Authors report a typical case of congenital fiber type disproportion (CFTD) with unique clinicopathologic characteristics. The patient was a 13-year-old boy who presented with weakness of lower extremities, especially proximal muscle, since his infancy. He has suffered from severe scoliosis which got worse since the age of 12. He showed mild dysarthria, high arched palate, and fish face. All routine laboratory data were within normal limits. EMG findings suggested myopathy. The muscle biopsy revealed fiber type disproportion with type 1 predominance. While most of the type 1 myofibers were atrophic or normal in size, the type 2 fibers showed universal hypertrophy. The difference of mean diameter between the larger and the smaller fibers was 27.9%. The patient's clinicopathologic settings fulfilled the criteria of CFTD.
Adolescent ; Biopsy ; Dysarthria ; Humans ; Hypertrophy ; Lower Extremity ; Male ; Muscular Diseases* ; Myopathies, Structural, Congenital* ; Palate ; Scoliosis

No abstract available.
Colon* ; Colonic Neoplasms* ; Dimenhydrinate* ; Oncogenes* ; Rats, Wistar*

No abstract available.
Birds* ; Orientia tsutsugamushi* ; Rickettsia*

Clinical observation was done on 302 cases of cerebrovascular accidents admitted at Sung-Sim hospital, Chung-Ang University from January, 1968 to August, 1976. 1) Of 302 cases of cerebrovascular accidents, the incidence of cerebral hemorrhage was 28.8, cerebral thrombosis 47,7 Subarachnoid hemorrhage 20.2% and cerebral embolism 3.3%. 2) The peak age incidence was in the fifth decade in cerebral hemorrhage, subarachnoid hemorrhage, whereas in cerebral thrombosis, it was in the sixth decade. 3) The most frequent predisposing factor in cerebral hemorrhage and subarachnoid hemorrhage were physical activity and emotional stress, whereas in thrombosis and embolism, it was rest. 4) Among disease preceding the onset of cerebrovascular accidents, hypertension were presented 65.4% in cerebral hemorrhage, 63.6% in cerebral thrombosis, 56.6% in subarachnoid hemorrhage and valvular heart disease was presented 40.0% in cerebral embolism. 5) Serum cholesterol level over 200mg% was seen in 33.0% of cerebrovascular accidents. 6) Leukocytosis was seen 58.6% of cerebrovascular accidents which was predominantly found in the hemorrhagic group. 7) The pressure of cerebrospinal fluid was elevated in 61.7% of cerebrovascular accidents, predominantliny the hemorrhagic group. 8) The peak duration of admission was present in 51.7% of cerebrovascular accidents within 7 days and mortality rate during hospitalization was 38.0% in cerebral hemorrhage, 10.4% in cerebral thrombosis and 27.8% in subarachnoid hemorrhage. 9)The mortality rate during hospitalization was 35.3% in all cerebrlavascular accidents within 24 hours.
Causality ; Cerebral Hemorrhage ; Cerebrospinal Fluid ; Cholesterol ; Embolism and Thrombosis ; Heart Valve Diseases ; Hospitalization ; Hypertension ; Incidence ; Intracranial Embolism ; Intracranial Thrombosis ; Leukocytosis ; Mortality ; Motor Activity ; Stress, Psychological ; Stroke ; Subarachnoid Hemorrhage

No abstract available.
Heart Valve Prosthesis* ; Heart Valves*

Since the first report by Drury and Szent-Gyorgyi in 1929, the inhibitory influences of adenosine on the heart have repeatedly been described by many investigators. A lot of investigations on the working mechanisms of adenosine have been focused mainly on the effects on the coronary blood flow. However, the cellular mechanisms underlyiag the inhibitory action of adenosine on the SA node are not well understood yet. Furthe-rmore, the physiological role of adenosine in the regulation of the heart beat remains still to be explored. Thus, this study was undertaken to examine the behavior of the rabbit SA node ander the influence of adenosine, and the interactions between adenosine and aminophylline on the SA node, and then to compare these results with those of acetylcholine. At the same dosage range, adenosine suppressed the sinus rate and atrial contractility even in the reserpinized preparation. The spontaneous firing rate of the SA node at 35degrees C (mean+/-SEM, n=16) was 154+/-3.3 beats/min. The parameters of action potential were: maximum diastolic potential(MDP), -73+/-1,7 mV; overshoot(OS), 9+/-1.4 mV; slope of pacemaker potential(SPP), 94+/-3.0 mV/sec. Adenosine suppressed the firing rate of the SA node in a dose-dependent manner. This inhibitory effect appeared at the concentration of 10(-4)M and was potentiated in parallel with the increase in adenosine concentration. Changes in the action potential by adenosine were dose-dependent as show by the increase of MDP and the decrease of SPP until 10(-4)M. Above this concentration, however, the amplitude of the action potential decreased markedly due to the simultaneous decrease of both MDP and OS. Dipyridamole, which is known to block the adenosine transport aross the cell membrane, definately potentiated the action of adenosine. The effects of adenosine on the SA node were inhibited by aminophylline. However, the similar effects of acetylcholine to those of adenosine were not reversed by aminophylline. These results suggest that adenosine suppressed the pacemaker activity by acting dire-ctly on the membrane of the SA node, and the effects of adenosine on SA node are sele-ctively inhibited by aminophylline.
Acetylcholine ; Action Potentials ; Adenosine ; Aminophylline ; Cell Membrane ; Dipyridamole ; Fires ; Heart ; Humans ; Membranes ; Research Personnel

Tracheal gaenesis is an extremely rare congenital anomaly and is characterized by totally or partially undeveloped trachea. In many instances, the lesion has been associated with malformations in other organ systems. This anomaly is classified into 3 types based on developmental period of the respiratory tract. We report a case of tracheal agenesis with tracheoesophageal fistula in a 2-hour-old male. Postmortem examination revealed that the upper and mid portion of the trachea was absent but 1 cm segment of the trachea above the carina was present. The proximal end to this trachea opened to the esophagus by a fistula, 1 cm in diameter. The well-formed larynx ended blindly in a fibrous sac at which thyroids and cartilage islands are noted. Associated anomalies were bilobed right lung and single umbilical artery.

Adenoid cystic carcinoma is a rare disease of which incidence is 10-15 % of bartholins gland carcinoma. The clinical presentation is characterized by a vulval mass that existed for a prolonged period to the onset of symptoms, usually infection, pain, and burningsensation Histologically, this tumor is characterized by cribriform pattern cell growing and perineural invasion. The obtaining of clear surgical margins is the most important aspect of treatment. Here we report a case of adenoid cystic carcinoma of the Bartholins gland with a brief review of literature.
Adenoids* ; Carcinoma, Adenoid Cystic* ; Incidence ; Rare Diseases

Congenital self-healing reticulohistiocytosis (CSHRH) is a rare Langerhans cell disorder usually showing spontaneous resolution within 3-4 months. By electron microscopy, the identification of Birbeck granules and laminated dense bodies in the infiltrated cells is mandatory for the diagnosis of CSHRH. However, in some reported cases, Birbeck granules could not be demonstrated and only cytoplasmic dense bodies were seen. If the lesion is more advanced, Birbeck granules are transformed to lysosomes, i.e., 'unique phagosomes', in which they are degraded. A 2-month-old Korean girl presented with congenital, numerous red-brown pigmented papules on the left side of trunk and upper extremity without systemic symptoms. A biopsy specimen demonstrated papillary dermis containing epidermotropic infiltrates of histiocytes with abundant eosinophilic cytoplasm. Some had kidney-shaped nuclei and PAS-positive cytoplasmic inclusions. Immunohistochemically, infiltrating cells expressed S-100 protein and ultrastructurally, no Birbeck granules but many dense laminated bodies and unique phagosomes were found. It was ten months since the skin lesions developed that they have started resolving.
Biopsy ; Cytoplasm ; Dermis ; Diagnosis ; Eosinophils ; Female ; Histiocytes ; Humans ; Inclusion Bodies ; Infant ; Lysosomes ; Microscopy, Electron ; Phagosomes ; S100 Proteins ; Skin ; Upper Extremity

We report a case of angiosarcoma of the face and scalp in a 76-year-old man. Immunochemical staining with factor 8-related antigen and ultrastructural find ings confirm that this tumor was origined from endothelium. The patient had a good initial response to treatment with radiotherapy.
Aged ; Endothelium ; Factor VIII ; Hemangiosarcoma* ; Humans ; Radiotherapy ; Scalp*