Sanjeev Gulati - Academia.edu (original) (raw)

Papers by Sanjeev Gulati

American Journal of Kidney Diseases, Oct 31, 1999

This study was conducted to analyze the trend of histopathologic subtypes in idiopathic nephrotic... more This study was conducted to analyze the trend of histopathologic subtypes in idiopathic nephrotic syndrome (INS) in a homogenous racial group in India population. A prospective analysis of 400 consecutive children with INS was performed. Kidney biopsies were performed according to standard indications. Steroids were administered following the Arbeitsgeminschaft fur Padiatrische Nephrologie protocol. Cyclophosphamide was administered to children in the frequent-relapser, steroid-dependent, and steroid-nonresponder categories. Of the various histopathologic subtypes, focal segmental glomerulosclerosis (FSGS) was the most common (87 of 222 subtypes; 39.1%). Children who underwent biopsy between July 1992 and December 1996 (group B, n ‫؍‬ 157) were compared with our initial published data of biopsies performed between January 1990 and June 1992 (group A, n ‫؍‬ 65), with similar indications for biopsy in both groups. The incidence of FSGS was significantly greater in biopsies performed in the recent period (group B, 47% versus group A, 20%; P ‫؍‬ 0.0002). The different clinical and biochemical parameters were also analyzed to differentiate FSGS from the other 2 subtypes. Hypertension (P ‫؍‬ 0.005), renal insufficiency at presentation (P ‫؍‬ 0.001), and steroid resistance (P ‫؍‬ 0.0006) were significantly greater in children with FSGS. On follow-up (mean, 5.4 years), children with FSGS were at a significantly greater risk for developing renal insufficiency (P ‫؍‬ 0.0001). We conclude there is a shift toward an increasing prevalence of FSGS over the years in the Indian population. This trend has immense therapeutic and prognostic significance.

A prospective study was done to evaluate the incidence and microbiological trend of peritoneal in... more A prospective study was done to evaluate the incidence and microbiological trend of peritoneal infection in patients undergoing acute intermittent peritoneal dialysis (PD). Complete sterile procedure was ensured and at the completion of the procedure PD fluid was sent for bacteriological culture, sensitivity, and total and differential cell count. During the period September 2000 to February 2001 a total of 100 patients were evaluated. Male female ratio was 72:28. Mean age was 43.17 +/- 17.2 years. In 26 patients cyclers were used. Bacterial culture was positive in total of 30 cases (30%). Gram positive, Gram negative and mixed infection was found in 10%, 15%, and 5% respectively. Number of exchanges (31.61 +/- 7.7 vs. 31.3 +/- 6, p = 0.8) were similar and number of repositioning was significantly more in the infected group (23.3% vs. 11.4%, p < 0.01). Total cell count was significantly higher in infected group (274.3 +/- 502 vs. 31.25 +/- 79.34, p < 0.01). Among Gram +ve organisms Staphylococcus was found in 7, Enterococcus faecalis in 4 and Coryne bacterium sps. in 2 cases. Among Gram -ve organisms, E. coli was found in 4, Enterobacter in 3, Klebsiella 1, Pseudomonas 1, Acinetobacter arinatus 5, Acinetobacter baumani 3, and Citrobacter freundii 3. Mixed flora comprised of Enterococcus faecalis 3, Enterobacter 1, Staphlococcus 1, E. coli 3, Citrobacter 1, Acinobacter baumani 1. Although with the cyclers using collapsible bags, staphylococcus was not isolated, the total incidence of infection (11/26 cases) was not decreased with the use of cyclers. We conclude that in acute intermittent peritoneal dialysis the incidence of bacterial infection is 30% with preponderance of Gram -ve over Gram +ve organisms and organism of fecal origin being commoner than those of skin origin. Use of cycler-assisted over manual PD do not improve the incidence of infection. Repositioning of the stiff catheter significantly increases the incidence of infection.

Indian Pediatrics, 2006

This study was conducted to (1) see the histopathological distribution of different subtypes in s... more This study was conducted to (1) see the histopathological distribution of different subtypes in steroid resistant nephrotic syndrome (SRNS) and (2) compare the clinical, biochemical parameters and outcome between Minimal Change Disease (MCD) with non-MCD subtypes in response to immunosuppressive therapy. A retrospective analysis was done of data on all biopsy proven children with idiopathic SRNS (no response to 4 weeks of standard prednisone therapy (60 mg/m(2)/day)) referred to our institute over last 12 years. They were treated with one of the following medications: oral or intravenous cyclophosphamide, cyclosporine or combination of dexamethasone and azathioprine. A comparison was done of the demographic clinical and biochemical features different histopathologies. We studied 136 children with SRNS (100 M, 36 F). They accounted for 15.1%(136/900) of all children with idiopathic nephrotic syndrome. Focal segmental glomerulosclerosis (FSGS) was the commonest 80/136 (59%), followed by MCD (17.6%). Children with non-MCD had a significantly greater prevalence of microhematuria as compared to MCD. The other baseline clinical and biochemical features including the glomerular filtration rate (GFR) were similar. After a mean follow up of 46 (8-148) months, a significantly greater children with non-MCD 65/112) continued to be proteinuric as compared to the MCD (3/24) (p=0.0001). FSGS was the commonest cause of SRNS in our patient population. Children with SRNS secondary to MCD are more likely to achieve remission as compared to non-MCD subtypes and have a better long-term prognosis. Hence kidney biopsy is of significant prognostic value in SRNS.

American Journal of Kidney Diseases the Official Journal of the National Kidney Foundation, Jan 6, 2003

Children with idiopathic nephrotic syndrome (INS) may be at risk for metabolic bone disease (MBD)... more Children with idiopathic nephrotic syndrome (INS) may be at risk for metabolic bone disease (MBD) because of biochemical derangements caused by the renal disease, as well as steroid therapy. No large study to date has shown conclusively that these children are prone to MBD. We prospectively studied 100 consecutive children with INS for clinical, biochemical, and radiological evidence of MBD. These children were treated with prednisone as follows: initial episode, prednisone, 60 mg/m2/d for 6 weeks, followed by 40 mg/m2 on alternate days for 6 weeks. Relapses were treated with 60 mg/m2/d until remission for 3 days, followed by 40 mg on alternate days for 4 weeks and tapered by 10 mg/m2/wk. Osteoporosis is defined as a bone mineral density (BMD) value evaluated by dual-energy X-linked absorptiometry of the lumbar spine of a z score of 2.5 SDs less than the mean. Univariate and multivariate analyses were performed to analyze for factors predictive of low BMD z score. Children were divided into two groups: those who had received repeated courses of steroid therapy (group II: frequent relapsers (FRs), steroid dependent (SD), or steroid nonresponders (SNRs) versus those who had received infrequent courses (group I: infrequent relapsers). Twenty-two of 100 children (22%) had osteoporosis. Comparing clinical features, we observed that 6 of 70 children in group II were symptomatic (hypocalcemic signs) compared with none of 30 children in group I (P = 0.10). However, children in group II had significantly lower mean BMD z scores compared with group I (-1.65 +/- 1.35 versus -1.08 +/- 1.0; P = 0.01). Also, 20 of 70 children in group II had osteoporosis compared with 2 of 30 children in group I (P = 0.012). Children in group II had been administered significantly greater doses of steroids compared with group I (P < 0.00001). On multivariate analysis, factors predictive of a low BMD score were older age at onset (P = 0.000), lower total calcium intake (P = 0.000), and greater cumulative steroid dose (P = 0.005). Children with INS are at risk for low bone mass, especially those administered higher doses of steroids (FRs, SD, or SNRs). These children should undergo regular BMD evaluations, and appropriate therapeutic interventions should be planned.

Neurol India, 2007

Nemaline rod myopathy (NM) is a rare form of congenital myopathy characterized by slowly progress... more Nemaline rod myopathy (NM) is a rare form of congenital myopathy characterized by slowly progressive or nonprogressive muscle weakness and pathognomonic rod-like structures within the muscle fibers. To the best of our knowledge, this is first documentation of the clinicopathological features of this rare entity from India. All cases of NM diagnosed in our laboratory were retrieved. Clinical and pathological features were reviewed. During a period of 1.5 years (Jan 2004 to June 2005), we received 750 muscle biopsies for various reasons. Of which, 15 were diagnosed as congenital myopathies and four as nemaline rod myopathies. Thus, NM comprises 0.53% of all muscle diseases and 22.6% of all congenital myopathies. All of them presented in childhood (first five years of life) with generalized hypotonia, feeding problems, repeated respiratory infections and muscle weakness. Both males and females were equally affected. The CPK levels were normal and EMG was myopathic. Microscopic examination revealed minimal changes but characteristic red-colored material was seen on modified Gomori trichrome staining which was immunopositive to alpha actinin. Ultrastructural examination confirmed this material to be nemaline rods. NM, although a rare form of congenital myopathies, should be suspected in children who present with generalized hypotonia, repeated chest infections and slowly progressive muscle weakness. This report highlights the importance of histochemistry and ultrastructural examination in the diagnosis of this entity, in the absence of the availability of methodology for genetic studies.

Peritoneal Dialysis International Journal of the International Society For Peritoneal Dialysis, Dec 1, 2003

S144 ♦ ♦ ♦ ♦ ♦ Background: The spectrum of bacterial peritonitis in patients on continuous ambula... more S144 ♦ ♦ ♦ ♦ ♦ Background: The spectrum of bacterial peritonitis in patients on continuous ambulatory peritoneal dialysis (CAPD) in India may be different from that seen in developed countries because of differences in culture and in social, environmental, financial, and educational status. We analyzed our data regarding the incidence and outcome of monomicrobial peritonitis in our CAPD patients. ♦ ♦ ♦ ♦ ♦ Patients and Methods: We reviewed the records of 225 patients on CAPD to retrieve data concerning demographics, peritonitis rate, organism isolated, and outcome. Polymicrobial and fungal peritonitis were excluded from the outcome analysis because of their different outcomes. ♦ ♦ ♦ ♦ ♦ Results: We identified 168 episodes of peritonitis (range: 1 -6 episodes per patient). Of the 168 episodes, 106 (63.1%) episodes were culture-positive. Total duration of CAPD was 264.16 patient-years. The overall peritonitis rate was 0.63 episodes per patient-CAPD year. The rates of gram-negative, gram-positive, polymicrobial, and fungal peritonitis were 0.17, 0.11, 0.04, 0.09 episodes per patient-year, respectively. Among the 75 episodes of monomicrobial peritonitis, gram-negative episodes [n = 45 (60%)] were significantly more frequent than gram-positive episodes [n = 30 (40%), p = 0.03]. Escherichia coli was the most commonly seen organism. Organisms of fecal origin (40/75) were significantly more frequent than those of skin origin (21/75, p = 0.0016). Catheter loss (17/45 vs 5/20, p = 0.04), hospitalization (31/ 45 vs 13/30, p = 0.03), death [9/45 vs 3/30, p = nonsignificant (NS)], switch to hemodialysis (8.9% vs 3.3%, p = NS), and reimplantation of the catheter (6.6% vs 3.3%, p = NS) were all more frequent in gram-negative episodes than in gram-positive episodes. ♦ ♦ ♦ ♦ ♦ Conclusions: Gram-negative peritonitis is more frequent than gram-positive peritonitis in our CAPD population in India and is associated with worse outcome. Perit Dial Int 2003; 23(S2):S144-S147 www.PDIConnect.com

Min Minimally Invasive Neurosurgery, Mar 1, 2009

The aims of this study of patients with high-grade gliomas in eloquent brain areas were 1) to ass... more The aims of this study of patients with high-grade gliomas in eloquent brain areas were 1) to assess the postoperative functional outcome, 2) to determine the extent of tumour resection in these difficult locations, 3) to evaluate the practical usefulness of navigated blood oxygenation level-dependent functional magnetic resonance imaging and diffusion tensor tractography. 25 consecutive patients were included in the study. The patients' gross functional neurological status was determined using the 7-step modified Rankin scale. The extent of tumour resection was determined using pre- and postoperative T(1)-weighted or T(1)-weighted, contrast-enhanced MRI images. The average preoperative modified Rankin scale was 1.56+/-0.77, whereas the average postoperative modified Rankin scale was 1.08+/-1.29. There was a significant improvement in mean modified Rankin scale score after surgery. The mean percentage of residual tumour was calculated to 16+/-22% of the original tumour volume (median 8%). Blood oxygenation level-dependent functional magnetic resonance imaging and diffusion tensor tractography were performed in 23 and 18 patients, respectively. Blood oxygenation level-dependent functional magnetic resonance imaging and diffusion tensor tractography facilitated identification of probable functional regions in 91% and 94% of the respective investigations. We feel that the combination of blood oxygenation level-dependent functional magnetic resonance imaging, diffusion tensor tractography, and 3D ultrasound facilitated maximal tumour resection with minimal deficits. The method permits an image-based functional monitoring of the brain during surgery that may aid the preservation of motor and language function.

Indian Pediatrics, May 1, 2006

Indian Journal of Urology, Jul 1, 2002

Total knee arthroplasty (TKA) can be associated with substantial blood loss, leading to increased... more Total knee arthroplasty (TKA) can be associated with substantial blood loss, leading to increased morbidity and need for blood transfusions. The study objective was to evaluate routine use of a thrombin-based topical hemostatic matrix sealant in reducing blood loss and transfusion requirements in primary TKA. 108 patients were enrolled in a prospective, randomized, single-center trial. Patients who received the hemostatic agent demonstrated a lower mean calculated blood loss (1325.2±464.8 mL vs. control, 1509.3±432.8 mL; p=0.02), drain output (415.6±202.0 mL vs. control, 579.9±306.7 mL; p=0.008), and length of stay (3.3±0.8 days vs. control, 3.7±1.1 days; p=0.03), without a statistically significant difference in mean hemoglobin loss or transfusion requirements. The clinical utility of this hemostatic agent to reduce transfusions after uncomplicated, primary TKA continues to remain unclear.

Seizure European Journal of Epilepsy, Jan 12, 2008

Nephrology Dialysis Transplantation, 2006

Background. The relationship between blood pressure (BP) and mortality in haemodialysis patients ... more Background. The relationship between blood pressure (BP) and mortality in haemodialysis patients is unconventional. It is not clear if this is the consequence of uraemia or related to the dialysis type. The goal of this project was to identify the relationship between BP and mortality in patients on chronic peritoneal dialysis (PD). Methods. Patients on PD (n ¼ 1053) from the USRDS prospective DMMS Wave 2 study were analysed. Primary outcomes were all-cause and cardiovascular mortality and duration of hospitalization. Results. Low systolic BP categories, <100 mmHg [hazard ratio (HR), 2.71, P<0.001; and HR 3.83, P<0.001, respectively] and 101-110 mmHg (HR 1.85, P<0.05; and HR 2.92, P<0.005, respectively), but not high systolic BP, increased the risk of all-cause and cardiovascular mortality, with systolic BP 111-120 mmHg as the reference. Pulse BP, but not diastolic BP, followed a similar trend. In subgroup analysis, this association was demonstrated only in patients with a history of heart failure, in patients with diabetes and in those treated with antihypertensive medications. Conclusion. Systolic BP <111 mmHg in PD patients is associated with higher mortality risk, while systolic BP >120 mmHg is associated with fewer hospital days. Aggressive treatment of hypertension in the PD population should be cautioned.

Indian Pediatrics, Apr 1, 1996

Neurology India, 2007

Nemaline rod myopathy (NM) is a rare form of congenital myopathy characterized by slowly progress... more Nemaline rod myopathy (NM) is a rare form of congenital myopathy characterized by slowly progressive or nonprogressive muscle weakness and pathognomonic rod-like structures within the muscle fibers. To the best of our knowledge, this is first documentation of the clinicopathological features of this rare entity from India. All cases of NM diagnosed in our laboratory were retrieved. Clinical and pathological features were reviewed. During a period of 1.5 years (Jan 2004 to June 2005), we received 750 muscle biopsies for various reasons. Of which, 15 were diagnosed as congenital myopathies and four as nemaline rod myopathies. Thus, NM comprises 0.53% of all muscle diseases and 22.6% of all congenital myopathies. All of them presented in childhood (first five years of life) with generalized hypotonia, feeding problems, repeated respiratory infections and muscle weakness. Both males and females were equally affected. The CPK levels were normal and EMG was myopathic. Microscopic examination revealed minimal changes but characteristic red-colored material was seen on modified Gomori trichrome staining which was immunopositive to alpha actinin. Ultrastructural examination confirmed this material to be nemaline rods. NM, although a rare form of congenital myopathies, should be suspected in children who present with generalized hypotonia, repeated chest infections and slowly progressive muscle weakness. This report highlights the importance of histochemistry and ultrastructural examination in the diagnosis of this entity, in the absence of the availability of methodology for genetic studies.

Seizure, 2008

In a patient with no prior history of seizures we were able to record static (mean) intracranial ... more In a patient with no prior history of seizures we were able to record static (mean) intracranial pressure (ICP) and pulsatile ICP (mean ICP wave amplitude) continuously during a first-time generalized epileptic seizure. The patient experienced episodic headache five months after a subarachnoid haemorrhage. In order to rule out low-pressure hydrocephalus she was admitted for a 24-hour intracranial pressure registration. ICP parameters were normal prior to the seizure. We observed an immediate and enormous rise in both static (mean) ICP and pulsatile ICP (mean ICP wave amplitude). Mean ICP and ICP wave amplitude peaked at 93 mmHg and 22 mmHg, respectively. Pulsatile ICP remained elevated after normalization of static ICP and may indicate impairment of intracranial compliance even after the static ICP was normalized.

Postgraduate medical journal, 1992

Pain is a most unusual manifestation of epilepsy, and it is rarer still for chest pain to be the ... more Pain is a most unusual manifestation of epilepsy, and it is rarer still for chest pain to be the primary manifestation of a seizure disorder. We report here a 6 year old boy with epileptic chest pain--'chest epilepsy'--an entity not previously described in children.

Archives of Biochemistry and Biophysics, 1992

Exogenously supplied catalase, a peroxisomal enzyme, has been found to be of therapeutic value in... more Exogenously supplied catalase, a peroxisomal enzyme, has been found to be of therapeutic value in ischemic injury. Therefore, we examined the effect of ischemic-reperfusion injury on the structure and function of kidney peroxisomes. Ischemic injury changed the density of peroxisomes from 1.21 g/cm3 (peak I) to a lighter density of 1.14 g/cm3 (peak II). The number of peroxisomes moving from the normal density population (peak I) to a lower density population (peak II) increased with an increase in ischemic injury. Latency experiments indicated both populations of peroxisomes to be of intact peroxisomes. Immunoblot analysis with antibodies against peroxisomal matrix and membrane proteins demonstrated that after 90 min of ischemia a significant number of matrix proteins were lost in the peak II population, suggesting that functions of these peroxisomes may be severally affected. Reperfusion following ischemic injury resulted in loss of peroxisomal matrix proteins in both peaks I and II, suggesting that peroxisomal functions may be drastically compromised. This change in peroxisomal functions is reflected by a significant decrease in peroxisomal catalase activity (35%) and beta-oxidation of lignoceric acid (43%) observed following 90 min of ischemia. The decrease in catalase activity was more pronounced in reperfused kidneys even after a shorter term of ischemic injury. Reperfusion restored the normal peroxisomal beta-oxidation in kidneys exposed up to 60 min of ischemia. However, 90 min of ischemia was irreversible as there was a further decrease in beta-oxidation upon reperfusion. The decrease in catalase activity during ischemia alone was due to the formation of an inactive complex, whereas during reperfusion, following 90 min of ischemia, inactivation and proteolysis or decreased synthesis of catalase contributed equally toward the injury. The observed changes in the structure and function of peroxisomes as a result of ischemic-reperfusion injury and the ubiquitous distribution of peroxisomes underlines the importance of this organelle in the pathophysiology of vascular injury in general.

Journal of Neuro-Oncology, 2011

There is a need for better predictors for short survival in patients with brain metastases underg... more There is a need for better predictors for short survival in patients with brain metastases undergoing open surgery. The graded prognostic assessment (GPA) has recently been developed to predict survival in patients with brain metastases. We explored the prognostic capabilities of GPA in a consecutive neurosurgical population of brain metastases. Secondarily, we evaluated if GPA scores can provide information on safety of the operation and postoperative functional outcome. We retrospectively included all adult (C18 years) patients undergoing open surgery for brain metastases from 2004 through 2009 (n = 141). The population was grouped into GPA 0-1 (n = 22, 16%), GPA 1.5-2.5 (n = 90, 64%), GPA 3 (n = 19, 14%), and GPA 3.5-4 (n = 10, 7%) according to the prognostic indices. Median survival times were 6.3 months (range 0.8-23.7) in GPA 0-1, 7.8 months in GPA 1.5-2.5 (range 0.2-75.0), 14.0 months in GPA 3 (range 0.0-77.4), and 18.4 months in GPA 3.5-4 (range 0.1-63.7). This represents a significant difference between groups (P = 0.010). There were no associations between GPA and 30-day mortality (P = 0.871), 3-month mortality (P = 0.750), complications (P = 0.330) or change in Karnofsky Performance status postoperatively (P = 0.558). GPA scores hold prognostic properties in patients operated for brain metastases. However, GPA did not predict short-term mortality, limiting the clinical usefulness in a neurosurgical population. The prognostic indices cannot be used alone to decide if surgery is warranted on an individual basis, or to evaluate risks and benefits of surgery.

Neurology, 2010

A 13-month-old boy was evaluated for seizures and developmental regression. He was well until the... more A 13-month-old boy was evaluated for seizures and developmental regression. He was well until the age of 9 months. Then he developed generalized tonic seizures and progressive loss of ability to sit, hold neck, interact, and smile. Examination revealed coarse facies, bilateral macular cherry red spots, and hepatosplenomegaly. A suggestive MRI of brain (figure) and absent ␤-galactosidase enzyme activity in leukocytes confirmed the diagnosis of GM1 gangliosidosis.

Indian pediatrics, 2006

This study was conducted to (1) see the histopathological distribution of different subtypes in s... more This study was conducted to (1) see the histopathological distribution of different subtypes in steroid resistant nephrotic syndrome (SRNS) and (2) compare the clinical, biochemical parameters and outcome between Minimal Change Disease (MCD) with non-MCD subtypes in response to immunosuppressive therapy. A retrospective analysis was done of data on all biopsy proven children with idiopathic SRNS (no response to 4 weeks of standard prednisone therapy (60 mg/m(2)/day)) referred to our institute over last 12 years. They were treated with one of the following medications: oral or intravenous cyclophosphamide, cyclosporine or combination of dexamethasone and azathioprine. A comparison was done of the demographic clinical and biochemical features different histopathologies. We studied 136 children with SRNS (100 M, 36 F). They accounted for 15.1%(136/900) of all children with idiopathic nephrotic syndrome. Focal segmental glomerulosclerosis (FSGS) was the commonest 80/136 (59%), followed ...

American Journal of Kidney Diseases, Oct 31, 1999

This study was conducted to analyze the trend of histopathologic subtypes in idiopathic nephrotic... more This study was conducted to analyze the trend of histopathologic subtypes in idiopathic nephrotic syndrome (INS) in a homogenous racial group in India population. A prospective analysis of 400 consecutive children with INS was performed. Kidney biopsies were performed according to standard indications. Steroids were administered following the Arbeitsgeminschaft fur Padiatrische Nephrologie protocol. Cyclophosphamide was administered to children in the frequent-relapser, steroid-dependent, and steroid-nonresponder categories. Of the various histopathologic subtypes, focal segmental glomerulosclerosis (FSGS) was the most common (87 of 222 subtypes; 39.1%). Children who underwent biopsy between July 1992 and December 1996 (group B, n ‫؍‬ 157) were compared with our initial published data of biopsies performed between January 1990 and June 1992 (group A, n ‫؍‬ 65), with similar indications for biopsy in both groups. The incidence of FSGS was significantly greater in biopsies performed in the recent period (group B, 47% versus group A, 20%; P ‫؍‬ 0.0002). The different clinical and biochemical parameters were also analyzed to differentiate FSGS from the other 2 subtypes. Hypertension (P ‫؍‬ 0.005), renal insufficiency at presentation (P ‫؍‬ 0.001), and steroid resistance (P ‫؍‬ 0.0006) were significantly greater in children with FSGS. On follow-up (mean, 5.4 years), children with FSGS were at a significantly greater risk for developing renal insufficiency (P ‫؍‬ 0.0001). We conclude there is a shift toward an increasing prevalence of FSGS over the years in the Indian population. This trend has immense therapeutic and prognostic significance.

A prospective study was done to evaluate the incidence and microbiological trend of peritoneal in... more A prospective study was done to evaluate the incidence and microbiological trend of peritoneal infection in patients undergoing acute intermittent peritoneal dialysis (PD). Complete sterile procedure was ensured and at the completion of the procedure PD fluid was sent for bacteriological culture, sensitivity, and total and differential cell count. During the period September 2000 to February 2001 a total of 100 patients were evaluated. Male female ratio was 72:28. Mean age was 43.17 +/- 17.2 years. In 26 patients cyclers were used. Bacterial culture was positive in total of 30 cases (30%). Gram positive, Gram negative and mixed infection was found in 10%, 15%, and 5% respectively. Number of exchanges (31.61 +/- 7.7 vs. 31.3 +/- 6, p = 0.8) were similar and number of repositioning was significantly more in the infected group (23.3% vs. 11.4%, p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.01). Total cell count was significantly higher in infected group (274.3 +/- 502 vs. 31.25 +/- 79.34, p &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.01). Among Gram +ve organisms Staphylococcus was found in 7, Enterococcus faecalis in 4 and Coryne bacterium sps. in 2 cases. Among Gram -ve organisms, E. coli was found in 4, Enterobacter in 3, Klebsiella 1, Pseudomonas 1, Acinetobacter arinatus 5, Acinetobacter baumani 3, and Citrobacter freundii 3. Mixed flora comprised of Enterococcus faecalis 3, Enterobacter 1, Staphlococcus 1, E. coli 3, Citrobacter 1, Acinobacter baumani 1. Although with the cyclers using collapsible bags, staphylococcus was not isolated, the total incidence of infection (11/26 cases) was not decreased with the use of cyclers. We conclude that in acute intermittent peritoneal dialysis the incidence of bacterial infection is 30% with preponderance of Gram -ve over Gram +ve organisms and organism of fecal origin being commoner than those of skin origin. Use of cycler-assisted over manual PD do not improve the incidence of infection. Repositioning of the stiff catheter significantly increases the incidence of infection.

Indian Pediatrics, 2006

This study was conducted to (1) see the histopathological distribution of different subtypes in s... more This study was conducted to (1) see the histopathological distribution of different subtypes in steroid resistant nephrotic syndrome (SRNS) and (2) compare the clinical, biochemical parameters and outcome between Minimal Change Disease (MCD) with non-MCD subtypes in response to immunosuppressive therapy. A retrospective analysis was done of data on all biopsy proven children with idiopathic SRNS (no response to 4 weeks of standard prednisone therapy (60 mg/m(2)/day)) referred to our institute over last 12 years. They were treated with one of the following medications: oral or intravenous cyclophosphamide, cyclosporine or combination of dexamethasone and azathioprine. A comparison was done of the demographic clinical and biochemical features different histopathologies. We studied 136 children with SRNS (100 M, 36 F). They accounted for 15.1%(136/900) of all children with idiopathic nephrotic syndrome. Focal segmental glomerulosclerosis (FSGS) was the commonest 80/136 (59%), followed by MCD (17.6%). Children with non-MCD had a significantly greater prevalence of microhematuria as compared to MCD. The other baseline clinical and biochemical features including the glomerular filtration rate (GFR) were similar. After a mean follow up of 46 (8-148) months, a significantly greater children with non-MCD 65/112) continued to be proteinuric as compared to the MCD (3/24) (p=0.0001). FSGS was the commonest cause of SRNS in our patient population. Children with SRNS secondary to MCD are more likely to achieve remission as compared to non-MCD subtypes and have a better long-term prognosis. Hence kidney biopsy is of significant prognostic value in SRNS.

American Journal of Kidney Diseases the Official Journal of the National Kidney Foundation, Jan 6, 2003

Children with idiopathic nephrotic syndrome (INS) may be at risk for metabolic bone disease (MBD)... more Children with idiopathic nephrotic syndrome (INS) may be at risk for metabolic bone disease (MBD) because of biochemical derangements caused by the renal disease, as well as steroid therapy. No large study to date has shown conclusively that these children are prone to MBD. We prospectively studied 100 consecutive children with INS for clinical, biochemical, and radiological evidence of MBD. These children were treated with prednisone as follows: initial episode, prednisone, 60 mg/m2/d for 6 weeks, followed by 40 mg/m2 on alternate days for 6 weeks. Relapses were treated with 60 mg/m2/d until remission for 3 days, followed by 40 mg on alternate days for 4 weeks and tapered by 10 mg/m2/wk. Osteoporosis is defined as a bone mineral density (BMD) value evaluated by dual-energy X-linked absorptiometry of the lumbar spine of a z score of 2.5 SDs less than the mean. Univariate and multivariate analyses were performed to analyze for factors predictive of low BMD z score. Children were divided into two groups: those who had received repeated courses of steroid therapy (group II: frequent relapsers (FRs), steroid dependent (SD), or steroid nonresponders (SNRs) versus those who had received infrequent courses (group I: infrequent relapsers). Twenty-two of 100 children (22%) had osteoporosis. Comparing clinical features, we observed that 6 of 70 children in group II were symptomatic (hypocalcemic signs) compared with none of 30 children in group I (P = 0.10). However, children in group II had significantly lower mean BMD z scores compared with group I (-1.65 +/- 1.35 versus -1.08 +/- 1.0; P = 0.01). Also, 20 of 70 children in group II had osteoporosis compared with 2 of 30 children in group I (P = 0.012). Children in group II had been administered significantly greater doses of steroids compared with group I (P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.00001). On multivariate analysis, factors predictive of a low BMD score were older age at onset (P = 0.000), lower total calcium intake (P = 0.000), and greater cumulative steroid dose (P = 0.005). Children with INS are at risk for low bone mass, especially those administered higher doses of steroids (FRs, SD, or SNRs). These children should undergo regular BMD evaluations, and appropriate therapeutic interventions should be planned.

Neurol India, 2007

Nemaline rod myopathy (NM) is a rare form of congenital myopathy characterized by slowly progress... more Nemaline rod myopathy (NM) is a rare form of congenital myopathy characterized by slowly progressive or nonprogressive muscle weakness and pathognomonic rod-like structures within the muscle fibers. To the best of our knowledge, this is first documentation of the clinicopathological features of this rare entity from India. All cases of NM diagnosed in our laboratory were retrieved. Clinical and pathological features were reviewed. During a period of 1.5 years (Jan 2004 to June 2005), we received 750 muscle biopsies for various reasons. Of which, 15 were diagnosed as congenital myopathies and four as nemaline rod myopathies. Thus, NM comprises 0.53% of all muscle diseases and 22.6% of all congenital myopathies. All of them presented in childhood (first five years of life) with generalized hypotonia, feeding problems, repeated respiratory infections and muscle weakness. Both males and females were equally affected. The CPK levels were normal and EMG was myopathic. Microscopic examination revealed minimal changes but characteristic red-colored material was seen on modified Gomori trichrome staining which was immunopositive to alpha actinin. Ultrastructural examination confirmed this material to be nemaline rods. NM, although a rare form of congenital myopathies, should be suspected in children who present with generalized hypotonia, repeated chest infections and slowly progressive muscle weakness. This report highlights the importance of histochemistry and ultrastructural examination in the diagnosis of this entity, in the absence of the availability of methodology for genetic studies.

Peritoneal Dialysis International Journal of the International Society For Peritoneal Dialysis, Dec 1, 2003

S144 ♦ ♦ ♦ ♦ ♦ Background: The spectrum of bacterial peritonitis in patients on continuous ambula... more S144 ♦ ♦ ♦ ♦ ♦ Background: The spectrum of bacterial peritonitis in patients on continuous ambulatory peritoneal dialysis (CAPD) in India may be different from that seen in developed countries because of differences in culture and in social, environmental, financial, and educational status. We analyzed our data regarding the incidence and outcome of monomicrobial peritonitis in our CAPD patients. ♦ ♦ ♦ ♦ ♦ Patients and Methods: We reviewed the records of 225 patients on CAPD to retrieve data concerning demographics, peritonitis rate, organism isolated, and outcome. Polymicrobial and fungal peritonitis were excluded from the outcome analysis because of their different outcomes. ♦ ♦ ♦ ♦ ♦ Results: We identified 168 episodes of peritonitis (range: 1 -6 episodes per patient). Of the 168 episodes, 106 (63.1%) episodes were culture-positive. Total duration of CAPD was 264.16 patient-years. The overall peritonitis rate was 0.63 episodes per patient-CAPD year. The rates of gram-negative, gram-positive, polymicrobial, and fungal peritonitis were 0.17, 0.11, 0.04, 0.09 episodes per patient-year, respectively. Among the 75 episodes of monomicrobial peritonitis, gram-negative episodes [n = 45 (60%)] were significantly more frequent than gram-positive episodes [n = 30 (40%), p = 0.03]. Escherichia coli was the most commonly seen organism. Organisms of fecal origin (40/75) were significantly more frequent than those of skin origin (21/75, p = 0.0016). Catheter loss (17/45 vs 5/20, p = 0.04), hospitalization (31/ 45 vs 13/30, p = 0.03), death [9/45 vs 3/30, p = nonsignificant (NS)], switch to hemodialysis (8.9% vs 3.3%, p = NS), and reimplantation of the catheter (6.6% vs 3.3%, p = NS) were all more frequent in gram-negative episodes than in gram-positive episodes. ♦ ♦ ♦ ♦ ♦ Conclusions: Gram-negative peritonitis is more frequent than gram-positive peritonitis in our CAPD population in India and is associated with worse outcome. Perit Dial Int 2003; 23(S2):S144-S147 www.PDIConnect.com

Min Minimally Invasive Neurosurgery, Mar 1, 2009

The aims of this study of patients with high-grade gliomas in eloquent brain areas were 1) to ass... more The aims of this study of patients with high-grade gliomas in eloquent brain areas were 1) to assess the postoperative functional outcome, 2) to determine the extent of tumour resection in these difficult locations, 3) to evaluate the practical usefulness of navigated blood oxygenation level-dependent functional magnetic resonance imaging and diffusion tensor tractography. 25 consecutive patients were included in the study. The patients&#39; gross functional neurological status was determined using the 7-step modified Rankin scale. The extent of tumour resection was determined using pre- and postoperative T(1)-weighted or T(1)-weighted, contrast-enhanced MRI images. The average preoperative modified Rankin scale was 1.56+/-0.77, whereas the average postoperative modified Rankin scale was 1.08+/-1.29. There was a significant improvement in mean modified Rankin scale score after surgery. The mean percentage of residual tumour was calculated to 16+/-22% of the original tumour volume (median 8%). Blood oxygenation level-dependent functional magnetic resonance imaging and diffusion tensor tractography were performed in 23 and 18 patients, respectively. Blood oxygenation level-dependent functional magnetic resonance imaging and diffusion tensor tractography facilitated identification of probable functional regions in 91% and 94% of the respective investigations. We feel that the combination of blood oxygenation level-dependent functional magnetic resonance imaging, diffusion tensor tractography, and 3D ultrasound facilitated maximal tumour resection with minimal deficits. The method permits an image-based functional monitoring of the brain during surgery that may aid the preservation of motor and language function.

Indian Pediatrics, May 1, 2006

Indian Journal of Urology, Jul 1, 2002

Total knee arthroplasty (TKA) can be associated with substantial blood loss, leading to increased... more Total knee arthroplasty (TKA) can be associated with substantial blood loss, leading to increased morbidity and need for blood transfusions. The study objective was to evaluate routine use of a thrombin-based topical hemostatic matrix sealant in reducing blood loss and transfusion requirements in primary TKA. 108 patients were enrolled in a prospective, randomized, single-center trial. Patients who received the hemostatic agent demonstrated a lower mean calculated blood loss (1325.2±464.8 mL vs. control, 1509.3±432.8 mL; p=0.02), drain output (415.6±202.0 mL vs. control, 579.9±306.7 mL; p=0.008), and length of stay (3.3±0.8 days vs. control, 3.7±1.1 days; p=0.03), without a statistically significant difference in mean hemoglobin loss or transfusion requirements. The clinical utility of this hemostatic agent to reduce transfusions after uncomplicated, primary TKA continues to remain unclear.

Seizure European Journal of Epilepsy, Jan 12, 2008

Nephrology Dialysis Transplantation, 2006

Background. The relationship between blood pressure (BP) and mortality in haemodialysis patients ... more Background. The relationship between blood pressure (BP) and mortality in haemodialysis patients is unconventional. It is not clear if this is the consequence of uraemia or related to the dialysis type. The goal of this project was to identify the relationship between BP and mortality in patients on chronic peritoneal dialysis (PD). Methods. Patients on PD (n ¼ 1053) from the USRDS prospective DMMS Wave 2 study were analysed. Primary outcomes were all-cause and cardiovascular mortality and duration of hospitalization. Results. Low systolic BP categories, <100 mmHg [hazard ratio (HR), 2.71, P<0.001; and HR 3.83, P<0.001, respectively] and 101-110 mmHg (HR 1.85, P<0.05; and HR 2.92, P<0.005, respectively), but not high systolic BP, increased the risk of all-cause and cardiovascular mortality, with systolic BP 111-120 mmHg as the reference. Pulse BP, but not diastolic BP, followed a similar trend. In subgroup analysis, this association was demonstrated only in patients with a history of heart failure, in patients with diabetes and in those treated with antihypertensive medications. Conclusion. Systolic BP <111 mmHg in PD patients is associated with higher mortality risk, while systolic BP >120 mmHg is associated with fewer hospital days. Aggressive treatment of hypertension in the PD population should be cautioned.

Indian Pediatrics, Apr 1, 1996

Neurology India, 2007

Nemaline rod myopathy (NM) is a rare form of congenital myopathy characterized by slowly progress... more Nemaline rod myopathy (NM) is a rare form of congenital myopathy characterized by slowly progressive or nonprogressive muscle weakness and pathognomonic rod-like structures within the muscle fibers. To the best of our knowledge, this is first documentation of the clinicopathological features of this rare entity from India. All cases of NM diagnosed in our laboratory were retrieved. Clinical and pathological features were reviewed. During a period of 1.5 years (Jan 2004 to June 2005), we received 750 muscle biopsies for various reasons. Of which, 15 were diagnosed as congenital myopathies and four as nemaline rod myopathies. Thus, NM comprises 0.53% of all muscle diseases and 22.6% of all congenital myopathies. All of them presented in childhood (first five years of life) with generalized hypotonia, feeding problems, repeated respiratory infections and muscle weakness. Both males and females were equally affected. The CPK levels were normal and EMG was myopathic. Microscopic examination revealed minimal changes but characteristic red-colored material was seen on modified Gomori trichrome staining which was immunopositive to alpha actinin. Ultrastructural examination confirmed this material to be nemaline rods. NM, although a rare form of congenital myopathies, should be suspected in children who present with generalized hypotonia, repeated chest infections and slowly progressive muscle weakness. This report highlights the importance of histochemistry and ultrastructural examination in the diagnosis of this entity, in the absence of the availability of methodology for genetic studies.

Seizure, 2008

In a patient with no prior history of seizures we were able to record static (mean) intracranial ... more In a patient with no prior history of seizures we were able to record static (mean) intracranial pressure (ICP) and pulsatile ICP (mean ICP wave amplitude) continuously during a first-time generalized epileptic seizure. The patient experienced episodic headache five months after a subarachnoid haemorrhage. In order to rule out low-pressure hydrocephalus she was admitted for a 24-hour intracranial pressure registration. ICP parameters were normal prior to the seizure. We observed an immediate and enormous rise in both static (mean) ICP and pulsatile ICP (mean ICP wave amplitude). Mean ICP and ICP wave amplitude peaked at 93 mmHg and 22 mmHg, respectively. Pulsatile ICP remained elevated after normalization of static ICP and may indicate impairment of intracranial compliance even after the static ICP was normalized.

Postgraduate medical journal, 1992

Pain is a most unusual manifestation of epilepsy, and it is rarer still for chest pain to be the ... more Pain is a most unusual manifestation of epilepsy, and it is rarer still for chest pain to be the primary manifestation of a seizure disorder. We report here a 6 year old boy with epileptic chest pain--'chest epilepsy'--an entity not previously described in children.

Archives of Biochemistry and Biophysics, 1992

Exogenously supplied catalase, a peroxisomal enzyme, has been found to be of therapeutic value in... more Exogenously supplied catalase, a peroxisomal enzyme, has been found to be of therapeutic value in ischemic injury. Therefore, we examined the effect of ischemic-reperfusion injury on the structure and function of kidney peroxisomes. Ischemic injury changed the density of peroxisomes from 1.21 g/cm3 (peak I) to a lighter density of 1.14 g/cm3 (peak II). The number of peroxisomes moving from the normal density population (peak I) to a lower density population (peak II) increased with an increase in ischemic injury. Latency experiments indicated both populations of peroxisomes to be of intact peroxisomes. Immunoblot analysis with antibodies against peroxisomal matrix and membrane proteins demonstrated that after 90 min of ischemia a significant number of matrix proteins were lost in the peak II population, suggesting that functions of these peroxisomes may be severally affected. Reperfusion following ischemic injury resulted in loss of peroxisomal matrix proteins in both peaks I and II, suggesting that peroxisomal functions may be drastically compromised. This change in peroxisomal functions is reflected by a significant decrease in peroxisomal catalase activity (35%) and beta-oxidation of lignoceric acid (43%) observed following 90 min of ischemia. The decrease in catalase activity was more pronounced in reperfused kidneys even after a shorter term of ischemic injury. Reperfusion restored the normal peroxisomal beta-oxidation in kidneys exposed up to 60 min of ischemia. However, 90 min of ischemia was irreversible as there was a further decrease in beta-oxidation upon reperfusion. The decrease in catalase activity during ischemia alone was due to the formation of an inactive complex, whereas during reperfusion, following 90 min of ischemia, inactivation and proteolysis or decreased synthesis of catalase contributed equally toward the injury. The observed changes in the structure and function of peroxisomes as a result of ischemic-reperfusion injury and the ubiquitous distribution of peroxisomes underlines the importance of this organelle in the pathophysiology of vascular injury in general.

Journal of Neuro-Oncology, 2011

There is a need for better predictors for short survival in patients with brain metastases underg... more There is a need for better predictors for short survival in patients with brain metastases undergoing open surgery. The graded prognostic assessment (GPA) has recently been developed to predict survival in patients with brain metastases. We explored the prognostic capabilities of GPA in a consecutive neurosurgical population of brain metastases. Secondarily, we evaluated if GPA scores can provide information on safety of the operation and postoperative functional outcome. We retrospectively included all adult (C18 years) patients undergoing open surgery for brain metastases from 2004 through 2009 (n = 141). The population was grouped into GPA 0-1 (n = 22, 16%), GPA 1.5-2.5 (n = 90, 64%), GPA 3 (n = 19, 14%), and GPA 3.5-4 (n = 10, 7%) according to the prognostic indices. Median survival times were 6.3 months (range 0.8-23.7) in GPA 0-1, 7.8 months in GPA 1.5-2.5 (range 0.2-75.0), 14.0 months in GPA 3 (range 0.0-77.4), and 18.4 months in GPA 3.5-4 (range 0.1-63.7). This represents a significant difference between groups (P = 0.010). There were no associations between GPA and 30-day mortality (P = 0.871), 3-month mortality (P = 0.750), complications (P = 0.330) or change in Karnofsky Performance status postoperatively (P = 0.558). GPA scores hold prognostic properties in patients operated for brain metastases. However, GPA did not predict short-term mortality, limiting the clinical usefulness in a neurosurgical population. The prognostic indices cannot be used alone to decide if surgery is warranted on an individual basis, or to evaluate risks and benefits of surgery.

Neurology, 2010

A 13-month-old boy was evaluated for seizures and developmental regression. He was well until the... more A 13-month-old boy was evaluated for seizures and developmental regression. He was well until the age of 9 months. Then he developed generalized tonic seizures and progressive loss of ability to sit, hold neck, interact, and smile. Examination revealed coarse facies, bilateral macular cherry red spots, and hepatosplenomegaly. A suggestive MRI of brain (figure) and absent ␤-galactosidase enzyme activity in leukocytes confirmed the diagnosis of GM1 gangliosidosis.

Indian pediatrics, 2006

This study was conducted to (1) see the histopathological distribution of different subtypes in s... more This study was conducted to (1) see the histopathological distribution of different subtypes in steroid resistant nephrotic syndrome (SRNS) and (2) compare the clinical, biochemical parameters and outcome between Minimal Change Disease (MCD) with non-MCD subtypes in response to immunosuppressive therapy. A retrospective analysis was done of data on all biopsy proven children with idiopathic SRNS (no response to 4 weeks of standard prednisone therapy (60 mg/m(2)/day)) referred to our institute over last 12 years. They were treated with one of the following medications: oral or intravenous cyclophosphamide, cyclosporine or combination of dexamethasone and azathioprine. A comparison was done of the demographic clinical and biochemical features different histopathologies. We studied 136 children with SRNS (100 M, 36 F). They accounted for 15.1%(136/900) of all children with idiopathic nephrotic syndrome. Focal segmental glomerulosclerosis (FSGS) was the commonest 80/136 (59%), followed ...