The use of two or more courses of low-dose systemic dexamethasone to extubate ventilator-dependent preterm neonates may be associated with a higher prevalence of cerebral palsy at two years of corrected age.

BACKGROUND: Our objective was to determine whether the use of two or more courses of low-dose systemic dexamethasone for extubation of ventilator-dependent preterm infants after the first week of life, as proposed in the DART study, is associated with greater neurodevelopmental harm at two years of corrected age, compared to a single course. METHODS: Retrospective review at seven level III neonatal intensive care units. Preterm infants who underwent only one course of systemic dexamethasone for extubation were grouped into DART-1; those who underwent two or more courses were grouped into DART-2. Data and outcomes of infants in DART-2 were compared with those in DART-1. RESULTS: 150 preterm infants were studied: 104 in DART-1 and 46 in DART-2. Patients in DART-2 had a lower gestational age (25 vs. 26 weeks, p = 0.031) and greater morbidity. The average dexamethasone cumulative dose for patients in DART-1 was 0.819 mg/kg, vs. 1.697 mg/kg for patients in DART-2. A total of 14 patients died. The neuromotor and neurosensory assessments at two years of corrected age revealed in the DART-2 survivors, after the multivariate analysis, a higher prevalence of cerebral palsy with functional motor class 2 (OR = 6.837; 95%CI: 1.054-44.337; p = 0.044) and ophthalmological problems requiring the use of glasses (OR = 4.157; 95%CI: 1.026-16.837; p = 0.046). CONCLUSIONS: In this cohort, the use of more than one course of systemic dexamethasone in low doses for extubation of ventilator-dependent premature infants after the first week of life was associated, at two years of corrected age, with a higher prevalence of cerebral palsy with functional motor class 2 and ophthalmological problems requiring the use of glasses.

Role of the cerebral ultrasound in a case of Sturge-Weber syndrome.

Cerebral ultrasound (CUS) can be a valuable non-invasive diagnostic tool for brain involvement in Sturge-Weber syndrome (SWS). Literature discussing the relevance of ultrasound in SWS is, however, scarce.We report a case of a newborn with SWS and serious brain abnormalities diagnosed on the first day of life with a CUS.

Cutis marmorata telangiectatica congenita and major lower limb asymmetry.

A 39-week-old male newborn presented at birth with atrophic erythematous and purpuric skin lesions, in a typical right-sided segmental distribution. Lesions were persistent and unaffected by rewarming in the postpartum period. Postnatal echocardiogram showed a predominance of the right cavities and an upper atrial septal defect. Cerebral and abdominal ultrasound were normal along with ophthalmological examination. On follow-up, lower limbs asymmetry was noted. The right lower limb was shorter in length and had a smaller diameter. At 6 months, the right lower limb was 1.5 cm shorter than the left, most likely related to nutritive vessels malformations. The discrepancy was even more pronounced at the age of 9 months. This leg-length asymmetry can lead to severe functional limitations in the future.

[Neonatal Morbidity and Gestational Diabetes: Coincidence or Consequence of the 2011 Protocol]. / Morbilidade Neonatal na Diabetes Gestacional: Coincidência ou Consequência do Consenso de 2011.

INTRODUCTION: Gestational diabetes is one of the diseases associated with pregnancy with higher rate of complications. Despite being a transitory condition, short and long term complications related to gestational diabetes have been described. There is scientific evidence to say that good metabolic control decreases perinatal complications. In 2011, new criteria was proposed for its diagnosis, which made possible its diagnosis during the 1st trimester of pregnancy. The aim of this study is to compare neonatal morbidity in two groups of women with gestational diabetes diagnosis before and after the latest Portuguese guidelines for diabetes and pregnancy were published (February 2011). MATERIAL AND METHODS: We included all newborns born in Maternidade Bissaya Barreto whose mother, followed at our maternity between 2008 and 2013, had unifetal pregnancy complicated by diabetes. We used a perinatal database and analysed the impact of the new guidelines in perinatal morbidity over two periods of three years. RESULTS: There were 774 women who met the inclusion criteria. We found that gestational diabetes was diagnosed earlier, insulin therapy was more frequent. Neonatal morbidity was increased, and there were more cases of neonatal hypoglycemia and congenital anomalies, and newborns became smaller for gestational age. DISCUSSION: The increase in neonatal morbidity was associated with early diagnosis and rigorous metabolic control. CONCLUSION: To analyse national data will be fundamental to understand this unexpected increase in morbidity.

Introdução: A diabetes gestacional constitui uma das doenças associada à gravidez com maior taxa de complicações. Apesar de ser uma condição habitualmente transitória, estão reconhecidas as suas complicações a curto e longo prazo. Há evidência científica para afirmar que um bom controlo metabólico reduz as complicações perinatais. Em 2011, foram propostos novos critérios para o seu diagnóstico, o que o tornou possível logo no primeiro trimestre de gravidez. Neste trabalho, propomo-nos comparar a morbilidade neonatal entre dois grupos de recém nascidos filhos de mulheres com diabetes gestacional submetidos a dois protocolos diferentes. Material e Métodos: Estudo observacional analítico, retrospetivo descritivo de recém nascidos de mães com diabetes gestacional com gravidez unifetal seguida na Maternidade Bissaya Barreto no período de 2008 a 2013. Utilizou-se a informação clinica de recém nascidos e mães com diabetes gestacional armazenada em base de dados. Analisaram-se as repercussões clínicas da utilização do novo consenso comparando dois períodos de três anos. Resultados: Analisaram-se 774 díades mãe-filho. No segundo período a diabetes gestacional foi diagnosticada mais precocemente e a terapêutica com insulina foi instituída com mais frequência. Registou-se um aumento significativo da morbilidade neonatal, com mais casos de hipoglicémia neonatal e de anomalias congénitas e maior taxa de recém nascidos leves para a idade gestacional. Discussão: O aumento da morbilidade neonatal, nos últimos anos, associou-se de um modo positivo à precocidade do diagnostico de diabetes gestacional e ao rigor do controlo metabólico. Conclusão: Analisar os dados nacionais será fundamental para compreender este inesperado e preocupante aumento da morbilidade.

Reset osmostat: a rare cause of hyponatraemia.

Neonatal hyponatraemia is common, and related to significant morbidity and mortality. We report a case of a preterm newborn (gestational age of 36 weeks) with hyponatraemia, and with a prenatal diagnosis of cleft lip and palate, with a normal fetal karyotype. On the seventh day of life, a biochemical evaluation for jaundice and mild signs of dehydration showed hyponatraemia of 124 mmol/L. Investigation showed normal adrenal and thyroid functions, plasma hyposmolality (258 mOsm/kg); high urinary sodium (73 mmol/L) and high urinary osmolality (165 mOsm/kg). Despite oral sodium supplementation and fludrocortisone treatment, sodium levels remained between 124 and 130 mmol/L. Cranial ultrasound, brain MRI and renal ultrasound were normal. The diagnosis of hyponatraemia was unpredicted and the investigation was suggestive of reset osmostat, a subtype of the syndrome of inappropriate secretion of antidiuretic hormone, characterised by a subnormal threshold for antidiuretic hormone secretion.

Neonatal lupus with atypical cardiac and cutaneous manifestation.

Neonatal lupus erythematosus is a rare, passively acquired autoimmune disease, caused by maternal autoantibodies. The most common manifestations are skin rash and congenital heart block. Cutaneous manifestations may be present at birth, but often develop within a few weeks after delivery. Congenital heart block may present as bradycardia in utero or during physical examination at birth. Approximately 40-60% of mothers are asymptomatic when the infants are diagnosed. We present a case of a child, born with erythematosus lesions in the face, scalp, trunk, limbs and nodules/papules on the palmar and plantar surfaces. He also had hepatosplenomegaly and thrombocytopenia. Echocardiography showed hyperechoic lesions on the anterior papilar muscle of the left ventricle and on the lateral cusp of the tricuspid valve. The mother had unexplained fever and vasculitic lesions in her hands and feet. Antinuclear antibodies, anti-SSa/Ro and anti-SSb/La were positive in the mother and child, making the diagnosis of neonatal lupus.

Isolated anaemia as a manifestation of Rh isoimmunisation.

Rh isoimmunisation leads to haemolytic anaemia and hyperbilirubinaemia in the first h of life. Isolated early onset neonatal anaemia has rarely been reported. The authors describe the case of a term infant, born to an 'A' negative, second gravida mother. On the second day of life, pallor was noticed. His haemoglobin (Hb) was 6.8 g/dl, he had reticulocytosis and a positive direct antiglobulin test. However, he did not have a high total serum bilirubin (TSB) (87.2 µmol/l). He was transfused with red blood cells and kept under phototherapy for 3 days. Three weeks later, he received another transfusion for severe anaemia (Hb 6 5 g/dl). During this period, he was never jaundiced and the maximum level of TSB was 122 µmol/l. On follow-up, his Hb stabilised and he had no further problems. This report highlights the possibility of early onset anaemia without jaundice as the sole manifestation of Rh isoimmunisation.