Evaluation of visceral leishmaniasis diagnosis using indirect fluorescent antibody tests in 4 pediatric patients.

Visceral Leishmaniasis (VL) in infancy is mostly seen between the ages of 2-4,and visceral infection causes long standing fever, weakness, weight loss, hepatosplenomegaly and pancytopenia. Leishmania infantum is responsible for VL in Turkey. In this study, 4 pediatric cases of VL were analysed retrospectively. Bone marrow aspirate was obtained in two cases and Leishmania amastigotes were not obtained in these cases. Leishmania antibodies by the indirect immun fluorescent antibody test (IFAT) were positive in all cases. We consider that IFAT was a suitable alternative to parasite detection in the conclusive diagnosis of visceral leishmaniasis in pediatric patients when strong clinical suspicion is present

Subgroup incompatibility due to anti-E In newborn: two case reports

Yenidoğanın hemolitik hastalığı, maternal eritrosit antikorlarının fetus eritrositlerini direkt etkilemesi sonucu görülen ağır bir durumdur. Çoğunlukla Rh ve ABO uygunsuzluğuna bağlı görülmekle birlikte, nadiren subgrup uyuşmazlığı nedeniyle de karşımıza çıkabilir. Subgrup uyuşmazlığında, hafif hiperbilirubinemiden kan değişimine ihtiyaç gösterecek kadar ciddi hemolitik reaksiyonlar görülebilmektedir. Biz bu yazıda, anti-E’ye bağlı yenidoğanın hemolitik hastalığı gelişen iki olgu sunduk. Birinci olgu, doğumdan sonra yoğun fototerapi ve intravenöz immunglobulin (İVİG) ile tedavi edildikten sonra taburcu edildi. Otuz üç günlükken ağır anemi ve sarılık ile yeniden hastanemize başvuran hastaya eritrosit transfüzyonu, fototerapi ve İVİG tedavisi uygulandı. İkinci olgu, postnatal ikinci gün total bilirubin 21 mg/dL ile hastanemize sevkedildi. Yoğun fototerapi ve İVİG verilen olgunun izleminde hemoliz komplikasyonu gelişmedi.Hemolytic disease of newborn is a severe disease, resulting from maternal red blood cell (RBC) alloantibodies directed against fetal RBCs. This is mostly due to Rh (D), ABO incompatibility and also rarely due to subgroup incompatibility. Mild hyperbilirubinemia to severe hemolytic reactions requiring exchange transfusion therapy can occur in subgroup incompatibility. In this article, we report two patients who developed hemolytic disease of the newborn due to anti-E. Case 1 was treated with intensive phototherapy and IVIG immediately after birth. This case was readmitted to the hospital at 33rd day of life because of jaundice and severe anemia and treated with IVIG, phototherapy and erythrocyte transfusion. Case 2 was referred to our hospital at postnatal second day because of total bilirubin levels of 21 mg/dL. This case was also treated with IVIG, intensive phototherapy and no any complications of hemolysis developed during clinical follow up

Anne sütüyle beslenen yenidoǧanlarda hipernatremik dehidratasyon

Introduction: Since it can cause life-threatening complications in newborns, diagnosis and treatment of hypernatremic dehydration associated with inadequate breastfeeding is important. Materials and Methods: Records of exclusively-breastfed newborns (37-42 weeks) with hypernatremic dehydration (serum Na ≥ 150mEq/L) admitted between 2006 and 2012 were reviewed retrospectively. Results: The mean gestational age, birth weight, weight loss, maternal age, and age at diagnosis of 26 newborns with hypernatremic dehydration were 38.8±1.1 weeks, 3292±458 gr, 13.5±5.5%, 27.6±4.9 years, and 3.9±3.5 days, respectively. The percentages of female patients, caesarean delivery, and primipar mothers were 57.6%, 61.6%, and 57.6% respectively. Admission complaints were fever (30.7%), poor feeding and jaundice (26.9%), restlessness and hypoactivity (7.6%). Hypernatremic dehydration frequency within first five days, in summer season, during hospitalization were 84.6%, 73%, and 42.3%, respectively. The mean serum BUN, creatinine, Na levels were found 45.6±64.1 mg/dl, 1.5±2.3 mg/dl, and 157±11.9 mEq/L, respectively. Of 26 mothers, 57.6% had received breastfeeding education and 84% had inadequate fluid intake. Among four patients with seizures, three had prerenal failure, one had renal failure requiring dialysis, and brain edema developed in one. Serum Na levels were higher in infants who were baby of primipar mother (p=0.002), born in another hospital (p=0.012), from young mothers (p=0.035), from mothers with no breastfeeding education (p=0.007), and with delayed hospital admission (p<0.01). Serum Na concentrations ≥160mEq/L were associated with complications (p<0.01). Serum Na levels were negatively correlated with maternal age (p=0.035) and positively correlated with (p=0.016) weight loss. Conclusions: Hypernatremic dehydration can be prevented in newborns by close monitoring of weight loss and by teaching successful breastfeeding techniques and signs of dehydration to the mothers within first week. © The Journal of Current Pediatrics, published by Galenos Publishing

Congenital arterial thrombosis in newborn: A case report

Neonatal thrombosis is a serious event that can cause mortality or severe morbidity. Although catheters are the most common cause of neonatal thrombosis, spontaneous events can also occur. Arterial thrombosis is very rare and accounts for approximately half of all thrombotic events in neonates. Genetic prothrombotic risk factors may affect the occurence of neonatal thrombosis. In this report, a case of left brachial, radial, and ulnar arterial thrombosis associated with methylene-tetrahydrofolate reductase (MTHFR) gene C677T and A1298C polymorphism heterozygosity is presented. Plasma homocysteine level and other prothrombotic components were normal. Standard heparin, aspirin, vitamin B12, B6 and folic acid were initiated for treatment. However, the left arm of the patient was amputated at the shoulder because its capillary stream could not be observed. We suggest that MTHFR gene C677T and A1298C polymorphism heterozygosity might be investigated in neonates with congenital arterial thrombosis in spite of normal serum homocysteine levels. © Trakya University Faculty of Medicine

Yenidoğan ratlarda oluşturulan astrosit hücre kültüründe bilirübin nörotoksisitesine rekombinant eritropoetinin etkisi

Yenidoğanlarda halen önemli bir problem olan bilirübin nörotoksisitesinin hücresel ve moleküler mekanizmaları tam olarak bilinmemektedir. Fizyolojik düzeylerde indirekt bilirübin oksidatif strese karşı koruyucu iken; orta-ağır hiperbilirübinemi oksidatif ve nitrözatif stres, lipid peroksidasyonu, proinflamatuar sitokinlerin salınımı ve ekstraselüler glutamat birikimini tetikleyerek sinir hücresinde nekroz veya apopitozise, klinik olarak akut bilirübin ensefalopatisi veya kernikterusa yol açabilmektedir. Eritropoetin (Epo), antiapopitotik, antiinflamatuar, antioksidan, antinitrozatif, anjiojenik ve nörotrofik mekanizmalarla nöronları sitotoksisiteden korumaktadır. Ancak hiperbilirübinemiye bağlı nörotoksisiteye Epo’nun etkisi henüz bilinmemektedir. Bu çalışmada primer astrosit hücre kültürü oluşturulan yenidoğan ratlarda bilirübin nörotoksisitesine Epo’nun etkisi araştırıldı. Çalışmamızda bir günlük Wistar albino ratlardan modifiye Cole ve De Vellis yöntemi ile elde edilen astrosit hücre kültürü kullanıldı. Astrosit hücrelerinin %50’sine toksik etkili olan indirekt bilirübin konsantrasyonu (TC50) 50 ?M, hücre canlılığını %100 arttıran Epo konsantrasyonu 2.5 IU/ml olarak saptandı. Bilirübin toksisitesine bağlı görülen apopitozis TUNEL boyama yöntemiyle değerlendirildi. İndirekt bilirübinden dört saat önce ve sonra Epo uygulanan astrosit hücre kültüründe 24 saat sonra apopitozis, katalaz, süperoksit dismutaz (SOD), glutatyon peroksidaz (GPx), laktat dehidrojenaz (LDH) aktivitesi, glutatyon, malondialdehid (MDA), total nitrat/nitrit, intelökin (IL)-1?, IL-6 ve TNF-? düzeyi, 72 saat sonra hücre canlılığı değerlendirildi. Bilirübin grubunda, apopitozis oranının kontrol grubundan 50 kat fazla arttığı; hücre canlılığının %75 azaldığı saptandı. Profilaktik ve tedavi edici Epo uygulaması, bilirübinin indüklediği apopitozisi, total nitrat/nitrit, IL-6, TNF-? düzeyini azaltırken; hücre canlılığı, katalaz, GPx aktivitesi ve glutatyon, IL-1? düzeyini arttırdı. Profilaktik Epo, LDH aktivitesini arttırırken; tedavi edici Epo LDH aktivitesini azalttı. Gruplar arasında SOD ve MDA düzeyleri bakımından anlamlı bir farklılık yoktu. Bu çalışmada, ilk kez profilaktik ve tedavi edici olarak uygulanan Epo’nun, antiapopitotik, antioksidan, antiinflamatuar ve antinitrozatif özellikleri ile bilirübin nörotoksisitesine karşı koruyucu etkisinin olduğu gösterildi. Cellular and molecular mechanism of bilirubin neurotoxicity, which is still an important problem of newborns, is not exactly known. While indirect bilirubin in physiological level is against oxidative stress; moderate-severe hyperbilirubinemia can cause necrosis or apoptosis in neurons, and cause clinically acute bilirubin encephalopathy or kernicterus, by inducing oxidative stress, lipid peroxidation, proinflammatory cytokine releasing and extracellular glutamate deposition. Erythropoietin (Epo) can protect the neuron cells from cytotoxicity by antiapoptotic, anti-inflammatory, antioxidant, antinitrosative, angiogenic and neurotrophic mechanisms. However, the effect of Epo on neurotoxicity caused by hyperbilirubinemia has not been known yet. In this study, the effect of Epo on bilirubin neurotoxicity in newborn rats’ primer astrocytes cell culture is investigated. In our study, astrocyte cell cultures of one-day-old Wistar albino rats were used, which were obtained from modified Cole and De Vellis method. The indirect bilirubin concentration that has toxic effect on 50% of astrocyte cells (TC50) was detected as 50 ?M and Epo concentration that increase cell viability as 100% was found 2.5 IU/ml. Apoptosis due to bilirubin toxicity was evaluated by TUNEL staining technique. After and before four hours bilirubin incubation Epo treatment was administered. In astrocyte cell cultures, apoptosis, catalase, superoxide dismutase (SOD), glutathione peroxidase (GPx), lactate dehydrogenase (LDH) activities, glutathione, malondialdehyde (MDA), total nitrate/nitrite, interleukin (IL)- 1?, IL-6 and TNF-? levels were investigated after 24 hours of bilirubin incubation and also cell viability after 72 hours was evaluated. In bilirubin group, it was detected that cell viability decreased in 75% and that apoptosis rate increased in fifty times more than the control group. Although prophylactic and therapeutic Epo application decreased apoptosis, total nitrate/nitrite, IL-6, and TNF-? levels induced by bilirubin, it increased cell viability, catalase and GPx activities, glutathione and IL-1? levels. While prophylactic Epo increased LDH activity; therapeutic Epo decreased it. There was not any significantly difference in between the groups in terms of SOD and MDA levels. In this study, it was the first time shown that prophylactic and therapeutic Epo had protective effects on bilirubin neurotoxicity because of antiapoptotic, antioxidant, antiinflammatory and antinitrosative properties

Akut infantil hemorajik ödem

Akut infantil hemorajik ödem iki yaş altında görülen, purpurik döküntü, ateş ve ödemle karakterize bir vaskülittir. Hastalık dramatik bir tablo izlenimi vermekle birlikte, klinik gidiş iyidir. Bu çalışmada ateş, her iki ayakta ödem ve alt extremitelerde purpurik döküntü nedeniyle acil servise başvuran bir olgu sunulmuştur

Pseudohypoaldosteronism: Case Report

Introduction: Pseudohypoaldosteronism is a disease which occurs as a result of peripheral resistance to aldosterone and is characterised by salt wasting. Case Report: Hyponatremia, hyperkalemia, metabolic acidosis, high renin and aldosterone levels were determined in the patient admitted with decrease in sucking and getting sleepy during breast-feeding. The case was diagnosed as systemic form of pseudohypoaldosteronism. His treatment was continued with oral salt. Conclusion: Pseudohypoaldosteronism has three types as primary, secondary and Gordon syndrome. Primary form is due to epithelial sodium channel and mineralocorticoid receptor gene mutation, whilst secondary form is frequently caused by urinary malformation and urinary tract infections. In Gordon syndrome, plasma aldosterone level is usuallly normal, and plasma renin activity is depressed, there is an adequate response to mineralocorticoids. Probability of secondary pseudohypoaldosteronism was investigated at the same time because of the urinary infection present in our subject. The patient was accepted as systemic form of primary pseudohypoaldosteronism because of positive sweat test result and prolonged salt necessity. © The Journal of Current Pediatrics, published by Galenos Publishing. All rights reserved

Psödohipoaldosteronizm: Olgu sunumu

Giriş: Psödohipoaldosteronizm, aldosterona periferik direnç sonucu gelişen ve tuz kaybı ile karakterize bir hastalıktır. Olgu Sunumu: Emmede azalma, emerken uyuklama şikayetiyle getirilen hastada hiponatremi, hiperkalemi, metabolik asidoz, yüksek renin ve aldosteron düzeyi saptandı. Olguya sistemik form psödohipoaldosteronizm tanısı konuldu. Oral tuz ile tedavisine devam edildi. Tartışma: Psödohipoaldosteronizm primer, sekonder ve Gordon sendromu olarak üç tiptir. Primer form epitelyal sodyum kanalı ve mineralokortikoid reseptör genindeki mutasyondan, sekonder form sıklıkla üriner malformasyon ve idrar yolu enfeksiyonlarından kaynaklanır. Gordon sendromunda ise plazma aldosteron düzeyi genellikle normal olup, mineralokortikoidlere yeterli cevap vardır ve plazma renin aktivitesi baskılanmıştır. Olgumuzda üriner enfeksiyon saptanması nedeniyle aynı zamanda sekonder psödohipoaldosteronizm olasılığı araştırıldı. Ter testinin pozitif olması ve tuz ihtiyacının uzun süre devam etmesi nedeniyle hasta sistemik form primer psödohipoaldosteronizm olarak kabul edildi

Pseudohypoaldosteronism: Case report

Giriş: Psödohipoaldosteronizm, aldosterona periferik direnç sonucu gelişen ve tuz kaybı ile karakterize bir hastalıktır. Olgu Sunumu: Emmede azalma, emerken uyuklama şikayetiyle getirilen hastada hiponatremi, hiperkalemi, metabolik asidoz, yüksek renin ve aldosteron düzeyi saptandı. Olguya sistemik form psödohipoaldosteronizm tanısı konuldu. Oral tuz ile tedavisine devam edildi. Tartışma: Psödohipoaldosteronizm primer, sekonder ve Gordon sendromu olarak üç tiptir. Primer form epitelyal sodyum kanalı ve mineralokortikoid reseptör genindeki mutasyondan, sekonder form sıklıkla üriner malformasyon ve idrar yolu enfeksiyonlarından kaynaklanır. Gordon sendromunda ise plazma aldosteron düzeyi genellikle normal olup, mineralokortikoidlere yeterli cevap vardır ve plazma renin aktivitesi baskılanmıştır. Olgumuzda üriner enfeksiyon saptanması nedeniyle aynı zamanda sekonder psödohipoaldosteronizm olasılı¤ı araştırıldı. Ter testinin pozitif olması ve tuz ihtiyacının uzun süre devam etmesi nedeniyle hasta sistemik form primer psödohipoaldosteronizm olarak kabul edildi.Introduction: Pseudohypoaldosteronism is a disease which occurs as a result of peripheral resistance to aldosterone and is characterised by salt wasting. Case Report: Hyponatremia, hyperkalemia, metabolic acidosis, high renin and aldosterone levels were determined in the patient admitted with decrease in sucking and getting sleepy during breast-feeding. The case was diagnosed as systemic form of pseudohypoaldosteronism. His treatment was continued with oral salt. Conclusion: Pseudohypoaldosteronism has three types as primary, secondary and Gordon syndrome. Primary form is due to epithelial sodium channel and mineralocorticoid receptor gene mutation, whilst secondary form is frequently caused by urinary malformation and urinary tract infections. In Gordon syndrome, plasma aldosterone level is usuallly normal, and plasma renin activity is depressed, there is an adequate response to mineralocorticoids. Probability of secondary pseudohypoaldosteronism was investigated at the same time because of the urinary infection present in our subject. The patient was accepted as systemic form of primary pseudohypoaldosteronism because of positive sweat test result and prolonged salt necessity