[Primary sclerosing cholangitis]. / Die primär-sklerosierende Cholangitis.

In a 40-year-old patient unexplained recurrent attacks of epigastric colic with transient cholestatic icterus occurred over a 9-year period. When the patient was again hospitalised because of progressive pain-free icterus associated with mild pruritus (alkaline phosphatase 900 U/l, direct bilirubin 305 mumol/l, GOT 187 U/l, GPT 103 U/l) sonography revealed liver enlargement to 17 cm, extended intrahepatic bile ducts and an echodense area of about 1 cm size in the region of the bifurcation of the common hepatic duct. Fine-needle puncture did not yield clear cytological findings. Endoscopic retrograde cholangiopancreatography pointed to sclerosing cholangitis. This diagnosis was confirmed by liver punch biopsy. Since the patient did not agree to a liver transplantation, he was treated with 450 mg ursodeoxycholic acid twice daily, resulting in marked reduction of the liver parameters until severe cholangiosepsis and acute renal failure occurred about 4 months later. The septic condition and its complications could not be managed despite thorough intensive-care measures so that a liver transplant had to be performed after all. Histology of the explantate revealed a cholangiocarcinoma in the region of the bifurcation of the common hepatic duct. At first the patient's condition improved markedly but one and half months later the transplant was rejected and the patient died.

[Eosinophilic fasciitis (Shulman's syndrome)]. / Eosinophile Fasziitis (Shulman-Syndrom).

A 36-year-old man had noted a firm and painful swelling of the upper and lower limb, increasing over the previous few weeks. He had no fever, but the erythrocyte sedimentation rate was slightly increased (20 mm in the first hour). In addition there was a mild normochromic, normocytic anaemia (13 g/dl) and thrombocytosis (517,000/microliters). Gamma-globulin fraction was raised to 26%. The blood eosinophilia of 44% and the histological findings in a wedge biopsy of the lower leg (oedematous widened connective tissue septa, inflammatory infiltrate with eosinophilic granulocytes) established the diagnosis of eosinophilic fasciitis (Shulman syndrome). During oral treatment with prednisone (100 mg/d) the clinical symptoms regressed and the eosinophilia in peripheral blood disappeared. In the course of the subsequent seven months the prednisone dose was reduced gradually to 10 mg daily. But, because the symptoms did not entirely disappear the prednisone dose had to be increased temporarily for four times until there was a further, stepwise, reduction of the symptoms. But mild induration of the lower leg persisted. Maintenance treatment with prednisone, 10 mg daily, has been continued in order to avoid recurrence.

[Paroxysmal non-hereditary angioedema]. / Paroxysmales nicht-hereditäres Angioödem.

Recurrent hypovolaemic shock had been occurring over the last five and four years, respectively, in a 53-year-old woman and a 46-year-old man who had previously been healthy. The attacks were characterized by a tension feeling and sometimes oedema in the limbs, as well as increased thirst. Within a few hours sweating, tachycardia, orthostatic complaints and shock would occur. The woman's systolic blood pressure would fall to 70 mm Hg and the pulse rate rise to 150/min. The man's blood pressure was not measurable by sphygmomanometer during his first attack. Haematocrit rose to 61 and 71.5%, haemoglobin concentration to 20.7 and 21.3 g/dl, respectively. On administration of plasma expanders all abnormal clinical and biochemical changes quickly disappeared, only to recur within weeks or months. The cause of the condition is an increased permeability of the tissue capillaries, while renal, pulmonary and cerebral vessels apparently are unaffected. During ketotifen and tebonin (gingko biloba extract) administration to the man, he required no further hospitalization for nine months, after which he had three severe attacks. The woman had a severe attack of hypovolaemic shock one month on this treatment. The prognosis of capillary leak syndrome is bad.

[Intestinal drug smuggling--a new diagnostic and therapeutic problem]. / Intestinaler Rauschgiftschmuggel--ein neues diagnostisches und therapeutisches Problem.

Since spring 1985 81 persons were examined because of suspected intestinal drug-smuggling (body-packing). 46 patients (57%) had drug-packages in the gastro-intestinal tract, in 35 persons (43%) the suspicion was not confirmed. 26 patients had swallowed the drugs (mostly cocaine) whereas in 20 cases drugs were found in the rectum (mostly heroine). Most important for diagnosis was abdominal x-ray whereas in our hands abdominal sonography was unreliable. When body-packing is diagnosed, observation and mild laxative in conjunction with sufficient beverages are recommended. Serious complications in our series comprised one patient with mechanical ileus and another with heroine-poisoning after rupture of a package.

[Idiopathic cold agglutinin disease. Clinical aspects, therapy and course in 6 patients]. / Die idiopathische Kälteagglutininkrankheit. Klinik, Therapie und Verlauf bei 6 Patienten.

Clinical features, diagnostic procedure, therapy, course of the disease and prognosis in 6 patients with severe idiopathic chronic cold agglutinin disease are described. In 5 patients the main complaint was cold mediated acrocyanosis. The cold agglutinin in all patients was of anti-I type and belonged to IgM immunoglobulin. Keeping warm provided symptomatic relief and the hemolysis decreased to a milder form. Treatment with glucocorticoids alone failed in two patients but succeeded in combination with chlorambucil or cyclophosphamid. One patient developed a lymphoproliferative disorder 11 years after diagnosis of idiopathic chronic cold agglutinin disease.

[Principles of modern antibiotic therapy in general practice and the clinic]. / Grundsätze moderner Antibiotikatherapie in Praxis und Klinik.

It is reported on the principles of modern therapy with antibiotics and here above all is referred to the differences in the antibiotic treatment in practice and clinic. Issuing from the infections usually occurring in practice with the spectre of causative agents known in many cases the therapeutic possibilities are discussed also taking into consideration economic points of view. In contrast to this in infections in the clinic, so-called nosocomial diseases, changing situations are present, which need a therapy on the basis of the antibiogramme. In these cases partly also combination therapies for the enlargement of the spectre of action and for the increase of the antibacterial activity are used.

[Which uric acid value is in need of treatment?]. / Welcher Harnsäurewert bedarf der Behandlung

Asymptomatic hyperuricemia should be treated only if the plasma uric acid levels are around 10 mg/100 ml or more on several determinations. In addition, patients on a purine-free diet who excrete more than 600 mg uric acid per 24 h should be treated. In both cases, treatment is intended to be prophylactic against gouty nephropathy. At present there is no evidence that primary hyperuricemia alone is a risk factor for early atherosclerosis and especially coronary artery disease. However, more attention should be paid to the accompanying risk factors such as obesity, hyperlipoproteinemia, diabetes mellitus and hypertension.