The purpose of this study was to evaluate the bone age and hand anomaly in Down syndrome patients. We reviewed radiographs in 52 children and youths with Down syndrome, aged from 2 to 23 years. The characteristic findings of hand anomaly were observed in the middle phalanx of the fifth finger, with the following incidence; brachymesophalangia (44%), clinodactyly (48%), and dysmesophalangia (15%). Pseudoepiphysis (21%) was found typically at the distal end of the first metacarpal bone and proximal end of the second metacarpal bone. Up to 8.5 years of age, bone maturation was retarded and thereafter bone age accelarated in advance of chronologic age. These findings may provide valuable information relative to the diagnosis and treatment of hand anomaly in Down syndrome.
Adolescent ; Child ; Diagnosis ; Down Syndrome* ; Fingers ; Hand* ; Humans ; Incidence

Lymphangioleiomyomatosis(LAM) is a rare disease of women of child-bearing age in which there is progressive hyperplasia of atypical smooth mucle along lymphatics in the lung, and/or axial lymphatics in the thorax and abdomen, resulting in honeycombing of lung. Interestingly there has been a speculation that it represents a forme furste or incomplete expression of tuberous sclerosis complex. This is based on the observation that patients with tuberous sclerosis can manifest pulmonary lesions indistinguishable from LAM. We report a case of LAM occuring in a 39-year-old female, who complained of recurrent pneumothorax, chest pain and shortness of breath. Three years ago, the patient had right nephrectomy under the diagnosis of ruptured angiomyolipoma. A X-ray film of the chest showed honeycombing with a diffusely reticulonodular pattern and cyst-like spaces. She had a characteristic facial appearance of adenoma sebaceum, which her father and uncle had. Microscopically, the lung showed a marked smooth muscle proliferation around the slit-like lymphatic spaces and also some respiratory bronchioles.
Female ; Humans ; Adenoma

Objective: To examine the effects of Tribulus terrestris L. (T. terrestris) extract on the modulation of calcium channels to evaluate its use in topical agents for treatment of atopic dermatitis. Methods: The 70% methanol extract of T. terrestris was prepared. Human HEK293T cells with over-expressed calcium release-activated calcium channel protein 1 (Orai1), transient receptor potential vanilloid 1, or transient receptor potential vanilloid 3 (TRPV3) were treated with T. terrestris extract. Modulation of ion channels was measured using a conventional whole-cell patch-clamp technique. Results: T. terrestris extract (100 mg/mL) significantly inhibited Orai1 activity in Orai1-stromal interaction molecule 1 co-overexpressed HEK293T cells. In addition, T. terrestris extract significantly increased the TRPV3 activity compared with 2-Aminoethyl diphe-nylborinate (100 mmol/L), which induces the full activation of TRPV3. Conclusions: Our results suggest that T. terrestris extract may have a therapeutic po-tential for recovery of abnormal skin barrier pathologies in atopic dermatitis through modulating the activities of calcium ion channels, Orai1 and TRPV3. This is the first study to report the modulatory effect of a medicinal plant on the function of ion channels in skin barrier.

No abstract available.
Syncope* ; Urination*

PURPOSE: To determine whether the compression technique is a valuable additional method for differentiating between benign and malignant breast masses. MATERIALS AND METHODS: The ultrasonographic findings of 95 benign and 53 malignant masses, all pathologically proven, were prospectively analyzed with regard to five diagnostic criteria: shape (regular/irregular), retrotumoral acoustic phenomena (posterior enhancement/posterior attenuation), internal echo pattern (homogeneous/inhomogeneous), compression effect on shape (distortion/no change), and compression effect on internal echo pattern (more homogeneous/no change). RESULTS: The number of cases of benign and malignant masses, respectively, was as follows: regular / irregular shape: 84/11, 9/44; posterior acoustic enhancement/posterior attenuation: 82/13, 16/37; homogeneous/inhomogeneous internal echo pattern: 78/17, 14/39; distortion/no change in shpae: 76/19, 5/48; and more homogeneous/ no change in internal echo pattern: 71/24, 3/50. For all diagnostic criteria for the differentiation of benign and malignant masses, the differences were statistically significant (p<.05). CONCLUSION: Ultrasonography is helpful for differentiating between benign and malignant breast masses. The compression technique is a valuable additional diagnostic method.
Acoustics ; Breast* ; Prospective Studies ; Ultrasonography*

BACKGROUND: The cardiac electrophysiological characteristics differ significantly among mammalian species or among various disease processes. However, difficulties in the procedures for harvesting and isolating tissue have precluded studies using human cardiac specimens. METHODS: The outward K+ -currents were recorded in human atrial myocytes isolated from patients undergoing open heart surgery. The electrophysiological characteristics of the voltage-dependent outward currents were investigated using a whole-cell patchclamp technique. RESULTS: Using depolarizing step pulses, the transient outward currents were activated within 10 msec, which slowly inactivated thereafter. After inactivation, the sustained components of the outward currents remained for up to 5.0 seconds of depolarizing step pulses. While the inactivating component was almost completely inactivated at potentials >+30 mV, the non-inactivating component showed only 10-15% inactivation. The non-inactivating component was highly sensitive to 4-AP and was inhibited by >80% at a concentration of 0.2 mM, while the inactivating component was inhibited by only 25%. The delayed rectifier potassium currents were not recorded. The ratios of the amplitudes of the inactivating and non-inactivating components varied. CONCLUSION: Two components of the voltage dependent outward K+ currents in human cardiac tissue were identified, which could be separated according to their kinetic and pharmacologic properties.
Atrial Appendage ; Humans* ; Muscle Cells* ; Patch-Clamp Techniques ; Potassium ; Potassium Channels ; Thoracic Surgery

BACKGROUND: The proper management of the patients with hypotention remains a difficult clinical problem. This study was designed to evaluate the efficacy and safety of gepefrin in the treament of symptomatic hypotensive patients in outpatient clinic of university hospital. METHODS: Thirty milligrams of Gepefrin was given twice daily for 4 weeks in 30 patients with hypotension(6 males, 24 females ; 23-60 years of age). Blood pressure, heart rate, subjective symptoms and side effects were checked before medication and at the end of 1st, 2nd, 3rd, 4th week of medication. RESULTS: 1) Grpefrin increased sitting systolic blood pressure significantly(p<0.05) by the end of 1st week and thereafter. 2) Hypotensive symptoms were improved in 76% of hypotensive patients and overall rate of usefulness was 70%. 3) Heart rate did not change significantly. 4) One patient complained of loose bowel movement and another one, of anorexia among 30 study patients and all laboratory parameters were within normal limits before and after drug treatment, thus the rate of safety was 93%. CONCLUSION: Results from this study suggests that gepefrin 60mg daily increases the blood pressure mildly and improves the symptoms moderately in symptomatic hypotensive patients.
Ambulatory Care Facilities ; Anorexia ; Blood Pressure ; Female ; Heart Rate ; Humans ; Hypotension* ; Male ; Quality of Life

No abstract available.
Pneumothorax, Artificial* ; Treatment Failure* ; Tuberculosis, Pulmonary*

No abstract available.
3,4-Dihydroxyphenylacetic Acid* ; Animals ; Brain* ; Diazepam* ; Rats*

Most of the malignant tumors of the pancreas are adenocarcinomas arising from the ductal epithelium. Primary leiomyosarcoma of the pancreas, even though it is the most common sarcoma of the pancreas, is very rare. We present a case of leiomyosarcoma of the pancreas, probably primary, with metastases to the stomach, lymph nodes, and abdominal wall. A 52-year-old woman visited the hospital with vague right upper abdominal pain and weigh loss of 6 kg for 2 months. The radiological and endoscopic examination revealed that she had a large heterogeneous mass, 11 cm in size, in the pancreatic body and tail, a 4 cm-sized mass in the paraaortic area, and a 3 cm-sized polypoid mass in the stomach. Histologically, they were all similar to one another and composed of markedly pleomorphic cells. Immunohistochemical and electron microscopic studies showed definite smooth muscle differentiation of the tumor cells. Two months later, the patient underwent an excision of a new 3 cm mass in the right lower abdominal wall, showing features of well differentiated leiomyosarcoma.
Abdominal Pain ; Abdominal Wall ; Adenocarcinoma ; Epithelium ; Female ; Humans ; Leiomyosarcoma* ; Lymph Nodes ; Middle Aged ; Muscle, Smooth ; Neoplasm Metastasis ; Pancreas* ; Sarcoma ; Stomach