Pheochromocytoma associated with von Hippel-lindau disease in a Pakistani family

Objectives: The aim was to study the presentation, disease characteristics, operative outcome, and prognosis in patients with familial Pheochromocytoma associated with von Hippel-Lindau (VHL) disease.Materials and Methods: There were six patients belonging to two generations of a single family who developed features of VHL over a period of 13 years and were treated at our institute. Patients\u27 characteristics, that is, age, gender, presenting complaints and clinical signs, laboratory and biochemical evaluation, and the presence of associated conditions was gathered from medical records. The preoperative and postoperative radiological imaging and histopathological results were also collected.Results: Out of six cases, five were male, and one was female. The mean age at first presentation was 25 years (16-40). All patients presented with uncontrolled hypertension and were found to have Pheochromocytoma on workup. Three patients had unilateral adrenal tumor, and three had bilateral disease. None of the patients had extra-adrenal Pheochromocytoma. All patients were managed with adrenalectomy and had benign pathology. Two patients subsequently had craniotomy for excision of cerebellar hemangioma, and one patient had bilateral partial nephrectomy at the time of adrenalectomy. There was no peri- post-operative mortality and all patients are being followed by the surgeon(s) and endocrinologist.Conclusion: Pheochromocytoma can be a part of familial conditions including VHL. Other associated features should be suspected, investigated, and treated in these patients that can influence patients\u27 clinical course and prognosis. Family members should also be screened to achieve early diagnosis

Nephron sparing surgery for renal tumors-Comparison of off-clamp partial nephrectomy with hilar clamping

Background and objective: Open partial nephrectomy (PN) is still considered gold standard procedure for T1 localized renal tumors. Conventional technique involves clamping of the renal artery with or without vein however, renal ischemia produces a certain level of damage to the kidneys. This study aims to investigate potential effect of off-clamp vs. hilar clamping PN on renal function.Methods: This is a retrospective cohort study of patients who underwent unilateral, open partial nephrectomy for renal tumors b/w January 2009 December 2016 at our institution. A total of 90 partial nephrectomies were performed of which 65 cases were eligible for analysis. Non clamping technique was used in 43 while clamp was applied in 22 patients. Variables studied were patients\u27 demographics, clinical variables, the laterality, tumors size and location, R.E.N.A.L nephrometry score, blood loss, tumor histology and surgical margins. Patients\u27 renal function (serum creatinine and eGFR) were determined pre-operatively, at 3 and 12 months follow up. Data was analyzed on SPSS v. 22.Results: Both the groups were comparable with regards to pre-operative renal function. Mean radiological size of tumor was 4.71±1.31 and 3.81±1.0 (0.003) in two groups respectively. Mean R.E.N.A.L nephrometry score was 6.1±1.5 in off-clamp group compared to 7.05 ± 1.7 in clamp group (p=0.04). No statistically significant difference was found in operative duration, blood loss, positive surgical margins and intra/ peri-operative complications. At three months and one year, renal function was better preserved in non-clamp group compared to clamp group (p=0.001 and 0.007 respectively).Conclusion: Off clamp open partial nephrectomy is safe and feasible option leading to less decline in renal function

Adrenalectomy for solitary metastasis of hepatocellular carcinoma post liver transplantation: case report and literature review

Abstract Liver transplantation (LT) is the treatment of choice for localized hepatocellular carcinoma (HCC) associated with cirrhosis. Extra hepatic metastasis is the most common cause of death in these patients. There is very little evidence regarding the natural history and treatment options for patients developing HCC recurrence after LT. Surgical resection offers a unique opportunity for solitary metastasis. We report a 61 year old male with solitary right adrenal metastasis 15 months post LT which was managed with open adrenalectomy. The patient is alive and disease free 24 months after the surgery. The case, histo-pathological findings and literature review is discussed

Multivessel Coronary Artery Fistula Presenting as Coronary Steal Syndrome Leading to Cardiac Arrest

The coronary steal phenomenon refers to myocardial ischemia caused by the diversion of blood away from normal myocardial circulation. A coronary artery fistula (CAF) is an abnormality of the coronary anatomy characterized by the aberrant termination of a coronary artery or its branches into cardiac chambers or great vessels. Although CAFs are often thought to be asymptomatic, fistulas that diverge a significant amount of blood flow and decrease the normal perfusion of myocardial tissue can cause ischemia and can present with acute coronary syndrome. We describe a unique case of a 70-year-old woman with no coronary artery disease (CAD) undergoing ventricular fibrillation and sudden cardiac arrest from myocardial ischemia secondary to coronary steal brought on by multivessel CAFs. This case was unique in that fistulas originating from the left anterior descending and from the circumflex artery draining into the left heart chambers are the least frequently observed. To our knowledge, only two other reports in the literature, demonstrating sudden cardiac arrest in patients with left anterior descending to left ventricle fistulas with no CAD, exist. The case presented, along with the literature reviewed, demonstrates that CAFs may be an important part of the differential diagnosis of symptoms of chest pain and myocardial ischemia, particularly in middle-aged adults with no history of coronary artery disease or related comorbidities

Congenital anomaly of coronary artery: absence of left circumflex artery

The prevalence of congenital coronary artery anomalies is approximately 1% in the general population. They are a common cause of sudden death in younger persons. Congenital absence of the left circumflex artery is usually a benign condition but can cause symptoms of exertional angina. We present a case of a 59-year-old female who presented with complaints of chest pain. She was evaluated by the cardiology service. An invasive angiogram identified the absence of the circumflex artery, a large right coronary artery, and large septal and diagonal branches of the left main coronary artery possibly as a compensatory mechanism to supply blood to the LCx territories. It is important to define coronary anatomy as anomalies dictate which cardiac intervention should be attempted in cases of ischemia

Political social media marketing:a systematic literature review and agenda for future research

We focus on political marketing and conduct a systematic literature review of journal articles exploring political marketing on social media. The systematic literature review delineates the current state of political social media marketing literature. It spans six databases and comprises sixty-six journal articles published between 2011 and 2020. We identify and categorize the variables studied in the literature and develop an integrative framework that links these variables. We describe the research themes that exist in the literature. The review demonstrates that the field is growing. However, the literature is fragmented, along with being predominantly based in the US context. Conceptual and theoretical shortcomings also exist. Moreover, the literature ignores pertinent contemporary topics such as co-creation, influencer marketing, and political advertising on social media. Nevertheless, a nascent domain with growing practical significance, political social media marketing provides various exciting avenues for further research, which we outline in this study.</p

VV-ECMO in Respiratory Insufficiency

Extracorporeal membrane oxygenation (ECMO) has advanced significantly in the last few decades. Although not FDA-approved in the United States for respiratory insufficiency, it is widely used to support cardiac and pulmonary function via Venoarterial (VA) and Venovenous (VV) ECMO, respectively. In the patient with worsening respiratory failure VV-ECMO is considered a salvaging therapy that gives patients’ lungs time to heal or as a bridge to lung transplant. Clinicians use tools like the Murray score to initiate a referral for VV-ECMO using indices like oxygen requirement, pulmonary compliance, and bilateral opacities. Early referral for VV-ECMO within 7 days of intubation has shown better results. Important factors that are considered in ECMO candidacy are patients’ age, comorbid conditions, and chronic conditions that would affect patients’ overall longevity. Extracorporeal life support organization (ELSO) gets data from ECMO centers worldwide and has general recommendations for centers guiding treatment and management. During the COVID pandemic, there was a huge surge in acute respiratory distress syndrome (ARDS) and rampant use of VV-ECMO for COVID-ARDS. Data from various centers have helped us understand the appropriate use of VV-ECMO for ARDS and other causes of hypoxic and hypercapnic respiratory failure. Early referral and careful screening for the patient for ECMO are of paramount importance for a better outcome

Effectiveness of 10-valent pneumococcal conjugate vaccine against vaccine-type invasive pneumococcal disease in Pakistan

Objective: To assess the effectiveness of 10-valent pneumococcal conjugate vaccine (PCV10) against invasive pneumococcal disease (IPD) due to vaccine serotypes of Streptococcus pneumoniae post introduction of the vaccine into the routine immunization program in Pakistan.Methods: A matched case-control study was conducted at 16 hospitals in Sindh Province, Pakistan. Children aged (eligible to receive PCV10) who presented with radiographically confirmed pneumonia and/or meningitis were enrolled as cases. PCR for the lytA gene was conducted on blood (for radiographic pneumonia) and cerebrospinal fluid (for meningitis) samples to detect S. pneumoniae. The proportion of IPD due to vaccine serotypes (including vaccine-related serogroups) was determined through serial multiplex PCR. For each case, at least five controls were enrolled from children hospitalized at the same institution, matched for age, district, and season.Results: Of 92 IPD patients enrolled during July 2013 to March 2017, 24 (26.0%) had disease caused by vaccine serotypes. Most case (87.5% of 24) and control (66.4% of 134) children had not received any PCV10 doses. The estimated effectiveness of PCV10 against vaccine-type IPD was 72.7% (95% confidence interval (CI) -7.2% to 92.6%) with at least one dose, 78.8% (95% CI -11.9% to 96.0%) for at least two doses, and 81.9% (95% CI -55.7% to 97.9%) for all three doses of vaccine.Conclusion: The vaccine effectiveness point estimates for PCV10 were high and increased with increasing number of doses. However, vaccine effectiveness estimates did not reach statistical significance, possibly due to low power. The findings indicate the likely impact of vaccine in reducing the burden of vaccine-type IPD if vaccine uptake can be improved

Differences in angiographic profile and immediate outcome of primary percutaneous coronary intervention in otherwise risk-free young male smokers

Introduction: Cigarette smoking is a well-established risk factor for the development and progression of coronary artery disease (CAD) and it is strongly related to cardiac morbidity and mortality. Therefore, this study aimed to compare the angiographic profile and immediate clinical outcomes in young male smokers and non-smokers without any other cardiac risk factors presented with ST-elevation myocardial infarction (STEMI).Methods: This study includes young (≤40 years) male patients presented without cardiac risk factors other than smoking. Angiographic profile and immediate outcome of primary percutaneous coronary intervention (PCI) were collected from the hospital database.Results: A total of 580 young male patients were included in this study, 51.2% (297) were smokers. Baseline characteristics and presentation were similar for smoker and non-smoker groups. Angiographic profile was not significantly different for smokers in terms of pre-procedure thrombolysis in myocardial infarction (TIMI) flow (p = 0.373), the number of vessels involved (p = 0.813), infarct-related artery (p = 0.834), and left ventricular dysfunction (p = 0.311). Similarly, in-hospital outcomes of primary PCI were not significantly different in smokers. Post-procedure no-reflow was in 3.4% vs. 2.8%; p = 0.708, acute stent thrombosis in 1.7% vs. 0.4%; p = 0.114 and in-hospital mortality in 1.0% vs. 1.4%; p = 0.657 of the smoker and non-smoker group, respectively.Conclusion: Our study concludes smoking has no significant impact on the angiographic profile and immediate clinical outcomes of primary PCI after STEMI in young males, without any other conventional cardiac risk factors. With these findings, further multicenter prospective studies are needed to identify other potential causes in such patients