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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.123, No.11, November 2019
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Original Article
Title
Small for Dates at Birth Give a Significant Effect on Subsequent Body Build until Preschool Age in Very-Low-Birth-Weight Infants
Author
Akinori Shichijo Hiroyuki Tomino Masakazu Egashira Yuka Matsunaga Tomoko Egashira Tomoko Mizukami Yuri Ishida Hiroi Eguchi Chiaki Iida and Toshimitsu Takayanagi
Department of Pediatrics, National Hospital Organization, Saga Hospital
Abstract
Aim
This study aims to clarify the influence of body build at birth on subsequent physical growth in very-low-birth-weight (VLBW) infants.
Methods
The study enrolled 246 VLBW infants who were divided into three groups based on the percentile of body length (BL) and weight (BW) at birth: AFD (BW ≥10th percentile), LFD (BL ≥10th percentile; BW <10th percentile), and SFD (both BL and BW <10th percentile). The z-scores of BL and BW were obtained in each subject at term (40 weeks after gestation) and at chronological ages 1, 2, 3, and 6 years, and the z-score of body mass index (BMI) at chronological ages 1, 2, 3, and 6 years. This study compared the obtained z-scores in each period among the three groups and also evaluated the influence of sex difference.
Results
The z-scores of BL and BW in every group continued to increase until preschool, but the z-score of BMI increased from chronological age 1 to 3 years but decreased from chronological age 3 to 6 years. The z-scores of BL, BW, and BMI in SFD infants continued to be smaller than the other two groups throughout the observation period. This characteristic was more distinct for male infants than for female infants. On the other hand, the z-scores of BL, BW, and BMI in the LFD group were close to that of in the AFD group beyond the infantile period, regardless of sex difference.
Conclusion
The body build of the SFD group, especially male SFD, was stunting than that of the LFD and AFD groups until preschool age in VLBW infants.
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Original Article
Title
Increased Incidence of "fuzengata"-Kawasaki Disease Consequent to Increasing Awareness of the Availability of Diagnostic and Aggressive Management Approaches
Author
Yuichi Nomura1) Mayumi Yashiro2) Kiminori Masuda1) and Yoshikazu Nakamura2)
1)Department of Pediatrics, Kagoshima City Hospital
2)Department of Public Health, Jichi Medical University
Abstract
We investigated the reason behind the increasing number of patients diagnosed with "fuzengata"-Kawasaki disease (fKD).
Methods: The study subjects included patients diagnosed with fKD, as reported between the 20th and the 24th nationwide surveys for KD (from 2007 to 2016). We investigated the number of reported principal symptoms (P-Symptoms), the presence/absence of acute phase coronary artery abnormalities (aCAAs), and whether the subjects were treated with intravenous immunoglobulin (IVIG).
Results: In total, 94% of patients with fKD presented 3-4 P-Symptoms. The incidence of patients with fKD increased from 17% to 21%, and the proportion of IVIG-treated patients increased from 61% to 80%. The proportion of patients with fKD who were administered IVIG within the 4th day of illness also increased from 26% to 30%. Among patients with fKD treated with IVIG, the incidence of patients who developed aCAAs was only 7%.
Discussion: The majority of patients with fKD were diagnosed and treated without the presence of aCAAs. This finding suggested an early, comprehensive diagnostic approach to KD. In 2007, a high incidence of CAA in patients with KD with 1-4 P-Symptoms was reported. Since then, the awareness about the risk of CAA in such patients has been spreading. Disease management with early diagnosis and aggressive treatment approaches is now permitted, resulting in an increase in the identification of patients with fKD.
Conclusions: The increase in the incidence of fKD can be attributed to the increasing awareness about the availability of aggressive management approaches.
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Original Article
Title
A New Innovative Family Support by a Short-term Stay Facility: Present Situation and Issues
Author
Sato Suzuki and Masaya Kubota
Division of Neurology, National Center for Child Health and Development
Abstract
The number of disabled children who require medical support to live at home is increasing. Their life depends on the caregiver's support. Sustained and highly developed care squeezes the lives of caregivers. We conducted a survey to evaluate the actual status and the activities of the "Momiji house" that provides short-term stay service in Japan. Results showed that the needs of caregivers include not only a temporary rest time but also a meaningful time for children. It is also important for families that provide short-term facilities as opportunities and places to take time off from care and set the time spent with disabled children.
This could expand the whereabouts of families and contribute toward reducing the mental burden of caregivers. Currently, it is difficult to operate a sustainable short-term service because of the difficulty to procure sufficient charges for the desired service.
For users, we must consider more about the subsidy system and continue to provide information about people with disabilities.
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Case Report
Title
Right Ventricular Outflow Tract Reconstruction for Tetralogy of Fallot with Caudal Regression Syndrome
Author
Fumitake Miura1) Isamu Hanada1) Jun Shimada1) Yosuke Kitagawa1) Tatsuya Yamamoto1) Katsuki Otani1) Hiroshi Fujita2) Toru Takahashi3) Susumu Yonesaka3) and Etsuro Ito1)
1)Department of Pediatrics, Hirosaki University School of Medicine and Graduate School of Medicine
2)Department of Pediatrics, Aomori National Hospital
3)Hirosaki University School of Health Sciences and Graduate School of Health Sciences
Abstract
Caudal regression syndrome (CRS) is a rare congenital disorder with various malformations. Diabetes during pregnancy has been suggested as a possible causative factor. The survival case of severe CRS has not been reported yet. Although it is reported that CRS patients are complicated with cardiovascular anomalies, to our knowledge, the case that underwent cardiac operation has not been reported in severe CRS patients. We report a case of a male newborn with CRS and cardiovascular anomaly born from a mother with poorly controlled diabetes. He was suspected of having CRS and cardiovascular anomalies in the fetal period. After he was born, we diagnosed him as having CRS with tetralogy of Fallot (TOF) and pulmonary hypoplasia. When he was 2 years and 1 month old, we performed right ventricular outflow tract reconstruction for TOF with some efficacy. This case suggests that cardiac surgery of CRS can contribute to the improvement of symptoms and transition to home care, which can be a treatment option.
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Case Report
Title
Recurrent Limbic Symptoms in Autoimmune Encephalitis/Encephalopathy Following Influenza Infection: A Case Report
Author
Taro Yoshida1) Atsushi Kamei1) Yukitoshi Takahashi2) Manami Akasaka1) Nami Araya1) Maya Asami1) Sachiko Tanifuzi1) Shuji Kusano1) and Kotaro Oyama1)
1)Department of Pediatrics, Iwate Medical University School of Medicine
2)Shizuoka Institute of Epilepsy and Neurological Disorders
Abstract
Several studies have reported autoimmune encephalitis/encephalopathy with limbic symptoms, which could recur despite symptomatic improvement, in patients with influenza infection. We report the case of a 13-year-old boy diagnosed with influenza infection. His fever persisted for 5 days, and he developed limbic symptoms, including incoherent verbalization. Despite fever resolution on the subsequent day, his limbic symptoms persisted. As the results of cerebrospinal fluid examination, electroencephalography, and head magnetic resonance imaging were normal, autoimmune encephalitis/encephalopathy was suspected. The patient received intravenous methylprednisolone pulse therapy for 3 days, starting on day 7, and was discharged on day 14. Cerebrospinal fluid analyses using ELISA revealed that the level of antibodies against anti-N-methyl D-aspartate (NMDA)-type glutamate receptors (GluRs) was high. He was diagnosed with influenza-associated anti-NMDA-type GluR antibody (ELISA)-mediated autoimmune encephalitis/encephalopathy. Although his symptoms recurred on days 32 and 54, they improved with steroid pulse therapy. He was administered oral prednisolone and did not experience a relapse. This report presents a case of autoimmune encephalitis/encephalopathy that developed with limbic symptoms following influenza infection. While some cases have reported improvement with a monophasic course in a short period, three cases have reported relapse after steroid treatment. Prolonged limbic symptoms after influenza infection could indicate autoimmune encephalitis, which warrants empirical treatment with immunoregulatory therapy.
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Case Report
Title
Non-invasive Positive Pressure Ventilation for Two Patients with Spinal Muscular Atrophy Type 1
Author
Hiroto Idesawa1) Keiichi Motoyama1) Ryuta Tanaka1)2) Ryutaro Suzuki1) Fujiko Fukushima1) Nobuaki Iwasaki1)3) Chie Kobayashi1)2) and Isho Izumi1)
1)Department of Pediatrics, Ibaraki Children's Hospital
2)Department of Child Health, Faculty of Medicine, University of Tsukuba
3)Department of Pediatrics, Ibaraki Prefectural University of Health Sciences
Abstract
<Background> Spinal muscular atrophy (SMA) type 1 is a lethal disorder of lower motor neurons, given its natural course. Nusinersen is a new medicine that can significantly improve disease prognosis due to its innovative mechanism of action; however, some patients may require mechanical ventilation for long-term survival even in the era of nusinersen. Although noninvasive positive pressure ventilation (NPPV) has been found to be effective for prolonging life while avoiding tracheostomy, its implementation can be difficult. <Method> We reviewed the clinical course of two patients with SMA type 1 who were treated in the pre-nusinersen era, considering the positive and negative relationships with NPPV. <Case presentation> The first patient was able to return home with NPPV at 4 months of age; however, he required frequent resuscitation for airway obstruction at home and died at 1 year and 3 months of age. The second patient was admitted to our hospital due to pneumonia at 5 months of age. She received NPPV, but she was restricted to staying in the intensive care unit and died at 2 years and 4 months of age. <Discussion> NPPV is probably introduced as a means of provisional lifesaving. However, repetitive airway obstruction occurring toward the terminal stage causes severe distress and anguish among patients and their parents and makes it difficult for them to return home. Therefore, we should consider providing palliative medicine/care since the introduction of NPPV.
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Case Report
Title
Scimitar Syndrome Complicated by Congenital Pulmonary Airway Malformation
Author
Masahiro Tahara Risa Morita Kotaro Urayama and Mitsunobu Sugino
Department of Pediatrics, Tsuchiya General Hospital
Abstract
Scimitar syndrome is complicated by right lung sequestration and pulmonary hypertension, and congenital pulmonary airway malformation (CPAM) is complicated by respiratory impairment, pneumonia, and occasionally malignant tumors. We report a case of scimitar syndrome complicated by CPAM in a female infant who was delivered at 35 weeks of gestation. Respiratory impairment developed soon thereafter, and computed tomography revealed a multicystic lung lesion in the right lower lobe with an aberrant artery originating from the celiac trunk to the affected lobe. A scimitar vein flowed to the inferior vena cava, and mild pleural thickening was identified at the age of 1 month. No bronchial abnormalities were evident. The symptoms improved after several days of ventilator support. Pneumonectomy was planned after the infant increased in stature. However, she developed pneumonia at the age of 13 months, and after open lung biopsy, she was pathologically diagnosed with CPAM, Stocker type 3. At that time, a solid adhesion identified in the pleura on the right lung indicated that a pneumonectomy might be difficult. Thereafter, the scimitar vein became obstructed. Currently, she has reached the age of 12 years without severe complications. We speculated that scimitar syndrome complicated by CPAM might be more likely to occur with inflammation and adhesion histologically. However, surgery should have been proceeded before the adhesion formed on the pleura. When scimitar syndrome is complicated by CPAM, early surgery should be considered to avoid adhesions, enhance the growth of the unaffected lung, and modify the risk for malignant tumors.
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Case Report
Title
A Case of Type 1 Gaucher Disease with Calcification of Mesenteric Lymphadenopathy and Inner Ear Hearing Loss despite Enzyme Replacement Therapy
Author
Keiko Hirano1) Akira Endo1) Masami Shirai1) Tokiko Fukuda2) and Tomoko Matsubayashi2)
1)Department of Pediatrics, Iwata City Hospital
2)Department of Pediatrics, Hamamatsu University School of Medicine
Abstract
We report a patient with Gaucher disease (GD) who developed progressive calcification of the lymph nodes and inner ear hearing loss, despite undergoing enzyme replacement therapy (ERT). Histopathologic examination of the mesenteric lymph nodes, and duodenal and inner ear mucous tissues revealed that they were replaced with numerous Gaucher cells. ERT for the treatment of GD was administered via glucocerebroside-laden macrophages through the macrophage mannose receptor (MMR). In this case, MMRs were detected on macrophages in mesenteric lymph nodes but not in the duodenal mucosa. To increase the cellular uptake of ERT products, we changed imiglucerase to velaglucerase alpha, which has a longer chain of high-mannose-type glycans. The patient found it difficult to attend school due to severe diarrhea and hearing loss, but enlargement of the calcification stopped after the ERT products were changed, and his abdominal symptoms gradually disappeared. Subsequently, he was able to attend school; however, he developed profound hearing loss and underwent cochlear implantation.
Although these complications are rare, they result in decreased quality of life in patients with GD. This is the first report that revealed the expression of MMRs in the gastrointestinal tract. We suggest that velaglucerase alpha, a high-mannose-type ERT product, may be effective for controlling mesenteric lymph node calcification in patients with GD.
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Case Report
Title
A Case of a Cystic Fibrosis with Nontuberculous Mycobacteriosis
Author
Yuya Kuwabara Miki Ogiso Norifumi Yokoyama and Kunihiro Shinoda
Department of Pediatrics, Gifu Municipal Hospital
Abstract
Cystic fibrosis is a disease with a relatively high prevalence in European countries; however, it rarely occurs in Japan. We report a case of cystic fibrosis complicated by nontuberculous mycobacteriosis. The patient was a 15-year-old girl admitted to our hospital; she had fever and cough that persisted for about 2 weeks. Although antibiotic treatment was started, she was placed on mechanical ventilation due to exacerbation of respiratory conditions. Based on the detection of Mycobacterium avium in her sputum specimen, she was diagnosed with nontuberculous mycobacteriosis and treated with multidrug therapy. The patient was also suspected to have cystic fibrosis as her condition got complicated by the nontuberculous mycobacteriosis, and she was finally diagnosed with cystic fibrosis based on the results of sweat Cl− analysis and genetic testing. Her respiratory condition improved, and oxygen therapy was no longer required after the administration of dornase alfa inhalation therapy and physical therapy. Although cystic fibrosis is a rare disease, it should be suspected in the differential diagnosis for cases with respiratory symptoms refractory to typical infection control methods or for pediatric cases complicated by nontuberculous mycobacteriosis.
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Case Report
Title
IgA Vasculitis with a High Fever and Increased Inflammatory Reaction Caused by Mesenteric Panniculitis
Author
Ryuhei Yasuoka1) Naoki Abe1) Toaki Kohagura1) Haruna Nakaseko1) Shinji Kawabe1) Kazuki Tanaka2) Satoshi Hibino2) Naoya Fujita2) and Naomi Iwata1)
1)Department of Infection and Immunology, Aichi Children's Health and Medical Center
2)Department of Nephrology, Aichi Children's Health and Medical Center
Abstract
IgA vasculitis is a systemic vasculitis that occurs more frequently in children and is characterized by skin purpura, arthritis, abdominal pain, and nephritis. It is sometimes accompanied by a slight fever and a slightly elevated inflammatory response or, albeit rarely, a high fever and a severely elevated inflammatory response. Characteristics associated with the latter response in patients are unclear.
Here we report the first pediatric cases of IgA vasculitis with mesenteric panniculitis presenting a sustained high fever and severely elevated inflammatory response. Abdominal sonography revealed thickening of the walls of the small intestine in both patients. The degree of echogenicity in thickened mesenteric adipose tissue was elevated and increased blood flow to the mesenteric adipose tissue was revealed by ultrasound color doppler examination, suggesting that the patients had mesenteric panniculitis.
If patients with IgA vasculitis show a sustained high fever, abdominal pain, and a severely elevated inflammatory response, mesenteric panniculitis should be suspected. Sonography is a useful diagnostic examination.
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Case Report
Title
Negative Pressure Pulmonary Hemorrhage Followed by Choking on a Candy in a Child with Down Syndrome
Author
Maho Umehara Natsuko Suzuki Akira Takei Mariko Terauchi and Masayasu Ohta
Department of Pediatrics, JA Toride General Medical Center
Abstract
The patient in our case was a 12-year-old boy with Down syndrome (DS). While eating a large candy, he suddenly choked on it, and severe cyanosis subsequently appeared. After he swallowed the candy, marked cyanosis disappeared, but he had hemoptysis and severe coughing. The oxygen saturation was 70%, and the breathing sounds were weak. The chest X-ray and computed tomography demonstrated pulmonary diffuse consolidation with air bronchograms, and the bronchoscopy and bronchoalveolar lavage revealed pulmonary hemorrhage. The child gradually recovered from clinical symptoms when given 4 days of supplementary oxygen. The pulmonary hemorrhage that followed removal of an upper airway foreign body and the rapid recovery suggested a negative pressure pulmonary hemorrhage (NPPH).
Although children with DS are prone to dysphagia because of functional and structural abnormalities of the oropharyngeal airway and are at a risk of developing negative pressure pulmonary edema (NPPE) after tonsillectomy due to fragility of pulmonary capillary integrity, this is the first report of NPPH followed by aspiration of a foreign body in a patient with DS.
NPPE and NPPH have a favorable prognosis with adequate respiratory assistance. In a patient with sudden respiratory distress after being released from airway obstruction and pulmonary diffuse consolidation on the X-ray, the physician should be concerned about NPPE/NPPH.
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