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1.
Dig Dis ; 40(2): 168-174, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-33895735

RESUMEN

INTRODUCTION: Low serum titer of anti-tissue transglutaminase (tTG) has been described in various conditions without any evidence of celiac disease (CD). Infectious agents have been suggested to trigger autoimmunity and promote the production of anti-tTG. The aim of this study was to investigate if there is a link between a positive celiac serology and concomitant Helicobacter pylori infection in children. METHODS: The data of 178 pediatric patients who underwent upper gastrointestinal endoscopy due to positive celiac serology were compiled. The patients whose histopathologic findings were not consistent with CD were followed on gluten-containing diet. The changes in the serum level of anti-tTG IgA on the follow-up were compared between H. pylori-infected and noninfected patients after the eradication of H. pylori. RESULTS: Of 155 patients who met the inclusion criteria, 119 (group 1) were diagnosed as CD, and duodenal histopathology of the remaining 36 children (group 2) was not compatible with CD. In group 2, 11 out of 36 (30.5%) patients were infected with H. pylori. After the eradication of H. pylori, anti-tTG IgA level either decreased or dropped below cutoff value in 9/11 (81%) patients while it was 20% in those who were not infected with H. pylori in the 6th month of the follow-up (p = 0.001). CONCLUSION: Our results suggest that H. pylori infection may be the cause of false or transient positive celiac serology. Thus, a positive celiac serology should be carefully interpreted in the presence of H. pylori infection before confirming the diagnosis of this life-long disease.


Asunto(s)
Enfermedad Celíaca , Infecciones por Helicobacter , Helicobacter pylori , Autoanticuerpos , Enfermedad Celíaca/diagnóstico , Niño , Infecciones por Helicobacter/complicaciones , Humanos , Inmunoglobulina A , Proteína Glutamina Gamma Glutamiltransferasa 2 , Transglutaminasas
2.
J Pediatr Gastroenterol Nutr ; 75(4): e61-e66, 2022 10 01.
Artículo en Inglés | MEDLINE | ID: mdl-35809240

RESUMEN

OBJECTIVES: It is not clear whether the characteristics of pediatric inflammatory bowel disease (IBD) differ between Eastern and Western countries. The aim of this study was to analyze the characteristics of PIBD in Turkey, according to the age at diagnosis. METHODS: The data of 176 children with IBD who were followed in our center were analyzed. Patients were divided into early (EO-IBD, onset at 2 to <10 years) and later-onset (LO-IBD, 10 to ≤17 years) IBD according to the age at diagnosis. Patients' data with ulcerative colitis (UC) and Crohn's disease (CD) were compared. RESULTS: Of 176 patients, 47 (26.7%) were diagnosed with EO-IBD. Patients with early-onset ulcerative colitis (EO-UC) had the highest rate of family history of IBD (17.6%). Pancolitis was the most common form of UC regardless of the age at onset. The rate of moderate-severe disease activity in later-onset UC (62.5%) was higher than in EO-UC (37.5%). A higher rate of extraintestinal manifestations was observed in EO-IBD patients, particularly in EO-UC (38.2%) than in LO-IBD patients. Patients with early-onset CD (EO-CD) had predominantly colonic involvement and nonstricturing, nonpenetrating disease behavior. The rate of perianal disease in patients with later-onset CD (LO-CD) (64.5%) was noticeably higher than those with EO-CD (23%). CONCLUSIONS: Our results suggest that patients with EO-UC represented a distinct phenotype with a mild disease activity, high rate of extraintestinal symptoms, and a high proportion of family history. The analysis of our IBD cohort also demonstrated remarkably high rate of perianal disease, particularly in patients with LO-CD.


Asunto(s)
Colitis Ulcerosa , Enfermedad de Crohn , Enfermedades Inflamatorias del Intestino , Colitis Ulcerosa/diagnóstico , Colitis Ulcerosa/epidemiología , Enfermedad de Crohn/diagnóstico , Enfermedad de Crohn/epidemiología , Humanos , Enfermedades Inflamatorias del Intestino/diagnóstico , Enfermedades Inflamatorias del Intestino/epidemiología , Fenotipo
3.
Eur J Pediatr ; 181(9): 3283-3289, 2022 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-35739293

RESUMEN

Recent guidelines suggest non-biopsy serology-based approach for the diagnosis of celiac disease; however, there is no evidence-based data regarding noninvasive follow-up of mucosal healing. The aim of this study is to investigate the efficacy of serology in reflecting mucosal status in the follow-up of pediatric patients with celiac disease. This is a validation study conducted at a university hospital. Patients who had biopsy proven celiac disease (Marsh III) at diagnosis, and had been followed-up for at least 12 months, were prospectively evaluated with duodenal biopsies. tTG-IgA and EMA tests were performed on the day of endoscopy. One hundred four patients with a mean age of 7.4 ± 4.02 years were included in the study. The sensitivity and specificity of tTG-IgA were 85.2% and 61% respectively, with a high negative predictive value (NPV) of 92.2% but a very low positive predictive value (PPV) of 43.4%. We found that a cutoff value of 68.5 U/mL for tTG-IgA had a sensitivity, specificity of 85.2% and 85.7% respectively. The AUC was 0.891. The sensitivity and specificity of EMA was 77.8% and 87% respectively, with a high NPV of 91.8% but low PPV of 67.7%. CONCLUSION: This study suggests that negative tTG-IgA and/or EMA can be used as an indicator of mucosal improvement in the follow-up of pediatric patients with celiac disease. However, positive serology (i.e., < 10 × ULN) may be misleading in reflecting mucosal status in the follow-up of pediatric patients with celiac disease. WHAT IS KNOWN: • The tissue transglutaminase IgA (tTG-IgA) and endomysium IgA (EMA) tests are widely used, sensitive and reliable diagnostic tests, but their role in monitoring adherence to dietary treatment in celiac patients has not yet been demonstrated. • There is still no reliable and non-invasive marker of persistent villous atrophy or mucosal recovery. WHAT IS NEW: • Negative celiac serology detected in the follow-up of pediatric patients with celiac disease was successful in demonstrating histopathological mucosal healing. • Positive celiac serology, which is highly reliable in the diagnosis of celiac disease, has not been successful in reflecting mucosal status when used in the follow-up of pediatric patients with celiac disease.


Asunto(s)
Enfermedad Celíaca , Autoanticuerpos , Enfermedad Celíaca/diagnóstico , Niño , Preescolar , Estudios de Seguimiento , Proteínas de Unión al GTP , Humanos , Inmunoglobulina A , Proteína Glutamina Gamma Glutamiltransferasa 2 , Sensibilidad y Especificidad , Transglutaminasas
4.
Dig Dis ; 39(5): 444-450, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33429389

RESUMEN

OBJECTIVES: Data regarding Helicobacter pylori (Hp) infection frequency in concurrent celiac disease (CD) and type 1 diabetes mellitus (T1DM) (CD + T1DM) are anecdotal. This study aimed to evaluate the association between Hp and concomitant CD + T1DM in children. METHODS: In this 2-center, case-control study, children who underwent esophago-gastro-duodenoscopy (EGD) were studied. CD diagnosis was established by favorable histology and serology. Hp infection was confirmed by both histology and the rapid urease test. Patients were divided into 3 groups as CD only (CDo), CD + T1DM, and non-CD children who underwent endoscopy as controls. RESULTS: Among the 1,431 EGDs performed, 783 cases were eligible. Overall, 215 cases had CDo (mean age: 9.12 ± 4.18 years, 58.1% girls), 63 cases had CD + T1DM (mean age: 9.29 ± 4.46 years, 50.8% girls), and 505 cases were controls (mean age: 9.69 ± 4.52 years, 56.6% girls). Hp infection rate was significantly lower in CD + T1DM group (controls: 49.7% vs. CDo: 32.1% vs. CD + T1DM: 20.6%, p < 0.01). After adjustment for age, gender, and socioeconomic status, the Hp infection rate was still significantly low (adjusted odds ratio [aOR]: 1.57, 95% confidence interval (CI): 1.35-1.83, p < 0.01). A difference in Hp infection rate between controls and CDo group (aOR: 1.43, 95% CI: 1.09-2.12, p < 0.05), and between CDo and CD + T1DM (aOR: 0.89, 95% CI: 0.65-1.54, p < 0.05) group was significant. The severity of duodenal lesions and the presence of Hp infection were not correlated in all celiac children (r: 0.113, p > 0.05). CONCLUSION: The frequency of Hp infection was significantly lower in CD + T1DM children, compared to the CDo group and the controls.


Asunto(s)
Enfermedad Celíaca , Diabetes Mellitus Tipo 1 , Infecciones por Helicobacter , Helicobacter pylori , Adolescente , Estudios de Casos y Controles , Enfermedad Celíaca/complicaciones , Enfermedad Celíaca/epidemiología , Niño , Preescolar , Diabetes Mellitus Tipo 1/complicaciones , Diabetes Mellitus Tipo 1/epidemiología , Femenino , Infecciones por Helicobacter/complicaciones , Infecciones por Helicobacter/epidemiología , Humanos , Masculino
5.
Helicobacter ; 25(3): e12691, 2020 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-32237105

RESUMEN

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.


Asunto(s)
Enfermedad Celíaca/complicaciones , Infecciones por Helicobacter , Adolescente , Niño , Preescolar , Estudios de Cohortes , Estudios Transversales , Duodeno/patología , Femenino , Infecciones por Helicobacter/complicaciones , Helicobacter pylori , Humanos , Masculino
6.
J Pediatr Gastroenterol Nutr ; 70(5): 574-579, 2020 05.
Artículo en Inglés | MEDLINE | ID: mdl-32044836

RESUMEN

OBJECTIVE: Continued progress in our understanding of the food protein-induced allergic proctocolitis (FPIAP) will provide the development of diagnostic tests and treatments. We aimed to identify precisely the clinical features and natural course of the disease in a large group of patients. Also, we investigated the predicting risk factors for persistent course since influencing parameters has not yet been established. METHODS: Infants who were admitted with rectal bleeding and had a diagnosis of food protein-induced allergic proctocolitis in 5 different allergy or gastroenterology outpatient clinics were enrolled. Clinical features, laboratory tests, and prognosis were evaluated. Risk factors for persistent course were determined by logistic regression analyses. RESULTS: Among the 257 infants, 50.2% (n = 129) were girls and cow's milk (99.2%) was the most common trigger. Twenty-four percent of the patients had multiple food allergies and had more common antibiotic use (41.9% vs 11.8%), atopic dermatitis (21% vs 10.2%), wheezing (11.3% vs 1.5%), colic (33.8% vs 11.2%), and IgE sensitization (50% vs 13.5%) compared to the single-food allergic group (P < 0.001, P = 0.025, P = 0.003, P < 0.001, respectively). In multivariate logistic regression analysis, presence of colic (odds ratio [OR]: 5.128, 95% confidence interval [CI]: 1.926-13.655, P = 0.001), IgE sensitization (OR: 3.964, 95% CI: 1.424-11.034, P = 0.008), and having allergy to multiple foods (OR: 3.679, 95% CI: 1.278-10.593, P = 0.001] were found to be risk factors for continuing disease after 1 year of age. CONCLUSION: Although most children achieve tolerance at 1 year of age, IgE sensitization, allergy to multiple foods, and presence of colic were risk factors for persistent course and late tolerance. In this context, these children may require more close and extended follow-up.


Asunto(s)
Hipersensibilidad a los Alimentos , Hipersensibilidad a la Leche , Proctocolitis , Alérgenos , Animales , Bovinos , Niño , Femenino , Hipersensibilidad a los Alimentos/complicaciones , Hipersensibilidad a los Alimentos/diagnóstico , Hipersensibilidad a los Alimentos/epidemiología , Humanos , Tolerancia Inmunológica , Lactante , Masculino , Hipersensibilidad a la Leche/complicaciones , Hipersensibilidad a la Leche/diagnóstico , Hipersensibilidad a la Leche/epidemiología , Proctocolitis/diagnóstico , Proctocolitis/etiología , Factores de Riesgo
7.
Dig Dis ; 37(4): 334-342, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-30836366

RESUMEN

BACKGROUND AND OBJECTIVES: Bowel preparation (BP) for colonoscopy is a challenging procedure in children and different regimens have been used for this purpose. Polyethylene glycol (PEG) is the most preferred agent in recent years. The primary aim of this study was to evaluate the efficacy of 1-day PEG-3350 with bisacodyl (PEG-B) and comparing it with 3-day sennosides A+B. METHOD: In this prospective, randomized, and single-blinded study, children aged 2-18 years were included in the PEG-B group for 1 day or in Senna group for 3 days. The effectiveness of BP was assessed according to the Ottawa and Boston BP scales, compliance and adverse effects were also recorded. Pre- and post-preparation biochemistry were obtained for investigation of safety of both regimens. RESULTS: Successful BP was observed in 88.3% (n = 53/60) of PEG-B and 86% (n = 55/64) of Senna groups according to Boston scale, and it was 85% (n = 51/60) and 84.4% (n = 54/64), respectively, according to Ottawa scale. The cecal intubation rate was 96.7% (n = 58/60) in the PEG-B group and 93.8% (n = 60/64) in the Senna group. Ease of administration and disturbance in regular daily activities was better in the PEG-B group (p < 0.05). There was no major adverse event and biochemical abnormality in both groups. The correlation between Ottawa and Boston scales was found to be excellent (r2 = -0.954, p < 0.01). CONCLUSIONS: The efficacy, safety, and adverse effect profile of 1-day BP with PEG-B regimen was found to be similar to 3-day sennosides regimen, however, the PEG-B regimen had advantages such as short duration, ease of administration, and better patient comfort. Also, high correlation rate between the Boston and Ottawa scales in pediatric patients was remarkable.


Asunto(s)
Bisacodilo/farmacología , Catárticos/farmacología , Colonoscopía , Polietilenglicoles/farmacología , Extracto de Senna/farmacología , Bisacodilo/efectos adversos , Catárticos/efectos adversos , Niño , Femenino , Humanos , Masculino , Cooperación del Paciente , Estudios Prospectivos , Extracto de Senna/efectos adversos , Senósidos
8.
Dig Dis ; 37(1): 45-52, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-30153682

RESUMEN

BACKGROUND: It has been reported that 5-50% of patients with primary immune deficiencies (PID) may present with or develop gastrointestinal (GI) manifestations. OBJECTIVE: This study was aimed at analyzing GI and related endoscopic, histopathological findings in children with PID. METHODS: Children with PID who were evaluated by endoscopy between 2005 and 2016 were enrolled in this study. Demographic data, growth parameters, signs and symptoms at diagnosis were obtained. RESULTS: Of 425 children with PID, 195 had GI manifestations. Forty-seven of 195 children required endoscopic investigation, 30 (63.8%) were male, and the mean age was 7.7 ± 5 years. The rate of consanguinity was 61.7%, and the most common symptom was chronic diarrhea (57.4%). Seventy-two percent of the patients were malnourished. Giardia intestinalis was detected in 4, and Helicobacter pylori was confirmed in 8/45 (17.7%) patients. Non-celiac villous flatting was discovered in 15.5% of patients. Twelve patients were diagnosed as having immunodeficiency associated inflammatory bowel disease (IBD)-like colitis. CONCLUSIONS: PID may present with GI manifestations or develop during the course of the disease. Investigating immunodeficiency in patients with atypical GI symptoms can provide an appropriate therapeutic option, and an improved quality of life, particularly in populations with a high rate of consanguinity.


Asunto(s)
Enfermedades Gastrointestinales/complicaciones , Enfermedades Gastrointestinales/inmunología , Síndromes de Inmunodeficiencia/complicaciones , Adolescente , Niño , Preescolar , Endoscopía , Femenino , Enfermedades Gastrointestinales/patología , Humanos , Síndromes de Inmunodeficiencia/patología , Lactante , Masculino , Fenotipo , Calidad de Vida
9.
J Pediatr Gastroenterol Nutr ; 68(1): 3-6, 2019 01.
Artículo en Inglés | MEDLINE | ID: mdl-30169453

RESUMEN

OBJECTIVES: Esophagogastroduodenoscopy (EGD) can cause fear and anxiety in children. Cortisol, which is the most important glucocorticoid hormone in humans, can increase under physiological stress. The purpose of this study was to measure the salivary cortisol level (SCL) and anxiety level in patients undergoing EGD and evaluate their effects on the procedure. METHODS: Children undergoing EGD under sedoanalgesia with propofol for various reasons were included. Their basal SCLs were compared with those of healthy age- and sex-matched controls. Moreover, SCL of the patient group at 30 minutes before EGD and 2 hours after the procedure were measured. Their anxiety scores were calculated using the modified Yale Preoperative Anxiety Scale before EGD. Duration of endoscopy, sedation, and recovery and total propofol doses were recorded. RESULTS: Demographic properties of the patient group (n = 119; 10.9 ±â€Š3.2 years; 43.7% boys) and control group (n = 85; 11.8 ±â€Š2.8 years; 45.1% boys) were not significantly different. Basal SCLs of both groups were similar (16.9 ±â€Š0.7 vs 19.7 ±â€Š1.8 ng/mL, P = 0.16). SCL before EGD in the patient group was significantly higher than basal and post-EGD values (P < 0.001 for each). Pre-EGD SCL was positively correlated with anxiety level, propofol dose, and duration of sedation, procedure, and recovery. Anxiety levels of patients were positively correlated with propofol dose and duration of sedation and recovery, and negatively correlated with age. CONCLUSIONS: Childhood EGD is a significant stress factor, which was reflected by the pre-procedural SCL in this study. Increased anxiety resulted in increased propofol doses and sedoanalgesia-related procedural durations, which may cause potential complications.


Asunto(s)
Ansiedad/diagnóstico , Sedación Consciente/estadística & datos numéricos , Endoscopía del Sistema Digestivo/psicología , Hidrocortisona/análisis , Complicaciones Posoperatorias/psicología , Adolescente , Ansiedad/etiología , Niño , Sedación Consciente/métodos , Sedación Consciente/psicología , Endoscopía del Sistema Digestivo/efectos adversos , Femenino , Humanos , Hipnóticos y Sedantes/administración & dosificación , Masculino , Periodo Preoperatorio , Propofol/administración & dosificación , Saliva/química , Estrés Fisiológico
10.
Turk Arch Pediatr ; 58(4): 388-394, 2023 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-37357452

RESUMEN

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.

11.
Indian Pediatr ; 59(11): 867-870, 2022 11 15.
Artículo en Inglés | MEDLINE | ID: mdl-36148747

RESUMEN

OBJECTIVES: The aim of this study was to evaluate the features of asymptomatic siblings of index celiac patients who were diagnosed with celiac disease (CD) at the initial screening. METHODS: We reviewed hospital records of 210 children with CD. The characteristics of sibling celiacs (n=24) were compared with index celiacs (n=186). RESULTS: At diagnosis, sibling celiacs were older than index celiacs (mean (SD) 10.4 (2.7) vs 8.2 (4.3) years; P=0.02). There were no significant differences between sibling and index celiacs in terms of serum anti-tTG IgA titer (≥10xULN, 83.3% vs 85%), and most of the patients had moderate/severe villous atrophy in both groups. The rates of iron deficiency anemia, folic acid deficiency, wasting and stunting were comparable between sibling and index celiac patients. CONCLUSIONS: Siblings with CD were older than index children with CD at diagnosis, and their characteristics were similar to symptomatic index children with CD, despite not having any complaints.


Asunto(s)
Enfermedad Celíaca , Niño , Humanos , Enfermedad Celíaca/diagnóstico , Enfermedad Celíaca/epidemiología , Transglutaminasas , Hermanos , Autoanticuerpos , Inmunoglobulina A
12.
Eurasian J Med ; 54(2): 186-190, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-35703528

RESUMEN

OBJECTIVE: Fetuin-A is a multifunctional non-collagen protein that plays a role in bone mineralization. Celiac disease is a chronic inflammatory disorder of the small intestine due to exposure to gluten. In this research, it was aimed to investigate levels of Fetuin-A and its relationship with bone mineral density in children with celiac disease. MATERIALS AND METHODS: The study was conducted on 59 children with celiac and 29 healthy children. The celiac disease group was composed of three groups, newly diagnosed, gluten-free diet compliant and, non- gluten- free diet compliant patients. Serum Fetuin-A concentrations were measured by an enzyme-linked immuno- sorbent assay kit. Measurement of bone mineral density was performed a dual-energy x-ray absorptiometry. RESULTS: Serum Fetuin-A levels were 136.85 ± 38.09 µg/L and 112.95 ± 44.39 µg/L in the celiac disease and healthy control groups, respectively. There was a statistically significant difference between groups in levels of serum Fetuin-A (P < .05). A significant positive correlation was observed between serum Fetuin-A and bone mineral density Z-score in the celiac patients. CONCLUSION: Increased Fetuin-A levels and positive correlation between Fetuin-A and bone mineral density in children with celiac disease suggest that Fetuin-A may be a biomarker for celiac disease.

13.
Turk J Gastroenterol ; 32(3): 240-247, 2021 03.
Artículo en Inglés | MEDLINE | ID: mdl-34160353

RESUMEN

BACKGROUND: Perianal disease is reported more widely in pediatric Crohn patients than in the past, and has been stated as an independent modifier of the disease behavior. In this study, we aimed to analyze the clinical characteristics and outcomes of fistulising perianal Crohn's disease (fpCD) in the pediatric age group. METHODS: A total number of 149 children with an established diagnosis of inflammatory bowel disease who have been diagnosed before 18 years of age and followed in our tertiary center were revised. Clinical, endoscopic, laboratory, and radiologic data of 50 patients with CD, who had at least 18 months follow-up data, were compiled. RESULTS: Of 50 patients, 26 (52%) were diagnosed as fpCD (38% at onset). More than half of the patients without any notable external orifices around the perianal area were diagnosed as fpCD by an magnetic resonance imaging (MRI). Pediatric fpCD patients had a higher disease activity score and platelet count, lower serum albumin level, and a higher rate of granuloma in the biopsy samples, compared with non-fistulising patients. A considerably high rate of surgical interventions (i.e., seton placement 46% and abscess drainage 15%) was performed in combination with infliximab. CONCLUSION: Fistulising perianal Crohn's disease seems to be more common than previously reported in the pediatric age group. A severe course of the disease might serve as a warning for the development of fpCD. A careful physical examination and use of perianal MRI with a high index of suspicion may increase the likelihood of fistula detection, hence may change the treatment strategy.


Asunto(s)
Enfermedad de Crohn , Fístula Rectal , Niño , Enfermedad de Crohn/terapia , Humanos , Fístula Rectal/terapia , Resultado del Tratamiento
14.
J Clin Endocrinol Metab ; 106(10): e4142-e4154, 2021 09 27.
Artículo en Inglés | MEDLINE | ID: mdl-33999151

RESUMEN

CONTEXT: Developmental disorders of the pituitary gland leading to congenital hypopituitarism can either be isolated or associated with extrapituitary abnormalities (syndromic hypopituitarism). A large number of syndromic hypopituitarism cases are linked to mutations in transcription factors. The forkhead box A2 (FOXA2) is a transcription factor that plays a key role in the central nervous system, foregut, and pancreatic development. OBJECTIVE: This work aims to characterize 2 patients with syndromic hypopituitarism due to FOXA2 gene defects. RESULTS: We report a novel heterozygous nonsense c.616C > T(p.Q206X) variant that leads to a truncated protein that lacks part of the DNA-binding domain of FOXA2, resulting in impaired transcriptional activation of the glucose transporter type 2 (GLUT2)-luciferase reporter. The patient is the sixth patient described in the literature with a FOXA2 mutation, and the first patient exhibiting pancreatic hypoplasia. We also report a second patient with a novel de novo 8.53 Mb deletion of 20p11.2 that encompasses FOXA2, who developed diabetes mellitus that responded to sulfonylurea treatment. CONCLUSION: Our 2 cases broaden the molecular and clinical spectrum of FOXA2-related disease, reporting the first nonsense mutation and the first case of pancreatic dysgenesis.


Asunto(s)
Diabetes Mellitus/congénito , Factor Nuclear 3-beta del Hepatocito/genética , Hipopituitarismo/congénito , Páncreas/anomalías , Hipófisis/anomalías , Codón sin Sentido , Transportador de Glucosa de Tipo 2/genética , Humanos , Lactante , Masculino , Síndrome , Factores de Transcripción/genética , Activación Transcripcional
15.
J Pediatr Endocrinol Metab ; 33(3): 409-415, 2020 Mar 26.
Artículo en Inglés | MEDLINE | ID: mdl-32069238

RESUMEN

Objectives We aimed to assess whether age at menarche and pubertal development (Tanner stage) are related to gluten-free diet (GFD) adherence, hormonal status, micro-nutrient levels and body mass index (BMI) in children with celiac disease (CD). Methods In this two-center, cross-sectional study, CD patients and healthy individuals were studied. CD patients were re-evaluated after 1 year of follow-up. Height, weight, Tanner stage, age at menarche and diet compliance were recorded. Blood samples for anti-tissue transglutaminase IgA (TTG), pituitary gonadotropins, gonadal hormones, prolactin and micronutrients (folate, vitamin B12, vitamin D, ferritin, iron and, iron-binding capacity [IBC]) were assayed. Results Consecutive age- and sex-matched 228 celiac patients (12.92 ± 2.35 years; 67.9% female) and 135 non-celiac healthy individuals (12.77 ± 2.25 years; 65.2% female) were studied. Tanner stage was significantly higher in the control group for both girls and boys (p < 0.05). The mean age at menarche was significantly lower in healthy individuals (13.13 ± 1.01 vs. 12.15 ± 0.97, p < 0.001). By multivariate analysis, GFD adherence, transferrin saturation (sTrf), total IBC (TIBC) and vitamin D status were found to be positively correlated with Tanner stage (p < 0.05). After 1 year, 192 of the CD patients were re-evaluated. GFD compliance was better (TTG levels: 135.59 ± 73.88 vs. 71.53 ± 69.40, p < 0.001) and it was correlated with Tanner stage (r = 0.49, p < 0.01), sTrf (r = 0.66, p < 0.01) and IBC (r = 0.23, p < 0.05). Conclusions Our data show that adequate weight gain, adherence to GFD, sufficient iron and vitamin D status are essential factors for salubrious puberty in CD patients.


Asunto(s)
Enfermedad Celíaca/complicaciones , Enfermedad Celíaca/dietoterapia , Dieta Sin Gluten , Pubertad , Adolescente , Antropometría , Estatura , Peso Corporal , Enfermedad Celíaca/patología , Niño , Estudios Transversales , Femenino , Hormonas/sangre , Humanos , Masculino , Menarquia , Cooperación del Paciente , Estudios Prospectivos , Maduración Sexual , Vitaminas/sangre
16.
Turk Pediatri Ars ; 55(4): 441-444, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-33414665

RESUMEN

Sarcoidosis is a chronic multisystemic granulomatous disease that predominantly involves the thoracic lymph nodes and lungs and primarily occurs in young adults. Isolated extrapulmonary localization is uncommon in adults, and exceptionally rare in the pediatric age group. A 4-year-old male patient with chronic diarrhea and abdominal distention for the last 8 months is presented. Endoscopic biopsies, obtained during gastroscopy and colonoscopy, revealed noncaseating granulomas in all segments of the gastrointestinal tract. A noncaseating granuloma was also demonstrated in the liver biopsy. Granulomatous inflammation of both the gastrointestinal system and liver along with elevated serum angiotensin-converting enzyme were consistent with sarcoidosis. The peculiarity of our pediatric sarcoidosis was the involvement of whole gastrointestinal system, which is exceptionally rare in all age groups. Furthermore, this is the youngest case in the literature with gastrointestinal and hepatic sarcoidosis in the absence of pulmonary involvement at onset.

17.
Saudi J Gastroenterol ; 25(4): 262-267, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-31044746

RESUMEN

BACKGROUND/AIMS: This study aimed to determine whether the anxiety levels of pediatric patients who undergo endoscopy are reduced after receiving preparatory information about the endoscopic procedure by monitoring their salivary cortisol (s-cortisol) levels. PATIENTS AND METHODS: A total of 184 children undergoing gastroscopy under sedoanalgesia were included in the study. All the patients received a brief explanation of the endoscopic procedure. Patients were divided into two groups; Group Unexplained did not receive any further information other than a brief explanation of the procedure, Group Explained received more detailed explanation of the procedure. To determine anxiety levels, saliva specimens were taken on the day before the procedure to examine cortisol levels before and after endoscopy. Anxiety scores before endoscopy were calculated by the modified Yale Preoperative Anxiety Scale. Patients were monitored throughout sedoanalgesia, including during the endoscopy, sedation and recovery, and total propofol dosages were recorded. RESULTS: Eighty-nine children undergoing gastroscopy (age 11.55 ± 2.52 years; 50.5% girls) constituted Group Explained and 95 children undergoing gastroscopy (age 11.44 ± 2.66 years; 56.8% male) constituted Group Unexplained. The anxiety score, duration of sedation, endoscopy and recovery, propofol dose, pre- and post-endoscopy s-cortisol levels were significantly reduced in Group Explained. CONCLUSIONS: We demonstrated that when endoscopic procedure is explained broadly to a child, the procedural stress is significantly less, as measured by the s-cortisol levels and the anxiety questionnaire. It is important for the attending physician to explain all aspects of examination carefully.


Asunto(s)
Ansiedad/prevención & control , Gastroscopía/efectos adversos , Hidrocortisona/metabolismo , Educación del Paciente como Asunto/métodos , Saliva/metabolismo , Estrés Psicológico/prevención & control , Adolescente , Ansiedad/etiología , Ansiedad/metabolismo , Biomarcadores/metabolismo , Niño , Femenino , Estudios de Seguimiento , Gastroscopía/psicología , Humanos , Masculino , Pronóstico , Estudios Prospectivos , Estrés Psicológico/complicaciones , Estrés Psicológico/metabolismo
18.
Turk J Gastroenterol ; 29(2): 215-220, 2018 03.
Artículo en Inglés | MEDLINE | ID: mdl-29749330

RESUMEN

BACKGROUND/AIMS: To investigate bone mineral density (BMD) in children with celiac disease (CD) and to evaluate the association between vitamin K levels and osteoporosis. MATERIALS AND METHODS: Children with CD and age- and sex-matched healthy control subjects were prospectively included in the study. BMD was measured, and serum anti-tissue transglutaminase IgA, ferritin, folate, vitamin B12, 25-hydroxy vitamin D and K2, calcium, phosphate, alkaline phosphatase, and parathormone were assayed in all subjects. RESULTS: Overall, 72 patients (mean age 11.69±3 years, 59.7% female) and 30 healthy subjects (mean age 12.27±2.12 years, 63.3% female) were enrolled. The mean BMD Z score of the celiac group was significantly lower than that of the control group (-1.23±1.07 vs. -0.35±1.04, p=0.001). Vitamin D and K2 values did not differ significantly between the two groups (p > 0.05). BMD was positively correlated with vitamin D (r=0.198, p=0.001) and negatively with PTH (r=-0.397, p=0.002). CONCLUSION: The BMD of celiac patients was lower than that of the control subjects. There was no difference in terms of vitamin D and K2 levels between the two groups. Further studies investigating the level and effect of vitamin K on bone in CD are needed.


Asunto(s)
Densidad Ósea/fisiología , Enfermedad Celíaca/fisiopatología , Estado Nutricional/fisiología , Vitamina K 2/sangre , Adolescente , Estudios de Casos y Controles , Enfermedad Celíaca/sangre , Niño , Femenino , Humanos , Masculino , Estudios Prospectivos , Vitamina D/análogos & derivados , Vitamina D/sangre
19.
Turk J Pediatr ; 59(5): 531-536, 2017.
Artículo en Inglés | MEDLINE | ID: mdl-29745114

RESUMEN

Gelatin tannate (GT) is a nonabsorbable antidiarrheal agent investigated in few clinical studies. The aim of this study was to investigate the effects of GT on children with acute gastroenteritis. This randomized, placebo-controlled, single-blinded, prospective study involved children aged from six months to 10 years with acute diarrhea. The study group received GT and the control group placebo for five days. Stool frequency and numbers of patients with diarrhea in each group were compared at 12, 24, 48, 72, 96, and 120 hours. Duration of diarrhea and weight changes after 120 hours was recorded. Mean stool frequency was lower in the study group at 0-12 hours (3±1.8 vs. 3.6±1.9, p=0.04). The study group exhibited more weight gain after 120 hours of treatment and shorter total duration of diarrhea, although the difference was not statistically significant. Fewer patients in the study group had diarrhea at the end of 12, 24, 96, and 120 hours. Patients treated with GT with Bristol scores of 7 at admission exhibited more weight gain than patients with Bristol scores of 6 (296±38 vs. 137±39, p=0.04). GT resulted in a decreased stool frequency at 12 hours in children with acute diarrhea. It shortened total duration of diarrhea and resulted in more weight gain compared to placebo. It also had a greater effect on weight gain in the presence of watery, rather than mushy stool.


Asunto(s)
Antidiarreicos/uso terapéutico , Diarrea/tratamiento farmacológico , Gastroenteritis/tratamiento farmacológico , Gelatina/uso terapéutico , Taninos/uso terapéutico , Enfermedad Aguda , Peso Corporal , Niño , Preescolar , Heces/microbiología , Femenino , Humanos , Lactante , Masculino , Estudios Prospectivos , Método Simple Ciego , Resultado del Tratamiento
20.
Eur J Gastroenterol Hepatol ; 26(11): 1202-8, 2014 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-25171023

RESUMEN

OBJECTIVE: There has been a marked decrease in the eradication rates of Helicobacter pylori infection with standard triple therapy worldwide. Hence, sequential therapy has gained attention as a promising treatment during the last few years. This study was carried out to compare the efficacy of sequential versus standard triple therapy in the context of clarithromycin (CLA) resistance. MATERIALS AND METHODS: In this study, children between 3 and 18 years of age, who had documented H. pylori infection, were randomized to receive either standard triple or sequential therapy. H. pylori eradication was ascertained using the C-urea breath test 4-6 weeks after the completion of the treatment. Real-time PCR was performed on gastric biopsy samples for assessment of CLA resistance. RESULTS: In all, 148 children (median age: 12.18±3.51 years) were recruited randomly into the study. The intention-to-treat eradication rates were 50% (37/74) for the sequential treatment group and 52.7% (39/74) for the standard triple treatment group (P=0.87). A total of 136 children completed the study. The per-protocol eradication rates were 56% (37/66) and 55.7% (39/70) for sequential and standard triple therapy groups, respectively. CLA resistance was assessed and 113 children were included in the final analysis. Of 113 participants, 53 were in the sequential treatment group and 60 were in the standard triple treatment group. The success rates of the respective therapies (29/53=54.7% in sequential, 33/60=55% in standard therapy) were similar (P=0.98). CLA resistance was detected in 29 (25.7%) of the patients. Eradication rates with sequential therapy in CLA susceptible and resistant cases were 60.5% (23/38) and 40% (6/15), respectively (P=0.23). The corresponding figures for the standard triple treatment group were 63% (29/46) and 28.6% (4/14) (P=0.033). Although a higher eradication rate was observed in CLA-resistant cases with sequential therapy, the difference did not reach statistical significance (P=0.69). CONCLUSION: In this study, standard triple treatment failed to eradicate H. pylori infection in the majority of the children, and sequential therapy offered only a small advantage over standard triple therapy in the eradication of CLA-resistant strains.


Asunto(s)
Antibacterianos/administración & dosificación , Claritromicina/administración & dosificación , Infecciones por Helicobacter/tratamiento farmacológico , Helicobacter pylori/efectos de los fármacos , Adolescente , Amoxicilina/administración & dosificación , Amoxicilina/efectos adversos , Amoxicilina/uso terapéutico , Antibacterianos/efectos adversos , Antibacterianos/uso terapéutico , Pruebas Respiratorias/métodos , Niño , Preescolar , Claritromicina/uso terapéutico , Esquema de Medicación , Farmacorresistencia Bacteriana , Quimioterapia Combinada , Femenino , Infecciones por Helicobacter/microbiología , Humanos , Lansoprazol/administración & dosificación , Lansoprazol/efectos adversos , Lansoprazol/uso terapéutico , Masculino , Cumplimiento de la Medicación , Metronidazol/administración & dosificación , Metronidazol/efectos adversos , Metronidazol/uso terapéutico , Resultado del Tratamiento
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