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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.121, No.5, May 2017
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Original Article
Title
Two Cases of Patients with Mycoplasma Pneumonia Complicated by Kawasaki Disease
Author
Sayo Takenaka Koichi Nihei Chieko Nemoto Noriko Mishima Misako Suzuki Hiroaki Nakamura Kiyoto Nasuno Junko Fujiwara and Takashi Sekine
Toho University Ohashi Medical Center
Abstract
We experienced two cases of patients complicated by Kawasaki disease (KD) while undergoing treatment for mycoplasma pneumonia (MP). These patients were difficult to diagnose because of no alleviation in fever and fulfilment of the KD criteria after 10 days of illness. To date, KD related to MP has been reported in approximately 30 patients in Japan. In these patients, we examined the time for the appearance of KD-like symptoms and noted two patterns. The first pattern involved patients producing KD-like symptoms from the first incidence of fever (early group). The second pattern was similar to that of our group, where patients gradually produced KD-like symptoms after long-term fever (late group). In the early group, CRP values were significantly high since the early course of the disease. On the other hand, LDH values on admission were significantly higher in the late group than in the early group. In addition, no significant difference was found in coronary artery abnormalities (CAAs) between the groups. However, a high frequency of CAAs was noted in approximately 28% of all patients. Currently, the macrolide resistance rate of MP is 30%-50% in Japan. Patients with long-term fevers or other serious conditions are recommended to be treated by steroid therapy. From the perspective of acute KD treatment, steroid therapy may facilitate CAA formation. For this reason, we believe that it is important to carefully distinguish between patients with KD and those suffering from MP with long-term fever or other serious conditions.
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Original Article
Title
A Questionnaire on End-of-life Care in Institutions for Severe Motor and Intellectual Disorders
Author
Masahisa Funato1) Kiyoshi Takemoto1) Yoshitaka Iijima1) Hiroshi Wada1) Masao Kumode2) and Takashi Miyanomae3)
1)Department of Pediatrics, Osaka Developmental Rehabilitation Center
2)Department of Pediatrics, Biwako Medical & Welfare Center
3)Department of Pediatrics, National Hospital Organization, Minami Kyoto Hospital
Abstract
Aim. As residents in institutions for severe motor & intellectual disorders (SMIDs) progress in aging and in severity, ethical dilemmas have become more critical concerning the extent to which medical invasive interventions should be given. Because of this problems, we developed a questionnaire regarding end-of-life care. This was sent to directors of these institutions in the Kinki area (private/public/national).
Subjects. The questionnaire was sent to 31 institutions. In addition to the basic data of each institution, issues concerning the ethical committee, palliative care team, experience in end-of-life care, ages and diseases of patients, proxy decision-making, special kinds of end-of-life care were asked.
Results. 18/31 institutions (58%) responded to the questionnaire. 15/18 (83%) had experiences with end-of-life care. In these institutions, the total number of patients who passed away was 69 (<6 years old: 11, >=60 years old: 21). In most cases a proxy decision was made through shared decision-making with family and the medical-care team (60%), or otherwise the decision focused on the family's wishes. The basic plan for special end-of-life care at the terminal stage was limitation of invasive interventions (87%), especially of active resuscitation and/or vasopressor drug. Other plans included the introduction of palliative care, the provision of a family room, encouraging family participation with patient care. After death, sending off the patient and family by the staff from the front entrance was practiced in 6 institutions. Ethical committees have been set up in 15 institutes (83%), and a palliative care team has been organized either on a regular or irregular schedule in 8 institutions (44%).
Conclusion. Further research might be necessary to treat them in respecting their dignity, including shared decision-making, palliative care and advance care planning made by multidisciplinary team in the institutes for SMIDs.
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Original Article
Title
Evaluation of Renal Function in Patients with Severe Intellectual Disabilities and/or Motor Disabilities
Author
Miwa Goto1) Emi Sawanobori2) Sayaka Ishii1) Itaru Kuroda1) Nobuyuki Katumata1) Kosuke Nakamura1) Yoshimi Kaga1) Yuko Kamiya1) Norihiko Uchida1) and Mikinori Hisatomi1)
1)Department of Pediatrics, National Hospital Organization Kofu National Hospital
2)Department of Pediatrics, University of Yamanashi Hospital
Abstract
Serum creatinine levels are abnormally low in patients with severe intellectual disabilities and/or motor disabilities because of loss of muscle mass. Thus, serum creatinine level is sometimes not appropriate for glomerular filtration rate estimation in these patients. Cystatin C is recommended for renal function evaluation in patients with emaciation or muscle loss by the 2012 Chronic Kidney Disease guidelines. However, this recommendation is not adhered to at all clinical facilities. We evaluated renal function using serum cystatin C level and kidney size on ultrasonography. Cystatin C levels did not differ between groups divided according to gross motor skills. The median serum creatinine levels were 0.35 mg/dl in the bedridden group, 0.36 mg/dl in the group capable of rolling over, 0.47 mg/dl in the group capable of assuming a seated position, and 0.55 mg/dl in the group capable of assuming a standing position; these levels were significantly lower in the bedridden group than in the seated position and standing position groups (p<0.05). The estimated glomerular filtration rate based on serum creatinine level was higher than that based on serum cystatin C level; in the bedridden group, about 50% of the patients showed a two-fold higher value. According to the estimated glomerular filtration rate based on cystatin C level, age-related changes in renal function in these patients were equivalent to those in normal subjects. Furthermore, kidney size correlated with height and weight in all ages; specifically in patients aged <18 years, the ratio of kidney size to height was almost equivalent to the standard value in healthy children. Serum cystatin C level measurement is useful to evaluate renal function in patients with severe intellectual disabilities and/or motor disabilities, and kidney size on abdominal ultrasonography should be evaluated based on height.
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Original Article
Title
Changes in Thyroid Function during the First 36 Months among Infants with Trisomy 21 Who Did Not Receive Thyroid Hormone Therapy
Author
Midori Yoda Tatsuhiko Urakami Nobuhiko Nagano Junichi Suzuki Kayo Yoshikawa Yukihiro Usukura Shigeharu Hosono and Shori Takahashi
Department of Pediatrics, Nihon University School of Medicine
Abstract
Thyroid dysfunction is the most common endocrine disorder that is associated with trisomy 21, although little is known regarding the changes in thyroid function during early infancy in cases of trisomy 21. Therefore, we investigated the changes in thyroid function during the first 36 months among 38 infants with trisomy 21 who did not receive thyroid hormone therapy. The infants were either average-for-gestational age (AGA: 29 infants) or small-for-gestational age (SGA: 9 infants). None of the infants exhibited low serum levels of direct free thyroxine (<1.0 ng/mL) during the study period. Thirty-four infants (89%) exhibited elevated serum thyroid-stimulating hormone levels (TSH: >5 μU/mL) during the first post-natal week. Among the 9 patients who completed the 36-month study, 5 patients (56%) continued to have elevated TSH levels. Significantly higher TSH levels were observed among AGA infants during the first 3 months, compared to SGA infants, although this difference disappeared over time. There were no other significant differences in the patients' TSH levels according to the various patient characteristics (e.g., sex, birth weight, gestational age, and complications). These results suggest that transient hyperthyrotropinemia may be common among infants with trisomy 21, and that some infants continue to exhibit elevated TSH levels despite having normal direct free thyroxine levels.
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Original Article
Title
Analysis of Clinical Symptoms Related to Influenza Encephalopathy
Author
Ikuo Tamai1)3) Shumpei Yokota1)2) Tomohide Goto3) Yoshihiro Watanabe4) Takahito Wada5) and Hitoshi Osaka3)6)
1)Department of Pediatrics, Yokohama City University Graduate School of Medicine
2)Atami Hospital, International University of Health and Welfare
3)Division of Neurology, Kanagawa Children's Medical Center
4)Department of Pediatrics, Yokohama City University Medical Center
5)Department of Medical Ethics and Medical Genetics, Kyoto University Graduate School of Medicine
6)Department of Pediatrics, Jichi Medical University
Abstract
We examined if early clinical symptoms, especially mild disturbances of consciousness, may predict the underlining encephalopathy or its progression in patients with influenza infection. Analyses were performed regarding the age, maximum body temperature, convulsions, muscle weakness, disturbed peripheral circulation of the extremities, pain, shivering, and delirium.
The items were compared between an encephalopathy group, which consisted of 111 patients who suffered from the neurological sequelae of or died from acute influenza encephalopathy, and a non-encephalopathy group, which consisted of 2,245 patients who exhibited neurologic symptoms during influenza infection. Significant differences (p<0.05) were observed for age, maximum body temperature, convulsions, muscle weakness, and disturbed peripheral circulation of the extremities. In addition, the encephalopathy group exhibited a higher rate of convulsions and severe disturbances of consciousness without delirium. Furthermore, there were patients with more profound delirium in the encephalopathy group than in the non-encephalopathy group. Our study indicated that the risk factors for acute encephalopathy are age of less than 3 years, high body temperature of 40°C or more, long duration of seizures, muscle weakness, and extreme peripheral coldness.
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Original Article
Title
Psychomotor Development in Low-birth-weight Infants Weighing between 1,500 Grams and 1,999 Grams during Early Infancy
Author
Keiko Hirabaru Yukari Suyama Kumiko Jin-nouchi Humiya Miyamura Masakazu Egashira Hiroharu Inoue Akiko Funakoshi Tomoko Mizukami Tomoko Egashira and Toshimitsu Takayanagi
Department of Pediatrics, National Hospital Organization Saga National Hospital
Abstract
In order to evaluate the psychomotor development in low birth weight infants whose birthweight is from 1,500 to 1,999 grams, we retrospectively, investigated their developmental quotient (DQ) by using the check-list of the Enjoji Questionaire sheet. The study subjects were 164 newborns discharged uneventfully from our NICU, and visiting our hospital for regular medical check at the age between 12 and 19 months of old.
As a result, the total DQ was 98±14 and the percentage of DQ<85 and <70 was 15.9% and 0.6%, respectively, which seems to be within normal range. However, the intra-individual comparison of six sub-scale has demonstrated that the task of hand movement (fine motor) and especially, speech were significantly weak because the mean DQ and the percentage of DQ <85, <70 were 95±18 and 30%, 3% in hand movement, and 87±20 and 55%, 19% in speech, respectively. Among the perinatal factor including gender, gestational age, z-score of physical constitution and duration of mechanical ventilation, male gender was only the risk factor for total DQ<85 by using a logistic regression analysis. In conclusion, closed intervention may be required in some of LBWI. It is difficult to determine this target by perinatal information.
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Case Report
Title
An Extremely Low-birth-weight Infant Who Died of Neonatal Sepsis Caused by Streptococcus Gallolyticus Subsp. pasteurianus
Author
Kanako Higashi and Hiroshi Koga
Department of Pediatrics, National Hospital Organization Beppu Medical Center
Abstract
We encountered a case of an extremely low-birth-weight infant in whom sepsis caused by Streptococcus gallolyticus subsp. pasteurianus rapidly led to death. The patient was a boy had been born at a gestational age of 28 weeks and 2 days with a birth weight of 884 g. Sepsis developed with fever and tachycardia on postnatal day 11. The S. gallolyticus subsp. pasteurianus that was isolated and identified from arterial blood culture exhibited good antimicrobial susceptibility to ampicillin, but the patient did not respond to administration of the drug, and died 15 hours after onset. The route of infection was unclear. In an investigation of 15 reported cases of S. gallolyticus infection in neonates in Japan, the route of infection was unclear in all cases. These cases included two deaths, which both occurred in low-birth-weight infants. Meningitis was confirmed in 14 cases, and abnormal findings were observed on brain imaging in five of these cases. In three cases, the patient did not respond to administration of antimicrobials exhibiting good susceptibility. S. gallolyticus infection in neonates was thought to have the following clinical characteristics: 1) an unclear route of transmission, and 2) a high mortality in spite of its high proportion of late-onset neonatal sepsis.
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Case Report
Title
Congenital Dyserythropoietic Anemia (CDA) Type I Causing Severe Hypoglycemia in a Neonate
Author
Itaru Hayakawa1) Hidekazu Homma2) Yuya Saito3) Yuki Yuza3) Takashi Kaneko3) Jun Shindo2) Ryota Kakinuma2) and Masatoshi Kondo2)
1)Department of General Pediatrics, Tokyo Metropolitan Children's Medical Center
2)Department of Neonatology, Tokyo Metropolitan Children's Medical Center
3)Department of Hematology and Oncology, Tokyo Metropolitan Children's Medical Center
Abstract
Congenital dyserythropoietic anemia (CDA) type I is a rare group of inherited bone marrow disorders characterized by anemia with ineffective erythropoiesis. Neonatal CDA type I typically presents with hemolytic anemia, jaundice, hepatomegaly and persistent pulmonary hypertension of the newborn. Here we report the first case of CDA type I who presented with severe hypoglycemia, as well as these characteristic features. Increased recognition of CDA type I by neonatologists may facilitate the early diagnosis of patients with severe hemolytic anemia and early jaundice.
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Case Report
Title
A Case of Racemose Hemangioma of the Bronchial Artery Treated by Segmentectomy
Author
Eri Koshino Yasuhiro Ikawa Naohisa Fujita Yusuke Yachi Natsumi Inoue Akiko Kato Michiko Okajima and Akihiro Yachie
Department of Pediatrics, Kanazawa University Hospital
Abstract
Since hemoptysis is a rare chief complaint in children, the correct diagnosis is difficult. Here, we report a 12-yr old boy with sudden hemoptysis diagnosed with racemose hemangioma of the bronchial artery (RHBA) by angiography and who received segmentectomy for radical cure. He was admitted to our hospital due to three episodes of sudden hemoptysis. Contrast-enhanced chest computed tomography (CT) demonstrated a 2-mm sized, nodular, high-density area in the left upper lobe with an infiltrative shadow, suggesting that the nodular high-density area can be a microaneurysm as the source of bleeding. Angiography revealed irregular curvature, tortuosity and dilation of the left bronchial artery, leading to a diagnosis of RHBA. Since the bronchial artery was too narrow to perform curative bronchial artery embolization, the patient underwent anterior segmentectomy of the left upper lobe. No hemoptysis was observed after the operation.
In the present case, since vascular malformation was strongly suspected on contrast-enhanced chest CT, we chose to perform invasive bronchial artery angiography to make a diagnosis. When vascular malformation is strongly suspected, it appears to be necessary to proactively perform angiography. Moreover, in pediatric cases, minimally invasive bronchial artery embolization is often chosen as the first-line of therapy. However, since it is associated with a high rate of recurrence requiring re-embolization, partial lung resection should be chosen as an important option.
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