Mid-Aortic Syndrome in a 3-Year-Old Girl Successfully Treated by Aorto�CAortic Grafting and Renal Artery Implantation into the Graft

Mid-aortic syndrome, an uncommon acquired or congenital condition characterized by segmental narrowing of the abdominal or distal descending thoracic aorta, is frequently accompanied by ostial stenosis of the aorta''s branches. If left untreated, it can result in life-threatening complications secondary to severe hypertension.We report the case of a 3-year-old girl with congenital mid-aortic syndrome, who was diagnosed by chance in the course of a viral illness, and whose high blood pressure values were first dismissed as inaccurate. Attempts to achieve medical or endovascular control of her hypertension were unsuccessful. She was thereafter successfully treated by aorto–aortic bypass grafting, resection of the stenotic segments of both renal arteries, and implantation of the patent arterial segments into the graft.Key words: Angioplasty, balloon; aorta, abdominal/abnormalities; aortic coarctation/etiology/surgery; arterial occlusive diseases/surgery; child; hypertension, renal/etiology/surgery; mid-aortic syndrome; reconstructive surgical procedures/methods; renal artery obstruction/surgeryMid-aortic syndrome, an uncommon condition characterized by segmental narrowing of the abdominal or distal descending thoracic aorta, is frequently accompanied by ostial stenosis of its branches.^1–5 It can be acquired or congenital. Acquired causes of mid-aortic syndrome include neurofibromatosis, fibromuscular dysplasia, retroperitoneal fibrosis, Williams syndrome, mucopolysaccharidosis, giant cell arteritis (Takayasu disease, temporal arteritis), and acquired insults in utero or in early life that result in developmental disorders of the growing aorta.^1–13 Congenital mid-aortic syndrome is caused by a developmental anomaly in the fusion and maturation of the paired embryonic dorsal aortas and typically manifests itself in young patients.^6,14–19 We report the case of a 3-year-old girl with congenital mid-aortic syndrome, who was diagnosed by chance in the course of a viral illness and in whom high blood pressure values had at first been dismissed as inaccurate. Attempts to achieve medical or endovascular control of her hypertension were unsuccessful, so she was treated surgically.     

Variability in Stroop Task Performance and Functional Activation among a Small Brain-Injured Group

This study evaluated the effects of moderate to severe brain injury on cognitive task performance and cortical activation. Five participants completed a Stroop task while undergoing functional magnetic resonance imaging (fMRI) at two time points post-injury. Results revealed activation within regions typically activated during a Stroop task (the region of interest: ROI), though variability among participants was evident. Regions outside of the ROI were activated among all participants, to a greater degree than was present within the ROIs. This finding may indicate that recruitment of outside regions was necessary for successful task completion at both time points, and may suggest functional plasticity in cognitive task completion.     

Homozygous dystroglycan mutation associated with a novel muscle–eye–brain disease-like phenotype with multicystic leucodystrophy

Defects in dystroglycan post-translational modification result in congenital muscular dystrophy with or without additional eye and brain involvement, are referred to as secondary dystroglycanopathies and have been associated with mutations in 11 different genes encoding glycosyltransferases or associated proteins. However, only one patient with a mutation in the dystroglycan encoding gene DAG1 itself has been described before. We here report a homozygous novel DAG1 missense mutation c.2006G>T predicted to result in the amino acid substitution p.Cys669Phe in the β-subunit of dystroglycan in two Libyan siblings. The affected girls presented with a severe muscle–eye–brain disease-like phenotype with distinct additional findings of macrocephaly and extended bilateral multicystic white matter disease, overlapping with the cerebral findings in patients with megalencephalic leucoencephalopathy with subcortical cysts. This novel clinical phenotype observed in our patients further expands the clinical spectrum of dystroglycanopathies and suggests a role of DAG1 not only for dystroglycanopathies but also for some forms of more extensive and multicystic leucodystrophy.     

Falsely Elevated Sodium Levels During Thiopental Treatment in the ICU: Technical Interference on a Laboratory Device with Important Clinical Relevance

Introduction

Thiopental is a cornerstone in the treatment of refractory status epilepticus and intractable intracranial hypertension. In our center we observed that thiopental might cause falsely elevated serum sodium levels.

Methods

Triggered by a recent case experience of extremely elevated serum sodium levels during thiopental treatment, we retrospectively identified 53 patients treated with thiopental in our intensive care unit between 2007 and 2011 and evaluated electrolyte changes. We differentiated the analysis before and after introduction of a new device for sodium assays (Dimension Vista, Siemens) in the central laboratory in April 2010. Standardized in vitro laboratory tests were performed to study the effect of thiopental on sodium analysis.

Results

Before April 2010, serum sodium levels determined in the central laboratory showed a good agreement with the bedside point-of-care (POC) device during thiopental therapy with [sodium]_laboratory ? [sodium]_POC of only 1.08 mmol/L (P = .0517). After April 2010, a strong discrepancy between laboratory values and POC values was observed with [sodium]_laboratory ? [sodium]_POC = 11.57 mmol/L (P < .0001). Standardized in vitro testing confirmed that thiopental induced a dose-dependent false hypernatremia (P = .002).

Conclusions

Thiopental treatment can result in falsely elevated serum sodium. This is a critical finding since high sodium levels preclude administrating mannitol or hypertonic saline for the treatment of elevated intracranial pressure. Moreover, a false high sodium level might lead to the inappropriate administration of hypotonic fluids potentially resulting in increased brain edema and even higher intracranial pressure. To our knowledge, this is the first paper describing this clinically relevant phenomenon.     

Change in Proportional Protein Intake in a 10-Week Energy-Restricted Low- or High-Fat Diet,in Relation to Changes in Body Size and Metabolic Factors

ObjectiveTo investigate in a secondary analysis of a randomised trial the effects of a low-/high-fat diet and reported change from baseline in energy% from protein (prot%), in relation to changes in body size and metabolic factors.MethodsObese adults (n = 771) were randomised to a 600 kcal energy-deficient low-fat (20-25 fat%) or high-fat (40-45 fat%) diet over 10 weeks. Dietary intake data at baseline and during the intervention were available in 585 completers. We used linear regression to calculate the combined effects of randomised group and groups of prot% change (<−2 /−2 to 2/>2) on outcomes.ResultsThe low-fat group with >2 prot% increase lost 1.1 kg more weight (p = 0.03) and reduced cholesterol by 0.25 mmol/l more (p = 0.003) than the high-fat group with >2 prot% decrease. These differences were 2.5-fold and 1.8-fold greater than the differences between the low-fat and high-fat groups while not considering prot% change. The high-fat group reduced plasma triglycerides more than the low-fat group, but not compared to those in the low-fat group with >2 units prot% increase (p fat-protein interaction = 0.01).ConclusionsUnder energy restriction, participants on a low-fat diet who had increased the percentage energy intake from protein showed the greatest reduction in weight and cholesterol, and a triglyceride reduction equally large to that of participants on a high-fat diet.Key Words: Obesity, Dietary fats, Dietary proteins, Lipids, Blood glucose     

Umgang mit Massivblutungen und assoziierten perioperativen Gerinnungsstörungen

Massive bleeding with coagulopathy and hemorrhagic shock poses a potential threat to life in numerous clinical settings. Optimal treatment including the prevention of exsanguination necessitates a standardized and interdisciplinary approach. Several studies have shown the importance of massive transfusion protocols and standardized coagulation algorithms to improve survival of severely bleeding patients and to avoid secondary complications. Thus, the Helsinki declaration for patient safety in anesthesiology demands the implementation of clinical practice guidelines for the treatment of patients requiring massive transfusion. This paper introduces a standardized algorithm for the treatment of patients with massive bleeding which was developed in consensus with the German Society of Anaesthesiology and Intensive Care Medicine (DGAI).