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Long-term clinical benefits of delayed-release cysteamine bitartrate capsules in patients with nephropathic cystinosis (response to "A comparison of immediate release and delayed release cysteamine in 17 patients with nephropathic cystinosis").
Langman CB. Langman CB. Orphanet J Rare Dis. 2023 Jun 23;18(1):162. doi: 10.1186/s13023-023-02724-3. Orphanet J Rare Dis. 2023. PMID: 37353838 Free PMC article.
The aim of this letter to the editor is to summarize the results from three clinical trial programs evaluating delayed-release cysteamine bitartrate (DR-CYS), which demonstrated the long-term clinical benefits in patients with nephropathic cystinosis when dosed every 12 h. …
The aim of this letter to the editor is to summarize the results from three clinical trial programs evaluating delayed-release cysteamine bi …
Cysteamine bitartrate delayed-release capsules control leukocyte cystine levels and promote statural growth and kidney health in an open-label study of treatment-naïve patients <6 years of age with nephropathic cystinosis.
Vaisbich MH, Caires Ferreira J, Price H, Young KD, Sile S, Checani G, Langman CB. Vaisbich MH, et al. Among authors: langman cb. JIMD Rep. 2021 Nov 1;63(1):66-79. doi: 10.1002/jmd2.12260. eCollection 2022 Jan. JIMD Rep. 2021. PMID: 35028272 Free PMC article.
Nephropathic cystinosis is a rare autosomal recessive lysosomal storage disease that is characterized by accumulation of cysteine and formation of crystals within cells of different organs and tissues causing systemic manifestations in childhood that include poor linear growth, o …
Nephropathic cystinosis is a rare autosomal recessive lysosomal storage disease that is characterized by accumulation of cysteine and format …
Incidence, Predictors, and Impact of Postoperative Acute Kidney Injury Following Fontan Conversion Surgery in Young Adult Fontan Survivors.
Patel SR, Costello JM, Andrei AC, Backer CL, Krawczeski CD, Deal BJ, Langman CB, Marino BS. Patel SR, et al. Among authors: langman cb. Semin Thorac Cardiovasc Surg. 2022 Summer;34(2):631-639. doi: 10.1053/j.semtcvs.2021.02.027. Epub 2021 Mar 7. Semin Thorac Cardiovasc Surg. 2022. PMID: 33691191
Chi-square and 2-sample t-tests assessed associations between AKI2 and postoperative outcomes. Mid-term heart-transplant-free survival among AKI0-1 vs AKI2-3 groups was compared using Kaplan-Meier curves and log-rank test. ...AKI 2-3 is associated with worse early postoper …
Chi-square and 2-sample t-tests assessed associations between AKI2 and postoperative outcomes. Mid-term heart-transplant-free surviva …
Pathophysiology and Treatment of Enteric Hyperoxaluria.
Witting C, Langman CB, Assimos D, Baum MA, Kausz A, Milliner D, Tasian G, Worcester E, Allain M, West M, Knauf F, Lieske JC. Witting C, et al. Among authors: langman cb. Clin J Am Soc Nephrol. 2021 Mar 8;16(3):487-495. doi: 10.2215/CJN.08000520. Epub 2020 Sep 8. Clin J Am Soc Nephrol. 2021. PMID: 32900691 Free PMC article.
Thus, further research is needed to better define the precise factors that influence risk of adverse outcomes, the long-term efficacy of available treatment strategies, and to develop new therapeutic approaches....
Thus, further research is needed to better define the precise factors that influence risk of adverse outcomes, the long-term efficacy …
Predictors of time to first cannulation for arteriovenous fistula in pediatric hemodialysis patients: Midwest Pediatric Nephrology Consortium study.
Onder AM, Flynn JT, Billings AA, Deng F, DeFreitas M, Katsoufis C, Grinsell MM, Patterson L, Jetton J, Fathallah-Shaykh S, Ranch D, Aviles D, Copelovitch L, Ellis E, Chadha V, Elmaghrabi A, Lin JJ, Butani L, Haddad M, Marsenic O, Brakeman P, Quigley R, Shin HS, Garro R, Liu H, Rahimikollu J, Raina R, Langman CB, Wood E; Midwest Pediatric Nephrology Consortium. Onder AM, et al. Among authors: langman cb. Pediatr Nephrol. 2020 Feb;35(2):287-295. doi: 10.1007/s00467-019-04396-3. Epub 2019 Nov 6. Pediatr Nephrol. 2020. PMID: 31696356
BACKGROUND: Permanent vascular access (PVA) is preferred for long-term hemodialysis. Arteriovenous fistulae (AVF) have the best patency and the lowest complication rates compared to arteriovenous grafts (AVG) and tunneled cuffed catheters (TCC). ...
BACKGROUND: Permanent vascular access (PVA) is preferred for long-term hemodialysis. Arteriovenous fistulae (AVF) have the best paten …
Race, obesity, and the renin-angiotensin-aldosterone system: treatment response in children with primary hypertension.
South AM, Arguelles L, Finer G, Langman CB. South AM, et al. Among authors: langman cb. Pediatr Nephrol. 2017 Sep;32(9):1585-1594. doi: 10.1007/s00467-017-3665-9. Epub 2017 Apr 14. Pediatr Nephrol. 2017. PMID: 28411317
CONCLUSIONS: Pretreatment aldosterone and PRA predicted short-term follow-up BP and LVMI, especially in nonobese and white patients. ...
CONCLUSIONS: Pretreatment aldosterone and PRA predicted short-term follow-up BP and LVMI, especially in nonobese and white patients. …
Maternal BMI Associations with Maternal and Cord Blood Vitamin D Levels in a North American Subset of Hyperglycemia and Adverse Pregnancy Outcome (HAPO) Study Participants.
Josefson JL, Reisetter A, Scholtens DM, Price HE, Metzger BE, Langman CB; HAPO Study Cooperative Research Group. Josefson JL, et al. Among authors: langman cb. PLoS One. 2016 Mar 4;11(3):e0150221. doi: 10.1371/journal.pone.0150221. eCollection 2016. PLoS One. 2016. PMID: 26942930 Free PMC article.
Neither birth weight nor neonatal adiposity was significantly associated with cord blood 25-OHD levels. ...The results also suggest that interpretation of neonatal 25-OHD levels may need to incorporate specific maternal factors in addition to season of birth and lat …
Neither birth weight nor neonatal adiposity was significantly associated with cord blood 25-OHD levels. ...The results also suggest t …
Quality of life is improved and kidney function preserved in patients with nephropathic cystinosis treated for 2 years with delayed-release cysteamine bitartrate.
Langman CB, Greenbaum LA, Grimm P, Sarwal M, Niaudet P, Deschenes G, Cornelissen EA, Morin D, Cochat P, Elenberg E, Hanna C, Gaillard S, Bagger MJ, Rioux P. Langman CB, et al. J Pediatr. 2014 Sep;165(3):528-33.e1. doi: 10.1016/j.jpeds.2014.05.013. Epub 2014 Jun 16. J Pediatr. 2014. PMID: 24948347 Free PMC article. Clinical Trial.
OBJECTIVES: To determine the long-term effects of delayed-release cysteamine bitartrate (DR-CYS) based on our previous work that established the short-term noninferiority of DR-CYS every 12 hours compared with immediate-release cysteamine bitartrate every 6 hours. . …
OBJECTIVES: To determine the long-term effects of delayed-release cysteamine bitartrate (DR-CYS) based on our previous work that esta …
Official positions of the International Society for Clinical Densitometry (ISCD) on DXA evaluation in children and adolescents.
Bianchi ML, Baim S, Bishop NJ, Gordon CM, Hans DB, Langman CB, Leonard MB, Kalkwarf HJ. Bianchi ML, et al. Among authors: langman cb. Pediatr Nephrol. 2010 Jan;25(1):37-47. doi: 10.1007/s00467-009-1249-z. Epub 2009 Jul 15. Pediatr Nephrol. 2010. PMID: 19603190
However, its use in pediatric patients, during growth and development, poses a much more complex problem in terms of both the technical aspects and the interpretation of the results. For the adults population, there is a well-defined term of reference: the peak value of BM …
However, its use in pediatric patients, during growth and development, poses a much more complex problem in terms of both the technical aspe …
Growth in X-linked hypophosphatemic rickets.
Ariceta G, Langman CB. Ariceta G, et al. Among authors: langman cb. Eur J Pediatr. 2007 Apr;166(4):303-9. doi: 10.1007/s00431-006-0357-z. Epub 2006 Dec 14. Eur J Pediatr. 2007. PMID: 17171343
In 16 children entirely controlled in our program upon presentation, a "catch up" phenomenon after the rickets had healed (P = 0.823) or throughout the long-term was not observed (P = 0.995). Eight patients had a Z-height </= -2SD at the last visit, and impaired linear …
In 16 children entirely controlled in our program upon presentation, a "catch up" phenomenon after the rickets had healed (P = 0.823) or thr …
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