Bella Eisenstein

Prevalence of Vesicoureteral Reflux in Neonatal Urinary Tract Infection

Vesicoureteral reflux (VUR) after a first episode of urinary tract infection (UTI) is apparently diagnosed much more frequently (25%-40%) in children than in neonates. The aims of the study were to determine the actual rate of VUR in neonates with UTI and to define the clinical clues to its diagnosis. The study sample included term infants with a diagnosis of UTI during their first month of life who were seen in this hospital between January 1997 and May 1999. All infants underwent complete diagnostic work-up (renal ultrasound and voiding cystourethrography [VCUG]). The medical files were reviewed for patient sex, age at UTI diagnosis, laboratory findings (including causative pathogen), and ultrasonographic findings. These parameters were correlated with the finding of VUR on VCUG. Sixty-four neonates (55 males, 9 females) with UTI were included in this study. UTI was 6 times more common in males than females, although the incidence of VUR was equal between the sexes (about 20%). The presence of VUR was associated with a significantly younger age at presentation of UTI (11.4±4 vs 16.9±6.6 days, p<0.01). VUR was diagnosed at a fourfold higher rate in neonates with Klebsiella-induced UTI compared to those with E. Coli-UTI. In 80% of those with significantly abnormal ultrasonographic findings VUR was found on VCUG. Jaundice was noted at UTI diagnosis 3 times more often in infants with VUR, and elevated creatinine level, 2.5 times more often.

Direct Measurement of TP/GFR: A Simple and Reliable Parameter of Renal Phosphate Handling

As the Walton-Bijvoet nomogram for estimating renal phosphate (P) threshold (TmP/GFR) is not applicable to children of all ages, we sought an alternative method for measuring renal handling of P. Recognizing that the nomogram represents an indirect correlation between TmP/GFR and TP/GFR under fasting conditions, we examined this directly in 26 children. An excellent correlation was found, expressed as TmP/GFR = (fasting TP/GFR X 1.1) -0.3 (r = 0.95). The regression line in adults, expressed as TmP/GFR = (fasting TP/GFR X 1.4) -0.9 (calculated from published studies) is markedly different at the higher values typical for children. Since no advantage could be seen in the use of a mathematically derived TmP, we investigated the direct use of measured TP/GFR (tubular P reabsorption per 100 ml glomerular filtrate) as a measure of renal P handling in clinical practice. No differences were found between morning fasting and nonfasting values. Measurements in 151 healthy subjects aged 3 days to 53 years established normal values in relation to age. The use of this parameter in patients is shown to accurately reflect defects and changes in renal P handling. We believe it to be the preferred parameter because it represents a directly measured physiologic function applicable to all age-groups.

Microcephaly and congenital nephrotic syndrome owing to diffuse mesangial sclerosis: an autosomal recessive syndrome.

Three sibs born to consanguineous parents had congenital nephrotic syndrome, microcephaly, and psychomotor retardation. Pathology of the kidneys showed diffuse mesangial sclerosis with deposits of IgG and C3 in the mesangium and glomerular basement membranes. All three children died before the age of 3 years. Of 19 published cases of children with the association of congenital nephrotic syndrome and microcephaly, only four had histological evidence of diffuse mesangial sclerosis, and two of their sibs probably had the same disease. The association of nephrotic syndrome owing to congenital diffuse mesangial sclerosis, microcephaly, and mental retardation appears to be a distinct syndrome with an autosomal recessive mode of inheritance.

Maxillomandibular brown tumor – a rare complication of chronic renal failure

Abstract We report a 17-year-old hemodialysis patient with a rapidly growing maxillary mass diagnosed as a brown tumor. Although successful control of the parathyroid hormone (PTH) levels was achieved by treatment with vitamin D pulse therapy, the lesion progressed, invaded the maxillary sinus, and caused severe eating and speech disabilities. No recurrence was observed following surgical excision. The differential diagnosis and considerations regarding the causes of the disease in a child, therapy options, and review of the literature are presented.

Renal functional reserve after acute poststreptococcal glomerulonephritis

Abstract. We evaluated renal functional reserve (RFR) in 36 patients aged 5 – 21 years, who had recovered from an acute episode of poststreptococcal glomerulonephritis (PSGN) 1 – 16 years previously, without apparent sequelae, as evidenced by normal serum creatinine, blood pressure, and urinary sediment. The control group consisted of 12 children aged 2 – 12 years with recurrent urinary tract infections or nocturnal enuresis, without active infection or anatomical anomalies. The basal creatinine clearance was similar in the PSGN and control groups: 140.0±27.4 ml/min per 1.73 m2 and 142.9±15.5 ml/min per 1.73 m2, respectively. The RFR in the PSGN group was significantly reduced compared with that of the control group: 18.6±12.9 ml/min per 1.73 m2 and 41.1±25.3 ml/min per 1.73 m2, respectively (P <0.02). In 7 PSGN patients (19.4%), no RFR was found. In 69% of patients who had recovered from PSGN more than 10 years before the protein loading tests, a significantly reduced RFR (less than 10% of baseline) was found. The same degree of reduction in RFR was found in only 26% of patients who had suffered from PSGN less than 10 years ago.

Unilateral duplicated system: comparative length and function of the kidneys.

Objective: The objective of this study was to estimate kidney length and function in patients with unilateral duplex kidney. Materials and Methods: Thirteen patients with a unilateral duplicated system were reviewed retrospectively. The length of the kidneys was measured by ultrasound, and the relative function of the kidneys was estimated by renal scan. Results: In all patients, the duplex kidney was the left one. The length of the right kidney on the renal ultrasound growth chart was from –1.5 to +0.4 standard deviations from the mean for age, and the left kidney length was from –0.5 to +4.3 standard deviations from the mean. On renal scans the kidneys with a duplicated system contributed 51 to 67% to total renal function; the contralateral ones, 33 to 49%. Conclusions: Kidneys with a duplicated system may be larger than their counterparts and they may contribute more to total renal function. When a disparity in length between the 2 kidneys is encountered, 1 of the possibilities that should be taken into account is a unilateral duplicated system.

Low serum C3, leukopenia, and thrombocytopenia: unusual features of henoch-schonlein purpura.

Abstract Henoch-Schonlein purpura (HSP) affects predominantly the skin, joints, gastrointestinal tract and kidney. Although the pathogenesis is probably of immune origin and complement activation is thought to play a role, laboratory findings including the serum level of the complement components are usually normal. We present a patient with a severe form of HSP nephritis who had unusual laboratory findings of a low level of C3, mild leukopenia and thrombocytopenia. These findings may further support the importance of complement activation in the pathogenesis of HSP.

Severe tubular resistance to aldosterone in a child with familial juvenile nephronophthisis

A 9.5-year-old girl, whose early symptoms were polyuria and growth retardation, is described. During the progression of her disease, hyperkalaemia developed out of proportion to the degree of renal insufficiency. Her fractional excretion of sodium increased from 3.3% to 35%, and her fractional excretion of potassium decreased from 55% to 22%. The plasma aldosterone level and plasma renin activity (PRA) were very high −290 ng/ml and 100 ng/dl per hour, respectively (normal range for this age 2.6–20.8 ng/ml and 1.2–2.7 ng/ml per hour, respectively). In an attempt to reduce these hormone levels, an acute and sustained saline load, captopril and peritoneal dialysis were used. Only the sustained saline load normalized the PRA, and only peritoneal dialysis sufficiently suppressed the plasma aldosterone level. Successful renal transplantation normalized both plasma aldosterone and PRA. This girl presents the unusual occurrence of pseudohypo-aldosteronism type I, during the course of familial juvenile nephronophthisis.

Tubular and glomerular function in children after renal transplantation.

Abstract:  Glomerular and tubular function of transplanted kidneys were assessed in 46 children aged 15.7 ± 4.6 yr, 4.2 ± 2.8 yr after renal transplantation. There were 34 cadaveric, and 12 living‐related donors. Twelve patients (26%) had acute episodes (acute tubular necrosis, rejection, or urinary tract infection) during follow‐up. All patients were on triple immunosuppression. The mean serum creatinine was 1.5 ± 0.6 mg/dL. Creatinine clearance (Ccreat) calculated from a 24‐h urine collection was 48.0 ± 19.7 mL/min/1.73 m2, and that estimated from the Schwartz formula, 61.0 ± 22.5 mL/min/1.73 m2. A positive correlation was found between the calculated and estimated clearances. Mean urine concentrating ability was 487 ± 184 mOsmol/kg, with a value lower than 400 mOsmol/kg in 35% of patients. There was a positive correlation between urine osmolality and estimated Ccreat. Metabolic acidosis (bicarbonate <22 mmol/L) was found in 41% of patients, with relatively alkaline urine and high chloride level. Fractional excretion (FE) of sodium was above 1% in 68% of patients (mean 1.66 ± 1.06%), and FEMg was above 3% (mean 10.9 ± 5.2%) in 93% of patients. Tubular reabsorption of phosphate (TP)/glomerular filtration rate (GFR) was 3.2 ± 0.8 mg/dL glomerular filtrate (GF). FEK, FEUA, and Ca/creatinine in urine were normal. There were no functional group differences between the cadaveric and living‐related kidneys. Significant group differences were found in those with acute episodes and those with a normal course. Estimated Ccreat was 54 ± 20 vs. 67 ± 20 mL/min/1.73 m2 in the acute episodes and the normal course groups, respectively. Also, the FENA, FEUA, and FEMg were higher in the acute episodes group −2.3 ± 1.6, 10.6 ± 4.4, and 14.8 ± 6.5%, respectively, compared with the normal course group −1.4 ± 0.6, 8.2 ± 2.8, and 9.6 ± 4.0%, respectively. There were no between‐group differences in plasma bicarbonate, FEK, TP/GFR, and urine osmolality. We believe that most, if not all tubular dysfunctions in the transplanted kidney are secondary to renal failure and interstitial damage from acute episodes and nephrotoxic drugs. These dysfunctions are similar to those in chronic renal failure, where interstitial fibrosis plays a role in kidney function deterioration.

Gaucher's disease and mesangiocapillary glomerulonephritis in childhood — a coincidence?

A 6-year-old boy, presenting with a nephritic syndrome, was diagnosed as suffering from Gauchers disease (GD) and mesangiocapillary glomerulonephritis (MCGN). GD was suspected because of aseptic necrosis of the femoral heads on X-ray and later confirmed by bone marrow aspiration and a lack of glucocerebrosidase activity in white blood cells; MCGN was documented on renal biopsy. The child was treated with prednisone, dipyridamole and aspirin, and recovered completely clinically. A second biopsy was not performed. The connection between these two rare diseases, and between nephritis and GD in general, is discussed.