Paediatric urology

Paediatric urology is a sub-specialty derived from urology and paediatric surgery. It is generally an elective surgical specialty with few acute conditions. Development of a good team incorporating a paediatric nephrologist, a radiologist, and a pathologist assists in the clinical diagnosis, management options, and follow-up. A paediatric anaesthetist and skilled experienced and compassionate nursing staff are essential to manage the children. This chapter covers the foreskin and circumcision, undescended testes and testis torsion, hypospadias, abnormalities of the urethra, ambiguous genitalia, and malignancies of the genitourinary tract.

Drug prescribing

Great care should be exercised when prescribing for children with kidney disease. Many drugs are renally excreted and require dose adjustment in the presence of chronic kidney disease. Children with kidney disease, particularly those who have undergone kidney transplantation, frequently receive a large number of drugs and there is significant potential for drug–drug interaction. Information is available in the British National Formulary for Children and other texts; however, advice from an experienced paediatric nephrologist or specialist renal pharmacist should be sought where there is uncertainty.

Pulsed Vincristine Therapy in Steroid-Resistant Nephrotic Syndrome

Steroid-resistant nephrotic syndrome (SRNS) poses a therapeutic challenge for the paediatric nephrologist. As relentless progression to renal failure occurs with continued proteinuria, such patients will be treated with different cytotoxic medications with variable success rates and side-effects. We present here our findings on administering the anticancer drug vincristine for SRNS patients at a single centre in Sri Lanka. Methods. Between 2002 and 2007, fifty-four children presenting with steroid and cyclophosphamide resistance were treated with vincristine at 1.5 mg/m2 in weekly intravenous pulses for 8 weeks along with a tapering steroid regimen of 6 months. All patients were closely followed up for 5 years. Results. Of the 54 patients 39 were males and 15 were females (age range 3.5–11.6 years, median 6.1 years). At the end of the treatment course, 21 patients achieved complete remission while 7 had partial remission and no response was seen in 26 patients. Sustained remission at 6, 12, 24, and 60 months were 15 (27.78%), 11 (20.37%), 9 (16.67%), and 7 (12.96%), respectively. Most side-effects observed were reversible and no serious side-effects were noted during vincristine therapy. Conclusion. Although its therapeutic mechanisms in nephrotic syndrome are still not elucidated, vincristine appears to be a potent alternative that could be considered for treating SRNS.

Growth and development

Enabling achievement of full height potential in a child with chronic kidney disease (CKD) is one of the major and most challenging goals for the paediatric nephrologist. Short stature is associated with psychological maladjustment and with increased morbidity and mortality. The causes of poor growth are multifactorial and include poor nutrition, and metabolic, haematological, and endocrine disturbances. The most vulnerable times are the periods of most rapid growth, that is, infancy and puberty. Growth during infancy is principally dependent on nutrition so many infants need supplemental enteral feeding. Growth delay correlates with severity of CKD, with those on dialysis faring the worst such that by CKD stage 5, approximately 25% of patients are below the normal range for height. Height achieved post transplant is dependent on graft function and is better in younger children and those who have the best height attainment pre transplant. The use of steroid-free immunosuppressive regimens is encouraging. The prognosis for final height is improving.