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1.
Am J Infect Control ; 52(8): 906-914, 2024 Aug.
Article in English | MEDLINE | ID: mdl-38437883

ABSTRACT

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.


Subject(s)
Catheter-Related Infections , Intensive Care Units , Urinary Tract Infections , Humans , Catheter-Related Infections/epidemiology , Catheter-Related Infections/prevention & control , Urinary Tract Infections/epidemiology , Urinary Tract Infections/prevention & control , Asia/epidemiology , Latin America/epidemiology , Middle East/epidemiology , Male , Female , Europe, Eastern/epidemiology , Infection Control/methods , Middle Aged , Cross Infection/prevention & control , Cross Infection/epidemiology , Aged
2.
Front Pediatr ; 11: 1179721, 2023.
Article in English | MEDLINE | ID: mdl-37601138

ABSTRACT

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.
Article in English | MEDLINE | ID: mdl-36813951

ABSTRACT

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.


Subject(s)
Critical Illness , Systemic Inflammatory Response Syndrome , Humans , Child , Cohort Studies , Intensive Care Units, Pediatric , Risk Factors , Lactates , Retrospective Studies
4.
Pediatr Allergy Immunol Pulmonol ; 33(2): 57-62, 2020 Jun.
Article in English | MEDLINE | ID: mdl-35863042

ABSTRACT

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.
Article in English | MEDLINE | ID: mdl-30968623

ABSTRACT

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.


Subject(s)
Cardiopulmonary Resuscitation/methods , Out-of-Hospital Cardiac Arrest/therapy , Adolescent , Cardiopulmonary Resuscitation/statistics & numerical data , Child , Child, Preschool , Cohort Studies , Emergency Medical Services/statistics & numerical data , Female , Humans , Infant , Male , Out-of-Hospital Cardiac Arrest/epidemiology , Out-of-Hospital Cardiac Arrest/mortality , Retrospective Studies , Survival Rate , Time Factors , Treatment Outcome , Turkey
7.
Turk J Pediatr ; 60(5): 497-505, 2018.
Article in English | MEDLINE | ID: mdl-30968624

ABSTRACT

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.


Subject(s)
Cardiac Surgical Procedures/adverse effects , Heart Defects, Congenital/surgery , Hyperglycemia/etiology , Postoperative Complications/etiology , Adolescent , Blood Glucose/analysis , Child , Child, Preschool , Cohort Studies , Female , Humans , Hyperglycemia/blood , Hyperglycemia/mortality , Infant , Insulin/therapeutic use , Intensive Care Units, Pediatric , Male , Postoperative Period , Retrospective Studies , Risk Factors
8.
Paediatr Int Child Health ; 37(2): 148-151, 2017 May.
Article in English | MEDLINE | ID: mdl-27077627

ABSTRACT

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.


Subject(s)
Adrenal Insufficiency/complications , Adrenal Insufficiency/diagnosis , Cardiomyopathies/complications , Cardiomyopathies/diagnosis , Heart Failure/etiology , Heart Failure/pathology , Adrenal Insufficiency/therapy , Autoimmune Diseases/complications , Cardiomyopathies/therapy , Child , Female , Furosemide/administration & dosage , Heart Failure/therapy , Humans , Hydrocortisone/administration & dosage , Sodium Potassium Chloride Symporter Inhibitors
9.
Turk J Pediatr ; 58(1): 86-89, 2016.
Article in English | MEDLINE | ID: mdl-27922241

ABSTRACT

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.


Subject(s)
Lung Neoplasms/secondary , Lung/pathology , Neuroblastoma/pathology , Adolescent , Fatal Outcome , Humans , Male , Prognosis , Respiration, Artificial , Tomography, X-Ray Computed/methods
10.
Turk J Pediatr ; 58(1): 116-118, 2016.
Article in English | MEDLINE | ID: mdl-27922249

ABSTRACT

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.


Subject(s)
Hyperphosphatemia/chemically induced , Laxatives/adverse effects , Phosphates/adverse effects , Seizures/chemically induced , Administration, Oral , Child , Humans , Hyperphosphatemia/therapy , Male , Phosphorus/blood , Renal Dialysis/methods
11.
Pediatr Int ; 58(6): 523-525, 2016 Jun.
Article in English | MEDLINE | ID: mdl-27322862

ABSTRACT

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.

12.
Pediatr Cardiol ; 37(7): 1241-9, 2016 Oct.
Article in English | MEDLINE | ID: mdl-27272692

ABSTRACT

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.


Subject(s)
Cardiac Surgical Procedures , Adolescent , Airway Extubation , Cardiopulmonary Bypass , Child , Child, Preschool , Heart Defects, Congenital , Humans , Infant , Prospective Studies , Retrospective Studies
13.
J Crit Care ; 32: 175-81, 2016 Apr.
Article in English | MEDLINE | ID: mdl-26795440

ABSTRACT

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.


Subject(s)
Intensive Care Units, Pediatric/statistics & numerical data , Intubation, Intratracheal/statistics & numerical data , Respiration, Artificial/methods , Respiratory Insufficiency/therapy , Adolescent , Adult , Child , Child, Preschool , Continuous Positive Airway Pressure , Female , Hospitals, University/statistics & numerical data , Humans , Infant , Intubation, Intratracheal/adverse effects , Length of Stay/statistics & numerical data , Male , Prospective Studies , Time Factors , Young Adult
14.
Turk J Pediatr ; 58(3): 297-300, 2016.
Article in English | MEDLINE | ID: mdl-28266196

ABSTRACT

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.


Subject(s)
Bronchodilator Agents/poisoning , Renal Dialysis/methods , Theophylline/poisoning , Child, Preschool , Female , Humans , Intensive Care Units, Pediatric , Seizures/etiology
15.
Pediatr Emerg Care ; 31(11): 748-52, 2015 Nov.
Article in English | MEDLINE | ID: mdl-26535496

ABSTRACT

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.


Subject(s)
Cardiopulmonary Resuscitation , Heart Arrest/therapy , Child, Preschool , Emergency Service, Hospital , Female , Heart Arrest/etiology , Heart Arrest/mortality , Humans , Intensive Care Units, Pediatric , Male , Out-of-Hospital Cardiac Arrest/etiology , Out-of-Hospital Cardiac Arrest/mortality , Out-of-Hospital Cardiac Arrest/therapy , Prospective Studies , Survival Rate , Turkey
16.
J Pediatr Endocrinol Metab ; 28(9-10): 1163-7, 2015 Sep.
Article in English | MEDLINE | ID: mdl-26024245

ABSTRACT

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.


Subject(s)
Carrier Proteins/genetics , Glycoproteins/genetics , Mutation , Niemann-Pick Disease, Type C/complications , Pulmonary Alveolar Proteinosis/etiology , Fatal Outcome , Female , Humans , Infant , Niemann-Pick Disease, Type C/genetics , Pulmonary Alveolar Proteinosis/genetics , Vesicular Transport Proteins
17.
Turk J Pediatr ; 56(1): 111-4, 2014.
Article in English | MEDLINE | ID: mdl-24827960

ABSTRACT

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.


Subject(s)
Fontan Procedure , Noninvasive Ventilation/methods , Child, Preschool , Female , Humans , Postoperative Complications , Respiration, Artificial , Ventilator Weaning
18.
J Pediatr Hematol Oncol ; 36(7): e473-5, 2014 Oct.
Article in English | MEDLINE | ID: mdl-24577553

ABSTRACT

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.


Subject(s)
Bronchiolitis Obliterans/etiology , Graft vs Host Disease/complications , Graft vs Host Disease/etiology , Hematopoietic Stem Cell Transplantation/adverse effects , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/therapy , Adolescent , Bronchiolitis Obliterans/pathology , Chronic Disease , Fatal Outcome , Humans , Male , Sclerosis/etiology , Sclerosis/pathology , Severity of Illness Index , Skin/pathology , Thoracic Wall/pathology , Young Adult
19.
J Clin Immunol ; 34(3): 309-15, 2014 Apr.
Article in English | MEDLINE | ID: mdl-24510376

ABSTRACT

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.


Subject(s)
Immunologic Deficiency Syndromes/epidemiology , Intensive Care Units, Pediatric , Adolescent , Cause of Death , Child , Child, Preschool , Female , Hematopoietic Stem Cell Transplantation , Hospital Mortality , Humans , Immunologic Deficiency Syndromes/diagnosis , Immunologic Deficiency Syndromes/therapy , Infant , Infant, Newborn , Male , Retrospective Studies , Risk Factors , Treatment Outcome
20.
J Pediatr Hematol Oncol ; 36(2): e88-90, 2014 Mar.
Article in English | MEDLINE | ID: mdl-23619107

ABSTRACT

INTRODUCTION: Thrombotic microangiopathy (TMA) is characterized by microvascular thrombosis, thrombocytopenia, and microangiopathic hemolytic anemia. Previous studies have shown that cyclosporine (CsA) is associated with TMA but the number of reported cases is very limited. We describe a 13-year-old girl with CsA-associated TMA and thrombocytopenia-associated multiple organ failure (TAMOF). CASE REPORT: The patient was diagnosed with polyglandular deficiency syndrome and had a history of celiac disease, autoimmune thyroiditis, and diabetes mellitus type I. CsA was started 7 months before her admission to our pediatric intensive care unit for persistent diarrhea associated with celiac disease. At the time of her admission to our pediatric intensive care unit, she was thrombocytopenic and anemic with multiple organ failure. Laboratory and clinical findings were consistent with TMA and TAMOF. CsA was discontinued and therapeutic plasma exchange was performed daily for 5 days. The patient improved clinically, laboratory findings normalized, and TMA and multiple organ failure dissolved. CONCLUSION: This case report indicates that therapeutic plasma exchange may be effective in the treatment of CsA-associated TMA and TAMOF, especially in the presence of systemic findings.


Subject(s)
Cyclosporine/adverse effects , Immunosuppressive Agents/adverse effects , Multiple Organ Failure/therapy , Plasma Exchange , Thrombotic Microangiopathies/therapy , Adolescent , Female , Humans , Multiple Organ Failure/chemically induced , Multiple Organ Failure/physiopathology , Polyendocrinopathies, Autoimmune/drug therapy , Thrombocytopenia/chemically induced , Thrombocytopenia/physiopathology , Thrombocytopenia/therapy , Thrombotic Microangiopathies/chemically induced , Thrombotic Microangiopathies/physiopathology
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