Neonatal Outcomes of Critical Congenital Heart Defects: A Multicenter Epidemiological Study of Turkish Neonatal Society : Neonatal Outcomes of CCHD.

Critical congenital heart disease (CCHD) is one of the leading causes of neonatal and infant mortality. We aimed to elucidate the epidemiology, spectrum, and outcome of neonatal CCHD in Türkiye. This was a multicenter epidemiological study of neonates with CCHD conducted from October 2021 to November 2022 at national tertiary health centers. Data from 488 neonatal CCHD patients from nine centers were entered into the Trials-Network online registry system during the study period. Transposition of great arteria was the most common neonatal CHD, accounting for 19.5% of all cases. Sixty-three (12.9%) patients had extra-cardiac congenital anomalies. A total of 325 patients underwent cardiac surgery. Aortic arch repair (29.5%), arterial switch (25.5%), and modified Blalock-Taussig shunt (13.2%). Overall, in-hospital mortality was 20.1% with postoperative mortality of 19.6%. Multivariate analysis showed that the need of prostaglandin E1 before intervention, higher VIS (> 17.5), the presence of major postoperative complications, and the need for early postoperative extracorporeal membrane oxygenation were the main risk factors for mortality. The mortality rate of CCHD in our country remains high, although it varies by health center. Further research needs to be conducted to determine long-term outcomes for this vulnerable population.

The Effects of Glutathione on Clinically Essential Fertility Parameters in a Bleomycin Etoposide Cisplatin Chemotherapy Model.

Chemotherapeutic agents used in the treatment of testicular cancer cause damage to healthy tissues, including the testis. We investigated the effects of glutathione on sperm DNA integrity and testicular histomorphology in bleomycin etoposide cisplatin (BEP) treated rats. Twelve-week-old male rats of reproductive age (n = 24) were randomly divided into three groups, the (i) control group, (ii) BEP group, and (iii) BEP+ glutathione group. Weight gain increase and testes indices of the control group were found to be higher than that of the BEP group and BEP+ glutathione group. While the BEP treatment increased sperm DNA fragmentation and morphological abnormalities when compared to the control group, GSH treatment resulted in a marked decrease for both parameters. Moreover, BEP treatment significantly decreased serum testosterone levels and sperm counts in comparison to the control group, yet this reduction was recovered in the BEP+ glutathione treated group. Similarly, seminiferous tubule epithelial thicknesses and Johnsen scores in testicles were higher in the control and BEP+ glutathione groups than in the BEP-treated group. In conclusion, exogenous glutathione might prevent the deterioration of male reproductive functions by alleviating the detrimental effects of BEP treatment on sperm quality and testicular histomorphology.

Giant pulmonary pseudoaneurysm following balloon dilatation of the pulmonary artery to relieve pulmonary band.

Pulmonary artery pseudoaneurysms are uncommon. They may occur secondary to trauma, infectious diseases, vasculitis syndromes, neoplasms, congenital diseases, and pulmonary hypertension. Due to increasing number of cardiac interventions, iatrogenic complications are among the major causes of pulmonary artery pseudoaneurysms.In this report, we present a 6-month-old patient with pulmonary pseudoaneurysm that occurred following pulmonary balloon angioplasty for the relief of a pulmonary band.

Congenital tricuspid insufficiency due to rupture of chordae tendinea secondary to intrauterine obliteration of ductus arteriosus.

Congenital rupture of tricuspid chordae tendinea leading to severe tricuspid insufficiency is an extremely rare pathology associated with signs and symptoms of congestive heart failure presenting at birth. If the diagnosis and treatment of this pathology are not made early in life fetal demise may become inevitable.We herein present a neonate with central cyanosis and congestive heart failure due to rupture of an anterior leaflet chordae resulting in severe insufficiency of the tricuspid valve who was treated with appropriate surgery.

High Flow Nasal Cannula versus Nasal Continuous Positive Airway Pressure for Primary Respiratory Support in Preterm Infants: A Prospective Randomized Study.

OBJECTIVE: This study compares high flow nasal cannula (HFNC) with nasal continuous positive airway pressure (nCPAP) within the first hour of life as the primary respiratory support in neonates of ≤32 weeks of gestational age. STUDY DESIGN: This prospective, randomized study was conducted in infants with a gestational age of ≤32 weeks who had spontaneous respiration. HFNC or nCPAP was used as a first line respiratory support after admission to intensive care unit. Primary outcome was primary treatment failure. Secondary outcomes were duration of noninvasive respiratory support and oxygen treatment, maximum FiO2 level, length of hospital stay, intubation rate, rates of respiratory distress syndrome, pneumothorax, and bronchopulmonary dysplasia. Subgroup analysis was performed for infants ≤28 weeks of gestational age. RESULTS: We enrolled 107 infants, 53 in HFNC and 54 to nCPAP group. There was no difference in primary outcome between the two groups. There was no difference between the groups in aspect of secondary outcomes. CONCLUSION: HFNC and nCPAP have no significant differences as a primary mode of respiratory support in preterm infants, in the time to wean off the devices and oxygen support, respiratory distress syndrome and bronchopulmonary dysplasia incidence, hospitalization duration, and rates of complications of prematurity.

Urgent surgical management of congenital intracranial hemangiopericytoma in a preterm neonate.

Hemangiopericytoma is a rare mesenchymal tumor originating from capillary pericytes, known as Zimmermann pericytes. The adult form is not uncommon and generally malignant but tumor is found rarely in children. Here we describe an intracranial hemangiopericytoma in a preterm newborn whose had the tumor resected successfully shortly after birth.

Tratamiento anticoagulante y vasodilatador para el síndrome de Nicolau tras la administración de una inyección de bencilpenicilina benzatínica intramuscular a un niño de cuatro años de edad / Anticoagulant and vasodilator therapy for Nicolau syndrome following intramuscular benzathine penicillin injection in a 4 year old boy

El síndrome de Nicolau (SN) es una complicación rara de la inyección de ciertos fármacos por vía intramuscular, intrarticular o subcutánea, que produce necrosis isquémica de la piel, las partes blandas y el tejido muscular circundante. La bencilpenicilina benzatínica es uno de los antibióticos más ampliamente empleados para las infecciones de las vías respiratorias altas y raramente se ha notificado que produzca SN. En este artículo presentamos el caso de un niño de sexo masculino de cuatro años de edad diagnosticado con SN tras la inyección de bencilpenicilina benzatínica tratado satisfactoriamente con heparina fraccionada (enoxaparina) y pentoxifilina. Los médicos deben estar atentos al uso innecesario de bencilpenicilina benzatínica para evitar las probables complicaciones.

Nicolau syndrome (NS) is a rare complication of intramuscular, intraarticular or subcutaneous injection of particular drugs leading to ischemic necrosis of the surrounding skin, soft tissue and muscular tissue. Benzathine penicilin one of the most widely used antibiotic for upper respiratory tract infections and has been rarely reported to cause NS. Here we describe a 4 year old boy with diagnosis of NS after the injection of benzathine penicillin who was successfuly treated with unfractionized heparin (enoxaparine) and pentoxifylline. The practitioners should pay attention for unnecessary use of benzathine penicillin to avoid from probable complications.

[The role of serum interleukin-6 and C-reactive protein levels for differentiating aetiology of neonatal sepsis]. / Función de la concentración sérica de interleucina 6 y proteína C-reactiva para diferenciar la etiología de la septicemia neonatal.

INTRODUCTION: In our clinical practice, we observed high interleukin-6 (IL-6) levels in gram-negative sepsis. OBJECTIVE: To investigate the relationship between IL-6 and C-reactive protein (CRP) levels and early determination of neonatal sepsis of gram-negative or gram-positive aetiology. POPULATION AND METHODS: White blood cell count, IL-6 and CRP levels were compared among different groups. RESULTS: Gram-negative, gram-positive and fungal infection groups consisted of 73, 82 and 15 patients, respectively. The optimal cut-off levels of IL-6 between gram-negative and gram-positive fungal infection groups were 202 and 57 pg/ml. The fungal infection group had higher CRP levels than gram-negative and positive infection groups. CONCLUSIONS: To our knowledge, this is the largest reported study aiming at determining of IL-6 cut-off levels to differentiate neonatal sepsis aetiology. Gram-negative microorganisms led to 10 fold higher IL-6 production. The evaluation of IL-6 and CRP is useful to diagnose and also differentiate neonatal sepsis aetiology.

Introducción. En la práctica clínica, hemos observado una concentración elevada de interleucina 6 (IL-6) en los casos de septicemia por gramnegativos. Objetivo. Investigar la relación entre las concentraciones de IL-6 y proteína C-reactiva (PCR) y la determinación temprana de la septicemia neonatal por gramnegativos o grampositivos. Población y métodos. Se compararon el recuento de leucocitos y las concentraciones de IL-6 y PCR entre los distintos grupos. Resultados. Los grupos de infección por gramnegativos, infección por grampositivos y micosis estaban formados por 73, 82 y 15 pacientes, respectivamente. Los valores de corte ideales de IL-6 entre el grupo de infección por gramnegativos y el de infección por grampositivos y el de micosis eran 202 pg/ml y 57 pg/ml, respectivamente. En el grupo de micosis se observaron concentraciones de PCR más altas que en los grupos de infección por gramnegativos e infección por grampositivos. Conclusiones. Hasta donde sabemos, este es el estudio de mayor envergadura notificado con el objetivo de determinar los valores de corte de IL-6 para diferenciar la etiología de la septicemia neonatal. Los microorganismos gramnegativos provocaron una producción de IL-6 diez veces mayor. La evaluación de IL-6 y PCR resulta útil para diagnosticar, y asimismo diferenciar, la etiología de la septicemia neonatal

Zekai Tahir Burak Women's Health Education and Research Hospital newborn hearing screening results and assessment of the patients.

AIM: Social, emotional, cognitive and language development of infants is provided with early diagnosis of hearing deficit. Hearing deficit is reported with a rate of 1-6 in 1000 live births in healthy newborns, while it reaches up to 10-30 in 1000 live births in newborns with risk factors. We aimed to compile the results of the hearing screening program applied in our hospital. MATERIAL AND METHODS: The records of the hearing screening program were examined and the results were compiled by reaching the records of the patients who were found to have hearing deficit. RESULTS: Hearing test was applied in a total of 142 128 patients between 2005 and 2011. Hearing test was performed by evoked autoaucistic emission for two times in 26 690 of these patients and for three times in 2 412. A diagnosis of hearing deficit was made in 385 patients (0.27%) after application of ARB (Auditory Brainstem Response). The medical records of 171 of the patients who were referred for advanced investigations and treatment were obtained. 116 of these patients had a history of hospitalization in neonatal intensive care unit, while 55 patients had no history of hospitalization in neonatal intensive care unit. 49 of the patients had a gestational age below the 32(th) week and 122 had a gestational age above the 32(th) week. The median gestational age and birth weight values and ranges were found to be 35 (22-43) and 2 140 g (580-4 590 g), respectively. The risk factors included intrauterine growth retardation (n=24), multiple pregnancy (n=22), hyperbilirubinemia (n=74), blood exchange because of hyperbilirubinemia (n=7), sepsis (n=52), hypoglycemia (n=2), use of aminoglycoside and glycopeptide (n=99), use of furosemide (n=27), mechanical ventilation therapy (n=37), polycythemia (n=12), prenatal asphyxia (n=2), respiratory distress syndrome (n=45), chronic lung disease (n=11), surgery for retinopathy of prematurity (n=8) and hearing deficit in the mother or father (n=7). CONCLUSIONS: In addition to the necessity of performing hearing screening in all newborns, infants with risk factors should be determined, hearing deficit should be screened with repeated hearing tests and social, emotional, cognitive and language development of the infant should be assured.

Congenital laryngeal cyst: a rare cause of polyhydramnios.

Congenital laryngeal cyst is a rare cause of airway obstruction that may require urgent diagnosis and treatment. We report a case of a neonate having history of polyhydramnios and severe respiratory distress at birth. A laryngeal cyst detected during intubation. The outcome of laryngoscopic treatment of the cyst was favorable.

Inflammatory responses to hepatitis B virus vaccine in healthy term infants.

UNLABELLED: Hepatitis B virus (HBV) infection continues to be a serious global health problem. During the course of HBV vaccination, we observed C-reactive protein (CRP) elevation in term infants without sepsis. Therefore, we prospectively studied interleukin-6 (IL-6) and CRP responses to HBV immunization. In 70 healthy term infants without signs and symptoms of sepsis and sepsis risk factors, IL-6, CRP, and white blood cell count levels were determined before and 24 h after immunization. Significant increases in CRP levels were seen 24 h after vaccination (p < 0.001). Although CRP levels of 22 infants at second evaluation were above the cutoff level for sepsis (4.82 mg/L), they had no clinical signs and symptoms of sepsis. After 48-72 h, CRP levels of these infants returned to normal levels with no blood culture positivity. CONCLUSION: our study showed that HBV vaccine is responsible for CRP elevation in term infants after vaccination at birth. To the best of our knowledge, this is the first study evaluating CRP response to HBV vaccine at birth in term infants. We suggest that this response should be considered in differentiation of early neonatal sepsis to avoid unnecessary antibiotic use.

Reference values of serum cystatin C in very low-birthweight premature infants.

AIM: To determine reference values for cystatin C (CysC) and its correlation with creatinine (Cr), gestational age, birthweight and maternal Cr status in very low-birthweight (VLBW) preterm infants. METHOD: The study included 113 VLBW premature infants (<1500 g) of ≤ 32 gestational week. Serum Cr and CysC of the infant and serum Cr of the mother were analysed. RESULTS: The mean level of CysC was 1.77 ± 0.38 mg/L on day 1 and 1.61 ± 0.37 mg/L on day 3, and the decrease was statistically significant. There was a significant correlation only between maternal Cr and first-day Cr values and negative correlations between Cr and gestational age and birthweight on third day. Creatinine was not correlated with CysC both on day 1 (r = -0.077, p = 0.417) and day 3 (r = 0.132, p = 0.164). CONCLUSION: Here, we report the reference ranges for CysC and Cr on first and third day in VLBW infants. CysC concentrations significantly decrease by day 3 compared with day 1 and are independent of gestational week, birthweight and maternal renal function status in VLBW preterm infants.

Effects of different pulmonary surfactants in the prevention of postoperative intraabdominal adhesion formation.

BACKGROUND: After abdominal surgery, the formation of postoperative adhesion is a serious problem. The aim of the study is to evaluate the efficacy of 2 different pulmonary surfactants, poractant and beractant, on adhesion prevention in an experimental model. MATERIALS AND METHODS: An experimental intraabdominal adhesion model was created in 18 adult female rats by cecal abrasion. The rats were randomly assigned to 3 groups. Group I received no further treatment, whereas groups II and III received intraperitoneal poractant and beractant, respectively, before closing the incision. On the 15th postoperative day, all rats underwent relaparotomy, intraabdominal adhesions were scored macroscopically according to Canbaz scoring system, and the cecum in each animal was evaluated microscopically. RESULTS: The median adhesion scores of group II and III rats were significantly lower when compared with group I (P = .02). Group III had a lower median adhesion score than did group II, but this did not reach significance (P > .05). CONCLUSION: These observations suggest that intraperitoneal instillation of both pulmonary surfactants is associated with lower adhesion scores, higher adhesion-free cases, and improved histologic findings.

Four neonates with giant ovarian cysts: difficulties in diagnosis and decision making process.

OBJECTIVE: Ovarian cysts compose most of the intraabdominal cysts in fetal period. Most of them regress spontaneously at intrauterine or postnatal period. The cysts that are complicated and do not regress are excised generally. METHODS: Here we report a case series that consists of four newborns having giant (≥ 10 cm) ovarian cysts. All of the patients were term infants and followed for ovarian cysts in intrauterine period. RESULTS: Patients were operated on 5, 11, 28, 47th days, respectively. Three patients had unilateral cysts (two right, one left) and one had bilateral cysts. One of the four patients had bilateral ovarian cysts, 100 × 95 mm in diameter on the left and 50 × 55 mm on the right, which was torsioned. CONCLUSION: The risk of complications is higher in bilateral cysts even they are smaller, and early surgical intervention should be done to these patients. During the operation of one of the patients, we detected the cyst at the opposite side that was shown by ultrasonography. Since the size of the mass is large, detecting the correct origin of the cyst is important for the selection of appropriate surgical approach.

Paraurethral cyst in two female newborns: which therapy option?

Paraurethral cysts are rare in females, especially in the neonatal period. The aetiology of congenital paraurethral cysts is obstruction of Skene's ducts from para/periurethral glands. Paraurethral cysts may be totally asymptomatic, non-tender, soft, cystic, ovoid masses about 6-10 mm in size and yellowish in colour. These lesions may be discovered on either side of the urethral meatus during routine examination and most of these resolve spontaneously. This study reports two female newborns with paraurethral cysts treated with needle aspiration because of difficulty in excretion of urine on the first day of life. The second case needed another course of needle aspiration 1 month later. There is no consensus on the treatment because of the benign nature of the paraurethral cysts and the probability of spontaneous regression. Surgical alternatives include excision, marsupialization and needle aspiration. If a surgical approach is needed, e.g. because of difficulty in urination, needle aspiration should be used as the initial surgical intervention.

The effects of ibuprofen on sepsis parameters in preterm neonates.

AIM: To examine the effects of ibuprofen used for patent ductus arteriosus (PDA) treatment on the production of the proinflammatory cytokines C-reactive protein (CRP) and interleukin 6 (IL-6) in preterm septic newborns. METHODS: Patients with acute phase reactant elevation were divided into two groups according to receiving ibuprofen (Group I, n=51) or not (Group II, n=38). Course of sepsis was evaluated by CRP and IL-6 levels. RESULTS: CRP and IL-6 levels at the time of diagnosis were not different between two groups [16±9.1 vs 16.4±13.2mg/dL (p=0.43) for CRP and 124±82 vs 119±73mg/dL (p=0.517) for IL-6, respectively]. Similarly, they were statistically insignificant between the groups at the 2nd or 3rd days of ibuprofen treatment [14.3±7.7 vs 13.7±5.9mg/dL (p=0.21) for CRP and 83±46 vs 86±37mg/dL (p=0.29) for IL-6, respectively]. However, CRP and IL6 levels showed significant difference between groups in the following days; 6.03±3.8 vs 9.1±4.9mg/dL (p=0.025) for CRP and 42±33.1 vs 58.9±27.1mg/dL (0.011) for IL-6 on 4th or 5th days of treatment and 2.3±3.2 vs 4.1±2.3mg/dL (p=0.032) for CRP and 16.1±12.4 vs 21.3±16.8mg/dL (p=0.016) for IL-6, on 7th to 10th days of treatment, respectively. CONCLUSIONS: IL-6 and CRP may decrease in infants receiving ibuprofen treatment more than infants who do not receive it. This decrease should be considered at the time of caring a preterm infant with both sepsis and PDA after ibuprofen treatment.

Dramatical recovery of a mechanical ventilatory dependent extremely low birth weight premature infant after Nissen fundoplication.

Gastroesophageal reflux disease (GERD) is a well-recognized condition in neonatal period. It is common in preterm infants, clinical spectrum is wide and the management may be difficult. Nissen fundoplication operation is well established as effective treatment for GERD in most children intractable to medical therapy, but its role in neonates is less clear and its effect in premature infants is not well established. Here, we report a case of a premature infant, who had severe respiratory system problems that needed mechanical ventilation despite all medical therapy, and demonstrate dramatical clinical and radiological improvement as a result of Nissen fundoplication surgery.

Rare clinical entity Perlman syndrome: is cholestasis a new finding?

Perlman syndrome is a rare syndrome characterized by polyhydramnios, fetal overgrowth, facial dysmorphism, visceromegaly, nephroblastomatosis and predisposition to Wilms tumor. Here we report on a newborn with a prenatal history of polyhydramnios who presented with nephromegaly, hypotonia, macrosomia, facial dysmorphism, cholestasis and characteristic ultrasonographic and computed tomographic appearances of renal abnormalities that are observed with Perlman syndrome. Perlman syndrome is a rare entity with a high neonatal mortality rate. This is the first case in which cholestasis has been observed. Close follow-up should be carried out for early detection of Wilms tumor.