Fecal calprotectin in children with familial Mediterranean fever in the attack-free period.

BACKGROUND: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent episodes of fever and serosal inflammation. The aim of this study was to evaluate fecal calprotectin (FC) in children with FMF during the non-attack period. METHODS: A retrospective evaluation was made of the data of a total 66 patients diagnosed with FMF in an attack-free period and without amyloidosis or inflammatory bowel disease (IBD). FC level in the FMF patients was compared with that in the patients with IBD and healthy control subjects. RESULTS: The FMF patients consisted of 37 boys (56.1%) with a mean age of 10.1 ± 3.9 years. Mean FC was 192.5 µg/g (range, 19.5-800 µg/g) in the FMF group, 597.9 µg/g (range, 180-800 µg/g) in the IBD group, and 43.8 µg/g (range, 19.5-144 µg/g) in the control group. The FC level in the children with FMF was higher than in the control group (P < 0.001), and the FC level of the IBD patients was higher than both the FMF and the control groups (P = 0.020, P < 0.001, respectively). CONCLUSIONS: FC was higher in FMF patients compared with healthy children even in the absence of IBD/amyloidosis. Even though colonoscopy is the gold standard in identifying intestinal inflammation in FMF patients, FC, a non-invasive and inexpensive method, can be used for screening. The presence of subclinical intestinal inflammation was also quantitatively identified in children with FMF.

Honeycomb gallbladder: a very rare cause of right upper quadrant pain.

Honeycomb gallbladder is a rare congenital malformation of the gallbladder. In some cases, it may be asymptomatic, whereas in others, it may lead to symptoms consistent with biliary colic even in the absence of cholelithiasis. We present the clinical and imaging findings of a case of a 10-year-old boy who was admitted to the emergency department with right upper quadrant pain. Ultrasonography and magnetic resonance imaging showed a honeycomb gallbladder. Honeycomb gallbladder is safely diagnosed with ultrasonography, which should be part of the investigation of patients with right upper quadrant pain in the emergency department.

Bilateral reexpansion pulmonary edema associated with pleural empyema: a case report.

Reexpansion pulmonary edema is an uncommon complication following rapid reexpansion of the lungs. The risk increases with a prolonged duration of pulmonary collapse, the amount of drained liquid or air, and with decreased time of draining. Treatment is supportive. In general, the prognosis is favorable. A nine-year-old boy was presented with fever, cough, and respiratory distress. Pneumonia and left-sided pleural empyema were determined and a chest tube was emplaced. Clinical deterioration occurred in just a few minutes following chest tube insertion. His chest radiography revealed a pulmonary edema in the left lung. Despite mechanical ventilation, antibiotics, and diuretic treatment, no significant improvement occurred. Acute respiratory distress syndrome and multiple organ dysfunctions developed in the follow-up. The patient died on day 5 of hospitalization. In this report, a complicated reexpansion pulmonary edema with a lathal outcome in a 9-year-old child is presented.

Fecal calprotectin levels used as a noninvasive method for screening for chronic gastritis in pediatric patients. A descriptive study.

BACKGROUND: Gastritis consists of inflammation of the gastric mucosa and is one of the main causes of dyspeptic symptoms in children. OBJECTIVE: To investigate the presence of inflammation by evaluating fecal calprotectin (FC) in children diagnosed with chronic gastritis. DESIGN AND SETTING: Descriptive study in Pediatric Gastroenterology Department of Ondokuz Mayis University Hospital in Turkey. METHODS: Between January 2016 and July 2018, FC levels were compared retrospectively in children with chronic gastritis (histopathology-based diagnosis), patients with inflammatory bowel disease (IBD) and healthy children. RESULTS: A total of 67 chronic gastritis patients (61.2% girls) with a mean age of 13.09 ± 3.5 years were evaluated. The mean FC levels were 153.4 µg/g in the chronic gastritis group, 589.7 µg/g in the IBD group and 43.8 µg/g in the healthy group. These levels were higher in chronic gastritis patients than in healthy individuals (P = 0.001) and higher in IBD patients than in the other two groups (P < 0.001). The FC level in the patients with chronic active gastritis (156.3 µg/g) was higher than in those with chronic inactive gastritis (150.95 µg/g) (P = 0.011). Among the patients with chronic active gastritis, the FC level was significantly higher in Helicobacter pylori-positive individuals than in negative individuals (P = 0.031). CONCLUSION: We confirmed the association between increased FC and chronic gastritis. Elevated FC levels may be seen in patients with chronic active gastritis. In order to be able to use FC as a screening tool for chronic gastritis, further studies in a larger study group are needed.

Endoscopic Ultrasonography in Pediatric Patients with Pancreatobiliary Disease: Single-Center Trial.

PURPOSE: The use of Endoscopic ultrasonography (EUS) in pediatric patients is not as common as in adults. The aim of this study is to evaluate the role of EUS in the diagnosis of pancreatobiliary disease in childhood. METHODS: Between December 2016 and January 2018, the findings of patients who underwent EUS were evaluated retrospectively. RESULTS: Of the 41 patients included in the study 25 were girls (61.0%), mean age was 12.2±4.2 years. EUS was performed for biliary colic in 21 (51.2%), for recurrent pancreatitis in 12 (29.2%), for cholecystitis/cholangitis in 5 (12.2%), and for acute pancreatitis in 3 (7.4%) patients. EUS had a significant clinical effect in the decision of treatment and follow-up of 6/21 biliary colic cases, in diagnosis and follow-up of 6/12 recurrent pancreatitis cases, in decision-making and monitoring of invasive procedures (ERCP/surgery) of 3/5 acute cholecystitis/cholangitis and 2/3 of acute pancreatitis cases as well as in follow-up of the other cases. The effectiveness of EUS in determining direct treatment and invasive intervention was 43.9%. None of the patients had complications related to the EUS procedure. CONCLUSION: Although current guidelines show that EUS can be used in pediatric patients, this is limited to a few published studies. In this study, it is shown that EUS is a safe method for the diagnosis, follow-up and treatment of common pancreatobiliary pathologies in childhood.

Pre- and Postgastrostomy Evaluation of Gastroesophageal Reflux in Children Using 24-Hour pH Monitoring.

Background: The aim of this study was to evaluate 24-hour pH monitoring results before and after gastrostomy in neurological impaired (NI) children who underwent gastrostomy or Nissen fundoplication (NF) concurrently with gastrostomy. Materials and Methods: Between March and December 2018, NI patients who had previously received pre- and postgastrostomy (Group 1) or gastrostomy + NF (Group 2) underwent pH monitoring pre- and postoperatively. Results: Twenty patients [12 males (60%) and the median age of 5.6 (14 months-14.7 years) years] with NI were followed up during the study period. When pre- and postgastrostomy weight and BMI z-scores were compared, the results were significantly higher in both groups after procedure (P = .043, P = .040, respectively). The clinical results of the patients after the operation showed improvement in both groups. The pH index of Group 1 (n = 11) was 2.32 (0-3.8) before gastrostomy and 3.18 (2.1-6.9) after gastrostomy (P = .061) and in Group 2 (n = 9) it was 5.85 (4.9-15.8) and 0.61 (0.3-1.3), before and after procedure (P < .001). There was a statistically significant difference in reflux index of each group. This difference was not statistically significant between the groups (P = .072). When the total bolus exposure index was compared, it was 4.01 (2.1-5.2) before gastrostomy and 5.67 (4.6-6.3) after gastrostomy in Group 1 (P = .041). In group 2, that index was found to be 9.8 (8.3-10.2) before and 1.28 (0.5-1.4) after (P < .001). There was a statistically significant difference in index after gastrostomy in Groups 1 and 2 (P = .023). Conclusions: Gastrostomy feeding is an effective and safe option to achieve enteral feeding in NI children. NF concurrent with gastrostomy should be decided according to the individual patient. Clinical trials protocol registration ID number was PRS NCT00445112.

Familial Mediterranean Fever Mutation Analysis in Pediatric Patients With Inflammatory Bowel Disease: A Multicenter Study.

BACKGROUND: The aim of the study was to evaluate familial Mediterranean fever (FMF) mutation analysis in pediatric patients with inflammatory bowel disease (IBD). The relation between MEFV mutations and chronic inflammatory diseases has been reported previously. METHODS: Children with IBD (334 ulcerative colitis (UC), 224 Crohn's disease (CD), 39 indeterminate colitis (IC)) were tested for FMF mutations in this multicenter study. The distribution of mutations according to disease type, histopathological findings, and disease activity indexes was determined. RESULTS: A total of 597 children (mean age: 10.8 ± 4.6 years, M/F: 1.05) with IBD were included in the study. In this study, 41.9% of the patients had FMF mutations. E148Q was the most common mutation in UC and CD, and M694V in IC (30.5%, 34.5%, 47.1%, respectively). There was a significant difference in terms of endoscopic and histopathological findings according to mutation types (homozygous/ heterozygous) in patients with UC (P < .05). There was a statistically significant difference between colonoscopy findings in patients with or without mutations (P = .031, P = .045, respectively). The patients with UC who had mutations had lower Pediatric Ulcerative Colitis Activity Index (PUCAI) scores than the patients without mutations (P = .007). CONCLUSION: Although FMF mutations are unrelated to CD patients, but observed in UC patients with low PUCAI scores, it was established that mutations do not have a high impact on inflammatory response and clinical outcome of the disease.

Acute pancreatitis as a presenting feature of Henoch-Schönlein purpura.

Henoch-Schönlein purpura (HSP) is the most common small vessel vasculitis of childhood. It is characterized by nonthrombocytopenic palpable purpura, abdominal pain, arthritis, and glomerulonephritis. Although HSP is typically known to be self-limited, serious complications can occur. Acute pancreatitis rarely presents as a complication of HSP. It is even rarer as an initial presenting feature of HSP, before the occurrence of characteristic palpable purpura. Herein, we report a 12-year-old girl with HSP who atypically presented with acute pancreatitis.

Association of obesity and non-alcoholic fatty liver disease with the fecal calprotectin level in children.

BACKGROUND AND STUDY AIMS: As the prevalence of obesity increased, obesity-related comorbidities such as non-alcoholic fatty liver disease (NAFLD) also increased. The aim of this study is to investigate the presence of intestinal inflammation by evaluating the faecal calprotectin (FC) level in children with obesity and NAFLD and to determine the factors affecting the FC level. PATIENTS AND METHODS: Between August 2018 and November 2018, the FC levels of obese patients (Group 1a = NAFLD (n = 30) and 1b = without NAFLD (n = 30)) were prospectively compared to that of healthy children (Group 2, n = 20). Patients with BMI > 2 z-score were considered obese. NAFLD was identified with liver contrast and brightness on ultrasound. RESULTS: Of the patients included in this study, 50 were male (62.5%), with a mean age of 11.4 ± 3.1 years. The mean FC levels were 121.6 ± 24.8 µg/g (19.5-800) in Group 1 (Group 1a = 128.4 and Group 1b = 84.5) and 43.8 ± 25.4 µg/g (19.5-144) in Group 2. In comparison, the FC levels were higher in Group 1. This difference was more significant when compared with Group 1a than with Group 2 (p = 0.018 and p = 0.007, respectively). When the FC levels of Group 1 (above 50) were compared to lower levels, the weight, BMI, waist circumference and waist circumference/height values were significantly higher (p = 0.006, p = 0.028, p = 0.035 and p = 0.026, respectively). CONCLUSION: The FC level increased as a sign of intestinal inflammation in obese and NAFLD patients. This is directly proportional to the weight, waist circumference and waist-to-height ratio. It is thought that FC, which is easily applicable and an inexpensive biomarker, can be used safely in demonstrating the presence of intestinal inflammation in obese children.

Plummer-Vinson syndrome in a 15-year-old boy.

Plummer-Vinson syndrome presents as a classical triad of dysphagia, iron deficiency anemia and upper esophageal web(s). The syndrome usually occurs in adults, and is rare in childhood. We report a case of this syndrome occurring in a 15-year-old boy. He presented with dysphagia and anemia. Radiological examination showed the presence of webs at the cervical esophagus. The boy was treated with endoscopic balloon dilation and iron supplementation and remains in good general condition six months after the treatment.

Tuberculous peritonitis in children: report of nine patients and review of the literature.

AIM: To present our experience with tuberculous peritonitis treated in our hospital from 2002-2007. METHODS: We reviewed the medical records of 9 children with tuberculous peritonitis. RESULTS: Nine patients (5 boys, 4 girls) of mean age 14.2 years were diagnosed with peritoneal tuberculosis. All patients presented with abdominal distention. Abdominal pain was seen in 55.5% and fever in 44.4% of the patients. Four cases had coexisting pleural effusion and two had pulmonary tuberculosis with parenchymal consolidation. Ultrasonography found ascites with septation in 7 patients. Two patients had only ascites without septation. Ascitic fluid analysis of 8 patients yielded serum-ascite albumin gradients of less than 1.1 gr/dL. Laparoscopy and laparotomy showed that whitish tuberculi were the most common appearance. Adhesions were also seen in three cases. The diagnosis of peritoneal tuberculosis was confirmed histo-pathologically in 7 patients and microbiologically in two. Two patients had been diagnosed by ascitic fluid diagnostic features and a positive response to antituberculous treatment. All patients completed the antituberculous therapy without any complications. CONCLUSION: Tuberculous peritonitis has to be clinically suspected in all patients with slowly progressive abdominal distension, particularly when it is accompanied by fever and pain. Laparoscopy and peritoneal biopsy are still the most reliable, quick and safe methods for the diagnosis of tuberculous peritonitis.

The presence and distribution of dental enamel defects and caries in children with celiac disease.

The aim of this study was to investigate the presence and distribution of developmental enamel defects and caries in children with celiac disease (CD) and compare the results obtained with those of a control group of children without CD. A total of 64 subjects (mean age 8.2 years) selected from patients diagnosed and treated for CD were studied. Sixty-four age/ sex-matched healthy children were enrolled as a control group. Permanent dentition enamel defects were recorded according to Aine's classification. The caries experience of the children was recorded according to the criteria of the World Health Organization (WHO). The prevalence of enamel defect in CD subjects was found to be significantly higher (42.2%) than in healthy subjects (9.4%) (p < 0.001). Grade I type enamel defects were most commonly diagnosed in both groups (20.3% and 6.3%, respectively). The number of caries-free subjects in the control group was higher (38%) than in the CD group (17%). This study clearly showed that children with CD were at an increased risk of dental enamel defects compared with healthy subjects. Enamel defects were associated with an increased caries incidence.

Comparison of two different regimens of combined interferon-alpha2a and lamivudine therapy in children with chronic hepatitis B infection.

AIM: To evaluate the efficacy of two regimens of combined interferon-alpha2a (IFN-alpha2a) and lamivudine (3TC) therapy in childhood chronic hepatitis B. METHODS: A total of 177 patients received IFN-alpha2a, 9 million units (MU)/m2 for 6 months. In group I (112 patients, 8.7 +/- 3.5 years), 3TC (4 mg/kg/day, max 100 mg) was started simultaneously with IFN-alpha2a, in group II (65 patients, 9.6 +/- 3.8 years) 3TC was started 2 months prior to IFN-alpha2a. 3TC was continued for 6 months after antiHBe seroconversion or stopped at 24 months in nonresponders. RESULTS: Baseline alanine aminotransferase (ALT) was 134.2 +/- 34.1 and 147.0 +/- 45.3; histological activity index (HAI) was 7.4 +/- 2.7 and 7.1 +/- 2.3; and HBV DNA levels were above 2,000 pg/ml in 76% and 66% of patients in groups I and II, respectively (P > 0.005). Complete response was 55.3% and 27.6% in groups I and II, respectively (P < 0.01). AntiHBe seroconversion was higher and earlier, and HBV DNA clearance was earlier in group I (P < 0.05). HBsAg clearance was 12.5% and 4.6% and antiHBs seroconversion was 9.8% and 6.2% in groups I and II, respectively (P > 0.05). Breakthrough occurred in 17.9% and 24.6%; breakthrough times were 15.9 +/- 4.6 and 14.1 +/- 5.1 months; and relapse rates were 6.8% and none in groups I and II, respectively (P > 0.05, P > 0.05, P > 0.05). Responders had higher HAI (HAI > 6) and higher pre-treatment ALT than non-responders. CONCLUSION: Simultaneous 3TC+IFN-alpha2a yields a higher response and earlier antiHBe seroconversion and viral clearance than consecutive combined therapy. Relapse rate is low. Predictors of response are high basal ALT and high HAI scores. 3TC can be administered for 24 months without any side effect and breakthrough rate is comparable with previous studies.

Lactose and Fructose Intolerance in Turkish Children with Chronic Abdominal Pain.

OBJECTIVE: To investigate the prevalence of lactose and fructose intolerance in children with chronic abdominal pain. METHODS: Hydrogen breath tests were done to detect lactose and fructose malabsorption in 86 children with chronic abdominal pain (44 irritable bowel syndrome, 24 functional abdominal pain and 17 functional abdominal pain syndrome as per Rome III criteria) presenting to a Pediatric Gastroentreology department. RESULTS: 14 (16.3%) of patients were diagnosed with lactose intolerance and 11 (12.8%) with fructose intolerance. CONCLUSION: Lactose and fructose intolerance in children can lead to chronic abdominal pain and symptoms improve with dietary modifications.

Detection of gastroesophageal reflux in children with cerebral palsy using combined multichannel intraluminal impedance-ph procedure.

Gastroesophageal reflux (GER) is a very common condition in children with neurological impairment and this can influence nutritional and respiratory outcomes. The aim of this study was to investigate the presence of GER in children with cerebral palsy (CP) using multiple intraluminal impedance (MII)-pH monitoring. The use of combined MII-pH allows for the detection of both acid and non-acid reflux episodes. A total of 29 CP patients with symptoms suggesting GER, aged 2 to 10 years old, underwent 24-hour combined MII-pH monitoring. There were a total of 3899 reflux episodes, of which 29% were acid, 60% were weakly acid and 11% were alkaline. The number of non-acid reflux episodes was statistically significantly greater (p < 0.01). These findings confirm that GER disease is seen frequently in children with cerebral palsy and most of the reflux episodes are not acidic. Non-acid reflux can also influence the morbidity in patients with cerebral palsy. It can be concluded that 70% of the reflux episodes would not have been recognized by pH measurement alone.

Bone mineral density and growth in children with coeliac disease on a gluten free-diet.

BACKGROUND/AIM: To evaluate changes in growth and bone metabolism during consumption of a gluten-free diet (GFD) in children with coeliac disease (CD). MATERIALS AND METHODS: Thirty-seven children with CD (mean age of 8.8 ± 4.6 years, 21 girls) were enrolled. Anthropometric measurements, bone mineral density (BMD) in lumbar 2-4 vertebrae, and serum alkaline phosphatase, calcium, and phosphorus levels at diagnosis and at follow-up were recorded. RESULTS: The mean follow-up period was 3.5 ± 2.3 years. The BMD of patients was significantly lower than that of control subjects at the time of diagnosis but not after 1 year of the GFD. Incidence of low BMD with respect to z-scores for chronological age (CA) was significantly higher than z-scores for height age (HA) (P = 0.006). At the first year of GFD, BMD, BMD z-score, height-for-age z-scores, and weight-for-age z-scores were significantly increased compared with the baseline, but not after 1 year of the GFD. CONCLUSION: In CD, the first year of GFD is important in weight gain, linear growth, and improvement of BMD. A considerable relation of low BMD in children with CD, with respect to z-scores for CA, may be a result of misinterpretation of low BMD due to short stature.

Different techniques for biliary diversion in progressive familial intrahepatic cholestasis.

BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is a cholestatic liver disease of childhood. Pruritus resulting from increased bile salts in serum might not respond to medical treatment, and internal or external biliary drainage methods have been described. In this study, we aimed to evaluate different internal drainage techniques in patients with PFIC. PATIENTS AND METHODS: Between 2009 and 2014, seven children (4 male, 3 female, 3months-5years old), (median 2years of age) with PFIC were evaluated. The patients were reviewed according to age, gender, complaints, surgical technique, laboratory findings and outcome. In each two patients, cholecystoileocolonic anastomosis, cholecystojejunocolonic anastomosis and cholecystocolostomy were performed. Cholecysto-appendico-colonic anastomosis was the technique used in one patient. RESULTS: Jaundice and excessive pruritus were the main complaints. One of the patients with cholecystoileocolonic anastomosis died of comorbid pathologies (cirrhosis, adhesive obstruction and severe sepsis). Temporary rectal bleeding was observed in all the patients postoperatively. Regardless of the surgical technique, pruritus was dramatically decreased in all the patients in the postoperative period. CONCLUSION: Regardless of the technique, internal biliary diversion methods are beneficial for the relief of pruritus in PFIC patients. Selection of the surgical method might vary depending on the surgeon's preference and the surgical anatomy of the gastrointestinal system of the patient.

Inflammatory bowel disease in Turkish children.

BACKGROUND: This study was undertaken to evaluate demographics, clinical manifestations, laboratory findings and outcomes of children with inflammatory bowel disease (IBD) in Turkey. METHODS: We analyzed the medical records of 127 children diagnosed with IBD (under 18 years old) between January 2004 and January 2012 in 8 pediatric gastroenterology centers. RESULTS: Of the 127 patients, 90 (70.9%) suffered from ulcerative colitis (UC), 29 (22.8%) from Crohn's disease (CD), and 8 (6.3%) from IBD unclassified. The mean age of the 127 patients was 11.6 ± 4.1 years, and 11.8% of the patients were below 5 years old. Of the patients, 49.6% were male, and males were more predominant in patients with CD than in those with UC (72.4% vs. 42.2%, P = 0.008; a male/female ratio of 2.62 in CD, P = 0.0016). Approximately one fifth of the patients had extra-intestinal manifestations and 13.3% of the patients had associated diseases. Extraintestinal manifestations and associated diseases were more common in early onset disease [P = 0.017, odds ratio (OR) = 4.02; P = 0.03, OR = 4.1]. Of the patients, 15% had normal laboratory parameters including anemia, high platelet count, hypoalbuminemia, hypoferritinemia, and high sedimentation rate. Area under receiver operation characteristics was used to predict pancolitis in patients with UC. The values of C-reactive protein, sedimentation rate and pediatric ulcerative colitis activity were 0.61 (P = 0.06), 0.66 (P = 0.01) and 0.76 (P = 0.0001), respectively. Four (4.4%) patients with UC underwent colectomy, and finally two (1.5%, 95% confidence interval: 0-3.7%) patients died from primary disease or complications. CONCLUSIONS: IBD is an increasing clinical entity in Turkey. Features of IBD are similar to those in other populations, but prospective multicenter studies are needed to analyze the true incidence of IBD in Turkish children.