Oi-Wa Chan

Red Blood Cell Transfusion and Clinical Outcomes in Extremely Low Birth Weight Preterm Infants.

BACKGROUND Red blood cell (RBC) transfusion is often considered a life-saving measure in critically ill neonates. The smallest and least mature infants tend to receive the largest amount of transfusions. RBC transfusion itself has also been suggested as an independent risk factor of poor clinical outcome in critical patients. Our aim is to study if there are associations between RBC transfusion and in-hospital mortality, short-term morbidities, and late neurodevelopmental outcome in extremely low birth weight (ELBW) preterm infants. METHODS A cohort of ELBW preterm infants admitted to our neonatal intensive care unit from January 2009 to December 2010 were recruited. The number of RBC transfusions within 7 days, 30 days, and 60 days of life were recorded. Clinical outcomes including in-hospital mortality, development of retinopathy of prematurity (ROP), necrotizing enterocolitis, chronic lung disease, and later neurodevelopmental outcome were assessed with follow-up of up to 2 years of age. Multivariable logistic regression was used to estimate the associations between RBC transfusion and clinical outcomes. RESULTS A total of 98 ELBW preterm infants survived at the time of discharge. Of these survivors, the mean numbers of RBC transfusions were 2.5 ± 1.7, 7.4 ± 3.1, and 11.3 ± 4.5 times within 7 days, 30 days, and 60 days after birth, respectively. The number of transfusions within 7 days of life was correlated with risk of death before 1 month of age (odds ratio: 1.54, 95% confidence interval: 1.04-2.27, p = 0.03) and the number of transfusions within 30 days was correlated with risk of developing threshold ROP (odds ratio: 1.27, 95% confidence interval: 1.04-1.55, p = 0.02). The number of transfusions within 7 days of life was positively correlated with cognitive performance (Mental Developmental Index score) at 18-24 months of corrected age. CONCLUSION RBC transfusion has a negative impact on survival in ELBW infants. It increases the risk of developing ROP and affects late neurodevelopment. Decisions of blood transfusion in these very immature infants should be made cautiously taking these deleterious results into consideration.

Incidence and outcomes of acute kidney injury in extremely-low-birth-weight infants

Background Acute kidney injury (AKI) is a common event in the neonatal intensive care unit (NICU), especially in extremely-low-birth-weight (ELBW) infants. This cohort study investigated the incidence of and risk factors for AKI in ELBW infants and their overall survival at the postmenstrual age (PMA) of 36 weeks. Methods All ELBW infants admitted to our NICU between January 2010 and December 2013 were enrolled. Those who died prior to 72 hours of life, had congenital renal abnormality, or had only one datum of the serum creatinine (SCr) level after the first 24 hours of life were excluded. The criteria used for the diagnosis of AKI was set according to the modified neonatal KDIGO AKI definition. Results AKI occurred in 56% of 276 infants. Specifically, stage 1, stage 2, and stage 3 AKI occurred in 30%, 17%, and 9% of ELBW infants, respectively. High-frequency ventilation support (adjusted odds ratio [OR]: 3.4, 95% confidence interval [CI]: 1.78–6.67, p< 0.001), the presence of patent ductus arteriosus (adjusted OR: 4.3, 95% CI: 2.25–8.07, p < 0.001), lower gestational age (adjusted OR for gestational age: 0.7, 95% CI: 0.58–0.83, < 0.001), and inotropic agent use (adjusted OR: 2.6, 95% CI: 1.31–5.21, p = 0.006) were independently associated with AKI. Maternal pre-eclampsia was a protective factor (adjusted OR: 0.4, 95% CI: 0.14–0.97, p = 0.044). Infants with AKI had higher mortality before the PMA of 36 weeks with an adjusted hazard ratio (HR) of 5.34 (95% CI: 1.21–23.53, p = 0.027). Additionally, infants with stage 3 AKI had a highest HR of 10.60, 95% CI: 2.09–53.67, p = 0.004). Conclusion AKI was a very common event (56%) in ELBW infants and was associated with a lower GA, high-frequency ventilation support, the presence of PDA, and inotropic agent use. AKI reduced survival of ELBW infants before the PMA of 36 weeks.

Combined Guillain-Barré syndrome and myasthenia gravis.

BACKGROUND Guillain-Barré syndrome and myasthenia gravis both lead to muscle weakness but the two combined is uncommon. Detection of these entities can help identify forms of autoimmune neuromuscular diseases that may respond to immunotherapy. This report sought to characterize the clinical features of these two entities when combined. METHODS This report is of a case of combined Guillain-Barré syndrome and myasthenia gravis. The clinical features were analyzed and correlated to those published in English literature from 1960 to 2012. Ten reports and 12 cases, including the present case, were reviewed. RESULTS There were 12 patients (4 women and 8 men), aged 17 to 84 years, with combined Guillain-Barré syndrome and myasthenia gravis. Four had post-infectious Guillain-Barré syndrome followed by the development of myasthenia gravis concurrently or concomitantly within one month. All cases had symptoms of ptosis and areflexia. The other common presentations were limb weakness, oculobulbar weakness, and respiratory involvement. Functional outcome was mentioned in 10 patients and seven had good outcome (Hughes scale ≤ 2). CONCLUSION Detection of ptosis with or without ophthalmoplegia, distribution of limb weakness, and reflex can help in recognizing combined Guillain-Barré syndrome and myasthenia gravis. The early recognition of this combination of peripheral nervous and neuro-muscular junction inflammation is important for initial treatment and prognosis.

Decreased ADAMTS 13 Activity is Associated With Disease Severity and Outcome in Pediatric Severe Sepsis.

AbstractDecreased ADAMTS 13 activity has been reported in severe sepsis and in sepsis-induced disseminated intravascular coagulation. This study aimed to investigate the role of ADAMTS 13 in different pediatric sepsis syndromes and evaluate its relationship with disease severity and outcome.We prospectively collected cases of sepsis treated in a pediatric intensive care unit, between July 2012 and June 2014 in Chang Gung Childrens Hospital in Taoyuan, Taiwan. Clinical characteristics and ADAMTS-13 activity were analyzed.All sepsis syndromes had decreased ADAMTS 13 activity on days 1 and 3 of admission compared to healthy controls. Patients with septic shock had significantly decreased ADAMTS 13 activity on days 1 and 3 compared to those with sepsis and severe sepsis. There was a significant negative correlation between ADAMTS 13 activity on day 1 and day 1 PRISM-II, PELOD, P-MOD, and DIC scores. Patients with mortality had significantly decreased ADAMTS 13 activity on day 1 than survivors, but not on day 3.Different pediatric sepsis syndromes have varying degrees of decreased ADAMTS 13 activity. ADAMTS 13 activity is strongly negatively correlated with disease severity of pediatric sepsis syndrome, whereas decreased ADAMTS 13 activity on day 1 is associated with increased risk of mortality.

Therapeutic burst-suppression coma in pediatric febrile refractory status epilepticus

BACKGROUND Evidence for the beneficial effect of therapeutic burst-suppression coma in pediatric patients with febrile refractory status epilepticus is limited, and the clinical outcomes of this treatment strategy are largely unknown. Therefore, the aim of this study was to explore the outcomes of therapeutic burst-suppression coma in a series of children with febrile refractory status epilepticus. METHODS We retrospectively reviewed consecutive pediatric patients with febrile refractory status epilepticus admitted to our pediatric intensive care unit between January 2000 and December 2013. The clinical characteristics were analyzed. RESULTS Thirty-five patients (23 boys; age range: 1-18years) were enrolled, of whom 28 (80%) developed super-refractory status epilepticus. All of the patients received the continuous administration of intravenous antiepileptic drugs for febrile refractory status epilepticus, and 26 (74.3%) achieved therapeutic burst-suppression coma. All of the patients received mechanical ventilatory support, and 26 (74.3%) received inotropic agents. Eight (22.9%) patients died within 1month. The neurologically functional outcomes at 6months were good in six (27.3%) of the 22 survivors, of whom two returned to clinical baseline. The patients with therapeutic burst-suppression coma were significantly associated with hemodynamic support than the patients with electrographic seizures control (p=0.03), and had a trend of higher 1-month mortality rate, worse 6months outcomes, and a longer duration of hospitalization. CONCLUSIONS Our results suggest that therapeutic burst-suppression coma to treat febrile refractory status epilepticus may lead to an increased risk of hemodynamic instability and a trend of worse outcomes.

Epidemiology and clinical analysis of critical patients with child maltreatment admitted to the intensive care units

Abstract Children with abuse who are admitted to the intensive care unit (ICU) may have high mortality and morbidity and commonly require critical care immediately. It is important to understand the epidemiology and clinical characteristics of these critical cases of child maltreatment. We retrospectively evaluated the data for 355 children with maltreatments admitted to the ICU between 2001 and 2015. Clinical factors were analyzed and compared between the abuse and the neglect groups, including age, gender, season of admission, identifying settings, injury severity score (ISS), etiologies, length of stay (LOS) in the ICU, clinical outcomes, and mortality. In addition, neurologic assessments were conducted with the Pediatric Cerebral Performance Category (PCPC) scale. The most common type of child maltreatments was neglect (n = 259), followed by physical abuse (n = 96). The mean age of the abuse group was less than that of the neglect group (P < .05). Infants accounted for the majority of the abuse group, and the most common etiology of abuse was injury of the central nervous system (CNS). In the neglect group, most were of the preschool age and the most common etiologies of abuse were injury of the CNS and musculoskeletal system (P < .001). The mortality rate in the ICU was 9.86%. The ISS was significantly associated with mortality in both the 2 groups (both P < .05), whereas the LOS in the ICU and injuries of the CNS, musculoskeletal system, and respiratory system were all associated with mortality in the neglect group (all P < .05). The PCPC scale showed poor prognosis in the abuse group as compared to the neglect group (P < .01). In the ICU, children in the abuse group had younger age, higher ISS, and worse neurologic outcome than those in the neglect group. The ISS was a predictor for mortality in the abuse and neglect groups but the LOS in the ICUs, injuries of the CNS, musculoskeletal system, and respiratory system were indicators for mortality in the neglect group. Most importantly, identifying the epidemiological information may provide further strategies to reduce the harm, lower the medical costs, and improve clinical care quality and outcomes in children with abuse.

Increased serum thrombomodulin level is associated with disease severity and mortality in pediatric sepsis

Background Endothelial dysfunction plays an important role in the pathophysiology of sepsis. As previously reported, the serum thrombomodulin is elevated in diseases associated with endothelial injury. Objective The aim of this study was to investigate the association of serum thrombomodulin level in different pediatric sepsis syndromes and evaluate the relationship with disease severity and mortality. Methods We prospectively collected cases of sepsis treated in a pediatric intensive care unit from June 2012 to July 2015 at Chang Gung Children’s Hospital in Taoyuan, Taiwan. Clinical characteristics and serum thrombomodulin levels were analyzed. Results Increased serum thrombomodulin levels on days 1 and 3 of the diagnosis of sepsis were found in different pediatric sepsis syndromes. Patients with septic shock had significantly increased serum thrombomodulin levels on days 1 and 3 [day 1: median, 6.9 mU/ml (interquartile range (IQR): 5.8–12.8) and day 3: median, 5.8 mU/ml (IQR: 4.6–10.8)] compared to healthy controls [median, 3.4 mU/ml (IQR: 2.3–4.2)] (p = <0.001 and 0.001, respectively) and those with sepsis [day 1: median, 2.9 mU/ml (IQR: 1.8–4.7) and day 3: median, 3 mU/ml (IQR: 1.5–3.5)] and severe sepsis [day 1: median, 3.3 mU/ml (IQR: 1.3–8.6) and day 3: median, 4.4 mU/ml (IQR: 0.5–6)] (p = <0.001 and 0.001, respectively). There was also a significant positive correlation between serum thrombomodulin level on day 1 and day 1 PRISM-II, PELOD, P-MOD and DIC scores. The patients who died had significantly higher serum thrombomodulin levels on days 1 and 3 [day 1: median, 9.9 mU/ml (IQR: 6.2–15.6) and day 3: median, 10.4 mU/ml (IQR: 9.2–11.7)] than the survivors [day 1; median, 4.4 mU/ml (IQR: 2.2–7.5) and day 3: [median, 3.5 mU/ml (IQR: 1.6–5.7)] (p = 0.046 and 0.012, respectively). Conclusion Increased serum thrombomodulin levels were found in different pediatric sepsis syndromes and correlated with disease severity and mortality.

Strong correlation between doppler snuffbox resistive index and systemic vascular resistance in septic patients

Purpose: To compare systemic vascular resistance index (SVRI) as measured by invasive transpulmonary indicator dilution (TPID) and non‐invasive Doppler‐derived resistive index in septic patients. Methods: We measured the snuffbox resistive index (SBRI) in both hands of septic patients who received hemodynamic monitoring by TPID prospectively. Results: Thirty‐six patients with septic shock were enrolled (median acute physiology and chronic health evaluation II score: 23; median age: 64 years). Four SBRI values were measured in each patient, for a total of 96 patient days and 951 ultrasound measurements. The correlation coefficients between SVRI and the four SBRI values were all higher than 0.87 (p < .001). A higher SVRI was associated with sharp waveforms and reversed diastolic flow. A resistive index (RI) of 0.97 was the lower limit of normal SVRI (1700 dyn*s*cm−5*m2), and an RI of 1.1 was the upper limit of normal SVRI (2400 dyn*s*cm−5*m2). Conclusions: Using ultrasound to measure RI is a noninvasive, inexpensive, reliable method to evaluate peripheral vascular resistance in septic patients, and it is highly correlated with SVRI. In addition, SBRI can be used to evaluate peripheral circulatory disturbances in septic patients.

Combination of intravenous immunoglobulin and steroid pulse therapy improves outcomes of febrile refractory status epilepticus

BACKGROUND Febrile infections are an important cause of paediatric refractory status epilepticus, and immune-mediated mechanisms and inflammatory processes have been associated with neurological manifestations in such patients. The aim of this study was to investigate the effects of immunotherapy as adjuvant treatment for febrile refractory status epilepticus. METHODS We retrospectively reviewed cases of febrile refractory status epilepticus in a paediatric intensive care unit between January 2000 and December 2013 and analysed their clinical characteristics. Patients positive for antineuronal antibodies against surface antigens were excluded. RESULTS We enrolled 63 patients (38 boys), aged 1-18 years, all of whom received multiple antiepileptic drugs. Twenty-nine (46%) of the patients received intravenous immunoglobulin alone, 16 (25.4%) received a combination of intravenous immunoglobulin and methylprednisolone pulse therapy, and 18 (28.6%) did not receive immunotherapy treatment. Overall, 12 (19%) patients died within 1 month. After 6 months, 12 (20%) patients had good neurological outcomes, including two who returned to baseline and 13 (29.5%) who had favourable seizure outcomes. We compared the outcomes of the different treatments, and found that a combination of intravenous immunoglobulin and methylprednisolone pulse therapy had the best neurological and seizure outcomes at 6 months compared to intravenous immunoglobulin alone and no immunotherapy. CONCLUSIONS Our observational study showed that a combination of intravenous immunoglobulin and methylprednisolone pulse therapy as adjuvant treatment for febrile refractory status epilepticus was associated with better neurological and seizure outcomes. Further prospective studies are needed to confirm these findings.

Multiple magnets trapped at the esophagogastric junction of a child

a Division of Gastroenterology, Department of Pediatrics, Chang Gung Children’s Medical Center, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, 5 Fu-Hsing Street, Guishan District, 33305, Taoyuan City, Taiwan b Division of Critical Care Medicine, Department of Pediatrics, Chang Gung Children’s Medical Center, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, 5 Fu-Hsing Street, Guishan District, 33305, Taoyuan City, Taiwan