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Biomedical subjects

Y Sultan

Publications and source records attributed to Y Sultan.

At least 37 records · Page 2Linked to original sources
PubMed1998

Clinical management of patients with von Willebrand's disease with a VHP vWF concentrate: the French experience.

Part of the French clinical experience using the solvent/detergent (S/D) treated VHP von Willebrand factor (vWF) concentrate (LFB, Les Ulis, France) characterized by a high vWF:RCoF specific activity and a low factor VIII (FVIII) content is reported. Since 1989 this concentrate has been routinely used in clinical practice taking into account the vWF:RCoF given by the manufacturer. Seventy-five patients with von Willebrand disease (type 1: 42, 2A: 11, 2B: 5, 2N: 6, 3: 4, acquired: 7) were treated on 99 occasions either to control spontaneous bleedings (15) or to prevent haemorrhagic risks associated with minor (< 5 days of treatment) (48) or major (5 days of treatment) (36) surgery including seven knee or hip-replacements. Forty lots of concentrate were used containing 58 +/- 13 U mL-1 vWF:RCoF with less than 10 units of FVIII per 100 units vWF:RCoF. Patients with type 2N were analysed separately. With the exception of gastro-intestinal bleedings, spontaneous bleedings were generally stopped after few infusions of 40-47 U kg-1 vWF:RCoF. Patients having more than 20 U dL-1 FVIII were treated on 54 surgical occasions with one preoperative infusion (51-55 U kg-1 vWF:RCoF) which allowed an increase in FVIII concentration to a mean level of 67-88 U dL-1. Patients with less than 20 U dL-1 were either treated with two preoperative infusions of vWF, 12 or 24 h apart (11 cases) or received a FVIII injection immediately after the preoperative infusion of vWF (10 cases). During the postoperative period vWF alone (30-35 U kg-1 vWF:RCoF) allowed FVIII to be kept at a mean level of 118-138 U dL-1 not exceeding 180 U dL-1. Patients with type 2N were treated taking in account only their baseline FVIII concentration. No haemorrhagic complications occurred in any of the patients. Thus it was found to be feasible and practical to manage replacement therapy in patients with von Willebrand disease (whatever the type or the circumstances) on the basis of the vWF:RCoF activity of the concentrate.

France↗
PubMed1998

Azithromycin compared with rifampin for eradication of nasopharyngeal colonization by Neisseria meningitidis.

OBJECTIVES: To evaluate the efficacy and safety of azithromycin compared with rifampin for eradication of nasopharyngeal carriage of Neisseria meningitidis METHODS: Pharyngeal swabs were obtained from 500 students attending nursing school in Cairo, Egypt, to determine the colonization rate with N. meningitidis. Colonized individuals were randomized to receive azithromycin (500 mg once) or rifampin (600 mg twice daily for four doses). Subjects were then recultured 1 and 2 weeks posttreatment to determine the effectiveness of the antibiotic therapy for eradication of meningococcal nasopharyngeal colonization. RESULTS: Individuals treated with azithromycin had a 93% eradication rate at 1 and 2 weeks posttreatment comparable with 95 and 91%, respectively, for rifampin. No significant side effects were reported by any subjects treated with either antibiotic. CONCLUSION: Azithromycin is effective in the eradication of N. meningitidis from the nasopharynx of asymptomatic colonized individuals and deserves further evaluation for use as prophylaxis against N. meningitidis.

Adolescent↗
PubMed1998

Tuberculosis meningitis, Abbassia Fever Hospital-Naval Medical Research Unit No. 3-Cairo, Egypt, from 1976 to 1996.

A total of 1,430 patients with the presumptive diagnosis of tuberculous meningitis were admitted to the U.S. Naval Medical Research Unit No. 3/Abbassia Fever Hospital in Cairo, Egypt from January 1976 to January 1996. Diagnosis was confirmed by culture of the mycobacteria from the cerebrospinal fluid CSF of 857 patients and these patients are included in the final analysis. There were 497 males and 360 females. The patients ranged in age from five months to 55 years. The number of patients admitted during the months of March, April, and May were more than double those admitted during October, November, and December. The duration of symptoms prior to admission ranged from seven to 90 days (mean = 29.5 days). Upon admission, 4% of the patients were alert, 34% were drowsy, and 62% were in a coma. Of the 857 patients studied, 490 (57%) died, 256 (30%) recovered completely, and 11 (13%) recovered with sequelae. The mortality and neurologic sequelae were directly related to the stage of disease and duration of symptoms prior to admission. Mortality was significantly lower in patients admitted in stage II and or with short duration of disease compared with those in stage III and or with prolonged duration of symptoms prior to admission. The use of dexamethasone in patients with tuberculous meningitis significantly reduced the ocular complications that occur in these patients and also significantly reduced the fatality rate.

Adolescent↗
PubMed1998

Nonsurgical synovectomy in the treatment of arthropathy in Von Willebrand's Disease.

Von Willebrand's disease is the most common inherited bleeding disorder, with an overall prevalence in the general population of 0.8% to 1.3%. Hemarthrosis occurs mainly in the severest forms of the disease (type III), with a frequency of 3.5% to 11%, and can cause severe arthropathy similar to that seen in hemophilia. We retrospectively reviewed our experience with nonsurgical synovectomy in the treatment of recurrent hemarthrosis with arthropathy in patients with von Willebrand's disease. Four of our six patients had type III disease and the remaining two had type II disease. The age range was 13 to 63 years. The frequency of hemarthrosis prior to synovectomy was one to four per month. One (n = 2) or both (n = 1) knees were treated in 4 cases, one (n = 1) or both (n = 1) ankles in 3 cases and an elbow in one case. We used yttrium 90 in a dose of 5 mCi for one knee, rhenium 186 in a dose of 2 mCi for two ankles and the elbow and osmic acid for two knees and one ankle. Clinical and radiological results were evaluated six months after synovectomy using the World Federation of Hemophilia score. Radiologic lesions remained stable and clinical manifestations improved in every case (p < 0.05). Five patients achieved a complete remission. Safety was satisfactory. The clinical efficacy of synovectomy done, using radiocolloids or osmic acid in arthropathy due to von Willebrand's disease, seems similar to that in hemophilia.

Adolescent↗
PubMed1997

Frequency of human anti-FVIII antibodies in humanized SCID mice elicited by recombinant deleted factor VIII and by a plasma derived factor VIII.

SCID mice were grafted with human PBL (hu-PBL-SCID) from healthy or haemophilia A donors. Those containing human and no murine Ig in their plasma, were injected with 100 U VIII:Ag of a plasma derived (pd) FVIII or recombinant deleted Factor VIII (FVIII deltaII) and with 10 microg of tetanus toxoid as control immunogen. The frequency and the intensity of the humoral specific responses were measured in 253 mice humanized with PBL from 13 different donors. There was no significant difference in the frequency or intensity of the anti-FVIII immune responses to pd FVIII and FVIII deltaII. Neutralizing antibodies were only detected in the plasma of mice humanized with cells from haemophiliacs having FVIII inhibitors in their blood. The immune responses observed in hu-PBL-SCID mice correlated with the immune status of the corresponding human donor.

Adult↗
PubMed1997

Prevalence, male germ-line origin and new patterns of inversions in haemophilia A.

Following the discovery of the FVIII gene inversion by Lakich et al. [1] and Naylor et al. [2], we have investigated this mutation in 108 French and Algerian severe haemophilia A patients. We have found that only 29 severe haemophiliacs (27%) exhibited the rearrangement whereas Lakich et al. [1] and Naylor et al. [2] respectively estimated the inversion frequency at 47% and 42% in severe haemophiliacs. The reason for this discrepancy is not accounted for. In this study, we observed two novel patterns of inversions as yet unreported. We did not find any correlation between the presence of the inversion and a particular RLFP haplotype, or ethnic origin, or the absence of a FVIII inhibitor. Among the cases with the inversion, the proportion of sporadic and transmitted cases was roughly equivalent and we also confirm that the inversion occurs preferentially in the male germ-line.

Algeria↗
PubMed1997

Multicenter retrospective study on the utilization of FEIBA in France in patients with factor VIII and factor IX inhibitors. French FEIBA Study Group. Factor Eight Bypassing Activity.

Factor VIII or factor IX replacement is frequently impossible in inhibitor-developing hemophiliacs, because of the level of the inhibitor titer. Activated prothrombin complex concentrates are one of the available options to treat the bleeding episodes in such patients. However, the efficacy of these products and the associated thrombogenic risk, particularly in prolonged administration such as employed during surgeries, are important concerns for hemophilia care providers. We performed a multicenter retrospective study to evaluate the use of FEIBA (Factor Eight Bypassing Activity) in France, and data is presented on 433 bleeding episodes, including surgical procedures, concerning 60 patients from 15 hemophilia centers. The efficacy was judged as good or excellent in 352 episodes (81.3%), poor in 73 episodes (16.9%) and non-existent in 8 episodes (1.8%). Minor and major surgical procedures were successfully performed using FEIBA as a second-line therapy after human or porcine factor VIII, and in some occasions FEIBA was utilized as the only substitution product. The tolerance was assessed as good in 428 episodes (98.8%), but in 5 cases adverse effects were reported. Only 3 patients out of 52 regularly evaluated (5.8%) were HIV-seropositive, and for two of them the seroconversion occurred prior to the first use of FEIBA. In contrast, 80.4% of the patients were HCV-seropositive. An anamnestic response after the administration of FEIBA was noted in 31.5% of cases. This study points out the main features of the use of FEIBA in France, and particularly the low HIV seroprevalence in the patients treated. The good efficacy and the excellent tolerance still confer to this product a place to consider in the therapeutic options for the treatment of inhibitor-developing hemophiliacs or in acquired hemophilia.

Adolescent↗
PubMed1997

Acquired hemophilia and its treatment.

The development of auto-antibodies against clotting factor VIII is a rare disease that occurs predominantly in adults: in pregnant women or people with various immunologic disorders or with no apparent underlying disease. As a consequence of an immune system dysregulation, the evolution of this condition is unpredictable. The aim of treatment is to restore normal factor VIII levels in circulation using desmopressin (DDAVP), massive doses of factor VIII (human or porcine), plasmapheresis and factor VIII infusions. In patients with high-titer inhibitors, products with 'bypasing' activity can be used. The most difficult task is to treat the immune disorder. High-dose infusions of immunoglobulins might be useful, due to the presence of anti-idiotypes to factor VIII inhibitors in the commercial preparations. Corticosteroids and cyclophosphamide are currently the most widely used treatments for the immune disease.

Adult↗
PubMed1996

HEMOCO: a French prospective study of hemophiliacs infected by human immunodeficiency virus type 1 (HIV-1).

HEMOCO is a multicenter prospective cohort set up in 1989 to monitor 407 French hemophiliacs infected by HIV-1 and recruited in 4 hemophilia treatment centers in the Paris region. As of 15 July 1995, 42% of the patients in the cohort had developed stage B HIV disease and 29% stage C disease (AIDS); 23.1% of the patients had died. The cumulative proportion of patients with AIDS was 4.5% at 5 years and 27.4% at 10 years, while the respective mortality rates were 3.8% and 19.5%. In our study, only age was predictive of AIDS, with an estimated relative risk of 1.2 per 10-year age increment; this factor was also predictive of death. After 10 years of follow-up, 6.1% of the study population had no clinical or laboratory signs of immunodepression. The follow-up protocol in the HEMOCO protocol is the same as that in the French SEROCO study, which includes men infected by HIV-1 through sexual contact. This will allow us to compare the progression of HIV infection between these two exposure groups.

Acquired Immunodeficiency Syndrome↗
PubMed1995

Factor VIII gene inversions in severe hemophilia A: results of an international consortium study.

Twenty-two molecular diagnostic laboratories from 14 countries participated in a consortium study to estimate the impact of Factor VIII gene inversions in severe hemophilia A. A total of 2,093 patients with severe hemophilia A were studied; of those, 740 (35%) had a type 1 (distal) factor VIII inversion, and 140 (7%) showed a type 2 (proximal) inversion. In 25 cases, the molecular analysis showed additional abnormal or polymorphic patterns. Ninety-eight percent of 532 mothers of patients with inversions were carriers of the abnormal factor VIII gene; when only mothers of nonfamilial cases were studied, 9 de novo inversions in maternal germ cells were observed among 225 cases (approximately 1 de novo maternal origin of the inversion in 25 mothers of sporadic cases). When the maternal grandparental origin was examined, the inversions occurred de novo in male germ cells in 69 cases and female germ cells in 1 case. The presence of factor VIII inversions is not a major predisposing factor for the development of factor VIII inhibitors; however, slightly more patients with severe hemophilia A and factor VIII inversions develop inhibitors (130 of 642 [20%]) than patients with severe hemophilia A without inversions (131 of 821 [16%]).

Blotting, Southern↗
PubMed1995

Synthetic sorbents for removal of factor VIII inhibitors from haemophilic A plasma.

Human factor VIII (FVIII) is a protein of the blood coagulation system that is absent or defective in patients with haemophilia A. A most serious complication following replacement therapy in 10-15% of patients treated with available FVIII concentrate is the development of inhibitors of FVIII (anti-FVIII). Some polymers functionalized with suitable chemical substituents which mimic part of the epitope of FVIII recognized by the inhibitors might be used in extracorporeal circulation to reduce the concentration of antibodies to FVIII. For this purpose, insoluble polystyrene bearing sulfonate and L-tyrosine methyl ester sulfamide groups have been synthesized. The in vitro removal of anti-FVIII inhibitors from plasmas of patients with haemophilia A was performed. Different chromatographic parameters were studied and optimized.

Adsorption↗
PubMed1995

Short course chemotherapy with cefixime in children with multidrug-resistant Salmonella typhi Septicaemia.

Increasing prevalence of multidrug-resistant (MDR) Salmonella typhi strains in pediatric cases of typhoid fever and chemotherapy restrictions in children, such as fluoroquinolones, require ongoing clinical evaluations of different antibiotic regimens. Previously reported clinical trials with oral cefixime therapy given as a 12-day regimen (20-30 mg/kg divided twice daily) demonstrated both safety and efficacy. An open trial was undertaken to investigate a short course (8-day) regimen of oral cefixime in an Egyptian public fever hospital. Eighty children were initially enrolled with blood culture confirmation in 60 children. Clinical cure was documented in 57 (95 per cent) with three children requiring a change in antibiotic regimen due to therapeutic failure and one child with culture-confirmed relapsed 21 days post-therapy. All S. typhi isolates were sensitive to cefixime as measured by disk diffusion. Cefixime was well-tolerated with only mild side-effects, including nausea/vomiting (8 per cent) and abdominal cramping with loose stools (6 per cent), which may have been secondary to typhoid fever. Cefixime given in a short 8-day course is safe and effective in the management of MDR typhoid fever in children.

Adolescent↗