AIM: To assess the distribution of human leukocyte antigen (HLA)-DQ2 and -DQ8 in Iranian celiac disease (CD) patients and compare them to healthy Iranian controls.
<strong>Background: </strong>Lupus nephritis (LN) is one of the most common presentations of Systemic lupus erythematosus (SLE). Cyclophosphamide is one of the key immunosuppressive agents for the manageme...<strong>Background: </strong>Lupus nephritis (LN) is one of the most common presentations of Systemic lupus erythematosus (SLE). Cyclophosphamide is one of the key immunosuppressive agents for the management of LN. Leflunomide is an isoxazole immunomodulatory agent has been shown to be safe, well tolerated and effective in SLE and LN. <strong>Objective: </strong>To evaluate the outcome of leflunomide in the treatment of proliferative lupus nephritis compared to cyclophosphamide. <strong>Method: </strong>This randomized clinical trial was held in Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh from July 2017 to August 2019. A total of 66 patients of proliferative lupus nephritis who need induction therapy were enrolled in this study. Leflunomide 100 mg/day for consecutive 3 days followed by 0.5 mg/kg/day in divided dose was given in experimental group (n = 32) and intravenous cyclophosphamide 0.5 gm/m2 of body surface area monthly pulse was given in control group (n = 34). All study patients have received prednisolone and hydroxychloroquine according to KDIGO guideline then followed up monthly for 6 months. Outcomes were measured at 6th month by renal function [S. Creatinine, 24 hours urinary total protein (24-hr UTP)], changes in SELENA-SLEDAI score, anti-ds DNA level, serum complement levels (serum C3 & C4), remission (complete/partial) and adverse drug responses.<strong> Result:</strong> In experimental group, remission occurred in 18 (56.3%) patients and no remission in 14 (43.7%) patients. In control group, remission occurred in 24 (70.6%) patients and no remission in 10 (29.4%) patients. Adverse effects in experimental group were: elevated ALT (6.3%), hypertension (12.5%), infection (6.3%) and amenorrhea (12.5%). In control group, adverse effects were mainly leucopenia (5.9%), infection (17.7%) and amenorrhea (29.4%). Intergroup analysis for treatment responses and adverse effects showed no significant difference (p > 0.05). <strong>Conclusion:</strong> Leflunomide combined with prednisolone is effective in the induction treatment of proliferative lupus nephritis in Bangladeshi patients in terms of response rate and adverse effects.展开更多
Transgrediens et progrediens palmoplantar keratoderma, known as Greither’ s syndrome,was originally described in 1952 and is characterized by diffuse keratoderma of the palms and soles,extending to the back aspects(t...Transgrediens et progrediens palmoplantar keratoderma, known as Greither’ s syndrome,was originally described in 1952 and is characterized by diffuse keratoderma of the palms and soles,extending to the back aspects(transgrediens)and involving the skin over the Achilles’ tendon. Patchy hyperkeratosis also develops on the shins,knees,elbows,and sometimes on the skin flexures. We describe two unrelated families affected with Greither’ s syndrome, in which the same dominant missense mutation gave rise to the amino acid change N188S in K1. The previously reported cases of Greither’ s syndrome showed phenotypic variability suggestive of different underlying gene defects. Our findings suggest that at least some cases of Grei- ther’ s syndrome are caused by keratin mutations.展开更多
Anti-tumour necrosis factor-α therapy of Crohn’ s disease (CD) with infliximab has proved a major advance in the treatment of patients with difficult disease. We report our experience of the use of infliximab in a p...Anti-tumour necrosis factor-α therapy of Crohn’ s disease (CD) with infliximab has proved a major advance in the treatment of patients with difficult disease. We report our experience of the use of infliximab in a patient with recalcitrant vulval CD. The introduction of infliximab has improved disease control, although a search for an optimum remittive dosing schedule is ongoing.展开更多
文摘AIM: To assess the distribution of human leukocyte antigen (HLA)-DQ2 and -DQ8 in Iranian celiac disease (CD) patients and compare them to healthy Iranian controls.
文摘<strong>Background: </strong>Lupus nephritis (LN) is one of the most common presentations of Systemic lupus erythematosus (SLE). Cyclophosphamide is one of the key immunosuppressive agents for the management of LN. Leflunomide is an isoxazole immunomodulatory agent has been shown to be safe, well tolerated and effective in SLE and LN. <strong>Objective: </strong>To evaluate the outcome of leflunomide in the treatment of proliferative lupus nephritis compared to cyclophosphamide. <strong>Method: </strong>This randomized clinical trial was held in Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh from July 2017 to August 2019. A total of 66 patients of proliferative lupus nephritis who need induction therapy were enrolled in this study. Leflunomide 100 mg/day for consecutive 3 days followed by 0.5 mg/kg/day in divided dose was given in experimental group (n = 32) and intravenous cyclophosphamide 0.5 gm/m2 of body surface area monthly pulse was given in control group (n = 34). All study patients have received prednisolone and hydroxychloroquine according to KDIGO guideline then followed up monthly for 6 months. Outcomes were measured at 6th month by renal function [S. Creatinine, 24 hours urinary total protein (24-hr UTP)], changes in SELENA-SLEDAI score, anti-ds DNA level, serum complement levels (serum C3 & C4), remission (complete/partial) and adverse drug responses.<strong> Result:</strong> In experimental group, remission occurred in 18 (56.3%) patients and no remission in 14 (43.7%) patients. In control group, remission occurred in 24 (70.6%) patients and no remission in 10 (29.4%) patients. Adverse effects in experimental group were: elevated ALT (6.3%), hypertension (12.5%), infection (6.3%) and amenorrhea (12.5%). In control group, adverse effects were mainly leucopenia (5.9%), infection (17.7%) and amenorrhea (29.4%). Intergroup analysis for treatment responses and adverse effects showed no significant difference (p > 0.05). <strong>Conclusion:</strong> Leflunomide combined with prednisolone is effective in the induction treatment of proliferative lupus nephritis in Bangladeshi patients in terms of response rate and adverse effects.
文摘Transgrediens et progrediens palmoplantar keratoderma, known as Greither’ s syndrome,was originally described in 1952 and is characterized by diffuse keratoderma of the palms and soles,extending to the back aspects(transgrediens)and involving the skin over the Achilles’ tendon. Patchy hyperkeratosis also develops on the shins,knees,elbows,and sometimes on the skin flexures. We describe two unrelated families affected with Greither’ s syndrome, in which the same dominant missense mutation gave rise to the amino acid change N188S in K1. The previously reported cases of Greither’ s syndrome showed phenotypic variability suggestive of different underlying gene defects. Our findings suggest that at least some cases of Grei- ther’ s syndrome are caused by keratin mutations.
文摘Anti-tumour necrosis factor-α therapy of Crohn’ s disease (CD) with infliximab has proved a major advance in the treatment of patients with difficult disease. We report our experience of the use of infliximab in a patient with recalcitrant vulval CD. The introduction of infliximab has improved disease control, although a search for an optimum remittive dosing schedule is ongoing.
