Characteristics and prognostic factors of previously healthy children who required respiratory support in a pediatric intensive care unit

PURPOSE: Comorbidities have been considered a mortality risk factor in pediatric critical care patients. We studied the characteristics and prognostic factors of children without comorbidities who were admitted to the intensive care unit (ICU) due to respiratory failure. METHODS: We reviewed the medical charts of patients (< 18 years) admitted to the ICU for respiratory support in a single tertiary center between January 2006 and December 2016. Patients with comorbidities and perioperative statuses were excluded. RESULTS: Of the 4,712 ICU patients, 73 (1.5%) were included in this study. The median age was 31 months (8–57) and 51 (69.9%) were boys. Twenty-nine patients (39.7%) presented with pneumonia, 14 (19.2%) with acute respiratory distress syndrome (ARDS), and 11 (15.1%) with obstructive airway disease. The median duration of ICU hospitalization was 5 days (2–14.5), and 45 of the 73 patients (61.6%) needed mechanical ventilation. Mortality was 13.7% (10/73). None of the patients with pneumonia or obstructive airway disease died. The most frequent cause of death was ARDS (5 of 10, 50%). In adjusted analysis, the extent of extrapulmonary organ dysfunction was significantly associated with mortality (odds ratio, 2.89; 95% confidence interval, 1.17–7.11; P=0.023). CONCLUSION: The mortality rate of previously healthy pediatric patients needing respiratory support in the ICU should not be negligible. Multiple organ dysfunctions might be a significant risk factor for mortality in such patients.

Validation of the Pediatric Index of Mortality 3 in a Single Pediatric Intensive Care Unit in Korea

To compare mortality rate, the adjustment of case-mix variables is needed. The Pediatric Index of Mortality (PIM) 3 score is a widely used case-mix adjustment system of a pediatric intensive care unit (ICU), but there has been no validation study of it in Korea. We aim to validate the PIM3 in a Korean pediatric ICU, and extend the validation of the score from those aged 0–16 to 0–18 years, as patients aged 16–18 years are admitted to pediatric ICU in Korea. A retrospective cohort study of 1,710 patients was conducted in a tertiary pediatric ICU. To validate the score, the discriminatory power was assessed by calculating the area under the receiver-operating characteristic (ROC) curve, and calibration was evaluated by the Hosmer-Lemeshow goodness-of-fit (GOF) test. The observed mortality rate was 8.47%, and the predicted mortality rate was 6.57%. For patients aged < 18 years, the discrimination was acceptable (c-index = 0.76) and the calibration was good, with a χ² of 9.4 in the GOF test (P = 0.313). The observed mortality rate in the hemato-oncological subgroup was high (18.73%), as compared to the predicted mortality rate (7.13%), and the discrimination was unacceptable (c-index = 0.66). In conclusion, the PIM3 performed well in a Korean pediatric ICU. However, the application of the PIM3 to a hemato-oncological subgroup needs to be cautioned. Further studies on the performance of PIM3 in pediatric patients in adult ICUs and pediatric ICUs of primary and secondary hospitals are needed.

A 75-Year-Old Natural Survivor with Uncorrected Tetralogy of Fallot Presenting with Hypoxic Spell / 이화의대지

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Only a few patients reach adulthood without surgical correction. Unrepaired TOF patients with mild to moderate right ventricular outflow tract (RVOT) obstruction may be clinically silent until adulthood. TOF with hypoxic spells present as periods of profound cyanosis that occur because of almost total RVOT obstruction. So, hypoxic spell typically occurs in a crying infant but is rare in an adult. In this report, we presented a case of a 75-year-old patient with uncorrected TOF presenting with hypoxic spell, consequent pulmonary hypertension and chronic heart failure. This is the oldest case of natural survivor with uncorrected TOF in Korea and the oldest patient presenting hypoxic spell worldwide.

Distinctive clinical features of HPeV-3 infection in 2 neonates with a sepsis-like illness / 소아과

We report a human parechovirus-3 (HPeV-3) infection in 2 neonates who had prolonged fever (>5 days) with palmar-plantar erythema. This distinctive rash was observed 4–5 days after fever onset, just before defervescence. Elevated aspartate aminotransferase, lactate dehydrogenase, and ferritin levels were characteristic laboratory findings in the 2 cases, suggesting tissue damage caused by hypercytokinemia. Case 1 was treated with intravenous immunoglobulin, considering the possibility of severe systemic inflammatory responses. The initial ferritin level was 385 ng/mL (range, 0–400 ng/mL); however, the level increased to 2,581 ng/dL on day 5 after fever onset. Case 2 presented with milder clinical symptoms, and the patient recovered spontaneously. HPeV-3 was detected in cerebrospinal fluid and/or blood samples, but no other causative agents were detected. The findings from our cases, in accordance with recent studies, suggest that clinical features such as palmar-plantar erythema and/or hyperferritinemia might be indicators of HPeV-3 infection in neonates with sepsis-like illness. In clinical practice, where virology testing is not easily accessible, clinical features such as palmar-plantar erythema and/or hyperferritinemia might be helpful to diagnose HPeV-3 infection.

A rare type of single coronary artery with right coronary artery originating from the left circumflex artery in a child / 소아과

The presence of a single coronary artery is a rare congenital anomaly; such patients often present with severe myocardial ischemia. We experienced the case of a 13-year-old girl with the right coronary artery originating from the left circumflex artery. She visited our Emergency Department owing to severe chest pain; her cardiac enzyme levels were elevated, but her initial electrocardiogram (ECG) was normal. Echocardiography showed normal anatomy and normal regional wall motion. When she presented with recurrent chest pain on admission, the ECG showed significant ST-segment elevation in the left precordial leads and inferior leads with ST-segment depression in aVR lead, suggesting myocardial ischemia, and her cardiac enzyme levels were also elevated. We performed coronary angiography that showed a single right coronary artery originating from the left circumflex artery without stenosis. We confirmed the presence of a single coronary artery using coronary computed tomography. In addition, the treadmill test that was performed showed normal results. She was discharged from the hospital without any medications but with a recommendation of a regular follow-up.

A study of the relationship between clinical phenotypes and plasma iduronate-2-sulfatase enzyme activities in Hunter syndrome patients / 소아과

PURPOSE: Mucopolysaccharidosis type II (MPS II or Hunter syndrome) is a rare lysosomal storage disorder caused by iduronate-2-sulfatase (IDS) deficiency. MPS II causes a wide phenotypic spectrum of symptoms ranging from mild to severe. IDS activity, which is measured in leukocyte pellets or fibroblasts, was reported to be related to clinical phenotype by Sukegawa-Hayasaka et al. Measurement of residual plasma IDS activity using a fluorometric assay is simpler than conventional measurements using skin fibroblasts or peripheral blood mononuclear cells. This is the first study to describe the relationship between plasma IDS activity and clinical phenotype of MPS II. METHODS: We hypothesized that residual plasma IDS activity is related to clinical phenotype. We classified 43 Hunter syndrome patients as having attenuated or severe disease types based on clinical characteristics, especially intellectual and cognitive status. There were 27 patients with the severe type and 16 with the attenuated type. Plasma IDS activity was measured by a fluorometric enzyme assay using 4-methylumbelliferyl-alpha-iduronate 2-sulphate. RESULTS: Plasma IDS activity in patients with the severe type was significantly lower than that in patients with the attenuated type (P=0.006). The optimal cut-off value of plasma IDS activity for distinguishing the severe type from the attenuated type was 0.63 nmol.4 hr-1.mL-1. This value had 88.2% sensitivity, 65.4% specificity, and an area under receiver-operator characteristics (ROC) curve of 0.768 (ROC curve analysis; P=0.003). CONCLUSION: These results show that the mild phenotype may be related to residual lysosomal enzyme activity.

A study of the relationship between clinical phenotypes and plasma iduronate-2-sulfatase enzyme activities in Hunter syndrome patients / 소아과

PURPOSE: Mucopolysaccharidosis type II (MPS II or Hunter syndrome) is a rare lysosomal storage disorder caused by iduronate-2-sulfatase (IDS) deficiency. MPS II causes a wide phenotypic spectrum of symptoms ranging from mild to severe. IDS activity, which is measured in leukocyte pellets or fibroblasts, was reported to be related to clinical phenotype by Sukegawa-Hayasaka et al. Measurement of residual plasma IDS activity using a fluorometric assay is simpler than conventional measurements using skin fibroblasts or peripheral blood mononuclear cells. This is the first study to describe the relationship between plasma IDS activity and clinical phenotype of MPS II. METHODS: We hypothesized that residual plasma IDS activity is related to clinical phenotype. We classified 43 Hunter syndrome patients as having attenuated or severe disease types based on clinical characteristics, especially intellectual and cognitive status. There were 27 patients with the severe type and 16 with the attenuated type. Plasma IDS activity was measured by a fluorometric enzyme assay using 4-methylumbelliferyl-alpha-iduronate 2-sulphate. RESULTS: Plasma IDS activity in patients with the severe type was significantly lower than that in patients with the attenuated type (P=0.006). The optimal cut-off value of plasma IDS activity for distinguishing the severe type from the attenuated type was 0.63 nmol.4 hr-1.mL-1. This value had 88.2% sensitivity, 65.4% specificity, and an area under receiver-operator characteristics (ROC) curve of 0.768 (ROC curve analysis; P=0.003). CONCLUSION: These results show that the mild phenotype may be related to residual lysosomal enzyme activity.

Successful Desensitization of Oxcarbazepine: First Case in Pediatric Patient

Aromatic antiepileptic drugs (AEDs), such as diphenylhydantoin, phenobarbital, or carbamazepine (CBZ), are frequently associated with hypersensitivity reactions. This may restrict treatment options considerably due to cross reactivity with other aromatic AEDs. Desensitization can be very helpful for patients who show cross sensitivity with other AEDs. We report a case an 8-year-old patient who had cross sensitivity to oxcarbazepine (OXC) and CBZ and successfully managed by desensitization to OXC. The patient presented with intractable frontal lobe epilepsy. He had become seizure free with OXC; however OXC had to be discontinued due to whole body rash. CBZ also caused a hypersensitivity reaction. Therefore, OXC desensitization was attempted; he then had very subtle seizures during sleep with a frequency of 5 to 6 episodes per month on the 18th month of desensitization. Desensitization can be considered in a patient with limited treatment options due to hypersensitivity to aromatic AEDs.

2009 H1N1 influenza virus infection and necrotizing pneumonia treated with extracorporeal membrane oxygenation / 소아과

A 3-year-old girl with acute respiratory distress syndrome due to a H1N1 2009 influenza virus infection was complicated by necrotizing pneumonia was successfully treated with extracorporeal membrane oxygenation (ECMO). This is the first reported case in which a pediatric patient was rescued with ECMO during the H1N1 influenza epidemic in Korea in 2009.