Serum and salivary antiendomysium antibodies in the screening of coeliac disease.

BACKGROUND: Antiendomysium antibodies (EMA) detection in serum is the best screening test for coeliac disease (CD): in saliva it has not yet been assayed. Aims of this study are: to verify the presence of EMA in saliva collected with a not invasive technique; to evaluate the validity of serum and salivary EMA in CD screening. METHODS: We investigated 130 subjects divided into 3 groups: "A": 45 untreated CD patients (mean age 6.11); "B": 18 CD patients treated with a gluten free diet (mean age 13.2); "C": 67 controls (mean age 8.9). We performed the EMA test using the indirect immunofluorescence technique, in serum and in saliva concentrated samples. RESULTS: Our results show: sensitivity EMA serum 100%; specificity EMA serum 96.5%; sensitivity EMA saliva 46.5%; specificity EMA saliva 100%; pos. pred. value EMA serum 93.5%; neg. pred. value serum 100%; pos. pred. value EMA saliva 100%; neg. pred. value saliva 78.7%. CONCLUSIONS: Conclusion indicates a high specificity of salivary EMA and a high sensitivity of serum EMA, anyway biopsy is still recommended for diagnosis of CD.

[What do celiac children eat? Dietary analysis of a group of children with celiac disease on a diet]. / Che cosa mangiano i bambini celiaci? Analisi dietologica su un gruppo di celiaci a dieta.

A diagnosis of Coeliac Disease (CD) indicates a lifelong compliance to a gluten-free diet (GFD), which implies a change in deeply ingrained dietary habits and may cause dietary imbalances. We studied the dietary intake in a group of children with CD on GFD. CD was diagnosed according to Espgan criteria. Strict compliance to GFD was ascertained by Hydrogen breath-test. For each patient a thorough dietary history was obtained; the Recommended Dietary Allowances (RDA) 1986/1987--Istituto Nazionale della Nutrizione were used as reference measurements. 71.3% of our patients had a daily calorie intake lower than recommended (mean +/- 1SD = -110 +/- 389 kcal/day). Calorie deficiency was mainly due to a low carbohydrate intake (50.2 +/- 7% of daily calorie intake vs. 59% RDA; difference = -4.7 +/- 7%). Fast absorbed simple carbohydrates exceeded by 46% the recommended 10% ratio to complex carbohydrates. Daily fat intake was higher than RDA (+7.7%) in 94.1% of our patients, who obtained from fat 35.7 +/- 5.2% of their daily calorie intake vs 28% recommended. Saturated to unsaturated fat ratio was unbalanced towards saturated fat intake (2.3 +/- 1.1 vs 0.33 recommended). Coeliac children on a GFD have low caloric and carbohydrate intakes and a high fat intake. An unbalance towards simple sugar and saturated fat ingestion was detected. A lifelong protraction of these dietary habits may favour the onset of metabolic diseases in mature age.

[Hydrogen breath test in celiac disease: relationship to histological changes in jejunal mucosa]. / Breath-test all'idrogeno nella malattia celiaca: alterazioni in rapporto alle modificazioni istologiche della mucosa digiunale.

Hydrogen concentration in expired breath depends on the fraction of ingested carbohydrates unabsorbed by the small intestinal mucosa which reach the large intestine and are fermented by the colonic flora. The aim of this study is to assess whether in coeliac children breath hydrogen excretion reflects the histological changes in the jejunal mucosa. Hydrogen breath test was performed on 40 children (15 males 25 females) divided into three groups. Group I (controls): 9 children with symptoms suggestive of coeliac disease who, after the appropriate workup, were found to suffer from other gastrointestinal disorders and had abnormal jejunal mucosa. Group II: 14 children who had been diagnosed as coeliacs according to the ESPGAN criteria, were kept on a gluten free diet for a minimum of 6 months and had a normal jejunal mucosa. Group III: 17 coeliac children who ate small quantities of gluten or were on a normal diet. At histology, 10 of them showed a total and 7 a partial atrophy of the jejunal mucosa. Breath hydrogen levels were measured both at baseline and after ingestion of a 2% sorbitol solution in water, at 30 minute intervals for four hours. The peak hydrogen level and the total surface area under the hydrogen excretion curve were also assessed. Coeliac children on a gluten containing diet excrete significantly more H2 than controls or coeliacs on a gluten free diet. Patients with more severe histological lesions had higher peak H2 levels and greater total excretion areas. In coeliac children, sorbitol breath H2 test represents a simple noninvasive technique to detect impaired jejunal function and it should have an important role as a screening test and in assessing dietary compliance.

[Liver transplant in children. II]. / Il trapianto epatico in età pediatrica. IIa parte.

The family physician has a central role in preparing patients for transplantation and in the follow up. In the preoperative period adequate nutritional, vitamin intake and vaccinations are the mainstay of medical therapy. Pretransplant evaluation in a Liver Transplantation Centre is an essential part of the procedure, permitting an assessment of candidate's suitability and their position on the emergency list. During or immediately after the operation immunosuppression starts, according to personalized schedules and protocols. Among various complications, rejection must be promptly identified and treated. Resumption of a normal life style is the best indicator of a successful transplantation.

[Liver transplant in children. I]. / Il trapianto epatico in età pediatrica. I parte.

Hepatic transplantation is the only therapeutic choice for end-stage pediatric liver diseases. The survival improvement, registered in the last few years is mainly due to the employment of cyclosporine in therapy, but also to new and sophisticated surgical techniques and immunosuppressive drugs. The indications in children are: biliary atresia after unsuccessful Kasai procedure, paucity of intrahepatic bile ducts (of syndromic and not syndromic type), some metabolic diseases (alfa1 antitrypsine deficiency, hereditary tyrosinemia), post infective cirrhosis, acute fulminant hepatic failure, hepatic malignancies. Absolute contraindications include severe systemic illness, severe cardiac or kidney failure, thrombosis or abnormalities of caval and portal veins, systemic sepsis, HIV infection. Other drawbacks are mental deficiency and the inability of family to care for the child and follow therapy after discharge. Relative contraindications are: HBsAg positivity, HIV positivity without infection, malnutrition. Finally the scarcity of donors of liver of adequate size is an important limitation for transplant especially in childhood.

Efficacy of pancreatic enzyme supplementation in children with cystic fibrosis: comparison of two preparations by random crossover study and a retrospective study of the same patients at two different ages.

Two studies were performed in children with cystic fibrosis (CF) to compare the efficacy and tolerability of pancreatic enzymes prepared as enteric-coated microspheres with that of a conventional enzyme preparation. The parameters evaluated included the following: fecal fat excretion, coefficient of fat absorption, daily caloric intake, percent of diet as fats, proteins and carbohydrates, increase in height and weight, frequency and consistency of stools, palatability of the preparation, and patient compliance. The first study was an open randomized crossover trial of an enteric-coated microsphere preparation (Pancrease) versus conventional pancreatin given alone or with cimetidine. With Pancrease compared to conventional pancreatin, a significant improvement was observed in all the digestive parameters in addition to better patient compliance. In the second study, the response of a group of CF patients given Pancrease for at least 3 months (3-67 months) was retrospectively compared with the response to conventional pancreatin, which had been given to the same patients from 10 months to 8 years earlier. In comparison to conventional pancreatin, Pancrease provided better digestive efficacy and greater increases in the growth rate of teenage patients. With Pancrease, the number of daily dosage units is decreased even when fat intake is increased. No adverse reactions were seen with either of the enzyme preparations used in these studies.