The nutritional status and factors contributing to malnutrition in children with chronic pancreatitis.

BACKGROUND/OBJECTIVES: The present study was undertaken to determine the prevalence of malnutrition among children with chronic pancreatitis (CP). Furthermore, we aimed to evaluate the relationship between etiological factors of CP, its clinical characteristics, and the severity of malnutrition. METHODS: The study included 208 children with CP (113 girls and 95 boys; mean age: 10.8 years, range: 1.6-18 years), hospitalized at our center between 1988 and 2012. The severity of malnutrition was graded on the basis of Cole's ratios, and its prevalence was analyzed according to the etiological factors of pancreatitis. Moreover, the analysis of discrimination was performed to identify the factors contributing to malnutrition among the following variables: age at CP onset, duration of CP, number of CP exacerbations, the number of ERCPs performed, the grade of pancreatic damage documented on imaging, co-occurrence of diabetes, and the results of 72-h fecal fat quantification. RESULTS: We documented features of malnutrition in 52 (25%) children with CP, including 36 (17.3%) patients with moderate malnutrition, and 2 (0.96%) with severe malnutrition. There was no significant difference in the prevalence of malnutrition between groups of patients with various etiological factors of chronic pancreatitis. The age at CP onset showed the best discrimination ability of malnourished patients: the mean age at disease onset in a subgroup of malnourished children was significantly higher than in children with Cole's index >85%. CONCLUSIONS: A considerable percentage of children with CP can suffer from clinically significant malnutrition. Later age at CP onset predisposes to development of malnutrition.

Eosinophilic esophagitis in children: frequency, clinical manifestations, endoscopic findings, and seasonal distribution.

PURPOSE: Eosinophilic esophagitis (EoE) is a chronic disease characterized by eosinophilic infiltration of the esophageal mucosa, which is associated with clinical and endoscopic manifestations. The objective of our study was to determine the frequency of EoE and to outline the clinical manifestations of EoE in Polish children. MATERIAL/METHODS: Ten large regional pediatric gastroenterology centers participated in the study. A database of endoscopy reports from January 2004 till December 2009 was reviewed. A total of 35,631 esophagogastroduodenal endoscopy studies in children, aged from 4 months to 18 years, were performed. Data pertaining to the children's age, gender, indications for endoscopy, clinical findings and histopathology diagnosis were made. RESULTS: In 84 children (20 girls and 64 boys), aged between 4 months and 18 years, EoE was diagnosed. This constituted one case per 424 endoscopic studies. In children with changes in the esophageal mucosa the frequency of EoE was higher and reached one case per 73 children. The most frequent symptoms of EoE differed between the younger (1-6 years old) and older children (aged 13-18 years old). Feeding aversion, vomiting and/or regurgitation were most frequently observed in the younger children, while in older children: abdominal pain, dysphagia and chest pain. Granular mucosa, longitudinal furrows, and mucosal rings belong to the findings most often observed in endoscopic studies. EoE was more frequently diagnosed in the spring (45.2%) and summer (28.5%). CONCLUSIONS: EoE was diagnosed in every age, with frequency of 1/424 gastrointestinal endoscopies, more frequently in boys than in girls.

Beneficial effect of liver transplantation on bone mineral density in small infants with cholestasis.

Reports of bone mineral density in children after liver transplantation are few. Eleven cholestatic children were analyzed before and 6 months after liver transplantation. No changes in serum levels of calcium, alkaline phosphates, or 25OHD were observed before versus after LTx. The serum levels of phosphorus and 1-25(OH)2D3 as well as total bone mass density and Cole index were significantly increased after liver transplantation.

The past and present of the Polish paediatric gastroenterology, hepatology and nutrition.

Gastroenterology has emerged from paediatrics as a separate discipline after 1978, due to the development of basic sciences, i.e., biochemistry, immunology, pathomorphology and introduction of miniaturized endoscopic and radiological equipment. This paper describes the most significant achievements in the areas of gastroenterology, hepatology, and nutrition in children in particular medical centres in Poland. It also discusses the role of the Polish Society for Paediatric Gastroenterology, Hepatology and Nutrition, the role of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN), and the role of other Scientific Societies and Foundations supporting the development of science and education. The emphasis has been placed upon utilitarian research and education with regard to the management of children with gastrointestinal and hepatic disease.

Further delineation of the classical Smith-Lemli-Opitz syndrome phenotype at different patient ages: clinical and biochemical studies.

We present here extensive clinical and biochemical data on thirteen SLOS (type I) patients with proven defect in cholesterol biosynthesis for further delineation of the classical SLOS phenotype at different patient ages.

[Evaluation of nonspecific inflammatory bowel disease in children using disease activity scoring systems]. / Ocena przebiegu nieswoistych zapalen jelit u dzieci wedlug systemu punktowego.

Scoring systems for assessment of clinical activity of IBD were used to evaluate 62 children with IBD (14 with CD, 35 with CU and 13 with CNS). The PCDAI system was most effective in children with CD. In children with CU, both modified Truelove-Witts and Rachmilewitz indexes are equally effective, however use of the Truelove-Witts scale is simpler. The Rachmilewitz scale is better in distinguishing between UC and CNS.

[Giant-cell hepatitis in a 10-year-old boy]. / Olbrzymiokomórkowe zapalenie watroby u 10-letniego chlopca.

In this paper we present a 10-year-boy with viral giant-cell hepatitis with a immune component. We observed clinical, biochemical and histological improvement after treatment.

[Changes in the gastrointestinal system of children with inflammatory systemic connective tissue diseases]. / Zmiany w ukladzie pokarmowym u dzieci chorych na zapalne ukladowe choroby tkanki lacznej.

Functional and morphological changes of the gastrointestinal system were assessed in patients suffering from juvenile chronic arthritis, juvenile systemic erythematosus and juvenile systemic scleroderma. The results of endoscopic investigation of the gastrointestinal tract showed inflammatory changes and cardiac and pylorus atony. Ultrasonography results suggested hepatic and pancreatic inflammatory changes, steatosis and/or amyloidosis. The above-mentioned changes were confirmed by morphological investigation (biopsy and/or autopsy). The results of the capacity tests performed in JCA (systemic form) patients revealed an impairment of liver detoxication function, and pancreas and bowel functional capacity as well. Additionally, intolerance against lactose, casein and alpha- and beta-lactoglobulin was found in most of the patients. Some symptoms of malnutrition were observed in all of them. Such factors as underlying disease severity, adverse drug effects, environmental factors (infections, nutrition) were taken into consideration as probable causes of the gastrointestinal system damage.

[Preliminary evaluation of the usefulness of passage time along the alimentary tract in the diagnosis of constipation in children]. / Ocena wstepna przydatnosci czasu pasazu przez przewód pokarmowy w diagnostyce zaparc u dzieci.

In children with constipation the usefulness of passage time along the large bowel was assessed, limiting the number of performed rectal enemas. The preliminary results suggest that it is possible to answer the clinician's questions - whether the passage time is prolonged, and if so, to determine in what segment of the large bowel. This method may be treated as a screening test in the diagnosis of constipation in children.

Internal organ involvement in juvenile connective tissue disease.

Using noninvasive investigative techniques, cardiac, pulmonary and gastrointestinal abnormalities were found in patients with all types of connective tissue diseases. Impedance rheography suggested impaired myocardial function. Abnormalities of pulmonary function included a decrease in diffusing lung capacity for CO (DL) values followed by restrictive ventilatory defects and mild alveolar hyperventilation. The results of endoscopic investigation of the gastrointestinal tract showed inflammatory changes. Ultrasonography revealed dyskinesis of the ducts of the gallbladder and some hepatic and pancreatic structural changes.

[Recurrent abdominal pain in children]. / Nawracajace bóle brzucha u dzieci.

In the paper the results are presented of an analysis of the causes of recurrent abdominal pain in 294 children hospitalized at the Gastroenterology Department, Childrens Health Centre, and the accepted diagnostic programme is evaluated. The children were referred to the CHC, as a rule with a suggestion of organic causes of recurrent abdominal pain. On the basis of carried out examinations the following was diagnosed: in 111 children organic causes (37.8% of the studied group), in 94 children recurrent abdominal pain of functional aetiology (32%), in 53 children psychological background (18%), and in 36 children mixed organic and functional background. These proportions between those groups is the result of the selection carried out at earlier stages of the diagnostic process. In the paper, the correctness and validity of the accepted diagnostic programme are also demonstrated.

[Clinical-morphologic analysis of children dying of intoxication with Amanita phalloides]. / Analiza kliniczno-morfologiczna dzieci zmarlych z powodu zatrucia muchomorem sromotnikowym.

The results were subjected to analysis of 23 autopsies carried out in children dying of intoxication with Amanita phalloides. The correlation of the pathomorphological findings with the clinical picture and biochemical pattern was evaluated. The morphological changes were also presented in organs other than the liver which is recognized as the target organ for Amanita phalloides toxins.

Evaluation of the liver blood flow in the children with portal hypertension.

The aim of the study was to evaluate the liver blood flow at different stages of hepatitis B as compared with prehepatic block. The examination was performed on 79 children aged 1-17 years who were divided in two groups. Group 1 consisted of 20 children with HBV chronic active hepatitis B: 10 children with liver efficiency (Gr. 1A) and 10 with liver cirrhosis (intrahepatic block) Gr. 1B. Group 2 comprised 59 children with prehepatic block. The liver blood flow was assessed with the help of a radioisotope liver scintiscan by the first flow technique using 99mTc-DTPA. The ratio of portal to total liver blood flow (HPI) and time of portal blood flow (T1/2) were estimated. In children from group 1A the HPI mean was 57% (N over 75%) and T1/2 was 7-8 sec (N4-7 sec) depending on age. In Group 1B the HPI mean was 25% and T1/2 was 9-13 sec. In most of the children with prehepatic block HPI was low (mean 22%) and was similar to that in children with cirrhosis due to HBV. The arterial blood flow increased while HPI showed a distinct decrease.

[Evaluation of selected parameters in calcium and phosphate metabolism in acute liver failure following poisoning with Amanita phalloides]. / Ocena wybranych parametrów gospodarki wapniowo-fosforanowej w ostrej niewydolnosci watroby po zatruciu muchomorem sromotnikowym.

Serum calcium, phosphate and 25-OHD8 levels as well as calcium and phosphate urine excretion were determined in 21 children between 5 and 7 days after poisoning with Amanita phalloides. Hypophosphatemia was seen in children with the symptoms of moderate intoxication whereas hypocalcemia in severely poisoned patients. Phosphates deficit should be corrected early in all patients poisoned with Amanita phalloides.

["Munchausen syndrome by proxy" as an example of diagnostic error]. / Zespól "Munchausen by proxy" jako szczególny przyklad bledu diagnostycznego.

A case of the girl who underwent multiple hospitalizations is presented. Gastrointestinal disorders were seen in the infancy together with skin rash of allergic type, hypoglycaemia without any clear reason in the fourth year of age, and polyuria with hyponatremia and hypokalemia since the sixth year of age. Mother's lack of concern was unexplainable in view of the deteriorating child's health. Samples of the urine and faeces supplied by the mother have shown the laboratory findings suggesting that potassium chloride was added to the faeces and natrium hydrocarbonate--to the urine. Urine collected during polyuria contained large quantities of furosemide. Long-term follow-up, numerous examinations and performed tests have led to the diagnosis of the particular form of the ill-treated child syndrome, so-called "Munchausenn by proxy" syndrome.

[Changes in vitamin D metabolism in children following partial intestinal resection]. / Veränderungen des Vitamin D-Stoffwechsels bei Kindern nach partieller Darmresektion.

Six children (five after partial intestinal resection, one after colectomy) were studied with respect to absorption and metabolism of vitamin D. The results showed decreased 25-OH-D3 serum concentrations below 20 ng/ml (in two patients even below 10 ng/ml), possibly due to disturbed enterohepatic circulation of vitamin D metabolites.