Suplementação mineral e vitamínica em doenças crônicas e de convalescença / Mineral and vitamin supplementation in chronic diseases and convalescence

A síndrome de fadiga crônica (SFC) é uma condição clínica que, apesar de muito prevalente, tem tratamento controverso. A suplementação com substratos como glutamina e vitaminas pode atuar como adjuvante terapêutico. Os autores descrevem um medicamento que pode atender essa finalidade, composto por glutamina 200mg, glutamato de cálcio 250mg, cloridrato de piridoxina 20mg e fosfato de ditetraetilamônio 6mg. São descritas também as ações de cada um dos componentes, e como podem auxiliar na terapêutica da SFC e em períodos de convalescença em diversas condições.

The chronic fatigue syndrome (CFS) is a clinical condition which, although highly prevalent, treatment is controversial and supplementation of substrates such as glutamine and vitamins can act as therapeutic adjuvant. A drug composition that can serve this purpose, the composition is glutamine 200mg, 250mg calcium glutamate, 20mg pyridoxine hydrochloride and phosphate ditetraetilammonium 6mg is described. Also described the actions of each component and how they can assist in the treatment of CFS and in periods of convalescence from various other conditions described.

Lipid profiles of rats fed with diets supplemented with vitamins niacin and pyridoxine

Hypercholesterolemia is a major risk factor for cardiovascular disease. Supplements containing the vitamins niacin (B3) and pyridoxine (B6) can promote the reduction of total cholesterol and an increase in HDL cholesterol. In this study, the effects of diets supplemented with niacin (B3) and pyridoxine (B6) on the hepatic and serum lipid profiles of Wistar rats were assessed. The diets were prepared with combinations of three concentrations of niacin (3, 4 and 5 g/kg) and pyridoxine (6, 12 and 18 mg/kg) and one with neither vitamin. The animals were divided into eleven experimental groups of six animals per group, and nine groups were fed on a standard diet with 7.5% fat and vitamin supplementation. Another group was fed with 7.5% fat without vitamin supplements. A control group received the standard diet (AIN-93M) without modifications (4% fat). The weight gain, food intake, serum and hepatic total cholesterol, serum cholesterol fractions (HDL, LDL, and VLDL), serum and hepatic triacylglycerols and hepatic and fecal lipid contents were measured after 30 days. The diet with the highest concentration of niacin and lowest concentration of pyridoxine had the lowest level of total hepatic cholesterol. Hepatic triacylglycerols were reduced by the highest concentration of niacin (5 g/kg), and this reduction was enhanced by increasing the pyridoxine concentration. The diets supplemented with niacin and pyridoxine reduced the levels of serum total cholesterol, LDL, VLDL, triacylglycerols and hepatic lipids. These effects on the lipid profile varied with the concentrations of the two vitamins and the interactions between them.

Use of pyridoxine and pyridostigmine in children with vincristine-induced neuropathy.

Four children with vincristine (VCR)-induced neuropathy are being reported. All cases were followed with the diagnosis of acute lymphoblastic leukemia. Two were boys aged between 2 and 13 year. Electromyographic examination consisted of sensoriomotor polyneuropathy with axonal involvement in three patients. In another patient, it consisted of motor axonal polyneuropathy. In all patients, pyridoxine and pyridostigmine were successfully used in the treatment of VCR-induced neuropathy. They recovered completely with this drug combination. Recovering period of symptoms was between 1-2 week.

Náuseas y vómitos durante el embarazo / Nausea and vomiting during pregnancy

Se hace una revisión de la etiología, fisiopatología y factores de riesgo de las náuseas y vómitos que ocurren durante el embarazo, así como del impacto, en casos severos o persistentes, en la salud de la madre, y del feto y del recién nacido. Se revisan los tratamientos propuestos, farmacológicos unos, no farmacológicos otros, resaltando el evidente beneficio terapéutico de la combinación de piridoxina con doxilamina y la evidencia de su inocuidad, que estuvo en entredicho a principio de la década de 1980...

Guidelines for diagnosis and management of childhood epilepsy.

Justification: Seizures constitute the most common neurological problem in children and the majority of epilepsy has its onset in childhood. Appropriate diagnosis and management of childhood epilepsy is essential to improve quality of life in these children. Evidence-based clinical practice guidelines, modified to the Indian setting by a panel of experts, are not available. Process: The Indian Academy of Pediatrics organized a consensus meeting on the diagnosis and management of childhood epilepsy on 22-23 of July 2006 at Mumbai. An expert committee was formed consisting of pediatricians, pediatric epileptologists, pediatric and adult neurologists, electrophysiologists, neuroradiologists, neurosurgeons and intensivists. A consensus was reached during the discussion that followed presentation by each of these experts. The process of updating these recommendations and arriving at consensus continued till 2009. Objectives: To develop practice guidelines for diagnosis and management of childhood epilepsy. Recommendations: Recommendations for diagnosis and management of following childhood seizures and epilepsies are given: neonatal seizures, acute symptomatic seizures, neurocysticercosis, febrile seizures, idiopathic partial and generalized epilepsies, first unprovoked seizure, newly diagnosed epilepsy, catastrophic epilepsies of infancy, refractory epilepsies of older children and adolescents, epilepsy with cognitive deterioration and status epilepticus.

Isoniazid Toxicity Presenting As Status Epilepticus and Severe Metabolic Acidosis.

Isoniazid (INH) is an integral component of treatment of tuberculosis. An acute overdose is potentially fatal and is characterized by the clinical triad of repetitive seizures unresponsive to the usual anticonvulsants, metabolic acidosis with a high anion gap and coma. The diagnosis of INH overdose should be considered in any patient who presents to emergency medical services (EMS) with the triad. We report a patient presenting with multiple generalised tonic clonic (GTC) convulsions with severe metabolic acidosis as a manifestation of INH toxicity. ©

Psoriasiform lupus vulgaris.

Tuberculosis is a major public health problem in both developing and developed countries. Cutaneous Tuberculosis constitutes a minor proportion of extra-pulmonary manifestations of Tuberculosis. Lupus Vulgaris (LV) is one of the clinical variants of Cutaneous Tuberculosis. A case of a large plaque type psoriasiform lesion of lupus vulgaris on the thigh, of 15 years' duration, in an 18-year-old girl is reported. This case highlights the ignorance level among the patients and consequent failure to avail proper anti-tuberculous treatment despite campaign in print and audio visual media.

Pyridoxine-dependent epilepsy initially responsive to phenobarbital

Pyridoxine-dependent epilepsy is a rare autossomal recessive disorder characterized by recurrent seizures that are not controlled by anticonvulsant medications but remits after administration of pyridoxine. We report on a 30 day-old girl who presented with seizures during the first day of life, initially responsive to anticonvulsant therapy, which remitted within two weeks. Seizures were characterized as multifocal myoclonic jerks of upper and lower limbs associated with buccal-lingual oral movements and eyelid blinking. Laboratory and neuroimaging studies were normal. Electroencephalographic record demonstrated a abnormal background activity with high-voltage epileptic discharges and a burst-suppression pattern. The seizures ceased after oral administration of pyridoxine, but recurred after withdrawal, confirming the diagnosis.

A epilepsia por dependência de piridoxina é uma doença autossômica recessiva rara caracterizada por crises recorrentes refratárias a tratamento medicamentoso, mas que remitem após a administração de piridoxina. Relatamos o caso de menina de 30 dias de vida que iniciou crises convulsivas desde o primeiro dia de vida, inicialmente responsivas a tratamento com drogas anticonvulsivantes, mas que reiniciaram após a segunda semana de vida. As crises eram caracterizadas por movimentos clônicos erráticos de membros superiores e inferiores associados a movimentos oromandibulares e piscamentos. Exames laboratoriais e de neuroimagem foram normais. O exame eletrencefalográfico evidenciou atividade de base desorganizada com descargas epilépticas de alta voltagem associadas a um padrão de surto-supressão. As crises cessaram após a administração de piridoxina e recorreram após a sua retirada, confirmando o diagnóstico.

INH induced status epilepticus: response to pyridoxine.

Isoniazid is an effective and widely used drug in tuberculosis treatment. The administration of toxic amounts of INH causes recurrent seizures, profound metabolic acidosis, coma and even death but therapeutic dose of isoniazid is a very rare cause of seizures. We present a case of 44-year-old HIV positive African-American female who was recently started on a preventive dose of INH after being found purified protein derivative (PPD) positive. She developed status-epilepticus that did not respond to most of the antiepileptics. As soon as she received intravenous pyridoxine, the seizures terminated abruptly.

Vincristine induced cranial polyneuropathy.

We describe a 5-year-old girl showed recovery of vincristine induced cranial polyneuropathy with pyridoxine and pyridostigmine treatment. A 5-year-old girl was diagnosed preB cell Acute Lymphoblastic Leukemia (ALL). She received chemotherapy according to the previously described modified St. Jude total therapy studies XIII. Five days after the fourth dose of vincristine, she presented with bilateral ptosis. Neurological examination revealed bilateral ptosis, and complete external opthalmoplegia with normal pupillary and corneal reflexes. She received 3.8 mg cumulative dose of vincristin before development of ptosis. A neuroprotective and neuroregenerative treatment attempt with pyridoxine and pyridostigmine was initiated. The bilateral ptosis markedly improved after 7 days of pyridoxine and pyridostigmine treatment and completely resolved after two weeks. The both agents were given for 3 weeks and were well tolerated without any side effects. During the follow up period we did not observe residue or recurrence of the ptosis.

Homocystinuria with congenital/developmental cataract.

The aim of the study is to screen patients for homocystinuria with and without cataract and analyse for homocystine and methionine. Fifty-eight samples from 29 patients, i.e., plasma and urine collected after overnight fasting were analysed by the screening test for homocystine, and paper chromatography for homocystine and methionine. Out of 29 homocystinuric patients, 24 had cataract. Only one had appreciable amounts of methionine in his serum. He also had mental retardation as expected and belongs to Type I. The other types did not have methionine but had only homocystine. There was no mental retardation or ectopia lentis. So they belonged to Types II, III or IV. As there is excess methionine in Type I, with low cystine, cataract may be due to deficiency of cysteine and reduced glutathione and might be averted by suitable therapy, i.e., high cystine-low methionine diet with B6. In other types with low methionine, cataract may be due to decreased availability of amino acids for the synthesis of lens proteins; the treatment of choice should be B12, and folate with methionine.

Porfirias en la infancia: revisión casuística de 17 casos de niños con porfiria en un servicio de dermatología pediátrica manifestaciones cutáneas evaluación de características hereditarias propuesta terapéutica revisión bibliográfica / Porphyria of infancy: review of 17 cases from a pediatric dermatology service the cutaneous manifestations and hereditary characteristics

Entre agosto de 1987 y diciembre de 1996 se observaron en nuetros servicio de dermatolgía pediátrica, sobrre un total de 37.467 pacientes con consulta de primera vez, 17 niños con diagnóstico confirmado, con una prevalencia del 0,057 por ciento (1 caso de porfiria cada 2.000 consultas de primera vez). Los 17 casos se distribuyeron de la siguiente manera. Porfiria Cutánea Tarda (PCT): 9 casos (52,9 por ciento); Protoporfiria eritropoyética (PPE): 6 casos (35,3 por ciento); Porfiria Variegata (PV): 1 caso (5,9 por ciento) y Porfiria Eritropoyéctica Congénita de Günther (PCE): 1 caso (5, 9 por ciento). Relación: PCT/PPE: 1,5:1. La distribución de frecuencia acorde a sexo y edad fue la siguiente: PCT: relación F/M: 2:1 (F= 6 casos; M= 3 casos); Edad: Rango= 3 a 12 años X= 7 años. PPE: relación F/M: 1:2 (F= 2 casos; M= 4 casos); Edad: Rango= 2 a 11 años X= 7 años. PV: 1 caso de una niña de 8 años. PEC: 1 caso de una niña de 2 meses. El diagnóstico de porfirinas en orina, plasma, eritrocitos y materia fecal y el índice de fluorescencia plasmática, acorde a su correlación clínica. Se evaluaron los antecedentes hereditarios en la mayoria de los pacientes, observándose diferentes cuadros de porfiria comprobados en los familiares, fundamentalmente en los niños portadors de PCT. Los síntomas iniciales estuvieron asociados con fenómenos de fotosensibilidad y aparición de lesiones vesico-ampollares en regiones expuetas, principalmente cara y dorso de manos. La hipertricosis fue constante en los casos de PCT. Hubo compromiso abdominal en los casos de PEC y PV y severa onicodistrofia con hipertricosis, anemia hemolítica y mal estado general en el caso de Günther. Un caso de PCT asociado con leucemia mieloide y otro en un paciente post-transplantado renal (no diálisis). En todos los pacientes con PPE se realizaron biopsias hepáticas con resultados normales...

Hiperhomocist(e)inemia / Hyperhomocyst(e)inemia

A través de numerosos datos derivados de observaciones epidemiológicas y experimentales, se ha establecido que existe una correlación positiva entre la hiperhomocist(e)inemia (HH(e)) y las enfermedades vasculares. Los datos clínicos confirman que la homocisteína (Hcy) es un factor de riesgo independiente de afecciones arteriales oclusivas (coronaria, cerebrovascular y periféricovascular) así como también trombosis venosa periférica. Este aminoácido contiene un sulfhidrilo y se forma por la desmetilación de la metionina. Es normalmente catalizado a cistationina por la enzima cistationina ß-sintetasa (CBS), dependiente de fosfato de piridoxal. Homocisteína también es remetilada a metionina por las enzimas 5-metiltetrahidrofolato-Hcy metiltransferasa (metionina sintetasa), dependiente de vitamina B12 y betaína-Hcy metiltransferasa. Estados nutricionales tales como deficiencias en vitamina B12, vitamina B6 o folato y defectos genéticos de las enzimas CBS o 5,10-metilentetrahidrofolato reductasa, pueden contribuir al aumento de los niveles plasmáticos de Hcy. La patogénesis del daño vascular inducido por Hcy puede ser multifactorial: daño directo sobre el endotelio, aumento de la peroxidación de lipoproteínas de baja densidad, incremento de tromboxano A2 plaquetario o menor activación de la proteína C. En el presente trabajo, se describen los más recientes estudios acerca de la patogénesis de la HH(e) y las implicancias para una óptima terapia

Effect of pyridoxine or riboflavin supplementation on plasma homocysteine levels in women with oral lesions.

BACKGROUND: A moderate increase in plasma homocysteine level has been reported to be involved in neural tube defects, which can be prevented with folic acid supplementation. Folic acid, vitamins B6- and B12-dependent enzymes are required to metabolize homocysteine. A study in rats showed higher tissue homocysteine levels in riboflavin as well as pyridoxine deficiency. We studied the effect of treatment with pyridoxine or riboflavin on plasma total homocysteine concentration in women with clinical and biochemical deficiencies of riboflavin and pyridoxine. METHODS: Plasma total homocysteine concentrations were measured in 20 women with glossitis and angular stomatitis before and after supplementation with pyridoxine or riboflavin. RESULTS: Pyridoxine treatment significantly reduced plasma homocysteine concentration while riboflavin treatment did not have a significant effect. CONCLUSIONS: Plasma total homocysteine levels tended to be higher in women with clinical and biochemical deficiency of vitamin B6 and therapy with pyridoxine reduced its level significantly. Riboflavin supplementation did not have a significant impact on plasma homocysteine concentration in women with glossitis and angular stomatitis.

Assessment of efficacy of pyridoxine in control of radiation induced sickness.

A randomised prospective study to evaluate the role of adjuvant administration of pyridoxine hydrochloride was carried out in 104 patients undergoing radiation therapy. In study group 52 patients received tablet pyridoxine (controlled release) 100 mg daily one hour before the radiation therapy, for a period of 7 days, while the control group was treated by irradiation alone. Reduced radiation induced sickness was observed in study group (32.6% versus 48.1%). Loss of appetite (0% versus 1.9%), nausea (11.5% versus 21.1%) and vomiting (21.1% versus 28.8%) were lower for pyridoxine treated patients than for control patients. The above observed reduction in radiation induced sickness was found to be statistically insignificant (p > 0.05). The present data also did not reveal a statistical correlation between integral dose and radiation sickness.

The tole of homocysteine, folate and other B-vitamins in the development of atherosclerosis / El papel de hemocisteina, folate y otras vitaminas B in el desarrollo de aterosclerosis

Recently, elevated homocysteine blood concentrations have been identified as an independent risks factor for the develoment of atherosclerotic lesions. The amino acid homocysteine is metabolized in the human body involving the vitamins folic acid, B12 and B6 as essential cofactors and coenzymes, respectively. There is an inverse relationship between the status of the relevant B-vitamins and the homocysteine blood concentration. supplementation if these vitamins results in a significant reduction of the homocysteine level. Nutritive amounts seem to be sufficient to obtain this reduction, even in the case of elevated homocysteine levels