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1.
Pediatr Crit Care Med ; 23(5): 399-404, 2022 05 01.
Artículo en Inglés | MEDLINE | ID: mdl-35583619

RESUMEN

OBJECTIVES: To determine the prevalence and time course of thiamine deficiency (TD) in PICU patients. DESIGN: Multicenter, prospective, cohort study between May 2019 and November 2019. SETTING: Three university-based tertiary care, mixed medical-surgical PICUs in Ankara, Turkey. PATIENTS: PICU patients 1 month to 18 years old. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We studied 476 patients and grouped them by TD status on days 1 and 3 of the PICU admission. There might be a risk of unintended bias since we excluded 386 patients because of the absence of consent, inadequate blood samples, loss of identifier information, and recent vitamin supplementation. On day 1, TD was present in 53 of 476 patients (11.1%) and median (minimum-maximum) thiamine levels were 65.5 ng/mL (5-431 ng/mL). On day 3, TD was present in 27 of 199 patients (13.6%) with repeated measurement. The median (minimum-maximum) thiamine levels were 63 ng/mL (13-357 ng/mL). The time course of TD from day 1 to day 3 in these 199 patients was as follows. In 21 of 199 patients (10.6%) with TD on day 1, 11 of 21 (52%) continued to have TD on day 3 and the other 10 of 21 patients (48%) improved to no longer having TD. In 178 of 199 patients (89.4%) without TD on day 1, 16 of 178 (9%) went on to develop TD by day 3, and the other 162 of 178 (91%) continued to have normal thiamine status. CONCLUSIONS: In the PICU population in three centers in Turkey, the prevalence of TD in the sample of patients was 11.1%. In those TD patients who had serial studies, we also identified that by day 3 some continued to be TD, and some patients improved to normal thiamine status. Of concern, however, is the population who develop TD over the course of PICU stay.


Asunto(s)
Enfermedad Crítica , Deficiencia de Tiamina , Niño , Estudios de Cohortes , Humanos , Unidades de Cuidado Intensivo Pediátrico , Prevalencia , Estudios Prospectivos , Tiamina , Deficiencia de Tiamina/epidemiología , Turquía/epidemiología
2.
Pediatr Emerg Care ; 37(11): e775-e778, 2021 Nov 01.
Artículo en Inglés | MEDLINE | ID: mdl-34731879

RESUMEN

ABSTRACT: Central venous catheters are commonly used in emergency department and the intensive care units for the treatment of critically ill patients. Reports on the rate of mechanical complications of catheter insertion are around 1%. Mechanical complications related with the insertion of the catheter mainly depend on the anatomic location, the use of ultrasound, and experience of the operator. The rate of complication is higher in places where central lines are not routinely inserted as in emergency department. Here, we report a case series of 4 patients who had life-threatening complications of central venous catheter insertion, including venous intimal injury leading to pleural effusion, kinked central venous catheter in the superior vena cava, knotted guidewire with the separation of coil and core, and a missed guidewire with their respective insertion history and imaging. Increasing the awareness of and developing strategies to prevent these rare but potentially fatal conditions may have impact on patient outcomes.


Asunto(s)
Cateterismo Venoso Central , Catéteres Venosos Centrales , Cateterismo Venoso Central/efectos adversos , Catéteres Venosos Centrales/efectos adversos , Enfermedad Crítica , Humanos , Unidades de Cuidados Intensivos , Vena Cava Superior
3.
Pediatr Emerg Care ; 37(12): e955-e961, 2021 Dec 01.
Artículo en Inglés | MEDLINE | ID: mdl-33170574

RESUMEN

OBJECTIVE: The objective of this study was to detect variables associated with burnout syndrome (BS) in pediatric intensive care units (PICUs) and pediatric emergency medicine departments (PEDs) in high-volume centers from different parts of Turkey. METHODS: An observational, cross-sectional multicenter study was performed. The Maslach Burnout Inventory scale was administered to all of health care providers working in PICUs and PEDs. In this study, health care providers were defined as physicians, nurses, and other staff (secretaries, cleaning and patient care staff) working in PICU and PEDs. RESULTS: A total of 570 participants completed the survey. The major finding of this study was that 76.1% (n = 434) of PICU and PED health care professionals had BS. The most prominent subscale of BS was emotional exhaustion (62.5%). The rate of BS was higher among health care providers working in PEDs compared with PICUs (79.1% vs 73.7%, P = 0.04). The frequency of BS according to emotional exhaustion and depersonalization subscales was higher in health care providers of PEDs. The rate of BS was also significantly higher in younger employees, females, those working 51 or more hours totally in a week, those having a low monthly salary, those single or divorced, those without children, those with no childcare at home, those not owning a home, those not doing regular exercise and not having regular breakfast, those with total employment time of less than 1 year, and those not having a car or not having a hobby. In PEDs, when the daily evaluated number of patients was equal to or more than 44 (sensitivity, 88%; specificity, 66%), it predicted the occurrence of BS. In PICUs, when the number of patients cared for by 1 nurse was equal to or more than 3, it predicted the occurrence of BS (sensitivity, 78%; specificity, 62%). CONCLUSIONS: By creating early intervention programs to prevent BS, shortages of health care professionals can be avoided and the costs of health care expenditures related to infections can be decreased.


Asunto(s)
Agotamiento Profesional , Médicos , Agotamiento Profesional/epidemiología , Niño , Estudios Transversales , Servicio de Urgencia en Hospital , Femenino , Humanos , Unidades de Cuidado Intensivo Pediátrico
4.
Minerva Pediatr ; 71(4): 349-357, 2019 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-31268280

RESUMEN

BACKGROUND: Infections due to carbapenem resistant pathogens have become a major health threat especially for hospitalized patients. Acinetobacter baumanii (AB) and Pseudomonas aeruginosa (PA) are important pathogens causing ventilator-associated pneumonia (VAP) with a trend of high resistance to carbapenems. The aim of this study is to investigate the risk factors for VAP due to carbapenem resistant Acinetobacter baumanii (CRAB) and Pseudomonas aeruginosa (CRPA) in children. METHODS: Between 2009 and 2013, an active, prospective observational study was conducted in Gazi University Hospital. Patients from Pediatric Intensive Care Unit (PICU), between 1 month and 12 years of age with VAP due to AB and PA were included. RESULTS: During this period, 74 children experienced 126 VAP episodes due to Acinetobacter baumanii (N.=58) and Pseudomonas aeruginosa (N.=68). Among these, 93.1% (N.=54) of AB and 51.5% (N.=35) of PA were carbapenem resistant. In univariate analysis, length of stay in PICU until the diagnosis of VAP, presence of central venous catheters, prior use of cefepime, ciprofloxacin, colistin, and teicoplanin were associated with VAP due to CRPA (P=0.02, P=0.02, P=0.006, P=0.01, P=0.001, and P=0.009 respectively). Significant association was not found between the development of VAP due to CRAB and the investigated risk factors. Regression analyses revealed previous use of cefepime (OR, 2.11; 95% CI, 0.016-0.595, P=0.039) and colistin (OR: 2.33; 95% CI: 0.061-0.789, P=0.023) to be independently associated with VAP due to CRPA. CONCLUSIONS: This study suggests that broad spectrum antibiotic usage was the most important risk factor for the development of VAP due to CRPA.


Asunto(s)
Infecciones por Acinetobacter/epidemiología , Antibacterianos/farmacología , Neumonía Asociada al Ventilador/epidemiología , Infecciones por Pseudomonas/epidemiología , Acinetobacter baumannii/aislamiento & purificación , Antibacterianos/administración & dosificación , Carbapenémicos/farmacología , Niño , Preescolar , Farmacorresistencia Bacteriana , Femenino , Humanos , Lactante , Unidades de Cuidado Intensivo Pediátrico , Tiempo de Internación , Masculino , Neumonía Asociada al Ventilador/microbiología , Estudios Prospectivos , Pseudomonas aeruginosa/aislamiento & purificación , Factores de Riesgo
5.
Indian J Crit Care Med ; 23(6): 263-269, 2019 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-31435144

RESUMEN

OBJECTIVES: To analyze the course of seasonal viral infections of respiratory tract in patients hospitalized in pediatric intensive care units (PICU) of 16 centers in Turkey. MATERIALS AND METHODS: It is a retrospective, observational, and multicenter study conducted in 16 tertiary PICUs in Turkey includes a total of 302 children with viral cause in the nasal swab which required PICU admission with no interventions. RESULTS: Median age of patients was 12 months. Respiratory syncytial virus (RSV) was more common in patients over one year of age whereas influenza, human Bocavirus in patients above a year of age was more common (p <0.05). Clinical presentations influencing mortality were neurologic symptoms, tachycardia, hypoxia, hypotension, elevated lactate, and acidosis. The critical pH value related with mortality was ≤7.10, and critical PCO2 ≥60 mm Hg. CONCLUSION: Our findings demonstrate that patients with neurological symptoms, tachycardia, hypoxia, hypotension, acidosis, impaired liver, and renal function at the time of admission exhibit more severe mortal progressions. Presence of acidosis and multiorgan failure was found to be predictor for mortality. Knowledge of clinical presentation and age-related variations among seasonal viruses may give a clue about severe course and prognosis. By presenting the analyzed data of 302 PICU admissions, current study reveals severity of viral respiratory tract infections and release tips for handling them. HOW TO CITE THIS ARTICLE: Kockuzu E, Bayrakci B, Kesici S, Citak A, Karapinar K, Emeksiz S, et al. Comprehensive Analysis of Severe Viral Infections of Respiratory Tract admitted to PICUs During the Winter Season in Turkey. Indian J Crit Care Med 2019;23(6):263-269.

6.
J Paediatr Child Health ; 54(5): 480-486, 2018 May.
Artículo en Inglés | MEDLINE | ID: mdl-29278447

RESUMEN

AIM: Although early enteral nutrition (EN) is strongly associated with lower mortality in critically ill children, there is no consensus on the definition of early EN. The aim of this study was to evaluate our current practice supplying EN and to identify factors that affect both the initiation of feeding within 24 h after paediatric intensive care unit (PICU) admission and the adequate supply of EN in the first 48 h after PICU admission in critically ill children. METHODS: We conducted a prospective, multicentre, observational study in nine PICUs in Turkey. Any kind of tube feeding commenced within 24 h of PICU admission was considered early initiated feeding (EIF). Patients who received more than 25% of the estimated energy requirement via enteral feeding within 48 h of PICU admission were considered to have early reached target EN (ERTEN). RESULTS: Feeding was initiated in 47.4% of patients within 24 h after PICU admission. In many patients, initiation of feeding seems to have been delayed without an evidence-based reason. ERTEN was achieved in 43 (45.3%) of 95 patients. Patients with EIF were significantly more likely to reach ERTEN. ERTEN was an independent significant predictor of mortality (P < 0.001), along with reached target enteral caloric intake on day 2 associated with decreased mortality. CONCLUSIONS: There is a substantial variability among clinicians' perceptions regarding indications for delay to initiate enteral feeding in critically ill children, especially after the first 6 h of PICU admission. ERTEN, but not EIF, is associated with a significantly lower mortality rate in critically ill children.


Asunto(s)
Cuidados Críticos/métodos , Enfermedad Crítica/terapia , Nutrición Enteral/métodos , Pautas de la Práctica en Medicina/estadística & datos numéricos , Adolescente , Niño , Preescolar , Cuidados Críticos/estadística & datos numéricos , Enfermedad Crítica/mortalidad , Nutrición Enteral/estadística & datos numéricos , Femenino , Humanos , Lactante , Unidades de Cuidado Intensivo Pediátrico , Modelos Logísticos , Masculino , Estudios Prospectivos , Factores de Tiempo , Resultado del Tratamiento , Turquía
7.
Clin Lab ; 62(5): 751-5, 2016.
Artículo en Inglés | MEDLINE | ID: mdl-27348998

RESUMEN

BACKGROUND: The aim of this study was to evaluate the diagnostic value of Upar, IL-33, and ST2 in comparison with C-reactive protein, TNF-α, and Interleukin-6 in childhood sepsis. METHODS: A total of 128 children were included and 20 of them were the control group. We used only data showing a high probability of sepsis with blood culture positive children, because of this reason 68 children were excluded. Blood was collected from children from first day of sepsis (1st value) and 48 - 72 hours later (2nd value). RESULTS: There were significant differences between control and sepsis (1st value) for IL-33 levels (1.1 ± 0.28 ng/ mL and 5.23 ± 1.80 ng/mL, p = 0.01), for sST2 levels (6.73 ± 5.3 ng/mL and 53.23 ± 28.30 ng/mL, p = 0.01), for sUpar levels (3.3 ± 1.7 ng/mL and 15.2 ± 6.3 ng/mL, p = 0.01), respectively. There were significant differences between sepsis (1st value) and sepsis (2nd value) for IL-33 levels, for sST2 levels, and for suPAR levels. CONCLUSIONS: In the light of these results, it may be suggested that Upar, IL-33, and ST2 can be used as an acute phase reactant like C-reactive protein, TNF-α, and Interleukin-6 in the diagnosis of childhood sepsis.


Asunto(s)
Interleucina-33/sangre , Receptores de Superficie Celular/sangre , Receptores del Activador de Plasminógeno Tipo Uroquinasa/sangre , Sepsis/diagnóstico , Niño , Preescolar , Femenino , Humanos , Proteína 1 Similar al Receptor de Interleucina-1 , Masculino , Sepsis/sangre
8.
Pediatr Emerg Care ; 31(11): 748-52, 2015 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-26535496

RESUMEN

OBJECTIVES: The objectives of this study were to determine the causes, location of cardiopulmonary arrest (CPA) in children, and demographics of cardiopulmonary resuscitation (CPR) in Turkish pediatric emergency departments and pediatric intensive care units (PICUs) and to determine survival rates and morbidities for both in-hospital and out-of-hospital CPA. METHODS: This multicenter descriptive study was conducted prospectively between January 15 and July 15, 2011, at 18 centers (15 PICUs, 3 pediatric emergency departments) in Turkey. RESULTS: During the study period, 239 children had received CPR. Patients' average age was 42.4 (SD, 58.1) months. The most common cause of CPA was respiratory failure (119 patients [49.8%]). The location of CPA was the PICU in 168 (68.6%), hospital wards in 43 (18%), out-of-hospital in 24 (10%), and pediatric emergency department in 8 patients (3.3%). The CPR duration was 30.7 (SD, 23.6) minutes (range, 1-175 minutes) and return of spontaneous circulation was achieved in 107 patients (44.8%) after the first CPR. Finally, 58 patients (24.2%) were discharged from hospital; survival rates were 26% and 8% for in-hospital and out-of-hospital CPA, respectively (P = 0.001). Surviving patients' average length of hospital stay was 27.4 (SD, 39.2) days. In surviving patients, 19 (32.1%) had neurologic disability. CONCLUSION: Pediatric CPA in both the in-hospital and out-of-hospital setting has a poor outcome.


Asunto(s)
Reanimación Cardiopulmonar , Paro Cardíaco/terapia , Preescolar , Servicio de Urgencia en Hospital , Femenino , Paro Cardíaco/etiología , Paro Cardíaco/mortalidad , Humanos , Unidades de Cuidado Intensivo Pediátrico , Masculino , Paro Cardíaco Extrahospitalario/etiología , Paro Cardíaco Extrahospitalario/mortalidad , Paro Cardíaco Extrahospitalario/terapia , Estudios Prospectivos , Tasa de Supervivencia , Turquía
9.
Tohoku J Exp Med ; 234(4): 295-8, 2014 12.
Artículo en Inglés | MEDLINE | ID: mdl-25519876

RESUMEN

Despite major advances in intensive care, sepsis continues to be a major cause of morbidity and mortality. Vitamin D is involved in various physiologic functions, including cellular responses during infection and inflammation. The aim of this study was to evaluate diagnostic value of 25-hydroxyvitamin D in childhood sepsis because it can be fatal if diagnosis delayed. The study included 40 children with sepsis and 20 children without sepsis (control group). We included only the patients with high probable sepsis, judged by clinical and laboratory findings, including positive blood culture. Blood samples were collected from patients with sepsis before treatment (pre-treatment group) and 48-72 hours later (post-treatment group). Treatment varied from ampicillin-sulbactam to cephalosporin. Blood samples were collected from control group once on admission. Serum 25-hydroxyvitamin D levels were significantly higher in sepsis (pre-treatment group) than control group (74 ± 8 ng/ml vs. 28 ± 12 ng/ml, p = 0.01) and the serum 25-hydroxyvitamin D levels were decreased to 44 ± 5 ng/ml (p = 0.01) after treatment. Moreover, we found significant positive correlation between 25-hydroxyvitamin D and each of well-know sepsis markers, C-reactive protein, tumor necrosis factor-α and interleukin-6. A cut-off point of 20 ng/mL for serum 25-hydroxyvitamin D showed 84% sensitivity and 76% specificity for sepsis diagnosis. This is the first study evaluating the diagnostic role of vitamin D in pediatric sepsis, thereby suggesting that serum 25-hydroxyvitamin D level can be used as a diagnostic marker for sepsis with high sensitivity and specificity.


Asunto(s)
Sepsis/sangre , Vitamina D/análogos & derivados , Adolescente , Biomarcadores/sangre , Estudios de Casos y Controles , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Vitamina D/sangre
11.
Hong Kong Med J ; 19(2): 186-7, 2013 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-23535682

RESUMEN

Nasopharyngeal encephalocele is a rare, benign congenital anomaly. It has the potential to be fatal due to airway obstruction. Here, we report on a 34-day-old infant with pneumonia who underwent mechanical ventilation. An upper airway evaluation was performed due to prolonged intubation, and revealed the presence of a nasopharyngeal encephalocele. The patient tolerated extubation and oral feeding after surgical resection of the lesion. Awareness of the condition can help clinicians arrive at an earlier diagnosis and enhance management.


Asunto(s)
Obstrucción de las Vías Aéreas/etiología , Encefalocele/diagnóstico , Recien Nacido Prematuro , Enfermedades Nasofaríngeas/diagnóstico , Obstrucción de las Vías Aéreas/patología , Obstrucción de las Vías Aéreas/cirugía , Biopsia con Aguja , Encefalocele/complicaciones , Encefalocele/cirugía , Femenino , Humanos , Inmunohistoquímica , Recién Nacido , Unidades de Cuidado Intensivo Neonatal , Imagen por Resonancia Magnética/métodos , Enfermedades Nasofaríngeas/complicaciones , Enfermedades Nasofaríngeas/cirugía , Enfermedades Raras , Medición de Riesgo , Resultado del Tratamiento
12.
Pediatr Emerg Care ; 29(8): 916-21, 2013 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-23903672

RESUMEN

OBJECTIVE: Previous studies have reported on a link between carboxyhemoglobin (COHb) levels and the severity of presenting findings. However, studies on pediatric populations evaluating the effect of age on presenting symptoms are severely lacking. The aim of this study was to investigate the presence of any link between age and presenting symptoms in children with carbon monoxide (CO) poisoning. METHODS: This retrospective study was undertaken in Ankara Children's Hematology and Oncology Hospital, a tertiary care center, between January 2007 and March 2010. The medical records of patients aged between 0 and 16 years with a confirmed diagnosis of CO poisoning, defined as the presence of a COHb level of more than 5%, were evaluated. Relevant information such as age, sex, source of CO, coaffected family members, month of presentation, time of presentation and presenting symptoms, duration of oxygen treatment in the emergency department, need for admission to an inpatient ward or intensive care unit, Glasgow Coma Scale scores, and administered treatments during follow-up was recorded for each patient on preprepared forms. For the purpose of comparison, patients were divided into 2 groups based on COHb levels (group 1, 5%-25%; group 2, >25%). Comparisons were also made after dividing patients into 3 age groups: infants (0-3 years), preschool and early-school children (4-8 years), and adolescents (9-16 years). RESULTS: The records of 261 patients were deemed sufficient for inclusion in the final analysis, 149 (57.1%) of which were female, and 112 (42.9%) were male, with a median age of 7.0 years (range, 1 month to 16 years) and a mean COHb level 16.9% (SD, 7.8%). Two hundred eighteen patients (83.5%) had a COHb between 5% and 25% on presentation, whereas the remaining 43 patients (16.5%) had a presenting COHb of greater than 25%. Neurologic symptoms such as headache, syncope, seizures, and confusion were encountered more frequently in the COHb greater than 25% group compared with the group with 5% to 25% COHb levels, with adolescents having more severe symptoms than do younger patients. CONCLUSIONS: In this study, we managed to demonstrate the presence of more severe symptoms in patients with a COHb level of 25% or greater. Further analysis revealed that severe symptoms were more pronounced in adolescents and that the severity of symptoms increased with age.


Asunto(s)
Intoxicación por Monóxido de Carbono/complicaciones , Carboxihemoglobina/análisis , Adolescente , Factores de Edad , Intoxicación por Monóxido de Carbono/sangre , Intoxicación por Monóxido de Carbono/diagnóstico , Niño , Preescolar , Femenino , Escala de Coma de Glasgow , Cefalea/etiología , Humanos , Lactante , Masculino , Náusea/etiología , Convulsiones/etiología , Índice de Severidad de la Enfermedad , Síncope/etiología
13.
Pediatr Emerg Care ; 29(4): 502-3, 2013 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-23558268

RESUMEN

Hydrogen peroxide solutions are often used in daily life as a household disinfectant and in cosmetic products and are therefore a common source of intentional poisonings, especially for children. When ingested in small amounts, it may cause severe central nervous system damage as a result of arterial emboli like our case. The benefit of hyperbaric oxygen treatment in this situation is known, but the neurologic deficits of our case did not improve with this method and finally brain death occurred. This is may be related by the timing of treatment after the event. We would like to emphasize that every clinician must be aware of the dangers of hydrogen peroxide ingestion and hyperbaric oxygen treatment may be of benefit if it is performed immediately.


Asunto(s)
Muerte Encefálica/diagnóstico , Peróxido de Hidrógeno/envenenamiento , Preescolar , Ingestión de Alimentos , Resultado Fatal , Femenino , Humanos
14.
Am J Physiol Lung Cell Mol Physiol ; 302(6): L530-40, 2012 Mar 15.
Artículo en Inglés | MEDLINE | ID: mdl-22207591

RESUMEN

Abnormalities of the lymphatic circulation are well recognized in patients with congenital heart defects. However, it is not known how the associated abnormal blood flow patterns, such as increased pulmonary blood flow (PBF), might affect pulmonary lymphatic function and structure. Using well-established ovine models of acute and chronic increases in PBF, we cannulated the efferent lymphatic duct of the caudal mediastinal node and collected and analyzed lymph effluent from the lungs of lambs with acutely increased PBF (n = 6), chronically increased PBF (n = 6), and age-matched normal lambs (n = 8). When normalized to PBF, we found that lymph flow was unchanged following acute increases in PBF but decreased following chronic increases in PBF. The lymph:plasma protein ratio decreased with both acute and chronic increases in PBF. Lymph bioavailable nitric oxide increased following acute increases in PBF but decreased following chronic increases in PBF. In addition, we found perturbations in the transit kinetics of contrast material through the pleural lymphatics of lambs with chronic increases in PBF. Finally, there were structural changes in the pulmonary lymphatic system in lambs with chronic increases in PBF: lymphatics from these lambs were larger and more dilated, and there were alterations in the expression of vascular endothelial growth factor-C, lymphatic vessel endothelial hyaluronan receptor-1, and Angiopoietin-2, proteins known to be important for lymphatic growth, development, and remodeling. Taken together these data suggest that chronic increases in PBF lead to both functional and structural aberrations of lung lymphatics. These findings have important therapeutic implications that warrant further study.


Asunto(s)
Cardiopatías Congénitas/fisiopatología , Pulmón/irrigación sanguínea , Pulmón/fisiopatología , Sistema Linfático/fisiopatología , Vasos Linfáticos/fisiopatología , Circulación Pulmonar/fisiología , Angiopoyetina 2/genética , Angiopoyetina 2/metabolismo , Animales , Cardiopatías Congénitas/genética , Cardiopatías Congénitas/metabolismo , Hemodinámica/fisiología , Pulmón/metabolismo , Sistema Linfático/metabolismo , Vasos Linfáticos/metabolismo , Óxido Nítrico/metabolismo , Arteria Pulmonar/metabolismo , Arteria Pulmonar/fisiopatología , Circulación Pulmonar/genética , Flujo Sanguíneo Regional/genética , Flujo Sanguíneo Regional/fisiología , Ovinos , Factor C de Crecimiento Endotelial Vascular/genética , Factor C de Crecimiento Endotelial Vascular/metabolismo
15.
Rheumatology (Oxford) ; 51(4): 743-8, 2012 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-22190688

RESUMEN

OBJECTIVES: Modified adult disease severity scoring systems are being used for childhood FMF. We aim to test the clinical consistency of two common severity scoring systems and to evaluate the correlation of scores with the type of FMF mutations in paediatric FMF patients since certain mutations are prone to severe disease. METHODS: Two hundred and fifty-eight children with FMF were cross-sectionally studied. Assessment of the disease severity was performed by using the modified scoring systems of Mor et al. and Pras et al. Genetic analysis was performed using PCR and restriction endonuclease digestion methods for the presence of 15 FMF gene mutations. FMF mutations were grouped into three based on well-known genotypic-phenotypic associations. Correlation between the mutation groups and the severity scoring systems was assessed. The consistency of the severity scoring systems was evaluated. RESULTS: The results of two scoring systems were not statistically consistent with each other (κ = 0.171). This inconsistency persisted even in a more homogeneous subgroup of patients with only homozygote mutations of M694V, M680I and M694I (κ = 0.125). There was no correlation between the mutation groups and either of the scoring systems (P = 0.002, r = 0,196 for scoring systems of Mor et al.; P = 0.009, r = 0.162 for Pras et al.). CONCLUSIONS: The inconsistency of the two scoring systems and lack of correlation between the scoring systems and mutation groups raises concerns about the reliability of these scoring systems in children. There is a need to develop a scoring system in children based on a prospective registry.


Asunto(s)
Fiebre Mediterránea Familiar/diagnóstico , Índice de Severidad de la Enfermedad , Adolescente , Factores de Edad , Niño , Preescolar , Estudios Transversales , Fiebre Mediterránea Familiar/genética , Femenino , Frecuencia de los Genes , Predisposición Genética a la Enfermedad , Humanos , Masculino , Mutación , Reproducibilidad de los Resultados
16.
Pediatr Emerg Care ; 28(11): 1236-7, 2012 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-23128656

RESUMEN

Clinical features of mercury poisoning are nonspecific, and a detailed history is very valuable. The silvery, shiny appearance of mercury makes it very exciting and attractive for children. The overall half-life of elemental mercury in the body averages approximately 2 months. Chelation therapy with dimercaptosuccinic acid is the treatment of choice if the urine or blood level of mercury is high or the symptoms are profound. Here, we describe a 14-year-old boy with fever, respiratory distress, and body rash. Investigation leading to a diagnosis of mercury poisoning was made only after his mother presented with the similar symptoms a few days later.


Asunto(s)
Quelantes/uso terapéutico , Intoxicación por Mercurio/diagnóstico , Penicilamina/uso terapéutico , Adolescente , Diagnóstico Diferencial , Humanos , Masculino , Intoxicación por Mercurio/tratamiento farmacológico
17.
Turk J Pediatr ; 54(4): 429-31, 2012.
Artículo en Inglés | MEDLINE | ID: mdl-23692728

RESUMEN

Pelvic ultrasonography (US) is a simple and non-invasive radiologic test to evaluate the pelvic organs. It requires a full bladder for better visualization. Our case is a 14-year-old female with diabetes insipidus (DI) who admitted to the pediatric emergency service with the complaints of seizure and agitation after drinking 4 liters of water in one hour for a pelvic US examination due to work-up for delayed puberty. Her biochemical and clinical evaluation revealed water intoxication (WI). To our knowledge, this is the first WI case developed in a patient with DI. Here, we discuss the underlying factors leading to this complication and recommended an approach to obtain a better sonographic image without necessitating oral water intake to fill the urinary bladder.


Asunto(s)
Diabetes Insípida/complicaciones , Pelvis/diagnóstico por imagen , Intoxicación por Agua/diagnóstico , Intoxicación por Agua/etiología , Adolescente , Femenino , Humanos , Enfermedad Iatrogénica , Ultrasonografía
18.
J Cell Physiol ; 226(12): 3104-13, 2011 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-21351102

RESUMEN

Pulmonary vasodilation is mediated through the activation of protein kinase G (PKG) via a signaling pathway involving nitric oxide (NO), natriuretic peptides (NP), and cyclic guanosine monophosphate (cGMP). In pulmonary hypertension secondary to congenital heart disease, this pathway is endogenously activated by an early vascular upregulation of NO and increased myocardial B-type NP expression and release. In the treatment of pulmonary hypertension, this pathway is exogenously activated using inhaled NO or other pharmacological agents. Despite this activation of cGMP, vascular dysfunction is present, suggesting that NO-cGMP independent mechanisms are involved and were the focus of this study. Exposure of pulmonary artery endothelial or smooth muscle cells to the NO donor, Spermine NONOate (SpNONOate), increased peroxynitrite (ONOO(-) ) generation and PKG-1α nitration, while PKG-1α activity was decreased. These changes were prevented by superoxide dismutase (SOD) or manganese(III)tetrakis(1-methyl-4-pyridyl)porphyrin (MnTMPyP) and mimicked by the ONOO(-) donor, 3-morpholinosydnonimine N-ethylcarbamide (SIN-1). Peripheral lung extracts from 4-week old lambs with increased pulmonary blood flow and pulmonary hypertension (Shunt lambs with endogenous activation of cGMP) or juvenile lambs treated with inhaled NO for 24 h (with exogenous activation of cGMP) revealed increased ONOO(-) levels, elevated PKG-1α nitration, and decreased kinase activity without changes in PKG-1α protein levels. However, in Shunt lambs treated with L-arginine or lambs administered polyethylene glycol conjugated-SOD (PEG-SOD) during inhaled NO exposure, ONOO(-) and PKG-1α nitration were diminished and kinase activity was preserved. Together our data reveal that vascular dysfunction can occur, despite elevated levels of cGMP, due to PKG-1α nitration and subsequent attenuation of activity.


Asunto(s)
Proteínas Quinasas Dependientes de GMP Cíclico/metabolismo , GMP Cíclico/metabolismo , Hipertensión Pulmonar/enzimología , Óxido Nítrico/metabolismo , Arteria Pulmonar/enzimología , Sistemas de Mensajero Secundario , Vasodilatación , Administración por Inhalación , Animales , Animales Recién Nacidos , Células Cultivadas , Proteína Quinasa Dependiente de GMP Cíclico Tipo I , Modelos Animales de Enfermedad , Células Endoteliales/enzimología , Activación Enzimática , Depuradores de Radicales Libres/farmacología , Hipertensión Pulmonar/tratamiento farmacológico , Hipertensión Pulmonar/fisiopatología , Metaloporfirinas/farmacología , Molsidomina/análogos & derivados , Molsidomina/farmacología , Músculo Liso Vascular/enzimología , Miocitos del Músculo Liso/enzimología , Óxido Nítrico/administración & dosificación , Donantes de Óxido Nítrico/farmacología , Ácido Peroxinitroso/metabolismo , Polietilenglicoles/farmacología , Procesamiento Proteico-Postraduccional , Arteria Pulmonar/efectos de los fármacos , Arteria Pulmonar/fisiopatología , Circulación Pulmonar , Ovinos , Espermina/análogos & derivados , Espermina/farmacología , Superóxido Dismutasa/farmacología , Vasodilatación/efectos de los fármacos , Vasodilatadores/farmacología
19.
Pediatr Emerg Care ; 27(12): 1180-1, 2011 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-22158280

RESUMEN

Fructose-1,6-diphosphatase (FDPase) enzyme deficiency is a rare inherited metabolic disease. Affected patients usually present with metabolic crisis including hypoglycemia, acidosis, ketonuria, and hyperuricemia. A previously healthy 8-month-old male infant presented with fever, vomiting, and hypoactivity. He had tachycardia, tachypnea, and a tendency to sleep. The patient had signs of severe dehydration and shock. Laboratory findings revealed significant lactic acidosis, hyperuricemia, hyperglycemia, elevated liver enzyme level, and hyperlipidemia. The urine analysis had evidence of glycosuria and ketonuria. Hyperuricemia, lactic acidemia, and hyperglycemia persisted despite insulin infusion, adequate hydration, and perfusion. Consequently, peritoneal dialysis was started. About 12 hours after dialysis, his metabolic derangements were normalized, and clinical status was improved dramatically. His metabolic disease workup was compatible with FDPase deficiency. Here, we described a metabolic attack of FDPase deficiency presented with hyperglycemia mimicking diabetic ketoacidosis.


Asunto(s)
Acidosis Láctica/diagnóstico , Cetoacidosis Diabética/diagnóstico , Deficiencia de Fructosa-1,6-Difosfatasa/diagnóstico , Hiperglucemia/diagnóstico , Alanina Transaminasa/sangre , Aspartato Aminotransferasas/sangre , Deshidratación/etiología , Diagnóstico Diferencial , Fiebre/etiología , Deficiencia de Fructosa-1,6-Difosfatasa/sangre , Deficiencia de Fructosa-1,6-Difosfatasa/complicaciones , Deficiencia de Fructosa-1,6-Difosfatasa/dietoterapia , Deficiencia de Fructosa-1,6-Difosfatasa/orina , Gluconeogénesis , Glucosuria/etiología , Hepatomegalia/sangre , Hepatomegalia/etiología , Humanos , Hiperlipidemias/etiología , Hiperuricemia/etiología , Lactante , Masculino , Diálisis Peritoneal , Choque Séptico/complicaciones
20.
Turk J Pediatr ; 53(5): 537-40, 2011.
Artículo en Inglés | MEDLINE | ID: mdl-22272455

RESUMEN

The aim of this study was to evaluate the indications, complications and outcomes of pediatric tracheotomies at a tertiary care center. Data were obtained retrospectively from 54 patients who underwent tracheotomy from July 2007 to May 2010. Over the three-year period, 54 tracheotomies were performed. Thirty-two patients (59.3%) were male and 22 (40.7%) were female. The mean and median ages of the patients were 54 and 14 months (6 days-17 years), respectively. Twenty-six patients (48.1%) were under 1 year of age. The most common indication for tracheotomy was prolonged intubation (87%), followed by upper airway obstruction (13%). Five patients (9.2%) underwent tracheotomy under semiurgent settings. The overall complication rate was 29.6% (16/54). Early complications occurred in 7 patients (13%), including accidental decannulation in 2 patients, subcutaneous emphysema in 4 patients and hemorrhage in 1 patient. Late complications occurred in 9 patients (16.7%) (stomal granulation in 7 patients and stomal infection in 2 patients). Eight patients (14.8%) were decannulated successfully. No tracheotomy-related deaths occurred, with an overall mortality rate of 27.7% (15/54). Pediatric tracheotomy is a relatively safe procedure with a low incidence of procedure-related morbidities. The indication for the majority of the procedures was prolonged intubation (87%). The lower decannulation rate is related to the higher percentage of patients needing assisted ventilation and the relatively short follow-up period.


Asunto(s)
Traqueotomía , Adolescente , Obstrucción de las Vías Aéreas/cirugía , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Complicaciones Posoperatorias/epidemiología , Respiración Artificial , Estudios Retrospectivos , Traqueotomía/efectos adversos , Traqueotomía/estadística & datos numéricos
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