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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.118, No.3, March 2014
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Review
Title
Effectiveness of Frenotomy in Breastfeeding Difficulties in Infants with Ankyloglossia: Systematic Review
Author
Yasuo Ito
Department of Pediatrics and Pediatric Surgery, International University of Health and Welfare, Atami Hospital
Abstract
The aim of this systematic review was to critically examine the existing literature regarding the effectiveness of tongue-tie division in infants with ankyloglossia. A clinical question was structured according to PICO as follows: In infants with poor breastfeeding and ankyloglossia (patient), does frenotomy (intervention) compared to lactation support alone (comparison) improve feeding (outcome)? An electronic literature search was systematically conducted from databases including PubMed, Japana Centra Revuo Medicina (Igaku Chuo Zasshi), CINAHL, and Cochrane Library using the key words "ankyloglossia," "tongue-tie," "frenotomy," and/or "breastfeeding".
The literature search yielded 4 randomized clinical trials, and 12 observational studies for analysis. The quality of the literature was rated in regard to the two most important outcomes (sucking/latching, and nipple pain) and five less important outcomes (milk supply/milk production, continuation of breastfeeding, weight gain, adverse events, and dyad distress) in accordance with the GRADE (Grades of Recommendations, Assessment, Development, and Evaluation) system.
The literature review supported an overall moderate quality of evidence for the effectiveness of a frenotomy for the treatment of breastfeeding difficulties in infants with ankyloglossia. No major complications from a frenotomy were reported most likely because they were performed by well-trained healthcare professionals.
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Original Article
Title
Clinical Comparison of Acute Focal Bacterial Nephritis with Acute Pyelonephritis in Our Institution
Author
Takao Tsujioka Yukiyo Ohshima Shuntarou Tsumagari Mitsuru Nawate Noriko Yanazume Mikio Yoshioka Takaaki Shikano and Yutaka Takahashi
Department of Pediatrics, KKR Sapporo Medical Center
Abstract
We compared 9 cases of acute focal bacterial nephritis (AFBN) with 91 cases of acute pyelonephritis (APN), who had been admitted to our hospital for treatment from April 1, 2006 through March 31, 2012. Compared with the APN patients, the AFBN patients showed significantly long duration of fever and duration of admission. Significantly many AFBN patients had associated manifestations including vomiting and abdominal pain. The AFBN patients were significantly older than the APN patients. Pyuria was not revealed on urinary sediment in significantly many AFBN patients, while they showed significantly high serum C-reactive protein levels and urinary β2-microglobulin levels. Since Escherichia coli was not detected from their urine as pyogenic bacteria, many AFBN patients had to receive different antibiotics after the start of treatment. Ultrasonographic examination revealed ureteral dilatation, renal swelling, and masses in the renal parenchyma in many AFBN patients. The incidence of the association of AFBN with vesicoureteral reflux was significantly high in the AFBN patients, and the frequency of renal scarring formation in the recovery phase was also significantly high in AFBN patients.
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Original Article
Title
A Report of 42 Cases in Which Vertical Transmission of HIV was Prevented at the National Center for Global Health and Medicine
Author
Shinichi Hosokawa1) Moe Akahira1) Tetsuya Kunikata1)2) Hirofumi Miyazawa1)3) and Takeji Matsushita1)
1)Department of Pediatrics, National Center for Global Health and Medicine
2)Department of Neonatology, Saitama Medical Center
3)Oonodai Clinic
Abstract
Between 1999 and 2010, mother-to-child transmission of human immunodeficiency virus (HIV) was prevented in 42 cases at the National Center for Global Health and Medicine. No sex difference in neonates was apparent (21 male, 21 female). Mothers comprised 21 Japanese mothers and 21 mothers of foreign nationality. Median gestational age at birth was 36 weeks and 3 days, and median birth weight was 2,552.5 g. In general, chemoprophylaxis with azidothymidine (AZT) was conducted for all patients. Anemia was identified in all infants and hemoglobin levels were reduced to <12 g/dl. In terms of neurological prognosis, 4 cases showed convulsions, with one of those cases given a diagnosis of epilepsy. Magnetic resonance imaging (MRI) of the head was performed in 24 patients. Two infants with mild enlargement of the cerebral ventricles were observed, and one infant showed findings of periventricular leukomalacia. Only one case showed developmental disorder with autism, and another displayed borderline developmental disorder. In the prevention of mother-to-child transmission of HIV, appropriate treatment in early pregnancy is necessary. However, for pregnant women of foreign nationality, the timing of visits to the hospital and identification of HIV may be delayed due to linguistic and social barriers. Protocols for long-term follow-up after completion of 6-week AZT chemoprophylaxis have not yet been defined in Japan, so we consider this as an issue related to developmental follow-up.
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Original Article
Title
Comparison of Denver Developmental Screening Test 2 and the Kyoto Scale of Psychological Development tTest
Author
Hidetoshi Mezawa1)2)3)4) Keiji Hashimoto3)4) Masutomo Miyao3)5) Yukihiro Oya3)6) and Hiroyuki Ida1)
1)Department of Pediatrics, Jikei University School of Medicine
2)Division of Molecular Epidemiology, Jikei University School of Medicine
3)Japan Environmental and Children's Study Medical Support Center, National Center for Child Health and Development
4)Division of Rehabilitation Medicine and Developmental Evaluation Center, National Center for Child Health and Development
5)Division of Developmental and Behavioral Medicine, National Center for Child Health and Development
6)Division of Allergy, National Center for Child Health and Development
Abstract
Background: Although many developmental evaluation scales are utilized in Japan, they have not yet been compared in a clinical study. Here, we compared the Japanese Denver Development Screening Test 2 (J-DENVER 2) and the Kyoto Scale of Psychological Development Test 2001 (KSPD).
Methods: We assessed children 6 years old younger using the J-DENVER 2 and KSPD. KSPD developmental quotients (DQ) less than 70 and 80 were defined as endpoints. Children were classified based on their J-DENVER 2 results, and the DQ with the KSPD was evaluated. The J-DENVER 2 items delay, caution, and normal were defined as weighting variables 1, 0.5, and 0, respectively. Receiver operating characteristic curves were generated, and the area under the curve (AUC) and sensitivity/specificity were analyzed.
Results: A total of 159 children were assessed. None of the children had a DQ less than 70 in the J-DENVER 2 test normal group. The AUCs for weighting variables showed high accuracy; i.e., 0.91 and 0.92 for DQ less than 70 and less than 80, respectively. The specificity was 95% when cutoff values were set at greater than 9 or greater than 6 for children whose DQs were less than 70 or less than 80, respectively.
Conclusion: J-DENVER 2 test results can predict DQs for the KSPD in children under 4 years of age who show developmental delay.
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Original Article
Title
An Infant Who Suffered Abusive Head Trauma with Abnormal Signal Intensity in the Subcortical White Matter on Diffuse-weighted Brain Magnetic Resonance Imaging
Author
Yo Okizuka1) Tatsuya Kawasaki1) Yoshinori Okumura2) Yusuke Ito1) Hatsuka Minamino1) Taku Koizumi1) Takamori Kanazawa1) Ryosuke Fukushima1) Hideo Aiba2) and Ikuya Ueta1)
1)Department of Pediatric Critical Care, Shizuoka Children's Hospital
2)Department of Neurology, Shizuoka Children's Hospital
Abstract
We report a 4-month-old girl who suffered abusive head trauma. She was referred to our hospital following the acute onset of fever, poor activity levels, prolonged generalized tonic-clonic seizures, and impaired consciousness. Lumbar puncture demonstrated a slightly elevated total leucocyte count, and diffusion-weighted brain MRI revealed abnormal signal intensity in the subcortical white matter. On the basis of these findings, we diagnosed acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) and administered methylprednisolone pulse therapy. However, 7 days after admission, her diagnosis was changed to abusive head trauma after the detection of severe bilateral retinal hemorrhage, subdural hematoma, and skull fractures. The follow-up radiography and retinal investigation are useful to make an accurate diagnosis in this case. Our case report suggested that it is important to consider abusive head trauma as part of the differential diagnosis in acute encephalitis/encephalopathy in pediatric patients.
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Original Article
Title
Novel SLC2A1 Mutation in a Patient with Glucose Transporter1 Deficiency
Author
Tomotaka Murakami1) Tetsuya Kibe1) Kenji Yokochi1) Naoya Matsumoto2) Satoru Takahashi2) and Katsumi Imai3)
1)Department of Pediatrics, Seirei-Mikatahara General Hospital
2)Department of Pediatrics, Asahikawa Medical University
3)Department of Pediatrics, Shizuoka Institute of Epilepsy and Neurological Disorders
Abstract
Glucose transporter 1 (GLUT1) transports glucose into the brain. SLC2A1 gene mutation causes GLUT1 deficiency, an autosomal dominant disorder characterized by reduced glucose transport across the blood-brain barrier, and is responsible for a broad spectrum of diseases, from classical to non-classical forms. Here we describe a 4-year-old Japanese boy with GLUT1 deficiency who presented with seizures during the neonatal period, developmental delay and ataxic gait that worsened with fasting but promptly improved after food intake. The cerebrospinal fluid-blood glucose ratio was 0.32 (normal≥0.45). Genetic testing analysis revealed a novel mutation of the SLC2A1 gene (c.223 G>A, p.G75R). Administration of a ketogenic diet was significantly beneficial for ataxic gait, electroencephalogram recordings and mood stabilization. p.G75R is the first novel mutation identified within exon 3 of the SLC2A1 gene and associated with a non-classical carbohydrate-responsive disease. This case demonstrates that GLUT1 deficiency should be investigated in individuals with unexplained developmental delay, even when epilepsy is not intractable, particularly in combination with fluctuating abnormal movement related to food intake.
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Original Article
Title
A Case of Fukuyama Congenital Muscular Dystrophy in Which Rhabdomyolysis Developed at Birth
Author
Hiroshi Kamiya1) Atsushi Tanaka2) Hiromu Umehara1) Daisuke Abe1) Kenji Kanda1) Setsuko Nishijima1) and Tuyoshi Ishigami1)
1)Department of Pediatrics, Hikone Municipal Hospital
2)Department of Pediatrics, Graduate School of Medicine, Kyoto University
Abstract
We examined a new-born male infant (0-day-old) who developed rhabdomyolysis and was clinically diagnosed with composite heterozygous FCMD at birth. He was born by vaginal delivery after 41 weeks and 0 days of gestation, and no fetal distress was observed during the progression of labor. He was transferred to our hospital because of macrosomia and hypoglycemia. His hypoglycemia improved; however, a marked rise in the creatine kinase (CK) was observed. Subependymal hemorrhage and left clavicle fracture were detected. The next day, we observed brawny edema-like changes throughout his body. We performed a full-body magnetic resonance imaging (MRI), and he was given a diagnosis of rhabdomyolysis. At 10 days of age, although the brawny edema-like changes throughout his body had improved, decreased muscle tension was observed. The CK level decreased after peaking on the 1st day of age at 78,195 IU/l, but high levels of about 5,000 IU/l were persistent. We performed a follow-up brain MRI that revealed pachygyria. Based on his decreased overall muscle tension and persistent abnormally high serum CK levels after rhabdomyolysis as well as characteristic findings from the brain MRI, we diagnosed FCMD. At 6 months of age, we performed genetic analysis and confirmed that he was composite heterozygous for fukutin. We believe that the rhabdomyolysis he developed at birth was caused by the gene responsible for muscle cell vulnerability due to FCMD, combined with the physical stress due to labor.
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Original Article
Title
Congenital Hepatic Fibrosis Mimicking Primary Sclerosing Cholangitis: A Case Report
Author
Sonoko Ijichi1)3) Ayano Inui1) Tomoyuki Tsunoda1) Manari Kawamoto1) Tsuyoshi Sogo1) Takashi Kusaka2) Susumu Itoh3) Atsuko Nakazawa4) and Tomoo Fujisawa1)
1)Department of Pediatric Hepatology and Gastroenterology, Saiseikai Yokohamashi Tobu Hospital
2)Maternal Perinatal Center, Department of Pediatrics, Faculty of Medicine, Kagawa University
3)Department of Pediatrics, Faculty of Medicine, Kagawa University
4)Division of Pathology, Department of Clinical Laboratory Medicine, National Center for Child Health and Development
Abstract
We report a case of congenital hepatic fibrosis (CHF) which was not easily distinguished from primary sclerosing cholangitis (PSC).
The patient was a 2-year-old girl. When she was examined due to acute gastroenteritis, hepatomegaly and elevations of hepatobiliary enzymes were noted, and PSC was suspected due to the high serum IgG level and positive autoantibodies. However, no bile duct lesion with typical structures compatible with PSC was noted on magnetic resonance -cholangiopancreatography, and a diagnosis of CHF was made on the basis of liver biopsy.
The patient was followed up with symptomatic management for esophageal varices due to portal hypertension including endoscopic variceal ligation, but PSC could not be excluded due to the concurrence of colitis accompanied by bloody stools, elevation of the IgG level, and positive autoantibodies.
Therefore, at the age of 5 years, the patient underwent endoscopic retrograde cholangiopancreatography, which revealed multiple dilations and stenoses in intra-and extrahepatic bile ducts. Medication with salazosulfapyridine and ursodeoxycholic acid was initiated, with improvements in the hepatobiliary enzyme levels, but she developed ascites at the age of 6 years and 3 months with exacerbation of hepatic dysfunction. Since ascites was not well controlled by medication, living-related liver transplantation from her father was performed when she was 6 years and 6 months. Histological findings of the resected liver showed severe portal fibrosis accompanied by numerous bile ductules with characteristic profiles, which were compatible with the diagnosis of CHF.
In this case, the clinical profile closely resembled that of PSC, but the condition was eventually diagnosed as CHF on the basis of histological findings in the resected liver tissue.
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Original Article
Title
Cardiac Tamponade Caused by Penetration of a Sewing Needle through the Chest Wall
Author
Airi Kawamoto1) Yoshihisa Okamoto1) Kimiko Honda1) Madoka Sawada1) Takashi Matsuoka1) Takashi Soga1) Hideshi Tomita2) Shigeru Uemura2) Akihiko Kitami3) Ryutaro Ukisu4) Yasuki Takenaka4) and Yoh Umeda1)
1)Children's Medical Center, Showa University Northern Yokohama Hospital
2)Cardiovascular Center, Showa University Northern Yokohama Hospital
3)Respiratory Disease Center, Showa University Northern Yokohama Hospital
4)Department of Radiology, Showa University Northern Yokohama Hospital
Abstract
A 13-year-old boy complaining of sharp chest pain was admitted to our hospital. Electrocardiogram showed ST segment elevation in leads II, III, aVF, and V4-V6, and a transthoracic echocardiogram revealed an echo-free space at the pericardium. A chest radiograph (CXR) disclosed a thin metallic shadow overlying the left edge of the cardiac silhouette. Computed tomography (CT) of the chest demonstrated a needle-shaped foreign body in the left pericardium and also showed an enlarged pericardial space. We finally determined there was a needle in the pericardium, which was complicated with cardiac tamponade. After draining approximately 100 ml of blood by placing a suction tube in the pericardial cavity, a 4-cm long needle piercing the pericardium was found using a thoracoscope. Because the violent movement of the needle was in synchrony with the cardiac contractions, the needle was safely removed by open thoracic surgery. The injury site on the surface of the left inferior pulmonary segment was confirmed during surgery. The patient was discharged 14 days after surgery. Migration of a sewing needle to the pericardium, which is very rare in children, can be considered when pediatric patients with chest pain of unknown origin are encountered. When a needle is suspected, the precise compartmental location should be assessed by CT.
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Original Article
Title
Case Report: Lead Poisoning in an Autistic Child with Pica
Author
Koji Tanoue1) Kiyoshi Matsui1) Tadahito Kato1) Ai Kataoka1) Yusuke Hayashi2) and Yoshimasa Wada2)
1)Department of General Medicine, Kanagawa Children's Medical Center
2)Department of Pediatrics, Saiseikai Yokohama Nanbu Hospital
Abstract
Although lead has been used for a variety of purposes, it is toxic and can cause poisoning. We report lead poisoning in an autistic child with pica and demonstrate the importance of vigilance in dealing with this disorder. A 12-year-old autistic boy presented with several episodes of vomiting. After radiographs demonstrated metallic objects in the stomach, 10 pieces of lead (3×7×60 mm) were retrieved by endoscopy. Twenty-six days later, the boy returned because the episodes of vomiting continued. He was admitted to the hospital for dehydration and normocytic normochromic anemia. His hemoglobin was 8.1 g/dl with MCV of 85.5 fl and MCH of 28.6 Pg. His total bilirubin was 2.6 mg/dl. His blood lead level was found to be 79 μg/dl. He was treated with dimercaprol and calcium disodium edetate (EDTA) followed by 2 further courses of EDTA, after which his blood lead level was 36 μg/dl. The vomiting episodes stopped and he had a good appetite. We managed his environment with assessment for potential lead hazards. This case demonstrates the need for continued vigilance and education regarding lead poisoning in children. Children are at much greater risk of lead toxicity for several reasons: increased hand-to-mouth behavior, increased lead absorption by concomitant iron deficiency anemia, and lead exposure due to pica. Clinicians must be aware of several risk factors for lead toxicity and the importance of the prevention of lead exposure.
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Brief Report
Title
A Neonatal Case of Cryptogenic Pulmonary Alveolar Proteinosis
Author
Satoshi Yamashita Sachiko Tokuda Masaharu Moroto Shinsuke Sasaki Kanae Nabeshima and Hajime Hosoi
Department of Pediatrics, Kyoto Prefectural University of Medicine
Abstract
We report a neonatal case of cryptogenic pulmonary alveolar proteinosis (PAP). The neonate had expiratory grunting and tachypnea on the first day of life, and her chest CT imaging showed a diffuse interstitial pattern. We finally diagnosed congenital PAP by bronchoalveolar lavage, but, subsequently, she died at the age of 17 months. Surfactant gene abnormality is known to cause PAP in neonates, but as in this case, there are many unidentified causes. A neonatal case of PAP is very rare, and this case provides a valuable clue to improving the diagnosis and treatment of congenital PAP.
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