Some FLC clones can promote rapid renal scarring. Significant reductions in cast formation on repeat biopsy may identify the potential for late renal recovery. Early diagnosis and treatment may prove crucial in determining renal recovery. Patients who have not recovered renal function after a period of treatment may be usefully reassessed by repeat biopsy for quantitative analysis of chronic damage and cast numbers.

No abstract

A peroxidase-antiperoxidase technique for S-100 protein has been applied to 68 salivary glands. These included 17 pleomorphic adenomas, seven adenoid cystic carcinomas, 23 adenolymphomas and a number of other neoplasms. In addition, five specimens of myoepithelial sialadenitis ('benign lymphoepithelial lesion') and five normal parotid glands were included. Consistent results were obtained, myoepithelial cells and cells in myxoid and chondroid areas in pleomorphic adenomas staining intensely. In adenoid cystic carcinoma, on the other hand, there was no staining. The adenolymphomas possessed intensely S-100 protein-positive cells in the interfollicular lymphoid areas; these were probably interdigitating reticulum cells. In addition, branching structures, probably corresponding to Langerhans' cells, were observed in the epithelium of adenolymphomas.

Voth' first recognised this rare condition in 1962 and defined it as arachnocytosis of the heart muscle. In 1965 Hudson2 referred to a child who had died in atrial tachycardia and whose heart showed an unusual form of lipid storage disease with no other organ affected.The cases reported have been predominantly in females and under 24 months of age. Various arrhythmias, mainly tachycardias, have been detected before death in several cases. Sudden death in an apparently healthy infant, however, has been documented in several others.At necropsy the heart is enlarged. Histological examination shows myocytes with a swollen, foamy, and granular cytoplasm. Ultrastructural studies have shown that this granularity is due to increased numbers of enlarged mitochondria.The aetiology is unknown, but a genetic basis has been proposed, based on the strong female preponderance.3 The following case reports add further evidence to a hypothesis of a genetic aetiology. Case reports CASES 1 AND 2Two children were born to caucasian nonconsanguineous parents (family A), were separated by an eight year interval. A third sibling, a 7 year old male, at the time of death of the second child, is alive and well at the time of writing.