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Biomedical subjects

R L Koul

Publications and source records attributed to R L Koul.

At least 19 recordsLinked to original sources

Poliomyelitis in Oman. I. The last outbreak?

Since 1988, the Sultanate of Oman has experienced three outbreaks of paralytic poliomyelitis. The last outbreak occurred in December 1993 and involved two children aged 10 months and 4 1/2 years. The children had received five and four doses, respectively, of trivalent oral polio vaccine (OPV) and lived in the same village. Serum neutralizing antibody tests suggested that paralytic polio in these children was due to poor antibody response to OPV. Wild poliovirus type 1 was isolated from both patients, as well as from seven of ten close contacts of the older child, and one of eight contacts of the younger child. All contacts had received three to six doses of OPV. Genomic sequence studies indicated that the virus isolates belonged to a genotypic group prevalent in southern and western Asia, but differed markedly from virus isolated during the 1988/89 outbreak, suggesting another importation of poliovirus. In response to the outbreak, supplementary immunization with OPV was given to children <6 years of age, initially in the affected district, and subsequently to children in the whole country. This study demonstrates that immunization with three to six doses of OPV did not prevent infection with wild poliovirus. In those children with sub-optimal response to OPV, infection resulted in paralytic poliomyelitis. The outbreak remained localized in one village, indicating that the outbreak control measures were effective.

Antibodies, Viral↗

Poliomyelitis in Oman. II. Toward eradication.

In the past decade, the Sultanate of Oman has experienced three outbreaks of paralytic poliomyelitis--a widespread polio type 1 epidemic in 1988/1989, four cases of polio type 3 in three different regions in 1991, and a localized type 1 outbreak in 1993. The lessons learnt from each of these epidemics have guided us to modify and improve our polio eradication activities. Currently, these activities include administration of five primary and three booster doses of trivalent oral polio vaccine, yearly national immunization campaigns (NIDs) since 1995 with coverage of >90%, localized immunization campaigns, acute flaccid paralysis (AFP) surveillance which involves reporting of all cases by facsimile to the Department of Surveillance within 24 h of detecting a case and weekly zero reporting from 22 sentinel sites, and virological testing of stool specimens of all AFP cases and their close contacts at the national, World Health Organization accredited laboratory. The cumulative success of these activities has resulted in Oman being free from polio for the past 6 years. However, the possibility of importation of wild poliovirus, particularly from southern and western Asia still exists.

Adolescent↗

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↗

An infant with seizure-related bradycardia and asystole.

We describe an infant girl with ictal bradycardia and asystole who died during an episode of seizure, despite anti-epileptic therapy and permanent cardiac pacemaker implantation. The mechanism of ictal bradycardia and the need to recognize it are therefore discussed. Timely cardiac intervention and adequate anticonvulsant therapy are essential for successful management.

Anticonvulsants↗

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↗

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↗

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↗

Continuous midazolam infusion as treatment of status epilepticus.

In a tertiary referral centre, midazolam infusion was tried as treatment for 20 children with status epilepticus over a period of two years. The mean age of the children was 4.07 years. Twelve children with refractory status epilepticus had received intravenous or per rectal diazepam and intravenous phenytoin/ phenobarbitone or both before midazolam was given (0.15 mg/kg bolus followed by 1-5 micrograms/kg/min infusion). Eight children required only midazolam to control the established status epilepticus. The seizures were controlled in 19 children. The mean time required for complete cessation of seizures was 0.9 hours. The mean infusion rate required was 2.0 micrograms/kg/min. All children had regained full consciousness by a mean of 5.1 hours after discontinuation of midazolam treatment. No metabolic derangement or compromise of vital functions was noted in any of the children. Midazolam infusion is thus an effective and safe therapeutic approach for the management of childhood status epilepticus.

Adolescent↗

Clinical and radiological features of juvenile onset olivopontocerebellar atrophy.

Olivopontocerebellar atrophy (OPCA) is uncommon in childhood. We encountered 17 consecutive patients with clinical and radiological features of OPCA with a juvenile onset (mean onset age 8.2 years). These patients were heterogenous on clinical and genetic profiles, but formed three major groups. The largest group consisted of eight patients with OPCA and pigmentary retinal degeneration. The other major groups seen were OPCAs with peripheral neuropathy and OPCA with pyramidal features. Other clinical features seen were extrapyramidal signs and lower cranial nerve palsies. The CT scan showed varying degrees of cerebellar and pontine atrophy. A more precise resolution of the underlying cause of these disorders requires characterization at the genetic and molecular level and awaits further study.

Adolescent↗

Neuropsychiatric manifestations of typhoid fever.

Various neuropsychiatric syndromes in typhoid fever were seen in nine patients of multiple drug resistant (both in vitro and in vivo) Salmonella typhi infection of a total of 270 suspected cases in the last two years. All but one patient received oral norfloxacin (400 mg 12 hourly) for two weeks with complete recovery, while the other patient died. Bacteremia and toxemia seem to be the only common factor responsible for neuropsychiatric complications.

Adolescent↗

Eating epilepsy.

Seventy eight cases of eating epilepsy seen from December 1982 to June 1989 are reported. Mastication of food produced seizures in 77 cases (98.72%) and swallowing in one (1.28%). Complex partial seizure was found in 76 cases (97.44%). Electroencephalogram was abnormal in 25 cases (32%).

Adolescent↗

Cerebrovascular disease in rural Kashmir, India.

We studied the Kuthar Valley in the Anantnag District of south Kashmir (northwestern India) during the year 1986 to ascertain the prevalence and pattern of completed stroke. We detected 91 cases, giving a crude prevalence rate of 143/100,000. However, age-specific prevalence was 41/100,000 in the group aged 15-39 years and 630/100,000 in the group aged greater than or equal to 40 years; 69.23% of the cases were in men. Hypertension was present in 58.24% of the cases, while strokes due to valvular heart disease and puerperium-related strokes were most common in the young. Our study is first of its kind in this part of India.

Adolescent↗