Understanding Cystic Fibrosis Comorbidities and Their Impact on Nutritional Management

Patel, Dhiren; Shan, Albert; Mathews, Stacy; Sathe, Meghana

doi:10.3390/nu14051028

Cited by 7 publications

(9 citation statements)

References 138 publications

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“…Due to this mutation, chloride cannot be transported out of cells and bicarbonate, which is essential to neutralize gastric acid to aid in digestion, is decreased due to the impaired pancreas. The pancreas impairment also causes less fluid emptied into the intestines 7–9 . Acidified mucous layer along with dehydrated intestinal fluid leads to delayed intestinal transit attributing to constipation in these patients.…”

Section: Constipationmentioning

confidence: 99%

Constipation and DIOS: Diagnosis, differential diagnosis, and management

Saikumar,

Sadauskas,

Izhar

et al. 2024

Pediatric Pulmonology

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Cystic Fibrosis (CF) is a complex disorder that requires multidisciplinary expertise for effective management. The GALAXY study estimated the prevalence of constipation to be about 25% among People with Cystic Fibrosis (PwCF), identifying it as one of the common gastrointestinal (GI) symptoms within this patient population. Quality of Life (QoL) assessments uncovered high patient dissatisfaction, highlighting the imperative need for enhanced treatment strategies. Similarly, Distal Intestinal Obstruction Syndrome (DIOS) is a unique condition exclusive to PwCF that, if left undiagnosed, can lead to considerable morbidity and mortality. Given the broad spectrum of differential diagnoses for abdominal pain, including constipation and DIOS, it is paramount for healthcare providers to possess a clear understanding of these conditions. This paper aims to delineate various differentials for abdominal pain while elucidating the pathogenesis, diagnostic criteria, and treatment options for managing constipation and DIOS in PwCF.

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Section: Constipationmentioning

confidence: 99%

Constipation and DIOS: Diagnosis, differential diagnosis, and management

Saikumar,

Sadauskas,

Izhar

et al. 2024

Pediatric Pulmonology

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“…Other complications of CF are represented by a CF-related diabetes, that could determine and worsen malnutrition and CF-related liver disease which associates specific nutritional deficiencies (fat-soluble vitamins, essential fatty acid, calcium), that can lead to osteopenia and osteoporosis [ 34 , 35 , 36 , 37 ].…”

Section: Clinical Considerations Of Exocrine Pancreatic Insufficiencymentioning

confidence: 99%

“…Research suggests that CFTR modulators may increase weight in CF patients, the underlying mechanism being multifactorial (improved pancreatic exocrine function, increased calorie intake, and nutrient absorption, decreased energy expenditure due to improved pulmonary function) [ 34 , 46 , 47 ].…”

Section: Clinical Considerations Of Exocrine Pancreatic Insufficiencymentioning

confidence: 99%

Current and Future Therapeutic Approaches of Exocrine Pancreatic Insufficiency in Children with Cystic Fibrosis in the Era of Personalized Medicine

et al. 2023

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This review presents current updates of pancreatic enzyme replacement therapy in children with cystic fibrosis based on literature published in the last decade and some special considerations regarding pancreatic enzyme replacement therapy in the era of new therapies, such as cystic fibrosis transmembrane conductance regulator modulator therapies. Few articles evaluate the efficacy of pancreatic enzyme replacement therapy in the pediatric population, and most studies also included children and adults with cystic fibrosis. Approximately 85% of cystic fibrosis patients have exocrine pancreatic insufficiency and need pancreatic enzyme replacement therapy. Fecal elastase is the most commonly used diagnostic test for exocrine pancreatic insufficiency, although this value can fluctuate over time. While it is used as a diagnostic test, it cannot be used for monitoring the effectiveness of pancreatic enzyme replacement therapy and for adjusting doses. Pancreatic enzyme replacement therapy, the actual treatment for exocrine pancreatic insufficiency, is essential in children with cystic fibrosis to prevent malabsorption and malnutrition and needs to be urgently initiated. This therapy presents many considerations for physicians, patients, and their families, including types and timing of administration, dose monitoring, and therapy failures. Based on clinical trials, pancreatic enzyme replacement therapy is considered effective and well-tolerated in children with cystic fibrosis. An important key point in cystic fibrosis treatment is the recent hypothesis that cystic fibrosis transmembrane conductance regulator modulators could improve pancreatic function, further studies being essential. Pancreatic enzyme replacement therapy is addressed a complication of the disease (exocrine pancreatic insufficiency), while modulators target the defective cystic fibrosis transmembrane conductance regulator protein. Exocrine pancreatic insufficiency in cystic fibrosis remains an active area of research in this era of cystic fibrosis transmembrane conductance regulator modulator therapies. This new therapy could represent an example of personalized medicine in cystic fibrosis patients, with each class of modulators being addressed to patients with specific genetic mutations.

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“…The prevalence of CFLD is high among CwCF, ranging from 5% to 68% based on the disease manifestation [71]. Focal biliary cirrhosis progressing to multi-lobular cirrhosis is seen in 5-10% of CwCF in the first decade [72].…”

Section: Pediatricsmentioning

confidence: 99%

A Gastroenterologist’s Guide to Care Transitions in Cystic Fibrosis from Pediatrics to Adult Care

Patel,

Baliss,

Saikumar

et al. 2023

IJMS

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Cystic Fibrosis is a chronic disease affecting multiple systems, including the GI tract. Clinical manifestation in patients can start as early as infancy and vary across different age groups. With the advent of new, highly effective modulators, the life expectancy of PwCF has improved significantly. Various GI aspects of CF care, such as nutrition, are linked to an overall improvement in morbidity, lung function and the quality of life of PwCF. The variable clinical presentations and management of GI diseases in pediatrics and adults with CF should be recognized. Therefore, it is necessary to ensure efficient transfer of information between pediatric and adult providers for proper continuity of management and coordination of care at the time of transition. The transition of care is a challenging process for both patients and providers and currently there are no specific tools for GI providers to help ensure a smooth transition. In this review, we aim to highlight the crucial features of GI care at the time of transition and provide a checklist that can assist in ensuring an effective transition and ease the challenges associated with it.

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Understanding Cystic Fibrosis Comorbidities and Their Impact on Nutritional Management

Cited by 7 publications

References 138 publications

Constipation and DIOS: Diagnosis, differential diagnosis, and management

Constipation and DIOS: Diagnosis, differential diagnosis, and management

Current and Future Therapeutic Approaches of Exocrine Pancreatic Insufficiency in Children with Cystic Fibrosis in the Era of Personalized Medicine

A Gastroenterologist’s Guide to Care Transitions in Cystic Fibrosis from Pediatrics to Adult Care

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