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Biomedical subjects

M Meroni

Publications and source records attributed to M Meroni.

At least 19 recordsLinked to original sources

Performance determinants and flexible ICU organisation.

We faced some of the most important aspects of the problem of the appropriateness of ICU resources use, that are the relationship between volume of activity and mortality, the analysis of cost-effectiveness in intensive care medicine, and the monitoring of the human resource use in ICU. For this aim three different surveys were utilized: one at European level, the second at country level and, third, a regional survey. After developing a new measure of volume called ''high-risk volume'', we explored the relationship between outcome and volume, founding that such association was very strong (from 3 to 1719% decrease in ICU/hospital mortality every five extra high-risk patients treated per bed per year), and that an occupancy rate larger than 80% was associated with higher mortality. Therefore, patients in all levels of risk are better treated in high-risk volume ICUs with a reasonable occupancy rate. Analysing cost-effectiveness in intensive care medicine using a national case-mix categorized in different diagnostic groups, we identified brain haemorrhage, ALI/ARDS and surgical unscheduled patients as users a high volume of monetary resources less efficiently, while the scheduled abdominal surgery patients admitted to receive intensive care and patients on the ICU for minor organ support made the best use of the fewer resources spent. Finally, we designed a new approach to measure the rate and appropriateness of nursing resource use in ICU on a daily basis. Testing this approach on a group of general non-specialist ICUs, we found that the method was powerful enough to adequately distinguish between ''over'' and ''under-utilization'' and to identify all the theoretical scenarios of nurse/resource utilization.

Cost-Benefit Analysis↗

[Use of protein C concentrate in adult patients with severe sepsis and septic shock].

AIM: The aim of this study is to describe the first experiences on the use of protein C concentrate (PC) in adult patients with severe sepsis and septic shock and clinical contraindications to activated protein C (APC). On the basis of the effectiveness demonstrated by the activated form in sepsis and of the encouraging results expressed in literature of protein C concentrate (PC) mainly about meningococcus fulminating infections, we carried out an observational study on protein C concentrate (PC) with 28-day follow-up and a daily analysis of the hemato-chemical and clinical parameters. Particular attention was paid to the variations in the PC plasma levels, to the modifications of the coagulation system, to the SOFA score as well as to the safety under bleeding risk conditions. METHODS: The study included 7 patients (5 females and 2 males) either with severe sepsis (2). or septic shock (5); one of them had DIC, with PC plasma levels less than 50%. APC could not be administered because of clinical reasons. Patients' mean age was 60.5 years (43-78), the average SAPS II 52.2 (36-72), the pathologies leading to sepsis were lung infections (3). and peritonitis (4). The average time elapsed between the onset of the organ failure and the beginning of treatment with PC was 27.7 hours (12-42). RESULTS: Mortality on day 28 was 42.8% (3 deaths), in all patients the PC plasma levels were brought again to the physiological values. Among the biochemical parameters recorded during the PC infusion, was observed in particular a significant decrease of PDFs, a general rise of the platelet count, and a reduction of the lactic acid levels. No adverse reaction or bleeding complication were seen, even if most of the patients' coagulation was altered or at risk due to neurological problems or repeated surgery. CONCLUSION: In our small number of patients, protein C concentrate has proven to be safe and particularly useful in the control of the coagulopathy triggered and sustained by sepsis.

Adult↗

Evolution of renal pathology in Fabry disease.

UNLABELLED: Fabry disease is a rare lysosomal storage disorder which results from deficient activity of the enzyme alpha-galactosidase A. The resultant deposition and progressive accumulation of glycosphingolipids in all types of body tissue leads to severe clinical manifestations involving the heart, CNS and kidney. Renal manifestations are observed relatively early in the course of the disease, and progression to end-stage renal failure is common in hemizygous males in the third to fifth decades of life. Renal biopsy specimens reveal evidence of diffuse intracytoplasmic glycosphingolipid accumulation, mainly affecting podocytes and epithelial cells of distal tubules, which are strikingly enlarged and vacuolated. On electron microscopy the deposits appear as typical osmiophilic inclusion bodies in the cytoplasm of all kinds of renal cells, and show a characteristic 'onion skin' or 'zebra' appearance. These pathological features are also evident in heterozygous females. Deposits occur before the development of renal impairment. As patients age, the disease progresses in cells throughout the kidney, and is associated with increasing glycosphingolipid accumulation. CONCLUSION: The age-related evolution of renal pathology in Fabry disease is closely correlated with progressive intracellular deposition of glycosphingolipid and ultimately leads to end-stage renal failure.

Disease Progression↗

Renal pathological changes in Fabry disease.

Fabry disease is a rare X-linked disorder, characterized by deficient activity of the lysosomal enzyme alpha-galactosidase A. This leads to systemic accumulation of the glycosphingolipid globotriaosylceramide (Gb3) in all body tissues and organs, including the kidney. Renal manifestations are less evident in female heterozygotes than in male hemizygotes, according to the Lyon hypothesis. Accumulation of Gb3 occurs mainly in the epithelial cells of Henle's loop and distal tubule, inducing early impairment in renal concentrating ability; involvement of the proximal tubule induces Fanconi syndrome. All types of glomerular cells are involved, especially podocytes, and glomerular proteinuria may occur at a young age. The evolution of renal Fabry disease is characterized by progressive deterioration of renal function to end-stage renal failure (ESRF). Ultrastructural study of kidney biopsies reveals typical bodies in the cytoplasm of all types of renal cells, characterized by concentric lamellation of clear and dark layers with a periodicity of 35-50 A. Management of progressive renal disease requires dietetic and therapeutic strategies, usually indicated in developing chronic renal failure, with dialysis and renal transplantation required for patients with ESRF. The recent development of enzyme replacement therapy, however, should make it possible to prevent or reverse the progressive renal dysfunction associated with Fabry disease.

Disease Progression↗

Hypocomplementemic type II membranoproliferative glomerulonephritis in a male patient with familial lecithin-cholesterol acyltransferase deficiency due to two different allelic mutations.

Patients with familial lecithin-cholesterol acyltransferase (LCAT) deficiency very often show progressive glomerulosclerosis with evolution to end-stage disease. High levels of an abnormal lipoprotein (lipoprotein X) cause glomerular capillary endothelial damage. The ultrastructural study of renal biopsy specimens shows characteristic glomerular deposits of membrane-like, cross-striated structures and vacuole structures. The gene encoding for LCAT has been mapped to chromosome 16q22.1, and several mutations of this gene cause LCAT deficiency which is inherited as an autosomal recessive trait and which is characterized by corneal opacities, normochromic normocytic anemia, and renal dysfunction. Herein we report clinical features and renal histological findings concerning a 24-year-old male patient with classical familial LCAT deficiency due to two different allelic mutations: a nonsense mutation inherited from the father and a missense mutation inherited from the mother. Moreover, the patient showed glomerular histological lesions and an immunofluorescent glomerular pattern typical of hypocomplementemic membranoproliferative type II glomerulonephritis (dense-deposit disease). The nature of electron-dense material that characterizes dense-deposit disease is still unknown, but there are suggestions that some chemical modifications might occur in the renal basement membranes. Therefore, this clinical case might induce to consider possible relations between disorders of the lipoprotein metabolism and renal dense-deposit disease.

Adult↗

[QTc interval in newborns with gastroesophageal reflux treated with cisapride and ranitidine].

Gastroesophageal reflux (GER) is very common in infants, especially in prematures and may be cause of gastrointestinal and cardiorespiratory symptoms. Cisapride, a prokinetic agent, is used in order to avoid the transient esophageal sphincter relaxation, but it is sometimes associated to transient prolongation of QT interval on EKG, especially with high dosage. The authors report the effects of cisapride therapy (0.8 mg/Kg/day) on QTc interval (QTc = QT interval corrected on heart frequency) in a pediatric population (50 infants) with GER. Results demonstrate the relatively safety of cisapride therapy at low dose also in the pediatric period.

Cisapride↗

Nosocomial outbreak of Aspergillus fumigatus infection among patients in a renal unit?

Aspergillus fumigatus is present in the environment worldwide and it is only able to infect debilitated or immunodepressed subjects. Nosocomial outbreaks of A. fumigatus infection have been associated with hospital reconstruction. Spores are released into the environment and are inhaled by immunodepressed patients housed in nearby Medical Units. Specific clinical syndromes are allergic bronchopulmonary aspergillosis and invasive pulmonary aspergillosis with characteristic radiological features. Invasive A. fumigatus infection is commonly fatal, even if promptly diagnosed and treated. Three consecutive cases of A. fumigatus infection occurred in debilitated patients housed in our Renal Unit while building renovation near the Unit was being performed. Two of these patients died and pulmonary and diffuse aspergillosis was found on postmortem examination. The third patient, highly suspected to be infected with Aspergillus, was aggressively and successfully treated with liposomal amphotericin B. Our experience suggests that fungal infections have gained increasing prominence in clinical medicine and they must be considered in chronic debilitated patients including dialysis patients, and that liposomal amphotericin B represents an important advance in the treatment of aspergillosis.

Aged↗