Background Functional disturbances of the gastrointestinal tract are caused by a number of neurodysplastic conditions, including diseases that are rarer than Hirschsprung’s disease (HSCR), such as ganglion cell immaturity and intestinal neuronal dysplasia (IND). Bcl-2 shows positive immunoreactivity in degenerative and immature ganglion cells (IGCs). This work evaluates the implication of the immunohistochemical expression of Bcl-2 in cases of pediatric intestinal pseudo-obstruction (IPO) with ganglionated specimens. This 2-year prospective observational study was conducted from January 2017 to December 2019 on all intestinal specimens for cases referred from a pediatric surgery department with clinical data suggesting IPO (abdominal distension > 3 months, intermittent bilious vomiting, no radiological evidence of the transitional zone of HSCR or evidence of mechanical obstruction). The exclusion criteria were patients with IPO related to myopathic disorders, aganglionic biopsies, and specimens with inadequate tissue blocks. The same number of intestinal specimens of cases without IPO was used as a control group. All specimens underwent a histologic examination of ganglion cells and nerve bundles and were also evaluated for Bcl-2 immunohistochemical expression. Results Twenty-one specimens were analyzed, including six colonic resection specimens and 15 intestinal biopsies taken by full-thickness transanal biopsy (n = 12) or incisional biopsy from inadequately functioning stomas (n = 3). The mean ± standard deviation age of the patient cohort was 22 ± 7.4 months (range, 19–153 months), and there were 13 (61.9%) male patients. Bcl-2 protein was strongly positive in 57.1% of the cases, weakly positive in 19%, and negative in 23.8% as well as in all 21 control specimens. A highly significant correlation was observed between Bcl-2 expression and ganglion cell number (P < 0.001), where all hyperganglionic specimens showed strong positivity compared with the hypoganglionic and adequately ganglionic specimens. A positive association was also found between Bcl-2 expression and IND compared with other disorders of dysganglionosis (P = 0.04) and post-HSCR cases (P = 0.002). Conclusion Bcl-2 immunohistochemistry is a valuable tool to diagnose allied disorders of HSCR through its expression in IGCs, which are difficult to identify by conventional hematoxylin and eosin staining.

BackgroundChildhood interstitial and diffuse lung diseases (chILD) encompass a broad spectrum of rare pulmonary disorders. In most developing middle eastern countries, chILD is still underdiagnosed. Objective: To describe and investigate patients diagnosed with chILD in a tertiary university hospital in Egypt.MethodsWe analyzed data of consecutive subjects (<18 years) referred for further evaluation at the Children's Hospital, Ain Shams University. Diagnosis of chILD was made in accordance with the chILD-EU criteria. The following information was obtained: demographic data, clinical characteristics, chest CT findings, laboratory studies, spirometry, BAL and histopathology findings.Results22 subjects were enrolled over 24 months. Median age at diagnosis was 7 years (range 3.5–14 years). The most common manifestations were dyspnea (100%), cough (90.9%), clubbing (95.5%) and tachypnea (90.9%). Systematic evaluation led to the following diagnoses: hypersensitivity pneumonitis (n=3), idiopathic interstitial pneumonias (n=4), chILD related to chronic granulomatous disease (n=3), chILD related to small airway disease (n=3), postinfectious chILD (n=2), Langerhans cell histiocytosis (n=2), Idiopathic pulmonary hemosiderosis (n=2), granulomatous lymphocytic interstitial lung disease (n=1), systemic sclerosis (n=1), familial interstitial lung disease (n=1). Among the subjects who completed the diagnostic evaluation (n=19), treatment was changed in 13 (68.4%) subjects.ConclusionSystematic evaluation and multidisciplinary peer review of chILD patients at our tertiary hospital led to changes in management in 68% of the patients. This study also highlights the need for an Egyptian chILD network with genetic testing, as well as the value of collaborating with international groups in improving health care for children with chILD.

Background Functional disturbances of the gastrointestinal tract is caused by a number of neurodysplastic conditions including other diseases, rarer than the Hirschsprung’s disease dysganglionosis, such as ganglion cells immaturity including Intestinal Neuronal Dysplasia (IND). Bcl-2 shows positive immunoreactivity in the degenerative and immature ganglion cells . Aim This work evaluates the role of immunohistochemical expression of Bcl-2 in pediatric functional bowel obstruction cases with ganglionated specimens. Material and Methods Twenty one cases of functional bowel obstruction were enrolled in our study including the Hirschsprung Allied Disorders (HAD) and operated cases of Hirschsprung’s Disease (HD) with adequate ganglionated resection margins and presented by obstructive symptoms following surgery. All cases underwent histologic examination of ganglion cells and nerve bundles as well as evaluation for Bcl-2 immunohistochemical expression. Results 57.1% of the cases were strongly positive for Bcl-2 protein, 19% were weakly positive and 23.8 % were negative. A highly significant correlation was demonstrated between Bcl-2 expression and ganglion cell number ( P &lt; 0.001 ) where all hyperganglionic specimens showed strong positivity in contrast to the hypoganglionic and adequately ganglionic ones. A positive association was also found between Bcl-2 expression and IND in comparison to other disorders of dysganglionsis (P = 0.04) and recurrent HD cases (P = 0.002). A significant correlation was also detected between ganglion cell number and nerve bundle hypertrophy (P = 0.02). Conclusion Bcl-2 immunohistochemistry can be used as a valuable tool in the diagnosis of Hirschsprung’s disease allied disorders through its expression in the immature ganglion cells which are difficult to be identified by conventional H & E staining.

Background Non neoplastic GI lesions in pediatrics are variable and differ in types and prevalence among each pediatric age group. Helicobacter pylori is an important pathogen that can cause gastritis and peptic ulcers in adults as well as in children. Celiac disease is a gluten-dependent autoimmune disorder which affects individuals having genetic susceptibility. Eosinophilic gastrointestinal diseases are disorders that primarily affect the gastrointestinal tract with eosinophil-rich inflammation in the absence of known causes for eosinophilia. Inflammatory bowel disease (IBD) is a chronic inflammatory disorder, mainly affecting the gastrointestinal tract with extraintestinal manifestations and associated immune disorders. It seems that it is one of the most common gastrointestinal diseases affecting children in the developed countries. Aim of the work To study different types of paediatric non neoplastic gastrointestinal lesions from gastrointestinal endoscopic biopsies received at the Pathology Department in Ain Shams University hospital during a period of 2 years (2017-2018), and to correlate them with the clinicopathological presentations and endoscopic findings. Patients and Methods A cross sectional study was conducted on all pediatric gastrointestinal biopsies received at Pathology Department in Ain Shams University Hospital during the period of two years (2017- 2018). Only cases with information for all the covariates (n = 580) were selected and the results were statistically analyzed. Results Total 580 pediatric cases were enrolled according to inclusion criteria. Nonspecific gastrointestinal inflammation represented (47.1%), Helicobacter pylori associated gastrointestinal inflammation represented (43.5%), Eosinophilic gastrointestinal disease represented (3.8%), Inflammatory bowel disease (IBD) represented (3.7%), Celiac disease represented (1.9%). Conclusion This is the first study conducted in Ain Shams University Hospitals to assess the different types of pediatric non neoplastic gastrointestinal lesions received with clinicopathological and endoscopic correlation. The most common pediatric non neoplastic GI lesion is Helicobacter pylori infection. The diagnosis of pediatric non neoplastic GI disorder necessitates interdepartmental teamwork between GI pediatricians and pathologists.