• Clinical and Experimental Pediatrics
• /
• v.49 no.8
• /
• pp.875-881
• /
• 2006

Purpose : The aims of this study were to analyze the clinical characteristics of children with Kikuchi's disease(KD) at a medical center and to investigate the etiologic role of human herpesvirus 8(HHV 8) or Epstein-Barr virus(EBV) in children with KD. Methods : Twenty six children who were diagnosed as KD between Jan. 1998 and Dec. 2005 were included. Medical records were reviewed on the clinical characteristics of children with KD. Follow up data were collected by chart review and telephone contact. Polymerase chain reaction(PCR) was performed in order to detect HHV 8 DNA, and in situ hybridization(ISH) was perfomed in order to detect EBV RNA from 20 lymph node tissues. Results : There were 15 girls and 11 boys with a mean age of 13 years. Posterior cervical lymph nodes were involved in 72 percent(18/25) of the patients. Extracervical lymphadenopathy was associated in one patient. Fever was an associated symptom in 31 percent(8/26) of the patients. Leukopenia was observed in six (46 percent) patients. The cervical lymphadenopathy usually resolved spontaneously within 6 months. Only one patient had a recurrence of lymphadenopathy with fever during follow-up. No children with KD in our series developed systemic lupus erythematosus. HHV 8 DNA was not amplified by nested PCR in any of the cases, and all cases were negative for EBV RNA by ISH. Conclusion : KD should be differentiated as a cause of cervical lymphadenopathy in children. HHV 8 and EBV may not play major causative roles in KD in children.

• Pediatric Infection and Vaccine
• /
• v.26 no.3
• /
• pp.179-187
• /
• 2019

Purpose: Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is a leading cause of periodic fever in children. This study describes the clinical characteristics of PFAPA syndrome in patients from a single center. Methods: Thirteen children diagnosed with PFAPA syndrome at Seoul National University Children's Hospital were included in this study. Retrospective medical chart reviews were performed. Results: Among the 13 patients, 8 (61.5%) were male. The median follow-up duration was 3.3 years (range, 10 months-8.3 years). The median age of periodic fever onset was 3 years (range, 1-6 years). All patients had at least 5 episodes of periodic fever and pharyngitis, managed with oral antibiotics, before diagnosis. The median occurrence of fever was every 3.9 weeks and lasted for 4.2 days. All patients had pharyngitis and 12 (92.3%) had cervical lymphadenitis. Blood tests were performed for 12 patients, and no patients had neutropenia. Both the C-reactive protein and erythrocyte sedimentation rate were elevated at medians of 4.5 mg/dL (range, 0.4-13.2 mg/dL) and 29 mm/hr (range, 16-49 mm/hr), respectively. Throat swab cultures and rapid streptococcal antigen tests were negative. Nine (69.2%) patients received oral prednisolone at a median dose of 0.8 mg/kg, and in 6 (66.7%) patients, fever resolved within a few hours. Three (23.1%) patients received tonsillectomy and adenoidectomy. Conclusions: PFAPA syndrome should be considered when a child presents with periodic fever along with aphthous stomatitis, pharyngitis, or cervical lymphadenitis. Glucocorticoid administration is effective for fever resolution and can reduce unnecessary use of antibiotics.

• Clinical and Experimental Pediatrics
• /
• v.51 no.9
• /
• pp.987-991
• /
• 2008

Purpose : Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis (HNL), is a self-limited disease characterized by cervical lymphadenopathy and fever. The etiology of KFD remains unknown; however, the self-limiting nature of HNL suggests the cause of this disease could be viral infection. For this reason, several viruses have been evaluated as possible etiologies of HNL, including Epstein-Barr virus (EBV), human herpesvirus 6 (HHV6), human herpesvirus 8 (HHV8), and cytomegalovirus (CMV). The aim of this study was to examine the relationship of EBV and HHV6 to HNL. Methods : Data pertaining to 51 cases with biopsy-confirmed HNL were collected between January 1999 and December 2005, from the Department of Pathology, College of Medicine, Pusan National University, Busan, Korea. The clinical records-including data regarding age, gender, duration of fever, and lymph node involvementwere reviewed retrospectively. The in situ hybridization (ISH) assay was performed by EBER PNA probe (Dako, Capinteria, CA, USA), and immunohistochemistry testing was performed with anti-HHV type 6 monoclonal antibodies (Chemicon, Temecula, CA, USA). Results : The HNL patients in this study were 24 males and 27 females, ranging in age from seven to 61 years (median: 25.9). ISH for EBV was positive in 8/51 (15.7%) biopsies, and immunohistochemistry for HHV6 was positive in 15/51 (29.4%) biopsies. Serologic analysis of EBV IgM was performed in 23 cases; only one patient was positive for EBV IgM and EBV ISH. Conclusion : Our study could not provide supportive evidence of a viral pathogenesis for HNL; therefore, cases of HNL may not have a dominant viral cause. However, some rare exceptional cases may have been caused by viral infection.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.7 no.1
• /
• pp.31-39
• /
• 2004

Purpose: Although acute nonspecific mesenteric lymphadenitis (ANML) is probably common cause of abdominal pain in children, which can be severe enough to be an abdominal emergency, the clinical features of mesenteric lymphadenitis are not clear. Also, a differential diagnosis with acute appendicitis (APPE) is indispensable to avoid serious complications. The clinical features of ANML were determined, and the risk factors for differential diagnosis with APPE were analyzed. Methods: Between November 2000 and May 2001, data from 26 patients (aged 1 to 11 years) with ANML and 21 patients (aged 2 to 13 years) with APPE were reviewed. ANML was defined as a cluster of five or more lymph nodes measuring 10 mm or greater in their longitudinal diameter in the right lower quadrant (RLQ) without an identifiable specific inflammatory process on the ultrasonographic examination. There were risk factors on patient's history, physical examination, and laboratory examination; the location of abdominal pain, abdominal rigidity, rebound tenderness, fever, nocturnal pain, the vomiting intensity, the diarrhea intensity, the symptom duration, and the peripheral blood leukocytes count. Results: Of the 26 ANML patients and 21 APPE patients, abdominal pain was noted on periumbilical (76.9% vs 14.2%), on RLQ (11.5% vs 71.4%), with abdomen rigidity (7.6% vs 80.9%), with rebound tenderness (0.0% vs 76.1%)(p<0.05), in the lower abdomen (11.5% vs 14.2%), and at night (80.8% vs 100.0%) (p>0.05). The clinical symptoms were vomiting (38.4% vs 90.4%), the vomiting intensity ($1.5{\pm}0.7$ [1~3]/day vs $4.5{\pm}2.9$ [1~10]/day), diarrhea (65.3% vs 28.5%) (p<0.05), and fever (61.5% vs 76.2%)(p>0.05). The period to the subsidence of abdominal pain in the ANMA patients was $2.5{\pm}0.5$ (2~3) days. The laboratory data showed a significant difference in the peripheral blood leukocytes count ($8,403{\pm}1,737[5,900{\sim}12,300]/mm^3\;vs\;15,471{\pm}3,749[5,400{\sim}20,800]/mm^3$)(p<0.05). Discriminant analysis between ANML and APPE showed that the independent discriminant factors were a vomiting intensity and the peripheral blood leukocytes count and the discriminant power was 95.7%. Conclusion: The clinical characteristics of ANML were abrupt onset of periumbilical pain without rigidity or rebound tenderness, a mild vomiting intensity, normal peripheral leukocytes count, and relatively short clinical course. If the abdominal pain persist for more than 3 days, and/or the vomiting intensity is more than 3 times/day, and/or the peripheral leukocytes count is over $13,500/mm^3$, abdominal ultrasonography is recommended to rule out APPE.

• Tuberculosis and Respiratory Diseases
• /
• v.70 no.5
• /
• pp.433-437
• /
• 2011

Sarcoidosis is a multisystemic disorder characterized by the presence of non-caseating granulomas in the involved organ. Tuberculosis is an infectious disease caused by $Mycobacterium$ $tuberculosis$ and is characterized by granuloma with caseous necrosis. The clinical and histological similarity between sarcoidosis and tuberculosis has stimulated research searching for an association between mycobacterium and sarcoidosis. We report a case of a 38-year-old male with sarcoidosis that developed soon after treatment of tuberculous lymphadenitis. He was diagnosed as tuberculous lymphadenitis by microbiological confirmation. He showed clinical improvement after treatment for tuberculosis. One year later, his chest radiography showed bilateral hilar enlargement with diffuse bilateral nodules. A noncaseating granuloma was confirmed by endobronchial ultrasound guided transbronchial needle aspiration and he was diagnosed with sarcoidosis. To our knowledge, this is the first report describing sarcoidosis after treatment of tuberculosis in South Korea.

• Childhood Kidney Diseases
• /
• v.16 no.2
• /
• pp.138-141
• /
• 2012

Rituximab, a chimeric anti-CD20 IgG1 monoclonal antibody, has been used as a rescue therapy for steroid-dependent or refractory nephrotic syndrome. However, the adverse effects of rituximab are yet to be investigated. We report a case of a 9-year-old boy with steroid-dependent nephrotic syndrome who developed Kikuchi-Fujimoto disease after several cycles of rituximab therapy. Kikuchi-Fujimoto disease is a benign, self-limited necrotizing histiocytic lymphadenitis of unknown etiology. In the present case, Kikuchi-Fujimoto disease developed when the peripheral blood B-cell count of the patient was at nadir, and the lesion regressed slowly but spontaneously after recovery of the B-cell count. To our knowledge, although the pathologic diagnosis of Kikuchi-Fujimoto disease was unavailable, this is the first report of Kikuchi-Fujimoto disease with clinical diagnosis as a possible adverse effect of rituximab.

• Korean Journal of Head & Neck Oncology
• /
• v.15 no.2
• /
• pp.217-221
• /
• 1999

Background and Objectives: Subacute necrotizing lymphadenitis or Kikuchi's disease has now become recognized in many parts of the world as a well-defined clinicopathologic entity. It typically affects cervical lymph nodes of young women, and subsides spontaneously within a few months without recurrences. The purpose of this study is to report the clinicopathologic finding, radiologic finding and many laboratory tests to elaborate the criteria that are useful in distinguishing this entity from inflammatory disease, granulomatous disease, lymphoma, malignant disease. Material and Methods: Authors evaluated 22 patients, who were diagnosed as subacute necrotizing lymphadenitis on excisional biopsy or fine needle aspiration cytology with retrospective chart review. Results: The patient population consisted of 21 women and 1 men. The mean age was 25 years old(range 17 to 42 years). All patient had palpable neck mass and associated fever, headache, weight loss, otalgia, diarrhea, night sweating. and associated disorder is dermatitis, hepatitis B, SLE, Hashimoto's thyroiditis, albinism et al. The multiple bilateral involvement of cervical lymph node was 20 cases(91%) and multiple unilateral 18 cases(82%), multiple bilateral 2 cases(9%). Solitary involvement was 2 cases(9%), mean size of involved lymph node was 2.0cm. In laboratory tests, leukopenia 17 cases(72%), elevated ESR 12 cases(54%), elevated GOT, GPT 3 cases(13%). In CT finding, lymph nodes enhanced in homogeneous fashion, and there was no evidence of central necrosis. Conclusion: Subacute necrotizing lymphadenitis, Kikuchi's disease may be easily confused clinically, pathologically, radiologically with malignant lymphoma, inflammatory disease, granulomatous disease. We should consider open biopsy of lymph node in the patient which had unilateral multiple cervical lymph node and in laboratory tests, were leukopenia, elevated ESR, especially in young women. otherwise, result of fine needle aspiration biopsy is nonspecific, excisional biopsy is recommended in order to differentiate other disease.

• Tuberculosis and Respiratory Diseases
• /
• v.53 no.3
• /
• pp.294-308
• /
• 2002

Background : The cell-mediated immune response plays an important role in tuberculosis. After being activated by mycobacterial antigens, T lymphocytes express a high affinity receptor (IL-2R) for interleukin-2 (IL-2) on their own surface and release a soluble fraction of the IL-2 receptor (sIL-2R) from the cell membrane into the circulation. Neopterin is a metabolite of guanosine-triphosphate, which is produced by stimulated macrophages under the influence of IFN-$\gamma$ with a T lymphocyte origin. Therefore, the utility of sIL-2R, IFN-$\gamma$ and the neopterin levels as immunologic indices of the cell-mediated immune response and severity of disease in patients with pulmonary tuberculosis was assessed. Methods : The serum sIL-2R, IFN-$\gamma$ and neopterin levels were measured in 39 patients with pulmonary tuberculosis, 6 patients with tuberculous lymphadenitis prior to treatment and 10 healthy subjects. The serum and pleural sIL-2R, neopterin and ADA levels were measured in 22 patients with tuberculous pleurisy. The patients with pulmonary tuberculosis were divided into a mild, moderate and severe group according to the severity by ATS guidelines. To compare the results from these patients with those of the pretreatment levels, the sIL-2R, IFN-$\gamma$ and neopterin levels were measured in 36 of the 39 patients(1 patient, expired; 2 patients were referred to a sanitarium) with pulmonary tuberculosis after 2 months of treatment. Results : 1) the serum sIL-2R and IFN-$\gamma$ levels were elevated in patients with tuberculosis when compared to those of healthy subjects (p>0.05). The neopterin concentration in the serum was significantly lower in patients with pulmonary tuberculosis($2967{\pm}2132.8$ pg/ml) than in healthy controls($4949{\pm}1242.1$ pg/ml)(p<0.05). 2) In the pulmonary tuberculosis group, the serum sIL-2R and IFN-$\gamma$ levels were higher in patients with severe disease than those in patients with mild and moderate disease. However, the neopterin levels declined as the pulmonary tuberculosis became more severe (p<0.01). 3) The mean serum sIL-2R and IFN-$\gamma$ levels declined from $1071{\pm}1139.4$ U/ml to $1023{\pm}1920.9$ U/ml(p>0.05), $41{\pm}52.8$ pg/ml to $22{\pm}23.9$ gm/ml(p<0.05), respectively, after 2 month of treatment. The mean serum neopterin levels increased from $3158{\pm}2272.6$ pg/ml to $3737{\pm}2307.5$ pg/ml(p>0.05) after a 2 month of treatment. These findings were remarkable in the severe group of pulmonary tuberculosis with a clinical correlation. 4) In the patients with tuberculous pleurisy, the serum sIL-2R and ADA were significantly higher than those in the pleural fluid, However, the neopterin levels in the sera and pleural effusion were similar. Conclusion : On the basis of this study, sIL-2R, IFN-$\gamma$ and neopterin measurements may not only provide an insight into the present state of the cell-mediated immune response, but also serve as parameters monitoring of the prognosis of the disease, particularly in patients with severe pulmonary tuberculosis. In addition, an assay of the pleural sIL-2R levels might signal a stimulated local immunity including T cell activation in the tuberculous pleural effusion.

• Pediatric Infection and Vaccine
• /
• v.5 no.1
• /
• pp.69-78
• /
• 1998

Purpose : The risk of severe tuberculous disease such as meningitis or miliary tuberculosis increases as younger is the child at the time of infection. Therefore, the early diagnosis and prompt treatment is mandatory for infants with tuberculosis. This study was undertaken to describe the epidemiology, clinical and radiographic manifestations, and response to therapy in infants with tuberculous disease. Methods : Medical records of 29 infants with tuberculosis diagnosed at the Seoul National University Children's Hospital from July, 1985, to April, 1997, were reviewed, retrospectively. A case of tuberculosis was confirmed if M. tuberculosis was isolated from any body site or if there was histologic proof of tuberculosis. Otherwise, the diagnoses were individualized considering history of contact with contagious adult case, clinical manifestations, chest X-ray findings, result of a Mantoux test reaction with 5 tuberculin unit of PPD, and the response to therapy. Results : The mean age at diagnosis was $7.00{\pm}2.65$ months (range, 3 to 12 months). Twelve cases had isolated pulmonary diseases, and the rest had pulmonary disease and meningitis, 5 cases; pulmonary disease and cervical lymphadenitis, 3; isolated meningitis, 3; and miliary tuberculosis, 6. Source case was identified in 19 cases, 7 of which were detected with retrograde manner. Twenty seven of 29 were symptomatic at their initial visit. The presenting symptoms were mainly respiratory or neurologic, and respiratory difficulty was accompanied in 7 cases. Physical examination revealed wheezing in 7 cases and decreased breath sounds in 9. Hepatomegaly or hepatosplenomegaly were frequent. Chest radiographs showed lung parenchymal disease with hilar lymphadenopathy in 18 cases, and focal or generalized emphysematous change in 7 cases. Conclusion : Most of the infants with tuberculosis are symptomatic at diagnosis, and many of infants with intrathoracic tuberculosis presented with symptoms of bronchial obstruction. When tuberculosis is suspected in an infant, the adult source case should be vigorously investigated to aid in diagnosis and for the prevention of further transmission of tuberculous disease. Almost half of infant tuberculosis are preventable if prophylaxis were given when adult cases were diagnosed.

• Tuberculosis and Respiratory Diseases
• /
• v.44 no.1
• /
• pp.59-68
• /
• 1997

Background : Tuberculous chest wall abscess is a rare complication of tuberculosis. However, there have been few reports about the variable extents and shapes of tuberculous chest wall abscesses. We analyzed the extent and shape of tuberculous chest wall abscess-es and grouped them according to combined pleuroparenchymal lesions by CT scans. Materials and Methods : CT findings were evaluated in 20 patients of tuberculous chest wall abscesses. We classified 29 abscesses in 20 patients into three types according to pleuroparnechymal lesions. Type 1 was defined when there was no active pleuroparenchymal lesion, Type 2, when intrathoracic tuberculosis was contacted with chest wall abscess, Type 3, when ipsilateral subpleural nodules were not contacted with chest wall abscess. Results : The type 1 included 6 abcesses in 6 patients. They showed rib and/or costal cartilage destruction in their center. They were relatively large and round. The type 2 included 13 abscesses in 10 patients. The abscesses in contact with pleural lesion or mediastinal lesion were mainly located in the outer muscle layer, and they were relatively large in size. However, the abscesses in contact with parenchymal lesion were mainly located in extrapleural space. They were relatively small and they were longest along the long axis of ribs. The type 3 included 10 abscesses in 6 patients. They were located mainly in the extrapleural space. Conclusion : Tuberculous chest wall abscess-es showed variable extents and shapes according 10 pleuroparenchymal lesions. CT is a good diagnostic modality to visualize the extent of tuberculous chest wall abscess and combined pleuroparenchymal lesion.