Primary tumors of the adrenal cortex are comparatively rare. In the medical literature they are most frequently reported as single case. Because of their infrequent occurence some features of their clinical behavior and pathologic anatomy are not widely known. For this reason the recording of the 7 cases from the Department of Pathology, School of Medicine, Chungnam National University is thought to be worth while. Of the seven tumors, five were associated with primary aldosteronism, one Cushing's syndrome, and the remaing one virilizing syndrome. All were benign. Females were affected more frequently than males. Adrenocortical adenomas tend to be small, weighing less than 42 gm. The tumors associated with primary aldosteronism were composed of zona fasciculata-like cells and "hybrid" cells. The tumor with Cushing's syndrome consisted of zona reticularis-like cells. The tumor with virilizing syndrome consisted of zone reticularis-like cells.
Female ; Humans ; Adenoma

No abstract available.
Congenital Abnormalities*

Cubitus varus is a complex three-dimentsional deformity. In addition to deformity on varus-valgus plane, internal torsion of distal humerus is frequently combined. Also limitation of flexion-extension could be associated in some patients. Surgical correction of the cubitus varus after skeletal maturity, could have some different clinical course from those of growing children. However there have been few papers dealing with the surgical treatment of cubitus varus in adults. From May 1988 to December 1993, 23 patients who were older than 17 years of age, had correc- tive osteotomy for cubitus varus considering its three planes of the deformity at Seoul National University Hospital. To prevent serpentine deformity medial displacement of distal fragment was also combined during operation. The avereage age was 26 years(17-47), and the average follow-up after the operation was 1 year and 6 months(6 months-3 years). The presumed diagnosis of initial injury deduced from history and preoperative radiograms, was supracondylar fracture in 19 cases(86.4%). Others were lateral condylar fracture of humerus in two, and fractures associated with elbow dislocation in two. The average age at the time of initial injury was 8 years(2-14), and average delay from occurrence of deformity to the operation, were 13 years. Tardy ulnar nerve palsy was associated in 3 cases(13.6%). The preoperative average angle of cubitus varus was 26 degrees(16-45), flexion contracture 11 degrees(-5
Adult ; Bony Callus ; Child ; Congenital Abnormalities ; Contracture ; Diagnosis ; Dislocations ; Elbow ; Follow-Up Studies ; Humans ; Humerus ; Myositis Ossificans ; Osteotomy ; Paralysis ; Radial Nerve ; Range of Motion, Articular ; Seoul ; Tourniquets ; Traction ; Ulnar Neuropathies

There has been a few articles dealing with the incidence of congenital anomalies of the hand in Korea. In this paper, we have analyzed patients who have visited the hand clinic of Seoul National University Children s Hospital from March 1993 to February 1995 to study the relative incidence of congenital anomalies of the hand. We regarded more than two visits of the same patients as one. They were categorized into two groups by our classification system. First group consisted of the patients who showed hand anomaly only. The other group consisted of the patients who had anomalies in the other sites as well as in the hand. The first group was divided into two subgroups; the patients who had single entity of hand anomaly, and those who had more than two entities of hand anomalies. From the clinical analysis based on above criteria, following results were obtained. There were 626 anomalies in 547 patients, 319 (58.3%) males and 228 (41.7%) females (1.4: 1). Unilateral involvements of hand anomaly were noted in 385 (70.3%) patients (229 in right, 156 in left) and bilateral in 162 (29.7%). As a whole, the most common anomaly was trigger thumb (16.5%) and the second was polydactyly (16.1%). Other anomalies, in order of frequency, were syndactyly, camptodactyly, hypoplasia, brachydactyly, cleft hand and so on. In the first group who had hand anomaly only, there were 407 patients who had single entity of hand anomaly. Among these, trigger thumb were 25.3%, and polydactyly 16.2%. There were 68 patients who had multiple entities of anomalies. Among these, the syndactyly was the most common anomaly (23.9%) and the polydactyly was the next (16.2%). Fifty eight (10.6%) patients had anomalies in the hand as well as in the other site. Among these, camptodactyly was the most commonly found (36.5%) and polydactyly was the next common variety (19.0%) in the hand. In the anomalies which occurred in other than hand, arthrogryposis multiplex congenita was the most common (30.0%). Syndactyly of the foot (22.9%) and polydactyly of the foot (18.6%) were the next common anomalies.
Arthrogryposis ; Brachydactyly ; Child ; Classification ; Female ; Foot ; Hand* ; Humans ; Incidence* ; Korea ; Male ; Polydactyly ; Seoul ; Syndactyly ; Trigger Finger Disorder

No abstract available.
Testis*

No abstract available.
Abortion, Spontaneous* ; Chorion* ; Chorionic Villi* ; Female ; Humans ; In Situ Hybridization ; Pregnancy

Pleomorphic (undifferentated) carcinoma is a rare histologic type of carcinomas of the gallbladder and an atypical carcinoid is thought to be an intermediated type between carcinoid tumor and small cell carcinoma. Dense core "neurosecretory" granules can be found in the above mentioned tumors. We experienced a case of a double tumor of the gallbladder in a 51-year old male patient. Grossly, a large solid mass, about 5.0 cm in diameter, was found in the fundic portion and the neck portion also had a small 1.5 cm-sized polypoid mass. Microscopically, these lesions had features of pleomorphic carcinoma and atypical carcinoid, respectively. Immunohistochemically, they manifested reactivity for neuron specific enolase. Ultrastructural study revealed neurosecretory granules in the cytoplasms of tumor cells of the fundic and neck masses. Although light microscopic features of these tumor masses are quite different, we consider that these tumors represent a spectrum of neuroendocrine differentiation.
Male ; Humans

No abstract available.
Insulin* ; Proteolysis*

No abstract available.
Hypopharyngeal Neoplasms*

The homeostasis for a number of cellular proteins is regulated by not only phosphorylation and dephosphorylation, but also ubiquitination and deubiquitination. A number of proteins involved in the degradation of polypeptides have been isolated in various eukaryotic organisms from Saccharomyces cerevisiae to human. Recently, several deubiquitinating enzymes, classified into either the Ub C-terminal hydrolase (UCH) or the Ub-specific processing protease (UBP), have been reported. It has been shown that they contain conserved domains including Cys, His, and Asp residues throughout the enzyme. These proteins have been demonstrated that Cys and His domains are critical for deubiquitinating enzymatic activity. Recently, we have shown that the Asp domain localized between Cys and His domains is also essential for cleaving the ubiquitin from protein substrates. Mouse deubiquitinating enzymes including DUB-1, DUB-2, and DUB-2A have been isolated and they showed the expression specificity. Of these, DUB- 1 and DUB-2 are expressed in lymphocytes depending on the presence of cytokines (interleukin-3 in B-lymphocytes and interleukin-2 in T- lymphocytes, respectively), indicating that they are involved in cytokine signaling pathways. Isolation of all putative DUBs will help to identify their substrates and to regulate the homeostasis of cellular proteins, especially in proliferative cells.
Amino Acid Sequence ; Animals ; Conserved Sequence ; Cytokines/metabolism ; Enzyme Activation ; Forecasting ; Human ; Lymphocytes/enzymology/*metabolism ; Models, Biological ; Molecular Sequence Data ; Protein Processing, Post-Translational ; Protein Structure, Tertiary ; Proteins/chemistry/isolation & purification/*metabolism ; Recombinant Fusion Proteins/metabolism ; Signal Transduction ; Ubiquitin/*metabolism