Global quantification of left ventricular myocardial perfusion at dynamic CT imaging: Prognostic value

Background

There is no published data on the prognostic value of global myocardial perfusion values at stress dynamic CT myocardial perfusion imaging (CTMPI).

Effect of adhesion boosters on indirect bracket bonding

Objective:To determine the influence of two adhesion boosters on shear bond strength and on the bond failure location of indirectly bonded brackets.Materials and Methods:Sixty bovine incisors were randomly divided into three groups (n  =  20), and their buccal faces were etched using 37% phosphoric acid. In group 1 (control), brackets were indirectly bonded using only Sondhi adhesive. In groups 2 and 3, the adhesion boosters Enhance Adhesion Booster and Assure Universal Bonding Resin, respectively, were applied before bonding with Sondhi. Maximum bond strength was measured with a universal testing machine, and the location of bond failure was evaluated using the Adhesive Remnant Index (ARI). One-way analysis of variance followed by the Tukey test (P < .05) was used to compare the shear bond strength among groups, and the differences in ARI scores were evaluated using the Kruskal-Wallis test (P < .05). The Pearson correlation coefficient was calculated to determine whether there was any correlation between bond strength and ARI scores.Results:The mean shear bond strength in group 3 was significantly higher (P < .01) than in the other groups. Evaluation of the locations of bond failure revealed differences (P < .05) among the three groups. There was a moderate correlation between bond strength and ARI scores within group 3 (r  =  0.5860, P < .01).Conclusion:In vitro shear bond strength was acceptable in all groups. The use of the Assure adhesion booster significantly increased both the shear bond strength of indirectly bonded brackets and the amount of adhesive that remained on the enamel after bracket debonding.     

The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease

OBJECT: The goals of this study were to define the natural history and growth pattern of hemangioblastomas of the central nervous system (CNS) that are associated with von Hippel-Lindau (VHL) disease and to correlate features of hemangioblastomas that are associated with the development of symptoms and the need for treatment. METHODS: The authors reviewed serial magnetic resonance images and clinical histories of 160 consecutive patients with VHL disease who harbored CNS hemangioblastomas and serially measured the volumes of tumors and associated cysts Six hundred fifty-five hemangioblastomas were identified in the cerebellum (250 tumors), brainstem (64 tumors, all of which were located in the posterior medulla oblongata), spinal cord (331 tumors, 96% of which were located in the posterior half of spinal cord), and the supratentorial brain (10 tumors). The symptoms were related to a mass effect. A serial increase in hemangioblastoma size was observed in cerebellar, brainstem, and spinal cord tumors as patients progressed from being asymptomatic to symptomatic and requiring surgery (p < 0.0001). Twenty-one (72%) of 29 symptom-producing cerebellar tumors had an associated cyst, whereas only 28 (13%) of 221 nonsymptomatic cerebellar tumors had tumor-associated cysts (p < 0.0001). Nine (75%) of 12 symptomatic brainstem tumors had associated cysts, compared with only four (8%) of 52 nonsymptomatic brainstem lesions (p < 0.0001). By the time the symptoms appeared and surgery was required, the cyst was larger than the causative tumor; cerebellar and brainstem cysts measured 34 and 19 times the size of their associated tumors at surgery, respectively. Ninety-five percent of symptom-producing spinal hemangioblastomas were associated with syringomyelia. The clinical circumstance was dynamic. Among the 88 patients who had undergone serial imaging for 6 months or longer (median 32 months), 164 (44%) of 373 hemangioblastomas and 37 (67%) of 55 tumor-associated cysts enlarged. No tumors or cysts spontaneously diminished in size. Symptomatic cerebellar and brainstem tumors grew at rates six and nine times greater, respectively, than asymptomatic tumors in the same regions. Cysts enlarged seven (cerebellum) and 15 (brainstem) times faster than the hemangioblastomas causing them. Hemangioblastomas frequently demonstrated a pattern of growth in which they would enlarge for a period of time (growth phase) and then stabilize in a period of arrested growth (quiescent phase). Of 69 patients with documented tumor growth, 18 (26%) harbored tumors with at least two growth phases. Of 160 patients with hemangioblastomas, 34 patients (median follow up 51 months) were found to have 115 new hemangioblastomas and 15 patients new tumor-associated cysts. CONCLUSION: In this study the authors define the natural history of CNS hemangioblastomas associated with VHL disease. Not only were cysts commonly associated with cerebellar, brainstem, and spinal hemangioblastomas, the pace of enlargement was much faster for cysts than for hemangioblastomas. By the time symptoms appeared, the majority of mass effect-producing symptoms derived from the cyst, rather than from the tumor causing the cyst. These tumors often have multiple periods of tumor growth separated by periods of arrested growth, and many untreated tumors may remain the same size for several years. These characteristics must be considered when determining the optimal timing of screening for individual patients and for evaluating the timing and results of treatment.     

Lumbosacral epidural lipomatosis: MRI grading

Lumbosacral epidural lipomatosis (LEL) is characterized by excessive deposition of epidural fat (EF). The purpose of our retrospective study was to quantify normal and pathologic amounts of EF in order to develop a reproducible MRI grading of LEL. In this study of 2528 patients (1095 men and 1433 women; age range 18–84 years, mean age 47.3 years) we performed a retrospective analysis of MRI exams. We obtained four linear measurements at the axial plane parallel and tangent to the superior end plate of S1 vertebral body: antero-posterior diameter of dural sac (A-Pd DuS), A-Pd of EF, located ventrally and dorsally to the DuS, and A-Pd of the spinal canal (Spi C). We calculated (a) DuS/EF index and (b) EF/Spi C index. We developed the following MRI grading of LEL: normal, grade 0: DuS/EF index ≥1.5, EF/Spi C index ≤40%; LEL grade I: DuS/EF index 1.49–1, EF/Spi C index 41–50% (mild EF overgrowth); LEL grade II: DuS/EF index 0.99–0.34, EF/Spi C index 51–74% (moderate EF overgrowth); LEL grade III: DuS/EF index ≤0.33, EF/Spi C index ≥75% (severe EF overgrowth). The MRI exams were evaluated independently by three readers. Intra- and interobserver reliabilities were obtained by calculating Kappa statistics. The MRI grading showed the following distribution: grade 0, 2003 patients (79.2%); LEL grade I, 308 patients (12.2%); LEL grade II, 165 patients (6.5%); and LEL grade III, 52 patients (2.1%). The kappa coefficients for intra- and interobserver agreement in a four-grade classification system were substantial to excellent: intraobserver, kappa range 0.79 [95% confidence interval (CI), 0.65–0.93] to 0.82 (95% CI, 0.70–0.95); interobserver, kappa range 0.76 (95% CI, 0.62–0.91) to 0.85 (95% CI, 0.73–0.97). In LEL grade I, there were no symptomatic cases due to fat hypertrophy. LEL grade II was symptomatic in only 24 cases (14.5%). In LEL grade III, all cases were symptomatic. A subgroup of 22 patients (42.3%) showed other substantial spinal pathologies (e.g., disk herniation). By means of simple reproducible measurements and indexes MRI grading enables a distinction between mild, moderate, and severe EF hypertrophy. Kappa statistics indicate that LEL can be reliably classified into a four-grade system by experienced observers.     

Free vascularized tibial periosteal graft with monitoring skin island for limb reconstruction: Anatomical study and case report

Several types of vascularized periosteal flaps have recently been described for the treatment or prevention of complex non‐union in pediatric patients. Among them, a vascularized tibial periosteal graft (VTPG), supplied by the anterior tibial vessels (ATV), has been used successfully as a pedicled flap in a few patients. The purpose of the study is to describe the periosteal branches of the ATV, as well as the cutaneous and muscular branches by means of an anatomical study. In addition, to report on the use of VTPG as a free flap with a monitoring skin island in a clinical case. A mean of 6.5 periosteal branches (range 5–7) were found. In all cases we located a cutaneous perforator branching from one of the periosteal branches located at the midlevel of the leg. We performed a two‐stage reconstruction of a recalcitrant non‐union and residual shortening of the right tibia in a 17‐year‐old boy. After nonunion focus distraction, we used a massive bone allograft fixed with a nail and covered by a VTPG as a biological resource. Allograft consolidation was achieved 5.5 months after surgery. At eighteen months after surgery, no complications were observed and the patient had resumed all his daily activities, despite a residual 2‐cm limb‐length discrepancy. VTPG may be considered as a valuable surgical option for bone reconstruction in complex biological scenarios in the young population. © 2015 Wiley Periodicals, Inc. Microsurgery 37:248–251, 2017.     

The burden of chronic rhinosinusitis and its effect on quality of life among patients re-attending an otolaryngology clinic in south western Uganda

Background

Worldwide, the burden of chronic rhinosinusitis (CRS) is variable, but not known in Uganda. CRS has significant negative impact on quality of life (QOL) and as such QOL scores should guide adjustments in treatment strategies. However, most of these studies have been done in the west. Our hypothesis was that QOL scores of the majority of CRS patients in low- to- middle income countries are poorer than those among patients without CRS. The aim of this study was to determine the burden of CRS among patients re-attending the Otolaryngology clinic and whether CRS is related to poor QOL.

Methods

A cross sectional study was conducted at Mbarara Regional Referral Hospital Otolaryngology clinic. One hundred and twenty-six adult re-attendees were consecutively recruited. Data was collected using a structured questionnaire and the Sinonasal Outcome Test 22 (SNOT 22) questionnaire measured QOL.

Results

The proportion of re-attendees with CRS was 39.0% (95% CI 30–48%). Majority of CRS patients had poor quality of life scores compared to non-CRS (88% versus 20% p?<?01). The poor quality of life scores on the SNOT 22 were almost solely as a result of the functional, physical and psychological aspects unique to CRS.

Conclusions

CRS is highly prevalent among re-attendees of an Otolaryngology clinic at a hospital in resource limited settings and has a significant negative impact on the QOL of these patients.

     

Removal of primary tumor improves survival in metastatic breast cancer. Does timing of surgery influence outcomes?

Background

Resection of intact primary tumor is controversial in metastatic breast cancer patients. The aim of this study is to review the impact of surgical resection of primary tumor on overall survival and to assess the role of timing of surgery on survival rates.

Methods

208 patients with metastatic breast cancer diagnosed between 1982 and 2005 in the Hospital Clinico of Valencia (Spain) were analysed. Exclusion criteria were age >80, PS 3–4, Charlson score 3 or follow-up < 90 days. 123 of these underwent surgery and 85 did not. In order to assess the role of timing, the "surgery" cohort was divided into two sub-groups: "before" (n = 78) or "after" (n = 45) diagnosis of disseminated disease.

Results

In the surgery group, patients underwent mastectomy with axillary dissection (82.9%), without axillary dissection (8.9%) and conservative surgery (8.1%). After a median follow-up of 29.68 months, median OS in the "surgery" and the "non-surgery" groups were, 40.4 and 24.3 months. Removal of the primary tumor therefore had a significant positive impact on survival rates (p < 0.001). Benefits of surgery were observed mainly in patients with visceral disease (p = 0.005); no statistical differences were found in those with bone disease (p = 0.79). Univariate analysis for overall survival (OS) identified surgery, performance status, clinical T stage, hormone receptors and number and type of metastases as variables that impacted on survival. In the multivariate test, only resection of primary tumor and estrogen receptors maintained statistical significance, surgery having a protective effect with an HR 0.52 (95% CI 0.35–0.77). No differences in survival were found between the two sub-groups according to the timing of surgery: "before" vs "after"(p = 0.996).

Conclusions

Resection of primary tumor should be considered not only as a palliative or preventive strategy but also as an approach that possibly contributes to the control of the disease in selected patients.     

Genotype–Phenotype Correlations by Ethnicity and Mutation Location in BRCA Mutation Carriers

The genotype–phenotype correlations of the specific BRCA1 and BRCA2 mutations in multi‐ethnic populations in USA have not yet been fully investigated. This study was designed to evaluate the effects of ethnicity at specific mutation locations and breast/ovarian cancer phenotypes. Our cohort included 445 women with different ethnic backgrounds who underwent BRCA genetic testing between 1997 and 2010. Known clinical and pathologic characteristics were compared with Chi‐Square Analysis or Fisher's Exact test as appropriate. The three most common mutation locations in BRCA1 (exons 2, 11, and 20) and BRCA2 (exons 10, 11, and 25) genes were chosen. Prevalence of BRCA1 exon 2 mutations were significantly higher in Ashkenazi Jewish (AJ) women compared to Caucasians (41% versus 15%; p = 0.001). Similarly, AJ women with breast cancer were more likely to have BRCA1 exon 2 mutation (47% positivity in AJ women versus 0–12.5% positivity in other ethnicities; p = 0.004). Women carrying the exon 20 BRCA1 mutation had the highest probability of having combined breast and ovarian cancers compared to women carrying other exon mutations (p = 0.05). The median age at initial cancer diagnosis, phenotypic features of breast cancer tumors, and overall survival did not vary significantly by ethnicity or mutation location. Our data suggest that ethnicity does not affect age of onset, overall survival or confer different risks of breast and ovarian cancer development in BRCA carriers. These results also suggest that women carrying the exon 20 BRCA1 mutation may warrant mutation‐specific counseling and be more aggressively managed for risk reduction.     

Mutation screening of <Emphasis Type="Italic">mitofusin 2</Emphasis> in Charcot-Marie-Tooth disease type 2

Charcot-Marie-Tooth (CMT) disease is among the most common inherited neurological disorders. Mutations in the gene mitofusin 2 (MFN2) cause the axonal subtype CMT2A, which has also been shown to be associated with optic atrophy, clinical signs of first motor neuron involvement, and early onset stroke. Mutations in MFN2 account for up to 20–30% of all axonal CMT type 2 cases. To further investigate the prevalence of MFN2 mutations and to add to the genotypic spectrum, we sequenced all exons of MFN2 in a cohort of 39 CMT2 patients. We identified seven variants, four of which are novel. One previously described change was co-inherited with a PMP22 duplication, which itself causes the demyelinating form CMT1A. Another mutation was a novel in frame deletion, which is a rare occurrence in the genotypic spectrum of MFN2 characterized mainly by missense mutations. Our results confirm a MFN2 mutation rate of ~15–20% in CMT2.