The first scientific meeting of the doctors of the Yelabuga District Hospital dated March 20, 1934

1934 ◽  
Vol 30 (5) ◽  
pp. 477-478
Keyword(s):  
Abdominal Aorta ◽  
Clinical Picture ◽  
District Hospital ◽  
Decisive Role ◽  
Scientific Meeting ◽  
Spontaneous Abortions ◽  
Cardiac Defect

Dr. Ter-Ayrapetyan G.S. Demonstrated a patient with lupus erytematdes. Localization and clinical picture of the disease are usual. But the patient undoubtedly has hereditary syphilis. Anamnesis: progressive paralysis in the mother, four spontaneous abortions, guttschinsonism, cardiac defect with sharply pronounced early vascular sclerosis, especially of the abdominal aorta. This circumstance prompted the speaker to hold the idea that hereditary syphilis played a decisive role in the etiology of this disease.

Scientific gathering of doctors of the Kazan District Hospital

1901 ◽  
Vol 1 (1-2) ◽  
pp. 95-97
Author(s):  
Editorial Board
Keyword(s):  
District Hospital ◽  
Scientific Meeting

Scientific meeting of the doctors of the Kazan District Hospital.(Annual appointment December 21, 1900).

scholarly journals To casuistry of idiocy

1907 ◽  
Vol XIV (2) ◽  
pp. 155-174
Author(s):  
N. A. Glushkov
Keyword(s):  
Clinical Picture ◽  
Rare Case ◽  
District Hospital ◽  
General Nature ◽  
Short Time

Looking through the stories of patients who passed through our hands in 1906 in a difficult department of the Kazan District Hospital, we drew attention to a rather rare case of idiocy, which, unfortunately, we had to observe for a very short time, why its study was of a general nature, and there is much in the clinical picture seemed unclear. However, we had to perform an autopsy on this patient and, examining his brain, we hoped to find the key to understanding those phenomena that seemed incomprehensible during his lifetime.

scholarly journals Disturbances of cerebral circulation in primary antiphospholipide syndrome and sneddon’s syndrome

1997 ◽  
Vol XXIX (3-4) ◽  
pp. 15-17
Author(s):  
L. A. Kalashnikova
Keyword(s):  
Heart Disease ◽  
Ischemic Heart Disease ◽  
Clinical Picture ◽  
Cerebral Circulation ◽  
Lupus Anticoagulant ◽  
Clinical Manifestations ◽  
Ischemic Heart ◽  
Spontaneous Abortions ◽  
Skin Change ◽  
Sneddon’S Syndrome

66 patients with Sneddons syndrome and 29 patients with primary antiphospholipide syndrome has been examinated. Clinical picture of Sneddons syndrome was characterized by combination of cerebrovascular disturbances (ischemic disturbance of cerebral circulation, primary disturbance of cerebral circulation) with skin change in the form of livedo. In some patients there were thromboses of peripheric veins, ischemic heart disease, spontaneous abortions or intrauterine fetus death in women, thrombocytopenia. In 64% of patients with Sneddons syndrome there were revealed antibodies to phospholipids cardiolipin and/or lupus anticoagulant, and this indicates that these cases belong to primary antiphospholipide syndrome. Clinical manifestations in 29 patients with primary antiphospholipide syndrome were similar to those in Sneddons syndrome except for the absence of livedo.

scholarly journals The profile and clinical picture of children with undernutrition admitted to National District Hospital

2020 ◽  
Vol 37 ◽  
Author(s):  
Hanneke Brits ◽  
Lezanne Botha ◽  
Wiseman Maakomane ◽  
Thabiso Malefane ◽  
Tayob Luthfiya ◽  
...  
Keyword(s):  
Clinical Picture ◽  
District Hospital

BLAU SYNDROME OR MONOGENOUS SARCOIDOSIS WITH EARLY ONSET: RUSSIAN COHORT OF PATIENTS

2021 ◽  
Vol 100 (5) ◽  
pp. 99-109
Author(s):  
E.S. Fedorov ◽  
◽  
S.O. Salugina ◽  
A.N. Shapovalenko ◽  
E.Yu. Zakharova ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Clinical Picture ◽  
Autoinflammatory Disease ◽  
Age Of Onset ◽  
Decisive Role ◽  
Pathogenic Mutation ◽  
Skin Lesions ◽  
Posterior Uveitis ◽  
Blau Syndrome ◽  
Card15 Gene

Blau syndrome (BS) is a rare monogenic granulomatous autoinflammatory disease (a variant of genetically determined sarcoidosis) caused by a mutation of the NOD2/CARD15 gene, transmitted in an autosomal dominant manner and manifested by a triad of signs: dermatitis, granulomatous arthritis with pronounced exudative component and involvement of periarticular tissues, uveitis. Objective of the study: to present the variants of the clinical picture and the type of pathogenic variants (mutations) in patients with a rare monogenic granulomatous autoinflammatory disease – BS – in the Russian Federation, the clinical picture of which can mimic juvenile idiopathic arthritis. Materials and methods of research: the observational study included patients with BS who were observed in the children's department of the V.A. Nasonova Research Institute of Rheumatology from 2014 to 2021, the diagnosis of which was confirmed by the detection of a pathogenic mutation in the NOD2/CARD15 gene. Results: the study included 8 children: 6 boys and 2 girls. Ethnic Russians were 5 patients, 1 Jew, 1 of mixed origin (peoples of Dagestan/Russians), 1 Tatar female. The age of onset of the disease is from the first days of life to 2,5 years. Skin lesions were observed in 6 (75%) patients. Atypical arthritis (boggy arthritis) was observed in all patients, in all cases the wrist, ankle and knee joints were involved in the pathological process. Uveitis developed after all other manifestations and was detected in 6 (75%) patients. At the onset, 5 patients had anterior uveitis, 2 of them with subsequent involvement of the posterior segment; in 1 child, the first ophthalmic manifestation was posterior uveitis. In 5 patients, there was no increase in acute phase markers (ESR, C-reactive protein). 5 (62,5%) patients had variant p.R334Q (c.1001G>A), 2 (25%) had variant p.R334W (c.1001G>A), 1 patient had variant p.M513T (c.1538T>C) of the NOD2/CARD15 gene. Conclusions: BS can be encountered in the practice of a pediatric rheumatologist in Russia and requires differentiation from polyarticular juvenile idiopathic arthritis. Identification of the pathogenic variant of the NOD2/CARD15 gene plays a decisive role in the diagnosis.

Jansky-Bielschowsky syndrome, a lysosomal disease: First diagnosed cash in Oman

1992 ◽  
Vol 50 (1) ◽  
pp. 632-633
Author(s):  
Line Buhl ◽  
David Muirhead
Keyword(s):  
Mental Retardation ◽  
Clinical Picture ◽  
Neurological Disorder ◽  
Clinical Presentation ◽  
Neuronal Ceroid Lipofuscinosis ◽  
Infantile Form ◽  
Lysosomal Disease ◽  
Juvenile Form ◽  
Progressive Encephalopathy ◽  
Lysosomal Diseases

There are four lysosomal diseases of which the neuronal ceroid lipofuscinosis is the rarest. The clinical presentation and their characteric abnormal ultrastructure subdivide them into four types. These are known as the Infantile form (Santavuori-Haltia), Late infantile form (Jansky-Bielschowsky), Juvenile form (Batten-Spielmeyer-Voght) and the Adult form (Kuph's).An 8 year old Omani girl presented wth myclonic jerks since the age of 4 years, with progressive encephalopathy, mental retardation, ataxia and loss of vision. An ophthalmoscopy was performed followed by rectal suction biopsies (fig. 1). A previous sibling had died of an undiagnosed neurological disorder with a similar clinical picture.

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