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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.117, No.9, September 2013
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Original Article
Title
Gender Dysphoria in Children with Disorders of Sexual Development in Japan
Author
Kenji Ohyama1) Maki Fukami2) and Tsutomu Ogata3)
1)Department of Pediatrics, Faculty of Medicine, University of Yamanashi
2)Department of Molecular Endocrinology, National Research Institute for Child Health and Development
3)Department of Pediatrics, Hamamatsu University School of Medicine
Abstract
The aim of this study was to examine patients with different forms of disorder of sexual development (DSD)(i.e., complete androgen insensitivity, 5-α reductase deficiency, StAR disorders with 46, XY and 21-hydroxylase deficiency, POR deficiency with 46, XX) with regard to gender identity in prepuberty. Pediatric endocrinologists and urologists treating patients with DSD filled out questionnaires on gender identity (DSM-IV-TR) and external genitalia (Prader's stage). Twenty-six patients with androgen insensitivity were raised as females and their gender identities were female. Of 7 patients with 5-α reductase deficiency, 2 were raised as females but did not show a clear female gender identity. The gender identity of the other 5 patients raised as males was male. Male gender identity was suspected in only 5 of 127 patients with 21-hydroylase deficiency raised as females. The gender identity in all of 7 POR-deficient and 13 StAR disorder patients raised as females was female. These results suggest that the majority of 46, XX patients with 21-hydroylase deficiency showed no gender identity problems, whereas in patients with 5-α reductase deficiency, the assumed importance of prenatal androgen exposure for brain masculinization must be considered when raised as females.
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Original Article
Title
An Outbreak of Enterohemorrhagic Escherichia Coli O111 Associated with the Consumption of Raw Beef in Toyama, Japan
Author
Hiromichi Taneichi1) Michio Konishi2) Noboru Igarashi3) Hisashi Kaneda4) Hiroyoshi Matsukura5) Kazumasa Ogura6) Mondo Kuroda7) Akihiro Yachie7) and Toshio Miyawaki1)
1)Department of Pediatrics, University of Toyama
2)Department of Pediatrics, Tonami General Hospital
3)Department of Pediatrics, Toyama Prefectural Central Hospital
4)Department of Pediatrics, Toyama City Hospital
5)Department of Pediatrics, Saiseikai Toyama Hospital
6)Department of Pediatrics, Fukui Red Cross Hospital
7)Department of Pediatrics, Kanazawa University
Abstract
In April 2011, a sudden outbreak of enterohemorrhagic Escherichia coli (EHEC) O111 infection associated with the consumption of a Korean raw beef dish called yukhoe occurred in Toyama and its outskirts. A total of 181 affected individuals were identified, with 5 deaths, including 3 children. The outbreak was characterized by a high incidence of the development of encephalopathy, characterized by neurological involvement due to EHEC infection with abnormal findings on computed tomography or magnetic resonance imaging. The cause of death in each case was considered to be encephalopathy. Regarding children with EHEC O111 infection, 20 patients between 1 and 14 years old have been reported. Among these patients, hemolytic uremic syndrome (HUS) was found in 10 patients (50%), 8 (80%) of whom presented with encephalopathy. Following the death of 3 children with encephalopathy in the early stages of the outbreak, aggressive treatment for acute encephalopathy (e.g., methylprednisolone pulse therapy) was introduced, resulting in no further fatalities. To improve the outcomes of EHEC infection, scrupulous assessment for encephalopathy in patients is important. This outbreak of EHEC O111 infection suggests that the establishment of an HUS surveillance system is necessary in Japan.
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Original Article
Title
Efficacy and Safety of Co-administration of Live Attenuated Varicella Vaccine and Measles-rubella (MR) Vaccine
Author
Masahiro Ohashi1) Yoshiki Kawamura1) Yoshizo Asano1)8) Yuji Matsumoto2) Tomochika Kato2) Naoko Nishimura3) Takao Ozaki3) Shigeru Suga4) Toshiaki Ihara4) Hitoshi Ochiai5) Koichi Takeuchi6) Koichi Baba7) and Tetsushi Yoshikawa1)
1)Department of Pediatrics, Fujita Health University School of Medicine
2)Department of Pediatrics, Toyokawa Municipal Hospital
3)Department of Pediatrics, Konan Kosei Hospital
4)Department of Pediatrics, Mie National Hospital
5)Ochiai Pediatric Clinic
6)Takeuchi Pediatric Clinic
7)Baba Pediatric Clinic
8)Zambia Project Research Center for Zoonosis Control, Hokkaido University
Abstract
The aim of this study was to elucidate the efficacy and safety of the co-administration of a varicella-zoster virus (VZV) vaccine and the measles-rubella (MR) vaccine. One hundred seventy-six children who had no history of varicella were enrolled. Eighty-two children received simultaneous administration of the VZV vaccine and MR vaccine (VZV/MR). As controls, 42 age-matched children received the VZV vaccine only and 51 age-matched children received the MR vaccine only. Serum samples were drawn before vaccination and 4 weeks after vaccination. Antibody titers were measured by immune adherence hemagglutination (VZV), glycoprotein antigen-based enzyme-linked immunosorbent assays (VZV), neutralization tests (measles), and hemagglutination inhibition (rubella). Additionally, cell-mediated immunity (CMI) against VZV was tested in 7 children after vaccination. There were no significant differences in the seroconversion rates and mean antibody titers between the VZV/MR simultaneous vaccination group and the VZV only or MR only vaccination group. Of the 7 (71.4%) cases, 5 had measurable CMI. The incidence of breakthrough varicella in the 1 year following vaccination was 11%. At 1 year after the initial vaccination, a second dose of the VZV vaccine was administered to 9 children without breakthrough varicella, and a booster effect was observed in all subjects. VZV vaccination did not affect the antibody responses induced by the co-administration of the VZV and MR vaccines, and there were no severe side effects. Furthermore, the second dose of VZV vaccine had booster activity.
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Original Article
Title
Effectiveness of the Bubble CPAP System for Severe Respiratory Syncytial Virus Infection in Early Infancy
Author
Tomohito Fujioka1) Yoshiaki Yano1) Munemitsu Koizumi2) Koji Takemoto1) and Eiichi Ishii3)
1)Division of Pediatrics, Sumitomo Bessi Hospital
2)Division of Pediatrics, Ehime Prefectural Central Hospital
3)Department of Pediatrics, Ehime University Graduate School of Medicine
Abstract
The bubble continuous positive airway pressure (CPAP) system is used as a non-invasive positive pressure ventilation for the newborn with respiratory distress. We treated 3 patients in early infancy who suffered from severe respiratory syncytial virus (RSV) infection with the bubble CPAP system. In all patients, various type of respiratory distress, such as tachypnea, retraction, apnea, and low SpO2, were rapidly improved without any complications. These findings indicate that the bubble CPAP system is effective and safe for severe RSV infection in infants.
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Original Article
Title
Late Effects of Childhood Cancer Survivors Who Decided to Stop Follow-up Physician Visits P (Part 1, 2 etc, May Be Considered a Single Patient)
Author
Naoko Maeda1) Keizo Horibe1)2) Koji Kato3) and Seiji Kojima4)
1)Department of Pediatrics, National Hospital Organization Nagoya Medical Center
2)Clinical Research Center, National Hospital Organization Nagoya Medical Center
3)Division of Pediatric Hematology Oncology, Japanese Red Cross Nagoya First Hospital
4)Department of Pediatrics, Nagoya University Graduate School of Medicine
Abstract
Background In recent years, medical care for the late effects of childhood cancer and the related long-term follow-up have increasingly attracted attention. The present study compared the health status of childhood cancer survivors who stopped follow-up physician visits with the health status of those who continued regular follow-up visits.
Methods Subjects included 88 childhood cancer survivors who stopped follow-up physician visits (non-attenders) and 109 childhood cancer survivors who continued regular follow-up physician visits (attenders). Both groups were given a diagnosis and treated in 3 hospitals in Nagoya between 1975 and 2001. We conducted a survey of the current health status of 88 non-attenders using a mailed questionnaire and compared the results with those of attenders. The median age at the time of the survey of the 88 non-attenders was 24 years and that of the 109 attenders was 20 years. The median time from diagnosis was 17 years for the non-attenders and 14 years for the attenders. Sixty-two of the 88 non-attenders and 82 of the 109 attenders were affected with hematological malignancies and solid tumors were diagnosed in 26 of 88 non-attenders and 27 of 109 attenders.
Results Non-attenders experienced hypogonadism, hearing impairment, and chronic constipation (11.4% each), and attenders experienced hypogonadism (28.4%), short stature (21.1%), and osteoporosis (13.8%).
From the questionnaires, 21% of non-attenders reported having more than one health problem. From the medical records, 38% of attenders had more than one health problem. Non-attenders wished for a place for health consultation to be established, introduction of a suitable medical institution, notices of follow-up visits to be sent, information about late effects, a written clinical summary to be provided, subsidies for the cost of regular medical check-ups and treatment for late effects, and a place for information exchange with other survivors.
Conclusions More than half of non-attenders had some health problem and were concerned about late effects.
Pediatric oncologists should provide a written clinical summary and information of possible late effects to survivors, their families and adult health care providers and support their health care comprehensively for an ideal transition to adult-focused risk based care.
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Original Article
Title
Effectiveness of Rescue Therapy with Oral Tacrolimus for Intractable Ulcerative Colitis in Children
Author
Tadahiro Yanagi Tatsuki Mizuochi Yoshitaka Seki Yoshiyuki Kagiyama Kosuke Ushijima Akihiko Kimura and Toyojiro Matsuishi
The Department of Pediatrics and Child Health, Kurume University School of Medicine
Abstract
Purpose: Pediatric patients with ulcerative colitis (UC) generally show a more extensive and severe disease course than adult patients. Morever, the prevalence of steroid-resistant and steroid-dependent colitis is higher in pediatric patients than in adult patients. The effectiveness of tacrolimus (FK506) for refractory UC in adult patients has been described. Here, we report the effectiveness of FK506 for pediatric UC.
Methods: We retrospectively evaluated 5 patients with intractable UC who were treated with oral tacrolimus at our hospital. The initial oral dose of tacrolimus ranged from 0.05 to 0.1 mg/kg per day given twice daily. The initial target plasma trough level was 10-15 ng/ml, and then the doses were adjusted to achieve plasma levels between 5 and 10 ng/ml as maintenance dose. In addition, we evaluated clinical parameters, plasma trough level, the number of days and measurement times to reach the target plasma trough level, remission, steroid sparing, adverse events, and the clinical course after administration.
Results: Rescue therapy with oral tacrolimus was effective in 3 male and 2 female patients. The patients' age ranged from 7 to 14 years and 6 months. Two patients were steroid-dependent, and 3 patients were steroid-resistant. The initial tacrolimus dose was 0.07-0.12 mg/kg/day. The tacrolimus induction dose was 0.13-0.4 mg/kg/day, which achieved a mean plasma trough level of 5.7-17.4 ng/ml. The numbers of days to reach the target plasma trough level were 6-15 days, which was measured by monitoring for 2-7 times. The tacrolimus dose used at maintenance was 0.08-0.4 mg/kg/day, which achieved a mean plasma trough level of 5.7-9.3 ng/ml. All cases achieved remission and were withdrawn from steroid therapy. Three cases underwent colectomy after 8, 14, and 16 months. Adverse events included all cases of hypomagnesemia and 1 case of headache.
Conclusion: FK506 was found to be effective for the rescue induction therapy of intractable UC in children, which was evaluated by the regular monitoring of its trough level.
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Original Article
Title
A Case of Central Diabetes Insipidus Possibly due to Lymphocytic Infundibuloneurohypophysitis
Author
Kenji Kondo1) Yuta Sasaoka1) Atsuo Togashi1) Yoshiyuki Sakai1) Minami Yoda1) Shin Hashimoto2) Hotaka Kamasaki3) and Hiroyuki Tsutsumi3)
1)Department of Pediatrics, Hakodate Municipal Hospital
2)Department of Pediatrics, Tomakomai City Hospital
3)Department of Pediatrics, Sapporo Medical University
Abstract
We report a case of central diabetes insipidus (CDI) suspected to be caused by lymphocytic infundibuloneurohypophysitis based on temporal changes in the pituitary MRI findings. A 16-month-old boy was brought to the pediatric clinic of our hospital because of polyuria and polydipsia. He had an uneventful perinatal history and a normal developmental history. On examination, urine osmolarity was decreased. Initial brain MRI showed an enlarged pituitary gland and pituitary stalk, with the absence of posterior pituitary hyperintensity on T1-weighted images. As markers of germinomas, his serum AFP and HCG levels were normal. The patient's condition was diagnosed as CDI from the result of a water restriction test. Polyuria and polydipsia improved after the nasal administration of DDAVP. Three months after the initial MRI, a second MRI revealed dramatic reversal of the previously enlarged pituitary gland and pituitary stalk. Although a pituitary biopsy was not performed, these improvements in the MRI findings suggested lymphocytic infundibuloneurohypophysitis as the cause of his CDI. Lymphocytic infundibuloneurohypophysitis is rare in children. To the best of our knowledge, our case is the youngest patient reported in the literature who had spontaneous regression of pituitary grand enlargement and suspected infundibuloneurohypophysitis. This case might have been misdiagnosed as idiopathic CDI, if not for the early repeat MRI showing rapid improvement of the pituitary lesion.
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Original Article
Title
Subtelomeric Screening by Multiplex Ligation Dependent Probe Amplification (MLPA) is Useful for the Diagnosis of Insulin-like Growth Factor 1 Receptor (IGF1R) Haploinsufficiency
Author
Tomotaka Kono1) Katsuya Aizu1) Kenji Shimizu2) Hirofumi Ohashi2) and Hiroshi Mochizuki1)
1)Division of Endocrinology and Metabolism, Saitama Children's Medical Center
2)Division of Genetics, Saitama Children's Medical Center
Abstract
We report the case of a 9-month-old Japanese girl with a short stature caused by haploinsufficiency of the insulin-like growth factor 1 receptor gene (IGF1R), as revealed using subtelomeric screening by multiplex ligation dependent probe amplification (MLPA). Small for gestational age (SGA) was diagnosed at birth. The infant showed persistent postnatal growth retardation, and her plasma IGF-1 standard deviation score was+0.60. These findings suggested that the patient was haploinsufficient for IGF1R. Moreover, she exhibited dysmorphic features and mild developmental retardation. Firstly, we determined the G-banded karyotype, and found that it was normal. Secondly, we performed subtelomeric screening by MLPA, which detected a terminal deletion of one copy of 15q, defined by FISH as a loss of 15q26.2-qter, including IGF1R. In conclusion, subtelomeric MLPA is a useful tool for detecting IGF1R haploinsufficiency due to the submicroscopic deletion of the terminal region of 15q.
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Original Article
Title
Helicobacter pylori Infection with Iron Deficiency Anemia and Growth Impairment
Author
Yoshiki Katsumi Osamu Otabe Fumihiro Matsui Satoshi Kidowaki Akiko Mibayashi Yusuke Tsuma Satoshi Sakaue and Hisato Ito
Department of Pediatrics, Nantan General Hospital
Abstract
Helicobacter pylori (HP) infection has been reported as a cause of iron deficiency anemia (IDA) and growth impairment. We describe a case of HP infection with IDA and growth impairment in an 11-year-old girl. The patient was admitted to our hospital for anemia, which was incidentally detected at another clinic. She complained of mild abdominal pain for the past 9 months. At 14 months, we detected growth impairment in the patient. We also detected IDA and a low serum somatomedin-C level for her age. The patient's condition was diagnosed as HP infection and nodular gastritis on the basis of the results of a urea breath test and upper gastrointestinal endoscopy, respectively. HP eradication therapy was performed for 1 week, and iron and cimetidine were subsequently administered. The HP infection, IDA and growth impairment were successfully treated. HP infection presents a wide variety of clinical presentations ranging from asymptomatic to gastrointestinal and even extradigestive disorders such as growth impairment. We conclude that HP infection should be considered even in children who had IDA or growth impairment without dyspeptic symptoms.
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Original Article
Title
A Case of Acute Encephalopathy with Febrile Convulsive Status Epilepticus Caused by HHV-6 Reactivation
Author
Masato Tamai1) Hiroyuki Namba1) Chikara Otsubo1) Yasuyuki Wada1) Masakatsu Kubo1) Hiroyuki Ida1) Nobuyuki Kobayashi2) and Kazuhiro Kondo2)
1)Department of Pediatrics, Jikei University Hospital
2)Department of Virology, Jikei University
Abstract
Many pathological conditions due to HHV-6 reactivation have been reported. Here, we report a case of encephalopathy accompanying HHV-6 reactivation in a 19-month-old girl. Her past medical history included febrile convulsion due to exanthema subitum at 8 months. After a sudden fever, refractory clonic convulsions started from the left upper limb and then became generalized. Artificial respiratory control and continuous administration of midazolam and thiamylal stopped the convulsions. On the third day after onset, administration of the above medications and respiratory control were stopped; the patient showed left incomplete paralysis, lowered articulation, and partial convulsions from the left upper limb to the face. At the time of hospital admission, blood serum HHV-6 IgM and HHV-6 IgG antibody titers (FA method) were elevated by less than 10-fold and 160-fold, respectively. No increases in antibody titers were observed in paired serum samples after 3 weeks. The HHV-6 DNA levels in the serum and spinal fluid were 38.6 copies/ml and 1,657.9 copies/ml, respectively. Based on serum antibody titers and PCR-DNA results, the patient's condition was diagnosed as acute encephalopathy with febrile convulsive status epilepticus accompanying reactivation of HHV-6 latently infecting the brain. HHV-6 DNA in the cerebrospinal fluid tested negative 1 month after onset, but the serum HHV-6 DNA level remained high (359.0 copies/ml). This was a case where acute encephalopathy accompanying HHV-6 reactivation could have been futher clarified, but we were unable to obtain a clear understanding of the patient's immunological abnormality.
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Original Article
Title
Exchange Blood Transfusion for Critical Pertussis in an Infant with Hyperleukocytosis
Author
Yasuhiro Shoji1) Tsukasa Higuchi2) Arata Oda1) Norimasa Kurosaka1) Yoko Akamine1) Masashi Kasai2) Kisei Minami2) Megumi Yoshimura3) Yoshiro Amano3) and Hikoro Matsui1)
1)Department of Pediatric Intensive Care, Nagano Children's Hospital
2)Department of General Pediatrics, Nagano Children's Hospital
3)Department of Pediatrics, Nagano Red Cross Hospital
Abstract
A 2-month-old girl was admitted to a local hospital with pneumonia and respiratory failure caused by pertussis. Her respiratory condition soon deteriorated, which necessitated endotracheal intubation and mechanical ventilation. She was referred to our intensive care unit with extreme leukocytosis (white blood cell count: 102,000/μl) and pulmonary hypertension. Exchange blood transfusion was performed for leukoreduction along with respiratory and cardiac support. The patient's cardiorespiratory condition gradually improved, and she was extubated successfully on day 22 of admission and discharged on day 36.
Severe infantile pertussis with pneumonia usually requires intensive care. In such cases, not only respiratory, but also cardiac, failure results in a poor prognosis. Hyperleukocytosis has been shown to be a major factor in pulmonary hypertension. We therefore advise that leukoreduction by exchange blood transfusion be performed in the early phase of critical pertussis.
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Original Article
Title
Two Cases of Aortico-left Ventricular Tunnel That Were Mistaken for Aortic Regurgitation
Author
Satoshi Hasegawa1) Jyunichi Ozawa1) Kenichi Watanabe1) Tsukasa Torigoe1) Hisanori Haniu1) Fujito Numano1) Hiroshi Suzuki1) Tooru Hirokawa2) and Makoto Uchiyama1)
1)Department of Pediatrics, Niigata Graduate School of Medical and Dental Sciences
2)Department of Pediatrics, Saiseikai Niigata Daini Hospital
Abstract
Aortico-left ventricular tunnel (ALVT) is a rare congenital abnormal paravalvular pathway between the aortic root and the left ventricular outflow tract. We report 2 cases of ALVT that were initially mistakenly diagnosed as aortic regurgitation. Case 1 was a 6-month-old girl who was found to have a heart murmur without any symptoms. We initially made a diagnosis of severe atypical aortic regurgitation (AR) with intraventricular septal aneurysm, and considered that aortic valve surgery would be necessary if she develops heart failure. However, we learned more about her condition, and confirmed that her cardiac anomaly was ALVT by echocardiography and computed tomography. She underwent ALVT repair at 1 year of age, and has then been asymptomatic and without medication. Case 2 was a 16-year-old girl with an initial diagnosis of AR. We promptly diagnosed her condition as ALVT by echocardiography when she was referred to our hospital. As she already had symptoms of heart failure, she therefore underwent ALVT repair immediately after her evaluation. However, postoperative echocardiography showed residual ALVT and mild AR. The clinical findings of ALVT are usually consistent with those of AR. Early ALVT repair is recommended even in infants in contrast to AR repair which is difficult at a young age. It is thus important to differentiate ALVT from AR.
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Original Article
Title
Trilateral Retinoblastoma with Intracranial Metastasis at Diagnosis
Author
Kohei Fukuoka and Takaaki Yanagisawa
Division of Pediatric Neuro-Oncology, Department of Neuro-Oncology/Neurosurgery, Saitama Medical University International Medical Center
Abstract
Trilateral retinoblastoma (TRB) is characterized by the presence of retinal tumors in 1 or both eyes as well as an asynchronous tumor in the pineal gland or the parasellar region of the brain. However, intracranial metastasis at diagnosis is rare. Metastasis to the central nervous system is incurable, although successful TRB treatment with intensive multimodal therapy has been reported recently. We present a case of TRB with intracranial metastasis at diagnosis in a 23-month-old girl. Her family detected leukocoria in her right eye at the age of 6 months. Although the patient was brought to her local doctor regarding this problem and for other conditions, her leukocoria was not investigated further for 17 months, until she developed exophthalmos periophthalmitis. Brain CT and MRI showed intraocular lesions with calcification in both eyes, a pineal mass, and a solitary lesion in the right sylvian fissure; these lesions were intensely enhanced with gadolinium. Histopathological examination of the enucleated right eyeball showed tumor invasion of the retinoblastoma cells in the choroidal membrane and vitreous body, and massive tumor extension to the cut end of the optic nerve. Despite intensive chemotherapy, the patient died of tumor dissemination 8 months after diagnosis. This case suggests that both the medical personnel and the general population in Japan still require continuous and up-to-date educational programs to help improve their skills in the early diagnosis of TRB, as is often proposed in developing countries.
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Original Article
Title
Hypernatremic Dehydration and Hypoglycemic Encephalopathy in an Exclusively Breast-fed Infant: An Alarming Case for Severe Complication
Author
Atsushi Ohashi Shin-ichiro Sekiya Masato Hirabayashi Jun-ichi Nakashima Kenji Mine Yuichi Kuroyanagi Shoji Tuji Yo Kinoshita and Kazunari Kaneko
Department of Pediatrics, Kansai Medical University
Abstract
We report a neonate with severe breastfeeding-associated hypernatremia and hypoglycemic encephalopathy. She was transferred to our hospital at the age of 2 days because of convulsion and severe hypoglycemia. Laboratory data on her admission revealed hypernatremic dehydration, severe hypoglycemia, polycythemia, renal dysfunction, and jaundice. Characteristic findings of hypoglycemic encephalopathy were observed on brain MRI.
Based on these findings, the diagnosis of breast-feeding associated hypernatremia and hypoglycemic encephalopathy was made. Intensive fluid replacement therapy improved her condition and abnormalities in laboratory data. She discharged from our hospital at the age of 20.
In the past 2 decades, breastfeeding is being promoted worldwide including Japan. However, recent observations have postulated that misunderstanding in an exclusive breast-feeding may lead to hypernatremic dehydration and hypoglycemia as in our case, which can be fatal. The present case alarms that management of appropriate water and nutrition is mandatory if an exclusive breast-feeding infant demonstrates the weight loss more than physiological range, i.e, 10% of birthweight.
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Original Article
Title
Two Cases of Mediastinal Lymphangiomatosis That Required Intensive Care Management
Author
Taiki Haga1) Chiaki Toida1) Takashi Muguruma1) and Akihiro Fujino2)
1)Department of Intensive Care Medicine, National Center for Child Health and Development
2)Department of Surgery, National Center for Child Health and Development
Abstract
Mediastinal lymphangiomatosis is a disease with a poor prognosis in which lymphangioma occurs diffusely or at multiple sites in the lung, bone, and mediastinum. We encountered 2 cases of thoracic lymphangiomatosis that required intensive care management. It is difficult to diagnose mediastinal lymphangiomatosis because of the diverse and ambiguous symptoms and findings from imaging. In the presented cases, however, thoracic lymphangiomatosis was suspected from imaging findings as well as from a complication of chylothorax. A definitive diagnosis was made from the histopathological results, and specific treatment was commenced. However, both cases exhibited massive pleural effusion and respiration disorder as well as serious and diverse complications due to lung parenchymatous infiltration of the lymphangioma, thereby requiring strict intensive care management. In the treatment of lymphangiomatosis in children at the acute phase, intensive care management including breathing circulation at the acute phase is essential in addition to specific treatment.
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