Cystic fibrosis transmembrane conductance regulator modulators reduce the risk of recurrent acute pancreatitis among adult patients with pancreas sufficient cystic fibrosis.
Pancreatology
; 19(8): 1023-1026, 2019 Dec.
Article
en En
| MEDLINE
| ID: mdl-31611131
ABSTRACT
BACKGROUND:
Approximately 1 in 5 patients with pancreas sufficient cystic fibrosis (PS-CF) will develop acute pancreatitis (AP). It is not known whether ivacaftor alone or in combination with other CFTR (cystic transmembrane regulator) modulators (tezacaftor or lumacaftor) can reduce the risk of AP in patients with PS-CF and AP history.METHODS:
We retrospectively queried the CF registry at our institution for adult patients with PS-CF, a documented history of AP and initiation of CFTR modulators for pulmonary indications. Patient characteristics including demographics, CFTR genotype, pancreatitis risk factors, pancreatic exocrine function and other relevant laboratory, imaging parameters were obtained from the time of the sentinel AP episode through the follow-up period.RESULTS:
A total of 15 adult CF patients were identified with mean age of 44.1 years (SD⯱â¯13.8). In the 24 months preceding CFTR modulator initiation, six of these patients had at least 1 episode of AP with median of 2 episodes [1.75, 2.5]. None of the patients had evidence of pancreatic calcifications or exocrine pancreas insufficiency at the time of CFTR modulator initiation. The mean duration of follow-up after CFTR modulator initiation was 36.7 months (SD⯱â¯21.5). None of the patients who remained on CFTR modulators developed an episode of AP or required hospitalization for AP related abdominal pain during follow-up.CONCLUSIONS:
CFTR modulators, alone or in combination, substantially reduce the risk of recurrent AP over a mean follow-up period of 3 years in adult patients with PS-CF and a history of prior AP. These data suggest that any augmentation of CFTR function can reduce the risk of pancreatitis.Palabras clave
Texto completo:
1
Base de datos:
MEDLINE
Asunto principal:
Insuficiencia Pancreática Exocrina
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Quinolonas
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Regulador de Conductancia de Transmembrana de Fibrosis Quística
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Fibrosis Quística
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Benzodioxoles
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Aminofenoles
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Aminopiridinas
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Indoles
Tipo de estudio:
Etiology_studies
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Observational_studies
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Prognostic_studies
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Risk_factors_studies
Límite:
Adult
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Aged
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Female
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Humans
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Male
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Middle aged
Idioma:
En
Revista:
Pancreatology
Asunto de la revista:
ENDOCRINOLOGIA
/
GASTROENTEROLOGIA
Año:
2019
Tipo del documento:
Article
País de afiliación:
Estados Unidos