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Y Sultan

Publications and source records attributed to Y Sultan.

At least 19 recordsLinked to original sources
PubMed2002

Polystyrene derivatives as candidates for extracorporeal adsorption of Factor VIII antibodies in the management of haemophiliac patients.

BACKGROUND AND OBJECTIVES: This article presents a new approach for removing the Factor VIII inhibitors (anti-FVIII) in haemophiliac patients by immunoadsorption using an affinity matrix. MATERIALS AND METHODS: Ten blood samples from haemophiliac patients with anti-FVIII were assayed for antibodies, total immunoglobulins, procoagulant proteins and complement C3 protein after circulation over one or two columns filled with the polymers under investigation. RESULTS: These new synthetic sorbents are able to remove in vitro 90% of anti-FVIII from haemophiliac plasma with inhibitors (up to 540 Bethesda Units/ml). Neither coagulation factor adsorption nor effects on complement system activation were observed. CONCLUSIONS: The data presented clearly show that these polymers allow a rapid and efficient reduction of inhibitor titre. In view of the parameters studied, these polymers fulfil the requirements for use in a blood purification process to decrease high inhibitor titres without losing essential proteins.

Autoantibodies↗
PubMed2000

Azithromycin versus ceftriaxone for the treatment of uncomplicated typhoid fever in children.

A total of 108 children aged 4-17 years were randomized to receive 7 days of azithromycin (10 mg/kg/day; maximum, 500 mg/day) or ceftriaxone (75 mg/kg/day; maximum, 2.5 g/day), to assess the efficacy of the agents for the treatment of uncomplicated typhoid fever. Salmonella typhi was isolated from the initial cultures of blood samples from 64 patients. A total of 31 (91%) of the 34 patients treated with azithromycin and 29 (97%) of the 30 patients treated with ceftriaxone were cured (P>.05). All 64 isolates were susceptible to azithromycin and ceftriaxone. Of the patients treated with ceftriaxone, 4 subsequently had relapse of their infection. No serious side effects occurred in any study subject. Oral azithromycin administered once daily appears to be effective for the treatment of uncomplicated typhoid fever in children. If these results are confirmed, the agent could be a convenient alternative for the treatment of typhoid fever, especially in individuals in developing countries where medical resources are scarce.

Adolescent↗
PubMed2000

Antibodies to the FVIII light chain that neutralize FVIII procoagulant activity are present in plasma of nonresponder patients with severe hemophilia A and in normal polyclonal human IgG.

We have analyzed the properties of anti-factor VIII (FVIII) immunoglobulin (Ig) G recovered by affinity chromatography on FVIII-Sepharose from the IgG fraction of the plasma of healthy individuals and nonresponder patients with hemophilia A. Affinity-purified anti-FVIII antibodies were found to neutralize FVIII activity and to bind to FVIII with an affinity similar to that of anti-FVIII IgG that had been affinity-purified from the plasma of inhibitor-positive hemophilia patients and of patients with anti-FVIII autoimmune disease. The antibodies also exhibited patterns of reactivity with thrombin-digested FVIII similar to those of FVIII inhibitors and preferentially recognized epitopes located in the light chain of FVIII. These observations suggest that FVIII inhibitors occurring in hemophilia A and in patients with anti-FVIII autoimmune disease originate from the expansion of preexisting natural anti-FVIII clones that exhibit FVIII-neutralizing properties.

Antibodies↗
PubMed2000

Epidemiological survey of the orthopaedic status of severe haemophilia A and B patients in France. The French Study Group. secretariat.haemophiles@cch.ap-hop-paris.fr.

One hundred and 16 patients contributed to an analysis of the impact of the consequences of severe haemophilia A or B (factor levels < 2%) on orthopaedic status, resources consumed in relation to this status and resultant cost, and quality of life as perceived by the patient, using the MOS 36-Item-Short-Form Health Survey (SF-36). This French cross-sectional study involved outpatients regularly attending a haemophilia treatment centre. Data were collected retrospectively over a period of 1 year by the physician of the haemophilia treatment centre. Patients had a mean age of 23, and consisted of 50% students, 25% salaried workers, 17.2% with no professional activity and 7.8% physically impaired; 82.8% of them had type A haemophilia. Mean pain score was 2.5 per patient for the six main joints; 7.7 for the clinical score and 18.8 for the radiological score, with a mean number of bleeds of 16.3 per year per patient. During the year prior to inclusion, and because of their orthopaedic status, 22.4% of patients were hospitalized, 76.7% attended for an outpatient visit and 76.7% required at least one special investigation; 97.4% received replacement therapy, 41.4% required treatment for joint pain and 42.2% orthopaedic equipment. The less affected dimensions were the physical function (76.8 +/- 22. 2) and the social relations (76.1 +/- 23.1). Least good quality of life scores concerned the pain (60.2 +/- 25.2), perception of general health (59.3 +/- 23.1) and vitality (57.8 +/- 19.5) dimensions. The age was a discriminant criterion since quality of life was better in patients of the 18-23 age group for five dimensions. Mean annual treatment costs of a patient with severe haemophilia were determined as 425 762 French francs ($73 029). Loss of production was estimated at a mean of 4609 French francs ($791) per active patient over the course of the year. Results showed indirect evidence of the usefulness of early home treatment.

Absenteeism↗
PubMed2000

Efficacy of a sucrose-formulated recombinant factor VIII used for 22 surgical procedures in patients with severe haemophilia A.

A sucrose-formulated recombinant FVIII (rFVIII-SF) was investigated under clinical trial conditions during surgical procedures in previously treated patients (PTPs). Fifteen PTPs with severe haemophilia A (FVIII < or = 1%) underwent 22 surgical procedures. The procedures performed cover a spectrum from minor to major surgery. Haemostatic outcome was assessed by the investigators to be excellent in 16 procedures and good in the remaining six procedures. It is concluded that rFVIII-SF is efficacious and safe in severe haemophilia A patients undergoing minor or major surgery.

Acute Disease↗
PubMed2000

Isolation and antibiotic susceptibility of Salmonella, Shigella, and Campylobacter from acute enteric infections in Egypt.

While Campylobacter, Salmonella, and Shigella remain major contributors to acute enteric infections, few studies on these pathogens have been conducted in Egypt. From January 1986 to December 1993, 869 Salmonella, Shigella and Campylobacter strains were isolated from stool specimens from 6,278 patients, presenting to the Abbassia Fever Hospital, Cairo, Egypt, with acute enteric infections. Salmonella predominated, totalling 465 isolates, followed by Shigella with 258 isolates, and Campylobacter with 146 isolates. Of the Shigella isolates, 124 were Shigella flexneri, 49 were S. sonnei, 47 were S. dysenteriae (mainly serotype 1, 2, and 3), and 38 were S. boydii. Campylobacter spp. comprised 92 Campylobacter jejuni and 54 C. coli isolates. Isolation of Salmonella was highest during the months of February-March, June-July, and October-November, while that of Shigella was maximal from July to October. Isolation of Campylobacter increased during May-June and again during August-October. Although Salmonella was sensitive to amikacin, aztreonam, ceftriaxone, and nalidixic acid, it was, however, resistant to erythromycin, streptomycin, ampicillin, chloramphenicol, and tetracycline. Shigella (> 80%) was sensitive to amikacin, ceftriaxone, cephalothin, sulphamethoxazole-trimethoprim (except S. sonnei), aztreonam, and nalidixic acid. Resistance (> 50%) was noted only for ampicillin, chloramphenicol, and tetracycline. C. jejuni and C. coli were resistant to cephalothin, aztreonam, and streptomycin. Some of the above antibiotics were employed to characterize the Egyptian isolates, but did not have any clinical utility in the treatment of diarrhoea. Significant differences (p < 0.05) were observed in the resistance profiles of Shigella and Salmonella between late 1980s and early 1990s. The results suggest the use of fluoroquinolones or a third-generation cephalosporin as an empirical treatment of enteric diseases. However, alternative control strategies, including the aggressive development of broadly protective vaccines, may be more effective approaches to curbing morbidity and mortality due to acute enteric infections.

Adolescent↗
PubMed1999

Catalytic activity of antibodies against factor VIII in patients with hemophilia A.

Hemophilia A is an X chromosome-linked recessive disorder resulting in defective or deficient factor VIII (FVIII) molecules, which, in its severe form, is a life-threatening and crippling hemorrhagic disease. Infusion of homologous FVIII to patients with severe hemophilia A results, in 25% of patients, in the emergence of alloantibodies against FVIII (inhibitors)( ref. 1) that inhibit FVIII procoagulant activity by steric hindrance of the interaction of FVIII either with stabilizing molecules, with molecules essential for its activity or with activating molecules. Here, we report on the proteolysis of FVIII by alloantibodies of two patients with severe hemophilia A, demonstrating a previously unknown mechanism by which FVIII inhibitors may prevent the pro-coagulant function of FVIII. The kinetic parameters of FVIII hydrolysis indicate a functional role for the catalytic immune response in the inactivation of FVIII in vivo. The characterization of alloantibodies against FVIII as site-specific proteases may provide new approaches to the treatment of FVIII inhibitors.

Antibodies, Catalytic↗
PubMed1999

A retrospective study on chemical and radioactive synovectomy in severe haemophilia patients with recurrent haemarthrosis.

Between 1970 and 1994, 116 chemical and 90 radioactive synovectomies were performed in 107 patients with severe haemophilia and two with type 3 von Willebrand's disease. The products used were osmic acid (OA) in 100 cases, 90-Yttrium in 35 cases, 186-Rhenium in 48, 169-Erbium in two, hexacetonide triamcinolone in 16 and radioactive gold in five cases. The use of radioactive colloids is not allowed in France in patients under 15 years of age. Twenty-nine patients had more than one synovectomy per joint. All patients were evaluated for 6 months post-synovectomy, using both a clinical and a radiological score. Six months after synovectomy, a good or excellent result was obtained for 81% of the joints treated with isotopes, compared with 44% of those treated with OA, P<0.001. This superiority of isotopes over osmic acid was still observed after 6 months for the 89 joints that were re-evaluated, with follow-up ranging from 1 to 9 years. It was possible to calculate a radiological score in 84 cases. With OA the best results were from the joints with the lowest scores pre-synovectomy (<7). No correlation could be established between the clinical and the radiological scores, due to the small size of the sample. In summary: (1) chemical and radioactive synovectomy are simple and safe procedures for haemophilic arthropathy, (2) in our series, after 6 months the efficacy of isotopic synovectomy was greater than that of chemical synovectomy, and this benefit seems to persist after 6 months, and up to 9 years in the group of patients with longer-term follow-up.

Adolescent↗
PubMed1999

Induction of immune tolerance with recombinant factor VIII in haemophilia A patients with inhibitors.

We report on 11 patients (nine unrelated and a brother pair) with severe haemophilia A and factor VIII (FVIII) inhibitor, in whom immune tolerance (IIT) was induced with recombinant FVIII (r-FVIII). Their age ranged from 11 months to 47 years. The number of exposure days (ED) at inhibitor detection varied from 11 to 130. Nine of the 11 patients were high responders ¿>10 Bethesda units (BU) with peak inhibitor levels ranging from 10 to 566 BU. The other two were low responders with peak levels between 0.7 and 2 BU. Before inhibitor detection, the patients had been receiving products of various purities. The IIT regimens were very heterogeneous, and the treatment schedule varied from a short period with 50 IU kg-1 every 2 days, followed by 100 IU kg-1 every 2 days and then 220 IU kg-1 daily. The outcome was considered successful when the inhibitor level fell to 0.6 BU or lower after IIT treatment. The outcome overall was successful in nine out of 11 patients (81.8%), with the nine successful cases comprising seven of the nine high responders (77.8%) and the two low responders. Definite failure of IIT was observed in one high responder after two different IIT regimens. A second high responder is still on IIT treatment. All patients in whom IIT was successful are currently receiving r-FVIII on demand or prophylactically at various dosages. Despite the variability of the patient characteristics and the IIT schedules, this study demonstrates that r-FVIII represents an effective alternative for the eradication of inhibitors through IIT.

Adolescent↗
PubMed1999

Azithromycin versus ciprofloxacin for treatment of uncomplicated typhoid fever in a randomized trial in Egypt that included patients with multidrug resistance.

To compare clinical and bacteriological efficacies of azithromycin and ciprofloxacin for typhoid fever, 123 adults with fever and signs of uncomplicated typhoid fever were entered into a randomized trial. Cultures of blood were positive for Salmonella typhi in 59 patients and for S. paratyphi A in 3 cases; stool cultures were positive for S. typhi in 11 cases and for S. paratyphi A in 1 case. Multiple-drug resistance (MDR; resistance to ampicillin, chloramphenicol, and trimethoprim-sulfamethoxazole) was present in isolates of 21 of 64 patients with positive cultures. Of these 64 patients, 36 received 1 g of azithromycin orally once on the first day, followed by 500 mg given orally once daily on the next 6 days; 28 patients received 500 mg of ciprofloxacin orally twice daily for 7 days. Blood cultures were repeated on days 4 and 10 after the start of therapy, and stool cultures were done on days 4, 10, and 28 after the start of therapy. All patients in both groups improved during therapy and were cured. Defervescence (maximum daily temperatures of </=38 degrees C) occurred at the following times [mean +/- standard deviation (range)] after the start of therapy: 3.8 +/- 1.1 (2 to 7) days with azithromycin and 3.3 +/- 1.0 (1 to 5) days with ciprofloxacin. No relapses were detected. Cultures of blood and stool during and after therapy were negative in all cases, except for one patient treated with azithromycin who had a positive blood culture on day 4. These results indicated that azithromycin and ciprofloxacin were similarly effective, both clinically and bacteriologically, against typhoid fever caused by both sensitive organisms and MDR S. typhi.

Adolescent↗
PubMed1999

Immune tolerance protocols using low-dose factor VIII: a review of the literature.

Low-dose immune tolerance protocols use doses of factor VIII of less than 50 IU/kg/day to induce immune tolerance. Such protocols are chosen for a variety of reasons including cost, patient acceptability and ease of administration. Early published reports of low-dose protocols appeared in 1981, described small numbers of patients and moderate success. It was proposed that such protocols might modify inhibitor responses from high to low. Later reports are sparse and clinical success is again moderate. Useful treatment and expectation guidelines have emerged from experience with these protocols, and improvements for patients with inhibitors can be achieved. Consideration of immunological success, however, raises doubts about whether real immune tolerance is achieved with such protocols.

Factor VIII↗
PubMed1999

[Knee replacement arthroplasty in hemophilia: results, complications and predictive elements of their occurrence].

PURPOSE OF THE STUDY: Determine the effect of supplementation rate and HIV disease on results and complications in total knee replacement in hemophilia. MATERIAL: Twenty-nine total knee arthroplasties were performed in twenty-one disabled patients with major hemophilia, from 1986 to 1995. All the implants were postero-stabilised prostheses. The average age of patients at the time of surgery was 40.8 years, average follow-up was 4.8 years. Preoperatively, all patients complained of severe pain, with stage IV or V arthropathies, according to Arnold classification. Twelve patients were HIV sero-positive. METHOD: Functional and radiological results, and postoperative complication rate was analyzed in relation with mean deficient factor titer, HIV status, and periarticular soft tissues quality. RESULTS: Results of the total knee arthroplasties, as determined by the IKS scoring system, were 86.2/100 for Pain, Motion, Stability and 88.7/100 for function. The average gain of motion was twenty-four degrees. One patient required amputation, and one an arthrodesis after deep infection. Postoperative complications, in addition to infections, included intra articular bleeding in nine patients, one peroneal nerve palsy, two instances of inhibition to factor VIII, four superficial skin necrosis, and one important gastric bleeding which required surgical treatment. A high titer of deficient factor (> 70 per cent of the average normal concentration) seemed correlated with a lower complication rate. In this study, surgery had no incidence on HIV disease evolution. DISCUSSION: Authors emphasize the high level of postoperative infections (6/29), particularly in patients with HIV infection (5/12) frequently after superficial skin necrosis, as well as the high postoperative complication rate. CONCLUSION: However good results were finally observed, association of HIV disease, insufficient deficient factor concentration, and altered quality of periarticular soft tissues increased particularly complication occurrence. With particular attention to these factors, and despite frequent complications, total knee arthroplasty in hemophilia restores good function and allows patients satisfaction.

Adult↗
PubMed1999

Incidence of factor IX inhibitor development in severe haemophilia B patients treated with only one brand of high purity plasma derived factor IX concentrate.

Fifteen previously untreated patients (Pups) with severe haemophilia B (factor IX activity < or = 2 U/dl) only treated with one brand of plasma-derived high purity factor IX concentrate (FIX LFB) were studied. Age at first injection varied from 1 to 137 months and follow-up since this first injection from 21 to 86 months (median: 35). Cumulative exposure days (CED) were from 4 to over 100 (median: 26). Among these 15 Pups only one developed an inhibitor. Mutation analysis performed in all patients showed total gene deletion in the patient with inhibitor, partial gene deletion in another one, and missense mutations in 9 families. Mutation was not found in one patient. Actually, according to the data already published, only two patients were at high risk for inhibitor development in our population. Our study, although rather small, confirms the previously reported low incidence of inhibitors in haemophilia B. Large studies on incidence of FIX inhibitors are indeed difficult to perform, due to both the overall small number of severe haemophilia B patients and the low incidence of FIX inhibitors. Consequently, the impact of bias, such as prevalence of different types of gene defects in a given population, is major. Therefore, any study, dealing with incidence of FIX inhibitors in severe haemophilia B should report, for each patient, the type of gene defect.

Antibodies↗
PubMed1999

Comparative study on different recent diagnostic and therapeutic regimens in acute typhoid fever.

Forty five positive blood culture acute typhoid cases were studied during a 2 years period (1997-1999) in Abbassia Fever hospital, Cairo, Egypt. Their ages ranged between 4-23 (12 +/- 2.5) years. Male: Female ratio was 1:1. Three of the 4 classical signs namely: toxic look (84%), bronchitic chest (47%), tumid tympanitic abdomen (84%) and just palpable receding spleen (69%) were found in almost all cases and offer a good bed side clinical diagnostic test. Blood picture revealed anaemia, within normal white blood count and thrombocytopenia. Liver function tests showed within normal total serum bilirubin, two or more folds increase of ALT and within normal serum alkaline phosphatase. Comparing the 3 tests, namely significant Widal titre (56%), modified Widal test (89%) and bright spleen (78%), it was found that modified Widal test is the most sensitive serological test. Ultrasonographic finding of bright spleen is an easy, safe, noninvasive and sensitive technique which is relatively cheap. Each of the 3 drugs in our study namely chloramphenicol, quinolones and ceftriaxone resulted in improvement of general condition, drop of fever, increase in haemoglobin, white blood count and platelet count. Also, there was a significant improvement of liver function tests by either of the 3 drugs. Ceftriaxone is the best drug from the clinical and laboratory points of view followed by quinolones in multidrug resistant (MDR) acute typhoid cases. Chloramphenicol is still the drug of choice in chloramphenicol sensitive salmonellae.

Acute Disease↗
PubMed1999

Intestinal tuberculosis in patients with fever of unknown origin in Egypt.

Nineteen patients with intestinal tuberculosis were admitted to the Abbassia Fever Hospital in the period 1991-1997. Fever, abdominal pain, diarrhea, and weight loss were the most common symptoms. Small bowel radiology and colonoscopy were helpful in establishing the diagnosis. The difficulties in diagnosing tuberculous enteritis and the need to consider seriously the possibility of this disease in such patients is most important and is discussed.

Abdominal Pain↗
PubMed1999

Extra-pulmonary tuberculosis in patients with fever of unknown origin: clinical features and diagnosis.

One hundred twenty-nine patients with extra-pulmonary tuberculosis admitted at the Abbassia Fever Hospital, NAMRU-3 during a seven year period (1991 to 1997) are described. Tuberculous peritonitis was diagnosed in 35 patients, tuberculous lymphadenitis in 32, intestinal tuberculosis in 19, genito-urinary tuberculosis in 14, skeletal tuberculosis in 13, disseminated miliary tuberculosis in 11, and splenic tuberculosis in 5 patients. The clinical picture included fever, and weight loss accompanied by anaemia and an elevated erythrocyte sedimentation rate in all patients.

Adolescent↗