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248 respiratory specimens were examined using by Nested-PCR and by conventional methods (solid and liquid media). 54 specimens were culture-positive for M. tuberculosis, 51 of these were detected by PCR and conventional methods, whereas 3 were missed by PCR and 15 were only positive by PCR. 12 of the 15 culture-negative specimens derived from patients under treatment for tuberculosis. The 3 specimens, which are PCR negative could also be detected after modification of the method. The very high sensitivity depends on the high positive rate (21.8%) of the investigated specimens, so it is necessary to look also for other than pulmonary specimens, but it seems that his Nested-PCR is a highly specific and sensitive method for detecting of M. tuberculosis.

Tumor-associated antigens were demonstrated in phenol water extracts of human malignant melanoma by migration tests with leukocytes from melanoma patients and controls: in 25 out of 60 melanoma patients weak reactivity was observed, usually stimulation of leukocyte migration, while leukocytes from 37 controls were negative in 100 tests. The frequency of positive reactions did not differ significantly in patients with and without metastases. Humoral antibodies against antigens of phenol water extracts were not detectable in melanoma patients. Rabbits did not produce antibodies against tumor typical substances after immunization with phenol water extracts. Tumor associated antigens of phenol water extracts are presumably not targets of antitumor immune reactions that can influence the clinical course.

We have observed non-myelomatous monoclonal hypergammaglobulinaemia in a 61-year-old man with Sézary syndrome. Similar cases have not been reported before. Sézary cells of patients studied by other investigators were found to be helper T cells. The abundant production of T-lymphocytic stimuli may possibly explain in our case the occurrence of a paraprotein.

A functional determination of C1 esterase inhibitor (C1 INH) can be easily performed with an amidolytic assay by monitoring the inactivation of plasma kallikrein. In patients with urticaria as well as in healthy donors kallikrein inactivation, determined as the ratio of kallikrein activity at t60/t15, was found to be 0.47 +/- 0.06; in patients with hereditary angioneurotic edema (HANE). However, it amounted to 0.80 +/- 0.06. There was a good correlation between the inactivation rate of kallikrein and the protein levels of C1 INH (r = -0.93, p less than 0.001). In patients with HANE, levels of plasma kallikrein were slightly decreased (mean = 0.37 +/- 0.11 U/ml, normal mean = 0.47 +/- 0.09 U/ml), but still sufficient for monitoring kallikrein inactivation. In the case of one patient with functionally inactive C1 INH protein, the inactivation of kallikrein was impaired as in the conventional form of the disease.

Hypotheses concerning phenotypic males with a female karyotype (46,XX males) are discussed. This peculiar chromosomal anomaly may be explained either by a mutation of autosomal sex determining genes or by a translocation of Y chromosomal genes to an X chromosome or an autosome. Recent studies concerning the H-Y antigen indicate that mosaicism with cell lines containing a Y chromosome may not be made responsible for phenotypic males with a 46,XX karyotype.

We report two cases with ichthyosis vulgaris, hypogenitalism and hypogonadism. So far, little endocrinological information has been available on this association and the exact type of ichthyosis was unknown. Our first patient suffered from very severe hypergonadotropic hypogonadism, whereas the second patient showed normal levels of luteinizing hormone, but slightly elevated follicle stimulating hormone values. In lipoprotein electrophoresis we found fast moving beta-lipoproteins in the first patient and a normal electrophoretic mobility of pre- beta and beta-lipoproteins in the second patient. Correspondingly, steroid sulfatase (STS) testing revealed STS deficiency in the first patient and normal STS activity in the second patient, thus excluding X-linked recessive ichthyosis. These two different types in the association of ichthyosis with hypogenitalism and hypogonadism could not be discriminated by clinical, morphological and cytogenetic studies.

Follicular hyperkeratosis was observed in a patient with multiple myeloma. This keratosis is considered to be a cutaneous manifestation of multiple myeloma, since similar cases have been observed before. In addition, the patient had cutaneous, ocular, and articular signs and symptoms of cryocrystalglobulinemia, ie, cutaneous vasculitis, blurring, and joint swellings.

Immunoelectrophoretic studies of patients' sera occasionally reveal a parallel splitting of the IgG precipitation line if the test is performed with polyvalent antihuman antiserum directed not only against the Fc-part of the IgG molecule. This phenomenon has been studied in a patient with discoid lupus erythematosus and another patient with lymphadenopathy of unknown origin. Apparently, this finding was not due to a monoclonal gammopathy: In the cellulose membrane electrophoresis no extragradient could be observed and assays with antisera directed against free and bound light chains showed a regular pattern. The splitting of the IgG precipitate is much rather caused by polyclonal IgG with a high proportion of slowly diffusing heterogenous IgG-x, as has been found in studies using the immunoelectrophoresis as well as the analytical ultracentrifuge. It was not due to Fab fragments.

In man, apolipoprotein A-IV is characterized by a genetically determined polymorphism controlled by two codominant alleles. Two isoforms of this apolipoprotein, designated A-IV-1 and A-IV-2, can be identified by isoelectric focusing. Among 1000 healthy factory workers participating in an epidemiological study, A-IV-1 (genotype 1-1) was observed in 85%; A-IV-2 (genotype 2-2), in 0.5%; and A-IV-1 in combination with A-IV-2 (genotype 1-2), in 14%. In four nonrelated subjects, an apolipoprotein A-IV variant (A-IV-Münster), characterized by a slightly more basic isoelectric focusing behavior than A-IV-2, was detected in combination either with A-IV-1 or A-IV-2. Mendelian inheritance of this variant could be demonstrated.

The intrinsic clotting, the kinin generating and the fibrinolytic systems were investigated in 10 patients with hereditary angioedema (HAE), 10 patients with chronic urticaria and 18 healthy volunteers. In spite of the fact that patients suffering from HAE severely lack C1 INH, neither the intrinsic coagulation nor the fibrinolytic systems are impaired. There was a slight decrease of plasma kallikrein--as already known--and moreover a greater decrease in HMW-kininogen, and increase in Factor XII levels. Furthermore, activation of pre-kallikrein was delayed in these patients. These findings make it apparent that lowered HMW-kininogen levels compensate the lack of C1 INH, thus preventing an enhanced activation of the intrinsic clotting and the fibrinolytic systems.

Clinical and laboratory findings of four patients with Sézary syndrome and monoclonal gammopathy are presented. In the literature five cases with the association of these symptoms have been reported. Among the authors' series of 13 patients with Sézary syndrome, monoclonal gammopathy was observed far more often than could be expected from its natural incidence. It appears likely that monoclonal gammopathy is related to Sézary syndrome.

A patient with an immediate type hypersensitivity reaction against poppy seed is reported. Clinical symptoms consisted of swelling of the oral mucosa, vomiting, respiratory distress, and urticaria. Specific IgE antibodies were demonstrable by RAST.

Chronic myeloid leukaemia was observed 20 months after onset of pyoderma gangraenosum in a 35-year-old female. In this case an induction by cytostatic drugs could be excluded, confirming the association of pyoderma gangraenosum with leukaemias.

Mycosis fungoides was observed in a 71-year-old male with Mediterranean lymphoma, a B-cell malignancy. It is proposed that this association is not incidental since hypergammaglobulinaemia and even monoclonal gammopathies have repeatedly been described in cutaneous T-cell lymphomas. Mediterranean lymphoma might have resulted from (a) helper cell activities of tumor T-lymphocytes, (b) common antigenic stimuli, or (c) deranged T-B cooperation due to concomitant mycosis fungoides.

A monoclonal gammopathy was observed in three patients with long-term and widespread scleredema (Buschke's disease). There was no evidence of multiple myeloma in any patient. Deposition of monoclonal immunoglobulins in the skin was not detected by direct immunofluorescence microscopy. In contrast to scleromyxedema (lichen myxedematosus), from which scleredema can be distinguished clinically and histologically, the monoclonal immunoglobulins in two cases were of IgG2-kappa and IgG3-kappa type. Only one of the three patients had IgG1-lambda paraproteinemia, which is frequently seen in scleromyxedema. Our findings suggest that diffuse scleredema may be characterized by paraproteinemia but that the possible role of monoclonal immunoglobulins in the pathogenesis of this disease has yet to be resolved.

A gammaAchi--paraproteinemia was found in a 73 year-old female patient with pyoderma gangraenosum. Since the paraporteins hitherto observed in pyoderma gangraenosum predominantly belong to the IgA class, a relationship may exist between pyoderma gangraenosum and this type of paraproteinemia.

Following an acute febrile infection of the respiratory tract, a 35-year-old man suffered from livid and painful discoloration of his acral regions even after moderate cold exposure. In his serum, high titers of cold agglutinins with anti-I specificity were present. Cold-related symptoms disappeared within 3 weeks. The clinical features and the finding of anti-I cold agglutinins are compatible with postinfections cold agglutinin disease following an infection with mycoplasma pneumoniae. However, antibodies directed against mycoplasma pneumoniae were only demonstrable after the cold agglutinin-dependent symptoms had disappeared.

A case with partial lipodystrophy and osteomyelitis is presented. Low C3 levels due to C3 nephritic factor may have contributed to the development of osteomyelitis.

Paraproteinaemia (IgGkappa) was observed in a 68 year-old patient with erythema elevatum diutinum. The association of this skin disease with paraproteinaemia as already reported by other authors may indicate a pathogenetic relationship.

Purpura, shallow ulcers, and livedo were noted in a patient with IgG3-kappa cryoglobulinaemia. The IgG3 cryoglobulin reacted with all IgG subclasses. No complement activation could be demonstrated in fresh normal serum, almost complete conversion of C3, but not of factor B occurred.

The chemoattractant efficiencies of a Propionibacterium acnes (P. acnes) cell wall preparation, a P. acnes culture supernatant, and a soluble comedonal extract in the presence and absence of autologous serum for polymorphonuclear leukocytes (PMNs) have been compared in the present study. It has been found that all three preparations have no or very little chemotactic activity for PMNs in the absence of serum. In the presence of autologous serum chemotactic factors is generated by all preparations via the alternative complement pathway. The relative efficiencies of the various preparations to induce chemotactic factor by the alternate complement pathway has been evaluated. Based on the bacterial numbers of the original preparations from which the test preparations had been derived the comedonal extract appears to be more efficient in generating chemotactic factor than the other preparations. It is concluded that in vivo generation of chemotactic factors occurs mainly via the alternate complement pathway activated by soluble comedonal factors diffusing through the follicular wall.

Seminal plasma was tested against purified C1q by double diffusion. Precipitation reactions were frequently observed in the seminal plasma of patients with normozoospermia and patients with azoospermia; most of these reactions were not due to soluble immune complexes binding to C1q. A factor resistant to DNAse and RNAse (mol wt: 25,000--150,000 daltons) was found to precipitate C1q. Its discovery adds to the list of substances which may interfere in C1q precipitation tests.

Seminal proteins (IgA, IgG, albumin) were measured in normozoospermic patients and in patients with normozoospermia associated with agglomerations of spermatozoa. The type of agglomeration was not taken into account. A slight yet significant increase in seminal IgA was detected in patients with agglomerations whereas the other proteins remained unchanged. By testing seminal plasma against C1q by double diffusion it was shown that many patients of both groups had nonimmune C1q precipitins. The significance of such precipitins in semen is not known.

Hormone levels were measured in patients with Klinefelter's syndrome after treatment with 100 mg mesterolone cipionate (twice monthly). There was no difference in plasma testosterone and FSH levels in treated and untreated patients. The basal and maximum LH levels were lower, but remained raised. The urinary excretion of testosterone as measured by liquid gaschromatography was higher in treated patients after treatment was discontinued.-From these results it is concluded that in spite of reported decreases of plasma testosterone during therapy with mesterolone cipionate this drug does not lead to severe impairment of the endogenous hormone production after discontinuing treatment.