Acute Hepatitis E Virus Infection Triggering Autoimmune Hepatitis in a Patient With Chronic Liver Disease: Case Report and the Review of the Literature.

Acute viral hepatitis E (HEV) is the most common form of acute viral hepatitis in India. It is associated with self-limiting disease in most cases. However, the chronic form of HEV is also being increasingly recognized. Other viral infections like the hepatitis A virus (HAV) have been implicated in inciting autoimmune hepatitis. HEV infection has been associated with the formation of circulating liver-directed autoantibodies, however autoimmune liver disease following acute HEV infection has been rarely reported. Here we present a case of a 72-year-old diabetic lady who presented to us with an asymptomatic rise of liver enzymes. Investigations suggested metabolic dysfunction associated with steatotic liver disease. After three months of the diagnosis, she developed acute-on-chronic liver failure and her anti-HEV came out positive. She was managed accordingly. Afterwards patient had persistent high liver enzymes, so she underwent a liver biopsy. Her liver biopsy was compatible with autoimmune hepatitis.

Implications of Gender on the Outcome in Patients With Autoimmune Hepatitis.

BACKGROUND: Autoimmune hepatitis (AIH) is uncommon and predominantly affects females. Data on AIH from India are scanty. We retrospectively analyzed the spectrum and outcome of adults with AIH and compared it between male and female patients. METHODS: AIH was diagnosed using a simplified AIH score. For suspected seronegative AIH, the revised score was used. Standard therapies for AIH and portal hypertension were administered and response was assessed at six months. Relapse rates and five-year mortality were also evaluated. RESULTS: Of the 157 patients with AIH, 85 (male: female 25: 60) were included in the study. The median age at diagnosis was 46 (interquartile range (IQR) 32-55.5) years in males vs 45 (IQR 34.2-54) years in females (p=0.91). A similar proportion of male and female patients presented with cirrhosis, acute severe AIH, or AIH-related acute on chronic liver failure (ACLF); Extra-hepatic autoimmune diseases were less common in male patients (16% vs 35.5% p=0.02). Other laboratory and histological features were comparable in both groups. During the median follow-up period of 51 months (IQR 45-67 months). The biochemical and clinical response at six months were seen in 64% of male patients and 63.3% of female patients (p= 0.57). Of patients, 75% relapsed in the male AIH group (12 of 16 patients) after initial remission compared to 42% in the female group (p=0.02). Five-year mortality was 14.1%, and no patient developed hepatocellular carcinoma. CONCLUSION: Male and female patients with AIH have similar clinical, biochemical, and histological profiles. More male patients relapsed after an initial response to therapy.

Tofacitinib in Steroid-Refractory Acute Severe Ulcerative Colitis: A Retrospective Analysis.

INTRODUCTION: Steroid-refractory acute severe ulcerative colitis (ASUC) patients are at the highest risk of colectomy. Among the available options, cyclosporine and infliximab have similar efficacy but infliximab is a costly drug and cyclosporine has multiple side effects like kidney injury, neurotoxicity, and dyselectrolytemia. Surgical management is often associated with higher morbidity. Newer oral small molecules like Janus kinase inhibitors are the ideal molecules to bridge the gap. Tofacitinib has already been extensively evaluated in patients with moderate to severe UC; however, data on ASUC treated by tofacitinib are limited. METHODS: We retrospectively analyzed the data of patients with ASUC who were admitted to our hospital's luminal gastroenterology unit between January 2021 and July 2023. Patients with ASUC who were managed with tofacitinib were included in the study. RESULTS: Eight patients with ASUC were identified who did not respond to intravenous hydrocortisone and were treated with tofacitinib. The mean age was 39 ± 15 years and 87.5% were female. The median duration of illness was 24 months (interquartile range (IQR): 12-120 months). Seven of eight patients (87.5%) responded to oral tofacitinib 10 mg twice a day by the fifth day of treatment. The median follow-up period was six months (IQR: 1-12 months). One patient required colectomy and one patient had varicella zoster reactivation requiring treatment discontinuation. CONCLUSION: Tofacitinib is an attractive alternative to the currently available salvage therapy for steroid-refractory ASUC; however, long-term efficacy and risk remain to be explored.

Budd Chiari Syndrome with Portal Vein Thrombosis Managed Successfully with Direct Intrahepatic Portosystemic Shunt: A Case Report and Review of the Literature.

Budd -Chiari syndrome (BCS) is a hepatic vascular disorder which affects hepatic veins or inferior vena cava. Portal vein thrombosis (PVT) occurs in around 15%-25% of patients with BCS. The presence of PVT in patients with BCS makes it more difficult to intervene radiologically. We present a case of a BCS-related chronic liver disease that presented with a history of variceal upper gastrointestinal bleeding and worsening ascites. The patient had thrombosed hepatic veins (HV) and partial right portal vein thrombosis. He was started on anticoagulation, and treatment for portal hypertension was initiated. Given the inaccessibility of all the HVs for trans-jugular intrahepatic portosystemic shunts (TIPS), the patient underwent direct intrahepatic portosystemic shunts (DIPS). Next-generation sequencing identified the factor V Leiden mutation. Following DIPS, the patient's ascites disappeared, and liver function tests improved. On a nine-month follow-up, the patient was symptom-free with a patent DIPS. DIPS has been widely used in patients with BCS with thrombosed hepatic veins, but there are only a few case reports on the feasibility of DIPS in BCS patients with PVT. This is one of the very few case reports where a patient with BCS-PVT was successfully managed with DIPS.

Guillain Barre syndrome with pulmonary tuberculosis: A case series from a tertiary care hospital.

INTRODUCTION: Guillain-Barre syndrome (GBS) is post-infectious autoimmune polyradiculopathy which characteristically presents with a monophasic illness with CSF albumino-cytological dissociation with partial or complete recovery. The incidence of GBS is about 1 to 2/100,000 per year.[1] Subtypes are described based on electrophysiological patterns, the most common being acute inflammatory demyelinating polyneuropathy (AIDP) and rarer ones being acute motor axonal neuropathy (AMAN), and acute motor and sensory axonal neuropathy (AMSAN). Tuberculosis is prevalent in India with various neurological manifestation including tuberculoma, brain abcess, pott's spine, and radiculomyelopathy.[2] Five cases have been published of tuberculosis and GBS.[34567] The main underlying pathophysiological mechanism is aberrant immune activation due to molecular mimicry against ganglioside in myelin. Although tuberculosis is mainly T-cell-mediated chronic disease, still there are cases reported with tuberculosis with GBS. Here we are going to present four cases of pulmonary tuberculosis presented with GBS. MATERIALS AND METHODS: This study describes clinical profile of four patients who presented with concomitant pulmonary tuberculosis and GBS over a period of 4 years in a tertiary hospital. Diagnosis was made according to Brighton criteria and alternative diagnosis were ruled out by clinical examination, serological markers, and MRI imaging of the spine. All patient underwent thorough investigation including HIV 1, 2, anti-CMV, anti-EBV to rule out other possible triggers of GBS, NCV, CSF study along with sputum AFB culture. ZN staining and CECT thorax were also done to support the diagnosis. RESULTS: Of total four cases, 3 were male and 1 was female who presented with weight loss, anorexia, cough with or without hemoptysis, and acute progressive LMN quadriparesis in which there was typical albumin-cytological dissociation in CSF. Nerve conduction studies were suggestive of AIDP in two patients, AMAN in one patient, and AMSAN in the fourth one. An exhaustive investigation for triggers of GBS were performed for all patients who were treated with IVIG and two of them completely recovered and rest of two did not recover completely after 6 weeks of follow-up. CONCLUSION: In pulmonary tuberculosis, patients with polyneuropathy demands urgent search for GBS as there has been case reports in literature though the association between tuberculosis and GBS is not clear.

Intravascular Hemolysis in Aluminum Phosphide Poisoning.

Hemolysis associated with aluminum phosphide poisoning is very rare. Intravascular hemolysis in presence of glucose-6-phosphate dehydrogenase (G6PD) deficiency has been reported rarely in literature. We are reporting a case of young male patient with history of aluminum phosphide poisoning and complicated with intravascular hemolysis without G6PD deficiency. It has been reported only once in the literature. HOW TO CITE THIS ARTICLE: Malakar S, Dass B et al. Intravascular Hemolysis in Aluminum Phosphide Poisoning. Indian J of Crit Care Med 2019;23(2):106-107.

Nephrocalcinosis in a patient with extrapulmonary tuberculosis - A rare entity.

Nephrocalcinosis is a rare condition in clinical practice where there is an increased renal deposition of calcium. Varied causes of this condition have been given in literature, and tuberculosis (TB) has been an important one. Hypercalcemia is a known complication of granulomatous diseases. We report a rare case explicitly showing relationship of extrapulmonary (genitourinary) TB with nephrocalcinosis.

Bilateral Parotid Enlargement and Trismus: An Unexpected Clinical Finding in a Patient with Common Krait (Bungarus caeruleus) bite.

We report a case of bilateral parotid enlargement and trismus due to the bite of common krait (Bungarus caeruleus). These clinical findings have not been reported in literature after the bite with this species.