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Biomedical subjects

P Hartley

Publications and source records attributed to P Hartley.

At least 19 recordsLinked to original sources

Hepatoblastoma--evolution of management and outcome and significance of histology of the resected tumor. A 31-year experience with 40 cases.

BACKGROUND/PURPOSE: The aim of this study was to retrospectively evaluate and compare the clinical features, treatment strategy, pathology, and outcome of all patients with hepatoblastoma treated at an African hospital over a 31-year period (1970 to 2001). METHODS: Forty patients with hepatoblastoma were divided into 3 groups according to the treatment given. Group I (1970 to 1983, 14 patients) had no protocol therapy; group II (1984 to 1988, 6 patients) received protocol treatment according to Children's Study Group (CCSG) guidelines; group III (1989 to 2001, 20 patients) received SIOPEL protocol therapy. All available clinical, surgical, radiologic, and pathologic data were reviewed and analyzed. RESULTS: Overall patient survival was as follows: group I, 14%; group II, 50%, and group III, 80%. Deaths in group II were caused by chemotherapy-induced immunosuppression only. Prognostic data for group III showed that all tumor-related deaths could be predicted by identifying multifocal disseminated growth patterns (P =.001) or vascular invasion (P =.001) in resected tumors. Of the 40 diagnostic tumor biopsies performed, 2 significant complications (1 death, 1 intraperitoneal tumor seeding) occurred. Histologic criteria evaluating these biopsies were not predictive of overall survival. CONCLUSIONS: The introduction of protocol therapy has resulted in a marked improvement in survival. Immunosuppression-related sepsis in our setting resulted in unacceptable mortality in patients treated according to CCSG guidelines. A diagnostic biopsy in hepatoblastoma is of value but not without complications. Preoperative chemotherapy followed by complete surgical excision according to International Society of Paediatric Oncology guidelines yields excellent results with a current survival rate of 80%.

Antineoplastic Combined Chemotherapy Protocols↗

Incidence of acute lymphoblastic leukaemia in white and coloured children in the Western Cape.

UNLABELLED: Objectives. To record the age-specific incidence rate (ASIR) for diagnosed acute lymphoblastic leukaemia (ALL) in coloured and white children aged 0 - 12 years in the Western Cape (WC). DESIGN: A retrospective population-based study using the 1991 population census to calculate the mean annual childhood population and the ASIR for ALL in the 0 - 4, 5 - 9 and 10 - 12-year age groups in rural and Cape Town metropolitan areas for the period 1983 - 1999. Odds ratios were calculated using EpiInfo 2000. SETTING: Registry records of the paediatric cancer units at Tygerberg and Red Cross War Memorial Children's hospitals where all children with ALL in the WC were initially treated. SUBJECTS: All white and coloured children aged 0 - 12 years diagnosed as having ALL from 1983 - 1999. OUTCOME MEASURES: The ASIR by age and ethnic group in rural and metropolitan patients in the WC. RESULTS: The estimated annual childhood population in 1991 was 709 151 with 80.4% coloured and 19.6% white children, of whom 60% were resident in the Cape Town metropolitan area and 40% in the rural area of the WC. Of 246 children with ALL diagnosed in the period 1983 - 1999, 144 were male and 102 female. The ASIR in coloured children aged 0 - 4 years was 17.1/10(6) in the rural and 30.5/10(6) in the metropolitan area, compared with 55.7/10(6) and 56.2/10(6) respectively in white children. In the 5 - 9-year age group the ASIR in coloured children was 10.0/10(6) in the rural and 16.6/10(6) in the metropolitan area compared with 27.6/10(6) and 26.7/10(6) respectively in white children. The 10 - 12-year age group had comparable incidence rates in both populations and geographical areas. Only one case occurred within a 20 km radius of the Koeberg nuclear reactor. CONCLUSIONS: White children have an ASIR for ALL comparable to rates of diagnosis in the USA, while only half as many coloured children aged 0 - 9 years were diagnosed in both the rural and metropolitan areas. This contrast may indicate significant underdiagnosis of ALL in coloured children over the period in question. The change in health policy since 1994, which has improved access to primary health care, may improve the rate of diagnosis among coloured and black children.

Adolescent↗

Haemophilia patients aged 0-18 years in the Western Cape.

OBJECTIVES: To record the number of haemophilicas aged 0-18 years in the Western Cape (WC), what event led to the diagnosis, the level of clotting factor, treatment, functional status of their joints and impact of the disease on the family. DESIGN: A prospective study of patients registered with the South African National Haemophilia Registry and new patients, utilising the patients' paediatricians, hospital records, patient and guardian interviews, physical examination and provincial nurse haemophilia co-ordinators. SETTING: Haemophilia care centres at the three WC academic hospitals, regional hospitals and homes of patients. Two elective medical students, MHH and JJH, collected the information. SUBJECTS: All boys with confirmed haemophilia A or B in the WC. OUTCOME MEASURES: Events that led to diagnosis, degree of haemophilia, use of clotting factor, functional status, and effect on family. RESULTS: Of 78 patients (59 haemophilia A, 19 haemophilia B) identified, 49 could be studied. Forty-three per cent had severe, 29% moderate and 22% mild disease (6% unknown). Family history was present in 49%, but led to diagnosis in only 12%. The most common first symptoms were subcutaneous and mucosal bleeding. Delay in diagnosis varied from 0 to 9 months. Twenty-nine per cent of guardians were suspected of child abuse. RSA produced clotting factor was used 'on demand' in 73% of patients, for periodic prophylaxis in 20% and as continuous prophylaxis in 7%. Joints were functionally restricted in 43% of patients. The majority of guardians (59%) said the disease had a major impact on the family. CONCLUSIONS: The diagnosis of haemophilia in children with a positive family history was often delayed. Haemophilia causes significant morbidity in our patients and their families.

Adolescent↗

Extended hepatic resection with transplantation back-up for an "unresectable" tumour.

Liver transplantation (LT) for malignancy has had disappointing long-term results due to tumour recurrence. Ex-vivo dissection and auto-transplantation have had poor results when the tumor was obstructing bile ducts. Advances in liver surgery have made extensive liver resection safer, but cases of "unresectable" tumours due to site and size still present. A 10-year-old boy was referred with jaundice due to a 6 x 8-cm central (segment 4) tumour shown on biopsy to be a fibrolamellar hepatocellular carcinoma. Ultrasound (US) and Computed Tomography also showed dilatation of intrahepatic bile ducts in both lobes. Angiography showed a large tumour mass supplied by the left branch of the hepatic artery, a low take-off of a right branch of the hepatic artery, and a very displaced but patent portal vein. The initial surgical consensus was that the tumour was unresectable. The patient was listed for LT with the plan of first attempting resection with a liver graft-in-waiting. An extended left hepatectomy was performed under total vascular exclusion with resection of the tumour, which had extended from segment 4 into surrounding segments 1, 3, 5, and 8. Intraoperative US assisted in planning the resection. The right hepatic vein, artery, and the right branch of the portal vein could be preserved and a Roux loop was anastomosed to a markedly dilated segment 6 and 7 intrahepatic duct for bile drainage. Vascular exclusion time was 30 min. The patient made a good recovery without major complications. Jaundice and bile-duct dilatation resolved. On follow up at 5 years there was no recurrence. The liver graft-in-waiting gave the surgical team confidence to proceed with an extensive resection beyond a "point of no return" and allowed good clearance of the disease and avoidance of LT with all the long-term consequences of immunosuppression. This mandates that extensive hepatic surgery in children should be carried out in centres that have a facility for LT should the need arise.

Carcinoma, Hepatocellular↗

The National Liver Transplantation audit: an overview of patients presenting for liver transplantation from 1994 to 1998. On behalf of the Steering Group of the UK Liver Transplantation Audit.

BACKGROUND: The aim of this study was to describe current clinical practice in liver transplantation in the UK and Ireland, to provide overall 1-year graft and patient survival rates, and to study some preoperative risk factors. METHODS: All patients receiving a liver transplant in the UK or Ireland between 1 March 1994 and 30 September 1998 were included. Data were collected on patients at the time of transplantation, 3 months after grafting and annually thereafter until the patient's death. The main outcome measures were graft and patient survival at 1 year. RESULTS: A total of 3102 liver transplants were carried out, of which 87 per cent were first transplants. The mean age at first transplantation was 42 (range 0-76) years. The most common indications for transplantation were primary biliary cirrhosis, alcoholic cirrhosis and posthepatitis C cirrhosis, but variations existed between sexes and centres. Risk factors associated with lower graft and patient survival were the presence of acute disease, being transplanted from hospital, and the need for renal and/or ventilatory support before operation. CONCLUSION: Donor and recipient demographics are consistent with data held by the European Liver Transplant Registry, as are 1-year graft and patient survival rates. Variation across centres in factors such as the primary indication for liver transplantation, population demographics, the clinical status of each patient, incidence of retransplantation and other risk factors contributes to the problem of adjusting for case mix.

Adolescent↗

Coherent probability from incoherent judgment.

People often have knowledge about the chances of events but are unable to express their knowledge in the form of coherent probabilities. This study proposed to correct incoherent judgment via an optimization procedure that seeks the (coherent) probability distribution nearest to a judge's estimates of chance. This method was applied to the chances of simple and complex meteorological events, as estimated by college undergraduates. No judge responded coherently, but the optimization method found close (coherent) approximations to their estimates. Moreover, the approximations were reliably more accurate than the original estimates, as measured by the quadratic scoring rule. Methods for correcting incoherence facilitate the analysis of expected utility and allow human judgment to be more easily exploited in the construction of expert systems.

Adult↗

Improved prognosis of Epstein-Barr virus associated childhood Hodgkin's lymphoma: study of 47 South African cases.

AIM: To study the distribution of Hodgkin's lymphoma in South African children and report the incidence of Epstein-Barr virus (EBV) as regards age, race, sex, and histological subtype; to investigate whether EBV is relevant to survival. METHODS: Immunohistochemistry (IHC) and in situ hybridisation (ISH) to detect EBV were performed on 47 South African children with classical Hodgkin's lymphoma, ranging in age from 3 to 14 years and coming from different ethnic backgrounds. The correlation between the presence of the virus and clinical outcome was assessed. RESULTS: The nodular sclerosing subtype predominated, comprising 89% of cases; the remaining 11% were of the mixed cellularity subtype. EBV was present in 68%. Full clinical data were available for 36 cases; EBV positive patients presented with less aggressive symptoms at diagnosis and had a significantly longer median survival than EBV negative patients. CONCLUSIONS: The distribution of EBV in South African childhood Hodgkin's lymphoma follows a pattern intermediate to that of industrialised and non-industrialized countries. Furthermore, our data suggest that there is an association between poor prognosis and the non-detection of EBV products in South African childhood Hodgkin's lymphoma.

Adolescent↗

Bulimia: implications for the practising dentist.

The incidence of eating disorders appears to be increasing, with the dental practitioner potentially being the first healthcare worker to make a diagnosis, due to the characteristic dental signs of tooth substance loss. It is therefore important that members of the dental team are aware of the dental sequelae of anorexia and bulimia and are able to offer advice and treatment to sufferers.

Anorexia↗

Invasive pulmonary aspergillosis associated with low dose methotrexate therapy for rheumatoid arthritis: a case report of treatment with itraconazole.

Opportunistic infections complicating low dose methotrexate therapy are rare, consisting predominantly of Pneumocystis carinii pneumonia. We report a case of invasive pulmonary aspergillosis which developed in a patient being treated for seropositive rheumatoid arthritis with low dose methotrexate (5-7.5 mg weekly) for 8 years. The patient was successfully treated with itraconazole 200 mg daily for 6 months. This case adds to the growing evidence implicating low dose methotrexate with opportunistic infections and should heighten clinical awareness in patients on this treatment.

Aged↗

Organizing for communication and integration.

Using the departmental philosophy as its foundation, an integrated organizational model was designed for a nursing department. This patient-centered model was implemented to streamline operations, focus resources, and provide a professional practice environment for nursing staff. The "molecule" is used as a teaching tool and organizational framework, with applications ranging from orientation of new staff to management of daily operations.

Communication↗

Fine-needle cytology of solid tumors: method, diagnostic accuracy, and role in management.

Fine-needle cytology was obtained from 14 solid tumors in 12 children. Both aspiration and nonaspiration techniques were used and several staining methods were applied. May Grünwald Giemsa and Papanicolaou stains were preferred. The nonaspiration method yielded a superior quality cytology smear with less blood contamination. There were no complications recorded. Confirmation of the diagnosis with cytology allowed for planned management with preoperative cytotoxic chemotherapy and/or radiotherapy in 10 children, immediate surgery in one, and radiotherapy to a vertebral recurrence in one. Fine-needle cytology is considered a useful technique in the management of a selected group of children with solid tumors.

Biopsy, Needle↗

Decreased intracranial self-stimulation in a new animal model of endogenous depression.

Neonatal treatment of rats with clomipramine may produce adult animals which model endogenous depression. We report here that a major factor of depression in humans, the diminished capacity for pleasure, appears present in these rats. At age 7 months, bar-press responding for rewarding hypothalamic stimulation is reduced across a range of intensities. At age 4 or 5 months this effect is not seen, although other behavioral abnormalities are present at the younger age. The delayed onset of diminished intracranial self-stimulation may relate to the gradual insidious onset of endogenous depression in humans.

Animals↗

Procedure- and age-dependent hyperactivity in a new animal model of endogenous depression.

We have replicated the findings of Mirmiran and colleagues that neonatal administration of the antidepressant clomipramine (CLI) to male rats results in hyperactivity in open-field tests in adulthood. We report that this effect does not reliably occur in a "Digiscan" activity device. The difference in effect between the two activity measuring devices may be due to more stress being present in the open-field test, and we propose that the CLI-treated rats may be more reactive to stress. This hypothesized enhanced reactivity to stress may be similar to the proposed vulnerability of depressed humans to stress. In addition, we have found that the open-field effect does not occur until the rats are at least 4 months old; this delayed effect may be analogous to the progressive onset of endogenous depression in humans.

Aging↗

Mucin-secreting Wilms' tumor. Report of two cases.

Two children, ages 7 years and 14 months, with mucin-secreting Wilms' tumor were treated at the Red Cross War Memorial Children's Hospital in 1987. In both cases, examination of peripheral blood films disclosed the characteristic appearance of circulating mucin. In each case the blood film appearances returned to normal following nephrectomy. Both patients are in good health and apparently tumor free at 16 and 18 months follow-up.

Antineoplastic Combined Chemotherapy Protocols↗