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Biomedical subjects

G Simkin

Publications and source records attributed to G Simkin.

9 recordsLinked to original sources
PubMed1990

Hereditary angioedema: therapeutic effect of danazol on C4 and C1 esterase inhibitors.

Hereditary angioedema (HAE) is an inherited deficiency of C1 esterase inhibitor (C1 inh). The two types of genetic C1 inh deficiency are type I, which is quantitative, and type II, which is functional. For the purpose of the present study, four HAE patients were selected. None of them had received any androgenic therapy. The group included three type I and one type II cases. All patients that entered the protocol received danazol, 400 mg/day for 14 days. The complement system was evaluated by monitoring C4, hemolytic complement 50% (CH50), circulating immune complexes (CIC), and antigenic and functional C1 inh during the study. The level of complement factors at the beginning and at the end of this period demonstrated a statistically significant increase in C4 and CH50 and the disappearance of CIC, while C1 inh remained unmodified. These results suggest that the therapeutic effect of danazol may have two mechanisms of action: (1) promotion of C4 synthesis by anabolic effect resulting in an improvement of the complement system with the disappearance of CIC and (2) a minor increase in C1 inh level primarily due to the lack of its consumption.

Adult↗
PubMed1987

[Hereditary angioedema: changes in serum levels of C4 in response to danazol].

12 patients (7 males and 5 females) suffering from the common form of HAE were included in the study protocol. All patients were older than 18 years. They were evaluated Cl INH, C4, Clq, Cls, C3, C5, C8, Bf, CH 50% and CIC. For the purpose of the study they were only considered Cl INH, C4, CH 50%, CIs and CIC levels. The rest of the complement components were among normal values. Data were recorded at day 0 and after 10 days on treatment with 400 mg/day of Danazol. Results were as follows: 50% of the patients had CIC when CH 50% values were below 120 U/ml., after treatment CIC disappeared and CH 50, Cls, C4 and Cl INH increased significatively. C4 seemed to increase more and quicker than Cl INH in terms of absolute values. We postulate that in the group of patients with CIC, the primary cause of the disease may be an alteration in C4 synthesis and that Cl INH may be lowered because of its consumption. We postulate that Danazol could act in these cases stimulating C4 synthesis independently or in accordance with Cl INH.

Adult↗
PubMedDate not supplied

[Hereditary angioedema. Effect of danazol on C4 and functional C1INH].

Hereditary angioedema (HAE) is an inherited deficiency of the inhibitor of C1 esterase (C1 inh). Two types of genetic C1 inh deficiency have been described, type I: quantitative, and type II: functional. For the purpose of the present study, 4 out of 51 HAE patients were selected. None of them had received any previous androgenic therapy. The group was integrated by two type I and one type II cases. All patients that entered in the protocol received 400 mg/day of danazol over 14 days. The complement system was evaluated by monitoring C4, Hemolytic complement 50% (CH50), Circulating Immune Complexes (CIC), and antigenic and functional C1 INH during the study. The level of the complement factors at the beginning and the end of this period demonstrated a statistically significant increase of C4 and CH50 and the disappearance of CIC, while C1INH remained unmodified. These results suggest that the therapeutic effect of Danazol may have two mechanisms of action: i. promotion of C4 synthesis by anabolic effect resulting in an improvement of the complement system with the disappearance of CIC, and ii, a minor increase of C1 inh level primarily due to the lack of its consumption.

Adult↗
PubMedDate not supplied

Hereditary angioedema: preliminary report on skin biopsy finding of fibrin and/or C4 through immunofluorescence.

Hereditary angioedema is considered an inherited disorder of the complement system, manifested by repeated crises of angioedema involving the skin, the gastrointestinal and respiratory tracts. Biopsies of normal skin obtained from 5 patients, diagnosed with hereditary angioedema, were found to be positive for fibrin and in one case also positive for C4. The sections stained with anti-fibrin serum showed fibrin deposits around isolated epidermal cells, while C4 staining revealed a homogeneous pattern along the epidermis. Fluorescence patterns and laboratory findings before and after treatment with danazol and/or anti-fibrinolytic treatment are reported.

Adolescent↗
PubMedDate not supplied

[Finding of Tart cells in asthmatic patients. Relation to IgE and other parameters].

Fifty randomly selected asthmatic patients were studied. In each the sex, age, time of onset of symptomatology, symptomatologic score (from 0 to 3), blood eosinophilia, IgE by the PRIST method and Tart cells in blood (investigated by the same method as is used in the search of LE cells in collagen diseases) were considered. Tart cells were recognized as in Miale Haemotology (9) as monocytes and occasionally polymophonuclar leucocytes with one or two round inclusions in their protoplasm owing to the phagocytosis of leukocyte nuclei. In contrast with LE cells, these inclusions are not homogeneous contain chromatin and nuclear membrane material and often have a dark ring of hyperchromic material. We have found that these cells can not be evoked passively by application of patient serum to normal leukocytes. In no case of positive Tart cells was antinuclear serum factor found by immunofluorescence as seen in lupus erythematous disease. 33 per cent of the 50 asthmatic patients tested had Tart cells in their blood. They were very scarce, only one or two in four Wright stained smears. This fact is probably why these cells were not recognized before. The positive Tart cell group was on the average the same age as the negative group. On the other hand, in the positive Tart cell group the proportion of men with respect to women, the time span from the onset of asthmatic symptoms (p less than 0,05), the symptomatology score (p less than 0,005) and blood eosinophilia (p less than 0,005) were increased. In contrast IgE was decreased on the average (p less than 0,05).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗