Pathological characteristics of fifty patients with renal amyloidosis in Sri Lanka

Renal involvement is the most important cause of morbidity and mortality in systemic amyloidosis. This retrospective analysis was conducted to analyse the clinico-pathological characteristics of renal amyloidosis in a group of Sri Lankan patients undergoing renal biopsy. Renal amyloidosis was observed in 50/ 9712 (0.5%) renal biopsies. The underlying cause for amyloidosis was not known in most. Of the known causes multiple myeloma was the commonest. Nephrotic range proteinuria was the most common clinical outcome and most had grade I to III chronic kidney disease at the time of diagnosis. Glomerulosclerosis was associated with the deterioration of renal function.

Pattern of clinical benign euthyroid goitre in a tertiary care center in the Central Province of Sri Lanka from 1981-2011

Objectives: To describe the pattern of clinical euthyroidgoitre in a tertiary care unit in Sri Lanka before and after iodination of salt in Sri Lanka. Methods: We reviewed our thirty-one year computerised database of patients with goitres, spanning iodination in 1995. Results: Prevalence of euthyroid clinical simple diffuse goitre did not reduce during the first thirteen years (p=0.822). However, it reduced in the latter four years from 2008 to 2011. There is significant reduction of prevalence in the younger age groups (p<0.001). A significant reduction of nodular change was observed in all groups within seven years after iodination (p<0.001). Conclusions: There was a significant reduction in the prevalence of clinical nodularity in the post-iodination era in all age groups. Reduction in prevalence of clinical euthyroid simple diffuse goitre was observed only during the period 2008-2011.

Pattern of lymphoma subtypes in a cohort of Sri Lankan patients.

OBJECTIVES: To investigate the pattern of World Health Organization (WHO) lymphoma sub types in a sample from Sri Lanka. METHODS: Retrospective, descriptive study was carried out using biopsy specimens of patients diagnosed or suspected to have a lymphoma received by the Department of Pathology, University of Peradeniya for WHO sub typing. A sample of 227 cases diagnosed to have a lymphoma. All lymphomas were sub classified according to WHO 2007 revised classification of haematopoietic and lymphoid neoplasm using immunohistochemistry. RESULTS: There were 35 (15.4%) Hodgkin Lymphoma (HL) and 192 (84.6%) non Hodgkin Lymphoma (NHL) specimens. Of the NHL the common sub types were diffuse large B cell lymphoma 87 (38.3%), follicular lymphoma 26 (11.5%) and peripheral T cell lymphoma 25 (11%). Of the HL the common sub types were mixed cellular 20 (8.8%) and nodular sclerosis 13 (5.7%). The mean age of the patients was 48.8 ± 19.3 years and male to female ratio was 1.4:1. The observed patterns of both HL and NHL in the study population were similar to those of other South Asian countries such as India and Pakistan. CONCLUSIONS: In the Sri Lankan sample, common sub types of lymphoma were diffuse large B cell lymphoma and follicular lymphoma. The frequency of lymphoma subtypes in the Sri Lankan sample are in accordance with the globally observed variations and similar to those observed in other South Asian countries.

Retrospective analysis of renal histology in asymptomatic patients with probable chronic kidney disease of unknown aetiology in Sri Lanka.

OBJECTIVES: To study the early pathological changes in renal lesions of asymptomatic patients with kidney diseases, with no definite aetiology living in regions endemic for chronic kidney disease of unknown etiology (CKDUe). Design Retrospective study. SETTING: Regions endemic for CKDUe in and around the North Central Province of Sri Lanka. STUDY POPULATION: Two hundred and eleven asymptomatic patients living in endemic regions detected with renal disease by screening for proteinuria using the dipstick method. Those with long standing hypertension, diabetes mellitus, histological diagnosis of primary glomerular diseases, immunocomplex mediated diseases or renal lesions secondary to systemic diseases were excluded. MEASUREMENTS: Renal lesions were divided into seven histological categories depending on the pathological changes: Category 0: no detectable changes. Category 1: Interstitial fibrosis ± mild interstitial inflammation ± tubular atrophy; no glomerulosclerosis. Category 2: Interstitial fibrosis ± mild interstitial inflammation ± tubular atrophy; glomerulosclerosis. Category 3: Moderate or severe interstitial fibrosis, interstitial inflammation and tubular atrophy ± glomerulosclerosis; Category 4: Interstitial inflammation ± tubular atrophy ± glomerulosclerosis; no interstitial fibrosis. Category 5: The prominent change is interstitial inflammation with tubulitis. Category 6: Severely scarred kidney. Histological categories were compared with calculated glomerular filtration rates and age of the patients. RESULTS: Number of cases in histological categories 0 to 6 were: 7 (3.3%), 71 (33.6%), 53 (25.1%), 63 (29.9%), 0, 2 (0.9%) and 15 (7.1%) respectively. The mean glomerular filtration rate was >90 ml/min in patients in category 0 and 1 and declined progressively in categories 2 and 3. Apart from category 0, all had interstitial fibrosis and in category 1, 62 (87.3%) had interstitial fibrosis without inflammation. Severity of interstitial inflammation increased from category 1 to 3. CONCLUSIONS: The early disease among asymptomatic patients is characterized by interstitial fibrosis without significant interstitial inflammation and glomerular sclerosis with preserved glomerular function. Although the role of interstitial inflammation in the initiation of the disease is not clear, it appears to have a role in the progression of the disease.

Chronic kidney disease in snake envenomed patients with acute kidney injury in Sri Lanka: a descriptive study.

BACKGROUND: Data on the long-term outcomes of acute kidney injury (AKI) in envenomed patients leading to chronic kidney disease (CKD) are scarce. The aim of the study was to investigate this issue and to determine the predictive factors in developing CKD. METHODS: The records of a series of 54 patients who had AKI following a snakebite during the period 2004-2009 and who had been followed up were reviewed in the nephrology unit, Kandy, Sri Lanka. The primary outcome measure was the failure of renal functions to return to normal within 1 year. The renal histology was studied in seven patients. RESULTS: The mean age of the group was 50 years (SD 13 years) and 39 (72%) patients were men. The offending snakes were Russell's viper and hump-nosed viper in 15 (28%) and 13 (24%) patients, respectively. At 1 year, 20 patients (37%) had developed CKD (CKD group) and the rest (63%) had recovered (recovered group). The acute stage serum creatinine was high in both groups with no difference (on admission, p=0.134; on discharge, p=0.323), but the CKD group showed significantly high serum creatinine at 2 months after AKI (p=0.004). Mean duration of renal replacement therapy (RRT) of the recovered group and CKD group were 7 (SD 5) and 16 (SD 12) days, respectively (p=0.015). Renal histology of six CKD patients showed predominant glomerular sclerosis and interstitial nephritis. CONCLUSIONS: CKD is an outcome of severe AKI following snake envenoming probably predicted by the length of RRT.

A histopathological study of cutaneous leishmaniasis in Sri Lanka.

UNLABELLED: INTRODUCTION; Cutaneous leishmaniasis is endemic in Sri Lanka. The immunopathogenesis of these lesions in Sri Lankans has not been documented. OBJECTIVES: To classify skin lesions into histological groups, to assess parasitic load, density of each inflammatory cell type and necrosis and to characterise the lymphocytic reaction in cutaneous leishmaniasis in comparison to leprosy. METHODS: Skin biopsies from 31 patients with demonstrable amastigotes in smears or tissue sections were studied. The lesions were classified by two independent observers into four distinct histological groups based on different cell types in the inflammatory infiltrate and formation of granulomata. Parasitic load and the presence of necrosis were recorded. Immunohistochemical staining for CD45RO and CD20 for counting T and B cells respectively was done. RESULTS: Histological groups of cutaneous leishmaniasis ranging from group I-IV were similar to that of the spectrum in leprosy ranging from lepromatous to tuberculoid leprosy. The histological groups from I-IV showed a significant inverse relationship with the mean parasitic index. Necrosis was not a prominent feature. The mean percentage of T cells in the histological spectrum from group I-IV in leishmaniasis was similar to the spectrum from lepromatous to tuberculoid leprosy. Mean percentage of T cells were 20.1% in group I, 20.5% in group II, 33.8% in group III and 47.8% in group IV. Lepromatous, borderline tuberculoid and tuberculoid leprosy had 21.3%, 33.4% and 48.0% T cells respectively. CONCLUSION: Cutaneous leishmaniasis is a spectral disease similar to leprosy. The mean percentage T cells from group I-IV were similar to those in the spectrum of leprosy and mean percentage B cells varied in a narrow range.

Multicentric Castleman disease of hyaline vascular variant presenting with unusual systemic manifestations: a case report.

BACKGROUND: Castleman disease is a rare lymphoproliferative disorder presenting with localized or disseminated lymphadenopathy and systemic manifestations. It can be categorized in numerous ways, such as unicentric versus multicentric, histopathological variants (hyaline-vascular, plasma cell, and mixed), or subtypes based on causative viral infections (human immunodeficiency virus, human herpesvirus-8, or Kaposi sarcoma herpesvirus). Presentation ranges from asymptomatic to symptoms involving multiple organs. Even though the exact mechanism of pathogenesis is unknown, treatment is directed toward possible etiologies such as interleukin-6, cluster of differentiation 20, and viral agents. CASE PRESENTATION: A 36-year-old Sri Lankan woman presented with generalized body swelling and foamy urine of 2 weeks' duration. Examination revealed pallor; generalized edema; axillary, cervical, and inguinal lymphadenopathy; hypertension; and hepatomegaly. Investigations showed bicytopenia, nephrotic range proteinuria with hypoalbuminemia, hypogammaglobulinemia, and features of hyaline-vascular type Castleman disease in a lymph node biopsy. She was managed with rituximab and had good clinical improvement. CONCLUSIONS: Castleman disease has a broad spectrum of clinical manifestations, disease pathogeneses, and associations and/or complications. Medical professionals need to be familiar with this spectrum because timely diagnosis and aggressive targeted therapy are the cornerstones of managing these patients.

Endemic chronic kidney disease of unknown etiology in Sri Lanka: Correlation of pathology with clinical stages.

Chronic kidney disease of unknown etiology (CKDU) is endemic among the rural farming communities in several localities in and around the North Central region of Sri Lanka. This is an interstitial type renal disease and typically has an insidious onset and slow progression. This study was conducted to identify the pathological features in the different clinical stages of CKDU. This is a retrospective study of 251 renal biopsies identified to have a primary interstitial disease from regions endemic for CKDU. Pathological features were assessed and graded in relation to the clinical stage. The mean age of those affected by endemic CKDU was 37.3 ± 12.5 years and the male to female ratio was 3.3:1. The predominant feature of stage I disease was mild and moderate interstitial fibrosis; most did not have interstitial inflammation. The typical stage II disease had moderate interstitial fibrosis with or without mild interstitial inflammation. Stage III disease had moderate and severe interstitial fibrosis, moderate interstitial inflammation, tubular atrophy and some glomerulosclerosis. Stage IV disease typically had severe interstitial fibrosis and inflammation, tubular atrophy and glomerulosclerosis. The mean age of patients with stage I disease (27 ± 10.8 years) was significantly lower than those of the other stages. About 79.2%, 55%, 49.1% and 50% in stage I, II, III and IV disease respectively were asymptomatic at the time of biopsy.

Star fruit toxicity: a cause of both acute kidney injury and chronic kidney disease: a report of two cases.

BACKGROUND: Star fruit (Averrhoa carambola) is commonly consumed as a herbal remedy for various ailments in tropical countries. However, the dangers associated with consumption of star fruit are not commonly known. Although star fruit induced oxalate nephrotoxicity in those with existing renal impairment is well documented, reports on its effect on those with normal renal function are infrequent. We report two unique clinical presentation patterns of star fruit nephrotoxicity following consumption of the fruit as a remedy for diabetes mellitus-the first, in a patient with normal renal function and the second case which we believe is the first reported case of chronic kidney disease (CKD) due to prolonged and excessive consumption of star fruits. CASE PRESENTATION: The first patient is a 56-year-old female diabetic patient who had normal renal function prior to developing acute kidney injury (AKI) after consuming large amount of star fruit juice at once. The second patient, a 60-year-old male, also diabetic presented with acute on chronic renal failure following ingestion of a significant number of star fruits in a short duration with a background history of regular star fruit consumption over the past 2-3 years. Both had histologically confirmed oxalate induced renal injury. The former had histological features of acute tubulo-interstitial disease whilst the latter had acute-on-chronic interstitial disease; neither had histological evidence of diabetic nephropathy. Both recovered over 2 weeks without the need for haemodialysis. CONCLUSION: These cases illustrate the importance of obtaining the patient's detailed history with respect to ingestion of herbs, traditional medication and health foods such as star fruits especially in AKI or CKD of unknown cause.

Mucosal changes in the upper gastrointestinal tract in Sri Lankan patients with squamous carcinoma of the oesophagus.

BACKGROUND: Squamous carcinoma of the oesophagus, is a common tumour in Sri Lanka. A thickened and pale appearance of the oral, hypopharyngeal and oesophageal mucosa, has been observed during endoscopy. The aim of this study was to document the histological changes of the mucosa thus affected in 93 patients with squamous carcinoma of the oesophagus. METHOD: 93 patients with oesophageal carcinoma had mucosal biopsies done from the left cheek, hypopharynx and 2.5 cm. and 5 cm above the tumour, during endoscopy for biopsy of the tumour. Paraffin sections of the biopsy stained with Haematoxylin and Eosin were examined. 25 specimens of oesophageal tissue, and tissue from the mucosa and hypopharyngeal mucosa obtained at coroners' autopsies from a comparable group served as controls (15 males, 10 females). RESULTS: The tumours were squamous carcinoma of varying grades of differentiation. The mucosal biopsies showed, acanthosis, basal cell hyperplasia, intra epithelial neoplasia (IEN) grades I-III, chronic oesophagitis, koilocytosis and papillomatosis. The mucosa of the control group only showed koilocytosis and papillomatosis in two cases. CONCLUSION: Pathological changes such as acanthosis, basal cell hyperplasia, intra epithelial neoplasia, chronic oesophagitis koilocytosis and papillomatosis occur in the oral hypopharyngeal and other areas of the oesophageal squamous mucosa, in association with squamous carcinoma of the oesophagus.

Changing patterns of thyroid cancer in Sri Lanka. Has the iodination programme helped?

OBJECTIVES: To ascertain if there has been a change in the pattern of thyroid cancer in Sri Lanka. If so, whether there is a correlation to the implementation of the programme of iodination. DESIGN: Retrospective (1974-1986) and prospective (1987-2001). SETTING: Kandy Hospital (1974-1982), Peradeniya Hospital (1982-2001) and private hospitals in Kandy (1979-2001). METHOD: Three hundred consecutive patients with cancer of the thyroid seen over 28 years (1974-2001) period were reviewed for demography, histopathology and extent of spread at presentation. Seventy one patients of this group had a pre-existing goitre of greater than 10 years' duration and were similarly reviewed. RESULTS: A highly significant reduction of anaplastic thyroid cancer and a highly significant reduction in the extent of extra-thyroidal spread at presentation of differentiated thyroid cancer were observed after 1995. In malignancy supervening on pre-existing goitre, a significant reduction in anaplastic carcinoma and a highly significant increase in papillary carcinoma were noted in the post-1996 period. A significant reduction of extra-thyroidal spread was also observed. CONCLUSION: A trend towards more differentiated thyroid cancer with lesser degree of spread was observed in recent years. The iodination programme implemented in 1995 is likely to be responsible for this change.

Skeletal fluorosis with neurological complications.

A case of skeletal fluorosis with spinal cord compression from Kekirawa following consumption of water with high fluoride content for about 20 years is described. The observations of other workers (4,5) and the present case report show that more extensive field studies among vulnerable populations is indicated in this region. The need to search for a cost effective method of defluoridation of water is stressed.