Lung transplantation began to expand in 1983, after the advent of cyclosporin and the publication of the Toronto lung transplant group study. Single lung transplantation was first performed in patients with interstitial pneumopathy to be extended later to pulmonary emphysema, then to primary or secondary pulmonary arterial hypertension. Double lung transplantation provides patients suffering from chronic lung infection (e.g. cystic fibrosis) with a useful alternative to their ordinary treatment. The experience acquired throughout these years has resulted in wider criteria for patients' inclusion. More than acute rejection, bacterial infections directly condition the immediate prognosis. The frequency and severity of cytomegalovirus lung diseases lead to a discussion on the possibility of prophylactic and curative antiviral therapy. The occurrence of obliterative broncholitis, which reflects chronic lung rejection, jeopardizes the long-term results of transplantation. The functional results of the various types of lung grafting are analysed, and the position of lung transplantation in thoracic surgery is reassessed.

Graft Rejection↗

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Sjögren's syndrome.

Explore the source record for details and available documents.

Humans↗

PubMedDate not supplied

[Early detection of hepatobiliary involvement in children with mucoviscidosis using duplex sonography of the portal system].

To investigate early signs of hepatobiliary disease in CF, we measured portal, splenic, superior mesenteric vein and hepatic artery diameters, maximal flow velocity (Vmax) and time average velocity (TAV) in 25 males and 17 females with CF. Hepatic artery resistance, regional blood flow and liver perfusion were calculated. According to liver enzyme data (aminotransferases raised greater than 30 U/l) and sonographic findings (nodular changes), there were 17 CF-patients (mean age 11.4 yrs; range 0.75-31) with and 25 CF-patients (mean age 7.8 yrs; range 0.25-32) without liver involvement (L+/L-). No patient had clinical signs of portal hypertension. 61 healthy children were studied for control. Diameter of portal vein (PVD) and flow data for portal vein showed consistent abnormalities (mean +/- SD): CF-L+ CF-L- Control PVC (mm/m2) 10.7 +/- 3.9*** 10.5 +/- 3.4*** 7.2 +/- 1.3 Vmax (m/sec) 0.23 +/- 0.06*** 0.30 +/- 0.06*** 0.40 +/- 0.14 TAV (m/sec) 0.12 +/- 0.04*** 0.16 +/- 0.04* 0.18 +/- 0.05 Differences were statistically significant (* p less than 0.05, *** p less than 0.001) for CF-patients versus controls. Data for splenic and mesenteric veins and for hepatic artery were moderately alterated, with a significant reduction in TAV and Vmax of splenic vein for CF-L+ versus controls. Liver perfusion and portal vein flow showed no relevant differences in CF-patients versus controls. It is concluded that portal system abnormalities, especially a decrease in Vmax and TAV of portal vein shown by duplex sonography, may be earlier indicators of CF liver disease than biochemical and clinical signs.

Adolescent↗

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[Hydrogen (H2) breath test following lactose loading in children with recurrent abdominal pain].

To define the practical diagnostic value of H2 breath testing after lactose load in children with chronic abdominal pain, we measured end-expiratory H2 every 30 minutes for 2 hours after peroral lactose (2 g/kg body weight, Lactoscreen). H2 testing was done in 62 children (28 female, 32 male), aged between 2 and 16 years. Abdominal pain had been observed in these children for more than 2 months. Causes of abdominal pain other than lactose intolerance had been excluded. In 2 out of 62 children, H2 testing was impossible for lack of cooperation. 17 out of the remaining 60 children showed a positive test result (28.3%). In 15 patients, the rise in H2 exceeded 40 ppm, and was thus easily distinguished from a nonspecific rise (less than 25 ppm). Stool pH and Clini-test results were not correlating well with H2 findings, In 16 out of 17 children with positive H2 tests, abdominal pain had ceased on a diet free of lactose after a month. After 6 months 15 out of 17 children were symptom free. These data confirm early indication of non-invasive H2 testing in children with chronic abdominal pain.

Abdominal Pain↗

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[Pancreatic enzyme replacement in mucoviscidosis (CF): clinical evaluation of a gastric acid-resistant pancreatin preparation in encapsulated microtablet form].

Pancreatic enzyme replacement therapy was tested in 17 patients with CF. Severe pancreatic insufficiency had been established by stool fat determination prior to therapy. Two enzyme preparations were tested that were equal in acid protection, but were different in the release of enzyme activities. Complaints, body weight, and stool frequency were not influenced by changing preparations. Stool weight and stool fat excretion did not show statistically significant differences, when the preparations were used in lipase equivalent doses. By using one of the preparations tested (Panzytrat 20,000), it was possible to reduce the number of capsules that had to be taken daily. Mild to moderate steatorrhoea was noted in spite of pancreatic enzyme replacement in all the CF patients. Massive steatorrhoea was seen in two patients with good nutritional status, due to high fat intake. It was proposed that moderate steatorrhoea may be tolerated in CF, as long as the nutritional status is adequate. If this is not the case, energy intake and, as a consequence, the dosage of pancreatic enzyme replacement therapy have to be raised.

Adolescent↗

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Concepts in coeliac disease: is there a receptor for gliadin?

Genetic, biochemical and immunological theories have added to our knowledge of coeliac pathogenesis. There are new data, however sparse, to support a unifying pathogenetic concept, namely the hypothesis of a glycoprotein specific cellular receptor for gliadin on lymphocytes and enterocytes.

B-Lymphocytes↗

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MRI of a hibernoma.

Hibernomas are benign tumors derived from brown fat that most often present as painless, slowly enlarging masses in the interscapular region. Because these lesions have a heterogeneous appearance and contain fat, they mimic liposarcomas on CT and MR, requiring biopsy for definitive diagnosis.

Female↗

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Effects of gliadin peptides B1-B4 in celiac disease. I. Organ culture studies.

Small intestinal organ culture was used as an in vitro system to study the enterotoxic effects of gliadin peptides. Measurement of enterocyte height proved to be a reliable and reproducible way of assessing mucosal change during organ culture. Enterocyte height decreases nonspecifically in normal cultured mucosa, whereas the height of enterocytes of celiac mucosa increases in vitro in controls. All the gliadin peptide fractions (B1, B2, B3, B4) that had been prepared by peptic-tryptic hydrolysis, ultrafiltration, and gel chromatography, equally inhibited the morphological increase of enterocyte height normally observed without gliadin in untreated celiac mucosa. Electrophoretic studies and amino acid analysis of B1-B4 revealed similarity between gliadin fractions with quantitative differences in molecular weight distribution of the peptide components. Our studies suggest that organ culture assessed by morphometry is a suitable model for the investigation of toxic peptides of gliadin in celiac disease. In the future, pure gliadin peptides will have to be examined.

Atrophy↗