The Marfan syndrome is classified as a heritable disorder of connective tissue with clinical and pathological alterations involving supporting elements. In its classic form, the Marfan syndrome is associated with abnormalities of the eye(ectopia lentis), aorta(aortic dilatation and aortic regurgitation) and skeleton(dolichostenomelia, arachnodactyly and pectus deformity). The authers have experienced a case of marfan syndrome recently. The patient was 30-years-old male complaining of anterior chest pain radiating to upper back. Physical examination showed tall status, increased length of the limbs as compared with the trunk and arachnodactyly. On auscultation, heart sound was normal without murmur. He wore glasses because of moderate myopia. Roentgenogram of the chest P-A revealed normal cardiac and aortic contours and lung fields. Left lateral view of chest X-ray revealed concave depression of sternum showing mild pectus excavatum, fusiform dilatation of aorta from aortic root to mid portion of thoracic aorta and resulting in obliteration of retrosternal clear space. Echocardiogram demonstrated moderate dilatation of aortic root A-P diameter(4.5cm) without aortic or mitral valvular abnormalities and normal size of left ventricular cavity(EDD:5.3cm). These data coupled with a distinct family history could permit the diagnosis of Marfan syndrome.
Aorta ; Aorta, Thoracic ; Arachnodactyly ; Chest Pain ; Connective Tissue ; Depression ; Diagnosis ; Dilatation ; Extremities ; Eyeglasses ; Funnel Chest ; Glass ; Heart Auscultation ; Humans ; Lung ; Male ; Marfan Syndrome* ; Myopia ; Physical Examination ; Sternum ; Thorax

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Pregnancy*

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PURPOSE: The purpose of this study was to evaluate and compare the scintigraphic findings and diagnostic accuracy of double-phase Tc-99m sestamibi scan in primary and secondary hyperparathyroidism (HPT). MATERIALS AND METHODS: We retrospectively reviewed 16 cases of primary (18 lesions) and 11 cases of secondary HPT (44 lesions) who underwent Tc-99m-sestamibi scan before the surgical intervention. Scan was performed using LEM camera (Siemens, Germany) after the injection of 740MBq of Tc-99m sestamibi. Routine image consisted of baseline and 3-hour delayed images and each image was obtained using both parallel and pine hole collimator. The study population was 27 patients (male/female=5/22, age: 49.1+/-10.8). RESULTS: Eighteen lesions of primary HPT consisted of 13 adenomas and 5 hyperplasias, while all lesions of secondary HPT were hyperplasias. Among the case of primary HPT, we could detect all the lesions of 13 adenomas but only 2 lesions of 5 hyperplasias (40%) could be detected by double phase scintigraphy. Three cases of primary lesion showed decreased uptake in delayed images compared with baseline. The sensitivity, specificity, positive predictive value and accuracy of primary and secondary HPT were 58.8% (10/17), 83.3% (10/12), 83.3% (10/12), 75.9% (22/29), and 37.5% (15/40), 50% (2/4), 88.2% (15/17), 38.6% (17/44), respectively. Overall sensitivity, specificity, positive predictive value and accuracy were 43.9% (25/57), 75% (12/16), 86.2% (25/29), and 53.4% (39/73). There were no statistical difference between the weight of primary and secondary HPT lesion (p>0.05). CONCLUSION: Tc-99m sestamibi scan is fairly good modality to detect parathyroid lesion in patient with primary HPT before the surgical intervention. However, since some of cases may reveal decreased uptake in delayed image, a careful attention to the findings of baseline images may be helpful. Still the low accuracy of sestamibi scan in diagnosis of secondary HPT prohibits routine use of it for this disease.
Adenoma ; Diagnosis ; Humans ; Hyperparathyroidism, Primary ; Hyperparathyroidism, Secondary* ; Hyperplasia ; Parathyroid Glands ; Radionuclide Imaging* ; Retrospective Studies ; Sensitivity and Specificity

PURPOSE: Total parathyroidectomy and forearm intramuscular autotransplantation (Wells' method) is currently widely accepted procedure for renal hyperparathyroidism because it is far easier to deal with in the case of a recurrence, caused by the autotransplanted tissue. The Wells method, however, is somewhat complex and time-consuming. We developed a simple, easy, and non-surgical injection technique for autografting parathyroid tissue into the forearm subcutaneous layer. This study was performed to evaluate the feasibility of this new method and to document the biochemical function of the parathyroid tissue that was autografted using the subcutaneous injection technique. METHODS: The new method was applied to 14 patients with renal hyperparathyroidism, and the short-term operative results, 4 months after surgery, were evaluated in terms of biochemical parameters such as calcium, inorganic phosphorus, intact PTH and alkaline phosphatase. The graft "uptake" was judged by a significant intact PTH ratio (>1.5) between the antecubital vein blood from the grafted and non-grafted arms. RESULTS: Biochemical parameters were normalized in all patients during the postoperative 4 months. All of the grafted parathyroid tissues were functional. In these patients with the graft "uptake", a significant intact PTH ratio (>1.5) between the grafted and non-grafted arms was demonstrated at 2 weeks post operation. A maximal intact PTH gradient was reached at 4 weeks post operation, and the biochemical function persisted in all patients at 4 months post operation. CONCLUSION: This study suggests that total parathyroidectomy and forearm autotransplantation by subcutaneous injection technique can be an alternative to the Wells' method in the surgical treatment of secondary hyperparathyroidism.
Alkaline Phosphatase ; Arm ; Autografts* ; Calcium ; Forearm ; Humans ; Hyperparathyroidism* ; Hyperparathyroidism, Secondary ; Injections, Subcutaneous* ; Parathyroidectomy* ; Phosphorus ; Recurrence ; Transplantation, Autologous ; Transplants ; Veins

PURPOSE: The purpose of this study is to evaluate the differences of the clinicopathological features and survival rates in gastric cancer according to the sex of the patients. MATERIALS AND METHODS: We reviewed 5,784 cases of gastric cancer patients who underwent laparotomy at the Department of Surgery, Seoul National University Hospital fmm Jan. 1986 to Dec. 1995. We have analyzed clinicopathologic features including tumor location, Bonmann type, depth of invasicm, lymph node metastasis, distant metastasis, TNM stage, histologic differentiation and survival rates according to the sex of the patients. RESULTS: The mean age of female patients was 52.4 years, which is lower than that of male, 54.8 years. There were no differences in tumor location, Borrmann type, depth of invasion, lymph node metastasis, distant metastasis, TNM stage of tumor between male and female. But there were some differences in histologic differentiation; well- and moderately differentiated cancers were more common in male and signet-ring cell cancers were more common in female. Female shows slightly better prognosis than male. But the prognosis of young female was poorer than that of young male. CONCLUSION: In gastric cancer patients, differences in histologic differentiation and more aggressive nature of the signet-ring cell cancer in female may affect the survival differences according to the sex.
Female* ; Humans ; Laparotomy ; Lymph Nodes ; Male ; Neoplasm Metastasis ; Prognosis ; Seoul ; Stomach Neoplasms* ; Survival Rate