligence. Bone dysplasias, include acro-osteolysis, bathrocephaly, and vertebral anomalies. In 1988, Exner [1988: Eur J Pediatr 147:544-546] coined the term "serpentine fibula-polycystic kidney syndrome" (SFPKS) when he reported on a girl with short stature, unusual facial appearance, polycystic kidneys, and elongated curved fibulae. He postulated that it was a new entity different from the Melnick-Needles syndrome. Since his report, five similar cases have been published. Similarities between both HCS and SFPKS were noticed first by us and then by other authors. In this report we show that many clinical and radiological characteristics are shared by the HCS and the SFPKS and hypothesize that they represent a single entity with a variable degree of expression.

Abnormalities, Multiple↗
PubMed1998

1H MR spectroscopy of the basal ganglia in childhood: a semiquantitative analysis.

Proton MR spectra of the basal ganglia were obtained from 28 patients, 24 male and 14 female, median age 16.3 months (5 weeks to 31 years). They included 17 patients with normal MRI of the basal ganglia without metabolic disturbance (control group) and 11 patients with various metabolic diseases: one case each of high serum sodium and high serum osmolarity, cobalamin C deficiency, Leigh disease, Galloway-Mowat syndrome, Pelizaeus-Merzbacher disease, hemolytic-uremic syndrome and Wilson disease and two cases of Alagille syndrome and methylmalonic acidemia with abnormal MRI of the basal ganglia or blood or urine analysis (abnormal group). The MR spectrum was measured by using STEAM. The MR-visible water content of the region of interest was obtained. Levels of myoinositol, choline, creatine and N-acetylaspartate were measured using a semiquantitative approach, with absolute reference calibration. In the control group, there was a gradual drop of water content over the first year of life; N-acetylaspartate, creatine and myoinositol levels showed no significant change with age, in contrast to the occipital, parietal and cerebellar regions. Choline showed a gradual decrease for the first 2 years of life and then remained fairly constant. In the abnormal group the water content was not significantly different. N-Acetylaspartate was decreased in patients with high serum sodium and high serum osmolarity, cobalamin C deficiency, Leigh disease and one case of methylmalonic acidemia. Decreased creatine was also found in Leigh disease, and decreased choline in Galloway-Mowat syndrome and Wilson disease. Myoinositol was elevated in the patient with abnormally high serum sodium, and decreased in the hemolytic-uremic syndrome.

Adolescent↗
PubMed1998

Acute nonoliguric renal failure in children associated with nonsteroidal antiinflammatory agents.

OBJECTIVE: Acute renal failure is commonly seen in adults after treatment with nonsteroidal antiinflammatory agents. This complication has rarely been reported in children. DESIGN: Four consecutive patients admitted with acute nonoliguric renal failure associated with ingestion of nonsteroidal antiinflammatory agents. SETTING: The emergency department and ward units at The Children's Hospital of Philadelphia. PATIENTS: Four patients, ages 3.5 to 19 years. INTERVENTIONS: Appropriate evaluations including measurement of serum creatinine concentrations in the emergency department and careful histories of drug ingestion. MAIN OUTCOME MEASURE: Rapid return to normal serum creatinine concentrations. RESULTS: Four patients were admitted to The Children's Hospital of Philadelphia between May 1996 and June 1997 with a diagnosis of acute, nonoliguric renal failure following ingestion of nonsteroidal antiinflammatory agents. None of the patients had features of hemolytic uremic syndrome, systemic lupus, dehydration, obstructive uropathy or sickle cell disease. Each patient had ingested nonsteroidal antiinflammatory agents before the onset of acute renal failure. None had oliguria or anuria, dehydration, abnormal serum electrolyte concentrations, or evidence of glomerulonephritis. One patient also had a urinary tract infection that was treated with antibiotics. Another patient was treated with methylprednisolone and prednisone. None was biopsied. Three recovered completely. CONCLUSION: With the increasing use of over-the-counter nonsteroidal antiinflammatory agents, this association may become more prevalent.

Acute Kidney Injury↗
PubMed1998

Limited evaluation of microscopic hematuria in pediatrics.

OBJECTIVE: The purpose was to determine the value of the standard laboratory and radiologic evaluation of microscopic hematuria in children, and to determine the prevalence of idiopathic hypercalciuria in those children referred for evaluation of unexplained microscopic hematuria. METHODS: This was a retrospective study of 325 children referred from 1985 to 1994 for the evaluation of asymptomatic microscopic hematuria. The diagnostic studies reviewed included serum creatinine, blood urea nitrogen, serum electrolyte studies, serum complement concentration, antinuclear antibody, urinalysis, urine calcium to creatinine ratios, urinary protein to creatinine ratio and/or 24-hour urinary protein excretion, renal ultrasounds, intravenous pyelograms, voiding cystourethrograms, and historical information. RESULTS: All creatinine and electrolyte values were normal for age, and none of the biochemical tests obtained in the children with hypercalciuria was abnormal. Of the 325 patients with idiopathic microscopic hematuria, only 18 had abnormal renal ultrasound examinations and 9 voiding cystourethrograms showed low-grade reflux. Hypercalciuria was found in 29 patients. The family history was positive for urolithiasis in 16% of patients without hypercalciuria compared with 14% of patients with hypercalciuria. A positive family history of hematuria was reported in 25% of patients; 62 patients did not have hypercalciuria and 4 of the patients had hypercalciuria. Microscopic hematuria in children is a benign finding in the vast majority of children. CONCLUSIONS: Our data demonstrate that a renal ultrasound, voiding cystourethrogram, cystoscopy, and renal biopsy are not indicated in the work-up of microscopic hematuria, and microhematuria in the otherwise healthy child is a minimal health threat, rarely indicative of serious illness.

Adolescent↗
PubMed1997

Cystic kidneys associated with connective tissue disorders.

Advances in molecular genetics have resulted in the identification of several forms of autosomal dominant polycystic kidney disease (PKD). Cystic kidneys have also been observed in tuberous sclerosis, von Hippel-Lindau syndrome, oro-facial-digital type I syndrome, Hajdu-Cheney syndrome, Ehlers-Danlos syndrome, and an "overlap" connective tissue disorder, and cannot be distinguished by ultrasonography from PKD. We have studied four children with similar cystic kidneys. None had a family history of PKD. One child has osteogenesis imperfecta type IV, two appeared to have a mild Ehlers-Danlos syndrome, and the fourth has inguinal hernias and undescended testes. We speculate that polycystic kidneys may occur in connective tissue dysplasias. We also realize that these may be chance associations with spontaneous mutations for PKD.

Abnormalities, Multiple↗
PubMed1997

Renal transplantation in adults with autosomal recessive inheritance of hemolytic uremic syndrome.

When hemolytic uremic syndrome (HUS) is occasionally inherited in an autosomal recessive mode, this occurs mainly in infants and children. We describe four families in which two adult siblings were affected with HUS in each kindred. HUS first occurred between the ages of 19 to 36 years, and the intervals between the onset of HUS in each sibling pair ranged from 6 months to 6 years. None of the patients had a typical prodrome of bloody diarrhea, and one had a recurrence of HUS before transplantation. All eight patients developed renal failure requiring dialysis and transplantation, and seven patients received kidney transplants. Donor kidneys were from parents, siblings, and cadavers. The initial renal transplants were performed from 6 months to 6 years after the onset of the syndrome. HUS recurred in six of the seven patients 2 weeks to 6.5 years after transplantation regardless of the interval between the onset of HUS and transplantation, the origin of the allograft, or the use of cyclosporin A. The only marker for autosomal recessive HUS is the occurrence of the syndrome in a second sibling several months to many years after its occurrence in the proband. In patients with the autosomal recessive form of HUS, the risk for a recurrence in an allograft is high regardless of the source of the kidney.

Adult↗
PubMed1996

Management of patients with hemolytic uremic syndrome demonstrating severe azotemia but not anuria.

There are no specific indications for dialysis in a patient with typical hemolytic uremic syndrome (D + HUS) who does not have anuria, hyperkalemia, volume overload, or severe acidemia. We managed five patients with D + HUS, aged 1.5-14 years, without dialysis despite marked azotemia, because they were not anuric and because they had none of the acid-base, fluid, or electrolyte perturbations that may have been indications for dialysis. Each had markedly elevated blood urea nitrogen (range 137-234 mg/dl) and serum creatinine concentrations (range 5.4-15.4 mg/dl). None was anuric and one was oliguric for 4 days. There were no complications and each recovered. We have reviewed the published literature on the use of dialysis in patients with D + HUS and have not found any guidelines that relate to the management of similar cases. It is our view that management of D + HUS patients without dialysis is appropriate when the patient is passing urine and the acid-base, serum electrolyte concentrations and fluid balances can be managed without dialysis.

Adolescent↗
PubMed1996

Discordant renal histopathologic findings and complement profiles in membranoproliferative glomerulonephritis type III.

Patients with membranoproliferative glomerulonephritis (MPGN) type III and a low serum C3 concentration tend to have evidence for a nephritic factor of the terminal complement pathway (Nft). Complement profiles were studied in three patients with MPGN type III and low serum C3 concentrations. Serum C3 concentrations were 52, 21, and 14 mg/dL (normal range, 83 to 177 mg/dL). Serum Clq, C2, C4, properdin, and C5 concentrations were normal in all patients, whereas two had a slight decrease of C7 or C8. This pattern of complement activation resembles that seen with MPGN type II in which a nephritic factor activates the amplification loop (NFa). We conclude that in patients with MPGN type III the previously reported profile/presence of Nft is not always found, at least in the chronic stage of the disease, despite a low C3 value.

Biopsy↗
PubMed1995

Infundibulopelvic stenosis, multicystic kidney, and calyectasis in a kindred: clinical observations and genetic analysis.

Congenital obstructive anomalies of the urinary tract usually occur sporadically. We describe inheritance in a three-generation kindred of a spectrum of kidney anomalies consistent with an autosomal-dominant mode of transmission, with incomplete penetrance, calyectasis (maternal grandmother), infundibulopelvic stenosis (uncle), and multicystic kidney (male proband, age 4 years). The proband's mother, father and half sister had normal renal imaging studies. Inheritance of informative polymorphic markers (3'-HVR, GGG1, GGG9, SM-7, KG8, and CW3) mapping close to the adult polycystic kidney disease type 1 (PKD-1) and tuberous sclerosis (TSC-2) loci on chromosome 16p was evaluated by Southern blot studies and by PCR-based, fluorescent genotyping for linkage to phenotype. The 3 affected individuals, as well as the unaffected mother (obligate carrier) and unaffected half-sister, inherit a common chromosome haplotype linked to the PKD1 locus. Our findings support the hypothesis that these anomalies may be part of a spectrum of obstructive renal dysplasia which are inherited as a simple Mendelian trait exhibiting an autosomal-dominant mode of transmission with variable expression and incomplete penetrance.

Adult↗
PubMed1995

Renal dysplasia associated with in utero exposure to gentamicin and corticosteroids.

We report on a patient with renal cystic dysplasia, whose mother was given gentamicin and corticosteroids early in pregnancy. We speculate that these drugs may be implicated in abnormal nephrogenesis in humans. This speculation is based on gentamicin-induced small kidneys (oligonephronia) in the rat. Renal cystic disease has been demonstrated following glucocorticoid administration early in gestation. We realize that there is no proof for a casual relationship between gentamicin and/or glucocorticoids in the pathogenesis of this patient's renal disease. However, it is possible that renal cystic dysplasia in humans is not solely the result of a genetic defect.

Adrenal Cortex Hormones↗
PubMed1995

Cystic kidney disease in Hajdu-Cheney syndrome.

We report on 2 unrelated patients with Hajdu-Cheney acroosteolysis syndrome, who had cystic kidneys with ultrasonographic changes similar to those of autosomal dominant polycystic kidney disease. Neither had a family history of Hajdu-Cheney syndrome or polycystic kidneys, nor manifestations of any other syndrome. On the basis of the findings in these 2 patients and a review of published cases, we suggest that cystic kidneys are an important component of Hajdu-Cheney syndrome.

Fatal Outcome↗