Chronic poisoning by copper in tap water: I. Copper intoxications with predominantly gastointestinal symptoms.

Copper can induce acute and chronic intoxications in humans. Copper in tap water has caused a series of severe systemic diseases in Germany in recent years (copper induced liver cirrhosis). Besides cirrhosis, another type of disease with predominantly gastrointestinal symptoms has occurred which likewise appeared to be induced by copper in tap water. - In a retrospective investigation we looked for additional indications and proof that chronic copper poisoning has been the cause of the observed gastrointestinal diseases. All patients suffering from this type of disease had copper plumbing in their houses. - The patients (children and adults) suffered from nausea, vomiting, colic, and diarrhoea. In the group of infants, one refused formula milk (prepared with tap water) and the others suffered from persistent restlessness, unexplainable screaming (especially at night) and/or long lasting diaper rash. - We accept the diagnosis of chronic copper intoxication as the cause of the gastrointestinal symptoms when at least one of the following criteria were fulfilled: 1. first manifestation, remission and relapse of the disease depend on intake and a non-intake of water containing copper, respectively. 2. hypercupric state of the patients (i.e. pathological high concentrations of the non-ceruloplasmin-bound copper in serum and/or elevated copper levels in urine) 3. signs of systemic copper intoxication in the same patient 4. signs of systemic copper intoxication or hypercupric states in members of the patient s family or in his neighbourhood (non-relatives) - We found that the disease can even be caused by copper concentrations below the allowed concentration given by the German Guidelines for Drinking Water (Trinkwasserverordnung). - The data prove that copper in drinking water can cause gastrointestinal diseases and not only the better known systemic diseases (i.e. copper induced liver cirrhosis). Copper poisoning must be considered as a possible cause of chronic gastrointestinal diseases in those countries in which copper plumbing is common.

[Disseminated intravascular coagulation and acute renal failure in an infant. Treated with streptokinase and peritoneal dialysis (author's transl)]. / Disseminierte intravasale Gerinnung und akutes Nierenversagen bei einem Saugling

An infant, aged seven months, developed toxic shock with acute renal failure as a sequel to the development of hypertonic dehydration. The anuric phase persisted despite treatment of the dehydration and diuretic infusions. The coagulation tests showed signs of disseminated intravascular coagulation and so the child was given fibrinolytic therapy for 36 hours following initial heparinization. Excretion of urine recommenced 8 hours after the initiation of fibrinolytic therapy. Peritoneal dialysis was carried out in parallel with the fibrinolytic treatment without haemorrhagic complications. It was possible to terminate dialysis on the fourth day already and renal function subsequently recovered completely.

[Hereditary fructose intolerance]. / Hereditäre Fruktoseintoleranz.

The paper gives an overview of current diagnostic procedures in patients with clinical suspicion of hereditary fructose intolerance. On the basis of the literature and of a 9 years' experience at the Department of Paediatrics of the University of Graz a different approach according to the clinical condition of the patients is proposed (good clinical condition, severe liver disease, or bad clinical condition--i.e. liver biopsy is ethically not justified). The aim of this approach is to minimize invasive procedures for the children.

[Cholelithiasis as the first manifestation of cystic fibrosis of the pancreas (mucoviscidosis)]. / Cholelithiasis als Erstmanifestation der zystischen Pankreasfibrose (Mukoviszidose).

A hitherto healthy 6-year-old girl developed cholestatic jaundice, due to multiple extra- and intrahepatic cholesterol bile calculi. Cystic fibrosis of the pancreas was established as the cause of the disease and of the biliary cirrhosis. Operative treatment and the ensuing substitution with pancreatic extracts have improved the cholestasis. Pulmonary involvement became subsequently evident and is now under treatment. The pathophysiology of bile acids in cystic fibrosis as relevant to the occurrence of gall stones in these patients is discussed.

Autosomal dominant familial Mediterranean fever--like syndrome.

UNLABELLED: We report a syndrome characterized by recurrent episodes of fever and serositis in an Austrian family. Three family members over two successive generations were affected. The febrile episodes had their onset at the age of 11-12 years, lasted 1-5 weeks, and occurred in intervals of 6-24 months. While the disorder resembles familial Mediterranean fever (FMF) clinically, ethnic distribution and other features suggest a distinct entity. Clinically, the attacks last longer than the usual FMF attacks, and in the male patients are associated with scrotal inflammation. Genetically, the disorder appears to be inherited as an autosomal dominant syndrome, whereas FMF is autosomal recessive. Molecular analysis made the involvement of a gene in the FMF region of chromosome 16p13.3 highly unlikely. CONCLUSION: An Austrian family with recurrent fever syndrome is reported. Ethnicity, clinical features, and molecular studies point to a distinct clinical entity.

Demonstration of Epstein-Barr virus DNA in a previously healthy boy with fulminant hepatic failure.

A previously healthy 9-year-old boy died from acute liver failure during an acute Epstein-Barr virus infection. Epstein-Barr virus DNA could be demonstrated in the liver by Southern blot--and by in situ hybridization techniques. The identification of the virus in the liver suggests a causal relation between the Epstein-Barr virus and the acute massive liver cell necrosis.

Gender, age and seasonal effects on IgA deficiency: a study of 7293 Caucasians.

BACKGROUND: The frequency of serum IgA deficiency (SIgAD) differs between populations. We examined the prevalence of SIgAD in healthy Caucasians. MATERIALS AND METHODS: Serum immunoglobulin A (SIgA) was measured in 7293 volunteers (2264 women, 5029 men) aged 30 +/- 14.2 years (mean +/- SD; range: 12-66). Serum immunoglobulin A and subnormal SIgA levels were defined by a SIgA level < 0.07 g L(-1), and between 0.07 and 0.7 g L(-1), respectively. Means were compared by analysis of variance (anova) and analysis of covariance (ancova); frequencies by the chi(2) test. RESULTS: Fifteen subjects (0.21%; one woman, 14 men) had SIgAD. Subnormal SIgA levels were found in 155 persons (2.13%): 21 females (0.93% of the females) and 134 males (2.66% of the males; difference: 1.74%; 95% CI: 1.12-2.33%; P < 0.001). Males were more likely to have subnormal SIgA levels or SIgAD (odds ratio 3.09, 95% CI: 1.97-4.85). The prevalence of SIgAD and subnormal SIgA was lowest in winter (chi(2) = 14.8; P = 0.002; 3 d.f.; and chi(2) = 43.2; P < 0.001; 3 d.f., respectively). Serum immunoglobulin A concentrations were significantly higher during winter. Serum immunoglobulin A levels increased with age on average by 0.2 +/- 0.06 g L(-1) per decade of life (P < 0.001). Taking into account the influence of age, SIgA concentration was lower in females as compared with males. CONCLUSION: The prevalence of SIgAD and subnormal SIgA levels is increased in males. There exists a significant influence of gender, age and seasons on SIgA levels.