Familial spontaneous pneumothorax in three generations and its HLA

We experienced a case of familial spontaneous pneumothorax in three generations. Six of 13 family members had episodes of spontaneous pneumothorax. It is well established that there are some diseases associated with human leukocyte antigen (HLA). We performed HLA phenotyping for HLA of A, B and C. In our study, we detected the HLA haplotype A2, B61 in three of 4 who had episodes of spontaneous pneumothorax. The HLA haplotype A2, B70 were also detected in three of 4 who had episodes. This suggests that familial spontaneous pneumothorax might have hereditary factors.     

Repeat operations in the management of clival chordomas: palliative surgery.

Some chordomas have a very poor prognosis because of their aggressive growth nature, but the efficacy of repeat operations for these cases has not been well documented. This report concerns 3 patients with aggressive chordoma of the clivus, who underwent operations 6 to 12 times over a period of 8 to 17 years because of symptomatic regrowth. Overall mean interval between repeat operations was 18 months with a range from 5 to 57 months and survival times were 9 to19 years after the first surgery. Main symptoms before each operation were diplopia and visual disturbance. Repeat palliative operations by intentional extradural debulking of the tumour to decompress offending neural structures, as well as maximal removal of the tumour, using appropriate skull base approaches, can mitigate progressive symptoms, and may result in better quality and some prolongation of life, although our patients gradually deteriorated neurologically throughout the clinical course.     

Airway troubles related to the double-lumen endobronchial tube in thoracic surgery

Purpose Several case reports indicate critical respiratory complications in relation to the double-lumen endobronchial tube (DLT). A prospective survey for the airway problems in using the DLT is presented. Methods One hundred adult patients undergoing thoracotomy for lung cancer were investigated. Tube malposition and airway obstruction were searched using a fiber-optic scope. The endobronchial cuff was positioned just below the trachcal carina while the trachea was intubated with a DLT (Rüsch). The distances of displacement, from the tracheal carina to the bronchial cuff, were measured during anesthesia using an epidural catheter, which had marks every 5 mm. The distances for correcting the tube position were measured at both the bronchial cuff and the level of the teethPaO₂,PaCO₂ andSPO₂ were also measured. Results Malposition (displacement over 5 mm from the correct position) was found in 42 patients, and 40 of them were in a withdrawal direction, occurring at the postural change and during one-lung ventilation, especially during manipulation of the lung hilum. Correcting distances at the level of the teeth were 15.3–3-times longer than those at the bronchial cuff. Airway deformities and gradual withdrawal of the bronchial cuff were found in association with surgical manipulation. Obstruction occurred at the tips of the tracheal tube in four patients and the bronchial tube in six patients, and at the tip of both in two patients. Hypoxemia (PaO₂<60 mmHg) occurred in four patients and hypercapnea (PaCO₂>60 mm Hg) in two patients. Conclusion Most of the DLT obstructions were associated with withdrawal malposition. Great attention to DLT displacement and airway deformity is advised.     

Testicular seminoma with human chorionic gonadotropin production

Testicular seminoma with elevated serum human chorionic gonadotropin level (hCG-positive seminoma) is regarded as more malignant than marker-negative seminoma, although Its prognosis is still unclear. To clarify the malignant potential of seminoma with hCG production, the serum levels of the beta subunit of hCG (β-hCG) and lactic acid dehydrogenase (LDH) were examined in 35 and 40 patients, respectively, and the Immunohistochemical expression of β-hCG examined in 45 tumors. The elevation of the LDH serum level correlated to the Invasive status, metestatic status and poor outcome, while that of the serum β-hCG level correlated only to the metastatic status. Immunohistochemical expression of β-hCG was observed in syncytiotrophoblastic giant cells in 11 tumors and a few mononuclear seminoma cells In 36 tumors. Expression was not associated with the malignancy potential, except where the expression In mononuclear cells Inversely correlated to the invasive status. These results suggest that most seminomas produce a slight amount of hCG; that an elevated hCG serum level Indicates the pressnce of metastatic tumors and mainly reflects an increase in tumor volume but not in cellular malignancy potential; and that the LDH serum level, rather than hCG, is more useful as a prognostic indicator for patients with seminoma.     

A case of lymphocytic adenohypophysitis associated with sarcoidosis of the lung and eye

Lymphocytic adenohypophysitis is considered to be an inflammatory disease of the adenohypophysis that is commonly present with visual disturbance and hypopituitarism. Its etiology remains unclear but it is often related to an autoimmune disorder involving other organs, such as the thyroid, parathyroid, or adrenal glands. We encountered a rare case of lymphocytic adenohypophysitis associated with sarcoidosis of the lung and eye during the follow-up period. A 23-year-old woman was hospitalized in July 1986, with a one-month history of headache and visual disturbances which began three days after her second normal delivery. On admission, she showed slight visual impairment and had a left temporal superior quadrantanopia. Endocrinological evaluation revealed thyroid and adrenal hypofunction, and low response of human growth hormone to the loading test. A skull X-ray showed normal shaped sella with some erosion of the dorsum. CT scan showed a rounded contrast-enhanced intrasellar mass extending into the suprasellar cistern. MRI (SR: 500/30) showed a homogeneous low intensity mass which contained a small high intensity area on the relative T2-weighted image (2000/50). A biopsy was performed via right frontotemporal craniotomy. The consistency of the resected tissue was firmer than that of pituitary adenoma. Histologically, the tissue showed diffuse lymphocytic infiltration with some normal adenohypophysis. Her postoperative course was uneventful and the visual impairment improved two months later after the operation. Six months after the operation, she was readmitted with complaints of general fatigue and breathlessness. Chest X-ray showed diffuse infiltration throughout both lung fields, but there was no bilateral hilar lymphadenopathy.(ABSTRACT TRUNCATED AT 250 WORDS)     

Accommodative intraocular lens with spring action. Part 1. Design and placement in an excised animal eye

We designed an intraocular lens (IOL) with spring action, which we call the Spring IOL, with the idea of providing a slight but adequate accommodative power. The Spring IOL consists of two 6.0-mm optics, held 4.38 mm apart, and four flexible loops. The optics are made of polymethylmethacrylate (PMMA) and the loops of polyvinylidene fluoride. The lens's refractive power is provided by the anterior optic; the posterior optic acts as a footplate to restrict the anteroposterior movement of the anterior optic. By torsionally compressing the obliquely arranged loops, a 10.0-mm 3-dimensional IOL can be reduced to 6.0 mm and inserted in the capsular bag through a 3.5-mm capsular opening. Because the Spring IOL is made of the same materials as conventional IOLs, most of the problems hindering the development of a refilling material (which could also provide accommodation) are avoided. In this first report we discuss the design of the lens, the refractive theory involved, and our experience inserting it in an excised animal eye.     

Surgical treatment of syphilitic ascending aortic aneurysm: a case report

A 67-year-old man with positive serum reaction for syphilis had been followed by cardiologist for his moderate-sized saccular ascending aortic aneurysm and small-sized abdominal aortic aneurysm. Because of his transient ischemic attack probably secondary to the thrombo-embolism of the aneurysm and rapid growing of its size, surgical treatment was recommended. Resection of the saccular aneurysm with patch plasty of the ascending aorta was performed under the cardiopulmonary bypass associated with right side cerebral perfusion. At the time of operation, the mildly dilated ascending aorta and arch with multiple intimal ulceration were noted. Although his postoperative hemodynamic condition was stable, he suffered from multiple cerebral infarction, probably due to embolism migrated from the fragile aortic intima. His neurological condition was improved promptly, trivial hemi-paralysis was remained. The specimen of resected aneurysmal wall revealed syphilitic changes microscopically. We concluded that the extent of the aortic replacement with prosthetic graft should be deceived not only with its external appearance, but also with the changes of its inside.     

Effects of Pyridoxalated Hemoglobin Polyoxyethylene Conjugate and Stroma-free Hemoglobin on Renal Vascular Responsiveness to Vasoactive Substances in Isolated Perfused Rat Kidney

Abstract: The effects of pyridoxalated hemoglobin polyoxyethylene conjugate (PHP) and stroma-free hemoglobin (SFH) on vascular responsiveness to various vasoactive substances were examined in isolated perfused rat kidneys. The kidneys isolated from rats were perfused with 6% PHP, 6% SFH, and 6% hydroxyethylstarch (HES) solution at a constant flow rate. Vascular responsiveness to acetylcholine (ACh), nitroglycerin (NG), norepinephrine (NE), and angiotensin-II (ANG-II) was examined by measuring the perfusion pressure (PP). Effects of inhibition of endothelium-derived relaxing factor (EDRF) by N^G-monomethyl L-arginine (L-NMMA) on NE-induced and ANG-II-induced renal vascular responses were examined. ACh and NG induced a dose-dependent decrease in perfusion pressure (PP) in all groups. NE and ANG-II induced an increase in PP in all groups, but NE-induced and ANG-II-induced responses in the PHP-perfused and SFH-perfused groups were significantly larger than those in the HES-perfused group. L-NMMA did not alter vascular responsiveness to NE and ANG-II. These results indicate that PHP and SFH do not inhibit EDRF induced by ACh, but hemoglobin moiety per se does augment the vascular responsiveness to NE and ANG-II in the isolated perfused rat kidney.