Acquired factor VII deficiency in pediatric inflammatory bowel disease: Report of three cases.

Bleeding disorders can exacerbate gastrointestinal bleeding in inflammatory bowel disease (IBD) at the time of diagnosis or flares. Factor VII (FVII) deficiency is a life-threatening rare congenital bleeding disorder in childhood. This study describes three adolescent patients with IBD accompanied by acquired FVII deficiency. This is the first case series of patients with IBD accompanied by FVII deficiency. We hypothesized that inflammation, accelerated consumption, disease severity, and weight loss can cause decreased FVII activity in patients diagnosed with IBD. To control intestinal bleeding, we must keep in mind factor deficiencies in IBD.

Evaluation of children diagnosed with a lower respiratory tract infection due to Human metapneumovirus.

BACKGROUND: Human metapneumovirus (hMPV) is one of the leading causes of acute respiratory infections and bronchiolitis in infants. A history of prematurity and chronic diseases such as congenital heart disease or asthma/reactive airway disease (RAD) increases the risk of severe lower respiratory tract infection (LRTI) due to hMPV. In this cross-sectional study, we aimed to analyze the clinical outcome and risk factors for severe disease in children with LRTI due to hMPV. METHODS: The current cross-sectional study included children between 28 days and 18 years of age with the diagnosis of hMPV-associated LRTI hospitalizations, over two years from January 2016 to September 2018 in Health Science University Dr. Behçet Uz Child Disease and Pediatric Surgery Training and Research Hospital. hMPV virus was detected by the multiplex polymerase chain test (PCR) (Commercial Multiplex Real-Time PCR: FTD Respiratory 21 plus, Fast Track Diagnostics, Luxembourg) from a nasopharyngeal swab. Patients who had positive results in multiplex PCR tests with other viral agents simultaneously were not included in the study. Data were retrospectively collected from the computerized hospital system. RESULTS: In this cross-sectional study, 62 patients who were hospitalized with the diagnosis of LRTI due to hMPV infection were included. Thirty-five (55.7%) of the patients were male. The median age was one year (2 months-15 years). Fifty-one (82.2%) patients were younger than two years. The median hospital length of stay was found to be 10 days (2-33 days) in patients with an underlying disease and 7,5 days (ranging from 2 to 20 days) in the patients without an underlying disease, this difference was significant (p=0.031). CONCLUSIONS: Clinicians should consider hMPV as an important pathogen of LRTI even in healthy children, although we expect a poor course of disease in children with an underlying disease.

COmparisoN of TastES of Available NutriTional integrated or non-integrated formulas for infants older than 1 year of age with cow's milk allergy: A multicenter, prospective, single blind, cross-sectional observational clinical study (CONTEST Study).

Palatability of the infant formulas lacking cow milk protein formulas is reported by parents to be an important drawback. The purpose of this study is to examine decisions made by mothers of infants having cow milk protein allergy, and physicians concerning the palatability of unflavored extensively hydrolyzed formulas and amino acid-based formulas. We conducted a multi-center, randomized, single-blinded, observational taste study involving 149 pediatricians from gastroenterology and allergy subspecialties at 14 tertiary healthcare units from different regions of Turkey and involving 94 mothers of infants with cow milk protein allergy. Blinding was performed for seven formulas available in the market, which were the most commonly prescribed for feeding: four AAFs (Neocate-Numil®, Aptamil Pregomin AS-Numil®, Alfamino-Nestle®, Comidagen-Mamma®), one AAF specifically designed to address the growing nutritional and lifestyle needs of children >1 year (Neocate Junior-Numil®), 2 eHFs (Bebelac Pepti Junior-Numil®, Similac Alimentum-Abott®). Considering all three formula characteristics, Neocate junior-Numil® ranked as the number 1 product among seven products by mothers (63.8%) and physicians (69.8%). The ratings of mothers were significantly higher than the physicians (8.1 points and 6.1 points, respectively; p < 0.001). No difference was found in terms of taste, smell, and appearance for Neocate junior-Numil® between the mothers' and physicians' ratings. Since caregivers have responsibility for careful selection of replacement products for infants with cow milk protein allergy, it is noteworthy that increased awareness and confidence in the palatability characteristics of these products should motivate mothers and physicians to comply with replacement treatment in the long term.

Influenza-related hospitalizations due to acute lower respiratory tract infections in a tertiary care children's hospital in Turkey.

BACKGROUND: The influenza virus is a significant cause of acute lower respiratory tract infections (LRTI) requiring hospitalization in childhood and leads to severe morbidity and mortality, especially in certain risk groups. OBJECTIVES: The study aims to evaluate acute LRTI due to influenza in a tertiary care hospital and the risk factors for hospitalization among Turkish children. STUDY DESIGN: Children between 1 month and 18 years of age who were hospitalized at Dr. Behçet Uz Children's Hospital between January 2016 and March 2018 with lower respiratory tract infection that tested positive for influenza by PCR were included. Children with viral coinfections were excluded. Patient files were retrospectively scanned from the hospital computerized system in terms of age, underlying diseases, whether antiviral therapy was used, and length of hospital stay. Statistical analysis was performed using SPSS statistical software. RESULTS: The study included 131 patients with a median age of 2 years (1 month-15 years). Sixty-seven (51,1%) patients were younger than two years. Influenza A was isolated in 129 patients and B in 2 patients. Fifty-two patients (39,7%) had underlying medical conditions, and the most common one was malignancies (12/52, 23%). This was followed by neurodevelopmental diseases (9/52, 17,3%), prematurity (9/52 patients, 17,3%), primary immunodeficiency (8/52, 15,4%), asthma (7/52, 13,4%), Down syndrome (4/52, 7,7%), chronic renal disease (2/52, 3,8%) and congenital heart diseases (1/52, 1,9%). The mean length of stay (LOS) was 12,3 ± 9,5 days (2-60 days). The LOS was found to be statistically longer (15,2 ± 12,1 days, 3-60 days) in patients with an underlying disease compared to previously healthy patients (10,4 ± 6,7 days, 2-35 days) (p = 0.01). CONCLUSIONS: Hospitalization due to influenza-related acute LRTI is not an issue only for patients with an underlying medical condition. Vaccination should be considered not only for those with underlying medical conditions but also for healthy children.

Validity and reliability study of the pediatric Rome III questionnaire for Turkish children and adolescents.

BACKGROUND/AIMS: Questionnaire on Pediatric Gastrointestinal Symptoms: Rome III version (QPGS-RIII), originally developed in English, was adapted to different languages in order to widen its use. The aim of this study was to evaluate the validity and reliability of a questionnaire on the Pediatric QPGS-RIII parent-report form for children and self-report form for children and adolescents, which has been adapted into Turkish. MATERIALS AND METHODS: The study group comprised 7-18-year-old children/adolescents (n=690) who presented to Ege University School of Medicine, Department of Child Health and Diseases outpatient clinic. In the study, the validity and reliability of the QPGS-RIII Turkish version of the questionnaire was established. RESULTS: Confirmatory factor analysis (CFA) resulted in a 10-factor model satisfactory construct for the validity and in acceptable indices of goodness of fit. Standardized coefficients determined with CFA in the Turkish version of the instrument ranged between 0.15 and 0.87 in the 7-9-year-old children and between 0.13 and 0.98 in the 10-18-year-old children/adolescents. t-values of all the factor loadings were significant. In addition, the test-retest analyses were above 0.70, except for the abdominal migraine factor. CONCLUSION: Findings relating to the validity and reliability of the study indicated that the Turkish version of the instrument could be adequately used to assess functional gastrointestinal disorders (FGIDs) in Turkish children and adolescents. The Turkish version of the instrument is therefore recommended to be used in epidemiologic studies and in clinical trials to be conducted in a Turkish-speaking population.

Prevelance of hepatitis D co-enfection in children with hepatitis B infection: cross-sectional analyses from Western Turkey.

BACKGROUND/AIMS: Effective hepatitis B virus control has warranted a decline in hepatitis B virus prevalence over the world with a relevant reduction in hepatitis B virus-associated delta hepatitis. However, despite the dramatic decline in hepatitis D virus infection rate, no further decrease was recorded after 2000. This cross-sectional study aims to investigate: I- The prevalence of hepatitis D virus co-infection in children with hepatitis B virus infection in Western Turkey; II- The influence of neonatal hepatitis B virus vaccination on hepatitis D virus co-infection rate; and III- The impact of co-infection on prognosis of liver disease. MATERIALS AND METHODS: Serological markers of hepatitis B virus and hepatitis D virus infections were determined by ELISA in patients with chronic hepatitis during immune tolerance, immunoactive, HBeAg-negative chronic, and inactive carrier state. Delta co-infection rate was evaluated in two groups, children born before and after the national neonatal mass vaccination has started (before and after 2000). Viral load, serum alanine aminotransferase, and histological grade were evaluated in co-infected cases. RESULTS: Overall hepatitis delta virus infection rate was 1,76% (3/170); two patients with eAg-negative chronic hepatitis B and one patient in the immunoactive phase were infected with hepatitis D virus. Mean fibrosis score of hepatitis D virus -infected cases and hepatitis B virus -infected counterparts were 4±1,7 and 1,3±1, respectively (p: 0,006). Hepatitis D virus infection was detected in 2 out of 158 children born before and in 1 of 12 born after the neonatal vaccination program. Hepatitis B e-antibody was detected in two patients with delta co-infection (11 and 6 years old), and all mothers of delta hepatitis cases were chronically hepatitis B virus-infected. CONCLUSIONS: Delta hepatitis is rare among hepatitis B virus-infected children in the Western region of Turkey. Despite the success of the national vaccination program, delta hepatitis is not a vanishing disease and it has a grave prognosis due to development of early cirrhosis.

Hypocalcemic seizure in an adolescent with Down syndrome: a manifestation of unrecognized celiac disease.

Celiac disease (CD) affects up to 1% of the general population. Classically, it manifests with intestinal symptoms (diarrhea, steatorrhea, abdominal pain or discomfort) associated with weight loss and anemia. Seizure is a rare form of presentation of CD. A 13-year-old female patient with Down syndrome was admitted to the pediatric emergency department with generalized tonicclonic seizure in addition to numbness around the mouth, paresthesias, and muscular cramping for seven days. Investigations revealed severe hypocalcemia and vitamin D deficiency, which were a consequence of malabsorption secondary to histopathologically confirmed CD. Physicians should be aware that unrecognized CD can cause severe hypocalcemia.

Gastroesophageal reflux in children with functional constipation.

BACKGROUND/AIMS: Functional constipation and gastroesophageal reflux disease are two major and commonly encountered components of childhood functional gastrointestinal disorders. Epidemiological studies in the adult population support that there is a significant overlap between the different functional disorders of the digestive tract. Therefore, we aimed to investigate the frequency of gastroesophageal reflux disease in children with functional constipation and to compare clinical findings and 24-h esophageal pH monitoring with a group of patients with suspected gastroesophageal reflux disease. MATERIALS AND METHODS: Children between 4 and 16 years old with functional constipation (based on Rome III criteria, Group 1; n=38) were prospectively evaluated. A control group was composed of patients with symptoms suggesting gastroesophageal reflux disease (Group 2; n= 40). All patients included in the study were asked about reflux-related symptoms, and then all cases underwent 24-h esophageal pH monitoring analysis. RESULTS: Delayed gastric emptying symptoms such as belching and hiccups were more common in patients in Group 1 (p=0.002, p=0.021, respectively), whereas chronic cough was more common in patients in Group 2 (p=0.012). According to the 24-h esophageal pH monitoring, pathologic acid reflux in the lower and/or laryngopharyngeal portion of the esophagus was determined in 39.5% of the patients in Group 1 and in 42.5% of the patients in Group 2 (p=0.96). No significant difference was found in terms of age, gender and duration of constipation in patients with and without acid reflux in Group 1 patients. Pyrosis (66.6 vs. 0%, p=0.00001)was more common in Group 1 patients with acid reflux, but hiccups (20 vs. 69.5%, p=0.007) and belching (33.3 vs. 60.8%, p=0.184) were more common in patients in Group 1 without acid reflux. CONCLUSIONS: Gastroesophageal reflux disease should be considered in the treatment and monitoring of patients with functional constipation. Further studies are needed using 24-h pH multichannel impedance.

Peptic ulcer disease in children: an uncommon disorder with subtle symptomatology.

BACKGROUND/AIMS: Data concerning peptic and infectious ulcers in children are limited. The aim of the study was to investigate the prevalence, presenting symptoms and significance of symptomatology in ulcer diagnosis in the pediatric age group. MATERIALS AND METHODS: Between January 2000 and 2009, upper gastrointestinal endoscopy charts were examined retrospectively. All children in whom a diagnosis of ulcer was established were included in the study. Demographic, clinical, endoscopic, and histopathologic data were obtained from the patients' records. Peptic ulcer disease prevalence, presenting symptoms and symptomatology were evaluated. RESULTS: Ulcer disease was observed in 31 (3.4%) of 902 patients. The mean age was 10.85 ± 4.25 (range: 2-17 years), and the male to female ratio was 2:1. The most common symptom was chronic abdominal pain (68%), hematemesis and melena (55%) and vomiting (39%). Helicobacter pylori was identified in 19 patients (61%) with ulcer. In the Helicobacter pylori-positive group, upper intestinal bleeding and pain were the major symptoms. Symptom frequency was not different between Helicobacter pylori-positive and -negative patients (p>0.05). CONCLUSIONS: Ulcer disease is an uncommon disorder in children with nonspecific clinical symptoms. Unlike the adult population, symptoms fail to diagnose peptic ulcer disease before gastrointestinal bleeding occurs.

Steroid response in moderate to severe pediatric ulcerative colitis: a single center's experience.

BACKGROUND: we aimed to analyze clinical and inflammatory markers of steroid non-response in patients with moderate/severe ulcerative colitis (UC) at the time of diagnosis. METHODS: this study included patients who were graded as having moderate/severe UC and received corticosteroids as first-line therapy. Demographic, clinical and laboratory findings and pediatric ulcerative colitis activity scores (PUCAS) were recorded. Response to corticosteroids was assessed 30 days after the induction and long-term therapy. RESULTS: twenty-eight children were diagnosed as having moderate/severe UC. Their mean age ± SD was 1 2.2 ± 4 years, and 17% were under 5 years of age. PUCAS at their initial admission was 56.9 ± 11.8. UC was observed at the left colon in 9 patients (32.1%), and pancolitis in 19 (67.9%). At the end of the 30th day, UC was completely remitted in 15 patients (53.5%), partially remitted in 2 (7.1%), and no response in 11 (39.2%). Short-term follow-up showed partial remission in 2 patients, and overall remission with steroid in 17 (60.7%). Non-responders were given second-line treatment; steroid dependency was documented in 2 patients (7.1%) and another 2 (7.1%) patients underwent colectomy. Predictors for steroid non-response were analyzed and only PUCAS at the initial admission was found to be associated with non-response to steroids (51.4 ± 11.4 vs. 65.4 ± 6.8, P<0.05). CONCLUSIONS: approximately half of the pediatric patients had complete response to steroid therapy in a long period. PUCAS could be used as a potential marker of "failed response" to steroid, but should be supported with a number of prospective randomized controlled studies.