Sclerosing encapsulating peritonitis: a case series from a single U.K. center during a 10-year period.

Sclerosing encapsulating peritonitis (SEP) is a serious complication of peritoneal dialysis (PD). Previous reports place the prevalence of SEP at 0.54%-7.3%. We estimated the prevalence of SEP in our unit to be 1.4% over the period 1989-1999. We here present the 6 identified cases. All of the patients presented with small-bowel obstruction; hemorrhagic ascites was identified in 3 cases. All 6 patients experienced ultrafiltration inadequacy, and 5 were treated with glucose polymer (icodextrin; duration of treatment: 1 month-2.5 years). Peritoneal dialysis was stopped at the time of diagnosis in 2 cases. In the other 4 cases, PD had been withdrawn some time prior to the SEP being diagnosed (2 weeks-5 years). Five of the patients have died; the 6th currently uses hemodialysis.

Calciphylaxis.

The phenomenon of calciphylaxis is rare, but potentially fatal. It has been recognised for a long time in patients with chronic renal failure with secondary hyperparathyroidism. Disturbed calcium and phosphate metabolism can result in painful necrosis of skin, subcutaneous tissue and acral gangrene. Appearance of the lesions is distinctive but the pathogenesis remains uncertain. The beneficial effects of parathyroidectomy are controversial. However, correction of hyperphosphataemia or occasionally hypercalcaemia is imperative. Fulminant sepsis as a consequence of secondary infection of necrotic and gangrenous tissue is a frequent cause of patient morbidity and mortality.

Does immunosuppression with prednisolone and azathioprine alter the progression of idiopathic membranous nephropathy?

The role of immunosuppressive drugs in the treatment of idiopathic membranous nephropathy (IMN) remains controversial. The effect of treatment with prednisolone and azathioprine in patients with nephrotic-range proteinuria and biopsy-proven IMN from a single center (Sheffield Kidney Institute, Sheffield, UK) is described. In this retrospective study, 58 patients with IMN and nephrotic-range proteinuria were followed up for 4 years. Thirty-eight patients were treated with prednisolone (1 mg/kg body weight/d) and azathioprine (2 mg/kg body weight/d) orally for a median period of 26 months (range, 6 to 48 months). Twenty patients received no specific treatment for IMN and served as a control group. Clinical, biochemical, and histopathologic features at presentation were similar between the groups. Renal function (RF), measured by serum creatinine (Scr) level, deteriorated in both treated and control groups during the follow-up period. The median initial and final Scr levels (at the end of follow-up) in the treated group were 1.6 and 2. 1 mg/dL, respectively, and in the control group were 1.3 and 1.7 m/dL, respectively (P = not significant). Neither the rate of RF decline (measured by the slope of reciprocal of Scr against time) nor the proportion of patients with deteriorating RF differed significantly between the groups (37%, treated group; 30%, control group). A significant reduction in proteinuria was observed in both groups (P < 0.01, either group). Also, the rate of remission of nephrotic-range proteinuria was not significantly different between groups (55%, treated group; 65%, control group). The only prognostic factor that correlated with RF outcome (expressed by final Scr level) in a given patient was the mean proteinuria during follow-up in either group (r = 0.493; P < 0.01, treated group; r = 0.651; P < 0.01, control group). Adverse effects of immunosuppressive treatment were observed in nine patients (24%). These were serious in four patients (10%) and included squamous cell carcinoma (two patients), bacterial meningitis (one patient), and septicemia (one patient). In conclusion, treatment with prednisolone and azathioprine for patients with IMN did not show significant beneficial effects on the progression of disease. Furthermore, this treatment was associated with frequent and serious adverse effects.

Can immunosuppressive drugs slow the progression of IgA nephropathy?

The role of immunosuppressive drugs in the treatment of IgA nephropathy (IgAN) remains controversial. The effect of treatment with prednisolone and azathioprine on the clinical course of patients with IgA nephropathy is described in this retrospective study. One hundred and fourteen patients, 66 treated (age 13-77 years) and 48 untreated (age 15-64 years), were evaluated. The two groups of patients differed significantly with respect to heavier proteinuria (median 3.6 g/day, range 0.2-18 g/day), lower serum albumin level (< 40 g/l) and more severe renal histopathological involvement in the treated group (P < 0.01). Oral prednisolone 40 mg/day and azathioprine 2 mg/kg BW/day was commenced initially and after gradual tapering was continued at low dose (5 mg/day) for a median duration of 24 months (range 12-98). The median duration of follow-up was 46 months (range 12-180). The clinical course was defined as progressive or non-progressive on the basis of serial serum creatinine (Scr). Of the patients who presented with renal impairment (Scr > 110 mumol/l), a non-progressive course was observed in 79.5% patients of the treated group (n = 39), while only in 36% of the untreated group (n = 22), the difference was statistically significant (P < 0.001). Slopes of reciprocal of Scr versus time were also calculated by linear regression analysis to represent the trend of renal function for patients who had had 3 or more years follow-up (n = 101). An analysis of variance of these trends in patients with renal impairment at presentation (n = 51) showed significant recovery of renal function in the treated group (n = 33) and a decline of renal function in the untreated group (n = 18, P = 0.004). There was no significant effect of the treatment on proteinuria. The histopathological features that favoured response to the treatment were mesangial proliferation, capsular adhesions and interstitial infiltration on light-microscopy, C3 and fibrin deposits on immunofluorescence (P < 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

The relationship between donor age and the growth characteristics of human smooth muscle cultures of aorta and stomach.

The relationship between donor age and the growth characteristics of normal human smooth muscle cell cultures derived from aorta and stomach was examined in a series of growth studies. A negative correlation was demonstrated between donor age and growth potential of both cell types in primary culture. This decreased growth potential of cultures derived from older donors was maintained in later passage cultures. The results suggest that the culture of human aortic and gastric smooth muscle cells from older donors is more difficult and that any cell cultures established may be limited by poor growth characteristics.

Immunohistochemistry and lectin histochemistry in sarcomatoid renal cell carcinoma: a comparison with classical renal cell carcinoma.

We investigated the expression of various cell markers in renal cell carcinoma, concentrating particularly on the sarcomatoid variety, using lectin and immunohistochemical techniques. The sarcomatoid variant showed stronger staining in a higher proportion of cases for vimentin and reduced positivity for epithelial membrane antigen, in comparison with classical renal cell carcinoma. All sarcomatoid tumours reacted with at least one cytokeratin, enabling them to be distinguished from true renal sarcomas; this is of diagnostic value when a panel of markers is used. Overall a similar pattern of markers is seen in sarcomatoid and classical renal cell carcinoma using lectin and immunohistochemistry, suggesting that the sarcomatoid variant arises as a metaplastic change rather than having a different histogenesis.

Systemic Weber-Christian disease with reversible bilateral ureteric obstruction.

It has been proposed that idiopathic retroperitoneal fibrosis may be a consequence of 'healed' retroperitoneal lesions of systemic Weber-Christian disease. However ureteric obstruction which is the hallmark of idiopathic retroperitoneal fibrosis, has not been described in systemic Weber-Christian disease. We report a patient with systemic Weber-Christian disease who, during a relapse, developed bilateral ureteric obstruction which resolved when the Weber-Christian disease remitted. The radiological appearances were consistent with a diagnosis of idiopathic retroperitoneal fibrosis, but the clinical course was slightly atypical in that the ureteric obstruction resolved completely and rapidly. Ureteric obstruction can complicate systemic Weber-Christian disease and this observation gives support to the hypothesis that idiopathic retroperitoneal fibrosis is related to systemic Weber-Christian disease.

Fatal haemopathological consequences of general anaesthesia.

A previously healthy 63-yr-old female died following an anaphylactoid response to anaesthesia with thiopentone and suxamethonium. Postmortem findings strongly suggested that disseminated intravascular coagulation played a significant role in her death. The local mechanism behind the reaction is unknown, but the formation of thiopentone-suxamethonium colloid aggregates during induction, may have led to "aggregate anaphylaxis".

Rhabdomyosarcoma of the nasopharynx a case with recurrence of tumour after 20 years.

A case of embryonal (botryoid) rhabdomyosarcoma of the nasopharynx originally occurring in a two year old male, with late recurrence in the neck 20 years after treatment by combined surgery and radiotherapy is presented. The histological diagnosis of rhabdomyosarcoma, and the significance of late recurrence are discussed.

Crystalloid material in cells of the murine mononuclear phagocyte system.

In an attempt to throw further light on the nature and distribution of crystalloid material in macrophages, tissues from a variety of locations in 9 normal adult mice of 36-46 g body weight were studied by light and electron microscopy. Crystalloid inclusions were widely distributed in mononuclear phagocytic cells, being present in macrophages of the bone marrow, spleen, lung and ileum and in the Kupffer cells of the liver. They were not observed in the macrophages of the thymus, parotid lymph node or skin nor were they seen within the lymphatic nodules of Peyer's patches. The observed distribution of crystalloid material seems consistent with its suggested origin from granulocyte breakdown.

Von Hippel-Lindau disease in a large British family: clinicopathological features and recommendations for screening and follow-up.

We describe the clinical and pathological features in nine members of a large British family with von Hippel-Lindau disease. Twenty living family members underwent detailed assessment for features of the disease, including retinal examination and computed tomography of the head and abdomen. The hospital records of three deceased family members were reviewed and detailed information about a fourth was obtained from members of the family. As a result of this study, three asymptomatic family members were found to have features of the disease and all three living affected family members were found to have additional features. Lesions discovered included retinal angiomas requiring treatment in four family members, one asymptomatic renal cell carcinoma and one asymptomatic pancreatic endocrine tumour. We also report the occurrence of a spermatic cord mesenchymal hamartoma in von Hippel-Lindau disease.

Glomerulonephritis and IgA deficiency.

A mild self-limiting mesangial proliferative glomerulonephritis is described in 3 patients with selective IgA deficiency (less than 0.05 g/l). In all cases there was irregular thickening of peripheral glomerular capillary loops and paramesangial deposits. Arteriolar hyalinosis was present in two of the three cases. By direct immunofluorescence, and immunoperoxidase techniques in one case, IgM was present in the glomeruli and C3 was present in blood vessel walls. Secretory component was not detected in the glomeruli of any of the patients. The polyethylene glycol (PEG) precipitation immune complex assay was positive for IgM and IgG immune complexes in one patient and weakly positive for IgM immune complexes alone in another. The serum of one patient contained milk precipitins. All three patients had suffered from recurrent upper respiratory tract infections. It is suggested that the glomerular IgM immune complex deposition is related to the IgA deficiency and that, although the immune complexes may be a consequence of antibodies to dietary bovine proteins, this could be due to recurrent upper respiratory tract infections. This type of glomerulonephritis appears to resemble IgM associated glomerulonephritis in some aspects. Careful appraisal is required to establish if this association constitute a specific entity.