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Mitochondrial diseases are a group of inherited diseases with highly varied and complex clinical presentations. Here, we report four individuals, including two siblings, affected by a progressive mitochondrial encephalopathy with biallelic variants in the cardiolipin biosynthesis gene CRLS1. Three affected individuals had a similar infantile presentation comprising progressive encephalopathy, bull's eye maculopathy, auditory neuropathy, diabetes insipidus, autonomic instability, cardiac defects and early death. The fourth affected individual presented with chronic encephalopathy with neurodevelopmental regression, congenital nystagmus with decreased vision, sensorineural hearing loss, failure to thrive and acquired microcephaly. Using patient-derived fibroblasts, we characterized cardiolipin synthase 1 (CRLS1) dysfunction that impaired mitochondrial morphology and biogenesis, providing functional evidence that the CRLS1 variants cause mitochondrial disease. Lipid profiling in fibroblasts from two patients further confirmed the functional defect demonstrating reduced cardiolipin levels, altered acyl-chain composition and significantly increased levels of phosphatidylglycerol, the substrate of CRLS1. Proteomic profiling of patient cells and mouse Crls1 knockout cell lines identified both endoplasmic reticular and mitochondrial stress responses, and key features that distinguish between varying degrees of cardiolipin insufficiency. These findings support that deleterious variants in CRLS1 cause an autosomal recessive mitochondrial disease, presenting as a severe encephalopathy with multi-systemic involvement. Furthermore, we identify key signatures in cardiolipin and proteome profiles across various degrees of cardiolipin loss, facilitating the use of omics technologies to guide future diagnosis of mitochondrial diseases.
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Encefalopatias , Doenças Mitocondriais , Animais , Camundongos , Encefalopatias/genética , Encefalopatias/metabolismo , Cardiolipinas/genética , Cardiolipinas/metabolismo , Mitocôndrias/genética , Mitocôndrias/metabolismo , Doenças Mitocondriais/genética , Doenças Mitocondriais/metabolismo , ProteômicaRESUMO
BACKGROUND: Are thermoregulation and golden hour practices in extremely preterm (EP) infants comparable across the world? This study aims to describe these practices for EP infants based on the neonatal intensive care unit's (NICUs) geographic region, country's income status and the lowest gestational age (GA) of infants resuscitated. METHODS: The Director of each NICU was requested to complete the e-questionnaire between February 2019 and August 2021. RESULTS: We received 848 responses, from all geographic regions and resource settings. Variations in most thermoregulation and golden hour practices were observed. Using a polyethylene plastic wrap, commencing humidity within 60 min of admission, and having local protocols were the most consistent practices (>75%). The odds for the following practices differed in NICUs resuscitating infants from 22 to 23 weeks GA compared to those resuscitating from 24 to 25 weeks: respiratory support during resuscitation and transport, use of polyethylene plastic wrap and servo-control mode, commencing ambient humidity >80% and presence of local protocols. CONCLUSION: Evidence-based practices on thermoregulation and golden hour stabilisation differed based on the unit's region, country's income status and the lowest GA of infants resuscitated. Future efforts should address reducing variation in practice and aligning practices with international guidelines. IMPACT: A wide variation in thermoregulation and golden hour practices exists depending on the income status, geographic region and lowest gestation age of infants resuscitated. Using a polyethylene plastic wrap, commencing humidity within 60 min of admission and having local protocols were the most consistent practices. This study provides a comprehensive description of thermoregulation and golden hour practices to allow a global comparison in the delivery of best evidence-based practice. The findings of this survey highlight a need for reducing variation in practice and aligning practices with international guidelines for a comparable health care delivery.
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Hipotermia , Lactente Extremamente Prematuro , Feminino , Humanos , Recém-Nascido , Masculino , Gravidez , Regulação da Temperatura Corporal , Hipotermia/prevenção & controle , Unidades de Terapia Intensiva Neonatal , Polietilenos , Inquéritos e QuestionáriosRESUMO
AIM: To investigate skincare practices in the first 2 weeks of life in extremely premature infants across tertiary neonatal intensive care units (NICUs). METHODS: A web-based secure survey invite was emailed to the medical directors of tertiary NICUs. The survey included questions on various aspects of skincare practices in the first 2 weeks of life in extremely premature infants (born before 28 weeks gestation). The person most familiar with local skincare practices was asked to complete the survey and only one response per unit was requested. We performed a descriptive analysis. RESULTS: We received responses from 30 out of 32 NICUs (response rate 93%). Twenty-five NICUs (89%) reported offering resuscitation and intensive care to infants born at ≥23 weeks gestation. All NICUs reported occurrences of skin breakdown, including medical adhesive-related skin injury (30%), abrasion/friction-associated skin injury (46%), perineal skin breakdown (55%), pressure site injury (47%) and diaper dermatitis (60%). A high level of consensus (≥75%) was observed for certain practices, such as the use of polyethylene occlusive plastic wraps at birth and aqueous chlorhexidine solution for sterile procedures, but a low level of consensus (<25%) was observed for many other practices, including the skin risk assessment tool used. CONCLUSIONS: Skin injuries in extremely premature infants are common and skincare practices vary considerably amongst NICUs. Clinical practice improvement projects and further clinical research will help improve consistency amongst NICUs. Further research is needed to assist the development of evidence-based guidelines and benchmarking for skincare practices in these vulnerable infants.
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Lactente Extremamente Prematuro , Unidades de Terapia Intensiva Neonatal , Austrália , Humanos , Recém-Nascido , Nova Zelândia , Inquéritos e QuestionáriosRESUMO
BACKGROUND: Pneumomediastinum is an uncommon cause of neonatal respiratory distress. Clinical history and examination of the neonate may be uninformative in determining the aetiology of the respiratory distress. Chest x-ray can be diagnostic of pneumomediastinum however is often difficult to interpret. CASE PRESENTATION: A 36 week gestation newborn was delivered by emergency caesarean section, intubated and given intermittent positive pressure ventilation via Neopuff™ for apnoea before being extubated to CPAP. Chest radiography initially showed bilateral upper zone opacities, presenting a diagnostic dilemma; however subsequent films demonstrated a pneumomediastinum which was managed conservatively and resolved. CONCLUSIONS: The "sail sign" describes an uncommon radiological appearance of a pneumomediastinum in neonates and infants. With careful conservative management, a spontaneous resolution without longterm sequelae can be expected.
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Enfisema Mediastínico/diagnóstico por imagem , Radiografia Torácica , Humanos , Recém-Nascido , MasculinoRESUMO
BACKGROUND: Transient Neonatal Diabetes Mellitus is the commonest cause of diabetes presenting in the first week of life. Majority of infants recover by 3 months of age but are predisposed to developing type 2 diabetes later on in life. This condition is usually due to genetic aberrations at the 6q24 gene locus, and can be sporadic or inherited. This disorder has three phases: neonatal diabetes, apparent remission, relapse of diabetes. CASE PRESENTATION: Our case, a neonate presented with low birth weight and growth retardation along with the metabolic profile consistent with transient diabetes mellitus at birth. We report a novel clinical observation of recurrent asymptomatic hypoglycaemia detected on pre-feed blood glucose level monitoring in our case with transient neonatal diabetes mellitus at 6 weeks of age, 4 weeks after the remission of diabetes mellitus. CONCLUSION: This case demonstrates that neonates in remission following transient diabetes mellitus can present with recurrent asymptomatic hypoglycaemia without any other obvious congenital malformations seen. This asymptomatic hypoglycaemia may persist for weeks and may be missed if pre-feed blood glucose level monitoring is not done in these infants. Also, these infants may require an aggressive enteral feeding regimen with high glucose delivery rate to maintain normoglycemia.
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Glicemia/metabolismo , Diabetes Mellitus Tipo 1/sangue , Hipoglicemia/sangue , Recém-Nascido de Baixo Peso , Doenças do Recém-Nascido/sangue , Humanos , Recém-Nascido , Insulina/sangue , MasculinoRESUMO
AIM: Preterm infants with respiratory distress syndrome (RDS) requiring surfactant treatment are often retrieved mechanically ventilated to the receiving hospital. INSURE (INtubate, SURfactant, Extubate) technique is not routinely performed by Newborn and Pediatric Emergency Transport Services NSW (NETS) during retrieval. This study aims to evaluate the likelihood of using INSURE technique during retrieval. We attempted to study the clinical characteristics of preterm infants with RDS who were favourably extubated (FE) shortly after admission to the receiving hospital. METHODS: Retrospective study of preterm infants, gestational age (GA) > 28 weeks with RDS requiring retrieval by NETS. RESULTS: Two hundred twenty-three infants, median GA of 33 weeks (range 29-36), median birthweight 2200 g (1000-4080) were examined. A percentage of 49.7 received CPAP, and 50.3% required MV. Eighteen (16%) infants were FE (<6 h) at receiving hospital. FiO2 on stabilisation (FiO2 (st)) by NETS correlated with FiO2 on admission to receiving hospital (r = 0.863). A percentage of 81 of ventilated infants received premedications including morphine. No significant differences were noted for GA, stabilisation ventilator settings, surfactant dose (mean 155 mg/kg) and mode of transport between FE and non-FE groups. FiO2 (st) post-surfactant treatment was significantly lower in FE compared with non-FE group (mean 0.28 vs. 0.41 respectively). The area under the curve from receiver operating characteristic based on FiO2 (st) was 0.646 (P = 0.050), the sensitivity and specificity of FiO2 (st) cut-off points (between 0.25 and 0.30) was low. CONCLUSION: FiO2 on stabilisation post-surfactant treatment has a weak predictive value and may not be adequate to be used as sole criteria to extubate to CPAP prior to transport. FiO2 at stabilisation should be included as an eligibility criteria for a randomised trial of INSURE during retrieval, but other clinical assessments are needed.
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Extubação/normas , Pressão Positiva Contínua nas Vias Aéreas/métodos , Recém-Nascido Prematuro , Oxigênio/análise , Surfactantes Pulmonares/uso terapêutico , Síndrome do Desconforto Respiratório do Recém-Nascido/terapia , Extubação/métodos , Feminino , Idade Gestacional , Humanos , Recém-Nascido , Masculino , Oxigênio/administração & dosagem , Gravidez , Surfactantes Pulmonares/administração & dosagem , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Ventiladores Mecânicos/normasRESUMO
BACKGROUND: Respiratory function monitors (RFMs) have been used extensively in manikin and infant studies yet have not become the standard of training. We report the outcomes of a new portable, lightweight RFM, the Juno, designed to show mask leak and deflation tidal volume to assist in positive pressure ventilation (PPV) competency training using manikins. METHODS: Two leak-free manikins (preterm and term) were used. Participants provided PPV to manikins using two randomised devices, self-inflating bag (SIB) and T-piece resuscitator (TPR), with Juno display initially blinded then unblinded in four 90 s paired sequences, aiming for adequate chest wall rise and target minimal mask leak with appropriate target delivered volume when using the monitor. RESULTS: 49 experienced neonatal staff delivered 15 569 inflations to the term manikin and 14 580 inflations to the preterm. Comparing blinded to unblinded RFM display, there were significant reductions in all groups in the number of inflations out of target range volumes (preterm: SIB 22.6-6.6%, TPR 7.1-4.2% and term: SIB 54.8-37.8%, TPR 67.2-63.8%). The percentage of mask leak inflations >60% was reduced in preterm: SIB 20.7-7.2%, TPR 23.4-7.4% and in term: SIB 8.7-3.6%, TPR 23.5-6.2%). CONCLUSIONS: Using the Juno monitor during simulated resuscitation significantly improved mask leak and delivered ventilation among otherwise experienced staff using preterm and term manikins. The Juno is a novel RFM that may assist in teaching and self-assessment of resuscitation PPV technique.
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For the care of neonatal infants, abdominal auscultation is considered a safe, convenient, and inexpensive method to monitor bowel conditions. With the help of early automated detection of bowel dysfunction, neonatologists could create a diagnosis plan for early intervention. In this article, a novel technique is proposed for automated peristalsis sound detection from neonatal abdominal sound recordings and compared to various other machine learning approaches. It adopts an ensemble approach that utilises handcrafted as well as one and two dimensional deep features obtained from Mel Frequency Cepstral Coefficients (MFCCs). The results are then refined with the help of a hierarchical Hidden Semi-Markov Models (HSMM) strategy. We evaluate our method on abdominal sounds collected from 49 newborn infants admitted to our tertiary Neonatal Intensive Care Unit (NICU). The results of leave-one-patient-out cross validation show that our method provides an accuracy of 95.1% and an Area Under Curve (AUC) of 85.6%, outperforming both the baselines and the recent works significantly. These encouraging results show that our proposed Ensemble-based Deep Learning model is helpful for neonatologists to facilitate tele-health applications.
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Auscultação , Aprendizado de Máquina , Recém-Nascido , Lactente , Humanos , Unidades de Terapia Intensiva NeonatalRESUMO
Introduction: Assessment of bowel health in ill preterm infants is essential to prevent and diagnose early potentially life-threatening intestinal conditions such as necrotizing enterocolitis. Auscultation of bowel sounds helps assess peristalsis and is an essential component of this assessment. Aim: We aim to compare conventional bowel sound auscultation using acoustic recordings from an electronic stethoscope to real-time bowel motility visualized on point-of-care bowel ultrasound (US) in neonates with no known bowel disease. Methods: This is a prospective observational cohort study in neonates on full enteral feeds with no known bowel disease. A 3M™ Littmann® Model 3200 electronic stethoscope was used to obtain a continuous 60-s recording of bowel sounds at a set region over the abdomen, with a concurrent recording of US using a 12l high-frequency Linear probe. The bowel sounds heard by the first investigator using the stethoscope were contemporaneously transferred for a computerized assessment of their electronic waveforms. The second investigator, blinded to the auscultation findings, obtained bowel US images using a 12l Linear US probe. All recordings were analyzed for bowel peristalsis (duration in seconds) by each of the two methods. Results: We recruited 30 neonates (gestational age range 27-43 weeks) on full enteral feeds with no known bowel disease. The detection of bowel peristalsis (duration in seconds) by both methods (acoustic and US) was reported as a percentage of the total recording time for each participant. Comparing the time segments of bowel sound detection by digital stethoscope recording to that of the visual detection of bowel movements in US revealed a median time of peristalsis with US of 58%, compared to 88.3% with acoustic assessment (p < 0.002). The median regression difference was 26.7% [95% confidence interval (CI) 5%-48%], demonstrating no correlation between the two methods. Conclusion: Our study demonstrates disconcordance between the detection of bowel sounds by auscultation and the detection of bowel motility in real time using US in neonates on full enteral feeds and with no known bowel disease. Better innovative methods using artificial intelligence to characterize bowel sounds, integrating acoustic mapping with sonographic detection of bowel peristalsis, will allow us to develop continuous neonatal bowel sound monitoring devices.
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This review describes the sonographic appearances of the neonatal bowel in Necrotising enterocolitis. It compares these findings to those seen in midgut-Volvulus, obstructive intestinal conditions such as milk-curd obstruction, and slow gut motility in preterm infants on continuous positive airway pressure (CPAP)-CPAP belly syndrome. Point-of-care bowel ultrasound is also helpful in ruling out severe and active intestinal conditions, reassuring clinicians when the diagnosis is unclear in a non-specific clinical presentation where NEC cannot be excluded. As NEC is a severe disease, it is often over-diagnosed, mainly due to a lack of reliable biomarkers and clinical presentation similar to sepsis in neonates. Thus, the assessment of the bowel in real-time would allow clinicians to determine the timing of re-initiation of feeds and would also be reassuring based on specific typical bowel characteristics visualised on the ultrasound.
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BACKGROUND: Globally, are skincare practices and skin injuries in extremely preterm infants comparable? This study describes skin injuries, variation in skincare practices and investigates any association between them. METHODS: A web-based survey was conducted between February 2019 and August 2021. Quantifying skin injuries and describing skincare practices in extremely preterm infants were the main outcomes. The association between skin injuries and skincare practices was established using binary multivariable logistic regression adjusted for regions. RESULTS: Responses from 848 neonatal intensive care units, representing all geographic regions and income status groups were received. Diaper dermatitis (331/840, 39%) and medical adhesive-related skin injuries (319/838, 38%) were the most common injuries. Following a local skincare guideline reduced skin injuries [medical adhesive-related injuries: adjusted odds ratios (aOR) = 0.63, 95% confidence interval (CI) = 0.45-0.88; perineal injuries: aOR = 0.66, 95% CI = 0.45-0.96; local skin infections: OR = 0.41, 95% CI = 0.26-0.65; chemical burns: OR = 0.46, 95% CI = 0.26-0.83; thermal burns: OR = 0.51, 95% CI = 0.27-0.96]. Performing skin assessments at least every four hours reduced skin injuries (abrasion: aOR = 0.48, 95% CI = 0.33-0.67; pressure: aOR = 0.51, 95% CI = 0.34-0.78; diaper dermatitis: aOR = 0.71, 95% CI = 0.51-0.99; perineal: aOR = 0.52, 95% CI = 0.36-0.75). Regional and resource settings-based variations in skin injuries and skincare practices were observed. CONCLUSIONS: Skin injuries were common in extremely preterm infants. Consistency in practice and improved surveillance appears to reduce the occurrence of these injuries. Better evidence regarding optimal practices is needed to reduce skin injuries and minimize practice variations.
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Dermatite , Lactente Extremamente Prematuro , Lactente , Recém-Nascido , Humanos , Unidades de Terapia Intensiva Neonatal , Higiene da Pele , Modelos LogísticosRESUMO
Background: The administration of live microbiota (probiotic) via enteral route to preterm infants facilitates intestinal colonization with beneficial bacteria, resulting in competitive inhibition of the growth of pathogenic bacteria preventing gut microbiome dysbiosis. This dysbiosis is linked to the pathogenesis of necrotizing enterocolitis (NEC), an acquired multi-factorial intestinal disease characterized by microbial invasion of the gut mucosa, particularly affecting preterm infants. Probiotic prophylaxis reduces NEC; however, variations in strain-specific probiotic effects, differences in administration protocols, and synergistic interactions with the use of combination strains have all led to challenges in selecting the optimal probiotic for clinical use. Aim: To compare any differences in NEC rates, feeding outcomes, co-morbidities in preterm infants receiving single or two-strain probiotics over a 4-year period. The two-strain probiotic prophylaxis was sequentially switched over after 2 years to the single strain probiotic within this 4-year study period, in similar cohort of preterm infants. Methods: During two consecutive equal 2-year epochs, preterm infants (<32 weeks and or with birth weight <1,500 g) receiving two-strain (Lactobacillus acidophilus and Bifidobacterium bifidum) and single strain (Bifidobacterium breve M-16 V,) probiotic prophylaxis for prevention of NEC were included in this retrospective, observational study. The primary outcome included rates of NEC; secondary outcomes included prematurity related co-morbidities and feeding outcomes. Time to reach full enteral feeds was identified as the first day of introducing milk feeds at 150 ml/kg/day. Results: There were 180 preterm infants in the two-strain, 196 in the single strain group from the two equal consecutive 2-year epochs. There were no differences in the NEC rates, feeding outcomes, all-cause morbidities except for differences in rates of retinopathy of prematurity. Conclusion: In our intensive-care setting, clinical outcomes of single vs. two-strain probiotic prophylaxis for prevention of NEC were similar. Although our study demonstrates single strain probiotic may be equally effective than two-strain in the prevention of NEC, small sample size and low baseline incidence of NEC in our unit were not sufficiently powered to compare single vs. two-strain probiotic prophylaxis in preventing NEC. Further clustered randomized controlled trials are required to study the effects of single vs. multi-strain probiotic products for NEC prevention in preterm infants.
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INTRODUCTION: This review acquaints neonatal clinicians using point-of-care ultrasound with a range of pathological bowel ultrasound findings, with the aim to promote utility of this skill as a diagnostic assessment tool in diseased neonatal intestinal states. OVERVIEW: A range of normal and pathological bowel ultrasound findings are illustrated with case examples from our neonatal intensive care unit. The technical challenges of lack of familiarity with sonographic appearance of bowel (healthy and diseased), occurrence of gas artefacts and requirements of high-resolution linear transducer probes are described to allow the development of skills amongst neonatologists trained in point-of-care ultrasound. Plain abdominal radiography continues to remain the standard investigation to differentiate benign bowel states such as continuous positive airway pressure (CPAP) belly syndrome in preterm infants from life-threatening pathological intestinal states such as necrotising enterocolitis. Although plain radiography is the gold standard modality in the evaluation of neonatal diseased bowel states, real-time point-of-care bowel ultrasound performed in conjunction can provide valuable information on bowel peristalsis, bowel wall thickness and bowel vascularity. Abnormal configuration of superior mesenteric vessels on colour Doppler can alert the clinician to the diagnosis of neonatal intestinal malrotation-a time critical emergency. CONCLUSION: Further research is needed to explore true-negative and true-positive predictive values of bowel ultrasound. However, with expansion of knowledge, appropriate training of techniques, neonatologists will be able to enhance their diagnostic acumen by performing point-of-care bowel ultrasound in conjunction with plain radiography in the evaluation of broad array of neonatal intestinal states.
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BACKGROUND: International neonatal resuscitation guidelines recommend the use of laryngeal mask airway (LMA) with newborn infants (≥34 weeks' gestation or >2 kg weight) when bag-mask ventilation (BMV) or tracheal intubation is unsuccessful. Previous publications do not allow broad LMA device comparison. OBJECTIVE: To compare delivered ventilation of seven brands of size 1 LMA devices with two brands of face mask using self-inflating bag (SIB). DESIGN: 40 experienced neonatal staff provided inflation cycles using SIB with positive end expiratory pressure (PEEP) (5 cmH2O) to a specialised newborn/infant training manikin randomised for each LMA and face mask. All subjects received prior education in LMA insertion and BMV. RESULTS: 12 415 recorded inflations for LMAs and face masks were analysed. Leak detected was lowest with i-gel brand, with a mean of 5.7% compared with face mask (triangular 42.7, round 35.7) and other LMAs (45.5-65.4) (p<0.001). Peak inspiratory pressure was higher with i-gel, with a mean of 28.9 cmH2O compared with face mask (triangular 22.8, round 25.8) and other LMAs (14.3-22.0) (p<0.001). PEEP was higher with i-gel, with a mean of 5.1 cmH2O compared with face mask (triangular 3.0, round 3.6) and other LMAs (0.6-2.6) (p<0.001). In contrast to other LMAs examined, i-gel had no insertion failures and all users found i-gel easy to use. CONCLUSION: This study has shown dramatic performance differences in delivered ventilation, mask leak and ease of use among seven different brands of LMA tested in a manikin model. This coupled with no partial or complete insertion failures and ease of use suggests i-gel LMA may have an expanded role with newborn resuscitation as a primary resuscitation device.
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Reanimação Cardiopulmonar/instrumentação , Intubação Intratraqueal/instrumentação , Máscaras Laríngeas , Respiração com Pressão Positiva/instrumentação , Estudos Cross-Over , Humanos , Lactente , Recém-Nascido , Intubação Intratraqueal/efeitos adversos , Manequins , Respiração com Pressão Positiva/efeitos adversosRESUMO
INTRODUCTION: To assess the accuracy of neonatal clinician-performed cardiac ultrasound (CPU) in detection of congenital heart disease (CHD) in newborn infants with no antenatally suspected cardiac abnormality. METHODS: We reviewed records of all infants diagnosed with CHD, identified all new cases of CHD detected by CPU and classified them as 'severe' or 'other'; the 'severe CHD' was further classified as 'critical' or 'non-critical CHD'. We compared the CPU diagnosis to the final diagnosis as per paediatric cardiologist echocardiography. RESULTS: A total of 357 infants identified to have any form of CHD; 50 newly diagnosed by neonatal CPU: 21 'severe' and 29 'other' CHD. The neonatologist CPU had a high concordance rate with the final diagnosis. There were three incorrect diagnoses identified by CPU. CONCLUSION: This audit demonstrates high accuracy, in our unit, of neonatal CPU in identifying new cases of CHD not suspected antenatally. A neonatal CPU could enhance the pickup rate and, with appropriate referral to a paediatric cardiologist, improve the clinical care of infants born with CHD.
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BACKGROUND: The T-piece resuscitator (TPR) is in common use worldwide to deliver positive pressure ventilation during resuscitation of infants <10â kg. Ease of use, ability to provide positive end-expiratory pressure (PEEP), availability of devices inbuilt into resuscitaires and cheaper disposable options have increased its popularity as a first-line device for term infant resuscitation. Research into its ventilation performance is limited to preterm infant and animal studies. Efficacy of providing PEEP and the use of TPR during term infant resuscitation are not established. AIM: The aim of this study is to determine if delivered ventilation with the Neopuff brand TPR varied with differing (preterm to term) test lung compliances (Crs) and set peak inspiratory pressures (PIP). DESIGN: A single operator experienced in newborn resuscitation provided positive pressure ventilation in a randomised sequence to three different Crs models (0.5, 1 and 3â mL/cmH2O) at three different set PIP (20, 30 and 40â cmH2O). Set PEEP (5â cmH2O), gas flow rate and inflation rate were the same for each sequence. RESULTS: A total of 1087 inflations were analysed. The delivered mean PEEP was Crs dependent across set PIP range, rising from 4.9 to 8.2â cmH2O. At set PIP 40â cmH2O and Crs 3â mL/cmH2O, the delivered mean PIP was significantly lower at 35.3â cmH2O. CONCLUSIONS: As Crs increases, the Neopuff TPR can produce clinically significant levels of auto-PEEP and thus may not be optimal for the resuscitation of term infants with healthy lungs.
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Recém-Nascido/fisiologia , Respiração com Pressão Positiva/instrumentação , Ressuscitação/instrumentação , Desenho de Equipamento , Humanos , Cuidado do Lactente/instrumentação , Cuidado do Lactente/métodos , Inalação/fisiologia , Complacência Pulmonar/fisiologia , Respiração com Pressão Positiva/métodos , Distribuição Aleatória , Ressuscitação/métodos , Volume de Ventilação Pulmonar/fisiologiaRESUMO
Vein of Galen aneurysmal malformation (VGAM) is a rare congenital vascular malformation. We report a case of VGAM being associated with presence of bilateral hydrocephalus, gross cardiomegaly and is unique in two ways. Firstly, clinical presentation was associated with a large discrete cystic swelling along right side of the neck, which has not previously been reported. Secondly, this was a missed diagnosis of VGAM due to omission of colour Doppler study while performing diagnostic fetal cranial ultrasound just prior to delivery, following referral for assessment on a previously identified abnormality.
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BACKGROUND: The self-inflating bag (SIB) is the most common device used to resuscitate newborn infants worldwide. Delivering positive end-expiratory pressure (PEEP) may be important in infant resuscitation and limited research using one brand (Laerdal) SIB has led to international guidelines stating SIBs 'often deliver inconsistent positive end-expiratory pressure'. AIM: To measure delivered PEEP using disposable and reusable Ambu SIBs fitted with Ambu PEEP valve and manometer comparing different rates of 20, 40 and 60 inflations per minute (IPM) and test lung compliance. DESIGN: Three experienced neonatal medical staff provided positive pressure ventilation each using different disposable and reusable Ambu SIBs, targeting peak inflation pressure of 30-35â cmâ H2O at three different set PEEP levels of 5, 7.5 and 10â cmâ H2O on test lungs of compliance of 0.5 and 3.0â mL/cmâ H2O. Inflation data were captured with Florian Monitor and analysed by analysis of variance for repeated measures. RESULTS: A total of 3265 inflations were analysed. The delivered PEEP was rate and lung compliance dependent. At set PEEP of 5â cmâ H2O, the adjusted measured PEEP was 3.6, 4.4 and 4.8â cmâ H2O at rates 20, 40 and 60 IPM, respectively, while at set PEEP of 10â cmâ H2O, the adjusted measured PEEP was 7.0, 8.8 and 9.8â cmâ H2O. The delivered PEEP was statistically higher with more compliant test lungs. CONCLUSIONS: The Ambu SIB with Ambu PEEP valve can deliver consistent mean levels of PEEP close to the operator set PEEP. The performance of SIB with PEEP valves is likely brand specific and requires further evaluation.
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Respiração com Pressão Positiva/instrumentação , Desenho de Equipamento , Humanos , Recém-Nascido , Insuflação/instrumentação , Complacência Pulmonar , ManometriaRESUMO
An accessory diaphragm, also known as diaphragmatic duplication, is a congenital anomaly in which there is a fibromuscular membrane on top of the normally formed diaphragm dividing the hemithorax into two compartments trapping part of the pulmonary parenchyma. It is a very rare anomaly with less than 40 cases reported in the literature, of which only five were diagnosed in the newborn period, with no reports on any clinical clues for the antenatal diagnosis of this condition. We describe a case of congenital accessory diaphragm presenting in the antenatal period as hydrops fetalis. We also describe the radiological features of this rare anomaly on the antenatal fetal ultrasound.
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Diafragma/anormalidades , Hidropisia Fetal/diagnóstico por imagem , Adulto , Diafragma/diagnóstico por imagem , Evolução Fatal , Feminino , Humanos , Recém-Nascido , Gravidez , Ultrassonografia Pré-NatalRESUMO
A 6-week-old ex-25-week gestation male infant developed candidal sepsis with subsequent bilateral acute obstructive nephropathy that was not responsive to intravenous antifungal therapy. Bilateral percutaneous nephrotomies were performed and irrigation with fluconazole started. Owing to the persistence of the candidaemia and left renal obstruction, streptokinase was administered through the nephrostomy with eventual resolution of obstruction and improvement in his kidney function. This case report discusses the use of the streptokinase therapy as an adjunct therapy in the clearance of fungal bezoars.