Efficacy of Adenosine in the Differential Diagnosis of Narrow QRS Complex Tachyarrhythmia: A Case Diagnosed with Atrial Flutter After Adenosine

The most common tachyarrhythmias in childhood are narrow QRS complex tachyarrhythmias. The majority of these are supraventricular tachycardia (SVT). If electrocardiography is inconsistent with typical SVT, another underlying arrhythmia should be considered. In this case, a patient with narrow complex tachycardia who was diagnosed with atrial flutter after adenosine was presented to increase awareness on the subject

A Case with Mega Cisterna Magna Renal and Ear Anomalies: Is This a New Syndrome?

Background. Extrarenal pathologies may be associated with renal position and fusion anomalies. According to the literature, our patient is the first horseshoe kidney case that had mega cisterna magna, arachnodactyly, and mild mental retardation. Case Report. A 9-year-old boy admitted because of the myoclonic jerks. He had a dysmorphic face, low-set and cup-shaped ears, arachnodactyly, and mild mental retardation. The patient’s laboratory findings were normal except for a mild leucocytosis and hypochromic microcytic anemia. His cerebrospinal fluid was cytologically and biochemically normal. Cranial MRI revealed 1.5 cm diametered mega cisterna magna in the retrocerebellar region. Although there were no significant epileptical discharges in the electroencephalography, there were slow wave discharges arising from the anterior regions of both hemispheres. Because he had stomachache, abdominal ultrasonography was performed, and horseshoe kidney was determined. Abdominal CT did not reveal any abnormalities except the horseshoe kidney. There were not any cardiac pathologies in echocardiography. He had normal 46XY karyotype and there were no repeated chromosomal derangements, but we could not evaluate for molecular and submicroscopic somatic changes. He was treated with valproic acid and myoclonic jerks did not repeat. Conclusion. We suggest that the presence of these novel findings may represent a newly recognized, separate syndrome

Childhood brucellosis in southeastern region of Turkey : a retrospective analysis

Amaç: Bu çalışmada dört yıllık süreçte brusellozis tanısıyla yatırılarak takip ve teda- vi edilen hastaların demografik ve klinik özelliklerinin değerlendirilmesi amaçlandı. Yöntemler: Retrospektif olarak yapılan bu çalışmaya 2003-2007 yılları arasında Dicle Üniversitesi Çocuk İnfeksiyon Hastalıkları Servisine brusellozis tanısı ile yatırılan 34 olgu alındı. Hastaların demografik özellikleri, klinik yakınmaları, fizik muayene ve laboratuvar bulguları değerlendirildi. Standart tüp aglütinasyon testi, Rose Bengal testi ve klinik bulgular tanı kriteri olarak kullanıldı. Bulgular: Hastaların 12 (%35)’si kız, 22 (%65)’si erkekti. Hastaların ortalama yaşı 9,2±3,4 yıl idi. 21 (%61,7) hasta kırsal alandan başvurmuştu. İki hasta dışında tüm hastalar, çiğ süt ve süt ürünleri tüketmişti. Hastaların yakınma ve bulguların arasın- da ateş (30, %88,2) ve kas-iskelet sistem yakınmaları (26, %76,4) ön plandaydı. Laboratuvar incelemelerinde hematolojik tutulum belirgindi. Tedavide sekiz yaşın- dan küçük çocuklara trimetoprim-sulfametoksazol+rifampisin, sekiz yaşından büyük- lere doksisiklin+rifampisin 6 hafta boyunca uygulandı. Dört hastada relaps görüldü. Sonuç: Brusellozisin başvuru nedenleri arasında kas iskelet sistem yakınmaları ve ateş ilk sıradadır. Hematolojik sistem bulguları olan hastada brusellozis ayırıcı tanı- lar arasında olmalıdır. Tedavinin başarılı olması ve relaps oluşmaması için kombine tedaviler yeterli süre verilmelidir.Objective: To evaluate the clinical and laboratory findings, treatment modalities and outcomes of children with brucellosis in a four-year period Methods: This retrospective study included 34 children who were admitted to Dicle University Pediatric Infectious Diseases Service with brucellosis between 2003-2007. Demographic features, clinical symptoms, physical examination and laboratory fin- dings of patients were evaluated. The standard tube agglutination test, Rose Bengal test and clinical findings were used as diagnostic criteria for brucellosis. Results: In the study, twelve patients (35%) were female and 22 (65%) were male. The mean age was 9.2±3.4 years. Twenty-one patients (61.7%) were from rural areas of Turkey. All of the children except two patients have reported to consume unprocessed milk and dairy products. The most frequent complaints and clinical findings were fever (88.2%) and musculo-skeletal system symptoms (76.4%). Hematological invol- vement was significantly apparent in laboratory results. Trimethoprim- sulfamethoxazole+rifampicin were administered to children smaller than 8 years and doxycycline+rifampicin to children older than 8 years during 6 weeks for treatment. Four patients were relapsed. Conclusion: Musculoskeletal system complaints and fever are the most common symptoms in brucellosis. Brucellosis should be considered in the differential diagnosis of patients with hematological signs. Combined treatments should be administered within a sufficient period to provide successful treatment and prevent relapse

Splenectomy in patients with thalassemia major: Evaluation of 35 cases

Amaç: Beta talasemi, b globin sentezinin azalması veya hiç üretilememesi sonucunda oluşan, otozomal resesif geçişli ve yeryüzünde yaygın görülen kalıtsal anemi tipidir. Splenektomi, birçok hematolojik hastalıkta tedavi amaçlı uygulanabilmektedir. Bu çalış- mada, kliniğimizden takipli talasemi major hastalarından splenektomi yapılan hastaların kayıtlı bilgilerinin tartışılması amaçlandı. Yöntemler: Takipli 782 talasemi major hastasının kayıtları geriye dönük olarak tarandı. Bu hastalardan splenektomi yapılmış ve kayıtlı bilgilerine ulaşılabilen 35 hasta çalışmaya dâhil edildi. Hastaların kayıtlı bilgileri, tanı alma yaşları, cinsiyet durumları, operasyon yaşları, operasyon endikasyonları, şelasyon tedavisi, transfüzyon sıklıkları ve ameliyat sonrası klinik yanıt durumları açısından detaylı incelendi. İstatistiksel incelemede SPSS 13.0 İstatistik programı kullanıldı. Bulgular: Hastaların 22si (%63) erkek, 13ü (%37) kız idi. Ortalama tanı alma yaşı 82 ay, splenektomi yaşı 8,5 yıl idi. En önemli splenektomi endikasyonu artmış transfüzyon gereksinimiydi (%57,14). Ameliyat öncesi transfüzyon gereksinimi, 23530 mL/kg/yıl iken; ameliyat sonrası gözlemlerde 11515 mL/kg/yıla kadar gerilemişti. Splenektomi öncesi ortalama serum ferritin düzeyi 1745761 ng/mL, splenektomi sonrası 1985570 ng/mL idi. İzlemlerde hiçbir hastada sepsis veya pıhtılaşma bozukluğu gibi herhangi bir komplikasyon gelişmedi. Sonuç: Talasemi hastalarında splenektomi yakınmaların azaltılması ve transfüyon sıklığının azaltılmasında faydalıdır. Demir şelasyon tedavisinin yetersiz uygulanması ile splenektomiye gidiş zamanı arasında bir korelasyon olabilir.Objective: Beta-thalassemia is common, worldwide, autosomal recessive and inherited type of anemia which results from absent or reduced synthesis of b globin. Splenectomy may be performed for the treatment of many hematologic diseases. The aim of this study is to discuss recorded data of thalassemia major patients who underwent splenectomy. Methods: The records of 782 patients with thalassemia major were reviewed retrospectively. Of these patients, 35 splenectomized patients with available data were included in the study. Recorded data of patients were evaluated as for age at diagnosis, gender, age at splenectomy, surgical indications, chelation therapy, annual transfusion requirements, and postoperative clinical response. Statistical analyses were performed with statistical package of SPSS version 13.0. Results: Twenty-two (63%) male and 13 (37%) female patients were included in the study. Mean age at diagnosis was 8±2 months and splenectomy was 8.5 years. The main indication for splenectomy was increased transfusion requirement (57.14%). The annual transfusion requirement reduced from preoperative 235±30 mL/kg to 115±15 mL/kg postoperatively. Mean serum ferritin levels were 1745±761 ng/mL, and 1985±570 ng/mL before, and after splenectomy. None of the patients developed any complications such as sepsis or coagulation disorder. Conclusion: These results suggest that splenectomy is beneficial to improve the quality of the lives of patients and significantly reducing blood consumption. Inadequate iron chelation therapy may be associated with progression to splenectomy

Splenectomy in patients with thalassemia major: Evaluation of 35 cases

Amaç: Beta talasemi, b globin sentezinin azalması veya hiç üretilememesi sonucunda oluşan, otozomal resesif geçişli ve yeryüzünde yaygın görülen kalıtsal anemi tipidir. Splenektomi, birçok hematolojik hastalıkta tedavi amaçlı uygulanabilmektedir. Bu çalış- mada, kliniğimizden takipli talasemi major hastalarından splenektomi yapılan hastaların kayıtlı bilgilerinin tartışılması amaçlandı. Yöntemler: Takipli 782 talasemi major hastasının kayıtları geriye dönük olarak tarandı. Bu hastalardan splenektomi yapılmış ve kayıtlı bilgilerine ulaşılabilen 35 hasta çalışmaya dâhil edildi. Hastaların kayıtlı bilgileri, tanı alma yaşları, cinsiyet durumları, operasyon yaşları, operasyon endikasyonları, şelasyon tedavisi, transfüzyon sıklıkları ve ameliyat sonrası klinik yanıt durumları açısından detaylı incelendi. İstatistiksel incelemede SPSS 13.0 İstatistik programı kullanıldı. Bulgular: Hastaların 22si (%63) erkek, 13ü (%37) kız idi. Ortalama tanı alma yaşı 82 ay, splenektomi yaşı 8,5 yıl idi. En önemli splenektomi endikasyonu artmış transfüzyon gereksinimiydi (%57,14). Ameliyat öncesi transfüzyon gereksinimi, 23530 mL/kg/yıl iken; ameliyat sonrası gözlemlerde 11515 mL/kg/yıla kadar gerilemişti. Splenektomi öncesi ortalama serum ferritin düzeyi 1745761 ng/mL, splenektomi sonrası 1985570 ng/mL idi. İzlemlerde hiçbir hastada sepsis veya pıhtılaşma bozukluğu gibi herhangi bir komplikasyon gelişmedi. Sonuç: Talasemi hastalarında splenektomi yakınmaların azaltılması ve transfüyon sıklığının azaltılmasında faydalıdır. Demir şelasyon tedavisinin yetersiz uygulanması ile splenektomiye gidiş zamanı arasında bir korelasyon olabilir.Objective: Beta-thalassemia is common, worldwide, autosomal recessive and inherited type of anemia which results from absent or reduced synthesis of b globin. Splenectomy may be performed for the treatment of many hematologic diseases. The aim of this study is to discuss recorded data of thalassemia major patients who underwent splenectomy. Methods: The records of 782 patients with thalassemia major were reviewed retrospectively. Of these patients, 35 splenectomized patients with available data were included in the study. Recorded data of patients were evaluated as for age at diagnosis, gender, age at splenectomy, surgical indications, chelation therapy, annual transfusion requirements, and postoperative clinical response. Statistical analyses were performed with statistical package of SPSS version 13.0. Results: Twenty-two (63%) male and 13 (37%) female patients were included in the study. Mean age at diagnosis was 8±2 months and splenectomy was 8.5 years. The main indication for splenectomy was increased transfusion requirement (57.14%). The annual transfusion requirement reduced from preoperative 235±30 mL/kg to 115±15 mL/kg postoperatively. Mean serum ferritin levels were 1745±761 ng/mL, and 1985±570 ng/mL before, and after splenectomy. None of the patients developed any complications such as sepsis or coagulation disorder. Conclusion: These results suggest that splenectomy is beneficial to improve the quality of the lives of patients and significantly reducing blood consumption. Inadequate iron chelation therapy may be associated with progression to splenectomy

ÇOCUKLUK ÇAĞI BRUSELLOZUNDA BİLATERAL SAKROİLEİT: OLGU SUNUMU

Brusella, ülkemizin de yer aldığı belirli coğrafyalarda daha sık olmak üzere tüm dünyada yaygın olarak görülen zoonotik, sistemik enfeksiyöz bir hastalıktır. Farklı klinik tablolarla hastaların başvurusu olabilirken, özgün tanısal bir testi bulunmamaktadır. Biz bu vaka sunumumuzda, ateş, halsizlik, karın ve bel ağrısı şikayeti ile başvuran, erken dönemde aglütünasyon testleri negatif olan ve izleminde aglütünasyon testleri pozitifleşen ve kemik iliği kültüründe brusella grubu bakteri üremesi saptanan 12 yaşında bir erkek hastayı değerlendirdik. Bu bağlamda kuvvetle bruselloz düşünüldüğünde tanısal testlerin tekrarlanmasının önemini vurgulamak ve ayrıca, radyolojik olarak sakroileit varlığında da brusellozun ayırıcı tanılar arasında yer aldığını belirtmek için tartışmak istedik.Anahtar kelimeler: Brusella, kemik sintigrafisi, sakroilleit.SUMMARYBrucellosis is well recognized throughout the world, more frequently seen in certain geographic areas including Turkey. Patients may have apply with different clinic symptoms but there is no spesific laboratory test for its diagnosis. In this case, we report a 12-years-old boy whose main symptom was back pain and whose Brucella agglutination tests were negative in the early period, turning to positive later, where Brucella genus bacteria was isolated from her bone marrow aspiration culture. Here we point out the importance of repeating diagnostic tests in highly suspected cases and think that Brucella may radiologically be diagnosed in the presence of sacroileitis.Key words: Brucellosis, Bone scintigraphy, sacroileitis

A case of Gianotti Crosti syndrome with HBV infection

dikici, bunyamin/0000-0001-7572-6525WOS: 000207602800035PubMed: 18614441Gianotti-Crosti syndrome (papular acrodermatitis of childhood), which was first described in 1955, is a nonspecific rash that usually consists of the abrupt onset of pink flesh coloring, smooth or lichenoid, flat-topped papules. It was first related to hepatitis B virus (HBV) infection; however, cases not associated with HBV infection were reported as well. Although a type of delayed hypersensitivity reaction is speculated as a cause, exact pathogenesis still remains unclear. The prognosis is favorable and successful management relies upon general supportive and symptomatic care. We report a seven-year-old boy diagnosed with Gianotti-Crosti syndrome with monomorphous papules on his cheeks, buttocks and extremities associated with hepatitis B virus infection

Acquired methemoglobinemia after application of prilocaine

Prilokain, pek çok alanda kullanılan lokal anestetik bir ajandır, ancak hayatı tehdit edebilen bir yan etki olan methemoglobinemiye neden olabilmektedir. Methemoglobinin O2 taşıma kapasitesi düşüktür ve doku düzeyinde hipoksiye neden olabilir. Sünnet operasyonundan iki saat sonra ağız bölgesinde, ellerinde ve ayak parmaklarında morarma yakınması ile acil servise başvuran 54 günlük bebeğin fizik muayenesinde mevcut durumu açıklayacak patoloji saptanmadı. Oksijen tedavisine yanıtsız olan hasta yoğun bakım ünitesine alındı. Sünnet işlemi öncesi 2 mg/kg prilokain yapıldığı öğrenilen, oksijen tedavisine rağmen satürasyon değerleri düşük seyreden ve kan gazı incelemesinde methemoglobin düzeyi %24,7 saptanan hastaya methemoglobinemi tanısı konuldu. Hastaya 300 mg/kg/doz askorbik asit başlandı ve 24 saatlik izlem sonrası şifa ile taburcu edildi. Cerrahi girişim sonrasında siyanoz gelişen ve SaO2 ile pO2 değerleri arasında uyumsuzluk saptanan hastalarda methemoglobinemi düşünülmeli ve gelişebilecek problemler açısından tam donanımlı çocuk yoğun bakım ünitelerinde izlenmelidirler.Prilocaine is a local anesthetic agent and is widely used in most of ambulatory procedures, but it can cause methemoglobinemia which is a life-threatening condition. Methemoglobinemia may cause hypoxia of tissue because methemoglobin has less oxygen carrying capability than hemoglobin. A 54-dayold boy who developed cyanosis around his mouth, on his hands and toes two hours after the circumcision operation was admitted to the emergency department. No abnormality was established on his physical examination except for cyanosis. Cyanosis did not improve with oxygen administration and the patient was admitted to intensive care unit. His history revealed that prilocaine hydrochloride (2 mg/ kg) was administered subcutaneously as local anesthesia prior to circumcision operation. Diagnosis of methemoglobinemia was established in the patient in whom saturation levels were low in spite of the oxygen therapy and in whom methemoglobinemia level was 24.7%. His cyanosis completely disappeared after administration of intravenous ascorbic acid (300 mg/kg/dose) and he was discharged after a 24-hour follow-up period with recovery. Methemoglobinemia should be considered in cases with cyanosis and inconsistency between oxygen saturation and partial arterial oxygen pressure. These cases should be monitored in pediatric intensive care units for lethal complications

Results of exchange transfusions in newborns without blood group incompatibility

Objective: Hyperbilirubinemia is a common problem ofneonatal period that has high morbidity and mortality.Blood exchange is the most effective and urgent treatmentmodality for very high bilirubin levels that can lead toneurotoxicity called as kernicterus. The aim of this studywas to compare 90 minutes exchange transfusion withthat of 120 minutes.Methods: This study was performed at Dicle UniversityMedical Faculty, Neonatal Unit between July 2007 andJune 2008. A total of 36 term newborn (38 - 42 gestationalweek) without blood group incompatibility and withtotal serum bilirubin levels over 25 mg/dl were included.Newborns were randomly assigned in two groups eachof them comprise 18 babies as Group 1 underwent 90minute-exchange and Group 2 120 minute. Effectivenessand complications of exchange transfusion were recorded.Newborns with Rh, ABO or subgroup incompatibilities,prematurity or small for gestational age, septicemia,hypothyroidism, G6PD enzyme deficiency, intrauterineinfections, diabetic mother’s baby, hemolytic disease ormetabolic diseases were excluded.Results: There were no significant differences in thebody weight, gestational age, postnatal age, age of mother,total bilirubin and albumin levels, the number of bloodexchange, hospital stay days and complications betweentwo groups (p>0.05). However, mean phototherapy durationwas significantly shorter in 120 minutes transfusiongroup compared with 90 minutes group (p<0.001).Conclusion: Our results indicated that 90 minutes wassufficient for an effective exchange transfusion in severehyperbilirubinemic newborn infants. However longer exchangetransfusion durations may shorten the duration ofphototherapy.Key words: Indirect hyperbilirubinemia, exchange transfusion,newborns, outcom