ABSTRACT This trial was performed to determine the ideal ratio of digestible methionine + cysteine (Met/Lys) with digestible lysine for meat-type quails from 1 to 14 days of age. A total of six hundred thirty, one-day-old, not sexed meat type quails were randomly assigned to six groups of treatments, each one with seven replicates and fifteen quails per experimental unit. A basal diet, methionine + cysteine-deficient, was graded supplemented with DL-Methionine (0.108; 0.169; 0.230; 0.291; 0.352 and 0.413%) to obtain the treatments, represented by six digestible Met/Lys ratios (0.60; 0.65; 0.70; 0.75; 0.80 and 0.85). Data was analyzed using ANOVA and polynomial regression and broken-line models were used to estimate the digestible Met/Lys ideal ratio, considering adjust of data to both models. Statistical significance was considered when p≤0.05. Feed intake, weight gain and final body weight was linearly increased as dig. Met/Lys ratio increased. Feeding conversion was improved with increasing in dig. Met/Lys ratios. A quadratic response on feathering percentage was observed with increasing dig. Met/Lys ratios. Digestible Met/Lys ratio which, maximized feathering percentage in meat-type quails, was 0.74. According to linear broken-line model, dig. Met/Lys ratio estimated for optimum feed intake, weight gain, final body weight and feeding conversion were, respectively, 0.66; 0.71; 0.71 and 0.74. The dig. Met/Lys ideal ratio for meat-type quails from one to 14 days of age is 0.74.

ABSTRACT Calcium (Ca) and available phosphorus (avP) requirements for egg production are different between the beginning and the end of the egg-production cycle. The objective of this study was to estimate the Ca and avP requirements of Japanese quails at end of the production phase. In total, 256 Japanese quails with 48 weeks of age were randomly distributed in 2 x 2 factorial arrangement consisting of two Ca levels (29 and 38 g/kg) and two avP levels (1.5 and 3.0 g/kg), totaling four treatments with eight replicates of eight hens each. The variables evaluated were feed intake (FI); egg production (EP); marketable egg production (MEP); egg weight (EW); egg mass (EM); feed conversion ratio per egg mass (FCRM); feed conversion ratio per dozen eggs (FCRD); livability (L); yolk weight (YW), albumen weight (AW), eggshell weight (SW); yolk percentage (YP), albumen percentage (AP), eggshell percentage (SP), specific egg weight (SEW); bone calcium percentage (BCa), bone phosphorus percentage (BP), bone ash weight (Bash) and bone ash percentage (PBash). There was no interaction between Ca and avP levels for any of the evaluated parameters, except for SEW. Quail performance was not influenced by the treatments, except for MEP. Bone characteristics were not influenced by the tested dietary Ca and avP levels. However, SW and SP increased and AP decreased as dietary Ca level increased. The dietary supplementation of 38 g Ca/kg and 3.0 g avP/kg may increase marketable egg production and the egg quality of Japanese quails at end of the production cycle.

ABSTRACT This study aimed at evaluating four dietary L-Glutamine (L-Gln) or L-Gln plus L-Glutamate (L-Glu) supplementation programs on the performance, breast yield, and uniformity of broilers. A total of 2,112 one-d-old male Cobb 500(r) broilers were distributed according to a randomized block design in a 2 × 4 factorial arrangement (L-Gln or L-Gln plus L-Glu × 4 supplementation programs), totaling eight treatments with 12 replicates of 22 broilers each. The supplementation programs consisted of the dietary inclusion or not of 0.4% of L-Gln or L-Gln plus L-Glu for four different periods: 0 days (negative control), 9d, 21d, and 42d. Feed intake (FI, g), body weight gain (BWG, g), feed conversion ratio (FCR, kg/kg), coefficient of variation of body weight (CV, %), body weight uniformity (UNIF, %), breast weight (BW, g), breast yield (BY, %), coefficient of variation of breast weight (CVB), breast uniformity (UNIFB), coefficient of variation of breast yield (CVBY), and breast yield uniformity (UNIFBY) were evaluated. Birds fed the diets treatments supplemented with L-Gln or L-Gln plus L-Glu for 9d presented 3% higher BWG (p<0.05) compared with the controls. The L-Gln or L-Gln plus L-Glu supplementation until broilers were 21 days old resulted in 14, 10, 16, and 12% improvements (p<0.05) in CV, UNIF, CVBY, UNIFBY, respectively. The supplementation of 0.4% L-Gln (L-Gln 99%) or L-Gln plus L-Glu (minimum 95%) to pre-starter and starter broiler diets is recommended to improve body weight gain and uniformity.

OBJETIVO: Lipofuscinose Ceróide Neuronal Juvenil (JNCL, CLN 3, Doença de Batten) (OMIM # 204200) pertence ao grupo mais comum de doenças neurodegenerativas na infância. É causada por mutações no gene CLN3, com padrão de herança recessiva. A deleção de 1,02 kb é a mutação mais comum. Relatamos os dados clínicos e análise molecular de uma família consanguínea numerosa. MÉTODO: Família composta por dois casais consanguíneos e trinta e duas crianças. Foram obtidos dados clínicos de dez pacientes e análises moleculares de 13 participantes. RESULTADOS: Foi detectada deleção de 1,02 kb. O fenótipo mais grave, com comportamento autista, tiques e parkinsonismo foi visto em uma paciente do sexo feminino de 12 anos e o fenótipo mais leve em um paciente do sexo masculino de 14 anos. Nictalopia foi o primeiro sintoma de uma criança falecida. A perda visual de seis pacientes foi observada pela primeira vez na escola e não em casa. CONCLUSÃO: Destaca-se a variação fenotípica intrafamiliar em 10 pacientes. A investigação molecular desta família numerosa tornou possível o diagnóstico, a abordagem correta e aconselhamento genético.

OBJECTIVE: Juvenile Neuronal Ceroid-Lipofuscinosis (JNCL, CLN 3, Batten Disease) (OMIM #204200) belongs to the most common group of neurodegenerative disorders of childhood. We report the clinical data and molecular analysis of a large Brazilian family. METHOD: Family composed of two consanguineous couples and thirty-two children. Clinical data of ten JNCL patients and molecular analyses on 13 participants were obtained. RESULTS: The large 1.02 kb deletion was detected. The most severe phenotype, with autistic behavior, tics and parkinsonism was seen in a 12-year-old female and a milder phenotype in a 14-year-old male. Nyctalopia was the first symptom in one deceased child. The visual loss of six patients has been first observed in the school and not at home. CONCLUSION: The report highlights the phenotypical intrafamily variation in 10 affected children of this family. The molecular investigation of this large family in our metabolic center turned possible the diagnosis, right approach and genetic counseling.