I. Timofte

Belatacept for renal rescue in lung transplant patients.

Renal failure causes morbidity and mortality after lung transplantation and is aggravated by exposure to nephrotoxic immunosuppressant (IS) drugs. We report an off‐label experience using belatacept for lung transplant recipients with severe renal insufficiency to reduce nephrotoxic IS exposure. We analyzed data retrospectively from a consecutive series of lung transplant patients with renal insufficiency in whom belatacept treatment was initiated between June 2012 and June 2014 at the University of Maryland Medical Center. Eight patients received belatacept because of acute or chronic renal insufficiency (median) GFR 24 (IQR 18–26). Glomerular filtration rate (GFR) remained stable in two patients and increased in five. One patient with established renal and respiratory failure received only the induction dose of belatacept and died 4 months later of respiratory and multisystem organ failure. Calcineurin inhibitor or sirolimus exposure was safely withheld or reduced without moderate or severe acute rejection during ongoing belatacept in the other seven patients. FEV1 remained stable over the 6‐month study interval. Belatacept use appears to permit safe transient reduction in conventional immunosuppressive therapy and was associated with stable or improved renal function in a small retrospective series of lung transplant recipients with acute or chronic renal insufficiency.

Venous Thromboembolic Complications of Lung Transplantation: A Contemporary Single-Institution Review

BACKGROUND The incidence and consequences of deep venous thrombosis (DVT) and pulmonary embolism (PE) have not been described recently in lung transplant recipients. We sought to characterize DVT and PE in a contemporary series of lung transplant recipients and describe their association with clinical outcomes. METHODS The records of all lung transplant recipients from July 1, 2008, to June 30, 2013, were reviewed and analyzed. DVT was diagnosed by venous duplex ultrasonography. PE was diagnosed by computed tomography angiography, nuclear ventilation/perfusion scanning, or pulmonary angiography. RESULTS The study comprised 117 patients who underwent 123 transplants. The median age was 63 years (range, 17 to 77 years). Forty-five patients (39%) had evidence of lower extremity DVT, 53 (45%) had no evidence of lower extremity DVT, and 19 (16%) were not tested. Fifty-three (45%) had evidence of upper extremity DVT, 30 (26%) had no evidence of upper extremity DVT, and 34 (29%) were not tested. Eighteen (15%) had evidence of PE, 82 (70%) had no evidence of PE, and 17 (15%) were not tested. A multivariable, stepwise Cox proportional hazards model revealed that the presence of lower extremity DVT (hazard ratio, 2.43; 95% confidence interval, 1.29 to 4.64), use of cardiopulmonary bypass (hazard ratio, 2.21; 95% confidence interval, 1.04 to 4.68), and unilateral lung transplantation (hazard ratio, 2.13; 95% confidence interval, 1.07 to 4.25) were associated with diminished survival. CONCLUSIONS The incidence of DVT and PE in lung transplant recipients is high. Posttransplant surveillance and treatment based on findings are warranted.

Adaptive periodic paralysis allows weaning deep sedation overcoming the drowning syndrome in ECMO patients bridged for lung transplantation: A case series

Purpose: Sedation in extracorporeal membrane oxygenation (ECMO) is challenging. Patients require deep sedation because of extremely high respiratory rates and increased work of breathing (“Drowning Syndrome”) resulting in altered intra‐thoracic pressure and reduced pump flow associated with hemodynamic compromise and decreased oxygenation. However, deep sedation impedes essential active rehabilitation with physical therapy. Methods: We reviewed data on 3 ECMO patients for whom we used a novel approach to replace continuous drips with periodic sedation/paralysis. Initially our patients were on high dose narcotics, propofol, and dexmedetomidine and unable to interact and breathe comfortably. IV narcotics were weaned over 24 h and were replaced by methadone. Dexmedetomidine was continued in order to block hyperadrenergic events. Propofol was weaned at a prescribed rate. When patients demonstrated agitation, decreased pump flow and hemodynamic compromise, diazepam was given in combination with a paralytic. Results: By replacing IV narcotic and propofol, with PRN diazepam and vecuronium, patients were off continuous drips in 1 week and were able to actively participate in physical therapy. Conclusion: Allowing patients to wake up by rapid weaning of continuous narcotics and anesthetic agents using Dexmedetomidine and periodic paralysis to favorably alter hemodynamics is a successful method to wean deep sedation in ECMO. HighlightsA weaning sedation algorithm is proposed for patients requiring ECMO.ECMO patients often require deep sedation that impedes physical therapy and recovery.Sedation is particularly difficult for ECMO patients awaiting lung transplantation.The proposed periodic paralysis‐based algorithm allowed weaning of sedative drips.In 2–3 weeks, patients were awake and actively involved in physical therapy.

Tuberous sclerosis complex: multisystem hamartomas

Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder resulting in the growth of benign tumours or hamartomas affecting any organ system. Up to 90% of patients present with seizures due to growth of benign tumours in the brain.1 Although significant morbidity is associated with cardiac, renal and pulmonary involvement, the neurological aspects and particularly seizures associated with tuberous sclerosis are extremely difficult to treat.1 The hallmark cutaneous manifestations include ash-leaf spots (figure 1 …

Diaphragmatic herniation due to massive hepatomegaly in a patient with pulmonary arterial hypertension.

A 56-year-old woman with severe pulmonary arterial hypertension (PAH) presented with right upper quadrant abdominal pain. The patient was diagnosed with PAH 5 years prior and started on oral therapy, but due to worsening pulmonary haemodynamics and symptoms, intravenous treprostinil was initiated. Imaging studies demonstrated hepatomegaly and a liver biopsy showed hepatic venous outflow obstruction and findings consistent with nodular regenerative hyperplasia. There was no evidence of …