A Case of the Hirayama Disease in Young Male Adolescent

염정숙,조재영,임재영,박찬후,우향옥,윤희상,박기종,곽병근,김영수 대한소아신경학회 2011 대한소아신경학회지 Vol.19 No.3

In the present study, we report muscular atrophy of the right distal upper extremity in a 14-year-old boy. The disease progressed insidiously for about 2 years, and during our first examination, he exhibited weakness and wasting in the right hand, and paresthesia on the C6-8 dermatomal area in the right upper extremity. Electromyography revealed neurogenic changes in atrophic muscles. Conduction velocity of the ulnar nerve of the affected hand was decreased. Magnetic resonance imaging (MRI) of the cervical spine in the neutral position revealed focal spinal cord atrophy and a small area of high signal intensity at C5-6 level. In the flexion-induced cervical spine MRI scan, the spinal cord was noticed to be compressed by the posterior dural sac with a forward shift and flow voids in the epidural space. All these parameters led to the diagnosis of Hirayama disease (HD). This is the first report of HD in Korea by pediatrician, even though it is characterized by juvenile onset.

The first pediatric case of tularemia in Korea: manifested with pneumonia and possible infective endocarditis

염정숙,Kyuyol Rhie,박지숙,서지현,박은실,임재영,박찬후,우향옥,윤희상 대한소아청소년과학회 2015 Clinical and Experimental Pediatrics (CEP) Vol.58 No.10

about tularemia can be embarrassing and could result in delayed treatment because of improper diagnosis. The diagnosis of tularemia is difficult, because the infections are rare and the clinical spectrum is broad. As only 1 adult case has been reported in Korea thus far, pediatricians in Korea may be unfamiliar with tularemia. We report our experience with a 14-year- old male adolescent with tularemia who presented with atypical pneumonia and possible infective endocarditis. Although the infectivity and mortality rates for tularemia are very high if left untreated, we did not suspect tularemia in this case until the incidental isolation of F. tularensis. The present case suggests that clinicians in Korea should be more aware of tularemia. This case also suggests that tularemia should be considered in undetermined cases of atypical pneumonia or acute febrile illness without local signs.

난소 종양의 임상적 양상을 보인 소아 복막 결핵

염정숙,김재희,조재민,이정희,박은실,서지현,임재영,박찬후,우향옥,윤희상,Yeom, Jung Sook,Kim, Jae Hui,Cho, Jae Min,Lee, Jeong Hee,Park, Eun Sil,Seo, Ji Hyun,Lim, Jae Young,Park, Chan Hoo,Woo, Hyang Ok,Youn, Hee Shang 대한소아소화기영양학회 2005 Pediatric gastroenterology, hepatology & nutrition Vol.8 No.2

복부 팽만과 복수, 복부 종괴 및 혈청 CA-125가 상승하는 경우 반드시 복막 결핵을 의심해야 하며 보다 정확하고 빠른 진단을 위해 복막 천자를 통한 염색 및 균 배양뿐 아니라 ADA, 중합 효소 연쇄 반응 및 진단적 복강경 검사 등이 필요하다. 저자들은 난소 종양의 임상 양상을 보인 5세 여아에서 복막 결핵을 진단한 예가 있어 문헌 고찰과 함께 보고하는 바이다. We report the case of a girl with peritoneal tuberculosis (Tb) who presented with mimicking features of ovarian cancer including massive ascites, elevation of CA-125 and ovarian masses. These ovarian masses cannot be ruled out malingnacy with image study. Gram stain, acid-fast stain, bacteriological and mycological culture of centrifuged ascites cells were negative. Tumor markers revealed an elevated CA-125 level of 112 U/mL and adenosine deaminase level were significantly high with 118 U/L. Diagnostic laparoscopy was done. The histology of the peritoneum and the ovary showed multiple granulomatous nodules with epithelioid cells and multi-nucleated giant cells. And there were no evidence of malignancy. Furthermore acid-fast bacilli (AFB) was identified in this specimen. Peritoneal Tb was difficult to diagnosis in our patient because it mimicked ovarian cancer. And this is the first case of abdominal Tb mimicking ovarian cancer in children in Korea.

선천성 횡격막 헤르니아로 오인된 좌측 횡격막 내장탈출 2예

염정숙,박지숙,박은실,서지현,임재영,박찬후,우향옥,정은정,조재민,윤희상,Yeom, Jung-Sook,Park, Ji-Sook,Park, Eun-Sil,Seo, Ji-Hyun,Lim, Jae-Young,Park, Chan-Hoo,Woo, Hyang-Ok,Jung, Eun-Jung,Jo, Jae-Min,Youn, Hee-Shang 대한소아소화기영양학회 2006 Pediatric gastroenterology, hepatology & nutrition Vol.9 No.1

선천성 횡격막 내장탈출은 드문 질환으로 산전 초음파로 횡격막 헤르니아와 감별하는 것은 쉽지 않다. 선천성 횡격막 내장탈출과 횡격막 헤르니아의 예후가 다르고 수술적 접근 방식이 다를 수 있어 정확한 감별 진단이 필요하다. 저자들은 산전 초음파 검사에서 선천성 횡격막 헤르니아로 진단되었으나 수술 시에 선천성 횡격막 내장탈출로 진단된 두 증례의 후향적 분석을 통하여 비록 출생 직후의 단순 흉부 사진으로는 두 질환의 감별 진단이 어려웠으나 연속적인 단순흉부사진에서 뚜렷하게 드러나는 병변 측 횡격막을 확인하는 경험을 하였다. 따라서 산전 초음파 검사에서 횡격막 헤르니아로 진단되었다 하더라도 출생 이후에 촬영한 연속적인 단순흉부사진의 주의 깊은 판독을 통한 재평가를 강조하고자 한다. Congenital diaphragmatic eventration is the abnormal elevation of the diaphragm into the thoracic cavity. Sometimes, it is not easy to differentiate congenital diaphragmatic eventration from diaphragmatic hernia by either prenatal sonography or postnatal chest radiography. However, differential diagnosis of both diseases is practical because of different prognosis and surgical approaches. Careful interpretation of postnatal serial chest X-rays is mandatory to differentiate between both diseases. We report two neonates with congenital diaphragmatic eventration of left diaphragm that initially misdiagnosed as diaphragmatic hernia by prenatal sonography and postnatal chest radiography.

소아 감염성 흉막삼출의 원인 분석

염정숙,배원태,박은실,서지현,임재영,박찬후,우향옥,윤희상 대한소아청소년과학회 2006 Clinical and Experimental Pediatrics (CEP) Vol.49 No.1

Purpose : This study was designed to document the etiologies and the characteristics of parapneumonic effusion in children. Methods : During a 17-year period from 1987 to 2004, parapneumonic effusion was confirmed in 86 children at Gyeongsang National University Hospital. The clinical records of these children were reviewed and radiological findings and laboratory data, especially results of thoracentesis, were analyzed retrospectively. Results : M. pneumoniae(34 subjects) was the most common pathogen at all over age, especially above 1-years-old. There were diagnosed with clinical characteristics and serologic tests. The 2nd most common pathogen revealed non tuberculous bacteria(14 subjects). A species of bacteria at no tuberculous bacteria revealed S. aureus(5), S. pneumoniae(3), P. aeroginosa(3), other staphylococcus (2), and K. pneumoniae(1). There were confirmed with sputum culture or pleural fluid culture or blood culture. S. aureus was most common pathogen in infants. The 3rd common pathogen was M. tuberculosis(7). There were confirmed with skin tuberculin tests and AFB stains. Another that was classified as a non bacteria was adenovirus(2). Complications of parapneumonic effusion such as pleural thickness occurred on M. tuberculosis(1). Non tuberculous bacteria, especially S. aureus revealed a serious predominance of polymorphocyte at pleural fluid, and lowest pleural pH and glucose, and highest pleural protein and LDH. Tuberculosis revealed high pleural protein and LDH. Conclusion : Age and chemistries of pleural fluid might be helpful in differentiating various etiologies of parapneumonic effusion. If there were suspicious of tuberculosis and non-tuberculous bacteria, more aggressive approaches were needed to prevent complication. 목 적 : 흉막삼출을 동반한 폐렴에서 빠른 원인병원체의 판단으로 조기진단과 적절한 치료로 후유증 발생을 최소화하는데 도움을 주는 자료를 얻기 위해 최근 경험한 감염성 흉막삼출의 원인병원체, 연도별 발생빈도 변화, 연령별 빈도 및 후유증 발생률을 알아보고자 하였다.방 법 : 1987년부터 2004년까지 17년간 경상대학교병원 소아과에 폐렴으로 입원한 환아 중 감염성 흉막삼출을 보인 86례를 대상자로 이들의 병록기록지를 후향적으로 조사하였다.결 과 : 총 대상자의 남녀 비율은 1.45 : 1(남 : 여=51 : 35명)로 남아가 많았으며, 평균 연령은 5.64세(±4.43)이었다. 발생빈도의 변화를 연도별로 분류해 보았을 때 평균 발생률은 감소를 보이지만 통계학적으로 의미 있는 변화를 보이지 않았다(r=-0.25, P=0.30). 총 대상자 86례 중 원인체가 밝혀진 경우는 57례(66.3 %)로 결핵 7례(8.2%), 비결핵 세균성 14례(16.3%), 미코플라스마 34례(39.5%), 기타 감염성 흉막삼출 2례(2.3%)였다. 나머지 29례(33.7%)는 원인불명 감염성 흉막삼출이었다. 비결핵 세균성 흉막삼출 14례를 병원균 별로 분류하면 황색포도알균이 5례(5.8 %)로 가장 많았고, 폐렴사슬알균이 3례(3.5%), 녹농균 3례(3.5 %), 기타 포도알균 2례(2.3%), 폐렴막대균 1례(1.2%)로 밝혀졌다. 미코플라스마 폐렴은 혈청검사를 통한 진단이 비교적 용이하고 후유증 동반율이 매우 낮고, 비결핵 세균성 흉막삼출에서 흉막액의 소견은 높은 백혈구수와 함께 중성구 우세를 보이며, 산도와 당이 매우 낮게 측정되고, 단백과 LDH가 매우 높게 나오는 특징을 보였다. 결핵성 흉막삼출 환자 1례에서 흉막비후의 후유증을 남겼는데, 결핵성 흉막삼출은 흉막액에 높은 백혈구수에 림프구와 중성구의 우세가 비슷하고, 단백과 LDH가 높게 나오는 특징을 보였다.

Mild encephalopathy with a reversible splenial lesion in a girl with acute pyelonephritis

염정숙,구정모,박지숙,서지현,박은실,임재영,우향옥,윤희상 대한소아청소년과학회 2018 Clinical and Experimental Pediatrics (CEP) Vol.61 No.2

We report the case of a 12-year-old girl who had mild encephalopathy with a reversible splenial lesion (MERS) associated with acutepyelonephritis caused by Escherichia coli. The patient was admitted with a high fever, and she was diagnosed with acute pyelonephritis based on pyuria and the results of urine culture, which detected cefotaxime-sensitive E. coli. Although intravenous cefotaxime and tobramycin were administered, her fever persisted and her C-reactive protein level increased to 307 mg/L. On day 3 of admission, she demonstrated abnormal neuropsychiatric symptoms, such as delirium, ataxia, and word salad. Magnetic resonance imaging (MRI) of the brain performed on day 4 showed marked hyperintensities in the bilateral corpus callosum and deep white matter on diffusion-weighted images, with corresponding diffusion restriction on apparent diffusion coefficient mapping. No abnormalities or pathogens were detected in the cerebrospinal fluid; however, lipopolysaccharides (LPS, endotoxin) were detected in plasma (41.6 pg/mL), associated with acute neurological deterioration. Her clinical condition gradually improved, and no neurological abnormalities were observed on day 6. Follow-up brain MRI performed 2 weeks later showed near-disappearance of the previously noted hyperintense lesions. In this patient, we first proved endotoxemia in a setting of MERS. The release of LPS following antibiotic administration might be related to the development of MERS in this patient. The possibility of MERS should be considered in patients who present with acute pyelonephritis and demonstrate delirious behavior.

White matter injury following rotavirus infection in neonates: new aspects to a forgotten entity, ‘fifth day fits’?

염정숙,박찬후 대한소아청소년과학회 2016 Clinical and Experimental Pediatrics (CEP) Vol.59 No.7

That rotavirus infection can cause neurological symptoms in young children has been well established. However, it is surprising why rotavirus infection has been overlooked as a cause of neonatal seizures for many years, despite significant research interest in neonatal rotavirus infection. Neonates are the age group most vulnerable to seizures, which are typically attributed to a wide range of causes. By contrast, because rotavirus infection is usually asymptomatic, it has been difficult to identify an association between this virus and neonatal seizures. The conventional wisdom has been that, although neonates are commonly infected with rotavirus, neurological complications are rare in this age. However, recent studies using diffusion-weighted imaging (DWI) have suggested a connection between rotavirus infection and neonatal seizures and that rotavirus infection can induce diffuse white matter injury without direct invasion of the central nervous system. The clinical features of white matter injury in rotavirus-infected neonates include the onset of seizures at days 4–6 of life in apparently healthy term infants. The recent findings seem to contradict the conventional wisdom. However, white matter injury might not be a completely new aspect of rotavirus infection in neonates, considering the forgotten clinical entity of neonatal seizures, ‘fifth day fits’. With increased use of DWI in neonatal seizures, we are just starting to understand connection between viral infection and white matter injury in neonates. In this review, we discuss the historical aspects of rotavirus infection and neonatal seizures. We also present the clinical features of white matter injury in neonatal rotavirus infection.

Kawasaki disease in infants

염정숙,우향옥,박지숙,박은실,서지현,윤희상 대한소아청소년과학회 2013 Clinical and Experimental Pediatrics (CEP) Vol.56 No.9

Kawasaki disease (KD) is an acute febrile illness that is the predominant cause of pediatric acquired heart disease in infants and young children. Because the diagnosis of KD depends on clinical manifestations,incomplete cases are difficult to diagnose, especially in infants younger than 1 year. Incomplete clinical manifestations in infants are related with the development of KD-associated coronary artery abnormalities. Because the diagnosis of infantile KD is difficult and complications are numerous, early suspicion and evaluation are necessary.

Signal change in hippocampus and current source of spikes in Panayiotopoulos syndrome

염정숙,김영수,박지숙,서지현,박은실,임재영,박찬후,우향옥,윤희상,권오영 대한소아청소년과학회 2012 Clinical and Experimental Pediatrics (CEP) Vol.55 No.2

A 4-year-old girl with Panayiotopoulos syndrome presented with a history of 4 prolonged autonomic seizures. The clinical features of her seizures included, in order of occurrence, blank staring, pallor, vomiting,hemi-clonic movement on the right side, and unresponsiveness. A brain magnetic resonance imaging (MRI) showed a slightly high T2 signal in the left hippocampus. Interictal electoencephalogram revealed spikes in the occipital area of the left hemisphere. We analyzed the current-source distribution of the spikes to examine the relationship between the current source and the high T2 signal. The current source of the occipital spikes was not only distributed in the occipital area of both cerebral hemispheres,but also extended to the posterior temporal area of the left hemisphere. These findings suggest that the left temporal lobe may be one of the hyperexcitable areas and form part of the epileptogenic area in this patient. We hypothesized that the high T2 signal in the left hippocampus of our patient may not have been an incidental lesion, but instead may be related to the underlying electroclinical diagnosis of Panayiotopoulos syndrome,and particularly seizure. This notion is important because an abnormal T2signal in the hippocampus may represent an acute stage of hippocampal injury, although there is no previous report of hippocampal pathology in Panayiotopoulos syndrome. Therefore, long-term observation and serial follow-up MRIs may be needed to confirm the clinical significance of the T2 signal change in the hippocampus of this patient.

소아 아급성 괴사성 림프절염의 임상적, 방사선학적, 면역조직화학적 소견

김현정,염정숙,박지숙,박은실,서지현,박찬후,우향옥,조재민,이정희,윤희상,임재영 대한소아청소년과학회 2008 Clinical and Experimental Pediatrics (CEP) Vol.51 No.11

Purpose:The cause of subacute necrotizing lymphadenitis, a rare disease in children, has not been completely clarified. This study was aimed to investigate the disease mechanism by examining clinical, radiologic, and immunohistochemical findings in children diagnosed with subacute necrotizing lymphadenitis after an excisional biopsy. Methods:We examined 19 lymph node tissue specimens from 17 children diagnosed with subacute necrotizing lymphadenitis at Gyeongsang National University Hospital from March, 1998 to July, 2006. A retrospective survey of the medical records was performed. CT findings were analyzed. Immunohistochemical staining was done on tissues obtained by excisional biopsy from all patients. Results:The patient's age ranged from 5 to 19 years (average age:11.8 years). The main symptoms included a neck mass (17/19), pain in the mass (6/17), and fever (12/19). The palpable lymph nodes were mostly cervical in location; the maximum diameter, which was measured radiologically, was less than 3 cm in all 10 cases. The masses were pathologically divided into proliferative, necrotic, and xanthomatous types. With immunohistochemical staining the masses were divided into lesion (L), perilesion (PL), and necrosis (N). The CD8 staining was stronger than the CD4 staining for all regions in three types. The CD4 staining intensity was mainly increased in the perilesion, and CD8 was mainly increased in the lesion. Conclusion:We compared the radiologic findings, clinical symptoms, and pathology to help understand the cause of disease in patients with subacute necrotizing lymphadenitis. (Korean J Pediatr 2008;51:1198-1204) Purpose:The cause of subacute necrotizing lymphadenitis, a rare disease in children, has not been completely clarified. This study was aimed to investigate the disease mechanism by examining clinical, radiologic, and immunohistochemical findings in children diagnosed with subacute necrotizing lymphadenitis after an excisional biopsy. Methods:We examined 19 lymph node tissue specimens from 17 children diagnosed with subacute necrotizing lymphadenitis at Gyeongsang National University Hospital from March, 1998 to July, 2006. A retrospective survey of the medical records was performed. CT findings were analyzed. Immunohistochemical staining was done on tissues obtained by excisional biopsy from all patients. Results:The patient's age ranged from 5 to 19 years (average age:11.8 years). The main symptoms included a neck mass (17/19), pain in the mass (6/17), and fever (12/19). The palpable lymph nodes were mostly cervical in location; the maximum diameter, which was measured radiologically, was less than 3 cm in all 10 cases. The masses were pathologically divided into proliferative, necrotic, and xanthomatous types. With immunohistochemical staining the masses were divided into lesion (L), perilesion (PL), and necrosis (N). The CD8 staining was stronger than the CD4 staining for all regions in three types. The CD4 staining intensity was mainly increased in the perilesion, and CD8 was mainly increased in the lesion. Conclusion:We compared the radiologic findings, clinical symptoms, and pathology to help understand the cause of disease in patients with subacute necrotizing lymphadenitis. (Korean J Pediatr 2008;51:1198-1204)