Challenges faced in establishing a pediatric liver transplant program in a lower-middle-income country with free healthcare service.

BACKGROUND: Liver transplant is the cure for children with liver failure. Sri Lanka is a lower-middle-income country with a predominant free, state health system. Pediatric liver transplant program in Sri Lanka is still in the budding state where the initial experience of the program is yet to be documented. METHODS: A retrospective review was performed including the clinical characteristics of all pediatric liver transplant recipients of Colombo North Centre for Liver Diseases since the inception of the program from June 2020 to May 2023. RESULTS: There were 14 PLT performed in 3 years. The median recipient age and weight were 8 years (6 months-15 years) and 23.3 kg (6.4-49.2), respectively. The majority were boys (64%). All were from low-income backgrounds. Indications for LT were acute liver failure (5/14), decompensated chronic liver disease (5/14), and acute on chronic liver failure (4/14). Underlying liver diseases were Wilson disease (6/14), autoimmune liver disease (3/14), biliary atresia (2/14) and progressive familial intrahepatic cholestasis type 3 (1/14), and unknown etiology (2/14). The majority were living donor liver transplants (86%). Of the living donors, 42% (5/12) were Buddhist priests. There were three immediate deaths and two late deaths. The 3-month survival was 78%, and overall survival was 64%. Living donor transplants carried a higher success rate (92%) compared to diseased donor transplants (0%; 2/2). CONCLUSIONS: Initial experience of pediatric liver transplant program of Sri Lanka is promising despite being established in a free healthcare system amidst the crisis circumstances.

Imaging in pediatric disorders of the gut-brain interactions: current best practice and future directions.

INTRODUCTION: Disorders of Gut-Brain Interactions (DGBI) are a common clinical problem in children and pose significant challenges to the attending pediatrician. Radiological investigations are commonly ordered to evaluate these children. AREA COVERED: This review focuses on the current best practice of using radiological investigations in DGBIs and how novel radiological investigations could revolutionize the assessment and therapeutic approach of DGBI in children. EXPERT OPINION: We believe imaging in DGBI is still in its early stages, but it has the potential to revolutionize how we diagnose and treat children with DGBI. As the understanding of the gut-brain axis continues to grow, we can expect to see the disappearance of conventional imaging techniques and the emergence of more sophisticated imaging techniques with less radiation exposure in the future which provide more clinically meaningful information regarding the gut-brain axis and its influence on intestinal function. Some of the novel imaging modalities will be able to broaden our horizon of understanding DGBI in children providing more useful therapeutic options to minimize their suffering.

Natalizumab-Related Progressive Multifocal Leukoencephalopathy-Immune Reconstitution Inflammatory Syndrome: A Case Report Highlighting Clinical and MRI Features.

Multiple sclerosis (MS) patients treated with natalizumab often face the uncommon but severe complication of developing progressive multifocal leukoencephalopathy (PML). PML may be further complicated by immune reconstitution inflammatory syndrome (IRIS) after the removal of the drug. Since both PML and IRIS are associated with high morbidity and mortality rates, early clinical and radiological diagnosis of these complications is of paramount importance. Here, we report a case of an adult male patient who was diagnosed with PML after receiving natalizumab therapy for 6 years for the treatment of MS. Upon cessation of natalizumab, he presented with a paradoxical worsening of clinical and radiological findings consistent with an inflammatory brain injury due to IRIS. He was treated with high dose corticosteroid therapy followed by a gradual improvement in clinical and imaging findings. This article illustrates the magnetic resonance imaging (MRI) features of natalizumab-associated PML-IRIS, along with a brief overview of its clinical features, complications and management strategies.

A case report on 1-year follow-up of bilateral thalamic glioma.

Bilateral thalamic glioma is one of the rarest tumor occurrences, representing a small fraction of thalamic gliomas, which only accounts for 1-1.5% of all brain tumors. It is usually a diffuse, low-grade astrocytoma (WHO grade II), seen mainly in adults, with approximately 25% of them involving children under the age of 15. Radiotherapy is the main mode of treatment since surgical intervention is limited to a role of biopsy and management of secondary effects, due to the deep brain location of the lesion and the complexity of the involved structures. We report a 1-year follow-up of a 55-year-old female patient with bilateral WHO grade II thalamic astrocytoma. Following histological and neuroradiological consensus regarding the diagnosis, the patient was referred for radiotherapy. The effectiveness of available therapy and long-term neuroradiological follow-up is not reliably established due to rapid fatal evolution following diagnosis. Contrary to the norm, our patient showed stable disease with radiotherapy for a 1-year period.

Balo's Concentric Sclerosis with Acute Presentation and Co-Existing Multiple Sclerosis-Typical Lesions on MRI.

Balo's disease also known as Balo's concentric sclerosis (BCS) is a rare demyelinating disorder which is characterized pathologically and radiologically by concentric rings of demyelinated and relatively myelin-preserved white matter. We describe the case of a 25-year-old female presenting with stroke-like symptoms who was diagnosed with BCS on MRI. At clinical onset, she also showed co-existing multiple sclerosis (MS)-typical lesions in the brain. This report extends the recent concept that BCS may share similar pathogenesis and radiological progression as MS lesions, that they may occur simultaneously in the same patient and that they may represent different manifestations of the same disease process. Also, BCS needs not be a fulminant disease and may often show a benign self-limiting course as in our case.