Deflazacort for long-term maintenance of remission in type I autoimmune hepatitis.

OBJECTIVE: most patients with autoimmune hepatitis require long-term treatment, but up to 80% of them will develop collateral effects. The aim of this study was to evaluate the efficacy of deflazacort, an oxazolinic derivative of prednisolone with fewer effects on bone and glucose metabolism, in the maintenance of remission of type I autoimmune hepatitis in patients treated previously with conventional immunosuppressive therapy. METHODS: fifteen patients with type I autoimmune hepatitis were included. All patients had been treated previously with prednisone with or without azathioprine until biochemical remission was obtained and the dose could be reduced. Prednisone was then discontinued and deflazacort was started at a dose adjusted to a ratio of 5 mg prednisone per 7.5 mg deflazacort. The biochemical activity (serum ALT and IgG levels) of liver disease was monitored during a follow-up period of 25.8 +/- 7. 7 months. RESULTS: prednisone therapy was followed by a statistically significant decrease in serum ALT (0P: 386 +/- 345 U/L vs 2M 80 +/- 22 U/L, p < 0.02) and IgG (0P 3029 +/- 1934 mg/dL vs 2M 2064 +/- 933 mg/dL, p < 0.05), from the second month of treatment. After changing to deflazacort no alterations in ALT and IgG serum levels were detected except for a mild, transient increase in serum IgG during the first 3 months. During follow-up, 94% of the patients had normal or slightly increased (less than 50% above normal) ALT levels. The titers of ANA and ASMA remained the same in 82% of the patients, decreased in 12%, and increased in the remaining 6%. During follow-up no patient developed arterial hypertension, diabetes mellitus, or changes in visual acuity. Eight patients, all women, complained of dorsolumbar pain which was not related to osteoporosis. CONCLUSIONS: deflazacort seems to be useful in maintaining remission of autoimmune hepatitis during a prolonged period of follow-up. Future studies should include a histological evaluation of the patients and a prospective comparative analysis of side-effects.

Value of ploidy pattern and nuclear texture in the diagnosis of primary hyperparathyroidism.

OBJECTIVE: To determine if there are subvisual differences (quantitation of DNA and study of chromatin texture) that distinguish single-gland parathyroid disease (adenoma) from multiple-gland disease (hyperplasia). STUDY DESIGN: Forty-eight parathyroid glands from 41 patients with primary hyperparathyroidism were studied. Cytometric differences between the chief cells and clear cells were sought. An image cytometer was used to study histologic sections stained with DNA stain reagent. In each case a total of 200 cells were measured in consecutive fields. To distinguish chief cells from clear cells, a sample of 15,600 cells collected in consecutive fields in 78 histologic sections was analyzed. RESULTS: The results indicated that none of the continuous variables differentiated between single-gland and multiple-gland parathyroid disease. The most common ploidy pattern was diploid (25/45), followed by hypodiploid (7/45) and polyploid (5/45). A tetraploid population > 3% was found in 18/45 of the cases and was more frequent in single-gland lesions (15/34) than in multiple-gland lesions (3/11). The most significant discrete variables were growth pattern and binucleation or multinucleation. Clear and chief cells were classified correctly in 61.3% of the crossover validation tests using the standard deviation of nuclear shape karyometric variable and in 76.3% using the standard deviation of maximal correlation coefficient karyometric variable. Although ploidy pattern did not show significant differences, polyploidy, aneuploidy and tetraploidy > 3% were slightly more common in chief cells. CONCLUSION: In primary hyperparathyroidism there are no subvisual differences that can distinguish adenoma from hyperplasia. Nonetheless, by cytometry, most parathyroid cells can be classified correctly as clear or chief cells.

Diffuse tenosynovial giant cell tumor of soft tissues. Report of a case with cytologic and cytogenetic findings.

Extraarticular diffuse tenosynovial giant cell tumor is an unusual lesion the cytologic picture of which has not been discussed much in the literature. Fine needle aspiration biopsy of a nonpainful mass in the right shoulder in an 18-year-old woman revealed a highly cellular lesion consisting of polygonal cells and multinuclear giant cells with scant nuclear pleomorphism and a marked tendency toward xanthomization. Electron microscopy identified two basic cell populations among many intermediate forms: cells with scant filopodia and abundant ribosomes and cells with well-developed prolongations and numerous mitochondria, lysosomes and lipid drops. The karyotype of the tumor cells obtained from a surgically excised specimen showed a clonal population with 45, XX, t(1;2) (pter->p22::q24->pter), t(1:14)(qter->p13::q13->ter). The cytologic differential diagnosis included other tenosynovial lesions containing xanthomatous cells. Cytogenetic findings are discussed in relation to chromosomal alterations previously found in related lesions (nodular tenosynovitis and pigmented articular villonodular synovitis).

[Pericentric inversion of chromosome 9. Summary of the authors' results]. / Inversión pericéntrica del cromosoma 9. Resumen de nuestros resultados.

A review is given of our experience in the pericentric inversions of chromosome 9 (26 cases). We examine the relevance of this pericentric inversion in the phenotype of the carriers, the significance of pericentric inversions of chromosome 9 in infertility, and the occurrence of aneusomic recombinants. We also discuss the importance of pericentric inversions of chromosome 9 in the genetic counselling and their use as a family maker.