National Hospital of Sri Lanka

The COVID-19 pandemic - and the inconsistent government responses it revealed - has made the institutionalisation of robust Science Advice to Policymakers (SAP) an urgent imperative. Barring exceptions such as initiatives by the International Network for Governmental Science Advice, very few training programmes aim to develop capacity of policy makers, especially in the Global South. In response to these needs, the National Academy of Sciences of Sri Lanka and the Association of Academies and Societies of Sciences in Asia (AASSA), conducted a project titled Institutionalising Science Advice to Governments. Its key objectives was to develop an appropriate framework to understand the process of institutionalisation of SAP. The Colombo Framework so developed integrated four clusters: the SAP process; guiding values and principles; supporting institutional and legislative structures; and evaluating SAP. It was validated by experts and used for designing a questionnaire to assess current SAP practices across AASSA member countries which enabled data gathering on the diversity of models of SAP, their challenges and best practices. The framework also helped structure an international workshop to share country-level situation analyses, formulate roadmaps for implementation and build consensus on the relevance of institutionalising SAP. The latter culminated in the Colombo Declaration, urging governments to strengthen science-policy engagements. Short-term and long-term feedback from participants indicated high utility of the framework for understanding and operationalising SAP in diverse contexts. The framework offers a systematic approach for countries, especially in the Asia-Pacific region, to institutionalise SAP and for scientists to gain in-depth understanding of the process.

Haemobilia, caused by hepatic artery aneurysms leading to acute pancreatitis is a rare presentation. Pancreatitis is hypothesized to result from a similar mechanism to gallstone obstruction at the ampulla of Vater. Herein, we report a large common hepatic artery (CHA) and right hepatic artery (RHA) aneurysm with a common bile duct (CBD) fistula causing acute pancreatitis and upper gastrointestinal bleeding. A 61-year-old male, with nicotine use disorder and hypertension, presented to the emergency department for the first time with a sudden onset of severe epigastric pain radiating to the back, relieved by bending forward, and two episodes of haematemesis and melena. His laboratory findings showed anaemia, elevated amylase and bilirubin with predominant direct component. Imaging revealed a partially thrombosed fusiform CHA aneurysm extending to the RHA and porta hepatis with a fistula into the CBD. Following stabilization, operative management was carried out, which included CBD exploration with clot evacuation, fistula oversewing, cholecystectomy, Roux-en-Y hepaticojejunostomy, and ligation of the common hepatic artery proximal to the aneurysm after ensuring the presence of a replaced left hepatic artery and collateral supply to the liver. One-year post-operative follow-up angiogram showed complete thrombosis of the aneurysm with preserved hepatic perfusion. This case highlights haemobilia as a rare cause of acute pancreatitis, emphasizing the need for high clinical suspicion and timely surgical intervention.

Acute hepatic porphyrias are rare metabolic disorders that often present with recurrent neurovisceral symptoms and are frequently misdiagnosed. Definitive diagnosis relies on biochemical and genetic testing, which may be unavailable in resource-limited settings. We report a 36-year-old woman with recurrent episodes of severe colicky abdominal pain associated with vomiting and bowel disturbances, with minimal abdominal findings and repeatedly normal routine investigations. During an acute episode, urinary screening demonstrated a positive Hoesch test for porphobilinogen (PBG). Spectrophotometric analysis of urine total porphyrins revealed a Soret peak at approximately 405 nm, and the total urine porphyrin concentration was elevated at 349.635 nmol/L (reference: <300 nmol/L). Alternative causes of elevated urinary porphyrins were excluded clinically and biochemically. Due to the unavailability of intravenous hemin, the acute episode was managed with analgesia, supportive care, and carbohydrate, leading to symptom resolution. The patient was counselled on the avoidance of precipitating factors and long-term surveillance. This case illustrates the diagnostic value of clinical suspicion supported by basic biochemical testing in identifying probable acute hepatic porphyria in a resource-limited setting.

Abstract Cerebral arteriovenous malformations (AVMs) during pregnancy pose significant risks of hemorrhage, requiring careful multidisciplinary management. The optimal anesthetic and obstetric strategies remain unclear due to limited data. We report two cases of pregnant women with cerebral AVMs managed by a multidisciplinary team. Both patients underwent close neurological and fetal monitoring and elective cesarean deliveries under spinal anesthesia combined with norepinephrine infusion to maintain hemodynamic stability. The first case involved a primigravida with a large, unruptured right parietal AVM presenting with headache and transient visual symptoms. The second was a secundigravida presenting with seizures and subacute hemorrhages. Both deliveries were uneventful, with stable mean arterial pressures maintained intraoperatively, no hypertensive surges, and favorable maternal and neonatal outcomes. Postpartum plans included further neurovascular evaluation. These cases highlight the importance of individualized, multidisciplinary care in managing cerebral AVMs during pregnancy. Spinal anesthesia with norepinephrine infusion provided stable hemodynamic control and allowed continuous neurological monitoring, potentially reducing rupture risk. Further research is needed to establish standardized protocols.

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BackgroundCold agglutinin disease is one of main two types of cold-antibody autoimmune hemolytic anemias, which can be either primary or, commonly, secondary to an infection or underlying lymphoproliferative disease [1]. We report a rare case of refractory cold autoimmune hemolytic anemia as an initial presentation of gastric B cell lymphoma.Case presentationA 53-year-old Sri Lankan woman with diabetes mellitus, dyslipidemia, and hyperthyroidism presented with increased fatigue and shortness of breath on mild exertion for 1 month. She reported yellowing of eyes and passage of dark urine. Examination findings revealed pallor, icterus, and moderate splenomegaly with no hepatomegaly or lymphadenopathy. She was found to have hemoglobin of 8.3 g/dl with mean corpuscular volume 90.8 fL and mean corpuscular hemoglobin 30.4 pg. Elevated reticulocyte count (8%), lactate dehydrogenase (357 U/L), and indirect hyperbilirubinemia (total bilirubin 1.2 mg/dl and indirect bilirubin 0.6 mg/dl) was suggestive of hemolysis. The presence of red cell autoagglutination in the peripheral blood smear with direct antibody testing positivity for C3d (3+) specificity confirmed cold autoimmune hemolytic anemia. Her initial contrast enhanced computed tomography of the chest, abdomen and pelvis failed to show any enlarged lymph nodes or underlying malignancy, and a bone marrow biopsy only showed moderate erythroid hyperplasia. She had poor response to intravenous methylprednisolone pulses, mycophenolate mofetil, and intravenous rituximab. In view of refractory hemolysis, she underwent a repeat imaging, which showed a heterogeneous mass lesion arising from the greater curvature of the stomach with regional lymph node involvement. Gastroscopy revealed a submucosal mass arising from the gastric fundus with a smooth surface. Endoscopic ultrasound guided biopsy confirmed the diagnosis of high-grade B cell gastric lymphoma, which was identified as the culprit for refractory cold autoimmune hemolytic anemia.ConclusionThis case report highlights that refractory cold autoimmune hemolytic anemia can be an initial presentation of gastric B cell lymphoma. This also emphasizes the importance of reassessments and reimaging to exclude underlying secondary causes in cases of refractory cold autoimmune hemolytic anemias.