Classification and diagnostic criteria of variants of Hirschsprung's disease.
Summary of "Classification and diagnostic criteria of variants of Hirschsprung's disease."
"Variants of Hirschsprung's disease" are conditions that clinically resemble Hirschsprung's disease (HD), despite the presence of ganglion cells in rectal suction biopsies. The diagnosis and management of these patients can be challenging. Specific histological, immunohistochemical and electron microscopic investigations are required to characterize this heterogeneous group of functional bowel disorders. Variants of HD include intestinal neuronal dysplasia, intestinal ganglioneuromatosis, isolated hypoganglionosis, immature ganglia, absence of the argyrophil plexus, internal anal sphincter achalasia and congenital smooth muscle cell disorders such as megacystis microcolon intestinal hypoperistalsis syndrome. This review article systematically classifies variants of HD based on current diagnostic criteria with an additional focus on pathogenesis, epidemiology, clinical presentation, management and outcome.
National Children's Research Centre, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland.
This article was published in the following journal.
Name: Pediatric surgery international
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/23943250
- DOI: http://dx.doi.org/10.1007/s00383-013-3351-3
The Assessment of Spondyloarthritis international Society (ASAS) classification criteria for axial spondyloarthritis (axSpA) have added nonradiographic axSpA (nr-axSpA) to the classic ankylosing spond...
To determine the proportion of idiopathic orbital inflammation (IOI) and orbital benign lymphoid hyperplasia (OBLH) accounted for by immunoglobulin (Ig)G4-related orbital disease (IgG4-ROD) using the ...
Hirschsprung disease (HSCR) is a congenital aganglionosis of myenteric and submucosal plexuses in variable length of the intestine. This study investigated the influence and a possible modifying funct...
Primary progressive aphasia (PPA) is a progressive language disorder associated with atrophy of the dominant language hemisphere, typically left. Current PPA criteria divide PPA into three variants: n...
Abstract The Diagnostic and Statistical Manual of Mental Disorders-Fifth Edition (DSM-5) diagnostic criteria for intellectual disability (ID) include a change to the definition of adaptive impairment....
Hirschsprung disease is a genetic condition caused by lack of nerve cells in varying lengths of the intestines. This study will investigate the complex genetic basis of the disease, which ...
Background: Existing criteria for AS/SpA such as mod. New York, ESSG, or Amor criteria for classification and/or diagnosis of spondyloarthritis have limitations when applied to early ...
Vasculitis is group of diseases where inflammation of blood vessels is the common feature. Patients typically present with fever, fatigue, weakness and muscle and joint aches. These sympt...
Obesity is a serious medical problem because it increases the risk of cardiovascular disease (CVD), type 2 diabetes mellitus, and certain forms of cancer. The definition of obesity is base...
Prospective, randomized, controlled trial to test if post-operative administration of probiotics in HD patients will lead to a reduction in the occurrence of HAEC.
Medical and Biotech [MESH] Definitions
Categorical classification of MENTAL DISORDERS based on criteria sets with defining features. It is produced by the American Psychiatric Association. (DSM-IV, page xxii)
Levels of severity of illness within a diagnostic group which are established by various measurement criteria.
Dilatation of the COLON, often to alarming dimensions. There are various types of megacolon including congenital megacolon in HIRSCHSPRUNG DISEASE, idiopathic megacolon in CONSTIPATION, and TOXIC MEGACOLON.
Criteria and standards used for the determination of the appropriateness of the inclusion of patients with specific conditions in proposed treatment plans and the criteria used for the inclusion of subjects in various clinical trials and other research protocols.
A noninvasive diagnostic technique that enables an experienced clinician to perform direct microscopic examination of the surface and architecture of pigmented SKIN lesions. The four major dermoscopic criteria are ABCD: asymmetry (A), borders (B), colors (C), and different structural components (D) providing a semiquantitative scoring system for each lesion.