Impact of oral astringent stimuli on surface charge and morphology of the protein-rich pellicle at the tooth-saliva interphase.

The proteinaceous pellicle layer, which develops upon contact with saliva on the surface of teeth, is important for the formation of oral biofilms and for the protection of teeth from abrasion and chemically induced erosion. Astringent food ingredients comprising polyphenols, cationic macromolecules, and multivalent metal salts are known to interact with the pellicle. However, astringent-induced changes in the physicochemical properties of the tooth-saliva interphase are not yet completely understood. Here we provide comprehensive insights into interfacial charging, ultrastructure, thickness, and surface roughness of the pellicles formed on the model substrates silicon oxide (SiO2), Teflon® AF, and hydroxyapatite, as well as on bovine enamel before and after incubation with the astringents epigallocatechin gallate, tannic acid, iron(III) salt, lysozyme, and chitosan. Quartz crystal microbalance with dissipation monitoring demonstrated viscous behavior of untreated pellicles formed in vitro on the different materials. Electrokinetic (streaming current) measurements revealed that cationic astringents reverse the charge of native pellicles, whereas polyphenols did not change the charge under physiological pH condition. In addition, transmission electron microscopy and atomic force microscopy showed a concentration-dependent increase in average film thickness and pellicle surface roughness as induced by astringents. These multifaceted alterations of the salivary pellicle may come along with an increase in roughness perceived on the teeth, which is part of the complex sensations of oral astringency.

Efficacy and safety of oral zinc sulfate in the prevention of chemotherapy-induced oral mucositis: Protocol for a meta-analysis of randomized controlled trials.

BACKGROUND: Oral mucositis has been an extremely serious complication resulted from cytotoxic effects of the chemotherapy among cancer patients. Several randomized controlled trials investigated the efficacy of zinc sulfate in prevention of this morbid condition among cancer patients undergoing chemotherapy, however conclusive findings has not yet been generated. This systematic review will assess the efficacy and safety of oral zinc sulfate for chemotherapy-induced oral mucositis. METHODS: We will electronically search all potential citations in PubMed, EMBASE, and Cochrane Central Register of Controlled Trials (CENTRAL) from their inception to April 2018. The randomized controlled trials, which investigated the efficacy of oral zinc sulfate for chemotherapy-induced oral mucositis will be considered. We will assign 2 independent investigators to perform search, screen citations, extract data, and appraise risk of bias. And then, the primary investigator will adopt RevMan 5.3 software to complete all statistical analyses. ETHICS AND DISSEMINATION: The findings from this systematic review and meta-analysis will be submitted to a peer-reviewed journal for publication. Moreover, we will disseminate all results in any topic-related conference. REGISTRATION NUMBER: This protocol has been registered with a number of CRD42018093605.

A multicenter randomized, controlled clinical trial comparing the use of displacement cords, an aluminum chloride paste, and a combination of paste and cords for tissue displacement.

STATEMENT OF PROBLEM: Gingival recession after soft tissue displacement for impression making in fixed prosthodontics may pose a problem for treatment success in the esthetic areas of the mouth. Knowledge about the soft tissue reaction of common gingival displacement methods is limited. PURPOSE: The purpose of this clinical randomized controlled trial (RCT) was to evaluate changes in the marginal soft tissue height with 3 different gingival tissue displacement techniques for definitive impression making of natural teeth. MATERIAL AND METHODS: A total of 67 individuals were randomized to 3 groups. In test group 1 (P; n=22), only aluminum chloride paste was used to displace the gingiva. In test group 2 (CP; n=23), a cord was inserted, and aluminum chloride paste was also used. In the control group (C; n=22), 2 cords were used to displace the gingiva (double-cord technique). Clinical measurements of the gingival position were made before treatment began and at 30 ±10 days after prosthesis delivery. Study casts were fabricated at different stages of the treatment, standardized photographs were made, and changes in the buccal gingival position were measured using graphics editing software. In addition, the participants' perception of the clinical procedure and the technicians' evaluation of the die preparation were recorded. One-way ANOVA models were applied to compare the response variables among the groups: (a) the position of the gingival margin (millimeters), (b) mean probing pocket depth (millimeters), (c) gingival thickness (millimeters), (d) amount of keratinized tissue (millimeters), and (e) mean changes in gingival margin height (millimeters). Unpaired t tests were also used to compare the mean values between groups. For comparisons between different categories, chi-square tests were performed (α=.05 for all tests). RESULTS: In the period between impression and delivery, a minor gain in gingival height of 0.058 mm (±0.13 SD) for P and 0.013 mm (±1.19 SD) for CP. However, a minor gingival recession of 0.049 mm (±0.13 SD) was reported for group C. The results for all groups showed that 21% of abutment teeth gained >0.1 mm in gingival height, 58% had stable gingival height (0 ±0.10 mm), 21% showed minor gingival recession (0.1 to 0.5 mm), and no abutment teeth showed moderate or severe gingival recession (>0.5 mm). The incidence of minor gingival recession was 8% in group P, 23% in group CP, and 32% in group C (P=.015). Fifteen participants (24%) experienced some discomfort after the procedure. The differences between the groups were not significant (P>.05). The laboratory technicians found the definitive die preparation significantly more challenging for group P (visual analog scale [VAS], 79) and CP (VAS, 82) than group C (mean VAS, 93; P=.003). CONCLUSIONS: Minor or moderate gingival recession (<1 mm) is more likely to occur when conventional cords are used during impression making. However, the laboratory technicians found the die preparation significantly less challenging when the double-cord technique was used than when impressions were made using the paste displacement technique.

Over-the-counter treatments for acne and rosacea.

Acne and rosacea are common inflammatory processes historically classified in the same disease category, but evolving understanding of their disparate pathophysiology and exacerbating factors have generated an enormous armamentarium of therapeutic possibilities. Patients seek over-the-counter therapies first when managing cutaneous disease; therefore, this review defines ingredients considered to be effective over-the-counter acne and rosacea products, their mechanisms, and safe formulations, including botanical components, oral supplements, and other anecdotal options in this vast skin care domain.

Efficacy of combination therapy of oral zinc sulfate with imiquimod, podophyllin or cryotherapy in the treatment of vulvar warts.

AIM: Zinc sulfate is beneficial in the treatment of epithelial warts. We conducted this study to compare the efficacy of combination therapy of oral zinc sulfate with conventional treatments in the treatment of vulvar warts. MATERIAL AND METHODS: This study was a randomized controlled trial. The sample size was 42 in each group. Women aged 20-50 years were placed by the block randomized method into six groups: the podophyllin-, imiquimod- and cryotherapy-treated groups, and another three groups receiving 8-week combination therapy of 400 mg oral zinc sulfate with one of the above-mentioned treatments. Data were analyzed using anova and Fischer's exact test with spss16. RESULTS: A total of 228 patients were recruited and completed the study in six treatment groups. No significant difference was observed in the response to treatment among these groups. Relapse after 6 months was significantly higher in the podophyllin-, imiquimod- and cryotherapy-treated patients compared to patients receiving these treatments in combination with oral zinc sulfate (P<0.05). CONCLUSIONS: Combined therapy of oral zinc sulfate with conventional treatments of vulvar warts appears to reduce the relapse rate.

Is topical zinc effective in the treatment of melasma? A double-blind randomized comparative study.

BACKGROUND AND OBJECTIVES: Zinc plays a role in skin health, and preliminary data have shown its beneficial effects for melasma. We compared the effect of topical zinc with that of hydroquinone as the standard treatment on severity of melasma. PATIENTS AND METHODS: Ninety-three women with melasma were randomized to receive zinc sulfate 10% or hydroquinone 4% solutions once daily for 2 months. They were followed for an additional 3 months while using sunscreen. The severity of melasma was assessed at baseline and at 2 and 5 months using the Melasma Area and Severity Index (MASI). RESULTS: Eighty-two patients completed the study. The MASI score fell significantly in both groups, but a greater decrease was seen in those who received hydroquinone (43.5 ± 15.5% vs 18.6 ± 20.8%, p < .001). Postinflammatory pigmentation occurred in 5.2% of the zinc group and irritation in 30.9% of the hydroquinone group. CONCLUSION: Topical zinc therapy is not highly effective in reducing the severity of melasma, but further trials are needed to determine whether adding zinc to current topical treatments could improve treatment response.

Zinc sulphate administered by transdermal iontophoresis improves breaking strength of surgical wounds in skin of alloxan-induced diabetic rats

PURPOSE:To investigate the effect of zinc sulphate administered by transdermal iontophoresis (TDI) on mechanical resistance of surgical wounds performed in the skin of diabetic rats. METHODS:One hundred and sixty male Wistar rats weighing approximately 250g were submitted to an incision surgery at the anterior region of abdomen and randomly distributed into four experimental groups with 40 non-diabetic control animals (G1) and 40 untreated diabetic animals (G2), both without any treatment of incisions; 40 non-diabetic animals (G3) and 40 untreated diabetic animals (G4), both with incisions treated with zinc sulphate, administered for a period of four consecutive days after surgery, in sessions of ten minutes duration, using a continuous-current electrostimulator (Zn + TDI). Each experimental group was further divided into four subgroups with ten rats each to be evaluated on the 4th, 7th, 14th, and 21st day after surgery. In each period were analyzed clinical and laboratory from the animals, and measured the breaking strength and hydroxyproline content (OH-P) of the skin scars. RESULTS: Breaking strength (BS) was significantly reduced (p<0.05) in skin scars of untreated diabetic rats (G2) on the 7th, 14th, and 21st postoperative days when compared to non-diabetic control rats (G1). In contrast, BS in skin scars of non-diabetic and untreated diabetic rats (G3, G4) treated with Zn + TDI showed significant increase (p<0.05) in those periods when compared with their respective controls with untreated incisions. The OH-P content of the scars did not show statistically significant variation in all studied groups at four different times evaluated after surgery. CONCLUSIONS: Zinc sulphate administered by transdermal iontophoresis had beneficial effect on the mechanical resistance of scars produced in the skin of diabetic rats. This therapeutic may have potential to reduce the complications observed in surgical wounds of the skin in diabetic subjects, mainly in most vulnerable stages of incisions to dehiscences, leakages and infections.

Zinc sulphate administered by transdermal iontophoresis improves breaking strength of surgical wounds in skin of alloxan-induced diabetic rats.

PURPOSE: To investigate the effect of zinc sulphate administered by transdermal iontophoresis (TDI) on mechanical resistance of surgical wounds performed in the skin of diabetic rats. METHODS: One hundred and sixty male Wistar rats weighing approximately 250 g were submitted to an incision surgery at the anterior region of abdomen and randomly distributed into four experimental groups with 40 non-diabetic control animals (G1) and 40 untreated diabetic animals (G2), both without any treatment of incisions; 40 non-diabetic animals (G3) and 40 untreated diabetic animals (G4), both with incisions treated with zinc sulphate, administered for a period of four consecutive days after surgery, in sessions of ten minutes duration, using a continuous-current electrostimulator (Zn + TDI). Each experimental group was further divided into four subgroups with ten rats each to be evaluated on the 4th, 7th, 14th, and 21st day after surgery. In each period were analyzed clinical and laboratory from the animals, and measured the breaking strength and hydroxyproline content (OH-P) of the skin scars. RESULTS: Breaking strength (BS) was significantly reduced (p<0.05) in skin scars of untreated diabetic rats (G2) on the 7th, 14th, and 21st postoperative days when compared to non-diabetic control rats (G1). In contrast, BS in skin scars of non-diabetic and untreated diabetic rats (G3, G4) treated with Zn + TDI showed significant increase (p<0.05) in those periods when compared with their respective controls with untreated incisions. The OH-P content of the scars did not show statistically significant variation in all studied groups at four different times evaluated after surgery. CONCLUSIONS: Zinc sulphate administered by transdermal iontophoresis had beneficial effect on the mechanical resistance of scars produced in the skin of diabetic rats. This therapeutic may have potential to reduce the complications observed in surgical wounds of the skin in diabetic subjects, mainly in most vulnerable stages of incisions to dehiscences, leakages and infections.

The efficacy of zinc for treatment of chronic prostatitis.

AIM: to investigate the efficacy of zinc supplementation in chronic prostatitis treatment. METHODS: present randomized clinical trial was conducted on 120 patients with diagnosis of chronic prostatitis (IIIA NIH) after preliminary evaluation and ruling out other conditions. The study group received oral zinc sulfate 220 mg daily as capsule without any other supplements. The control group received placebo. Subjects were examined for NIH-CPSI scores every 4 weeks for 12 weeks. RESULTS: 101 subjects completed the study. There were no statistically significant differences in scores and sub-scores of NIH-CPSI between groups before intervention. Decline in the score and sub-scores were more prominent in case group after beginning of the study; though the differences were not statistically significant. Furthermore, the differences in total score and pain score at 12 weeks follow was statistically significant (p=0.003 and p=0.02, respectively). CONCLUSION: zinc supplements may benefit in management of patients with chronic prostatitis NIH-IIIA. It can be attributable to anti-bacterial and immuno-modulatory functions of organic zinc in the body.

Complications of cosmetic wide conjunctivectomy combined with postsurgical mitomycin C application.

PURPOSE: To raise awareness among clinicians about the risk of severe complications after cosmetic conjunctivectomy plus postsurgical topical mitomycin C (MMC) treatment. METHODS: We retrospectively reviewed the medical records of 48 consecutive patients who received cosmetic wide conjunctivectomy plus postsurgical topical MMC treatment to eliminate conjunctival injection and visited our outpatient clinic between January 2009 and January 2011. RESULTS: Forty-four patients (91.67%) developed complications after cosmetic wide conjunctivectomy plus postsurgical topical MMC treatment. Complications included chronic conjunctival epithelial defects (22 patients), scleral thinning with (19 patients) or without (2 patients) calcified plaques, fibrovascular conjunctival adhesion at the muscle insertion site (15 patients), chronic dysfunctional tear syndrome (11 patients), avascular zones (10 patients), abnormal vessel growth (5 patients), lymphangiectasis (3 patients), adhesions of Tenon capsule and the conjunctiva at the extraocular muscle insertion site (1 patient), extraocular muscle fiber exposure (1 patient), and diplopia (9 patients). The mean time between surgery and the onset of diplopia was 1.67 months (range, 1-3 months), and the mean deviation was 11.0 prism diopters (range, 0-25 prism diopters) in the primary position. Three patients underwent strabismus surgery due to incomitant esotropia. Eight patients underwent calcified plaque removal because of severe progression of the calcification. Five patients underwent conjunctival flap operation because of severe progressive scleral thinning. CONCLUSIONS: Cosmetic wide conjunctivectomy, which has been advertised as a simple and effective surgical option for the treatment of conjunctival injection, has caused disruption in the normal physiology of the ocular surface, which leads to various devastating complications.

Regional conjunctivectomy with postoperative mitomycin C to treat chronic hyperemic conjunctiva.

PURPOSE: To introduce a new surgical technique to treat persistent irreversible hyperemic conjunctiva and evaluate the clinical results. METHODS: A total of 571 patients with chronic conjunctival injection refractory to medical treatment were included. During surgery of the medial bulbar area, the conjunctiva and Tenon capsule were excised from the limbus to near the plica semilunaris. The lateral conjunctiva and Tenon capsule were removed from the limbus to beside the lateral canthus. Mitomycin C 0.02% was applied 4 times daily for 2 to 5 days postoperatively. The complications and surgical results of regional conjunctivectomy were evaluated. RESULTS: A total of 1815 ocular regions of 571 patients underwent regional conjunctivectomy. The mean period of conjunctival injection was 9.9 ± 6.9 years. The patients were followed for 2 to 27 months (average, 12.9 months). The overall postoperative satisfaction rate was 94.6%; 328 regions (18.1%) underwent surgical revision for growth of fibrovascular scar tissue. No serious complications developed intraoperatively. Postoperative complications included intraocular pressure elevations (98 of 571; 17.2%), development of granuloma (153 of 1815; 8.4%), transient subjective visual decreases (43 of 571; 7.5%), pigment deposition (96 of 1815; 5.3%), scleral calcification (70 of 1815; 3.9%), and diplopia because of adhesion esotropia (9 of 571; 1.6%). All complications were managed surgically and/or medically. No permanent deficits resulted. CONCLUSIONS: Regional conjunctivectomy with postoperative mitomycin C 0.02% can be useful to successfully treat patients with chronic hyperemic conjunctivae refractory to medical treatment.

Aluminum chloride hexahydrate in a salicylic acid gel base: a case series of combination therapy with botulinum toxin type A for moderate to severe hyperhidrosis.

Hyperhidrosis is a common condition that has a tremendous impact on the quality of life of patients. For moderate to severe hyperhidrosis, topical aluminum chloride hexahydrate (AC), iontophoresis, and botulinum toxin type A injections are first-line therapies. Botulinum toxin type A has been a useful addition to the hyperhidrosis armamentarium and typically is utilized when topical therapy or iontophoresis have failed. Although highly effective for most patients, there remains a subset of patients who do not completely respond to botulinum toxin type A injections. For these patients, combination therapy with AC can greatly improve patient response. We present a case series of 10 patients with hyperhidrosis and a history of partial response to botulinum toxin type A monotherapy. With the addition of AC 15% in a salicylic acid 2% gel base, 5 patients achieved 75% to 100% reduction in sweating and 5 patients achieved 100% reduction in sweating. Aluminum chloride hexahydrate in a salicylic acid gel base offers a novel and effective topical therapy in combination with botulinum toxin type A for patients with moderate to severe hyperhidrosis.

The effects of ingested aluminium on brain cytochrome oxidase activity / Efectos de la ingestión de aluminio sobre la actividad de la oxidasa citocrómica del cerebro

Aluminium has a unique combination of physical and chemical properties which has enabled man to put this metal to very wide and varied use. However, prolonged exposure to aluminium ions may lead to adverse health effects. In this study, we evaluated the effects of dietary aluminium on the protein composition and the intrinsic activity of cytochrome oxidase (COX) for brain mitochondria. New Zealand white rabbits were maintained on a diet of commercial rabbit pellets and distilled water for a period of 12 weeks. For the experimental group, AlCl3, 330mg/kg/L was added to the drinking water. When compared to the control, mitochondria isolated from the brains of the AlCl3 fed rabbits showed no change in Km but an approximate 35% decrease in both the low and high affinity Vmax values. Also, whereas the protein composition of the mitochondria from both sources appeared to be normal, isolation of highly purified COX proved to be difficult and for the AICI3 fed rabbits, a number of the enzyme's low molecular weight subunits were absent. These results appear to confirm a relationship between long term aluminium consumption and low brain COX activity; they further suggest that an altered COX structure may be the cause of the low enzymic activity.

El aluminio posee una combinación única de las propiedades físicas y químicas que ha permitido al ser humano hacer un uso amplio y variado de este metal. Sin embargo, un número de estudios recientes, sugiere que la exposición prolongada a los iones de aluminio puede tener efectos nocivos sobre la salud. En el presente estudio, evaluamos los efectos del aluminio dietético sobre la composición proteínica y la actividad intrínseca de la oxidasa citocrómica (COX) para la mitocondria cerebral. Conejos blancos de Nueva Zelanda, fueron mantenidos con una dieta de alimento para conejos y agua destilada por un período de 12 semanas. Para el grupo experimental AlCl3, 330mg/kg/L fueron añadidos al agua potable. En comparación con el grupo de control, las mitocondrias aisladas de los cerebros de los conejos alimentados con AlCl3 no mostraron cambios en Km pero hubo una disminución de aproximadamente 35% tanto en los valores Vmax de baja y alta afinidad. Por otro lado, mientras que la composición proteica de las mitocondrias de ambas fuentes parecía ser normal, resultó difícil aislar el COX altamente purificado y un número de enzimas de subunidades de bajo peso molecular MMMM estuvieron ausentes. Estos resultados parecen confirmar una relación entre el consumo de aluminio a largo plazo y la baja actividad del COX del cerebro. Asimismo, sugieren que una alteración de la estructura del COX puede ser la causa de una baja actividad enzimática.

An open-label trial of the efficacy of 15% aluminum chloride in 2% salicylic acid gel base in the treatment of moderate-to-severe primary axillary hyperhidrosis.

Primary focal hyperhidrosis (HH) is a chronic disorder of excessive sweating. A single-center, open-label study was performed to determine the efficacy and safety of 15% aluminum chloride (AC) in 2% salicylic acid gel base (SAGB) Hydrosal in adults with moderate-to-severe primary axillary HH. Thirty subjects were given 15% AC in 2% SAGB to apply to their bilateral axillae nightly for the first week, then twice-weekly application as tolerated. The primary objective was change in mean Hyperhidrosis Disease Severity Score (HDSS) score from baseline to week 4. Participants who achieved an HDSS score < 2 were designated as "responders." Mean change in HDSS from baseline to week 4 was 1.32 (p = 0.0001). At week 4, 21 of 29 (72%) were responders, and at week 12, 18 of 25 (72%) were responders. Based on these results, 15% AC in 2% SAGB may be an effective, high-strength AC topical therapy for treatment of patients with moderate-to-severe axillary HH.

Lack of classic histology should not prevent diagnosis of necrolytic acral erythema.

We describe a patient who was referred for management of psoriasis unresponsive to treatment. Given the predominantly acral distribution of the patient's rash and his known diagnosis of hepatitis C, we considered the diagnosis of necrolytic acral erythema (NAE) and empirically began treatment with oral zinc sulfate. At follow-up 3 weeks later, the patient had exceptional improvement in his cutaneous disease, supporting our diagnosis of NAE. NAE is a recently described entity, and limited information is known regarding its etiology and pathogenesis; however, previous reports have been fairly consistent regarding the clinical and histopathologic presentation. We describe a patient without the classic histologic or clinical findings of previously described cases of NAE. We believe that it is important to keep the diagnosis of NAE in one's differential for a predominantly acrally distributed rash. Furthermore, we recommend additional work-up, including hepatitis C status and zinc levels, as well as possible treatment with oral zinc sulfate in patients with a potential diagnosis of NAE.

Superiority of thoracoscopic sympathectomy over medical management for the palmoplantar subset of severe hyperhidrosis.

Severe hyperhidrosis is a disabling disorder whose management is controversial. Medical treatment consists of topical aluminum chloride, oral anticholinergics, ionotophoresis, and botulinum toxin A (Botox) injections. Despite the minimally invasive nature of thoracoscopic sympathectomy, there is a common perception that surgery is only a "last resort." The palmoplantar subtype of hyperhidrosis is particularly problematic for patients professionally and socially. The purpose of our study was to determine the safety, efficacy, and side effects of the various medical treatments vs. bilateral thoracoscopic sympathectomy (BTS) for palmoplantar hyperhidrosis. Consecutive patients (n = 192) were selected based on massive palmar sweating, similar level of plantar sweating, bimodal onset in early childhood or puberty, and exacerbation with ordinary hand lotion. A prospective cohort of 47 patients underwent medical treatment with their responses monitored on a prospective basis, and 145 patients underwent retrospective evaluation of their medical treatment based on their histories. Patients whose medical treatments failed or resulted in intolerable side effects were offered outpatient BTS surgery at the T2-T3 level. Of the 47 prospective patients, 46 received topical aluminum chloride, 40 anticholinergics, six iontophoresis, and 45 BTS surgery. Only one patient was successfully treated with aluminum chloride (2.2%) and one successfully treated with anticholinergics (2.5%), and these did not undergo surgery. Iontophoresis was not successful in any prospectively followed patient. BTS was effective in curing palmar hyperhidrosis in 100% of patients. The superiority of BTS vs. topical aluminum chloride, anticholinergics, and iontophoresis to successfully treat palmar hyperhidrosis was highly statistically significant (p < 0.001). For the retrospective group of 145 patients, 89 had been treated with topical aluminum chloride, 38 with oral anticholinergics, 31 with iontophoresis, eight with Botox, one with no medical treatment, and 144 with BTS surgery. All medical treatments failed with the exception that one patient was satisfied with anticholinergic treatment (2.6%), and this patient did not undergo BTS. BTS was successful in curing bilateral palmar hyperhidrosis in 99.3% (one unilateral failure due to adhesions). BTS was superior in treating palmar hyperhidrosis compared to aluminum chloride, anticholinergics, iontophoresis, and Botox (p < 0.001). The medically treated patients suffered significant side effects ranging from local stinging, cracking, and blistering to xerostomia, xerophthalmia, and blunted mentation. Overall, compensatory hyperhidrosis (CH) was present in 56% of patients undergoing BTS, but only 3.2% of BTS patients had severe CH with significant discomfort; all were men. There were no other significant operative complications. The safety and overwhelming efficacy of BTS compared to medical management of severe palmoplantar hyperhidrosis is demonstrated. Rather than being a "last resort," BTS can be confidently recommended as first-line treatment for the typical, severe form of palmoplantar hyperhidrosis.

[Wilson's disease in paediatric age: diagnosis and treatment. Recent advances]. / Diagnosi e terapia del morbo di Wilson in età pediatrica. Recenti progressi.

Wilson's disease is an autosomal-recessive disorder caused by mutation in the ATP7B gene, with resultant impairment of biliary excretion of copper. Subsequent copper accumulation, first in the liver but ultimately in the brain and other tissues, produces clinical manifestations that may include hepatic, neurological, psychiatric, ophthalmological, and other derangements. This article discusses the recent progress in diagnosis and treatment of this disease in paediatric age.