New variation of scimitar syndrome with tracheal bronchus, upper lobe agenesis, and ventricular septal defect in a child

Scimitar syndrome (SS) is a rare and complex disease charac-terized by cardiovascular and bronchopulmonary malforma-tions. In typical SS, anomalous pulmonary drainage of part orall of the right lung into the inferior vena cava, anomaloussystemic arterial supply of the right lung from either the tho-racic or abdominal aorta, and hypoplasia of the right lungoccur.1Abnormal bronchial anatomy, abnormal diaphragm,hemivertebrae, and genitourinary tract anomalies can alsomanifest in SS.2,3Generally, the symptoms of this syndromeappear 7 months after birth.4According to the literature, therehave been numerous cases where additional features of thetypical syndrome have been reported with Scimitar variants

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

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

Retrospective Analysis of Cases with Guillain-Barré Syndrome in Pediatric Intensive Care Unit

Introduction: The aim of this study was to evaluate treatment approaches towards Guillain-Barré syndrome and treatment response in patients with Guillain-Barré syndrome admitted to the pediatric intensive care unit. Methods: We retrospectively evaluated patients aged between 1 month and 18 years who were admitted to our pediatric intensive care unit between January 2006 and January 2016 with the diagnosis of Guillain-Barré syndrome. Results: A total of 27 patients (10 girls; 37%) were included in this study. The mean age of the patients was 6.24 years (IQR: 4.07-10.03). Nine patients (33.3%) had a Hughes functional grading scale score of 3, 16 (59.3%) had 3 and 2 (7.4%) had 5. Electrophysiological studies were performed in 22 cases (81.4%) and acute inflammatory demyelinating polyneuropathy was detected in 10 cases (45.5%), acute motor axonal neuropathy in 9 cases (40.9%), and acute motor and sensory axonal neuropathy in 1 case (4.5%). Two (9.1%) patients were evaluated as normal. All the patients were administered intravenous immunoglobulin (IVIG). In 12 (52.17%) patients, plasmapheresis was performed for a median of 8 sessions (5-9) before IVIG. Mechanical ventilation was required in 6 patients (22.2%) and was performed for a median of 24 days (5-41). The mean period of time of the first supported sitting was 6 (3-10) days and the mean period of time of the first walking with aid was 9 (7-15) days. Conclusion: Although there have been studies claiming that plasmapheresis was more successful, it has been generally accepted that plasmapheresis and IVIG have the same efficacy. Under the conditions of our country, we believe that both plasmapheresis and IVIG can be safely used at experienced units in patients with Guillain-Barré syndrome who need intensive care treatment