Hearing loss in beta-thalassaemia: An Italian multicentre case-control study.

BACKGROUND: Despite numerous studies, the true scenario of hearing loss in beta-thalassaemia remains rather nebulous. MATERIALS AND METHODS: Pure tone audiometry, chelation therapy, demographics and laboratory data of 376 patients (mean age 38.5 ± 16.6 years, 204 females, 66 non-transfusion-dependent) and 139 healthy controls (mean age 37.6 ± 17.7 years, 81 females) were collected. RESULTS: Patient and control groups did not differ for age (p = 0.59) or sex (p = 0.44). Hypoacusis rate was higher in patients (26.6% vs. 7.2%; p < 0.00001), correlated with male sex (32.6% in males vs. 21.8% in females; p = 0.01) and it was sensorineural in 79/100. Hypoacusis rate correlated with increasing age (p = 0.0006) but not with phenotype (13/66 non-transfusion-dependent vs. 87/310 transfusion-dependent patients; p = 0.16). Sensorineural-notch prevalence rate did not differ between patients (11.4%) and controls (12.2%); it correlated with age (p = 0.01) but not with patients' sex or phenotype. Among adult patients without chelation therapy, the sensorineural hypoacusis rate was non-significantly lower compared to chelation-treated patients while it was significantly higher compared to controls (p = 0.003). CONCLUSIONS: Sensorineural hypoacusis rate is high in beta-thalassaemia (about 21%) and it increases with age and in males while disease severity or chelation treatment seems to be less relevant. The meaning of sensorineural-notch in beta-thalassaemia appears questionable.

Oleothorax: An Ultrasound Sign of an Old Practice.

INTRODUCTION: The high morbidity and mortality of tuberculosis has led to the development of numerous therapeutic interventions during the pre-antibiotic era. In 1894, Forlanini proposed the technique of collapse therapy, using spontaneous pneumothorax. In 1926, Bernou developed the oleothorax technique to induce an iatrogenic collapse of the lung through the instillation of oil into the pleural cavity, which was subsequently removed. Nowadays, there are few patients that still represent a living testimony of this historic technique and have been described through traditional radiology. CASE PRESENTATION: We report the case of a patient with evidence of a right oleothorax that was investigated not only with traditional radiology but also with the use of chest ultrasonography. Ultrasounds were able to show peculiar characteristics of the oleothorax, including its particular echogenicity, the rigidity and static nature of the collection, the presence of peripheral calcifications, and the negative impact of the collection on diaphragmatic mobility and excursion. CONCLUSION: To our knowledge, this is the first report of an ultrasound description of oleothorax. We have observed that ultrasound examination, in cases of basal oleothorax, is able to add information regarding its radiological appearance and physiopathological implications on ventilatory mechanics and diaphragmatic distress.

The Impact of COVID-19 Pandemic Lockdown on the Relationship between Pediatric MAFLD and Renal Function.

BACKGROUND: Both direct and indirect effects of COVID-19 have been found in all age groups. In particular, adult data demonstrated significant changes in patients with chronic and metabolic disease (e.g., obesity, diabetes, chronic kidney disease (CKD), and metabolic associated fatty liver dysfunction (MAFLD)), while similar pediatric evidence is still limited. We aimed at investigating the impact of the COVID-19 pandemic lockdown on the relationship between MAFLD and renal function in children with CKD due to congenital abnormalities of the kidney and urinary tract (CAKUT). METHODS: A total of 21 children with CAKUT and CKD ≥ stage 1 underwent a comprehensive evaluation within 3 months before and 6 months after the first Italian lockdown. RESULTS: At follow-up, CKD patients with MAFLD presented higher BMI-SDS, serum uric acid, triglycerides, and microalbuminuria levels and lower eGFR levels than those without MAFLD (all p < 0.05). Higher ferritin and white blood cell concentrations were also found in patients with CKD diagnosed with MAFLD than peers without MAFLD (both p = 0.01). Compared to children without MAFLD, a higher delta of BMI-SDS, eGFR levels, and microalbuminuria levels was found in patients with MAFLD. CONCLUSIONS: Due to the negative influence of the COVID-19 lockdown on cardiometabolic health in childhood, a careful management of children with CKD is warranted.

[Newer anti - diabetic therapies and chronic kidney disease]. / Il trattamento del diabete mellito di tipo 2 nei pazienti affetti da malattia renale cronica: cosa aspettarsi dai nuovi ipoglicemizzanti orali.

Worldwide, an estimated 200 million people have chronic kidney disease (CKD), whose most common causes include hypertension, arteriosclerosis, and diabetes. About 40% of patients with diabetes develop CKD. Intensive blood glucose control through pharmacological intervention can delay CKD progression. Standard therapies for the treatment of type 2 diabetes include metformin, sulfonylureas, meglitinides, thiazolidinediones and insulin. While these drugs have an important role in the management of type 2 diabetes, only the thiazolidinedione pioglitazone can be used across the spectrum of CKD (stages 25) and without dose adjustment. Newer therapies, particularly dipeptidyl peptidase-IV inhibitors, glucagon-like peptide-1 receptor agonists and sodium-glucose cotransporter-2 inhibitors are increasingly being used in the treatment of type 2 diabetes. However, a major consideration is whether these newer therapies can also be used safely and effectively across the spectrum of renal impairment.

[Acute Kidney Injury, Type - 3 cardiorenal syndrome, Biomarkers, Renal Replacement Therapy]. / Aki (Acute Kidney Injury) e patologia cardiovascolare: dai dati epidemiologici alle strategie terapeutiche.

Cardiovascular disease and major cardiovascular events represent main cause of death in both acute and chronic kidney disease patients. Kidney and heart failure are common and frequently co-exist This organ-organ interaction, also called organ cross-talk, leads to well-known definition of cardiorenal syndrome (CRS). Here we will describe cardiovascular involvement in patients with acute kidney injury (AKI). Also known as Type-3 CRS or acute reno-cardiac CRS, it occurs when AKI contributes and/or precipitates development of acute cardiac injury. AKI may directly or indirectly produces an acute cardiac event and it can be associated with volume overload, metabolic acidosis and electrolytes disorders such as hyperkalemia and hypocalcemia, coronary artery disease, left ventricular dysfunction and fibrosis which has been also described in patients with AKI with the consequence of direct negative effects on cardiac performance.

[The nephrologist and the role of ultrasound imaging in the diagnosis of cardiorenal syndrome]. / Il nefrologo e il ruolo dell'imaging ultrasonografico nella diagnosi di sindrome cardio-renale.

The term cardiorenal syndrome (CRS) refers to multiple possible clinicopathological correlations between heart and kidney failure. The most recent classification recognizes five types of CRS: types I and II originate from heart failure (acute and chronic, respectively), type III and IV from kidney failure (again acute and chronic), while type V originates from a range of systemic diseases. Echocardiography and renal ultrasound are important means to arrive at a correct diagnosis. Basic echocardiography (defined by some as "echocardioscopy") allows the assessment of the left and right ventricles (diastolic and systolic function), atrial size, pulmonary circulation markers such as systolic pulmonary arterial pressure (PAPs) and tricuspid annular plane excursion (TAPSE), pericardial effusions, valve dysfunctions, and volume repletion. Renal ultrasound is of help in distinguishing between chronic and acute renal failure (kidney volume, parenchymal thickness, echogenicity) and excluding obstructive kidney disease.