Pamir Gülez

Efficacy of continuous midazolam infusion and mortality in childhood refractory generalized convulsive status epilepticus

PURPOSE Continuous midazolam infusion is commonly used for the management of status epilepticus (SE). The purpose of this study was to assess the efficacy of midazolam and mortality in childhood refractory generalized convulsive SE. METHODS We included 27 children with refractory generalized convulsive SE. Midazolam was given 0.2 mg/kg as bolus, followed by 1-5 microg/kg/min as continuous infusion. Clinical data and response to treatment were recorded for each patient. RESULTS Acute symptomatic SE accounted for 52%, and central nervous system (CNS) infections were the most frequently associated etiologic condition (44%). Complete control of seizures was achieved with midazolam infusion in the 26 (96%) children within 65 min; at a mean midazolam infusion rate of 3.1 microg/kg/min. Adverse effects such as hypotension, bradycardia or respiratory depression did not occur during midazolam infusion. In one (4%) patient with acute meningoencephalitis, SE could not be controlled. Five (19%) patients died; four had acute symptomatic aetiology and one had progressive encephalopathy. CONCLUSION Midazolam is effective and safe in the control of refractory generalized convulsive SE. The response to treatment and mortality were related to the underlying aetiology.

A case of hypomagnesemia with secondary hypocalcemia caused by Trpm6 gene mutation

An offspring of marriage between two first cousins presented with atonic seizures developed on the 20th day of life. The physical examination of the case was normal. In laboratory results, Ca+2 level was 5,7 mg/dl, Mg+2: 0,4 mg/dl (1,3–2,1), PTH: 28,4 pg/ml (12–92), and P-: 4,5 mg/dl. The case was diagnosed as hypomagnesemia with secondary hypocalcemia (HSH) and TRPM6 gene mutation analysis revealed a homozygote mutation of E157X.

Kasabach-Merritt syndrome with İnfantile hepatic hemangioendotheliomas: a case report.

İnfantil hepatik hemanjio-endotelioma, bebeklik çağının en sık görülen benign hepatik vasküler tümörüdür. Kızlarda erkeklere oranla üç kat daha fazla görülür. Olguların %75’i ilk üç ayda tanı alır. Bütün dev hemajiomlar gibi Kasabach-Merritt sendromuna neden olabilen bu hastalık, klinik olarak hepatomegali, yüksek debili kalp yetmezliği ve dev hemanjiomlar ile karakterizedir. Özellikle yenidoğan ve erken sütçocukluğu döneminde şant hiperbilirubinemisi sonucu uzamış sarılığa yol açabilir. Kasabach-Merritt sendromunda dev hemanjiomlar hızlı büyüyerek yaşamı tehdit eden trombositopeni, mikroanjiopatik hemolitik anemi ve tüketim koagülopatisine yol açabilir. Bu sendromun tedavisi trombositopeni, anemi, tüketim koagülopatisinin yönetimini kapsar. Küçük lezyonları için cerrahi eksizyon uygulanabilir. Mortalite oranı yüksektir. Bu makalede uzamış sarılık nedenleri araştırılırken, Kasabach-Merritt sendromu bulguları ile karşılaşılan infantil hepatik hemanjioendoteliomalı bir olgu sunulmuştur.

Evaluation of drug poisoning in children.

Objective: The aim of this prospective study was to evaluate the pattern of acute poisoning related to drugs in children with relation to different age groups. Methods: The age, gender, active pharmaceutical ingredients, mode of drug provision, time elapsed from drug intake to the hospital entry, duration of hospitalization and the outcome of all drug-related cases of poisoning that were admitted to our hospital during 2006 were recorded. Results: A total of 257 cases, aged between 1 month and 18 years with drug-related poisoning were included in the study which comprised 2.2‰ of all emergency admissions. Among all, 80.9% of the cases were between 0-6 years of age. In 85.2% of the cases, the drugs were ingested accidentally and in 14.8% for suicidal purposes. Accidental drug ingestion was more frequent in boys and in children under the age of 6, while ingestion for suicidal purposes was more common in girls and in children over 7 years of age. The most common drug poisonings were related to drugs affecting the central nervous system (36.9%). The 5.4% of the cases were admitted to the critical care unit and 0.8% of them died. Conclusion: The study revealed that children of all ages could easily have access to drugs. Amitryptilin, an easy-to buy and low-cost drug was determined as the most common cause of drug-related poisonings.