Aim: To analyze clinical manifestations, course and outcome of Langerhans Cell Histiocytosis in children in resource limited settings lacking salvage therapy. Study design: Observational retrospective study Place and duration of study: Department of Haematology/Oncology, The Children’s Hospital, Lahore Pakistan from 1st January 2011 to 31st December 2018. Methodology: Sixty-five patients with age range from<1 to 8 years included analysing their age, gender, clinical classification, course of therapy and outcome. The major treatment was composed of either prednisolone and vinblastine or cytarabine pulses. Results: There were 59% males and 41% females. Forty-seven (72%) patients presented with multi system-LCH with 49% Risk Organ involvement. Most of them 42 (65%) had bone lesions while 15patients (23%) presented with central nervous system involvement. Forty patients (61%) have completed treatment, 11(17%) left against medical advice and 12(18%)patients expired due to progressive disease and worsening infection. Only 2 patients were put on palliation with progressive brain parenchyma disease. 22 patients (34%) had reactivations of disease requiring therapy for more than one-year (p-value=0.06), while 15 (23%) patients received two cycles of initiation therapy before continuation therapy started. The treatment initiated >6 months after the onset of symptoms in 48 (74%) patients. Conclusion: Early diagnosis and timely initiation of therapy are of utmost importance to reduce mortality and morbidity. There is a dire need of social support to reduce treatment abandonment in low-middle-income countries LMIC. Keywords: Paediatric Langerhans cell histiocytosis, Resource-limited settings, Delayed diagnosis, Abandonment
Prednisolone is a 1, 2-dehydrocortisolwhich is highly potent synthetic glucocorticosteroid which has minimal mineralocorticoid activity. Prednisolone is used as antinflammatory or immunosuppressive agent, which indicated in the treatment of various conditions, including congenital adrenal hyperplasia, psoriatic arthritis, systemic lupus erythematous, bullous dermatitis herpetiformis, seasonal or perennial allergic rhinitis. Majorly it causes Cushing Syndrome, peptic ulceration, edema, hypokalemia, muscle weakness, behavioral changes. A 55 year old female patient was consulted in the dermatology department with chief complaints of swelling and redness of blisters on mouth and fore head, difficulty while swallowing since 2 months, past medical history of patient includes, she was already taking treatment for this condition which includes Tab. Chlophenaramine malate 4mg, Tab. Prednisolone 25 mg, Tab. Vitamin C 500 mg, Oint. Clobestalol 0.05% w/v, Glycerin lotion 5ml. After 15 days, patient had complaints of swelling of both lower limbs after administration of Tab. Prednisolone, this is the reason for hospital admission. Better vigilance is necessary for implementation of safe and effective treatment for each individual patient. In-order to prevent serious adverse drug reactions of this drug, close monitoring during treatment course, creating awareness, recognition of the problem and careful management of all patients who receive this medication are essential, because use of corticosteroids causes toxic effects like abdominal tenderness, fluid retention, insomnia, muscle weakness etc. and if close monitoring is not provided during treatment course, it can cause permanent disability, morbidity, mortality.
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