Kanagawa Children's Medical Research Articles

Late-gestation prediction of outcome in tricuspid valve dysplasia and Ebstein's anomaly using fetal tricuspid regurgitation waveform analysis.

To investigate the criteria, based on fetal TR waveforms in late gestation, to predict biventricular circulation (BV) after birth in cases of tricuspid valve dysplasia (TVD) or Ebstein's anomaly diagnosed during the fetal period. We included 35 consecutive cases diagnosed with TVD or Ebstein's anomaly during the fetal period between January 2008 and December 2021 at Kanagawa Children's Medical Center, Kanagawa, Japan. The maximum velocity and change in pressure over time of tricuspid regurgitation (TR) jet (dP/dt), estimated using TR waveforms obtained during the late-gestation period (gestational age ≥ 28 weeks), were collected from patient records. dP/dt was calculated by dividing the change in estimated right ventricular pressure obtained using Bernoulli's principle by the time taken for the TR maximum velocity to change from one-third to two-thirds of its peak value. The outcome was divided into four categories: BV, single ventricular circulation, neonatal death and fetal death. Patients with BV were included in the BV group, while patients with single ventricular circulation, neonatal death or fetal death were included in the non-BV (NBV) group. Overall, 19 and 16 patients were included in the BV and NBV groups, respectively. The median TR maximum velocity was 3.3(range, 2.4-3.6) m/s in the BV group and 1.9(range, 1.0-3.3) m/s in the NBV group. There were no cases of postnatal BV in fetuses with TR maximum velocity < 2.4 m/s; cases with TR maximum velocity of 2.4-3.3 m/s were observed in both BV and NBV groups. Receiver-operating-characteristics-curve analysis was performed on the 11 patients in the BV group and five patients in the NBV group with a TR maximum velocity of 2.4-3.3 m/s. dP/dt ≥ 350 mmHg/s and TR maximum velocity ≥ 2.9 m/s were identified as criteria for predicting the outcome in such cases. The performance of dP/dt ≥ 350 mmHg/s in predicting BV after birth in fetuses with TVD or Ebstein's anomaly was higher compared to that of TR maximum velocity ≥ 2.9 m/s (sensitivity, 90.9% vs 72.3% and specificity, 80.0% vs 80.0%, respectively). In fetuses with TVD or Ebstein's anomaly, the postnatal outcome may be BV or NBV when the TR maximum velocity is 2.4-3.3 m/s. In such cases, by combining the TR maximum velocity with dP/dt ≥ 350 mmHg/s, BV after birth may be predicted with greater accuracy. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.

Lung disease due to FLNA mutation improved after shunt closure for congenital heart disease

Pediatric PulmonologyVolume 56, Issue 5 p. 1280-1282 LETTER TO THE EDITOR Lung disease due to FLNA mutation improved after shunt closure for congenital heart disease Satomi Mori MD, Corresponding Author Satomi Mori MD satomiforest@gmail.com orcid.org/0000-0002-8026-6954 Department of General Medicine, Kanagawa Children's Medical Center, Yokohama, Japan Correspondence Satomi Mori, MD, Department of general medicine, Kanagawa Children's Medical Center, Mutsukawa 2-138-4, Minami-ku, Yokohama, Kanagawa 232-8555, Japan. Email: satomiforest@gmail.comSearch for more papers by this authorKoji Tanoue MD, Koji Tanoue MD orcid.org/0000-0003-3295-5306 Department of General Medicine, Kanagawa Children's Medical Center, Yokohama, JapanSearch for more papers by this authorHiroyuki Shimizu MD, Hiroyuki Shimizu MD Department of Critical Care Medicine, Kanagawa Children's Medical Center, Yokohama, JapanSearch for more papers by this authorHiroyuki Nagafuchi MD, Hiroyuki Nagafuchi MD Department of Critical Care Medicine, Kanagawa Children's Medical Center, Yokohama, JapanSearch for more papers by this authorKi-Sung Kim MD, PhD, Ki-Sung Kim MD, PhD Department of Cardiology, Kanagawa Children's Medical Center, Yokohama, JapanSearch for more papers by this authorHiroaki Murakami MD, PhD, Hiroaki Murakami MD, PhD orcid.org/0000-0002-6646-6263 Department of Medical Genetics, Kanagawa Children's Medical Center, Yokohama, JapanSearch for more papers by this authorKenji Kurosawa MD, PhD, Kenji Kurosawa MD, PhD orcid.org/0000-0002-6817-7748 Department of Medical Genetics, Kanagawa Children's Medical Center, Yokohama, JapanSearch for more papers by this authorKiyoshi Matsui MD, Kiyoshi Matsui MD Department of General Medicine, Kanagawa Children's Medical Center, Yokohama, JapanSearch for more papers by this author Satomi Mori MD, Corresponding Author Satomi Mori MD satomiforest@gmail.com orcid.org/0000-0002-8026-6954 Department of General Medicine, Kanagawa Children's Medical Center, Yokohama, Japan Correspondence Satomi Mori, MD, Department of general medicine, Kanagawa Children's Medical Center, Mutsukawa 2-138-4, Minami-ku, Yokohama, Kanagawa 232-8555, Japan. Email: satomiforest@gmail.comSearch for more papers by this authorKoji Tanoue MD, Koji Tanoue MD orcid.org/0000-0003-3295-5306 Department of General Medicine, Kanagawa Children's Medical Center, Yokohama, JapanSearch for more papers by this authorHiroyuki Shimizu MD, Hiroyuki Shimizu MD Department of Critical Care Medicine, Kanagawa Children's Medical Center, Yokohama, JapanSearch for more papers by this authorHiroyuki Nagafuchi MD, Hiroyuki Nagafuchi MD Department of Critical Care Medicine, Kanagawa Children's Medical Center, Yokohama, JapanSearch for more papers by this authorKi-Sung Kim MD, PhD, Ki-Sung Kim MD, PhD Department of Cardiology, Kanagawa Children's Medical Center, Yokohama, JapanSearch for more papers by this authorHiroaki Murakami MD, PhD, Hiroaki Murakami MD, PhD orcid.org/0000-0002-6646-6263 Department of Medical Genetics, Kanagawa Children's Medical Center, Yokohama, JapanSearch for more papers by this authorKenji Kurosawa MD, PhD, Kenji Kurosawa MD, PhD orcid.org/0000-0002-6817-7748 Department of Medical Genetics, Kanagawa Children's Medical Center, Yokohama, JapanSearch for more papers by this authorKiyoshi Matsui MD, Kiyoshi Matsui MD Department of General Medicine, Kanagawa Children's Medical Center, Yokohama, JapanSearch for more papers by this author First published: 26 January 2021 https://doi.org/10.1002/ppul.25269Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article. Volume56, Issue5May 2021Pages 1280-1282 RelatedInformation

Factors influencing platelet normalization of transient abnormal myelopoiesis.

Abnormal blood cell counts are characteristic of patients with Down syndrome and transient abnormal myelopoiesis (TAM). Although some patients with TAM experience prolonged anemia or thrombocytopenia, hematological factors predicting blood cell count recovery have not been reported yet. The aim of this study was to investigate the factors influencing platelet normalization in TAM. A retrospective review of the medical records of 21 patients with TAM admitted to the neonatal intensive care unit at Kanagawa Children's Medical Center between January 2007 and October 2014 was undertaken. In the 16 of 21 patients (76%) experiencing transient thrombocytopenia, a large number of blasts at diagnosis was found to be significantly associated with late platelet recovery (R = 0.669, P < 0.05), and higher platelet counts at diagnosis were significantly associated with later recovery (R = 0.719, P < 0.01). Indeed, a strong positive correlation between blast and platelet counts at diagnosis was found (R = 0.730, P < 0.01). Our data suggest that high platelet counts at TAM diagnosis might reflect abnormal thrombocyte production from blasts. Thus, physicians should be aware of the possibility of prolonged thrombocytopenia in patients with TAM who exhibit a high platelet and/or blast count at diagnosis.

Hypofractionated radiotherapy in children with diffuse intrinsic pontine glioma.

BackgroundOverall survival (OS) of patients with diffuse intrinsic pontine glioma (DIPG) is poor, with radiation therapy (RT) the only intervention that transiently delays tumor progression. Hypofractionated RT and re‐irradiation at first progression have gained popularity in improving the quality of life of such patients.MethodsWe performed a retrospective review of children with DIPG treated at Kanagawa Children's Medical Center from 2000 to 2018.ResultsA total of 24 cases were reviewed. Median age at diagnosis was 6.3 years (1.6–14.0). Twenty patients received RT only once. Thirteen patients received conventionally fractionated RT, and seven patients received hypofractionated RT as up‐front RT. Severe toxicities were not observed in patients who received hypofractionated RT. Median OS and time to progression were similar between conventionally fractionated and hypofractionated RT groups.(9.7 [95% confidence interval(CI): 7.1‐11.2] versus 11.0[95% CI: 5.2‐13.6] months, P = 0.60; 4.2[95% CI: 1.8‐8.3] versus 7.1 [95% CI:4.5‐8.7] months, P = 0.38). Four patients received re‐irradiation at first progression and all patients showed transient neurological improvement and survival more than a year after diagnosis. A 4‐year‐old boy was re‐irradiated 5‐and‐a‐half months after the first re‐irradiation; following transient neurological improvement. He survived a further 5 months.ConclusionHypofractionated RT for children with newly diagnosed DIPG is well tolerated and feasible from the viewpoint of reducing a patient's burden of treatment. Re‐irradiation at first progression is suggested to be beneficial.

Evaluation of Congenital Maxillary Growth Using Computed Tomography in Patients With Bilateral Cleft Lip and Palate.

To analyze congenital lateral maxillary growth for patients with bilateral cleft lip and palate (BCLP). A retrospective study. Kanagawa Children's Medical Center. Images from computed tomography (CT) of patients with BCLP and control patients that were previously used for treatment. The following landmarks were used: A, the posterior most point of the piriform aperture; B, the superior most point of the acoustic meatus; C, the point at which line A-B intersects the line drawn perpendicular from line A-B to the maxillary tuberosity; and D, the apical most point of the nasal bone. The following distances were then measured using these landmarks: (1) A-B distance; (2) A-C distance; (3) A-C/A-B; (4) the angle between lines A-B and A-D (∠BAD); and (5) B-D distance. Mean A-B and A-C distances and A-C/A-B were significantly smaller in the BCLP group than in the control group (P < .01 each). Mean ∠BAD was significantly larger in the BCLP group than in the control group (P < .01). Mean B-D distance did not differ significantly between groups. Our results indicated that the lateral maxillary segments of patients with BCLP were more posterior than those of the control group, and segment length was shorter compared to the control group on 3D-CT analysis. The lateral maxillary segments of patients with BCLP were basically suggested to originally be underdeveloped.

Prepubertal testicular tumors: a single-center experience of 44 years

PurposeTo present the clinical and histological features of prepubertal testicular tumors (PTTs), the long-term experience of a single institution was reviewed. Materials and methodsA total of 62 prepubertal children who were treated for testicular tumors at Kanagawa Children's Medical Center from 1971 to 2014 were retrospectively reviewed. Histopathological findings, age at operation, clinical stage, and outcomes were analyzed. Clinical findings between the two eras, 1971–1990 and 1991–2014, were also compared. ResultThe median age at operation was 17months. Pathology revealed 29 teratomas (47%), 26 yolk sac tumors (42%), 5 epidermoid cysts (8%), 1 Sertoli cell tumor (1.5%), and 1 benign cyst (1.5%). Teratoma was the most common tumor in this series, and children with immature teratomas were operated at a significantly younger age than those with mature teratomas. Yolk sac tumor was the second most common. The clinical stages of yolk sac tumors were stage I in 23 (89%) and stage II in 3 (11%). Clinical findings were not significantly different between the early and late eras. ConclusionsTo the best of our knowledge, this is the largest single-center study of PTTs in Japan. The most common PTT in this study was teratoma, followed by yolk sac tumor. There was no significant difference in the histological distribution of PTTs between the two eras. Compared with the current data of single-center series in North America, the incidence of yolk sac tumor was markedly higher in the present study. This discrepancy is possibly explained by racial differences.

Differences between abusive head trauma and physical abuse in Japan.

We examined differences in characteristics between abusive head trauma (AHT) and physical abuse (PA). We examined 45 AHT patients and 119 PA patients during the period 1 April 2000-31 March 2014, at Kanagawa Children's Medical Center (Kanagawa, Japan). We compared patient age, young maternal or parental age, being the first child, child factors (chronic illness, developmental disabilities and preterm birth), parental factors (substance abuse/alcohol abuse and depression or other mental illness), environmental factors (single-parent home, non-biologically related man living in the home and family or intimate partner violence). Logistic regression was used to examine predictors of differences between AHT and PA for children <1 year of age. There were differences in characteristics between AHT and PA in those <1 year old in child factors (OR, 3.47; 95%CI: 1.01-11.9) and environmental factors (OR, 14.2; 95%CI: 1.45-139). The risk factors for AHT differ from those of PA. We speculate that AHT is not merely physical abuse of young infants; the characteristics of AHT differ significantly from those of PA. These observations are important for understanding and thereby preventing child abuse.

Mediastinoscopic extended thymectomy for pediatric patients with myasthenia gravis

BackgroundExtended thymectomy is indicated for children with myasthenia gravis (MG) when drug-resistance or dependence is seen. We have employed a technique for mediastinoscopic extended thymectomy (MET) on children with MG. MethodA total of 14 children underwent MET at Kanagawa Children's Medical Center between 2005 and 2013. A mediastinal operation field was made by a V-shaped hook infrasternally to extirpate the thymus with adipose tissue around the thymus. ResultsThe operation time and the amount of blood loss were 182±44minutes and 34±43ml, respectively. Postoperative complications, in the form of transient paralysis of the right recurrent nerve, occurred in 2 patients. The median length of postoperative hospital stay was 4.5days. After MET, 6 patients achieved complete remission and 7 patients achieved steroid dose reduction, but no improvement was seen in 1 patient. ConclusionsThis procedure offers the advantage of good surgical access for dissection around the bilateral phrenic nerves in extended total thymectomy, while achieving good cosmetic results.

Prenatal Management of the Fetus with Lethal Malformation: From a Study of Oligohydramnios Sequence

47 cases of oligohydramnios sequence (OS) diagnosed at Kanagawa Children's Medical Center from 1992 to 2008 were studied retrospectively. Early termination of pregnancy was chosen in 9 cases, observed natural labor was chosen in 23 cases, and 18 cases resulted in natural deliveries. Fetal demise occurred during labor in 44.4% of terminated cases, while it occurred in 5.6% of observed cases. Preterm labors and breech presentations occurred most frequently. Most infants died within 18 hours after their births. There were 3 familial cases. These results provide important information for planning the perinatal care when fetuses are diagnosed with OS.

Response to Letter to the Editor Entitled Comments on Comparison of Tracheal Intubation Using Airway Scope and Macintosh Laryngoscope in Patients With Simulted Limitation of Neck Movements

Department of Anesthesia, Fujisawa Shounandai Hospital, Fujisawa, Kanagawa, Japan (Aoi) Department of Anesthesia, Kanagawa Children's Medical Center, Yokohama, Kanagawa, Japan (Inagawa) Department of Anesthesiology and Critical Care Medicine, Graduate School of Medicine, Yokohama City University, Yokohama, Kanagawa, Japan (Goto)

Long‐term Outcome of Children With Biliary Atresia Who Were Not Transplanted After the Kasai Operation: &gt;20‐year Experience at a Children's Hospital

Sequential strategies combining the Kasai operation as a first-line treatment and liver transplantation as a second-line option, if necessary, have been accepted for patients with biliary atresia (BA). To understand the role of the Kasai operation in the treatment of BA, it is necessary to analyze the long-term outcome of the operation alone and to evaluate the present status of survivors retaining their native livers. A retrospective chart review was carried out for a group of 80 patients who had undergone the Kasai operation between 1970 and 1986 at the Kanagawa Children's Medical Center. The 5-, 10-, and 20-year survival rates of patients with their native livers were 63%, 54%, and 44%, respectively. The survival rates varied significantly depending on the type of BA, age at initial Kasai operation, era of surgery, and surgical method. By age 20, nearly half of the adult survivors had already developed liver cirrhosis and its sequelae. Episodes of cholangitis and gastrointestinal bleeding occurred after 20 years of age in 37% and 17% of the adult patients, respectively, and 20% of the adult patients died of liver failure or underwent living-related partial liver transplantation in their 20s. Five female patients gave birth to a total of 9 children, and 1 male patient fathered a child. Although increasing numbers of patients with BA survive 20 years or more after the Kasai operation, meticulous lifelong postoperative care should be continued for the survivors because of the possibility of hepatic deterioration.

Twelve-month-old girl with myocardial ischaemia

A 12-month-old girl was admitted to Kanagawa Children's Medical Center because of the diagnosis of anomalous origin of the right coronary artery from the main pulmonary artery (ARCAPA). The electrocardiogram (ECG) recorded non-significant findings at rest but the ST depression from V1 to V3 at her crying. On the coronary angiography, it looked like a single coronary artery from the left coronary sinus; …

Free Communications: Genetics

Abstract *SumimasaYamashita, *HirokoIwamoto, *MichikoYamada, *MizueIai, *KaoriMasuko, *KazuyoWakabayashi, and †KenjiSugai *Kanagawa Children's Medical Center, Division of Child Neurology, Yokohama ; and †National Center of Neurology and Psychiatry, Musashi Hospital, Division of Child Neurology, Kodaira, Japan. Purpose: We encountered four cases of early myoclonic encephalopathy, consisting of two sets of siblings in two families. In family S, the elder sister is 7 years old now, severely handicapped and demonstrating both uncontrolled myoclonic and short tonic seizures as well as severe hepatic cirrhosis. The younger brother died at age 9 months. In another family O, the elder sister died at age 1 year, and the younger sister died at age 3 months. Each pregnancy was normal, and the deliveries were term, and all infants showed normal birth weight and normal head circumference. None of the infants was severely asphyxiated. Methods: The clinical record of these four cases revealed that the first seizure commenced within 1.5 months after birth, and seizure types were tonic spasms and myoclonic and clonic seizures. A suppression burst pattern was found in all cases at the beginning and then changed to focal or multifocal abnormalities thereafter. None of the cases showed hypsarrhythmia on EEG. In family S, both cases had minor anomalies such as characteristic facial appearances and finger overlapping. Results: Brain MRI showed severe brain atrophy in all cases but none of the cases showed cortical dysgenesis. In family O, the elder sister showed progressive cerebral and cerebellar atrophy, and the younger sister showed myelinating arrest of the cerebral white matter. Developmental milestones were arrested since the neonatal period in four cases. All cases had severe microcephalus, spastic tetraplegia, and severe mental retardation. Laboratory examination revealed no abnormal findings including CBC, chemistry, lactate, pyruvate, amino acids analysis, organic acids analysis, lysosomal enzymes assay using white blood cells, cerebrospinal fluid examination, and urinalysis. Chromosomal study was normal in all cases. Anticonvulsants such as oral high-dose phenobarbital, intravenous phenytoin for focal onset seizures, and zonisamide for apneic attacks were all effective to a moderate extent in some cases but did not reach complete seizure control. Autopsies were performed in two cases. Severe gliosis of cerebral white matter and marked dendritic expansions of cerebellar Purkinje cells were seen in both cases. No storage materials of neuronal and glial cells or laminar structural abnormalities of the cerebral cortex were found. Conclusions: It is important to differentiate from early infantile epileptic encephalopathy, which is characterized by severe asphyxia, brain anomaly, or cortical dysgenesis as a cause, tonic spasm in the seizure pattern of the onset, and alteration to West syndrome. Our cases did not fulfill these points. The main type of seizure was myoclonic or erratic myoclonia. Early myoclonic encephalopathy (EME) is known in cases of nonketotic hyperglycinemia or other inherited metabolic disorders as a cause of the disease. We could not detect metabolic abnormalities in the cases described. However, EME was suspected to have developed because of some genetic factors that might have influenced the development of the infantile nervous system.

Maternal, neonatal, and placental features associated with diffuse chorioamniotic hemosiderosis, with special reference to neonatal morbidity and mortality.

Our purpose was to examine the significance of diffuse chorioamniotic hemosiderosis (DCH) on neonatal morbidity and mortality. Using data from a retrospective case-control study, we analyzed 46 singleton placentas with DCH from infants who were delivered and/or admitted to the neonatal intensive care unit of Kanagawa Children's Medical Center during 1987-2001 and 92 control placentas without DCH from infants of comparable gestational age, birth weight, and duration. Mean and standard deviation of gestational age and infants' birth weight at delivery from the DCH group were 27 +/- 3 weeks and 939 +/- 342 g, respectively. Macroscopically, the placentas with DCH were more likely to show old peripheral blood clots (46% in the DCH group vs 8% in control group), subchorionic hematoma (20% vs 1%), and circumvallation (13% vs 1%). Histologically, amniotic necrosis was significantly more frequent in the DCH group (63% vs 24%). Of the obstetric factors, incidence of recurrent episodes of vaginal bleeding (70% vs 11%), oligohydramnios (59% vs 8%), and chronic abruption-oligohydramnios sequence (57% vs 5%) were significantly higher in the DCH group. Of the neonatal factors, persistent pulmonary hypertension of the newborn (29% vs 8%) and dry lung and/or pulmonary hypoplasia (20% vs 4%) were more common. However, respiratory distress syndrome was rare (15% vs 45%) in the DCH group. Neonatal death including stillbirth was increased in the DCH group but was not significant (24% vs 15%). Of infants who survived beyond day 28, chronic lung disease (CLD) was more frequent in the DCH group (51% vs 22%). The association of DCH, especially accompanied by amniotic necrosis, with CLD was still evident using likelihood ratio test. DCH is closely associated with preterm delivery, pulmonary hypertension of the newborn, and dry lung syndrome and is a significant risk factor for CLD.

「小児の脳腫瘍」- 神奈川県立こども医療センター 20年間(1982-2002)の症例の検討

「小児の脳腫瘍」- 神奈川県立こども医療センター 20年間(1982-2002)の症例の検討

Prophylactic cranial irradiation of acute lymphoblastic leukemia in childhood: outcomes of late effects on pituitary function and growth in long-term survivors.

It is well known that prophylactic cranial irradiation is highly effective in preventing central nervous system (CNS) relapse of acute lymphoblastic leukemia (ALL). Nevertheless, there have been very few reports on the late effects, especially pituitary function and growth, in long-term survivors who were treated with 18 Gy cranial irradiation in childhood. The subjects consisted of 35 children with ALL who were treated with prophylactic 18 Gy cranial irradiation at Kanagawa Children's Medical Center between October 1981 and February 1995. All patients received cranial irradiation after first attaining complete remission with induction chemotherapy, according to the treatment protocols prescribed by the Tokyo Children's Leukemia Study Group (TCLSG) and Tokyo Children's Cancer Study Group (TCCSG). Their ages at the time of cranial irradiation ranged from 2.2-15.0 years (mean 6.8). We evaluated their pituitary functions by measuring their pituitary hormone values 0.7-11.3 years (mean 6.0) after cranial irradiation and their growth by analyzing their height standard deviation score (SDS) at diagnosis of ALL and their final height SDS at the mean follow-up period of 8.2 years after cranial irradiation. Height SDS is defined as the difference between the patient's height and the mean height of their age and sex, divided by the standard deviation of their age and sex. Eight of 35 patients had ALL relapse, involving the bone marrow in seven patients and the CNS in only one. Six of the eight patients with relapse died 1.5-6.6 years after cranial irradiation and the other two patients were salvaged by further intensive therapies. The remaining 27 relapse-free patients have survived for 1.4-15.8 years (mean 10.1) after cranial irradiation. Twenty-six of the 29 survivors are long-term survivors of more than 5 years. Although there was one patient with an abnormal result in each value of growth hormone (GH), adrenocorticotropic hormone (ACTH), and prolactin (PRL), and two patients with abnormal results in thyroid-stimulating hormone (TSH) values, none of the patients had clinical symptoms of pituitary hormone abnormality and none required hormone supplements. The final height SDS decreased significantly compared with the height SDS at diagnosis of ALL in the long-term survivors (P = 0.001) and the height SDS of the patients who had received cranial irradiation at a young age tended to decrease gradually (P = 0.019). However, no patient showed severe growth failure. It is considered that prophylactic 18 Gy cranial irradiation plus chemotherapy for ALL in childhood can effectively prevent CNS relapse and is unlikely to produce clinically significant late effects, although it may cause slight pituitary hormone abnormality.

Large intracranial vessel occlusive vasculopathy after radiation therapy in children: Clinical features and usefulness of magnetic resonance imaging

Purpose : To assess the relationship between large intracranial vessel occlusive vasculopathy (vasculopathy) and radiation therapy, and to clarify the clinical efficacy of magnetic resonance (MR) imaging in the diagnosis and screening of the vasculopathy. Methods and Materials : We retrospectively evaluated the medical records and Serial MR images for 32 pediatric patients, in whom radiation therapy had been given to fields including the circle of Willis and major cerebral arteries. All children had periodically undergone follow-up neurologic assessment and MR imaging examination at Kanagawa Children's Medical Center for more than one year after radiation therapy (range 1.3–1.14 years). Patients who had no remained free off tumor progression up to the time of final evaluation were excluded. Results : Vasculopathy developed in t of 32 patients 2–13 years after radiation therapy. Three of them presented with transient ischemic attacks (TIA) and the other three shoed infarctions without preceding TIA. Steno-occlusive changes of major cerebral arteries were identified by MR imaging in all six patients, but not obtained in the remaining 26 patients. In the patients with tIA, MR imaging demonstrated steno-occlusive changes at the time of TIA, before irreversible infarction. They have been doing well subsequent to encephaloduroarteriosynnangiosis. In the three patients who presented infarction without preceeding TIA, MR imaging did not demonstrate the vascular change before the onsent of infarction, and two of them developed neurologic deficits. the mean exposure dose for the circle of Willis and major cerebral arteries in these six patients was significnatly higher than that in the remaining 26 patients without sequela (61Gy vs 50Gy, p < 0.05). The mean age at radaition therapy of the six patients was lower, but the difference was not significant. Conclusion : The incidence of vasculopathy after radiation therapy has a considerable correlation with radaition dose and age at radiation therapy. MR examination is useful for the diagnostic evaluation of vasculopathy, and it is also effective in screening for vasculopathy in patients with tIA, and may be helpful in the prevention of neurologic sequela

An infant having Potter facies without pulmonary hypoplasia after prolonged oligohydramnios due to a placental mass

Sir: We have read with interest the article by Hoekstra and Boer [1] and would like to present a similar case with a different cause of oligohydramnios. A 23-yearold, gravida 2, para 1 woman was seen at a hospital because of threatened abortion at 7 weeks gestation. At 10 weeks gestation, ultrasonography revealed a high-echo mass in the placenta. Follow up ultrasonography at 14 weeks gestation revealed that the mass had enlarged to equal the fetal size and was accompanied by oligohydramnios. Fetal movements were reported to be decreased, though fetal breathing movements were not reported. When the woman was admitted to hospital because of massive genital bleeding at 28 weeks gestation, ultrasonography failed to demonstrate the placental mass or the amniotic fluid space. She underwent caesarean section at 29 weeks gestation for suspected amniotic infection 4 days after rupture of the membranes. A female baby weighing 1125g was delivered with Apgar scores of 1 and 5 at 1 and 5 min, respectively. Examination of the placenta (150g) revealed abscence of amniotic membranes, as well as a 100g haematoma, the histological examination of which suggested a subchorionic haematoma. Amnion nodosum or inflammatory change was not seen. The infant was intubated and transfered to NICU of Kanagawa Children's Medical Center. On admission, deformities with Potter facies, right equinovarus, left valgus, and bell-shaped thorax were noted. Chest X-ray films showed clear lung fields without signs of pulmonary hypoplasia. Initial respiratory settings were very severe, FiOa of 1.0 and mean airway pressure of 20 cmH20 which improved to 0.5 and 10, respectively, 2h later. She was exubated at 69h after birth. Urination started 3 h after birth, renal ultrasonography was normal. At age 2.5 years she was mentally and physically normal and the deformities of the feet had been successfully corrected. McInthosh [2] reported four cases of "dry lung syndrome" after oligohydramnios due to prolonged rupture of the membranes. In three of them onset of membranes' rupture was as late as 31, 24, or 21 weeks gestation. In all cases including our own clinical course was benign and quite similar to that reported by Hoekstra and Boer; i.e., initial asphyxia and severe cyanosis which rapidly improved. Right-to-left shunt via the ductus arteriosus was suspected in Hoekstra and Boer 's case. We think that a small number of infants delevered after prolonged oligohydroamnios do not have lethal pulmonary hypoplasia, although changes in their external features follow those of the Potter sequence. Initial severe cyanosis seems to be due to persistent pulmonary hypertension, as all of the infants had ashyxia and cardiorespiratory failure which rapidly improved. Neonatologists should not relinquish resuscitating these babies, because their prognosis is good if they are skillfully treated for asphyxia and for subsequent transient pulmonary hypertension.

Two Cases of Idiopathic Vitamin K Deficiency in Infancy who Failed to be Prevented

Two cases of Idiopathic vitamin K deficiency in infancy we experienced recently are reported. The first case was admitted to Kanagawa Children's Medical Center at 62 days of age, because of intracranial hemorrhage due to vitamin K (VK) deficiency. She fell ill in spite of prophylactic administration of oral VK at her birth and 6 days after birth. She exhibited hyperbilirubinemia and elevation of serum transaminase. It was suspected that Cytomegalovirus (CMV) infection was related to her liver dysfunction, because IgM antibody to CMV was increased and CMV was isolated from her urine and breast milk of her mother. The second case was admitted at 31 days of age, and made a diagnosis of subdural hemorrhage and hemothorax due to VK deficiency. He had not received VK administration prophylacticaly, because his Normotest was normal at 6 days of age. He exhibited slight elevation of serum transaminase on admission. However CMV infection could not be elucidated in this case. We think that liver dysfunction plays important role in the onset of VK deficiency in infancy.The prophylactic administration of VK during the neonatal period has been carried out widely in Japan, and a remarkable decrease of intracranial hemorrhage due to Idiopathic VK deficiency in infancy has been reported. It is important to study the cases that failed to be prevented, for making a plan to prevent VK deficiency in infancy furthermore.

Allergy: Bronchial Sensitivity and Course of Asthma in Children

AbstractWe conducted the study on factors affecsting progonsis of bronchial asthama, clinical course and bronchial sensitivity to acetycholine 127 children with asthma, who received acetylcholine inhalation test and were subjected to observation for at least 3 years in Kanagawa children's Medical Center. First, the children were divided into two groups on the basis of asthmatic attacks. Those without asthamatic attacks for over 2 years were classified into the remission group and those with recent asthamtic episodes, the non‐remission group. A further calssification was based on bronchial sensitivity of acetylcholine. The two groups derived were the hypersensitivity group and the low sensitivity group.The following conclusion were drawn from the long‐term onservation of the patients. 1) One‐rank change in acetylcholine threshold does not correspond to a change in bronchial sensitivity. 2) Imporovement in brochial sensittivity to acetylcholine accompanies the disapperance of asthamtic attacks. 3) Bronchial hypersensitvity to acetylcholine is maintained by the presence of asthmatic attacks. 4) Significant change in brochial sensitivity to acetylcholine does not appear within 2 years of the presence or absence of asthmatic episodes.