Publications by authors named "Yoshifumi Kawano"

164 Publications

Early diagnosis of infantile Danon disease complicated by tetralogy of Fallot.

Pediatr Int 2021 Jun 4. Epub 2021 Jun 4.

Department of Pediatrics, Kagoshima University, Kagoshima, Japan.

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http://dx.doi.org/10.1111/ped.14542DOI Listing
June 2021

Prednisolone Suppresses the Extracellular Release of HMGB-1 and Associated Inflammatory Pathways in Kawasaki Disease.

Front Immunol 2021 17;12:640315. Epub 2021 May 17.

Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.

Innate immune activity plays an essential role in the development of Kawasaki disease (KD) vasculitis. Extracellular release of high mobility group box-1 (HMGB-1), an endogenous damage-associated molecular pattern protein that can activate the innate immune system and drive host inflammatory responses, may contribute to the development of coronary artery abnormalities in KD. Prednisolone (PSL) added to intravenous immunoglobulin treatment for acute KD may reduce such abnormalities. Here, we evaluate the dynamics of HMGB-1 and therapeutic effects of PSL on HMGB-1-mediated inflammatory pathways on KD vasculitis . Serum samples were collected prior to initial treatment from patients with KD, systemic juvenile idiopathic arthritis (sJIA), and from healthy controls (VH), then incubated with human coronary artery endothelial cells (HCAECs). Following treatment of KD serum-activated HCAECs with PSL or PBS as a control, effects on the HMGB-1 signaling pathway were evaluated. Compared to that from VH and sJIA, KD serum activation induced HCAEC cytotoxicity and triggered extracellular release of HMGB-1. KD serum-activated HCAECs up-regulated extracellular signal-regulated kinase (ERK)1/2, c-Jun N-terminal kinase (JNK) and, p38 phosphorylation in the cytoplasm and nuclear factor kappa B (NF-κB) phosphorylation in the nucleus and increased interleukin (IL)-1β and tumor necrosis factor (TNF)-α production. PSL treatment of KD serum-activated HCAECs inhibited extracellular release of HMGB-1, down-regulated ERK1/2, JNK, p38, and NF-κB signaling pathways, and decreased IL-1β and TNF-α production. Our findings suggest that extracellular HMGB-1 plays an important role in mediating KD pathogenesis and that PSL treatment during the acute phase of KD may ameliorate HMGB-1-mediated inflammatory responses in KD vasculitis.
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http://dx.doi.org/10.3389/fimmu.2021.640315DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8165186PMC
May 2021

[Improvement in platelet count and bleeding symptom during treatment with eltrombopag in a patient with X-linked thrombocytopenia].

Rinsho Ketsueki 2021 ;62(4):257-261

Department of Pediatrics, Graduate School of Medical and Dental Sciences, Kagoshima University.

Herein, we describe a 13-year-old male adolescent who had chronic thrombocytopenia since infancy. In this case, X-linked thrombocytopenia (XLT) was suspected owing to a family history of chronic thrombocytopenia and small-sized platelets. Moreover, the patient was refractory to immunoglobulin therapy. The Wiskott-Aldrich syndrome protein (WASP) expression analysis revealed a decreased expression. Results showed a missense mutation [c.296A>G (p.Gln99Arg)] in exon 3 of the WASP-interacting protein region. Therefore, a diagnosis of XLT was made. To lift exercise restrictions, we initiated treatment with eltrombopag at a dose of 12.5 µg/day. The platelet count of the patient increased to approximately 50×10/µl after the treatment dose was escalated to 25 µg/day, and bleeding symptoms decreased after the patient resumed exercise. Ultrastructural platelet abnormalities and abnormal platelet aggregation were observed on transmission electron microscopy after the administration of eltrombopag. Therefore, eltrombopag treatment can increase platelet count and reduce bleeding symptoms in patients with XLT.
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http://dx.doi.org/10.11406/rinketsu.62.257DOI Listing
May 2021

Case Report: F-FDG PET-CT for Diagnosing Prosthetic Device-Related Infection in an Infant With CHD.

Front Pediatr 2021 8;9:584741. Epub 2021 Mar 8.

Department of Pediatrics, Kagoshima University, Kagoshima, Japan.

Patients who have undergone cardiac surgery using prosthetic devices have an increased risk of developing prosthetic device-related infection and mediastinitis. However, accurate diagnosis of prosthetic device-related infection can be difficult to evaluate and treat with antibiotic therapy alone. In recent years, F-fluorodeoxyglucose positron emission tomography-computed tomography (F-FDG PET-CT) has made promising contributions to detect infective endocarditis, pacemaker infections, or other inflammations. Nevertheless, F-FDG PET-CT for congenital heart disease (CHD) with device infection has been sparsely reported. We present an infantile girl diagnosed with pulmonary atresia with a ventricular septal defect who underwent replacement of the right ventricle-to-pulmonary artery (RV-PA) conduit for improvement cyanosis. She developed high fever and was diagnosed with mediastinitis and bacteremia by on postoperative day 4. Mediastinal drainage and 6 weeks of antibiotic therapy improved her condition, but bacteremia flared up on postoperative day 56. Despite a long course of antibiotic therapy, she had two more recurrences of bacteremia with the detection of . Echocardiography and chest contrast CT showed no evidence of vegetation and mediastinitis. On postoperative day 115, F-FDG PET-CT revealed an accumulation on the RV-PA conduit (SUV max 3.4). Finally, she developed an infectious ventricular pseudo-aneurysm on postoperative day 129 and underwent aneurysm removal and RV-PA conduit replacement on postoperative day 136. Our case showed the importance of F-FDG PET-CT for diagnosing specific localization of prosthetic device-related infection which is hard to detect using other imaging techniques. It can be a useful diagnostic tool for infantile patients with CHD with cardiac prosthetic devices and improve subsequent clinical treatments.
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http://dx.doi.org/10.3389/fped.2021.584741DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7982821PMC
March 2021

Successful treatment of post chemotherapy esophageal cicatricial atresia in a pediatric patient with anaplastic large cell lymphoma through minimally invasive esophagectomy: a case report.

Surg Case Rep 2021 Feb 5;7(1):41. Epub 2021 Feb 5.

Department of Digestive Surgery, Breast and Thyroid Surgery, Graduate School of Medical and Dental Sciences, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima-shi, Kagoshima, 890-8520, Japan.

Background: Anaplastic large cell lymphoma (ALCL) is a CD30-positive T-cell lymphoma, which is a rare type of non-Hodgkin lymphoma. ALCL rarely presents in the gastrointestinal tract, and the esophageal involvement in of ALCL is extremely rare.

Case Presentation: An 11-year-old boy who complained of abdominal pain and cough was diagnosed with ALK-positive ALCL on the basis of systemic lymphadenopathy findings and immunohistochemistry results of pleural effusion. Although remission was observed after chemotherapy at 5 months after diagnosis, dysphagia persisted, and esophagoscopy revealed a severe stricture in the middle thoracic esophagus. At 9 months after diagnosis, allogeneic bone marrow transplantation was performed to ensure that complete remission was maintained; however, dysphagia and saliva retention did not improve. Approximately 10 months after diagnosis, esophagoscopy revealed a blind end in the middle thoracic esophagus, similar to that in congenital esophageal atresia. Subsequently, we performed minimally invasive subtotal esophagectomy under thoracoscopy and laparoscopy and gastric conduit reconstruction via the retrosternal route more than 2 years after allogeneic bone marrow transplantation. The final pathological diagnosis was esophageal atresia with esophagitis, with no malignancy. During postoperative evaluation, the patient required swallowing training for a few months, although no major complications were noted. Oral intake was possible, and complete remission was maintained at 14 month post-surgery.

Conclusions: Oncologists must consider the possibility of acquired esophageal cicatricial atresia as a complication during chemotherapy for ALCL. If esophageal obstruction or esophageal atresia occur and if remission is maintained, esophagectomy and esophageal reconstruction are useful treatment options for maintaining oral intake.
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http://dx.doi.org/10.1186/s40792-021-01108-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7865044PMC
February 2021

Successful Salvage of Very Early Relapse in Pediatric Acute Lymphoblastic Leukemia With Inotuzumab Ozogamicin and HLA-haploidentical Peripheral Blood Stem Cell Transplantation With Posttransplant Cyclophosphamide.

J Pediatr Hematol Oncol 2021 Jan 27. Epub 2021 Jan 27.

Department of Pediatrics, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Kagoshima Prefecture, Japan.

Herein, we describe a 14-year-old female patient with B-cell precursor acute lymphoblastic leukemia who relapsed in early consolidation. Minimal residual disease-negative complete remission was obtained after 1 cycle of inotuzumab ozogamicin therapy. She underwent HLA-haploidentical peripheral blood stem cell transplantation after a myeloablative conditioning regimen. Posttransplant cyclophosphamide, tacrolimus, and mycophenolate mofetil were administered for the prophylaxis of graft-versus-host disease. At 23 months, she was in complete remission. Although the administration of inotuzumab ozogamicin followed by haploidentical peripheral blood stem cell transplantation with posttransplant cyclophosphamide has been limited in children, this strategy may be an effective treatment for pediatric refractory acute lymphoblastic leukemia.
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http://dx.doi.org/10.1097/MPH.0000000000002079DOI Listing
January 2021

Bacillus Calmette-GuÉrin-Associated Cervical Spondylitis in a 3-Year-Old Immunocompetent Girl.

Pediatr Infect Dis J 2020 12;39(12):e466-e469

From the Department of Pediatrics.

Bacillus Calmette-Guérin (BCG)-associated osteomyelitis is a rare adverse event following BCG vaccination, and there have been no previous reports of BCG-associated cervical spondylitis. Here, we describe the case of a 3-year-old immunocompetent girl who developed BCG-associated cervical spondylitis and was successfully treated by prompt surgical drainage of the abscess and administration of isoniazid and rifampicin for 9 months without sequelae.
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http://dx.doi.org/10.1097/INF.0000000000002893DOI Listing
December 2020

Successful treatment of pulmonary hypertension with unilateral absent pulmonary artery.

Pediatr Int 2020 Sep 7;62(9):1117-1118. Epub 2020 Sep 7.

Department of Pediatrics, Kagoshima University, Kagoshima, Japan.

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http://dx.doi.org/10.1111/ped.14262DOI Listing
September 2020

In-Hospital Management Might Reduce Induction Deaths in Pediatric Patients With Acute Lymphoblastic Leukemia: Results From a Japanese Cohort.

J Pediatr Hematol Oncol 2021 03;43(2):39-46

Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima.

Induction deaths (ID) remain a critical issue in the treatment of pediatric patients with acute lymphoblastic leukemia (ALL). The reported rate of ID in this population is 1% or higher. We speculate that this proportion might be lower in Japan because of mandatory hospitalization during induction therapy to manage complications. We retrospectively analyzed the incidence of ID among children with ALL enrolled in 4 Japanese study groups between 1994 and 2013. Among 5620 children, 41 (0.73%) cases of ID were noted. The median age was 6.5 years; 24 children were female, and 7 had T-cell ALL. Infection was the most common cause of ID (n=22), but the incidence (0.39%) was lower than that reported in western countries. Mortality within 48 hours from the onset of infection was low, comprising 25% of infection-related deaths. The incidence of infections caused by Bacillus species was low. Only 1 patient died because of Aspergillus infection. Fatal infections mostly occurred during the third week of induction therapy. Our findings suggest that close monitoring, stringent infection control, and immediate administration of appropriate antibiotics through hospitalization might be important strategies in reducing the rate of infection-related ID in pediatric patients with ALL.
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http://dx.doi.org/10.1097/MPH.0000000000001926DOI Listing
March 2021

A case of cryopyrin-associated periodic fever syndrome during canakinumab administration complicated by inflammatory bowel disease.

Clin Rheumatol 2021 Jan 5;40(1):393-397. Epub 2020 Jul 5.

Department of Pediatrics, Kagoshima University Hospital, 35-1 Sakuragaoka Kagoshimashi, Kagoshima, 890-8520, Japan.

Cryopyrin-associated periodic fever syndrome (CAPS) is a highly debilitating disorder, which is characterized by unregulated interleukin-1β production driven by autosomal dominantly inherited mutations in the NLRP3 gene. Patients with CAPS often present with early-onset episodes of fever and rash. These patients also present with variable systemic signs and symptoms, such as arthritis, sensorineural hearing loss, chronic aseptic meningitis, and skeletal abnormalities, but minimal gastrointestinal symptoms. Recently, effective therapies for CAPS targeted against interleukin-1 have become available. We report a case of a young Japanese woman with CAPS who developed inflammatory bowel disease during canakinumab therapy. The patient had colostomy after intestinal perforation and changed canakinumab to infliximab. To the best of our knowledge, this is the first report of a case of inflammatory bowel disease secondary to CAPS complicated by gastrointestinal symptoms and arthritis which canakinumab could not control. Patients with CAPS who have symptoms that cannot be controlled by canakinumab should be considered for possible co-morbidities.
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http://dx.doi.org/10.1007/s10067-020-05267-1DOI Listing
January 2021

Basophil Activation Test Based on CD203c Expression in the Diagnosis of Fish Allergy.

Allergy Asthma Immunol Res 2020 Jul;12(4):641-652

Department of Pediatrics, Kagoshima University Graduate School of Medicine, Kagoshima, Japan.

Purpose: The basophil activation test (BAT) has been reported to be useful for the diagnosis of various food allergies, such as allergy to peanut, but not to fish. This study aimed to evaluate the diagnostic performance of the BAT for fish allergy.

Methods: We performed a retrospective review of patients with fish allergy who underwent the BAT using a panel of fish extracts (15 kinds) to examine the differential reactivity to several species of fish. The BAT score for each extract was expressed as the ratio of CD203chigh% with the extract to that with anti-IgE antibody. Clinical reactivity to each fish was confirmed by positive oral food challenge or a typical history of fish-induced immediate allergy symptoms. Receiver-operating-characteristic (ROC) analysis was performed to evaluate the diagnostic performance.

Results: Fifty-one patients with fish allergy were analyzed. Using extracts of 15 species of fish, the BAT was performed a total of 184 times on the patients. Clinical allergy to each species of fish was confirmed in 90 (48.9%) of those tests. ROC analysis yielded high areas under the curve for the BAT scores for the 5 most common fish species (0.72-0.88). The diagnostic accuracy ranged from 0.74 to 0.86. Using a tentative cutoff value of 0.3 deduced from the ROC analyses of the 5 fish species, the accuracy for other fish allergic reactions was generally high (0.6-1.0), except the fish tested in a small number of patients.

Conclusions: The BAT score based on CD203c expression may be useful for fish allergy diagnosis, especially since a large variety of fish can be tested by the BAT using fish extracts prepared by a simple method.
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http://dx.doi.org/10.4168/aair.2020.12.4.641DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7224992PMC
July 2020

Letter to the Editor (Author comments) "Determining risk factors of acute kidney injury after neonatal cardiac surgery".

Clin Exp Nephrol 2020 07 12;24(7):648-649. Epub 2020 Apr 12.

Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, 8-35-1 Sakuragaoka, Kagoshima, 890-8544, Japan.

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http://dx.doi.org/10.1007/s10157-020-01891-yDOI Listing
July 2020

Right-ventricular outflow tract obstruction by adrenocorticotrophic hormone therapy for infantile spasms after Norwood operation.

Pediatr Int 2020 Jan 19;62(1):96-97. Epub 2020 Jan 19.

Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.

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http://dx.doi.org/10.1111/ped.14040DOI Listing
January 2020

Novel AP3B1 compound heterozygous mutations in a Japanese patient with Hermansky-Pudlak syndrome type 2.

J Dermatol 2020 Feb 9;47(2):185-189. Epub 2019 Dec 9.

Department of Pediatrics, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan.

Hermansky-Pudlak syndrome type 2 (HPS2) is an extremely rare autosomal recessive inherited disease characterized by partial oculocutaneous albinism (OCA), bleeding diathesis due to a storage pool deficiency and immunodeficiency. The disorder is caused by disruption of the adapter protein 3 complex, which is involved in impaired intracellular vesicle transport. Here, we report the first case of a 1-year-old girl with HPS2 in Asia. She had no specific symptoms other than OCA and neutropenia. We analyzed her platelet function using transmission electron microscopy and a platelet aggregation test, cytotoxic degranulation assay of her natural killer (NK) cells and bleeding time, the results of which led to the diagnosis of HPS2. Although her NK-cell cytotoxic degranulation was impaired, she had not developed signs of hemophagocytic lymphohistiocytosis (HLH) or fibrosing lung disease. Molecular genetic analyses showed novel heterozygous mutations (c.188T>A [p.M63K] and c.2546>A [p.L849X]) in AP3B1. When examining patients with OCA, blood tests should be performed to confirm neutrophil count, bleeding time and platelet agglutination. When HPS2 is suspected, detailed immunological tests should be considered, and attention should be paid to HLH and pulmonary lesions immediately and over the long term.
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http://dx.doi.org/10.1111/1346-8138.15177DOI Listing
February 2020

Kidney Disease: Improving Global Outcomes in neonates with acute kidney injury after cardiac surgery.

Clin Exp Nephrol 2020 Feb 1;24(2):167-173. Epub 2019 Nov 1.

Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, 8-35-1 Sakuragaoka, Kagoshima, 890-8544, Japan.

Background: Acute kidney injury (AKI) after cardiac surgery (CS-AKI) in children with congenital heart disease is a serious complication closely associated with high morbidity and mortality. Kidney Disease: Improving Global Outcomes (KDIGO) AKI staging demonstrates high sensitivity for detecting AKI and predicting associated in-hospital mortality. However, neonatal-modified KDIGO criteria (n-KDIGO), recently introduced as a standard diagnostic tool, for CS-AKI have not been fully validated. Here, we evaluated the incidence of risk factors and postoperative outcomes of neonatal CS-AKI.

Methods: We retrospectively studied 114 consecutive neonates who underwent cardiac surgery at the Kagoshima University Hospital. CS-AKI was classified using the n-KDIGO criteria. Risk adjustment in congenital heart surgery (RACHS-1) score was used to predict the complexity-adjusted mortality and % fluid overload (%FO) was used to monitor fluid balance in pediatric cardiac surgery.

Results: Among 81 patients, neonatal CS-AKI occurred in 57 (70.4%) patients according to n-KDIGO criteria. Of these, 28 (34.6%) patients reached n-KDIGO 1, 17 (21.0%) reached n-KDIGO 2, and 12 (14.8%) reached n-KDIGO 3. Patients with CS-AKI had significantly higher vasoactive-inotropic score levels, longer operative times, and higher %FO than patients without CS-AKI. Notably, increased duration of cardiopulmonary bypass times and %FO were risk factors for the development of neonatal CS-AKI. The n-KDIGO-based severe AKI grade had higher risk of in-hospital mortality; however, the n-KDIGO-based mild AKI grade was not associated with any postoperative outcomes.

Conclusions: CS-AKI based on n-KDIGO criteria is common in neonates and is closely associated with higher mortality, especially in patients with severe CS-AKI.
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http://dx.doi.org/10.1007/s10157-019-01805-7DOI Listing
February 2020

Nationwide survey of pediatric hypodiploid acute lymphoblastic leukemia in Japan.

Pediatr Int 2019 Nov 21;61(11):1103-1108. Epub 2019 Nov 21.

Department of Hematology/Oncology, Saitama Children's Medical Center, Saitama, Japan.

Background: Ploidy is a highly significant prognostic factor for pediatric acute lymphoblastic leukemia (ALL). Children with hypodiploid ALL have poor outcomes despite current intensive chemotherapy. Little has been investigated with regard to hypodiploid ALL in Japanese children.

Methods: We retrospectively collected clinical data on hypodiploid ALL cases from the registries of prospective multicenter trials conducted by the four independent clinical study groups in Japan between 1997 and 2012.

Results: A total of 117 ALL patients with hypodiploidy were analyzed in this study. There were 101, eight, and eight patients with 45, 44, and fewer than 44 chromosomes, respectively. The 5 year overall survival rates differed significantly: 86.0%, 87.5%, and 62.5% for patients with 45, 44, and fewer than 44 chromosomes, respectively (P = 0.037). Of the eight patients with 44 chromosomes, seven were alive, including five patients who maintained complete remission without undergoing hematopoietic stem cell transplantation (HSCT). Of the eight patients with fewer than 44 chromosomes, six were good responders to prednisolone and none had induction failure, but the relapse rate was high (5/8). No patients had central nervous system relapse. Four patients underwent HSCT after relapse, but only one survived.

Conclusions: Outcomes of Japanese ALL patients with fewer than 44 chromosomes were poor, as previously reported in other countries. Although the sample size was small, patients with 44 chromosomes had better prognoses than those previously reported. Further studies including international collaboration are needed to improve outcomes for pediatric ALL patients with fewer than 44 chromosomes.
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http://dx.doi.org/10.1111/ped.14006DOI Listing
November 2019

Celecoxib as a Potential Treatment for Intractable Lymphatic Malformation.

Pediatrics 2019 09;144(3)

Department of Pediatrics, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan.

Lymphatic malformation (LM) is a congenital disorder resulting from an abnormal development of lymphatic vessels. LM may result in problems of cosmesis and functional impairment, including airway compression. An 11-year-old girl was referred to our department with increasing dysphagia caused by a large left cervical LM with a long history of treatment. Because of the LM location, surgical resection was not an option, and various therapies, including use of picibanil, had proven ineffective. Celecoxib treatment (100 mg/day) was initiated for local pain management. Softening of the lesion was observed 2 weeks after treatment initiation, and the dose was increased to 200 mg/day with additional shrinking of the LM over the next 2 weeks. With parental consent, celecoxib was continued, with a 65% reduction in volume achieved at 6 months. The patient discontinued treatment at 12 months, and the LM volume increased. Control over the LM was achieved with resumption of celecoxib treatment. After 2 years of treatment, the LM persists, but the size of the malformation is significantly smaller. No adverse effects of celecoxib treatment were observed. The anti-cyclooxygenase-2 effect of celecoxib prevented lymphatic vessel growth through an inhibition of cyclooxygenase-2 activity in the conversion of prostaglandin to prostaglandin E2. In conclusion, celecoxib may be a promising therapeutic agent for LM management.
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http://dx.doi.org/10.1542/peds.2019-0319DOI Listing
September 2019

Prominent dermal Langerhans cells in an Omenn syndrome patient with a novel mutation in the IL2RG gene.

J Dermatol 2019 Nov 27;46(11):1019-1023. Epub 2019 Aug 27.

Departments of, Dermatology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.

Prominent dermal infiltration by Langerhans cells (LC) is a rare finding in patients with Omenn syndrome (OS). Here, we report the case study of a 7-month-old boy with OS and with prominent dermal infiltration by LC, which is a rare histological manifestation of the skin. Striking erythroderma appeared in the patient 2 weeks after birth. We also noted alopecia, lymphadenopathy, hepatosplenomegaly, eosinophilia and an elevated serum immunoglobulin E level with hypogammaglobulinemia. Peripheral blood flow cytometry showed the T NK B immunophenotype and genetic analysis, a novel mutation in the IL2RG gene (c.337_339delTCT, p.Ser113del). The final diagnosis was that of OS. He responded well to an allograft umbilical cord blood transplantation that was performed when the patient was 8 months of age. We speculate that the LC accumulated in the dermis will eventually migrate to the regional lymph node, then stimulate autoreactive T cells by overpresenting antigens, thus causing OS-specific skin symptoms.
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http://dx.doi.org/10.1111/1346-8138.15054DOI Listing
November 2019

Slowly progressive acute lymphoblastic leukemia after stem cell transplantation.

Pediatr Int 2019 Aug 27;61(8):831-832. Epub 2019 Aug 27.

Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.

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http://dx.doi.org/10.1111/ped.13932DOI Listing
August 2019

Brentuximab Vedotin and High-dose Methotrexate Administrated Alternately for Refractory Anaplastic Large-cell Lymphoma With Central Nervous System Disease.

J Pediatr Hematol Oncol 2020 08;42(6):e456-e458

Department of Pediatrics, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan.

Pediatric anaplastic large-cell lymphoma (ALCL), which is characterized by strong expression of CD30, is usually responsive to multidrug chemotherapy. Brentuximab vedotin (BV) which is an anti-CD30 antibody-drug conjugate is a promising drug with effects on relapsing or refractory ALCL. However, its effects may not be sufficient for the central nervous system disease. The authors herein reported an 11-year-old boy with ALCL that progressed as central nervous system disease receiving intensive induction chemotherapy has achieved and maintained remission by BV and high-dose methotrexate administrated alternately. Alternate therapy with high-dose methotrexate may complement these shortcomings of BV to provide safe treatment without worsening adverse events.
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http://dx.doi.org/10.1097/MPH.0000000000001550DOI Listing
August 2020

Liver abscess due to Sterigmatomyces halophilus in a boy with acute lymphoblastic leukemia.

J Infect Chemother 2019 Dec 10;25(12):1047-1049. Epub 2019 Jun 10.

Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.

We report the first case of liver abscess due to Sterigmatomyces halophilus. Because this pathogen grows poorly in culture medium without added salts, it was identified by sequencing analysis targeting the rRNA gene internal transcribed spacer (ITS) region. This method could be useful for pathogens that cannot be cultured using standard methods.
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http://dx.doi.org/10.1016/j.jiac.2019.05.021DOI Listing
December 2019

[Acute myeloid leukemia evolving from KIT D816-mutated systemic mastocytosis relapsing two months after completion of chemotherapy].

Rinsho Ketsueki 2019 ;60(5):378-381

Division of Pediatrics, Faculty of Medicine, University of Miyazaki.

Here, we report the case of a 9-year-old girl with acute myeloid leukemia (AML) developed from systemic mastocytosis (SM). She experienced bladder and rectal disturbance due to an extramedullary nodule in the paraspinal region of the sacrum. Cytogenetic and genetic analyses of leukemic cells revealed the KIT D816Y mutation besides t (8;21) (q22:q22) /RUNX1-RUNX1T1. Despite receiving proton beam therapy after conventional chemotherapy, the patient relapsed after 2 months. As SM-AML with the KIT D816 mutation in adults exhibits a poor prognosis, hematopoietic stem cell transplantation is recommended. Owing to a few reports of SM-AML in children, the standard therapy for pediatric cases has not been established to date. Based on our experience and the related literature, the prognosis of childhood SM-AML could be as poor as in adults. Hence, further investigation, including mutational analyses of the KIT gene, is warranted to establish a risk-oriented strategy for managing childhood SM-AML.
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http://dx.doi.org/10.11406/rinketsu.60.378DOI Listing
August 2019

Influence of GST polymorphisms on busulfan pharmacokinetics in Japanese children.

Pediatr Int 2019 Jun;61(6):558-565

Department of Pediatrics, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan.

Background: Fatal adverse effects or relapse can occur with excessive or insufficient busulfan exposure in hematopoietic stem cell transplantation. Given that busulfan is mainly metabolized by glutathione S-transferase (GST), we investigated the influence of GST polymorphisms on busulfan pharmacokinetics in Japanese pediatric patients.

Methods: Blood samples were taken from patients receiving high-dose i.v. busulfan as the first dose. Plasma busulfan concentration was measured using high-performance liquid chromatography. The area under the plasma busulfan concentration-time curve (AUC) was calculated. The genotype of GSTA1 was determined on polymerase chain reaction (PCR)-restriction fragment length polymorphism. Multiplex PCR was used to detect the presence or absence of GSTM1 and GSTT1 in the genomic DNA samples.

Results: Twenty patients were consecutively enrolled. Phenotype prediction was defined as follows: poor metabolizer (n = 4), one or more GSTA1*B haplotype or GSTM1/GSTT1 double-null genotypes; and extensive metabolizer (n = 16), other genotypes. GSTA1, M1, and T1 independently had no significant differences in AUC , clearance or elimination rate constant. For the infant with unexpectedly high AUC (2,591 μmol/L min), the GSTA1, M1, and T1 polymorphisms were wild type. On further analysis, the poor metabolizer group had lower clearance and higher AUC except for the aforementioned patient, compared with the extensive metabolizer group (1,531 vs 1,010 μmol/L min; P < 0.01).

Conclusions: GST polymorphisms may have affected busulfan pharmacokinetics, but these effects were obscured by other factors, such as underlying disease, systemic conditions, treatment history, and race.
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http://dx.doi.org/10.1111/ped.13859DOI Listing
June 2019

Neonatal ventricular tachycardia: Adverse event possibly due to maternal ritodrine.

Pediatr Int 2019 Mar 7;61(3):298-299. Epub 2019 Feb 7.

Department of Pediatrics, Kagoshima University, Kagoshima, Japan.

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http://dx.doi.org/10.1111/ped.13763DOI Listing
March 2019

S100A12 and vascular endothelial growth factor can differentiate Blau syndrome and familial Mediterranean fever from systemic juvenile idiopathic arthritis.

Clin Rheumatol 2019 Mar 8;38(3):835-840. Epub 2018 Nov 8.

Department of Pediatrics, Kagoshima University Hospital, 35-1 Sakuragaoka Kagoshimashi, Kagoshima, 890-8520, Japan.

Objectives: Systemic juvenile idiopathic arthritis (sJIA) has recently become regarded as one of the autoinflammatory syndromes (AIS). However, other AIS, such as familial Mediterranean fever (FMF) and Blau syndrome, have been initially misdiagnosed as sJIA because of the clinical similarities. Making the correct diagnosis in the early stage of these AIS is desirable. Therefore, we evaluated serum S100A12 and vascular endothelial growth factor (VEGF) levels to determine if they could be biomarkers for differentiating these AIS.

Method: Serum S100A12 and VEGF levels were examined in patients with Blau syndrome (n = 4), FMF (n = 4), and sJIA (n = 11) in the active and inactive phases.

Results: In the active phase, S100A12 levels were significantly higher in patients with sJIA and FMF compared with those with Blau syndrome (p < 0.001). VEGF levels of patients with sJIA were significantly higher than those of patients with others (p = 0.001). In the inactive phase, there was no significant difference in VEGF levels. However, colchicine-resistant patients or patients without treatment with FMF showed high levels of S100A12 compared with others.

Conclusions: Measuring both serum S100A12 and VEGF levels may be useful for differentiating patients with Blau syndrome and FMF from those with sJIA at the early stage.
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http://dx.doi.org/10.1007/s10067-018-4359-9DOI Listing
March 2019

Importance of Acute Lymphoblastic Leukemia-type Therapy for Bilineal Acute Leukemia.

J Pediatr Hematol Oncol 2019 08;41(6):504-506

Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.

We examined 3 pediatric patients with bilineal acute leukemia. Patient 1 with B-cell acute lymphoblastic leukemia (ALL) and acute myelogenous leukemia (AML) with B-ALL dominance responded well to prednisolone and ALL-type induction therapy. Patients 2 and 3 with T-ALL and AML with AML dominance responded poorly to prednisolone. Patient 2 was resistant to AML-type therapy; patient 3 was resistant to ALL-type induction therapy until day 15. However, all 3 patients eventually achieved complete remission after ALL-type induction therapy. Thus, ALL-type induction therapy should be initiated for bilineal acute leukemia even with AML-dominant, poor prednisolone response, or poor early response features.
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http://dx.doi.org/10.1097/MPH.0000000000001309DOI Listing
August 2019

Giant radiation-induced cavernous haemangioma before reduced-intensity bone marrow transplantation for acute lymphoblastic leukaemia.

Bone Marrow Transplant 2019 02 23;54(2):312-315. Epub 2018 Jul 23.

Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.

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http://dx.doi.org/10.1038/s41409-018-0272-8DOI Listing
February 2019

Risk Factor Analysis for Secondary Malignancy in Dexrazoxane-Treated Pediatric Cancer Patients.

Cancer Res Treat 2019 Jan 14;51(1):357-367. Epub 2018 May 14.

Department of Pediatrics, Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea.

Purpose: Dexrazoxane has been used as an effective cardioprotector against anthracycline cardiotoxicity. This study intended to analyze cardioprotective efficacy and secondary malignancy development, and elucidate risk factors for secondary malignancies in dexrazoxane-treated pediatric patients.

Materials And Methods: Data was collected from 15 hospitals in Korea. Patients who received any anthracyclines, and completed treatment without stem cell transplantation were included. For efficacy evaluation, the incidence of cardiac events and cardiac event-free survival rates were compared. Data about risk factors of secondary malignancies were collected.

Results: Data of total 1,453 cases were analyzed; dexrazoxane with every anthracyclines group (D group, 1,035 patients) and no dexrazoxane group (non-D group, 418 patients). Incidence of the reported cardiac events was not statistically different between two groups; however, the cardiac event-free survival rate of patients with more than 400 mg/m2 of anthracyclines was significantly higher in D group (91.2% vs. 80.1%, p=0.04). The 6-year cumulative incidence of secondary malignancy was not different between both groups after considering follow-up duration difference (non-D, 0.52%±0.37%; D, 0.60%±0.28%; p=0.55). The most influential risk factor for secondary malignancy was the duration of anthracycline administration according to multivariate analysis.

Conclusion: Dexrazoxane had an efficacy in lowering cardiac event-free survival rates in patients with higher cumulative anthracyclines. As a result of multivariate analysis for assessing risk factors of secondary malignancy, the occurrence of secondary malignancy was not related to dexrazoxane administration.
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http://dx.doi.org/10.4143/crt.2017.457DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6333985PMC
January 2019

Validation of acute kidney injury according to the modified KDIGO criteria in infants after cardiac surgery for congenital heart disease.

Nephrology (Carlton) 2019 Mar;24(3):294-300

Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.

Aim: We aimed to validate the incidence of, risk factors for, and postoperative outcomes of acute kidney injury (AKI) according to the modified Kidney Disease Improving Global Outcomes (m-KDIGO) criteria and compare this criteria with both the paediatric Risk, Injury, Failure, Loss, End-stage disease (pRIFLE) and Acute Kidney Injury Network (AKIN) criteria in infants after cardiac surgery.

Methods: We retrospectively enrolled 145 consecutive infants who underwent open-heart surgery at Kagoshima University Hospital.

Results: Acute kidney injury was present in 55 (37.9%), 111 (75.9%), and 95 (65.5%) patients according to the m-KDIGO, pRIFLE, and AKIN criteria, respectively. Among these, 71.9% of patients pRIFLE Risk patients and 60.5% of AKIN 1 patients were categorized in the 'no-AKI' group according to the m-KDIGO criteria. Low body weight (m-KDIGO odds ratio [OR], 0.73; P = 0.015; pRIFLE OR, 0.66; P = 0.001; AKIN OR 0.69, P = 0.002) and prolonged cross-clamp time (m-KDIGO OR, 1.02;
Conclusion: Application of the three criteria resulted in different AKI incidences, but each criterion could be useful for detecting risk factors for AKI. Notably, using m-KDIGO criteria provides more important subsequent postoperative outcomes. The m-KDIGO AKI criteria describe clinically relevant AKI in infants after cardiac surgery.
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http://dx.doi.org/10.1111/nep.13240DOI Listing
March 2019

Guillain-Barré syndrome and optic neuritis after Mycoplasma pneumoniae infection.

Brain Dev 2018 May 9;40(5):439-442. Epub 2018 Feb 9.

Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.

We report the case of a 12-year-old girl who developed Guillain-Barré syndrome (GBS) and optic neuritis (ON) following Mycoplasma pneumoniae infection. Her symptoms, including bilateral vision impairment and tingling in her hands and right foot, were resolved after methylprednisolone pulse therapy. Serum anti-galactocerebroside (Gal-C) IgM antibodies were detected in our patient. This is the first report of a child with GBS and ON associated with M. pneumoniae infection.
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http://dx.doi.org/10.1016/j.braindev.2018.01.007DOI Listing
May 2018