Cure of Goodpasture's disease.

Goodpasture's Disease is an explosive multisystem disease presenting initially as a pulmonary-renal syndrome. There is often little margin for error in making an early correct diagnosis to avoid respiratory and renal failure. Complications of invasive diagnostic testing and aggressive immunosuppressive treatment often lead to other organ dysfunction due to infection, hemodynamic disturbances, fluid and electrolyte challenges, and nutritional deficiency. Artificial organ therapy is often needed for pulmonary and renal support, for immunomodulation, and for nutritional replacement. The outcome is often considered satisfactory if the patient survives the acute presentation. Persistent organ failure is quite often the case, especially renal failure. Quality of life is often improved if there is subsequent renal transplantation. By then the patient may be considered "cured". Two cases are described below, contrasting young and elderly white females both in previous good health who developed rapid onset of Goodpasture's disease. One was cured after transplantation, and one was cured without transplantation. The severity of the renal involvement was the same for both patients. Cure of Goodpasture's Disease in the native kidneys is uncommon; a review of the literature is provided.

Cerebral vasculitis associated with acute post-streptococcal glomerulonephritis.

The following is a case study involving a 13-year-old girl who presented initial symptoms of an upper respiratory infection. One week later she experienced a short seizure and hours later a grand mal seizure. MRI examination of the brain demonstrated multiple changing abnormal foci of increased density in white and gray matter suggestive of a vasculitic inflammatory pattern. As a result of proteinuria and red cell casts on urinalysis, a renal biopsy was performed resulting in a diagnosis of acute post-streptococcal glomerulonephritis (APSGN). We concluded on the basis of the MRI that vasculitis was secondary to APSGN. The following paper is a description of our findings in this case and a review of the literature supporting this new interpretation of CNS disease due to APSGN.

Ultrastructural evaluation of renal cell oncocytomas.

The utility of ultrastructural evaluation of eosinophilic renal cell neoplasms is illustrated by two case studies. The differential diagnosis between granular renal cell carcinoma and renal cell oncocytoma may be difficult. Ultrastructural demonstration of the presence of abundant mitochondria is useful in the definitive diagnosis of fine-needle aspiration specimens, those neoplasms with nuclear pleomorphism, or in patients requiring renal parenchymal sparing surgery.

Enhancement of murine susceptibility to oral lactate dehydrogenase-elevating virus infection by non-steroidal anti-inflammatory agents, and antagonism by misoprostol.

The murine lactate dehydrogenase-elevating virus (LDV) was used to study the effects of prostaglandin-acting agents on mucosal resistance to virus infection. Mice treated with non-steroidal anti-inflammatory drugs (NSAIDs) prior to oral exposure to LDV demonstrated a reduction in the mucosal barrier to LDV infection. Histological studies indicated that these NSAID effects were not a result of gross or microscopic tissue damage. The effects of two NSAIDs, indomethacin and diclofenac, were inhibited by co-treatment of mice with misoprostol, a synthetic PGE1 analog. The ability of misoprostol to modulate NSAID effects was not due to direct antiviral activity or to actions on LDV-permissive macrophages. These results show that the mammalian mucosal barrier to virus infection is prostaglandin-sensitive, and provide a model for the study of resistance to viral infection.

Effect of age on the sensitivity of cell cultures to Clostridium difficile toxin.

The effect of age on the sensitivity of four cell lines, human foreskin fibroblasts (HFS), CHO-K1, HEp-2, and WI-38 to detect Clostridium difficile toxin was tested. This study also addressed the sensitivity of these cell lines as expressed by early toxin detection. Twenty-eight positive and 13 negative patient specimens were tested. Cell cultures were inoculated at ages 3, 4, 5, 6, 7, 9 and 14 days and examined for cytopathic effects at 4, 24, and 48 hours post-inoculation. The sensitivity of three of the four cell cultures to C. difficile toxin decreased as the age of the cell cultures increased. However, the sensitivity of the HFS cell line was not influenced by the age of the culture or the time the assay was read in comparison to the other three cell lines. Five- to six-day-old HFS cell cultures detected 22/28 positive samples within 4 hr after inoculation and 28/28 positive samples by 24 hr post-inoculation.

Idiopathic arterial calcification of infancy in newborn siblings with unusual light and electron microscopic manifestations.

We report two cases of idiopathic arterial calcification of infancy (IACI) occurring in newborn siblings. Unusual gross and light microscopic findings included calcification of multiple nonarterial tissues, renal infarction, and macroscopic aortic involvement. One case manifested an extensive acute panarteritis suggesting that IACI may be the result of an inflammatory or infectious process. Ultrastructural examination confirmed that the mineral deposits were hydroxyapatite and also indicated that they contained iron. Calcified tissue components included smooth-muscle cells, fibroblasts, and collagen fibers, as well as elastic fibers. No matrix vesicles or mitochondrial calcifications that may serve as nucleation sites for crystalline calcium phosphate were identified. Thus, the mechanism of calcification in IACI may be unique, and may relate to altered iron metabolism.