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Racial-ethnic disparities in childhood hypertension
Coral D. HanevoldÂ
doi : 10.1007/s00467-022-05707-x
Volume 38, issue 3, March 2023
Genetic assessment in primary hyperoxaluria: why it matters
Giorgia Mandrile1 · Bodo Beck2 · Cecile Acquaviva3 · Gill Rumsby4 · Lisa Deesker5 · Sander Garrelfs 5 · Asheeta Gupta6 · Justine Bacchetta7 · Jaap Groothoff
doi : 10.1007/s00467-022-05613-2
Accurate diagnosis of primary hyperoxaluria (PH) has important therapeutic consequences. Since biochemical assessment can be unreliable, genetic testing is a crucial diagnostic tool for patients with PH to define the disease type.
Long-term complications of primary distal renal tubular acidosis
Fernando Santos & Helena Gil-Peña
doi : 10.1007/s00467-022-05546-w
The clinical manifestations of primary distal renal tubular acidosis usually begin in childhood, but the disease is caused by a genetic defect that persists throughout life.
“Don’t let me be misunderstood�: communication with patients from a different cultural background
Christina Taylan & Lutz T. Weber
doi : 10.1007/s00467-022-05573-7
In recent years, migration and the social changes associated with it have increasingly become the focus of scientific interest. The diversity of cultures in hospitals poses a major challenge. Medical teams are often confronted with language barriers and different concepts of illness, health, and healing.
A clinical approach to tubulopathies in children and young adults
Rachael Kermond, Andrew Mallett & Hugh McCarthy
doi : 10.1007/s00467-022-05606-1
Kidney tubules are responsible for the preservation of fluid, electrolyte and acid-base homeostasis via passive and active mechanisms. These physiological processes can be disrupted by inherited or acquired aetiologies.
Cardiovascular disease risk factors and lifestyle modification strategies after pediatric kidney transplantation: what are we dealing with, and what can we target?
Se Ri Bae, Alexandra Bicki, Sarah Coufal, Ethan Jin & Elaine Ku
doi : 10.1007/s00467-022-05589-z
Kidney transplantation in pediatric patients can lead to partial improvement of some of the cardiometabolic parameters that increase the risk for cardiovascular disease (CVD) in patients with chronic kidney disease.
A patient presenting with fever after graft loss: Questions
Gülşah Kaya Aksoy, Mustafa Koyun, Elif Çomak, Adil Boz & Sema Akman
A patient presenting with fever after graft loss: Answers
Gülşah Kaya Aksoy, Mustafa Koyun, Elif Çomak, Adil Boz & Sema Akman
Severe acute kidney injury and difficult catheterization in a 6-month-old female infant: Questions
Abishek Selvam, Bobbity Deepthi, Sudarsan Krishnasamy, Gunasekaran Dhandapany, Bikash Kumar Naredi & Sriram Krishnamurthy
Severe acute kidney injury and difficult catheterization in a 6-month-old female infant: Answers
Abishek Selvam, Bobbity Deepthi, Sudarsan Krishnasamy, Gunasekaran Dhandapany, Bikash Kumar Naredi & Sriram Krishnamurthy
Variants in genes coding for collagen type IV α-chains are frequent causes of persistent, isolated hematuria during childhood
Joseph L. Alge, Nasim Bekheirnia, Alexandra R. Willcockson, Xiang Qin, Steven E. Scherer, Michael C. Braun & Mir Reza Bekheirnia
doi : 10.1007/s00467-022-05627-w
Children with persistent, isolated microscopic hematuria typically undergo a limited diagnostic workup and are monitored for signs of kidney disease in long-term longitudinal follow-up, which can delay diagnosis and allow disease progression in some cases.
X-linked hypophosphatemia, obesity and arterial hypertension: data from the XLH21 study
Louisa Bloudeau, Agnès Linglart, Sacha Flammier, Aurélie Portefaix, Aurélia Bertholet-Thomas, Sanaa Eddiry, Anna Barosi, Jean-Pierre Salles, Valérie Porquet-Bordes, Anya Rothenbuhler, Christelle Roger & Justine Bacchetta
doi : 10.1007/s00467-022-05636-9
The underlying mechanisms of obesity in X-linked hypophosphatemia (XLH) are not known. We aimed to evaluate whether FGF21, an endocrine FGF involved in the regulation of carbohydrate–lipid metabolism, could be involved.
Prevalence of cardiac valvar abnormalities in children and young people with autosomal dominant polycystic kidney disease
Alexandra Savis, John M. Simpson, Saleha Kabir, Kelly Peacock, Hayley Beardsley & Manish D. Sinha
doi : 10.1007/s00467-022-05500-w
Valvar abnormalities in children and adults with autosomal dominant polycystic kidney disease (ADPKD) have previously been reported as a frequent occurrence. Mitral valve prolapse (MVP), in particular, has been reported in almost one-third of adult patients and nearly 12% of children with ADPKD.
A rare cause of nephrotic syndrome—sphingosine-1-phosphate lyase (SGPL1) deficiency: 6 cases and a review of the literature
Tugba Tastemel Ozturk, Nur Canpolat, Seha Saygili, Umut Selda Bayrakci, Oguz Soylemezoglu, Fatih Ozaltin & Rezan Topaloglu
doi : 10.1007/s00467-022-05656-5
Recently, recessive mutations in SGPL1 (sphingosine-1-phosphate lyase), which encodes the final enzyme of sphingolipid metabolism, have been reported to cause steroid-resistant nephrotic syndrome, adrenal insufficiency, and many other organ/system involvements. We aimed to determine the clinical and genetic characteristics, and outcomes in patients with SGPL1 mutations.
Urinary protein to creatinine ratio during the first month of life in very preterm infants—a prospective cohort study (PROTIPREMA)
Marine Trigolet, Francesco Bonsante, Jean-Pierre Guignard, Jean-Bernard Gouyon & Silvia Iacobelli
doi : 10.1007/s00467-022-05653-8
Preterm infants have physiological proteinuria and values of urine protein to creatinine ratio (UPr/Cr) are higher compared to full-term infants during the first week of life. Few investigations explored the changes of proteinuria in very preterm infants (VPI, ≤ 31 weeks of gestation) older than a week, and it is unclear whether high and persistent proteinuria is associated with kidney injury in this population.
Urine podocyte mRNA loss in preterm infants and related perinatal risk factors
Qi Gao, Congchao Lu, Xiuying Tian, Jun Zheng & Fangrui Ding
doi : 10.1007/s00467-022-05663-6
Preterm birth has been identified as a risk factor for development of long-term chronic kidney disease. Podocyte loss has been reported to contribute to this process in preterm animal models. However, details about podocyte loss in preterm infants and related perinatal risk factors have not been well clarified.
Etiological diagnosis of post-diarrheal hemolytic uremic syndrome (HUS): humoral response contribution
Gabriela A. Fiorentino, Elizabeth Miliwebsky, MarÃa Victoria Ramos, Gisela Zolezzi, Isabel Chinen, Glenda Guzmán, Rubén Nocera, Romina Fernández-Brando, Adriana Santiago, Ramón Exeni & Marina S. Palermo
doi : 10.1007/s00467-022-05671-6
Hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolysis, thrombocytopenia, and thrombus formation leading to tissue injury. HUS is classified according to its etiology as post-diarrheal or atypical HUS. Differential diagnosis of both entities continues to be a challenge for pediatric physicians.
Vitamin D supplementation in children and young adults with persistent proteinuria secondary to glomerular disease
Amy J. Kogon, Lance S. Ballester, Jarcy Zee, Natalie Walker, Joshua J. Zaritsky, Meredith A. Atkinson, Christine B. Sethna, Andrew N. Hoofnagle, Mary B. Leonard & Michelle R. Denburg
doi : 10.1007/s00467-022-05660-9
Vitamin D deficiency is common in glomerular disease. Supplementation may be ineffective due to ongoing urinary losses of vitamin D binding protein. We sought to determine if daily cholecalciferol supplementation would increase vitamin D concentrations in children with glomerular disease and persistent proteinuria, without adverse effects.
Clinical and pathological investigation of oligomeganephronia
Hideaki Kitakado, Tomoko Horinouchi, Chika Masuda, Atsushi Kondo, Sadayuki Nagai, Yuya Aoto, Nana Sakakibara, Takeshi Ninchoji, Norishige Yoshikawa & Kandai Nozu
doi : 10.1007/s00467-022-05687-y
Oligomeganephronia (OMN) is a rare congenital anomaly involving the kidney and urinary tract, characterized by decreased number and compensatory hypertrophy of the nephron. It is caused by abnormal kidney development during the embryonic period, especially in patients with low birth weight; however, the actual etiology and clinical features remain unknown.
Association of Pediatric Vasculitis Activity Score with immunoglobulin A vasculitis with nephritis
Begüm Avcı, Tuba Kurt, Fatma Aydın, Elif Çelikel, Zahide Ekinci Tekin, Müge Sezer, Nilüfer Tekgöz, Cüneyt Karagöl, Serkan Coşkun, Melike Mehveş Kaplan, Umut Selda Bayrakçı & Banu Acar
doi : 10.1007/s00467-022-05675-2
Immunoglobulin A vasculitis with nephritis (IgAVN) is the most serious complication affecting long-term prognosis. Understanding the risk factors and markers for the development of IgAVN is essential.
Circadian rhythm of water and solute excretion in nocturnal enuresis
Sevasti Karamaria, Lien Dossche, Vincent Delens, Eva Degraeuwe, Ann Raes, Reiner Mauel, Caroline Vande Walle, Karel Everaert & Johan Vande Walle
doi : 10.1007/s00467-022-05645-8
Nocturnal polyuria (NP) due to a suppressed vasopressin circadian rhythm is a well-documented pathogenetic mechanism in enuresis, mainly studied in monosymptomatic enuresis.
Risk factors for developing hyperoxaluria in children with Crohn’s disease
Amr Salem, Happy Sawires, Ayman Eskander, Radwa Marwan & Engy Boshra
doi : 10.1007/s00467-022-05674-3
For the purpose of a better understanding of enteric hyperoxaluria in Crohn’s disease (CD) in children and adolescents, we investigated the occurrence and risk factors for development of hyperoxaluria in those patients.
Urinary HSP70 improves diagnostic accuracy for urinary tract infection in children: UTILISE study
Alev Yilmaz, Alberto Caldas Afonso, Ipek Akil, Bagdagul Aksu, Harika Alpay, Bahriye Atmis, Ozlem Aydog, Aysun Karabay Bayazıt, Meral Torun Bayram, Ilmay Bilge, Ipek Kaplan Bulut, Bahar Buyukkaragoz, Elif Comak, Belde Kasap Demir, Nida Dincel, Osman Donmez, Mehmet Akif Durmus, Hasan Dursun, Ruhan Dusunsel, Ali Duzova, Pelin Ertan, Asuman Gedikbasi, Nilufer Goknar, Sercin Guven, …Rezan Topaloglu
doi : 10.1007/s00467-022-05664-5
The accuracy of conventional urinalysis in diagnosing urinary tract infection (UTI) in children is limited, leading to unnecessary antibiotic exposure in a large fraction of patients. Urinary heat shock protein 70 (uHSP70) is a novel marker of acute urinary tract inflammation.
Incidence of cardiovascular disease and mortality in childhood solid organ transplant recipients: a population-based study
Sandeep Brar, Stephanie N. Dixon, J. Michael Paterson, Jade Dirk, Emma Hahn, S. Joseph Kim, Vicky Ng, Melinda Solomon, Jovanka Vasilevska-Ristovska, Tonny Banh, Paul C. Nathan, Rulan S. Parekh & Rahul Chanchlani
doi : 10.1007/s00467-022-05635-w
With improved survival among children after transplantation, our understanding of the risk for developing other comorbidities is improving, yet little is known about the long-term risk of cardiovascular events and mortality after solid organ transplantation.
Benefits of BNP/NT-proBNP serum level evaluation for dry weight adjustment in pediatric hemodialysis patients
Antoine Mouche, Cyrielle Parmentier, Fatma Fendri, Claire Herbez-Rea, Anne Couderc, Laurène Dehoux, Marina Avramescu, Theresa Kwon, Julien Hogan, Jean-Daniel Delbet & Tim Ulinski
doi : 10.1007/s00467-022-05658-3
Dry weight (DW) adjustment in children on hemodialysis (HD) can be challenging. It relies on clinical evaluation and additional supports. Our aim was to study the benefits of cardiac biomarker assessment, in addition to the more commonly used technique, bioimpedance spectroscopy (BIS), and clinical signs for DW prescription in pediatric HD patients.
Racial-ethnic diversity in ambulatory blood pressure monitoring in children with chronic kidney disease
Reut Pagi, Ora Yadin, Katherine Wesseling-Perry, Keith Norris & Marciana Lee Laster
doi : 10.1007/s00467-022-05659-2
Black adults with chronic kidney disease (CKD) have higher rates of hypertension as compared to White adults with CKD. Little is known of how race and ethnicity associate with the prevalence of hypertension in pediatric CKD patients.
A longitudinal analysis of the effect of anemia on executive functions in children with mild to moderate chronic kidney disease
Nisha S. Singh, Rebecca J. Johnson, Matthew B. Matheson, Joann Carlson, Stephen R. Hooper & Bradley A. Warady
doi : 10.1007/s00467-022-05682-3
Children with chronic kidney disease (CKD) are at risk for cognitive dysfunction. The aim of this study was to investigate associations between executive functions (EF), anemia, and iron deficiency.
Deep learning imaging features derived from kidney ultrasounds predict chronic kidney disease progression in children with posterior urethral valves
John K. Weaver, Karen Milford, Mandy Rickard, Joey Logan, Lauren Erdman, Bernarda Viteri, Neeta D’Souza, Andy Cucchiara, Marta Skreta, Daniel Keefe, Salima Shah, Antoine Selman, Katherine Fischer, Dana A. Weiss, Christopher J. Long, Armando Lorenzo, Yong Fan & Greg E. Tasian
doi : 10.1007/s00467-022-05677-0
We sought to use deep learning to extract anatomic features from postnatal kidney ultrasounds and evaluate their performance in predicting the risk and timing of chronic kidney disease (CKD) progression for boys with posterior urethral valves (PUV).
Monitoring of blood glucose after pediatric kidney transplantation: a longitudinal cohort study
Doaa M. Salah, Mona Hafez, Ftaina I. Fadel, Yasmen Ahmed Said Selem & Noha Musa
doi : 10.1007/s00467-022-05669-0
Glucose metabolism after kidney transplantation (KT) is highly dynamic with the first post-transplantation year being the most critical period for new-onset diabetes after transplantation (NODAT) occurrence.
Immunologic response to SARS-CoV-2 mRNA vaccination in pediatric kidney transplant recipients
Rachael F. Kermond, Justyna E. Ozimek-Kulik, Siah Kim, Stephen I. Alexander, Deirdre Hahn, Alison Kesson, Nicholas Wood, Hugh J. McCarthy & Anne M. Durkan
doi : 10.1007/s00467-022-05679-y
COVID-19 disease in kidney transplant (KT) recipients is associated with increased morbidity, mortality, and hospitalization rates. Unfortunately, KT recipients also have a reduced response to SARS-CoV-2 immunization.
Kidney replacement therapy in neonates: utilization trends and outcomes
Marwa M. Elgendy, Hasan F. Othman, Mohamed A. Mohamed, Raed Bou Matar & Hany Aly
doi : 10.1007/s00467-022-05575-5
We aimed to assess prevalence and clinical characteristics of newborns receiving kidney replacement therapy (KRT).
IPNA clinical practice recommendations for the diagnosis and management of children with steroid-sensitive nephrotic syndrome
Agnes Trautmann, Olivia Boyer, Elisabeth Hodson, Arvind Bagga, Debbie S. Gipson, Susan Samuel, Jack Wetzels, Khalid Alhasan, Sushmita Banerjee, Rajendra Bhimma, Melvin Bonilla-Felix, Francisco Cano, Martin Christian, Deirdre Hahn, Hee Gyung Kang, Koichi Nakanishi, Hesham Safouh, Howard Trachtman, Hong Xu, Wendy Cook, Marina Vivarelli & Dieter Haffner on behalf of the International Pediatric Nephrology Association
doi : 10.1007/s00467-022-05739-3
Idiopathic nephrotic syndrome is the most frequent pediatric glomerular disease, affecting from 1.15 to 16.9 per 100,000 children per year globally. It is characterized by massive proteinuria, hypoalbuminemia, and/or concomitant edema.
Aggressive immunotherapy combined with bortezomib and rituximab for membranous nephropathy associated with enzyme replacement therapy in Pompe disease
Keigo Sasaki, Toru Uchimura, Aya Inaba, Masako Otani, Junko Hanakawa & Shuichi Ito
doi : 10.1007/s00467-022-05672-5
Pompe disease (PD) is a lysosomal glycogen storage disorder caused by a deficiency in acid α-glucosidase (GAA) activity. Various organs, including the skeletal muscle, cardiac muscle, and liver, are commonly involved.
Use of extracorporeal immunomodulation in a toddler with hemophagocytic lymphohistiocytosis and multisystem organ failure
Stuart L. Goldstein, Lenar T. Yessayan, Kelli A. Krallman, Michaela Collins, Stefanie Benoit, Angela Westover & H. David Humes
doi : 10.1007/s00467-022-05692-1
Hemophagocytic lymphohistiocytosis (HLH) is a dysregulated immune disorder in children, associated with Epstein–Barr virus (EBV) infection or malignancies. In severe forms, HLH presents with signs and symptoms of hyperinflammation that progress to life-threatening multiorgan failure.
HOXA11 is another monogenic cause of congenital anomalies of the kidney and urinary tract
Seha Saygili, Salim Caliskan & Fatih Ozaltin
HOXA11 is another monogenic cause of congenital anomalies of the kidney and urinary tract—Reply
Maayan Kagan, Oren Pleniceanu & Asaf Vivante
