We describe the case of a pregnant woman with idiopathic pulmonary arterial hypertension, a responder in right heart catheterization, followed since the first trimester in outpatient consultations, admitted to hospital at 23 weeks gestation. She was treated with inhaled iloprost until delivery (at 34 weeks gestation) and continuous infusion of iloprost throughout the perioperative period and following days. This line of therapy has proved efficacious in previous cases. The authors present echocardiographic images that document acute changes in ventricular synchrony during inhalation of iloprost. 相似文献
Bone scintigraphy plays a major role in the diagnosis of bone metastases. The clinical utility of new biochemical markers
of bone metabolism has recently been investigated in various bone diseases. This study evaluated the role of some bone metabolism
markers in comparison with bone scan in the follow-up of breast cancer patients. We studied 149 patients with breast cancer,
33 (22%) of whom had bone metastases. IRMAs were used for the evaluation of blood levels of osteocalcin, bone alkaline phosphatase
(BAP), the C-terminal propeptide of type I procollagen and the C-terminal cross-linked telopeptide of type I collagen (ICTP).
Multivariate regression analysis showed that menopausal status (P=0.007) and metastatic bone lesions (P=0.001) affected bone marker levels. When considering post-menopausal women, the only subset in which bone metabolism marker
behaviour could be reliably investigated, we found a high degree of overlap in marker distribution for scan-positive and scan-negative
patients. Discrimination between scan-negative and scan-positive patients based on the above markers, taken singly or jointly,
was assessed by means of logistic discriminant analysis. The best discrimination was achieved with BAP, closely followed by
ICTP. BAP and ICTP together gave a slight improvement over the use of the two markers separately. However, even in this case
the degree of discrimination was poor and its clinical utility was limited. In fact, to achieve a specificity of 95%, the
sensitivity of the test was about 20%; conversely, with a sensitivity of 95%, the specificity was below 10%. In conclusion,
based on our findings, we believe that blood levels of the investigated markers cannot replace bone scintigraphy in the follow-up
of breast cancer patients for the early detection of bone metastases.
Received 14 April and in revised form 5 July 1997 相似文献
We report a case of cervical dystonia occurring in a 33-year-old without personal history of movement disorder but with family history of essential tremor, primigravid, primiparous woman at 1 weeks' amenorrhea, resolved completely after delivery in the course of 3 months. Dystonia never recurred in the following 5 years. Several neurological disorders are known to occur or worsen during pregnancy. As far as we know, this is the second reported case of dystonia occurring during pregnancy, thus confirming that dystonia gravidarum represents a new entity and should be considered in women of reproductive age affected by dystonia, especially when presenting with rapid-onset cervical dystonia. 相似文献
Status dystonicus (SD) is a life threatening disorder that develops in patients with both primary and secondary dystonia, characterized by acute worsening of symptoms with generalized and severe muscle contractions. To date, no information is available on the best way to treat this disorder. We review the previously described cases of SD and two new cases are reported, one of which occurring in a child with static encephalopathy, and the other one in a patient with pantothenate kinase-associated neurodegeneration. Both patients were admitted to an intensive care unit and treated with midazolam and propofol. This approach proved to be useful in the former while the progressive nature of the dystonia of the second patient required the combination of intrathecal baclofen infusion and bilateral pallidal deep brain stimulation. We believe that a rapid and aggressive approach is justified to avoid the great morbidity and mortality which characterize SD. Our experience, combined with the data available in the literature, might permit to establish the best strategies in managing this rare and severe condition. 相似文献
Preschool children with autism and their normally developing peers were compared on the Stanford-Binet IV and Preschool Language Scale before and after 1 school year. Both measures showed that although the children with autism functioned at a lower level than their normally developing peers, the children with autism had narrowed this gap after treatment, making a nearly 19-point increase in IQ and an 8-point gain in language quotient. The IQ measure remained stable for the normally developing peers while their language showed a 7.73-point increase. The data support the notion that young children with autism can make very significant developmental gains.Special thanks to the parents and the children who participated in this study. Thanks also to Michael Alessandri, Jean Burton, David Celiberti, Nancy Gera-Moglia, Anne Nathan, and Lisa Kamean who assisted in the data collection process. We appreciate the cooperation of Alicia MacWright and Leisa Tomchek who taught two of the classes from which participants were drawn. 相似文献
The micrografting technique, employed to treat patients with extensive TBSA burns and reduced areas of healthy donor skin, was assessed simultaneously with traditional mesh grafts (STSG) or Tiersch grafts in the treatment five severely burned subjects (average TBSA burned 35.6 per cent; average per cent of full thickness wounds 32.6 per cent).
At the first clinical control on day 6 post-surgery 93 per cent of the micrografted area was in situ and healthy: epithelialization of the wound sites was complete at day 21. Meshed STSG yielded 90 per cent attachment at day 6 post-surgery, with epithelialization complete at 12 ± 2 days post-surgery.
Functional results were equivalent no matter what technique was used, while aesthetic results were better in the areas where micrografting was carried out.
Although the micrograft technique is labour-intensive, if the expansion needed is at least 1:6, the aesthetic and functional results obtained are comparable to, or better than, those with meshed grafts. Also, large segments of micrograft are not compromised if a small area of mesh becomes detached, and epithelialization is faster and more uniform, enabling a reduction in both infection and length of hospital stay. 相似文献
An abnormal fibrinogen was identified in a 10-year-old male with a mild bleeding tendency; several years later, the patient developed a thrombotic event. Fibrin polymerization of plasma from the propositus and his mother, as measured by turbidity, was impaired. Plasmin digestion of fibrinogen and thrombin bound to the clot were both normal. The structure of clots from both plasma and purified fibrinogen was characterized by permeability, scanning electron microscopy and rheological measurements. Permeability of patients' clots was abnormal, although some measurements were not reliable because the clots were not mechanically stable. Consistent with these results, the stiffness of patients' clots was decreased approximately two-fold. Electron microscopy revealed that the patients' clots were very heterogeneous in structure. DNA sequencing of the propositus and his mother revealed a new unique point mutation that gives rise to a fibrinogen molecule with a missing amino acid residue at Aalpha-Asn 80. This new mutation, which would disrupt the alpha-helical coiled-coil structure, emphasizes the importance of this part of the molecule for fibrin polymerization and clot structure. This abnormal fibrinogen has been named fibrinogen Caracas VI. 相似文献