23 research outputs found

    Newborn treated with continuous renal replacement therapy for citrulinemia-type 1

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    Introduction: Hyperammonemia occurs as a result of the inability to convert ammonia, a metabolic toxin, into urea due to a block in the urea cycle, and there resulting neurotoxicity is responsible for the pathogenesis. Case Presentation: Our patient was 7 days old when followed up in an external center for 3 days with a preliminary diagnosis of neonatal sepsis. Lethargy, vomiting, tachypnea, and convulsions, which are frequently seen in the first neonatal forms of urea cycle disorders, were also present in our patient. He was referred to us as a result of high ammonia levels when he was examined in terms of congenital metabolic diseases. He was intubated due to the rapid development of respiratory failure. When he was admitted to our intensive care unit with hyperammonemia, light reflex could not be obtained, and widespread cutis marmaratus was developed. Continuous renal replacement therapy was started in our patient and administered intermittently for 120 hours. The glucose infusion rate was followed by high fluid. When it orally tolerated, it is supported with sodium benzoate and sodium stearyl fumarate to reduce ammonia. Nutrition was limited to protein with Basic P. Conclusion: After staying in the intensive care unit for 30 days, our patient was discharged with the recommendation of outpatient follow-up by the pediatric metabolism physician. When our patient came for his check up after two months,there was no nystagmus and no seizures

    Pediatric Tracheotomy: A Relatively Rare Indication Limited to Pediatric Intensive Care Subjects?

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    Objective:To evaluate indications, underlying conditions and outcome in pediatric tracheotomy subjects.Methods:Between January 2004 and September 2013, pediatric subjects who underwent a tracheotomy operation were included for study. All subjects were under the age of 14. Subjects’ primary diseases, indication of tracheotomy, age, gender and type of complications were recorded.Results:Forty five subjects were identified. The mean age of subjects was 26.3 months (range between 2 to 140 months). Twenty one (46.6%) subjects were under the age of 1 year. In 34 (75.5%) out of 45 subjects, tracheotomy indication was prolonged intubation. In the remaining 11 (24.4%) subjects, tracheotomy indication was upper respiratory tract obstruction. Thirteen (n=13- 28.8%) out of 45 experienced early complications and 2 (n=2-4.5%) out of 45 subjects experienced late complications. One tracheotomy related death occurred in the immediate period. Eight (18.1%) of subjects died during the study period from underlying conditions. Decannulation was successfully performed in 13 (36.1%) subjects. In the remaining 23 (63.8%) subjects, decannulation could not be done.Conclusion:All tracheotomies in this report were performed on pediatric intensive care subjects on an elective basis. No tracheotomy was performed for acute upper airway obstruction. Even when planned and elective tracheotomy is performed it still has significant mortality and morbidity

    Pediatric Fulminant Leptospirosis Complicated by Pericardial Tamponade, Macrophage Activation Syndrome and Sclerosing Cholangitis

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    Background: Leptospirosis is a zoonotic infectious disease caused by pathogenic spirochetes of the genus Leptospira. Although it is usually asymptomatic and self-limited, severe potentially fatal illness accompanied by multi-organ failure may occur. Case Report: Here we report an unusual case of severe leptospirosis successfully treated with continuous venovenous hemofiltration (CVVHF) and therapeutic plasma exchange (TPE). The patient presented with pericardial tamponade, renal failure and macrophage activation syndrome, and later suffered prolonged jaundice and sclerosing cholangitis during hospitalization in the pediatric intensive care unit (PICU). To the best of our knowledge, sclerosing cholangitis due to leptospirosis has not been reported in the literature. Conclusion: Leptospirosis should be kept in mind in the differential diagnosis of sepsis and septic shock with fever, thrombocytopenia, jaundice and renal failure. TPE and CVVHF should start early after the diagnosis of leptospirosis with multiorgan failure
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