Hemophagocytic lymphohistiocytosis as a rare complication of dengue haemorrhagic fever: a case report.

BACKGROUND: Haemophagocytic lymphohistiocytosis (HLH) is an uncommon systemic inflammatory syndrome that can happen secondary to numerous conditions. It rarely occurs due to dengue infection causing significant mortality and morbidity even with appropriate treatment. The outcome is further poor if the diagnosis of HLH is delayed or left untreated. Therefore, a high degree of clinical suspicion is paramount in diagnosing HLH. CASE PRESENTATION: A 17-year-old Sinhalese boy was admitted to a tertiary care hospital in Sri Lanka with a 4-day history of fever, headache, nausea, vomiting, and diarrhea. He was hemodynamically stable, and the serological investigation confirmed a dengue infection. On the fifth day of fever, he entered the critical phase of dengue infection, confirmed by ultrasound evidence of plasma leaking. However, he had ongoing high fever spikes during the critical phase, and even after the critical phase was over, the fever spikes continued. Simultaneously, hepatosplenomegaly was noticed, and he showed persistent thrombocytopenia, neutropenia, and anemia despite the resolution of the critical phase. Further, the workup revealed a serum ferritin level of > 3000 ng/mL triglyceride level of 314 mg/dL, and the bone marrow biopsy revealed an increased haemophagocytic activity. Secondary HLH was diagnosed on the basis of criteria used in the HLH-2004 trial and successfully managed with intravenous dexamethasone 10 mg/body surface area/day for the first 2 weeks, followed by a tapering regimen over 8 weeks. CONCLUSION: This case emphasizes the need to consider HLH as a potential complication when persistent fever and cytopenias are present after recovering from dengue fever, particularly in patients with unusual clinical features like hepatosplenomegaly. Early recognition and prompt treatment with appropriate immunosuppressive therapy, such as intravenous dexamethasone, can lead to a successful response and good prognosis.

Blood transfusion in severe dengue infection: a case series.

BACKGROUND: Dengue is still a recurrent challenge to the global population, without specific antiviral therapy. Clinical management strategies are aimed to mitigate the deaths. The use of blood products in dengue is recommended mainly in cases of bleeding. CASE PRESENTATION: We prospectively collected data on Sri Lankan dengue cases in the Teaching Hospital, Peradeniya, Sri Lanka from 2017, and selected ten severe cases where blood transfusions were involved in the management. The series comprises seven females and three males, with a median age of 36 years (range 12-53 years). All patients were critically ill at the time of blood transfusion, with dramatic stabilization of vital parameters after the transfusions. Only one patient had detectable bleeding, while five patients had occult blood loss as indicated by dropping hematocrit. Even though four patients had stable hematocrit, they had metabolic acidosis. Two patients had a very high increase of hepatic transaminases along with acidosis. Two patients had myocarditis with dropping hematocrit, suggestive of occult bleeding. CONCLUSIONS: Clinical deterioration despite fluid management commonly occurs due to occult bleeding in dengue infection. Blood transfusion is lifesaving in such cases of blood loss, acidosis, and severe hepatic damage. The mechanism of this effect needs an explanation, such as enhanced oxygen delivery to the tissues and hemostasis to hypothesize a few possibilities.

Pure red cell aplasia secondary to rheumatoid arthritis: a case report.

BACKGROUND: Rheumatoid arthritis is a common autoimmune disease with many extra-articular manifestations. Pure red cell aplasia is a rare manifestation of rheumatoid arthritis and is sparsely documented in the literature, with a variable clinical outcome following immunosuppressive therapy. CASE PRESENTATION: A 63-year-old Sinhalese female presented with transfusion-dependent anemia associated with deforming inflammatory arthritis. She also had leukopenia, right subclavian venous thrombosis, and generalized lymphadenopathy. The diagnosis of rheumatoid arthritis following initial clinical workup and additional blood and bone marrow investigations revealed pure red cell aplasia as a secondary manifestation of rheumatoid arthritis after excluding other secondary causes, such as infections, thymoma, thrombophilic conditions, and hematological malignancy. She responded well to oral prednisolone, cyclosporine A, and hydroxychloroquine, and she attained complete recovery in 2 months. CONCLUSION: Pure red cell aplasia is a disabling illness that may lead to transfusion-dependent anemia, which may occur due to rare extrapulmonary manifestation of rheumatoid arthritis. The diagnosis of pure red cell aplasia secondary to rheumatoid arthritis may be challenging where hematological investigations, including bone marrow biopsy, will aid in the diagnosis, and early diagnosis and treatment will bring about a better outcome.

Kounis Syndrome Secondary to Medicine-Induced Hypersensitivity.

INTRODUCTION: Kounis syndrome is the concurrence of an acute coronary syndrome (ACS) caused by coronary vasospasms, acute myocardial infarctions, or stent thromboses in case of allergic or hypersensitivity reactions. Kounis syndrome is mediated by mast cells that interact with macrophages and T-lymphocytes, causing degranulation and inflammation with cytokine release. It is a life-threatening condition that has many trigger factors and is most commonly caused by medicines. Case Presentation. A 71-year-old male was admitted with a fever of five days' duration associated with cellulitis, for which he had been treated with clindamycin and flucloxacillin before admission. He was a diagnosed patient with hypertension and dyslipidemia five years ago. After taking the antibiotics, he had developed generalized itching followed by urticaria suggesting an allergic reaction. Therefore, he was admitted to the hospital. After admission, he developed an ischaemic-type chest pain associated with autonomic symptoms and shortness of breath. An immediate ECG was taken that showed ST-segment depressions in the chest leads V4-V6, confirmed by a repeat ECG. Troponin I was 8 ng/mL. Acute management of ACS was started, and prednisolone 10 mg daily dose was given. After complete recovery, the patient was discharged with aspirin, clopidogrel, atorvastatin, metoprolol, losartan, isosorbide mononitrate, and nicorandil. Prednisolone 10 mg daily dose was given for five days after discharge. CONCLUSION: In immediate hypersensitivity, with persistent cardiovascular instability, Kounis syndrome should be considered, and an electrocardiogram and other appropriate assessments and treatments should be initiated. Prompt management of the allergic reaction and the ACS is vital for a better outcome of Kounis syndrome.

Too Aggressive Drop in Blood Pressure in a Hypertensive Male Leading to "Man-in-the-Barrel Syndrome".

INTRODUCTION: "Man-in-the-barrel syndrome" is a neurological phenotype with brachial diplegia, normal sensation, and preserved motor function of the lower limb. It has been described in various neuropathological conditions affecting the cerebral hemispheres, pons, upper spinal cord, and peripheral neurons. Severe hypotension leading to watershed infarctions leading to this phenotype has been reported. We describe the first case of "man-in-the-barrel syndrome" in a patient with a precipitous drop in blood pressure following oral antihypertensive medications. Case Presentation. A 75-year-old Sri Lankan male presented following a generalized tonic-clonic seizure to a tertiary care hospital. Upon recovery, he was noted to have severe brachia diplegia affecting shoulder movements with preserved hand muscle power and motor functions of the lower limb. The previous day, he was newly diagnosed with markedly elevated blood pressure without acute end organ involvement. Treatment with three antihypertensives had been initiated. Noncontrast CT of the brain revealed watershed infarctions affecting both cerebral hemispheres. CONCLUSION: It is generally unwise to lower blood pressure very rapidly, as ischemic damage can occur in vascular beds that are habituated to high levels of blood pressure in the brain. Ischemic damage caused by rapid lowering of blood pressure may rarely result in "man-in-the-barrel syndrome" leading to severe functional disability.

Young Male Presenting with an Acute Diarrheal Illness with Unexplained Transudative Ascites: An Atypical Presentation of Appendicular Tuberculosis.

INTRODUCTION: Appendicular tuberculosis is a rare form of extrapulmonary tuberculosis involving the gastrointestinal tract. Diagnosis of appendicular tuberculosis is difficult due to its atypical presentation. Histological confirmation remains the gold standard in diagnosis. Case Presentation. We report a 37-year-old Sri Lankan male presenting with a diarrheal illness with high fever for 8 days in the background of constitutional symptoms for 1-month duration. He was pale and had moderate amount of free fluid in the abdomen. Inflammatory markers were elevated, and CT abdomen revealed a thickened elongated appendix. Diagnostic paracentesis revealed a lymphocytic transudative ascites. A macroscopically minimally inflammed appendix removed at laparotomy and histology confirmed presence of tuberculous granulomata with caseation. He made an uneventful recovery by the anti-tuberculous therapy. CONCLUSION: High degree of suspicion is needed in diagnosis of appendicular tuberculosis due to its nonspecific presentation, and we emphasize the need of histological assessment of the appendix resected for the case of clinical appendicitis, as it may prompt the diagnosis of a rare but treatable case of tuberculosis.

Guillain-Barré syndrome with associated unilateral ptosis without ophthalmoplegia - a rare presentation: a case report and review of the literature.

BACKGROUND: Guillain-Barré syndrome is an acute inflammatory polyradiculoneuropathy. Nearly half of patients with Guillain-Barré syndrome have cranial nerve involvement. However, isolated bilateral ptosis without ophthalmoplegia is a rare manifestation, and isolated unilateral ptosis without ophthalmoplegia in Guillain-Barré syndrome has not previously been reported in the literature. Furthermore, only few cases of Guillain-Barré syndrome with cranial nerve enhancement visualized by gadolinium-enhanced magnetic resonance imaging have previously been reported. We describe the first reported case of unilateral ptosis without ophthalmoplegia in Guillain-Barré syndrome and associated multiple cranial nerve enhancement seen by gadolinium-enhanced magnetic resonance imaging. CASE PRESENTATION: Our patient was a 55-year-old Sinhalese man who was admitted to a tertiary care hospital in Sri Lanka with acute-onset progressive weakness in the lower limbs followed by the upper limbs. He had bilateral symmetrical flaccid quadriparesis with absent reflexes and flexor plantar response. Left-sided isolated partial ptosis without associated ophthalmoplegia was noted with normal pupils. The patient's neurological examination was otherwise normal. A nerve conduction study showed a severe demyelinating type of polyneuropathy. No decremental response to repetitive nerve stimulation was observed, and the result of a single-muscle-fiber electromyogram was negative. A diagnosis of Guillain-Barré syndrome was made, and the patient was treated with intravenous immunoglobulin. His condition gradually deteriorated over the next few days, and he became quadriplegic despite the completion of immunoglobulin therapy. Later he developed multiple cranial nerve palsies, including bi-lateral lower motor neuron type facial nerve palsy, and he required mechanical ventilation. By this time, he had complete left-sided ptosis with a normal right eye. He never developed ophthalmoplegia or ataxia. Magnetic resonance imaging of the brain showed contrast enhancement in the intracranial part of multiple cranial nerve roots and basal leptomeninges. He gradually improved with plasmaparesis, and ptosis was the first to improve. CONCLUSIONS: Even though Guillain-Barré syndrome was recognized a century ago, there are still many unanswered questions about it and its florid presentation. Large-scale studies are needed for better understanding of its pathophysiology and prototypes and to find answers for still-unanswered questions. The clinician must have a high index of suspicion and be familiar with mimics and prototypes to diagnose Guillain-Barré syndrome accurately without delay.

Infective myositis, an uncommon presentation of melioidosis: a case report and review of the literature.

BACKGROUND: Melioidosis is considered endemic in certain areas of the world. Musculoskeletal and soft tissue involvement are relatively uncommon presentations in melioidosis. We present a case of infective myositis in a patient with melioidosis in Sri Lanka, which is not considered an endemic country. Even though multiple cases of melioidosis have been reported with an increasing number in Sri Lanka, infective myositis secondary to melioidosis was not reported previously. CASE PRESENTATION: A 60-year-old Sinhalese man with diabetes presented with fever of 4 months' duration and a limp with a painful lump on the right side of the upper thigh of 2 months' duration. He had been treated in a local hospital for community-acquired pneumonia 3 weeks prior to this admission, for which he had received intravenous meropenem and teicoplanin with intensive care unit admission. He had a 0.5-cm × 0.5-cm tender lump over the right vastus lateralis muscle, and contrast-enhanced computed tomography of the area showed an ill-defined, heterogeneously enhancing, hypodense area involving the vastus lateralis, vastus intermedius, and quadratus femoris, suggestive of infective myositis but without abscess formation. Histopathology of the muscle biopsied from the vastus lateralis showed suppurative inflammation of subcutaneous fat with connective tissue necrosis and muscle infiltrated by lymphocytes. These features are suggestive of infective myositis possibly due to melioidosis. Although the result of a culture taken from the muscle biopsy was negative, the patient's antibody titer was strongly positive for melioidosis. He did not show any other areas with infected foci. He was treated with intravenous meropenem for 2 weeks and responded well. He was discharged with trimethoprim-sulfamethoxazole for 6 months as a maintenance therapy. CONCLUSION: Melioidosis is commonly an undiagnosed disease that has a wide variety of clinical presentations. Myositis in melioidosis is uncommon, and careful evaluation is mandatory to avoid misdiagnosis of this treatable but fatal disease. The clinician should have a high index of clinical suspicion, and further clinical and epidemiological studies are needed to determine the true burden of the disease.

Unilateral pulmonary edema: a case report and review of the literature.

BACKGROUND: Unilateral pulmonary edema is an uncommon condition and is a rare clinical entity that is often misdiagnosed at the initial stages. In a majority of patients it occurs in the upper lobe of the right lung. There are many causes of unilateral pulmonary edema, but the commonest is the presence of a grade 3 mitral regurgitation. Due to its rare presentation, a high index of suspicion is required, and correct management is necessary to reduce the morbidity and mortality. CASE PRESENTATION: We present a case of right-sided unilateral pulmonary edema in an 86-year-old Sinhalese Sri Lankan woman who presented with acute onset dyspnea with cardiogenic shock due to acute non-ST elevation myocardial infarction, complicated with grade 3 mitral regurgitation. She had clinical features of heart failure and pulmonary edema, but a chest X-ray showed unilateral infiltrates only on the right side. Distinguishing pneumonia from pulmonary edema according to chest X-ray findings was a challenge initially, and she was therefore initially treated for both conditions. She had remarkable clinical and radiological improvement after 12 hours of intravenously administered furosemide and glyceryl trinitrate therapy. Her brain natriuretic peptide level was elevated and further supported and confirmed the diagnosis retrospectively. CONCLUSIONS: Unilateral pulmonary edema is a completely reversible condition with good patient outcome if it is suspected early and treated early. Even in the absence of readily available echocardiogram skills, a clinical examination is of paramount importance in making a clinical decision in low-resource settings to reduce mortality.

Value of peripheral blood count for dengue severity prediction.

OBJECTIVE: This retrospective study was conducted in 2017 with the objective of evaluating the value of acute phase peripheral blood parameters in predicting dengue haemorrhagic fever (DHF). Patients, who were admitted to Teaching Hospital Peradeniya between January and August 2017 due to dengue illness, were recruited into this study. RESULTS: A total of 515 patients participated in the study. Among them, 333 were DHF patients while 182 patients were managed as DF. There was a significant difference in mean values of platelets and haemoglobin observed during acute phase in non-leakers compared to the patients who progressed to DHF, while no significant difference was observed for white blood cells, neutrophils, lymphocytes and haematocrit values. A significantly higher mean value was observed in white blood cells and hemoglobin in leakers compared to non-leakers during day 5. Mean day 5 platelet value was significantly lower among leakers compared to non-leakers but no significant difference between haematocrit, neutrophil and lymphocyte values were observed. ROC curve performed for acute phase platelet values and haemoglobin values to gain a predictive value for female and male DHF patients and cut off values with high sensitivity and specificity to predict DHF could be obtained for the platelet count.

A case of acute necrotizing pancreatitis complicated with non ST elevation myocardial infarction.

BACKGROUND: Acute pancreatitis is an inflammatory condition with varying severity and a range of local and systemic complications. Here we report a patient with acute necrotizing pancreatitis complicated with a true non ST elevation myocardial infarction. CASE PRESENTATION: A 58 year old lady was admitted to our unit with acute onset epigastric pain and vomiting for 4 h duration. Following admission she complained of retrosternal tightening type of a chest pain. She had elevated serum amylase and cardiac troponin. Electrocardiogram (ECG) revealed lateral ischaemia. Contrast computerized tomography abdomen revealed acute severe necrotizing pancreatitis. CONCLUSIONS: Nonspecific ECG changes can occur in patients with acute pancreatitis. But the diagnosis of true myocardial infarction in a context of acute pancreatitis using ECGs, 2D echocardiography, cardiac biomarkers and coronary angiograms can be challenging with the choice of revascularization therapy and safety of antiplatelet agents and anticoagulant therapy. Decision making regarding the management of such a patient is also critical.

Translation and validation of patient-practitioner orientation scale in Sri Lanka.

BACKGROUND: Practice of family medicine and patient centeredness does not get the deserved attention in clinical practice and teaching in Sri Lanka. Non-availability of tools for assessment of patient centeredness deters the process of curricular development and research. The Patient-Practitioner Orientation Scale (PPOS) is a self-administered tool that assesses patient-centeredness in both health care professionals and patients. This study has translated and validated the PPOS to Sinhala language. METHODS: Translation and cross-cultural adaptation were carried out using forward and backward translation method. The psychometric properties of a pretested new Sinhala version of PPOS (PPOS-Sinhala) was tested in a convenience sample of 1367 patients and health professionals. Temporal stability was tested in a sub-sample of 140 individuals. The comparability of the PPOS scores and association with sex and level of education with those reported for Western populations were examined to establish construct validity. RESULTS: The sample included 543 medical students, 67 doctors, 335 allied health students and 422 patients. Cronbach's alpha for these groups ranged from 0.48 to 0.53 for sharing, 0.42 to 0.53 for caring, and 0.62 to 0.65 for total score. Intraclass correlation coefficients of 0.56, 0.6, and 0.4 were observed for the Total, Sharing, and Caring sub-scales, respectively. PPOS scores did not vary significantly for men and women. Health professionals exhibited higher scores than patients. Age was negatively associated and education level was positively associated with PPOS. DISCUSSION: PPOS-Sinhala is stable, sufficiently valid and reliable to evaluate patient centeredness among Sinhala speaking health care professionals and patients. Lower internal consistency is found for a few items in the instrument which requires further development. PPOS scores and their correlates for this Sri Lankan population were more similar to that found in other populations in this region than for scores and correlates found in the US.

Epidemiology, clinical manifestations, and long-term outcomes of a major outbreak of chikungunya in a hamlet in sri lanka, in 2007: a longitudinal cohort study.

Chikungunya outbreaks occurred in the central province, Sri Lanka in 2006. This community-based study reports the epidemiology and the natural history of the infection from an affected village. Of the 199 families and 1001 individuals in the village, 159 (80%) and 513 (51%) were affected, respectively, comprising 237 (46%) males with peak incidence at 40-50 years. The acute illness caused polyarthritis in 233 (46%), and of them 230 (98%) progressed to chronic arthritic disability (CAD). Of the CAD patients, 102 (44%) had recovered in 141 days (range 30-210 days) from the disability state whilst 128 (56%) had persisting disability which lasted 12, 24, and 36 months in 41 (17.8%), 22 (9.5%), and 14 (6.1%) individuals, respectively. Carpal tunnel syndrome (CTS) manifested in 110 (21%). Females showed preponderance for complications over males: acute arthritis 147 versus 86, P = 0.001; CAD 136 versus 84, P = 0.029; CTS 88 versus 22, P = 0.001; relapses 105 versus 68, P = 0.001. Chikungunya was highly communicable and caused lasting crippling complications.

Spot urine osmolality/creatinine ratio in healthy humans.

BACKGROUND: Spot urine albumin/creatinine ratio is a reliable estimate of 24-hour urine albumin excretion. In a pilot study, we observed that the spot urine osmolality/creatinine ratio (U(osm)/U(cr)) in healthy adults is reproducible. Therefore, we postulated that U(osm)/U(cr) of a spot urine sample may give an overall estimate of urinary excretion of solutes, renal concentrating ability and body hydration status. METHOD: Early morning spot urine samples were collected from healthy humans, frozen and analyzed in batches to establish spot U(osm)/U(cr) and its variation in relation to sex, age, body weight and height. RESULTS: Two hundred and twenty-nine healthy volunteers participated. They were stratified into seven age groups: (a) 1.5-5, (b) >5-10, (c) >10-20, (d) >20-30, (e) >30-45, (f) >45-60, and (g) >60 years. Fifteen males and 15 females were allocated to each age category. A spot urine sample was collected from all subjects in the morning after the first void and was analyzed for osmolality and creatinine. The influence of age, sex, body weight and height on spot U(osm)/U(cr) was investigated using multiple linear regression. Only height showed a significant correlation (R(2) = 0.02). Further analysis after excluding the 1.5-5 years age group revealed no significant correlation between age, sex, body weight and height and the U(osm)/U(cr) ratio. CONCLUSION: Spot U(osm)/ U(cr) of healthy humans is a consistent index in steady state and needs no correction for sex, age and body weight above the age of 5 years.