The national incidence and clinical picture of SLE in children in Australia - a report from the Australian Paediatric Surveillance Unit.

OBJECTIVES: The objectives of this paper are to prospectively determine the incidence of paediatric systemic lupus erythematosus (pSLE) in Australia as well as describe the demographics, clinical presentation and one-year outcome. STUDY DESIGN: Newly diagnosed cases of pSLE were ascertained prospectively from October 2009 to October 2011 through the Australian Paediatric Surveillance Unit (a national monthly surveillance scheme for notification of childhood rare diseases) as well as national subspecialty groups. Questionnaires were sent to notifying physicians at presentation and at one year. RESULTS: The annual incidence rate was 0.32 per 10(5) children aged less than 16 years. The incidence was significantly higher in children of Asian or Australian Aboriginal and Torres Strait Islander parents. Approximately one-third of children underwent a renal biopsy at presentation and 7% required dialysis initially although only one child had end-stage kidney disease (ESKD) at one-year follow-up. CONCLUSION: The incidence of pSLE in Australia is comparable to that worldwide with a significantly higher incidence seen in children of Asian and Australian Aboriginal and Torres Strait Islander backgrounds. Renal involvement is common but progression to ESKD, at least in the short term, is rare.

Safety and adequacy of percutaneous biopsies in pediatric orthotopic kidney transplantation.

BACKGROUND: The gold standard for investigating the cause of renal graft dysfunction is renal biopsy. However, as this procedure is invasive and has inherent risks, its safety must be established. OBJECTIVE: To determine the safety of percutaneous renal biopsy in pediatric orthotopic renal transplantation. METHODS: Percutaneous renal biopsies performed on pediatric orthotopic renal transplants in a single center between 1987 and 2010 were studied. Biopsy specimen adequacy and post-procedure complications were reviewed by prospectively collected data. RESULTS: A total of 54 ultrasound "real-time" guided biopsies in 25 patients were performed. Minimum specimen adequacy was achieved in 98% of biopsy specimens. No major complications were identified; 6% of patients developed minor complications-e.g., grade 3 macroscopic hematuria that did not require intervention. CONCLUSION: Percutaneous renal biopsies using "real-time" ultrasound guidance on pediatric orthotopic kidney transplants is safe.

BK viral infection in an Australian pediatric renal transplant population.

BK virus (BKV) is recognized as a significant cause of renal allograft dysfunction in adults, and there is growing awareness of its importance in the pediatric population. Eighteen pediatric renal transplant recipients and 18 age-matched controls were prospectively studied. Anti-BKV immunoglobulin G (IgG) and IgM titres were assayed in all subjects at entry to the study. Polymerase chain reaction (PCR) for BKV DNA was performed on urine and serum at entry, and prospectively tested again at 4, 8 and 12 months. Mean age +/- s.d. of transplant recipients and controls was 14.6 +/- 3.3 and 13.9 +/- 0.33 yr respectively [not significant (NS)]. Transplant patients were studied at a mean time of 5.6 +/- 4.2 yr post-transplant. 56% of transplant patients and 39% of controls were seropositive (+ve BKV IgG) (NS). Plasma BKV PCR was positive in one transplant patient (who also had positive urine PCR) and in none of the controls. The prevalence of positive urine PCR in transplant patients was greater than in controls (33% vs. 0%, p = 0.02). Positive urine BKV PCR was more commonly found in patients treated with mycophenolate than azathioprine (p = 0.04). We conclude that the prevalence of BKV seropositivity and viral activation in this Australian pediatric renal transplant population is similar to that reported in adult and pediatric populations in other countries. BK viruria was more common in children with greater immunosuppression, suggesting that this group is at higher risk of BKV induced nephropathy.

Perception of fear, distress and pain by parents of children undergoing a micturating cystourethrogram: a prospective study.

OBJECTIVES: To investigate whether parents' expectations of their child's fear, distress or pain during a micturating cystourethrogram (MCU) are realized. METHODOLOGY: Prospective study in which parents were asked to fill out two questionnaires using a visual analogue scale, one before (pre) and the other after the MCU procedure (post), was conducted at a tertiary level paediatric hospital in Sydney, Australia. The questionnaires were designed to compare the parents' anticipated and experienced anxiety about their child's procedure and their perception of fear, distress and pain in their child during and after the procedure. The parents' satisfaction with information provided to them on the procedure was also recorded. Twenty-five parents participated in the study. RESULTS: There were significant differences between anticipated and experienced parental anxiety. Parents' reporting of fear, distress and pain in their child during the MCU and after the procedure was lower than they had anticipated. There was a significant correlation between the parents' anxiety and their perception of severity of their child's fear (r = 0.52, P = 0.009), distress (r = 0.48, P = 0.017) and pain (r = 0.50, P = 0.01) during the procedure, but less so with the child's distress after the procedure (r = 0.39, P = 0.059). The parents were satisfied with the information given to them regarding the MCU procedure. CONCLUSIONS: Parents' perception of their child's fear, distress and pain during the MCU, as well as distress following the MCU, was not as severe as they had anticipated. Parental anxiety is an important factor in the perception of fear, distress and pain in children during and after the procedure.

Pediatric biopsy of a single native kidney.

Technological improvements have reduced the frequency of complications in children receiving a percutaneous renal biopsy. No study has systematically compared the safety of open and percutaneous kidney biopsy. Yet many nephrologists consider a single native kidney an absolute contraindication to percutaneous biopsy. We have established an international registry of single native kidney biopsies in children and we now report our early results. Eight biopsies are included. Seven patients had percutaneous biopsies and one an open biopsy. None of the patients had major complications, and adequate tissue was obtained from all. Our limited experience indicates that the presence of a single native kidney is not an absolute indication for an open approach. We encourage our colleagues to report to the international registry in order to further document the safety of percutaneous biopsy of the single native kidney in children.

Metanephric adenoma with embryonal hyperplasia of Bowman's capsular epithelium: previously unreported association.

We report a case of a 9-year-old boy with focal, segmental glomerulosclerosis who, following peritoneal dialysis, underwent renal transplantation and bilateral nephrectomy. The kidneys showed histological features of embryonal hyperplasia of Bowman's capsular epithelium, an uncommon lesion that is seen most often in patients with chronic renal failure who are being maintained on dialysis. In addition, a 1-cm tumor in the left kidney showed features of metanephric adenoma. Although both lesions are uncommon, they share many similarities on a morphological, immunohistochemical, and ultrastructural basis. This association has not been previously reported and may shed some light on the histogenesis of these recently described lesions.

Low incidence of new renal scars after ureteral reimplantation for vesicoureteral reflux in children: a prospective study.

PURPOSE: The major aim of treating vesicoureteral reflux in children is the prevention of renal scars. Dimercapto-succinic acid (DMSA) is the modality of choice for detecting renal scars. We documented the incidence of new renal scarring and measured changes in differential renal function after ureteral reimplantation using DMSA studies. MATERIALS AND METHODS: We evaluated 45 boys and 98 girls with a median age of 2 years who had vesicoureteral reflux and underwent ureteral reimplantation. DMSA scans were done preoperatively and at a median of 3.4 years postoperatively. Maximal reflux grade was III in 84 children (59%), IV in 27 (19%) and V in 6 (4%). RESULTS: Preoperatively DMSA studies showed scarred or contracted kidneys in 106 of the 143 patients (74%). After reimplantation mean change in differential function was 2.5%. New scars developed in 3 children (2%). We noted greater than 6% decrease in relative differential function without new scarring in 7 cases (5%). CONCLUSIONS: The incidence of new renal scars in our study using DMSA was lower than that in previous series using excretory urography and imaging. Surgical correction of vesicoureteral reflux may offer better protection of kidneys in childhood than previously believed.

Elevated creatinine after ingestion of model aviation fuel: interference with the Jaffe reaction by nitromethane.

A 2 years 9 month-old-boy who ingested model aviation fuel was found to have an elevated serum creatinine concentration of 0.53 mmol/L (normal range 0.02-0.05 mmol/L) by the Jaffe (alkaline picrate) reaction. However, when the creatinine concentration was measured using a specific enzymatic assay, it was within the normal range. It was shown that nitromethane, a constituent of aviation fuel, interferes with the Jaffe reaction, leading to a falsely elevated creatinine reading. This phenomenon has been reported only once previously and, despite its potential clinical importance, nitromethane does not appear in published lists of substances that interfere with the Jaffe reaction.

Recurrent bacteremia with enteric pathogens in recessive polycystic kidney disease.

Eight children with autosomal recessive polycystic kidney disease (ARPKD) and recurrent bacteremia with enteric pathogens are described. Typical clinical features of bacterial cholangitis were absent, although in five patients histological and/or microbiological data indicated that the bacteremic episodes originated in the biliary tree. Bacteremia with enteric pathogens or recurrent culture-negative febrile illness in a child with ARPKD should raise suspicion of cholangitis, even in the absence of typical clinical findings.

MAG3 renal scintigraphy: improved ability to make anatomical diagnoses in neonates.

Technetium-99m mercaptoacetyltriglycine (MAG3) is the most recently introduced renal radiopharmaceutical in Australia and is established as the agent of choice for use in diuresis renography, particularly in neonates and infants. It provides superior anatomical information compared to previously used agents. Three cases are reported in which MAG3 diuresis renography was performed in neonates, who were found to have hydronephrosis detected antenatally. In two neonates, a previously unrecognized horseshoe kidney was demonstrated and in case 3 there were scan features characteristic of a ureterocele. It is highly unlikely that these abnormalities would have been delineated with 99mTc dimethyltriamine pentaacetic acid (DTPA) study, as confirmed in case 1, because of the relatively poor uptake of DTPA when compared to MAG3.

Effects of growth hormone treatment for short stature on calcium homeostasis, bone mineralisation, and body composition.

We investigated the effect of growth hormone (GH) treatment on mineral and vitamin D homeostasis, bone mineralisation, and body composition in short-statured children without GH deficiency (GHD). 11 children received GH (0.50 +/- 0.08 IU/kg/week) for 24 weeks. 1,25-Dihydroxyvitamin D3 levels (mean +/- SD in pmol/l) rose from a baseline of 73.7 +/- 39.2 to 114.0 +/- 32.7 at 8 weeks (p < 0.05) and 111.9 +/- 39.7 at 24 weeks (p < 0.01). Body composition evaluation using dual-energy X-ray absorptiometry revealed increased lean tissue mass and a reduction in fat tissue. As a percentage of total body mass, fat decreased from 19.0 +/- 11.8% at baseline to 17.3 +/- 11.5% at 8 weeks (p < 0.005) and 16.8 +/- 11.5% at 24 weeks (p < 0.05). L2-L4 bone mineral density was 0.637 +/- 0.155 g/cm2 at baseline and 0.666 +/- 0.160 g/cm2 at 24 weeks (NS). We conclude that recombinant human GH treatment of short children without GHD has significant effects on vitamin D homeostasis and body composition.

Renal effects of growth hormone. II. Electrolyte homeostasis and body composition.

Growth hormone (GH), either directly or through insulin-like growth factor-1 (IGF-1), has a wide spectrum of physiological and renal effects. This review concentrates on the effects of GH (derived from either pituitary or recombinant technology) and IGF-1 in three main areas: (1) sodium and water homeostasis; (2) calcium and phosphate balance, bone density and interactions with mineral regulating hormones; (3) fat and lean body mass. Observations of physiological changes in states of GH deficiency and excess in humans and animal models are presented. The lack of long-term toxicological data indicates that GH treatment for short stature in non-GH deficient children, with or without renal disease, should proceed with caution.

Renal effects of growth hormone. I. Renal function and kidney growth.

Growth hormone (GH) affects renal function and kidney growth. Pituitary-derived or recombinant human GH (rhGH), acting via insulin-like growth factor-1 (IGF-1), increases glomerular filtration rate (GFR) and renal plasma flow (RPF) in GH-deficient as well as in normal adults. Furthermore, GFR and RPF are low in hypopituitarism and elevated in acromegaly. These effects of GH on GFR and RPF have not been demonstrated in moderate renal insufficiency. IGF-1 is implicated in compensatory renal hypertrophy. Markedly elevated levels of serum GH accelerate glomerular sclerosis in rodents, although the significance of these findings for GH treatment in humans is uncertain. rhGH therapy offers great promise to children with short stature from various aetiologies. Preliminary reports on the use of rhGH in children with renal disease and after renal transplantation have not shown any consistent change in kidney function, although follow-up times are short. The long-term impact of rhGH therapy on kidney function in short children needs further evaluation.

Hypercalciuria due to combined growth hormone and calcitriol therapy in uremia: effects of growth hormone on mineral homeostasis in 75% nephrectomized weanling rats.

The administration of growth hormone (GH) in conjunction with calcitriol in uremia may increase urinary calcium and decrease renal phosphate excretion, which could have an adverse effect on the kidney in chronic renal insufficiency. The effect of 40 d of ovine GH, calcitriol, and the combination of GH and calcitriol on mineral excretion was studied in rapidly growing uremic rats. Uremia was produced by 75% nephrectomy, and the animals were fed a diet containing 8% protein with equal quantities of calcium (0.6%) and phosphate (0.6%). The uremic rats treated with ovine GH were significantly longer and heavier than the uremic control rats and the uremic rats treated with calcitriol alone. However, the combination of calcitriol and GH abolished the beneficial effect of GH on growth and increased urinary calcium excretion 4-fold over uremic controls whether expressed as calcium excretion per 100 g body weight, urine calcium to creatinine ratio, or as fractional calcium excretion. Calcitriol therapy alone also significantly increased calcium excretion, but not to the extent that the combination therapy did. This increased urinary calcium excretion in the GH plus calcitriol group was not associated with an increase in calcium and sodium intake, plasma ionized calcium, or urinary sodium excretion. The calcium content of the femurs from all uremic rat groups was significantly lower than that of the sham control rats; however, there was also no further decrease in bone calcium content in the GH plus calcitriol group compared with uremic controls. This indicated that bone was not the source of this excess urinary calcium.(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormal cortical appearances in pediatric renal allografts.

Renal cortical studies were performed in 19 children with renal transplants. There were 10 normal studies and 9 abnormal studies, 8 of which showed multiple large focal peripheral cortical defects. The following factors showed a positive correlation: (a) the ischemia time of the transplant kidney was significantly shorter in patients with normal studies; (b) cadaver grafts were more likely to have abnormal scan appearances than living related donor grafts; and (c) in four of the five patients with double renal arteries, the scans were abnormal in multiple sites. A possible pathophysiologic mechanism to explain these scan appearances is asymptomatic segmental graft infarction secondary to progressive vascular disease. These infarcts may be a long-term sequela of ischemic insult at the time of or prior to the insertion of the renal allograft.

Glomerulonephritis, pulmonary hemorrhage and anemia associated with Campylobacter jejuni infection.

A 5-year-old girl suffered Campylobacter jejuni enteritis. Over the ensuing weeks she developed glomerulonephritis, pulmonary hemorrhage and anemia. Renal biopsy revealed immune-complex mediated, crescentic glomerulonephritis. Campylobacter jejuni antigen was identified in the glomeruli suggesting a causal role of Campylobacter jejuni in the disease process.