Effectiveness and safety of ciclosporin as therapy for autoimmune diseases of the liver in children and adolescents.

BACKGROUND: Conventional treatment for autoimmune hepatitis results in a significant percentage of failures and several, poorly tolerated, side-effects. Therapy for autoimmune cholangitis and giant cell hepatitis associated with autoimmune haemolysis is poorly documented. Ciclosporin is a promising treatment for all of these diseases. METHODS: We reviewed the records of 12 patients treated in our unit between 1987 and 2001. Eight had autoimmune hepatitis, two had autoimmune cholangitis and two had giant cell hepatitis. Indications for ciclosporin were treatment failure (four patients) and contraindications to/refusal of steroids (eight patients). Ciclosporin was administered in five untreated cases and in seven patients during relapse. The mean duration of ciclosporin administration was 35.6 months (8-89 months). The median follow-up was 6.5 years (1.5-15 years). RESULTS: All patients achieved complete remission in a median period of 4.5 weeks (2-12 weeks). No treatment withdrawal due to side-effects occurred. Three patients required a combination of ciclosporin with conventional treatment due to severe liver function impairment. Tolerance to ciclosporin was excellent. A 20% transient elevation of serum creatinine occurred in one case, gingival hypertrophy in two and moderate hypertrichosis in two. CONCLUSIONS: Ciclosporin may be considered as a safe treatment for all autoimmune liver diseases and as an effective alternative for front-line therapy.

Cholestasis as a presenting feature of acute Epstein-Barr virus infection.

Biochemical evidence of hepatic involvement in Epstein-Barr virus disease is common but clinical features of cholestasis are rare in children. We present three children with cholestasis as a presenting feature of Epstein-Barr virus disease.

Erosive immune complex-mediated arthritis associated with meningococcal meningitis.

A case of erosive sterile arthritis following meningococcal meningitis is described. High levels of immune complexes were detected in serum and synovial fluid. This is the first case in literature in which destruction of subchondral bone is documented. The erosion showed a progressive remineralization in the six months following clinical recovery.

Muscle thickness in infants hypertrophic pyloric stenosis.

Hypertrophic pyloric stenosis (IHPS) is the most common abdominal abnormality requiring surgery in infants. It occurs due to the hypertrophic and hyperplasia of the muscular layers of the pyloric. The usual age of clinical presentation is about three weeks of life. The most important symptom is non bilious emesis, intermittent or after each feeding. From march 1996 to June 2001, 21 infants, 20 males and 1 female, were subjected to ultrasonographic, radiographic exams and after diagnosis to the pyloromyotomy extramucosa. Ultrasonography was the study of choice used to identify hypertrophic pyloric stenosis; the markers to analyse were the length and the overall diameter of the pyloric canal and the muscle thickness of the wall. The results showed that a length of the pyloric canal 20 +/- 6 mm, a diameter 13.6 +/- 2.5 mm and a muscle thickness 4.1 +/- 1 mm are diagnostics for hypertrophic pyloric stenosis.

[Intolerance to cow's milk proteins: cases contribution]. / Intolleranza alle proteine del latte vaccino: contributo casistico.

The authors report their own case-report about cow's milk protein intolerance observed in the last seven years in the gastroenterology-department of Pediatric Clinic in Pisa. They underline the symptomatologic polymorphism of the illness and, analysing the laboratory data, point out the absence of absolute diagnostic assurance tests. For this reason they conclude that at the present state the diagnosis of certainty is overall committed to the resolution of the symptomatology after exclusion-diet and following clinic relapse after milk load.

[Fecal chymotrypsin in the evaluation of exocrine pancreatic function]. / La chimotripsina fecale nella valutazione della funzionalità pancreatica esocrina.

In this study the Authors examine the usefulness in determining Fecal Chymotrypsin (FC) as a screening test for pancreatic exocrine insufficiency. The FC was measured in 503 children (337 control subjects and 166 with various intestinal and hepatobiliary diseases). The results demonstrate that FC is usefull as a screening test for pancreatic insufficiency. However, in certain cases, this should be supplemented by the more discriminant analysis of function provided by duodenal studies.

[Initial experiences in the use of intravenous immunoglobulins in Guillain-Barré-Strohl syndrome]. / Prime esperienze sull'impiego delle immunoglobuline per via endovenosa nella sindrome di Guillain-Barré-Strohl.

The Authors demonstrate the successful therapeutic results obtained in two cases of Guillain-Barré-Strohl syndrome treated intravenously with high dosages of intact immunoglobulins. The possible immunological mechanisms behind this therapy are then discussed. The Authors believe that this treatment could very positively influence the prognosis of this disease.

[Anti-beta-lactoglobulin antibodies as an index of increased intestinal permeability in celiac disease]. / Gli anticorpi anti beta-lattoglobulina come indice di aumentata permeabilità intestinale in corso di malattia celiaca.

IgG antibodies to beta-lactoglobulin (ABLG) were assayed in children with coeliac disease (20 at gluten-containing diet and 35 at gluten-free diet) and in 28 "gastrointestinal" controls. Pathologic levels of ABLG were found in 16 patients with active coeliac disease (80%). In these patients the ABLG mean value was significantly higher than that found in the controls (p less than 0.001). In coeliac subjects at gluten-free diet, normal levels of ABLG were found after 6 months of diet. The presence of ABLG in the majority of untreated coeliac patients may reflect an increased permeability of the intestinal mucosa during the active stage of the disease.

[Peutz-Jeghers syndrome]. / La sindrome di Peutz-Jeghers.

An observation of Peutz-Jeghers syndrome, in a 13 year old girl, provides the Authors with the opportunity of reviewing the clinical features, the natural history and the complications of the disease, mainly the malignancies. In recent reviews the occurrence of cancer was further on investigated: gastrointestinal and non gastrointestinal tumors occurred in 22-48% of the patients examined. The surveillance protocols of the subjects at risk and genetic counseling are discussed.

[2 cases of cardial achalasia in childhood. Diagnostic aspects and therapeutic possibilities]. / Due casi di cardioacalasia in età pediatrica. Aspetti diagnostici e possibilità terapeutiche.

The authors present two case-studies of achalasia in infancy and the emphasize the rarity of the illness in this stage of life. They point out that a correct diagnosis requires a meticulous anamnesis which should be followed by a radiological, endoscopic and manometric study. The authors discuss their therapeutical experience with Nifedipine and they suggest that it should be used while waiting for surgery.

[Celiac disease associated with chronic autoimmune hepatitis. Description of a case]. / Associazione tra malattia celiaca ed epatite cronica autoimmune. Descrizione di un caso.

The authors describe a case of coeliac disease and autoimmune chronic hepatitis in the same patient. Case description allows a critical review of the literature and evaluation of pathogenetic hypotheses.

Recurrent meningitis: a case report.

A 25-month-old girl is described who experienced three successive attacks of purulent meningitis due to Proteus mirabilis. Third generation cephalosporins were employed as treatment. Cranial radiography and computed tomography revealed a malformation, in the form of an epidermoid cyst, in a central occipital position; small cerebellar abscesses were also present. The lesion was removed by surgery which led to a complete recovery.

[Selenium in celiac disease]. / Il selenio nella malattia celiaca.

Statistically significant lower levels of selenium (p less than 0.001) have been found both in 37 celiac subjects at free diet and in 36 at gluten-free diet with respect to controls. In patients at free diet the deficit of selenium can be attributed to malabsorption, while in patients at gluten-free diet it may be due to the diet itself. Recently low serum levels of selenium have been observed in several neoplasias; furthermore it's known that celiac patients show an increased incidence of gastrointestinal tumors related to known levels in standard population. Long term monitoring is therefore necessary to integrate diet with selenium in patients showing persistent deficit of this element.

[Clinical aspects of celiac disease. Comparison of 2 periods: before and after the introduction of antigliadin antibody determination in clinical practice]. / Gli aspetti clinici della malattia celiaca. Confronto tra due periodi: prima e dopo l'introduzione del dosaggio degli anticorpi antigliadina nella pratica clinica.

The clinical aspects of coeliac disease before and after anti-gliadin antibodies (AGA) assessment in clinical practice, referring to personal experience (107 cases in the period 1976-1988) are described. AGA determination has executed by two different ELISA methods. The diagnosis of coeliac disease in the period 1976-1986 has been made according to ESPGAN criteria, while in the last two years following the recent SIP advice. After 1987 with the introduction of AGA assay, the number of diagnosis/year of coeliac disease has increased three times in respect of the period 1976-1986. We have observed a more marked increase of the late beginning forms (from 2.8 to 10 diagnosis/year) in respect of the early beginning ones (from 3.7 to 7.5 diagnosis/year) and of the atypical forms (from 0.7 to 9 diagnosis/year) in respect of the typical ones (from 5.8 to 8.5 diagnosis/year). According to these data we think that prevalence of coeliac disease in our country is probably underestimated. AGA determination is at time most effective mean to make a screening of coeliac disease in the population. According to us the largest employment of this method in the next years could take a most exact estimate of the coeliac disease prevalence in our country.

[Plasma L-carnitine levels in children with celiac disease]. / Concentrazioni plasmatiche di L-carnitina in bambini celiaci.

Carnitine is a very important co-factor for the metabolism of fatty acids, because it is the necessary carrier for the passage of acyl groups inside the mitochondria, where beta-oxidation takes place. In the human body the total pool of carnitine is made by two fractions, one being endogenous and the second exogenous. The absorption of exogenous carnitine takes place mainly at duodenal-jejunal level by an active transport mechanism based on amino acid carrier. Plasma L-carnitine concentrations have been measured in 66 coeliac patients (mean age: 7 years and 4 months). Thirty three of them were on a free diet and the other 33 were on a gluten-free diet for at last six month. In 10 patients we studied plasma L-carnitine levels both on a free diet and on a gluten-free diet. As controls we examined 33 healthy children comparable for age. Plasma L-carnitine concentrations have been measured by a spectrophotometrical method according to Marquis and Fritz's technique and subsequently modified by Pearson and Seccombe. In the 66 coeliac patients the mean values of serum L-carnitine were significantly lower than those in the controls (p < 0.001). The levels were significantly lower in patients a free diet with respect to those on gluten-free diet (p < 0.01). The 10 subjects who were examined both on free diet and on gluten-free diet showed an increase of plasma concentrations in the latter condition.(ABSTRACT TRUNCATED AT 250 WORDS)

[24-hour esophageal pH-metry in the evaluation of gastroesophageal reflux pathology]. / La pH-metria esofagea delle 24 ore nella valutazione della patologia da reflusso gastroesofageo.

Gastro-esophageal reflux (GER) in infants was studied using 24-hour esophageal pH monitoring. Gastro-esophageal reflux was detected in 32/41 subjects. In our patients the main symptoms were vomiting, regurgitation, failure-to-thrive, chronic respiratory problems such as asthma, apnea, recurrent pneumonia. All patients with GER were treated appropriately with prone positioning and medical therapy (prokinetic agent and, eventually, ranitidine). Successful treatment of the reflux was obtained in all patients. In our opinion the 24-hour intraesophageal pH monitoring is a highly diagnostic test to identify the presence of GER and evaluate its gravity.

Gluten-dependent diabetes-related and thyroid-related autoantibodies in patients with celiac disease.

Patients with celiac disease are at high risk of having autoimmune disorders. Moreover, untreated patients with celiac disease have been found to have a higher than expected prevalence of organ-specific autoantibodies. In a prospective study of 90 patients with celiac disease, we found that the prevalence of diabetes and thyroid-related serum antibodies was 11.1% and 14.4%, respectively. Like antiendomysium autoantibodies, these organ-specific antibodies seem to be gluten-dependent and tend to disappear during a gluten-free diet.