Therapeutic thoracic duct drainage: A systematic review of the Eastern European experience and future potential.

Thoracic duct drainage (TDD) is gaining renewed interest, largely due to accumulation of evidence supporting the gut-lymph model, where toxic mesenteric lymph from the intestine contributes to development of multi-organ failure in acute and critical illness (ACI). Advances in minimally invasive TDD have added to this growing interest. The English TDD literature has been previously reviewed, but the more extensive Eastern European literature has not been available to English readers. Therefore, we undertook a systematic search of Eastern European human TDD studies using Scopus and PubMed databases and Russian language websites. Indications for TDD, clinical outcomes, and complications were reviewed. 113 studies, published between 1965 and 2015, were reviewed. The most common indications for TDD were hepatic failure, acute pancreatitis, and peritonitis. It was often used late and when other treatment options had been exhausted. Human TDD appeared safe and probably effective, especially when combined with lymphosorption. The benefit appeared to correlate with the volume of lymph drained. A randomized controlled trial (and some case-control studies) showed reduced mortality in patients with ACI with TDD. Other benefits included rapid normalization of blood parameters and decreased organ edema. This review provides further support for the gut-lymph model and justification for high quality randomized controlled trials of TDD in ACI. It also highlights other potential indications for TDD, such as bridging patients with liver failure to surgery or transplant.

The effect of preexisting hypertension on early neurologic results of patients with an acute spinal cord injury.

STUDY DESIGN: Retrospective case-control. OBJECTIVES: To characterize changes in American Spinal Injury Association Motor Score (AMS) in patients treated with relative hypertension (HTN) (mean arterial pressure (MAP) > 85 mm Hg for 5 days) with and without preexisting HTN. SETTING: A regional spinal cord injury (SCI) center in Pennsylvania, United States. METHODS: All patients with an acute SCI who were treated with induced HTN (MAP goal above 85) in the intensive care unit (ICU) for at least 5 days were identified. Patients were stratified based on the presence of preexisting HTN, and the change in the AMS between admission and day 5 was determined. Predictors of outcome were identified using correlation analysis and multiple linear regression. RESULTS: Ninety-two patients met inclusion criteria of which 22 had a previous history of HTN. HTN was a predictor of poor early outcome. Patients with HTN had an average decline in their AMS of 7.6, compared with an average decrease of only 0.6 in the AMS of patients without HTN (P=0.04). HTN had no effect (P>0.05) on other in-hospital variables including length of stay, length of stay in the ICU, complications or mortality. Additionally, multiple linear regression analysis demonstrated that diabetes, coronary artery disease and pulmonary disease had no effect on the change in AMS. CONCLUSION: Chronic HTN is an independent risk factor for poor early neurologic recovery in patients treated with relative HTN for an acute SCI. This is independent of age and other comorbidities.

Clinical outcomes following invasive versus noninvasive preoperative stabilization of closed diaphyseal femur fractures.

BACKGROUND: The use of invasive traction (INV-T) to stabilize femur fractures prior to fixation (open reduction and internal fixation, ORIF) remains controversial. Some centers have utilized noninvasive traction (NINV-T) or splinting preoperatively. It is possible that INV-T decreases hemorrhage. However, the use of INV-T in pediatric patients and for femoral neck fractures in adults is associated with worsened outcomes. We hypothesized that there is no difference in the need for transfusion between those who receive INV-T and NINV-T. METHODS: A retrospective study was performed at two level I and one level II trauma center from January 2006 to December 2009. Patients ≥18 years with a closed diaphyseal femur fracture who underwent ORIF within 48 h of arrival were included. Patients were grouped by method of preoperative fracture stabilization. Primary endpoint was need for transfusion. A power analysis found that 94 patients were needed to detect a 25 % difference with 80 % power. RESULTS: Fifty-six (22 %) received INV-T and 199 (78 %) received NINV-T stabilization. No significant differences were found between groups in terms of age, injury severity score, or ORIF method. There was no significant difference between the two groups in the hemoglobin value on arrival, preoperative hemoglobin value, or the difference between admission and preoperative hemoglobin values. We did not find a significant difference in the need for red blood cell transfusion between the two groups. There was no difference in length of stay or discharge destination. CONCLUSION: INV-T is not associated with improved outcomes in adult patients with closed mid-shaft femoral fractures who are operated upon within 48 h of arrival.

New height, weight and head circumference charts for British children with Williams syndrome.

AIM: To produce a growth reference for British children with Williams syndrome. METHODS: The children and adults recruited into the study were all affiliated to the Williams Syndrome Foundation, a parent support group founded in 1979. They have all been shown to have a deletion of chromosome 7q11.23. One growth nurse (WRS) prospectively measured the weight, height and head circumference of individuals from 19 regions in Great Britain including Scotland, England and Wales. 169 children and adults were measured on up to four occasions between 2001 and 2004 (275 measurements). In addition, retrospective data were obtained from the hospital notes of 67 of these individuals (586 measurements). Centile curves were constructed using Cole's LMS method. RESULTS: The centile charts differ from charts previously derived in the USA and Germany and provide more appropriate standards for the British population. CONCLUSIONS: We propose that these charts be adopted for routine clinical practice as abnormalities in growth are an important feature of this syndrome.

Autosomal recessive lissencephaly with cerebellar hypoplasia is associated with human RELN mutations.

Normal development of the cerebral cortex requires long-range migration of cortical neurons from proliferative regions deep in the brain. Lissencephaly ("smooth brain," from "lissos," meaning smooth, and "encephalos," meaning brain) is a severe developmental disorder in which neuronal migration is impaired, leading to a thickened cerebral cortex whose normally folded contour is simplified and smooth. Two identified lissencephaly genes do not account for all known cases, and additional lissencephaly syndromes have been described. An autosomal recessive form of lissencephaly (LCH) associated with severe abnormalities of the cerebellum, hippocampus and brainstem maps to chromosome 7q22, and is associated with two independent mutations in the human gene encoding reelin (RELN). The mutations disrupt splicing of RELN cDNA, resulting in low or undetectable amounts of reelin protein. LCH parallels the reeler mouse mutant (Reln(rl)), in which Reln mutations cause cerebellar hypoplasia, abnormal cerebral cortical neuronal migration and abnormal axonal connectivity. RELN encodes a large (388 kD) secreted protein that acts on migrating cortical neurons by binding to the very low density lipoprotein receptor (VLDLR), the apolipoprotein E receptor 2 (ApoER2; refs 9-11 ), alpha3beta1 integrin and protocadherins. Although reelin was previously thought to function exclusively in brain, some humans with RELN mutations show abnormal neuromuscular connectivity and congenital lymphoedema, suggesting previously unsuspected functions for reelin in and outside of the brain.

A sibship with a neuronal migration defect, cerebellar hypoplasia and congenital lymphedema.

We describe a sibship of three males, including monozygous twins, with cerebral and cerebellar malformations and congenital lymphedema. The parents of these children are related, being half second cousins. The clinical, radiologic and histopathologic features do not fit a previously recognized pattern. We feel this sibship represents a syndrome that has not been previously described, though it closely resembles the Walker Warburg syndrome.

An in vivo method for the evaluation of catheter thrombogenicity.

A new method has been developed to evaluate the relative thrombogenicity of vascular catheters. The technique provides a means to quantitatively differentiate between catheters made from different polymeric materials. Autologous In-111 labeled platelets were infused into a dog model and catheters were then inserted into the external jugular vein of the dog. The neck region was scanned using gamma camera imaging. Comparisons between catheter materials were made using computer generated uptake slopes during the first 40 min of the scan. In addition to scintigraphy, visual assessment of thrombus deposition, thrombus weight, platelet deposition, and scanning electron microscopy were used to validate the technique. Poly(vinyl chloride), polyurethane, heparinized polyurethane, and silicone catheter materials were tested. It was found that heparinized polyurethane was the least thrombogenic of all materials evaluated.

Age at diagnosis and abilities in idiopathic hypercalcaemia.

Forty three children aged 6 to 16 years with a documented history of idiopathic infantile hypercalcaemia were assessed on a variety of cognitive and behavioural measures. No relation was found between the age at which hypercalcaemia was diagnosed and subsequent full scale intelligence quotients (IQ) and reading or spelling abilities. Verbal IQ was, however, significantly higher than performance IQ, which had an inverse relation with age at diagnosis. Furthermore, scores for deviant behaviour were found to correlate with age at diagnosis. Thus the characteristic psychological profile of this syndrome may be in part a function of hypercalcaemia during infancy.

Vitamin D metabolites in idiopathic infantile hypercalcaemia.

Metabolites of vitamin D were measured in plasma from 83 patients with idiopathic infantile hypercalcaemia syndrome who were mentally handicapped but had normal calcium values at the time of the study. No significant difference was detected in the mean plasma concentrations of 25-hydroxyvitamin D2, 1,25-dihydroxyvitamin D, 24,25-dihydroxyvitamin D3, or 25,26-dihydroxyvitamin D3 between patients and age matched controls. The mean plasma concentration of 25-hydroxyvitamin D3 was significantly lower in patients than controls but this may be a secondary phenomenon related to less sunlight exposure. In addition, two hypercalcaemic patients with this syndrome were studied during the first year of life, and were found to have normal concentrations of vitamin D metabolites. These findings do not support a role for abnormal vitamin D metabolism in the pathogenesis of this syndrome.

Exaggerated psychophysiological reactivity: issues in quantification and reliability.

Marked physiological reactivity to challenging mental tasks has been associated with elevated risk for, as well as the presence of, coronary heart disease. However, little systematic enquiry into the reliability and quantification of such exaggerated reactivity has emerged. Subjects were 32 male, managerial employees, ranging in age from 22 to 56 yr, who satisfied the following criteria: no history or current signs of heart disease, presence of Type A behavior pattern as revealed by the Structured Interview, and an increase during an initial psychosocial stress testing of at least 25% over baseline in at least three out of five psychophysiological indices. Heart rate, systolic blood pressure, diastolic blood pressure, plasma epinephrine and plasma norepinephrine levels were monitored while challenging mental tasks were performed in three sessions (screening, pretraining and posttraining) spaced several weeks apart. Psychophysiological reactivity during the tasks emerged as a consistent trait. For all five measures, change scores from baseline during the screening session were significantly correlated with change scores during the pretraining session. Moreover, the magnitude of the change scores were similar in the screening and pretraining sessions. Analysis of cross correlations within and between indices provided little support for the use of data transformations such as residual scores or analysis of covariance. Finally, on four out of five measures, the challenging tasks were found to be comparable in the degree of reactivity elicited. These findings suggest that, for selected Type A men, exaggerated psychophysiological reactivity occurs reliably when monitored with multiple indices, appears insensitive to mere passage of time, and can be uniformly elicited by a variety of tasks.

Idiopathic infantile hypercalcaemia--a continuing enigma.

Seventy six children with documented Fanconi-type idiopathic infantile hypercalcaemia were studied and compared with 41 with the Williams-Beuren syndrome. Clinical comparison showed, as expected, very close similarities but also considerable differences, particularly in the severity of feeding problems and the degree of failure to thrive. The estimated incidence of idiopathic infantile hypercalcaemia alone has remained constant for the past 20 years, at approximately 18 cases per year in the United Kingdom (1 per 47 000 total live births). Long term morbidity in these children is mainly due to mental handicap and arteriopathy, but hypertension (29%), kyphoscoliosis (19%), hyperacusis (75%), and obesity (50%) may be added complications. In one child, hypercalcaemia recurred during adolescence but this seems to be excessively rare. More detailed investigation before treatment is required to discover the aetiology of hypercalcaemia in this condition.

Sarcoidosis presenting with pleurisy and bilateral pleural effusions.

A patient with acute sarcoidosis is described who presented with pleurisy and bilateral pleural effusions, but no other abnormalities on examination or chest X-ray. This presentation of sarcoidosis is unusual and is discussed.

Endogenous serum TSH levels and metastatic survey scans in thyroid cancer patients using triiodothyronine withdrawal.

Using a triiodothyronine withdrawal method to produce a hypothyroid state in 38 patients with prior thyroidectomies for differentiated thyroid cancer, 56 H-TSH and T4 levels were measured prior to metastatic survey scans performed with 131I. All the T4 levels were below normal and 55 of the 56 H-TSH levels were elevated. No qualitative differences could be observed in the eight patients who had scans performed after bovine TSH injections and later after triiodothyronine withdrawal. Triiodothyronine withdrawal does induce high endogenous TSH levels and under normal circumstances bovine TSH injections are not required for metastatic scans in patients with thyroid cancer.

Thyroid function and growth hormone secretion in amitriptyline-treated depression.

The authors studied changes in indices of thyroid function prospectively in a group of 11 patients given amitriptyline to treat depression. The drug caused no significant alteration in these indices, but scores on the Hamilton Depression Rating Scale improved significantly. In another group of subjects with depression, the stimulation of growth hormone secretion by L-dopa was unaffected by amitriptyline therapy.

Noninvasive myocardial imaging with potassium-43 and rubidium-81 in patients with left bundle branch block.

Noninvasive myocardial imaging with potassium-43 and rubidium-81 has been used successfully to identify areas of infarction and exercise-induced ischemia as regions of decreased radioactivity. The image defects observed are believed to be due to a decreased radionuclide uptake in regions of myocardial scar or to heterogeneous myocardial accumulation of tracer as a result of regional ischemia. Of 27 patients with left bundle branch block studied with noninvasive imaging at rest and during exercise, 25 manifested at rest reduced radioactivity in the region of the interventricular septum. This pattern is similar to that seen in patients with anteroseptal myocardial infarction. Sixteen of the 27 patients underwent diagnostic coronary arteriography and left ventriculography. Only five of these patients had evidence of either previous infarction or significant obstructive coronary artery disease as assessed with clinical or angiographic criteria, or both. Although the image defect was routinely demonstrated at rest in patients with left bundle branch block, this defect was generally normalized or less distinct with exercise in patients with no anatomic heart disease. In contrast, a larger, more distinct or new image defect with exercise correctly identified the presence of significant obstructive coronary artery disease in patients with left bundle branch block. In the clinical application of noninvasive myocardial imaging, these image defects observed at rest can lead to the false pasitive radionuclide interpretation of anteroseptal myocardial infarction.

A community dental health project. I. Self applied SnF2-ZrSiO4 prophylactic paste and dental caries in primary school children.

A dental health programme which employed group self prophylaxis by school children aged 5-9 years is described. The group used 10 per cent SnF2-ZrSiO4 paste and was supervised within the school by an auxiliary of parents under the direction of a dentist. There was a 36 per cent reduction in DMFT increment at the end of two years in the children aged 7-9 years.