Biomedical subjects
Publications and source records attributed to J Flament.
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We report our single institution's effort to standardize the method of collecting peripheral blood progenitor cells (PBPC) used for autologous transplantation. PBPC were mobilized by different types of chemotherapy followed by G-CSF (24 patients) or G-CSF alone (six patients) in 30 patients with various underlying neoplastic diseases. A median of three aphereses (range: 2-7), using the CS3000 cell separator was performed and a blood volume of 101 was processed. We studied the relationship between the absolute numbers of circulating leukocytes, mononuclear cells and CD34+ cells and the amount of PBPC collected during a single apheresis procedure. CD34+ cells were quantified by leukocyte subset analysis based on flow cytometry. Both CFU-GM and CD34+ cell contents of the apheresis products (69 procedures analyzed) correlated strongly: rho = 0.936, P = 0.0001). Regression analysis showed that the total number of CD34+ cells or CFU-GM content of the apheresis products could be predicted (r = 0.915, P = 0.0001) from the absolute number of CD34+ cells in the blood. A number of 10 circulating CD34+ cells per mm3 blood ensured a minimum of 0.5 x 10(6) CD34+ cells per kg, collected on the same day. Of the 30 patients, 17 received an autologous graft that contained only PBPC in 13. Long-term and complete hematological reconstitution was observed in all patients who had a minimum of 2 x 10(6) CD34+ cells per kg reinfused.
BACKGROUND: Endogenous posterior uveitis (PU) can be associated with systemic diseases, and certain forms have strong association with HLA antigens. Much less is known regarding intermediate uveitis (IU). The purpose of this study was to determine whether IU is associated with the HLA system and whether it can be associated with systemic symptoms. METHODS: In 179 consecutive patients consulting for uveitis, a detailed history was obtained and a physical examination performed. HLA typing for 71 HLA-A, B, DR and DQ antigens, laboratory tests, and radiography of the chest, sinuses, and sacroiliac joints were systematically performed. RESULTS: Thirty-two patients (18%) had IU; 51 (28.5%) had PU and constituted our internal control group. Nine of the patients with IU (28%) had the HLA-A28 antigen, compared with 8.1% of a healthy control population and 8.6% of the patients with PU (P < 0.001). An associated disease was found in four patients with IU (12.5%) (none was HLA-A28) and in 45% of the patients with PU (P < 0.01). Some 67% of HLA-A28 patients with IU had arthralgias affecting the knee(s), compared with 17% of non-HLA-A28 patients and 18% of patients with PU (P < 0.05 and P < 0.01 respectively); 55% had gonalgias and hypocomplementemia compared with 9% and 2% respectively (P < 0.01 and P < 0.001). CONCLUSIONS: IU is significantly associated with HLA-A28; patients having this antigen may represent a subset of the disease characterized by an increased prevalence of arthralgias and hypocomplementemia.
The human leukocyte antigen (HLA) class II genotype DQA1*0301-DQB1*0302/DQA1*0501-DQB1*0201 has been identified as a marker strongly predisposing to insulin-dependent diabetes mellitus (IDDM) in Caucasian populations. Its frequency in control populations (1-3%) is still, however, 1 order of magnitude higher than the prevalence of IDDM, suggesting that its penetrance can be modified by protective factors. In this study we searched for such a factor in the DRB1 locus by studying DRB1*04 polymorphism in 174 European Caucasian IDDM patients and 73 nondiabetic control subjects, all sharing the HLA-DR3/DR4 phenotype. Significant protection was encoded by the DRB1*0403 allele, which was observed in 5 of 49 control subjects (10%) and none of 171 IDDM patients (0%) with the DQA1*0301-DQB1*0302/DQA1*0501-DQB1*0201 genotype (RR = 0.02 [0.01-0.18], P < 0.0005). These data support the concept that protective HLA class II genes can overrule the risk caused by HLA-DQ susceptibility dimers. They also contribute to a possible strategy to screen for nondiabetic individuals with increased genetic risk of developing IDDM.
We relate the case of a 15 years old girl who suffered a sudden and severe bilateral lowering of the eyesight, on the occasion of a dermatomyositis. The evolution, over a year, showed a remission of the systematic appearances but there was a limited recovery of the eyesight, the diffuse retinal lesions are the sign of a more extensive vascular problem, found in case of paediatric dermatomyositis.
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The true incidence of bedside transfusion errors, i.e. those happening when blood products have left the blood bank, is underestimated because published figures rely on reporting of clinically relevant events or on indirect methods. The SAnGUIS project assessing blood practice in a prospective and randomized fashion for 6 elective surgical procedures gave the opportunity to trace all transfused units and to identify steps at risk during blood delivery in surgery. We considered transfusion of a wrong unit as a major error and poor execution or documentation as a recording error. Over 15 months, 808 patients out of 1,448 were transfused with 3,485 units. A total of 165 errors were found after blood products had left the blood banks. Seven were misidentifications (0.74% of patients, 0.2% of units). Eight other major errors occurred in 4 (0.5%) patients. Major errors occurred during nonemergency situations, in wards or intensive care units. The remaining ('recording') 150 errors consisted of misrecordings (61), mislabellings (6), or failures to document transfusions in the medical records (83). All errors were uneventful except one misidentification which induced a transient, yet unreported, reaction. The 'descending' inquiry method used for this study showed that most errors pass unnoticed and are therefore not reported. Measurement of error rates may constitute an important quality indicator. Retrospective information of this survey to the concerned staff people provided an impetus to take adequate measures to reduce these bedside errors.
A case of a stellate hemorrhage of the macula occurring in a young man is reported. Analysis of this rare clinical entity allowed us to link it to combined occlusion of the central retinal vein and cilioretinal artery. With regard to this case and to a review of the literature, clinical and angiofluorographic features of this syndrome are recalled. The usually good prognosis of this disease was not observed in our case. Finally, the controversial pathophysiological data are discussed.
Four cases of inadvertent intracapsular lens delivery secondary to zonular lysis occurring during planned manual extracapsular cataract extraction were reported. Theoretical and practical considerations were presented based on these observations and literature review. These considerations would suggest relaxing incisions when capsulorhexis and planned extracapsular cataract extraction are associated.
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The polymorphic variable number of tandem repeats in the 5' upstream region of the human insulin gene is a well-known non-human leukocyte antigen locus contributing to genetic susceptibility to IDDM. Controversy exists about the question as to whether INS susceptibility haplotypes are or are not preferentially inherited together with HLA-DR4 haplotypes. We investigated whether genetic interaction between INS and the HLA complex can be better defined using DQ genotypic and phenotypic markers in addition to DR serology. The 5' INS 1/1 genotype was positively associated with IDDM both in non-DR4 subjects (relative risk = 4.3; 95% confidence interval, 1.6-11.5) and DR4 subjects (relative risk = 4.2; 95% confidence interval, 1.9-9.0). Further subdivision of IDDM patients and matched control subjects according to HLA-DQA1 and HLA-DQB1 genotype or phenotype also failed to show any association between 5' INS and HLA class II genes in diabetic patients. The 5' INS and HLA class II polymorphisms therefore provide independent risk markers, which may both contribute to the genetic screening of a high-risk population among nondiabetic individuals.
After reviewing the various etio-pathogenic hypotheses for pterygium in which the discontinuity of the lacrymal film at the corneo-limbic junction appears to play an essential role, the authors discuss the current therapeutic choices. The value and place of lamellar keratoplasty overlapping the limbus are discussed.
The course of Trypanosoma cruzi infection was studied in an experimental model, using the offspring of mice that were chronically infected with T. cruzi. When infected two months after birth, a higher mortality rate in heavily parasitized mice occurred in these offspring than in controls born to uninfected mothers. The harmful maternal influence reached a maximum when offspring were exposed both to prenatal (placental) and postnatal (lactating) influences. It was a reversible phenomenon that led to a T. cruzi-specific failure of the offspring to control the acute phase of the infection. Such features are suggestive of a maternally-induced impairment of the immune response of the offspring.
The multiple-dose twice-daily efficacy of the topical carbonic anhydrase inhibitor MK-927, a racemic compound, was compared with that of its pharmacologically more active S-enantiomer in a four-center, double-masked, randomized, placebo-controlled, parallel study of 1.8% sezolamide hydrochloride (MK-417), 2% MK-927, and placebo, given twice daily to 48 patients with bilateral primary open angle glaucoma or ocular hypertension and morning intraocular pressure greater than 24 mm Hg in both eyes following washout of ocular hypotensive medications. Parallel 10-hour modified diurnal curves were performed before the study and on day 14, with 4-hour curves on days 1 and 4. Both compounds demonstrated significant lowering of intraocular pressure at 8 AM, 12 hours following the evening dose, and through 10 and 6 hours following the 8 AM dose for sezolamide and MK-927, respectively. Morning trough (evening) activity as measured by mean percent change in intraocular pressure from prestudy was -9.2% for sezolamide and -11.1% for MK-927 (-13.5% and -9.6%); peak effect occurred 2 hours after dose administration and was -19.4% and -19.2% for sezolamide and MK-927, respectively. From 2 hours after dose administration, sezolamide consistently demonstrated a slightly greater decrease in intraocular pressure than MK-927; however, these differences were not statistically significant.
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Moebius syndrome is unusual and associates facial diplegia with a failure of lateral movements, and seems to be of nuclear or supranuclear origin, appearing during foetal life. Association with somatic malformations are frequent such as those of Poland syndrome. Surgery's envisaged when palsy of lateral movements is associated with deviation of the eyes.
To increase the number of HLA typed corneas a microlymphocytotoxicity assay on lymphocytes and PHA lymphoblasts was investigated. A double immunofluorescence technique using magnetic beads coated with anti-T8 (for class I) and anti-DR (for class II) monomorphic antibodies was applied. Blood samples from 50 non selected donors were obtained. HLA class I and class II typing was possible in 74% of the cases. Using PBL's (Peripheral Blood Lymphocytes) HLA class I could be defined in 29 out of 50 Cases and class II in 15 out of 50 cases. On PHA blasts HLA class I and class II antigens could be defined in 32 and 33 out of 50 cases respectively. Mean time of culture was 10 days (6-20). No influence of donor age and post partum time could be observed. By combination of both methods a significant proportion of eye donors could be reliably typed within a short time.