24 research outputs found
In Vitro Recovery of ATP-Sensitive Potassium Channels in Ī²-Cells From Patients With Congenital Hyperinsulinism of Infancy
Gonadectomy in conditions affecting sex development: a registry-based cohort study
Objectives
To determine trends in clinical practice for individuals with DSD requiring gonadectomy.
Design
Retrospective cohort study.
Methods
Information regarding age at gonadectomy according to diagnosis; reported sex; time of presentation to specialist centre; and location of centre from cases reported to the International DSD Registry and who were over 16 years old in January 2019.
Results
Data regarding gonadectomy were available in 668 (88%) individuals from 44 centres. Of these, 248 (37%) (median age (range) 24 (17, 75) years) were male and 420 (63%) (median age (range) 26 (16, 86) years) were female. Gonadectomy was reported from 36 centres in 351/668 cases (53%). Females were more likely to undergo gonadectomy (nā= 311, Pā< 0.0001). The indication for gonadectomy was reported in 268 (76%). The most common indication was mitigation of tumour risk in 172 (64%). Variations in the practice of gonadectomy were observed; of the 351 cases from 36 centres, 17 (5%) at 9 centres had undergone gonadectomy before their first presentation to the specialist centre. Median age at gonadectomy of cases from high-income countries and low-/middle-income countries (LMIC) was 13.0 years (0.1, 68) years and 16.5 years (1, 28), respectively (Pā< 0.0001) with the likelihood of long-term retention of gonads being higher in LMIC countries.
Conclusions
The likelihood of gonadectomy depends on the underlying diagnosis, sex of rearing and the geographical setting. Clinical benchmarks, which can be studied across all forms of DSD will allow a better understanding of the variation in the practice of gonadectomy
Essay Review: Hilary Marland. Dangerous Motherhood: Insanity and Childbirth in Victorian Britain. Basingstoke and New York: Palgrave Macmillan, 2004. Pp. xii + 304. Ā£52.50. ISBN 1403920389
Impact of intercurrent illness on calcium homeostasis in children with hypoparathyroidism: a case series
Background: Hypoparathyroidism is characterised by hypocalcaemia, and standard management is with an active vitamin D analogue and adequate oral calcium intake (dietary and/or supplements). Little is described in the literature about the impact of intercurrent illnesses on calcium homeostasis in children with hypoparathyroidism.
Methods: We describe three children with hypoparathyroidism in whom intercurrent illnesses led to hypocalcaemia and escalation of treatment with alfacalcidol (1-hydroxycholecalciferol) and calcium supplements.
Results: Three infants managed with standard treatment for hypoparathyroidism (two with homozygous mutations in GCMB2 gene and one with Sanjad-Sakati syndrome) developed symptomatic hypocalcaemia (two infants developed seizures) following respiratory or gastrointestinal illnesses. Substantial increases in alfacalcidol doses (up to three times their pre-illness doses) and calcium supplementation were required to achieve acceptable serum calcium concentrations. However, following resolution of illness, these children developed an increase in serum calcium and hypercalciuria, necessitating rapid reduction to pre-illness dosages of alfacalcidol and oral calcium supplementation.
Conclusion: Intercurrent illness may precipitate symptomatic hypocalcaemia in children with hypoparathyroidism, necessitating increase in dosages of alfacalcidol and calcium supplements. Close monitoring is required on resolution of the intercurrent illness, with timely reduction of dosages of active analogues of vitamin D and calcium supplements to prevent hypercalcaemia, hypercalciuria and nephrocalcinosis
Death and the dead-house in Victorian asylums: necroscopy versus mourning at the Royal Edinburgh Asylum, c
Organ extracts and the development of psychiatry: Hormonal treatments at the Maudsley hospital 1923-1938
The use of organ extracts to treat psychiatric disorder in the interwar period is an episode in the history of psychiatry which has largely been forgotten. An analysis of case-notes from The Maudsley Hospital from the period 1923ā1938 shows that the prescription of extracts taken from animal testes, ovaries, thyroids, and other organs was widespread within this London Hospital. This article explores the way in which Maudsley doctors justified these treatments by tying together psychological theories of the unconscious with experimental data drawn from laboratory studies of human organs. It explores the logic behind these treatments and examines beliefs about their efficacy. The connection between this historical episode and current research in endocrinology and psychology is explored. C Ā© 2012 Wiley Periodicals, Inc. In 1923, The Maudsley Hospital opened in London, United Kingdom, to pioneer new forms of treatment for mental illness. An ambitious agenda designed to research and treat severe psychiatric illness was drafted in 1907 by the mental scientist Henry Maudsley and Frederick Mott, a lecturer and physician at Charing Cross Hospital and director of the Centra