Bu çalýþmada, kan basýncýndaki akut düþmenin sol ventrikül diyastolik fonksiyonlarýna etkisini araþtýrmayý amaçladýk.

Çalýþmaya, sistolik kan basýnçlarý >180mmHg ve/veya diyastolik kan basýnçlarý >110 mmHg olan, EKG’lerinde patolojik Q dalgasý bulunmayan, sinüs ritmindeki 12 erkek, 21 kadýn olmak üzere toplam 33 olgu alýndý. Hastalar sol ventrikül duvar kalýnlýklarýna göre hipertrofik olan (>12 mm) ve hipertrofik olmayan (<12 mm) olmak üzere 2 gruba ayrýldý. Tüm hastalarýn sublingual kaptopril ile kan basýnçlarýnda düþme saðlandý. Kaptopril öncesi ve sonrasý ekokardiyografik olarak, mitral kapak erken (E) ve geç akým (A) hýzlarý ölçüldü.

Hipertofi görülmeyen hipertansif grupta (n=16) E/A oraný kan basýncýnýn düþmesiyle 0.751±0.187’den 0.875±0.218’e çýkýyordu (p<0.001). Buna karþýlýk, hipertrofi görülen olgularýn (n=17) kaptopril öncesi ve sonrasý E/A oraný 0.839±0.202 ve 0.819±0.198 idi.(a.d).

Bu bulgular, hipertansiyonun sol ventriküler hipertrofi geliþmeden tedavi edilmesinin, sol ventrikül diyastolik fonksiyonlarýný kýsa sürede olumlu yönde etkileyeceðini göstermektedir.

In this study, it’s aimed to search the effect of sudden decrease in blood pressure on diastolic functions of the left ventricle.

It was studied on 33 cases. 12 of them were men, and 21 of them were women who had sistolic blood pressures of >180 mmHg and/or diastolic blood pressures of >110 mmHg with sinus rhythm not having abnormal Q waves. The cases were divided into two groups by the wall thickness of their left ventricles; as the hypertrophic ones (>12 mm) and the nonhypertrophic ones (<12 mm). A decrease in blood pressures of all the patients was achieved by applying sublingual captopril. Mitral valve early (E) and late (A) flow rates were measured echocardiographically before and after captopril.

E/A ratio rised from 0.751±0.187 to 0.875±0.218 (p<0.001) in hypertensive group which didn’ t have hypertrophy (n=16). However, the E/A ratio of hypertrophic cases before and after captopril are; 0.839±0.202 and 0.819±0.198 (n.s.).

These findings show that, treatment of hypertension before growing up a left ventricular hypertrophy, would improve diastolic functions of left ventricle.

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Vaginal kanama þikayeti ile gelen, kollumu kapalý 80 gebe çalýþma grubunu normal gebelik muayenesine gelen 30 gebe kontrol grubunu oluþturdu. Tüm hastalar 20. gebelik haftasý tamamlanana kadar izlendi. Çalýþma grubu, gebeliði abort ile sonuçlanan (n=30) ve gebeliði devam eden (n=50) olarak iki gruba ayrýldý. Tüm gruplar ve kontrol grubunda serum progesteron, β-HCG deðerlerinin tek baþlarýna veya birlikte kullanýldýklarý taný oranlarý yaklaþýk olarak ayný bulunmuþtur. Bu parametrelere Doppler direnç indeks deðerlerinin eklenmesi doðru taný oranýný artýrmamýþtýr.

In this study 80 pregnant women presenting with vaginal bleeding without dilation of the cervix and 30 pregnant women presenting for a routine examination were evaluated. All patients were studied until the end of the 20th week of pregnancy. The study group consisted of 30 pregnant women whose pregnancy ended with abortion and 50 pregnant women whose pregnancy continued. Serum progesterone and b-HCG values alone or combined were not significant in determining the outcome of the pregnancy. Addition of the doppler resistance index value did not increase the predictive value either.

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Bu çalýþmada, kliniðimizde Kinosian yöntemi ile dakriosistorinostomi ve bikanaliküler nasolakrimal silikon tüp uygulamasý yapýlmýþ hastalarýn uzun süreli takip sonuçlarýný belirlemek amaçlanmýþtýr.

SSK Göztepe Eðitim Hastanesi göz hastalýklarý kliniðinde Ocak 1996 ile Aralýk 1999 tarihleri arasýnda kronik dakriosistit tanýsý ile ameliyat edilen 118 hastanýn 140 gözü incelendi. Hastalara Kinosian yöntemi ile dakriosistorinostomi ve bika-naliküler nasolakrimal silikon tüp implantasyonu uygulandý.

Ýncelemeye alýnan hastalarýn 96’sý (% 81) kadýn, 22’si (% 19) erkek idi. Hastalarýn yaþ ortalamasý 39.9 yýl (9-68 yýl) idi. Postoperatif izlem süreleri benzer olup, ortalama 16.23 ay (4-46 ay) idi. Postoperatif uzun süreli izlem sonucunda 140 gözün 122 tanesinde (% 87.15) kanalýn açýk, 18 tanesinde (% 12.85) kanalýn kapalý olduðu, yapýlan lavaj sonucunda görülmüþtür.

Kronik dakriosistit nedeniyle kinosian tekniði kullanýlarak yapýlan dakriosistorinostomi ve bikanaliküler silikon tüp uyglamasýnýn % 87.15’lik baþarý oraný ile etkili bir cerrahi prosedür olduðu sonucuna varýlmýþtýr.

In this study, our purpose is to evaluate the results of followup time for the patients who were opareted by Kinosian technique and bicanallicular silicone tube intubation.
Between January 1996-December 1999 140 eyes of 118 patients who had been operated for chronic dacriosysthitis were evaluated. 96 (81 %) of 118 patients were female and 22 (19 %) were male. Mean age is 39.9 (9-68 year). Postoperative follow-up time was 16.23 months (4-46 months). Postoperatively 122 eyes were controled by canallicular irrigation and lacrimal drainage were found to be patent with a success rate of 87.15 %, in 18 eyes (12.85 %) lacrimal drainage were found to be occluded after saline irrigation.

According to these results; Kinosian technique which was supported by silicone tube intubation is a successful procedure.

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Bu çalýþmada sinus sigmoideus’un üç olasý emisser veninin delikleri ve v. emissaria occipitalis’in deliði üzerinde çalýþýldý. Deliklerin ölçümlerini ve lokalizasyonlarýný saptamak amacýyla 464 adet temporal kemik kullanýldý. Emisser venler ve bu venlere ait delikler, özellikle baþ-boyun cerrahisinde önem taþýmaktadýr. Diðer taraftan geniþlemiþ v.condylaris posterior’un da objektif tinnitus nedeni olabileceði bilinmektedir. Bu nedenlerle sonuçlar önceki çalýþmalarla karþýlaþtýrýlarak emisser venin klinik önemi gözden geçirildi.

Three possible emissary vein foraminas of the sigmoid sinus and the foramen of the occipital emissary vein were studied in this study. 464 temporal bones were used for measurements and the locations of the foraminas. The emissary veins and their foraminas are important especially in head and neck surgery. On the other hand, it is known that objective tinnitus can be related with enlarged posterior condylar emissary vein. Because of these reasons, the clinical importance of emisary vein anatomy is reviewed by comparing the results with the previous studies.

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Hormon replasman tedavisinin postmenopozal dönemde kardiyovasküler sisteme etkisini, internal karotis arter ve uterin arter pulsatilite indeksi üzerine etkisini araþtýrarak göstermeyi amaçladýk.

Menopozal þikayetler ile poliklini¤imize baþvuran 30 olgu çalýþmaya alýndý. Olgulara kesintisiz konjuge östrogen 0.625 mg/gün ve siklusun son 12 gününde 5 mg/gün medroksiprogesteron asetat tedavisi baþlandý. Ýnternal karotid ve uterin ar-ter pulsatilite indekslerinin bazal de¤erleri renkli doopler ul-trasonografi kullanýlarak ölçüldü. Tedavinin 3. ayýnda tekrar internal karotid ve uterin pulsatilite indeks deðerlendirmesi yapýldý.

Hormon replasman tedavisi sonrasý internal karotis ve uterin arter ortalama pulsatilite indeks oranlarýnda anlamlý düzeyde azalma izlendi. Bazal ortalama internal karotis arter pulsatilite indeksi 1.28±0.32 iken, tedavi sonrasý 1.07±0.25’e (p<0.001) ve bazal ortalama uterin arter pulsatilite indeksi 2.13±0.36 iken 1.89±0.35’e (p<0.05) düþtü.

Oral hormon replasman tedavisi, kullanýmýnýn üçüncü ayýnda internal karotis ve uterin arterde kan akýmýna olan direnci azaltýr. Vasküler sistem üzerindeki bu etki, kadýnlarda hormon replasman tedavisinin kardiovasküler sistem üzerindeki koruyucu etkisini açýklamaktadýr.

We aimed to demonstrate the effects of oral hormone replacement theraphy on cardiovascular system at postmenopausal period by researching its effect on internal carotid artery and uterin artery.

We worked on 30 patients who applied for our policlinic with menapousal complaints. 0.625 mg/day continue conjuge estrogen and last 12 days of the cyclus 5 mg/day medroxyprogesterone acetate treatment were given. Color doppler ultrasonography was used to measure the impedance to flow (pulsatility index) within the internal carotid and uterine artery at baselin. On the 3rd month of the treatment pulsatility index evaluation were repeated.

After hormone replacement therapy the reductions n mean pulsatility index values of the internal carotid artery and uteri-ne with respect to baseline were at a significant level. The mean internal carotid artery pulsatilitl index was decreased from 1.28±0.32to 1.07±0.25 (p<0.001) and the mean uterin artery pulsatility index also fell from 2.13±0.36
to 1.89±0.35 (p<0.05) after treatment.

Oral hormone replacement theraphy effective at 3 month in reducing impedance to flow in internal carotid artery and uterin artery. This vascular effect explain how hormone
replacement therapy protects women cardiovascular disease.

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Pediatrik yaþ grubunda üreteropelvik darlýkta dismembered piyeloplasti en çok tercih edilen cerrahi giriþimdir. Drenajýn nasýl yapýlacaðý konusunda halen pek çok tartýþma vardýr. Haziran 2001-Eylül 2002 tarihleri arasýnda stent konan ve konmayan piyeloplasti sonuçlarýmýzý karþýlaþtýrdýk. Üreteropelvik darlýk tanýsý konan 18 hastanýn 7’sine stentsiz, 11’ine double J stent (DJS) konarak piyeloplasti uygulandý. Hiçbir hastada diversiyon ile piyeloplasti uygulanmadý. Postoperatif erken dönem komplikasyon ve hastanede kalýþ süresi yönünden her iki grup karþýlaþtýrýldý. Stentsiz 7 hastanýn 2’sinde komplikasyon izlendi. Bir hastada uzamýþ drenaj ve 1 hastada geçici obstrüksiyon nedeniyle stent takýlmasý gerekti. Stent takýlan grupta hiç bir ürolojik komplikasyon izlenmedi. Median hospitalizasyon stent takýlan grupta ve stent takýlmayan grupta 6 gündü. Stent takýlan grupla stent takýlmayan grup arasýnda sonuçlar birbirine yakýn görülmektedir.

Dismembered pyeloplasty remains the principal surgical therapy for pediatric ureteropelvic junction obstruction. The method of postoperative drainage continues to be debated. We compared 7 stented versus 11 nonstented repairs. Diversion was not used for any patient. We evaluated 18 pediatric dismembered pyeloplasties performed for ureteropelvic junction obstruction from June 2001 to September 2002. Hospital stay and complication rates were reviewed. Of the 7 nonstented repairs urological complications developed in 2 including prolonged leakage and temporary obstruction that necessitated stenting. Non of the stented repairs devoloped urological complications. Median hospitalization was 6 days in each group. In children the outcome of stented pyeloplasty is similar to that of nonstented repair.

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Febril konvulsiyonlar, çocukluk çaðýndaki en sýk konvulsif olay olup,tüm çocuklarýn % 2.5’inde görülür. Çoðu çocukta prognoz oldukça iyidir. Basit febril konvulsiyonlarý takiben epilepsi geliþme riski, genel populasyondan hafifçe yüksek olup, basit febril konvulsiyonlardaki asýl problem üçte bir çocukta görülen nöbet rekürrensidir. Nöbetlerin erken yaþta baþlamasý, 1. derecedeki akrabalarda febril konvulsiyon hikayesi olmasý, rekürren febril konvulsiyonlarýn oluþumu ve nöbetlerin düþük dereceli ateþler ile meydana gelmesi çocuklardaki rekürrens riskini artýrýr. Ailede epilepsi hikayesi olmasý, geliþimsel nörolojik anormallik bulunmasý ve nöbetlerin düþük dereceli ateþler ile meydana gelmesi febril konvulsiyonlu çocuklarda epilepsi geliþimi için riskli faktörlerdir.

Febrile seizures are most common convulsive event in childhood, occuring in 2 % to 5 % of all children. The most important side of febrile convulsions for children is most children
have an excellent prognosis. During follow up evaluation, the risk of epilepsy after a simple febrile seizure was shown to be only slightly higher than that of the general population, where as the chief risk associated with simple febrile seizures was recurrence in one third of the children. Predictors in identifying children with very high risk of recurrence are young age at onset, family history of febrile seizures a first degree relative, a history of recurrent febrile sizures and a lower degree of fever at the onset of febrile conculsion are predictors for later epilepsy in children who have febril seizures.

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En az iki kez ve üzerinde tekrarlayan düþük ve ölüm doðum hikayesi olan, yaþlarý 19-49 arasýnda deðiþen, 45 kadýn hasta grubumuzu oluþturdu; en az bir kez canlý doðum yapmýþ olan 30 saðlýklý kadýn kontrol grubumuzu oluþturdu. Kardiyolipin, fosfotidil serin, fosfotidik aside karþý antifosfolipid antikorlarý (APA; Ig G, Ig M, Ig A tipi), ELÝSA (Stago) metoduyla araþtýrdýk. Hastalarda APA prevalansýný % 49 oranýnda pozitif olarak bulduk. Daha geniþ hasta gruplarýnda çalýþýlmasý önerilir. Standart APTT testi ile lupus antikoagülanýnýn araþtýrýlmasý güvenilir deðildir.

45 women, aged between 19-49, who had at least twice or more recurrent abortus and intrauterin death made up our patient group; 30 healthy women who had at least one living
birth made up our control group. We studied antiphospholipid antibodies (APA; Ig G, Ig M, Ig A types) mixed of antibodies against cardiolipin, phosphotydil serin, phosphotidic acid using ELISA (Stago) method. We found prevalance of APA positivity % 49 in patients. It is recommended to study in bigger, similar patient groups. Searching for lupus anticoagulants
by using standard APTT test is not so reliable.

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Bu çalýþmada allerjik konjonktivitli olgularda kullanýlan topikal % 0.1’lik olopatadin hidrokloridin oküler semptom ve bulgulara etkisini saptamak amaçlanmýþtýr.

Kliniðimizde Mart-Mayýs 2001 tarihleri arasýnda allerjik konjonktivit tanýsý konulan 60 hastanýn 120 gözü çalýþma kapsamýna alýndý. Olgular, tedavi öncesi oftalmolojik muayene ile deðerlendirildi. Olgularýn her iki gözüne günde iki kez 12 saat ara ile birer damla % 0.1’lik olopatadin hidroklorid damlatýldý ve oküler semptom ve bulgularý tedaviye baþladýktan sonra 30. dakika, 48. saat ve 7. günde tekrar deðerlendirildi.

Hastalarýn 39’u bayan, 21’i erkek ve yaþlarý 16 ile 64 yýl arasýnda ortalama 39.2 yýl idi. Tedaviye baþladýktan sonraki 30.dakikada oküler semptomlarýn % 47’si, bulgularýn ise % 29’u düzeldi. 48. saatte semptomlarýn % 68’i, bulgularýn % 53’ü düzeldi. 7. günde semptomlarýn % 83’ü, bulgularýn % 76’sý düzeldi. Hastalarýn tamamý tedavilerini düzenli olarak uyguladý ve hiç bir hastada ilaç intoleransý geliþmedi.

Sonuç olarak, allerjik konjonktivit tedavisinde topikal olarak günde iki kez kullanýlan % 0.1’lik olopatadin hidrokloridin oküler semptom ve bulgular üzerinde hýzlý ve etkin tedavi saðladýðý saptanmýþtýr.

In this study we were evaluated effect of 0.1 % Olopatadine hidrochloride for the ocular symptoms and signs of allergic conjunktivitis.

Between March-May 2001, 120 eyes of 60 patients were included in the study. Preoperatif routine controls of patients were done. 1 drop of 0.1 % Olopatadine hidrochloride were applied to the eyes of patients twice a day. After the begining of therapy, cases were controlled on 30th minute, 48th hour and an the 7th day.

39 patients were female, 21 were male. Mean age was 39.2 (16-64 years). On 30th minute 47 % of ocular symptoms, 29 % of ocular sign were relieved. On 48th hour 68 % of symptoms, 53 % of signs were relieved. On 7th day 83 % of symptoms, 76 % of signs were relieved. All of the patients had medical therapy regularly and drug intolerance were not noticed.
0.1 % olopatadine hidrochloride is an effective alternative medical agent for allergic conjunctivitis, because of its rapid effect on symptoms and signs of patients and easy way of application.

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GÝRÝÞ ve AMAÇ: Yaþamlarýnýn ilk yýlý içinde üç farklý þekilde beslenen fenilketonürili bebeklerin diyetlerinde aldýklarý enerji, protein ve fenilalanin miktarlarýna göre büyümelerinin farklý olup olmadýðýný araþtýrmaktýr.
YÖNTEM ve GEREÇLER: Çalýþmaya 1994-97 yýllarý arasýnda Ýstanbul Üniversitesi Týp Fakültesi Çocuk Kliniði’nde yenidoðan tarama testi ile tanýsý konmuþ ve diyet tedavisine baþlanmýþ 24 kýz, 33 erkek, toplam 57 fenilketonürili hasta alýndý Hastalar 0-1 yaþtaki beslenme þekillerine göre anne sütü, formül süt ve karýþýk beslenenler olmak üzere üç grupta incelendi. Diyetlerinde alacaklarý anne sütü ve/veya formül süt miktarý kan fenilalanin düzeylerine göre belirlendi. Anne sütü ve formül süt dýþýnda, diyete fenilalanin içermeyen bir amino asit karýþýmý ile niþasta, maltodekstrin ve sývý yað ile hazýrlanan, protein içermeyen bir enerji kaynaðý ilave edildi. Kan fenilalanin düzeyi yükseldiðinde kullanýlan sütün miktarý azaltýlýrken, enerji ve protein kaynaklarýnýn miktarý gereksinmeyi karþýlayacak þekilde artýrýldý. Düþtüðünde ise bunun tersi uygulandý. Hastalarýn büyüme durumlarý ayda bir kez poliklinik kontrollerinde
yapýlan aðýrlýk, boy ve baþ çevresi ölçümleri ile deðerlendirildi. Kan fenilalanin düzeyleri ve büyüme durumlarýna göre diyetlerinde alacaklarý enerji, protein ve fenilalanin miktarlarý hesaplanarak kaydedildi. Kan fenilalanin düzeyini istenilen sýnýrlar içinde tutmak ve bebeðin ayýna uygun ek besinleri zamanýnda baþlayabilmek için hastalara ait kayýtlar üçer aylýk dönemlere ayrýlarak incelenmiþtir. Verilerin istatistiksel analizi SPSS 5.0 for windows programýnda varyans analizi, t testi ve kruskal walls testleriyle yapýlmýþtýr.

BULGULAR: Her üç beslenme grubunda yer alan hastalarýn yaþamlarýnýn ilk bir yýlý içindeki besin tüketimleri protein için 2.0-2.5 g/kg/gün, enerji için 100-110 kcal/kg/gün, fenilalanin için 30-35 mg/kg/gün arasýndadýr. Beslenme þekillerine göre aðýrlýk ve boy ortalama deðerleri yönünden gruplar arasýnda anlamlý farklýlýk bulunamadý (p>0.05), fakat protein ve enerji tüketimlerinin ilk yaþta tüketilmesi önerilen referans deðerlerin altýnda olduðu bulundu.
TARTIÞMA ve SONUÇ: Taný konmadan önce uygulanmakta olan ve tanýdan sonra da ayný sütün kullanýlmasýyla sürdürülen üç farklý beslenme þeklindeki fenilketonürili bebeklerin 0-1 yaþtaki büyümeleri birbirinden farklý deðildir. Bu deðerler Türk çocuklarý için geliþtirilen büyüme standartlarý ile de uygunluk göstermektedir.

INTRODUCTION: To investigate if there is any differences between growth and nutrition of patients having phenylketonuria (PKU) in their first year of life.
METHODS: Fifty seven infants (24 females and 33 males) with PKU who were diagnosed at the University of Istanbul, Children’s Hospital between 1994-97 years were taken into the study. Patients were grouped into three according to their nutrition as breast feed (BF), formula feed (FF) or both in relation to their primary source of phenylalanine (phe). Volume of breast and/or formula milk ingested by infants was adjusted according to blood phe concentrations. A phenylalanine-free amino acid mixture and a protein-free energy mixture were added to diet. Growth of the patients were controlled once a month as body weight, height and head circumference. Energy, protein and phe contents of the diet were calculated according to patient’s growth and blood phe levels. Because we aimed to maintain blood phe between desired levels, and to add new foods to babies diet at the right month, it became necessary to make dietary changes throughout the therapy while babies
were growing. Therefore, the records of patients on their nutrition and growth were evaluated as 3 month’s periods. All results were statistically analysed by varians analyses, student t and kruskal walls tests on SPSS 5.0 for windows program.
RESULTS: Protein, energy and phe intakes of infants were between 2.0-2.5 g/kg/day, 100-110 kcal/kg/day and 30-35 mg/kg/day respectively. There was no significant differences in growth between groups with respect to their nutrition, but protein and energy intakes were below the standards recommended for phenylketonuric infants.
DISCUSSION AND CONCLUSION: Growth of infants with PKU having 3 different types of nutrition were not different. Findings on growth of
PKU patients were similar with the standards of growth developed for Turkish children. It was concluded that following up the patients regularly and parent education programmes played important roles for this results.

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Adet öncesi sýkýntýlar, özellikle yetiþkin üreme çaðýndaki kadýnlarýn karþýlaþtýklarý bir sorundur ve bazen normal hayatýný engelleyebilen bir durumdur. Hemen hemen her kadýn adet öncesi bir gerginlik ve huzursuzluk yaþar, ancak bu durum onun günlük hayatýný çok etkilemez. Bu belirtiler kadýnlarýn yarýsýndan daha azýnda rahatsýz edici, ancak
dayanabilecek þiddette olurken, % 5 kadýn oldukça þiddetli belirtiler hisseder.

Bu araþtýrma, en sýk görülen premenstruel semptomlarý belirlemek, premenstruel sendrom görülme sýklýðýný ve ilgili deðiþkenleri saptamak amacýyla Ýstanbul Anadolu Yakasý’ndaki 15-45 yaþ arasý 257 bayan örneklem alýnarak yapýldý. Araþtýrmada anket yöntemi kullanýldý. Araþtýrma ile bayanlarýn yaþlarý, medeni halleri, öðrenim durumlarý, meslekleri, aile öyküleri ve saðlýk kurumuna yaptýklarý baþvurular incelendi.

Araþtýrma grubundaki bayanlarda premenstruel sendrom görülme sýklýðý % 32.7 olarak saptanmýþtýr. Premenstruel sendrom ile araþtýrýlan baðýmsýz deðiþkenlerden aile öyküsü ve bu dönemdeki þikayetler nedeniyle saðlýk kurumuna baþvuranlar arasýnda istatistiksel olarak anlamlý bir fark bulunmuþtur.

Premenstrual syndromes which sometimes obstacle daily lives, are mostly observed especially in women at age of reproduction. Almost every woman experiences premenstrual stress and discomfort. These kinds of syndromes are felt disturbing by less than 50 % of women, 5 % of them quite feel terrible.

This research has been done in Anatolian side of Istanbul in 257 women in an age range of 15 to 45 in order to determine the most frequent premenstrual symptoms and to determine the frequency and the related variables of premenstrual syndromes as well. A survey system has been used in this research. By this research the women’s ages, marital statuse, levels of education, professions, family stories, and their application to health authorities have been searched.

In this research premenstrual syndrome has been found in 32.7 % of women. A statistically significant difference has been found between family stories which is on independent variables premenstrual syndrome and the women who applied to health authorities due to increased complaints in this period.

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Scleroderma is an autoimmune disease that is characterized by excessive collagen collection in various internal organs like skin, lung, heart and gastrointestinal system. There are two forms of the disease, that’s; the systemic one manifested by multiorgan failure due to fibrosis of the internal body systems, and the localized one presented with only the cutaneous, subcutaneous and muscular tissue involment. In this report, three children presenting with various clinical manifestations involving both forms of the disease are evaluated according to their laboratuary parameters and prognostic factors through last seven years. Cases are reported as this disease is diagnosed very rarely in the childhood period. The first case is an example of localized scleroderma, the second and third ones
are examples of systemic sclerosis.

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Multiple sclerosis (MS) is a chronic disease that begins most commonly in young adults and is characterized clinically by relapses, remissions and in few cases rapid progressive course and shows a variety of neurological deficits. Cesarean section under general anesthesia was planned for 40th week pregnant patient aged 30. In patients with MS, factors such as, hyperthermia, postpartum period, trauma, surgery and anesthesia are known to relapse the symptoms of the disease. In this case report, a MS case with application of cesarean section operation under general anesthesia is presented.

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Leptospirosis is a zoonotic disease of worldwide distribution caused by spirochetes of the genus leptospira. Leptospires infect a variety of wild and domestc animals that excrete the
organism in their urine. Humans, infected through direct contact with infected animals or through exposure to fresh water or soil contaminated by infected animal urine, develop an
acute febrile illness that can be followed by a more severe, sometimes fatal illness that may include jaundice and renal failure (Weil’s disease), menengitis, miyocarditis, hemodynamic
collapse.

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A case of SWS is presented here in a five-year-old female with, facial hemangiomata, glaucome, seizures, cerebral calcification, cerebral cortical atrophy.

The aim of this presentation is to emphasize the need of being kept in mind SWS which is rarely seen in patients with facial hemangiomata, glaucome, seizures, cerebral calcification,
cerebral cortical atrophy.

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Inflammatory pseudotumor is rare pulmonary tumor of unknown etiology. It is often asymptomatic and shows radiographic findings of lung tumor. Based on clinical features
and biologic behavior, it has been generally regarded as a benign disease. The diagnosis of inflammatory pseudotumor can not be usually obtained by preoperative diagnostic methods.
Two cases of pulmonary inflammatory pseudotumor were presented in this paper.

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Myasthenia gravis is an autoimmune disease, resulting from the production of antibodies against the acetylcholine receptors of the neuromuscular endplate. These antibodies reduce the active receptors, brought about either by functional block of the receptors, by increased rate of receptor degration, or by complement-mediated lysis. Myasthenic patients are special for anaesthesia. The myasthenic patient may demonstrate an abnormal neuromuscular response to both depolarizing and nondepolarizing muscle relaxans. Volatil induction and maintenance was successfully employed for patients without myo-pathy. Inhalational agents can be used for anaesthetic induction of myasthenics with the advantage of avoiding the use of neuromuscular blocking agents.

In this study, we present a myasthenic case, for her umblical hernia operation in which sevoflurane was used in induction and in maintainence of anaesthesia as a single agent.

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Mixt connective tissue disease is a distinct rheumatic disease. Mixt connective tissue disease should be considered as an important syndrome in any patient who presents with heterogeneous clinical presentation and who do not fit into any definite criteria of systemic connective tissue disorders. The characteristic features of mixt connective tissue disease are: 1) the presence of anti-U1 snRNP (small nuclear ribonucleoprotein)
antibody with high titers in sera, 2) an increased frequency of HLA-DR 4 in the leukocytes, and 3) death due to pulmonary hypertension (1). The disease is primarly localized on joints, muscles and skin; however there are not widely used diagnostic criteria. We wish to point out the importance of immunologic approach to the aetiology of lymphadenitis in a middle-aged patient, as well as the therapeutical dilemmas in the treatment of the disease.

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Ectodermal dysplasia (ED) is a rare, hereditary, congenital disease that affects teeth, skin, hair, nails, eccrine and sebaceous glands. Symptoms of ED are hypohidrosis or anhidrosis, heat intolerance, dental and nail abnormalities, hypotrichosis, inability of lacrimation, thinning and hypopigmentation of skin. ED has got three types: Hypohidrotic (unhydrotic), hidrotic (clouston type) and EEC syndrome (ectrodactyly, ED, cleft lip and palate). In hypohidrotic type there are decreased or absent sweating glands, abnormal dental structure and hypotrichosis.

Our patient is 7.5 years old, male. When he come to our clinic, he has got afebrile seizure, inabilitiy to sweat, decreased tears and abnormal skin presentation. When we focus on history of patient’s symptoms, we decided that this signs and symptoms are related to hypohidrotic ED and perinatal asphyxia sequel.

In this case, we diagnosed ED with history and typical signs of patient. We present this case because of its rare and congenital disease.

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