Alkylphenols in sediments of the Atlantic Rainforest south-west of São Paulo, Brazil.

Concentrations of technical 4-nonylphenol, 4-n-octylphenol, and 4-tert-octylphenol were investigated in surface water and sediment samples of four reservoirs southwest of São Paulo. Three of them (Takimoto 1, Takimoto 2, Pedro) were established in intensively cropped landscapes, one (Morro Grande) was surrounded by dense forests. Total alkylphenol concentrations in sediments generally ranged between 1 and 10 microgkg-1dw with 4-tert-octylphenol being the dominant alkylphenol. Because 4-tert-octylphenol concentration patterns at Takimoto 2, Pedro, and in the Morro Grande forest reservoir were found to be quite similar (upto 5 microgkg-1dw), this value is considered as the ubiquitous background level. In contrast, the Takimoto 1 sediments showed significantly higher 4-tert-octylphenol concentrations, possibly due to accelerated inputs from adjacent intensively cropped fields. Analysed alkylphenols were not detectable in water samples.

Calcium-stimulated insulin secretion in diffuse and focal forms of congenital hyperinsulinism.

OBJECTIVES: To identify infants with hyperinsulinism caused by defects of the beta-cell adenosine triphosphate-dependent potassium channel complex and to distinguish focal and diffuse forms of hyperinsulinism caused by these mutations. STUDY DESIGN: The acute insulin response to intravenous calcium stimulation (CaAIR) was determined in 9 patients <20 years with diffuse hyperinsulinism caused by defective beta-cell sulfonylurea receptor (SUR1(-/-)), 3 patients with focal congenital hyperinsulinism (6 weeks to 18 months), a 10-year-old with insulinoma, 5 with hyperinsulinism/hyperammonemia syndrome caused by defective glutamate dehydrogenase (6 months to 28 years), 4 SUR1(+/-) heterozygotes with no symptoms, and 9 normal adults. Three infants with congenital focal disease, 1 with diffuse hyperinsulinism, and the child with insulinoma underwent selective pancreatic intra-arterial calcium stimulation with hepatic venous sampling. RESULTS: Children with diffuse SUR1(-/-) disease and infants with congenital focal hyperinsulinism responded to CaAIR, whereas the normal control group, patients with hyperinsulinism/hyperammonemia syndrome, and SUR1(+/-) carriers did not. Selective arterial calcium stimulation of the pancreas with hepatic venous sampling revealed selective, significant step-ups in insulin secretion that correlated anatomically with the location of solitary lesions confirmed surgically in 2 of 3 infants with congenital focal disease and in the child with insulinoma. Selective arterial calcium stimulation of the pancreas with hepatic venous sampling demonstrated markedly elevated baseline insulin levels throughout the pancreas of the infant with diffuse hyperinsulinism. CONCLUSIONS: The intravenous CaAIR is a safe and simple test for identifying infants with diffuse SUR1(-/-) hyperinsulinism or with focal congenital hyperinsulinism. Preoperative selective arterial calcium stimulation of the pancreas with hepatic venous sampling can localize focal lesions causing hyperinsulinism in children. The combination of these calcium stimulation tests may help distinguish focal lesions suitable for cure by local surgical resection.

[Dog tourism and import: an inquiry in Germany on the extent as well as on the spectrum and preference of countries of residence and origin respectively]. / Hundetourismus und -import: eine Umfrage in Deutschland zu Ausmass sowie Spektrum und Präferenz der Aufenthalts- bzw. Herkunftsländer.

A survey was conducted among practicing veterinarians and at pet clinics in Germany in order to estimate the extent of tourism with dogs and import of dogs and to determine the range and preference of the foreign countries involved. The survey covered the years from 1985 to 1995 and included 5240 dogs, of which 4567 (87.2%) were born in Germany and 673 (12.8%) were born abroad. Among the latter, 263 (39.1%) originated from Mediterranean countries and Portugal, 344 (51.1%) were born in other European countries and 66 (9.8%) were from non-European countries. Of all 5240 dogs surveyed, 2894 (55.2%) had been taken abroad at least once between 1985 and 1995. Of these, 2424 (53.1%) were born in Germany, 470 (69.8%) were born abroad. Of the 2894 dogs taken abroad, 1929 (66.7%) travelled to Mediterranean countries or Portugal and 1152 of these had additional travels to other countries as well. The spectrum of all countries travelled to was very broad, but numerous dogs were taken regularly, repeatedly and exclusively to Austria, Switzerland, Italy, Spain or France. Other countries were visited only once for the majority of dogs. The analysis of the annual survey data revealed a steady increase of dogs along on trips from Germany to other countries, rising from 31.1% in 1990 to 40.8% in 1994. In any of these years, always more than 56% of these dogs were taken to Mediterranean countries.

Familial hyperinsulinism with apparent autosomal dominant inheritance: clinical and genetic differences from the autosomal recessive variant.

We describe three families with hypoglycemia caused by familial hyperinsulinism (HI) in whom vertical transmission of the disorder occurred, suggesting autosomal dominant (AD) inheritance. We therefore examined the relationship between the apparent AD disorder and the more common autosomal recessive (AR) form of HI, which has recently been linked to the sulfonylurea receptor on chromosome 11p15.1. The clinical features of the 11 patients with AD HI were milder than those seen in 14 patients with AR HI. Hypoglycemia was readily controlled with either diet alone or with diazoxide in 10 of 11 patients with AD HI but in none of those with the AR form. In one large pedigree, analysis of genomic DNA with polymorphic simple sequence repeat markers excluded linkage of AD HI to the SUR locus in a dominant manner. The possibility of linkage to the SUR locus could not be absolutely excluded in the two smaller pedigrees. None of the published mutations of the SUR gene identified in patients with AR HI were detected in the patients with the AD form. We conclude that the AD form of hyperinsulinism is phenotypically different from the AR variant. The identification of more families with this form of HI may make it possible to locate the responsible gene by the use of linkage analysis.

Persistent hyperinsulinemic hypoglycemia of infancy: long-term octreotide treatment without pancreatectomy.

Eight patients with persistent hyperinsulinemic hypoglycemia of infancy who were treated with octreotide without pancreatectomy are described. All had severe, early-onset disease that would have required partial pancreatectomy had octreotide not been available. Along with octreotide, frequent feedings and raw cornstarch at night were required by all. Octreotide was given in three or four daily subcutaneous injections in four patients and in a continuous subcutaneous infusion with an insulin infusion pump in four. All had mild, transient gastrointestinal symptoms (vomiting, abdominal distention, steatorrhea) after the start of therapy. Asymptomatic gallstones were found in 1 patient after 1 year of treatment. No other long-term untoward effects were noted, including no detrimental effect on psychomotor development. Growth was not affected in five of six patients treated for more than 6 months. In five patients, octreotide was discontinued after 9 months to 5 1/2 years; patients were given diazoxide instead, two required percutaneous gastrostomy, and one 5 1/2-year-old child required no further treatment. The remaining three patients (aged 5 to 9 months) are still being treated with octreotide. We conclude that, with the use of octreotide, pancreatectomy can be avoided in some patients. Particularly in light of our findings of a high incidence of diabetes years after partial pancreatectomy, and clinical improvement after months to years of octreotide treatment, we believe that aggressive medical therapy, when effective, is preferable to partial pancreatectomy.