Methemoglobinemia Associated with Late-Onset Neonatal Sepsis: A Single-Center Experience

Objective â Methemoglobinemia (MetHb) is a rare congenital or acquired cause of infantile cyanosis. We examined the role of MetHb in a neonatal intensive care unit (NICU). Study Design â A retrospective observational study was conducted reviewing blood gas analyses of hospitalized newborns over a 2-year period. MetHb-positive patients (MetHb >1.8%) were matched with a control group for gestational age, weight, disease, and illness severity at admission. Maternal, neonatal, clinical, and laboratory parameters were collected and analyzed in both groups. Results â MetHb incidence was 6%. The mean MetHb in the case group was 7.2%, and the first positive samples were observed at a mean of 22 days of life, 6 days prior to clinical or culture-proven sepsis. We identified low maternal age (31 vs. 34 years; p = 0.038), sepsis (90 vs. 45%; p = 0.022), and protracted parenteral nutrition (46 vs. 23 days; p = 0.013) as risk factors for MetHb, and early minimal enteral feeding as protective factor (12th vs. 9th day; p = 0.038). Conclusion â MetHb has a high occurrence in NICU and can be a helpful prognostic indicator of an infectious process. Understanding and prompt identification of MetHb can allow pediatricians to implement a life-saving therapy

Recognizable neonatal clinical features of aplasia cutis congenita

Background: Aplasia cutis congenita (ACC), classified in nine groups, is likely to be underreported, since milder isolated lesions in wellbeing newborns could often be undetected, and solitary lesions in the context of polymalformative syndromes could not always be reported. Regardless of form and cause, therapeutic options have in common the aim to restore the deficient mechanical and immunological cutaneous protection and to limit the risk of fluid leakage or rupture of the exposed organs. We aimed to review our institutional prevalence, comorbidities, treatment and outcome of newborns with ACC. Methods: We conducted a retrospective study including all newborns affected by ACC and admitted at the University Mother-Child Department from October 2010 to October 2019. Anthropometric and clinical characteristics of ACC1 versus a non-isolated ACC group were analyzed. Results: We encountered 37 newborns, 16 with ACC1 versus 21 with non-isolated ACC. The incidence rate of 0.1% in ACC1 was higher than expected, while 19% of cases showed intrafamilial autosomal dominant transmission. Higher birth weight centile, though lower than reference population, being adequate for gestational age, normal Apgar score and euglycemia characterizing ACC1 resulted associated to a rapid tissue regeneration. Non-isolated ACC, in relation to concomitant congenital anomalies and higher prematurity rate, showed more surgical and medical complications along with the risk of neonatal death. Specifically, newborns with ACC4 were characterized by the frequent necessity of abdominal wall defect repair, responsible for the occurrence of an abdominal compartment syndrome. Conclusion: Prompt carefully assessment of the newborn with ACC in order to exclude concomitant other congenital malformations, provides clues to the underlying pathophysiology, and to the short-term prognosis. Family should be oriented toward identification of other family members affected by similar pathology, while obstetric history should exclude initial multiple pregnancy with death of a co-twin, placental anomalies and drug assumption. Molecular-genetic diagnosis and genetic counseling are integrative in individualized disease approach

Influence of slope of rise and event order of aircraft noise events on high resolution actimetry parameters

Zusammenfassung: Starts und Landungen in der Nacht sind bei den meisten Verkehrsflughäfen durch eine Nachtflugsperre eingeschränkt. Die Empfindlichkeit der Bevölkerung, durch Fluglärm im Schlaf gestört zu werden, ist daher zu Randzeiten, also abends und morgens, besonders gross. In einer experimentellen Feldstudie bei Anwohnern in der Umgebung des Flughafens Zürich untersuchten wir die Wirkung von Fluglärmereignissen auf Körperreaktionen vor dem Aufwachen am Morgen. Dabei wurden in den Schlafzimmern der Versuchspersonen zuvor aufgezeichnete Fluglärm-Geräusche abgespielt. Mit einem nichtinvasiven Aufzeichnungsverfahren für schlafphysiologische Parameter (Hochauflösende Aktimetrie-, Herz- und Atemparameter) wurden Körperbewegungen ereigniskorreliert registriert. Die Resultate ergaben, dass vor allem die ersten paar Lärmereignisse am frühen Morgen die stärksten Reaktionen evozierten und diese mit zunehmender Anzahl erlebter Lärmereignisse abnehmen, was vermuten lässt, dass die zuvor erlebte Anzahl Überflüge eine systematische Wirkung hat, insbesondere wenn die Ereignisse innerhalb kurzer Zeitintervalle auftreten. Es zeigte sich auch, dass die Motilität von der Anstiegsgeschwindigkeit des Schallpegels abhängt, steilere Anstiegsflanken - wie sie typischerweise direkt unterhalb von Landeanflugschneisen auftreten - erzeugen stärkere Bewegungsreaktionen. Dies ist im Einklang mit einer neueren Untersuchung über den Zusammenhang zwischen nächtlichem Flug- und Eisenbahnlärm und Aufwachwahrscheinlichkei

Microcephaly and macrocephaly. A study on anthropometric and clinical data from 308 subjects

Head circumference is the auxological parameter that most correlates with developmental anomalies in childhood. Head circumference (HC) two standard deviations (SD) below or above the mean defines microcephaly and macrocephaly, respectively. The aim of this retrospective study was to explore anthropometric parameters and clinical characteristics among subjects with abnormalities in HC who had been referred for developmental assessment. One hundred and sixty four subjects with microcephaly and 144 subjects with macrocephaly were enrolled from birth to 18 months of age. Head circumference at birth and the association with variables related to maternal health status, gestational age, growth pattern, brain imaging and clinical characteristics were analyzed. In some cases, an etiological diagnosis was made. In the two considered conditions, we found different anthropometric and clinical associations, some of which were statistically significant, with implications for ongoing neurodevelopmental surveillance

Dilated azygos arch mimicking an aortic arch anomaly during thoracic surgery

Cardiovascular malformations are frequently associated in patients with esophageal atresia (EA). We observed azygos continuation mimicking an aortic arch anomaly in four newborns with type III EA. They presented concomitant rib anomalies indicating a common developmental defect. Foreknowledge is important for planning thoracotomy or interventional cardiac catheterization in this population

2q13 microdeletion syndrome: Report on a newborn with additional features expanding the phenotype

We describe an additional newborn with craniofacial dysmorphisms, congenital heart disease, hypotonia, and a 2q13 deletion of 1.7 Mb. The clinical and genomic findings observed are consistent with the diagnosis of 2q13 microdeletion syndrome

Determining physiological reaction probabilities to noise events during sleep

Some of the activations that occur during sleep, e.g. awakening reactions, can be considered adverse effects of noise events (e.g., airplane overflights or train passings) during the night. The occurrence of such reactions is an important indicator of the sleep disturbing potential of the particular noise stimulus and it is often desired to exactly quantify that potential in terms of a probability. Awakenings are considered the strongest form of reaction to noise stimuli during sleep and are one of the most often adopted criteria in night time noise protection concepts. However, the correct determination of noise induced awakening probability has given rise to debate in the scientific community in recent years. Because during every night's sleep, spontaneous awakenings can occur at any time, it remains unknown in principle, whether a particular awakening observed during the presence of a noise stimulus was induced by that stimulus or emerged spontaneously. Nevertheless, correctly determining the awakening probability in question is key when it comes to forecasting noise effects during the night. This article introduces two definitions of reaction probability, discusses their advantages and disadvantages, and develops a model of the influence of the time window duration in which reactions of sleepers are screened on the calculated reaction probabilit

Early intestinal perforation secondary to congenital mesenteric defects

Gastrointestinal perforation (GIP) in preterm neonates may be idiopathic, due to necrotizing enterocolitis (NEC), or mechanical obstruction. The predominant cause of GIP in the neonatal period is NEC. Differential diagnosis with congenital malformations, including mesenteric defects leading to internal hernias, is mandatory if the onset is early. We describe two newborns with trans-mesenteric herniation resulting in GIP, and we discuss the presence of possible additional risk factors such as prematurity and predisposing vascular disruption in connective tissue disorders (Ehlers-Danlos syndrome), twinning, and use of assisted reproductive technologies. These cases prompted us to review our exploratory laparotomies performed for intestinal obstruction, complicated/or not with perforation, to identify the frequency of neonatal trans-mesenteric hernias in a referral hospital. The prevalence of GIP and of internal hernia was 25% and 3.3%, respectively. In conclusion, time-onset and particular conditions associated with GIP should lead to a high index of suspicion for internal hernias in order to achieve appropriate diagnosis and therapy

Necrotizing enterocolitis in the preterm: newborns medical and nutritional Management in a Single-Center Study

Necrotizing enterocolitis (NEC) is a typical disorder of preterm newborns, with a high mortality and morbidity rate. The therapeutic and nutritional management of disease depends on several factors. Its prognosis is linked, in addition to the severity of the disease and the need for surgery, to a correct enteral feeding in these patients. This study aims to identify the clinical characteristics of 18 patients with NEC, evaluating the different therapeutic paths undertaken, the type of formula used and the survival rate of this population. Average time of enteral nutrition before the NEC onset was 11,3 ± 11,6 days, with an average fasting period since the onset of 24 ± 18.9 days. 77.8% of patients received surgery and resumed enteral nutrition 17.7 ± 17.9 days after the intervention. The overall survival rate of our cohort was 55.5%. More prospective studies are needed to evaluate the long-term outcomes of survived children with NEC