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Background: An increasing number of children with hereditary tubular disorders (HTD) reach adult life due to diagnostic and therapeutic advances which results in growing need to manage these patients by adult centres. Data on the prevalence and the late clinical problems of these patients are limited. Methods: We observed 177 paediatric patients with isolated or complex HTD between 1969 and 1994. The median age at the time of diagnosis was 3 (range 0–18) years and the median observation period 10 (range 1–43) years. The long-term outcomes with respect to renal function, bone disease, and body growth were analyzed. Results: The prevalence of HTD was 3.2% of all patients observed in our renal unit and 14% of those patients with chronic renal failure and/ or end-stage renal disease. The three most frequent disorders observed were nephropathic cystinosis (n = 34), X-linked hypophosphataemic rickets (n = 26), and idiopathic hypercalciuria (n = 17). At the last observation, 12% of the patients with isolated HTD and 30% of those with complex HTD had developed preterminal chronic renal failure; end-stage renal disease was observed in 5 and 25%, respectively (p < 0.001). Progressive disease occurred mainly in patients having cystinosis, primary hyperoxaluria, the syndrome of hypomagnesaemia/hypercalciuria, primary Fanconi syndrome, Fanconi-Bickel syndrome, and methylmalonic aciduria. Nephrocalcinosis was found in 42%, urolithiasis in 14%, bone deformities and/or fractures in 28%, and other extrarenal alterations in 29% of all patients. The median body height at last observation was 2.0 SD below the normal mean (range from –10.4 to +2.6), and the adult height was subnormal in 48% of 67 grown-up patients. Growth retardation was more severe in complex than in isolated HTD. The mortality decreased from 17% in 1969–1981 to 12% in 1982–1994. Conclusion: Although HTD are rare nephropathies, their frequently progressive course associated with extrarenal complications requires the attention of nephrologists beyond the paediatric age.

Using strict criteria for diagnosis, 23 patients having benign Barrett's esophagus, and 20 patients with adenocarcinoma arising in this epithelium have been analyzed. Evidence supports severe gastroesophageal reflux as a cause of Barrett's esophagus. Successful antireflux surgery leads to stabilization and possibly regression of the dysplasia in Barrett's epithelium, and can be followed by squamous epithelial regeneration in some. Antireflux surgery is advocated in all patients with Barrett's esophagus demonstrated to have abnormal reflux regardless of symptoms. The malignant potential of the columnar epithelium is higher in men who smoke, in patients with intestinal-type metaplasia who continue to have severe reflux, and in patients who develop dysplasia. In those with high grade dysplasia, the probability of carcinoma is high and esophagectomy should be seriously considered in the hopes that the pathological stage of the neoplasm is still favorable.

Reports from various countries raise the question of changes in the incidence and etiology of acute pancreatitis. This study covers patients admitted with acute pancreatitis to a single university department of medicine between 1965 and 1970 and between 1980 and 1985. The absolute number of patients admitted with acute pancreatitis doubled, whereas the percentage of pancreatitic patients in the total population of admitted patients decreased by 38%. No firm conclusion can be made about an increasing incidence of acute pancreatitis from 1970 to 1985. However, the dominating etiological factor was no longer biliary tract disease but significantly shifted to alcoholism, whereas changes in the severity of the disease were not significant. There was a downward trend in mortality with mortality rates of 13 and 8.6% for the first and second period, respectively; however, because of the small number of patients this change did not reach the level of significance.

Studies were undertaken in 40 patients with chronic pancreatitis six months to seven and a half years (mean 25 months) after operation, results being compared with pre-operative findings. Measurements included: exercise capacity, absence of pain, body weight, endocrine (36) and exocrine (25) pancreatic function. Almost all patients returned to full or only slightly impaired activity, were free of pain or had less pain and weight increase. Exocrine pancreatic function (secretin-pancreozymin test and faecal fat) was noted in 11 of 25 patients. In another 11 pre-operative progression was arrested. But endocrine function improved in only three of 36 and worsened in 13 (manifestation of subclinical diabetes in eight, worse glucose tolerance in five). The results justify a more active surgical approach in the treatment of chronic pancreatitis in order to save the patients from an often long and painful "burning out" of the disease on purely conservative treatment. Furthermore, exocrine pancreatic function, at least, is maintained or improved.