Raphael Wilson, Neije Mukherjee-Roy, Jyothsna Gattineni
Phosphate is essential for numerous biological processes, and serum levels are tightly regulated to accomplish these functions. The regulation of serum phosphate in a narrow physiological range is a well-orchestrated process and involves the gastrointestinal (GI) tract, bone, kidneys, and several hormones, namely, parathyroid hormone, fibroblast growth factor 23 (FGF23), and 1,25-dihydroxyvitamin D (1,25 Vitamin D). Although primarily synthesized in the bone, FGF23, an endocrine FGF, acts on the kidney to regulate phosphate and Vitamin D homeostasis by causing phosphaturia and reduced levels of 1,25 Vitamin D...
June 14, 2024: Pediatric Nephrology
Jolanta Sołtysiak, Bogda Skowrońska, Katarzyna Maćkowiak-Lewandowicz, Andrzej Blumczyński, Kaczmarek Elżbieta, Danuta Ostalska-Nowicka, Jacek Zachwieja
BACKGROUND: This study aimed to evaluate the blood pressure (BP) status, including arterial stiffness parameters, hemodynamic indicators, circadian profile, and its association with albuminuria in adolescents with type 1 diabetes mellitus (DM1). METHODS: The analysis included 46 patients, with diabetes duration of 7.38 ± 3.48 years. Ambulatory blood pressure monitoring (ABPM) was conducted using an oscillometric device, the Mobil-O-Graph, which is a Pulse Wave Analysis Monitor...
June 12, 2024: Pediatric Nephrology
Eva Svabova, Jakub Zieg, Martina Sukova, Eva Flachsova, Martin Kment, Vladimir Tesar
There is no specific treatment for proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID), a disease that is very rare in the pediatric population. We report the case of a 15-year-old boy who presented with mildly reduced kidney function and nephrotic syndrome. Kidney biopsy revealed PGNMID with monoclonal deposits of IgG3 with kappa light chain restriction. Flow cytometry showed a significant CD38 plasma cell population in the peripheral blood in the absence of other signs of hematological malignancy...
June 11, 2024: Pediatric Nephrology
Soumya Reddy, Nicole Scholes-Robertson, John Michael Raj, Priya Pais
BACKGROUND: Caregivers of children with chronic kidney disease (CKD) in low resource settings must provide complex medical care at home while being burdened by treatment costs often paid out-of-pocket. We hypothesize that caregiver burden in our low resource setting is greater than reported from high income countries and is associated with frequent catastrophic healthcare expenditure (CHE). METHODS: We conducted a mixed-methods study of primary caregivers of children with advanced CKD (stage 3b-5) in our private-sector referral hospital in a low resource setting...
June 10, 2024: Pediatric Nephrology
Malina Brügelmann, Sophia Müller, Alina V Bohlen, Katharina Hohenfellner, Anja Büscher, Markus J Kemper, Kerstin Fröde, Nele Kanzelmeyer, Jun Oh, Heiko Billing, Jutta Gellermann, Dominik Müller, Lutz T Weber, Birgit Acham-Roschitz, Klaus Arbeiter, Burkhard Tönshoff, Martina Hagenberg, Mislav S Žebec, Dieter Haffner, Miroslav Zivicnjak
BACKGROUND: Infantile nephropathic cystinosis (INC) is a rare lysosomal storage disorder, mostly and often firstly affecting the kidneys, together with impaired disharmonious growth and rickets, eventually resulting in progressive chronic kidney disease (CKD). With the introduction of cysteamine therapy, most pediatric patients reach adulthood with no need for kidney replacement therapy. Still, detailed changes in INC patients' clinical and morphological presentation over the past decades have not yet been thoroughly investigated...
June 8, 2024: Pediatric Nephrology
Kanza Baqai, Jennifer A Bassetti, Arzu Kovanlikaya, Surya V Seshan, Oleh Akchurin
Fanconi-Bickel syndrome (FBS) is a rare genetic disorder of carbohydrate metabolism due to pathogenic variants in SLC2A2, a gene encoding glucose transporter 2 (GLUT2), which leads to accumulation of glycogen in the kidney and liver. While consequential complex proximal tubular dysfunction is well acknowledged in the literature, long-term trajectories of kidney function in patients with FBS have not been well characterized, and kidney biopsy is performed infrequently. Here, we report on a patient with FBS followed from infancy through young adulthood who presented early on with hypercalciuria, phosphaturia, and hypophosphatemia, complicated by chronic kidney disease development during childhood...
June 7, 2024: Pediatric Nephrology
Shrea Goswami, Amy E Hanson, Evan Rajadhyaksha, Pankaj P Dangle, Andrew L Schwaderer
Tumor lysis syndrome (TLS) is a life-threatening metabolic disorder caused by massive tumor lysis. Allopurinol, a xanthine oxidase inhibitor, is initiated during chemotherapy to prevent hyperuricemia and subsequent acute kidney injury (AKI). We report two cases of xanthine nephrolithiasis during TLS in newly diagnosed hematologic malignancy patients receiving prophylactic allopurinol. Allopurinol use likely promoted xanthine crystallization, stone formation, and AKI.
June 6, 2024: Pediatric Nephrology
Suchismita Saha, Rajiv Sinha
An 11-year-old male child who presented with increased frequency of urination, thirst and feeling of incomplete void was initially diagnosed with diabetes mellitus (DM) based on elevated blood sugar. Polyuria and polydipsia were confirmed even after normalisation of blood sugar. A standardised water deprivation test showed presence of central diabetes insipidus (DI) and patient was started on desmopressin. Presence of DM and DI led to suspicion of DIDMOAD/Wolfram syndrome and ophthalmic examination confirmed bilateral optic atrophy...
June 6, 2024: Pediatric Nephrology
Fatina I Fadel, Doaa M Salah, Mohamed A Abdel Mawla, Eman Galal, Shaimaa Sayed
BACKGROUND: Accurate volume status assessment and dry weight achievement are the most challenging goals for a nephrologist. We aimed to evaluate the role of ultrasonographic parameters including lung ultrasound and inferior vena cava (IVC) measurements as practical methods of volume status assessment in children on hemodialysis by comparing them with established techniques, such as clinical evaluation and bioimpedance spectroscopy. METHODS: A prospective cross-sectional study compared pre- and post-dialysis volume status using bioimpedance spectroscopy (BIS) parameters and clinical data with ultrasonographic lung B-lines and IVC parameters in children on regular hemodialysis...
June 6, 2024: Pediatric Nephrology
Tugba Erem, Asli Kavaz Tufan, Omer Kilic, Aysun Caltik Yilmaz, Yalcın Kara, Mahmut Can Kizil, Meltem Dinleyici, Nuran Cetin, Mucahit Kaya, Ener Cagri Dinleyici
BACKGROUND: Patients with nephrotic syndrome (NS) are at a higher risk of developing invasive pneumococcal disease (IPD). Pneumococcal carriage studies are helpful tools for detecting potentially infectious serotypes and guiding immunization efforts. Pneumococcal nasopharyngeal colonization is common, and IPD can easily occur in an immunosuppressed state. Limited information is available regarding the frequency of pneumococcal carriage in individuals with NS. The aim of this study was to evaluate pneumococcal carriage and serotype distribution in children with NS...
June 5, 2024: Pediatric Nephrology
Shunsuke Yokota, Koichi Kamei, Shuichiro Fujinaga, Riku Hamada, Aya Inaba, Kentaro Nishi, Mai Sato, Masao Ogura, Koji Sakuraya, Shuichi Ito
BACKGROUND: The efficacy of rituximab in steroid-resistant nephrotic syndrome (SRNS) is controversial. We previously reported that rituximab in combination with methylprednisolone pulse therapy (MPT) and immunosuppressants was associated with favorable outcomes. We determined risk factors for poor response following rituximab treatment, which remains unknown. METHODS: This retrospective study included 45 patients with childhood-onset SRNS treated with rituximab across four pediatric kidney facilities...
June 4, 2024: Pediatric Nephrology
Alexandra Alvarez, Ashley Montgomery, Nhu Thao Nguyen Galván, Eileen D Brewer, Abbas Rana
No abstract text is available yet for this article.
June 4, 2024: Pediatric Nephrology
Andrew Limavady, Matko Marlais
BACKGROUND: Tuberous sclerosis (TSC)-associated kidney disease is a leading cause of mortality in adults with TSC. This study aimed to understand TSC features in children, particularly kidney involvement, to inform clinical care for this specific group. METHODS: This retrospective cohort study included all paediatric (< 19 years) TSC cases at a large tertiary paediatric nephrology centre. Relevant data were collected from patients' records, statistical analyses were performed to identify associations between variables, survival probabilities were estimated with Kaplan‒Meier curves, and log-rank tests were conducted to assess survival differences among genetic mutations...
June 4, 2024: Pediatric Nephrology
Kirsten Kusumi, Muhammad S Islam, Himanshi Banker, Fayez F Safadi, Rupesh Raina
The gut microbiome is made up of trillions of bacteria, viruses, archaea, and microbes that play a significant role in the maintenance of normal physiology in humans. Recent research has highlighted the effects of the microbiome and its dysbiosis in the pathogenesis and maintenance of kidney disease, especially chronic kidney disease (CKD) and its associated cardiovascular disease. While studies have addressed the kidney-microbiome axis in adults, how dysbiosis may uniquely impact pediatric kidney disease patients is not well-established...
June 3, 2024: Pediatric Nephrology
Ruchi Gupta Mahajan, Michael Evans, Sarah Kizilbash
BACKGROUND: Heart transplant recipients frequently require kidney transplantation for concomitant advanced chronic kidney disease. Data on simultaneous (heart and kidney transplants performed simultaneously) versus sequential (heart transplant performed before kidney) heart-kidney transplants in children are limited. Herein, we compare kidney transplant outcomes between the two groups. METHOD: We used the Scientific Registry of Transplant Recipients to identify all pediatric (age <21 years) heart transplant recipients who also received a kidney transplant within 10 years of the heart transplant...
June 1, 2024: Pediatric Nephrology
Demet Öztürk, Aynur Ayşe Karaduman, Türkan Akbayrak
BACKGROUND: The purpose was to investigate the frequency of lower urinary tract symptoms (LUTS) and lower urinary tract dysfunction (LUTD) in Duchenne muscular dystrophy (DMD) and the relationship between these symptoms and independence and quality of life (QoL). METHODS: The cross-sectional study included children aged 5-18 years and diagnosed with DMD and their families. Data were collected using the Dysfunctional Voiding and Incontinence Scoring System (DVISS), the Barthel Index, and the Pediatric Quality of Life™ 3...
June 1, 2024: Pediatric Nephrology
Sushmita Banerjee, Jayati Sengupta, Rajiv Sinha, Suparna Chatterjee, Subhankar Sarkar, Shakil Akhtar, Rana Saha, Amitava Pahari
BACKGROUND: Levamisole is less expensive and has a better toxicity profile compared to other steroid sparing agents used in nephrotic syndrome. It has a plasma half-life of 2.0 to 5.6 hours, but is conventionally administered on alternate days. We aimed to assess whether daily levamisole is safe and more effective than standard alternate-day therapy in maintaining remission in children with frequently relapsing or steroid-dependent nephrotic syndrome (FR/SDNS). METHODS: An open-label randomized controlled trial was conducted in children with FR/SDNS...
June 1, 2024: Pediatric Nephrology
Chiara De Mutiis, Scott E Wenderfer, Biswanath Basu, Arvind Bagga, Alvaro Orjuela, Tanmoy Sar, Amita Aggarwal, Avinash Jain, Olivia Boyer, Hui-Kim Yap, Shuichi Ito, Ai Ohnishi, Naomi Iwata, Ozgur Kasapcopur, Audrey Laurent, Eugene Yu-Hin Chan, Antonio Mastrangelo, Masao Ogura, Yuko Shima, Pornpimol Rianthavorn, Clovis A Silva, Vitor Trindade, Kjell Tullus
BACKGROUND: Lupus nephritis (LN) is a very severe manifestation of lupus. There is no consensus on which treatment goals should be achieved to protect kidney function in children with LN. METHODS: We retrospectively analyzed trends of commonly used laboratory biomarkers of 428 patients (≤ 18 years old) with biopsy-proven LN class ≥ III. We compared data of patients who developed stable kidney remission from 6 to 24 months with those who did not...
May 28, 2024: Pediatric Nephrology
Klara Kristin Brüning, Elena Pelivan, Marie-Christine Heinrich, Philip Bufler, Angela Kaindl, Julia Thumfart
We present a case of lamotrigine-triggered DRESS (drug reaction with eosinophilia and systemic symptoms) syndrome with acute kidney injury stage 3. A 17-year-old girl with known epilepsy treated with lamotrigine presented with acute kidney injury as well as skin eruption, fever, and apathy. Extended diagnostics, considering infectious and autoimmune diseases, remained unremarkable. Lamotrigine blood levels were within the target range. Kidney biopsy showed acute interstitial nephritis with tubular necrosis...
May 27, 2024: Pediatric Nephrology
Junxin Li, Xida Yan, Zhigui Wu, Jing Shen, Yalin Li, Yueshui Zhao, Fukuan Du, Mingxing Li, Xu Wu, Yu Chen, Zhangang Xiao, Shurong Wang
Macrophages, crucial components of the human immune system, can be polarized into M1/M2 phenotypes, each with distinct functions and roles. Macrophage polarization has been reported to be significantly involved in the inflammation and fibrosis observed in kidney injury. MicroRNA (miRNA), a type of short RNA lacking protein-coding function, can inhibit specific mRNA by partially binding to its target mRNA. The intricate association between miRNAs and macrophages has been attracting increasing interest in recent years...
May 27, 2024: Pediatric Nephrology
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