Selective Formation of 4,4'-Biphenols by Anodic Dehydrogenative Cross- and Homo-Coupling Reaction.

A simple and selective electrochemical synthesis by dehydrogenative coupling of unprotected 2,6- or 2,5-substituted phenols to the desired 4,4'-biphenols is reported. Using electricity as the oxidizing reagent avoids pre-functionalization of the starting materials, since a selective activation of the substrates takes place. Without the necessity for metal-catalysts or the use of stoichiometric reagents it is an economic and environmentally friendly transformation.

Mutations in the MPV17 gene are responsible for rapidly progressive liver failure in infancy.

Unlabelled: MPV17 is a mitochondrial inner membrane protein of unknown function recently recognized as responsible for a mitochondrial DNA depletion syndrome. The aim of this study is to delineate the specific clinical, pathological, biochemical, and molecular features associated with mitochondrial DNA depletion due to MPV17 gene mutations. We report 4 cases from 3 ethnically diverse families with MPV17 mutations.

Delayed contrast enhancement magnetic resonance imaging in congenital aortic stenosis.

We report a case of a 12-year-old boy with severe congenital aortic stenosis in whom magnetic resonance imaging (MRI) with delayed contrast enhancement demonstrated extensive subendocardial hyperenhancement within the left ventricle. The hyperenhancement was confirmed to be subendocardial infarct and fibrosis by histopathology. This case supports the utility of MRI with delayed contrast enhancement in evaluating myocardial viability in patients with congenital heart disease.

Components in seminal plasma regulating sperm transport and elimination.

Seminal plasma has been suggested to be involved in sperm transport, and as a modulator of sperm-induced inflammation, which is thought to be an important part of sperm elimination from the female reproductive tract. This article reports on recent experiments on the importance of seminal plasma components in sperm transport and elimination. In Experiment 1, hysteroscopic insemination in the presence (n = 3) or absence (n = 3) of 2 ng/mL PGE showed an increased portion of spermatozoa crossing the utero-tubal junction in the presence of PGE in two mares, while no difference was observed between treatments in a third mare.

Ischemia/reperfusion: a clinically relevant model of intestinal injury yielding systemic inflammation.

Background/purpose: Multisystem organ failure (MSOF) is a major cause of morbidity and mortality in the critically ill patient. Animal models of endotoxin-induced sepsis were used to develop therapeutic regimens, which thus far have failed in clinical trials. Because multiple etiologies of MSOF affect the intestine, the authors hypothesized that during sepsis the gut may act as a possible trigger of the inflammatory cascade.

Fatal congenital systemic juvenile xanthogranuloma with liver failure.

This is the second reported patient with systemic juvenile xanthogranuloma (JXG) to die with liver failure. The infant was born with multiple skin lesions and mild hepatomegaly. Direct hyperbilirubinemia was noted on the 2nd day of life, followed by progressive hepatomegaly, cholestasis, and death at 29 days of age.

Reflux esophagitis: sequelae and differential diagnosis in infants and children including eosinophilic esophagitis.

Gastroesophageal reflux disease (GERD) is a common condition in infants and children and has many clinical mimics. Most pediatric pathology departments process many mucosal biopsies from the proximal gastrointestinal tract to evaluate the presence or absence of reflux esophagitis. Since this subject was last reviewed in the 1997 edition of Perspectives in Pediatric Pathology devoted to gastrointestinal diseases in children (Dahms BB.

Clinical profile of 30 infants with acute pulmonary hemorrhage in Cleveland.

Between 1993 and 2000, 30 infants were hospitalized with acute pulmonary hemorrhage at Rainbow Babies and Children's Hospital in Cleveland. Most infants presented with severe pulmonary symptoms requiring intensive support, but a few infants had less severe hemorrhage. Three quarters of the patients required ventilator support and blood transfusions.

IL-10 is not protective in intestinal ischemia reperfusion injury.

Background: Ischemia/reperfusion of the small intestine disrupts gut barrier function, increases bacterial translocation, and activates systemic pro-inflammatory responses. Pharmacological treatment with the anti-inflammatory cytokine interleukin-10 (IL-10) following ischemia to muscle reduces the severity of local and systemic inflammation. While endogenous IL-10 is protective in murine models of acute endotoxemia, its physiological role during direct gut injury is unknown.

Severe pulmonary hypertensive vascular disease in two newborns with aneurysmal vein of galen.

Arteriovenous malformation of the vein of Galen (AVG) is a rare entity in the newborn with a high morbidity and mortality. We present two cases of fatal AVG with persistent pulmonary hypertension of the newborn and significant pulmonary hypertension documented by autopsy histopathology. The pathophysiology is reviewed and a proposed mechanism of the association between AVG and pulmonary hypertension is discussed.

Severe perinatal liver disease associated with fetal thrombotic vasculopathy.

Three neonates with fetal thrombotic vasculopathy in the placenta and severe neonatal liver disease are described. Symptoms included a bleeding disorder on the first day of life, followed by direct hyperbilirubinemia and elevated liver transaminases. All patients also had evidence of thrombosis outside the placenta, including cerebral infarct in two infants and thrombosis of the inferior vena cava in one infant.

Interaction between equine semen and the endometrium: the inflammatory response to semen.

Insemination of mares with bacteria-free equine spermatozoa results in an influx of polymorphonuclear neutrophils (PMNs) into the uterine lumen. In vitro studies have demonstrated that equine spermatozoa activate complement, resulting in cleavage of factors C5a and C3b. Since uterine secretion is rich in complement, it is likely that an interaction between spermatozoa and uterine secretion results in C5a-mediated chemotaxis and migration of PMNs into the uterine lumen.

Pulmonary atresia with intact ventricular septum, right-sided aortic arch and ventriculocoronary connection--prenatal echocardiographic diagnosis.

The authors describe the prenatal echocardiographic diagnosis of a rare case of pulmonary atresia with intact ventricular septum, right-sided aortic arch, and ventriculocoronary connection in a fetus at 21 weeks gestation. The diagnosis was confirmed at autopsy.

Intrinsic hyperreactivity of mucosal T cells to interleukin-2 in pediatric Crohn's disease.

Objectives: To cells play a crucial role in many chronic inflammatory diseases. Mucosal T cells are particularly important in the pathogenesis of Crohn's disease (CD). We investigated the response of T cells in CD and other intestinal inflammatory conditions to interleukin-2 (IL-2), a cytokine essential for T-cell activation, growth, and function.

Pulmonary intralobar sequestration in a patient with cystic fibrosis.

We report a case in which pulmonary Intralobar Sequestration (ILS) was an incidental finding at autopsy in an adult with Cystic Fibrosis. Two aberrant arteries from the descending thoracic aorta supplied a bronchial cystic lesion in the right lower lobe. Termination of the segmental bronchus and scar formation proximal to the cyst suggested prior bronchial obliteration.

Genetic and morphological findings in progressive familial intrahepatic cholestasis (Byler disease [PFIC-1] and Byler syndrome): evidence for heterogeneity.

Byler disease (ByD) is an autosomal recessive disorder in which cholestasis of onset in infancy leads to hepatic fibrosis and death. Children who have a clinically similar disorder, but are not members of the Amish kindred in which ByD was described, are said to have Byler syndrome (ByS). Controversy exists as to whether ByD and ByS (subtypes of progressive familial intrahepatic cholestasis [PFIC]) represent one clinicopathological entity.

Liver biopsy findings in patients with juvenile rheumatoid arthritis receiving long-term, weekly methotrexate therapy.

We performed percutaneous liver biopsy in nine children who had received a weekly dose of methotrexate, 10 mg/m2 per week, for at least 3 years to address the concern about subclinical liver toxicity from single, weekly, low-dose methotrexate therapy for juvenile rheumatoid arthritis. No patient had clinical or biochemical evidence of liver injury. All biopsy results were interpreted as normal.

Renal histological changes secondary to ureteropelvic junction obstruction.

The treatment of children with apparent ureteropelvic junction obstruction is controversial. In an asymptomatic infant or child the decision to recommend pyeloplasty usually is based on interpretation of the renal scan. We retrospectively analyzed the renal biopsy obtained during pyeloplasty in 55 children.

Influence of vas deferens mobilization on rat fertility: implications regarding orchiopexy.

Children who undergo bilateral orchiopexy often have oligospermia or azoospermia in adulthood. This subfertility generally has been attributed to histological alterations in the cryptorchid testis. However, the possibility that an iatrogenic factor, such as mobilization of the vas deferens during orchiopexy, may have an adverse effect on fertility has not been studied.