Development of necrotizing enterocolitis in full-term infants with duct dependent congenital heart disease.

BACKGROUND: Although many studies have described an increased risk of necrotizing enterocolitis in duct dependent congenital heart diseases, very few have investigated its occurrence in full-term infants with duct dependent congenital heart diseases. METHODS: To evaluate the characteristics and risk factors of necrotizing enterocolitis, we performed a retrospective review of 355 full-term infants with duct dependent congenital heart diseases who received prostaglandin E1 therapy from April 2000 to May 2020. RESULTS: Necrotizing enterocolitis was observed in 10 patients (3.0%). Their average gestational age and birth weight were 38.2 weeks and 2783.5 g, respectively. The median age at diagnosis was 8.0 days (2-70 days). One patient was diagnosed with necrotizing enterocolitis stage IIA, five with stage IIB, two with stage IIIA, and two with stage IIIB; two (20%) received surgical treatment. The duct dependent pulmonary circulation group had higher frequencies of necrotizing enterocolitis (4.4%) than the duct dependent systemic circulation (2.0%) and parallel circulation (1.3%) groups. The necrotizing enterocolitis and the other groups had significantly different birth weight (2783.5 g vs 3170.9 g, respectively) and gestational age (38.2 weeks vs 39.1 weeks, respectively). Gestational age under 38 weeks (OR 8.87, p = 0.002), birth weight of < 2500 g (OR 5.1, p = 0.042), need for mechanical ventilation (OR 4.6, p = 0.021), parenteral nutrition (OR 107.7, p < 0.001), and functional single ventricle (OR 5.8, p = 0.009) were significant risk factors. The case-fatality rate was higher in the necrotizing enterocolitis (40.0%) than in the other group (8.3%, p = 0.009). CONCLUSIONS: Three percent of full-term infants with duct dependent congenital heart diseases developed necrotizing enterocolitis. Neonates with low birth weight, gestational age less than 38 weeks, functional single ventricle, or receiving assisted mechanical ventilation or parenteral nutrition are at increased risk.

How can stretched ductus diameter be predicted from angiographic diameter?

BACKGROUND: An appropriate size of device for patent ductus arteriosus (PDA) could be chosen by a stretched PDA size. We propose prediction of stretched size from intact PDA size. METHODS: A total of 361 patients was enrolled. Intact size was measured on angiography before closure, and stretched size was the narrowest diameter immediately after device implantation. RESULTS: The median patient age was 2.8 years. Intact diameter and stretched diameter were 3.6±1.4 mm and 5.1±1.3 mm. The difference and difference ratio were 1.5±0.8 mm and 51.3±38.1% and had negative linear correlations with age (P<0.001). Patients were divided into four groups: <2 years old (159), 2-15 years old (68), 15-40 years old (72), and ≥40 years old (62). Among groups, difference and difference ratio were significantly different. Stretched diameter was inferred by the following formulas: stretched diameter = 0.660 × intact diameter + 2.791 (<2 years old); = 0.971×intact diameter + 0.058 × age + 1.131 (2-15 years old); = 0.790 × intact diameter + 2.263 (15-40 years old); and = 0.837 × intact diameter + 1.353 × BSA - 0.096 (≥40 years old). CONCLUSIONS: Stretched PDA diameter can be predicted from intact diameter and might be helpful for safe PDA closure especially in small infants.

Influenza-associated Neurologic Complications in Hospitalized Pediatric Patients: A Multicenter Retrospective Study in Republic of Korea.

BACKGROUND: The rates of influenza-associated neurologic complications are variable among studies, and a difference has been observed between the Western and Asian countries. The study aims to evaluate the frequency and characteristics of influenza-associated neurologic complications. METHODS: We performed a retrospective review of hospitalized cases of influenza infection from October 2010 to April 2017 from 3 referral hospitals. RESULTS: A total of 1988 influenza cases were identified. Influenza-associated neurologic complications were 161 cases (8.1%); influenza virus A was detected in 113 (70.2%) cases, B in 47 (29.2%) cases and both A and B in 1 case (0.6%). Twenty-four patients (15%) had underlying neurologic diseases. The most common diagnosis was a simple febrile convulsion (44%), followed by complex febrile convulsion (29%), fever-provoked seizure under pre-existing neurologic disease or afebrile seizure (14%), encephalopathy/encephalitis (8%) and meningitis (5%). Most of the patients fully recovered (96%). Three patients (1.9%) died of myocarditis (n = 1), encephalopathy (n = 1), and simultaneous encephalitis and myocarditis (n = 1). Pre-existing neurologic disease, age groups of 6 months to 6 years and 6-12 years were a risk factor of influenza-associated neurologic complications with an adjusted odds ratio of 5.41 (95% confidence interval [CI] 3.23-9.06, P < 0.001), 12.99 (95% CI 1.77-95.19, P = 0.01) and 8.54 (95% CI 1.14-64.79, P = 0.04), respectively. There was no association between neuropsychiatric adverse events and oseltamivir prescription (P = 0.17). CONCLUSIONS: Influenza-associated neurologic complications are not uncommon, and most patients fully recovered. The frequency of influenza-associated neurologic complications in Korean children was not significantly different from that of children in Western countries.

Fatal Systemic Capillary Leak Syndrome after SARS-CoV-2Vaccination in Patient with Multiple Myeloma.

A young man with smoldering multiple myeloma died of hypotensive shock 2.5 days after severe acute respiratory syndrome coronavirus 2 vaccination. Clinical findings suggested systemic capillary leak syndrome (SCLS); the patient had experienced a previous suspected flare episode. History of SCLS may indicate higher risk for SCLS after receiving this vaccine.

Childcare Exposure to Severe Acute Respiratory Syndrome Coronavirus 2 for 4-Year-Old Presymptomatic Child, South Korea.

Data on transmission of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) from preschool-age children to children and adults are limited. We investigated SARS-CoV-2 exposure at a childcare center in South Korea. A 4-year-old child, probably infected by his grandmother, attended the center during the presymptomatic period (February 19-21, 2020). Fever developed on February 22, and he was given a diagnosis SARS-CoV-2 infection on February 27. At the center, 190 persons (154 children and 36 adults) were identified as contacts; 44 (23.2%) were defined as close contacts (37 children and 7 adults). All 190 persons were negative for SARS-CoV-2 on days 8-9 after the last exposure. Two close contacts (1 child and 1 adult) showed development of symptoms on the last day of quarantine. However, subsequent test results were negative. This investigation adds indirect evidence of low potential infectivity in a childcare setting with exposure to a presymptomatic child.

The First Korean Case of Baraitser-Winter Cerebro-Fronto-Facial Syndrome with a Novel Mutation in ACTB Diagnosed Via Targeted Gene Panel Sequencing and Literature Review.

Baraitser-Winter Cerebro-fronto-facial syndrome (BWCFF, OMIM #243310, #614583) is caused by a heterozygous gain-of-function mutation of ACTB and ACTG1 that encodes actin. The syndrome is characterized by striking facial features, structural brain abnormalities, ocular coloboma, hearing loss, cardiac defects, intellectual disabilities, short stature, and developmental delay. We report a two-year-old girl who had distinctive facial features, including hypertelorism, arched eyebrows, bilateral ptosis, short broad nose with a flat nasal tip, long philtrum, retrognathia, low-set ears, and a thin upper lip. In addition, she also exhibited short stature, pectus excavatum, developmental delay, brain malformation, and hearing loss. Targeted gene panel sequencing identified a de novo heterozygous missense variant c.826G>A (p.Glu276Lys) in ACTB This is the first Korean case of BWCFF with a novel mutation in ACTB.

Outcomes of transcatheter closure of ductus arteriosus in infants less than 6 months of age: a single-center experience.

PURPOSE: Transcatheter device closure of patent ductus arteriosus (PDA) is challenging in early infancy. We evaluated PDA closure in infants less than 6 months old. METHODS: We performed a retrospective review of infants less than 6 months of age who underwent attempted transcatheter device closure in our institution since 2004. To compare clinical outcomes between age groups, infants aged 6-12 months in the same study period were reviewed. RESULTS: A total of 22 patients underwent transcatheter PDA closure during the study period. Patient mean age was 3.3±1.5 months, and weight was 5.7±1.3 kg. The duct diameter at the narrowest point was 3.0±0.8 mm as measured by angiography. The most common duct type was C in the Krichenko classification. Procedural success was achieved in 19 patients (86.3%). Major complications occurred in 5 patients (22.7%), including device embolization (n=1), acquired aortic coarctation (n=2), access-related vascular injury requiring surgery (n=1), and acute deterioration requiring intubation during the procedure (n=1). Two patients had minor complications (9.1%). Twenty-four infants aged 6-12 months received transcatheter device closure. The procedural success rate was 100%, and there were no major complications. The major complication rate was significantly higher in the group less than 6 months of age (P=0.045). There was a trend toward increased major complication and procedural failure rates in the younger age group (P<0.01). CONCLUSION: A relatively higher incidence of major complications was observed in infants less than 6 months of age. The decision regarding treatment modality should be individualized.

Severe hematuria in a hematopoietic cell transplant recipient caused by Ureaplasma urealyticum not by BK virus or adenovirus infection.

A 17-year-old male with acute lymphoblastic leukemia developed severe hematuria and scrotal swelling after haploidentical hematopoietic cell transplantation (HCT). Urine culture was negative. BK virus and adenovirus were negative. However, Ureaplasma urealyticum was detected. He showed dramatic improvement after doxycycline treatment. This is the first report in the literature of hemorrhagic cystitis caused by U. urealyticum in a HCT recipient. In HCT recipients with hemorrhagic cystitis, U. urealyticum should be considered as a potential cause.