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Wael El-Matary

Publications and source records attributed to Wael El-Matary.

12 recordsLinked to original sources
PubMed2006

Tufting enteropathy and skeletal dysplasia: is there a link?

INTRODUCTION: Tufting enteropathy (intestinal epithelial dysplasia), a rare congenital enteropathy, presents in the first few months of life with chronic watery diarrhoea and impaired growth. The molecular basis for this condition is not known. We report our experience with a case of tufting enteropathy that developed an unusual skeletal dysplasia with an abnormal blood picture. After extensive investigations including repeated gastrointestinal endoscopies and biopsies, the diagnosis of tufting enteropathy was made. During the third year of her life, the patient's height was static. A full skeletal survey was performed and demonstrated features of generalised skeletal dysplasia, some of them consistent with those of parastremmatic dwarfism. At the age of five years, she developed Coomb's positive haemolytic anaemia and thrombocytopenia with a negative auto-antibody screen including anti-enterocyte antibodies. CONCLUSION: There might be generalised matrix (including cartilage matrix protein), basement membrane abnormalities or both. A secondary protein leak might occur in the intestine with autosensitisation and development of autoimmune phenomena. More molecular research is needed to identify a possible link.

Bone Diseases, Developmental↗
PubMed2006

Successful conservative management of idiopathic fibrosing pancreatitis in children.

UNLABELLED: Idiopathic fibrosing pancreatitis, a rare cause of obstructive jaundice and abdominal pain in children, which has certain features in common with the emerging entity of autoimmune pancreatitis as described in adults, has frequently been managed surgically. We present our experience of successful conservative management of this condition in children. Three children (6-12 years; two girls, one boy) presented with a short history of abdominal pain followed by obstructive jaundice. Abdominal ultrasonographic examination in each case showed dilated intrahepatic and common bile ducts with a bulky pancreas, predominantly the head. These findings were confirmed by magnetic resonance imaging. In two cases, the diagnosis of fibrosing pancreatitis was made by exclusion after extensive investigation. The third case had a percutaneous ultrasound-guided pancreatic needle biopsy. Two patients were managed by supportive medical therapy alone, whilst the third, with symptomatic obstructive jaundice, underwent temporary endoscopic stenting of the common bile duct. Cases have been followed-up for 12-49 months. There was complete clinical and biochemical resolution of obstructive jaundice in all three cases. Plasma bilirubin concentrations decreased to normal within 3-8 weeks. Serial abdominal imaging showed a gradual resolution of biliary dilatation and abnormal pancreatic morphology with subsequent pancreatic atrophy. Two children developed steatorrhoea that responded to pancreatic enzyme supplements, and one patient developed diabetes mellitus. None of the cases needed invasive surgery for diagnosis or management. CONCLUSION: With careful radiological and biochemical assessment and monitoring, invasive surgery can be avoided in the management of fibrosing pancreatitis. The eventual outcome is no different from reported surgically treated cases.

Abdominal Pain↗
PubMed2006

Dilated cardiomyopathy and adipic aciduria in nutritional rickets.

Hypocalcemia secondary to nutritional rickets is a rare cause of dilated cardiomyopathy. It is also not a recognized cause of dicarboxylic aciduria. We report the first case of adipic aciduria, presenting with dilated cardiomyopathy, secondary to hypocalcemia.

Adipates↗
PubMed2005

Long-term outcome of nutritional therapy in paediatric Crohn's disease.

BACKGROUND AND AIMS: Long-term effects of using enteral feed therapy to induce remission in paediatric Crohn's disease are poorly documented. The aim of this study is to examine the short and long-term impact of enteral nutrition as primary therapy for children with newly diagnosed Crohn's disease. METHODS: Since 1994, a data base was set up in Bristol for all children with inflammatory bowel diseases. The data of newly diagnosed patients with Crohn's disease in whom enteral nutrition was used as the primary therapy (44 children) was analysed, with particular reference to time to remission, to first and subsequent relapse, and to first steroid usage. RESULTS: Forty out of 44 patients (90%) responded to enteral nutrition, with a median time to remission of 6 weeks. 25 of these 40 (62%) relapsed, with a median duration of remission of 54.5 weeks (range 4-312). 15 (38%) have not relapsed. 21 of the 44 (47%) have not received steroids. In those who eventually required steroids, their use was postponed for a median 68 weeks (range 6-190). Site of disease activity had no impact on response to enteral nutrition, but there was a trend towards earlier relapse in those with isolated colonic involvement. CONCLUSIONS: This data suggests that there are long-term benefits to the use of enteral nutrition to induce remission in children with Crohn's disease Steroids may be avoided in nearly half the cases and, in others, their use postponed by 68 weeks.

Adolescent↗
PubMed2005

Omeprazole-induced hepatitis.

Omeprazole; the first proton pump inhibitor (PPI) showing an effective acid inhibitory ability, provides the satisfactory therapy either in gastro-esophageal reflux symptom relief or in healing of erosive esophagitis. It's also effective in peptic ulcer disease. Up to date, omeprazole efficacy and safety are well established in many trials. Omeprazole-related hepatotoxicity is not very well recognized especially in pediatric population. We report a child who developed hepatitis following omeprazole intake. We believe that this is the first case report of omeprazole-induced hepatitis in pediatric population.

Adolescent↗
PubMed2004

Irritable bowel syndrome: the commonest cause of recurrent abdominal pain in children.

UNLABELLED: Apley, working in Bristol, UK, defined recurrent abdominal pain (RAP) in 1958. After extensive investigations, he found that 8% of children presenting to his clinic with RAP had an organic pathology. The aims of this study were to identify (1) causes of RAP using modern methodology, (2) factors associated with organic RAP and (3) children with non-organic RAP who fulfill the diagnostic criteria for irritable bowel syndrome (IBS). Children, aged over 3 years, presenting with RAP were prospectively recruited to this study. They had a detailed questionnaire completed, a full examination with screening tests (blood for coeliac screen, Helicobacter pylori antibody titre, inflammatory markers, serum amylase, liver function tests, and full blood count, urine and stool analyses and abdominal ultrasonography). Endoscopy and oesophageal pH monitoring were performed if clinically indicated. IBS was diagnosed if the child had no organic pathology and fulfilled the Rome II criteria. Out of 103 children (median age of 10 years, mean 10.04, SD +/-3.44), 31 children (30%) had organic pathologies. Factors associated with organic pain were nocturnal symptoms (P<0.01) and abdominal tenderness (P<0.005) and with non-organic pain were periumbilical locality (P<0.002), pain alleviation on defaecation (P<0.04) and low fibre diet (P<0.005). Of children with non-organic pain, 37/52 (51%) fulfilled the criteria for IBS (36% of the total). CONCLUSION: Of children presenting with recurrent abdominal pain in a hospital setting, 30% have a diagnosable organic aetiology compared to 8% in Apley's time. Irritable bowel syndrome, however, may be the commonest cause of recurrent abdominal pain and should be considered.

Abdominal Pain↗
PubMed2004

The effect of high-calorie diet on nutritional parameters of children with beta-thalassaemia major.

BACKGROUND: Impaired growth accompanying thalassaemia major poses diagnostic and therapeutic problems. AIM: To test the hypothesis that impaired growth of children with thalassaemia major might be corrected, partially or totally, by increasing their caloric intake. PATIENTS AND METHODS: In a prospective controlled study, thirty selected children with thalassaemia major and 30 normal age and sex-matched controls were recruited. The dietary intake of both groups was evaluated. Nutritional status was assessed by measuring the weight, body mass index (BMI), mid-arm circumference (MAC), triceps skin fold thickness (SFT) and serum albumin and insulin-like growth factor-I (IGF-I) concentrations. The thalassaemic group was then, randomly divided into two equal groups. One group was given 8 weeks of high-caloric diet (130-150% of the caloric recommendation for age and sex) and the other was given the normal caloric requirement. RESULTS: Initially the BMI, triceps SFT and MAC of children with thalassaemia were significantly decreased compared to those for the normal control group. IGF-I and albumin concentrations of thalassemic children before nutritional supplementation (69 +/- 20.5 ng/m and 3.65 +/- 0.67 g/dl, respectively) were significantly lower than those for normal age and sex-matched children (162.5 +/- 24 ng/ml and 4.29 +/- 0.66 g/dl, respectively). After nutritional supplementation for 8 weeks the MAC, SFT and BMI, IGF-I (88.4 +/- 27.3 ng/ml) and albumin concentrations (3.85 +/- 0.85 g/dl) increased significantly (P < 0.05) in the thalassaemic children given the supplementation compared to those without supplementation, however, they were still lower than normal children. CONCLUSIONS: Increased caloric dietary intake increased significantly IGF-I levels in thalassaemic children. This was accompanied with increased BMI, mid-arm circumference and skin fold thickness. Growth impairment of children with thalassaemia major, without endocrinopathy and/or cardiomyopathy, can be partially corrected by increasing caloric intake.

Anthropometry↗