[A female patient with splenomegaly, interstitial pneumopathy and giant foam cells in bone marrow]. / Eine Patientin mit Splenomegalie, retikulärem Lungenbild und Schaumzellen im Knochenmark.

We describe a case of Niemann-Pick disease type B. A 13-year-old female adolescent of Turkish origin suffered from abdominal pain for several months, finally leading to hospitalisation. The investigations revealed splenomegaly and interstitial pneumopathy. The bone marrow contained giant foam cells typical of Niemann-Pick disease. Enzymatic analysis of a fibroblast culture confirmed the diagnosis of Niemann-Pick disease type B, with reduced activity of acid sphingomyelinase. Niemann-Pick disease is an inherited autosomal recessive lysosomal storage disorder of sphingolipids, resulting in an accumulation of sphingomyelin in the cells of the reticulo-histiocytic system due to an enzymatic defect. In Niemann-Pick disease type B the spleen and lung are the main organs affected. At present no treatment exists for this disorder.

[Respiratory insufficiency and absent left radial pulse after hemicolectomy]. / Respiratorische Insuffizienz und fehlender Radialispuls links nach Hemikolektomie.

A 65 year old female developed right thoracic pain, productive cough and fever four weeks after hemicolectomy because of a cancer of the sigmoid. In spite of antibiotic treatment the condition of the patient deteriorated and she was admitted to the hospital with pneumonia of an upper lobe. Chest X-ray visualized prominent proximal pulmonary arteries. Progressive respiratory failure developed and blood gas analysis revealed hypocapnic hypoxemia. The patient had to be intubated and ventilated mechanically. Later, left arm blood pressure measurements could no longer be taken and the radial pulse was missing. Thereafter, an ischemic syndrome of the right leg developed. Embolectomy from the superficial femoral artery was carried out the same day. The patient died five days later. Autopsy revealed an almost complete occlusion of the pulmonary arteries. The organization of thrombotic material indicated recurrence. Emboli were also found in the systemic circulation. A large patent foramen ovale together with signs of pulmonary arterial hypertension are indicative of paradoxical thromboembolism.

Familial transmission of a serious disease--producing group A streptococcus clone: case reports and review.

Invasive group A streptococcus (GAS) infections are emerging diseases; however, person-to-person transmission of invasive GAS producing life-threatening infection has been observed rarely. We report a small intrafamilial cluster of life-threatening GAS infections. A previously healthy 47-year-old father developed necrotizing fasciitis of the neck. Two days later, his 16-year-old daughter developed streptococcal angina, pneumonia, and pleural empyema. Both patients had signs of streptococcal toxic shock syndrome. Pulsed field gel electrophoresis revealed that the M6 strains of GAS isolated from the father and daughter had identical patterns. Cases of person-to-person transmission of invasive GAS infection reported in the literature are also reviewed.

Letter from Yekaterinburg.

The Russian city of Yekaterinburg, known as Sverdlovsk before the influence of perestroika, lies at the eastern foot of the Ural Mountains on the border between Europe and Asia. It was the site of large armaments factories, most of which are now closed, but the area continues to feel the aftermath of their heavy pollution. The annual Congress of the Russian Society of Internal Medicine was held here in 1995 and was attended by physicians from all corners of this vast country. Leaders in the field of internal medicine from Russia presented state-of-the-art papers on many aspects of the theme of the conference, Emergencies in Internal Medicine. The problem of the acquired immunodeficiency syndrome was also discussed, but this subject is still taboo as far as the official health authorities are concerned. The Russian physicians talked about how they deplored the great shortage of disposable medical equipment. One of the most serious health problems in Russia is alcoholism; surprisingly, this problem was hardly mentioned at the congress. An open international exchange of information is again possible in Russia, but financial considerations restrict attendance at meetings abroad and the availability of professional journals domestically.

[Evaluation of the QBC-Autoread System in hematological screening in a hospital laboratory]. / Evaluation des QBC-Autoread-Systems zum hämatologischen Screening in einem Spitallaboratorium.

The QBC Autoread System is based on fully automatic, electro-optical linear measurements of the discrete layers of packed blood cells in a microhematocrite tube. The layering results from differing cell densities during high-speed centrifugation. The determination of hemoglobin, hematocrit, leucocytes, platelets and of the percentual share of granulocytes and lymphocytes/monocytes with the QBC Autoread System was evaluated concerning reliability and precision in comparison with the conventional analytical methods. We used a Coulter Counter System and the manual differentiation of the stained blood smears as reference methods. The evaluation of the first 400 blood samples showed a close correlation for all hematological parameters examined, but no significant deviation relative to the reference methods. Only 83 of totally 1000 (8.3%) examined venous blood samples could not be evaluated completely by the QBC Autoread System. For the analysis of very pathological cell populations, especially of hematological oncological diseases, the QBC System is not qualified. The extreme portability, the quick practicability and the good accordance with the conventional analytical methods make the QBC Autoread System a qualified screening method for practice and emergency laboratories.

Poststreptococcal glomerulonephritis flare-up in a patient with varicella infection.

A 15-year-old boy with poststreptococcal glomerulonephritis is described. During his recovery from nephritis he acquired varicella, which led to a flare-up of the glomerulonephritis with a transitory increase in blood pressure, body weight, and serum creatinine. We can only speculate on the pathogenetic mechanism of the flare-up. The glomerulonephritis may have been reactivated by immune complexes containing varicella antigens, or the varicella infection may have provoked immunologic changes leading to a new burst of immune complexes containing streptococcal antigens.

Acute tubulo-interstitial nephritis with uveitis (TINU syndrome) in a patient with serologic evidence for Chlamydia infection.

A 38-year-old female patient with acute tubulo-interstitial nephritis and uveitis (TINU syndrome) is described. The cause of this rare disease is unknown; most patients affected are adolescent females. Serologic investigations in the patient reported point to a possible etiologic role of Chlamydia infection in this disease.

[Petechiae, microhematuria, colic-like abdominal pain]. / Petechien, Mikrohämaturie, kolikartige Abdominalschmerzen.

A 46-year-old patient was referred because of recurring bouts of abdominal colics, petechiae and an accompanying nephropathy. The clinical setting pointed towards a cryoglobulinemia, proved by serum cryoprecipitation and electrophoresis and classified as type II. The primary disease was a low-grade non-Hodgkin lymphoma involving bone-marrow and liver. A renal biopsy showed a proliferative glomerulonephritis. Treatment with prednisone and chlorambucil led to improvement of renal function, and the patient became asymptomatic.

[Oxidated sterols--a contributing factor in atherogenesis?]. / Oxidierte Sterole--ein Teilfaktor bei der Atherogenese?

Sterols are not only important metabolic precursors, they are also indispensable structural components of cell membranes. There is some evidence that synthesis of cholesterol is regulated by oxidized derivatives of cholesterol. They are able to inhibit the key enzyme of cholesterol synthesis, i.e. 3-hydroxy-3-methylglutaryl-CoA-reductase. In addition, oxidized sterols are inserted into the lipid bilayer of the cell membrane as cholesterol analogues. This insertion results in serious disturbances of morphology and function of the membrane. In animal models oxidized sterols have angiotoxic effects; they are also found in atheromas of human arteries. Therefore they may be a causative factor in atherogenesis.

[Lidocaine treatment of poisoning with yew needles (Taxus baccata L.)]. / Lidocainbehandlung einer Vergiftung mit Eibennadeln (Taxus baccata L.).

A 40-year-old patient attempted suicide by drinking an extract made from 120 g of yew needles. Shortly after severe ventricular arrhythmia occurred characterized by extremely prolonged QRS-intervals. Recurrent episodes of ventricular tachycardia were successfully treated with high doses of lidocaine. In most cases intoxication by yew needles ends fatally. Lidocaine may be the drug of choice to treat life-threatening tachyarrhythmia in such cases.

[Provisional treatment results of intensive combination chemotherapy (m-BACOD) in non-Hodgkin lymphoma of intermediate and high malignancy]. / Vorläufige Behandlungsergebnisse durch intensive Kombinationschemotherapie (m-BACOD) bei Non-Hodgkin-Lymphomen (NHL) mittlerer und hoher Malignität.

10 patients with diffuse large cell lymphoma were treated with 10 cycles of m-BACOD. 7 patients presented with a pathological stage IV, 2 with a clinical stage III and one with a clinical stage II disease. 8 patients have been in complete remission for 3-21 months (mean 9 months) after completion of therapy. One patient with T-immunoblastic lymphoma and bulky mass died of progressive disease after 6 cycles of m-BACOD. A second patient with T-immunoblastic lymphoma relapsed 24 months after completion of therapy. Treatment-related major complications, especially MTX-related ulcerative stomatitis, did not occur.

Toxoplasmosis in hairy-cell leukaemia.

Toxoplasma serology was performed in 28 patients with hairy-cell leukaemia and was positive in eight patients (29%). In two patients (7%) reactivated toxoplasmosis was proven by either isolation of Toxoplasma gondii or by significant antibody titre rise with generation of specific IgM-antibodies. In four patients (14%), a clinical diagnosis of active toxoplasmosis was based on signs and symptoms, serologic tests, and response to specific treatment. The high proportion of patients in which active toxoplasmosis was proven or probable (six; 21%) may be related to the presence of severe monocytopenia. In patients with hairy-cell leukaemia developing fever of unknown origin and myositis, toxoplasma serology should be performed, particularly because treatment of active toxoplasmosis usually is successful.

Central nervous system involvement in hairy cell leukemia.

A patient with central nervous system involvement by hairy cell leukemia is reported. Hairy cells were identified in the cerebrospinal fluid by electron microscopy and by tartrate-resistant acid phosphatase positive staining. Intrathecal treatment with methotrexate resulted in neurologic improvement, but was complicated by Cryptococcus neoformans meningitis. The leukemic phase of the disease was later successfully controlled by treatment with alpha interferon. Surface marker studies indicated a B- and T-cell phenotype of the hairy cells.