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F Raafat

Publications and source records attributed to F Raafat.

At least 55 records · Page 3Linked to original sources
PubMed1993

Thirty-year population-based review of childhood renal tumours with an assessment of prognostic features including tumour DNA characteristics.

We have reviewed all paediatric kidney tumours seen in the West Midlands Health Authority Region over a 30-year period. There were 205 cases confirmed after a review of the pathology by three paediatric pathologists. Seven were cases of bone metastasising renal tumour (clear cell sarcoma), 5 were rhabdoid tumours, 2 were renal cell carcinomas, and 13 were mesoblastic nephromas. In 3 cases, it was not possible to define further the histological diagnosis. The remaining 175 cases were considered to be Wilms' tumour (86%), which is equivalent to an incidence of 5.7/10(6)/year. In the cases of Wilms' tumour, there were 91 boys and 84 girls (1.1:1). The majority of patients were Caucasian with only 7% of non-Caucasian origin. At presentation, 78% of the patients were less than 5 years old. All of these patients except 9 had surgery as part of their treatment, 154 children had total nephrectomy, 3 had partial nephrectomy, and 9 had other surgical procedures. The majority also received chemotherapy and radiotherapy. Sex, chemotherapy, and stage all had prognostic significance in univariate analysis. The actuarial survival at 10 years increased from 17% for patients treated in the first decade of the study to 78% for patients treated in the third. DNA characteristics were investigated using flow cytometry in paraffin-embedded material and adequate information was obtained in 73 cases of Wilms' tumour. Only 7 had aneuploid tumours. Univariate survival analysis of these 73 results showed that stage, sex, the percentage of cells in the synthetic phase and the proliferative index from the DNA investigations had predictive value.

Actuarial Analysis↗
PubMed1993

Neuroblastoma: a 32-year population-based study--implications for screening.

This paper describes a retrospective population-based study of neuroblastoma in the West Midlands Health Authority Region--childhood population 1.12 million (OPCS, census 1981)--in which 239 cases were diagnosed between 1st January 1957 and 31st December 1988. The age standardised rate of tumour incidence has remained constant at 7.2 cases per million children per year. The median age at diagnosis was 2 years with 18% of children presenting before the age of 6 months. Fifty children (21%) presented before the age of one year, and for this group of children, the prognosis has improved significantly over the 32-year period (10 year survival increasing from 63% in 1957-67 to 87% in 1978-88), whereas for the 189 (79%) children who presented after one year of age, the prognosis has remained very poor during the study period (10 year survival 1957-67 = 9.5%, 1978-88 = 8.5%). This study supports the need for a prospective study of mass screening at several intervals rather than only at 6 months of age.

Abdominal Neoplasms↗
PubMed1993

Cancer associated haemolytic uraemic syndrome developing prior to treatment with cytotoxic agents.

The haemolytic uraemic syndrome has been reported in children and adults with neoplasias treated with mitomycin C and occasionally in adults prior to chemotherapeutic treatment, but it has not been reported in a child prior to the use of cytotoxic drugs. This case report concerns the development of the haemolytic uraemic syndrome in a 7-year-old male child with a metastatic hepatocellular carcinoma. The child had persisting haemolysis and renal impairment, and died three weeks after presentation. Though our experience with similar tumours suggested the outlook would be poor, we had hoped to provide palliative chemotherapy. This proved impossible as optimal chemotherapy could not be used because of the renal impairment.

Anemia, Hemolytic↗
PubMed1993

Malignant epithelial tumours in children: incidence and aetiology.

The purpose of this study was to establish the incidence of carcinomas in children, changes in incidence over a 30-year period, and to identify features of possible aetiological significance. A total of 173 cases were identified, but after review of the histopathology, 30 patients were excluded because they were considered to have benign epithelial tumours or malignant tumours of nonepithelial origin. Seven other cases were excluded because pathology material was not available. Overall, in 28% of cases, the diagnoses were changed by pathology review. Thus, 136 children in the West Midlands Region diagnosed 1957-1986 were included, with carcinoid tumours (44) and tumours of skin (22), nasopharynx (14), salivary gland (13), adrenal cortex (13), thyroid (9), large bowel (5), other (16). Excluding carcinoids, the age-standardised incidence rate was 2.4 x 10(6) per year. Male:female ratio was 0.7:1 and 66% were aged > 10 years. Incidence increased from 1.5 to 3.3 x 10(6) per year. Genetic factors predisposing to carcinoma included tyrosinosis, MEN II and III, congenital adrenal hyperplasia and basal cell naevus syndrome. There was a case of Li-Fraumeni syndrome and several other patients had relevant family histories. Probable "environmental" causes included antenatal exposure to stilboestrol or hydroxyprogesterone hexanoate, stilboestrol given for premature menarche, neonatal hepatitis and prior radiotherapy. The aetiology of carcinomas in children is multifactorial, both genetic and environmental factors being important. The incidence is increasing.

Adolescent↗
PubMed1993

Renal parenchymal malakoplakia--a case report and review of the literature.

Malakoplakia is an inflammatory condition associated with persisting bacterial antigen in macrophages and characterized histologically by the Michaelis-Gutmann body, containing bacterial fragments. We review the pathogenesis of malakoplakia and report a novel form of treatment successfully used in an 8-week-old infant with bilateral renal malakoplakia. The patient presented with an acute Escherichia coli urinary tract infection and enlarged kidneys. Antibiotic regimes were ineffective, but once the diagnosis was made treatment was changed to an immunosuppressive regime of prednisolone and azathioprine, to which she responded promptly. Renal malakoplakia should be considered in any patient with a urinary tract infection unresponsive to antibiotics and enlarged kidneys. Although a large proportion of patients with malakoplakia have an underlying systemic disorder, which may account for their abnormal macrophage function, the rest demonstrate either an isolated macrophage defect or no detectable anomaly at all. It is in this latter group we suggest that an immunomodulating regime can be curative.

Azathioprine↗
PubMed1993

Glomerular morphometry in reflux nephropathy: functional and radiological correlations.

Using computerized digitometry, we investigated the relationships between renal size, glomerular filtration rate (GFR), proteinuria, incidence of segmental and global glomerulosclerosis, glomerular size, hilar arteriolar wall thickness and hyaline deposition in renal biopsies obtained from 24 children and adolescents with reflux nephropathy, of whom only 4 were hypertensive. Age-matched controls comprised minimal-change nephrotic syndrome (6) and recurrent haematuria with normal biopsy (13). The mean sectional area of patients' glomeruli was double that of controls. Glomerular size correlated with the amount of proteinuria (measured as protein/creatinine ratios in early morning urine) and inversely with renal size and GFR. Segmental sclerosis, invariably of hilar origin, was observed in 8 patients and the percentage of glomeruli affected correlated strongly with glomerular size and proteinuria. Global sclerosis was found equally in patients and controls, and showed no similar correlations. Compared with controls, patients' hilar arterioles showed increased wall thickness, more intramural hyaline deposits and decreased luminal diameter when related to glomerular size. The proteinuria and glomerular changes are consistent with hyperfiltration, while the previously undescribed hilar vascular changes, which both precede and accompany sclerosis, resemble abnormalities reported experimentally following renal ablation.

Adolescent↗
PubMed1993

Distinctive patterns of renal neoplasms containing Tamm-Horsfall protein.

Sections of 114 renal neoplasms from adults, and 2 renal rhabdoid tumours from children, were examined by an indirect immunoperoxidase method using two antibodies to Tamm-Horsfall protein. Forty-five of the adult neoplasms were also examined with an antibody to proximal tubular brush border. Tamm-Horsfall protein is normally only found in the cells of the thick limb of the loop of Henle, and there are widely divergent reports on its occurrence in renal neoplasms. In the present series, Tamm-Horsfall protein was detected in parts of 31 neoplasms. Four distinctive patterns of cell contained the protein: cells with a paranuclear inclusion typical of rhabdoid tumours; 'plasma rich' cells, which were large cells with cytoplasm that was centrally dense and peripherally clear; eosinophilic cells forming one type of papillary structure; and giant cells. The areas containing Tamm-Horsfall protein did not express markers of proximal tubular brush border, and appeared white to the naked eye, rather than the yellow of typical clear cell carcinomas. Tamm-Horsfall protein can therefore be found in renal neoplasms. The four distinctive patterns of positive cells appear to represent neoplastic phenotypes of thick limb cells. This has implications for the classification of renal neoplasms and for theories of their origin.

Adult↗
PubMed1993

Crohn's disease with pulmonary involvement in a 3 year old boy.

Extra-intestinal or metastatic Crohn's disease is being recognised increasingly, most commonly in the skin and genitourinary system. Only very rarely has it been reported in the lung. A 3 year old boy who presented with swollen gums and a persistent abnormality on his chest x ray is reported. Lung biopsy specimens showed multiple non-caseating epithelioid granulomas. Subsequent investigation showed the presence of intestinal Crohn's disease. The evidence for abnormal lung function in Crohn's disease and the possible pathogenesis of metastatic pulmonary Crohn's disease are discussed.

Child, Preschool↗
PubMed1993

Retrospective study of prognostic value of DNA ploidy and proliferative activity in neuroblastoma.

AIM: To assess the prognostic value of age and stage at diagnosis, site of primary tumour, cell ploidy and N-myc copy number in children with neuroblastoma. METHODS: Flow cytometry was used to determine the cellular DNA content of paraffin wax embedded archival material from 69 cases of neuroblastoma and was successful in 52. RESULTS: The age, stage, and survival distribution of the sampled cases was not significantly different from that in a larger population based series. There were seven diploid ("non-aneuploid") and 45 aneuploid (including two tetraploid and four triploid) tumours. The 10 year survival was significantly better for cases of aneuploid rather than diploid tumours (p < 0.05). An important new finding was that 10 year survival was also significantly better for tumours with a low percentage of cells in S phase (p < 0.03). CONCLUSION: The percentage of cells in S phase, a measure of the proliferative activity of the tumour, correlated with prognosis in neuroblastoma. This should be measured with other biological features of the disease, such as N-myc copy number, when prognostic indicators are being assessed.

Adolescent↗
PubMed1992

Epstein-Barr virus (EBV) and Hodgkin's disease in children: incidence of EBV latent membrane protein in malignant cells.

Previous studies have detected EBV DNA by Southern blotting or in situ hybridization in biopsy material from up to 30 per cent of adult cases of Hodgkin's disease. Here we have used monoclonal antibodies specific for the EBV latent membrane protein LMP1 to examine archival material from children with Hodgkin's disease. Material from 74 cases (54 males and 20 females) was examined and 37 (30 males and 7 females) were classified as LMP1-positive in the malignant cells. LMP1 positivity was present in 4/13 (31 per cent) of lymphocyte predominant, 14/36 (39 per cent) of nodular sclerosis, 17/20 (85 per cent) of mixed cellularity, 1/2 (50 per cent) of lymphocyte depletion, and 1/3 (33 per cent) of unclassified subtypes. The positive cases by clinical stage were I 9/22 (41 per cent), II 9/20 (45 per cent), III 11/24 (46 per cent), and IV 8/8 (100 per cent). LMP1 positivity was present in 2/5 (40 per cent) children aged less than 5 years, 12/27 (44 per cent) aged 5-10 years, and 23/42 (48 per cent) aged between 10 and 15 years. The association between EBV and Hodgkin's disease in children thus appeared to be more frequent in patients with mixed cellularity and advanced disease, but examples of EBV-positive tumours were found in all histological subtypes, stages, and ages. Stepwise discriminant function analysis showed that clinical stage IV and mixed cellularity histology are independently associated with LMP1 positivity. These observations indicate that Hodgkin's disease in children is at least as strongly linked to EBV as is the disease in adults.

Adolescent↗
PubMed1991

Hypereosinophilic syndrome in childhood appearing as inflammatory bowel disease.

A 7-year-old girl with hypereosinophilic syndrome is described, the first such patient to present with inflammatory bowel disease. Hepatosplenomegaly, anaemia, and hypergammaglobulinaemia were also prominent features. Colonoscopy revealed a frank colitis of nonspecific histologic appearance. An elemental diet was unhelpful, but there was a prompt clinical and partial haematological response to systemic steroids. She has remained well receiving a small maintenance dose of prednisolone for 3 years.

Child↗
PubMed1991

Congenital mesoblastic nephroma: possible prognostic and management value of assessing DNA content.

The case records and pathology of all children with kidney tumours treated in the West Midlands Health Authority Region (WMHAR) from 1957 to 1986 were reviewed. The histology was reviewed by a panel of three paediatric pathologists. Thirteen (6%) out of 211 cases were considered to have congenital mesoblastic nephroma (CMN). Nine were of the conventional type, three of the atypical cellular type, and one mixed. DNA ploidy was investigated and showed two of the tumours to be aneuploid and nine diploid (tissue was not available in the two other cases). The two aneuploid tumours were of atypical cellular and mixed histology, respectively; the diploid tumours were of the conventional type in eight cases and atypical cellular in one. The atypical cellular type has been reported to behave more aggressively, but the benefit of additional treatment after surgery to prevent recurrence remains unclear. Measurement of DNA content by flow cytometry, together with histological subclassification, may be useful in selecting patients who will benefit from further treatment after surgery.

DNA, Neoplasm↗
PubMed1990

The glomerular changes in children with reflux nephropathy.

While heavy proteinuria and focal segmental glomerulosclerosis (FSGS) are well-recognized features of progressive reflux nephropathy in adults, little is known of their early evolution. We have studied the glomerular changes in renal biopsy specimens obtained from 24 patients aged 5.2-18.8 years, in whom urinary protein excretion was measured as early morning urine protein creatinine ratios, using the Coomassie blue dye-binding method. Segmental sclerotic lesions were found in eight biopsies and traced through serial sections to a hilar origin in every instance. There was a strong positive correlation between the extent of glomerular involvement and the amount of proteinuria (P less than 0.0001). Parahilar hyaline deposits were observed in 16 biopsies, including five of the eight showing FSGS. All unsclerosed glomeruli were enlarged, and the hilar arterioles showed both enlargement and thickening, their walls frequently containing subendothelial hyaline deposits. Since in most patients renal function was comparatively well preserved, despite extensive loss of renal substances, we believe that these glomerular and vascular changes represent the stages in the evolution of hyperfiltration. Microproteinuria is the earliest clinical manifestation of FSGS, and should be sought routinely in all patients with reflux nephropathy.

Adolescent↗
PubMed1990

The UK Children's Cancer Study Group: testicular malignant germ cell tumours 1979-1988.

The United Kingdom Children's Cancer Study Group (UKCCSG) malignant germ cell tumour (MGCT) studies were undertaken to establish standard protocols of investigation, staging, and treatment. The efficacy of new drug combinations and the value of serial measurements of serum alphafetoprotein (AFP) and human chorionic gonadotrophin (HCG) were evaluated. Following the initial surgery, staging of the tumour was performed using a variety of investigative approaches. In stage 1 testicular tumours, orchidectomy was performed. In more advanced tumours, and in stage 1 tumours that failed to show the expected decline in AFP or recurred, chemotherapy was used after appropriate surgery. Seventy-three boys, under 14 years of age, with testicular MGCTs have been entered into the UKCCSG studies since 1979. Serum AFP was measured preoperatively, or within 2 weeks of operation, in 70 boys. It was unequivocally elevated in 69. Monitoring by serial AFP measurement proved valuable in assessing response and in early detection of recurrence. HCG was measured in 46 boys, and was raised in three. Sixty-seven (91%) of the tumours were yolk sac (Teilum) tumours, four were immature teratoma, and two were mixed MGCTs. The only non-AFP producing tumour was an immature polydermal teratoma in a 1-year-old boy. Serum HCG was raised in three boys with yolk sac tumours, one with a mixed teratoma, and one 14-year-old boy who had a mixed MGCT. The results of treatment were assessed on April 1, 1989 (median time from diagnosis, 3 years 4 months). Seventy-one boys were alive, 48 of whom had been cured by orchidectomy alone. The remaining 25 patients received chemotherapy.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1990

'Clustering'--real or apparent? Probability maps of childhood cancer in the West Midlands Health Authority region.

The West Midlands Regional Children's Tumour Registry collects detailed information on all cases of childhood cancer in the West Midlands Health Authority Region (WMHAR). The distribution by electoral ward of all cases diagnosed in the WMHAR between 1980 and 1984 has been determined. Analysis has also been performed for leukaemias/non-Hodgkin's lymphomas alone. We suggest that this latter grouping should be universally employed, owing to the difficulty of accurately separating out cases of leukaemia. Both spatial analyses showed several wards with significantly excessive rates on the basis of their cumulative Poison probability. Observed/expected ratios of 3-35 were seen for cases in significant wards, which are similar to the ratios seen in analysis of incidence around nuclear installations. However, further detailed consideration of these individual significance levels in the light of the number of statistically significant wards which would occur by chance alone, due to the multiple use of the test, accounted completely for the number of wards obtained in each of the groups considered. Thus, apparent 'clustering' of cases could be mere statistical artefact. In the WMHAR, therefore, using the technique of probability mapping, no true spatial pattern of incidence was found, other than that which would occur by chance alone. This, in a large area without nuclear installations and an even mix of rural and industrialised regions, could be seen as control data for those studies which have considered cases of childhood leukaemia around nuclear facilities, where the observation of single point clusters associated with suspected sites restricts assessments of spatial pattern in the rest of the area.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1990

Eosinophilic proctitis with giant cells: a manifestation of cow's milk protein intolerance.

Two infants, subsequently shown to have cow's milk protein intolerance, presented with rectal bleeding in the first week of life. Rectal biopsies showed eosinophilic infiltration of the mucosa with several multinucleated giant cells. IgG, IgM, and IgA containing plasma cells were present but IgE could not be detected in rectal biopsies. While cow's milk protein intolerance is a well-recognized cause of proctocolitis in infancy, eosinophilic granulomatous-like lesions with giant cells may be a prominent feature and do not exclude the diagnosis.

Biopsy↗
PubMed1990

Malignant hepatic tumours in children: incidence, clinical features and aetiology.

Congenital defects and other disorders have been reported in association with malignant liver tumours. In order to assess their significance, a population-based survey was undertaken on children aged less than 15 years diagnosed with malignant liver tumours during the 30 years 1957-1986. The cases were identified from information collected by the West Midlands Regional Children's Tumour Registry. Pertinent data were extracted from their clinical records, and the original biopsy and any necropsy material were reviewed by a panel of three paediatric pathologists. Of the 50 eligible cases registered, eight were excluded because histology review showed that they had non-malignant conditions (3) or malignancies of extrahepatic origin (4) or because no pathological material was available (1). The diagnoses in the remaining 42 cases were hepatoblastoma (27), hepatocellular carcinoma (3), rhabdomyosarcoma (6), rhabdoid tumour (3) and yolk sac tumour (3). The incidence of primary malignant liver tumours was 1.20 per 10(6) person years and that of the hepatoblastoma sub-group was 0.77 (average childhood population of the West Midlands for the time period being 1,166,500). The presenting clinical, radiological and biochemical features were similar to those reported in other series and the ethnic and social class distributions were unremarkable compared with the local population. Congenital defects or other possibly related features were present in nine (21%) patients. Our results, taken with other reports, suggest that hepatoblastoma is a malignant tumour related to maldevelopment, possibly associated with 11p or 5q mutations, whereas hepatocellular carcinoma is more usually a complication of metabolic and other disorders which lead to cirrhosis.

Child↗