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BACKGROUND: Pediatric migraine prophylaxis is indicated when headaches are frequent and/or disabling. We aimed to conduct a study to compare the efficacy of cinnarizine and amitriptyline in pediatric migraine prophylaxis. METHODS: In a randomized, double-blind trial, patients aged 4-17 years with migraine who were eligible for prophylaxis enrolled. The primary outcome was a reduction response rate of ≥50% with p < 0.005 with respect to headache characteristics. The secondary outcome was migraine disability assessment. We evaluated patients every four weeks for three months: T1: week 4, T2: week 8 and T3: week 12. The safety profile was also assessed. RESULTS: Thirty patients were randomly assigned to each group. However, 43 patients completed the trial. Headache frequency decreased in amitriptyline group more effectively in T1 (p = 0.004). Amitriptyline was more successful in reducing the headache duration in all three periods (p < 0.005). There was no significant difference in severity improvement and reducing disability score between the two groups (p > 0.005). No serious adverse events were observed. CONCLUSIONS: Both medications are effective in ameliorating migraine headaches and related disabilities. However, amitriptyline appears be a preferable option over cinnarizine, given its faster onset of action, efficacy in reducing headache duration and longer-lasting effects.Trial Registration: The study was registered with the Iranian Registry of Clinical Trials (IRCT) under the code IRCT-20191112045413N1.
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Cinarizina , Transtornos de Enxaqueca , Humanos , Criança , Cinarizina/uso terapêutico , Amitriptilina/uso terapêutico , Irã (Geográfico) , Resultado do Tratamento , Transtornos de Enxaqueca/tratamento farmacológico , Transtornos de Enxaqueca/prevenção & controle , Transtornos de Enxaqueca/induzido quimicamente , Cefaleia/tratamento farmacológico , Analgésicos/uso terapêutico , Método Duplo-CegoRESUMO
BACKGROUND: Whole-exome sequencing (WES) provides a powerful diagnostic tool for identifying primary immunodeficiency diseases (PIDs). This study explores the utility of this approach in uncovering previously undiagnosed PIDs in children with community-acquired sepsis (CAS), with a medical history of recurrent infections or a family history of PIDs. METHODS: We performed WES on DNA samples extracted from the blood of the 34 enrolled patients, followed by bioinformatic analysis for variant calling, annotation, and prioritization. We also performed a segregation analysis in available family members to confirm the inheritance patterns and assessed the potential impact of the identified variants on protein function. RESULTS: From 34 patients enrolled in the study, 29 patients (85%) with previously undiagnosed genetic diseases, including 28 patients with PIDs and one patient with interstitial lung and liver disease, were identified. We identified two patients with severe combined immunodeficiency (SCID), patients with combined immunodeficiency (CID), six patients with combined immunodeficiency with syndromic features (CID-SF), four patients with defects in intrinsic and innate immunity, four patients with congenital defects of phagocyte function (CPDF), and six patients with the disease of immune dysregulation. Autoinflammatory disorders and predominantly antibody deficiency were diagnosed in one patient each. CONCLUSION: Our findings demonstrate the potential of WES in identifying undiagnosed PIDs in children with CAS. Implementing WES in the clinical evaluation of CAS patients with a warning sign for PIDs can aid in their timely diagnosis and potentially lead to improved patient care.
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Doenças da Imunodeficiência Primária , Sepse , Imunodeficiência Combinada Severa , Criança , Humanos , Sequenciamento do Exoma , Doenças da Imunodeficiência Primária/diagnóstico , Doenças da Imunodeficiência Primária/genética , Sepse/diagnóstico , Sepse/genética , Unidades de Terapia Intensiva PediátricaRESUMO
Due to the rapid growth of information technology and medical information resources, medical personnel need to search and retrieve valid and updated information. However, with limited time to access these resources, there is a need for clinical librarians to connect medical staff to evidence-based medicine (EBM). The present study was conducted to identify the challenges in the absence and the benefits of the presence of clinical librarians in the application of EBM in clinical departments. Ten clinical physicians working at Children's Medical Center Hospital in Tehran, Iran were interviewed for this qualitative study. Most of the hospital-based physicians did not make any systematic use of EBM and seven were not familiar with the term "clinical librarian." In their opinion, the clinical librarians' activities included training the clinical and research teams, providing them with the required information, and providing an EBM-oriented approach to morning report and educational rounds. Therefore, the services delivered by clinical librarians in various hospital departments might positively influence the hospital-based physicians' information-seeking behavior.
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Bibliotecários , Criança , Humanos , Papel Profissional , Irã (Geográfico) , Currículo , Medicina Baseada em Evidências/educaçãoRESUMO
Candida meningitis is a rare life-threatening yeast infection mostly involving immunocompromised or paediatric patients undergoing neurosurgical procedures or shunt placement. Due to difficulties in diagnosis because of diverse clinical manifestations, the number of patients affected is most likely underestimated. Therefore, the correct diagnosis may be delayed for months, and accurate species identification is highly recommended for administering appropriate antifungal therapy. We report the first case of fluconazole-resistant Candida auris meningitis in a paediatric patient in Iran. This strain was probably imported, as it genotypically belonged to Clade I from South Asia. Furthermore, we include a literature review of C auris meningitis cases, as the number of cases with C auris meningitis has increased with reports from the United Kingdom, India and Iran. This problem might increase further in the era of COVID-19 due to attrition of experienced healthcare personnel and a high workload of hospital healthcare workers. To understand the precise prevalence of this emerging multidrug resistance pathogen, epidemiological surveillance studies are urgently warranted.
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Candida auris , Candidíase/diagnóstico , Meningite , Antifúngicos/uso terapêutico , Criança , Humanos , Irã (Geográfico) , Meningite/diagnóstico , Meningite/microbiologia , Testes de Sensibilidade MicrobianaRESUMO
Introduction: In late February 2020, after we had informed about the presence of some cases of COVID-19 in Iran and its rapid spread throughout the country, we decided to make the necessary arrangements for patients with critical conditions in Pediatric Intensive Care Unit (PICU) at Children's Medical Center. There are a little data on critically ill children with COVID-19 infection with ICU requirements. The aim of this study was to describe clinical characteristics, laboratory parameters, treatment, and outcomes of the pediatrics population infected by SARS-CoV-2 admitted to PICU. Materials and Methods: This study was performed between February 2020 and May 2020 in the COVID PICU of the Children's Medical Center Hospital in Tehran, Iran. Patients were evaluated in terms of demographic categories, primary symptoms and signs at presentation, underlying disease, SARS-CoV-2 RT-PCR test result, laboratory findings at PICU admission, chest X-ray (CXR) and lung CT findings, and treatment. Moreover, the need to noninvasive ventilation (NIV) or mechanical ventilation, the length of hospital stay in the PICU, and outcomes were assessed. Results: In total, 99 patients were admitted to COVID PICU, 42.4% (42 patients) were males, and 66 patients had positive SARS-CoV-2 real-time reverse transcriptase-polymerase chain reaction (RT-PCR). There was no statistically significant difference in the frequency of clinical signs and symptoms (except for fever) among patients with positive SARS-CoV-2 RT-PCR and negative ones. Among all admitted patients, the presence of underlying diseases was noticed in 81 (82%) patients. Of 99 patients, 34 patients were treated with NIV during their admission. Furthermore, 35 patients were intubated and treated with mechanical ventilation. Unfortunately, 11 out of 35 mechanically ventilated patients (31%) passed away. Conclusion: No laboratory and radiological findings in children infected with COVID-19 were diagnostic in cases with COVID-19 admitted to PICU. There are higher risks of severe COVID-19, PICU admission, and mortality in children with comorbidities.
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COVID-19 , Criança , Masculino , Humanos , Feminino , COVID-19/epidemiologia , Pandemias , SARS-CoV-2 , Irã (Geográfico)/epidemiologia , Unidades de Terapia Intensiva Pediátrica , Hospitais , Encaminhamento e Consulta , Estudos Retrospectivos , Unidades de Terapia IntensivaRESUMO
OBJECTIVES: Children in the intensive care unit experience a high level of pain and anxiety. This study aimed to reveal the health care providers experience of non-pharmacological pain and anxiety management and its barriers in the pediatric intensive care unit. METHODS: This qualitative-descriptive study was conducted with thirteen nurses and five physicians in the pediatric intensive care unit in Iran. Individual, in-depth and semi-structured interviews were conducted, which were analyzed by conventional content analysis. RESULTS: Five main categories were identified from data analysis: 1) The importance of parents' presence, 2) Disturbance in the presence of parents and communication during the COVID-19 pandemic, 3) Choosing non-pharmacological approaches according to the child's interests and conditions, 4) Building trust in the child through non-pharmacological interventions 5) Barriers to non-pharmacological pain and anxiety management in the pediatric intensive care unit. CONCLUSION: Health care providers implement some non-pharmacological methods to manage pain and anxiety in the pediatric intensive care unit. Facilitating the open presence of parents, using innovative methods to communicate with children, and training and psychological support for nurses and parents, especially during the COVID-19 pandemic are recommended.
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COVID-19 , Pandemias , Ansiedade/terapia , Criança , Pessoal de Saúde , Humanos , Unidades de Terapia Intensiva Pediátrica , Dor , Pais , SARS-CoV-2RESUMO
The process monitoring of the top side and the evaluation of surface quality were applied in adjustable ring mode (ARM) laser welding of 316 stainless steel by using visual monitoring and confocal sensor technology. Experiments were conducted in different power arrangements of an ARM laser. In this study, the process status was evaluated based on the area of keyhole entrance and the width of the pool. The geometric characteristics of the topside weld were measured by a confocal sensor. The shape of the fusion zone was analyzed based on its cross section. The process mechanism of a different mode of ARM laser welding was investigated by analyzing the process status and geometric characteristics of the surface bead. Experimental results showed that dual-mode laser welding could stabilize the keyhole entrance with a uniform weld surface formation in comparison to the pure ring-mode laser weld. The dual-mode laser also generated a wider molten pool with a wider fusion zone than the pure center laser weld.
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Leukodystrophies are heterogeneous group of genetic white matter disorders with a wide range of neurologic and systemic manifestations. Defects in genes encoding aminoacyl tRNA (transfer ribonucleic acid) synthetase enzymes (aaRSs) are recently identified as the etiology of some leukodystrophies. Herein, we described two unrelated children referred to Children's Medical Center, Tehran, Iran, with developmental delay, nystagmus, seizures, psuedo-bulbar palsy and dystonia. Whole exome sequencing (WES) in both patients identified a homozygous (c.2T > C) variant in exon one of RARS gene, encoding cytoplasmic arginyl-tRNA synthetase. Our finding was confirmed by segregation analysis. In silico analyses of the c.2T > C variant showed its possible pathogenic role due to the absence of the start codon. Severe hypomyelination was the common neuroimaging finding of both cases. Spinal cord involvement was found in one of our patients which was not previously reported in studies. We, therefore, showed that RARS-related hypomyelination might affect spinal cord.
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Aminoacil-tRNA Sintetases/genética , Variação Genética/genética , Doenças Desmielinizantes Hereditárias do Sistema Nervoso Central/diagnóstico por imagem , Doenças Desmielinizantes Hereditárias do Sistema Nervoso Central/genética , Medula Espinal/diagnóstico por imagem , Aminoacil-tRNA Sintetases/química , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Estrutura Secundária de ProteínaRESUMO
OBJECTIVES: To assess the effect of the Infant intensive care unit (InICU), a specialized unit for critically ill infants established in 2016 in authors' hospital, on infant mortality and compare the outcome with the current Pediatric intensive care units (PICU). METHODS: In this retrospective cross-sectional study, two groups were defined; the first included expired patients aged 1 mo to 2 y admitted to PICU before the establishment of the InICU (the PICU group). The second included age-matched expired patients admitted to the InICU (the InICU group). Data were recorded using a questionnaire. RESULTS: The authors found that the age, sex, underlying diseases, the leading cause of admission to the ICU, time of death, hospital and ICU length of stay, and the pediatric index of mortality 2 (PIM 2) score were same between the two groups. The incidence of mortality in the PICU group was 10.66 in 1000 person-month. This value was 6.37 for the InICU group (P-value <0.001). The relative risk of mortality of patients admitted to the PICU group compared to the InICU group was 1.67 (P-value <0.001). CONCLUSIONS: Establishment of age specific InICU for infants may be beneficial in reducing infant mortality.
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AIM: The aim of this network meta-analysis (NMA) was to investigate the effects of different dietary supplements on the mortality and clinical status of adults with sepsis. METHODS: We searched PubMed, EMBASE, and the Cochrane Library Central Register of Controlled Trials until February 2023. The inclusion criteria were: 1) randomized controlled trials (RCT)s; 2) adults suffering sepsis or septic shock; 3) evaluation of short- or long-mortality; and 4) publications between 1994 and 2023. The general information of studies and details of interventions were extracted. The primary outcome was short-term mortality (<90 days), and the secondary outcomes were long-term mortality (≥90 days), length of ICU and hospital stays, and duration of mechanical ventilation (MV). The risk of bias of RCTs was assessed using the Cochrane risk of bias tool 2 (ROB2). A random effect NMA was performed to rank the effect of each intervention using a frequentist approach. RESULTS: Finally, 56 RCTs with 5957 participants met the criteria. Approximately, one-third of RCTs were low risk of bias. NMA analysis revealed that there was no treatment more effective in short- or long-term mortality than control or other interventions, except for magnesium (RR: 0.33, 95% CI: 0.14, 0.79; GRADE = low) and vitamin C (RR: 0.81, 95% CI: 0.67, 0.99; low certainty evidence), which had beneficial effects on short-term mortality. Moreover, eicosapentaenoic acid, gamma-linolenic acid, and antioxidants (EPA + GLA + AOs) combination was the most effective, and magnesium, vitamin D and vitamin C were the other effective approaches in terms of duration of MV, and ICU length of stay. There was no beneficial dietary supplement for hospital stay in these patients. CONCLUSIONS: In septic patients, none of the dietary supplements had a substantial effect on mortality except for magnesium and vitamin C, which were linked to lower short-term mortality with low certainty of evidence. Further investigation into high-quality studies with the use of dietary supplements for sepsis should be highly discouraged.
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Suplementos Nutricionais , Metanálise em Rede , Sepse , Choque Séptico , Humanos , Sepse/mortalidade , Sepse/terapia , Sepse/tratamento farmacológico , Choque Séptico/mortalidade , Choque Séptico/terapia , Choque Séptico/tratamento farmacológico , Ensaios Clínicos Controlados Aleatórios como Assunto , Resultado do Tratamento , Tempo de Internação/estatística & dados numéricos , Adulto , Respiração Artificial/mortalidadeRESUMO
Candidemia caused by rare and uncommon Candida species is becoming more prevalent in pediatric healthcare settings, resulting in significant morbidity and mortality. One such species, Candida palmioleophila, is resistant to fluconazole but highly susceptible to echinocandins. Here, we report the first documented case of C. palmioleophila candidemia in Iran that occurred in a male infant with biliary atresia who had been hospitalized for 2 months. The patient's blood and urine cultures were positive for both yeast and bacterial species. Through DNA sequence analysis, the yeast isolate was identified as C. palmioleophila. In vitro antifungal susceptibility testing of the isolate against amphotericin B, fluconazole, itraconazole, voriconazole, isavuconazole, posaconazole, and nystatin revealed MIC values of 2, 16, 0.25, 0.0625, 0.125, 0.25, and 4 µg/mL, respectively, and minimum effective concentration for caspofungin was 0.031 µg/mL. Despite receiving antibacterial and antifungal therapies, the patient unfortunately expired due to bradycardia and hypoxemia. Proper identification and epidemiological surveillance studies are needed to understand the exact prevalence of these emerging yeast pathogens. Previously reported cases of C. palmioleophila infection, primarily associated with bloodstream infections and catheter-related candidemia, were reviewed.
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Infecções Bacterianas , Atresia Biliar , Candidemia , Coinfecção , Humanos , Lactente , Masculino , Antifúngicos/farmacologia , Antifúngicos/uso terapêutico , Infecções Bacterianas/tratamento farmacológico , Atresia Biliar/tratamento farmacológico , Candida/genética , Candidemia/diagnóstico , Candidemia/tratamento farmacológico , Coinfecção/tratamento farmacológico , Fluconazol , Testes de Sensibilidade Microbiana , Saccharomyces cerevisiaeRESUMO
Despite the fact that unscheduled readmission to pediatric intensive care units (PICUs) has significant adverse consequences, there is a need for a predictive tool appropriate for use in the clinical setting. The aim of this study was to assess the ability of the modified Brighton pediatric early warning score (PEWS) to identify children at high risk for early unplanned readmission. In this retrospective cohort study, all patients aged 1 month to 18 years of age discharged from PICUs of two tertiary children's hospitals during the study interval were enrolled. Apart from demographic data, the association between PEWS and early readmission, defined as readmission within 48 hours of discharge, was analyzed by multivariable logistic regression. From 416 patients, 27 patients had early PICU readmission. Patients who experienced readmission were significantly younger than the controls. (≤12 months, 70.4 vs. 39.1%, p = 0.001) Patients who were admitted from the emergency room (66.7 and 33.3% for emergency department (ED) and floor, respectively, p = 0.012) had higher risk of early unplanned readmission. PEWS at discharge was significantly higher in patients who experienced readmission (3.07 vs. 0.8, p < 0.001). A cut-off PEWS of 2, with sensitivity 85.2% and specificity 78.1%, determined the risk of unplanned readmission. Each 1-point increase in the PEWS at discharge significantly increases the risk of readmission (odds ratio [OR] = 3.58, 95% confidence interval [CI]: [2.42-5.31], p < 0.001). PEWS can be utilized as a useful predictive tool regarding predicting unscheduled readmission in PICU. Further large-scale studies are needed to determine its benefits in clinical practice.
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BACKGROUND: COVID-19 infection and its neurological manifestations were seen in children although less common than adults. The aim of this study was to determine the frequency of different types of neurologic findings of hospitalized children with COVID-19. ]. METHODS: This retrospective study was performed on hospitalized pediatric patients aged≤18 years with confirmed SARS-CoV-2 at Children's Medical Center Hospital. Neurological manifestations were defined as the presence of any of the following symptoms: seizure, altered mental status, behavioral/personality change, ataxia, stroke, muscle weakness, smell and taste dysfunctions, and focal neurological disorders. RESULTS: Fifty-four children with COVID-19 were admitted and their mean age was 6.94±4.06 years. Thirty-four of them (63%) were male. The most frequent neurological manifestation was seizure (19 [45%]) followed by muscle weakness (11 [26%]), loss of consciousness (10 [23%]), and focal neurological disorders (10 [23%]). Other neurological manifestations consisted of headache (n=7), movement disorders (n=6), behavioral/personality change (n=5), ataxia (n=3), and stroke (n=3). Twenty-nine percent of our patients had leukocytosis. A neutrophil count above 70% was seen in 31% of participants. Among our patients, 81% had a positive reverse-transcription polymerase chain reaction (RT-PCR) test for SARS-CoV-2. CONCLUSION: During the current pandemic outbreak, hospitalized children with COVID-19 should be evaluated for neurological signs because it is common among them and should not be under-estimated.
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COVID-19 , Acidente Vascular Cerebral , Adulto , Humanos , Masculino , Criança , Pré-Escolar , Feminino , COVID-19/epidemiologia , SARS-CoV-2 , Irã (Geográfico)/epidemiologia , Estudos Retrospectivos , Convulsões , Ataxia/etiologia , HospitaisRESUMO
BACKGROUND: The integration of paediatric palliative care into the Iranian health system is essential. AIMS: The aim of this study was to identify the challenges of palliative care in the paediatric intensive care unit during COVID-19 through the experiences of healthcare providers. METHODS: A qualitative study with content analysis approach was conducted. Fifteen physicians and nurses were selected by purposeful sampling. The semi-structured, in-depth interviews were applied in the data collection. FINDINGS: Ten main categories were extracted from data analysis, including 'caring in COVID-19', 'communication and family centre care', 'breaking bad news', palliative care training', 'pain and symptom management', 'support of the child, family and clinical team', 'physical environment', 'guidelines', 'specialised staff' and 'home based palliative care'. CONCLUSION: Palliative care in the PICU faces several challenges, especially during COVID-19, but the clinical team are making every attempt to improve the comprehensive care of children and their families. Telehealth is important in COVID-19, and education is also a key component to improve palliative care in the PICU in Iran.
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COVID-19 , Cuidados Paliativos , Pandemias , Criança , Humanos , Unidades de Terapia Intensiva , Irã (Geográfico) , Pesquisa Qualitativa , SARS-CoV-2RESUMO
Coronavirus disease 2019 is the third zoonotic acute respiratory disease after SARS virus and Middle East respiratory syndrome. Most cases are mild in healthy children. In contrast, the infection is more severe in patients with underlying health conditions. Because there are few posttransplant reports in hematopoietic stem celltransplant patients, here we described COVID19 infection in 4 confirmed cases among pediatric hematopoietic stem cell transplant recipients: 3 boys and 1 girl with a median age of 6 years. Three patients presented with symptoms of lower respiratory tract disease, whereas 1 patient presented with extrapulmonary symptoms without fever or pulmonary involvement. All of the patients were on immunosuppressivedrugs, ie, 1patientforgraft-versus-hostdisease prophylaxis and 3 patients for graft-versus-host disease treatment.Thosewhowerediagnosedwith active graftversus-hostdisease requiredmechanical ventilationand intensive care. Two patients died from multiple organ dysfunction and resistant coinfection, and 1 patient developed pulmonary hypertension and mild cardiomegaly and remained at the hospital for more than 2 months, whereas the patient with no graft-versus-host disease was discharged and recovered. Our findings showed that COVID-19 infection among hematopoietic stem cell transplant recipients may be more severe and associatedwithlong-termhospitalization and complications. Active graft-versus-hostdisease, coinfections, and long-term use of immunosuppressive agents are risk factors for poor outcomes.
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COVID-19/imunologia , COVID-19/terapia , Doença Enxerto-Hospedeiro/imunologia , Transplante de Células-Tronco Hematopoéticas , Imunossupressores/uso terapêutico , SARS-CoV-2/fisiologia , Transplantados , Criança , Pré-Escolar , Evolução Fatal , Feminino , Doença Enxerto-Hospedeiro/prevenção & controle , Hospitalização , Humanos , Masculino , Respiração Artificial , Fatores de RiscoRESUMO
It is about half a century since free-living amoebae were recognized as pathogenic organisms, but there is still much we should learn about these rare fatal human infectious agents. A recently introduced causative agent of granulomatous amoebic encephalitis, Balamuthia mandrillaris, has been reported in a limited number of countries around the world. A 3-year-old girl was referred to our tertiary hospital because of inability to establish a proper diagnosis. She had been experiencing neurologic complaints including ataxia, altered level of consciousness, dizziness, seizure, and left-sided hemiparesis. The patient's history, physical examination results, and laboratory investigations had led to a wide differential diagnosis. Computed tomography (CT) scan and magnetic resonance imaging analyses revealed multiple mass lesions. As a result, the patient underwent an intraoperative frozen section biopsy of the brain lesion. The frozen section study showed numerous cells with amoeba-like appearances in the background of mixed inflammatory cells. Medications for free-living amoebic meningoencephalitis were administered. PCR assay demonstrated B. mandrillaris as the pathogenic amoeba. Unfortunately, the patient died 14 days after her admission. To our knowledge, this is the first report of B. mandrillaris meningoencephalitis in the Middle East and the first time we have captured the organism during a frozen-section study.
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Amebíase/parasitologia , Ataxia/parasitologia , Balamuthia mandrillaris/patogenicidade , Infecções Protozoárias do Sistema Nervoso Central/parasitologia , Tontura/parasitologia , Paresia/parasitologia , Convulsões/parasitologia , Amebíase/diagnóstico por imagem , Amebíase/patologia , Ataxia/diagnóstico por imagem , Ataxia/patologia , Balamuthia mandrillaris/crescimento & desenvolvimento , Biópsia , Infecções Protozoárias do Sistema Nervoso Central/diagnóstico por imagem , Infecções Protozoárias do Sistema Nervoso Central/patologia , Pré-Escolar , Tontura/diagnóstico por imagem , Tontura/patologia , Evolução Fatal , Feminino , Humanos , Irã (Geográfico) , Imageamento por Ressonância Magnética , Paresia/diagnóstico por imagem , Paresia/patologia , Convulsões/diagnóstico por imagem , Convulsões/patologia , Tomografia Computadorizada por Raios XRESUMO
To determine the prevalence of vaccine-associated paralytic poliomyelitis (VAPP) in immunodeficient infants, we reviewed all documented cases caused by immunodeficiency-associated vaccine-derived polioviruses in Iran from 1995 through 2008. Changing to an inactivated polio vaccine vaccination schedule and introduction of screening of neonates for immunodeficiencies could reduce the risk for VAPP infection.
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Síndromes de Imunodeficiência/complicações , Poliomielite/etiologia , Vacina Antipólio Oral/efeitos adversos , Pré-Escolar , Feminino , Humanos , Lactente , Irã (Geográfico) , Masculino , Fatores de TempoRESUMO
UNLABELLED: Patients with CVID are at greater risk of developing lung complications than patients with XLA because of delayed diagnosis and possible immune dysregulation. Early diagnosis and appropriate treatment reduces the incidence of pulmonary infections in both groups of patients. However, CVID patients are prone to progressive lung disease despite optimal immunoglobulin therapy. BACKGROUND AND OBJECTIVE: Pulmonary disease is the most common complication in patients with common variable immunodeficiency (CVID) or X-linked agammaglobulinaemia (XLA). Pulmonary disease may progress despite immunoglobulin replacement therapy. In this study pulmonary complications were compared in patients with CVID or XLA. METHODS: Pulmonary complications were evaluated in 115 patients (76 with CVID and 39 with XLA) by reviewing hospital records of chest infections, pulmonary function tests and high-resolution CT scans. RESULTS: Thirty-two patients with XLA (82%) presented with 59 episodes of pneumonia before diagnosis, whereas 15 patients (38.4%) experienced pneumonia after immunoglobulin replacement therapy (1.67 vs 0.45 episodes per patient per year). Among the CVID patients, 196 episodes of pneumonia were documented in 59 patients (77.6%) before diagnosis, while 36 patients (47.3%) experienced pneumonia after therapy (1.11 vs 0.58 episodes of pneumonia per patient per year). Forty-seven (41%) patients (38 with CVID and 9 with XLA) developed chronic lung disease. The CVID patients developed more complications, including bronchiectasis and lymphoid interstitial pneumonitis, than the XLA patients. CONCLUSIONS: Patients with CVID had a greater likelihood of developing lung disease, possibly due to delayed diagnosis and immune dysregulation, as compared with XLA patients. Early diagnosis of patients with primary antibody deficiencies and adequate i.v. immunoglobulin replacement therapy substantially reduces the number of pulmonary infections. However, CVID patients are prone to progression of lung disease despite optimal immunoglobulin therapy because of the nature of the disease. This important issue should be addressed in further studies.
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Agamaglobulinemia/complicações , Agamaglobulinemia/diagnóstico , Imunodeficiência de Variável Comum/complicações , Imunodeficiência de Variável Comum/diagnóstico , Doenças Genéticas Ligadas ao Cromossomo X/complicações , Doenças Genéticas Ligadas ao Cromossomo X/diagnóstico , Pneumopatias/epidemiologia , Adolescente , Adulto , Agamaglobulinemia/terapia , Bronquiectasia/epidemiologia , Criança , Pré-Escolar , Imunodeficiência de Variável Comum/terapia , Progressão da Doença , Feminino , Doenças Genéticas Ligadas ao Cromossomo X/terapia , Humanos , Imunização Passiva , Doenças Pulmonares Intersticiais/epidemiologia , Masculino , Pessoa de Meia-Idade , Pneumonia/epidemiologia , Prevalência , Testes de Função Respiratória , Estudos Retrospectivos , Fatores de Risco , Adulto JovemRESUMO
Meningiomas are slow-growing neoplasms which recur locally, their morphologic grading is simple but do not always correlate with patient outcome. The aim of present study is to evaluate the status of progesterone receptor (PR) and proliferation marker Ki67 in various grades of meningioma in a group of Iranian patients. 78 cases of meningioma were selected from the file of a hospital university. All archival H&E stained sections were reviewed and regraded according to WHO criteria. Immunohistochemical analysis for PR and Ki67 was performed on formalin-fixed, paraffin-embedded samples. PR status considered positive if >10% of tumor cell's nuclei were strongly immunoreactive, or if >50% of nuclei were stained with medium intensity. The Ki67 labeling index (LI) is defined as the percentage area with strongest immunostaining. PR were positive in 61/63 (96.8%) of grade I tumors, 2/10 (20%) of grade II, and 0/5 (0%) of grade III tumors. Ki67 LI was %2.98 +/- 2.27 in grade I tumors, %9.30 +/- 5.79 in grade II tumors and %34.00 +/- 5.47 in grade III tumors. For both markers, differences between grade I, II and III tumors were significant (P < 0.001). There was a reverse relationship between mean of Ki67 LI and PR status, with increasing grade of tumor. Evaluation of PR status and Ki67 LI together with conventional histologic evaluation can help in providing more information about the biologic behaviour of meningiomas, especially for those that histological grading is not straightforward.
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Antígeno Ki-67/metabolismo , Neoplasias Meníngeas/metabolismo , Meningioma/metabolismo , Receptores de Progesterona/metabolismo , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores Tumorais/metabolismo , Distribuição de Qui-Quadrado , Feminino , Humanos , Imuno-Histoquímica , Irã (Geográfico) , Masculino , Neoplasias Meníngeas/patologia , Meningioma/patologia , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Coloração e RotulagemRESUMO
BACKGROUND: Hemodynamic monitoring is an essential part in the treatment of critically ill patients. Establishment of intravascular volume and creation of a normal systemic perfusion are the most important part to reduce the risk of organ failure and mortality. This study aimed to determine the correlation between the inferior vena cava (IVC) sonographic indices and the central venous pressure (CVP) to provide a useful guide for noninvasive intravascular volume status assessment in children. MATERIALS AND METHODS: Target sample of children who were admitted to the pediatric critical care unit and required CVP monitoring were enrolled in this study. The collapsibility index (CI) and IVC/aorta (AO) ratio, from bedside ultrasonography measurement of the IVC, were calculated. RESULTS: Of the 70 participants, 22 patients (31.4%) revealed a CVP of 8 mm/Hg or less and 48 patients (68.6%) revealed a CVP >8 mm/Hg. Fifty-six patients (80%) had an IVC-CI of 0.5 or greater and 17 patients (24.3%) had an IVC/AO of 0.8 or less. IVC-CI index is 45.5% sensitive and 91.7% specific with positive predictive value of 71.4 and negative predictive value of 78.6 to predict CVP <8, and the IVC/AO index is 50.8% sensitive and 87.5% specific with a positive predictive value of 64.7 and a negative predictive value of 79.2 to predict CVP <8. CONCLUSION: Based on the present finding, the IVC sonographic indices provide a useful guide for noninvasive intravascular volume status assessment in children.