Alagille's syndrome associated with cystic renal disease

Although renal abnormalities have been described in children with Alagille's syndrome, cystic kidney disease has not often been documented, and then usually only at necropsy. Three children with Alagille's syndrome are described, in two of whom a unilateral multicystic dysplastic kidney was detected by prenatal ultrasound; in the other, a solitary cortical cyst was found later in childhood. All have normal renal function, growth, and liver synthetic function but continue to have clinical and biochemical signs of cholestasis. These cases show that unilateral cystic kidney disease with or without renal dysplasia may be associated with Alagille's syndrome, that the clinical course is not necessarily unfavourable, and that Alagille's syndrome should be included in the differential diagnosis of cystic kidney disorders associated with cholestatic liver disease. Patients with Alagille's syndrome should be evaluated by renal ultrasound.     

Knowledge of renal histology alters patient management in over 40% of cases

There is great debate as to whether the benefit gained fromthe knowledge of renal histology outweighs the risk to the patientfrom the biopsy procedure. We conducted a prospective studyof 276 native renal biopsies performed on 266 patients froma single centre in 1991 to assess the effect of the knowledgeof renal histology on patient management. Biopsies were performedunder ultrasound guidance using the Trucut biopsy needle. Theindications for biopsy were: non-nephrotic proteinuria alone(25), haematuria and proteinuria (28), nephrotic range proteinuria(28), acute renal failure (31), haematuria alone (36), and chronicrenal failure (128). Two hundred and sixty-three biopsies weresuccessful. The mean number of glomeruli obtained was 23, range0–115. Eight patients developed macroscopic haematuriaof which two required blood transfusion. The result of the biopsyaltered management in 24/28 (86%) of cases of nephrotic rangeproteinuria, 22/31(71%) of cases of acute renal failure, 58/128(45%) of cases of chronic renal failure, 9/28 (32%) of caseswith haematuria and pro teinuria, 3/25 (12%) of cases with non-nephroticproteinuria alone, and 1/36 (3%) of cases with haematuria alone.Management was altered in 42% of cases overall. These data suggestthat knowledge of renal histology is essential in the managementof patients with renal disease.     

On the fine structure and distribution of secretory cells in the supraoesophageal ganglion of the lugworm (Arenicola marina L.)

The ultrastructure of the brain in Arenicola has been investigated with special reference to secretory cells and processes. Electron-dense secretory granules (elementary granules) are found in neurones, both in the brain proper and in the neuroectodermal connectives. Based on differences in fine structure four categories of secretory neurones appear distinguishable: (i) large dorsal neurones with elementary granules < 1200 Å in mean diameter, probably containing nonpeptidergic secretion; (ii) equally large neuroectodermal cells with elementary granules > 1200 Å in mean diameter, which may contain peptidergic secretion; and (iii) two types of elongate or fusiform cells, which, in some cytological details, mimic either dorsal neurones or neuroectodermal cells; they contain, respectively, elementary granules < 1200 Å and > 1200 Å in mean diameter. The similarities which exist between the elongate cells and the two categories of larger neurones leave some doubt about their separate status. The neuroectodermal cells and the elongate cells with larger granules are confined to the subectodermal nervous tissue and the connectives. The majority of the processes of these cells terminate on the perineurium of the connectives. Dorsal neurones and their processes are chiefly confined to the brain proper. From previous experiments it is known that the maturation hormone produced by the brain is found only in the posterior half of the brain. The zonation of secretory cells and processes, described here, fails to match this known variation in hormone content. No obvious storage/release site has yet been detected, and it seems likely that hormone is released via terminals scattered over the perineurium.