Long-term use of tube feeding in children with cystic fibrosis: results from two Belgian CF centers.

BACKGROUND: Enteral tube feeding (ETF) is often used in an attempt to optimize the nutritional status. The aim of this study was to observe the long term effect of ETF and to compare the start of ETF with the current European guidelines on nutrition care in CF. METHOD: From all patients who received ETF (ETFp) between February 2000 and September 2016 in the Ghent University Hospital (GUH) or Brussels University Hospital (BUH), z-scores for body weight (W), height (H), growth velocity (GV) and BMI, FEV1%, and FVC% were retrospectively collected from the patients' medical record, 3 years before and 5 years after the year of ETF initiation. Gender, age, and pancreatic status matched controls were selected from the GUH database. RESULTS: All baseline (T0) measurements in ETFp were worse compared to controls. Only 11% of the controls had a Hz < -1.6 compared 58% of the ETFp. After the initiation of ETF a rapid weight gain was noted until the second year (T + 2:-1.9 (-2.8; -1.0) vs. T0:-2.7 (-3.2; -2.1) (p = 0.01) with a stabilization afterwards. A rapid GVz increase was noted at T + 1:1.0 (-0.8; 1.9) vs. T0:-1.5 (-2.0;-0.3). After the start of ETF until T + 3, a stabilization of FEV1% was noted. However, compared to controls, it remained significantly lower (p < 0.05). CONCLUSION: ETF as a nutritional intervention has its effect on weight, height, GV, and BMI. To our knowledge this is the first study that describes the evolution of growth in ETFp. The effect on GV argues for a faster introduction of ETF in malnourished children with CF.

Acid-base disturbances in dehydrated patients with cystic fibrosis : four case reports with review of literature.

Most episodes of vomiting, reduced intake and diarrhoea in children can be evaluated and treated without additional tests. However, when the degree of clinical dehydration is not in line with the patient's medical history, other diagnoses should be suspected. In the presence of a hyponatraemic hypochloraemic metabolic alkalosis, cystic fibrosis (CF) should be included in the differential diagnosis, especially if there is failure to thrive even in the absence of respiratory symptoms. Furthermore, young patients diagnosed with CF have a higher risk for an acute electrolyte decompensation caused by increased salt and fluid losses. We present 4 paediatric cases to raise the awareness of electrolyte disturbances in CF patients.

ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis

"BACKGROUND: Malnutrition is both a frequent feature and a comorbidity of cystic fibrosis (CF), with nutritional status strongly associated with pulmonary function and survival. Nutritional management is therefore standard of care in CF patients. ESPEN, ESPGHAN and ECFS recommended guidelines to cover nutritional management of patients with CF. METHODS: The guidelines were developed by an international multidisciplinary working group in accordance with officially accepted standards. The GRADE system was used for determining grades of evidence and strength of recommendation. Statements were discussed, submitted to Delphi rounds, reviewed by ESPGHAN and ECFS and accepted in an online survey among ESPEN members. RESULTS: The Working Group recommends that initiation of nutritional management should begin as early as possible after diagnosis, with subsequent regular follow up and patient/family education. Exclusive breast feeding is recommended but if not possible a regular formula is to be used. Energy intake should be adapted to achieve normal weight and height for age. When indicated, pancreatic enzyme and fat soluble vitamin treatment should be introduced early and monitored regularly. Pancreatic sufficient patients should have an annual assessment including fecal pancreatic elastase measurement. Sodium supplementation is recommended and a urinary sodium:creatinine ratio should be measured, corresponding to the fractional excretion of sodium. If iron deficiency is suspected, the underlying inflammation should be addressed. Glucose tolerance testing should be introduced at 10 years of age. Bone mineral density examination should be performed from age 8-10 years. Oral nutritional supplements followed by polymeric enteral tube feeding are recommended when growth or nutritional status is impaired. Zinc supplementation may be considered according to the clinical situation. Further studies are required before essential fatty acids, anti-osteoporotic agents, growth hormone, appetite stimulants and probiotics can be recommended. CONCLUSION: Nutritional care and support should be an integral part of management of CF. Obtaining a normal growth pattern in children and maintaining an adequate nutritional status in adults are major goals of multidisciplinary cystic fibrosis centers."

Gastro-intestinal manifestations in cystic fibrosis patients.

Cystic fibrosis (CF) is a life-limiting disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR). This defective chloride channel, present in different organ systems such as respiratory system, gastrointestinal tract, reproductive system and sweat glands, disturbs the ion and water transport over the membranes leading to the well known CF symptoms. CF has outgrown paediatric care, as half of CF patients are currently adults. The CF gastrointestinal tract has its own particularities. Some gastrointestinal manifestations are the direct consequence of the CFTR defect whilst others are secondary to treatment. The gastrointestinal diseases are classified according to the way they usually present in symptoms at diagnosis, acute and chronic abdominal pain and silently evolving conditions. (Acta gastroenterol. belg., 2016, 79, 481-486).

Three-dimensional building detection and modeling using a statistical approach.

In this paper, we address the problem of building reconstruction in high-resolution stereoscopic aerial imagery. We present a hierarchical strategy to detect and model buildings in urban sites, based on a global focusing process, followed by a local modeling. During the first step, we extract the building regions by exploiting to the full extent the depth information obtained with a new adaptive correlation stereo matching. In the modeling step, we propose a statistical approach, which is competitive to the sequential methods using segmentation and modeling. This parametric method is based on a multiplane model of the data, interpreted as a mixture model. From a Bayesian point of view the so-called augmentation of the model with indicator variables allows using stochastic algorithms to achieve both model parameter estimation and plane segmentation. We then report a Monte Carlo study of the performance of the stochastic algorithm on synthetic data, before displaying results on real data.