Asahikawa Kosei Hospital

Myoclonic epilepsy in infancy (MEI) is a rare epilepsy characterized by myoclonic seizures that begin between 4 months and 3 years of age.1-3 These seizures occur during wakefulness or drowsiness, mainly involving the upper limbs and head, and may appear in clusters, making their differentiation from epileptic spasms difficult1-3 (Video 1). Epileptic spasms are the hallmark of infantile epileptic spasms syndrome (IESS), which may lead to developmental stagnation or regression. Early diagnosis and treatment of IESS are crucial, even when developmental delay or typical hypsarrhythmia is absent.1 Therefore, distinguishing between seizure types is essential. Myoclonic seizures show brief electromyographic (EMG) bursts (<50 ms) without sustained tonic components, whereas epileptic spasms consist of an initial phasic contraction (<2 s) followed by a less intense tonic phase lasting 2–10 s4-6 (Table 1). These features aid in accurate diagnosis. We thank the patient and his family for their participation and for providing consent for the publication of this case report. The authors declare no conflicts of interest. The data that support the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions. Data S1: Tanaka, teaching slides. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article. Answers may be found in the supporting information.

We aimed to clarify the clinical characteristics and outcomes of patients with in-hospital onset ischemic stroke (IOS) compared with those in patients with community-onset ischemic stroke (COS). Patients from the Japan Stroke Data Bank, a hospital-based multicenter prospective registry, who were diagnosed with acute ischemic stroke (AIS) within 24 h of onset between January 2001 and December 2020 were included in this study. We assessed favorable outcomes at discharge corresponding to a modified Rankin Scale (mRS) score of 0-2, unfavorable outcomes corresponding to an mRS score of 5-6, and mortality. We also examined trends in these outcomes at 4-year intervals over a period of 20 years. Of the 100,865 patients analyzed, 2979 had IOS (1416 women, mean age 77 ± 12 years) and were older than those with COS (n = 97,886; 39,110 women, mean age 74 ± 12 years). Multivariate analysis revealed that younger age, higher premorbid mRS score, absence of stroke history, normotension, congestive heart failure, coronary artery disease, chronic kidney disease, liver disease, malignancy, tendency to bleed, and cardioembolic stroke were positively associated with IOS. Compared with COS, IOS was inversely associated with a favorable outcome (42.1% vs 64.8%, adjusted odds ratio [aOR] 0.72 [95% confidence interval (CI) 0.63-0.82]), positively associated with an unfavorable outcome (mRS 5-6 at discharge; 34.3% vs 15.5%, aOR 1.31 [95% CI 1.16-1.48]), and mortality (11.8% vs 4.6%, aOR 1.59 [95% CI 1.37-1.84]). Over 20 years, the mortality rate significantly decreased in both patients with IOS and COS (p < 0.01 both). IOS is associated with unfavorable outcomes and higher mortality rates during acute hospitalization. The mortality rates in patients with IOS decreased over time, similar to those observed in patients with COS.

Forty-five cases with aquaporin-4 antibody-positive neuromyelitis optica spectrum disorders (NMOSD) who underwent convalescent rehabilitation were studied. After excluding three cases with recurrence during rehabilitation treatment, the Expanded Disability Status Scale of Kurtzke improved a median of 1 point. Corticosteroids were the most used disease-modifying drugs (DMDs). Three cases relapsed during rehabilitation treatment. Six cases developed febrile infections. Thirty-four cases were discharged home, but half of the cases in their 80s were transferred to a medical care hospital. In the rehabilitation treatment of NMOSD, reducing the risk of recurrence by appropriate DMDs and preventing infections are important. Information sharing using a regional collaborative medical care plan is useful.

Acute myeloid leukemia (AML) is an aggressive hematological malignancy with genetic alterations. The FMS-like tyrosine kinase 3 (FLT3) gene is frequently mutated in adult de novo AML, with two types of mutations: internal tandem duplication (ITD) and point mutations in the tyrosine kinase domain. This study aimed to investigate the impact of FLT3 inhibitors and hematopoietic cell transplantation (HCT) on survival outcomes in patients with FLT3-ITD AML in a real-world setting. We retrospectively analyzed 139 patients with FLT3-ITD-positive AML between 2012 and 2021. The median age was 63 years. In the propensity score-matched cohort, FLT3 inhibitors improved overall survival (OS) compared with patients treated without FLT3 inhibitors (3-year OS: 33.7% vs. 25.8%, p = 0.021). Patients who underwent HCT had superior outcomes to those without HCT (3-year OS: 45.3% vs. 2.2%, p < 0.0001). The combination of FLT3 inhibitors and HCT resulted in the highest OS and relapse-free survival (RFS) rates (3-year OS: 62.4%; 3-year RFS: 44.4%). Disease status before transplantation did not predict the prognosis, but use of FLT3 inhibitors increased survival in patients without complete remission before HCT. These results demonstrate the clinical impact of FLT3 inhibitors on survival outcomes in patients with FLT3-ITD-positive AML, particularly when combined with HCT. FLT3 inhibitors can improve the prognosis of AML with FLT3 mutations, especially in patients who undergo HCT.

Medical institutions restricted visitation to neonatal intensive care units (NICUs) during the coronavirus disease (COVID-19) pandemic. Therefore, this study aimed to investigate the impact of COVID-19 NICU visitor restrictions on parents. We conducted a questionnaire of 378 parents of infants who were hospitalised for more than 1 week at two NICUs in our area and discharged between 1 April 2020 and 31 March 2022. While the visiting rules for NICUs during this period varied depending on the phase of the COVID-19 epidemic, generally, only parents were allowed to visit the NICUs for a few hours daily. A total of 157 parents (A University Hospital (AMUH), n = 79; AK General Hospital (AKGH), n = 78) responded to the survey (41.3% response rate), with 40% stating that their emotions towards their infants were affected by restrictions due to the COVID-19 pandemic. Parents' feelings towards their infants were influenced by mode of delivery, parity, duration of hospitalisation and opportunities to visit the NICU. More than 70% of respondents felt stressed due to COVID-19 restrictions, and anxiety and emotional stress related to visitor restrictions were significantly affected by the number and duration of visits and allowability of family member visits. Parents felt that the restrictions had a negative impact on breastfeeding, overall infant care and infant-family bonding. As parents felt an impact on the bond with their infant during visitation restrictions, it is essential for facilities to implement measures to protect infant-family bonding during potential future pandemics.

BackgroundSodium-glucose cotransporter-2 inhibitors (SGLT2i) have been reported to have effects beyond lowering blood glucose levels, with certain SGLT2i expanding their indications to chronic kidney disease and chronic heart failure. We focused on the hepatoprotective and renoprotective effects of six SGLT2i and assessed whether the effects were unique to each drug or common class effects, in addition to whether the renal and hepatoprotective effects vary based on renal and hepatic status.MethodsPatients with diabetes (ipragliflozin: 837, empagliflozin: 850, canagliflozin: 922, dapagliflozin: 590, tofogliflozin: 288, and luseogliflozin: 193) who initiated SGLT2i treatment and were monitored for one year were included. The propensity score (PS) was calculated using patient backgrounds (age, sex, height, weight, body mass index [BMI], disease duration, concomitant diabetes medications, underlying conditions, glycated hemoglobin [HbA1c], estimated glomerular filtration rate [eGFR], aspartate aminotransferase [AST], alanine aminotransferase [ALT], high-density lipoprotein [HDL], low-density lipoprotein [LDL], and triglyceride [TG] levels) as covariates. Additionally, the inverse probability of treatment weighting (IPTW) approach was used to compare liver and renal function test values.ResultsPre- and 12-month post-treatment comparisons demonstrated a significant reduction in hepatic function (AST and ALT) and an increase in renal function (eCcr and eGFR) for all SGLT2i. Comparison of differences between pre- and 12-month post-treatment using the IPTW approach demonstrated no significant differences in AST, ALT, and eGFR levels between SGLT2i. At 12 months post-treatment, 67 patients were classified as having a more severe CKD than those at pre-treatment, representing only 1.8% of all patients (67/3,680). Similarly, 107 patients with AST and 147 patients with ALT were classified as having progressed to a more severe grade than at pre-treatment, representing only 2.9 and 4.0%, respectively.ConclusionsRenoprotective and hepatoprotective effects are class effects of SGLT2i, and their effects are thought to be independent of kidney or liver status.

Unsedated peroral endoscopy, including ultrathin endoscopy (UE) and conventional endoscopy (CE), is feasible in clinical practice but requires improved endoscopic operability and patient tolerance. Currently, the impact of the breathing method on these factors remains unclear. We conducted the first randomized controlled trial comparing oral breathing (OB) and nasal breathing (NB) during both UE and CE to assess their influence. About 252 eligible patients undergoing CE or UE were randomly assigned to OB or NB groups. Endoscopists and patients rated endoscopic operability and patient tolerance using a 100-mm visual analog scale. Visibility from the oral cavity to the middle pharynx was recorded. OB led to a higher rate of improved visibility from the oral cavity to the middle pharynx compared with NB, ranging from 79.3% to 81.0%. Multivariate correlation analyses showed significantly lower visual analog scale scores for endoscopic operability with OB compared with NB in both UE and CE groups ( P < 0.05). No significant differences were found in the overall evaluation of patient tolerance between OB and NB groups in UE and CE, whereas the smaller diameter of UE exhibited better patient tolerance compared with CE. Discriminant analysis comparing endoscope types and breathing methods revealed that UE with OB outperformed other combinations in the overall evaluation of endoscopic operability and patient tolerance ( P < 0.05). OB facilitates endoscopic operability compared with NB in peroral endoscopy. UE with OB is recommended as the preferred choice for unsedated peroral endoscopy in daily practice.

The Japanese Society of Hematology performed an observational cross-sectional study to clarify the morbidity, prognosis, and prognostic factors in patients with COVID-19 with hematological diseases (HDs) in Japan. The study included patients with HDs who enrolled in our epidemiological survey and had a COVID-19 diagnosis and a verified outcome of up to 2 months. The primary endpoints were characteristics and short-term prognosis of COVID-19 in patients with HDs. A total of 367 patients from 68 institutes were enrolled over 1 year, and the collected data were analyzed. The median follow-up among survivors was 73 days (range, 1–639 days). The 60-day overall survival (OS) rate was 86.6%. In the multivariate analysis, albumin ≤ 3.3 g/dL and a need for oxygen were independently associated with inferior 60-day OS rates (hazard ratio [HR] 4.026, 95% confidence interval (CI) 1.954–8.294 and HR 14.55, 95% CI 3.378–62.64, respectively), whereas 60-day survival was significantly greater in patients with benign rather than malignant disease (HR 0.095, 95% CI 0.012–0.750). Together, these data suggest that intensive treatment may be necessary for patients with COVID-19 with malignant HDs who have low albumin levels and require oxygen at the time of diagnosis.