LAPAROSCOPIC SANTULLI INTESTINAL ANASTOMOSIS IN AN INFANT WITH CYSTIC FIBROSIS

2021 ◽  
Vol 100 (6) ◽  
pp. 187-191
Author(s):  
Yu.A. Kozlov ◽  
◽  
L.V. Bregel ◽  
A.A. Rasputin ◽  
P.J. Baradieva ◽  
...  

The aim of the study was to evaluate the technical feasibility of performing a Santulli-type side-toend inter-intestinal anastomosis in infants with cystic fibrosis (CF) using laparoscopy. A clinical case of a 3-month-old child with CF who was operated on in the neonatal period for meconium ileus is presented. The baby's gestational age was 32 weeks. The primary operation consisted of the formation of a terminal enterostomy. In the postoperative period, a malabsorption syndrome was noted, accompanied by a lack of growth. As a temporary measure to restore nutritional status, a Santulli-style side-to-end laparoscopic intestinal anastomosis was performed. The observed child was found to have the F508del mutation in both alleles of the CFTR gene. The patient's weight at the time of the Santulli anastomosis construction was 2900 g, the age – 3 months. During the operation, there were no difficulties associated with the mobilization of the separated segments of the ileum. The duration of the surgical intervention was 70 min. Enteral nutrition was started on the 3rd day after the operation. The recovery period for intestinal transit through the rectum was 15 days. The postoperative period was uneventful. The duration of hospitalization was 18 days. No electrolyte imbalance or excessive fluid loss or underweight associated with enterostomy was observed. Over the next 6 months, the normalization of age-related weight and height parameters was achieved, after which the continuity of the digestive tract was restored by closing the terminal enterostomy. Currently, the patient has minimal respiratory symptoms and is receiving adequate CF therapy with pancreatic enzyme replacement therapy included with each meal. The initial experience of performing laparoscopic Santulli inter-intestinal anastomosis in an infant with CF presented in the study showed the possibility and reproducibility of this technique, expanding the boundaries of laparoscopy in pediatric practice. The decision to close the enterostomy in these patients should be deferred until a full diet is introduced and should be made in conjunction with a pediatrician specializing in the treatment of CF.

2010 ◽  
Vol 2010 ◽  
pp. 1-7 ◽  
Author(s):  
Michael W. Konstan ◽  
Theodore G. Liou ◽  
Steven D. Strausbaugh ◽  
Richard Ahrens ◽  
Jamshed F. Kanga ◽  
...  

Background. Pancreatic enzyme replacement therapy is the standard of care for treatment of malabsorption in patients with cystic fibrosis (CF) and exocrine pancreatic insufficiency (PI).Aim. To evaluate efficacy and safety of a new formulation of pancrelipase (Ultrase MT20) in patients with CF and PI. Coefficients of fat absorption (CFA%) and nitrogen absorption (CNA%) were the main efficacy parameters. Safety was evaluated by monitoring laboratory analyses, adverse events (AEs), and overall signs and symptoms.Methods. Patients (n=31) were randomized in a crossover design comparing this pancrelipase with placebo during 2 inpatient evaluation periods (6-7 days each). Fat and protein/nitrogen ingestion and excretion were measured from food diaries and 72-hour stool collections. CFA% and CNA% were calculated for each period and compared.Results. Twenty-four patients provided analyzable data. This pancrelipase increased mean CFA% and CNA% (+34.7% and +25.7%, resp.,P<.0001for both), reduced stool frequency, and improved stool consistency compared with placebo. Placebo-treated patients reported more AEs, with gastrointestinal symptoms being the most frequently reported AE.Conclusions. This pancrelipase is a safe and effective treatment for malabsorption associated with exocrine PI in patients with CF.


2021 ◽  
Vol 4 (2) ◽  
pp. 84-93
Author(s):  
Muzal Kadim ◽  
William Cheng

Background Cystic fibrosis (CF) is an inherited genetic disorder with high mortality and morbidity. CF is strongly correlated with malnutrition due to higher energy losses, pancreatic insufficiency, chronic inflammation, higher resting energy expenditure, and feeding problems. Malnutrition in CF patients associated with worse survival. Thus, appropriate and prompt nutritional intervention should be addressed to reduced malnutrition in CF patients. Methods The literature search was performed on 9 August 2021 in four major databases such as MEDLINE, EBSCOhost, Cochrane Reviews, and Web of Sciences to find the role of nutrition and pancreatic enzyme replacement therapy in pediatrics population with cystic fibrosis. Recent findings In recent decades, early nutritional management and pancreatic enzyme replacement therapy (PERT) have been shown to improve CF patient’s outcomes. Nutrition should be given in higher calories compared to healthy individuals with close and regular nutritional status monitoring. High protein and fat diets are essential for CF patient’s overall survival. Adequate level of micronutrients should be ensured to avoid morbidity caused by micronutrients deficiency. Regular pancreatic insufficiency screening should be done annually in order to start PERT early.  Further research focusing on body composition, growth chart, protein intake, and PERT are needed to further improve the management of CF patient. Conclusion Nutritional intervention and PERT play an important role in prolonging CF patient survival. Both treatments should be initiated early with nutritional status close monitoring and tailored to each individual. Collaboration with parents and children is critical to warrant that CF patients followed the dietary advice.


Author(s):  
Nicky Heather

This chapter covers the nutritional management of cystic fibrosis. This includes discussion of the risk factors for malnutrition, assessment of nutritional status, assessment of nutritional requirements, and practical management. The chapter includes a section on pancreatic enzyme replacement therapy.


1977 ◽  
Vol 73 (5) ◽  
pp. 1023-1028 ◽  
Author(s):  
John B. Watkins ◽  
Ann M. Tercyak ◽  
Patricia Szczepanik ◽  
Peter D. Klein

2020 ◽  
Vol 17 ◽  
pp. 147997311990061 ◽  
Author(s):  
Bruce C. Trapnell ◽  
Su Chen ◽  
Rupal Khurmi ◽  
Amit Bodhani ◽  
Mudra Kapoor ◽  
...  

We investigated the relationship between self-reported adherence to pancreatic enzyme replacement therapy (PERT), nutritional status, and all-cause hospitalization in cystic fibrosis (CF) patients with a record of PERT use. Association of self-reported annual PERT use rate (adherence) with annual hospital admission rate (HAR) and annual total hospital nights (THNs) were analyzed for 5301 children (2000–2012) and 13,989 adults (2000–2013) from the CF Foundation Patient Registry. Multivariate linear regression was used to determine the association of HAR and THN with mean annual PERT use rate, cumulative PERT use rate, mean body mass index (BMI) (adult) or BMI percentile (pediatric), age, and sex. The median annual PERT use rate was 87% in children and 80% in adults. Statistically, higher annual PERT use, longer cumulative PERT, and higher BMI percentile (children) or BMI (adults) were significantly ( p < 0.0001) associated with lower annual HAR and fewer annual THN in children and adults. Female sex was associated with higher annual HAR and more annual THN in children and adults ( p < 0.05). Results indicate self-reported adherence to PERT, increased BMI, and male sex were associated with fewer hospital admissions and annual hospital nights in CF patients.


2000 ◽  
Vol 118 (4) ◽  
pp. A775
Author(s):  
Julia Schlichting ◽  
Nadine Schaeuble ◽  
Joachim Bargon ◽  
Wolfgang F. Caspary ◽  
Juergen Stein

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