Etiopathogenesis of sirenomelia on the framework of a case report

Kaudal regresyon sendromu grubu içinde değerlendirilen si- renomeli nadir görülen bir anomalidir. Birçok organda ciddi malformasyonla beraberlik gösterir. Bu yazıda daha önce tıbbi yazında bildirilmemiş olan sirenomeli ile 13 kaburga ve torakal vertebranın birlikteliği sunuldu. Olgumuzu, etiyopatogenezle ilgili yeni yorumlara ışık tutabileceği düşüncesiyle sunduk.Sirenomelia is a rare anomaly evaluated in the group of caudal regression syndrome. It is associated with serious malforma- tion in many organs. In this article, we present a sirenomelia case with coexistence in 13 ribs and thoracic vertebrae. We re- ported our case to shed light on new comments with etio- pathogenesis

Iron poisoning in lethal dose : Case report

Demir tüm hücreler için gerekli olan temel bir elementtir. En önemli görevi hemoglobin aracılığı ile oksijen taşımaktır. Demir fizyolojik olaylarda kullanılmak üzere her gün belli miktarlarda alınması zorunlu bir mineraldir. Demirin yüksek dozda alınması vücutta toksik etkiye neden olabilmektedir. Demir zehirlenmesinde en çok etkilenen organ karaciğerdir. Kalp, böbrek, akciğerler ve hematolojik sistem de zarar görebilecek diğer başlıca organlardır. Bu yazıda, yüksek dozda demir alımına bağlı gelişen toksikasyon olgusu tartışıldı. Yirmi bir aylık kız çocuk yüksek dozda demir preparatı içmesi nedeniyle çocuk acilimize getirildi. Hastanın 153 mg/kg demir almış olduğu saptandı. İlaç alımını takiben 4. saatte bakılan kan demir düzeyi 377 ?g/dl idi. Hastaya desferroksamin tedavisi başlandı. Demir düzeyi giderek düşen hastanın gelişinin 4. gününde kan demir düzeyi 23 ?g/dl saptandı. Hasta ayaktan izlenmek üzere evine gönderildi. Demir preparatlarının tek dozluk paketlenmesi, ilaçların çocukları cezbedici tat ve görünümde olmaması, ilaç kutularında çocuk koruyucu kapakların kullanılması, doktorların erişkin hastalara demir preparatlarını reçete ederken toksisite açısından aileyi uyarması ve gerekli bilgileri vermesi mortalite ve morbiditeyi azaltacağı görüşündeyiz.Iron is an essential element for all human cells, and it is required in certain quantities every day for use in physiological events. The primary use of iron is in hemoglobin, used for vital oxygen transport. Large quantities of iron can be toxic to the human body. The liver is the primary organ which is subject to its toxic effects. Secondary damage occurs to the heart, kidneys, lungs, and the hematologic system. In this study, we discuss a case of high dose iron intake intoxication. A twenty-one month old girl was brought to our child emergency unit due to consumption of a high dose iron drug. History of the case revealed that 153mg/kg elemental iron was taken orally. Blood iron level in the patient was 377 μg/dl four hours after ingestion. Desferrioxamine was started for treatment of iron toxicity. Blood iron level was gradually reduced and detected as 23 μg/dl on the fourth day. The patient was sent back home for outpatient follow-up. This report suggests that doctors must caution families and provide information about the toxicity of iron preparations. Warning parents will reduce mortality and morbidity due to poisoning. Additionally, single-dose packaging, the use of unpleasant appearances for toxic drugs, and the use of protective covers may be beneficial for reducing the incidence of toxicity

The use of Chronic Total Occlusion (CTO) wires for perforation of atretic pulmonary valve; two centers experience

Pulmonary valve atresia with intact ventricular septum (PA-IVS) can be treated either surgically or transcatheterly for eligible patients. Perforation of pulmonary valves using chronic total occlusion (CTO) guidewires has been reported as an alternative to radiofrequency (RF) perforation. We sought to report our experience with CTO guidewires for perforation of atretic pulmonary valves and subsequent balloon dilatation (with or without patent ductus arteriosus stenting) in patients with PA-IVS from two centers. A retrospective study was carried out on PA-IVS patients who underwent intervention between March 2014 and September 2019, in which CTO guidewire was employed for pulmonary valve perforation. A total of 26 patients were identified. The median age and weight of the patients were 5.2 days (range 1-21 days) and 3.1 kg (range 2.2-3.8 kg), respectively. All patients were situs solitus, except one patient with left atrial isomerism. The right ventricle (RV) morphology was bipartite in 22/26 patients and tripartite in 4/26 patients. Before the procedure, the mean saturation was 76% (range 70-86%) while the patients were under prostaglandin infusion. The pulmonary valve perforation attempt was performed with the Asahi Conquest Pro 9 CTO wire (n = 6) or Asahi Pro 12 CTO wire (n = 18) and/or Asahi Miracle CTO wire (n = 2). The procedure was successful in 20/26 (77%) patients using CTO wires. We analyzed the efficiency of CTO wire based on the subtypes: Conquest Pro 9 in 6/6 (100%) patients, Conquest Pro 12 in 12/18 (67%) patients, and Miracle in 2/2 (100%) patients. Before CTO wire usage in 3 patients, radiofrequency (RF) perforation was unsuccessful. Among these 3 patients, pulmonary valve perforation was successful in 2 patients with CTO wire; hence, in the remaining patient, perforation was also unsuccessful with CTO wire. After CTO wire perforation was unsuccessful in 6 patients, RF perforation was attempted in 3 patients (2 successful attempts and 1 unsuccessful attempts), and one patient as referred to surgery. Desaturation was persistent in 19 cases, which necessitated ductus arteriosus stenting. Early procedural complication was observed in 3/26 (11%) patients. Two of these patients had vascular complications due to the sheath, which was treated with heparin infusion and streptokinase, and the remaining patient had sudden bradycardia and cardiac arrest during the procedure and did not respond to cardiac resuscitation. CTO wires should be keep in mind for atretic pulmonary valve perforation as a first choice or when RF perforation is unsuccessful

Electroanatomic mapping guided cryoablation of Mahaim pathways in children with limited fluoroscopy exposure

WOS: 000350505800012PubMed ID: 25546471BackgroundThe presence of Mahaim accessory pathways (MAP) with anterograde decremental conduction is a disorder that leads to antidromic atrioventricular reentrant tachycardia. There are rare reports of cryoablation use in MAP. This study aims at sharing our experience with using cryoablation to treat MAP in children. MethodsElectrophysiology study and catheter ablation were performed in 14 patients diagnosed with Mahaim tachycardia between January 2010 and December 2013. Cryoablation was used in nine of the cases. A three-dimensional navigation system with surface electrode patches (EnSite System, St. Jude Medical Inc., St. Paul, MN, USA) was used for all procedures. ResultsThe patients (two girls and seven boys) had a median age of 11.5 years (8-18 years) and a median weight of 67 kg (31-80 kg). Mahaim conduction was localized in the right posterolateral (n = 4), right lateral region (n = 2), right posteroseptal (n = 1), right anterolateral (n = 1), and right anterior (n = 1). A pathway potential was noted in six of nine cases at the tricuspid annulus. Catheter choices and acute success rates were as follows: cryoablation in four (three of four successful), radiofrequency catheter ablation (RFA) and cryoablation in five (successful in four of five). No fluoroscopy was used in six of nine patients. The mean procedure duration was 249 90 minutes. No major complications were observed. The final long-term success rate for cryoablation was seven of nine (78%). ConclusionsCryoablation can be used as a reliable and effective alternative to RFA in the treatment of Mahaim accessory conduction pathways in children. Prospective comparative studies are necessary in order to further evaluate the long-term efficacy of this method

Cryoablation with an 8-mm-Tip -catheter for right-sided accessory pathways in children

WOS: 000380902800003PubMed ID: 27197083BackgroundCryoablation is increasingly utilized in children because of its safety profile. Recently, larger catheter tips have been more widely used to improve long-term success rates. The aim of this study was to assess the safety and efficacy of 8-mm-tip catheters for cryoablation of right-sided accessory pathways (APs) in children. MethodsElectrophysiological procedures were performed using the EnSite system (St. Jude Medical Inc., St. Paul, MN, USA). ResultsBetween July 2010 and July 2014, 54 patients (mean age: 13.1 3.7 years) underwent cryoablation using an 8-mm-tip catheter. In 18 of 54 (33%) patients where an 8-mm-tip catheter was the first-choice catheter, the success rate was 18 of 18 (100%). There was a history of previous failed attempts or recurrence with radiofrequency ablation and/or 6-mm-tip cryoablation in 36 of 54 (67%) patients. The success rate in these patients was 24 of 36 (67%). No fluoroscopy was used in 34 of 54 procedures. The recurrence rate was six of 42 (14%) during a mean follow-up period of 32 +/- 15 months. In one patient, transient atrioventricular block occurred. ConclusionsCryoablation with an 8-mm-tip catheter for right-sided APs in children who weigh over 40 kg appears to be safe and acutely effective in cases where conventional ablation methods fail and also as a first choice for ablation procedure. However, the recurrence rate still seems to be high

A rare ventriculoarterial connection: Double outlet of both ventricles

Ventriculoarterial connection is one of the important points of the segmental approach to congenital cardiac malformations. Double outlet of both ventricles is a rare form where both great arterial roots override the interventricular septum. In this article, we aimed to draw attention to this very rare form of ventriculoarterial connection by presenting an infant case diagnosed using echocardiography, CT angiography, and 3-dimensional modelling

Yenidoğan ve infant döneminde sağ ventrikül çıkım yoluna stent yerleştirilmesi: Çok merkezli, retrospektif çalışma

Background: The aim of this study was to evaluate the outcomes of right ventricular outflow tract stenting for palliation during the newborn and infancy periods. Methods: Between January 2013 and January 2018, a total of 38 patients (20 males, 18 females; median age 51 days; range, 3 days to 9 months) who underwent transcatheter right ventricular outflow tract stenting in three centers were retrospectively analyzed. Demographic characteristics, cardiac pathologies, angiographic procedural, and clinical follow-up data of the patients were recorded. Results: The diagnoses of the cases were tetralogy of Fallot (n=27), double outlet right ventricle (n=8), complex congenital heart disease (n=2), and Ebstein's anomaly (n=1). The median weight at the time of stent implantation was 3.5 (range, 2 to 10) kg. Five cases had genetic abnormalities. The median pre-procedural oxygen saturation was 63% (range, 44 to 80%), and the median procedural time was 60 (range, 25 to 120) min. Acute procedural success ratio was 87%. Reintervention was needed in seven of patients due to stent narrowing during follow-up. During follow-up period, seven cases died. Total correction surgery was performed in 26 patients without any mortality. While a transannular patch was used in 22 patients, valve protective surgery was implemented in two patients, and the bidirectional Glenn procedure was performed in two patients. Conclusion: Based on our study results, right ventricular outflow tract stenting is a form of palliation which should be considered particularly in cases in whom total correction surgery is unable to be performed due to morbidity.Amaç: Bu çalışmada yenidoğan ve infant döneminde palyasyon amaçlı sağ ventrikül çıkım yoluna stent uygulanan olguların değerlendirilmesi amaçlandı. Ça­lış­ma pla­nı: Ocak 2013 ve Ocak 2018 tarihleri arasında, üç merkezde, sağ ventrikül çıkım yoluna transkateter yoldan stent yerleştirilen toplam 38 hasta (20 erkek, 18 kadın; medyan yaş 51 gün; dağılım 3 gün-9 ay) retrospektif olarak incelendi. Hastaların demografik özellikleri, kardiyak patolojileri, anjiyografi işlem ve klinik izlem verileri kaydedildi. Bulgular: Tanılar Fallot tetralojisi (n=27), çift çıkışlı sağ ventrikül (n=8), kompleks doğumsal kalp hastalığı (n=2) ve Ebstein anomalisi (n=1) idi. Stent yerleştirme sırasında medyan ağırlık 3.5 (dağılım, 2-10) kg idi. Beş olguda genetik anormallikler mevcuttu. İşlem öncesi medyan oksijen satürasyonu %63 (dağılım, %44-80) ve medyan işlem süresi 60 (dağılım, 25-120) dk. idi. Akut işlem başarısı %87 idi. Takip döneminde stent daralması nedeniyle yedi hastaya tekrar girişim uygulandı. Takip döneminde, yedi olgu kaybedildi. Hastaların 26’sına mortalite olmaksızın tam düzeltme ameliyatı yapıldı. Yirmi iki hastada transanüler yama kullanılırken, iki hastada kapak koruyucu tam düzeltme ve iki hastada çift yönlü Glenn operasyonu gerçekleştirildi. So­nuç: Çalışma sonuçlarımıza göre, sağ ventrikül çıkım yoluna stent yerleştirilmesi, özellikle morbidite nedeni ile tam düzeltme ameliyatı yapılamayan olgularda akla gelmesi gereken bir palyasyon çeşididir