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Membranoproliferative GN (MPGN) was recently reclassified as alternative pathway complement–mediated C3 glomerulopathy (C3G) and immune complex–mediated membranoproliferative GN (IC-MPGN). However, genetic and acquired alternative pathway abnormalities are also observed in IC-MPGN. Here, we explored the presence of distinct disease entities characterized by specific pathophysiologic mechanisms. We performed unsupervised hierarchical clustering, a data-driven statistical approach, on histologic, genetic, and clinical data and data regarding serum/plasma complement parameters from 173 patients with C3G/IC-MPGN. This approach divided patients into four clusters, indicating the existence of four different pathogenetic patterns. Specifically, this analysis separated patients with fluid-phase complement activation (clusters 1–3) who had low serum C3 levels and a high prevalence of genetic and acquired alternative pathway abnormalities from patients with solid-phase complement activation (cluster 4) who had normal or mildly altered serum C3, late disease onset, and poor renal survival. In patients with fluid-phase complement activation, those in clusters 1 and 2 had massive activation of the alternative pathway, including activation of the terminal pathway, and the highest prevalence of subendothelial deposits, but those in cluster 2 had additional activation of the classic pathway and the highest prevalence of nephrotic syndrome at disease onset. Patients in cluster 3 had prevalent activation of C3 convertase and highly electron-dense intramembranous deposits. In addition, we provide a simple algorithm to assign patients with C3G/IC-MPGN to specific clusters. These distinct clusters may facilitate clarification of disease etiology, improve risk assessment for ESRD, and pave the way for personalized treatment.

In the medical field, the attention to the environmental impact of industrial processes and products is still limited. In recent years there has been an increased sensitivity towards the environment; meanwhile, the economic burden of hazardous waste disposal is becoming evident. Dialysis is a "big producer" of waste and it has been estimated that disposal costs can be up to 10-40% of the cost of disposables. So there are several reasons of interest on "ecodialysis": the high amount of waste defined as "potentially hazardous", which requires a very expensive management and the recyclability potential of the non-contaminated waste, that has not yet been fully explored in dialysis. This primary study has been performed in collaboration with the Politecnico di Torino. Its aim has been to define a schedule of activities by a few brainstorming sessions. This schedule is to be readily performed or it should be developed in detail to optimize, by reducing and recycling, the waste production during the dialysis session. The discussion identified seven basic points for the eco-sustainability of haemodialysis to: [1] reduce packaging; [2] facilitate separation of materials, and [3] their discharge; [4] differentiate materials; [5] clearly highlight the potentially hazardous materials; [6] improve the recyclability of plastic products; [7] propose a path of recovery and reuse. Although a full optimization requires a close cooperation with the manufacturers and is achievable only in the long term, the reduction of one pound of potentially contaminated materials could presently lead, on a national scale, to a saving of several million euros, which can be better employed in investments to improve our treatments.

We evaluated the rate of seroclearance of the hepatitis B surface antigen (HBsAg) and its clinical significance in patients with chronic hepatitis delta virus (HDV).Antibody to HDV was tested in HBsAg-positive subjects admitted to our Hospital from 1991 to 1995. In 1997, a biochemical and virologic study was performed in the surviving anti-HD-positive patients who had not undergone transplantation. As a control, a cohort of 106 HBsAg-positive, anti-HD-negative patients was studied.One hundred and forty-one subjects were originally positive for anti-HD. After 4 years of follow-up, six of the 60 patients who underwent re-evaluation (10%) had cleared the HBsAg: three of the six patients had minimal changes at the initial liver histology and normal ALT, whereas in the remaining three patients with chronic active hepatitis ALT normalized during the observation. Anti-HD persisted in five of the six patients. Only one patient had raised anti-HBs. In contrast, three of 106 HBsAg carriers without HDV infection (2.8%) cleared the HBsAg within the same time and seroconverted to anti-HBs (p=0.002).HBsAg clearance is increased over the years in HDV patients compared to ordinary HBsAg carriers, and is often associated with improvement of HDV disease without seroconversion to anti-HBs.

We describe a patient with Philadelphia-chromosome-positive (Ph' +) chronic myelogenous leukemia (CML), who developed an anaplastic large cell lymphoma (ALCL) with T-phenotype, after 43 months successful treatment with alpha-interferon (IFN). Characterization studies of lymphoma cells showed positivity for Ki-1 monoclonal antibody, T-cell surface markers, T-cell receptor beta chain rearrangement, and germline configuration of the BCR gene. At the time of lymphoma diagnosis, the patient had achieved complete hematologic remission from CML with partial karyotypic conversion (50% Ph' + cells). After twelve weekly courses of polychemotherapy, he obtained complete remission from lymphoma. At present, five years from CML diagnosis, the patient has a remarkably stable disease, being in remission from lymphoma and in well controlled CML chronic phase. Our case thus represents the first well documented description of a T-cell non-Hodgkin's lymphoma developed during the course of CML.

A case of warm autoimmune hemolytic anemia due to IgA antibody is described. The patient had clinical and hematologic signs of hyperhemolysis, but all specific tests were negative. The direct antiglobulin test was positive only when it was performed with anti-IgA monospecific antiserum. The autoantibody eluted from the patient's red cells showed anti-e specificity. The sensitivity of broad-spectrum antiglobulin serum and the possible hemolytic mechanisms of IgA-coated red cells are discussed.

Injuries of the axillary nerve subsequent to recurrence of glenohumeral dislocation have received only minimal attention. It is the purpose of this study to define the prevalence and the progression in time of injury of the axillary nerve in patients with recurrence of anterior shoulder dislocation. For two years we observed a total of 185 patients who had had primary shoulder dislocation. Excluded from the study were patients who had fractures associated with metabolic disorders that favored neurologic deficit. During the period of study, 98 patients contacted us again after recurrence of the dislocation: there were 89 patients aged over 60 years and 9 aged below 60 years. All of the patients were evaluated clinically and submitted to EMG in order to verify the condition of the axillary nerve. Four patients (4%) had neuroapraxia of the axillary nerve. One of these also had neuroapraxia of the radial nerve. Of the four patients, one was a male aged 34 years; the others were all aged over 60 years. In all of the cases, function of the axillary nerve completely recovered after a mean period of 4 months (3-5.3 months) after recurrence. Injury of the axillary nerve can occur at the time of the first recurrence of the injury. However, prevalence is significantly lower than that observed after primary dislocation. The occurrence of this injury should be taken into consideration, particularly in elderly patients, in order to avoid erroneous clinical diagnosis and massive rupture of the cuff subsequent to recurrence of the dislocation.

Background Symptoms of irritable bowel syndrome (IBS) have been associated to altered colonic motility and sensation. Smoking affects pain perception and is a risk factor in the development of post-infectious IBS, but its effect on abdominal pain and colonic transit remains to be elucidated in IBS. Methods Forty patients with IBS-C and 28 with IBS-M were selected based on Rome IV criteria. Colonic transit time was studied and smoking habit was recorded. Presence of mild or severe abdominal pain and the prevalent pain characteristics (diffuse or localized, chronic or acute, with cramps or gradually distending) were recorded. Data were analyzed by univariate and stepwise multiple logistic regression analysis to verify the risk association between pain and all other variables. Results IBS-C patients had a longer transit time in the right colon and scored more chronic pain than IBS-M patients. When severity of abdominal pain was used as discriminating factor, a significant number of subjects reporting severe pain were males and smokers (16/30 vs. 4/38 and 20/30 vs. 4/38, both ƿ Conclusions Smoking was the only factor independently associated with severe abdominal pain. As smoking does not seem to affect colonic transit time, we suggest that smoking may influence visceral perception and symptoms severity in IBS patients.

Lipofuscin-like granules, first described by Biava and West in 1965, are a subcellular, quasi-physiologic finding mainly seen in the smooth muscle cells of renal arterioles, but also in juxtaglomerular cells and the lacis cells of human kidneys. They increase in number in subjects affected by arterial hypertension and diabetes. They do not correlate with a specific primary renal disease. Lipofuscin-like granules are not related to renin granules. The world literature on this subject is almost non-existent, and the awareness of this finding or its clinical significance among either pathologists or nephrologists is very poor. We incidentally observed these lipofuscin-like granules in 8 cases during the routine electron microscope examination of 440 renal biopsies, and report herein on their ultrastructural features. Six of these 8 patients were affected by arterial hypertension, one of whom was also concomitantly affected by diabetes mellitus. These lipofuscin-like granules appear as dense bodies with a lipid component, a coarsely granular matrix, and a crystalloid component which may appear in a band or dot pattern, according to the plane of sectioning. The pathologist has to be aware of these lipofuscin-like granules in order not to confuse them with the semicircularly organized (fingerprint) linear immune deposits associated with some specific glomerulopathies.

Clinical criteria for the diagnosis of Prader-Willi Syndrome (PWS) were established by consensus in 1993 (Holm et al.). Specific molecular testing is now available and the purpose of diagnostic criteria has shifted to identify individuals to test, thus avoiding the expense of unnecessary analysis. The aim of this study was to find clinical indicators to select patients with suspected PWS for laboratory testing. We analyzed the prevalence of clinical signs and symptoms in 147 genetically diagnosed Italian patients with PWS (67 males and 80 females), aged from 9 months to 34.6 years (13.6 +/- 8.3 years), using the consensus diagnostic criteria, and according to age, sex and type of genetic abnormality. The prevalence of several clinical features changed significantly with age, but very few with sex. According to genetic subtypes (deletion vs UPD), only hypopigmentation and acromicria were more frequent in patients with deletion. Some criteria considered as minor or supportive by Holm et al. have higher prevalence than some major criteria. In conclusion, in order to identify patients with suspected PWS to submit to laboratory testing, we recommend a classification of clinical criteria according to age, giving more attention to those so-called minor or supportive criteria.