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Welcome and farewell
Lesley Rees, Michel Baum & Joseph Flynn
doi : 10.1007/s00467-020-04868-x
Pediatric Nephrology volume 36, page217(2021)
CKDu: the known unknowns
A. S. Abeyagunawardena & R. Shroff
doi : 10.1007/s00467-020-04596-2
Pediatric Nephrology volume 36, pages219–221(2021)
Adult complications of nephropathic cystinosis: a systematic review
Rachel Nora Kasimer & Craig B Langman
doi : 10.1007/s00467-020-04487-6
Pediatric Nephrology volume 36, pages223–236(2021)
While nephropathic cystinosis is classically thought of as a childhood disease, with improved treatments, patients are more commonly living into adulthood. We performed a systematic review of the literature available on what complications this population faces as it ages. Nearly every organ system is affected in cystinosis, either from the disease itself or from sequelae of kidney transplantation. While cysteamine is known to delay the onset of end-stage kidney disease, its effects on other complications of cystinosis are less well determined. More common adult-onset complications include myopathy, diabetes, and hypothyroidism. Some less common complications, such as neurologic dysfunction, can still have a profound impact on those with cystinosis. Areas for further research in this area include additional study of the impact of cysteamine on the nonrenal manifestations of cystinosis, as well as possible avenues for new and novel treatments.
The importance of the epithelial sodium channel in determining salt sensitivity in people of African origin
Erika Jones & Brian Rayner
doi : 10.1007/s00467-019-04427-z
Pediatric Nephrology volume 36, pages237–243(2021)
Hypertension is highly prevalent in Black Africans and has been found to be associated with worse blood pressure (BP) control and more cardiovascular disease. Black Africans are more salt sensitive with low renin and aldosterone levels. This can be explained in part by variants in the epithelial sodium channel (ENaC) causing an increase in channel activity resulting in sodium and water retention. These variants in the ENaC are increased in the Black African populations presumably due to selective pressure for sodium retention in traditionally low-salt diets. Furthermore, increased endothelial sodium channel activity contributes to the risk of vascular stiffness, which may also result in more difficult to control hypertension. Patients with increased activity of the ENaC are more likely to respond to amiloride (a selective sodium channel antagonist), which has implications for the management of severe and resistant hypertension in Black Africans. A large-scale controlled trial on the use of amiloride compared to usual care is warranted in Blacks with severe or resistant hypertension.
Donor considerations in pediatric kidney transplantation
Jayanthi Chandar, Linda Chen, Marissa Defreitas, Gaetano Ciancio & George Burke III
doi : 10.1007/s00467-019-04362-z
Pediatric Nephrology volume 36, pages245–257(2021)
This article reviews kidney transplant donor options for children with end-stage kidney disease (ESKD). Global access to kidney transplantation is variable. Well-established national policies, organizations for organ procurement and allocation, and donor management policies may account for higher deceased donor (DD transplants) in some countries. Living donor kidney transplantation (LD) predominates in countries where organ donation has limited national priority. In addition, social, cultural, religious and medical factors play a major role in both LD and DD kidney transplant donation. Most children with ESKD receive adult-sized kidneys. The transplanted kidney has a finite survival and the expectation is that children who require renal replacement therapy from early childhood will probably have 2 or 3 kidney transplants in their lifetime. LD transplant provides better long-term graft survival and is a better option for children. When a living related donor is incompatible with the intended recipient, paired kidney exchange with a compatible unrelated donor may be considered. When the choice is a DD kidney, the decision-making process in accepting a donor offer requires careful consideration of donor history, kidney donor profile index, HLA matching, cold ischemia time, and recipient’s time on the waiting list. Accepting or declining a DD offer in a timely manner can be challenging when there are undesirable facts in the donor’s history which need to be balanced against prolonging dialysis in a child. An ongoing global challenge is the significant gap between organ supply and demand, which has increased the need to improve organ preservation techniques and awareness for organ donation.
One size does not fit all: understanding individual living kidney donor risk
Elham Asgari & Rachel M. Hilton
doi : 10.1007/s00467-019-04456-8
Pediatric Nephrology volume 36, pages259–269(2021)
Living donor kidney transplantation is the optimal treatment for end-stage kidney disease (ESKD) but confers a risk upon the donor, both in the short term and many years after donation. While perioperative mortality is low and longevity does not appear to be adversely affected, there are small increases in the risk of other important morbidities. The overall risk of ESKD among donors is low but appears to be three- to five-fold higher than among healthy non-donors, and this relative risk is even higher among donors of African ancestry. For these individuals, apolipoprotein L1 genotyping may be helpful. Kidney donors also have an increased risk of developing hypertension post-donation and a modestly increased risk of developing gout. Living kidney donation also increases the risk of gestational hypertension and preeclampsia while not affecting other important pregnancy outcomes. As our understanding of donor risk grows, it is important to counsel prospective donors according to their individual risk and so obtain better informed donor consent. As knowledge advances, it is also important that all clinicians who manage kidney transplant candidates have an up to date understanding of donor risk to inform shared decision making.
Distribution and management of the pediatric refugee population with renal replacement: A German pediatric cohort
Johanna Lemke, Raphael Schild, Martin Konrad, Lars Pape, Jun Oh & Members of the German Society of Pediatric Nephrology (GPN)
doi : 10.1007/s00467-019-04374-9
Pediatric Nephrology volume 36, pages271–277(2021)
With migration rising, the pediatric nephrology community is faced with challenges concerning the management of end-stage kidney disease (ESKD) in the pediatric refugee population. Data on the care of the pediatric refugee cohort on renal replacement therapy (RRT) is not available. A survey conducted by us in 2018 showed that the group of refugee children arriving to Germany during the years 2015–2017 accounts for approximately 20% of the total pediatric dialysis population in Germany. Provision of (medical) care for these children and their families is often hampered by psychosocial problems, cultural differences, language barriers, and administrative issues. Treating centers need to provide additional human as well as financial and logistic resources. In this educational review, we raise awareness and discuss possible challenges occurring in the treatment of refugee children with ESKD.
Renal involvement in paediatric inflammatory bowel disease
Mohamed Mutalib
doi : 10.1007/s00467-019-04413-5
Pediatric Nephrology volume 36, pages279–285(2021)
Inflammatory bowel disease (IBD), which includes Crohn’s disease, ulcerative colitis and inflammatory bowel disease unclassified, is a chronic inflammatory disorder that predominantly affects the gastrointestinal (GI) tract and has a rising incidence in both children and adults. Symptoms are caused by inappropriate inflammatory response triggered by interaction between the environment, gut microbiome and host immune system in a genetically susceptible individual. Extranintestinal manifestations of IBD are common and can affect any body system outside the gut; they can precede or run parallel to GI inflammation. Renal involvement in IBD is uncommon and can be part of extraintestinal manifestation or metabolic complications of IBD. Many medications used to treat IBD can cause renal damage. Renal manifestation in children with IBD can range from asymptomatic biochemical abnormalities to variable stages of renal impairment with significant morbidity and even mortality burden.
Protein energy wasting; what is it and what can we do to prevent it?
Lesley Rees
doi : 10.1007/s00467-019-04424-2
Pediatric Nephrology volume 36, pages287–294(2021)
Some children with declining height and BMI SDS fail to respond to optimisation of nutritional intake. As well as poor growth, they have muscle wasting and relative preservation of body fat. This is termed protein energy wasting (PEW). The process results from an interaction of chronic inflammation alongside poor nutritional intake. This review discusses the causes and potential preventative therapies for PEW.
A 5-year-old boy with refractory rickets, polyuria, and hypokalemic metabolic alkalosis: Questions
Aakash Chandran Chidambaram, Sriram Krishnamurthy, Saragondlu Lakshminarasappa Darshith, Pediredla Karunakar, Bobbity Deepthi, Dhandapany Gunasekaran & Jaikumar Govindaswamy Ramamoorthy
doi : 10.1007/s00467-020-04610-7
Pediatric Nephrology volume 36, pages295–296(2021)
A 5-year-old boy with refractory rickets, polyuria, and hypokalemic metabolic alkalosis: Answers
Aakash Chandran Chidambaram, Sriram Krishnamurthy, Saragondlu Lakshminarasappa Darshith, Pediredla Karunakar, Bobbity Deepthi, Dhandapany Gunasekaran & Jaikumar Govindaswamy Ramamoorthy
doi : 10.1007/s00467-020-04616-1
Pediatric Nephrology volume 36, pages297–302(2021)
Facial nerve paralysis in hypertension: Questions
Alice Morag MacArthur & Susie Minson
doi : 10.1007/s00467-020-04627-y
Pediatric Nephrology volume 36, pages303–304(2021)
Facial nerve paralysis in hypertension: Answers
Alice Morag MacArthur & Susie Minson
doi : 10.1007/s00467-020-04628-x
Pediatric Nephrology volume 36, pages305–306(2021)
Unrelenting lumbar pain in a female adolescent: Questions
Agustina Alonso, Gonzalo Anés, Ana Vivanco-Allende, Ana Menezes, Miguel Hevia & Fernando Santos
doi : 10.1007/s00467-020-04625-0
Pediatric Nephrology volume 36, pages307–308(2021)
Unrelenting lumbar pain in a female adolescent: Answers
Agustina Alonso, Gonzalo Anés, Ana Vivanco-Allende, Ana Menezes, Miguel Hevia & Fernando Santos
doi : 10.1007/s00467-020-04626-z
Pediatric Nephrology volume 36, pages309–312(2021)
Acute kidney injury in an adolescent: Questions
Hulya Nalcacioglu, Demet Tekcan, Bilge Can Meydan & Ozan Ozkaya
doi : 10.1007/s00467-020-04641-0
Pediatric Nephrology volume 36, pages313–314(2021)
Acute kidney injury in an adolescent: Answers
Hulya Nalcacioglu, Demet Tekcan, Bilge Can Meydan & Ozan Ozkaya
doi : 10.1007/s00467-020-04648-7
Pediatric Nephrology volume 36, pages315–317(2021)
Acute urinary retention during menstruation: Questions
Hannah Jeffery & Swati Jha
doi : 10.1007/s00467-020-04637-w
Pediatric Nephrology volume 36, pages319–320(2021)
Acute urinary retention during menstruation: Answers
Hannah Jeffery & Swati Jha
doi : 10.1007/s00467-020-04646-9
Pediatric Nephrology volume 36, pages321–322(2021)
Chronic kidney disease impacts health-related quality of life of children in Uganda, East Africa
Peace D. Imani, Judith Aujo, Sarah Kiguli, Poyyapakkam Srivaths & Eileen D. Brewer
doi : 10.1007/s00467-020-04705-1
Pediatric Nephrology volume 36, pages323–331(2021)
Limited data exist about causes of chronic kidney disease (CKD) and impact on health-related quality of life (HRQoL) in African children. We evaluated types of kidney disease in Ugandan children 0–18 years and compared HRQoL in children with CKD or with benign or resolving kidney disease (non-CKD) to assess predictors of HRQoL.
Kidney failure risk in type 1 vs. type 2 childhood-onset diabetes mellitus
Oren Pleniceanu, Gilad Twig, Dorit Tzur, Noah Gruber, Michal Stern-Zimmer, Arnon Afek, Tomer Erlich, Lital Keinan-Boker, Karl Skorecki, Ronit Calderon-Margalit & Asaf Vivante
doi : 10.1007/s00467-020-04631-2
Pediatric Nephrology volume 36, pages333–340(2021)
Diabetic kidney disease (DKD) is becoming increasingly common among children. We aimed to estimate the risk of end-stage renal disease (ESKD) and mortality among adolescents with type 1 diabetes mellitus (T1DM) or type 2 diabetes mellitus (T2DM) and normal renal function compared with non-diabetics. We hypothesized that childhood onset T1DM vs. T2DM would be associated with a different risk profile for developing ESKD and its complications.
Mycophenolic acid area under the concentration-time curve is associated with therapeutic response in childhood-onset lupus nephritis
Astrid Godron-Dubrasquet, Jean-Baptiste Woillard, Stéphane Decramer, Marc Fila, Vincent Guigonis, Stéphanie Tellier, Denis Morin, Maud Sordet, Frank Saint-Marcoux & Jérôme Harambat
doi : 10.1007/s00467-020-04733-x
Pediatric Nephrology volume 36, pages341–347(2021)
Mycophenolic acid (MPA), the active compound of mycophenolate mofetil (MMF), is widely used in lupus nephritis treatment. Therapeutic drug monitoring of adults suggests that area under the concentration-time curve (AUC) of MPA (MPA-AUC) is associated with clinical outcomes, but childhood data are scarce.
Steroid treatment promotes an M2 anti-inflammatory macrophage phenotype in childhood lupus nephritis
Yohei Ikezumi, Tomomi Kondoh, Yuji Matsumoto, Naonori Kumagai, Masahiro Kaneko, Hiroya Hasegawa, Takeshi Yamada, Utako Kaneko & David J. Nikolic-Paterson
doi : 10.1007/s00467-020-04734-w
Pediatric Nephrology volume 36, pages349–359(2021)
M1-type proinflammatory macrophages (M?) promote glomerular injury in lupus nephritis (LN). However, whether this phenotype is altered by steroid therapy is unclear. Therefore, we investigated the effect of steroid treatment on M? phenotype in LN.
Evolution of isolated systolic hypertension with normal central blood pressure in adolescents—prospective study
?ukasz Obrycki, Janusz Feber, Gra?yna Brzezi?ska & Mieczys?aw Litwin
doi : 10.1007/s00467-020-04731-z
Pediatric Nephrology volume 36, pages361–371(2021)
The clinical significance of isolated systolic hypertension with normal central blood pressure known as spurious hypertension (sHT) in adolescents and its evolution over time is not known.
Challenges of diagnosing pediatric hypertension using ambulatory blood pressure monitoring
Jason Thomas, Emily Stonebrook, Brett Klamer, Hiren P. Patel & Mahmoud Kallash
doi : 10.1007/s00467-020-04725-x
Pediatric Nephrology volume 36, pages373–378(2021)
Ambulatory blood pressure monitoring (ABPM) measures mean arterial pressure (MAP) then extrapolates systolic and diastolic blood pressure (BP) values. Pediatric guidelines recommend using calculated systolic and diastolic BP rather than measured MAP for diagnosis of ambulatory hypertension (HTN). The 95th percentile BP that defines ambulatory HTN is higher in some children than thresholds used to define ambulatory HTN in adults.
Masked hypertension and its associations with glycemic variability metrics in children and adolescents with type 1 diabetes
Warinpapha Homhuan, Preamrudee Poomthavorn, Witchuri Paksi, Patcharin Khlairit, Arkom Nongnuch & Kwanchai Pirojsakul
doi : 10.1007/s00467-020-04720-2
Pediatric Nephrology volume 36, pages379–386(2021)
Masked hypertension defined as having normal office blood pressure (BP) but hypertension detected by continuous BP monitoring has been observed in children and adolescents with type 1 diabetes (T1D). However, no study has evaluated whether masked hypertension is associated with glycemic variability (GV) in these patients. We hypothesized that masked hypertension might be associated with high GV in patients with T1D.
Biomarkers of kidney injury among children in a high-risk region for chronic kidney disease of uncertain etiology
Jessica H. Leibler, Oriana Ramirez-Rubio, Juan José Amador Vel?zquez, Damaris L?pez Pilarte, Wassim Obeid, Chirag R. Parikh, Salini Gadupudi, Madeleine K. Scammell, David J. Friedman & Daniel R. Brooks
doi : 10.1007/s00467-020-04595-3
Pediatric Nephrology volume 36, pages387–396(2021)
Mesoamerican Nephropathy (MeN), a form of chronic kidney disease of uncertain etiology, is a leading cause of death in Central America. The disease often presents in young adult male agricultural workers and progresses rapidly. Given the young age at presentation, we hypothesized that children in Central America experience subclinical kidney injury prior to working life.
Vitamin C deficiency and impact of vitamin C administration among pediatric patients with advanced chronic kidney disease
Nattaphorn Hongsawong, Notethasoung Chawprang, Kulnipa Kittisakmontri, Parach Vittayananan, Konggrapun Srisuwan & Wattana Chartapisak
doi : 10.1007/s00467-020-04662-9
Pediatric Nephrology volume 36, pages397–408(2021)
Vitamin C deficiency is common in chronic kidney disease (CKD) due to losses through dialysis and dietary intake below requirement. We investigated prevalence of vitamin C deficiency and impact of vitamin C treatment in deficient/insufficient patients.
The need for hemodialysis is associated with increased mortality in mechanically ventilated children: a propensity score–matched outcome study
Madhuradhar Chegondi, Sushil Devarashetty, Niveditha Balakumar, Prithvi Sendi & Balagangadhar R. Totapally
doi : 10.1007/s00467-020-04703-3
Pediatric Nephrology volume 36, pages409–416(2021)
Kidney replacement therapy (KRT) is frequently used in critically ill children. The objective of this study is to investigate if the requirement for hemodialysis (HD) is an independent risk factor for mortality in mechanically ventilated children
Evaluation of non-infectious complications of peritoneal dialysis in children: a multicenter study
Gulsah Kaya Aksoy, Mesiha Ekim, Sevcan A. Bakkalo?lu, Seda Co?kun, Ali Deliba?, Seçil Conkar, Dilek Y?lmaz, Asl?han Kara, Seha K. Sayg?l?, Bahar Büyükkarag?z, Zeynep Y. Y?ld?r?m, Elif ?omak, Metin K. Gürg?ze, Lale Sever, Aytül Noyan, Aysun K Bayaz?t & Ruhan Dü?ünsel
doi : 10.1007/s00467-020-04719-9
Pediatric Nephrology volume 36, pages417–423(2021)
Peritoneal dialysis (PD) is the most common kidney replacement therapy in children. Complications associated with PD affect treatment success and sustainability. The aim of this study was to investigate the frequency of PD-related non-infectious complications and the predisposing factors.
Association between relative fat mass, uric acid, and insulin resistance in children with chronic kidney disease
Vasiliki Karava, John Dotis, Antonia Kondou, Athanasios Christoforidis, Vassilios Liakopoulos, Konstantina Tsioni, Konstantinos Kollios, Fotios Papachristou & Nikoleta Printza
doi : 10.1007/s00467-020-04716-y
Pediatric Nephrology volume 36, pages425–434(2021)
This cross-sectional study investigates the association between insulin resistance (IR) and serum uric acid (sUA) and relative fat (RFM) and lean mass (RLM) profiles in children with chronic kidney disease (CKD).
Vitamin C overload may contribute to systemic oxalosis in children receiving dialysis
Sabina S. Kennedy, Ashley Perilloux, Renata C. Pereira, Garry Handelman, Katherine Wesseling-Perry & Isidro B. Salusky
doi : 10.1007/s00467-020-04702-4
Pediatric Nephrology volume 36, pages435–441(2021)
Malnutrition and anorexia are common in children with chronic kidney disease (CKD) and gastrostomy tubes (GT) as well as nasogastric tubes (NGT) have been recommended to maximize nutritional support. The optimal requirement of vitamin C in children with CKD remains to be defined but oxalate is a breakdown product of vitamin C. Elevated vitamin C intake and bone oxalate were identified in two formula-fed dialyzed children with negative genetic testing for primary hyperoxaluria.
Native vs. active vitamin D in children with chronic kidney disease: a cross-over study
Happy Sawires, Fatina Fadel, Ahmed Hussein & Rasha Helmy
doi : 10.1007/s00467-020-04721-1
Pediatric Nephrology volume 36, pages443–450(2021)
The rationale for the prescription of vitamin D analogues in patients with chronic kidney disease (CKD) is still a matter of debate. We aimed to compare native vs. active forms of vitamin D on pre-dialysis children with CKD and evaluate effects on calcium (Ca), phosphorus (P), and parathyroid hormone (PTH).
Association of early hypotension in pediatric sepsis with development of new or persistent acute kidney injury
Julie C. Fitzgerald, Michelle E. Ross, Neal J. Thomas, Scott L. Weiss, Fran Balamuth, Marianne Chilutti, Robert W. Grundmeier & Amanda Hyre Anderson
doi : 10.1007/s00467-020-04704-2
Pediatric Nephrology volume 36, pages451–461(2021)
To determine how hypotension in the first 48 h of sepsis management impacts acute kidney injury (AKI) development and persistence.
Different approaches to long-term treatment of aHUS due to MCP mutations: a multicenter analysis
Verena Kl?mbt, Charlotte Gimpel, Martin Bald, Christopher Gerken, Heiko Billing, Sebastian Loos, Matthias Hansen, Jens K?nig, Tobias Vinke, Carmen Montoya, B?rbel Lange Sperandio, Martin Kirschstein, Imke Hennies, Martin Pohl & Karsten H?ffner
doi : 10.1007/s00467-020-04714-0
Pediatric Nephrology volume 36, pages463–471(2021)
Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening microangiopathy, frequently causing kidney failure. Inhibition of the terminal complement complex with eculizumab is the only licensed treatment but mostly requires long-term administration and risks severe side effects. The underlying genetic cause of aHUS is thought to influence the severity of initial and recurring episodes, with milder courses in patients with mutations in membrane cofactor protein (MCP).
Erythropoietin-stimulating agent-resistant vitamin B6 deficiency anemia in a pediatric patient on hemodialysis
Kristie Searcy, Sarah Rainwater, Majed Jeroudi & Radhakrishna Baliga
doi : 10.1007/s00467-020-04810-1
Pediatric Nephrology volume 36, pages473–476(2021)
Vitamin B6 is a rate-limiting coenzyme that plays an important role in the biosynthesis of heme and the incorporation of iron into protoporphyrin. Its deficiency is often seen in chronic kidney disease (CKD), particularly those requiring dialysis and following administration of erythropoietin-stimulating agent (ESA).
Correction to: Multiple office blood pressure measurement: a novel approach to overcome the weak cornerstone of blood pressure measurement in children. Data from the SPA project
Gianluigi Ardissino, Silvia Ghiglia, Patrizia Salice, Michela Perrone, Sandra Piantanida, Francesco L. De Luca, Silvia Di Michele, Lucia Filippucci, Elena R. A. Dardi, Tiziana Bollani, Antonella Mezzopane, Bertrand Tchana, Sebastiano A. G. Lava & SPA Project investigators
doi : 10.1007/s00467-020-04801-2
Pediatric Nephrology volume 36, page477(2021)
