Browsing by Author "SAĞLAM, HALİL"

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A determination of the quality of life of children and adolescents with type 1 diabetes and their parents
(Elsevier Science Bv, 2017-04-10) Avdal, Elif Unsal; ÖZYAZICIOĞLU, NURCAN; Sağlam, Halil; SAĞLAM, HALİL; Bursa Uludağ Üniversitesi/Veteriner Fakültesi/Pediatri Anabilim Dalı.; Bursa Uludağ Üniversitesi/Sağlık Bilimleri Fakültesi/Hemşirelik Anabilim Dalı.; 0000-0001-9645-7221; 0000-0002-6598-8262; AAI-6835-2021; C-7392-2019; GLN-8241-2022
Objective: Type 1 diabetes is a chronic illness which can have a negative effect on the health care and development of children and can put their lives in danger. This descriptive study aimed to determine the quality of life and the factors affecting it of children and adolescents with type 1 diabetes using the Pediatric Quality of Life Inventory (PedsQL).Methods: The study was conducted in the Child Endocrinology Unit of a University Hospital in Bursa, Turkey. Totally 64 children eight- 12 years and 85 adolescents aged 13-18 with type 1 diabetes and their parents were recruited. HbA1c measurements were obtained from the records of the endocrinology clinicare, which were made once every three months, and the average of the last three measurements was taken in the study. The PedsQL was used to measure quality of life of the children and adolescents. The data was analyzed using SPSS version 20.0 and P < 0.05 was considered significant. The demographic data of the children and parents were analyzed using means and percentiles. Pearson's correlation analysis was used to assess the relationship between two averages.Results: Of the sub-groups on the scale, affective and school function scores were somewhat low, and social function scores were high. The quality of life scores of the children and adolescents were found to correlate with those of their parents. A negative correlation was found between HbA1c levels and adolescent quality of life, a positive correlation was found between the child's age and the quality of life of the child and parents, and a negative correlation was found between the number of children in the family and the quality of life of the child and parents.Conclusions: An evaluation of the quality of life after a diagnosis of diabetes can be used to assess the problems which may be faced by children and adolescents and to combat these problems.
Bone mineral density and the influencing factors in the survivors of childhood all
(Blackwell Publishing, 2008-04-01) Can, E.; SAĞLAM, HALİL; Güneş, A. M.; Özarda, Yeşim; ÖZARDA, YEŞİM; Sağlam, Halil; Baytan, B.; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Pediatri Anabilim Dalı.; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Biyokimya Anabilim Dalı.; 0000-0003-1214-0177; 0000-0002-6598-8262; 0000-0002-9375-2855; AAL-8873-2021; C-7392-2019
Clinical and laboratory characteristics of the children with diabetic ketoacidosis
(Galenos Yayıncılık, 2008-12-01) Sağlam, Halil; Eren, Erdal; Çakır, Esra Deniz; Yüce, Necla; Yıldız, Nalan; Çakır, Seher; Özgür, Taner; Tarım, Ömer; SAĞLAM, HALİL; EREN, ERDAL; Çakır, Esra Deniz; Yüce, Necla; Yıldız, Nalan; Çakır, Seher; ÖZGÜR, TANER; TARIM, ÖMER FARUK; Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı.; Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Endokrinoloji Anabilim Dalı.; 0000-0002-6598-8262; 0000-0002-1684-1053; 0000-0003-4664-7435; 0000-0003-0710-5422; C-7392-2019; JPK-3909-2023; GQO-9634-2022; JKN-1212-2023; IPY-4170-2023; EPI-7647-2022; AAG-8381-2021; EBM-1323-2022
Aim: Diabetic ketoacidosis (DKA) is a serious condition with the highest rates of morbidity and mortality in children with type 1 diabetes mellitus (T1 DM). Hospitalization due to DKA constitutes about 2 to 8 percent of all-cause admissions. About 25% of children with newly diagnosed T1DM present with DKA. In this study, we aimed to evaluate sociodemographic and clinical characteristics of children admitted with DKA.Materials and Method: Records of 490 children admitted with T1DM between January 2003 and October 2008 were retrospectively reviewed and data on sociodemographic, clinical and laboratory characteristics of 163 children with the diagnosis of DKA was investigated in detail.Results: Of 490 children with T1DM, 163 (33.2%) had the diagnosis of DKA and total number of DKA episodes was 190. The recurrence rate was 14.2% and 85% (6 out of 7) of those with recurrent episodes were girls. The mean age was 11.02 4.57 years and DKA episodes were most frequently seen in 10-15 (39.3) age-group. DKA episodes peaked in the periods of January-February-March and children (i.e, 0-5 age group) (54 5%) The mean duration of recoveiy from acidosis was 14.29+/-12.14 hours the mean blood glucose level at baseline was 473.09 +/- 141,07 mg/dI and the mean initial HbAl c level vvas 12.04+/-2.42%, In this approximately six-year-penod, only one patient was lost and the mortality rate was 0,5% (1 tit of 190 episodes).Conclusion: Increasing the knowledge of primary care physicians who see the patients first on DKA and al3plying the DKA treatment protocol more slowly and cautiously in younger children (0-5 age group) will definitely loldver the morbidity and mortalibt of DKA.
Comparison of plasmapheresis with medical apheresis in terms of efficacy and cost in the acute treatment of hypertriglyceridemia in children with
(Walter, 2021-11-01) Erdöl, Şahin; Bilgin, Hüseyin; Sağlam, Halil; ERDÖL, ŞAHİN; BİLGİN, HÜSEYİN; SAĞLAM, HALİL; Uludağ Üniversitesi/Tıp Fakültesi/Pediatri Anabilim Dalı/Metabolizma Bilim Dalı; 0000-0002-6598-8262; GLN-8241-2022; JMQ-9930-2023; HSF-7083-2023
Objectives: We aimed to compare plasmapheresis and medical apheresis as lipid-lowering therapies in children with familial lipoprotein lipase (LPL) deficiency. Methods: The data of 13 patients who were followed up after a diagnosis of LPL deficiency were retrospectively analyzed. Plasma triglyceride, cholesterol, amylase, and lipase values and complications were recorded before and after each patient underwent plasmapheresis or medical apheresis. Results: The mean follow-up period of the patients was 99.64 +/- 52.92 months in the medical apheresis group and 118 +/- 16.97 months in the plasmapheresis group. While the mean triglyceride level before plasmapheresis was 1,875.38 +/- 547.46 mg/dL, it was 617 +/- 228.28 mg/dL after plasmapheresis. While the mean triglyceride level before medical apheresis was 1,756.86 +/- 749.27 mg/dL, it was found to be 623.03 +/- 51.36 mg/dL after medical apheresis. Triglyceride levels were decreased by 59.62% with medical apheresis and 65.57% with plasmapheresis. The cost of treatment for medical apheresis was found to be lower compared to plasmapheresis 296.93 +/- 29.94 Turkish lira (USD 43.34 +/- 4.01) vs. 3,845.42 +/- 156.17 Turkish lira (USD 561.37 +/- 20.93; p<0.001). Conclusions: Although there is no standardized strategy for the acute treatment of hypertriglyceridemia due to LPL deficiency, medical apheresis is a safe and effective treatment with a low risk of side effects. Unlike plasmapheresis, medical apheresis can be performed in any center, which is another important advantage of the procedure. A greater catabolism. diabetes, tions gene the titis, xhantoms acute zil, familial and these there treatment. ting
Diagnostic criteria of growth hormone deficiency needs to be reevaluated
(Galenos Yayincilik, 2012-12-01) Salı, Enes; Tarım, Ömer; TARIM, ÖMER FARUK; Sağlam, Halil; SAĞLAM, HALİL; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı.; 0000-0002-6598-8262; 0000-0002-5322-5508; GLN-8241-2022; C-7392-2019
Introduction: In this study, we aimed to investigate the factors that determine response to growth hormone (GH) treatment in children with isolated idiopathic GH deficiency treated in our university hospital.Materials and Methods: The data of 149 patients followed in our university hospital between January 1995 and December 2005 were evaluated in patient charts retrospectively.Results: The maximum GH response to clonidine and glucagon stimulation tests was 4.58 and 6.01 ng/ml respectively. The mean GH dose was 0.215 (0.18-0.25) mg/kg/week. Height SDS significantly increased after the first and second years of GH treatment (p<0.05). There was a negative correlation between the maximum response to stimulation tests and the response to GH treatment (p<0.05). When the patients were grouped according to the maximum response to stimuli as <3, 3-5, 5-7, and >7 ng/ml, the best response to therapy was observed in the group with the least response (<3ng/ml) to both clonidine and glucagon stimulation tests. A positive correlation was determined between height gain and GH dose, and a negative correlation between height gain and age at the beginning of therapy (p<0.05). There was no correlation between target height, baseline pubertal stage, relative weight and IGF-1 SDS with the response to GH treatment (p>0.05).Conclusions: Patients who were started treatment at an early age, whose response to GH stimulation tests were lower, and who were given higher doses benefited more from GH treatment. Therefore, we suggest that the diagnostic criteria for GH deficiency must be reevaluated and the random cut-off of <10ng/ml for stimulated GH level may lead to overdiagnosis. This issue must be further investigated in larger patient populations with consideration of final height as well.
Does growth hormone treatment advance bone age?
(Galenos Yayincilik, 2017-08-01) Sağlam, Halil; SAĞLAM, HALİL; Tarım, Ömer; TARIM, ÖMER FARUK; Salı, Enes; Eren, Erdal; EREN, ERDAL; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı.; 0000-0002-6598-8262; 0000-0002-1684-1053; 0000-0002-5322-5508; JPK-3909-2023; C-7392-2019
Introduction: Growth hormone (GH) treatment may have a stimulatory effect on bone maturation which raises concern about acceleration of bone maturation leading to early epiphyseal fusion. In this study, we aimed to investigate this possibility and determine whether or not accelerated bone maturation affects predicted or final height.Methods: A total of 230 patients who received GH treatment between 1995 and 2005 were retrospectively studied. The patients were classified as isolated growth hormone deficiency (GHD) (Group-1), Turner syndrome (Group-2), and others (Group-3). The previous X rays were obtained from the medical records and follow-up X rays were requested during the clinic visit. Bone age (BA) assessment was done according to the atlas of Greulich and Pyle. Based on BA, adult height prediction was calculated by the method of Bayley and Pinneau. The predicted height (PH) at baseline and after the first and second years of treatment were evaluated along with the change in chronological age (CA)-BA and CA/BA.Results: Height SDS significantly increased in all three groups (p<0.05). CA-BA decreased in Groups-1 and 2 during the first and second years of treatment (p<0.05). The BA acceleration was verified by the decrement in CA/BA ratio. However, predicted height was not negatively influenced despite BA advancement.Discussion and Conclusion: BA advances with GH treatment as evident from the reduction in CABA and CA/BA. However, predicted height is not adversely affected by accelerated bone maturation in our cohort over two years of treatment. This issue needs to be investigated in other patient groups such as precocious puberty where bone maturation is further accelerated.
Efficacy of biphosphonates in patients with osteogenesis imperfecta
(Galenos Yayincilik, 2011-12-01) Eren, Erdal; EREN, ERDAL; Sincar, Sahin; Çakır, Esra Deniz Papatya; Saglam, Halil; SAĞLAM, HALİL; Tarım, Ömer; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı.; 0000-0002-1684-1053; 0000-0003-4664-7435; 0000-0002-6598-8262; 0000-0002-5322-5508; GQO-9634-2022; C-7392-2019; JPK-3909-2023; GLN-8241-2022
Introduction: Osteogenesis imperfecta (OI) is a genetic disorder resulting in increased bone fragility due to defective collagen synthesis. Biphosfonates have been used in children with OI. Herein, we aimed to present clinical and laboratory features of the patients with OI and to evaluate response to biphosphonate therapy.Materials and Methods: The data of 21 patients with OI were evaluated retrospectively and clinical classification was made. Age, gender, auxological data, bone mineral density (BMD), and bone fragility before and after therapy were evaluated. Efficacy of alendronate and pamidronate on the bone density were compared.Results: Of the 21 patients, 12 were male (57.1%), 9 were female (42.9%) and median age was 5.64 years. According the Sillence classification, 10 patients were classified as type I, 9 cases as type III, and 2 cases as type IV. Diagnoses were made by multiple fractures (10 cases), pathological sole fracture (5 cases), blue sclera with history of fracture (2 cases), maternal OI, blue sclera, and decreased BMD (1 case), bone deformity, family history, and blue sclera (1 case). Pamidronate (13 cases) and alendronate (8 cases) were given as medical therapy. Basal DEXA z-score increased from -4.00 at baseline to -2.80 after 12 months of therapy (p< 0.001). When the two therapy models were compared, there was no statistical difference. After one year of therapy, height SDS increased from -2.55 to -1.74 (p= 0.433), and weight SDS increased from -1.79 to -0.51 (p= 0.042). Annual fracture frequency decreased significantly from 2.14 to 0.62 per year (p< 0.001).Conclusions: Three monthly pamidronate or daily alendronate therapy is effective to reduce clinical symptoms and annual fracture rate. Both biphosphonates enhance BMD safely without remarkable side effects. However, duration of therapy and possible side effects in the long run are still unclear and further research in larger groups is necessary.
Evaluation of children presenting with diabetic ketoacidosis
(Galenos Yayıncılık, 2016-12-01) Koyuncu, Ekrem; Sağlam, Halil; Tarım, Ömer; Koyuncu, Ekrem; SAĞLAM, HALİL; TARIM, ÖMER FARUK; Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı.; Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı/Çocuk Endokrinoloji Bilim Dalı.; 0000-0003-0710-5422; CZY-1453-2022; C-7392-2019; JHM-8761-2023
Introduction: Diabetic ketoacidosis (DKA) is a serious result of insulin deficiency and cause serious mortality and morbidity in children with type 1 diabetes mellitus (DM). Retrospective evaluation of DKA cases is of importance in terms of the evaluation of responses to treatment and reducing the complications.Materials and Methods: A total of 412 DKA attacks of 379 patients, who were treated with the diagnosis DKA in Uludag University Faculty of Medicine Department of Child Endocrinology between the dates 01.01.1997 and 15.11.2012, were retrospectively examined. Those who were not diagnosed before applying were named as new diagnosis type 1 DM; and those who were diagnosed with type 1 DM before were named as old type 1 DM.Results: While the median symptom time in new diagnosis type 1 DM group is 14 days and the most frequent symptoms are polyuria, polydipsia and weight loss; and the median symptom time in old diagnosis type 1 DM group is 2 days and the most frequent symptoms are vomiting, polyuria and polydipsia. The most frequent DKA causes in old diagnosis type 1 DM Group are; skipping insulin dose (58.6%) and not following the diet (56%). Within a period of 15 years, 2 ptients in new diagnosis type 1 DM group died due to cerebral edema and related complications.Conclusions: DDKA is the most important complication of type 1 DM and the main reason of type 1 DM related deaths. DKA development can be reduced by training parents on the fact that symptoms like polyuria, polydipsia and weight loss, may be symptoms of type 1 DM. Particular attention shall be paid for younger age group because the most severe DKA is observed between 0-5 years of age.
Evaluation of children with goiter and treatment outcomes
(Bursa Uludag Univ, 2008-06-01) Karalı, Yasin; KARALI, YASİN; KARALI, ZUHAL; Sağlam, Halil; SAĞLAM, HALİL; Kamber, Kadri; Sığırlı, Deniz; SIĞIRLI, DENİZ; Tarım, Ömer; TARIM, ÖMER FARUK; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı.; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Biyokimya Anabilim Dalı.; 0000-0002-6598-8262; 0000-0002-5322-5508; C-7392-2019; AAA-7472-2021
Aim: Goiter is defined as the enlargement of thyroid gland independent of the etiology. It is common in childhood, especially in adolescence. The prevalence of goiter in children aged 6 to 12 years of age was found as 24.9 to 92% in different studies performed in different regions of Turkey. The most common cause in endemic regions is iodine deficiency and it is chronic lymphocytic thyroiditis in non-endemic areas. The aim of this study was to evaluate the demographic and diagnostic characteristics; and physical, laboratory and radiological findings of the children who were presented and followed-up with goiter, and the efficacy of the treatments applied was also assessed.Materials and Methods: A total of 116 children diagnosed as having goiter by physical and ultrasonographic examination in the outpatient clinic of Pediatric Endocrinology Division of Uludag University Faculty of Medicine, between January 2000 and December 2005. Data of the patient were collected retrospectively from the files/records of the patients. Goiter grades of the patients were determined according to that recommended by WHO. Thyroid volumes were calculated from the ultrasonographic measurement of thyroid dimensions. Patients with euthyroid or hypothyroid goiter had L-thyroxine therapy and those with hyperthyroidism had propiltiourasil and/or propranolol.Results: Of all children, 80 (69%) were girls and the remaining 36 were boys (31%). At the time of initial diagnosis, the mean ages of the girls and the boys were 10.1 +/- 2.9 years and 9.02 +/- years, respectively. The mean follow-up period was 3.18 +/- 1.83 years. Most children (76.7%) had grade Ib (38.8%) or II (37.9%) goiter at the time of diagnosis. Of all children with goiter, 62 (53.4%) were euthyroid, 47 (40.5%) were hypothyroid and 7 (6.0%) were hyperthyroid. Seventy nine (68.1%) patients had history of thyroid disease in their families. There were additional endocrinological disturbances, epilepsy and other systemic diseases in 11 (9.5%), 11 (9.5%) and 7 (6.0%) patients, respectively. Eighty three (71.6%) patients had simple diffuse goiter, 18 had Hashimoto thyroiditis (4 of whom also having nodules), 11 (10.4%) had isolated noduler/multinodler goiter, 3 (2.6%) had Graves disease and 1 (0.9%) had papillary thyroid carcinoma. Mean thyroid volume by ultrasonography was significantly decreased from 12.68 +/- 3.57 (3.4 to 35.7) ml to 9.2 +/- 3.57 (3.19 to 22.1) ml with the treatment (p<0.05). Similarly goiter grades by physical examination were also significantly decreased, with only 36.2% of the patients having grade Ib (26.7%) or grade II (9.5%) goiter after the treatment (vs. a total of 76.7% at baseline) (p<0.01). Thyroid nodules were significantly regressed in 11 of 16 patients with noduler goiter (p<0.01).Conclusion: Since TSH (Thyroid-stimulating hormone) elevation seems to be the main driving force in the pathogenesis of thyroid enlargement, thyroxine treatment is warranted to suppress the TSH in hypothyroid and euthyroid patients. The number of hyperthyroid patients in our study is not sufficient to draw conclusions, and the pathogenesis of goiter in hyperthyroidism does not involve TSH which is already suppressed by endogenous thyroid hormones. Nevertheless, control of hyperthyroidism with antithyroid medication and appropriate introduction of thyroxine seem to remedy the goiter as well.
Evaluation of late effects of postoperative radiotherapy in patients with medulloblastoma
(Turkish J Pediatrics, 2015-03-01) Karadağ, Oya; Demiröz-Abakay, Candan; Özkan, Lütfi; Sağlam, Halil; Demirkaya, Metin; DEMİRÖZ ABAKAY, CANDAN; Özkan, Lütfi; SAĞLAM, HALİL; Demirkaya, Metin; Uludağ Üniversitesi/Tıp Fakültesi/Radyasyon Onkolojisi Anabilim Dalı.; Uludağ Üniversitesi/Tıp Fakültesi/Pediatrik Endokrinoloji Anabilim Dalı.; Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı.; 0000-0003-0710-5422; GLN-8241-2022; C-7392-2019; ETY-3982-2022; JGQ-9310-2023; EUG-4353-2022
We aimed to evaluate long-term toxicity in children with medulloblastoma treated with postoperative radiotherapy (RT).This study included 21 patients aged 4-16 who had been diagnosed with medulloblastoma. All of the patients in the study received postoperative craniospinal RT. Postoperative RT followed by chemotherapy was the treatment protocol. A total of 13 patients (62%) received chemotherapy concurrently with RT.Overall survival was 50 months (range, 1-169 months) and disease-free survival was 39 months (range, 4-171 months). In the data analysis, the heights of 11 patients (91.6%) were found to be below 50% on the height curve, and 8 (66.6%) patients had weights below 50% on the weight curve. Mean sitting height was 72.58 +/- 6.33 cm, and this was statistically correlated with parameters such as LH level (p=0.037), testosterone level (p=0.020), height (p=0.002), weight (p=0.033) and age at diagnosis (p=0.002).Radiation therapy for medulloblastoma seems to have a late toxic effect on long-term survivors. With the improving survival rate of medulloblastoma patients, RT doses should be as low as possible without sacrificing efficacy.
Evaluation of the endocrine functions in pediatric patients with cyanotic congenital heart disease
(Scientific Publishers India, 2013-01-01) Eren, Erdal; Çakır, Esra Deniz Papatya; Bostan, Özlem; Sağlam, Halil; Tarım, Ömer; EREN, ERDAL; Çakır, Esra Deniz Papatya; BOSTAN, ÖZLEM MEHTAP; SAĞLAM, HALİL; TARIM, ÖMER FARUK; Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı/Çocuk Endokrinolojisi Bilim Dalı.; Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı/Çocuk Kardiyoloji Bilim Dalı.; 0000-0002-1684-1053; 0000-0003-4664-7435; 0000-0001-7707-2174; 0000-0002-6598-8262; 0000-0002-5322-5508; GQO-9634-2022; AAG-8558-2021; JPK-3909-2023; C-7392-2019; GLN-8241-2022; CCU-8073-2022
Our aim was to investigate effects of chronic hypoxia on endocrine function in pediatric patients with cyanotic congenital heart disease (CHD). Thirty patients with cyanotic CHD (16 boys, 14 girls), and 35 control subjects (22 boys, 13 girls) were enrolled. Age means of patients and controls were 4.37 +/- 4.51 and 4.28 +/- 4.96 years, respectively. Standard deviation scores (SDS) of height and weight were significantly lower among patients compared to controls. Mean fasting glucose levels were 75 +/- 15 mg/dL and 83 +/- 12 mg/dL among patients and controls, respectively (p=0.033). Insulin-like growth factor (IGF) 1 and its SDS were significantly lower among patients (p=0.010). There was no significant difference in ACTH and cortisol levels between groups. ACTH levels were very low in six patients. Oxygen saturation level was positively correlated with ACTH (p=0.041, r=0.439) and negatively correlated with HOMA-IR (p=0.046, r=-0.420) and insulin (p=0.017, r=-0.494). There was no difference in insulin resistance between groups. Chronic hypoxia has negative impact on growth by reducing IGF-1 along with the nutritional deficiency in children with cyanotic CHD. ACTH-adrenal axis is also affected. While cyanotic CHD has decreased serum glucose level, it had no effect on insulin level and insulin resistance. Negative correlations between oxygen saturation, and HOMA-IR, fasting insulin levels, have suggested that these patients should be monitorized for insulin resistance.
Evaluation of the patients with congenital hypothyroidism: Effect of the national screening program
(Galenos Yayincilik, 2011-04-01) EREN, ERDAL; SAĞLAM, HALİL; Zengin, Aysel; Sağlam, Halil; Çakır, Esra Deniz P.; Gül, Yahya; Tarım, Ömer; TARIM, ÖMER FARUK; Gül, Yahya; Özgür, Taner; ÖZGÜR, TANER; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı.; 0000-0002-1684-1053; 0000-0002-6598-8262; 0000-0003-4664-7435; 0000-0002-5322-5508; AAG-8381-2021; GQO-9634-2022; C-7392-2019; JPK-3909-2023; GLN-8241-2022
Introduction: Congenital hypothyroidism (CH) is the most common cause of preventable mental retardation. The course of the disease can be silent and may cause serious health problems when the diagnosis is delayed. "Screening Program for CH" has facilitated early diagnosis and treatment preventing severe complications.Materials and Method: The records of the 140 patients with CH were retrospectively analyzed. The patients with CH were compared before and after the screening program.Results: Of the 140 cases, 74 (52.9%) were female and 66 (48.4%) were male. The clinical presentations of the patients with CH were jaundice (32.1%), constipation (32.1%), referral due to high TSH (25.7%), and growth retardation (10.7%). The etiologies of CH based on scintigraphic imaging of the thyroid gland ( 32 cases) were thyroid dyshormonogenesis in 43.7%, thyroid agenesis in 43.7%, and ectopic thyroid in 12.6%. The mean age at diagnosis was 292 +/- 632 days (370.9 +/- 704.6 days before screening, 35.2 +/- 48.3 days after screening). A total of 36 patients were diagnosed with mental retardation (35 were diagnosed prior to screening). Mean height SDS of patients diagnosed after screening program was significantly higher than that of those diagnosed before screening program (p=0.02). Mean serum TSH, TT4, and FT4 at admission were 144.8 +/- 227.1 mIU/ml, 4.92 +/- 4.44 mcg/dl, and 0.63 +/- 0.41 ng/L, respectively.Conclusions: Significant reduction in the number of patients with mental retardation after the screening program shows once again the importance of this program.
Growth curves for Turkish girls with Turner syndrome: Results of the Turkish Turner syndrome study group
(Galenos Yayıncılık, 2015-04-21) Darendeliler, Feyza; Yeşilkaya, Ediz; Bereket, Abdullah; Baş, Firdevs; Bundak, Rüveyde; Sarı, Erkan; Aydın, Banu Küçükemre; Darcan, Şükran; Dündar, Bumin; Büyükinan, Muammer; Kara, Cengiz; Mazicioğlu, Mümtaz M.; Adal, Erdal; Akıncı, Ayşehan; Atabek, Mehmet Emre; Demirel, Fatma; Çelik, Nurullah; Özkan, Behzat; Özhan, Bayram; Orbak, Zerrin; Ersoy, Betül; Doğan, Murat; Ataş, Ali; Turan, Serap; Göksen, Damla; Tarım, Ömer; Yüksel, Bilgin; Ercan, Oya; Hatun, Sükrü; Şimşek, Enver; Ökten, Ayşenur; Abacı, Ayhan; Döneray, Hakan; Özbek, Mehmet Nuri; Keskin, Mehmet; Önal, Hasan; Akyürek, Nesibe; Bulan, Kezban; Tepe, Derya; Emeksiz, Hamdi Cihan; Demir, Korcan; Kızılay, Deniz; Topaloğlu, Ali Kemal; Eren, Erdal; Özen, Samim; Demirbilek, Hüseyin; Abalı, Saygın; Akın, Leyla; Eklioğlu, Beray Selver; Kaba, Sultan; Anık, Ahmet; Baş, Serpil; Ünüvar, Tolga; Sağlam, Halil; Bolu, Semih; Özgen, Tolga; Doğan, Durmuş; Çakır, Esra Deniz; Şen, Yaşar; Andiran, Nesibe; Çizmecioğlu, Filiz; Evliyaoğlu, Olcay; Karagüzel, Gülay; Pirgon, Özgür; Çatlı, Gönül; Can, Hatice Dilek; Gürbüz, Fatih; Binay, Çiğdem; Baş, Veysel Nijat; Sağlam, Celal; Gül, Davut; Polat, Adem; Açıkel, Cengizhan; Çınaz, Peyami; Can, Hatice Dilek; Doğan, Durmuş; Çakır, Esra Deniz; SAĞLAM, HALİL; EREN, ERDAL; TARIM, ÖMER FARUK; Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Endokrinolojisi Anabilim Dalı.; 0000-0002-1684-1053; 0000-0003-0710-5422; 0000-0003-4664-7435; JHM-8761-2023; JPK-3909-2023; C-7392-2019; AID-3610-2022; GQO-9634-2022; IRJ-2200-2023
Objective: Children with Turner syndrome (TS) have a specific growth pattern that is quite different from that of healthy children. Many countries have population-specific growth charts for TS. Considering national and ethnic differences, we undertook this multicenter collaborative study to construct growth charts and reference values for height, weight and body mass index (BMI) from 3 years of age to adulthood for spontaneous growth of Turkish girls with TS.Methods: Cross-sectional height and weight data of 842 patients with TS, younger than 18 years of age and before starting any therapy, were evaluated.Results: The data were processed to calculate the 3rd, 10th, 25th, 50th, 75th, 90th and 97th percentile values for defined ages and to construct growth curves for height-for-age, weight-for-age and BMI-for-age of girls with TS. The growth pattern of TS girls in this series resembled the growth pattern of TS girls in other reports, but there were differences in height between our series and the others.Conclusion: This study provides disease-specific growth charts for Turkish girls with TS. These disease-specific national growth charts will serve to improve the evaluation of growth and its management with growth-promoting therapeutic agents in TS patients.
Growth hormone deficiency
(Galenos Yayıncılık, 2010-04-01) Tarım, Ömer; Sağlam, Halil; TARIM, ÖMER FARUK; SAĞLAM, HALİL; Uludağ Üniversitesi/Tip Fakültesi/Çocuk Sağlığı Hastalıkları Anabilim Dalı/Çocuk Endokrinoloji Bilim Dalı; 0000-0002-5322-5508; 0000-0002-6598-8262; C-7392-2019; CCU-8073-2022
Growth hormone deficiency is the most promising entity in terms of response to therapy among the treatable causes of growth retardation. It may be due to genetic or acquired causes. It may be isolated or a part of multiple hormone deficiencies. Diagnostic criteria and therefore treatment indications are still disputed.
Maltodextrin may be a promising treatment modality after near-total pancreatectomy in infants younger than six months with persistent hyperinsulinism: A case report
(Galenos Publ House, 2023-03-01) Öngen, Yasemin Denkboy; EREN, ERDAL; DENKBOY ÖNGEN, YASEMİN; Eren, Erdal; SAĞLAM, HALİL; Sağlam, Halil; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Endokrinoloji Anabilim Dalı.; 0000-0002-1684-1053; 0000-0002-6598-8262; KHZ-1491-2024
Persistent hypoglycemia in infants with congenital hyperinsulinism (CHI) can be challenging in approximately half of these cases, even after undergoing a near-total pancreatectomy. While maltodextrin has been recommended in the nutritional management of CHI cases younger than six months, information regarding its efficacy in managing hypoglycemia are not yet clear. Here, we present a male infant with CHI who experienced persistent hypoglycemia even after undergoing a near-total pancreatectomy and despite multiple medical treatments. The infant's hypoglycemic episodes were successfully controlled by adding maltodextrin to his diet.
Novel mutation of the electron transferring flavoprotein dehydrogenase (ETFDH) gene in the isolated myopathic form of coenzyme q10 deficiency
(Medecine Et Hygiene, 2015-01-01) Görükmez, O.; Görükmez, Orhan; Sağ, Şebnem Özemri; Erdöl, Şahin; Sağlam, Halil; Yakut, T.; Görükmez, Orhan; ÖZEMRİ SAĞ, ŞEBNEM; ERDÖL, ŞAHİN; SAĞLAM, HALİL; Yakut, T.; Uludağ Üniversitesi/Tıp Fakültesi/Pediatri Bölümü; 0000-0002-9241-0896; 0000-0002-9241-0896; 0000-0002-6598-8262; GLN-8241-2022; AFZ-0764-2022; AAH-8355-2021; C-7392-2019
Pediatric patients with pheochromocytoma: Experience of a tertiary health center
(Wiley, 2015-10-01) Eren, Erdal; Sağlam, Halil; Çalışkan, Yusuf; Kiriştioğlu, İrfan; Tarım, Ömer; EREN, ERDAL; SAĞLAM, HALİL; Çalışkan, Yusuf; KIRIŞTIOĞLU, İRFAN; TARIM, ÖMER FARUK; Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Endokrinolojisi Anabilim Dalı.; 0000-0002-1684-1053; 0000-0002-6598-8262; 0000-0002-5322-5508; JPK-3909-2023; C-7392-2019; GLN-8241-2022; JPO-3445-2023; ITE-9417-2023; CCU-8073-2022
BackgroundThe aim of this retrospective study was to investigate pheochromocytoma (pheo), which is a rare endocrine tumor in the pediatric population.MethodsThe medical records of five children with pheo were studied. The age, gender, clinical presentation, family history, physical findings, coexisting pathology, laboratory evaluation, surgical treatment, and postoperative course were investigated.ResultsThe patients were four girls and one boy with a mean age of 13.2years (range, 9.57-15.95years). None of the patients had paroxysmal hypertension and one had normal blood pressure. No malign pheo was identified. Mean height and weight standard deviation scores (SDS), body mass index (BMI), and BMI SDS were -0.24, 0.04, 20.9kg/m(2), and 0.20 at the time of diagnosis, and 0.03, 0.43, 23.8kg/m(2) and 0.49 1year after operation, respectively. BMI increased significantly after operation. Three patients had normal epinephrine and metanephrine, but elevated norepinephrine and normetanephrine on 24h urine. Vanillylmandelic acid on 24h urine sample was elevated in all patients. Ultrasonography failed to visualize tumors in two patients with bilateral pheo. One patient had postoperative severe hypotension. Insulin resistance associated with severe acanthosis nigricans observed in one patient regressed postoperatively.ConclusionsPheo in children may present with different symptoms and findings. Decreased catecholamine in the postoperative period may lead to weight gain.
Propionic acidemia: Case report
(Galenos Yayincilik, 2007-06-01) SAĞLAM, HALİL; Kamber, Kadri; ERDÖL, ŞAHİN; Erdöl, Şahin; Karalı, Yasin; Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı.; 0000-0003-0710-5422; 0000-0002-6598-8262; C-7392-2019; GLN-8241-2022
A three and a half-month-old boy was referred to us to be investigated due to pancytopenia. He had vomiting, lethargy and failure to thrive. It was reported that seventy two hours after an uneventful labor, he developed feeding difficulty, vomiting, hypotonia and lethargy; and he was hospitalized with an initial diagnosis of neonatal sepsis for 15 days. There was second degree consanguinity between his parents.On physical examination; he was hypotonic, lethargic, and tachypneic with a slight hepatomegaly of 2 cm. Neonatal reflexes were absent. During follow-up, he developed persistent myoclonic convulsions and metabolic acidosis. Quantitative plasma and cerebrospinal fluid glycine levels were increased. On tandem mass spectrometric study, C3-propionyl carnitin level was increased. Urine organic acid analysis revealed increased 3-OH propionic acid, 3-OH butyric acid, and propionylglycine levels. He was diagnosed as propionic acidemia and a protein restricted diet with oral L-carnitine and biotin supplementations were started.In our country, where the prevalence of consanguineous marriages is very high, inborn errors of metabolism should be strongly considered in all of those full term neonates and children who develop hypotonia, convulsions, lethargy and failure to thrive after an uneventful labor, as well as in those developing signs and symptoms of neonatal sepsis without any risk factors both in the baby and in the mother.
Results of thyroid function tests in premature infants
(Galenos Yayıncılık, 2016-04-01) Doğan, Pelin; Bağcı, Onur; Köksal, Nilgün; Özkan, Hilal; Varal, İpek Güney; Eren, Erdal; Canbolat, Cansu; Sağlam, Halil; Doğan, Pelin; Bağcı, Onur; Köksal, Nilgün; ÖZKAN, HİLAL; Varal, İpek Güney; EREN, ERDAL; Canbolat, Cansu; SAĞLAM, HALİL; Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı/Neonatoloji Bilim Dalı.; Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı/Endokrinoloji Bilim Dalı.; Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı.; 0000-0002-3298-066X; 0000-0002-1684-1053; 0000-0002-6598-8262; CNZ-3688-2022; KYP-5736-2024; JGS-7600-2023; JJY-3921-2023; AAI-5981-2020; JPK-3909-2023; CKF-4697-2022; C-7392-2019
Introduction: To determine the rate, etiology and morbidity association of disorders of thyroid function tests (TFTs) in premature babies.Materials and Methods: A total of 139 premature babies who were admitted to Uludag University Faculty of Medicine Hospital Neonatal Intensive Care Unit between January 2009 and January 2012 were included in this study. Prenatal, natal and postnatal characteristics along with TFTs results were recorded. TFTs were performed for all patients in the first and third weeks of life. Thyroid stimulation hormone (TSH) values of > 10 IU/L was considered elevated. Free T3 and T4 levels were evaluated according to laboratory cut-off values. Weight, height and head circumference values of all individuals with and without the diagnosis of hypothyroidism on the 6th, 12th, and 18th months of their polyclinic follow-ups.Results: Abnormal TFTs were detected in 41 (24%) patients. Twenty two patients (53%) had transient TSH elevation, 9 (22%) had primary hypothyroidism, 9 (22%) had non-thyroidal disease and 1 (2.4%) had transient hypothyroxinemia. Among morbidities, respiratory distress syndrome rate was found to be significantly higher in patients with thyroid function disorders (p=0.007). The rate of thyroid function disorders in patients with mothers with hypothyroidism was significantly more frequent compared to patients without maternal hypothyroidism (p=0.049). The mean head circumference in 18 month was significantly lower in patients with abnormal TFTs (p=0.047).Conclusions: Thyroid function disorders are common morbidities in premature babies and are important for neuromotor development. Maternal thyroid function disorder can lead to impairment of TFTs in infants. Thyroid function tests should be performed in all premature babies and hypotyhroidism should be treated. Avoidance of iodine exposure in premature infants can reduce the rate of abnormal TFTs and transient hypothyroidism.
Retrospective evaluation of patients diagnosed with osteogenesis imperfecta
(Galenos Yayıncılık, 2017-04-01) Aslan, Mustafa Törehan; Eren, Erdal; Sağlam, Halil; Tarım, Ömer; Aslan, Mustafa Törehan; EREN, ERDAL; SAĞLAM, HALİL; TARIM, ÖMER FARUK; Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı.; Uludağ Üniversitesi/Tıp Fakültesi/Pediatri Anabilim Dalı/Çocuk Endokrinolojisi Bilim Dalı.; 0000-0002-1684-1053; 0000-0002-6598-8262; 0000-0002-5322-5508; GLN-8241-2022; C-7392-2019; AAS-8336-2020; JPK-3909-2023
Introduction: Osteogenesis imperfecta (OI) is a hereditary disease that impairs the quality of life by frequent bone fractures. The objective of our study is to retrospectively evaluate patients diagnosed with OI and to come up with helpful data that will assist developing new diagnosis and treatment protocols.Materials and Methods: Twenty-eight cases with OI who were followed-up in our clinic were retrospectively evaluated. Clinical classification of OI was done. Age, sex, and oxologic data were evaluated. Height, weight and body mass index (BMI) data was given as standard deviation score (SDS). Family history of fracture and consanguineous marriage was sought. Blue sclera and presence of deformity was evaluated on physical examination.Results: Out of the 28 cases in our study, 14 (50%) were boys, 14 (50%) were girls, and mean age was 7.48 +/- 5.09 years. Mean age of diagnosis was 25.59 +/- 39.59 months. Ten cases (47.6%) had OI, and 7 cases (25%) had consanguineous marriage in their family history. The cases were separated into autosomal dominant 4 clinical types according to Sillence classification as follows; 13 cases (46.4%) type 1, 10 cases (35.7%) type 3, and 5 cases (17.9%) type 4. The mean average basal dual energy X-ray absorptiometry Z score, mean height SDS, mean weight SDS and BMI SDS significantly increased for the cases after treatment (p<0.001).Conclusions: Treatment of OI with pamidronate was observed to increase bone mineral density, decrease number of fractures and pain, and improve the patient's quality of life with inreasing mobility. Pamidronate is one of the most effective treatments of OI until a more effective treatment is found. On the other hand, since the long-term side effect of pamidronate on bones is not well-known, we think that randomised controlled studies still need to be done to determine the optimal time, interval and dose for bisphosphonate use.