Evaluation and management of neonatal onset hyperinsulinemic hypoglycemia: a single neonatal center experience.

OBJECTIVES: To evaluate the clinical characteristics and treatment options of neonates requiring prolonged hospitalization due to persistent hyperinsulinemic hypoglycemia (HH). METHODS: This retrospective cohort study included infants >34 weeks of gestation at birth who were born in our hospital between 2018 and 2021, diagnosed with HH, and required diazoxide within the first 28 days of life. The baseline clinical characteristics, age at the time of diagnosis and treatment options in diazoxide resistance cases were recorded. Genetic mutation analysis, if performed, was also included. RESULTS: A total of 32 infants diagnosed with neonatal HH were followed up. Among the cohort, 25 infants were classified as having transient form of HH and seven infants were classified as having congenital hyperinsulinemic hypoglycemia (CHI). Thirty-one percent of the infants had no risk factors. The median birth weight was significantly higher in the CHI group, whereas no differences were found in other baseline characteristics. Patients diagnosed with CHI required higher glucose infusion rate, higher doses, and longer duration of diazoxide treatment than those in the transient HH group. Eight patients were resistant to diazoxide, and six of them required treatment with octreotide and finally sirolimus. Sirolimus prevented the need of pancreatectomy in five of six patients without causing major side effects. Homozygous mutations in the ABCC8 gene were found in four patients with CHI. CONCLUSIONS: The risk of persistent neonatal hyperinsulinism should be considered in hypoglycemic neonates particularly located in regions with high rates of consanguinity. Our study demonstrated sirolimus as an effective treatment option in avoiding pancreatectomy in severe 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.

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.

A novel marker for predicting type 1 retinopathy of prematurity: C-reactive protein/albumin ratio.

PURPOSE: We aimed to investigate whether the C-reactive protein (CRP) to albumin ratio (CAR) an inflammatory predictor can be used as a marker for the development of ROP. METHODS: Gestational age, birth weight, gender, neonatal, and maternal risk factors were recorded. The patients were divided into two groups: those who did not develop ROP (ROP -) and those who developed ROP (ROP +). The ROP + group was further separated into two groups: those who required treatment (ROP + T) and those who were not treated (ROP + NT). The following parameters were noted in the first postnatal week and at the end of the first postnatal month: CRP, albumin, CAR, white blood cell (WBC), neutrophil, lymphocyte, neutrophil-to-lymphocyte ratio (NLR), distribution red cell width (RDW), platelet (Plt), and RDW/platelet ratio. RESULTS: We evaluated 131 premature infants who met the inclusion criteria. There was no difference between the main groups in hemogram parameters and CAR at the postnatal first week. WBC count (p = 0.011), neutrophil count (p = 0.002), and NLR were high (p = 0.004) in the ROP + group at the end of the postnatal 1st month. The CAR level at the end of the first month was higher in the ROP + group (p = 0.027). CAR was similar between the ROP + T and ROP + NT groups (p = 0.112) in the postnatal first week but higher in the treatment-required group at the end of the first month (p < 0.01). CONCLUSION: High CAR and high NLR at the end of the postnatal first month can be used to predict the development of severe ROP.

The Effect of Continuous PEEP Administration during Surfactant Instillation on Cerebral Hemodynamics in Intubated Preterm Infants: A NIRS Study.

OBJECTIVE: There is an ongoing debate about the best and comfortable way to administer surfactant. We hypothesized that uninterrupted respiratory support and continuous PEEP implementation while instilling surfactant via endotracheal tube (ETT) with side port will result in higher regional cerebral tissue oxygenation (rcSO2) and the alterations in cerebral hemodynamics will be minimal. STUDY DESIGN: Preterm infants who required intubation in the delivery room and/or in the first 24 hours of life with gestational age <32 were enrolled. Patients were intubated either via conventional ETT or ETT with side port (Vygon) with appropriate sizes. Following neonatal intensive care unit admission a near-infrared spectroscopy (NIRS) probe was placed on the forehead and each infant was started to be monitored with NIRS. In conventional ETT group, patients separated from the ventilator while surfactant was instilled. In ETT with side port group, respiratory support was not interrupted during instillation. Heart rate, oxygen saturation, rcSO2, cerebral fractional tissue oxygen extraction (cFTOE), and blood pressures were recorded. RESULTS: A total of 46 infants were analyzed. Surfactant was instilled with conventional ETT in 23 and ETT with side port in 23 infants. Birth weights (1,037 ± 238 vs. 1,152 ± 277 g) and gestational ages (28 ± 2.3 vs. 29 ± 1.6 weeks) did not differ between groups. During instillation of surfactant, rcSO2 levels [61.5 (49-90) vs. 70 (48-85)] and cFTOE levels 0.28 (0.10-0.44) vs. 0.23 (0.03-0.44)] were similar (p = 0.58 and 0.82, respectively). CONCLUSION: Interruption of respiratory support during surfactant instillation did not significantly alter the cerebral tissue oxygenation. These results did not support our hypothesis and should be confirmed with further studies. KEY POINTS: · Monitoring intracerebral oxygenation changes during surfactant administration with NIRS is feasible.. · The surfactant administration method does not significantly alter the cerebral oxygenation.. · Surfactant administration itself rather than the method caused a transient drop in cerebral NIRS readings..

Comparison of Assist/Control Ventilation with and without Volume Guarantee in Term or Near-Term Infants.

OBJECTIVES: This study aimed to compare the effects of volume guarantee (VG) combined with assist/control (AC) ventilation to AC alone on hypocarbia episodes and extubation success in infants born at or near term. METHODS: In this prospective cohort study, infants >34 weeks of gestation at birth, who were born in our hospital supported by synchronized, time-cycled, pressure limited, assist/control ventilation (AC) or assist-controlled VG mechanical ventilation (AC + VG) were included. After admission, infants received either AC or VG + AC using by Leoni Plus ventilator. The ventilation mode was left to the clinician. In the AC group, peak airway pressure was set clinically. In the VG + AC group, desired tidal volume was set at 5 mL/kg, with the ventilator adjusting peak inspiratory pressure to deliver this volume. The study was completed once the patient extubated. RESULTS: There were 35 patients in each group. Incidence of hypocarbia was lower in the VG + AC compared with AC (%17.1 and 22.8%, respectively) but statistically not significant. Out-of-range partial pressure of carbon dioxide (PCO2) levels were lower in the VG + AC group and it reached borderline statistical significance (p = 0.06). The median extubation time was 70 (42-110) hours in the VG + AC group, 89.5 (48.5-115.5) hours in the AC group, and it did not differ between groups (p = 0.47). CONCLUSION: We found combining AC and VG ventilation compared with AC ventilation alone yielded similar hypocarbia episodes and extubation time for infants of >34 gestational weeks with borderline significance lower out-of-range PCO2 incidence. KEY POINTS: · Underlying lung pathology requiring mechanical ventilation support in term infant is heterogeneous.. · VG ventilation compared with conventional modes yielded similar hypocarbia episodes in term infants.. · Combining VG ventilation lead to borderline significance lower out-of-range PCO2 incidence..

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 < 0.001) and were associated with parity (p < 0.001). The ratio of neonatal to maternal antibody levels increased with gestational age. The protective levels were 1.6 times higher (95% CI: 1.1-2.4) in mothers ≥ 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.

Sustained lung inflation at birth via short binasal prong in very low birth weight preterm infants: A retrospective study.

BACKGROUND AND OBJECTIVES: It is believed, that sustained lung inflation (SLI) at birth in preterm infants reduces the need for mechanical ventilation (MV) and improves respiratory outcomes. The aim of this study was to compare need for MV in preterm infants at high risk for respiratory distress syndrome (RDS) after prophylactic SLI via short binasal prongs at birth combined with early nasal continuous positive airway pressure (nCPAP) versus nCPAP alone. METHODS: Medical records of infants born at 260/7 to 296/7 weeks gestation through 2015 and 2017 were retrospectively assessed. Infants who get sustained inflations at 25 cmH2 O pressure for 15 s following delivery via binasal short prongs comprised the study group. Gestational age matched infants who was supported solely with nCPAP (6 cmH2 O PEEP) comprised the control group. Early rescue surfactant (200 mg/kg poractant alfa) was delivered using the less invasive surfactant administration technique in infants with established RDS. RESULTS: A total of 215 infants were analyzed. Fewer infants in the SLI group required MV within the first 72 h of life compared to the control group (25.7% vs 56.9%, P < 0.001). In multiple logistic regression analysis, SLI emerged as an independent factor for reduced MV need. Bronchopulmonary dysplasia (BPD) incidence including mild BPD was significantly lower in the SLI group (31.9% vs 48%, P = 0.01); however, moderate and severe BPD rates did not reach to a statistical significance (11.5% vs 20.6%, P = 0.06). CONCLUSION: Prophylactic SLI maneuver at birth for preterm infants with impending RDS reduces the need for MV with no adverse effects.

Patch testing is an effective method for the diagnosis of carbamazepine-induced drug reaction, eosinophilia and systemic symptoms (DRESS) syndrome in an 8-year-old girl.

Drug reaction, eosinophilia and systemic symptoms (DRESS) is an acute and life-threatening disease, characterised by fever, rash and systemic symptoms, including lymphadenopathy, abnormal liver function, interstitial nephritis, pulmonary and cardiac infiltrates and haematological abnormalities with eosinophilia and atypical lymphocytes. The drugs mostly associated with DRESS are anticonvulsants, allopurinol, minocycline and sulfonamides. This syndrome is rarely seen in childhood even though a large number of children have anticonvulsant treatment. An 8-year-old girl was admitted with fever, lymphadenopathy and skin eruptions on her trunk. Her medical history was notable for epilepsy and carbamazepine treatment had been started 5 weeks previously. Laboratory studies showed a white cell count of 6200/µL (normal, 4100-11 200/µL) with 22% eosinophils and a γ-glutamyl transpeptidase level of 296 U/L (normal, 0-23 U/L). Laboratory tests for infections and collagen diseases were in the normal range. Persistence of fever and maculopapular eruption with generalised desquamation and the appearance of cheilitis and facial angioedema suggested a hypersensitivity reaction to carbamazepine. The carbamazepine was replaced with levetiracetam. All clinical symptoms improved within a week with corticosteroids and antihistamine treatment. Six weeks after complete recovery an epicutaneous patch test with carbamazepine was performed and a carbamazepine-induced positive skin reaction was observed at 48-h. Carbamazepine-induced DRESS syndrome is a rare entity in children. An epicutaneous patch test is a useful tool for identifying the inducing agent for the DRESS syndrome and for identifying a safe anticonvulsant drug.