• Pediatric Infection and Vaccine
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• 제14권1호
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• pp.116-119
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• 2007

범저감마글로불린혈증은 X 염색체에 의해 유전되는 질환으로 주로 1세 이전에 진단이 가능하지만, 진단이 늦어질 경우 반복된 감염 및 중증 감염으로 인한 심각한 후유증을 남길 수 있으므로 조기 진단과 치료가 매우 중요하다. 증상이 다양하게 나타날 수 있으므로 진단을 위한 각별한 주의가 요구된다. 저자들은 반복적이고 심각한 감염의 병력에도 8세가 돼서야 범저감마글로불린혈증 진단을 받은 환아와 그로 인해 함께 진단 받은 친형의 사례를 경험하였기에 보고하는 바이다.

• 대한두개안면성형외과학회지
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• 제17권3호
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• pp.103-110
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• 2016

Fractures of frontal sinus account for 5%-12% of all fractures of facial skeleton. Inadequately treated frontal sinus injuries may result in malposition of sinus structures, as well as subsequent distortion of the overlying soft tissue. Such inappropriate treatment can result in aesthetic complaints (contour deformity) as well as medical complications (recurrent sinusitis, mucocele or mucopyocele, osteomyelitis of the frontal bone, meningitis, encephalitis, brain abscess or thrombosis of the cavernous sinus) with potentially fatal outcomes. Frontal contour deformity warrants surgical intervention. Although deformities should be corrected by the deficiency in tissue type, skin and soft tissue correction is considered better choice than bone surgery because of minimal invasiveness. Development of infection in the postoperative period requires all secondary operations to be delayed, pending the resolution of infectious symptoms. The anterior cranial fossa must be isolated from the nasal cavity to prevent infectious complications. Because most of the complications are related to infection, frontal sinus fractures require extensive surgical debridement and adequate restructuring of the anatomy. The authors suggest surgeons to be familiar with various methods of treatment available in the prevention and management of complications following frontal sinus fractures, which is helpful in making the proper decision for secondary frontal sinus fracture surgery.

• Clinical and Experimental Pediatrics
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• 제57권9호
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• pp.384-395
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• 2014

Febrile seizure (FS) is the most common seizure disorder of childhood, and occurs in an age-related manner. FS are classified into simple and complex. FS has a multifactorial inheritance, suggesting that both genetic and environmental factors are causative. Various animal models have elucidated the pathophysiological mechanisms of FS. Risk factors for a first FS are a family history of the disorder and a developmental delay. Risk factors for recurrent FS are a family history, age below 18 months at seizure onset, maximum temperature, and duration of fever. Risk factors for subsequent development of epilepsy are neurodevelopmental abnormality and complex FS. Clinicians evaluating children after a simple FS should concentrate on identifying the cause of the child's fever. Meningitis should be considered in the differential diagnosis for any febrile child. A simple FS does not usually require further evaluation such as ordering electroencephalography, neuroimaging, or other studies. Treatment is acute rescue therapy for prolonged FS. Antipyretics are not proven to reduce the recurrence risk for FS. Some evidence shows that both intermittent therapy with oral/rectal diazepam and continuous prophylaxis with oral phenobarbital or valproate are effective in reducing the risk of recurrence, but there is no evidence that these medications reduce the risk of subsequent epilepsy. Vaccine-induced FS is a rare event that does not lead to deleterious outcomes, but could affect patient and physician attitudes toward the safety of vaccination.

• Imaging Science in Dentistry
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• 제51권4호
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• pp.351-361
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• 2021

Purpose: The clivus is a region in the anterior section of the occipital bone that is commonly imaged on large-volume cone-beam computed tomography (CBCT). There have been several reports of incidental clivus variations and certain pathological entities that have been attributed to the variations. This study aimed to evaluate the effects of these variations within the scope of dentistry. Materials and Methods: Medical databases (PubMed, Scopus, and Web of Science) were searched using a controlled vocabulary (clival anomalies, cone-beam CT, canalis basilaris medianus, fossa navicularis magna, clival variation). The search was limited to English language, humans, and studies published in the last 25 years. The articles were exported into RefWorks^® and duplicates were removed. The remaining articles were screened and reviewed for supporting information on variations of the clivus on CBCT imaging. Results: Canalis basilaris medianus and fossa navicularis magna were the most common anomalies noted. Many of these variations were asymptomatic, with most patients unaware of the anomaly. In certain cases, associated pathologies ranged from developmental (Tornwaldt cyst), to acquired (recurrent meningitis). While no distinct pathognomonic aspects were noted, there were unique patterns of radiographic diagnosis and treatment modalities. Most patients had a normal course of follow-up. Conclusion: Interpretation of CBCT volumes is a skill every dentist must possess. When reviewing large-volume CBCT scans, the clinician should be able to distinguish pathology from normal anatomic variations within the skull base. The majority of clivus variations are asymptomatic and will remain undetected unless incidentally noted on radiographic examinations.

• 대한두개안면성형외과학회지
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• 제11권2호
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• pp.95-98
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• 2010

Purpose: CSF (Cerebrospinal fluid) leakage is the most common complication of neurosurgery. Early management with conservative care or surgery must be followed appropriately due to the increased risk of lethal complications, such as meningitis. We report a case of intractable CSF leakage that occurred after a cerebellar tumor resection, which was treated successfully. Methods: A 53-year old male consulted our department for continuous CSF leakage for 3 months after having received conservative care and lumbar drainage. CSF collection was observed in the dead space of the posterior fossa after a cerebellar tumor resection and postoperative radiotherapy. Using a free latissimus dorsi muscle flap, the dead space within the skull was filled and the defects were covered successfully. Results: At 6 weeks after surgery, the follow-up MRI and CT revealed proper coverage and filling in the area where cerebellar tumor had been removed. No CSF leakage was observed at the postoperative 3 month follow-up. Conclusion: Recurrent CSF leakage was treated after cerebellar tumor resection with a relatively satisfactory result. In terms of the patient's treatment, much better results can be achieved by performing dead space filling using a flap with a sufficient size, in addition to coverage of the defects of the dura.

• Clinical and Experimental Pediatrics
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• 제54권6호
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• pp.234-240
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• 2011

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system (CNS) that typically presents as a monophasic disorder associated with multifocal neurologic symptoms and encephalopathy. ADEM is considered an autoimmune disorder that is triggered by an environmental stimulus in genetically susceptible individuals. The diagnosis of ADEM is based on clinical and radiological features. Most children with ADEM initially present with fever, meningeal signs, and acute encephalopathy. The level of consciousness ranges from lethargy to frank coma. Deep and subcortical white-matter lesions and gray-matter lesions such as thalami and basal ganglia on magnetic resonance imaging (MRI) are associated with ADEM. In a child who presents with signs of encephalitis, bacterial and viral meningitis or encephalitis must be ruled out. Sequential MRI is required to confirm the diagnosis of ADEM, as relapses with the appearance of new lesions on MRI may suggest either multiphasic ADEM or multiple sclerosis (MS). Pediatric MS, defined as onset of MS before the age of 16, is being increasingly recognized. MS is characterized by recurrent episodes of demyelination in the CNS separated in space and time. The McDonald criteria for diagnosis of MS include evidence from MRI and allow the clinician to make a diagnosis of clinically definite MS on the basis of the interval preceding the development of new white matter lesions, even in the absence of new clinical findings. The most important alternative diagnosis to MS is ADEM. At the initial presentation, the 2 disorders cannot be distinguished with certainty. Therefore, prolonged follow-up is needed to establish a diagnosis.

• Clinical and Experimental Pediatrics
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• 제52권6호
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• pp.721-724
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• 2009

수막구균(Meningococcus) 질환은 보체계의 이상과 관련이 있을 수 있다. 보체 7번은 5개의 말단 보체 단백질(terminal complement protein) 중 하나로 이것이 결핍되면 보체의 세포를 용해하는 작용을 잃게 되어 반복적인 감염, 특히 수막구균 감염에 대한 감수성이 증가한다. 2003년 9월 고열, 하지 동통, 두통과 점상 출혈로 외래에 내원한 11세 된 여자 환자가 입원 후 급격히 혼수 상태로 빠졌으나 즉각적인 치료로 결국 완전히 회복되었다. 환자의 최종 진단은 수막구균성 패혈증과 관절염이었다. 환자의 오빠도 비슷한 세균성 뇌수막염 가족력이 있어 저자들은 보체계 검사와 유전자 돌연변이(gene mutation)에 대해 분석하였고, 환자와 환자의 오빠는 보체 7번 유전자의 exon 4에 G394C에 돌연변이가 있는 선천성 보체 7번 결핍 환자였고, 아빠는 보인자로 밝혀졌다. 저자들은 예방적으로 4가 수막구균 백신(tetravalent polysaccharide meningococcal vaccine, $Menomune^{(R)}$ A/C/Y/W-135, Aventis Pasteur, Canada)을 3년마다 접종하기로 하였고 2004년 10월에 처음으로 접종하였다. 그러나 2006년 9월 오빠는 급성 세균성 뇌수막염(meningoencephalitis)으로 사망하였다. 이에 저자들은 2년마다 예방적 접종을 하기로 하였고, 환자는 2008년 9월에 3번째로 접종하였으며 16세 된 환자는 현재까지 건강한 상태이다. 저자들은 수막구균 감염과 보체 7번 유전자 네번째 intron의 3' 말단 splice acceptor 위치에 G to T 돌연변이(g.IVS4-1G> T)가 있는 선천성 보체 7번 결핍을 가진 한국인 한 가족을 보고하는 바이다.