No abstract

There is a remarkable variation of incidence of biliary atresia within England and Wales, some of which may have been caused by factors related to a different aetiological and racial background.

There is a marked detrimental effect of age at KP on groups with a presumed "developmental" origin, not seen in the majority with isolated BA.

SUMMARY Ninety three children (51 boys, 42 girls) who had been treated for brain tumours not affecting the hypothalamopituitary axis, were studied for evidence of gonadal dysfunction. All had received cranial irradiation, 59 spinal irradiation, and 28 adjuvant chemotherapy. Mean age at treatment was 6-3 years (range 1.5-15). Mean follow up after completion of radiotherapy was 8-5 years (range 1-27). Primary ovarian damage occurred in seven out of 11 (64%) girls treated with craniospinal irradiation alone and in nine out of 14 (64%) of those treated with craniospinal irradiation and chemotherapy. The association with spinal irradiation was significant. Primary gonadal damage also occurred in three out of four children treated with chemotherapy combined with cranial irradiation and in three out of nine boys treated with chemotherapy and craniospinal irradiation but in no boy given craniospinal irradiation alone. The only common chemotherapeutic agent was a nitrosurea. Hypogonadotrophic hypogonadism was found in seven boys, 5-8% of children of pubertal age.Primary gonadal dysfunction is a well known complication of the treatment of some childhood malignancies, particularly leukaemia and lymphoma.'-8The prevalence and aetiology of gonadal dysfunction after treatment of brain tumours in childhood are unclear; this is because reported series are relatively small and because of differences in treatment. Most authors have attributed gonadal damage to scatter from spinal irradiation9 10 but more recently Ahmed et al concluded that adjuvant chemotherapy was responsible.' Brown et al also referred to girls with primary ovarian damage after treatment with lomustine (CCNU) without spinal irradiation.'0) Three affected girls with ovarian damage attributed to spinal irradiation, described by RapVaport et al, had also received chemotherapy.Gonadal dysfunction can also be secondary to gonadotrophin deficiency caused by cranial irradiation 9 12 but there are few data on the incidence after the treatment of childhood brain tumours. Rappaport et al reported that gonadotrophin deficiency may occur infrequently in these patients.9We have studied the aetiology and prevalence of primary and secondary gondal dysfunction in a large cohort of children treated for brain tumours. Patients and methodsAltogether 125 children who were in clinical remission after receiving radiotherapy for brain tumours not affecting the hypothalamopituitary region were studied. Information about gonadal function is reported for 93 of them (51 boys, 42 girls). The remaining 32 children were still prepubertal with serum gonadotrophin concentrations appropriate for age. They were not included in the analysis because hypogonadotrophic gonadal dysfunction cannot be excluded until they reach puberty.' 3 The mean age of the 93 children at treatment was 6-3 years (range 1-5-15) and mean follow up since completion of radiotherapy 8 5 years (range 1-27).Eighty one children were prepubertal when treated, 86 children (48 boys, 38 girls) were of pubertal age when studied. ...

SUMMARY We investigated thyroid function in 119 survivors of treatment for brain tumours not involving the hypothalamo-pituitary region. Cranial irradiation did not effect thyroid function but 11 of 47 children (23%) who had spinal irradiation had raised concentrations of thyroid stimulating hormone. Chemotherapy further increased the incidence of thyroid dysfunction: two of four patients who had cranial irradiation and chemotherapy and 20 of 29 patients (69%) who had spinal irradiation and chemotherapy had increased thyroid stimulating hormone concentrations. Only six patients with raised thyroid stimulating hormone concentrations had low serum thyroxine concentrations. Four children had secondary hypothyroidism.Thyroid function should be monitored in children who have received chemotherapy or radiotherapy. A rise in thyroid stimulating hormone concentrations is the most sensitive indicator of thyroid dysfunction. Children with raised thyroid stimulating hormone concentrations should be treated with thyroxine.Endocrine complications of radiotherapy and chemotherapy given to children with brain tumours separated from the hypothalamo-pituitary axis have been documented.1 Growth hormone insufficiency is the commonest disorder,2 followed by primary thyroid and gonadal dysfunction, and then secondary thyroid and gonadal dysfunction.3 4 Irradiation is the major aetiological factor, but chemotherapy has been found to cause primary gonadal damage.-7There is only limited information from small studies about thyroid function and we report our observations on the prevalence, aetiology, and management of thyroid dysfunction in a large series of survivors. Patients and methodsThe thyroid function of 119 children in clinical remission after treatment of a brain tumour not involving the hypothalamo-pituitary region was studied. The tumour diagnoses were medulloblastoma (n=52), astrocytoma (n=29), ependymoma (n= 15), pinealoblastoma (n=9), glioma (n=8), optic nerve glioma (n=5), and meningioma (n=1). Cranial irradiation in a median (range) estimated hypothalamic dose of 47 Gy