Severe itching in the patient with burns.

After discharge, the patient with burns is often plagued with persistent, unrelenting itching. The purpose of this study was to document the incidence and severity of itching in discharged patients with burns, to determine factors that may help in prediction of the problem, and to study the effectiveness of Benadryl (Parke-Davis, Morris Plains N.J.), Atarax (Roerig Div. of Pfizer Inc., New York, N.Y.) and Polyhist Forte (Mikart Inc., Atlanta, Ga.) in relieving the symptom. All adult patients who were discharged to our outpatient clinic were entered into this prospective study. Patients were started on one of the three agents, and agents were changed monthly in a randomized fashion. Severity of itching was determined by a visual linear analogue scale. The study population had a mean age of 35.9 +/- 12.8 years, and a mean burn size of 19.1% +/- 15.3% total body surface area. Eighty-seven percent of discharged patients complained of itching. The average severity of the problem was 7.6 +/- 1.9. A significant difference (p less than 0.05) among groups was found when the population was analyzed for size of burn and duration of time to wound closure. Symptoms varied with anatomic area burned. One hundred percent of patients with leg burns and 70% of those with arm burns complained of itching. Facial burns did not cause itching in any of the patients. The agents that were tested produced complete relief in only 20% of patients, partial relief in 60%, and no relief in 20%. There were no differences in response to the three agents tested. All three of the drugs that were tested had similar onset of action.(ABSTRACT TRUNCATED AT 250 WORDS)     

Sleep abnormalities in traumatic apallic syndrome.

Sleep patterns in 10 patients with traumatic apallic syndrome were studied, together with 10 healthy controls matched for sex and age. All patients underwent neurological examination, brain CT, and polysomnographic recording within six months (mean 99 (SD 45) range 47-180 days) from the onset of symptoms. Clinical follow up was performed six months after enrollment in the study. Sleep patterns were recorded in nine out of 10 patients. In the tenth patient there was no rhythm resembling physiological sleep. This patient was the only one who remained in a persistent vegetative state and died before the six month follow up. The severity of neurological deficit at follow up was significantly related to the duration of coma. There was no significant difference between patients and controls with respect to sleep architecture. The time spent awake after sleep onset was longer in patients than controls. Our data highlight the presence of sleep fragmentation in traumatic apallic syndrome, which might be due to changes in brain structures responsible for sleep maintenance. The absence of sleep-wake cycles might indicate a poor outcome.     

Efficacy of trimetazidine on functional capacity in symptomatic patients with stable exertional angina — The VASCO-angina study

Background/objectives

Trimetazidine (TMZ) is a metabolic agent of proven efficacy in improving myocardial ischemia and angina. VASCO, a randomised double-blinded, placebo-controlled trial, assessed anti-anginal efficacy and safety of standard and high dose of modified-release TMZ (70 mg/d and 140 mg/d) in symptomatic and asymptomatic patients with chronic ischemic heart disease receiving background atenolol 50 mg/d on exercise test parameters.The VASCO-angina study assessed the efficacy of the two doses of TMZ on total exercise duration (TED) and time to 1-mm ST segment depression (T1), in symptomatic patients with chronic stable angina receiving background atenolol treatment.

Methods and results

In the all cohort of chronic stable angina patients TMZ significantly improved TED compared to baseline and to placebo. Both doses of TMZ significantly increased TED (p = 0.0044 and p = 0.0338 for TMZ 140 mg/d and TMZ 70 mg/d, respectively). A greater TED improvement was observed in TMZ 140 mg/d than in TMZ 70 mg/d, although the difference was not significant. Amongst patients with limiting angina during exercise test, both doses of TMZ significantly improved both T1 and TED. No difference in serious adverse events was noted between TMZ and placebo.

Conclusions

The VASCO-angina gives evidence for the efficacy and tolerability of standard and high dose of TMZ in improving effort-induced myocardial ischemia and functional capacity in patients with chronic stable angina receiving background beta-blockers.     

Expanding spectrum of TNFRSF1A gene mutations among patients with idiopathic recurrent acute pericarditis

Although idiopathic recurrent acute pericarditis (IRAP) is generally presumed to derive from an autoimmune process, increasing interest is currently being devoted to autoinflammatory diseases, a group of disorders of the innate immune system caused by mutations of genes involved in the regulation or activation of the inflammatory response, without any apparent involvement of autoimmunity. The tumour necrosis factor receptor‐1‐associated periodic syndrome is the most common autosomal dominant autoinflammatory disorder and is caused by mutations in the TNFRSF1A gene encoding the 55‐kD receptor for tumour necrosis factor‐α. IRAP patients carrying TNFRSF1A gene mutations have been recently described. We report herein the first IRAP patients carrying the rare R104Q and D12E TNFRSF1A gene mutations, thus expanding the spectrum of tumour necrosis factor receptor‐1‐associated periodic syndrome mutations in IRAP patients.     

Polygenic Parkinson's Disease Genetic Risk Score as Risk Modifier of Parkinsonism in Gaucher Disease

Background

Biallelic pathogenic variants in GBA1 are the cause of Gaucher disease (GD) type 1 (GD1), a lysosomal storage disorder resulting from deficient glucocerebrosidase. Heterozygous GBA1 variants are also a common genetic risk factor for Parkinson's disease (PD). GD manifests with considerable clinical heterogeneity and is also associated with an increased risk for PD.

Objective

The objective of this study was to investigate the contribution of PD risk variants to risk for PD in patients with GD1.

Methods

We studied 225 patients with GD1, including 199 without PD and 26 with PD. All cases were genotyped, and the genetic data were imputed using common pipelines.

Results

On average, patients with GD1 with PD have a significantly higher PD genetic risk score than those without PD (P = 0.021).

Conclusions

Our results indicate that variants included in the PD genetic risk score were more frequent in patients with GD1 who developed PD, suggesting that common risk variants may affect underlying biological pathways. © 2023 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society. This article has been contributed to by U.S. Government employees and their work is in the public domain in the USA.     

Extracorporeal membrane oxygenation for severe respiratory failure in adult patients: A systematic review and meta-analysis of current evidence

Background

Extracorporeal membrane oxygenation (ECMO) for acute respiratory failure is still a matter of debate.

Methods

We performed a structured search on Pubmed, EMBASE, Lilacs, and the Cochrane Library for randomized controlled trials and observational case-control studies with severity-paired patients, evaluating the use of ECMO on severe acute respiratory failure in adult patients. A random-effect model using DerSimonian and Laird method for variance estimator was performed to evaluate the effect of ECMO use on hospital mortality. Heterogeneity between studies was assessed with Cochran's Q statistic and Higgin's I².

Results

Three studies were included on the metanalysis, comprising 353 patients in the main analysis, in which 179 patients were ECMO supported. One study was a randomized controlled trial and two were observational studies with a propensity score matching. The most common reason for acute respiratory failure was influenza H1N1 pneumonia (45%) and pneumonia (33%). ECMO was not associated with a reduction in hospital mortality (OR = 0.71; CI 95% = 0.34 - 1.47; P = 0.358). If alternative severity-pairing method presented by the two observational studies was included, a total of 478 cases were included, in which 228 received ECMO support. In the former analysis, ECMO had a benefit on hospital mortality (OR = 0.52; CI 95% = 0.35 - 0.76; P < 0.001).

Conclusion

Extracorporeal membrane oxygenation benefit on hospital mortality is unclear. Results were sensitive to statistical analysis, and no definitive conclusion can be drawn from the available data. More studies are needed before the widespread use of ECMO can be recommended.     

Bipolar and Related Disorders Induced by Sodium 4-Phenylbutyrate in a Male Adolescent with Bile Salt Export Pump Deficiency Disease

Bile Salt Export Pump (BSEP) Deficiency disease, including Progressive Familial Intrahepatic Cholestasis type 2 (PFIC2), is a rare disease, usually leading within the first ten years to portal hypertension, liver failure, hepatocellular carcinoma. Often liver transplantation is needed. Sodium 4-phenylbutyrate (4-PB) seems to be a potential therapeutic compound for PFIC2. Psychiatric side effects in the adolescent population are little known and little studied since the drug used to treat children and infants. So we described a case of Caucasian boy, suffering from a late onset PFIC2, listed for a liver transplant when he was sixteen and treated with 4-FB (200 mg per kilogram of body weight per day). The drug was discontinued for the onset of bipolar and related disorders. This case illustrates possible psychiatric side effects of the drug.     

Accuracy of positive airway pressure titration through telemonitoring of auto-adjusting positive airway pressure device connected to a pulse oximetry in patients with obstructive sleep apnea

Purpose

In COVID-19 era, all forms of access of patients to the sleep units should be reduced as much as possible when implementing telemedicine. In the field of obstructive sleep apnea (OSA) therapy with positive airway pressure (PAP) devices, telemedicine includes the use of built-in software (BIS) and storage of PAPs and remote-controlled data (BISrc data) that are processed and transmitted daily to sleep units. We compared two methods of evaluating the final residual severity of OSA patients in home PAP titration: BISrc data versus nocturnal portable multichannel monitoring (PM) data in PAP (reference method) and to verify whether the efficacy PAP therapy guided by BISrc data was clinically adequate.

Methods

We conducted a real-life prospective study in newly diagnosed patients with OSA. Patients used an auto-adjusting positive airway pressure (AirSense 10 ResMed) with a pulse oximeter that allows daily transfer of BISrc data (apnea hypopnea index [AHI] and SaO₂) and remote changes in ventilator setting. Once the PAP titration was completed, the pressure value or ranges were kept constant for 3 days and home PM was repeated.

Results

There were 41 patients with moderate to severe OSA who completed the study. When considering AHI only, the diagnostic accuracy of BISrc on the third day was equal to 97.5%; when considering AHI > 10/h, ODI > 10/h, and SaO₂ < 90%, the diagnostic accuracy slightly decreased to 90.2%.

Conclusion

In clinical practice, the two measurement methods are equivalent. The use of BISrc data for home titration would reduce the access to sleep units. We urge that widespread use of BISrc be promoted in the current practice of management of OSA.