Renal Medullary Carcinoma: a Report of the Current Literature.

PURPOSE OF THE REVIEW: We present an updated report of renal medullary carcinoma (RMC), a rare and aggressive condition. RECENT FINDINGS: There is a majority of male patients, of African descent, in the second or third decade of life. In differential diagnosis, other tumors, such as malignant rhabdoid tumor (MRT), vinculin-anaplastic lymphoma kinase (VCL-ALK) translocation renal cell carcinoma, and collecting duct carcinoma, may present difficulties. Abnormalities of tumor suppressor gene SMARCB1 have been found in RMC. Reported symptoms were hematuria, pain, weight loss, respiratory distress, palpable mass, cough, and fever. Most patients present with metastases at diagnosis. There is no definite recommended treatment, and protocols are extrapolated from other malignancies, with nephrectomy and systemic therapies being most frequently used. Response to treatment and prognosis remain very poor. RMC is a rare and aggressive tumor. Definitive diagnosis requires histological assessment and the presence of sickle-cell hemoglobinopathies.

Progressive Multifocal Leukoencephalopathy in a Kidney Transplant Recipient.

Progressive multifocal leukoencephalopathy (PML) is a debilitating and often fatal viral disease of the central nervous system. A 39-year-old male kidney transplant recipient presented to our hospital with weakness of the left arm and leg, his immunosuppressive regimen consisted of tacrolimus, mycophenolate sodium, and meprednisone. Magnetic resonance imaging of the brain revealed hypercellularity in neural tissue and a lesion affecting the right ventral and lateral areas of the thalamus and the posterior limb of the internal capsule. The cerebrospinal fluid tested negative for bacteria, fungi, and acid-fast bacilli; cytology revealed no malignant cells. The clinical presentation, brain imaging, biopsy results showing a demyelinating process, and absence of atypical proliferation all suggested that the first diagnosis to be ruled out was PML. The weakness resolved after withdrawal of tacrolimus and mycophenolate sodium. On day 250 after presentation, no further neurologic deterioration was evident, and renal function was stable. The diagnosis of PML may not always be straightforward. Polymerase chain reaction for detecting John Cunningham virus was not available at our center or in the region. However, a negative result using polymerase chain reaction does not rule out PML.

Work of being an adult patient with chronic kidney disease: a systematic review of qualitative studies.

INTRODUCTION: Chronic kidney disease (CKD) requires patients and caregivers to invest in self-care and self-management of their disease. We aimed to describe the work for adult patients that follows from these investments and develop an understanding of burden of treatment (BoT). METHODS: Systematic review of qualitative primary studies that builds on EXPERTS1 Protocol, PROSPERO registration number: CRD42014014547. We included research published in English, Spanish and Portuguese, from 2000 to present, describing experience of illness and healthcare of people with CKD and caregivers. Searches were conducted in MEDLINE, Embase, CINAHL Plus, PsycINFO, Scopus, Scientific Electronic Library Online and Red de Revistas Científicas de América Latina y el Caribe, España y Portugal. Content was analysed with theoretical framework using middle-range theories. RESULTS: Searches resulted in 260 studies from 30 countries (5115 patients and 1071 carers). Socioeconomic status was central to the experience of CKD, especially in its advanced stages when renal replacement treatment is necessary. Unfunded healthcare was fragmented and of indeterminate duration, with patients often depending on emergency care. Treatment could lead to unemployment, and in turn, to uninsurance or underinsurance. Patients feared catastrophic events because of diminished financial capacity and made strenuous efforts to prevent them. Transportation to and from haemodialysis centre, with variable availability and cost, was a common problem, aggravated for patients in non-urban areas, or with young children, and low resources. Additional work for those uninsured or underinsured included fund-raising. Transplanted patients needed to manage finances and responsibilities in an uncertain context. Information on the disease, treatment options and immunosuppressants side effects was a widespread problem. CONCLUSIONS: Being a person with end-stage kidney disease always implied high burden, time-consuming, invasive and exhausting tasks, impacting on all aspects of patients' and caregivers' lives. Further research on BoT could inform healthcare professionals and policy makers about factors that shape patients' trajectories and contribute towards a better illness experience for those living with CKD. PROSPERO REGISTRATION NUMBER: CRD42014014547.

Prophylaxis of Pulmonary Embolism in Kidney Transplant Recipients.

PURPOSE OF REVIEW: Kidney transplant recipients have an increased risk of pulmonary embolism; however, thromboprophylaxis poses a challenge as the risk of thrombosis must be balanced against the risk of bleeding. This review summarizes the evidence on whether thromboprophylaxis is required in kidney transplantation. RECENT FINDINGS: Incidence of venous thromboembolism, comprising pulmonary embolism and deep venous thrombosis, is increased in kidney transplant recipients compared to the general population, with augmented risk of death and graft loss. Findings suggest a benefit of prophylaxis with heparin and mechanical prophylaxis for low-risk patients. For patients at high risk, with previous thrombosis, and/or abnormal thrombophilia screen, dose and prophylaxis time need to be increased. There is no established thromboprophylaxis strategy. It is crucial to evaluate patient's risk profile and opt for a multidisciplinary approach for the development of appropriate prophylaxis. There remains a paucity of high-quality evidence for effective prophylaxis strategies in this population.

Recurrent Psoriasis After Introduction of Belatacept in 2 Kidney Transplant Recipients.

Organ transplant recipients may have skin diseases as a result of immunosuppression, but psoriasis is reported infrequently. This skin condition may be induced by immunosuppression imbalance. We present 2 cases of recurrent psoriasis in 2 kidney transplant patients with belatacept-based immunosuppressive regimens. Two years after transplant, upon suspicion of calcineurin inhibitor neurotoxicity in the first patient, tacrolimus was replaced with belatacept. The patient's neurological signs resolved but the patient presented with skin lesions compatible with psoriatic plaques, successfully treated with betamethasone dipropionate and hydrocortisone. The second patient had a history of obesity and dyslipidemia, left foot amputation, and psoriasis. He received a kidney transplant, and maintenance immunosuppression included prednisone, mycophenolate mofetil, and belatacept. At posttransplant month 15, the patient presented with cutaneous erythematosus, maculopapular, and desquamative lesions compatible with psoriasis, treated with betamethasone dipropionate. The belatacept-based immunosuppressive regimens were maintained and psoriasis resolved. Psoriasis is a potential complication in kidney recipients that may recur when belatacept is used and/or tacrolimus is withdrawn as it could have happened in the first patient. The characteristics of the second case may suggest that belatacept might not have been the inciting agent. Good results were obtained with topical treatment.

Kidney transplant recipients infected with blaKPC-2-producingKlebsiella pneumonia / Pacientes trasplantados renales con infección por Klebsiella pneumoniae productora de blaKPC-2

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Belatacept-based, ATG-Fresenius-induction regimen for kidney transplant recipients: a proof-of-concept study.

Belatacept provides effective immunosuppression while avoiding the nephrotoxicities associated with calcineurin inhibitors (CNIs). However, existing belatacept-based regimens still have high rates of acute rejection. We hypothesized that therapy with belatacept, mycophenolic acid (MMA), steroids and induction therapy with rabbit anti-thymocyte globulin Fresenius (ATGF), rejection rate could be reduced. Prospective, single center, proof-of-concept study including males and females aged ≥18years, Epstein-Barr virus (EBV)-seropositive recipients of a first, HLA non-identical, live or deceased donor kidney allograft. Only patients with a calculated panel reactive antibody score of 0% were included. Three donors were positive for Chagas disease. Six of twelve patients had at least one infection and five were readmitted to the hospital for treatment. One patient had a Trypanosoma cruzi infection via the graft treated successfully. Median cold ischemia time for the transplant patients with a deceased donor was 21.5h. Mean serum creatinine levels at 1, 3 and 6months were 1.76±0.59, 1.55±0.60 and 1.49±0.60mg/dl, respectively. Two of twelve patients experienced clinical, biopsy-proven rejection, successfully treated with methylprednisolone. No patient developed post-transplant lymphoproliferative disorder (PTLD) or any other malignancy and no patient lost their graft or died during follow-up. The potential of this approach makes it worthy of further investigation.

[Nephrologists' perspectives on kidney donation and transplantation in Argentina]. / La perspectiva de los nefrólogos acerca de la donación y el trasplante renal en la Argentina.

Our aim was to explore and describe Argentine transplant nephrologists' perspectives on the renal transplant situation. Professionals (n = 22) in charge of teams and/or responsible for accepting grafts, donors, and transplant candidates, were specifically chosen. In-depth semistructured interviews were recorded, transcribed, analyzed and thematically coded. More than 50% of these professionals agreed on the need to promote organ donation. Almost half felt that dialysis centers did not refer their patients to transplant centers for their evaluation; as they should. Half of participants stated that the information provided by the allocation team was unreliable. Patients' non-compliance was identified as a major problem, mainly due to socio economic issues. Participants (n = 5/22) maintained that transplantation procedures were stagnant mainly due to low donor rates, poor training or lack of information at hand for nephrologists and transplant nephrologists. Participants alleged the need of further information, training and commitment of health professionals in the identity and referral of donors and transplant candidates. Also, it must be taken into account that socioeconomic difficulties affect the process at different stages. These issues need to be addressed in order to improve transplantation outcome where transparency and equality are concerned.

[Kidney transplantation in patients with donor specific antibodies]. / Trasplante renal en pacientes con anticuerpos dirigidos contra donante.

In renal transplantation, donor specific antibodies (DSAs) detected by sensitive solid-phase assay foresee early antibody-mediated rejections, even with negative complement-dependent cytotoxicity or flow cytometry results. We describe the immunosuppression protocols and outcomes at 10 months of four renal transplant patients in whom anti-HLA DSAs were detected by Luminex® but not by CDC and flow cytometry. The four patients underwent induction treatment with five doses of thymoglobulin at 1.25 mg/kg and 5 doses of intravenous immunoglobulin (IVIG) at 400 mg/kg. In addition, one patient received 20 mg basiliximab on the day of transplant and on post-operative day 4; another patient underwent three sessions of plasmapheresis on days -5, -3, and -1 and also received 1200 mg eculizumab prior to transplant, 900 mg on day 1, and 600 mg each week during one month. In all of them, the maintenance immunosuppressive regimen consisted of tacrolimus, mycophenolate acid and deltisone. All patients had good short-term outcomes. Our findings suggest that patients with anti-HLA DSAs detected only by Luminex® should be monitored closely and can be treated successfully with induction therapy based on thymoglobulin and IVIG.

La perspectiva de los nefrólogos acerca de la donación y el trasplante renal en la Argentina

El objetivo de este trabajo cualitativo fue explorar y describir la perspectiva de los nefrólogos acerca de la situación de la donación y el trasplante en la Argentina. Participaron 22 jefes de equipo de trasplante y/o profesionales a cargo de la toma de decisiones sobre la aceptación de injertos, donantes y candidatos a trasplante, de una muestra intencional. Se realizaron entrevistas en profundidad, semi-estructuradas, que se grabaron, transcribieron, analizaron y codificaron temáticamente. Más de la mitad de los participantes coincidió en la necesidad de promover la donación de órganos; casi la mitad dijo que los centros de diálisis no enviaban todos los pacientes que deberían a realizar estudios para trasplante. La mitad de los participantes confirmó que la información brindada por el equipo de procuración no era confiable. Se identificó la falta de adherencia de los pacientes al tratamiento como un problema serio, principalmente a causa de la situación socio-económica. 5/22 participantes expresaron que la cantidad de trasplantes no aumentaba por bajas tasas de donación, entrenamiento deficiente o falta de información de los nefrólogos. Los participantes percibieron una necesidad de información, entrenamiento y compromiso de los profesionales de la salud en la identificación y derivación de posibles donantes y receptores y señalaron que los problemas socioeconómicos afectan al proceso en diferentes etapas. Estos temas deberían ser tenidos en cuenta para mejorar los resultados del trasplante en un contexto de transparencia y equidad.(AU)

Our aim was to explore and describe Argentine transplant nephrologists perspectives on the renal transplant situation. Professionals (n = 22) in charge of teams and/or responsible for accepting grafts, donors, and transplant candidates, were specifically chosen. In-depth semistructured interviews were recorded, transcribed, analyzed and thematically coded. More than 50% of these professionals agreed on the need to promote organ donation. Almost half felt that dialysis centers did not refer their patients to transplant centers for their evaluation; as they should. Half of participants stated that the information provided by the allocation team was unreliable. Patients non-compliance was identified as a major problem, mainly due to socio economic issues. Participants (n = 5/22) maintained that transplantation procedures were stagnant mainly due to low donor rates, poor training or lack of information at hand for nephrologists and transplant nephrologists. Participants alleged the need of further information, training and commitment of health professionals in the identity and referral of donors and transplant candidates. Also, it must be taken into account that socioeconomic difficulties affect the process at different stages. These issues need to be addressed in order to improve transplantation outcome where transparency and equality are concerned.(AU)

Trasplante renal en pacientes con anticuerpos dirigidos contra donante

En trasplante renal, los anticuerpos donante-específicos por ensayos de fase sólida predicen el rechazo temprano mediado por anticuerpos, incluso con resultados negativos de citometría de flujo o citotoxicidad dependiente del complemento. Aquí se describen los protocolos de inmunosupresión y los resultados a diez meses de cuatro pacientes en los que se detectó anticuerpos donante-específicos anti-antígenos leucocitarios humanos (HLA) por Luminex«, pero no detectados por el método de citotoxicidad dependiente de complemento (CDC) ni por citometría de flujo. Los cuatro pacientes recibieron tratamiento de inducción con 5 dosis de timoglobulina de 1.25 mg/kg y 5 dosis de inmunoglobulina intravenosa (IVIG) de 400 mg/kg. Además, uno recibió 20 mg de basiliximab el mismo día del trasplante y el día 4 postrasplante; otro recibió 3 sesiones de plasmaféresis en los días -5, -3, y -1 y eculizumab en dosis de 1200 mg antes del trasplante, 900 mg el día 1, and 600 mg por semana durante un mes. En todos los casos, la inmunosupresión de mantenimiento consistió en tacrolimus, micofenolato y deltisona. Todos presentaron buenos resultados en el corto plazo. Nuestra experiencia sugiere que los pacientes con anticuerpos donante-específicos anti-HLA detectados solo por Luminex« deben recibir un seguimiento estricto y que en esta población se pueden obtener buenos resultados a partir del uso de terapia de inducción con timoglobulina e IVIG.(AU)

In renal transplantation, donor specific antibodies (DSAs) detected by sensitive solid-phase assay foresee early antibody-mediated rejections, even with negative complement-dependent cytotoxicity or flow cytometry results. We describe the immunosuppression protocols and outcomes at 10 months of four renal transplant patients in whom anti-HLA DSAs were detected by Luminex« but not by CDC and flow cytometry. The four patients underwent induction treatment with five doses of thymoglobulin at 1.25 mg/kg and 5 doses of intravenous immunoglobulin (IVIG) at 400 mg/kg. In addition, one patient received 20 mg basiliximab on the day of transplant and on post-operative day 4; another patient underwent three sessions of plasmapheresis on days -5, -3, and -1 and also received 1200 mg eculizumab prior to transplant, 900 mg on day 1, and 600 mg each week during one month. In all of them, the maintenance immunosuppressive regimen consisted of tacrolimus, mycophenolate acid and deltisone. All patients had good short-term outcomes. Our findings suggest that patients with anti-HLA DSAs detected only by Luminex« should be monitored closely and can be treated successfully with induction therapy based on thymoglobulin and IVIG.(AU)

La perspectiva de los nefrólogos acerca de la donación y el trasplante renal en la Argentina

El objetivo de este trabajo cualitativo fue explorar y describir la perspectiva de los nefrólogos acerca de la situación de la donación y el trasplante en la Argentina. Participaron 22 jefes de equipo de trasplante y/o profesionales a cargo de la toma de decisiones sobre la aceptación de injertos, donantes y candidatos a trasplante, de una muestra intencional. Se realizaron entrevistas en profundidad, semi-estructuradas, que se grabaron, transcribieron, analizaron y codificaron temáticamente. Más de la mitad de los participantes coincidió en la necesidad de promover la donación de órganos; casi la mitad dijo que los centros de diálisis no enviaban todos los pacientes que deberían a realizar estudios para trasplante. La mitad de los participantes confirmó que la información brindada por el equipo de procuración no era confiable. Se identificó la falta de adherencia de los pacientes al tratamiento como un problema serio, principalmente a causa de la situación socio-económica. 5/22 participantes expresaron que la cantidad de trasplantes no aumentaba por bajas tasas de donación, entrenamiento deficiente o falta de información de los nefrólogos. Los participantes percibieron una necesidad de información, entrenamiento y compromiso de los profesionales de la salud en la identificación y derivación de posibles donantes y receptores y señalaron que los problemas socioeconómicos afectan al proceso en diferentes etapas. Estos temas deberían ser tenidos en cuenta para mejorar los resultados del trasplante en un contexto de transparencia y equidad.

Our aim was to explore and describe Argentine transplant nephrologists' perspectives on the renal transplant situation. Professionals (n = 22) in charge of teams and/or responsible for accepting grafts, donors, and transplant candidates, were specifically chosen. In-depth semistructured interviews were recorded, transcribed, analyzed and thematically coded. More than 50% of these professionals agreed on the need to promote organ donation. Almost half felt that dialysis centers did not refer their patients to transplant centers for their evaluation; as they should. Half of participants stated that the information provided by the allocation team was unreliable. Patients' non-compliance was identified as a major problem, mainly due to socio economic issues. Participants (n = 5/22) maintained that transplantation procedures were stagnant mainly due to low donor rates, poor training or lack of information at hand for nephrologists and transplant nephrologists. Participants alleged the need of further information, training and commitment of health professionals in the identity and referral of donors and transplant candidates. Also, it must be taken into account that socioeconomic difficulties affect the process at different stages. These issues need to be addressed in order to improve transplantation outcome where transparency and equality are concerned.