Enfermedad de Wilson rápidamente evolutiva y con presentación inusual / Wilson’s disease with unusual presentation and rapid evolution

Presentamos el caso de un paciente con enfermedad de Wilson que tuvo un comportamiento poco habitual. Previoa la aparición de las manifestaciones neurológicas, tuvo tos como único síntoma, sospechándose una discinesiarespiratoria como forma de presentación. La forma clínica de su enfermedad fue de tipo neurológico puro y sin evidencias de compromiso hepático. No hubo una respuesta satisfactoria al tratamiento instituido y la evolución fue rápida y fatal en poco tiempo.

We present a case of Wilson’s disease with an unusual course. Before the onset of neurological manifestations, cough was the only symptom, suggesting a respiratory dyskinesia as the form of presentation. The disease took a purely neurological type, without signs of hepatic compromise. There was no response to medical treatment, and the evolution was rapid and fatal.

Zinc-Induced Gastric Ulcer: Case Report on Two Patients with Wilson’s Disease.

Zinc has been developed as an effective and nontoxic therapy in Wilson’s disease. Zinc salts are generally well tolerated. Mild gastrointestinal discomfort is the major observed side effect and may be dependent on the zinc salt employed. Here, we report two Wilson’s disease patients who presented with severe gastric ulceration few months after beginning treatment with zinc acetate 50 mg three times a day. Our patients were not taking any ulcerogenic drugs and had no evidence of Helicobacter pylori infection. In both patients, zinc acetate was replaced by penicillamine and proton pump inhibitor therapy was initiated with complete resolution of gastrointestinal symptoms. To our knowledge, this is the first report of zinc acetate-induced gastric ulceration, which should be looked for in Wilson’s disease patients who develop abdominal discomfort while on this drug.

Evaluation of dental staining using a dentifrice containing chlorhexidine and zinc acetate. A doble blind randomizedclinical trial / Avaliação de dentifrício contendo clorexidina e acetato de zinco. Estudo clínico controlado randomizado duplo cego

Objective: The aim of this study was to evaluate whether the association of chlorhexidine with zinc acetate in dentifrices formulations could reduce the emergence of extrinsic tooth stain. As second outcome check the clinical gingival parameters. Methods: 30 volunteers were randomly divided into three groups: CHX+Z, consisting of 10 participants who used a dentifrice with 0.8% chlorhexidine gluconate and zinc acetate 1.16%; CHX group, with 10 participants who used a similar formulation dentifrice without zinc acetate, and the Placebo group also with 10 participants who used a dentifrice formulation similar but without the chlorhexidine and zinc acetate. Patients were assessed at baseline and 60 days when the indexes of plaque, gingival bleeding and staining were collected. Results: The results showed that by day 60, there was a decrease of both plaque index(PI) and the gingival index(GI) for all groups. Additionally, it was observed that both groups using dentifrice containing chlorhexidine, showed more stain than placebo. The CHX+Z group showed less stain compared to the CHX group, but the difference was not statistically significant. The difference between Placebo and Chlorhexidine Groups was statistically significant (p < 0.05) when considered the stain intensity and area plus intensity scores. The CHX+Z group was as efficient in PI and GI reduction as the CHX group. Conclusions: The association of chlorhexidine with zinc acetate showed no additional benefits regarding reducing the staining. The dentifrices containing chlorhexidine presented higher reduction of GI and PI when compared to Placebo group.

Objetivo: O objetivo deste estudo foi avaliar se a associação de clorexidina com acetato de zinco em dentifrícios poderia reduzir o aparecimento de manchas dentárias extrínsecas. Como segundo objetivo verificar os parâmetros clínicos gengivais. Materiais e métodos: 30 voluntários foram divididos aleatoriamente em três grupos: CHX + Z, composta por 10 participantes que usaram um creme dental com 0,8% de gluconato de clorexidina e acetato de zinco 1,16%; grupo CHX, com 10 participantes que usaram uma formulação de dentifrício semelhante, porém sem acetato de zinco, e o grupo do placebo também com 10 participantes que usaram uma formulação dentífrica semelhante, mas sem a clorexidina e sem acetato de zinco. Os pacientes foram avaliados no início do estudo e após 60 dias, quando foram coletados os índices de placa, sangramento gengival e manchas. Resultados: Os resultados revelaram que ao dia 60, havia uma redução em ambos os índices, de placa (IP) e do índice gengival (IG) em todos os grupos. Além disso, observou-se que em ambos os grupos que utilizou dentifrício contendo clorexidina, mostrou um maior índice de manchas maior do que o placebo. O grupo CHX + Z apresentou um índice de manchas menor em relação ao grupo

Doença de Wilson (degeneração hepatolenticular): revisão bibliográfica e relato de caso / Wilson’s disease (hepatolenticular degeneration): revision and case report

Este estudo faz, inicialmente, revisão dos aspectos mais atuais referentes a conceito, quadro clínico, diagnóstico e tratamento do distúrbio metabólico do cobre, definido como doença de Wilson. E relata o caso clínico de um jovem acometido de uma sequência de sintomas superpostos de origem gastrintestinal, neurológico e psiquiátrico. Pela multiplicidade e gravidade dos sintomas, teve o diagnóstico final de transtorno psicótico agudo polimórfico, com intensa inibição psicomotora. A partir de uma análise integrada dos exames já solicitados, suspeitou-se de um distúrbio metabólico de origem hereditária ou adquirida que justificasse simultaneamente os sintomas. O distúrbio da excreção do cobre, doença de Wilson, veio justificar toda a sintomatologia referida e foi confirmado a partir da dosagem sanguínea baixa de ceruloplasmina e da presença dos anéis de Kaiser-Fleischer na córnea do paciente.

It will be initially revised by the authors the most actual aspects of the concept, clinical situation, diagnosis and treatment concerning to a metabolic disturbance of the copper, Wilson?s disease. Afterwards it will be described the clinical case of a young man attacked of a sequence of superposed symptoms of gastrintestinal, neurological and psychiatric origin. For the multiplicity and gravity of the symptoms acute polimórfico with intense psicomotora inhibition had the final diagnosis of "psychotic Upheaval". Starting from an integrated analysis of the exams, it was suspected about a metabolic disturbance of hereditary or acquired origin that justify all the symptoms simultaneously. The disturbance of the excretion of copper, Wilson's disease, came to justify all the referred symptomatology and it was confirmed by the decrease sanguine dosage of ceruloplasmin, the presence of rings of Kayser-Fleischer in the córnea of the patient and of neurological lesion at the magnetic nuclear ressonance. The diagnosis of Wilson's disease in patients with simultaneous digestive (hepática cirrhosis), neurological and inexplicable psychiatric disturbances will always have to be faneed because the precocious treatment will mainly prevent serious and permanent organic damages for the liver and brain. The specific treatment was initiated and the maintenance of exactly has provoked significant improvements and a gradual new outbreak of the symptoms reintegrating the patient the family and the society.

Doença de Wilson: apresentação clínica, diagnóstico e tratamento / Wilson's disease: clinical presentation, diagnosis and treatment

A doença de Wilson é um erro inato do metabolismo do cobre causado por uma mutação no gene ATP7B, responsável por seu transporte. É uma doença de herança autossômica recessiva, caracterizada pela deposição excessiva de cobre principalmente no fígado e no cérebro. Clinicamente, os pacientes apresentam manifestações hepáticas, neurológicas e psiquiátricas. O diagnóstico pode ser feito quando as seguintes características estiverem presentes: anéis de Kayser-Fleischer na córnea, diminuição dos níveis plasmáticos de ceruloplasmina e sintomas neurológicos típicos. A prevenção de danos permanentemente severos depende do reconhecimento e diagnóstico precoces pelo médico, seguidos de tratamento apropriado. A doença de Wilson pode ter prognóstico excelente, desqe que o tratamento seja feito durante toda a vida.

Wilson's disease is an inborn error of copper metabolismo caused by a mutation to the cooper-transporting gene ATP7B. This disease has an autosomal recessive mode of inheritance, and is characterized by excessive cooper deposition, predominantly in the liver and brain. Clinically, patients usually present hepatic, neurologic or psychiatric manifestations. The diagnosis can be done when these symptoms are present: Kayser-Fleischer rings, low serum ceruloplasmin levels and typical neurological symptoms. The prevention of severe permanente damage depends upon early recognition and diagnosis by the physician, followed by appropriate anticopper treatment. Wilson's disease it can have an excellent prognosis since that treatment either for all the life.

Estimation of hydrogen sulphide in the human lymphocytes.

Hydrogen sulphide (H2S), a signaling gasotransmitter and a potent vasorelaxant is endogenously produced by the enzymes cystathionine-beta-synthase (CBS) and cystathionine-gamma-lyase (CSE). CBS is a predominant source of H2S in the central nervous system, while CSE is the major H2S producing enzyme in the brain and other nervous tissues. Though the expression of these enzymes in the blood lymphocytes is known, H2S formation in the lymphocytes has not been reported so far. In the present study, H2S levels in the lymphocytes of healthy control subjects were estimated, after suitable modifications in a routine method [Stipanuk M H & Beck P W (1982) Biochem J 206, 267-277] used for detecting tissue levels of H2S. In this method, homocysteine (Hcys) due to its higher solubility was used as the substrate in place of L-cysteine and NaOH was used in place of zinc acetate to increase the entrapment of H2S in the central well. A mean H2S level of 11.64 +/- 6.36 microM/min/mg protein was detected in the lymphocytes of 8 subjects (mean age, 24 +/- 2; 2 male, 6 female). The modified method was found to be more sensitive for H2S estimation in human lymphocytes. As endogenous H2S is reported to be involved in the pathogenesis of various cardiovascular and pulmonary diseases, the levels of H2S in lymphocytes can be a marker of the endogenous tissue levels.

Effect of zinc supplementation on morbidity due to acute diarrhoea in infants and children in Sanaa, Yemen. A randomized controlled double blind clinical trial

To study the impact of zinc administration on the morbidity and mortality attributed to diarrhoea among children less than 5 years old. The study design was a randomized double blinded controlled clinical trial, held at Elsabeen Hospital for Maternity and Childhood, Sana'a, Yemen. The study was conducted during the period September 2005 to October 2006 on 180 children less than 5 years old with acute diarrhoeal episodes. They were randomly allocated to two groups; one of them received a placebo and the other received zinc acetate syrup for 14 days. Both groups were followed up for 2 months. Zinc was able to decrease the mean number of diarrhoeal episodes: 1.39 in the intervention group versus 2.59 in the control group. It also reduced the mean frequency of stools per day in each attack [3.57 in the intervention group versus 5.47 in the control group] and the volume of stool in each attack during the follow-up period. Moreover, zinc was significantly more palatable. We can conclude from the study that administration of zinc for two weeks during acute diarrhoeal episodes could decrease the incidence of further diarrhoeal episodes as well as the severity of these episodes. The lower rates of child morbidity with zinc treatment represent substantial benefits from a simple and inexpensive intervention that can be incorporated in existing efforts to control diarrhoeal disease

Anti-inflammatory, antinociceptive and ulcerogenic activity of a zinc-diclofenac complex in rats

We investigated the anti-inflammatory, antinociceptive and ulcerogenic activity of a zinc-diclofenac complex (5.5 or 11 mg/kg) in male Wistar rats (180-300 g, N = 6) and compared it to free diclofenac (5 or 10 mg/kg) and to the combination of diclofenac (5 or 10 mg/kg) and zinc acetate (1.68 or 3.5 mg/kg). The carrageenin-induced paw edema and the cotton pellet-induced granulomatous tissue formation models were used to assess the anti-inflammatory activity, and the Hargreaves model of thermal hyperalgesia was used to assess the antinociceptive activity. To investigate the effect of orally or intraperitoneally (ip) administered drugs on cold-induced gastric lesions, single doses were administered before exposing the animals to a freezer (-18ºC) for 45 min in individual cages. We also evaluated the gastric lesions induced by multiple doses of the drugs. Diclofenac plus zinc complex had the same anti-inflammatory and antinociceptive effects as diclofenac alone. Gastric lesions induced by a single dose administered per os and ip were reduced in the group treated with zinc-diclofenac when compared to the groups treated with free diclofenac or diclofenac plus zinc acetate. In the multiple dose treatment, the complex induced a lower number of the most severe lesions when compared to free diclofenac and diclofenac plus zinc acetate. In conclusion, the present study demonstrates that the zinc-diclofenac complex may represent an important therapeutic alternative for the treatment of rheumatic and inflammatory conditions, as its use may be associated with a reduced incidence of gastric lesions.

Differential effects of lead and zinc on inhibitory avoidance learning in mice

We studied the effects of chronic intoxication with the heavy metals lead (Pb2+) and zinc (Zn2+) on memory formation in mice. Animals were intoxicated through drinking water during the pre- and postnatal periods and then tested in the step-through inhibitory avoidance memory task. Chronic postnatal intoxication with Pb2+ did not change the step-through latency values recorded during the 4 weeks of the test (ANOVA, P>0.05). In contrast, mice intoxicated during the prenatal period showed significantly reduced latency values when compared to the control group (day 1: q = 4.62, P<0.05; day 7: q = 4.42, P<0.05; day 14: q = 5.65, P<0.05; day 21: q = 3.96, P<0.05, and day 28: q = 6.09, P<0.05). Although chronic postnatal intoxication with Zn2+ did not alter a memory retention test performed 24 h after training, we noticed a gradual decrease in latency at subsequent 4-week intervals (F = 3.07, P<0.05), an effect that was not observed in the control or in the Pb2+-treated groups. These results suggest an impairment of memory formation by Pb2+ when the animals are exposed during the critical period of neurogenesis, while Zn2+ appears to facilitate learning extinction

Prótesis completa: impresiones (trans) / Complete dentures: impressions

La toma de impresiones al paciente totalmente desdentado tiene como objetivo registrar la anatomía de la cavidad bucal para reproducirla en escayola, y poder confeccionar las prótesis a su medida. Exponemos en este artículo los criterios recomendados para conseguir unas buenas impresiones. Aportamos pequeñas sugerencias que pueden facilitar al profesional la adquisición de la destreza necesaria para obtener unas impresiones correctas

Effect of Cadmium Chloride on the Mature Rat Testis / 대한비뇨기과학회지

For the study of the effects of cadmium chloride and zinc acetate on the testis and relationship between regeneration of degenerated testis and mast cells, the Sprague-Dawley strain albino rats weighing from 180gm to 250 gm were used. Twenty seven rate were injected with cadmium chloride in the doses of 0.03 mM per kg body weight and twenty seven rats were injected the same amount of cadmium chloride and 3.0 mM per kg body weight of zinc acetate simultaneously. The testes were removed under ether anesthesia at different time intervals (3 hours, 6 hours, 12 hours, 1 day, 2 days, 4 days, 1 week, and 4 weeks) and the sections were stained with hematoxylin and eosin, and toluidine blue, respectively. Through the histological and gross observation the following results were obtained. From 3 hours after the injection of cadmium chloride, the testis began to have degenerative changes in the interstitium and tubules changes began at 2 days after the injection of cadmium chloride. Active regeneration of the damaged interstitium was recognized at 2 weeks after the injection of cadmium chloride while the tubules were still in the state of necrosis. No regenerative changes of the tubular epithelium were observed throughout the experiment. The first appearance of tissue mast cells in the interstitium was observed at 2 days after the injection of cadmium chloride and the cells were increased in number as the time elapsed. The simultaneous injection of zinc acetate prevented the destruction of testes caused by toxic effect of cadmium chloride and no tissue mast cells were found in the interstitium of the testes throughout the experiment. The testes were diminished in the weight and size after the treatment of cadmium chloride but those mgross change were not found in the testes treated with zinc acetate simultaneously.