Enzyme replacement therapy with galsulfase in 34 children younger than five years of age with MPS VI.

BACKGROUND: Mucopolysaccharidosis type VI (MPS VI) is a progressive, chronic and multisystem lysosomal storage disease with a wide disease spectrum. Clinical and biochemical improvements have been reported for MPS VI patients on enzyme replacement therapy (ERT) with rhASB (recombinant human arylsulfatase B; galsulfase, Naglazyme®, BioMarin Pharmaceutical Inc.), making early diagnosis and intervention imperative for optimal patient outcomes. Few studies have included children younger than five years of age. This report describes 34 MPS VI patients that started treatment with galsulfase before five years of age. METHODS: Data from patients who initiated treatment at <5 years of age were collected from patients' medical records. Baseline and follow-up assessments of common symptoms that led to diagnosis and that were used to evaluate disease progression and treatment efficacy were evaluated. RESULTS: A significant negative correlation was seen with treatment with ERT and urinary GAG levels. Of those with baseline and follow-up growth data, 47% remained on their pre-treatment growth curve or moved to a higher percentile after treatment. Of the 9 patients with baseline and follow-up sleep studies, 5 remained unaffected and 1 patient initially with mild sleep apnea showed improvement. Data regarding cardiac, ophthalmic, central nervous system, hearing, surgical interventions and development are also reported. No patient discontinued treatment due to an adverse event and all that were treatment-emergent resolved. CONCLUSIONS: The prescribed dosage of 1mg/kg IV weekly with galsulfase ERT is shown to be safe and effective in slowing and/or improving certain aspects of the disease, although patients should be closely monitored for complications associated with the natural history of the disease, especially cardiac valve involvement and spinal cord compression. A long-term follow-up investigation of this group of children will provide further information on the benefits of early treatment as well as disease progression and treatment efficacy and safety in this young patient population.

Association between tongue, lips position and breathing in newborns. / Associação entre posição de língua, lábios e respiração em recém-nascidos.

PURPOSE: To verify the association between breathing nasal expiratory flow and posture of lips and tongue at rest, presence of repeated forward movements of the tongue and maternal complaint of respiratory difficulty in the newborn in the first days of life. METHOD: A observational study was carried out in 130 babies, in a university hospital. Included newborn with Apgar score greater than or equal to 8 in exclusive breast milk. It was the following data: position of lips and tongue at rest, nasal expiratory flow and maternal complaint of difficulty in breathing in the newborn. The data were subjected to statistical analysis using the tests, Fisher's exact test and the Chi-Square test, adopting a significance level of 5% . RESULTS: there was a significant association between maternal complaint of newborn difficulty breathing with repeated forward tongue movements and nasal expiratory flow; tongue position with resting lips position at rest, repeated tongue forward movements with nasal expiratory flow and tongue position at rest; nasal expiratory flow exit with tongue position at rest. CONCLUSION: Symmetrical nasal expiratory flow is associated with an elevated tongue position and closed lips at rest; on the other hand, increased and/or absent nasal expiatory flow in one nostril is associated with maternal complaints of difficulty in breathing, open/ half-open lips position and low tongue position during rest, as well as, repeated forward tongue movements.

OBJETIVO: verificar a associação entre fluxo expiratório nasal e postura de lábios e língua no repouso, presença de movimentos repetidos de anteriorização de língua e queixa materna de dificuldade respiratória do recém-nascido nos primeiros dias de vida. MÉTODO: estudo observacional, realizado com 130 recém-nascidos do alojamento conjunto de um hospital Universitário. Foram incluídos recém-nascidos a termo, com idade entre 1 e 5 dias de vida, APGAR maior ou igual a oito, em aleitamento materno exclusivo. Foram coletados os seguintes dados: posição de lábios e língua no repouso, fluxo expiratório nasal, presença de movimentos repetidos de anteriorização de língua e queixa materna de dificuldade do recém-nascido para respirar. Os dados foram submetidos à análise estatística, sendo aplicado o Teste Exato de Fisher e o Qui-Quadrado, adotando-se o nível de significância de 5%. RESULTADOS: Há uma associação significativa entre queixas maternas de dificuldade do recém-nascido para respirar pelo nariz com movimentos repetidos de anteriorização de língua e fluxo expiratório nasal; posição de língua com posição de lábios no repouso, movimentos repetidos de anteriorização de língua com fluxo expiratório nasal e posição de língua no repouso; fluxo expiratório nasal com posição de língua no repouso. CONCLUSÃO: O fluxo expiratório nasal simétrico está associado com a posição de língua elevada e lábios fechados no REPOUSO; por outro lado, o fluxo expiratório nasal maior e/ou ausente em uma narina está associado com queixa materna de dificuldade do recém-nascido para respirar, posição de lábios abertos/entreabertos E posição de língua baixa no repouso, bem como, com movimentos repetidos de anteriorização de língua.

Anthropometric orofacial measures of newborns and their relationship with sex, weight and height. / Medidas antropométricas orofaciais de recém-nascidos e sua relação com sexo, peso e altura.

PURPOSE: To compare orofacial anthropometric measurements, with weight, height and sex of newborns. METHODS: Observational cross-sectional study carried out with 130 newborns on exclusive breastfeeding. Data collection was performed by properly trained and calibrated speech therapists. The orofacial measurements of the newborns were performed with flexible and transparent ruler 10 cm long, in the following segments: heights of the upper third(tr-g), the middle third(g-sn) and the lower third on the face (sn-gn); filter height (sn-Is), distance between the corner of the eye and the labial commissure on the right and left side (ex-ch). Weight and height measurements were collected from the newborns' medical records. The data were submitted to statistical analysis, using the Mann-Whitney test, adopting a significance level of 5%. RESULTS: Of the 130 newborns, 61 were male and 69 female. The median weight was 3.3 kg and the median height was 49 cm. There was significant difference between weight and measurement distance between the corner of the eye and the left and right labial commissure (ex-ch). There was no significant difference in orofacial measurements with sex and height. CONCLUSION: There was no difference in orofacial anthropometric measurements of full-term newborns when compared with sex and height; however, when compared to weight, there is a difference in the measurements of the distance between the corner of the eye and the labial commissure on the right and left side.

OBJETIVO: Comparar as medidas antropométricas orofaciais, com peso, altura e sexo de recém-nascidos. MÉTODO: Estudo transversal observacional realizado com 130 recém-nascidos em aleitamento materno exclusivo. A coleta de dados foi realizada por fonoaudiólogas devidamente treinadas e calibradas. As medidas orofaciais dos recém-nascidos foram realizadas com uma régua maleável e transparente de 10 cm de comprimento, nos seguintes segmentos: alturas do terço superior (tr-g), do terço médio (g-sn) e do terço inferior da face (sn-gn); altura do filtro (sn-Is); distância entre o canto do olho e a comissura labial do lado direito e esquerdo (ex-ch). A medida do peso e da altura foram coletadas nos prontuários dos recém-nascidos. Os dados foram submetidos à análise estatística, sendo aplicado o teste de Mann-Whitney, adotando nível de significância de 5%. RESULTADOS: Dos 130 recém-nascidos, 61 eram do sexo masculino e 69 do sexo feminino. A mediana do peso foi de 3,3 kg e da altura de 49 cm. Houve diferença significativa entre o peso e a medida da distância entre o canto do olho e a comissura labial do lado direito e esquerdo (ex-ch). Não foi encontrada diferença significativa das medidas orofaciais com sexo e altura. CONCLUSÃO: Não houve diferença nas medidas antropométricas orofaciais de recém-nascidos a termo quando comparadas com sexo e altura; porém, quando comparadas com peso, há diferença nas medidas da distância entre o canto do olho e a comissura labial do lado direito e esquerdo.

Position of lips and tongue in rest in newborns with and without ankyloglossia.

PURPOSE: Verify the position of lips and tongue at rest in newborns with and without ankyloglossia. METHODS: Cross-sectional study, carried out with 130 newborns in University Hospital. Data collection was performed by the researcher and speech-language pathologists from the Hospital. Information on gestational age, sex, weight, height and days of life was collected. The position of the lips and tongue at rest was evaluated through visual inspection with the newborns asleep. After the newborns were awakened, Neonatal Screening of the validated Protocol for the evaluation of the lingual frenulum for infants was performed to detect the alteration of the lingual frenulum. The data obtained were described and submitted to statistical analysis using the Chi-Square test to verify the association between the position of the lips with the tongue and to compare the position of the lips and tongue with and without ankyloglossia. The Mann-Whitney test was used to verify the behavior of the variables the differed between newborns with and without ankyloglossia. The significance level of 5% was adopted. RESULTS: When comparing the data, a significant difference was found between: weight and height with and without ankyloglossia; position of lips and tongue. An association between the position of the tongue and lips with and without ankyloglossia was also found. CONCLUSION: Newborns without alteration of the lingual frenulum have a tendency to remain with their lips closed and their tongue elevated during rest and newborns with ankyloglossia have a tendency to keep their lips parted and their tongue low during rest.

Rare and intractable fibrodysplasia ossificans progressiva shows different PBMC phenotype possibly modulated by ascorbic acid and propranolol treatment.

Fibrodysplasia Ossificans Progressiva (FOP) is a rare congenital intractable disease associated with a mutation in ACVR1 gene, characterized by skeleton malformations. Ascorbic acid (AA) and propranolol (PP) in combination is reported to minimize flare-ups in patients. FOP leukocyte phenotype may possibly be modulated by AA and PP treatment. In this study, expression of 22 potential target genes was analyzed by RT-PCR in peripheral blood mononuclear cells culture (PBMC) from FOP patients and controls to determine effectiveness of the combination therapy. PBMC were treated with AA, PP and AA+PP combination. Basal expression of 12 of the 22 genes in FOP PBMC was statistically different from controls. ACVR1, ADCY2, ADCY9 and COL3 were downregulated while COL1 was upregulated. ADRB1, ADRB2, RUNX2, TNF-α and ACTB, were all overexpressed in FOP PBMC. In control, AA upregulated COL1, SVCT1, ACTB, AGTR2 and downregulated ADCY2. In FOP cells, AA upregulated ACVR1, BMP4, COL1, COL3, TNF-α, ADCY2, ADCY9, AGTR2 and MAS, while downregulated ADBR2, RUNX2, ADCY1, SVCT1 and ACTB. PP increased ADBR1 and decreased RUNX2, TNF-α, AGTR1, ACTB and CHRNA7 genes in treated control PBMC compared to untreated. PP upregulated ADBR1, ADBR2 and MAS, and downregulated TNF-α and ACTB in treated FOP PBMC versus untreated. AA+PP augmented ADRB1 and ADRB2 expressions in control PBMC. In FOP PBMC, AA+PP augmented ACVR1, COL1, COL3, ADBR1, AGTR2 and MAS expression and downregulated ADBR2, RUNX2, ACTB and MRGD. These data show distinct gene expression modulation in leukocytes from FOP patients when treated with AA and or PP.

Association between ankyloglossia and breastfeeding.

PURPOSE: To analyze the association between ankyloglossia and breastfeeding. METHODS: A cross-sectional study was undertaken on 130 newborn infants in exclusive breastfeeding with Apgar score ≥ 8 within the first five days of life. The research was approved by the Ethics Committee on Human Research. The data collection was performed by the researcher and by three trained speech therapists of the team. The protocols applied were the Neonatal Tongue Screening Test from the Lingual Frenulum Protocol for Infants, the UNICEF Breastfeeding Observation Aid, and the collection of maternal complaints related to the difficulty in breastfeeding was also considered. The data were submitted to statistical analysis - chi-square test and Fisher's exact test, with a significance level of 5%. RESULTS: When correlating the data, the statistical analysis revealed an association between ankyloglossia and the items of suckling category of the Breastfeeding Observation Aid. The association between complaint of difficulty in breastfeeding and ankyloglossia was also seen. CONCLUSION: On the first days of life, ankyloglossia is associated with the mother's breastfeeding complaint and with the newborn's sucking difficulty.

Propranolol and ascorbic acid in control of fibrodysplasia ossificans progressiva flare-ups due to accidental falls.

Fibrodysplasia ossificans progressiva (FOP) is a rare, intractable and devastating genetic connective tissue disorder characterized by progressive ectopic ossification in the soft tissues and skeleton. Three patients, one teenage girl (P1), one male adult (P2) and one male child (P3), were studied and treated with FOPCON (combined formulation of 14 mg of propranolol and 250 mg of ascorbic acid), given three times per day. P1 started treatment in March 2012, P2 in October 2012 and P3 in July 2015. The clinical follow-up of these three patients, before initiating treatment with FOPCON, showed that FOP flare-ups used to occur frequently and that under FOPCON therapy, none of these patients had flare-ups. The striking feature of this treatment with FOPCON, is that, all three cases suffered accidental falls with documented injures until complete healing and that where major flare-ups should occur, injures or sequels, there was none. The present clinical observation shows that ascorbic acid plus the nonspecific beta blocker propranolol can be effectively useful, when administered previously and continually, in the prophylaxis of FOP flare-ups, especially for accidental falls. In this regard, FOPCON could be a prophylactic aid in cases of surgery of patients with FOP, hoping that it may benefit patients from having the severe sequels, characteristic of heterotopic bone formation. All three patients reported, to date, they no longer had flare-ups nor heterotopic ossification and showed normal scar healing.

Associação entre posição de língua, lábios e respiração em recém-nascidos / Association between tongue, lips position and breathing in newborns

RESUMO Objetivo verificar a associação entre fluxo expiratório nasal e postura de lábios e língua no repouso, presença de movimentos repetidos de anteriorização de língua e queixa materna de dificuldade respiratória do recém-nascido nos primeiros dias de vida. Método estudo observacional, realizado com 130 recém-nascidos do alojamento conjunto de um hospital Universitário. Foram incluídos recém-nascidos a termo, com idade entre 1 e 5 dias de vida, APGAR maior ou igual a oito, em aleitamento materno exclusivo. Foram coletados os seguintes dados: posição de lábios e língua no repouso, fluxo expiratório nasal, presença de movimentos repetidos de anteriorização de língua e queixa materna de dificuldade do recém-nascido para respirar. Os dados foram submetidos à análise estatística, sendo aplicado o Teste Exato de Fisher e o Qui-Quadrado, adotando-se o nível de significância de 5%. Resultados Há uma associação significativa entre queixas maternas de dificuldade do recém-nascido para respirar pelo nariz com movimentos repetidos de anteriorização de língua e fluxo expiratório nasal; posição de língua com posição de lábios no repouso, movimentos repetidos de anteriorização de língua com fluxo expiratório nasal e posição de língua no repouso; fluxo expiratório nasal com posição de língua no repouso. Conclusão O fluxo expiratório nasal simétrico está associado com a posição de língua elevada e lábios fechados no REPOUSO; por outro lado, o fluxo expiratório nasal maior e/ou ausente em uma narina está associado com queixa materna de dificuldade do recém-nascido para respirar, posição de lábios abertos/entreabertos E posição de língua baixa no repouso, bem como, com movimentos repetidos de anteriorização de língua.

ABSTRACT Purpose To verify the association between breathing nasal expiratory flow and posture of lips and tongue at rest, presence of repeated forward movements of the tongue and maternal complaint of respiratory difficulty in the newborn in the first days of life. Method A observational study was carried out in 130 babies, in a university hospital. Included newborn with Apgar score greater than or equal to 8 in exclusive breast milk. It was the following data: position of lips and tongue at rest, nasal expiratory flow and maternal complaint of difficulty in breathing in the newborn. The data were subjected to statistical analysis using the tests, Fisher's exact test and the Chi-Square test, adopting a significance level of 5% . Results there was a significant association between maternal complaint of newborn difficulty breathing with repeated forward tongue movements and nasal expiratory flow; tongue position with resting lips position at rest, repeated tongue forward movements with nasal expiratory flow and tongue position at rest; nasal expiratory flow exit with tongue position at rest. Conclusion Symmetrical nasal expiratory flow is associated with an elevated tongue position and closed lips at rest; on the other hand, increased and/or absent nasal expiatory flow in one nostril is associated with maternal complaints of difficulty in breathing, open/ half-open lips position and low tongue position during rest, as well as, repeated forward tongue movements.

Zinc and copper in colostrum.

The Zn (Zinc) and Cu (Copper) contents of the first day milk from 117 Brazilian mothers were examined in relation to maternal age, parity and number of miscarriages. In parallel, household water was analyzed for the contents of the same trace elements. No significant relationship was found between levels of Zn and Cu and the aforementioned factors.

Effect of evaporation and pasteurization in the biochemical and immunological composition of human milk.

OBJECTIVE: To assess the effects of evaporation and pasteurization of human milk on its biochemical and immunological composition and on its osmolarity. METHODS: The samples of mature human milk were categorized into four study groups: in natura human milk, pasteurized human milk, human milk evaporated at 70% of the baseline volume and human milk pasteurized and evaporated at 70%, with 12 different samples of milk in each group. The samples were used to determine the concentrations of sodium, potassium, calcium, phosphorus, magnesium, protein, fat, lactose, immunoglobulin A and osmolarity. RESULTS: The pasteurization of human milk did not show statistically significant changes in the concentration of sodium, potassium, calcium, phosphorus, magnesium, protein, fat, lactose, or in osmolarity; however, it showed remarkable reduction in the mean concentration of immunoglobulin A. Evaporation had a mean increase of 38% in the concentration of sodium, potassium, calcium, phosphorus, magnesium, protein, fat and lactose and mean reduction of 45% in the concentration of immunoglobulin A, without significant change in osmolarity in unprocessed milk. CONCLUSION: By evaporation at 70% of the baseline value of human milk, it is possible to obtain human milk that meets the nutritional requirements recommended for preterm infants, except for calcium and phosphorus.

Medidas antropométricas orofaciais de recém-nascidos e sua relação com sexo, peso e altura / Anthropometric orofacial measures of newborns and their relationship with sex, weight and height

RESUMO Objetivo Comparar as medidas antropométricas orofaciais, com peso, altura e sexo de recém-nascidos. Método Estudo transversal observacional realizado com 130 recém-nascidos em aleitamento materno exclusivo. A coleta de dados foi realizada por fonoaudiólogas devidamente treinadas e calibradas. As medidas orofaciais dos recém-nascidos foram realizadas com uma régua maleável e transparente de 10 cm de comprimento, nos seguintes segmentos: alturas do terço superior (tr-g), do terço médio (g-sn) e do terço inferior da face (sn-gn); altura do filtro (sn-Is); distância entre o canto do olho e a comissura labial do lado direito e esquerdo (ex-ch). A medida do peso e da altura foram coletadas nos prontuários dos recém-nascidos. Os dados foram submetidos à análise estatística, sendo aplicado o teste de Mann-Whitney, adotando nível de significância de 5%. Resultados Dos 130 recém-nascidos, 61 eram do sexo masculino e 69 do sexo feminino. A mediana do peso foi de 3,3 kg e da altura de 49 cm. Houve diferença significativa entre o peso e a medida da distância entre o canto do olho e a comissura labial do lado direito e esquerdo (ex-ch). Não foi encontrada diferença significativa das medidas orofaciais com sexo e altura. Conclusão Não houve diferença nas medidas antropométricas orofaciais de recém-nascidos a termo quando comparadas com sexo e altura; porém, quando comparadas com peso, há diferença nas medidas da distância entre o canto do olho e a comissura labial do lado direito e esquerdo.

ABSTRACT Purpose To compare orofacial anthropometric measurements, with weight, height and sex of newborns. Methods Observational cross-sectional study carried out with 130 newborns on exclusive breastfeeding. Data collection was performed by properly trained and calibrated speech therapists. The orofacial measurements of the newborns were performed with flexible and transparent ruler 10 cm long, in the following segments: heights of the upper third(tr-g), the middle third(g-sn) and the lower third on the face (sn-gn); filter height (sn-Is), distance between the corner of the eye and the labial commissure on the right and left side (ex-ch). Weight and height measurements were collected from the newborns' medical records. The data were submitted to statistical analysis, using the Mann-Whitney test, adopting a significance level of 5%. Results Of the 130 newborns, 61 were male and 69 female. The median weight was 3.3 kg and the median height was 49 cm. There was significant difference between weight and measurement distance between the corner of the eye and the left and right labial commissure (ex-ch). There was no significant difference in orofacial measurements with sex and height. Conclusion There was no difference in orofacial anthropometric measurements of full-term newborns when compared with sex and height; however, when compared to weight, there is a difference in the measurements of the distance between the corner of the eye and the labial commissure on the right and left side.

Etiological profile of early neonatal bacterial sepsis by multiplex qPCR.

INTRODUCTION: Given the major impact in terms of morbidity and mortality that episodes of early neonatal sepsis (ENS) have on both newborns and health systems, this study aimed to identify the etiological profile of early neonatal bacterial sepsis by a multiplex quantitative real-time polymerase chain reaction (qPCR). METHODOLOGY: Blood samples from newborns diagnosed with clinical ENS and hospitalized in neonatal intensive care units (NICUs) were collected and analyzed using the multiplex qPCR method to detect Streptococcus agalactiae, Escherichia coli, Klebsiella pneumoniae, Pseudomonas aeruginosa, Enterobacter sp., Serratia sp., and Staphylococcus aureus. A universal primer was used in the analysis. RESULTS: A total of 150 neonates with clinical sepsis and 10 newborns as healthy controls were included in the study. The group with clinical sepsis was 100% positive for the presence of bacterial genomic DNA through the universal primer. The control group showed negativity by qPCR. The multiplex qPCR analysis showed that 76% of the samples were positive for Escherichia coli, 34% for Staphylococcus aureus, 13.3% for Streptococcus agalactiae, 7.3% for Pseudomonas aeruginosa, and 0.7% for Enterobacter sp. and Serratia sp. Multiplex qPCR of patients with clinical sepsis matched with 8.1% of the blood samples that tested positive by the microbiological method. CONCLUSIONS: Rapid and sensitive detection of the pathogens causing ENS by this new multi-target approach based on multiplex qPCR could potentially excel compared to microbiological methods, with the simple objective of facilitating the progression to a more rapid and specific antimicrobial therapy, avoiding the abuse of antibiotics in NICUs.

[Homologous human milk supplement for very low birth weight preterm infant feeding]. / Aditivo homólogo para a alimentação do recém-nascido pré-termo de muito baixo peso.

OBJECTIVE: To develop a homologous additive of human milk for feeding the very low weight infants with an original and simplified methodology, to know the nutritional composition of fortified human milk with this additive and to evaluate its suitability for feeding these infants. METHODS: For the production and analysis of human milk with the homologous additive, 25 human milk samples of 45 mL have undergone a lactose removal process, lyophilization and they were diluted in 50 mL of human milk. Doses of lactose, proteins, lipids, energy, sodium, potassium, calcium, phosphorus and osmolality were measured. RESULTS: The composition of the additive milk was lactose 9.22 ± 1.00 g/dL; proteins 2.20 ± 0.36 g/dL; lipids 2.91 ± 0.57 g/dL; calories 71.93 ± 8.69 kcal/dL; osmolality 389.6 ± 32.4 mOsmol/kg H2O; sodium 2.04 ± 0.45 mEq/dL; potassium 1.42 ± 0.15 mEq/dL; calcium 43.44 ± 2.98 mg/dL; and phosphorus 23.69 ± 1.24 mg/dL. CONCLUSIONS: According to the nutritional contents analyzed, except for calcium and phosphorus, the human milk with the proposed additive can achieve the nutritional needs of the very low birth weight preterm infant.

[Continuous fenoterol inhalation by children with severe acute asthma: immediate clinical effects]. / Inalação contínua com fenoterol na criança com asma aguda grave: efeitos clínicos imediatos.

OBJECTIVE: To evaluate the alterations of heart rate, blood pressure, psychological aspects and oxygen saturation after continuous fenoterol inhalation (0.5 mg/Kg) by children with severe acute asthma. METHODS: We studied 30 patients with severe acute asthma who were treated at the pediatric ward of Hospital Universitário-UFMS. The patients inhaled 0.5 mg/Kg of fenoterol (two drops/Kg) during one hour. Psychological aspects, oxygen arterial saturation, heart rate and blood pressure were evaluated at three different moments: before, after and one hour after the fenoterol inhalation. RESULTS: There were 17 males (56.6%) and 13 females (43.4%). Sleepiness was observed in 16 (53.3%), psychomotor agitation in one (33%) and nausea and vomiting in 12 patients (40%). The average of oxygen arterial saturation increased from 90.9 +/- 2.8% to 92.7 +/- 2.5% (P<0.05) after inhalation. There was statistically significant increase in the average heart rate before and after inhalation (139.5 +/- 13.5 beats/min, 166.5 +/- 11.1 beats/min, respectively), P<0.05. A significant decrease in blood pressure rate was observed from 117.56 +/- 10.3 / 74.6 +/- 7 mmHg, to 107.6 +/- 11 / 63.6 +/- 9.3 mmHg (P<0.05). CONCLUSIONS: Continuous fenoterol (0.5 mg/Kg) inhalation by children with severe acute asthma caused sleepiness, nausea, vomits, palpitation and decrease in blood pressure rate. The authors suggest that patients submitted to this treatment need clinical monitorship at hospital settings. Children with concomitant diseases such as diarrhea, vomits, and dehydration require special attention.

[Assessment of pulmonary function of preterm newborn infants with respiratory distress syndrome at different positive end expiratory pressure levels]. / Avaliação da função pulmonar de recém-nascidos com síndrome do desconforto respiratório em diferentes pressões finais expiratórias positivas.

OBJECTIVE: To verify the alterations of pulmonary function in preterm newborn infants with respiratory distress syndrome (RDS). The parameters analyzed were Dynamic Compliance (Cdyn), Inspiratory Tidal Volume (TVinsp), partial arterial pressure of carbon dioxide (PaCO2). METHODS: Eleven preterm newborn infants, with gestational age <35 weeks, and birth weight <2,500 g, were include in a control case study. All infants presented RDS and were treated with 120 mg/Kg of porcine surfactant. The initial positive end expiratory pressure (PEEP) was 3 cm H2O. A pneumotachograph with a graphical monitor was used to assess the pulmonary function. After each increase in the PEEP (4 and 6 cm H2O), there was an interval of 20 minutes before measuring the arterial data of pulmonary function and arterial gases. RESULTS: There were three males and eight females (1:2.7) among the infants with RDS. The mean gestational age was 30.78. +/- 2.05 weeks, ranging from 26 to 34 weeks. The increase in the PEEP from 3 to 6 cm H2O caused significant decrease in the TVinsp (6.46 +/- 3.43 to 4.20 +/- 2.35, P=0.0262). With the increase in the PEEP from 4 to 6 cm H2O, there was also a decrease in the TVinsp (5.98 +/- 3.33 to 4.20 +/- 2.35), (P=0.0044). Regarding the Cdyn, when there was an increase in the PEEP from 3 to 6 cm H2O, the reduction was statistically significant (0.58 +/- 0.27 to 0.46 +/- 0.25, P=0.0408) and from 4 to 6 cm H2O, the reduction in the Cdyn was also important (0.77 +/- 0.27 to 0.46 +/- 0.25), (P=0.0164). Increases in the PEEP from 4 to 6 cm H2O caused increases in the PaCO2 (52.81 +/- 15.49 to 64.90 +/- 12.69), (P= 0.0141). A more significant increase was observed when the PEEP was elevated from 3 to 6 cm H2O (41.45 +/- 7.87 to 64.90 +/- 12.69, P=0.0033). CONCLUSIONS: The study showed that the PEEP from 3 to 4 cm H2O produces better results in terms of Cdyn and less collateral effects regarding respiratory acidosis and pulmonary hyperventilation with impairment of the alveolar ventilation, evidenced by the increase in the PaCO2 and the decrease in the TVinsp.

Association between ankyloglossia and breastfeeding / Associação entre anquiloglossia e amamentação

ABSTRACT Purpose To analyze the association between ankyloglossia and breastfeeding. Methods A cross-sectional study was undertaken on 130 newborn infants in exclusive breastfeeding with Apgar score ≥ 8 within the first five days of life. The research was approved by the Ethics Committee on Human Research. The data collection was performed by the researcher and by three trained speech therapists of the team. The protocols applied were the Neonatal Tongue Screening Test from the Lingual Frenulum Protocol for Infants, the UNICEF Breastfeeding Observation Aid, and the collection of maternal complaints related to the difficulty in breastfeeding was also considered. The data were submitted to statistical analysis - chi-square test and Fisher's exact test, with a significance level of 5%. Results When correlating the data, the statistical analysis revealed an association between ankyloglossia and the items of suckling category of the Breastfeeding Observation Aid. The association between complaint of difficulty in breastfeeding and ankyloglossia was also seen. Conclusion On the first days of life, ankyloglossia is associated with the mother's breastfeeding complaint and with the newborn's sucking difficulty.

RESUMO Objetivo Verificar associação entre anquiloglossia e amamentação. Método Estudo transversal, realizado em 130 recém-nascidos, em um Hospital Universitário. Foram incluídos recém-nascidos entre um a cinco dias de vida, com Apgar score ≥ 8, em aleitamento materno exclusivo. Participaram da pesquisa apenas recém-nascidos de termo e saudáveis. Esta pesquisa foi aprovada pelo Comitê de Pesquisa com Seres Humanos. A coleta de dados foi realizada pela pesquisadora e por três fonoaudiólogas da equipe, devidamente treinadas e calibradas. Os protocolos aplicados foram: avaliação anatomofuncional do Protocolo de avaliação do frênulo da língua em bebês, o Protocolo de Observação da Mamada da UNICEF e coleta das queixas maternas referentes a dificuldade ou não para amamentar. Os dados obtidos foram submetidos à análise estatística, sendo aplicado o teste Qui-quadrado e teste exato de Fisher, adotando nível de significância de 5%. Resultados Quando correlacionados os dados, a análise estatística demonstrou associação entre anquiloglossia e os itens da categoria de sucção do Protocolo de Observação da Mamada. Encontrou-se também associação entre queixa de dificuldade para amamentar e anquiloglossia. Conclusão Nos primeiros dias de vida, a anquiloglossia está associada com queixa da mãe para amamentar e com a dificuldade de sucção do recém-nascido.

Enterococcus faecium and Enterococcus faecalis in blood of newborns with suspected nosocomial infection.

Enterococci are Gram-positive cocci saprophyte of the human gastrointestinal tract, diners who act as opportunistic pathogens. They can cause infections in patients hospitalized for a long time or who have received multiple antibiotic therapy. Enterococcus faecalis and Enterococcus faecium are the most common species in human infections. To evaluate the possibility of rapid detection of these species and their occurrence in the blood of newborns with suspected nosocomial infection, blood samples were collected from 50 newborns with late infections, admitted to the Neonatal Care Unit of the University Hospital Federal de Mato Grosso do Sul (UFMS-HU), from September 2010 to January 2011. The samples were subjected to conventional PCR and real time PCR (qPCR) to search for Enterococcus faecium and Enterococcus faecalis, respectively. The PCR results were compared with respective blood cultures from 40 patients. No blood cultures were positive for Enterococci, however, eight blood samples were identified as genomic DNA of Enterococcus faecium by qPCR and 22 blood samples were detected as genomic DNA of Enterococcus faecalis by conventional PCR. These findings are important because of the clinical severity of the evaluated patients who were found positive by conventional PCR and not through routine microbiological methods.

Serum phenylalanine in preterm newborns fed different diets of human milk.

OBJECTIVE: To evaluate phenylalanine plasma profile in preterm newborns fed different human milk diets. METHODS: Twenty-four very-low weight preterm newborns were distributed randomly in three groups with different feeding types: Group I: banked human milk plus 5% commercial fortifier with bovine protein, Group II: banked human milk plus evaporated fortifier derived from modified human milk, Group III: banked human milk plus lyophilized fortifier derived from modified human milk. The newborns received the group diet when full diet was attained at 15 ± 2 days. Plasma amino acid analysis was performedon the first and last day of feeding. Comparison among groups was performed by statistical tests: one way ANOVA with Tukey's post-test using SPSS software, version 20.0 (IBM Corp, NY, USA), considering a significance level of 5%. RESULTS: Phenylalanine levels in the first and second analysis were, respectively, in Group I: 11.9 ± 1.22 and 29.72 ± 0.73; in Group II: 11.72 ± 1.04 and 13.44 ± 0.61; and in Group III: 11.3 ± 1.18 and 15.42 ± 0.83 µmol/L. CONCLUSION: The observed results demonstrated that human milk with fortifiers derived from human milk acted as a good substratum for preterm infant feeding both in the evaporated or the lyophilized form, without significant increases in plasma phenylalanine levels in comparison to human milk with commercial fortifier.

Comparison between homologous human milk supplements and a commercial supplement for very low birth weight infants.

OBJECTIVES: To describe the methodology for the preparation of two additives derived from human milk, liquid and powdered, and to compare this composition with the commercial additive FM85®. METHODS: For the preparation of the liquid and powdered supplements, 40 samples of human milk were used. Both supplements have been through three preparation phases: skimming, evaporation and lactose removal. After these phases, the liquid supplement is ready, and the powdered requires a fourth phase - lyophilization. To each sample of the liquid and powdered supplements were added, respectively, 80 mL (group I) and 100 mL (group II) of pooled banked human milk. For comparison, 20 samples of 100 mL of the pool were added to 5 g of the FM85® supplement (Nestlé) (group III). Analyses of carbohydrates, protein, lipids, calcium, phosphorus, sodium, osmolality and caloric content were performed, considering a significant difference p < 0.05. RESULTS: Groups I, II, and III showed, respectively, the following results: protein = 1.81, 2.38 and 1.96 g/dL (p < 0.001); carbohydrates = 6.70, 7.25 and 10.06 g/dL (p = 0.006); fat = 3.75, 3.75 and 3.73 g/dL (p = 0.96); calcium = 36.92, 44.75 and 79.37 mg/dL (p = 0.001); phosphorus = 20.02, 23.28 and 56.30 mg/dL (p = 0.02); sodium = 14.32, 14.40 and 20.33 mEq/L (p = 0.143); osmolality = 391.45, 412.47 and 431.00 mOsmol/kgH(2)O (p = 0.074); and caloric content = 67.78, 72.27 and 81.65 kcal (p = 0.001). CONCLUSION: The studied additives differ significantly from the commercial additive FM85® in some of its components, and its composition may or may not meet the quantity of nutrients suggested by the most recent recommendations.

Zinc and copper concentrations in human preterm milk.

Zinc and copper are important trace elements in the nutrition of preterm infants. This study determined and compared the concentrations of zinc and copper in preterm milk of mothers receiving and not receiving zinc supplementation diets. The effects of maternal supplementation on the blood levels and anthropometric parameters were evaluated. Thirty-eight mothers and their preterm infants were enrolled in the study. Eighteen mothers were given a daily supplementation of 50 mg zinc, whereas the other 20 were not. Zinc and copper levels in milk were determined at 15-day intervals, as were blood zinc levels along with anthropometric parameters. Zinc and copper contents were determined by atomic absorption spectrophotometry. No significant differences were found between groups either for zinc values in maternal milk and infant blood or for anthropometric measurements. A pronounced decrease in copper levels was observed in mothers receiving supplemental zinc. Zinc supplementation given to mothers who were breastfeeding preterm infants had no significant effect on zinc secretion in milk.