Abstract
Background
Loeys–Dietz syndrome (LDS) is a rare autosomal dominant genetic disorder of connective tissue, characterised by a broad spectrum of craniofacial, vascular (arterial tortuosity and ...aneurysms) and skeletal clinical signs. Four subtypes have been described, related to mutations in genes encoding for components of the transforming growth factor beta signalling pathway (TGFBR1, TGFBR2, SMAD2, SMAD3, TGFB2 and TGFB3). Diagnosis is based on the evaluation of clinical manifestations and family history. Therefore, many radiologists remain unfamiliar with the imaging and clinical findings in LDS.
The Case
75–year–old woman, history of surgery for ruptured gastric a. aneurysm and ectasia of visceral arterial vessels. One year onset of worsening dyspnoea and asthenia, at echocardiography (ECOCG) severe aortic insufficiency has been detected and patient underwent aortic valve replacement with bioprosthesis. Cardiac surgery was complicated by aortic dissection (Stanford A; DeBakey I type) treated with replacement of ascending aorta and anterior hemiarch with tubular prosthesis. On that occasion a family diagnosis of SMAD3 gene mutation was made (both sister and nephew typed), compatible with Loeys–Dietz syndrome type 3. At the control angioTC evidence of proximal and distal anastomosis in order, in the absence of signs of periprosthetic leak. At ECOCG (FE 55%), thickened septum with marked postcardiac dyskinesia. No abnormality detected on ECG. Due to persistence of dyspnoea on mild exertion (NYHA class III) and reduced tolerance to orthostatism, cardiac rehabilitation program was undertaken on an outpatient basis for three months, providing an improvement in exertion tolerance (NYHA II) and a benefit on functional autonomy. LDS is a multisystem connective tissue disorder that is associated with a high burden of complications, a correct diagnosis will allow clinicians to appropriately establish the best therapeutic strategy, especially in cardiac surgery, taking into account the intraoperative and perioperative potential risks.
Abstract
Background
Amphotericin B (AmB) is an antifungal drug commonly used in a broad spectrum of infections, including leishmaniasis. Leishmaniasis, transmitted through insect bites, has ...cutaneous, mucocutaneous, or visceral (particularly liver and spleen) involvement. The main adverse effects of AmB are cardiotoxicity and nephrotoxicity, often reversible, but with yet unclear pathophysiology. Reversible dilated cardiomyopathy is a rare disease with very few cases described in the literature (less than 10).
The Case
A 41–year–old man with a silent cardiological history (only cocaine and alcohol abuse in past) is admitted for visceral leishmaniasis. Cardiological evaluation: LBBB in ECG (none previous available); no abnormality detected on ECOCG (EF 55%). Ten days after the AmB cycle, onset of worsening dyspnoea needing hospital treatment after a further two weeks for heart failure (HF). At ECOCG severe left ventricular dysfunction (EF 30%), no rise of cardiac enzymes was detected. Coronary angiography showed 50–75% stenosis on RCA and LAD, treated by angioplasty and stenting. Cardiac MRI showed: dilated left ventricle with diffuse biventricular hypokinesis (FE 16% left and 22% right), areas of LGE of non–ischemic appearance. HFrEF GDMTs were prescribed, with progressive clinical improvement over the next four weeks; at cardiac MRI performed one month later, partially recovery of biventricular function (FE sn 41% right 48%) was found. The ECG showed regression of LBBB. No myocardial biopsy was performed, so it is not possible to get to an unequivocal cardiac dysfunction’s cause. However, the time relationship between the use of AmB and the clinical findings evolution, consistently with the few literature available, suggests a probable diagnosis of AmB toxic cardiomyopathy. An ischaemic cause of the dysfunction is unlikely by the MRI feature and by coronary angiography findings. Literature data suggest that the presence of predisposing factors for cardiac dysfunction, rather than the duration of treatment or preparation used, is the most relevant factor in the development of AmB–related cardiomyopathy. It’s important to perform cardiac monitoring in all patients receiving AmB therapy. In all reported cases, cardiac function recovered substantially after cessation of AmB treatment.
Increased climate variability is driving changes in water storage across the contiguous United States (CONUS). Observational estimates of these storage changes are important for validation of ...hydrological models and predicting future water availability. We estimate CONUS terrestrial water storage anomalies (TWSA) from 2007–2017 using Global Positioning System (GPS) displacements, constrained by lower‐resolution TWSA observations from Gravity Recovery and Climate Experiment (GRACE) satellite gravity—a combination that provides higher spatiotemporal resolution than previous estimates. The relative contribution of seasonal, interannual, and subseasonal TWSA varies widely across CONUS watersheds, with implications for regional water security. Separately, we find positive correlation between TWSA and the El Niño/Southern Oscillation in the southeastern Texas‐Gulf and South Atlantic‐Gulf watersheds and an unexpected negative correlation in the southwest. In the western United States, atmospheric rivers (ARs) drive a large fraction of subseasonal TWSA, with the top 5% of ARs contributing 73% of total AR‐related TWSA increases.
Key Points
A novel combination of GPS and GRACE provides higher‐resolution water storage than GRACE alone, improving water availability analysis
The ratio of interannual to seasonal water storage change varies widely across the United States, highlighting local vulnerability to water stress
Atmospheric river (AR) events drive rapid water storage increases across the West, with the top 5% of ARs contributing 73% of the AR total
Aim
To assess whether LC diets are associated with long-term improvement in glycemic control and weight loss in people with T2DM, and their cardiovascular and renal safety.
Methods
Meta-analysis of ...randomized controlled trials lasting more than 3 months, retrieved through extensive search on PubMed, Embase, ClinicalTrial.gov, Cochrane databases up to March 1st, comparing LC diets and balanced carbohydrate diets in people with T2DM.
Results
We retrieved 37 trials, including 3301 patients. Average carbohydrate intake in LC diets was 36% of total energy. LC diets were associated with significant reduction of HbA1c at 3 months (MD − 0.17%, 95% CI − 0.27, − 0.07), no difference at 6 and 12 months, and significant increase at 24 months (MD 0.23%, 95% CI MD 0.02, 0.44). VLC diets were associated with significant HbA1c reduction at 3 and 6 months (MD − 0.43% − 0.60, − 0.26%, and MD − 0.40% 95% CI − 0.59, − 0.22, respectively), but not at 12 and 24 months. LC diets were associated with significant BMI reduction at 6 months (− 1.35 kg/m
2
95% CI, − 2.18, − 0.52), but not at other time points. Only a minority of trials reported data on renal function, so renal safety could not be assessed. No significant differences in body weight, lipid profile, or blood pressure were found in the long term.
Conclusion
LC diets may produce small short-term improvements in HbA1c and weight, which are not maintained in the long term. Data on their renal safety are insufficient.
Objective
To assess whether ballet dancers have higher eating psychopathology mean scores than the general population.
Methods
Meta-analysis of cross-sectional observational studies comparing the ...scores of one or more of the validated eating psychopathological scales between ballet dancers and any control groups.
Results
Twelve studies were included in the metanalysis. Ballet dancers had a significantly higher EAT score (12 studies retrieved, SMD 0.82 95% CI 0.44–1.19,
p
< 0.00001,
I
2
= 84); subgroup analysis suggested a possible role of control subjects’ choice in explaining heterogeneity. Scores on the EDI subscales of Drive for Thinness, Bulimia, and Body dissatisfaction were available from four studies; Drive for Thinness was higher in ballet dancers (SMD 0.62 0.01, 1.22), as well as the Bulimia scale (SMD 0.38 0.02, 0.73,
p
= 0.04) and the Body Dissatisfaction scale (SMD 0.34 0.15, 0.53). Data on Perfectionism, Interpersonal problems, Ineffectiveness, and Maturity fears, were available from three studies. Higher scores in Perfectionism (SMD 0.68 0.24, 1.12
p
= 0.02), Interpersonal problems (SMD 0.24 0.02, 0.47, in Inefficacy, (SMD 2.18 1.31, 3.06) were found for ballet dancers; on the other hand, Maturity fears scores were not significantly different between ballet dancers and controls (IV-MD = 0.15 − 0.07, 0.36). Seven studies reported tests not performed elsewhere.
Discussion
Ballet dancers show a higher level of restriction and drive for thinness than controls, and they may be, therefore, at higher risk for the development of eating disorders. Available studies do not allow the discrimination of dysfunctional eating attitudes and behaviors from adaptive responses.
Level of evidence
Level I (evidence obtained from systematic reviews and meta-analyses).
Background
Degludec is a long-acting insulin with a longer duration of action and a greater day-to-day reproducibility of absorption in comparison with previous long-acting insulin formulations. The ...aim is the definition of the change in insulin needs in patients switching from detemir/glargine to degludec in real-life conditions.
Methods
In this retrospective cohort observational study, all outpatients with either type 1 or type 2 diabetes, starting therapy with degludec insulin—after a prior treatment with either detemir or glargine insulin for at least 6 months—were included.
Results
The analysis was performed on 266 patients, 172 and 96 with type 1 and type 2 diabetes, respectively. The equations describing the relationship between baseline and follow-up doses of basal insulin (6 months) were
Y
= 3.39 + 0.78
X
and
Y
= 0.44 + 0.69
X
, in patients receiving detemir/glargine either once or twice daily, respectively (
Y
= degludec dose at 6 months and
X
= basal insulin dose at switch). The corresponding equations for prandial insulin doses were
y
= 1.83 + 0.83*
x
and
y
= 2.85 + 0.80*
x
for those on pre-switch once or twice-daily basal insulin, respectively. In type 2 diabetes, the switch was associated with a reduction of basal insulin doses only in those with a prior twice-daily treatment with basal insulin. The reduction of prandial insulin reached statistical significance only in patients previously treated with basal insulin once daily.
Conclusions
The present results provide a suggestion for a simple method for the adjustment of basal and prandial insulin doses in type 1 diabetic patients, switching from glargine or detemir to degludec.
Summary
Although in females of infertile couples abnormal prolactin (PRL) has a definitive role in the medical flowchart, its role in males is less clear. Animal models suggest that PRL does not play ...a major role in male reproduction, although its trophic action on male accessory glands was often observed. Studies in humans are scanty. We systematically evaluated possible clinical and ultrasound correlates of PRL in males of infertile couples. Of 288 consecutive males of infertile couples, 269 (36.6 ± 4.4 years) without genetic abnormalities were studied. All men underwent physical, biochemical, seminal evaluation and scrotal and transrectal ultrasound before and after ejaculation. Ejaculatory and erectile functions were assessed by Premature Ejaculation Diagnostic Tool (PEDT) and International Index of Erectile Function (IIEF)‐15 respectively; prostate‐related symptoms by National Institutes of Health‐Chronic Prostatitis Symptom Index and International Prostate Symptom Score; psychological symptoms by Middlesex Hospital Questionnaire. Among semen parameters, only the positive association between PRL and ejaculate volume was significant, even adjusting for age, total testosterone and thyroid‐stimulating hormone (adj. r = 0.126, p < 0.05). In a logistic ordinal model, adjusting for the aforementioned confounders and ejaculate volume, PRL was negatively associated with delaying ejaculation according to PEDT#1 score (Wald = 4.65, p < 0.05). In an age‐ and ejaculate volume‐adjusted, iterative binary logistic model, low PRL was associated with a fivefold risk of any failure in controlling ejaculation (HR = 5.15 1.15–23, p < 0.05). Among scrotal and transrectal ultrasound features, we found a significant positive association between PRL and seminal vesicles (SV) volume and inhomogeneity, before and after ejaculation, and with deferential ampullas diameter. Associations with PRL were confirmed in nested 1 : 1 case–control analysis. No significant associations were found between PRL and other clinical parameters. For the first time, this study extends the concept of a trophic effect of PRL on male accessory glands from animals to humans. We report a positive association among PRL and ejaculate and SV volume, before and after ejaculation. Low PRL is associated with a lessened ability to control ejaculation.
Purpose
Hyponatremia occurs in about 30% of patients with pneumonia, including those with SARS-CoV-2 (COVID-19) infection. Hyponatremia predicts a worse outcome in several pathologic conditions and ...in COVID-19 has been associated with a higher risk of non-invasive ventilation, ICU transfer and death. The main objective of this study was to determine whether early hyponatremia is also a predictor of long-term sequelae at follow-up.
Methods
In this observational study, we collected 6-month follow-up data from 189 laboratory-confirmed COVID-19 patients previously admitted to a University Hospital. About 25% of the patients (
n
= 47) had hyponatremia at the time of hospital admission.
Results
Serum Na
+
was significantly increased in the whole group of 189 patients at 6 months, compared to the value at hospital admission (141.4 ± 2.2 vs 137 ± 3.5 mEq/L,
p
< 0.001). In addition, IL-6 levels decreased and the PaO
2
/FiO
2
increased. Accordingly, pulmonary involvement, evaluated at the chest X-ray by the RALE score, decreased. However, in patients with hyponatremia at hospital admission, higher levels of LDH, fibrinogen, troponin T and NT-ProBNP were detected at follow-up, compared to patients with normonatremia at admission. In addition, hyponatremia at admission was associated with worse echocardiography parameters related to right ventricular function, together with a higher RALE score.
Conclusion
These results suggest that early hyponatremia in COVID-19 patients is associated with the presence of laboratory and imaging parameters indicating a greater pulmonary and right-sided heart involvement at follow-up.
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