The Institute of Medicine's report on medical error. Implications for pathology.

CONTEXT: During the past several years, more attention has been focused on the topics of medical error and patient safety than in the past. At the end of 1999, the Institute of Medicine (IOM) published a seminal report concerning medical error in the United States; this report will have sweeping implications for all disciplines of medicine, including pathology. OBJECTIVE: To review the major findings of the IOM report on medical error and to discuss their implications for the field of pathology. METHODS: Review of the IOM report on medical error and discussion of other relevant literature on medical error. RESULTS: The IOM report on medical error highlights an unacceptable rate of medical error in the United States and mandates a 50% reduction in medical error during the next 5 years. It recommends regulatory solutions to this problem, as well as organizational approaches to error reduction. It proposes both mandatory and voluntary systems for reporting of medical error. The report suggests that systems should be examined for latent flaws and that individual culpability for error should not be overemphasized. The report recommends that error-reduction strategies that have been applied to other industries should be studied and that known concepts of error reduction should be applied to medicine. Strategies that the IOM suggests can be applied to pathology. CONCLUSIONS: Medical error occurs at an unacceptably high rate. Recommendations made in the IOM report on medical error and patient safety should be applied to the practice of pathology.

The intrathyroidal hyperfunctioning parathyroid gland.

Six cases of primary hyperparathyroidism due to hyperfunctioning intrathyroidal parathyroid glands are reported. In five cases, hyperparathyroidism was due to an intrathyroidal parathyroid adenoma; in the sixth case, hyperparathyroidism resulted from an intrathyroidal parathyroid carcinoma. All five patients with adenoma were female with ages ranging from 40 to 70 yr. The patient with carcinoma was a 55-yr-old male. In all five patients with intrathyroidal parathyroid adenoma, thyroidectomy was performed when an abnormal parathyroid gland could not be located in the neck during surgery for hyperparathyroidism. The patient with intrathyroidal parathyroid carcinoma presented with hypercalcemia and a palpable right thyroid mass. The differential diagnosis of intrathyroidal parathyroid adenoma includes thyroid follicular adenoma. In some cases, the possibility of medullary carcinoma of thyroid might also be considered. Immunocytochemical staining for parathormone (PTH), thyroglobulin, and calcitonin is valuable in establishing the correct diagnosis.

Unique sensitivity of Hb Zürich to oxidative injury by phenazopyridine: reversal of the effects by elevating carboxyhemoglobin levels in vivo and in vitro.

Phenazopyridine (PAP) causes a hemolytic anemia in normal individuals who receive an overdose or in patients with decreased renal function given therapeutic doses. There are no reports of PAP-induced hemolysis in individuals with unstable hemoglobins. Therapeutic doses of PAP administered to a subject with Hb Zürich (His E7 (63) beta leads to Arg) caused a severe hemolytic anemia with many large Heinz bodies appearing in the red cells. Incubation of whole blood from three asymptomatic Hb Zürich subjects with PAP at a molar ratio of PAP/Hb of 1.3/1 produced a moderate to marked increase in methemoglobin (MHb) and Heinz body formation accompanied by a slight to moderate decrease in levels of reduced glutathione. The rates of MHb formation were proportional to the concentration of PAP. In two of the subjects the rates of formation of Heinz bodies and MHb were substantially higher than in the third subject. Red cells from five normal adults were not affected. In Hb Zürich red cells the affinity of the abnormal beta chain for carbon monoxide is much greater than that of the normal alpha and beta chains. The two subjects with higher rates of MHb and Heinz body formation were nonsmokers with in vivo carboxyhemoglobin (HbCO) levels of 4-6%. The third subject with low rates of MHb and Heinz body formation was a smoker with in vivo HbCO levels of 15-18%. Increasing levels of HbCO from 8.2% to 14.3% by the in vitro addition of CO caused a marked reduction in the rate of Heinz body formation and a moderate decrease in MHb formation. Rates of MHb formation similar to those observed in normal red cells occurred at HbCO percentages of 89.2 and 99.2. Red cells containing Hb Zürich are extremely sensitive to oxidative injury by PAP, either in vivo or in vitro. The degree of oxidative injury diminishes as the level of HbCO increases, a phenomenon that is enhanced by preferential binding of CO to the abnormal beta subunit of Hb Zürich.

Mixed tumors of the vagina. A clinicopathological analysis of eight cases.

Eight mixed tumors of the vagina are described. Seven of them were situated in or near the hymenal ring. All but one were well-circumscribed masses unconnected to the surface epithelium. They were composed of small stromal-type cells usually containing islands of mature squamous cells and glands lined by mucinous epithelium. Their histogenesis is not clear. Follow-up of 2--9 years in seven of the cases has revealed no evidence of recurrence or metastases after local excision.

Infant with abnormal pigmentation, malformations, and immune deficiency.

An infant had swirling hyperpigmentation, streaks of hypopigmentation, abnormal T-cell function, cleft palate, patent ductus arteriosus, and arrhinencephaly. This pattern of abnormalities is distinct from other disorders with abnormal pigmentation and malformations; such as incontinentia pigmenti, incontinentia pigmenti achromians, and the epidermal nevus syndrome.

Epithelioid sarcoma of the hand and wrist: patterns of extension.

The sites of local and remote extension of primary epithelioid sarcoma of the hand and wrist were studied in six patients. The tumor was found to spread to noncontiguous areas of skin, subcutaneous tissue, fascia, bone, and lymph nodes as well as by direct extension along fascial planes. Neither local nor remote extension of the tumor could be determined accurately or dependably by clinical examination, radiography, xerography, lymphangiography or radioisotope scanning. Wide en bloc excision of the tumor or amputation is recommended as the primary treatment of epithelioid sarcoma of the hand. Axillary node dissection is advised in all cases.

Kaposi's sarcoma presenting as autoimmune hemolytic anemia.

A case of Kaposi's sarcoma which presented as a warm type of immune hemolytic anemia is described. The malignancy was discovered at the time of splenectomy, which was required for control of the hemolytic anemia. Three other cases of immune hemolytic anemia in patients with Kaposi's sarcoma have been reported. An association between Kaposi's sarcoma and immune hemolytic anemia is suggested. Careful examination of the skin for Kaposi's sarcoma seems appropriate in cases of immune hemolytic anemia.