An update on the ophthalmic features in hereditary haemorrhagic telangiectasia (Rendu-Osler-Weber syndrome)

Hereditary haemorrhagic telangiectasia (HHT) or Osler-Rendu-Weber syndrome is a rare autosomal dominant disease, characterised by systemic angiodysplasia. Dysfunction of the signalling pathway of β transforming growth factor is the main cause of HHT principally owing to mutations of the genes encoding for endoglin (ENG) and activin A receptor type II-like 1 (ACVRL1). Clinical manifestations can range from mucocutaneous telangiectasia to organ arterio-venous malformations and recurrent epistaxis. The early clinical manifestations may sometimes be subtle, and diagnosis may be delayed. The main ophthalmic manifestations historically reported in HHT are haemorrhagic epiphora, and conjunctival telangiectasia present in 45–65% of cases, however, imaging with wide-field fluorescein angiography has recently shown peripheral retinal telangiectasia in 83% of patients. Optimal management of HHT requires both understanding of the clinical presentations and detection of early signs of disease. Advances in imaging methods in ophthalmology such as wide-field fluorescein angiography, spectral domain optical coherence tomography, and near infrared reflectance promise further insight into the ophthalmic signs of HHT towards improved diagnosis and early management of possible severe complications.

Late-Stage Presentation of Testicular Cancer Patients Attended a Specialized Hospital: Findings of a Crosssectional Study

Background: Testicular Cancer (TC) is of interest and importance because its incidence has been increasing in most countries over the past four decades. The purpose of this study is to determine the frequency of patients with common histopathology, most common stage during presentation, biochemical and radiological findings, common levels of tumour markers. Methods: This cross-sectional study was carried out in the Department of Medical Oncology of National Institute of Cancer Research and Hospital (NICRH), Mohakhali, Dhaka, Bangladesh from July 2015 to June 2016. A total number of 52 patients were taken as study participants who are admitted as newly diagnosed at the NICRH. Data collection: tumour markers, USG of whole abdomen and chest X-ray were performed to all the patients for determination of the staging of the tumour. Results: The present study found the majority of the patients were found in stage III disease. Patients with Cryptorchidism needed to be educated regarding its early management to reduce the incidence of testicular tumour among them. It was observed that young people could be adequately knowledgeable and optimal awareness could be developed regarding early symptoms of the disease, then they may be found in earlier stage of their disease, which is curable with modern management method. Targeting to alter the cancer patients’ presentation by rising consciousness about TC, prognosis can be largely improved in future. Conclusion: This study was conducted to find out the features which were unknown in perspective of Bangladesh, so that burden of TC can be reduced, and prognosis can be further improved of this potentially curable disease. JOPSOM 2021; 40(1): 26-33

Wellens Syndrome: A Review Article

Wellens syndrome is a pre-infarct stage of coronary syndrome and a clinical condition associated with left descending proximal anterior artery stenosis. Diagnostic criteria for Wellens syndrome include clinical history, ECG changes, and laboratory results. The pathognomonic ECG of Wellens syndrome is anterior inverted T-wave in precordial leads, which can be seen during pain-free period, accompanied by isoelectric or minimally elevated ST segment (<1 mm), no precordial Q waves, and normal or slightly elevated cardiac serum markers. Early diagnosis leads to early management and a better prognosis. Early management of Wellens syndrome involves maintaining an airway, breathing, circulation, monitoring vital signs, and medical management. Still, the only definitive management is urgent cardiac angiography to prevent further myocardial ischemia. The combination of early diagnostic and appropriate management will reduce complications and mortality rates. Keywords: Wellens Syndrome, Acute Coronary Syndromes, pre-Infarct MI.

Controversies and evidence gaps in the early management of severe traumatic brain injury: back to the ABCs

Traumatic brain injury (TBI) accounts for around 30% of all trauma-related deaths. Over the past 40 years, TBI has remained a major cause of mortality after trauma. The primary injury caused by the injurious mechanical force leads to irreversible damage to brain tissue. The potentially preventable secondary injury can be accentuated by addressing systemic insults. Early recognition and prompt intervention are integral to achieve better outcomes. Consequently, surgeons still need to be aware of the basic yet integral emergency management strategies for severe TBI (sTBI). In this narrative review, we outlined some of the controversies in the early care of sTBI that have not been settled by the publication of the Brain Trauma Foundation’s 4th edition guidelines in 2017. The topics covered included the following: mode of prehospital transport, maintaining airway patency while securing the cervical spine, achieving adequate ventilation, and optimizing circulatory physiology. We discuss fluid resuscitation and blood product transfusion as components of improving circulatory mechanics and oxygen delivery to injured brain tissue. An outline of evidence-based antiplatelet and anticoagulant reversal strategies is discussed in the review. In addition, the current evidence as well as the evidence gaps for using tranexamic acid in sTBI are briefly reviewed. A brief note on the controversial emergency surgical interventions for sTBI is included. Clinicians should be aware of the latest evidence for sTBI. Periods between different editions of guidelines can have an abundance of new literature that can influence patient care. The recent advances included in this review should be considered both for formulating future guidelines for the management of sTBI and for designing future clinical studies in domains with clinical equipoise.

Early Management of Cervical Impairments and the Incidence of Delayed Recovery in Adolescents With Acute Concussion

ObjectiveTo determine if concussed adolescents with positive cervical findings on examination provided with early cervical treatment demonstrate recovery time and incidence of persistent post-concussive symptoms (PPCS, recovery = 30 days) comparable to those without cervical findings.BackgroundCervical impairments resulting from concussive injury are historically associated with longer recovery times. Concomitant cervical injuries may result in symptoms that overlap with concussion, including headache, dizziness, tinnitus, sleep disturbances, and blurred vision. Current recommendations include assessment of the cervical spine; however, there is limited evidence for the effect of immediate cervical intervention on recovery.Design/MethodsRetrospective case-controlled study. Patients included adolescents with acute concussion presenting with (n = 132, 14.99 ± 1.9 years, 58% male, 5.70 ± 3.3 days since injury) and without (n = 138, 14.85 ± 1.8 years, 65% male, 6.13 ± 3.4 days since injury) cervical findings on physical exam. Patients were assessed with the Buffalo Concussion Physical Examination (BCPE), Neck Disability Index (NDI) and Post-Concussion Symptom Scale (PCSS) instruments. Groups were separated based on positive or negative cervical findings on the BCPE. Cervical impairments were addressed by physicians specializing in concussion management using a clinical algorithm. Mild impairments were managed conservatively (over-the-counter analgesics, warm/cold compresses, and/or neck stretching exercises). Patients with moderate or greater impairments were assessed by a physical therapist at the initial clinic encounter and given specific cervical interventions.ResultsPhysician-observed cervical findings had moderate agreement with self-reported function on the NDI (? = 0.414, p < 0.001). Patients with cervical findings reported greater symptom severity on the PCSS (37.9 ± 22.1 vs 30.8 ± 20.2, p = 0.011); however, there was no significant difference in recovery time (34.44 ± 33.2 vs 34.81 ± 39.0 days, p = 0.933) or incidence of persistent symptoms (39% vs 35%, p = 0.511).ConclusionsIn this group of adolescents, cervical impairment managed early after concussion was not associated with prolonged recovery. Early management of cervical impairments may reduce the development of PPCS. Prospective evaluation is warranted.

Role of Neuromuscular Electrical Stimulation in Bell’s Palsy: A Case Study of Three Patients

Bell's palsy is an idiopathic, unilateral facial paralysis, caused by a malfunction anywhere along the facial nerve's peripheral portion, from the pons distally. Bell's palsy is treated by removing the cause of nerve injury, strengthening the face muscles, and restoring facial function. Physical therapy in the form of neuromuscular electrical stimulation (NMES), massage and facial exercises is used as adjuvant to hasten recovery. The aim of this study is to access of role of neuromuscular electrical stimulation (NMES) treatment in Bell’s palsy patients. A detailed neurological assessment of three patients was done with emphasis on facial muscles and severity of paralysis was graded according to House Brackmann scale (HBS). Conventional physiotherapy was given in the form of electrical stimulation, facial massage, exercises and functional re-education on a daily basis. Patients were assessed at weekly and 1months after the treatment. They experienced complete recovery within 1month follow-up, no recurrence was observed and all patients have normal facial movement. Physiotherapy in the form of NMES and facial exercises has a effective role in the early management of Bell’s palsy. Keywords: Bell’s palsy; neuromuscular electrical stimulation; House Brackmann scale; physiotherapy.

Spectrum of dermatophytes in a tertiary care centre in Northern India

Dermatophytes is major public health challenge in many parts of the world, mainly in developing countries due to poor housing facilities, high population per capita, and poor sanitary conditions. Early diagnosis and identification is must for preventing and early treatment of dermatophytosis. Also, some studies suggest that in prepubescent children there is an inadequate amount of fungi inhibiting fatty acids synthesized predisposing them to dermatophytic infections. Reduction in the synthesis of these fungistatic triglycerides in sebum premenopausal women is also seen predisposing them to infection by dermatophytes. However, low socioeconomic status along with illiteracy and overpopulation has been a main predisposing factor to dermatophytic infections in developing parts of the world. The incidence also been increased due to the rise in the number of immunocompromised patients and considerable use of broad-spectrum antibioticsEarly finding of infection is must for prevention and early management of dermatophytosis. Dermatophytes enter keratinized tissue via keratinases, which produce a dermal inflammatory response causing burning, itching and rednessTo determine prevalence of species of dermatophytes. This Study was conducted on 334 samples i.e. skin scrapings, nail clippings, and hair for fungal culture in the Mycology laboratory over a period of one year extending from December 2019 to December 2020. Specimens were cultured on modified Sabouraud's dextrose agar media containing antibiotics and incubated at 25°C and 37°C for a period of 4 weeks. Isolation and identification of various species of dermatophytes were done. A cross-sectional study was conducted on patients who came to our hospital in the department of dermatology or were referred to the department of microbiology over a period of one year extending from November 2019 to November 2020. Microbiological tests of suspected patients included potassium hydroxide (KOH) mount and fungal culture examination. Cases with culture-positive results were correlated with clinical diagnosis. In the study total of 334 samples (skin scrapings, nail clippings, and hair) were received for fungal culture in the microbiology laboratory during the study period. Samples obtained were cultured on modified Sabouraud's dextrose agar (SDA) media containing antibiotics and incubated at 25°C and 37°C for a period of 4 weeks. Species identification was performed based on colony's morphology, finding of the teased mount by using lactophenol cotton blue stain (LCB) and slide culture, and also with urea hydrolysis test as seen.The Study was performed on 334 samples received from the department of dermatology for fungal culture. Fungal elements were seen in 31% of cases and were isolated in 30% of cases. In these culture-positive cases, dermatophytes were reported in 90% cases, Candida species in 4%, and another fungus was reported in 6% cases. Trichophyton species is most commonly isolated (27.6%). Microsporum and Epidermophyton species were isolated in 5.1% cases. T. mentagrophytes was the most common fungal isolate among all the culture-positive cases.