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Preview: QJM - current issue

QJM: An International Journal of Medicine Current Issue

Published: Tue, 09 May 2017 00:00:00 GMT

Last Build Date: Tue, 09 May 2017 11:53:24 GMT


Pokémon GO & driving


Leaning points for cliniciansPokémon GO can be a substantial distraction for drivers and pedestrians. Calm and balanced discussions for prioritizing pedestrian safety are imperative to manage the increasing use of Pokémon GO while driving.

FDG PET diagnosis of primary intracranial lymphoma: radiology–pathology correlation


A 61-year-old gentleman presented to the emergency department with fevers and ataxia. He had a 2-year history of haemophagocytic lymphohistiocytosis (HLH), a rare disorder associated with haematological malignancy, infection or autoimmune disease.1

Creutzfeld Jacob disease


Learning points for clinicians
  • Prions are small proteinaceous particles. Their penetration in the nervous central system results in further replication, suppression of cellular function and death.
  • We recently treated three successive patients with Creutzfeld Jacob Disease, a form of prion disease.
  • This rare and fatal disease should be recognized and prevention efforts implemented.

Mobile digital health devices and the diagnosis in real-time of myocardial ischaemia


Learning points for clinicians
  • Mobile digital health devices that provide remote, patient-generated information are commercially available. This can challenge traditional health care delivery models, but can potentially reduce healthcare expenditure and improve clinical outcomes. Previous, difficult to establish diagnoses, such as coronary artery spasm, may be made with greater certainty using these devices.

Minocycline-induced hyperpigmentation


An 85-year-old woman presented to the dermatology outpatient department with bluish discoloration on her legs and arms. Non-palpable, non-pruritic blue patches of hyperpigmentation were noted over the lower legs bilaterally (Figure 1). She has been receiving minocycline for bullous pemphigoid for 2 years. The patient was diagnosed with minocycline-induced hyperpigmentation. The antimicrobial medication was stopped immediately. Several medications can cause hyperpigmentation, including minocycline, chloroquine, amiodarone and cyclophosphamide.1 It is important to recognize minocycline-induced hyperpigmentation early and to consider changing or withdrawing medication. Minocycline-induced hyperpigmentation is classified into four types. Type I is the most common, with blue-black macules commonly occurring on the face with previous inflammation and acne scars. Type II is described as blue-gray pigmentation related to pigmented minocycline metabolites that occurs on the forearms and legs with normal skin. Type III is the least common and is described as diffuse muddy brown macules on sun-exposed skin. Type IV has the same causality as type III, but only occurs on the thorax on pre-existing scars and is not limited to sun-exposed skin. In addition to the skin, minocycline-induced hyperpigmentation can also affect different body parts and organs, including the nails, sclera, teeth, gingivae, oral mucosa, bone and thyroid gland. This patient presented with type II hyperpigmentation because of prolonged minocycline intake. The cutaneous hyperpigmentation can take months to years to fade after minocycline withdrawal. Figure 1Photograph of the patient’s legs showed a diffuse bluish discoloration.

Sarcoidosis—the great masquerader


A 36-year-old Caucasian female presented with acute left-sided facial weakness with intermittent fevers. She was diagnosed with idiopathic Bell’s Palsy. After 3 weeks, she again presented with acute right sided facial weakness (Figure 1A). A history of pain in the bilateral preauricular and mandibular area was elicited. She was found to have bilateral parotid gland enlargement. Her eye exam was suggestive of bilateral uveitis as evidenced by bilateral conjunctival injection and posterior synechiae. A chest CT demonstrated mediastinal and hilar adenopathy (Figure 1B). Endobronchial ultrasound guided needle aspiration of the mediastinal lymph nodes was suggestive of a granulomatous process (Figure 1C). Based on these findings, a diagnosis of Heerfordt’s syndrome was made. She was started on prednisone 30 mg PO daily and has had a near complete resolution of her right sided facial palsy and her mediastinal and hilar lymphadenopathy. Figure 1(A) Patient demonstrated facial nerve palsy on the right side and bilateral swelling of the parotid gland. (B) Representative image of chest CT mediastinal window showing hilar (arrows) and mediastinal adenopathy (star). (C) Transbronchial needle aspirate demonstrating predominantly mixed lymphocytic population with groups of macrophages, some of which were multinucleated, suggestive of a granulomatous process.

Nonne-Froin sign


A 69-year old man presented with a 1-week history of ascending progressive weakness and low back pain. His past medical history was remarkable for diabetes mellitus, hypertension and heavy smoking. On physical examination, the patient was afebrile with paraparesis, his knee and ankle reflexes were absent, and a sensory level could not be established. A lumbar computed tomography (CT) was non-contributory. Guillian-Barre syndrome (GBS) was considered as the most likely diagnosis and the patient was referred to lumbar puncture and further workup. His lumbar puncture revealed a yellow cerebrospinal fluid (CSF) that became instantly gelatinous (Figure 1A). CSF analysis revealed a protein concentration of 2583 mg/dl (normal values: 20–40 mg/dl), glucose of 21 mg/dl (glucose serum = 84 mg/dl) and 5 cells. CSF cytologic examination was negative for malignant cells. The patient underwent head CT that showed two hypodense lesions in the right cerebellum and right fronto-parietal lobe with slight central enhancement, consistent with brain metastasis, these lesions did not explain his neurological findings. Lumbar magnetic resonance imaging (MRI) showed an intra-dural mass measuring 25 mm by 11 mm at the level of T12-L1 with extension into the extradural space (Figure 1B). The patient underwent extensive diagnostic testing in search of a primary tumour outside the central nervous system. Chest CT showed a left lower lobe lung mass. Immunohistochemistry studies from a CT-guided biopsy of the lung mass were consistent with non-small cell lung carcinoma. Figure 1(A) CSF is yellow and coagulated in the tube. (B) Sagittal T2-weighted MRI showing a well-demarcated intradural mass at the T12-L1 level (arrow).

Corkscrew esophagus


A 51-year-old woman presented with a 4-year history of acid reflux symptoms of retrosternal burning and chest tightness, without significant dysphagia. The patient exhibited obvious anxiety during the onset. She denied family history and past medical history. According to her description, these symptoms developed initially following an occasional hiccup. Physical examination revealed all vital signs was normal. And then, a single-contrast barium examination of upper gastrointestinal was performed, which showed that the esophagus presented suddenly an abnormal spiral appearance of peristalsis (Figure 1A). Upper gastrointestinal endoscopy also revealed the corkscrew appearance in distal esophagus like a winding staircase (Figure 1B). Manometry of esophagus confirmed an alternative form of normal peristalsis and an absence of peristalsis in the esophageal body and relaxation of lower esophageal sphincter. Based on these findings above, we made the diagnosis of corkscrew esophagus. The patient was treated with antireflux and acid suppression therapies for six months without significant relief of symptoms. Figure 1(A) Barium examination of upper gastrointestinal initially showed the esophagus presented typical corkscrew features of the distal esophagus. (B) Upper gastrointestinal endoscopy revealed an abnormal appearance of the esophagus-like winding staircase.

Disseminated tuberculosis


By definition, the patient in the case report1 had disseminated tuberculosis,2 given the fact that Mycobacteriumtuberculosis (M.tuberculosis) was identified from specimens obtained from two non-contiguous organs, namely, the lungs and the calcaneus. Tuberculosis is deemed to be disseminated ‘when m tuberculosis is isolated from blood or from bone marrow … … or from specimens from two or more non contiguous organs in a single patient’.2 In the modern era of molecular diagnosis, this definition should include molecular identification of M.tuberculosis. Lymphocytopenia may have been a risk factor, both for m tuberculosis infection,3, and for its dissemination, given the fact that adequate numbers of CD4+ and CD8+ T lymphocytes are required to mount a host defence(via interferon gamma production) against M.tuberculosis infection,3 and also in view of the observation that, even among subjects who do not have human immune deficiency virus(HIV) coinfection, those with disseminated disease(miliary or localization in more than one tissue) have significantly(P = 0.000 lower CD4 cell counts than patients who have disease localized either in lymph nodes or in the lungs.4 Furthermore, in that study, post-treatment CD4 counts in disseminated disease remained significantly (P = 0.005) lower than corresponding post-treatment cell counts in localized disease.4 Accordingly, we also need to know the post treatment lymphocyte count in the reported case,1 given the fact that m tuberculosis infection can, in its own right be a cause of reversible lymphocytopenia.5

Response: Disseminated tuberculosis


Lymphopaenia is described among patients with tuberculosis. The lymphocyte count for the patient in our publication1 prior to treatment was 0.8 (1–2 × 1016 ml). At the time of writing, he is still receiving his anti-tuberculous treatment and his last measured lymphopaenia is 1.2.

Idiosyncratic drug-induced neutropenia and agranulocytosis


Background/Introduction: Few data is currently available on neutropenia and agranulocytosis related to drug intake.Aim: We report here data on 203 patients with established idiosyncratic drug-induced agranulocytosis, followed up in a referral centre within a university hospital.Design: Data from 203 patients with idiosyncratic drug-induced agranulocytosis were retrospectively reviewed.Methods: All cases were extracted from a cohort study on agranulocytosis in the Strasbourg University Hospital (Strasbourg, France).Results: The mean age was 61.6 years old (range: 18-95), the gender ratio (F/M) was 1.3. Several comorbidities were present in 63.5%. The most frequent causative drugs were: antibiotics (49.3%), especially β-lactams and cotrimoxazole; antithyroid drugs (16.7%); neuroleptic and anti-epileptic agents (11.8%); antiviral agents (7.9%); and platelet aggregation inhibitors as ticlopidine and acid acetylsalicylic (6.9%). The main primary clinical manifestations during hospitalization included: isolated fever (26.3%); septicaemia (13.9%); documented pneumonia (13.4%); sore throat and acute tonsillitis (9.3%); and septic shock (6.7%). The mean neutrophil count at nadir was 0.148 × 109/L (range: 0-0.48). All febrile patients were treated with broad-spectrum antibiotics and 107 (52.7%) with hematopoietic growth factors. The mean duration of haematological recovery (neutrophil count ≥1.5 × 109/L) was 7.8 (range: 2-20). This mean duration was reduced to 2.1 days (range: 2-16) (p=0.057) with hematopoietic growth factors. Outcome was favourable in 91.6% of patients; seventeen died. Thirty-seven patients (18.2%) required intensive care.Discussion/Conclusion: The present study demonstrated that idiosyncratic drug-induced agranulocytosis is a relative rare events; that antibiotics, antithyroid, neuroleptic and anti-epileptic agents, and platelet aggregation inhibitors are the main incriminated drug classes; that agranulocytosis typically serious, with at least 50% exhibiting severe sepsis and a mortality rate <10%; and that modern management of such disorder may reduce the infection-related mortality.

Lung lymphoma with ground glass opacity CT image


The patient was a 69-year-old man who had previously undergone subtotal esophagectomy plus gastric tube reconstruction for lower esophageal carcinoma. Approximately 1 month prior to hospitalization, he presented with expectoration and coughing. A thoracic computed tomography (CT) scan revealed ground glass opacity and infiltrative shadows in both the lungs. Moxifloxacin treatment failed to result in any improvement. The patient then presented with a >38 °C remittent fever and was admitted to our hospital. Blood tests revealed a high level of soluble interleukin-2 receptor (10 100 U/ml). Although malignant lymphoma was suspected, contrast-enhanced thoracoabdominal and pelvic CT did not reveal evident lymph node enlargement or hepatosplenomegaly. Chest CT revealed exacerbated ground glass opacity in both the lungs (Figure 1a), while positron emission tomography-CT revealed accumulation of fluorodeoxyglucose in the same sites (Figure 1b). Transbronchial lung biopsy revealed infiltration of diffuse, large B-cell lymphoma cells localized in the pulmonary vessels. The patient subsequently underwent rituximab + CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) chemotherapy, which resulted in an improvement in the lung lesions. Figure 1(a) Chest computed tomography (CT). Chest CT revealed bilateral diffuse ground glass opacity and infiltrative shadows distributed around the bronchovascular bundles. (b) Positron emission tomography-computed tomography (CT). Positron emission tomography-CT revealed accumulation of fluorodeoxyglucose consistent with the opacity sites observed in CT.

Successful removal of multiple long foreign bodies: an unusual neck hyperextension technique


A 22-year-old female with a background of depression and personality disorder underwent an emergency endoscopy after swallowing multiple long foreign bodies (FB). She had a previous history of several instances of ingesting FB, requiring a laparotomy a year previously.

Survival analysis of weekend emergency medical admissions


Background: We previously reported weekend emergency admissions to have a higher mortality; we have now examined the time profile of deaths, by weekday or weekend admission, in all emergency medical patients admitted between 2002 and 2014.Methods: We divided admissions by a weekday or weekend (After 17.00 Friday–Sunday) hospital arrival. We examined survival following an admission using Cox proportional hazard models and Kaplan-Meier time to event analysis.Results: In total 82 368 admissions were recorded in 44, 628 patients. Weekend admissions had an increased mortality of 5.0% (95% CI 4.7, 5.4) compared with weekday admissions of 4.5% (95% CI 4.3, 4.7) (P = 0.007). The univariate adjusted Odds Ratio (OR) of death for a weekend admission was significantly increased OR = 1.15 (95% CI 1.05, 1.24) (P = 0.001). Mortality following an admission declined exponentially over time with a long tail, ∼25% of deaths occurred after day 28. Only 11.4% of deaths occurred on the weekend of the admission. Survival curves showed no mortality difference at 28 days (P = 0.21) but a difference at 90 days (P = 0.05). The higher mortality for a weekend admission was attributable to late deaths in the cohort with an extended stay; compared with weekday, these weekend admissions were more likely to be older and have greater co-morbidity.Conclusion: Survival rates following a weekend or weekday admission were similar out to 28 days. The higher overall mortality for weekend admissions is due to divergence in survival between 28 and 90 days. Most deaths in weekend admissions occurred when the hospital was fully staffed.

Underuse of screening in Osler–Weber–Rendu syndrome


According to the view expressed by the authors the management of Hereditary Haemorrhagic Telangiectasia (HHT) should include prevention of complications of vascular lesions.1 Accordingly, the opportunity should have been taken to screen the patient for pulmonary arteriovenous malformations (PAVM), the latter a feature in 15–50% of patients with HHT.2 With transthoracic contrast echocardiography as the imaging modality, the International Guidelines recommend screening all HHT patients for PAVM (level of evidence III, strength of evidence: 96% agreement), on the premise that screening will detect PAVM before the development of life-threatening or debilitating complications.2 The latter include stroke (in 33% of HHT patients) and cerebral abscess in 23%.3 Embolization is the mainstream therapy for PAVM, one of its aims being the prevention of strokes and cerebral abscesses attributable to PAVM-related right to left shunting. According to the 2015 update of the Cochrane systematic review of the literature, embolotherapy appears to reduce mortality and morbidity compared with no treatment.3 This is exemplified by the strongest model for HHT-related ischaemic stroke where embolization at a median age of 45 significantly (P = 0.028) reduced the rate of this complication.4 There are, however, no randomized controlled trials (RCT) to provide definitive evidence for or against embolotherapy for PAVM.3 Purely on ethical grounds, such trials would not be feasible.3

Pulmonary artery sarcoma mimicking pulmonary embolism: a case series


Background: Pulmonary artery sarcoma (PAS) is a rare malignant neoplasm with an aggressive behavior and often difficult to distinguish from pulmonary thromboembolic disease.Aim: To assess the demographic, clinical, and radiological characteristics of PAS and clinical course.Design and Methods: We retrospectively identified and analyzed all patients with PAS seen at Mayo Clinic in Rochester, Minnesota, between January 1, 1996 and July 31, 2015.Results: Of nine patients (5 women and 4 men; median age 55 years [range, 24–74 years]), eight were diagnosed while alive with surgical (n = 6) or catheter-based endovascular biopsy (n = 2); the remaining patient was diagnosed at postmortem examination. All tumors manifested on CT as filling defect in the main, right or left pulmonary artery and were not associated with peripheral filling defects in seven patients. Seven patients were initially treated with anticoagulant therapy for presumed PE; two patients were suspected to have tumor based on constrictive or expanding effect seen on CT. Five patients died after a mean duration of 2.1 years (10 months–4.25 years) after diagnosis. Two patients are alive with recurrence and metastases of the disease 23 and 27 months after diagnosis, respectively; one remaining patient is alive and disease-free 116 months after diagnosis.Conclusions: Although PAS is associated with a poor prognosis, long-term survival is possible and can be improved by early diagnosis and prompt surgical resection. Atypical appearance on CT including central mass-like lesion without peripheral emboli and constrictive or expanding effect should raise suspicion of PAS.

Familial Mediterranean fever-associated diseases in children


Background: MEditerranean FeVer (MEFV) gene encodes for the pyrin protein and a mutated pyrin is associated with a prolonged or augmented inflammation. Hence, various diseases were reported to be associated with familial Mediterranean fever (FMF) or carriers of MEFV mutations. However, systematic evaluation of all associated diseases in children with FMF has not been done previously.Aim: The aim of this study was to investigate the frequency and type of FMF-associated diseases in children.Design and Methods: Files of FMF patients who had been seen in two reference hospitals in Ankara, in the last two years, were retrospectively evaluated. Patients with FMF and concomitant diseases were included to the study.Results: Among 600 FMF patients, 77 were found to have a concomitant disease (12.8%). Thirty patients (5%) had vasculitis; 21 (3.5%) had juvenile idiopathic artritis (JIA); 7 (1.16%) had inflammatory bowel disease (IBD) and 19 had other diseases including 5 patients with isolated sacroiliitis. Overall, 13 (2.17%) patients had sacroiliitis in our cohort. The most frequent mutation was M694V/M694V (44%) and 81% of the patients had at least one M694V mutation. Majority of the patients (74%) developed associated diseases while they were not receiving colchicine therapy.Conclusions: Certain inflammatory diseases including vasculitis, chronic arthritis and IBD were more frequently detected in patients with FMF during childhood. M694V mutation is a susceptibility factor for associated diseases. In countries where FMF is prevalent, clinicians dealing with FMF and other inflammatory diseases should be aware of these associations.

Clinical outcomes following unilateral adrenalectomy in patients with primary aldosteronism


Background: In approximately half of cases of primary aldosteronism (PA), the cause is a surgically-resectable unilateral aldosterone-producing adrenal adenoma. However, long-term data on surgical outcomes are sparse.Aim: We report on clinical outcomes post-adrenalectomy in a cohort of patients with PA who underwent surgery.Design: Retrospective review of patients treated for PA in a single UK tertiary centre.Methods: Of 120 consecutive patients investigated for PA, 52 (30 male, median age 54, range 30–74) underwent unilateral complete adrenalectomy. Blood pressure, number of antihypertensive medications, and serum potassium were recorded before adrenalectomy, and after a median follow-up period of 50 months (range 7–115). Recumbent renin and aldosterone were measured, in the absence of interfering antihypertensive medication, ≥3months after surgery, to determine if PA had been biochemically cured.Results: Overall, blood pressure improved from a median (range) 160/95 mmHg (120/80–250/150) pre-operatively to 130/80 mmHg (110/70–160/93), P < 0.0001. 24/52 patients (46.2%) had cured hypertension, with a normal blood pressure post-operatively on no medication. 26/52 (50%) had improved hypertension. 2/52 patients (3.8%) showed no improvement in blood pressure post-operatively. Median (range) serum potassium level increased from 3.2 (2.3–4.7) mmol/l pre-operatively to 4.4 mmol/l (3.3–5.3) post-operatively, P < 0.0001). Median (range) number of antihypertensive medications used fell from 3 (0–6) pre- to 1 post-operatively (range 0–4), P < 0.0001.Conclusions: Unilateral adrenalectomy provides excellent long-term improvements in blood pressure control, polypharmacy and hypokalaemia in patients with lateralizing PA. These data may help inform discussions with patients contemplating surgery.

Heisenberg's uncertainty principle


Most of the physicians that I know are chary of Physics, especially the theory of elementary particles. Even if in Isaac Newton's mathematically precise universe, everything follows clear-cut laws and predictions are easy given appropriate starting conditions.

Make the healthy choice the easy choice: using behavioral economics to advance a culture of health


Despite great advances in the science and technology of health care, a large gap separates theoretically achievable advances in health from what individuals and populations actually achieve. Human behavior sits on the final common pathway to so many of our health and health care goals, including the prevention and management of illness and the fostering of wellness. Behavioral economics is a relatively new field offering approaches to supplement many of the conventional approaches to improving health behaviors that rely on education or standard economic theory. While those conventional approaches presume that an educated public will naturally make decisions that optimize personal welfare, approaches derived from behavioral economics harness existing and predictable patterns of behavior that often lead people to make choices against their best interests. By keeping these predictable patterns of behavior in mind when designing health insurance, health care programs or the health-related aspects of everyday life, behavioral economists aim to meet people half-way: no longer asking them to reshape their behavior to something more health promoting, but helping the behavioral patterns they already follow lead them to better health.

Death by neurological criteria: expert definitions and lay misgivings


For decades, there has been persistent controversy concerning brain death, or the determination of death by neurological criteria, among physicians, philosophers, and the lay public. This article examines the various ways that brain death is conceptualized and justified, as well as the persistent questions and controversies related to brain death, particularly within pluralistic, multicultural societies. A culturally sensitive and practical way forward is proposed.