11 research outputs found
An Unusual Case of Neonatal Hypotonia and Femur Fracture: Neuromuscular Variant of Glycogen Storage Disease Type IV
Glycogen storage disease type IV (GSD IV) (OMIM #232500) is an autosomal recessive disorder caused by deficiency of the glycogen-branching enzyme. Here, we report a patient presenting with prematurity and severe hypotonia resulting from a complicated pregnancy with polyhydramnios. During her stay in the neonatal unit, the infant remained dependent on a ventilator, and her movements were mostly absent, except for occasional small movements of her fingers. A spontaneous fracture of femur shaft occurred in the postnatal fourth week. Whole-exome sequencing of DNA from the patient revealed a homozygous missense variant in the GBE1 gene (c.1693C>T, p.Arg565Trp). The variation detected in the index case was also confirmed by Sanger sequencing in the patient and respective parents. This study showed that the neuromuscular subtypes of GSD-IV should be considered as a possible differential diagnosis in severe neonatal hypotonia cases
Comparison of Three Different Multiple Organ Dysfunction Scores for Predicting Mortality after Neonatal Cardiac Surgery
Infants who undergo cardiac surgery frequently have complications that may advance to multiple organ failure and result in mortality. This study aims to compare three different multiple organ dysfunction scoring systems: the Neonatal Multiple Organ Dysfunction (NEOMOD) score, the modified NEOMOD score, and the Pediatric Logistic Organ Dysfunction-2 (PELOD-2) score in predicting postoperative 30-day mortality in neonates undergoing cardiac surgery. This retrospective cohort study was conducted between January 2019 and February 2021 in a single unit on neonates operated on due to congenital heart disease in the first 28 days of life. Patients who underwent off-pump surgeries were excluded from the study. The NEOMOD, modified NEOMOD, and PELOD-2 scores were calculated for each of the first 3 days following surgery. A total of 138 patients were included. All scores had satisfactory goodness-of-fit and at least good discriminative ability on each day. The modified NEOMOD score consistently demonstrated the best prediction among these three scores after the first day, reaching its peak performance on day 2 (area under curve: 0.824, CI: 0.75–0.89). Our findings suggest that NEOMOD and modified NEOMOD scores in the first 72 h could potentially serve as a predictor of mortality in this population
The common triggers of urticaria in children admitted to the pediatric emergency room
Background Urticaria frequently causes pediatric emergency department (PED) admissions. Children with urticaria may unnecessarily avoid suspected allergens. We aimed to investigate the possible and exact triggers of urticaria in children admitted to the PED. Methods Medical records of children admitted to the PED within a 1-year period were evaluated for the International Classification of Diseases 10 (ICD-10) L50 urticaria code, noting symptoms, and possible triggers of urticaria. We performed telephone interviews to complete the missing data and further diagnostic tests for IgE-mediated allergies to identify the exact triggers of urticaria. Results Among 60,142 children, 462 (0.8%) with the L50 code were evaluated. Possible triggers based on the history and physical examination could be identified in 46%: infections (18%), drugs (11%), foods (8%), infections and drugs (3%), insects (3%), pollen (1%), blood products (0.4%), and vaccines (0.4%). The most frequent infections related to urticaria were upper respiratory tract infections (74.5%), urinary tract infections (13.2%), gastroenteritis (8.2%), and otitis media (4.1%). After a diagnostic workup, IgE-mediated allergic diseases were diagnosed in 6% of patients. Twenty-two percent of the patients had multiple PED admission for the same urticaria flare. Urticaria severity was found to be the most important risk factor for readmissions to the PED (odds ratio: 3.86; 95% confidence interval: 2.39-6.23; p .001). No relationship between urticaria severity, duration, and the triggers was present. Conclusions Despite detailed diagnostic tests, IgE-mediated allergic triggers were rarely the cause of urticaria in children admitted to the PED. Infections are the most frequent trigger. Severe urticaria causes more frequent readmissions to the PED
A Multicentered Study on Epidemiologic and Clinical Characteristics of 37 Neonates With Community-acquired COVID-19
Background: Coronavirus disease 2019 (COVID-19) primarily affects adults and spares children, whereas very little is known about neonates. We tried to define the clinical characteristics, risk factors, laboratory, and imagining results of neonates with community-acquired COVID-19. Methods: This prospective multicentered cohort study included 24 neonatal intensive care units around Turkey, wherein outpatient neonates with COVID-19 were registered in an online national database. Full-term and premature neonates diagnosed with COVID-19 were included in the study, whether hospitalized or followed up as ambulatory patients. Neonates without severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) via reverse transcriptase-polymerase chain reaction testing or whose mothers had been diagnosed with COVID-19 during pregnancy were excluded. Results: Thirty-seven symptomatic neonates were included. The most frequent findings were fever, hypoxemia, and cough (49%, 41%, 27%, respectively). Oxygen administration (41%) and noninvasive ventilation (16%) were frequently required; however, mechanical ventilation (3%) was rarely needed. Median hospitalization was 11 days (1-35 days). One patient with Down syndrome and congenital cardiovascular disorders died in the study period. C-reactive protein (CRP) and prothrombin time (PT) levels were found to be higher in patients who needed supplemental oxygen (0.9 [0.1-8.6] vs. 5.8 [0.3-69.2]p= 0.002, 11.9 [10.1-17.2] vs. 15.2 [11.7-18.0]p= 0.01, respectively) or who were severe/critical (1.0 [0.01-8.6] vs. 4.5 [0.1-69.2]p= 0.01, 11.7 [10.1-13.9] vs. 15.0 [11.7-18.0]p= 0.001, respectively). Conclusions: Symptomatic neonates with COVID-19 had high rates of respiratory support requirements. High CRP levels or a greater PT should alert the physician to more severe disease
Prevalence Of Protective Measles Virus Antibody Levels In Umbilical Cord Blood Samples And Sera Of Mothers And Transplacental Transport Ratio In Turkey
In Turkey, the Measles Elimination Program has been implemented since 2002. The aim of this study was to evaluate the measles-specific antibody levels of mothers admitted to a hospital for birth and their infants, to determine the factors influencing the antibody levels of both, and to evaluate the transplacental transport ratio. We selected healthy women who came to the hospital for birth and their healthy newborns. We collected blood samples from 1,547 mothers and 1,529 infants. The protective prevalence of measles antibody levels of mothers was 80% (95% confidence interval [CI]: 78-82%) and that of newborns was 85% (95% CI: 83-86%). The antibody levels of mothers and newborns were positively linearly correlated (R: 0.922, p = 32 years of age and 2.1 times higher (95% CI: 1.4-3.3) in naturally immune mothers. Two factors affecting the antibody levels of newborns were the mothers' antibody levels and their immunization status. The antibody level of mother was the most significant factor that influenced the infant's antibody level. Vaccination of women before pregnancy could enhance passive antibody protection by increasing the level of transplacental transmission.WoSScopu
A multicentered study on epidemiologic and clinical characteristics of 37 neonates with community-acquired COVID-19
Can, Emrah/0000-0002-2163-6564; Reis, AlessanRSS/0000-0001-8486-7469Background: Coronavirus disease 2019 (COVID-19) primarily affects adults and spares children, whereas very little is known about neonates. We tried to define the clinical characteristics, risk factors, laboratory, and imagining results of neonates with community-acquired COVID-19. Methods: This prospective multicentered cohort study included 24 neonatal intensive care units around Turkey, wherein outpatient neonates with COVID-19 were registered in an online national database. Full-term and premature neonates diagnosed with COVID-19 were included in the study, whether hospitalized or followed up as ambulatory patients. Neonates without severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) via reverse transcriptase-polymerase chain reaction testing or whose mothers had been diagnosed with COVID-19 during pregnancy were excluded. Results: Thirty-seven symptomatic neonates were included. the most frequent findings were fever, hypoxemia, and cough (49%, 41%, 27%, respectively). Oxygen administration (41%) and noninvasive ventilation (16%) were frequently required; however, mechanical ventilation (3%) was rarely needed. Median hospitalization was 11 days (1-35 days). One patient with Down syndrome and congenital cardiovascular disorders died in the study period. C-reactive protein (CRP) and prothrombin time (PT) levels were found to be higher in patients who needed supplemental oxygen (0.9 [0.1-8.6] vs. 5.8 [0.3-69.2]p= 0.002, 11.9 [10.1-17.2] vs. 15.2 [11.7-18.0]p= 0.01, respectively) or who were severe/critical (1.0 [0.01-8.6] vs. 4.5 [0.1-69.2]p= 0.01, 11.7 [10.1-13.9] vs. 15.0 [11.7-18.0]p= 0.001, respectively). Conclusions: Symptomatic neonates with COVID-19 had high rates of respiratory support requirements. High CRP levels or a greater PT should alert the physician to more severe disease.Turkish Neonatal SocietyThe Turkish Neonatal Society funded the online registration system of the study. Funders had no role in study design, data collection, data analysis and interpretation, writing of the report, and decision to publish of the article. the corresponding author has full access to all the data in the study and takes final responsibility for the decision to submit for publication. All authors approved the final version of the manuscript. M.K.K. wrote the first draft of the manuscript, no honorarium, grant, or other form of payment was given to anyone to produce the manuscript. To the best of our knowledge, no conflict of interest, financial or other, exists. There are no prior publications or submissions with any overlapping information, including studies and patients
Caffeine Use in Preterm Neonates: National Insights Into Turkish Nicu Practices
Ulu, Ersin/0000-0002-9798-2367Objective: Caffeine is a proven medication used for the prevention and treatment of apnea in premature infants, offering both short- and long-term benefits. International guidelines provide a range of recommendations regarding the preterm population eligible for caffeine prophylaxis, including the timing, dosage, and duration of treatment. Our national guidelines, published prior to the most recent updates of the international guidelines, recommend the use of caffeine citrate starting from the first day after delivery for preterm infants with a gestational age of 28 weeks. For infants up to 32 weeks, if positive pressure ventilation is required, the decision should be made on an individual basis. This study aims to describe the variability in caffeine usage across neonatal intensive care units in our country. Methods: An online survey was sent to neonatologist who are members of the Turkish Neonatology Society to describe the variability in caffeine usage in neonatal intensive care units in our country. Results: We collected responses from 74 units. Prophylactic caffeine usage was observed as; GA = 27(6/7): 98.6%, GA 28(0/7)-28(6/7): 89.0%, GA 29(0/7)-29(6/7): 75.3%, GA 30(0/7)-31(6/7): 53.4%. 62.2% of units reported administering loading dose within the first two hours. The initial maintenance dose was 5 mg/kg in 64.8% of units, 10 mg/kg in 32.4% of units, and intermediate dose in 5.3% of units. 47.3% of units reported no routine dose adjustment. The postmenstrual age that caffeine treatment was stopped was found to be 34 (min-max; 32-36) weeks for infants without apnea and respiratory support, 36 (min-max; 34-52) weeks for infants without apnea but any respiratory support. The time to discharge after treatment cessation was found as; 1-4 days: 37.8%, 5-7 days: 68.9%. Among the 56 units with multiple responsible physicians, 32.1% reported intra-unit variations. Conclusion: The significant differences in caffeine usage characteristics between and within units highlight the need for clear recommendations provided by standardized guidelines
Epidemiologic and clinical characteristics of neonates with late-onset COVID-19: 1-year data of Turkish Neonatal Society
© 2022, The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.The literature on neonates with SARS-CoV-2 is mainly concerned with perinatal cases, and scanty data are available about environmentally infected neonates. To fill knowledge gaps on the course and prognosis of neonatal cases, we analyzed 1-year data from the Turkish Neonatal Society in this prospective cohort study of neonates with postnatal transmission. Data from 44 neonatal intensive care units (NICUs), of neonates with positive RT-PCR results at days 5–28 of life, were extracted from the online registry system and analyzed. Of 176 cases, most were term infants with normal birth weight. Fever was the most common symptom (64.2%), followed by feeding intolerance (25.6%), and cough (21.6%). The median length of hospitalization was 9 days, with approximately one quarter of infants receiving some type of ventilatory support. Myocarditis (5.7%) was the most common complication during follow-up. Among the clinical findings, cough (odds ratio [OR]: 9.52, 95% confidence interval [CI]: 4.17–21.71), tachypnea (OR: 26.5, 95% CI: 9.59–73.19), and chest retractions (OR: 27.5, 95% CI: 5.96–126.96) were associated with more severe clinical disease. Also, there were significant differences in the C-reactive protein level, prothrombin time (PT), partial thromboplastin time, international normalized ratio, and days in the NICU (p = 0.002, p = 0.012, p = 0.034, p = 0.008, and p < 0.001, respectively) between patients with mild-moderate and severe-critical presentations. A PT above 14 s was a significant predictor of severe/critical cases, with a sensitivity of 64% and specificity of 73%. Conclusions: Our data showed that late-onset COVID-19 infection in neonates who need hospitalization can be severe, showing associations with high rates of ventilatory support and myocarditis. Cough, tachypnea, and retractions on admission suggest a severe disease course. Trial registration: ClinicalTrials.gov identifier: NCT04401540.What is Known:• Neonatal cases of COVID-19 infection are mainly reported as perinatal COVID-19 cases.• Neonates with perinatal transmission have a mild course and favorable prognosis.What is New:• Among symptomatic neonates with late-onset COVID-19 infection, fever was the most common symptom, and almost one quarter of hospitalized cases needed some type of respiratory support. Myocarditis was the most common complication.• The presence of cough, tachypnea, retractions, and a PT above 14 s were associated with an increased risk of severe COVID-19
Caffeine use in preterm neonates: national insights into Turkish NICU practices
Objective: Caffeine is a proven medication used for the prevention and treatment of apnea in premature infants, offering both short- and long-term benefits. International guidelines provide a range of recommendations regarding the preterm population eligible for caffeine prophylaxis, including the timing, dosage, and duration of treatment. Our national guidelines, published prior to the most recent updates of the international guidelines, recommend the use of caffeine citrate starting from the first day after delivery for preterm infants with a gestational age of <28 weeks. For infants up to 32 weeks, if positive pressure ventilation is required, the decision should be made on an individual basis. This study aims to describe the variability in caffeine usage across neonatal intensive care units in our country. Methods: An online survey was sent to neonatologist who are members of the Turkish Neonatology Society to describe the variability in caffeine usage in neonatal intensive care units in our country. Results: We collected responses from 74 units. Prophylactic caffeine usage was observed as; GA <= 27(6/7): 98.6%, GA 28(0/7)-28(6/7): 89.0%, GA 29(0/7)-29(6/7): 75.3%, GA 30(0/7)-31(6/7): 53.4%. 62.2% of units reported administering loading dose within the first two hours. The initial maintenance dose was 5 mg/kg in 64.8% of units, 10 mg/kg in 32.4% of units, and intermediate dose in 5.3% of units. 47.3% of units reported no routine dose adjustment. The postmenstrual age that caffeine treatment was stopped was found to be 34 (min-max; 32-36) weeks for infants without apnea and respiratory support, 36 (min-max; 34-52) weeks for infants without apnea but any respiratory support. The time to discharge after treatment cessation was found as; 1-4 days: 37.8%, 5-7 days: 68.9%. Among the 56 units with multiple responsible physicians, 32.1% reported intra-unit variations. Conclusion: The significant differences in caffeine usage characteristics between and within units highlight the need for clear recommendations provided by standardized guidelines
Clinical and Demographic Characteristics of Treatment Requiring Retinopathy of Prematurity in Big Premature Infants in Turkiye: Report No. 1 (BIG-ROP Study)
Introduction: The aim of the study was to analyse the clinical and demographic features of infants with gestational age (GA) of 32-37 weeks and birth weight (BW) of >1,500 g who developed treatment requiring retinopathy of prematurity (ROP). Methods: Data on the infants with a GA of 32-37 weeks and BW >1,500 g who developed treatment requiring ROP (TR-ROP) were collected retrospectively from the 33 ROP centres in Turkiye. GA, BW, type of hospital, neonatal intensive care units (NICUs) level, presence of an ophthalmologist and neonatologist in the same hospital, length of stay in NICU, duration of oxygen therapy, comorbidities, type of ROP, and timing for TR-ROP development were analysed. Results: A total of366 infants were included in the study. Mean GA and BW were 33 1 weeks and 1,896 316 g, respectively. Duration of hospitalization was 3-4 weeks in 46.8% of them. The first ROP examination was performed at postnatal 4-5 weeks in 80.3% of infants, which was significantly later in level 2 and lower NICUs and non-university clinics. At the first ROP examination, any stage of ROP was detected in 90.9% and TR-ROP was detected in 15.3% of the infants. The mean postnatal week of TR-ROP development was 6.16 2.04. Conclusion: Routine ROP screening thresholds need to be expanded in hospitals with suboptimal NICU conditions considering the development of TRROP in more mature and heavier preterm infants. The first ROP examination should be earlier than the fourth postnatal week. 2024 S. Karger AG, Basel
