Little is known about the association of angiomyolipoma and adult renal-cell neoplasia. We studied the clinicopathologic features of 36 patients with concurrent angiomyolipoma and renal-cell neoplasia from the consultation and surgical pathology files of nine institutions. HMB-45 immunoreactivity was analyzed in both neoplasms. Twenty-five sporadic cases of patients with angiomyolipoma and renal-cell neoplasia and 11 cases of patients with tuberous sclerosis, as defined by Gomez' criteria, had mean ages of 59 and 53 years, respectively, and female-male ratios of 2:1 and 5:1, respectively. The mean size of the angiomyolipomas was 1 cm in the sporadic cases and 3 cm in those patients with tuberous sclerosis (medians: 0.5 and 3 cm, respectively, P ‫؍‬ .002). The mean sizes of the renal-cell neoplasms were 5 cm in sporadic cases and 6 cm in patients with tuberous sclerosis (medians: 4 and 5 cm, respectively; P ‫؍‬ .88). In both clinical settings, angiomyolipoma was more commonly the incidental tumor. Clear-cell (conventional) renal-cell carcinoma was the most common renal-cell neoplasm in both groups of patients, accounting for approximately two thirds of the tumors. In patients with tuberous sclerosis, 27% of renal-cell neoplasms were oncocytomas, compared with 8% in sporadic cases (P ‫؍‬ .15). Papillary neoplasia, chromophobe, and collecting-duct renal-cell carcinoma were found only in sporadic cases. All of the 22 renal-cell neoplasms studied were negative for HMB-45, whereas all 25 angiomyolipomas studied were positive.

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Three patients with concurrent hidradenitis suppurativa and Crohn's disease are presented. The notable histological feature in each hidradenitis resection was the presence of numerous discrete epithelioid granulomas in areas of non-inflamed dermis. The purpose of the study was to determine the incidence of epithelioid granulomas in 101 hidradenitis patients and their significance in relation to systemic granulomatous disease. Discrete epithelioid granulomas were identified in 8% of the resections (10 patients). One patient had Crohn's disease and one other pulmonary sarcoidosis. Seven patients with granulomatous hidradenitis neither had nor developed any other disease during the 3-year follow-up period. Clinical review identified a further two patients with Crohn's disease but associated with non-granulomatous changes in the skin resections. Foreign body type granulomas were identified in 25% of the resections adjacent to ruptured hair follicles, sinus tracts or nearby degenerate sweat glands. The study shows that, although foreign body type granulomas are a common finding in hidradenitis, the presence of discrete epithelioid granulomas in the dermis away from the site of active inflammation is unusual and should alert the pathologist to the possibility of systemic granulomatous disease such as Crohn's disease or sarcoidosis.

Trehalose is a disaccharide, the main dietary source being mushrooms. It has been approved as an additive in the preparation of dried food. Isolated intestinal trehalase deficiency is found in 8 % of Greenlanders, but is rare elsewhere. The normal range of trehalase activity and the incidence of isolated trehalase deficiency in the UK have not been reported. Patients (n 400) were investigated for suspected malabsorption. Endoscopic distal duodenal biopsies were taken for histological assessment and maltase, sucrase, lactase and trehalase estimation. Disaccharidase activities were determined by Dahlqvist's technique (Dahlqvist, 1968). Most patients (n 369) had normal duodenal histology. In these, square root transformation of trehalase activity produced a normal distribution. The normal range (mean Ϯ 2 SD) was 4⋅79-37⋅12 U/g protein. One patient had an isolated borderline trehalase deficiency. The thirty-one patients with villous atrophy had significantly reduced disaccharidase activities. With ingestion of a gluten-free diet, maltase, sucrase and trehalase activities recovered to normal in most patients, whereas lactase activity did not. The normal range and very low incidence of isolated enzyme deficiency is comparable with that described in populations from the USA and mainland Europe. Activity is significantly reduced in untreated coeliac disease and recovers with treatment with a gluten-free diet. There is no place for routine determination of trehalase activity in the UK population and there should be no concern over the introduction of trehalose-containing dried foods. Disaccharidase: Coeliac diseaseTrehalose is a disaccharide found in mushrooms, algae and insect haemolymph. The high concentration of trehalose in cryptobiotic plants is responsible for their remarkable ability to go through cycles of desiccation and rehydration without injury. This has led to interest by the food industry, as the addition of trehalose to foodstuffs improves the quality of dried food (Roser, 1991a, b;Roser & Colaço, 1993).Intestinal trehalase is a b-galactosidase which catalyses the hydrolysis of trehalose to two glucose molecules for absorption. It is present throughout the small intestine with highest levels in the proximal jejunum. Isolated trehalase deficiency occurs in at least 8 % of the Greenland population ) but only three cases have been reported elsewhere (Bergoz, 1971;Madzarovová-Nohejlova, 1973), two of whom were first degree relatives. There are no reported studies of the incidence of intestinal trehalase deficiency in a British population.The activities of other disaccharidases (maltase, sucrase and lactase) are low in patients with untreated coeliac disease. After treatment with a gluten-free diet, there is recovery of maltase, sucrase and sometimes lactase activities, although the latter only after several months.The aim of this present study was to assay trehalase activity in duodenal biopsies from patients presenting with suspected malabsorption and to determine a reference range in those with normal histol...

We report two pairs of siblings with the syndrome cranioectodermal dysplasia who have subsequently developed chronic renal failure secondary to tubulo-interstitial nephropathy. Renal disease has not been described before in this rare syndrome but has now occurred in four of the seven reported cases. Children with cranioectodermal dysplasia appear to be at significant risk of developing chronic renal failure. This is important information for clinicians looking after such children, and their families, when considering the long-term prognosis and management.