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Biomedical subjects

S Gulati

Publications and source records attributed to S Gulati.

At least 37 records · Page 2Linked to original sources
PubMed2001

Spectrum of adolescent-onset nephrotic syndrome in Indian children.

There are few data regarding adolescent-onset nephrotic syndrome (NS) and no guidelines for biopsy criteria and treatment protocol. This study was conducted to analyze the clinical spectrum of adolescent-onset NS and evaluate possible biopsy criteria in these children. A prospective analysis was carried out on all patients with idiopathic NS (fulfilling the ISKDC criteria) with onset between 1 and 18 years of age. They were evaluated clinically, followed by biochemical investigations and kidney biopsy. These characteristics of patients with onset between 1 and 12 years (group A) were compared with the same parameters in patients with onset between 12 and 18 years of age (group B) referred to our hospital over the same period. Among all clinical parameters, microhematuria was significantly more prevalent in adolescents (P<0.001). Kidney biopsy was performed in 88% of adolescent patients. Focal segmental glomerulosclerosis (FSGS) was the most-common histopathology in group B (46.3%) compared with minimal change disease (MCD) in group A (42.9%). Group B had a significantly higher frequency of membranoproliferative glomerulonephritis (MPGN) (P<0.005) and a significantly lower frequency of MCD (P<0.001). The biochemical parameters at the onset were similar. On comparing microhematuria, hypertension, and renal insufficiency at presentation, we observed that two or more of these features were present in all patients with MPGN and only in 19.6% of adolescents with MCD, mesangioproliferative glomerulonephritis, and FSGS. The frequency of steroid resistance was significantly higher in group B (P<0.001). In conclusion, adolescent-onset NS differs from the childhood variety in having a significantly higher frequency of hematuria, steroid resistance, and evidence of non-MCD, especially MPGN, on histopathology. Kidney biopsy can be restricted to those adolescents who have at least two abnormal clinical/biochemical features or are steroid non-responders.

Adolescent↗
PubMed2001

West syndrome and other infantile epileptic encephalopathies--Indian hospital experience.

Children with infantile epileptic encephalopathies comprising 3.5% of the Pediatric Neurology Clinic registrations in a tertiary care hospital were retrospectively analyzed. Data were retrieved from case records and analyzed for seizure semiology, prenatal and perinatal insults, developmental status and relevant investigations. The various therapeutic modalities and their influence on spasm frequency, long-term development and final seizure status were compared. The two primary outcome variables analyzed included final seizure status and developmental outcome. Of the 94 infantile epileptic encephalopathies, West syndrome was the commonest (55.3%), of which two thirds were symptomatic. Etiological factors were prenatal in 66.6% and perinatal in 33.3%. The initial response to ACTH was good in 54.5% with subsequent relapse in 27.8% and for prednisolone was 52.9 and 44.4%, respectively, compared to 25.3% spasms control with conventional antiepileptic drugs. Disease category of infantile epileptic encephalopathies evolved in 4, i.e. early myoclonic encephalopathy to West syndrome 1, early infantile epileptic encephalopathy to West syndrome 1, West syndrome to Lennox-Gastaut syndrome 2. Psychomotor retardation was seen in 88.2%, with 16.1% having normal development at onset of spasms. Microcephaly was associated with delayed development but did not influence final seizure outcome. Final seizure outcome was poor in children with delayed development at onset (adjusted odds ratio [OR]=4), delay in diagnosis >12 months (OR=2.27) and in children with Lennox-Gastaut syndrome (OR=4.75). ACTH/prednisolone and antiepileptic treatment versus antiepileptics alone showed a good final seizure response in 36.6% versus 20%. Development on follow up was delayed in children with initial psychomotor retardation (OR=23.4) and abnormal electroencephalogram (OR=7.46). Perinatal factors constituted one third of symptomatic West syndrome. The use of ACTH/corticosteroids resulted in good initial spasm control though final seizure outcome and development were unaffected. Prednisolone had similar response to ACTH in spasm control but higher subsequent relapse rate. Vigabatrin was useful though often unaffordable. The identification of a neurometabolic etiology, though uncommon, has significant therapy implications. Delay in diagnosis was common and negatively influenced final seizure outcome.

Adrenocorticotropic Hormone↗
PubMed2001

C4bp binding to porin mediates stable serum resistance of Neisseria gonorrhoeae.

Screening of 29 strains of Neisseria gonorrhoeae revealed that 16/21 serum resistant strains and 0/8 serum sensitive strains bound C4bp, suggesting that C4bp binding to gonococci could contribute to serum resistance. C4bp bound to gonococci retained cofactor (C4b-degrading) function. Using allelic exchange to construct strains with hybrid Por1A/B molecules, we demonstrate that the N-terminal loop (loop 1) of Por1A is required for C4bp binding. Serum resistant Por1B gonococcal strains also bind C4bp via their Por molecule. Using allelic exchange and site-directed mutagenesis, we have shown that loops 5 and 7 together form a negatively charged C4bp binding domain. C4bp-Por1B interactions are ionic in nature (inhibited by high salt as well as by heparin), while the C4bp-Por1A bond is hydrophobic. mAbs directed against SCR1 of the alpha-chain of C4bp inhibit C4bp binding to both Por1A and Por1B. Furthermore, only recombinant C4bp mutant molecules that contain alpha-chain SCR1 bind both Por1A and Por1B gonococci, confirming that SCR1 contains Por binding sites. C4bp alpha-chain monomers do not bind strains with either Por molecule, suggesting that the polymeric form of C4bp is required for binding to gonococci. Inhibition of C4bp binding to serum resistant Por1A and Por1B strains in a serum bactericidal assay using fAb fragments against C4bp SCR1 results in complete killing at 30 min of otherwise fully serum resistant strains in only 10% normal serum, underscoring the role of C4bp in mediating gonococcal serum resistance.

Amino Acid Sequence↗
PubMed2001

Effect of lovastatin, an HMG CoA reductase inhibitor, on acute renal allograft rejection.

3-Hydroxy-3-methyl glutaryl coenzyme A (HMG CoA) reductase inhibitors are established anti-lipidemic agents. They also exert immunomodulatory effects. Two recent reports suggest that pravastatin may be useful in decreasing the incidence and severity of acute rejections (ARs) in heart and kidney transplant recipients. We undertook this prospective, randomized, placebo-controlled, double blind trial to investigate the effect of lovastatin on acute renal allograft rejection. Sixty-five consecutive, one-haplotype-matched, living related first renal transplant recipients were randomized to receive either lovastatin 20 mg/d or placebo for 3 months, in addition to cyclosporine, azathioprine, and steroids. Lipid levels, AR episodes, and liver and muscle enzymes were followed for 3 months post-transplant. At the end of the study period, lovastatin had successfully controlled lipid levels. However, there was no effect on AR episodes (15.15% in the treatment group vs. 18.75% in the placebo group).

Adult↗
PubMed2001

Children with hypoalbuminemia on continuous peritoneal dialysis are at risk for technique failure.

BACKGROUND: Few data are available on the clinical significance of hypoalbuminemia [serum albumin (SA) <35 g/L] in children with end-stage renal disease (ESRD) on continuous peritoneal dialysis (CPD). This study was conducted to analyze the prevalence of hypoalbuminemia, its predictive factors, and its clinical impact in these children. METHODS: A retrospective analysis was done of 180 patients on CPD over the last 22 years. Patients were excluded from the study if they were on CPD for less than four months or had nephrotic syndrome. Demographic, clinical, and biochemical variables were studied. Children continued on CPD until they received a transplant or were transferred to an adult unit or to hemodialysis as a result of technique failure. The subjects were divided into two groups based on SA levels at last follow-up. RESULTS: A total of 135 children was included. After a mean duration of CPD of 573 +/- 437 (120 to 2960) days, 54 children (40%) were observed to have hypoalbuminemia. Four patients (2.9%) died, 7 (5.2%) continued on continuous cyclic peritoneal dialysis, and 13 (9.6%) were transferred to an adult unit for continuation of CPD. Ninety-five (70.3%) were transplanted, and 16 (11.8%) were transferred to hemodialysis because of technique failure. Children in group I (N = 54, SA <35 g/L), compared with group II (N = 81, SA > or =35 g/L), were younger at initiation of PD, more likely to have hypoalbuminemia at one month and six months after initiation of PD, and have more episodes of peritonitis. No differences were seen between the groups in gender, modality of CPD, body surface area, initial body mass index, and presence of hypertension or acidosis. The only factors predictive of hypoalbuminemia on follow-up were low SA at one month after PD and recurrent peritonitis using multiple logistic regression analysis. Evaluating the clinical impact of hypoalbuminemia, we observed a higher incidence of failed PD in children who had hypoalbuminemia. CONCLUSION: Low SA at one month after PD and recurrent peritonitis are predictive of hypoalbuminemia in children on CPD, which is associated with an increased incidence of CPD failure.

Adolescent↗
PubMed2001

Pulse cyclophosphamide therapy in frequently relapsing nephrotic syndrome.

BACKGROUND: The treatment of frequently relapsing (FR) and steroid-dependent (SD) idiopathic nephrotic syndrome (INS) with oral cyclophosphamide (OCP) poses problems of compliance, side-effects and infections. METHODS: We prospectively evaluated the usefulness of intravenous cyclophosphamide (IVCP) in children with steroid sensitive INS who were frequent relapsers or steroid dependent. Fifty-one children were included in the study of whom 22 were FR and 29 were SD. IVCP was administered in a dose of 500 mg/m(2)/month for 6 months after achieving a steroid-induced remission. The response to IVCP was evaluated in terms of remission, change in the steroid response status of the patient, duration of remission (i.e. proteinuria-free days), side effects and compliance with therapy. RESULTS: The proteinuria-free days (mean 19.9+/-3.5 before IVCP therapy vs 1256+/-167 days after IVCP therapy) (P<0.00001), and serum albumin levels (23+/-1.6 g/l before IVCP therapy vs 34+/-2 g/l after IVCP therapy) (P<0.001) were significantly higher following IVCP therapy. The cumulative remission rate in the study group was 49% at 5 years and was comparable to that achieved with oral cyclophosphamide at a 40% lower cumulative dose. CONCLUSIONS: We conclude that IVCP is a safe and effective therapeutic modality in children with INS who are FR and SD. Its efficacy is comparable to the results obtained with oral cyclophosphamide based on historical comparisons with previous studies.

Adolescent↗
PubMed2001

Vitamin E administration and reversal of neurological deficits in protein-energy malnutrition.

Neurological signs including posterior column, spinocerebellar, retinal, and peripheral nerve deficits are being increasingly recognized in vitamin E deficiency states. Children suffering from protein-energy malnutrition (PEM) revealed significantly reduced serum alpha-tocopherol levels compared to age-matched normal children, the deficient subjects also exhibited the widely recognized signs of tocopherol deficiency. In this prospective therapeutic intervention study moderate PEM subjects were administered aqueous oral vitamin E supplementation for 6 weeks and compared with control PEM subjects. The parameters studied included pre- and post-therapy serum alpha-tocopherol levels, alpha-tocopherol lipid ratio, lipid profile, creatine phosphokinase levels, and electroneurophysiological studies. Vitamin E supplementation normalized serum alpha-tocopherol levels (p < 0.001), alpha-tocopherol lipid ratio (p < 0.001), reduced creatine phosphokinase levels (p < 0.01), and reduced neurological signs in PEM subjects (p < 0.001). The observed improvement in neurological dysfunction among PEM subjects is of great interest, especially in developing countries. While larger studies are recommended, the importance of vitamin E administration in PEM is being reported.

Child↗
PubMed2001

Comparative utility of technetium-99m hexamethylpropylenamine oxime single photon emission computed tomography (SPECT) with anatomic neuroimaging and electroencephalography (EEG) in childhood intractable epilepsy.

Intractable epilepsies pose a therapeutic challenge. Precise localization of the epileptic focus is imperative before planning surgical intervention. Functional imaging is an important component of presurgical work-up. Positron emission tomography is unavailable in developing countries; hence, the need to evaluate the available imaging modality, single photon emission computed tomography (SPECT), was felt. We investigated 61 children with intractable epilepsy, identified by predefined criteria, by performing electroencephalography (EEG), magnetic resonance imaging (MRI), computed tomography (CT), and ictal and interictal SPECT. The localizing value of ictal and interictal SPECT imaging for epileptic foci was correlated with clinical, electrophysiologic, and anatomic neuroimaging data. An ictal SPECT was obtained in 9, and interictal SPECT was performed in all (61). Ictal SPECT was localizing in 8 of 9 (88.8%). Interictal SPECT was localizing in a significantly higher proportion of patients (47.54%) than either the scalp EEG (16.39%) (P = .0003) or CT scan (21.56%) (P = .0046). Our data demonstrated that interictal and ictal SPECT identified more focal changes in children with intractable epilepsy than interictal EEG, CT, and probably MRI. The definitive proof of the SPECT-based findings being epileptogenic foci awaits correlation with intraoperative monitoring and postoperative follow-up.

Adolescent↗
PubMed2001

Strategies for mimicking Neisserial saccharide epitopes as vaccines.

Monoclonal antibody (mAb) 2C7 recognizes a conserved and widely expressed oligosaccharide (OS) epitope on Neisseria gonorrhoeae. This OS epitope evokes a significant bactericidal and opsonic immune response after natural infection and vaccination. The OS epitope structure represents an excellent target for a potential protective gonococcal vaccine. Because carbohydrate antigens are T-cell independent, inducing weak antibody responses, OS molecules are not useful immunogens. We developed and examined two different strategies to mimic the 2C7 OS epitope: (i) an anti-idiotope (mAb CA1); and (ii) a peptide (PEP-1). These surrogate immunogens elicited antibody responses in mice (CA1 and PEP-1) and rabbits (CA1) that were bactericidal in vitro against gonococci. Both CA1 and PEP-1 are true immunologic mimics of OS and may form a basis for the development of vaccine candidates for human immunization against N. gonorrhoeae.

Animals↗
PubMed2001

Nosocomial infections due to Acinetobacter baumannii in a neurosurgery ICU.

Invasive infections caused by Acinetobacter baumannii in a post-operative neurosurgery ICU were studied. Sixty one patients admitted during a span of 11 months were culture positive for acinetobacter species from blood and/or CSF samples. They were followed up prospectively for evidence of infection and clinical outcome. 40 cases had clinical evidence of infection due to acinetobacter species while in 21 patients, the isolation of the organism was considered a contaminant. Acinetobacter baumannii was the most common organism associated with invasive infections. Respiratory tract was found to be the most common primary source of infection in patients with bacteraemia or meningitis. The age, sex and pre-operative hospital stay were not significantly different in the two groups (p>0.05), while post-operative hospital stay and mortality was significantly higher in patients with invasive infection (p<0.05). Acinetobacter baumannii was isolated from multiple sites (p<0.05) and repeatedly from the same site (p<0.001) in a significantly higher number of patients with invasive infections. Mortality was high in the patients infected with Acinetobacter baumannii. Even amongst the infected group, the patient shaving meningitis showed a higher mortality as compared to the patients having bacteraemia.

Acinetobacter↗
PubMed2000

Infantile-onset leukoencephalopathy with discrepant mild clinical course.

Four children characterised by megalencephaly and cerebral leukoencephalopathy with infantile onset, defined on the basis of clinical and neuroimaging findings are reported. The course of the disease is characterised by stabilization of the macrocephaly and slow clinical deterioration. The CT scan findings include supratentorial diffuse hypodensities in the white matter and swelling. The characteristic MRI findings include the discrepant severity in comparison with the clinical picture, diffuse supratentorial white matter abnormalities with subcortical cysts. The basic defect of the disease is unknown. Considering the high rate of consanguinity among the parents and the presence of two affected sibs in one family, an autosomal recessive inheritance is assumed. We report four unrelated cases of this entity.

Brain↗
PubMed2000

Fraser-cryptophthalmos syndrome.

Fraser or Cryptophthalmos syndrome is a variable syndrome to the extent that cryptophthalmos might not be present in all cases. However, the main features are a "hidden eye", other craniofacial abnormalities, renal abnormalities, syndactyly and abnormal genitalia. It may be classified as isolated cryptophthalmos or cryptophthalmos sequence and cryptophthalmos syndrome. The cryptophthalmos syndrome has an autosomal recessive mode of inheritance. Isolated cryptophthalmos has been reported as an autosomal dominant trait. Prenatal diagnosis is possible using ultrasonography and fetoscopy. We report three cases of cryptophthalmos. One with renal agenesis had cryptophthalmos syndrome and the other two had isolated cryptophthalmos or cryptophthalmos sequence.

Abnormalities, Multiple↗
PubMed2000

Plumbism--a mimicker of common childhood symptoms.

Lead poisoning is a preventable entity that can affect almost every system of the body. Its toxic effects range from subtle common childhood symptoms to even death. In a prospective study forty six children with at least one symptom compatible with plumbism were enrolled after screening patients attending medical services at AIIMS. The work-up included a detailed clinical history and examination including intelligence quotient (IQ) and behaviour. Blood lead levels were estimated in all with due precautions. Their ages ranged from 2.5 to 18 years and M:F ratio was 2:1. The common symptoms included unexplained recurrent colic (39), anorexia (21), sporadic vomiting and constipation (13), pigmented gum lines (11), growth failure (11), history of pica (9) etc. None of the children had acceptable blood lead levels (< 10 micrograms/dl). Ninety three per cent children had levels > 20 micrograms/dl and 47.8% had > 45 micrograms/dl. Statistically significant correlation with blood lead levels was seen with most symptoms. Plumbism can mimic common childhood illnesses and should be investigated in children with a clinically compatible profile. Community awareness with regard to pica reduction may be an important preventive measure. This silent epidemic needs to be aggressively tackled by a multipronged approach.

Adolescent↗
PubMed2000

I-cell disease (Mucolipidosis II).

I-cell disease (Mucolipidosis II) is one of the lysosomal storage diseases which presents in the neonatal period, and within six months will phenotypically resemble the severe forms of the group of disorders called the "mucopolysaccharidoses" but without mucopolysacchariduria. In Mucolipidosis II, fibrocytes exhibit "abnormal lysosomes". Activities of several lysosomal enzymes are low in fibroblast cultures but high in mucolipidosis II serum. We present a patient with I-cell disease diagnosed on the basis of clinical, radiological and biochemical features. The mother of this child was pregnant and the fetus was also found to be affected.

Child, Preschool↗
PubMed2000

Juvenile neuronal ceroid lipofuscinosis.

A case of juvenile neuronal ceroid lipofuscinosis (JNCL) diagnosed on the basis of clinical features, electrophysiologic studies and skin electron microscopy is reported. JNCL was suspected on the basis of characteristic symptoms including progressive loss of vision, seizures, mental retardation and motor disabilities. Diagnosis was confirmed by neurophysiological and biopsy studies. The disease is caused by 23 different mutations in a gene recently isolated on chromosome 16 p11.2-12.1. Although universally fatal, characterisation of mutations can help in prenatal diagnosis in future pregnancies.

Adolescent↗