Association of vitamin D status with disease severity and outcome in Indian patients with IgA nephropathy.

BACKGROUND: Vitamin D deficiency has been examined as a risk factor for severity and progression of kidney disease due to its immunomodulatory effects. There is paucity of data about its impact in IgA nephropathy (IgAN). METHODS: In a retrospective cohort study, 25 (OH) vitamin D assay was performed in bio-banked baseline serum samples collected during kidney biopsy of 105 adult patients with primary IgAN diagnosed between 2015 and 2019. A level of < 10 ng/mL was defined as Vitamin D deficiency. RESULTS: Mean age of patients was 34 ± 10.6 years, 69.5% were males. Mean baseline 25(OH) Vitamin D levels was 15.9 ± 11.9 ng/mL and 41(39%) patients had vitamin D deficiency. Serum albumin level was lower in vitamin D deficient patients compared to those who had higher vitamin D levels (3.7 ± 0.9 vs 4.1 ± 0.7 g/dl, p = 0.018)but there was no significant difference in baseline proteinuria and eGFR. Crescentic lesions were more frequent in vitamin D deficient group (19.5% vs 6.3%, p = 0.022). At median follow up of 21.5 months (6 - 56 months), there was no difference in remission (68.3% vs 65.6%, p = 0.777) and disease progression (12.5% vs 9.4%, p = 0.614) in those with and without Vitamin D deficiency respectively. On multivariate cox proportional hazard analysis, vitamin D deficiency was not a significant risk factor for renal survival (HR-1.79, 95% confidence interval:0.50-6.34, p = 0.368). CONCLUSION: There was no association between vitamin D deficiency and disease profile as well as renal outcome in Indian patients with IgAN.

Technology and Medical Care in India: Growth of Telehealth Awareness during the COVID-19 Pandemic.

BACKGROUND: COVID-19 pandemic has highlighted the importance of telemedicine in health care delivery worldwide. However, the true success of telemedicine depends on patients' acceptance, which in turn is decided by their interest in telemedicine. In this study, we aim to assess the population interest in telemedicine services across India during the COVID-19 pandemic. METHODS: We measured national-level public interest in "telemedicine" using terms related to telemedicine in Google Trends during the years 2019 and 2020. The relationship between population search volume for telemedicine (composite score) and the number of COVID-19 cases during the early phase of COVID-19 was analyzed. The literacy rate and relative interest in telemedicine in the states were analyzed to assess the impact of education on telemedicine interest. RESULTS: The interest in telemedicine in the year 2020 is higher compared to the year 2019 (U = 269.5, z = -7.043, p<0.001). The search trends for telemedicine increased consistently during the early phase of the COVID-19 pandemic. The greatest search volume was seen in Andhra Pradesh. There was a strong correlation (r = 0.65, p < 0.001) between the initial increase in the number of COVID-19 cases and population-level interest in telemedicine over time. The relative interest in telemedicine for the year 2019 showed a significant direct relationship with the literacy rate (r = 0.47, p = 0.04). However, the relative interest in telemedicine for the year 2020 showed no relationship with the state's literacy rate signifying the spread of telemedicine across literacy barriers. CONCLUSION: Population interest in telemedicine was higher in the year 2020 compared to the previous year and remained high even after the easing of lockdown. The COVID-19 pandemic has played an important role in increasing the Indian public's interest in telemedicine.

Top 100 cited articles in one year of COVID-19 research - A bibliometric analysis.

BACKGROUND: Coronavirus disease (COVID-19)-related scientific publications have increased exponentially during the present pandemic but their influence on biomedical literature is not known. The characteristics of highly cited articles help us to identify important advances and their scientific impact. OBJECTIVES: In the present study, we have identified and analyzed the top 100 most highly-cited articles of COVID-19 research published in the year 2020. METHODS: A cross-sectional bibliometric analysis was using the search terms "severe acute respiratory syndrome-coronavirus-2," "COVID," "nCoV," "Coronavirus," and "HCoV" querying the Google Scholar database using the program "Publish or Perish." The first 100 English language articles with the maximum number of citations were identified and evaluated in detail. RESULTS: The top 100 COVID-19 articles in 2020 had citations ranging from 1147 to 20,440. The median (interquartile range [IQR]) number of citations was 1970 (1456-2939). The number of authors ranged from 1 to 79 (median 10; IQR 5.25-19). The majority of first authors was from China (58%), followed by the United States of America (16%) and the United Kingdom (7%). The top three journals in terms of the number of published articles (37%) were the New England Journal of Medicine, Journal of the American Medical Association, and The Lancet. Most of the top-cited COVID-19 literature were descriptive studies focusing on epidemiology (48%) and clinical course (60%) of COVID-19. CONCLUSION: Clinical course and epidemiology have been the predominant areas of research interest in COVID-19 in 2020. Citation analysis of COVID-19 literature helps us to map out the most important focus for research in this pandemic and to identify gaps in knowledge which would guide further research.

Dengue fever in renal allograft recipients: Clinical course and outcome.

BACKGROUND: There are annual outbreaks of dengue infection in tropical and subtropical countries. This retrospective study aimed to assess the clinical manifestation of dengue and outcome in renal transplant recipients. METHODS: Renal transplant recipients diagnosed with dengue in the nephrology department during the outbreak from August 2015 to December 2015 were included in the study. RESULTS: Twenty patients developed dengue presenting during the outbreak. Mean age was 31.9 ± 8.8 years and all were males. Two patients had severe dengue (dengue hemorrhagic fever, dengue shock syndrome). Clinical presentation included febrile illness (95%), myalgia (65%), headache (30%), retro-orbital pain (10%), and mucocutaneous bleeding manifestations (10%). Three (15%) had third space fluid accumulation and 2 (10%) had hypotension. Ninety percent patients had thrombocytopenia, with 4 requiring platelet transfusion. Leucopenia (WBC < 4000/mm3 ) developed in 50% patients. About 60% had transient transaminitis. One patient with severed dengue expired and 1 recovered with IV immunoglobulin therapy. About 40% patients had rise in serum creatinine, with complete recovery in all patients. CONCLUSION: Clinical manifestations of dengue infection in renal transplant recipients were similar to that in general population. However, leucopenia necessitating temporary withdrawal of immunosuppression was common. Renal dysfunction was frequent but completely reversible.

Impact of anxiety and depression on disease activity and quality of life in patients with lupus nephritis.

Background of the Study: Lupus is an autoimmune disease that affects multiple body systems and requires long-term treatment. The multisystem effect of this disease and long treatment may cause anxiety and depression in patients with lupus nephritis (LN) and ultimately deteriorate their quality of life and also affects the activity of the disease. Aim of the Study: This study aims to assess anxiety, depression, and quality of life in patients with LN and their relationship with disease activity. Material and Methods: A descriptive cross-sectional study was conducted to assess anxiety, depression, and quality of life among patients with LN. A total enumerative technique was used for the recruitment of 100 patients and data collected using standardized tools were analyzed. Results: The results of the study showed that the majority of patients (60.0%) with LN had moderate anxiety and most of them (61.0%) had moderate depression that affected their quality of life and impacted the disease activity index in lupus. Conclusion: LN patients experience significant levels of anxiety and depression, which deteriorates their quality of life and negatively impacts disease activity. Active surveillance for these conditions and early diagnosis might help in the improvement of health-related outcomes in such patients.

Clinical Profile of Nonproteinuric Kidney Disease in Type 2 Diabetic Patients in India.

Background: Diabetic kidney disease (DKD) is the commonest cause of end-stage renal disease (ESRD) across the world. Development of microalbuminuria is the earliest marker of DKD and predicts progressive decline in estimated glomerular filtration rate (eGFR). However, recent evidence has suggested that a significant proportion of type 2 diabetic patients have chronic kidney disease (CKD) without proteinuria. Methods: In this single-center, prospective observational study, 400 consecutive type 2 diabetic patients with either overt proteinuria (>500 mg/day) and/or renal dysfunction eGFR <60 ml/min/1.73 m2) were recruited. Baseline demographic and clinical data were recorded. eGFR and proteinuria were recorded at 6 months and 1 year. Patients with proteinuric (proteinuria >0.5 g/day) and nonproteinuric phenotypes were compared for progression of renal dysfunction in terms of doubling of serum creatinine and need for dialysis. Results: In our study cohort, 106 (26.5%) were nonproteinuric. Both the groups were similar in terms of gender, duration of diabetes, comorbidities, body mass index (BMI), blood pressure control, and glycemic control. The nonproteinuric group was older (56.5 ± 2.1 vs. 54.7 ± 11.6 years, P = 0.012), had lesser prevalence of diabetic retinopathy (49 [46.2%] vs. 218 [74.1%], P < 0.001), higher hemoglobin levels (11.3 ± 1.7 vs. 10.5 ± 2.0 g/dl, P < 0.001), and higher cholesterol levels (169.3 ± 43.3 vs 157.1 ± 58.1 mg/dl, P = 0.025). The nonproteinuric phenotype had higher eGFR at baseline, 6 months, and 1 year. However, doubling of serum creatinine (10 [9.4%] vs. 48 [16.3%]) and progression to ESRD (5 [4.7%] vs. 19 [6.5%], P = 0.159) were not different between the two phenotypes. Conclusion: Nonproteinuric DKD is common. Patients with nonproteinuric DKD tend to be older with a slower decline in eGFR.

Clinical Practice Patterns in IgA Nephropathy: A Global Questionnaire-Based Survey.

Introduction: IgA nephropathy (IgAN) displays ethnic differences in disease phenotype. We aimed to examine how this common disease is managed worldwide. Methods: An online 2-step questionnaire-based survey was conducted among nephrologists globally focusing on various management strategies used in IgAN. Results: A total of 422 nephrologists responded to the initial survey and 339 to the follow-up survey. Of the nephrologists, 13.7% do not get MEST-C scores in biopsy reports; 97.2% of nephrologists use renin-angiotensin-aldosterone system (RAAS) blockade with angiotensin-converting-enzyme inhibitors (ACEi) / angiotensin receptor blockers (ARB) as initial treatment. Other supportive treatments commonly employed are fish oil (43.6%) and sodium-glucose co-transporter-2 (SGLT2) inhibitors (48.6%) with regional differences. Immunosuppression is generally (92.4%) initiated when proteinuria >1 g/d persists for ≥3 months.Main considerations for initiating immunosuppression are level of proteinuria (87.9%), estimated glomerular filtration rate (eGFR) decline (78.7%), lack of response to RAAS blockade (57.6%) and MEST-C score (64.9%). Corticosteroids (89.1%) are universally used as first-line immunosuppression; mycophenolate mofetil is commonly used in resistant patients (49.3%). Only 30.4% nephrologist enroll patients with persistent proteinuria >1 g/d in clinical trials. Nephrologists in Europe (63.6%), North America (56.5%), and Australia (63.6%) are more likely to do so compared to South America (31.3%) and Asia (17.2%). Only 8.1% nephrologists in lower-middle income countries (LMICs) enroll patients in clinical trials, though 40% of them are aware of such trials in their nations. Conclusion: Although most nephrologists agree on common parameters to assess clinical severity of IgAN, use of RAAS blockade, and blood pressure control, there is heterogeneity in use of other supportive therapies and initiation of immunosuppression. There is reluctance to enroll patients in clinical trials with novel treatments, principally in LMICs.

Utility and patient acceptance of telemedicine in nephrology.

PURPOSE: There is an increasing burden of kidney diseases worldwide and access to specialist care is limited. Telemedicine, has been relatively less used in developing countries like India. The current study aims to assess the feasibility and acceptance of telenephrology services at our institute, a public hospital. METHODS: A total of 150 patients were selected by stratified random sampling from the list of attendees who had undergone both in-person outpatient consultation and telenephrology consultation. Patient's attitude towards, and knowledge and acceptance of telenephrology services were evaluated. RESULTS: The average age of the study cohort was 42.52 ± 15.1 years. More than one-third (39.3%) of our patients belonged to the lower middle socioeconomic class. The median distance traveled to reach our outpatient clinic was 113.5 km (3-2249 km). Patients reported lost workdays in 54.7% cases. The majority (95%) of patients managed to consult through teleservices successfully. Ninety percent of the patients gave a satisfaction score of 4 (out of 5) or above for their tele-consultation experience. The most important perceived benefit of teleconsultation was the reduced risk of infection (40.6%) followed by economic benefits (32%). The major disadvantage (36%) was the absence of physical examination. A combination of physical and telenephrology services was the option preferred by 84% of the patients. CONCLUSION: In developing countries like India, with the majority of the population residing outside major cities and with limited medical access, telenephrology has a huge potential to provide quality nephrology care to the remotest parts of the country.

The International IgA Nephropathy Network Prediction Tool Underestimates Disease Progression in Indian Patients.

Introduction: International IgA nephropathy (IgAN) network (IIgANN) prediction tool was developed to predict risk of progression in IgAN. We attempted to externally validate this tool in an Indian cohort because the original study did not include Indian patients. Methods: Adult patients with primary IgAN were stratified to low, intermediate, higher, and highest risk groups, as per the original model. Primary outcome was reduction in estimated glomerular filtration rate (eGFR) by >50% or kidney failure. Both models were evaluated using discrimination: concordance statistics (C-statistics), time-dependent receiver operating characteristic (ROC) curves, R2d, Kaplan-Meier survival curves between risk groups and calibration plots. Reclassification with net reclassification improvement and integrated discrimination improvement (IDI) was used to compare the 2 models with and without race. Results: A total of 316 patients with median follow-up of 2.8 years had 87 primary outcome events. Both models with and without race showed reasonable discrimination (C-statistics 0.845 for both models, R2d 49.9% and 44.7%, respectively, and well-separated survival curves) but underestimated risk of progression across all risk groups. The calibration slopes were 1.234 (95% CI: 0.973-1.494) and 1.211 (95% CI: 0.954-1.468), respectively. Both models demonstrated poor calibration for predicting risk at 2.8 and 5 years. There was limited improvement in risk reclassification risk at 5 and 2.8 years when comparing model with and without race. Conclusion: IIgANN prediction tool showed reasonable discrimination of risk in Indian patients but underestimated the trajectory of disease progression across all risk groups.

Multi-response optimization to obtain better performance and emission level in a diesel engine fueled with water-biodiesel emulsion fuel and nanoadditive.

The present study aims to investigate the optimum condition of stationary diesel engine's operating parameters to obtain better performance and emission level, where the diesel engine is fueled with different concentrations of soybean biodiesel (SB), water, and alumina (Al) nanoadditive. Taguchi method coupled with gray relational analysis has been implemented in this study to obtain the optimum concentration of SB, water, and Al nanoparticle, and statistical analysis of variance (ANOVA) is applied to obtain the individual response of operating parameters on overall engine performance and emission level. Various concentration of SB (10%, 20%, and 30%), water (10%, 20%, and 30%), and Al nanoparticle (50 ppm, 100 ppm, and 150 ppm) are mixed with base diesel (BD) by mechanical agitation and followed by an ultra-sonication process. The fuel properties are measured based on EN590 standards, and the experiments are conducted in a single-cylinder, four-stroke, natural aspirated stationary diesel engine based on an L9 orthogonal array fuel combination. From the obtained gray relational co-efficient (GRC) and signal-to-noise (S/N) ratio, the optimum concentration of SB, water, and nanoadditive are identified as 20%, 10%, and 100 ppm, respectively, and a confirmation experiment has also been carried out to confirm the improvements at optimum condition. The ANOVA results imply that water concentration (WC) has the maximum influence on overall diesel engine's performance and emission level followed by nanoparticle and SB concentrations. Overall, it can be concluded that the engine exhibits better performance and greener emissions at optimal condition.

To Study the Impact of Donor Nephrectomy on Blood Pressure as Measured by Ambulatory Blood Pressure Monitoring and Renal Function.

Prospective living kidney donors need meticulous evaluation prior to kidney donation. Ambulatory blood pressure monitoring (ABPM) is considered the reference standard for diagnosing hypertension. With no prior study available in India in this context, we undertook this study to evaluate the utility of ABPM in kidney donors and effect of donor nephrectomy on renal function. This was a prospective observational study involving healthy prospective kidney donors between 18 and 70 years with normal office blood pressure measurements (OBPM). Detailed clinical and biochemical parameters were recorded. OBPM and 24-hour ABPM was done preoperatively and 3 months following donor nephrectomy. There were 51 donors with a mean age of 46.1 ± 11.3 years, of which 40 (78.4%) were females. Preoperatively, three (5.8%) donors were hypertensive on ABPM but normal on OBPM (P = 0.08). Three months post nephrectomy, hypertension was present in seven (13.7%) donors by ABPM, while only two (3.9%) donors were diagnosed as hypertensive by OBPM (P = 0.02). Median pre-nephrectomy proteinuria was 70 mg (10 mg-180 mg) with a mean estimated glomerular filtration rate (eGFR) using the Modification of Diet in Renal Disease (MDRD) formula of 86.86 ± 19.1 ml/min. Six donors developed >300 mg/day proteinuria, and 17 (33.3%) had a 24-hour urinary protein excretion greater than 150 mg/day. Mean serum creatinine (0.79 ± 0.11 vs 1.03 ± 0.16 mg/dl) significantly increased post donation, more so in donors >55 years of age (1.14 ± 0.25 mg/dl). Our study shows that in transplant donors, ABPM is better for diagnosing hypertension, which otherwise remains masked in 10% of the donors on routine OBPM. Significance of post-nephrectomy hypertension and increasing proteinuria needs further evaluation.

Novel Heterozygous Mutations of Congenital Thrombotic Thrombocytopenic Purpura: A Rare Case Report.

Hereditary thrombotic thrombocytopenic purpura (TTP) is a genetic condition caused by mutations in ADAMTS13 gene, leading to very low levels of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type I domain 13) activity. It is a rare condition associated with multiple reported mutations. Here, we describe a case of hereditary TTP with a compound novel heterozygous mutation along with secondary focal segmental glomerulosclerosis. The patient responded clinically to plasma infusions with resolution of thrombocytopenia, stabilization of renal function, and control of blood pressures. Genetic analysis of the entire family helped in the characterization of the inheritance of this mutation. Our case illustrates the need for focused genetic analysis in a subset of patients presenting with features of TTP to decide the therapeutic plan and manage accordingly.

Combustion, performance, and emission analysis of diesel engine fueled with water-biodiesel emulsion fuel and nanoadditive.

The present study is aimed to analyze the combustion, performance, and emission characteristics of water-emulsified soybean biodiesel fueled diesel engine with alumina nanoadditive and the results compared with conventional diesel fuel (BD). Experiments were conducted in a single-cylinder, four-stroke, variable compression ratio, and natural aspirated diesel engine with an eddy current dynamometer at a constant speed of 1500 rpm. Water-soybean biodiesel emulsion fuel was prepared using a mechanical agitator, in which the water concentration was limited to 10%, whereas soybean biodiesel (SB) and surfactant concentrations were 89% and 1% by volume respectively. Alumina (Al) was chosen as a nanoadditive, and the mass fractions of 50 ppm and 100 ppm were blended with emulsion fuel using ultrasonicator and the physicochemical properties were measured. The physicochemical properties of water-emulsified biodiesel and nanoadditive included emulsified biodiesel are at par with EN14214 limits. The in-cylinder pressure (ICP) and net heat release rate (NHR) values of SB are 5.3% and 7.2% lower than BD respectively, whereas the water inclusion significantly increases the ICP and NHR values by 6.9% and 15.9% compared to SB. Brake-specific fuel consumption (BSFC) of SB is higher than BD, and brake-specific energy consumption (BSEC) is lower than BD. An inclusion of 10% water in SB improves the BSFC and BSEC by 4% and 10.6% respectively compared to SB. The Al nanoparticle inclusion in water-emulsified soybean biodiesel further improves the combustion and performance parameters. The exhaust gas temperature (EGT) of sample fuels seems to be lesser than BD due to efficient combustion. As far as the emission characteristics are concerned, the SB promotes lower level of hydrocarbon (HC), carbon monoxide (CO), and smoke emissions with notable increases in oxides of nitrogen (NOx) and carbon dioxide (CO2) emissions. An inclusion of 10% water in SB reduces the NOx, HC, CO, and smoke emission by 21.2%, 16.7%, 16.9%, and 11.8% respectively under peak brake mean effective pressure (BMEP) condition. The addition of Al nanoparticle in biodiesel emulsion fuel further reduce NOx, HC, CO, and smoke emissions and marginally increases the CO2 emission.

Intravenous immunoglobulin therapy for dengue capillary leak syndrome in a renal allograft recipient.

A 45-year-old man presented 4 months after ABOi renal transplantation with febrile illness and bicytopenia necessitating cessation of mycophenolate mofetil. Dengue non-structural protein 1 antigen (NS1 Ag) test was positive. Lowest total leucocyte count was 3.1×109/L and platelet count was 14×109/L. As fever subsided, patient became tachypneic with abdominal distention and hypotension. Ultrasonographic evaluation revealed ascites, gall bladder wall oedema and bilateral pleural effusion consistent with dengue capillary leak syndrome. He developed massive ascites with abrupt weight gain of 4 kg within 24 hours and worsening renal dysfunction. Patient was deteriorating rapidly in spite of adequate supportive care and we gave a trial of intravenous immunoglobulin (0.5 g/kg/day) for 5 days. Patient improved from day 2, and by day 3, he became haemodynamically stable and recovered completely. Patient was stable at discharge and is on regular follow-up.

Colovesical fistula: a rare complication after renal transplantation.

Colovesical fistula per se is a rare condition and most commonly occurs secondary to diverticular disease in normal patients. Colovesical fistula in the setting of post-renal transplantation is even rarer and very few cases have been reported in literature. Patients with autosomal-dominant polycystic kidney disease (ADPKD) are predisposed to diverticulosis and hence are at a higher risk for fistula formation. Herein, we report a case of colovesical fistula in a renal allograft recipient with ADPKD in the absence of diverticulosis. The patient was successfully operated and is stable with no complications at 1-year follow-up.

The Prevalence of New Onset Diabetes Mellitus after Renal Transplantation in Patients with Immediate Posttransplant Hyperglycemia in a Tertiary Care Centre.

OBJECTIVES: This study aimed to determine the prevalence of immediate posttransplant hyperglycemia and new onset diabetes after renal transplantation (NODAT). It also aims at answering whether posttransplant hyperglycemia per se is a risk factor for future development of NODAT. METHODS: A retrospective study was conducted among patients undergoing kidney transplantation under a single surgical unit in a tertiary care hospital in the past 5 years. All known patients with diabetes were excluded from the study. Immediate postoperative hyperglycemia was defined as random blood sugar (RBS) ≥200 mg/dl or requirement of insulin. NODAT was defined as fasting plasma glucose ≥126 mg/dl or RBS ≥200 mg/dl or if the patient is receiving therapy for glycemic control at 6 weeks or 3 months posttransplantation. RESULTS: The study population included 191 patients. The overall prevalence of posttransplant hyperglycemia and NODAT was 31.4% and 26.7%, respectively. NODAT developed in 28 patients (46.7%) of those who had posttransplant hyperglycemia. Thus, posttransplant hyperglycemia was associated with a fourfold increased risk of NODAT (P = 0.000). Posttransplant hyperglycemia was associated with increased infections (P = 0.04) and prolonged hospital stay (P = 0.0001). Increased age was a significant risk factor for NODAT (P = 0.000), whereas gender, acute rejection episodes, cadaveric transplant, hepatitis C virus status, human leukocyte antigen mismatch, and high calcineurin levels were not significantly associated with the future development of NODAT. CONCLUSION: The significant risk of NODAT posed by posttransplant hyperglycemia makes it prudent to follow up these patients more diligently in a resource-limited setting wherein routine monitoring in all patients is cumbersome.

Bleomycin-induced skin toxicity: is it always flagellate erythema?

Bleomycin-induced skin toxicity is a rare and unique complication. We report a 35-year-old man with nodular lymphocytic predominant Hodgkin's lymphoma, stage IVB, who was started on adriamycin, bleomycin, vinblastin and dacarbazine (ABVD) chemotherapy. He developed pruritic hyperpigmented, patchy skin lesions on the neck, back, chest and thighs after IA cycle of ABVD chemotherapy. Lesions were not typical flagellate rash but hyperpigmented, patchy and mildly pruritic lesions over the trunk and proximal extremities. Lesions increased with continuation of bleomycin and improved gradually after removing the drug from chemotherapy schedule. The patient was in complete remission after VI cycles of chemotherapy (AVD Regimen) and skin lesions healed with minimal residual hyperpigmentation.

Chronic myeloid leukaemia with extreme thrombocytosis.

We report two cases of chronic myeloid leukaemia (CML) with extreme thrombocytosis. The first patient was a 65-year-old man who presented with prolonged history of upper abdominal discomfort, anorexia and two episodes of recent gum bleeds without fever or other bleeding manifestations. He was a chronic smoker with no other comorbidities. Examination revealed moderate hepatosplenomegaly. On investigation, he was found to have extreme thrombocytosis (3,500,000/mm(3)) and leucocytosis with moderate anaemia. In view of the leucocytosis, he was investigated for CML and found to be positive for BCR-ABL by reverse transcription PCR (RT-PCR). He received imatinib 400 mg/day and achieved complete haematological response at the end of 3 months. The second patient was a 7-year-old boy who presented with fever, cough and cold of 2-week duration. Examination revealed mild hepatomegaly with palpable spleen tip. Haemogram and peripheral smear revealed moderate leucocytosis with extreme thrombocytosis (2,800,000/mm(3)). On evaluation, he was found to be BCR-ABL positive and responded well to imatinib treatment. In both these cases, massive thrombocytosis was an unusual presentation of a well-known entity, namely, CML. This degree of thrombocytosis is usually seen only in essential thrombocytosis.