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BACKGROUND: Over the last decade, extracorporeal membrane oxygenation (ECMO) use in critically ill children has increased and is associated with favorable outcomes. Our study aims to evaluate the current status of pediatric ECMO in Korea, with a specific focus on its volume and changes in survival rates based on diagnostic indications. METHODS: This multicenter study retrospectively analyzed the indications and outcomes of pediatric ECMO over 10 years in patients at 14 hospitals in Korea from January 2012 to December 2021. Four diagnostic categories (neonatal respiratory, pediatric respiratory, post-cardiotomy, and cardiac-medical) and trends were compared between periods 1 (2012-2016) and 2 (2017-2021). RESULTS: Overall, 1065 ECMO runs were performed on 1032 patients, with the annual number of cases remaining unchanged over the 10 years. ECMO was most frequently used for post-cardiotomy (42.4%), cardiac-medical (31.8%), pediatric respiratory (17.5%), and neonatal respiratory (8.2%) cases. A 3.7% increase and 6.1% decrease in pediatric respiratory and post-cardiotomy cases, respectively, were noted between periods 1 and 2. Among the four groups, the cardiac-medical group had the highest survival rate (51.2%), followed by the pediatric respiratory (46.4%), post-cardiotomy (36.5%), and neonatal respiratory (29.4%) groups. A consistent improvement was noted in patient survival over the 10 years, with a significant increase between the two periods from 38.2% to 47.1% (P = 0.004). Improvement in survival was evident in post-cardiotomy cases (30-45%, P = 0.002). Significant associations with mortality were observed in neonates, patients requiring dialysis, and those treated with extracorporeal cardiopulmonary resuscitation (P < 0.001). In pediatric respiratory ECMO, immunocompromised patients also showed a significant correlation with mortality (P < 0.001). CONCLUSION: Pediatric ECMO demonstrated a steady increase in overall survival in Korea; however, further efforts are needed since the outcomes remain suboptimal compared with global outcomes.
Subject(s)
Cardiopulmonary Resuscitation , Extracorporeal Membrane Oxygenation , Infant, Newborn , Humans , Child , Retrospective Studies , Heart , Republic of Korea/epidemiologyABSTRACT
Safety and efficacy are essential in the process of disease treatment. However, off-label medication use is inevitable because various medications do not contain regulatory labels for pediatric use. We aimed to examine off-label medication use and analyze the risk factors correlated with adverse drug reactions (ADRs). This study was performed retrospectively using electronic medical data from a pediatric intensive care unit (PICU) of a tertiary hospital in Korea from July 2019 to June 2020. A total 6,183 prescribed medications from 502 PICU patients were examined in the present study. A total of 80% were infants or children, and 96.0% of them were treated with off-label medications. It was discovered that 4,778 off-label cases (77.2%) of the top 100 drugs had prescriptions with dosage (67.8%). Drugs prescribed to patients admitted to the cardiothoracic department (odds ratio [OR], 3.248; p = 0.019), total number of medications (OR, 1.116; p = 0.001), and length of PICU stay of ≥ 7 days (OR, 4.981; p = 0.008) were significantly associated with ADRs. ADRs were noted to be more severe in off-label use (p = 0.0426). For appropriate medication use, evidence regarding the safety of off-label medications is required and ultimately reflected in the official regulation.
ABSTRACT
DC is caused by defects at the level of telomere maintenance, and cells from patients with this disease have abnormally short telomeres and show premature senescence. One consequence of DC is bone marrow failure. Thus, patients with DC often require HSCT. However, HSCT does not ameliorate other DC-related manifestations. In fact, HSCT can accelerate organ dysfunction due to treatment-related complications, and solid organ transplantation is required in some patients with DC. In this report, we describe the clinical course of a 5-year-old boy who was transferred to our hospital because of progressive dyspnea, 2 years after HSCT. At admission, he had tachypnea and hypoxemia. A liver biopsy was performed for suspected HPS caused by PH, and LT was considered. Eventually, his hypoxemia worsened, and he was transferred to a PICU and started on VA ECMO. He subsequently underwent a CLLT. ECMO was stopped on post-operative day 12, extubation was achieved on post-operative day 29, and the patient recovered well from the surgery. Our results show that CLLT could be a life-saving treatment option for DC patients with very severe HPS in whom a poor outcome is expected after LT.
Subject(s)
Dyskeratosis Congenita/complications , Hepatopulmonary Syndrome/surgery , Hypertension, Portal/surgery , Liver Transplantation/methods , Lung Transplantation/methods , Child, Preschool , Hepatopulmonary Syndrome/etiology , Humans , Hypertension, Portal/etiology , Male , Patient AcuityABSTRACT
BACKGROUND: Some infants with severe bronchopulmonary dysplasia (sBPD) are referred to higher-level centers for multidisciplinary care, including the pediatric intensive care unit (PICU). However, information regarding these infants is limited in PICUs. METHODS: We investigated the characteristics and outcomes of preterm infants with sBPD referred to the PICU of a tertiary hospital. This retrospective cohort study included 14 preterm infants with sBPD who were transferred to the PICU beyond 40 weeks' postmenstrual age (PMA) because of weaning failure, from January 1, 2014, to September 30, 2018. RESULTS: The median age at referral was 47.1 weeks (range, 43.6-55.9 weeks), and the median length of stay in the previous neonatal intensive care unit was 154 days (range, 105.8-202.3 days) after birth. After referral the following major comorbidities were found in the patients: large airway malacia, n = 7 (50.0%); significant upper airway obstruction, n = 3 (21.4%); and pulmonary arterial hypertension, n = 8 patients (57.1%). Finally, eight patients (57.1%) were successfully extubated without tracheostomy. Final respiratory support of the patients was determined at a median PMA of 56 weeks (range, 48-63 weeks). Age at referral (P = 0.023) and large airway obstruction (P = 0.028) were significantly related to a decrease in successful extubation. CONCLUSION: Based on a timely and individualized multidisciplinary approach, some of the prolonged ventilator-dependent infants, even those beyond term age, could be successfully extubated.
Subject(s)
Bronchopulmonary Dysplasia , Bronchopulmonary Dysplasia/epidemiology , Bronchopulmonary Dysplasia/therapy , Child , Humans , Infant , Infant, Newborn , Infant, Premature , Intensive Care Units, Neonatal , Retrospective Studies , TracheostomyABSTRACT
BACKGROUND: The number of technology-dependent children (TDC) is increasing in South Korea, but available healthcare services after their discharge are poor. This study aimed to examine how TDC and caregivers live at home and identify their difficulties and needs regarding home care with few services to support them. METHODS: This cross-sectional study was conducted in a tertiary hospital for children in South Korea. A self-reported questionnaire was completed by primary caregivers of TDC who were younger than 19 years and had been dependent on medical devices for more than 3 months. Technologies included home mechanical ventilation, oxygen supplementation, suction equipment, enteral feeding tube, and home total parenteral nutrition. Patterns of healthcare use and home care of TDC and caregivers' perception toward child were assessed. RESULTS: A total of 74 primary caregivers of TDC completed a self-reported questionnaire. About 60% children were aged under 5 years. There were 31.1% children who required both respiratory and nutritional support. On average, caregivers took care of a child for 14.4 (±6.1) hours, slept for 5.6 (±1.6) hours, and spent 2.4 h per day on personal activities. Children used hospital services for 41.3 (±45.6) days in 6 months, and most (78.1%) were transported through private car/ambulance. Participants (75.6%) reported taking more than an hour to get to the hospital. More than 80% of caregivers responded that child care is physically very burdensome. The only statistically significant relationships was between economic status and financial burden (p = 0.026). CONCLUSIONS: Caregivers of TDC reported having significant time pressure regarding childcare-related tasks, insufficient time for personal activities, and inefficient hospital use because of inadequate medical services to support them in South Korea. Thus, it is necessary to support caregivers and develop a home care model based on current medical environment.
Subject(s)
Caregivers , Home Care Services , Aged , Child , Cross-Sectional Studies , Humans , Republic of Korea , TechnologyABSTRACT
BACKGROUND: To evaluate the efficacy and safety of fentanyl for sedation therapy in mechanically ventilated children. METHODS: This was a double-blind, randomized controlled trial of mechanically ventilated patients between 2 months and 18 years of age. Patients were randomly divided into two groups; the control group with midazolam alone, and the combination group with both fentanyl and midazolam. The sedation level was evaluated using the Comfort Behavior Scale (CBS), and the infusion rates were adjusted according to the difference between the measured and the target CBS score. RESULTS: Forty-four patients were recruited and randomly allocated, with 22 patients in both groups. The time ratio of cumulative hours with a difference in CBS score (measured CBS-target CBS) of ≥ 4 points (i.e., under-sedation) was lower in the combination group (median, 0.06; interquartile range [IQR], 0-0.2) than in the control group (median, 0.15; IQR, 0.04-0.29) (P < 0.001). The time ratio of cumulative hours with a difference in CBS score of ≥ 8 points (serious under-sedation) was also lower in the combination group (P < 0.001). The cumulative amount of midazolam used in the control group (0.11 mg/kg/hr; 0.07-0.14 mg/kg/hr) was greater than in the combination group (0.07 mg/kg/hr; 0.06-0.11 mg/kg/hr) (P < 0.001). Two cases of hypotension in each group were detected but coma and ileus, the major known adverse reactions to fentanyl, did not occur. CONCLUSION: Fentanyl combined with midazolam is safe and more effective than midazolam alone for sedation therapy in mechanically ventilated children. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT02172014.
Subject(s)
Adjuvants, Anesthesia/therapeutic use , Anxiety/prevention & control , Fentanyl/therapeutic use , Hypnotics and Sedatives/therapeutic use , Midazolam/therapeutic use , Adjuvants, Anesthesia/adverse effects , Adolescent , Child , Child, Preschool , Double-Blind Method , Drug Therapy, Combination , Female , Fentanyl/adverse effects , Humans , Hypnotics and Sedatives/adverse effects , Hypotension/etiology , Infant , Intensive Care Units , Male , Midazolam/adverse effects , Respiration, Artificial , Treatment OutcomeABSTRACT
BACKGROUND: The aim of this study was to describe the structure, organization, management, and staffing of pediatric critical care (PCC) in Korea. METHODS: We directed a questionnaire survey for all Upper Grade General Hospitals (n = 43) in Korea in 2015. The first questionnaire was mainly about structure, organization, and staffing and responses were obtained from 32 hospitals. The second questionnaire was mainly about patients and management. Responses to second questionnaire were obtained from 18 hospitals. RESULTS: Twelve from 32 Upper Grade General Hospitals had pediatric intensive care units (PICUs) and 11 of them had the PICU which was exclusive for children. Total number of PICU beds in Korea was 113. The ratio of the number of PICU beds to the number of children was 1:77,460 in Korea and this ratio is lower than that of other developed countries. The mean number of beds in the PICUs was 9.4 ± 9.3 (range, 2-30). There were 16 medical doctors who were assigned for PCC and only 5 of them were full time pediatric intensivists. In the 18 Upper Grade General Hospitals that responded to the second questionnaire survey, there were 97 patients in the PICUs with an average number of 5.7 ± 7.2 (range, 0-22) on the survey day. The mean age of the patients was 3.4 ± 5.6 years. The mean length of hospital stay was 82 ± 271 days. The mean Pediatric Risk of Mortality score III was 9.4 ± 7.8 at the time of admission to the PICUs. CONCLUSION: There is a considerable shortage of PICU beds compared to those in developed countries. In addition, the proportion of PICUs with PCC specialists is much lower than those in the US and European countries.
Subject(s)
Critical Care/statistics & numerical data , Child , Child, Preschool , Critical Care/organization & administration , Female , Hospitals, General , Humans , Infant , Infant, Newborn , Intensive Care Units, Pediatric , Length of Stay , Male , Republic of Korea , Surveys and QuestionnairesABSTRACT
PC are a main cause of death following HSCT in children. We aimed to evaluate early predictors of mortality in paediatric recipients with PCs. A retrospective observational study of 35 patients with 49 episodes of PI on chest radiography (of 124 patients) who had undergone HSCT at a tertiary university hospital between January 2011 and December 2012 was performed. During follow-up (median 26.1 months), 15 episodes led to death (30.6%). An aetiologic diagnosis was made by non-invasive tests in 24 episodes (49.0%) and by adding bronchoalveolar lavage and/or lung biopsy in 7 episodes with diagnostic yield (77.8%, P = .001). Thus, a specific diagnosis was obtained in 63.3% of the episodes. Aetiology identification and treatment modification after diagnosis did not decrease mortality (P = .057, P = .481). However, the number of organ dysfunctions at the beginning of PI was higher in the mortality group, compared to the survivor group (1.7 ± 1.2 vs 0.32 ± 0.59; P = .001). Hepatic dysfunction (OR, 11.145; 95% CI, 1.23 to 101.29; P = .032) and neutropaenia (OR, 10.558; 95% CI, 1.07 to 104.65; P = .044) were independently associated with risk of mortality. Therefore, hepatic dysfunction and neutropaenia are independent early predictors of mortality in HSCT recipients with PCs.
Subject(s)
Hematopoietic Stem Cell Transplantation/adverse effects , Lung Diseases/etiology , Lung Diseases/mortality , Adolescent , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Infant , Kaplan-Meier Estimate , Lung Diseases/diagnosis , Lung Diseases/therapy , Male , Multivariate Analysis , Odds Ratio , Radiography , Retrospective Studies , Risk Factors , Treatment OutcomeSubject(s)
Respiration, Artificial , Respiratory Insufficiency , Child , Humans , Intensive Care Units , Republic of KoreaABSTRACT
Considerable evidence has been published since the 2020 Korean Cardiopulmonary Resuscitation Guidelines were reported. The International Liaison Committee on Resuscitation (ILCOR) also publishes the Consensus on CPR and Emergency Cardiovascular Care Science with Treatment Recommendations (CoSTR) summary annually. This review provides expert opinions by reviewing the recent evidence on CPR and ILCOR treatment recommendations. The authors reviewed the CoSTR summary published by ILCOR in 2021 and 2022. PICO (patient, intervention, comparison, outcome) questions for each topic were reviewed using a systemic or scoping review methodology. Two experts were appointed for each question and reviewed the topic independently. Topics suggested by the reviewers for revision or additional description of the guidelines were discussed at a consensus conference. Forty-three questions were reviewed, including 15 on basic life support, seven on advanced life support, two on pediatric life support, 11 on neonatal life support, six on education and teams, one on first aid, and one related to COVID-19. Finally, the current Korean CPR Guideline was maintained for 28 questions, and expert opinions were suggested for 15 questions.
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CONTEXT: Since pediatric palliative care (PPC) aims to improve the health-related quality of life (HRQoL) of children with life-limiting conditions (LLC), assessment of their HRQoL and identification of its determinants is crucial. OBJECTIVES: To examine the clinical and family factors associated with HRQoL of children with LLC METHODS: This was a cross-sectional study of 136 pediatric patients with LLC who were enrolled in the PPC services at Seoul National University Hospital in South Korea. Patients' HRQoL was measured using the Pediatric Quality of Life 4.0. Clinical and family characteristics were gathered from the medical records and PPC registry database. RESULTS: Most children with LLC have a poor HRQoL at their enrollment for PPC services with significant variation in their total HRQoL scores according to the diagnostic categories. Patients with nonmalignant conditions showed significantly lower HRQoL scores than patients with malignancy. Lower HRQoL scores were associated with more caregiver depressive symptoms. In a multivariable regression model, total HRQoL scores of patients were significantly associated with diagnostic categories and caregiver's depressive symptoms after controlling for other clinical and family variables. Physical health summary scores were significantly associated with diagnostic categories and caregiver depressive symptoms. Psychosocial health summary scores were significantly associated with diagnostic categories, patient location, and caregiver's depressive symptoms. CONCLUSION: The HRQoL of children with LLC receiving PPC differed among underlying disease categories. Lower HRQoL was associated with more caregiver depressive symptoms. These findings suggest the needs for optimized intervention in palliative care for children with nonmalignant conditions and family-centered intervention to address caregivers' psychosocial problems.
Subject(s)
Hospice and Palliative Care Nursing , Quality of Life , Caregivers/psychology , Child , Cross-Sectional Studies , Humans , Palliative Care , Quality of Life/psychologyABSTRACT
BACKGROUND: Pediatric Index of Mortality 3 (PIM 3) and Pediatric Logistic Organ Dysfunction-2 (PELOD-2) are validated tools for predicting mortality in children. Research suggests that these tools may have different predictive performance depending on patient group characteristics. Therefore, we designed this study to identify the factors that make the mortality rates predicted by the tools different. METHODS: This retrospective study included patients (<18 years) who were admitted to a pediatric intensive care unit from July 2017 to May 2019. After defining the predicted mortality of PIM 3 minus the predicted mortality rate of PELOD-2 as "difference in mortality prediction," the clinical characteristics significantly related to this were analyzed using multivariable regression analysis. Predictive performance was analyzed through the Hosmer-Lemeshow test and area under the receiver operating characteristic curve (AUROC). RESULTS: In total, 945 patients (median [interquartile range] age, 3.0 [0.0-8.0] years; girls, 44.7%) were analyzed. The Hosmer-Lemeshow test revealed AUROCs of 0.889 (χ2=10.187, P=0.313) and 0.731 (χ2=6.220, P=0.183) of PIM 3 and PELOD-2, respectively. Multivariable linear regression analysis revealed that oxygen saturation, partial pressure of CO2, base excess, platelet counts, and blood urea nitrogen levels were significant factors. Patient condition-related factors such as cardiac bypass surgery, seizures, cardiomyopathy or myocarditis, necrotizing enterocolitis, cardiac arrest, leukemia or lymphoma after the first induction, bone marrow transplantation, and liver failure were significantly related (P<0.001). CONCLUSIONS: Both tools predicted observed mortality well; however, caution is needed in interpretation as they may show different prediction results in relation to specific clinical characteristics.
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BACKGROUND: Early recognition of deterioration events is crucial to improve clinical outcomes. For this purpose, we developed a deep-learning-based pediatric early-warning system (pDEWS) and aimed to validate its clinical performance. METHODS: This is a retrospective multicenter cohort study including five tertiary-care academic children's hospitals. All pediatric patients younger than 19 years admitted to the general ward from January 2019 to December 2019 were included. Using patient electronic medical records, we evaluated the clinical performance of the pDEWS for identifying deterioration events defined as in-hospital cardiac arrest (IHCA) and unexpected general ward-to-pediatric intensive care unit transfer (UIT) within 24 hours before event occurrence. We also compared pDEWS performance to those of the modified pediatric early-warning score (PEWS) and prediction models using logistic regression (LR) and random forest (RF). RESULTS: The study population consisted of 28,758 patients with 34 cases of IHCA and 291 cases of UIT. pDEWS showed better performance for predicting deterioration events with a larger area under the receiver operating characteristic curve, fewer false alarms, a lower mean alarm count per day, and a smaller number of cases needed to examine than the modified PEWS, LR, or RF models regardless of site, event occurrence time, age group, or sex. CONCLUSIONS: The pDEWS outperformed modified PEWS, LR, and RF models for early and accurate prediction of deterioration events regardless of clinical situation. This study demonstrated the potential of pDEWS as an efficient screening tool for efferent operation of rapid response teams.
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Despite the high prevalence of potential drug-drug interactions in pediatric intensive care units, their clinical relevance and significance are unclear. We assessed the characteristics and risk factors of clinically relevant potential drug-drug interactions to facilitate their efficient monitoring in pediatric intensive care units. This retrospective cohort study reviewed the medical records of 159 patients aged <19 years who were hospitalized in the pediatric intensive care unit at Seoul National University Hospital (Seoul, Korea) for ≥3 days between August 2019 and February 2020. Potential drug-drug interactions were screened using the Micromedex Drug-Reax® system. Clinical relevance of each potential drug-drug interaction was reported with official terminology, magnitude of severity, and causality, and the association with the patient's clinical characteristics was assessed. In total, 115 patients (72.3%) were exposed to 592 potential interactions of 258 drug pairs. In 16 patients (10.1%), 22 clinically relevant potential drug-drug interactions were identified for 19 drug pairs. Approximately 70% of the clinically relevant potential drug-drug interactions had a severity grade of ≥3. Exposure to potential drug-drug interactions was significantly associated with an increase in the number of administrated medications (6-7 medications, p = 0.006; ≥8, p<0.001) and prolonged hospital stays (1-2 weeks, p = 0.035; ≥2, p = 0.049). Moreover, clinically relevant potential drug-drug interactions were significantly associated with ≥8 prescribed drugs (p = 0.019), hospitalization for ≥2 weeks (p = 0.048), and ≥4 complex chronic conditions (p = 0.015). Most potential drug-drug interactions do not cause clinically relevant adverse outcomes in pediatric intensive care units. However, because the reactions that patients experience from clinically relevant potential drug-drug interactions are often very severe, there is a medical need to implement an appropriate monitoring system for potential drug-drug interactions according to the pediatric intensive care unit characteristics.
Subject(s)
Drug Monitoring , Intensive Care Units, Pediatric , Length of Stay , Pharmaceutical Preparations/administration & dosage , Adolescent , Child , Child, Preschool , Drug Interactions , Female , Humans , Infant , Infant, Newborn , Male , Republic of Korea , Retrospective StudiesABSTRACT
Vancomycin is known to be unintentionally eliminated by continuous renal replacement therapy, and the protein bound fraction of vancomycin is also known to be different in adults and children. However, there are only a few studies investigating the relationship between the dose of continuous venovenous hemodiafiltration (CVVHDF) parameters and serum concentration of vancomycin in pediatric patients. The aim of this study was to determine clinical and demographic parameters that significantly affect serum vancomycin concentrations. This retrospective cohort study was conducted at a pediatric intensive care unit in a tertiary university children's hospital. Data from oliguric patients who underwent CVVHDF and vancomycin therapeutic drug monitoring were collected. The correlation between factors affecting serum concentration of vancomycin was analyzed using mixed effect model. A total of 177 serum samples undergoing vancomycin therapeutic drug monitoring were analyzed. The median age of study participants was 2.23 (interquartile range, 0.3-11.84) years, and 126 (71.19%) were male patients. Serum concentration of vancomycin decreased significantly as the effluent flow rate (EFR; P < 0.001), dialysate flow rate (DFR; P = 0.009), replacement fluid flow rate (RFFR; P = 0.008), the proportion of RFFR in the sum of DFR and RFFR (P = 0.025), and residual urine output increased. The adjusted R2 of the multivariate regression model was 0.874 (P < 0.001) and the equation was as follows: Vancomycin trough level (mg/L) = (0.283 × daily dose of vancomycin [mg/kg/d]) + (365.139 / EFR [mL/h/kg])-(15.842 × residual urine output [mL/h/kg]). This study demonstrated that the serum concentration of vancomycin was associated with EFR, DFR, RFFR, the proportion of RFFR, and residual urine output in oliguric pediatric patients receiving CVVHDF.
Subject(s)
Acute Kidney Injury/therapy , Anti-Bacterial Agents/pharmacokinetics , Hemodiafiltration/methods , Oliguria/therapy , Staphylococcal Infections/drug therapy , Vancomycin/pharmacokinetics , Acute Kidney Injury/blood , Acute Kidney Injury/complications , Anti-Bacterial Agents/blood , Anti-Bacterial Agents/therapeutic use , Child , Child, Preschool , Critical Illness , Female , Humans , Infant , Male , Metabolic Clearance Rate , Oliguria/blood , Oliguria/complications , Retrospective Studies , Staphylococcal Infections/blood , Staphylococcal Infections/complications , Vancomycin/blood , Vancomycin/therapeutic useABSTRACT
Acute cerebral infarctions are rare in children, however they can occur as a complication of a Mycoplasma pneumoniae (MP) infection due to direct invasion, vasculitis, or a hypercoagulable state. We report on the case of a 5-year-old boy who had an extensive stroke in multiple cerebrovascular territories 10 days after the diagnosis of MP infection. Based on the suspicion that the cerebral infarction was associated with a macrolide-resistant MP infection, the patient was treated with levofloxacin, methyl-prednisolone, intravenous immunoglobulin, and enoxaparin. Despite this medical management, cerebral vascular narrowing progressed and a decompressive craniectomy became necessary for the patient's survival. According to laboratory tests, brain magnetic resonance imaging, and clinical manifestations, the cerebral infarction in this case appeared to be due to the combined effects of hypercoagulability and cytokine-induced vascular inflammation.
ABSTRACT
INTRODUCTION: Children with hereditary neuromuscular diseases (NMDs) are at a high risk of morbidity and mortality related to respiratory failure. The use of home mechanical ventilation (HMV) has saved the lives of many children with NMD but, due to a lack of studies, dependable guidelines are not available. We drew upon our experience to compare the various underlying NMDs and to evaluate HMV with regard to respiratory morbidity, the proper indications and timing for its use, and to develop a policy to improve the quality of home noninvasive ventilation (NIV). METHODS: We retrospectively analyzed the medical records of 57 children with childhood-onset hereditary NMDs in whom HMV was initiated between January 2000 and May 2013 at Seoul National University Children's Hospital. The degree of respiratory morbidity was estimated by the frequency and duration of hospitalizations caused by respiratory distress. RESULTS: The most common NMD was spinal muscular atrophy (SMA, n = 33). Emergent mechanical ventilation was initiated in 44% of the patients before the confirmed diagnosis, and the indicators of pre-HMV respiratory morbidity (e.g., extubation trials, hypoxia, hospitalizations, and intensive care unit stay) were greater in these patients than in others. The proportion of post-HMV hospitalizations (range, 0.00-0.52; median, 0.01) was lower than that of pre-HMV hospitalizations (0.02-1.00; 0.99) (P < 0.001). Eight patients were able to maintain home NIV. The main causes of NIV failure were air leakage and a large amount of airway secretions. CONCLUSIONS: The application of HMV helped reduce respiratory morbidity in children with childhood-onset hereditary NMD. Patients with SMA type I can benefit from an early diagnosis and the timely application of HMV. The choice between invasive and noninvasive HMV should be based on the patient's age and NIV trial tolerance. Systematic follow-up guidelines provided by a multidisciplinary team are needed.
Subject(s)
Neuromuscular Diseases/mortality , Adolescent , Adult , Child , Child, Preschool , Female , Home Care Services , Hospitalization , Humans , Infant , Male , Noninvasive Ventilation/methods , Republic of Korea , Respiration, Artificial/methods , Respiratory Insufficiency/mortality , Retrospective Studies , Young AdultABSTRACT
PURPOSE: Although few adverse effects have been reported for itraconazole, a widely used antifungal therapy for febrile neutropenia, we found intravenous (IV) itraconazole to be associated with serious cases of blood pressure (BP) drop. We therefore evaluated the incidence and risk factors for BP drop during IV administration of the drug. MATERIALS AND METHODS: We reviewed the medical records of children with hemato-oncologic disease who were treated with IV itraconazole from January 2012 to December 2013. By analyzing systolic BP (SBP) measurements made from 4 hours before through to 4 hours after itraconazole administration, we evaluated the changes in SBP and the risk factors for an SBP drop, especially clinically meaningful (≥ 20%) drops. RESULTS: Itraconazole was administered 2,627 times to 180 patients. The SBP during the 4 hours following itraconazole administration was lower than during the 4 hours before administration (104 [53.0-160.33 mmHg] versus 105 [59.8-148.3 mmHg]; P<0.001). The decrease in SBP was associated with the application of continuous renal replacement therapy (CRRT) (P=0.012) and the use of inotropic (P=0.005) and hypotensive drugs (P=0.021). A clinically meaningful SBP drop was seen in 5.37% (141 out of 2,627) of the administrations, and the use of inotropics (odds ratio [OR] 6.70, 95% confidence interval [CI] 3.22-13.92; P<0.001), reducing the dose of inotropics (OR 8.08; 95% CI 1.39-46.94; P=0.02), CRRT (OR 3.10, 95% CI 1.41-6.81; P=0.005), and bacteremia (OR 2.70, 95% CI 1.32-5.51; P=0.007) were risk factors, while age was a protective factor (OR 0.93, 95% CI 0.89-0.97; P<0.001). CONCLUSION: A decrease in SBP was associated with IV administration of itraconazole. It was particularly significant in younger patients with bacteremia using inotropic agents and during application of CRRT. Careful attention to hypotension is warranted during IV administration of itraconazole in this group of patients.