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  1. Book ; Online ; E-Book: Pediatric kidney disease

    Schaefer, Franz / Greenbaum, Larry

    2023  

    Author's details Franz Schaefer, Larry A. Greenbaum editors
    Keywords Electronic books
    Language English
    Size 1 Online-Ressource (xii, 2072 Seiten), Illustrationen
    Edition Third edition
    Publisher Springer
    Publishing place Cham
    Publishing country Switzerland
    Document type Book ; Online ; E-Book
    Note Description based on publisher supplied metadata and other sources
    Remark Zugriff für angemeldete ZB MED-Nutzerinnen und -Nutzer
    HBZ-ID HT021878845
    ISBN 978-3-031-11665-0 ; 9783031116643 ; 3-031-11665-8 ; 303111664X
    Database ZB MED Catalogue: Medicine, Health, Nutrition, Environment, Agriculture

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  2. Article ; Online: Application of GFR estimating equations to children with normal, near-normal, or discordant GFR.

    Schwaderer, Andrew L / Maier, Paula / Greenbaum, Larry A / Furth, Susan L / Schwartz, George J

    Pediatric nephrology (Berlin, Germany)

    2023  Volume 38, Issue 12, Page(s) 4051–4059

    Abstract: Background: The objective was to determine the extent that eGFR formulas correspond to measured plasma iohexol clearance (iGFR) in children with normal or near normal kidney function, particularly how different eGFR formulas yield discordant results.: ...

    Abstract Background: The objective was to determine the extent that eGFR formulas correspond to measured plasma iohexol clearance (iGFR) in children with normal or near normal kidney function, particularly how different eGFR formulas yield discordant results.
    Methods: iGFR from 2 (iGFR-2pt) and 4 (iGFR-4pt) time points along with creatinine and/or cystatin C-based eGFR were measured in children with mild CKD, stages 1-2. eGFR was calculated using 6 equations: 3 under 25 (U25) formulas from the Chronic Kidney Disease in Children (CKiD) study, the full age-combined cystatin C (cysC) and creatinine spectrum (FAS-combined), the European Kidney Function Consortium (EKFC-creatinine) equation, and the Chronic Kidney Disease Epidemiology Collaboration (CKD-epi) cysC-based equation.
    Results: Twenty-nine children were included, of which 22 had discordant creatinine vs. cystatin C-based eGFR by ≥ 15mL/min/1.73 m
    Conclusion: The formulas that most closely approximated the measured GFR varied depending on the pattern of discordant eGFR results. Based on the results, we recommend using the CKiD U25-combined formula to screen for children with a low GFR. Either the CKiD U25-combined or FAS-combined would be recommended for changes in eGFR longitudinally. However, because all formulas were discordant from the iGFR-4pt in over a third of participants, further refinement of pediatric eGFR formulas is needed at the normal/near-normal range. A higher resolution version of the Graphical abstract is available as Supplementary information.
    MeSH term(s) Humans ; Child ; Glomerular Filtration Rate ; Cystatin C ; Creatinine ; Kidney Function Tests ; Renal Insufficiency, Chronic
    Chemical Substances Cystatin C ; Creatinine (AYI8EX34EU)
    Language English
    Publishing date 2023-07-07
    Publishing country Germany
    Document type Journal Article
    ZDB-ID 631932-4
    ISSN 1432-198X ; 0931-041X
    ISSN (online) 1432-198X
    ISSN 0931-041X
    DOI 10.1007/s00467-023-06045-2
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Atypical hemolytic uremic syndrome.

    Greenbaum, Larry A

    Advances in pediatrics

    2014  Volume 61, Issue 1, Page(s) 335–356

    MeSH term(s) Atypical Hemolytic Uremic Syndrome/diagnosis ; Atypical Hemolytic Uremic Syndrome/epidemiology ; Atypical Hemolytic Uremic Syndrome/therapy ; Child ; Disease Management ; Global Health ; Humans ; Morbidity/trends
    Language English
    Publishing date 2014-08
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 210524-x
    ISSN 1878-1926 ; 0065-3101
    ISSN (online) 1878-1926
    ISSN 0065-3101
    DOI 10.1016/j.yapd.2014.04.001
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Nephrotic Syndrome.

    Wang, Chia-Shi / Greenbaum, Larry A

    Pediatric clinics of North America

    2018  Volume 66, Issue 1, Page(s) 73–85

    Abstract: Nephrotic syndrome is characterized by edema, proteinuria, hypoalbuminemia, and hyperlipidemia. Minimal change disease, the most common cause in childhood, generally responds to corticosteroids, although most patients experience disease relapses. Focal ... ...

    Abstract Nephrotic syndrome is characterized by edema, proteinuria, hypoalbuminemia, and hyperlipidemia. Minimal change disease, the most common cause in childhood, generally responds to corticosteroids, although most patients experience disease relapses. Focal segmental glomerulosclerosis is usually resistant to corticosteroids and carries a significant risk of kidney failure, necessitating renal transplantation. Nephrotic syndrome may also be secondary to gene mutations and systemic diseases such as lupus. Clinical evaluation involves distinguishing primary and secondary causes and monitoring for disease complications, including blood clots and serious infections such as spontaneous bacterial peritonitis. Immunosuppressive medications are used to prevent relapses and treat corticosteroid-resistant disease.
    MeSH term(s) Adrenal Cortex Hormones/therapeutic use ; Child ; Diagnosis, Differential ; Humans ; Nephrotic Syndrome/diagnosis ; Nephrotic Syndrome/drug therapy ; Nephrotic Syndrome/etiology ; Risk Factors
    Chemical Substances Adrenal Cortex Hormones
    Language English
    Publishing date 2018-09-08
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 215711-1
    ISSN 1557-8240 ; 0031-3955
    ISSN (online) 1557-8240
    ISSN 0031-3955
    DOI 10.1016/j.pcl.2018.08.006
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Risk factors for renal scarring and clinical morbidity in children with high-grade and low-grade primary vesicoureteral reflux.

    Mathias, Sitarah / Greenbaum, Larry A / Shubha, A M / Raj, John A Michael / Das, Kanishka / Pais, Priya

    Journal of pediatric urology

    2022  Volume 18, Issue 2, Page(s) 225.e1–225.e8

    Abstract: Introduction: Primary vesicoureteral reflux (VUR) is associated with urinary tract infections (UTIs) and renal damage. However, the importance of early diagnosis of VUR has been questioned. Moreover, most studies have few patients with high-grade VUR. ... ...

    Abstract Introduction: Primary vesicoureteral reflux (VUR) is associated with urinary tract infections (UTIs) and renal damage. However, the importance of early diagnosis of VUR has been questioned. Moreover, most studies have few patients with high-grade VUR. Hence, we retrospectively analyzed a large cohort of patients with primary high-grade and low-grade VUR and assessed risk factors for renal damage and clinical morbidity.
    Material and methods: We included patients (<18 years) at diagnosis with low-grade (1-3) or high-grade (4-5) primary VUR and noted their clinical history and presence of hypertension, low eGFR (<60ml/in/1.73 m
    Results: Of 399 primary VUR patients, 255 (64%) had high-grade VUR. Indications for voiding cystourethrogram were recurrent UTI (38%), first UTI (28%) and antenatal hydronephrosis (17%). At diagnosis, 252 (65%) had renal scars (focal in 170 [44%], generalised in 82 [21%]), and 188 (47%) had reduced DRF. High-grade VUR patients were more likely than low-grade VUR patients to have renal scarring (75% vs. 49%, p < 0.01), low eGFR (23% vs. 13%, p = 0.04) and significant hypertension (26% vs. 13%, p = 0.02). High-grade VUR was associated with generalised scars (odds ratio [OR] 11, p < 0.001), focal scars (OR 3.1, p < 0.001) and reduced DRF (OR 2.3, p < 0.001) shown in the table. Male sex was a risk factor for generalised scars (OR 2.3, p = 0.005). Focal scars were associated with recurrent UTIs (OR = 1.8, p = 0.004) and reduced DRF (OR 1.4, p = 0.027). Patients with multiple focal scars were diagnosed at an older age (2 years [1,4] than those with single scars (1.5 years [1,4] or no scars (1 year [0, 3]), p = 0.04).
    Discussion: The prevalence of renal damage and clinical morbidity at VUR diagnosis was higher than other studies. High-grade VUR patients had a greater prevalence of renal damage, low eGFR and hypertension than low-grade VUR patients and was a risk factor for focal scars, generalised scars and reduced DRF. Focal scars were independently associated with recurrent UTI. Those with multiple scars were diagnosed later than those with single scars or no scars.
    Conclusions: High-grade VUR was associated with renal damage and clinical morbidity. Our study highlights the importance of diagnosing VUR early to identify patients who may warrant long-term follow-up and intervention to minimize morbidity.
    MeSH term(s) Child ; Cicatrix/complications ; Cicatrix/epidemiology ; Female ; Humans ; Hyperplasia ; Hypertension/complications ; Infant ; Male ; Morbidity ; Pregnancy ; Retrospective Studies ; Risk Factors ; Urinary Tract Infections/complications ; Urinary Tract Infections/epidemiology ; Vesico-Ureteral Reflux/complications ; Vesico-Ureteral Reflux/epidemiology
    Language English
    Publishing date 2022-01-08
    Publishing country England
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2237683-5
    ISSN 1873-4898 ; 1477-5131
    ISSN (online) 1873-4898
    ISSN 1477-5131
    DOI 10.1016/j.jpurol.2021.12.017
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Clinical Safety and Efficacy of Pegcetacoplan in a Phase 2 Study of Patients with C3 Glomerulopathy and Other Complement-Mediated Glomerular Diseases.

    Dixon, Bradley P / Greenbaum, Larry A / Huang, Liwei / Rajan, Sandeep / Ke, Chunlei / Zhang, Yiwei / Li, Li

    Kidney international reports

    2023  Volume 8, Issue 11, Page(s) 2284–2293

    Abstract: Introduction: Dysregulated complement activation is likely the primary driver of disease in C3 glomerulopathy (C3G) and contributes to other complement-mediated diseases, including immunoglobulin A nephropathy (IgAN), lupus nephritis (LN), and primary ... ...

    Abstract Introduction: Dysregulated complement activation is likely the primary driver of disease in C3 glomerulopathy (C3G) and contributes to other complement-mediated diseases, including immunoglobulin A nephropathy (IgAN), lupus nephritis (LN), and primary membranous nephropathy (PMN). No complement inhibitors are proven to halt disease progression in these diseases. Pegcetacoplan, a targeted C3 and C3b inhibitor, may mitigate complement-mediated kidney damage in C3G and other glomerular diseases in which complement may have a pathogenic role.
    Methods: This open-label, phase 2, 48-week study evaluated the preliminary efficacy and safety of subcutaneous pegcetacoplan for patients with complement-mediated glomerular diseases. The primary end point was proteinuria reduction, measured as 24-hour urine protein-to-creatinine ratio. Secondary end points included remission status, changes in estimated glomerular filtration rate (eGFR), and pharmacodynamic biomarkers. Treatment-emergent adverse events (TEAEs) were monitored.
    Results: Efficacy results for the C3G cohort are reported herein, along with safety results for the study population. In the C3G cohort, mean proteinuria reduction from baseline to week 48 was 50.9% in the intent-to-treat (ITT) population (
    Conclusion: Pegcetacoplan may provide therapeutic benefit for C3G and has a favorable safety profile across the 4 glomerular diseases studied.
    Language English
    Publishing date 2023-08-25
    Publishing country United States
    Document type Journal Article
    ISSN 2468-0249
    ISSN (online) 2468-0249
    DOI 10.1016/j.ekir.2023.08.033
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article ; Online: Design and Rationale of the APPELHUS Phase 3 Open-Label Study of Factor B Inhibitor Iptacopan for Atypical Hemolytic Uremic Syndrome.

    Kavanagh, David / Greenbaum, Larry A / Bagga, Arvind / Karki, Rajeshri G / Chen, Chien-Wei / Vasudevan, Sajita / Charney, Alan / Dahlke, Marion / Fakhouri, Fadi

    Kidney international reports

    2023  Volume 8, Issue 7, Page(s) 1332–1341

    Abstract: Introduction: Atypical hemolytic uremic syndrome (aHUS) is a rare, progressive, and life-threatening form of thrombotic microangiopathy (TMA) which is caused by dysregulation of the alternative complement pathway (AP). Complement inhibition is an ... ...

    Abstract Introduction: Atypical hemolytic uremic syndrome (aHUS) is a rare, progressive, and life-threatening form of thrombotic microangiopathy (TMA) which is caused by dysregulation of the alternative complement pathway (AP). Complement inhibition is an effective therapeutic strategy in aHUS, though current therapies require intravenous administration and increase the risk of infection by encapsulated organisms, including meningococcal infection. Further studies are required to define the optimal duration of existing therapies, and to identify new agents that are convenient for long-term administration. Iptacopan (LNP023) is an oral, first-in-class, highly potent, proximal AP inhibitor that specifically binds factor B (FB). In phase 2 studies of IgA nephropathy, paroxysmal nocturnal hemoglobinuria, and C3 glomerulopathy, iptacopan inhibited the AP, showed clinically relevant benefits, and was well tolerated. Iptacopan thus has the potential to become an effective and safe treatment for aHUS, with the convenience of oral administration.
    Methods: Alternative Pathway Phase III to Evaluate LNP023 in aHUS (APPELHUS; NCT04889430) is a multicenter, single-arm, open-label, phase 3 study to evaluate the efficacy and safety of iptacopan in patients (
    Conclusion: APPELHUS will determine if iptacopan is safe and efficacious in patients with aHUS.
    Language English
    Publishing date 2023-04-29
    Publishing country United States
    Document type Journal Article
    ISSN 2468-0249
    ISSN (online) 2468-0249
    DOI 10.1016/j.ekir.2023.04.029
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: Characterization of patients with aHUS and associated triggers or clinical conditions: A Global aHUS Registry analysis.

    Licht, Christoph / Al-Dakkak, Imad / Anokhina, Katerina / Isbel, Nicole / Frémeaux-Bacchi, Véronique / Gilbert, Rodney D / Greenbaum, Larry A / Ariceta, Gema / Ardissino, Gianluigi / Schaefer, Franz / Rondeau, Eric

    Nephrology (Carlton, Vic.)

    2024  

    Abstract: Introduction: Atypical haemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA) associated with complement dysregulation; aHUS may be associated with other 'triggers' or 'clinical conditions'. This study aimed to characterize ...

    Abstract Introduction: Atypical haemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA) associated with complement dysregulation; aHUS may be associated with other 'triggers' or 'clinical conditions'. This study aimed to characterize this patient population using data from the Global aHUS Registry, the largest collection of real-world data on patients with aHUS.
    Methods: Patients enrolled in the Global aHUS Registry between April 2012 and June 2021 and with recorded aHUS-associated triggers or clinical conditions prior/up to aHUS onset were analysed. aHUS was diagnosed by the treating physician. Data were classified by age at onset of aHUS (< or ≥18 years) and additionally by the presence/absence of identified pathogenic complement genetic variant(s) and/or anti-complement factor H (CFH) antibodies. Genetically/immunologically untested patients were excluded.
    Results: 1947 patients were enrolled in the Global aHUS Registry by June 2021, and 349 (17.9%) met inclusion criteria. 307/349 patients (88.0%) had a single associated trigger or clinical condition and were included in the primary analysis. Malignancy was most common (58/307, 18.9%), followed by pregnancy and acute infections (both 53/307, 17.3%). Patients with an associated trigger or clinical condition were generally more likely to be adults at aHUS onset.
    Conclusion: Our analysis suggests that aHUS-associated triggers or clinical conditions may be organized into clinically relevant categories, and their presence does not exclude the concurrent presence of pathogenic complement genetic variants and/or anti-CFH antibodies. Considering a diagnosis of aHUS with associated triggers or clinical conditions in patients presenting with TMA may allow faster and more appropriate treatment.
    Language English
    Publishing date 2024-04-11
    Publishing country Australia
    Document type Journal Article
    ZDB-ID 1303661-0
    ISSN 1440-1797 ; 1320-5358
    ISSN (online) 1440-1797
    ISSN 1320-5358
    DOI 10.1111/nep.14304
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Article ; Online: Restless Legs Syndrome in Chronic Kidney Disease: Is Iron or Inflammatory Status To Blame?

    Riar, Sandeep K / Greenbaum, Larry A / Bliwise, Donald L / Leu, Roberta M

    Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine

    2019  Volume 15, Issue 11, Page(s) 1629–1634

    Abstract: Study objectives: Restless legs syndrome (RLS) is increased in pediatric chronic kidney disease (CKD). In adults without CKD, central nervous system iron deficiency is involved in RLS pathogenesis and a low serum ferritin levels is consequently an ... ...

    Abstract Study objectives: Restless legs syndrome (RLS) is increased in pediatric chronic kidney disease (CKD). In adults without CKD, central nervous system iron deficiency is involved in RLS pathogenesis and a low serum ferritin levels is consequently an indication for initiation of iron therapy. However, children with CKD are at risk for iron deficiency and inflammation, which raises serum ferritin. We examined the role of iron deficiency and inflammation in RLS in pediatric CKD.
    Methods: This cross-sectional study examined RLS prevalence in three groups of pediatric patients with CKD: nontransplant, nondialysis CKD (estimated GFR < 60 mL/min/1.73 m²) (n = 27); renal transplant recipients (n = 65); and dialysis (n = 32). RLS was diagnosed using a validated questionnaire. Serum ferritin < 100 ng/mL or transferrin saturation < 20% defined iron deficiency. Serum high sensitivity C-reactive protein ≥ 1 mg/L defined inflammation.
    Results: Among 124 patients, RLS prevalence was 15.3%; this did not differ across groups. There was no significant difference in RLS prevalence between those with and without iron deficiency, defined by either reduced ferritin or transferrin. Median ferritin levels in patients with RLS tended to be higher than in those without RLS (51.2 versus 40.1 ng/mL; P = .08). Inflammation (elevated CRP) also did not differ significantly by RLS status (57.9% [with RLS] versus 41.2% [without RLS], P = .18).
    Conclusions: Neither ferritin nor inflammation differentiated pediatric patients with CKD with and without RLS. This study suggests that the factors mediating the pathogenesis and, potentially, treatment, of RLS in pediatric CKD may be different from non-CKD populations.
    MeSH term(s) Adolescent ; Anemia, Iron-Deficiency/complications ; C-Reactive Protein/analysis ; Cross-Sectional Studies ; Female ; Ferritins/blood ; Humans ; Inflammation/complications ; Kidney Transplantation/adverse effects ; Male ; Prevalence ; Renal Insufficiency, Chronic/complications ; Restless Legs Syndrome/epidemiology ; Restless Legs Syndrome/etiology ; Risk Factors ; Surveys and Questionnaires ; Transferrin/analysis
    Chemical Substances Transferrin ; C-Reactive Protein (9007-41-4) ; Ferritins (9007-73-2)
    Language English
    Publishing date 2019-11-14
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2397213-0
    ISSN 1550-9397 ; 1550-9389
    ISSN (online) 1550-9397
    ISSN 1550-9389
    DOI 10.5664/jcsm.8028
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article ; Online: Renal and urologic abnormalities in the perinatal period.

    Rheault, Michelle N / Greenbaum, Larry A

    Clinics in perinatology

    2014  Volume 41, Issue 3, Page(s) xix–xx

    MeSH term(s) Female ; Global Health ; Humans ; Incidence ; Kidney/abnormalities ; Pregnancy ; Prenatal Diagnosis/methods ; Urinary Tract/abnormalities ; Urologic Diseases/congenital ; Urologic Diseases/diagnosis ; Urologic Diseases/epidemiology
    Language English
    Publishing date 2014-09
    Publishing country United States
    Document type Editorial
    ZDB-ID 193116-7
    ISSN 1557-9840 ; 0095-5108
    ISSN (online) 1557-9840
    ISSN 0095-5108
    DOI 10.1016/j.clp.2014.06.001
    Database MEDical Literature Analysis and Retrieval System OnLINE

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