Endocrinology and diabetes.(CHILDHOOD CRANIOPHARYNGIOMA: MORBIDITY AND MORTALITY IN MANCHESTER OVER THE LAST 20 YEARS)(GROWTH HORMONE (GH) PROVOCATION TESTS AND THE RESPONSE TO GH THERAPY IN CHILDREN WITH GH DEFICIENCY)(IMPROVED FINAL HEIGHT IN TURNER'S SYNDROME FOLLOWING GROWTH PROMOTING TREATMENT AT A SINGLE CENTRE)(COGNITIVE FUNCTION IN CHILDREN WITH CONGENITAL HYPOPITUITARISM)(PITUITARY RADIOTHERAPY: AN EFFECTIVE SECOND LINE TREATMENT FOR PAEDIATRIC CUSHING'S DISEASE)(IS THE GNRH TEST A PREDICTOR OF NEUROLOGICAL LESION IN CENTRAL PRECOCIOUS PUBERTY IN GIRLS?)(FAMILIAL DYSALBUMINAEMIC HYPERTHYROXINAEMIA (FDH), A THYROID TRAP!)(DRIED BLOOD SPOTS TO SCREEN THYROID FUNCTION IN CHILDREN WITH DIABETES)(INSULIN LISPRO: ITS ROLE IN REDUCING NOCTURNAL HYPOGLYCAEMIA IN YOUNG CHILDREN WITH T1DM)(INFLUENCE OF SIZE AT BIRTH AND POSTNATAL CATCH-UP GROWTH ON FASTING INSULIN LEVELS AND POST-ORAL GLUCOSE INSULIN RESPONSE IN 8YR OLD CHILDREN)(FIVE YEAR-OLD GIRLS ARE INTRINSICALLY MORE INSULIN RESISTANT THAN BOYS: THE EARLYBIRD STUDY)(SURVEY OF NON-TYPE 1 DIABETES SHOWS UK TYPE 2 DIABETES PREVALENCE 0.4% DISTINCT FROM MODY, AND ASSOCIATED WITH OVERWEIGHT, PUBERTY, FEMALE SEX AND ETHNIC MINORITY STATUS)(MOLECULAR GENETICS OF CONGENITAL ADRENAL HYPERPLASIA (21 HYDROXYLASE DEFICIENCY): IMPLICATIONS FOR BEHAVIOURAL MASCULINISATION IN GIRLS)(Abstract)

[G1] CHILDHOOD CRANIOPHARYNGIOMA: MORBIDITY AND MORTALITY IN MANCHESTER OVER THE LAST 20 YEARS

D.A. lies (1), H.R. Gattamaneni (2), J. Leggate (1), J. Thorne (1), C. West (1), D.A. Price (1), P.E. Clayton', C.M. Hall'.

Background: Childhood craniopharyngioma (CC) is a locally aggressive tumour with significant associated morbidity and the goals of treatment include removal of the tumour, prevention of recurrence, adequate hormonal replacement and minimisation of damage to the hypothalamus and optic tracts. Management strategies vary: neurosurgery may be partial (PS) or complete (C) and postoperative radiotherapy (XRT) may follow PS or be used in the event of tumour recurrence (TR).

Aims: To assess the morbidity and mortality associated with different treatment modalities.

Methods: The casenotes of 25 children diagnosed with CC in Manchester over the lost 20 years were reviewed. Comparisons were made between treatment groups: (A) PS and immediate XRT (n= 10) versus CS and no XRT (n=5), (B) PS and no XRT (n=7) versus CS and no XRT (n=5) and (C) XRT (n=12) versus surgery alone (n=13). Neurosurgical information was incomplete in 3 subjects. RESULTS: The mean age (sd) at diagnosis was 7.7 (4.5) years and the mean length of follow up was 8.2 (4.3) years. 2 patients died: neither had XRT and both had multiple operations. Treatment modality did not significantly affect the following postoperative markers of morbidity: endocrinopathy (hypopituitarism in 24 of 25), change in BMISDS (0.5 [1 .5]), change in height SDS (0.7 [1.3]), visual impairment (present in 17 of 25) or hypothalamic morbidity (recorded in 16 of 25). Treatment modality significantly affected TR: In group (A) tumour recurred in 3 of 5 patients treated with CS alone compared with 0 of 10 treated with PS and XRT (p=0.02). In group (B), there was no significant difference in TR between PS alone (3 of 7) compared to CS alone (3 of 5). In group (C), TR was significantly lower in XRT (0 of 12) versus no XRT (7 of 13) (p=0.005).

Conclusions: Our data indicate that immediate postoperative XRT prevents tumour recurrence and suggest that treatment modality does not affect morbidity. However long term prospective neuropsychological follow up will be necessary to confirm these findings.

(1.) Royal Manchester Children's Hospital

(2.) The Christie Hospital, Mancheste

[G2] GROWTH HORMONE (GH) PROVOCATION TESTS AND THE RESPONSE TO GH THERAPY IN CHILDREN WITH GH DEFICIENCY

T.J.Cole, D.B. Dunger. Centre for Paediatric Epidemiology & Biostatistics, Institute of Child Health, London WC1N 1EH; Department of Paediatrics, University of Cambridge, Addenbrookes Hospital, Cambridge CB2 2QQ

Aims: to identify factors, particularly the response to the GH provocation test, that affect the growth response to GH therapy in children with growth hormone deficiency (GHD).

Methods: data for 513 (72% male) patients with a diagnosis of GHD were obtained from the Pharmacia KIGS UK database (inclusion criteria: a response of <20 mU/I to a GH provocation test, prepubertal and <10 years old at diagnosis, and 1+ years of GH therapy). Growth response was defined as the change in height SDS (revised UK reference) in each of the first 2 years of treatment, and was adjusted for height SDS 1 year earlier and other significant covariates; GH provocation test response, GH dose and frequency, age, weight SDS, birth weight, gestation, and midparent height. Significant covariates were identified using backwards stepwise multiple regression.

Results: mean height SDS at baseline was -3.4 (SD 0.9) at mean age 7.8 (SD 1.3) years. Growth response in the 1st (2nd) year was +0.72 (+0.37) SDS units. Nine factors predicted 1 year response (N = 316), in particular GH provocation test response (t = -7.5), previous height SDS (t = -5.8), midparent height (t = 4.7), GM frequency (t = 4.2) and age (t = -3.3). They explained 42% of variance. In contrast only 3 factors predicted 2nd year response (N = 246), primarily GH provocation test response (t = -3.8), explaining 7% of variance. 1" year growth response, added to the 2nd year growth response model, was not only highly significant (t = 6.8) but it also rendered insignificant the GH provocation test respanse (t = 0.7). This extended made) explained 22% of variance.

Conclusions: the GH provocation …