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The pierid butterfly Colias croceus (Geoffroy in Fourcroy, 1785), established in the Azores archipelago, is polymorphic with six forms, C. croceus f. croceus ♂ and ♀, C. c. f. cremonae ♂ and ♀, C. c. f. helice ♀, and C. c. f. cremonaehelice ♀. We investigated the optical mechanisms underlying the wing colouration of the butterflies by performing spectrophotometry and imaging scatterometry of the variously coloured wing areas and scales. The scale colouration is primarily due to wavelength-selective absorption of incident light by pterins expressed in granular beads in the wing scales, but thin film reflections of the scales’ lower lamina and scale stacking also contribute. Three forms (croceus ♂ and ♀ and helice ♀) are consistent with the patterns of the well-known ‘alba’ polymorphism. We postulate the coexistence of a second polymorphism, ‘cremonae’, to understand the three other forms (cremonae ♂ and ♀, and cremonaehelice ♀), which are characterized by the absence of red pigment, presumably due to the differential blocking of erythropterin expression.

Background/Purpose: Gastroesophageal reflux is a major cause of complications after esophageal atresia repair. The suitability of the Nissen fundoplication in these patients is still disputed. Therefore, the authors evaluated the results of their prospective treatment protocol in those patients who underwent a Nissen fundoplication. Methods: From 1984 to 1996, 125 patients underwent anastomosis for esophageal atresia. A Nissen fundoplication was later performed in 29 patients. The prospective protocol included x-ray after 10 days, 6 weeks, 12 weeks, 6 months, and 12 months. Forty-eightNhour pH measurements were performed between 6 and 12 weeks. Mean postfundoplication follow-up was at least 5 years (range, 2 to 13 years). Results: Two of the 29 patients died after the Nissen fundoplication from unrelated causes. A third patient was excluded from the study group. Nineteen of the remaining 26 patients showed severe stricture. pH-metry succeeded in 18 patients, showing pathological reflux in 17. In 24 patients the fundoplication was performed between 1 and 24 months (median, 4 months), in the other 2 patients much later. In 4 of the 26 patients (15%) the Nissen proved to be insufficient and had to be redone. The remaining 22 patients had no short-term or long-term complications. Conclusion: The authors' findings in this group of patients, comparing them with the results reported in the literature, indicate that there is no reason to change their prospective treatment protocol nor their policy to perform Nissen fundoplications at an early stage. J Pediatr Surg 35:573-576. Copyright © 2000 by W.B. Saunders Company.

In case of congenital diaphragmatic hernia (CDH), survival generally depends not on prenatal diagnosis, planned delivery, and immediate postnatal operation, but on the gravity of pulmonary hypoplasia and persistent hypertension (PPH). Many vasoactive drugs have become available for lowering PPH, but the mortality rate for CDH still amounts to 40% to 70%. Preoperative stabilization might prevent or at least reduce the risk of PPH. This method was evaluated in a pilot study lasting 15 months and involving 13 patients. All were admitted to the pediatric surgical intestive care unit within six hours of birth, all requiring mechanical ventilation. Continuous suction of the stomach and bowel proved successful in reducing the mediastinal shift. Study parameters were alveolar-arterial oxygenation differences ((A-a)DO 2 ), mean airway pressure (MAP), oxygenation index (OI), and ventilation index (VI), measured on admission and at set times before and after surgery. Eight patients did not survive, but in two cases death was not directly related to CDH. The following conclusions were reached: (1) satisfactory ventilation parameters on admission will remain good during the preoperative stabilization phase and will not be affected by its duration or by subsequent surgery, spelling survival; (2) unsatisfactory ventilation parameters on admission may improve with preoperative stabilization, giving these patients a better chance of survival; and (3) poor ventilation parameters on admission that fail to improve with preoperative stabilization will not improve with surgery or postoperatively, spelling death.

alpha-L-Iduronidase is a glycosyl hydrolase involved in the sequential degradation of the glycosaminoglycans heparan sulphate and dermatan sulphate. A deficiency in alpha-L-iduronidase results in the lysosomal accumulation and urinary secretion of partially degraded glycosaminoglycans and is the cause of the lysosomal storage disorder mucopolysaccharidosis type I (MPS I; Hurler and Scheie syndromes; McKusick 25280). The premature stop codons Q70X and W402X are two of the most common alpha-l-iduronidase gene (IDUA) mutations accounting for up to 70% of MPS I disease alleles in some populations. Here, we have reported a new mutation, making a total of 15 different mutations that can cause premature IDUA stop codons and have investigated the biochemistry of these mutations. Natural stop codon read-through was dependent on the fidelity of the codon when evaluated at Q70X and W402X in CHO-K1 cells, but the three possible stop codons TAA, TAG and TGA, had different effects on mRNA stability and this effect was context dependent. In CHO-K1 cells expressing the Q70X and W402X mutations, the level of gentamicin-enhanced stop codon read-through was slightly less than the increment in activity caused by a lower fidelity stop codon. In this system, gentamicin had more effect on read-through for the TAA and TGA stop codons when compared to the TAG stop codon. In an MPS I patient study, premature TGA stop codons were associated with a slightly attenuated clinical phenotype, when compared to classical Hurler syndrome (e.g. W402X/W402X and Q70X/Q70X genotypes with TAG stop codons). Natural read-through of premature stop codons is a potential explanation for variable clinical phenotype in MPS I patients. Enhanced stop codon read-through is a potential treatment strategy for a large sub-group of MPS I patients.