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Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal disease characterized by excessive immune response activation. Numerous conditions, including infectious etiologies, are implicated in its development. We report the case of a 16-year-old girl with HLH associated with polyserositis and Salmonella typhi infection. A 16-year-old girl presented with a high-grade fever and abdominal pain that had been ongoing for 20 days. She had been treated for malaria at a local hospital but was referred to our hospital due to the worsening of her condition. On examination, she was found to have an enlarged liver and spleen, pale skin, and hypotension, with bilateral basal crackles on chest examination. Her blood profile revealed pancytopenia, elevated C-reactive protein, and a deranged coagulation profile. Peripheral smears showed anisocytosis, microcytes, hypochromia in RBCs, and a few platelet clumps. A bone marrow biopsy revealed increased megakaryocytes and hemophagocytes. Ultrasound and computed tomography of the abdomen and pelvis showed hepatosplenomegaly, pericholecystic edema, mild ascites, and long-segment diffuse colonic wall thickening, suggesting pancolitis. Blood culture revealed S. typhi , which is rarely associated with HLH. The patient was started on the HLH-2004 protocol and showed improvement on the fourth day of initiating therapy, but due to a delayed diagnosis, the patient collapsed on the sixth day of admission. HLH is a rare but life-threatening disease with various underlying causes. The diagnosis of HLH is challenging, and early diagnosis and prompt treatment are crucial for a better prognosis. The association between HLH and S. typhi infection is rare, and this case highlights the importance of considering unusual etiologies in HLH. Clinicians should be vigilant about this association, especially in endemic regions, to ensure early diagnosis and prompt treatment., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Achakzai et al.)

Esophageal adenocarcinoma arises after a normal squamous mucosa undergoes metaplasia to the specialized columnar epithelium, which can then ultimately progress to dysplasia and subsequent malignancy. It typically presents at an advanced stage, and despite optimal management, the prognosis remains poor. Recently, the ToGA (Trastuzumab for Gastric Cancer) trial evaluated the role of trastuzumab (Herceptin®) in human epidermal growth factor receptor 2 (HER2) over-expressing gastric and gastroesophageal junction (GEJ) adenocarcinoma and showed improvement in overall survival when trastuzumab was added to the standard chemotherapy compared to standard chemotherapy without trastuzumab. However, data and experience are lacking in the literature regarding the use of trastuzumab outside of combination chemotherapy. The optimal length and safety of trastuzumab use as maintenance therapy in the long-term are also unclear in patients with gastric or GEJ adenocarcinomas after sustained remission. We report a case of metastatic GEJ adenocarcinoma, where the patient first showed an exceptional response to standard chemotherapy combined with trastuzumab and subsequently could sustain a long-lasting remission on maintenance therapy with trastuzumab alone. Trastuzumab was ultimately discontinued owing to the lack of clear knowledge regarding its length and safety of use as maintenance therapy in the long-term., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2020, Haider et al.)

Brugada syndrome (BrS) is a rare autosomal dominant mutation affecting sodium channels. Electrocardiography can show two Brugada patterns (BrP). Type 1 BrP usually causes sudden cardiac arrest (SCA). Type 2 BrP can appear during circumstances that result in delayed sodium channel opening, such as fever, pneumonia, or use of sodium channel blockers. Patients with type 2 BrP often have underlying type 1 BrP; this can be confirmed by an ajmaline challenge test. We describe the case of a patient who presented with SCA. He later had an interval type 2 BrP secondary to aspiration pneumonia, followed by type 1 BrP pattern confirmed by an ajmaline challenge test. The patient ultimately underwent implantable cardiac defibrillator placement to prevent future SCA., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2020, Sattar et al.)

Cardiac tamponade is a condition characterized by the accumulation of pericardial fluid, compromising the hemodynamics of the circulation. It has several known causes, including traumatic injury to the pericardium, idiopathic, neoplastic or purulent pericarditis, and, rarely, iatrogenic etiology. Inferior vena cava (IVC) filter removal can lead to multiple complications including but not limited to IVC perforation, air embolism, pneumothorax or filter migration. Here, we present a case of a middle-aged woman presenting with cardiac tamponade after IVC filter removal. She was successfully managed with pericardiocentesis followed by pericardial window placement. As this case and literature review illustrates, cardiac tamponade is a rare but potentially devastating complication of IVC filter manipulation., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2020, Roomi et al.)

The combination of bradycardia, renal failure, atrioventricular (AV)-nodal blocker medications, shock, and hyperkalemia (BRASH) is a new syndrome that is a consequence of a positive loop of bradycardia due to AV-nodal blockers and hyperkalemia secondary to renal insufficiency. We present a case of BRASH syndrome in which the patient on chronic AV-nodal blockers presented with bradycardia, hypotension, underlying kidney dysfunction, and hyperkalemia. The patient was medically managed and discharged upon clinical improvement. The purpose of this report is to highlight the rare cases of BRASH syndrome and improve its management., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2020, Sattar et al.)

Radiofrequency catheter ablation has been commonly used for the treatment of drug-refractory atrial fibrillation. The esophageal injury along with the development of atrial-esophageal fistula (AE fistula) is fairly rare but is a devastating complication of catheter ablation. Described in 2004 for the first time, it is the most lethal of all the complications of catheter ablation with a high mortality rate. The clinical presentation of an AE fistula is variable, however, early diagnosis and treatment can prevent a fatality. We have reported a case of an AE fistula post catheter ablation for drug-resistant atrial fibrillation, along with its treatment, diagnosis, and possible preventive measures., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2020, Khan et al.)

Gastroparesis is a complex dysmotility disorder characterized by chronic dyspepsia and delayed gastric emptying in the absence of mechanical obstruction. Postsurgical gastroparesis is the third most common cause and accounts for 13% of total cases. Studies have shown that catheter ablation procedures for atrial fibrillation can rarely result in gastroparesis, secondary to damage to the vagus nerve. Once the diagnosis is confirmed, treatment options include: dietary management, prokinetic drugs, and new invasive treatments. Botulinum toxin injection is an emerging pyloric intervention, increasingly used in the management of gastroparesis refractory to pharmacological therapy. It is given as an injection into the pyloric sphincter, with the help of an endoscope. Botulinum toxin acts by inhibiting smooth muscle contraction through a decreased response to acetylcholine. Here we report a case of postsurgical gastroparesis that responded well to botulinum toxin therapy., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2020, Sarvepalli et al.)

Alemtuzumab, a humanized monoclonal antibody that targets surface molecule CD52, causes rapid and complete depletion of circulating T- and B-lymphocytes through antibody-dependent cell-mediated and complement-mediated cytotoxicity. Alemtuzumab has demonstrated superior efficacy compared to subcutaneous interferon beta-1a (SC IFNB-1a) in patients with multiple sclerosis (MS). Alemtuzumab treatment causes a rare and distinct form of secondary immune thrombocytopenic purpura (ITP), characterized by delayed onset, responsiveness to conventional therapies, and prolonged remission following treatment. In phase two and three clinical trials, the incidence of ITP was higher with alemtuzumab treatment compared to the patients receiving SC IFNB-1a. Here we report a case of ITP occurring two years after the first treatment with alemtuzumab. The patient recovered completely after a timely diagnosis and adequate treatment. Rigorous patient education and careful complete blood count (CBC) monitoring by the physician are critical for early identification and treatment of this potentially fatal disorder., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2019, Sarvepalli et al.)

We report a case of rare and aggressive gallbladder neuroendocrine carcinoma (GB-NEC), diagnosed with the help of endoscopic ultrasound (EUS). A 65-year-old asymptomatic male, with a past medical history of hypertension, underwent abdominal ultrasound for the screening of an abdominal aortic aneurysm. He was found to have a mixed echogenicity area near the stomach, an incidental finding on abdominal ultrasound. The patient had an upper gastrointestinal (GI) endoscopy exam, which revealed an antral mass that was biopsied. The tissue specimen showed an epithelioid mesenchymal tumor of unclassified type and, eventually, the patient underwent partial gastrectomy. Surgical pathology reported a low-grade sub-serosal gastrointestinal stromal tumor (GIST) of the resected tissue specimen. He was later discharged and advised to follow up with abdominal computed tomography (CT) every year. Two years later, his abdominal CT revealed a new 3.7 cm x 2.0 cm mass in the posterior gallbladder fundus. Subsequently, the patient underwent laparoscopic cholecystectomy and the excisional biopsy reported a T3NXM1 neuroendocrine small cell carcinoma. Then, he received six cycles of systemic chemotherapy with carboplatin and etoposide, showing excellent response initially. However, a repeat CT abdomen/pelvis with contrast, on his eighth-month follow-up, demonstrated the interval development of an infiltrative mass in the pancreatic head. The gastroenterology team was then consulted, who performed sphincterotomy with temporary stent placement and celiac plexus neurolysis. Also, a transduodenal fine-needle aspiration (FNA) of the pancreatic mass was performed, which revealed metastatic small cell carcinoma. Based on these findings, the patient received an additional three cycles of carboplatin/etoposide chemotherapy, along with one cycle of immunotherapy. However, the patient had a poor response to chemotherapy, and he eventually chose hospice care., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2019, Hussain et al.)

Acquired hemophilia A is a disorder of rare entity, resulting in spontaneous bleeding in individuals with no history of bleeding disorders. It is believed to be caused by spontaneous inhibition of clotting factor VIII by autoantibodies, and is usually associated with other autoimmune conditions. The hallmark of this condition is mucocutaneous bleeding leading to ecchymosis, melena, hematoma or hematuria. Our discussion revolves around the case of an elderly male with no history of anticoagulant use presenting with hematuria. Imaging showed left kidney hemorrhage, his labs were significant for a prolonged partial thromboplastin time (PTT), and subsequent tests revealed low factor VIII levels and high factor VIII inhibitor levels, which led to the diagnosis of acquired hemophilia A in the patient. He was managed with medications resulting in normalization of factor VIII levels., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2019, Yousphi et al.)

Chronic mesenteric ischemia (CMI) is a condition defined by a state of attenuated blood circulation in the mesenteric vasculature affecting one or more abdominal viscera, and is more common in the female and elderly populations. Amongst the many causes, it occurs most frequently in connection with diffuse atherosclerosis. Its presentation is variable with symptoms such as weight loss, nausea, vomiting, diarrhea and/or constipation, with postprandial pain classically present in the majority of the cases; this, in addition to the chronic course of the disease, makes timely diagnosis a challenge. Physical examination may reveal signs of malnutrition and other findings usually linked with the underlying medical condition. It can have grave consequences if not managed promptly. In our case, an 81-year-old woman came in with loss of appetite, nausea, vomiting, diarrhea and/or constipation, and weight loss. Computed tomography angiography (CTA) of the abdomen/pelvis confirmed chronic occlusion of the mesenteric vessels. She was treated surgically. This discussion is based on chronic mesenteric ischemia and its nonspecific symptomatology, particularly its association with weight loss., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2019, Bakhtiar et al.)

Hodgkin's lymphoma (HL) is a hematological disorder that has a high cure rate. It usually presents as asymptomatic lymphadenopathy or a mass on chest radiograph along with constitutional symptoms ("B" symptoms such as fever, night sweats, or unintended weight loss) in less than half the cases. Optic neuritis is a demyelinating condition that is rarely associated with HL. We present a case of HL that presented with optic neuritis as a paraneoplastic syndrome., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2019, Khan et al.)

Anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis is a rare clinical entity that typically presents with psychiatric disturbances and neurological deficits. It is commonly associated with ovarian teratomas. Although these patients demonstrate a predilection to develop cardiac arrhythmias, such complications are frequently self-limited. We chronicle here a unique case of a young woman with adnexal teratoma who experienced a tonic-clonic seizure and cardiac arrest. Electrocardiogram showed polymorphic ventricular tachycardia, consistent with torsade de pointes. Based on extensive diagnostic workup and exclusion of probable etiologies, she was diagnosed with anti-NMDA receptor encephalitis. To the best of our knowledge, this report represents the first case of anti-NMDA receptor encephalitis complicated by ictal torsades de pointes, leading to cardiac arrest. This paper illustrates that patients with anti-NMDA receptor encephalitis can develop life-threatening cardiac dysrhythmia and cardiac arrest, requiring urgent management. Clinicians should be vigilant for severe autonomic dysfunction as prompt etiology establishment is of paramount importance in these patients., Competing Interests: The authors have declared that no competing interests exist.

Cardiac papillary fibroelastoma (CPF) is the second most common primary cardiac tumor, which is diagnosed incidentally or with embolic phenomena, mostly in the form of a transient ischemic attack (TIA) and stroke. We present a case of a 58-year-old female who presented with fatigue and low-grade fever and was found to have multiple systemic infarcts. Her blood cultures and transthoracic echocardiography (TTE) were negative, ruling out infective endocarditis. However, transesophageal echocardiography (TEE) revealed a mobile mass at the aortic valve. The mass was surgically removed, and the aortic valve was repaired. The histological examination of the mass finally revealed a papillary fibroelastoma. To our knowledge, this is the first reported case where fibroelastoma presented with splenic and renal infarcts in combination with the cerebral infarcts. Since cardiac fibroelastoma can cause embolization to the cerebral, splenic, and renal vessels, we, therefore, advocate that it should be considered as one of the possible causes of widespread embolism. We also stress upon the importance of doing TEE in case of a suspected cardiac mass, as the TTE is more likely to give false-negative results., Competing Interests: The authors have declared that no competing interests exist.

Substance abusers are at increased risk of acute kidney injury (AKI) compared to the general population due to nontraumatic rhabdomyolysis. The primary target of these nephrotoxic agents is the tubulointerstitial compartment and the most frequent findings on biopsy are consistent with acute tubular necrosis (ATN) and acute interstitial nephritis. We present a rare case of an intravenous cocaine and heroin abuser who presented with recent onset oliguria, hematuria, and reduced peroral intake. The urine dipstick testing showed guaiac positivity but no red blood cells on microscopy. The blood workup showed elevated serum creatinine and urea levels but normal creatinine kinase (CK) level. Renal biopsy showed tubular epithelial cell necrosis and positive immunoperoxidase staining for myoglobin pigment casts in renal tubules. The diagnosis of rhabdomyolysis-associated ATN secondary to substance abuse was suggested. However, normal serum CK levels as well as urine drug panel supported the delayed presentation of rhabdomyolysis complicated with ATN. The patient returned to normal health with no residual kidney dysfunction after undergoing temporary hemofiltration., Competing Interests: The authors have declared that no competing interests exist.

Hemosuccus pancreaticus is a rare but life-threatening cause of upper gastrointestinal bleeding through the main pancreatic duct. This clinical entity is a difficult diagnosis due to its rarity, intermittent nature of the hemorrhage, and peculiar clinical presentation. It is still considered a surgical problem but advances in medical therapy may enable clinically stable patients to undergo less-invasive angiographic embolization. We chronicle here a unique case of hemosuccus pancreaticus in a patient presenting with melena who could not be diagnosed on multiple standard forward-viewing esophagogastroduodenoscopies and computed tomography angiography. Eventually, side-viewing duodenoscope identified the intermittent bleeding through the ampulla of Vater. This paper illustrates that clinicians should be vigilant for this etiology, especially in patients with intermittent crescendo-decrescendo abdominal pain, acute gastrointestinal hemorrhage, and elevated serum lipase levels. A multidisciplinary team approach with the centralization of gastrointestinal bleed services and a well-established management protocol is of paramount importance to reduce the morbidity and mortality of this disorder. Additionally, this article serves to outline our current understanding of the epidemiology of and risk factors for hemosuccus pancreaticus, the pathophysiology of this disease, and currently available approaches to diagnosis and treatment., Competing Interests: The authors have declared that no competing interests exist.

Lafora disease is fatal intractable progressive myoclonic epilepsy. It is frequently characterized by epileptic seizures, difficulty walking, muscle spasms, and dementia in late childhood or adolescence. We chronicle here an unusual case of an asymptomatic young male soccer player who presented with elevated liver enzymes. Neurological examination was unremarkable. The diagnostic workup for hepatitis, infectious etiologies, autoimmune disorders, hemochromatosis, Wilson's disease, alpha-1 antitrypsin deficiency, and other related diseases was inconclusive. He subsequently underwent an uneventful percutaneous liver biopsy. Based on the pathognomonic histopathological findings, Lafora disease was considered the likely etiology. The present study is a unique illustration of this rare disorder initially manifesting with abnormal liver enzymes. It underscores the importance of clinical suspicion of Lafora disease in cases with unexplained hepatic dysfunction. Prompt liver biopsy and genetic testing should be performed to antedate the onset of symptoms in these patients., Competing Interests: The authors have declared that no competing interests exist.

Spontaneous lung herniation is a rare phenomenon in which the lung parenchyma along with the pleural membranes protrudes outside their usual boundaries and can lead to a wide variety of complications. We are reporting a case of a middle-aged male who presented with chronic obstructive pulmonary disease (COPD) exacerbation with severe bouts of cough. Initial computed tomography (CT) chest was unrevealing, but two days later, he developed spontaneous lung herniation, which was initially managed conservatively, but later it progressed to pneumothorax, pneumomediastinum, with striking CT scan images showing extensive subcutaneous emphysema. Blowhole incisions were done on the anterior chest wall which led to ultimate recovery., Competing Interests: The authors have declared that no competing interests exist.

Epidermal nevus syndrome (ENS) is a term used to describe the occurrence of an epidermal nevus in association with other extra-cutaneous developmental anomalies, most commonly involving the nervous and musculoskeletal systems. The nevus is classified on the basis of the main component which may be keratinocytic, sebaceous, follicular, apocrine, or eccrine. Most patients who present with ENS is at the time of birth, though some become apparent later in life. This case describes a young female who presented with seizures and cognitive impairment along with a linear epidermal nevus on the midline of her face. The presence of the nevus prompted brain imaging which showed cortical dysplasia, multiple hamartomas in the temporal lobe, thalamus, and periventricular regions along with cerebellar atrophy and Dandy-Walker variant. To our knowledge, this is the first case in which three different types of brain lesions were found in the same patient., Competing Interests: The authors have declared that no competing interests exist.