Nails and nail disorders in children and adults.

Abnormalities of the nails can provide both subtle and obvious clues to common medical problems or severe systemic diseases. Heredity, minor injuries and a variety of infections account for many changes in the appearance of nails. Several dermatologic disorders, such as psoriasis and alopecia areata, and other multisystem disorders may present with nail changes; abnormalities of the nails may be the single most prominent feature. Common habits and the use of various cosmetics also may result in nail changes. Awareness of normal nail variants and well-defined abnormalities and their disease associations will be beneficial to most clinicians. Specific management and therapy for most nail problems are usually simple and appropriate for primary care physicians.

Hemoglobin-specific antibody in a multiply transfused patient with sickle cell disease.

Human hemoglobins (Hbs) are known to be immunogenic, and both normal and variant forms of Hb have been shown to stimulate antibody formation in a variety of animal species. In patients who are homozygous for the sickle Hb (HbS) mutation, transfusion of normal, HbA-containing erythrocytes provides a potential stimulus for HbA alloimmunization. We tested serum samples for the presence of anti-Hb antibody by a solid-phase enzyme-linked immunosorbent assay (ELISA) using Hb-coated polystyrene microtiter plates. Hb-bound antibody was identified using an antihuman IgG antibody. Serum samples from 89 patients with sickle cell disease were initially tested for evidence of Hb antibody. The serum from three individuals exhibited antibody activity against HbA with little or no activity against HbS. Only one of them, a multiply transfused adult with HbSS, was available for further study. When this patient's antibody was tested against a variety of normal and mutant Hbs using antibody either to human IgG or to kappa chains, the anti-Hb antibody demonstrated specificity for the region of the Hb beta chain corresponding to the site of the amino acid substitution of HbS. The level of activity of the patient's anti-HbA showed no significant change over 1.5 years of observation. The transfusion of erythrocytes containing Hb structurally different from that of the recipient appeared to be capable of stimulating the production of Hb-specific alloimmune antibody.

Endoscopic laser therapy for gastrointestinal bleeding from congenital vascular lesions.

Endoscopic laser therapy may be useful in controlling upper gastrointestinal bleeding due to vascular abnormalities. This report presents the case of an 11-year-old boy with an unusual vasculocutaneous disorder complicated by chronic recurrent bleeding that necessitated frequent blood transfusions. Of a number of investigative tests, only endoscopy was diagnostic in determining that the bleeding was secondary to multiple gastric vascular lesions. Moreover, laser photocoagulation of the identified lesions resulted in a dramatic decrease in both transfusion requirements and hospitalization.

Sickle cell anemia in two White American children: essential laboratory criteria for diagnosis.

A number of hematologic disorders share diagnostic and clinical features of sickle cell anemia but have significantly different genetic implications and prognosis. Because of these differences, the establishment of a precise diagnosis is essential for the child in whom any form of sickle cell disease is identified. To illustrate the requirements for a definitive laboratory diagnosis of sickle cell anemia, this report presents the approach to establishing this diagnosis in two white American patients. From a review of the literature, these patients appear to be the only white Americans with sickle cell anemia in whom the diagnosis has been unequivocally established.