The paraproteinemia is a disorder in which a single clone of plasma cells (monoclonal gammopathy) is responsible for the proliferation of monoclonal proteins (M-proteins). Approximately 10% of patients with idiopathic peripheral neuropathy have monoclonal gammopathy. Some M-proteins have the properties of an antibody to the components of peripheral nerve myelin, but the pathophysiological relationship between the neuropathy and the M-protein is often obscure. The relationship between peripheral neuropathy and monoclonal gammopathy requires the appropriate neurological and hematological investigations for precise diagnosis and treatment. In this review, we provide an update on the causal associations between peripheral neuropathy and monoclonal gammopathy as well as characteristics of clinical and electrophysiologic features.
Clone Cells ; Diagnosis ; Humans ; Myelin Sheath ; Paraproteinemias ; Peripheral Nerves ; Peripheral Nervous System Diseases ; Plasma Cells ; Polyneuropathies

Takayasu's arteritis is a chronic inflammatory disease of unknown etiology involving the aorta, major branches of aorta, and pulmonary arteries and leads either stenosis and occlusion of the involved artery or aneurysm formation or both. The clinical course and prognosis are variable according to two major prognostic factors, ie, complications and the pattern of the past clinical course, as well as by ESR. Though the aggressive medical and surgical treatment are required for patients with a major complication and a progressive course, surgical reconstruction entails a high incidence of suture line complications including stenosis or dilatation. Moreover all the vascular lesions are amenable for vascular surgery. Initial reports revealed excellent results of percutaneous transluminal angioplasty (PTA) in patients with Takayasu's arteritis. However the suboptimal results and restenosis have been the main limitations of the PTA. Stenting has some benefits for early elastic recoil of the fibrotic vessels and restenosis as in other large vessels in Takayasu's arteritis or atherosclerosis. We report early and long-term results of two cases of carotid stenting in patients with symptomatic carotid stenosis and Takayasu's arteritis, which revealed variable angiographic results according to clinical courses and recommend that stenting in Takayasu's arteritis may be another treatment modality in inactive Takayasu's arteritis.
Aneurysm ; Angioplasty ; Aorta ; Arteries ; Atherosclerosis ; Carotid Arteries* ; Carotid Stenosis ; Constriction, Pathologic ; Dilatation ; Follow-Up Studies* ; Humans ; Incidence ; Prognosis ; Pulmonary Artery ; Stents* ; Sutures ; Takayasu Arteritis

No abstract available.
Antibodies, Antineutrophil Cytoplasmic ; Fever* ; Ulcer ; Wegener Granulomatosis

Objective: Atopic dermatitis (AD) is a chronic inflammatory disease of the skin that is diagnosed by clinical characteristics including itchiness, eczema, dry skin, etc. High levels of indoor air pollutants may exacerbate atopic diseases, along with various allergic respiratory diseases, especially for those who spend most of their lives indoors. This study was conducted to evaluate the main components responsible for the aggravation of AD symptoms. Methods: A total of 31 patients with AD aged 6 months and 6 years old were enrolled. The measurement of air quality included particulate matter with a diameter of 2.5 μm or less (PM2.5), temperature, relative humidity, and CO2 in their homes. The symptom severity of AD was assessed by the daily record of symptom scores and the degree of skin hydration. Results: The skin hydration level at the most severe area selected by the patient’s caregiver was decreased by median 1.7% (interquartile range [IQR], 0.4%–3.0%) or median 15% (IQR, 5.3%–24%) with a unit increase of indoor PM2.5 (P=0.0133) or room temperature (P=0.0034). CO2 also showed a potentially negative association with the change of skin hydration level but it was not statistically significant. Conclusion Our study showed that indoor PM2.5 and temperature could impact the aggravation of skin hydration in children. Therefore, further studies including a large number of cases and interventions are necessary.

Objective: Atopic dermatitis (AD) is a chronic inflammatory disease of the skin that is diagnosed by clinical characteristics including itchiness, eczema, dry skin, etc. High levels of indoor air pollutants may exacerbate atopic diseases, along with various allergic respiratory diseases, especially for those who spend most of their lives indoors. This study was conducted to evaluate the main components responsible for the aggravation of AD symptoms. Methods: A total of 31 patients with AD aged 6 months and 6 years old were enrolled. The measurement of air quality included particulate matter with a diameter of 2.5 μm or less (PM2.5), temperature, relative humidity, and CO2 in their homes. The symptom severity of AD was assessed by the daily record of symptom scores and the degree of skin hydration. Results: The skin hydration level at the most severe area selected by the patient’s caregiver was decreased by median 1.7% (interquartile range [IQR], 0.4%–3.0%) or median 15% (IQR, 5.3%–24%) with a unit increase of indoor PM2.5 (P=0.0133) or room temperature (P=0.0034). CO2 also showed a potentially negative association with the change of skin hydration level but it was not statistically significant. Conclusion Our study showed that indoor PM2.5 and temperature could impact the aggravation of skin hydration in children. Therefore, further studies including a large number of cases and interventions are necessary.

BACKGROUND: Currently available data suggests that uric acid (UA) functions as an antioxidant after acute ischemic stroke (AIS). Nevertheless, the prognostic value of serum UA in AIS is controversial. The aim of this study was to determine the relationship between UA and functional outcomes after AIS. METHODS: UA levels were analyzed within 48 hours of stroke onset in patients between 2007 and 2012. Mean serum UA levels were compared between patients with good and poor functional outcomes (modified Rankin Scale [mRS] score, 0-2 versus 3-6, respectively) at 3 months poststroke, and with and without early neurological improvement (ENI, > or = 4-versus <4-point differences on the National Institutes of Health Stroke Scale [NIHSS] score after 7 days). RESULTS: Serum UA levels differed according to the sex, age, stroke subtype, and presence of diabetes mellitus, smoking, and atrial fibrillation. Multivariate logistic regression analysis revealed an association between good functional outcome at 3 months and male gender, young age, history of dyslipidemia, good functional status before stroke (mRS score, 0-2), and low stroke severity (i.e., NIHSS score) on admission. However, higher serum UA levels were no longer associated with a good functional outcomes (odds ratio, 1.387; 95% confidence interval, 0.857-2.244; p=0.521). The mean serum UA levels did not differ significantly between patients with and without ENI. CONCLUSIONS: There was no association between serum UA levels and functional outcomes at 3 months in AIS.
Atrial Fibrillation ; Cerebral Infarction ; Cerebrovascular Disorders ; Diabetes Mellitus ; Dyslipidemias ; Humans ; Logistic Models ; Male ; Myocardial Infarction ; National Institutes of Health (U.S.) ; Smoke ; Smoking ; Stroke* ; Uric Acid*

Lymphedema is a common complication after mastectomy in breast cancer patients. Many treatment options are available, but no treatment results in a complete cure. We report a case of lymphedema that occurred after modified radical mastectomy in a breast cancer patient who showed objective improvement after delayed breast reconstruction with an latissimus dorsi myocutaneous flap. A 41-year-old female patient with left breast cancer had undergone modified radical mastectomy with axillary lymph node dissection and postoperative radiotherapy 12 years previously. Four years after surgery, lymphedema developed and increased in aggravation despite conservative treatment. Eight years after the first operation, the patient underwent delayed breast reconstruction using the extended latissimus dorsi myocutaneous flap method. After reconstruction, the patient's lymphedema symptoms showed dramatic improvement by subjective measures including tissue softness and feeling of lightness, and by objective measures of about 7 mL per a week, resulting in near normal ranges of volume. At a postoperative follow-up after 3 years, no recurrence was observed. Delayed breast reconstruction with extended latissimus dorsi myocutaneous flaps may be helpful to patients with lymphedema after mastectomy. This may be a good option for patients who are worried about the possibility of the occurrence or aggravation of secondary lymphedema.
Adult ; Breast ; Breast Neoplasms ; Female ; Follow-Up Studies ; Humans ; Light ; Lymph Node Excision ; Lymphedema ; Mammaplasty ; Mastectomy ; Mastectomy, Modified Radical ; Recurrence ; Reference Values ; Surgical Flaps ; Upper Extremity

Frontometaphyseal dysplasia is a rare genetic syndrome first described by Gorlin and Cohen in 1969. This disease affects the skeletal system and connective tissue, and could be characterized by hyperostosis of the skull, prominence of supraorbital ridges, additional skeletal and extraskeletal abnormalities. It is believed that the condition is an X-linked dominant trait with severe manifextations in males and extreme variability in females. We described a case of 15-year-old boy manifested a pronounced supraorbital ridge associated with exorbitism. He also had bilateral progressive hearing deficit, thoracic spine scoliosis, chest wall deformity, bilateral maxillary sinusitis and both 5th finger arachnodactyly. The patient underwent a fronto-temporo-orbital cranioplasty through a coronal incision. The frontal bone including supraorbital region, orbital lateral rim and temporal bone were extensively contoured with burr. And then, burring of the medial aspect of lateral orbital wall was made to increase both orbital volume for correction of exorbitism. Postoperative results show well corrected prominent supraorbital ridge, hyperostosis of frontotemporal bone and exorbitism. The patient was satisfied with the improved appearance. No recurrence occurred during the 6 months of follow-up period. We report this case as it shows esthetically good result without any complication.
Adolescent ; Arachnodactyly ; Congenital Abnormalities ; Connective Tissue ; Female ; Fingers ; Follow-Up Studies ; Frontal Bone ; Hearing ; Humans ; Hyperostosis ; Male ; Maxillary Sinus ; Maxillary Sinusitis ; Orbit ; Recurrence ; Scoliosis ; Skull ; Spine ; Temporal Bone ; Thoracic Wall

AIDS related malignancies have gradually decreased after the introduction of highly active antiretroviral therapy (HAART). Nevertheless, non-Hodgkin's lymphoma is still a major malignancy in resource limited countries that have difficulty supplying HAART, and same holds true for Korea. We report a case of non-Hodgkin's lymphoma involving the heart in a Korean AIDS patient, who presented with sudden arrhythmia and superior vena cava syndrome. He was diagnosed with AIDS in 2008, but refused to receive HAART. Immunohistologic findings were compatible diffuse large B cell lymphoma, and futher studies demonstrated involvement of the liver, adrenal gland, heart, and pericardiac space. HAART was initiated and non Hodgkin's lymphoma was successfully managed with intensive chemotherapy. This is the first cardiac involvement non Hodgkin's lymphoma in AIDS patients in Korea.
Adrenal Glands ; Antiretroviral Therapy, Highly Active ; Arrhythmias, Cardiac ; Heart ; Humans ; Korea ; Liver ; Lymphoma, B-Cell ; Lymphoma, Non-Hodgkin ; Superior Vena Cava Syndrome

A nipple can be reconstructed either by nipple sharing or local flap. But donor site scar is inevitable when nipple is reconstructed with local flap method. The scar which remains inside the nipple areolar complex can be hidden by medical tattooing, but elongated scar, which cross over the areolar border leads to poor esthetic result. So as to solve this problem, we used purse string suture method. We reconstructed nipple with C-V flap for 9 patients who previously reconstructed breast with extended latissimus dorsi flap. Purse string suture with 3-0 nylon was used for donor site closure. We minimize 10.7% of the scar length with this method. Enhancement of nipple projection due to the flattening of V limb was attained in addition. We expect the reduction of V-limb of C-V flap and amount of absorption of the reconstructed nipple with purse string suture method.
Absorption ; Breast ; Cicatrix ; Extremities ; Humans ; Nipples* ; Nylons ; Superficial Back Muscles ; Sutures ; Tattooing ; Tissue Donors