Manju Chandra

Occipital-parietal encephalopathy: a new name for an old syndrome.

A boy presented with hypertension, seizures, lethargy, headache, and occipital blindness. He improved with antihypertensive therapy. Other reported children with a similar distinctive clinical condition are compared with adults with a syndrome termed reversible posterior leukoencephalopathy. Because both gray and white matter are involved, we suggest that the name be changed to occipital-parietal encephalopathy syndrome.

Transient urodynamic dysfunction of infancy: relationship to urinary tract infections and vesicoureteral reflux.

PURPOSE Urinary tract infections and vesicoureteral reflux are more common in male than female infants. Since these problems can result from voiding dysfunction, we obtained a detailed history of voiding patterns and urodynamic testing in infants with urinary tract infections in the first year of life. MATERIALS AND METHODS We evaluated 39 male and 22 female infants, including 40 with primary vesicoureteral reflux and 21 with no reflux or obstruction. RESULTS Voiding abnormalities were noted in 97% of the male and 77% of the female infants, including high voiding detrusor pressure of greater than 40 cm. water in 92% of the male and 66% of the female infants, residual urine greater than 2 ml./kg. in 13% of the male and 23% of the female infants, and detrusor hyperreflexia with filling pressure greater than 40 cm. water in a third of the male infants. Voiding detrusor pressure was significantly higher in male than female infants and in male infants with grade IV to V reflux than those with lower grades of reflux or no reflux. Followup urodynamic testing in 15 infants with high voiding detrusor pressure revealed resolution of detrusor hyperreflexia and improvement in post-void residual in all and decreased voiding detrusor pressure in 14. CONCLUSIONS We coined the term transient urodynamic dysfunction of infancy to describe this constellation of abnormalities, which predisposes infants to urinary tract infections and vesicoureteral reflux but improves spontaneously. The higher incidence of urinary tract infections and reflux in male infants may be related to higher intravesical pressures.

Hypertension secondary to renin-secreting juxtaglomerular cell tumor: case report and review of 38 cases.

A 15-year-old girl with severe high renin hypertension caused by a juxtaglomerular cell tumor (JCT) was successfully treated with the calcium channel blocker nifedipine until surgical removal effected a permanent cure. This case was incorporated into a review of the 37 cases previously published. Comparison of the children and adolescents with the adult population showed that the features of JCT were similar in the two groups except for the average duration of symptoms prior to diagnosis (pediatric group 2.6 years vs. 6.0 years for the adult group). Analysis of all 38 cases demonstrated the following:1.Teenagers constituted the largest single population with JCT (39%) and approximately two-thirds of the entire population were female.2.Many patients failed to show persistent hypokalemia despite high plasma renin activity and secondary hyperaldosteronism.3.Renal angiography was initially negative in more than half the cases.4.Renal vein renin failed to show lateralization to the affected kidney in 52% of the cases.5.Computerized tomography demonstrated a renal mass in all of the cases in which it was performed, even when other imaging studies were negative.6.Calcium channel blockers may evolve as the preferred treatment for the high renin hypertension of JCT.

Giggle Incontinence in Children: A Manifestation of Detrusor Instability

PURPOSE To our knowledge the prevalence and cause of giggle incontinence in children is not known. We hypothesized that laughter may induce unstable detrusor contractions in children susceptible to detrusor instability. We evaluated the prevalence of diurnal voiding symptoms of urinary urgency, urge incontinence, pelvic withholding maneuvers and hesitancy in patients with giggle incontinence, the prevalence of giggle incontinence in patients with diurnal voiding symptoms, the prevalence of the 2 conditions in first degree relatives of patients with giggle incontinence, the influence of treatment for detrusor instability on the frequency of giggle incontinence and the prevalence of diurnal voiding symptoms in control children with giggle incontinence. MATERIALS AND METHODS Of 1,421 children 5 to 15 years old referred to the pediatric nephrology department for various problems 109 were diagnosed with giggle incontinence and 460 had diurnal voiding symptoms. A total of 627 children visiting the pediatrician office whose parents completed a survey questionnaire served as controls. RESULTS Diurnal voiding symptoms were noted in 95% of the patients with giggle incontinence, while giggle incontinence was noted in 23% of those with diurnal voiding symptoms. Of the patients with giggle incontinence a positive family history for that entity and diurnal voiding symptoms was noted in 13% and 28%, respectively. Giggle incontinence improved in all patients after treatment for detrusor instability and it resolved completely in 89%. Giggle incontinence recurred with a relapse of diurnal voiding symptoms in 28 cases and improved with improved diurnal voiding symptoms during modification of therapy. Diurnal voiding symptoms were present in 43% of the 157 controls with giggle incontinence. CONCLUSIONS Giggle incontinence results from detrusor instability induced by laughter and it improves with effective treatment of detrusor instability.

Urodynamic dysfunction in infants with vesicoureteral reflux

PATIENTS AND METHODS Vesicoureteral reflux (VUR) may result from inadequate length of the intravesical ureteric tunnel and from urodynamic dysfunction. VUR and symptomatic urinary tract infection (UTI) are both more common in male infants and may be related to urodynamic dysfunction and functional intravesical obstruction. We therefore conducted urodynamic studies in 75 infants with primary VUR and retrospectively evaluated their voiding cystourethrogram (VCUG) for evidence of inadequate sphincteric relaxation. RESULTS High voiding detrusor pressure (>70 cm H(2)O), increased postvoid residual (>2 mL/kg body weight), or detrusor-sphincter dyssynergy were observed in 79% of infants with primary VUR without significant differences between male and female infants or in infants with or without UTI. Infants with bilateral grade IV-V VUR showed a higher prevalence of detrusor hyper-reflexia and higher voiding detrusor pressure than infants with lower grades of VUR or unilateral grade IV-V VUR. On VCUG, inadequate external urethral sphincter relaxation during voiding was noted in 10 of 47 male infants. CONCLUSIONS The findings suggest that high intravesical pressures contribute to the development and severity of VUR, and that high voiding detrusor pressure in some infants with primary VUR may be related to inadequate relaxation of the external urethral sphincter.

Idiopathic arterial calcification of infancy: a case with prolonged survival

We describe a patient with idiopathic arterial calcification of infancy and the following unusual features of the disease: (1) prolonged survival until age 11 years, (2) discordance between the extent of vascular calcification and clinical manifestations of arterial luminal occlusion, (3) a large area of myocardial calcification, (4) symptoms of cerebral-vascular insufficiency, and (5) spontaneous resolution of hypertension.

Intraperitoneal production of erythropoietin with continuous ambulatory peritoneal dialysis

Higher hematocrit and serum erythropoietin (EPO) levels have previously been shown in end-stage renal disease patients treated with continuous ambulatory peritoneal dialysis (CAPD) compared with hemodialysis. We investigated whether EPO was produced intraperitoneally in CAPD patients. EPO concentration was 3.5±0.3 mU/ml by radioimmunoassay in 26 samples of peritoneal dialysis effluent obtained from 15 CAPD patients. EPO was not detectable in the fresh unused dialysate. No correlation was observed between EPO levels in the serum and dialysis effluent. Peritoneal macrophages were isolated from the dialysis effluent of 9 CAPD patients after an overnight dwell. The culture supernatant obtained after 24 h of in vitro culture of a million cells yielded EPO of 3.5±0.3 mU/ml. Our study demonstrated that peritoneal macrophages from CAPD patients produce EPO on in vitro stimulation, and EPO is present in the dialysis effluent of CAPD patients.

Splenic Hypofunction in the Nephrotic Syndrome of Childhood

The reticuloendothelial system, including the spleen, subserves important immunologic functions. Loss of splenic function results in an increased incidence of severe bacterial infections and is accompanied by thrombocytosis. Several nephrotic children were noted to have remarkably high platelet counts and predisposition to bacterial infection with encapsulated organisms. We, therefore, investigated the splenic function of nine children with primary nephrotic syndrome and measured the phagocytic function of the spleen by sequestration of Technetium-99-labelled heat-treated autologous RBC, administered intravenously. Four children had decreased splenic function. Repeat studies performed in two of these children after remission of the nephrotic syndrome gave normal results. There were six episodes of bacterial infection (3 peritonitis, 1 septic arthritis, 1 cellulitis, and 1 Escherichia coli urinary tract infection) among the four patients with decreased splenic function. There were no episodes of bacterial infection among the five nephrotic children with normal splenic function. Nephrotic patients with decreased splenic function had significantly increased platelet counts (921,000 +/- 196,000; mean +/- SEM) compared to those with normal function (435,000 +/- 46,000; P less than 0.001). Our findings suggest the possibility that some nephrotic children may have decreased splenic function in association with increased susceptibility to bacterial infections.

Reduction in Liver Iron in Hemodialysis Patients With Transfusional Iron Overload by Deferoxamine Mesylate

Four hemodialysis patients with transfusional iron overload were treated with three times weekly intravenous (IV) deferoxamine mesylate during the dialysis treatment. Using a gamma ray scattering technique, significant reductions in liver iron content were documented, with a mean follow-up of 20 months. Three of the four patients showed significant improvements in liver enzymes. This decrease in liver iron content could not be predicted by clinical parameters or serum ferritin. Therapy proved to be safe and effective, but follow-up requires monitoring of tissue iron by means other than standard laboratory tests.