Partial Splenic Embolization: Successful treatment of hypersplenism, secondary to biliary cirrhosis and portal hypertension in cystic fibrosis

AbstractPartial Splenic Embolization (PSE) is a non-surgical treatment for hypersplenism. It has been reported only in a limited number of patients with Cystic Fibrosis (CF). We report a case of a female cystic fibrosis patient who developed hypersplenism at the age of 14 and underwent PSE. Long term results over a period of 14 years after the procedure are presented

Adiponectin and body composition in cystic fibrosis

AbstractThe aim of the study was to evaluate adiponectin (AD) serum concentrations in 43 stable CF patients and 27 healthy subjects and to correlate them with their nutritional status. Body Composition (Bioelectrical Impedance), visceral/subcutaneous adipose tissue (VAT-SAT) in CF patients (CT-scan at L4), insulin resistance (HOMA-IR) and AD serum concentrations (ELISA) were studied. CF patients and controls had comparable weight, height, %BF, %FFM, fasting glucose, insulin and insulin resistance. CF patients had significantly lower BMI-SDS. CF males had higher %FFM and total FFM and lower %BF and total BF than females (p<0.001). Serum AD was higher in CF patients than controls (11.53±5.37 vs. 9.07±4.41 μg/ml) and comparable between females and males. AD was lowest among young malnourished patients (8.06±1.85 μg/ml) and highest among young patients with normal nutrition (14.56±7.69 μg/ml). Patients with biliary cirrhosis had higher levels than patients with normal liver (10.52±5.49 vs. 14.04±4.52 μg/ml, p<0.05). AD correlated with %BF, %FFM, FFM (kg) (p<0.05).VAT was significantly increased in malnourished patients. AD was not affected by VAT. Conclusions: Adiponectin is higher in CF patients than healthy individuals. It is decreased in malnourished young patients and increased in patients with normal nutrition and in patients with liver disease. This may be attributed to the reduced BF and to the energy deficit inherent to the disease

CLINICAL APPLICATION OF IMMUNOLOGICAL MARKER AS DIAGNOSTIC AND MONITORING TESTSIN COELIAC DISEASE

IMMUNOLOGICAL MARKERS SUCH AS THE ANTIGLIADIN (AGA) ANTI - ENDOMYCIAL (EMA) ANDANTI - RETICULIN (ARA) ANTIBODIES ARE FREQUENTLY FOUND IN PATIENTS WITH UNTREATED COELIAC DISEASE. ANTIBODY LEVELS WERE MEASURED IN PATIENTS WITH COELIAC DISEASE DURING DIFFERENT PHASES OF GLUTEN - FREE DIET OR CHALLENGE AND IN CONTROL GROUPS, WITH THE PURPOSE TO EVALUATE THE DIAGNOSTIC SENSITIVITY AND PREDICTIVE VALUE OF THESE MARKERS. THE PRESENT STUDY SUPPORTS THE CONCEPT THAT WHEN ALL MARKERS ARE POSITIVE DURING THE FIRST EVALUATION IN CHILDREN WITH SUSPECTED COELIAC DISEASE, THE FINAL DIAGNOSIS IS COELIAC DISEASE. IMMUNOLOGICAL MARKERS REPRESENT A NON - INVASIVE AND VERY HELPFUL METHODOLOGY FOR THE DIAGNOSISOF COELIAC DISEASE AND, ALSO FOR MONITORING DIETARY COMPLIANCE OF PATIENTS CONFIRMED TO HAVE COELIAC DISEASE.ΣΤΗ ΜΕΛΕΤΗ ΑΥΤΗ ΠΡΟΣΔΙΟΡΙΣΘΗΚΑΝ ΑΝΤΙΣΩΜΑΤΑ ΕΝΑΝΤΙ ΤΗΣ ΓΛΙΑΔΙΝΗΣ (AGA) ΕΝΑΝΤΙ ΤΟΥ ΕΝΔΟΜΥΙΟΥ (ΕΜΑ) ΚΑΙ ΕΝΑΝΤΙ ΤΗΣ ΔΙΚΤΥΩΤΗΣ ΟΥΣΙΑΣ (ARA) ΣΕ ΑΣΘΕΝΕΙΣ ΜΕ ΚΟΙΛΙΟΚΑΚΗ ΣΕ ΔΙΑΦΟΡΑ ΧΡΟΝΙΚΑ ΔΙΑΣΤΗΜΑΤΑ ΠΕΡΙΟΔΟΥ ΣΤΕΡΗΣΗΣ Η ΠΡΟΚΛΗΣΗΣ ΜΕ ΓΛΟΥΤΕΝΗ ΚΑΙ ΣΕ ΟΜΑΔΕΣ ΕΛΕΓΧΟΥ ΜΕ ΣΚΟΠΟ ΝΑ ΥΠΟΛΟΓΙΣΘΕΙ Η ΕΥΑΙΣΘΗΣΙΑ ΤΟΥΣ, Η ΕΞΕΙΔΙΚΕΥΣΗΤΟΥΣ ΚΑΙ Η ΠΡΟΓΝΩΣΤΙΚΗ ΤΟΥΣ ΑΞΙΑ ΣΤΗΝ ΔΙΑΓΝΩΣΗ ΤΗΣ ΚΟΙΛΙΟΚΑΚΗΣ ΑΛΛΑ ΚΑΙ Η ΑΞΙΑ ΤΟΥΣ ΣΤΗΝ ΠΑΡΑΚΟΛΟΥΘΗΣΗ ΑΣΘΕΝΩΝ ΜΕ ΚΟΙΛΙΟΚΑΚΗ. ΑΠΟ ΤΑ ΑΠΟΤΕΛΕΣΜΑΤΑ ΑΥΤΗΣ ΤΗΣ ΜΕΛΕΤΗΣ ΠΡΟΚΥΠΤΕΙ ΟΤΙ Η ΣΥΜΒΟΛΗ ΤΩΝ ΑΝΟΣΟΛΟΓΙΚΩΝ ΔΕΙΚΤΩΝ ΤΟΣΟ ΣΤΗΝ ΔΙΑΓΝΩΣΗΟΣΟ ΚΑΙ ΣΤΗΝ ΠΑΡΑΚΟΛΟΥΘΗΣΗ ΑΣΘΕΝΩΝ ΜΕ ΚΟΙΛΙΟΚΑΚΗ ΕΙΝΑΙ ΣΗΜΑΝΤΙΚΗ. ΕΙΝΑΙ ΠΛΕΟΝ ΔΥΝΑΤΗ Η ΔΙΑΓΝΩΣΗ ΤΗΣ ΝΟΣΟΥ, ΧΩΡΙΣ ΒΙΟΨΙΑ ΛΕΠΤΟΥ ΕΝΤΕΡΟΥ ΥΠΟ ΟΡΙΣΜΕΝΕΣ ΑΥΣΤΗΡΕΣ ΠΡΟΥΠΟΘΕΣΕΙΣ, ΟΠΩΣ ΕΙΝΑΙ Η ΕΠΑΡΚΕΙΑ ΤΗΣ ΑΝΟΣΟΣΦΑΙΡΙΝΗΣ Α ΚΑΙ Η ΔΙΑΠΙΣΤΩΣΗΚΛΙΝΙΚΗΣ ΑΛΛΑ ΚΥΡΙΩΣ ΑΝΟΣΟΛΟΓΙΚΗΣ ΑΝΤΑΠΟΚΡΙΣΗΣ ΣΤΟΥΣ ΔΙΑΦΟΡΟΥΣ ΔΙΑΙΤΗΤΙΚΟΥΣ ΧΕΙΡΙΣΜΟΥΣ ΔΗΛΑΔΗ ΤΗΝ ΑΦΑΙΡΕΣΗ ΚΑΙ ΤΗΝ ΕΠΑΝΑΧΟΡΗΓΗΣΗ ΤΗΣ ΓΛΟΥΤΕΝΗΣ

Association of rs738409 Polymorphism in Adiponutrin Gene with Liver Steatosis and Atherosclerosis Risk Factors in Greek Children and Adolescents

Non-alcoholic fatty liver disease (NAFLD) shares several risk factors with atherosclerosis, as it is associated with components of the metabolic syndrome. However, genetic variations have also been linked to the risk of NAFLD, such as adiponutrin/patatin-like phospholipase domain-containing the protein 3 (PNPLA3) rs738409 polymorphism. The aim of the study was to determine the associations of thePNPLA3 rs738409 polymorphism with NAFLD and atherosclerosis risk factors in children and adolescents from northern Greece. A total of 91 children/adolescents who followed a Mediterranean eating pattern with no particular restrictions were studied. They were divided into three subgroups, according to their body mass index (BMI) and the presence or absence of liver disease. Diagnosis of NAFLD was based on a liver ultrasound, while the distribution of the PNPLA3 rs738409 polymorphism was investigated in all the participants. From the components of metabolic syndrome, only BMI, waist circumference, blood pressure, and the homeostasis model of insulin resistance (HOMA-IR) differed significantly between groups. The rs738409 polymorphism was significantly associated with BMI and NAFLD, while lipid values had no significant association with either NAFLD or gene polymorphism. This study shows that in Greekchildren, there is a significant association between the rs738409polymorphism in the PNPLA3 gene and hepatic steatosis, regardless of bodyweight

Mealtime Environment and Control of Food Intake in Healthy Children and in Children with Gastrointestinal Diseases

Parental feeding practices and mealtime routine significantly influence a child’s eating behavior. The aim of this study was to investigate the mealtime environment in healthy children and children with gastrointestinal diseases. We conducted a cross-sectional case–control study among 787 healthy, typically developing children and 141 children with gastrointestinal diseases, aged two to seven years. Parents were asked to provide data on demographics and describe their mealtime environment by answering to 24 closed-ended questions. It was found that the majority of the children had the same number of meals every day and at the same hour. Parents of both groups exerted considerable control on the child’s food intake by deciding both when and what their child eats. Almost one third of the parents also decided how much their child eats. The two groups differed significantly in nine of the 24 questions. The study showed that both groups provided structured and consistent mealtime environments. However, a significant proportion of children did not control how much they eat which might impede their ability to self-regulate eating. The presence of a gastrointestinal disease was found to be associated with reduced child autonomy, hampered hunger cues and frequent use of distractions during meals

Primary Intestinal Lymphangiectasia: Is It Always Bad Two Cases with Different Outcome

Primary intestinal lymphangiectasia (PIL) or Waldmann's disease is a rare protein-losing gastroenteropathy of unknown etiology. Less than 200 cases have been reported globally. Patients may be asymptomatic or present edema, lymphedema, diarrhea, ascites and other manifestations. We report two pediatric cases with PIL with extremely different outcome in a 3-year follow-up period. The first patient presented with persistent diarrhea, hypoalbuminemia and failure to thrive, while the second patient presented with an abrupt eyelid edema. Hypoproteinemia was the common laboratory finding for the two patients and upper gastrointestinal endoscopy established the diagnosis. The first patient relapsed five times during the follow-up period after the diagnosis had been made and required intravenous albumin administration and micronutrient supplementation. The second patient revealed normal gastrointestinal endoscopy 4 months after the diagnosis had been established; he followed an unrestricted diet and remained asymptomatic throughout the follow-up period. PIL can be either severe, affecting the entire small bowel, leading to lifetime disease, or sometimes affects part of the small bowel, leading to transient disorder

The Impact of Pulmonary Arterial Pressure on Exercise Capacity in Mild-to-Moderate Cystic Fibrosis: A Case Control Study

Background. Pulmonary hypertension (PH) is an often complication of severe cystic fibrosis (CF); however, data on the presence and impact of pulmonary vasculopathy in adult CF patients with milder disease, is very limited. Aim. To investigate, for the first time, the impact of systolic pulmonary arterial pressure (PASP) on maximal exercise capacity in adults with mild-to-moderate cystic fibrosis, without PH at rest. Methods. This is a Case Control study. Seventeen adults with mild-to-moderate CF, without PH at rest (cases) and 10 healthy, nonsmoking, age, and height matched controls were studied. All subjects underwent maximal cardiopulmonary exercise testing and echocardiography before and within 1 minute after stopping exercise. Results. Exercise ventilation parameters were similar in the two groups; however, cases, compared to controls, had higher postexercise PASP and decreased exercise capacity, established with lower peak work rate, peak O2 uptake, anaerobic threshold, and peak O2 pulse. Furthermore, the change in PASP values before and after exercise was strongly correlated to the parameters of exercise capacity among cases but not among controls. Conclusions. CF adults with mild-to-moderate disease should be screened for the presence of pulmonary vasculopathy, since the elevation of PASP during exercise might contribute to impaired exercise capacity

International prospective study of distal intestinal obstruction syndrome in cystic fibrosis: Associated factors and outcome

Background Distal intestinal obstruction syndrome (DIOS) is a specific complication of cystic fibrosis. Methods A study was performed in 10 countries to prospectively evaluate the incidence, associated factors, and treatment modalities in children and adults. Results 102 patients presented 112 episodes. The incidence of DIOS was similar in children and adults. Medical treatment failed only in cases of complete DIOS (11%). Children with meconium ileus had a higher rate of surgery for DIOS (15% vs. 2%, p = 0.02). Complete DIOS entailed longer hospitalisation (4 [3; 7] days vs. 3 [1; 4], p = 0.002). Delayed arrival at hospital and prior weight loss had a significant impact on the time needed for DIOS resolution. Associated CF co-morbidities for DIOS included meconium ileus (40% vs. 18%, p < 0.0001), exocrine pancreatic insufficiency (92% vs. 84%, p = 0.03), liver disease (22% vs. 12%, p = 0.004), diabetes mellitus (49% vs. 25%, p = 0.0003), and Pseudomonas aeruginosa (68% vs. 52%, p = 0.01); low fibre intake and insufficient hydration were frequently observed. Female gender was associated with recurrent DIOS (75% vs. 52%, p = 0.04), constipation with incomplete episodes (39% vs. 11%, p = 0.03), and poor patient compliance in taking pancreatic enzyme therapy during complete episodes (25% vs. 3%, p = 0.02). Conclusion DIOS is a multifactorial condition having a similar incidence in children and adults. We show that delayed arrival at hospital after the initial symptoms causes significant morbidity. Early recognition and treatment would improve the prognosis