Primary systemic amyloidosis

Opinion statement Primary amyloidosis is a plasma cell dyscrasia in which insoluble immunoglobulin light chain fragments are produced and polymerize into fibrils that deposit extracellularly, causing visceral organ dysfunction and death. The disorder is rare. Its recognition requires understanding the association between nephrotic syndrome, cardiomyopathy, peripheral neuropathy, and hepatomegaly with amyloidosis. The most important screening test for amyloidosis is immunofixation of the serum and urine to detect a monoclonal immunoglobulin light chain. All patients need the diagnosis confirmed histologically. The least invasive source of tissue for amyloid detection is the subcutaneous fat. The most important prognostic factor is whether there is cardiac involvement, which is best assessed by echocardiography with Doppler studies. Therapies used include oral melphalan/prednisone and high-dose corticosteroids. High-dose chemotherapy followed by stem cell reconstitution seems to provide the highest reported response rates. Transplant is associated with unique morbidities not seen in the transplantation of patients with other hematologic malignancies.     

Levodopa dependency in Parkinson's disease: case report and review

Only a few patients with Parkinson's disease and levodopa dependency or abuse have been reported. We present a 35-year-old patient with young-onset Parkinson's disease who developed motor complications, levodopa dependency, and drug-induced psychosis after primary treatment with levodopa. Diagnostic criteria and treatment guidelines for this neuropsychiatric disorder are presented as well as a tentative neurobiological answer to the question of why levodopa dependency is observed only in a minority of patients with Parkinson's disease.     

Extracts of Monascusus Purpureus beyond Statins——Profile of Efficacy and Safety of the Use of Extracts of Monascus Purpureus

Monascus purpureus is a fungus that has been tradi-tionally used in China for thousands of years to produce ricewine . Traditionally the fungus is used to ferment non-gluti-nous cooking of rice ,to produce a kind of famous red ricewine .Its main traditional indications consist of disordersdue to the accumulation of fats (nosologically linked withthe problems of stasis of circulation in traditional Chinesemedicine) ,liver disorders and some forms of tumor as ithelps i mprove the circulation of …     

Standards for total body fat and fat-free mass in infants

Data on body composition in conjunction with reference centiles are helpful in identifying the severity of growth and nutritional disorders in infancy and for evaluating the adequacy of treatment given during this important period of rapid growth. Total body fat (TBF) and fat-free mass (FFM) were estimated from total body electrical conductivity (TBEC) measurements in 423 healthy term Caucasian infants, aged 14-379 days. Cross sectional age, weight, and length related centile standards are presented for TBF and FFM. Centiles were calculated using Altman's method, based on polynomial regression and modelling of the residual variation. The TBF percentage steeply increased during the first half year of life, and slowly declined beyond this age. Various simple TBEC derived anthropometric prediction equations for TBF and FFM are available to be used in conjunction with these standards. Regression equations for the P50 and the residual SD, depending on age, weight, or length, are provided for constructing centile charts and calculating standard deviation scores.     

Phase II study of etoposide (VP-16) in patients with thyroid cancer with no prior chemotherapy: an Eastern Cooperative Oncology Group Study (E1385)

This study of etoposide in thyroid cancer was designed to determine the activity and toxicity of etoposide in a variety of inoperable, thyroid hormone insensitive, and radio-iodine resistant primary cancers of the thyroid. The patients were required to have an ECOG performance status of at least 3 and no previous exposure to chemotherapy. The etoposide was given at a dose of 140 mg/m² daily for 3 days and every 3 weeks until progression. The study was closed after 18 months because of poor accrual. There were no responses seen among the 10 patients accrued. The toxicity was primarily hematologic. There was no evidence of activity of etoposide in thyroid carcinoma, although this study lacked significant power because of the poor accrual.     

Accuracy at radiography and probing for the diagnosis of proximal caries

In 63 teenagers the proximal surfaces of premolars to be extracted for orthodontic reasons and the adjacent surfaces of neighboring teeth, 598 surfaces in all, were examined radiographically and by probing. Direct inspection after extraction revealed that 51% of the surfaces had incipient carious lesions and 5% lesions with cavities. Of the surfaces with cavities 82.1% were correctly diagnosed radiographically if any radiolucency, regardless of its extent, was used as a diagnostic criterion. However, this yielded a false positive rate of 19.6%. If only radioluccncies extending at least into the inner third of the enamel were called positive the true positive rate was 36.7% and the associated false positive rate 1.6%. At a 5% cavity frequency the predictive positive values were 17% and 53%, respectively. Probing yielded a true positive rate of about 29% and a false positive rate of 1.1% with a predictive positive value of 50-57%. All radiolucencies extending into the dentin were associated with cavities. When the most liberal radiographic criterion was used 37.5% of all carious lesions, whether associated with a cavity or not, were detected and 3.8% of sound surfaces were falsely called carious. The corresponding predictive positive value at the actual rate of incipient lesions and lesions with cavities was 92.6%.