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1.
Pediatr Neurol ; 156: 85-90, 2024 Apr 21.
Artigo em Inglês | MEDLINE | ID: mdl-38733859

RESUMO

BACKGROUND: Ataxia telangiectasia (AT) is a genetic multisystemic disorder affecting the nervous system. Data on neurocognitive functioning in AT are limited and focused on patients at various stages of disease. Because of the genetic nature of the disorder, parents of patients may also display subtle neurological problems. This study aimed to evaluate neurocognitive functioning in patients with AT and their unaffected parents. METHODS: The study included 26 patients with AT and 41 parents among which 13 patients and 18 parents were evaluated with neurocognitive tests. Clinical and radiological data were reviewed retrospectively. Data were analyzed with descriptive statistics. RESULTS: The median ages of patients and parents were 12.5 years (interquartile range [IQR] = 9.5) and 38.0 years (IQR = 12.0), respectively. Median intelligence quotients were 62.0 (IQR = 21.3) and 82.5 (IQR = 16.8), respectively, for patients and parents. Rates of intellectual disability for patients and parents were 100.0% and 83.3%, respectively. Areas of impairment in patients in decreasing order of frequency were motor skills, visual perception/memory, visual-manual coordination, spontaneous/focused and sustained attention (100.0% for each), social judgment, as well as vocabulary and arithmetic skills (75.0% for each). Areas of impairment in unaffected parents in decreasing order of frequency were visual-manual coordination (77.8%), working memory (76.5%), and visual perception and motor skills (66.7% for each). CONCLUSION: Intellectual disabilities, visual-spatial disabilities, and reduced visual-motor coordination seem to be similar in patients with AT and their parents. These results should be replicated with larger samples from multiple centers and may form putative cognitive endophenotypes for the disorder.

2.
Children (Basel) ; 11(1)2024 Jan 22.
Artigo em Inglês | MEDLINE | ID: mdl-38275444

RESUMO

OBJECTIVES: Septic arthritis (SA) is a serious bacterial infection that must be treated efficiently and timely. The large number of culture-negative cases makes local epidemiological data important. Accordingly, this study aimed to evaluate the etiology, clinical characteristics, and therapeutic approach of SA in children in Turkiye, emphasizing the role of real-time polymerase chain reaction (PCR) techniques in the diagnosis. METHODS: In this multi-center, prospective study, children hospitalized due to SA between February 2018 and July 2020 in 23 hospitals in 14 cities in Turkiye were included. Clinical, demographic, laboratory, and radiological findings were assessed, and real-time PCR was performed using synovial fluid samples. RESULTS: Seventy-five children aged between 3 and 204 months diagnosed with acute SA were enrolled. Joint pain was the main complaint at admission, and the most commonly involved joints were the knees in 58 patients (77.4%). The combination of synovial fluid culture and real-time PCR detected causative bacteria in 33 patients (44%). In 14 (18.7%) patients, the etiological agent was demonstrated using only PCR. The most commonly isolated etiologic agent was Staphylococcus aureus, which was detected in 22 (29.3%) patients, while Streptococcus pyogenes was found in 4 (5.3%) patients and Kingella kingae in 3 (4%) patients. Streptococcus pyogenes and Kingella kingae were detected using only PCR. Most patients (81.3%) received combination therapy with multiple agents, and the most commonly used combination was glycopeptides plus third-generation cephalosporin. CONCLUSIONS: Staphylococcus aureus is the main pathogen in pediatric SA, and with the use of advanced diagnostic approaches, such as real-time PCR, the chance of diagnosis increases, especially in cases due to Kingella kingae and Streptococcus pyogenes.

3.
Eur J Pediatr ; 183(3): 1153-1162, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37971516

RESUMO

To investigate coronavirus disease 2019 (COVID-19) in infants aged 0 to 3 months because there is currently a significant gap in the literature on the subject. A cross-sectional study was conducted with the involvement of 19 medical centers across Turkey and 570 infants. The majority of the patients were male (58.2%), and the three most common symptoms were fever (78.2%), cough (44.6%), and feeding intolerance (39.9%). The results showed that a small percentage of infants had positive blood (0.9%) or urine cultures (10.2%). Most infants presented with fever (78.2%). Children without underlying conditions (UCs) had mostly a complicated respiratory course and a normal chest radiography. Significant more positive urine culture rates were observed in infants with fever. A higher incidence of respiratory support requirements and abnormal chest findings were seen in infants with chronic conditions. These infants also had a longer hospital stay than those without chronic conditions.  Conclusions: Our study discloses the clinical observations and accompanying bacterial infections found in infants aged under 3 months with COVID-19. These findings can shed light on COVID-19 in infancy for physicians because there is limited clinical evidence available. What is Known: • COVID-19 in infants and older children has been seen more mildly than in adults. • The most common symptoms of COVID-19 in infants are fever and cough, as in older children and adults. COVID-19 should be one of the differential diagnoses in infants with fever. What is New: • Although most infants under three months had fever, the clinical course was uneventful and respiratory complications were rarely observed in healthy children. • Infants with underlying conditions had more frequent respiratory support and abnormal chest radiography and stayed longer in the hospital.


Assuntos
COVID-19 , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Doença Crônica , Tosse/etiologia , COVID-19/epidemiologia , COVID-19/complicações , Estudos Transversais , Turquia/epidemiologia
4.
Eur J Pediatr ; 182(12): 5531-5542, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37782350

RESUMO

PURPOSE: Due to its link with the 2019 coronavirus, the multisystem inflammatory syndrome in children (MISC) has garnered considerable international interest. The aim of this study, in which MISC patients were evaluated multicenter, and the data of the third period of the Turk-MISC study group, to compare the clinical and laboratory characteristics and outcomes of MISC patients who did and did not require admission to an intensive care unit (ICU). METHODS: This retrospective multicenter observational study was carried out between June 11, 2021, and January 01, 2022. The demographics, complaints, laboratory results, system involvements, and outcomes of the patients were documented. RESULTS: A total of 601 patients were enrolled; 157 patients (26.1%) required hospitalization in the intensive care unit (ICU). Median age was 8 years (interquartile range (IQR) 4.5-11.3 years. The proportion of Kawasaki disease-like features in the ICU group was significantly higher than in the non-ICU group (56.1% vs. 43.2% p = 0.006). The ICU group had considerably lower counts of both lymphocytes and platelets (lymphocyte count 900 vs. 1280 cells × µL, platelet count 153 vs. 212 cells × 103/ µL, all for p< 0.001). C-reactive protein, procalcitonin, and ferritin levels were significantly higher in the ICU group (CRP 164 vs. 129 mg/L, procalcitonin 9.2 vs. 2.2 µg/L, ferritin 644 vs. 334 µg/L, all for p< 0.001). Being between ages 5-12 and older than 12 increased the likelihood of hospitalization in the ICU by four [95% confidence intervals (CI)1.971-8.627] and six times (95% CI 2.575-14.654), respectively, compared to being between the ages 0-5. A one-unit increase in log D-dimer (µg/L) and log troponin (ng/L) was also demonstrated to increase the need for intensive care by 1.8 (95% CI 1.079-3.233) and 1.4 times (95% CI 1.133-1.789), respectively.     Conclusion: By comparing this study to our other studies, we found that the median age of MISC patients has been rising. Patients requiring an ICU stay had considerably higher levels of procalcitonin, CRP, and ferritin but significantly lower levels of lymphocyte and thrombocyte. In particular, high levels of procalcitonin in the serum might serve as a valuable laboratory marker for anticipating the need for intensive care. WHAT IS KNOWN: • Lymphopenia and thrombocytopenia were an independent predictor factors in patients with MISC who needed to stay in intensive care unit. • The possibility of the need to stay in the intensive care unit in patients with MISC who had Kawasaki disease-like findings was controversial compared with those who did not. WHAT IS NEW: • A one-unit increase log D dimer and log troponin was demonstrated to require for intensive care unit by 1.8 and 1.4 times, respectively. • Serum procalcitonin levels had the best performance to predict stay in the intensive care unit stay.


Assuntos
Síndrome de Linfonodos Mucocutâneos , Criança , Humanos , Recém-Nascido , Lactente , Pré-Escolar , Pró-Calcitonina , Unidades de Terapia Intensiva , Ferritinas , Troponina , Estudos Retrospectivos
5.
BMC Pediatr ; 23(1): 121, 2023 03 18.
Artigo em Inglês | MEDLINE | ID: mdl-36932373

RESUMO

BACKGROUND: Antibiotic-associated diarrhea is one of the most frequent side effects of antimicrobial therapy. We assessed the epidemiological data of antibiotic-associated diarrhea in pediatric patients in our region. METHODS: The prospective multi-center study included pediatric patients who were initiated an oral antibiotic course in outpatient clinics and followed in a well-established surveillance system. This follow-up system constituded inclusion of patient by the primary physician, supply of family follow-up charts to the family, passing the demographics and clinical information of patient to the Primary Investigator Centre, and a close telephone follow-up of patients for a period of eight weeks by the Primary Investigator Centre. RESULTS: A result of 758 cases were recruited in the analysis which had a frequency of 10.4% antibiotic-associated diarrhea. Among the cases treated with amoxicillin-clavulanate 10.4%, and cephalosporins 14.4% presented with antibiotic-associated diarrhea. In the analysis of antibiotic-associated diarrhea occurrence according to different geographical regions of Turkey, antibiotic-associated diarrhea episodes differed significantly (p = 0.014), particularly higher in The Eastern Anatolia and Southeastern Anatolia. Though most commonly encountered with cephalosporin use, antibiotic-associated diarrhea is not a frequent side effect. CONCLUSION: This study on pediatric antibiotic-associated diarrhea displayed epidemiological data and the differences geographically in our region.


Assuntos
Antibacterianos , Pacientes Ambulatoriais , Criança , Humanos , Estudos Prospectivos , Antibacterianos/efeitos adversos , Combinação Amoxicilina e Clavulanato de Potássio/efeitos adversos , Cefalosporinas/efeitos adversos , Diarreia/induzido quimicamente , Diarreia/epidemiologia , Diarreia/tratamento farmacológico
7.
Hum Vaccin Immunother ; 18(5): 2044707, 2022 11 30.
Artigo em Inglês | MEDLINE | ID: mdl-35714279

RESUMO

INTRODUCTION: Health care workers (HCWs) are disproportionately exposed to infectious diseases and play a role in nosocomial transmission, making them a key demographic for vaccination. HCW vaccination rates are not optimal in many countries; hence, compulsory vaccination policies have been implemented in some countries. Although these policies are effective and necessary under certain conditions, resolving HCWs' hesitancies and misconceptions about vaccines is crucial. HCWs have the advantage of direct contact with patients; hence, they can respond to safety concerns, explain the benefits of vaccination, and counter antivaccine campaigns that escalate during pandemics, as has been observed with COVID-19. METHOD: A short survey was carried out in May-June 2020 on the vaccination status of HCWs working with pediatric patients with COVID-19. The survey inquired about their vaccination status (mumps/measles/rubella [MMR], varicella, influenza, and diphtheria/tetanus [dT]) and willingness to receive hypothetical future COVID-19 vaccines. The respondents were grouped according to gender, age, occupation, and region. RESULTS: In total, 4927 HCWs responded to the survey. Most were young, healthy adults. The overall vaccination rates were 57.8% for dT in the past 10 years, 44.5% for MMR, 33.2% for varicella, and 13.5% for influenza. Vaccination rates were the highest among physicians. The majority of HCWs (81%) stated that they would be willing to receive COVID-19 vaccines. CONCLUSION: Although vaccination rates for well-established vaccines were low, a majority of HCWs were willing to receive COVID-19 vaccines when available. Education and administrative trust should be enhanced to increase vaccination rates among HCWs.


Assuntos
COVID-19 , Varicela , Vacinas contra Influenza , Influenza Humana , Sarampo , Adulto , COVID-19/prevenção & controle , Vacinas contra COVID-19 , Criança , Pessoal de Saúde , Humanos , Influenza Humana/prevenção & controle , Sarampo/prevenção & controle , SARS-CoV-2 , Vacinação
8.
Eur J Pediatr ; 181(5): 2031-2043, 2022 May.
Artigo em Inglês | MEDLINE | ID: mdl-35129668

RESUMO

Multisystemic inflammatory syndrome (MIS-C) diagnosis remains difficult because the clinical features overlap with Kawasaki disease (KD). The study aims to highlight the clinical and laboratory features and outcomes of patients with MISC whose clinical manifestations overlap with or without KD. This study is a retrospective analysis of a case series designed for patients aged 1 month to 18 years in 28 hospitals between November 1, 2020, and June 9, 2021. Patient demographics, complaints, laboratory results, echocardiographic results, system involvement, and outcomes were recorded. A total of 614 patients were enrolled; the median age was 7.4 years (interquartile range (IQR) 3.9-12 years). A total of 277 (45.1%) patients with MIS-C had manifestations that overlapped with KD, including 92 (33.3%) patients with complete KD and 185 (66.7%) with incomplete KD. Lymphocyte and platelet counts were significantly lower in patients with MISC, overlapped with KD (lymphocyte count 1080 vs. 1280 cells × µL, p = 0.028; platelet count 166 vs. 216 cells × 103/µL, p < 0.001). The median serum procalcitonin levels were statistically higher in patients overlapped with KD (3.18 vs. 1.68 µg/L, p = 0.001). Coronary artery dilatation was statistically significant in patients with overlap with KD (13.4% vs. 6.8%, p = 0.007), while myocarditis was significantly more common in patients without overlap with KD features (2.6% vs 7.4%, p = 0.009). The association between clinical and laboratory findings and overlap with KD was investigated. Age > 12 years reduced the risk of overlap with KD by 66% (p < 0.001, 95% CI 0.217-0.550), lethargy increased the risk of overlap with KD by 2.6-fold (p = 0.011, 95% CI 1.244-5.439), and each unit more albumin (g/dl) reduced the risk of overlap with KD by 60% (p < 0.001, 95% CI 0.298-0.559). CONCLUSION: Almost half of the patients with MISC had clinical features that overlapped with KD; in particular, incomplete KD was present. The median age was lower in patients with KD-like features. Lymphocyte and platelet counts were lower, and ferritin and procalcitonin levels were significantly higher in patients with overlap with KD. WHAT IS KNOWN: • In some cases of MIS-C, the clinical symptoms overlap with Kawasaki disease. • Compared to Kawasaki disease, lymphopenia was an independent predictor of MIS-C. WHAT IS NEW: • Half of the patients had clinical features that overlapped with Kawasaki disease. • In patients whose clinical features overlapped with KD, procalcitonin levels were almost 15 times higher than normal. • Lethargy increased the risk of overlap with KD by 2.6-fold in MIS-C patients. • Transient bradycardia was noted in approximately 10% of our patients after initiation of treatment.


Assuntos
COVID-19 , Síndrome de Linfonodos Mucocutâneos , COVID-19/complicações , COVID-19/diagnóstico , Criança , Pré-Escolar , Humanos , Letargia , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/tratamento farmacológico , Pró-Calcitonina , Estudos Retrospectivos , SARS-CoV-2 , Síndrome de Resposta Inflamatória Sistêmica
9.
J Pediatr Hematol Oncol ; 44(1): e296-e298, 2022 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-33828033

RESUMO

INTRODUCTION: Roseola infantum is always considered to be among the differential diagnosis of young patients with fever and leukopenia whom to be strictly isolated with the preliminary diagnosis of COVID-19 until otherwise proven during the pandemic. RESULTS: Human herpes virus-6 (HHV-6) polymerase chain reaction (PCR) blood test was performed in 4 of 7 patients with a clinical diagnosis of roseola infantum and all found to be HHV-6 PCR positive. The most striking laboratory finding in all patients was leukopenia. HHV-6 PCR tests were found to be positive. Severe acute respiratory syndrome coronavirus-2 testing were found to be negative in all patients. CONCLUSION: During the peak of the pandemic, children continued to present with fever because of viral infections other than COVID-19.


Assuntos
Exantema Súbito/diagnóstico , Herpesvirus Humano 6/isolamento & purificação , COVID-19/diagnóstico , Pré-Escolar , Feminino , Humanos , Lactente , Leucopenia/diagnóstico , Masculino , SARS-CoV-2/isolamento & purificação
10.
Int J Infect Dis ; 113: 184-189, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34592441

RESUMO

BACKGROUND: Understanding SARS-CoV-2 seroprevalence among health care personnel is important to explore risk factors for transmission, develop elimination strategies and form a view on the necessity and frequency of surveillance in the future. METHODS: We enrolled 4927 health care personnel working in pediatric units at 32 hospitals from 7 different regions of Turkey in a study to determine SARS Co-V-2 seroprevalence after the first peak of the COVID-19 pandemic. A point of care serologic lateral flow rapid test kit for immunoglobulin (Ig)M/IgG was used. Seroprevalence and its association with demographic characteristics and possible risk factors were analyzed. RESULTS: SARS-CoV-2 seropositivity prevalence in health care personnel tested was 6.1%. Seropositivity was more common among those who did not universally wear protective masks (10.6% vs 6.1%). Having a COVID-19-positive co-worker increased the likelihood of infection. The least and the most experienced personnel were more likely to be infected. Most of the seropositive health care personnel (68.0%) did not suspect that they had previously had COVID-19. CONCLUSIONS: Health surveillance for health care personnel involving routine point-of-care nucleic acid testing and monitoring personal protective equipment adherence are suggested as important strategies to protect health care personnel from COVID-19 and reduce nosocomial SARS-CoV-2 transmission.


Assuntos
COVID-19 , Pandemias , Anticorpos Antivirais , Criança , Atenção à Saúde , Pessoal de Saúde , Humanos , SARS-CoV-2 , Estudos Soroepidemiológicos , Turquia/epidemiologia
11.
Pediatr Transplant ; 25(7): e14061, 2021 11.
Artigo em Inglês | MEDLINE | ID: mdl-34076953

RESUMO

BACKGROUND: In 2019, SARS-CoV-2 causing COVID-19 emerged. Severe COVID-19 symptoms may evolve by virtue of hyperactivation of the immune system. Equally, immunocompromised patients may be at increased risk to develop COVID-19. However, treatment guidelines for children following liver transplantation are elusive. METHODS: As a liver transplantation center, we diagnosed and followed up 10 children (male/female: 8/2) with a median age of 8.5 years (IQR: 5.2-11.0), with COVID-19 post-liver transplant between March 2019 and December 2020. COVID-19 diagnosis was based on PCR test and or florid X-ray findings compatible with COVID-19 in the absence of other cause. We retrospectively collected clinical and laboratory data from electronic patient records following written consent from patients/parents. RESULTS: Nine patients were diagnosed as definitive (PCR positive) with one patient being diagnosed as probable COVID-19. Seven patients recovered without any support whereas three were admitted for non-invasive oxygenation. Lymphopenia and/or high levels of serum IL-6 were detected in four patients. Six patients mounted anti-SARS-CoV-2 antibodies at median 30 days (IQR: 26.5-119.0) following COVID-19 diagnosis. Antibiotic therapy, favipiravir, anakinra, and IVIG were used as treatment in 4,1,1 and 2 patients, respectively. Furthermore, we kept the tacrolimus with or without everolimus but stopped MMF in 2 patients. Importantly, liver allograft function was retained in all patients. CONCLUSIONS: We found that being immunocompromised did not affect disease severity nor survival. Stopping MMF yet continuing with tacrolimus was an apt treatment modality in these patients.


Assuntos
COVID-19/terapia , Gerenciamento Clínico , Hepatopatias/cirurgia , Transplante de Fígado , RNA Viral/análise , SARS-CoV-2/genética , Transplantados , COVID-19/epidemiologia , COVID-19/virologia , Pré-Escolar , Comorbidade , Progressão da Doença , Feminino , Seguimentos , Humanos , Hospedeiro Imunocomprometido , Lactente , Hepatopatias/epidemiologia , Masculino , Pandemias , Estudos Retrospectivos
12.
Front Pediatr ; 9: 631547, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34055680

RESUMO

Objectives: The aim of this study is to identify the epidemiological, clinical, and laboratory features of coronavirus disease 2019 (COVID-19) in children. Methods: A retrospective study was conducted by pediatric infectious disease specialists from 32 different hospitals from all over Turkey by case record forms. Pediatric cases who were diagnosed as COVID-19 between March 16, 2020, and June 15, 2020 were included. Case characteristics including age, sex, dates of disease onset and diagnosis, family, and contact information were recorded. Clinical data, including the duration and severity of symptoms, were also collected. Laboratory parameters like biochemical tests and complete blood count, chest X-ray, and chest computed tomography (CT) were determined. Results: There were 1,156 confirmed pediatric COVID-19 cases. In total, male cases constituted 50.3% (n = 582) and females constituted 49.7% (n = 574). The median age of the confirmed cases was 10.75 years (4.5-14.6). Of the total cases, 90 were younger than 1 year of age (7.8%), 108 were 1-3 years of age (9.3%), 148 were 3-6 years of age (12.8%), 298 were 6-12 years of age (25.8%), 233 were 12-15 years of age (20.2%), and 268 cases were older than 15 years of age (23.2%). The most common symptom of the patients at the first visit was fever (50.4%) (n = 583) for a median of 2 days (IQR: 1-3 days). Fever was median at 38.4°C (38.0-38.7°C). The second most common symptom was cough (n = 543, 46.9%). The other common symptoms were sore throat (n = 143, 12.4%), myalgia (n = 141, 12.2%), dyspnea (n = 118, 10.2%), diarrhea (n = 112, 9.7%), stomachache (n = 71, 6.1%), and nasal discharge (n = 63, 5.4%). When patients were classified according to disease severity, 263 (22.7%) patients were asymptomatic, 668 (57.7%) patients had mild disease, 209 (18.1%) had moderate disease, and 16 (1.5%) cases had severe disease. One hundred and forty-nine (12.9%) cases had underlying diseases among the total cases; 56% of the patients who had severe disease had an underlying condition (p < 0.01). The need for hospitalization did not differ between patients who had an underlying condition and those who do not have (p = 0.38), but the need for intensive care was higher in patients who had an underlying condition (p < 0.01). Forty-seven (31.5%) of the cases having underlying conditions had asthma or lung disease (38 of them had asthma). Conclusions: To the best of our knowledge, this is one of the largest pediatric data about confirmed COVID-19 cases. Children from all ages appear to be susceptible to COVID-19, and there is a significant difference in symptomatology and laboratory findings by means of age distribution.

13.
J Trop Pediatr ; 67(2)2021 05 17.
Artigo em Inglês | MEDLINE | ID: mdl-34028559

RESUMO

Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) has caused a pandemic affecting many countries and millions of people. Physicians have encountered some rare and challenging cases related to SARS-CoV-2, a novel virus with still many unknowns. In order to share our experience of a such clinical picture, we present here a child with SARS-CoV-2-induced macrophage activation syndrome in the setting of juvenile idiopathic arthritis.


Assuntos
Artrite Juvenil , COVID-19 , Síndrome de Ativação Macrofágica , Artrite Juvenil/complicações , Criança , Humanos , Síndrome de Ativação Macrofágica/diagnóstico , Síndrome de Ativação Macrofágica/etiologia , Pandemias , SARS-CoV-2
14.
Pediatr Allergy Immunol ; 32(2): 349-357, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33012025

RESUMO

BACKGROUND: Heterozygous relatives of ataxia-telangiectasia (AT) patients are at an increased risk for certain AT-related manifestations. We also show that there is an increase of infection frequency in parents of AT patients. Thus, we hypothesized that the parents might exhibit immune alterations similar to their affected children. METHODS: Lymphocyte phenotyping to enumerate T- and B-cell subsets was performed. Functional analyses included in vitro quantified γ-H2AX, poly (ADP-ribose) polymerase (PARP) and caspase-9 proteins. Chromosomal instability was determined by comet assay. RESULTS: We analyzed 20 AT patients (14F/6M), 31 parents (16F/15M), and 35 age-matched healthy controls. The AT patients' parents exhibited low frequency of naive CD4+ T- (n = 14, 45%) and recent thymic emigrants (n = 11, 35%) in comparison with the age-matched healthy donors. Interestingly, parents with low naive T cells also demonstrated high rate of recurrent infections (9/14, 64%). In comparison with age-matched controls, parents who had recurrent infections and low naive T cells showed significantly higher baseline γ-H2AX levels and H2 O2 -induced DNA damage as well as increased cleaved caspase-9 and PARP proteins. CONCLUSION: Parents of AT patients could present with recurrent infections and display cellular defects that mimic AT patients. The observed immunological changes could be associated with increased DNA double-strand breaks.


Assuntos
Ataxia Telangiectasia , Ataxia Telangiectasia/diagnóstico , Ataxia Telangiectasia/genética , Proteínas Mutadas de Ataxia Telangiectasia/genética , Proteínas de Ciclo Celular/genética , Proteínas de Ciclo Celular/metabolismo , Proteínas de Ligação a DNA/genética , Humanos , Pais , Fenótipo , Proteínas Supressoras de Tumor/genética , Proteínas Supressoras de Tumor/metabolismo
15.
Eur J Gastroenterol Hepatol ; 32(9): 1251-1255, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32541245

RESUMO

Immunocompromised patients may be at increased risk to develop COVID-19 during the 2019 ß-coronavirus infection. We present the unique opportunity we had to monitor the liver, IL-6 and immune cell course before, during and after COVID-19 in a boy with autoimmune hepatitis (AIH) and type 1 diabetes (T1D). CD4 and CD8 T cells frequencies decreased because of prednisolone, followed by a plateauing increase whereas CD19CD20 B cell increased strongly and was unaffected by COVID-19 infection. Moreover, the percentage of activated CD8 T cells expressing HLA-DR (CD8HLA-DR) increased during COVID-19 and subsided after its clearance. Total regulatory T cells (Tregs: CD4CD25CD127FOXP3) remained stable. Although activated Tregs (CD4CD45RAFOXP3) strongly increased upon prednisolone, it decreased afterwards. Furthermore, regulatory B cells (Bregs: CD19CD20CD24CD38) declined sharply owing to prednisolone. Serum IL-6 remained undetectable at all times. We demonstrated for the first time immune monitoring in a child with AIH and T1D before, during and after COVID-19. We hypothesize that continuing with low level of prednisolone without azathioprine may have abrogated activated Tregs, Bregs and IL-6 production and therefore permitting the activation of CD8 T cells, clearing the virus.


Assuntos
Betacoronavirus , Infecções por Coronavirus/imunologia , Infecções por Coronavirus/terapia , Diabetes Mellitus Tipo 1/imunologia , Hepatite Autoimune/imunologia , Pneumonia Viral/imunologia , Pneumonia Viral/terapia , Linfócitos T CD4-Positivos , Linfócitos T CD8-Positivos , COVID-19 , Pré-Escolar , Infecções por Coronavirus/complicações , Diabetes Mellitus Tipo 1/complicações , Diabetes Mellitus Tipo 1/terapia , Hepatite Autoimune/complicações , Hepatite Autoimune/terapia , Humanos , Contagem de Linfócitos , Masculino , Monitorização Imunológica , Pandemias , Pneumonia Viral/complicações , SARS-CoV-2
16.
Pediatr Blood Cancer ; 67(2): e28091, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31736244

RESUMO

BACKGROUND: Regarding the difficulties in recognition and management of the malignancies in primary immune deficiencies (PIDs), we aimed to present the types, risk factors, treatment options, and prognosis of the cancers in this specific group. METHODS: Seventeen patients with PID who developed malignancies or malignant-like diseases were evaluated for demographics, clinical features, treatment, toxicity, and prognosis. RESULTS: The median age of malignancy was 12.2 years (range, 2.2-26). Lymphoma was the most frequent malignancy (n = 7), followed by adenocarcinoma (n = 3), squamous cell carcinoma (n = 2), cholangiocarcinoma (n = 1), Wilms tumor (n = 1), and acute myeloid leukemia (n = 1). Nonneoplastic lymphoproliferation mimicking lymphoma was observed in five patients. The total overall survival (OS) was 62.5% ± 12.1%. The OS for lymphoma was 62.2% ± 17.1% and found to be inferior to non-PID patients with lymphoma (P = 0.001). CONCLUSION: In patients with PIDs, malignancy may occur and negatively affect the OS. The diagnosis can be challenging in the presence of nonneoplastic lymphoproliferative disease or bone marrow abnormalities. Awareness of susceptibility to malignant transformation and early diagnosis with multidisciplinary approach can save the patients' lives.


Assuntos
Tecido Linfoide/patologia , Linfoma/classificação , Linfoma/diagnóstico , Doenças da Imunodeficiência Primária/complicações , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Linfoma/etiologia , Linfoma/terapia , Masculino , Doenças da Imunodeficiência Primária/patologia , Prognóstico , Taxa de Sobrevida , Adulto Jovem
17.
Turk J Pediatr ; 60(3): 340-343, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30511552

RESUMO

Sütçü M, Aktürk H, Gülümser-Sisko S, Acar M, Erol OB, Somer A, Bilgiç B, Salman N. Granulomatous amebic encephalitis caused by Acanthamoeba in an immuncompetent child. Turk J Pediatr 2018; 60: 340-343. Acanthamoeba may lead to granulomatous amebic encephalitis (GAE) with high mortality rates generally in patients with immunosupression and/or chronic disease. Here, we present a rare GAE case, who was a previously healthy child. A Georgian 9 year old boy presented with focal seizure on his left arm and confusion. Since computed tomography (CT) demonstrated hypodense lesion on right occipital lobe, brain biopsy was performed. Histopathological examination of the biopsy material revealed Acanthamoeba cysts and trophozoites together with granulomatous inflammation. The patient, who had no clinical and laboratory findings consistent with immunedeficiency, was diagnosed as GAE. He was treated with a combination drug therapy. Even if it is very rare, amebic meningoencephalitis may also be seen in immunocompetent children, as in our case. Definitive diagnosis is made by microbiological and histopathological examination of brain biopsy material.


Assuntos
Acanthamoeba/isolamento & purificação , Amebíase/diagnóstico , Anti-Infecciosos/uso terapêutico , Infecções Protozoárias do Sistema Nervoso Central/diagnóstico , Encefalite Infecciosa/diagnóstico , Amebíase/tratamento farmacológico , Animais , Encéfalo/parasitologia , Infecções Protozoárias do Sistema Nervoso Central/tratamento farmacológico , Criança , Quimioterapia Combinada , Evolução Fatal , Granuloma/diagnóstico , Humanos , Encefalite Infecciosa/tratamento farmacológico , Imageamento por Ressonância Magnética , Masculino , Tomografia Computadorizada por Raios X
18.
Turk Neurosurg ; 28(1): 79-87, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-27858396

RESUMO

AIM: To evaluate the neurological outcomes of children diagnosed with brain abscesses in the early post-treatment period. < b > MATERIAL and METHODS: This study was a retrospective analysis of pediatric brain abscess patients between January 2000 and December 2015, during a 16-years period. Patients were divided into two groups according to their outcome at the end of the treatment. The patients with "good outcome" were the ones without any neurological sequelae [Glasgow Outcome Scale (GOS) score 5]. "Unfavorable outcome" was defined as having any kind of neurological deficit (GOS score 1-4). RESULTS: A total number of 31 patients (22 male, 71%) with the median age at diagnosis of 84 months (range, 1-202 months) were enrolled in this study. The most common presenting symptom was fever being encountered in 71% of the patients (n=22), followed by focal neurological deficit (FND)(n=17, 54.8%), vomiting (n=14, 45.2%), headache (n=13, 41.9%), seizure (n=13, 41.9%), change in mental status (n=12, 38.7%) and visual disturbance (n=2, 6.5%). Twenty-four patients (77.4%) had predisposing factors. The most common pathogens were gram-positive cocci (n=9, 29%). Seventeen patients (54.8%) had unfavorable outcome; 2 patients (6.4%) died. All patients were treated with parenteral antibiotherapy with median duration of 73 days (range, 28-540 days). Surgical procedures were performed in 83.9% (n=26) of patients [isolated aspiration (n=19, 61.3%), only resection (n=5, 16.1%), aspiration and resection (n=2, 6.5%)]. CONCLUSION: Glasgow coma scale score below 12 and the presence of FND on admission were found to be independent risk factors for unfavourable neurological outcome in children with brain abscesses.


Assuntos
Abscesso Encefálico/diagnóstico por imagem , Escala de Resultado de Glasgow/normas , Doenças do Sistema Nervoso/diagnóstico por imagem , Adolescente , Abscesso Encefálico/sangue , Abscesso Encefálico/complicações , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Doenças do Sistema Nervoso/sangue , Doenças do Sistema Nervoso/etiologia , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento
19.
Turk Pediatri Ars ; 52(3): 145-153, 2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29062248

RESUMO

AIM: Non-albicans Candida species and resistant microorganisms have been more commonly isolated in invasive candidiasis in recent years. The aim of this study was to evaluate the distrubution of Candida spp and antifungal resistance in our clinic. MATERIAL AND METHODS: Fifty-four Candida isolates and antifungal susceptibility results obtained from patients diagnosed as having invasive candidiasis between December 2012 and June 2016 were included. Clinical and laboratory data were retrospectively analyzed. E-test method was used in order to determine antifungal susceptibilities of Candida spp for amphotericin B, fluconazole, voriconazole, ketoconazole, itraconazole, anidulafungin, caspofungin, and flucytosine. RESULTS: The clinical diagnoses of the patients were candidemia (n=27, 50%), catheter-related blood stream infection (n=1, 1.8%), urinary tract infection (n=13, 24%), surgical site infection (n=4, 7.4%), intraabdominal infection (n=3, 5.5%), empyema (n=2, 3.7%), and pneumonia (n=4, 7.4%). The most common isolated agent was C. albicans (n=27, 50%) and the others were C. parapsilosis (n=13, 24%), C. tropicalis (n=6, 11.1%), C. glabrata (n=3, 5.6%), C. lusitaniae (n=2, 3.7%), and unspecified Candida spp. (n=3, 5.6%). Fluconazole resistance was 7.4% among all isolates. Resistance against itraconazole, ketoconazole, anidulafungin, voriconazole and caspofungin were 33.3%, 12.5%, 11.1%, 5%, and 2.5%, respectively. Isolates presented intermediate resistance against itraconazole (41.7%), voriconazole (5.6%), and amphotericin B (3.7%) to varying extents. All of the isolates were susceptible to flucytosine. CONCLUSIONS: In our clinic, C. albicans and non-albicans Candida species were equally distributed and antifungal susceptibilities against major antifungal agents such as fluconazole, amphotericin B, and caspofungin were found considerably high.

20.
Balkan Med J ; 34(3): 239-245, 2017 May 05.
Artigo em Inglês | MEDLINE | ID: mdl-28443591

RESUMO

BACKGROUND: Paediatric HIV infection is different from the adult type of disease in many ways, including transmission routes, clinical findings and treatment strategies. AIMS: To evaluate clinical data of paediatric patients with HIV disease. STUDY DESIGN: Retrospective cross-sectional study. METHODS: The charts of 22 paediatric patients diagnosed with HIV infection in our clinic during a 14 year period through 2001-2015 were retrospectively analysed. Clinical data, laboratory findings, treatment modalities and outcomes were recorded. RESULTS: The mean age of diagnosis 61.9±49.2 months and the mean follow-up period was 60.3±37.5 months. Seven patients (31.8%) were foreigners and the most common transmission route was vertical transmission (n=16, 72.7%). The most common presenting symptom and the sign were history of recurrent upper respiratory tract infections (n=8, 36.4%) and lymphadenopathy (n=12, 54.5%), respectively. Recurrent pneumonia (n=6, 27.3%), prolonged fever (n=5, 22.7%), recurrent otitis media (n=4, 18.2%), and gastroenteritis (n=4, 18.2%) were other clinical symptoms. Other than bacterial sinopulmonary infections, tuberculosis was the most frequent opportunistic infection (n=3, 13.6%). Mortality occurred in two patients (9.1%). CONCLUSION: Although mostly vertically transmitted, HIV infection may be diagnosed throughout the childhood. Frequently encountered signs and symptoms may be the reason for doctor admission. High clinical suspicion together with detailed anamnestic data and physical findings constitute the basis for pediatric HIV diagnosis.


Assuntos
Infecções por HIV/diagnóstico , Infecções por HIV/epidemiologia , Pediatria/estatística & dados numéricos , Criança , Pré-Escolar , Estudos Transversais , Feminino , Febre/epidemiologia , Febre/etiologia , Gastroenterite/epidemiologia , Gastroenterite/etiologia , Infecções por HIV/complicações , Humanos , Lactente , Transmissão Vertical de Doenças Infecciosas/estatística & dados numéricos , Linfadenopatia/epidemiologia , Linfadenopatia/etiologia , Masculino , Otite Média/epidemiologia , Otite Média/etiologia , Pneumonia/epidemiologia , Pneumonia/etiologia , Infecções Respiratórias/epidemiologia , Infecções Respiratórias/etiologia , Estudos Retrospectivos , Centros de Atenção Terciária/organização & administração , Centros de Atenção Terciária/estatística & dados numéricos , Turquia/epidemiologia
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