ABSTRACT
Background: Hirschsprung's Disease [HD] is a developmental disorder of enteric nervous system characterised by the absence of ganglion cells in submucosal [Meissner's] and myenteric [Aurbach's] plexuses of distal bowel. The purpose of the present study was to observe and report the morphological patterns of ganglion related enteric neuronal abnormalities in children presented with clinical features of [HD] in a Pakistani population. Methods: A total of 92 patients with clinical presentation of HD wereenrolled between March 2009 and October 2009. Among them, 8 were excluded according to the exclusion criteria. After detailed history and physical examination, paraffin embedded H and E stained sections were prepared from the serial open biopsies from colorectum. The data was analysed using SPSS-17. Frequencies and percentages are given for qualitative variables. Non-parametric Binomial Chi-Square test was applied to observe within group associations and p<0.05 was considered statistically significant. Results: Among 84 patients, 13 [15.5%] proved to be normally ganglionic whereas 71 [84.5%] showed ganglion related enteric neuronal abnormalities namely isolated hypoganglionosis 9 [12.7%], immaturity of ganglion cells 9 [12.7%], isolated hyperganglionosis [IND Type B] 2 [2.8%] and Hirschsprung's disease 51 [71.8%]. Among HD group, 34 [66.7%] belonged to isolated form and 17 [33.3%] showed combined ganglion related abnormalities. Conclusions: Hirschsprung's disease is common in Pakistani population, followed by hypoganglionosis, immaturity of ganglion cells and IND type B. The presence of hypertrophic nerve fibres was significant in HD, hyperganglionosis and hypoganglionosis, whereas, no hypertrophic nerve fibres were appreciated in immaturity of ganglion cell group. Keywords: Hirschsprung's disease [HD], hypoganglionosis, immaturity of ganglion cells [IGC], intestinal neuronal dysplasia [IND]
ABSTRACT
Enteric neuronal abnormalities include Hirschsprung's Disease [HD], hypoganglionosis, intestinal neuronal dysplasia [IND Type A and B], and immaturity of ganglion cells. All of these are mostly present with the same clinical features of the HD. A total of 92 patients presented with the clinical features of HD were recruited to this study from two tertiary care Children Hospitals from March 2009 to October 2009. They either had their first presentation or called for definitive surgeries with clinical presentation of HD. After applying exclusion criteria, 84 patients were finally left for the study. Among 84 patients, 13 [15.5% proved to be normally ganglionic on rectal biopsies and 71 [84.5%] showed enteric neuronal abnormalities. In these, 51 [71.8%] children had Hirschsprung's disease, 9 [12.7%] revealed immaturity of ganglion cells [IGC], 9 [12.7%] belonged to isolated hypoganglionic group and 2 [2.8%] showed isolated IND type B. In HD group, M: F ratio was 4.1:1, mean age at diagnosis was 1.9 years and the presenting complaints were in the descending order i.e., abdominal distention, constipation, vomiting and delayed passage of meconium. Among the group that showed IGC, M: F ratio was 2: 1, mean age at diagnosis was 12 days of life. The presenting complaints were same as that in HD in a similar order. In isolated hypoganglionic group, M: F ratio was 3.5.: 1, mean age at diagnosis was 2.3 years. The presenting complaints included constipation [88.9%], abdominal distension [77.8%], vomiting [33.3%] and delayed passage of meconium [33.3%]. In isolated IND type B, both patients were males, they came with constipation, abdominal distension and vomiting whereas one of them had history of delayed passage of meconium. HD was the most common enteric neuronal abnormality. Other neuronal abnormalities included isolated hypoganglionosis, immaturity of ganglion cells and isolated hyperganglionos [IND Type B]. Most common presenting complaints were abdominal distension, constipation, vomiting and delayed passage of meconium in all the groups
Subject(s)
Humans , Male , Female , Child , Intestinal Diseases , Nervous System DiseasesABSTRACT
The incidence of extranodal non-Hodgkin lymphoma [EN - NHL] is increasing particularly among the populations of South Asia and Middle East. On the whole 25 - 40% of NHL arise in the sites other than lymph nodes. This study was designed to assess the pathological patterns and the prevalence ofEN-NHL in Pakistan and in Saudi Arabia. A total 0/106 and 42 cases, from one centre in Pakistan and Saudi Arabia respectively, were included during a five year period [1998 - 2003] with a detailed account of their demographic and clinical characteristics. We observed that most of the Pakistani and Saudi patients presented in the 5[th] - 7[th] decades with the mean age being 43.2 and 46 years and a male to female ratio 0/1.07: i and 1.7: i respectively. Apart from a diverse pattern observed, diffuse large B cell lymphoma [57.5% and 63.4%] followed by MALT lymphoma [28.3% and 26.8%] were the commonest morphological subtypes of EN- NHL in both genders belonging to Pakistan and Saudi Arabia respectively. The tumours were found to be widespread, however, a predominant gastrointestinal tract [45% and 41.3%], in particular, gastric involvement was seen in [20.8% and 19.5%] Pakistani and Saudi patients respectively. However, predominant orbital involvement was seen exclusively in the latter. EN - NHL is an emerging malignancy in Pakistan and Saudi Arabia. Apart from a slight variation, no significant divergence was observed in the histological patterns of the EN -NHL in both geographical areas