MR Brain Screening in ADPKD Patients : To Screen or not to Screen?

BACKGROUND: Adult polycystic kidney disease (ADPKD) still represents a major cause of renal failure and intracranial aneurisms (IA) have a higher prevalence in ADPKD than in the general population. Current guidelines suggest performing brain MRI only in the subjects with a positive familiar history of IAs or subarachnoid hemorrhage (SAH). This is a retrospective case-control analysis to evaluate the usefulness of a MR screening program in ADPKD patients. METHODS: We retrospectively analyzed all ADPKD patients followed in our outpatient clinic between 2016 and 2019 who underwent a brain MRI screening. We evaluated the presence of IAs and others brain abnormalities and compared our results with a non-ADPKD population (n = 300). We performed univariate and multivariate regression analysis to evaluate if general and demographic features, laboratory findings, clinical parameters and genetic test results correlated with IAs or other brain abnormalities presence. RESULTS: Among the patients evaluated 17 out of 156 (13.6%) ADPKD patients had IAs, compared to 16 out of 300 (5.3%) non-ADPKD controls (p < 0.005). Considering ADPKD patients presenting IAs, 12 (70.6%) had no family history for IAs or SAH. Genetic analysis was available for 97 patients: in the sub-population with IAs, 13 (76.5%) presented a PKD1 mutation and none a PKD2 mutation. We found that arachnoid cysts (AC) (p < 0.001) and arterial anatomical variants (p < 0.04) were significantly more frequent in ADPKD patients. CONCLUSION: In our population ADPKD patients showed a higher prevalence of IAs, AC and arterial variants compared to non-ADPKD. Most of the IAs were found in patients presenting a PKD1 mutation. We found a significant number of alterations even in those patients without a family history of IAs or SAH. The practice of submitting only patients with familial IAs or kidney transplantation candidates to MRI scan should be re-evaluated.

An integrated gait rehabilitation training based on Functional Electrical Stimulation cycling and overground robotic exoskeleton in complete spinal cord injury patients: Preliminary results.

The aim of this study is to investigate the effects of an integrated gait rehabilitation training based on Functional Electrical Stimulation (FES)-cycling and overground robotic exoskeleton in a group of seven complete spinal cord injury patients on spasticity and patient-robot interaction. They underwent a robot-assisted rehabilitation training based on two phases: n=20 sessions of FES-cycling followed by n= 20 sessions of robot-assisted gait training based on an overground robotic exoskeleton. The following clinical outcome measures were used: Modified Ashworth Scale (MAS), Numerical Rating Scale (NRS) on spasticity, Penn Spasm Frequency Scale (PSFS), Spinal Cord Independence Measure Scale (SCIM), NRS on pain and International Spinal Cord Injury Pain Data Set (ISCI). Clinical outcome measures were assessed before (T0) after (T1) the FES-cycling training and after (T2) the powered overground gait training. The ability to walk when using exoskeleton was assessed by means of 10 Meter Walk Test (10MWT), 6 Minute Walk Test (6MWT), Timed Up and Go test (TUG), standing time, walking time and number of steps. Statistically significant changes were found on the MAS score, NRS-spasticity, 6MWT, TUG, standing time and number of steps. The preliminary results of this study show that an integrated gait rehabilitation training based on FES-cycling and overground robotic exoskeleton in complete SCI patients can provide a significant reduction of spasticity and improvements in terms of patient-robot interaction.

[Non-insulin-dependent diabetes mellitus associated with nonalcoholic liver cirrhosis: an evaluation of treatment with the intestinal alpha-glucosidase inhibitor acarbose]. / Diabete mellito non insulino-dipendente associato a cirrosi epatica non etilica: valutazione del trattamento con un inibitore delle alpha-glucosidasi intestinali, acarbose.

Non-insulin-dependent diabetes mellitus not responding to diet only in patients with non-alcoholic liver cirrhosis is characterized by high post-prandial hyperglycemia. The aim of this study was to evaluate the safety and efficacy of 24 weeks of treatment with 300 mg acarbose per day in 76 consecutive outpatients affected by type 2 diabetes and well-compensated liver cirrhosis. The study design was double-blind cross-over vs placebo. All patients tolerated both treatments well, and no significant variations in liver function tests were observed (< 5% vs pre-treatment). A significant reduction of several parameters was observed only after acarbose: fasting glycemia (19 +/- 6 vs 2 +/- 0.5%; p < 0.01), post-prandial glycemia (41 +/- 9 vs 3 +/- 0.6%; p < 0.01), mean glycemia (30 +/- 8 vs 14 +/- 5%; p < 0.01), daily glycemic variation (52 +/- 8 vs 8 +/- 1%; p < 0.01), HbA1c (16 +/- 1 vs 2 +/- 0.5; p < 0.05), incremental area of C-peptide after a standard meal (80 +/- 19 vs 200 +/- 36 ng/mL/300 min; p < 0.01). After acarbose a significant increase of intestinal voiding/week (98 vs 28%; p < 0.01) and a parallel reduction of blood ammonia levels (52 +/- 9 vs 9 +/- 5%; p < 0.01) were observed. Results clearly document the good tolerability and the absence of toxic effects of acarbose on the liver, due to a theoretic absence of both absorption by the gut and hepatic metabolism of the drug. In fact, acarbose increases peristaltic movement of the gut, stimulates the proliferation of saccharolytic bacteria and simultaneously reduces proteolytic bacterial proliferation, thus actively reducing blood ammonia levels. These unexpected effects of acarbose may be used to advantage for the treatment of type 2 diabetes mellitus in patients with well-compensated liver cirrhosis.

Blood volume modeling and refilling rate estimation in hemodialysis by continuous hemoglobin monitoring.

Eleven bicarbonate hemodialyses (HD) of 6 patients under constant ultrafiltration were continuously monitored with an optical Hb-meter, considered to be a marker of blood volume (BV) changes. A theoretical model was fed experimental data for prediction of blood volume and estimation of vascular parameters, and a time course of rate of refilling was extrapolated. The adequacy of the model was very good for the time course of BV prediction (r2 = 0.85-0.95, n = 11) and for plasma protein concentration (r2 = 0.83-0.86, n = 2). Parameters estimated included (mean-DS): filtration coefficient (Cf) = 0.22 (0.16) dl/min*mmHg, transcapillary hydrostatic pressure (DP) = 17.80 (3.44) mmHg and protein concentration of the refilling fluid (Cref) = 0.45 (0.30) g/dl. In conclusion our study has shown that the model chosen fits the observed BV profile well in all cases, thus the Hb data series can be used for BV dynamic modeling and for estimation of vascular parameters.

Molecular mapping and detoxification of the lipid A binding site by synthetic peptides.

Endotoxin [lipopolysaccharide (LPS)], the major antigen of the outer membrane of Gram-negative bacteria, consists of a variable-size carbohydrate chain that is covalently linked to N,O-acylated beta-1,6-D-glucosamine disaccharide 1,4'-bisphosphate (lipid A). The toxic activity of LPS resides in the lipid A structure. The structural features of synthetic peptides that bind to lipid A with high affinity, detoxify LPS in vitro, and prevent LPS-induced cytokine release and lethality in vivo were defined. The binding thermodynamics were comparable to that of an antigen-antibody reaction. Such synthetic peptides may provide a strategy for prophylaxis and treatment of LPS-mediated diseases.

[Which moments in the respiratory cycle have the most influence on right ventricular dynamics?]. / Quali momenti del ciclo respiratorio influiscono maggiormente sulla dinamica del ventricolo destro?

The performance of the right heart during respiratory activity has mostly been studied in terms of changes in flow and pressure in pulmonary circulation. The aim of this study was to identify which moments of the respiratory cycle exert the greatest influence on right ventricular dynamics. Thus, the behaviour of the right ventricular systolic time intervals and pulmonary artery pressures, expressed both as intravascular (Piv) and transmural (Ptm), were investigated to this end. Investigations were carried out on 10 anesthetized spontaneously breathing beagle dogs using high-fidelity pressure transducers (MPC 500, Millar Instr.) and by making use of a computerized system of signal recording and analysis. Changes in right ventricular systolic time intervals were evident during transition from inspiration to expiration and at the beginning of expiration. In fact, compared to spontaneous post-expiratory pause values, the so-called Rapid Ejection and Slow Ejection Phases, and therefore the Total Ejection Period, were significantly shortened (p less than 0.01 for both) only at early expiration, whereas during the same phase of the respiratory cycle the Isovolumetric Contraction Time and the total Pre-ejection Period were significantly prolonged (p less than 0.01 for both). During the transition from inspiration to expiration, the right ventricular systolic "plateau" very often presented an ascending slant, i.e., reaching maximum pressure in late instead of early systole, as usually observed in the other moments of the respiratory cycle.(ABSTRACT TRUNCATED AT 250 WORDS)

[Torsade de pointes caused by tricyclic antidepressive agents. Description of a clinical case]. / "Torsione di punta" da antidepressivi triciclici. Descrizione di un caso clinico.

Tricyclic antidepressant drugs are known to cause often electrocardiographic abnormalities and to induce sometimes cardiac rhythm disturbances. We report a case of a patient on antidepressant therapy (Desipramine Hydrochloride, 50 mg/die, and Dothiepin Hydrochloride, 150 mg/die), without any underlaying heart disease, admitted to our Coronary Care Unit for recurrent syncopal episodes. An ECG on admission showed Sinus Tachycardia with Ectopic Ventricular Beats and recurrent runs of Torsade de Pointes, a distinctive form of Ventricular Tachycardia. Lignocaine i.v. was only transiently effective. Both Isoprenaline and Atropine Sulphate i.v. were uneffective. Ventricular Fibrillation occurred and cardioversion was achieved by a single DC shock. Amiodarone i.v. and electrical overdrive only temporarily suppressed ventricular arrhythmias. Magnesium Sulphate i.v. (bolus + infusion) induced a definitive suppression of Torsades de Pointes. One day later no more arrhythmias were present.