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Ailesel Akdeniz Ateşi sistemik otoinflamatuar bir hastalıktır. Hepsidin ise demir metabolizamasında rol oynayan inflamasyon ve enfeksiyonda yükselen bir peptiddir. Biz çalışmamızda Ailesel Akdeniz Ateşi ataklarında hepsidin düzeylerini ve hastalık patogenezinde hepsidinin olası rolünü araştırmayı hedefledik. YÖNTEM ve GEREÇLER: Çalışmamıza 42 erkek hasta ve 28 erkek kontrol dahil edildi. Hastaların ataklı ve ataksız dönemlerinden ve kontrol hastalarından prohepsidin, interlökin-6, serum demiri, serum demir bağlama kapasitesi, ferritin, fibrinojen ve eritrosit sedimantasyon oranı değerleri analiz edildi. BULGULAR: Hasta grubunun serum prohepsidin seviyeleri kontrol grubuna göre düşük iken, IL-6 seviyeleri hasta grubunda anlamlı yüksek saptandı (p<0.05). Serum prohepsidin ve IL-6 seviyeleri atak periyodunda anlamlı olarak yüksekti (p<0.01); atak sonrası dönemde IL-6 anlamlı olarak azalırken prohepsidinde anlamlı azalma gözlenmedi. Prohepsidin ve IL-6 seviyeleri arasında korelasyon saptanmadı. TARTIŞMA ve SONUÇ: Demir metabolizmasının Ailesel Akdeniz Ateşi patogenezinde inflamatuar mekanizmalara katkısal rolü olabilir.

s i t y of Minnesota, Minneapolis. (spon. by Robert A. Ulstrom.) We have observed t h e a s s o c i a t i o n of profound hypoglycemia and a n t e r i o r h y p o p i t u i t a r i s m i n f o u r newborns. A l l p r e s e n t e d w i t h hypoglycemia during t h e f i r s t 30 hours of l i f e , which reached l e v e l s l e s s than 10 mg.%. Rates of 20-30 mg./kg./min. of 20% d e x t r o s e were required t o m a i n t a i n g l u c o s e l e v e l s above 30 mg.%. A l l i n f a n t s were products of complicated pregnancies and d e l i v e r i e s and had APGAR s c o r e s of f i v e o r l e s s a t one and f i v e minutes Measurements of a n t e r i o r p i t u i t a r y f u n c t i o n i n c l u d i n g serum TSH, T4, ACTH, c o r t i s o l and growth hormone, i n a d d i t i o n t o metapyrone and cosyntropin s t i m u l a t i o n , revealed a n t e r i o r panhypop i t u i t a r i s m i n t h r e e i n f a n t s and i s o l a t e d ACTH d e f i c i e n c y i n t h e o t h e r . P o s t e r i o r p i t u i t a r y f u n c t i o n was normal i n a l l cases. Unresponsiveness of TSH t o i n t r a v e n o u s t h y r o t r o p i n r e l e a s i n g hormone (TRH) and normal serum p r o l a c t i n l e v e l s f u r t h e r support a n t e r i o r p i t u i t a r y d y s f u n c t i o n i n c o n t r a s t t o a hypothalamic e t i o l o g y . Autopsy on one i n f a n t revealed no a n t e r i o r p i t u i t a r y t i s s u e . The t h r e e s u r v i v i n g p a t i e n t s a r e a l l doing w e l l on a p p r o p r i a t e hormone replacement. Micropenis was p r e s e n t i n t h e one male and a l l i n f a n t s showed a widening of t h e midfacies. T h i s unappreciated cause of e a r l y n e o n a t a l hypoglycemia may b e r e l a t e d t o b i r t h hypoxia o r anatomic malformation of t h e ante r i o r p i t u i t a r y and i t s rudiments.These may b e more s u s c e p t i b l t o e v e n t s a t b i r t h than p r e v i o u s l y a p p r e c i a t e d and consequently I t h i s system should b e more a g r e s s i v e l y evaluated i n newborns.ANTIBODIES TO LUTEINIZING HORMONE I N A PATIENT TREAT-ED WITH GROWTH HORMONE. S. B u r s t e i n , F.A. Conte. S .L. Kaplan, and M.M. Grumbach. Dept. P e d i a t r i c s , Univ. of an Francisco, San Francisco, Ca. Many c l i n i c a l grade p r e p a r a t i o n s of hGH (human growth hormone) a r e contaminated w i t h hLH and hFSH. However, no evidence of a c l i n i c a l e f f e c t of such contamination has been d e s c r i b e d . We rep o r t t h e development of a n t i b o d i e s t o hLH i n a p a t i e n t t r e a t e d with hGH f o r i s o l a t e d G H d e f i c i e n c y .J.C. was f i r s t i n v e s t i g a t e d f o r s h o r t s t a t u r e a t 7-11/12 y e a r s and G H therapy was begun a t 8-9/12 y e a r s f o r a d i a g n o s i s of i s ol a t e d GH d e f i c i e n c y . On LRF ( l u t e i n i z i n g hormone r e l e a s i n g f a c t o r t e s t i n g , t h e LH r o s e from 0 . 8 t o 1 . 2 ng/ml (LER 960); FSH respons s l s o was normal....

entral diabetes insipidus (CDI) is a clinic condition characterized by polyuria and antidiuretic hormone (ADH) deficiency. Autoimmunity is mostly responsible for the etiology but rarely travma, encephalopathy, surgery and genetic disorders can play role in the etiology. 1Paracetamol intoxication is the most common cause of acute liver failure and the disease associated with encephalopathy. 2 We present a case of neurogenic diabetes insipidus that developed after paracetamol induced subacute fulminant liver failure. CASE REPORTA 20-years-old male patient admitted to emergency room with nausea and vomitting. In history 20 grams of paracetamol intake due to severe headache associated with migraine was present. In laboratory analysis international normalized ratio (INR): 2.65, alanine transferase (ALT):8400 U/L, aspartate transaminase (AST): 4900 U/L, total biluribin: 3 mg/dl, direct biluribin: 1.5 mg/dl, albumine: 3.4 g/dL, sodium: 141 mmol/L, creatinin: 0.8 mg/dl, pH: 7.4, HCO 3 :24 mmol/L were detected. Serum lactate levels were normal. Autoimmune and viral screen results were negative. Physical examination re-vealed grade 2 encephalopathy. An ultrasound scan showed a normal liver. TreatWorld Clin J Med Sci 2017;1(1) 55Central Diabetes Insipidus Due to Parasetamol Induced Subacute Fulminant Liver Failure: Case Report A AB BS ST TR RA AC CT T A 20-years-old male patient admitted to our hospital with nausea and vomitting after high dose paracetamol intake. With laboratory analysis and physical examination findings patient was diagnosed subacute fulminant liver failure. Urgent liver transplant was not considered according to King's College criteria and medical treatment was started. At follow up the patient's laboratory values and encephalopathy improved progressively but polyuria developed by day 7 of treatment (11 L/day). His serum sodium level increased to 152 mmol/L. His measured plasma osmolalite was 352 mOsmol/L, urine osmolalities were 171 mOsmol/L, the urinary specific gravity was 1003. The patient was diagnosed as CDI. After 10 mcg desmopressin therapy, urinary output fell under 3L/ day and patient discharged. As in our case, in liver failure patients CDI must be considered in cases that show polyuria.

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