• Childhood Kidney Diseases
• /
• 제22권2호
• /
• pp.91-96
• /
• 2018

Nephrotic syndrome in the first year of life, characterized by renal dysfunction and proteinuria, is associated with a heterogeneous group of disorders. These disorders are often related to genetic mutations, but the syndrome can also be caused by a variety of other diseases. We report an infant with nephrotic syndrome associated with a neuroblastoma. A 6-month-old girl was admitted with a 10% weight loss over 10 days and nephrotic-range proteinuria. She was ill-looking, and her blood pressure was higher than normal for her age. Her cystatin-C glomerular filtration rate was decreased, and levels of plasma renin, aldosterone, and catecholamines were elevated. Renal ultrasonography and abdominal computed tomography showed a retroperitoneal prevertebral mass encasing both renal arteries and the left renal vein. The mass was partially resected laparoscopically, and the pathologic diagnosis was neuroblastoma. Findings on a simultaneous renal biopsy were unremarkable. The patient was treated with chemotherapy and several anti-hypertensive drugs, including an alpha blocker. Two months later, the mass had decreased in size and the proteinuria and hypertension were gradually improving. In an infant with abnormal renin-angiotensin system activation, severe hypertension, and nephrotic-range proteinuria, neuroblastoma can be considered in the differential diagnosis.

• 한국임상수의학회지
• /
• 제22권2호
• /
• pp.148-152
• /
• 2005

5년령 페키니즈가 촉진가능한 복강종괴 때문에 서울대학교 부속동물병원에 내원하였다. 실험실 검사에서 특별한 이상은 없었다. 방사선 검사에서 경계가 분명한 좌측 복부중간에 종괴가 있고 복강내 장막선 손실, 후복강의 비정형성투명도, 그리고 방사선 불투과성 방광 결석 소견을 나타냈다. 복강초음파에서 좌측신장에 불규칙한 모양을 한 비균질성 실질종괴가 발견되었고 좌측대형신장종괴가 장간막, 소장, 비장, 췌장을 침습한 소견을 관찰하였다. 편측성 신장요관절제술을 실시하였다. 조직병리소견에서 신장세포암종으로 확진하였다. 수술후 다음날 환축은 폐사하였다. 비록 초음파검사로 종괴에 관한 진단적 정보를 알 수 있지만, 컴퓨터 단층촬영은 종괴의 특성에 관한 핵심 영상 소견을 나타낸다

• Clinical and Experimental Pediatrics
• /
• 제57권11호
• /
• pp.500-504
• /
• 2014

Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor of intermediate malignancy with resemblance to Kaposi sarcoma. It occurs predominantly in pediatric age groups as a cutaneous lesion with focal infiltration into the adjacent soft tissue and bone. Although visceral involvement is very uncommon, several cases with bone, retroperitoneal, or mediastinal involvement have been described. KHE has been reported to occasionally occur in unusual sites such as the thymus, tonsils, larynx, paranasal sinuses, deltoid muscle, spleen, uterine cervix, thoracic spine, and even the breast. Multifocal KHE is an extremely rare entity with few reports available in the literature, none of which describes pulmonary involvement. Herein, we report a unique case of multifocal KHE in a 13-year-old boy presenting with a huge soft tissue mass in the upper extremity complicated by bilateral pulmonary nodules that developed into large, necrotic tumor masses.

• Journal of Korean Neurosurgical Society
• /
• 제30권9호
• /
• pp.1127-1129
• /
• 2001

The vast majority of schwannomas occur on cranial nerves, and rarely in the retroperitoneum. Solitary schwannoma in the psoas muscle is extremely rare. The authors present a case of retroperitoneal neoplasm in the psoas muscle identified as schwannoma which is not associated with von Recklinghausen's disease. A 68 years old female patient was admitted because of low back pain and weakness at the left leg. CT and MRI revealed a large cystic mass with well-defined margin and multiple internal septation within the left psoas muscle. The tumor was totally extirpated and histologically confirmed as a schwannoma.

• Journal of Korean Neurosurgical Society
• /
• 제47권4호
• /
• pp.295-297
• /
• 2010

Candida is a relatively rare cause of spinal infections that commonly affects immunocompromised patients. A 70-year-old woman, who underwent a lumbar discectomy on L5-S1 two months earlier, was admitted to our department complaining of persistent back and leg pain. Magnetic resonance imaging showed irregular enhancing mass lesion in L5-S1 intervertebral space, suggest of pyogenic discitis with epidural abscess. The surgery was performed via retroperitoneal approach and the infected material at L5-S1 intervertebral space was removed. The histological examination of the specimen revealed chronic inflammation involving the bone and soft tissue, and a culture of the excised material was positive for Candida parapsilosis. The patient received intravenous fluconazole for 4 weeks after surgery and oral fluconazole 400 mg/day for 3 months after surgery. The patient made a full recovery with no symptoms 6 months after surgery. We present a rare case of spondylodiscitis after a lumbar discectomy due to Candida parapsilosis and discuss treatment option with a review of the literatures.

• Advances in pediatric surgery
• /
• 제1권2호
• /
• pp.122-132
• /
• 1995

Teratoma contains elements derived from all three embryonic germ layers and mature teratomas are entirely composed of mature differentiated tissues, while immature types always contain additional embryonic tissues of variable degree of immaturity, especially neuroepithelial elements. Twenty cases of teratoma in infancy and childhood were reviewed and the clinical features and pathologic findings including PCNA expression were studied. Sacrococcygeal teratomas were the most common type(45%), followed by retroperitoneal and ovarian teratomas. There was a predilection of females in a ratio of 4:1 and age distribution was most prevalent below the first year of age(45%). But in ovarian teratomas, the age at diagnosis was above 4 years of age in all cases. Serum alpha-fetoprotein levels were checked in 18 cases. In all mature teratomas and 1 of 5 immature teratomas, the levels were normal. But in 4 of 5 immature teratomas, the serum levels were elevated and progressively declined to normal range after mass excision. Radiologically, calcifications in tumor were found in 60.0% of teratomas and was higher in mature teratomas(69.2%) than immature teratomas(42.9%). Immunohistochemical staining for PCNA(proliferating cell nuclear antigen) was done in 16 cases and PCNA expression was higher in grade III immature teratomas than grade I and II. The operative modes were complete mass excisions. Five immature teratomas were treated with multiagent PEB(Bleomycin, Etoposide, Cisplatin) adjuvant chemotherapy, 3 tolerated well without significant complications, but in one case, severe bone marrow suppression was developed and died of sepsis. In conclusion, grade III immature teratoma showed higher PCNA expression than mature or lower grade immature teratoma, which suggests that chemotherapy after surgical excision may be effective modality for grade III immature teratoma. We think, however, multicenter study is necessary because of low incidence of immature teratoma.

• Tuberculosis and Respiratory Diseases
• /
• 제67권4호
• /
• pp.359-363
• /
• 2009

Immunoglobulin G4 (IgG4) related autoimmune diseases are characterized by high serum IgG4 concentrations, sclerosing inflammation of numerous IgG4-positive lymphoplasma cells of varying origin, and a positive response to steroid treatment. Autoimmune pancreatitis, sclerosing cholangitis, and retroperitoneal fibrosis are representative presentations of IgG4 related autoimmune disease. Herein, we describe 2 patients (40-years-old woman and 47-years-old man) diagnosed with pulmonary involvement of IgG4-related autoimmune disease. The patients were admitted for an evaluation of the lung mass or multiple lung nodules found on chest radiography. Surgical lung biopsies were performed and pathologic finding revealed lymphoplasmacytic sclerosing inflammation with numerous IgG4 positive cells. The patients had elevated serum total IgG and IgG4 levels. Treatment consisted of high dose methylpredinisolone (1 mg/kg/day) and demonstrated good responsiveness. However, one patient experienced 2 relapses while being tapered off of steroid treatment.

• Preventive Nutrition and Food Science
• /
• 제15권1호
• /
• pp.1-6
• /
• 2010

The effect on body fat accumulation on male Wistar rats undergoing continuous feeding with a histidine-enriched diet was investigated. Five-week-age rats were assigned to two groups and were fed either the control diet (purified diet AIN-$76^{TM}$) or the histidine-enriched diet containing 3% histidine for 28 days. It was observed that both adipose tissue masses in retroperitoneal and epididymal areas of rats fed histidine-enriched diet significantly decreased (p<0.05) compared to those of control rats, while there was no significant difference in the food efficiency ratio between them. The blood levels of histidine derivatives of 3-methylhistidine and carnosine were significantly (p<0.05) increased in the rats fed a histidine-enriched diet, whereas there were no significant different between the histidine-enriched diet and control groups in the general amino acid distribution. Our results demonstrate that a histidine-enriched diet suppresses body fat accumulation in rats.

• Advances in pediatric surgery
• /
• 제9권2호
• /
• pp.94-97
• /
• 2003

The teratoma is a unique complex neoplasm and is one of the most frequent pediatric tumors originated from the extragonadal germ cells. Mature teratoma is composed of mature differentiated tissues, while immature teratoma always contains embryonic tissues of variable degrees of immaturity, especially in the neuroepithelial elements. Diagnosis of teratoma is relatively easy by conventional radiologic study, but the immaturity can be identified only by histopathological examination. Between January 1993 to December 2002, 63 cases of teratoma were operated and analysed retrospectively at the Chonnam University Hospital Female to male ratio was about 3:1 and age distribution was relatively even. Among 63 cases, gonadal teratoma was the most common (52.4%), followed by sacrococcygeal (25.4%), retroperitoneal (9.5%) and mediastinal teratoma (9.5%). Fifty-six cases were mature teratomas and seven were immature teratomas. Alpha-fetoprotein (AFP) was elevated in 4 of 6 immature cases, but in 2 of 51 mature ones Elevated AFP progressively returned to normal range by 1 month after operation in all. Complete excision of the mass was performed, and major complication was not noticed. In five immature cases, PEB chemotherapy (Cisplatin, Etoposide, Bleomycin) was performed. Two of 2 cases in histological grade II were well tolerated to the aggressive chemotherapy. One of three cases in grade III expired due to severe bone marrow depression, and two of them expired by tumor recurrences. In conclusion, immature teratoma in histological grade III showed high potentiality of recurrence. Therefore, postoperative chemotherapy has to be applied to the high graded immature tumors.

• Journal of Nutrition and Health
• /
• 제49권6호
• /
• pp.411-419
• /
• 2016

본 연구는 고지방식이와 쑥추출물이 흰쥐의 지질대사, 인슐린저항성, 아디포카인 분비에 미치는 효과를 측정하였다. 고지방식이에 의해 부고환지방과 복막후강지방의 축적이 증가되었고, 그 결과 혈장 항염증성 아디포카인은 감소하고 염증성 아디포카인은 증가하여 아디포넥틴/렙틴 및 아디포넥틴/PAI-1 비가 저하되는 불균형을 초래하였다. 내장지방 축적과 관련된 아디포카인의 불균형은 TG, TG/HDL-C, TC/HDL-C를 증가시키고 HOMA-IR의 증가를 유발한 것으로 추정된다. 이에 비해 쑥 에탄올추출물의 첨가는 부고환지방과 복막후강지방의 축적을 완화함으로써 PAI-1과 렙틴을 감소시켜 아디포넥틴/렙틴 및 아디포넥틴/PAI-1 비의 저하를 억제하고 아디포카인의 균형을 상대적으로 유지하였으며, TG, TG/HDL-C, TC/HDL-C 및 HOMA-IR 수치가 감소하였다. 따라서 쑥 에탄올추출물은 내장지방 축적을 억제하고 아디포카인의 분비, 특히 염증성물질인 렙틴과 PAI-1의 분비를 저하시켜 아디포카인의 균형을 조절함으로써 지질대사 및 인슐린저항성을 개선하는 효과가 있는 것으로 나타났다.