Suppressed pediatric asthma hospitalizations during the COVID-19 pandemic in Japan, from a national survey.

BACKGROUND: Acute asthma exacerbation in children is often caused by respiratory infections. In this study, a coordinated national surveillance system for acute asthma hospitalizations and causative respiratory infections was established. We herein report recent trends in pediatric acute asthma hospitalizations since the COVID-19 pandemic in Japan. METHODS: Thirty-three sentinel hospitals in Japan registered all of their hospitalized pediatric asthma patients and their causal pathogens. The changes in acute asthma hospitalization in children before and after the onset of the COVID-19 pandemic and whether or not COVID-19 caused acute asthma exacerbation were investigated. RESULTS: From fiscal years 2010-2019, the median number of acute asthma hospitalizations per year was 3524 (2462-4570), but in fiscal years 2020, 2021, and 2022, the numbers were 820, 1,001, and 1,026, respectively (the fiscal year in Japan is April to March). This decrease was observed in all age groups with the exception of the 3- to 6-year group. SARS-CoV-2 was evaluated in 2094 patients from fiscal years 2020-2022, but the first positive case was not detected until February 2022. Since then, only 36 of them have been identified with SARS-CoV-2, none of which required mechanical ventilation. Influenza, RS virus, and human metapneumovirus infections also decreased in FY 2020. In contrast, 24% of patients had not been receiving long-term control medications before admission despite the severity of bronchial asthma. CONCLUSION: SARS-CoV-2 was hardly detected in children with acute asthma hospitalization during the COVID-19 pandemic. This result indicated that SARS-CoV-2 did not induce acute asthma exacerbation in children. Rather, infection control measures implemented against the pandemic may have consequently reduced other respiratory virus infections and thus acute asthma hospitalizations during this period. However, the fact that many hospitalized patients have not been receiving appropriate long-term control medications is a major problem that should be addressed.

Neonatal case of novel KMT2D mutation in Kabuki syndrome with severe hypoglycemia.

A newborn Japanese girl with Kabuki syndrome had neonatal persistent hyperinsulinemic hypoglycemia, which seemed to be a rare complication of Kabuki syndrome. On sequence analysis she was found to have a novel heterozygous KMT2D mutation. Diazoxide therapy was effective for the hypoglycemia. Hypoglycemia should be considered when Kabuki syndrome patients have convulsion or other non-specific symptoms. Diazoxide may help to improve hypoglycemia in patients with Kabuki syndrome complicated with hyperinsulinemic hypoglycemia.

[A case of neurofibromatosis-1 in which severe bleeding was observed from a neurofibroma under the scalp].

Neurofibroma is a representative external abnormality observed along with café-au-lait spots in association with neurofibromatosis type 1 (NF-1). We encountered a case of NF-1 in which severe bleeding was observed from a neurofibroma under the scalp due to minor trauma. Only four similar cases have been reported in the past literature and we believed that it was a significantly rare case, and we herein report the case with bibliographical considerations. The subject was a 23-year-old male. He was gently hit on the right side of the head during work and the bruised site gradually became bloated. Even on the following day, the bloating continued and he also started feeling severe pain, and as a result, he visited our emergency department. A head CT scan revealed a subcutaneous high-density area from the right-frontal area of the head to the side of the head that appeared to be a hematoma. The pain was severe and we therefore performed emergency surgery to remove the subcutaneous hematoma, but due to severe bleeding during the operation, we ultimately removed only part of the hematoma. However, because the pain was relieved, he was discharged from the hospital and he subsequently stopped visiting the hospital regularly. Three years later, he visited our department again with similar head bloating due to a mild head bruise. When surgery was performed again, an obvious neoplastic lesion was observed along with the subcutaneous hematoma. The pathological findings suggested it was a neurofibroma but no malignant findings were observed.

True intraspinal neurenteric cyst in the lumbosacral region--case report.

A 56-year-old man presented with a very rare true neurenteric cyst in the conus medullaris without evidence of vertebral or visceral anomaly manifesting as a 6-month history of mild low back and bilateral inguinal pain. No motor weakness was found in the bilateral lower extremities. He had also suffered dysesthesia in the bilateral feet for several weeks before admission. Lumbar spine magnetic resonance (MR) imaging demonstrated a cystic intradural extramedullary mass at the L1-2 levels without enhancement after gadolinium injection. MR imaging, computed tomography, and radiography detected no vertebral anomaly. Lumbar laminectomy at the L1-2 levels was performed and the lesion was incompletely removed. Histological examination showed the cystic wall lined with ciliated columnar epithelium. Neurenteric cyst should be considered in the diagnosis of isolated cystic mass lesion at the lumbosacral region even in the absence of vertebral or visceral abnormality.

Genotype-phenotype correlation of 5p-syndrome: pitfall of diagnosis.

To clarify the genotype-phenotype correlation of 5p- syndrome, FISH analyses were performed for six patients by using a series of probes spanning 5p13.1-p15.33. Genotypically, break points of deletion were quite different. Three of the six patients were diagnosed as interstitial deletion on chromosome 5p by G-banding method and FISH analysis; however, all of them proved to be entire distal deletions of 5p caused by unbalanced chromosomal translocations. Furthermore, one 5p- syndrome patient was diagnosed only by the FISH analysis using a single probe but not by ordinary chromosomal analyses. Therefore, when ordinary chromosomal analysis cannot detect any deletion in a patient who is phenotypically suspected of 5p- syndrome, multiple FISH analysis or parental chromosomal analysis would be needed for correct diagnosis. Interestingly, one patient with terminal deletion between 5p15.31-pter lacks mental retardation and cat-like crying, indicating that this region might not be responsible for those cardinal features of 5p- syndrome. Further studies on genotype-phenotype correlation will help us better understand 5p- syndrome and also determine functional mapping of the 5p region.

[Middle meningeal artery embolization for refractory chronic subdural hematoma: 3 case reports].

The authors present three cases of refractory chronic subdural hematoma (CSDH) treated by embolization of the middle meningeal artery (MMA) after several unsuccessful drainage procedures. The patients were initially treated by the usual method of burr hole and irrigation of the hematoma. After recurrence, several percutaneous puncture and drainage procedures were unable to prevent re-collection of the hematoma. Then the authors embolized the MMA which was thought to be the feeding artery of the outer membrane of the hematoma cavity. No enlargement of the hematoma was seen after embolization and, gradually, complete resolution of the hematoma was obtained. The outcome of the patients was excellent in all three cases. This new therapeutic approach to recurrent CSDH is discussed.