Icteric plasma suggests Gilbert's syndrome in the blood donor.

BACKGROUND: In a recent quality assurance audit of component returns over a 6-month period, 9 of 81 returns were due to icteric plasma. With the sensitive, new methods used to screen donors for anemia and hepatitis, it seemed likely that the icteric discoloration reflected benign unconjugated hyperbilirubinemia (Gilbert's syndrome) in the donor, rather than liver disease or hemolysis. The donors were recalled for repeat blood study to resolve this question. STUDY DESIGN AND METHODS: Seven of the nine donors could be reached, and they submitted blood samples for measurement of serum levels of conjugated (direct-reacting) and total bilirubin and for complete blood and reticulocyte counts. RESULTS: All seven donors had mild unconjugated hyperbilirubinemia, with total bilirubin levels ranging from 1.3 to 2.8 mg per dL. None showed evidence of overt hemolysis. CONCLUSION: All seven donors of the components with icteric plasma have Gilbert's syndrome, a benign genetic anomaly occurring in approximately 3 to 5 percent of the general population. With the sensitive screening tests for viral hepatitis used today, the presence of icteric plasma in a component suggests that the donor has Gilbert's syndrome. Policies about the acceptability of icteric components from blood donors merit reassessment.

Freud's Jocasta and Sophocles' Jocasta: clinical implications of the difference.

Evidence is presented that the Jocasta Sophocles depicted in Oedipus Rex is significantly different from the Jocasta of Freud's synopsis of the play. Sophocles' Jocasta prefers her son to her husband and this preference results in parricide and incest. The clinical implications of this difference are discussed in relation to a group of patients whose mothers resembled Sophocles' Jocasta. The analysis of one such patient is briefly presented. All these patients had difficulties at work and in their psychosexual development. It is argued that these difficulties resulted from the mother's attitude interfering with the dissolution of the Oedipus complex and that quite severe pathology does not necessarily mean pre-oedipal conflicts.

Veno-occlusive disease of the liver in children following chemotherapy for acute myelocytic leukemia.

Three children developed acute veno-occlusive disease of the liver following combination chemotherapy for acute myelocytic leukemia. The clinical presentation was similar in all three, with acute onset of hepatomegaly and thrombocytopenia in the absence of significant transaminasemia or icterus. In all three patients, radionuclide imaging with technetium-99m sulfur colloid showed hepatosplenomegaly, decreased liver uptake, and increased splenic activity. The results of liver biopsy established the diagnosis, revealing marked centrilobular congestion with hemorrhage into the spaces of Disse, atrophy of central hepatic cords, and edema of the walls of the central and sublobular veins. Each patient showed marked improvement following temporary cessation of chemotherapy. The diagnosis of veno-occlusive disease is suggested by the triad of: (1) clinical signs and symptoms; (2) scintigraphic findings; and (3) temporal relationship to chemotherapy.

Sternal infarction in sickle-cell anemia: concise communication.

Bone scintigraphy in six children with sickle-cell anemia has demonstrated infarction of bony segments of the sternum. Anterior oblique views of the thorax provided best visualization of the infarcts which were seen as areas of decreased bone tracer accumulation.

Brown tumor of the orbit associated with primary hyperparathyroidism.

A 70-year-old woman had swelling of her left superior orbital ridge. A tumor was surgically removed, and histologic examination disclosed a tumor containing giant cells. Subsequent tests showed primary hyperparathyroidism caused by a carcinoma of the parathyroid. This rare instance of brown tumor of the orbit led us to review the differential diagnoses of giant-cell lesions of the orbit and to examine published case reports. Laboratory determinations of serum calcium, phosphorus, and alkaline phosphatase levels for all patients with lytic orbital bone lesions can establish whether they have hyperparathyroidism, a diagnosis making orbital surgery unnecessary.

Scrotal imaging in the Henoch-Schönlein syndrome.

Testicular involvement in patients with Henoch-Schönlein syndrome may occur in as high as 15 per cent of the cases. Scrotal imaging is extremely accurate in differentiating hyperemia (vasculitis) from impairment of blood flow encountered in torsion of the spermatic cord, the most common cause of acute scrotal swelling in children.

A new syndrome of refractory sideroblastic anemia with vacuolization of marrow precursors and exocrine pancreatic dysfunction.

In the past decade, we have studied four unrelated children with what we believe is a previously unreported disorder affecting the bone marrow and exocrine pancreas. During infancy these patients had the onset of severe, transfusion-dependent, macrocytic anemia plus a variable degree of neutropenia and thrombocytopenia. Their bone marrows had normal cellularity but were characterized by remarkable vacuolization of erythroid and myeloid precursors, hemosiderosis, and ringed sideroblasts. The vacuoles probably represented manifestations of cellular degeneration and death. In two patients, in vitro bone marrow cultures showed abnormal erythroid and myeloid progenitor cell growth and, in one child, abnormal vacuolated erythroid colonies. Family histories were unrevealing, parents were hematologically normal, and both sexes were involved. There was no evidence of specific nutritional deficiencies or exposure to agents associated with marrow vacuolization. A number of therapeutic interventions produced no effect. One child had clinical malabsorption. This child and one other had extensive pancreatic fibrosis at autopsy. The other two patients had findings indicating exocrine pancreatic dysfunction. Two children had splenic atrophy. This new syndrome, with associated bone marrow and exocrine pancreatic dysfunctions, differs in several respects from the syndrome of pancreatic liposis and neutropenia described by Shwachman et all and Bodian et al, and from other conditions with vacuolization of the marrow or sideroblastosis.

Retrolental fibroplasia in hypoxic newborn.

A premature infant with hypoxia caused by severe cardiac malformations developed stage IB retrolental fibroplasia, which was documented after death. Despite arterial oxygen partial pressures of 91 mm Hg or less the classical histologic changes were observed in the temporal periphery of each eye.