Clinical course of pediatric large vascular anomalies located in the extremities.

OBJECTIVE: Difficulties encountered in the diagnosis and treatment of vascular anomalies located in the extremities of the children. The most common vascular lesions are hemangiomas and venous malformations. The complex malformations, such as, Klippel-Trenaunay Syndrome are much less commonly encountered lesions. Treatment of vascular malformations are variable based on the etiology of the lesion and clinical presentation. In this study, we aimed to share our experience on the clinical features of vascular lesions in the extremities of the children. MATERIAL AND METHODS: The demographic, clinical and prognostic features of 330 children with vascular anomalies followed at IUC, Cerrahpasa Medical Faculty, Department of Pediatric Hematology and Oncology were retrospectively reviewed. Fifty-one patients with lesions >5 cm in diameter were included into the study. The diagnosis, age, sex, history of prematurity, lesion type and location, imaging and biopsy findings, complications, details of treatment, and follow-up were evaluated. RESULTS: Twenty-nine (57%) of patients were female and 22 (43%) were male. The female to male ratio was 1.3:1. The median age at admission was 15 months (10 days-180 months). Eight patients (16%) had a history of premature birth. Thirty-one patients (61%) had lesions since birth, eight lesions (8%) appeared in the first month of life and 6 (12%) occurred after 1 year of age. Sixteen of the patients (31%) had hemangioma, 11 (22%) had lymphangioma, 19 (37%) had venous malformation and 5 (10%) were diagnosed as Klippel Trenaunay Syndrome. The lesions were in the upper extremity in 21 patients (41%), in the lower extremity in 27 patients (53%), and both lower and upper extremities were affected in 3 patients (6%). Of all patients, six had intramuscular and two had intraarticular lesions. The diagnosis was made on clinical grounds in most of the cases. In 22 children Magnetic Resonance Imaging was performed for differential diagnosis and to demonstrate the infrastructure of the lesion and the extent of local infiltration. Histopathologic examination by biopsy was done in four patients. Complications developed in 19 patients as follows: Disseminated intravascular coagulation in 6, bleeding in 4, thrombosis in 3, and soft tissue infection in 6. Twenty-one patients were not given any treatment. Medical treatments were propranolol in 14 patients, sirolimus in 4 patients, propranolol and sirolimus in 5 patients. Intralesional bleomycin injection was performed in 3 children. CONCLUSION: The diagnosis, classification and treatment of extremity located vascular malformations in children are complex. Treatment strategy should be defined as in accordance with a combination of the type of the vascular malformation, the age of the patient and the clinical picture.

Neurofibromatosis Type 1 in Children: A Single-Center Experience.

OBJECTIVE: Neurofibromatosis (NF) is the most common autosomal dominantly inherited neurocutaneous syndrome. The characteristic features of NF type 1 (NF-1) are café au lait spots, axillary and inguinal freckling, peripheral neurofibromas, optic pathway glioma, and Lisch nodules. The present study aimed to analyze the clinical features of children with NF-1. MATERIALS AND METHODS: In this study, the children with NF-1 diagnosed and followed-up in our center between 2000 and 2020 were retrospectively evaluated. Demographic and clinical features of patients were defined. RESULTS: The study group consisted of 52 patients. Of those, 25 were boys and 27 were girls. The children's median age at diagnosis was 5.9 years (1-15.8). Café au lait (CAL) spots and axillary/inguinal freckling were observed in 50 and 24 patients, respectively. Neurofibroma was present in 22 cases. Ten of the cohort had optic gliomas, and 39 of them had cranial hamartomas. Orthopedic complications such as scoliosis, tibial pseudoarthrosis, and osteoporosis were observed in 13 patients. Eleven children had neurocognitive disorders. CONCLUSIONS: Early diagnosis is important in neurofibromatosis to prevent the complications of the disease. Also, neurological development and secondary malignancy follow-up should be done carefully in this group of patients.

Chitosan-co-Hyaluronic acid porous cryogels and their application in tissue engineering.

In this work, the usability of chitosan-co-hyaluronic acid cryogels as a tissue-engineering scaffold was investigated. Chitosan-co-hyaluronic acid cryogels were synthesized at subzero temperature. Cryogels which were composed of various compositions of chitosan and hyaluronic acid (0, 10, 20, 30 and 50wt% hyaluronic acid) was prepared. Morphological studies showed that the macroporous cryogels have been developed with 90-95% porosity. Particularly, the mechanical and biomaterial property of pure chitosan was improved by making copolymer with hyaluronic acid in different concentration. The MTT cell viability results demonstrated that the cryogels have no significant cytotoxicity effect on 3T3 fibroblast and SAOS-2 cells.

Zwitterionic phosphorylcholine grafted chitosan nanofiber: Preparation, characterization and in-vitro cell adhesion behavior.

In this study, zwitterionic phosphorylcholine grafted electrospun chitosan fiber was accomplished in three steps: (1) Azide groups on the chitosan were regioselectively replaced with hydroxyl side group and then the product was electrospun. (2) Chitosan based macroinitiator was prepared using an azide-alkyne click reaction from azide-functionalized electrospun chitosan fiber. (3) Poly(2-methacryloyloxyethyl phosphorylcholine) (MPC) was grafted onto the electrospun chitosan fiber by atom transfer radical polymerization (ATRP) in order to enhance cellular viability and proliferation of 3T3, ECV and Saos. The structure of surface modified chitosan was characterized by Fourier transform infrared spectrometer (FT-IR) and 1H nuclear magnetic resonance (1H NMR). The surface morphology of the nanofibers was investigated by scanning electron microscope (SEM). In-vitro cellular attachment and spreading experiments of 3T3, ECV304 and Saos were performed on electrospun chitosan fibers in the presence and the absence of MPC grafting. Poly(MPC) grafted electrospun fiber showed an excellent performance due to phosphorylcholine groups mimicking the natural phospholipid.

Immobilization of α-amylase onto poly(glycidyl methacrylate) grafted electrospun fibers by ATRP.

In this study, novel α-amylase immobilized poly(vinyl alcohol) (PVA) nanofibers were prepared. The PVA nanofiber surfaces were functionalized with 2-bromoisobutyryl bromide (BiBBr) and followed by surface initiated atom transfer radical polymerization (SI-ATRP) of glycidyl methacrylate (GMA). The morphology of the poly(glycidyl methacrylate) (PGMA) grafted PVA nanofibers was characterized by scanning electron microscopy (SEM). Also PGMA brushes were confirmed by X-ray photo electron microscopy (XPS). α-Amylase was immobilized in a one step process onto the PGMA grafted PVA nanofiber. The characteristic properties of the immobilized and free enzymes were examined. The thermal stability of the enzyme was improved and showed maximum activity at 37 °C by immobilization. pH values of the maximum activity of the free and immobilized enzymes were also found at 6.0 and 6.5, respectively. Free enzyme lost its activity completely within 15 days. The immobilized enzyme lost only 23.8% of its activity within 30 days.

Bilateral multiple tumor-like endobronchial tuberculosis, diagnosed with bronchoscopic examination.

Endobronchial tuberculosis is defined as tuberculosis infection of tracheobronchial tree and it is not seen often in adult population. In the absence of parenchymal disease endobronchial tuberculosis is less well-recognized and can lead to difficulties in diagnosis. Our aim is to introduce a rare form of tuberculosis that is important because of high probability of developing severe bronchostenosis during its course. We report a 20-year-old woman who presented with two-month history of severe non-productive cough, shortness of breath, and hemoptysis. After clinical and radiological evaluation, flexible bronchoscopy showed bilateral multiple tumorous lesions that were seen from main carina down to the both main bronchus. The biopsy samples revealed EBTB diagnosis and antituberculosis therapy was given. At the second month of the therapy, rebronchoscopy revealed almost disappearance of the polypoid lesions. The patient healed without any stenosis. This case report is a reminder that endobronchial tuberculosis must take into consideration in differential diagnosis of endobronchial lesions. In patients with endobronchial tuberculosis healing without any complication could be achieved with timely diagnosis and commencement of early treatment.

Evaluation of a single-channel portable monitor for the diagnosis of obstructive sleep apnea.

STUDY OBJECTIVE: To validate the ApneaLINK (AL) as an accurate tool for determining the presence of obstructive sleep apnea (OSA) in an at-risk sleep clinic population in a home test environment. METHODS: Consecutive participants referred with the suspicion of OSA were evaluated in the home with the AL portable monitor (AL Home), followed by simultaneous data collection with diagnostic polysomnography (PSG) and AL in the sleep laboratory (AL Lab). Prevalence, sensitivity, specificity, and ROC curves were calculated for PSG vs. AL Lab, PSG vs. AL Home, and AL Lab vs. AL Home test. Pearson correlations and Bland-Altman plots were constructed. RESULTS: Fifty-three (55% female) participants completed the entire study. The mean age of the population was 45.1 ± 11.3 years, and body mass index was 35.9 ± 9.1 kg/m(2). The prevalence of an apnea hypopnea index (AHI) ≥ 15 in the cohort was 35.9%. The results demonstrated a high sensitivity and specificity of the AL respiratory disturbance index (RDI-AL) compared with the AHI from the PSG. The AL Lab had the highest sensitivity and specificity at RDI-AL values ≥ 20 events/h (sensitivity 100%, specificity 92.5%). The AL Home was most sensitive and specific at an RDI-AL ≥ 20 events/h (sensitivity 76.9%, specificity 92.5%). The Pearson correlations for PSG vs. AL Lab and PSG vs. AL Home were ρ = 0.88 and ρ = 0.82, respectively. The Bland-Altman Plots demonstrated good agreement between the methodologies. CONCLUSION: The AL home test is an accurate alternative to PSG in sleep clinic populations at risk for moderate and severe OSA. TRIAL REGISTRATION: clinicaltrials.gov ID: NCT00354614.

[Vocal cord functions in patients with asthma attack]. / Astim atakli hastalarda vokal kord fonksiyonlari.

This study was planned to evaluate vocal cord functions and to establish underlying vocal cord dysfunctions (VCD) in patients with asthma attack. All patients admitted to emergency service of our hospital with asthma attack between February 01, 2007 and June 01, 2007 were included in the evaluation. After the evaluation, all patients regarded to have asthma attacks based on GINA 2006 guide were enrolled in the study. After first intervention, patients underwent endoscopic larynx examination for the evaluation of vocal cord functions. Twenty four (65.7%) male and 11 female (31.4%), overall 35 patients diagnosed with asthma and who did not have the history of another disease were included in the study. At endoscopic larynx examination carried out after first medical examination, at the moment of asthma attack, tongue, tongue base, epiglottis and arytenoid were observed to be within normal limits. In 9 (25.7%) patients, upper respiratory tract was hyperemic and in 2 (5.7%) odematous. One patient had nasal polyposis (p> 0.05). In the evaluation of vocal cord functions, restriction in adduction was observed in merely one patient. All other functions were normal. Rima opening width was established to be mean 8.34 + or - 0.725. VCD was deteced in none of the patients included in the study. Although we did not detect VCD in any patient, VCD should be borne in mind in cases which presents with the clinical picture of asthma and responds weakly to the treatment or in cases of unexplained shortness of breath. This may prevent many unnecessary procedures such as medication, entubation, tracheostomy and iatrogenic mortality. Further longutudial studies are required in order to shed light on the assocation of asthma with VCD.

Evaluation of the relationship between heart type fatty acid binding protein levels and the risk of cardiac damage in patients with obstructive sleep apnea syndrome.

The aim of this study was to establish cardiac damage related to nocturnal ischemia using heart type fatty acid binding protein (h-fabp), which reaches detectable levels in plasma after being released from myocytes in case of ischemia in obstructive sleep apnea syndrome (OSAS) patients without coronary artery disease (CAD). Fifty patients diagnosed with OSAS in our sleep laboratory with polysomnographic analysis (PSG), who did not have any previous history of cardiac disease and in whom CAD was ruled out with myocardium perfusion scintigraphy, were included in the study. Control group comprised 19 volunteers without history of cardiac disease and risk factors in whom OSAS was excluded with PSG analysis. Blood samples were drawn from the patients to examine h-fabp, creatine kinase (CK), creatine kinase-MB (CK-MB), aspartate aminotransferase (AST), troponin I levels before and after sleep. No significant difference was found in CK, CK-MB, AST, Troponin I, and h-fabp levels before and after sleep in patient and control groups (p > 0.05). No significant difference was found between groups in terms of CK, CK-MB, AST, and Troponin I levels before and after sleep, while a significant difference was found between them with regard to h-fabp levels before (p = 0.006) and after sleep (p = 0.022). When arithmetical mean of the fabp levels before and after sleep was taken in the patient group, it was found that mean value of h-fabp was associated with the desaturated period in sleep which was under 80% (p = 0.04). H-fabp seems to be a marker that will enable the detection of cardiac injury in the early asymptomatic period in OSAS patients before development of disease that can be detected by imaging methods. Further studies are required to investigate the relation between the value of h-fabp and the development of cardiac dysfunction in the long term.