Biomedical subjects

G Gupte

Publications and source records attributed to G Gupte.

7 recordsLinked to original sources

PubMed2003

Acute disseminated encephalomyelitis: a review of 18 cases in childhood.

OBJECTIVE: Acute disseminated encephalomyelitis (ADEM) is a treatable inflammatory demyelinating disorder seen more commonly in children than in adults. It typically presents to general paediatricians, often, like encephalitis, with non-specific cerebrospinal fluid findings. The brain computerized tomography scan is usually normal, so is falsely reassuring and delays the diagnosis, which might result in considerable morbidity. The present study was initiated to report on the various modes of presentation and raise the awareness of the diagnosis of ADEM among general paediatricians. METHODS: A retrospective review of the case notes of 18 children with a diagnosis of ADEM established in a tertiary referral centre from 1995 to 2000 was undertaken with particular reference to clinical features, investigations and treatment. RESULTS: The most common presenting features were ataxia (10 cases), followed by headache (eight cases) and weakness (five cases). Magnetic resonance imaging (MRI) of the brain was needed to confirm the diagnosis in all 18 children. Treatment usually included a course of intravenous methylprednisolone followed by a tapering dose of oral prednisolone over several weeks. Although the outcome for most of the children was generally good, two relapsed after cessation of steroids and five children had ongoing disabilities. CONCLUSIONS: The investigation of choice for establishing the diagnosis of ADEM was MRI of the brain. Other investigations were seldom helpful in reaching the diagnosis. Early diagnosis and prompt treatment of ADEM will probably reduce morbidity.

Acute Disease↗

PubMed2003

Acute disseminated encephalomyelitis: recognition in the hands of general paediatricians.

Acute disseminated encephalomyelitis will often present to the general paediatrician as an acute polysymptomatic encephalopathy, and initially the diagnosis may not be clear. A brain MRI scan is essential in establishing the diagnosis and so enabling appropriate advice and treatment to be given. Multicentre clinical audit of outcome and controlled therapeutic trials are needed to secure an evidence base for current practice.

Anti-Bacterial Agents↗

PubMed2001

Prothrombotic disorders and abnormal neurodevelopmental outcome in infants with neonatal cerebral infarction.

BACKGROUND AND PURPOSE: The aim of this study was to evaluate the occurrence of prothrombotic disorders in a well-characterized cohort of infants with neonatal stroke and to document any association of prothrombotic disorders with the type of infarct seen on magnetic resonance imaging (MRI) and clinical outcome. METHODS: Twenty-four infants with perinatal cerebral infarction confirmed by neonatal MRI were enrolled in the study. All the infants and, when possible, both parents were tested to identify inherited and acquired prothrombotic disorders. RESULTS: None of the infants had a significant bleeding diathesis, but 10 (42%) had at least 1 prothrombotic risk factor. Five children showed heterozygosity for factor V Leiden, and 6 had high factor VIIIc concentrations. There was a striking association between the occurrence of these abnormalities and both the presence of cerebral hemorrhage on MRI and poor neurologic outcome. Eight of the 11 patients (73%) with hemiplegia or global developmental delay had factor V Leiden and/or raised factor VIIIc, whereas only 1 of the 13 patients (8%) with normal outcome had any prothrombotic risk factors. In particular, all 5 infants with factor V Leiden had hemiplegia, compared with only 4 of the 19 infants without factor V Leiden (21%). CONCLUSIONS: These data suggest that the presence of prothrombotic risk factors and, in particular, of the factor V Leiden mutation, is significantly associated with poor outcome after perinatal cerebral infarction.

Activated Protein C Resistance↗

PubMed1997

Isolation and characterization of rcsB mutations that affect colanic acid capsule synthesis in Escherichia coli K-12.

Regulation of colanic acid polysaccharide capsule synthesis in Escherichia coli requires the proteins RcsC and RcsB, in addition to several other proteins. By sequence similarity, these two proteins appear to be members of the two-component sensor-effector regulatory family found in bacteria. The present study characterizes the functional domains of RcsB. We have isolated mutations in rcsB that are able to suppress an rcsC "up" mutation (i.e., leading to increase in cps transcription) that normally results in constitutive expression of the capsule. In addition, constitutive capsule mutations in rcsB have been isolated. From the characterization of the mutants and by analogy to the three-dimensional structure of CheY, we have begun to define different domains of RcsB and to assign functions to them. A few of the constitutive capsule mutations were localized in an acidic pocket that has been proposed to play a crucial role in phosphorylation of RcsB. As seen in other two-component systems, an aspartate-to-glutamate substitution at the presumed site of phosphorylation of RcsB resulted in constitutive capsule expression. Lastly, several of our rcsB mutants were found to be allele specific (rcsC137 specific) for rcsC, suggesting a physical as well as functional interaction between RcsC and RcsB proteins.

Amino Acid Sequence↗

PubMedDate not supplied

Is serum gentamicin level a good predictor of graft injury in intestinal transplantation?

INTRODUCTION: Following intestinal transplant (SBT), the early diagnosis and treatment of rejection is a major management aim. The diagnosis of rejection is based on histology of stomal biopsies. Oral gentamycin (2.5 mg/kg) was used for selective decontamination of the digestive system. Our hypothesis was that gentamycin might be absorbed in the presence of graft dysfunction. AIM: Our goal was to assess the correlation between serum gentamycin level and the health of the intestinal graft. SUBJECTS AND METHODS: Among 33 SBT performed from 1993 to 2005, serum gentamycin levels were performed once weekly or more often when there was a suspicion of rejection. All data were analyzed retrospectively. RESULTS: Adequate trough levels were achieved for only 23 patients, six of whom had histologically proven rejection and only one did not have a raised gentamycin content. Five patients with raised levels but no rejection included two with severe intestinal ischemia and three with bowel obstruction/ileus. Four of the five patients required laparotomies. CONCLUSION: We concluded that in our study raised serum gentamycin levels were a good predictor of rejection or significant injury to the graft.

Biomarkers↗

PubMedDate not supplied

Wildervanck syndrome (cervico-oculo-acoustic syndrome).

Wildervanck syndrome i.e. cervico (Klippel-Feil anomalad) -oculo (Duane-Stilling-Turk phenomenon with bilateral abducens palsy)-acoustic (deafness) is a rare syndrome. We report here 4 cases diagnosed as Wildervanck syndrome and analyse their findings. One patient had an an atrial septal defect. Such association of congenital heart disease with Wildervanck syndrome has not been reported previously.

Abnormalities, Multiple↗

PubMedDate not supplied

Pseudoachondroplastic dysplasia.

Pseudoachondroplasia is a heterogeneous inherited skeletal dysplasia in which dwarfism is a major feature. We report here a case of a 7 year old girl misdiagnosed as rickets, who presented with short stature, lordosis, genu varum and flexion deformities at both the elbows. Skeletal survey revealed epiphyseal and metaphyseal irregularities. A review of literature is also presented.

Achondroplasia↗