Kim, Hyo Jung;Kim, Eun Young;Woo, Young Jong;Kook, Hoon
Clinical and Experimental Pediatrics
/
v.48
no.11
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pp.1232-1238
/
2005
Purpose : Though more than 15 years have passed after introduction of Lorenzo's oil therapy in X-linked adrenoleukodystrophy(ALD), its efficacy is still fully not known. In patients who already have neurologic symptoms, most reports indicate that the neurologic disability continues to increase. We evaluated its efficacy in X-linked ALD patients. Methods : Four patients who were treated with Lorenzo's oil for at least 12 months were enrolled from 1996 to 2003. During treatment, changes of neurologic symptoms, brain magnetic resonance imaging(MRI) findings, and serum saturated very long-chain fatty acid(C26:0) were assessed. Results : Two patients with childhood cerebral ALD had progression of neurologic symptoms and MRI lesions during treatment. One asymptomatic patient developed childhood cerebral ALD after six year treatment of Lorenzo's oil. One "Addison only" patient remained neurologically intact after three years. During Lorenzo's oil therapy, serum C26:0 levels which had increased at diagnosis decreased in all four patients. Conclusion : Treatment with Lorenzo's oil did not prevent disease progression in childhood cerebral ALD patients who were already symptomatic. Long term follow up will be needed in asymptomatic ALD including "Addison only" to reveal the efficacy of Lorenzo's oil.
Nho Young Ju;Chang Hyesook;Choi Eun Kyung;Kim Jong Hoon
Radiation Oncology Journal
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v.15
no.3
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pp.207-213
/
1997
Purpose : Intracranial germinoma is the most radiocurable tumor of theprimary intracranial neoplasm. But, the optimum radiation dose and target volume remain controversial In this retrospective study, we analysed the spreading pattern at presentation and the pattern of the failure and survival of intracranial germinoma, Materials and Methods : From 1989 to 1996, 23 Patients were treated for intracranial germinoma at Department of Radiation Oncology, Twenty-one Patients were treated at their initial Presentation and 2 Patients were treated for recurrent disease. Six patients had multiple tumor masses on MRI and 7patients had ventricular seeding on MRI. The examination of cerebrospinal fluid cytology was done in 15 patients and 3 out of 15 patients had positive cerebrospinal cytology. In tumor marker study of $\alpha-FP\;and\;\beta-hCG$, 6 patients had mildly elevated $\beta-hCG$ in serum or cerebrospinal fluid. Twentyone Patients were treated with whole craniospinal axis irradiation and 2 Patients were given whole ventricular radiation therapy. The total dose was ranged between 4500cGy and 5600cGy to primary tumor site (median 5580cGy) Dose to the entire ventricular system ranged from 1980cGy to 3960 cGy (median 2700cGy) and dose to the spinal axis ranged from 2160cGy to 3900cGy (median 2700cGy) Results : Of 23 patients, 21 Patients are alive without evidence of diseasefor median 4 years follow-up. One Patient who had markedly elevated $\alpha-FP\;and\;\beta-hCG$, suffered from Persistent disease after radiation therapy and received 2 cycles of chemotherapy. She died 9 months after chemotherapy One patient who developed ventricular seeding after gamma-knife was treated with whole craniospinal irradiation, he died after 1 year due to probably brain necrosis. The hematologic toxicity of 3 or 4 grade were seen in 7 patients and patient's endocrinologic dysfunction was not deteriorated after radiation therapy. One patient had been treated with growth hormone replacement due to short stature. Conclusions : This retrospective study has confirmed the excellent result of radiation therapy in intracranial germinoma. The complication rate during or after radiation therapy is considered within acceptable range. ft is necessary to further investigate the optimal dose and treatment volume of radiation therapy. The role of chemotherapy in the treatment of intracranial germinoma should be further investigated.
Yoo, Eun Jung;Kim, Eun Young;Kook, Hoon;Woo, Young Jong
Journal of The Korean Society of Inherited Metabolic disease
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v.5
no.1
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pp.33-43
/
2005
Purpose: X-linked adrenoleukodystrophy(ALD) is a rare disorder that shows a great deal of phenotypic variability. We subdivided chidhood X-linked ALD patients into several phenotypes by the age at onset, the sites of most severe clinical involvement and the rate of progression of neurologic symptoms. Methods: Thirteen patients who had been diagnosed as X-linked ALD and followed up for at least one year were enrolled from 1996 to 2003. Results: 1. Ten had childhood cerebral ALD, who showed first neurologic symptoms at 7.02 years and progressed rapidly: interval between first symptoms and vegetative state was 1.35 years, and interval from initial symptoms to death was 3.35 years. Treatment with Lorenzo's oil did not prevent neurologic progression. Two patients who underwent umbilical cord blood transplantation died. 2. Two had adolescent cerebral ALD. They had first symptoms at 11.5 years, and showed tendency to progress less rapidly than childhood cerebral form patients. 3. One "Addison only" patient who had adrenal insufficiency without nervous system involvement remained asymptomatic during Lorenzo's oil treatment. 4. All cerebral form patients except one showed the lesions in both parieto-ocipital white matter in brain magnetic resonance imaging. Conclusion: The cerebral ALD was the most common form in childhood and was asoociated with a serious prognosis.
Due to the recent advancement in digital imaging technology, development of intervention equipment has become generalize. Video arbitration procedure is a process to insert a tiny catheter and a guide wire in the body, so in order to enhance the effectiveness and safety of this treatment, the high-quality of x-ray of image should be used. However, the increasing of radiation has become the problem. Therefore, the studies to improve the performance of x-ray detectors are being actively processed. Moreover, this intervention is based on the reference of the angiographic imaging and 3D medical image processing. In this paper, we propose a guidance system to support this intervention. Through this intervention, it can solve the problem of the existing 2D medical images based vessel that has a formation of cerebrovascular disease, and guide the real-time tracking and optimal route to the target lesion by intervention catheter and guide wire tool. As a result, the system was completely composed for medical image acquisition unit and image processing unit as well as a display device. The experimental environment, guide services which are provided by the proposed system Brain Phantom (complete intracranial model with aneurysms, ref H+N-S-A-010) was taken with x-ray and testing. To generate a reference image based on the Laplacian algorithm for the image processing which derived from the cerebral blood vessel model was applied to DICOM by Volume ray casting technique. $A^*$ algorithm was used to provide the catheter with a guide wire tracking path. Finally, the result does show the location of the catheter and guide wire providing in the proposed system especially, it is expected to provide a useful guide for future intervention service.
Background: The objective responses of cisplatin and etoposide (PVP) combination chemotherapy as second-line therapy following CAV was high (40~50%) and, in several reports, PVP yields survival results that are at least as good as those obtained with cyclophosphamide or doxorubicin-based regimens and with less host-related toxicity in chemotherapy-naive patients. We conducted a phase II study to evaluate the effect of a combination of cisplatin and etoposide as a fitst-line therapy in patients with small cell lung cancer. Methods: Sixty-one previously untreated small cell lung cancer patients with measurable lesion (s) received cisplatin(30 $mg/m^2$ IV, day 1~3) and etoposide(100 $mg/m^2$ IV, day 1~3). In patients with limited disease, after completion of 6 cycles of PVP chemotherapy, chest and prophylatic brain irradiation was performed in case of complete responder, chest irradiation on1y in partial responder. Results: 1) Of 55 evaluable patients, 13(24%) had a complete response and 29(53%) had a partial response. 2) The median survival time was 55.8 weeks for all patients(N=55), 61.1 weeks for limited disease(N=31), 51.3 weeks for extensive disease(N=24). 3) The response duration was 29.1 weeks for responders(N=42). 4) There was no significant prognostic factors influencing response rates. 5) The toxicity was tolerable and there was no treatment-related deaths. Conclusion: The PVP combination chemotherapy as a first-line therapy was effective and well-tolerated in patients with small cell lung cancer.
Yangkyuk-Sanhwa-Tang(YST) has been widely used as a formula for the Soyangin cerebral infarction (CI) patients according to Sasang constitutional philosophy. Brain cells produce cytokines and chemokines during the inflammatory process after stroke both in animal models and in patients. Previously, regulation of serum cytokine levels by YSThas been observed in individuals at the acute stage of CI disease, but there have not been other scientific investigations on YST. The author investigated the effect of YST on theproduction of various cytokines using peripheral blood mononuclear cells (PBMCs)from the Soyangin (CI) patients, and Soyangin normal group. The cytokine production was analyzed using enzyme-linked immunosorbent assay (ELISA). The amount of interleukin (IL)-1, IL-1, IL-6, IL-8, and tumor necrosis factor (TNF)- in culture supernatant significantly increased in the LPS-treated cells compared with unstimulated-cells (P < 0.05). However, in LPS-stimulated PBMCs, cytokines level in CI patients group was higher than that of normal group. YST (1 mg/ml) significantly inhibited IL-1, IL-1, and IL-8 production in PBMCs stimulated with LPS (about 85% for IL-1, 87% for IL-1, and 53% for IL-8, P < 0.05), but did not significantly inhibit IL-6 and TNF- production in the CI patients group. We also show that YST significantly increased LPS-induced IL-1, IL-6, and TNF- production in the normal group. Thesedata suggest that YST has a regulatoryeffect on the cytokine production, which might explain its beneficial effect in the treatment of CI.
Background: In malignancies, detection of metastatic foci is of value in making therapeutic plans for treatment of disease and prevention of life-threatening complications. Common sites for metastasis of bronchogenic cancer include lymph nodes, liver, brain, adrenals and bone. Skull, vertebrae, ribs and long bones are common sites for bone metastasis. But in epidermoid carcinoma, the incidence of bone metastasis is relatively low and especially to the distal phalangeal bone is rare. Methods: We experienced a case of epidermoid carcinoma with the first distant metastasis to the fifth distal phalangeal bone, right toe. Results: The initial stage in the diagnosis of epidermoid carcinoma was T4N3MO. During the third round of anticancer chemotherapy, we recognized the distant metastasis to the fifth distal phalangeal bone for the first time. Localized abnormal findings were noted by bone X-ray and scanning. By a histopathologic examination of the amputated toe, we confirmed the metastasis of epidrmoid bronchogenic carcinoma. Conclusion: If localized abnormal finding is discovered at an unusual site for metastasis, we recommend physicians to consider the possibility of metastasis even though it is very low.
Park, Byung Hoon;Park, Seon Cheol;Shin, Sang Yun;Jeon, Han Ho;Jung, Kyung Soo;Chung, Woo Young;Byun, Min Kwang;Moon, Ji Ae;Kim, Young Sam;Kim, Se Kyu;Chang, Joon;Kim, Sung Kyu;Park, Moo Suk
Tuberculosis and Respiratory Diseases
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v.62
no.6
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pp.549-553
/
2007
Sarcoidosis is a multi-systemic syndrome of an unknown etiology, and it is characterized by the formation of multiple noncaseating granulomas that disrupt the architecture and function of the tissues in which they reside. The most commonly affected organs are lung, skin and lymph nodes. Overt clinical involvement of the nervous system is uncommon and this occurs in about 5% of all patients during the course of their disease. The most common manifestations are granulomatous leptomeningitis, cranial nerve palsy, electrolyte or other endocrinologic abnormalities, but isolated memory impairment is a rare manifestation. This is a case of 59 years-old male with recent memory impairment, and he was previously diagnosed with pulmonary sarcoidosis by transbronchial lung biopsy. The brain MRI imaging revealed the leptomeningeal and parenchymal involvement of sarcoidosis. He was treated with high dose corticosteroid and his memory function was improved to nearly a normal level. We report here on a case of successful treatment of pulmonary sarcoidosis combined with neurosarcoidosis with using high dose corticosteroid, and the patient presented with recent memory impairment.
Objective: Yikgeebohyul-tang (Yiqib$\check{u}$xu$\grave{e}$-t$\bar{a}$ng) is one of the representative prescriptions for invigoration of vitality and nourishing of the blood. This study was carried out to investigate the effects of Yikgeebohyul-tang (Yiqib$\check{u}$xu$\grave{e}$-t$\bar{a}$ng) on hair regrowth and cytokine changes in a shaving model of C57BL6 mice. Method: Five-week-old mice were acclimated for 1 week at a temperature between $21-23^{\circ}C{\acute{E}}$, 40-60% relative humidity, and 12h of a light/dark cycle before beginning of the experiment. There were two groups including normal saline (control) and a positive control of oral intake of Yikgeebohyul-tang (Yiqib$\check{u}$xu$\grave{e}$-t$\bar{a}$ng) extract (sample) in 18 female mice. The test compounds were topically treated once a day over 14 days. The hair regrowth was photographically and histologically determined during the experimental period of 14 days. Revelation of TGF-${\beta}1$ and EGF were also determined using immunohistochemistry. In addition to that, IFN-$\gamma$, IL-4 and IL-10 were determined in serum. Results: Hair regrowth in the sample group was promoted earlier and faster than the control group, as shown by concentrations of hairs and thick-hair ratio in the sample group. TGF-${\beta}1$ was not revealed in either control or sample group. EGF was strongly positive in out root sheath of some thick hair of the sample group. Serum IFN-$\gamma$ was significantly decreased in the sample group compared with the control group at 7 experimental days. However, it was not significantly decreased at 14 experimental days. Serum IL-4 was significantly increased in the sample group compared with the control group at 7 experimental days. However, it was not significantly decreased at 14 experimental days. Serum IL-10 was decreased in the sample group compared with the control group, but with no real statistical significance. Conclusion: These results suggest that Yikgeebohyul-tang (Yiqib$\check{u}$xu$\grave{e}$-t$\bar{a}$ng) has hair growth promoting activity and it can be used for treatment of alopecia. Also, these effects relate to EGF revelation of hair roots, a decrease in serum IFN-$\gamma$, and an increase of serum IL-4.
From December 1984 to February 1990, 16 patients with tumors of pineal and suprasellar location were treated with radiation therapy. Tissue diagnoses were obtained before radiation therapy in 5 patients and 11 were irradiated without histologic confirmation. Initial treatments for these patients were craniospinal plus boost primary irradiation(six), whole brain plus boost primary irradiation(nine), primary tumor site irradiation(one). The 5 year actuarial survival rate is $71\%$. Three cases with elevated beta-human chorionic gonadotropin(HCG) responded favorably to radiation, but pineal tumors with elevated alpha-fetoprotein(AFP) did not respond well. Spinal metastasis developed in 2 cases(2/15) with elevated AFP : one received prophylactic spinal irradiation, another did not. Our studies suggest that more aggressive treatment would be necessary in patient with elevated AFP and in this patient, radiation therapy may be initiated without pathologic confirmation. From the result of our study, routine use of prophylactic spinal irrdiation for all patients with pineal region tumor is not indicated and use of prophylactic spinal irradiation is considered for the patients with positive craniospinal fluid cytology, meningeal seeding, disease extension along the ventricular wall and biopsy proven germinoma.
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