[W.D.H.A. Syndrome due to occult neuroendocrine malignancy with concomitant liver metastases]. / Okkulte, maligne neuroendokrine Neoplasie mit Lebermetastasen als Ursache eines WDHA-Syndroms.

In June 1999, a 62-year-old man is hospitalised to evaluate the sonographic suspicion of liver metastases. The biopsy of the liver shows a malignant neuroendocrine tumour. Further diagnostic investigation including gastroscopy, colonoscopy, enteroclysis, thoracal and abdominal CT and somatostatin-receptor-scintigraphy does not localise the primary tumour. In the absence of clinical symptoms a wait and see procedure with clinical and imaging controls at regular intervals is arranged. Beginning in spring of 2001--nearly two years after the initial diagnosis--the patient suffers from progredient diarrhoea and weight loss leading to hospitalisation in September 2001. The existence of secretory diarrhoea, hypokalaemia and hypercalcaemia arouses suspicion of vipoma. This is proven by a remarkably elevated plasma concentration of vasoactive intestinal peptide (VIP). Once more, an accurate investigation is started but no primary tumour can be discovered despite extensive liver metastases. A vipoma is a rare differential diagnosis of secretory diarrhoea. This case report describes the remarkable constellation of liver metastases of a malignant neuroendocrine neoplasm without a primary tumour and the clinical presentation of a W.D.H.A. syndrome (watery diarrhoea, hypokalaemia and hypo- or achlorhydria). Despite extensive disease, therapy with octreotide and prednisolone provides a good clinical response.

The incidence of hyperthyroidism in Austria from 1987 to 1995 before and after an increase in salt iodization in 1990.

Between 1963 and 1990, Austria had iodized salt prophylaxis of endemic goitre with 10 mg KI (7.5 mg I) per kg. This was obviously insufficient, as urinary iodine excretion ranged from 42 to 78 microg I per g of creatinine and goitre in adults remained in the endemic range of 15%-30%. Therefore salt iodization was doubled in 1990. The aim of this study was to assess the annual incidence of different types of hyperthyroidism (HT) before and after this increase in salt iodization. The incidence of HT was recorded in 14 nuclear medicine centres from 1987 to 1995. In five additional centres data were available from 1992 onwards. Data prior to 1992 were documented retrospectively, while those after 1992 were recorded prospectively. The 14 centres drew patients from an area with a population of approximately 4.23 million while all 19 institutes were estimated to cover an area with a population of 5.4 million (the total population of Austria is 7.86 million). A total of 414232 persons were examined for the first time in the participating centres. HT and the type of HT were defined by clinical examination, serum TSH, thyroid hormone levels in blood, ultrasonography, scintigraphy and serum autoantibody titres. HT was classified into immunogenic HT (Graves' or Basedow's disease, GD) and HT with intrinsic thyroid autonomy (uni-, multinodular or disseminated Plummers' disease, PD). HT was also divided into overt (o) or subclinical (sc) disease. The following data were calculated: annual incidence per 100000 and the relative risk (RR) for HT with 95% confidence intervals (CI). In addition, linear trends were calculated for each type of HT by means of logistic regressions. In the 19 centres a total of 47834 patients with HT were registered from 1987 to 1995. PD accounted for 75% of all cases of HT and GD for 19%, while other types of HT were present in 6%. From 1987 to 1989 (time period T0), the annual incidence of oPD was 30.5 (95% CI 29.6-31.5) per 100000. The RR compared to the baseline period T0 was highest in 1992 (1.37; 1.3-1.45) and decreased to 1.17 (1.1-1.24) in 1995. The annual incidence of scPD in T0 was 27.4 (26.5-28.3) per 100000. The RR was highest in 1991 (1.64; 1.56-1.73) and was 1.60 (1. 51-1.69) in 1995. In oPD and scPD a higher RR was observed in persons older than 50 years of age, particularly in men. The incidence of oGD in T0 was 10.4 (9.8-10.9) per 100000; the maximum RR increased to 2.19 (2.01-2.38) in 1993 and decreased to 1.95 (1.78-2.13) in 1995. The incidence of scGD was 1.9 (1.6-2.1) in T0. The maximum RR was observed in 1994 (2.47; 2.04-3.0) and it was still 2.26 (1.85-2.77) in 1995. The increased incidence of oGD and scGD was evenly distributed in all ages and both sexes. The time course of different types of HT following the increase in salt iodization could be divided into two phases: an increase in the incidences of HT with peaks after 1-4 years and a subsequent decrease, the only exception being scGD. The effect was more pronounced in GD than in PD. PD showed an age and gender dependency over time, while GD did not.

[Behavior of angiotensin converting enzyme in diseases of the thyroid]. / Das Verhalten des Angiotensin-Converting-Enzyms bei Erkrankungen der Schilddrüse.

Serum ACE activity was examined in cases of diseases of the thyroid gland. The enzyme activity was compared with the concentration of total serum thyroxine (TT4) and total serum triiodothyronine (TT3). For this purpose the medical data of a group of healthy test persons (85 male and 85 female) were contrasted with those of a group of 162 patients attending the out-patient department for thyroid gland diseases in our hospital (28 male and 134 female, of whom 112 were aged between 21 and 60, and 50 above the age of 60). Among the patients there were 36 cases of euthyroid goiter, 59 of untreated and 36 of treated hyperthyroidism, 25 of hypothyroidism of varied genesis, and 6 patients suffering from as yet untreated thyroid cancer. We observed significant differences in ACE activity in the different groups. In cases of disorder of the thyroid gland there was a positive correlation between enzyme activity and hormone data. Where other causes which may influence its activity can be excluded, ACE reflects the effect of the hormones of the thyroid gland on the tissue. We kept under observation 15 patients suffering from thyroid cancer altogether, of whom 6 had no previous treatment, whereas in 9 thyroidectomy had been carried out, followed by radioactive iodine therapy. Irrespective of the timing of the examination, there was a significant increase in serum ACE activity (on average 365 U/l, as against 282 U/l, p less than 0.01), if metastasis had occurred.