Subclavian artery pseudoaneurysm in type IV Ehlers-Danlos syndrome

We report case of a subclavian artery pseudoaneurysm in a patient with type IV Ehlers-Danlos Syndrome. A 16-year-old boy underwent successful repair of a subclavian artery pseudoaneurysm that occurred after a cervical hyperextension injury. Subsequent workup included skin biopsy and fibroblast culture, which were consistent with a diagnosis of type IV Ehlers-Danlos Syndrome. This condition is a dominantly inherited connective tissue disorder, which in this patient was found to be caused by a spontaneous point mutation in the COL3A1 gene that encodes the chains of type III procollagen. The clinical, genetic, and molecular characteristics of type IV Ehlers-Danlos Syndrome are briefly reviewed.     

Implementing nurse sensitive outcomes into care planning at a long-term care facility

This article describes one long-term care facility's efforts to implement standardized language in the care planning process. Federal regulations for long-term care mandate the use of a uniform comprehensive assessment tool. Eighteen Resident Assessment Protocols (RAPs) are identified for data collection. Computer databases were revised for care planning. Appropriate North American Nursing Diagnosis Association (NANDA) diagnoses were linked to each RAP. Nursing-Sensitive Outcomes (NOCs) were linked to each NANDA as goals. Nursing Interventions Classifications (NICs) were linked to NANDA diagnosis and NOC outcomes as approaches. The databases are illustrated, and frequently used NANDAs and NOCs are identified.     

A family of human receptors structurally related to Drosophila Toll

Interhelical electrostatic interactions at specific heptad positions can regulate dimerization specificity of alpha-helical coiled-coils. We have analyzed 20 vertebrate myosin sequences from a variety of organisms and tissues in order to determine if interhelical ionic interactions correlate with the observed myosin dimerization specificity. We find that the sites for potential interhelical ion pairing are identical in virtually all sarcomeric myosins whether they form homo- or heterodimers. We also show that smooth muscle and non-muscle myosin rod sequences exhibit a different conserved pattern of potential interhelical ion pairing. These observations suggest that myosin rod residues involved in interhelical electrostatic interactions do not regulate dimerization specificity, but may contribute to the specific arrangements of myosin molecules that determine differences in the filament morphology of sarcomeric and non-sarcomeric muscles.     

Expenditure and loss of income incurred by tuberculosis patients before reaching effective treatment in Bangladesh

Fistulae arising from the intestinal tract are associated with significant morbidity and mortality rates. Most contemporary studies of fistulae report mortality rates between 6 and 20 per cent. The major causes of death in these patients are sepsis, electrolyte imbalance, and malnutrition. A total of 48 patients with either external or internal intestinal fistulae were reviewed in this study over a 5-year period at the Louisiana State University Medical Center at Shreveport. Intestinal fistulae were classified into three types, anatomic site, physiologic type, and etiology, to evaluate morbidity and mortality rates. We also attempted to evaluate the role of parenteral nutrition in this patient population, but our data were inconclusive because of the limited number of patients. There was no difference in mortality rates associated with anatomical sites. High-output fistulae were associated with a higher mortality rate compared to low-output fistulae. Fifty-six per cent of the patients achieved closure. The overall mortality rate was 21 per cent. Spontaneous closure rates were lower when compared to those in other studies. This was attributed to sepsis, malignancy, and history of previous radiation therapy. Management of intestinal fistulae includes control of sepsis, correction of electrolyte disturbances, nutritional support, and operative intervention if necessary.     

Outbreaks of trypanosomosis due to Trypanosoma vivax in cattle in Bolivia

Some new commercial methods for the extraction of viral RNA have been introduced recently. In addition to the study published previously (Verhofstede, C., Reniers, S., Van Wanzeele. F., Plum J., 1996. AIDS 8, 1421-1427), seven different methods (four newly developed and three reference methods) for extraction of HIV-1 RNA from plasma have been evaluated. The RNA preparation method that gave the best results (acceptable reproducibility, highest sensitivity, reasonable price, fast and easy to perform), was the QIAamp Viral RNA kit from QIAgen. The High Pure Viral RNA Kit (Boehringer Mannheim) as well as the non-commercialised extraction kits were also very sensitive. The non-commercial tests seem less suitable for routine use and for the processing of large number of samples. Two methods, RNA Insta-Pure LS (Eurogentec) and PANext RNA extraction kit 1 (NTL, PANsystems GmbH) are not adapted for HIV plasma extraction. The single step methods using glass fibre or silica column are rapid (from 60 to 75 min depending on the number of wash steps) and although the price is high they are cheaper than the Boom extraction methods: High Pure Viral RNA Kit (Boehringer Mannheim) ($3.3/sample), QIAamp Viral RNA Kit (Qiagen) ($3.6/sample), Boom extraction ($5/sample). The Qiagen kit is the only kit that combines sensitivity with reproducibility, it is commercialised, rapid and affordable in price and can be automated. For most of the methods evaluated the inter-test variability was acceptable (mean variation coefficient between duplicate extractions varied between 26.4 and 48.6%).     

NMDA receptor-dependent and metabotropic glutamate receptor-dependent forms of long-term depression coexist in CA1 hippocampal pyramidal cells

The development and maturation of the endolymphatic sac (ES) and duct (ED) were studied in the newt Cynops pyrrhogaster. The ES first appears as an oval capsule at the dorsal-medial tip of the otic vesicle at stage 39, about 11 days after oviposition. The ES consists of polymorphous epithelial cells with a minimum of cytoplasm. The intercellular space (IS) between the epithelial cells is narrow and has a smooth surface. At stage 44, the size of the ES increases as many vacuoles in the IS become filled. At stage 46, 18 days after oviposition, the ES elongates markedly and a slit-like lumen is found in the ES. The epithelium contains a few cell organelles which are scattered in the cytoplasm. The vacuoles in the IS are fused, which expands the IS. Two days later (stage 48), floccular material (endolymph) is present in the expanded lumen. The IS dilates and has a wide and irregular appearance. At stage 50, approximately 26 days after oviposition, the ES extends and expands significantly and crystals (otoconia) can now be seen in the widened lumen of the ES. The cytoplasm of the cuboidal epithelial cells contains an abundance of vesicles surrounded by ribosomes and Golgi complexes. Intercellular digitations are formed in the expanded IS. At stage 54, the ES forms a large bellow-like pouch. Numerous otoconia accumulate in the lumen. Free floating cells and cell debris can be seen in the lumen at this stage. The epithelial cells contain numerous cytoplasmic organelles which are evenly distributed in the cytoplasm. Granules are found in the apical and lateral cytoplasm. The IS is loose and displays a labyrinthine appearance. The primitive ED first appears as a connection between the ES and the saccule but no lumen is present inside at stage 39. At stage 46, a narrow lumen is formed in the ED, which corresponds to the formation of the ES lumen. At stage 50, as the ED extends, floccular material is seen in the lumen. At stage 54, the ED bears numerous microvilli on its luminal surface. Otoconia and endolymph are present in the ED. Tight junctions between the epithelial cells are formed at stage 46. A fully developed intercellular junctional complex is produced at stage 54. Based on the development of the ES and ED, the maturation of function of the ES and ED are discussed.     

Changes in sputum composition during sputum induction in healthy and asthmatic subjects

BACKGROUND: Home oxygen therapy improves survival and quality of life in adults with chronic obstructive airways disease. The few studies about home oxygen therapy in children show improvements in weight gain, school performance and decreases in hospitalization expenses. AIM: To report our experience in home oxygen therapy in children followed for six months to four years. PATIENTS AND METHODS: Fifty five children, less than 15 years old, discharged from a University hospital with the diagnosis of chronic respiratory failure, were followed up at their homes. RESULTS: Discharge diagnoses were bronchopulmonary dysplasia in 36% of children, postinfectious pulmonary damage in 22%, neonatal distress in 13%, chronic aspiration in 9%, cystic fibrosis in 7% and miscellaneous in 13%. Forty six completed at least 6 months of follow up, five moved to other hospitals, three required ventilatory support and one died. Oxygen was discontinued in 33 patients, and this occurred before the ninth month of follow up in 88% of those children. Neonatal distress and bronchopulmonary dysplasia had the best prognoses, and oxygen was discontinued at 4 +/- 1 and 5.7 +/- 3 months respectively. Patients with postinfectious pulmonary disease had a higher incidence of bronchoneumoniae, and those with bronchopulmonary dysplasia a higher incidence of acute bronchiolitis, that motivated hospital admissions. Expenses due to home oxygen were lower than hospitalization costs. No adverse effects were detected. CONCLUSIONS: Infants and newborns on home oxygen therapy have a good prognosis, specially those with reversible diseases. This type of therapy allows an earlier hospital discharge with considerable cost reductions.     

Primary antibody deficiency in Arabs: first report from eastern Saudi Arabia

The purpose of this study was to determine whether a staged, algorithmic evaluation of infantile spasms could be developed to minimize patient discomfort, treatment delay, and overall costs. A retrospective chart review of patients diagnosed with infantile spasms at the authors' institution during a 10-year period was performed, with 29 patients identified; 28 charts were reviewed. By history and physical examination, 21 were classified as symptomatic and seven as cryptogenic. Of the 21 symptomatic patients, 13 had a known etiology at presentation; with further testing the specific etiology was determined in two more. Two in the cryptogenic group were reclassified on the basis of neuroimaging findings. Evaluations included neuroimaging (27, 15 abnormal), cerebrospinal fluid studies (nine, all normal), comprehensive metabolic studies (17, all normal), chromosomal analysis (11, two abnormal), and ophthalmologic evaluation (27, six abnormal). The average cost of the studies per patient was $5,076 at the authors' institution. Etiologic yield was increased by 20% with neuroimaging. The other investigations either confirmed a known etiology or were noncontributory. On the basis of these findings, the authors propose an algorithm for a more focused evaluation of infantile spasms. Using the algorithm, the authors suggest directly proceeding to therapy in patients with specific etiologies determined by history and examination. Further evaluation should start with neuroimaging. Subsequent evaluations should be on the basis of those results. The authors estimate a potential 60-90% reduction in total costs if this algorithm is applied.