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Biomedical subjects

A Kelsey

Publications and source records attributed to A Kelsey.

At least 19 recordsLinked to original sources
PubMed2007

Incidence and survival of childhood Langerhans cell histiocytosis in Northwest England from 1954 to 1998.

BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of pathological Langerhans cells, for which the aetiology and pathogenesis remain largely unknown. PROCEDURE: Information on the 101 children with LCH registered with the population-based Manchester Children's Tumour Registry (MCTR) between 1954 and 1998 was extracted from the records of the MCTR. This included age, sex, date of diagnosis, systems affected at diagnosis and follow-up. RESULTS: The overall incidence rate for LCH was 2.6 cases per million child years. In those under 1 year of age the incidence rate was 9.0 cases per million child years, compared to 0.7 cases per million in those aged 10-14 years (P < 0.0001 for age trend). There was no evidence of seasonal variation in presentation by month of birth or first symptom. Bone was the most common site of disease involvement (67% of cases), followed by skin (37%) and soft tissue (22%). The overall survival rate has improved over time, from 57% in 1954-1968 to 74% in 1985-1998. Ninety percent of deaths were due to disease progression, the remainder were due to complications of intensive therapy. The site of LCH lesions and extent of disease present at diagnosis strongly predicted survival outcome. Patients with initial liver involvement had a 5-year survival rate of 25% compared with 93% for those with bone lesions alone at diagnosis. CONCLUSIONS: Incidence rates varied significantly by age at diagnosis, and have been stable over time. Survival has improved considerably over time, but varies strongly by age and systems affected at diagnosis.

Adolescent↗
PubMed2004

Outcome of patients with stage III or inoperable WT treated on the second United Kingdom WT protocol (UKWT2); a United Kingdom Children's Cancer Study Group (UKCCSG) study.

BACKGROUND: The aims of UKWT2 included consolidating the results for stage III patients obtained in UKWT1 and improving the outcome for patients with inoperable tumours by giving vincristine, actinomycin-D and doxorubicin in an intensive schedule (Intensive AVA). PROCEDURE: The second UK WT trial (UKWT2) ran between July 1986 and September 1991 accruing 448 patients. One hundred and six patients were diagnosed and treated for stage III disease. Six had clear cell sarcoma of the kidney (CCSK) and seven had rhabdoid tumours of the kidney (RTK) and are analysed separately. One other patient was excluded from overall analysis. Ninety-two patients were followed for a median of 115 months. Seventy-five received standard chemotherapy and abdominal radiotherapy according to protocol. Seventeen had stage III disease at immediate nephrectomy, but radiotherapy was omitted by physician choice. Thirty-three patients had inoperable disease at diagnosis and received pre-nephrectomy chemotherapy. RESULTS: Overall survival (OS) at 4 years for stage III favourable histology (FH) patients receiving abdominal RT was 83% (CI: 73-89). For children with stage III disease in whom RT was omitted the OS was 82% (CI: 59-97) and for inoperable disease 94% (CI: 78-98). The overall and event-free survival (EFS) of children with stage III CCSK was 100% and was achieved with the majority of patients not receiving radiotherapy (CI: 48-100). Three of seven children with RTK are alive EFS and OS 43% (CI: 10-73). For patients treated by abdominal radiotherapy the overall local control rate was 94.4% (CI: 86.4-98.5*%), 96.7% (CI: 88.5-99.6%) for flank RT and 83.3% (51.6-98.0%) for whole abdominal radiotherapy (WRT). CONCLUSIONS: The outcome for stage III FH disease was similar to that reported for UKWT1 and NWTS-3. The combination of abdominal RT together with 3-drug chemotherapy achieves a high abdominal tumour control rate. Flank RT is probably sufficient for localised tumour rupture. The high cure rates for children in this trial with 'inoperable disease' suggests that treatment should be modified according to their post-chemotherapy stage in order to avoid over-treatment. The high OS for stage III CCSK on this protocol suggests that treatment duration could be curtailed and the role of RT reviewed, though the numbers are small. The prognosis for older children with RTK seems to be better than for younger children although larger studies are required to confirm this.

Adolescent↗
PubMed2004

Isolated intracranial Rosai-Dorfman disease mimicking meningioma in a child.

A 9-year-old boy presented with a 12-month history of headaches and recent grand mal seizures. Computed tomography and magnetic resonance imaging of the brain revealed an enhancing right frontal convexity lesion. The preoperative diagnosis was meningioma. However, histological examination was diagnostic of Rosai-Dorfman disease. Rosai-Dorfman disease confined to the intracranial compartment is very rare, of 34 reported cases only one presented in the first decade. Optimal treatment has not been established, but complete surgical resection alone seems effective and allows histological distinction from meningioma.

Brain↗
PubMed2003

Older age is an adverse prognostic factor in stage I, favorable histology Wilms' tumor treated with vincristine monochemotherapy: a study by the United Kingdom Children's Cancer Study Group, Wilm's Tumor Working Group.

PURPOSE: To identify clinical prognostic factors in children with stage I, favorable histology (FH) Wilms' tumor treated with vincristine monochemotherapy after immediate nephrectomy to define subgroups for consideration of further reduction in treatment intensity. PATIENTS AND METHODS: During two consecutive trials of the United Kingdom Children's Cancer Study Group (UKW2 and UKW3, 1986 to 2001), 242 children with stage I FH Wilms' tumor were treated with immediate nephrectomy followed by 10 weekly injections of vincristine 1.5 mg/m2. Event-free survival (EFS) and overall survival (OS) were compared by age group. RESULTS: The 4-year EFS rate was 93.2%, 87.2%, and 71.3% for children less than 2 years old, 2 to 4 years old, and 4 years old or older at diagnosis, respectively (log-rank, P =.001); the corresponding 4-year OS rate was 98.1%, 95.0%, and 87.2% (log-rank, P =.01). There were no toxicity- or procedure-related deaths. In multivariate analysis, specimen weight was not of independent prognostic value (P =.66). Among the 186 children younger than 4 years at diagnosis, there were 17 relapses and five deaths, compared with 16 relapses and eight deaths among the 56 children at least 4 years old at diagnosis. OS after relapse was surprisingly poor (61.6% at 4 years). CONCLUSION: Treatment for stage I FH Wilms' tumor is generally successful using vincristine monotherapy after immediate nephrectomy, and therefore, the risks of dactinomycin hepatopathy can be avoided. However, age at least 4 years is a significant adverse prognostic factor. This treatment schedule should be considered in any trial of treatment reduction in very young children with stage I FH Wilms' tumor, regardless of tumor size, and we suggest that the upper age limit for the reduced therapy be set at 4 years.

Adolescent↗
PubMed2003

Early and severe presentation of X-linked myotubular myopathy in a girl with skewed X-inactivation.

X-linked myotubular myopathy is a severe congenital myopathy in males, caused by mutations in the myotubularin (MTM1) gene on chromosome Xq28. In heterozygous carriers of MTM1 mutations, clinical symptoms are usually absent or only mild. We report a 6-year-old girl presenting at birth with marked hypotonia and associated feeding and respiratory difficulties. A muscle biopsy performed at 5 months suggested a diagnosis of myotubular myopathy. On examination at 6 years she had marked facial weakness with bilateral ptosis and external ophthalmoplegia, severe axial and proximal weakness and a mild scoliosis. Muscle magnetic resonance imaging showed a distinctive pattern of muscle involvement. Molecular genetic investigation of the MTM1 gene identified a heterozygous mutation in exon 12. X-inactivation studies in lymphocytes showed an extremely skewed pattern (97:3). This case emphasizes that investigation of the MTM1 gene and X-inactivation studies are indicated in isolated females with histopathological and clinical findings suggestive of myotubular myopathy.

Child↗
PubMed2003

Childhood medulloblastoma in northwest England 1954 to 1997: incidence and survival.

The incidence, survival patterns, and presenting symptoms of children with medulloblastoma were studied. Data were ascertained from the Manchester Tumour Registry which is population-based and has collected data on all childhood malignancies in northwest England since 1954. Incidence rates standardized to the European standard population were calculated and Poisson regression models were used to examine temporal changes in the incidence rates during the period 1954 to 1997. Kaplan-Meier survival curves were derived and used to study changes in survival patterns. World-standardized incidence rates were 5.5 per million child years in males and 3.4 per million child years in females. Incidence rates increased from the 1950s to the 1980s but have declined recently. The 5-year survival rate has improved from 29 to 58% with similar rates for males and females. The 1-year survival rate has also improved, but females had worse survival at this point (58%) than males (75%). The type of symptom or sign at presentation is strongly affected by age, with 10 of the 22 recorded symptoms or signs showing significant age differences. The older the child is, the more likely is the presentation to show pressure features of headache, vomiting, and ophthalmic signs. Younger children present with non-specific features such as lethargy, behavioural disturbance, or increasing head size. Ataxia is seen in about 75% of children across the age range.

Adolescent↗
PubMed2001

Detection of the PAX3-FKHR fusion gene in paediatric rhabdomyosarcoma: a reproducible predictor of outcome?

Rhabdomyosarcoma has 2 major histological subtypes, embryonal and alveolar. Alveolar histology is associated with the fusion genes PAX3-FKHR and PAX7-FKHR. Definition of alveolar has been complicated by changes in terminology and subjectivity. It is currently unclear whether adverse clinical behaviour is better predicted by the presence of these fusion genes or by alveolar histology. We have determined the presence of the PAX3/7-FKHR fusion genes in 91 primary rhabdomyosarcoma tumours using a combination of classical cytogenetics, FISH and RT-PCR, with a view to determining the clinical characteristics of tumours with and without the characteristic translocations. There were 37 patients with t(2;13)/PAX3-FKHR, 8 with t(1;13) PAX7-FKHR and 46 with neither translocation. One or other of the characteristic translocations was found in 31/38 (82%) of alveolar cases. Univariate survival analysis revealed the presence of the translocation t(2;13)/PAX3-FKHR to be an adverse prognostic factor. With the difficulties in morphological diagnosis of alveolar rhabdomyosarcoma on increasingly used small needle biopsy specimens, these data suggest that molecular analysis for PAX3-FKHR will be a clinically useful tool in treatment stratification in the future. This hypothesis requires testing in a prospective study. Variant t(1;13)/PAX7-FKHR appears biologically different, occurring in younger patients with more localised disease.

Adolescent↗
PubMed2001

The making of health visitors. An historical perspective, Part II.

A previous paper (IHNJ Summer 2000) traced the development of the education and training of health visitors from its beginnings to the post second world war period. This second paper begins with the post-war review of health visiting undertaken by the Jameson Working Party and follows developments up to the present day.

Community Health Nursing↗
PubMed2000

Pseudosarcoma of the bladder associated with residual urachus in a 3-year-old girl.

Bladder pseudosarcoma or pseudosarcomatous myofibroblastic proliferation (PMP) is a rare but nevertheless very important differential diagnosis of any bladder mass. In childhood most such masses are usually malignant soft-tissue sarcomas, and distinction from these is therapeutically crucial. To date no aetiological factor for PMP has been identified in children, and treatment is limited to surgical intervention with no role for chemotherapy or radiotherapy. We report clinical, anatomical, and histological findings in a 3-year-old girl with a pseudosarcoma of the urinary bladder associated with a residual urachus, suggesting this as the originating tissue.

Child, Preschool↗
PubMed2000

Minicore myopathy in children: a clinical and histopathological study of 19 cases.

Minicore myopathy is a congenital myopathy characterized by multifocal areas of degeneration in muscle fibres. Genetic heterogeneity expected on the basis of clinical variability awaits further resolution. We reviewed 19 cases in order to further delineate the phenotype. Marked hypotonia was the predominant presenting feature, with evidence of antenatal onset in 30% of cases. Weakness was most pronounced axially and proximally, often more severely affecting the shoulder girdle. Mild facial involvement was frequent. Varying degrees of scoliosis were obvious in all patients older than 10 years. In addition, two patients who were also the most severely affected had complete external ophthalmoplegia. One patient showed marked distal involvement. Respiratory failure developed in half of all patients after 10 years of age and correlated strongly with the degree of scoliosis. Cardiac involvement occurred mainly secondary to respiratory impairment. The course appeared static in most cases. Loss of independent walking was observed only in one case at the age of 10 years. On ultrasound scan, differential involvement within the quadriceps was documented in several patients. Variability in fibre size, type 1 predominance and atrophy with occasional type 2 hypertrophy were prominent but nonspecific histological changes. Apart from typical minicores, a marked increase in internal nuclei was the most prominent histological feature. With the exception of one family in which two generations were affected, inheritance appeared autosomal-recessive or sporadic in all cases.

Adolescent↗
PubMed2000

The treatment of Wilms' tumour: results of the United Kingdom Children's cancer study group (UKCCSG) second Wilms' tumour study.

The aims of the UKW2 study were: (1) to further refine treatment for stage I and II favourable histology (FH) patients; (2) to consolidate the UKW1 results for stage III FH patients; (3) to improve the outlook for patients with inoperable primary tumours and those patients with stage IV and unfavourable histology disease. Treatment consisted of primary nephrectomy, wherever possible, followed by chemotherapy and radiotherapy, as dictated by stage and histology. Treatment was refined successfully for stage I and II FH patients. The 4-year event-free survival for these two groups was 94% and 91%, respectively. Stage III FH patients had a 4-year event free survival of 84%. The outlook for patients with clear cell sarcoma of the kidney is as good as for patients with favourable histology, whilst that for patients with anaplastic or rhabdoid variants remains poor. The outlook for the majority of children with Wilms' tumour is now excellent.

Adolescent↗
PubMed2000

Successful treatment of aspergillus brain abscess in a child with acute lymphoblastic leukemia.

Cerebral aspergillosis carries a high mortality in immunocompromised patients. However, favorable outcome can be achieved by the prolonged use of antifungal agents and the maintenance of adequate drug levels. The authors report a 2-year-old girl who developed an aspergillus brain abscess during treatment for acute lymphoblastic leukemia. Predisposing factors for the fungal infection and details of the antifungal therapy are described. Prolonged treatment with AmBisome and 5-flucytosine successfully eradicated the lesion, but the girl's antileukemic therapy was compromised due to the infection. She developed a central nervous system and bone marrow relapse 9 and 15 months, respectively, after the initial presentation. The report emphasizes the need for further consideration of effective, long-term antifungal prophylaxis and a careful balance between aggressive treatment for severe infection and antileukemic therapy.

Amphotericin B↗
PubMed2000

The making of health visitors: an historical perspective part I.

The question of separate registration for health visitors has recently been reviewed. The separate registration of health visitors with the UKCC is to be retained. In the light of this decision, it is interesting to review how preparation for health visiting work has evolved, and the relationships between health visiting and nurse education. This historical perspective illuminates the reasons why health visitors may want to maintain the separate identity of their occupation, in nursing. In two papers, of which this is the first, the author outlines the history of the education and training of health visitors from its origins in the Victorian era to the present day. The first paper covers the period up to the early days of the NHS. The second paper will conclude with the most recent changes in the education of health visitors.

History, 19th Century↗
PubMed1999

The needs of neurology patients after discharge.

Patients discharged from neurosciences units and their carers often express a range of needs. Patients' needs relate to the illness and its physical effects. Carers experience mainly psychosocial effects. Needs can be met through planning and co-operation between the statutory and voluntary sectors and the use of liaison nurses.

Continuity of Patient Care↗