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We report the case of a young man and good PS, diagnosed with a stage II A Durie-Salmon, II ISS and II Revised-ISS IgM kappa Multiple Myeloma. After refractoriety to the I and II-line induction therapies, including novel and novel novel agents, we decided to treat the patient with conventional high dose chemotherapy, with dexamethasone, cyclophosphamide, etoposide and cisplatin (DCEP), achieving a very good partial response (VGPR). Furthermore, we discuss histopathologic patterns, to make a correct differential diagnosis with others IgM-correlated disorders. Analyzing the literature, we demonstrated that more intensive therapy correlates with better therapeutic goals, therefore, empathizing that today IgM Multiple Myeloma is still a difficult pathological subtype to treat.

The Italian nephrology has a long tradition and experience in the field of dietetic-nutritional therapy (DNT), which is an important component in the conservative management of the patient suffering from a chronic kidney disease, which precedes and integrates the pharmacological therapies. The objectives of DNT include the maintenance of an optimal nutritional status, the prevention and / or correction of signs, symptoms and complications of chronic renal failure and, possibly, the delay in starting of dialysis. The DNT includes modulation of protein intake, adequacy of caloric intake, control of sodium and potassium intake, and reduction of phosphorus intake. For all dietary-nutritional therapies, and in particular those aimed at the patient with chronic renal failure, the problem of patient adherence to the dietetic-nutritional scheme is a key element for the success and safety of the DNT and it can be favored by an interdisciplinary and multi-professional approach of information, education, dietary prescription and follow-up. This consensus document, which defines twenty (20) essential points of the nutritional approach to patients with advanced chronic renal failure, has been written, discussed and shared by the Italian nephrologists together with representatives of dietitians (ANDID) and patients (ANED)., (Copyright by Società Italiana di Nefrologia SIN, Rome, Italy.)

Objective: Although a concern exists that 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitors (statins) might increase the risk of intracerebral haemorrhage (ICH), the contribution of these agents to the relationship between serum cholesterol and disease occurrence has been poorly investigated., Methods: We compared consecutive patients having ICH with age and sex-matched stroke-free control subjects in a case-control analysis, as part of the Multicenter Study on Cerebral Haemorrhage in Italy (MUCH-Italy), and tested the presence of interaction effects between total serum cholesterol levels and statins on the risk of ICH., Results: A total of 3492 cases (mean age, 73.0±12.7 years; males, 56.6%) and 3492 control subjects were enrolled. Increasing total serum cholesterol levels were confirmed to be inversely associated with ICH. We observed a statistical interaction between total serum cholesterol levels and statin use for the risk of haemorrhage (Interaction OR (IOR), 1.09; 95% CI 1.05 to 1.12). Increasing levels of total serum cholesterol were associated with a decreased risk of ICH within statin strata (average OR, 0.87; 95% CI 0.86 to 0.88 for every increase of 0.26 mmol/l of total serum cholesterol concentrations), while statin use was associated with an increased risk (OR, 1.54; 95% CI 1.31 to 1.81 of the average level of total serum cholesterol). The protective effect of serum cholesterol against ICH was reduced by statins in strictly lobar brain regions more than in non-lobar ones., Conclusions: Statin therapy and total serum cholesterol levels exhibit interaction effects towards the risk of ICH. The magnitude of such effects appears higher in lobar brain regions., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/)

Background: We sought to compare the benefit of percutaneous closure to that of medical therapy alone for the secondary prevention of embolism in patients with patent foramen ovale (PFO) and otherwise unexplained ischemic stroke, in a propensity scored study., Methods and Results: Between 2000 and 2012, we selected consecutive first-ever ischemic stroke patients aged 18 to 45 years with PFO and no other cause of brain ischemia, as part of the IPSYS registry (Italian Project on Stroke in Young Adults), who underwent either percutaneous PFO closure or medical therapy for comparative analysis. Primary end point was a composite of ischemic stroke, transient ischemic attack, or peripheral embolism. Secondary end point was brain ischemia. Five hundred and twenty-one patients qualified for the analysis. The primary end point occurred in 15 patients treated with percutaneous PFO closure (7.3%) versus 33 patients medically treated (10.5%; hazard ratio, 0.72; 95% confidence interval, 0.39-1.32; P=0.285). The rates of the secondary end point brain ischemia were also similar in the 2 treatment groups (6.3% in the PFO closure group versus 10.2% in the medically treated group; hazard ratio, 0.64; 95% confidence interval, 0.33-1.21; P=0.168). Closure provided a benefit in patients aged 18 to 36 years (hazard ratio, 0.19; 95% confidence interval, 0.04-0.81; P=0.026) and in those with a substantial right-to-left shunt size (hazard ratio, 0.19; 95% confidence interval, 0.05-0.68; P=0.011)., Conclusions: PFO closure seems as effective as medical therapy for secondary prevention of cryptogenic ischemic stroke. Whether device treatment might be more effective in selected cases, such as in patients younger than 37 years and in those with a substantial right-to-left shunt size, deserves further investigation., (© 2016 American Heart Association, Inc.)

Unlabelled: Rapid-onset Obesity with Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation (ROHHAD) is a rare and complex pediatric disorder. Children typically show ROHHAD after the first years of life with rapid weight gain and subsequently autonomic nervous system dysregulation (altered pain perception, pupillary dysfunction, hypothermia and bradycardia); alveolar hypoventilation with risk of cardiorespiratory arrest and hypothalamic dysfunction (central diabetes insipidus, hypothyroidism, growth hormone and corticotrophin deficiency). Tumours of neural crest origin, such as ganglioneuroblastoma and ganglioneuronoma, are reported in 33% of the patients and may be found in the chest or abdomen. Here we describe two girls who presented with rapid weight gain, at the age of 5 and 9 years respectively. The first was admitted due to obesity and central hypothyroidism. After two months she rapidly developed a clinical picture characterized by thermal dysregulation, hypodipsia and severe hypernatriemia, hypertrigliceridemia, alveolar hypoventilation supported by mechanical ventilation. The second presented with rapid-onset obesity and a mild hyperprolactinemia. After three months of follow-up she was admitted due to a clinical picture of hypothermia, seizures and hyponatremia. Subsequentely she developed altered water balance (severe hypernatremia) and severe hypoventilation. Chest CT and MR imaging showed a posterior mediastinal mass. Endocrinological investigation showed corticotrophin deficiency and central hypothyroidism treated with specific replacement therapies., Conclusions: On the basis of our experiences we can infer that it is necessary perform specific further investigations of hypothalamic function in all the children with rapid onset obesity in order to early prevent the catastrophic consequences that may occur in this syndrome.

The zebrafish pineal gland plays a fundamental role in the regulation of the circadian rhythm through the melatonin secretion. The pinealocytes, also called photoreceptive cells, are considered the morphofunctional unit of pineal gland. In literature, the anatomical features, the cellular characteristics, and the pinealocytes morphology of zebrafish pineal gland have not been previously described in detail. Therefore, this study was undertaken to analyze the structure and ultrastructure, as well as the immunohistochemical profile of the zebrafish pineal gland with particular reference to the pinealocytes. Here, we demonstrated, using RT-PCR, immunohistochemistry and transmission electron microscopy, the expression of the mRNA for rhodopsin in the pineal gland of zebrafish, as well as its cellular localization exclusively in the pinealocytes of adult zebrafish. Moreover, the ultrastructural observations demonstrated that the pinealocytes were constituted by an outer segment with numerous lamellar membranes, an inner segment with many mitochondria, and a basal pole with the synapses. Our results taken together demonstrated a central role of zebrafish pinealocytes in the control of pineal gland functions., (Copyright © 2011 Wiley Periodicals, Inc.)

Aim of the Study: The superior pedicle mammaplasty is a technique frequently employed in the treatment of breast ptosis, associated or not with hypertrophy of the gland, followed by satisfactory results. This technique is normally not indicated in severe breast ptosis (pendulous breast) because of the excessive length of the pedicle supplying nipple-areola-complex (NAC), with the risk of ischemia. In these cases the standard technique is the free-nipple-graft mammaplasty. However, the deepen knowledge about vascular anatomy of the breast and the aptitude to perform superior pedicle mammaplasty, induced the authors to indicate this technique even in these cases improving the aesthetic and functional outcomes., Material and Methods: The authors present a series of 30 patient with pendulous breasts, with sternal notch-nipple distance equal or superior to 32 cm (45 cm maximum; mean value 35.1), treated with the superior pedicle mammaplasty with inverted "T" scar. The results confirm the reliability of superior pedicle for the nipple-areolar complex blood supply, associated with satisfactory aesthetic results due especially to the good breast projection., Conclusions: They conclude that superior pedicle technique mammaplasty, even if normally not indicated in these cases, is instead suitable for the treatment of pendulous breasts with great sternal notch-nipple distance, permitting to take advantages of this technique.

Objective: Oxidative stress is a condition caused by an increase of Reactive Oxygen Species (ROS) or by a shortage of the mechanisms of cellular protection and antioxidant defence. ROS have a potential oxidative effect towards various cellular macromolecules: proteins, nucleic acids, proteoglycans, lipids, with consequent damages in several cellular districts and promotion of the ageing process of the organism. However, some substances are able to prevent and/or reduce the damages caused by ROS; therefore, they are defined antioxidant. The present research studied, in a group of subjects, the antioxidant effects of the green tea, that was administered with fruit and vegetables in a strictly controlled diet., Materials and Methods: 50 subjects were selected and requested to daily consume 2-3 fruit portions (especially pineapple), 3-5 portions of vegetables (especially tomato) and 2-3 glasses of green tea for about 2 months to integrate the controlled basic diet. Some indicators of the oxidative stress were measured in the plasma before and after the integration period., Results: The integration of a basic diet with supplements of fruit, vegetables and green tea turned out to be able in increasing both plasmatic total antioxidant capacity and endogenous antioxidant levels and to reduce the lipid peroxidation of the membranes, suggesting a reduction of the oxidative stress., Conclusions: These data suggest that an adequate supplement of antioxidants can prevent oxidative stress and correlated pathologies.

The extraction of silica from powdered glass cullet with an aqueous solution of sodium hydroxide has been proposed as an alternative to glass recycling aimed to the low temperature production of sodium silicates. The unextracted residue obtained after a counter current two-step extractive process at approximately 100 degrees C and room pressure is mainly made of calcium and sodium silicate and shows high porosity and a large surface area. We thought that it could be active as an agent for the removal of heavy metals from wastewater. In this paper the capacity of the unextracted residue of removing six metal ions (i.e., Cu2+, Ni2+, Zn2+, Cd2+, Pb2+ e Cr3+) was studied in a stirred batch reactor. The data obtained demonstrate that the removal of metal ions from wastewater is achieved with high capacity in a short time and their concentration is lowered under the legal limits without any appreciable influence from changes of physical and chemical conditions. Sodium and calcium ions take the place of heavy metals in water while pH keeps almost neutral. The exchange mechanism was identified.

Mc Kusick-Kaufman Syndrome (SMK) is an autosomal recessive multiple malformation Syndrome characterized by hydrometrocolpos and polydactyly. This Syndrome is more frequent in females, whose parents are first-degree cousins (frequency 1:8 liveborn). We describe two cases of SMK: a male and a female. Patients were siblings and their parents were first cousins. The first child presented postaxial hexadactily, of the hands and feet, cardiovascular malformation and Arnold-Chiari II malformation. The baby died on the 17th day of life, for neurosurgical complications. Second baby, in which a prenatal ultrasonography showed a large cystic dilatation in the pelvis, presented with urinary hydrometrocolpos associated with persistent urogenital sinus (UGS) and polydactyly. The patient was temporary treated with intermittent vaginal catheterization. At age of 6 months she underwent reconstructive surgery via the anterior sagittal transanorectal approach (ASTRA), previous creation of a colostomy. Thirty-two months of the operation, the patient is continent for stool and urine and the vagina looks normal.

Urogenital Sinus (UGS) malformation can be ascribed to an arrest of normal embryonic vaginal development. Neonates with UGS frequently have ambiguous genitalia, rarely the vulva may be normal. The aim of this work is to define the role of prenatal sonography in the diagnosis of UGS associated with hydrocolpos and/or hydrometrocolpos. The Authors report their experience on 3 cases of UGS without ambiguous genitalia with hydrometrocolpos, in which prenatal sonography had shown a cystic dilatation in the pelvis. After birth the 3 neonates presented with female genitalia and a single orifice between the labia. The pelvis sonography showed in all the cases an hydrometrocolpos with a large vagina and a compressed and anteriorly located bladder. Voiding cystourethrogram, genitography and genitoscopy confirmed the presence of an UGS with urinary retention inside the vagina and stenosis of the distal portion of the vagina itself. An early drainage of the capacious vagina was performed in the three patients. There are very few reports in the literature of UGS with hydrometrocolpos diagnosed in utero. The cystic dilatation of the vagina is always misdiagnosed with a distended bladder. In utero, infact, the bladder can not be identified being displaced anteriorly by the vagina. The presence of a fluid-debris level inside the cystic anechoic mass must be considered a crucial finding. Multiple echoes are due to vaginal secretions. Prenatal ultrasound has then a definitive role in detecting an obstructed genital tract. This allows to rapidly drain the vagina relieving urinary tract obstruction.

The occurrence of lignicolous marine fungi in Antarctic marine water far from the coast is reported. Thirty-six wooden baits (beech and poplar), hung in a metallic structure were immersed in Penguin Bay (Adelie Cove) at a depth of 50 m for a year. Fourteen species belonging to Deuteromycotina and six Ascomycotina were observed on the baits examined in Italy. Phoma sp., Trichocladium achrasporum, Trichocladium constrictum and Trichocladium lignincola were the predominant species. Phoma sp., the first species, are not typically marine, but rather of terrestrial origin. Penguin dung proximity can explain this occurrence.

Videourodynamic investigation was carried on 62 patients with enuresis (34 females, 28 males). Patients were all grouped in four different clinical types according to the International Continence Society: (Type I) monosymptomatic enuresis nocturnal (24 cases); (Type II) nocturnal and diurnal enuresis without daytime frequency (8 cases); Type III) nocturnal enuresis with daytime frequency (22 cases); (Type IV) nocturnal and diurnal enuresis with daytime frequency and/or urgency (8 cases). In children with monosymptomatic enuresis nocturnal the videourodynamic study revealed, in 85% of cases, a normal bladder; in only 17% of cases was observed a detrusor instability. In patients with Type II enuresis a normal bladder was observed in 6 cases, whereas in two a detrusor instability was present. Among the 22 patients with Type III enuresis, 13 cases (59%) had a detrusor instability, 3 cases (14%) had a vesicosfinteric dyssinergia and 6 (27%) had a normal activity. In this group of patients it was documented in 10 cases a trabeculated bladder together with a vesicoureteral reflux in 4 patients, meatal stenosis and bladder diverticula in other two cases. All patients with Type IV enuresis documented pathological data: detrusor instability in 4 patients, vesico-sfinteric dyssinergia in two and neurogenic non-neurogenic bladder in the other two cases. A severe trabeculated bladder was present in all this cases, with vesico-ureteral reflux in two patient. Maximum cystometric capacity was low in 41 cases (66%), normal in 20 (32%), high in 1 (2%), independently of the types of enuresis.

Hemangioma of the bladder is a rare benign tumor. Hematuria is the most common symptom of the disease. The Authors report their experience on two isolated cases of bladder hemangioma. The lesions were diagnosed by endoscopy and biopsy. In both cases the hemangiomas were electrocoagulated endoscopically. Follow-up examination after 1 year, has shown complete eradication of the lesion.

Histologic and Transforming Growth Factor Beta (TGF-beta) immunostain patterns were sought in resected distal urinary tracts from 17 Primary Megaureter (PM) affected children, referred to surgery. Comparative observations were also carried out on embryonal and fetal ureteral buds of both humans and bovines. A reciprocal resemblance was mainly objectivized between the resected "narrowed" ureters of patients under 18 months, and the fetal ureteral buds at 26th and 38th gestational week. A development delay was irrespectively observed in PM "narrowed" ureters, at the longitudinal muscle-bundles in the parietal juxta-luminal compartment. A consistent TGF-beta immunostain cytoplasmic reaction there selectively depicted the growing mesenchymal lines, including both the undifferentiated single cells and the muscle-like profiled ones. These results agree with very recent reports perspecting a segmental maturation delay as a pathogenetic moment of PM. Because of the acquired potent TGF-beta inhibitory role on myoblasts differentiation, the present study substantiates a persistent TGF-beta role in perinatal ureter dilations.

Background: Some neonatal benign convulsions are genetic in origin, with a dominant mode of inheritance., Case Report: A girl was placed on continuous EEG recording from her 2d day of life because of her family history. The first clonic seizures occurred on the 4th day; they appeared again on the 6th day and became prolonged with an abnormal EEG pattern. The seizures were well controlled with phenobarbital, that was gradually discontinued when the child was 3 months old. Seizures occurred again when she was 4 months old and were again controlled with phenobarbital. Her father had had neonatal convulsions which were not well analysed. Her brother also had clonic seizures at the 4th day of life; they disappeared after 1 month. Her sister suffered from clonic seizures when she was 3 days old, and these became prolonged. She was given phenobarbital until she was 1 year old. She developed benign rolandic epilepsy at the age of 10 years., Conclusion: This family suffers from neonatal familial benign convulsions and rolandic epilepsy. The frequency of neonatal familial benign epilepsy is probably under-estimated.

Mitochondrial abnormalities have been previously reported in some patients with myotonic dystrophy (DM). The aim of the present paper was to study muscle mitochondria in 32 DM patients by morphological, biochemical (when suggested by morphology) and genetic analysis. A single ragged red fiber, but no cytochrome c oxidase-negative fibers were found in the muscle specimen of 1 patient. However, mitochondrial enzyme activities resulted within the normal range. An electron microscopy study showed no significant mitochondrial changes. Southern blot analysis did not reveal any mitochondrial DNA heteroplasmy in all 32 patients. An explanation for the discordant results between this study and some previous reports of mitochondrial alterations in DM might be that their occasional presence is not related to the disease but to ageing. Another possibility is that among patients with a myotonic dystrophy phenotype, a small subgroup of subjects with a mitochondrial disease may exist and be differentiated.