An efficient three-component one-pot approach to the synthesis of 2,3,4,5-tetrahydro-1H-2-benzazepines by means of rhodium-catalyzed hydroaminomethylation.

We have developed a novel and efficient three-component one-pot, catalytic approach for the synthesis of 2,3,4,5-tetrahydro-1H-2-benzazepines, using a rhodium catalyst that does not require phosphine. The isolated yields are excellent and the protocol tolerates anilines of diverse basicity.

Molecular analysis of PROP1, PIT1, HESX1, LHX3, and LHX4 shows high frequency of PROP1 mutations in patients with familial forms of combined pituitary hormone deficiency.

Unlabelled: Combined Pituitary Hormone Deficiency (CPHD) is a prevalent disease in Neuroendocrinology services. The genetic form of CPHD may originate from mutations in pituitary transcription factor (PTF) genes and the pituitary image in these cases may give a clue of what PTF is most probably mutated: defects in LHX4 are usually associated with ectopic posterior pituitary (EPP); defects in LHX3, PIT1, and PROP1, with normally placed posterior pituitary (NPPP); HESX1 mutations are associated with both.

Objective: To identify mutations in PTF genes in patients with idiopathic hypopituitarism followed in our service, based on the presence or absence of EPP on sellar MRI.

Central precocious puberty associated with pituitary duplication and midline defects.

Central precocious puberty (CPP) is due to premature activation of the hypothalamic-pituitary-gonadal axis. It may be idiopathic or result from congenital or acquired CNS lesions. We describe a 7.

Assessment of food intake in infants between 0 and 24 months with congenital heart disease.

Background: Children with congenital heart disease are usually malnourished and present some degree of functional and/or structural impairment of organs. There is also deficiency in nutrient intake, due to the control of fluids required by some patients which restrains the nutrient intake of some cardiac children.

Objective: To assess the food intake of children with congenital heart disease hospitalized in the pediatric heart unit of a "Public Teaching Hospital".

Congenital granular cell tumor (congenital epulis): a lesion of multidisciplinary interest.

Congenital granular cell tumor (CGCT), or congenital epulis, is a very uncommon benign soft tissue lesion that usually arises from the alveolar mucosa of neonates and may cause respiratory and feeding problems. We report a case of a 3-day-old female newborn, who presented an intraoral tumor mass which was protruding from her mouth, and compromising feeding. Under general anesthesia, the lesion was completely removed and the patient had an uneventful postoperative course.

Brain MRI in mucopolysaccharidosis: effect of aging and correlation with biochemical findings.

Objective: To investigate the influence of aging on conventional MRI and magnetic resonance spectroscopy (MRS) findings of mucopolysaccharidosis (MPS) patients and to test the correlation of enzyme levels, urinary glycosaminoglycans (GAG), and neuroimaging findings.

Methods: Sixty patients with MPS types I (n = 8), II (n = 31), IV-A (n = 4), and VI (n = 17) underwent T2, fluid-attenuated inversion recovery (FLAIR), and MRS of the brain. For analysis of MRI variables, we measured the normalized cerebral volume (NCV), CSF volume (NCSFV), ventricular volume (NVV), and lesion load (NLL) on FLAIR using semiautomated and automated segmentation techniques.

Rhodium(I)-catalyzed hydroaminomethylation of 2-isopropenylanilines as a novel route to 1,2,3,4-tetrahydroquinolines.

A new atom economical approach for the preparation of 1,2,3,4-tetrahydroquinolines can be achieved by means of the intramolecular hydroaminomethylation of 2-isopropenylanilines, mediated by an ionic diamino rhodium catalyst that does not require phosphine--this reaction is highly chemo- and regioselective, and it occurs in good isolated yields.

The natural history of the R120C PROP1 mutation reveals a wide phenotypic variability in two untreated adult brothers with combined pituitary hormone deficiency.

Background: Combined pituitary hormone deficiency (CPHD) corresponds to impaired production of growth hormone (GH) and other anterior pituitary hormones. The genetic form of CPHD may result from mutations in pituitary transcription factor genes, and PROP1 is the most commonly mutated gene in these cases. Patients with PROP1 mutations may have variable CPHD phenotypes but, because they are usually treated in childhood, the wide phenotypic variability caused by these mutations may not be thoroughly appreciated.

Reversible Kallmann syndrome: report of the first case with a KAL1 mutation and literature review.

Kallmann syndrome (KS) describes the association of isolated hypogonadotropic hypogonadism with hypo/anosmia. A few KS patients may reverse hypogonadism after testosterone withdrawal, a variant known as reversible KS. Herein, we describe the first mutation in KAL1 in a patient with reversible KS and review the literature.

Highly regioselective anti-markovnikov palladium-borate-catalyzed methoxycarbonylation reactions: unprecedented results for aryl olefins.

[Structure: see text] A general, highly efficient and regioselective methoxycarbonylation, by means of a palladium-salicylicborate-catalyzed protocol, of terminal alkyl and aryl olefins is described. The substrates include aliphatic alkenes, allylbenzenes, and styrene derivatives. The yields are very good (60-92%) and the regioselectivity, in favor of the linear ester, is up to quantitative--unprecedented in the case of styrenes.

Cherubism: long-term follow-up of 2 patients in whom it regressed without treatment.

Cherubism is a rare hereditary, self-limiting fibrous dysplasia characterised by painless enlargement of the jaws in childhood. Although, it is accepted that the lesions of cherubism are eventually replaced by bone, there have been few long-term follow-up reports with clinicoradiographic documentation of spontaneous remission of the disease, without treatment. We report two cases of cherubic boys who were followed for 17 and 19 years.

Antioxidant properties of violacein: possible relation on its biological function.

Violacein, a violet pigment produced by Chromobacterium violaceum, has attracted much attention in recent literature due to its pharmacological properties. In this work, the antioxidant properties of violacein were investigated. The reactivity with oxygen and nitrogen reactive species and 1,1-diphenyl-2-picryl-hydrazyl (DPPH), a stable free radical, was evaluated.

Prospective study of 11 Brazilian patients with mucopolysaccharidosis II.

Objective: To assess the progression of mucopolysaccharidosis II in 11 Brazilian patients over a 12-month period.

Methods: Eleven Brazilian patients with mucopolysaccharidosis II were prospectively studied at the Division of Medical Genetics of Hospital de Clínicas de Porto Alegre. The initial assessment and the assessment at 12 months included: anamnesis, physical examination, abdominal nuclear magnetic resonance, echocardiogram, 6-minute walk test, audiometry, serum biochemical tests and urinary glycosaminoglycan concentration.

[Evaluation of nematode predacious fungus Duddingtonia flagrans on infective Haemonchus contortus and Strongyloides papillosus larvae of goats].

One brazilian isolate of nematode-trapping fungus Duddingtonia flagrans AC 001 was evaluated regarding the capacity of supporting passage through the gastrointestinal tract of goats without losing the ability to entrap infective Haemonchus contortus and Strongyloides papillosus larvae (L3). Ten saneen goats of eight months old, males and infected naturally were divided in two groups of five animals. In the group 1, the animals received orally 20 g of pellets of the D.

Relationship between the lipophilicity of gallic acid n-alquil esters' derivatives and both myeloperoxidase activity and HOCl scavenging.

The gallic acid and several n-alkyl gallates, with the same number of hydroxyl substituents, varying only in the side carbonic chain length, with respective lipophilicity defined through the C log P, were studied. It evidenced the structure-activity relationship of the myeloperoxidase activity inhibition and the hypochlorous acid scavenger property, as well as its low toxicity in rat hepatic tissue. The gallates with C log P below 3.

[Lactational mastitis and Baby-Friendly Hospital Initiative, Feira de Santana, Bahia, Brazil].

This study aimed to evaluate whether follow-up of breastfeeding mothers at maternity hospitals classified as "Baby-Friendly Hospitals" in Feira de Santana, Bahia, Brazil, was a protective factor against mastitis. This was a cross-sectional study in which 2,543 mothers of infants (< 1 year of age) were selected by simple stratification and interviewed by 104 college students at immunization services. Measures of association (odds ratio, 95%CI) and statistical significance were calculated by the chi-square test and respective p values.

Waxing and waning of a pituitary mass in a young woman with combined pituitary hormone deficiency (CPHD) due to a PROP-1 mutation.

We describe a 23-yr old woman with congenital combined pituitary hormone deficiency (CPHD) diagnosed at 10 years of age and a large sellar mass discovered at the age of 19 years, when her first pituitary MRI was performed. The mass (height: 13 mm) extended to the suprasellar region, close to the optic chiasm, showed signal hyperintensity in T1- and hypointensity in T2-weighted images, with no enhancement after gadolinium injection. Although these MRI features were suggestive of Rathke's cleft cyst, cystic craniopharyngioma or previous hemorrhage, no visual symptoms, diabetes insipidus and/or hyperprolactinemia were present.

[Glycoprotein-secreting pituitary adenomas: pathogenesis, diagnosis and treatment].

The glycoprotein-secreting pituitary adenomas comprise two distinctive clinical and pathological entities, the gonadotroph and the thyrotroph cell pituitary adenomas. Although they can be grouped together for producing hormones and/or subunits that are glycoproteins, these tumors originate from distinctive cell types (gonadotrophes and thyrotrophes) that are only remotely related. Gonadotroph cell adenomas are among the commonest types of pituitary adenomas, corresponding to the majority of the so-called "nonfunctioning" or clinically silent adenomas, while thyrotroph cell adenomas are extremely rare and usually present with hyperthyroidism due to inappropriate TSH secretion.

Evaluation of anti-herpetic and antioxidant activities, and cytotoxic and genotoxic effects of synthetic alkyl-esters of gallic acid.

The n-alkyl esters of gallic acid (CAS 13857-8) have a diverse range of uses as antioxidants in food, cosmetics and pharmaceutical industries. Pharmaceutical studies performed with these compounds have found that they have many therapeutic potentialities including anti-cancer, antiviral and antimicrobial properties. However, more interest has been devoted to their antioxidant activity due to the ability to scavenge and reduce reactive oxygen species (ROS) formation.

Sildenafil selectively inhibits acute pulmonary embolism-induced pulmonary hypertension.

Selective pulmonary vasodilators attenuate acute pulmonary embolism (APE)-induced pulmonary hypertension. We examined the effects of intravenous sildenafil on the hemodynamic and respiratory changes caused by APE in anesthetized dogs. Sham operated animals (n=3) received only saline infusions.

[Feeding habits of breastfed and non-breastfed children up to 1 year old].

Objective: To describe the eating habits of breastfed and non-breastfed children through the analysis of food intake by children younger than one year of age in Feira de Santana in 2001.

Methods: Cross-sectional study. The mothers of children younger than one year answered a questionnaire during the national vaccination day in 44 (71%) vaccination units selected by simple stratification.

Acharan sulfate, the new glycosaminoglycan from Achatina fulica Bowdich 1822. Structural heterogeneity, metabolic labeling and localization in the body, mucus and the organic shell matrix.

Acharan sulfate, a recently discovered glycosaminoglycan isolated from Achatina fulica, has a major disaccharide repeating unit of -->4)-2-acetyl,2-deoxy-alpha-d-glucopyranose(1-->4)-2-sulfo-alpha-l-idopyranosyluronic acid (1-->, making it structurally related to both heparin and heparan sulfate. It has been suggested that this glycosaminoglycan is polydisperse, with an average molecular mass of 29 kDa and known minor disaccharide sequence variants containing unsulfated iduronic acid. Acharan sulfate was found to be located in the body of this species using alcian blue staining and it was suggested to be the main constituent of the mucus.

A novel mutation in the anion exchanger 1 gene is associated with familial distal renal tubular acidosis and nephrocalcinosis.

Objective: The anion exchanger gene (AE1) or band 3 encodes a chloride-bicarbonate (Cl(-)/HCO(3)(-)) exchanger expressed in the erythrocyte and in the renal alpha-intercalated cells involved in urine acidification. The purpose of the present study was to screen for mutations in the AE1 gene in 2 brothers (10 and 15 years of age) with familial distal renal tubular acidosis (dRTA), nephrocalcinosis, and failure to thrive.

Methods: AE1 mutations were screened by single-strand conformation polymorphism, cloning, and sequencing.

[Violence and death by external causes].

An ecological study highlighted violence and death by external causes in Salvador and in Bahia through data furnished by the Fundação Nacional de Saúde (Health National Foundation) at the Ministério da Saúde (Ministry of Health), based on mortality records and population estimates by IBGE. The risk of death by homicide in Brazil is three times greater than in the United States, and up to forty times higher than in Japan. Homicide was the primary cause of loss of potential years of life (13.