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[卢本巴希镰状细胞贫血相关消化系统疾病:流行病学与临床特征]

[Digestives diseases associated to sickle cell anemia in Lubumbashi: epidemiological and clinical aspects].

作者信息

Banza Manix Ilunga, Mulefu Jules Panda, Lire Lire Ipani, N'dwala Yannick Tietie Ben, Tshiamala Israel Badypwyla, Cabala Vincent de Paul Kaoma

机构信息

Université de Lubumbashi, Faculté de Médecine, Département de Chirurgie, Cliniques Universitaires de Lubumbashi, Lubumbashi, République Démocratique du Congo.

出版信息

Pan Afr Med J. 2019 Jul 26;33:253. doi: 10.11604/pamj.2019.33.253.18017. eCollection 2019.

Abstract

INTRODUCTION

sickle cell disease is a genetic disease with autosomal inheritance associated with haemoglobin structure abnormality which causes the formation of hemoglobin S. The purpose of our study was to collect data on digestive diseases in patients with sickle cell disease in Lubumbashi and to highlight their epidemiological and clinical features.

METHODS

We conducted a retrospective, descriptive, cross-sectional study at the Research Center for Sickle Cell Disease in Lubumbashi. All the records of patients on follow-up for sickle cell disease with digestive disease during our 3-year period (January 2015 to December 2017) were analyzed. Data were collected using a survey taking into account different study parameters including: age, sex, the reason for consultation, diagnosis, the type of vaso-occlusive crisis, the paraclinical examinations made, hydroxyurea treatment.

RESULTS

out of a total of 403 medical records examined we found 206 cases (n=206) of sickle cell disease associated with digestive disease, accounting for a rate of 51,11% of patients with sickle cell disease who suffered from digestive diseases. Both sexes were represented with a slight female predominance (51.94%) and a sex ratio M/F of 0.92. The most represented age ranges 1-6 years (32.52%), the average age was 11.8 years; the standard deviation was 21.9; the extreme ages were 13 months and 38 years. The reason for consultation was dominated by fever (60,67%), abdominal pain (44.66%) and digestive disorders (30,09%). Vaso-occlusive abdominal crises were found in 65 patients (31.55%) among whom 36 had only 1 crisis, 24 had 2 crises and 5 had 3 crises. Intestinal diseases were found in 121 patients (69,41%) dominated by intestinal parasites (found in 58 patients whose collection of stool samples showed 4 parasites: Yersinia enterocolitis, Entamoeba histolytica, Giardia intestinalis and Clostridium difficile). Gastric diseases were found in 105 patients ( 50,97%) divided into peptic ulcer (45 patients) and gastritis (60 patients); biliary vesicular disease was found in 40 patients (19.41%) including vesicular lithiasis without cholecystitis (32 patients), lithiasic cholecystitis (5 patients) and lithiasis in the main biliary tract (3 cases); there was 1 single case diagnosed with acute pancreatitis. The most common associated diseases in our study were respiratory diseases (169 cases;82,03%), oto-rhino-laryngological diseases (157 cases;76.21%), bony, vaso-occlusive crises (146 cases; 70,87%), urogenital diseases (64 cases; 31.06%) and malaria (51 patients; 24.75%). Hepatic diseases and diseases of the spleen were found in 18 cases (8.73%) and 47 cases (22,81%) respectively. Ultrasound was requested in 79 patients but only 31 of them underwent it because of the lack of financial means (it costs 20 U.S. dollars). In the case of clinically obvious splenomegaly, the search for Howell-Jolly bodies was requested in 23 patients but it was only performed in 2 patients because it costs 10 U.S. dollars). Routine blood count, hemoglobin, hematocrit, inflammatory assessment and thick drop examination were performed in all our patients but liver assessment, tests done on stool samples, urine test were recommended based on patient's complaint. Out of 206 patients, only 60 were under hydroxyurea treatment (29,16%).

CONCLUSION

digestive diseases are common in patients with sickle cell disease and account for almost half of patients with diagnosed sickle cell disease. Unfortunately, best management is limited by poverty leading to less very useful paraclinical examinations in patients with digestive diseases resulting from sickle cell disease.

摘要

引言

镰状细胞病是一种常染色体遗传性疾病,与血红蛋白结构异常有关,可导致血红蛋白S的形成。我们研究的目的是收集卢本巴希镰状细胞病患者消化系统疾病的数据,并突出其流行病学和临床特征。

方法

我们在卢本巴希镰状细胞病研究中心进行了一项回顾性、描述性横断面研究。分析了我们3年期间(2015年1月至2017年12月)镰状细胞病伴消化系统疾病患者的所有随访记录。使用一项调查收集数据,该调查考虑了不同的研究参数,包括:年龄、性别、就诊原因、诊断、血管闭塞性危机类型、所做的辅助检查、羟基脲治疗。

结果

在总共检查的403份病历中,我们发现206例(n = 206)镰状细胞病伴消化系统疾病,占患消化系统疾病的镰状细胞病患者的51.11%。男女均有,女性略占优势(51.94%),男女比例为0.92。最常见的年龄范围是1 - 6岁(32.52%),平均年龄为11.8岁;标准差为21.9;年龄极值为13个月和38岁。就诊原因主要是发热(60.67%)、腹痛(44.66%)和消化系统紊乱(30.09%)。65例患者(31.55%)出现血管闭塞性腹部危机,其中36例仅发生1次危机,24例发生2次危机,5例发生3次危机。121例患者(69.41%)患有肠道疾病,主要是肠道寄生虫(在58例患者中发现,粪便样本检测显示有4种寄生虫:小肠结肠炎耶尔森菌、溶组织内阿米巴、肠贾第虫和艰难梭菌)。105例患者(50.97%)患有胃部疾病,分为消化性溃疡(45例)和胃炎(60例);40例患者(19.41%)患有胆囊疾病,包括无胆囊炎的胆囊结石(32例)、结石性胆囊炎(5例)和主胆管结石(3例);有1例被诊断为急性胰腺炎。我们研究中最常见的相关疾病是呼吸系统疾病(169例;82.03%)、耳鼻喉科疾病(157例;76.21%)、骨骼、血管闭塞性危机(146例;70.87%)、泌尿生殖系统疾病(64例;31.06%)和疟疾(51例;24.75%)。分别有18例(8.73%)和47例(22.81%)患者患有肝脏疾病和脾脏疾病。79例患者被要求进行超声检查,但由于资金短缺,其中只有31例进行了检查(超声检查费用为20美元)。在临床上明显脾肿大的情况下,23例患者被要求检查豪-焦小体,但由于费用为10美元,仅对2例患者进行了检查。我们所有患者都进行了血常规、血红蛋白、血细胞比容、炎症评估和厚血膜检查,但根据患者的主诉建议进行肝脏评估、粪便样本检测、尿液检测。在206例患者中,只有60例接受羟基脲治疗(29.16%)。

结论

消化系统疾病在镰状细胞病患者中很常见,几乎占确诊镰状细胞病患者的一半。不幸的是,由于贫困,最佳治疗受到限制,导致对镰状细胞病所致消化系统疾病患者进行的非常有用辅助检查较少。

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