galen arteriovenous malformation: Topics by WorldWideScience.org

Susceptible gene single nucleotide polymorphism and hemorrhage risk in patients with brain arteriovenous malformation

Hereditary hemorrhagic telangiectasia (Osler-Rendu-Weber disease) is a genetic disorder with autosomal dominance, variable penetrance, and an estimated prevalence of 1/10,000 inhabitants in France. Diagnosis is based on clinical criteria including epistaxis, telangiectasia, visceral manifestations, and familial occurrence. Pulmonary arteriovenous malformations, present in 15-33% of patients, are its primary visceral complications. The disease may be revealed by infectious and ischemic neurological manifestations due to paradoxical embolism. The high frequency of neurologic complications even in asymptomatic patients justifies systematic screening for pulmonary arteriovenous malformations. Treatment of these malformations is based on percutaneous transcatheter coil embolization of the feeding artery. Pulmonary arterial hypertension is rare in this disease. It may be due to systemic ...

2005-11-01

British Library Electronic Table of Contents (United Kingdom)

The relationship between single nucleotide polymorphism (SNP) of interleukin-17 (IL-17A), transforming growth factor @b (TGF-@b), as well as its receptor (TGFR-@b2) and susceptibility to intracerebral hemorrhage in patients with brain arteriovenous malformation (BAVM) was investigated in the present study. A total of 53 patients with BAVM and 120 healthy controls were recruited, all of whom were Han Chinese from South China. There were no statistically significant differences in the IL-17A-197 guanine/adenine (G/A) or TGF-@b1-509 cytosine/thymine (C/T) genotypes or gene frequencies between BAVM patients and controls (p>0.05), but the gene frequency of the TGFR-@b2-875 A/G genotype in patients with BAVM was significantly higher (p<0.05). Furthermore, the frequencies of the G allele of IL-17...

2011-01-01

A Study of PRT-201 Administered Immediately After Arteriovenous Fistula Creation in Patients With Chronic Kidney Disease

Induction of mortality and malformation in Xenopus laevis embryos by water sources associated with field frog deformities.

Chronic Kidney Disease

2011-07-19

A Fuzzy System for Helping Medical Diagnosis of Malformations of Cortical Development

Water samples from several ponds in Minnesota were evaluated for their capacity to induce malformations in embryos of Xenopus laevis. The FETAX assay was used to assess the occurrence of malformations...Full Text Available

1998-12-01

Immunohistochemical expression of p63 in human prenatal tooth primordia

Malformations of the cerebral cortex are recognized as a common cause of developmental delay, neurological deficits, mental retardation and epilepsy. Currently, the diagnosis of cerebral cortical...Full Text Available

2007-06-01

DEFF Research Database (Denmark)

development, histochemical, human, malformation, p63 gene, tooth

2005-01-01

Congenital Inner Ear Malformation: Three Dimensional Volume Rendering Image Using MR CISS Sequence

KoreaScience (Korea)

Full Text Available

2003-10-01

Pelvic CT for the prognostic evaluation of anorectal malformation

Congenital anal anomalies in two families with the Opitz G syndrome.

The anatomical patterns of anorectal malformations have so far been studied according to the principles which inspire Pena's technique for the surgical treatment of anorectal anomalies. Thus, the diagnostic study of anorectal malformation has by the authors been considered a work of classification, but of identification. Among the diagnostic procedures in use in author's Institute, preoperative CT of pelvis is performed to assess the presence and to define the development of muscular sphincteric structure towards prognostic evaluation of continence, the major long-term goal. Seven patients, aged 1-30 months, with anorectal anomalies were studied with preoperative CT of the pelvis. CT scans showed well-developed sphinteric muscles in 3 patients (2 with recto-ureteral and 1 with recto-vestibular fistulas), poorly-developed muscular structure in 3 patients (with recto-vaginal, cloacal and prostatic fistulas), absence of muscular structure in 1 ...

1989-01-01

A Case Report of a Patient with Hereditary Hemorrhagic Telangiectasia Treated Successively with Thalidomide and Bevacizumab

Five children from two families presented to a regional neonatal surgical unit between 1959 and 1984 with congenital anal anomalies and other malformations resulting from an autosomal dominant inherited...Full Text Available

1987-11-01

Effect of percutaneous transluminal angioplasty in insufficiency of arteriovenous fistula for hemodialysis

Hereditary hemorrhagic telangiectasia is characterized by mucocutaneous and visceral telangiectasia and involves several organs with vascular malformations. It is an autosomal dominant disease and is...Full Text Available

Successful coil embolization of an unusual aorto-superior mesenteric vein fistula.

To evaluate the effectiveness of percutaneous transluminal angioplasty(PTA) and to determine the factors affecting the long-term patency rate in the management of insufficient hemodialytic arteriovenous fistula(AVF). Forty-nine cases of insufficient hemodialytic AVF were treated in 44 patients(native AVF:20, graft AVF:29, M:17, F:27, Age:22-70 years). In 28 thrombus patients, thrombolysis was performed with urokinase, and was followed by PTA. The initial success rate and complications of PTA were evaluated. According to the site and length of the stenosis, type and age of the AVF, the presence or abscence of thrombus, a history of diabetic mellitus, the patient's age, and the duration of renal failure, patency rates were compared within each subgroup using the Kaplan-Meier logrank test. The initial success rate of PTA for insufficient hemodialytic AVF was 88%(43/49), the patency rate of PTA was 67% at 6 months, and 50% at 12 months. The initial success ...

1999-06-01

A delayed case of renal artery pseudoaneurysm presented with gross hematuria and azotemia in solitary kidney following percutaneous nephrostomy: treated by transcatheter coil embolization.

The aortovenous fistulas are rare, most of them are aortocaval fistula. The non-caval communication of the fistula is sparse. Herein we report a 47-year-old female diagnosed as traumatic aorto-superior mesenteric vein (Ao-SMV) fistula. The abdominal pain, fullness, and frank bruit on abdominal auscultation suggested the diagnosis of an intra-abdominal arteriovenous fistula. The diagnosis of Ao-SMV was further confirmed by the computed tomography (CT) and aortography. The fistula was successfully treated with transcatheter coil embolization. This is the first case of Ao-SMV fistula. It provides an alternative option of treatment other than conventional surgery. PMID:19327940

2009-03-26

Percutaneous treatment of thrombosed native arteriovenous dialysis fistula insufficiency: efficacy of mechanical thrombectomy with using the stone basket

Renal arteriovenous fistula or pseudoaneurysms developing after invasive procedures are either asymptomatic or show only transient symptoms. We describe here a case of symptomatic renal artery pseudoaneurysm that presented with gross hematuria and azotemia. The pseudoaneurysm of renal artery was diagnosed by ultrasonography and abdominal magnetic resonance imaging (MRI), and it was successfully treated by percutaneous transcatheter coil embolization. A high index of suspicion is essential for the early diagnosis and treatment of delayed gross hematuria in patients with history of percutaneous nephrostomy. PMID:17180737

2006-12-19

What is the heritable component of spinal deformities in the European sea bass (Dicentrarchus labrax)?

We wanted to evaluate the procedural success after percutaneous treatment of thrombosed native arteriovenous dialysis fistula insufficiency and the efficacy of performing mechanical thrombectomy with using the stone basket. From March 2004 to June 2005, 36 thrombosed native hemodialysis access shunts in the upper limbs (brachiocephalic fistulas: 16 and radiocephalic fistulas: 20) were percutaneously treated in 30 patients. Declotting procedures were performed with using urokinase (100,00-200,000 unit) and manual catheter-directed thrombo-aspiration in all the patients. Angioplasty (6 mm in diameter and 4 cm in length) was performed at the identified area of the stenosis and /or with maceration of the thrombus. In 14 cases with massive thrombosis that was refractory to the above mentioned declotting procedures, mechanical thrombectomy with using a Wittich nitinol stone basket (Cook, Bloomington, IN) was performed. Data regarding the procedural success rate and the ...

2006-06-15

British Library Electronic Table of Contents (United Kingdom)

Malformations in farmed fish are a prime research topic as it has a major impact on fish welfare and on the economical value of the product. In this study, the genetic component of spine deformities was estimated in European sea bass, a major species of Mediterranean aquaculture, with the use of a posteriori reconstruction of pedigree with microsatellites. Our population exhibited a very high malformation rate (81%), probably due to intense swimming of fish during pre-growing. The heritability of global spine deformities was evaluated to be 0.21?0.04 on the underlying scale (0.33?0.06 for lordosis, 0.13?0.04 for scoliosis). Deformities scored by an internal or external examination turned out to be genetically the same trait (genetic correlations>0.9), although phenotypic correlations were ...

2009-01-01

Separation and Measurement of Plant Alkaloid Enantiomers by RP-HPLC Analysis of their Fmoc-Alanine Analogs

Normal and abnormal neuronal migration during brain development

Plants often synthesize secondary metabolites that are enantiomers. Enantiomers can cause very different physiological responses. Ammodendrine (1) and anabasine (2) are teratogens that can cause congenital malformations in livestock and enantiomeric forms of each have been found in Lupinus spp. an...

Normal and abnormal fetal brain development during the third trimester as demonstrated by neurosonography

Conceptual and factual advances in understanding neuronal migration in the past two decades have provided new insight into the pathogenesis of brain malformations at the cellular, molecular, and functional levels. Some of these results may have direct implications in understanding the consequences of ionizing radiation on the fetal central nervous system in utero. (orig.).

The multiplanar neurosonographic examination of the fetus enables superb visualization of brain anatomy during pregnancy. The examination may be performed using a transvaginal or a transfundal approach and it is indicated in patients at high risk for CNS anomalies or in those with a suspicious finding during a routine examination. The purpose of this paper is to present a description of the normal brain and of abnormal findings usually diagnosed late in pregnancy, including malformations of cortical development, infratentorial anomalies, and prenatal insults.

2006-02-01

MRI of the foetal brain

Computed tomography in migratory disorders of human brain development

Ultrasound examinations for foetal brain abnormalities have been a part of the routine antenatal screening programme in the UK for many years. In utero brain magnetic resonance imaging (MRI) is now being used increasingly successfully to clarify abnormal ultrasound findings, often resulting in a change of diagnosis or treatment plan. Interpretation requires an understanding of foetal brain development, malformations and acquired diseases. In this paper we will outline the technique of foetal MRI, relevant aspects of brain development and provide illustrated examples of foetal brain pathology.

2007-04-01

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Percutaneous intervention in the menagement of insufficient native arteriovenous hemodialysis fistulae

Computed tomographic findings in developmental brain anomalies are more easily classified when the system used is based on embryogenesis related to morphology. Analysis of computed tomographic findings in a series of 154 patients with brain anomalies (Chiari malformation not included) revealed that specific examples of abnormalities occurring in major stages of brain development may be recognized by computed tomography. This paper deals with a group of patients with abnormalities that reflect a disturbance in neuronal migration. (orig.).

Diabetic patients have abnormal cerebral autoregulation during cardiopulmonary bypass

To determine the effectiveness and patency of percutaneous intervention in insufficient native arteriovenous hemodialysis fistulae (AVFs). Between March 1997 and September 2001, 67 cases of insufficient native AVFs resulted from central vein lesion, PTA was performed in 48 cases, and thrombolytic therapy with or without PTA in 11. In eight of the cases, in which central vein stenosis had led to the insufficency, percutaneous transluminal angioplasty (PTA) was performed, and in three of the eight, a stent was inserted. Angiography findings and complications, as well as success and patency rates in the non-thrombosis and thrombosis group, were evaluated; the central vein lesion group was analysed separately. Among 84 lesions observed at angiography, three were 54 cases of stenosis, 17 of occlusion, and 13 of combined thrombosis. The lesions were located in a proximal vein (n=51), distal vein (n=14), artery (n=6), and at the site of anastomosis (n=13). In the central ...

2002-11-01

Sprengel deformity in a nine-year-old boy: diagnosis and therapy; Sprengel-Deformitaet bei einem neunjaehrigen Jungen: Diagnostik und Therapie

We tested the hypothesis that insulin-dependent diabetic patients with coronary artery bypass graft surgery experience altered coupling of cerebral blood flow and oxygen consumption. In a study of 23 patients (11 diabetics and 12 age-matched controls), cerebral blood flow was measured using 133Xe clearance during nonpulsatile, alpha-stat blood gas managed cardiopulmonary bypass at the conditions of hypothermia and normothermia. In diabetic patients, the cerebral blood flow at 26.6 +/- 2.42 degrees C was 25.3 +/- 14.34 ml/100 g/min and at 36.9 +/- 0.58 degrees C it was 27.3 +/- 7.40 ml/100 g/min (p = NS). The control patients increased cerebral blood flow from 20.7 +/- 6.78 ml/100 g/min at 28.4 +/- 2.81 degrees C to 37.6 +/- 8.81 ml/100 g/min at 36.5 +/- 0.45 degrees C (p less than or equal to 0.005). The oxygen consumption was calculated from jugular bulb effluent and increased from hypothermic values of 0.52 +/- 0.20 ml/100 g/min in diabetics to 1.26 +/- 0.28 ml/100 g/min (p = 0.001) ...

1990-11-01

Three-dimensional cranio-facial computed tomography

Sprengel deformity is a congential malformation with usually unilateral elevation and medial rotation of the scapula. An omovertebral bone is generally present. Associated skeletal malformations are frequently present. Though functional impairment is mild, the cosmetic and psycho-social impairment can be considerable. The prognosis of early surgical therapy is good. The presented case also justifies surgical correction in the older child. (orig.) [German] Die Sprengel-Deformitaet ist eine angeborene Fehlbildung mit meist einseitiger Elevation und Medialrotation der Skapula. Meist ist ein Os omovertebrale vorhanden. Assoziierte skelettale Fehlbildungen sind haeufig. Obgleich funktionell wenig einschraenkend, ist die kosmetische und psycho-soziale Beeintraechtigung oft erheblich. Die Prognose der fruehen operativen Therapie ist gut. Der vorliegende Fall ermutigt zur operativen Therapie auch im hoeheren Kindesalter. (orig.)

2003-02-01

Reticulate eruptions. Part 1: Vascular networks and physiology

Computed tomography allows today to reconstruct three-dimensional (eD) images fram axial scans. The authors report their experience in cranio-facial pathology achived in two Departments of Radiology (University of Trieste, Italy and University of Standford, California). 3D images have been realized using two different softwares, one of which allows to reconstruct both soft tissue and bone structures. The application in maxillo-facial traumas, cranio-facial malformations and head tumours are disscussed. 3D images turned out to be very useful for the optimal visualization and for the spatial demostration of the lesion and have potential applications in cranio-facial surgery and radiotherapy.

1986-01-01

British Library Electronic Table of Contents (United Kingdom)

Abstract Reticulate pattern is one of the most important dermatological signs of a pathological process involving the superficial vascular networks. Vascular malformations, such as cutis marmorata congenita telangiectasia and benign forms of livedo reticularis, and sinister conditions, such as meningococcal meningitis or Sneddon's syndrome, can all present with a reticulate pattern. The clinical presentation and morphology is determined by the nature and extent of the underlying pathology and the involvement of a particular vascular network. This review has been divided into four instalments. In the present paper, we discuss the anatomy and physiology of the complex network of vascular structures that support the function of the skin and subcutis.

2011-01-01

Isolated laevocardia and corrected transposition of the great vessels with total situs inversus

Induction of sterility in adults of Earias vittella Fabricius through late pupal irradiation

Isolated laevocardia combined with corrected transposition of the great arteries and complete situs inversus is rare. Due to discordant interrelationships between atria and ventricles and between ventricles and great arteries, both anomalies result in a normal circulation. In the presented case no associated cardiac malformations were present and the 55-year-old woman remained symptom-free for a long time until she developed an acquired valve disease. A systematic approach to chamber localisation in complex morphologic lesions of the heart is reviewed, and the value of CT, which can be applied successfully in older children and adults is demonstrated. (orig.).

Magnetic Resonance Perfusion Imaging in Malformations of Cortical Development

Studies on pupal irradiation of Earias vittella Fabricius, were undertaken to determine the optimum dose for sterile and competitive adults. Late pupae were irradiated with gamma radiation doses ranging from 150 to 300 Gy. Emerging adults were normal and without malformation. When males that emerged from irradiated pupae were crossed with normal female, fecundity as well as longevity was comparable to control, but there was drastic reduction in percent egg hatch in all the treatments. However, when females emerging from irradiated pupae were crossed with normal male, there was reduction in the fecundity as well as fertility and this effect was dose dependent. (author).

Corrected transposition of the great arteries

Background: Malformations of cortical development vary in neuronal maturity and level of functioning. Purpose: To characterize regional relative cerebral blood volume (rCBV) and difference in first moment transit time (TTfm) in polymicrogyria and cortical tubers using magnetic resonance (MR) perfusion imaging. Material and Methods: MR imaging and dynamic T2*-weighted MR perfusion imaging were performed in 13 patients with tuberous sclerosis complex, 10 with polymicrogyria, and 18 controls with developmental delay but no macroscopic brain abnormality. Regions of interest were placed in cortical tubers or polymicrogyric cortex and in the contralateral normal-appearing side in patients with malformations. In 'control' subjects, regions of interest were placed in the frontal and parietal lobes in both hemispheres. The rCBV and TTfm of the tuber/contralateral side (rCBVRTSC and TTFMTSC) as well as those of the polymicrogyria/contralateral side ...

2007-10-01

Utilization of intestinal triglyceride-rich lipoproteins in mammary gland of cows.

The corrected transposition of the great arteries is an usual congenital cardiac malformation, which consists of transposition of great arteries and ventricular inversion, and which is caused by abnormal development of conotruncus and ventricular looping. High frequency of associated cardiac malformations makes it difficult to get accurate morphologic diagnosis. A total of 18 cases of corrected transposition of the great arteries is presented, in which cardiac catheterization and angiocardiography were done at the Department of Radiology, Seoul National University Hospital between September 1976 and June 1981. The clinical, radiographic, and operative findings with the emphasis on the angiocardiographic findings were analyzed. The results are as follows: 1. Among 18 cases, 13 cases have normal cardiac position, 2 cases have dextrocardia with situs solitus, 2 cases have dextrocardia with situs inversus and 1 case has levocardia with situs ...

1981-12-01

Endoluminal vascular prostheses; Endoluminale Gefaessprothesen

Elution profiles of total lipoproteins, apolipoprotein B (apoB) concentrations in lipoproteins, and plasma triglyceride (TG) levels were examined in early-, late-, and non-lactating cows. Additionally, arteriovenous (A-V) differences were also measured to elucidate the uptake of TG and apoB-containing lipoproteins in mammary gland. Non-lactating cows showed three major peaks corresponding to triglyceride-rich lipoprotein (TRL), low density lipoprotein (LDL), and high density lipoprotein (HDL) fraction, whereas both early- and late-lactating cows revealed two peaks corresponding to TRL and HDL. The peak area of TRL in early- and late-lactating cows were significantly (p < 0.05) smaller than that in non-lactating cows. The plasma TG levels and apoB-48 concentrations of TRL in early- and late-lactating cows were also significantly (p < 0.01) lower. Furthermore, early lactating cows showed significantly (p < 0.05) larger A-V differences in both plasma TG and ...

1999-10-01

The chance finding of an aneurysm of the right sinus of Valsalva in an 11-year-old child with a ventricular septal defect and a pericardial effusion

Endoluminal vascular prostheses that can be implanted by percutaneous routes represent the most recent development in vascular interventional radiology. Various commercially available types of prosthesis are presented and the construction principles and applications are described. At present secure indications for the implantation of endoluminal prostheses are limited to the elimination of aneurysms and arteriovenous fistulae of the large vessels near the trunk in sections that do not cross a joint. The wide use in peripheral occlusive diseases cannot yet be recommended because confirmed data are not available. (orig.) [German] Endoluminale Gefaessprothesen, die perkutan implantierbar sind, stellen die juengste Entwicklung in der vaskulaeren interventionellen Radiologie dar. Verschiedene kommerziell erhaeltliche Prothesentypen werden vorgestellt, ihr Konstruktionsprinzip und ihre Applikation erlaeutert. Die gesicherten Indikationen zur endoluminalen ...

2000-06-01

British Library Electronic Table of Contents (United Kingdom)

Ventricular septal defects can occur as part of other congenital cardiac malformations or as an isolated finding. Aneurysms of the sinus of Valsalva are rare, most commonly involving the right or noncoronary sinuses. They can be congenital or acquired through infection, trauma, or degenerative diseases. They frequently co-exist with ventricular septal defects, aortic valve dysfunction, or other cardiac abnormalities. More commonly, sinus of Valsalva aneurysms are diagnosed after the clinical sequelae of rupture. Several etiologic factors may lead to the development of pathologic pericardial effusion and the detection of pericardial effusion was one of the first applications of echocardiography to gain widespread acceptance. We present a case of a chance finding of an aneurysm of the right ...

2011-01-01

Magnetization transfer contrast effect on multislice fast spin echo

Fetal MRI of pathological brain development

Magnetization transfer contrast (MTC) on fast spin echo (FSE) images was evaluated in normal brain tissue as well as in brain tumors to better understand contrast of FSE images. On multislice FSE images, attenuation of the signal intensity of brain tissue due to MTC is observed. The rate of MTC (MTR) is obtained by comparing with additional single slice images. The effect of signal attenuation is greater on images with smaller interslice gap, larger number of slices, and longer echo train length. MTR of brain tumors is less than that of normal brain tissue. Among them, meningiomas have relatively high MTR, and cavernous malformation has relatively low MTR. Determination of MTC can be easily specified by obtaining multislice and single slice FSE images, and it may help differentiate brain tumors. (author).

1994-03-01

Endoscopic management of congenital esophageal stenosis

Because of the superior tissue contrast, high spatial resolution, and multiplanar capabilities, fetal magnetic resonance imaging (MRI) can depict fetal brain pathologies with high accuracy. Pathological fetal brain development may result from malformations or acquired conditions. Differentiation of these etiologies is important with respect to managing the actual pregnancy or counseling future pregnancies. As a widened ventricular system is a common hallmark of both maldevelopment and acquired conditions, it may cause problems in the differential diagnosis. Fetal MRI can provide detailed morphological information, which allows refinement of the diagnosis of ventricular enlargement in a large number of cases. Systematic work-up of morphological details that may be recognized on MR images provides an approach for achieving a correct diagnosis in cases of ventricle enlargement. (orig.)

2006-02-01

British Library Electronic Table of Contents (United Kingdom)

Background/Purpose: Congenital esophageal stenosis (CES) is a rare malformation. Endoscopic dilations represent a therapeutic option. This study retrospectively evaluated the efficacy and safety of a conservative treatment of CES. Patients and Methods: Patients diagnosed with CES since 1980 by a barium study or endoscopy were reviewed. Endoscopic ultrasonography (Olympus UM-3R-20-MHz radial miniprobe, Olympus Corporation, Tokyo, Japan), available from 2001, allowed for the differential diagnosis of tracheobronchial remnants (TBR) and fibromuscular hypertrophy (FMH) CES. All children underwent conservative treatment by endoscopic dilations (hydrostatic and Savary). Results: Forty-seven patients (20 men) had CES. Fifteen were associated with esophageal atresia; and 8, with Down syndrome. Mea...

2011-01-01

Early-stage focal nodular hyperplasia: US/CT/MR features correlated with histology

Chernobyl accident: the crisis of the international radiation community

Two cases of focal nodular hyperplasia (FNH), in which ultrasound (US), computed tomography (CT), and magnetic resonance imaging (MR) studies detected an atypical hemorrhagic pattern associated with an intrahepatic arterio venous malformation (AVM) around the growths, are presented. In both cases, histology demonstrated a very early regenerative stage and necrotic-hemorrhagic areas within the lesions. In these cases, the analysis of radiological findings, surgical specimens and histology seemed to confirm the pathogenetic hypothesis suggested by Wanless: in normal liver parenchyma, a ``blood steal`` phenomenon due to congenital or acquired intrahepatic AVM could cause ischemic damage, appearing as a hemorrhagic necrotic area, the extent of which depends on the degree of residual portal supply. (orig.)

1994-02-01

Fecal incontinence in operated cases for anorectal malformations

The information given in the present report about the Chernobyl accident and its radiological consequences indicates a serious crisis of the international radiation community. The following signs of this crises can be discerned: The international radiation community did not recognize the real reasons of the accident for a long time. It could not make a correct assessment of the damage to the thyroid of the affected populations of Belarus, Russia and the Ukraine. Up to present time it rejects the reliable data on hereditary malformations. It is not able to accept reliable data on the increase in the incidence in all categories of people affected by the Chernobyl accident. The international radiation community supported the Soviet authorities in their attempts to play down the radiological consequences of the Chernobyl accident for a long time. (author)

1998-03-01

Biological Effects after Prenatal Irradiation

In spite of great progress in surgical treatment of anorectal malformations, fecal incontinence is still, in variable degrees, a frequent and unpleasant postsurgical sequela. The most frequent causes of incontinence are: 1) the incorrect placement of the pulled-through colon in the levator ani and sphincteric muscular complex during abdomino-perineal surgical procedures; 2) the poor development of sphinteric musculature; 3) the associated sacral anomalies. Postoperative CT helps to evaluate all the above-mentioned conditions, in view of possible new surgical procedure for improving continence (besides postoperative CT can help in choosing the more suitable surgical technique). Nine patients, aged 3 to 13 years (2 with good continence and 7 with various degrees of incontinence), were studied with pelvic postoperative CT. In the cases (2) with good continence the CT picture was: good development of sphincteric musculature and neo-anorectum correctly placed into ...

1989-01-01

Intestinal circulation during inhalation anesthesia

A Task Group of the International Commission on Radiological Protection (ICRP) has finished a report Biological Effects after Prenatal Irradiation (Embryo and Fetus) which has been approved by the Main Commission and Will be Published. Some new important scientific data shall be discussed in this contribution. During the preimplantation period lethality of the mammalian embryo is the dominating radiation effect. However, in mouse strains with genetic predispositions it has been shown that also malformations can be caused. This effect is genetically determined and its mechanisms is different from the induction of malformations during major organogenesis. Radiation exposures during this prenatal period leads ato an increase of genomic instability of cells in the normal appearing fetuses. These radiation effects can be transmitted to the next generation. A renewed analysis of individuals with severe mental retardation after exposures during the ...

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Intestinal circulation during inhalation anesthesia

This study was designed to evaluate the influence of inhalational agents on the intestinal circulation in an isolated loop preparation. Sixty dogs were studied, using three intestinal segments from each dog. Selected intestinal segments were pumped with aortic blood at a constant pressure of 100 mmHg. A mixture of /sub 86/Rb and 9-microns spheres labeled with /sup 141/Ce was injected into the arterial cannula supplying the intestinal loop, while mesenteric venous blood was collected for activity counting. A very strong and significant correlation was found between rubidium clearance and microsphere entrapment (r = 0.97, P less than 0.0001). Nitrous oxide anesthesia was accompanied by a higher vascular resistance (VR), lower flow (F), rubidium clearance (Cl-Rb), and microspheres entrapment (Cl-Sph) than pentobarbital anesthesia, indicating that the vascular bed in the intestinal segment was constricted and flow (total and nutritive) decreased. Halothane, enflurane, and isoflurane ...

1985-04-01

Treatment of proliferative haemangiomas with the 585 nm pulsed dye laser.

This study was designed to evaluate the influence of inhalational agents on the intestinal circulation in an isolated loop preparation. Sixty dogs were studied, using three intestinal segments from each dog. Selected intestinal segments were pumped with aortic blood at a constant pressure of 100 mmHg. A mixture of _8_6Rb and 9-microns spheres labeled with "1"4"1Ce was injected into the arterial cannula supplying the intestinal loop, while mesenteric venous blood was collected for activity counting. A very strong and significant correlation was found between rubidium clearance and microsphere entrapment (r = 0.97, P less than 0.0001). Nitrous oxide anesthesia was accompanied by a higher vascular resistance (VR), lower flow (F), rubidium clearance (Cl-Rb), and microspheres entrapment (Cl-Sph) than pentobarbital anesthesia, indicating that the vascular bed in the intestinal segment was constricted and flow (total and nutritive) decreased. Halothane, enflurane, and isoflurane anesthesia ...

The mouse formin (Fmn) gene: Genomic structure, novel exons, and genetic mapping

Haemangiomas usually develop within the first few weeks of life, most regressing spontaneously before the age of 7 years. Some may ulcerate or compromise a vital function, in which case systemic corticosteroids, surgery or radiotherapy may be helpful. All of these treatment modalities are associated with significant morbidity. Treatment with the 585 nm flashlamp pulsed dye laser is safe and effective in the management of superficial vascular malformations. We report seven patients, under 12 months of age, who presented with proliferative haemangiomas, causing functional impairment. Ulcerated lesions were present in four patients. The patients were treated with the 585 nm pulsed dye laser (fluences 7.0-9.25 J/cm2), at intervals of 4-8 weeks. All of the lesions showed a significant reduction in size, together with improvement in the colour and integrity of the overlying skin. Treatment with the 585 nm pulsed dye laser should be considered in the management of infants ...

1996-04-01

Some considerations on the processes of axon bundling and the early phases of capillarization in the CNS

Mutations in the mouse formin (Fmn) gene, formerly known as the limb deformity (ld) gene, give rise to recessively inherited limb deformities and renal malformations or aplasia. The Fmn gene encodes many differentially processed transcripts that are expressed in both adult and embryonic tissues. To study the genomic organization of the Fmn locus, we have used Fmn probes to isolate and characterize genomic clones spanning 500 kb. Our analysis of these clones shows that the Fmn gene is composed of at least 24 exons and spans 400 kb. We have identified two novel exons that are expressed in the developing embryonic limb bud as well as adult tissues such as brain and kidney. We have also used a microsatellite polymorphism from within the Fmn gene to map it genetically to a 2.2-cM interval between D2Mit58 and D2Mit103. 36 refs., 6 figs., 1 tab.

1997-02-01

Pre-operative MRI of anorectal anomalies in the newborn period

Bundling of axons and capillarization of the neuroepithelium represent two of the numerous important events in brain development. Prerequisite for these two processes is a directed growth in the matrix-containing intercellular space before a final pattern is formed. The formation of the optical nerve in the region of the retina served as example to show that an extracellular substance with adhesive properties, the glycoprotein fibronectin, plays an important role during bundling and directed growth the axons. However, only small amounts of fibronectin are detected in the region of the capillary sprouts that penetrate into the neuroepithelium. In this area other substances, especially basement membrane components are present, e.g. for anchorage and stabilization. Hence, intercellular substances are also involved in the morphogenesis of the brain. Brain development comprises a great number of individual steps. Their knowledge is the prerequisite for an analysis of teratogenic and ...

Gene expression analysis after low dose ionising radiation exposure of the developing organism

Nine infants (six boys, three girls) with anorectal anomalies were examined in the immediate newborn period, prior to corrective surgery, with MRI. Three high, one intermediate and five low anomalies were found at MRI - one patient with a `low` lesion was subsequently found at surgery 2 months later to have a high anorectal anomaly. This infant had passed meconium per urethram soon after the MRI study, prompting the need for a protective colostomy and stressing the importance of a thorough clinical examination of babies with anorectal malformations. The MRI results and findings at surgery were in agreement in all other patients (n=8). Hydronephrosis was evident in two and renal agenesis in one patient. Sacrococcygeal hypoplasia was found in two and two hemivertebrae in one infant. No spinal cord lesion was identified. One fistula was evident on MRI but four were later found at surgery. Uniformly hyperintense T1 signal meconium was seen in all nine newborns, ...

1995-11-01

Ambient air pollution and congenital heart disease: a register-based study.

Measuring gene expression using microarrays is relevant to many areas of biology and medicine, such as follow up of developmental stages and diseases onset, and treatment study. Since there can be tens of thousands of distinct probes on an array, each micro array experiment can accomplish the equivalent number of genetic tests in parallel. Arrays have therefore dramatically accelerated many types of investigations. For example, microarrays can be used to identify stress response genes by comparing gene expression in challenged versus normal cells. In the Molecular and Cellular Biology lab (MCB), the micro array experiments are performed within the Genomic Platform, fully equipped to analyse either the behaviour of bacteria during long space flight, the effect of low dose ionising radiation on the developing organism in mice, or the human individual radiation sensitivity. For the low dose effect, two main stages of development are of interest; 1) the gastrula stage at which ionizing ...

2007-09-01

A characteristic ventricular shape in myelomeningocele-associated hydrocephalus? A CT stereology study

Maternal exposure to ambient air pollution has increasingly been linked to adverse pregnancy outcomes. The evidence linking this exposure to congenital anomalies is still limited and controversial. This case-control study investigated the association between maternal exposure to ambient particulate matter with aerodynamic diameter less than 10 ?m (PM(10)), sulfur dioxide (SO(2)), nitrogen dioxide, nitric oxide (NO), ozone (O(3)), and carbon monoxide (CO) and the occurrence of congenital heart disease in the population of Northeast England (1993-2003). Each case and control was assigned weekly average (weeks 3-8 of pregnancy) of pollutant levels measured by the closest monitor to the mother's residential postcode. Using exposure as both continuous and categorical variables, logistic regression models were constructed to quantify the adjusted odds ratios of exposure to air pollutants and the occurrence of each outcome group. We found exposure to CO and NO to be associated with ...

2011-02-17

Radiation effects on shoot tip culture of chrysanthemum, 2

We measured the volume of the supratentorial ventricles in 39 consecutive children with myelomeningocele (MMC) and associated hydrocephalus, using a stereological method based on the Cavalieri theorem of systematic sampling. We distinguished the following groups: newborns before and after cerebrospinal fluid shunting (14), a somewhat larger group of newborns with an untreated MMC-associated hydrocephalus (25) and a group of shunted children at a mean age of 1.5 years (28). We paid special attention to the shape of the lateral ventricles, looking separately at the anterior and posterior halves. The measurements were compared with a healthy control group (10) and with children with hydrocephalus unrelated to MMC (15). The average volume ratio of the posterior to the anterior half of the lateral ventricles was 1.05 #+-# 0.39 in non-hydrocephalic children, 1.11 #+-# 0.55 in untreated hydrocephalic children without MMC, and 2.15 #+-# 0.65 in MMC-associated hydrocephalus prior to shunting. ...

Magnetic resonance imaging of cerebral anomalies in subjects with resistance to thyroid hormone

(1) Young chrysanthemums were planted after being irradiated at the time of shoot tip culture and the influence of irradiation was evaluated from the point of view of establishment of mutants. (2) Two cultivars, Kin-tenryu and Tenryu-no-asa were used in this experiment. The percentage of cultivars healthy enough to transplant on June 10, 1972 showed a decrease in proportion to the increase of dosage. Some plants of cultivar Kin-tenryu died after being transplanted. (3) Few plants of either cultivar could overwinter, and in particular no plant irradiated by gamma-rays of 20 kR. (4) Plant height showed a tendency of inhibition in both cultivars. (5) Three chlorophyll mutants showing chimera appeared. One of them was in the cultivar Tenryu-no-asa irradiated by 10 kR at 3 days after planting; two of them were in the cultivar Tenryu-no-asa irradiated by 10 kR at 5 days after planting. One mutant exhibiting malformation of entire leaves appeared in the cultivar ...

Inherited partial direct duplication of 11q: First report and possible association with a midline developmental field defect

Resistance to thyroid hormone (RTH) is an autosomal dominant disease caused by mutations in the human thyroid receptor beta gene on chromosome 3. Individuals with RTH have an increased incidence of attention deficit hyperactivity disorder (ADHD). The purpose of this study was to search for developmental brain malformations associated with RTH. Forty-three subjects (20 affected males [AM], 23 affected females [AF]) with resistance to thyroid hormone and 32 unaffected first degree relatives (18 unaffected males [UM], 14 unaffected females [UF]) underwent MRI brain scans with a volumetric acquisition that provided 90 contiguous 2 mm thick sagittal images. Films of six contiguous images beginning at a standard sagittal position lateral to the insula were analyzed by an investigator who was blind with respect to subject characteristics. The presence of extra or missing gyri in the parietal bank of the Sylvian fissure (multimodal association cortex) and multiple Heschl`s ...

1995-06-19

Impact of diisobutyl phthalate and other PPAR agonists on steroidogenesis and plasma insulin and leptin levels in fetal rats

A 36-year-old woman underwent amniocentesis for advanced maternal age. The fetal karyotype had an extra dark staining G band on the long arm of chromosome 11 with no other identifiable abnormalities. FISH studies using a chromosome 11 paint probe confirmed the origin of the extra band. The abnormality was identified as a partial duplication of 11q: 46,XX dir dup (11)(q13.5q21) or (q21q23.1). The specific duplicated band could not be identified with certainty. Detailed fetal sonograms were normal. Family studies revealed the identical duplication in the mother but normal karyotypes in both maternal grandparents. The mother had strabismus and a short tongue frenulum which required surgical correction. Menses occurred late in adolescence and complete development of secondary sexual characteristics was delayed until adulthood. An infertility evaluation revealed duplication of the uterus, cervix, and vagina. An evaluation for metorrhagia identified a pituitary adenoma which was resected. ...

1994-09-01

Disorders of brain development and phakomatosis

Endocrine disrupting chemicals can induce malformations and impairment of reproductive function in experimental animals and may have similar effects in humans. Recently, the environmental obesogen hypothesis was proposed, suggesting that environmental chemicals contribute to the development of obesity and insulin resistance. These effects could be related to chemical interaction with nuclear receptors such as the peroxisome proliferator activated receptors (PPARs). As several testosterone-reducing drugs are PPAR activators, we aimed to examine whether four PPAR agonists were able to affect fetal testosterone production and masculinization of rats. Additionally, we wished to examine whether these chemicals affected fetal plasma levels of insulin and leptin, which play important roles in the developmental programming of the metabolic system. Pregnant Wistar rats were exposed from gestation day (GD) 7-21 to diisobutyl phthalate (DiBP), butylparaben, ...

2008-09-04

Computerized tomography and head growth curve infantile macrocephaly with normal psychomotor development

Full text: Disorders of brain development and phakomatosis are resulting from disturbed embryonic-foetal development One third of all major embryological anomalies involve CNS, and over 2000 different anomalies have been described. Anomalies of the brain often cause foetal and neonatal death, and mental and physical retardation in pediatric group. The majority of disorders of brain development and phakomatosis are idiopathic, and most of them are not hereditary or familial. Ultrasonography plays the important role in screening foetal and neonatal brain, but after closure of fontanels it is difficult to find the acoustic window. CT has limited contrast resolution, and disadvantage exposing infant to ionizing radiation. It is helpful to demonstrate the presence of calcifications. MR imaging has proved to be a diagnostic tool of major importance in children with disorders of brain development and phakomatosis. The excellent grey/white matter differentiation and multiplanar imaging ...

Macrocephaly was defined as a head measuring larger than 98th percentile. We have evaluated CT findings and head growth curves in 25 infants with large heads. Ten (40%) of 25 infants with large heads were normal developmentally and neurologically. Five (20%) of those were mentally retarded. The other 10 infants (40%) included hydrocephalus (4 cases), malformation syndrome (3 cases), brain tumor (1 case), metabolic disorder (1 case) and degenerative disorder (1 case). Their head growth curves were typed as (I), (II) and (III): Type (I) (excessive head growth curve to 2 SDs above normal); Type (II) (head growth curve gradually approached to 2 SDs above normal); Type (III) (head growth curve parallel to 2 SDs above normal). Ten of macrocephaly with normal psychomotor development were studied clinically and radiologically in details. They were all male. CT pictures of those showed normal or various abnormal findings: ventricular dilatations, wide frontal and temporal ...

1982-01-01

Cardiac Malpositions

Transpositions and malpositions of the great arteries

Cardiac Malposition refers to any position of the heart other than a left-sided heart in a situs solitus individual. Associated cardiac malformations are so complex that even angiocardiographic and autopsy studies may not afford an accurate information. Although the terms and classifications used to describe the internal cardiac anatomy and their arterial connections in cardiac malpositions differ and tend to be confusing, common agreement exists on the need for a segmental approach to diagnosis. Authors present 18 cases of cardiac malpositions in which cardiac catheterization and angiocardiography were done at the Department of Radiology, Seoul National University Hospital between 1971 and 1979. Authors analyzed the clinical, radiographic, operative and autopsy findings with the emphasis on the angiocardiographic findings. The results are as follows: 1. Among 18 cases with cardiac malpositions, 6 cases had dextrocardia with situs inversus, 9 cases had dextrocardia ...

1979-06-01

Clinically silent heterotaxy with polysplenia syndrome and IVC azygous continuation draining to SVC: CT findings. Case report

The transposition of the great arteries afters to an abnormal connection and relation of the great arteries in which the aorta arises from the morphologic right ventricle and the pulmonary artery from the morphologic left ventricle, while the malposition of the great arteries indicates an abnormal relationship between the great arteries in which the great arteries are not transposed across the ventricular septum but they are malposed, as is seen in double outlet right ventricle, double outlet left ventricle and single ventricles. Transpositions and malpositions of the great arteries are understood as a spectrum of malformation caused by abnormal conal development and can be included in the common category of 'conotruncal malpositions'. Authors analized 32 cases of transpositions and malpositions of the great arteries in which cineangiocardiographies were done at the Department of Radiology, Seoul National University Hospital in recent 17 months, with specific ...

1980-12-01