An unusual clinical presentation of bilateral schizencephaly

Summary We present a case with a characteristic magnetic resonance image (MRI) of bilateral open-lipped schizencephaly and atypical clinical presentation. The patient is still alive and in good health in her forties, she has never presented seizures, and although the motor dysfunction is well correlated with cerebral lobe involvement, neurobehavioral dysfunction is not proportional to the MR image of the cerebral malformation.

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Un cas inhabituel de schizencéphalie bilatérale

Résumé Nous présentons un cas de schizencéphalie bilatérale ouverte caractérisé par une présentation clinique atypique et une imagerie par résonance magnétique nucléaire caractéristique. La patiente est encore vivante, en bonne santé, à plus de 40 ans, elle n'a jamais présenté de crise comitiale et, bien que les troubles moteurs soient bien corrélés aux altérations cérébrales, les troubles neuro-comportementaux ne sont pas proportionnels aux images IRM de cette malformation cérébrale.

     

Intertubular growth in pure seminomas: associations with poor prognostic parameters

Clinical stage I seminomas are effectively treated with surgery raising concerns as to when to give adjuvant radiation therapy given the risk of secondary malignancies. A recent randomized trial found tumor size and rete testis invasion to be the strongest predictors of relapse in clinical stage I seminomas. These 2 parameters may be surrogate measures of tumor volume. Intertubular seminoma (ITS) of the testis describes the presence of neoplastic germ cells within the interstitium of the testis. These cells are detected away from the main macroscopic mass. Because ITS can infiltrate in a 3-dimensional fashion, it may also represent a measure of tumor volume not usually noted in standard pathology reporting. The goal of this study was to determine the incidence of ITS in pure seminomas and its association with other prognostic parameters. One hundred twenty consecutive pure seminomas surgically removed between 1998 and 2003 were evaluated. ITS was defined as the presence of an interstitial or intertubular growth pattern of tumor cells, which was noncontiguous with the main tumor and present at least 3 high-power fields away from the tumor mass. The average tumor size was 3.4 cm. Of the entire cohort of patients, which included pathological stages T1 through T3, 11% had invasion through the tunica albuginea, 51% had rete testis invasion, 51% had lymphovascular invasion, 93% had associated intratubular germ-cell neoplasia, and 36% had ITS. ITS was significantly associated with rete testis invasion ( P = .001). Logistic regression analysis looking at ITS, tumor size, patient age, and lymphovascular invasion revealed that only ITS was associated with rete testis invasion (RR, 4.1, P < .0001). ITS is present in a significant proportion of pure seminomas and has a significant association with rete testis invasion. The presence of ITS may therefore be an important prognostic factor, not only because it alters the calculated size of the tumor but also because it has an association with rete testis invasion.     

Apoptotic cell death in mouse models of GM2 gangliosidosis and observations on human Tay-Sachs and Sandhoff diseases

Tay-Sachs and Sandhoff diseases are autosomal recessive neurodegenerative diseases resulting from the inability to catabolize GM2 ganglioside by beta-hexosaminidase A (Hex A) due to mutations of the alpha subunit (Tay-Sachs disease) or beta subunit (Sandhoff disease) of Hex A. Hex B (beta beta homodimer) is also defective in Sandhoff disease. We previously developed mouse models of both diseases and showed that Hexa-/- (Tay-Sachs) mice remain asymptomatic to at least 1 year of age while Hexb-/- (Sandhoff) mice succumb to a profound neurodegenerative disease by 4-6 months of age. Here we find that neuron death in Hexb-/- mice is associated with apoptosis occurring throughout the CNS, while Hexa-/- mice were minimally involved at the same age. Studies of autopsy samples of brain and spinal cord from human Tay-Sachs and Sandhoff diseases revealed apoptosis in both instances, in keeping with the severe expression of both diseases. We suggest that neuron death is caused by unscheduled apoptosis, implicating accumulated GM2 ganglioside or a derivative in triggering of the apoptotic cascade.      

Ambient air and its potential effects on conception in vitro

Incidences of chemical air contamination (CAC) are common in assisted reproductive technology, but not reported in peer review format. Justified fear of car and industrial emissions clearly exists among reproductive specialists, but standards for air contents and gaseous emission limits have not been reported. Here, we describe air sampling methods and assay systems which can be applied to any laboratory or laboratory item. It was found that unfiltered outside air may be cleaner than high efficiency particulate air filtration (HEPA) filtered laboratory air or air obtained from incubators, due to accumulation of volatile organic compounds derived from adjacent spaces or specific laboratory products such as compressed CO2, sterile Petri dishes and other materials or devices known to release gaseous emissions. Specific groups of products such as anaesthetic gases, refrigerants, cleaning agents, hydrocarbons and aromatic compounds such as benzene and toluene are described. The latter were shown to accumulate specifically in incubators. Isopropyl alcohol was the most dominant product found, though it was not used by the laboratory staff. Concentrations of this agent were low in incubator air, indicating that it was probably absorbed by the water in the pan or by culture medium. Measures to counter CAC are proposed, including the use of activated carbon filters and oxidizing material placed in the central air handling systems, in separate free-standing units or even inside the incubators.      

Anatomic bases for liver transplantation

Summary This study gathers the anatomic implications for a good liver transplantation. During hepatic removal a left hepatic a.exists in 20% of cases; a right hepatic artery originating from the superior mesenteric a. (SMA) can be the only arterial supply in 9% of cases; the whole lesser omentum has to be removed and the SMA from 6 cm to its origin. The SMA must be freed from the celiac ganglia and its ostium removed with the celiac trunk in an aortic patch cut on the anterior side in order to avoid the renal ostia. During total hepatectomy, dissection of the portal triad is often difficult because of portal hypertension dilating accessory portal veins (parabiliary arcade) and pedicular lymphatics. Nerve plexuses are thick in front of the hepatic artery or behind the portal triad. Transection of triangular ligaments leads to the retrohepatic inferior vena cava (IVC) that must be freed from its posterior tributaries (right suprarenal vein and inferior phrenic veins flowing either into the IVC or into the hepatic veins). One big problem during hepatic replacement is the biliary anastomosis which must be well irrigated. In the recipient, dissection up to the hilum preserves hepatic and pancreatico-duodenal pedicles. The biliary tract of the graft must be cut low, behind the pancreas, and several centimeters of the gastroduodenal artery must be preserved to save hepatic and gastroduodenal pedicles.

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Bases anatomiques de la transplantation hépatique

Résumé Ce travail rassemble les notions anatomiques nécessaires au bon déroulement d'une transplantation hépatique. Le prélèvement du greffon doit enlever tout le petit omentum contenant une éventuelle a. hépatique gauche née de l'a. gastrique gauche (20%) et emporter l'a. mésentérique supérieure jusqu'à 6 cm de son origine pour ne pas oublier une a. hépatique droite née de cette dernière: son ostium est pris avec le tronc clique dans un patch aortique découpé sur la face antérieure. Lors de l'hépatectomie totale, la dissection du pédicule hépatique est rendue délicate par l'hypertension portale qui dilate les veines portes diets accessoires (arcade parabiliaire) et les lymphatiques pédiculaires. Les plexus nerveux sont riches devant l'artère hépatique et derrière le pédicule. La section des ligaments triangulaires droit et gauche amène à la veine cave inférieure (VCI) rétro-hépatique qu'il faut libérer de ses afférences postérieures (en particulier la veine surrénale principale droite toujours haut située et les veines phréniques inférieures qui s'abouchent soit dans la VCI soit dans les veines hépatiques du carrefour). Lors du remplacement, l'anastomose biliaire doit être vascularisée. Chez le receveur la dissection jusqu'au hile permet de conserver les pédicules. La voie biliaire du greffon doit être coupée bas derrière le pancréas et les premiers centimètres de l'artère gastro-duodénale conservés pour préserver les pédicules hépatique et pancréaticoduodénal.

     

Evaluating self-managed learning--Part 3: Developing leaders in a US health system

The last in a series which evaluates the use of self-managed learning in management development processes within health care settings. Analyses the evolution of the leadership development programme established by the Lutheran Health System in the mid-West USA.     

Synthesis, corticotropin-releasing factor receptor binding affinity, and pharmacokinetic properties of triazolo-, imidazo-, and pyrrolopyrimidines and -pyridines

The synthesis and CRF receptor binding affinities of several new series of N-aryltriazolo- and -imidazopyrimidines and -pyridines are described. These cyclized systems were prepared from appropriately substituted diaminopyrimidines or -pyridines by nitrous acid, orthoester, or acyl halide treatment. Variations of amino (ether) pendants and aromatic substituents have defined the structure-activity relationships of these series and resulted in the identification of a variety of high-affinity agents (Ki's < 10 nM). On the basis of this property and lipophilicity differences, six of these compounds (4d,i,n,x, 8k, 9a) were initially chosen for rat pharmacokinetic (PK) studies. Good oral bioavailability, high plasma levels, and duration of four of these compounds (4d,i,n,x) prompted further PK studies in the dog following both iv and oral routes of administration. Results from this work indicated 4i,x had properties we believe necessary for a potential therapeutic agent, and 4i1 has been selected for further pharmacological studies that will be reported in due course.