A review is presented of the clinical and morphological manifestations of lymphangioleiomyomatosis (LAM), a systemic disorder of unknown etiology that affects women. The clinical features include dyspnea, hemoptysis, recurrent pneumothorax, chylothorax, and chylous ascites. It is characterized by: 1) proliferation of abnormal smooth muscle cells (LAM cells) in pulmonary interstitium and along the axial lymphatics of the thorax and abdomen; 2) thin-walled pulmonary cysts, and 3) a high incidence of angiomyolipomas. The pulmonary cystic lesions have a characteristic appearance on high resolution computed tomography. The most specific method for diagnosing LAM is lung biopsy to demonstrate the presence of LAM cells, either by their characteristic histological appearance or by specific immunostaining with HMB-45 antibody. LAM cells differ in several important respects from the types of smooth muscle cells normally present in lung. Their reactivity with HMB-45 antibody is localized in stage I and stage II melanosomes. LAM cells show additional evidence of incomplete melanogenesis, and the significance of these observations remains to be determined. Two types of LAM cells are recognized: 1) small, spindle-shaped cells that are centrally located in the LAM nodules and are highly immunoreactive for matrix metalloproteinase-2 (MMP-2), its activating enzyme (MT-1-MMP), and proliferating cell nuclear antigen (PCNA), and 2) large, epithelioid cells that are distributed along the periphery of the nodules and show a high degree of immunoreactivity with HMB-45 antibody and with antibodies against estrogen and progesterone receptors. Types of treatment used for LAM include oophorectomy, administration of Lupron or progesterone and in very severe cases, pulmonary transplantation (following the onset of respiratory insufficiency, not relieved by O2).
Objective: Our aim was to obtain new information about the relationship between infant responses to surfactant replacement therapy and histopathological changes in vital organs. Study design: To accomplish this, the autopsy findings and clinical backgrounds of 41 very low birth weight infants (gestational week 25.6± 2.3; birth weight 806.4± 251.6g) who had died after receiving surfactant replacement therapy were reviewed, and those who responded to therapy were compared with those who did not. Responders were infants in whom the required FiO2 declined by > 20% or mean airway pressure declined by > 20% within six hours of instilling surfactant (n=18) ; non-responders were infants who did not meet those criteria (n=23).
Result: Gestational age, birth weight and time at treatment were similar in responders and non-responders, but survival was significantly longer in responders. The incidences of hyaline membrane disease, pulmonary interstitial emphysema, hemorrhagic necrosis and parenchymal degeneration of the liver and kidney were all higher in non-responders, whereas the incidences of bronchopulmonary dysplasia and pneumonia were higher in responders. Prior to treatment, acidosis and hypothermia were significantly more severe in non-responders, and perinatal complications, such as fetal distress and intrauterine infection, were observed more often in non-responders. Substantial degradation of vital organs had already occurred during the early post-natal or intrauterine life of the non-responders, which would be expected to interfere with the clinical response to instilled surfactant.
Conclusion: It is anticipated that in the future improved monitoring of immature fetuses will be indispensable to improve intrauterine fetal management and to achieve better control over the timing and mode of delivery.
Background: One of the factors that affect the recurrence rate after peripheral directional atherectomy (DA) is the degree of residual stenosis. A new method of peripheral DA to reduce residual stenoses was evaluated with a rotational digital angiography (RDA) system that provides both angiography and fluoroscopy at multiple projections within 360 degrees.
Patients and methods: Between March 1995 and July 1999, severe short segmental stenoses of six iliac arteries and two superficial femoral arteries (SFA) in eight patients were treated with the Simpson DA catheter under RDA system guidance. After pre-procedural RDA evaluation, the first series of DA were performed under ordinary PA fluoroscopic guidance. The residual stenoses were evaluated with RDA. If the residual stenoses exceeded 30%, a second series of DA were performed covering the residual plaque with the cutter window of the DA catheter. To this purpose the fluoroscopy of the RDA system was fixed in the direction in which the residual stenoses were largest and most eccentric. The end point was defined to be a residual stenosis of less than 30% evaluated with the RDA system, and the procedures were repeated until the end point was achieved.
Results: Five of six iliac artery lesions were curved at the pre-procedural RDA evaluation. After the first series of DA, only two of six iliac lesions but all SFA lesions achieved the end point. Among the four other iliac lesions, three achieved the end point with one or two additional series of DA using the RDA system guidance to control the selective cuts of the residual plaques. One patient had a residual stenosis of 50% because the procedure could not be completed by balloon rupture of the DA catheter. In the patients with iliac stenoses, there was no final residual stenosis in one, and the range was from 20% to 25% in the four patients. The residual stenoses were located on the greater curvature side of the curved artery in three of these four patients.
Conclusion: The RDA system is a valuable tool in aiding reduction of the residual stenoses during peripheral DA. Minimal stenoses often remain on the greater curvature side of the wall because the rigid and straight metallic capsule (cylindrical housing) of the Simpson DA catheter does not completely fit the curved wall. This phenomenon was thought to be a mechanical limitation of this device.
Although endothelin-1 (ET-1) is involved in balloon-induced neointima formation, the role of ET-1 in balloon-induced neointima formation in hypercholesterolemia is unclear. In addition, it remains to be determined whether ET-1 is produced by endothelial cells or vascular smooth muscle cells, or both. We investigated tissue immunoreactive ET-1 levels by immunoblot analysis, localization of ET-1 immunoreactivity by immunohistochemistry, and expression of preproET-1 mRNA by in situ hybridization in balloon-induced neointima formation in experimental hypercholesterolemic rats. Serum total cholesterol levels were significantly higher (p<0.01) in the 5%cholesterol-diet group (194±17 mg/dl, n=20) than in the normal-diet group (64±2 mg/dl, n=20). Before and after endothelial denudation, plasma ET-1 levels and tissue immunoreactive ET-1 levels were significantly higher in cholesterol-diet rats. The expression of preproET-1 mRNA by in situ hybridization was observed in the nuclei of endothelial cells, but not medial smooth muscle cells in normal- or cholesterol diet rats. After endothelial denudation, plasma ET-1 levels and serum total cholesterol levels did not change in either the normal- or the cholesterol-diet rats. Tissue level of ET-1 tended to increase at 3 days after denudation in normal-diet rats (1.0± 0.1 vs 2.6± 0.2 density ratio, p< 0.05), although endothelial cells had not yet regenerated. The expression of preproET-1 mRNA by in situ hybridization was not observed at 3 days after endothelial denudation in either endothelial or medial smooth muscle cells in normal-diet rats. Four weeks after denudation, regeneration of endothelial cells was almost complete, and an intimal hyperplasia was observed. Tissue ET-1 levels were significantly elevated 4 weeks after endothelial denudation in normal-diet rats (1.0±0.1 vs 7.6±0.2 density ratio, p<0.05). The expression of preproET-1 mRNA by in situ hybridization was observed in the nuclei of regenerated endothelial cells after endothelial denudation, and in smooth muscle cells migrating into the intima, but was not observed in medial smooth muscle cells in normal-diet rats. A similar pattern was observed in cholesterol-diet rats. We concluded that ET-1 was involved in neointima formation and that ET-1 was produced by both endothelial and neointimal smooth muscle cells, but not medial smooth muscle cells after endothelial denudation in experimental hypercholesterolemic rats.
Objective: Lower uterine segment thickness was measured by transvaginal ultrasound examination and its correlations with the occurrence of uterine dehiscence and rupture was examined.
Methods: The thickness of the muscular layer of the lower uterine segment was measured in 186 term gravidas with previous uterine scars and its correlation with uterine dehiscence/rupture was investigated.
Results: Uterine dehiscence was found in 9 cases or 4.7%. There were no cases of the uterine rupture. The thickness of the lower uterine segment among the gravidas with dehiscence was significantly less in than those without dehiscence (p< 0.01). The cut-off value for the thickness of the lower uterine segment was 1.6 mm as calculated by the receiver operating characteristic curve. The sensitivity was 77.8%; specificity 88.6%; positive predictive value 25.9%; negative predictive value 98.7%.
Conclusion: Measurement of the lower uterine segment is useful in predicting the absence of dehiscence among gravidas with previous cesarean section. If the thickness of the lower uterine segment is more than 1.6 mm, the possibility of dehiscence during the subsequent trials of labor is very small.
Mercury spilled from a mercurial sphygmomanometer on a hot carpet can vaporize and pollute the environment. We observed the vaporization of mercury in model experiments. Mercury (0.15g) was heated on a hot carpet and the near-by air was sampled with a midget impinger. The evaporated mercury levels were 5.0, 6.3, 8.1 and 10.0mg/m3 at 20, 40, 60 and 80 minutes, respectively at a height of 30cm from carpet. The result indicated that even if a small quantity of mercury remained on the hot carpet, it could evaporate and pollute the indoor air. Little is known about the influence on human health of low mercury exposure, especially on children. In order not to pollute the air, we need to pay attention to mercury.
We removed a biliary stone and the metallic stent placed two years previously in a patient with benign biliary strictures.
An 80-year-old woman who had been inplanted with an expandable metallic stent (EMS) to prevent obstruction by a large common bile duct stone about two years before as an emergency measure in another hospital, was afficted with acute occlusive pyogenic cholangitis (AOPC) and hospitalized in our hospital. After treating the AOPC, we successfully removed the EMS with a cholangioscope and normal biopsy forceps through the percutaneous transhepatic channel under fluoroscopy.
The type of the EMS was Accufulex® stent. To remove it was easier than expected. Once it started to unravel, it was removed from the common bile duct within a few minutes.
We present two cases of a very rare tumor, intracranial lipoma, diagnosed by computed tomography (CT) and magnetic resonance imaging (MRI). In one case, the lipoma was in the superior cerebellar cistern, the other was in the periphery of the corpus callosum. In the case in which MRI was used, identification of the lipoma using a routine MRI examination was difficult. These cases are reported now because the incidental diagnosis of intracranial lipoma is likely to increase due to advanced neuroradiological techniques such as CT and MRI.