Rapidly progressive glomerulonephritis accompanying Legionnaires' disease.

A rapidly progressive, crescentic glomerulonephritis with acute oliguric renal failure occurred simultaneously with legionnaires' disease (LD) in a 52-year-old man. The diagnosis of LD was based on a sixfold rise in indirect fluorescent antibody titer against Legionella pneumophila serogroup 4. Treatment with erythromycin lactobionate resulted in a clinical resolution of pulmonary manifestations. The impairment of kidney function, however, was progressive and within two weeks led to end-stage renal failure requiring regular hemodialysis. This observation suggests that LD may trigger severe acute glomerulonephritis.

Role of immune complexes in pathogenesis of renal disease.

When judged by histological criteria, kidney tissue is practically devoid of immune tissue; as yet a broad spectrum of immunological diseases are targeted at the kidneys, especially at the glomeruli. Glomerular epithelial cells exhibit immune protein receptors and hence could clear immune complexes decorated with complement from the circulation. Alternatively, circulating immune complexes could become trapped in the glomerular filter and start off inflammatory reactions. Anti-glomerular basement membrane antibody reacts with glomeruli and causes glomerulonephritis. The present text is an analysis of the impact of immune complex formation on renal pathology and contains retrospective clinical data of our own hospital obtained on 192 patients undergoing diagnostic renal biopsy.

Paramyotonia congenita. A clinical, electrophysiological and histological study of 12 patients.

The present paper describes the clinical, electrophysiological and histological findings made in 12 patients belonging to 2 genealogical lineages in Switzerland, suffering from paramyotonia congenita. This is the first report of this disease in Switzerland. The myopathy, transmitted by autosomal dominant inheritance, is characterized by a typical past medical history and by the persistent contraction of the muscles of the face, arms and legs provoked by exposure to cold. Also of diagnostic importance are the myotonic reactions inducible at room temperature, such as percussion myotonia, active myotonia and paradoxical myotonia. During paramyotonic episodes provoked by exposure of the subjects in a refrigeration chamber, serum potassium concentrations remained within normal limits. Potassium loading producing serum levels above 6 mEq/1 in 2 patients gave rise to stiffness and weakness of the arms and legs, but no signs of paralysis. Induced hypokalemia in 3 cases caused no paramyotonic symptoms. Electromyographic recordings in 5 patients showed myotonic discharges, which disappeared upon cooling of the limb, giving way to progressive muscular stiffness. Histological, histochemical and electronmicroscopical examination of the muscle tissue revealed only diagnostically unspecific myopathological changes.

Biocompatibility of different hemodialysis membranes: activation of complement and leukopenia.

The ability of three hollow-fiber dialyzers (Cuprophane [CU], polymethylmethacrylate [PMMA], and polyacrylonitrile [PAN]) to activate complement and to induce leukopenia was studied prospectively in six patients on long-term hemodialysis. CU membranes caused the most intense complement activation with C3a, C3d, and C5a levels peaking 15 min after beginning dialysis. Total white blood cell (WBC) counts dropped simultaneously by 76%, and the decrease in leukocytes was inversely correlated with the levels of C3a and C5a. In contrast, PMMA membranes led only to slight complement activation with an associated fall in WBC counts of 29%, and PAN membranes induced very little complement activation without leukopenia. In vitro studies involving incubation of normal human plasma with each of the three membranes corroborated these findings. The results suggest that the biocompatibility of PMMA and PAN dialyzers is superior to CU.

[Current therapy of nephrotic syndrome]. / Aktuelle Therapie des nephrotischen Syndroms.

The etiology and therapy of the nephrotic syndrome are reviewed. Current therapeutic procedures include symptomatic and causal treatment with steroids and/or cytotoxic drugs, as well as some new modalities of therapy.

[The therapeutic significance of plasmapheresis in immunologic diseases]. / Die therpeutische Bedeutung der Plasmapherese bei Immunkrankheiten.

By its rapid and efficient elimination of pathogenic antibodies, antigen-antibody complexes, inflammatory mediators and toxic factors, plasmapheresis may favorably influence the course of several immune diseases. The treatment should be confined to patients who do not react to more conservative therapy, i.e. steroids and/or immunosuppressive agents. Should this attempt at conservative therapy fail, however, plasma exchange should be installed immediately, particularly in severe disease with marked progression or if dosage levels of immunosuppressive drugs must be reduced because of toxicity.

Effect of intravenous immunoglobulin therapy on plasma complement.

The effect of the intravenous immunoglobulin preparation (Sandoglobulin) on the complement system and the formation of circulating immune complexes (CIC) was investigated in 10 patients with primary humoral immunodeficiency and in 12 normal volunteers. Infusion of 9 g of the pH4 treated preparation did not show relevant changes in total hemolytic complement activity, plasma levels of C4, C3, and factor B. C3dg fragments, however, increased markedly in some individuals in both groups immediately after infusion. CIC measured by 125 I-C1q binding were not detectable. Furthermore, the initially low C1q concentrations in immune-deficient patients correlated with the pre-infusion IgG levels (p < 0.05) and rose significantly 24 h after therapy (p < 0.001). In contrast, in vitro incubation of the immunoglobulin preparation with normal human serum did not reveal spontaneous anticomplementary activity nor did it lead to formation of immune aggregates. The results indicate that immunoglobulin infusions may moderately activate complement in vivo, as evidenced by increased C3dg fragments; in addition, partial C1q deficiency in hypogammoglobulinemia seems to be related to IgG concentration and can be corrected by immunoglobulin substitution.

[Primary retroperitoneal seminoma--a rare germ cell neoplasm]. / Primär retroperitoneales Seminom--ein seltenes Keimzellneoplasma.

A 44-year old male presented himself with left-sided abdominal pain. On clinical examination a painless, enlarged supraclavicular lymph node on the left side and small atrophic testes were detected. Ultrasonography revealed a huge retroperitoneal mass. By biopsy of the retroperitoneal tumor the diagnosis of a seminoma was made, but neither in the orchidectomy specimen of the right side nor in the testicular biopsy of the left side a primary tumor or a scar could be identified. Thus, the diagnosis of a extragonadal primary retroperitoneal seminoma was made. The patient responded well to the combined chemotherapy consisting of cisplatin, bleomycin and etoposide. For distinction from an occult and/or burned-out seminoma testicular biopsy or surgical exploration of the testes with histology are mandatory.

[Activation of the complement system in different forms of glomerulonephritis]. / Komplementaktivierung bei Glomerulonephritiden.

The complement system may be activated by two pathways, the classical and the alternate. To evaluate their respective participation in different forms of glomerulonephritis, the plasma values of C3, C4, C3PA, C1q and properdin were determined in 70 patients. In systemic lupus erythematosus (LED), acute poststreptococcal glomerulonephritis (AGN) and septicemia the classical pathway appears to be mainly involved, whereas the amplification loop and the alternate pathway seem to be of secondary importance. By contrast, in membranoproliferative glomerulonephritis (MPGN) the alternate pathway plays a major role. However, the present data suggest that activation of the classical pathway may often be involved as well. In minimal change glomerulonephritis no signs indicating involvement of the complement system were apparent. Follow-up observation demonstrated a correlation between decreases in plasma complement concentrations and the clinical severity of the primary disease in LED, AGN and septicemia, but not in MPGN.

Complement components, degradation products and immune complexes after kidney transplantation.

Levels of complement components and the presence of immune complexes were determined in blood samples from 23 patients is a function of time after kidney transplantation. During the first three post-transplantation weeks a decrease in the concentration of plasma C3 with a simultaneous increase of one of its breakdown products (C3d) was generally observed. This pattern often accompanied acute rejection episodes beyond 4 weeks after transplantation, while in the absence of complications normal and stable levels prevailed. In contrast, the presence of circulating immune complexes appeared not to correlate with rejection reactions. All 7 cases with detectable immune complexes presented with various concomitant neoplastic (renal carcinoma, Kaposi sarcoma) or infectious diseases (pneumonia, septicaemia, Herpes zoster or Cytomegalovirus infection). Thus, monitoring of plasma C3 and C3d may represent a helpful additional criterion for the assessment of acute rejection in recipients of kidney allografts; the presence of circulating immune complexes, although not correlating with graft rejection, may be taken as a sign of complicating additional disease.

[2 cases of acute sickle cell crisis in subjects with sickle cell trait following high altitude exposure]. / Zwei Fälle akuter Sichelzellkrisen bei Sichelzell-Heterozygoten nach Höhenexposition.

We present two patients with sickle cell trait (HbAS) who developed acute sickle cell crisis after passive exposure to high altitude on the Jungfraujoch (3454 meters, 11,333 ft). Both suffered splenic infarction and the first patient also presented with acute renal failure. Splenic infarction is known to be a complication of altitude exposure in carriers of the trait, whereas acute renal failure due to altitude exposure alone has as far as we know never been described in the literature. These forms of high altitude complications are totally unusual in Switzerland.

[Malignant non-Hodgkin's lymphoma with visual disorders and nephrotic syndrome]. / Malignes Non-Hodgkin-Lymphom mit Visusstörungen und nephrotischem Syndrom.

A favourable outcome is reported in a 65-year-old patient with malignant non-Hodgkin lymphoma, in whom early manifestations were papillitis and nephrotic syndrome due to membranoproliferative glomerulonephritis. The pathogenesis of the ophthalmologic and renal disease may be associated with the malignant lymphoma. Under cytostatic treatment (prednisone, chlorambucil, vincristine) the malignant lymphoma went into still-lasting remission accompanied by a normalization of the ophthalmologic and renal functions.