Histiocytosis-X is a term used to define three diseases with similar morphologic characteristics : Letterer-Siwe diseae, Hand-Schuller-Christian disease and Eosinophilic granuloma. In general, they differ in terms of their age of onset, severity of clinical course and site of involvement. Eosinophilic granuloma typically is seen in young adults. Eosinophilic granuloma is diagnosed in the presense of diffuse pulmonary infiltrate, bony involvement. However, the Pulmonary radiologic findings of eosinophilic granuloma are variable accordinary to stage of disease. therefore pathologic diagnosis of involving site is essential for confirmative diagnosis of eosinophilic grananuloma. Pathologically. the three disease are characterized by granulomatous infiltration of alveolar septa and bronchial walls and often involvement of bone. The hallmark of this disease is proliferation of the Langerhans' cell. The identifying feature is the X-body or Birbeck granule that is present in Langerhans' cells and histiocytic cells found in the lung of EG patient. We report a case of bilateral, recurrent and spontaneous pneumothoraces in a 21 year old man with pulmonary histiocytosis-X which is confined by eosinophilc granuloma in bone marrow biopsy and ultrastructural examination in cells obtained from BAL.
Age of Onset ; Biopsy ; Bone Marrow ; Diagnosis ; Eosinophilic Granuloma ; Eosinophils ; Granuloma ; Histiocytosis, Langerhans-Cell* ; Humans ; Lung ; Pneumothorax* ; Young Adult

Mucinous ductal ectasia(MDE) is a newly described entity of mucinous neoplasm of pancreas with characteristic endoscopic and pancreatographic findings. It is charaeterized by a patulous duodenal papilla extruding mucus and a pancreatogram showing dilatation with amorphous filling defects, communication of the mass with the pancreatic duct. MDE is intraductal lesion consisting of dilated "cystified" ducts lined by mucin-producing columnar cells. The lesion is usually located in the head or uncinate process. When the radiographic appearance of the ERCP cannot provide sufficient information for definite diagnosis, additional endoscopic visualization is desirable. With the advent of pancreatoscopy, an endoscopic procedure is now available that has proven to enhance diagnostic accuracy. The 0.8-mm ultrathin pancreatoscope allows macroscopic diagnosis under direct vision. Endoscopic sphincterotomy is not required, and insertion into the pancreatic duct is feasible in most cases with the aid of guidewires. Major indications are unclear filling defects on ERCP, strictures of uncertain origin, and duct cut-offs. A limitation of this procedure are the lack of angulation, insufficient illumination, fragility of endoscopic equipment, and nonspecific findings. In addition, biopsy while directly viewing the lesion is impossible at present. We experienced one case of MDE who presented with the symptom of weight loss. Ultrathin pancreatoscopy was useful for the direct visualization af pancreatic duct in the differential diagnosis of filling defects of main pancreatic duct.
Biopsy ; Cholangiopancreatography, Endoscopic Retrograde ; Constriction, Pathologic ; Diagnosis ; Diagnosis, Differential ; Dilatation ; Dilatation, Pathologic* ; Head ; Lighting ; Mucins* ; Mucus ; Pancreas ; Pancreatic Ducts ; Sphincterotomy, Endoscopic ; Weight Loss

Periodic limb movements during sleep (PLMS) are frequently observed in the general population, although such movements may be associated with a variety of medical and neurological disorders. Human T-lymphotropic virus type I-associated myelopathy (HAM) is a rare progressive disease in which abnormalities are rarely observed on spinal images. We present the case of a 55-year-old woman with PLMS who was later diag-nosed with HAM. The current case indicates that HAM can be considered a possible cause of PLMS.
Extremities* ; Female ; Human T-lymphotropic virus 1 ; Humans* ; Middle Aged ; Nervous System Diseases ; Nocturnal Myoclonus Syndrome ; Spinal Cord Diseases*

Flail arm syndrome (FAS), an atypical presentation of amyotrophic lateral sclerosis (ALS), is characterized by progressive, predominantly proximal, weakness of upper limbs, without involvement of the lower limb, bulbar, or respiratory muscles. When encountering a patient who presents with this symptomatic profile, possible diagnoses include upper limb onset ALS (UL-ALS), and FAS. The lack of information regarding FAS may make differential diagnosis between FAS and UL-ALS difficult in clinical settings. The aim of this study was to compare clinical and electromyographic findings from patients diagnosed with FAS with those from patients diagnosed with UL-ALS. To accomplish this, 18 patients with FAS and 56 patients with UL-ALS were examined. Significant differences were observed between the 2 groups pertaining to the rate of fasciculation, patterns of predominantly affected muscles, and the Medical Research Council scale of the weakest muscle. The presence of upper motor neuron signs and lower motor neuron involvement evidenced through electromyography showed no significant between-group differences.
Amyotrophic Lateral Sclerosis* ; Arm* ; Diagnosis ; Diagnosis, Differential ; Electromyography ; Fasciculation ; Humans ; Lower Extremity ; Motor Neuron Disease ; Motor Neurons ; Muscles ; Respiratory Muscles ; Upper Extremity*

To understand the changes in expression of calcium binding proteins(CaBP) during the experimental focal ischemia, expression of two kinds of CaBP, paralvumin(PV) and calbindin D-28K(Calbindin), immunocytochemically, and activities of cytochrome oxidase(CO) and acetylcholinesterase(AchE), histochemically, in focal ischemic brain of the rat were investigated. Two groups of focal ischemic infarction were produced in Sprague Dawley rats(200-350 mg):Group I, Clip compression of left middle cerebral artery(MCA) for 5-10 mins and release;Group II, Electric coagulation of left MCA for 2-24 hrs. In the group I, CO activity and PV- and Calbindin-immunoreactivity(IR) were decreased in the left MCA territory, and decreased in number of PV- and Calbindin-IR neurons and degree of IR, but AchE activity was nearly same as that of control cortex. In the group II, decrease of CO and AchE activities, and marked increase of PV- and Calbindin IRs were noted on neuropil in the layers I through VI of ischemic region. Characteristically pyramidal cells, which did not express the both CaBPs in the control cortex, of layer V of ischemic cortex showed PV- and Calbindin Irs in the cell body and apical dendrite. These findings suggest that 1) PV- and Calbindin-IR neurons, mainly non-pyramidal cells, are more vulnerable than pyramidal cell to ischemic injury, 2) CaBP may have some roles in hypoxic neuronal injury, and 3) PV and Calbindin-immunocytochemistry can be used as useful technique in evaluation of experimental ischemia.
Animals ; Brain ; Calbindins ; Calcium ; Cytochromes ; Dendrites ; Infarction ; Ischemia* ; Neocortex* ; Neurons* ; Neuropil ; Pyramidal Cells ; Rats*

Pituitary apoplexy is a life-threatening condition resulting from hemorrhage or necrosis of a pituitary tumor with subsequent compression of the optic nerves and cavernous sinuses. This is the first case report of a patient who experienced pituitary apoplexy due to hemorrhage of pituitary adenoma, which was initially recognized during pregnancy, and submitted to minimally invasive neuroendoscopic transnasal transsphenoidal approach in the third trimester of pregnancy.
Cavernous Sinus ; Female ; Hemorrhage ; Humans ; Necrosis ; Optic Nerve ; Pituitary Apoplexy* ; Pituitary Neoplasms ; Pregnancy Trimester, Third ; Pregnancy*

Guanine aminohydrolase (GAH; Guanine deaminase, EC 3.5.4.3) is an enzyme that has a role in purine catabolism. This enzyme produces xanthine and ammonia by hydrolysis of guanine, and xanthine is further degraded to uric acid and hydrogen peroxide by another enzyme, xanthine oxidase. Most of the enzymes involved in purine catabolism have been studied for their biological functions, physiological roles and amino acid sequences, and biochemical activity of GAH is known to be detected in various organs such as liver, kidney, small intestine and brain. Its activity is also known to be changed during brain development. In this study, we hoped to reveal expression pattern of GAH in developing rat brain by western blotting and immunohistochemistry. In western blotting, GAH immunoreactivity was not detected on 14-, 16- and 18-days-old fetal rat brains. Its reactivity was first detected from 20-days-old fetal rat brain and highly increased after birth. And it was maintained at steady level from 2 weeks after birth. In immunohistochemistry, no positive cells were found on 14- and 16-days-old fetal rat brain sections. A few GAH-immunoreactive cells appeared from 18-days-old fetal rat brain and they were localized at olfactory bulb, cerebral cortex, midbrain, pons and medulla. The 20-days-old fetal rat brain also showed immunoreactive cells at hippocampus and the staining intensity was still weak. Postnatal 2-days-old rat brain also showed immunoreactive cells at basal ganglia and the number of positive cells and staining intensity were increased. Thereafter, immunoreactivity appeared on many neuronal cells around various areas in the brain and nerve fibers also showed reactivity on postnatal brains. The number of positive cells decreased from 1 week after birth and a few positive cells were observed on olfactory bulb and cerebellum from 2 weeks after birth. In mature brain most of GAH were localized on nerve fibers and few positive cells could be found on olfatory bulb only. From these, we can suspect that GAH may have some functional relationship with nerve fibers.
Amino Acid Sequence ; Ammonia ; Animals ; Basal Ganglia ; Blotting, Western ; Brain* ; Cerebellum ; Cerebral Cortex ; Guanine Deaminase* ; Guanine* ; Hippocampus ; Hope ; Hydrogen Peroxide ; Hydrolysis ; Immunohistochemistry ; Intestine, Small ; Kidney ; Liver ; Mesencephalon ; Metabolism ; Nerve Fibers ; Neurons ; Olfactory Bulb ; Parturition ; Pons ; Rats* ; Uric Acid ; Xanthine ; Xanthine Oxidase

Background: This is the first clinical study conducted among Asian women using breast implants manufactured by an Asian company. Four-year data regarding the safety and efficacy of BellaGel breast implants have already been published, and we now report 6-year data. Methods: This study was designed to take place over 10 years. It included 103 patients who underwent breast reconstruction or augmentation using BellaGel breast implants. The rates of implant rupture and capsular contracture were measured and analyzed to evaluate the effectiveness of the breast implant. Results: At patients’ 6-year postoperative visits, the implant rupture and capsular contracture rates were 1.15% and 2.30%, respectively. The implant rupture rate was 3.77% among reconstruction cases and 0% among augmentation cases. The capsular contracture rate was 5.66% among reconstruction cases and 0.83% among augmentation cases. Conclusions The 6-year data from this planned 10-year study suggest that the BellaGel cohesive silicone gel-filled breast implant is an effective and safe medical device that can be used in breast reconstruction and augmentation.

BACKGROUND: Several epidemiological studies have shown that high plasma concentration of lipoprotein(a) [Lp(a)] is associated with an increased risk for atherosclerotic cardiovascular disease and works as an independent risk factor for atherosclerosis. But, the significance of Lp(a) in diabetic microangiopathy & neuropathy is unclear essentially due to a paucity of relevant studies. This study was designed to evaluate whether Lp(a) concentration may be increased in patients with diabetic microangiopathy & neuropathy. METHODS:We studied 96 patients who visited the department of internal medicine in Pusan National University Hospital from May 1995 to May 1996. The patients were grouped according to the presence of diabetic complications(microangiopathy and neuropathy, microangiopathy included retinopathy and nephropathy) and therapeutic modalities(diet, insulin, insulin with oral hypoglycemic agent, and oral hypoglycemic agent). RESULTS: 1) Concentration of Lp(a) was significantly higher(p < 0.05) in patients with diabetic retinopathy(nonproliferative, 38.6+/-33.6 mg/dl, proliferative, 39.5+/-32.1 mg/dl) than that of patients without retinopathy(23.3+/-25.3 mg/dl). The duration of diabetes was significantly longer(p < 0.05) in patients with diabetic retinopathy(nonproliferative, 12.0 years, proliferative, 13.2 years) than that of patients without retinopathy(5.9 years). 2) Concentration of Lp(a) was significantly higher(p < 0.05) in patients with diabetic nephropathy(36.5+/-39.3 mg/dl) than that of patients without nephropathy(23.3+/-17.8 mg/dl) and the duration of diabetes was also longer in patients with diabetic nephropathy(10.7+/-7.2 years vs 6.3+/-5.8 years, p < 0.005). 3) Concentration of Lp(a) was significantly higher and the duration of diabetes was longer in patients with diabetic neuropathy than that of patients without neuropathy(35.9+/-31.7 mg/dl vs 23.2+/-25.1 mg/dl, p < 0.05 and 10.8 years vs 6.2 years, p < 0.005). 4) Concentration of Lp(a) was significantly higher in patients with three complications(53.6 mg/dl, p < 0.005) and duration of diabetes was significantly longer in patients with two or three complications(11.3 years, 13.6 years, respectively, p < 0.0001). than those in patients without complications. 5) When the patients were subgrouped according to the treatment modalities, there were no significant difference in Lp(a) concentration, however the duration of diabetes was longer in patient group treated with combination of insulin and oral hypoglycemics than that of the other groups(p < 0.05). 6) In multivariate logistic regression analysis, concentration of Lp(a) > or = 50 mg/dl was significantly correlated with diabetic retinopathy & nephropathy, but was not significantly correlated with diabetic neuropathy. Duration of diabetes(> or =7 years) and total cholesterol(> or =240 mg/dl) were significantly correlated with diabetic retinopathy, nephropathy and neuropathy. CONCLUSIONS: Lp(a) concentration is increased in patients with diabetic microangiopathy and neuropathy compared with patients without these complications. So, Lp(a) may works as risk factor for diabetic microangiopathy and neuropathy, and further study to evaluate the role of Lp(a) as a risk factor of such complications would be necessary in large number of patients.
Atherosclerosis ; Busan ; Cardiovascular Diseases ; Diabetic Angiopathies* ; Diabetic Neuropathies ; Diabetic Retinopathy ; Epidemiologic Studies ; Humans ; Hypoglycemic Agents ; Insulin ; Internal Medicine ; Lipoprotein(a)* ; Logistic Models ; Plasma ; Risk Factors

BACKGROUND AND PURPOSE: Upper respiratory infection (URI), including influenza, may exacerbate the symptoms of myasthenia gravis (MG), which is an autoimmune disease that causes muscle weakness. There is also concern that the influenza vaccine may trigger or worsen autoimmune diseases. The objective of this study was to determine the impacts of influenza infection and vaccination on symptom severity in MG patients. METHODS: Patients diagnosed with MG were enrolled from 10 university-affiliated hospitals between March and August 2015. Subjects completed a questionnaire at the first routine follow-up visit after enrolling in the study. The patient history was obtained to determine whether a URI had been experienced during the previous winter, if an influenza vaccination had been administered before the previous winter, and whether their MG symptoms were exacerbated during or following either a URI or vaccination. Influenza-like illness (ILI) was defined and differentiated from the common cold as a fever of ≥38℃ accompanied by a cough and/or a sore throat. RESULTS: Of the 258 enrolled patients [aged 54.1±15.2 years (mean±SD), 112 men, and 185 with generalized MG], 133 (51.6%) had received an influenza vaccination and 121 (46.9%) had experienced a common cold (96 patients) or ILI (25 patients) during the analysis period. MG symptoms were aggravated in 10 (40%) patients after ILI, whereas only 2 (1.5%) experienced aggravation following influenza vaccination. The rate of symptom aggravation was significantly higher in patients experiencing an ILI (10/25, 40%) than in those with the common cold (15/96, 15.6%, p=0.006). CONCLUSIONS: The results of this study suggest that the potential risk of aggravating autoimmune disease is higher for ILI than for influenza vaccination, which further suggests that influenza vaccination can be offered to patients with MG.
Autoimmune Diseases ; Common Cold ; Cough ; Fever ; Follow-Up Studies ; Humans ; Influenza Vaccines ; Influenza, Human* ; Male ; Muscle Weakness ; Myasthenia Gravis* ; Pharyngitis ; Vaccination*