Management of Neonatal Isolated and Combined Growth Hormone Deficiency: Current Status.

Congenital growth hormone deficiency (GHD) is a rare disease caused by disorders affecting the morphogenesis and function of the pituitary gland. It is sometimes found in isolation but is more frequently associated with multiple pituitary hormone deficiency. In some cases, GHD may have a genetic basis.

Polycystic ovary syndrome in pediatric obesity and diabetes.

Polycystic ovary syndrome is characterized by anovulation (amenorrhea, oligomenorrhea, irregular menstrual cycles) combined with symptoms of androgen excess (hirsutism, acne, alopecia). The clear definition and diagnosis in adolescents could be challenging considering that most of symptoms occur as part of the expected physiological hormonal imbalance of puberty. Therefore, different diagnostic criteria have been elaborated.

Q-switched Nd-YAG laser alone and in combination with innovative hyaluronic acid gels improve keratinocytes wound healing in vitro.

During the last years, several attempts have been accomplished to improve the wound healing. Device application aimed at enhancing skin ability to reconstruct its damaged sites through a proper dermal regenerative process. In particular, Q-switched Nd-YAG laser (Medlite C6 laser, Conbio, USA) applied with a fluence of 8 J/cm, a pulse width of 5 ns, and a spot size of 4 mm exerts a photo-mechanical action that improve skin repair.

Auxological and Endocrinological Features in Children With McCune Albright Syndrome: A Review.

McCune-Albright syndrome is a rare and challenging congenital sporadic disease involving the skin and skeletal and endocrine systems with a prevalence ranges from one in 100,000 to 1,000,000. In addition to the classical triad of fibrous dysplasia of bone, pigmented skin lesions and precocious puberty, other multiple endocrinological features, including hyperthyroidism, growth hormone excess, hypercortisolism, and hypophosphatemic rickets, have been reported. A brief review of the syndrome in children is here reported.

Early onset Charcot-Marie-Tooth neuropathy type 2A and severe developmental delay: expanding the clinical phenotype of MFN2-related neuropathy.

Charcot-Marie-Tooth (CMT) syndromes are a group of clinically heterogeneous disorders of the peripheral nervous system. Mutations of mitofusin 2 (MFN2) have been recognized to be associated with CMT type 2A (CMT2A). CMT2A is primarily an axonal disorder resulting in motor and sensory neuropathy.

The role of chemotherapy and surgical removal in the treatment of Choroid Plexus carcinomas and atypical papillomas.

Introduction: We performed a retrospective study on clinical assessment, tumor location, radiological imaging, histopathological characteristics, and therapeutic management of 7 patients affected by choroid plexus carcinoma (CPC) or atypical choroid plexus papilloma (ACPP) who have been observed in the last 12 years.

Methods: Four patients fulfilled the criteria for classification as ACPP and three cases as CPC. The median age of the patients at the diagnosis was 42 months (range 3-190 months).

Evaluation of a method based on coherence in aqueous systems and resonance-based isotherapeutic remedy in the treatment of chronic psoriasis vulgaris.

Psoriasis is a chronic skin disorder that exhibits three main features: lymphocytic infiltration into the dermis and epidermis, uncontrolled proliferation and abnormal differentiation of keratinocytes. In this study we have evaluated the effect of treatment with WHITE Holographic Bioresonance Method and a resonance-based isotherapeutic remedy on patients affected by chronic psoriasis vulgaris. The WHITE Holographic Bioresonance Method is based on the principles of electrodynamic coherence.

In vitro antiviral and immunomodulatory activity of arbidol and structurally related derivatives in herpes simplex virus type 1-infected human keratinocytes (HaCat).

Arbidol (ARB) is an antiviral drug that has broad-spectrum activity against a number of viral infections. To date, there are no specific data regarding its effects against a herpesvirus. Here, the in vitro antiviral effect of ARB and structurally related derivatives were evaluated in HaCat cells on different steps of herpes simplex virus type 1 replication: adsorption, entry and post-entry.

Effect of long-term GH treatment in a patient with CHARGE association.

CHARGE association is characterized by ocular Coloboma, Heart malformations, choanal Atresia, Retardation of growth and development, Genital abnormalities and inner and external Ear abnormalities. Growth failure is a frequent find mainly associated with feeding difficulties or systemic diseases. To date, GH deficiency has been reported in only few patients with CHARGE association however long-term effects of GH treatment, up to final height, have never been reported.

AlphaVBeta5 integrins mediates Pseudomonas fluorescens interaction with A549 cells.

Interaction of pathogenic bacteria with human cells is usually an essential step in the infection process. The bacterial invasion is stimulated by microbial binding to mammalian extracellular matrix proteins such as vitronectin, fibronectin or integrins. We have recently shown that some strains isolated from a clinical environment are able to grow at/or above 37°C.

What evidence exists to support antiviral treatment in children with chronic hepatitis B?

Many questions are unanswered on the optimal management of chronically HBV-infected children. Chronic hepatitis B is generally a mild disease in children; however, response to therapy is partial and limited to specific subgroups, and available drugs have no proven advantage on long-term course of disease versus no treatment, and are hampered by numerous limitations. Studies on the natural history of chronic hepatitis B and the long-term results of the therapeutic schedules adopted so far should be critically appraised.

Constitutional 11q14-q22 chromosome deletion syndrome in a child with neuroblastoma MYCN single copy.

Constitutional 11q deletion is a chromosome imbalance possibly found in MCA/MR patients analyzed for chromosomal anomalies. Its role in determining the phenotype depends on extension and position of deleted region. Loss of heterozygosity of 11q (region 11q23) is also associated with neuroblastoma, the most frequent extra cranial cancer in children.

Amygdalin analogues inhibit IFN-γ signalling and reduce the inflammatory response in human epidermal keratinocytes.

Peptide T (PT), an octapeptide fragment located in the V2 region of the HIV-1 gp120-coating protein, appears to be beneficial in the treatment of psoriasis. Our previous investigations suggest that keratinocytes play a key role in conditioning the therapeutic effects of PT in psoriasis. The aim of this study was to explore the effects of PT and the peptidomimetic natural products, Dhurrin and Prunasin, on the expression of the IL-6, IL-8, IL-23, HSP70 and ICAM-1 on IFN-γ and TNF-α-NHEK activated cells.

Design of inhibitors of influenza virus membrane fusion: synthesis, structure-activity relationship and in vitro antiviral activity of a novel indole series.

The fusion of virus and endosome membranes is an essential early stage in influenza virus infection. The low pH-induced conformational change which promotes the fusogenic activity of the haemagglutinin (HA) is thus an attractive target as an antiviral strategy. The anti-influenza drug Arbidol is representative of a class of antivirals which inhibits HA-mediated membrane fusion by increasing the acid stability of the HA.

Innate immune response in human keratinocytes infected by a feline isolate of Malassezia pachydermatis.

Malassezia pachydermatis is a normal inhabitant of canine and feline skin that can spread to other pets. The outer layer or epidermis is made up primarily of keratinocytes, which are capable of releasing various factors and expressing receptors that are significantly involved in the immune regulation. Little is known about the mechanism by which M.

The Helicobacter pylori protein HspB interferes with Nrf2/Keap1 pathway altering the antioxidant response of Ags cells.

Background: Helicobacter pylori infection causes chronic oxidative stress on gastric mucosa, thereby causing mucosal damage and increasing the risk of gastric adenocarcinoma. Nrf2 is an important transcription factor, regulating the antioxidant response in the cells. Nrf2 signaling is repressed by Keap1 at basal condition and induced by oxidative stress.

Polydatin, a natural precursor of resveratrol, induces β-defensin production and reduces inflammatory response.

It is well known that human keratinocytes produce the anti-microbial peptide β-defensin 2. Its production is enhanced by pathogenic microorganisms or other environmental stressors. In this study, we evaluated the effect of resveratrol, a polyphenol found in several dietary source as grape seed, and its natural precursor, polydatin on heat-stressed human keratinocytes.

Patenting Penicillium strains.

Penicillium species are a widespread source of biologically active compounds and enzymes which are exploited in biotechnologies. The ongoing discovery of new species, their biochemical and molecular characterization, and the application of the new findings in diverse industrial processes stimulate an increasing interest of patentees worldwide. An overview of the patents released in the last four years in agriculture, bioremediation, and in several industrial fields for the production of biofuels, food, cosmetics and pharmaceuticals is proposed for an exhaustive appreciation of the potential cues offered to inventors by these fungi.

Patented natural avocado sugar modulates the HBD-2 and HBD-3 expression in human keratinocytes through toll-like receptor-2 and ERK/MAPK activation.

Keratinocytes stimulated by microbial organisms secrete not only a variety of cytokines, chemokines and growth factors, but also antimicrobial peptides such as beta-defensins (HBDs) such as HBD-2 and HBD-3. AV119, a patented blend of avocado sugar, triggers the up-regulation of HBD-2 in skin epithelia upon contact with AV119, but the signalling mechanisms involved are not completely understood. The purpose of this study was to determine if AV119 was able to induce also the expression of HBD-3 in human keratinocytes.

Aurantoside J: a new tetramic acid glycoside from Theonella swinhoei. Insights into the antifungal potential of aurantosides.

The chemical investigation of an Indonesian specimen of Theonella swinhoei afforded four aurantosides, one of which, aurantoside J (5), is a new compound. The structure of this metabolite, exhibiting the unprecedented N-α-glycosidic linkage between the pentose and the tetramate units, has been determined through detailed spectroscopic analysis. The four obtained aurantosides have been tested against five fungal strains (four Candida and one Fusarium) responsible of invasive infections in immuno-compromised patients.

Dry powders based on PLGA nanoparticles for pulmonary delivery of antibiotics: modulation of encapsulation efficiency, release rate and lung deposition pattern by hydrophilic polymers.

Although few experimental studies have been handled so far to exploit the potential of poly(lactic-co-glycolic acid) (PLGA) nanoparticles (NPs) in the production of dry powders for antibiotic inhalation, there has been no comprehensive study on the role played by NP composition. In this work, we try to shed light on this aspect by designing and developing a pulmonary delivery system for antibiotics, such as tobramycin (Tb), based on PLGA NPs embedded in an inert microcarrier made of lactose, referred to as nano-embedded micro-particles (NEM). At nanosize level, helper hydrophilic polymers were used to impart the desired surface, bulk and release properties to PLGA NPs prepared by a modified emulsion-solvent diffusion technique.

Cell-growth and migration inhibition of human mesothelioma cells induced by 3-O-methylfunicone from Penicillium pinophilum and cisplatin.

Malignant pleural mesothelioma is a fatal malignancy linked to asbestos exposure. The main challenge for mesothelioma treatment is to go beyond the drug resistance, in particular against cisplatin (CDDP), one of the most used chemotherapeutic drug. 3-O-methylfunicone (OMF) is a metabolite produced by the fungus Penicillium pinophilum; its antiproliferative properties have been previously studied in vitro.

Captopril modulates acetylcholinesterase in human keratinocytes.

Human keratinocytes synthesize and secrete non-neuronal acetylcholine, which acts as a local cell signaling molecule, regulating functions like proliferation, cell adhesion, motility, desmosomal cell contact, and glandular activity. The keratinocyte acetylcholine axis is composed of the enzymes mediating acetylcholine synthesis (acetyltransferase) and degradation (acetylcholinesterase), and two classes of acetylcholine receptors. In this study we investigated the effect of captopril, an ACE-inhibitor, on acetylcholinesterase and acetylcholine secretion in human keratinocytes.