Aloni Michel Ntetani, Sysleyne Létycia Mizèle, Ekulu Pépé Mfutu, Babio Francis Lukebadio, Ngiyulu René Makuala, Gini-Ehungu Jean Lambert
Division of Paediatric Haemato-oncology and Nephrology, Department of Paediatrics, University Hospital of Kinshasa, School of Medicine, University of Kinshasa, Kinshasa, Democratic Republic of Congo.
Acta Paediatr. 2014 Aug;103(8):e365-9. doi: 10.1111/apa.12647. Epub 2014 Apr 29.
The care of children with resistant nephrotic syndrome (NS) in the Democratic Republic of Congo is compromised by resource deficiencies that range from inadequate healthcare budgets, to scarce laboratory facilities and inconsistent drug supplies. The aim of this study was to describe the clinical profile and management of children with nephrotic syndrome in Kinshasa, the country's capital and its largest city.
We retrospectively reviewed the medical records of 62 patients with a diagnosis of NS, who were seen in the Paediatric Nephrology Unit at the University Hospital of Kinshasa between January 1983 and January 2008.
Of the 62 children diagnosed with nephrotic syndrome, 33 were boys and 29 were girls, giving a male to female ratio of 1.14:1. Their median age at initial presentation was 7.5 years (range: 1.5-13 years) with a peak incidence at six to nine years of age (38.7%). No cases of plasmodium malariae were isolated in our series. Sixteen (25.8%) of the children had resistant corticoid nephrotic syndrome and six of the children (9.7%) died.
Age, the prevalence of steroid-resistant NS and the mortality rate in our series were higher than those reported in Asian and Western countries.
刚果民主共和国资源匮乏,从医疗预算不足到实验室设施稀缺以及药品供应不稳定,这使得耐药性肾病综合征(NS)患儿的护理受到影响。本研究的目的是描述该国首都及最大城市金沙萨肾病综合征患儿的临床特征及治疗情况。
我们回顾性分析了1983年1月至2008年1月期间在金沙萨大学医院儿科肾病科就诊的62例确诊为NS的患者的病历。
在62例诊断为肾病综合征的儿童中,33例为男孩,29例为女孩,男女比例为1.14:1。他们初次就诊时的中位年龄为7.5岁(范围:1.5 - 13岁),发病高峰在6至9岁(38.7%)。我们的系列病例中未分离出间日疟原虫病例。16例(25.8%)儿童患有皮质激素耐药性肾病综合征,6例(9.7%)儿童死亡。
我们系列病例中的年龄、激素耐药性NS的患病率和死亡率高于亚洲和西方国家报道的水平。