Methemoglobinemia is a rare dyshemoglobin disorder which can either be congenital or acquired. Dyshemoglobin disorders can be asymptomatic or symptomatic. We narrate the case of a 12-year-old girl who presented with a fever, cough, and oxygen saturation of 85%. She was diagnosed with COVID-19, along with a large atrial septal defect and pulmonary arterial hypertension. Arterial blood gas analysis revealed normal partial pressure of oxygen and on 100% exposure to oxygen, blood color turned chocolate brown. After the resolution of COVID-19 in 10 days, the patient was treated with oral ascorbic acid and successful atrial septal defect repair. It is important to suspect dyshemoglobin disorder in a patient who presents with hypoxia/hypoxemia.

Keywords: Methemoglobinemia; Cyanosis; Pediatrics; Heart Disease; India.

Idiopathic capillary leak syndrome, also known as Clarkson’s Disease, is a rare cause of hypovolemic shock that physicians should be aware of. It is characterized by a state of hypovolemia with features of widespread fluid third spacing and poses diagnostic and therapeutic challenges. Here, we present a challenging case of a 36-year-old woman who experienced recurrent episodes of widespread edema and hypovolemic shock symptoms suggestive of capillary leak syndrome. The resuscitative and therapeutic measures employed in managing this disease are described in this case report.

Keywords: Capillary Leak Syndrome; Hypovolemia; Shock; Edema.

Autosomal dominant polycystic kidney disease (ADPKD) is a common inherited condition characterized by the growth of multiple bilateral cysts in the kidneys. We describe the case of a 35-year-old male with combined ADPKD and type 1 diabetes mellitus with a strong family history of both. At the age of 32, he developed end-stage kidney disease for which he underwent preemptive simultaneous pancreatic and kidney transplant, which in turn led to multiple perioperative complications. Evaluation of familial clustering of genetic disease is critical in genetic epidemiology and precision medicine as it enables estimation of lifetime disease risk and early assessment as well as detection of the disease among one’s siblings.

Keywords: Polycystic Kidney Disease; Type 1 Diabetes Mellitus; End-Stage Kidney Disease; Pancreatic Islets Transplantation; Kidney Transplantation; Genetic Diseases; Oman.

An 11-year-old girl presented with an extremely rare complication of Meckel's diverticulum. The patient presented with complaints of abdominal distension, abdominal pain, decreased appetite, and non-bilious vomiting for 20 days with a history of mass protruding per rectum. Examination revealed a distended abdomen and prolapsing bowel loops during rectal examination, resembling intussusception. Radiological findings indicated intestinal obstruction. Surgical exploration revealed Meckel's diverticulum invading the rectum, accompanied by dense inter-bowel adhesions. The patient underwent resection of Meckel’s diverticulum and repair of the rectal rent. This case highlights the rarity of Meckel's diverticulum fistulating into the rectum.

Keywords: Meckel Diverticulum; Intestinal Obstruction; Pediatric Surgery.

Cardiac papillary fibroelastoma (PFE) is a rare benign cardiac tumor, usually associated with cardiac valves. Cases of non-valvular, left ventricular (LV) cavity PFE are extremely rare. We report the case of a 75-year-old man with a history of exertional chest pain. He was referred to our tertiary-care hospital for evaluation. On the echocardiogram, the patient was found to have a LV lesion related to the papillary muscle. Cardiac magnetic resonance imaging revealed an enhancing mobile lesion related to the LV anterolateral papillary muscle which suggested a myxoma. The patient underwent surgical resection of the lesion, later histopathologically confirmed to be PFE.

Keywords: Cardiac Papillary Fibroelastoma; Cardiac Tumor; Oman.

Kikuchi disease, also called, Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis, is a rare self-limiting illness with an unknown etiology and pathogenesis. It is predominantly seen among young females. The cardinal clinical features include fever and cervical lymphadenopathy. Skin eruptions have also been reported. In Oman, two cases have been reported to date, in 2005 and 2020, with only one case exhibiting skin manifestations. There is a paucity of disease in our country and worldwide. In this case report, we discuss the diagnosis of Kikuchi disease in a previously healthy 17-year-old Omani female who presented with fever, cervical lymphadenopathy, and malar rash. The clinical picture in this case resembled that of systemic lupus erythematosus. Due to the rarity of Kikuchi disease, particularly in our region, it is crucial to consider it as a differential diagnosis when a patient exhibits the aforementioned symptoms to prevent misdiagnosis and inappropriate treatment, as it can easily be misdiagnosed as systemic lupus erythematosus.

Keywords: Histiocytic Necrotizing Lymphadenitis; Lymphadenopathy; Oman.

Objectives: To evaluate the impact of Pulmonary Arterial Hypertension (PAH) therapies on the incidence of pericardial effusion and its prognostic implications for patient survival. Methods: This retrospective cohort study included 60 patients diagnosed with PAH at a high-volume tertiary care center, treated with intravenous or subcutaneous prostanoids. Data were collected from 2015 to 2019, including echocardiographic assessments, right heart catheterization, World Health Organization functional class evaluations, six-minute walk distance tests, and biomarkers such as brain natriuretic peptide and N-terminal prohormone of brain natriuretic peptide. Follow-up was conducted at least 90 days post-treatment initiation. Results: Pericardial effusion was observed in 31.7% of patients before therapy. Patients with moderate to large effusions had a significantly higher mortality risk (HR = 1.92; 95% CI 1.1–44.78; p = 0.0044), while small effusions appeared protective (HR = 0.27; 95% CI 0.15–0.48; p = 0.006). Survival rates declined from 89% at one year to 71% at three years post-therapy, with effusion presence correlating with more severe PAH manifestations. Conclusions: Initial pericardial effusion severity is a critical predictor of mortality in PAH patients. Early assessment and stratified management of pericardial effusion are essential for optimizing therapeutic outcomes in PAH management. Future research should explore targeted interventions for managing pericardial effusion to improve patient prognosis.

Keywords: Pericardial Effusion; Hypertension, Pulmonary; Vasodilator Agents.