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Biomedical subjects

Veena Kalra

Publications and source records attributed to Veena Kalra.

16 recordsLinked to original sources
PubMed2004

Diagnostic dilemmas in fulminant subacute sclerosing panencephalitis (SSPE).

This report describes an eleven-year-old boy with atypical features of subacute sclerosing panencephalitis (SSPE), a rare complication of measles. He had only visual symptoms for 2 months followed by rapid neurological worsening to a vegetative state in 10 days. A diagnosis of SSPE was made based on the history of measles, characteristic ocular findings, compatible magnetic resonance imaging and electroencephalographic changes, and elevated ratio of cerebrospinal fluid to serum anti-measles antibody titers.

Child↗
PubMed2004

Intermittent hyperammonemic encephalopathy in a child with ornithine transcarbamylase deficiency.

Ornithine transcarbamylase (OTC) deficiency is an X-linked disorder and the most common inherited cause of hyperammonemia. Clinical manifestations are more severe in hemizygous males who often present in neonatal period. Heterozygous females may be asymptomatic until juvenile or adulthood. Fluctuating concentration of ammonia, glutamine and other excitotoxic amino acids result in a chronic or episodically recurring encephalopathy. The authors report a heterozygous female with OTC deficiency who presented with recurrent encephalopathy.

Brain Diseases, Metabolic↗
PubMed2004

Erythema annulare centrifugum with autoimmune hepatitis.

Erythema annulare centrifugum (EAC) is a rare disease entity characterized by dense perivascular lymphocytic infiltrates in dermis. It has been associated with a few conditions, though its etiology is largely unknown. To our knowledge there has been no reported association with autoimmune hepatitis described earlier. This child also was positive for hepatitis C virus antibodies, though HCV RNA was negative. We should keep in mind the false positivity of hepatitis C antibodies before deciding on its therapy.

Erythema↗
PubMed2004

Familial hypercholesterolemia.

Abnormalities in the lipid profile though uncommon in pediatric practice pose an increased risk for developing heart disease. Studies suggest that adult cardiovascular disease has its roots in children and young adults. A significant correlation between atherosclerotic changes in children and young adults and total and LDL cholesterol levels also exists. The association is particularly true for Familial Hypercholesterolemia. We report a young boy aged 14 years who presented with all the features of Familial Hypercholesterolemia.

Adolescent↗
PubMed2003

Magnetic resonance spectroscopy in pediatric neurology.

In the last three decades a range of non-invasive biophysical techniques have been developed, of which Magnetic Resonance (MR) has proved to be the most versatile. Its non-invasive and safe nature has made it the most important diagnostic and research tool in clinical medicine. MR Spectroscopy (MRS) is the only technique in clinical medicine that provides non-invasive access to living chemistry in situ. This article focuses mainly on proton MRS in brain and also phosphorus MRS in calf muscle, with particular reference to the pediatric population, the normal spectrum and its use in various disease conditions in the practice of pediatric neurology. Few representative case studies among different disease groups have also been detailed.

Adolescent↗
PubMed2003

Management of childhood epilepsy.

Epilepsy is a common childhood neurological morbidity needs careful evaluation, relevant investigations and precise therapy with appropriate antiepileptic drugs for optimal duration. Majority of childhood epilepsy remits with antiepileptic drugs and should be managed in the community. Practising pediatricians must counsel the family for possible etiology of epilepsy, compliance during treatment and possible outcome. It is important to distinguish pseudo seizures and nonepileptic events from true epilepsy, as this would reduce the burden of unwanted medication. It is also equally important to enable ourselves to recognize early predictors of intractability and refer them to appropriate referral units. Epilepsy is a social and an economic burden for the family. Preventive strategies by improved perinatal care, prevention and managemnt of neuro infections and infestations which will mitigate epilepsy burden in the community are essential. In this context it is important to familarize appropriate and relevant use of investigations. Inappropriate antiepileptic drug usage is common in the community and drug therapy should be rationalized. Epilepsy management requires a close interaction between the patient, family and the treating physician and a concerted effort is essential.

Anticonvulsants↗
PubMed2003

Schinzel acrocallosal syndrome.

Acrocallosal syndrome (ACLS), also known by its synonyms: Schinzel Acrocallosal syndrome and Hallux duplication, Postaxial polydactyly and absence of corpus callosum, is a rare genetic disorder that is apparent at birth. Although autosomal recessive inheritence has been suggested, ACLS often appears to occur sporadically. Typical characteristics of ACLS are hypoplasia/agenesis of corpus callosum, moderate to severe mental retardation, characteristic craniofacial abnormalities, distinctive digital malformations and growth retardation. It has not been reported from India so far. This article reports a 5-month-old boy with combination of abnormalities consistent with acrocallosal syndrome.

Abnormalities, Multiple↗
PubMed2003

Stroke in children.

Stroke is defined as the sudden occlusion or rupture of cerebral arteries or veins resulting in focal cerebral damage and neurological deficits. Forms of stroke resulting from vascular occlusion are arterial ischemic stroke (AIS) and sinovenous thrombosis (SVT) and those resulting from vascular rupture are called hemorrhagic stroke. Stroke in children is relatively rare and frequently results in a lack of recognition and delay in diagnosis. The etiologies of stroke in children are legion and multiple risk factors coexist unlike unifactorial etiology in adults. Heart disease whether congenital or acquired, malformations, metabolic and hematological disorders and vasospastic conditions like migraine are seen more often in childhood strokes. The purpose of diagnostic evaluation includes confirmation of the presence of a cerebrovascular lesion, exclusion of other types of neurological dysfunction and identification of etiology of the stroke. The treatment of stroke in children has been primarily directed toward stabilizing systemic factors and management of the underlying causes. Various antithrombotic and non antithrombotic therapies are discussed. The use of anticoagulant therapy appears to be increasing in pediatric AIS. Mortality after stroke in children ranges from 20% to 30% depending on the location and the underlying cause. Residual neurological dysfunction is present in more than 50% of survivors.

Adolescent↗
PubMed2003

Efficacy of albendazole and short-course dexamethasone treatment in children with 1 or 2 ring-enhancing lesions of neurocysticercosis: a randomized controlled trial.

OBJECTIVE: To determine the efficacy of albendazole plus dexamethasone in children with 1 or 2 ring-enhancing lesions (by computed tomography scan) on resolution of lesions and recurrence of seizure. STUDY DESIGN: Randomized controlled open trial. METHODS: Children of either sex, 1 to 14 years of age, with seizures and 1 or 2 ring-enhancing lesions <20 mm in diameter on computed tomography scan, likely to have neurocysticercosis, were assigned to treatment l groups. Children assigned to the treatment group (n=61) were given 0.15 mg/kg per day dexamethasone for 5 days plus 15 mg/kg per day albendazole for 28 days, starting on the third day of dexamethasone. Control group (n=62) children were given neither dexamethasone or albendazole. Anti-epileptic therapy was given to both the study groups. RESULTS: The lesions resolved completely or partially in more children in the treated group compared with the control group (79% versus 57%; P=.02). The proportion of children who had seizures was significantly lower in the treated group compared with the control group at 3 months (10% versus 32%; P=.006) and 6 months (13% versus 33%; P=.03). CONCLUSIONS: Albendazole plus dexamethasone increased complete or partial resolution of lesions and reduced the risk of subsequent recurrence of seizures among children with neurocysticercosis who had with seizures and 1 or 2 ring-enhancing lesions on computed tomography.

Adolescent↗
PubMed2003

Blood lead levels and risk factors for lead toxicity in children from schools and an urban slum in Delhi.

This cross-sectional study was conducted to estimate the mean blood lead levels (BLL) and prevalence of lead toxicity in a representative sample of schoolchildren and children residing in an urban slum. In addition, the association of potential environmental risk factors with elevated BLL was studied. Children aged 4-6 years were selected from schools of the South zone of Delhi (n = 125) and from an urban slum (n = 65). Risk factors were recorded using a pre-tested questionnaire and blood lead and zinc protoporphyrin (ZPP) levels were estimated. The mean BLL was 7.8 microg/dl (SD 3.9) and the proportion of children with blood lead > or = 10 microg/dl was 18.4 per cent. Distance of the residence or school from a main road appeared to be associated with higher blood lead concentrations, but these differences were not statistically significant. In our setting, vehicular pollution may be a major contributing factor in lead contamination of the environment.

Child↗
PubMed2003

Gamma-sarcoglycanopathy.

The best known muscular dystrophies are X-linked dystrophinopathies. A clinically and genetically heterogeneous group presenting with weakness of the pelvic and shoulder girdles is that of the limb-girdle muscular dystrophies (LGMDs). Sarcoglycanopathies (SGPs) are autosomal recessive LGMDs. We report a rare case of primary gamma-sarcoglycanopathy (SGP) which emphasizes the evolving concept of dystrophinopathy to sarco-glycanopathy.

Biopsy, Needle↗
PubMed2002

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.

Anticonvulsants↗
PubMed2002

Hypothalamic hamartoma, gelastic epilepsy, precocious puberty--a diffuse cerebral dysgenesis.

Childhood epileptic syndrome characterized by early onset gelastic seizures, hypothalamic hamartoma and precocious puberty is well recognized though rare. We report association of agenesis of corpus callosum, Dandy-Walker complex and heterotopic gray matter with this childhood epileptic syndrome which is hitherto an unreported association. The child showed a satisfactory response to gonadotropin releasing hormone agonist.

Agenesis of Corpus Callosum↗