Dengue virus (DENV) infection is increasing with rapid urbanization in India. Treatment of DENV infection is mainly supportive with no specific antiviral therapy. Although most patients show mild illness, some have a severe disease course such as dengue hemorrhagic syndrome, dengue shock, multi-organ failure, and death. The cause for severity is not fully understood. Currently, there are no methods available to predict the course of the illness. Hence, it is crucial to develop an early biomarker to predict the course of dengue illness which can aid in vigorous monitoring and early intervention. Here, we tried to establish a correlation between serum ferritin and severity of dengue illness. We measured ferritin levels in 100 dengue-positive cases on day 1 (D1) (febrile phase) and day 4 (D4) (defervescence or convalescent) of admissions to compare the levels with the severity of the disease. On D1, the serum ferritin level was a "good" predictor of severe dengue, with an area under the curve (AUC) of 0.863 with standard error (SE) = 0.043 and a 95% CI from 0.778 to 0.947 (P < 0.05). On D4, serum ferritin was an "excellent" predictor of severe dengue, with an AUC of 0.947 with SE = 0.021 and a 95% CI from 0.907 to 0.988 (P < 0.05). Serum ferritin is an inexpensive and easily accessible biomarker that can assist in monitoring and prognosticating the dengue-positive patients. This biomarker also directs us to explore the underlying pathogenetic mechanism in severe dengue, which can lay a foundation for future targeted therapeutic options to combat severe illness.
<b><i>Background:</i></b> Knobloch syndrome (KS) is a rare autosomal recessive disorder associated with multiple ocular and cranial abnormalities. Occult occipital skull defect or encephalocele should raise suspicion of this disease. It is never reported in neurosurgical literature, possibly due to a lack of clinician familiarity, leading to underdiagnosis and inadequate management. Our patient also had seizures, which is a sporadic presentation of this syndrome. <b><i>Case Description:</i></b> Here, we report a clinico-radiologic finding of a 7-year-old boy who presented with seizures, cataracts, and an occipital bone defect along with bilateral subependymal heterotopias and polymicrogyria. <b><i>Conclusions:</i></b> This case highlights the importance of consideration of this syndrome in children with a midline occipital bone defect with or without encephalocele and seizures. Early recognition of this presentation is critical for obtaining access to appropriate genetic counseling and subsequent monitoring and prevention of complications by surgical intervention.
Objective To study the prevalence of different NPSLE manifestations in our cohort and to compare clinical and immunological features and outcomes including mortality of patients with NPSLE and SLE controls without NP involvement. Methods This was a retrospective study in a tertiary care referral centre. All patients of SLE seen in the last 10 years and fulfilling the SLICC criteria with neuropsychiatric manifestations as per the ACR definitions were included. Patients of SLE without NP involvement were sequentially assigned as controls in a ratio of 1:2. Results Of the 769 patients diagnosed with SLE from Jan 2011 to December 2020, 128 (16.6%) had NPSLE manifestations as per the ACR definitions. The commonest NPSLE manifestation was seizures (6.5%) followed by cerebrovascular accident (3.9%). NPSLE manifestation occurred at the first presentation of SLE in 99/128 (77.3%) patients and 58 (45.3%) patients had more than one NPSLE manifestation. Lupus anticoagulant and anticardiolipin antibody were tested in 120 patients and were positive in 16 (13.3%) and 12 (10%), respectively. No difference was found in anti-ribosomal p, lupus anticoagulant and anticardiolipin antibodies between the cases and controls. Twenty-one (16.4%) deaths occurred in patients with NPSLE (median follow-up of 40 months) as compared to 13 (5%) in controls (median follow-up of 32 months) ( p = <0.001). The cumulative survival of patients with NPSLE was lower as compared to controls ( p < 0.001). Relapse of NPSLE was seen in 11(8.6%) patients and was associated with mortality ( p = 0.017). Conclusions Seizures and cerebrovascular accidents are the commonest NPSLE syndromes in our patients. The presence of NPSLE was associated with high mortality in Indian patients with lupus.
Background: Septic emboli are commonly attributed to infective endocarditis and can present with a variety of symptoms including altered mental status and focal neurological deficits. Here, we reviewed images of septic emboli with hemorrhagic conversion in a patient with sepsis and a psoas abscess. We aim to show the classical image findings in septic embolism to brain, which is sparsely described in literature and the report differentiates the septic embolism from disseminated intravascular coagulation which can present with almost identical image findings. Case Description: A 53-year-old male patient who was operated on for a right inguinal hernia developed a postoperative wound infection 2 weeks after surgery and was started on IV antibiotics. Despite medical management, his infection did not improve, prompting a computed tomography (CT) scan which revealed a psoas abscess. The abscess was drained, and antibiotics continued. A few days later, he developed altered sensorium prompting a head CT which revealed septic emboli and hemorrhage at the gray-white junction. Cultures grew multidrug-resistant Escherichia coli; the patient was treated with IV tigecycline and improved over the following 4 weeks. Conclusion: In patients with a known ongoing infectious process with hemodynamic stability who develop altered mental status in the setting of a normal coagulation profile, D-dimer, positive blood cultures, and absent signs of multiorgan failure, a diagnosis of septic emboli should be entertained. Although CT can reveal macrobleeds, MRI is more sensitive in confirming cerebral microbleeds. Thus, patients in sepsis with unexplained altered sensorium should undergo an MRI of the brain to rule out septic emboli and microbleeds.
Diabetic ketoacidosis (DKA) is a triad of uncontrolled hyperglycemia, metabolic acidosis, and increased total body ketone concentration1. Diabetic ketoacidosis (DKA) is a well-known manifestation of Type 1 diabetes2; however, there has been an increase in the incidence of DKA as the first presentation in Type 2 diabetes. This subtype of diabetes is typically seen in non-Caucasian middle-aged obese males due to non-autoimmune disruption of glucose metabolism, which requires an initial intensive insulin regimen. After initial insulin treatment for several weeks to several months, a significant percentage of people can become insulin independent with an oral hypoglycemic agent(OHA) and /or just lifestyle modification, and in remission for many years3. This hybrid form of diabetes sharing the characteristics of T1DM and T2DM is called "Flatbush diabetes", also known as "ketosis-prone T2DM4 5. This article aims to emphasize the importance of early recognition of this heterogeneous syndrome through the two representative case reports of Ketosis-prone T2DM. Case presentation We describe two cases of African American men (elderly and young adult) with no past medical histories, who presented to the Emergency Department with a few days history of polyphagia, polydipsia, polyuria and fatigue. New onset of Type 2 diabetes and unprovoked DKA were diagnosed. Both of them received standard treatment with intravenous fluid and insulin drip, recovered uneventfully and discharged with insulin. Both of them followed up closely in our endocrinology clinic, their GAD antibodies were 0. 00(n<=0. 02nmol/L), Islet cell antibody<1: 4(n<1: 4) and C-peptide were 2.6ng/mL and 6ng/mL(1.1-4.4ng/mL) respectively. Their insulin was gradually withdrawn with the addition of an oral hypoglycemic agent. Both the patients are currently insulin independent for more than 15 months. Conclusion This is an important clinical entity to recognize ketosis-prone T2DM as insulin independence positively impacts quality of life and decreases economic burden on the health care system. For patients with suspected ketosis-prone T2DM, B-cell autoantibodies and C-peptide level should be obtained 1-3 weeks at the first outpatient visit, as it can predict patients’ prognosis. These patients should also be referred to an endocrinologist and need careful follow-up. Among all ketosis-prone T2DM patients, those with preserved B-cell function are more likely to be insulin independent and go into remission phase. Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m.
Background: Extramedullary hematopoiesis (EH) is common in patients with ineffective erythropoiesis like thalassemia major (TM). EH commonly involves intra-abdominal organs (e.g., spleen and liver), but rarely involves vertebral bodies. Here, we reviewed images of EH contributing to spinal canal stenosis. Case Description: A 19-year-old male with beta-thalassemia major (TM) presented with pain and bilateral lower extremity neurogenic claudication. Bilaterally, on examination, he had positive straight leg raising to 30°, a loss of the Achilles responses, and decreased pain appreciation in the L5S1 distributions. The lumbar MR showed anterior epidural lobulated mass lesions at L5 and S1, contributing to marked canal stenosis. Following an L5/S1 laminectomy for decompression, the biopsy revealed extramedullary hematopoietic tissue. Conclusion: Patients presenting with the lower extremity symptoms/signs, ranging from low back pain to neurogenic claudication and even paraplegia, may have EH secondary to TM. Treatment options include hypertransfusion, local radiation therapy, and/or surgical decompression.
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