Chronic cytomegalovirus infection,immunodeficiency, and monoclonal gammopathy-antigen-driven malignancy?

An infant with severe combined immunodeficiency had normal numbers of lymphocytes which bore E rosette and surface Ig markers in an appropriate distribution. However, only minimal responsivity to in vitro stimulation by mitogens and allogenic cells, and none to antigens could be elicited; functional antibody responses were also nil, except to cytomegalovirus. Intrauterine-acquired cytomegalovirus may have caused his immune dysfunction, although the possibility of a postnatal infection cannot be excluded. Therapy with transfer factor and thymus transplantation was unsucessful in restoring immunity and may have aggravated a pre-existing monoclonal gammopathy. It is possible that the monoclonal protein was derived from B-cells transplacentally received from the patients mother.     

Adriamycin cardiotoxicity in a child with Wilms tumor. Report of a case and review of the literature.

A case of adriamycin-induced carciomyopathy in a 5 3/4-year-old girl with metastatic Wilms tumor is reported. Potentiation of the adriamycin cardiotoxicity by irradiation to the heart is proposed; the clinical and pathologic findings are discussed.     

Antidiuretic hormone excess.

Known physiologic mechanisms explain the elevated blood ADH levels observed in most patients with the syndrome of inappropriate ADH. Therefore the word "inappropriate" is a misnomer. It implies that the mechanisms that regulate ADH release are not functioning normally--which is not true. The term misleads the physician who, ideally, should determine why a patient has an excessive blood ADH level and initiate appropriate treatment. Patients with ectopic production of ADH and hyponatremia should be so labeled: "Hyponatremia due to ectopic ADH production." The term SIADH, if used at all, should be reserved for the rare patient with CNS injury or disease that causes increased ADH release and in which the hypothalamic center does not respond normally to afferent peripheral stimuli.     

Small intestinal disease in T cell deficiency

Biopsies of the proximal small bowel were obtained in four children with impairment of cell-mediated immunity, chronic diarrhia, and malabsorption; one child had an isolated T cell defect and three had significant B and T cell defects. All of them had malabsorption and, in addition to alterations of the small bowel epithelium, large vacuolated macrophages were seen in the lamina propria in all biopsies. Electron microscopy demonstrated lipid and patches of small pleomorphic inclusions within the macrophages. Since large vacuolated macrophages have also been described in Whipple's disease and chronic granulomatous disease, we suggest the possibility of a common thread of defective host defense and gastrointestinal malfunction in these three conditions.     

Noninvasive estimation of arterial oxygenation in newborn infants

Two noninvasive methods of estimating arterial oxygenation were compared in a group of 48 infants ranging in birth weight from 870 to 4,000 gm, with diagnoses including apnea of prematurity, hyaline membrane disease, meconium aspiration, and congenital heart disease. Both transcutaneous oxygen measurements and ear oximetry gave reasonably accurate estimations of arterial oxygen levels within commonly used clinical ranges (PO2 50 to 70 mm Hg, arterial saturation 90 to 98%). Infants with shock demonstrated a wide range of values for transcutaneous oxygen levels, suggesting that this method has limited usefulness in this situation. Ear oximetry had limited ability to distinguish high, but safe, levels of arterial oxygen from excessively elevated levels. While neither method can be recommended for replacement of arterial oxygen sampling, both methods may be useful in a clinical setting if care is exercised in interpretation of the results and if the values obtained are checked against those from arterial blood.