INTRODUCTION:Hereditary Angioedema (HAE) is a rare, autosomal dominant disorder characterized by episodic, non-pruritic, non-pitting swelling of the skin, respiratory tract, and gastrointestinal system resulting from C1 esterase inhibitor (C1-INH) deficiency or dysfunction. It is frequently underdiagnosed, particularly in developing countries like India, due to its nonspecific presentation and overlap with allergic angioedema. CASE PRESENTATION:We report a case of a 28-year-old Indian woman who presented with a fivemonth history of progressive facial and periorbital swelling. She had no urticaria, known allergies, or systemic symptoms and did not respond to antihistamines or corticosteroids. The initial workup revealed normocytic anemia and elevated inflammatory markers, with normal renal, hepatic, thyroid, and autoimmune profiles. Radiological evaluation showed bilateral pre-septal edema. Complement C4 was normal; however, serum C1-INH level was markedly reduced, confirming type I HAE. She responded well to intravenous plasma-derived C1-INH concentrate (Cinryze). Unfortunately, she succumbed to a later episode of upper airway edema, highlighting the life-threatening potential of untreated or delayed HAE. CONCLUSION:This case emphasizes the need for high clinical suspicion of HAE in patients with unexplained, non-histaminergic angioedema, especially when conventional therapies fail. Early biochemical confirmation and targeted therapy are essential for effectively managing and preventing fatal complications.

BACKGROUND:Leptospirosis is a globally important zoonotic disease with substantial morbidity and mortality, yet it remains underreported in Northern India. This study aimed to evaluate the seroprevalence, clinical characteristics, and severity determinants of acute leptospirosis in a tertiary care setting. METHODS:We conducted a retrospective cross-sectional analysis of 174 hospitalized leptospirosis patients from 20,162 admissions between 2019 and 2023. The diagnosis was established using Leptospira IgM ELISA testing. Clinical, haematological, and imaging parameters were systematically evaluated. RESULTS:The incidence of leptospirosis was 0.9%, peaking at 38.5% post-monsoon. Rural residents (64.9%), males (59.2%), and individuals involved in agricultural work (62.6%) were predominantly affected. Common presentations were fever (100%), headache (70.7%), myalgia (62%), and calf pain (50%), followed by jaundice (39.7%), and reduced urine output (33.3%).. The mortality rate was 20.1%. Severity correlated significantly with nausea/vomiting, abdominal pain, cough, and complications, including diffuse alveolar haemorrhage (DAH), disseminated intravascular coagulation (DIC), multiorgan dysfunction syndrome (MODS), hepatic dysfunction, acute renal injury, and leukocytosis. DISCUSSION:The findings emphasize the critical need for early risk stratification and timely intervention to improve clinical outcomes. Identifying clinical and laboratory predictors of severity can significantly guide early management strategies in endemic areas. CONCLUSION:Several clinical and laboratory parameters are significant predictors of leptospirosis severity. Early recognition and risk stratification based on these indicators are crucial to improving patient outcomes. Enhanced surveillance, preventive measures, and clinician awareness are urgently needed to address this neglected tropical disease.

INTRODUCTION:Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multisystem involvement due to autoantibody production and immune complex deposition. While classical cutaneous manifestations, such as malar rash, are common, atypical presentations, like periorbital erythema and swelling, are rare and pose diagnostic challenges. Early recognition is crucial to prevent disease progression and complications. CASE PRESENTATION:A 16-year-old girl presented with a three-month history of intermittent bilateral periorbital swelling. Clinical examination revealed pallor and localized alopecia with no significant systemic abnormalities. Laboratory investigations showed pancytopenia with normal renal, hepatic, and thyroid functions and unremarkable urinalysis, chest X-ray, and ECG. Autoimmune markers were positive, with a strongly positive ANA titer of 1:1000 and significantly elevated anti- dsDNA antibodies of 380 IU/mL (reference range: 0-200 IU/mL). According to the 2019 EULAR/ ACR classification criteria, a diagnosis of SLE was established. The patient was treated with pulse intravenous methylprednisolone (1g daily for three days), followed by oral prednisolone (1 mg/kg/day), in a tapering regimen and hydroxychloroquine at standard doses. She showed marked improvement, with resolution of periorbital swelling, recovery of pancytopenia, and hair regrowth. At two-month follow-up, she remained asymptomatic and continued hydroxychloroquine for maintenance therapy. CONCLUSION:This case underscores the importance of considering SLE in patients with atypical presentations, like periorbital erythema and pancytopenia. Early diagnosis based on clinical and serological findings, followed by appropriate therapy, can achieve remission and prevent complications. The case highlights the need for heightened clinical suspicion and multidisciplinary management in young patients.

INTRODUCTION:Immune thrombocytopenia (ITP) is an autoimmune condition characterized by reduced platelet counts due to increased peripheral destruction and impaired platelet generation. An estimated incidence of ITP is 2 to 5 cases per 100,000 individuals in the general population. While mucocutaneous bleeding is common, life-threatening complications, such as spontaneous lingual hematoma and intracerebral hemorrhage (ICH), are extremely rare. Rapid progression of lingual haematomas might compromise airway function and necessitate immediate medical intervention. It is well established that most patients with ITP respond to first-line therapy; however, severe bleeding events, such as intracerebral hemorrhage, occur in less than 1% of cases and are associated with significant morbidity and mortality. CASE PRESENTATION:A 21-year-old male with a 6-month history of chronic ITP and poor compliance with therapy presented with a 2-day history of progressive reddish discoloration and swelling of the tongue. The clinical examination revealed stable vital signs and a remarkable general and systemic evaluation. The relevant blood routine showed a critically low platelet count at 8×103/μL, with normal coagulation parameters. No other bleeding manifestations were noted. Four hours after admission, the patient developed generalized tonic-clonic seizures and altered sensorium. Computed tomography (CT) of the head revealed an ICH. He was managed with single- donor platelet transfusions, intravenous anti-epileptics, pulse corticosteroid therapy, eltrombopag, and supportive care. The patient demonstrated a favorable clinical response, characterized by a rising platelet count and resolution of symptoms. He was discharged in stable condition with counseling on therapy adherence. CONCLUSION:The present case emphasizes the rarely yet life-threatening complication of inadequately managed ITP, such as spontaneous lingual hematoma and intracranial haemorrhage. It highlights the vital significance of therapy adherence and timely interdisciplinary intervention to avert disastrous consequences. Timely detection and intervention are crucial for positive outcomes in these intricate cases.

Heparin, a commonly used anticoagulant, has been associated with several cutaneous adverse effects, including skin necrosis, bruising/ecchymosis, urticaria, angioedema, red plaques, nodular lesions, and allergic contact dermatitis. Bullous hemorrhagic dermatosis (BHD) caused by heparin is a rare skin-related side effect. A 34-year-old woman diagnosed with anti-phospholipid syndrome was recently observed to develop hemorrhagic bullous dermatosis distant from the site of intravenous unfractionated heparin. Heparin therapy was continued for 5 days, followed by daily oral warfarin 2 mg, along with aspirin 75 mg, with monitoring of lesions. Two weeks later, skin lesions resolved spontaneously. BHD is nonthreatening and typically resolves on its own without the need for extensive treatment. Clinicians should be aware of the presentations of this self-limiting illness to avoid unnecessary workups.

INTRODUCTION:POEMS syndrome is a rare multisystem disorder associated with plasma cell dyscrasia and abnormal cytokine production, including vascular endothelial growth factor (VEGF). The mandatory criterion for its diagnosis includes polyneuropathy and monoclonal plasma cell disorder, along with other major and minor criteria. This case highlights the diagnostic and therapeutic challenges of POEMS syndrome by depicting the case of a 61-year-old male with progressive sensory-motor polyneuropathy, lymphadenopathy, and splenomegaly. CASE PRESENTATION:The patient presented with a year-long history of bilateral limb weakness and sensory disturbances, accompanied by abdominal distention, weight loss, and other systemic symptoms. Clinical examination revealed skin hyperpigmentation, splenomegaly, and a right axillary lymph node enlargement. Neurological evaluation showed distal limb hypotonia, absent reflexes, and sensory deficits. Diagnostic investigations, including nerve conduction studies, imaging, and bone marrow biopsy, confirmed POEMS syndrome based on polyneuropathy, monoclonal IgG lambda plasma cells, Castleman disease, sclerotic bone lesions, elevated VEGF, and minor criteria, such as endocrinopathy and skin changes. The treatment comprised lenalidomide and dexamethasone, resulting in significant improvement at the three-month follow-up, including normalized VEGF levels and resolution of ascites. CONCLUSION:This case highlights the necessity of identifying the many presentations of POEMS syndrome for prompt diagnosis and treatment. Despite its rarity and diagnostic complexity, prompt treatment can significantly improve clinical outcomes. POEMS syndrome should be considered in patients with unexplained neuropathy and systemic features, enabling better outcomes through targeted therapies.

Objectives Management of culture-negative sepsis in intensive care unit (ICU) admitted patients is difficult as these events are infectious and non-infectious in etiology. Thus, in the era of modern medicine, serological tests might help clinicians for better management of culture-negative sepsis events in ICU-admitted patients. Materials and Methods We prospectively enrolled 100 patients having culture-negative sepsis, admitted to the ICU during the COVID-19 pandemic. Baseline serum mannan and galactomannan (GM) levels by enzyme-linked immunosorbent assay-based method were determined in each patient. Concomitantly, C-reactive protein (CRP) and procalcitonin (PCT) values were also determined and the results were compared with other clinicoradiological evidence. Statistical analysis We determined the correlation between serum mannan and PCT, serum GM, and PCT by Spearman correlation coefficient. Results Baseline serum GM, mannan, PCT, and CRP were determined in 100 patients having culture-negative sepsis. A significantly higher mannan (125 pg/mL) was determined in 16 patients, whereas GM antigen (GM index [GMI] >0.5) was positive in 47 enrolled patients. 45 enrolled patients with higher GMI (>0.5) had respiratory symptoms. Out of 16 patients having higher mannan levels, only 3 had higher GMI. We also observed a significant negative correlation between serum mannan and PCT; a negative correlation between GMI and PCT; and a positive correlation between acute physiology and chronic health evaluation II score and PCT. Conclusions Composite tests alike serum mannan, GM, PCT, and CRP might be helpful in the management of culture-negative sepsis events in ICU-admitted patients.

Hereditary Hemorrhagic Telangiectasia (HHT), or Osler-Weber-Rendu syndrome, is a rare autosomal dominant vascular disorder characterized by mucocutaneous telangiectasias and visceral arteriovenous malformations (AVMs). We report the case of a 45-year-old male with a 20-year history of recurrent spontaneous epistaxis, chronic fatigue, and iron deficiency anemia, unresponsive to repeated nasal cauterizations. A strong family history of similar bleeding episodes in his mother and son heightened clinical suspicion. Examination revealed severe pallor without visible telangiectasias. Upper gastrointestinal endoscopy demonstrated telangiectatic lesions, while imaging excluded pulmonary and cerebral AVMs. Genetic testing confirmed HHT type 2 by detecting a pathogenic ACVRL1 gene variant. The patient was managed with intravenous iron, blood transfusions, topical nasal care, and laser therapy for refractory epistaxis. This case underscores the diagnostic challenges of HHT without classical telangiectasias and highlights the importance of early genetic testing and family screening to prevent serious complications.

INTRODUCTION:Epstein-Barr Virus (EBV) causes heterophile-positive Infectious Mononucleosis (IM), which manifests fever, sore throat, lymphadenopathy, and atypical lymphocytosis. In the Central Nervous System (CNS), EBV can cause acute encephalitis, cerebellar ataxia, Acute Disseminated Encephalomyelitis (ADEM), myelitis, meningitis, and radiculopathy. Reports of acute transverse myelitis linked to EBV infection are limited; therefore, Longitudinally Extensive Transverse Myelitis (LETM) due to EBV infection is extremely uncommon. CASE REPORT:An 18-year-old male, otherwise healthy, was admitted to the medicine department with ten days of fever, headache, and vomiting and five days of altered sensorium. Subsequently, his neurological test showed bilateral upper motor neuron quadriparesis, sensory impairment, and bladder-bowel involvement. Spinal T2W MRI indicated extensive cervical, thoracic, and lumbar hyperintense lesions. Laboratory investigations supported the diagnosis, which revealed a positive IgM Antibody for EBV Viral Capsid Antigen (VCA) in serum and EBV DNA PCR in Cerebrospinal Fluid (CSF). The final diagnosis was EBVinduced acute meningoencephalitis with longitudinally extensive transverse myelitis and incidental aortic coarctation. Following methylprednisolone pulse therapy, the patient recovered significantly. CONCLUSION:The present case report aims to share our experience by highlighting awareness of the rarity and treatment outcome of EBV-induced LETM.

INTRODUCTION:Tuberculosis (TB) is a widespread infectious disease caused by Mycobacterium tuberculosis. It predominantly affects the lungs but can involve any organ in the body. Tracheo-oesophageal fistula (TEF) is one of the rare extrapulmonary manifestations of TB. CASE REPORT:A 27-year-old male, otherwise healthy, reported to our outpatient department with complaints of fever, persistent cough, and significant weight loss. Subsequently, he was diagnosed with tuberculous tracheo-oesophageal fistula and pulmonary tuberculosis. DISCUSSION:The patient had an elevated ESR (52 mm) and underwent multiple imaging studies, including two normal barium swallow tests. Upper gastrointestinal endoscopy (UGIE) revealed two esophageal ulcers, one with a fistulous tract. Biopsy results suggested chronic esophagitis with granulomatous inflammation. Contrast-enhanced CT (CECT) of the thorax showed esophageal irregularities, air foci, and contrast extravasation into the bronchi, along with mediastinal lymphadenopathy and centrilobular nodules. Clinical and investigative findings suggested pulmonary tuberculosis with a tracheoesophageal fistula. The patient was discharged on a six-month antitubercular regimen with nutritional support via a nasogastric tube. Stent installation was planned if follow-up results were unfavorable. CONCLUSION:Although tuberculosis is highly prevalent in India, TEF of tuberculous origin has been infrequently documented, particularly in young, healthy, immunocompetent individuals. The patient was successfully cured after initiating antitubercular therapy and subsequent follow-up.

Background: Central nervous system (CNS) infections remain a major public health challenge globally, particularly in developing nations. These infections, including meningitis, encephalitis, and brain abscess, are associated with high morbidity and mortality. Their clinical and microbiological patterns vary according to geography, patient demographics, and immune status. Objectives: The present study aimed to determine the clinical spectrum, laboratory characteristics, microbial etiology, and antimicrobial resistance patterns among patients diagnosed with CNS infections. Materials and Methods: A hospital-based cross-sectional study was conducted on 160 patients with clinically and laboratory-confirmed CNS infections at Sir Sunder Lal Hospital, Varanasi, between June 2017 and May 2019. Cerebrospinal fluid (CSF) samples were analyzed for cytological, biochemical, and microbiological parameters. Statistical analysis was performed using SPSS version 21.0, with p < 0.05 considered significant. Results: Of 160 patients, 58% were males and 42% females, with the majority aged 21–40 years. Tubercular meningitis was the most common infection (53.1%), followed by viral (26.9%) and bacterial meningitis (8.8%). Fever (97.5%), headache (95.6%), and altered sensorium (75%) were the predominant symptoms. CSF findings revealed elevated protein and ADA levels in tubercular meningitis, neutrophilic predominance in bacterial, and lymphocytic predominance in viral infections. Streptococcus species (41.7%) and E. coli (33.3%) were the most frequent bacterial isolates. Conclusion: Tubercular meningitis remains the leading CNS infection in this region. CSF analysis remains pivotal for diagnosis, and early microbiological identification can guide prompt, targeted therapy, reducing complications and mortality.

Abstract Chronic myeloid leukemia is a bone marrow cancer that spreads slowly. Platelets (thrombocytes), which aid in blood clotting, white blood cells (leukocytes), which protect the body from infection, and red blood cells (erythrocytes), which supply oxygen, are all produced by normal bone marrow. In chronic myeloid leukemia, the bone marrow produces an abnormally large amount of white blood cells. At first, these cells appear to function correctly. However, as the disease progresses, the blood and bone marrow become overpopulated with immature white blood cells known as myeloblasts, also known as blasts. Overgrowth of myeloblasts hinders the generation of other blood cells, resulting in anemia (low red blood cell count) and platelet shortage. In twelve CML patients who either lost their response or did not obtain it within the allotted timeframes, we examined the pattern of kinase domain mutations. The most frequent reason to request a mutation investigation was loss of molecular responsiveness. It was discovered that four patients had identifiable mutations. We identified four mutations: E255V, L387M, T315I, and F359S in our cohort.

Background:: Dengue is a major health burden worldwide, notably in tropical and subtropical countries, with symptoms ranging from asymptomatic infection to severe hemorrhagic or shock syndrome. The clinical and biochemical profile of dengue has been shown in the literature to be expanding as more atypical signs have been added over the past few decades. Most dengue-related symptoms are typically mild to moderate and self-limited. However, intracranial hemorrhage and acute respiratory distress syndrome in severe dengue is an event that has been encountered uncommonly. Case presentation:: A 30-year-old female presented with complaints of fever, headache, cough, and altered sensorium. Later, she was diagnosed with severe dengue fever complicated by dengue hemorrhagic fever, which manifested as subarachnoid and subdural bleeding and acute respiratory distress syndrome. She was managed conservatively with a positive outcome. Conclusion:: Atypical manifestation, though rare, should be kept in mind while dealing with severe dengue patients, as early detection based on anticipated risk factors and timely treatment have the potential to save lives.

Rickettsial diseases cover a broad spectrum of illnesses. Scrub typhus is present worldwide, and Orientia tsutsugamushi is the causative agent of this chigger mite-borne infectious illness. Infections exhibit a range of severity, from mild illness to the more severe manifestation of multiorgan failure. We report three cases of scrub fever (55-year-old female, 63-year-old female, and 29-year-old male), all cases developed focal or pan-digital gangrene. All cases were successfully treated by administering doxycycline and additional supportive measures. Digital gangrene is uncommon in scrub typhus. Clinical suspension for early diagnosis and appropriate treatment may avoid further complications.

Dengue continues to pose a global health challenge, particularly in tropical and subtropical areas. Dengue can impact various organs, including the central nervous system, and can cause various neurological symptoms, although stroke is an uncommon complication. Among strokes, ischemic stroke is very uncommon in dengue. A 27-year-old unmarried female was referred to our institution with 8 days of fever and myalgia, 5 days of vomiting, and 3 days of altered sensorium. Small-sized bilateral pupils reacted slowly to light. Chest auscultation revealed diffuse coarse crepitation. Poor general condition and labored breathing led to intensive care unit transfer and intubation. Her contrast-enhanced brain magnetic resonance image showed chronic pontine infarction. She was diagnosed with dengue fever (NS1Ag positive) complicated by bilateral pontine infarction. After a long course of illness, she was finally discharged to home in good recovery status. Clinicians need to be aware of the uncommon dengue presentation of stroke. Treatment is supportive with variable outcomes.

INTRODUCTION:Dengue fever is prevalent in tropical nations, especially India. Leucopenia and thrombocytopenia are distinctive features of acute dengue fever that revert to normal levels after the patient's recovery. Dengue fever is associated with numerous unusual clinical manifestations of different body systems. Additionally, the emergence of severe thrombocytosis following thrombocytopenia is extremely rare. Based on our extensive knowledge, only three cases similar to this have been documented in the literature. CASE REPORT:Here, we present a case of a 36-year-old healthy man who had acute dengue and subsequently developed severe reactive thrombocytosis. The patient was treated conservatively and discharged. Subsequently, he developed thrombocytosis. Aspirin was given for a short period to alleviate any potential repercussions. CONCLUSION:Thrombocytosis, a rare consequence of dengue infection, is usually asymptomatic. Nevertheless, ongoing monitoring of dengue patients is required to avoid complications.

BACKGROUND:Although the microplate system is commonly used for the treatment of maxillofacial fractures, its use in the fixation of mandibular fractures is not widely accepted. OBJECTIVES:The study aimed to evaluate and compare the efficacy of microplates and miniplates in osteosynthesis for the internal fixation of undisplaced and minimally displaced anterior mandibular fractures. MATERIAL AND METHODS:A total of 40 patients diagnosed with undisplaced or minimally displaced symphyseal and parasymphyseal fractures were randomly assigned to 2 study groups (group A and group B). Patients in group A (microplate group) were treated with two 0.8-mm microplates, whereas patients in group B (miniplate group) received two 2.0-mm miniplates. Bite force values were recorded in 30 healthy individuals (control group) to establish baseline values. Postoperative bite force values were recorded at various intervals and compared between the study groups and the control group. RESULTS:Both groups demonstrated a progressive improvement in the bite force. However, the bite force values recorded at the 2nd, 4th and 6th postoperative weeks were comparatively lower in the microplate group. At the six-week follow-up, the bite force values were lower in both study groups in comparison to the control group. There were no differences in the incidence of postoperative complications between the study groups. CONCLUSIONS:The use of microplates in the management of undisplaced or minimally displaced anterior mandibular fractures results in a reduction in the recovery of biting force in comparison to the conventional miniplate system.

To compare ankle brachial pressure index (ABPI) in normal weight and obese/overweight type 2 diabetes mellitus patients (T2DM) to see the impact of obesity on the occurrence of peripheral artery disease (PAD) in T2DM patients. Secondly to investigate the relationship between ABPI, insulin resistance, and beta cell function and between adipocytokines and obesity parameters. A total of 120 BMI-categorized Normal weight (NW) T2DM (n = 53) patients and obese/overweight T2DM (n = 67) patients were recruited in this study. ABPI measurements were performed for the assessment of PAD. The anthropometry and body composition of the patients were measured. Plasma fasting insulin, adiponectin, and IL-6 levels were measured by ELISA kits. ABPI scores were found to be comparable between both groups of patients (p = 0.787). A significant positive correlation was observed between ABPI and beta cell function. Insulin resistance was found to correlate positively while adiponectin negatively with obesity parameters. The ABPI score was comparable between both groups of patients, suggesting that vascular complications may occur at the same rate in NW as well as in obese/overweight diabetic patients. The positive association of insulin resistance as well as the negative association of adiponectin with obesity parameters, are suggestive of the importance of body fat distribution in predicting insulin resistance and the inflammatory status of the cells.

Dengue virus is an arbovirus transmitted through the bite of infected Aedes mosquitoes. Many unusual clinical features are being reported in dengue. Dengue complicated with hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal condition. Here, we report an 18-year-old otherwise healthy female with dengue fever complicated with HLH. The diagnosis was made by fulfilling the clinical and laboratory criteria of HLH. She was managed successfully with a methylprednisolone pulse regimen. Our case highlights the importance of early recognition of complications and prompt treatment for a better outcome.

BACKGROUND:Tuberculosis is one of the major infectious diseases of mankind and remains a significant health concern, especially in developing countries. Clinical manifestations of TB are broad and sometimes very challenging for clinicians to diagnose early. Tuberculous psoas abscess was generally secondary to spinal tuberculosis or direct extension from adjacent structures in immunocompromised individuals, but tuberculous psoas abscess in the immunocompetent state is very infrequent. In addition, pancytopenia and new onset neck swelling simultaneously make this presentation a very unusual clinical entity in tuberculosis.CASE PRESENTATION:We now present a case of a 21-years-old, unmarried, otherwise healthy girl presented with fever, lower abdominal pain and weight loss for two months. She also noticed painless neck swelling for 15 days. She later had a tuberculous left sided psoas abscess with pancytopenia and a cold abscess on the left side of the neck with no sign of any other apparent focus, according to the evidence. Diagnosis of disseminated TB without lung involvement was established and ATT was started. The outcome was successful on follow up.CONCLUSION:Among the broad spectrum of atypical manifestations of TB, this case report draws attention to its rarity, diagnostic challenge and awareness of the clinical spectrum, especially in developing countries.