Introduction: Flexion deformity of the knee is a common complication following recurrent haemarthrosis in persons with haemophilia (PWH) on episodic factor replacement therapy, restricting independent mobility. There is limited literature on the comprehensive management of this condition. This report provides the outcome of a staged multidisciplinary approach for the correction of knee flexion deformity (KFD) even in limited resource settings.
View Article and Find Full Text PDFPost- transplantation lymphoproliferative disorders (PTLD) are uncommon neoplasms that complicate the post transplantation period. The incidence of PTLD and outcome post liver transplantation is sparsely described. Children who undergo liver transplantation are at higher risk of PTLD than adults.
View Article and Find Full Text PDFClin Pharmacol Ther
January 2024
A toxicity-reduced conditioning regimen with treosulfan, fludarabine, and thiotepa in patients with high-risk β-thalassemia major has significantly improved hematopoietic stem cell transplantation (HCT) outcomes. However, complications resulting from regimen-related toxicities (RRTs), mixed chimerism, and graft rejection remain a challenge. We evaluated the dose-exposure-response relationship of treosulfan and its active metabolite S, S-EBDM, in a uniform cohort of patients with β-thalassemia major to identify whether therapeutic drug monitoring (TDM) and dose adjustment of treosulfan is feasible.
View Article and Find Full Text PDFIntroduction: Haemophilia patients not treated with primary or secondary prophylaxis, often present with recurrent haemarthrosis. Knee is the most frequently involved joint leading to disabling knee flexion deformity (KFD). Here, we present a retrospective study of our experience on the role of mobilization under general anaesthesia in the correction of KFD.
View Article and Find Full Text PDFIndian J Endocrinol Metab
January 2019