Mustafa Akcakus | Akdeniz University (original) (raw)

Papers by Mustafa Akcakus

Journal of Pediatric Hematology Oncology, 2004

Nonketotic hyperosmolar coma is uncommon in children. Splenic rupture in congenital afibrinogenem... more Nonketotic hyperosmolar coma is uncommon in children. Splenic rupture in congenital afibrinogenemia is also a rare event. The authors described a 5-year-old girl with congenital afibrinogenemia who presented with nonketotic hyperosmolar coma associated with spontaneous splenic rupture. Management consisted of correction of the nonketotic hyperosmolar condition and increasing fibrinogen concentration by blood products, followed by splenectomy, resulting in the survival of the patient.

Paediatrics & child health, 2003

Joubert syndrome is an autosomal recessive disorder that is characterized by a variable combinati... more Joubert syndrome is an autosomal recessive disorder that is characterized by a variable combination of central nervous system, respiratory and eye anomalies. It is a syndrome with a variable phenotype: partial or complete absence of the cerebellar vermis is seen in all patients, while other cardinal findings include episodic tachypnea and apnea in the neonatal period, jerky eye movements, hypotonia, severe mental handicap, developmental delay, ataxia and impaired equilibrium. Even within sibships the phenotype may vary, making it difficult to establish the exact clinical diagnostic boundaries of Joubert syndrome. A case of Joubert syndrome in a newborn is reported and the importance of recognizing the syndrome in the neonatal period so that specific and effective supportive measures can be started as soon as possible is stressed.

The Turkish journal of pediatrics

Brucellosis is a zoonotic infection, transmitted to humans primarily by consumption of unpasteuri... more Brucellosis is a zoonotic infection, transmitted to humans primarily by consumption of unpasteurized milk and milk products. Transmission by blood transfusion is possible but very unusual. Herewith we present two newborns with positive blood cultures for Brucella melitensis after exchange transfusions. However, the standard tube agglutination titers against Brucella were not elevated and the newborns did not develop brucellosis. It is suggested that, in areas endemic for brucellosis, blood donors should be questioned about symptoms of brucellosis, and if suspected, serological tests for brucellosis should be indicated before blood transfusion. At the same time, the prevalence of the disease among animals should be reduced with effective animal disease control programs.

The Turkish journal of pediatrics

Asymmetric crying facies is caused by congenital hypoplasia or agenesis of the depressor anguli o... more Asymmetric crying facies is caused by congenital hypoplasia or agenesis of the depressor anguli oris muscle. Associations of this facial defect with major congenital anomalies have been reported, most commonly in the cardiovascular system and less frequently involving the genitourinary, musculoskeletal, cervicofacial, respiratory, and rarely, the endocrine system. CATCH 22 is a medical acronym for cardiac defects, abnormal facies, thymic hypoplasia, cleft palate and hypocalcemia and a variable deletion on chromosome 22q11. The deletion within chromosme region of 22q11 may occur in patients with dysmorphologic and cardiological syndromes: DiGeorge syndrome, velo-cardiofacial syndrome and conotruncal anomaly face syndrome. We report a newborn infant who had asymmetric crying facies associated with congenital hypoparathyroidism, severe neonatal hypocalcemia and tetralogy of Fallot. Genetic confirmation of chromosome 22q11 deletion was made.

Journal of Clinical Research in Pediatric Endocrinology, 2009

Ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome is characterized by ectodermal dysplasi... more Ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome is characterized by ectodermal dysplasia, ectrodactyly and facial clefting with multiple congenital anomalies such as urinary tract anomaly, lacrimal duct obstruction, and hearing loss. This syndrome is a rare disease transmitted by autosomal dominant inheritance with variable penetrance. Clinical expression is variable. In EEC syndrome with midline defect hypothalamo-pituitary endocrinopathy is expected, however hormonal disorders in EEC syndrome have rarely been reported. We present two patients with EEC syndrome associated with hypothalamo-pituitary insufficiency.

Pediatrics International, 2002

Background : Septic arthritis is an uncommon, but serious disorder in neonates. Most patients sur... more Background : Septic arthritis is an uncommon, but serious disorder in neonates. Most patients survive with permanent handicaps. Due to the rarity of this condition in neonates and paucity of signs and symptoms, the diagnosis of septic arthritis in newborns is more difficult than in older children. Methods : Septic arthritis or suppurative arthritis is an infection of the joint by a variety of microorganisms, including bacteria, viruses, mycobacteria and fungi. Purulent synovial fluid, positive culture and positive Gram stain were accepted as a gold standard for exact diagnosis. Fourteen neonates who were followed-up in a neonatal intensive care unit, with septic arthritis, were included in a study based on a review of medical reports and a long-term clinical and radiological follow-up. Clinical symptoms, bacteriology, risk factors and outcomes are discussed. Results : Staphylococcus aureus was the predominant causative organism. Risk factors for septic arthritis were prematurity (4/14), umbilical catheterization or venous catheterization (3/14), sepsis (3/14), perinatal asphyxia (2/14) and difficult birth (1/14). All cases of septic arthritis in neonates were improved without squealae except in two patients. One patient died and one patient had severe squealae. In these two patients, the duration of disease from clinical onset to initiation of therapy was long. Conclusion : The most important prognostic factor in predicting a favorable outcome in neonatal septic arthritis is early diagnosis and therapy. When appropriate treatment is delayed, catastrophic sequelae are inevitable. Early diagnosis of the condition and rapid removal of pus are mandatory for the survival of the joint. Long-term follow-up may reveal effects of epiphyseal damage, early degenerative changes and limitation of the range of motion.

Hemangioblastomas (HBLs) comprise approximately 2% of all primary central nervous system (CNS) tu... more Hemangioblastomas (HBLs) comprise approximately 2% of all primary central nervous system (CNS) tumors. Although histological features of this rare tumor are generally benign, its outcome is often unfavorable due to high risk of recurrence and multifocal localization. HBLs can be detected as sporadic or associated with Von Hippel-Lindau disease. Diffuse neonatal hemangiomatosis (DNH) presents with multiple, progressive, rapidly growing cutaneous hemangiomas associated with widespread visceral hemangiomas in the liver, lungs, gastrointestinal tract, brain, and meninges. DNH with predominant CNS involvement is rarely reported. Herein, we present a neonatal case of cerebellar HBL associated with DNH. A 5-day-old male baby was referred with complaints of multiple cutaneous lesions. Purple papules were noted on the trunk, extremities, and the head. Thoracic magnetic resonance imaging demonstrated multiple hyperintense lesions on the chest wall and apex of the right lung. On MRI, a 3×2-cm mass lesion in the right cerebellar hemisphere was detected. Total resection of the mass and ventriculoperitoneal shunting was performed. Histopathologic examination confirmed the diagnosis of HBL. Steroid therapy was administered for disseminated hemangiomatosis, and the lesions showed regression; the patient showed good clinical recovery. The parents refused further treatment, and he was out of our control when he was 9 months old. According to our knowledge, the presented newborn is the second case of cerebellar HBL associated with diffuse skin and visceral hemangiomas in the English medical literature. Clinicians must be vigilant about the predictive value of visceral and/or cutaneous hemangioma for an associated intracranial HBL.

Pacing and clinical electrophysiology : PACE, 2003

Naxos disease is a recessively inherited arrhythmogenic right ventricular cardiomyopathy in which... more Naxos disease is a recessively inherited arrhythmogenic right ventricular cardiomyopathy in which the cardiac phenotype is associated with palmoplantar keratoderma and woolly hair. This disease is a heart muscle disorder causing life-threatening ventricular arrhythmias, heart failure, and sudden cardiac death. The pathological hallmark of the disease is the progressive replacement of myocardial cells by fat and fibrous tissue. It appears in families descending from the Hellenic island of Naxos. We presented a 13-year-old Turkish boy with Naxos disease associated with ventricular tachycardia because of its rarity, and reviewed the literature.

Pediatrics International, 2003

... Neonatal uterine prolapse. Mustafa Akcakus 1 ,; Tamer Gunes 1 ,; Selim Kurtoglu 1 ,; Neside C... more ... Neonatal uterine prolapse. Mustafa Akcakus 1 ,; Tamer Gunes 1 ,; Selim Kurtoglu 1 ,; Neside Cetin 1 ,; Aysenur Pac 1 ,; Hamit Okur 2. Article first published online: 26 JUN 2003. DOI: 10.1046/j.1442-200X.2003.01733.x. Issue. Pediatrics International. ...

Clinical pediatrics, 2004

... Mustafa Akcakus, MD1 Tahir Patiroglu, MD2 Kemal Deniz, MD2 Neside Cetin, MD1 Mehmet Akif Ozde... more ... Mustafa Akcakus, MD1 Tahir Patiroglu, MD2 Kemal Deniz, MD2 Neside Cetin, MD1 Mehmet Akif Ozdemir, MD3 Mehtap Kala, MD2 ... infiltrates (leukemia cutis) in ad-dition to the characteristic signs of congenital leukemia.3 Leuk-emia cutis may be the first mani-festation of ...

Journal of Clinical Research in Pediatric Endocrinology, 2010

Neonatal ovarian cysts (NOC) are usually self-limiting structures. However, large or complex cyst... more Neonatal ovarian cysts (NOC) are usually self-limiting structures. However, large or complex cysts may lead to severe complications. A standard guide to management, treatment and follow-up of NOC is not yet available. The aim of this study was to evaluate retrospectively the records of NOC patients from two medical centers. A total of 20 newborns with NOC were included in the study. The size and localization of the cyst, the age, the signs and symptoms at presentation, and the possible maternal and fetal-neonatal etiologic factors were recorded. Follow-up procedures and treatment modalities were evaluated. The mean age at diagnosis was 34 gestational weeks. The cysts (mean size 53±15 mm) were predominantly in the right ovary (75%) and were evaluated as large cysts in 16 (80%) of the patients. In 5 of the patients with large cysts and in 1 of the 4 patients with small cysts, the cysts were evaluated as complex cysts. Torsion of the ovary was detected in five (25%) cases and these cases were treated surgically. Patients with simple cysts were closely followed by ultrasonography until the cysts disappeared. To date, there is no precise guide for the monitoring and treatment of NOCs. Surgical treatment should always be performed in a way to protect the ovaries and to ensure future fertility. In our NOC series, it has been possible to apply a non-invasive follow-up program and minimally invasive surgical procedures.

Tohoku Journal of Experimental Medicine, 2002

Atherosclerosis is an important cause of cardiovascular morbidity and mortality in recent years. ... more Atherosclerosis is an important cause of cardiovascular morbidity and mortality in recent years. Hyperhomocysteinemia is recognized as an independent risk factor for premature atherosclerosis and venous thrombosis. It is suggested that administration of folic acid, vitamin B6 and vitamin B12 may decrease homocysteine levels. In our study, we induced hyperhomocysteinemia in rabbits by giving methionine and studied the effects of folic acid, vitamin B6 and vitamin B12 on homocysteine levels. A total of 40 (20 female, 20 male New Zealand rabbits) were divided into four groups, each consisting of 10 rabbits. Methionine (100 mg/kg/day), methionine (100 mg/kg/day) plus vitamin B6 (30 mg/kg/day), methionine (100 mg/kg/day) plus vitamin B12 (80 mg/kg/day) and methionine (100 mg/kg/day) plus folic acid (20 mg/kg/day) were given to the first, second, third and forth groups respectively. These rabbits were followed up for two months. We studied homocysteine levels on the 0, 20th, 40th and 60th days in all groups. In rabbits we induced hyperhomocysteinemia by giving methionine for 2 months. The decreases of homocysteine levels in the forth group were significant with respect to the second and third groups. Folic acid supplementation clearly resulted in a reduction of plasma homocysteine levels, whereas vitamin B12 was little effective and vitamin B6 failed to show an effect. We conclude that even folic acid treatment alone may be sufficient for decreasing negative effects of homocysteine.

European Journal of Pediatrics, 2007

Macrosomia is associated with alterations in lipoprotein composition and concentration at birth. ... more Macrosomia is associated with alterations in lipoprotein composition and concentration at birth. Exposure to diabetes in utero has been established as a significant risk factor for some of the components of metabolic syndrome. The aim of this study was to investigate the effect of macrosomia on lipid metabolism, aortic intima-media thickness (aIMT) and subsequent atherogenic risk in newborn infants. Aortic

Erciyes Tıp Dergisi/Erciyes Medical Journal, 2012

International Journal of Dermatology, 2006

ABSTRACT

TURKISH JOURNAL OF MEDICAL SCIENCES, 2014

There are close interactions among the developing oral cavity, pituitary gland, and central nervo... more There are close interactions among the developing oral cavity, pituitary gland, and central nervous system (CNS) in early embryonic life. In this study we aimed to screen endocrine abnormalities in patients with orofacial clefts in the neonatal period. Thirty-one patients with isolated orofacial median clefts wereincluded in the study. Pituitary, thyroid, and adrenal hormones were measured at the first week and remeasured in the third or fourth weeks. Imaging studies were done for detection of CNS anomalies in all patients. Endocrine abnormality was detected in 22 (70.9%) patients. The number of patients with single and multiple endocrine abnormalities were 13 (41.9%) and 9 (29%), respectively. Thyroid hormone-related disorders were detected in 10 (32.3%) patients. Growth hormone deficiency was detected in 4 (12.9%) patients. Adrenocorticotrophic hormone and/or glucocorticoid deficiency was detected in 5 (16.1%) patients. Neonatal hypoglycemia due to endocrinological abnormalities was detected in 6 (19.4%) patients. Defected mini-puberty was seen in 2 (15.4%) patients. There was no relationship between the types of orofacial cleft and endocrine abnormalities. Endocrinological evaluation of the patients with orofacial clefts in the neonatal period is a worthwhile endeavor to detect hormone deficiencies regardless of the type of the cleft.

Pediatric Research, 2007

Neonates with intrauterine growth restriction (IUGR) are associated with reduced concentrations o... more Neonates with intrauterine growth restriction (IUGR) are associated with reduced concentrations of IGF-I that might contribute to arterial wall thickening. Direct atherogenic effects of leptin have been described. We aimed to investigate the relationship among abdominal aortic intima-media thickness (aIMT), serum IGF-I, IGF binding protein-3, and leptin levels in neonates with IUGR. Abdominal aIMT was measured in 40 term neonates with IUGR and in 40 controls. Mean aIMT was significantly greater in neonates with IUGR (0.45 +/- 0.03 mm) than in controls (0.39 +/- 0.04 mm, p < 0.0001). Serum IGF-I and leptin levels were lower in neonates with IUGR than in controls. There was a significant positive correlation between aIMT and gestational age, whereas a significant negative correlation was determined between aIMT and IGF-I in the IUGR neonates. For aIMT, significant associations included serum IGF-I level (beta = -0.406, p = 0.006) and gestational age (beta = 0.331, p = 0.022) in a multiple stepwise linear regression analysis. In control neonates, serum IGF-I levels were negatively related to aIMT (beta = -0.750, p < 0.001). Neonates with IUGR have significant aIMT with decreased IGF-I. IGF-I levels determine aIMT not only in neonates with IUGR but also in healthy controls.

Pediatric Diabetes, 2002

Thiamine-responsive megaloblastic anemia syndrome (TRMA)

Pediatric Dermatology, 2003

Collodion baby is a distinct subset of neonatal erythroderma that can be a clinical marker for a ... more Collodion baby is a distinct subset of neonatal erythroderma that can be a clinical marker for a variety of underlying abnormalities. The phenotype includes parchment-like hyperkeratosis, pseudocontractures, ectropion, eclabium, absence of eyebrows, and sparse hair. Asymmetric crying facies is caused by congenital hypoplasia or agenesis of the depressor anguli oris muscle. Associations of this facial defect with major congenital anomalies have been reported, most commonly in the cardiovascular system, less frequently involving the genitourinary, musculoskeletal, cervicofacial, and respiratory systems, and rarely the endocrine system. We report a newborn with a collodion membrane and asymmetric crying facies. To the best of our knowledge, this association has not been previously published.

Pediatric Dermatology, 2003

Pseudomonas aeruginosa bacteremia or sepsis often occurs in hospitals, affecting mainly children ... more Pseudomonas aeruginosa bacteremia or sepsis often occurs in hospitals, affecting mainly children with underlying disease. Ecthyma gangrenosum is classically considered a pathognomonic sign of sepsis by P. aeruginosa. The harlequin baby, a severe variant of ichthyosis, occurs rarely, and these infants are at high risk of cutaneous infections and sepsis. We herein report a harlequin baby who developed ecthyma gangrenosum.

Journal of Pediatric Hematology Oncology, 2004

Nonketotic hyperosmolar coma is uncommon in children. Splenic rupture in congenital afibrinogenem... more Nonketotic hyperosmolar coma is uncommon in children. Splenic rupture in congenital afibrinogenemia is also a rare event. The authors described a 5-year-old girl with congenital afibrinogenemia who presented with nonketotic hyperosmolar coma associated with spontaneous splenic rupture. Management consisted of correction of the nonketotic hyperosmolar condition and increasing fibrinogen concentration by blood products, followed by splenectomy, resulting in the survival of the patient.

Paediatrics & child health, 2003

Joubert syndrome is an autosomal recessive disorder that is characterized by a variable combinati... more Joubert syndrome is an autosomal recessive disorder that is characterized by a variable combination of central nervous system, respiratory and eye anomalies. It is a syndrome with a variable phenotype: partial or complete absence of the cerebellar vermis is seen in all patients, while other cardinal findings include episodic tachypnea and apnea in the neonatal period, jerky eye movements, hypotonia, severe mental handicap, developmental delay, ataxia and impaired equilibrium. Even within sibships the phenotype may vary, making it difficult to establish the exact clinical diagnostic boundaries of Joubert syndrome. A case of Joubert syndrome in a newborn is reported and the importance of recognizing the syndrome in the neonatal period so that specific and effective supportive measures can be started as soon as possible is stressed.

The Turkish journal of pediatrics

Brucellosis is a zoonotic infection, transmitted to humans primarily by consumption of unpasteuri... more Brucellosis is a zoonotic infection, transmitted to humans primarily by consumption of unpasteurized milk and milk products. Transmission by blood transfusion is possible but very unusual. Herewith we present two newborns with positive blood cultures for Brucella melitensis after exchange transfusions. However, the standard tube agglutination titers against Brucella were not elevated and the newborns did not develop brucellosis. It is suggested that, in areas endemic for brucellosis, blood donors should be questioned about symptoms of brucellosis, and if suspected, serological tests for brucellosis should be indicated before blood transfusion. At the same time, the prevalence of the disease among animals should be reduced with effective animal disease control programs.

The Turkish journal of pediatrics

Asymmetric crying facies is caused by congenital hypoplasia or agenesis of the depressor anguli o... more Asymmetric crying facies is caused by congenital hypoplasia or agenesis of the depressor anguli oris muscle. Associations of this facial defect with major congenital anomalies have been reported, most commonly in the cardiovascular system and less frequently involving the genitourinary, musculoskeletal, cervicofacial, respiratory, and rarely, the endocrine system. CATCH 22 is a medical acronym for cardiac defects, abnormal facies, thymic hypoplasia, cleft palate and hypocalcemia and a variable deletion on chromosome 22q11. The deletion within chromosme region of 22q11 may occur in patients with dysmorphologic and cardiological syndromes: DiGeorge syndrome, velo-cardiofacial syndrome and conotruncal anomaly face syndrome. We report a newborn infant who had asymmetric crying facies associated with congenital hypoparathyroidism, severe neonatal hypocalcemia and tetralogy of Fallot. Genetic confirmation of chromosome 22q11 deletion was made.

Journal of Clinical Research in Pediatric Endocrinology, 2009

Ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome is characterized by ectodermal dysplasi... more Ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome is characterized by ectodermal dysplasia, ectrodactyly and facial clefting with multiple congenital anomalies such as urinary tract anomaly, lacrimal duct obstruction, and hearing loss. This syndrome is a rare disease transmitted by autosomal dominant inheritance with variable penetrance. Clinical expression is variable. In EEC syndrome with midline defect hypothalamo-pituitary endocrinopathy is expected, however hormonal disorders in EEC syndrome have rarely been reported. We present two patients with EEC syndrome associated with hypothalamo-pituitary insufficiency.

Pediatrics International, 2002

Background : Septic arthritis is an uncommon, but serious disorder in neonates. Most patients sur... more Background : Septic arthritis is an uncommon, but serious disorder in neonates. Most patients survive with permanent handicaps. Due to the rarity of this condition in neonates and paucity of signs and symptoms, the diagnosis of septic arthritis in newborns is more difficult than in older children. Methods : Septic arthritis or suppurative arthritis is an infection of the joint by a variety of microorganisms, including bacteria, viruses, mycobacteria and fungi. Purulent synovial fluid, positive culture and positive Gram stain were accepted as a gold standard for exact diagnosis. Fourteen neonates who were followed-up in a neonatal intensive care unit, with septic arthritis, were included in a study based on a review of medical reports and a long-term clinical and radiological follow-up. Clinical symptoms, bacteriology, risk factors and outcomes are discussed. Results : Staphylococcus aureus was the predominant causative organism. Risk factors for septic arthritis were prematurity (4/14), umbilical catheterization or venous catheterization (3/14), sepsis (3/14), perinatal asphyxia (2/14) and difficult birth (1/14). All cases of septic arthritis in neonates were improved without squealae except in two patients. One patient died and one patient had severe squealae. In these two patients, the duration of disease from clinical onset to initiation of therapy was long. Conclusion : The most important prognostic factor in predicting a favorable outcome in neonatal septic arthritis is early diagnosis and therapy. When appropriate treatment is delayed, catastrophic sequelae are inevitable. Early diagnosis of the condition and rapid removal of pus are mandatory for the survival of the joint. Long-term follow-up may reveal effects of epiphyseal damage, early degenerative changes and limitation of the range of motion.

Hemangioblastomas (HBLs) comprise approximately 2% of all primary central nervous system (CNS) tu... more Hemangioblastomas (HBLs) comprise approximately 2% of all primary central nervous system (CNS) tumors. Although histological features of this rare tumor are generally benign, its outcome is often unfavorable due to high risk of recurrence and multifocal localization. HBLs can be detected as sporadic or associated with Von Hippel-Lindau disease. Diffuse neonatal hemangiomatosis (DNH) presents with multiple, progressive, rapidly growing cutaneous hemangiomas associated with widespread visceral hemangiomas in the liver, lungs, gastrointestinal tract, brain, and meninges. DNH with predominant CNS involvement is rarely reported. Herein, we present a neonatal case of cerebellar HBL associated with DNH. A 5-day-old male baby was referred with complaints of multiple cutaneous lesions. Purple papules were noted on the trunk, extremities, and the head. Thoracic magnetic resonance imaging demonstrated multiple hyperintense lesions on the chest wall and apex of the right lung. On MRI, a 3×2-cm mass lesion in the right cerebellar hemisphere was detected. Total resection of the mass and ventriculoperitoneal shunting was performed. Histopathologic examination confirmed the diagnosis of HBL. Steroid therapy was administered for disseminated hemangiomatosis, and the lesions showed regression; the patient showed good clinical recovery. The parents refused further treatment, and he was out of our control when he was 9 months old. According to our knowledge, the presented newborn is the second case of cerebellar HBL associated with diffuse skin and visceral hemangiomas in the English medical literature. Clinicians must be vigilant about the predictive value of visceral and/or cutaneous hemangioma for an associated intracranial HBL.

Pacing and clinical electrophysiology : PACE, 2003

Naxos disease is a recessively inherited arrhythmogenic right ventricular cardiomyopathy in which... more Naxos disease is a recessively inherited arrhythmogenic right ventricular cardiomyopathy in which the cardiac phenotype is associated with palmoplantar keratoderma and woolly hair. This disease is a heart muscle disorder causing life-threatening ventricular arrhythmias, heart failure, and sudden cardiac death. The pathological hallmark of the disease is the progressive replacement of myocardial cells by fat and fibrous tissue. It appears in families descending from the Hellenic island of Naxos. We presented a 13-year-old Turkish boy with Naxos disease associated with ventricular tachycardia because of its rarity, and reviewed the literature.

Pediatrics International, 2003

... Neonatal uterine prolapse. Mustafa Akcakus 1 ,; Tamer Gunes 1 ,; Selim Kurtoglu 1 ,; Neside C... more ... Neonatal uterine prolapse. Mustafa Akcakus 1 ,; Tamer Gunes 1 ,; Selim Kurtoglu 1 ,; Neside Cetin 1 ,; Aysenur Pac 1 ,; Hamit Okur 2. Article first published online: 26 JUN 2003. DOI: 10.1046/j.1442-200X.2003.01733.x. Issue. Pediatrics International. ...

Clinical pediatrics, 2004

... Mustafa Akcakus, MD1 Tahir Patiroglu, MD2 Kemal Deniz, MD2 Neside Cetin, MD1 Mehmet Akif Ozde... more ... Mustafa Akcakus, MD1 Tahir Patiroglu, MD2 Kemal Deniz, MD2 Neside Cetin, MD1 Mehmet Akif Ozdemir, MD3 Mehtap Kala, MD2 ... infiltrates (leukemia cutis) in ad-dition to the characteristic signs of congenital leukemia.3 Leuk-emia cutis may be the first mani-festation of ...

Journal of Clinical Research in Pediatric Endocrinology, 2010

Neonatal ovarian cysts (NOC) are usually self-limiting structures. However, large or complex cyst... more Neonatal ovarian cysts (NOC) are usually self-limiting structures. However, large or complex cysts may lead to severe complications. A standard guide to management, treatment and follow-up of NOC is not yet available. The aim of this study was to evaluate retrospectively the records of NOC patients from two medical centers. A total of 20 newborns with NOC were included in the study. The size and localization of the cyst, the age, the signs and symptoms at presentation, and the possible maternal and fetal-neonatal etiologic factors were recorded. Follow-up procedures and treatment modalities were evaluated. The mean age at diagnosis was 34 gestational weeks. The cysts (mean size 53±15 mm) were predominantly in the right ovary (75%) and were evaluated as large cysts in 16 (80%) of the patients. In 5 of the patients with large cysts and in 1 of the 4 patients with small cysts, the cysts were evaluated as complex cysts. Torsion of the ovary was detected in five (25%) cases and these cases were treated surgically. Patients with simple cysts were closely followed by ultrasonography until the cysts disappeared. To date, there is no precise guide for the monitoring and treatment of NOCs. Surgical treatment should always be performed in a way to protect the ovaries and to ensure future fertility. In our NOC series, it has been possible to apply a non-invasive follow-up program and minimally invasive surgical procedures.

Tohoku Journal of Experimental Medicine, 2002

Atherosclerosis is an important cause of cardiovascular morbidity and mortality in recent years. ... more Atherosclerosis is an important cause of cardiovascular morbidity and mortality in recent years. Hyperhomocysteinemia is recognized as an independent risk factor for premature atherosclerosis and venous thrombosis. It is suggested that administration of folic acid, vitamin B6 and vitamin B12 may decrease homocysteine levels. In our study, we induced hyperhomocysteinemia in rabbits by giving methionine and studied the effects of folic acid, vitamin B6 and vitamin B12 on homocysteine levels. A total of 40 (20 female, 20 male New Zealand rabbits) were divided into four groups, each consisting of 10 rabbits. Methionine (100 mg/kg/day), methionine (100 mg/kg/day) plus vitamin B6 (30 mg/kg/day), methionine (100 mg/kg/day) plus vitamin B12 (80 mg/kg/day) and methionine (100 mg/kg/day) plus folic acid (20 mg/kg/day) were given to the first, second, third and forth groups respectively. These rabbits were followed up for two months. We studied homocysteine levels on the 0, 20th, 40th and 60th days in all groups. In rabbits we induced hyperhomocysteinemia by giving methionine for 2 months. The decreases of homocysteine levels in the forth group were significant with respect to the second and third groups. Folic acid supplementation clearly resulted in a reduction of plasma homocysteine levels, whereas vitamin B12 was little effective and vitamin B6 failed to show an effect. We conclude that even folic acid treatment alone may be sufficient for decreasing negative effects of homocysteine.

European Journal of Pediatrics, 2007

Macrosomia is associated with alterations in lipoprotein composition and concentration at birth. ... more Macrosomia is associated with alterations in lipoprotein composition and concentration at birth. Exposure to diabetes in utero has been established as a significant risk factor for some of the components of metabolic syndrome. The aim of this study was to investigate the effect of macrosomia on lipid metabolism, aortic intima-media thickness (aIMT) and subsequent atherogenic risk in newborn infants. Aortic

Erciyes Tıp Dergisi/Erciyes Medical Journal, 2012

International Journal of Dermatology, 2006

ABSTRACT

TURKISH JOURNAL OF MEDICAL SCIENCES, 2014

There are close interactions among the developing oral cavity, pituitary gland, and central nervo... more There are close interactions among the developing oral cavity, pituitary gland, and central nervous system (CNS) in early embryonic life. In this study we aimed to screen endocrine abnormalities in patients with orofacial clefts in the neonatal period. Thirty-one patients with isolated orofacial median clefts wereincluded in the study. Pituitary, thyroid, and adrenal hormones were measured at the first week and remeasured in the third or fourth weeks. Imaging studies were done for detection of CNS anomalies in all patients. Endocrine abnormality was detected in 22 (70.9%) patients. The number of patients with single and multiple endocrine abnormalities were 13 (41.9%) and 9 (29%), respectively. Thyroid hormone-related disorders were detected in 10 (32.3%) patients. Growth hormone deficiency was detected in 4 (12.9%) patients. Adrenocorticotrophic hormone and/or glucocorticoid deficiency was detected in 5 (16.1%) patients. Neonatal hypoglycemia due to endocrinological abnormalities was detected in 6 (19.4%) patients. Defected mini-puberty was seen in 2 (15.4%) patients. There was no relationship between the types of orofacial cleft and endocrine abnormalities. Endocrinological evaluation of the patients with orofacial clefts in the neonatal period is a worthwhile endeavor to detect hormone deficiencies regardless of the type of the cleft.

Pediatric Research, 2007

Neonates with intrauterine growth restriction (IUGR) are associated with reduced concentrations o... more Neonates with intrauterine growth restriction (IUGR) are associated with reduced concentrations of IGF-I that might contribute to arterial wall thickening. Direct atherogenic effects of leptin have been described. We aimed to investigate the relationship among abdominal aortic intima-media thickness (aIMT), serum IGF-I, IGF binding protein-3, and leptin levels in neonates with IUGR. Abdominal aIMT was measured in 40 term neonates with IUGR and in 40 controls. Mean aIMT was significantly greater in neonates with IUGR (0.45 +/- 0.03 mm) than in controls (0.39 +/- 0.04 mm, p < 0.0001). Serum IGF-I and leptin levels were lower in neonates with IUGR than in controls. There was a significant positive correlation between aIMT and gestational age, whereas a significant negative correlation was determined between aIMT and IGF-I in the IUGR neonates. For aIMT, significant associations included serum IGF-I level (beta = -0.406, p = 0.006) and gestational age (beta = 0.331, p = 0.022) in a multiple stepwise linear regression analysis. In control neonates, serum IGF-I levels were negatively related to aIMT (beta = -0.750, p < 0.001). Neonates with IUGR have significant aIMT with decreased IGF-I. IGF-I levels determine aIMT not only in neonates with IUGR but also in healthy controls.

Pediatric Diabetes, 2002

Thiamine-responsive megaloblastic anemia syndrome (TRMA)

Pediatric Dermatology, 2003

Collodion baby is a distinct subset of neonatal erythroderma that can be a clinical marker for a ... more Collodion baby is a distinct subset of neonatal erythroderma that can be a clinical marker for a variety of underlying abnormalities. The phenotype includes parchment-like hyperkeratosis, pseudocontractures, ectropion, eclabium, absence of eyebrows, and sparse hair. Asymmetric crying facies is caused by congenital hypoplasia or agenesis of the depressor anguli oris muscle. Associations of this facial defect with major congenital anomalies have been reported, most commonly in the cardiovascular system, less frequently involving the genitourinary, musculoskeletal, cervicofacial, and respiratory systems, and rarely the endocrine system. We report a newborn with a collodion membrane and asymmetric crying facies. To the best of our knowledge, this association has not been previously published.

Pediatric Dermatology, 2003

Pseudomonas aeruginosa bacteremia or sepsis often occurs in hospitals, affecting mainly children ... more Pseudomonas aeruginosa bacteremia or sepsis often occurs in hospitals, affecting mainly children with underlying disease. Ecthyma gangrenosum is classically considered a pathognomonic sign of sepsis by P. aeruginosa. The harlequin baby, a severe variant of ichthyosis, occurs rarely, and these infants are at high risk of cutaneous infections and sepsis. We herein report a harlequin baby who developed ecthyma gangrenosum.