Light and electron microscope study of a case with abnormal lysosomes of granulocytes. Chediak-Higashi's disease?
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Biomedical subjects
Publications and source records attributed to D R Kelly.
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A multiinstitutional study to define the impact of total treatment programs involving radical neck dissection (RND) and modified neck dissection (MND) on patients' permanent disability was undertaken. A total of 243 patient responses were included in the study. Comparative analyses between the treatment groups show no advantage of one surgical operation over the other in returning patients to their pretreatment employment status. Radiation therapy was identified as adding significantly to the patient's permanent disability.
Pyruvate carboxylase deficiency results in congenital lactic acidosis. We report the significant finding in a child with infantile spasms controlled with adrenocorticotrophin hormone (ACTH) but who then developed severe lactic acidosis; pyruvate carboxylase deficiency was subsequently diagnosed. Blood lactate, pyruvate, and alanine levels were elevated, as well as cerebrospinal fluid alanine. Plasma alanine concentration was doubled by ACTH therapy. Fibroblasts contained extremely low pyruvate carboxylase activity. The patient died at 12 weeks of age after recurrent episodes of profound acidosis. At autopsy, the brain manifested cystic degeneration and demyelination. Pyruvate carboxylase deficiency is associated with neonatal onset of acidosis, delayed development, seizures, hypotonia, recurrent profound acidosis, and early death. The dramatic rise in plasma alanine content coincident with ACTH therapy suggest that ACTH played a role in precipitating the catastrophic metabolic acidosis.
Multiple parameters in the postlaryngectomy patient were statistically investigated with regard to acquisition and quality of alaryngeal speech. The 15 most significant variables are discussed. Esophageal peristalsis occurred during attempts to inject air for vocalization in 100% of the poor or nonspeakers. This may serve as an early indication to proceed with a synthetic vocal prosthesis.
More than 300 million routine clinical analyses are performed annually in the United States. Methods for routine clinical urine examination, including detection of bacteriuria, are briefly reviewed. Prospects of some newer, better techniques to carry out such analyses are introduced. A preliminary report is presented on the use of supravital microscopic fluorescence technique (SMFT), employing acridine orange as a non-specific staining fluorochrome. Results of examining 218 unspun urine specimens by SMFT are compared to traditional bacteriologic culture at a large pediatric hospital reference laboratory.
Congenital diaphragmatic hernia (CDH) was diagnosed using CT following intraamniotic contrast medium injection. Computed tomographic amniography may be helpful when the sonographic diagnosis of CDH is uncertain or prenatal surgical repair is being considered.
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Forty consecutive patients with small cell bronchogenic carcinoma were treated. The first 18 patients were treated with CCNU and doxorubicin (Adriamycin) (CA). The next 22 patients were treated with CCNU, doxorubicin, procarbazine, and vincristine (CAPO). Patient characteristics were similar. The partial plus complete response rate was 55% (ten of 18 patients) in the CA group compared to 41% (nine of 22 patients) in the CAPO group. The median survival from treatment was 28 weeks in the CA group compared to 33 weeks in the CAPO group. There were no drug-related deaths among the patients receiving CA compared to two definite and three probably drug-related deaths among the patients receiving CAPO. The addition of procarbazine and vincristine to CA for the treatment of small cell bronchogenic carcinoma resulted in increased toxicity and no survival benefit.