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[Clinico-morphologic characteristics of periodic disease].
The article deals with clinico-anatomical characteristics of two observations over periodical disease. Peculiar features of the developing in this case of genetic amyloidosis, which is considered as a disease of accumulation, are analysed.
[Muscular manifestations in periodic disease].
Between 1976 and 1983, the authors studied 50 new patients affected with periodic disease. 16 of them-mean age: 29-presented muscular manifestations. They may be grouped into two clinical aspects: muscular pain and contractures. The authors consider that these symptoms, first described in 1945, are an inherent part of the clinical picture of the disease. They discuss their relationship with periarteritis nodosa and remind the fact that their pathogenesis remains unknown.
[Structural and functional changes in the parathyroid glands in patients with periodic disease].
The results obtained during radioimmunoassay, biochemistry and morphological studies in patients with periodic disease point to the development of hypoparathyroid hormonemia, reduction of the substrate of the parathyroid parenchyma, responsible for the synthesis of parathyroid hormone. These alterations became aggravated in the course of the development of amyloidosis. The latter is not always related to amyloidosis of the parathyroid glands themselves.
[Periodic disease in reduced forms evolving in several members of the same family].
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[Familial forms of periodic disease].
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[Periodic disease: ten cases and two hypotheses].
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[Clinical, biologic and therapeutic comments on periodic disease, based on 24 personal cases].
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[Further cases of periodic disease; biological and pathogenetic study].
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[Five cases of periodic disease].
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[The periodic disease; 38 personal cases].
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[Periodic disease; reports of four typical cases].
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[First autochthonous case from Lyon of periodic disease].
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[Acetylation phenotype in patients with periodic disease].
Acetylation phenotype distribution (activity of N-acetyltransferase enzyme) was studied in 29 patients with periodical disease (PD), 73 healthy persons (Armenians), and 20 patients (also Armenians) suffering from epithelial coccygeal cysts. The results obtained indicated the prevalence of slow acetylation phenotype (67%) in the entire Armenian population. PD was characterized by slow acetylation type (28 of 29 patients had slow acetylation with a high frequency of very slow inactivators). The other hereditary pathology, also specific for Armenians (epithelial coccygeal cysts), was characterized by another type of acetylation--a rapid one. The role of slow acetylation as a genetic marker of PD was discussed.
[Periodic disease (familial paroxysmal polyseritis). 52 cases].
On the basis of well defined diagnostic criteria, the authors conclude that periodic disease affects males in particular. It commences before the age of 20 years in 80 percent of cases. In particular it occurs in Armenian and Shiite communities. Its evolution is normally benign ; amyloidosis is found in only 8 percent of cases. Joint manifestations are found in 48 percent of cases and may take on different aspects and occur in several different sites : myaglia or arthralgia, monoarthitis, oligoarthritis, polyarthritis, neck or sacroiliac pain. The authors have not noted prolonged peripheral joint episodes. No cases of amyloidosis were diagnosed before the appearance of the clinical signs of the disease. An autosomal, dominant heredity with incomplete penetration seems to be the most likely hypothesis.
[Selective involvement of the gastrointestinal tract in amyloidosis in a female patient with periodic disease and intact kidneys].
The authors describe a rare case of amyloidosis in a female patient suffering from periodic disease (PD) for 18 years without any clinico-laboratory signs of renal impairment but with marked clinical, (malabsorption, cachexia), endoscopic, x-ray and other manifestations of gastrointestinal amyloidosis. This case is of interest since patients suffering from amyloidosis due to PB develop malabsorption very rarely, namely in 2-3% of cases. As a rule, it develops in patients with pronounced chronic renal failure on hemodialysis or with a history of kidney transplantation. In this particular case, the patient demonstrated selective marked damage to the gastrointestinal tract, with the kidneys remaining practically intact. A possibility of the indicated variety of amyloidosis should be considered in specification of the genesis of persistent diarrhea in PB patients.
[The local immune mechanisms of the involvement of the teeth and periodontium in periodic disease].
The aim of our investigations was to elucidate some immune aspects of combination of caries and periodontitis with periodic disease (PD), also known as familial Mediterranean fever. In this regard in patients with active and non-active stage of PD we have studied dynamic changes of concentration of secretory immunoglobulin A (SIgA) in saliva and phagocytic activity of neutrophils derived from gum blood. It has been shown that in patients with PD these indices of local immunity of oral cavity had tendency to a decrease especially in case of PD and periodontitis combination. Disturbances of local immunity was significant in active stage of PD. Based on the obtained data and analysis of latest literature data we suppose that above mentioned changes in local immunity depended on the exhaustion of adaptive properties of patients' local immunity more pronounced in case of chronic inflammation and infection foci formation in oral cavity.