Mohammed Hasosah

IgG4-related sclerosing mesenteritis in a 7-year-old Saudi Girl

Sclerosing mesenteritis (SM) is a rare, benign inflammatory disorder of unknown etiology, affecting the membranes of the digestive tract that involves lymphoplasmacytic inflammation, fat necrosis, and fibrosis of the mesentery. We report a child patient with a history of recurrent abdominal pain and fever who was found to have an intra-abdominal mass suspicious for malignancy. A tissue biopsy revealed the diagnosis of SM associated with IgG4-related systemic disease. The patient is currently maintained on 5 mg prednisone daily and no recurrence of symptoms was noted during the 24-month follow-up period. We emphasize, therefore, that SM can present clinical challenges and the presence of SM should cue clinicians to search for other coexisting autoimmune disorders that can have various outcomes.

Eosinophilic esophagitis in Saudi children: symptoms, histology and endoscopy results.

Background/Aim: Eosinophilic esophagitis (EE) is a clinicopathologic entity characterized by esophageal symptoms in association with a dense eosinophilic infiltrate currently defined as >15 eosinophils per high power field in the appropriate clinical context. This is the first pediatric study in Saudi Arabia to give the experience with EE and examine its symptom, histology and endoscopy results. Materials and Methods: Retrospective chart review of all patients diagnosed with EE at National Guard Hospital, Jeddah Between 2007 and 2009. The authors identified EE on histologic criteria (≥15 eosinophils per high-power field) together with their clinical context. The authors reviewed medical records for details of clinical presentation, laboratory data, radiologic, endoscopic, and histologic findings, and the results of treatment. Results: We identified 15 patients in our database in the last three years. 100% of the patients were males. The median age at presentation was 10 years (range, 3-17 years). The commonly reported symptoms were failure to thrive (86%), epigastric abdominal pain (53%), poor eating (40%), dysphagia with solid food (26%), food impaction (13%), and vomiting (20%). Asthma was reported in 46% and allergic rhinitis in 40%. Peripheral eosinophilia (>0.7 × 10/l) was found in 66%. High serum IgE Level (>60 IU/ml) was found in 60%. Upper endoscopic analysis revealed esophageal trachealization in 46%, esophageal erythema in 46%, white specks on the esophageal mucosa in 33%, esophageal narrowing in 13%, and normal endoscopy in 13%. The mean eosinophils per high-power field was 30.4 (range, 20–71). Histologic characteristics included degranulated eosinophils (86%), basal cell hyperplasia (93%) and eosinophils clusters (micro-abscess) in 73%. The treatment of EE revealed that they used swallowed corticosteroid in 50%, proton pump inhibitors in 66%, elemental diet/ food elimination in 13% and systemic corticosteroid in 13%. Conclusions: Failure to thrive and abdominal pain in a male, atopic school-aged child was the most common feature of EE. Peripheral eosinophilia, high serum IgE and endoscopic esophageal erythema and trachealization should significantly raise the clinical index of suspicion for the diagnosis of EE.

Prevalence and Risk Factors of Helicobacter pylori Infection in Saudi Children: A Three-Year Prospective Controlled Study

Helicobacter pylori (H. pylori) infection is the most common chronic infections. The risk factors for H. pylori infection in both developing and developed countries are closely related to poor living conditions in childhood. This study aimed to establish the prevalence of H. pylori infection and its associated risk factors among children in the western and central regions of Saudi Arabia.

Blue Rubber Bleb Nevus Syndrome: Extensive small bowel vascular lesions responsible for gastrointestinal bleeding

Blue Rubber Bleb Nevus Syndrome is a rare condition characterised by multiorgan venous malformations, in particular of cutaneous area and the gastrointestinal (GI) tract. We report here a child with chronic iron deficiency anaemia, melena and skin lesions. She had severe hypochromic microcytic anaemia. Upper and lower endoscopy revealed hundreds of red‐bluish polypoid lesions involving the entire GI tract primarily of the small bowel. Due to localisation of the most severe lesions, the patient responded well to surgical treatment, allowing a sustained clinical remission.

Perinatal cytomegalovirus hepatitis in Saudi infants: A case series

Background/Aim: Cytomegalovirus (CMV) is the most common congenital viral infection, occurring in 0.4%–2.3% of all live births. The clinical manifestations of CMV are multiorgan involvement. Currently, the numbers of studies of hepatic CMV infection in immunocompetent infants are insufficient and little information exists in the medical literature about the hepatic manifestations and complications of CMV. Patients and Methods: Nine infants diagnosed with hepatic CMV infection were included in the study. The diagnosis was based on the presence of IgM anti-CMV antibodies titer in serum and detection of CMV-DNA in blood. The authors identified clinical characteristics, biochemical characteristics, immunologic markers, and the outcome of hepatic CMV with or without treatment. Results: Jaundice was the most common clinical feature of CMV infection in infancy (100%). Hepatic abnormalities in the form of cholestasis (defined as a serum conjugated bilirubin concentration greater than 17.1 μmol/L or greater than 20% of the total serum bilirubin) were found in all patients (100%), hepatitis (77%), hypoalbuminemia (55%), elevated alkaline phosphatase, and gamma-glutamyltransferase (77%). Other findings showed hepatosplenomegaly (44%), thrombocytopenia (22%) and low birth weight (11%) The treatment of hepatic CMV infection was indicated in 66% and was not indicated in 33%. Both of them had resolved cholestasis and hepatitis. Conclusion: Jaundice and cholestasis were the most common clinical features of hepatic CMV infections. Hepatic CMV infection in young infants is often a self-limited illness that does not require antiviral therapy. Most of the patients with hepatic CMV infection had a favorable outcome.

Esophageal candidiasis in an immunocompetent girl

BackgroundInfections of the esophagus are rare and most commonly seen in children with immune suppression resulting from malignancy, chronic metabolic or infectious disease, or immunosuppressive drug therapy.MethodsAn 18-month-old girl on inhaled corticosteroid for bronchial asthma presented with coffee-ground emesis and melena. Upper endoscopy revealed yellow-white plaques scattered over the mucosa of the distal esophagus. Biopsy results showed chronic esophagitis with features of reflux disease. Gram staining of esophageal brushing showed pseudohyphae, and the culture was positive for candida species.ResultsThe patient was treated with omeprazole 2 mg/kg per day and fluconazole 6 mg/kg per day for three weeks. Immunological workup was normal and she was negative for human immunodeficiency virus. Post treatment endoscopy showed normal appearance of esophageal mucosa and normal histology. At 12 months after treatment, the child remained asymptomatic and continued to thrive.ConclusionsWe believe that this child developed esophageal candidiasis secondary to long-term use of inhaled corticosteroid associated with immune suppression on a background of reflux esophagitis. Anti-acid and antifungal therapies are effective in the treatment.

Seroconversion of hepatitis B envelope antigen (HBeAg) by entecavir in a child with chronic hepatitis B.

Hepatitis B virus (HBV) infection is a worldwide health problem. Consensus guidelines for the treatment of chronic HBV in children have not been established, and indications for antiviral therapy in adults with chronic HBV infection may not be applicable to children. The medications that are Food and Drug Administration approved for the treatment of children with HBV include interferon (IFN)-alpha and lamivudine. Nondetectable serum HBV deoxyribonucleic acid, Hepatitis B envelope antigen (HBeAg) loss, and HBeAg seroconversion following 1 year duration of entecavir treatment. A review of the literature of entecavir treatment of chronic hepatitis B in children is also provided.

Neuroblastoma amplified sequence gene mutation: A rare cause of recurrent liver failure in children

Neuroblastoma amplified sequence (NBAS) gene mutation or infantile liver failure syndrome type 2 (ILFS type 2) is an extremely rare disease characterized by episodic liver failure precipitated by intercurrent febrile illness, and liver function recovering completely. Here, we report a 4-year-old girl with recurrent hepatitis. A diagnosis of ILFS type 2 was made based on NBAS mutation gene found by whole-exome sequencing. Our case provides a new insight toward considering NBAS mutation as a part of the differential diagnoses of any infant presenting with recurrent liver failure or hepatitis. We recommend sequencing NBAS in cases of recurrent hepatitis in infancy of unknown cause, especially in individuals with fever-associated hepatic dysfunction.

A rare association of hepatitis A virus infection with type-1 diabetes

Patients with type-1 diabetes are predisposed to develop a spectrum of liver diseases, which includes fatty liver, steatohepatitis and cirrhosis. The association of hepatitis A infection with type-1 diabetes is extremely rare. To the best of our knowledge, our case is the first case report in children. We described a nine year-old girl who had type-1 diabetes and developed hepatitis A virus infection without complications. A review of the literature is also provided.

Visual Diagnosis: Chronic Diarrhea and Failure to Thrive in a 5-Year-Old Girl

Chronic GVHD usually develops 100 days after BMT and is a serious and potentially life-threatening long-term complication. Chronic GVHD can occur after previous or ongoing acute GVHD or in patients without a history of acute GVHD (eg, de novo disease) as illustrated by this case. In evaluating a patient presenting with chronic diarrhea more than 100 days after BMT, one must consider a serious underlying illness such as chronic GVHD because it is a major cause of morbidity and mortality of allogeneic stem cell transplantation. As indicated by this case, chronic GVHD can cause diarrhea. Complete evaluation of the diarrhea state should be part of the initial investigation. In many cases, however, the diagnosis of GVHD is not straightforward, and gastrointestinal biopsy and histologic confirmation is often necessary to corroborate a clinical impression of chronic GVHD.