• Tuberculosis and Respiratory Diseases
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• 제70권1호
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• pp.63-68
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• 2011

Multiple symmetrical lipomatosis (MSL), also called Madelung's disease, is a rare disorder of unknown etiology and characterized by abnormal accumulation of large subcutaneous fatty masses in neck, shoulder, and upper trunk. MSL has known to predominantly affect middle-aged men with a history of alcoholism. Although the clinical course of MSL is considered to be slowly progressive, in advanced stage, fatty masses in the neck may compress the upper aerodigestive tract, resulting in dyspnea and dysphagia. The treatment of MSL is surgical resection, but radical excision is very difficult and recurrence after surgery is frequent. We report the case of 55-year-old man with long lasting MSL, which caused severe airway obstruction. This patient was admitted with progressive dyspnea and massive accumulation of fat around the vocal cord that was detected on a neck CT scan. This abnormal fatty infiltration in supraglottic region caused upper airway obstruction.

• Journal of Chest Surgery
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• 제48권2호
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• pp.151-154
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• 2015

Extensive tumoral calcinosis affecting a large joint is uncommon in patients with systemic sclerosis. We report the case of a 52-year-old female patient referred for a growing calcified mass in the shoulder. She was diagnosed with interstitial lung disease and progressive systemic sclerosis. Although the pain and disability associated with the affected joint was not severe, the patient underwent surgical excision because the mass continued to grow and was likely to produce shoulder dysfunction and skin ulceration. The patient appeared well 10 months after surgery with no signs of recurrence. This report highlights the timing and indication of surgical excision in similar cases.

• Childhood Kidney Diseases
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• 제3권1호
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• pp.95-99
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• 1999

Idiopathic membranous nephropathy is one of the most common causes of nephrotic syndromes in adults but rare in childhood. The occurrence of crescentic glomerulonephritis and membranous nephropathy in a patient is rare. This report describes a patient who initially was diagnosed as a membranous glomerulonephropathy at age 12 years and subsequently developed a crescentic, rapidly progressive glomerulonephritis at age 19 years. The patient responded to methylprednisolone pulse therapy and he maintained with partial remission and normal renal function with residual proteinuria.

• 대한한의학회지
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• 제30권4호
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• pp.162-168
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• 2009

Objective: This report aimed to study fibromyalgia's diagnostic criteria, differential diagnoses and its pathology from the viewpoint of oriental medicine. Method: We observed and diagnosed a female patient who complained of prolonged whole body pain, even several times hospitalized for treatment. Result: The patient's symptoms correspond with fibromyalgia and we ruled out other suspected diseases. Conclusion: Fibromyalgia is prone to be misdiagnosed because the patient has complicated symptoms such as various pains and chronic fatigue as well as depression unexplained by medical tests. Furthermore, the symptoms often never disappear completely, although it isn't progressive or life-threatening, and can be improved. So accumulation of data and analysis from the viewpoint of oriental medicine is necessary for development of oriental medical standard treatment.

• Annals of Clinical Neurophysiology
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• 제22권1호
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• pp.33-36
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• 2020

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder with numerous causes that include genetic factors. Efforts to reveal the genetics of ALS have identified several candidate genes that are associated with familial and sporadic ALS. Here we report a Korean ALS patient who showed prominent upper motor-neuron-related symptoms with marked brain atrophy and neuropsychological deficits. The findings were highly suggestive of ALS in a patient with a likely pathogenic FIG4 variant.

• The Korean Journal of Pain
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• 제13권1호
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• pp.97-100
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• 2000

Thoracic outlet syndrome (TOS) is a combination of signs and symptoms caused by the compression of the vital neurovascular structure at the thoracic outlet region. It may stem from a number of abnormalities, including degenerative or bony disorders, trauma to cervical spine, fibromuscular bands, vascular abnormalities and spasm of the anterior scalene muscle. CPT (current perception threshold) is defined as the minimum amount of current applied transcutaneously that an individual consciously perceives. It enables quantification of the hyperesthesia that precedes progressive nerve impairment, as well as hypoesthetic conditions. We experienced a case of thoracic outlet syndrome caused by fibrosis of anterior scalene muscle. The patient was a 30 years old woman with a 3 years history of numbness on the ulnar side, progressive weakness and coldness of both hand, tiredness in the left arm, nocturnal pain in the left forearm, and pain in the left elbow, shoulder and neck. Conservative treatment, stellate ganglion block, cervical epidural block, anterior scalene block and previous operation, including both carpal tunnel release, provided no remarkable relief to the patient. A left scalenectomy and first rib resection were performed by transaxillary approach and left cervical root neurolysis was done. After surgery, we measured CPT using neurometer and found conditions worsening in the opposite arm. We performed the same procedure on right side, and followed by CPT measurement. This case suggests that CPT is a useful measurement of recovery and progression of TOS.

• 대한한의학방제학회지
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• 제18권2호
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• pp.267-277
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• 2010

Objective : Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke like episodes (MELAS) syndrome is a progressive neurodegenerative disorder. The typical presentation of patients with MELAS syndrome includes features such as mitochondrial encephalomyopathy, lactic acidosis, and stroke like episodes. Other features, such as seizures, diabetes mellitus, hearing loss, cardiac disease, short stature, endocrinopathies, exercise intolerance, and neuropsychiatric dysfunction are clearly part of the disorder. Approximately 80% of patients with the clinical characteristics of MELAS syndrome have a genetic mutation. This progressive disorder is reported to have a high morbidity and mortality. This case report is intended to estimate clinical effects of oriental meedicinal treatment of MELAS syndrome. Methods : A 44 year old female patient diagnosed as MELSA syndrome was treated with general oriental medicinal therapy including acupuncture, moxibustion, cupping, pharmacupunture and herbal medication in 20XX in Wonkwang Medical Center Gwangju. And the changes in symptoms and signs were evaluated as time dependently. Results : Although there is currently no curable treatment and MEALS syndrome is tend to progress, our treatment showed improvement in general weakness, gait disturbance and pain in the patient. Conclusion : Our case report suggests that various oriental medicinal treatment could be effective for improvement of MELAS syndrome and may represent a new potential therapeutic approach to control the disease. It could be applied to improve general condition, prevent relapse, enhance the quality of life and reduce complaints in the patient.

• 한국간담췌외과학회지
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• 제26권1호
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• pp.69-75
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• 2022

Backgrounds/Aims: Progressive familial intrahepatic cholestasis (PFIC) is an autosomal recessive inherited disease requiring liver transplantation (LT). The objective of this study was to investigate the clinicopathological features and posttransplant courses of seven LT recipients with PFIC. Methods: This was a retrospective single-center study of patients with PFIC who underwent LT from January 2013 to June 2020. Results: Two and five patients were diagnosed with PFIC type 1 and type 2, respectively. For all seven patients, age of PFIC onset was at birth. Jaundice was present in all cases. Mean pretransplant total and direct bilirubin levels were 16.1 ± 8.1 mg/dL and 12.4 ± 6.2 mg/dL, respectively. Median patient age and body weight at LT were 10 months and 7 kg, respectively. Types of donors were mothers of patients in four and deceased donors in three. All five patients with PFIC type 2 recovered uneventfully. One patient each with PFIC type 1 underwent retransplantation due to graft failure or died due to multi-organ failure. Overall graft and patient survival rates at five years were 66.7% and 83.3%, respectively. Bile salt export pump immunohistochemical staining showed normal canalicular expression in two patients with PFIC type 1, focal loss in two patients with PFIC type 2, and total loss in three patients with PFIC type 2. Conclusions: LT is currently the only effective treatment for PFIC-associated end-stage liver diseases. It is mandatory to perform regular follow-up due to the risk of complications including steatohepatitis, especially for patients with PFIC type 1.

• 대한장애인치과학회지
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• 제12권2호
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• pp.96-100
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• 2016

저자는 후기 영아형 이염성 백질 이영양증으로 인해 신경학적인 퇴행이 관찰되고 섭식 장애로 인한 다발성 우식을 보이는 환아를 전신마취 하 치료하였기에 문헌 고찰과 함께 보고하는 바이다. 저작기능 장애로 인한 영양 결핍으로 환아는 신체 발육이 매우 저하되었으며 긴 식사 시간으로 다발성 치아 우식에 이환된 상태였다. 협조도 부족 및 치료 범위 등을 고려하여 전신마취 하에 치과 치료를 시행하였고 정기적인 검진을 시행 중이다. 진행성, 신경성 퇴행질환의 특징을 이해하고 환아의 상태를 고려한 구강 건강 관리 및 보호자 교육을 제공하는 것이 필요할 것으로 사료된다.

• 생물정신의학
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• 제4권1호
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• pp.74-83
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• 1997

With increasing tendency of incidence and interest for the late onset schzophrenia, concerns about whether this disorder is etiologically or phenomenogically distinctive entity or not have increased also. To clarify the disease entity of the late onset schzophrenia and the role of structural brain changes in its etiology, authors tried to prove following hypothesis : Are there any evidences of structural brain changes in the lateonset schizophrenia? ; If present, are they not different from those of the early-onset schizophrenia or progressive schizophrenia? ; And are they not different from those of senile dementia? Subjects were 6 patients with the late-onset schizophrenia, 6 patients with the early-onset schizophrenia, 6 patients with progressive schizophrenia, 6 patients with Alzheimer's dementia, and 6 controls. We measured regions of interest of the magnetic resonance images by computer assisted planimetry using the AutoCad and digitizer. Our study results may suggest that the third ventricular enlargement and a reversal of normal difference between left and right temporal lobe and left-right difference in posterior lateral ventricle are common brain pathology for all types of schizophrenia including the late onset schzophrenia. And also suggest that brain structural changes of the late onset schizophrenia are related with neurodevelopmental abnormality rather than degenerative change.