Contractor Jaikumar B, Radha Venkatesan, Shah Krati, Singh Praveen, Tadepalli Sunil, Nimbalkar Somashekhar, Mohan Viswanathan, Shah Pratik
GMERS Medical College, Panchmahal, Godhra 389120, Gujarat, India.
Congenital Hyperinsulinism India Association (CHIA), Anand 388325, Gujarat, India.
Med Sci (Basel). 2025 Apr 1;13(2):37. doi: 10.3390/medsci13020037.
India's population complexity presents varied challenges in genetic research, and while facilities have gained traction in tier-1 and -2 cities, reliance on international collaborations often delays such investigations. COVID-19 further exacerbated the issues with such sample sharing. Congenital Hyperinsulinism (CHI) is a rare genetic disorder of pancreatic β-cells causing hypoglycaemia in children due to abnormal insulin secretion. Given India's high birth rate and consanguineous populations, annual CHI cases are estimated to be around up to 10,000, with up to 50% having unexplained genetic causes. Diffuse or atypical lesions in such patients often necessitate near-total-pancreatectomy, risking pancreatic exocrine insufficiency and diabetes, requiring lifelong therapy. Also, novel genetic variations complicate accurate diagnosis, risk assessment, and counselling, emphasising the need for rapid genetic assessment to prevent neurological injuries and inform treatment decisions. Despite significant efforts at many institutes, there are no dedicated organisations for CHI in India. With the implementation of the National Policy for Rare Diseases 2021, we plan to form a non-profit organisation, "Congenital Hyperinsulinism India Association (CHIA)", comprising paediatric endocrinologists, paediatricians, geneticists, and independent researchers. The aims of this association are to generate a national database registry of patients, formulate a parent support group and CHIA consortium, design patient information leaflets, as well as foster genomic collaborations and promote clinical trials. Such steps will help sensitise the health authorities and policy makers, urging them to improve the allocation of health budgets for rare diseases, as well as empower patients and their families, contributing towards a better quality of life.
印度的人口复杂性给基因研究带来了各种挑战。虽然一级和二级城市的相关机构已逐渐受到关注,但对国际合作的依赖常常会延误此类调查。新冠疫情进一步加剧了样本共享方面的问题。先天性高胰岛素血症(CHI)是一种罕见的胰腺β细胞基因紊乱疾病,由于胰岛素分泌异常,会导致儿童低血糖。鉴于印度的高出生率和近亲通婚人口,估计每年的CHI病例多达10000例,其中高达50%的病例有不明的基因病因。这类患者的弥漫性或非典型病变往往需要进行近乎全胰腺切除术,存在胰腺外分泌功能不全和糖尿病的风险,需要终身治疗。此外,新的基因变异使准确诊断、风险评估和咨询变得复杂,这凸显了进行快速基因评估以预防神经损伤并为治疗决策提供依据的必要性。尽管许多机构付出了巨大努力,但印度仍没有专门针对CHI的组织。随着《2021年罕见病国家政策》的实施,我们计划成立一个非营利组织,即“印度先天性高胰岛素血症协会(CHIA)”,成员包括儿科内分泌学家、儿科医生、遗传学家和独立研究人员。该协会的目标是建立一个全国性的患者数据库登记处,组建一个家长支持小组和CHIA联盟,设计患者信息手册,促进基因组合作并推动临床试验。这些举措将有助于提高卫生当局和政策制定者的认识,促使他们增加对罕见病卫生预算的分配,同时增强患者及其家庭的能力,为提高生活质量做出贡献。