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Biomedical subjects

A Chacko

Publications and source records attributed to A Chacko.

At least 19 recordsLinked to original sources
PubMed2001

Severe autosomal recessive rippling muscle disease.

Rippling muscle disease (RMD) has previously been reported as a skeletal myopathy that was attributed to a defect in the sarcomere. Here we report a new form of RMD that is more severe, characterized by fatal arrhythmic cardiomyopathy and delayed bone age. Mortality has previously not been associated with RMD. With this report we hope to raise awareness that a subset of patients with this clinical entity are predisposed to severe cardiac disease.

Adolescent↗
PubMed2001

West syndrome: a university hospital based study from Oman.

Forty-four children (20 male: 24 female) with West syndrome (infantile spasms, mental retardation/regression and hypsarrhythmia) diagnosed at Sultan Qaboos University Hospital (Pediatric Neurology Division of the Department of Child Health) are reported, with thirty-four (77.3%) children constituting the symptomatic group. All children were followed up for an initial 1 year at this hospital. Thirty-seven cases (84%) still continue their follow-up with us. The age of onset ranged from 1 to 14 months (mean, 6.0 months). Developmental delay before the onset of infantile spasms was noted in 29 (65.9%) children. Brain computed tomography was abnormal in 29 (65.9%). Sodium valproate and vigabatrin were the most often used drugs, though other antiepileptic drugs were also used. Nine (24.5%) children achieved good seizure control, out of which five have normal development. Only one child could be weaned off antiepileptic drugs completely. There was one death in the whole series, related to aspiration pneumonia.

Anticonvulsants↗
PubMed2001

Enhanced visualization processing: effect on workflow.

RATIONALE AND OBJECTIVES: Soft-copy viewing of digital radiographs allows for image processing to improve visualization of anatomy and lesions, but it can take more time than film-based viewing. Enhanced visualization processing (EVP) was developed to increase the latitude of an image without reducing the vital contrast, potentially reducing the need for the radiologist to manipulate images. This study examined the influence of processing radiographic images with EVP on workflow in a picture archiving and communications system (PACS). MATERIALS AND METHODS: Portable computed radiographic chest images were obtained and processed either with EVP or without. A security camera with a videocassette recorder was positioned above the PACS workstation. Four radiologists reviewed the images during their normal work schedule. The current diagnostic image was used to determine if the case contained EVP or non-EVP images. The videotapes of the sessions were reviewed to determine diagnostic viewing times and how zoom and/or window and level manipulation was used. RESULTS: Viewing time was significantly longer for the non-EVP than the EVP cases. The difference occurred with all readers. Window and level manipulation was used on 35% of the EVP and 41% of the non-EVP images. Zoom was used on 64% of the EVP and 69% of the non-EVP images. Average time spent using zoom and window and level manipulation was significantly shorter with the EVP than with the non-EVP images. CONCLUSION: EVP of chest images displayed on PACS monitors significantly improved workflow as measured by viewing time. EVP decreased use of window and level manipulation and zooming and the amount of time each one was used.

Humans↗
PubMed2001

Vigabatrin associated retinal dysfunction in children with epilepsy.

BACKGROUND: Recent reports have established that eye changes occur in patients treated with vigabatrin. AIM: To identify the eye changes associated with vigabatrin, based on a prospective study of children treated for seizures. METHODS: Twenty nine children on vigabatrin (mainly as add on therapy) were followed up for 6.5 years. Ophthalmic examination was performed before starting treatment and then six monthly in the outpatient clinic. RESULTS: Twenty one children fulfilled the inclusion criteria. Most had epileptic syndromes with infantile spasms-namely West syndrome, Lennox-Gastaut syndrome, and partial seizures. Vigabatrin dose was 25-114 mg/kg/day (mean 55.8); duration of therapy was 6-85 months (mean 35.7). Four children (19%) developed eye changes (retinal pigmentation, hypopigmented retinal spots, vascular sheathing, and optic atrophy). Visual evoked potentials were abnormal in 16 children. Electroretinography and electro-oculography, which could have picked up eye changes in early stages, were not performed, as this facility was not available. CONCLUSIONS: Vigabatrin causes eye damage. Most children with epileptic syndromes on vigabatrin cannot complain of their eye problems, hence 3-6 monthly ophthalmic follow up is strongly advised, along with regular electroretinography, electro-oculography, and visual evoked potentials if possible.

Adolescent↗
PubMed2001

Aicardi-Goutieres syndrome in siblings.

Two siblings with familial encephalopathy, calcification of the basal ganglia, and cerebrospinal fluid lymphocytosis, constituting the triad of Aicardi-Goutieres syndrome, are reported. This syndrome resembles congenital intrauterine infections, which must be meticulously excluded. Aicardi-Goutieres syndrome is extremely rare and is being reported from the Arab world for the first time to our knowledge.

Atrophy↗
PubMed2001

Corpus callosum agenesis.

OBJECTIVE: The objectives are to analyse corpus callosum agenesis in children with various neurological problems in a hospital set-up, and to study the neurological and systemic abnormalities associated with this condition. METHODS: The children with various neurological problems who underwent computerized tomography brain from January 1993 to December 1997, and were found to have corpus callosum agenesis, formed the subjects of this study. These children were examined for any syndromic association, congenital infections or metabolic defects. RESULTS: Out of 2164 children who underwent computerized tomography brain, 22 had corpus callosum agenesis (1%). Most cases were not syndromic and 64% were males. Epileptic disorders were noted in about one third of cases. CONCLUSION: Corpus callosum agenesis is an important anomaly in children with neurodevelopment handicaps, usually detected by neuroradiology.

Agenesis of Corpus Callosum↗
PubMed2000

New facility picture archiving and communication system implementation strategy.

Strategies for deployment of picture archiving and communications systems (PACS) in new hospitals often involve the establishment of initial PACS operations. Such a strategy is flawed in the sense that the clinical and radiological users must adapt to PACS operations, while being faced with several other new facility learning curves. This increases the complexity and risk of the radiological services. A strategy of implementing PACS operations in the old facility and performing a zero-downtime transition into the new facility offers several advantages to this method. The successful undertaking of such a project will support not only the physical movement of the existing PACS, but the accomplishment of other re-engineering goals associated with the new hospital. This report will describe the strategy used in two successful transitions of PACS into newly constructed hospitals.

Hospital Design and Construction↗
PubMed2000

The filmless radiology reading room: a survey of established picture archiving and communication system sites.

The purpose of this study was to survey radiologists experienced in soft-copy diagnosis using computer workstations about their current reading room environment, their impressions of the efficacy of their reading room design, and their recommendations based on their experience for improvement of the soft-copy reading environment. Surveys were obtained from radiologists at seven sites representing three major picture archiving and communication system (PACS) vendors throughout the world that have had extensive experience with soft-copy interpretation of radiology studies. The radiologists filled out a detailed survey, which was designed to assess their current reading room environment and to provide them with the opportunity to make suggestions about improvement of the PACS reading rooms. The survey data were entered into a database and results were correlated with multiple parameters, including experience with PACS, types of modalities interpreted on the system, and number of years of experience in radiology. The factors judged to be most important in promoting radiologist productivity were room lighting, monitor number, and monitor brightness. Almost all of the radiologists indicated that their lighting source was from overhead rather than indirect or portable light sources. Approximately half indicated they had the capability of dimming the brightness of the overhead lighting. Most radiologists indicated that they were able to adjust room temperature but that they did not have individual temperature controls at their workstations. The radiologists indicated that the most troublesome sources of noise included background noise, other radiologists, and clinicians much more than noise from computer monitors, technologists, or patients. Most radiologists did not have chairs that could recline or arm rests. Most did have wheels and the capability to swivel, both of which were judged important. The majority of chairs also had lumbar support, which was also seen to be important. Radiologists commonly adjusted room lighting and their reading chair, but rarely adjusted room temperature or monitor brightness. The median number of hours spent at the workstation before taken a "break" was 1.5. Common recommendations to improve the room layout included compartmentalization of the reading room and availability of the hospital/radiology information system at each workstation. The survey data suggest several areas of potential improvement based on radiologists' experience. Optimization of soft-copy reading room design is likely to result in decreased fatigue and increased productivity.

Attitude of Health Personnel↗
PubMed2000

Munchausen syndrome by proxy.

Five children (3F:2M), in the age group 1 years to 11 years, with Munchausen syndrome by proxy are reported from the Sultanate of Oman. They were seen over a four years period from 1996-1999. In all these children, the mother came up with history of uncontrolled epilepsy. Carbamazepine was the most common antiepileptic drug used. One of these children remained hospitalized elsewhere for nearly 9 months, as a case of uncontrolled status epilepticus. It took 18 months to 6 years (mean 2.8 years) to establish the diagnosis and the mother was the offender in all. The main lead to diagnosis, was the disparity between history and clinical presentation to hospital. The carbamazepine levels were several times above the upper limit of therapeutic range. Munchausen syndrome by proxy very much exists here, but is possibly less recognized and needs immediate attention to formulate policies to identify and manage these children. It is necessary to create awareness even in the medical community, to recognise this problem. There is an urgent need to develop a child protection council at the national or regional level.

Adult↗
PubMed2000

Long term intubation and successful weaning in two children with Guillain-Barre syndrome.

No definite criteria exists in Guillian-Barre syndrome in children regarding prolonged ventilation through an endo-tracheal tube without tracheostomy and successful weaning using a T-piece. Here we report two such cases of Guillian-Barre syndrome requiring prolonged intubation for 56 days and ventilation for 30 days and ultimately successfully weaning them using the T-piece. Both the children eventually made a complete recovery, highlighting the point that in children prolonged intubation and ventilation using the portex tube is equally good, if not, better than tracheostomy with its attendant risks.

Child↗
PubMed2000

Dandy-Walker syndrome in association with neurofibromatosis in monozygotic twins.

Dandy-Walker syndrome in monozygotic twins is reported. The twins reported, presented with delayed development, big head and dysmorphic features. In addition, there were significant cafe-au-lait spots on the trunk and other minor features consistent with the diagnosis of neurofibromatosis. To the best of our knowledge, Dandy-Walker syndrome in combination with neurofibromatosis in monozygotic twins has not been previously reported.

Dandy-Walker Syndrome↗
PubMed1999

Cytomegalovirus infection of the human gastrointestinal tract.

BACKGROUND: Current interest in cytomegalovirus (CMV) is largely due to an increase in the number of cases of acquired immunodeficiency syndrome and organ transplantation in recent years. The proper recognition of CMV-infected cells in gastrointestinal mucosal biopsies is critical for effective treatment of this condition. METHODS: A total of 6580 endoscopic mucosal biopsies from 6323 patients in the 8-year period (1989-1996) were examined for CMV inclusion bodies. The endoscopic appearance and particularly the presence of ulcers were also analysed. RESULTS AND CONCLUSIONS: The prevalence of cytomegalovirus (CMV) inclusions was 9 per thousand in the gastrointestinal mucosal biopsies from an unselected group of patients. Of the 54 patients with CMV infection, 37 were immunocompromised and 17 apparently immunocompetent. Typical Cowdry inclusions and atypical inclusions were present, the latter more frequently in immunocompromised patients. The maximum prevalence of inclusions was in the oesophageal mucosa in immunocompromised individuals.

Adolescent↗
PubMed1999

Use of serology, the urease test & histology in diagnosis of Helicobacter pylori infection in symptomatic & asymptomatic Indians.

Age-specific prevalence of IgA and IgG antibodies in 714 subjects without gastrointestinal complaints aged 6 months to 90 yr was measured by an enzyme linked immunoassay using an acid-glycine extract of H. pylori as the antigen. The urease test and histology were used for the diagnosis of H. pylori infection in 83 subjects with a clinical diagnosis of dyspepsia, and these results were compared with measurement of IgG, IgA and IgM antibodies. The age specific prevalence of IgG and IgA antibodies respectively was 57 and 43 per cent for subjects aged 6 months to 4 yr and showed an increase with age to a maximum of 90 per cent for IgG in subjects > 60 yr of age and to 87 per cent for IgA in subjects between 51 and 60 yr. In symptomatic patients, there was a high degree of correlation between severity of H. pylori infection on histopathological examination and IgG (P < 0.02) levels. The use of IgG and IgA estimation could have identified H. pylori infection without endoscopy in 50 of the 83 patients. Serology for IgG and IgA antibodies against H. pylori may play a major role in decreasing the need for endoscopy, but cut-off values must be determined for each assay based on the prevalence of antibodies in the population.

Adolescent↗
PubMed1999

Efficacy of non-breath-hold magnetic resonance cholangiography at midfield strength.

The efficacy of non-breath-hold magnetic resonance (MR) cholangiography at mid-field strength (0.5 Tesla) was evaluated for delineating biliary anatomy and the cause and extent of biliary obstruction. We performed 65 MR cholangiograms on a mid-field 0.5 Tesla MR unit and correlated them with contrast cholangiography and/or surgery. MR cholangiography was found to be both sensitive and specific in the detection of biliary obstruction and in the definition of its cause (sensitivity 98%, specificity 100%, positive predictive value 100%, negative predictive value 85.7%, accuracy 98%). MR cholangiography accurately predicted the level of obstruction in 94 per cent of strictures. Normal caliber intra-hepatic biliary radicles were visualised in only 6 per cent of the MR cholangiograms. In contrast, 94 per cent of dilated intrahepatic biliary radicles were demonstrated. The confluence, and right and left hepatic ducts were visualized in 98 per cent; the gall bladder in 65 per cent; the cystic duct in 45 per cent and the cystic duct insertion in 25 per cent. The extrahepatic bile duct was seen in 82.7 per cent. A normal caliber pancreatic duct was seen in 18 per cent while a dilated pancreatic duct was seen in 86 per cent. The pancreatico-biliary junction was visualised in 7 per cent. Non-breath-hold MR cholangiography at midfield strength is a highly accurate method of evaluating the cause and level of biliary obstruction, comparable to high-field MR cholangiography. The spatial resolution however is inadequate for the detection of variations in biliary or pancreatic ductal anatomy when the ducts are of normal caliber.

Adolescent↗
PubMed1998

External hydrocephalus--a report of 16 cases from Oman.

Sixteen cases of external hydrocephalus (EH) were seen from January 1993 to June 1995. There were 13 (81.3 per cent) male and three female children. Fourteen (87.5 per cent) were under 12 months of age. Three siblings with EH were seen in one family. All but three of the 16 recovered over time without medical or surgical intervention. These three needed cerebral decongestants in the acute phase.

Age Distribution↗