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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY

Vol.121, No.11, November 2017


Original Article
1. Variability in Antibiotic Use in Perinatal Medical Centers
2. Survey of a Home Medical Care System for Medically Complex Patients with Special Health Care Needs
3. Regional Outbreak of Childhood Pertussis
4. Characterization of Helicobacter Pylori Infection in Children: a Single-center Study in Okinawa
Case Report
1. Erythritol Allergy without a Positive Reaction on the Skin Prick Test and Basophil Activation Test
2. Sequential Liver and Kidney Transplantation in Two Children with Autosomal Recessive Polycystic Kidney Disease with Hepatic Complications
3. A Case of Turner Syndrome without Short Stature Diagnosed with Hashimoto's Disease as a Clue
4. Orbital Cellulitis Caused by Non-typeable Haemophilus Influenzae in a Healthy 1-month-old girl
5. Secondary Carnitine Deficiency due to Pivalate-conjugated Antibiotics Diagnosed with Tandem Mass Spectrometry: A Case Report
6. A Case of Biliary Tract Abnormalities Associated with Duodenal Atresia in a Two-month Old Girl with Trisomy 21


Original Article
Title
Variability in Antibiotic Use in Perinatal Medical Centers
Author
Kaoru Okazaki1) Kenta Ito2) Yuho Horikoshi2) and Masatoshi Kondo1)
1)Division of Neonatology, Department of Pediatrics, Tokyo Metropolitan Children's Medical Center
2)Division of Infectious Diseases, Department of Pediatrics, Tokyo Metropolitan Children's Medical Center
Abstract
We collected data from 25 perinatal medical centers in Japan on the clinical and demographic characteristics of their patients and the types and quantities of systemic antimicrobials used [expressed as days of therapy (DOT)] between April 1, 2014 and March 31, 2015. We conducted a retrospective cohort study of 8,808 consecutive neonatal inpatients, including 506 less-than-gestational age (28 weeks) infants and 1,280 very low birth weight infants. Antimicrobial use totaled 38,588 days and ranged from 27.1 to 338.5 DOTs/1,000 patient-days. Ampicillin (35%), amikacin (11.4%), and cefotaxim (9.6%) were the most frequently used agents. Perinatal medical centers that used more antimicrobial agents also used a higher proportion of penicillin, aminoglycoside, and cephem. In addition, an increasing proportion of premature neonates born before 28 weeks' gestation was associated with a low penicillin or a high carbapenem use rate. In conclusion, we showed the diversity of antimicrobial use in perinatal medical centers in Japan. To achieve the goal of antimicrobial stewardship, DOT may be useful as an approach to assessing antibiotic use in each center.




Original Article
Title
Survey of a Home Medical Care System for Medically Complex Patients with Special Health Care Needs
Author
Yoshiko Tsubouchi1)2) Akiko Tamasaki2) Ayako Itakura2) and Yoshihiro Maegaki2)
1)Department of Pediatrics, National Hospital Organization Yonago Medical Center
2)Division of Child Neurology, Faculty of Medicine, University of Tottori
Abstract
A questionnaire survey of 310 practitioners, pediatricians, and neurologists working in hospitals and clinics in Tottori Prefecture was conducted. Eighty-eight (28.4%) subjects answered. Ten (11%) of those surveyed provided medical care for medically complex cases and seven (70%) of the 10 answered that they could continue to provide medical care for these patients. Sixteen (21%) of those who were not providing medical care responded that they could provide it. Those who replied that providing medical care was impossible gave the following reasons: anxiety in treating these patients (62%) and pressure of daily business (40%). The most frequent answer about essential duties providing medical care for medically complex patients was to respond in hospital at emergency clinics (92%). In the free description section, the following was noted: little information regarding home medical care, the necessity for preliminary informed consent about home doctors' participation in the patients' medical care, and the need for a backup system outside business hours for home doctors. In conclusion, to politely provide patients' medical information to home doctors, to build a backup system at emergency and outside home doctors' business hours, and to spread understanding about a home medical care for patients requiring home medical care are necessary.




Original Article
Title
Regional Outbreak of Childhood Pertussis
Author
Tomoko Saikawa1) Emi Maeda2) Asaka Watanabe2) Taizou Wada3) and Kunio Ohta3)
1)Department of Pediatrics, Nanto Central Hospital
2)Department of Microbiological Laboratory, Nanto Central Hospital
3)Department of Pediatrics, Kanazawa University Graduate School of Medicine
Abstract
This report describes an outbreak of pertussis in Nanto City, Toyama between October 2015 and May 2016 that mainly affected primary school and junior high school children living in five neighboring regions (designated A-E). Fifty-two patients were clinically suspected of pertussis, 30 of whom were confirmed by laboratory tests. Two genotypically distinct strains of Bordetella (B.) pertussis, MT27a and MT28, were isolated from four patients in regions B and C, and from one in region E, respectively. These data suggested that several strains of B. pertussis coexisted in the affected regions. Several school-age children had anti-pertussis toxin IgG levels of < 10 EU/mL at initial diagnosis. Waning vaccine-induced immunity to pertussis with advancing age might account for the development of local epidemics. Loop-mediated isothermal amplification assays detected B. pertussis with 44% positivity when patients were tested within 14 days of onset, whereas 10 patients tested > 15 days after onset were all negative. The outbreak lasted for eight months. The primary activity implemented to control pertussis outbreaks is to decrease morbidity and mortality among newborns and infants. Outbreak guidelines should be developed early in the outbreak with information about clinical presentations, case definitions, diagnosis, surveillance, notification, and public health measures (prophylaxis, immunization, and isolation).




Original Article
Title
Characterization of Helicobacter Pylori Infection in Children: a Single-center Study in Okinawa
Author
Toshifumi Yodoshi Itaru Iwama Taisuke Tsuji Kensuke Kanie Kei Matayoshi Saori Kinjo and Moriyasu Kohama
Department of Pediatrics, Okinawa Chubu Hospital
Abstract
Background: Helicobacter pylori (H. pylori) can be a risk factor for not only gastrointestinal symptoms but also for gastric cancer in young people. Therefore, it is important to identify and treat the infection in childhood. We investigated the current status of H. pylori infection in children at Okinawa Chubu Hospital.
Methods: We undertook a referral-hospital-based retrospective descriptive study between January 2003 and June 2016 in Okinawa, Japan. For children aged 15 years or younger who underwent H. pylori eradication in our department, we investigated their clinical background and findings on upper gastrointestinal (G.I.) endoscopy. In addition, we compared drug susceptibility test results and eradication rates between an early group receiving standardized treatment (2003-2011) and a later group receiving selective treatment based on the results of drug susceptibility tests (2012-2016).
Results: Of 29 patients (14 patients in the early group, 15 patients in the later group), all showed abnormal mucosal findings on upper G.I.endoscopy. The early group was given the standard PAC (PPI, Amoxicillin, Clarithromycin) first-line treatment in all cases. The later group, however, was treated based on drug susceptibility tests: PAC therapy or PAM (PPI, Amoxicillin, Metronidazole) therapy. Cure rates on first-line treatment were 44.4% for the early group and 93.3% for the later group.
Conclusions: In Okinawa, since clarithromycin-resistant H. pylori is increasing, upper endoscopy and drug susceptibility tests were useful for the successful initial treatment of H. pylori infection in children.




Case Report
Title
Erythritol Allergy without a Positive Reaction on the Skin Prick Test and Basophil Activation Test
Author
Akira Sakai Osamu Natsume Yukiko Kato Tomohide Taguchi and Tatsuki Fukuie
Department of Pediatrics, Hamamatsu University School of Medicine
Abstract
We encountered a case of erythritol allergy in which skin prick tests (SPTs) and the basophil activation test (BAT) with erythritol were negative. The case was 5-year-old boy. He had facial rash, swelling, and wheezing after ingestion of konjac jelly. Although SPT with konjac jelly was negative, an oral food challenge test with konjac jelly was positive. SPTs with individual materials of the jelly, including 300 mg/ml of erythritol, and BAT using erythritol were negative. However, an oral food challenge test with 0.4 g of erythritol was positive. Even though SPTs and BAT with erythritol are negative in patients whose causative food cannot be identified, the differential diagnosis of erythritol allergy should be kept in mind and OFC would lead to the diagnosis.




Case Report
Title
Sequential Liver and Kidney Transplantation in Two Children with Autosomal Recessive Polycystic Kidney Disease with Hepatic Complications
Author
Yuri Nawashiro Kenichiro Miura Kiyonobu Ishizuka Shoichiro Kanda Naoto Kaneko Tomoo Yabuuchi Norimasa Tada Takayuki Miyai Noriko Sugawara Hiroko Chikamoto Yuko Akioka and Motoshi Hattori
Department of Pediatric Nephrology, Tokyo Women's Medical University
Abstract
Autosomal recessive polycystic kidney disease (ARPKD) is a hereditary cystic kidney disease in which liver complications are of paramount importance in determining the treatment strategy for end-stage renal disease (ESRD). Most cases do not require liver transplantation because liver function is well preserved, while some individuals with recurrent cholangitis should undergo liver and kidney transplantation (LKTx). At present, there is no consensus on the criteria of recurrent cholangitis which requires LKTx.
We reported two children with ARPKD who had ESRD complicated by recurrent cholangitis and eventually underwent sequential LKTx from a single living donor. Patient 1, a 13-year-old boy, suffered from recurrent cholangitis in spite of prophylactic antibiotics. Patient 2, an 8-year-old boy, was free from recurrence of cholangitis with prophylactic antibiotics and isolated kidney transplantation was scheduled. However, acute cholangitis occurred just before kidney transplantation. Both patients underwent sequential LKTx because of recurrent cholangitis which could not be prevented by prophylactic antibiotics. During the follow up periods of 1 to 3 years after LKTx, no recurrences of acute cholangitis have occurred and renal functions are well preserved. The treatment strategy for ARPKD patients with ESRD should be guided by thorough assessment of individual hepatic complications.




Case Report
Title
A Case of Turner Syndrome without Short Stature Diagnosed with Hashimoto's Disease as a Clue
Author
Hideyuki Iguchi1) Yukie Izumita1) Hidetoshi Sato1) Masamichi Matsunaga1) and Keisuke Nagasaki1)2)
1)Department of Pediatrics, Shibata Hospital-Niigata Prefectual Hospital
2)Department of Pediatrics, Niigata University Medical & Dental Hospital
Abstract
Turner syndrome (TS) is a chromosomal disorder characterized by short stature and ovarian dysfunction with various kinds of physical and internal organ malformations caused by the deletion of the X chromosome. Diagnosis of TS is triggered by neonatal lymphedema and congenital heart disease, short stature in childhood, and ovarian dysfunction in puberty, etc. The clinical manifestations of TS vary, and cases without short stature or ovarian dysfunction may need time to diagnose. This case was a 12-year-old girl with Hashimoto's disease diagnosed due to swelling of the anterior neck at 7 years of age. She was started on oral administration of levothyroxine sodium. Her height at her first visit was 118.3 cm (-0.3 SD). Chromosome banding was performed due to evidence of a high arched palate and micrognathia at 10 years of age. She was diagnosed with TS with a 46,X,idic (X) (p11.4)/ 45, X karyotype by G-banding of peripheral blood lymphocytes. At 11 years old, she began to show breast budding and did not present short stature. Autoimmune thyroid disease is known to be common in TS, but is not found in cases like this with a diagnosis of TS with Hashimoto's disease in childhood as a clue. In cases in which Hashimoto's disease is diagnosed in childhood, we should confirm the Turner phenotype, short stature, and ovarian failure. Hashimoto's disease in childhood should be considered as a possible indication of TS.




Case Report
Title
Orbital Cellulitis Caused by Non-typeable Haemophilus Influenzae in a Healthy 1-month-old girl
Author
Hiroo Yamamoto1) Yusuke Ito2) Masashi Kasai2) Hiroki Takeda3) Junichiro Nishi4) Chisato Miyakoshi1) Yoshinori Kobayashi1) and Satoru Tsuruta1)
1)Department of Pediatrics, Kobe City Medical Center General Hospital
2)Department of Infectious Disease, Hyogo Prefectural Kobe Children's Hospital
3)Department of Emergency and Critical Care Medicine, Hyogo Prefectural Kobe Children's Hospital
4)Department of Microbiology, Graduate School of Medical and Dental Sciences, Kagoshima University
Abstract
A 1-month-old, previously healthy, full-term girl presented with fever. Physical examination revealed no pathological findings. On admission, blood examination showed a leukocyte count of 16,000 cells/uL. Blood cultures were obtained repeatedly, as we considered the possibility of severe bacterial infection. Because of the patient's good appearance, we planned to closely observe her without using antimicrobial treatment.
After admission, we observed swelling in the left eye of the patient. Only one of the four blood cultures was positive for non-typeable Haemophilus influenzae (NTHi). We also found orbital cellulitis of the left eye using computed tomography.
Recently, there has been growing concern regarding severe bacterial infections caused by NTHi. However, cases of orbital cellulitis have been rarely reported. In this case, however, repetitive physical examination made it possible for us to diagnose the bacterial infection without delay. We found that multiple blood cultures for the evaluation of infants with suspected bacterial sepsis may improve pathogen detection.




Case Report
Title
Secondary Carnitine Deficiency due to Pivalate-conjugated Antibiotics Diagnosed with Tandem Mass Spectrometry: A Case Report
Author
Waka Kajiwara1) Shigekuni Tuchiya2) Mika Sasaki1) Akio Takahashi1) Yuhei Takasago1) and Syunichi Maisawa1)
1)Department of Pediatrics, Morioka Children's Hospital
2)Department of Pediatrics, Iwate Medical University
Abstract
An 18-month-old girl was admitted to our hospital in the early morning as an emergency, presenting with convulsions and unconsciousness. Four days previously, she had been prescribed cefditoren pivoxil (CDTR-PI) by a local doctor for a common cold. She was noted as having decreased appetite two days before presenting to hospital. Clinical examination revealed severe hypoglycemia. After treatment with intravenous glucose, the patient rapidly regained consciousness. Tandem mass spectrometry, using a filter paper blood spot at the time of the consultation, revealed a drop in C0 and an increased level of C5. This was consistent with secondary carnitine deficiency induced by pivalate-conjugated antibiotics. Subsequent urinary organic acid analysis and carnitine clearance were negative. Isovaleric acidemia and systemic primary carnitine deficiency were also excluded. The use of tandem mass spectrometry in young children is not common beyond the neonatal period, but may be a useful adjunct for screening of metabolic disease in the emergency room.




Case Report
Title
A Case of Biliary Tract Abnormalities Associated with Duodenal Atresia in a Two-month Old Girl with Trisomy 21
Author
Takanori Noto1) Ryosuke Tonouchi1) Naohide Kin1) Jyunji Fukuhara1) Masao Murabayashi1) and Shori Takahashi2)
1)Department of Pediatrics, Numazu City Hospital
2)Department of Pediatrics and Child Health, Nihon University School of Medicine
Abstract
A two-month-old girl with trisomy 21 was admitted after vomiting milk. Abdominal X-ray demonstrated a mildly dilated stomach and proximal duodenum with bowel gas distally. An upper gastrointestinal series ruled out malrotation. The stomach and duodenal bulb were moderately dilated, but the duodenal bulb was narrow. A small amount of contrast medium was also observed at the proximal jejunum and the common bile duct was opaque. Upper gastrointestinal endoscopy revealed duodenal atresia, and duodenoduodenostomy was performed.
In patients with duodenal atresia, a "double bubble sign" is conventionally present on plain radiographs. When bowel gas exists distal to the duodenum, duodenal atresia is often excluded by differential diagnosis. The authors present a case in which contrast could be observed in the small bowel and biliary system on an upper gastrointestinal series in a patient with duodenal atresia and an anomalous common bile duct.
Clinical presentation may occur relatively late if the biliary communication is large enough to permit passage of some milk or formula. Duodenal atresia is rarely associated with anomalous biliary ducts that permit communication between the proximal and distal duodenal segments. Duodenal atresia with an anomalous biliary system should be considered as a possible cause if vomiting continues.




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