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1.
J Pediatr Gastroenterol Nutr ; 78(2): 197-203, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38374549

RESUMO

OBJECTIVES: The aim of this study was to examine the clinical features of acute terminal ileitis in children and evaluate its rate before and during the COVID-19 pandemic. METHODS: This retrospective study was performed in our pediatric emergency department between 2018 and 2022. The records of 5363 patients who required abdominal imaging due to acute abdomen were analyzed, and 143 patients with terminal ileitis were included. The rate and etiological causes were compared during and before the COVID-19 pandemic. RESULTS: The rate of acute terminal ileitis has increased over the years. The fastest increase was in 2021, when the COVID-19 pandemic was experienced. While 59 (41.2%) patients showed acute nonspecific ileitis, the most common etiologic cause that could be identified was acute gastroenteritis. It was determined that multisystem inflammatory syndrome in children was among the causes of ileitis after the COVID-19 pandemic and was one of the top three causes. CONCLUSIONS: Acute terminal ileitis, which has many etiologies, is one of the rare radiological findings in acute abdominal pain. Examination and laboratory findings are not specific. Guidelines are needed for the investigation of the underlying etiology of acute terminal ileitis in children. The incidence of acute terminal ileitis is increasing, and the increase has been found to be faster after the COVID-19 pandemic.


Assuntos
Abdome Agudo , COVID-19 , COVID-19/complicações , Doença de Crohn , Ileíte , Síndrome de Resposta Inflamatória Sistêmica , Criança , Humanos , Estudos Retrospectivos , Pandemias , COVID-19/epidemiologia , Ileíte/diagnóstico , Ileíte/epidemiologia
2.
Turk J Med Sci ; 54(1): 291-300, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38812630

RESUMO

Background/aim: Congenital anomalies of the kidney and urinary tract(CAKUT) are the leading causes of childhood chronic kidney disease (CKD). The etiology of most of the cases is thought to be multifactorial. In this study, risk factors for CAKUT and the effect of mobile phone-related electromagnetic field (EMF) exposure during pregnancy were investigated. Materials and methods: Fifty-seven cases and 57 healthy controls under 2 years of age were included and their mothers were subjected to a questionnaire. Groups were compared for parents' demographics, pregestational (chronic disease, body mass index, use of the folic acid supplements) and antenatal variables (gestational disease, weight gain during pregnancy,) and exposures during pregnancy. To assess mobile phone-related radiation exposure, all participants were asked about their daily call time, the proximity of the phone when not in use, and the models of their mobile phones. The specific absorption rate (SAR) of the mobile phones and the effective SAR value (SAR × call time) as an indicator of EMF exposure were recorded. Results: Excess weight gain according to BMI during pregnancy was related to an increased risk of CAKUT (p=0.012). Folic acid use before pregnancy was protective for CAKUT (p = 0.028). The call time of mothers of the CAKUT group was significantly longer than the control (p = 0.001). An association was observed between higher effective SAR values and increased risk of CAKUT (p = 0.03). However the proximity of the mobile phone to the mother's body when not in use was not found as a risk factor. Conclusion: The etiology of CAKUT is multifactorial. Our results suggest that prolonged phone call and higher EMF exposure during pregnancy increases the risk of CAKUT in the offspring.


Assuntos
Telefone Celular , Campos Eletromagnéticos , Humanos , Feminino , Gravidez , Fatores de Risco , Campos Eletromagnéticos/efeitos adversos , Adulto , Estudos de Casos e Controles , Anormalidades Urogenitais/epidemiologia , Anormalidades Urogenitais/etiologia , Exposição Materna/efeitos adversos , Efeitos Tardios da Exposição Pré-Natal/epidemiologia , Refluxo Vesicoureteral
3.
Eur J Pediatr ; 181(4): 1481-1486, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-34993623

RESUMO

In this study, our purpose is to evaluate cochlear and vestibular function in juveniles with IgA vasculitis using audiometry, distortion product otoacoustic emissions, and cervical vestibular evoked myogenic potential (cVEMP) tests. Forty children diagnosed with IgA vasculitis from the pediatry clinic and 40 age- and sex-matched healthy children were evaluated with distortion product otoacoustic emissions, audiometry, and cVEMP test in a tertiary hospital. The audiometry average values for both ears of the IgA vasculitis group and the control subjects were compared, and as a result, median 4.7-dB sensorineural hearing loss (SHL) was found for the IgA vasculitis group compared to the control group at 250 Hz and it was statistically significant (p < 0.001). An average of 6.4-dB SHL was detected at 8000 Hz (p < 0.001). There was a statistically significant difference among IgA vasculitis and control groups regarding measurement results of average p1-n1 latency time of both ears (0.9 ms (ms) increase, p = 0.035). In IgA vasculitis patients, the median amplitude difference of both ears' average p1 n1 was found to be 5.6 mV, statistically significantly decreased compared to the control group (p = 0.003). CONCLUSION: This study, firstly in literature, demonstrated that IgA vasculitis may have association with hearing loss and vestibular dysfunction in children. We think this might be due to autoimmune mechanisms. WHAT IS KNOWN: • Ig A vasculitis is a leukocytoclastic vasculitis with unknown etiology, involving the skin, joints, gastrointestinal system, kidneys, and rarely other organs. • No study has been reported for the cochlear and vestibular association of Ig A vasculitis in current literature. WHAT IS NEW: • This study demonstrated that Ig A vasculitis may have association with hearing loss in children. • This study also demonstrated that Ig A vasculitis may have association with vestibular dysfunction in children.


Assuntos
Perda Auditiva Neurossensorial , Vasculite por IgA , Potenciais Evocados Miogênicos Vestibulares , Audiometria , Estudos de Casos e Controles , Criança , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/etiologia , Humanos , Vasculite por IgA/complicações , Vasculite por IgA/diagnóstico , Potenciais Evocados Miogênicos Vestibulares/fisiologia
4.
J Pak Med Assoc ; 69(5): 647-654, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31926114

RESUMO

OBJECTIVE: To investigate whether some ratios obtained from complete blood count could be favourable predictors in differentiating appendicitis from mesenteric lymphadenitis, appendicitis and familial Mediterranean fever. METHODS: The retrospective cross-sectional study was conducted at AfyonKocatepe University Medical Faculty, Afyonkarahisar, Turkey, from January 1, 2014, to December 31, 2016, and comprised data of patients who presented to the department of paediatric surgery and paediatrics with symptoms of acute abdomen. Mean platelet volume, neutrophil/lymphocyte ratio, lymphocyte/monocyte ratio, and platelet/lymphocyte ratio of the patients calculated at the time of admission were analysed. SPSS 20 was used for data analysis. RESULTS: Of the 301 patients, there were 139(46.2%) males and 162(53.8%) were females. The overall mean age was 11.5 } 4.33 years. Of the total, 137(45%) had appendicitis, 65(22%) familial Mediterranean fever, and 99(33%) had mesenteric lymphadenitis. Mean platelet volume was significantly different among the study groups (p<0.05). Neutrophil/lymphocyte ratio and platelet/lymphocyte ratio were statistically significantly higher (p<0.05) while lymphocyte/monocyte ratio was significantly lower (p<0.05) in appendicitis cases compared to the others. CONCLUSIONS: Mean platelet volume, neutrophil/lymphocyte ratio and platelet/lymphocyte ratio were found to be the potent predictors for the differential diagnosis of familial Mediterranean fever and mesenteric lymphadenitis which are mostly confused with appendicitis.


Assuntos
Apendicite/sangue , Apendicite/diagnóstico , Adolescente , Biomarcadores/sangue , Criança , Pré-Escolar , Estudos Transversais , Diagnóstico Diferencial , Febre Familiar do Mediterrâneo/sangue , Febre Familiar do Mediterrâneo/diagnóstico , Feminino , Humanos , Contagem de Leucócitos , Masculino , Volume Plaquetário Médio , Linfadenite Mesentérica/sangue , Linfadenite Mesentérica/diagnóstico , Contagem de Plaquetas , Estudos Retrospectivos , Sensibilidade e Especificidade
5.
Pol J Radiol ; 81: 86-9, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-26985245

RESUMO

BACKGROUND: Sandhoff disease is an autosomal recessive disorder caused by ß-hexosaminidase deficiency in which the ganglioside GM2 and other glycolipids accumulate intracellularly within lysosomes. This process results in progressive motor neuron manifestations, death from respiratory failure and infections in infantiles. CASE REPORT: This report presents a 22-month-old girl with infantile type Sandhoff disease that was hospitalized for generalized seizures and psychomotor retardation. She was diagnosed with a genetically proven novel mutation and by demonstrating it's specific imaging findings. CONCLUSIONS: Determination of spesific changes in neuroimaging which are initial findings for GM2 gangliosidosis is important from the point of diagnosis and follow-up in infants suspected of having a neurodegenerative disease.

6.
Am J Perinatol ; 30(3): 215-8, 2013 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-22875658

RESUMO

OBJECTIVE: Fetal calprotectin levels increase in the early stages of necrotizing enterocolitis. Although the effects of several factors on fetal calprotectin have been studied, the effect of phototherapy is not known. In this study, we analyzed the effect of phototherapy on fetal calprotectin levels. METHODS: Ninety breast-fed newborns (46 male, 44 female) who were hospitalized for indirect hyperbilirubinemia and treated with phototherapy were included to the study. Forty-two of them were term and 44 of them were preterm. Newborns treated with phototherapy (n = 53) constituted the phototherapy group (29 preterm, 24 term) and 37 newborns who did not receive phototherapy (19 preterm, 18 term) constituted the control group. Fecal samples were collected 24 hours after phototherapy had been started. Fecal samples (100 mg) were weighed with sensitive scales and preserved at -80°C after buffering with a special solution. All samples were studied at the same time with a fecal calprotectin kit by using enzyme-linked immunosorbent assay. RESULTS: There were no statistically significant difference between fecal calprotectin levels of term and preterm babies who received phototherapy and babies who did not receive phototherapy. CONCLUSION: There was no effect of 24-hour phototherapy on fecal calprotectin levels in preterm and term newborns.


Assuntos
Fezes/química , Hiperbilirrubinemia Neonatal/metabolismo , Hiperbilirrubinemia Neonatal/terapia , Complexo Antígeno L1 Leucocitário/metabolismo , Fototerapia , Estudos de Casos e Controles , Feminino , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Masculino
7.
Rev Paul Pediatr ; 42: e2023016, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37937678

RESUMO

OBJECTIVE: To examine the neutrophil-lymphocyte ratio, red cell distribution width and mean platelet volume in patients with febrile seizure and to determine their role in febrile seizure classification. METHODS: This was a retrospective hospital-based study conducted among patients aged 5 to 72 months admitted with febrile seizure. Children who had febrile seizures due to upper respiratory tract infection were included in the study. The children were divided into two groups: simple febrile seizures and complex febrile seizures. Patients with a history of febrile status epilepticus, previous convulsions, use of antiepileptic or other chronic drugs, foci of infection other than the upper respiratory tract infection, abnormal biochemical parameters, and chronic mental or physical disease were excluded from the study. Clinical and laboratory findings of the patients were obtained from digital medical records. RESULTS: The records of 112 febrile seizure patients were reviewed, and 89 were grouped as simple and 23 as complex febrile seizures. Although there was no statistically significant difference between the two groups in terms of the mean red cell distribution width values (p=0.703), neutrophil-lymphocyte ratio and mean platelet volume were significantly higher in patients with complex febrile seizures (p=0.034, p=0.037; respectively). CONCLUSIONS: This study showed that neutrophil-lymphocyte ratio and mean platelet volume could be practical and inexpensive clinical markers for febrile seizure classification. A similar result could not be reached for red cell distribution width in this study. These findings should be supported by multicenter studies with large samples.


Assuntos
Infecções Respiratórias , Convulsões Febris , Criança , Humanos , Volume Plaquetário Médio , Índices de Eritrócitos , Neutrófilos , Convulsões Febris/diagnóstico , Estudos Retrospectivos , Linfócitos
8.
Front Pediatr ; 11: 1230905, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37601126

RESUMO

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.

9.
Front Pediatr ; 11: 1179721, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37601138

RESUMO

Introduction: Malnutrition is defined as a pathological condition arising from deficient or imbalanced intake of nutritional elements. Factors such as increasing metabolic demands during the disease course in the hospitalized patients and inadequate calorie intake increase the risk of malnutrition. The aim of the present study is to evaluate nutritional status of patients admitted to pediatric intensive care units (PICU) in Turkey, examine the effect of nutrition on the treatment process and draw attention to the need for regulating nutritional support of patients while continuing existing therapies. Material and Method: In this prospective multicenter study, the data was collected over a period of one month from PICUs participating in the PICU Nutrition Study Group in Turkey. Anthropometric data of the patients, calorie intake, 90-day mortality, need for mechanical ventilation, length of hospital stay and length of stay in intensive care unit were recorded and the relationship between these parameters was examined. Results: Of the 614 patients included in the study, malnutrition was detected in 45.4% of the patients. Enteral feeding was initiated in 40.6% (n = 249) of the patients at day one upon admission to the intensive care unit. In the first 48 h, 86.82% (n = 533) of the patients achieved the target calorie intake, and 81.65% (n = 307) of the 376 patients remaining in the intensive care unit achieved the target calorie intake at the end of one week. The risk of mortality decreased with increasing upper mid-arm circumference and triceps skin fold thickness Z-score (OR = 0.871/0.894; p = 0.027/0.024). The risk of mortality was 2.723 times higher in patients who did not achieve the target calorie intake at first 48 h (p = 0.006) and the risk was 3.829 times higher in patients who did not achieve the target calorie intake at the end of one week (p = 0.001). The risk of mortality decreased with increasing triceps skin fold thickness Z-score (OR = 0.894; p = 0.024). Conclusion: Timely and appropriate nutritional support in critically ill patients favorably affects the clinical course. The results of the present study suggest that mortality rate is higher in patients who fail to achieve the target calorie intake at first 48 h and day seven of admission to the intensive care unit. The risk of mortality decreases with increasing triceps skin fold thickness Z-score.

10.
Sudan J Paediatr ; 22(1): 61-69, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35958075

RESUMO

Exploring the determinants of timely initiation of breastfeeding is necessary for planning efficient breastfeeding promotion programmes. The present study aimed to investigate the frequency and related factors of timely initiation of breastfeeding among Turkish mothers. This study was a cross-sectional, descriptive, mixed model study, which was carried out on mothers with healthy children up to 3 years old who presented to the paediatric clinic between 01 November 2019 and 30 December 2019 in a university hospital. Of the 307 participant mothers, the mean age was 28.3 ± 3.9 years. The frequency of timely initiation of breastfeeding was 70.7%. Significant associations were found between timely initiation of breastfeeding status and residence, type of delivery, type of anaesthesia, birth weight of babies, counselling on timely initiation of breastfeeding during antenatal care and consultant medical staff. In order to increase the breastfeeding rate in the first hour, spontaneous vaginal delivery should be supported and spinal/epidural anaesthesia method should be preferred in cases where caesarean delivery is indicated. Other important steps to increase the ratio of timely initiation of breastfeeding are strengthening breastfeeding counselling services and the midwifery system.

11.
World J Gastrointest Pathophysiol ; 13(6): 178-185, 2022 Nov 22.
Artigo em Inglês | MEDLINE | ID: mdl-36532302

RESUMO

BACKGROUND: Celiac disease (CD) is one of the most prevalent chronic disorders. The clinical manifestations of CD are diverse and may present with gastrointestinal findings, extra-intestinal findings or no symptoms. Although there has been a marked increase in the prevalence of CD in the past 30 years, up to 95% of patients with CD remain undiagnosed. As most cases have atypical signs or no symptoms, the diagnosis of CD is either missed or delayed. In addition, one of the most important reasons for the delay in diagnosis may be the poor knowledge of healthcare professionals (HCPs) regarding CD. AIM: To evaluate the knowledge of HCPs, patients and their caregivers (parents) regarding CD. METHODS: The current study was carried out between June 2021 and February 2022 prospectively, as part of the Focus IN CD project. Patients with CD and their caregivers participated in the study from 6 different cities in Turkey. General practitioners, pediatricians, pediatricians with other subspecialities and pediatric gastroenterologists from different cities participated in the study. RESULTS: The questionnaire was completed by 348 HCPs, 34 patients with CD, and 102 mothers and 34 fathers of patients with CD. Most of the participants were general practitioners (37.07%). There were 89 (25.57%) pediatricians and 72 (20.69%) pediatric gastroenterologists in the study. The highest score in all categories was achieved by pediatric gastroenterologists. There were significant differences between the four groups of HCPs in terms of the subsections of overall mean score, epidemiology and clinical presentation, treatment and follow-up. No significant difference was found between the groups (patients with CD, mothers of patients with CD and fathers of patients with CD) in terms of the questionnaire subsections. CONCLUSION: The level of knowledge on CD among HCPs, patients and their caregivers was unsatisfactory. We consider that it is necessary to increase awareness and to develop e-learning activities on CD among HCPs, patients and their caregivers. Consequently, they may benefit from e-learning programs similar to the one created as part of the EU-funded project Focus IN CD (https://www.celiacfacts.eu/focusincd-en).

12.
J Pediatr Endocrinol Metab ; 35(4): 451-462, 2022 Apr 26.
Artigo em Inglês | MEDLINE | ID: mdl-35038814

RESUMO

OBJECTIVES: To reveal the different clinical presentations of liver glycogen storage disease type IX (GSD IX), which is a clinically and genetically heterogeneous type of glycogenosis. METHODS: The data from the electronic hospital records of 25 patients diagnosed with liver GSD IX was reviewed. Symptoms, clinical findings, and laboratory and molecular analysis were assessed. RESULTS: Of the patients, 10 had complaints of short stature in the initial presentation additionally other clinical findings. Elevated serum transaminases were found in 20 patients, and hepatomegaly was found in 22 patients. Interestingly, three patients were referred due to neurodevelopmental delay and hypotonia, while one was referred for only autism. One patient who presented with neurodevelopmental delay developed hepatomegaly and elevated transaminases during the disease later on. Three of the patients had low hemoglobin A1C and fructosamine values that were near the lowest reference range. Two patients had left ventricular hypertrophy. Three patients developed osteopenia during follow-up, and one patient had osteoporosis after puberty. The most common gene variant, PHKA2, was observed in 16 patients, 10 variants were novel and six variants were defined before. Six patients had variants in PHKG2, two variants were not defined before and four variants were defined before. PHKB variants were found in three patients. One patient had two novel splice site mutations in trans position. It was revealed that one novel homozygous variant and one defined homozygous variant were found in PHKB. CONCLUSIONS: This study revealed that GSD IX may present with only hypotonia and neurodevelopmental delay without liver involvement in the early infantile period. It should be emphasized that although liver GSDIX is thought of as a benign disease, it might present with multisystemic involvement and patients should be screened with echocardiography, bone mineral densitometry, and psychometric evaluation.


Assuntos
Doença de Depósito de Glicogênio Tipo III , Doença de Depósito de Glicogênio , Doença de Depósito de Glicogênio/diagnóstico , Doença de Depósito de Glicogênio/genética , Doença de Depósito de Glicogênio Tipo III/diagnóstico , Doença de Depósito de Glicogênio Tipo III/genética , Hepatomegalia , Humanos , Mutação , Fosforilase Quinase/genética
13.
Am J Gastroenterol ; 106(8): 1512-7, 2011 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-21691340

RESUMO

OBJECTIVES: Epidemiological studies of celiac disease (CD) in Turkey have been performed only within some regions of the country. The aim of this study was to determine the prevalence of CD in Turkish school children. METHODS: Between 2006 and 2008, serum samples were collected from 20,190 students (age range, 6-17 years) in 139 schools in 62 cities from different regions of Turkey. CD was screened using IgA antitissue transglutaminase (IgA-tTG) and total serum IgA. Subjects with selective IgA deficiency were further tested for IgG-tTG. Serum samples positive for IgA or IgG-tTG were further tested for IgA antiendomysial antibodies (IgA-EMAs) using an indirect immunofluorescence method. Small-intestinal biopsy was offered to all subjects with tTG antibody positivity. RESULTS: Of the 20,190 subjects, 489 were antibody positive (IgA-tTG only in 270, both IgA-tTG and IgA-EMA in 215, and IgG-tTG in 4). Selective IgA deficiency was detected in 108 patients, and 4 of them were positive for IgG-tTG. An intestinal biopsy was conducted in 215 subjects (IgA-tTG positive in 110, IgA-tTG and IgA-EMA positive in 104, and IgG-tTG positive in 1). The biopsy findings of 95 children were consistent with CD. Thus, the estimated biopsy-proven prevalence was 1:212 children. The positive predictive value (PPV) for IgA-tTG plus EMA was 75.9%. PPV was 44.3% when only IgA-tTG was used. CONCLUSIONS: We estimate that the prevalence of CD is at least 0.47% in healthy Turkish school children. Screening for IgA-tTG plus EMA provided better results for diagnosis when compared with testing for IgA-tTG alone.


Assuntos
Anticorpos/sangue , Doença Celíaca/diagnóstico , Doença Celíaca/epidemiologia , Glutens/imunologia , Imunoglobulina A/imunologia , Programas de Rastreamento , Transglutaminases/imunologia , Dor Abdominal/etiologia , Adolescente , Autoanticorpos/sangue , Biomarcadores/sangue , Biópsia , Doença Celíaca/complicações , Doença Celíaca/imunologia , Doença Celíaca/patologia , Criança , Constipação Intestinal/etiologia , Diarreia/etiologia , Feminino , Técnica Indireta de Fluorescência para Anticorpo , Humanos , Imunoglobulina A/sangue , Intestino Delgado/metabolismo , Intestino Delgado/patologia , Masculino , Programas de Rastreamento/métodos , Valor Preditivo dos Testes , Prevalência , Sensibilidade e Especificidade , Turquia/epidemiologia
14.
Eur J Pediatr ; 170(6): 803-5, 2011 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-21165748

RESUMO

Papillon-Lefevre syndrome (PLS) is an autosomal recessive disease that is characterized by symmetric palmoplantar keratodermatitis and severe periodontal destruction. Mutations in the cathepsin C gene (CTSC) have recently been detected in PLS. Immune dysregulation, due to a mutation in CTSC, increases the risk of pyogenic infections in PLS patients. A child with PLS is presented here with liver abscesses and peritonitis caused by Rhizopus oryzae. His liver abscess and peritonitis were cured with amphotericin B without surgical care. This is the first case in the literature liver abscess due to Rhizopus oryzae in a child with PLS.


Assuntos
Abscesso Hepático Piogênico/microbiologia , Mucormicose/complicações , Mucormicose/diagnóstico , Doença de Papillon-Lefevre/diagnóstico , Periodontite/microbiologia , Rhizopus/isolamento & purificação , Adolescente , Anfotericina B/uso terapêutico , Humanos , Abscesso Hepático Piogênico/tratamento farmacológico , Masculino , Mucormicose/tratamento farmacológico , Periodontite/tratamento farmacológico , Resultado do Tratamento
15.
Eur J Pediatr ; 170(6): 689-91, 2011 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-21165749

RESUMO

Papillon-Lefevre Syndrome (PLS) is a very rare autosomal recessive disorder characterized by palmoplantar hyperkeratosis and severe early onset periodontitis, affecting the primary and permanent dentition. The syndrome was first described by Papillon and Lefevre in 1924. Genetic, immunologic, and microbiologic factors are suggested as responsible for the initiation and progression of the disease. A point mutation of cathepsin C gene has recently been detected in PLS. A multidisciplinary approach is important for management .The prognosis has improved with the early recognition of the syndrome, effective professional supervision, and home care.


Assuntos
Catepsina C/genética , Epônimos , Doença de Papillon-Lefevre/diagnóstico , Doença de Papillon-Lefevre/terapia , Criança , Diagnóstico Precoce , Humanos , Doença de Papillon-Lefevre/genética , Mutação Puntual , Prognóstico
16.
Nutr Hosp ; 38(1): 43-49, 2021 Feb 23.
Artigo em Inglês | MEDLINE | ID: mdl-33176428

RESUMO

INTRODUCTION: Background: the incidence of childhood acute pancreatitis has been increasing in the last two decades. The number of studies on the effects of diet on the pathogenesis of pancreatitis is very small. The aim of this study was to evaluate adherence to the Mediterranean diet among children with acute pancreatitis. Material and method: this study was conducted by administering the Mediterranean Diet Quality Index (KIDMED) to 100 children -children diagnosed with acute pancreatitis (n = 50) and a control group (n = 50). Results: there was a statistically significant difference between groups in terms of body mass index Z-score (p = 0.017). When the KIDMED scores of the children diagnosed with pancreatitis (4.48 ± 2.45) and the control group (6.62 ± 2.18) were compared, the KIDMED scores of the children diagnosed with pancreatitis group were significantly lower (u = 638.5; p < 0.001). Those with a moderate KIDMED index (score: 4-7) (OR: 3.734, 95 % CI, 1.068-13.058, p = 0.039) and those with a poor KIDMED index (score ≤ 3) (OR: 6.444, 95 % CI, 1.595 -26.075, p = 0.009) were found to have an increased risk of developing pancreatitis. It was found that the risk of pancreatitis increased with age (OR: 1.136, 95 % CI, 1.020-1.266, p = 0.021). Conclusion: according to the data obtained from our study, it was found that the pancreatitis group had poorer dietary habits than the control group. Larger and multicenter studies are needed to evaluate the role of diet in pancreatitis development.


INTRODUCCIÓN: Introducción: la incidencia de la pancreatitis aguda infantil ha aumentado en las últimas dos décadas. La pancreatitis aguda es una inflamación reversible del páncreas que comienza con una lesión de las células acinares. El diagnostico de pancreatitis aguda se realiza según los criterios INSPPIRE. El número de estudios sobre los efectos de la dieta en la patogenia de la pancreatitis es muy pequeño. El objetivo de este estudio fue evaluar la adherencia a la dieta mediterránea de los niños con pancreatitis aguda. Material y métodos: este estudio se realizó administrando el Índice de Calidad de la Dieta Mediterránea (KIDMED) a 100 niños: niños diagnosticados de pancreatitis aguda (n = 50) y un grupo de control (n = 50). Resultados: hubo una diferencia estadísticamente significativa entre los grupos en términos del puntaje Z del índice de masa corporal (p = 0,017). Cuando se compararon las puntuaciones KIDMED de los niños diagnosticados de pancreatitis (4,48 ± 2,45) y del grupo de control (6,62 ± 2,18), las puntuaciones KIDMED del grupo diagnosticado de pancreatitis fueron significativamente más bajas (u = 638,5; p < 0,001). Aquellos con un índice KIDMED moderado (puntaje: 4-7) (OR: 3,734; IC 95 %: 1,068-13,058, p = 0,039) y aquellos con un índice KIDMED pobre (puntaje ≤ 3) (OR: 6,444; IC 95%: 1,595-26,075, p = 0,009) presentaban mayor riesgo de desarrollar pancreatitis. Se encontró que el riesgo de pancreatitis aumentaba a medida que aumentaba la edad (OR: 1,136; IC 95%: 1,020-1,266, p = 0,021). Conclusión: según los datos obtenidos de nuestro estudio, se encontró que el grupo con pancreatitis presentaba peores hábitos alimentarios que el grupo de control. Se necesitan estudios más grandes y multicéntricos para evaluar el papel de la dieta en el desarrollo de la pancreatitis.


Assuntos
Dieta Mediterrânea/estatística & dados numéricos , Pancreatite/diagnóstico , Cooperação do Paciente/estatística & dados numéricos , Doença Aguda , Adolescente , Fatores Etários , Índice de Massa Corporal , Estudos de Casos e Controles , Criança , Pré-Escolar , Intervalos de Confiança , Inquéritos sobre Dietas , Feminino , Humanos , Masculino , Razão de Chances , Pancreatite/etiologia , Fatores Sexuais , Inquéritos e Questionários
17.
Sao Paulo Med J ; 139(3): 201-209, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33681884

RESUMO

BACKGROUND: Coronavirus disease 2019 (COVID-19) causes negative life changes brought about through lockdowns, in addition to severe complications and death. Among these changes, asplenism or hyposplenism has been reported in patients with celiac disease. It has been reported that the risk of pneumococcal sepsis is higher in celiac patients with hyposplenism. Moreover, celiac patients present high risk of admission to hospital due to influenza. OBJECTIVE: To determine the degree of awareness of COVID-19 among parents of children with celiac disease and examine the measures that they take. DESIGN AND SETTING: Cross-sectional study at a university hospital in the Middle Anatolian region of Turkey. METHODS: The diagnosis of celiac disease was confirmed through a survey conducted online among 73 parents between May and July 2020. RESULTS: The mean age was 37.57 ± 6.56 years for the mothers, 41.15 ± 5.56 years for the fathers and 11.36 ± 4.36 years for the children. 90.4% of the parents reported that COVID-19 was transmitted through "speaking, coughing, sneezing and infection of the face after contact with virus-infected surfaces". Moreover, 78.1% indicated that they did not have any difficulty in finding gluten-free foods. CONCLUSION: These parents of children with celiac disease believed that their children's risk of developing COVID-19 did not differ from that of healthy children. It was also observed that appetite and states of nervousness were higher among these children with celiac disease during lockdowns and that their sleep patterns were affected.


Assuntos
COVID-19 , Doença Celíaca , Telemedicina , Adulto , Criança , Controle de Doenças Transmissíveis , Estudos Transversais , Feminino , Humanos , Pandemias , Pais , SARS-CoV-2 , Inquéritos e Questionários , Turquia/epidemiologia
18.
North Clin Istanb ; 8(6): 619-622, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-35284794

RESUMO

Lightning strike is a devastating disaster, leading to various life-threating complications and even death. In lightning striking victims, high-voltage electric current can destroy many tissues and organs through various mechanisms. One of these mechanisms is the blunt trauma that injures the organs by creating a blast effect. Although not frequent, blunt trauma may result in various solid organ injuries such as pulmonary contusion. In this article, we reported a 15-year-old male patient who was admitted to the emergency department because of lightning strike in open terrain. Although he was conscious and vital signs were normal at presentation, respiratory distress developed on the 4th day. Unilateral pulmonary contusion was detected on the computerized tomography of the thorax. The patient was treated with supportive oxygen and intravenous hydration therapy. His respiratory distress improved on the 6th day and control posteroanterior chest radiograph revealed that pulmonary hemorrhage was spontaneously resorbed. On the 9th day, he was discharged with normal respiratory findings. The patient did not have any complaints during the 3-month follow-up after discharge.

19.
Turk Arch Pediatr ; 56(1): 22-26, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-34013225

RESUMO

OBJECTIVE: The aim of this study is to compare the screen time and digital gaming habits of Turkish children before and during the coronavirus disease 2019 (COVID-19) pandemic. MATERIAL AND METHODS: This cross-sectional descriptive study was carried out in a university hospital between May 7, 2020 and June 27, 2020. Healthy children aged 3-10 years who applied to the pediatrics clinics for outpatient care were included in the study. A structured survey was applied to the parents who agreed to participate. RESULTS: A total of 253 children were included in the study. It was found that the ratio of children with screen time of ≥1 hour during the pandemic was significantly higher than before the pandemic (p<0.001). It was determined that the ratio of children watching both adult and children's programs increased during the pandemic (p<0.001). The ratio of children playing digital games during the pandemic was also found to be increased significantly compared with that before the pandemic (p<0.001). CONCLUSION: This study demonstrated that screen and digital gaming time increased independently of sociodemographic characteristics during the COVID-19 pandemic compared with before the pandemic.

20.
J Pediatr Genet ; 10(1): 74-76, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33552644

RESUMO

Mucopolysaccharidosis type IIIB (Sanfilippo's B; OMIM no.: 252920) is a lysosomal storage disorder caused by defective degradation of heparan sulfate. The enzyme that has decreased function in this disease is α-N acetylglucosaminidase. This enzyme is encoded by the NAGLU gene. A 9-year-old male patient was referred to us with speech disability, developmental delay, hepatomegaly, mild learning disability, and otitis media with effusion complaints. Whole exome sequencing (WES) was performed because of consanguinity between the parents of the patient and the lack of specific prediagnosis. As a result of the patient's WES analysis, a homozygous mutation was detected in the NAGLU gene. The leukocyte enzyme activity was then evaluated to confirm the diagnosis. Alpha-N acetylglucosaminidase deficiency was found. Alpha-N acetylglucosaminidase activity was 0.2 nmol/mLh. WES is a successful diagnostic method in the diagnosis of the mild clinical diseases with recessive inheritance. In addition, our case is a good example of genotype to phenotype diagnosis. Because in storage diseases, the diagnosis is made by leukocyte enzyme analysis first, and then the result is confirmed by gene analysis. The opposite situation occurred in our case.

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