UNASSIGNED: The purpose of this study was to present our experience in patients who had been treated with posterior vertebral column resection (PVCR) for various spinal deformities.
UNASSIGNED: Thirty-seven patients who performed PVCR between 2015 and 2018 were evaluated retrospectively. The mean follow-up period was 24 months (range: 12-50 months). The demographic data of the patients, mean blood loss, amount of blood replacement, duration of operation, intensive care and hospitalization period, PVCR level, instrumentation level, amount of preoperative curvature, amount of postoperative curvature improvement, preoperative and postoperative neurological status, and complications were examined. Angular measurements were performed on X-ray.
UNASSIGNED: The mean age of the patients was 37.5 years (range: 3-80 years). PVCR was applied to patients due to different pathologies (congenital, tumor metastasis, posttraumatic kyphosis, revision scoliosis, and infection). The mean operation time was 445.5 min (260-720) with an average blood loss of 1903 ml (400-7000 ml). It was observed that the average local kyphosis angle decreased from 67.65° to 7.42° in 26 patients who were operated for advanced deformity (P < 0.001). When these values were compared in all 34 patients, the preoperative angle value decreased from 55.1° to 3.5° (P < 0.001) and decreased from 70° to 0° in 13 congenital kyphosis patients.
UNASSIGNED: PVCR is an effective method for correcting severe spinal deformities and can be used to correct curvature in different patient groups.
UNASSIGNED: Level 3.

In previous reports, hypothyroidism, hypopituitrism, and hypogonadism were common endocrine causes of SCFE, but this is the first time that congenital adrenal hyperplasia has been observed. As such, patients who have undergone long-term endocrine treatment for congenital adrenal hyperplasia could potentially be subjected to a higher risk for SCFE.

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Maternal parasitemia and placental parasite load were examined in mother-newborn pairs to determine their effect on the congenital transmission of Trypanosoma cruzi. Parasitemia was qualitatively assessed in mothers and newborns by the microhematocrit test; parasite load was determined in the placental tissues of transmitting and non-transmitting mothers by the detection of T. cruzi DNA and by histology. Compared to transmitter mothers, the frequency and prevalence of parasitemia were found to be increased in non-transmitter mothers; however, the frequency and prevalence of parasite load were higher among the transmitter mothers than among their non-transmitter counterparts. Additionally, serum levels of interferon (IFN)-γ were measured by an enzyme-linked immunosorbent assay (ELISA) in peripheral, placental, and cord blood samples. Median values of IFN-γ were significantly increased in the cord blood of uninfected newborns. The median IFN-γ values of transmitter and non-transmitter mothers were not significantly different; however, non-transmitter mothers had the highest total IFN-γ production among the group of mothers. Collectively, the results of this study suggest that the anti-T. cruzi immune response occurring in the placenta and cord is under the influence of the cytokines from the mother\'s blood and results in the control of parasitemia in uninfected newborns.

Objectives: Congenital diaphragmatic hernia (CDH) is defined as organ protrusion from the abdominal to the thoracic cavity. The Hadlock formula is the most commonly used tool for calculating estimated fetal weight (EFW). The anatomical nature of CDH usually leads to underestimation of the abdominal circumference, resulting in underestimation of fetal weight. Accurate weight estimation is essential before birth for counselling, preparation before surgery and ECMO. The research is made to compare the accuracy of Hadlock\'s formula and Faschingbauer\'s formula for fetal weight estimation in CDH fetuses population. Methods: In our study, we investigated differences between EFW and actual birthweight in 42 fetuses with CDH as compared to 80 healthy matched controls. EFW was calculated using the Hadlock formula and a recently introduced formula described by Faschingbauer et al., which was tailored for fetuses with CDH. Additionally, both of the formulas were adjusted for the interval between the ultrasound and delivery for both of the groups. Results: The majority of hernias were left-sided (92.8% vs. 7.2%). EFW adjusted for the interval between the ultrasound and delivery had the highest correlation with the actual birthweight in both, study group and controls. We compared the results for both tools and found the Hadlock formula to predict birthweight in CDH children with a 7.8 ± 5.5% error as compared to 7.9 ± 6.5% error for the Faschingbauer\'s formula. Conclusions: The Hadlock formula adjusted for the interval between the ultrasound and delivery is a more precise method of calculating EFW in fetuses with CDH. Routine biometry scan using Hadlock\'s formula remains reliable for predicting birthweight.

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Protein-losing enteropathy (PLE) is a rare disorder with diverse causes, but the treatments are limited and understudied. It is often associated with significant mortality and morbidity. The survival of hypoplastic left heart syndrome (HLHS) in infants without any intervention is usually 4.5 days, and 30-day mortality is 95%. However, with surgical intervention, survival at 20 years is 80%. HLHS can lead to protein-leading enteropathy and is corrected by the three-step procedures (Norwood, Glenn, and Fontan) during infancy. We report a case of Fontan procedure postoperative HLHS associated with PLE and describe its clinical course and outcome. The main intention of reporting this case is to provide awareness among physicians while dealing with refractory cases of hypoproteinemia and appropriate management based on the literature.

Generalized arterial calcification of infancy (GACI) is a rare genetic condition with varied clinical presentation. Consequently, diagnosis is frequently delayed or missed. GACI has a poor prognosis, with more than half of patients dying before the age of 6 months. Early diagnosis and treatment with bisphosphonates have been shown to improve survival in these patients. This is a case report of a newborn with respiratory distress who was initially diagnosed with coarctation of the aorta at echocardiography. Further imaging with CT revealed the aortic narrowing to be associated with GACI. Keywords: Genetic Defects, Congenital, Vascular, Calcification/Calculi, Aorta, Pulmonary Arteries, CT Angiography, Echocardiography, Pediatrics © RSNA, 2024.

Background: This study aimed to gather data on physical activity (PA), bleeding, health-related quality of life, and health status, using a wearable device and an electronic patient-reported outcome (ePRO) app, in individuals with moderate or severe hemophilia A (HA) without inhibitors receiving treatment according to the clinical practice. Methods: This is a 12-month multicenter cohort study conducted in Italy. The primary outcomes included the description of PA by type and intensity, adherence to World Health Organization guidelines, bleeding, and health-related quality of life by EQ-5D questionnaire. PA data were collected continuously through a fitness tracker worn by the patient; all the other variables were collected through ePRO questionnaires. Results: Only 54 of the 103 enrolled subjects (52.4%) used their fitness tracker for the defined valid period; adolescents were the least compliant age group. PA was performed at low rates and intensity. Approximately 52% of the subjects had sedentary behavior. The mean EQ-5D values did not change over time. At least one bleeding was reported in 43.7% of the subjects, mostly with sedentary behavior. The PA in the 2 days preceding the bleeding was comparable to the one observed in the overall observational period. Conclusions: The systematic recording of data through a fitness tracker and ePRO app shows that subjects with HA without inhibitors have lower-than-expected PA and that they still experience issues related to bleeding.