Angioleiomyoma of the inferior turbinate: a rare cause of isolated facial pain.

We present the case of a 33-year-old man with right-sided facial pain. Clinical examination revealed an isolated mass attached to the right inferior turbinate. This was confirmed with computed tomography. Excision was achieved endoscopically and histology revealed an angioleiomyoma. Full symptomatic relief was achieved after surgical excision. Less than 1% of angioleiomyoma lesions are found within the sinonasal cavity. We describe the first documented presentation of angioleiomyoma as a cause of isolated, unilateral facial pain; a very common presentation to the otorhinolaryngology clinic. We promote consideration of angioleiomyoma as a different diagnosis in the presence of facial pain and a unilateral sinonasal lesion. Endoscopic resection provides complete symptomatic resolution.

Immunological profiling of key inflammatory drivers of nasal polyp formation and growth in chronic rhinosinusitis.

BACKGROUND: Chronic rhinosinusitis (CRS) is a chronic inflammatory condition of the upper airways, often associated with the formation of nasal polyps (CRSwNP). It is well established that macroscopically normal (non-polypoidal) sinonasal mucosa in CRSwNP patients can undergo polypoidal change over time, turning into frank polyps. However, little is known about what drives this process. This study aimed to investigate potential drivers of nasal polyp formation or growth through comparison of the immunological profiles of nasal polyps with contiguous non-polypoidal sinonasal mucosa, from the same patients. METHODS: The immune profiles of three types of tissue were compared; nasal polyps and adjacent non-polypoidal sinonasal mucosa from 10 CRSwNP patients, and sinonasal mucosa from 10 control patients undergoing trans-sphenoidal pituitary surgery. Nasal polyp and control samples were also stimulated with Staphylococcus aureus enterotoxin B (SEB) using a nasal explant model, prior to cytokine analysis. Real time quantitative polymerase chain reaction (IL-5, T-bet, IL-17A, FoxP3, TLR-4, IL-8, IL-1beta and IL-6) and Luminex (IFNgamma, IL-5 and IL-17A) were used to quantify pro-inflammatory responses. RESULTS: Nasal polyps and contiguous non-polypoidal sinonasal mucosa from CRSwNP patients displayed a very similar pro-inflammatory profile. When stimulated with SEB, nasal polyps displayed a Th2/Th17 mediated response when compared to controls. CONCLUSIONS: In CRSwNP, nasal polyps and non-polypoidal sinonasal mucosa from the same patient displayed a similar pro-inflammatory profile skewed towards the Th2/Th17 pathway in nasal polyps following SEB stimulation, with evidence of disordered bacterial clearance. These factors may contribute to enhanced survival of bacteria and development of a chronic inflammatory milieu, potentially driving new polyp formation and recurrence following surgical removal.

Nasal obstruction secondary to an inverted midline supernumerary tooth.

We present the case of a 36-year-old man who presented with left-sided nasal obstruction and facial pain. Clinical examination and computed tomography revealed an inverted midline supernumerary tooth buckling and deviating the nasal septum to the left. Full surgical resection of the tooth was achieved through a minimally invasive endoscopic septoplasty with full resolution of symptoms. There is little precedent within the literature to guide our management in this case and therefore we offer a successful surgical treatment strategy.

Rhabdomyosarcoma of the middle ear cavity presenting with Horner's syndrome.

Rhabdomyosarcoma is a rare malignant tumour of striated muscle accounting for 3% of all primary soft tissue tumours of the middle ear cavity. We describe a unique case of a rhabdomyosarcoma of the middle ear cavity in a four-year-old boy, presenting as an otherwise asymptomatic ipsilateral Horner's syndrome. This case involved tumour erosion through the tensor tympani semicanal, accessing the carotid canal and the sympathetic plexus running through it. This child was treated successfully with chemotherapy and proton beam therapy, and remains disease free at six years of follow-up. This case highlights the importance of investigating patients with non-resolving ear infections, polyps or granulation tissue.

Complete nasal obstruction secondary to pacemaker insertion.

We present the case of a 71-year-old man who presented to the ear, nose and throat department with complete nasal obstruction and facial plethora on bending forward. Clinical examination was positive for Pemberton's sign. Computed tomography and ultrasonography demonstrated bilateral brachiocephalic vein thrombosis secondary to pacemaker insertion. This case highlights a novel complication of pacemaker insertion.

BDNF genotype is associated with hippocampal volume in mild traumatic brain injury.

The negative long-term effects of mild traumatic brain injury (mTBI) have been a growing concern in recent years, with accumulating evidence suggesting that mTBI combined with additional vulnerability factors may induce neurodegenerative-type changes in the brain. However, the factors instantiating risk for neurodegenerative disease following mTBI are unknown. This study examined the link between mTBI and brain-derived neurotrophic factor (BDNF) genotype, which has previously been shown to regulate processes involved in neurodegeneration including synaptic plasticity and facilitation of neural survival through its expression. Specifically, we examined nine BDNF single-nucleotide polymorphisms (SNPs; rs908867, rs11030094, rs6265, rs10501087, rs1157659, rs1491850, rs11030107, rs7127507 and rs12273363) previously associated with brain atrophy or memory deficits in mTBI. Participants were 165 white, non-Hispanic Iraq and Afghanistan war veterans between the ages of 19 and 58, 110 of whom had at least one mTBI in their lifetime. Results showed that the BDNF SNP rs1157659 interacted with mTBI to predict hippocampal volume. Furthermore, exploratory analysis of functional resting state data showed that rs1157659 minor allele homozygotes with a history of mTBI had reduced functional connectivity in the default mode network compared to major allele homozygotes and heterozygotes. Apolipoprotein E (APOE) was not a significant predictor of hippocampal volume or functional connectivity. These results suggest that rs1157659 minor allele homozygotes may be at greater risk for neurodegeneration after exposure to mTBI and provide further evidence for a potential role for BDNF in regulating neural processes following mTBI.

Medial Temporal Lobe Contributions to Episodic Future Thinking: Scene Construction or Future Projection?

Previous research has shown that the medial temporal lobes (MTL) are more strongly engaged when individuals think about the future than about the present, leading to the suggestion that future projection drives MTL engagement. However, future thinking tasks often involve scene processing, leaving open the alternative possibility that scene-construction demands, rather than future projection, are responsible for the MTL differences observed in prior work. This study explores this alternative account. Using functional magnetic resonance imaging, we directly contrasted MTL activity in 1) high scene-construction and low scene-construction imagination conditions matched in future thinking demands and 2) future-oriented and present-oriented imagination conditions matched in scene-construction demands. Consistent with the alternative account, the MTL was more active for the high versus low scene-construction condition. By contrast, MTL differences were not observed when comparing the future versus present conditions. Moreover, the magnitude of MTL activation was associated with the extent to which participants imagined a scene but was not associated with the extent to which participants thought about the future. These findings help disambiguate which component processes of imagination specifically involve the MTL.

Comparison between colloid preload and crystalloid co-load in cesarean section under spinal anesthesia: a randomized controlled trial.

BACKGROUND: Hypotension is a common problem during spinal anesthesia for cesarean delivery. Intravenous fluid loading is used to correct preoperative dehydration and reduce the incidence and severity of hypotension. Different fluid regimens have been studied but colloid preload and crystalloid co-load have not been compared. METHODS: In this randomized double-blind study, 210 patients scheduled for elective cesarean section under spinal anesthesia were randomly allocated to receive either 6% hydroxyethyl starch 130/0.4 500 mL before spinal anesthesia (colloid preload) or Ringer's acetate solution 1000 mL administered rapidly starting with intrathecal injection (crystalloid co-load). Maternal hypotension (systolic blood pressure <80% of baseline or <90 mmHg) and severe hypotension (systolic blood pressure <80 mmHg) were treated with 5 and 10mg ephedrine boluses, respectively. The primary outcome was the incidence of hypotension. Secondary outcomes included the incidence of severe hypotension, total ephedrine dose, nausea and vomiting and neonatal outcome assessed by Apgar scores and umbilical artery blood gas analysis. RESULTS: Data analysis was performed on 205 patients; 103 in the colloid preload group and 102 in the crystalloid co-load group. There were no significant differences in the incidence of hypotension (52.4% vs. 42.2%; P=0.18) or severe hypotension (15.5% vs. 9.8%; P=0.31) between colloid preload and crystalloid co-load groups, respectively. The median [range] ephedrine dose was 5 [0-45]mg in the colloid preload group and 0 [0-35]mg in the crystalloid co-load group (P=0.065). There were no significant differences in maternal nausea or vomiting or neonatal outcomes between groups. CONCLUSION: The use of 1000 mL crystalloid co-load has similar effect to 500 mL colloid preload in reducing the incidence of hypotension after spinal anesthesia for elective cesarean delivery. Neither technique can totally prevent hypotension and should be combined with vasopressor use.

Maxillary dentinogenic ghost cell tumour.

Dentinogenic ghost cell tumours are extremely rare, and pose significant diagnostic and therapeutic challenges as this case clearly demonstrates. An awareness of different clinical presentations and distinct histopathological features is important in establishing an early definitive diagnosis and instituting appropriate management. Furthermore, there is little precedent in the literature to guide management in such a case, and we therefore consider this report to be noteworthy and instructive in this respect.

Solitary extra-skeletal sinonasal metastasis from a primary skeletal Ewing's sarcoma.

INTRODUCTION: Ewing's sarcoma is a rare, malignant tumour predominantly affecting young adolescent males. We describe a unique case of an isolated extra-skeletal metastasis from a skeletal Ewing's sarcoma primary, arising in the right sinonasal cavity of a young man who presented with severe epistaxis and periorbital cellulitis. RESULTS: Histologically, the lesion comprised closely packed, slightly diffuse, atypical cells with round, hyperchromatic nuclei, scant cytoplasm and occasional mitotic figures, arranged in a sheet-like pattern. Immunohistochemical analysis showed positive staining only for cluster of differentiation 99 glycoprotein. Fluorescent in situ hybridisation identified the Ewing's sarcoma gene, confirming the diagnosis. MANAGEMENT: Complete surgical resection was achieved via a minimally invasive endoscopic transnasal approach; post-operative radiotherapy. Ten months post-operatively, there were no endoscopic or radiological signs of disease. CONCLUSION: Metastatic Ewing's sarcoma within the head and neck is incredibly rare and can pose significant diagnostic and therapeutic challenges. An awareness of different clinical presentations and distinct histopathological features is important to enable early diagnosis. This case illustrates one potential management strategy, and reinforces the evolving role of endoscopic transnasal approaches in managing sinonasal cavity and anterior skull base tumours.

A randomized controlled trial of resistance exercise in individuals with ALS.

OBJECTIVE: To determine the effects of resistance exercise on function, fatigue, and quality of life in individuals with ALS. METHODS: Subjects with a diagnosis of clinically definite, probable, or laboratory-supported ALS, forced vital capacity (FVC) of 90% predicted or greater, and an ALS Functional Rating Scale (ALSFRS) score of 30 or greater were randomly assigned to a resistance exercise group that received a home exercise program consisting of daily stretching and resistance exercises three times weekly or to a usual care group, who performed only the daily stretching exercises. ALSFRS, the Fatigue Severity Scale (FSS), and Short Form-36 (SF-36) were completed at baseline and monthly for 6 months. FVC and maximum voluntary isometric contraction (MVIC) were monitored monthly throughout the study. RESULTS: Of 33 subjects screened, 27 were randomly assigned (resistance = 13; usual care = 14). Eight resistance exercise subjects and 10 usual care subjects completed the trial. At 6 months, the resistance exercise group had significantly higher ALSFRS and SF-36 physical function subscale scores. No adverse events related to the intervention occurred, MVIC and FVC indicated no negative effects, and less decline in leg strength measured by MVIC was found in the resistance exercise group. CONCLUSION: Our study, although small, showed that the resistance exercise group had significantly better function, as measured by total ALS Functional Rating Scale and upper and lower extremity subscale scores, and quality of life without adverse effects as compared with subjects receiving usual care.

Group 1 CD1 genes in rabbit.

CD1 is an Ag-presenting molecule that can present lipids and glycolipids to T cells. The CD1 genes were first identified in the human, and since then, homologs have been identified in every mammalian species examined to date. Over a decade ago, CD1B and CD1D homologs were identified in the rabbit. We have extended this earlier study by identifying additional CD1 genes with the goal of developing the rabbit as an animal model to study the function of CD1 proteins. We constructed a thymocyte cDNA library and screened the library with CD1-specific probes. Based on nucleotide sequence analyses of the CD1(+) cDNA clones obtained from the library, we have identified two CD1A genes and one CD1E gene as well as determined the complete sequence of the previously identified CD1B gene. The CD1E(+) cDNA clones lacked the transmembrane and cytoplasmic domains and, if translated, would encode for a soluble or secreted CD1E protein. In addition, expression studies demonstrated that the CD1 genes were expressed in peripheral lymphoid tissues as well as in skin, gut, and lung. Of interest is the finding that CD1A2, CD1B, and CD1E genes were found to be expressed by rabbit B cell populations. The rabbit, with a complex CD1 locus composed of at least two CD1A genes, one CD1B gene, one CD1D gene, and one CD1E gene, is an excellent candidate as an animal model to study CD1 proteins.

Lichen planus--report of successful treatment with aloe vera.

Lichen planus is a disease that involves the skin and mucous membranes. It is characterized by unique eruptions. The cause of this disease is unknown, but has been linked to emotional stress, and has also been attributed to viral infections. A case is described of a successful treatment of lichen planus.

Maternity care practices of navy family practice residency graduates after leaving the military.

BACKGROUND: Nationwide, 32% of residency-trained family physicians deliver babies compared with 73% to 90% in the military. This study describes and defines issues that could ultimately help revive family practice maternity care. METHOD: We surveyed 112 family physicians who had left the navy. RESULTS: Ninety-one percent had delivered babies in the navy, 45% since leaving the military, and 25% currently. Principal maternity care incentives both in and out of the military were personal and professional satisfaction. Reasons for not providing civilian maternity care included malpractice risks, insurance costs, and lifestyle issues. The decision for providing maternity care was usually made before or during residency, whereas the decision against was most often made upon leaving the military. Among more recent graduates (1990-1995), 48% continued to deliver babies in civilian practice. (This is about 20% more than recent civilian graduates.) Malpractice concerns were less important to this group than to earlier graduates. CONCLUSIONS: Factors discouraging family physicians from providing maternity care arise from their practice environment and are not easily overcome with improved training and experience. Recent navy residency graduates are not as easily discouraged.

The maternity care practice of Navy family practice residency graduates while on active duty.

BACKGROUND AND OBJECTIVES: Military family practice residency programs produce a high percentage of graduates who provide maternity care. This study will define the scope of maternity care practice for one military family practice residency program's graduates while they were serving on active duty in the U.S. Navy. METHODS: Two hundred eight surviving graduates of the family practice residency at Naval Hospital, Jacksonville, Florida, from 1971 to 1995 were surveyed by mail regarding their maternity care practice while on active duty. One hundred eighty-one (87%) responded to the survey, and the data were analyzed with descriptive statistics. FINDINGS: The vast majority of these Navy family practice residency graduates provided prenatal care (88.4%) and routine vaginal delivery services (85.1%) while on active duty. The majority repaired third- and fourth-degree perineal lacerations and performed vacuum- or forceps-assisted vaginal delivery. Additionally, a significant minority provided more advanced maternity care services such as dilation and curettage, tubal ligation, and cesarean section. The overwhelming majority (97%) of these graduates felt that their residency education had adequately prepared them to provide these maternity care services while on active duty. CONCLUSIONS: During the past 25 years, Navy residency-trained family physicians provided a wide range of maternity care services while on active duty and felt that their Navy residency training program had prepared them well to meet this responsibility.

Role of IL-7 in the shaping of the pulmonary gamma delta T cell repertoire.

Gamma delta T cells bearing the canonical fetal-type V gamma 6/V delta 1 rearrangements are the predominant gamma delta T cells in the lungs of adult mice. In contrast, these V gamma 6/V delta 1 T cells are virtually absent in the pulmonary epithelia of nude mice. The intraepithelial dominance of gamma delta T cells that express this particular TCR is thought to result from a preferred thymic pathway of gene rearrangement and not from TCR-mediated positive selection. We now show that gamma delta T cell precursors in the lung epithelium of both euthymic and athymic neonatal mice generate this rearrangement in situ. In athymic mice, these clonotypes do not survive, but can be rescued in vitro and in vivo by the lymphokine IL-7.

Localization of prothymocytes from wild-type and viable motheaten mice following intravenous injection into irradiated adoptive recipients.

Bone marrow cells from viable motheaten (me(v)) mutant mice fail to repopulate the thymus of irradiated recipients upon i.v. adoptive transfer. This defect can be corrected by the addition of wild-type marrow to the me(v)/me(v) marrow prior to i.v. injection. The present study focused on the nature of this apparent defect in me(v)/me(v) prothymocyte activity. We established an adoptive transfer assay system that facilitated the investigation of the migration patterns of wild-type and me(v)/me(v) marrow prothymocytes in adoptive recipients. Wild-type prothymocytes were found to seed the thymus of recipients as early as 1 day after i.v. injection. This early wave of prothymocyte seeding peaked at 4 days and diminished to undetectable levels at 6 days postinjection. After an interval of no detectable wild-type precursor activity in the thymus of primary recipients, a second wave of activity was observed on Day 15. This second wave resulted in permanent engraftment and persisted throughout the observation period. In contrast, only one initial wave of me(v)/me(v) prothymocyte seeding occurred in the recipient thymus. Detection of mev/mev precursors in the recipient's thymus was dependent upon the coinjection of wild-type marrow cells. These results suggest that me(v)/me(v) prothymocytes initially seed to the thymus of adoptive recipients, but fail to proliferate and/or differentiate due to a defect in marrow-derived intrathymic accessory cell activity.

Evidence for elevated prothymocyte activity in the bone marrow of New Zealand Black (NZB) mice. Elevated prothymocyte activity in NZB mice.

New Zealand Black (NZB) mice spontaneously develop an autoimmune syndrome similar to that of systemic lupus erythematosus. Numerous abnormalities in T lymphocyte development have been reported in NZB mice, and the autoimmune syndrome can be adoptively transferred to naive recipients using bone marrow cells. In the present study, we have used an adoptive transfer system to study quantitatively the relative prothymocyte activity of marrow in either young (4-6 weeks of age) or older (8-10 months of age) NZB mice. Our results demonstrate that NZB marrow has approximately 7-fold more prothymocyte activity than that of marrow in SEA mice, a histocompatible, non-autoimmune prone normal strain of mice. This was ascertained by a competitive repopulation assay, in which mixtures of NZB and SEA prothymocytes were compared directly for their ability to repopulate the thymus of adoptive recipients. This increase in prothymocyte activity in the primary recipient of NZB marrow was associated with an increased competitive advantage of NZB marrow prothymocytes over that of SEA marrow prothymocytes in repopulating the hemopoietic (bone marrow) compartment of the primary host. These findings suggest that elevated prothymocyte activity in NZB mice, along with our previously presented evidence for abnormalities of thymocytopoiesis in NZB mice, may be important in their predisposition for autoimmunity.

Thymic involution in viable motheaten (me(v)) mice is associated with a loss of intrathymic precursor activity.

Mice homozygous for the viable motheaten (me(v)) allele manifest abnormalities in thymocytopoiesis, are severely immunodeficient, and develop autoimmune disorders early in life. Premature thymic involution occurs in me(v)/me(v) mice, and their bone marrow prothymocytes are unable to repopulate the thymus of adoptive recipients following intravenous (i.v.) transfer. However, analysis of thymocytopoiesis following intrathymic (i.t.) adoptive transfer of bone marrow from me(v)/me(v) mice demonstrates the presence of normal numbers of prothymocytes. To investigate intrathymic development in me(v)/me(v) mice, we determined intrathymic precursor cell number and activity. Dual labeling analyses showed that an involuted me(v)/me(v) thymus is relatively enriched (fivefold) in CD4-CD8- thymocytes (intrathymic precursor phenotype) compared with wild-type (+/+) thymus. However, thymocytes from me(v)/me(v) mice were deficient in precursor activity when adoptively transferred i.t. into irradiated recipients. Thymocytes recovered from the involuted thymus of aged or steroid-treated normal mice also displayed reduced precursor activity. However, the phenotypic profile of thymocyte subsets from steroid-treated mice was enriched in single positive cells (mature phenotype) and was distinctly different from the subset distribution of thymocytes in me(v)/me(v) and aged mice. These results suggest that intrathymic precursor activity in me(v)/me(v) mice is decreased, and may be reflective of decreased prothymocyte seeding to the thymus in vivo. In addition, the results suggest that the thymic involution in me(v)/me(v) mice is not due solely to effects of corticosteroids.