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Nutrition deficiencies are common in children with chronic liver disease. To determine whether age, hepatic dysfunction, or energy intake influences this malnutrition, we evaluated the nutritional status of 49 children aged 2.5 mo to 13 y (mean: 35 mo; median: 12 mo). The children were divided into two groups according to age: group 1-29 patients aged < or = 1 y (mean: 7 mo; median: 7 mo); and group 2-20 patients > 1 y (mean: 75 mo; median: 59 mo). Hepatic dysfunction was defined according to the Malatack criteria. Seventy-two-hour dietary intakes were recorded by a nutritionist. Nutritional status was assessed by anthropometric measures when the patients were enrolled on the waiting list for liver transplants. We evaluated the following indexes: weight, height, fat body mass, and lean body mass on the basis of height-age (age at which height reached 50th Italian height percentile). Mean height Z scores were low in both groups, but the difference was not significant. Mean weight Z scores and mean percentages of fat body mass were significantly lower (P < 0.001) in group 1 than in group 2 patients. In group 2, lean body mass and fat body mass were significantly lower (P < 0.05) in patients with moderate-to-severe hepatic failure than in patients with mild hepatic dysfunction. The mean energy intake was in the range of the recommended daily allowances for age but was insufficient for both groups of patients. The evidence of significant acute and chronic malnutrition confirmed the need for nutritional support, especially for younger and older children with moderate-to-severe hepatic dysfunction. We emphasize the necessity of accurate assessment of nutritional status by simple anthropometric measurements to be sure of the effects and adequacy of the nutritional intervention.

Systemic virus infection impairs the steady state of auxin hormone in plant with consequent morphogenetic alterations. Most reports indicate a reduction of auxin activity in diseased plants, generally associated with stunting, but a substantial increase in auxin activity has sometimes been observed in cases of severe symptomatology. Treatments of virus-diseased plants with exogenous auxins may inhibit virus replication and reduce symptom severity, although results have been obtained by empirical application and must therefore be considered with caution. There is no information on how virus infection alters auxin metabolism or how this alteration affects both plant growth and development. The considerable progress reached in auxin biochemistry now allows more accurate re-investigations of this important relationship.

The paediatric liver transplantation programme was started, at our institution, in 1988 and since then 75 children have received 79 transplants, which corresponds to about 85% of all the paediatric liver transplantations performed in Italy. The main indications to transplantation were biliary atresia, familiar cholangiopathies and metabolic diseases. The patients came from all the regions of Italy, except for 2 of them who came from abroad. Patients with malnutrition and impaired growth underwent an aggressive nutritional programme whilst waiting for transplantation. The transplants were performed using whole grafts, reduced size grafts and, recently, with grafts from split livers, in 7 cases. Immunosuppression was based on a double drug therapy with Cyclosporine and low dose steroids. Steroid resistant acute rejection was treated, with OKT3 until 1993 and with conversion from Cyclosporine to FK506, thereafter. Interventional radiology was most useful in treating biliary complications. The introduction of Gancyclovir greatly reduced the incidence and severity of cytomegavirus related complications. Three years survival after liver transplantation was 62% for children under 2 years of age and 78% for those between 2 and 15 years. These results do not differ substantially from those reported by the European Liver Transplant Registry.

Hypertrophic scars and Dupuytren's disease are characterized by the presence of modified fibroblasts or myofibroblasts which are allegedly responsible for tissue retraction and excessive connective tissue production. gamma-Interferon, a cytokine produced by T-helper lymphocytes, has been shown to decrease fibroblast replication, alpha-smooth-muscle actin (the actin isoform characterizing myofibroblasts) expression, and collagen production. We have investigated in an open pilot study the possibility that intralesional injections of gamma-interferon exert a beneficial effect on the evolution of hypertrophic scars and Dupuytren's disease. In the 14 selected patients, gamma-interferon decreased the symptoms and the size of the lesions of both diseases; in hypertrophic scars, immunofluorescence examination showed that alpha-smooth-muscle actin expression also was decreased in myofibroblasts. Moreover, in fibroblasts cultured from 4 patients with hypertrophic scars, gamma-interferon decreased replication and alpha-smooth-muscle actin expression in vitro. Our results suggest that gamma-interferon could represent a useful adjunct to the nonsurgical therapy of hypertrophic scars and Dupuytren's disease. Larger controlled clinical studies, however, should test the validity of these preliminary observations.

For patients with cerebral palsy, chronic drooling is the result of an abnormality in the cerebral control of orofacial motor functions. Sialorrhea is a troublesome problem for these patients and their attendants. Among various surgical techniques, rerouting of the Stensen's ducts and bilateral excision of the submandibular salivary glands, as described by Wilkie, gives good results. Transposition of the submandibular gland ducts in the tonsillar fossae is an easier procedure but less favorable. We present 19 patients treated by these two methods.

Aspecific chronic diarrhea is one of the most common causes of protracted diarrhea in infancy. The pathology is characterised by an increased frequency of daily bowel movement with half-formed feces; it is not associated to malabsorption syndrome or enteric infections and appears to be correlated to an unbalanced diet and changes in microflora involved in the fermentation processes. A controlled randomised single-blind clinical trial was performed, using commercially available crystalline lactulose and lactic enzymes, in order to normalise the modified intestinal ecosystem. Twenty-four children (mean age 21.76 months) affected by aspecific chronic diarrhea were studied; weight and height parameters were not influenced. Patients were first divided by age and then randomly divided into two groups of 12. Group 1 received treatment with crystalline lactulose (Laevolac crystals, BBR) and Group 2 received lyophilised Lactipan. In both cases treatment continued for 15 days. At the end of the trial all subjects showed a complete remission of intestinal disorders. Children in Group 1 showed more fully formed feces following treatment. Fecal pH values were considerably reduced only in those subjects treated with crystalline lactulose. In the latter subjects H2 excretion diminished during the trial, a fact which may probably be attributed to reduced colic pH values. On the basis of these results, it is possible to conclude that both treatments proved efficacious. The re-establishment of a balanced intestinal ecosystem remains the choice treatment for subjects affected by aspecific chronic diarrhea.

Acidic and basic pathogenesis-related proteins (PR-Ps) were extracted from the intercellular fluid (IF) of soybean leaves, locally infected with tobacco necrosis virus and showing necrotic local lesions. Proteins were detected by two-dimensional polyacrylamide gel electrophoresis (2D-PAGE) using PhastSystem and precast commercially available gels. Extracts from healthy leaves were run as controls. PR-Ps were first run under native PAGE conditions or isoelectric focusing (IEF), the gels stained with Coomassie Blue, then run under sodium dodecyl sulfate (SDS)-denaturing conditions and finally stained with silver. Ten major acidic PR-Ps were separated; their Mr's were close to those found by conventional PAGE. Their isoelectric points ranged from 3.5 to 5.0. Ten basic PR-Ps were separated and their Mr's estimated. None of these acidic or basic soybean PR-Ps was a glycoprotein. PAGE with PhastSystem and precast gels gives reliable results, comparable with those from conventional 2D-PAGE, with simpler experimental procedures. By electrophoresing Coomassie-stained gels with SDS in the second dimension, we were able to control the first-dimensional separation and to avoid laborious protocols generally adopted with unstained gels.

Sodium salicylate reduced both the size and viral antigen content of non-self-limiting necrotic lesions produced by tobacco necrosis virus (TNV) in detached tobacco leaves during the partly localized reaction to virus. The antiviral effect of salicylate occurred at concentrations close to the limits of toxicity, and depended on the timing of administration. Both viral antigen accumulation and lesion size were strongly inhibited by a continuous supply of salicylate before or just after virus infection. Salicylate treatment did not prevent TNV accumulation when given after the establishment of infection or when arrested 24 h after TNV inoculation. Both procedures, however, did limit lesion enlargement. These results constitute a limit for the use of salicylate as a chemotherapeutic agent but do not exclude its use in limiting the pathogenetic effects of the virus. The salicylate treatments induced the formation of four pathogenesis-related proteins (PRs). The PRs formation was stimulated during the first 8 h of treatment and persisted for some time after the salicylate supply was discontinued. No correlation was found between the presence of PRs and the reduction of TNV accumulation: low salicylate concentrations (0.25 mM) inducing the formation of the PRs did not induce resistance against the multiplication and/or cell-to-cell spread of TNV.

Giardia lamblia is the first protozoan to be identified and recognized as an important pathogen in human disease. We studied 8 pediatric patients with giardiasis in order to examine the clinical spectrum, the structural changes of the small intestinal mucosa and mainly the protozoan's ultrastructural features. The most common clinical manifestations were diarrhea, abdominal pain, anorexia, vomiting, failure to thrive. Infection was confirmed by excreted cysts in the stools in one patient, by the presence of trophozoites in duodenal aspirate and on jejunal mucosa. Giardiasis was not associated with hypogammaglobulinemia in our patients and no or only slight mucosal abnormalities were present in jejunal biopsies, except one which showed a flat mucosa. Specimens for transmissions and scanning electron microscopy were taken. We could establish the protozoan's features, its normal distribution, its relationship to intestinal mucosa and structural indications of the normal reaction of intestine with the use of ultrastructural techniques. The trophozoites colonized the proximal intestine, adhered to microvilli of columnar cells near the bases of villi, wedged or lodged in mucus. The sticky mucus producing an effective diffusion barrier to nutrients could explain malabsorption phenomena. Numerous intraluminal lymphocytes were seen, suggesting an immune response. These observations indicate that in giardiasis the clinical spectrum and structural changes of the small intestinal mucosa vary widely, suggesting a different reaction of immune system and/or a different degree of infection.

We have identified a patient with a condition called Hypophosphatemic Bone Disease (HBD). This disorder of phosphate metabolism is unrecorded by Dent in his final classification of metabolic bone diseases and has been described by Scriver C.R. and coll. Although the condition is in same ways analogous to X-Linked Hyophosphatemia (XLH), there are important differences between the two diseases. For example, there is selective impairment in the tubular reabsorption of phosphate in HBD but the defect is less severe and it is clearly different from that described in XLH. Clinical manifestations of HBD appear in infancy, but the dwarfism and the bone changes are less severe than in XLH at comparable concentrations of plasmatic phosphorus in the two diseases. While in both conditions there is osteomalacia of endostal trabecular bone, only in XLH is florid rickets present, affecting the epiphyses and compromising linear growth. The phosphaturic response to PTH infusion is abnormal in qualitative aspects, but it is present in HBD, and this differs considerably from that described in XLH. The treatment with oral load of phosphates and 1,25(OH)2D3 in every patient with HBD, and so in our patient, is accompanied by increase in serum phosphorus, with improved tubular reabsorption of phosphate anion and a fall of hydrossiprolinuria with bone healing; this combination of responses is not present in XLH.

The Great Arteries Transposition corrected by Mustard's repair is often complicated with inferior vena cava obstruction with consequent intestinal lymphangiectasia and protein-losing enteropathy. We report a case of a 9 years old boy who underwent Mustard's repair and presented diarrhoea, abdominal pain, hypoproteinemia and ascites. The fecal elimination of 51Cr-labeled albumin was remarkable increased and the intestinal biopsy showed a noteworthy lymphangiectasia due to an hemodynamic overload. Therefore subjects with Great Arteries Transposition corrected by Mustard's repair should be valued in the post-surgical follow-up by 51Cr-labeled albumin fecal elimination and by intestinal biopsy, already sensitive in asymptomatic phase.

The peptic disease is not the commonest cause of recurrent abdominal pain, among the organic aetiology. Only recently the gastric disease as cause of abdominal pain has been described in pediatric age. We have studied 11 children aged 6 to 12 years, who were examined for recurrent abdominal pain. We found that this symptom was due to chronic gastritis. We have valued these patients on the basis of the clinical manifestations, endoscopic and biopsy findings. A family history of peptic disease was found to be highly significant. We can therefore consider the gastritis as a possible organic aetiology of recurrent abdominal pain after ruling out any other organic causes.

We describe three cases of juvenile intestinal polyposis in children aged 9-13 years. Ulcerative colitis in two of them and Crohn's disease in one of them were initially diagnosed. These subjects came to us for symptom persistence in spite of medical (sulfasalazine and or corticosteroids) and dietetic therapy. Further an appropriate investigations were therefore necessary; endoscopy and histological study of the specimen drawn by fibroscopy were decisive for the diagnosis. The accuracy of the endoscopy is of valuable help in uncertain situations as in those described.

A technique and apparatus are described for obtaining electrophoretic elution of virus from immunoadsorbent matrices. The method was applied to a well-known and easily purifiable virus, carnation mottle virus (CarMV). The technique was applicable even when the starting material was diluted and the virus recovered was indistinguishable from the original. Our aim was to avoid more drastic procedures that could damage virus structure (e.g. chaotropic chemicals, detergents, acidic pH's) and develop the mildest procedure possible. The apparatus is easy to work with and the procedure is simple and rapid.

Many authors have recently discussed the real value of the xylose test in the evaluation of intestinal malabsorption, especially its correlation with the morphological damage of the duodenal-jejunal mucosa. In our study we have performed in 48 pediatric patients one-hour blood xylose test and breath H2 test, method used for the diagnosis of sugar malabsorption, in order to value small bowel function and to know indirectly small intestinal mucosal structure. We have compared the values of the xylose test with those of the breath H2 test and both with hystological findings. The D-xylose absorption test was performed using the technique described by Roe and Rice. A serum concentration of more than 25 mg/dl was considered normal. For the breath H2 test the technique described by Douwes was used. A hydrogen concentration lower than 20 p.p.m. in expired air was considered normal. All of the patients in whose breath hydrogen was not detected after lactose oral load (2g/Kg body-weight, maximum 50 g), underwent a second test with lactulose (0,8 - 1 g/Kg body-weight), is not absorbed. We performed this test to rule out a false negative result (2% of the normal population). On histologic criteria, the patients were assigned to one of three study groups. Group I: normal duodenal-jejunal mucosal structure (11 patients). Group II: structural abnormalities not diagnostic of celiac diseases (7 patients). Group III: mucosal structural abnormalities typical of celiac disease (30 patients). In the first group the one-hour blood xylose test was normal in 9 patients (81.81%), whilst the breath H2 test was normal in 11 patients (100%).(ABSTRACT TRUNCATED AT 250 WORDS)

The amounts of tobacco necrosis virus antigen from necrotic lesions in tobacco leaves were estimated by ELISA. Less than 10% variation among different plates was obtained for the same sample placed in 3 wells randomly selected among the 60 internal wells of the plates, including in each test a dilution series of purified virus. Huxley's simple allometry equations y = bxm (y, absorbance; x, virus concentration) were calculated for each plate and gave reproducible results within a large range of virus concentration. The amount of viral antigen recovered from the necrotic centres of lesions was always significantly lower than those from the living tissues of the halo surrounding the centre. The serological activity recovered from both necrotic and halo tissues was not increased upon various treatments with disaggregating agents. During the continuous growth of the lesions the amounts of viral antigen extracted from the necrotic centre linearly decreased with time, suggesting virus degradation, whereas the amounts of antigen extracted from the living halo tissues increased with time, indicating that mechanisms of restricting viral spread and multiplication were not operating.

The diagnostic and therapeutic value of gastrointestinal endoscopy in adults is well established. Our study, performed in pediatric patients confirms its usefulness and emphasises its easy execution and little risk in children. The availability of small caliber fiberendoscopes and the simple medication permit its wide and sure use. The direct visualization of mucosal surface allows to see even small alteration always not found by roentgenological examination and to pick up aimed bioptic specimens. The therapeutic contribution revealed itself affective in our experience, permitting polipectomy and consequently rectal bleeding removal. It was possible at last by this technique to make the follow-up of patients with ulcerative colitis avoiding them repeated irradiations. We can thus conclude that gastrointestinal endoscopy has an important diagnostic and therapeutic value also in pediatric patients for the study of gastrointestinal diseases.