Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 86
Filtrar
Más filtros

País/Región como asunto
Tipo del documento
País de afiliación
Intervalo de año de publicación
1.
Pediatr Transplant ; 20(1): 83-8, 2016 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-26607307

RESUMEN

AKI is an important complication after LT. As our LT series contains a quite high number of children with ALF unlike published studies, we aimed to determine pre-LT and long-term renal functions in children both with ALF and with CLD. Demographic and disease-related data of 134 transplanted children were evaluated retrospectively. Pre-LT and follow-up GFR and pediatric RIFLE scores were determined. Mean pre-LT GFR was not dependent on the disease presentation or severity of chronic disease. While there was an initial decline until first week of post-LT in CLD children, an increase was observed in ALF. Neither mean GFR nor the pRIFLE on follow-up was different with respect to the type of LT or disease presentation. Mean GFR at first and sixth months were lower in children on cyclosporine compared to tacrolimus (p = 0.001 and p = 0.002, respectively). In conclusion, GFR-time curve was different in children with or without ALF. Type of LT, and severity of the CLD were not risk factors for CKD in any time, but younger age at LT, CLD, and cyclosporine usage were at sixth months of follow-up.


Asunto(s)
Lesión Renal Aguda/etiología , Riñón/fisiología , Fallo Hepático/cirugía , Trasplante de Hígado , Adolescente , Inhibidores de la Calcineurina/uso terapéutico , Niño , Preescolar , Ciclosporina/administración & dosificación , Femenino , Tasa de Filtración Glomerular , Humanos , Inmunosupresores/uso terapéutico , Lactante , Fallo Hepático/complicaciones , Masculino , Prevalencia , Estudios Retrospectivos , Factores de Riesgo , Tacrolimus/administración & dosificación , Factores de Tiempo , Resultado del Tratamiento
2.
Korean J Gastroenterol ; 83(1): 17-22, 2024 Jan 25.
Artículo en Inglés | MEDLINE | ID: mdl-38268164

RESUMEN

Background/Aims: Solitary rectal ulcer syndrome (SRUS) can be overlooked, diagnosed late, or misdiagnosed, particularly in childhood. This study reviewed the 13-year experience of the authors' institution to increase clinicians' awareness of SRUS in the presence of symptoms. This paper reports the endoscopic and histopathological findings in children presenting with hematochezia. Methods: The clinical and laboratory findings of 22 patients diagnosed with biopsy-proven SRUS in the authors' clinic between 2007 and 2020 were evaluated retrospectively. Results: The mean age at diagnosis was 12.5±2.6 years, and 59.1% of the patients were male. The median time of diagnosis was 24 months. A single ulcer lesion was found by colonoscopy in 18 patients (81.8%), two ulcers in two patients (9%), and more than two ulcers in two patients (9%). The pathology reports of all biopsies taken from the lesions were consistent with a solitary rectal ulcer. In the first stage, the treatment was started with toilet training, a high-fiber diet, and laxatives. In 11 patients (50%) who did not respond to the initial treatment, a 5-ASA enema was added. A glucocorticoid enema was added to treatment in five patients (22%) whose complaints did not regress despite this treatment. Clinical remission was achieved in five of the patients (18.1%). The time to diagnosis was significantly shorter in those in remission than those not in remission (p=0.04). Conclusions: This study is the first large series on Turkish children. An increased awareness of SRUS in children will increase the rate of early diagnosis and treatment, allowing remission in more patients.


Asunto(s)
Enfermedades del Colon , Enfermedades del Recto , Úlcera , Niño , Femenino , Humanos , Masculino , Colonoscopía , Hemorragia Gastrointestinal/diagnóstico , Hemorragia Gastrointestinal/etiología , Estudios Retrospectivos , Úlcera/diagnóstico , Úlcera/etiología
3.
J Pediatr Gastroenterol Nutr ; 56(2): 211-4, 2013 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-22983377

RESUMEN

OBJECTIVE: Although it is well known that celiac disease (CD) is associated with neurologic disorders, association with psychiatric problems is not well defined. In this report, we aimed to detect CD prevalence in patients with attention-deficit hyperactivity disorder (ADHD). METHODS: A total of 362 patients between the ages 5 and 15 years with the diagnosis of ADHD according to the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, Text Revision (DSM-IV-TR) diagnostic criteria and 390 sex- and age-matched healthy children were included in the present study. Serum levels of tissue transglutaminase (tTg) immunoglobulin (Ig) A and IgG were studied in both groups. Serum IgA levels were also studied in patients with positive tTG IgG for the exclusion of selective IgA deficiency. Endoscopic duodenal biopsy was provided in seropositive patients, whose parents approved the procedure. Biopsy samples were evaluated according to Marsh-Oberhuber classification. RESULTS: tTg IgA was positive in 4 patients with ADHD (1.1%). Endoscopic duodenal biopsy was suggestive of CD in one of them (0.27%). tTg IgA was positive in 3 of control group patients (0.8%). Duodenal biopsy of the only patient from control group, who underwent upper gastrointestinal endoscopy, revealed normal intestinal mucosa. CONCLUSIONS: The seropositivity rates for CD were found similar in ADHD and control groups. Thus, neither routine screening for CD nor empirical recommendation of gluten-free diet seems necessary in children with ADHD.


Asunto(s)
Trastorno por Déficit de Atención con Hiperactividad/complicaciones , Enfermedad Celíaca/complicaciones , Duodeno/patología , Inmunoglobulina A/sangre , Inmunoglobulina G/sangre , Mucosa Intestinal/patología , Transglutaminasas/inmunología , Adolescente , Trastorno por Déficit de Atención con Hiperactividad/inmunología , Trastorno por Déficit de Atención con Hiperactividad/patología , Biopsia , Estudios de Casos y Controles , Enfermedad Celíaca/inmunología , Enfermedad Celíaca/patología , Niño , Endoscopía , Femenino , Humanos , Masculino , Prevalencia
4.
Pediatr Int ; 55(1): 85-9, 2013 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-23039878

RESUMEN

BACKGROUND: Hair follicle mites, Demodex folliculorum and Demodex brevis, are known to accompany immune-deficiency states, however no study so far has investigated their presence in malnutrition. In this study we aimed to determine the prevalence of those mites in childhood malnutrition, malignancy and risk factors. METHODS: One hundred children with malnutrition, 31 children with malignancy and 63 children without any chronic disease and infection were included in this study. History, physical examination, anthropometric measurements and routine laboratory findings were recorded. Demodex spp. were investigated by standard superficial skin biopsies. RESULTS: Demodex was found in 25 patients (25%), 10 patients (32.3%), and one patient (1.6%) among malnutrition, malignancy, and control groups, respectively (P = 0.001). By using multilogistic regression binary method, it was found that malnutrition, malignancy and low socioeconomic level increased the risk 17.37 times (P = 0.006), 27.29 times (P = 0.002), and 2.3 times (P = 0.037), respectively. Of 22 children who were evaluated after 6 months, 13 (59.1%) were negative for Demodex. In 11 (84.6%) of those 13, nutritional status was improved. CONCLUSION: Demodex was detected in approximately in one-quarter and one-third of children with malnutrition and malignancy, respectively. Eliminating the cause of immunosuppression, such as poor nutritional status, seems also to be an effective method for eliminating Demodex.


Asunto(s)
Desnutrición/complicaciones , Infestaciones por Ácaros/etiología , Neoplasias/complicaciones , Adolescente , Estudios de Casos y Controles , Niño , Preescolar , Femenino , Humanos , Lactante , Modelos Logísticos , Masculino , Infestaciones por Ácaros/epidemiología , Prevalencia , Factores de Riesgo , Factores Socioeconómicos
5.
J Ren Nutr ; 23(2): 119-22, 2013 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-22633990

RESUMEN

BACKGROUND: Although it is known that obesity predisposes to urolithiasis, a tendency for malnutrition in children with urolithiasis owing to recurrent urinary infections and abdominal pain also makes sense. AIMS: In this study, we aimed to determine the nutritional status of infants and children with urolithiasis, and to observe whether obesity or malnutrition is more prevalent in that population. METHODS: One hundred eighty-seven children aged 4 months to 17 years (mean, 4.9 ± 4.4 years) with urolithiasis, and 278 age- and sex-matched children without any chronic diseases were included. Anthropometric evaluations, including weight and height standard deviation score (SDS), body mass index, and triceps and subscapular skinfold thickness (SFT), were performed. RESULTS: Mean weight SDSs of the patients was statistically lower than that of the control subjects (P < .0001). Malnutrition rate was statistically higher in the patients with urolithiasis when evaluated according to weight SDS and percentiles of body mass index and SFT. When the age factor was taken into account, the percentage of malnutrition, determined by the percentiles of triceps and subscapular SFT measurements, was found to be higher in children younger than 2 years. Short stature was more prevalent in older children. CONCLUSION: Malnutrition among children with urolithiasis is not as rare as thought previously. A careful anthropometric evaluation should be included in the clinical assessment of those children.


Asunto(s)
Desnutrición/complicaciones , Obesidad/complicaciones , Urolitiasis/complicaciones , Adolescente , Factores de Edad , Antropometría , Composición Corporal , Índice de Masa Corporal , Estudios de Casos y Controles , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Desnutrición/epidemiología , Estado Nutricional , Prevalencia , Urolitiasis/epidemiología
6.
J Clin Res Pediatr Endocrinol ; 15(1): 62-68, 2023 02 27.
Artículo en Inglés | MEDLINE | ID: mdl-36264034

RESUMEN

Objective: It has been reported that bone mineral density (BMD) is decreased in children with Celiac disease (CD) compared to their healthy peers. The aim of this study was to reveal possible risk factors for low BMD in Turkish children newly diagnosed with CD. Methods: Eighty-six patients (2-18 years old) with CD were included in this retrospective study. The relationship between their lumbar BMD z-scores calculated according to their chronological age (CA) and height age (HA) and their clinical, laboratory [biochemical parameters, tissue transglutaminase antibody-IgA (TTGA) levels, human leukocyte antigen (HLA) types] and histopathological parameters were evaluated. Results: The mean age of the patients at diagnosis was 8.06±4.08 years. The BMD z-score CA was ≤-2 standard deviation (SD) in 26.7% of the patients. The BMD z-score HA was ≤-2 SD in 12.8% of the patients. The BMD z-score HA only correlated with their age at diagnosis of CD (rs value 0.269). However, there was no statistically difference between the BMD z-score HA >-2 SD and ≤-2 SD subgroups regarding their clinical, laboratory and histopathological parameters. Conclusion: Low BMD is common in children with newly diagnosed CD. Age at diagnosis, gender, body size, Celiac symptoms, biochemical parameters, TTGA level, HLA type, and histopathological stage had no predictive values in terms of low BMD in this patient group.


Asunto(s)
Enfermedades Óseas Metabólicas , Enfermedad Celíaca , Humanos , Niño , Preescolar , Adolescente , Enfermedad Celíaca/diagnóstico , Estudios Retrospectivos , Absorciometría de Fotón/efectos adversos , Densidad Ósea
7.
Front Pediatr ; 11: 1081139, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36950173

RESUMEN

This review by a panel of pediatric gastroenterology-hepatology-nutrition and pediatric neurology experts aimed to address the significance of mid-upper arm circumference (MUAC) assessment in diagnosis of pediatric malnutrition. Specifically, the potential utility of recently developed MUAC z-score tape in clinical practice for larger patient populations was addressed including the neurologically disabled children. In accordance with the evidence-based data, four statements were identified by the participating experts on the utility of MUAC z-score tape, including (1) MUAC z-scores correlate with body mass index (BMI) and weight for height/length (WFH/l) z-scores in diagnosing malnutrition; (2) MUAC z-score tape offers a higher sensitivity to diagnose the mild and moderate malnutrition and better ability to track the changes in nutritional status over time than the other single datapoint measurements; (3) Using single-step MUAC z-score tape in children with cerebral palsy (CP) seems to provide more reliable data on anthropometry; and (4) The clinical value of the tool in classifying secondary malnutrition in CP should be investigated in large-scale populations. In conclusion, enabling single-step estimation of nutritional status in a large-scale pediatric population regardless of age and within a wide range of weight, without formal training or the need for ancillary reference charts and calculators, MUAC z-tape offers a favorable tool for easier and earlier diagnosis of pediatric malnutrition. Nonetheless, further implementation of MUAC z-score screening in larger-scale and/or special populations is necessary to justify its utility in relation to other primary anthropometric indicators in diagnosis of malnutrition as well as in treatment monitoring in the community and hospital setting.

8.
Front Pediatr ; 11: 1272177, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37818166

RESUMEN

Introduction: Elevated transaminases and/or creatine phosphokinase can indicate underlying muscle disease. Therefore, this study aims to determine the frequency of Duchenne muscular dystrophy/Becker muscular dystrophy (DMD/BMD) in male children and Pompe disease (PD) in male and female children with isolated hypertransaminasemia. Methods: This multi-center, prospective study enrolled patients aged 3-216 months with serum alanine transaminase (ALT) and/or aspartate transaminase (AST) levels >2× the upper limit of normal (ULN) for ≥3 months. Patients with a known history of liver or muscle disease or physical examination findings suggestive of liver disease were excluded. Patients were screened for creatinine phosphokinase (CPK) levels, and molecular genetic tests for DMD/BMD in male patients and enzyme analysis for PD in male and female patients with elevated CPK levels were performed. Genetic analyses confirmed PD. Demographic, clinical, and laboratory characteristics of the patients were analyzed. Results: Overall, 589 patients [66.8% male, mean age of 63.4 months (standard deviation: 60.5)] were included. In total, 251 patients (188 male and 63 female) had CPK levels above the ULN. Of the patients assessed, 47% (85/182) of male patients were diagnosed with DMD/BMD and 1% (3/228) of male and female patients were diagnosed with PD. The median ALT, AST, and CPK levels were statistically significantly higher, and the questioned neurological symptoms and previously unnoticed examination findings were more common in DMD/BMD patients than those without DMD/BMD or PD (p < 0.001). Discussion: Questioning neurological symptoms, conducting a complete physical examination, and testing for CPK levels in patients with isolated hypertransaminasemia will prevent costly and time-consuming investigations for liver diseases and will lead to the diagnosis of occult neuromuscular diseases. Trial Registration: Clinicaltrials.gov NCT04120168.

9.
Pediatr Crit Care Med ; 13(4): 452-4, 2012 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-22079952

RESUMEN

BACKGROUND: Early detection of necrotizing enterocolitis can improve the prognosis, however, there is not a reliable laboratory test to detect either newborns at risk for necrotizing enterocolitis development or those at early stages of the disease. Since fecal lactoferrin and fecal calprotectin are inflammatory markers of gastrointestinal diseases, it was hypothesized that both these biomarkers could be successfully used in the diagnosis of necrotizing enterocolitis. METHODS: In a prospective study, fecal lactoferrin and fecal calprotectin concentrations of 14 newborns with necrotizing enterocolitis and consecutively admitted 40 healthy preterm, and 23 healthy full-term newborns were measured with enzyme-linked immunosorbent assay technique. RESULTS: Mean fecal lactoferrin and fecal calprotectin were not different between preterm and full-term newborns (p = .235 and p = .845, respectively), or those who were diagnosed with necrotizing enterocolitis or not (p = .545 and p = .968, respectively). Prevalence of necrotizing enterocolitis was 1.51% (14 of 2734). Stage of the disease did not have a statistical effect on mean levels (p = .694 and p = .267, respectively). Mean fecal lactoferrin and fecal calprotectin levels were not different in the case of breastfeeding (p = .623 and p = .792, respectively). CONCLUSION: Neither fecal lactoferrin nor fecal calprotectin has a role in the identification of necrotizing enterocolitis, especially in early stages of the disease. Further studies on wider necrotizing enterocolitis series are needed for a more definite conclusion.


Asunto(s)
Enterocolitis Necrotizante/diagnóstico , Heces/química , Lactoferrina/análisis , Complejo de Antígeno L1 de Leucocito/análisis , Biomarcadores/análisis , Ensayo de Inmunoadsorción Enzimática , Humanos , Recién Nacido , Recien Nacido Prematuro , Estudios Prospectivos
10.
Turk J Pediatr ; 54(4): 382-6, 2012.
Artículo en Inglés | MEDLINE | ID: mdl-23692719

RESUMEN

There are a few studies suggesting a relationship between celiac disease (CD) and kidney disease, but no study has investigated CD in patients with urolithiasis. In this study, we aimed to determine the prevalence of CD in infants and children with urolithiasis. One hundred and eighty-seven infants and children (4 months-17 years) with urolithiasis, and 278 age- and sex-matched healthy children were included. CD was screened using tissue transglutaminase (tTG) immunoglobulin (Ig)A. Seropositive cases, whose parents gave consent, underwent upper gastrointestinal system endoscopy for duodenal biopsy. Seven (3.7%) among those with urolithiasis and one (0.3%) among controls were positive for tTG IgA (p=0.008). Six of the urolithiasis group and one from the control group underwent upper gastrointestinal endoscopy. Intestinal biopsy revealed Marsh-Oberhuber type 1 intestinal lesions in two children. The other five had normal histology. Biopsy-proven CD was detected in two (1%) children with urolithiasis. The prevalence of biopsy-proven CD among all cases was 0.4%. When children were evaluated with respect to age factor, it was found that seropositivity in children younger and older than two years was not different (4% vs. 3.6%; p=0.880). In this first study investigating CD prevalence in children with urolithiasis, we found a higher seropositivity for CD in children with urolithiasis compared to controls, but in terms of biopsy-proven CD, no difference was found.


Asunto(s)
Enfermedad Celíaca/epidemiología , Urolitiasis/epidemiología , Adolescente , Biopsia , Estudios de Casos y Controles , Distribución de Chi-Cuadrado , Niño , Preescolar , Endoscopía Gastrointestinal , Femenino , Humanos , Lactante , Masculino , Prevalencia , Estadísticas no Paramétricas , Turquía/epidemiología
11.
J Clin Res Pediatr Endocrinol ; 14(4): 409-414, 2022 12 01.
Artículo en Inglés | MEDLINE | ID: mdl-35770950

RESUMEN

Objective: A close relationship has been suggested between Celiac disease (CD) and glandular autoimmunity. The aim of this study was to determine the predictive factors for autoimmune glandular disease (AGD) in children with CD. Methods: The study included 228 pediatric patients, diagnosed with CD between 2010 and 2019. The cases with AGD (Group 1) and those without AGD (Group 2) and the patients with type 1 diabetes mellitus (T1DM) (Group A) and those without T1DM (Group B) were retrospectively reviewed and compared in terms of clinical and laboratory features. Results: AGD was detected in 8.8% (n=20) of the patients: T1DM in 13 (65%), T1DM and Hashimoto's thyroiditis (HT) in 3 (15%), HT only in 2 (10%), T1DM and Graves disease (GD) in 1 (5%), and GD only in 1(5%). The mean age at the diagnosis of CD was significantly higher in Group 1 (10.93±4.15 years) compared to Group 2 (8.10±4.19 years) (p<0.05) and also was significantly higher in Group A compared to Group B (p<0.05). Most of the diagnoses of AGD were made before the diagnosis of CD and age was an effective factor. There was no difference between Group 1 and Group 2 and Group A and Group B in terms of gender, typical/atypical CD ratio, tissue transglutaminase IgA (TTGA) level, human leucocyte antigen (HLA)-DQ2 and/or HLA-DQ8 positivity rate, and histopathological stage. Conclusion: Although patients with a diagnosis of co-existent CD and AGD were significantly older than patients with isolated CD, gender, celiac symptoms, TTGA level, HLA type, and histopathological stage had no predictive value for the coexistence of AGD in patients with CD.


Asunto(s)
Enfermedad Celíaca , Diabetes Mellitus Tipo 1 , Enfermedad de Graves , Enfermedad de Hashimoto , Niño , Humanos , Enfermedad Celíaca/complicaciones , Enfermedad Celíaca/diagnóstico , Enfermedad Celíaca/epidemiología , Diabetes Mellitus Tipo 1/complicaciones , Diabetes Mellitus Tipo 1/diagnóstico , Estudios Retrospectivos , Autoinmunidad , Enfermedad de Hashimoto/complicaciones
12.
J Clin Res Pediatr Endocrinol ; 14(1): 1-9, 2022 03 03.
Artículo en Inglés | MEDLINE | ID: mdl-34538045

RESUMEN

It is well-known that in children with type 1 diabetes (T1D), the frequency of Celiac disease (CD) is increased due to mechanisms which are not fully elucidated but include autoimmune injury as well as shared genetic predisposition. Although histopathologic examination is the gold standard for diagnosis, avoiding unnecessary endoscopy is crucial. Therefore, for both clinicians and patients' families, the diagnosis of CD remains challenging. In light of this, a joint working group, the Type 1 Diabetes and Celiac Disease Joint Working Group, was convened, with the aim of reporting institutional data and reviewing current international guidelines, in order to provide a framework for clinicians. Several controversial issues were discussed: For CD screening in children with T1D, regardless of age, it is recommended to measure tissue transglutaminase-immunoglobulin A (tTG-IgA) and/or endomysial-IgA antibody due to their high sensitivity and specificity. However, the decision-making process based on tTG-IgA titer in children with T1D is still debated, since tTG-IgA titers may fluctuate in children with T1D. Moreover, seronegativity may occur spontaneously. The authors' own data showed that most of the cases who have biopsy-proven CD had tTG-IgA levels 7-10 times above the upper limit. The decision for endoscopy based solely on tTG-IgA levels should be avoided, except in cases where tTG-IgA levels are seven times and above the upper limit. A closer collaboration should be built between divisions of pediatric endocrinology and gastroenterology in terms of screening, diagnosis and follow-up of children with T1D and suspicious CD.


Asunto(s)
Enfermedad Celíaca , Diabetes Mellitus Tipo 1 , Autoanticuerpos , Enfermedad Celíaca/complicaciones , Enfermedad Celíaca/diagnóstico , Niño , Toma de Decisiones Clínicas , Diabetes Mellitus Tipo 1/complicaciones , Diabetes Mellitus Tipo 1/diagnóstico , Humanos , Inmunoglobulina A , Transglutaminasas
13.
J Obstet Gynaecol Res ; 37(11): 1615-9, 2011 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-21733039

RESUMEN

AIM: Celiac disease (CD) may present with atypical symptoms, including poor pregnancy outcomes, such as preterm and low birthweight (LBW) deliveries, thus we aimed to investigate the frequency of CD in mothers and fathers of preterm or LBW newborns. MATERIALS AND METHODS: In this study, 316 parents of 164 preterm or LBW newborns and 246 parents of 123 healthy newborns were included. CD was screened using tissue transglutaminase immunoglobulin A. Endoscopic duodenal biopsy was provided in the seropositive cases. RESULTS: Positive tissue transglutaminase immunoglobulin A was found in six (1.1%; 1/94) individuals (three mothers and three fathers); five were from the study group (1.6%; 1/63) and one was from the control group (0.4%; 1/246). CD prevalence in mothers, fathers and parents of preterm newborns was 1/57 (1.8%), 1/57 (1.8%) and 1/29 (3.5%), respectively. In the LBW group, seropositivity in fathers was 1/50 (2%) with no seropositive mothers. Biopsy-proven CD was found in 1/159 mothers (0.6%) and 1/79 fathers (1.3%). Mean birthweights of the newborns of seropositive mothers and fathers were 214 g (P < 0.05) and 320 g lower than those of seronegative ones, respectively. However, in logistic regression analysis it was found that seropositivity of mothers or fathers did not affect gestational age or birthweight of the newborns. CONCLUSION: Because the prevalence of CD in parents of preterm or LBW newborns is not statistically higher than the healthy population, routine CD screening in that group cannot be recommended at the time being. For more definite conclusions further studies are needed.


Asunto(s)
Enfermedad Celíaca/epidemiología , Enfermedades del Prematuro/epidemiología , Adulto , Femenino , Edad Gestacional , Humanos , Recién Nacido de Bajo Peso , Recién Nacido , Recien Nacido Prematuro , Masculino , Padres , Embarazo , Resultado del Embarazo , Prevalencia
14.
Turk J Pediatr ; 53(3): 241-9, 2011.
Artículo en Inglés | MEDLINE | ID: mdl-21980803

RESUMEN

Celiac disease (CD) has a wide variety of clinical presentations; together with the "classical form", in which the intestinal symptomatology is prevalent, there are "atypical forms" with predominating extra-intestinal clinical features, and the "silent form", with no clinical symptom. The "atypical forms" of the disease are characterized by few or no gastrointestinal symptoms, and predominating extra-intestinal features such as neurologic, dermatologic, hematologic, endocrinologic, reproductive, renal, psychiatric, skeletal, and liver involvement(s). Silent presentation of CD may be identified through screening of high-risk groups. Today, it is well known that CD might account for several chronic health issues, so it is essential for healthcare professionals to have a high level of suspicion for the atypical presentations of CD.


Asunto(s)
Enfermedad Celíaca/diagnóstico , Enfermedad Celíaca/complicaciones , Humanos
15.
Arab J Gastroenterol ; 22(4): 310-315, 2021 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-34840097

RESUMEN

BACKGROUND AND STUDY AIMS: Progressive familial intrahepatic cholestasis (PFIC) is an autosomal recessively inherited disease that causes intrahepatic-hepatocellular cholestasis. PFIC constitutes approximately 10-15% of cholestatic liver diseases in children. The aim of this study is to draw attention to this group of diseases, which pose a higher risk, in societies where consanguineous marriage is more common, and to share our experiences since the studies in the literature, regarding this group of diseases are case series with small number of patients. PATIENTS AND METHODS: This cross-sectional study was conducted on 34 patients who were admitted with jaundice and diagnosed by genetic analysis, between January 2015 and July 2020. RESULTS: We found 17.6% of patients with PFIC type 1, 55.9% patients had PFIC type 2, 14.7% patients had PFIC type 3, 8.8% patients had PFIC type 4 and 2.9% patients had PFIC type 5. Partial internal biliary diversion was performed in 5 (14.7%) patients, who had severe itching during follow-up, did not respond to medical treatment, and did not have significant fibrosis in liver biopsy yet. The degree of itching before PIBD was rated as +4 (cutaneous erosion, bleeding and scarring), in 5 patients and the rates were 0 (absent) in two patients, and +1 (mild itching) in 3 patients, 6 months after PIBD, these differences were statistically significant(p = 0.027). The mean weight z score was-1.43 (-3.72-+0.73), before PIBD, while it was 0.39(-1.86 -+2.45), six months after PIBD; the diference was statistically significant(p = 0.043). Liver transplantation was performed in 12 (35.3%) patients with significant fibrosis in liver biopsy and developing signs of portal hypertension. CONCLUSION: The PFIC disease group is a heterogeneous disease group that is difficult to diagnose and treat. It should be considered in patients with cholestasis and/or pruritus and those with a history of consanguineous marriage between parents and death of a sibling with similar clinical symptoms.


Asunto(s)
Procedimientos Quirúrgicos del Sistema Biliar , Colestasis Intrahepática , Colestasis , Niño , Colestasis Intrahepática/diagnóstico , Colestasis Intrahepática/genética , Colestasis Intrahepática/terapia , Estudios Transversales , Humanos
16.
Front Pediatr ; 9: 610275, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34164352

RESUMEN

This review focuses on nutritional support in malnourished children with compromised gastrointestinal function addressing the interplay between malnutrition and gastrointestinal dysfunction, and the specific role of peptide-based enteral therapy in pediatric malnutrition. Malnutrition is associated with impaired gut functions such as increased intestinal permeability, malabsorption, and diarrhea, while pre-existing functional gastrointestinal disorders may also lead to malnutrition. Presence of compromised gastrointestinal function in malnourished children is critical given that alterations such as malabsorption and increased intestinal permeability directly interfere with efficacy of nutritional support and recovery from malnutrition. Appropriate nutritional intervention is the key step in the management of malnutrition, while alterations in gastrointestinal functions in malnourished children are likely even in those with mild degree malnutrition. Therefore, nutritional therapy in children with compromised gastrointestinal function is considered to involve gut-protective interventions that address the overlapping and interacting effects of diarrhea, enteropathy and malnutrition to improve child survival and developmental potential in the long-term. Peptide-based enteral formulas seem to have clinical applications in malnourished children with compromised gastrointestinal function, given their association with improved gastrointestinal tolerance and absorption, better nitrogen retention/ balance, reduced diarrhea and bacterial translocation, enhanced fat absorption, and maintained/restored gut integrity as compared with free amino acid or whole-protein formulas.

17.
J Clin Gastroenterol ; 44(1): 4-8, 2010 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-20027009

RESUMEN

Celiac disease is a common multisystemic disorder that may be diagnosed at any age. Owing to the increasing prevalence, prevention strategies, including encouragement of breastfeeding and introduction of small amounts of gluten whereas still on breastfeeding at fourth to sixth month of age, become more important than before. For avoiding complications of celiac disease, a strict gluten-free diet is essential. A lifelong gluten-free diet may bring about difficulties as avoiding gluten completely is problematic owing to the contamination with gluten of presumably gluten free foods. New therapeutic approaches include enzyme supplementation, correction of the intestinal barrier defect against gluten entry, blocking of gliadin presentation by human leukocyte antigen blockers and tissue transglutaminase inhibitors.


Asunto(s)
Enfermedad Celíaca/terapia , Dieta Sin Gluten , Glútenes/administración & dosificación , Animales , Lactancia Materna , Enfermedad Celíaca/inmunología , Enfermedad Celíaca/prevención & control , Terapia Enzimática , Gliadina/inmunología , Glútenes/inmunología , Antígenos HLA/inmunología , Humanos , Lactante , Mucosa Intestinal/metabolismo , Transglutaminasas/antagonistas & inhibidores
18.
J Clin Gastroenterol ; 44(8): 544-6, 2010 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-20054281

RESUMEN

GOALS: We aimed to determine fecal calprotectin (FC) concentration and its relation with histopathologic findings of children with celiac disease (CD) and to observe the probable alterations under gluten-free diet (GFD). BACKGROUND: As FC is regarded as a marker of inflammation in the gastrointestinal tract, we hypothesized that it might be increased in untreated CD. STUDY: The study included 29 newly diagnosed patients with CD (mean age: 6.6+/-0.6 y) and sex and age-matched 10 healthy children. All of the children with CD admitted to the hospital were classical form who has chronic diarrhea and failure to thrive. The degree of mucosal damage was graded according to the modified Marsh criteria. FC concentration was determined by enzyme-linked immunosorbent assay method on admission and after 1 year of GFD. RESULTS: Mean FC concentration of children with CD on admission and of healthy children were 13.40+/-8.5 and 4.3+/-3.3 mg/L, respectively (P=0.004). FC concentration under GFD was 4.6+/-2.7 mg/L and there was a significant statistical difference between untreated patients and those under GFD for 1 year (P=0.001). There was no statistical difference between FC concentration of those under GFD and healthy children (P=0.8). Mean FC concentrations of children with total-villous atrophy and partial-villous atrophy were significantly different (13.8+/-9.3 mg/L vs. 3.7+/-1.8 mg/L, P=0.005). CONCLUSIONS: It was found that FC concentration is increased in childhood CD, related to the severity of histopathologic findings and responsive to GFD. The pathogenetic mechanism by which FC is increased in CD should be investigated in further studies.


Asunto(s)
Enfermedad Celíaca/fisiopatología , Dieta Sin Gluten , Heces/química , Complejo de Antígeno L1 de Leucocito/metabolismo , Adolescente , Biomarcadores/metabolismo , Estudios de Casos y Controles , Enfermedad Celíaca/dietoterapia , Niño , Preescolar , Ensayo de Inmunoadsorción Enzimática , Femenino , Estudios de Seguimiento , Humanos , Inflamación/etiología , Inflamación/fisiopatología , Masculino , Índice de Severidad de la Enfermedad
19.
Scand J Gastroenterol ; 45(4): 395-402, 2010 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-20017709

RESUMEN

Reproductive problems, such as delayed menarche, amenorrhea, early menopause, infertility, impotence, hypogonadism, recurrent abortions, and low-birth-weight or preterm deliveries, are now known to be among the atypical symptoms of coeliac disease (CD). The pathogenesis of reproductive disorders in CD is unclear, but some hypotheses have been suggested, including autoimmunity and macro- and/or micronutrient deficiency. Recent investigations which have focused on tissue transglutaminase are promising with respect to the clarification of the mechanism of infertility and poor pregnancy outcomes in CD. In this review, the effects of CD on male and female reproductive disorders and pregnancy outcomes are discussed and the need for CD screening in the case of reproductive problems is emphasized.


Asunto(s)
Enfermedad Celíaca/complicaciones , Infertilidad Femenina/etiología , Infertilidad Masculina/etiología , Femenino , Humanos , Masculino , Embarazo , Complicaciones del Embarazo/etiología , Resultado del Embarazo , Factores de Riesgo
20.
Turk J Pediatr ; 52(4): 360-6, 2010.
Artículo en Inglés | MEDLINE | ID: mdl-21043380

RESUMEN

In this retrospective study, we aimed to share our experience with different treatment modalities for chronic hepatitis B in a series of children. The study included 126 children (mean: 9.5 +/- 3.8 years). Normalization of alanine aminotransferase (ALT), loss of hepatitis B virus (HBV)-DNA and hepatitis B e antigen (HBeAg), and development of antibody to HBeAg (anti-HBe) altogether at the end of the treatment was considered as end of therapy response (ETR). Seroconversion ongoing one year after the cessation of therapy was considered as sustained response. Of the total children, 90 (71.4%) were treated, whereas the remaining were just followed-up. High-dose interferon (IFN)-alpha (10 MU/m2) alone, standard-dose IFN-alpha (6 MU/m2) plus lamivudine (4 mg/kg/d), high-dose IFN-alpha plus lamivudine, or lamivudine alone was used, IFN-alpha thrice weekly for six months, and lamivudine daily for one year. Of children who had completed their treatment, 34 (37.8%) achieved ETR. Sustained response rate was 36.7%. Response rates were different in the different treatment groups (p: 0.01). The highest response rate was observed in those who received standard-dose IFN-alpha plus lamivudine treatment (61.5%). Of children without treatment, one (2.8%) had anti-HBe seroconversion. Standard-dose IFN-alpha plus lamivudine treatment was found superior to the other treatment modalities. Predictors of ETR were similar to those found in previous studies.


Asunto(s)
Antivirales/uso terapéutico , Hepatitis B Crónica/tratamiento farmacológico , Interferón-alfa/uso terapéutico , Lamivudine/uso terapéutico , Adolescente , Análisis de Varianza , Distribución de Chi-Cuadrado , Niño , Preescolar , Quimioterapia Combinada , Femenino , Humanos , Lactante , Pruebas de Función Hepática , Masculino , Estudios Retrospectivos , Estadísticas no Paramétricas , Resultado del Tratamiento
SELECCIÓN DE REFERENCIAS
DETALLE DE LA BÚSQUEDA