B Br ro on nc ch ho oa al lv ve eo ol la ar r a an nd d s sy ys st te em mi ic c c cy yt to ok ki in ne e p pr ro of fi il le es s i in n p pa at ti ie en nt ts s w wi it th h A AR RD DS S, ,s se ev ve er re e p pn ne eu um mo on ni ia a a an nd d c ca ar rd di io og ge en ni ic c p pu ul lm mo on na ar ry y o oe ed de em ma a (PN) and a combined group (PN+ARDS).In all patients with ARDS and/or PN, markedly elevated BAL levels of IL-6 and IL-8 were detected, which were significantly greater than levels in CPO and healthy controls. Absolute quantities and time-course of these cytokines did not differentiate between the absence and presence of lung infection, or different categories of PN. Similarly, circulating IL-6 levels were comparably elevated in patients with ARDS and/or PN, whereas circulating IL-8 concentrations were inconsistently increased. TNF-α was rarely detected in BAL samples, but increased serum concentrations were measured in ARDS and/or PN patients.Bronchoalveolar lavage levels of interleukin-6 and interleukin-8, but not tumour necrosis factor-α, and serum concentrations of interleukin-6 are consistently elevated in acute respiratory distress syndrome and/or severe pneumonia, discriminating these entities from cardiogenic pulmonary oedema. Alveolar and systemic cytokine profiles do not differentiate between acute respiratory distress syndrome in the absence of lung infection and states of severe primary or secondary pneumonia, which evidently present with comparable local and systemic inflammatory sequelae.

Twenty members of a family with adult hypophosphatasia were examined clinically and biochemically. Severe caries causing early loss of permanent teeth was the only clinical symptom which could be attributed to hypophosphatasia. None of them had a history of defective bone mineralization, rachitic skeletal alterations, and recurrent pseudofractures or fractures. An iliac crest bone biopsy of the proposita showed a normal finding corresponding to the age of the patient. Four family members in two subsequent generations were affected, thus suggesting an autosomal dominant inheritance. Their serum and leukocyte alkaline phosphatases were reduced. The phosphoethanolamine (PEA) excretion in the urine was increased to a level which suggests a heterozygote state. The serum alkaline phosphatase activity could be ascribed to the liver isoenzyme fraction. This was shown by polyacrylamide electrophoresis, by inhibition studies with organ-specific inhibitors, heat inactivation, inhibition by antibodies, and treatment with neuraminidase. The proposita had an unexplained, diffuse fatty infiltration of the liver. Thus, not only alterations of bone but also of liver metabolism in hypophosphatasia should be considered. The variety of adult hypophosphatasia described in this paper is characterized by the lack of severe bone abnormalities, the apparently autosomal dominant inheritance, and the reduction of bone and intestinal isoenzyme in the serum. Our study suggests that hypophosphatasia is a heterogeneous disorder which includes both severe and clinically mild forms.

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Innerhalb von weniger als 14 Monaten brachten 32 Kliniken 97 Patienten in eine multizentrische Studie zur Prüfung einer niedrigdosierten Interferonbehandlung der Haarzelleukämie ein. Verwendet wurden 1,2 ×10⁶ IU/m² (humanes Rekombinanten-Interferon-Alpha 2c arg (Hr IFn – 2c (arg)) x 28 Tage s.c. mit vereinbarter Reduktion der Dosis nach Eintritt einer klinischen Besserung oder eine ultraniedrige Dosis von 1,2×10⁶ IU/m² s.c. täglich und sich steigernd bis zu einer gut tolerierten Dosis. Die Induktionsphase war auf 84 Tage begrenzt. Patienten, die in diesem Zeitraum das klinische Stadium Jansen I oder A erreichten, wurden randomisiert und in zwei Armen der Erhaltungstherapie zugeführt. Ein Teil der Patienten erhielt wei-terhin Interferon (2× wöchentlich), die anderen wurden ohne Therapie belassen. Für alle Patienten sollte die Therapie wieder eingesetzt werden, wenn die Jansen-Stadien A oder I verlorengingen. Die Studie umschloß obligatorische zentrale diagnostische Maßnahmen zur Sicherung der Diagnose und Messung des Therapieerfolgs. Während der Laufzeit entwickelte sich eine besondere Problematik in der Gruppe der 34 Patienten mit wiederkehrendem

Extensive ‘vacuolization’ could be demonstrated in nearly all plasma cells and in some lymphocytes of an adult with glycogenosis type II (Pompe’s disease). The biochemically defined diagnosis acid maltase deficiency (AMD) could be ascertained by examination of the maltase activity of the patient’s leukocytes. Elecron microscopical, microspectrographic, and cytochemical investigations revealed electron denseinclusions, which show an UV absorption at 276 nm and a positive reaction after PAS staining of plastic embedded material. Since no other abnormalities of the plasma cells could be found, our results are presumably indicative for a connection of AMD and a glycoprotein storage in the plasma cells of the patient.