Lymph node biopsy for early diagnosis in Kawasaki disease.

Kawasaki disease or mucocutaneous lymph node syndrome is an acute exanthematous illness of childhood of unknown etiology with a recent marked increase in incidence. Occasional fatalities occur usually as a result of coronary thromboarteritis. Diagnosis is based on a spectrum of clinical signs and symptoms, some of which occur late in the acute phase of the illness. We recently examined cervical lymph node biopsies from two children during the early stage of illnesses which subsequently met the clinical criteria of Kawasaki disease and in which electron microscopy, cultures, serology, and other special studies failed to identify an etiologic agent. Both lymph node biopsies revealed multiple foci of necrosis and fibrin thrombi within the microvasculature, changes which have received little attention in the pathologic literature. These pathologic alterations are distinctive and probably characteristic. Early presumptive diagnosis of Kawasaki disease in our second case was based on the histopathologic findings and resulted in prompt institution of antithrombotic therapy. Lymph node biopsy has been underutilized in the diagnosis, and characteristic acute pathologic changes which may occur have been underpublicized.

XY gonadal dysgenesis associated with the congenital nephrotic syndrome.

The occurrence of a nephrotic syndrome in a female infant with a 46,XY karyotype is reported. The internal genitalia were female but a microscopic region of immature testicular tissue was present in 1 of 2 streak gonads. The relationship of this child's abnormal genital differentiation to her renal disease is discussed in the light of reported cases of infantile nephritis and Wilms' tumor in male pseudohermaphrodites. It is suggested that a common teratogen operating on the embryonic urogenital ridge may cause both genital and renal anomalies.