Sivagini Ganesh

Rapidly fatal disseminated acanthamoebiasis in a single lung transplant recipient

BACKGROUND Lung transplant recipients are at great risk for developing various infectious complications. These infections portend a significant morbidity and mortality throughout their lifetime following transplantation. At times, cutaneous manifestations are the only clues to systemic infection. CASE REPORT A 62 year-old man with a history of idiopathic pulmonary fibrosis presented 6 months after receiving bilateral sequential cadaveric lung transplantation for anorexia, early satiety, weight loss, exertional dsypnea, arthralgia, and depression. On exam, two rapidly growing non-painful 1.5-3 centimeter erythematous nodules with purulent draining on the anterior chest wall were noted. On Hospital Day 7, the patent was found to be un-responsive, hypotensive, and febrile. Brain imaging revealed diffuse thick nodular enhancement of leptomeningeal surface and multiple areas of hypodenisty associated with mass effect in the bilateral vermis and cerebellar hemispheres with effacement of the fourth ventricle. CSF PCR analysis showed Acanthamoeba sp. confirmed by the Center for Disease Control. Despite multi-modal therapy, his clinical course deteriorated and resulted in brain death. CONCLUSION Acanthamoeba infection is extremely rare in thoracic organ recipients. We report the fifth case of progressive disseminated acanthamoebiasis in a lung transplant recipient.

Use of Foscarnet Therapy for EBV Infection following Control of PTLD with Enhancement of Cellular Immunity in a Lung-Transplant Recipient

Posttransplant lymphoproliferative disorder (PTLD) is a serious complication following solid organ transplantation with an annual incidence rate of 3–5% in lung-transplant recipients. Pathogenesis indicates a strong association with functional over-immunosuppression and EBV infection. Clinical improvement is generally observed with reduction in immunosuppression intensity alone. We present a case of a 24-year-old woman with EBV-associated PTLD following lung transplant where decreasing the immunosuppression improved PTLD but was ineffective against controlling the EBV infection. Foscarnet in combination with immunoglobulins was successfully administered to cause a remission of the EBV infection. This is the second case reported of a persistent EBV infection after reducing immunosuppression levels and evidence of PTLD remission that required foscarnet for EBV infection control.

A 63-Year-Old Woman With Neurofibromatosis Type 1 and Pulmonary Hypertension With Worsening Hypoxemia

CASE PRESENTATION A 63-year-old woman with a history of neurofibromatosis type-1 (NF-1) and pulmonary arterial hypertension (PAH) thought to be secondary to the NF-1 presented with a few weeks of worsening dyspnea on exertion. She took no medications other than sildenafil for her pulmonary hypertension (PH). She denied tobacco, alcohol, and illicit or anorectic drug use. She had previously worked as a waitress. Her mother and her brother had NF-1 but no PH or lung disease.

Clinical features of sarcoidosis associated pulmonary hypertension: Results of a multi-national registry

BACKGROUND Pulmonary hypertension (PH) is a significant cause of morbidity and mortality in sarcoidosis. We established a multi-national registry of sarcoidosis associated PH (SAPH) patients. METHODS Sarcoidosis patients with PH confirmed by right heart catheterization (RHC) were studied. Patients with pulmonary artery wedge pressure (PAWP) of 15 mmHg or less and a mean pulmonary artery pressure (mPAP) ≥ 25 Hg were subsequently analyzed. Data collected included hemodynamics, forced vital capacity (FVC), diffusion capacity of carbon monoxide (DLCO), chest x-ray, and 6-min walk distance (6MWD). RESULTS A total of 176 patients were analyzed. This included 84 (48%) cases identified within a year of entry into the registry and 94 (53%) with moderate to severe PH. There was a significant correlation between DLCO percent predicted (% pred) andmPAP (Rho = -0.228, p = 0.0068) and pulmonary vascular resistance (PVR) (Rho = -0.362, p < 0.0001). PVR was significantly higher in stage 4 disease than in stage 0 or 1 disease (p < 0.05 for both comparisons). About two-thirds of the SAPH patients came from the United States (US). There was a significant difference in the rate of treatment between US (67.5%) versus non-US (86%) (Chi Square 11.26, p = 0.0008) sites. CONCLUSIONS The clinical features of SAPH were similar across multiple centers in the US, Europe, and the Middle East. The severity of SAPH was related to reduced DLCO. There were treatment differences between the US and non-US centers.