• Journal of Chest Surgery
• /
• v.29 no.6
• /
• pp.632-638
• /
• 1996

A retrospective analysis of primary mediastinal tumors and cysts was performed on 42 patients who underwent surgical resection at our institution from january, 1985 to December, 1995. The patients consisted of 27 males and 15 females. The mean age was 40 years with a range of 10 month to 76 years. The patients were composed of thymlc tumor 12 cases (28.6 %), germ cell tumor 8 cases (19.0 %), primary cyst 7 cases (16.7 %), neurogenic tumor 6 cases (14.3 %) and other miscellanious tumor 9 cases. Overall, 3) (78.6 %) of the tumors were histologically benign, and 9 (21.4 %) were malig- nant. The noted clinical manifestations were respiratory symptoms such as chest pain, dyspnea and coughing. All of the patients with malignancy and 55 oyo of the patients with benign tumor were symptomatic on presentation. All of the patients were operated for tis ue diagnosis and curative resection. All the benign tumors ex- cept two cases of sarcoidosis were performed adequate curative resection. A few patients with malignant unresectable tumors were treated with chemotherapy or radiotherapy There were 7 (18 %) postoperative complications without mortality. In conclusion, Mediastinal tumors have long fascinated the thoracic surgeon because of their variety and unpredictability of diagnosis prior to exploration. We consider that active surgery and various combined modality can be accomplished with satisfactory result.

• Tuberculosis and Respiratory Diseases
• /
• v.38 no.2
• /
• pp.128-134
• /
• 1991

This report is an analysis of 60 cases of primary mediastinal tumors and cysts which were experienced in chonbuk National University Hospital, Wonkwang University Hospital and Chonju Presbyterian Medical Center from january 1985 to October 1990. The result was summarized like this; 1) Age distribution was various from ages of 5 to 75 years. There were 26 males and 34 females, sex ratio of patients was 1: 1.3 (M:F). 2) The most common chief compliant was dyspnea (21 cases, 35%) and followed by chest pain, coughing and chest discomfort. 17 patients (28%) were asymptomatic. 3) The most common primary mediastinal tumor was thymoma, which comprise 28% (17 cases) of all our cases and followed by neruogenic tumor (14 cases, 23%) and germ cell tumor (11 cases, 18%). 4) The incidence of malignancy of all case was 22%; 2 cases were asymptomatic and the most common malignancy was malignant lympoma (5 cases, 38.5%). 5) The anterior mediastinum was the most common tumor location and followed by posterior and middle mediastinum. Anterior mediastinal tumors mainly consisted of thymomas and germ cell tumors and posterioc mediastinal tumors mainly did neurogenic tumors. 6) Of 60 cases, 21 cases were confirmed histopathologically by percutaneous transthoracic needle lung biopsy. 7) Of 60 cases, 40 cases were received radical tumor resection.

• Journal of Korean Society of Spine Surgery
• /
• v.25 no.4
• /
• pp.169-174
• /
• 2018

Study Design: Case report. Objectives: We report a case of pure epidural cavernous hemangioma located at the thoracolumbar spine in a 53-year-old woman that mimicked a neurogenic tumor on magnetic resonance imaging (MRI). Summary of Literature Review: A pure spinal epidural cavernous hemangioma without bony involvement is a very rare lesion about which limited information is available in the literature. Materials and Methods: A 53-year-old woman visited our clinic for hypoesthesia with a tingling sensation in the left anterolateral thigh that had begun a month ago. No other neurologic symptoms or signs were present upon a neurologic examination. MRI from an outside hospital showed a $2.0{\times}0.5cm$ elongated mass at the T11-12 left neural foramen. The tumor was completely removed in piecemeal fashion. Results: The histopathologic examination revealed a cavernous hemangioma, which was the final diagnosis. The outcome was favorable in that only operation-related mild back pain remained, without any neurologic deficits, after a postoperative follow-up of 2 years and 3 months. No recurrence was observed on MRI at 2 years postoperatively. Conclusion: Pure epidural spinal cavernous hemangioma is very rare, and it is very difficult to differentiate from other epidural lesions. However, we believe that it should be included in the differential diagnosis of spinal epidural tumors due to its favorable prognosis.

• The korean journal of orthodontics
• /
• v.29 no.1 s.72
• /
• pp.73-81
• /
• 1999

Midpalatal suture expansion is often used for patients haying narrow maxillary arch, cleft palate, respiratory handicap with narrow nasal cavity. CGRP has been known as a modulator of pain transmission in central nervous system and a local effector to peripheral tissue causing vasodilation, increase of blood flow, modulation of immune system, regulation of macrophagic function and stimulation of bone formation. To investigate changes of CGRP-immunoreactive nerve fibers in midpalatal suture during the expansion, immunohistochemical study was performed by using rats. Experimental rats (10 weeks, 250 gm) were divided into five groups (control, 1, 4, 7, 14 days group (each n=4) and applied orthodontic force (approximately 200gm) to upper anterior incisors. Frozen sections of midpalatal suture area were immunostained by using rabbit antisera. The results were as follows. ${\cdot}$ The CGRP-immunoreactive nerve fibers were hardly observed in control group. ${\cdot}$ In 1 day group, the CGRP-immunoreactive nerve fibers were more increased around the vessels than control group. ${\cdot}$ In 4 days group, the CGRP-immunoreactive nerve fibers were more increased than control group, but not more increased than 1 day group. Vascular diameter was more enlarged. ${\cdot}$ In 7 days group, especially, hematoxilin affinity of cells was remarkable and cells were arranged along the bone margin. The CGRP-immunoreactive nerve fibers were more reduced than 4 days group and vascular diameter was also reduced. ${\cdot}$ In 14 days group, the CGRP-immunoreactive nerve fibers were similar to those of 7 days group and the irregularity of bone margin was almost recoverd. In Conclusion, the CGRP-immunoreactive nerve fibers nay be related to initial neurogenic inflammatory reaction in expanding mid-palatal suture.

• Childhood Kidney Diseases
• /
• v.11 no.2
• /
• pp.280-287
• /
• 2007

Purpose : Urinary lithiasis is uncommon in children, however, it may lead to chronic renal insufficiency and even end stage renal disease. The etiology of stone formation in children is largely unknown; although the most common causes are known to be associated with congenital anomalies of the genito-urinary(G-U) tract, urinary tract infections(UTI), and metabolic diseases. Methods : A total of 73 children(male:female=42:31, mean age $6.6{\pm}5.3$ years) presented with urinary lithiasis between Sep. 1998 and Jul. 2007 at Seoul National University Children's Hospital. The medical records were reviewed retrospectively. Results : The most common presenting symptoms were gross hematuria(28/73, 38%) and flank or abdominal pain(23/73, 32%). The stones were located in the upper urinary tract in 48 patients(66%), in the bladder in 18(24%), and in both the bladder and upper urinary tract in 2 (3%). Congenital anomalies of the G-U tract with/without UTI were detected in 30 children (41%), hypercalciuria with/without hypercalcemia in 15(20%), and other metabolic diseases in 8(11%). In 17 patients(23%), no underlying cause of stone formation was detected. The majority of stones were infected stones(24/36, 67%), which were followed by calcium stones(8/36, 22%), uric acid stones(3/36, 8%). and cystine stones(1/36, 3%). Thirty-four patients(46%) underwent surgical procedures and/or extracorporeal shockwave lithotripsy for stone removal, and 13(18%) passed stones spontaneously with/without medical management. Stones recurred in 6 patients(8%): 4 with neurogenic bladder augmented by ileocystoplasty, 1 with cystinuria, and 1 with unknown etiology. Conclusion : The common causes of urinary lithiasis in children were congenital anomalies of the G-U tract with/without UTI and metabolic disorders including hypercalciuria/hypercalcemia. For the management of stones, minimally invasive procedures should be chosen on the basis of accompanying symptoms and the composition, locations and etiology of stones.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.23 no.1
• /
• pp.25-30
• /
• 2023

Leigh syndrome is a rare progressive neurodegenerative mitochondrial disorder with clinical and genetic heterogeneity. Recently, balletic IARS2 variants have been identified in a number of patients presenting broad clinical phenotypes from Leigh and West syndrome to a rare syndrome CAGSSS characterized by cataracts, growth hormone deficiency, sensory neuropathy, sensorineural hearing loss, and skeletal dysplasia syndrome (OMIM#616007). We describe a child with Korean Leigh syndrome with urologic manifestations resulting from a compound heterozygote mutation in IARS2. A 5-year-old girl visited the emergency room with a complaint of abdominal pain accompanied by abdominal distension. Abdominal-pelvic CT showed a markedly distended urinary bladder without definite obstructive lesions. She was diagnosed with neurogenic bladder dysfunction based on a urodynamic study. She had global delayed development due to neurologic regression after 6 months of age and a history of bilateral cataract surgery at the age of 2 years. Her brain magnetic resonance imaging showed symmetrically increased signal intensities in the bilateral putamen and caudate nuclei with diffuse cerebral atrophy. No gene variants were identified through whole-mitochondrial genome analysis. Whole exome sequencing was performed for diagnosis, and compound heterozygous pathogenic variants were identified in IARS2: c.2446C>T (p. Arg816Ter) and c.2450G>A (p. Arg817His). To the best of our knowledge, this is the first case report of bladder dysfunction manifestation in a patient with IARS2-related Leigh syndrome. Thus, it broadens the clinical and genetic spectrum of IARS2-associated diseases.