ResearchPad - pediatrics https://www.researchpad.co Default RSS Feed en-us © 2020 Newgen KnowledgeWorks <![CDATA[Paradoxical Vocal Cord Motion Presaging Bilateral Vocal Cord Paresis in an Infant]]> https://www.researchpad.co/article/elastic_article_14227 Paradoxical vocal cord motion (PVCM) is a condition characterized by inappropriate adduction of the vocal cords during respiration. Usually seen in children and adolescents, PVCM presentation in infants is uncommon. Once thought to be a product of psychiatric disease, there are now several other proposed etiologies including irritant-induced and secondary to neurologic disease. Previous studies showed that the treatment of gastric reflux in this age group leads to a resolution of symptoms. We present a case of PVCM in an infant with hydrocephalus and Chiari II malformation. She received reflux therapy and ventriculoperitoneal (VP) shunting with two revisions. Despite these interventions, she continued with symptoms and eventually progressed to bilateral vocal cord paralysis (VCP). There is a paucity of literature describing PVCM as a precursor to VCP. Clinicians should be aware that in this population, refractory PVCM may serve as a warning sign for further vocal cord function decline.

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<![CDATA[Test of Strength: Figure-of-Eight versus Spiral Wrapping Technique for Fiberglass Casts]]> https://www.researchpad.co/article/elastic_article_14141 Pediatric fractures are a common injury, and treatment often includes cast immobilization. For pediatric patients being treated in a cast, cast damage is among the most common reasons patients return to the emergency room. The figure-of-eight wrapping technique interdigitates layers of fiberglass which may create a stronger cast. The aim of this study was to assess the strength of the figure-of-eight wrapping technique in comparison to the spiral wrapping technique. A total of 10 casts were wrapped with a three-inch fiberglass using the spiral technique and 10 casts were wrapped using the figure-of-eight technique. Each cast was then subjected to a three-point bending test and loaded until failure using an Instron machine. The figure-of-eight technique had an average load to failure of 278.2 + 27.6 N/mm which was similar to the spiral technique’s load to failure of 281.2 + 25.4 N/mm (p=0.795). Prior to normalizing for thickness, the load to failure of the figure-of-eight technique was 949.8 + 109.5 N, which was significantly higher than the spiral technique of 868.2 + 65.1 N (p=0.038). The figure-of-eight casts were slightly thicker than the spiral casts (average 0.3 mm, p=0.004). This suggests that the thickness of the fiberglass cast may improve the strength. The figure-of-eight wrapping technique had similar biomechanical characteristics to spiral wrapping techniques. Providers should wrap in whichever technique they feel most comfortable performing as there is no difference in strength of the cast. If a stronger cast is desired, then thickness of the cast can be increased.

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<![CDATA[Low Body Weight Predicted Bradycardia and Desaturation in Retinopathy of Prematurity Surgeries: A Retrospective Cohort Study]]> https://www.researchpad.co/article/elastic_article_14085 Background: As a leading cause of childhood blindness, the epidemic of retinopathy of prematurity (ROP) in China is characterized by advanced stage of ROP in more mature infants than those in the West. More advanced stage of disease necessitates more complicated surgical procedures and consequently exposure to general anesthesia. These ex-prematurely born infants are at risk of developing desaturation especially after surgery under general anesthesia. Physical status, anesthetic management and surgical profile are three main facets of perioperative setting and need to be investigated to identify useful predictors for perioperative adverse events in this population of fragile infants.

Methods: In this retrospective cohort study, we enrolled all infants undergoing ROP surgeries at Peking University People's Hospital, Beijing, China from November 1, 2016 to October 31, 2017. Physical status, anesthesia and surgical management were analyzed by exploratory factor analysis and component matrix to explore risk factors for adverse events.

Results: During the 12 months, 267 cases were included, among whom 61 infants underwent two surgeries required by their ophthalmological conditions. The median postconceptual age at the time of surgery was 46 (40, 53) weeks, and median body weight was 4.0 (3.0, 6.5) kg. None of the infants was dependent on caffeine, oxygen or ventilator before surgery. Bradycardia (29/267, 10.9%) and postoperative desaturation (34/267, 13.4%) were identified as major cardiac and respiratory adverse events. Preoperative atropine, intubation and bigger body weight would prevent patients suffering from bradycardia. Infants with a body weight less than 3.15 kg had a significantly higher chance of desaturation and neonatal intensive care unit admission after ROP surgeries than those who weighed more than 3.15 kg (27.8 vs. 5.1%, OR 5.46 (95% CI 2.66-11.21), P = 0.000).

Conclusion: This study found that preoperative atropine and intubation would prevent bradycardia and low body weight was a predictor for both bradycardia and postoperative desaturation in preterm infants undergoing ROP surgeries.

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<![CDATA[Management of COVID-19-Positive Pediatric Patients Undergoing Minimally Invasive Surgical Procedures: Systematic Review and Recommendations of the Board of European Society of Pediatric Endoscopic Surgeons]]> https://www.researchpad.co/article/elastic_article_14029 Introduction: Hospital response to the COVID-19 outbreak has involved the cancellation of elective, deferrable surgeries throughout Europe in order to ensure capacity for emergent surgery and a selection of elective but non-deferrable surgeries. The purpose of this document is to propose technical strategies to assist the pediatric surgeons to minimize the potential aerosolization of viral particles in COVID-19 patients undergoing urgent or emergent surgical treatment using laparoscopic approaches, based on the currently available literature. The situation and recommendations are subject to change with emerging information.

Materials and Methods: The Scientific Committee and the Board of the European Society of Pediatric Endoscopic Surgeons gathered together in order to address the issue of minimally invasive surgery during this COVID-19 pandemic. A systematic search through PubMed, Embase, and World Wide Web of the terms “COVID-19,” “Coronavirus,” and “SARS-CoV-2” matched with “pneumoperitoneum,” “laparoscopy,” “thoracoscopy,” “retroperitoneoscopy,” and “surgery” was performed. Non-English language papers were excluded. A PRISMA report was performed. Criticalities were identified and a consensus was achieved over a number of key aspects.

Results: We identified 121 documents. A total of 11 full-text documents were assessed to address all concerns related to the adoption of minimally invasive surgery. All aspect of pediatric minimally invasive surgery, including elective surgery, urgent surgery, laparoscopy, thoracoscopy, retroperitoneoscopy, and pneumoperitoneum creation and maintainance were extensively addressed through systematic review. A consensus regarding urgent laparoscopic procedures, setting and operation techniques was obtained within the Committee and the Board.

Conclusions: The ESPES proposes the following recommendations in case minimally invasive surgery is needed in a COVID-19 positive pediatric patients: (1) consider conservative treatment whenever safely possible, (2) dedicate a theater, columns and reusable laparoscopic instrumentation to COVID-19 pediatric patients, (3) prefer disposable instrumentation and cables, (4) use low CO2 insufflation pressures, (5) use low power electrocautery, (6) prefer closed-systems CO2 insufflation and desufflation systems, and (7) avoid leaks through ports. These recommendations are subject to change with emerging information and might be amended in the near future.

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<![CDATA[Inflammatory Bowel Disease in Children: Experience and Constraints in a Resource-limited Setting]]> https://www.researchpad.co/article/elastic_article_13937 Introduction: Inflammatory bowel disease (IBD), though well described in the Caucasian population, is rarely encountered in the black African children. The aim of this study was to increase the awareness of this emerging condition in African children and highlight the constraints of management in a resource-limited setting like Nigeria.

Methods: This study included an audit of children with IBD who were seen between January 2015 and February 2020 at the Lagos University Teaching Hospital (LUTH). The clinical records of children aged one to 16 years who presented with recurrent abdominal pain, weight loss, and gastrointestinal (GI) bleeding with clinical suspicion of IBD were reviewed. Clinical features, endoscopic findings, histopathologic findings, and treatment were documented.

Results: Eight children with IBD were seen during the study period. The median age was 12.0 years (range: five to 15 years). The most common reported concerns in the children were chronic abdominal pain [seen in four patients (50%)] and bloody diarrhea [seen in three patients (42.30%)]. Weight loss and arthritis were seen in three (37.5%) and one (12.5%) children, respectively. Endoscopy confirmed two cases of Crohn’s disease (CD), three cases of ulcerative colitis (UC), and three cases of indeterminate colitis (IC). The children with CD were treated with steroids and exclusive enteral nutrition, with one patient receiving methotrexate, while the UC and IC patients received 5-aminosalicylate therapy.

Conclusion: Although IBD is uncommon in Nigeria, a high index of suspicion is vital to enable early diagnosis and appropriate treatment. Management in the African setting is severely constrained by limited access to endoscopy facilities and nonavailability of other effective treatment options such as biologic agents.

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<![CDATA[Likely questionnaire-diagnosed food allergy in 78, 890 adults from the northern Netherlands]]> https://www.researchpad.co/article/elastic_article_13854 It is challenging to define likely food allergy (FA) in large populations which limited the number of large studies regarding risk factors for FA.ObjectiveWe studied the prevalence and characteristics of self-reported FA (s-rFA) in the large, population-based Dutch Lifelines cohort and identified associated risk factors.MethodsLikely food allergic cases (LikelyFA) were classified based on questionnaire reported characteristics consistent with FA. Subjects with atypical characteristics were classified as Indeterminate. We investigated 13 potential risk factors for LikelyFA such as birth mode and living on a farm and addressed health-related quality of life (H-RQOL).ResultsOf the 78, 890 subjects, 12.1% had s-rFA of which 4.0% and 8.1% were classified as LikelyFA and Indeterminate, respectively. Younger age, female sex, asthma, eczema and nasal allergy increased the risk of LikelyFA (p-value range <1.00*10−250–1.29*10−7). Living in a small city/large village or suburb during childhood was associated with a higher risk of LikelyFA than living on a farm (p-value = 7.81*10−4 and p = 4.84*10−4, respectively). Subjects classified as Indeterminate more often reported depression and burn-out compared to those without FA (p-value = 1.46*10−4 and p = 8.39*10−13, respectively). No association was found with ethnicity, (duration of) breastfeeding, birth mode and reported eating disorder. Mental and physical component scores measuring H-RQOL were lower in both those classified as LikelyFA and Indeterminate compared to those without FA.ConclusionThe prevalence of s-rFA among adults is considerable and one-third reports characteristics consistent with LikelyFA. Living on a farm decreased the risk of LikelyFA. The association of poorer H-RQOL as well as depression and burn-out with questionable self-perceived FA is striking and a priority for future study. ]]> <![CDATA[Massively Dilated Lower Pole Ectopic Megaureter with Involuted Lower Pole Renal Moiety and Collecting System: An Exception to the Meyer-Weigert Rule]]> https://www.researchpad.co/article/elastic_article_11609 Duplicated collecting systems have a predictable draining pattern that is described by the Meyer-Weigert rule. When there are abnormalities associated with duplicating collecting systems, the upper pole moiety drains inferomedially (most commonly associated with obstruction), and the lower pole moiety drains superolaterally (most commonly associated with vesicoureteral reflux). We present a case of an infant with a duplicated collecting system that violates the Meyer-Weigert rule with lower pole megaureter with massive dilation, ectopic insertion, and associated involuted lower pole renal moiety. To our knowledge, this is the only reported case of a lower pole ectopic ureter with an involuted lower pole renal moiety. 

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<![CDATA[Clinical and Biochemical Assessment of Liver Function Test and Its Correlation with Serum Ferritin Levels in Transfusion-dependent Thalassemia Patients]]> https://www.researchpad.co/article/elastic_article_11605 Aims

The aim of our study was to correlate liver function tests with serum ferritin levels in multi-transfused thalassemia patients.

Methods 

This was a descriptive cross-sectional study conducted in the department of hematology, Khyber Medical University, from January 2018 to December 2018. Thalassemia patients of either sex dependent on transfusion ≥ 1 year and having a confirmatory report of the disease were included in our study. The nonprobability convenience sampling technique was used. The Pearson correlation coefficient was applied to observe the correlation between serum ferritin level and liver function tests. A p-value of ≤0.05 was considered statistically significant. SPSS version 23 (SPSS Inc., Chicago, Illinois) was used for data analysis.

Results

A total of 138 subjects of age range 2-23 years, with a mean age of 12.08 ± 6.02 years, were included in our study. The mean serum ferritin of patients in our study was 3278.64 ng/ml with the lowest of 285.2 ng/mL and the highest of 10940.2 ng/ml. With the increase in serum ferritin levels, a rapid increase in alanine aminotransferase (ALT), aspartate aminotransferase (AST), and alkaline phosphatase (ALP) levels was seen. When serum ferritin levels were correlated with total bilirubin level, the bilirubin level remains static with a further increase in serum ferritin levels.

Conclusion

It was deduced that iron deposition is the ultimate reason for increased liver enzymes. There was a positive correlation between serum ferritin and ALT, AST, and ALP while a weak connection was found between serum ferritin and bilirubin levels.

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<![CDATA[Cutaneous Myiasis: An Unusual Cause of Posterior Auricular and Occipital Lymphadenopathy in a Toddler]]> https://www.researchpad.co/article/elastic_article_11585 Cutaneous myiasis is a condition that involves an infestation of fly larvae into human tissue, most commonly caused by Dermatobia (D.) hominis or the bot fly. While this is a condition most commonly seen in tropical regions of the globe due to increased travel to endemic regions, physicians must increasingly be aware of this as a potential diagnosis. In addition, there is minimal literature on cutaneous myiasis in the pediatric patient and its potential associated symptoms. This case report thus highlights a toddler that presented to our facility with a raised, erythematous scalp lesion and associated preauricular and occipital lymphadenopathy. Of note, the patient had a recent travel history to Belize, an endemic area where she was likely infected. As there are multiple other differentials for not only scalp swellings in the pediatric population, in addition to regional adenopathy, a high index of suspicion was needed to make the diagnosis. Ultrasound was the imaging modality used to visualize the fly larva, and surgical excision was the mechanism of treatment. Thus, this case highlights a unique presentation of cutaneous myiasis in a toddler and aims to add to the growing body of literature on a condition likely to be encountered by physicians at a greater frequency.

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<![CDATA[Fecal Calprotectin as a Useful Non-Invasive Screening Marker for Eosinophilic Gastrointestinal Disorder in Korean Children]]> https://www.researchpad.co/article/elastic_article_10900

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<![CDATA[Prevalence of Vitamin D Deficiency in Children with Type 1 Diabetes Mellitus]]> https://www.researchpad.co/article/elastic_article_10713 Background

In the recent years, controversy has emerged regarding the relationship between vitamin D deficiency and the potential effects it could have on glycemic control in patients with type 1 diabetes mellitus (T1D). This study investigates the prevalence of vitamin D insufficiency/deficiency in pediatric patients with T1D from a single, large volume practice.

Methods

This was a retrospective chart review that collected clinical/demographic data as well as serum 25(OH) D levels from medical records of 395 children between the ages of 3 and 18 years with T1D followed at Nemours Children’s Hospital. This data was compared to the National Health and Nutrition Examination Survey (NHANES) database. A Pearson’s Chi-square test was used between group associations. All statistical tests were two-sided and p < 0.05 was used for statistical significance.

Results

Of the 395 children included in these analyses, 4% were vitamin D deficient and 60% were vitamin D insufficient. There were no significant associations of vitamin D deficiency based on sex and age. Vitamin D deficiency was more common among White children when compared to Hispanic children and African American children (42% vs 29%; p < 0.001). Of those that were vitamin D insufficient (n = 235), most were Hispanic (51%), 36% White and 13% African American. There was a significant association between vitamin D deficiency and body mass index (BMI) (p = 0.035). In the summer, children were less likely to be vitamin D deficient (3% vs 6% in winter) and less likely to be vitamin D insufficient (55% vs 71% in winter) (p = 0.007).

Conclusions

Vitamin D insufficiency is highly prevalent among pediatric type 1 diabetics of Central Florida and statistically significant correlation was found between vitamin D status and ethnicity, BMI as well as seasonal variation.

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<![CDATA[rhIGF-1/BP3 Preserves Lung Growth and Prevents Pulmonary Hypertension in Experimental Bronchopulmonary Dysplasia]]> https://www.researchpad.co/article/elastic_article_10247 Rationale: Antenatal factors, such as chorioamnionitis, preeclampsia, and postnatal injury, are associated with an increased risk for bronchopulmonary dysplasia (BPD) and pulmonary hypertension (PH) after preterm birth. IGF-1 (insulin-like growth factor-1) is markedly decreased in normal preterm infants, but whether IGF-1 treatment can prevent BPD or PH is unknown.

Objectives: To evaluate whether postnatal treatment with rhIGF-1 (recombinant human IGF-1)/BP3 (binding peptide 3) improves lung growth and prevents PH in two antenatal models of BPD induced by intraamniotic exposure to endotoxin (ETX) or sFlt-1 (soluble fms-like tyrosine kinase 1), and in a postnatal model due to prolonged hyperoxia.

Methods: ETX or sFlt-1 were administered into the amniotic sac of pregnant rats at Embryonic Day 20 to simulate antenatal models of chorioamnionitis and preeclampsia, respectively. Pups were delivered by cesarean section at Embryonic Day 22 and treated with rhIGF-1/BP3 (0.02–20 mg/kg/d intraperitoneal) or buffer for 2 weeks. Study endpoints included radial alveolar counts (RACs), vessel density, and right ventricular hypertrophy (RVH). Direct effects of rhIGF-1/BP3 (250 ng/ml) on fetal lung endothelial cell proliferation and tube formation and alveolar type 2 cell proliferation were studied by standard methods in vitro.

Measurements and Main Results: Antenatal ETX and antenatal sFlt-1 reduced RAC and decreased RVH in infant rats. In both models, postnatal rhIGF-1/BP3 treatment restored RAC and RVH to normal values when compared with placebo injections. rhIGF-1/BP3 treatment also preserved lung structure and prevented RVH after postnatal hyperoxia. In vitro studies showed that rhIGF-1/BP3 treatment increased lung endothelial cell and alveolar type 2 cell proliferation.

Conclusions: Postnatal rhIGF-1/BP3 treatment preserved lung structure and prevented RVH in antenatal and postnatal BPD models. rhIGF-1/BP3 treatment may provide a novel strategy for the prevention of BPD in preterm infants.

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<![CDATA[Therapeutic Drug Monitoring Characteristics in a Tertiary University Hospital in 2019]]> https://www.researchpad.co/article/elastic_article_9457 Introduction

Therapeutic drug monitoring (TDM) is defined as measuring drug concentration in a biological sample to optimize pharmacotherapy. This study aims to evaluate TDM requests in a tertiary university hospital retrospectively.

Materials and methods

TDM requests were evaluated retrospectively for lithium, valproic acid, carbamazepine, and digoxin in 2019. The age and gender of the patient, requesting department, and measurement results were evaluated. Lower levels than the reference values were considered as subtherapeutic, while levels higher than the reference were considered as toxic.

Results 

A total of 415 drug level measurement records were found. The pediatric age sample ratio was 13.7%, and the elderly age sample ratio was 11.8%. When all samples were evaluated according to the relevant laboratory cut-off values, 72.8% of samples were within the therapeutic level range, 21.9% of samples were subtherapeutic, and 5.3% were toxic. The pediatric age group had a higher ratio of toxic levels for the four drugs studied (54.5%).

Conclusions

Tests for lithium, valproic acid, carbamazepine, and digoxin would not be considered sufficient for TDM. Multidisciplinary teamwork might be appropriate for further implementation and interpretation of TDM.

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<![CDATA[Life within a limited radius: Investigating activity space in women with a history of child abuse using global positioning system tracking]]> https://www.researchpad.co/article/elastic_article_7709 Early experiences of childhood sexual or physical abuse are often associated with functional impairments, reduced well-being and interpersonal problems in adulthood. Prior studies have addressed whether the traumatic experience itself or adult psychopathology is linked to these limitations. To approach this question, individuals with posttraumatic stress disorder (PTSD) and healthy individuals with and without a history of child abuse were investigated. We used global positioning system (GPS) tracking to study temporal and spatial limitations in the participants’ real-life activity space over the course of one week. The sample consisted of 228 female participants: 150 women with PTSD and emotional instability with a history of child abuse, 35 mentally healthy women with a history of child abuse (healthy trauma controls, HTC) and 43 mentally healthy women without any traumatic experiences in their past (healthy controls, HC). Both traumatized groups—i.e. the PTSD and the HTC group—had smaller movement radii than the HC group on the weekends, but neither spent significantly less time away from home than HC. Some differences between PTSD and HC in movement radius seem to be related to correlates of PTSD psychopathology, like depression and physical health. Yet group differences between HTC and HC in movement radius remained even when contextual and individual health variables were included in the model, indicating specific effects of traumatic experiences on activity space. Experiences of child abuse could limit activity space later in life, regardless of whether PTSD develops.

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<![CDATA[Managing possible serious bacterial infection of young infants where referral is not possible: Lessons from the early implementation experience in Kushtia District learning laboratory, Bangladesh]]> https://www.researchpad.co/article/elastic_article_7649 Serious infections account for 25% of global newborn deaths annually, most in low-resource settings where hospital-based treatment is not accessible or feasible. In Bangladesh, one-third of neonatal deaths are attributable to serious infection; in 2014, the government adopted new policy for outpatient management of danger signs indicating possible serious bacterial infections (PSBI) when referral was not possible. We conducted implementation research to understand what it takes for a district health team to implement quality outpatient PSBI management per national guidelines.MethodsPSBI management was introduced as part of the Comprehensive Newborn Care Package in 2015. The study piloted this package through government health systems with limited partner support to inform scale-up efforts. Data collection included facility register reviews for cases seen at primary level facilities; facility readiness and provider knowledge and skills assessments; household surveys capturing caregiver knowledge of newborn danger signs and care-seeking for newborn illness; and follow-up case tracking, capturing treatment adherence and outcomes. Analysis consisted of descriptive statistics.ResultsOver the 15-month implementation period, 1432 young infants received care, of which 649 (45%) were classified as PSBI. Estimated coverage of care-seeking increased from 22% to 42% during the implementation period. Although facility readiness and providers’ skills increased, providers’ adherence to guidelines was not optimal. Among locally managed PSBI cases, 75% completed the oral antibiotic course and 15% received the fourth day follow-up. Care-seeking remained high among private providers (95%), predominantly village health doctors (over 80%).ConclusionsFacility readiness, including health care provider knowledge and skills were strengthened; future efforts should focus on improving provider adherence to guidelines. Social and behavior change strategies targeting families and communities should explore shifting care-seeking from private, possibly less-qualified providers. Strategies to improve private sector management of PSBI cases and improved linkages between private and public sector providers could be explored. ]]> <![CDATA[Predicting First-Year Growth in Response to Growth Hormone Treatment in Prepubertal Korean Children with Idiopathic Growth Hormone Deficiency: Analysis of Data from the LG Growth Study Database]]> https://www.researchpad.co/article/elastic_article_8430

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<![CDATA[Risk Factors for Necrotizing Enterocolitis in Infants With Patent Arterial Duct. A Retrospective Matched Paired Analysis]]> https://www.researchpad.co/article/elastic_article_7858 Background: The development of necrotizing enterocolitis (NEC) in neonates with patent ductus arteriosus (PDA) is not well-understood. Our aim was to find risk factors for NEC in children with a significant PDA and to assess differences in mortality and duration of hospital stay between patients with PDA and those with PDA and NEC.

Methods: We performed a retrospective single center case control study including infants with PDA scheduled for treatment. We compared multiple patient data between patients with PDA and those with PDA and NEC from 2004 to 2018 using 1:2 and 1:1 matching.

Results: We used 1:2 matching with 26 NEC patients (cases) and 52 PDA patients without NEC (controls) and 1:1 matching with 5 NEC patients and 5 PDA patients without NEC. NEC patients had lower Apgar score (1′), more congenital malformations, more suspected sepsis, less hypotension, higher minimum platelet count and higher CRP-values during the week before NEC (P < 0.05, respectively). The mortality was higher in NEC cases [29% (9/31)] compared to the control patients [2% (1/57), P < 0.001]. Lower Apgar score (1′) was correlated with an increased risk of NEC stage III. Hypotension was inversely correlated with the odds of NEC (OR 0.3).

Conclusions: NEC increased mortality in infants with PDA. Hypotension did not increase the risk of NEC in infants with PDA. Routine clinical parameters were not able to predict NEC in infants who suffer from PDA.

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<![CDATA[The Impact of the Current SARS-CoV-2 Pandemic on Neonatal Care]]> https://www.researchpad.co/article/elastic_article_7602 <![CDATA[Silent Sinus Syndrome and Williams Syndrome: Two Rare Diseases Found in a Pediatric Patient]]> https://www.researchpad.co/article/elastic_article_7502 Silent sinus syndrome (SSS) is a rare disease process characterized by progressive enophthalmos and hypoglobus due to ipsilateral maxillary sinus hypoplasia and orbital floor resorption. Patients may also present with eye asymmetry, unilateral ptosis, or diplopia. Most reported cases in the literature describe its occurrence in adults, but it can also affect children. The etiology remains speculative, even though the most accepted theory is that during the first or second decade of life, occlusion of the maxillary ostium causes an interruption in normal sinus development. Williams syndrome (WS) is a rare genetic, multisystem disorder characterized by a constellation of distinctive phenotypic features, including psychomotor delay and cardiovascular abnormalities. We report a case of a 7-year-old female diagnosed at 1 year old with WS and who gradually developed SSS. This last condition was diagnosed at 7 years of age, when she started showing progressive facial asymmetry in addition to typical facial features of WS; subsequent neuroimaging definitively supported the diagnosis. This case report describes for the first time in the literature an uncommon situation in which SSS and WS, both rare syndromes, are present in the same pediatric patient. We speculate that the particular facial features typical of WS could either be the basis of the development of SSS in our patient or make the SSS clinical course more severe, with signs presenting at the age of 7 years. This case report shows for the first time that facial asymmetry in WS can be caused by SSS and highlights the need for early identification of this complication in patients with syndromes characterized by dysmorphic facial features. Further studies are needed to understand whether there is a link between the two syndromes as well as to evaluate the prevalence of SSS in patients with facial dysmorphisms and define the best management.

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<![CDATA[Tobacco Smoking During Pregnancy Is Associated With Increased Risk of Moderate/Severe Bronchopulmonary Dysplasia: A Systematic Review and Meta-Analysis]]> https://www.researchpad.co/article/elastic_article_7432 Epidemiological evidence and animal studies support that intrauterine exposure to tobacco smoke disturbs lung development and has a negative effect in the pulmonary health of the offspring. Individual studies suggest an association between fetal exposure to maternal smoking and risk of developing bronchopulmonary dysplasia (BPD). However, this association has not yet been systematically investigated. We aimed to conduct a systematic review of studies reporting on tobacco smoking during pregnancy as potential risk factor for BPD. PubMed/MEDLINE and EMBASE databases were searched. BPD was defined as requirement of supplemental oxygen on postnatal day 28 (BPD28; all BPD), at the postmenstrual age (PMA) of 36 weeks (BPD36; moderate/severe BPD), or as requirement of more than 30% oxygen and/or positive pressure at 36 weeks PMA (severe BPD). Pooled risk ratios (RR) and 95% confidence intervals (CI) were calculated using a random-effects model. Of 2,894 potentially relevant studies, 33 met the inclusion criteria. The included studies evaluated 171,772 infants and included 30,445 cases of exposure to maternal smoking and 25,340 cases of BPD of any severity. Meta-analysis showed a significant association between tobacco smoking during pregnancy and BPD36 (17 studies, RR 1.126, 95% CI 1.008–1.259, p = 0.036), but could not demonstrate a significant association between tobacco smoking during pregnancy and BPD28 (16 studies, RR 1.021, 95% CI 0.924–1.129, p = 0.681), or severe BPD (3 studies, RR 1.143, 95% CI 0.528–2.478, p = 0.734). In conclusion, our data suggest that tobacco smoking during pregnancy increases the risk of moderate/severe BPD. Our results highlight the detrimental effects of tobacco smoking and reinforce the hypothesis of the involvement of prenatal insults in the etiopathogenesis of BPD.

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