[Idiopathic hypoparathyroidism--a rare disease?]. / Der idiopathische Hypoparathyreoidismus--eine seltene Krankheit?

Five patients with nonfamilial idiopathic hypoparathyroidism were observed in a peripheral hospital. There was no association with other autoimmune disorders such as hypothyroidism, adrenal insufficiency or pernicious anemia. Only in one patient with tetany was the diagnosis clinically obvious; all the others presented with unusual clinical symptoms. These manifestations of chronic hypocalcemia are presented, as well as the diagnostic workup and therapeutic management. We suggest that idiopathic hypoparathyroidism is not a very rare disease, but one which is often missed because of the unusual clinical picture.

[Visceral leishmaniasis (kala-azar) as an opportunistic infection in immunosuppressed patients]. / Viszerale Leishmaniose (Kala-Azar) als opportunistischer Infekt bei immunsupprimiertem Patienten.

The occurrence of visceral leishmaniasis is rare in Switzerland. We discovered this disturbance in a patient while on immunosuppressive medication for mycosis fungoides. Untreated visceral leishmaniasis has a high mortality. It can be expected that more cases will be observed in patients treated with immunosuppressive drugs or in patients with the acquired immunodeficiency syndrome (AIDS).

[Pseudohyperchloremia in bromide poisoning]. / Pseudohyperchlorämie bei Brom-Intoxikation.

Hyperchloremia in a patient with intoxication by a bromide containing mixture is described. Loss of the normal anion gap in combination with hyperchloremia is only seen in bromide intoxication. An explanation for this observation is suggested.

[Hypercalcemia in hospitalized patients. Diagnostic and prognostic aspects]. / Hypercalciämie bei hospitalisierten Patienten. Diagnostische und prognostische Aspekte.

Over a period of 6 1/2 years hypercalcemia has been diagnosed in 86 patients. In 55.8% of cases a malignant tumor was the cause of the elevated calcium levels, and in 22.1% of cases primary hyperparathyroidism (p.Hp.) was diagnosed. In the remaining 19 patients hypercalcemia was related to renal insufficiency (5 patients), vitamin-D intoxication (4) and thiazide therapy (3), while other, rarer causes were identified in 7 cases. As expected, bronchial and breast carcinomas were most frequent (52%) in the malignancy group. In patients below age 40 malignancy was never responsible for the hypercalcemia, but in patients over 50 malignancy was present in 65%. Of the patients with solid tumors 54.8% had skeletal metastases, while other organ metastases were found in 33.3% of these. The occurrence of a hypercalcemia in patients with malignancy is associated with a poor prognosis since over 60% of the patients died within as little as one month and over 90% within a year after the occurrence of hypercalcemia. Renal function impairment in hypercalcemia was significantly more marked in the patients with malignancy than in patients with p.Hp. and was closely related to the calcium levels. Pharmacological reduction of the blood calcium level produced a distinct improvement in renal function in 70% of the patients treated. The current hypothesis on the pathogenesis of hypercalcemia among carcinoma patients is briefly discussed.

[Vasospastic syndrome in a young women with migraine, Raynaud's disease, cerebral ischemia and myocardial infarction]. / Vasospastisches Syndrom bei junger Frau mit Migräne, Raynaud-Syndrom, Hirninfarkt und Herzinfarkt.

The case is described of a 32-year-old female patient who has suffered since puberty from acute migraine attacks and Raynaud syndrome of both hands. In June 1981 acute posterior cerebral ischemia occurred. 11/2 years later the patient was hospitalized for acute myocardial infarction with normal and patent coronary arteries. Thereafter, the patient was treated with nifedipin and anticoagulation, and no other vascular complications have since occurred. The authors suggest that the patient is suffering from a diffuse vasospastic disorder leading to migraine attacks, Raynaud syndrome, cerebrovascular ischemia and myocardial infarction, in view of the fact that the patient has no other known risk factors for early vascular complications.

[Osteomalacia and antiepileptic drugs: study on the pathogenesis]. / Osteomalazie und Antiepileptida: Beitrag zur Pathogenese

Severe osteomalacia with secondary hyperparathyroidism was observed in a 19-year-old mentally retarded girl, who had been treated for several years with antiepileptic drugs. Vitamin D3 orally administered in low doses led to complete reversal of all symptoms and normalization of blood chemistry, X-ray pictures demonstrated healing of all lesions. It is suggested that the alterations of vitamin-D metabolism occuring during the administration of phenobarbital and hydantion are of importance only in patients with an already lowered intake of vitamin D3 or reduced exposure to ultraviolet rays.

[Lidocain poisoning. 5 patients with letal (2 patients) and reversible course]. / Lidocain-Intoxikation. Beobachtung bei 5 Patienten mit letalem (2 Patienten) und reversiblem Verlauf.

A report is presented on two cases of lethal intoxication with lidocain. One death occurred after paracervical anesthesia for legal abortion and the other after accidental oral administration. In three instances intoxication occurred during intensive care for ventricular arrhythmia. Immediate therapy in these cases led to rapid and complete recovery. The clinical signs of lidocain, intoxication are discussed and appropriate treatment for prevention of lethal complications is outlined.

[Long-term follow-up of a patient with accidental phosphor 32 overdose]. / Langzeitbeobachtung einer Patientin mit akzidenteller Phosphor-32-Uberdosierung.

The follow-up of a 93-year-old-patient with polycythaemia vera (PV) diagnosed in 1977 is presented. The patient accidentally received a ten-fold overdose of radioactive P32 due to an incorrectly labelled vial. 14 days after the administration of the overdose of P32, the patient was admitted to the University Hospital of Zurich with bone marrow aplasia. She recovered from the aplasia within 6 weeks. During the following 15 years she has suffered no relapse nor developed leukemia as a secondary complication.

[Bronchobiliary fistula in carcinoma of the hepatic duct bifurcation]. / Biliobronchiale Fistel bei Hepaticusgabelkarzinom.

Carcinoma of the hepatic duct bifurcation was diagnosed in a 67-year-old women with obstructive jaundice. As metastatic spread could not be demonstrated the carcinoma was removed with a view of achieving a cure (hemihepatectomy, resection of the hepatic duct and the bifurcation, cholecystectomy and hepatojejunostomy). Histological examination indicated adenocarcinoma of the biliary tract. Seven months postoperatively the patient was found to be cachectic and cough up greenish liquid sputum. Bilirubin concentration in sputum was 500 mumol/l. There was no jaundice and total bilirubin concentration was 33 mumol/l. Alkaline phosphatase was 508 U/l, but GOT and GPT were normal (23 U/l and 21 U/l). Computed tomography confirmed the clinical diagnosis of a biliobronchial fistula. The patient died 9 days after renewed hospitalization of tumour cachexia. The biliobronchial fistula was found at necropsy.

[Portal hypertension without liver cirrhosis in chronic vitamin A intoxication]. / Portale Hypertonie ohne Leberzirrhose bei chronischer Vitamin-A-Intoxikation.

The ysmptoms and findings in a patient after prolonged intake of excessive amounts of vitamin A for treatment of psoriasis are presentd. All the characteristic signs of chronic intoxication were reversible after withdrawal of the medication. Initially portal hypertension could be demonstrated, although histologically there was no cirrhosis of the liver. During the period of observation the disturbed liver function returned to normal and the signs of portal hypertension disappeared completely. Reversibility of portal hypertension is a unusual observation and is discussed in relation to similar situations in toxic liver disease.

[Acquired hemophilia A due to factor VIIIC inhibitor: detection of circulating immune complexes containing factor VIIIC but not factor VIIIR]. / Erworbene Hämophilie A durch Faktor-VIIIC-Hemmkörper: Nachweis zirkulierender Immunkomplexe, die nur Faktor VIIIC, nicht aber Faktor VIIIR enthalten.

In a 79-year-old, otherwise healthy woman who had developed multiple hematomas, laboratory tests revealed a factor VIII inhibitor (8.0 Bethesda units) which caused a decrease in factor VIIIC activity to 1%; factor VIIIR antigen and ristocetin cofactor activity were above normal values. Incubation of patient's plasma with a factor VIIIC sepharose immunoadsorbent partially removed the inhibitor, which was again detected in the IgG-containing eluate. In addition, immune complexes were found in the patient's serum. Whereas incubation of the serum with an immunoadsorbent containing rabbit antihuman factor VIIIR antibodies did not influence the titer of the immune complexes, 43% of which were removed by a rabbit antihuman factor VIIIC immunoadsorbent. This suggests that the factor VIIIC part only may be trapped by the inhibitor, leaving the factor VIIIR part of the molecule unchanged in circulation. This hypothesis is further supported by the fact that the amount of factor VIIIR antigen is likewise not diminished by incubation of patient's plasma with Raji cells.

[Lymphangiomyomatosis. Presentation of a case and review of the literature]. / Lymphangiomyomatose. Fallbeschreibung und Literaturübersicht

Diffuse lymphangiomyomatosis is a rare disorder of the mediastinal and retroperitoneal lymph nodes due to chronic benign proliferation of the smooth muscle cells in the lymphatics. This process leads to chylothorax and chylascites and eventually to cystic lung disease. The pulmonary complications are usually result in respiratory failure and death. The disease is only observed in females. A typical lethal course in a young woman is described and the literature is reviewed. The possible etiologic and therapeutic implications are discussed.

[Familial Wilson's disease: copper induced hemolysis, hypersplenism and hyperpigmentation as the main symptoms]. / Familiärer Morbus Wilson: kupferinduzierte Hämolyse, Hyperspleniesyndrom und Hyperpigmentation als Leitsymptome

Wilson's disease was diagnosed in a 16-year-old adolescent who presented with signs of hypersplenism due to cirrhosis, with marked hyperpigmentation of both lower legs and neurological disturbances. In view of progressive thrombocytopenia and leukocytopenia, splenectomy was performed during therapy with penicillamine later in the course, and the result was good. The patient's 12-year-old sister was found to have a hepatic form of Wilson's disease with typical biochemical findings. During the initial hospitalization a severe, spontaneous copper-induced hemolysis was noted. Another sister probably has a presymptomatic form of the disease. The parents are healthy but heterozygote carriers with regard to biochemical findings. The importance is stressed of hypersplenism, hyperpigmentation of the legs and especially of acute hemolysis in infancy as pointers in the diagnosis of Wilson's disease. Further diagnostic and therapeutic aspects are discussed.

[Sporadic adult hypophosphatemic vitamin D resistant osteomalacia (phosphate diabetes) and hyperparathyroidism]. / Sporadische hypophosphatämische vitamin-D-resistente Osteomalazie (Phosphatdiabetes) im Erwachsenenalter und Hyperparathyroidismus

Report on a patient with osteomalacia due to renal phosphate loss. At onset the patient was aged 43 years. No other tubular defects were detected except glycinuria. The patient was followed over a period of 12 years. 3 years after treatment with oral phosphate and vitamin D3 persistent hypercalcemia developed with highly elevated levels of parathyroid hormone. After removal of one enlarged parathyroid gland serum calcium normalized, but elevated parathyroid hormone levels still persist. The spontaneous occurrence of hypophosphatemic osteomalacia in an adult male and its connections with the development of hyperparathyroidism are discussed.

[Pathogenesis of bone marrow necrosis in a patient with cancer]. / Zur Pathogenese der Knochenmarknekrose beim Malignom.

Bone marrow necrosis was observed in a 23-year-old pregnant woman with severe anemia and thrombocytopenia due to bilateral ovarian carcinoma. After removal of the primary tumors, the hematological findings returned to normal, but the progression of the metastasis lead to respiratory insufficiency and death. The possible mechanisms leading to bone marrow necrosis are discussed. In our case, it could be shown that emboli of tumor cells into the vessels of the bone marrow lead to the necrosis of the marrow. The embolization was temporarily stopped after removal of the ovaries.