Riyaz Ahmad Bhat

Gastric varices: Classification, endoscopic and ultrasonographic management

Gastric varices (GV) are responsible for 10-30% of all variceal hemorrhage. However, they tend to bleed more severely with higher mortality. Around 35-90% rebleed after spontaneous hemostasis. Approximately 50% of patients with cirrhosis of liver harbor gastroesophageal varices. In this review, new treatment modalities in the form of endoscopic treatment options and interventional radiological procedures have been discussed besides discussion on classification and pathophysiology of GV.

Fabry's disease: Case series and review of literature

Fabrys disease is an X-linked lysosomal storage disorder caused by a deficiency of alpha-galactosidase A enzyme with the progressive accumulation of globotriaosylceramide in vascular endothelial cells leading to cardiovascular, renal, gastrointestinal, neuropathic, lenticular, and dermatological manifestations. It is a rare cause of end-stage renal disease. It classically affects males whereas 10–15% of female heterozygote carriers are affected depending on localization. Both the FD and its association with ESRD is rare. With this background, this case series of five patients along with the review of literature is presented here.

Clinical course, laboratory parameters and outcome of TTP pediatric patients presenting with posterior reversible encephalopathy syndrome

Abstract Aim: The clinical course and outcome of children with thrombotic thrombocytopenic purpura and posterior reversible encephalopathy has not been observed and studied till date. The aim of the present study was to know the clinical course and outcome of children with thrombotic thrombocytopenic purpura and posterior reversible encephalopathy. Results from our observation invite potential insight for further research on this subject. Methods: From January 2005 to February 2013, seven children diagnosed with thrombotic thrombocytopenic purpura and posterior reversible encephalopathy syndromes were admitted in a tertiary care hospital in Srinagar, Kashmir. The demographic parameters, clinical characteristics and laboratory data were noted. The outcome was defined in the form of complete recovery or death. Thrombotic thrombocytopenic purpura was diagnosed on clinical grounds, laboratory parameters and renal biopsy. The diagnosis was established after an expert opinion from a hematologist. Posterior reversible encephalopathy syndrome was defined on neuroimaging. Results: The common clinical parameters which were shared by all the patients were hypertension and altered sensorium. Four (57.1%) patients showed clinical deterioration and died within one week of admission even after intensive management. Three (42.8%) patients improved clinically and recovered fully and were discharged in stable clinical condition. Repeat imaging on discharge was normal. Conclusion: This series of seven pediatric patients is the first series on this subject. The presence of posterior reversible encephalopathy syndrome in pediatric patients with thrombotic thrombocytopenic purpura complicates the clinical course and worsens the prognosis.

A Haemodynamic Analysis to Assess the Safe Dose of Carvedilol across Different Child Class of Liver Disease

Background: Literature regarding safe dose of carvedilol is limited and also safe dose across different child classes of chronic liver disease is not very clear. Aim: We aimed primarily to study, the effect of reasonably safe dose (12.5 mg) of carvedilol in acute reduction of portal pressure and compared it with chronic reduction of portal pressure, after Original Research Article Wani et al.; BJMMR, 7(5): 355-368, 2015; Article no.BJMMR.2015.342 356 proper optimization of dose of carvedilol. Second aim of our study was to define predictors of response for acute and chronic reduction of portal pressure and to assess difference in dose tolerated and response across different child class on chronic basis. Methods: One hundred two consecutive patients of cirrhosis of liver with significant portal hypertension were included and hepatic venous pressure gradient was measured at the base line and after 90 minutes of administration of 12.5 mg carvedilol. After proper dose optimization of carvedilol, hepatic venous pressure gradient was again measured after 3 months to assess the chronic response. Results: The mean age of study population was 58.3±6.6 years. A total of 42.2%, 31.9% and 26.6% patients had child class A, child class B and Child class C cirrhosis, respectively. Mean pre-drug hepatic venous pressure gradient was 16.75±2.12 mmHg which dropped to 13.07±2.32 mmHg after 90 minutes of administration of 12.5 mg of carvedilol. The mean drop of hepatic venous pressure gradient was 4.5±2.2 mmHg and 2.4±1.9 mmHg among responders and non-responders, respectively. Overall, 51% showed acute response while 49% were nonresponders. Low cardiac output and high mean arterial pressure were significantly predicting the acute response, while, low baseline cardiac output was found as an independent predictor. After dose optimization, number of responders increased from 52 to 62. Mean dose of carvedilol was higher in non–responders as compared to responders, though statistically insignificant (p>0.05). Mean reduction of hepatic venous pressure gradient from baseline and after 3 months was 5.5±1.7 mmHg and 2.8±1.6 mmHg among responders and non responders on chronic basis, respectively (p<0.001). Absence of any adverse events (OR 11.3, 95% CI; 1.9-67.8), and more than 2.5 mmHg fall in hepatic venous pressure gradient during acute response (OR 8.7, 95% CI; 3.1-25.3) were found as independent predictors of chronic response (p<0.05). Univariate analysis found that no adverse events, no ascites, low baseline cardiac output, more than 2.5 mmHg fall in hepatic venous pressure gradient during acute response, as predictors of chronic response. However, etiology, child class, variceal size (large vs small) and gender were not significantly associated with chronic response Conclusion: At safe dose and with proper optimization of dose, carvedilol may achieve greater response with minimum side effects among different child classes of liver disease.

Renal allograft transplant recipient with ruptured hydatid native kidney

Echinococcosis of the kidneys in a renal transplant recipient is extremely rare and its occurrence being related to immunosuppression is a possibility which needs further characterisation. Ruptured renal hydatid in a renal transplant recipient is not reported so far to our best knowledge. We present a 42-year-old renal allograft receipient who presented one year after transplant with left flank pain, palpable left lumbar mass and gross hydatiduria. Investigations revealed a ruptured native hydatid kidney. Patient was managed with a combination of chemotherapy and left native nephrectomy and discharged in a satisfactory condition.

Systemic lupus erythematosus presenting as acute adrenal insufficiency: A rare clinical presentation

Systemic lupus erythematosus is a complex autoimmune disease with multisystem involvement with varied presentation. Autoimmune adrenal disease, on the other hand, can be associated with other autoimmune diseases. Adrenal insufficiency as a presenting feature of Systemic lupus erythematosus is a rare occurrence. We hereby report a case of a 20 year-old female who presented to us in an acute hypoadrenal state and was found to have Systemic lupus erythematosus with renal involvement. Patient was successfully managed with steroids and improved clinically.

The Prevalence of Non-alcoholic Fatty Liver Disease and its Associationwith Metabolic Syndrome and Obesity in Pediatric Population of NorthIndia

Background: This study was conducted to evaluate the prevalence of fatty liver and its associations with the components of metabolic syndrome in school children of Kashmir valley of north India. Methods: In this cross-sectional study, a total of 1112 children aged 4-18 years were selected from different schools of Kashmir valley. Anthropometric assessments, blood pressure measurements and fasting blood samples were obtained after proper consent. Ultrasonography was performed by a single sonologist on prefixed dates. Fatty liver was defined by already established criteria for fatty liver disease. Metabolic syndrome was defined according to Adult treatment panel III criteria. Analysis and inferences were drawn using Student’s test, chi-square test, Man Whitney U test and logistic regression analysis. Results: The overall prevalence of fatty liver was 7.4%. The Prevalence of fatty liver in children with metabolic syndrome was 44.4%% and in obese children it was 61%. Body Mass Index, waist circumference and metabolic syndrome are strongly correlated with the prevalence of fatty liver. Conclusion: This study is the first study from India on the prevalence of fatty liver disease in children. The estimates of our study are unexpectedly high and immediate attention is needed to address the problem.

Emphysematous pyelonephritis caused by Candida parapsilosis: an unknown etiological agent.

Emphysematous pyelonephritis (EPN) a rare complication commonly seen in diabetic patients is a necrotising gas producing infection of the renal parenchyma and perinephric tissue predominantly caused by uropathogenic bacteria. Fungi have been rarely reported as the etiological agents, isolated from blood and/ or urine culture. We report a case of EPN caused by a rare etiological agent. A 60 year old diabetic female with no previous history of hospitalization presented to us with a short history of febrile illness associated with abdominal pain. Investigations revealed unilateral EPN “Wan type 1”. Patient was treated with systemic antifungal therapy as per culture sensitivity and it alone proved to be an effective treatment of this clinically difficult condition. Patient was discharged in a satisfactory clinical condition. A rare etiology should always be kept in mind while evaluating a case of EPN.

Does acute response to safe dose of carvedilol vary across different child class of liver disease: A large cohort hemodynamic study

Background: Carvedilol is a nonselective beta-blocker with an additional alpha 1 adrenoceptor blocking action, causing a much greater decrease in portal pressure as compared to propranolol. Materials and Methods: One hundred two consecutive patients of cirrhosis of the liver with significant portal hypertension were included, and hepatic venous pressure gradient (HVPG) was measured at the baseline and after 90 min of administration of 12.5 mg carvedilol. Results: A total of 102 patients with mean age of 58.3 ± 6.6 years were included. A total of 42.2%, 31.9%, and 26.6% patients had child Class A, child Class B, and Child Class C cirrhosis, respectively. Mean baseline HVPG was 16.75 ± 2.12 mmHg that dropped to 13.07 ± 2.32 mmHg, after 90 min of administration of 12.5 mg of carvedilol. The mean drop of HVPG was 4.5 ± 2.2 mmHg and 2.4 ± 1.9 mmHg between responders and nonresponders, respectively. Overall, 52 patients (51%) showed acute response while 50 (49%) were nonresponders. Baseline low cardiac output (CO) and high mean arterial pressure (MAP) were significant predictors of acute response on univariate analysis. On multivariate analysis, low baseline CO was found as an independent predictor. Conclusion: Carvedilol is a drug of choice among beta-blockers for primary prophylaxis of variceal bleed. Hemodynamic parameters like baseline low CO high MAP are significantly predicting acute response while as etiology, child class, and variceal size are not significantly associated with acute response to a safe dose of carvedilol.

Factor analysis of metabolic syndrome components in North Indian adult population of Kashmir

Aim: The Kashmiri population is ethnically distinct, culturally unique, and has distinct lifestyle and dietary habits. There is high prevalence of obesity in the Kashmiri population. With this background, we designed this study to evaluate important metabolic parameters contributing to the prevalence of metabolic syndrome (MS). Materials and Methods: In this prospective study, a total of 500 subjects were recruited over a period of 1 year. Informed consent was taken from all the subjects before selection. Proper permission was sought from the hospital′s Ethical Committee. The subjects were selected from among the attendants who accompanied patients at the inpatient and outpatient departments of Sher-i-Kashmir Institute of Medical Sciences, Srinagar, Kashmir. A stratified random sampling procedure was adopted for the study. All subjects underwent anthropometric assessments, blood pressure measurements, and biochemical analysis. Subjects were screened for the components of MS according to criteria given by the Adult Treatment Panel (ATP) III. Statistical Analysis: Analysis was made and inferences were drawn using the student′s test, chi-square test, and Mann-Whitney U test. Data were analyzed by SPSS version 11.5. Results: The mean age of both the men and women was 37 years. The overall prevalence of MS was 8.6% (n = 43), with males constituting 7.4% and females constituting 9.9%. The prevalence of hypertension was 24.9% for males and 12.3% for females. The prevalence of hyperglycemia was 9.3% for males and 7.8% for females; 9.7% males and 25.9% females had low high-density lipoprotein (HDL) cholesterol; and 17.1% males and 13.2% females had elevated triglyceride levels. The prevalence of obesity in males was 1.9% and in females it was 8.6%. Hypertension was the commonest factor affecting the estimates of MS in men, whereas central obesity and low HDL cholesterol were the common contributing factors in women. Conclusion: Prevalence of component factors such as diabetes, hypertension, and dyslipidemia is high, which needs attention.