Publications by authors named "Daisuke Hata"

49 Publications

Edi catheter-related gastric perforation in a 373 g infant.

Pediatr Int 2021 Jun 25;63(6):734-736. Epub 2021 May 25.

Departments of, Pediatrics, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka, Japan.

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http://dx.doi.org/10.1111/ped.14482DOI Listing
June 2021

Early therapy with corticosteroid and surfactant for acute idiopathic pulmonary hemorrhage in infants: Two case reports.

Medicine (Baltimore) 2020 May;99(21):e20281

Department of Pediatrics, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka, Japan.

Rationale: Acute idiopathic pulmonary hemorrhage (AIPH) in infants is a rare condition, and a clear treatment protocol has not yet been established.

Patient Concerns: We report 2 infant cases of AIPH in a 3-month-old male and a 1-month-old female, who presented at an emergency room with epistaxis and respiratory distress. Both were immediately intubated, which revealed a bloody intratracheal aspirate.

Diagnosis: Pulmonary hemorrhage was confirmed by X-ray and computed tomography imaging in both cases. The extensive evaluation revealed no specific etiology for the acute pulmonary hemorrhage, and AIPH was therefore diagnosed in both cases.

Interventions: Intravenous methylprednisolone resulted in a rapid improvement in oxygenation and a reduction in high airway pressure during mechanical ventilation. Methylprednisolone was subsequently tapered off within 13 and 3 days in cases 1 and 2, respectively. In case 1, intratracheal administration of a surfactant also resulted in an immediate improvement in respiratory condition and the patient was extubated after 2 days; no effect was seen in case 2, and the patient was extubated after 10 days.

Outcome: Both infants recovered well without sequelae or further relapse after 23 and 71 months of follow-up, respectively.

Lessons: Early administration of corticosteroid therapy and intratracheal administration of diluted surfactant should be considered for severe acute pulmonary hemorrhage in infants.
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http://dx.doi.org/10.1097/MD.0000000000020281DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7249888PMC
May 2020

Management of a case of transient neonatal diabetes mellitus using continuous glucose monitoring.

Clin Pediatr Endocrinol 2020 16;29(2):77-80. Epub 2020 Apr 16.

Department of Pediatrics, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka, Japan.

Neonatal diabetes mellitus (NDM) is a very rare disorder and its diagnosis can be challenging especially in mild and transient cases. Herein, we describe a 2.4-kg female infant born at 38 wk of gestation who showed hyperglycemia (388 mg/dL) on Day 1. Intermittent blood sampling showed glucose concentrations of 100-150 mg/dL on Day 2-5. However, continuous glucose monitoring (CGM) from Day 7 revealed hyperglycemia (> 200 mg/dL) after every feeding. The patient required low-dose (0.1-0.2 U/kg/d) insulin therapy for a short period (7 d). During the treatment, hypoglycemic (< 50 mg/dL) events were not detected by real- time CGM. Follow-up CGM from Day 32 showed normoglycemia for 3 full days; therefore, we ascertained that the diabetes had been transient. Later genetic analysis revealed an abnormal methylation pattern on chromosome 6q24, which is the most frequent cause of transient NDM. Most cases of 6q24-related NDM relapse after puberty, implying that long term follow up is required. We speculate that the NDM in this case might not have been diagnosed without CGM. This report highlights the usefulness of CGM for the initial diagnosis, monitoring during insulin therapy, and confirmation of improvement in patients with transient NDM.
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http://dx.doi.org/10.1297/cpe.29.77DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7160458PMC
April 2020

Colonic Perforation in a Term Newborn with Hereditary Protein C Deficiency.

Indian Pediatr 2019 12;56(12):1057-1059

Department of Pediatrics, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka, Japan.

We describe a term infant who experienced recurrent apnea associated with intracranial hemorrhage and later, developed colonic perforation. Plasma protein C activity was below detectable limits and a heterozygous PROC mutation was identified. Neonatal colonic perforation is rare, and this case report highlights the importance of considering congenital Protein C deficiency.
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December 2019

Successful Conservative Treatment of Mycotic Pulmonary Artery Aneurysms Caused by MRSA Bacteremia.

Pediatrics 2019 11 17;144(5). Epub 2019 Oct 17.

Department of Pediatrics, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka City, Osaka, Japan; and

Mycotic pulmonary artery aneurysms (MPAAs) are rare and life-threatening with currently no recommended treatment strategies. In this report, we describe a successfully treated case of ventricular septal defect in an 11-month-old girl who developed bacteremia, infective endocarditis, and MPAA caused by methicillin-resistant (MRSA). We first started vancomycin, gentamycin, and panipenem-betamipron for infective endocarditis but switched to teicoplanin and arbekacin on day 3 after initiating treatment because bacteremia persisted, and vancomycin minimum inhibitory concentration was relatively high at 2 mg/L. Although we added clindamycin on day 5 and fosfomycin on day 7, MRSA bacteremia persisted, and we finally added daptomycin at 10 mg/kg per day on day 8, whereupon the bacteremia subsided within a day. Although the bacteremia subsided, the patient developed septic pulmonary embolisms and septic arthritis on her left knee. We continued daptomycin but switched the concomitant drug to linezolid, trimethoprim-sulfamethoxazole, and rifampicin on day 11. After several repeats of puncture and lavage of her knee joint, she became afebrile on day 16. Computed tomography scans taken on day 32 revealed right pulmonary artery MPAAs. She was treated with long-term multidrug therapy, and MPAAs were absent on subsequent computed tomography scans on day 184. Multidrug therapy mainly based on daptomycin could be a possible salvage therapy for refractory MRSA bacteremia with high vancomycin minimum inhibitory concentration. Conservative treatment should be selectively considered as a treatment option for clinically stable MPAA instead of surgical and endovascular treatment.
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http://dx.doi.org/10.1542/peds.2019-0672DOI Listing
November 2019

Transition of gastric pH after birth for verification of feeding tube placement.

Pediatr Int 2019 Aug 26;61(8):832-834. Epub 2019 Aug 26.

Department of Pediatrics, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka, Japan.

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http://dx.doi.org/10.1111/ped.13918DOI Listing
August 2019

Gene Mutation in a Dizygous Twin With Transient Neonatal Hyperparathyroidism.

J Endocr Soc 2019 Mar 3;3(3):602-606. Epub 2019 Jan 3.

Department of Pediatrics, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka, Japan.

Maternal-fetal transport of calcium (Ca) is important for bone mineralization in fetal development. Insufficient Ca transport causes transient neonatal hyperparathyroidism (TNHP). Transient receptor potential cation channel, subfamily V, member 6 (TRPV6), has been found to play an important role in the active transport of Ca through the placenta. Recently, gene was found to be the gene responsible for TNHP with severe skeletal undermineralization. To date, only seven cases of TNHP caused by recessive mutations have been reported. We present a case of TNHP caused by gene mutations. A female newborn was hospitalized because of respiratory distress. Marked undermineralization of the skeleton was observed in X-ray imaging. Laboratory examination revealed markedly high PTH and absence of hypercalcemia along with vitamin D deficiency. Her twin brother presented with almost no symptoms. Maternal laboratory findings indicated normocalcemia, but vitamin D deficiency with a high PTH level for the lactation period was observed. We initially diagnosed the patient as having secondary hyperparathyroidism because of maternal vitamin D deficiency. Nevertheless, the reasons underlying the discordant clinical manifestations between the twin siblings remained unclear. Our analysis of gene clarified that the patient had compound heterozygote mutations, which were reported previously (p.Ile223Thr and p.Gly428Arg). Pathologic mutations in gene were not detected in the other sibling. The clinical symptoms in the patient were transient: they resolved during infancy. TNHP caused by gene mutations is a unique disease in terms of its transient pathology and relief after birth.
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http://dx.doi.org/10.1210/js.2018-00374DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6389352PMC
March 2019

Introduction of laryngeal mask airway in Japan, and its rescue use for newborns.

Pediatr Int 2018 Oct;60(10):954-956

Department of Pediatrics, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka, Japan.

Background: In neonatal resuscitation, laryngeal mask airway (LMA) is recommended when both face mask ventilation and endotracheal intubation fail. Experience of LMA among obstetricians, nurses and midwives in Japan, however, is limited. The aims of the present study were to (i) offer an LMA training course to professionals dealing with low-risk pregnancies at institutions across Japan; and (ii) assess the subsequent use and value of LMA at the participating institutions.

Methods: Between August 2016 and March 2017, a total of 18 training courses for 60 min were provided for around 350 medical personnel from 51 institutes. LMA use over the subsequent 12 months was assessed via a postal questionnaire.

Results: After training, a total of 38 institutes introduced LMA. Of 13 254 live births, seven cases of rescue use LMA in "cannot ventilate, cannot intubate" situations were reported. None of these seven newborns had any malformation of the upper airway. LMA insertion resulted in adequate ventilation in all seven cases.

Conclusion: LMA can be a life-saving tool in neonatal resuscitation. All medical institutions dealing with low-risk pregnancies in Japan should be equipped with this device.
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http://dx.doi.org/10.1111/ped.13670DOI Listing
October 2018

Recurrence of meningitis in a preterm infant and co-infection of echovirus 18.

IDCases 2017 10;10:135-137. Epub 2017 Nov 10.

Department of Pediatrics, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka, Japan.

Introduction: Bacterial meningitis may relapse after adequate antibiotic treatment. In most cases, however, the pathophysiology cannot be identified.

Presentation Of Case: We describe a preterm infant with recurrent episodes of meningitis due to infection with an identical strain both at birth and at 10 days after cessation of a 3 week course of appropriate antibiotic treatment. At the time of recurrence, the patient presented with fulminant severe cardiac failure due to acute myocarditis, coupled with a concurrent echovirus 18 infection (confirmed by stool culture and serological analysis).

Conclusion: Co-infection by echovirus may underlie recurrence of meningitis in this case.
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http://dx.doi.org/10.1016/j.idcr.2017.11.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5695537PMC
November 2017

Herpes zoster meningitis in immunocompetent children: Two case reports and a literature review.

Pediatr Int 2017 Oct;59(10):1116-1118

Department of Pediatrics, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka City, Osaka, Japan.

We encountered two cases of Herpes zoster (HZ) meningitis, a rarely occurring complication of HZ, in previously healthy children. One patient treated with i.v. acyclovir (ACV, 31 mg/kg/day) did not recover. His symptoms were relieved somewhat by increased ACV dosage, but it caused transient renal dysfunction. Another patient treated with i.v. ACV (30 mg/kg/day) recovered. Treatment for HZ meningitis in immunocompetent children has not been established. In a literature review, 80% of 20 patients were treated with the usual dose of ACV 15-30 mg/kg/day. The present cases suggest that a high dosage of ACV up to 60 mg/kg/day should be considered (while monitoring for side-effects) unless symptoms improve. In the review, one of every three vaccine-strain Varicella zoster virus (VZV) cases was severe, whereas the present cases resulted from wild type. Further investigations must examine different clinical characteristics of HZ meningitis caused by wild-type and vaccine-strain VZV.
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http://dx.doi.org/10.1111/ped.13362DOI Listing
October 2017

Vaccine-strain herpes zoster found in the trigeminal nerve area in a healthy child: A case report.

J Clin Virol 2016 12 3;85:44-47. Epub 2016 Nov 3.

Department of Pediatrics, Kitano Hospital, The Tazuke Kofukai Medical Research Institute, 2-4-20, Ohgimachi, Kita-ku, Osaka city, Osaka, Japan. Electronic address:

A previously healthy 2-year-old girl, vaccinated for varicella at 17 months, was admitted because of left-sided facial herpes zoster caused by vaccine-strain varicella-zoster virus (VZV). She recovered fully with no complication after intravenous treatment using acyclovir. Earlier reports have described that herpes zoster (HZ) rashes caused by vaccine-strain VZV tend to occur on the dermis corresponding to the skin area where the varicella vaccine was received. However, rashes appeared on this girl only in the trigeminal nerve area, which is unrelated to the vaccinated site. Results underscore the importance of distinguishing vaccine-strain VZV from wild-type VZV whenever encountering HZ cases after vaccination, even in immunocompetent children, irrespective of the skin lesion site. Monitoring vaccine-strain HZ incidence rates is expected to elucidate many aspects of varicella vaccine safety.
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http://dx.doi.org/10.1016/j.jcv.2016.10.022DOI Listing
December 2016

Two mild cases of Dravet syndrome with truncating mutation of SCN1A.

Brain Dev 2017 Jan 17;39(1):72-74. Epub 2016 Aug 17.

Department of Pediatrics, Kitano Hospital, The Tazuke Kofukai Medical Institute, Osaka, Japan.

Background: SCN1A is the gene that codes for the neuronal voltage-gated sodium-channel alpha-subunit 1. It is generally considered that an SCN1A truncating mutation causes the severe phenotype of Dravet syndrome.

Patients: We describe 11- and 4-year-old male patients presenting with mild Dravet syndrome with a truncating mutation of SCN1A. The former patient showed moderate mental retardation; however, seizure was controlled to almost one incident a year by levetiracetam and topiramate. Carbamazepine was also effective, which is atypical of Dravet syndrome. The latter patient showed a borderline developmental quotient and did not have episodes of afebrile seizure.

Conclusion: Two patients presented with mild Dravet syndrome, even though they had a truncating mutation of SCN1A. Not all truncating mutations of SCN1A cause the severe phenotype of Dravet syndrome.
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http://dx.doi.org/10.1016/j.braindev.2016.07.006DOI Listing
January 2017

The utility of electrocardiogram for evaluation of clinical cardiac arrest in neonatal resuscitation.

Resuscitation 2016 07 28;104:e3-4. Epub 2016 Apr 28.

Department of Pediatrics, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka, Japan.

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http://dx.doi.org/10.1016/j.resuscitation.2016.04.012DOI Listing
July 2016

Novel AVPR2 mutation causing partial nephrogenic diabetes insipidus in a Japanese family.

J Pediatr Endocrinol Metab 2016 May;29(5):591-6

Background: X-linked recessive congenital nephrogenic diabetes insipidus (NDI) is caused by mutations of the arginine vasopressin type 2 receptor gene (AVPR2). More than 200 mutations of the AVPR2 gene with complete NDI have been reported although only 15 mutations with partial NDI has been reported to date.

Methods: We herein report a Japanese kindred with partial NDI. The proband is an 8-year-old boy who was referred to our hospital for nocturnal enuresis. Water deprivation test and hypertonic saline test suggested partial renal antidiuretic hormone arginine vasopressin (AVP) resistance.

Results: Analysis of genomic DNA revealed a novel missense mutation (p.L161P) in the patient. The patient's mother was heterozygous for the mutation. Three-dimensional (3-D) modeling study showed that L161P possibly destabilizes the transmembrane domain of the V2 receptor, resulting in its misfolding or mislocalization.

Conclusions: Distinguishing partial NDI from nocturnal enuresis is important. A clinical clue for diagnosis of partial NDI is an incompatibly high level of AVP despite normal serum osmolality.
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http://dx.doi.org/10.1515/jpem-2015-0323DOI Listing
May 2016

Intraday glucose fluctuation is common in preterm infants receiving intermittent tube feeding.

Pediatr Int 2016 May 2;58(5):359-62. Epub 2016 Feb 2.

Department of Pediatrics, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka, Japan.

Background: We previously reported on three preterm infants with blood glucose abnormalities after reaching full enteral feeding. Recently, it has been shown that clinically stable preterm infants may have large fluctuations in blood glucose after the establishment of enteral nutrition. We hypothesized that intraday glucose fluctuation is a common finding in preterm infants, but improves at term post-conceptual age. This report describes a case series.

Methods: From June 2010 to July 2012, 13 preterm infants (29.5 ± 2.1 post-conceptual weeks, 1144 ± 319 g) were enrolled in this study. Continuous glucose monitoring (CGM) was conducted on average at 33.5 ± 1.4 post-conceptual weeks, when they received gastric tube feeding every 3 h in the absence of i.v. glucose supply.

Results: Eight infants (62%) had large intraday glucose fluctuation with repeated hyperglycemic (>150 mg/dL) and hypoglycemic (<50 mg/dL) events. In five infants, follow-up CGM at 36-38 weeks post-conceptual age showed more stable glycemic changes without any abnormal glucose levels.

Conclusions: On CGM, in some preterm infants intermittent tube feeding resulted in large intraday glucose fluctuation at 31-35 post-conceptual weeks, but the pattern disappeared before discharge (36-38 post-conceptual weeks).
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http://dx.doi.org/10.1111/ped.12838DOI Listing
May 2016

Kawasaki disease with pulmonary nodules and coronary artery involvement: a report of two cases and a review of the literature.

Int J Rheum Dis 2017 Nov 22;20(11):1862-1864. Epub 2015 Jul 22.

Department of Pediatrics, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka city, Japan.

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http://dx.doi.org/10.1111/1756-185X.12692DOI Listing
November 2017

Fetal Erythroblastosis May Be an Indicator of Neonatal Transient Hyperinsulinism.

Neonatology 2015 2;108(2):88-92. Epub 2015 Jun 2.

Department of Pediatrics, Medical Research Institute, Kitano Hospital Tazuke Kofukai, Osaka, Japan.

Background: Small for gestational age and birth asphyxia are associated with neonatal transient hyperinsulinism (THI). Some newborns with THI showed marked erythroblastosis on admission to our neonatal intensive care unit.

Objective: This study was designed to test our hypothesis that fetal erythroblastosis may be a risk factor for developing THI.

Methods: The records of all babies admitted to our neonatal intensive care unit within 24 h of birth between January 2010 and May 2014, and who were born after 34 weeks of gestation, were retrospectively reviewed. Hyperinsulinism was diagnosed as hypoglycemia concomitant with high serum insulin in babies requiring >6 mg/kg/min intravenous glucose and THI as hyperinsulinism without maternal diabetes or genetic disorders. The following three possible risk factors for THI were evaluated: (1) birth weight z-score, (2) 1-min Apgar score and (3) absolute nucleated red blood cell (aNRBC) count on admission.

Results: Of 705 infants, 8 were diagnosed with THI. Multivariate logistic regression analysis revealed that the aNRBC count was the most significant risk factor for THI. The median aNRBC count was 181/µl (interquartile range 0-538/µl), and 8 of 71 infants (11.3%) having an aNRBC count >1,413/µl (90th percentile in this study) had THI. The aNRBC counts in the 8 cases with THI were significantly higher than those in the 5 cases with hyperinsulinism caused by maternal diabetes or genetic disorders.

Conclusions: This study showed that the aNRBC count was strongly associated with subsequent THI. Fetal erythroblastosis, characterized by chronic fetal hypoxia, may be an indicator of perinatal stress sufficient to cause THI.
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http://dx.doi.org/10.1159/000381701DOI Listing
May 2016

Somatic mosaicism for a NRAS mutation associates with disparate clinical features in RAS-associated leukoproliferative disease: a report of two cases.

J Clin Immunol 2015 Jul 21;35(5):454-8. Epub 2015 Apr 21.

Department of Pediatrics, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka, Japan,

RAS-associated leukoproliferative disease (RALD) is a newly classified disease; thus its clinical features and management are not fully understood. The cases of two patients with characteristic features of RALD are described herein. Patient 1 was a 5-month-old female with clinical features typical of autoimmune lymphoproliferative syndrome (ALPS) and markedly elevated TCRαβ(+)CD4(-)CD8(-) T cell numbers. Genetic analyses failed to detect an ALPS-related gene mutation; however, whole exome sequencing and other genetic analyses revealed somatic mosaicism for the G13D NRAS mutation. These data were indivative of NRAS-associated RALD with highly elevated αβ-double-negative T cells. Patient 2 was a 12-month-old girl with recurrent fever who clearly met the diagnostic criteria for juvenile myelomonocytic leukemia (JMML). Genetic analyses revealed somatic mosaicism, again for the G13D NRAS mutation, suggesting RALD associated with somatic NRAS mosaicism. Notably, unlike most JMML cases, Patient 2 did not require steroids or hematopoietic stem cell transplantation. Genetic analysis of RAS should be performed in patients fulfilling the diagnostic criteria for ALPS in the absence of ALPS-related gene mutations if the patients have elevated αβ-double-negative-T cells and in JMML patients if autoimmunity is detected. These clinical and experimental data increase our understanding of RALD, ALPS, and JMML.
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http://dx.doi.org/10.1007/s10875-015-0163-3DOI Listing
July 2015

Expiratory CO2 as the first sign of successful ventilation during neonatal resuscitation.

Pediatr Int 2015 ;57(1):186-8

Department of Pediatrics, Tazuke Kofukai, Medical Research Institute, Kitano Hospital, Osaka, Japan.

Three-lead electrocardiography and expired CO2 monitoring were used during positive pressure ventilation of seven non-intubated newborns (gestational age, 31-37 weeks; birthweight, 1503-2885 g). In all cases, adequate CO2 (>15 mmHg) was detected prior to the achievement of stable heart rate (>100 beats/min). The delay between detection of adequate CO2 and improvement of bradycardia ranged from 8 to 73 s (median, 15 s). Inadequate expired CO2 during positive pressure ventilation indicates airway obstruction or poor aeration of the newborn lungs. Thus, positive expiratory CO2 can be the first recognizable sign of successful ventilation during neonatal resuscitation.
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http://dx.doi.org/10.1111/ped.12553DOI Listing
December 2016

Transient neonatal hyperinsulinism with adaptation disorders: a report of three cases.

J Pediatr Endocrinol Metab 2015 Mar;28(3-4):337-40

Transient hyperinsulinism can occur in neonates following exposure to perinatal stress, such as intrauterine growth restriction and birth asphyxia. However, little is known about its pathophysiology and clinical manifestations. We report three neonatal cases of transient severe hyperinsulinism complicated with cardiopulmonary problems, thrombocytopenia, and marked erythroblastosis at birth. All cases showed signs of placental insufficiency, indicating that chronic hypoxia and malnutrition during fetal development might be associated with characteristic clinical features after birth. Perinatal stress-associated hyperinsulinism can be regarded as a systemic syndrome characterized by cardiopulmonary and hematological problems due to fetal chronic hypoxia.
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http://dx.doi.org/10.1515/jpem-2014-0106DOI Listing
March 2015

Severe Hemolytic Jaundice in a Neonate with a Novel COL4A1 Mutation.

Pediatr Neonatol 2016 12 23;57(6):522-525. Epub 2014 May 23.

Department of Pediatrics, Kitano Hospital, Tazuke Kofukai, Medical Research Institute, Osaka, Japan.

We report our experience with a preterm infant with severe hemolytic jaundice who required exchange transfusion just after birth. The patient was negative for alloimmune hemolysis as a result of maternal-fetal blood type incompatibility, and tests for inherited defects in erythrocyte metabolism, membrane function, and hemoglobin synthesis were normal. We also performed a bone marrow examination, but could not identify the cause of hemolysis. The patient had several other complications, including porencephaly, epilepsy, elevated serum levels of creatine kinase, and persistent microscopic hematuria. Later, we detected a genetic mutation in COL4A1, which was recently found to be associated with hemolytic anemia. We therefore believe that all of the patient's clinical features, including hemolytic anemia, were due to the mutation in COL4A1. Genetic testing for COL4A1 mutations is recommended in neonates who exhibit hemolytic disease of unknown etiology, especially when other complications compatible with COL4A1-related disorders are present.
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http://dx.doi.org/10.1016/j.pedneo.2014.04.001DOI Listing
December 2016

Continuous glucose monitoring for suspected dumping syndrome in infants after Nissen fundoplication.

Pediatr Int 2013 Dec;55(6):782-5

Department of Pediatrics, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka, Japan.

Dumping syndrome is infrequently reported, but known to occur after Nissen fundoplication in children. However, it may be difficult both to diagnose and manage. Here we presented four infants who received Nissen fundoplication for severe gastroesophageal reflux disease, two of whom developed dumping syndrome whilst the other two did not. Continuous glucose monitoring (CGM) was very useful to clearly detect large glycemic fluctuation around each feeding. CGM was also helpful to prove the effect of treatment to avoid abnormal glucose levels. We believe that dumping syndrome in children may be underdiagnosed if clinicians rely solely on the recognition of symptoms or limited frequency of blood samplings. CGM might be the most sensitive diagnostic tool.
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http://dx.doi.org/10.1111/ped.12133DOI Listing
December 2013

A haploinsufficiency of FOXG1 identified in a boy with congenital variant of Rett syndrome.

Brain Dev 2014 Sep 16;36(8):725-9. Epub 2013 Oct 16.

Department of Pediatrics, Kitano Hospital, The Tazuke Kofukai Medical Institute, Osaka, Japan.

Background: Forkhead box G1 gene (FOXG1) mutations and deletions are associated with a congenital variant of Rett syndrome (RTT). Nucleotide alterations of the coding region of FOXG1 have never caused dysmorphic features.

Patient: An 8-year-old boy with the congenital variant of RTT who showed severe psychomotor deterioration, epilepsy, acquired microcephaly, and involuntary movements including jerky movements of the upper limbs and tongue protrusion. He showed dysmorphic features including round face, anteverted nostrils, and tented upper lips. Brain magnetic resonance imaging showed hypoplasia of the frontal lobes and the rostral part of the corpus callosum. The molecular cytogenetic analysis confirmed a de novo deletion of 14q12 including FOXG1 in this patient.

Conclusion: We identified the smallest deletion of 14q12 involving FOXG1 among those previously reported. Dysmorphic facial features are a characteristic for the patients with chromosomal deletion including FOXG1. In our patient, C14orf23 is the only transcript other than FOXG1. Therefore, C14orf23 might be responsible for facial dysmorphism.
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http://dx.doi.org/10.1016/j.braindev.2013.09.006DOI Listing
September 2014

Glycemic variability in preterm infants receiving intermittent gastric tube feeding: report of three cases.

Pediatr Int 2013 Apr;55(2):e25-8

Department of Pediatrics, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka, Japan.

Late-onset hypoglycemia (day 12-16, blood glucose <50 mg/dL) was detected in three preterm infants (birthweight 998-1780 g; gestational age 27-30 weeks) by routine screening. All infants showed high serum insulin levels and extremely low ketone levels at the time of hypoglycemia. Continuous glucose monitoring was conducted at 31-34 weeks' postconceptual age when the infants were receiving intermittent gastric tube feeding with no intravenous glucose infusion. The continuous glucose monitoring results showed characteristic postprandial glucose increases and subsequent sharp deceases along with many hyper- and hypoglycemic events. This fluctuating pattern disappeared at 38-40 weeks' postconceptual age. These observations suggest that prolonged insulin oversecretion may be associated with early aggressive intravenous nutrition, and that large glycemic variability is a common feature of tube-fed preterm infants that can be explained by immature glucose homeostasis.
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http://dx.doi.org/10.1111/j.1442-200X.2012.03728.xDOI Listing
April 2013

Refeeding syndrome in a small-for-dates micro-preemie receiving early parenteral nutrition.

Pediatr Int 2012 Oct;54(5):715-7

Department of Pediatrics, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka, Japan.

This report describes a small-for-date extremely low birth weight infant who manifested bradycardic events, respiratory failure, and hemolytic jaundice during her first week of life. These complications were attributed to severe hypophosphatemia and hypokalemia. Inadequate supply and refeeding syndrome triggered by early aggressive parenteral nutrition were responsible for electrolyte abnormalities.
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http://dx.doi.org/10.1111/j.1442-200X.2012.03590.xDOI Listing
October 2012

Low response to a monovalent inactivated unadjuvanted influenza A (H1N1) pdm09 vaccine in pediatricians of a general hospital in Japan.

Hum Vaccin Immunother 2012 May 1;8(5):587-91. Epub 2012 May 1.

Department of Pediatrics, Kitano Hospital, The Tazuke Kofukai Medical Research Institute, Osaka, Japan.

Immunization of health care personnel (HCP) is critically important to reduce healthcare-associated influenza infections substantially. During 2009-2010, 74% of all HCP at Kitano Hospital, Osaka, Japan, including 94% of pediatricians, received the monovalent unadjuvanted influenza A (H1N1) pdm09 vaccine. We evaluated the vaccine's immunogenicity. Sixteen pediatricians received 15 μg hemagglutinin antigen subcutaneously. Antibody titer assays were conducted using hemagglutination-inhibition antibody assay on days 0 and 21, and at 5 mo after vaccination. Seroprotection rates, seroconversion rates, and geometric mean titer folds at 21 d were, respectively, 43.8%, 43.8%, and 5.4 in all subjects, 70.0%, 70.0%, and 8.0 in subjects aged 27-34 y, and 0.0%, 0.0%, and 8.0 in subjects aged ≥ 35 y. None of the latter group met the European Medicines Agency criteria. We hope to adopt intradermal routes and further the development of the influenza vaccine using new technology to improve immunogenicity in Japan.
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http://dx.doi.org/10.4161/hv.19296DOI Listing
May 2012

Electrocardiogram shows reliable heart rates much earlier than pulse oximetry during neonatal resuscitation.

Pediatr Int 2012 Apr 22;54(2):205-7. Epub 2011 Dec 22.

Department of Pediatrics, Kitano Hospital, Tazuke Kofukai Medical Research Institute, Osaka, Japan.

Background: The aim of this study was to determine the usefulness of the three-lead electrocardiogram (ECG) during neonatal resuscitation.

Methods: Both pulse oximetry (PO) and ECG were applied immediately after delivery to measure heart rate (HR). We reviewed video recordings of the respective monitors, and checked the time at which each monitor started to display reliable rate values.

Results: In 20 deliveries, ECG showed HR much earlier than PO (median 38 s vs 122 s after delivery). ECG displayed reliable HR throughout resuscitation. We were able to confirm the effectiveness of the initial respiratory support from the elevation in HR.

Conclusions: ECG was a safe and reliable method for showing HR, and was used to determine the initiation and the effectiveness of resuscitation in the delivery room.
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http://dx.doi.org/10.1111/j.1442-200X.2011.03506.xDOI Listing
April 2012
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