Langerhans cell histiocytosis (LCH) is a rare disorder in which granulomatous deposits occur at multiple sites within the body, but which often involves the hypothalamo-pituitary axis (HPA). Although ...diabetes insipidus (DI) is a well recognized complication, the frequency of anterior pituitary and other nonendocrine hypothalamic (NEH) involvement has not been well defined, particularly in adult patients with the disease. We have evaluated the frequency and progression of LCH-related anterior pituitary and other NEH dysfunction and their responses to treatment in 12 adult patients with histologically proven LCH and DI. They were followed up for a median of 11.5 yr (range, 3-28 yr) after the diagnosis of DI was made. Study evaluations comprised clinical (including formal psychometric assessment where appropriate), basal and dynamic pituitary function tests, and radiology with computed tomography and/or magnetic resonance imaging scanning. Eleven patients received systemic treatment, and 5 patients received external beam radiotherapy confined to the HPA. The median age at diagnosis of DI was 34 yr (range, 2-47 yr); DI was the presenting symptom in four patients, whereas the remaining eight each developed DI 1-20 yr (median, 2 yr) after the diagnosis of LCH. Eight patients developed one or more anterior pituitary hormonal deficiencies at a median of 4.5 yr (range, 2-22 yr) after the diagnosis of DI: GH deficiency developed in eight patients (median, 2 yr; range, 2-22 yr), FSH-LH deficiency in 7 patients (median, 7 yr; range, 2-22 yr), and TSH and ACTH deficiency in five patients (median, 10 yr; range, 3-16 and 3-19 yr), respectively; five patients developed panhypopituitarism. In addition, seven patients with anterior pituitary dysfunction also developed symptoms of other NEH dysfunctions at a median of 10 yr (range, 1-23 yr): five morbid obesity (body mass index, >35), five short term memory deficits, four sleeping disorders, two disorders of thermoregulation, and one adipsia. All patients developed disease outside of the hypothalamus during the course of the study, and no fluctuation of disease activity in the HPA region was noted. Radiological examination of the HPA was abnormal in each of the eight patients with anterior pituitary involvement and in the seven patients with NEH dysfunction (one or more abnormalities): seven had thickening of the infundibulum, and one had hypothalamic and thalamic signal changes. All patients who had a magnetic resonance imaging scan had absence of the bright spot of the posterior pituitary on the T1-weighted sequences, and in four patients with DI and normal anterior pituitary function this was the only abnormality. The five patients who received radiotherapy to the HPA achieved a partial or complete radiological response, and there was no evidence of tumor progression in this region. No form of therapy, including chemotherapy, improved any established hormonal deficiencies or symptoms of NEH. In summary, in our adult patients with hypothalamic LCH and DI, anterior pituitary hormonal deficiencies developed in 8 of 12 patients; these occurred over the course of 20 yr. They were frequently accompanied by structural changes of the HPA, although these were often subtle in nature. In addition, symptoms of NEH dysfunction developed in up to 90% of such patients and complicated management. Radiotherapy may be useful in achieving local control of tumor, but established anterior, posterior pituitary, and other NEH dysfunctions do not improve in response to current treatment protocols. Patients with LCH and DI, particularly those with multisystem disease and a structural lesion on radiology, should undergo regular and prolonged endocrine assessment to establish anterior pituitary deficiency and provide appropriate hormonal replacement.
Objectives To estimate global, regional (21 regions) and national (187 countries) sodium intakes in adults in 1990 and 2010. Design Bayesian hierarchical modelling using all identifiable primary ...sources. Data sources and eligibility We searched and obtained published and unpublished data from 142 surveys of 24 h urinary sodium and 103 of dietary sodium conducted between 1980 and 2010 across 66 countries. Dietary estimates were converted to urine equivalents based on 79 pairs of dual measurements. Modelling methods Bayesian hierarchical modelling used survey data and their characteristics to estimate mean sodium intake, by sex, 5 years age group and associated uncertainty for persons aged 20+ in 187 countries in 1990 and 2010. Country-level covariates were national income/person and composition of food supplies. Main outcome measures Mean sodium intake (g/day) as estimable by 24 h urine collections, without adjustment for non-urinary losses. Results In 2010, global mean sodium intake was 3.95 g/day (95% uncertainty interval: 3.89 to 4.01). This was nearly twice the WHO recommended limit of 2 g/day and equivalent to 10.06 (9.88–10.21) g/day of salt. Intake in men was ∼10% higher than in women; differences by age were small. Intakes were highest in East Asia, Central Asia and Eastern Europe (mean >4.2 g/day) and in Central Europe and Middle East/North Africa (3.9–4.2 g/day). Regional mean intakes in North America, Western Europe and Australia/New Zealand ranged from 3.4 to 3.8 g/day. Intakes were lower (<3.3 g/day), but more uncertain, in sub-Saharan Africa and Latin America. Between 1990 and 2010, modest, but uncertain, increases in sodium intakes were identified. Conclusions Sodium intakes exceed the recommended levels in almost all countries with small differences by age and sex. Virtually all populations would benefit from sodium reduction, supported by enhanced surveillance.
Authors from London looked at patients diagnosed with germ cell tumours of the testis who presented between 1983 and 2002. They found that there was an overall increase in the percentage of patients ...presenting with stage I seminoma, with a significant reduction in the size of the primary tumour. The authors do not feel that they can clarify the reason for these changes.
There is a 20‐year follow‐up of patients who had tumours of the ureter and renal pelvis treated with resection and renal autotransplantation. In this fascinating series from Gothenberg, it was found that in patients with a normal contralateral kidney, resection and autotransplantation was not indicated. It was possibly beneficial in patients with solitary kidneys, but only after other treatments have been considered.
OBJECTIVE
To prospectively investigate the presentation of germ cell tumours (GCTs) of the testis in terms of stage or histology, as the incidence of this disease in increasing.
PATIENTS AND METHODS
Patients diagnosed with GCT of the testis between 1983 and 2002 were categorised into three periods depending on the date of diagnosis of the GCT, and the presentational characteristics assessed.
RESULTS
There was a significant increase in the proportion of patients presenting with stage I disease (59% to 78%) and seminoma (43% to 58%) over this period. There was also a significant reduction in the size of the primary tumour (5 to 4 cm).
CONCLUSION
A greater proportion of patients with GCT are presenting with stage I seminoma, the reasons for which are unclear, although earlier diagnosis through improved awareness of GCT may be important.
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