The aim of the present study is to clarify the low density lipoprotein apheresis procedure for pe... more The aim of the present study is to clarify the low density lipoprotein apheresis procedure for pediatric patients with homozygous familial hypercholesterolemia (FH) in terms of efficacy, adverse effects and difficulties. The follow-up was carried out using an open, prospective uncontrolled clinical design. Data were collected from 10 patients (with an average age of 8.4 Ϯ 4.7 years) with FH treated with double filtration plasmapheresis. The total time span of follow-up covered five years (30.2 Ϯ 17.8 months [range 9-60 months]) and more than 600 sessions (62.1 Ϯ 35.5 sessions per patient [range 18-120 sessions]) were evaluated. The mean low density lipoprotein cholesterol (LDL-C) pre-treatment value was 375.5 Ϯ 127.5 mg/dL, and the post-treatment value was 147.5 Ϯ 73.9 mg/dL. This corresponded to a 62.8 Ϯ 10.3% (43-73%) acute reduction of LDL-C, while the mean high density lipoprotein cholesterol losses amounted to 41%. The chronic reduction in LDL-C ranged from 18 to 52%, with a mean level of 36.4 Ϯ 11.7%. The most frequently occurring technical problems were related to blood lines: puncture difficulties (4.5%), insufficient blood flow (3.5%), and obturation of the blood lines (2.4%). The main clinical adverse effects were hypotension (0.2%), chills/feeling cold (0.1%), and nausea and vomiting (0.2%). We observed that the low pediatric patient tolerance is the main problem in compliance with treatment. In conclusion, LDL apheresis, started under the age of eight years, combined with lipidlowering drugs, provides a safe and effective lowering of the mean LDL-C levels in pediatric homozygous FH; and there are more problems with compliance for pediatric LDL apheresis than in the adult population.
Griscelli Hastalýðý otozomal resesif geçiþli nadir bir hastalýk olup, açýk ten rengi, gümüþ grisi... more Griscelli Hastalýðý otozomal resesif geçiþli nadir bir hastalýk olup, açýk ten rengi, gümüþ grisi saçlar, tekrarlayan ateþ ataklarý ve enfeksiyonlarla karakterizedir. Sendromda ayrýca deðiþik derecelerde immun yetersizlik ve geniþ bir spektrum içerisinde nörolojik bozukluklar görülebilir. Bu yazýda ateþ yüksekliði ve öksürük yakýnmalarýyla baþvuran 3.5 aylýk bir kýz olgu sunulmuþtur. Öyküsünden, iki ay önce idrar yolu enfeksiyonu nedeniyle hastanede yatýrýldýðý, 3. dereceden akraba bir anne-babanýn, 2. çocuklarý olup, ilk çocuklarýnýn da benzer fizik özelliklere sahip olduðu, 5 aylýkken ateþ nedeniyle yatýrýldýðý hastanede eksitus olduðu öðrenildi. Fizik bakýsýnda; geliþimi normal, ateþ 37.8 C(aksiller), cildinin açýk renkli, saçlarýnýn gümüþ grisi renkte, karaciðer ve dalaðýn midklavikuler hatta 3'er cm palpabl olmasý dýþýnda, diðer sistem bakýlarý olaðan olarak deðerlendirildi. Rutin laboratuvar incelemelerinde, lökopeni dýþýnda patoloji saptanmadý. Kemik iliði aspirasyonu normal, lökopeni sepsise sekonder olarak deðerlendirildi. Serum immunoglobulinleri normal, lenfosit panelinde; CD19: %11 (ND:31-48) olarak düþük bulundu. Soygeçmiþinde kardeþ öyküsü olmasý ve hastanýn fenotipik özellikleri ile Griscelli Hastalýðý düþünüldü. Ýkili antibiyotik tedavisinin 4. gününde ateþ yüksekliðine hakim olundu ve pansitopenisinde de düzelme saptandý. Taný saç telinin ýþýk mikroskobik incelemesi ve kliniðe dayalý olarak kondu ve hasta kemik iliði transplantasyonu yapýlan bir merkeze sevk edildi. Tekrarlayan enfeksiyon etyolojisinde, belirgin fenotipik özellikler gö steren olgularda Griscelli Sendromu da düþünülmelidir. Griscelli, gümüþ grisi saç, açýk ten rengi
... Bronşektazili 50 Pediatrik Olgunun Değerlendirilmesi. Hakan Gerçek, Demet Can, Serdar Altınöz... more ... Bronşektazili 50 Pediatrik Olgunun Değerlendirilmesi. Hakan Gerçek, Demet Can, Serdar Altınöz,Gökmen Bilgili, Saniye Gülle, Sema Kalkan, Pelin Akgün Gerçek, Mustafa Bak. ... En sık başvuru yakınmaları öksürük (%54.3) ve ateş (%48.5) olarak saptanmıştır. ...
Primary IgA nephropathy is a disease characterized by recurrent macroscopic or microscopic hematu... more Primary IgA nephropathy is a disease characterized by recurrent macroscopic or microscopic hematuria and diffuse mesangial IgA deposition. Although IgA nephropathy had previously been suggested to have a benign prognosis, long term follow-up of the patients revealed that it might lead to chronic renal failure. In this study, the association of the initial clinical and laboratory findings with the renal histological changes was evaluated in 14 cases with primary IgA nephropathy who were at follow-up with a mean duration of 43.07 + 16.88 months. Finally the correlation between the clinicopathological findings and prognosis was investigated. In 92.8% of the patients, macroscopic hematuria was the presenting complaint. Proteinuria was detected in 42.9% of the cases mild proteinuria in 14.3% and moderate in 28.6%. Renal biopsy specimens, evaluated according to Churg-Sobin's classification, showed grade 1 changes in 35.7% cases, grade 2 in 35.7%, grade 3 in 14.3% and grade 4 in 14.3%. Both the patients with grade 4 histology had moderate proteinuria, and developed chronic renal failure requiring hemodialysis. Prognosis was found to be associated with the degree of proteinuria and the severity of the histopathological findings.
Turk Pediatri Arsivi-turkish Archives of Pediatrics, 1993
This study was performed to determine the prevalence hepatitis B surfage antigen other hepatitis ... more This study was performed to determine the prevalence hepatitis B surfage antigen other hepatitis B markers anti HCV antibodies the variations during the course of haemodialysis and immun response to a recombinant hepatitis B vaccine in children with chronic renal failure on haemodialysis nbsp; 54 patients 26 males and 28 females median age 12 1±2 7 years range 5 16 years treated with haemodialysis for median of 12 9±13 9 months were studied All hepatitis markers were detected in serum by an enzyme immunassey in the beginning of haemodialysis and 3 6 months interval during the period of haemodialysis In the beginning of haemodialysis 5 of the patients 9 2 were HBsAg positive 17 31 5 HBsAg negative anti HBs positive and or anti HBc positive nbsp; 33 patients were evaluated for anti HCV antibodies and 9 of these patients 30 3 were anti HCV positive One of the seronegative patients treated with haemodialysis for three years developed hepatitis B infection but none of the seronegative patients developed hepatitis C infection 8 patients with negative hepatitis B markers were immunized with three doses of 20 micrograms of hepatitis B vaccine administered at monthly intervals 5 patients 62 5 developed seroconversion after three vaccinations Key words: Hemodialysis Hepatitis B Hepatitis C Hepatitis B Vaccine
İzmir Dr.Behçet Uz çocuk hastanesi dergisi, Apr 5, 2013
Amaç: Bu çalışmada kolestaz saptanan yenidoğanlardaki karaciğer biyopsi bulgularının değerlendiri... more Amaç: Bu çalışmada kolestaz saptanan yenidoğanlardaki karaciğer biyopsi bulgularının değerlendirilmesi ve biliyer atrezi ile neonatal hepatit prevalansının belirlenmesi amaçlanmıştır. Gereç ve Yöntem: İzmir Dr. Behçet Uz Çocuk Hastanesi'nde 1992 ile 2011 yılları arasında neonatal kolestaz nedeniyle karaciğer biyopsisi yapılan 153 bebek (0-4 ay) retrospektif olarak incelenmiştir. Bebeklerin biyopsi yapıldığı tarihte gün olarak yaşı, cinsiyeti ve radyolojik inceleme bulguları benzeri parametrelerin birbiriyle ilişkisi değerlendirilmiştir. Bulgular: Olguların 93'ü erkek (%60,8), 60'ı kız (%39,2) olup, ortalama yaş 73,8±30,5 gün (10-120 gün) bulunmuştur. Yüz on yedi olguda (%76,5) ayırıcı tanı tek biyopside yapılmıştır. Histopatolojik olarak 82 bebek (%53,6) neonatal hepatit, 71 bebek (%46,4) biliyer atreziyle uyumlu olarak değerlendirilmiştir. Kasai operasyonu biliyer atrezili 23 olguda (%32,4), karaciğer transplantasyonu 11 olguda (%15,4) uygulanmıştır. Olguların yaşı ile hastalık arasında istatistiksel anlamlı ilişki saptanmıştır (p=0,010). Tartışma: Bu çalışmada 0-4 aylık neonatal kolestazlı bebeklerin karaciğer biyopsi bulgularının kapsamlı profili çıkartılmıştır. Bu hastalıklarda ayırıcı tanı için ipuçları tartışılmıştır.
Anemi sınırı olarak İVHO'nun önerdiği Hh< llg/dl değeri göz önüne alındığında olguların %78'inde ... more Anemi sınırı olarak İVHO'nun önerdiği Hh< llg/dl değeri göz önüne alındığında olguların %78'inde anemi sap kındı. Anemi prevalansı %84.5 ile en yüksek orunda 13-24 ay arası yaş grubunda izlendi. Anemi ve malnütrisyon prevalansının bu denli yüksek oluşu toplumumuzun sosyoekonomik koşulları orta ve kötü olan kesimindeki sağlık problemlerinin boyutunu göstermesi bakımından anlamlı bulundu.
Journal of Dr. Behcet Uz Children s Hospital, 2014
OBJECTIVE: In preschool children, diagnosis of asthma is difficult because of not using spirometr... more OBJECTIVE: In preschool children, diagnosis of asthma is difficult because of not using spirometry objectively. instead of spirometry, other techiques that not required sedation and cooperation to evaluate pulmonary functions. Interrupter technique that measures the resistance of the airway, is one of these methods. Insufficient of references values hamper the use of this technique commonly. In this study,to determine the referencesal values of Turkish preschool children for interrupter technique is intended. METHODS: 50 healthy children who applied between January 2008 and September 2008 to the Behcet Uz Children Hospital, were accepted for this study. During the expiratory phase of tidal breathing interrupter technique for airway resistance measurements were performed. At least 5 measurements were made for each case, and median values of these measurements were the base. References values were determined according to age and height. RESULTS: There were 28 boys (%56), 22 girls (%44...
Introduction and Aims: Hepcidin synthesis occurs in response to iron overload, inflammation and i... more Introduction and Aims: Hepcidin synthesis occurs in response to iron overload, inflammation and inhibition of erythropoiesis in patients with hemodialysis (HD). In this study, we investigated the early response of hepcidin-25 (hep) to administration of short-acting epoetin-beta (epo) and long-acting methoxy polyethylene glycol-epoetin-beta (PEG-epo) and whether these erythropoietin drugs (rHuEPO) make a difference in ferrokinetics of HD patients. Methods: The study included 9 patients with HD but no diabetes mellitus (4 men and 5 women with a mean age of 59 ± 7.0 years and a mean duration of HD of 120 ± 95 months). Serum levels of hep were measured before and 3, 6, 9 and 18 hours after intravenous injection with either epo or PEG-epo. The measurements of hep in response to epo or PEG-epo were performed using the same patients separately at an interval of 2 years. The hep level was measured using a high-throughput LC-MS/MS method. Serum levels of ferritin, haemoglobin (Hb), and CRP were measured at the start of dialysis. Data are expressed as mean ± SD. Statistical analysis was performed using the Mann-Whitney U test or Fisher`s exact probability test. Results: Serum ferritin level was 38.2 ± 50.3ng/mL when epo was given, which did not differ significantly from that (15.3 ± 11.1ng/mL) when PEG-epo was given. The hep level was significantly increased from the basal level of 6.8 ± 9.8ng/mL to 15.9 ± 20.9ng/mL at 6 hours after epo administration, and decreased significantly at 18 hours (4.6 ± 8.7 ng/mL). The hep level was significantly increased from the basal level of 10.9 ± 13.6 to 25.0 ± 29.3ng/mL at 6 hours after PEG-epo administration, and decreased at 18 hours (15.2 ± 18.1 ng/mL). There was no difference in the time-course effects of epo and PEG-epo on serum hep levels. Based on the basal ferritin level, we divided 18 samples into two groups; high ferritin group: 7 samples with serum fer level of > 15.0 ng/mL and lower ferritin group: 11 samples with lower limits of normal. In the lower ferritin group, the hep level was 5.0 ng/mL or less at all 5 measurement time points. However, in the high ferritin group, the hep level was significantly increased from the basal level of 19.8 ± 12.0 to 46.8 ± 19.8 ng/mL at 6 hours after rHuEPO administration. There was no difference in the number of samples taken from the patients receiving PEG-epo between the two groups. The Hb levels did not differ between the low ferritin group (10.6 ± 2.6g/dL) and the high ferritin group (11.2 ± 0.7g/dL). The values for CRP were < 0.1 mg/dl at all measurements. Conclusions: Our data suggest that up-regulation of hepcidin-25 in the early phase of rHuEPO-induced erythropoiesis, which occurs regardless of short-or long-acting agents, is dependent on serum ferritin levels in HD patients.
Griscelli Hastalýðý otozomal resesif geçiþli nadir bir hastalýk olup, açýk ten rengi, gümüþ grisi... more Griscelli Hastalýðý otozomal resesif geçiþli nadir bir hastalýk olup, açýk ten rengi, gümüþ grisi saçlar, tekrarlayan ateþ ataklarý ve enfeksiyonlarla karakterizedir. Sendromda ayrýca deðiþik derecelerde immun yetersizlik ve geniþ bir spektrum içerisinde nörolojik bozukluklar görülebilir. Bu yazýda ateþ yüksekliði ve öksürük yakýnmalarýyla baþvuran 3.5 aylýk bir kýz olgu sunulmuþtur. Öyküsünden, iki ay önce idrar yolu enfeksiyonu nedeniyle hastanede yatýrýldýðý, 3. dereceden akraba bir anne-babanýn, 2. çocuklarý olup, ilk çocuklarýnýn da benzer fizik özelliklere sahip olduðu, 5 aylýkken ateþ nedeniyle yatýrýldýðý hastanede eksitus olduðu öðrenildi. Fizik bakýsýnda; geliþimi normal, ateþ 37.8 C(aksiller), cildinin açýk renkli, saçlarýnýn gümüþ grisi renkte, karaciðer ve dalaðýn midklavikuler hatta 3'er cm palpabl olmasý dýþýnda, diðer sistem bakýlarý olaðan olarak deðerlendirildi. Rutin laboratuvar incelemelerinde, lökopeni dýþýnda patoloji saptanmadý. Kemik iliði aspirasyonu normal, lökopeni sepsise sekonder olarak deðerlendirildi. Serum immunoglobulinleri normal, lenfosit panelinde; CD19: %11 (ND:31-48) olarak düþük bulundu. Soygeçmiþinde kardeþ öyküsü olmasý ve hastanýn fenotipik özellikleri ile Griscelli Hastalýðý düþünüldü. Ýkili antibiyotik tedavisinin 4. gününde ateþ yüksekliðine hakim olundu ve pansitopenisinde de düzelme saptandý. Taný saç telinin ýþýk mikroskobik incelemesi ve kliniðe dayalý olarak kondu ve hasta kemik iliði transplantasyonu yapýlan bir merkeze sevk edildi. Tekrarlayan enfeksiyon etyolojisinde, belirgin fenotipik özellikler gö steren olgularda Griscelli Sendromu da düþünülmelidir. Griscelli, gümüþ grisi saç, açýk ten rengi
The aim of the present study is to clarify the low density lipoprotein apheresis procedure for pe... more The aim of the present study is to clarify the low density lipoprotein apheresis procedure for pediatric patients with homozygous familial hypercholesterolemia (FH) in terms of efficacy, adverse effects and difficulties. The follow-up was carried out using an open, prospective uncontrolled clinical design. Data were collected from 10 patients (with an average age of 8.4 Ϯ 4.7 years) with FH treated with double filtration plasmapheresis. The total time span of follow-up covered five years (30.2 Ϯ 17.8 months [range 9-60 months]) and more than 600 sessions (62.1 Ϯ 35.5 sessions per patient [range 18-120 sessions]) were evaluated. The mean low density lipoprotein cholesterol (LDL-C) pre-treatment value was 375.5 Ϯ 127.5 mg/dL, and the post-treatment value was 147.5 Ϯ 73.9 mg/dL. This corresponded to a 62.8 Ϯ 10.3% (43-73%) acute reduction of LDL-C, while the mean high density lipoprotein cholesterol losses amounted to 41%. The chronic reduction in LDL-C ranged from 18 to 52%, with a mean level of 36.4 Ϯ 11.7%. The most frequently occurring technical problems were related to blood lines: puncture difficulties (4.5%), insufficient blood flow (3.5%), and obturation of the blood lines (2.4%). The main clinical adverse effects were hypotension (0.2%), chills/feeling cold (0.1%), and nausea and vomiting (0.2%). We observed that the low pediatric patient tolerance is the main problem in compliance with treatment. In conclusion, LDL apheresis, started under the age of eight years, combined with lipidlowering drugs, provides a safe and effective lowering of the mean LDL-C levels in pediatric homozygous FH; and there are more problems with compliance for pediatric LDL apheresis than in the adult population.
Griscelli Hastalýðý otozomal resesif geçiþli nadir bir hastalýk olup, açýk ten rengi, gümüþ grisi... more Griscelli Hastalýðý otozomal resesif geçiþli nadir bir hastalýk olup, açýk ten rengi, gümüþ grisi saçlar, tekrarlayan ateþ ataklarý ve enfeksiyonlarla karakterizedir. Sendromda ayrýca deðiþik derecelerde immun yetersizlik ve geniþ bir spektrum içerisinde nörolojik bozukluklar görülebilir. Bu yazýda ateþ yüksekliði ve öksürük yakýnmalarýyla baþvuran 3.5 aylýk bir kýz olgu sunulmuþtur. Öyküsünden, iki ay önce idrar yolu enfeksiyonu nedeniyle hastanede yatýrýldýðý, 3. dereceden akraba bir anne-babanýn, 2. çocuklarý olup, ilk çocuklarýnýn da benzer fizik özelliklere sahip olduðu, 5 aylýkken ateþ nedeniyle yatýrýldýðý hastanede eksitus olduðu öðrenildi. Fizik bakýsýnda; geliþimi normal, ateþ 37.8 C(aksiller), cildinin açýk renkli, saçlarýnýn gümüþ grisi renkte, karaciðer ve dalaðýn midklavikuler hatta 3'er cm palpabl olmasý dýþýnda, diðer sistem bakýlarý olaðan olarak deðerlendirildi. Rutin laboratuvar incelemelerinde, lökopeni dýþýnda patoloji saptanmadý. Kemik iliði aspirasyonu normal, lökopeni sepsise sekonder olarak deðerlendirildi. Serum immunoglobulinleri normal, lenfosit panelinde; CD19: %11 (ND:31-48) olarak düþük bulundu. Soygeçmiþinde kardeþ öyküsü olmasý ve hastanýn fenotipik özellikleri ile Griscelli Hastalýðý düþünüldü. Ýkili antibiyotik tedavisinin 4. gününde ateþ yüksekliðine hakim olundu ve pansitopenisinde de düzelme saptandý. Taný saç telinin ýþýk mikroskobik incelemesi ve kliniðe dayalý olarak kondu ve hasta kemik iliði transplantasyonu yapýlan bir merkeze sevk edildi. Tekrarlayan enfeksiyon etyolojisinde, belirgin fenotipik özellikler gö steren olgularda Griscelli Sendromu da düþünülmelidir. Griscelli, gümüþ grisi saç, açýk ten rengi
... Bronşektazili 50 Pediatrik Olgunun Değerlendirilmesi. Hakan Gerçek, Demet Can, Serdar Altınöz... more ... Bronşektazili 50 Pediatrik Olgunun Değerlendirilmesi. Hakan Gerçek, Demet Can, Serdar Altınöz,Gökmen Bilgili, Saniye Gülle, Sema Kalkan, Pelin Akgün Gerçek, Mustafa Bak. ... En sık başvuru yakınmaları öksürük (%54.3) ve ateş (%48.5) olarak saptanmıştır. ...
Primary IgA nephropathy is a disease characterized by recurrent macroscopic or microscopic hematu... more Primary IgA nephropathy is a disease characterized by recurrent macroscopic or microscopic hematuria and diffuse mesangial IgA deposition. Although IgA nephropathy had previously been suggested to have a benign prognosis, long term follow-up of the patients revealed that it might lead to chronic renal failure. In this study, the association of the initial clinical and laboratory findings with the renal histological changes was evaluated in 14 cases with primary IgA nephropathy who were at follow-up with a mean duration of 43.07 + 16.88 months. Finally the correlation between the clinicopathological findings and prognosis was investigated. In 92.8% of the patients, macroscopic hematuria was the presenting complaint. Proteinuria was detected in 42.9% of the cases mild proteinuria in 14.3% and moderate in 28.6%. Renal biopsy specimens, evaluated according to Churg-Sobin's classification, showed grade 1 changes in 35.7% cases, grade 2 in 35.7%, grade 3 in 14.3% and grade 4 in 14.3%. Both the patients with grade 4 histology had moderate proteinuria, and developed chronic renal failure requiring hemodialysis. Prognosis was found to be associated with the degree of proteinuria and the severity of the histopathological findings.
Turk Pediatri Arsivi-turkish Archives of Pediatrics, 1993
This study was performed to determine the prevalence hepatitis B surfage antigen other hepatitis ... more This study was performed to determine the prevalence hepatitis B surfage antigen other hepatitis B markers anti HCV antibodies the variations during the course of haemodialysis and immun response to a recombinant hepatitis B vaccine in children with chronic renal failure on haemodialysis nbsp; 54 patients 26 males and 28 females median age 12 1±2 7 years range 5 16 years treated with haemodialysis for median of 12 9±13 9 months were studied All hepatitis markers were detected in serum by an enzyme immunassey in the beginning of haemodialysis and 3 6 months interval during the period of haemodialysis In the beginning of haemodialysis 5 of the patients 9 2 were HBsAg positive 17 31 5 HBsAg negative anti HBs positive and or anti HBc positive nbsp; 33 patients were evaluated for anti HCV antibodies and 9 of these patients 30 3 were anti HCV positive One of the seronegative patients treated with haemodialysis for three years developed hepatitis B infection but none of the seronegative patients developed hepatitis C infection 8 patients with negative hepatitis B markers were immunized with three doses of 20 micrograms of hepatitis B vaccine administered at monthly intervals 5 patients 62 5 developed seroconversion after three vaccinations Key words: Hemodialysis Hepatitis B Hepatitis C Hepatitis B Vaccine
İzmir Dr.Behçet Uz çocuk hastanesi dergisi, Apr 5, 2013
Amaç: Bu çalışmada kolestaz saptanan yenidoğanlardaki karaciğer biyopsi bulgularının değerlendiri... more Amaç: Bu çalışmada kolestaz saptanan yenidoğanlardaki karaciğer biyopsi bulgularının değerlendirilmesi ve biliyer atrezi ile neonatal hepatit prevalansının belirlenmesi amaçlanmıştır. Gereç ve Yöntem: İzmir Dr. Behçet Uz Çocuk Hastanesi'nde 1992 ile 2011 yılları arasında neonatal kolestaz nedeniyle karaciğer biyopsisi yapılan 153 bebek (0-4 ay) retrospektif olarak incelenmiştir. Bebeklerin biyopsi yapıldığı tarihte gün olarak yaşı, cinsiyeti ve radyolojik inceleme bulguları benzeri parametrelerin birbiriyle ilişkisi değerlendirilmiştir. Bulgular: Olguların 93'ü erkek (%60,8), 60'ı kız (%39,2) olup, ortalama yaş 73,8±30,5 gün (10-120 gün) bulunmuştur. Yüz on yedi olguda (%76,5) ayırıcı tanı tek biyopside yapılmıştır. Histopatolojik olarak 82 bebek (%53,6) neonatal hepatit, 71 bebek (%46,4) biliyer atreziyle uyumlu olarak değerlendirilmiştir. Kasai operasyonu biliyer atrezili 23 olguda (%32,4), karaciğer transplantasyonu 11 olguda (%15,4) uygulanmıştır. Olguların yaşı ile hastalık arasında istatistiksel anlamlı ilişki saptanmıştır (p=0,010). Tartışma: Bu çalışmada 0-4 aylık neonatal kolestazlı bebeklerin karaciğer biyopsi bulgularının kapsamlı profili çıkartılmıştır. Bu hastalıklarda ayırıcı tanı için ipuçları tartışılmıştır.
Anemi sınırı olarak İVHO'nun önerdiği Hh< llg/dl değeri göz önüne alındığında olguların %78'inde ... more Anemi sınırı olarak İVHO'nun önerdiği Hh< llg/dl değeri göz önüne alındığında olguların %78'inde anemi sap kındı. Anemi prevalansı %84.5 ile en yüksek orunda 13-24 ay arası yaş grubunda izlendi. Anemi ve malnütrisyon prevalansının bu denli yüksek oluşu toplumumuzun sosyoekonomik koşulları orta ve kötü olan kesimindeki sağlık problemlerinin boyutunu göstermesi bakımından anlamlı bulundu.
Journal of Dr. Behcet Uz Children s Hospital, 2014
OBJECTIVE: In preschool children, diagnosis of asthma is difficult because of not using spirometr... more OBJECTIVE: In preschool children, diagnosis of asthma is difficult because of not using spirometry objectively. instead of spirometry, other techiques that not required sedation and cooperation to evaluate pulmonary functions. Interrupter technique that measures the resistance of the airway, is one of these methods. Insufficient of references values hamper the use of this technique commonly. In this study,to determine the referencesal values of Turkish preschool children for interrupter technique is intended. METHODS: 50 healthy children who applied between January 2008 and September 2008 to the Behcet Uz Children Hospital, were accepted for this study. During the expiratory phase of tidal breathing interrupter technique for airway resistance measurements were performed. At least 5 measurements were made for each case, and median values of these measurements were the base. References values were determined according to age and height. RESULTS: There were 28 boys (%56), 22 girls (%44...
Introduction and Aims: Hepcidin synthesis occurs in response to iron overload, inflammation and i... more Introduction and Aims: Hepcidin synthesis occurs in response to iron overload, inflammation and inhibition of erythropoiesis in patients with hemodialysis (HD). In this study, we investigated the early response of hepcidin-25 (hep) to administration of short-acting epoetin-beta (epo) and long-acting methoxy polyethylene glycol-epoetin-beta (PEG-epo) and whether these erythropoietin drugs (rHuEPO) make a difference in ferrokinetics of HD patients. Methods: The study included 9 patients with HD but no diabetes mellitus (4 men and 5 women with a mean age of 59 ± 7.0 years and a mean duration of HD of 120 ± 95 months). Serum levels of hep were measured before and 3, 6, 9 and 18 hours after intravenous injection with either epo or PEG-epo. The measurements of hep in response to epo or PEG-epo were performed using the same patients separately at an interval of 2 years. The hep level was measured using a high-throughput LC-MS/MS method. Serum levels of ferritin, haemoglobin (Hb), and CRP were measured at the start of dialysis. Data are expressed as mean ± SD. Statistical analysis was performed using the Mann-Whitney U test or Fisher`s exact probability test. Results: Serum ferritin level was 38.2 ± 50.3ng/mL when epo was given, which did not differ significantly from that (15.3 ± 11.1ng/mL) when PEG-epo was given. The hep level was significantly increased from the basal level of 6.8 ± 9.8ng/mL to 15.9 ± 20.9ng/mL at 6 hours after epo administration, and decreased significantly at 18 hours (4.6 ± 8.7 ng/mL). The hep level was significantly increased from the basal level of 10.9 ± 13.6 to 25.0 ± 29.3ng/mL at 6 hours after PEG-epo administration, and decreased at 18 hours (15.2 ± 18.1 ng/mL). There was no difference in the time-course effects of epo and PEG-epo on serum hep levels. Based on the basal ferritin level, we divided 18 samples into two groups; high ferritin group: 7 samples with serum fer level of > 15.0 ng/mL and lower ferritin group: 11 samples with lower limits of normal. In the lower ferritin group, the hep level was 5.0 ng/mL or less at all 5 measurement time points. However, in the high ferritin group, the hep level was significantly increased from the basal level of 19.8 ± 12.0 to 46.8 ± 19.8 ng/mL at 6 hours after rHuEPO administration. There was no difference in the number of samples taken from the patients receiving PEG-epo between the two groups. The Hb levels did not differ between the low ferritin group (10.6 ± 2.6g/dL) and the high ferritin group (11.2 ± 0.7g/dL). The values for CRP were < 0.1 mg/dl at all measurements. Conclusions: Our data suggest that up-regulation of hepcidin-25 in the early phase of rHuEPO-induced erythropoiesis, which occurs regardless of short-or long-acting agents, is dependent on serum ferritin levels in HD patients.
Griscelli Hastalýðý otozomal resesif geçiþli nadir bir hastalýk olup, açýk ten rengi, gümüþ grisi... more Griscelli Hastalýðý otozomal resesif geçiþli nadir bir hastalýk olup, açýk ten rengi, gümüþ grisi saçlar, tekrarlayan ateþ ataklarý ve enfeksiyonlarla karakterizedir. Sendromda ayrýca deðiþik derecelerde immun yetersizlik ve geniþ bir spektrum içerisinde nörolojik bozukluklar görülebilir. Bu yazýda ateþ yüksekliði ve öksürük yakýnmalarýyla baþvuran 3.5 aylýk bir kýz olgu sunulmuþtur. Öyküsünden, iki ay önce idrar yolu enfeksiyonu nedeniyle hastanede yatýrýldýðý, 3. dereceden akraba bir anne-babanýn, 2. çocuklarý olup, ilk çocuklarýnýn da benzer fizik özelliklere sahip olduðu, 5 aylýkken ateþ nedeniyle yatýrýldýðý hastanede eksitus olduðu öðrenildi. Fizik bakýsýnda; geliþimi normal, ateþ 37.8 C(aksiller), cildinin açýk renkli, saçlarýnýn gümüþ grisi renkte, karaciðer ve dalaðýn midklavikuler hatta 3'er cm palpabl olmasý dýþýnda, diðer sistem bakýlarý olaðan olarak deðerlendirildi. Rutin laboratuvar incelemelerinde, lökopeni dýþýnda patoloji saptanmadý. Kemik iliði aspirasyonu normal, lökopeni sepsise sekonder olarak deðerlendirildi. Serum immunoglobulinleri normal, lenfosit panelinde; CD19: %11 (ND:31-48) olarak düþük bulundu. Soygeçmiþinde kardeþ öyküsü olmasý ve hastanýn fenotipik özellikleri ile Griscelli Hastalýðý düþünüldü. Ýkili antibiyotik tedavisinin 4. gününde ateþ yüksekliðine hakim olundu ve pansitopenisinde de düzelme saptandý. Taný saç telinin ýþýk mikroskobik incelemesi ve kliniðe dayalý olarak kondu ve hasta kemik iliði transplantasyonu yapýlan bir merkeze sevk edildi. Tekrarlayan enfeksiyon etyolojisinde, belirgin fenotipik özellikler gö steren olgularda Griscelli Sendromu da düþünülmelidir. Griscelli, gümüþ grisi saç, açýk ten rengi
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