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Zusammenfassung Hintergrund Trifokale Linsen ergänzen seit wenigen Jahren die Möglichkeiten der chirurgischen Presbyopiekorrektur. In dieser Studie werden 2 Intraokularlinsen (IOL) auf Basis des gleichen trifokalen diffraktiven Designs verglichen, ein Modell ohne und eines mit torischer Komponente zur Astigmatismuskorrektur. Patienten/Material und Methoden Prospektive nicht randomisierte Vergleichsstudie mit 142 Augen von 77 Patienten (Alter 40–73 Jahre) mit komplikationsloser Linsenchirurgie. Die Patienten wurden entsprechend dem implantierten Linsenmodell in 2 Gruppen aufgeteilt, die trifokale Gruppe (98 Augen von 50 Patienten) mit der trifokalen diffraktiven IOL AT LISA tri 839MP (Carl Zeiss Meditec) und die trifokal-torische Gruppe (44 Augen von 27 Patienten) mit Implantation der trifokal-torischen diffraktiven IOL AT LISA tri toric 939MP (Carl Zeiss Meditec). Sehschärfe und Refraktion wurden 3 Monate postoperativ ausgewertet. Ergebnisse Es gab keine signifikanten Unterschiede zwischen den Gruppen in Bezug auf postoperative Refraktion (p ≥ 0,144), monokularen und binokularen unkorrigierten Intermediärvisus (UIVA; p = 0,519 bzw. 0,398) und binokularen unkorrigierten Nahvisus (UNVA; p = 0,073). Dagegen wurden in der trifokalen Gruppe ein signifikant besserer monokularer und binokularer unkorrigierter Fernvisus (UDVA; p ≤ 0,002) und monokularer UNVA (p = 0,005) beobachtet. Das sphärische Äquivalent lag bei 98 bzw. 100 % der Patienten innerhalb von ± 1,00 dpt bei der trifokalen Gruppe und der trifokal-torischen Gruppe. Ein postoperativer binokularer UDVA, UIVA und UNVA von 0,63 dezimal oder besser wurde bei 100, 95 bzw. 100 % der Augen in der trifokalen Gruppe und bei 96,3, 95,8 bzw. 90,9 % der Augen mit dem torischen Linsenmodell beobachtet.

Ziel: Beurteilung der visuellen und refraktiven Ergebnisse 3 Monate nach Implantation einer „Extended-Range-of-Vision“-Intraokularlinse (ERV-IOL) im Rahmen der Kataraktoperation. Methode: In einer prospektiven multizentrischen Studie wurde bei Patienten mit einem kalkulierten postoperativen Astigmatismus ≤ 1,5 dpt im Rahmen der Kataraktoperation eine diffraktive ERV-IOL (TECNIS Symfony, Modell ZXR00, Abbott Medical Optics, USA) implantiert. Drei Monate postoperativ wurden der monokulare und binokulare Nah-, Intermediar- und Fernvisus, unkorrigiert und korrigiert, sowie die Refraktion evaluiert. Ergebnis: 18 Patienten (36 Augen) mit einem mittleren Alter von 63,4 ± 4,6 Jahren wurden im Rahmen der Studie bilateral an einer Katarakt operiert. Drei Monate postoperativ zeigte sich ein binokularer unkorrigierter Fernvisus (UDVA) von logMAR − 0,05 ± 0,11 bei einem korrigierten Fernvisus (CDVA) von logMAR − 0,14 ± 0,05. Der unkorrigierte binokulare Intermediarvisus (DIVA) lag bei logMAR − 0,09 ± 0,02, der binokulare unkorrigierte Nahvisus (UNVA) bei logMAR 0,19 ± 0,09. Eine postoperative Zielrefraktion von ± 0,75 erreichten 89 % der Patienten. Schlussfolgerung: Die Implantation einer ERV-Intraokularlinse bietet eine effektive Wiederherstellung fur das Sehen in der Ferne und im Intermediarbereich. Auch in der Nahe erreichen die Patienten ein funktionelles Sehvermogen.

Hintergrund: Evaluierung der klinischen Ergebnisse 3 Monate nach Implantation einer neuen diffraktiven multifokalen Intraokularlinse (MIOL) mit einer reduzierten Nahaddition von + 2,75 dpt. Methoden: Im Rahmen einer prospektiven Studie wurden Patienten nach erfolgreicher Kataraktoperation oder refraktivem Linsenaustausch mit Implantation einer MIOL (Tecnis ZKB00, Abbott Medical Optics, Santa Ana, California, USA) eingeschlossen. Drei Monate postoperativ wurde die Sehscharfe in unterschiedlichen Testentfernungen bewertet. Bei den postoperativen Untersuchungen nach einer bilateralen IOL-Implantation wurde zusatzlich ein Fragebogen zur Evaluierung der subjektiven Zufriedenheit, der Sehqualitat und der Tragegewohnheiten einer Brille vorgelegt und in die Auswertung mit einbezogen. Ergebnisse: Es wurde zwischen Oktober 2013 und August 2014 bei 115 Augen von 62 Patienten die ZKB00-IOL implantiert. Die postoperative Refraktion lag im spharischen Aquivalent bei − 0,27 ± 0,44 dpt. Der korrigierte binokulare Fernvisus lag bei − 0,01 ± 0,3 logMAR. Der unkorrigierte binokulare Fernvisus wurde mit 0,06 ± 0,08 logMAR nach 3 Monaten ermittelt. Im Nahbereich konnte die unkorrigierte binokulare Sehscharfe mit 0,07 ± 0,10 logMAR nach 3 Monaten postoperativ bestimmt werden. Schlussfolgerung: Die ZKB00-IOL gehort einer neuen Generation von MIOL an, die einen deutlich verbesserten Intermediarvisus anbieten. Unsere Studie belegte fur die Implantation dieser IOL eine ausgezeichnete Sehfunktion in allen Bereichen in Korrelation mit einer sehr hohen allgemeinen Patientenzufriedenheit und einer subjektiv hohen Abbildungsqualitat.

To evaluate the clinical outcome in eyes with significant corneal astigmatism after cataract surgery with implantation of a new diffractive multifocal toric intraocular lens (IOL).Prospective, non-randomised multicentre clinical study including 57 eyes of 38 consecutive patients with an age between 37 and 84 years that underwent cataract surgery with implantation of the toric multifocal IOL Tecnis ZMT (Abbott Medical Optics, Santa Ana, California, USA). Changes in uncorrected and corrected logMAR distance, intermediate and near visual acuity ((uncorrected distance visual acuity (UDVA), uncorrected intermediate visual acuity (UIVA) uncorrected near visual acuity (UNVA), corrected distance visual acuity (CDVA), corrected near visual acuity) and manifest refraction were evaluated during a 2-4 month follow-up. Additionally, patients were asked about photic phenomena and spectacle dependence. The surgeons subjectively assessed various aspects of the surgery.A significant improvement in CDVA was observed postoperatively (p0.01), with a significant reduction in manifest cylinder (p0.01). Mean postoperative binocular UDVA and UNVA were 0.04±0.10 and 0.06±0.12, respectively. Monocular UDVA and UNVA was 0.20 or better in 85.4% and 87.0% of eyes, respectively. Mean binocular logMAR UIVA was 0.21±0.20. Only 10.5% of patients required postoperative correction for near or intermediate distance. The incidence of moderate to severe photic phenomena was limited. Surgeons defined the IOL implantation in most cases as easy or very easy, with a satisfaction rate with the procedure of 84%.The implantation of the multifocal toric IOL is a safe procedure that provides a very good visual rehabilitation in eyes with corneal astigmatism.

Evaluation of the visual and refractive results 3 months after implantation of a diffractive extended range of vision (ERV) intraocular lens (IOL) during cataract surgery.In a prospective multicentre study, patients with a calculated postoperative corneal astigmatism of ≤ 1.5 D received a diffractive ERV IOL (TECNIS Symfony, model ZXR00, Abbott Medical Optics, USA) during cataract surgery. After 3 months, the monocular and binocular corrected and uncorrected far, intermediate and near visual acuity, as well as refraction, were evaluated.18 patients (36 eyes) with a mean age of 63.34 ± 4.6 years underwent bilateral cataract surgery. After 3 months, the binocular uncorrected distance visual acuity (UDVA) of logMAR was - 0.05 ± 0.11 and the corrected distance visual acuity (CDVA) of logMAR - 0.14 ± 0.05. Binocular uncorrected intermediate (UIVA) and near visual acuity (UNVA) were logMAR - 0.09 ± 0.02 and 0.19 ± 0.09, respectively. A target refraction of ± 0.75 D was reached by 89 % of the patients.Implantation of an extended range of vision intraocular lens offers an effective way for visual rehabilitation at far and intermediate distances. Near vision is still in a functional range.

Evaluation of the clinical data 3 months after implantation of a new diffractive multifocal intraocular lens (MIOL) with a reduced near add power of + 2.75 D.In a prospective study, patients who underwent cataract surgery or refractive lens exchange with implantation of an MIOL (Tecnis ZKB00, Abbott Medical Optics, Santa Ana, California, USA) were included. Three months postoperative corrected and uncorrected visual acuities at different distances were measured and evaluated. Those patients that underwent bilateral MIOL implantation additionally filled out a questionnaire 3 months postoperatively.Between October 2013 and August 2014, 115 eyes of 62 patients were implanted with the ZKB00 IOL. Mean postoperative refractions were - 0.27 ± 0.44 D for the spherical equivalent, respectively. Mean binocular CDVA was - 0.01 ± 0.3 logMAR with a mean binocular UDVA of 0.06 ± 0.08 logMAR. For near distance in 40 cm, an UNVA of 0.07 ± 0.10 logMAR three months postoperatively was measured.The ZKB00 IOL belongs to a group of novel MIOL with an increased intermediate visual performance. Our study shows good visual acuity at all distances, as well as a high rate of satisfaction and subjectively good image quality.

A 36-year-old patient developed marked pigmentation, marked myopathy and severe hypokalaemic alkalosis which at first pointed towards an ectopic ACTH syndrome. The dexamethasone test at a high dose indicated cortisol suppression. A mediastinal tumour was seen radiologically, but the sella was of normal size. Computed tomography provided indirect signs of a sellar space-occupying lesion which suggested an ectopic production of corticotropin-releasing factor (CRF) as cause of the Cushing's syndrome. CRF concentration in antecubital venous blood was markedly elevated to 280 ng/l. The mediastinal tumour was excised and proved to be a carcinoid histologically. Postoperatively the CRF concentration fell to 70 ng/l. An extract of the carcinoid contained 15.5 ng/g wet-weight of CRF and 254 ng/g wet-weight of beta-endorphin. The patient died 5 weeks postoperatively of sepsis with bilateral pneumonia. At autopsy the hypophysis was of normal size but showed nodular ACTH-cell hyperplasia. This was thus a case of Cushing's syndrome resulting from ectopic CRF production in a mediastinal carcinoid tumour.

BACKGROUND AND OBJECTIVE: Drug treatment of hypothalamic-pituitary Cushing's syndrome is indicated if standard surgical intervention is not possible or has failed. The question arises whether, after unsatisfactory treatment with various adrenostatic drugs, mitotane (o,p'-DDD), used against adrenal cortical cancer, is efficacious and free of significant side effects when used long-term. PATIENTS AND METHODS: The results of long-term administration of mitotane to six patients, including one pregnant woman, were analysed retrospectively. After a moderate initial dosage of 3.0 g daily a maintenance dose of minimally 0.5 g per week was given or the treatment temporarily interrupted. The concentration of urinary free cortisol served as the main criterion of efficaciousness, together with the clinical course. The plasma concentrations of cortisol, aldosterone and ACTH were also determined, as well as routine clinicochemical parameters. RESULTS: Cortisol excretion became normal in all patients between the 2nd and 10th treatment month, falling from 919 +/- 621.3 nmol daily in the six months before treatment to 162 +/- 93.0 nmol daily in the third six-month treatment period (mean +/- standard deviation). Normal cortisol excretion and regression of symptoms was noted, dose-dependent, as long as the 12th year after start of treatment. Adrenocortical insufficiency occurred in one patient and at times required hormone substitution, followed by lasting remission without special treatment. Significant side effects were not observed other than a reversible increase in gamma-glutamyl transpeptidase. CONCLUSION: Mitotane proved to be an efficacious drug which in exceptional cases can be used without significant side effects in low dosage for the long-term treatment of hypothalamic-pituitary Cushing disease.

Background: Multiple-endocrine-neoplasia-type-1 (MEN1) is an autosomal-dominant inherited disorder characterized by the combined occurrence of primary hyperparathyroidism (pHPT), gastroenteropancreatic neuroendocrine tumors (GEP), adenomas of the pituitary gland (APA), adrenal cortical tumors (ADR) and other tumors. As the tumors appear in an unpredictable schedule, uncertainty about screening programs is persisting. Objective: To optimize screening and to analyze possible differences in sporadic versus familial cases. Methods: We analyzed data of 419 individuals including 306 MEN-1 patients (138 isolated and168 familial cases out of 102 unrelated families). Results: A total of 683 tumors occurred consisting of 273 pHPT, 138 APA, 166 GEP, 57 ADR, 24 thymic- and bronchial-carcinoids as well as 25 neoplasms of other tissues. The age-related penetrance was determined as 10%, 35%, 67%, 81% and 100% at 20, 30, 40, 50 and 65 years respectively. Although pHPT being the most frequent first manifestation (41%), also GEP (22%) or APA (21 %) were found to be the first presentation. APA occurred significantly more frequent (p

PURPOSE: To evaluate the visual, refractive, and contrast sensitivity outcomes, as well as the level of photic phenomena, after cataract surgery with implantation of a trifocal diffractive toric intraocular lens (IOL). METHODS: This prospective study included 56 eyes with corneal astigmatism of 1.00 diopters (D) or greater of 28 patients (age: 23 to 78 years) undergoing cataract surgery with implantation of the trifocal toric IOL AT LISA tri toric 939MP (Carl Zeiss Meditec, Jena, Germany). Monocular and binocular visual outcomes, refractive changes, contrast sensitivity, and photic phenomena perception (Halo & Glare Simulator; Eyeland-Design Network GmbH, Vreden, Germany) were evaluated at 3 months postoperatively. RESULTS: Mean 3-month postoperative monocular uncorrected distance (UDVA), intermediate (UIVA), and near (UNVA) visual acuities were 0.13 ± 0.15, 0.08 ± 0.15, and 0.13 ± 0.18 logMAR, respectively. Binocular postoperative CDVA, DCIVA, and DCNVA values were 0.10 logMAR or better in all cases. A total of 88.2%, 88.2%, and 95.5% of eyes achieved binocular UDVA, UIVA, and UNVA values of 0.20 logMAR or better, respectively. Postoperative refractive cylinder was 0.50 D or less and 1.00 D or less in 78.6% and 98.2% of eyes, respectively. Photopic contrast sensitivity was significantly better than mesopic values for the spatial frequencies of 6 ( P = .007), 12 ( P = .005), and 18 cycles/degree ( P = .011). Mean size and intensity of halos were 50.67 ± 15.69 and 54.89 ± 17.86, respectively. Mean glare size and intensity were 39.67 ± 3.51 and 44.67 ± 15.01, respectively. CONCLUSIONS: The evaluated trifocal diffractive toric IOL provides an effective restoration of the distance, intermediate, and near vision after cataract surgery with good levels of visual quality and minimal photic phenomena. [ J Refract Surg. 2015;31(8):504–510.]

Purpose To evaluate visual outcomes following implantation of a trifocal diffractive intraocular lens (IOL) and to analyze their correlation with patient satisfaction and ease of performing daily tasks. Methods This was a prospective study enrolling 100 eyes of 50 patients undergoing cataract surgery with implantation of trifocal IOL AT LISA tri 839MP. Visual and refractive outcomes were evaluated during a 3-month follow-up. Postoperatively, a questionnaire was used to evaluate patient satisfaction with regard to surgical outcome, spectacle independence, perception of photic phenomena, and ease of performing some vision-related activities. Results A total of 91%, 87%, and 79% of eyes achieved a monocular uncorrected distance, near, and intermediate visual acuity of 0.1 logarithm of the minimum angle of resolution or better, respectively. After the surgery, 96% of the patients could perform their daily activities without problems. The mean spectacle independence scores for reading, doing computer work, and for distance were 10.33 ± 12.47, 5.71 ± 11.90, and 3.92 ± 9.77, respectively (scale: 0 = no spectacles needed; 40 = spectacles always needed). No correlation was found between spectacle independence and visual outcome (-0.101 ≤ r ≤ 0.244, p ≥ 0.087). Mean scores (0 = no symptoms; 40 = strong symptoms) for glare at night, ghost images, and halos were 15.15 ± 12.02, 4.49 ± 7.92, and 13.34 ± 10.82, respectively. No correlation was found between photic phenomena and visual outcome (-0.199 ≤ r ≤ 0.209, p ≥ 0.150). A total of 80% of patients reported satisfaction with the surgery outcome, and 86% would recommend the surgery to friends and family. Conclusions Implantation of the AT LISA tri 839MP IOL after cataract surgery provides effective visual restoration associated with a minimal level of photic phenomena, a positive impact on the performance of vision-related daily activities, and a high level of postoperative patient satisfaction.

Nowadays, further developments in the field of intraocular lenses offer a higher level of spectacle independence for our patients. As light gets scattered on different focal points a wider range of defocus is created. This greater defocus area makes it more difficult for us to determine the objective or subjective refraction. This contribution is concerned with the difficulties of measuring visual acuity in different intraocular lens designs and different measurement distances. Measuring refraction after implantation of a multifocal intraocular lens is a complex procedure and the experience of the examiner plays a crucial role. Retinoscopy, keratometry and the defocus curve are reliable methods for testing, while the auto refractometer, bichromatic testing and the cross-cylinder have limitations.

Background To evaluate binocular visual outcome for near, intermediate and distance compared to monocular visual outcome at the same distances in patients implanted with a diffractive trifocal intraocular lens (IOL). Methods The study comprised of 100 eyes of 50 patients that underwent bilateral refractive lens exchange or cataract surgery with implantation of a multifocal diffractive IOL (AT LISA tri 839MP, Carl Zeiss Meditech, Germany). A complete ophthalmological examination was performed preoperatively and 3 month postoperatively. The main outcome measures were monocular and binocular uncorrected distance (UDVA), corrected distance (CDVA), uncorrected intermediate (UIVA), and uncorrected near visual acuities (UNVA), keratometry, and manifest refraction. Results The mean age was 59.28 years ± 9.6 [SD] (range 44–79 years), repectively. There was significant improvement in UDVA, UIVA, UNVA and CDVA. Comparing the monocular results to the binocular results there was a statistical significant better binocular outcome in all distances (UDVA p = 0.036; UIVA p

INTRODUCTION Endovascular therapy is being used increasingly also to treat ruptured infrarenal aortic aneurysms. Non-traumatic rupture of non-aneurysmatic infrarenal aorta is an absolute rarity. METHODS The feasibility of endovascular repair of infrarenal aortic rupture is demonstrated with a case history and a literature review. RESULTS A 58-year-old male developed spontaneous rupture of his infrarenal aorta after successful chemotherapy resulting in regression of a periaortic tumor. The patient suffered from a sepsis for several days before aortic rupture was discovered during computed tomography for focus search. Immediate endovascular stent-graft repair was performed under emergency conditions. There is no recurrence and the patient is doing well with the stent-graft in place without any pathological finding after a follow-up of 24 months. So far only three further cases have been reported of endovascular repair for penetrating atherosclerotic ulcer with rupture of the infrarenal aorta. DISCUSSION This is the first report of endovascular repair of aortic rupture due to successful chemotherapy of a periaortic mesothelioma. Furthermore, this is the fourth case of successful stent-graft placement to treat non-aneurysmatic rupture of the infrarenal aorta. Minimally invasive endovascular therapy should become a standard treatment option for aortic rupture.

History and clinical findings A 40-year-old woman complained of weight gain for 10 years. Because of this, she yearly went on a excessive weight reduction diet with weight loss of up to 12 kg. Nevertheless, a total weight gain of 15 kg as well as changing degrees of hypertension, muscular weakness and severe depression were registered during this long period. For 5 years ecchymosis and for 2 years oligomenorrhoea as well as oedema had become evident. Recently large macronodular adrenal glands were found on routine abdominal ultrasound. At the time of clinical investigation early this year she presented with relatively mild Cushing features, such as moon face, central obesity, hirsutism, ecchymosis and hypertension. Investigations Endocrine testing demonstrated suppressed plasma ACTH, low to normal plasma cortisol after overnight fasting and an inverse diurnal rhythm. During 24 hours of fasting plasma cortisol levels and free urinary cortisol excretion were within normal range, but highly elevated under normal food intake. Plasma cortisol levels increased dramatically in response to a lipid-rich meal (5.6-fold), to a protein-rich meal (6.6-fold) and to oral glucose (3.7-fold). Increases in plasma cortisol were closely correlated to increases of gastric inhibitory polypeptide (GIP) concentrations. Subcutaneous injection of octreotide completely inhibited the plasma cortisol and GIP response to oral glucose. Plasma ACTH and cortisol levels did not increase after CRH administration, but fasting plasma cortisol promptly increased after ACTH stimulation. Non-fasting plasma cortisol levels and free urinary cortisol excretion were not suppressed by high doses of dexamethasone. Abdominal computed tomography showed macronodular enlargement of both adrenal glands. Treatment and course The patient underwent bilateral adrenalectomy. Postoperatively, she was treated with replacement doses of hydrocortisone and fludrocortisone. All Cushing features have regressed, a normal menstrual cycle and normal plasma ACTH concentrations have become evident. Conclusion This case report not only presents a very rare cause of Cushing's syndrome--food-dependent or gastric inhibitory peptide (GIP)-dependent hypercortisolism--but it also demonstrates a special course of this disease of relatively long duration. Severe adrenal hyperplasia contrasted with mild clinical symptoms obviously caused by frequent periods of weight reduction diet. Considering this moderate clinical course it might well be that food-dependent hypercortisolism is more frequent than supposed, but not diagnosed owing to relatively mild clinical symptoms.

HISTORY AND CLINICAL FINDINGS A 54-year-old man was admitted to hospital with dyspnoea, giddiness, lack of appetite and burning sensation in the tongue for 3 years. Gastritis was known from his history. Clinical examination showed jaundice, glossitis, systolic murmur and pallhypesthesia of both legs. INVESTIGATIONS Blood and bone marrow investigations revealed pernicious anaemia. At gastroscopy, fundal gastritis was diagnosed and two small polyps of the fundus were removed. Histologically, one of the two polyps turned out to be a gastric carcinoid type 1 (according to [16]). Additional investigations (x-ray, ultrasound of the abdomen, somatostatin-receptor scintigraphy) did not find any metastases. TREATMENT AND COURSE With the diagnosis of a pernicious anaemia the patient was treated with vitamin B12. The original symptoms and the anaemia disappeared. Additional therapy of the gastric carcinoid was not necessary. One year after beginning of the treatment, no signs of the gastric carcinoid were found. CONCLUSION In gastric polyps of patients with pernicious anemia neoplastic transformations will often be found. That is why polypectomy should always be done. The best therapy of the gastric carcinoids type 1, which are the most frequently gastric carcinoids, is endoscopic tumour removal. Surgery is the treatment of choice only for gastric carcinoids type 2 to 4. Concerning quality of life and costs, endoscopic treatment is better for patients with gastric carcinoids type 1 than conventional surgical therapy.

We studied plasma cortisol levels at 00:00 h and 08:00 h in 103 patients with Cushing's syndrome and 144 patients in whom this diagnosis had been excluded. These patients were hospitalized in our department from 1975 to 1996. Additionally, we measured these parameters in 20 healthy volunteers and in 5 patients with nonendocrine disease. Corresponding data of urinary free cortisol and low-dose dexamethasone suppression testing were included in the evaluation. Values (mean+/-SD) from patients with Cushing's syndrome: 510+/-232 nmol/l (range 165-1488) for plasma cortisol 00:00 h, 574+/-242 nmol/l (range 236-1612) for plasma cortisol 08:00 h, 991+/-885 nmol/24 h (range 154-4866) for urinary free cortisol and 479+/-304 nmol/l (range 34 - 1,393) for plasma cortisol after 1.5 mg dexamethasone. Values from the patients excluded from Cushing's syndrome: 99+/-76 nmol/l (range 5-371) for plasma cortisol 00:00 h, 393+/-136 nmol/l (range 119-812) for plasma cortisol 08:00 h, 126+/-84 nmol/24 h (range 30-485) for urinary free cortisol, and 64+/-82 nmol/l (range 5-395) for plasma cortisol after 1.5 mg dexamethasone. Values of the healthy volunteers respectively patients with non-endocrine disease: 59+/-30 nmol/l (range 25-130) respectively 127+/-80 nmol/l (range 62-265) for plasma cortisol 00:00 h and 388+/-144 nmol/l (range 157-651) respectively 498+/-113 nmol/l (range 302-581) for plasma cortisol 08:00 h. None of the Cushing patients exhibited a 00:00 h plasma cortisol below 140 nmol/l and only one had a urinary free cortisol below 200 nmol/24 h, whereas 4 were complete dexamethasone suppressors. The diagnostic value of these parameters was examined based on various cutoffs. We recommend determination of midnight plasma cortisol as an efficient and simple additional procedure for the diagnosis of Cushing's syndrome. The sensitivity and specificity of this procedure is similar to urinary free cortisol and slightly above the low-dose dexamethasone suppression testing in our hospitalized patients.

To record weak light scattering spectra with a Fabry-Perot interferometer, long measuring times are often necessary. For long term stability of the parallel alignment of the mirrors, a microcomputer controlled interferometer system has been developed for both single and multipass operation. The three piezo stacks, which are responsible for the alignment of the mirrors, are supplied from three digital-to-analog converters controlled by a personal computer. The test and correction steps of the mirror alignment are performed around two independent axes perpendicular to each other. A special function generator provides a nonlinear scanning ramp for the piezostacks, thereby allowing a temporally linear scanning of the Fabry-Perot.

Endovascular therapy is being used increasingly also to treat ruptured infrarenal aortic aneurysms. Non-traumatic rupture of non-aneurysmatic infrarenal aorta is an absolute rarity.The feasibility of endovascular repair of infrarenal aortic rupture is demonstrated with a case history and a literature review.A 58-year-old male developed spontaneous rupture of his infrarenal aorta after successful chemotherapy resulting in regression of a periaortic tumor. The patient suffered from a sepsis for several days before aortic rupture was discovered during computed tomography for focus search. Immediate endovascular stent-graft repair was performed under emergency conditions. There is no recurrence and the patient is doing well with the stent-graft in place without any pathological finding after a follow-up of 24 months. So far only three further cases have been reported of endovascular repair for penetrating atherosclerotic ulcer with rupture of the infrarenal aorta.This is the first report of endovascular repair of aortic rupture due to successful chemotherapy of a periaortic mesothelioma. Furthermore, this is the fourth case of successful stent-graft placement to treat non-aneurysmatic rupture of the infrarenal aorta. Minimally invasive endovascular therapy should become a standard treatment option for aortic rupture.

The vast majority of Caucasian patients presenting with hereditary hemochromatosis demonstrate a single homozygous missense mutation in the HFE gene (C282Y). The underlying genetic defects in hemochromatosis patients of non-Caucasian origin are largely unknown. A 48-year-old man of Vietnamese origin presented with insulin-dependent diabetes mellitus, tertiary adrenocortical insufficiency, and laboratory results highly indicative of hereditary hemochromatosis. Because the patient was negative for the known HFE gene mutations C282Y, H63D, and S65C HFE, the entire coding region and intron/exon boundaries of the HFE gene was investigated. Sequencing studies identified a homozygous G-to-A transition at position +1 of intron 5 (IVS5+1 G/A). This newly described mutation alters the invariant G at position +1 of the 5' splice site causing altered mRNA splicing and exon skipping with exon 4 being spliced to exon 6. Both heterozygously affected children (age 19 and 20 years) had moderately increased ferritin levels with normal serum iron concentration and transferrin saturation. The newly described mutation was not detected in a control group consisting of 220 Caucasian individuals as verified by allele-specific polymerase chain reaction. We describe for the first time a homozygous HFE splice site mutation (IVS5+1 G/A) in a non-Caucasian patient with hereditary hemochromatosis. Although the absence of this novel HFE gene mutation in Caucasian subjects suggests that the mutation is exclusive to this family, mutation screening in populations of different ethnic background is recommended to precisely define its contribution to hereditary hemochromatosis in non-Caucasian patients.

A 40-year-old woman complained of weight gain for 10 years. Because of this, she yearly went on a excessive weight reduction diet with weight loss of up to 12 kg. Nevertheless, a total weight gain of 15 kg as well as changing degrees of hypertension, muscular weakness and severe depression were registered during this long period. For 5 years ecchymosis and for 2 years oligomenorrhoea as well as oedema had become evident. Recently large macronodular adrenal glands were found on routine abdominal ultrasound. At the time of clinical investigation early this year she presented with relatively mild Cushing features, such as moon face, central obesity, hirsutism, ecchymosis and hypertension.Endocrine testing demonstrated suppressed plasma ACTH, low to normal plasma cortisol after overnight fasting and an inverse diurnal rhythm. During 24 hours of fasting plasma cortisol levels and free urinary cortisol excretion were within normal range, but highly elevated under normal food intake. Plasma cortisol levels increased dramatically in response to a lipid-rich meal (5.6-fold), to a protein-rich meal (6.6-fold) and to oral glucose (3.7-fold). Increases in plasma cortisol were closely correlated to increases of gastric inhibitory polypeptide (GIP) concentrations. Subcutaneous injection of octreotide completely inhibited the plasma cortisol and GIP response to oral glucose. Plasma ACTH and cortisol levels did not increase after CRH administration, but fasting plasma cortisol promptly increased after ACTH stimulation. Non-fasting plasma cortisol levels and free urinary cortisol excretion were not suppressed by high doses of dexamethasone. Abdominal computed tomography showed macronodular enlargement of both adrenal glands.The patient underwent bilateral adrenalectomy. Postoperatively, she was treated with replacement doses of hydrocortisone and fludrocortisone. All Cushing features have regressed, a normal menstrual cycle and normal plasma ACTH concentrations have become evident.This case report not only presents a very rare cause of Cushing's syndrome--food-dependent or gastric inhibitory peptide (GIP)-dependent hypercortisolism--but it also demonstrates a special course of this disease of relatively long duration. Severe adrenal hyperplasia contrasted with mild clinical symptoms obviously caused by frequent periods of weight reduction diet. Considering this moderate clinical course it might well be that food-dependent hypercortisolism is more frequent than supposed, but not diagnosed owing to relatively mild clinical symptoms.

A 54-year-old man was admitted to hospital with dyspnoea, giddiness, lack of appetite and burning sensation in the tongue for 3 years. Gastritis was known from his history. Clinical examination showed jaundice, glossitis, systolic murmur and pallhypesthesia of both legs.Blood and bone marrow investigations revealed pernicious anaemia. At gastroscopy, fundal gastritis was diagnosed and two small polyps of the fundus were removed. Histologically, one of the two polyps turned out to be a gastric carcinoid type 1 (according to [16]). Additional investigations (x-ray, ultrasound of the abdomen, somatostatin-receptor scintigraphy) did not find any metastases.With the diagnosis of a pernicious anaemia the patient was treated with vitamin B12. The original symptoms and the anaemia disappeared. Additional therapy of the gastric carcinoid was not necessary. One year after beginning of the treatment, no signs of the gastric carcinoid were found.In gastric polyps of patients with pernicious anemia neoplastic transformations will often be found. That is why polypectomy should always be done. The best therapy of the gastric carcinoids type 1, which are the most frequently gastric carcinoids, is endoscopic tumour removal. Surgery is the treatment of choice only for gastric carcinoids type 2 to 4. Concerning quality of life and costs, endoscopic treatment is better for patients with gastric carcinoids type 1 than conventional surgical therapy.

Cystic degenerations of parathyroid adenoma appear in 4 to 5% of all cases. These tumors might be localized in the mediastinum. Until now very few cases of cystic parathyroid adenoma have been published. Difficulties in the diagnosis are common because these adenomas are often thought to belong to other anatomic structures. Persistence of hyperparathyroidism and elevated surgical complications can be the consequences.We report on the case of a 76-year-old patient with primary hyperparathyroidism. Pre-operative ultrasound revealed a suspicious lesion near the left upper lobe of the thyroid and an unclear cystic structure in the upper posterior mediastinum, suspected as cervical cyst or dysontogenetic cyst. Additional investigations concerning the cystic mediastinal lesion suggested the following differential diagnoses: goiter (X-ray), bronchogenic cyst, cervical cyst or esophageal diverticulum (CT). During the operation no adenoma could be found on the left side, but when the cystic mediastinal lesion was finally removed it turned out to be a giant cystic activated parathyroid adenoma. Postoperatively calcium, phosphate and PTH levels returned to normal.In patients with primary hyperparathyroidism, unclear cystic mediastinal masses should be taken into consideration.

Molecular genetics recently uncovered the mystery of the protean picture of McCune-Albright syndrome by identification of the somatic gain of function mutations in the GNAS1 gene. Here we present an adult patient with fibrous dysplasia and an endocrinopathy resulting in unusual giant height. The clinical diagnosis in the patient could be confirmed by molecular investigations in tissues involved in the process of fibrous dysplasia.

Disturbances of glucose metabolism with hyperinsulinism and peripheral insulin resistance are frequently observed in patients with hyperparathyroidism. The mechanism of how hyperparathyroidism affects glucose metabolism is not known. Hypercalcemia, hypophosphatemia and the parathyroid hormone itself seem to be involved. However, parathyroidectomy exerted rather variable effects on glucose metabolism: In patients with fully developed diabetes mellitus both, a complete normalisation of glucose tolerance as well as no change in the metabolic situation have been observed. We report a 64-year old female patient with primary hyperparathyroidism and diabetes mellitus. The patient had severe insulin resistance with insulin requirements of 200 IU/day. Fasting insulin and C-peptide levels were elevated. After successful operation of a parathyroid adenoma there was a marked improvement in diabetes, and the patient's insulin requirement decreased to one third of the preoperative dose. This case further illustrates the association between primary hyperparathyroidism and diabetes mellitus and the potential improvement of the metabolic situation after parathyroidectomy.

Drug treatment of hypothalamic-pituitary Cushing's syndrome is indicated if standard surgical intervention is not possible or has failed. The question arises whether, after unsatisfactory treatment with various adrenostatic drugs, mitotane (o,p'-DDD), used against adrenal cortical cancer, is efficacious and free of significant side effects when used long-term.The results of long-term administration of mitotane to six patients, including one pregnant woman, were analysed retrospectively. After a moderate initial dosage of 3.0 g daily a maintenance dose of minimally 0.5 g per week was given or the treatment temporarily interrupted. The concentration of urinary free cortisol served as the main criterion of efficaciousness, together with the clinical course. The plasma concentrations of cortisol, aldosterone and ACTH were also determined, as well as routine clinicochemical parameters.Cortisol excretion became normal in all patients between the 2nd and 10th treatment month, falling from 919 +/- 621.3 nmol daily in the six months before treatment to 162 +/- 93.0 nmol daily in the third six-month treatment period (mean +/- standard deviation). Normal cortisol excretion and regression of symptoms was noted, dose-dependent, as long as the 12th year after start of treatment. Adrenocortical insufficiency occurred in one patient and at times required hormone substitution, followed by lasting remission without special treatment. Significant side effects were not observed other than a reversible increase in gamma-glutamyl transpeptidase.Mitotane proved to be an efficacious drug which in exceptional cases can be used without significant side effects in low dosage for the long-term treatment of hypothalamic-pituitary Cushing disease.

In a retrospective study we analysed the outcome of 27 pregnancies in 17 hyperprolactinaemic patients. Cranial CT or MRI scans showed a microadenoma in 9, a macroadenoma in 7 and no change in 1 case. In 5 patients macroadenomas were surgically removed but a residual hyperprolactinaemia remained. In 2 patients with selectively removed microadenomas through the transsphenoidal route only in one case normal prolactin levels could be achieved. This patient became two times pregnant. In 24 patients pregnancy was induced by dopaminergic treatment whereas another patient with microadenoma became spontaneously pregnant after surgery and treatment with bromocriptine. The treatment was discontinued as soon as the pregnancy was recognised. 19 pregnancies were finished by spontaneous delivery and 3 by Caesarean section. Termination of pregnancy was artificially induced in 3 patients. One miscarriage was observed. Another patient was suffering from an extrauterine pregnancy. During pregnancy prolactin levels increased in comparison to basal levels. After delivery and lactation the same prolactin level was found compared to basal levels in patients with microprolactinomas. In patients suffering from macroadenomas prolactin levels decreased in comparison to basal levels. CT of MRI scan evaluations performed after delivery revealed a clinical not relevant increase as well as a decrease of tumor size in 2 cases, respectively. During pregnancy there was no complication due to tumor found in treated patients for hyperprolactinaemia. Only about one half of mothers were able to perform regular breast feeding.

Reported in this paper is the case of a boy aged 15 years with sellar germinoma which led to one singular pulmonary metastasis, three years after subtotal surgical removal and subsequent successful irradiation of the tumor rest. The primary tumor together with the lung metastasis were characterised as germinoma with trophoblastic giant cells as additional elements. Interpretation of the pulmonary tumour was somewhat difficult, since diagnosis of the primary tumor had encouraged assumption of a chromophobic hypophyseal adenoma with polymorphic manifestation, with no relationship between the intracranial and pulmonary tumors being presumed by the surgeon. The true nature of the metastasis and, consequently, the histogenetic identity of both tumors was cleared up by immunohistochemical detection of beta-HCG in almost all syncytiotrophoblasts. Comprehensive analysis of the literature on metastasising intracranial germinomas may be helpful in disclosing possible relationships between the histological type of a primary tumor or neurosurgical action and the metastatic potential of tumors.

A 36-year-old patient developed marked pigmentation, marked myopathy and severe hypokalaemic alkalosis which at first pointed towards an ectopic ACTH syndrome. The dexamethasone test at a high dose indicated cortisol suppression. A mediastinal tumour was seen radiologically, but the sella was of normal size. Computed tomography provided indirect signs of a sellar space-occupying lesion which suggested an ectopic production of corticotropin-releasing factor (CRF) as cause of the Cushing's syndrome. CRF concentration in antecubital venous blood was markedly elevated to 280 ng/l. The mediastinal tumour was excised and proved to be a carcinoid histologically. Postoperatively the CRF concentration fell to 70 ng/l. An extract of the carcinoid contained 15.5 ng/g wet-weight of CRF and 254 ng/g wet-weight of beta-endorphin. The patient died 5 weeks postoperatively of sepsis with bilateral pneumonia. At autopsy the hypophysis was of normal size but showed nodular ACTH-cell hyperplasia. This was thus a case of Cushing's syndrome resulting from ectopic CRF production in a mediastinal carcinoid tumour.

Die Verbindungen BaTeS3 · 2 H2O und (NH4)2TeS3 wurden dargestellt und strukturell untersucht. Die kristallographischen Daten sind: Nach den vollstandigen rontgenographischen Strukturaufklarungen stellt das Anion der trithiotellurigen Saure TeS in diesen Salzen eine verzerrte trigonale Pyramide dar. Die TeS-Atomabstande liegen zwischen 2,34–2,36 A. Preparation and Crystal Structure of the Thiotellurites BaTeS3·2H2O and (NH4)2TeS3 The new compounds BaTeS3 · 2 H2O and (NH4)2TeS3 have been prepared and their structures determined. According to these the anion of the trithiotelluric acid in these compounds represents a distorted trigonal TeS−pyramid. The TeS-distances are 2.34–2.36 A. Crystallographic data see „Inhaltsubersicht”.

Endokrine active tumours of the adrenal cortex are rare diseases in man. Benign as well as malignant tumours of the adrenal cortex can alone excrete cortisol, aldosterone, androgens or oestrogens. More frequent are, however, tumours with a mixed incretion. Publications about tumours forming sexual hormones are above all descriptions of individual cases. After a short explanation of the most important clinical, diagnostic and therapeutic aspects on androgen-producing tumours of the adrenal cortex four own cases are discussed.

Sm-C concentrations in serum were found significantly different in either active acromegaly or following successful treatment with pituitary adenomectomy. Although after normalization of serum GH the Sm-C levels sometimes exceeded the normal range no overlap was found between both groups. Exceptionally two acromegalic patients showed elevated Sm-C levels in spite of normal GH values. Likewise, a high Sm-C concentration was found in one patient suspective of ectopic GH secretion with only moderately elevated serum GH. Sm-C determinations are judged as a good adjunct to usual diagnostic methods which in special cases of acromegaly can be even superior to measurements of serum GH.

According to modern knowledge, acromegaly can develop in at least three ways. A pituitary adenoma with growth hormone overproduction is the most frequent. Much rarer is ectopic growth hormone secretion by extra-hypophyseal tumors. A further possibility is the production of growth hormone releasing factor (GRF) by hypothalamic or ectopic tumors. This involves the secretion of a substance which selectively stimulates the GH producing cells of the pituitary. Special features of the clinical and morphological picture of this condition are described, based on the authors own observations. Two patients developed acromegaly: one had a retroperitoneal paraganglioma and the other a bronchial carcinoid. Ectopic GRF secretion could be confirmed radioimmunologically and immunohistologically in both cases. As a result of the on-going, tumor related GRF stimulation the patients developed nodular or diffuse GH-cell hyperplasia in the adenohypophysis. Since ectopic GH secretion does not cause hyperplasia of the adenohypophyseal cells, morphologic examination of the hypophysis can contribute to the differential diagnosis in such cases.

Based on a sensitive TSH-RIA (measuring-range 0.2-25 mU/l, 50%-intercept 2 mU/l, coefficients of between-assay-variation 5-10%) and on nearly 1200 cases, it is demonstrated that clinical results can be received by determination of basal serum-TSH alone. Values above 0.5 mU TSH/l indicate positive TRH-tests in a good correlation between increasing basal concentrations and pituitary TSH-reserve. TSH-levels below 0.3 mU/l proceed with negative TRH-tests. In selected healthy subjects, we found all TSH-values between 0.7 and nearly 5 mU/l (means = 1.99). In euthyroid goiters, TSH-levels are normally in the same range, but in nodular goiters, subnormal values were found accumulated. TSH-levels below 0.3 mU/l are helpful to discover non-suppressibility. Hormone-treatment of goiters is successful above all in case of high-normal pretherapeutic TSH-values. "Fine-tuning" of hormone-dosage (goiter-treatment, postoperative prophylaxis, suppression-therapy in thyroid carcinoma) as well as monitoring of thyrotoxicosis-treatment is recommended to be performed by means of basal TSH-determination. In pituitary disorders, thyroid hormone-treatment seems to be necessary only at TSH-levels below 0.5 mU/l. Combination of basal TSH and total T-3 is economical and describes nearly all functional situations in diagnosis and treatment of thyroid diseases.

The changes of the endocrine pancreas in two cases of pluriglandular neoplasia type I were studied. A 15 year-old girl showed an amyloid containing ductuloinsular B cell adenoma, microadenomas and nesidioblastic processes. The simultaneous presence of hyperprolactinemia and nephrolithiasis indicates the existence of the endocrine polyadenomatosis. A 33 year-old woman had four amyloid containing B cell adenomas with ductular proliferation, a main cell adenoma of the parathyroid and a prolactinoma of the adenohypophysis. Solitary or multiple ductuloinsular adenomas, amyloid of the endocrine type and nesidioblastic activity are not specific features of the endocrine pancreas in pluriglandular adenomatosis. Similar structures can be found in isolated islet cell tumors or in nesidioblastosis. Ductuloinsular proliferations can also be observed in chronic pancreatitis.