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Pathophysiological aspects of the thick ascending limb and novel genetic defects: HELIX syndrome and transient antenatal Bartter syndrome
Rosa Vargas-Poussou
doi : 10.1007/s00467-021-05019-6
Pediatric Nephrology volume 37, pages239–252 (2022)
The thick ascending limb plays a central role in human kidney physiology, participating in sodium reabsorption, urine concentrating mechanisms, calcium and magnesium homeostasis, bicarbonate and ammonium homeostasis, and uromodulin synthesis. This review aims to illustrate the importance of these roles from a pathophysiological point of view by describing the interactions of the key proteins of this segment and by discussing how recently identified and long-known hereditary diseases affect this segment. The descriptions of two recently described salt-losing tubulopathies, transient antenatal Bartter syndrome and HELIX syndrome, which are caused by mutations in MAGED2 and CLDN10 genes, respectively, highlight the role of new players in the modulation of sodium reabsorption the thick ascending limb.
Population-based studies reveal an additive role of type IV collagen variants in hematuria and albuminuria
Moumita Barua & Andrew D. Paterson
doi : 10.1007/s00467-021-04934-y
Pediatric Nephrology volume 37, pages253–262 (2022)
Specific variants in genes that encode the ?3?4?5 chains of type IV collagen cause Alport syndrome (AS), which encompass a clinical spectrum from isolated hematuria to multisystem disease affecting sight, hearing and kidney function. The commonest form is X-linked Alport syndrome (XLAS; COL4A5) with autosomal AS (COL4A3 and COL4A4) comprising a minority of cases. While historic data estimates the frequency of AS at 1:5000–10,000, recent population-based genetic studies suggest the prevalence is considerably higher. Genome-wide association studies (GWAS) have been performed in the Icelandic (deCODE) and UK (UK Biobank) populations, demonstrating an association of type IV collagen gene variants with AS relevant kidney traits. In the Icelandic population, 1 in 600 carries a 2.5-kb COL4A3 coding deletion or a COL4A3 missense variant (rs200287952[A], Gly695Arg), both of which are strongly associated with hematuria and albuminuria (P values?=?1.9?×?10?5 to 2.5?×?10?20). In the UK Biobank, COL4A4 rs35138315 (Ser969X; carrier frequency 0.13%) is strongly associated with both hematuria and albuminuria (P?=?1.5?×?10?73). Thus, the frequency for autosomal AS is 5–16 times higher than the historic prevalence of all forms of the disorder. Furthermore, COL4A4 rs3518315 (Ser969X) is also a reported founder mutation in families with autosomal dominant focal and segmental glomerulosclerosis and autosomal recessive forms of AS. This supports an additive mode of inheritance for specific variants, wherein a number of copies of a mutation influence disease severity in a cumulative fashion. These studies did not include the X chromosome, excluding analysis of COL4A5, which represents an area for future study.
Dialysis disequilibrium syndrome (DDS) in pediatric patients on dialysis: systematic review and clinical practice recommendations
Rupesh Raina, Andrew Davenport, Bradley Warady, Prabhav Vasistha, Sidharth Kumar Sethi, Ronith Chakraborty, Prajit Khooblall, Nirav Agarwal, Manan Vij, Franz Schaefer, Kunal Malhotra & Madhukar Misra
doi : 10.1007/s00467-021-05242-1
Pediatric Nephrology volume 37, pages263–274 (2022)
Dialysis disequilibrium syndrome (DDS) is a rare neurological complication, most commonly affecting patients undergoing new initiation of hemodialysis (HD), but can also be seen in patients receiving chronic dialysis who miss regular treatments, patients having acute kidney injury (AKI), and in those treated with continuous kidney replacement therapy (CKRT) or peritoneal dialysis (PD). Although the pathogenesis is not well understood, DDS is likely a result of multiple physiological abnormalities. In this systematic review, we provide a synopsis of the data available on DDS that allow for a clear picture of its pathogenesis, preventive measures, and focus on effective management strategies.
Acute kidney injury in premature and low birth weight neonates: a systematic review and meta-analysis
Yang Wu, Haoran Wang, Jiao Pei, Xiaoping Jiang & Jun Tang
doi : 10.1007/s00467-021-05251-0
Pediatric Nephrology volume 37, pages275–287 (2022)
Acute kidney injury (AKI) is common and it is associated with poor clinical outcomes in premature and low birth weight neonates. This systematic review and meta-analysis was performed to summarize the literature and evaluate the prevalence, risk factors, and mortality of premature and low birth weight neonates with AKI.
Genetic causes of neonatal and infantile hypercalcaemia
Caroline M. Gorvin
doi : 10.1007/s00467-021-05082-z
Pediatric Nephrology volume 37, pages289–301 (2022)
The causes of hypercalcaemia in the neonate and infant are varied, and often distinct from those in older children and adults. Hypercalcaemia presents clinically with a range of symptoms including failure to thrive, poor feeding, constipation, polyuria, irritability, lethargy, seizures and hypotonia. When hypercalcaemia is suspected, an accurate diagnosis will require an evaluation of potential causes (e.g. family history) and assessment for physical features (such as dysmorphology, or subcutaneous fat deposits), as well as biochemical measurements, including total and ionised serum calcium, serum phosphate, creatinine and albumin, intact parathyroid hormone (PTH), vitamin D metabolites and urinary calcium, phosphate and creatinine. The causes of neonatal hypercalcaemia can be classified into high or low PTH disorders. Disorders associated with high serum PTH include neonatal severe hyperparathyroidism, familial hypocalciuric hypercalcaemia and Jansen’s metaphyseal chondrodysplasia. Conditions associated with low serum PTH include idiopathic infantile hypercalcaemia, Williams-Beuren syndrome and inborn errors of metabolism, including hypophosphatasia. Maternal hypocalcaemia and dietary factors and several rare endocrine disorders can also influence neonatal serum calcium levels. This review will focus on the common causes of hypercalcaemia in neonates and young infants, considering maternal, dietary, and genetic causes of calcium dysregulation. The clinical presentation and treatment of patients with these disorders will be discussed.
Update on the treatment of steroid-sensitive nephrotic syndrome
Federica Zotta, Marina Vivarelli & Francesco Emma
doi : 10.1007/s00467-021-04983-3
Pediatric Nephrology volume 37, pages303–314 (2022)
Steroid-sensitive nephrotic syndrome (SSNS) is a rare condition that develops primarily in preadolescent children after the age of 1 year. Since the 1950s, oral corticosteroids have been the mainstay of treatment of all children presenting with nephrotic syndrome, with most patients responding within 4 weeks to an oral course of prednisone (PDN). However, corticosteroids have important side effects and 60–80 % of patients relapse, developing frequently relapsing or steroid-dependent forms. For these reasons, many patients require second-line steroid-sparing immunosuppressive medications that have considerably improved relapse-free survival, while avoiding many PDN-related toxicities. Since most patients will eventually heal from their disease with a normal kidney function, the morbidity of SSNS is primarily related to side effects of drugs that are used to maintain prolonged remission. Therefore, treatment is essentially based on balancing the use of different drugs to achieve permanent remission with the lowest cumulative number of side effects. Treatment choice is based on the severity of SSNS, on patient age, and on drug tolerability. This review provides an update of currently available therapeutic strategies for SSNS.
Role of therapeutic apheresis in the treatment of pediatric kidney diseases
Shweta Shah, Catherine Joseph & Poyyapakkam Srivaths
doi : 10.1007/s00467-021-05093-w
Pediatric Nephrology volume 37, pages315–328 (2022)
Therapeutic apheresis utilizes apheresis procedures in the treatment of a variety of conditions including kidney disease. Therapeutic plasma exchange (TPE) is the most common modality employed with the rationale of rapid reduction of a pathogenic substance distributed primarily in the intravascular compartment; however other techniques which adsorb such pathogenic substances or alter the immune profile have been utilized in diseases affecting native and transplanted kidneys. This article discusses the modalities and technical details of therapeutic apheresis and summarizes its role in individual diseases affecting the kidney. Complications related to pediatric apheresis procedures and specifically related to apheresis in kidney disease are also discussed. Though therapeutic apheresis modalities are employed frequently in children with kidney disease, most experiences are extrapolated from adult studies. International and national registries need to be established to elucidate the role of apheresis modalities in children with kidney disease.
A rare cause of arterial hypertension in a child with developmental delay: Questions
Cemile Pehlivanoglu, Hulya Maras Genc & Sevinc Kal?n
doi : 10.1007/s00467-021-05299-y
Pediatric Nephrology volume 37, pages329–331 (2022)
A rare cause of arterial hypertension in a child with developmental delay: Answers
Cemile Pehlivanoglu, Hulya Maras Genc & Sevinc Kal?n
doi : 10.1007/s00467-021-05302-6
Pediatric Nephrology volume 37, pages333–336 (2022)
Macroscopic hematuria, facing an uncommon disease: Questions
Hulya Nalcac?oglu, Demet Tekcan, Bilge Can Meydan, Hulya Gozde Onal & Ozlem Aydog
doi : 10.1007/s00467-021-05277-4
Pediatric Nephrology volume 37, pages337–338 (2022)
Macroscopic hematuria, facing an uncommon disease: Answers
Hulya Nalcacioglu, Demet Tekcan, Bilge Can Meydan, Hulya Gozde Onal & Ozlem Aydog
doi : 10.1007/s00467-021-05285-4
Pediatric Nephrology volume 37, pages339–343 (2022)
Kidney disease in a child with familial Mediterranean fever: Questions
Hakan Kisaoglu, Ozge Baba, Sevdegul Ayd?n Mungan & Mukaddes Kalyoncu
doi : 10.1007/s00467-021-05315-1
Pediatric Nephrology volume 37, pages345–346 (2022)
Kidney disease in a child with familial Mediterranean fever: Answers
Hakan Kisaoglu, Ozge Baba, Sevdegul Aydin Mungan & Mukaddes Kalyoncu
doi : 10.1007/s00467-021-05317-z
Pediatric Nephrology volume 37, pages347–349 (2022)
What is the cause of kidney dysfunction in a newborn with trisomy 21? Questions
Katsuo Tao, Naoya Morisada & Midori Awazu
doi : 10.1007/s00467-021-05275-6
Pediatric Nephrology volume 37, pages351–352 (2022)
What is the cause of kidney dysfunction in a newborn with trisomy 21? Answers
Katsuo Tao, Naoya Morisada & Midori Awazu
doi : 10.1007/s00467-021-05284-5
Pediatric Nephrology volume 37, pages353–355 (2022)
Anti-rituximab antibodies in pediatric steroid-dependent nephrotic syndrome
Quentin Bertrand, Sabine Mignot, Theresa Kwon, Anne Couderc, Anne Maisin, Alexandra Cambier, Véronique Baudouin, Marine Peyneau, Georges Deschênes, Julien Hogan & Claire Dossier
doi : 10.1007/s00467-021-05069-w
Pediatric Nephrology volume 37, pages357–365 (2022)
Rituximab is a chimeric anti-CD20 monoclonal antibody that induces sustained remission in children with steroid-dependent nephrotic syndrome. However, there is no consensus on the optimal regimen and monitoring of rituximab. In other autoimmune diseases, anti-rituximab antibodies (ARA) have been reported in 10–40% of patients, but their clinical relevance remains unclear. In nephrotic syndrome, data are scarce.
Clinical manifestations, prognosis, and treat-to-target assessment of pediatric lupus nephritis
Shiyuan Qiu, Hengci Zhang, Sijie Yu, Qin Yang, Gaofu Zhang, Haiping Yang, Qiu Li & Mo Wang
doi : 10.1007/s00467-021-05164-y
Pediatric Nephrology volume 37, pages367–376 (2022)
Pediatric lupus nephritis (pLN) is one of the most refractory secondary kidney diseases in childhood. The treat-to-target (T2T) strategy has become the standard treatment for systemic lupus erythematosus (SLE). This study reviewed clinical features, overall remission status, and factors affecting prognosis, to guide pLN management according to T2T strategy.
Belimumab for the treatment of children with frequently relapsing nephrotic syndrome: the BELNEPH study
Marina Vivarelli, Manuela Colucci, Antonio Gargiulo, Chiara Bettini, Anna Lo Russo & Francesco Emma
doi : 10.1007/s00467-021-05175-9
Pediatric Nephrology volume 37, pages377–383 (2022)
Effectiveness of rituximab in pediatric idiopathic nephrotic syndrome suggests that B cells play a pathogenic role. We tested safety and efficacy of the B-cell-modulating agent belimumab in frequently relapsing nephrotic syndrome (FRNS).
Novel urine biomarkers to distinguish UTI from culture-negative pyuria
Elaise B Hill, Joshua R Watson, Daniel M Cohen, David Kline, Andrew L Schwaderer & John D Spencer
doi : 10.1007/s00467-021-05202-9
Pediatric Nephrology volume 37, pages385–391 (2022)
Emergency departments (EDs) often rely on urinalysis (UA) to rapidly identify urinary tract infections (UTIs) in children. However, the suboptimal test characteristics of UA can lead to false-positive results. Novel urinary biomarkers may increase the diagnostic precision of UA. In this study, we compared the concentrations of 6 pre-selected proteins: BH3 interacting domain death agonist (BID), B-cell lymphoma 6 protein, ras GTPase-activating protein 1, cathepsin S (CTSS), 3-hydroxyanthranilate 3,4-dioxygenase, and transgelin-2.
Kidney disease profile and encountered problems during follow-up in Syrian refugee children: a multicenter retrospective study
Ayse Balat, Beltinge Demircioglu Kilic, Bagdagul Aksu, Mehtap Akbalik Kara, Mithat Buyukcelik, Ayse Agbas, Fehime Kara Eroglu, Tulin Gungor, Demet Alaygut, Nurdan Yildiz, Funda Bastug, Bahriye Atmis, Engin Melek, Midhat Elmaci, Sebahat Tulpar, Cemile Pehlivanoglu, Serra Surmeli Doven, Elif Comak, Yilmaz Tabel, Atilla Gemici, Berfin Uysal, Gamze Seval Ozzorlar, Nuran Kuçuk, Ali Delibas, Gul Ozcelik, Nilufer Goknar, Ismail Dursun, Pelin Ertan, Ipek Akil Ozunan & Ferah Sonmez
doi : 10.1007/s00467-021-05046-3
Pediatric Nephrology volume 37, pages393–402 (2022)
Children are one of the most vulnerable groups in conflict zones, especially those with chronic diseases. This study aimed to investigate kidney disease profiles and problems during follow-up in a population of Syrian refugee children residing in Turkey.
The impact of a needs-oriented dental prophylaxis program on bacteremia after toothbrushing and systemic inflammation in children, adolescents, and young adults with chronic kidney disease
Karolin Höfer, Anna Turnowsky, Rasmus Ehren, Christina Taylan, Georg Plum, Hanna Witte, Michael J. Noack & Lutz T. Weber
doi : 10.1007/s00467-021-05153-1
Pediatric Nephrology volume 37, pages403–414 (2022)
Chronic kidney disease (CKD) still leads to high mortality rates, mainly due to cardiovascular disease. One important influencing factor is persisting low-grade chronic inflammation partly maintained by gingivitis that favors transient bacteremia during daily activities such as toothbrushing.
Randomized trial of two maintenance doses of vitamin D in children with chronic kidney disease
Shahid Nadeem, Vin Tangpricha, Thomas R. Ziegler, James E. Rhodes, Traci Leong, Yijin Xiang & Larry A. Greenbaum
doi : 10.1007/s00467-021-05228-z
Pediatric Nephrology volume 37, pages415–422 (2022)
Correction of nutritional vitamin deficiency is recommended in children with chronic kidney disease (CKD). The optimal daily dose of vitamin D to achieve or maintain vitamin D sufficiency is unknown.
Cystatin C relates to metabolism in healthy, pubertal adolescents
Niels Ziegelasch, Mandy Vogel, Antje Körner, Eva Koch, Anne Jurkutat, Uta Ceglarek, Katalin Dittrich & Wieland Kiess
doi : 10.1007/s00467-021-05209-2
Pediatric Nephrology volume 37, pages423–432 (2022)
The cystatin C (CysC) serum level is a marker of glomerular filtration rate and depends on age, gender, and pubertal stage. We hypothesize that CysC might overall reflect energy homeostasis and be regulated by components of the endocrine system and metabolites in pubertal adolescents.
The effect of continuous venovenous hemodiafiltration on amino acid delivery, clearance, and removal in children
Richard P Lion, Molly R Vega, E O’Brien Smith, Sridevi Devaraj, Michael C Braun, Nathan S Bryan, Moreshwar S Desai, Jorge A Coss-Bu, Talat Alp Ikizler & Ayse Akcan Arikan
doi : 10.1007/s00467-021-05162-0
Pediatric Nephrology volume 37, pages433–441 (2022)
In critically ill children with acute kidney injury (AKI), continuous kidney replacement therapy (CKRT) enables nutrition provision. The magnitude of amino acid loss during continuous venovenous hemodiafiltration (CVVHDF) is unknown and needs accurate quantification. We investigated the mass removal and clearance of amino acids in pediatric CVVHDF.
Severe hospital-acquired hyponatremia in acutely ill children receiving moderately hypotonic fluids
Saara Lehtiranta, Minna Honkila, Merja Kallio, Kimmo Halt, Niko Paalanne, Tytti Pokka & Terhi Tapiainen
doi : 10.1007/s00467-021-05227-0
Pediatric Nephrology volume 37, pages443–448 (2022)
Hypotonic fluids have been associated with hospital-acquired hyponatremia. The incidence of life-threatening severe hyponatremia associated with hypotonic fluids has not been evaluated.
Immunologic response of mRNA SARS-CoV-2 vaccination in adolescent kidney transplant recipients
Clarkson Crane, Erin Phebus & Elizabeth Ingulli
doi : 10.1007/s00467-021-05256-9
Pediatric Nephrology volume 37, pages449–453 (2022)
In the general population, mRNA SARS-CoV-2 vaccines are highly efficacious. Early reports suggest a diminished antibody response in immunosuppressed adult solid organ transplant (SOT) patients, but this has not been reported in pediatrics.
Circulating plasmablasts in children with steroid-sensitive nephrotic syndrome
Federica Zotta, Marina Vivarelli, Rita Carsetti, Simona Cascioli, Francesco Emma & Manuela Colucci
doi : 10.1007/s00467-021-05273-8
Pediatric Nephrology volume 37, pages455–459 (2022)
The therapeutic efficacy of B cell–depleting anti-CD20 treatment in both pediatric and adult steroid-sensitive nephrotic syndromes (SSNS) suggests that B cells play a pathogenic role in the disease. In adults with minimal change disease (MCD), only circulating plasmablasts are increased during the active phase of the disease, among B cell subsets. These cells have not been studied yet in children with SSNS.
IgA nephropathy presenting as rapidly progressive glomerulonephritis following first dose of COVID-19 vaccine
Olivier Niel & Cosmin Florescu
doi : 10.1007/s00467-021-05351-x
Pediatric Nephrology volume 37, pages461–462 (2022)
A novel prognostic index in hemolytic uremic syndrome related to Shiga toxin–producing Escherichia coli
Luciana Meni Battaglia & Alejandro Balestracci
doi : 10.1007/s00467-021-05355-7
Pediatric Nephrology volume 37, pages463–464 (2022)
Response to Battaglia and Balestracci
Sebastian Loos
doi : 10.1007/s00467-021-05356-6
Pediatric Nephrology volume 37, page465 (2022)
Correction to: Severe hospital?acquired hyponatremia in acutely ill children receiving moderately hypotonic fluids
Saara Lehtiranta, Minna Honkila, Merja Kallio, Kimmo Halt, Niko Paalanne, Tytti Pokka & Terhi Tapiainen
doi : 10.1007/s00467-021-05268-5
Pediatric Nephrology volume 37, page467 (2022)
