Processing of adenoid and tonsil specimens in children: a national survey of standard practices and a five-year review of the experience at the Children's Hospital of Pittsburgh.

The best means of pathologically examining routine tonsillectomy and adenoidectomy specimens in children remains controversial. Otolaryngologists fear missing an unsuspected diagnosis. However, the cost-effectiveness of microscopic analysis, given the rare incidence of unsuspected diagnosis, is questionable. If a significant pathologic diagnosis is missed, the medicolegal implications could be significant. A questionnaire was sent to 111 members of the American Society of Pediatric Otolaryngology. Additionally, we reviewed our experience at the Children's Hospital of Pittsburgh for the 5-year span from 1989 to 1994 to determine our incidence of unsuspected pathologic diagnoses. Sixty-five questionnaires were returned (59% response rate). More than half (56%) of the respondents stated that microscopic analysis was routinely performed on all specimens, and 42% replied that only gross examination was performed, reserving microscopic examination for selected cases. Three respondents said that they discarded their specimens in the operating room. From March 1989 to October 1994, in 1985 children undergoing bilateral tonsillectomy and adenoidectomy at the Children's Hospital of Pittsburgh, no significant pathologic diagnoses were found. Twenty-seven additional children who underwent only tonsillectomy between January 1991 and October 1994 were also reviewed. One lymphoma, suspected before surgery, and a glycogen storage disorder, not suspected before surgery, were diagnosed. Therefore, in a total of 2012 children, we found only one clinically significant unsuspected diagnosis. In conclusion, we found no national consensus governing the best way to examine routine adenotonsillectomy specimens in children. Given that unsuspected diagnoses are rare, reserving microscopic analysis for specific clinical indications may be both more cost-effective and medically feasible.

A micro-radiochemical assay for alpha-1,4-glucosidase and its use in the assessment of type II glycogenosis (Pompe's disease).

An assay for alpha-1,4-glucosidase (acid maltase) activity which is deficient in Pompe's disease is described. The assay can be used to measure the enzyme in cultured skin fibroblasts, cultured amniotic cells and peripheral blood leucocytes. [U-14 C]Maltose is used as the substrate in a total assay volume of 8 microliter. The product, [U-14C]glucose, is separated from the substrate by cellulose thin-layer chromatography. The procedure permits replicate assays from 400 microliter whole blood and from amniotic cells in primary culture. Discrimination of the heterozygous Pompe state appears to be facilitated.