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Intravenous immune globulin (IVIg) has been advocated as efficacious therapy for a variety of disorders including idiopathic thrombocytopenic purpura and Kawasaki disease. Several reports have also documented the effectiveness of IVIg in systemic lupus erythematosus (SLE). Two patients with symptomatic SLE and ESRD were treated with IVIg. Both patients tolerated IVIg administration well and demonstrated clinical and serologic improvement. Both individuals also experienced a transient decline in serum albumin concentration with IVIg treatment. The mechanisms by which IVIg might have effected improvement in these patients are varied and are likely related to the immunomodulatory actions of IVIg. The reversible change in albumin concentration seen in these individuals may be secondary to abrupt alterations in oncotic homeostasis. Despite this unusual effect, the documented improvement in these patients suggests that IVIg therapy may be of benefit in patients with active SLE and ESRD. Further studies are warranted to examine the mechanisms by which IVIg may exert its therapeutic effect.
We report five cases of nephrotic syndrome due to focal segmental glomerulosclerosis (FSGS) in mentally retarded children with severe infantile spasms. Four of the five children diagnosed as West syndrome, Lennox syndrome, or petit mal epilepsy also had cerebral palsy and microcephaly. The other patient had petit mal epilepsy without cerebral palsy and microcephaly. All patients first developed infantile spasms, with the time of onset ranging from 1 week to 2 years of age, and subsequently developed proteinuria, followed by the nephrotic syndrome at 3 to 14 years of age. Four of the five developed terminal renal failure between 7 and 11 years of age. Three subsequently died, but the other underwent kidney transplantation and is still living without further complications. The light, electron microscopic, and immunohistochemical studies performed on the renal biopsies from all the patients and on the autopsy specimens from two cases exhibited FSGS-like lesions. Besides segmental hyalinosis, differing degrees of mesangiolysis were seen, which sometimes developed into dissecting microaneurysms of the glomerular capillary loops. The clinical picture described can be differentiated from congenital nephrotic syndrome (CNS) or infantile nephrotic syndrome (INS) with respect to the age of onset, outcome, and morphological appearance. We reviewed the previous literature and extended earlier observations about an unusual association between the nephrotic syndrome due to FSGS-like lesion, mental retardation, infantile spasms, and/or microcephaly in children.
The immune system of marine mammals is of comparative interest because of its adaptation to the aquatic environment. Little information, however, is available on its cellular and molecular components. Here, we used a cross-reactive antibody to MHC class II molecules and an immunoglobulin-specific antiserum for identifying these molecular species on lymphocytes of the bottlenosed dolphin, Tursiops truncatus. Limited structural analyses indicated that class II molecules and immunoglobulins of dolphin closely resemble those of other vertebrates. In the peripheral blood of most land mammals both class II and immunoglobulins are usually found on B but not T lymphocytes. Expression of immunoglobulins on dolphin peripheral blood lymphocytes suggests a ratio of B cells to T cells comparable to that of land mammals. However, unlike the majority of land mammals, virtually 100% of the peripheral T cells display pronounced expression of class II molecules, generally considered an indication of T cell activation. It is therefore possible that the physiology of T cell activation has unusual attributes in the dolphin. It is especially interesting that some land mammals, namely swine (ungulates) and dogs and cats (carnivores), also express class II molecules on peripheral blood T lymphocytes. Since ungulates and carnivores are thought to share a common distant ancestry with toothed whales, the evolutionary history may be more relevant than the environmental history in determining these unusual attributes.