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1.
Adv Neonatal Care ; 21(3): E65-E72, 2021 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-33427753

RESUMO

BACKGROUND: Standardized parenteral nutrition (PN) formulations are used in at-risk neonates to provide nutrition immediately following birth. However, evidence for the optimal formulation(s) to maximize growth while reducing the risks of glucose and electrolyte abnormalities is limited. PURPOSE: The purpose of this study was to compare the rates of hypernatremia and hyperglycemia with 2 weight-based standardized PN formulations versus one standard PN in low birth-weight preterm neonates. METHODS: This was a single-center observational study of infants less than 1800 g birth weight and less than 37 weeks' gestation who received standardized PN in the first 48 hours of life. Patients in the weight-based PN group were compared with a historical group of patients receiving single standard PN. Rates of hypernatremia and hyperglycemia were compared by χ2 analysis. RESULTS: There was a nonsignificant (P = .147) reduction in hypernatremia in the weight-based PN group (9 of 87; 10.3%) compared with the single PN group (16 of 89; 18.0%). However, hyperglycemia was significantly more frequent in the weight-based group than in the single PN group (24.1% vs 12.4%, P = .035). IMPLICATIONS FOR PRACTICE: The 2 weight-based PN standardized formulations studied did not significantly decrease the incidence of hypernatremia or hyperglycemia. IMPLICATIONS FOR RESEARCH: Future studies to determine optimal standardized PN to provide early nutrition in high-risk neonates are warranted.


Assuntos
Hiperglicemia , Hipernatremia , Humanos , Hiperglicemia/epidemiologia , Hiperglicemia/prevenção & controle , Hipernatremia/epidemiologia , Hipernatremia/prevenção & controle , Incidência , Lactente , Recém-Nascido , Recém-Nascido Prematuro , Nutrição Parenteral
2.
Pediatr Pulmonol ; 57(2): 411-417, 2022 02.
Artigo em Inglês | MEDLINE | ID: mdl-34850610

RESUMO

INTRODUCTION: Cystic fibrosis (CF)-related liver disease (CFLD) manifests as a wide spectrum of hepatobiliary disease and can progress to need liver transplantation. Elexacaftor/tezacaftor/ivacaftor (elx/tez/iva) is a cystic fibrosis transmembrane conductance regulator modulator that has superior efficacy compared to previously approved modulators. Use of elx/tez/iva, should be approached with caution in individuals with CFLD or following liver transplantation due to possible increases in liver function tests (LFTs) and drug-drug interactions with several immunosuppressant medications. OBJECTIVE: The purpose of this case series is to explore if the use of elx/tez/iva is safe and tolerable in patients with CF postliver transplantation. METHODS: A retrospective case series including patients prescribed elx/tez/iva following liver transplantation and an immunosuppressive regimen consisting of drug therapy metabolized by P-glycoprotein was completed. RESULTS: Ten patients at six CF centers with a median age of 22.1 years (range 14-43.4 years) and the median time from the transplant of 6.9 years (range 0.6-22 years) were included. Most patients (8, 80%) received a reduced or full dose of elx/tez/iva for a mean duration of 10.4 months (range 7-12 months). Fluctuations in LFTs occurred in all patients (10, 100%) and led to therapy discontinuation in two patients (20%). Elx/tez/iva initiation resulted in elevations in tacrolimus trough concentration in seven patients (70%). Most patients who tolerated elx/tez/iva had symptomatic and quality of life improvement, increased body mass index, and maintained or improved lung function. CONCLUSION: Initiation of elx/tez/iva in patients with CF who received liver transplantation may be safe with clinical benefits.


Assuntos
Fibrose Cística , Adolescente , Adulto , Aminofenóis , Benzodioxóis , Agonistas dos Canais de Cloreto/uso terapêutico , Fibrose Cística/complicações , Fibrose Cística/tratamento farmacológico , Fibrose Cística/cirurgia , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Combinação de Medicamentos , Humanos , Indóis , Mutação , Pirazóis , Piridinas , Pirrolidinas , Qualidade de Vida , Quinolonas , Estudos Retrospectivos , Adulto Jovem
3.
J Cyst Fibros ; 21(2): 339-343, 2022 03.
Artigo em Inglês | MEDLINE | ID: mdl-34742667

RESUMO

Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA, Trikafta) is the newest Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulator drug approved by the Food and Drug Administration. Post-marketing reports with earlier CFTR modulators suggest these medications can impact mood, and in clinical trials an adverse effect of headache was reported with all currently approved CFTR modulators. However, there are no other documented reports of mental status changes during clinical trials or in post-marketing reports with elexacaftor/tezacaftor/ivacaftor. In this case series, we describe 6 patients who reported "mental fogginess" or other mental status changes shortly after initiation of this drug. The mechanism of this patient-reported side effect is still unclear. All patients noticed a change within the first 3 months of therapy. The management differed in each case, with all four cystic fibrosis (CF) care teams utilizing a patient-centered decision-making approach to address this concern.


Assuntos
Afeto , Regulador de Condutância Transmembrana em Fibrose Cística , Fibrose Cística , Afeto/efeitos dos fármacos , Aminofenóis , Benzodioxóis , Agonistas dos Canais de Cloreto , Fibrose Cística/tratamento farmacológico , Fibrose Cística/genética , Regulador de Condutância Transmembrana em Fibrose Cística/efeitos dos fármacos , Combinação de Medicamentos , Humanos , Indóis , Fadiga Mental , Pirazóis , Piridinas , Pirrolidinas , Quinolonas , Transtornos Somatoformes
4.
J Cyst Fibros ; 21(6): 1066-1069, 2022 11.
Artigo em Inglês | MEDLINE | ID: mdl-36088208

RESUMO

Elexacaftor/tezacaftor/ivacaftor (ELX-TEZ-IVA) is a Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulator shown to improve lung function and reduce sweat chloride in people with Cystic Fibrosis (CF). The only commonly reported dermatologic adverse effect with CFTR modulators including ELX-TEZ-IVA is rash. In this case series, we describe 19 patients who reported new onset or worsening of acne after initiation of this drug to their CF pharmacist or another member of their CF care team. The mechanism and frequency of this adverse effect is unknown.


Assuntos
Acne Vulgar , Fibrose Cística , Humanos , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Fibrose Cística/tratamento farmacológico , Fibrose Cística/genética , Agonistas dos Canais de Cloreto/efeitos adversos , Aminofenóis/efeitos adversos , Benzodioxóis/efeitos adversos , Acne Vulgar/tratamento farmacológico , Acne Vulgar/induzido quimicamente , Mutação
5.
J Cyst Fibros ; 20(3): 506-510, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-32736949

RESUMO

Individuals with cystic fibrosis (CF) have an increased risk for gallbladder abnormalities and biliary tract disease, but the reported incidence of these manifestations of CF varies widely in the literature. With the approval of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA), increasing numbers of CF patients have been initiated on highly effective cystic fibrosis transmembrane regulator (CFTR) modulator therapy. While elevations in hepatic panel are known potential side effects of CFTR modulators, there have been no published cases of biliary disease or acute cholecystitis attributed to these medications. In this case series, we describe seven patients at two adult CF centers with biliary colic shortly after initiation with ELX/TEZ/IVA, six of whom required cholecystectomy.


Assuntos
Doenças Biliares/induzido quimicamente , Doenças Biliares/cirurgia , Agonistas dos Canais de Cloreto/efeitos adversos , Colecistectomia , Fibrose Cística/tratamento farmacológico , Adulto , Aminofenóis/efeitos adversos , Benzodioxóis/efeitos adversos , Combinação de Medicamentos , Feminino , Humanos , Indóis/efeitos adversos , Masculino , Pirazóis/efeitos adversos , Piridinas/efeitos adversos , Pirrolidinas/efeitos adversos , Quinolonas/efeitos adversos
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