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INTRODUCTION: Paroxysmal nocturnal haemoglobinuria (PNH) is a clonal hematopoietic disorder, where there is deficiency of glycosylphosphatidylinositol (GPI) anchored proteins in the cell membrane, leading to increased complement sensitivity of red blood cells, intravascular hemolysis and vascular inflammation. Arterial and venous strokes in patients with PNH are a rarity posing significant diagnostic and therapeutic challenges. We report our experience with management of PNH patients with cerebrovascular emergencies. METHODS: We report 2 patients with PNH, one who was previously diagnosed with PNH and had arterial stroke, the other had an index presentation of cerebral venous sinus thrombosis (CVT) and was subsequently diagnosed with PNH. We also present the systematic review of literature reporting similar cases, highlighting the challenges in management. RESULTS: Both patients presented to our centre with cerebrovascular emergency. The first patient was a diagnosed with PNH, and presented with left hemispheric infarction caused by thrombosis of middle cerebral artery. He was thrombolysed and underwent mechanical thrombectomy, which was unsuccessful in view of repeated re - thrombosis of the vessel. The patient survived with significant disability. The second patient had severe cerebral venous sinus thrombosis with large right hemispheric hemorrhagic venous infarction. She underwent emergency decompressive hemicraniectomy complicated by massive blood loss and disseminated intravascular coagulation. She subsequently had recurrent life threatening intracranial bleed secondary to platelet transfusions, thrombocytopenia, and use of contrast agents. She progressed to develop Budd Chiari syndrome and was initiated on Eculuzimab. She became transfusion independent, however remained in minimally conscious state and succumbed to sepsis. CONCLUSIONS: Management of arterial and venous strokes is complex in patients with PNH. Invasive procedures and platelet transfusions are to be avoided in acute thrombosis, till robust evidence is available establishing the safety of the same in patients with PNH. Eculuzimab is a promising option, but far from reach for patients in developing countries.
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BACKGROUND: Multiple cerebral venous sinus thrombosis (CVT) registries from various geographical regions indicate that female gender, the use of contraceptive pills, pregnancy and puerperium are important risk factors. In this study, we report the changes in the epidemiology of patients with CVT managed over the past 26 years. METHODS: The CMC Vellore CVT registry is a prospectively maintained database at the Christian Medical College, Vellore since January 1995. Stata software was used to analyse the data and assess the changes in the incidence, age and gender distribution over the previous 26 years. RESULTS: Among 1701 patients treated during the study period, 908 (53%) were women and 793 (47%) were men. The mean incidence of CVT was 49 per 100 000 admissions before 2010, which increased to 96 per 100 000 after 2010. Male gender had a higher odds of developing CVT (OR - 2.07 (CI 1.68 to 2.55, p<0.001). This could be attributed to the declining incidence of postpartum CVT after 2010 compared with the decade before 2010 (50% vs 20%). The mean age at presentation had increased from 24.5 to 33.2 years in the last decade. CONCLUSIONS: There was a clear change in the gender pattern from being a condition with female preponderance, to one where equal or more men are being affected. Lower incidence of postpartum CVT cases could be the driving factor. An increase in the overall incidence of CVT cases was noted, probably due to a higher index of clinical suspicion and better diagnostic imaging modalities.
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We conducted a retrospective observational study to describe the clinical profile and outcomes of patients admitted with a diagnosis of dengue fever in a tertiary hospital in South India. A total of 159 patients admitted from April 2014 to October 2018 were included in the study. Vomiting (70.4%), myalgia (60.4%), headache (42.1%), abdominal pain (38.4%), bleeding (38%), and rash (37.1%) were the most common symptoms at presentation. The mean duration of hospital stay was 4.9 days (SD ± 2.4), and the median cost was INR 19,708 ($285) (IQR INR 12,968-32,056 ($188-$305)). Major bleeding was associated with elevated SGOT and SGPT, severe dengue, and secondary dengue. Mortality was associated with elderly age; elevated total leukocyte count, serum bilirubin, serum creatinine, SGOT, and SGPT; and high SOFA score. In view of these observations, we recommend stratifying patients according to the WHO classification of dengue and avoiding the use of thrombocytopenia as a single marker of the severity of the illness.
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Listeriosis is a food borne illness of significant public health concern, caused by consumption of food contaminated by gram negative bacilli, Listeria monocytogenes. Clinical listeriosis is relatively rare and it has varying spectrum of presentation, ranging from severe sepsis in immune-compromised individuals, febrile gastroenteritis and meningo-encephalitis in infants and adults. This disease is under reported in developing nations due to the lack of awareness and inadequate laboratory facilities to promptly isolate and identify the organism. We report a case of sporadic food-borne listeriosis, in an otherwise healthy individual presenting with meningo-encephalitis. Prompt identification and appropriate antibiotic therapy led to a favorable outcome.