[Caroli's disease: a clinical case].
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Biomedical subjects
Publications and source records attributed to M Corti.
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Static and dynamic laser light scattering measurements on micellar aqueous solutions of gangliosides GM2, GM1, GD1a are reported. The aggregation number, the hydrodynamic radius and the micellar shape depend on the type of ganglioside and the unsaturation degree of the hydrocarbon chains. At a temperature of 25 degrees C the molecular weights of GM2, GM1 and GD1a are 740,000, 470,000 and 418,000 DA respectively. A significant decrease of micellar size with temperature has been found for saturated GM1 in the region 25 degrees-40 degrees C. The strong sensitivity of the micellar parameters to the ganglioside structure is explained by making reference to some simple model which takes into account geometrical packing considerations. By measuring the scattered light intensity at low ionic strength of the solution (0.1-30 mE) it was possible to evaluate the ganglioside micellar charge, 100 electronic units for GM2, 48 for GM1 and 60 for GD1a.
Data are few and conflicting about the prevalence and risk factors for antiphospholipid (aPL) antibodies in end-stage renal disease (ESRD). We studied the prevalence, risk factors and clinical manifestations of lupus anticoagulant (LA) and anticardiolipin antibodies (aCL) among ESRD patients (chronic hemodialysis (HD) patients and kidney transplant recipients) and blood donors. LA was assessed in a large cohort (n=180) of patients by the activated partial thromboplastin time (aPTT), dilute Russel's viper venom test (dRVVT) and lupus anticoagulant-sensitive aPTT reagent (PTT-LA). IgM- and IgG-aCL were measured by a solid-phase enzyme-linked immunosorbent assay (ELISA) in 111 patients (61.5%). The prevalence of aPL was low but, it was higher in ESRD than blood donors (8.8% (16/180) vs. 0%, P=0.005); the frequency of aCL was also higher in ESRD than controls (10.8% (12/111) vs. 0%, P=0.002). LA was similar in the study and control groups (2.2% (4/180) vs. 0%, NS). Among HD patients and kidney allograft recipients there was no difference in LA (3.9% (4/101) vs. 0% (0/79), NS) and aCL frequency (18.6% (8/43) vs. 5.9% (4/68), NS). aPL was not associated with sex, age, time on HD, post-transplantation follow-up, ESRD etiology, thrombotic or hemorrhagic events, or type of HD membrane; however, these findings must be interpreted with caution, given the low frequency of aPL. In one HD patient LA activity was associated with multiple thrombosis of the access graft and native veins. In summary, the prevalence of aPL in ESRD is low but nevertheless higher than controls; LA does not appear to be related to membrane bio-incompatibility and it may be linked to vascular thrombosis; the lack of concordance between LA and aCL was apparent. Further studies are needed to clarify the issue of aPL in ESRD. LA testing should be incorporated into the diagnostic evaluation of recurrent thrombotic episodes in patients on HD.
Patients with end-stage renal disease (ESRD) appear to be at high risk for GBV-C/HGV infection. This information has been obtained with virological techniques (RT-PCR) but few serological data exist. A prototype enzyme immunoassay has now been developed to detect antibodies against the putative envelope protein (E2) located on the surface of the GBV-C/HGV virion particle. We studied the prevalence of GBV-C/HGV infection, as detected by RT-PCR and anti-E2 GBV-C/HGV antibody, in a cohort of chronic dialysis patients (n=157) and renal transplant (RT) recipients (n=77); as a control group, 136 healthy blood donors were tested. The total prevalence of GBV-C/HGV in ESRD was 23% (54/234). The frequency of GBV-C/HGV viremia was 7.7% (18/234) in ESRD and 4.4% (3/68) among healthy blood donors; the prevalence of anti-E2 GBV-C/HGV was 15% (36/234) and 8.8% (12/136) in ESRD and controls, respectively. No relationship was seen between anti-E2 GBV-C/HGV antibody (or GBV-C/HGV viremia) and age, sex, time on renal replacement therapy, anti-HCV, HBsAg and transfusion requirement. No statistical association was observed between GBV-C/HGV and AST/ALT activity. Two of 54 GBV-C/HGV positive patients (3.7%) had raised ALT but were negative for HBV/HCV. In the majority of patients (35/36, 97%) the presence of anti-E2 GBV-C/HGV antibody was linked with the loss of GBV-C/HGV viremia from serum. In conclusion, GBV-C/HGV infection, as detected by RT-PCR and anti-E2 antibody, was common in ESRD, and the rate of infection was higher than in controls. No association was seen between GBV-C/HGV and various demographic or clinical factors. A small group of GBV-G/HGV positive patients tested negative for HBV/HCV and had raised ALT. In many patients exposed to GBV-C/HGV infection the virus was cleared. The clinical significance of GBV-C/HGV in ESRD remains controversial. Prospective studies with additional serological assays are in progress.
BACKGROUND: Rhabdomyolysis is known as one of the possible causes of acute renal failure and can be triggered by different situations. In recent years, Parkinson's disease emerged as a condition that can be complicated by the development of rhabdomyolysis and consequently, in some cases, of acute renal failure. We report two cases of rhabdomyolysis, one of which complicated by an oligo-anuric renal failure, which occurred in patients affected by Parkinson's disease and admitted to our Division. METHODS AND RESULTS: The first case occurred in a 90-year-old Parkinsonian woman, under treatment with Levodopa-Benserazide and Bornaprine. She developed rhabdomyolysis (CPK 1746 U/L with MB isoenzyme 3.5 ng/ml, LDH 610 U/L, GOT 78 U/L) after she had been found lying on the floor, in a state of mental confusion, after 24 hours in which her relatives had not heard from her. During the first two days of hospitalization, the patient also had a fever (axillary temperature between 37 degrees C and 38 degrees C), accompanied by mild leukocytosis (WBC 13000/mm3) on entrance. The second case occurred in a 78-year-old Parkinsonian woman, under treatment with Levodopa-Carbidopa, Levodopa-Benserazide and Pramipexol. She developed a severe rhabdomyolysis (CPK 34800 U/L with MB isoenzyme 771 ng/ml, LDH 2133 U/L, GOT 785 U/L) complicated by acute renal failure with anuria, after two days characterized by several episodes of vomit and diarrhea. In the following days we learned that in the last 20 days before admission to hospital the patient had increased the dose of her anti-Parkinson therapy and was almost always disturbed by severe choreiform and dystonic movements. CONCLUSIONS: Regarding the first case, the clinical conditions in which the patient was found, the simultaneous presence of fever and leukocytosis and the absence of any reasonable explanation for a hypothetical fall to the floor induced us to think that this was a case of rhabdomyolysis in the context of an akinetic hyperthermic crisis. This is a syndrome that can develop in Parkinsonian patients after discontinuance or simply reduction of therapy with dopaminergic agents and is characterized also by rhabdomyolysis. The fact that the patient was living alone would confirm this hypothesis. On the other hand, the second case of rhabdomyolysis must probably be related to the severe choreiform and dystonic movements that the patient developed in the days just prior to admission. These movements were surely caused by the increase of the substitutive therapy with Levodopa by the patient herself. In conclusion, these two clinical cases, together with others previously reported by other Authors, show how the circumstances leading to the development of acute episodes of rhabdomyolysis in Parkinson's disease can be different and, at the same time, how these circumstances are always somehow related to the treatment of the basic disease with dopaminergic drugs.
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