Outcomes of and factors associated with the development of bronchopulmonary dysplasia with pulmonary hypertension in very low birth weight infants: A retrospective study in a medical center

IntroductionBronchopulmonary dysplasia (BPD) with pulmonary hypertension (PH) leads to increased morbidity and mortality in extremely preterm infants. Recent studies have analyzed factors associated with development of PH in BPD; however, this research remains inconclusive, and controversy exists regarding the correlation between BPD and PH. This study aimed to investigate potential associated factors, clinical characteristics, and outcomes of BPD with pulmonary hypertension in very low birth weight (VLBW) preterm infants.MethodsWe conducted a retrospective study, reviewing the records of infants with gestational age (GA) <32 weeks and birth weight <1,500 g admitted to a tertiary neonatal intensive care unit between January 2020 and October 2021 who were diagnosed with moderate to severe BPD. Echocardiogram was performed at the postmenstrual age of 36 weeks or before discharge. The diagnosis of PH was based on the findings of echocardiogram. Prenatal and postnatal characteristics, demographic data, treatment details, and outcomes were collected and analyzed.ResultsA total of 139 VLBW infants with BPD were enrolled and divided into a PH group (n = 25) and a non-PH group (n = 114). The mean GA was 27.3 ± 2.3 weeks and the mean birth weight of infants with BPD was 927.3 ± 293.3 g. A multivariate logistic regression model revealed that a high positive end-expiratory pressure (PEEP) setting (OR: 2.105; 95% CI: 1.472–3.011; p < 0.001) in established BPD and surgical closure of patent ductus arteriosus (PDA; OR: 6.273; 95% CI: 1.574–24.977; p = 0.009) were associated with BPD–PH. Neonates with BPD who developed pulmonary hypertension remained hospitalized for longer (p < 0.001), received invasive mechanical ventilation support for longer (p < 0.001), had a higher incidence of retinopathy of prematurity (ROP; OR: 4.201; 95% CI: 1.561–11.304; p = 0.003), were more likely to require oxygen support at discharge (OR: 5.600; 95% CI: 2.175–14.416; p < 0.001), and were more likely to undergo tracheostomy (OR: 35.368; 95% CI: 4.03–310.43; p < 0.001).ConclusionPDA ligation and a higher PEEP setting were associated with BPD–PH in our cohort study. Compared with VLBW infants with BPD but without PH, infants with BPD and PH were hospitalized for longer, and also had a higher incidence of oxygen support after discharge, ROP, and tracheostomy

Headache in the Pediatric Emergency Service: A Medical Center Experience

BackgroundHeadache is a common complaint in children and is one of the most common reasons for presentation at a pediatric emergency department (PED). This study described the etiologies of patients with headache seen in the PED and determined predictors of intracranial pathology (ICP) requiring urgent intervention. A secondary objective was to develop rapid, practical tools for screening headache in the PED.MethodsWe conducted a retrospective chart review of children who presented with a chief complaint of headache at the PED during 2008. First, we identified possible red flags in the patients' history or physical examination and neurological examination findings. Then, we recorded the brain computed tomography results.ResultsDuring the study period, 43,913 visits were made to the PED; in 409 (0.9%) patients, the chief complaint was headache. Acute viral, respiratory, and febrile illnesses comprised the most frequent cause of headache (59.9%). Six children (1.5%) had life-threatening ICP findings. In comparison with the group without ICP, the group with ICP had a significantly higher percentage of blurred vision (p = 0.008) and ataxia (p = 0.002).ConclusionBlurred vision and ataxia are the best clinical parameters to predict ICP findings

CTLA-4+49 A/G polymorphism and antiglutamic acid decarboxylase antibody-associated encephalopathy in Taiwanese children

Background: Anti-glutamic acid decarboxylase antibodies are associated with encephalopathy, an autoimmune central nervous system inflammatory disease. The cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4)+49 A/G polymorphism has been shown to confer genetic susceptibility to positive anti-glutamic acid decarboxylase antibodies in patients with type 1 diabetes mellitus in Japan. We aimed to investigate the association of the CTLA-4+49 A/G (rs231775) polymorphism in Taiwanese children with anti-glutamic acid decarboxylase antibody-associated encephalopathy. ;Methods: This was a case-control study from July 2011 to June 2012 performed at Chang Gung Children's Hospital in Taiwan. Genotyping of the CTLA-4+49 A/G polymorphism was performed by polymerase chain reaction-restriction fragment length polymorphism. ;Results: Seventeen patients with anti-glutamic acid decarboxylase antibody-associated encephalopathy and 97 controls were enrolled. The genotype, allele and carrier frequencies of the CTLA-4+49 A/G polymorphism were equally distributed in the patients and controls, with no significant differences between the two groups. In addition, we found a positive trend between the level of antiglutamic acid decarboxylase antibodies and the G allele of the CTLA-4+49 A/G polymorphism, although this trend was not statistically significant. ;Conclusions: Our results suggest that the CTLA-4+49 A/G (rs231775) polymorphism does not confer an increased susceptibility to anti-glutamic acid decarboxylase antibody-associated encephalopathy in Taiwanese children. (c) 2015 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved

Outcome of Long-Term Mechanical Ventilation Support in Children

Improved technology and care in recent years have significantly improved the prognosis and quality of life for patients on long-term mechanical ventilation. This study examined the status of children on long-term mechanical ventilation (MV) support in Taiwan. Methods: The medical records of patients between January 1998 and December 2006 were retrospectively reviewed, and the clinical factors were systematically reviewed. Results: One hundred and thirty-nine (139) patients aged 3 months to 18 years, with 53 (38.1%) girls and 86 (61.9%) boys, were enrolled. The common underlying disorders included neurologic/neuromuscular diseases (n=100, 71.9%) and airway/lung dysfunction (n=19, 13.7%). After instituting MV, the children returned to the medical center mainly for infection (n=157, 47.7%) and elective surgery or procedures (n=46, 13.9%). After long-term follow-up, 37 (26.6%) died, 81 (58.3%) were transferred to respiratory care wards in local hospitals, and 21 (15.1%) received home care support. Conclusions: There are now more children on long-term MV support in Taiwan and most are in respiratory care wards in local hospitals. The shift in underlying diagnoses from pulmonary disease to neurogenic respiratory insufficiency affects hospitalization. The main cause of respiratory insufficiency is neurologic insult

Analysis of Fulminant Cerebral Edema in Acute Pediatric Encephalitis

Acute pediatric encephalitis with fulminant cerebral edema can rapidly become fatal or result in devastating neurological sequelae. Methods: All cases coded with the discharge diagnosis of acute encephalitis between January 2000 and December 2010 were reviewed. Of the 1038 children with acute pediatric encephalitis, 25 were enrolled in our study with ages ranging from 5 months to 16 years. Results: The major neurological symptoms included an altered level of consciousness (72%), vomiting (60%), and headache (48%). The onset of neurological symptoms to signs of brain herniation ranged from 0 days to 9 days. Nineteen (76%) patients had a seizure 24–48 hours prior to showing signs of fulminant cerebral edema, and 12 (48%) patients developed status epilepticus. Sixteen patients died, and no survivors returned to baseline. Risk factors for seizures and status epilepticus were compared between the fulminant cerebral edema group (n = 25, 19 seizures, including 12 status epilepticus) and control group (nonfulminant cerebral edema) (n = 1013, 444 seizures, including 141 status epilepticus; p = 0.001 for seizures and p < 0.001 for status epilepticus). Conclusion: Our findings indicate that preceding seizures and status epilepticus are significant risk factors for fulminant cerebral edema in children with acute encephalitis

Hemorrhagic Stroke Associated with Pulmonary Edema and Catastrophic Cardiac Failure

Cerebral arteriovenous fistula (AVF) is a vascular malformation that is rare in the pediatric population. Older children with cerebral AVF tend to present with neurologic problems related to intracranial venous hypertension or intracranial hemorrhage. Cardiac and pulmonary complications following acute neurologic injury such as subarachnoid hemorrhage are common in adults, but are rarely reported in children. However, complications have been reported in cases of enterovirus 71 rhombencephalitis in infants and children and can cause high morbidity and mortality. Here, we report a 14-year-old boy who presented with cardiac failure associated with pulmonary edema following cerebral hemorrhagic stroke due to AVF. After aggressive investigation and management, we intervened before significant hypoxia and hypotension developed, potentially reducing the risk of long-term adverse neurologic consequences in this patient

Clinical Features of Peritonsillar Abscess in Children

Peritonsillar abscess is an acute infection located between the capsule of the palatine tonsil and the superior constrictor muscle of the pharynx. The reports regarding pediatric cases have been scanty. Methods: A retrospective study was conducted to investigate the patients less than 18 years of age hospitalized at a medical center with a final diagnosis of peritonsillar abscess from January 1999 through December 2009. Only those who were confirmed by the drainage of pus from the peritonsillar spaces or confirmed by a computed tomography (CT) scan were enrolled. Results: In total, 56 children, 31 male and 25 female, were included. The mean age was 12.9 ± 4.6 years, ranging from 9 months to 17.9 years. Nineteen (34%) were less than 12 years of age. All 37 children ≧12 years of age complained of sore throat, but only 68% of children less than 12 years of age complained of sore throat. Thirty-one patients (55%) with a characteristic presentation of fever and sore throat plus an asymmetric swollen/bulging tonsil with or without uvular deviation suggestive of the diagnosis received surgical drainage at emergency department immediately, and 87% of them were older than 12 years of age. Twenty-five (45%) children needed a CT scan to confirm the diagnosis. The most common empirical antibiotics were penicillin-containing regimens. The predominant organisms identified were Streptococcus species. Eight children were treated successfully with antibiotics alone. No case was fatal. Conclusion: Two-thirds of the children with peritonsillar abscess were ≥12 years of age. Not every patient <12 years of age had a characteristic presentation, and a CT scan was usually needed to confirm the diagnosis

Nonketotic Hyperglycinemia of Infants in Taiwan

Nonketotic hyperglycinemia (NKH) is a rare, inherited disease, with very poor outcome. It is difficult to confirm the diagnosis due to nonspecific presentations and rapid progression. The incidence was reported in a few countries. We report the clinical and genetic features of typical neonatal NKH with novel splicing mutation, c.1058+3A>C, in the intron 7 of the glycine decarboxylase (GLDC) gene. Furthermore, this study aimed to delineate the estimated incidence and clinical characteristics of NKH in the Taiwanese population. Methods: Reports of Health Promotion Administration, Ministry of Health and Welfare of Taiwan, during the period from 2000 to 2013; the Human Gene Mutation Database; and literature regarding NKH in Taiwan were reviewed. Demographic information, age of onset, clinical characteristics, genetic analysis, electroencephalography examinations, and outcome of the patients were analyzed. Results: The estimated incidence of NKH in the Taiwanese population was 7.2 cases per 1,000,000 live births. Among the 12 cases reported in Taiwan, more than 90% were of neonatal type. Fifty-five percent of affected patients died within 5 years, and all survivors had severe neurologic outcomes. Only three infants underwent genetic analysis during the study period. Two neonatal NKH infants had mutation in the GLDC gene, and the other one, who had late-onset NKH, had mutation in the glutaredoxin 5 gene. Conclusion: Compared with other countries, the estimated incidence of NKH was relatively rare in the Taiwanese population. It is important to characterize all index cases at the genetic level. With more awareness of NKH, increased knowledge of gene mutation, and improvement of diagnostic tools, NKH can be diagnosed more accurately

Occult child abuse presenting as pneumatosis intestinalis and portomesenteric venous gas - a case report

Abstract Background Pneumatosis intestinalis and portomesenteric venous gas are usually caused by necrotizing enterocolitis; however they can occur secondary to abusive abdominal trauma with bone fractures and bruising. It is difficult to recognize initially if there is no bruising on the skin or bone fractures. Case presentation We report a 1-year-old child with no obvious history of trauma who presented with conscious disturbance. Abdominal computed tomography showed acute ischemic bowel complicated with pneumatosis intestinalis and portomesenteric venous gas. The first impression was septic shock with acute ischemic bowel. Two weeks after admission, brain magnetic resonance imaging showed subdural hemorrhage of different stages over bilateral fronto-parietal convexities and diffuse axonal injury, suggesting abusive head trauma. He was subsequently diagnosed with occult child abuse. Conclusion Pneumatosis intestinalis and portomesenteric venous gas are rare except in cases of prematurity. Occult abusive abdominal trauma should be considered as a differential diagnosis in patients with pneumatosis intestinalis and portomesenteric venous gas, even without any trauma on the skin or bone fractures

Hemodynamic Analysis of Pediatric Septic Shock and Cardiogenic Shock Using Transpulmonary Thermodilution

Septic shock and cardiogenic shock are the two most common types of shock in children admitted to pediatric intensive care units (PICUs). The aim of the study was to investigate which hemodynamic variables were associated with mortality in children with shock. We retrospectively analyzed 50 children with shock (37 septic shock cases and 13 cardiogenic shock cases) in the PICU and monitored their hemodynamics using transpulmonary thermodilution from 2003 to 2016. Clinical factors were analyzed between the patients with septic and cardiogenic shock. In addition, hemodynamic parameters associated with mortality were analyzed. The 28-day mortality was significantly higher in the septic group than in the cardiogenic group (p=0.016). Initially, the parameters of cardiac output and cardiac contractility were higher in the septic group (p<0.05) while the parameters of preload and afterload were all higher in the cardiogenic group (p<0.05). Cardiac index was significantly lower in the nonsurvivors of cardiogenic shock at the time of initial admission and after the first 24 hours (both p<0.05), while systemic vascular resistance index (SVRI) was significantly lower in the nonsurvivors of septic shock (p<0.001). Therefore, during the first 24 hours after intensive care, SVRI and cardiac index are the most important hemodynamic parameters associated with mortality