Anorectal malformations associated with enteric dysganglionosis in Danforth's short tail (Sd) mice

BACKGROUND: The spontaneous mutant Danforth's short tail (Sd) mouse has been studied over the last 60 years from the morphological, embryological, and genetic point of view. The Sd mutation affects a gene essential to notochordal development, and the Sd mouse phenotype represents an analogue of human caudal regression syndrome. The Sd/Sd mouse presents different types of anorectal malformations (ARM) and was suggested as a simple and cheap model of investigation of ARM morphology and embryology. In the current study, the Sd mouse enteric nervous system (ENS) was thoroughly investigated with specific immunohistochemical markers. METHODS: Macroscopic analysis, normal histology, and immunohistochemical techniques for detecting neurofilaments (NF) and NOS1 were used to study ENS of 138 Sd mice and 25 controls. RESULTS: The surprising results of this study showed that Sd mutation is associated with different degrees of hypoganglionosis and aganglionosis. In 41% of Sd/SD-affected mice, the rectal pouch was aganglionic and in the remaining 58% was severely hypoganglionic. In addition, 4.1% of heterozygous mice presented a distal aganglionosis and 8.3% hypoganglionosis. CONCLUSIONS: These results suggest that Sd mutation independently affects distinct cell lines during early organogenesis, as notochord cells, ventral hingut endoderm, and neuroblasts migrating from neural crest cells. Comparing the Sd murine model with human pathology, this study confirms that the association between ARM and intestinal dysganglionosis is not rare and underlines the importance of detecting in every ARM patient the innervation abnormalities of rectal pouch and fistulas.     

Correlation between angiotensin-converting enzyme gene insertion/deletion polymorphism and kidney graft long-term outcome in pediatric recipients: a single-center analysis

BACKGROUND: Despite numerous advances in the areas of organ preservation, histocompatibility, and immunosuppression, chronic deterioration of organ allograft function, referred to as "chronic rejection," still remains the main obstacle to long-term graft survival. The common feature of chronic rejection is a concentric generalized graft arteriosclerosis associated with interstitial fibrosis that reflects an allogeneic injury to graft arteries, possibly worsened by other alloantigen-independent risk factors. The presence of the angiotensin I-converting enzyme (ACE) gene-deleted (D) allele has been associated, when in homozygosity, with increased risk of cardiovascular diseases and with an accelerated progression of organ damage in a variety of kidney diseases. In this study, we analyzed whether the insertion/deletion polymorphism of the ACE gene, because of its negative prognostic impact on cardiovascular and renal pathology, could have any influence on kidney graft survival in pediatric recipients. METHODS: DNA was isolated from peripheral blood mononuclear cells from 146 pediatric dialysis patients (mean age: 12.9 years) who received a first kidney graft at our center between December 1985 and July 1997. To rule out any bias due to acute graft losses, only 119 patients who reached a minimum of 12 months of graft survival were considered for statistical analysis. The insertion/deletion polymorphism of the ACE gene was detected using a polymerase chain reaction technique with two flanking primers. RESULTS: The results demonstrated that (i) the distribution of DD and non-DD (ID + II) genotypes was 36.1% (43 patients) and 63.8% (76 patients), respectively; (ii) actuarial graft survival at 7, 8, 9, and 10 years in patients with non-DD genotype was significantly higher than that in patients with DD genotype (7 years: 94.6% vs. 72.4%, P<0.05; 8 years: 94.6% vs. 62%, P<0.025; 9 years: 87.3% vs. 51.4%, P<0.025; 10 years: 76.3% vs. 25.7%, P<0.01). CONCLUSIONS: In conclusion, the above data indicate that DD genotype is associated in pediatric kidney graft recipients with a shorter long-term kidney graft survival and suggest a possible role of this genotype as a cofactor in the progression of nonimmunological injuries leading to chronic kidney graft failure.     

Latex allergy. An not rare intraoperative event

Latex allergy has become a real problem among both surgical staff (paramedics and physicians) and patients especially pediatric patients with urogenital malformations and spina bifida. Latex allergy is produced from both natural molecules which compose the substance produced from Hevea brasiliensis (rubber tree) and industrial additives contents in latex devices. Diagnosis of latex allergy may be carried out through a preoperative Prik-test. A characteristic of latex allergy reaction is the starting of symptoms (more than 15 minutes after allergen contact). Pathophysiology of latex allergy is the same of all allergic reactions; it is an antigen-antibody reaction and type I or II reaction may occur. There are a lot of devices that surgeons and anesthesists use in the operative theatre and that should not be used in presence of a patient with latex allergy. Guaranteed latex-free devices should always be present in store.     

Pyrrolo[3,2-c]quinoline derivatives: a new class of kynurenine-3-hydroxylase inhibitors.

A series of pyrrolo[3,2-c]quinoline derivatives were synthesised and evaluated as inhibitors of selected enzymes of the kynurenine pathway. 7-Chloro-3-methyl-1H-pyrrolo[3,2-c]quinoline-4-carboxylic acid (7a) was found to be a relatively potent and selective inhibitor of kynurenine-3-hydroxylase (KYN-3-OHase). A molecular modelling study showed a good superimposition of 7a with PNU-156561 and kynurenine the natural substrate of KYN-3-OHase.     

Monoamine oxidase-dependent metabolism of dopamine in the striatum and substantia nigra of

We describe the first case of chronic Chagas heart disease in México, in whom the illness was corroborated by means of the "xenodiagnosis". The patient had changing bundle branch block and also A-V block that required insertion of a pacemaker. This disease is seldom seen in Mexico.     

Chagasic heart disease in Mexico. 1st case diagnosed by positive xenodiagnosis

We describe the first case of chronic Chagas heart disease in México, in whom the illness was corroborated by means of the "xenodiagnosis". The patient had changing bundle branch block and also A-V block that required insertion of a pacemaker. This disease is seldom seen in Mexico.     

Relaxation therapy as an adjunct treatment for essential hypertension

Essential hypertension is one of the leading problems seen by a family physician, and it is not easily treated. In some instances, a behavioral therapy may serve as a useful adjunct to the pharmacological treatment of this disease. Evidence indicates that relaxation therapy in combination with medical treatment results in significantly greater reductions in systolic and diastolic blood pressure than the use of medical treatment alone. Progressive muscle relaxation, Benson's "relaxation response," hypnosis, and blood pressure biofeedback are the four most common behavioral treatments for essential hypertension. The first two of these are both effective and well suited to use in a family practice.     

The no-reflow phenomenon is a post-mortem artifact

Summary Post-ischemic reperfusion impairment, (no-reflow phenomenon), was studied in rats subjected to 8–30 minutes of global brain ischemia. During ischemia, rapid and complete loss of cerebral blood flow, EEG and³¹P-high energy phosphates (ATP/PCr) was observed.Brain intravascular perfusion defects were examined by injecting carbon blackintravenously in a group of rats with stable cardiopulmonary function and in another group subjected to rapid thoracotomy andintraarterial infusion of the carbon marker. Results indicate that global brain ischemic or non-ischemic control rats givenintraarterial carbon black after thoracotomy had varying degrees of vessel filling defects in brain resulting in pale tissue areas suggestive of impaired perfusion (no-reflow). All rats given carbon blackintravenously whether global brain ischemic or not, showed normal cerebrovascular filling of the carbon black and absence of pale tissue areas. In addition, post-ischemic cerebral reperfusion following 8–30 minutes global brain ischemia can reverse neuroelectric, energy metabolite and cerebral blood flow loss in rats whose cardiopulmonary function is not compromised.These findings indicate that the no-reflow phenomenon is an agonal or post-mortem artifact observed in the presence of cardiopulmonary failure.     

Lomest psychiatric case register: Old and new long-stay patients

Summary Data derived from a psychiatric case-register are presented on the attrition of the cohort of theold long-stay in-patients, and the accumulation of thenew long-stay cases in Lomest, a town in northern Italy, from 1975 to 1980. The characteristics of high user groups of out-patients attending the non-residential services are also described. The analysis seeks to provide some information on who has been left behind by the massive deinstitutionalization programme that has been carried out in Italy since 1970.     

Alterations in neurotrophin and neurotrophin receptor gene expression patterns in the rat central nervous system following perinatal Borna disease virus infection

Infection of newborn rats with Borna disease virus (BDV) leads to persistence in the absence of overt signs of inflammation. BDV persistence, however, causes cerebellar hypoplasia and hippocampal dentate gyrus neuronal cell loss, which are accompanied by diverse neurobehavioral abnormalities. Neurotrophins and their receptors play important roles in the differentiation and survival of hippocampal and cerebellar neurons. We have examined whether BDV can cause alterations in the neurotrophin network, thus promoting neuronal damage. We have used RNase protection assay to measure mRNA levels of the neurotrophins nerve growth factor (NGF), brain-derived neurotrophic factor (BDNF), and neurotrophin-3 (NT-3), and their trkC and trkB receptors, as well as the growth factors insulin-like growth factor I (IGF-1) and basic fibroblast growth factor (bFGF), in the cerebellum and hippocampus of BDV-infected and control rats at different time points p.i. Reduced mRNA expression levels of NT-3, BDNF and NGF were found after day 14 p.i. in the hippocampus, but not in the cerebellum, of newborn infected rats. Three weeks after infection, trkC mRNA expression levels were reduced in both hippocampus and cerebellum of infected rats, whereas decreased trkB mRNA levels were only observed in the cerebellum. Reduced trkC mRNA expression was confined to the dentate gyrus of the hippocampus, as assessed by in situ hybridization. TUNEL assay revealed massive apoptotic cell death in the dentate gyrus of infected rats at days 27 and 33 p.i. Increased numbers of apoptotic cells were also detected in the cerebellar granular layer of infected rats after 8 days p.i. Moreover, a dramatic loss of cerebellar Purkinje cells was seen after day 27 p.i. Our results support the hypothesis, that BDV-induced alterations in neurotrophin systems might contribute to selective neuronal cell death.