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Biomedical subjects

S Venugopal

Publications and source records attributed to S Venugopal.

At least 19 recordsLinked to original sources

Postsplenectomy course in homozygous sickle cell disease.

OBJECTIVE: To determine whether children with homozygous sickle cell (SS) disease and splenectomy are at greater risk of death, overwhelming septicemia, or other complications. METHODS: A total of 130 patients with SS treated by splenectomy (46 recurrent acute splenic sequestration, 84 chronic hypersplenism) over a 22.5-year period at the Sickle Cell Clinic of the University Hospital of the West Indies, Kingston, Jamaica, were compared with a control group matched for sex, age, and duration of follow-up in a retrospective review. Deaths and bacteremias were examined over the whole study period. Painful crises, acute chest syndromes, and febrile episodes were compared in the 90 patients completing 5 years of postsplenectomy follow-up. FINDINGS: Mortality and bacteremic episodes did not differ between the splenectomy and control groups. Painful crises were more common in the splenectomy group than in the control group (P =.01) but did not differ between splenectomy indications. Acute chest syndrome was more common in the splenectomy group than in the control group (P <.01) and was more common in the acute splenic sequestration group than in the hypersplenism group (P =.01). Febrile events did not differ between the groups or between the indications for splenectomy. CONCLUSION: Splenectomy does not increase the risk of death or bacteremic illness in patients with SS disease and, if otherwise indicated, should not be deferred for these reasons.

Adolescent

Dumping syndrome in a young Jamaican child.

The dumping syndrome in childhood is an uncommon complication of gastro-oesophageal surgery, principally Nissen fundoplication. A Jamaican child developed the syndrome after fundoplication and pyloroplasty to relieve gastro-oesophageal reflux complicating the repair of a congenital tracheo-oesophageal fistula. He developed marasmus and failed to gain weight on the standard remedial milk-based high-energy diet. An oral glucose tolerance test confirmed the diagnosis of dumping syndrome. A low sugar low milk diet based on adult type meals with continuous nibbling of fried dumplings relieved his diarrhoea and hypoglycaemia and he gained weight. This is a cheaper and more practical dietary therapy than the regimens described previously.

Dumping Syndrome

Hydrostatic reduction of acute intussusception. A prospective study.

Data were collected prospectively on 57 Jamaican children presenting with 62 episodes of acute intussusception over a two year period, for whom operative and hydrostatic methods of reduction were employed. 31 (54%) of 57 episodes were reduced successfully using barium (42), saline (11) and air (4) hydrostatically. Among the 31 other episodes, 15 had ileo-colic intussusception, seven caeco-colic, six ileo-ileo-colic and one ileo-ileal. Two patients had spontaneous reduction discovered at surgery. There were two episodes of barium hydrostatic perforation of the colon leading to death in one patient. Hydrostatic reduction is recommended as the first therapeutic option for acute intussusception because it spares the patient a major operative procedure when successful.

Acute Disease

Identification and characterization of excreted/secreted products of Trichuris trichiura.

This study provides the first description of the range and immunogenicity of proteins excreted and/or secreted by living T. trichiura adult worms following their recovery from the human large intestine. Metabolic labelling of T. trichiura excretory/secretory (ES) products with [35S]-methionine revealed a range of proteins with prominent components at 52-54 kDa, 35-45 kDa & 17 kDa. In contrast, the major component of unlabelled T. trichiura ES, somatic whole worm and isolated stichosome extracts, and of [35S]-methionine labelled somatic extracts, was present at approximately 47 kDa. Similarly, the major 43 kDa protein present in unlabelled T. muris ES, somatic worm extract and [35S]-methionine labelled somatic worm extract, was only weakly detected in labelled T. muris ES. Pulse chase experiments demonstrated that after 20 h, the 43 kDa was a prominent component of T. muris ES. These data suggest that the 43/47 kDa protein of Trichuris adult worms is not a major constituent of newly synthesized ES but is either synthesized at a slower rate than other proteins, or sequestered or stored, most likely in the stichocytes, before release. Immunoprecipitations using a range of sera from T. trichiura-infected individuals demonstrated that many of the ES components are immunogenic. Antibody responses were vigorous in children with intense infections and negligible in parasitologically negative children. There was marked heterogeneity in responses to a 17 kDa antigen, with the age profile of anti-17 kDa antibody levels reflecting age-dependent infection intensities at the population level.

Adolescent

Acceleration in linear growth after splenectomy for hypersplenism in homozygous sickle cell disease.

Chronic hypersplenism in homozygous sickle cell (SS) disease markedly increases haemolysis and the resulting erythropoietic expansion is likely to have a high metabolic cost. Splenectomy for hypersplenism in SS disease is followed by highly significant changes in haematological indices and an increase in height, but not weight, velocity. This pattern is similar to that observed in the trichuris dysentery syndrome after treatment, and differs from the increases in both height and weight velocity that follow nutritional supplementation of severely malnourished children. It is postulated that accelerated linear growth after the reduction in erythropoietic stress may implicate a specific nutrient deficiency in hypersplenic children with SS.

Body Height

Childhood portal hypertension in Jamaica.

Portal hypertension and bleeding from oesophageal varices in children remain a difficult medical problem. The clinical course and management of children with portal hypertension seen over a 14-year period was reviewed. There were 5 females and 2 males with a mean age of 3.6 years at presentation. Five patients presented with severe upper gastrointestinal bleeding and two with severe hypersplenism. All patients had extra-hepatic portal hypertension. Five patients were treated with endoscopic sclerotherapy, including one who had bleeding five years post-splenectomy. A mean of 9 sclerotherapy sessions was performed in each patient. Complete obliteration of varices was not achieved in any patient and a single rebleeding episode occurred in four. Three children underwent operative management consisting of splenectomy in two and splenectomy and central spleno-renal shunt in one. There was no mortality in either group after a mean follow-up of 4.3 years. Sclerotherapy may not be totally successful in long-term management of childhood portal hypertension. Surgical therapy or a combination of sclerotherapy and surgery may be the best approach.

Child

Prognosis of neuroblastic tumours in children.

A retrospective analysis of 26 children with neuroblastic tumours treated at the University Hospital of the West Indies (UHWI) between 1970 and 1991 was undertaken to evaluate factors affecting prognosis. The peak incidence was between 48 months and 60 months of age, and 75% of the deaths occurred in children older than 3 years. The abdomen was the most common site of the tumours 79% of which were from the adrenal gland and carried a dismal outcome. Other sites were thoracic, pelvic and cervical. Extra-adrenal tumours have a good outcome even when the histology is unfavourable; 92% of the tumours were in Stages III or IV at the time of initial presentation. Stage IV disease accounted for all but one of the deaths. Of the 8 children with favourable histology (ganglioneuroma or well-differentiated ganglioneuroblastoma), only one (12.5%) died, wheras of the remaining 18 cases with unfavourable histology (neuroblastoma and undifferentiated ganglioneuroblastoma), eleven (61.1%) died. When managed by surgery alone or with adjuvant chemo- and/or radio-therapy, only 36.4% succumbed while all 4 children with chemotherapy only died. Ten children are alive without disease for more than three years post-therapy. A scoring system was designed which takes into account the factors influencing the outcome in neuroblastic tumours, namely, age, location, stage and histological types of the tumours and therapy. All the children with a score of 21 or less survived, whereas all those with scores of 22 or above succumbed. This underlines the multifactorial influences on the final outcome of neuroblastic tumours.

Abdominal Neoplasms

Outcome of solid renal tumours in children at the University Hospital of the West Indies.

Of the twenty-nine children with solid renal tumours treated at the University Hospital of the West Indies (UHWI) between January, 1972 and December, 1991, there were twenty-eight cases of nephroblastoma and one of mesoblastic nephroma. Peak incidence was between the ages of two and four years. Twenty-five children had radical nephrectomy while one had bilateral partial nephrectomy. In thirteen cases, pre-operative chemotherapy +/- radiotherapy was used. Post-operative chemotherapy and radiotherapy were used in 24 and 13 cases, respectively. Stage of the tumour was the most decisive factor influencing the outcome. Whereas there was a 100% cure rate in Stages I and II, Stage III had only a 55.5% survival rate and none of the Stage IV survived. Bilateral (Stage V) tumours are curable if individual tumours are localised, as in one of the two cases. The benign mesoblastic nephroma, in a one-month-old infant, was cured by nephrectomy alone. While the present therapy of radical nephrectomy along with combination chemotherapy is satisfactory for early stages, more aggressive adjuvant therapy is needed for improving the results in Stages III and IV.

Child

The Rapunzel syndrome. Report of a case and review of the literature.

The Rapunzel Syndrome, a rare manifestation of trichobezoar, occurs when bolus gastrointestinal obstruction is produced by an unusual trichobezoar with a long tail that extends to or beyond the ileocaecal valve. A five-year-old Jamaican girl presented with this abnormality and was found at laparotomy also to have an ileo-ileal intussusception. For the Rapunzel Syndrome, we recommend bezoar extraction at laparotomy via multiple enterotomies. In addition, psychiatric evaluation and therapy is essential due to the commonly associated finding of underlying emotional stress.

Bezoars

The low frequency of reflux in Jamaican children.

Fifty consecutive micturating cystourethrograms (MCUG) performed at the UHWI between June 1989 and December 1991 were reviewed. There were forty boys and ten girls. Infants under the age of one year accounted for 58%. Urinary tract infection was the commonest presenting feature (72%), voiding difficulties (10%) and associated malformations (14%) were the other reasons for doing the MCUG. Only 10% of the children revealed vesico-ureteral reflux (VUR) which supports the view that reflux is less common in the noncaucasian population. In our population, the yield from MCUG is low. However, when detected, the reflux was of significant degree as to warrant active therapy. All but one of the VU refluxes diagnosed by MCUG had ultrasonographic abnormalities. Black children with single urinary infections have a low likelihood of VUR. A clear history of pyelonephritis and abnormalities on ultrasound or radionuclide cystogram may be used as criteria to select those in whom MCUG will be useful.

Child

Inguinal hernia in children.

Two hundred and seventy-one cases of inguinal hernias and hydrocoeles in children treated at the University Hospital of the West Indies over a five-year period have been reviewed. The dominance of the boys and the right side is borne out. The younger infant has the highest risk of incarceration which is more common in boys and in right-sided hernias. Females with inguinal hernia were screened for testicular feminization and none was found. There were three girls with hydrocoeles of the Canal of Nück. The incidence of metachronous presentation of contralateral hernia occurred only in 6.6% of cases. When routine exploration of the contralateral side was undertaken in 5.2% of cases, a hernial sac was found only in less than half of them. These findings support the present policy of not routinely exploring the opposite side in unilateral inguinal hernias. Herniotomy was adequate for 92.6% of cases. Herniorrhaphy was reserved for large hernias and sliding hernias. Complications were minor and infrequent. Recurrence was seen in only one case. One testicular atrophy occurred following incarceration. Eight-six per cent of these children were managed as day cases. In-patient management was required when the hernia was complicated or when there were associated other medical conditions.

Child, Preschool

Colo-mucosal proctectomy with ileo-anal anastomosis. The Jamaican experience.

A total colectomy and a mucosal proctectomy with ileo-anal anastomosis (c-mp-iaa) effectively removes all debilitating, potentially malignant colonic mucosa. Preservation of the anorectal sphincter predictably results in good to excellent anal continence with a low mortality and acceptable morbidity. Since 1983 at the University Hospital of the West Indies (U.H.W.I.), Jamaica, five (5) patients have undergone c-mp-iaa for colonic mucosal disease. All have good to excellent anal continence with an average of 2-6 continent, formed stools per day. There were no operative deaths. The five (5) cases are described and a plea is made for the earlier use of this definitive surgical procedure where indicated.

Adolescent

Immediate hypersensitivity in colon of children with chronic Trichuris trichiura dysentery.

There are few data on mucosal immune responses to intestinal helminths in human beings, especially those involving the IgE system, which is thought to be important in parasite expulsion. We sought evidence of an immediate hypersensitivity reaction in the colon of children with chronic dysentery due to Trichuris trichiura. 28 children with Trichuris dysentery syndrome (TDS) were compared with 16 control children (with no TDS or worms visible on colonoscopy). All children were aged 1-11 years. Rectal biopsy samples were taken before and after expulsion of the worms by means of mebendazole treatment. Children with TDS had significantly greater numbers than controls of mast cells (mean [SD] 10.9 [1.3] vs 3.9 [0.6]% of all cells; p less than 0.0003) and of cells with surface IgE (median [range] 11.1 [7.5-11.6] vs 1.0 [0-1.5]%; p less than 0.001) in the subepithelial region of the mucosa. On electronmicroscopy, degranulating mast cells were prominent in parasitised children. In culture, rectal biopsy samples from parasitised children showed high rates of spontaneous histamine release, but only low rates of antigen-specific release. After treatment, spontaneous histamine release was significantly reduced and antigen-specific histamine release could be provoked. Thus, an IgE-mediated immune mucosal response to a helminth infection does occur in human beings but is not sufficient to cause appreciable parasite expulsion.

Anaphylaxis

Histopathology and immunohistochemistry of the caecum in children with the Trichuris dysentery syndrome.

Caecal biopsy specimens from Jamaican children with the Trichuris dysentery syndrome (TDS) and age matched Jamaican controls were investigated by immunohistochemistry and by light microscopy. Biopsy specimens from all children (with TDS and controls) showed a mild to moderate increase in inflammatory cells. Except in the vicinity of the worm, where the epithelium was flattened, there was no other epithelial abnormality. Compared with controls, children with TDS had increased IgM lamina propria plasma cells and decreased intraepithelial T cells. There was also an increase in crypt epithelial cell proliferation. Lamina propria T cells (both activated and non-activated) were no more common in children with the Trichuris syndrome than controls. Epithelial cell HLA-DR and VLA-1 expression (which are increased in other colitides) were the same in both groups. Despite the presence of large worm burdens and chronic dysentery, therefore, only minor changes were seen in the caecal mucosa of children with TDS.

Antigens, CD

Apurinic endonuclease activity from wild-type and repair-deficient mei-9 Drosophila ovaries.

An endonuclease which acts on apurinic (AP) sites in DNA was partially purified from Drosophila ovaries. The enzyme present in the female germ line has a molecular weight of 63,000 daltons, is Mg++ dependent, and produces a site upon cleaving depurinated DNA that supports DNA repair synthesis. Although the same characteristics are shared by the enzyme present in the excision-deficient mutant mei-9, specific activity for the AP endonuclease is reduced 98% when compared with that found for its wild-type counterpart. Moreover, cross-reactivity toward an antibody that recognizes the wild-type AP endonuclease protein is reduced roughly 90% for partially purified preparations from mei-9. Mixing experiments between extracts of mei-9 and wild type suggest that the mei-9 structural gene somehow alters or influences the levels of the AP endonuclease protein, but in view of the complex phenotype of this mutant the endonuclease is probably not the product of the gene itself.

Animals

Caustic strictures of the oesophagus.

Ingestion of caustic material is a major management problem which occurs most commonly in children. Twenty-five patients with caustic ingestion seen over a ten-year period are reported. Twelve patients had severe burns, resulting in oesophageal strictures. Of these, ten were children below six years of age (median age, 3 years). Dysphagia and vomiting were the main presenting features. Oropharyngeal burn was present in 83% of patients with oesophageal burns. The strictures involved the entire oesophagus in two patients, the upper third in four, the mid oesophagus in two and the lower third in four. Periodic dilatations were successful in restoring an adequate lumen in 7 patients with short strictures. Five patients required surgery. There was one death. Caustic damage to the oesophagus is preventable. Education of the public and simple measures by manufacturers of caustics are urgently needed.

Accidents, Home

Antibody to a human DNA repair protein allows for cloning of a Drosophila cDNA that encodes an apurinic endonuclease.

The cDNA of a Drosophila DNA repair gene, AP3, was cloned by screening an embryonic lambda gt11 expression library with an antibody that was originally prepared against a purified human apurinic-apyrimidinic (AP) endonuclease. The 1.2-kilobase (kb) AP3 cDNA mapped to a region on the third chromosome where a number of mutagen-sensitive alleles were located. The cDNA clone yielded an in vitro translation product of 35,000 daltons, in agreement with the predicted size of the translation product of the only open reading frame of AP3, and identical to the molecular size of an AP endonuclease activity recovered following sodium dodecyl sulfate-polyacrylamide gel electrophoresis of Drosophila extracts. The C-terminal portion of the predicted protein contained regions of presumptive DNA-binding domains, while the DNA sequence at the amino end of AP3 showed similarity to the Escherichia coli recA gene. AP3 is expressed as an abundant 1.3-kb mRNA that is detected throughout the life cycle of Drosophila melanogaster. Another 3.5-kb mRNA also hybridized to the AP3 cDNA, but this species was restricted to the early stages of development.

Amino Acid Sequence