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Neonatal encephalopathy (NE) is a condition of neurologic dysfunction with heterogeneous severity. The terms hypoxic-ischemic encephalopathy (HIE) and NE are often used interchangeably, although the differential diagnosis is vast. We aimed to evaluate the etiologies behind NE in newborns (NB) treated with therapeutic hypothermia (TH) for a presumed diagnosis of HIE. A retrospective analysis between January 2012 and July 2020 was conducted. Demographic data, information regarding pre- and perinatal factors, systemic dysfunction parameters, neuroimaging and neurologic sequelae were collected. A comparative analysis between the group considered with hypoxic-ischemic versus non-hypoxic-ischemic NE was performed. Forty-six NB were included. HIE was confirmed in 29 (63.0%) patients (group 1). There was no evidence of perinatal asphyxia in 17 (37.0%) patients (group 2). In the latter group, intracranial hemorrhage was the most frequent etiology (7; 15.2%), followed by infection (5; 10.9%). In group 1, there was a higher prevalence of emergency cesarean section (p = 0.013), clinical seizures at admission (p = 0.048) and a higher encephalopathy severity (p = 0.027). In this group, amplitude-integrated electroencephalogram improvement at 48 hours of TH was less frequent (p = 0.027) and major neurologic sequelae were more prevalent at 12 (p = 0.006) and 24 months (p = 0.041). HIE was the main cause of NE. Despite the clinical overlap, clinicians should recognize other etiologic factors beyond anoxic events. Our findings might help to prospectively differentiate between HIE and NE from different etiologies early after birth, ideally prior to initiation of TH, in the future.

Isolated fetal ascites is a rare condition and its natural history needs further research. We describe a case of progressive fetal isolated ascites diagnosed at 22 weeks of gestation associated with hyperechogenic bowel in a primiparous woman. Genetic abnormalities, infection and metabolic causes were excluded. An elective C-section was performed at 29 weeks of gestation after no clinical improvement was obtained with paracentesis. After birth, X-ray and ultrasonography showed high-volume ascites and enlarged liver. On the 5th day of life, no meconium had passed and an abdominal X-ray was repeated: pneumoperitoneum was diagnosed. Laparotomy identified intestinal atresia type IIIB associated with meconial peritonitis and a derivative jejunostomy was performed. There were complications caused by late-onset sepsis and necrotizing enterocolitis. Reconstruction of jejunostomy was successfully performed on day 114. At 15 months of age, the infant is in good health and has normal psychomotor development.

Introduction: Sudden and unexpected postnatal collapse is a rare event with potentially dramatic consequences. Intervention approaches are limited, but hypothermia has been considered after postnatal collapse. The aim of this study was to analyse sudden and unexpected postnatal collapse cases that underwent therapeutic hypothermia in the five Portuguese hypothermia centres. Material and Methods: In this multicentre, retrospective and descriptive study, clinical, ultrasonography, amplitude-integrated electroencephalography and brain magnetic resonance findings of newborns with postnatal collapse that underwent therapeutic hypothermia are reported (2010 - 2018). Statistical analysis was performed by using IBM SPSS Statistics version 21. Results: Twenty-two cases of sudden and unexpected postnatal collapse were referred for therapeutic hypothermia (82% outborn), all ≥ 36 weeks, with Apgar 5´ ≥ 8. Collapse occurred during the first two hours in 73% (all < 24 hours), 50% during skin-to-skin care, 55% related to feeding and 23% during co-bedding. Moderate-severe encephalopathy and severe acidosis were observed (median: Thompson score 16, pH 6.90, base deficit 22 mmol/L). Amplitude-integrated electroencephalogram was abnormal in 95% and magnetic resonance imaging showed severe brain injury in 46%. The mortality rate was 50%. A possible cause was identified in 27%. Discussion: The incidence rate of 2.7 sudden cases of postnatal collapse per 100 000 births, is possibly under-estimated. All infants suffered the collapse in the first day, mostly within the first two hours, as reported before. Possible causes were identified in less than a third of cases, but multiple predisposing conditions were identified, suggesting that prevention may be possible. Newborn positioning and skin-to-skin care have been the most discussed practices. A significant proportion of infants had poor outcomes. Lower Thompson score, electroencephalogram amplitude normalization and normal magnetic resonance imaging seemed to indicate better outcomes. Although conclusive trials on therapeutic hypothermia after postnatal collapse are not available, its use has been considered individually. No severe adverse effects directly related to hypothermia were registered in this study, but the results do not allow drawing meaningful conclusions. Conclusion: In our national sample of 22 infants who suffered sudden and unexpected postnatal collapse and underwent therapeutic hypothermia, a significant proportion had poor outcomes. Absolute conclusions from our experience with hypothermia in postnatal collapse cannot be drawn, but systematic reporting of cases and long-term clinical evaluation would facilitate understanding of the real benefits of hypothermia. As this procedure has not been validated with clinical trials for this indication, its use should be considered on a case-by-case approach. The potentially avoidable nature of unexpected postnatal collapse is evident from its association with certain behaviours and risk factors. Surveillance practices during the first hours should be implemented, whilst the benefits of breastfeeding and skin-to-skin care should continue to be widely promoted.

A patent urachus is the presence of structures found early in pregnancy, which could be associated with other abnormality in 46% cases. Hemangiomas are benign tumors and are more common in the skin. Umbilical cord hemangiomas are very rare, with fewer than 50 cases described in the literature. Here we report a clinical case of a neonate with a prenatal diagnosis of an umbilical cord hemangioma and a patent urachus. We would like to draw attention to the differential diagnosis of cord masses, as well as to the importance of early diagnosis and the appropriate management of the newborn. To our knowledge, this is the first case reported of a newborn diagnosed with this association.

Pericardial effusion (PCE) is a rare condition in neonates. The most common cause is iatrogenic due to central venous catheters (CVCs) and symptoms vary from asymptomatic to more severe presentations, such as cardiac tamponade. Treatment of this condition in neonates remains controversial. The authors present a case report of a preterm neonate with a PCE and a review of the literature. A preterm infant was born at 26 weeks of gestation and with a birth weight of 690 grams. A peripherally inserted central catheter (PICC) was inserted on day 4, which was uneventful. Due to a hemodynamically significant patent ductus arteriosus with no response to two cycles of treatment with ibuprofen, she was submitted to surgical ligation on day 38. In the postoperative echocardiogram, the tip of the PICC was seen in the right ventricle and the catheter was retracted. An echocardiogram was performed on day 55, showing a PCE with a slight diastolic compromise of the right atrium. The catheter was withdrawn and diuretic therapy with furosemide was initiated. PCE presented a gradual reduction until complete resolution within 3 weeks. A review of the literature about postnatal PCE was performed. A final sample of 34 articles was included. The main PCE causes were iatrogenic (due to CVCs, and postoperative of major cardiac surgery) and infection. Management of this condition was variable between cases ranging from clinical surveillance in asymptomatic newborns with small effusions to pericardiocentesis in large effusions with signs of hemodynamic instability. In conclusion, PCE is a condition that is often underdiagnosed in neonates. The most common cause, as occurred in the case presented above, is a mispositioned CVC. Management of this condition is still controversial and further studies are needed to establish therapeutic protocols in neonates.

Female neonate born at 29+4 gestational weeks by spontaneous vaginal delivery to a 37-year-old mother, with adequate prenatal surveillance and an uneventful pregnancy. Prophylactic antibiotics, tocolytic therapy and a single dose of steroids were given before birth. She was born with very low birth weight (1,440 grams) and with an Apgar score of 8 and 9 (first and fifth minutes, respectively). She was admitted in the Neonatal Intensive Care Unit and maintained on nCPAP due to mild respiratory distress. Within 4 hours she developed progressive respiratory insufficiency, without cardiovascular collapse. Three chest X-rays are presented. • What do you see in each chest radiograph? • What is your diagnosis? • Which procedures are recommended?

Air leak syndromes include pulmonary inter­stitial emphysema, pneumomediastinum, pneumo­thorax, pneumopericardium, pneumo­peritoneum, subcutaneous emphysema and systemic air embolism. These syndromes are more common and severe among neonates with underlying lung disease, especially those with low birth weight and respiratory distress syndrome. Antenatal steroids and early surfactant administration can prevent and improve their outcome. In the case reported, pneumopericardium was the first air leak presentation. Pericardial drainage was postponed due to cardiorespiratory stability on aminergic support. Air dissection into the pleural space led to tension pneumothorax, with gradual clinical improvement after thoracocentesis and chest tube placement. The thoracic drain was removed after 3 days with elective extubation on the 7th day of life. She was discharged home at 36 weeks of postmenstrual age without any respiratory dysfunction.

Introduction: Perfusion index (PI) is a noninvasive indirect method of microcirculation measurement that might have a potential to predict morbimortality in preterm newborns. This study aims: 1) to define a critical value for post-ductal PI related to the morbimortality in preterm newborns under 32 weeks; 2) to define a prognostic value for post-ductal PI to predict that risk of morbimortality; and 3) to associate post-ductal PI values with the Apgar score at five minutes and the Clinical Risk Index for Babies II (CRIB-II) score. Material and methods: This is an observational study, with a prospective character, performed in a Neonatal Intensive Care Unit that enrolled 34 preterm newborns with less than 32 weeks admitted between 1st February 2016 and 1st February 2017. Post-ductal PI values were evaluated in the newborns’ feet at 24 hours of life. The Apgar score was registered at birth and CRIB-II score was calculated. The other clinical variables, including the presence of an adverse outcome and/or death, was assessed in clinical records. Measurements and main results: We found significant correlations between post-ductal PI values, Apgar score at five minutes and CRIB-II score. The area under ROC curve was higher for CRIB-II score and lower for Apgar score at five minutes. The best cut-off points were: 1) post-ductal PI value ≤ 0.72; 2) Apgar score at five minutes < 6; and 3) CRIB-II score > 9. Preterms within these cohorts have higher rates of morbimortality. Post-ductal PI values ≤ 0.72 were an independent predictor of mortality in preterm newborns (p-value = 0.047). Conclusions: This study suggests that post-ductal PI value ≤ 0.72 at 24 hours of life might be a potential predictor of morbimortality in preterm newborns. However, it is important to clarify that this study has a small representative population which can reduce the accuracy of the results. The very high morbimortality rate in this cohort is another strong limitation for the quality of the results.

Introduction: Perfusion index (PI) is a noninvasive method of peripheral perfusion measurement. Previous publications suggest that PI might be an useful and accurate predictor of morbidity and mortality risk in preterm newborns. This study aims: 1) to assess the PI values of preterm newborns (< 37 weeks) in the first seven days of life according to gestational age; 2) to assess differences in PI values between healthy preterm newborns and those who developed adverse outcomes during the neonatal period. Material and methods: This is a prospective observational study performed in a Neonatal Intensive Care Unit that enrolled 60 preterm newborns with less than 37 weeks admitted between 1st February 2016 and 1st February 2017. Post-ductal PI was evaluated in the newborns’ feet in the first hour and at 24 hours, 48 hours, 72 hours and 168 hours of life. The presence of an adverse outcome and/or death in the neonatal period was assessed in clinical records, along with several other clinical variables. Results: We found a non-significant trend towards decreasing PI values in the first 2 days of life, with an increase at 48 hours and stable values at 72 hours after birth. PI values had an inverse relationship with gestational age (p for linear trend: 24 hours, p = 0.029; 48 hours, p = 0.001; 72 hours, p = 0.037; 168 hours, p = 0.001). The most prevalent adverse outcomes were shock (n = 8, 13.2%), anemia (n = 10, 16.7%) and intraventricular hemorrhage grade > 2 (n = 10, 16.7%). Median PI values were found to be significantly lower in newborns with an adverse outcome (0.90 vs. 0.70 at 24 hours; 0.87 vs. 0.72 at 48 hours; 0.91 vs. 0.79 at 72 hours; and 0.90 vs. 0.80 at 168 hours) and/or death in neonatal period (0.87 vs. 0.55 at 1 hour; 0.80 vs. 0.70 at 24 hours; 0.81 vs. 0.55 at 48 hours; 0.88 vs. 0.74 at 72 hours; and 0.89 vs. 0.49 at 168 hours). Conclusions: PI values differed according to gestational age and to the presence of comorbidities, confirming that it might represent a valuable tool in the early identification of adverse outcomes in the neonatal period.

We report the case of an infant born at 28 weeks of gestation with a history of intestinal dysmotility over the first week of life, which was solved with the introduction of erythromycin. The infant was fed with breast milk and sporadic doses of special formula for preterm newborns. On the 17th day of life, post conceptional age of 30 weeks, she presented an episode of necrotizing enterocolitis treated with antibiotics. On the 45th day of life, post conceptional age of 34 weeks, the infant presented episodes of apnea, hemodynamic instability, abdominal distension, vomiting and mucous and bloody stools. Owing to the suspicion of a new episode of necrotizing enterocolitis, feeding was stopped and antibiotic therapy was started. Hypereosinophilia was detected in peripheral blood and tests were positive for specific IgE antibodies to cow’s milk proteins. Antibiotics were stopped after negative sepsis workups and feeding with breastmilk and extensively hydrolyzed formula was resumed. The newborn presented with good tolerance. Diagnosing allergy to cow’s milk protein in a newborn infant requires a high degree of suspicion, as it presents with non-specific symptoms. In most cases it manifests as non-IgE-mediated proctocolitis and cases of enterocolitis with specific IgE antibodies to cow’s milk proteins are rare. Some authors argue that the development of cow’s milk protein allergy requires an immunological maturation level not present before a gestational age of 30-32 weeks. Therefore, in preterm newborns, there may be an asymptomatic period of life with subsequent development of an allergy. In the case described, the diagnosis of IgE-mediated cow’s milk protein allergy was confirmed at 34 weeks of post conceptional age. However, the question remains whether the previous digestive symptoms were related to the allergy subsequently diagnosed.

The pathogenesis of bronchopulmonary dysplasia (BPD) is clearly multifactorial. Specific pathogenic risk factors are prematurity, respiratory distress, oxygen supplementation, mechanical ventilation (MV), inflammation, patent ductus arteriosus (PDA), etc. Aim: To evaluate BPD prevalence and to identify risk factors for BPD in five Portuguese Neonatal Intensive Care Units in order to develop better practices the management of these newborns. Material and methods: 256 very low birth weight infants with gestational age (GA) 0.30 (85 vs 5 days, respectively in BPD and no BPD patients, pA displasia broncopulmonar (DBP) é multifactorial. Prematuridade, doença da membrana hialina, oxigénio, ventilação mecânica, inflamação e canal arterial são alguns dos factores na sua patogénese. Objectivo: Avaliar a prevalência da DBP e seus factores de risco em cinco unidades portuguesas, para implementar boas práticas no tratamento deste doentes. Material e métodos: 256 recém-nascidos (RN) com idade gestacional (IG) 0,30 (85 vs 5 dias, respectivamente nos doentes com e sem DBP, p

Resumo: A displasia broncopulmonar (DBP) é multifactorial. Prematuridade, doença da membrana hialina, oxigénio, ventilação mecânica, inflamação e canal arterial são alguns dos factores na sua patogéneseObjectivo: Avaliar a prevalência da DBP e seus factores de risco em cinco unidades portuguesas, para implementar boas práticas no tratamento deste doentes.Material e métodos: 256 recém-nascidos (RN) com idade gestacional (IG) ;0,30 (85 vs 5 dias, respectivamente nos doentes com e sem DBP, p;0.30 (85 vs 5 days, respectively in BPD and no BPD patients, p

Some experimental work suggests that exposure to intrauterine infection is associated, not only, with lung maturation and a reduced risk of respiratory distress syndrome, but also, with delayed alveolarization and increased risk of bronchopulmonary dysplasia. Aim: To evaluate the association between histological chorioamnionitis and lung disease in extremely low birth weight preterm infants. Methods: A retrospective chart review of 63 less than 1000 g birthweight, appropriated for gestational age neonates, delivered at three tertiary medical centers in the north of Portugal, between 2001 and 2002. The association between histological chorioamnionitis and lung damage (respiratory distress syndrome and bronchopulmonary dysplasia) was evaluated through the calculation of crude and adjusted odds ratio. Results: There were 32 newborns from mothers with histological chorioamnionitis and 31 without the condition. The association between histological chorioamnionitis and respiratory distress syndrome was OR 0.23 (95% CI 0.01 - 2.51). The association between chorioamnionitis and bronchopulmonary dysplasia was OR 1.61 (95% CI 0.38 - 6.97). The association between histological chorioamnionitis and bronchopulmonary dysplasia when adjusted for gestational age, multiple birth and C-section revealed no statistical significance: OR 2.66 (95% CI 0.36 - 19.60) for chorioamnionitis without funisitis or vasculitis and OR 1.68 (95% CI 0.25 - 11.18) for funisitis and/or vasculitis. Conclusion: In this study we could not confirm a decreased risk of respiratory distress syndrome nor an increased risk of bronchopulmonary dysplasia in extremely low birth weight preterm neonates with histological chorioamnionitis.Alguns estudos experimentais sugerem que a exposição a infecção intra-uterina associa-se, não só, a maturação pulmonar e menor risco de doença das membranas hialinas, mas também a atraso na formação alveolar e maior risco de displasia broncopulmonar. Objectivo: Avaliar a associação entre corioamnionite histológica e lesão pulmonar no recém-nascido pré-termo de extremo baixo peso. Métodos: Estudo retrospectivo em 63 recém-nascidos com peso ao nascimento inferior a 1000 g, apropriados à idade gestacional, nascidos em três centros hospitalares do Norte de Portugal, entre 2001 e 2002. A associação entre corioamnionite histológica e lesão pulmonar (doença das membranas hialinas e displasia broncopulmonar) foi avaliada através do cálculo de odds ratio. Resultados: Em 32 recém-nascidos as mães apresentaram corioamnionite histológica e em 31 a condição não estava presente. A associação entre corioamnionite histológica e doença das membranas hialinas foi OR 0,23 (IC 95% 0,01 - 2,51). A associação entre corioamnionite histológica e displasia broncopulmonar foi OR 1,61 (IC 95% 0,38 - 6,97). A associação entre corioamnionite histológica e displasia broncopulmonar ajustada para a idade gestacional, gestação múltipla e parto por cesariana não foi estatisticamente significativa: OR 2,66 (IC 95% 0,36 - 19,60) para orioamnionite sem funisite ou vasculite e OR 1,68 (IC 95% 0,25 - 11,18) para corioamnionite com funisite e/ou vasculite. Conclusão: Este estudo não nos permitiu confirmar a existência de menor risco de doença das membranas hialinas ou de maior risco de displasia broncopulmonar no recém-nascido pré-termo de extremo baixo peso com corioamnionite histológica.

Introdução: O espectro clínico da malformação adenomatóide cística pulmonar congénita (MACPC) varia desde lesões assintomáticas e dificuldade respiratóriano recém-nascido a hidrópsia fetal. Objectivos: Avaliação da casuística de MACPC, realçando a história natural, tratamento e evolução. Material e métodos: Análise retrospectiva dos processos clínicos de todos os recém-nascidos com o diagnóstico de MACPC, admitidos nas unidades de cuidados intensivos neonatais de cinco centros terciários do Norte de Portugal, entre 1996 e 2005. Resultados: Foram identificados 15 recém-nascidos, 9F/6M, peso ao nascimento 3100 g (645-3975), idade gestacional de 38 semanas (24-40). A incidência de MACPC foi de 1:9300 nascimentos. Onze (73%) recém-nascidos apresentaram diagnóstico pré-natal de lesão pulmonar cística pelas 22 semanas (19-30). Esta ocorreu no hemitórax direito em seis (40%) casos e no lado esquerdo em 9 (60%). Foi documentada regressão in utero da lesão em dois casos. Foi necessária intervenção ante-natal (pleurocentese e colocação de derivação toraco-amniótica) num feto com hidrópsia fetal. Ao nascimento, cinco recém-nascidos apresentaram radiografia de tórax dentro da normalidade, sendo a tomografia axial computorizada diagnóstica da lesão. Três (20%) recém-nascidos tornaram-se sintomáticos durante o período neonatal (dificuldade respiratória) e um (7%) após o período neonatal (pneumotórax espontâneo). Dois (13%) faleceram. Seis (40%) doentes foram submetidos a toracotomia e exérese da lesão. O estudo anatomopatológico demonstrou achados definitivos de MACPC (classificação de Stoker: tipo I = 4; tipo II = 1; tipo III = 2). Oito (53%) crianças mantiveram-se assintomáticas e permanecem em vigilância clínica. Conclusões: A MACPC com diagnóstico pré-natal tem bom prognóstico na ausência de sofrimento fetal; uma radiografia de tórax sem alterações ao nascimento não exclui MACPC; o tratamento da lesão assintomática é controverso; a cirurgia está indicada devido à baixa morbilidade e possibilidade de prevenção de complicações tardias, como a degenerescência maligna.Background: The clinical spectrum of congenital cystic adenomatoid malformation of the lung (CCAML) ranges from asymptomatic lesions to neonatal respiratory distress and hydrops fetalis. Aim: To review our experience with CCAML, emphasising natural history, management and outcome. Material and methods: A retrospective chart review of all CCAML-diagnosed neonates admitted to the neonatal intensive care units of five tertiary medical centres in the north of Portugal between 1996 and 2005. Results: Fifteen neonates with CCAML were identified, 9F/6M, birth weight 3100 g (645-3975), gestational age 38 weeks (24-40). The incidence of CCAML was 1: 9300 births. There were 11 (73%) cases of cystic lung lesion diagnosed during pregnancy, median age 22 weeks (19-30). The lesion was right sided in six (40%) and left sided in nine (60%) cases. In utero spontaneous regression of the lesion was observed in two cases. Antenatal intervention (pleurocentesis and thoracoamniotic shunting) was performed in one foetus with impending hydrops. Normal lung radiographic findings at birth were present in five cases, with an abnormal CT scan. Three (20%) neonates became symptomatic during the neonatal period (respiratory distress) and one (70%) after the neonatal period (spontaneous pneumothorax). Two neonates (13%) died. Six (40%) patients underwent thoracotomy and appropriate excisional surgery. Histological examination showed definitive features of CCAML (Stocker classification: type I = 4; type II = 1; type III = 2). Eight (53%) patients remain asymptomatic and did not undergo surgery. Conclusions: Antenatally diagnosed CCAML has a good prognosis in the absence of severe foetal distress; normal radiographic findings at birth do not rule out CCAML; treatment of asymptomatic CCAML is controversial; surgery may be advocated because of its low morbidity and the prevention of late complications such as malignancy.

Hemophilia A is a X-linked hereditary condition that lead to decreased factor VIII activity, occurs mainly in males. Decreased factor VIII activity leads to increased risk of bleeding events. During neonatal period, diagnosis is made after post-partum bleeding complication or unexpected bleeding after medical procedures. Subgaleal hemorrhage during neonatal period is a rare, severe extracranial bleeding with high mortality and usually related to traumatic labor or coagulation disorders. Subgaleal hemorrhage complications result from massive bleeding. We present a neonate with unremarkable family history and uneventful pregnancy with a vaginal delivery with no instrumentation, presenting with severe subgaleal bleeding at 52 hours of life. Aggressive support measures were implemented and bleeding managed. The unexpected bleeding lead to a coagulation study and the diagnosis of severe hemophilia A. There were no known sequelae. This case shows a rare hemophilia presentation reflecting the importance of coagulation studies when faced with unexplained severe bleeding.

Noonan syndrome is a relatively common and heterogeneous genetic disorder, associated with congenital heart defect in about 50% of the cases. If the defect is not severe, life expectancy is normal. We report a case of Noonan syndrome in a preterm infant with hypertrophic cardiomyopathy and lethal outcome associated to acute respiratory distress syndrome caused by Adenovirus pneumonia. A novel mutation in the RAF1 gene was identified: c.782C>G (p.Pro261Arg) in heterozygosity, not described previously in the literature. Consequently, the common clinical course in this mutation and its respective contribution to the early fatal outcome is unknown. No conclusion can be established regarding genotype/phenotype correlation.

Respiratory syncytial virus (RSV) lower respiratory tract infection is the most common viral respiratory infection in infants. Several authors have sought to determine which risk factors are the best predictors for severe RSV disease. Our aim was to evaluate if a specific chest radiographic pattern in RSV disease can predict the disease severity. We conducted a multicenter retrospective cohort study in term and preterm neonates with confirmed lower respiratory tract RSV infection, admitted to neonatal intensive care units (NICU) from 2000 to 2010. To determine which factors independently predicted the outcomes, multivariate logistic regression analysis was performed. A total of 259 term and preterm neonates were enrolled. Patients with a consolidation pattern on the chest radiograph at admission (n=101) had greater need for invasive mechanical ventilation (OR: 2.5; P=.015), respiratory support (OR: 2.3; P=.005), supplemental oxygen (OR: 3.0; P=.008), and prolonged stay in the NICU (>7 days) (OR: 1.8; P=.025). Newborns with a consolidation pattern on admission chest radiograph had a more severe disease course, with greater risk of invasive mechanical ventilation, respiratory support, supplemental oxygen, and prolonged hospitalization.

Liver and biliary tract disorders in the neonate are relatively rare and often complex.To evaluate the incidence of neonatal liver and biliary tract disorders, main causes, clinical presentation, treatment and outcome. Material andClinical, imagiological, laboratory, pathological and autopsy data concerning all newborns with liver and biliary tract disorder admitted to the neonatal intensive care unit of five tertiary medical centers from the north of Portugal, between 1997 and 2006, were retrospectively analysed.77 neonates (incidence 0.5% - 77/14505 admissions); 44M/33F; gestational age 34 weeks (25-41); preterm 50 (65%); birthweight 1980 g (570-4130), < 1500 g 29 (38%). Several causes were identified and classified as infectious, metabolic, anatomic/structural, neoplastic, vascular, traumatic, immune, genetic and idiopathic. Clinical signs appeared between days 1 and 61 of life. Jaundice was the most frequent clinical sign (92%). Cholestasis occurred in 67 (87%) patients. Duration of hospital stay was 35 days (5-146); 18 patients (23%) were deceased. Autopsy study was diagnostic in 8 cases (10%).Nosocomial and intrauterine infection were the most common causes of liver and biliary tract disease. Several other rare causes represented an important challenge in diagnosis and treatment, and some were fatal. Awareness of the spectrum of liver and bile duct disorders in the neonate and recognition of the key clinical features are essential to optimize outcome.

Several studies highlight the association between perinatal infection/inflammation and neonatal morbidity, mainly bronchopulmonary dysplasia and periventricular leukomalacia.To evaluate the role of histological chorioamnionitis on the overall morbidity of preterm newborns.A retrospective study on preterm newborns less than 34 weeks gestational age at birth, and respective mothers, at three tertiary medical centers (Hospital de São João, Maternidade Júlio Dinis and Centro Hospitalar de Vila Nova de Gaia) in the north of Portugal, between January 2001 and December 2002. We evaluated the association between histological chorioamnionitis and the overall neonatal morbidity. The association between histological chorioamnionitis and acute (respiratory distress syndrome) and chronic (bronchopulmonary dysplasia) lung damage was also evaluated in the subgroup of less than 1000 g birthweight preterm neonates.452 [ M 253 / F 217; birthweight 1440 (515-2620) g; gestational age 31 (23-33) weeks] preterm newborns were included. The association between histological chorioamnionitis and the overall neonatal morbidity was: respiratory distress syndrome OR 1.5 (95% CI 0.94-2.31); bronchopulmonary dysplasia OR 2.6 (95% CI 1.16-6.03); patent ductus arteriosus OR 2.5 (95% CI 1.17-5.44); sepsis OR 1.2 (95% CI 0.9-2.13); necrotizing enterocolitis OR 1.4 (95% CI 0.9-1.76); intraventricular hemorrhage grades III-IV OR 2.5 (1.20-5.11); cystic periventricular leukomalacia OR 3.0 (1.5-6.07); retinopathy of prematurity OR 1.4 (95% CI 0.8-1.35). The association adjusted to birthweight and gestational age was: bronchopulmonary dysplasia OR 1.2 (95% CI 0.51-2.95); patent ductus arteriosus OR 0.9 (95% CI 0.4-2.35); intraventricular hemorrhage grades III-IV OR 0.9 (95% CI 0.39-2.28); cystic periventricular leukomalacia OR 2.2 (95% CI 1.03-4.61). The association between histological chorioamnionitis and lung damage in the subgroup of less than 1000 g birthweight preterm neonates was: respiratory distress syndrome OR 0.23 (95% CI 0.01-2.51); bronchopulmonary dysplasia OR 1.61 (95% CI 0.38-6.97).This study confirms the association between histological chorioamnionitis and cystic periventricular leukomalacia of the preterm newborn.

Congenital diarrhea comprises a broad range of pathologies and often requires a thor- ough workup and immediate treatment. Although rare, microvillous inclusion disease (MVID) should be included in differential diagnosis of this presentation in the neonate. We report the case of a 36-week newborn who developed signs of severe dehydration and lethargy, requiring fluid resuscitation and total parenteral nutrition. MVID was diagnosed by recognition of profuse secretory diarrhea after an exhaustive etiological investigation, confirmed by DNA analysis.