Case report on: - A case report on management and outcomes of Bart Syndrome

Abstract

A limited number of (neutropenia), a weaker and enlarged heart, skeletal myopathy, recurrent infections, and short height are all features of the rare illness known as Bart syndrome. Bart syndrome nearly always affects men. Dilated cardiomyopathy frequently manifests at birth or during the first few months of life in males with Bart syndrome. The heart muscle weakens and loses its capacity to pump blood with time. Elastic fibres may replace muscle fibres in some parts of the heart muscle in people with Bart syndrome, which adds to cardiomyopathy. Endocardial fibroelastosis is the name of the disorder that causes the muscle to thicken and affects how well it pumps blood. Heart failure can result from heart issues in persons with Bart syndrome. Rarely the cardiomyopathy improves with time, and those affected eventually show no signs of cardiac illness. Main symptoms and significant findings: A 5 months old baby boy was admitted to the hospital. Wardha on dated Jun 23 2022. Chief complaints of enlarged heart, low blood cell count, weakness of muscles, and fatigue.Additionally, there can be increased levels of chemicals like 3-methyglutaconic acid and 2-ethyl hydracrylic acid in the urine or blood. The primary diagnosis, therapeutic interventions, and outcomes: The patient was treated conservatively without surgery after the doctor discovered Bart syndrome following a physical examination and investigation. When the patient was brought to our hospital, the wounds had crusts and dead tissue but showed no evidence of infection. We removed crusts and dead tissue using mechanical and hydrodebridement techniques with sterile saline. Next, we administered an antimicrobial ointment to prevent disease and encourage wound contraction. A gauze bandage infused with petrolatum was also used to keep wounds moisturized. Antibiotics weren't administered intravenously or orally. The skin lesions were entirely epithelialized and healed after three months, leaving just a pigmented scar in their place. He underwent all treatments, and the results were positive. Her complaints have slightly decreased. Conclusion: We describe a case where Bart syndrome symptoms were found following our patient's enlarged heart, low blood cell count, muscle weakness, and exhaustion. These symptoms exactly matched those listed as official Bart syndrome symptoms. An uncommon congenital skin condition known as Bart syndrome has a distinctive clinical appearance. It's crucial to search for related oddities. The prognosis for the syndrome is generally good, however, the best results can be achieved by treating it as soon as feasible.