This case is of interest because of the rarity

of this presentation. The importance of thinking for inborn errors of metabolism in children with metabolic acidosis in late childhood is emphasized.”
“Klippel-Trenaunay syndrome (KTS) is an uncommon congenital angiodysplasia that manifests in infancy and is characterized by venous and lymphatic malformations of the skin, soft tissue, and bone causing limb hypertrophy. We report 2 patients with long-term Nepicastat KTS who developed lethal complications from uncommon and unusual manifestations. The 1st patient was a female with KTS who at 2 years of age underwent a below-the-knee amputation for a massively hypertrophied and malformed left

foot. Two years later she required additional surgical removal of vascular malformations involving her left calf with extension to the groin, pubis, and ipsilateral abdomen. Fifteen years later she underwent splenectomy (400 g) revealing multifocal, cystically dilated vascular channels distorting the splenic architecture and died click here suddenly of massive intra-abdominal hemorrhage on the 2nd postoperative day. The 2nd patient was a 72-year-old male with long-standing KTS who presented with debilitating chronic penile and scrotal edema. Surgical excision of his lymphedematous scrotal and penile skin revealed a low-grade angiosarcoma arising AG-014699 inhibitor in the setting of chronic lymphedema. The patient died shortly after surgery from massive hemorrhage due to traumatic rupture of malformed leg vessels. KTS may lead to significant morbidity and mortality, and pathologic consequences from long-term KTS

have been rarely reported. These cases illustrate the risk of lethal hemorrhage, organomegaly from protracted vascular malformation, and development of vascular neoplasia associated with chronic lymphedema in KTS.”
“The mechanical and optical properties of iPP nucleated with bicyclo[2.2.1]heptenedicarboxylate salts (BCHED) have been investigated. The results showed that aluminum bicyclo[2.2.1]heptenedicarboxylate (BCHE13) is the most effective nucleating agent to improve the mechanical and optical properties. Then the effects of the BCHE13 concentration on mechanical and optical properties and crystallization behaviors were studied. The results indicated that the saturated concentration of BCHE13 is about 0.2 wt %, at which nucleated iPP showed the better comprehensive mechanical and optical properties and high crystallization peak temperature. Nonisothermal crystallization kinetics of iPP nucleated with different BCHE13 contents have been investigated by Caze method. The results indicated Avrami exponents of nucleated iPP gradually increased with the increasing of BCHE13 concentration.