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Bartter's Syndrome is characterized by the impairment of chloride reabsorbtion in the distal tubules of the kidney. As a result, the kidney loses chloride and persistent hypokalemia, hypochloremia and metabolic alkalosis ensue. Most cases come to the attention of the pediatrican for the early failure to thrive observed in childhood. Its mode of inheritance is thought to be autosomal dominant. in the 1. 5 month old male infant, who admitted to our clinic with the complaints of failure to suck and gain weight, we detected an increase in renal excretion of sodium, potassium and chloride. Plasma renin activity and plasma aldosteron levels were also high. With these results we diagnosed the case as Bartter's Syndrome. We treated the patient with oral potassium and rectal indomethacin. With this therapy, the patient's clinical status and laboratory results improved.

Background The objective of this study was to evaluate juridical and medical responsibilities of health-care professionals accused of malpractice in Turkey while they care for patients under the age of 18. Methods The cases sent to the Council of Forensic Medicine Institution by the courts, including claims of medical malpractice (n = 1458), in order to get an expert opinion between 2002 and 2006, were examined retrospectively. Cases of negligence by health-care professionals who gave medical care to children between the ages of 0 and 18 were evaluated statistically. Results There was medical malpractice in 28.8% of the cases; and 68.2% of the cases were male. A large proportion of the cases were found to occur in emergency departments; and 57.9% of injuries resulted in death. The specialist doctors seemed to be sued or to be accountable for compensation more frequently than the general practitioners. This can be attributed to the fact that they perform more complicated medical interventions. Conclusions Courts specializing in health legislation may be useful to decrease the time consumed by trials. These specialized courts will also support logical judgment. In addition, postgraduate education is useful in reducing malpractice claims.

Alexander disease is a rare autosomal recessive disorder that is characterized by degeneration of the white matter in the central nervous system. Alexander disease is a leukodystrophy that is usually observed in early childhood but rarely in adults. It is characterized by megalencephaly, demyelinization and multiple Rosenthal fibers. Specific magnetic resonance imaging (MRI) findings and genetic investigations are necessary to diagnose the disorder. Signs of leukodystrophy were found in the bilateral white matter on a brain MRI of our four-year-old patient. He had megalencephaly since birth. We use this case to discuss Alexander disease.

Amaç: Bu çalışmada amaç 0 18 yaş grubu olgulara tıbbi müdahalede bulunan sağlık mesleği çalışanları hakkında tıbbi uygulama hatası iddiası ile ilgili açılan davaların adli tıbbi boyutunu değerlendirmektir nbsp; Gereç ve Yöntem: Adli Tıp Kurumu rsquo;na 2002 2006 yılları arasında gönderilen 3 İhtisas Kurulu rsquo;nca incelenen ve tıbbi uygulama hatası iddiası bulunan 0 18 yaş grubu 378 olgu çalışmaya alındı Olgular hangi sağlık çalışanlarından şikayetçi olunduğu hangi sağlık kuruluşlarının davalı olduğu sağlık kuruluşlarının hangi bölümlerinden şikayetçi olunduğu tıbbi uygulama hatası tespit edilen olgularda ortaya çıkan zararların neler olduğu ve saptanan hataların hangi süreçlerde meydana geldiği açısından değerlendirilmiştir. Bulgular: Dava konusu sağlık kuruluşları içinde ilk sırada devlet hastaneleri gelmekte 527 ve bunu özel hastaneler 16 4 izlemekteydi Tıbbi uygulama hatası iddiası ile suçlanan sağlık çalışanı dağılımında ilk sırayı çocuk sağlığı ve hastalıkları uzmanı alırken bunu pratisyen hekimler takip etmekteydi Olguların 219 rsquo;unda 57 9 ortaya çıkan zarar ölümdü Tıbbi uygulama hatalarının değerlendirilmesinde 195 olguda 51 6 uygulamaların tıbbi kurallara uygun olduğu yönünde ve 109 olguda 28 8 tıbbi uygulama hatası olduğu şeklinde görüş bildirilmişti. Çıkarımlar: Ülkemizde 0 18 yaş grubunda artan tıbbi uygulama hataları TUH nedeniyle bu gruba yönelik çalışan sağlık mesleği çalışanlarının özellikle çocuk doktorları ve pratisyenlerin; tanı tedavi bakım ve sevk konularında daha dikkatli olmaları ve özenli nbsp;davranmaları gerekmektedir Bunların yanı sıra hastaya ve yakınlarına ayrıntılı bilgi verilerek önerilen tedavinin komplikasyon ve yan etkileri mutlaka hastanın anlayacağı dil ile anlatılmalıdır., Aim: The objectives of this study are to evaluate malpractice from forensic medicine nbsp; perspective studying lawsuits filed against healthcare professionals who performed medical interventions on cases aged between 0 18 years Material and Method: Medical malpractice claims nbsp; filed against nbsp; healthcare professionals n= 378 submitted nbsp; to The Council of Forensic Medicine during the years 2002 ndash;2006 by the courts to form expert opinion were evaluated by 3 Specialty Board were examined nbsp;retrospectively The cases were evaluated in regard to being complaint about which health workers being engaged with a lawsuit of which health instutions being complaint about which department of health institutions and the damages arising and fault finding processes which occur in medical malpractice cases Results: Among the healthcare institutions subjected to malpractice claims state hospitals ranked first 52 7 followed by private hospitals 16 4 Pediatricians took the lead among healthcare professionals accused of malpractice followed by practicians 219 cases of malpractice 57 9 ensued in death nbsp; Opinions in favour of or against medical malpractice were expressed in 28 8 n=109 and 51 6 n=195 nbsp; of the cases respectively Conclusions: Because of increasing medical malpractices by age group 0 18 in our country the group of health professionals especially pediatricians and practitioners should be more careful and attentive of the diagnosis treatment and care Moreoever the patient his her relatives and intimates should be provided with detailed information about the complications and adverse effects of the recommended treatment using eligible words Turk Arch Ped 2011; 46: 151 8

To evaluate the usefulness of renal zinc clearance/glomerular filtration rate ratio (R(ClZn)/GFR) as an indicator of marginal zinc deficiency that is generally associated with iron deficiency in childhood.Zinc status was evaluated in 36 iron-deficient children (22 boys and 14 girls) who ranged in age from 1 to 10 years using serum zinc concentration and U(Zn/Cr) and R(ClZn)/GFR ratios. The results were compared with the zinc status of 36 similar-aged healthy children (24 boys and 12 girls).Serum zinc concentrations were 96.72 +/- 2.13 microg/dL and 93.93 +/- 1.95 microg/dL in iron-deficient and healthy subjects, respectively (p0.05). U(Zn/Cr) ratios were 0.54 +/- 0.04 microg/mg and 0.88 +/- 0.04 microg/mg (p0.0001); R(ClZn)/GFR ratios were 2.27 x 10(-3) +/- 0.20 and 3.32 x 10(-3) +/- 0.20 (p0.001) in iron-deficient and healthy subjects, respectively. Individual values of R(ClZn)/GFR and U(Zn/Cr) ratios correlated with hemoglobin (Hb) concentrations (r = 0.34, p0.01 and r = 0.26, p0.05). Data grouped according to the ranges of Hb concentrations and R(ClZn)/GFR and U(Zn/Cr) ratios fit the following equations: The statistically significant difference in U(Zn/Cr) and R(ClZn)/GFR ratios between groups indicates decreased urinary estimation of marginal zinc deficiency, whereas no change was observed in serum zinc concentrations. According to the regression equation, it can be postulated that the R(ClZn)/GFR ratio is a linear function of Hb concentration and the U(Zn/Cr) ratio.R(ClZn)/GFR ratio was a reliable indicator for reduction in urinary zinc excretion; it estimated the marginal zinc deficiency associated with iron deficiency. The R(ClZn)/GFR ratio can be calculated using one sample of blood and urine; thus it could serve as an alternative indicator of marginal zinc deficiency, especially in routine health care.

Meperidine is an opiod analgesic used in a variety of clinical situations. The active metabolite, normeperidine, is a central nervous system excitatory agent and has the ability to cause irritability, hyperreflexia, tremor, myoclonus and seizures. Previously identified risk factors for the development of meperidine-related seizures include renal failure, high meperidine dosages, and co-adminestration of hepatic enzyme inducing medications or phenothiazines which decreases seizure treshold. Patients with normal renal function rarely manifest seizure activity when given meperidine. Here we report a 10 year old boy with a femur fraction who had normal renal function. We used low dose meperidine due to post operative pains.

Factor V deficiency is an inherited disorder, in which the clotting factor V is low. The disorder is very rare, occurring in only one in one million people. It is inherited as an autosomal recessive disorder. The results of coagulation studies include a prolonged prothrombin time and partial thromboplastin time associated with reduced plasma factor V content. Patients with factor V deficiency have a hemophiliac like hemorrhagic disorder. Epistaxis, bruising, and menorrhagia are some of the common features. If treatment is needed, fresh frozen plasma is typically given. In this report we present a 12 year old girl who was admitted to our clinic with recurrent nosebleeds and intracranial hemorrage after head trauma. After examination, factor V deficiency was diagnosed. She also had congenital cardiac disorder (VSD), probably a co-incidental finding.

Introduction: The aim of our study is to evaluate thrombocytosis at the time of the diagnosis in the patients with pneumonia, bronchiolitis and asthma, and its importance in terms of the diagnosis. Materials and Methods: We evaluated 583 patients diagnosed as asthma (n=36), bronchiolitis (n=387) and pneumonia (n=160) in pediatry clinic of GATA Haydarpasa Training Hospital and Zeynep Kamil Research Hospital between January 1, 2007 and January 1, 2010; and the ratio of patients with thrombocytosis at the time of diagnosis retrospectively. Results: We found that the mean thrombocyte counts in patients who had been diagnosed as pneumonia and as bronchiolitis were found significantly higher than control group (p

Thomas syndrome is a rare syndrome including Potter sequence, renal anomalies, heart defects, cleft palate with other oropharyngeal anomalies. Here, we report a newborn with Potter sequence, bilateral renal hypoplasia and cystic dysplasia, multiple cardiovascular malformations, long large ears, frontal bossing, small lips, partial simple toe syndactyly, and cleft palate. To our best knowledge, this patient may be considered as a new variant of Thomas syndrome or a new syndrome.

Objective: The effect of ghrelin on growth of the newborn has long been argued, but not fully clarified. In this study, we aimed to investigate the relationship between ghrelin levels and growth parameters in the first 3 months of life. Methods: The study included 60 babies (27 girls and 33 boys) born at gestational ages between 38-42 weeks. The newborns were divided into three groups according to the Lubchenco curves as: small for gestational age (SGA), appropriate for gestational age (AGA) and large for gestational age (LGA). The relationship between ghrelin levels and growth parameters in the third month was investigated. Results: Ghrelin concentrations were significantly higher in SGA (2.4±2.6 ng/dL) babies than in AGA (1.3±0.9 ng/dL) and LGA (1.0±0.8 ng/dL) babies. The lowest ghrelin levels were in the LGA group. In SGA infants, ghrelin concentrations were inversely correlated with change in weight (r=-0.577; p=0.001), change in length (r=-0.361; p=0.005), and change in head circumference (r=-0.387; p=0.002). Conclusion: The results show that at age 3 months, SGA infants had higher ghrelin levels than AGA and LGA infants. Our findings indicate that ghrelin may be involved in the process of catch-up growth in these infants. Conflict of interest:None declared.