Platypnea-Orthodeoxia Syndrome Caused by Patent Foramen Ovale with Right-To-Left Shunt.

BACKGROUND Patent Foramen Ovale (PFO) is an important part of fetal circulation. It allows the oxygenated blood from the umbilical cord to bypass the lungs. PFOs usually close after birth due to the sudden change of the hemodynamics associated with the expansion of the lungs however they are known to persist in about 25% of the total population. One of their rare manifestations is Platypnea-Orthodeoxia Syndrome (POS) that presents as dyspnea upon assuming an upright position, which improves upon recumbency, accompanied by hypoxemia. CASE REPORT We report a case of a 63-year-old man, known to have systemic lupus erythematosus (SLE) and positive anti-phospholipid antibodies but with no prior thrombotic events, admitted with symptoms of SARS-COV2 infection, and developed symptoms of Platypnea-Orthodeoxia Syndrome during his hospitalization, further evaluation by a transthoracic echocardiography revealed he had PFO with a right-to-left shunt which was treated successfully with percutaneous device closure. CONCLUSIONS Platypnea-Orthodeoxia Syndrome (POS) can be associated with various cardiac defects resulting in right-to-left shunts and other non-cardiac pathologies such as pulmonary AV malformations, lung parenchymal diseases and hepatopulmonary syndrome. In cases of cardiac right-to-left shunts Contrast-enhanced Transthoracic Echocardiography (TTE) can effectively diagnose Platypnea-Orthodeoxia Syndrome, and percutaneous closure has shown to be an efficacious treatment option in alleviating the symptoms. This case report highlights the necessity of actively exploring the possibility of PFOs with right-to-left shunts in patients exhibiting POS symptoms, while considering other potential aetiologies.

Encapsulating Peritoneal Sclerosis: A Case Report and Literature Review.

BACKGROUND Encapsulating peritoneal sclerosis (EPS) is a rare, life-threatening, and serious complication of long-term peritoneal dialysis (PD). No evidence-based management strategy has been established until now. Surgical management, including enterolysis and excision of the sclerotic and obstructing adhesions, should be considered as soon as conservative management fails to work. We report a case of EPS soon after transplantation in a patient with end-stage kidney disease who had been on long-term PD. CASE REPORT A 26-year-old man had been found to have advanced chronic kidney disease secondary to glomerulonephritis on pre-employment investigation. He was on continuous ambulatory PD for 5 years, after which he underwent a living donor renal transplant from his full HLA-matched sibling. He did well postoperatively, with excellent graft function. One month after transplantation, he repeatedly presented to our Emergency Department with signs and symptoms of complete small-bowel obstruction. Computed tomography of the abdomen showed features of small-bowel obstruction secondary to interloop adhesions. The patient was initially managed conservatively; however, as his condition continued to deteriorate, an exploratory laparotomy was carried out. Operative findings were suggestive of early EPS localized to the terminal ileum. Total enterolysis along with peritonectomy was performed along with resection of the diseased and obstructing terminal ileum. The patient did well, and he was discharged home day 10 postoperatively. CONCLUSIONS EPS remains a serious and fatal complication of long-term PD. Early definitive diagnosis, treatment, and ultimately surgical intervention may be required to prevent the morbidity and mortality associated with this condition.

Is there a role for immunosuppression in immunoglobulin A nephropathy?

The most common primary glomerular disease globally is IgA nephropathy (IgAN). It is often a slowly progressive disease, and ∼40% of patients will progress to kidney failure. Due to a lack of large clinical trial networks and a lack of surrogate markers of treatment efficacy, there are relatively few large multicenter clinical trials in IgAN. Given that both the pathogenesis and progression of IgAN are linked to defects in mucosal immune regulation and inflammation, use of immunosuppression to prevent kidney failure is well founded. However, recent clinical trials have supported improvement in disease parameters, but this has not always translated to parallel amelioration in longer-term outcome. In this review we summarize the most current clinical research examining the efficacy of immunosuppression in IgAN.

Metabolic syndrome definitions and components in predicting major adverse cardiovascular events after kidney transplantation.

Metabolic syndrome (MetS) associates with cardiovascular risk post-kidney transplantation, but its ambiguity impairs understanding of its diagnostic utility relative to components. We compared five MetS definitions and the predictive value of constituent components of significant definitions for major adverse cardiovascular events (MACE) in a cohort of 1182 kidney transplant recipients. MetS definitions were adjusted for noncomponent traditional Framingham risk factors and relevant transplant-related variables. Kaplan-Meier, logistic regression, and Cox proportional hazards analysis were utilized. There were 143 MACE over 7447 patient-years of follow-up. Only the World Health Organization (WHO) 1998 definition predicted MACE (25.3 vs 15.5 events/1000 patient-years, P = 0.019). Time-to-MACE was 5.5 ± 3.5 years with MetS and 6.8 ± 3.9 years without MetS (P < 0.0001). MetS was independent of pertinent MACE risk factors except age and previous cardiac disease. Among MetS components, dysglycemia provided greatest hazard ratio (HR) for MACE (1.814 [95% confidence interval 1.26-2.60]), increased successively by microalbuminuria (HR 1.946 [1.37-2.75]), dyslipidemia (3.284 [1.72-6.26]), hypertension (4.127 [2.16-7.86]), and central obesity (4.282 [2.09-8.76]). MetS did not affect graft survival. In summary, although the WHO 1998 definition provides greatest predictive value for post-transplant MACE, most of this is conferred by dysglycemia and is overshadowed by age and previous cardiac disease.