Stephen A. Hinchliffe

The effect of intrauterine growth retardation on the development of renal nephrons

Objective To investigate the effect of Type II (asymmetrical) intrauterine growth retardation (IUGR) on renal development.

Renal hypoplasia and postnatally acquired cortical loss in children with vesicoureteral reflux

We reviewed histologically 86 nephrectomy specimens from patients with vesicoureteral reflux (with or without ureterovesical obstruction) to investigate the relationship between coexisting hypoplasia and postnatally acquired cortical damage. Hypoplasia was assessed independently of the acquired cortical loss using medullary ray glomerular counting. Severe hypoplasia (glomerular number <25% of normal) was detected in 47 of 86 patients. These patients underwent nephrectomy at a significantly younger age than those with minimal or no hypoplasia (P<0.01). There was no significant relationship between the severity of hypoplasia and the presence or absence of obstruction. Severe acquired cortical loss was found in 68 of 86 patients. There was no significant association between the severity of cortical loss and the presence or absence of obstruction, age at nephrectomy or degree of coexisting hypoplasia. The findings suggest a strong association of hypoplasia and vesicoureteral reflux. Therefore, early postnatal presentation with minimal renal function need not necessarily reflect a failure of management but rather a pre-existing limitation of renal capacity. Further-more, in a significant proportion of fetuses with ultrasonographic evidence of urinary tract abnormality, renal pathology may be present prior to the time at which in utero surgical intervention may be considered.

Composition of the inflammatory infiltrate in pediatric penile lichen sclerosus et atrophicus (balanitis xerotica obliterans): a prospective, comparative immunophenotyping study.

Dermatopathological evaluation of pediatric preputial inflammatory disease rarely allows for specific diagnosis other than pediatric penile lichen sclerosus et atrophicus (balanitis xerotica obliterans, LSA/BXO). A prospective immunopathological study was performed on 20 consecutive, unselected, clinically and histopathologically confirmed LSA/BXO cases to determine the relative presence of T and B lymphocytes. There were seven cases with early stages of disease, eight with florid disease, and five with later stages of disease. Two ritual circumcision specimens and 12 specimens with non-LSA/BXO balanitis, collected during the same period, were used as controls. The infiltrate in LSA/BXO patients was wholly composed of T cells (positive with UCLH-1 antibody) in all cases. B cells (positive with L-26 antibody) were found only focally in small, discreet, easily recognizable (follicular or early follicle-like) aggregates, positioned slightly deeper than the band-like infiltrate of T cells. T cells were inconspicuous in 9 of the 12 control specimens. In the three other controls, T cells were much more obvious and these patients showed clinical features possibly suggestive of LSA/BXO in early, prediagnosable phases of development. We conclude that limited immunophenotyping may be a useful adjunct to diagnosis in pediatric cases in which only limited tissue is available or the disease may be more difficult to classify with confidence.

An Assessment of Volume-weighted Mean Nuclear Volume Estimates as a Prognostic Index for Neuroblastoma

This study evaluates an objective, unbiased, stereologic parameter (volume-weighted mean nuclear volume vV) as a prognostic indicator for survival of neuroblastoma in comparison with three histopathologic grading systems. In this retrospective study 24 consecutive, nonselected patients from the registry of Royal Liverpool Childrens Hospital, Alder Hey, England were analyzed. Only primary lesions obtained before chemotherapy were used. Follow-up time in surviving patients (n = 10) was 4.5 to 20 years. All lesions were regraded blind and twice by two pathologists. vV was estimated on routinely processed, hematoxylin and eosin-stained, 5-microns sections, requiring on average less than 20 min per patient. Results show an absolute cut-off point for survival at vV = 270 microns 3. No patient with vV less than this value is alive at present (n = 7). In addition, actuarial survival curves for nonsurvivors show a bimodal pattern of survival time, separating patients with vV greater than (n = 7) and less than (n = 7) 270 microns 3. In comparison with the same analysis for the results of regrading by means of the Hughes or Beckwith system, results of vV estimation were superior. In comparison with the Shimada system the results confirmed the strong dichotomy for survivors and nonsurvivors, although with more overlap. vV has the advantage of predicting length of survival in nonsurvivors. The combination of Shimada grading and vV measurement, on the basis of the material studied, seems to offer useful prediction of biologic behavior. vV was always estimated on the small cell population, reducing the problems of biopsy representability.