Bisphosphonates have been used in the treatment of vitamin D intoxication (VDI) after failure of ... more Bisphosphonates have been used in the treatment of vitamin D intoxication (VDI) after failure of conventional therapy including prednisolone. Safety concerns restrict the use of bisphosphonates as the first-line therapy for VDI in children. The aim of our study was to evaluate the efficacy and safety of pamidronate in comparison with prednisolone in children with VDI. We reviewed the hospital records of children consecutively diagnosed with VDI at two medical centers over the last 15-year period. The study group contained 21 children (aged 0.3-4.2 years) who were treated with prednisolone and/or bisphosphonates. Pamidronate(n=18) or alendronate(n=3) were administered in 6 patients after unsuccessful prednisolone treatment and in 15 patients from baseline. Initial serum levels of calcium and 25-hydroxyvitamin D were 16.1±1.9 mg/dl and 493±219ng/ml, respectively. The average time to reach normocalcemia in the patients treated with pamidronate, alendronate and prednisolone was 3(2-12), 4(3-6) and 17(12-26) days, respectively (p=0.013). Pamidronate treatment compared with prednisolone shortened about five times the duration of hospital stay. Although three patients initially treated with prednisolone have developed nephrocalcinosis, it has not occurred in any patient treated with bisphosphonates from baseline. Apart from transient fever and moderate hypophosphatemia, no side effect attributable to bisphosphonate treatment was observed. Pamidronate is an efficient and safe agent in treatment of children with VDI. Pamidronate use significantly shortens the duration of treatment, and thereby may prevent the development of nephrocalcinosis. Instead of prednisolone, pamidronate should be used together with hydration and furosemide as the first-line therapy for VDI.
Hemangioendotheliomas can express type 3 iodothyronine deiodinase and cause severe hypothyroidism... more Hemangioendotheliomas can express type 3 iodothyronine deiodinase and cause severe hypothyroidism. The risk of congenital malformations such as vertebral and cardiac abnormalities in infants of diabetic mothers is higher than babies of healthy women. Here we report an infant of a diabetic mother with hypothyroidism caused by liver hemangioendothelioma. Consumptive hypothyroidism should be an indicator to search for a vascular tumor in infants. Supranormal doses of L-thyroxine might be required for normalization of thyroid function until the tumor involutes or is resected.
Journal of pediatric endocrinology & metabolism : JPEM, Jan 22, 2015
We aimed at evaluating the urinary levels of kidney injury molecule-1 ( KIM-1) and neutrophil gel... more We aimed at evaluating the urinary levels of kidney injury molecule-1 ( KIM-1) and neutrophil gelatinase associated lipocalin (NGAL), and the relationship between these markers and clinical and laboratory variables in normoalbuminuric children with type 1 diabetes (T1D). The study group consisted of 60 (F/M: 28/32) children with T1D with a median age of 13 (min: 7.1-max: 17.9) years and a mean HbA1c of 8.6%. The average period of treatment was 6.8±2.2 years. The control group consisted of 60 healthy children [(F/M: 32/28); median age: 13.6 (min: 6.9-max: 17.9) years]. Urinary KIM-1 and NGAL levels were significantly elevated in the diabetic group (KIM-1: 0.50±0.34 ng/mg-cr; NGAL: 33±31 ng/mg-cr) compared with the nondiabetic control subjects (KIM-1: 0.26±0.25 ng/mg-cr, NGAL 13.3±14.5 ng/mg-cr) (p<0.001). No significant associations were observed between NGAL or KIM-1 and the duration of diabetes and HbA1c levels. NGAL was found to be weakly correlated with KIM-1 (p<0.005, r=0....
Van Wyk-Grumbach syndrome is a rare cause of precocious puberty due to hypothyroidism. We report ... more Van Wyk-Grumbach syndrome is a rare cause of precocious puberty due to hypothyroidism. We report a case of Van Wyk-Grumbach syndrome in a 4.3-year-old female patient with Down syndrome. She was investigated for hematuria for three months before she was referred to our clinic. Physical examination revealed typical morphologic features of Down syndrome and hypothyroidism. Pubertal development stages were: breast at stage III and pubic hair at stage I. In luteinizing hormone releasing hormone (LHRH) stimulation test, peak LH level remained less than 0.1 mIU/ml. Serum estradiol level was 117.7 pg/ml, which was higher than normal for her age (normal range: 2-15 pg/ml). The pelvic ultrasonographic evaluation revealed bilateral multicystic enlarged ovaries. Serum thyroid stimulating hormone (TSH) concentration was higher than 500 microIU/ml and free thyroxin (FT4) and free triiodothyronine (FT3) levels were as low as 0.4 ng/dl (0.7-1.48) and 1.0 pg/ml (1.71-3.71), respectively. L-thyroxin ...
Journal of clinical research in pediatric endocrinology, Jan 14, 2018
Congenital adrenal hyperplasia (CAH) is the most common form of primary adrenal insufficiency in ... more Congenital adrenal hyperplasia (CAH) is the most common form of primary adrenal insufficiency in children. Neonatal screening for CAH is effective in detecting the salt-wasting form and reducing mortality. To estimate incidence of CAH in Turkey and to assess the characteristics and efficacy of the adopted newborn CAH screening strategy. A pilot newborn CAH screening study was carried out under the authority of Turkish Directorate of Public Health. Newborn babies ≥32 gestational weeks and ≥1500 gr birth weight from four cities between March 27- September 15, 2017 were included. Screening protocol included one sample two-tier testing. In the first step, 17α-hydroxyprogesterone (17-OHP) was measured by fluoroimmunoassay in dried blood spots obtained at 3-5th days of life. The cases with positive initial screening were tested by steroid profiling in dried blood spots using liquid chromatography-tandem mass spectrometry method to measure 17-OHP, 21-deoxycortisol, cortisol, 11-deoxycortis...
Clinical pediatric endocrinology : case reports and clinical investigations : official journal of the Japanese Society for Pediatric Endocrinology, 2017
A 20-mo-old girl was brought to our department by her mother because of breast enlargement. She w... more A 20-mo-old girl was brought to our department by her mother because of breast enlargement. She was diagnosed with premature thelarche. One month later, she returned to our hospital with a complaint of vaginal bleeding. During the subsequent 6 mo, her vaginal bleeding recurred every month while her breast development disappeared. We performed laboratory tests and imaging. At the end of 6 mo, we realized that her mother's menstrual bleeding and the patient's blood staining were concurrent. The mother confessed applying her vaginal flow to her daughter's underwear. Factitious disorder should be included in the differential diagnosis of unexplained vaginal bleeding in childhood.
Pellagra is clinically manifested by a photosensitive dermatitis, diarrhea, and dementia. The ful... more Pellagra is clinically manifested by a photosensitive dermatitis, diarrhea, and dementia. The full triad of symptoms is usually not well developed in infants and children. We report a case of a 14-year-old boy with classic symptoms of pellagra. All his symptoms responded to treatment with nicotinic acid.
Bisphosphonates have been used in the treatment of vitamin D intoxication (VDI) after failure of ... more Bisphosphonates have been used in the treatment of vitamin D intoxication (VDI) after failure of conventional therapy including prednisolone. Safety concerns restrict the use of bisphosphonates as the first-line therapy for VDI in children. The aim of our study was to evaluate the efficacy and safety of pamidronate in comparison with prednisolone in children with VDI. We reviewed the hospital records of children consecutively diagnosed with VDI at two medical centers over the last 15-year period. The study group contained 21 children (aged 0.3-4.2 years) who were treated with prednisolone and/or bisphosphonates. Pamidronate(n=18) or alendronate(n=3) were administered in 6 patients after unsuccessful prednisolone treatment and in 15 patients from baseline. Initial serum levels of calcium and 25-hydroxyvitamin D were 16.1±1.9 mg/dl and 493±219ng/ml, respectively. The average time to reach normocalcemia in the patients treated with pamidronate, alendronate and prednisolone was 3(2-12), 4(3-6) and 17(12-26) days, respectively (p=0.013). Pamidronate treatment compared with prednisolone shortened about five times the duration of hospital stay. Although three patients initially treated with prednisolone have developed nephrocalcinosis, it has not occurred in any patient treated with bisphosphonates from baseline. Apart from transient fever and moderate hypophosphatemia, no side effect attributable to bisphosphonate treatment was observed. Pamidronate is an efficient and safe agent in treatment of children with VDI. Pamidronate use significantly shortens the duration of treatment, and thereby may prevent the development of nephrocalcinosis. Instead of prednisolone, pamidronate should be used together with hydration and furosemide as the first-line therapy for VDI.
Hemangioendotheliomas can express type 3 iodothyronine deiodinase and cause severe hypothyroidism... more Hemangioendotheliomas can express type 3 iodothyronine deiodinase and cause severe hypothyroidism. The risk of congenital malformations such as vertebral and cardiac abnormalities in infants of diabetic mothers is higher than babies of healthy women. Here we report an infant of a diabetic mother with hypothyroidism caused by liver hemangioendothelioma. Consumptive hypothyroidism should be an indicator to search for a vascular tumor in infants. Supranormal doses of L-thyroxine might be required for normalization of thyroid function until the tumor involutes or is resected.
Journal of pediatric endocrinology & metabolism : JPEM, Jan 22, 2015
We aimed at evaluating the urinary levels of kidney injury molecule-1 ( KIM-1) and neutrophil gel... more We aimed at evaluating the urinary levels of kidney injury molecule-1 ( KIM-1) and neutrophil gelatinase associated lipocalin (NGAL), and the relationship between these markers and clinical and laboratory variables in normoalbuminuric children with type 1 diabetes (T1D). The study group consisted of 60 (F/M: 28/32) children with T1D with a median age of 13 (min: 7.1-max: 17.9) years and a mean HbA1c of 8.6%. The average period of treatment was 6.8±2.2 years. The control group consisted of 60 healthy children [(F/M: 32/28); median age: 13.6 (min: 6.9-max: 17.9) years]. Urinary KIM-1 and NGAL levels were significantly elevated in the diabetic group (KIM-1: 0.50±0.34 ng/mg-cr; NGAL: 33±31 ng/mg-cr) compared with the nondiabetic control subjects (KIM-1: 0.26±0.25 ng/mg-cr, NGAL 13.3±14.5 ng/mg-cr) (p<0.001). No significant associations were observed between NGAL or KIM-1 and the duration of diabetes and HbA1c levels. NGAL was found to be weakly correlated with KIM-1 (p<0.005, r=0....
Van Wyk-Grumbach syndrome is a rare cause of precocious puberty due to hypothyroidism. We report ... more Van Wyk-Grumbach syndrome is a rare cause of precocious puberty due to hypothyroidism. We report a case of Van Wyk-Grumbach syndrome in a 4.3-year-old female patient with Down syndrome. She was investigated for hematuria for three months before she was referred to our clinic. Physical examination revealed typical morphologic features of Down syndrome and hypothyroidism. Pubertal development stages were: breast at stage III and pubic hair at stage I. In luteinizing hormone releasing hormone (LHRH) stimulation test, peak LH level remained less than 0.1 mIU/ml. Serum estradiol level was 117.7 pg/ml, which was higher than normal for her age (normal range: 2-15 pg/ml). The pelvic ultrasonographic evaluation revealed bilateral multicystic enlarged ovaries. Serum thyroid stimulating hormone (TSH) concentration was higher than 500 microIU/ml and free thyroxin (FT4) and free triiodothyronine (FT3) levels were as low as 0.4 ng/dl (0.7-1.48) and 1.0 pg/ml (1.71-3.71), respectively. L-thyroxin ...
Journal of clinical research in pediatric endocrinology, Jan 14, 2018
Congenital adrenal hyperplasia (CAH) is the most common form of primary adrenal insufficiency in ... more Congenital adrenal hyperplasia (CAH) is the most common form of primary adrenal insufficiency in children. Neonatal screening for CAH is effective in detecting the salt-wasting form and reducing mortality. To estimate incidence of CAH in Turkey and to assess the characteristics and efficacy of the adopted newborn CAH screening strategy. A pilot newborn CAH screening study was carried out under the authority of Turkish Directorate of Public Health. Newborn babies ≥32 gestational weeks and ≥1500 gr birth weight from four cities between March 27- September 15, 2017 were included. Screening protocol included one sample two-tier testing. In the first step, 17α-hydroxyprogesterone (17-OHP) was measured by fluoroimmunoassay in dried blood spots obtained at 3-5th days of life. The cases with positive initial screening were tested by steroid profiling in dried blood spots using liquid chromatography-tandem mass spectrometry method to measure 17-OHP, 21-deoxycortisol, cortisol, 11-deoxycortis...
Clinical pediatric endocrinology : case reports and clinical investigations : official journal of the Japanese Society for Pediatric Endocrinology, 2017
A 20-mo-old girl was brought to our department by her mother because of breast enlargement. She w... more A 20-mo-old girl was brought to our department by her mother because of breast enlargement. She was diagnosed with premature thelarche. One month later, she returned to our hospital with a complaint of vaginal bleeding. During the subsequent 6 mo, her vaginal bleeding recurred every month while her breast development disappeared. We performed laboratory tests and imaging. At the end of 6 mo, we realized that her mother's menstrual bleeding and the patient's blood staining were concurrent. The mother confessed applying her vaginal flow to her daughter's underwear. Factitious disorder should be included in the differential diagnosis of unexplained vaginal bleeding in childhood.
Pellagra is clinically manifested by a photosensitive dermatitis, diarrhea, and dementia. The ful... more Pellagra is clinically manifested by a photosensitive dermatitis, diarrhea, and dementia. The full triad of symptoms is usually not well developed in infants and children. We report a case of a 14-year-old boy with classic symptoms of pellagra. All his symptoms responded to treatment with nicotinic acid.
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