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Exercise as a test of growth hormone secretion

J. M. PARKIN

My interest in children with growth problems was stimulated by hearing the 1964 Windermere Lecture given to the British Paediatric Association (Prader et al). Since these early exciting studies of growth hormone treatment there has been debate about the best way of identifying patients who might benefit from it. When the only preparations available were derived from human pituitaries, and supply was limited, the opinion was readily accepted that only patients with very low serum levels after pharmacological stress should be treated. The demonstration of a serum growth hormone of more than 15 mU/1 after any stress was accepted as an indication that treatment was not indicated.

Recently the situation has changed. Biosynthetic growth hormone is available and, though expensive, in potentially limitless quantities. In addition, there is evidence that many more children than originally thought may be helped by the hormone (Zadik et al 1985). Indeed there may be

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Pituitary adenomas in childhood

S.M. SHALET

Pituitary adenomas are uncommon in childhood (Zimmerman, 1969). The majority of pituitary adenomas are benign hormonally active tumours arising from the 5 hormonal secreting cell types of the adenohypophysis (Asa and Kovacs, 1983). With the availability of modern immunohistochemical techniques it is apparent that only 25% of pituitary adenomas are non-functioning.

In paediatric practice the important hormonally active adenomas are GH (gigantism), prolactin and ACTH (Cushing's disease). Both functional and non-functional pituitary tumours may be associated with hypopituitarism resulting from compression of the normal pituitary tissue or from tumour interfering with transport and synthesis of hypothalamic releasing hormones. This may lead to impaired growth, hypothyroidism, hypoadrenalism and failure of normal pubertal development.

Gigantism

The excessive secretion of GH before fusion of the epiphyses leads to gigantism. After fusion it produces the typical features of acromegaly. In nearly all cases, gigantism results from a pituitary tumour. Some tumours are mixed and

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Factors influencing cure by transsphenoidal selective adenomectomy in paediatric Cushing’s disease

Helen L Storr, Farhad Afshar, Matthew Matson, Ian Sabin, Kate M Davies, Jane Evanson, P Nicholas Plowman, G Michael Besser, John P Monson, Ashley B Grossman, and Martin O Savage

Objective: Early diagnosis and effective treatment of paediatric Cushing’s disease (CD) is necessary to minimise associated morbidity. Accepted first-line treatment is selective transsphenoidal microadenomectomy (TSS), which can be technically difficult, and cure rates vary considerably between centres. In our paediatric CD patient group we have assessed the possible factors which may influence cure by TSS.

Subjects and methods: From 1983–2004, 27 paediatric patients (16 males, 11 females; mean age±s.d., 13.1±3.2 yr; range, 6.4–17.8 yr) with CD were managed in our centre and underwent TSS. Sixteen patients (59%), seven males and nine females (mean age±s.d., 14.2±2.5 yr; range, 8.2–17.8 yr), were cured (post-operative serum cortisol < 50 nM). Eleven patients, nine males and two females (mean age±s.d., 11.5±3.6 yr; range, 6.4–17.8 yr) had post-operative cortisol levels above 50 nM (2–20 days), with mean serum cortisol levels at 09:00 h of 537 nM (range 269–900 nM) indicating a lack of cure. These 11 patients received external beam pituitary radiotherapy (RT). One patient with a pituitary macroadenoma had a post-operative cortisol level of < 50 nM but 0.8 yr later showed an elevated cortisol and residual disease.

Results: The patients cured by TSS alone were significantly older than those not cured (P = 0.038; Student’s t test). All patients had CT/MRI pituitary imaging: 14 were reported to have microadenomas and one macroadenoma, while 12 were reported as normal. Bilateral simultaneous inferior petrosal sinus sampling (BSIPSS) with i.v. corticotropin-releasing hormone (CRH) administration was introduced as a pre-operative investigation in 1986 and was performed in 21 patients (78%), on BSIPSS, 16 (76%) had evidence suggesting pituitary adrenocorticotropic hormone (ACTH) secretion (central to peripheral (IPS:P) ACTH ratio after CRH of ≥ 3.0) and 16 (76%) showed lateralisation of ACTH secretion (IPSG of ≥ 1.4). There was concordance between the BSIPSS finding and the position of the microadenoma at surgery in 17/21 (81%) patients. Of the 16 patients showing lateralisation of ACTH secretion, 12 (75%) were cured by TSS. Of the four without lateralisation of ACTH, suggesting a midline lesion, 3 (75%) were cured by TSS. Post-operative pituitary hormone deficiencies in the patients cured by TSS were: pan-hypopituitarism 1/16, isolated growth hormone deficiency (GHD) (peak GH on glucagon/ITT < 1–17.9 mU/l) 9/16 and diabetes insipidus 3/16.

Conclusion: Over a 21-year period selective adenomectomy by TSS cured 59% of all paediatric CD patients, with higher age favouring cure. Introduction of BSIPSS resulted in the demonstration of a high rate of lateralisation of ACTH secretion consistent with the surgical identification of the adenoma, and therefore appears likely to have contributed to the higher surgical cure rate.

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German Endocrine Society

Wolfgang Wuttke

The German Endocrine Society (Deutsche Gesellschaft fur Endocrinologie; DGE) was founded in 1953 and currently has more than 1300 members. They are engaged in research, teaching or are practising endocrinologists. About half of the members are medically qualified and the others are biologists, biochemists or agricultural scientists. This multidisciplinary membership fosters the exchange and integration of ideas, and this is one of the particular strengths of the DGE.

The DGE is a member of the European Federation of Endocrine Societies (EFES) and also of the International Society of Endocrinology.

The Society is organized into sections that cover the major topics in endocrinology: Molecular and Cellular Endocrinology; Calcium Regulating Hormones and Bone Metabolism; Thyroid; and Paediatric and Applied Endocrinology. Sections of Gynaecological Endocrinology and Reproductive Medicine and Neuroendocrinology are currently being formed. Another important activity of the Society is the Commission for Hormone Toxicology, which consists of experts who advise on

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Thyrotoxicosis in childhood

Hans Perrild and Bendt Brock Jacobsen

Thyrotoxicosis during the first two decades of life is rare compared to that in adults, with an incidence of 0.8 per 100000 children per year (1). This limits the experience in the paediatric clinics, and adult treatment strategies are often applied to the thyrotoxic child.

About 95% of thyrotoxic children have Graves' disease (1, 2), in contrast to findings in adults where a significant number of toxic adenomas and multinodular toxic goitres are seen, especially in areas with a relatively low iodine intake (3). It is suggested that Graves' disease in childhood is a self-limiting disorder with a remission rate of 25% every 2 years (4). These observations have to be considered in the choice of therapy.

There are three principal treatments today: antithyroid drugs (ATD), surgery or radioiodine. It is well known that the primary management of thyrotoxicosis in adults is different in Europe, Japan and the USA (5).

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Type 2 diabetes in children in the Netherlands: the need for diagnostic protocols

Joost Rotteveel, Eline J Belksma, Carry M Renders, Remy A Hirasing, and Henriette A Delemarre-Van de Waal

Objective: The worldwide trend towards obesity in childhood is also observed in the Netherlands and one of the consequences may be type 2 diabetes. In this study, we assessed the number of children with type 2 diabetes, diagnosed by paediatricians, in the Netherlands.

Methods: In 2003 and 2004 the Dutch Paediatric Surveillance Unit, a nationwide paediatric register, was used to assess new cases of diabetes mellitus. Data on socio-demographic and clinical characteristics were collected by means of a questionnaire. A second questionnaire was sent to the reporting paediatrician if the diagnosis was inconclusive or if the diagnosis was type 1 diabetes in combination with overweight or obesity, according to international criteria.

Results: During the 24 months of registration, the paediatricians reported 1142 new cases of diabetes, 943 of which were eligible for analysis. Initially, 14 patients (1.5%) were reported with type 2 diabetes. Only seven of these patients were classified as type 2 diabetes according to the ADA criteria, as information on C-peptides or antibodies was often missing. Based on clinical characteristics, the other seven patients were very likely to have type 2 diabetes. After the second questionnaire, six more patients met the ADA criteria and two were very likely to have type 2 diabetes. Most of the patients were female (95%), 14% were of Turkish and 18% of Moroccan origin.

Conclusion: This study shows a discrepancy between the number of patients with type 2 diabetes diagnosed by paediatricians in daily practice and diagnosed according to the ADA criteria. Moreover, a considerable amount of reported patients were misclassified. Finally, 2.4% patients were classified as (very likely) type 2 diabetes. The development of programmes and protocols for prevention, diagnosis and classification applicable in daily practice is warranted.

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Comorbidity and cardiovascular risk factors in adult GH deficiency following treatment for Cushing's disease or non-functioning pituitary adenomas during childhood

Oskar Ragnarsson, Charlotte Höybye, Peter J Jönsson, Ulla Feldt-Rasmussen, Gudmundur Johannsson, Beverly M K Biller, and Maria Kołtowska-Häggström

Objective

Cushing's disease (CD) and non-functioning pituitary adenoma (NFPA) are rare in paediatric patients. The aim of this study was to describe long-term consequences in adults with GH deficiency (GHD) treated for CD or NFPA during childhood.

Design, patients and methods

This was a retrospective analysis of data from KIMS (Pfizer International Metabolic Database). Background characteristics, anthropometry and comorbidity were studied in 47 patients diagnosed with childhood-onset (CO)-CD and 62 patients with CO-NFPA. Data from 100 ACTH-sufficient patients with CO-idiopathic hypopituitarism (CO-Idio) were used for comparison. Cardiovascular risk profile was analysed at baseline and at 1 year on GH treatment in a subgroup of patients (17 CO-CD, 24 CO-NFPA and 55 CO-Idio) not receiving GH treatment at study entry.

Results

The median age at diagnosis of pituitary tumour was 14.0 years (range 10–17) in patients with CO-CD and 13.7 years (range 8–17) in CO-NFPA. In addition to GHD, 41% of patients with CO-CD had three or four other pituitary hormone deficiencies compared with 78% of patients with CO-NFPA (P<0.001). Eighty-nine per cent of patients with CO-CD had height SDS lower than 0 compared with 61% of patients with CO-NFPA (P=0.002). Hypertension was more common in CO-CD compared with CO-Idio (23 vs 9%, P=0.018). At 1 year on GH treatment, total- and low-density lipoprotein-cholesterol decreased significantly in CO-CD but not in CO-NFPA.

Conclusion

Adult patients with GHD following treatment for paediatric CD and NFPA have long-term adverse consequences. Despite more severe hypopituitarism in CO-NFPA, patients with CO-CD have more frequently compromised final stature.

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The neuroendocrine sequelae of paediatric craniopharyngioma: a 40-year meta-data analysis of 185 cases from three UK centres

Timothy Shao Ern Tan, Leena Patel, Jaya Sujatha Gopal-Kothandapani, Sarah Ehtisham, Esieza Clare Ikazoboh, Richard Hayward, Kristian Aquilina, Mars Skae, Nicky Thorp, Barry Pizer, Mohammed Didi, Conor Mallucci, Joanne C Blair, Mark N Gaze, Ian Kamaly-Asl, Helen Spoudeas, and Peter E Clayton

Objectives

The management of paediatric craniopharyngiomas was traditionally complete resection (CR), with better reported tumour control compared to that by partial resection (PR) or limited surgery (LS). The subsequent shift towards hypothalamic sparing, conservative surgery with adjuvant radiotherapy (RT) to any residual tumour aimed at reducing neuroendocrine morbidity, has not been systematically studied. Hence, we reviewed the sequelae of differing management strategies in paediatric craniopharyngioma across three UK tertiary centres over four decades.

Methods

Meta-data was retrospectively reviewed over two periods before (1973–2000 (Group A: n = 100)) and after (1998–2011 (Group B: n = 85)) the introduction of the conservative strategy at each centre.

Results

Patients had CR (A: 34% and B: 19%), PR (A: 48% and B: 46%) or LS (A: 16% and B: 34%), with trends reflecting the change in surgical approach over time. Overall recurrence rates between the two periods did not change (A: 38% vs B: 32%). More patients received RT in B than A, but recurrence rates were similar: for A, 28% patients received RT with 9 recurrences (32%); for B, 62% received RT with 14 recurrences (26%). However, rates of diabetes insipidus (P = 0.04), gonadotrophin deficiency (P < 0.001) and panhypopituitarism (P = 0.001) were lower in B than those in A. In contrast, post-operative obesity (BMI SDS >+2.0) (P = 0.4) and hypothalamic (P = 0.1) and visual (P = 0.3) morbidity rates were unchanged.

Conclusion

The shift towards more conservative surgery has reduced the prevalence of hormone deficiencies, including diabetes insipidus, which can be life threatening. However, it has not been associated with reduced hypothalamic and visual morbidities, which remain a significant challenge. More effective targeted therapies are necessary to improve outcomes.

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NEUE METHODISCHE MÖGLICHKEITEN ZUR KLINISCHEN FUNKTIONSDIAGNOSTIK DER NEBENNIERENRINDE

H. Gerdes and W. Teller

ABSTRACT

The results are reported of routine determinations of free C21-steroids in plasma and urine by a method which involved thin layer and gas liquid chromatography. The following steroids were simultaneously determined: Cortisol, cortisone, 6β-hydroxycortisol and -cortisone, 20-hydroxycortisol and -cortisone, tetrahydrocortisol and -cortisone, and 11-deoxycortisol (Reichstein S). The specificity, reproducibility and accuracy are discussed in detail. The overall recoveries of the procedure were examined by the addition of 1,2-3H-cortisol and 1,2-3H-cortisone prior to extraction. They ranged between 50 and 60 per cent.

The method was clinically applied to various tests of adrenal cortical function and its pituitary regulation (e. g. ACTH test, dexamethasone suppression, metopirone test). The simultaneous determination in plasma and urine of a number of C21-steroid-hormones including their metabolites proved to yield valuable information about adrenal cortical function and C21-steroid metabolism. In the paediatric age group the metopirone test measuring 11-deoxycortisol in plasma rather than its tetrahydro derivative in urine was particularly useful since it avoided unpleasant 24 h-urine collections in young children.

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Management of children with idiopathic short stature

Leo Dunkel

The Food and Drug Administration (FDA) approved the use of biosynthetic GH for the treatment of children with idiopathic short stature (ISS) in the US in 2003. Primarily, the decision was based on two studies: a randomized placebo-controlled study and a dose–response study, both demonstrating an increase in adult height over the predicted height at baseline and over placebo-treated controls by an average of 4–7 cm. Despite these data and FDA approval of GH treatment for ISS, there is still a significant controversy among paediatric endocrinologists about how, and to what extent, GH should be used in this indication. GH is clearly efficacious in several growth disorders and has the potential to alleviate debilitating short stature. However, it has been questioned whether ISS should be considered a condition warranting pharmacological treatment, whether the degree of morbidity of untreated ISS is clinically significant, and whether improved psychosocial status or well-being is achieved through GH treatment and height gain. The benefits must outweigh treatment costs and risks to justify GH treatment in ISS. The safety of GH treatment in ISS has been the main subject in two recent articles from pharmaceutical companies that conducted the pioneering studies mentioned earlier. No new safety concerns were observed in the ISS populations, but there were some limitations in study designs that prevent clinicians, their patients and families from ‘resting assured’. Studies addressing these controversial issues are needed before the widespread use of GH treatment in ISS is warranted.