Minimal Change Nephrotic Syndrome in a Child with Systemic Lupus Erythematosus.

Renal involvement in systemic lupus erythematosus (SLE) is well known. We present a 16-year-old boy, who was in remission on treatment for SLE for the past three years and now presented with nephrotic syndrome. The kidney biopsy was normal with the immunofluorescence revealing no deposits. He went into remission by day 9 of treatment and completed the course of treatment with prednisolone alone with no relapses. The clinical picture along with the histology and autoimmune markers for SLE indicate that he developed minimal change nephrotic syndrome that was responsive to prednisolone. It is important to be aware that minimal change nephrotic syndrome can occur in a patient as part of lupus podocytopathy and heavy immunosuppression may be unwarranted.

Undescended testis and torsion: is the risk understated?

Undescended testis (UDT) is seen in 3% to 5% of all newborn boys. Complications such as infertility and malignant transformation have been well documented in UDT. However, torsion of a UDT can also occur and the diagnosis is often missed or delayed, leading to loss of testis. This event may occur even before the currently recommended age for surgery, which is at 6-9 months. We present a case series of six children with torsion of undescended testes and their subsequent diagnosis and management. The risk of torsion of UDT is understated. Paediatricians should be educated about this complication and torsion should be included in the differential diagnosis when a boy with an empty scrotum presents with acute abdomen or red and tender swelling in the groin, as early detection and intervention can help salvage the testes.

Local steroid therapy as the first-line treatment for boys with symptomatic phimosis - a long-term prospective study.

AIM: Phimosis is a common paediatric urological disorder and often necessitates circumcision. We prospectively evaluated local steroid therapy (LST) as the first choice therapy for such children. METHODS: Two hundred and sixty symptomatic boys up to 15 years of age (mean 34 months) with phimosis were started on betamethasone dipropionate (0.05%) application on gently stretched prepuce twice a day. Follow-up visits were arranged at the end of weeks 1, 2 and 4 and 6 months. Grade of phimosis was objectively graded. RESULTS: Ninety one percent of the boys showed a successful outcome at the end of 4 weeks; 72% responded in first week, further 16% responded in week 2, and only 2.6% achieved alleviation of phimosis on further application of LST beyond 2 weeks. Fourty two (17.8%) boys had a recurrence of phimosis on a long-term follow-up (mean - 25.4 months, range 6-48 months); thus, the long-term success rate was 77%, while 60 (23%) boys underwent surgery. CONCLUSION: Local steroid therapy is safe and successful in alleviating symptomatic tight foreskin in a large majority of children. The response can be seen as early as 1 week; most of the children respond by week 2 and continuing therapy further may not be very effective.

Pulmonary thrombosis in steroid-sensitive nephrotic syndrome.

Thrombo-embolic episodes are an uncommon but known complication of nephrotic syndrome. However, pulmonary thrombosis/thromboembolism is rare, especially in children. We describe the cases of two girls, aged 12 years, who presented with severe oedema in relapse. They had intermittent tachypnoea, and CT pulmonary angiography (CTPA) provided a less invasive and more definitive way of confirming pulmonary thrombosis/thromboembolism. They received heparin with resolution of the tachypnoea. Anticoagulation was continued for 6 months after the episode in one patient. They have been in remission for more than 1 year, and a thrombophilia screen does not indicate a predisposing tendency to the formation of clots. Pulmonary thrombosis/thromboembolism could present with subtle symptoms and needs prompt diagnosis and treatment to prevent a fatal outcome.

Laparoscopic renal biopsy in obese children.

Percutaneous kidney biopsy is routinely used to obtain renal tissue for histological examination. It is usually successful and has very few contraindications. We describe two children with clinical obesity in whom the percutaneous approach failed to yield renal tissue for histology. They underwent successful laparoscopic kidney biopsies that yielded adequate renal tissue for diagnosis, although the first attempt in one patient yielded renal medulla necessitating a repeat biopsy. We recommend that laparoscopic kidney biopsies should be considered in obese children when percutaneous kidney biopsies are considered impossible.

Tubulointerstitial nephritis and uveitis in monozygotic twin boys.

We describe monozygotic male twins who developed tubulointerstitial nephritis and uveitis (TINU) almost 2 years apart. They presented with non-specific symptoms and were noted to have glycosuria and renal impairment. Both children have uveitis. One had biopsy-proven interstitial nephritis and the other had biochemical evidence of transient tubular dysfunction. While the renal parameters improved, they are still under treatment for uveitis. The occurrence of TINU in identical twins at an interval of just under 2 years supports a strong genetic element in the aetiology of this syndrome. We believe this is the first report of male twins with TINU.

Ondansetron for paediatric uraemic pruritus: a case report.

Pruritus, secondary to chronic renal failure, can be an extremely distressing symptom. A 15-year-old girl, in end-stage renal disease, presented with pruritus that did not improve on starting peritoneal dialysis. Despite normalising the calcium, phosphate and parathyroid levels, pruritus worsened and showed no response to antihistamines. She was commenced on ondansetron and showed dramatic improvement. Severe uraemic pruritus is rare, but distressing in children and can improve with ondansetron.

Improved efficacy of basiliximab over antilymphocyte globulin induction therapy in paediatric renal transplantation.

BACKGROUND: Basiliximab is a chimeric human/mouse monoclonal antibody directed against the alpha chain of the IL-2 receptor, CD25, which has been reported as successfully reducing rejection in adult renal transplant recipients. Reported clinical experience of basiliximab in paediatric renal transplantation is limited. METHODS: Using two intravenous doses on day 0 (pre-operatively) and day 4 with prednisolone and cyclosporin A (dual) maintenance immunosuppression in 42 children undergoing renal transplantation in our unit (SIM group), we have compared patient and graft outcome, rejection rates in the first 6 months, renal function and the incidence of Cytomegalovirus (CMV) infection with 42 consecutive children who previously received antilymphocyte globulin immunoprophylaxis with prednisolone, cyclosporin A and azathioprine (triple) maintenance immunosuppression (ALG group). The two groups were similar, including HLA mismatching, apart from age and size at transplantation (SIM=10.3+/-5.4 years vs ALG=12.4+/-4.2 years, P<0.05). RESULTS: One patient in the SIM group died from food inhalation with a functioning kidney and one patient in the ALG group from Pneumocystis pneumonia and post-transplant lymphoproliferative disorders with a rejecting graft. Both 1- and 2-year actuarial graft survivals were 93% for the SIM group and 86% for the ALG group (NS). Three grafts were lost in the SIM group-none from rejection (thrombosis 2, death 1)-and seven in the ALG group-three from rejection. Occurrence of biopsy documented rejection in the first 6 months after transplantation was 0.15+/-0.22 for the SIM group and 0.35+/-0.51 episodes per pt-month at risk for ALG treatment (P<0.04). Early rejection within 30 post-operative days occurred in only four SIM patients, three of whom had undergone retransplantation. Forty-seven per cent of rejection episodes occurred between days 30 and 44 in SIM treated patients. Switching to tacrolimus was similar in both groups; 24% of the SIM groups were prescribed triple therapy. Estimated glomerular filtration rate was 46.0 and 46.2 ml/min for SIM and ALG groups, respectively, six months after transplantation. Ten per cent of SIM and 19% of ALG treated patients developed clinically significant CMV infection (NS) but none of 16 (R(+)) SIM children had CMV infection compared with 8 out of 15 (R(+)) ALG patients (P<0.01). CONCLUSIONS: Basiliximab immunoprophylaxis and dual therapy reduces rejection episodes in the first six months and maintains graft survival and function after paediatric renal transplantation. Seventy-six per cent of children receiving basiliximab immunoprophylaxis were successfully maintained on long-term dual immunosuppression. This immunosuppressive protocol reduces CMV disease in CMV(+) recipients compared with ALG induction and triple therapy.