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ECFS standards of care on CFTR-related disorders: Towards a comprehensive program for affected individuals.
De Wachter E, De Boeck K, Sermet-Gaudelus I, Simmonds NJ, Munck A, Naehrlich L, Barben J, Boyd C, Veen SJ, Carr SB, Fajac I, Farrell PM, Girodon E, Gonska T, Grody WW, Jain M, Jung A, Kerem E, Raraigh KS, van Koningsbruggen-Rietschel S, Waller MD, Southern KW, Castellani C; ECFS Diagnostic Network Working Group. De Wachter E, et al. Among authors: gonska t. J Cyst Fibros. 2024 Feb 21:S1569-1993(24)00011-0. doi: 10.1016/j.jcf.2024.01.012. Online ahead of print. J Cyst Fibros. 2024. PMID: 38388234
Topics included are: 1) benefits and obstacles to collect data from pwCFTR-RD are discussed, together with the opportunity to integrate them into established CF-registries; 2) the potential of infants designated CRMS/CFSPID to develop a CFTR-RD and how to communicate this informa …
Topics included are: 1) benefits and obstacles to collect data from pwCFTR-RD are discussed, together with the opportunity to integrate them …
Weight increase in people with cystic fibrosis on CFTR modulator therapy is mainly due to increase in fat mass.
Mouzaki M, Dupuis A, Avolio J, Griffin K, Ratjen F, Tullis E, Gonska T. Mouzaki M, et al. Among authors: gonska t. Front Pharmacol. 2023 Jul 13;14:1157459. doi: 10.3389/fphar.2023.1157459. eCollection 2023. Front Pharmacol. 2023. PMID: 37521467 Free PMC article.
Background: Ivacaftor, the first CFTR modulator drug, leads to significant long-term improvement in lung function and weight gain. The mechanism as well as the long-term impact of ivacaftor on weight, resting energy expenditure (REE) and body composition remains to …
Background: Ivacaftor, the first CFTR modulator drug, leads to significant long-term improvement in lung function and weight gain. Th …
Aquagenic wrinkling of the palms in cystic fibrosis patients treated with ivacaftor.
Jacobi E, Solomon M, Avolio J, Shaw M, Gonska T, Ratjen F, Grasemann H. Jacobi E, et al. Among authors: gonska t. J Cyst Fibros. 2022 Mar;21(2):e102-e105. doi: 10.1016/j.jcf.2022.01.005. Epub 2022 Jan 19. J Cyst Fibros. 2022. PMID: 35063397 Free article.
Aquagenic wrinkling of palms (AWP) in cystic fibrosis (CF) patients and common CFTR mutations is recognized as a frequent symptom of the disease. The long-term effect of CFTR targeting therapy on AWP has not been studied. ...
Aquagenic wrinkling of palms (AWP) in cystic fibrosis (CF) patients and common CFTR mutations is recognized as a frequent symptom of the dis …
Vascular Complications in Pediatric Pancreatitis: A Case Series.
Dike CR, Cress G, Fishman DS, Gonska T, Ooi CY, Perito ER, Troendle D, Tsai CM, Lowe ME, Uc A. Dike CR, et al. Among authors: gonska t. J Pediatr Gastroenterol Nutr. 2021 Oct 1;73(4):e94-e97. doi: 10.1097/MPG.0000000000003218. J Pediatr Gastroenterol Nutr. 2021. PMID: 34224489 Free PMC article.
Anti-coagulants appear to be safe in children with acute pancreatitis, their long-term benefit needs to be further investigated....
Anti-coagulants appear to be safe in children with acute pancreatitis, their long-term benefit needs to be further investigated....
Clinical Characteristics and Long-term Outcomes of Children With Fibrosing Pancreatitis.
Scheers I, Chavhan GB, Chami R, Carman N, Scaini V, Gaisano H, Marcon M, Gonska T. Scheers I, et al. Among authors: gonska t. J Pediatr Gastroenterol Nutr. 2020 Jun;70(6):801-807. doi: 10.1097/MPG.0000000000002726. J Pediatr Gastroenterol Nutr. 2020. PMID: 32443035
This study aimed to assess the presence of AIP criteria in an historic FP patient cohort and investigate the clinical features, management, and long-term outcomes of pediatric FP (P-FP). METHODS: Clinical data of 14 P-FP patients from Toronto and 42 P-FP cases from a liter …
This study aimed to assess the presence of AIP criteria in an historic FP patient cohort and investigate the clinical features, management, …
Immunoreactive trypsinogen levels in newborn screened infants with an inconclusive diagnosis of cystic fibrosis.
Ooi CY, Sutherland R, Castellani C, Keenan K, Boland M, Reisman J, Bjornson C, Chilvers MA, van Wylick R, Kent S, Price A, Mateos-Corral D, Hughes D, Solomon M, Zuberbuhler P, Brusky J, Durie PR, Ratjen F, Gonska T. Ooi CY, et al. Among authors: gonska t. BMC Pediatr. 2019 Oct 22;19(1):369. doi: 10.1186/s12887-019-1756-4. BMC Pediatr. 2019. PMID: 31640630 Free PMC article.
CF transmembrane conductance regulator (CFTR)-related metabolic syndrome (CRMS) or CF screen positive inconclusive diagnosis (CFSPID). These infants have an uncertain long-term outcome and it is currently unclear around time of diagnosis, which infants are at higher risk o …
CF transmembrane conductance regulator (CFTR)-related metabolic syndrome (CRMS) or CF screen positive inconclusive diagnosis (CFSPID). These …
Long-term effect of CFTR modulator therapy on airway nitric oxide.
Grasemann H, Klingel M, Avolio J, Prentice C, Gonska T, Tullis E, Ratjen F. Grasemann H, et al. Among authors: gonska t. Eur Respir J. 2020 Jan 9;55(1):1901113. doi: 10.1183/13993003.01113-2019. Print 2020 Jan. Eur Respir J. 2020. PMID: 31601715 Free article. No abstract available.
Cystic Fibrosis Transmembrane Conductance Regulator-Related Metabolic Syndrome and Cystic Fibrosis Screen Positive, Inconclusive Diagnosis.
Ren CL, Borowitz DS, Gonska T, Howenstine MS, Levy H, Massie J, Milla C, Munck A, Southern KW. Ren CL, et al. Among authors: gonska t. J Pediatr. 2017 Feb;181S:S45-S51.e1. doi: 10.1016/j.jpeds.2016.09.066. J Pediatr. 2017. PMID: 28129812
However, between 10% and 20% of asymptomatic infants can develop clinical features concerning for CF, such as a respiratory culture positive for Pseudomonas aeruginosa. Most studies have only reported short-term outcomes in the first 1-3 years of life; the long-term …
However, between 10% and 20% of asymptomatic infants can develop clinical features concerning for CF, such as a respiratory culture positive …
Causal Evaluation of Acute Recurrent and Chronic Pancreatitis in Children: Consensus From the INSPPIRE Group.
Gariepy CE, Heyman MB, Lowe ME, Pohl JF, Werlin SL, Wilschanski M, Barth B, Fishman DS, Freedman SD, Giefer MJ, Gonska T, Himes R, Husain SZ, Morinville VD, Ooi CY, Schwarzenberg SJ, Troendle DM, Yen E, Uc A. Gariepy CE, et al. Among authors: gonska t. J Pediatr Gastroenterol Nutr. 2017 Jan;64(1):95-103. doi: 10.1097/MPG.0000000000001446. J Pediatr Gastroenterol Nutr. 2017. PMID: 27782962 Free PMC article.
RESULTS: The panel reached consensus on 27 statements covering the definitions of pediatric ARP and CP, evaluation for potential etiologies of these disorders, and long-term monitoring. Statements for which the group reached consensus to make no recommendation or could not …
RESULTS: The panel reached consensus on 27 statements covering the definitions of pediatric ARP and CP, evaluation for potential etiologies …
Total pancreatectomy and islet autotransplantation in chronic pancreatitis: recommendations from PancreasFest.
Bellin MD, Freeman ML, Gelrud A, Slivka A, Clavel A, Humar A, Schwarzenberg SJ, Lowe ME, Rickels MR, Whitcomb DC, Matthews JB; PancreasFest Recommendation Conference Participants; Amann S, Andersen DK, Anderson MA, Baillie J, Block G, Brand R, Chari S, Cook M, Cote GA, Dunn T, Frulloni L, Greer JB, Hollingsworth MA, Kim KM, Larson A, Lerch MM, Lin T, Muniraj T, Robertson RP, Sclair S, Singh S, Stopczynski R, Toledo FG, Wilcox CM, Windsor J, Yadav D. Bellin MD, et al. Pancreatology. 2014 Jan-Feb;14(1):27-35. doi: 10.1016/j.pan.2013.10.009. Epub 2013 Nov 13. Pancreatology. 2014. PMID: 24555976 Free PMC article.
CONCLUSIONS: TPIAT is an effective method of managing the disabling complications of chronic pancreatitis and risk of pancreatic cancer in very high risk patients. Careful evaluation and long-term management of candidate patients by qualified multidisciplinary teams is req …
CONCLUSIONS: TPIAT is an effective method of managing the disabling complications of chronic pancreatitis and risk of pancreatic cancer in v …