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PubMed2000

[Immunological indices in development of periodic disease].

AIM: To specify immune changes in periodical disease (PD) in different periods of PD development as well as peculiarities of their changes in colchicin therapy. MATERIAL AND METHODS: Clinicoimmunological investigations including blast transformation of lymphocytes with lymphocytic mitogen and renal antigen, leukocyte migration inhibition with renal antigen, spontaneous and complementary rossette formation were made in 828 PD patients and 43 donors. RESULTS: Decreased functional activity and quantity of T-lymphocytes, their suppressory subpopulation was found in PD. The indices did not correlate with the disease variants and gender but with age and PD complication. In amyloidosis immunological indices deteriorate with positive trend after colchicin treatment. CONCLUSION: PD is characterized by strain in immunological process.

Adolescent↗
PubMed1977

[HL-A and periodic disease].

In the department of Professor Dausset 41 cases of periodic disease referred by 7 clinics in Paris were examined. Two studies were carried out. A series of 31 not related patients was testes with 30 locus A and B antigen and the frequencies observed were compared with the frequencies in a French and Yemenite Jew population. No statistically valid increase of an HLA gene indicative of a relationship between periodic disease and HLA was found. In 5 cases of amyloidosis HLA A 28 was present and it is possible that this gene is related to the amyloid complication. A family study on 12 families, 7 of which included at least 2 affected children, confirmed the recessive hereditary character of the disease, but not in relation to the HLA system. A parallel clinical study was made, which corroborated the previous studies as regards the frequency of clinical signs. The therapeutic study proved the value of long-term colchicin treatment with or without antihistaminics. The frequency of pain and abdominal crises was reduced, but there was little effect on the articular manifestations.

Adolescent↗
PubMedDate not supplied

[Cutaneous manifestations of mediterranean periodic disease. Concerning an observation. Review of the literature (author's transl)].

Méditerranean Periodic Disease is frequently encountered in non-aschenazic Jews and in Armenians. Aside from the classic triad of pseudo-palustral febrile crises, paroxysms of abdominal and articular pain, and a biological syndrome of inflammation; cutaneous manifestations were noted in 25 to 35% of the cases according to various authors. The most commonly encountered lesions consisted of erysipel-like plaques and subcutaneous nodules. We observed the case of a 47 years old Armenian male, afflicted with Mediterranean Periodic Disease for 30 years in which the dermatologic symptoms are quite classic aside from a vitiligo having progressively appeared since 15 years. Possibly a coincidental association, but to the best of our knowledge, which has not as yet been reported.

Erythema↗
PubMed1984

[Immunomorphological evaluation of intestinal function in periodic disease patients].

Based on clinico-endoscopic, x-ray, immunological and immunohistochemical studies the authors demonstrate the role of disorders in the components of humoral and cellular immunity in the development of intestinal inflammation and amyloidosis in patients with periodical disease. Circulatory disorders, congestive hyperemia, edema of the intestinal mucosa, and spasmodic intestinal dyskinesia that develop during an attack of periodical disease are the initial components in the pathogenesis of inflammatory intestinal lesions seen during periodical disease.

Amyloidosis↗
PubMed1977

An investigation of the complement system in patients with periodic disease (results from 29 cases).

The complement system was investigated in 29 patients suffering from authentic periodic disease. A statistically significant increase in C4, also in total complement and C3 could be demonstrated. It is possible that the increase in C4 was due to the macrophages which are always present in the infiltrates of periodic disease. This biological observation is of clear practical importance for the diagnosis of the condition both before and after colchicine therapy.

Complement C1 Inactivator Proteins↗
PubMed2000

[Immune disorders in periodic disease].

The examination of 404 patients with periodic disease (301 with uncomplicated form and 104 with amyloidosis complication) has detected decreased functional activity of T-lymphocytes and their suppressor subpopulation, their subnormal quantity. There was a rise in T-helper/T-suppressor index, level of B- and 0-lymphocytes, sensitivity to the renal antigen. Colchicin therapy stopped the attacks and stimulated T-suppressor activity. Combined treatment with tactivin is proposed.

Adolescent↗
PubMed1990

[Gastroenteropathies in patients with periodic disease and amyloidosis].

The reported and the authors' data are provided on the pathogenesis, morphofunctional characteristics and clinical manifestations of acute and chronic gastroenteropathies in patients suffering from periodic disease with and without amyloidosis. Under discussion is the significance of changes in the gastrointestinal tract for the diagnosis of periodic disease, its association with other diseases and early recognition of amyloidosis.

Amyloid↗
PubMed2006

[Study of volatile fatty acids in the blood of patients with periodic disease].

Microecological "failures" are an important pathogenetic factor of different diseases and, in the authors' opinion, periodic disease (PD) is one of them. PD is a recessive disease characterized by fever attacks and neutrophil-mediated serous inflammation. A genetic factor has been established to be responsible for half the cases of PD, the influence of non-hereditary factors, particularly a role of the host automicroflora in the genesis of an inflammatory process, has been little studied. The authors' early studies indicate that there are changes in the qualitative and quantitative composition of microbial molecules in the blood of patients with PD. The anaerobic bacterial metabolites that are volatile fatty acids (VFAs) represent biologically active substances that affect the growth of the microflora, on the one hand, and the host's immunological responsiveness, on the other. Out of VFAs, only is acetic acid detectable in small quantities in the blood of healthy individuals. The other VFAs, namely propionic, valeric, butyric, and caproic acids and their isomers, are absent. Gas chromatography was used for qualiitative and quantitative determination of the metabolites of anaerobic microorganisms in the blood of patients with PD (n = 13) during an attack and remission and in that of healthy volunteers (Armenians) (n = 5) of a control group from one Yerevan region. The blood samples from all the patients with PD displayed a significantly higher concentration of caproic acid while the latter was absent in the blood of the controls. This finding suggests that there is a specific shift in the structure of the microbiocenosis in patients with PD. It is conceivable that caproic acid plays a certain role in the pathogenesis of the disease under study. Further studies will deal with the association of some microbial molecules with the manifestation of an attack of PD, which may provide the key to the goal-oriented regulation of detected homeostatic disorders and to the management of the frequency of its attacks.

Armenia↗
PubMedDate not supplied

[Periodic disease].

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Disease↗