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1.
Anatol J Cardiol ; 26(10): 750-756, 2022 10.
Artigo em Inglês | MEDLINE | ID: mdl-35943317

RESUMO

BACKGROUND: The endothelium is crucial for the control of vascular homeostasis and plays a role in angiogenesis. Leptin, a protein released mainly by adipose tissue, plays a key role in the regulation of energy balance and angiogenesis. We aimed to investigate the changes of endothelial nitric oxide synthetase expression on human umbilical vein endo- thelial cells wound healing model after leptin treatment. METHODS: In this study, 5 groups were planned as Group 1: control (untreated), Group 2: treated with 0.1 ng/mL leptin, Group 3: treated with 1 ng/mL leptin, Group 4: treated with 10 ng/mL leptin, and Group 5: treated with 100 ng/mL leptin. Closure rates of wound areas were calculated by the Image J program after 24 hours of leptin treatment. The WST-1 assay was used to calculate the cell viability. Immunocytochemical analysis was performed for endothelial nitric oxide synthase expression and H-Score was calculated. RESULTS: The closure rates of wound areas were calculated as 80.24%, 89.73%, 87.40%, 90.73%, and 93.70%, respectively. When all groups treated with leptin were comparedwith the control group, there was a statistically significant difference (P < .05). The WST-1 results showed that the most increasing levels of viable cells were found in the groups treated with 0.1 ng/mL leptin and 100 ng/mL leptin when compared to the control group. H-Score values of each group were calculated as 284.8 ± 15.22, 288.6 ± 8.41, 291 ± 8.16, 295.2 ± 11.60, and 308.8 ± 4.32, respectively. The difference between the control group and the group treated with 100 ng/mL leptin was statistically significant (P < .05). CONCLUSIONS: Endothelial nitric oxide synthase expression in human umbilical vein endo- thelial cells increased depending on the leptin dose and the highest increase was in the group treated with 100 ng/mL leptin.


Assuntos
Leptina , Óxido Nítrico Sintase Tipo III , Endotélio Vascular , Humanos , Leptina/metabolismo , Leptina/farmacologia , Neovascularização Patológica , Óxido Nítrico Sintase Tipo III/metabolismo , Veias Umbilicais/metabolismo , Cicatrização
2.
Turk Pediatri Ars ; 54(2): 113-118, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31384146

RESUMO

AIM: The primary purpose of the present study is to evaluate the effect of chenodeoxycholic acid treatment on skeletal system findings in patients with cerebrotendinous xanthomatosis. MATERIAL AND METHODS: This retrospective study was conducted between June 2013 and December 2018 with seven patients with cerebrotendinous xanthomatosis in Cerrahpasa Medical Faculty Pediatric Nutrition and Metabolism Department. The clinical, epidemiologic, and genotypic features of the patients were reviewed in detail and the following items, especially related with skeletal system involvement, were recorded from medical data: history of a bone fracture, plasma calcium, phosphate, alkaline phosphatase and 25-hydroxy-vitamin D concentrations, bone mineral density values of the posteroanterior lumbar spine (L1-L4), and femoral neck before and after chenodeoxycholic acid treatment. RESULTS: Regarding the bone mineral metabolism, plasma calcium, phosphate, alkaline phosphatase levels were found in normal ranges in all patients. Plasma 25-hydroxy-vitamin D evaluation at the time of diagnosis showed deficiency in three patients and insufficiency in three patients. Following chenodeoxycholic acid therapy, 25-hydroxy-vitamin D deficiency persisted in only one patient, but insufficiency was observed in four patients. According to the bone mineral density assessments, four patients had Z-scores below the expected range for age both at initial examination and after chenodeoxycholic acid therapy. No significant difference was observed between plasma 25-hydroxy-vitamin D levels and bone mineral density Z-scores before or after treatment. CONCLUSION: This study elucidates the necessity of additional medical treatment as a part of chenodeoxycholic acid therapy to improve skeletal system findings in cerebrotendinous xanthomatosis.


AMAÇ: Bu çalismanin temel amaci serebrotendinöz ksantomatozis tanili hastalarda kullanilan kenodeoksikolik asit tedavisinin iskelet sistemi bulgulari üzerine olan etkisini tanimlamaktir. GEREÇ VE YÖNTEMLER: Bu geriye dönük çalismaya Haziran 2013 ve Aralik 2018 yillari arasinda Cerrahpasa Tip Fakültesi Çocuk Metabolizma ve Beslenme Bilim Dali'nda izlenen serebrotendinoz ksantomatozis tanili yedi hasta alindi. Hastalara ait klinik, epidemiyolojik ve genotipik özellikler geriye dönük incelendi ve özellikle iskelet sistemini ilgilendiren kemik kirigi öyküsü, kenodeoksikolik asit tedavisi öncesi ve sonrasinda plazma kalsiyum, fosfat, alkalen fosfataz, 25-hidroksi-vitamin D düzeyleri, posteroanterior lomber vertebra (L1-L4) ve femur boynu kemik mineral dansitelerine ait bilgiler kaydedildi. BULGULAR: Kemik mineral metabolizmasinin degerlendirilmesi açisindan ölçülen plazma kalsiyum, fosfat, alkalen fosfataz seviyeleri tüm hastalarda normal sinirlardaydi. Tani aninda yapilan plazma 25-hidroksi-vitamin D degerlendirilmesi; üç hastada eksiklik, üç hastada yetersizlik ile uyumluydu. Kenodeoksikolik asit tedavisi sonrasinda 25-hidroksi-vitamin D eksikligi bir hastada devam ederken, dört hastada yetersizlik saptandi. Kemik mineral dansitometri degerlendirmelerinde, dört hastada kemik mineral dansitesi Z-skorlarinin baslangiç aninda ve tedavi sonunda yasa göre düsük olarak devam ettigi görüldü. 25-hidroksi-vitamin D ve kemik mineral dansitometrisi Z-skorlari arasinda tedavi öncesi ve sonrasinda istatistiksel olarak anlamli farklilik izlenmedi. ÇIKARIMLAR: Bu çalismada, serebrotendinöz ksantomatozis tanili hastalarda iskelet sistemi bulgularinin iyilestirilmesi amaciyla kenodeoksikolik asit tedavisine ek olarak farkli medikal tedavilerin gerekli olabilecegi üzerinde durulmustur.

3.
J Pediatr Endocrinol Metab ; 31(3): 345-348, 2018 Mar 28.
Artigo em Inglês | MEDLINE | ID: mdl-29305569

RESUMO

BACKGROUND: As KATP channel mutations are the most common cause of neonatal diabetes mellitus (NDM) and patients with these mutations can be treated with oral sulfonylureas, empiric therapy is a common practice for NDM patients. CASE PRESENTATION: A non-syndromic, small for gestational age baby born to first-degree consanguineous parents was diagnosed with NDM. Because of hypo- and hyperglycemic episodes and variability in insulin requirement, we initiated a trial of glibenclamide, with a presumptive diagnosis of NDM caused by a KATP channel mutation. However, this empiric sulfonylurea trial did not improve the patient's glycemic control and resulted in resistance to exogenous insulin. Genetic testing identified a previously reported homozygous INS promoter mutation (c.-331C>G), which was not responsive to sulfonylurea therapy. CONCLUSIONS: In light of our results, we recommend to confirm the genetic diagnosis as soon as possible and decide on sulfonylurea treatment after a genetic diagnosis is confirmed.


Assuntos
Diabetes Mellitus/genética , Glibureto/uso terapêutico , Doenças do Recém-Nascido/genética , Resistência à Insulina/genética , Insulina/genética , Glicemia/análise , Consanguinidade , Diabetes Mellitus/tratamento farmacológico , Glibureto/efeitos adversos , Homozigoto , Humanos , Recém-Nascido , Doenças do Recém-Nascido/tratamento farmacológico , Recém-Nascido Pequeno para a Idade Gestacional , Insulina/uso terapêutico , Masculino , Mutação , Regiões Promotoras Genéticas/genética
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