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JOURNAL ARTICLE
Alicia Paessler, Colin Forman, Kishore Minhas, Premal Amrishkumar Patel, James Carmichael, Luke Smith, Fayyad Jaradat, Sergio Assia-Zamora, Zainab Arslan, Francis Calder, Samiran Ray, Nicos Kessaris, Jelena Stojanovic
BACKGROUND: 3D printing has been used in different medical contexts, although it is underutilised in paediatrics. We present the first use of 3D printing in the management of three paediatric patients with complex renovascular disease. METHODS: Patient-specific 3D models were produced from conventional 2D imaging and manufactured using 3D polyjet printing technology. All three patients had different underlying pathologies, but all underwent multiple endovascular interventions (renal artery balloon angioplasty) prior to 3D printing and subsequent vascular surgery...
April 16, 2024: Archives of Disease in Childhood
#2
JOURNAL ARTICLE
Alice Ming-Jie Chuah, Eugene Yu-Hin Chan, Pak-Chiu Tong, Alison Lap-Tak Ma
Acquired cystic kidney disease (ACKD) can occur in patients with chronic kidney disease and kidney failure, and its incidence increases with the duration of dialysis. In adults, ACKD is less common in the pre-dialysis group (~ 7%), but its incidence can be as high as 80% for those who are on dialysis for more than ten years. There is, however, very little information about the prevalence of ACKD in children. We report a case of malignant transformation of ACKD following a kidney transplant, highlighting the importance of surveillance of the native kidneys in paediatric patients who have been in long-term kidney replacement therapy...
April 10, 2024: Pediatric Nephrology
#3
JOURNAL ARTICLE
Louise McAlister, Vanessa Shaw, Rukshana Shroff
OBJECTIVE: This study aimed to review the quality and content of phosphate educational materials used in pediatric chronic kidney disease. METHODS: The quality of text-based pediatric phosphate educational materials was assessed using validated instruments for health literacy demands (Suitability Assessment of Materials [SAM] and Patient Education Material Assessment Tool [PEMAT-P]) and readability (Flesch Reading Ease [FRE] and Flesch-Kincaid [FK] Grade Level)...
March 12, 2024: Journal of Renal Nutrition
#4
REVIEW
Giuseppe Basile, Alessio Pecoraro, Andrea Gallioli, Angelo Territo, Camille Berquin, Jorge Robalino, Alejandra Bravo, Jorge Huguet, Óscar Rodriguez-Faba, Pavel Gavrilov, Carmen Facundo, Lluis Guirado, Josep Maria Gaya, Joan Palou, Alberto Breda
Kidney transplantation is the best treatment option for patients with end-stage renal disease owing to improved survival and quality of life compared with dialysis. The surgical approach to kidney transplantation has been somewhat stagnant in the past 50 years, with the open approach being the only available option. In this scenario, evidence of reduced surgery-related morbidity after the introduction of robotics into several surgical fields has induced surgeons to consider robot-assisted kidney transplantation (RAKT) as an alternative approach to these fragile and immunocompromised patients...
March 13, 2024: Nature Reviews. Urology
#5
Punit Jain, Poonam Jain, Robert S Ohgami, Veena Pawar, Kunal Sehgal, Pradnya Chaudhari, Ravindra Nikalji, Tejinder Singh, Vipin Khandelwal, Sanjay Khare, Vaishali Lokhande, Ashwathy Haridas, Laxman Jessani, Kanika Khandelwal
Primary renal involvement by T lymphoblasts is rare among adults with T acute lymphoblastic leukaemia. We report a 28-year-old man presenting with acute renal failure due to infiltration by T lymphoblasts and his response to paediatric-inspired modified BFM-90 protocol. The patient achieved an initial complete remission (CR) but developed central nervous system relapse. He achieved CR2 with cranial irradiation and intrathecal chemotherapy. He underwent a haploidentical transplant in CR2 and remains in remission post-transplant day 330...
February 2024: EJHaem
#6
JOURNAL ARTICLE
Charlotte Withers, Rishil Patel, Ben C Reynolds, Martin Christian, Mordi Muorah, Yincent Tse, Liz Edwards, Pallavi Yadav, Shuman Haq, Shivaram Hegde, Chris J Callaghan, Alasdair Bamford, Stephen D Marks
INTRODUCTION: During the COVID-19 pandemic, evidence emerged that immunosuppressed children were less affected by COVID-19 infections compared with immunosuppressed adults. The aim of our study was to investigate how COVID-19 infections affected paediatric kidney transplant recipients (pKTR) in the UK. METHODS: Questionnaires regarding COVID-19 infection data and care of pKTR during the COVID-19 pandemic were sent to all 13 UK paediatric nephrology centres examining asymptomatic and symptomatic pKTR with positive COVID-19 PCR testing from 1 April 2020 to 1 December 2021...
February 9, 2024: Archives of Disease in Childhood
#7
JOURNAL ARTICLE
Sevcan A Bakkaloğlu, Bahar Büyükkaragöz, Ayşe Seda Pınarbaşı, Emre Leventoğlu, Seha Saygılı, Elif Çomak, Zeynep Y Yıldırım, Nurver Akıncı, İsmail Dursun, Aysun Karabay Bayazıt, Aslı Kavaz Tufan, Sema Akman, Alev Yılmaz, Aytül Noyan, Ayşe Ağbaş, Erkin Serdaroğlu, Ali Delibaş, Ahmet Midhat Elmacı, Mehmet Taşdemir, Fatih S Ezgü, Lale Sever
BACKGROUND: Primary hyperoxaluria type 1 (PH1) is characterized by increased endogenous oxalate production and deposition as calcium oxalate crystals. The main manifestations are nephrocalcinosis/nephrolithiasis, causing impaired kidney function. We aimed to evaluate the clinical characteristics and overall outcomes of paediatric PH1 patients in Turkey. METHODS: This is a nationwide, multicentre, retrospective study evaluating all available paediatric PH1 patients from 15 different paediatric nephrology centres in Turkey...
January 30, 2024: Nephrology
#8
JOURNAL ARTICLE
Katharina Rohner, Matko Marlais, Yo Han Ahn, Alaa Ali, Abrar Alsharief, Anja Blejc Novak, Marta Brambilla, Evrim Kargin Cakici, Cengiz Candan, Nur Canpolat, Eugene Yu-Hin Chan, Stéphane Decramer, Madeleine Didsbury, Filipa Durao, Anne M Durkan, Ali Düzova, Thomas Forbes, Valentina Gracchi, Tulin Güngör, Tomoko Horinouchi, Belde Kasap Demir, Yasuko Kobayashi, Mikael Koskela, Eda Didem Kurt-Sukur, Claudio La Scola, Dean Langan, Xiaozhong Li, Gabriele Malgieri, Antonio Mastrangelo, Jeesu Min, Malgorzata Mizerska-Wasiak, Nabila Moussaoui, Aytul Noyan, Matti Nuutinen, Jennifer O'Gormon, Takayuki Okamoto, Louise Oni, Michiel Oosterveld, Malgorzata Pańczyk-Tomaszewska, Gonul Parmaksiz, Andrea Pasini, Pornpimol Rianthavorn, Joris Roelofs, Yunyan Shen, Rajiv Sinha, Rezan Topaloglu, Diletta Domenica Torres, Tomohiro Udagawa, Martin Wennerström, Yok Chin Yap, Kjell Tullus
BACKGROUND AND HYPOTHESIS: IgA vasculitis with nephritis (IgAVN) is the most common vasculitis in children. Treatment recommendations are, due to a lack of evidence, based on expert opinion resulting in variation. The aim of this study was to describe clinical presentation, treatment and outcome of an extremely large cohort of children with biopsy proven IgAVN to identify prognostic risk factors and signals of treatment efficacy. METHODS: Retrospective data were collected on 1148 children with biopsy proven IgAVN between 2005 and 2019 from 41 international paediatric nephrology centres across 25 countries and analyzed using multivariate analysis...
January 11, 2024: Nephrology, Dialysis, Transplantation
#9
JOURNAL ARTICLE
Gülşah Kaya Aksoy, Mine Erkan, Mustafa Koyun, Elif Çomak, Havva Serap Toru, Derya Mutlu, Bahar Akkaya, Sema Akman
OBJECTIVES: BK polyomavirus-associated nephropathy is a clinicopathological entity that negatively affects graft function in kidney transplant recipients. We compared the efficacy of leflunomide and cidofovir to treat BK polyomavirus-associated nephropathy in pediatric kidney transplant recipients. MATERIALS AND METHODS: Medical records of pediatric recipients with BK viremia for the period 2004 through 2019 were reviewed retrospectively, and patients diagnosed with BK polyomavirusassociated nephro-pathy were included in the study...
December 26, 2023: Experimental and Clinical Transplantation
#10
JOURNAL ARTICLE
Shuichi Ito, Atsuro Saito, Ayako Sakurai, Kenichiro Watanabe, Shuhei Karakawa, Takako Miyamura, Tomoko Yokosuka, Hideaki Ueki, Hiroaki Goto, Hiroshi Yagasaki, Mariko Kinoshita, Michio Ozeki, Norifumi Yokoyama, Hirofumi Teranishi
Haematopoietic stem-cell transplantation (HSCT)-associated thrombotic microangiopathy (HSCT-TMA) is a serious complication with high mortality. Accumulating evidence suggests that complement dysregulation is potentially involved in the development of HSCT-TMA. We retrospectively analysed the clinical characteristics and outcomes of thirteen paediatric patients who were diagnosed with atypical haemolytic uremic syndrome and treated with eculizumab to manage HSCT-TMA during post-marketing surveillance in Japan...
March 2024: Bone Marrow Transplantation
#11
JOURNAL ARTICLE
Julie Martin, Élise Jandot, Valentine Bréant, Anne-Laure Sellier-Leclerc, Christelle Mouchoux, Claire Lattard, Justine Bacchetta, Magali Larger, Xavier Dode, Delphine Hoegy
The pediatric renal graft pathway is at risk of care discontinuation, even though therapeutic adherence is essential. The objective is to evaluate the integration of clinical pharmacy activities into this care pathway. This feasibility study is divided into three stages: structuring, implementing and evaluation. In pre-transplant, immediate and remote post-transplant, interviews were proposed as well as the pharmaceutical analysis of medication prescriptions. In 8 months duration, 32 patients were included...
December 7, 2023: Néphrologie & Thérapeutique
#12
REVIEW
Julia Jefferis, Rebecca Hudson, Paul Lacaze, Andrew Bakshi, Carmel Hawley, Chirag Patel, Andrew Mallett
Kidney function is strongly influenced by genetic factors with both monogenic and polygenic factors contributing to kidney function. Monogenic disorders with primarily autosomal dominant inheritance patterns account for 10% of adult and 50% of paediatric kidney diseases. However, kidney function is also a complex trait with polygenic architecture, where genetic factors interact with environment and lifestyle factors. Family studies suggest that kidney function has significant heritability at 35-69%, capturing complexities of the genome with shared environmental factors...
November 21, 2023: Journal of Nephrology
#13
JOURNAL ARTICLE
Julia Fijo, Ana Sánchez-Moreno
INTRODUCTION AND OBJECTIVES: There is currently no doubt that a kidney transplant with good function is the best treatment we can offer a child with severe kidney failure, improving their growth, development and life in general. But there are few works that follow these patients over the years to find out what their life is like as adults, their achievements and if there are any difficulties that may have arisen from their illness. That has been the objective of this work. MATERIAL AND METHODS: We have collected the evolution of 287 patients who received at least one kidney transplant in pediatric age, analyzing not only the survival of grafts and recipients but, fundamentally, their current quality of life...
November 17, 2023: Nefrología
#14
JOURNAL ARTICLE
Ugonna T Offor, Paolo Hollis, Milos Ognjanovic, Gareth Parry, Abbas Khushnood, Heather M Long, Andrew R Gennery, Chris M Bacon, Jacob Simmonds, Zdenka Reinhardt, Simon Bomken
INTRODUCTION: Paediatric heart transplant patients are disproportionately affected by Epstein-Barr virus (EBV)-related post-transplant lymphoproliferative disease (PTLD) compared with other childhood solid organ recipients. The drivers for this disparity remain poorly understood. A potential risk factor within this cohort is the routine surgical removal of the thymus-a gland critical for the normal development of T-lymphocyte-mediated antiviral immunity-in early life, which does not occur in other solid organ transplant recipients...
October 21, 2023: BMJ Open
#15
JOURNAL ARTICLE
Roelie M Wösten-van Asperen, Hannah M la Roi-Teeuw, Rombout Be van Amstel, Lieuwe Dj Bos, Wim Je Tissing, Iolanda Jordan, Christian Dohna-Schwake, Gabriella Bottari, John Pappachan, Roman Crazzolara, Rosanna I Comoretto, Agniezka Mizia-Malarz, Andrea Moscatelli, María Sánchez-Martín, Jef Willems, Colin M Rogerson, Tellen D Bennett, Yuan Luo, Mihir R Atreya, E Vincent S Faustino, Alon Geva, Scott L Weiss, Luregn J Schlapbach, L Nelson Sanchez-Pinto
BACKGROUND: Identifying phenotypes in sepsis patients may enable precision medicine approaches. However, the generalisability of these phenotypes to specific patient populations is unclear. Given that paediatric cancer patients with sepsis have different host response and pathogen profiles and higher mortality rates when compared to non-cancer patients, we determined whether unique, reproducible, and clinically-relevant sepsis phenotypes exist in this specific patient population. METHODS: We studied patients with underlying malignancies admitted with sepsis to one of 25 paediatric intensive care units (PICUs) participating in two large, multi-centre, observational cohorts from the European SCOTER study (n = 383 patients; study period between January 1, 2018 and January 1, 2020) and the U...
November 2023: EClinicalMedicine
#16
JOURNAL ARTICLE
Bahaaldin Alsoufi, Deborah Kozik, Andrea Nicole Lambert, Sarah Wilkens, Jaimin Trivedi, Shriprassad Deshpande
OBJECTIVE: Infants awaiting paediatric heart transplantation (PHT) experience long waitlist duration and high mortality due to donor shortage. Using the United Network for Organ Sharing database, we explored if increasing donor-recipient weight ratio (DRWR) >2.0 (recommended cutoff) was associated with adverse outcomes. METHODS: Between 2007-2020, 1392 infants received PHT. We divided cohort to 3 groups: A (DRWR ≤1.0, n = 239,17%), B (DRWR 1...
September 13, 2023: European Journal of Cardio-thoracic Surgery
#17
JOURNAL ARTICLE
Étienne Bérard, Jérôme Harambat, Julien Hogan, Cyrielle Parmentier, Clémence Béchade, Mathilde Lassalle
On the occasion of the 20th anniversary of the REIN (French Renal Epidemiology and Information Network), a summary work on the contributions of the national French ESKD register was carried out. On the issue of paediatric CKD patients, the following key messages were retained. Paediatric stage 5 chronic kidney disease (CKD) has particularities that require to be analysed and taken into account because the mortality of these patients remains 30 times higher than that of children of the same age. The REIN registry enables illustrating the specificities of stage 5 CKD in the paediatric age-group in France and providing a set of indicators making it possible to describe the future of these patients as well as the choices made concerning the modalities of replacement therapy...
August 28, 2023: Néphrologie & Thérapeutique
#18
JOURNAL ARTICLE
Valérie Chatelet, Sahar Bayat, Bénédicte Driollet, Véronique Merle, Olivier Moranne, Mathilde Lassalle
On the occasion of the 20th anniversary of the REIN (French Renal Epidemiology and Information Network), a summary work on the contributions of the national French ESKD register was carried out. On the issue of Social Inequalities in Health, the following key messages were retained. Social inequalities in health exist throughout the journey of a patient with chronic kidney disease and manifest as territorial inequalities in access to home-based or independent dialysis treatment and to transplant, whether preemptive or otherwise...
August 28, 2023: Néphrologie & Thérapeutique
#19
JOURNAL ARTICLE
María Azpilicueta-Idarreta, María Prieto-Torre, Lucía Montijano-Herrero, Lorena Fernández-Ruiz, Montserrat Antón-Gamero
INTRODUCTION: Kidney injury associated with paediatric liver transplantation (LT) is common, but its evaluation is challenging. Our aim was to analyse the presence of perioperative acute kidney injury (AKI) and study the prevalence of chronic kidney disease (CKD) using different glomerular filtration rate (GFR) estimation formulas. METHODS: We conducted a cross-sectional study in a cohort of children aged less than 18 years with a history of LT followed up for 5...
October 2023: Anales de pediatría
#20
REVIEW
Kazunori Goto, Takahiro Imaizumi, Riku Hamada, Kenji Ishikura, Tomoki Kosugi, Ichiei Narita, Hitoshi Sugiyama, Akira Shimizu, Hitoshi Yokoyama, Hiroshi Sato, Shoichi Mauryama
BACKGROUND: The Japan Renal Biopsy Registry (J-RBR), a nationwide, web-based, registry system, started in 2007. This study aimed to summarise the epidemiology of biopsy-diagnosed kidney disease in Japan over 10 years. METHODS: We analysed the J-RBR database, from 2007 to 2017. Patients' clinical data collected at the time of biopsy and histopathological diagnoses were used for epidemiological and clinicopathologic analyses. RESULTS: The predominant renal biopsy diagnoses were immunoglobulin A nephropathy (39...
November 2023: Journal of Nephrology
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