Atypical carcinoid arising from the mediastinal tissue is a rare neuroendocrine tumor and an association with parathyroid adenoma is very unusual. We report an unusual case of atypical carcinoid of mediastinum...
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Spontaneous hematoma within the iliopsoas muscle (SIH) is a rare complication most commonly seen in coagulopathic patients. Often, patients undergoing microvascular free tissue transfer are anticoagulated for anastomotic patency. Here we describe two cases of postoperative SIH following contralateral anterolateral thigh (ALT) free tissue transfer for reconstruction of oncologic head and neck defects. Both patients described hip pain after mobilization and had a corresponding acute blood loss anemia. Diagnosis of SIH was confirmed by CT and both patients were managed conservatively. Given that anticoagulation is a common practice following head and neck free tissue transfer, surgeons should be aware of this potential complication.
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To the Editor: Merkel cell carcinoma (MCC) represents a rare, aggressive cutaneous neuroendocrine carcinoma. The majority of MCCs are positive for genomic insertions of Merkel cell polyomavirus (MCPyV). Favorable outcomes have been reported for MCPyV+ versus MCPyV− MCCs1 and a high level of tumor-infiltrating lymphocytes.2-4 In addition, higher numbers of natural killer (NK) cells have been identified in MCPyV+ versus MCPyV−,3 but the prognostic significance of this remains to be determined.
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Lamotrigine (LTG) was first synthesized in the early 1980s and approved by the US Food and Drug Administration in 1994. Since then, it has been used worldwide as an effective anticonvulsant and as a mood stabilizer. However, LTG can cause adverse reactions including cutaneous adverse drug reactions with approximately 10% probability.1 Severe cutaneous adverse reactions (SCARs) are the most serious form and include Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and drug rash with eosinophilia and systemic symptoms (DRESS).
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Primary bronchopulmonary mucoepidermoid carcinoma (BPMEC) is a rare tumor. The fusion protein MECT1-MAML2 has been implicated as a causative genetic event in salivary and BPMECs. Several studies have shown the impact of MECT1-MAML2 on the diagnosis and prognosis of salivary gland mucoepidermoid carcinoma; however, few studies have been published regarding MECT1-MAML2 in the context of primary BPMEC. We describe the clinicopathologic, genetic, and outcome data of 16 patients with BPMEC. Clinicopathologic features were recorded from the electronic medical records. All tumors were reviewed by two expert pulmonary pathologists and graded according to previously established criteria. The presence of MECT1-MAML2 was evaluated with reverse transcription polymerase chain reaction using RNA extracted from formalin-fixed paraffin-embedded tumor tissue. Patients included 9 women and 7 men with a median age of 50 years (range, 7 to 82 years). Tumors exhibited low (n = 14, 88%), and high (n = 2, 12%) grade histologic features. Eight of nine tested tumors (89%) were positive for MECT1-MAML2. The median follow-up time was 40.8 months (range, 1.8–120). Median overall survival for patients with high-grade tumors was 12 months, which was significantly (p = 0.002) shorter than that for patients with low-grade tumors (survival undefined). We also provide a comprehensive review of literature of cases of primary bronchopulmonary mucoepidermoid carcinoma and summarize our findings in this context. MECT1-MAML2 fusion transcript is a driver genetic event in the pathogenesis of primary BPMEC. Histologic grade continues to play a pivotal role in the survival of patients with primary bronchopulmonary mucoepidermoid carcinoma.
Publication date: Available online 25 March 2017
Source:Brazilian Journal of Otorhinolaryngology
Author(s): Luiz Felipe Palma, Fernanda Aurora Stabile Gonnelli, Marcelo Marcucci, Adelmo José Giordani, Rodrigo Souza Dias, Roberto Araújo Segreto, Helena Regina Comodo Segreto
IntroductionThe procedure used to evaluate salivary flow rate is called sialometry. It can be performed through several techniques, but none appears to be really efficient for post-radiotherapy patients.ObjectiveTo adequate sialometry tests for head and neck cancer patients submitted to radiotherapy.Methods22 xerostomic patients post-radiotherapy (total radiation dose ranging from 60 to 70Gy) were included in this study. Ten patients were evaluated using sialometries originally proposed by the Radiation Therapy Oncology Group and twelve were assessed by our modified methods. Unstimulated and stimulated sialometries were performed and the results were classified according a grading scale and compared between both groups.ResultsThere was no statistically significant difference between the salivary evaluations of both groups (p=0.4487 and p=0.5615). Also, most of these rates were classified as very low and low.ConclusionThis novel method seems to be suitable for patients submitted to radiotherapy.
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Publication date: Available online 25 March 2017
Source:Journal of Allergy and Clinical Immunology
Author(s): Lisbeth Marianne Thøstesen, Lone Graff Stensballe, Gitte Thybo Pihl, Jesper Kjærgaard, Nina Marie Birk, Thomas Nørrelykke Nissen, Aksel Karl Georg Jensen, Peter Aaby, Annette Wind Olesen, Dorthe Lisbeth Jeppesen, Christine Stabell Benn, Poul-Erik Kofoed
BackgroundRecurrent wheeze is frequent in childhood. Studies have suggested that Bacillus Calmette-Guérin (BCG) vaccination may have non-specific effects, reducing general non-tuberculosis morbidity, including respiratory infections and atopic diseases. The mechanisms behind these non-specific effects of BCG are not fully understood, but a shift from Th2 to Th1-response has been suggested as a possible explanation.ObjectiveWe hypothesized that BCG at birth would reduce the cumulative incidence of recurrent wheeze during the first year of life.MethodsThe Danish Calmette Study is a multicenter randomized trial conducted from 2012-2015 at three Danish hospitals. The 4262 newborns of 4184 included mothers were randomized 1:1 to BCG (SSI strain 1331) or to a no-intervention control group within 7 days of birth; siblings were randomized together as one randomization unit. Exclusion criteria were gestational age <32 weeks, birth weight <1000g, known immunodeficiency, or no Danish-speaking parent. Information was collected through telephone interviews and clinical examinations at 3 and 13 months of age; the data collectors were blinded to randomization group.Recurrent wheeze was defined in several ways, the main definition being: "physician-diagnosed and medically treated recurrent wheeze up to 13 months of age".ResultsBy 13 months 211/2100 (10.0%) children in the BCG group and 195/2071 (9.4%) children in the control group had been diagnosed by a medical doctor with recurrent wheeze and received anti-asthmatic treatment (relative risk 1.07 (95% confidence intervals 0.89-1.28)). Supplementary analyses were made, including an analysis of baseline risk factors for developing RW.ConclusionNeonatal BCG had no effect on the development of recurrent wheeze before 13 months of age.
Publication date: Available online 25 March 2017
Source:Journal of Allergy and Clinical Immunology
Author(s): Ann-Marie M. Schoos, Bo L. Chawes, Erik Melén, Anna Bergström, Inger Kull, Magnus Wickman, Klaus Bønnelykke, Hans Bisgaard, Morten A. Rasmussen
BackgroundAssessment of sensitization at an isolated time-point during childhood provides limited clinical information. We hypothesized that sensitization develops as specific patterns with respect to age at debut, development over time, and involved allergens, and that such patterns might be more biologically and clinically relevant.ObjectiveTo explore latent patterns of sensitization during the first 6 years of life and investigate whether such patterns associate to development of asthma, rhinitis, and eczema.MethodsWe investigated 398 children from the at-risk Copenhagen Prospective Studies on Asthma in Childhood 2000 birth cohort (COPSAC2000) with specific-IgE against 13 common food and inhalant allergens at ages ½, 1½, 4, and 6yrs. An unsupervised cluster analysis for three-dimensional data (NNS-PARAFAC) was used to extract latent patterns explicitly characterizing temporal development of sensitization, while clustering allergens and children. Subsequently, these patterns were investigated in relation to asthma, rhinitis, and eczema. Verification was sought in an independent unselected birth cohort, BAMSE, constituting 3051 children with specific-IgE against the same allergens at 4 and 8yrs.ResultsThe NNS-PARAFAC analysis indicated a complex latent structure involving seven age- and allergen-specific patterns in the COPSAC2000 data: (1) dog/cat/horse; (2) timothy grass/birch; (3) molds; (4) house dust mites; (5) peanut/wheat flour/mugwort; (6) peanut/soybean; and (7) egg/milk/wheat flour. Asthma was solely associated with pattern 1 (OR=3.3 [1.5-7.2]), rhinitis with patterns 1-4 and 6 (ORs=2.2–4.3) and eczema with patterns 1-3 and 5-7 (ORs=1.6–2.5). All seven patterns were verified in the independent BAMSE cohort (R2>0.89).ConclusionThis study suggests the presence of specific sensitization patterns in early childhood differentially associated to development of clinical outcomes. Using such patterns in future research might provide more robust and clinically relevant results.
Publication date: April 2017
Source:Journal of Cranio-Maxillofacial Surgery, Volume 45, Issue 4
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Publication date: April 2017
Source:Journal of Cranio-Maxillofacial Surgery, Volume 45, Issue 4
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Publication date: April 2017
Source:Journal of Cranio-Maxillofacial Surgery, Volume 45, Issue 4
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Recent studies suggest that cyclooxygenase 2 (COX-2) inhibitors may enhance the toxic effects of anticancer drugs on tumor cells, including oral squamous cell carcinoma(OSCC), but its long-term use can cause side effects such as stomach ulcers and myocardial infarction. Our aim was to investigate proliferative effects of a downstream product of COX-2, prostaglandin E2(PGE2), in human oral squamous carcinoma cell line Tca8113 and explore the effects of PGE2 receptors, especially EP4 receptor, on the growth of Tca8113 cells.
To evaluate the effects of PGE2 and EP receptors on Tca8113 cells, CCK8 assay, Western blotting, cell cycle analysis, and apoptosis assay were performed.
We found that the EP4 receptor agonist, PGE1-OH, could mimick PGE2 rescued the inhibitory effect of celecoxib and induced cell growth via ERK phosphorylation, and the EP4 receptor antagonist, L-161,982, completely blocked PGE2-stimulated ERK phosphorylation and proliferation of Tca8113 cells. Furthermore, L-161,982 may induce apoptosis and block cell cycle progression at s phase by upregulating Bax and p21 protein levels and by downregulating Bcl-2, CDK2, and cyclinA2 protein levels.
Our results indicate that EP4 receptor mediates PGE2 induced cell proliferation through ERK signaling, and inhibition of EP4 receptor may represent an alternative therapeutic strategy for the prevention and treatment of OSCC.
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Platelet derived growth factors alpha (PDGFA) is a tyrosine kinase receptor activator which known to be amplified in the malignancies and their expression levels are correlated to tumor progression and reduced overall survival. The expression of PDGFRA is different among the tumors and normal tissues, furthermore; their expression level is site-specific. Under a physiological condition, PDGFRA and its ligand are expressed in distinct cell populations and activated in a paracrine manner. Nevertheless, heterodimer characteristic of PDGFRA allows it to be trans-activated by non-specific ligands or via autocrine manner. The future of cancer therapy can be based on PDGFRA receptor blockade and therefore warrants further investigation to determine the differing expression of PDGFRA between controls and Oral Squamous Cell Carcinoma (OSCC) patients.
We performed a case-control study of 111 patients with newly diagnosed tongue squamous cell carcinoma and 111 control subjects without a cancer diagnosis, matched for age and gender, to evaluate the association between PDGFRA expression level in oral mucosal. We then performed smoking stratification in each cohort. Independent t-test analysis was applied for case-control comparisons.
Mean value of PDGFRA mRNA level (−ΔCt) for normal cohort is -30.242, whereas mean value of PDGFRA mRNA level for OSCC patients is -11.516. PDGFRA mRNA level (−ΔCt) was significantly higher in oral cancer cohort, p<0.001. Smokers have a significantly higher PDGFRA mRNA expression in comparison to non-smokers (p=0.002) among the non-cancer group. Likewise, this trend is observed in cancer cohort too, p=0.044.
PDGFRA expression is significantly higher in oral cancer cohort with or without the establishment of tobacco risk factor.
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Publication date: Available online 25 March 2017
Source:International Journal of Oral and Maxillofacial Surgery
Author(s): P. Suton, I. Salaric, M. Granic, D. Mueller, I. Luksic
The presence of extracapsular spread (ECS) in patients with oral squamous cell carcinoma (OSCC) indicates a poor prognosis and is associated with a higher risk of regional recurrence and distant metastasis. The aim of this study was to analyse this important feature of cervical lymph nodes in the clinically node-negative setting. The study included 61 patients with clinically T1–T3N0 OSCC who underwent primary surgical treatment; 52 were male and nine were female, and their median age was 57 years. The 5-year disease-free survival, disease-specific survival, and overall survival rates were 30.6%, 28.3%, and 14.3%, respectively, in the ECS group compared to 61.9%, 61.9%, and 48.2%, respectively, in the pN+/ECS-negative group and 76.7%, 81.9%, and 47.0%, respectively, in the pN0 group. The differences between the survival curves were highly significant (P=0.023, P=0.003, and P=0.029, respectively). The incidence of local (50% vs. 14.9%, P=0.011) and regional (28.6% vs. 2.1%, P=0.008) recurrence was significantly greater in the ECS group compared to the other subgroups of patients. Furthermore, the time to recurrence was significantly shorter in the ECS subjects. The presence of ECS in patients with oral cancer indicates a poor prognosis. ECS is a frequent feature in clinically node-negative settings and may be more common in smaller lymph nodes than is generally appreciated.
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The term dermatoporosis has been proposed to describe clinical signs and functional consequences of age-related extreme skin fragility.
To create a simple dermatoporosis self-diagnosis tool (IDA: Index Dermatoporosis Assessment) and to use this tool to estimate the prevalence of dermatoporosis in France.
A specific dermatoporosis questionnaire was developed with the help of senior dermatologists and survey experts. This questionnaire was submitted to consecutive individuals aged ≥65 years who consulted a dermatologist. At the end of the consultation, the dermatologist was asked to assess "whether or not"dermatoporosis was present. In a second step, the final questionnaire was mailed to a representative sample of the French population aged ≥65 years in order to estimate the prevalence of dermatoporosis.
The initial questionnaire, consisting of two modules (24 questions), was validated in 173 individuals aged ≥65 years) during a dermatologist consultation. Dermatologists diagnosed 46% of the individuals with dermatoporosis. The final validated questionnaire consisted of 14 items, 12 consisting in presence or absence of clinical signs and 2 items consisting of the self-assessment by individuals of skin aging on neckline and hands (none/moderate/significant/very significant). A scoring system was generated to quote quantitatively dermatoporosis (from 0 if no sign of dermatoporosis to 20 maximal dermatoporosis). The area under the receiver operator curve was 0.8535, indicating a very good ability of the questionnaire to differentiate between individuals. A cut-off value of 11 was linked to positive and negative predictive values of 0.78 and 0.81, respectively. In a second step, using the questionnaire in a representative sample of the French population (n=533), the estimated overall prevalence of dermatoporosis was 37.5% in French subjects aged ≥65 years (27.5% [males] vs. 43.9% [females]; P<0.05). The estimated prevalence of dermatoporosis was twice higher in subjects with eczema or atopic dermatitis during childhood than in the population without dermatoporosis (60.6% vs. 33.4%, P<0.001). Individuals with dermatoporosis also reported a higher prevalence of itching, long term corticosteroid use, anticoagulant use, and prior sun exposure.
Using a new simple dermatoporosis self-diagnosis tool, this study provides a previously unprecedented insight into the high prevalence of dermatoporosis in elderly individuals. IDA questionnaire is a short (14-item) and easy to use tool for evaluating dermatoporosis in adults and may allow an easy evaluation of each subject.
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Venous leg ulcers (VLU) are the latest manifestation of chronic venous insufficiency (CVI) and the leading cause of chronic leg ulcers. Compression therapy is considered the main treatment. Unfortunately, they require long-term treatment to cure and have a high rate of recurrence (up to 78%). 1,2
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Disorders of skin or its appendages may be the tell-tale signs of an underlying disease. Over the last one year we had a series of patients attending the dermatology outpatient services at our institute for bizarre nail changes. Evaluation of these patients led us to diagnose chronic selenium toxicity.
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Several autosomal dominant disorders may manifest in mosaic patterns with cutaneous involvement. Genomic mosaicism results from postzygotic autosomal mutations, giving rise to clonal proliferation of two genetically distinct cell groups, which clinically present as lesions following the lines of Blaschko.
To increase the awareness of the clinical variability of mosaic manifestations in autosomal dominant skin disorders in order to avoid delayed diagnosis.
Clinicopathologic correlation in a case series including three patients with mosaic manifestations of different autosomal dominant skin diseases.
Here we describe a patient with type 1 segmental mosaicism of epidermolytic ichthyosis (case 1) and two patients with either type 1 (case 2) or type 2 (case 3) segmental neurofibromatosis 1 (NF1).
Dermatologists should be familiar with mosaic manifestations of autosomal dominant skin diseases to ensure appropriate guidance of the affected patient. Genetic counselling is mandatory as even limited forms of mosaicism may involve the patient's germline with a moderately increased risk to transmit the mutation to their offspring, resulting in a more severe, generalized form of the respective disease.
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A young man presented to accident and emergency with a short history of diarrhoea and vomiting, with no medical history. He deteriorated rapidly during triage and never regained consciousness. He was pronounced dead after hours of attempted resuscitation. He was found to have organisms suggestive of diplococci on his blood film and subsequently had MenY found via PCR testing. This case illustrates a highly unusual presentation of invasive meningococcal disease caused by MenY which is quietly and dangerously increasing in incidence in the UK, particularly in young healthy patients. All clinicians are reminded to be vigilant in order to diagnose and treat this often fatal disease as well as to promote uptake of the quadrivalent MenACWY vaccine.
Immunocheckpoint inhibitors targeting the programmed cell death-1 (PD-1) and PD-1 ligand 1 (PD-L1) axis have shown promising results in patients with non-small-cell lung cancer (NSCLC). Recent research has shown that epidermal growth factor receptor (EGFR) signaling affects PD-L1 expression in NSCLC cells; however, the mechanism regulating PD-L1 expression in tumor cells remains unclear. Using immunohistochemistry, we evaluated the impact of expression of PD-L1 and EGF family receptors EGFR and human epidermal growth factor receptor 2 (HER2) in tumor cells from 91 patients with pathological Stage IA–IIIA NSCLC. Overexpression of PD-L1 was observed in 14% of the resected tumors, and associated with poor recurrence-free survival (p = 0.021) and overall survival (p = 0.033). PD-L1 expression is positively correlated with EGFR expression and inversely correlated with HER2. NSCLC cell lines were treated in vitro with the EGFR ligand EGF with or without inhibition of EGFR or HER2, after which PD-L1 expression was evaluated using flow cytometry. Consistent with previous reports, PD-L1 expression was clearly enhanced by EGF. EGFR-tyrosine kinase inhibitors or EGFR small interfering RNA (siRNA) blocked EGF-induced PD-L1 overexpression in NSCLC cell lines, but HER2 siRNA did not. Moreover, our findings suggest that PD-L1 expression could be partially regulated via the PI3K/AKT and JAK/STAT pathways. We conclude that PD-L1 overexpression is associated with poor prognosis and is positively correlated with EGFR expression but inversely correlated with HER2 expression in NSCLC. We also showed that EGFR and HER2 have different effects on EGF-induced PD-L1 expression in NSCLC cell lines.
CD38, an adenine dinucleotide phosphate (ADP) ribose cyclase and a cyclic ADP ribose hydrolase, is widely expressed on the surface of multiple myeloma (MM) cells. It is known to play a pivotal role in the downstream pathways that mediate MM cell growth, signal transduction, and adhesion. The clinical use of CD38 monoclonal antibodies (MoAbs), such as daratumumab, either as monotherapy or in combination with other anti-MM agents, has produced impressive results in patients who have failed standard MM therapy. CD38 MoAbs exhibit several cytotoxic mechanisms on MM cells. In addition to the classical effector mechanisms associated with antibody therapy, CD38 MoAbs induce MM apoptosis and clonal T-cell expansion. Here, we summarize the results of some pivotal clinical studies using a human CD38 MoAb, daratumumab, in patients with MM, discuss the anti-MM effector mechanisms induced by CD38 MoAbs, and review the potential tumor antigens that may be suitable targets for immunotherapy of MM. Finally, we present a paradigm of immunotherapy for MM patients using CD38 MoAbs followed by GM-CSF and an immune checkpoint inhibitor in patients who have undergone high dose chemotherapy and autologous stem cell transplant. CD38 MoAbs have emerged as a novel and ultimately very promising immunotherapeutic agent for MM because of its ability to induce MM cytotoxicity through both arms of the adaptive immune responses.
Description
A patient in their 30s presented with a 3-day history of lower back pain, lower limb weakness and new onset of urinary incontinence. The patient had a history of metastatic melanoma, including to brain, for which they had previously been treated with adjuvant chemotherapy and radiotherapy. Clinical exam revealed a palpable bladder at the umbilicus and reduced power in the lower limbs bilaterally.
An MRI whole spine with gadolinium contrast agent revealed multiple enhancing lesions at the T5/6, T9 and T12–L3 levels, pronounced spinal cord oedema and a markedly distended bladder (figures 1 and 2). The location of these lesions within the spinal canal was mixed, including intramedullary metastases (ie, within the substance of the spinal cord) and intradural extramedullary metastases (ie, in the subarachnoid space but extrinsic to the cord). Lesions obliterated the cauda equina resulting in a cauda equina syndrome.
...
Acute aortic dissection is associated with significant morbidity and mortality, often from complications including aortic regurgitation, cardiac tamponade and myocardial infarction. Typical clinical presentation includes a sudden onset of severe chest pain, although this is not always consistent. Clinical signs and symptoms are diverse with an estimated 38% of cases being missed on initial evaluation. Primary neurological symptoms at presentation are rare but have been reported often to coexist with chest pain. We present a case of acute aortic dissection in which the initial presenting symptoms were predominantly neurological. Stanford type A dissection is a surgical emergency with a high burden of cardiovascular death; thus, aggressive identification and management is paramount. Our case re-emphasises the importance of having a higher index of suspicion and a keen clinical eye for atypical presentations of acute aortic dissection.
Background:
Tuberculosis is a major health problem worldwide. Gastrointestinal tuberculosis presenting as isolated involvement of the duodenum is a rare case.
Case Presentation:A 13 year male, presented with features of gastric outlet obstruction. CT enterography scan showed circumferential mural thickening in first and second part of duodenal junction causing luminal narrowing. Upper GI endoscopy confirmed the narrowing of D1-D2 junction. Duodenal biopsy showed duodenitis with negative result for AFB stain, Helicobacter Pylori. Patient underwent roux-en-y gastro-jejunostomy. Histo-pathological findings were consistent with tuberculosis. Patient was started on AKT and discharged. At 3 months follow up; patient asymptomatic.
Conclusion:The unusual location of gastrointestinal tuberculosis, lack of specific signs and symptoms, radiological studies and endoscopy findings makes diagnosis a challenge. The treatment of duodenal tuberculosis is still medical and surgery should be reserved for emergency like gastric outlet obstruction causing nutritional compromise.
We present a case of a 59-year-old man with left upper alveolar numbness of 2 years' duration in the absence of sinonasal symptoms. On physical examination, he demonstrated mild left facial asymmetry and diminished sensation of his left upper alveolus from the left second upper incisor to first canine. CT imaging revealed chronic sinusitis changes of the left maxillary sinus, with reduced volume and depressed anterior wall. The patient underwent functional endoscopic sinus surgery to re-establish maxillary sinus ventilation. He was noted to have some improvement of his upper alveolar paraesthesia postoperatively. Silent sinus syndrome is part of the spectrum of chronic maxillary atelectasis. In the presented case, chronic osteitic bony sclerosis, as opposed to osteopenic change of the maxillary sinus, was seen. We postulate that bony encasement of the anterior superior alveolar nerve resulted in chronic nerve compression and the patient's unusual symptom of upper alveolar paraesthesia.
We report a rare complication in an immunosuppressed patient with IgA nephropathy who suffered from severe acute respiratory distress syndrome, severe capillary leakage and shock after placement of a double lumen central venous catheter. He could be successfully treated by extracorporeal membrane oxygenation (ECMO) and therapeutic plasma exchange. This report highlights the severity of late-onset complications of catheter placements and shows the potential of ECMO treatment for the management of acute illnesses with bridge to recovery.
De Garengeot's hernia is defined as a femoral hernia that contains the appendix. Owing to the extreme rarity of de Garengeot's hernia, it could represent a diagnostic challenge; however, it should remain in the differential diagnosis, particularly in this patient demographic. A female patient aged 96 years, who presented as an emergency with acute right iliac fossa pain, was found to have de Garengeot's hernia. The diagnosis was made intraoperatively, where she was treated with an appendicectomy and repair of the hernia defect. The incidence of de Garengeot's hernia is 0.5–5% and the incidence of appendicitis within a femoral hernia is extremely rare. Preoperative diagnosis is challenging to the treating surgeon, which requires a high index of suspicion and is usually made intraoperatively. There is no standard treatment; however, simple appendicectomy and hernia repair seems to be an accepted management.
We present an interesting case where a patient is presented with a droopy left eyelid (as part of Horner syndrome) and Cushingoid features which were a result of a Pancoast tumour (apical lung tumour in superior pulmonary sulcus) involving the left lung. This tumour was secreting ectopic adrenocorticotropic hormone (ACTH), a paraneoplastic endocrine phenomenon, which resulted in Cushing syndrome symptomatology. Though most ectopic ACTH-producing lung cancers are either small cell or carcinoid tumours, this was in fact a large cell neuroendocrine cancer (LCNEC). Patient underwent surgical resection and adjuvant/neoadjuvant chemotherapy with radiation; however, he succumbed to LCNEC given aggressive nature of the disease.
Uterine rupture in pregnancy is a rare and catastrophic complication with a high incidence of fetal and maternal morbidity. Very few cases have been reported in the literature.
Case presentationA 28-year-old fifth gravid woman with a history of one caesarean section presented to our department at 39 weeks and 6 days gestation with complaints of headache, epigastric pain and nausea. Her blood pressure was elevated and there was proteinuria. Emergency caesarean section was performed in view of symptoms. Uterine rupture was found during the surgery. A live male infant was delivered in good condition. Postnatal recovery was unremarkable and the woman discharged on postoperative day 5.
ConclusionRupture of the uterus can present in third trimester even before labour with minimal or no symptoms.
Description
A 16-year-old boy consulted for fever, left eyelid swelling, headache and vomiting. In the previous weeks, he reported having often bathed in the Ardèche river in France and has made many somersaults in jumping in the water. CT scan demonstrated filling of left ethmoid, maxillary and frontal sinuses with left eyelid infiltration and intraorbital extension. The patient was diagnosed with left ethmoiditis and was treated with intravenous amoxicillin–clavulanate. After 5 days of treatment, his fever had subsided but left exophthalmos, proptosis and ophthalmoplegia appeared with light reflex conservation (figure 1A). Fundus examination of the left eye was unremarkable, and dexamethasone and aminoside eye drops were added. A new CT scan was performed that showed an intraorbital and supraocular collection (9x23 mm) with left frontal sinus and left ethmoid opacification (figure 1B–D).
Figure 1
The 16-year-old boy with complicated ethmoiditis (A); CT scan revealed left...
Description
A Nigerian-born British national aged 24 years presented to our tuberculosis (TB) clinic after a positive, screening, quantiFERON blood test. She had been in the country for 12 years, had no known exposure to TB and no previous medical history. She was counselled regarding the risks and benefits of treating latent TB and then, following normal liver and kidney function blood tests, was started on combined rifampicin and isoniazid. She returned 4 weeks later to follow-up clinic with no systemic symptoms or side effects but with nail changes as shown in figure 1.
Figure 1
Photograph November 2015 showing the nail changes across all fingers and close up.
The diagnosis is half-and-half nails, in this case caused by isoniazid, likely due to its effects on niacin metabolism. However, half-and-half nails are most commonly seen in renal failure, and therefore, renal function tests...
Publication date: June 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 97
Author(s): Taro Iwasaki, Takashi Hirano, Satoru Kodama, Yoshinori Kadowaki, Munehito Moriyama, Toshiaki Kawano, Masashi Suzuki
ObjectiveAcute otitis media (AOM) is one of the most common infectious diseases in children. Nontypeable Haemophilus influenzae (NTHi) is Gram-negative bacteria that are considered major pathogens of AOM and respiratory tract infections. In this study, we used monophosphoryl lipid A (MPL), a toll-like receptor (TLR) 4 agonist, as an adjuvant to induce mucosal immune responses against NTHi to enhance bacterial clearance from the nasopharynx.MethodsMice were administered 10 μg outer membrane protein (OMP) from NTHi and 0, 10, or 20 μg MPL intranasally once a week for 3 weeks. Control mice were administered phosphate-buffered saline alone. After immunization, these mice were challenged with NTHi. At 6 and 12 h after bacterial challenge, the mice were killed and nasal washes and sera were collected. The numbers of NTHi- and OMP-specific antibodies were quantified by enzyme-linked immunosorbent assay.ResultsThe MPL 10 and 20 μg group produced a significant reduction in the number of bacteria recovered from the nasopharynx at 12 h after bacterial challenge compared to the control group. OMP-specific IgA titers were also augmented in the MPL groups compared to the control and OMP groups.ConclusionMPL is suitable for eliciting effective mucosal immune responses against NTHi in the nasopharynx. These results demonstrate the possibility of an adjuvant that involves stimulation of the innate immune system by TLR4 agonists such as MPL for mucosal vaccination.
http://ift.tt/2nh2P0Q
Publication date: June 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 97
Author(s): Sahba Sedaghat, Mario Tapia, Felipe Fredes, Pablo Rojas
IntroductionBilateral vocal cord paralysis in adducted position (BVCPAd) is a severe cause of airway obstruction and usually debuts with stridor and airway distress necessitating immediate intervention. Tracheostomy has long been the gold standard for treating this condition, but has significant associated morbidity and mortality in pediatric patients. New conservative procedures have emerged to treat this condition thus avoiding tracheostomy, like endoscopic anterior and posterior cricoid split (EAPCS). The objective of this paper was to review our experience with EAPCS in newborns and infants.MethodsProspective study involving patients undergoing endoscopic EAPCS for symptomatic BVCPAd. The primary outcomes were tracheostomy avoidance and resolution of airway symptoms.ResultsThree patients underwent EAPCS between January 2016 and December 2016. All patients stayed at least 7 days in the Intensive Care Unit (ICU) intubated. All patients presented complete resolution of their symptoms due to airway obstruction, without the need for tracheostomy.ConclusionEAPCS is a novel and effective alternative to treat BVCPAd in patients under 1 year old. Our study is an initial experience; more cases are required to identify the real impact and benefits of this technique and to determine the proper selection of patients.
http://ift.tt/2nmKFwj
Publication date: June 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 97
Author(s): Alessandra Spada Durante, Beatriz Massa, Beatriz Pucci, Nicolly Gudayol, Marcella Gameiro, Cristiane Lopes
ObjectiveTo determine the effect of passive smoking on auditory temporal resolution in primary school children, based on the hypothesis that individuals who are exposed to smoking exhibit impaired performance.DesignAuditory temporal resolution was evaluated using the Gaps In Noise (GIN) test. Exposure to passive smoking was assessed by measuring nicotine metabolite (cotinine) excreted in the first urine of the day.Study sampleThe study included 90 children with mean age of 10.2 ± 0.1 years old from a public school in São Paulo. Participants were divided into two groups: a study group, comprising 45 children exposed to passive smoking (cotinine > 5 ng/mL); and a control group, constituting 45 children who were not exposed to passive smoking. All participants had normal audiometry and immittance test results.ResultsStatistically significant differences (p < 0.005) in performance on the GIN test were found between the two groups, with mean thresholds of 5.3 ms and 68.9% correct responses in the study group versus 4.6 ms and 74.0% in the control group.ConclusionThe children exposed to passive smoking had poorer performance both in terms of thresholds and correct responses percentage on auditory temporal resolution assessment.
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Clear cell squamous cell carcinoma (CCSCC) is a rare histological subtype of squamous cell carcinoma (SCC) that was originally described in the skin. Here, we report a case of a 66-year-old female patient who presented with a fungating ulcerative mass of the left lateral tongue extending anteriorly to the floor of the mouth, and posteriorly to the left retromolar fossa and the oropharynx. The patient had a history of SCC of the left posterior tongue that was treated with partial glossectomy and adjuvant radiotherapy. Representative biopsies were obtained from the floor of the mouth, tongue and retromolar fossa. The examined biopsies showed various degrees of dysplastic surface epithelium with transition into infiltrating epithelial tumor nests and cords with clear cytoplasm and malignant cellular features. Pancytokeratin, CK5/6, and p63 were all diffusely positive. S-100, Calponin, and smooth muscle actin (SMA) were negative. PAS stain was diffusely positive and diastase labile in the tumor clear cells. Sparse areas of mucicarmine positivity were noted. Based on these findings a final diagnosis of a glycogen-rich CCSCC was given. This case represents a very rare histological variant of oral SCC, which is significant for the histological differential diagnosis of clear cell tumors of the oral cavity.
