Recent publications
This guideline addresses the use of hypoxia-inducible factor prolyl hydroxylase inhibitors (HIF-PHIs) in patients >18 years with chronic kidney disease (CKD) and anemia in South Asia (Bangladesh, Bhutan, Nepal, India, Pakistan, Sri Lanka). It also summarizes recommendations for anemia treatment for individual HIF-PHI molecules under two categories: dialysis-dependent and non-dialysis-dependent CKD patients. The recommendations do not apply to pediatric (≤12 years) and adolescent (12 to 18) patients or those with primary anemia or anemia secondary to other causes such as blood loss, cancer (any type), polycystic kidney disease and infectious diseases.
Dead foetal remains retained within the abdomen following an ectopic pregnancy leads to the formation of lithopedion, a rare entity. A 44-year-old woman sub-fertile for 16 years presented with sub-acute abdominal pain. She had not sought medical help earlier, on account of her religious or cultural beliefs. Imaging revealed a calcified mass containing bones. The differential diagnoses were lithopedion or a fetiform teratoma. During laparotomy, cutting open the mass revealed multiple long, short and flat bones, which together formed an almost complete foetal skeleton. Histopathology revealed an old ectopic gestation sac. This confirmed the diagnosis of lithopedion, estimated to be 32–34 weeks old at the time of foetal demise. This rare case highlights the impact of limited health literacy, along with strong religious and cultural influences, in delaying timely diagnosis and intervention.
Background
This paper reports the first case of basaloid squamous cell carcinoma clinically and radiologically masquerading as a head and neck paraganglioma.
Case presentation
A 66-year-old Sinhalese male with unilateral hearing impairment and 7th–12th (excluding 11th) cranial nerve palsies was diagnosed radiologically with a head and neck paraganglioma by magnetic resonance imaging of the brain, which revealed a hypointense and hyperintense punctate mass centered at the jugular fossa with intracranial extension. The ascending pharyngeal artery, recognized as the major feeder, was embolized by percutaneous embolization following digital subtraction angiography. Gross total resection of the tumor was followed by an uneventful postoperative recovery. Combined immunohistochemistry and histopathological morphology revealed a basaloid squamous cell carcinoma, following which the patient completed radiotherapy and is at 3-month follow-up currently.
Conclusion
This case report discusses the diagnostic pitfalls and management challenges of this rare entity on the basis of prior evidence, as well as a literature review and clinical and surgical analysis.
Synopsis
This report of a dumbbell spinal neurofibroma in pregnancy provides insights into hormonal influence on its occurrence and highlights the use of the seated‐up position for surgery.
PURPOSE
Sepsis is the main cause of nonrelapse mortality, and there are no published data on applicability of supportive care protocols from high-income countries such as Sri Lanka. The aim of the study was to investigate management and mortality of neutropenic episodes among Hemato-Oncology patients.
MATERIALS AND METHODS
Retrospective analysis of clinical characteristics, management, morbidity, and mortality of neutropenic Hemato-Oncology patients presented to the Lanka Hospital Blood Cancer Centre from January 1, 2019 to December 31, 2019 was performed.
RESULTS
A total of 169 neutropenic episodes were identified; 115 (68%) of such episodes were related to chemotherapy. Acute leukemia, lymphoproliferative disorders, and plasma cell disorders accounted for 23%, 69%, and 8% of patients, respectively. The median age of patients who had sepsis was 56 years, whereas that of those who had no sepsis was 53 years ( P = .49). The median time to neutropenia was 9 days for those in the sepsis group compared with 8 days in the group that had no sepsis (0.64). The median neutrophil count in the group that had sepsis was 0.06, whereas it was 0.69 in the group that had no sepsis ( P ≤ .05). The median time to commencement of antibiotics was 20 minutes.
CONCLUSION
To our knowledge, this is the only documented study related to outcome and successful applicability of western supportive care protocols to Sri Lankan patients with neutropenia. In this study, we have shown that neutropenic sepsis can be successfully managed in the setting of limited resources with service development, following guidelines and staff training.
Background: This paper reports the first case of basaloid squamous cell carcinoma clinically and radiologically masquerading as a head and neck paraganglioma (HNPGL).
Case Presentation: A 66-year-old male with unilateral hearing impairment and 7th – 12th (excluding 11th) cranial nerve palsies was diagnosed radiologically with HNPGL by magnetic resonance imaging of the brain, which revealed a hypointense and hyperintense punctate mass centered at the jugular fossa with intracranial extension. The ascending pharyngeal artery, recognized as the major feeder, was embolized by percutaneous embolization following digital subtraction angiography. Gross total resection of the tumor was followed by an uneventful postoperative recovery. Combined immunohistochemistry and histopathological morphology revealed a basaloid squamous cell carcinoma following which the patient completed radiotherapy and is at 3-month follow-up currently.
Conclusion: This case report discusses the diagnostic pitfalls and management challenges of this rare entity based on prior evidence, as well as a literature review and clinical and surgical analysis.
Amongst many interventional procedures performed in a cardiac catheterisation laboratory, the coronary angiography (CAG) is the most frequently performed cardiac interventional procedure. A diagnostic reference level (DRL) is an effective tool to optimise the radiation exposure to patients and staff whilst maintaining the adequate diagnostic image quality. The aim of the study was to establish institutional DRLs for the CAG procedures performed at a selected private hospital in Colombo, Sri Lanka. A total of 325 CAG procedures were selected for this study from two C-arm machines. The institutional DRLs of cumulative dose length product (DAP) and fluoroscopic time for the CAG procedure were calculated. The established institutional DRL for accumulated DAP and fluoroscopic time are 10 610 mGycm2 and 2.31 min, respectively. As this study conducted at only one institute we recommend to develop national DRLs for mostly performing interventional procedures in Sri Lanka by considering all influencing factors to optimise the patient dose.
PURPOSE
There is a significant disparity in global cancer care and outcome between countries. Progress in the treatment of symptomatic plasma cell myeloma (PCM) in high-income countries is not seen in low- and middle-income countries.
MATERIALS AND METHODS
This is was a retrospective cohort study of all patients diagnosed with PCM between May 1, 2013, and September 30, 2021, at the first hemato-oncology center in Sri Lanka. We aimed to provide data on clinicopathologic characteristics, response, and survival estimates.
RESULTS
A total of 79 patients with PCM received first-line therapy during the study period. The median age was 64 years, and approximately one third (33%) of patients were older than 70 years. There were 42 (53%) males and 37 females. Hypercalcemia, renal impairment, anemia, and bone disease were detected in 36.7%, 38%, 72.1%, and 81%, respectively. Thirty-nine, 34, and six patients received a combination of cyclophosphamide, thalidomide, and dexamethasone; bortezomib, thalidomide, and dexamethasone; and other treatments, respectively. The overall response rate (≥ partial response) was approximately 97% for both cyclophosphamide, thalidomide, and dexamethasone and bortezomib, thalidomide, and dexamethasone. Twenty-three (29%) of these patients died during the study period, but only 14 (18%) died due to PCM or associated sepsis. After a median follow-up of 40.6 months (range, 35.2-59.07 months), the median overall survival was 84.2 months (95% CI, 60.87 to not available). The 5-year estimated overall survival was 65%.
CONCLUSION
To our knowledge, this is the only well-characterized study on long-term survival of patients with PCM in Sri Lanka. We have shown that it is possible to successfully apply Western treatment and supportive care protocols to the local population. These published data will help to benchmark and improve the treatment and develop blood cancer care in the local setting.
Bone marrow failure (BMF) in children can be idiopathic (70-80%) or inherited. Haematopoietic stem cell transplantation (HSCT) is the only cure for both causes. Allogeneic HSCT requires a suitable donor. Many children will not have a HLA matched sibling or unrelated donor. A haploidentical donor is available for all children as eaazch parent will have at minimum a 50% HLA match. This report of a 7-year old girl with BMF treated with a haplo-HSCT, the first in Sri Lanka, highlights the importance of developing a haploidentical HSCT programme as a potential cure for a disease with a dismal outcome.
Background
Worldwide, haemoglobin E β-thalassaemia is the most common genotype of severe β-thalassaemia. The paucity of long-term data for this form of thalassaemia makes evidence-based management challenging. We did a long-term observational study to define factors associated with survival and complications in patients with haemoglobin E thalassaemia.
Methods
In this prospective, longitudinal cohort study, we included all patients with haemoglobin E thalassaemia who attended the National Thalassaemia Centre in Kurunegala, Sri Lanka, between Jan 1, 1997, and Dec 31, 2001. Patients were assessed up to three times a year. Approaches to blood transfusions, splenectomy, and chelation therapy shifted during this period. Survival rates between groups were evaluated using Kaplan-Meier survival function estimate curves and Cox proportional hazards models were used to identify risk factors for mortality.
Findings
109 patients (54 [50%] male; 55 [50%] female) were recruited and followed up for a median of 18 years (IQR 14–20). Median age at recruitment was 13 years (range 8–21). 32 (29%) patients died during follow-up. Median survival in all patients was 49 years (95% CI 45–not reached). Median survival was worse among male patients (hazard ratio [HR] 2·51, 95% CI 1·16–5·43), patients with a history of serious infections (adjusted HR 8·49, 2·90–24·84), and those with higher estimated body iron burdens as estimated by serum ferritin concentration (adjusted HR 1·03, 1·01–1·06 per 100 units). Splenectomy, while not associated with statistically significant increases in the risks of death or serious infections, ultimately did not eliminate a requirement for scheduled transfusions in 42 (58%) of 73 patients. Haemoglobin concentration less than or equal to 4·5 g/dL (vs concentration >4·5 g/dL), serum ferritin concentration more than 1300 μg/L (vs concentration ≤1300 μg/L), and liver iron concentration more than 5 mg/g dry weight of liver (vs concentration ≤5 mg/g) were associated with poorer survival.
Interpretation
Patients with haemoglobin E thalassaemia often had complications and shortened survival compared with that reported in high-resource countries for thalassaemia major and for thalassaemia intermedia not involving an allele for haemoglobin E. Approaches to management in this disorder remain uncertain and prospective studies should evaluate if altered transfusion regimens, with improved control of body iron, can improve survival.
Funding
Wellcome Trust, Medical Research Council, US March of Dimes, Anthony Cerami and Ann Dunne Foundation for World Health, and Hemoglobal.
Background:: There is a significant disparity in global cancer care and out-come between countries. We aimed to provide data on characteristics, average cost of treatment and survival estimates in patients with Hodgkin Lymphoma in Sri Lanka.
Methods: All patients diagnosed with Hodgkin Lymphoma between 01.05.2013 and 01.10.2020 were included in the analysis.
Findings: Classical Hodgkin Lymphoma(cHL) diagnosed in 85%; 68% presented with B symptoms and 61% had advanced stage of disease. Treatment was discontinued by 23% either before or just after starting treatment of whom 72% percent were females. The complete response (CR) rate of patients who continued treatment was 86% while the estimated five-year survival rate is 92%. Seventeen percent of these patients died but only two percent due to Hodgkin Lymphoma or associated treatment in the group which continued treatment compared to 45% in the group who defaulted treatment (p-value 0.0002). Five-year survival rate of patients who defaulted treatment was 50% while patients who continued treatment have an estimated five-year survival rate of 90%. Average cost of first line treatment was between US 7642. First treatment failure may incur substantially higher health care costs.
Interpretation: This is the only well characterized study on long-term survival of patients with Hodgkin Lymphoma in Sri Lanka. We have shown that it is possible to successfully apply western treatment and supportive care protocols to the local population. This published data will help to bench mark and improve the treatment and develop blood cancer care in the local setting.
Introduction:
This study compares liver enzymes, inflammatory markers and bilirubin levels in patients with and without fatty liver disease (FLD) presenting with common bile duct (CBD) obstruction.
Methods:
CBD colic was diagnosed based on clinical, radiological and biochemical criterion. Presence of FLD was diagnosed by ultra sound scan and the macroscopic appearance of liver during surgery. Liver enzymes, inflammatory markers and bilirubin levels were prospectively assessed and compared between the two groups.
Results:
Out of 42, there were 22 (52.3%) patients with FLD. Median body mass index was 26.9 (24.1-30.8) in fatty liver group compared to 25.7 (23.5-26.2) in others. Individuals with FLD showed high aspartate transaminase (558.5 vs. 247.0, p = 0.005), alanine trasaminase (467 vs. 228.5, p = 0.005) and bilirubin (3.8 vs. 2.2, p = 0.015) levels compared to those without FLD. According to multiple linear regression models, high AST and ALT levels showed significant associations with FLD after adjusting for age, gender, body mass index, amylase and C reactive protein levels. The median enzyme level at two weeks did not show a difference among patients with and without FLD.
Conclusions:
Presence of FLD causes unusual rise of AST and ALT levels in patients with CBD stones. This rise is transient.
Background
Sepsis is the main cause of non-relapse mortality and there are no published data on applicability of supportive care protocols from high-income countries in Sri Lanka. The aim of the study was to investigate management and mortality of neutropenic episodes among Haemato-Oncology patients. Methods
We analysed retrospectively clinical characteristics, management, morbidity and mortality of neutropenic adult Haemato-Oncology patients from 01.01.2019 to 31.12.2019. ResultsA total of 169 neutropenic episodes were identified; 115 (68%) of such episodes were related to chemotherapy. Acute leukaemia, Lymphoproliferative disorders and Plasma cell disorders accounted for 23%, 69% and 8% of patients respectively. The median age of patients who had sepsis was 56 while that of those who had no sepsis was 53 (p-value 0.49). Median time to neutropenia was 9 days for those in the sepsis group compared to 8 days in the group that had no sepsis (0.64). Median neutrophil count in the group who had sepsis was 0.06 while it was 0.69 in the group that had no sepsis (p-value 0.000). Median time to commencement of antibiotics was 20 minutes. There were no deaths in the group of chemotherapy induced neutropenic sepsis. Conclusion
This is the only documented study relating to outcome and successful applicability of western supportive care protocols to Sri Lankan patients with neutropenia. In this study we have shown that survival data comparable to developed countries can be achieved in the setting of limited resources with service development, following guidelines and staff training.
Background
Proper retraction of soft and hard tissues is essential to a successful surgery. The TechniqueThe authors wish to report the use of a self-retaining photography cheek retractor during a Le Fort 1 surgery and an alveolar bone graft surgery which proved beneficial by achieving very good retraction. Conclusion
This is an eye opener to modify the traditional photography retractor to maximise its benefits as a retractor in Oral and Maxillofacial surgery.
There are no published data on long‐term survival and applicability of treatment protocols from developed countries in acute myeloid leukaemia (AML) in Sri Lanka. Eighty‐seven AML patients were reviewed; there were 56 newly diagnosed patients between 18 and 65 years. Thirty‐one out of 33 who started treatment achieved complete remission after first cycle of treatment. The induction mortality was one of 33. Twelve out of 20 patients who completed treatment are alive at the time of analysis. The estimated 5‐year overall survival rate is 0.629. Strict infection control and treatment and superior clinical experience may have contributed towards better outcome.
We assessed perceived satisfaction of patients and related factors for treatment provided by the oral surgery student dental clinic of the only Sri Lankan dental school. A descriptive cross-sectional study was conducted among 288 consenting patients who received oral surgery treatment at the student dental clinic of Department of Oral and Maxillofacial Surgery, Faculty of Dental Sciences, University of Peradeniya, Sri Lanka. A self-administered, validated questionnaire and a 19-item multidimensional patient satisfaction scale were used for data collection. Patients of all ages were represented, but were dominated by females, aged 30 to 44 years, possessing educational attainment up to General Certificate of Education ordinary or advanced level of which 54.9% were unemployed. They had travelled distances of less than 10 and 10 to 20 km (28.1% and 27.5%, respectively) predominantly to receive tooth extractions exacerbated by symptoms. Overall, 90% of responding patients were highly satisfied with the items of many dimensions of dental treatment. Despite high levels of perceived satisfaction expressed, further improvements were warranted for waiting time, optimal pain control with more kind, and courteous staff.
COVID-19 pandemic revolutionised the practice of oral & maxillofacial surgery in addition to almost all specialties in health care across the globe. Stringent infection control is the cornerstone in this regard. However, there are some hidden benefits of COVID-19 lockdown and social distancing in reducing the incidence of maxillofacial trauma.
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