The Prevalence and Epidemiological Features of Ischaemic Heart Disease in Sri Lanka.

Background: There is limited evidence on the prevalence of ischaemic heart disease (IHD) and its association with risk factors and socioeconomic status (SES) in low- and middle-income countries (LMICs). Given the relatively high levels of access to healthcare in Sri Lanka, the association of IHD with SES may be different from that observed in other LMICs. Objectives: To estimate the prevalence of IHD in Sri Lanka, determine its associated risk factors and its association with SES. Methods: We analysed data from 6,513 adults aged ≥18 years examined in the 2018/19 Sri Lanka Health and Ageing Study. We used the Rose angina questionnaire to classify participants as having angina (Angina+) and used self-report or medical records to identify participants with a history of IHD (History+). The association of Angina+ and History+ with age, ethnicity, sector of residence, education level, household SES wealth quintile, area SES wealth quintile, hypertension, diabetes, smoking, total cholesterol, cholesterol-to-HDL ratio, waist-to-hip ratio and body mass index were analysed in unadjusted and adjusted models. Additional analyses were performed to investigate sensitivity to correction for missing data and to benchmark estimates against evidence from other studies. Conclusions: We estimated prevalence of History+ of 3.9% (95% CI 3.3%-4.4%) and Angina+ of 3.0% (95% CI 2.4%-3.5%) in adults aged 18 years and over. The prevalence of Angina+ was higher in women than men (3.9% vs. 1.9%, p < 0.001) whilst prevalence of History+ was lower (3.8% vs. 4.0%, p = 0.8), which may suggest a higher rate of undiagnosed IHD in women. A history of IHD was strongly associated with age, hypertension and diabetes status even after adjusting for sociodemographic factors. Though the prevalence of History+ was higher in the most developed area SES tertile and urban areas, History+ was also associated with less education but not household SES, consistent with patterns emerging from other LMICs.

Estimation of body weight using anthropometric parameters in Sri Lankan hospitalized adult patients.

Body weight is an important clinical parameter for accurate dosing of drugs with a narrow therapeutic window, However, it is difficult to measure the body weight of a patient if they cannot stand on a scale. There are several anthropometrics-based equations to estimate the body weight, but most of these are derived from white Caucasian populations and are not validated for South Asians. This study aimed to validate existing anthropometrics-based weight estimation equations and develop a new equation for the same purpose for Sri Lankan adults. This prospective study was conducted at the National Hospital of Sri Lanka over a 6-month period, split into a development and a validation phase. During the development phase, estimated body weight of patients by doctors and nurses and patients themselves were noted and compared against their actual body weight. In addition, 13 anthropometric measurements were taken, which were used to validate 12 anthropometrics-based equations to estimate body weight described in literature previously. Two new gender specific regression models to estimate the body weight in the local population was also derived and validated. A total of 502 (males = 249) and 217 (males = 108) patients were recruited for the development and validation phases respectively. Both doctors and patients had comparable accuracy in predicting body weight (p>0.05). All anthropometric based equations were significantly correlated with actual body weight (correlation coefficients: 0.741-0.869), and the new equations derived from the local data performed similarly to the best performing equation identified from the literature during validation phase. However, even the best of these equations could not outperform patient/physician estimates. When the patient weight cannot be measured, an estimate by the patient or the doctor may be the best substitute.

Prevalence of diabetes and pre-diabetes in Sri Lanka: a new global hotspot-estimates from the Sri Lanka Health and Ageing Survey 2018/2019.

INTRODUCTION: This study's objective was to produce robust, comparable estimates of the prevalence of diabetes and pre-diabetes in the Sri Lankan adult population, where previous studies suggest the highest prevalence in South Asia. RESEARCH DESIGN AND METHODS: We used data on 6661 adults from the nationally representative 2018/2019 first wave of the Sri Lanka Health and Ageing Study (SLHAS). We classified glycemic status based on previous diabetes diagnosis, and either fasting plasma glucose (FPG), or FPG and 2-hour plasma glucose (2-h PG). We estimated crude and age-standardized prevalence of pre-diabetes and diabetes and by major individual characteristics weighting the data to account for study design and subject participation. RESULTS: Crude prevalence of diabetes in adults was 23.0% (95% CI 21.2% to 24.7%) using both 2-h PG and FPG, and age-standardized prevalence was 21.8% (95% CI 20.1% to 23.5%). Using only FPG, prevalence was 18.5% (95% CI 7.1% to 19.8%). Previously diagnosed prevalence was 14.3% (95% CI 13.1% to 15.5%) of all adults. The prevalence of pre-diabetes was 30.5% (95% CI 28.2% to 32.7%). Diabetes prevalence increased with age until ages ≥70 years and was more prevalent in female, urban, more affluent, and Muslim adults. Diabetes and pre-diabetes prevalence increased with body mass index (BMI) but was as high as 21% and 29%, respectively, in those of normal weight. CONCLUSIONS: Study limitations included using only a single visit to assess diabetes, relying on self-reported fasting times, and unavailability of glycated hemoglobin for most participants. Our results indicate that Sri Lanka has a very high diabetes prevalence, significantly higher than previous estimates of 8%-15% and higher than current global estimates for any other Asian country. Our results have implications for other populations of South Asian origin, and the high prevalence of diabetes and dysglycemia at normal body weight indicates the need for further research to understand the underlying drivers.

Prevalence and Associations of Hypertension in Sri Lankan Adults: Estimates from the SLHAS 2018-19 Survey Using JNC7 and ACC/AHA 2017 Guidelines.

Background: Sri Lanka lacks robust estimates of hypertension (HTN) prevalence owing to few national studies, hindering optimization of control strategies. Evidence on how the revised 2017 American College of Cardiology/American Heart Association (ACC/AHA) HTN definition affects prevalence in low- and middle-income countries (LMICs) is also limited. Objectives: To make robust estimates of HTN prevalence in the Sri Lankan adult population, and to assess impact of the ACC/AHA 2017 definitions. Methods: Data were sourced from the 2018-2019 first wave of the Sri Lanka Health and Ageing Study (SLHAS), a nationally representative longitudinal study of the noninstitutionalized adult population. After excluding those with missing data and aged <18 years, 6,342 participants (95.1%) were included in the analysis. HTN was defined using either the traditional threshold of systolic BP (SBP) ≥140 mmHg or a diastolic BP (DBP) ≥90 mmHg, or the ACC/AHA 2017 threshold of SBP ≥130 mmHg or DBP ≥80 mmHg, or if taking antihypertensive medication. Results: Estimated prevalence of HTN in all Sri Lankan adults was 28.2% using the traditional definition, and it doubled to 51.3% when applying the ACC/AHA 2017 definition. Of those classified as hypertensive according to the older and ACC/AHA 2017 definitions, 53.4% and 31.2%, respectively, were previously diagnosed. Of the 23.2% of adults reclassified as hypertensive by the ACC/AHA 2017 definition, 16.6% had a history of CVD or diabetes. Increased prevalence was associated with urban residence, socioeconomic status, obesity, and Muslim ethnicity. Prevalence increased with age, but the increase was steeper in women from their 30s. Conclusions: Nearly one in three adult Sri Lankans are hypertensive, requiring antihypertensive treatment. Applying the ACC/AHA 2017 definitions almost doubles numbers, but many of those reclassified would require treatment under recent WHO guidelines. Study findings also suggest that design effects in HTN surveys may be higher than usually assumed.

Attitudes on euthanasia among medical students and doctors in Sri Lanka: a cross sectional study.

BACKGROUND: Euthanasia is a topic of intense ethical debate and it is illegal in most countries at present, including Sri Lanka. The aim of this descriptive cross-sectional study of medical students and practicing doctors was to explore the acceptance of euthanasia and physician assisted suicide (PAS), and factors influencing this opinion. METHODS: A customised online questionnaire which explored opinions on euthanasia was administered to first and final year medical undergraduates in University of Colombo and practicing doctors with more than 5 years of work experience at The National Hospital of Sri Lanka. Attitudes on euthanasia and PAS were also assessed with the attitudes towards euthanasia (ATE) Scale, which is a 10-item questionnaire. RESULTS: A total of 425 individuals responded (males: 178, 42%, age: median - 27 years), which included 143 (33.6%) first-year medical undergraduates, 141 (33.2%) final-year medical undergraduates and 141 (33.2%) practicing doctors. More participants (200, 47.1%) favoured legalizing euthanasia than those directly opposing it (110, 25.9%), but a significant proportion (27%) remained undecided. The mean scores of ATE questionnaire from the whole sample were generally unfavourable towards euthanasia/PAS. Accepting euthanasia as an option for oneself (p = < 0.001) was the strongest predictor of favouring euthanasia/PAS or supporting its legalization. CONCLUSION: In this cross-sectional survey, more respondents supported legalisation of euthanasia in Sri Lanka than those openly opposing it. Yet, a significant minority that responded as "undecided" for legalisation, were more likely to have unfavourable ATE.

Outcomes of stroke patients undergoing thrombolysis in Sri Lanka; an observational prospective study from a low-middle income country.

BACKGROUND: Stroke related deaths are relatively higher in low- and middle-income countries where only a fraction of eligible patients undergo thrombolysis. There is also limited evidence on post-thrombolysis outcomes of patients from Asian countries in these income bands. METHODS: This is a single center prospective observational study of a patient cohort with acute ischaemic stroke, undergoing thrombolysis with alteplase (low and standard dose), over a 24-month period in 2019/2020. Modified Rankin scale (mRS) for dependency at 3 months (primary outcome), duration of hospital stay, incidence of symptomatic intracranial haemorrhages and all-cause mortality at 3 months (secondary outcomes) were recorded. Demographic, clinical and treatment related factors associated with these outcomes were explored. RESULTS: Eighty-nine patients (males - 61, 69%, mean age: 60 years ±12.18) were recruited. Time from symptom onset to reperfusion was 174 min ± 56.50. Fifty-one patients were independent according to mRS, 11 (12.4%) patients died, and 11 (12.5%) developed symptomatic intracranial haemorrhages by 3 months. Functional independence at 3 months was independently associated with National Institutes of Health Stroke Scale (NIHSS) on admission (p < 0.05). Thrombolysis with low dose alteplase did not lead to better or worse outcomes compared to standard dose. CONCLUSIONS: On admission NIHSS is predictive of functional independence at 3 months post-thrombolysis. Low dose alteplase may be as efficacious as standard dose alteplase with associated cost savings, but this needs to be confirmed by a prospective clinical trial for the Sri Lankan population.

Anterior clinoid mucocoele presenting as progressive fluctuating visual loss mimicking inflammatory optic neuropathy.

Optic nerve lesions usually produce monocular visual loss, and in compressive optic neuropathies, gradual vision loss is the more usual presentation. Mucocele in the anterior clinoid process is a rare cause of compressive optic neuropathy that can lead to monocular visual loss. A 19-year-old Sri Lankan girl presented with progressive, painless fluctuating right-sided monocular visual loss over 1-year duration. On presentation, right side visual acuity was 6/60 and fundoscopy revealed pale disk on the right side. Ocular tomography showed right-sided temporal retinal thinning. Magnetic resonance imaging revealed right anterior clinoid process mucocele causing compression and kinking of the right optic nerve. Because the right side, optic disc was already pale and retina was thinned, we decided to manage conservatively. Early imaging in compressive optic neuropathy is useful for the diagnosis and early neurosurgical intervention. Delaying the diagnosis can lead to permanent visual loss.

Elevated nitric oxide and carbon monoxide concentration in nasal-paranasal sinus air as a diagnostic tool of migraine: a case - control study.

BACKGROUND: A recent study showed that 60-s paranasal air suction results in an immediate pain relief in acute migraine. This is the study to assess the Nitric Oxide (NO) and Carbon Monoxide (CO) concentration in nasal-paranasal sinus air of migraine patients and to compare it with healthy controls. METHODOLOGY: The NO and CO levels of air sucked out from nasal-paranasal sinuses of 20 migraine adolescent and young adults among school students, aged 16 -19 years, and 22 healthy similar aged school students as controls were measured as key responses using a portable NO and a portable CO analyzer. RESULTS: Patients had comparatively high values compared to the controls for paranasal NO (both left and right sides), paranasal CO (both left and right sides), Fraction Exhaled NO (FeNO) and Fraction Exhaled CO (FeCO). Patients had median paranasal NO contents of 132.5 ppb and 154 ppb on left and right sides respectively compared to 36 ppb and 34.5 ppb corresponding values in controls (P <  0.0001). Similar pattern was observed with paranasal CO (P <  0.0001). FeNO and FeCO content were also higher in patients (P <  0.0001). Receiver characteristic operating curves of all gas measurements showed that they all could classify patients and controls effectively and NO was the most effective followed by paranasal CO. After air suction, the mean pain scores of general headache and tenderness dropped by a very large margin in migraine patients (P <  0.0001). CONCLUSIONS: Suctioned out high nasal-paranasal sinus NO and CO levels can be used to distinguish migraine patients from healthy subjects. In fact, suctioned out paranasal NO measurements of both sides with a cutoff point of 50 ppb provided a perfect classification of patients and controls. Increased sinus NO and CO during acute episode of migraine is an observation we had and we agree that further studies are needed to conclude that NO and CO can be a causative molecule for migraine headache. TRAIL REGISTRATION: Clinical Trial Government Identification Number - 1548/2016. Ethical Clearance Granted Institute - Medical Research Institute, Colombo, Sri Lanka (No 38/2016). Sri Lanka Clinical Trial Registration number: SLCTR/ 2017/018 (29/06/2017). Approval Granting Organization to use the device in the clinical trial- National Medicines Regulatory Authority Sri Lanka (16/06/2018), The device won award at Geneva international inventers exhibition in 2016 and President award in 2018 in Sri Lanka. It is a patented device in Sri Lanka and patent number was SLKP/1/18295. All methods were carried out in accordance with CONSORT 2010 guidelines.

Effectiveness of paranasal air suction on acute migraine using portable air sucker - a randomized, double blind study.

BACKGROUND: Migraine is a primary headache disorder and is the most common disabling primary headache disorder that occurs in children and adolescents. A recent study showed that paranasal air suction can provide relief to migraine headache. However, in order to get the maximum benefit out of it, an easy to use effective air sucker should be available. Aiming to fulfil the above requirement, a randomized, double blind control clinical trial was conducted to investigate the efficacy of a recently developed low-pressure portable air sucker. METHODS: Eighty-six Sri Lankan school children of age 16-19 years with migraine were enrolled for the study. They were randomly allocated into two groups, and one group was subjected to six intermittent ten-second paranasal air suctions using the portable air sucker for 120 s. The other group was subjected to placebo air suction (no paranasal air suction). The effect of suction using portable air sucker was the primary objective but side of headache, type of headache, and gender were also studied as source variables. The primary response studied was severity of headache. In addition, left and right supraorbital tenderness, photophobia, phonophobia, numbness over the face and scalp, nausea and generalized tiredness/weakness of the body were studied. The measurements on all those variables were made before and after suction, and the statistical analysis was performed based on before and after differences. As a follow-up, patients were monitored for 24-h period. RESULTS: There was a significant reduction in the severity of headache pain (OR = 25.98, P < 0.0001), which was the primary outcome variable, and other migraine symptoms studied, tenderness (left) (OR = 289.69, P < 0.0001), tenderness (right) (OR > 267.17, P < 0.0001), photophobia (OR = 2115.6, P < 0.0001), phonophobia (OR > 12.62, P < 0.0001) nausea (OR > 515.59, P < 0.0001) and weakness (OR = 549.06, P < 0.0001) except for numbness (OR = 0.747, P = 0.67) in the treatment group compared to the control group 2 min after the suction. These symptoms did not recur within 24-h period and there were no significant side effects recorded during the 24-h observation period. CONCLUSION: This pilot study showed that low-pressure portable air sucker is effective in paranasal air suction, and suction for 120 s using the sucker can provide an immediate relief which can last for more than 24-h period without any side effects. TRAIL REGISTRATION: Clinical Trial Government Identification Number - 1548/2016. Ethical Clearance Granted Institute - Medical Research Institute, Colombo, Sri Lanka (No 38/2016). Sri Lanka Clinical Trial Registration No: SLCTR/2017/018 . Date of registration = 29/ 06/2017. Approval Granting Organization to use the device in the clinical trial- National Medicines Regulatory Authority Sri Lanka (16 Jan 2018), The device won award at Geneva international inventers exhibition in 2016 and President award in 2018 in Sri Lanka. It is a patented device in Sri Lanka and patent number was SLKP/1/18295. All methods were carried out in accordance with CONSORT 2010 guidelines.

Eosinophilic Gastroenteritis with a Relapsing and Remitting Course with Presence of Autoimmune Antibodies.

BACKGROUND: Eosinophilic gastroenteritis (EGE) is an uncommon disease characterized by eosinophilic infiltration of the digestive tract, which occurs due to an uncertain aetiology. Although autoimmune diseases can later present as EGE, it is unusual for EGE to have positive autoimmune antibodies without the presence of an overt autoimmune disease. Case presentation. We report a 38-year-old previously healthy man who presented with abdominal discomfort and loose stools with pleural and peritoneal effusions progressing over several weeks. His investigations revealed severe eosinophilia in peripheral blood and ascitic fluid, and a laparoscopic full-thickness biopsy from the ileum demonstrated infiltration of eosinophils in all three layers of the intestine. There were no clinical features or investigations suggestive of parasitic disease, other diseases associated with eosinophilia, or autoimmune disease. Further investigations showed a highly positive ANA, positive p-ANCA, but did not meet the criteria to diagnose a specific autoimmune disease. The eosinophilia responded to an elimination diet with gradual resolution of eosinophilia and effusions, and once it reappeared after introduction of a normal diet. CONCLUSION: EGE presenting as peripheral blood and ascitic fluid eosinophilia with the presence of pleural and/or peritoneal effusions is uncommon. Eosinophilic gastroenteritis can be associated with autoantibody positivity without any evidence of overt autoimmune disease manifestations. Elimination diet can be used as a potential option to prevent recurrences of EGE.

Artery of Percheron infarction presenting as nuclear third nerve palsy and transient loss of consciousness: a case report.

BACKGROUND: Thalamic blood supply consists of four major vascular territories. Out of them paramedian arteries supply ipsilateral paramedian thalami and occasionally rostral mid brain. Rarely both paramedian arteries arise from a common trunk that arise from P1 segment of one sided posterior cerebral artery (PCA). This is usually due to hypoplastic or absent other P1 and this common trunk is termed Artery of Percheron (AOP). Its prevalence is in the range of 7-11% among the general population and AOP infarcts account in an average of 0.4-0.5% of ischemic strokes. Clinical presentation of AOP infarction is characterized by impaired arousal and memory, language impairment and vertical gaze palsy. It also can present with cerebellar signs, hemi paresis and hemi sensory loss. We herein present a case of AOP infarction presenting as transient loss of consciousness and nuclear third nerve palsy. CASE PRESENTATION: A 51 year old previously healthy male, was brought to us, with a Glasgow coma scale (GCS) of 7/15. GCS improved to 11/15 by the next day, however he had a persisting expressive aphasia. Right sided nuclear third nerve palsy was apparent with the improvement of GCS. He did not have pyramidal or cerebellar signs. Thrombolysis was not offered as the therapeutic window was exceeded by the time of diagnosis. Diagnosis was made using magnetic resonance imaging (MRI) that was done after the initial normal non-contrast computer tomography (NCCT) brain. He was enrolled in stroke rehabilitation. Aspirin and atorvastatin was started for the secondary prevention of stroke. He achieved independency of advanced daily living by 1 month, however could not achieve full recovery to be employed as a taxi driver. CONCLUSIONS: Because of the rarity and varied clinical presentation with altered levels of consciousness, AOP infarcts are easily overlooked as a stroke leading to delayed diagnosis. Timely diagnosis can prevent unnecessary investigations and the patient will be benefitted by early revascularization. As it is seldom reported, case reports remain a valuable source of improving awareness among physicians about this clinical entity.

Coexistence of mycobacterial infections - Mycobacterium tuberculosis and Mycobacterium leprae - in Sri Lanka: a case series.

BACKGROUND: Leprosy is one of the oldest mycobacterial infections and tuberculosis is the most common mycobacterial infection with a higher degree of infectivity than leprosy. Although both diseases are prevalent in clusters in developing countries, simultaneous occurrence of them in an individual is a rare entity, even in an endemic setting. CASE PRESENTATION: We describe six cases of tuberculosis and leprosy coinfection: a 57-year-old Sinhalese woman, a 47-year-old Tamil woman, a 72-year-old Tamil man, a 59-year-old Sinhalese man, a 54-year-old Sinhalese man, and a 50-year-old Sinhalese man. In this case series, five patients had lepromatous leprosy and the majority of patients were men. Three patients were detected to have tuberculosis at the outset of treatment of leprosy, while two developed tuberculosis later and one had extrapulmonary tuberculosis 5 years before the diagnosis of leprosy. The latter developed pulmonary tuberculosis as a reactivation while on treatment for leprosy. A majority of our patients with pulmonary tuberculosis had positive Mantoux test, high erythrocyte sedimentation rate, radiological evidence, and acid-fast bacilli in sputum. Human immunodeficiency virus and diabetes were detected in one patient. One patient had rifampicin-resistant tuberculosis, while she was on monthly rifampicin therapy for leprosy. CONCLUSION: An immunocompromised status, such as human immunodeficiency virus infection, diabetes, and immunosuppressive drugs, are risk factors for tuberculosis infection. The use of steroids in the treatment of leprosy may increase the susceptibility to develop tuberculosis. Development of rifampicin resistance secondary to monthly rifampicin in leprosy is a major concern in treating patients coinfected with tuberculosis. Despite the paucity of reports of coinfection, it is advisable to screen for tuberculosis in patients with leprosy, especially if there are respiratory or constitutional symptoms, high erythrocyte sedimentation rate, and abnormal chest X-ray. The fact is that positive Mantoux and QuantiFERON Gold tests and presence of acid-fast bacilli in sputum are misleading, chest X-ray evidence of active tuberculosis and positive tuberculosis cultures are important diagnostic clues for active tuberculosis infection in a patient with leprosy. This is important to avoid monthly rifampicin in patients with suspected coinfections, which may lead to development of drug resistance to tuberculosis treatment. Whether prolonged steroid therapy in leprosy is a risk factor for development of tuberculosis is still controversial.

Systemic lupus erythematosus presenting with status epilepticus and acute cardiomyopathy with acute heart failure: case report.

INTRODUCTION: Systemic lupus erythematosus is a connective tissue disorder, which causes complex multi organ involvement. Neurological and cardiac manifestations have been well noted but complications such as status epilepticus and acute myocarditis with heart failure at presentation remains uncommon. CASE DESCRIPTION: A 15-year-old, previously healthy, South Asian, Sri Lankan female presented with status epilepticus and the seizures only responded to intravenous midazolam and thiopentone sodium. On the fourth day, she developed tachycardia and shortness of breath and was found to have cardiomyopathy with heart failure with an ejection fraction 40%. Along with a positive urinary sediment, a positive ANA with a very high level of ds-DNA and low C3 and C4 levels confirmed our suspicion of systemic lupus erythematosus. DISCUSSION AND EVALUATION: Systemic lupus erythematosus presents in a variety of clinical presentations and the spectrum may range from unique to ubiquitous. Clinicians should have a high index of suspicion specially when encountering atypical presentations with multi-organ involvement, especially when patients tend to be young females. Status epilepticus and myocarditis are uncommon manifestations of systemic lupus erythematosus, and should be appreciated early, as if inappropriately managed would have a deleterious impact on mortality and morbidity.

Disseminated Mycobacterium simiae infection in a patient with adult-onset immunodeficiency due to anti-interferon-gamma antibodies - a case report.

BACKGROUND: Mycobacterial species other than Mycobacterium tuberculosis and Mycobacterium leprae are generally free-living organisms and Mycobacterium simiae is one of the slowest growing Non-tuberculous mycobacteria. This is the first case report of Mycobacterium simiae infection in Sri Lanka and only very few cases with extrapulmonary manifestation reported in the literature. CASE PRESENTATION: A 24-year-old, previously healthy Sri Lankan male presented with generalized lymphadenopathy with discharging sinuses, evening pyrexia, weight loss, poor appetite and splenomegaly. Lymph node biopsies showed sheets of macrophages packed with organisms in the absence of granulomata. Ziehl Neelsen, Wade Fite and Giemsa stains revealed numerous red coloured acid-fast bacilli within foamy histiocytes. Slit skin smear for leprosy was negative and tuberculosis, fungal and bacterial cultures of the lymph node and bone marrow did not reveal any growth. Later he developed watery diarrhea and colonoscopy revealed multiple small polyps and ulcers throughout the colon extending up to the ileum, Which was confirmed to be due to cytomegalovirus confirmed by PCR and successfully treated with ganciclovir. Positron emission tomography scan guided biopsies of the gut and lymph nodes confirmed presence of mycobacterial spindle cell pseudo-tumours and PCR assays revealed positive HSP65. The culture grew Mycobacterium Simiae. Flow cytometry analysis on patient's blood showed extremely low T and B cell counts and immunofixation revealed low immunoglobulin levels. His condition was later diagnosed as adult onset immunodeficiency due to anti- interferon - gamma autoantibodies. He was initially commenced on empirical anti-TB treatment with atypical mycobacterial coverage. He is currently on a combination of daily clarithromycin, ciprofloxacin, linezolid with monthly 2 g/kg/intravenous immunoglobulin to which, he had a remarkable clinical response with complete resolution of lymphadenopathy and healing of sinuses. CONCLUSIONS: This infection is considered to be restricted to certain geographic areas such as mainly Iran, Cuba, Israel and Arizona and this is the first case report from Sri lanka. Even though the infection is mostly seen in the elderly patients, our patient was only 24 years old. In the literature pulmonary involvement was common presentation, but in this case the patient had generalized lymphadenopathy and colonic involvement without pulmonary involvement.

Pulmonary Onset of Adult Onset Still's Disease Complicated with Kikuchi Lymphadenitis.

BACKGROUND: Adult onset Still's disease (AOSD) is a rare inflammatory disorder with a variety of clinical presentations. Even though pneumonitis and pleurisy are known to occur in AOSD, pulmonary onset presentations are exceedingly rare. Case Presentation. We present a 40-year-old male, presenting with fever and bilateral alveolar shadows with pleural effusions mimicking community-acquired severe pneumonia. He was initially treated as severe pneumonia with poor response to broad spectrum antibiotics. Subsequently, he was managed as AOSD-induced pneumonitis, as he fulfilled Yamaguchi criteria. Few weeks later, he developed macular rash and arthralgia with generalized lymphadenopathy with lymph node histology, showing Kikuchi lymphadenitis. He responded well to steroids and had a complete recovery. CONCLUSION: Non-infective causes of pneumonitis should be suspected in the setting of poorly resolving pneumonias, especially when microbiological and serological investigations does not support an infective etiology. Presence of systemic symptoms with arthralgia, rash, and disproportionately elevated ferritin level supports the diagnosis of AOSD. Kikuchi lymphadenitis is a reported association with AOSD, and there could be a causal link between the two disorders.

Frequently Relapsing Anterior Nodular Scleritis as the Initial Presentation of a Lethal Systemic Infection: Disseminated Tuberculosis with HIV Coinfection.

BACKGROUND: Scleritis is a painful inflammatory ocular disease often associated with an underlying systemic illness mostly having an autoimmune aetiology. Tuberculosis usually presents as pulmonary infection, and the ocular presentation is considered to be rare. Case presentation. We present a male who developed prolonged pyrexia following systemic steroids while being investigated for a frequently relapsing anterior scleritis. Biopsy of sclera demonstrated acid-fast bacilli, and histology of sclera and lymph node showed granulomatous inflammation with caseation. Contrast CT demonstrated mediastinal and visceral lymphadenopathy with pulmonary changes suggesting disseminated tuberculosis. Later, Western blot confirmed coinfection with HIV with a CD4 count of 71 cells/mm3. He was treated with antituberculous medications, and then HAART was initiated within two months. He showed good response and showed a partial resolution of scleritis at the end of two months. CONCLUSION: Tuberculosis tends to occur in unusual sites when coinfected with HIV. Scleritis is a rare extrapulmonary manifestation of tuberculosis. High degree of suspicion is critical in making diagnosis and commencing early treatment.

Paranasal sinus air suction for immediate pain relief of acute migraine - a randomized, double blind pilot study.

BACKGROUND: Migraine is a primary headache disorder, which cause significant disability in adolescence. This double blind, randomized clinical trial assessed the immediate effects of suction of paranasal sinus air during an acute migraine episode. METHODS: A randomized, double blind study was conducted with 56 selected Sri Lankan school children of 16-19 years of age. Participants who met International Headache Society criteria for migraine (with or without aura) were included in the study. Subjects were randomly allocated into 2 groups where one group was subjected to three intermittent 10 sec paranasal air suctions with a ten sec suction free interval between two suctions for each nostril and the other group was subjected to placebo air suction (no paranasal air suction) in similar arrangement. Severity of headache and sub-orbital tenderness before and after suction were recorded using standard pain rating scale. RESULTS: After dropouts, treated and placebo groups consisted of 27 and 23 subjects respectively. The mean headache pain score drop in the treated group was significantly higher compared to that of the control group. Moreover, there was a difference in the treatment response between the types of headache (with or without aura). With respect to tenderness there was a statistically significant drop in the treated group compared to the control. In general, airflow rates in left and right nostrils were different in these subjects. However such difference was not seen in the tenderness on two sides. Nevertheless it was revealed that airflow rate has a slight negative correlation with the tenderness irrespective of the side. CONCLUSION: Sixty-second paranasal air suction can provide an immediate pain relief for acute migraine in adolescents. We did not assess pain outcomes beyond 60 s, but the initial responses suggests the need to further study the efficacy of paranasal suction in migraine. A further study is suggested to evaluate the acute effects, efficacy and side effects of paranasal air suction using follow up over a prolong period. TRIAL REGISTRATION: Sri Lanka Clinical Trials Registry SLCTR/2017/018 , 29 Jun 2017. Retrospectively registered.

A seven-year retrospective analysis of patch test data in a cohort of patients with contact dermatitis in Sri Lanka.

BACKGROUND: Patch testing with a baseline series is a common tool employed when the sensitizing agent in contact dermatitis is unclear. However, for Asian countries, there are no locally validated baseline series to utilize in screening. METHODS: We completed a retrospective analysis of all patients that had undergone patch testing with the European Baseline series, Shoe Series or Comprehensive International Baseline series, over 7 years from 2012 to 2018 in a tertiary care reference dermatology clinic in Sri Lanka to evaluate the suitability of these investigations to identify causes for contact dermatitis in the local study population. RESULTS: Out of 438 patients tested, 239 (54.8%) reacted to at least one substance in the series. The Shoe Series was significantly more likely to yield a positive result than the European Baseline Series (70.2% vs 46.9%, p < 0.05). The top three sensitizers identified by all series were nickel sulfate (16%, 70/438), p-phenylenediamine (12.3%, 54/438) and 2-mercaptobenzothiazole or mercapto mix (10.5%, 46/438). CONCLUSION: Shoe series has a comparatively high yield in the local population compared to European Baseline series. Since little less than half of the study population did not have any reactivity to any of the allergens tested it is important to develop or modify and validate a locally relevant, more suitable baseline series which is based on the Shoe Series in Sri Lanka. This is further evidence for the continuously changing nature of allergens in the environment and the need to modify existing patch testing standards accordingly.

Scleredema associated with immunoglobulin A-κ smoldering myeloma: a case report and review of the literature.

BACKGROUND: Scleredema is a rare sclerodermoid skin condition characterized by diffuse symmetrical thickening of the upper part of the body. Its association with monoclonal gammopathy and myeloma was recently described; very few cases have been reported to date. CASE PRESENTATION: A 66-year-old Sri Lankan woman who had been followed in a dermatology unit for 34 years with diffuse systemic sclerosis presented with an acute exacerbation of the skin disease. Absence of Raynaud's phenomenon; sclerodactyly; characteristic lung, gastrointestinal, and cardiac involvement of systemic sclerosis; and repeatedly negative antinuclear antibodies test results led to reevaluation for the possibility of scleredema. Skin biopsies from four body sites showed normal epidermis and thickened reticular dermis with swollen collagen bundles separated from one another by clear spaces, resulting in fenestration. The skin appendages were not atrophied or bound down. Alcian blue staining showed interstitial mucin deposition. Serum protein electrophoresis demonstrated an abnormal monoclonal band in the ß-region with a paraprotein level of 8.9 g/dl. Immunofixation showed an abnormal band in the γ-region consisting of immunoglobulin A and κ. Bone marrow biopsy revealed abnormal monoclonal plasma cells (15%) with multinuclearity. There was no evidence of end organ damage, and whole-body magnetic resonance imaging did not reveal any evidence of bone involvement. The patient's diagnosis was revised as scleredema type 2 associated with IgA-κ, and she was referred to a hemato-oncologist for chemotherapy, which led to significant improvement in the skin condition. CONCLUSIONS: Scleredema is a rare disorder that has an enigmatic, rare association with monoclonal gammopathy. Dermatologists should be aware of this rare but important association.