Gokalp Basbozkurt

Measures of pituitary gland and stalk: from neonate to adolescence.

Abstract Objective: The aim of this study is to provide normative data about pituitary diameters in a pediatric population. Pituitary imaging is important for the evaluation of the hypothalamo-pituitary axis defect. However, data about normal pituitary gland diameters and stalk are limited, especially in children. Structure and the measurements of pituitary gland and pituitary stalk may change due to infection, inflammation, or neoplasia. Methods: Among 14,854 cranial/pituitary gland magnetic resonance imaging scans performed from 2011 to 2013, 2755 images of Turkish children aged between 0 and 18 were acquired. After exclusions, 517 images were left. Four radiologists were educated by an experienced pediatric radiologist for the measurement and assessment of the pituitary gland and pituitary stalk. Twenty cases were measured by all radiologists for a pilot study and there was no interobserver variability. Results: There were 10–22 children in each age group. The maximum median height of the pituitary gland was 8.48±1.08 and 6.19±0.88 mm for girls and boys, respectively. Volumes were also correlated with gender similar to height. Minimum median height was 3.91±0.75 mm for girls and 3.81±0.68 mm for boys. The maximum and minimum pituitary stalk basilar artery ratios for girls were 0.73±0.12 and 0.59±0.10 mm. The ratios for boys were 0.70±0.12 and 0.56±0.11 mm. Conclusion: Our study demonstrated the pituitary gland and stalk size data of children in various age groups from newborn to adolescent. It is thought that these data can be applied in clinical practice. Future prospective follow-up studies with larger samples, which correlate the structural findings with the clinical and laboratory results are awaited.

Interleukin-8 and Its Receptors in Human Milk from Mothers of Full-Term and Premature Infants.

In addition to its nutritional benefits, human milk also has bioactive elements. Limited immunological functions of newborns are supported and altered by the immunological elements of mother milk. Chemokines are of importance among these immune factors. Interleukin-8 (IL-8) has been demonstrated in mothers milk, and its receptors, CXC chemokine receptors (CXCR)-1 and CXCR-2, were detected on cells, responsible for immunological reactions and mammary glandular cells. The soluble forms of these receptors are yet to be described in human milk. In this study, it was aimed to assess the IL-8 levels and the concentrations of its receptors in colostrum and mature mothers milk in regard to preterm and term delivery. The results of this study indicated a decline in IL-8 levels with the lactation stage, but no difference was observed between term and preterm mothers milk. Regarding the CXCR-1 and CXCR-2, the concentrations of these receptors were similar in both colostrum and mature milk. Furthermore, there was not any significant difference between term and preterm mothers milk. In conclusion, this is the first study to investigate the concentrations of CXCR-1 and CXCR-2 with the levels of IL-8 in colostrum and mature human milk of term and preterm newborns. The alterations in IL-8 levels were similar in some of the studies reported. CXCR-1 and CXCR-2 levels did not demonstrate any significant difference. Further studies are required to investigate the soluble forms of these receptors and their relation to IL-8 with larger cohort.

A New Mutation in Blau Syndrome

Blau syndrome is a rare, autosomal dominant, granulomatous autoinflammatory disease. The classic triad of the disease includes recurrent uveitis, granulomatous dermatitis, and symmetrical arthritis. Blau syndrome is related to mutations located at the 16q12.2–13 gene locus. To date, 11 NOD2 gene mutations causing Blau syndrome have been described. Here, we describe a 5-year-old male patient who presented with Blau syndrome associated with a novel sporadic gene mutation that has not been reported previously.

PReS-FINAL-2204: Developing of a new scale for assessing the adherence to colchicines treatment in pediatric patients with FMF

Familial Mediterranean Fever (FMF) is a disease characterized by attacks and colchicine is the medication considered most effective in reducing the intensity and frequency of attacks. Adherence to the medication regiment is important not only to manage FMF symptoms, but also to prevent amyloidosis.

A Rare Reason of Hyperinsulinism: Munchausen Syndrome by Proxy

Hyperinsulinism, one of the most important causes of hypoglycaemia, can be congenital or acquired. Rarely, drug toxicity can be a reason for hyperinsulinism. In the context of Munchausen syndrome by proxy (MSP), toxicity usually occurs in children due to drug administration by a parent or caregiver. A 7-year-old girl was referred to our department due to a hyperglycaemic period and hypoglycaemic episodes. On admission, gliclazide was initiated due to her hyperglycaemia, which we attributed to maturity onset diabetes of the young. However, during follow-up, hypoglycaemic levels were detected. Despite cessation of gliclazide, hypoglycaemic seizures occurred. Even with the medications administered, hypoglycaemia could not be prevented. During follow-up, the mothers affect, characterized by anxiety and interest in her daughters medical care, appeared discordant with the situation. Due to our suspicion of MSP, we discovered toxic levels of gliclazide in the blood and urine samples which had been sent to the toxicology laboratory to search for hypoglycaemic agents. The patient was isolated, and all medications were stopped. After isolation, her hypoglycaemia disappeared, and she became hyperglycaemic (250 mg/dl). Physicians should consider the possibility of MSP in hyperinsulinaemic patients with discordant laboratory results and clinical symptoms, even if the childs parents display great concern.

Collapsing Glomerulopathy in a Child with Galloway-Mowat Syndrome

Galloway-Mowat syndrome (GMS) is an autosomal recessive disorder with a poor prognosis that was first defined as a triad of central nervous system involvement, hiatal hernia, and nephrotic syndrome. However, this syndrome is now known to have a heterogeneous clinical presentation. The nephrotic syndrome is steroid resistant and is responsible for the outcome. The combination of collapsing glomerulopathy and GMS is very rare. A 26-month-old boy presented with steroid-resistant nephrotic syndrome associated with neurologic findings, including microcephaly, psychomotor retardation, and nystagmus. Magnetic resonance imaging showed marked cerebral atrophy, optic atrophy, and hypomyelination. A renal biopsy was consistent with collapsing glomerulopathy. If collapsing glomerulopathy is associated with neurological abnormalities, especially with microcephaly, clinicians should consider GMS as a possible underlying cause.

Developing of a new scale for assessing the adherence to colchicine treatment in pediatric patients with FMF.

Familial Mediterranean Fever (FMF) is a disease characterized by attacks and colchicine is the medication considered most effective in reducing the intensity and frequency of attacks. Adherence to the medication regiment is important not only to manage FMF symptoms, but also to prevent amyloidosis.

Adrenomedullin levels in patients with Familial Mediterranean Fever: a long term follow-up.

Familial Mediterranean Fever (FMF) is the most common periodic fever syndrome, characterized by recurrent fever and serositis attacks. It has been shown that there might be an ongoing subclinical inflammation between attacks. Adrenomedullin (ADM) is synthesized in endothelium, and has been shown to have high levels in patients with inflammation such as FMF. Colchicine is the treatment of choice and given once or twice daily depending on expert opinion.

The Relationship between Blood Pressure and Sleep Quality among Obese Adolescents Patients

Objective: To clarify the relation between blood pressure and sleep quality in obese adolescents. Methods: The study was designed and performed as descriptive study. Sleep quality questionnaire, 24hr ambulatory systemic blood pressure monitoring and anthropometric measurements were performed in both study and control groups (age range 10-18, n=22). Demographic data, Pittsburgh Sleep Quality Index and blood pressure measurements were compared. Results: No statistically meaningful association between blood pressure and sleep quality was found. Obese patients (n=30) had been found to have difficulty breathing in their sleep (p=0.001). There were no statistical differences between the obese and non-obese groups with their means of Pittsburg Sleep Quality Index (p=0.059). Conclusions: On contrary to other studies we found that sleep quality does not affect blood pressure in obese adolescents. We concluded that obese adolescents have trouble breathing in their sleep.

P01-038 – QT and JT dispersion in children with FMF

Familial Mediterranean Fever (FMF) is an autoimmune, autosomal recessive inherited disorder, and characterized by recurrent episodes of peritonitis, ploritis and arthritis. Patients with inflammatory disease are at increased risk of cardiovascular complications due to rhythm disorders. QT and JT dispersions are simple and non-invasive arrhythmogenic markers and can be used to assess the homogeneity of cardiac repolarization.