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1.
Mymensingh Med J ; 33(1): 219-228, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38163796

RESUMO

Successful percutaneous coronary intervention (PCI) to anomalous coronary arteries is technically challenging, particularly through transradial route. The application of appropriate techniques and devices may help overcome these challenges. The objective of this study is to explore the technical and procedural challenges in percutaneous coronary intervention (PCI) of anomalous origin of right coronary artery (AORCA) through the trans-radial route. This prospective study consisted of 25 consecutive patients who underwent PCI for an angiographically significant stenosis in AORCA from November 2017 to May 2019 at Ibrahim Cardiac Hospital & Research Institute (ICHRI). Demographic details and procedural data including numbers of catheters used, access, hardware, techniques, duration of procedure, volume of contrast and complications were recorded and statistically analyzed. The origin of AORCA was 48.0% each from the right and left coronary sinus, with 4.0% arising from the ascending aorta. Among those of right coronary sinus origin, superior take off was 83.3% and inferior take-off was 8.3%, with a further 8.3% originating from the left main, with a common origin with the left anterior descending (LAD) artery, from right coronary sinus. The mean age was 55.8±7.5 years. Diabetics were 84.0%, hypertensive 88.0%, dyslipidemic 68.0% and 20.0% had a history of smoking. Percutaneous coronary intervention (PCI) was performed successfully in 100.0% cases. Transradial access was the default route for coronary angiography in all cases. Angioplasty was performed trans-radially in 92.0% and trans-femoral in 8.0%, for two cases requiring switch over from radial to femoral route. The average number of guide catheters used was (2.0±1.0), (range: 1-4). The guide catheter hooked the coronary ostium selectively in 32.0%, off ostium in 56.0% and deep intubation was done in 12.0% cases. Anchoring wire to enhance guide support was used in 12.0%. 6 Fr guide extension catheter Guidezilla was used in 8.0% cases. The average duration of the procedure was 39.4 (range; 15-90) minutes, the average volume of contrast used was 67.0 (range: 30-150) ml. Average stent length was 28.6 (range; 12-43) mm. For PCI, Judkin's left (JL) and Judkin's Right (JR) were most commonly used guides (36.0% and 28.0% respectively), followed by multipurpose angled (MPA) guide (12.0%). The majority of the lesions stented were of ACC/AHA classification of type B (48.0%) followed by type A (36.0%) and type C (16.0%). Thrombus extraction was performed in a single case. One case was complicated by coronary artery dissection. PCI of AORCA through transradial route is technically challenging but feasible with a reasonable amount of contrast and radiation, and appropriate use of guides and techniques. Proper localization of ostium and selection of suitable guide is the key to success, aided by additional devices in the armamentarium of interventional cardiology such as guide extension catheter and anchoring wires.


Assuntos
Intervenção Coronária Percutânea , Humanos , Pessoa de Meia-Idade , Intervenção Coronária Percutânea/métodos , Vasos Coronários , Estudos Prospectivos , Resultado do Tratamento , Angioplastia , Angiografia Coronária/métodos
2.
Mymensingh Med J ; 30(3): 657-665, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34226452

RESUMO

Intramedullary nailing is a pillar in the treatment of femoral shaft fractures. But it is not possible in all cases especially in comminuted fractures. This study has been designed to explain the importance of Minimally Invasive Plate Osteosynthesis (MIPO) with the locking plate in the treatment of comminuted Femoral Shaft Fracture. Twenty (20) such patients were treated by MIPO and analysis has been done in this study to get fruitful result and to find out the effectiveness of this procedure who were admitted at Mymensingh Medical College Hospital, Mymensingh, a tertiary level hospital of Bangladesh from February 2018 to January 2019. Mean age of the patients were 49.20±14.41 years. Based on AO classification, there were 4, 8 and 8 patients belong to type A, B and C respectively. The union period for all the patients was in between 12 to 14 weeks. The mean union period was 12.90±1.997 weeks. Mean follow up period was 19.70±2.77 weeks. Mean full weight bearing period was 16.50±1.10 weeks. In Thoresen scoring system excellent result was 9(45%), good result was 10(20%) and fair result was 01(5%). Mal-alignment happened in two cases. However, delayed union and broken screws were found in two cases each of which was treated accordingly. Comminuted Femoral shaft fracture with MIPO procedure is more effective treatment than intramedullary nailing. Furthermore, mal-alignment is the basic complexity that must be taken away intraoperatively.


Assuntos
Fraturas do Fêmur , Fraturas Cominutivas , Adulto , Bangladesh , Placas Ósseas , Fraturas do Fêmur/diagnóstico por imagem , Fraturas do Fêmur/cirurgia , Fixação Interna de Fraturas , Fraturas Cominutivas/diagnóstico por imagem , Fraturas Cominutivas/cirurgia , Humanos , Pessoa de Meia-Idade , Procedimentos Cirúrgicos Minimamente Invasivos , Resultado do Tratamento
3.
Thromb Haemost ; 76(6): 1004-8, 1996 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-8972025

RESUMO

Despite many reports of individuals with congenital plasminogen deficiency and thrombosis, there is still uncertainty whether heterozygous deficiency represents a real thrombophilic risk factor or simply a coincidental finding. We have addressed this issue by testing for plasminogen deficiency in a cohort of 9611 blood donors. Out of 66 donors with reduced plasminogen activity on two occasions 28 were shown to have a familial deficiency state (including 3 with dysplasminogenaemia). Our observed prevalence rate for familial plasminogen deficiency, calculated at 2.9/1000 (95% CI = 1.9-4.2 per 1000), was not significantly different from that calculated from published reports of congenital plasminogen deficiency in thrombotic cohorts (5.4/1000). Furthermore, with only two exceptions, all 80 donors and relatives with familial deficiency were asymptomatic with regard to thrombosis-including a 29 year old donor with suspected compound heterozygous hypoplasminogenaemia. These findings add further weight to the argument that familial heterozygous plasminogen deficiency, at least in isolation, does not constitute a significant thrombotic risk factor. However, it remains uncertain whether plasminogen deficiency, when combined with other thrombophilic conditions, may become more clinically important.


Assuntos
Doadores de Sangue , Plasminogênio/deficiência , Trombose/etiologia , Adulto , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Plasminogênio/genética , Fatores de Risco
4.
Thromb Haemost ; 70(2): 281-5, 1993 Aug 02.
Artigo em Inglês | MEDLINE | ID: mdl-8236135

RESUMO

Interpretation of protein C (PC) levels in a given individual has several limitations. A normal PC activity does not necessarily exclude a genetic deficiency nor can a reduced level confirm it. Measuring PC amidolytic activity in 9,648 healthy blood donors has allowed identification of demographic factors which cause variation in PC activity and further hinder interpretation. PC activity displays a log normal distribution and significant variation with age. This is most marked in young adult males when mean PC activity rises from 0.86 iu/ml (15-19 years) to 1.04 iu/ml (45-49 years; P < 0.0001). Pre-menopausal females, who for most age ranges, have mean PC activity below their male contemporaries, show a less marked rise with age until the menopause when PC activity rises further. The use of hormonal contraceptive preparations is associated with an increase in mean PC activity of 0.05-0.08 iu/ml while smoking habit has no influence on PC activity. In view of these findings we strongly recommend the use of age and sex restricted reference ranges when interpreting PC activity.


Assuntos
Anticoncepcionais Orais Hormonais/farmacologia , Proteína C/análise , Fumar/sangue , Adolescente , Adulto , Fatores Etários , Idoso , Doadores de Sangue , Feminino , Humanos , Masculino , Menopausa , Pessoa de Meia-Idade , Valores de Referência , Fatores Sexuais
5.
Thromb Haemost ; 73(1): 87-93, 1995 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-7740502

RESUMO

Reported prevalence rates for protein C (PC) deficiency in the population at large have varied widely. The differences presumably reflect the existence of an apparently high number of clinically recessive forms of the deficiency. In an attempt to document more precisely the prevalence of PC deficiency in the healthy population we have measured PC activity in just under 10,000 blood donors in the West of Scotland. After repeat testing of donors with low results and then further observation and selection, 32 donors were identified who had individual mean PC activities below the age- and gender-specific study reference range. Assessment of available first degree relatives, and also PC gene analysis in 23 of these donors, allowed identification of at least 14 with an inheritable deficiency (8 by both family study and gene analysis, 3 by family study alone and 3 by gene analysis alone). Two recurring and seven unique point mutations, only one of which has been previously described, were identified. The observed prevalence of inherited PC deficiency was 1.45 per 1000 (95% CI, 0.79/1000 to 2.43/1000). However after correcting for the possibility of missing some genuine inherited deficiencies we estimated the prevalence to be as high as 1 in 500. All cases of hereditary deficiency were asymptomatic with regard to thrombosis and none had a strong family history of thrombosis.(ABSTRACT TRUNCATED AT 250 WORDS)


Assuntos
Deficiência de Proteína C , Adolescente , Adulto , Idoso , Sequência de Bases , Estudos de Coortes , Análise Mutacional de DNA , Suscetibilidade a Doenças/epidemiologia , Feminino , Genes Dominantes , Genes Recessivos , Predisposição Genética para Doença , Humanos , Masculino , Pessoa de Meia-Idade , Dados de Sequência Molecular , Mutação Puntual , Prevalência , Proteína C/genética , Fatores de Risco , Escócia/epidemiologia , Tromboembolia/genética
6.
Thromb Haemost ; 68(5): 506-10, 1992 Nov 10.
Artigo em Inglês | MEDLINE | ID: mdl-1455395

RESUMO

There is considerable doubt as to the importance of reduced plasminogen (PLG) activity as a risk factor for venous thrombosis. In the present study we have identified a wide range of PLG activities (25-200%) in a cohort of 9,611 blood donors. Males and females not taking hormonal contraceptives show a similar distribution of PLG, however, variation related to age appears to follow a different pattern in males and females. These differences are of doubtful clinical importance as are differences related to smoking. In contrast, females taking hormonal contraceptives or hormone replacement therapy (HRT) have up to 25% higher mean PLG levels in younger females but a less marked elevation (10%) is seen in 40-50 year olds. A PLG activity < 65% was recorded in 61 donors, none of whom appeared to have a history of thrombosis. These findings do not support the notion that reduced PLG is an important thrombophilic risk factor, however, further investigation of the donors with low PLG is required.


Assuntos
Plasminogênio/metabolismo , Adolescente , Adulto , Fatores Etários , Idoso , Anticoncepcionais Orais/efeitos adversos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Valores de Referência , Fatores de Risco , Fatores Sexuais , Fumar/efeitos adversos , Fumar/sangue , Tromboflebite/etiologia
7.
Am J Ophthalmol ; 129(2): 251-3, 2000 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-10682982

RESUMO

PURPOSE: To report a case of intracorneal hematoma occurring in association with Mooren ulceration. METHOD: Case report. RESULTS: In an 81-year-old man with bilateral Mooren ulceration, a dense intracorneal hemorrhage occurred in the right eye secondary to peripheral corneal neovascularization and was followed by slow resolution over a 3-year period. Following subsequent lamellar and penetrating keratoplasty, histopathologic examination demonstrated the association between the stromal neovascularization and the residual interlamellar hemorrhage, as well as phagocytosis of residual hemosiderin by macrophages. CONCLUSION: Intracorneal hematoma with spontaneous resolution has been documented clinically and histopathologically in an eye with Mooren ulceration.


Assuntos
Neovascularização da Córnea/complicações , Substância Própria/patologia , Úlcera da Córnea/complicações , Hemorragia Ocular/etiologia , Hematoma/etiologia , Idoso , Idoso de 80 Anos ou mais , Neovascularização da Córnea/patologia , Úlcera da Córnea/patologia , Hemorragia Ocular/patologia , Hemorragia Ocular/fisiopatologia , Hematoma/patologia , Hematoma/fisiopatologia , Humanos , Ceratoplastia Penetrante , Masculino , Remissão Espontânea
8.
Life Sci ; 33(12): 1113-8, 1983 Sep 19.
Artigo em Inglês | MEDLINE | ID: mdl-6888166

RESUMO

The kinetics of lipid peroxide decomposition catalysed by microsomal enzymes and inhibited by SKF-525 A, hexobarbital, phenobarbital and aniline were investigated. The results indicate that the in vitro interaction of hexobarbital and SKF-525 A (type I binding compounds) with microsomal cytochrome p-450 inhibits the peroxidase activity while the in vitro interaction of aniline (type II binding compound) only slightly affect the peroxidase activity. It is suggested that LAHPO and type I binding compounds are competing for the hydrophobic binding site on cytochrome p-450, while type II binding compounds such as aniline negate electron transfer non-competitively by combining with the heme.


Assuntos
Sistema Enzimático do Citocromo P-450/metabolismo , Peróxidos Lipídicos/metabolismo , Microssomos Hepáticos/metabolismo , Peroxidases/metabolismo , Compostos de Anilina/farmacologia , Animais , Hexobarbital/farmacologia , Cinética , Masculino , Fenobarbital/farmacologia , Proadifeno/farmacologia , Ratos , Ratos Endogâmicos
9.
Cornea ; 20(7): 708-10, 2001 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-11588421

RESUMO

PURPOSE: To report a family with aggressive, early-onset pterygium. METHODS: We examined all living relatives (with one exception) of a Saudi Arabian family and found three members with pterygium (onset occurred when the patients were at early 20s, 6, and 4 years of age). RESULTS: Multiple attempts at surgical removal, even in conjunction with application of topical mitomycin C and use of conjunctival autograft, failed to prevent recurrent pterygium, which advanced across the visual axis and produced profound visual disability in the three cases. CONCLUSION: Contrary to previously published reports of familial pterygium, two of the three cases had childhood age at onset and all three had an aggressive clinical course after the onset of recurrent pterygium after surgical resection; genetic identification may be beneficial.


Assuntos
Pterígio/genética , Adolescente , Adulto , Idade de Início , Feminino , Humanos , Masculino , Linhagem , Pterígio/patologia , Pterígio/cirurgia , Recidiva , Transtornos da Visão/etiologia , Acuidade Visual
10.
Int J Clin Pharmacol Ther ; 33(6): 315-21, 1995 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-7582381

RESUMO

With over a decade of extensive clinical use of cyclosporin A (CsA), assessment of its long-term safety implications is due. In this study the impact of long-term continuous use of CsA on a number of hematological and biochemical parameters in renal transplant patients was evaluated. Two groups of 13 patients each, one on conventional therapy (azathioprine + prednisolone) and the other on triple therapy (azathioprine + prednisolone + CsA) for 4 to 15 years post-transplantation were compared with respect to their current and overall laboratory values and clinical outcome. Laboratory values were also compared with those of 23 matched healthy subjects. No significant difference in the clinical outcome was found between conventional and triple therapy groups, however, the triple therapy group had significantly less favorable mean values compared to the conventional therapy group with respect to hemoglobin (12.1 +/- 2.2 vs 13.3 +/- 2.1 g/dl, p < 0.02), hematocrit (0.36 +/- 0.06 vs 0.42 +/- 0.03 l/l, p < 0.05), urea (13.0 +/- 3.7 vs 6.7 +/- 4.3 mmol/l, p < 0.01) and uric acid (460.0 +/- 112 vs 330 +/- 88 mumol/l, p < 0.05). The increase in serum uric acid levels in the triple therapy group was progressive throughout the post-transplant period. For the 19 other parameters measured corresponding mean values in the 2 groups were comparable. Mean laboratory values for many parameters in both groups, however, still differed from those in the control group. These results showed that kidney transplant patients on long-term triple therapy have more hematological and biochemical abnormalities and no better clinical outcome than those on conventional therapy.(ABSTRACT TRUNCATED AT 250 WORDS)


Assuntos
Ciclosporina/uso terapêutico , Imunossupressores/uso terapêutico , Transplante de Rim , Adulto , Azatioprina/administração & dosagem , Azatioprina/uso terapêutico , Biomarcadores/sangue , Ciclosporina/administração & dosagem , Ciclosporina/efeitos adversos , Quimioterapia Combinada , Feminino , Polarização de Fluorescência , Hematócrito , Hemoglobinas/metabolismo , Humanos , Imunossupressores/administração & dosagem , Imunossupressores/efeitos adversos , Masculino , Prednisolona/administração & dosagem , Prednisolona/uso terapêutico , Resultado do Tratamento , Ureia/sangue , Ácido Úrico/sangue
11.
J Pharm Pharmacol ; 38(8): 572-7, 1986 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-2876072

RESUMO

The Abbott TDx fluorescence polarization immunoassay (FPIA) system has been evaluated and compared with well-established high performance liquid chromatography (HPLC) for the determination of three anticonvulsant drugs: phenytoin, phenobarbitone and carbamazepine. These assays were evaluated for precision, calibration curve stability, specificity and accuracy. Within-run precision studies using control samples (n = 15) in the subtherapeutic, therapeutic, and toxic concentrations, resulted in coefficients of variation in the range of 1.79-3.99% (FPIA) and 1.16-2.52% (HPLC), respectively. Between-run precision ranged from 2.32-6.34% for FPIA and from 2.04-3.38% for HPLC. Comparison of 122 patient samples assayed with both methods indicated an extremely good analytical correlation (r = 0.96) for all three comparisons. The FPIA method offers significant advantages in calibration curve stability while maintaining accuracy and precision comparable with those of established HPLC procedures.


Assuntos
Carbamazepina/sangue , Fenobarbital/sangue , Fenitoína/sangue , Cromatografia Líquida de Alta Pressão , Polarização de Fluorescência , Humanos , Imunoensaio/métodos , Controle de Qualidade
12.
Pharmazie ; 30(9): 606-8, 1975 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-127182

RESUMO

The effect of a series of steroid hormones on the pentetrazol convulsing action, hexobarbital narcotic action and hepatic drug metabolizing enzyme activities was determined in rats. All steroid compounds used antagonized the pentetrazol effect: the most potent was cortisone and the least potent testosterone. Glucocorticoids and androgens shortened the hexobarbital sleeping time and increased the hepatic drug metabolizing enzyme activity. Estradiol exhibited the opposite effect, whereas progesterone and desoxycorticosterone did not affect these two parameters.


Assuntos
Corticosteroides/farmacologia , Hexobarbital/antagonistas & inibidores , Pentilenotetrazol/antagonistas & inibidores , Animais , Cortisona/farmacologia , Desidroepiandrosterona/farmacologia , Desoxicorticosterona/farmacologia , Dexametasona/farmacologia , Interações Medicamentosas , Ativação Enzimática/efeitos dos fármacos , Estradiol/farmacologia , Feminino , Fígado/metabolismo , Masculino , Metandrostenolona/farmacologia , Prednisolona/farmacologia , Progesterona/farmacologia , Ratos , Sono/efeitos dos fármacos , Testosterona/farmacologia , Fatores de Tempo
13.
Saudi Med J ; 22(12): 1092-5, 2001 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-11802183

RESUMO

OBJECTIVE: To evaluate the impact of Saudi national protocol on the diagnosis and management of asthma for optimizing theophylline use in children with severe asthma. To also review theophylline clearance and provide guidelines for individualization of doses. METHODS: Theophylline level was estimated in blood samples of 66 children with severe asthma who were admitted to King Abdulaziz University Hospital during the period 1998-1999. The theophylline doses given to these patients was reviewed and patients were categorized into 2 groups: Group one had received doses recommended by Saudi national protocol +/- 10%, group 2 had received doses <90% of that recommended by Saudi national protocol. The distribution of theophylline levels in blood samples of each group was estimated. Theophylline steady state level was used to estimate theophylline clearance using a standard pharmacokinetic equation. RESULTS: Out of the total samples from group one, 70% of theophylline levels were within therapeutic range, while only 10% of the total samples of group 2 were within therapeutic range. The mean theophylline clearance in children (1-8 years) was estimated as 0.092 +/- 0.023 and was found significantly higher than the mean theophylline clearance (0.069 +/- 0.014) which is observed in older children (9-13 years). CONCLUSION: Saudi national protocol theophylline dose guidelines had a favorable impact on the optimization of theophylline use in children with severe asthma. Guidelines that ensure accurate adjustment of doses on individual basis in view of drug level were suggested.


Assuntos
Teofilina/administração & dosagem , Adolescente , Criança , Pré-Escolar , Feminino , Fidelidade a Diretrizes , Humanos , Lactente , Masculino , Guias de Prática Clínica como Assunto , Arábia Saudita , Teofilina/sangue
14.
Saudi Med J ; 21(10): 945-9, 2000 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-11369959

RESUMO

OBJECTIVE: To record and update the sero-epidemiological status of Hepatitis A virus in Eastern Saudi Arabia. To investigate the main viral etiology of clinical hepatitis in children and discuss the possibility of introducing a Hepatitis A virus vaccine in this Province. METHODS: Examining serum specimens by Enzyme Linkage Immuno-Sorbet Assay technique for these parameters: Immunoglobulin M anti-hepatitis A virus, total immunoglobulin anti-hepatitis A virus, and in selected cases we checked for hepatitis B surface antigen and anti-hepatitis C virus. The study was carried out in the Virology Diagnostic Labs, of Dammam Regional Laboratories & Blood Bank, Dammam. A total of 12,357 serum samples were collected from 5876 healthy children, 5798 healthy adults, and 683 from clinically diagnosed hepatitis in children. The period of study was 12 years from February 1987 to January 1999. RESULTS: Hepatitis A virus prevalence showed 3% for pre-school age, 80% in older children and 93% in adults, while total prevalence was 86%. Breaking down the prevalence among children showed 3% in the <6 years age group, 62% in the 6 - <8 years age group, 71% in the 8 - <10 years age group, 83% in the 10 - <12 years age group and 93% in the 12 - <18 years age group. While the grand total among children was 78%. The prevalence of hepatitis viruses causing clinical hepatitis in children showed: 65% for hepatis A virus, 21% for hepatitis B virus, 7% for hepatitis C virus, 2% for double infection of hepatitis B virus + hepatitis C virus and 5% for non A, non B, non C. CONCLUSION: Hepatitis A virus infection starts dramatically high in school-age children, and then rises gradually with an increase in age. This reflects that our region is of pattern I class. There is no difference in the prevalence due to seasons of year, climate or sex. Hepatitis A virus is the leading cause of clinical hepatitis in children, followed by hepatitis B virus and hepatitis C virus. There is a possibility of starting to introduce hepatitis A virus vaccine among pre-school age children, as well as among hepatitis A virus negative adults that live in a higher socioeconomic environment within the country, which can be considered as islands of pattern II among pattern I areas.


Assuntos
Hepatite A/epidemiologia , Hepatite A/etiologia , Anticorpos Anti-Hepatite/sangue , Antígenos de Hepatite/sangue , Hepatovirus/imunologia , Imunoglobulina M/sangue , Adolescente , Distribuição por Idade , Biomarcadores/sangue , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Hepatite A/sangue , Hepatite A/imunologia , Hepatite A/prevenção & controle , Humanos , Masculino , Avaliação das Necessidades , Vigilância da População , Prevalência , Arábia Saudita/epidemiologia , Estudos Soroepidemiológicos , Fatores Socioeconômicos , Vacinação
15.
Saudi Med J ; 20(5): 378-81, 1999 May.
Artigo em Inglês | MEDLINE | ID: mdl-27631292

RESUMO

Full text is available as a scanned copy of the original print version.

16.
Saudi Med J ; 20(1): 71-4, 1999 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-27605277

RESUMO

Full text is available as a scanned copy of the original print version.

17.
East Mediterr Health J ; 5(6): 1188-95, 1999 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-11924110

RESUMO

In a 7-year retrospective analysis (1991-97) of the records of the Department of Haematology, Regional Laboratory and Blood Bank (Dammam), 54 patients from all parts of Saudi Arabia's Eastern Province were diagnosed with hereditary coagulation factor deficiencies. The largest group of patients, 42 haemophiliacs, included 4 non-Saudi patients. There were 39 haemophilia A or factor VIII deficiency patients, 2 haemophilia B or factor IX deficiency patients and 1 combined factor VIII and V deficiency patient. There were 5 Saudi patients with probable factor XIII deficiency, and 7 patients, all but one who were Saudi, had von Willebrand disease. The distribution of haemophilia patients in Eastern Province showed some differences compared with those reported from Riyadh and from Western countries. Among Saudis in Eastern Province, the number with suspected factor XIII deficiency, although low, was higher than that reported for other regions. The number of patients with haemophilia B and von Willebrand disease was lower than expected, when compared with the number of haemophilia A cases.


Assuntos
Deficiência do Fator V/epidemiologia , Deficiência do Fator V/genética , Hemofilia A/epidemiologia , Hemofilia A/genética , Hemofilia B/epidemiologia , Hemofilia B/genética , Doenças de von Willebrand/epidemiologia , Doenças de von Willebrand/genética , Deficiência do Fator V/diagnóstico , Feminino , Testes Genéticos , Hemofilia A/sangue , Hemofilia A/diagnóstico , Hemofilia B/sangue , Hemofilia B/diagnóstico , Humanos , Masculino , Linhagem , Contagem de Plaquetas , Vigilância da População , Prevalência , Características de Residência/estatística & dados numéricos , Estudos Retrospectivos , Arábia Saudita/epidemiologia , Índice de Gravidade de Doença , Doenças de von Willebrand/diagnóstico
18.
Ann Saudi Med ; 20(5-6): 367-70, 2000.
Artigo em Inglês | MEDLINE | ID: mdl-17264624

RESUMO

BACKGROUND: AA+/--thalassemia is known to be prevalent in the Eastern region of Saudi Arabia. There are no large scale reports regarding the effect of alpha-thalassemia on red cell indices of cord blood from Saudi Arabia. Similarly, there are no reports regarding the interaction of AA+/--thalassemia and the sickle cell gene in relation to red cell indices in cord blood. To address these issues, we undertook a study on neonatal cord blood samples. MATERIALS AND METHODS: In a prospective study, cord blood samples from 504 neonates from the Qatif area of the Eastern Province of Saudi Arabia were analyzed for complete blood counts (CBC) and cellulose acetate Hb electrophoresis. Hb S was confirmed by citrate agar Hb electrophoresis. RESULTS: There were 243 case samples with normal Hb electrophoresis (Hb A 27.2+/-7% and Hb F 72.6+/-7.7%). Their mean Hb (g/dL), RBC (x10(12) /L), Hct (%), MCV (fl), MCH (pg), MCHC (g/dL), RDW-SD (fl) and RDWCV (%) were 15.05+/-1.6, 4.5+/-0.5, 47.4+/-5.3, 106+/-8, 33.6+/-2.3, 31.8+/-1.7, 69.2+/-9.5 and 17.9+/-1.7, respectively. There were 136 cases with alpha-thalassemia trait (alphaTT), 57 cases with sickle cell trait (SCT) and 50 cases of sickle cell trait with alpha-thalassemia trait (SCT/alphaTT). There were 10 cases of Hb H disease (6 definite), including one with sickle cell disease (SCD) and two with SCT, Hb Bart's 23.9%-43.6%; four probable with Hb Bart's 10.9%-16.1%, and one with SCT. The effect on red cell parameters in Hb H disease were most pronounced. In addition, there were seven cases of SCD, four of whom had coexistent AA+/--thalassemia trait (SCD/alphaTT). CONCLUSION: The prevalence of alpha-thalassemia in this cohort of Saudi population was 39.99%. Hb H disease appeared as common as SCD. Sickle cell gene was seen in 23.4% of neonatal samples. alpha-Thalassemia gene significantly reduces MCH, MCV, RDW-SD, Hct, Hb, and increases RBC count in both normal or sickle cell trait neonates. Generally, the variation of red cell parameters is directly proportional to the amount of Hb Bart's in the cord blood. Sickle cell gene in itself produces low MCV, RDW-SD and MCH in cord blood. Further, normal reference values for red cell parameters of cord blood are established.

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