Improved diagnostic effectiveness with a sequential diagnostic paradigm in idiopathic pediatric sensorineural hearing loss.

OBJECTIVES: To determine whether a stepwise diagnostic paradigm is more diagnostically efficient and cost-effective than a simultaneous testing approach in the evaluation of idiopathic pediatric sensorineural hearing loss (SNHL). DESIGN: Prospective prevalence study. SETTING: Tertiary referral children's hospital. PATIENTS: Consecutive children (n = 150) presenting with idiopathic SNHL in the last 2 years. INTERVENTIONS: All children were evaluated with full diagnostic evaluations including GJB2 screens, temporal bone computed tomography scans, and laboratory investigations. MAIN OUTCOME MEASURES: 1) Diagnostic yields of GJB2 screens, imaging, and laboratory results per SNHL category; 2) Cost analysis comparing a sequential versus a simultaneous testing approach. RESULTS: Overall, 12.0% of patients had biallelic mutations in the GJB2 gene, whereas 30% of patients had an abnormality on temporal bone scan. Laboratory testing did not reveal the SNHL etiology in any patient. While maintaining diagnostic accuracy, significant cost savings were inferred by using a sequential diagnostic algorithm. Our data show children with severe to profound SNHL should first be tested with a GJB2 screen, as opposed to those with milder SNHL, who should undergo imaging as the initial testing step. In patients with initially positive GJB2 or imaging screens, logistic regression analysis significantly predicted negative results on further testing. CONCLUSIONS: A stepwise diagnostic paradigm tailored to the level of the hearing loss in children with bilateral SNHL is more diagnostically efficient and cost effective than the more commonly used full, simultaneous testing approach. Laboratory investigation should not be routine but based on clinical history.     

Endoscopic treatment of benign tumors of the nose and paranasal sinuses.

OBJECTIVE: The endoscopic approach can be successfully employed for the treatment of benign tumors such as fibroosseus and vascular lesions, pleomorphic adenomas, gliomas, and schwannomas. STUDY DESIGN AND SETTING: Nineteen patients diagnosed with benign tumors of the nasal cavity and paranasal sinuses and treated using an endoscopic approach were retrospectively reviewed. The endoscopic approach was used exclusively in 15 cases and was associated with an external approach in 4 cases (due to an intracranial extension of the tumor in 3 patients and its location at the level of the anterior wall of the frontal sinus in the last case). RESULTS: The resection of the lesions was radical in 18 patients and subtotal in 1 case. The mean follow-up was 27 months and only 1 recurrence (5%) was observed in the juvenile angiofibroma group 20 months postoperatively. CONCLUSION: and significance In selected cases, endoscopic surgery can be considered an effective treatment for the resection of benign tumors involving the sinonasal tract.     

Actin-free Gc globulin: a rapidly assessed biomarker of organ dysfunction in acute liver failure and cirrhosis.

Reductions in serum levels of Gc globulin, a hepatically synthesized component of the extracellular actin scavenger system responsible for complexing circulating actin and attenuating intravascular microthrombus formation, are associated with poor outcome in acute liver failure. Clinically applicable assays of the important actin-free fraction (Af-Gc) have not been available until now. We measured actin-free Gc globulin levels with a novel, rapid assay in 61 cases of acute liver failure (ALF) and in 91 patients with cirrhosis (40 of whom were clinically unstable with extrahepatic organ dysfunction), and studied associations with liver dysfunction, extrahepatic organ dysfunction, indices of disseminated coagulation, and outcome. Reductions in Af-Gc levels mirrored hepatic dysfunction and organ dysfunction in both groups, and discriminated patients with poor prognosis from those with good prognosis in the ALF cohort. Levels were lowest in patients with ALF (10% of control values), but levels were also markedly reduced in both unstable (28%) and stable (44%) patients with cirrhosis. Associations with markers of disseminated intravascular coagulation were seen in both groups, most notably in the cirrhosis cohort, supporting a pathophysiological role for reduced Af-Gc in the evolution of organ dysfunction. In acetaminophen-induced ALF, Af-Gc identified patients with poor prognosis as well as did the Acute Physiology and Chronic Health Evaluation (APACHE II) score (area under the receiver operating characteristic curve, 0.7), and in cirrhosis, Af-Gc was an independent predictor of mortality by multifactorial analysis. In conclusion, the importance of Af-Gc reductions in the development of multiple organ dysfunction in ALF and cirrhosis is highlighted, probably resulting from reduced hepatic production and peripheral exhaustion of this arm of the extracellular actin scavenger system.     

Arthroscopic Latissimus Dorsi Transfer

The patient is placed in lateral decubitus. A 6-cm incision made in the axilla allows access to the latissimus dorsi tendon and its neurovascular pedicle. Holding the arm in internal rotation, the surgeon detaches sharply the tendon off the humeral shaft and then reinforces it with wrapping sutures. Pulling the free limbs of the sutures exposes the under surface of the muscle and helps to identify the neurovascular pedicle. Special lighting retractors suited for a large diameter scope are helpful. Mobilization is completed when 2 cm of the tendon crosses the posterior edge of the acromion. The standard lateral portal is used for visualization. A silicon drain tube stiffened by a Wissinger rod is advanced from the posterior portal under direct visualization in the space between teres minor and deltoid, exiting in the auxiliary incision. A suture loop passed down the tube retrieves the tendon sutures out the posterior portal. These are then moved out the anterior portal, thus pulling the tendon over the tuberosity. The first anchor is inserted at the anterior aspect of the greater tuberosity, close to the articular cartilage and long head of the biceps tendon. Two to 3 anchors are inserted fixing the tendon to the tuberosity until it is stable.     

Time to REST: epidemiology and burden

Restless legs syndrome (RLS) is a neurological disorder characterized by a compelling urge to move, accompanied by disturbing sensations within the legs. It is now recognized that RLS is a common condition that has a substantial impact on sleep, daily activities and quality of life. Recent data from the REST (RLS Epidemiology, Symptoms, and Treatment) general population study show that symptoms of RLS are present in approximately 7% of the general population, and that 2–3% experience moderate or severe symptoms at least twice a week. Amongst this RLS cohort, approximately 88% reported sensory disturbances and 76% reported sleep-related symptoms. These symptoms had a marked effect on everyday life, with up to 50% of the RLS cohort reporting disruption of everyday activities or personal relationships. Sleep disturbances in turn lead to impaired daytime functioning. RLS is associated with a significant impairment of quality of life, comparable with that seen in chronic medical conditions such as diabetes or depression. Given the significant burden of morbidity associated with RLS, there is a strong case for treatment in patients with troublesome symptoms.     

Correlates of suicide in building industry workers.

Suicide within the construction industry in Queensland, Australia was reportedly high in a recent Royal Commission report. The current study examined the incidence and causes of suicide in this industry using psychological autopsy and focus group investigations. A total of 64 male suicides occurred over the seven-year period, representing a crude suicide rate of 40.3 per 100,000, significantly greater than the working age Australian male rate. Young employees were at excessive risk with separation/divorce, relationship problems, and untreated psychiatric conditions the major contributors. Focus groups emphasized the importance of work/home interface factors and industry-specific factors preceding suicide.     

Computed tomography in gunshot wounds to the neck: Can we predict vascular injury?

The purpose of this study was to evaluate the ability of helical computed tomography (CT) to detect arterial injuries in gunshot wounds to the neck. In a blinded retrospective review, 54 helical CT scans of the cervical spine were evaluated for bullet/bone fragments, subcutaneous air, bullet path, hematoma, spine fractures, and pharyngoesophageal compromise. The distance of fragments to a major vessel was calculated. CT findings that correlated significantly with major arterial injury included the presence of fragments (bullet/bone) close to a major vessel (2.5 mm) and spine fractures. Visualizing fragments <5 mm from a vessel or a transcervical bullet trajectory predicted 12 of 13 major arterial injuries. We conclude that CT clearly depicts anatomic damage. Specific findings, such as the location of fragments and bullet trajectory adjacent to a vessel and spine fractures, indicate a higher probability of vascular damage, thus directing more definitive evaluation.     

Popliteal artery entrapment syndrome

Three cases of popliteal artery entrapment syndrome, one of them bilateral, are presented with a review of the literature, with special reference to the embryological development of the popliteal space and the diagnostic and therapeutic problems presented by this syndrome.     

Cancer risk in farmers: Results from a multi-site case-control study in north-eastern italy

The relationship between farming and cancer risk was investigated in an integrated series of case-control studies conducted from 1985 to 1991 in the Friuli Venezia Giulia region, north-east Italy. Patients with cancer of the oral cavity and pharynx, larynx, oesophagus, colon and rectum, pancreas, breast, thyroid gland, kidney and urinary tract, bladder, prostate, soft-tissue sarcomas, Hodgkin's diseases, non-Hodgkin's lymphomas and multiple myelomas, and controls admitted to hospital for acute, non-neoplastic conditions, were interviewed. For males, a significantly elevated relative risk was seen for oral cavity and pharynx. Farming, however, was associated with a significant protection against cancer of the colon and rectum and bladder. In females, only one significant association emerged, for multiple myeloma. A few significant interactions between cancer risk and year of birth (i.e., before 1930 or 1930 and after) were observed. The risk of cancer of the larynx was significantly elevated in younger male farmers but not in older ones. Our multi-site case-control study confirms that farmers have, for some cancer sites, a distinctive pattern. Excesses of cancer of the oral cavity and pharynx in farmers are characteristic of the present study area and, possibly, of similar European rural populations who have in common high levels of alcohol consumption and, at least in the past, unbalanced diets.