Pediatrics international : official journal of the Japan Pediatric Society
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Hereditary sensory and autonomic neuropathy (HSAN) is a group of genetic disorders involving varying sensory and autonomic dysfunction. HSAN types IV and V are characterized by congenital generalized loss of pain and thermal sensation. HSAN type IV is additionally accompanied by decreased sweating and intellectual disability. ⋯ Therefore, we produced the "Guideline of Total Management and Care for Congenital Insensitivity to Pain (Ver.1)" in 2012, to provide information for medical specialists based on our knowledge and experience. This guideline includes medical issues, as well as descriptions of social participation and welfare. This review outlines the situation of HSAN types IV and V in Japan, and the recommendations of treatment and care for patients, mostly based on research conducted by the Japanese Research Group.
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Current guidelines for pediatric cardiopulmonary resuscitation suggest that supraglottic devices are alternatives for tracheal intubation with minimal interruption of chest compression. We examined the utility of three intubating supraglottic devices, air-Q® (air-Q), Ambu® aura-i (aura-i), and i-gel® (i-gel), utilizing manikin simulation. Twenty-two novice physicians performed securing of airway on an infant manikin with the three devices. ⋯ The insertion time with air-Q and aura-i did not extend significantly for chest compression. In contrast, the insertion time with i-gel was significantly extended in chest compression (P < 0.05). Air-Q and aura-i are more useful for airway management during chest compression than i-gel.
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Pott's puffy tumor (PPT) is a rare complication of sinusitis characterized by subperiosteal abscess and osteomyelitis of the frontal bone. Early diagnosis and treatment is vital before it causes intracranial complications such as subdural empyema or brain abscess. Herein we describe the case of a 12-year-old patient who developed preseptal cellulitis and PPT, and was successfully treated with abscess drainage, sinus surgery and long-term antibiotic therapy.
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Case Reports
Concomitant percutaneous aortic embolectomy and excision of left atrial myxoma in a child.
Cardiac myxoma is rare in children and often associated with cerebrovascular events. Reported herein is the case of a 9-year-old boy who presented with acute obstruction of the aortic bifurcation associated with a huge left atrial (LA) myxoma, thereby leading to acute ischemia of the lower extremities. Immediate surgical removal of the LA myxoma with concomitant percutaneous embolectomy using a Forgathy catheter was done, with relief of ischemic symptoms. Cardiac myxoma should always be considered in the case of acute embolic events in children, given that it needs immediate intervention to prevent further complications.
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Case Reports
Successful treatment of relapsing autoimmune thrombotic thrombocytopenic purpura with rituximab.
Thrombotic thrombocytopenic purpura (TTP) is a rare but life-threatening condition characterized by thrombotic microangiopathy. The standard treatment for TTP is plasmapheresis. ⋯ TTP is rarer in the pediatric age group and the use of rituximab in children with TTP is limited. Reported herein is the successful treatment of relapsing autoimmune TTP with rituximab in a 12-year-old girl.