A rare cause of air-leak syndrome in a premature newborn: a case report.

BACKGROUND: Pulmonary complications, such as airway leak syndrome, are common in preterm neonates; however, bronchial rupture is a rarely seen phenomenon. CASE PRESENTATION: In this case, we present a preterm newborn who developed pneumomediastinum and pneumothorax. The pneumothorax persisted, despite placement of a thorax tube, requiring a thoracotomy to detect and treat the bronchial rupture. CONCLUSION: Physicians should have a high suspicion of bronchial rupture in patients with persistent air leak syndrome, even after thorax tube placement and continuous negative pressure implementation.

27 years of experience with the Fontan procedure: characteristics and clinical outcomes of children in a tertiary referral hospital.

BACKGROUND: The Fontan operation has improved the survival of children born with congenital heart disease with single ventricle physiology. The most widely adopted variations of the Fontan procedure are the extracardiac conduit, the lateral tunnel ve the intra/extracardiac conduit with fenestration. Despite advances in the treatment and prevention of early and late complications that may develop after Fontan surgery, morbidity still remains an important problem. METHODS: 304 patients who underwent Fontan surgery in our center between 1995 and 2022 were included in our study. The complications that developed in patients who underwent primary Fontan or lateral tunnel surgery and extracardiac conduit Fontan application were compared. RESULTS: Classic Fontan surgery and lateral tunnel surgery were performed in 26 of the patients, and extracardiac Fontan surgery was performed in 278 patients. 218 of 304 cases were patients with single ventricular pathology. 86 cases were patients with two ventricular morphologies but complex cardiac pathology. Fenestration was performed in only 6 patients, other patients did not require fenestration. The mean follow-up period of our patients was 12 years (3 months-27 years). When the complications between Fontan procedures were compared in our study, it was found that the length of hospital stay and mortality were statistically significantly reduced in patients who underwent extracardiac Fontan surgery. There was no significant difference in terms of complications that can be seen after Fontan surgery and the length of stay in the intensive care unit. CONCLUSION: Fontan complex is a palliative surgery for children with complex heart disease. Palliative surgical operations aimed at the preparation of the Fontan circulation lead to the preparation of the pulmonary vascular bed and the preservation of ventricular function. The techniques applied in Fontan surgery affect the early and long-term complications and the survival of the patients. In our study, when we examined the patients who extracardiac conduit Fontan procedure for the non-cardiac route, we found that mortality and morbidity were minimal.

Effects of Targeted Versus Adjustable Protein Fortification of Breast Milk on Early Growth in Very Low-Birth-Weight Preterm Infants: A Randomized Clinical Trial.

BACKGROUND: Breast milk is preferred for the feeding of very low-birth-weight (VLBW) infants, but it does not meet nutrition requirements unless it is fortified. Adequate protein intake to maintain the growth of preterm infants cannot be provided by standard fortification methods because of variation in the protein content of human milk. Individualization is necessary to achieve target protein intakes. The goal of this study was to compare the effects of 2 different methods of individualized protein fortification of breast milk on the early growth of VLBW preterm infants. METHODS: In a prospective observational study, VLBW preterm infants ≤32 weeks of gestational age were randomized into 2 groups according to the method of breast milk fortification. Anthropometric measurements were performed in both groups weekly for 4 weeks to compare their growth. RESULTS: During the trial period, the daily protein intake (targeted vs adjustable fortification groups) was (median [range]) 4.5 (4.4-4.6) vs 4.01 (3.5-4.4) g/kg/d (P = 0.001); the daily weight gains (g/d and g/kg/d; mean ± SD) were 25.7 ± 3.9 vs 22.2 ± 6.4 g/d (P = 0.048) and 23.1 ± 4.3 vs 18.7 ± 4.3 g/kg/d (P = 0.014); and the weekly increase in head circumference was 9.8 ± 1.5 vs 8.4 ± 2.1 mm/wk (P = 0.040). All parameters were significantly higher in the targeted than the adjustable fortification group. CONCLUSIONS: Individualized protein fortification using the targeted method for VLBW preterm infants had more positive effects on short-term growth compared with the adjustable fortification method.

Prenatal thrombosis of renal veins and the inferior vena cava in a newborn with double heterozygosity for the factor V Leiden and prothrombin gene G20210A mutations: a case report.

: Renal vein thrombosis in a neonate is a rare but well recognized condition with low mortality but high morbidity. The cause has not been explained clearly yet but is probably a multifactorial process that includes inherited prothrombotic abnormalities. Antenatal onset of renal vein thrombosis is important due to the increased risk for permanent organ damage. We report a case of prenatal thrombosis of the renal veins and the inferior vena cava in a newborn with double heterozygosity for factor V Leiden and prothrombin gene mutations who had persistently impaired renal function requiring chronic peritoneal dialysis.

A tertiary care center's experience with febrile seizures: evaluation of 632 cases.

BACKGROUND: The aim of this paper was to evaluate demographic and prognostic features of febrile seizures (FSs) in a tertiary center in Turkey. METHODS: A retrospective study of 632 children with FS was conducted from January 1995 to January 2002 in the pediatric neurology and general pediatrics departments of Istanbul University, Istanbul Medical School. Patients data was collected and eligible patients were included in the study. RESULTS: There were 386 male (61.1%) and 246 female (38.9%) patients, with a male-to-female ratio of 1.57. Twenty six (4.1%) patients had prenatal, 104 (16.5%) patients had perinatal-neonatal problems. Age at first seizure was 3-72 months with an average of 20.1 months. While 193 patients (30%) were admitted with two seizures, 246 (39%) were admitted with three or more. Out of 632 patients, 501 (79.2%) had recurrences. In an average of 5.8 years (4-8.8), 30 out of 632 patients (4.7%) were diagnosed with epilepsy. First degree relative with FS, age at first FS less than 18 months, height of peak temperature (<38.5 °C), less than 1 or 3 hours between onset of fever and seizure, complex first seizure, complex FS were all related to febrile seizure recurrence in a statistically significant way. Some risk factors for subsequent epilepsy development included complex FS and less than one hour of fever before FS. No patient with FS had died. CONCLUSIONS: Complex FS and less than 1 hour of fever before FS are common risk factors for both epilepsy and FS recurrence.

Comparison of tuberculin skin test and QuantiFERON®-TB Gold In-Tube for the diagnosis of childhood tuberculosis.

BACKGROUND: Tuberculosis (TB) is an important worldwide ongoing health issue. To be able to control TB, one should not only cure active TB but also identify childhood TB patients who have the possibility to develop active disease in the future. The aim of this study was to compare a century-old tuberculin skin test (TST) and QuantiFERON-TB Gold In-Tube (QFT-GIT) test, developed as an alternative to TST and which has been claimed to be superior to TST in several ways, in the diagnosis of childhood TB. METHODS: Fifty-three children with TB between 5 months and 17.5 years of age and 92 healthy children from the same age group with no risk factors for TB were recruited into the study. All children underwent TST and QFT-GIT test and their demographic, clinic and laboratory data were recorded. Data were analyzed using SPSS 14. RESULTS: A total of 53 patients were diagnosed with TB. Mean patient age was 8.5 ± 4.3 years (range, 5 months-17.5 years). A total of 41.7% of the patients were female. Sixteen of 53 patients had confirmation on culture. QFT-GIT test was positive in 16 and TST was positive in 15 among 16 children with culture-confirmed TB. The sensitivity of TST and QFT-GIT were 93.8% and 100.0%, and the specificity of TST and QFT-GIT were 100.0% and 97.8%, respectively. With regard to the 53 TB children including those without bacteriological confirmation, QFT-GIT was positive in 33 children, and TST was positive in 44 children. The sensitivity of TST and QFT-GIT was then 83.0% and 62.3%, and the specificity, 100.0% and 97.8%, respectively. CONCLUSION: Although positive QFT-GIT test is very significant for TB, negative results will not exclude TB infection. TST and QFT-GIT used together may provide more efficient results.

The exon 3-deleted/full-length growth hormone receptor polymorphism and response to growth hormone therapy in growth hormone deficiency and Turner syndrome: a multicenter study.

BACKGROUND/AIM: The exon 3-deleted/full-length (d3/fl) growth hormone (GH) receptor (GHR) polymorphism has been associated with responsiveness to GH therapy in some diagnostic groups. However, there are still controversies on this issue. To evaluate the effect of the GHR exon 3 polymorphism on growth after 1 and 2 years of GH therapy in Turkish patients with GH deficiency (GHD) and Turner's syndrome (TS) and the distribution of GHR exon 3 isoforms. MATERIALS AND METHODS: 218 patients with GHD (125 males/93 females) and 43 patients with TS were included in the study. The control group included 477 healthy adults aged from 18 to 57 years (54 females/423 males). Anthropometric parameters and insulin-like growth factor (IGF)-1 and IGF binding protein (IGFBP)-3 were evaluated annually. GHR isoforms were studied using simple multiplex PCR. Height and body mass index were expressed as standard deviation score (SDS). RESULTS: There were no differences among TS, GHD and healthy adults regarding the distribution of GHR exon 3 isoforms (fl/fl, fl/d3 and d3/d3). There was a significant increase in height SDS in both diagnostic groups on GH therapy; however, there were neither differences in height SDS and Δheight velocity between fl/fl, fl/d3 and d3/d3 groups nor a correlation between the distribution of GHR exon 3 isoforms and change in IGF-1 SDS and IGFBP-3 SDS levels on GH therapy in either of the diagnostic groups. There was also no gender difference in GHR isoforms in healthy adults. CONCLUSION: The results suggest that responsiveness to GH therapy does not depend on the exon 3 GHR genotypes in GHD and TS patients.