SUMMARY Primary pigmented nodular adrenocortical disease (PPNAD) is a rare adrenocorticotropin hormone (ACTH)-independent Cushing's syndrome (CS). Pediatric patients with PPNAD typically have unusual skin lesions and slow growth with unknown causes. We present a case of a female Chinese patient with PPNAD caused by the germline PRKACA gene copy number gain of chromosome 19. The patient initially presented with kidney stones, short stature, and obesity. After further testing, it was discovered that the patient had diabetes, mild hypertension, low bone mass, a low ACTH level, and hypercortisolemia, and neither the low-dose or high-dose dexamethasone suppression test was able to inhibit hematuric cortisol, which paradoxically increased. PPNAD was pathologically diagnosed after unilateral adrenalectomy. Chromosome microarrays and whole exon sequencing analyses of the peripheral blood, as well as testing of sectioned adrenal tissue, showed a rise in the copy number of the duplication-containing PRKACA gene on chromosome 19p13.13p13.12, a de novo but not heritable gene defect that causes disease. The clinical signs and symptoms supported the diagnosis of Carney complex (CNC). One significant mechanism of CNC pathogenesis may be the rise in germline PRKACA copy number of chromosome 19. When assessing PPNAD patients for CNC, the possibility of PRKACA gene amplification should be considered. The effect of PRKACA gene amplification on the clinical manifestations of CNC needs to be confirmed by more cases. (PPNAD ACTHindependent independent Cushings Cushing s CS. CS . (CS) 19 stones stature obesity diabetes hypertension mass level hypercortisolemia lowdose dose highdose high cortisol increased adrenalectomy blood tissue duplicationcontaining duplication containing 19p1313p1312 pp 19p13 13p13 12 p 19p13.13p13.12 CNC. (CNC) considered cases (CS 1 19p1313p131 19p1 13p1 19p13.13p13.1 (CNC 19p1313p13 19p 13p 19p13.13p13. 19p1313p1 19p13.13p13 19p1313p 19p13.13p1 19p13.13p

Objectives: To explore the effects of computer radiation on weight and oxidant-antioxidant status of rats, and further to confirm that whether vitamin C has protective effects on computer radiation. Methods: Sixty Male adult ICR mice were randomly divided into six groups. each group give different treatment as follows: group A was control, group B given vitamin C intake, group C given 8 h/day computer radiation exposure, group D given vitamin C intake and 8 h/day computer radiation group E given 16 h/day computer radiation exposure, group F given vitamin C intake plus exposure to 16 h/day computer radiation. After seven weeks, mice was executed to collect the blood samples, for detecting total antioxidant capacity (T-AOC) and alkaline phosphates (ALP)content in serum or liver tissue were determined by ELIASA. Results: No difference was found for the change of weight among six groups at different week. In the group C, D and F, the liver tissue T-AOC level were higher than the group A. In the group B, C and E, the serum ALP level were lower than the group A (P<0.05). Conclusions: The study indicate that computer radiation may have an adverse effect on T-AOC and ALP level of mice, and vitamin C have protective effect against computer radiation.

Objetivos: Explorar los efectos de la radiación de ordenador sobre el peso y el estado oxidativo-antioxidativo de los ratones, y además para confirmar si la vitamina C tiene efectos protectores contra la radiación de ordenador. Métodos: Sesenta ratones machos adultos ICR fueron aleatoriamente divididos en seis grupos. Cada grupo recibió un tratamiento diferente del modo siguiente: el grupo A fue el grupo de control, el grupo B recibió vitamina C, el grupo C fue sometido a una exposicion a la radiacion de ordenador de 8 h/día, el Grupo D recibió vitamina C y fue sometido a una exposicion a la radiacion de ordenador de 8 h/día, el Grupo E fue sometido a una radiación de ordenador de 16 h/día, el grupo F recibió vitamina C y fue sometido a una exposicion a la radiacion de ordenador de 16 h/día. Al cabo de siete semanas, los ratones fueron ejecutados para extraer las muestras de sangre, para detectar la capacidad antioxidante total (T-AOC) y el contenido de fosfatasa alcalina (ALP) en suero o en tejido hepático fue determinado mediante ELISA. Resultados: No se encontraron diferencias en cuanto a cambio de peso entre los seis grupos diferentes. En los grupos C, D y F, el nivel en de T-AOC en tejido hepático fue más alto que en el grupo A. En los grupos B, C y E, el nivel de ALP en suero fue más bajo que en el Grupo A (P < 0,05). Conclusiones: El estudio indican que la radiación de ordenador puede tener un efecto adverso en los niveles de T-AOC y ALP de ratones, y que la vitamina C tendría un efecto protector contra la radiación del ordenador.

In order to study the dynamic recrystallization (DRX) behavior of as-extruded 42CrMo high-strength steel, a series of isothermal upsetting experiments were carried out in a temperature range of 1123~1348 K and a strain rate range of 0.01~10 s-1 on Gleeble 1500. It was found that DRX softening is more recognizable at higher temperatures and lower strain rates, and stress level increases with increasing strain rate and decreasing deformation temperature. The types of flow stress evolution were distinguished by the following three characteristics: WH followed by DRX, WH followed by DRV, and WH followed by no dynamic softening. At a fixed temperature, the average grain size refined by DRX linearly decreases with increasing strain rate in log scale. At a fixed strain rate, the average grain size remains almost constant below 1273 K, while it rapidly increases above 1273 K. At a larger strain rate, data set of grain sizes has a smaller standard deviation to the average size value, and data have tightly grouped. This indicates that as strain rate increases, the microstructure becomes more and more uniform. The relationships between the average grain size and Zener-Hollomon parameter were nonlinearly fitted by the equation D A = 319.81202 - 13.6114 lnZ + 0.15322 (lnZ)². The results from this equation show that the average grain size decreases with increasing Zener-Hollomon parameter. On the plot of D A versus lnZ, the regions corresponding to DRV (lnZ 37.8) and DRX (lnZ 37.8) were clarified clearly.