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Weight Gain and Growth After Fundoplication in the Pediatric Patient: A Case Report in the Pediatric Patient With Cystic Fibrosis and Literature Review.
Gomes, A, Lapin, C, Ziegler, J
Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition. 2020;(4):745-754
Abstract
Gastroesophageal reflux (GER) is common among infants, but when symptoms become troublesome, that is defined as gastroesophageal reflux disease (GERD). Making a diagnosis of GERD is difficult because there is no gold standard. GERD can be especially problematic for infants with cystic fibrosis (CF). There are signs and symptoms (ie, malnutrition, recurring aspiration pneumonias, refusal to eat, wheezing, coughing, and asthma) in addition to invasive and noninvasive methods used to determine a diagnosis. The treatments for GERD span from nonpharmacological to surgical, with a laparoscopic Nissen fundoplication being the gold standard of surgical intervention. Although surgical interventions have been demonstrated to reduce symptoms associated with GERD, there is little known about the weight/growth-related outcomes. This case report discusses an infant with CF and GERD requiring multiple interventions and ultimately a laparoscopic Nissen fundoplication and the weight changes from presurgery to postsurgery. The case report is expanded upon with a structured literature review of fundoplication and growth studies. There were 4 available studies that assessed changes in weight/growth before and after fundoplication among children with GERD. Because of the heterogeneity of the nutrition outcomes used, the weight/growth benefits after fundoplication are unclear at this time. Further research in needed to assess the nutrition outcomes among patients with GERD requiring fundoplication.
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2.
Cystic Fibrosis as a Cause of Malabsorption and Increased Requirement of Levothyroxine.
Giuffrida, G, Magazzù, G, Campennì, A, Lucanto, MC, Trimarchi, F, Cannavò, S, Ruggeri, RM
Thyroid : official journal of the American Thyroid Association. 2020;(7):1095-1096
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3.
Isotretinoin therapy for the treatment of acne in patients with cystic fibrosis: a case series and review of the literature.
Bari, O, Paravar, T
Dermatology online journal. 2016;(3)
Abstract
BACKGROUND Cystic fibrosis (CF) is the most common severe autosomal recessive disorder in Caucasians. Viscous secretionstypically obstruct the lungs, pancreas, and gastrointestinal tract. As disease management improves, patients will increasingly seek care for conditions such as acne. Isotretinoin therapy for acne in patients with CF is controversial owing to concerns that the medication may exacerbate CF-related hepatic, pulmonary, and ocular complications. PURPOSE We describe two patients with CF treated with isotretinoin from our clinic and also provide a literature review of 11 similar cases. We describe patient outcomes, common complications, and the risks for severe adverse effects. MATERIALS AND METHODS The clinical courses of two patients with CF who were treated with isotretinoin for moderate-severe acne are presented. Using PubMed, we analyzed previous case reports of patients with CF who were prescribed isotretinoin and review complications associated with systemic retinoids. RESULTS Based on a synthesis of the literature and our own experience, it appears that isotretinoin therapy for CF patients with moderate-severe acne may be an appropriate option when clinically indi ated. If dermatologists monitor lab values and adverseeffects carefully, patients with CF can benefit from isotretinoin therapy.
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4.
Critical timing of gastrostomy insertion in a child with cystic fibrosis.
Grime, CJ, Greenaway, C, Clarke, S, Balfour-Lynn, IM
Paediatric respiratory reviews. 2015;:19-21
Abstract
Pulmonary exacerbations and malabsorption in children with cystic fibrosis (CF) can lead to faltering growth and poor weight gain. Children with a higher BMI (body mass index) show a slower decline in lung function. Our specialist CF centre has experienced a death following gastrostomy insertion in a young CF child, despite maximal medical intervention, which has made us reflect on our practice and the urgency with which we discuss the option for a gastrostomy to improve nutrition.
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5.
Pseudo-Bartter's Syndrome in Patients with Cystic Fibrosis: A Case Series and Review of the Literature.
Vilotijević-Dautović, G, Stojanović, V
Srpski arhiv za celokupno lekarstvo. 2015;(11-12):748-51
Abstract
INTRODUCTION Pseudo-Bartter syndrome (PBS) is characterized by hyponatremic, hypochloremic metabolic alkalosis that mimics Bartter syndrome but with no pathology in the renal tubules. We present five patients with cystic fibrosis (CF) and PBS. CASES OUTLINE Four children aged between three and five-and-one-half months with previously diagnosed CF and one aged 17 months with previously undiagnosed disease, were hospitalized during the summer season, with severe dehydration, oliguria, apathy and adynamia. Additionally, one of them had an ileostomy due to meconium ileus after birth. All children were on a diet without additional salt intake. Laboratory analysis on admission showed hyponatremia (115-133 mmol/L, mean 122.4 mmol/L), high plasma renin activity (229-500 pg/ml, mean 324 pg/ml) and metabolic alkalosis (pH 7.5-7.6, mean 7.56) in all the patients, and in four of them high blood level of aldosterone (74-560 pg/ml, mean 295.9 pg/ml), hypokalemia (2.3-2.8 mmol/L, mean 2.6 mmol/L), hypochloremia (59-71 mmol/L, mean 66 mmol/L) and low urinary sodium (5-12 mmol/L, mean 9 mmol/L). After intravenous rehydration followed by additional use of sodium and chloride in mean dosis of 1.78 mmol/kg per day, all the patients made a complete recovery. With advice for additional use of salt in the mentioned amount, the patients were discharged from the hospital. CONCLUSION PBS is one of CF complications, especially in infants and young children in situations accompanied by increased sweating and/or other causes of additional loss of sodium and chlorine. Sometimes, as was the case with one of our patients, PBS may be the initial presentation form of the disease.
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6.
Two successful pregnancies in a woman affected by cystic fibrosis: case report and review of the literature.
Ciavattini, A, Ciattaglia, F, Cecchi, S, Gagliardini, R, Tranquilli, AL
The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians. 2012;(2):113-5
Abstract
BACKGROUND Pregnancies in patients with cystic fibrosis (CF) are subject to an increased risk of complications. METHODS We have made a systematic review of the literature concerning pregnancies in women with CF to evaluate prognostic factors of pregnancy outcome such as lung function and nutritional status, also including and describing the case of a woman suffering from CF who had two pregnancies in her late thirties, both with a good outcome. RESULTS Thirteen case series and 22 case reports involving 523 pregnancies in 401 women were extracted. 83.1% of 516 pregnancies whose outcome was known resulted in the delivery of live births, with preterm birth rate of 24%. Miscarriage occurred in 6.3% and therapeutic abortion in 10% of pregnancies. Indirect mother's death occurred in seven cases. In our case report, the course of the second pregnancy proved to be much more difficult than the first, with preterm delivery probably related to a worsening of lung disease in the third trimester of pregnancy. CONCLUSIONS Pre-pregnant lung function as well as lung function deterioration, CF-related diabetes mellitus and weight gain in pregnancy, are parameters to consider in the counseling about the outcome of pregnancy.
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7.
Update on enteral nutrition support for cystic fibrosis.
Erskine, JM, Lingard, C, Sontag, M
Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition. 2007;(2):223-32
Abstract
Cystic fibrosis (CF) is an inherited disease affecting the respiratory, gastrointestinal, hepatobiliary, and reproductive systems. Nutrition status in persons with CF is often compromised due to increased energy needs, frequent infections, pancreatic insufficiency, lung disease, or CF-related diabetes. Maintaining good nutrition status has been associated with better pulmonary function, reduced hospitalizations, and increased longevity. Nutrition support as oral supplementation (used in >37% of the CF population) or tube feeding (used in >13% of the CF population) is often required for children and adults with CF. The purpose of this update is to describe current consensus and evidence for enteral nutrition support guidelines, reported complications of enteral feeding in the CF population, evidence of expected outcomes, and to discuss related areas requiring further research. A case report is provided to illustrate potential outcomes of aggressive enteral support.