Effect of cyclosporin A on glomerular filtration rate in children with minimal change nephrotic syndrome

Cyclosporin A (CyA) is now commonly used in the management of children with steroid-dependent nephrotic syndrome. In order to assess nephrotoxicity related to CyA therapy, we measured glomerular filtration rate (GFR) on 123 occasions in 24 children with minimal change nephrotic syndrome receiving CyA. GFR was estimated from the plasma clearance of⁵¹chromium-EDTA every 3 months during CyA therapy of up to 27 months duration. There was a significant reduction in GFR after 3 months of CyA therapy [118±33 (SD) to 93±24 ml/min per 1.73 m²] but no further fall thereafter, although the reduction in GFR was sustained for the duration of CyA therapy. This reduction in GFR appeared to be reversible upon cessation of CyA, but careful monitoring of renal function is necessary in such patients to prevent the development of longer term nephrotoxic sequelae.     

Clinical Assessment of Gestational Age

There is disagreement among authors as to what criteria are most reliable in assessing postnatal fetal age. A comparison–for objectivity, accuracy, and frequency of use– of specific criteria was made using three independent examiners at different educational levels: a second-year medical student, a perinatal nurse practitioner, and a pediatrics resident. It was shown that trained examiners develop favorite criteria which differ among examiners and thus sacrifice accuracy; however, accurate assessment of fetal age can be accomplished by an examiner with minimum clinical knowledge.     

XY sex-reversed campomelia

A phenotypic female with campomelic syndrome and XY gonadal dysgenesis is described. A minor structural chromosome abnormality was detected in the mother.     

Working With Parents Through a Devastating Experience-The Birth of a Mongoloid Child

The author believes that professional advice given the parents of a mongoloid infant determines their initial decision on institutionalization. Multidisciplinary counseling by the health team can help parents decide on a course of action that will prove satisfactory in the long run. Consultation should include health t e r n efforts to help parents work through the mourning process and presentation of a) an objective, comprehensive view of the child's condition, b) information on integrating a mongoloid into the family unit, and c) outside resources available to the parents. Deliberation with the parents to help them reach their own decision is essential.     

Difficulties in Immunohaematology : The Weak D Antigen

Background

The Rh blood system is one of the most polymorphic and immunogenic systems known to humans. The expression of Rh blood group antigen is complex. The Rh D antigen is the most important of the antigens that constitute the Rh antigen system. In most cases, D antigen can easily be detected. However, due to variability of expression, weak forms antigen are encountered. The reactivity of weak D with antisera is variable and presents as a problem in blood banking.

Methods

A retrospective analysis for a five-year period was done. Blood samples that were negative for Rh D by immediate spin tube method were tested for weak D antigen by additional lab tests.

Result

Of 34932 serial Rh grouping tests done in our Blood Bank, the incidence of weak D Rh antigen was 0.189%. All these were confirmed by the antiglobulin test.

Conclusion

These patients present as a problem for the blood banker and a curiosity to the clinician. Although uncommon, all health care workers should be aware of this entity to avoid anti D alloimmunisation.Key Words: Weak D, Rh Blood Group     

What is the calcineurin inhibitor of choice for pediatric renal transplantation?

Cyclosporine microemulsion (CyA) and tacrolimus (Tac) are the principal immunosuppressants prescribed for adult and pediatric renal transplantation. In the majority of patients, these calcineurin inhibitors have been used in combination with other immunosuppressive drugs, such as azathioprine or mycophenolate mofetil (MMF). In this review we will address the question of what calcineurin inhibitor we should use in an individual pediatric renal transplant patient. Well-designed randomized studies in children showed no difference in short-term patient and graft survival with cyclosporine microemulsion and tacrolimus. However Tac is significantly more effective than CyA microemulsion in preventing acute rejection after renal transplantation in a pediatric population when used in conjunction with azathioprine and corticosteroids. This difference disappears when calcineurin inhibitors are used in combination with MMF as both Tac and CyA produce similar rejection rates and graft survival. However, Tac is associated with improved graft function at 1 and 2 yr post-transplant. Adverse events of hypomagnesaemia and diarrhea seem to be higher in Tac group whereas hypertrichosis, flu syndrome and gum hyperplasia occurs more frequently in the CyA group. The incidence of post-transplant diabetes mellitus was almost identical between Tac and CyA treated patients. The recommendation drawn from the available data is that both CyA and Tac can be used safely and effectively in children. However Tac may be preferable to CyA because of steroid sparing effect and less hirsutism. We recommend that cyclosporine should be chosen when patients experience Tac-related adverse events. Nevertheless, the best calcineurin inhibitor should be decided on individual patients according to variable risk factors, such as risk of rejection in sensitized patient or delayed graft function. The possibility of adverse events should also be considered.     

Cognitive and psychosocial outcome of infants dialysed in infancy

OBJECTIVE: To contribute further to the understanding of cognitive and psychosocial outcome of children with end-stage renal disease undergoing long-term peritoneal dialysis. METHODS: In total, 16 surviving infants at a single centre beginning peritoneal dialysis in the first year of life were studied. The age range of the children at assessment was 1.6-12.1 years. Children were assessed using the Griffiths Mental Development Scales, the Wechsler Intelligence Scale for Children-Third Edition UK, and the Strengths and Difficulties Questionnaire. Information regarding the child's hospital stay and family background was also collated. A Pearson's Product Moment correlation was used to analyse the results. RESULTS: Although 67% of the children's scores fell within the average range, 87% were within at least two SDs of the norms (mean IQ = 86.6). Psychosocial adjustment measures revealed that 50% of scores fell within the borderline to abnormal category, suggesting that the frequency of psychological difficulties was above that of the normal population. CONCLUSIONS: These findings lend support to recent studies indicating that, developmentally, children undergoing long-term peritoneal dialysis are faring better than in the past. This may indeed be a reflection of improvements in renal treatment and diet. The behavioural results suggest the need to monitor psychological adjustment in this group of children.