Seventy-five children treated for craniopharyngioma between 1973 and 1994 were studied to demonstrate which pre- and intraoperative factors were indicative of a poor outcome as defined by a quantitative assessment of morbidity. This involved a retrospective review of 65 patients and a prospective study of 10 patients focused on clinical details and cranial imaging and a follow-up "study assessment" of 66 survivors performed over the last 2 years. As part of the assessment, 63 patients underwent magnetic resonance imaging with a three-dimensional volume acquisition sequence 1.5 to 19.2 years after initial surgery. Predictors of high morbidity included severe hydrocephalus, intraoperative adverse events, and young age ( < or = 5 years) at presentation. Predictors of increased hypothalamic morbidity included symptoms of hypothalamic disturbance already established at diagnosis, greater height ( > or = 3.5 cm) of the tumor in the midline, and attempts to remove adherent tumor from the region of the hypothalamus at operation. Large tumor size, young age, and severe hydrocephalus were predictors of tumor recurrence, whereas complete tumor resection (as determined by postoperative neuroimaging) and radiotherapy given electively after subtotal excision were less likely to be associated with recurrent disease. Based on these findings, the authors propose an individualized, more flexible treatment approach whereby surgical strategies may be modified to provide long-term tumor control with the lowest morbidity.

SUMMARY A growth chart for girls with Turner syndrome has been prepared using data from four published series of European patients, and evaluated using retrospective data on the heights of girls with Turner syndrome seen at this hospital. The results indicate that calculation of height standard deviation score from this chart allows a reasonable prediction of adult stature in any patient with Turner syndrome. In addition, the results indicate that while oestrogen treatment causes an initial acceleration of growth, it has no significant effect on adult height.

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Review of 20 patients with glucocorticoid deficiency (three cases also with salt loss) associated with absent tear secretion (19 cases) and sensory neuropathy have also been described,89 together with ataxia, optic atrophy and impaired intelligence, "I indicating that the disorder has a wide spectrum of clinical manifestations. This paper describes the findings in a multicentre collaborative study carried out to ascertain the incidence of disordered neurological and automatic funciton in the syndrome. MethodsIn order to obtain information on patients with the adrenal insufficiency/alacrima/achalasia syndrome in the UK, questionnaires were sent to 39 senior members of the British Society for Paediatric Endocrinology asking for information on any cases under their care. Thirty seven questionnaires were returned, and six cases with the disorder were identified in the UK (cases 4, 5, 6, 13, 15, 17 described below).To increase the number of patients available for study, members of the European Society for Paediatric Endocrinology known to have had patients with the disorder under their care were invited to participate in the study and were sent questionnaires asking for information on the presence of adrenal insufficiency, alacrima, achalasia of the cardia, abnormal neurological findings, tests of autonomic function, evidence of intellectual impairment, skin manifestations, and family history.In some families siblings have died in early infancy, probably as a result of adrenal insufficiency. These have not been included in the study as little information is available on their neurological status.

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Diabetes insipidus is a well-recognized complication of Langerhans-cell histiocytosis (histiocytosis X), but its frequency and natural history are not well defined. Of 52 children with histiocytosis whom we studied, 12 (23 percent) had diabetes insipidus. Only two children had diabetes insipidus at presentation with histiocytosis, but the cumulative risk that it would develop during the first four years after the presentation and diagnosis of histiocytosis was found to be 42 percent. Diabetes insipidus occurred most often among children with multisystem disease and those with proptosis. To determine the natural history of diabetes insipidus in children with histiocytosis, we measured the response of urinary arginine vasopressin to water deprivation every six months in 21 children who did not have diabetes insipidus and who had had histiocytosis for less than four years. Five of the 21 children (24 percent) had subnormal responses during the initial test. One subsequently had spontaneous improvement in the functioning of the posterior pituitary, and diabetes insipidus subsequently developed in two, as it did in one of the children who initially had normal function of the posterior pituitary. Two of the children received irradiation to the pituitary within two to four weeks after diabetes insipidus developed, but they had no improvement in pituitary function. However, diabetes insipidus improved transiently during prednisolone therapy in one of these children and improved permanently after etoposide therapy in another child. We conclude that prospective study with the use of a simple water-deprivation test will allow partial defects of posterior-pituitary function to be detected in patients with histiocytosis and will permit a more appropriate evaluation of the effects of therapeutic intervention.

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