Treatment of acid maltase deficiency with a diet high in branched-chain amino acids.

Acid maltase deficiency in adults is associated with progressive muscle weakness and may effect respiratory muscles resulting in respiratory failure. The biochemical and clinical manifestations of acid maltase deficiency arise from a marked deficiency of the lysosomal enzyme alpha-glucosidase (acid maltase), which normally degrades glycogen to free glucose. In the past few years, high-protein diets have provided an alternative energy source for these patients and resulted in improved muscle strength. Recently, we treated a ventilator-dependent acid maltase-deficient patient with a general diet supplemented with branched-chain amino acids. Branched-chain amino acids are the principal amino acids involved in muscle protein synthesis and utilization. While on this diet, the patient had improvement of respiratory function and muscle strength and was able to be weaned from the ventilator during the day. In addition to his nutritional status, levels of serum branched-chain amino acids, showed improvement within 2 months after the diet started. This diet shows potential advantages over a high-protein diet without supplemented branched-chain amino acids for the treatment of acid maltase deficiency. These include theoretical sparing of amino acids required for muscle protein synthesis by providing higher concentrations of postprandial branched-chain amino acids in the circulation. Also, the liquid formula would be better tolerated by a ventilator-dependent or debilitated patient rather than a high-protein general diet. Further experience with branched-chain amino acid formulas will be needed to substantiate their efficacy in the treatment of acid maltase deficiency.

Diagnosis of carcinoid-like metastatic prostatic carcinoma by an immunoperoxidase method.

An unusual case of carcinoma of the prostate with metastases is described. the prostate and the metastases showed adenocarcinoma with carcinoid-like areas. A tumor with the same histologic features was found at the tip of the appendix and proved to be metastatic. The possibility of primary carcinoid of the prostate was considered; however, Fontana-Masson stain and electron microscopy failed to confirm this. Immunoperoxidase stain for prostatic acid phosphatase was done on the prostate and metastases. This stain is specific for tissues of prostatic origin. The stain was positive in the carcinoid-like areas, indicating that it was not a true carcinoid but rather prostatic carcinoma with a carcinoid-like pattern. Apparently, this is the first case of a metastatic prostatic carcinoma showing carcinoid-like areas that were positive for prostatic acid phosphatase.

The morphologic identification of Pneumocystis carinii.

The diagnosis of Pneumocystis carinii infection is dependent upon morphologic identification of the organism, making the staining properties of P. carinii important to the cytologist engaged in clinical service or research. Several special stains specific for P. carinii are described; in addition, the staining characteristics of the organism in Papanicolaou and in hematoxylin and eosin stains are described. The successful identification of P. carinii with the latter two stains has not been reported previously. Because these stains are often used on lung preparations, it may be possible to identify P. carinii when using these latter two techniques; however, for optimal results, we feel that it is essential to utilize one or more of the special stains discussed herein.