Accessory bile duct: a rare but important anatomical variant

An 84-year-old man presented with pancreatic adenocarcinoma. Following neoadjuvant chemoradiation, the patient underwent a pancreaticoduodenectomy, complicated by early bile leak. Re-exploration and intraoperative cholangiogram identified an accessory common bile duct draining segment 5 of the right hepatic lobe, which was then ligated. The patient underwent a complicated postoperative course eventually developing sepsis secondary to biliary stasis. He elected for comfort measures and passed away secondary to complications of sepsis.

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Identification of left ventricular chamber-like aneurysm related to cardiac sarcoidosis

Description

A 58-year-old woman was referred to us with an abnormal ECG, showing complete right bundle branch block and left anterior fascicular block. An echocardiogram showed left ventricular (LV) septal thinning and hinted at the presence of an LV aneurysm. An enhanced CT indeed revealed an LV aneurysm-like structure. After appropriate testing to exclude coronary artery disease, this LV anomaly led us to suspect the possibility of cardiac sarcoidosis (CS). Cine cardiac magnetic resonance (CMR) imaging showed an apparent chamber-like aneurysm, and late gadolinium enhancement (LGE) CMR showed transmural positive enhancement in the mid-portion of the LV at the region of the aneurysm (figure 1A,B). As our institution lacked an FDG-PET scanner, gallium scintigraphy was instead performed, which showed uptake in the heart and bilateral hilar lymph nodes (figure 2A,B); hilar lymphadenopathy was undetected on enhanced CT, and its presence strongly indicated sarcoidosis. We finally made...

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Concurrent metastases of papillary thyroid carcinoma to the scalp and Meckels cave

We present the case of a 65-year-old man with severe headaches and unilateral facial weakness, seen in consultation by the dermatology service to rule out primary cutaneous melanoma after brain imaging identified an enlarging mass within the right trigeminal (Meckel's) cave. Examination revealed only a pair of erythematous papules on the scalp, for which biopsy demonstrated metastatic papillary thyroid carcinoma. Further evaluation and subsequent thyroidectomy confirmed the origin of widespread internal disease, followed by definitive excision of scalp lesions and multimodal management of systemic involvement. Whereas presentation of metastasis to the skin is highly variable, a low threshold for biopsy may allow for histological identification of internal disease not otherwise considered in the clinical differential.

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Varicella zoster lumbosacral plexopathy: a rare cause of lower limb weakness

This is a rare case of Varicella zoster virus (VZV) lumbosacral plexopathy in an 84-year-old women presenting with lower limb weakness and rash. Contrast-enhanced MRI showed enhancement of the left L3–L5 descending nerves and left lumbosacral plexus consistent with inflammatory/infectious aetiology. Cerebrospinal fluid PCR confirmed VZV DNA and cerebrospinal fluid serological testing was positive for VZV immunoglobulin (Ig)M and IgG antibodies. The patient was treated with intravenous acyclovir but this was complicated by the development of acute renal failure attributed to acyclovir-induced nephropathy, requiring dose adjustment. After a prolonged course of oral acyclovir and inpatient rehabilitation, the patient made a partial neurological and functional recovery.

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Bone inside eye: choroidal osteoma presenting as exudative retinal detachment: a challenge to diagnosis

Description 

A 38-year-old woman presented to outpatient department with the chief complaints of gradual onset of painless diminution of vision in the right eye since last 1 month. At presentation, the visual acuity in OD was perception of light and 20/20 in OS. Slit-lamp examination of anterior segment was found to be within normal limits in both eyes. On fundus evaluation of right eye, exudative retinal detachment1 with shifting fluid along with oedematous disc with blurry margin and juxtapapillary subretinal yellowish brown mass were noticed (figure 1A). Fundus in the left eye was within normal limits (figure 1B). With the above clinical examination, a provisional diagnosis of right-eye choroidal osteoma with exudative retinal detachment was made and confirmed on sonography, with an elevated highly reflected choroidal mass persisting at lower scanning sensitivity and acoustic shadow along with retinal detachment (figure 2A). CT scan depicted evidence of...

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Tracheal agenesis in a new born: lessons learnt

Tracheal agenesis is a rare but fatal congenital tracheal malformation. Lack of prenatal symptom and a typical clinical presentation lead to failure to arrive at a correct diagnosis and confusion during resuscitation. We report a case of a newborn male child with type 2 tracheal agenesis. Despite a typical presentation, diagnosis was delayed after unsuccessful intubation, examination under anaesthesia and emergency tracheostomy. The embryology, diagnostic criteria and potential treatment options are discussed. This case report is valuable in increasing awareness of this rare condition and will help us in being better prepared in managing these children. Future studies should aim to find the optimal replacement for the tracheal.

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Isolated thyroid metastasis from benign phyllodes tumour

We present a rare and interesting case of a 35-year-old woman who initially underwent an uneventful wide excision for a 13 cm left benign phyllodes tumour. She then noted a slowly growing left thyroid nodule 8 months postsurgery which on thyroidectomy 4 years later was shown to be a 6.9cm isolated thyroid metastasis from the phyllodes tumour. As this may be the first reported such case in the literature, implications on histological classification, predictive factors for disease progression, mechanisms of metastasis, and evaluation, management and surveillance of benign phyllodes tumours and thyroid nodule/s with a history of phyllodes tumour can thus be significantly impacted.

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Beware of beta! A case of salbutamol-induced lactic acidosis in severe asthma

A 22-year-old woman presented with symptoms and signs consistent with acute severe asthma. After significant doses of beta-agonist, she developed a significant lactic acidosis. Significant issues arose in this patient's history with regards to purchase of medications, compliance and follow-up with respiratory service. Beta-adrenergic receptors when stimulated have been hypothesised to increase lipolysis, producing free fatty acids, which inhibit the conversion of pyruvate to coenzyme A within the Krebs cycle. Additional pyruvate is generated through stimulation of glycolysis and glycogenolysis through simultaneous catecholamine surge. This increased pyruvate load is shunted through anaerobic glycolysis, producing increased lactate. Steroid use during an asthma attack enhances the beta-2 receptor sensitivity, further potentiating lactate production. The hyperadrenergic state in this young asthmatic likely resulted in pyruvate and therefore lactate rise and thus metabolic acidosis as mentioned before. This piece highlights a physiological phenomenon that may occur in the context of iatrogenic hyperadrenergism.

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Phemphigoid gestationis

Description 

A 32-year-old nulliparous woman presented at 33 weeks' gestation with intense pruritus initiated 1 month earlier. She was under treatment for scabies, with no improvement. She was healthy and her pregnancy had had no intercurrences so far. During the physical examination at the emergency room, extensive erythematous plaques with vesicles and bullae could be seen on her abdomen, trunk, neck and all four extremeties (figures 1 and 2).

Figure 1

Macroscopic skin lesions of 33-week pregnant woman: initially, lesions are like erythematous papules or targetoid lesions (A, abdomen; B, back).

Figure 2

Macroscopic skin lesions of 33-week pregnant woman: over time, small vesicles or bullae appear on normal skin or on top of urticarial plaques (C, feet).

Due to the severity of her symptoms, she was admitted to the obstetrics ward in order to undergo intravenous...

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Case of posterior cortical atrophy (PCA) evolved to PCA-CBS

A 68-year-old lawyer developed insidious disturbances in topographic orientation and apraxia. He underwent a geriatric evaluation, only documenting slight cognitive disturbances, and a 18F-fluorodeoxyglucose positron emission tomography (FDG-PET), showing mild right-lateralised frontoparietal hypometabolism. After 1 year, because of worsening in spatial orientation and the onset of dressing apraxia, he was referred to our memory clinic. The neuropsychological evaluation documented proeminent visuospatial, praxis deficits and dysgraphia. Cerebrospinal fluid biomarker analysis showed mild increase of total-, with normal Aβ1–42, ruling out Alzheimer's disease. Progression of the right parietal hypometabolism at FDG-PET and right superior longitudinal fasciculus damage at high-field MRI revealed a probable neurodegenerative aetiology. The neurological examination disclosed then Gerstmann's and Balint's syndromes, and extrapyramidal signs later appeared, suggesting the diagnosis of posterior cortical atrophy associated with corticobasal syndrome. Genetic analysis for mutations inmicrotubule-associated protein tau (MAPT), C9orf72 and GRN genes was negative. A 1-year follow-up documented significant worsening of the cognitive and functional impairment, revealing a frank dementia.

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Beliefs of a traditional rural Indian family towards naturalistic and faith healing for treating epilepsy: a case study

In this case study, we describe our experiences with a rural poor family from north India that initially contacted faith healers for treatment of their child who was having symptoms suggestive of epilepsy, but the seizures continued even after this. The family migrated to a city, where they started allopathic treatment, but eventually they had to discontinue it as there was no apparent relief. Again, they went back to their native village and restarted the treatment from the faith healer.

This case study highlights the fact that in spite of the significant development of medical science, many questions pertaining to epilepsy treatment are still unanswered. Such dissatisfaction with the allopathic treatment of epilepsy is very common. Complexity of the disease and high cost of modern medication, side effects of drugs, efficient but heavy treatment protocols and unpredictable outcome are responsible for continued practice of people consulting faith healers for treatment of epilepsy. However, these remain unnoticed and undocumented.

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Correction: Multiloculated mesothelial cyst presenting as a malignant mimic

Peng S, Park L, Kilpatrick SE, et al. Multiloculated mesothelial cyst presenting as a malignant mimic. BMJ Case Rep 2018. doi: 10.1136/bcr-2017-222280.

This article was published with the author order listed incorrectly. The correct author order is as follows: June S Peng, Lisa Park, Scott E Kilpatrick, Sricharan Chalikonda.

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Explanation and abstraction from a backward-error analytic perspective

Abstract

We argue that two powerful error-theoretic concepts (backward error and conditioning) provide a general framework that satisfactorily accounts for key aspects of the explanation of physical patterns. This method gives an objective criterion to determine which mathematical models in a class of (possibly idealized) neighboring models are just as good as the exact one. The method also emphasizes that abstraction is essential for explanation and provides a precise conceptual framework that determines whether a given abstraction is explanatorily relevant and justified. Hence, it increases our epistemological understanding of how one should go about reconstructing scientific practices by making clear that, at a fundamental level, a key aspect of mathematical modeling consists in exactly solving nearby problems.

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Epidemiology of spider mite sensitivity: a meta-analysis and systematic review

Spider mites, including Tetranychus urticae, Panonychus citri, and Panonychus ulmi, are common pests in gardens, greenhouses, and orchards. Exposure, particularly occupational exposure, to these organisms may lea...

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Comparative immunological study of the snail Physella acuta (Hygrophila, Pulmonata) reveals shared and unique aspects of gastropod immunobiology

Publication date: September 2018
Source:Molecular Immunology, Volume 101
Author(s): Jonathan H. Schultz, Lijing Bu, Coen M. Adema
The freshwater snail Physella acuta was selected to expand the perspective of comparative snail immunology. Analysis of Physella acuta, belonging to the Physidae, taxonomic sister family to Planorbidae, affords family-level comparison of immune features characterized from Biomphalaria glabrata, the model snail often used to interpret general gastropod immunity. To capture constitutive and induced immune sequences, transcriptomes of an individual Physella acuta snail, 12 h post injection with bacteria (Gram –/+) and one sham-exposed snail were recorded with 454 pyrosequencing. Assembly yielded a combined reference transcriptome containing 24,288 transcripts. Additionally, genomic Illumina reads were obtained (∼15-fold coverage). Recovery of transcripts for two macin-like antimicrobial peptides (AMPs), 12 aplysianins, four LBP/BPIs and three physalysins indicated that Physella acuta shares a similar organization of antimicrobial defenses with Biomphalaria glabrata, contrasting a modest AMP arsenal with a diverse set of antimicrobial proteins. The lack of predicted transmembrane domains in all seven Physella acuta PGRP transcripts supports the notion that gastropods do not employ cell-bound PGRP receptors, different from ecdysozoan invertebrates yet similar to mammals (vertebrate deuterostomes). The well-documented sequence diversification by Biomphalaria glabrata FREPs (immune lectins comprising immunoglobulin superfamily domains and fibrinogen domains), resulting from somatic mutations of a large FREP gene family is hypothesized to be unique to Planorbidae; Physella acuta revealed just two bonafide FREP genes and these were not diversified. Furthermore, the flatworm parasite Echinostoma paraensei, confirmed here to infect both snail species, did not evoke from Physella acuta the abundant expression of FREP proteins at 2, 4 and 8 days post exposure that was previously observed from Biomphalaria glabrata. The Physella acuta reference transcriptome also revealed 24 unique transcripts encoding proteins consisting of a single fibrinogen-related domain (FReDs), with a short N-terminal sequence encoding either a signal peptide, transmembrane domain or no predicted features. The Physella acuta FReDs are candidate immune genes based on implication of similar sequences in immunity of bivalve molluscs. Overall, comparative analysis of snails of sister families elucidated the potential for taxon-specific immune features and investigation of strategically selected species will provide a more comprehensive view of gastropod immunity.



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Cerebellar liponeurocytoma – a rare entity: a case report

Cerebellar liponeurocytoma is a rare tumor of the central nervous system occurring mainly in the posterior fossa, which shows neuronal and variable astrocytic differentiation with foci of lipomatous differenti...

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Application of “banana cochleostomy” and looped electrode insertion for cochlear implantation in children with common cavity malformation and cystic forms of cochlear hypoplasia

Publication date: September 2018
Source:International Journal of Pediatric Otorhinolaryngology, Volume 112
Author(s): Józef Mierzwiński, Egwin Van Den Heuvel, Andrew J. Fishman, Arnaldo L. Rivera, Karolina Haber, Jiri Skrivan
ObjectivesPatients with profound hearing loss due to inner ear malformations may benefit from cochlear implantation; however, the surgery may present a substantial problem for the cochlear implant surgeon due to anatomical variations.The authors describe a new surgical and technical advancement for implantation in patients with small inner ear cavities that make the surgery easier and safer. On the basis of experience involving five consecutive surgeries performed in four patients with inner ear malformations, we present the advantages and application possibilities of the technique.MethodsThe technique does not change the surgical approach in general; however, modification of the cochleostomy shape and looping of the cochlear implant electrode enables safe advancement of the electrode with optimal positioning in the cavity. Additionally, these modifications protect against the insertion of the electrode into the internal auditory canal minimizing the risk of gushing and extracochlear stimulation.ResultsThe present technique has been used in five cases of cystic implantable inner ear spaces in three independent institutions by different surgeons. It has proven to be a reliable, relatively easy and safe procedure performed with very good anatomic and initially functional effects (positive intraoperative neural response telemetry measurements).ConclusionsWe hope that utilization of the "banana cochleostomy" and insertion of the looped cochlear implant electrode in the implantable cystic spaces of children with malformed inner ears will facilitate and simplify the surgical technique in this difficult procedure and additionally, in revision surgical cases. To our knowledge, the looped insertion and banana-shaped cochleostomy have not been reported previously.



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