Fibrocartilaginous mesenchymoma with low-grade malignancy

In a review of cases of fibrous cartilaginous dysplasia of bone, five of fibrocartilaginous lesions were found to be different in clinical behavior and radiographic and morphologic features from the others. We have named these previously undescribed tumors fibrocartilaginous mesenchymomas with low-grade malignancy in the fibrous elements.Dr. Bertoni is a visiting surgical pathologist from the Servizio di Anatomia ed Istologia Patologica, Bologna, Italy     

Microsurgical versus endoscopic trans-sphenoidal approaches for clivus chordoma: a pooled and meta-analysis

Neurosurgical Review - Chordoma is a rare slow-growing neoplastic bone lesion. However, they show an invasive local growth and high recurrence rate, leading to an overall survival rate of 65% at 5...     

Rett's syndrome: a neurophysiological study

Thirteen girls with Rett's syndrome were evaluated with wakefulness and sleep polygraphic records and EMG studies. The main EEG findings were paroxysmal activity during sleep, multifocal in younger and unifocal in older girls, and "pseudorhythmic flattening". In the opinion of the authors, the changes with age are related to evolution of the disease or maturational changes of CNS. The affectation of the peripheral nervous system was demonstrated in almost half the patients.     

The accompanying male parent in the intensive care unit

A study is made in the Intensive Care Unit of "José L. Miranda" Provincial Pediatric Teaching Hospital in Santa Clara in order to know the sequence with which the father occupies the place of the mother in the care of hospitalized children. With this objective, 54 male companions are surveyed in the first trimester of 1987. The prevailing occupational profile was that of worker. A higher number of the respondents were between 17 and 25 years of age. The school level of secondary school prevailed among patient companions. Most of them reported no economic inconveniences during the child's disease. The desire to remain at the bedside and the inability of the mother to do so were the major reasons for their stay. The advantages recognized by the nursing personnel in male companions are made known.     

Pacific Americans and the National Health Care Act: Where we fit

PURPOSE OF THE PAPER. The purposes of this report were to identify Pacific American health care priorities, recommend how these needs can be addressed in national health care reform legislation, and inform national leaders of the cultural and social context withing which these needs must be met. SUMMARY OF METHODS UTILIZED. In concert with a team of researchers, the authors compiled and analyzed extant literature on the health care of Pacific Americans; discussions were held with health care professionals, state and territorial health care leaders, and agencies and organizations with substantial experience and expertise in health care promotion and development; a two-day health symposium was conducted by Pacific American health care community leaders to review the initial findings, conclusions, and recommendations of the draft report; and follow up meetings were heald with actual communitiy health care providers to validate the data and recommendations of the report. PRINCIPAL FINDINGS. Pacific Americans have collective historical and political relationship not only with the federal government and the continental United States, but also to each other's island state or territory. Thus, common needs and unique interrelationships serve to highlight the importance of the differences between western-oriented and Pacific American health care providers. There is a profound lack of health resources in many Pacific American communities, and even when health resources are potentially available, poor access to health care compounds the need. Pacific Americans are a mobile people with large numbers residing in California, Oregon, Washington, Nevada, Texas, Utah, and the New York-Virginia corridor. Their health needs continue even when they do not reside in their home bases. CONCLUSIONS. Pacific Americans know best how to bridge the cultural gap that continues to exist between them and western-oriented health care providers; improvements in their health care is best accomplished by Pacific Americans themselves. Pacific American health care did not start from an "Even playing field"; hence, the mismatch in resources and needs is so pronounced that it argues not only for continued but also greatly expanded federal support for enabling services, research development, and professional education and development. The federal government must collect and develop better demographic and health access information specifically and separately for Pacific Americans. Baseline data for Pacific American populations, regardless of their residence, must be available and analyzable in terms of community and/or geographic areas. Only then can effective programs be established, managed, evaluated, and improved. KEY WORDS. American Samoans; Chamorrors; Hawaiians; Pacific Americans; Pacific Islanders&semi.     

Early-onset encephalomyopathy associated with tissue-specific mitochondrial DNA depletion: A morphological,biochemical and molecular-genetic study

A male infant, born from consanguineous parents, suffered from birth with a progressive neuromuscular disorder characterized by psychomotor delay, hypotonia, muscle weakness and wasting, deep-tendon areflexia and spastic posture. High levels of lactic acid in blood and cerebrospinal fluid suggested a mitochondrial respiratory chain defect. Muscle biopsy revealed raggedred and cytochromec oxidase-negative fibres, lipid accumulation and dystrophic changes. Multiple defects of respiratory complexes were detected in muscle homogenate, but cultured fibroblasts, myoblasts and myotubes were normal. Southern blot analysis showed markedly reduced levels of mitochondrial DNA (mtDNA) in muscle, while lymphocytes, fibroblasts and muscle precursor cells were normal. Neither depletion of mtDNA nor abnormalities of the respiratory complexes were observed in innervated muscle fibres cultured for as long as 4 months. No mutations were observed in two candidate nuclear genes,mtTFA andmtSSB, retro-transcribed, amplified and sequenced from the proband's mRNA. Sequence analysis of the mtDNA D-loop and of the origin of replication of the mtDNA light strand failed to identify potentially pathogenic mutations of these replicative elements in the proband's muscle mtDNA. Our findings indicate that mtDNA depletion is due to a nuclear encoded gene and suggest that the abnormality underlying defective mtDNA propagation must occur after muscle differentiation in vivo.     

Pregnancies in an 18-Year Follow-up after Biliopancreatic Diversion

Background: 239 pregnancies occurred in 1136 women who had undergone biliopancreatic diversion (BPD). Methods: There were 73 abortions, and 14 pregnancies are presently in their course. The 152 term pregnancies (six twins) occurred in 129 women 2-173 months (mean 42.7) after BPD. Mean age and current excess weight were 31.4 years (20-42) and 29.1% (-6.9-78.2), and mean excess weight loss was 72.9% (30.4-110.5). Results: Mean weight gain during pregnancy was 6.2 kg (-21-25). In 32 patients (21%), parenteral nutritional support was needed. In all the other patients (79%), the usual supplementations were given. Of the newborns, 122 were delivered at term (84.7%) with a mean weight of 2842.4 g (1760-4600 g) and a mean length of 48.5 cm (43-59 cm), while the 22 preterm babies (15.3%) weighed 2151.1 g (1400-3850 g) and had a length of 44.6 cm (33-56 cm). Forty infants (27.8%) were small for gestational age but 17 of them weighed more than 2500 g. Eleven twins (one abortion at 26th week) were also delivered, with a mean weight of 2088.6 g (1200-3100 g) and a mean length of 45.6 cm (35-50 cm). Delivery was spontaneous in 85 instances (56%), while vacuum extractor was used in one, and 66 cesarean sections were performed. There were two birth malformations, one infant died after surgery for meconium obstruction and two died from unknown causes. Of the 129 women, 35 had been infertile before BPD. Conclusions: Disappearance of infertility and decrease of pregnancy risk are to be considered among the beneficial effects of weight reduction following BPD.