Statistik

Ohne Zusammenfassung     

Use of sirolimus in the treatment of lymphangioleiomyomatosis: favorable responses in patients with different extrapulmonary manifestations

OBJECTIVE:

Lymphangioleiomyomatosis (LAM) is a rare disease that is currently considered a low-grade neoplasm with metastatic potential and variable progression. Mammalian target of rapamycin (mTOR) inhibitors, such as sirolimus and everolimus, have recently become a treatment option for LAM patients, especially those with extrapulmonary manifestations. The objective of the present study was to describe a case series of four patients with LAM in Brazil who showed significant improvement, particularly in their extrapulmonary manifestations, after treatment with sirolimus (at 1-4 mg/day).

METHODS:

We describe four cases of LAM patients with different extrapulmonary manifestations who were treated with sirolimus.

RESULTS:

After treatment with sirolimus for 12 months, one patient presented resolution of severe chylothorax; one had a significant reduction in renal angiomyolipoma volume; and one showed significant regression of retroperitoneal lymphangioleiomyomas and abdominal lymph node enlargement. After treatment with sirolimus for 6 months, the remaining patient had a significant reduction in the volume of a massive retroperitoneal lymphangioleiomyoma.

CONCLUSIONS:

Our findings confirm that mTOR inhibitors are beneficial for patients with LAM, especially those with extrapulmonary manifestations, such as renal angiomyolipoma, lymphangioleiomyomas, and chylous effusions. However, certain aspects, such as the optimal dose, duration of treatment, and long-term adverse effects, have yet to be sufficiently clarified for mTOR inhibitors to be incorporated into LAM management protocols.     

Reizwirkungen

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Biologie

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Accuracy of psychiatric diagnosis performed under indirect supervision

This work aimed at comparing the accuracy of the psychiatric diagnoses made under indirect supervision to the diagnoses obtained through Structured Clinical Interview for DSM-III-R (SCID). The study was conducted in 3 university services (outpatient, inpatient and emergency). Data from the emergency service were collected 3 years later, after changes in the training process of the medical staff in psychiatric diagnosis. The sensitivity for Major Depression (outpatient 10.0%; inpatients 60.0%, emergency 90.0%) and Schizophrenia (44.4%; 55.0%; 80.0%) improved over time. The reliability was poor in the outpatient service (Kw = 0.18), and at admission to the inpatient service (Kw = 0.38). The diagnosis elaborated in the discharge of the inpatient service (Kw = 0.55) and in the emergency service (Kw = 0.63) was good. Systematic training of supervisors and residents in operational diagnostic criteria increased the accuracy of psychiatric diagnoses elaborated under indirect supervision, although excellent reliability was not achieved.     

Clinical-experimental study on the temperature behaviour of the mucosa of mouth related to the season of the year

A long-term investigation of 20 juvenile patients indicated a great seasonal and interdiurnal variation of the gingival temperature, which had a trend to a high interindividual correlation. The absolute individual differences was upto 2 degrees C. The lower jaw had a higher temperature than the upper jaw. The temperature of the right and left sides of the jaw differed from each other. The calculation of a standard temperature is only useful, when it is related to the individual sublingual temperature.     

Metastasen

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