Smooth muscle from aganglionic bowel in Hirschsprung's disease impairs neuronal development in vitro

1994 ◽  
Vol 276 (1) ◽  
pp. 181-186 ◽  
Author(s):  
Jacob C. Langer ◽  
Pierre-A. Betti ◽  
Michael G. Blennerhassett
Keyword(s):  
Smooth Muscle ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Neuronal Development ◽  
Development In Vitro

Hirschsprung's disease: A comparison of the nervous control of ganglionic and aganglionic smooth muscle in vitro

1987 ◽  
Vol 22 (5) ◽  
pp. 431-435 ◽  
Author(s):  
Lars Torsten Larsson ◽  
Gerhard Malmfors ◽  
Claes Wahlestedt ◽  
Stefan Leander ◽  
Rolf Håkanson
Keyword(s):  
Smooth Muscle ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Nervous Control

scholarly journals Recurrent candidaemia in a neonate with Hirschsprung's disease: fluconazole resistance and genetic relatedness of eight Candida tropicalis isolates

2006 ◽  
Vol 55 (4) ◽  
pp. 423-428 ◽  
Author(s):  
Pei Pei Chong ◽  
David Ching-Soo Chieng ◽  
Lee Yean Low ◽  
Asma Hafeez ◽  
Mariana Nor Shamsudin ◽  
...  
Keyword(s):  
Candida Tropicalis ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Genetic Relatedness ◽  
Infectious Agent ◽  
Venous Catheter ◽  
Drug Susceptibility ◽  
Fluconazole Resistant ◽  
Susceptibility Tests

The incidence of candidaemia among immunocompromised patients in Malaysia is increasing at an alarming rate. Isolation of clinical strains that are resistant to fluconazole has also risen markedly. We report here the repeated isolation of Candida tropicalis from the blood of a neonatal patient with Hirschsprung's disease. In vitro fluconazole susceptibility tests of the eight isolates obtained at different time points showed that seven of the isolates were resistant and one isolate was scored as susceptible dose-dependent. Random amplification of polymorphic DNA fingerprinting of the isolates using three primers and subsequent phylogenetic analysis revealed that these isolates were highly similar strains having minor genetic divergence, with a mean pairwise similarity coefficient of 0·893±0·041. The source of the infectious agent was thought to be the central venous catheter, as culture of its tip produced fluconazole-resistant C. tropicalis. This study demonstrates the utility of applying molecular epidemiology techniques to complement traditional mycological culture and drug susceptibility tests for accurate and appropriate management of recurrent candidaemia and highlights the need for newer antifungals that can combat the emergence of fluconazole-resistant C. tropicalis strains.

Smooth muscle proteins from Hirschsprung’s disease facilitates stem cell differentiation

2011 ◽  
Vol 28 (2) ◽  
pp. 135-142 ◽  
Author(s):  
Cornelia Irene Hagl ◽  
Sabine Heumüller ◽  
Markus Klotz ◽  
Ulrike Subotic ◽  
Lucas Wessel ◽  
...  
Keyword(s):  
Smooth Muscle ◽  
Stem Cell ◽  
Cell Differentiation ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Stem Cell Differentiation ◽  
Muscle Proteins

Impaired astrocytic extracellular matrix distribution under congenital hypothyroidism affects neuronal development in vitro

2010 ◽  
Vol 88 (15) ◽  
pp. 3350-3360 ◽  
Author(s):  
Cláudia Beatriz Nedel Mendes-de-Aguiar ◽  
Ricardo Alchini ◽  
Juliana Klein Zucco ◽  
Bruno Costa-Silva ◽  
Helena Decker ◽  
...  
Keyword(s):  
Extracellular Matrix ◽  
Congenital Hypothyroidism ◽  
Neuronal Development ◽  
Matrix Distribution ◽  
Development In Vitro

Nitric Oxide Synthase Is Deficient in the Aganglionic Colon of Patients With Hirschsprung's Disease

PEDIATRICS ◽  
1994 ◽  
Vol 93 (4) ◽  
pp. 647-651
Author(s):  
John F. Bealer ◽  
Eileen S. Natuzzi ◽  
Cori Buscher ◽  
Alan W. Flake ◽  
N. Scott Adzick ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Enzyme Activity ◽  
Smooth Muscle ◽  
Nitric Oxide Synthase ◽  
Nicotinamide Adenine Dinucleotide ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Nicotinamide Adenine Dinucleotide Phosphate ◽  
No Synthase ◽  
Oxide Synthase

Objectives. The cause of Hirschsprung's disease is unknown but defects in nonadrenergic, noncholinergic innervation could prevent relaxation of aganglionic colon in patients with this disease. Nonadrenergic, noncholinergic nerves induce relaxation by using nitric oxide synthase to produce the smooth muscle relaxant nitric oxide (NO). In this study we asked whether aganglionic colon in patients with Hirschsprung's disease is deficient in NO synthase-containing nerves. Methodology. Using the tetrazolium blue dye method of demonstrating nicotinamide adenine dinucleotide phosphate-diaphorase enzymes, we examined eight colon specimens (four aganglionic and four ganglionic) from patients with Hirschsprung's disease for the presence of NO synthase. We further quantified NO synthase enzyme activity in these eight specimens by using the [3H]arginine-to-[3H]citrulline conversion assay. Results. The nicotinamide adenine dinucleotide phosphate-diaphorase staining showed that aganglionic colon contained less NO synthase than ganglionic colon. This NO synthase deficiency was located primarily in the nerves of the circular muscle layer of the colon. In addition, there was a striking difference in the NO synthase enzyme activity between aganglionic and ganglionic colon as measured by the [3H]arginine-to-[3H]citrulline conversion assay. Total NO synthase activity, as measured by this assay, was found to be less in aganglionic than in ganglionic colon. When the total activity was divided into its four known isoforms, aganglionic colon was noted to be striking deficient in the isoform derived primarily from nerves. Conclusion. We conclude that aganglionic colon is deficient in NO synthase-containing nerves. This deficiency could prevent smooth muscle relaxation in the aganglionic colon of patients with Hirschsprung's disease.

Effect of nitric oxide on the colonic smooth muscle of patients with Hirschsprung's disease

1994 ◽  
Vol 29 (8) ◽  
pp. 1025-1029 ◽  
Author(s):  
John F. Bealer ◽  
Eileen S. Natuzzi ◽  
Alan W. Flake ◽  
N. Scott Adzick ◽  
Michael R. Harrison
Keyword(s):  
Nitric Oxide ◽  
Smooth Muscle ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease
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