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W Pernice

Publications and source records attributed to W Pernice.

15 recordsLinked to original sources
PubMed1991

[Tuberculous arthritis--a rare, but important differential diagnosis in juvenile chronic arthritis].

We observed a 13 year old Turkish boy suffering from chronic arthritis of the left knee since 1983. Clinical symptoms as slow progression of the disease and laboratory data including a positive HLA B 27 test suggests the diagnosis of juvenile chronic arthritis. A tuberculous arthritis was initially excluded by X-ray examination of the chest. The positive tuberculin test was attributed to the BCG vaccination. Because of insufficient response to antiinflammatory drug therapy a synovectomy was performed for diagnostic as well as therapeutic reasons. Histopathological results suggested sarcoidosis. A second synovial biopsy of the affected joint revealed granulomas combined with multiple necrosis typical for tuberculous infection. The animal experiments showed positive results. Tuberculostatic therapy was successful with INH, myambutol and rifampicin. Joint function and MRI results markedly improved.

Adolescent↗
PubMed1989

Therapy for systemic juvenile rheumatoid arthritis with gamma-interferon: a pilot study of nine patients.

Nine severely ill patients with a confirmed diagnosis of systemic juvenile rheumatoid arthritis were treated with recombinant gamma-interferon (gamma-IFN) in addition to the therapy they were previously receiving for their disease. Improvements in clinical symptoms were noted in 7 of the patients, and median laboratory values also showed a marked improvement after gamma-IFN treatment. A relapse occurred in 1 patient. The results of this study should stimulate further research on the use of gamma-IFN in systemic juvenile rheumatoid arthritis, particularly in determining the appropriate effective dosage.

Arthritis, Juvenile↗
PubMed1989

Hereditary progressive muscular dystrophies: serum myoglobin pattern in patients with different types of muscular dystrophies.

Serum myoglobin (Mb) levels and creatine kinase (CK) activity were investigated in patients with different types of progressive muscular dystrophy and controls. The Mb levels were determined by radioimmunoassay and found to be significantly elevated in all patients under resting conditions. There was no correlation between Mb levels and CK activity. Physical exercise was followed by an increase in Mb levels and CK activity in patients and a minor variation in controls. Isoelectric focusing, electroblotting and application of a specific Mb antibody (rabbit anti-human Mb) make it possible to recognize marked differences between the Mb bands of patients and controls. All patients with progressive muscular dystrophy had an additional fourth Mb band (isoelectric point pH 6.3) in contrast to controls with three Mb bands.

Creatine Kinase↗
PubMed1988

A double-blind placebo controlled trial of diltiazem in Duchenne dystrophy.

The role of calcium accumulation in the pathogenesis of Duchenne muscular dystrophy (DMD) has already been discussed. Several trials with different calcium-blocking drugs have revealed no clinical benefit. In addition, the present study includes histological investigations and computer tomography to verify therapeutic effects. In a randomized placebo-controlled double-blind study, 13 DMD patients aged from 3-10 years (mean, 7 years) were treated with 5 mg/kg diltiazem daily for 1 year. Compared with before therapy, the number of calcium-positive muscular fibres was remarkably reduced in the treated DMD patients, but not in the placebo group. The evaluation of all other biochemical and clinical parameters revealed no significant effects of the diltiazem therapy. The muscular X-ray density measured by computer tomography decreased under treatment. After the evaluation of the double-blind study, the code was broken. Therapy, however, was continued in the treated group and started in the placebo group. After 3 years of diltiazem therapy the clinical status of all 26 patients of the study and 20 additional DMD patients who were treated with diltiazem was compared with 46 untreated DMD patients of the same age and stage in our department. No obvious clinical benefit of diltiazem therapy could be observed.

Calcium↗
PubMed1986

A mathematical analysis of creatine kinase activity in the course of Duchenne muscular dystrophy.

Recently, clinical follow-up studies have shown that the age of first symptoms and the speed of progress may vary considerably in Duchenne muscular dystrophy (DMD). Prognostic factors would be of interest not only for the patients themselves, but also for the selection of DMD patients for therapeutic studies. Creatine kinase (CK) activity reflects the dystrophic degeneration of the muscle cells. Therefore, the coherence of the course of CK activity and its relationship to the clinical state was investigated with the aid of a heuristic mathematical formula. Two constants were calculated, b and c. The data of 88 DMD patients proved the validity of the mathematical formula. Constant b seemed to be of no clinical relevance. The height of constant c was related to the age at which the ability to walk was lost (r = -0.76). This constant provides an objective individual assessment of the prognosis in DMD. In addition, the mathematical analysis of the CK course opens the way for a prospective CK estimation, which is relevant for the evaluation of therapeutic trials.

Adolescent↗
PubMed1985

[Improved immunocompetence in two children with chronic Epstein-Barr virus infection under treatment with a standardized thymus hormone preparation (thymostimulin)].

Two children with chronic Epstein-Barr virus infection were treated with a standardized thymic hormone preparation (thymostimulin, TP-1 Serono). The treatment was well tolerated. No adverse reactions, no side effects were observed. Results of immunological investigations showed a trend towards normalisation. Additional clinical evidence showed up, which pointed to a response to the therapy.

Child↗
PubMed1985

[Persistent Epstein-Barr virus infection].

2 boys aged 4 and 6 1/2 years and a 1 1/2-year-old girl in whom persistent EBV-infections developed are described. Serological investigations showed a markedly increased IgG-antibody titer against the virus-capsid antigen (VCA-IgG). Furthermore there was a persistence of anti-early-antigen-antibodies (anti-EA-antibodies) resp. VCA-IgA indicating a chronic infection. The observation period was 1 to 4 years. Cellular immunity in these children was depressed. They show the typical clinical symptoms of infectious mononucleosis; additionally they often suffer from other infectious diseases.

Antibodies, Viral↗
PubMed1979

[Henoch-Schoenlein purpura (author's transl)].

Routine EEG investigations and observance of discrete neurological and psychological symptoms in 13 children in the acute phase of Henoch-Schoenlein purpura showed that involvement of the central nervous system in this disease is the rule rather than the exception. Capillary resistance was reduced in 51 out of 76 investigated children. On the other hand a reduction in factor XIII activity was much less commonly found (n =6). Immune complex determination in 28 children, together with antibody studies, showed that in 13 of them the Henoch-Schoenlein purpura was triggered off by an influenza-A-virus infection of the upper respiratory tract. Two thirds of the patients had markedly raised levels of total serum complement which fell weeks within several.

Adolescent↗
PubMed1979

Antigen-specific detection of soluble immune complexes by a solid phase specific antibody system.

An antigen-specific immune complex assay based on the following principle has been developed: xenogeneic, antigen-specific antibodies are attached to a solid phase. During first incubation with patient's serum, immune complexes in antigen excess are bound to the xenogeneic antibody by their free antigenic determinants. In a second incubation a labeled antibody, specific for human immunoglobulins, is combined with the antibody part of the immune complex. Quantitation of the label allows the determination of the immune complexes. The principle of the method has been varied using artificial soluble immune complexes of tetanus toxoid and human anti-tetanus toxoid antibody, and immune complexes prepared by mixing patient sera containing either HBsAg or anti-HBsAg antibodies. The reliability of the results is shown by their coefficient of variation (2.5%). With the method described soluble immune complexes predominantly in slight antigen excess, which are thought to be responsible for development of immune complex disease, have been detected.

Antibody Specificity↗
PubMed1979

Antigen-specific detection of HBsAG-containing immune complexes in the course of hepatitis B virus infection.

In recent studies extrahepatic manifestations of viral hepatitis have been recognized as immune complex diseases. Hepatitis B surface antigen (HBsAg) has been successfully identified in immune complexes, but the pathogenic role of HBsAg-containing immune complexes (IC) remains questionable. The subject of the present study was the antigen-specific determination of IC in the course of hepatitis B virus infection using a new HBsAg-specific IC test (Pernice & Sedlacek, 1978). This test is based on the following principle: rabbit anti-HBs-coated polystyrole test tubes are incubated with the IC-containing test sample. The HBsAg-containing IC bind to the solid phase by their free antigenic determinants. There they can be quantified using a peroxidase-labelled anti-human IgG antibody. A good correlation was found between the level of HBsAg-containing immune complexes and the clinical state of six patients in a follow-up study. IC could be detected simultaneously with HBsAg and either decreased or disappeared before the occurrence of free anti-HBs. In the sera of an additional twenty-eight patient suffering from chronic active hepatitis, HBsAg-containing immune complexes were detected in 85% of cases. One patient suffering from polyarteritis nodosa was also positive. Occasionally, extremely high levels of IC were found in the course of these diseases.

Antigen-Antibody Complex↗
PubMed1978

[Baby hamster kidney cells as antigen for demonstration of antinuclear antibodies (author's transl)].

Baby hamster kidney cells fixed in acetone on glass slides were used as antigen for demonstration of antinuclear antibodies. Where certain storage conditions were observed (drying agent, 4 degrees C) they have kept for 12 months up to now. As regards specificity, sensitivity, reproducibility, and differentiation of fluorescent types the baby hamster kidney cell test appears superior to other immunofluorescence methods used (chicken erythrocytes, rat liver sections, and crithidiae). These results were obtained in 73 sera from patients with disseminated lupus erythematodes, drug-induced lupus erythematodes, discoid erythematodes, allergic vasculitis, progressive scleroderma, dermatomyositis, and 36 control sera.

Antibodies, Antinuclear↗
PubMedDate not supplied

[Enzyme diagnosis in progressive muscular dystrophies, especially in the Duchenne type].

The serum activities of muscular enzymes are important for the diagnosis and follow-up of muscular diseases. They reflect the degeneration of muscular tissue. The serum enzyme activities (creatine kinase (CK), creatine kinase MB (CKMB), Aldolase (ALD), Lactat-dehydrogenase (LDH), a hydroxy-buty-rat-dehydrogenase (a-HBDH), glutamat-oxalacetat-transaminase (GOT), and glutamat-pyruvat-transaminase (GPT) in Duchenne muscular dystrophy show an increase during the first three years of life, a maximum between the age of 3 and 4 and an asymptotic decline thereafter. Taking the blood samples under non-standardised conditions the coefficients of variation (VK) differed considerably. For a -HBDH the lowest value was obtained (VK = 0.37). The VK-value of ALD was comparatively high (VK = 0.68). The correlation among the enzymes themselves was high and a-HBDH correlated closest to LDH and a-HBDH also to GPT. Thus we conclude that LDH, ALD- and GPT-determinations may be abandoned in Duchenne dystrophy. In Duchenne muscular dystrophy the course of the different enzyme activities can be described by an heuristic mathematical formula (y = Ae-at + bte-ct). With its aid it is possible to calculate the value of the constant c individually for each patient, if at least 4 enzyme values distributed over 3 or more years are available. The evaluation of the enzymes and clinical data of 88 Duchenne patients has shown that the value of constant c is individually correlated to the speed of progression of the disease. This proved most reliable in the case of the CK (r = 0.43 resp. 0.76) and CKMB (r = 0.45). The mean of the constant c in the group of Duchenne patients (n = 88) was -0.29/year, in Beckers dystrophy (n = 7) -0.14/year and in limb girdle dystrophy (n = 12) -0.21/year. In Duchenne muscular dystrophy the calculation of constant c renders it possible to select patients with similar velocity of progression to reinforce the "power of therapeutic studies". The evaluation of pilot studies may be more objective, if the enzyme values actually measured under therapy are compared with those prospectively estimated by mathematical analysis of the course of the enzyme values before treatment.

Adolescent↗
PubMedDate not supplied

[Nuclear spin tomography studies (MRI) of joints in juvenile chronic arthritis].

MRT (magnetic resonance imaging) is a new method of producing pictures of internal organs; the main advantage compared to radiography is the ability to subtly differentiate soft tissues. 14 children suffering from juvenile chronic arthritis of different subgroups are described. MRT pictures of knee joints show clearly the structure and form of the articular cartilage, ligaments as well as the nature, density and thickness of the inflammatory tissue. MRT permits a very early diagnosis of a progression in the inflammatory articular process without accompanying ionising radiation, an advantage in childhood.

Adolescent↗