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1.
Am J Infect Control ; 2024 Mar 02.
Artigo em Inglês | MEDLINE | ID: mdl-38437883

RESUMO

BACKGROUND: Catheter-Associated Urinary Tract Infections (CAUTIs) frequently occur in the intensive care unit (ICU) and are correlated with a significant burden. METHODS: We implemented a strategy involving a 9-element bundle, education, surveillance of CAUTI rates and clinical outcomes, monitoring compliance with bundle components, feedback of CAUTI rates and performance feedback. This was executed in 299 ICUs across 32 low- and middle-income countries. The dependent variable was CAUTI per 1,000 UC days, assessed at baseline and throughout the intervention, in the second month, third month, 4 to 15 months, 16 to 27 months, and 28 to 39 months. Comparisons were made using a 2-sample t test, and the exposure-outcome relationship was explored using a generalized linear mixed model with a Poisson distribution. RESULTS: Over the course of 978,364 patient days, 150,258 patients utilized 652,053 UC-days. The rates of CAUTI per 1,000 UC days were measured. The rates decreased from 14.89 during the baseline period to 5.51 in the second month (risk ratio [RR] = 0.37; 95% confidence interval [CI] = 0.34-0.39; P < .001), 3.79 in the third month (RR = 0.25; 95% CI = 0.23-0.28; P < .001), 2.98 in the 4 to 15 months (RR = 0.21; 95% CI = 0.18-0.22; P < .001), 1.86 in the 16 to 27 months (RR = 0.12; 95% CI = 0.11-0.14; P < .001), and 1.71 in the 28 to 39 months (RR = 0.11; 95% CI = 0.09-0.13; P < .001). CONCLUSIONS: Our intervention, without substantial costs or additional staffing, achieved an 89% reduction in CAUTI incidence in ICUs across 32 countries, demonstrating feasibility in ICUs of low- and middle-income countries.

2.
Front Pediatr ; 11: 1179721, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37601138

RESUMO

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

3.
Pediatr Res ; 94(2): 730-737, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-36813951

RESUMO

BACKGROUND: This study evaluated of clinical characteristics, outcomes, and mortality risk factors of a severe multisystem inflammatory syndrome in children admitted to a the pediatric intensive care unit. METHODS: A retrospective multicenter cohort study was conducted between March 2020 and April 2021 at 41 PICUs in Turkey. The study population comprised 322 children diagnosed with multisystem inflammatory syndrome. RESULTS: The organ systems most commonly involved were the cardiovascular and hematological systems. Intravenous immunoglobulin was used in 294 (91.3%) patients and corticosteroids in 266 (82.6%). Seventy-five (23.3%) children received therapeutic plasma exchange treatment. Patients with a longer duration of the PICU stay had more frequent respiratory, hematological, or renal involvement, and also had higher D-dimer, CK-MB, and procalcitonin levels. A total of 16 patients died, with mortality higher in patients with renal, respiratory, or neurological involvement, with severe cardiac impairment or shock. The non-surviving group also had higher leukocyte counts, lactate and ferritin levels, and a need for mechanical ventilation. CONCLUSIONS: In cases of MIS-C, high levels of D-dimer and CK-MB are associated with a longer duration of PICU stay. Non-survival correlates with elevated leukocyte counts and lactate and ferritin levels. We were unable to show any positive effect of therapeutic plasma exchange therapy on mortality. IMPACT: MIS-C is a life-threatening condition. Patients need to be followed up in the intensive care unit. Early detection of factors associated with mortality can improve outcomes. Determining the factors associated with mortality and length of stay will help clinicians in patient management. High D-dimer and CK-MB levels were associated with longer PICU stay, and higher leukocyte counts, ferritin and lactate levels, and mechanical ventilation were associated with mortality in MIS-C patients. We were unable to show any positive effect of therapeutic plasma exchange therapy on mortality.


Assuntos
Estado Terminal , Síndrome de Resposta Inflamatória Sistêmica , Humanos , Criança , Estudos de Coortes , Unidades de Terapia Intensiva Pediátrica , Fatores de Risco , Lactatos , Estudos Retrospectivos
4.
Pediatr Allergy Immunol Pulmonol ; 33(2): 57-62, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35863042

RESUMO

Background: Acute bronchiolitis is one of the most common diseases of early childhood. There are many recent changes in the treatment of acute bronchiolitis. The aim of this study is to evaluate treatment approaches to acute bronchiolitis among clinicians and to observe compliance with clinical guidelines. Materials and Methods: Our study was designed as a multicenter cross-sectional descriptive study. A cohort of pediatric residents, fellows, and attendants were surveyed with a questionnaire including general and occupational characteristics of pediatricians and treatment choices in acute bronchiolitis. Results: A total of 713 questionnaires were collected. Most commonly applied treatment among pediatricians was inhaled salbutamol, followed by intravenous hydration, hypertonic saline, and inhaled steroid. Most commonly preferred treatment in the management of mild bronchiolitis was oral hydration and inhaled salbutamol in severe bronchiolitis. Conclusion: Although recent guidelines for the treatment of acute bronchiolitis does not support the use of many different therapies, pediatricians still tend to use them, especially bronchodilators, corticosteroids, and antibiotics.

6.
Turk J Pediatr ; 60(5): 488-496, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30968623

RESUMO

Kurt F, Kendirli T, Gündüz RC, Kesici S, Akça H, Sahin S, Kalkan G, Derbent M, Tuygun N, Ödek Ç, Gültekin-Keser A, Oguz S, Polat E, Derinöz O, Tekin D, Teksam Ö, Bayrakci B, Suskan E. Outcome of out-of-hospital cardiopulmonary arrest in children: A multicenter cohort study. Turk J Pediatr 2018; 60: 488-496. The aim of this study was to evaluate the demographic characteristics of children who experienced out-of-hospital cardiopulmonary arrest (CPA), and to assess the impact of the bystander cardiopulmonary resuscitation (CPR) on the survival rate of witnessed arrests and the effects of the arrest and CPR durations on the neurological outcomes. This multicenter, retrospective study included a total of 182 patients who underwent CPR for out-of-hospital CPA between January 2008 and December 2012 at six centers in Ankara, Turkey. The median [interquartile range (IQR)] age was 22 (5-54) months; 60.4% of the patients were males, and 44% were younger than one year of age. The witnessed arrest rate was 75.8% (138/182) and the rate of bystander CPR was 13.9% (13/93). In these patients the rate of the return of spontaneous circulation (ROSC) was higher (76.9%). Following resuscitation in the patients for whom the spontaneous circulation was able to be returned, the median (IQR) duration of arrest was 5 (1- 15) min, while it was 15 (5-40) min for the remaining patients (p < 0.001). The ROSC rate was 94.9% in patients who underwent CPR for less than 20 min and 22% in patients requiring CPR longer than 20 min (p < 0.001). Survival to hospital discharge was 14.3%. Of these patients, 57.7% experienced neurological disability. The short duration of an arrest and the presence of CPR are both critical for survival. We suggest that a witness to the CPA, performing early and efficient CPR, yields better results.


Assuntos
Reanimação Cardiopulmonar/métodos , Parada Cardíaca Extra-Hospitalar/terapia , Adolescente , Reanimação Cardiopulmonar/estatística & dados numéricos , Criança , Pré-Escolar , Estudos de Coortes , Serviços Médicos de Emergência/estatística & dados numéricos , Feminino , Humanos , Lactente , Masculino , Parada Cardíaca Extra-Hospitalar/epidemiologia , Parada Cardíaca Extra-Hospitalar/mortalidade , Estudos Retrospectivos , Taxa de Sobrevida , Fatores de Tempo , Resultado do Tratamento , Turquia
7.
Turk J Pediatr ; 60(5): 497-505, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30968624

RESUMO

Ödek Ç, Kendirli T, Yildirim-Yildiz N, Yaman A, Uçar T, Eyileten Z, Ates C, Uysalel A, Tutar E, Atalay S. Perioperative factors associated with hyperglycemia after pediatric cardiac surgery and impact of hyperglycemia on morbidity and mortality Turk J Pediatr 2018; 60 497-505. This retrospective, observational, single-center study aimed to determine the perioperative factors associated with postoperative hyperglycemia (blood glucose level ≥126 mg/dl) and the impact of hyperglycemia on morbidity and mortality in a cohort of children undergoing cardiac surgery. Non-diabetic children aged between 1 month to 18 years who were consecutively admitted to pediatric intensive care unit (PICU) after cardiac surgery for congenital heart disease between January 2008 and December 2013 were included. One hundred and twenty-six patients were qualified for inclusion during the study period. Seventy-four (57.8%) of the patients had at least one glucose measurement ≥ 126 mg/dl. Higher PRISM III-24 (OR 1.1, 95% CI 1.02-1.18, p= 0.004) and PELOD (p=0.006) scores, higher Wernovsky inotropic score (p=0.027) and vasoactive-inotropic score (p=0.029) were associated with hyperglycemia. Postoperative hyperglycemia was not associated with duration of mechanical ventilation), length of PICU stay, healthcare associated infections, or mortality. Our study establishes that hyperglycemia is common after pediatric cardiac surgery but not associated with short-term morbidity and mortality. Insulin therapy can be accomplished without hypoglycemia when a permissive glycemic target is used. A large prospective multiple institution trial is necessary to facilitate defined guidelines for postoperative hyperglycemia after pediatric cardiac surgery.


Assuntos
Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Cardiopatias Congênitas/cirurgia , Hiperglicemia/etiologia , Complicações Pós-Operatórias/etiologia , Adolescente , Glicemia/análise , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Hiperglicemia/sangue , Hiperglicemia/mortalidade , Lactente , Insulina/uso terapêutico , Unidades de Terapia Intensiva Pediátrica , Masculino , Período Pós-Operatório , Estudos Retrospectivos , Fatores de Risco
8.
Paediatr Int Child Health ; 37(2): 148-151, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-27077627

RESUMO

Acute adrenal crisis is a life-threatening disorder. Cardiovascular complications of the condition are usually limited to hypovolaemic hypotension and shock. An acute reversible cardiomyopathy and heart failure in association with acute adrenal crisis is rarely reported, particularly in children. A 6-year-old girl with adrenal crisis which was complicated by acute reversible cardiomyopathy is reported. Inotropic and ventilatory support in addition to intravenous hydrocortisone and furosemide therapy were required to achieve cardiovascular stability. The cardiomyopathy resolved over 5 days and she was discharged with normal cardiac and intellectual functions. Cardiomyopathy should be considered in patients with acute adrenal crisis demonstrating any symptoms or signs of heart failure.


Assuntos
Insuficiência Adrenal/complicações , Insuficiência Adrenal/diagnóstico , Cardiomiopatias/complicações , Cardiomiopatias/diagnóstico , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/patologia , Insuficiência Adrenal/terapia , Doenças Autoimunes/complicações , Cardiomiopatias/terapia , Criança , Feminino , Furosemida/administração & dosagem , Insuficiência Cardíaca/terapia , Humanos , Hidrocortisona/administração & dosagem , Inibidores de Simportadores de Cloreto de Sódio e Potássio
9.
Turk J Pediatr ; 58(1): 86-89, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27922241

RESUMO

Neuroblastoma (NB) is the most frequently diagnosed neoplasm during infancy and its incidence declines within the first 3-5 years of life. It can be rarely diagnosed in adolescents and young adults. Adolescents have advanced stage of disease, higher frequency of uncommon metastatic sites such as lungs, and worse outcomes. Herein, we describe an unusual case of NB in a 17-year-old adolescent presented with lung metastasis at diagnosis. The patient was diagnosed with stage IV NB. Thorax high-resolution computed tomography (HRCT) scan revealed irregular septal thickening with ground glass opacity consistent with pulmonary parenchymal metastases. After the first cycle of chemotherapy he developed pulmonary hemorrhage and respiratory distress. He required ventilation support and mechanical ventilation was started. Metastatic nodules were determined on second thorax HRCT. We lost the patient due to septic shock and multiple organ failure 2 months after diagnosis. In conclusion, adolescents with NB have unfavorable prognosis. These patients may have lung metastases at diagnosis. Therefore, detailed chest imaging at initial diagnosis is crucial.


Assuntos
Neoplasias Pulmonares/secundário , Pulmão/patologia , Neuroblastoma/patologia , Adolescente , Evolução Fatal , Humanos , Masculino , Prognóstico , Respiração Artificial , Tomografia Computadorizada por Raios X/métodos
10.
Turk J Pediatr ; 58(1): 116-118, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27922249

RESUMO

Sodium phosphate based laxatives are commonly used for constipation and pre-procedural bowel cleansing. Phosphate intoxication related with these preparations is well recognized. Herein, we present a case of severe hyperphosphatemia and seizure in a 7-year-old male patient after administration of an oral sodium phosphate based laxative. At the time of admission, serum phosphorus level was 25.6 mg/dl. Aggressive fluid therapy was started. Although serum phosphorus level decreased to 20.9 mg/dl eight hours after admission, hemodialysis was performed because of the preexisting renal disease and declined glomerular filtration rate. Serum phosphorus level and blood gas analysis returned to normal after hemodialysis and the patient was discharged on hospital day two. In conclusion, sodium phosphate based laxatives should be used carefully in patients with preexisting renal diseases. Intravenous hydration and correction of hypocalcemia are important components of treatment. Hemodialysis is indicated in patients with renal failure.


Assuntos
Hiperfosfatemia/induzido quimicamente , Laxantes/efeitos adversos , Fosfatos/efeitos adversos , Convulsões/induzido quimicamente , Administração Oral , Criança , Humanos , Hiperfosfatemia/terapia , Masculino , Fósforo/sangue , Diálise Renal/métodos
11.
Pediatr Cardiol ; 37(7): 1241-9, 2016 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-27272692

RESUMO

This prospective, observational, single-center study aimed to determine the perioperative predictors of early extubation (<24 h after cardiac surgery) in a cohort of children undergoing cardiac surgery. Children aged between 1 month and 18 years who were consecutively admitted to pediatric intensive care unit after cardiac surgery for congenital heart disease between January 2012 and June 2014. Ninety-nine patients were qualified for inclusion during the study period. The median duration of mechanical ventilation was 20 h (range 1-480), and 64 patients were extubated within 24 h. Four of them failed the initial attempt at extubation, and the success rate of early extubation was 60.6 %. Older patient age (p = .009), greater body weight (p = .009), absence of preoperative pulmonary hypertension (p = .044), lower RACHS-1 category (OR, 3.8; 95 % CI 1.35-10.7; p < .05), shorter cardiopulmonary bypass (p = .008) and cross-clamp (p = .022) times, lower PRISM III-24 (p < .05) and PELOD (p < .05) scores, lower inotropic score (p < .05) and vasoactive-inotropic score (p < .05), and lower number of organ failures (OR, 2.26; 95 % CI 1.30-3.92; p < .05) were associated with early extubation. Our study establishes that early extubation can be accomplished within the first 24 h after surgery in low- to medium-risk pediatric cardiac surgery patients, especially in older ones undergoing low-complexity procedures. A large prospective multiple institution trial is necessary to identify the predictors and benefits of early extubation and to facilitate defined guidelines for early extubation.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Adolescente , Extubação , Ponte Cardiopulmonar , Criança , Pré-Escolar , Cardiopatias Congênitas , Humanos , Lactente , Estudos Prospectivos , Estudos Retrospectivos
12.
Pediatr Int ; 58(6): 523-525, 2016 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-27322862

RESUMO

A 13-year-old girl who had been on home parenteral nutrition for 6 months has been presented with multifocal atrial tachycardia and atrial fibrillation. Echocardiography and multislice computed tomography showed fat accumulation on the interatrial septum. Lipomatous hypertrophy of the interatrial septum has never been reported in children.

13.
J Crit Care ; 32: 175-81, 2016 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-26795440

RESUMO

PURPOSE: To determine the efficiency of noninvasive mechanical ventilation (NIV) both in protection from intubation and in preventing reintubation of postextubation in patients in the pediatric intensive care unit (PICU). METHODS: A prospective observational study was conducted in a multidisciplinary 10-bed tertiary PICU of a university hospital. All patients were admitted to our unit from June 2012 to May 2014 and deemed to be candidates to receive continuous positive airway pressure or bilevel positive airway pressure. MEASUREMENTS AND RESULTS: We performed 160 NIV episodes in 137 patients. Their median age was 9 months (range, 1-240 months), and their median weight was 7.5 kg (range, 2.5-65 kg). Fifty-seven percent of patients were male. Noninvasive mechanical ventilation was successful in 70% (112 episodes) of patients. There was an underlying illness in 83.8% (134 episodes) of the patients. Bilevel positive airway pressure support was given to 57.5% (92 episodes) of the patients, whereas the remaining 42.5% (68 episodes) received continuous positive airway pressure support. Among the causes of respiratory failure in our patients, the most frequent were postextubation, pneumonia, bronchiolitis, atelectasia, and cardiogenic pulmonary edema. Sedation was applied in 43.1% of the episodes. Complications were detected in 29 episodes (18.1 %). The NIV failure group showed higher Pediatric Risk of Mortality III-24 score, shorter NIV duration, more frequent underlying disease, lower number fed, longer length of PICU stay, and hospital stay, and mortality was higher. CONCLUSIONS: Noninvasive mechanical ventilation effectively and reliably reduced endotracheal intubation in the treatment of respiratory failure due to different clinical situations. Our results suggest that NIV can play an important role in PICUs in helping to avoid intubation and prevent reintubation. Although there were serious underlying diseases in most of our patients, such as immunosuppression, 70% avoided intubation with use of NIV.


Assuntos
Unidades de Terapia Intensiva Pediátrica/estatística & dados numéricos , Intubação Intratraqueal/estatística & dados numéricos , Respiração Artificial/métodos , Insuficiência Respiratória/terapia , Adolescente , Adulto , Criança , Pré-Escolar , Pressão Positiva Contínua nas Vias Aéreas , Feminino , Hospitais Universitários/estatística & dados numéricos , Humanos , Lactente , Intubação Intratraqueal/efeitos adversos , Tempo de Internação/estatística & dados numéricos , Masculino , Estudos Prospectivos , Fatores de Tempo , Adulto Jovem
14.
Turk J Pediatr ; 58(3): 297-300, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-28266196

RESUMO

Theophylline poisoning generally occurs due to acute high dose intake as well as chronic intake of the medication. Toxicity symptoms can be seen with a plasma concentration of theophylline over 20 µg/ml. The consequences of theophylline toxicity include metabolic disturbances (hypokalemia, hyperglycemia, and metabolic acidosis), nausea, vomiting, and in severe cases seizures, cardiac arrhythmias, and death. Theophylline poisoning in children is rarely described in the literature. A 3-year-old girl was referred from another hospital to our pediatric intensive care unit (PICU) due to prolonged refractory status epilepticus and respiratory failure linked with severe theophylline poisoning. The patient was admitted to our PICU 24 hours after the patient took theophylline. The referring center could not measure the serum theophylline level. The patient's first serum theophylline level that was checked at admission was 54 µg/ml. We started continuous venovenous hemodialysis (CVVHD) 3 hours after PICU admission and the patient's theophylline level successfully decreased within 9 hours. The patient was discharged at the 40th day of admission from our hospital with severe neurological disability. In conclusion, severe theophylline poisoning may be seen in children. We must consider CVVHD in critically ill children with severe theophylline poisoning.


Assuntos
Broncodilatadores/intoxicação , Diálise Renal/métodos , Teofilina/intoxicação , Pré-Escolar , Feminino , Humanos , Unidades de Terapia Intensiva Pediátrica , Convulsões/etiologia
15.
Pediatr Emerg Care ; 31(11): 748-52, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26535496

RESUMO

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.


Assuntos
Reanimação Cardiopulmonar , Parada Cardíaca/terapia , Pré-Escolar , Serviço Hospitalar de Emergência , Feminino , Parada Cardíaca/etiologia , Parada Cardíaca/mortalidade , Humanos , Unidades de Terapia Intensiva Pediátrica , Masculino , Parada Cardíaca Extra-Hospitalar/etiologia , Parada Cardíaca Extra-Hospitalar/mortalidade , Parada Cardíaca Extra-Hospitalar/terapia , Estudos Prospectivos , Taxa de Sobrevida , Turquia
16.
J Pediatr Endocrinol Metab ; 28(9-10): 1163-7, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-26024245

RESUMO

Niemann-Pick disease type C (NPC) is a fatal autosomal recessive lipid storage disease associated with impaired trafficking of unesterified cholesterol and glycolipids in lysosomes and late endosomes. This disease is commonly characterized by hepatosplenomegaly and severe progressive neurological dysfunction. There are two defective genes that cause this illness. One of these genes is NPC1 gene which is the cause of illness in 95% of the patients. The other gene is the rare type NPC2 which is the cause of illness in 5% of the patients. Patients with NPC2 usually present with respiratory distress in early infancy, which is rather unusual with NPC1. This article discusses about a patient who died at an early age from pulmonary involvement and who subsequently was found to have a novel homozygous mutation of NPC2 gene.


Assuntos
Proteínas de Transporte/genética , Glicoproteínas/genética , Mutação , Doença de Niemann-Pick Tipo C/complicações , Proteinose Alveolar Pulmonar/etiologia , Evolução Fatal , Feminino , Humanos , Lactente , Doença de Niemann-Pick Tipo C/genética , Proteinose Alveolar Pulmonar/genética , Proteínas de Transporte Vesicular
17.
Turk J Pediatr ; 56(1): 111-4, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-24827960

RESUMO

For many decades, the primary treatment of acute respiratory failure has been mechanical ventilation. Intubation and mechanical ventilation carry complications such as upper respiratory system trauma, barotrauma, volutrauma, oxytrauma, atelectrauma, and nosocomial infection, and they increase mortality. Noninvasive mechanical ventilation (NIMV) applied to a specially selected group of patients has more advantages than invasive ventilation, especially the decreased number of complications. We present a three-year-old girl who had prolonged invasive mechanical ventilation (IMV) and weaning failure due to severe tracheal bleeding after Fontan operation. She could not be extubated for 20 days after admission to our Pediatric Intensive Care Unit (PICU) because of life-threatening airway bleedings from within the endotracheal tube. Owing to the severe bleedings during endotracheal aspirations, it was decided to change her mechanical ventilation to NIMV. NIMV was performed successfully for 12 days without difficulty, and she was discharged from the hospital on the 55th postoperative day with normal clinical findings. Noninvasive ventilation has an increasing use in critically ill children. NIMV should be considered in prolonged mechanical ventilation and weaning failure from IMV in critically ill children.


Assuntos
Técnica de Fontan , Ventilação não Invasiva/métodos , Pré-Escolar , Feminino , Humanos , Complicações Pós-Operatórias , Respiração Artificial , Desmame do Respirador
18.
J Pediatr Hematol Oncol ; 36(7): e473-5, 2014 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-24577553

RESUMO

Pulmonary chronic graft-versus-host disease (cGvHD) is one of the most common causes of morbidity and mortality after allogeneic hematopoietic stem cell transplantation (aHSCT). Herein, we describe a patient with severe restrictive lung defect secondary to cGvHD. A 21-year-old male patient was admitted to our pediatric intensive care unit (PICU) with pneumonia and respiratory distress. He had a history of aHSCT for chronic myelogeneous leukemia at the age of 17 years. Six months after undergoing aHSCT, he had developed cGvHD involving skin, mouth, eye, lung, liver, and gastrointestinal tract. At the time of PICU admission he had respiratory distress and required ventilation support. Thorax high-resolution computed tomography was consistent with bronchiolitis obliterans. Although bronchiolitis obliterans is an obstructive lung defect, a restrictive pattern became prominent in the clinical course because of the sclerotic chest wall skin. The activity of cGvHD kept increasing despite the therapy and we lost the patient because of severe respiratory distress and massive hemoptysis secondary to bronchiectasis. In conclusion, pulmonary cGvHD can present with restrictive changes related with the advanced sclerosis of the chest wall skin. Performing a fasciotomy or a scar revision for the rigid chest wall in selected patients may improve the patients ventilation.


Assuntos
Bronquiolite Obliterante/etiologia , Doença Enxerto-Hospedeiro/complicações , Doença Enxerto-Hospedeiro/etiologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Leucemia Mielogênica Crônica BCR-ABL Positiva/terapia , Adolescente , Bronquiolite Obliterante/patologia , Doença Crônica , Evolução Fatal , Humanos , Masculino , Esclerose/etiologia , Esclerose/patologia , Índice de Gravidade de Doença , Pele/patologia , Parede Torácica/patologia , Adulto Jovem
19.
J Clin Immunol ; 34(3): 309-15, 2014 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24510376

RESUMO

PURPOSES: The aims of this study were to review the frequency, characteristics, and the clinical course of primary immunodeficiency (PID) patients admitted to pediatric intensive care unit (PICU) and attempt to identify factors related with mortality that might predict a poor outcome. METHODS: We performed a retrospective review of children with PID aged 1 month to 18 years and admitted to PICU from January 2002 to January 2012 in our tertiary teaching children's hospital. RESULTS: There were a total of 51 patients accounting for 71 admissions to the PICU. The most common diagnosis was severe combined immunodeficiency. Respiratory problems were the leading cause for admission. A total of 20 patients received hematopoietic stem cell transplantation. Immune reconstitution was achieved in 9 (45 %) patients and eight of them did survive. In all 56 % of all admission episodes resulted in survival. Risk factors for mortality included requirement of mechanical ventilation (P < .001), number of organ system failure (P = .013), need for renal replacement therapy (P < .001), use of inotropes (P < .001), higher Pediatric Logistic Organ Dysfunction (PELOD) score (P = .005), and length of PICU stay (P < .001). CONCLUSIONS: This is the first study regarding the outcome and mortality-related risk factors for PID patients requiring PICU admission. We suggest that PICU management is as important as early diagnosis and treatment for these patients. Prediction of those at risk for poorer outcome might be beneficial for accurate intensive care management and survival.


Assuntos
Síndromes de Imunodeficiência/epidemiologia , Unidades de Terapia Intensiva Pediátrica , Adolescente , Causas de Morte , Criança , Pré-Escolar , Feminino , Transplante de Células-Tronco Hematopoéticas , Mortalidade Hospitalar , Humanos , Síndromes de Imunodeficiência/diagnóstico , Síndromes de Imunodeficiência/terapia , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento
20.
Pediatr Infect Dis J ; 33(1): e19-24, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-24346607

RESUMO

BACKGROUND: Colistin is active against most multidrug-resistant, aerobic Gram-negative bacteria. Because of the reported nephrotoxicity during the first years of use of colistin, there were concerns of its use in pediatrics where there was limited experience The aim of this study is to document the clinical characteristics and outcomes of use of colistin in pediatric patients at a pediatric intensive care unit in Turkey. METHODS: We reviewed the medical and laboratory records of 29 critically ill children who were treated with colistin for 38 courses between January 2011 and December 2011 at the Department of Pediatric Intensive Care Unit in Ankara University Medical School, Turkey. RESULTS: The median age was 17 months (range 3-217 months). Male-to-female ratio was 1:1.37. Ventilator-associated pneumonia (21 courses) was the leading diagnosis followed by catheter-related blood stream infection (6 courses), bacteremia (4 courses), ventriculoperitoneal shunt infection, peritonitis and pneumonia (1 course). The most commonly isolated microorganisms were Acinetobacter baumanni, Pseudomonas aeruginosa, Klebsiella pneumoniae, Serratia marcescens, Stenotrophomonas maltophilia, and Enterobacter cloacae. Two colistin formulations were used. Colimycin (Kocak Farma) was used in 21 colistin treatment episodes. The median dosage of colistin in this group was 5.0 mg/kg/d (2.3-5.6 mg/kg/d). Colomycin (Forest Laboratories) was used in 17 colistin treatment episodes. The median dosage of colistin in the second group was 75,000 International Unit/kg/d (50,000-80,000 International Unit/kg/d). Thirty colistin treatment episodes (79%) had a good or partial clinical response and 8 (21%) had a poor clinical response. Of the 8 colistin treatment episodes with poor clinical response, 3 were in the Colimycin group and 5 were in the Colomycin group. Ten patients died. There was no evidence of neurotoxicity in this study. Nephrotoxicity was observed in 1 patient but was not attributed to colistin because the patient had multiorgan failure at the same time. CONCLUSIONS: This study in a small cohort of patients suggests that the use of colistin in severe nosocomial infections caused by multidrug-resistant Gram-negative bacteria is well-tolerated and efficacious.


Assuntos
Antibacterianos/uso terapêutico , Colistina/uso terapêutico , Infecção Hospitalar/tratamento farmacológico , Unidades de Terapia Intensiva Pediátrica , Adolescente , Antibacterianos/efeitos adversos , Criança , Pré-Escolar , Colistina/efeitos adversos , Estado Terminal , Infecção Hospitalar/epidemiologia , Farmacorresistência Bacteriana Múltipla , Feminino , Humanos , Lactente , Masculino , Estudos Prospectivos , Fatores de Risco , Resultado do Tratamento
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