|
THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
|
Vol.123, No.6, June 2019
|
Original Article
Title
Clinical Characteristics of Patients with Kawasaki Disease with Marked Cervical Lymph Node Swelling and/or Pain
Author
Yoko Tabei1)2) Satoru Kumaki1) Tomomi Ogata2) Saeko Yamamura1) Yuu Katata1) Tomoyuki Isida1) Natsuko Kusaka1) Ryouichi Oonuma1) Rie Noguchi1) and Setsuko Kitaoka1)
1)Sendai Medical Center
2)Department of Pediatrics, Gunma University Graduate School of Medicine
Abstract
In patients with Kawasaki disease (KD) who present with marked cervical lymph node swelling, there are controversies over their responsiveness to intravenous immunoglobulin therapy (IVIG) and their association with coronary artery complications. In the present study, we examined 154 patients with KD who were admitted to Sendai Medical Center, Japan, from July 2011 to June 2013. The clinical features of 26 patients with marked cervical lymph node swelling and/or pain were significantly older in age and showed higher levels of white blood cell counts, neutrophil counts, C-reactive protein, and serum amyloid A, than those of the other 128 patients with KD. In addition, they also tended to require additional therapies and result in higher frequencies of acute coronary artery abnormalities. Furthermore, we performed multi-variable analysis in patients with KD who had a Gunma risk score of four points or lower, which were expected to have good intravenous immunoglobulin responsiveness. The result was that patients with marked cervical lymph node swelling and/or pain were shown to require more additional therapies than those without swelling or pain. In conclusion, marked cervical lymphadenopathy could be one of the risk factors for IVIG resistance and coronary artery abnormalities in KD patients.
|
|
Original Article
Title
The Impact of Implementation of a Rapid Response System (RRS) in a Children's Hospital in Japan
Author
Naoki Fujiwara1) Arata Sashinami1) and Yusuke Seino2)
1)Division of Pediatric Critical Care Medicine, Okinawa Prefectural Nanbu Medical Center & Children's Medical Center
2)Division of Pediatric Critical Care Medicine, Hyogo Prefectural Kobe Children's Hospital
Abstract
Background: Currently, rapid response systems (RRS) for children are still not fully recognized and implemented in Japan, and reports showing the real effects of RRS are very limited. Objective: The study was performed to evaluate the impact of implementation of RRS in a children's hospital. Methods: The RRS, which was introduced from January 2013 in our hospital, can be activated by all doctors and nurses. We prospectively recorded the clinical data after RRS introduction, and conducted a before-and-after study (3 years each) to compare the incidence of unexpected cardiac arrest in the general wards and all-cause hospital mortality. Results: There were 97 cases of RRS activation during the study period (10.2 cases per 1,000 hospital admissions). The incidence of unexpected cardiac arrest in the wards remained approximately unchanged, but hospital mortality rate was significantly reduced from 8.16 to 5.58 per 1,000 admissions [RR 0.68 (95% CI 0.48-0.98)]. Among patients who were unexpectedly admitted from the wards to the PICU, the frequency of emergency endotracheal intubation in the wards was significantly lower in the post-RRS period [17% vs. 8%; RR 0.48 (95% CI 0.24-0.95)]. Conclusions: An improvement in hospital mortality rate was achieved after pediatric RRS introduction, and it seems that RRS has a definite function as a medical safety net.
|
|
Case Report
Title
Clinical and Pathological Examination of Three Cases of Congenital Pulmonary Lymphangiectasia
Author
Yuji Fujita1)2) Tomoki Ishida1) Koh Sasaki1) Toru Inoue3) Yoshiteru Ohsone2) and Mika Tomita1)
1)Department of Neonatology, Kimitsu Chuo Hospital
2)Department of Pediatrics, Chiba University Graduate School of Medicine
3)Department of Pathology, Kimitsu Chuo Hospital
Abstract
Congenital pulmonary lymphangiectasia (CPL) has a poor prognosis because it causes dilatation of the lung alveoli, resulting in severe respiratory failure after birth.
Case 1 had primary CPL, and cases 2 and 3 had CPL secondary to pulmonary venous atresia and total anomalous pulmonary venous return. In case 1, excessive amniotic fluid was observed, resulting in the birth of the patent at 32 weeks of gestation due to fetal hydrops; however, cases 2 and 3 showed no abnormalities during fetal life and were delivered at full term. No difference was found in the clinical course after admission to the neonatal intensive care unit, and all patients died within 48 h of birth. No difference was observed in lung weight/body weight or radial alveolar count. The VEGF family is speculated to provide important signals in the development of lymphatic vessels. The VEGF family molecules may be involved in the onset of the disease; however, our examination showed no obvious differences in the immunostaining of anti-VEGF receptor 3 antibody.
It is necessary to analyze more cases to develop a screening method at the fetal stage to clarify the disease etiology and establish a treatment course.
|
|
Case Report
Title
A Case of Constrictive Pericarditis Caused by Parvovirus B19 Detected from the Pericardium of a Patient with Protein-losing Gastroenteropathy
Author
Kanako Kano1)2) Hiroshi Ono1) Takanori Suzuki1) Katsutoshi Nakano1) Ryo Mafune1) Taiyu Hayashi1) Nobutaka Shimizu1) Yasushi Misaki1) and Hitoshi Kato1)
1)Division of Cardiology, National Center for Child Health and Development
2)Division of Pediatrics, Tokyo Medical University Hospital
Abstract
A 35-month-old boy was found to have Parvovirus B19 infection and mitral valve regurgitation when he was being closely examined for protein-losing gastroenteropathy (PLE). A diagnosis of constrictive pericarditis (CP) was made after 2 years of observation, and Parvovirus B19 was detected by polymerase chain reaction from the intraoperatively resected pericardium. Patients with PLE should always be closely investigated for CP.
|
|
Case Report
Title
Two Male Patients with Long QT Syndrome Who Developed Spontaneous Shortening at Puberty
Author
Hiromu Yamada1) Hideki Motomura1)2) Mari Yokokawa1) Tomoyuki Hasuwa1) Mari Nakagaki1) Sumito Dateki1) Masao Yoshinaga3) Takeshi Aiba4) and Hiroyuki Moriuchi1)
1)Department of Pediatrics, Nagasaki University Hospital
2)Department of Pediatrics, National Hospital Nagasaki Medical Center
3)Department of Pediatrics, National Hospital Kagoshima Medical Center
4)Division of Arrhythmia, Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center
Abstract
Since serum levels of testosterone are associated with the QTc interval variation, the duration of the QTc interval in healthy boys shortens at the time of onset of puberty. Therefore, the frequency of syncopal attacks as a result of cardiac arrhythmia is expected to decrease in male patients with long QT syndrome (LQTS) type 1, 2 decreases during adolescence. We herein report two boys with LQTS who had recurrent syncopal attacks. We followed their clinical courses and electrocardiographic findings chronologically before and during adolescence. Shortening of the QTc interval and reduction of syncopal attacks became evident at 11 and 16 years of age, respectively, when they showed puberty-related growth spurts demonstrated by their growth charts. A growth chart can be created easily and is useful to assume onset of puberty by recognizing a growth spurt, especially for clinical management of sex hormone-dependent conditions like LQTS type 1, 2.
|
|
Case Report
Title
Cerebral artery infarction associated with heritable protein C deficiency
Author
Fumihito Nozaki1) Tomohiro Kumada1) Minoru Shibata1) Shouichi Ohga2) and Takashi Kusunoki1)
1)Department of Pediatrics, Shiga Medical Center for Children
2)Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University
Abstract
Heritable protein C (PC) deficiency is an inherited thrombophilia that causes thromboembolism. Accurate diagnosis of heritable PC deficiency is difficult during the neonatal period and early infancy, because the physiological plasma PC activity levels are lower than those in adults. Here, we present the case of a boy with cerebral infarction during the neonatal period who received a genetic diagnosis of PC deficiency at 8 years of age. He had no family history suggestive of a thrombophilic predisposition. He was born at 32 weeks of gestation, weighing 2,290 g at birth, and had no asphyxia. Magnetic resonance imaging of the brain, which is routinely performed for preterm infants at 28 days after birth, revealed cerebral infarction in the territory of the left middle cerebral artery. The plasma PC activity level (34%) was normal according to age-based standards. Spastic hemiplegic cerebral palsy was diagnosed in our hospital 7 months after birth. The cause of cerebral palsy was reevaluated when the patient was 8 years old. The PC antigen level was 70%, and the PC activity level was 48%, which indicated a diagnosis of heritable PC deficiency. The ratio of the PC activity to protein S activity was 0.364. Following genetic counselling, genetic studies identified a reported heterozygous mutation (c.1043G>A; p.Arg348Gln) in exon 9 of PROC. Heritable PC deficiency should be considered in patients with cerebral artery infarction during the perinatal or neonatal period.
|
|
Case Report
Title
Three cases of Kasabach-Merritt Phenomenon Treated with Steroid and Vincristine
Author
Kento Ikegawa1) Satoko Suzuki1) Yuya Saito2) Yuki Yuza2) and Hiroshi Hataya1)
1)Department of General Pediatrics, Tokyo Metropolitan Children's Medical Center
2)Department of Hematology and Oncology, Tokyo Metropolitan Children's Medical Center
Abstract
Vascular tumors with Kasabach-Merritt Phenomenon (KMP) can be life-threatening if left untreated. Historically, systemic corticosteroids have been recommended as first-line treatment for tumors with KMP, but many patients are unresponsive to the therapy. In recent years, vincristine has become the recommended, alternative first-line agent for treating vascular tumors with KMP. We performed a review of 3 pediatric patients with a vascular tumor with KMP treated with steroid and vincristine. All patients responded to the therapy in the acute period but experienced recurrence much later. Two patients received steroid and vincristine after each decline in the platelet count, and another patient received sirolimus, an mTOR inhibitor. All therapies were eventually discontinued in all patients, who have not suffered a recurrence for over 18 months. Our study found that KMP can be treated with steroid and vincristine in the acute period although recurrences can occur much later. Our findings further suggest that the KMP recurrence was due to the short steroid and vincristine treatment period. Sirolimus was also found to be effective against steroid and vincristine-resistant KMP; however, the treatment period for sirolimus has not yet been established. Further studies are needed to standardize the treatment period for steroid, vincristine, and sirolimus.
|
|
Case Report
Title
A Case of Tubulointerstitial Nephritis Caused by Multi-ingredient Cold Medication Effectively Treated with Mycophenolate Mofetil
Author
Momoko Kashiwado1) Emi Sawanobori1) Hiroaki Kanai1) Anna Kobayashi1) Miwa Goto1) Yumiko Mitsui2) Kazumasa Sato2) Kosuke Higashida1) and Kanji Sugita1)
1)Department of Pediatrics, University of Yamanashi Hospital
2)Department of Pediatrics, Kyonan Medical Center Fujikawa Hospital
Abstract
Drugs are one of the main causes of tubulointerstitial nephritis (TIN). The treatment of the steroid-resistant or steroid-dependent drug-induced TIN (DITIN) is not yet well established. We report a case of steroid-dependent DITIN that was treated successfully with mycophenolate mofetil (MMF). A 15-year-old boy presenting persistent fever and pyuria for a month resistant to antibiotics was admitted to our hospital. Several diagnostic interviews revealed ingestion of a multi-ingredient cold medication containing ibuprofen at the beginning of the clinical presentation of fever and general fatigue. His kidney function had deteriorated with an increase of low molecular weight proteinuria. The diagnosis of TIN was made on the basis of clinical findings obtained from contrast enhancement abdominal computed tomography, a gallium scintigraphy, and a renal biopsy. Orally administered prednisolone at a dose of 60 mg per day immediately relieved his symptoms, and kidney function normalized. However, TIN relapsed after prednisolone was switched to alternate day therapy. Combined treatment with MMF allowed dose reduction and finally discontinuation of oral prednisolone treatment. Moreover, symptomatic uveitis progressed three months after prednisolone withdrawal, at 23 months from TIN onset. It is suggested that hypersensitivity to several drugs demonstrated by drug lymphocyte stimulation tests was involved in both TIN and uveitis.
|
|
Case Report
Title
A Girl with Primary Ovarian Insufficiency Who Had Exhibited Some Symptoms of Precocious Puberty
Author
Hinako Yamamura Akiko Furuya Shigeru Suzuki Yusuke Tanahashi and Hiroshi Azuma
Department of Pediatrics, Asahikawa Medical University
Abstract
Patients with primary ovarian insufficiency (POI) usually lack pubertal development completely or partially. We report a rare female case who presented precocious puberty before she was given a diagnosis of POI.
The girl was born at 25 weeks gestation and her birth weight was 826 g. She had mild cerebral palsy and intellectual disability. She exhibited breast development and a growth spurt at 7 years of age, and pubic hair at 8 years of age. Her bone age was accelerated, but serum luteinizing hormone (LH) and follicle-stimulating hormone (FSH) levels showed prepubertal response by GnRH stimulation test. At 8 years and 7 months of age, she started LH-releasing hormone (LHRH) agonist treatments for central precocious puberty because her basal serum LH and FSH were elevated, though estradiol level was low. Her pubertal development did not progress during LHRH agonist treatments. She stopped the treatment at 12 years of age, but she had not presented menarche at 16 years of age. At that time, serum LH, FSH and estradiol levels were 46.66 mIU/mL, 88.54 mIU/mL and less than 5.0 pg/mL, respectively. Her karyotype was 46, XX. On MRI, her right ovary was not identified and her uterus was smaller than expected for her age.
Physicians should suspect POI when the laboratory test of a patient shows low estradiol levels and elevated serum gonadotropins, even if the patient has signs of precocious puberty.
|
|
Case Report
Title
Two Cases of Neonatal Escherichia Coli Ventriculitis
Author
Ayu Okuno1) Junko Yamanaka1) Yuri Yoshimoto1) Yoshiaki Okuma1) Masao Kaneshige1) Mizue Tanaka1) Hideko Uryu1) Ayumi Mizukami1) Keiji Goishi1) Noriko Sato1) Atsushi Sato2) and Hiroyuki Shichino1)
1)Department of Pediatrics, National Center for Global Health and Medicine
2)Department of Pediatrics, The University of Tokyo Hospital
Abstract
Neonatal Escherichia coli (E. coli) meningitis is more frequently complicated by ventriculitis than Group B Streptococcal meningitis. Early diagnosis of ventriculitis is difficult because of its unspecified clinical symptoms. Magnetic resonance imaging (MRI), especially diffusion-weighted MR imaging, has been reported as the key for diagnosing ventriculitis. Here we present 2 cases of neonatal E. coli meningitis complicated by ventriculitis. One patient suffered from persistent high fever and enlarged cranial circumference, despite receiving appropriate antibiotics. On the third day we promptly diagnosed the patient with ventriculitis via contrast-enhanced MRI. Another patient showed a positive response to antibiotic treatment. During the therapy we performed MRI which showed no signs of ventriculitis. However, meningitis recurred, and contrast-enhanced MRI showed signs of ventriculitis. From our experience it is important to assess images in various conditions, including contrast-enhanced MRI, because there might be cases in which it is difficult to detect the ventriculitis by identification of diffusion-weighted MRI ventriculitis in spite of previous studies. Even when E. coli meningitis is effectively treated, it is important to rule out the complications of ventriculitis via several imaging methods. Furthermore, there is not enough evidence to determine the ideal duration of antibiotic therapy for ventriculitis. Therefore, it is difficult to decide when to finish the antibiotic therapy. In our two cases, we determined the treatment plan comprehensively according to the clinical course, severity, blood examination, cerebrospinal fluid findings and imaging test. It is important to consider possible ventriculitis complications when determining a treatment strategy for E. coli meningitis.
|
|
Case Report
Title
Successful Treatment of a Child with Ethylene Glycol Poisoning Using Fomepizole and Hemodialysis
Author
Shokei Murakami1) Hiromichi Taneichi1) Tomomi Tanaka1) Shohei Kusabiraki1) Shinobu Shida2) Hidenori Yamazaki2) Tsutomu Koike2) Yuji Fujita3) and Yuichi Adachi1)
1)Department of Pediatrics, University of Toyama
2)Second Department of Internal Medicine, University of Toyama
3)Advanced Emergency and Critical Care Medical Center, Iwate Medical University
Abstract
Fomepizole, an alcohol dehydrogenase inhibitor, was approved in 2015 for treating ethylene glycol (EG) poisoning in Japan. However, there have been few cases of fomepizole use in the pediatric population in Japan. We report a 14-year-old boy with EG poisoning who was successfully treated with fomepizole and hemodialysis. After ingesting a potentially fatal amount of EG-containing antifreeze, he developed consciousness disturbance. At a local hospital, severe metabolic acidosis was detected. Sodium bicarbonate and vitamin B1 were administered intravenously and whisky was given via a nasogastric tube, and he was then transferred to our hospital. Hemodialysis was started after six hours and fomepizole was infused eight hours after ingestion. The metabolic acidosis and osmolal gap gradually improved. Hemodialysis was completed in 7 hours, and the second dose of fomepizole was then given. His consciousness improved the next day, and he was discharged on the 9th hospital day with no apparent complications. Fomepizole can be used even in cases of pediatric EG poisoning.
|
|
Brief Report
Title
Acute Status Changes during Short-stays in Hospices among Severely Disabled Children
Author
Yukiko Mizutani Hidenori Nakagawa Michiru Sasaki Satoshi Mizutani and Makoto Nabetani
Department of Pediatrics, Yodogawa Christian Hospital
Abstract
We retrospectively evaluated data of severely disabled pediatric patients who experienced acute changes during short-stays in our children's hospice center. Over a 5-year period, there were 387 short-stay service users, a total of 3,035 short-stays, and 107 acute changes in patient status requiring 62 hospital changes. There were no statistically significant differences between those who required respiratory support and those who did not, based on acute changes in patient status and hospital changes. Ultimately, we should closely monitor patients for changes in their condition, whether or not respiratory support is required. For patient safety, we evaluate each patient's condition before their short-stay admission and collaborate with the introducing hospital regarding hospital change.
|
|
|
Back number
|
|
