Faculty Bibliography

Rituximab has been widely used alone or in combination therapy to treat B-cell non-Hodgkin lymphoma, chronic lymphocytic leukemia, and various autoimmune diseases. Although it is a relatively safe drug, rare rituximab-induced interstitial lung disease (RTX-ILD) has been reported and can be potentially fatal. Here, we report a patient with stage 4 mantle cell lymphoma on rituximab who presented with non-ST segment elevation myocardial infarction in the setting of severe respiratory distress. He underwent left heart catheterization that revealed no new obstructive lesions and patent grafts. Extensive Infectious and autoimmune workup was negative except for Stenotrophomonas maltophilia pneumonia. The patient was diagnosed later with probable RTX-ILD after exclusion of other etiologies, and he did not show any signs of clinical improvement despite antibiotics and steroid therapy. The patient was then discharged to a long-term acute care hospital, where he eventually passed away.

BACKGROUND:Infectious complications have been shown to increase the morbidity of venous-venous extracorporeal membrane oxygenation (VV-ECMO) population, including the use of right ventricular assist devices. AIM/OBJECTIVE:We aimed to evaluate our VV-ECMO population for ECMO related bloodstream infections (E-BSI) and characteristics that affect risk and overall outcomes. METHODS:A retrospective chart review of adult patients (>18 years of age)supported with VV ECMO was conducted. Demographic data as well as antimicrobial use and presecence of bacteremia was collected. RESULTS:We report a low infection rate of 2.7%. CONCLUSIONS:We postulate our low BSI rate may be due to our use of perioperative antimicrobials as well as a majority of our cannulations occurring in the operating room. We do not routinely utilize prophylactic antimicrobials on ECMO. Further investigation into trends, risks, and outcomes related to E-BSI is needed.

Heterotaxy syndrome, also called atrial isomerism, is a rare congenital condition in which the internal organs are abnormally arranged across the left-right axis of the body. It is classified into polysplenia syndrome or left atrial isomerism and asplenia syndrome or right atrial isomerism. It is associated with high morbidity and mortality due to the severity of cardiac anomalies. It is important to be aware of the syndrome findings as they can be incidentally found on imaging in adults. Here, we report a case of a 33-year-old female who presented with worsening shortness of breath, found to have a pulmonary embolism, and heterotaxy was incidentally identified on her imaging. A concise review follows.

Focal segmental glomerulosclerosis (FSGS) is one of the most frequent and severe glomerular kidney disease with frequent progression to end-stage renal disease and a high rate of recurrence in renal transplantations. Due to intolerance or resistance to the current immunomodulatory treatments, the management of FSGS is a therapeutic challenge. Over the last few years, development in extracorporeal therapies has shown potential beneficial outcomes in drug-resistant and recurrent FSGS patients. Thus, this study reviews the current literature on the use of extracorporeal therapies, such as plasma exchange therapy, immunoadsorption, and low-density lipoprotein apheresis, for the treatment of FSGS in the pediatric population.

Malaria is a tropical disease secondary to the Plasmodium parasite with clinical features ranging from febrile illness to acute renal failure and further renal sequelae. We present a case of a woman minimal change disease secondary to Plasmodium falciparum who developed nephrotic range proteinuria and ultimately acute renal failure requiring renal replacement therapy. With proper treatment of her malarial infection as well as long-term renal replacement therapy, she made a full recovery. This case is one of the few cases that highlight the association between severe malarial infections and renal failure necessitating long-term hemodialysis.

The transition from pediatric to adult medical services is an important time in the life of an adolescent or young adult with a renal transplant. Failure of proper transition can lead to medical non-adherence and subsequent loss of graft and/or return to dialysis. The aim of this study was to conduct a systematic review and survey to assess the challenges and existing practices in transition of renal transplant recipient children to adult services, and to develop a transition protocol. We conducted a literature review and performed a survey of pediatric nephrologists across the United States to examine the current state of transition care. A structured transition protocol was developed based on these results. Our literature review revealed that a transition program has a positive impact on decline in renal function and acute rejection episodes, and may improve long-term graft outcomes in pediatric kidney transplant patients. With a response rate of 40% (60/150) from nephrologists in 56% (49/87) of centers, our survey shows inconsistent use of validated tools despite their availability, inefficient communication between teams, and lack of use of dedicated clinics. To address these issues, we developed the "RISE to Transition" protocol, which relies on 4 competency areas: Recognition, Insight, Self-reliance, and Establishment of healthy habits. The transition program decreases acute graft rejection episodes, and the main challenges in transition care are the communication gap between health care providers and inconsistent use of transition tools. Our RISE to transition protocol incorporates transition tools, defines personnel, and aims to improve communication between teams.

The transition from pediatric to adult medical services has a greater impact on the care of adolescents or young adults with chronic diseases such as cystinosis. This transition period is a time of psychosocial development and new responsibilities placing these patients at increased risk of non-adherence. This can lead to serious adverse effects such as graft loss and progression of the disease. Our transition protocol will provide patients, families, physicians, and all those involved a structured guide to transitioning cystinosis patients. This structured protocol depends on four areas of competency: Recognition, Insight, Self-reliance, and Establishment of healthy habits (RISE). This protocol has not been tested and therefore challenges not realized. With a focus on medical, social, and educational/vocational aspects, we aim to improve transition for cystinosis patients in all aspects of their lives.

The blood-spinal cord barrier (BSCB) regulates molecular exchange between blood and spinal cord. Pericytes are presumed to be important cellular constituents of the BSCB. However, the regional abundance and vascular functions of spinal cord pericytes have yet to be determined. Utilizing wild-type mice, we show that spinal cord pericyte capillary coverage and number compared with the brain regions are reduced most prominently in the anterior horn. Regional pericyte variations are highly correlated with: (1) increased capillary permeability to 350 Da, 40,000 Da, and 150,000 Da, but not 2,000,000 Da fluorescent vascular tracers in cervical, thoracic, and lumbar regions and (2) diminished endothelial zonula occludens-1 (ZO-1) and occludin tight junction protein expression. Pericyte-deficient mutations (Pdgfrβ(F7/F7) mice) resulted in additional pericyte reductions in spinal cord capillaries leading to overt BSCB disruption to serum proteins, accumulation in motor neurons of cyotoxic thrombin and fibrin and motor neuron loss. Barrier disruption in perciyte-deficient mice coincided with further reductions in ZO-1 and occludin. These data suggest that pericytes contribute to proper function of the BSCB at the capillary level. Regional reductions in spinal cord pericytes may provide a cellular basis for heightened spinal cord barrier capillary permeability and motor neuron loss.