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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.118, No.7, July 2014
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Review
Title
Genetic Cholestasis
Author
Tatsuki Mizuochi1)2) and Akihiko Kimura1)3)
1)Department of Pediatrics and Child Health, Kurume University School of Medicine
2)Division of Gastroenterology, Hepatology, and Nutrition, Cincinnati Children's Hospital Medical Center
3)Kumamoto-Ashikita Medical Center for the Severely Disabled
Abstract
It is important to consider a genetic cause of cholestasis when assessing children with jaundice. Genetic disorders associated with cholestasis, such as Alagille syndrome, citrin deficiency (NICCD), bile acid transporter defects, inborn errors of bile acid synthesis (IEBAS), mitochondrial hepatopathy, bilirubin transporter defects, inborn errors of metabolism, and ductal plate malformation, have been reported in Japan. Cholestasis associated with these genetic disorders is intrahepatic, not extrahepatic. Therefore, it is important to evaluate serum γ-GTP, total bile acids (TBA), and coagulation blood tests in children with cholestasis. Bile acid transporter defects should be considered in cholestatic patients with normal γ-GTP and elevated TBA. In cholestatic patients with normal γ-GTP and normal TBA, urinary bile acid analysis should be performed to distinguish IEBAS. Any cholestatic child with a recognizable coagulopathy should receive intravenous Vitamin K. There are some genetic disorders of cholestasis that are amenable to pharmacotherapy, such as synthetic bile acid supplementation in IEBAS. Non-pharmacologic therapies, such as partial biliary diversion in the patient with bile acid transporter defects, can improve survival with native liver, delay liver transplantation, and improve quality of life.
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Original Article
Title
Trends in Pediatric Bacteremia after the Introduction of Hib and PCV-7 Vaccines
Author
Hiroyuki Shimizu and Tetsunori Funabiki
Children's Medical Center, Fujisawa City Hospital
Abstract
Several years have passed since the introduction of combined Haemophilus influenzae type b and 7-valent pneumococcal conjugate vaccination in Japan. We analyzed the effects of 2 vaccines during 5 years including the period of increasing vaccination rate in Fujisawa City Hospital, Japan. The average number of bacteremia cases from 2008 to 2010 (pre-vaccine era) was 17.3 cases per year. The average number of cases from 2011 to 2012 (post-vaccine era) was 8 cases per year (reduction rate, 53.8%). H. influenzae was isolated in 8 cases in 2008 and in 9 cases in 2009. However, the average number of cases was subsequently greatly reduced to 1.7 cases. Additionally, the number of Streptococcus pneumoniae cases was also reduced to only 1 case each in 2011 and 2012, which was isolated in an average of 6.7 cases per year from 2008 to 2010. The total number of cases caused by these 2 organisms was significantly decreased owing to the introduction of vaccines.
The infected organ also changed. Bacterial meningitis was observed in 6 or 7 cases in 2008 or 2009. Subsequently, an average of only 2 cases per year were observed. The number of cases of bacterial pneumonia with bacteremia was also decreased.
In conclusion, the number of pediatric invasive bacterial infection cases was decreased markedly due to these 2 vaccines. However, we should be aware of non-vaccine serotype H. influenzae and S. pneumoniae, and pay careful attention to epidemiological surveillance.
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Case Report
Title
A Case of Haemophilus influenzae Type f Meningitis after Three Doses of Hib Vaccinations
Author
Asami Oohara1) Hiroyuki Shimizu1) Ryouki Hara1)2) Ryousuke Fukushima1) Takuma Sakon1) Tetsunori Funabiki1) Sachiko Takahashi3) Haruka Hishiki3) and Naruhiko Ishiwada4)
1)Children's Medical Center, Fujisawa City Hospital
2)Department of Pediatrics, Yokohama City University School of Medicine
3)Department of Pediatrics, Chiba University Graduate School of Medicine
4)Division of Control and Treatment of Infectious Diseases, Chiba Universal Hospital
Abstract
We report an immunocompetent 7-month-old girl with bacterial meningitis, who had previously been immunized with three doses of Haemophilus influenzae serotype b (Hib) conjugate vaccine and three doses of pneumococcal conjugate vaccine. She presented to the emergency department with fever and vomiting. Cerebrospinal fluid (CSF) examination revealed a leukocyte count of 2,288/mm3. She had a diagnosis of bacterial meningitis and treatment with an antimicrobial agent was started. Complications developed including a subdural and subarachnoid abscess, and she underwent a craniectomy. The blood culture grew Haemophilus influenzae, which was later identified as type f. She also had> 9 μg/ml of anti-polyribosyl ribitol phosphate (PRP) antibody titers, demonstrating she had developed anti-Hib immune responses in the acute phase. In Europe and the United States, where Hib conjugate vaccine was introduced in the 1980s, the incidence of the most virulent serotype, Hib, has decreased dramatically, whereas incidence of Haemophilus influenzae non-b, type a, type e, and type f, is increasing. In Japan, invasive disease caused by Haemophilus influenzae non-b is rare, but it is likely that following vaccination, the incidence of Haemophilus influenzae non-b will increase.
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Case Report
Title
A Boy with Intestinal Hemorrhage due to Helicobacter pylori Infection While Being Treated for Acute Kawasaki Disease
Author
Masami Ukiami1) Junpei Soumura1) Hironori Sagawa1) Ouki Furukawa1) Masao Nakagawa1) Satoko Ueba2) Kazuo Nonomura2) and Yoshihiro Takeuchi1)
1)Shiga University of Medical Science
2)Moriyama Municipal Hospital
Abstract
We describe a 4-year-old boy with Kawasaki disease and aneurysms in the coronary arteries, who presented serious intestinal hemorrhages from duodenal ulcers. The patient was treated with intravenous immunoglobulin administration (IVIG) and oral administration of flurbiprofen and prednisolone in the acute phase of Kawasaki disease, and then with anticoagulant agents including warfarin potassium and heparin sodium for the prevention of thrombosis in the coronary aneurysms. The ulcerative bleeding was probably induced by these agents, and the intestinal ulcers were surgically treated. A stool examination detected Helicobacter pylori, which appeared to have caused the intestinal ulcers.
This case strongly suggests that we have to pay attention to H. pylori infection and subsequent ulcerative bleeding even in infants, especially when treating with aspirin, prednisolone or some anticoagulant agents in the acute phase of Kawasaki disease.
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Case Report
Title
Eltrombopag Olamine for the Treatment of Pediatric Idiopathic Thrombocytopenic Purpura
Author
Toshiyuki Takahashi1) Ryoji Kobayashi2) Kenji Kishimoto2) Hirozumi Sano2) Daisuke Suzuki2) Kazue Yasuda2) Yasuto Suzuki1) Masanori Nakanishi1) Tetsuro Nagashima1) and Kunihiko Kobayashi2)
1)Department of Pediatrics, Kushiro Red Cross Hospital
2)Department of Pediatrics, Sapporo Hokuyu Hospital
Abstract
Idiopathic thrombocytopenic pupura (ITP) is generally regarded as a disorder characterized by autoantibody-induced platelet destruction and impairment in platelet production. Therefore, thrombopoietin (TPO) receptor agonist is thought to be effective against ITP but, reports on TPO receptor simulator for pediatric patients with ITP are very few. We report 5 cases with chronic ITP treated with TPO receptor agonist, eltrombopag olamine (eltrombopag).
Five cases with chronic ITP were 5 to 18 years old, 2 were boys and 3 were girls. All patients had been treated with many medicines including prednisolone and cephalantin. In 4 eligible patients, eltrombopag were effective. In all 3 adolescent patients, they were able to discontinue all drugs other than eltrombopag One infantile patient was able to reduce the dose of prednisolone. Her obesity and growthimproved. Unfortunately, one patient, a 14-year-old boy complained of headache and did not take eltrombopag constantly, preventing the evaluation of effectiveness of eltrombopag in this patient. Eltrombopag is thought to be effective to chronic ITP in children. Although long term side effect of eltrombopag is not clear, this drug can be useful for the treatment of chronic ITP in children.
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Case Report
Title
A Two-month-old Infant with Croup-like Symptoms Caused by a Cerebellar Tumor
Author
Yumi Terakawa1) Yuichi Sugahara1) Eri Hayashi1) Kenji Bando1) Minoru Asada1) Seiko Murakami1) Ayako Konishi2) and Makoto Enomoto3)
1)Department of Pediatrics, Izumi Municipal Hospital
2)Department of Pediatrics, Graduate School of Medicine, Osaka City University
3)Department of Pediatrics, Osaka City Sumiyoshi Hospital
Abstract
A 2-month-old female infant was hospitalized for suspected croup syndrome with inspiratory stridor, trachyphonia, and retractive breathing. Her symptoms showed temporary improvement with administration of dexamethasone, but adrenergic aspiration was not effective. On the third day after admission, she developed apnea and bradycardia. Head computed tomography showed dilation of the cerebral ventricles and a tumor extending from the left vermis into the hemisphere. She was transferred to another hospital for neurosurgery.
Several diseases can cause inspiratory stridor. Laryngeal fiberscopy, ultrasonography, upper digestive tract contrast, and cervical computed tomography are useful for making the differential diagnosis. In this case, vocal cord palsy caused by a brain cerebral tumor had produced inspiratory stridor.
When treating inspiratory stridor patients with atypical presentations and those in which therapy is not effective, rare diseases should be included in the differential diagnosis.
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