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G Defuentes

Publications and source records attributed to G Defuentes.

9 recordsLinked to original sources

[Hemolytic uremic syndrome as a complication of gemcitabine treatment: report of six cases and review of the literature].

UNLABELLED: Hemolytic uremic syndrome is a rare condition during gemcitabine therapy. METHODS: We report six new cases of hemolytic uremic syndrome related to gemcitabine, three issued from a retrospective study of 136 consecutive patients treated with gemcitabine for which a systematic screening of this side effect has been performed and 29 cases with clinical data available identified in the literature in order to better characterised frequency and clinical presentation of this side effect. RESULTS: In our series, frequency of HUS is 2.2% and is higher than this previously reported (0.015%) or estimated with the data of clinical trials analysed (0.072 %). For 35 cases with clinical data available, the patients were always treated for a local advanced and/or metastatic disease. For our cases and for literature cases, at the time of diagnosis of hemolytic uremic syndrome, mean number of doses received (mean+/-standard deviation. Minimum/maximum)) (personal cases: 26.5+/-6.6. 16/36, literature cases: 21+/-11. 8/54), cumulative dose received (g/m2) (personal cases : 24.5+/-6.3. 16/31.6, literature cases: 21.7+/-12.4. 2.4/54) and duration of treatment (months) (personal cases: 8.2+/-1.9. 5.6/11, literature cases: 8.5+/-4.0. 3/18) are very closed and high individual variations observed for these factors are not consistent with a time and/or dose dependant toxicity. New-onset hypertension or exacerbation of underlying hypertension is the most common clinical manifestation, with mild anemia; thrombocytopenia is inconstant. The degree of severity of renal failure is highly variable. The existence of subacute clinical form with progressive worsening of the symptoms and biological form at the time of diagnosis suggest the interest of a systematic clinical and biological screening of this side effect, before each injection of gemcitabine. Early prognosis is linked to the evolution of hemolytic uremic syndrome and after hemolytic uremic syndrome healing, cancer progression. Treatment include gemcitabine discontinuation, antihypertensive drugs and if necessary fresh frozen plasma. CONCLUSIONS: Systematic clinical and biological screening of hemolytic uremic syndrome during gemcitabine therapy should allow to better know this complication, to recognize and treat it earlier with a potential positive impact for patients.

Aged↗

[Means syndrome. 8 cases].

BACKGROUND: Euthyroid Graves' disease is defined as an ophthalmopathy without dysthyroidism. We report 8 cases in 6 women and 2 men with an average follow-up of 16 months after diagnosis. CASE REPORTS: Unilateral ex-opythalmos was the first and most frequent sign. Ultrasonographic and immunological thyroid abnormalities were frequent but of questionable significance. Thyroid hormone levels continued to remain normal. The orbital CT-scan, used to screen for infra-clinical exophthalmos, evidenced focal or diffuse hypertrophy of the extraocular muscles and normal tendon insertions. More than 3 years after diagnosis, only one patient developed overt Graves' disease and clinical signs resolved spontaneously in one other. DISCUSSION: In mild to moderate forms of euthyroid Grave's disease, therapeutic abstention is advisable. Corticosteroids and orbital radiotherapy are interesting options in functionally disabling forms. Decompressive surgery is rarely needed.

Adult↗

[Incidence and clinical profile of familial forms of Parkinson's disease. A study of 428 index-cases from a department of neurology].

Although the cause of Parkinson's disease remains to be determined, several lines of evidence favor the role of a genetic factor. We therefore studied a series of 428 index-cases of Parkinson's disease that were referred to our Department of Neurology between 1986 and 1993, with the aim to identify secondary family cases. Index-cases were divided into 2 groups. In groups A (279 cases), a retrospective analysis of patients records was performed. In group B (149 cases), a prospective study was carried out by 1992, which allowed a more complete investigation of family history. In group A, 31/279 index-cases (11.1 p. 100) had secondary familial cases of Parkinson's disease. This percentage increased up to 22.8 p. 100 among index-cases in group B (34/149 cases). In most instances, only one secondary case was detected, and very few proponents had 2, or 3 other family cases. No large family with numerous Parkinson's disease cases was disclosed. Age at onset of disease was similar in group A between sporadic and familial index-cases, whereas in group B age at onset was earlier index-cases with positive family history as compared to those without (53.9 +/- 10.4 years versus 59.7 +/- 12.1 years respectively). This may be due to the different sizes of groups A and B, whereas clinical profile analysis did not differentiate index-cases with positive family history from those without family history (sporadic cases). An anticipation of age at onset of illness of 13.9 +/- 12.2 years was found in 9 of the 15 index-cases from group B with first degree parental vertical inheritance, where clinical data were available for the second family case. These findings about age at onset may be at least partly explained by a more accurate estimation of age at onset in index-cases than that in secondary family cases. Further analysis on the possible mode of transmission of the disease among familial cases was consistent with the implication of a genetic factor in the ethiopathogenesis of the disease, with a mendelian autosomal dominant inheritence with reduced penetrance.

Disease Transmission, Infectious↗