How useful is electrocardiography in children with cochlear implantation?

OBJECTIVES: Hearing loss is an important health problem in children and cochlear implantation is used in its treatment. In addition, in children with hearing loss, accompanying renal, neurological, structural and cardiac conductive pathologies can often accompany. In this study, it was aimed to evaluate the electrocardiographic findings, especially QT duration in children who underwent cochlear implantation. METHODS: The data were obtained from the medical records of our hospital, which is a tertiary referral cochlear implant center. All children who had cochlear implantation surgery due to sensorineural hearing loss and admitted to our hospital for speech therapy between 2019 and 2020 were evaluated with 12-channel ECG. RESULTS: Sixty children (15 girls and 45 boys) with a mean age of 6.5 years were analyzed. Children with a history of heart disease were excluded. In cardiac examinations, five (8.3%) had systolic murmur, four (6.6%) had mid systolic click, and two (3.3%) had stiffening in the first heart sound. Abnormalities were detected in 25% (n = 15) of the electrocardiograms. The most common ECG abnormalities were prolonged QT in 4 cases, left ventricular hypertrophy in 3 cases, left axis deviation in 2 cases, and T wave changes in 2 cases. Long QT syndrome (3.3%) was diagnosed in 2 of the cases with long QT. CONCLUSION: This study demonstrates the need for electrocardiography and cardiac examinations in children undergoing cochlear implantation for hearing loss. These cases should be evaluated especially in terms of the presence of cardiac arrhythmias and potential for fatal ion channelopathy.

The use of sternal wedge osteotomy in pectus surgery: when is it necessary?

BACKGROUND: The Ravitch procedure is a well-established surgical procedure for correction of chest wall deformities. Sternal wedge osteotomy is an important part of this procedure. We studied the incidence of wedge osteotomy with respect to the type of chest wall deformity in patients undergoing surgical correction with the use of a recently developed chest wall stabilization system. METHODS: A total of 47 patients, 39 (83%) male and 8 (17%) female with a mean age of 14.9 ± 2.1 years, underwent the Ravitch procedure. Twenty-four (51.1%) had pectus carinatum, 19 (40.4%) had pectus excavatum, and 4 (8.5%) had pectus arcuatum. A conventional or oblique sternal wedge osteotomy was performed as indicated, followed by chest wall stabilization using the MedXpert system. RESULTS: Of the 47 patients, 27 (57.4%) had a sternal wedge osteotomy. All cases of pectus arcuatum and redo cases underwent sternal wedge osteotomy. Pectus excavatum cases tended to have a greater incidence of wedge osteotomy compared to pectus carinatum cases (68.4% vs. 41.7%, p = 0.052). Patients with more resected ribs had a greater rate of wedge osteotomy (63.4%) compared to those with fewer resected ribs (16.7%, p = 0.043). CONCLUSIONS: A sternal wedge osteotomy is more commonly performed in patients with pectus excavatum compared to those with pectus carinatum. All redo and pectus arcuatum cases need a wedge osteotomy for proper correction. Wedge osteotomy is very likely in more aggressive corrections with more rib resections.

Successful treatment of multiresistant Achromobacter xylosoxidans bacteremia in a child with acute myeloid leukemia.

Achromobacter xylosoxidans is an aerobic gram-negative bacillus and important cause of bacteremia in immunocompromised patients. We describe a leukemia pediatric patient with severe neutropenia who developed bacteremia with A xylosoxidans resistant to multiple antibiotics, and treated the patient with tigecycline and piperacillin-tazobactam in addition to supportive medications.

Non-alcoholic fatty liver disease in obese children and the relationship between metabolic syndrome criteria.

AIM: To investigate metabolic syndrome (MetS) and MetS criteria, and to establish whether metabolic syndrome criteria were associated with non-alcoholic fatty liver disease (NAFLD) in obese children. METHODS: A total of 451 pubertal obese children (8-18 years old) were enrolled in the study. Patients were divided into three groups according to the degree of steatosis. Antropometric and laboratory measurements of the participants were recorded. RESULTS: Of 451 obese children, 217 (48.1%) were diagnosed as having NAFLD and 96 (21.3%) as having MetS. The frequency of abdominal obesity, hypertension, impaired fasting glucose, hyperinsulinemia, dyslipidemia and type 2 diabetes mellitus (T2DM) were 61.8% (279), 25.7% (116), 4.4% (20), 54.3% (245), 41% (185) and 2.2% (10), respectively. The prevalence of NAFLD among patients with MetS [73% (70/96)], was significantly higher than the frequency of hypertension [55% (53/96)] and abnormalities of glucose metabolism [23% (22/96)], but almost equal to the frequency of dyslipidemia [78% (75/96)]. The prevalence of MetS criteria were higher in patients with NAFLD than those without NAFLD. Except impaired fasting glucose, blood pressure and T2DM significant difference was found between groups for all. It was observed that the number of MetS criteria increased in parallel with the severity of steatosis. CONCLUSION: NAFLD in obese children is strongly associated with multiple MetS criteria. In addition to NAFLD is not only a liver disease, but also early mediator that reflects metabolic disorder, and liver ultrasound can be a useful tool for MetS screening.

The utility of serial plasma sE-selectin measurements in the prediction of retinopathy of prematurity in premature infants.

BACKGROUND: sE-selectin has recently been suggested as a surrogate marker for prediction of ROP development. AIMS: The possible role of serial plasma sE-selectin measurements in early prediction and diagnosis of ROP was evaluated. STUDY DESIGN: Prospective observational study SUBJECTS: Forty six preterm infants aged <34weeks of gestation and weighing <1500 g were enrolled. Of these, 26 constituted the ROP group and 20 constituted the no-ROP group. sE-selectin levels were measured serially in blood samples on the 1st day and on 14th and 28th postnatal days. OUTCOME MEASURES: The primary outcome measure was to evaluate the role of sE-selectin concentrations in prediction of ROP. RESULTS: The mean gestational age and birth weight were significantly lower in the ROP group. The mean sE-selectin concentrations in ROP group were significantly greater than those in no-ROP group at each time point (1st, 14th and 28th days of postnatal life). A receiver operating characteristic (ROC) analysis showed that at a plasma concentration of ≥86ng/mL on the 1st postnatal day, sE-selectin had a sensitivity of 100% and a specificity of 94.1% with a positive predictive value of 96.3% and a negative predictive value of 100%. Plasma sE-selectin concentrations were significantly greater in infants who developed ROP in three different time points. CONCLUSIONS: This study shows for the first time that measurement of plasma sE-selectin concentrations as early as the first day of life might help identify preterm infants at risk of ROP.

Effects of ACE inhibitors on insulin resistance and lipid profile in children with metabolic syndrome.

OBJECTIVE: The aim of this study was to evaluate the effects of using ACE inhibitors on insulin resistance, glucose metabolism, body fat composition, and lipid profile in children over 10 years of age with obesity-associated metabolic syndrome (MS). METHODS: A total of 53 children with MS, who had been followed for at least one year were included in the study. The sample was divided into two groups: Group 1-30 obese children (13 female, 17 male) who were not using an ACE inhibitor and Group 2-23 obese children (13 female, 10 male) who were using an ACE inhibitor. Anthropometric and laboratory data obtained at baseline and at the 3rd, 6th, and 12th months of follow-up were compared in the two groups. RESULTS: Comparison of the data in the two groups at 3rd, 6th, and 12th months revealed no statistically significant differences in terms of weight standard deviation score (SDS), body mass index SDS, weight for height percentile, body fat percentage, and very low-density lipoprotein (VLDL)values. However, there were statistically significant differences in mean glucose and insulin levels, homeostasis model assessment for insulin resistance, LDL and high-density lipoprotein values, and highly significant differences in mean triglyceride values. CONCLUSIONS: The positive effects of ACE inhibitor drugs, particularly on hypertriglyceridemia and insulin resistance, might bring them forth as first-line drugs in the treatment of obese and hypertensive children. Randomized, controlled, double-blind, and long-term studies are needed for a definitive conclusion.

Suprasellar mass mimicking a hypothalamic glioma in a patient with a complete PROP1 deletion.

Mutations in PROP1 are the most frequent defect detected in patients with combined pituitary hormone deficiency (MIM #262600), characterized by a clinical phenotype of proportionate growth deficit due to impaired production of growth hormone in combination with deficiency of one or more of the additional anterior pituitary hormones. Approximately one third of patients with PROP1 inactivating mutations present with abnormal development of the anterior lobe of the pituitary gland as revealed by MRI. We report on the clinical and molecular characterization of the fourth complete PROP1 deletion in a girl with proportional short stature, combined pituitary hormone deficiency and a suprasellar mass mimicking a hypothalamic glioma. The proband, born to consanguineous parents, presented with proportional growth failure (height 108.8 cm, -3.48 SDS), combined pituitary hormone deficiency (GH, TSH, PRL and gonadotropins) and a suprasellar mass with optic chiasm invasion, compatible with a diagnosis of chiasmatic hypothalamic glioma, as revealed by MRI. PROP1 mutation screening by PCR and MLPA detected a homozygous deletion of the entire PROP1. The deletion was delimited to at least 7.7 kb upstream of PROP1 and more finely to ∼541-74 bp downstream from PROP1 by aCGH and PCR mapping. We describe the fourth case with a complete PROP1 deletion in homozygosis. The apparent location of the respective 5' (within a highly repetitive region, rich in Alu sequences) and 3' (within an Alu sequence) breakpoints, suggests that the deletion may have arisen through homologous recombination. The differentiation between PROP1 mutation associated pituitary enlargements from craniopharyngioma, pituitary adenoma, dys-germinoma, or Rathke's pouch cyst, is critical for the correct patient management. It is important to recognize that PROP1 mutations can present associated with evolving pituitary masses and/or other MRI alterations of the pituitary during early childhood and that surgery is not indicated in these patients. Therefore, in the presence of combined pituitary hormone deficiency and a pituitary or hypothalamic mass, PROP1 analysis should be considered before referring the patient to a neurosurgeon.

Transient neonatal diabetes mellitus in a Turkish patient with three novel homozygous variants in the ZFP57 gene.

Neonatal diabetes mellitus (NDM) is a rare form of diabetes that presents within the first six months of life. Nearly 70% of these cases have loss of methylation at the differentially methylated region on chromosome 6q24. To describe the findings in a Turkish male patient with NDM caused by a loss of methylation at chromosome 6q24 and three novel homozygous mutations in the ZFP57 gene, methylation-specific PCR was carried out at 6q24 and mutation analysis of ZFP57 gene was maintained by direct sequencing. Sequencing of ZFP57 gene revealed the hypomethylation of chromosome 6q24 and three novel mutations (chr6:29.641.413 A>T, 29.641.073 C>T, and 29.640.855 G>C), respectively. The latter mutation seems to display the patient's condition due to a highly conservative amino acid substitution in the protein. We suggest the ZFP57 gene as a causative factor for NDM and it should be considered in genetic testing. Further studies including functional analysis of the detected mutations will provide precise information regarding the effect of the mutations.