Presentation
In the present case, longstanding constipation could have resulted in IND-like colon changes or IND could have been the cause of chronic constipation. The patient presented with megacolon and received surgical treatment. [ncbi.nlm.nih.gov]
The diffuse form of IND type B (16% patients) usually presents with chronic pseudoobstruction. In the remaining patients with rectocolonic dysplasia initial symptoms are constipation with pseudo-HSCR presentation. Symptoms usually start in infancy. [czytelniamedyczna.pl]
: A familial neuronal disease presenting as intestinal pseudo-obstruction. Gastroenterology 1978 ;75: 889 - 898. Google Scholar Medline ISI Maldonado JE, Gregg JA, Green PA, Brown AL : Chronic idiopathic intestinal pseudo-obstruction. [journals.sagepub.com]
The history of the histopathological diagnosis of Hirschsprung's disease and neuronal dysplasia (IND), up to the present state of knowledge Summary Background information: A review is given from the first histochemical publication about Hirschsprung's [thieme-connect.com]
Entire Body System
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Asymptomatic
The patient was discharged on postoperative day 10 and during a 2-year follow-up has remained asymptomatic with normal bowel motility. The WCS score is 8 points, showing a significant difference compared to 20 points preoperatively. [ncbi.nlm.nih.gov]
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Short Stature
There is also a report of two siblings with IND, mental retardation, dysmorphic features, hearing loss and short stature (13). Additional anomalies are more often observed in cases with diffuse IND (80%). [czytelniamedyczna.pl]
Gastrointestinal
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Abdominal Tenderness
Due to excess abdominal tenderness and radiological findings, it was decided that an exploratory laparotomy would be appropriate. [ncbi.nlm.nih.gov]
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Hypertrophic Pyloric Stenosis
In 30% of cases there may be IND-associated anomalies that include anorectal malformation, intestinal malrotation, megacystis, congenital short small bowel, hypertrophic pyloric stenosis, necrotizing enterocolitis, intestinal atresia, diffuse intestinal [czytelniamedyczna.pl]
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Loss of Appetite
She was admitted to hospital at 5 months because of a history of chronic constipation (she passed a hard stool with difficulty, every 4-5 days), loss of appetite, vomiting, and failure to thrive. She weighed 6700 g (10 th percentile). [czytelniamedyczna.pl]
Psychiatrical
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Suggestibility
Gálvez et al. [ 12 ] suggested that enteric nervous system dysplasia, including histological features of neuronal intestinal dysplasia, could be induced by chronic intestinal obstruction. [ncbi.nlm.nih.gov]
They also concluded that familial recurrence of associated IND-Malrotation-Short Bowel Syndrome suggests the possible presence of an autosomal recessive mode of inheritance. [czytelniamedyczna.pl]
Mitochondrial DNA analysis of an intestinal biopsy of patient 1 showed heteroplasmy consisting of a normal 16.5-kb band and an approximately 28-kb band, suggestive of a duplication. [journals.sagepub.com]
Onset
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Neonate-Onset
Neonatal onset of symptoms with ileus, meconium plug syndrome or neonatal constipation occurs in those with a coincidence of IND and aganglio-nosis (21). There is no significant difference in sex distribution. [czytelniamedyczna.pl]
Neurologic
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Confusion
This difficulty in exact estimation of the frequency of IND results from the considerable confusion regarding the essential diagnostic criteria. [czytelniamedyczna.pl]
Treatment
The patient presented with megacolon and received surgical treatment. [ncbi.nlm.nih.gov]
Similar results of the efficacy of conservative treatment in the majority of patients with IND were recorded by Ure etal. (23). [czytelniamedyczna.pl]
Consideration of the pathogenesis and treatment of primary chronic constipation in adults. Int J Colorectal Dis. 1990; 5 106-112 45 Stoss F, Meier-Ruge W. [thieme-connect.com]
Etiology
The role of genetic factors in etiology in that disorder is also suggested by two reports of IND in monozygotic twins (10,16). The procedure of choice in diagnosis of IND is a rectal biopsy. [czytelniamedyczna.pl]
Epidemiology
Epidemiology of congenital innervation defects of the distal colon. Virch Arch (A). 1992; 420 171-177 28 Meier-Ruge W, Schmidt P C, Stoss F. Intestinal neuronal dysplasia and its morphometric evidences. [thieme-connect.com]
Pathophysiology
The main diagnostic criteria, the underlining pathophysiology and the recommended therapeutic approach of this rare entity are extensively reviewed. [ncbi.nlm.nih.gov]
The main diagnostic criteria, the underlining pathophysiology and the recommended therapeutic approach of this rare entity are extensively reviewed. 2014 S. [karger.com]