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Purpose: Cystic-fibrosis-associated liver disease (CFLD) may lead to portal hypertension (PHT) and cirrhosis. Clinical signs and biochemistry of liver involvement are not discriminating. The aim of the study was to evaluate the performance of acoustic radiation force impulse (ARFI) with virtual tissue quantification in comparison with clinical signs, biochemistry and standard hepatic ultrasound (US) patterns., Materials and Methods: Virtual Touch Tissue Quantification, an implementation of US ARFI with shear-wave velocity (SWV) measurements was used in 75 children with cystic fibrosis (CF) and suspected CFLD to quantify hepatic stiffness. In each patient, ten measurements of SWV were performed on the right hepatic lobe. Patients were also evaluated by standard diagnostic tools (standard US, liver- and lung function tests, oesophagogastroscopy)., Results: Among CF patients, median SWV was significantly higher in patients with clinical, biochemical and US signs of hepatic involvement than in patients without US evidence of liver disease 1.08 m/s [(95% confidence interval (CI), 1.02-1.14]. Median SWV values in patients with portal hypertension, splenomegaly and oesophageal varices were 1.30 (95% CI, 1.17-1.43), 1.54 (95% CI, 1.32-1.75) and 1.63 (95% CI, 1.26-1.99), respectively. Differences were significant (p<0.001)., Conclusions: ARFI is an innovative screening technique able to help identify CFLD in children.

Three-dimensional reconstruction techniques have been used since the development of computed tomography. In skeletal radiology they have been used to better visualize fractures and spatial relationships between fragments in maxillo-facial surgery. Aim of this paper is to illustrate the most important reconstruction techniques and their application in skeletal radiology. Images can be obtained by means of a Spiral CT scanner with the following parameters: collimation/pitch/reconstruction interval 2/1/1. Acquired images are sent to a workstation with a software running on a Window NT platform Vitrea 2.01 (Vital Images, USA) equipped with Volume Rendering algorithm. SSD as well as MPR and curved reformatted reconstruction algorithms is available at the CT console. Three-dimensional reconstruction have been shown to be useful for the diagnosis and for a preoperative planning. Other applications of three-dimensional reconstruction techniques are prosthesis engineering and biomechanic research. 3D images help the communication between radiologist and surgeons. Technical advances in acquisition methods and in reconstruction algorithms are needed to improve image quality and use in skeletal radiology.

Lesions of cruciate ligament are common after knee trauma of different degree of gravity. Lesions evaluation is possible thanks to CT and MRI. Our purpose is to verify their value in diagnosis of cruciate ligaments lesions. We retrospectively evaluated 160 patients (80 CT and 80 MRI) with subsequent diagnosis of cruciate ligaments injury. CT and MRI were performed in the acute phase and in the subacute phase within 2-3 weeks from trauma. On the basis of our results CT and MRI shown a reduced sensitivity in acute phase owing to the intraarticular haemorrhage; in this phase MRI gives more information about fibres interruption. In subacute or chronic phase, both methods are correct in recognising the lesions but MRI is more sensitive and accurate. It is a real wish that, with more MRI machines diffusion, this method will be preferred in the study of cruciate ligaments both in acute phase and subacute-chronic phase.

Cystic Fibrosis (CF) is a recessive autosomic genetic disease with an incidence in mediterranean countries of about 1:3500 born alive. In Italy the considerable genetic variability makes it difficult to identify all the homozygous subjects and, consequently, to estimate the incidence of the disease in healthy carriers. The disease is evolutive and affects various systems, most of all the respiratory and gastrointestinal systems. Not many years ago, when the clinical definition of CF was first introduced, average survival did not exceed the pediatric age. Nowadays with ever advancing diagnostic and therapeutical techniques many CF patients survive until an adult age. It is therefore necessary to plan adequate health service interventions so as to satisfy as much as possible the needs of both the patients and their families. To this end data collected since 1.1.1988 by the Italian registry for CF (year of birth, sex, region of birth and residence, diagnosis procedures, results of sweat test, pancreatic insufficiency, DNA analysis, status: alive, dead, lost to follow up) of all the patients, diagnosed in the 18 Reference Centres and the 3 local Centres for CF, have proved to be extremely useful. Since the birth of the Registry on 31.12.1997, data relating to 2458 patients alive on 1.1.1988 and 1159 born during the last ten years, for a total of 3617 subjects (1756 females and 1861 males), have been recorded. As already mentioned a considerable increase in life expectancy of CF patients (from 1988 to 1990 the average age of death was 14 years, from 1994 to 1997 it was 19) and a consequent increase in the percentage of adult patients have been observed.

The Authors report their experience in the surgical rehabilitation of patients with complicated ileo- or colostomy. Mechanical and psychosocial implications as well as different rehabilitative methods are discussed. The results of a surgical protocol in the treatment of stomal diseases observed in 63 patients are herein reported. In 14 patients the surgical treatment was performed in general anaesthesia, while in 49 local anaesthesia was used. The latter was better tolerated by the patients. In conclusion, surgery should play a major role in this rehabilitation protocol, either in terms of prevention or definitive treatment.

Spontaneous bacterial peritonitis in patients with chronic hepatitis represents a very controversial matter in terms of frequency, pathology and treatment. This unusual complication mainly due to decreased immunological defences and ascitic fluid opsonic activity is not accepted by all the Authors as a rare event. However, there is agreement as far as management is concerned: cultural examination of the ascitic fluid and the relative antibiogram are the best tools in guiding the approach to an adequate antibiotic therapy.

We report a study of 128 patients, affected by cystic fibrosis, followed by Ospedale Bambino Gesù Center for diagnosis and therapy of cystic fibrosis. We evaluated, in a retrospective study, the principal symptoms at diagnosis of cystic fibrosis, underlining the frequent finding of not classically related symptoms. Median age at diagnosis was of 29 months, similar to that of other national centers. Furthermore, we report the percentages of diagnostic mistakes, which preceded cystic fibrosis diagnosis (22% of total). Considering the incidence of the disease in Italy (1:3000 live births), a careful valuation of related symptoms is necessary, even if gastrointestinal and/or respiratory disturbances are mild or underestimated, in order to reduce median age at diagnosis, still too high in Italy, compared to that reported by many North-European countries.

Cystic fibrosis (C.F.), a congenital lethal disease involving many organs, is responsible of chronic pulmonary disease and maldigestion. At the beginning symptoms can be feeble and diagnosis is often delayed, especially in those cases with an isolated pulmonary expression. It is demonstrated that early diagnosis and immediate prophylaxis of pulmonary infections and maldigestion improves survival. Thus a neonatal screening test is required. Although various attempts have been done, dating from 1968, there is no evidence, up to now, of a real utility of neonatal screening tests in C.F. The only test with a minor frequency of false negatives and positives is the RIA trypsin serum dosage to be executed within 3-5 days of life.

Authors report their experience of a population of 308 children affected by cow's milk allergy who presented gastrointestinal, respiratory and cutaneous symptoms. Diagnosis was based on challenge with cow's milk proteins and on laboratory specific tests (RAST, prick test) and non specific ones (PRIST, eosinophil count, 1-h xylose test, occult blood in the stools), following ESPGAN criteria. The patients were subdivided in 3 groups: a) patients with prevalent gastrointestinal symptoms; b) patients with prevalent cutaneous symptoms and c) patients with both, gastrointestinal and cutaneous symptoms. The sensitivity of the employed tests was evaluated either in the whole patient population or in the there group according to symptoms. Our data show a high sensitivity of the RAST for cow's milk (70%) versus a lower sensitivity of prick test (53%) when the whole patients population was considered. Moreover statistically important differences of the sensitivity of the various tests were found when the three groups of patients were considered, as well as when such a comparison was done in patients subdivided according to age (more or less than 6 months). Finally the concord between RAST and prick test was evaluated in the three groups.

IgA antigliadin antibodies (IgA-AGA) have been determined with an enzyme immunoassay in 2.141 pediatric patients. High levels of IgA were found in 98% of 53 celiac patients (1st biopsy), in 81% of 16 celiac patients after gluten challenge, while high levels of these antibodies were not found in 200 patients on gluten-free diet. Moreover high levels of IgA-AGA were found in 29% of 48 patients with normal jejunal biopsy and in 4% of 1.824 patients with gastrointestinal problems other than celiac disease. Our results confirm the data report in literature about the sensibility and the specificity of the IgA-AGA dosage as a screening test for celiac disease, but the possibility of false pathological and false normal values confirms the intestinal biopsy, as the main procedure for the diagnosis of celiac disease.

The authors report a phenotype-genotype correlation in a population of patients from center and south Italy affected by cystic fibrosis (CF). Thirteen (21%) of patients with pancreatic insufficiency (PI) were homozygous for delta F508, seventeen (27%) were delta F508 heterozygous with an associated unknown allele. Two other described mutations (N1303K, G542X) were also found in a heterozygote status and associated with a severe clinical expression of disease.

Ascites is a late and uncommon symptom of Chronic Granulomatous Disease (CGD) of childhood following liver damage with portal hypertension due to hepatic granulomatosis. The Authors describe two patients with CGD, in whom ascites was the main symptom in the first case and the initial symptom in the second case. Ascites in these cases was not due to liver fibrosis but it was a consequence of primary bacterial peritonitis.

The intestinal absorption of vitamin E is often thought to be closely correlated to that of fatty acids, but the biochemical mechanisms involved in the process are still not clear. For this reason, the present study aimed to assess the absorption of triglycerides and vitamin E, following oral administration, in 11 children with suspected malabsorption syndromes. Intestinal biopsy in 9 children showed the presence of a flat mucosa, whereas no changes were present in 2 patients. The results confirm the inverse correlation between the absorption of triglycerides and vitamin E, as if tocopherols used an independent system of transport. These findings contrast with those reported in the literature perhaps due to the low level of precision of colorimetric techniques previously used to assay vitamin E.

Prostanoid content prostaglandin E2 (PGE2) and thromboxane B2 (TxB2) and endocrine cells population were evaluated in jejunal biopsies from celiac children; findings were compared to active celiac patients on a challenge diet. Patients were divided as follows: Group A: 14 children with active untreated celiac disease; Group B: 7 celiac children on gluten challenge who had received diet therapy for the past 2 years; Group C: 8 normal control children. Jejunal biopsies were used for endocrine cell population measurement by immunocytochemistry, using a specific marker (chromogranin), and for prostanoid radioimmunological evaluation. The quantitative assessment of the endocrine cell population in Groups A and B revealed a significantly higher number of endocrine cells (20 +/- 11.5; 18.4 +/- 9.8 n. cell/visual field respectively) compared to Group C (8.44 +/- 2.3 n. cell/visual field) (p less than 0.05). In the jejunal extract the PGE2 content (341.8 +/- 82.3) ng/g) for Group A biopsies was significantly higher than that of Group C biopsies (93 +/- 23 ng/g) (p less than 0.05. The PGE2 content (69.4 +/- +13.2 ng/g) for Group B did not show any statistically significant change. In contrast, TxB2 content in jejunal biopsies from all three groups was not significantly different.

The authors describe six subjects with Reye's syndrome. All subjects died nevertheless the treatment (exchange-transfusions infusions of citrulline and ornithine). The autoptical studies showed cerebral oedema and fatty degeneration of the liver. Hepatic and seric OTC activity was measured in three patients: enzyme activity was virtually absent in one patient and normal in the other two. Instead in one patient was found partial CPS deficiency. However, Reye's syndrome is not only correlated with enzymatic deficiency of urea's cycle but sometimes also with toxic and metabolic causes.

IgG gliadin antibodies (AGA-IgG) were detected by immunofluorescence in 78 celiac children diagnosed by jejunal biopsy. AGA-IgG were also detected in patients affected by other gastrointestinal disease, in patients with diabetes, in health children and in children with normal mucosa. AGA-IgG were found in 81% of celiac patients, while they were not detected in patients affected by other intestinal or extraintestinal diseases, neither in healthy controls. It is conclude that determination of AGA-IgG is a very specific and sensitive enough screening test before small intestinal biopsy.

The results of four diagnostic tests have been critically evaluated (xylosemia, serum Ig A level, steatorrhea, X-Ray of the digestic tract) in 80 cases of atrophy of the small intestinal mucosa discovered by biopsy, and in 20 cases in which the biopsy has proved normal. In the celiac subjects the best correlation with the biopsy was found with xylosemia (90%), followed by serum Ig A (87%), by X-Ray of the digestive tract (86%) and finally by steatorrhea (59%). Even in cases without histological alteration of the mucosa, and xylosemia proved abnormal in 70% of the cases, the seric IgA resulted higher greater than 2 delta in 35%, the steatorrhea in 29% while the X-Ray of the digestive tract has never given a false diagnosis. The presence of false negative or positive results in all four tests (except the radiological tests) reconfirms the absolute necessity for biopsy of the digestive tract in the diagnosis of celiac disorder, and has led the AA. Carry out whenever possible the xylosemia, the steatorrhea and the serum IgA level because in none of the 80 cases of celiac disorder has there been a false negative result in all three such tests at the same time.