Growth, tolerance and safety outcomes with use of an extensively hydrolyzed casein-based formula in infants with cow's milk protein allergy.

Objective: To evaluate growth, tolerance and safety outcomes with use of an extensively hydrolyzed casein-based formula (eHCF) in infants with cow's milk protein allergy (CMPA). Methods: A total of 226 infants (mean ± SD age: 106.5 ± 39.5 days, 52.7% were girls) with CMPA who received eHCF comprising at least half of the daily dietary intake were included. Data on anthropometrics [weight for age (WFA), length for age (LFA) and weight for length (WFL) z-scores] were recorded at baseline (visit 1), while data on infant feeding and stool records, anthropometrics and Infant Feeding and Stool Patterns and Formula Satisfaction Questionnaires were recorded at visit 2 (on Days 15 ± 5) and visit 3 (on Days 30 ± 5). Results: From baseline to visit 2 and visit 3, WFA z-scores (from -0.60 ± 1.13 to -0.54 ± 1.09 at visit 2, and to -0.44 ± 1.05 at visit 3, p < 0.001) and WFL z-scores (from -0.80 ± 1.30 to -0.71 ± 1.22 at visit 2, and to -0.64 ± 1.13 at visit 3, p = 0.002) were significantly increased. At least half of infants never experienced irritability or feeding refusal (55.7%) and spit-up after feeding (50.2%). The majority of mothers were satisfied with the study formula (93.2%), and wished to continue using it (92.2%). Conclusions: In conclusion, eHCF was well-accepted and tolerated by an intended use population of infants ≤ 6 months of age with CMPA and enabled adequate volume consumption and improved growth indices within 30 days of utilization alongside a favorable gastrointestinal tolerance and a high level of parental satisfaction.

Advancing dentistry: fractal assessment of bone health in pediatric patients with celiac disease using dental images.

OBJECTIVES: To assess the effects of a gluten-free diet on bone structure in children with celiac disease using fractal analysis on panoramic radiographs. METHOD AND MATERIALS: A total of 49 patients with celiac disease aged 6 to 13 years, separated into two groups as previously and newly diagnosed, and a control group of 32 healthy individuals were evaluated. In previously and newly diagnosed patients with celiac disease, body mass index Z-scores were calculated, calcium, alkaline phosphatase, vitamin D3, and parathormone levels were measured, and bone mineral density Z-scores were obtained from dual energy x-ray absorptiometry. In all patients, the fractal dimensions of the right and left temporomandibular condyles were evaluated with the fractal analysis method on panoramic radiographs. RESULTS: The mean values of serum biomarker levels and the body mass index and bone mineral density Z-scores for both celiac groups were within the normal reference range. No statistically significant difference was determined between right and left condyle fractal dimensions values in the three groups examined. In terms of both right and left condyle fractal dimensions values, there was a statistically significant difference between groups. The highest fractal dimensions values were determined in the previously diagnosed group. CONCLUSIONS: Differences in fractal dimensions values were observed among patients with celiac disease following the gluten-free diet. Utilizing fractal analysis on panoramic radiographs can prove valuable for dental practitioners in evaluating bone mineral density due to its cost-effectiveness, easy accessibility, and reduced radiation exposure for patients, enabling them to provide comprehensive oral health care and potential early interventions for patients with celiac disease.

Long-Term Follow-Up and Outcome of Pediatric Acute Pancreatitis: A Multicenter Study.

OBJECTIVE: Over the past decades, the incidence of acute pancreatitis is increasing, but the progression of acute recurrent pancreatitis and chronic pancreatitis is still not well documented in children. The aim of this multicenter study is to delineate the changes that occur in a certain time period in the course of childhood pancreatitis. MATERIALS AND METHODS: The data of consecutive patients hospitalized with acute pancreatitis between 2010 and 2017 in 4 different pediatric gastroenterology units were reviewed. The clini- cal characteristics of the disease were defined. RESULTS: A total of 165 patients (55.2% female) were included. Over the years, the rate of acute pancreatitis admissions increased while the duration of hospitalization decreased (P < .05). Nearly two-thirds of the patients with acute pancreatitis resolved spontaneously, 30.9% and 4.3% of the cases developed acute recurrent pancreatitis and chronic pancreatitis, respectively. Furthermore, 27.4% patients with acute recurrent pancreatitis progressed to chronic pancre- atitis, and eventually, 12.7% of cases developed chronic pancreatitis within 3-4 years. Local complications developed in 13.3% of the patients with pancreatitis in this cohort. CONCLUSION: The result of this study confirmed the increased incidence of acute pancreatitis in recent years. Conversely, the length of hospital stay decreased over the years. Patients with pancreaticobiliary abnormalities or genetic risk factors had a higher rate of progression to acute recurrent pancreatitis or chronic pancreatitis. Therefore, genetic testing and radiological imaging should be considered early in the follow-up of patients with acute pancreatitis having risk factors for progression to acute recurrent pancreatitis/chronic pancreatitis.

Dental Caries, Dental Erosion and Periodontal Disease in Children with Inflammatory Bowel Disease.

Background: There is reportedly a higher prevalence of dental caries and periodontal disease in adults with inflammatory bowel disease (IBD) than in healthy adults. Similar data for children are lacking in the literature. We aimed to evaluate the prevalence of dental erosion, dental caries, and periodontal disease in children with IBD. Methods: This was a cross-sectional comparative study. Using the established criteria of the World Health Organization, oral investigations and detailed questionnaires that covered nutritional habits were completed by the same pediatric dentist for 32 patients with IBD, aged 11 to 18 years (15.53 ± 2.00), and 32 healthy controls. Results: The decayed, missing, and filled tooth index showed no significant difference between the groups (p = 0.072). The frequency of consumption of salad, lemon gum, candy and sweetened milk was significantly higher in the control group (p = 0.041, 0.012, 0.001, and 0.001, respectively) than in the IBD group. No dental erosion was observed in the IBD group. Oral mucosal history determined that 20/32 patients with IBD (62.5%) had at least one oral extraintestinal manifestation. Despite no significant differences in plaque scores between the two groups, the gingival evaluation showed a much higher mean value of gingival index scores in the IBD group than in the control group (p = 0.003). Conclusion: Although the number of patients included in the study is small, we can conclude that oral extraintestinal manifestations and periodontal disease are more prevalent in paediatric patients with IBD than in healthy populations.

Genotype-phenotype relationships of truncating mutations, p.E297G and p.D482G in bile salt export pump deficiency.

Background & Aims: Bile salt export pump (BSEP) deficiency frequently necessitates liver transplantation in childhood. In contrast to two predicted protein truncating mutations (PPTMs), homozygous p.D482G or p.E297G mutations are associated with relatively mild phenotypes, responsive to surgical interruption of the enterohepatic circulation (siEHC). The phenotype of patients with a compound heterozygous genotype of one p.D482G or p.E297G mutation and one PPTM has remained unclear. We aimed to assess their genotype-phenotype relationship. Methods: From the NAPPED database, we selected patients with homozygous p.D482G or p.E297G mutations (BSEP1/1; n = 31), with one p.D482G or p.E297G, and one PPTM (BSEP1/3; n = 30), and with two PPTMs (BSEP3/3; n = 77). We compared clinical presentation, native liver survival (NLS), and the effect of siEHC on NLS. Results: The groups had a similar median age at presentation (0.7-1.3 years). Overall NLS at age 10 years was 21% in BSEP1/3 vs. 75% in BSEP1/1 and 23% in BSEP3/3 (p <0.001). Without siEHC, NLS in the BSEP1/3 group was similar to that in BSEP3/3, but considerably lower than in BSEP1/1 (at age 10 years: 38%, 30%, and 71%, respectively; p = 0.003). After siEHC, BSEP1/3 and BSEP3/3 were associated with similarly low NLS, while NLS was much higher in BSEP1/1 (10 years after siEHC, 27%, 14%, and 92%, respectively; p <0.001). Conclusions: Individuals with BSEP deficiency with one p.E297G or p.D482G mutation and one PPTM have a similarly severe disease course and low responsiveness to siEHC as those with two PPTMs. This identifies a considerable subgroup of patients who are unlikely to benefit from interruption of the enterohepatic circulation by either surgical or ileal bile acid transporter inhibitor treatment. Impact and implications: This manuscript defines the clinical features and prognosis of individuals with BSEP deficiency involving the combination of one relatively mild and one very severe BSEP deficiency mutation. Until now, it had always been assumed that the mild mutation would be enough to ensure a relatively good prognosis. However, our manuscript shows that the prognosis of these patients is just as poor as that of patients with two severe mutations. They do not respond to biliary diversion surgery and will likely not respond to the new IBAT (ileal bile acid transporter) inhibitors, which have recently been approved for use in BSEP deficiency.

Investigation of serum phoenixin levels in patients with hypertension.

OBJECTIVE: Hypertension is a major modifiable risk factor for cardiovascular disease and premature death worldwide. Phoenixin is a newly identified neuropeptide with multiple bioactivity. However, there was no published data about phoenixin levels in hypertension. The aim of this study was to evaluate the relationship between phoenixin and hypertension. METHODS: This study was performed in 36 patients with hypertension and 36 healthy controls. Serum phoenixin-14 and phoenixin-20 levels were determined by Enzyme-Linked ImmunoSorbent Assay method. RESULTS: Serum phoenixin-14 and phoenixin-20 values were significantly lower in hypertension patients compared with the control group (p<0.001). The levels of phoenixin-14 were negatively correlated with weight (r=-0.376; p<0.005), body mass index (r=-0.407; p<0.001), systolic blood pressure (r=-0.586; p<0.001), and diastolic blood pressure (r=-0.319; p<0.01). There was a negative correlation between serum phoenixin-20 and weight (r=-0.378; p<0.005), body mass index (r=-0.383; p<0.005), systolic blood pressure (r=-0.551; p<0.001), and diastolic blood pressure (r=-0.306; p<0.01). We used receiver operating characteristic curve analyses to compare the diagnosis value of Phoenixin-14 and Phoenixin-20 levels in hypertensive patients. We found that Phoenixin-14 value is an area under the curve of 0.87 (cutoff value 404.7 ng/L, sensitivity 92%, specificity 72%) and Phoenixin-20 value is an area under the curve of 0.83 (cutoff value 209.9 ng/L, sensitivity 86%, specificity 75%). Phoenixin-14 did nearly show equally compared to phoenixin-20 in predicting hypertension. CONCLUSION: Serum phoenixin-14 and phoenixin-20 may be related to the pathogenesis of hypertension. Our findings indicated that serum phoenixin-14 and phoenixin-20 may serve as a novel biomarker for the diagnosis of hypertension.

Investigation of serum phoenixin levels in patients with hypertension

SUMMARY OBJECTIVE: Hypertension is a major modifiable risk factor for cardiovascular disease and premature death worldwide. Phoenixin is a newly identified neuropeptide with multiple bioactivity. However, there was no published data about phoenixin levels in hypertension. The aim of this study was to evaluate the relationship between phoenixin and hypertension. METHODS: This study was performed in 36 patients with hypertension and 36 healthy controls. Serum phoenixin-14 and phoenixin-20 levels were determined by Enzyme-Linked ImmunoSorbent Assay method. RESULTS: Serum phoenixin-14 and phoenixin-20 values were significantly lower in hypertension patients compared with the control group (p<0.001). The levels of phoenixin-14 were negatively correlated with weight (r=-0.376; p<0.005), body mass index (r=-0.407; p<0.001), systolic blood pressure (r=-0.586; p<0.001), and diastolic blood pressure (r=-0.319; p<0.01). There was a negative correlation between serum phoenixin-20 and weight (r=-0.378; p<0.005), body mass index (r=-0.383; p<0.005), systolic blood pressure (r=-0.551; p<0.001), and diastolic blood pressure (r=-0.306; p<0.01). We used receiver operating characteristic curve analyses to compare the diagnosis value of Phoenixin-14 and Phoenixin-20 levels in hypertensive patients. We found that Phoenixin-14 value is an area under the curve of 0.87 (cutoff value 404.7 ng/L, sensitivity 92%, specificity 72%) and Phoenixin-20 value is an area under the curve of 0.83 (cutoff value 209.9 ng/L, sensitivity 86%, specificity 75%). Phoenixin-14 did nearly show equally compared to phoenixin-20 in predicting hypertension. CONCLUSION: Serum phoenixin-14 and phoenixin-20 may be related to the pathogenesis of hypertension. Our findings indicated that serum phoenixin-14 and phoenixin-20 may serve as a novel biomarker for the diagnosis of hypertension.

Investigation of Vitamin B12 Deficiency in Patients with Acute Coronary Syndrome and its Relationship with Gensini Score.

BACKGROUND: Vitamin B12 deficiency is not an independent risk factor for cardiovascular disease. However, anemia due to vitamin B12 deficiency and also hyperhomocysteinemia are among the cardiovascular risk factors. The study aimed to determine the frequency of vitamin B12 deficiency in patients with acute coronary syndrome. We also aimed to ascertain whether there is a significant difference between obstructive coronary artery disease presence and its severity in patients with and without vitamin B12 deficiency using the Gensini score. METHODS: Patients who underwent coronary angiography due to acute coronary syndrome between June 1, 2018, and November 30, 2019, and whose vitamin B12 levels were measured were retrospectively analyzed. Coronary angiography results of the patients were evaluated with the Gensini scoring system. RESULTS: Anemia was observed in 32.6% (n = 135) of the patients who underwent coronary angiography with a diagnosis of acute coronary syndrome, and vitamin B12 deficiency was observed in 14.7% (n = 61). The median age was 69 years in anemic patients and 68 years in those with Vitamin B12 deficiency and was significantly higher than patients without anemia and vitamin B12 deficiency (p < 0.001 and p = 0.038, respectively). There was no statistically significant differences between the patients' Gensini scores with or without Vitamin B12 deficiency (p = 0.554). CONCLUSIONS: We concluded that anemia and vitamin B12 deficiency were higher in elderly patients with acute coronary syndrome. We found no significant difference when the Gensini score was used to evaluate obstructive coronary artery disease presence and its severity according to anemia and vitamin B12 deficiency. Investigating vitamin B12 levels in elderly patients with acute coronary syndrome should not be ignored.

Turkish Translation, Validation, and Reliability Analysis of Pediatric Eosinophilic Esophagitis Symptom Severity Module Version 2.0.

The Paediatric Eosinophilic Esophagitis Symptom Severity Modules Version 2.0 (T-PEESv2.0) was developed in English as a valid, reliable questionnaire for follow up. This work aimed to develop a Turkish version of T-PEESv2.0 via translation and cultural adaptation and then to test its validation and reliability. Methods: The PEESv2.0 was translated into Turkish by standardized procedural steps completed in cooperation with the Mapi Research Trust. The final version of the questionnaire was submitted to eosinophilic oesophagitis patients or their parents at 2 times point separated by 1 week. An age-matched control group was used to test the discriminant validity. Construct validity was tested using the Wilcoxon test, and internal consistency was tested using Cronbach's alpha. Test-retest reliability was measured with Cohen's kappa and intraclass correlation coefficient. Results: One hundred twenty-eight participants (70 patients, 58 parents) were enrolled. Fifty-eight (39.1%) of them completed T-PEESv2.0-parent by proxy and 70 (54.7%) were T-PEESv2.0. The Cronbach's alpha coefficient and intraclass correlation coefficient for test-retest reliability were >0.70 for both questionnaires and for all domain (frequency and severity) and total scores. For discriminant validity analysis, subscale (frequency and domain) and total scores of the patient group were compared with those of the control group. The subscale and total scores were significantly different between the groups (P < 0.05). Conclusion: T-PEESv2.0 appeared to be valid and reliable, ready to be introduced as a clinical and research tool for the assessment of patients with eosinophilic oesophagitis.

Standard immunosuppressive treatment reduces regulatory B cells in children with autoimmune liver disease.

Introduction: Autoimmune hepatitis (AIH) is a chronic liver disease caused by a perturbed immune system. The scarcity of short- and long-term immune monitoring of AIH hampered us to comprehend the interaction between immunosuppressive medication and immune homeostasis. Methods and patients: We recruited children with AIH at the time of diagnosis and at the 1st, 3rd, 6th, 12th, 18th, and 24th months of immunosuppression (IS). We also enrolled children with AIH being on IS for >2 years. Children with drug-induced liver injury (DILI), and those receiving tacrolimus after liver transplantation (LT), were enrolled as disease/IS control subjects. Healthy children (HC) were also recruited. Peripheral blood mononuclear cells (PBMCs) were isolated from all participants. Healthy liver tissue from adult donors and from livers without inflammation were obtained from children with hepatoblastoma. By using flow cytometry, we performed multi-parametric immune profiling of PBMCs and intrahepatic lymphocytes. Additionally, after IS with prednisolone, tacrolimus, rapamycin, or 6-mercaptopurine, we carried out an in vitro cytokine stimulation assay. Finally, a Lifecodes SSO typing kit was used to type HLA-DRB1 and Luminex was used to analyze the results. Results: Untreated AIH patients had lower total CD8 T-cell frequencies than HC, but these cells were more naïve. While the percentage of naïve regulatory T cells (Tregs) (CD4+FOXP3lowCD45RA+) and regulatory B cells (Bregs, CD20+CD24+CD38+) was similar, AIH patients had fewer activated Tregs (CD4+FOXP3highCD45RA - ) compared to HC. Mucosal-associated-invariant-T-cells (MAIT) were also lower in these patients. Following the initiation of IS, the immune profiles demonstrated fluctuations. Bregs frequency decreased substantially at 1 month and did not recover anymore. Additionally, the frequency of intrahepatic Bregs in treated AIH patients was lower, compared to control livers, DILI, and LT patients. Following in vitro IS drugs incubation, only the frequency of IL-10-producing total B-cells increased with tacrolimus and 6MP. Lastly, 70% of AIH patients possessed HLA-DR11, whereas HLA-DR03/DR07/DR13 was present in only some patients. Conclusion: HLA-DR11 was prominent in our AIH cohort. Activated Tregs and MAIT cell frequencies were lower before IS. Importantly, we discovered a previously unrecognized and long-lasting Bregs scarcity in AIH patients after IS. Tacrolimus and 6MP increased IL-10+ B-cells in vitro.

Cytomegalovirus Colitis in Immunocompetent and Immunocompromised Children: A 2-Center Study.

BACKGROUND: Data about cytomegalovirus (CMV) colitis in children are scarce. We aimed to describe the characteristics of childhood CMV colitis in terms of risk factors, clinical symptoms, diagnosis, therapeutic approaches, and outcomes. METHODS: Inflammatory bowel disease (IBD) and non-IBD patients with CMV colitis diagnosed by histology and tissue CMV PCR at 2 tertiary centers between January 2017 and November 2019 were studied. Clinical and laboratory data were retrieved from medical records. Underlying conditions, immune status, response to therapy and outcomes were described and followed up to 6 months after diagnosis. RESULTS: A total of 16 children (8 non-IBD, 7 ulcerative colitis and 1 Crohn's disease) with CMV colitis were included. All patients had persistent diarrhea (bloody in 13 cases). There was a significant age difference between IBD and non-IBD children (P < 0.05). The final diagnosis in 1 patient was immunodeficiency with a mutation in JAK1 gene. Three children were categorized as apparently immunocompromised and 4 children as apparently immunocompetent. Ulcer was not visible in 2 children from the non-IBD group. The mean fecal calprotectin level of IBD children was significantly higher than that of non-IBD children (376.12 ± 231.21 µg/g vs. 160.96 ± 69.94 µg/g, P < 0.05). After follow-up, 1 patient died because of another reason. Ganciclovir was used in 14 of 16 children for 3 weeks and the treatment was continued with valganciclovir in selected 6 children. CONCLUSIONS: CMV colitis is a rare but overlooked cause of prolonged diarrhea in immunocompetent and immunocompromised children. CMV colitis might present without any ulcer formation at colonoscopy in infants.

Platelet Indices' Usefulness in Determining whether Patients with COVID-19 Should be Treated as an Outpatient or Inpatient: a Retrospective Study.

BACKGROUND: Coronavirus disease 2019 (COVID-19) has affected the health system around the world. It is necessary to facilitate health care services with useful parameters in patient follow-up. In this study, we wanted to determine whether platelet indices can be used as an assistant parameter in the clinician's decision-making process regarding which of the patients diagnosed with COVID-19 should be treated by hospitalization. METHODS: This retrospective study was performed in a secondary care hospital in Mugla, Turkey. Demographic information such as age, gender, and comorbidities of patients admitted to the emergency pandemic outpatient clinic within five months and diagnosed with COVID-19, as well as discharge, hospitalization, or intensive care needs (ICU), and thirty-day mortality were noted. Also, patients with platelet count (PLT), mean platelet volume (MPV), platelet distribution width (PDW), thrombocytocrit (PCT), MPV/PLT, MPV/PCT, PDW/PLT, PDW/PCT values for outpatient follow-up, hospitalization, intensive care unit need, and 30-day mortality relationships were examined. RESULTS: A total of 93 patients diagnosed with COVID-19 were included in the study. It was observed that those with comorbidity had a statistically significant higher rate of hospitalization (p = 0.013). No statistically significant difference was found between outpatients and inpatients in terms of PLT, MPV, PDW, PCT, MPV/PLT, MPV/ PCT, PDW/PLT, and PDW/PCT (p > 0.05 for each). CONCLUSIONS: Platelet indices such as PLT, MPV, PDW, PCT, MPV/PLT, MPV/PCT, PDW/PLT, and PDW/PCT are not useful parameters for the clinician to distinguish between outpatient and inpatient treatment of patients with COVID-19.

The frequency of and factors affecting functional gastrointestinal disorders in infants that presented to tertiary care hospitals.

This study aimed to determine the prevalence of infantile functional gastrointestinal disorders (FGIDs) based on Rome IV diagnostic criteria, and to determine the associated patient demographic and nutritional characteristics. A total of 2383 infants aged 1-12 months which were evaluated by 28 general pediatricians and pediatric gastroenterologists on the same day at nine tertiary care hospitals around Istanbul, Turkey, between November 2017 and March 2018, were included in the study. Patients included consulted the pediatric outpatient clinics because of any complaints, but not for vaccines and/or routine well child follow-ups as this is not part of the activities in the tertiary care hospitals. The patients were diagnosed with FGIDs based on Rome IV diagnostic criteria. The patients were divided into a FGID group and non-FGID group, and anthropometric measurements, physical examination findings, nutritional status, risk factors, and symptoms related to FGIDs were evaluated using questionnaires. Among the 2383 infants included, 837 (35.1%) had ≥1 FGIDs, of which 260 (31%) had already presented to hospital with symptoms of FGIDs and 577 (69%) presented to hospital with other symptoms, but were diagnosed with FGIDs by a pediatrician. Infant colic (19.2%), infant regurgitation (13.4%), and infant dyschezia (9.8%) were the most common FGIDs. One FGID was present in 76%, and ≥2 FGIDs were diagnosed in 24%. The frequency of early supplementary feeding was higher in the infants in the FGID group aged ≤6 months than in the non-FGID group (P = 0.039).Conclusion: FGIDs occur quite common in infants. Since early diversification was associated with the presence of FGIDs, nutritional guidance and intervention should be part of the first-line treatment. Only 31% of the infants diagnosed with a FGID were presented because of symptoms indicating a FGID. What is Known: • The functional gastrointestinal disorders (FGIDs) are a very common disorder and affect almost half of all infants. • In infants, the frequency of FGIDs increases with mistakes made in feeding. When FGIDs are diagnosed in infants, nutritional support should be the first-line treatment. What is New: • This study shows that only a third of children presented to hospital because of the symptoms of FGIDs, but pediatricians were able to make the diagnosis in suspected infants after appropriate evaluation. • The early starting of complementary feeding (<6 months) is a risk factor for the development of FGIDs.

Assessment of Hearing Function in Children with Inflammatory Bowel Disease.

OBJECTIVES: Inflammatory bowel disease (IBD), a chronic pathology that affects many organ systems, appears after dysregulated immune response in genetically predisposed patients. Inner organ involvement has been shown in various autoimmune diseases because of its immunosensitivity. In this study, we aimed at showing sensorineural hearing loss (SNHL) as a result of possible subclinical inflammation in patients with IBD during the remission period. MATERIALS AND METHODS: We included 32 children with IBD and 31 healthy volunteers with comparable sex and age. Detailed ear-nose-throat examination was conducted for all, and patients were excluded if they had a history of ear infectionor trauma. Thereafter, the results of pure tone audiometry (PTA), high-frequency audiometry, and distortion product otoacoustic emissions testing were compared between the groups. RESULTS: There were no differences in terms of age, sex, and PTA values between controls and children with IBD. No statistical differences were found between responses at 250; 500; 2,000; 4,000; DP1000; DP1400; DP2000; DP2800;and DP4000 Hz as well as the signal-to-noise ratio (SNR) at 1,000 Hz when the controls and children with IBD (p>0.05 for all) were compared. However, the mean responses at 1,000; 8,000; 10,000; 12,500; 16,000; SNR1400; SNR2000; SNR2800; and SNR4000Hz of the children with IBD were significantly higher than those of the controls (p<0.05 for all). CONCLUSION: Initial SNHL appears at high frequencies in pediatric patients with IBD.

Helicobacter pylori infection in children with celiac disease: Multi-center, cross-sectional study.

BACKGROUND: An inverse association has been suggested between celiac disease (CD) and Helicobacter pylori (Hp) infection in children; however, there are inconsistent data. The purpose of this multi-center study is to evaluate the association between Hp and CD in childhood. METHODS: Children who underwent endoscopy between July 2016 and November 2017 in four pediatric gastroenterology centers were included in the study. Patients with a history of previous Hp eradication, antibiotic or acid-suppressive drug therapy in the last 4 weeks, and any underlying chronic disease were excluded. The presence of Hp infection was confirmed by both histopathology and the rapid urease test. The ones who had the diagnosis of CD were compared with the children who underwent endoscopy during the same period and had another diagnosis. Duodenal histopathology of children with CD was categorized according to the modified Marsh classification. RESULTS: Of 3056 endoscopies performed in the study period, 2484 cases were eligible for the study. A total of 482 CD patients (mean age: 9.71 ± 4.63 years, 58.5% girls) and 2060 controls (mean age: 9.92 ± 4.66 years, 54.6% girls) were included in the study. The rate of Hp infection was significantly lower in CD group (26.3% vs 50.1%, P < .01). The difference was prominent even in children younger than 6 years old (P < .01). There was no correlation between Hp infection and the modified Marsh scores in CD (P > .05). CONCLUSION: In this cross-sectional study, where Hp infection is common even in the pediatric population, the frequency of Hp infection was significantly lower in children with CD compared with the controls. Systematic cohort studies are necessary to clarify causal association between Hp infection and the development of celiac disease.

Genotype correlates with the natural history of severe bile salt export pump deficiency.

BACKGROUND & AIMS: Mutations in ABCB11 can cause deficiency of the bile salt export pump (BSEP), leading to cholestasis and end-stage liver disease. Owing to the rarity of the disease, the associations between genotype and natural history, or outcomes following surgical biliary diversion (SBD), remain elusive. We aimed to determine these associations by assembling the largest genetically defined cohort of patients with severe BSEP deficiency to date. METHODS: This multicentre, retrospective cohort study included 264 patients with homozygous or compound heterozygous pathological ABCB11 mutations. Patients were categorized according to genotypic severity (BSEP1, BSEP2, BSEP3). The predicted residual BSEP transport function decreased with each category. RESULTS: Genotype severity was strongly associated with native liver survival (NLS, BSEP1 median 20.4 years; BSEP2, 7.0 years; BSEP3, 3.5 years; p <0.001). At 15 years of age, the proportion of patients with hepatocellular carcinoma was 4% in BSEP1, 7% in BSEP2 and 34% in BSEP3 (p = 0.001). SBD was associated with significantly increased NLS (hazard ratio 0.50; 95% CI 0.27-0.94: p = 0.03) in BSEP1 and BSEP2. A serum bile acid concentration below 102 µmol/L or a decrease of at least 75%, each shortly after SBD, reliably predicted NLS of ≥15 years following SBD (each p <0.001). CONCLUSIONS: The genotype of severe BSEP deficiency strongly predicts long-term NLS, the risk of developing hepatocellular carcinoma, and the chance that SBD will increase NLS. Serum bile acid parameters shortly after SBD can predict long-term NLS. LAY SUMMARY: This study presents data from the largest genetically defined cohort of patients with severe bile salt export pump deficiency to date. The genotype of patients with severe bile salt export pump deficiency is associated with clinical outcomes and the success of therapeutic interventions. Therefore, genotypic data should be used to guide personalized clinical care throughout childhood and adulthood in patients with this disease.

Cardiac functions and aortic elasticity in children with inflammatory bowel disease: effect of age at disease onset.

AIM: Childhood onset inflammatory bowel disease is more aggressive and has rapidly progressive clinical course than adult inflammatory bowel disease. Early-onset inflammatory bowel disease has more severe clinical progression as a subspecialised group of monogenic inflammatory bowel disease. We studied cardiac functions and aortic elasticity in children with early- and late-onset inflammatory bowel disease in remission period. METHODS: Thirty-three paediatric patients were divided into subgroups according to age of disease onset (<10 and >10 years of age). Twenty-five healthy children were admitted as control group. M-Mode echocardiography and pulsed wave Doppler echocardiography were performed. Strain, distensibility, stiffness index of ascending, and abdominal aorta were evaluated. RESULTS: Interventricular septum (mm) and left ventricular end-systolic diameter were higher (6.9 ± 1.2, 26.2 ± 4.6) in early-onset inflammatory bowel disease patients than control patients (6.1 ± 1.27, 22.7 ± 4.12) (p = 0.050, p = 0.050). Mitral E/E' ratio and myocardial performance index were increased in inflammatory bowel disease and early-onset inflammatory bowel disease groups than control group (p = 0.046, p = 0.04; p = 0.023, p = 0.033). Diastolic functions were found to be impaired in inflammatory bowel disease and early-onset inflammatory bowel disease groups according to control group, while there was no difference between late-onset inflammatory bowel disease and control groups in terms of diastolic functions. Mitral E/A ratio was lower in inflammatory bowel disease patients and early-onset inflammatory bowel disease patients (1.46 ± 0.32, 1.4 ± 0.21) than control patients (1.70 ± 0.27) (p = 0.013, p = 0.004). Aortic elasticity did not differ between groups. CONCLUSION: Chronic low-grade inflammation has effects on left ventricular diameters and diastolic function in remission period. Aortic elasticity is not affected in our study groups.