Acute Encephalopathy in Children From Muzaffarpur, Bihar, India, and the Potential Role of Ambient Heat Stress-Induced Mitochondrial Dysfunction.

BACKGROUND: Periodic outbreaks of acute encephalopathy in children have been reported from Muzaffarpur, Bihar, India. No infectious cause has been identified for this. This study presents the clinical and metabolic profile of children hospitalized with acute encephalopathy and the potential role of ambient heat stress. METHODS: This cross-sectional study included children (<15 years) with acute encephalopathy admitted from April 4, 2019, to July 4, 2019. The clinical and laboratory investigations included infections, metabolic abnormalities, and muscle tissue analysis. The children who had metabolic derangements but no infectious cause were labeled as acute metabolic encephalopathy. The descriptive analysis summarized the clinical, laboratory, and histopathology findings, and their association with the ambient heat parameters was explored. RESULTS: Out of the 450 children hospitalized (median age, four years), 94 (20.9%) died. Children had early morning onset (89%), seizures (99%), fever (82%), hypoglycemia at admission (64%), raised aminotransferases (60%), and high blood urea (66%). Blood lactate (50%), lactate dehydrogenase (84%), pyruvate (100%), ammonia (32%), and creatinine phosphokinase (69%) were raised. Viral marker tests were negative. The patients had abnormal metabolic markers like decreased blood-free carnitine, elevated blood acylcarnitines, and elevated urinary lactate, oxalate, maleate, adipate, and fatty acid metabolites. Blood carnitine and acylcarnitine levels normalized in 75% of the patients treated with carnitine and coenzyme-Q. Muscle tissues showed megamitochondria on electron microscopy and reduced respiratory enzyme complex-I activity. A significant correlation between the number of admissions and ambient heat indices was observed. CONCLUSIONS: The findings suggest secondary mitochondrial dysfunction as a possible mechanism for acute encephalopathy in children from Muzaffarpur, Bihar, and ambient heat stress as a possible risk factor.

Rituximab for acute plasma-refractory thrombotic thrombocytopenic purpura: A case report.

Introduction and importance: Thrombotic thrombocytopenic purpura (TTP) is a rare disease due to deficiency of ADAMTS13 which can present with anemia and thrombocytopenia. The study highlights the role of PLASMIC score in diagnosis and rituximab in the treatment of this condition. Case presentation: Herein, we report a case of 38 years old female who had presented with fever, irritability, and altered sensorium. On investigations, she had hemolytic anemia, and thrombocytopenia with peripheral blood smear showing occasional schistocytes and managed with steroids and plasma exchange. As her platelet, LDH, and a few other lab parameters failed to normalize and met the criteria of refractory TTP, hence she was started on 5 cycles of rituximab and her condition improved. Clinical discussion: Thrombotic thrombocytopenic purpura can be presumed based upon PLASMIC score where if the score is 5 or more while ADAMTS13 assay is required for confirmation. It is a life-threatening condition where treatment options include therapeutic plasma exchange (PEX), glucocorticoids, Rituximab, and caplacizumab. Rituximab is considered particularly in refractory cases. Conclusion: Thrombotic thrombocytopenic purpura can lead to complications due to low platelet counts. Hence, early diagnosis and intervention are crucial to prevent such complications.

Ruptured dermoid cyst of ovary developing into chronic peritonitis; a rare complication diagnosed by contrast CT: A case study.

Introduction: Dermoid cyst also called Mature cystic teratoma is the most common ovarian germ cell tumor of pre-menopausal females, composed of skin, hair, teeth, and sebum covered by thick fibrous tissue. It can present with complications like torsion, rupture, infection, and autoimmune hemolytic anemia. The case highlights the role of imaging in the diagnosis of ruptured dermoid cyst which can have subtle clinical features. Case Presentation: Herein we present a case of 53 years multiparous postmenopausal female who presented with lower abdominal pain. Examination findings at presentation were normal. 2 years back patient was evaluated for the abdominopelvic mass which was diagnosed radiologically as an ovarian dermoid cyst. This time, Ultrasonography (USG) of the abdomen and pelvis followed by Contrast-enhanced computed tomography (CECT) of the abdomen and pelvis revealed the features consistent with a ruptured dermoid cyst. Exploratory laparotomy and histopathological examination of the specimen confirmed the diagnosis. Clinical Discussion: Rupture of a dermoid cyst is a very infrequent complication. Following rupture patient may present with peritonitis which may be acute or chronic. Chronic peritonitis may not show any clinically distinguishable features such that the clinical diagnosis of the rupture dermoid cyst is difficult to make. The radiological assessment helps to make an accurate diagnosis so that appropriate surgical intervention can be instituted. Conclusion: Following the rupture of the dermoid patients may progress to a stage of chronic peritoneal inflammation. At this stage, the radiological assessment may be crucial for appropriate diagnosis and thus further management.

Tuberous sclerosis in a 16 years old female: A case report.

INTRODUCTION: Tuberous sclerosis complex (TSC) is an uncommon multisystem disorder that can affect the lungs, skin, kidneys, and brain. The study highlights the importance of genetic and clinical diagnostic criteria in identifying this rare condition and the role of surveillance in preventing complications. CASE PRESENTATION: Herein, we report a case of 16 years old female presenting with right flank pain, hematuria, hypopigmented macule over the back, ash leaf spots over the right upper and lower limb, and a palpable mass over the right lumbar region. Laboratory tests showed low hemoglobin with plenty of red blood cells in urine. She was admitted for symptomatic management of pain and blood transfusion was done to manage anemia. After a diagnostic workup for tuberous sclerosis, she was diagnosed with the condition and is under regular follow-up. CLINICAL DISCUSSION: Tuberous sclerosis complex is one of the neurocutaneous syndromes, diagnosed based on the genetic or clinical diagnostic criteria as per the second International Tuberous Sclerosis Complex Consensus Conference 2012, which have been updated in 2021 with no changes in genetic diagnostic criteria and slight changes in clinical diagnostic criteria. After diagnosis, along with the management, surveillance is also crucial. CONCLUSION: Tuberous sclerosis runs a progressive course and can lead to various complications. Thus, early diagnosis with the help of genetic and clinical diagnostic criteria is important along with regular surveillance of different body systems to prevent debilitating complications.

Is Ambient Weather a Risk Factor for Acute Encephalopathy Outbreaks in Children in Muzaffarpur, Bihar, India? Insight from a 9-Year Analysis.

OBJECTIVES: Despite periodic outbreaks, the causes and risk factors of acute encephalitis syndrome (AES) in children of Muzaffarpur, Bihar, India, remain unknown. We explored the correlation between AES caseload and the climate parameters. METHODS: Data for 1318 hospitalized children with AES during 2012-20 were used. The correlation between AES cases and daily climate parameters (temperature, sunshine, rainfall, humidity and wind speed) for the previous 24, 48 and 72 h were examined using Pearson's and Spearman's rank-order correlation and Poisson regression or negative binomial regression analyses. RESULTS: Most (91.8%) of the AES cases occurred during the summer season (May-July months), especially June month. Pearson's and Spearman's rank-order correlation analyses revealed that AES caseload had positive correlations with maximum (r = 0.275, ρ = 0.293) and minimum (r = 0.306, ρ = 0.306) temperatures during past 24 h and heat index (r = 0.325, ρ = 0.325) and negative correlation with humidity (r = -0.222, ρ = -0.222) and rainfall (r = -0.183, ρ = -0.183) (all p < 0.05). The correlation was consistent for the climate parameters for the past 24, 48 and 72 h. Regression analysis also documented a significant association of AES cases with daily maximum (ß: 0.32-0.36) and minimum (ß: 0.53-0.62) temperatures and heat index (ß: 0.92-1.03) over past 24, 48 and 72 h (all p < 0.01). The number of AES cases exponentially increased when the daily maximum and minimum temperatures crossed 40°C and 31°C, respectively. CONCLUSIONS: The climate parameters, especially temperature appears to be a risk factor for AES in children. The definite aetiological role of heat for AES in children needs further exploration.

Repeated seasonal outbreaks of acute encephalitis syndrome (AES) in children have been observed in Muzaffarpur, Bihar (India), since 1995. These children mostly present during the summer months (May­June) of the year with sudden early morning onset of seizures, altered sensorium, hypoglycaemia, with or without fever. A high fatality (27­63%) or sequels among those who survive have been observed. Large outbreaks were observed in 2005, 2009, 2011, 2012, 2014 and 2019. The despite periodic outbreaks, the causes and risk factors of AES in these children remain mostly unknown. We explored the correlation between the AES caseload (data for 1318 hospitalized children with AES during 2012­2020) and the daily climate parameters (temperature, heat index, sunshine, rainfall, humidity and wind speed) for the previous 24, 48 and 72 h using various statistical tests. Most (91.8%) of the AES cases occurred during the summer season (May­July months), especially June month. The AES caseload had significant positive correlation with maximum and minimum temperatures and heat index and negative correlation with humidity and rainfall. The number of AES cases exponentially increased when the daily maximum and minimum temperatures crossed 40°C and 31°C, respectively.

Pericardial effusion in hypothyroidism: A case report.

INTRODUCTION: and importance: Hypothyroidism is an endocrine disorder with multiorgan involvement and various complications. One of the significant but less often seen complications is pericardial effusion. Since it can progress to life-threatening conditions like cardiac tamponade and hemodynamic instability, early diagnosis, and management of the pericardial effusion in hypothyroidism is a must. CASE PRESENTATION: We present a case of a 35-year-old male who presented with bilateral lower limb swelling, facial puffiness, cold intolerance, fatigue, and hoarseness of voice for one week. Laboratory investigation showed high thyroid-stimulating hormone (TSH), low triiodothyronine (T3), and raised serum anti-thyroid peroxidase (anti-TPO). The lipid profile demonstrated hypertriglyceridemia. Ultrasonography of the neck revealed normal thyroid size with decreased echo texture and increased vascularity. An electrocardiogram showed low voltage complexes with sinus bradycardia. 2D echocardiography revealed minimal pericardial effusion with normal ventricular function. The patient was managed with thyroxine therapy which gradually resolved his symptoms and pericardial effusion. CLINICAL DISCUSSION: Pericardial effusion in hypothyroidism is due to the increased capillary permeability and albumin distribution volume and reduced lymph drainage in the pericardial cavity. Its presence in mild cases of hypothyroidism is uncommon although it can be seen in severe, long-standing hypothyroidism. Pericardial effusion in hypothyroidism, though rare, can present in mild cases and if overlooked can be fatal due to conditions like cardiac tamponade. CONCLUSION: With early cardiac assessment and adequate thyroid replacement therapy, pericardial effusion in hypothyroidism can be reversible at an early stage. So, pericardial effusion which can be overlooked in mild cases of hypothyroidism needs to be identified and managed early.