Kyushu University Academic Staff Educational and Research Activities Database
List of Papers
Kaku Noriyuki Last modified date:2021.07.31

Assistant Professor / Emergency & Critical Care Center / Kyushu University Hospital

1. Mari Kurokawa, Kei Nishiyama, Yuhki Koga, Katsuhide Eguchi, Takashi Imai, Utako Oba, Akira Shiraishi, Hazumu Nagata, Noriyuki Kaku, Masataka Ishimura, Satoshi Honjo, Shouichi Ohga, Hyperferritinemia and acute kidney injury in pediatric patients receiving allogeneic hematopoietic cell transplantation, Pediatric Nephrology, 10.1007/s00467-020-04619-y, 35, 10, 1977-1984, 2020.10, Background: Acute kidney injury (AKI) often occurs in pediatric patients who received allogeneic hematopoietic cell transplantation (HCT). We evaluated the risk and effect of HCT-related AKI in pediatric patients. Methods: We retrospectively studied the survival and renal outcome of 69 children 100 days and 1-year posttransplant in our institution in 2004–2016. Stage-3 AKI developed in 34 patients (49%) until 100 days posttransplant. Results: The 100-day overall survival (OS) rates of patients with stage-3 AKI were lower than those without it (76.5% vs. 94.3%, P = 0.035). The 1-year OS rates did not differ markedly between 21 post-100-day survivors with stage-3 AKI and 29 without it (80.8% vs. 87.9%, P = 0.444). The causes of 19 deaths included the relapse of underlying disease or graft failure (n = 11), treatment-related events (4), and second HCT-related events (4). Underlying disease of malignancy (crude hazard ratio (HR) 5.7; 95% confidence interval (CI), 2.20 to 14.96), > 1000 ng/mL ferritinemia (crude HR 4.29; 95% CI, 2.11 to 8.71), stem cell source of peripheral (crude HR 2.96; 95% CI, 1.22 to 7.20) or cord blood (crude HR 2.29; 95% CI, 1.03 to 5.06), and myeloablative regimen (crude HR 2.56; 95% CI, 1.24 to 5.26), were identified as risk factors for stage-3 AKI until 100 days posttransplant. Hyperferritinemia alone was significant (adjusted HR 5.52; 95% CI, 2.21 to 13.76) on multivariable analyses. Conclusions: Hyperferritinemia was associated with stage-3 AKI and early mortality posttransplant. Pretransplant iron control may protect the kidney of pediatric HCT survivors..
2. Kei Nishiyama, Mari Kurokawa, Michiko Torio, Yasunari Sakai, Mitsuru Arima, Shoko Tsukamoto, Satoshi Obata, Shogo Minamikawa, Kandai Nozu, Noriyuki Kaku, Yoshihiko Maehara, Koh Hei Sonoda, Tomoaki Taguchi, Shouichi Ohga, Gastrointestinal symptoms as an extended clinical feature of Pierson syndrome
A case report and review of the literature, BMC Medical Genetics, 10.1186/s12881-020-01019-9, 21, 1, 2020.04, Background: Pierson syndrome (PS) is a rare autosomal recessive disorder, characterized by congenital nephrotic syndrome and microcoria. Advances in renal replacement therapies have extended the lifespan of patients, whereas the full clinical spectrum of PS in infancy and beyond remains elusive. Case presentation: We present the case of a 12-month-old boy with PS, manifesting as the bilateral microcoria and congenital nephrotic syndrome. He was born without asphyxia, and was neurologically intact from birth through the neonatal period. Generalized muscle weakness and hypotonia were recognized from 3 months of age. The infant showed recurrent vomiting at age 5 months of age, and was diagnosed with gastroesophageal reflux and intestinal malrotation. Despite the successful surgical treatment, vomiting persisted and led to severely impaired growth. Tulobuterol treatment was effective in reducing the frequency of vomiting. Targeted sequencing confirmed that he had a compound heterozygous mutation in LAMB2 (NM_002292.3: P.Arg550X and p.Glu1507X). A search of the relevant literature identified 19 patients with severe neuro-muscular phenotypes. Among these, only 8 survived the first 12 months of life, and one had feeding difficulty with similar gastrointestinal problems. Conclusions: This report demonstrated that severe neurological deficits and gastrointestinal dysfunction may emerge in PS patients after the first few months of life..
3. Motoshi Sonoda, Masataka Ishimura, Katsuhide Eguchi, Akira Shiraishi, Shunsuke Kanno, Noriyuki Kaku, Hirosuke Inoue, Yoshitomo Motomura, Masayuki Ochiai, Yasunari Sakai, Manabu Nakayama, Osamu Ohara, Shouichi Ohga, Prognostic factors for survival of herpes simplex virus-associated hemophagocytic lymphohistiocytosis, International journal of hematology, 10.1007/s12185-019-02738-3, 111, 1, 131-136, 2020.01, Hemophagocytic lymphohistiocytosis (HLH) occurs in neonates with disseminated infection of herpes simplex virus (HSV). Little has been reported on the control of rapid HLH progression. We studied the cytokine profile and genetic basis of two index cases with divergent outcomes after early treatment of type 2 HSV infection. One survivor had fever and elevated serum levels of tumor necrosis factor (TNF)-α, interleukin-6 (IL-6), interferon (IFN)-β, and IFN-γ at diagnosis. The other neonate had no fever or TNF-α production, but significant IL-6 or IFN responses during the treatment course, and died 19 days after birth. Among 16 reported cases of neonatal HSV-HLH including index cases, eight deceased neonates experienced significantly less fever at presentation (p = 0.028), lower platelet counts (p = 0.019), and lower ratios of soluble IL-2 receptor (sIL-2R) to ferritin levels (p = 0.044) than eight survivors. The 100-day overall survival rates were significantly higher in patients with fever (p = 0.004), > 100 × 109/L of platelet counts (p = 0.035) or > 20 of sIL-2R/ferritin ratio at diagnosis (p = 0.004). The first febrile and cytokine responses to HSV infection predict the early outcome of neonatal HSV-HLH..
4. Koichiro Yoshimaru, Toshiharu Matsuura, Yusuke Yanagi, Yoshiaki Takahashi, Kenichi Kohashi, Noriyuki Kaku, Yoshinao Oda, Shouichi Ohga, Tomoaki Taguchi, Successful Urgent Living Donor Liver Transplantation for Massive Liver Necrosis Accompanied by Nonocclusive Mesenteric Ischemia in a Biliary Atresia Infant
A Case Report, Transplantation Proceedings, 10.1016/j.transproceed.2020.06.010, 2020, Background: Treatment options for patients presenting with life-threatening splanchnic ischemia, including that of the intestine and liver, could previously only receive salvage surgery and attempted medical revascularization. We propose that urgent liver transplantation (LT) for acute liver failure (ALF) due to massive liver necrosis should be considered as a life-saving treatment. We successfully performed urgent living donor LT for nonocclusive hepatic ischemia accompanied by nonocclusive mesenteric ischemia (NOMI). Case: An 11-month-old boy with biliary atresia whose jaundice was re-elevated after Kasai portoenterostomy underwent cysto-jejunostomy. Three hours after the uneventful operation, tachycardia, hypotension, and unconsciousness suddenly occurred. Computed tomography revealed whole-liver and massive splenic and focal intestinal ischemia without any vessel occlusion. Urgent LT was performed on postoperative day 3 because intensive therapies, including prostaglandin E1 administration, blood transfusion, and continuous hemodiafiltration, were not effective and the patient had developed life-threatening bradycardia and hypotension. Intraoperative findings included whole-liver necrosis and splenic ischemia and segmental ileal necrosis without any vessel thrombus. LT and necrotic intestinal resections by end-to-end anastomosis were performed. Massive liver necrosis with Gram-positive cocci was histopathologically identified, indicating bacterial translocation due to NOMI. The post-LT course was uneventful, and the neurologic outcomes were uncomplicated. Conclusions: Urgent LT was successfully completed for ALF with NOMI. LT is the sole treatment for the refractory ALF, and undelayed determination is important to rescue..
5. Koichiro Yoshimaru, Toshiharu Matsuura, Yasuyuki Uchida, Keisuke Kajihara, Yukihiro Toriigahara, Yuki Kawano, Takuya Kondo, Yoshiaki Takahashi, Wakato Matsuoka, Noriyuki Kaku, Jun Maki, Tomoaki Taguchi, Ultrasound-guided double central venous access for azygos vein via the ninth and tenth intercostal veins, Journal of Vascular Access, 10.1177/1129729820937133, 2020, Some patients with intestinal failure, who are dependent on total parenteral nutrition for long periods, suffer from a lack of suitable conventional venous access points, including axillary, external jugular, internal jugular, subclavian, saphenous, and the brachio-cephalic and femoral veins, due to their occlusion. Furthermore, extensive central venous stenosis and/or thrombosis of the superior and inferior vena cava may preclude further catheterization, so uncommon routes must be used, which can be challenging. In such patients, the azygos vein via the intercostal vein is a viable candidate. Thoracotomy-assisted, thoracoscopy-assisted, and cut-down procedures are currently suggested such access. We found that ultrasound-guided percutaneous puncture method was a safe and minimally invasive approach and successfully placed two central venous lines in preparation for small bowel transplantation via two different intercostal veins (ninth and tenth). Although the lung was actually located just below the target veins, an ultrasound provided augmented and clear vision, which contributed to the safe performance of the procedure without the need for invasive surgical intervention, such as thoracotomy, thoracoscopy, or rib resection using the cut-down technique. Furthermore, constant positive-pressure ventilation during vein puncture under general anesthesia also helps avoid venous collapse. Despite carrying a slight risk of light injury to the lung, artery, and nerve along with the vein compared to other procedures, we believe that ultrasound-guided puncture under general anesthesia is feasible as a minimally invasive method..
6. Soichi Mizuguchi, Yoshitomo Motomura, Jun Maki, Rieko Baba, Yuko Ichimiya, Kentaro Tokuda, Noriyuki Kaku, Hidetoshi Takada, Yoshihiko Maehara, Shouichi Ohga, Tracheal Size and Morphology on the Reconstructed CT Imaging, Pediatric Critical Care Medicine, 10.1097/PCC.0000000000001996, 20, 8, E366-E371, 2019.08, Objectives: To characterize the real size and morphology of tracheas in childhood for the optimal selection of endotracheal tube. Design: A retrospective cohort study of pediatric patients who received CT scan of the cervical spine from July 2011 to March 2018. Cross-sectional CT images vertical to trachea were reconstructed and the accurate tracheal diameters were measured. The validity of the traditional age-based formula for predicting the endotracheal tube size was assessed for the best fit to trachea. Setting: Tertiary Emergency and Critical Care Center of Kyushu University Hospital. Patients: Children, who are 1 month to 15 years old, received CT scan of the cervical spine. Interventions: None. Measurements and Main Results: We enrolled 86 children with median age of 53 months. The cross-sectional shape of pediatric trachea was circular at the cricoid level and elliptical at the infraglottic level. The narrowest part of pediatric trachea was the transverse diameter at the infraglottic level at any age. Significant positive correlation between age and the narrowest diameter was observed. When compared the transverse diameter at the infraglottic level with the outer diameter of endotracheal tubes, uncuffed endotracheal tubes selection based on the traditional age-based formula ran a significant risk of oversized endotracheal intubation until 10 years old compared with cuffed endotracheal tubes selection (60.0% vs 23.8%; p < 0.05). Conclusions: These findings indicate the safety and efficacy of cuffed endotracheal tubes in infants and children and the reconsideration for the airway management in pediatric anesthesia and intensive care..
7. Ryuichi Takemoto, Yoshitomo Motomura, Noriyuki Kaku, Yuko Ichimiya, Mamoru Muraoka, Shunsuke Kanno, Tamami Tanaka, Yasunari Sakai, Yoshihiko Maehara, Shouichi Ohga, Late-onset sepsis and encephalopathy after bicycle-spoke injury
A case report, BMC Infectious Diseases, 10.1186/s12879-019-4082-4, 19, 1, 2019.05, Background: Bicycle-spoke injuries rarely cause late complications of infection, including sepsis and sepsis-associated encephalopathy, with appropriate treatments. Case presentation: We experienced a 2-year-old girl who developed the signs of encephalopathy with fever 6 months after a spoke-injury. On admission, the injured skin was inflamed with cellulitis. The blood culture was positive for methicillin-sensitive Staphylococcus aureus. Electroencephalogram showed diffuse slow-wave activity. Diffusion-weighted magnetic resonance imaging detected a high-intensity lesion with decreased diffusivity at the right frontal cortex. She received immunoglobulin and combined antibiotics treatments in the intensive care unit, and successfully overcame the sepsis-associated encephalopathy without neurological impairments. Conclusion: This is the first report demonstrating that sepsis and its associated encephalopathy occurs in a remote period after the bicycle-spoke injury..
8. Sayaka Okuzono, Ryoko Fukai, Marie Noda, Noriko Miyake, Sooyoung Lee, Noriyuki Kaku, Masafumi Sanefuji, Satoshi Akamine, Shunsuke Kanno, Yoshito Ishizaki, Hiroyuki Torisu, Ryutaro Kira, Naomichi Matsumoto, Yasunari Sakai, Shouichi Ohga, An acute encephalopathy with reduced diffusion in BRAF-associated cardio-facio-cutaneous syndrome, Brain and Development, 10.1016/j.braindev.2018.10.012, 41, 4, 378-381, 2019.04, Background: Cardio-facio-cutaneous syndrome (CFCS) is a rare genetic disorder characterized by cardiovascular anomalies, dysmorphic faces, ectodermal abnormalities and developmental delays. Mutations in BRAF and other RAS-MAPK pathway-associated genes are commonly identified in patients with CFCS. While this molecular pathway is known to be associated with neuro-inflammatory conditions, only one case with CFCS has been reported thus far to develop acute encephalopathy in childhood. Case report: A 3-year-old boy with dysmorphic features and mild psychomotor delay developed acute encephalopathy. After a 45-min long, generalized seizure, the magnetic resonance imaging revealed that the restricted diffusion signals spread to the bilateral subcortical white matters on day 1 of illness. Despite the 14 days of intensive care, the acute symptoms of encephalopathy left him intractable epilepsy and severe neurocognitive impairments. The whole-exome sequencing analysis identified a de novo heterozygous mutation of BRAF (NM_004333:p.Thr241Met) in this case. Conclusion: The present case suggests that the hyperactive condition of ERK signals might augment the development of acute encephalopathy and post-encephalopathic epilepsy in childhood..
9. Kousuke Yonemoto, Yuko Ichimiya, Masafumi Sanefuji, Noriyuki Kaku, Ayumi Sakata, Rieko Baba, Fumiya Yamashita, Satoshi Akamine, Michiko Torio, Yoshito Ishizaki, Yoshihiko Maehara, Yasunari Sakai, Shouichi Ohga, Early Intervention With Adrenocorticotropin for Acute Encephalopathy-Associated Epileptic Spasms
Report of Two Cases, Clinical EEG and Neuroscience, 10.1177/1550059418786381, 50, 1, 51-55, 2019.01, Purpose. Acute encephalopathy with biphasic seizures and reduced diffusion (AESD) is a leading cause of childhood-onset encephalopathy in Japan. Children with AESD frequently develop intractable epilepsy, whereas their treatment options remain to be determined. Method. We present 2 unrelated girls, who developed AESD at 25 months (case 1) and 12 months of age (case 2). Both cases underwent intensive cares from the first day of illness, whereas severe neurological impairments were left on discharge. They showed repeated signs of epileptic spasms at 2 months (case 1) and 8 months (case 2) after the onset of AESD. Video-monitoring electroencephalograms (EEG) detected the recurrent attacks accompanying slow-wave bursts and transient suppressions of the precedent epileptiform discharges, as typically observed in epileptic spasms. Results. Intramuscular injection of adrenocorticotropic hormone (ACTH, 0.0125 mg/kg/d) was introduced within 1 month from the onset of epileptic spasms and continued for 2 weeks. The ACTH treatment disrupted the paroxysmal activity in EEG, and it has relieved these patients from epileptic seizures for more than 1 year. Conclusion. This report illustrates the potential efficacy of ACTH for a group of children with epileptic spasms after AESD..
10. Masafumi Sanefuji, Yuko Ichimiya, Noriyuki Kaku, Momoko Sasazuki, Kosuke Yonemoto, Michiko Torio, Soichi Mizuguchi, Yoshitomo Motomura, Mamoru Muraoka, Sooyoung Lee, Haruhisa Baba, Kazuhiro Ohkubo, Yuri Sonoda, Yoshito Ishizaki, Yasunari Sakai, Shouichi Ohga, Vascular pathomechanism in acute encephalopathy with biphasic seizures and late reduced diffusion, Journal of the Neurological Sciences, 10.1016/j.jns.2018.10.007, 395, 141-146, 2018.12, Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a childhood-onset encephalopathy, but the precise pathophysiology remains unclear. We encountered a child with Moyamoya syndrome and AESD. He exhibited left-predominant stenosis of the middle cerebral artery (MCA), and later developed broad lesions in the left hemisphere, raising the possibility that insufficient blood supply relates to formation of the lesions. To test the hypothesis, we investigated the relationship between MCA volume and lesion extent in seven AESD children without preexisting diseases. The MCA volume and lesion extent were quantified with time of flight images for construction of magnetic resonance angiography and apparent diffusion coefficient maps, respectively. Lateralization indices ([right − left]/[right + left]) of the MCA volume and lesion extent were calculated. We found that the lateralization indices were negatively correlated (r = −0.786, p =.036), that is, when the MCA volume was smaller in one side than the other side, the lesions were likely to develop more extensively in the ipsilateral side than the contralateral side. This indicates the association of insufficient blood supply with the lesions. The present study provides the first observation to suggest the involvement of vascular mechanism in AESD and has potential implications for novel therapeutic approach..
11. Noriyuki Kaku, Kenji Ihara, Yuichiro Hirata, Kenji Yamada, Sooyoung Lee, Hikaru Kanemasa, Yoshitomo Motomura, Haruhisa Baba, Tamami Tanaka, Yasunari Sakai, Yoshihiko Maehara, Shouichi Ohga, Diagnostic potential of stored dried blood spots for inborn errors of metabolism
A metabolic autopsy of medium-chain acyl-CoA dehydrogenase deficiency, Journal of Clinical Pathology, 10.1136/jclinpath-2017-204962, 71, 10, 885-889, 2018.10, Aim It is estimated that 1-5% of sudden infant death syndrome (SIDS) cases might be caused by undiagnosed inborn errors of metabolism (IEMs); however, the postmortem identification of IEMs remains difficult. This study aimed to evaluate the usefulness of dried blood spots (DBSs) stored after newborn screening tests as a metabolic autopsy to determine the causes of death in infants and children who died suddenly and unexpectedly. Methods Infants or toddlers who had suddenly died without a definite diagnosis between July 2008 and December 2012 at Kyushu University Hospital in Japan were enrolled in this study. Their Guthrie cards, which had been stored for several years at 4-8°C, were used for an acylcarnitine analysis by tandem mass spectrometry to identify inborn errors of metabolism. Results Fifteen infants and children who died at less than 2 years of age and for whom the cause of death was unknown were enrolled for the study. After correcting the C0 and C8 values assuming the hydrolysation of acylcarnitine in the stored DBSs, the corrected C8 value of one case just exceeded the cut-off level for medium-chain acyl-CoA dehydrogenase (MCAD) deficiency screening. Genetic and biochemical analyses confirmed this patient to have MCAD deficiency. Conclusion DBSs stored after newborn screening tests are a promising tool for metabolic autopsy. The appropriate compensation of acylcarnitine data and subsequent genetic and biochemical analyses are essential for the postmortem diagnosis of inborn errors of metabolism..
12. Yuko Ichimiya, Noriyuki Kaku, Masafumi Sanefuji, Michiko Torio, Soichi Mizuguchi, Yoshitomo Motomura, Mamoru Muraoka, Sooyoung Lee, Haruhisa Baba, Yuri Sonoda, Yoshito Ishizaki, Momoko Sasazuki, Yasunari Sakai, Yoshihiko Maehara, Shouichi Ohga, Predictive indicators for the development of epilepsy after acute encephalopathy with biphasic seizures and late reduced diffusion, Epilepsy Research, 10.1016/j.eplepsyres.2018.04.006, 143, 70-74, 2018.07, Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a newly defined clinicoradiologic syndrome characterized by biphasic seizures and altered consciousness followed by restricted diffusion in the white matter on magnetic resonance imaging in acute phase. Intractable epilepsy commonly occurs as the late complication. This study aimed to search predisposing factors to the development of epilepsy after AESD. Consecutively treated 22 patients with AESD in our institution from 2006 to 2016 were grouped into those with post-encephalopathic epilepsy (PEE, n = 10) or without PEE (n = 12). There was no difference between two groups in age at the onset of AESD, duration of the initial seizures, or the follow-up periods after discharge. PEE group patients more frequently showed coma or involuntary movements during the course of AESD than non-PEE group patients (36% vs. 8%, p = 0.008). The quantitative analysis of apparent diffusion coefficient (ADC) map revealed that PEE group showed broader areas with reduced diffusion in the posterior lobes at the onsets of AESD than non-PEE group (0.113 vs. 0.013, p = 0.035). On the other hand, the atrophy on day 30-ADC map did not correlate with the development or control of epilepsy. These results suggest that the clinical severity and ADC profiles in acute phase, rather than the brain atrophy in convalescent phase, may predict the development of post-AESD epilepsy..
13. Sayaka Okuzono, Masataka Ishimura, Shunsuke Kanno, Motoshi Sonoda, Noriyuki Kaku, Yoshitomo Motomura, Hisanori Nishio, Utako Oba, Masuo Hanada, Jun ichi Fukushi, Michiyo Urata, Dongchon Kang, Hidetoshi Takada, Shouichi Ohga, Streptococcus pyogenes-purpura fulminans as an invasive form of group A streptococcal infection, Annals of Clinical Microbiology and Antimicrobials, 10.1186/s12941-018-0282-9, 17, 1, 2018.07, Background: Streptococcus pyogenes is an uncommon pathogen of purpura fulminans, and the pathogenesis of S. pyogenes-purpura fulminans remains unclear because of paucity of cases. We reported a pediatric case of S. pyogenes-purpura fulminans with literature review of the disease. Case presentation: A 3-year-old boy showed limping, lethargy and acral gangrene within 24 h. A diagnosis of S. pyogenes-purpura fulminans was made for bacterial isolation from throat and peripheral blood. Intensive therapy led to a survival with amputation of the left distal metatarsal bone, and normal development. The isolated M12 carried no mutation of csrS/R or rgg. Thrombophilia or immunodeficiency was excluded. Discussion: Twelve-reported cases (9 pediatric and 3 elderly) of S. pyogenes-purpura fulminans started with shock and coagulopathy. Five patients age < 8 years had no underlying disease and survived. One youngest and two immunocompromised patients died. Conclusion:Streptococcus pyogenes-acute infectious purpura fulminans is a distinctive rare form of aggressive GAS infections..
14. Noriyuki Kaku, Masahiko Nitta, Takashi Muguruma, Yuichiro Hirata, Kohei Tsukahara, Emily Knaup, Nobuyuki Nosaka, Yuki Enomoto, Medical equipment deployment in pediatric emergency prehospital medical units in Japan, Pediatrics International, 10.1111/ped.13432, 60, 1, 93-95, 2018.01, The deployment status of pediatric emergency equipment in ambulances in Japan is unknown. To investigate the status of and issues associated with prehospital emergency medical care for pediatric patients, we conducted a descriptive epidemiological study. We carried out a Web-based survey of 767 fire defense headquarters in Japan, of which 671 responded (valid response rate, 88%). Most of the fire defense headquarters equipped all of their ambulances with oxygen masks (82%), bag-valve masks (for neonates, 83%; for children, 84%), straight laryngoscope blades (for neonates, 47%; for children 68%), blood pressure cuffs for children (91%), oximeter probes (78%), and stiff neck collars (91%); but despite the need for other equipment such as nasopharyngeal and oropharyngeal airways, and Magill forceps, they were insufficiently deployed. In Japan, prehospital emergency medical equipment deployment does not meet the needs of pediatric patients. Minimum equipment standards need to be established for pediatric prehospital care..
15. Yuji Shono, Tomohiko Akahoshi, Satomi Mezuki, Kenta Momii, Noriyuki Kaku, Jun Maki, Kentaro Tokuda, Tetsuro Ago, Takanari Kitazono, Yoshihiko Maehara, Clinical characteristics of type A acute aortic dissection with CNS symptom, American Journal of Emergency Medicine, 10.1016/j.ajem.2017.06.011, 35, 12, 1836-1838, 2017.12, Background and purpose Accurate diagnosis of acute aortic dissection (AAD) is sometimes difficult because of accompanying central nervous system (CNS) symptoms. The purpose of this study was to investigate the clinical characteristics of Type A AAD (TAAAD) with CNS symptoms. Methods We retrospectively reviewed the medical records of 8403 patients ambulanced to our emergency and critical care center between April 2009 and May 2014. Results We identified 59 TAAAD patients for the analysis (mean age, 67.3 ± 10.5 years; 37 (62.0%) male). Eleven patients (18.6%) presented CNS symptoms at the onset of TAAAD, and these patients complained less frequently of typical chest and back pain than those without CNS symptoms (p < 0.0001). Initial systolic and diastolic blood pressure were lower (p = 0.003, and p = 0.049, respectively) and involvement of the supra-aortic artery was more frequent in patients with CNS symptoms (p < 0.0001). Conclusion Because CNS symptom can mask chest and back pain caused by TAAAD, physicians should always consider the possibility of TAAAD in patients with CNS symptoms in emergency medicine settings..
16. Yuko Ichimiya, Noriyuki Kaku, Yasunari Sakai, Fumiya Yamashita, Wakato Matsuoka, Mamoru Muraoka, Satoshi Akamine, Soichi Mizuguchi, Michiko Torio, Yoshitomo Motomura, Yuichiro Hirata, Yoshito Ishizaki, Masafumi Sanefuji, Hiroyuki Torisu, Hidetoshi Takada, Yoshihiko Maehara, Shouichi Ohga, Transient dysautonomia in an acute phase of encephalopathy with biphasic seizures and late reduced diffusion, Brain and Development, 10.1016/j.braindev.2017.03.023, 39, 7, 621-624, 2017.08, Paroxysmal sympathetic hyperactivity (PSH) is a dysautonomic condition that is associated with various types of acquired brain injuries. Traumatic brain lesions have been documented as the leading cause of PSH. However, detailed clinical features of pediatric PSH caused by intrinsic brain lesions remain to be elusive. We present a 3-year-old boy, who had been diagnosed as having cerebral palsy, developmental delay and epilepsy after perinatal hypoxia-induced brain injury. He developed status epilepticus with fever on the third day of respiratory infection. Whereas the seizure was terminated by systemic infusion of midazolam, consciousness remained disturbed for the next 48 h. Serial magnetic resonance imaging studies revealed that acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) evolved on 3 days after the seizure. Therapeutic hypothermia was immediately introduced, however, the brain lesion extended to the whole subcortical white matters on day 8. The intermittent bilateral dilation of pupils with increased blood pressure and tachycardia were observed until day 12. Real-time monitoring of electroencephalograms ruled out the recurrent attacks of seizures. The abnormal signs of autonomic nervous system gradually ceased and never relapsed after recovery from the hypothermia. PSH or a transient condition of dysautonomia may emerge and persist during the acute phase of AESD..
17. Sooyoung Lee, Masafumi Sanefuji, Michiko Torio, Noriyuki Kaku, Yuko Ichimiya, Soichi Mizuguchi, Haruhisa Baba, Yasunari Sakai, Yoshito Ishizaki, Hiroyuki Torisu, Ryutaro Kira, Toshiro Hara, Shouichi Ohga, Involuntary movements and coma as the prognostic marker for acute encephalopathy with biphasic seizures and late reduced diffusion, Journal of the Neurological Sciences, 10.1016/j.jns.2016.09.018, 370, 39-43, 2016.11, Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) occurs in children associated with infection. It is characterized by a prolonged febrile seizure in the first phase, and a cluster of seizures, deterioration of consciousness and the white matter lesions with reduced diffusion in the second phase. The patients often have severe neurological sequelae, but the prognostic indicators remain unknown. The present study aimed to clarify the characteristics of AESD patients who subsequently exhibited severe neurological sequelae. We retrospectively analyzed the clinical and laboratory findings along with the brain imaging in patients who had severe (n = 8) and non-severe neurodevelopmental outcomes (n = 12). Severe group more frequently showed coma (p = 0.014) or involuntary movements including dystonia and oral dyskinesia (p = 0.018) before the second phase than non-severe group. Severe group exhibited higher levels of serum alanine aminotransferase than non-severe group (p = 0.001). Quantitatively assessed MRI in the second phase revealed that severe group had more extensive lesions than non-severe group, in the anterior (p = 0.015) and posterior parts (p = 0.011) of the cerebrum and basal ganglia (p = 0.020). Early appearing involuntary movements or coma might account for the extension of acute brain lesions and the poor neurological outcomes in AESD patients..
18. Etsuro Nanishi, Yuichirou Hirata, Sooyoung Lee, Noriyuki Kaku, Kenta Momii, Kensuke Kubota, Hisanori Nishio, Yoshihiko Maehara, Toshiro Hara, Polymyxin-B immobilized column–direct hemoperfusion for adolescent toxic shock syndrome, Pediatrics International, 10.1111/ped.13087, 58, 10, 1051-1054, 2016.10, Toxic shock syndrome (TSS) is a critical illness associated with toxin from Staphylococcus aureus. Despite recent advances in critical care, mortality remains high and additional effective therapy is required. We report an adolescent case of TSS successfully treated with direct hemoperfusion using polymyxin-B immobilized fiber (PMX-DHP). The patient with spina bifida also had ischial pressure ulcer, and developed TSS associated with methicillin-resistant S. aureus. Despite conventional treatment, the patient developed refractory shock, which was immediately improved with PMX-DHP. PMX-DHP has been widely used for the treatment of sepsis to remove circulating endotoxins produced by Gram-negative bacteria, but beneficial effects have also been reported for Gram-positive bacterial infection. To our knowledge, this is the first report on PMX-DHP for TSS in an adolescent patient, and we propose that PMX-DHP could be a new treatment strategy for severe TSS in children as well..
19. Akahoshi Tomohiko, Yasuda Mitsuhiro, Momii Kenta, Kubota Kensuke, Shouno Yuji, Kaku Noriyuki, Kentaro Tokuda, Nagata Takashi, Yoshizumi Tomoharu, Shirabe Ken, Hashizume Makoto, Maehara Yoshihiko, Sarcopenia is a predictive factor for prolonged intensive care unit stays in high-energy blunt trauma patients, Acute medicine & Surgery, 3, 326-331, 2016.05.
20. Naoko Toda, Takayuki Hoshina, Yuhki Koga, Masayuki Ochiai, Noriyuki Kaku, Kenichiro Yamamura, Hiroyuki Torisu, Kenji Ihara, Hidetoshi Takada, Yoshihiko Maehara, Toshiro Hara, Analysis of death due to infectious diseases in patients hospitalized in the pediatric ward of a single Japanese tertiary medical facility, Japanese Journal of Infectious Diseases, 10.7883/yoken.JJID.2015.591, 69, 6, 464-470, 2016, SUMMARY: In developed countries,the infection related-mortality rates in children hospitalized in tertiary medical facilities,where many patients with underlying disease are also hospitalized,are uncertain. We investigated the characteristics of infectious diseases-related fatal cases in the pediatric ward of a Japanese tertiary medical facility. A total of 188 patients who died in the pediatric ward or intensive care unit at Kyushu University Hospital from 2002 to 2011 were enrolled. The patient characteristics were investigated based on their medical records. A total of 35 patients died of infections,31 of whom had underlying diseases. Most patients died of sepsis or pneumonia (n = 27). All 9 patients who died within 7 days of birth were premature. Nine of the 13 patients with malignancy or hematological disorders died of hematopoietic stem cell transplantation (HSCT)-associated infections. The ratio of infectious disease-related fatal cases to the total cases decreased in the latter half of the study period. In particular,the proportion of preterm infants who died of infections was significantly lower in the latter 5 years (p = 0.02). Many of the infectious disease-related fatal cases were in premature infants and HSCT or post-HSCT patients. However,the mortality due to infectious diseases decreased in these patient groups..
21. T. Akahoshi, H. Sugimori, N. Kaku, K. Tokuda, T. Nagata, E. Noda, M. Morita, M. Hashizume, Y. Maehara, Comparison of recombinant human thrombomodulin and gabexate mesylate for treatment of disseminated intravascular coagulation (DIC) with sepsis following emergent gastrointestinal surgery
a retrospective study, European Journal of Trauma and Emergency Surgery, 10.1007/s00068-014-0478-4, 41, 5, 531-538, 2015.10, Purpose: Recombinant thrombomodulin (rTM) has been available in Japan since 2008, but there is concern about its association with postoperative hemorrhage. The efficacy and safety of rTM were examined in patients with disseminated intravascular coagulation (DIC) caused by a septic condition after gastrointestinal surgery. Methods: Forty-two patients were emergently admitted to the intensive care unit after emergent gastrointestinal surgery in Kyushu University Hospital from May 2008 to April 2013. Of these patients, 22 had DIC (defined as an acute DIC score ≥4). All but three patients received treatment with gabexate mesylate (GM) (n = 9) or rTM (n = 10). The causes of sepsis were peritonitis with colorectal perforation, anastomotic leakage, and intestinal necrosis. Acute DIC score, sepsis-related organ failure assessment score, platelet count, and a variety of biochemical parameters were compared between rTM and GM recipients after treatment administration. Results: There were no significant differences between the groups for any parameter except C-reactive protein levels. The CRP level tended to be lower in the rTM group than in the GM group. Acute DIC score in the rTM group resolved significantly earlier than that in the GM group. No patient stopped the administration of rTM because of postoperative bleeding. Conclusion: rTM may be an effective therapeutic drug for the treatment of septic patients with DIC following emergent gastrointestinal surgery..
22. Takuya Hara, Shouichi Ohga, Sagano Hattori, Miho Hatano, Noriyuki Kaku, Akihiko Nomura, Hidetoshi Takada, Hisashi Kokuba, Koichi Ohshima, Toshiro Hara, Prolonged severe pancytopenia preceding the cutaneous lesions of juvenile xanthogranuloma, Pediatric Blood and Cancer, 10.1002/pbc.20514, 47, 1, 103-106, 2006.07, We report a case of juvenile xanthogranuloma (JXG) having progressive pancytopenia for 6 months until the proliferating skin lesions. A 2-month-old infant presented recurrent fever, anemia, and hepatosplenomegaly mimicking hemophagocytic lymphohistiocytosis (HLH) or juvenile myelomonocytic leukemia (JMML). At 8 months of age, the biopsy of a growing papule on the elbow made the diagnosis. Bone marrow (BM) specimens showed clustering foamy cells including hemophagocytosis by histiocytes. Treatment with etoposide followed by vinblastine plus prednisolone (PSL) therapy improved the disease. Although JXC is a benign non-Langerhans cell histiocytosis, the multisystem-visceral form should be considered as a potential aggressive disease when associated with BM failure in early infancy..