Factors Affecting Psychological and Health-Related Quality-of-Life Status in Children and Adolescents with Congenital Heart Diseases.

Congenital heart disease (CHD), a severe cardiac defect in children, has unclear influences on young patients. We aimed to find the impacts of differently structure heart defects and various treatments on psychology and health-related quality of life (HRQoL) in CHD children and adolescents. CHD patients aged between 6 and 18 years old visited our hospital from 1 May 2018 to 31 September 2018, and their principal caregivers were asked to participate. We used two validated questionnaires, Children Depression Inventory-TW (CDI-TW) and Child Health Questionnaire-Parent Form 50 (CHQ-PF 50), to evaluate CHD patients' psychological and HRQoL conditions. Participants were grouped based on their cardiac defects and previous treatments. We analyzed the results via summary independent-samples t-test with post hoc Bonferroni correction and multivariant analysis. Two hundred and seventy-seven children and their principal caregivers were involved. There was no apparent depressive condition in any group. Single cardiac defect patients exhibited similar HRQoL to controls; simultaneously, those with cyanotic heart disease (CyHD), most multiple/complex CHDs children and adolescents, and those who received invasive treatments had poorer HRQoL. CyHD impacted the most on patients' psychological and HRQoL status. Patients with sole cardiac defect could live near-normal lifes; on the other hand, CyHD had the worst effects on patients' psychology and HRQoL.

Serotype distribution and clinical correlation of Streptococcus agalactiae causing invasive disease in infants and children in Taiwan.

BACKGROUND: Streptococcus agalactiae, or group B Streptococcus (GBS), remains to be one of the leading pathogens causing invasive infections in infants. METHODS: The clinical GBS isolates from sterile sites of patients younger than 18 years old were collected from October 1998 to December 2014 in two hospitals in Taiwan. Medical records were retrospectively reviewed. Every isolate was serotyped with a multiplex PCR assay. Multilocus sequence typing (MLST) was performed in representative isolates of different serotypes. A total of 205 GBS isolates were collected from 181 patients with 182 infection episodes. RESULTS: Serotype Ia was the most common in patients less than 72 h old, whereas III the most common in patients older than 72 h. In early-onset disease (0-6 days), Ia and III each caused 27.5% of the infection, followed by Ib (14.5%). In late-onset disease (7-89 days), serotype III predominated (75.3%), followed by Ia (10.1%) and Ib (6.8%). Thirty-one episodes (17%) were complicated with culture-confirmed meningitis. We compared serotype Ia and III patients, and found that serotype Ia patients were significantly younger (median age, 3 days), had more perinatal maternal fever and higher mortality. ST17 and ST19 were exclusively found in serotype III, while ST23 and ST24 comprised of 85% of serotype Ia. CONCLUSION: In Taiwan, serotypes Ia and III are the most common cause for early-onset and late-onset neonatal GBS infections, respectively. Some differences in the clinical features of invasive GBS infections caused by serotype Ia and III were observed.

Acute flaccid myelitis associated with enterovirus D68 infection: A case report.

RATIONALE: We present the first case of enterovirus (EV) D68, lineage B3 infection, associated with acute flaccid myelitis (AFM) in Taiwan. AFM caused by EV D68 is relatively rare. This report highlights the importance of clinical recognition of the disease and discusses treatments that can benefit such patients. PATIENT CONCERNS: A 5-year-old boy experienced sudden onset of acute flaccid paralysis (AFP) involving left arm after fever and respiratory symptoms for 3 days. DIAGNOSES: Magnetic resonance imaging (MRI) of the spinal cord revealed signal changes over segments C1 to T5 on a T2-weighted image (T2WI), compatible with the diagnosis of AFM. The EV D68 strain, cultured from the throat of the patient was identified. INTERVENTIONS: We administered intravenous immunoglobulin (IVIG, 1g/kg, twice), pulse steroid therapy (methylprednisolone, 30 mg/kg, twice) and oral prednisolone (1mg/kg/day). Rehabilitation was also arranged. OUTCOMES: The patient still had mild muscle atrophy over left arm after following-up for 1 year. LESSONS: Early diagnosis and prompt management are essential for managing this kind of patient. IVIG, pulse therapy, and oral prednisolone may play crucial roles in controlling its clinical course.

A Case Report and Literature Review of Scrub Typhus With Acute Abdomen and Septic Shock in a Child-The Role of Leukocytoclastic Vasculitis and Granulysin.

Scrub typhus is becoming a clinically important cause of acute undifferentiated febrile illness in Taiwan. The incubation period is between 6 and 21 days after exposure. It is transmitted by chiggers (larva of trombiculid mite) in long grasses and in dirt-floor homes, with infection characterized by a flu-like illness of fever, headache, and myalgia lasting approximately 1 week. It has various systemic manifestations, including GI symptoms. In some, the illness progresses to multiorgan dysfunction syndrome and death. We report on a 13-year-old boy who lived in Taipei City and who had initially tentative diagnosis of acute pyrexia of unknown origin with high fever up to 40.3°C for 1 week, but later had thrombocytopenia and diffuse abdominal pain with peritoneal sign suspected acute appendicitis. During the clinical course, septic shock and disseminated intravascular coagulopathy (DIC) were noted. There were skin rash in his trunk and extremities and an eschar with black crust surrounded by a scaling erythematous rim on his right buttock. In addition, we got the information of his travel history in Green Island and Orchid Island for 10 days.With the correct antibiotics, vancomycin, meropenem, and doxycycline, the patient was getting better and corresponding with high level of granulysin and tumor necrosis factor-alpha. The diagnosis of scrub typhus was confirmed by the biopsy of eschar and high quantitative real-time polymerase chain reaction values of Orientia tsutsugamushi (16sRNA and 56 kDa) tested by Centers for Disease Control and Prevention, Taiwan. Histopathological findings of the eschar revealed the leukocytoclastic vasculitis, crust and thrombus formation with many gram-negative microorganisms, O. tsutsugamushi demonstrated by 47 kDa monoclonal antibody immunohistochemical stain and electromicroscopy. OUTCOMES: After the careful selection of appropriate antibiotics including meropenem, vancomycin, and doxycycline, he recovered and was subsequently discharged 7 days after admission. LESSON SUBSECTIONS: This case highlights that scrub typhus infection can mimic acute abdomen and septic shock with DIC. This rare presentation of acute abdomen and septic shock with thrombocytopenia and DIC caused by scrub typhus should remind physicians to be alert to the possibility of acute abdomen and febrile illness resulting from scrub typhus.

Mycoplasma pneumoniae-associated encephalitis complicated by cerebral salt wasting syndrome.

Cerebral salt wasting syndrome can occur in children with encephalitis. Clinicians should be aware of hyponatremia in patients who develop polyuria with the signs of dehydration and deteriorated consciousness. Furthermore, patients who present with status epilepticus or who are suspected to have high intracranial pressure may have an increased risk of cerebral salt wasting syndrome.

Clinical analysis of fever of unknown origin in children: A 10-year experience in a northern Taiwan medical center.

BACKGROUND: Fever of unknown origin (FUO) was first described in 1961 as fever >38.3°C for at least 3 weeks with no apparent source after 1 week of investigations in the hospital. Infectious disease comprises the majority of cases (40-60%). There is no related research on FUO in children in Taiwan. The aim of this study is to determine the etiologies of FUO in children in Taiwan and to evaluate the relationship between the diagnosis and patient's demography and laboratory data. METHODS: Children under 18 years old with fever >38.3°C for >2 weeks without apparent source after preliminary investigations at Taipei Veterans General Hospital during 2002-2012 were included. Fever duration, symptoms and signs, laboratory examinations, and final diagnosis were recorded. The distribution of etiologies and age, fever duration, laboratory examinations, and associated symptoms and signs were analyzed. RESULTS: A total of 126 children were enrolled; 60 were girls and 66 were boys. The mean age was 6.7 years old. Infection accounted for 27.0% of cases, followed by undiagnosed cases (23.8%), miscellaneous etiologies (19.8%), malignancies (16.6%), and autoimmune disorders (12.7%). Epstein-Barr virus (EBV) and cytomegalovirus (CMV) were the most commonly found pathogens for infectious disease, and Kawasaki disease (KD) was the top cause of miscellaneous diagnosis. CONCLUSIONS: Infectious disease remains the most common etiology. Careful history taking and physical examination are most crucial for making the diagnosis. Conservative treatment may be enough for most children with FUO, except for those suffering from malignancies.

Globotriaosylsphingosine (lyso-Gb3) might not be a reliable marker for monitoring the long-term therapeutic outcomes of enzyme replacement therapy for late-onset Fabry patients with the Chinese hotspot mutation (IVS4+919G>A).

BACKGROUND: In Taiwan, DNA-based newborn screening showed a surprisingly high incidence (1/875 in males and 1/399 in females) of a cardiac Fabry mutation (IVS4 + 919G > A). However, the natural course, long-term treatment outcomes and suitable biomarkers for monitoring the therapeutic outcomes of these patients are largely unknown. METHODS: Fabry disease (FD) patients who had received enzyme replacement therapy (ERT) for more than 1 year were enrolled in this study from December 2008 to April 2013. Periodic echocardiography and serum globotriaosylsphingosine (lyso-Gb3) analysis were carried out. Before and after ERT, left ventricular mass index (LVMI) and serum lyso-Gb3 level were compared and the correlation between the change of LVMI and the change of serum lyso-Gb3 were also analyzed. RESULTS: Thirty-six patients, in four patient groups, were enrolled: (1) 16 males with IVS4 + 919G > A mutation; (2) 7 females with IVS4 + 919G > A mutation; (3) 2 males with classical mutations; and (4) 11 females with classical mutations. The follow-up period was 13-46 months. There were significant LVMI reductions after ERT in all four groups after excluding confounding factors. However, interestingly, serum lyso-Gb3 decreased significantly in the early period after ERT in all groups, but increased gradually after an average of 11.1 months after ERT in late-onset male and female Fabry groups, even when their LVMI still decreased or remained stable. Furthermore, there was no correlation between the change of serum lyso-Gb3 and the change of LVMI in both classical and IVS4 + 919G > A FD patients. CONCLUSION: Although lyso-Gb3 has a high diagnostic sensitivity in late-onset Fabry patients and has a good response to ERT during the early stages, it might not be a reliable marker for monitoring the long-term therapeutic outcomes of ERT for late-onset Fabry patients with the Chinese hotspot mutation (IVS4 + 919G > A).

Incidence and renal survival of ESRD in the young Taiwanese population.

BACKGROUND AND OBJECTIVES: ESRD in the young represents a heavy burden to patients, families, and health care systems. This nationwide retrospective study characterized the incidence of ESRD and analyzed diagnoses associated with renal survival in the young population in Taiwan. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Through use of Taiwan's National Health Insurance Research Database, the population of young patients (age<30 years, including children and young adults) with ESRD between January 1998 and December 2009 were enrolled. The medical claims were used to derive the date when the cause of ESRD was first determined. The medical data were reviewed and the renal survival time (time from first diagnosis of the cause to the start of ESRD) was calculated by experts, including clinical physicians and a large-database specialist. RESULTS: The incidence rate of ESRD in the young population was high compared with the worldwide rate at 21.1 per million person-years, whereas the incidence in the pediatric group was still similar to that in other countries at 10.3 per million person-years. A total of 2304 patients with new-onset ESRD and identified renal diseases during the study period were enrolled. All preschool-age patients (100%) began receiving peritoneal dialysis as their initial treatment for ESRD. The leading causes, which varied by sex and onset age, were glomerulonephropathy followed by hypertension for the young adult group and glomerulonephropathy followed by congenital anomalies of the kidney and urinary tract (CAKUT) for the pediatric group. Renal survival was cause-dependent. The median overall renal survival duration was 0.8 year (interquartile range [IQR], 0.7-3.5 years). CAKUT-related ESRD had the longest progression time (median renal survival, 16.0 years; IQR, 10.7-23.5 years); glomerulonephropathy progressed more rapidly into ESRD and had the shortest median renal survival of 0.5 year (IQR, 0.1-2.7 years). CONCLUSIONS: The incidence and causes of ESRD greatly differ between pediatric patients and young adults. Moreover, renal survival in the young population markedly varies depending on the cause of renal disease.

Clinical manifestations vary with different age spectrums in infants with Kawasaki disease.

BACKGROUND: Kawasaki disease (KD) is an acute systemic vasculitis with unknown etiology. The diagnosis of KD depends on clinical manifestations. The prevalence of coronary artery abnormality (CAA) is 11.0% and results in cardiac sequelae, such as myocardial infarction or coronary aneurysm, which are the most serious complications in KD. METHODS: We divided KD's children into different age groups: ≤6 months old, 7 months to 1 year old, and >1 year old, respectively. Different parameters were compared in each group. RESULTS: Infants ≤6 months old are less likely to fulfill KD's major diagnostic criteria within 10 days, are prone to develop incomplete KD with the lowest cholesterol level, and have the greatest chance to have CAA and the laboratory features associated with CAA, such as the longest time needed to confirm CA diagnosis, lower hemoglobin level, lower albumin level, and higher platelet count. Infants <1 year old develop higher percentage of leukocytosis and sterile pyuria. But this group has fewer patients with neck lymphadenopathy.

Various modalities for evaluation of a fused heart in conjoined twins.

Conjoined twins are a rare congenital anomaly with an estimated incidence of 1/50,000 to 1/100,000. Among thoracopagus conjoined twins, 75% have a fused heart. We compare the usefulness of various modalities for evaluating cardiovascular structure in fused-heart conjoined twins. We report a series of 20 sets of thoracopagus conjoined twins as well as the results of a PubMed database literature review literature from 1982 to 2009. Twenty sets of fused-heart thoracopagus conjoined twins were evaluated by echocardiography, cardiac catheterization, magnetic resonance image (MRI), and three-dimensional computed tomography angiography (3D-CTA). Imaging results were compared to findings at surgery or autopsy. All sets of conjoined twins underwent postnatal echocardiography; 11 sets (55%) underwent cardiac catheterization; 4 sets (20%) underwent MRI; and 1 set (5%) underwent 3D-CTA. All intracardiac anatomy (ICA) was identified by echocardiography. Cardiac catheterization, MRI, and 3D-CTA were able to identify extracardiac vascular structures as well as the ICA. 3D-CTA, which can be performed as early as the first week of life, is a noninvasive, less expensive, and the safe examination with minimal risk due to its short procedural time. Three-dimensional CTA is an effective and safe modality for evaluating the cardiovascular anatomy of fused-heart conjoined twins before surgery.

Application of a molecular method for the classification of human enteroviruses and its correlation with clinical manifestations.

BACKGROUND/PURPOSE: A new molecular classification scheme has recently been adopted that groups all enteroviruses into four species, designated human enterovirus A (HEV-A) through D. In this study, we tried to demonstrate the correlation between this molecular classification scheme and clinical manifestations in patients. METHODS: We retrospectively reclassified the clinical isolates of enteroviruses from the preceding 4.5 years in our virology laboratory using reverse transcription-polymerase chain reaction, and reviewed the clinical manifestations of 138 pediatric patients. RESULTS: We reclassified 23 isolates of the five serotypes into the HEV-A group, 110 isolates of 16 sero-types into the HEV-B group, five isolates into the HEV-C group, and no isolate of the HEV-D group. HEV-A species caused significantly more hand-foot-and-mouth disease (p < 0.001), herpangina (p = 0.029), and myoclonic jerks (p < 0.001) compared with HEV-B species. However, HEV-B species caused significantly more pharyngitis (p = 0.043), respiratory tract infections (p = 0.046), nausea and vomiting (p = 0.007), and aseptic meningitis (p = 0.001). The only death in our report was caused by coxsackievirus A16, which belonged to the HEV-A group. CONCLUSION: The association between the molecular classification of enteroviruses and related disease patterns is an important finding. We suggest that this molecular classification could be applied in a clinical laboratory as an alternative method under certain circumstances, such as limited availability of antisera or questionable serotyping results, to identify the untypeable isolates.

Therapeutic lung lavage with diluted surfactant in neonates with severe meconium aspiration syndrome.

Meconium aspiration syndrome (MAS) may result in considerable morbidity and mortality in newborn infants. The current standard treatment is still in need of improvement for the most severe patients. We report 3 cases with devastating MAS that was successfully treated with therapeutic lung lavage. These cases were all delivered in local obstetrics clinics or hospitals with meconium-stained amniotic fluid and non-vigorous appearance at birth. However, no endotracheal suction was performed when they were born. All of them suffered from severe hypoxia and unstable vital signs despite there being high ventilatory settings when they were transferred to the tertiary medical center. Therapeutic lung lavage with diluted surfactant (Survanta, 5 mg/mL, 30 mL/kg in 2 aliquots) was performed within 24 hours of age. Bloody fluid (about 40-50% of total lavage amount) was recovered in all 3 cases. Although brief desaturation and bradycardia were observed during the procedures, 2 of them tolerated the procedures well and improved soon after lavage. The other patient received lung lavage in a relatively unstable condition and needed chest tapping to relieve bilateral pleural effusion. Their respiratory condition improved after the procedures, and they were all discharged within 1 month without major respiratory complications. These successful experiences are compatible with previous animal studies and other case reports with different lavage protocols. We conclude that therapeutic lung lavage may improve the outcome in newborn infants with severe MAS, and there were no significant adverse side effects observed. Before performing lung lavage, stabilization and optimal support may prevent unexpected results during and after lavage.