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ÖZETAmaç: Bakteriyel menenjit tıbbi acil bir durumdur. Tedavi edilmemiş bakteriyel menenjitlerde mortalite oranı %100'dür. Çalışmada, Türkiye'de Haemophilus influenzae tip b ve Streptococcus pneumoniae'ya karşı geliştirilmiş konjuge aşıların Ulusal Bağışıklama Programı'na girmesinden önce ve sonra, 2 farklı dönemde, 2 farklı merkezde izlenen akut bakteriyel menenjitler değerlendirilerek, Türkiye'de yıllar içinde akut bakteriyel menenjit epidemiyolojisi, tedavi seçenekleri, komplikasyon oranlarındaki değişikliklerin ortaya konulması amaçlanmıştır. ABSTRAcTObjective: Bacterial meningitis is a medical emergency. The mortality rate in untreated bacterial meningitis is 100%. The aim of the present study was to determine the changes in acute bacterial meningitis epidemiology, treatment options and complication rates in Turkey over the years by evaluating acute bacterial meningitis treated in two different centers before and after the introduction of the conjugate vaccines against Haemophilus influenzae type b and Streptococcus pneumoniae into the National Immunization Program in Turkey. Material and Methods:The study is a retrospective study. The study included 54 patients who were followed up for the diagnosis of acute bacterial meningitis at the Infectious Diseases

We report on two siblings with tyrosinaemia type 2, a 6-year-old girl and her 3.5-year-old brother with a main complaint of painful palmo-plantar hyperkeratosis. The boy presented also conjunctival injection, photophobia, lacrimation and conjunctivitis. Blood tyrosine levels were increased in both patients, 150.6 and 202.3 lmol/dl, respectively (reference value: 5.8±2.2 lmol/dl). After 1 month of dietary treatment with low protein, low phenylalanine and tyrosine, tyrosine levels decreased to 37.7 and 65.6 lmol/dl, respectively; the cutaneous lesions improved in both of them, and conjunctivitis disappeared. Conclusion: the association of bilateral ulcero-conjunctivitis with photophobia and palmo-plantar hyperkeratosis since early infancy is the clue to the diagnosis of tyrosinaemia type 2.

A nine-year old girl with T cell acute lymphoblastic leukemia (ALL) had acute severe neurologic complications at the end of the remission-induction chemotherapy course. Thirty-six hours following triple intrathecal (IT) therapy and intravenous (IV) administration of L-asparaginase (L-asp), tetraplegia developed and she became unconscious. She had bouts of hypertension and persistent tachycardia unresponsive to digitalis therapy. Magnetic resonance imaging (MRI) showed multiple brain white matter hyperintensities and filling defects in the saggital sinus, suggesting thrombosis. Over the 40 days, in addition to her neurologic compromise she also had transient diabetes mellitus, severe hyperlipidemia, hypoproteinemia and edema, liver and heart failure and staphylococcus aureus sepsis with prolonged bone marrow depression. Despite, coexistence of all these chemotherapy related complications, her neurologic functions and multiple organ failure improved gradually. After a 70 days' period of interruption, chemotherapy was resumed and continued without any further complications. Although, the etiology of her extensive sensitivity to some drugs remains unclear, we believe that it is important to document these unusual events in this child.

Konjenital klor diyaresi (KKD) kronik ishal ve dışkıda yüksek klor konsantrasyonu ile karakterize nadir görülen bir hastalıktır. Otozomal ressesif geçişli olan bu hastalıkta SLC26A3 genindeki mutasyonlar sonucu distal ileum ve kolondaki HCO 3 /Cldeğişimi bozulur ve sonuçta sodyum-klor emiliminin bozulmasıyla sulu, klordan zengin diyare, hipokloremi, hipokalemi, metabolik alkaloz gelişir (1,2). Tanı için dışkı klorunun yüksek olması (90mmol/L) spesifiktir. Çocuklarda NaCl ve KCl replasman tedavileriyle yüz güldürücü sonuçlar alınmaktadır (1-3). Burada yüksek dışkı klor konsantrasyonu ile KKD tanısı alan 12 aylık erkek hasta sunulmuştur.3 değişim mekanizmasındadır. Sulu, kronik ishal ve yüksek dışkı klor içeriği, hiponatremi ve hipokloremik metabolik alkaloz ile karakterizedir. Yüksek dışkı klor konsantrasyonu ile konjenital klor diyaresi tanısı alan 12 aylık erkek hasta sunulmuştur.

ÖZET Semptomları yaşamın ilk yılında başlayan ve be-

A 6-week-old girl, the first child of nonconsanguineous parents, was admitted to the hospital for evaluation of vomiting. She was small for gestational age (1500 g). On admission, she weighed 1830 g, and appeared dehydrated. The blood glucose was 880 mg/dL. Insulin and C-peptide levels were <1 μIU/ml and 0.1 pmol/L, respectively. Antibodies of diabetes were negative. The serum triglyceride level was markedly elevated (5322 mg/dL). After a few days of insulin therapy, the triglyceride levels dramatically decreased, but cholestasis persisted. A liver biopsy revealed diffuse iron deposition and the diagnosis of neonatal hemochromatosis was established. In neonatal hemochromatosis, diabetes may occur as a result of iron deposition in the pancreas. The coexistence of neonatal diabetes secondary to neonatal hemochromatosis with a fatal course during the infancy period has not been previously reported. In this report, an infant with neonatal diabetes secondary to neonatal hemochromatosis is presented as the first case in the literature involving the coexistence of these two conditions. KEY WORDSneonatal hemochromatosis, neonatal diabetes mellitus, hyperglycaemia, hyperlipidemia.