A Case of Pulmonary Artery Sling Anomaly with Tracheal Stenosis and Management of Difficult Airway.

Pulmonary artery sling is a rare congenital vascular abnormality, where the left pulmonary artery originates from the right pulmonary artery, passes between trachea, and esophagus and reaching the left hilum. Cough, wheezing, and difficulty in feeding are three major symptoms. Untreated pulmonary sling carries high morbidity and mortality, most of which is due to the airway and other associated anomalies. Herein, we reported a 40-day-old male infant who admitted to the pediatric intensive care unit with progressive respiratory distress and diagnosed with left pulmonary sling with tracheal stenosis. We discussed the diagnosis and management of pulmonary artery sling and present the successful use of laryngeal mask in difficult airway management.

Expanding the clinical spectrum of mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase deficiency with Turkish cases harboring novel HMGCS2 gene mutations and literature review.

Mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase (mHS) deficiency is a very rare autosomal recessive inborn error of ketone body synthesis and presents with hypoketotic hypoglycemia, metabolic acidosis, lethargy, encephalopathy, and hepatomegaly with fatty liver precipitated by catabolic stress. We report acute presentation of two patients from unrelated two families with novel homozygous c.862C>T and c.725-2A>C mutations, respectively, in HMGCS2 gene. Affected patients had severe hypoketotic hypoglycemia, lethargy, encephalopathy, severe metabolic and lactic acidosis and hepatomegaly after infections. Surprisingly, molecular screening of the second family showed more affected patients without clinical findings. These cases expand the clinic spectrum of this extremely rare disease.

Evaluation of Thiol/Disulfide Homeostasis in Pediatric Patients with Diabetic Ketoacidosis.

AIMS AND OBJECTIVE: This study aimed to investigate the value of Thiol/Disulfide homeostasis in pediatric diabetic ketoacidosis patients suffering from type 1 diabetes mellitus. MATERIALS AND METHODS: This study featured children who were diagnosed with diabetic ketoacidosis and who were consecutively admitted to pediatric intensive care within one year of their diagnosis. Thiol/disulfide homeostasis was evaluated in 45 pediatric patients suffering from DKA, as well as 45 healthy controls of parallel gender and age. Thiol/disulfide homeostasis parameters were measured using a novel automated measurement method and the correlation between demographic data and parameters was measured. RESULTS: Pediatric patients were found to have low native thiols, total thiols and disulfide levels with type 1 diabetes after DKA (331.82±106.40, 362.71±113.31, 17.02±5.33 µmol/L, respectively) as compared to the control group (445.08±24.41, 481.21± 28.47, 18.06±5.12 µmol/L, respectively). CONCLUSION: Thiol/disulfide homeostasis was distorted in pediatric patients with DKA. Furthermore, it was found that they are not likely to return to normal, immediately after treatment.

Role of Ischemia Modified Albumin Serum Levels as an Oxidative Stress Marker in Children with Diabetic Ketoacidosis.

AIM AND OBJECTIVE: Ischemia modified albumin (IMA) is a biomarker that has been introduced recently for use in the evaluation of oxidative stress. The aim of this study was to measure the ischemia modified albumin serum levels in pediatric patients with diabetic ketoacidosis (DKA) during acidosis and after the patient recovered from acidosis and to compare these with the control group. MATERIALS AND METHODS: Pediatric patients with Type I diabetes mellitus (T1DM) who were admitted to the pediatric intensive care unit with the diabetic ketoacidosis were assigned as the study group and healthy children who were admitted to the outpatient clinic and decided as healthy after clinic and laboratory evaluation were selected as the control group. IMA and adjusted IMA levels were evaluated in the blood samples from the control group and the study group when admitted first time to the intensive care unit during the acidosis period (DKA before treatment, DKA-BT), and after recovering from acidosis (DKA after treatment, DKA-AT). RESULTS: A total of 24 pediatric patients with diabetic ketoacidosis and 30 healthy control children matching age and sex were included in the current study. The albumin levels in pediatric patients with T1DM during DKA-BT were higher than the albumin levels after acidosis (4.101±0.373, 3.854±0.369 g/dL, respectively) (p<0.05). However, there was no significant difference when these values were compared to the control group. Mean values of IMA and Adj-IMA were statistically higher in DKAAT compared to the control group (0.748±0.150 vs 0.591±0.099, p< 0.001; 0.708±0.125 vs 0.607±0.824, p< 0.001, respectively). IMA and adjusted IMA levels measured after recovered from acidosis were significantly higher compared to the level of IMA during DKA (0.748±0.150 vs 0.606±0.105 as absorbance unit, p<0.001; 0.708±0.125 vs 0.625±0.100, p<0.05, respectively). CONCLUSION: In children with T1DM, even though acidosis recovered following the treatment in diabetic ketoacidosis, which is an oxidative stress marker, the ischemia modified albumin levels and adjusted ischemia modified albumin levels were high.

Stress induced gastrointestinal bleeding in a pediatric intensive care unit: which risk factors should necessitate prophilaxis?

BACKGROUND: The aim of this study was to determine the frequency and the risk factors of stress induced gastrointestinal bleeding (GIB) in critically ill children, and to investigate the effect of prophilaxis. The setting was a 14-beded, tertiary care PICU. METHODS: Records of 182 children admitted consecutively from December 2012 to May 2013 were retrospectively reviewed. 136 patients were eligible. The age ranged from 40 days to 18 years. Diagnosis, demographic data, risk factors, administration of prophilaxis, drugs used in medication, prescence and degree of GIB and complications were recorded. RESULTS: The male-female ratio was 1.3. Mean age was 5.9. Mean PRISM III score was 12.2 and 49.3% had PRISM Score ≥10. Most frequent diagnosis was infectious diseases. Sixtyone (44.9%) children received prophylaxis in which antacids was used in 28 (45.9%), sucralfate in 18 (29.5%), proton pomp inhibitors (PPIs) in 51 (83.6%) and 5 (8.2%) received H2 reseptor antagonist. The incidence of GIB was 15.4% (N.=21), in which 66.7% (N.=14) were mild, 23.8% (N.=5) were moderate, 4.8% (N.=1) was significant and 4.8% (N.=1) was massive. In children who received prophylaxis 17 (27.9%) cases developed GIB. Mechanical ventilation was found to be the only risk factor significantly associated with stress induced GIB. Also; mechanical ventilation and trauma was strongly significant (P<0.001) and coagulopathy/thrombocytopenia, PRISM III ≥10, renal and hepatic failure, hypotension, and heart failure/arrhythmia was found to be associated with the development of GIB in critically ill children (P<0.05). CONCLUSION: GIB is a serious concern for PICU clinicians and intensivists are confused about the conflicting evidence supporting prophilaxis. We believe that prophylaxis could be beneficial for mechanically ventilated children. Also trauma, coagulopathy/thrombocytopenia, PRISM III≥10, renal and hepatic failure, hypotension, and heart failure/arrhythmia must be kept in mind as risk factors requiring attention in PICU setting.