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F Raafat

Publications and source records attributed to F Raafat.

At least 37 records · Page 2Linked to original sources
PubMed1996

The role of Epstein-Barr virus in Hodgkin's disease from different geographical areas.

Recent studies have suggested that Epstein-Barr virus (EBV) may play a role in the aetiology of Hodgkin's disease. To determine the role of EBV in childhood Hodgkin's disease in different geographical areas, immunohistochemical staining and in situ hybridisation were used to analyse latent membrane protein 1 (LMP 1) and small nuclear non-transcribed RNAs (EBER-1) respectively. Testing for EBV within the Reed-Sternberg and Hodgkin's cells was carried out in childhood Hodgkin's disease from 10 different countries. The proportion of LMP 1 positive cases varied significantly, being 50% of cases from the United Kingdom (38/75), South Africa (9/18), Egypt (7/14), and Jordan (8/16), 60% from the United Arab Emirates (6/10), 70% from Australia (11/16), 81% from Costa Rica (34/42), 88% from Iran (7/8), 90% from Greece (20/22), and 100% of the 56 cases from Kenya. A sensitive polymerase chain reaction based EBV strain typing technique was established using archival tissues. EBV strain type 1 was shown to be predominant in childhood Hodgkin's disease from the United Kingdom, South Africa, Australia, and Greece. Type 2 was predominant in Egypt. EBV strain types 1 and 2 were both detected in some cases of childhood Hodgkin's disease in the United Kingdom, Costa Rica, and Kenya. The high incidence of EBV and the presence especially in developing countries of dual infection with both strain types 1 and 2 may reflect socioeconomic conditions leading to malnutrition induced immunological impairment. The possibility of HIV infection also needs to be explored.

Adolescent↗
PubMed1995

Secondary amyloidosis from long-standing bacterial endocarditis.

Survival of patients with increasingly complex congenital heart disease has produced a population of children and adolescents who are susceptible to subacute bacterial endocarditis (SBE). We report a child whose endocarditis went unrecognised, and who developed amyloidosis. Asymptomatic proteinuria, haematuria and renal impairment are occasionally seen in SBE and usually indicate glomerulonephritis. Amyloidosis should also be suspected in children with long-standing bacterial endocarditis with proteinuria or other evidence of renal impairment, especially if associated with organomegaly. The diagnosis is made by renal biopsy.

Amyloidosis↗
PubMed1995

Neonatal pulmonary vascular disease in hypoplastic left heart syndrome.

A term neonate with hypoplastic left heart syndrome underwent surgical palliation using the Norwood procedure on day 2 of life. The postoperative course was complicated by recurrent systemic desaturation leading to death. Postmortem histologic examination of lung tissue confirmed the clinical impression of fixed pulmonary vascular disease. Some neonates with a restrictive interatrial communication may have pulmonary vascular disease at birth, which will negatively influence survival irrespective of the type of palliation undertaken.

Fatal Outcome↗
PubMed1995

Juvenile hyaline fibromatosis.

A case of juvenile hyaline fibromatosis in a 7-year-old Asian boy is presented. This autosomal recessive inherited condition has not been described in the otolaryngology literature before. We demonstrate the benefits surgical intervention, for treatment of gingival hypertrophy, can bring to the patient and outline the other features of this rare condition. The literature on juvenile hyaline fibromatosis and infantile systemic hyalinosis is also reviewed.

Child↗
PubMed1995

Glomerular morphometry in childhood reflux nephropathy, emphasizing the capillary changes.

As a consequence of nephron loss, reflux nephropathy (RN) causes considerable glomerular hypertrophy. To examine the relative contributions of capillary dilatation and growth in producing hypertrophy, glomeruli contained in unscarred areas of renal biopsies from 19 children with RN were compared with those in 16 children with minimal change nephrotic syndrome and 16 with recurrent hematuria, who were used as controls representing normal childhood growth. Using computerized digitometry we measured the mean glomerular tuft area (GTA) in all complete, undistorted, nonsclerotic glomeruli in periodic acid-Schiff (PAS) stains. Measurements were repeated in four glomeruli of uniform size in periodic acid-silver methenamine stains, the results (GTA4) correlating significantly with GTA. In the same four glomeruli we measured the mean individual capillary luminal area (CLA) and counted the mean number of lumens per glomerulus (N). Mean mesangial area (MA) was calculated as GTA4 - (CLA x N). Cells per distal mesangial region were counted in PAS stains. GTA, GTA4, N, MA and mesangial cell counts were significantly greater in RN than controls, but CLA and fractional MA (MA/GTA%) did not differ. N correlated highly significantly with GTA4 in both RN and controls, but CLA did not do so. These findings are consistent with capillary growth by subdivision being the main mechanism of glomerular hypertrophy when nephron loss occurs during childhood, and the identity of the regressions of N versus GTA4 in RN and controls suggests that compensatory hypertrophy resembles the normal glomerular growth pattern in this age group.

Adolescent↗
PubMed1994

Human glomerular growth during childhood: a morphometric study.

Morphometric measurements of glomerular size were made on renal biopsy specimens obtained from 16 children with minimal change nephrotic syndrome (MCNS) and 16 with isolated recurrent haematuria (RH), whose ages ranged from 1.8 to 15.2 years. Glomeruli were normal on light and electron microscopy, and immunofluorescence was essentially negative. Tracings were made of the outlines of glomerular capsules, tufts, and individual capillary lumens; using computerized digitometry, the mean areas of each item were determined. The number of capillary lumens per glomerulus was counted automatically. From these data, the mean mesangial area was also calculated. Mesangial cells were counted in at least 100 distal mesangial areas per biopsy. The mean glomerular capsular area (GCA) correlated well with the mean glomerular tuft area (GTA) but frequent capsular artefacts rendered the former measurement less suitable for comparison with diseased glomeruli. The mean GTA correlated significantly with age and body surface area in both MCNS and RH, as did the number of capillary lumens per glomerulus, but the mean capillary lumina area showed no such correlations. The mean mesangial area increased with age in proportion to GTA, whereas mesangial cell counts remained normal. These findings indicate that glomerular growth during childhood is proportional to body growth, and are consistent with capillary subdivision as the mechanism of enlargement.

Adolescent↗
PubMed1994

Intestinal microvillous dystrophy: a variant of microvillous inclusion disease or a new entity?

We report three patients with intestinal microvillous dystrophy, two of whom were siblings. The relatively delayed clinical presentation and the lack of classical microvillous inclusions distinguish these cases from the previously described microvillous inclusion disease (MVID). There appears to be an underrecognized spectrum of microvillous disorders leading to fatal intractable secretory diarrhea in infants. In our three cases the diagnosis was suggested by periodic acid-Schiff (PAS) and alkaline phosphatase preparations of a jejunal biopsy specimen showing thinning or absence of brush border staining, which was confirmed by electron microscopy. The latter showed poorly developed and haphazardly arranged microvilli with intracytoplasmic vesicular bodies but no true inclusions. As in MVID, the prognosis of intestinal microvillous dystrophy is poor. The occurrence of the disease in two siblings of consanguinous parents suggests an autosomal recessive inheritance, and like MVID, genetic counselling of affected families is essential.

Female↗
PubMed1994

Ewing's sarcoma of the retropharynx.

Ewing's sarcoma arising in the head and neck region is very rare. A case arising from C2 in a young child is presented. The unusual presentation, differential diagnosis and pathological features are discussed.

Bone Neoplasms↗
PubMed1994

DNA ploidy and proliferative activity (S-phase) in childhood soft-tissue sarcomas: their value as prognostic indicators.

The value of DNA ploidy as a prognostic indicator is well established in many cancers, but recent studies in childhood rhabdomyosarcoma (RMS) have been contradictory. In a retrospective study of 128 cases of soft-tissue sarcoma (STS) diagnosed since 1980, the prognostic value of clinical, histological and flow cytometric parameters was compared, using univariate and multivariate methods. Eighty-one RMSs, 18 extraosseous Ewing's (EOE)/peripheral neuroectodermal tumours (PNETs) and 29 other non-RMS STSs were histologically and clinically reviewed. Five year actuarial survival was 63.4% for all STSs and 69.4% for RMSs. Paraffin-embedded tissue blocks were available for flow cytometry in 90 cases. Of the RMSs, 65.5% were aneuploid [DNA index (DI) > 1.1] compared with 23% of the EOE/PNETs and 31% of non-RMS STSs. Median S-phase was also significantly higher in RMSs (17.0%) than in other STSs (10.8%) (P = 0.0023). Univariate analysis in RMSs showed that stage, ploidy status, S-phase, site and tumour size all had a significant impact on survival. In multivariate analysis of 59 cases of RMS, one clinical and two flow cytometric parameters were independently associated with poor prognosis. These were stage (IV), nonhyperdiploidy (DI < 1.10 and > 1.8) and a high rate of proliferative activity (S-phase > 14.0%). These results confirm that ploidy and S-phase are important new prognostic indicators in rhabdomyosarcoma.

Adolescent↗
PubMed1994

Siblings with microvillous inclusion disease.

Two male siblings from a consanguinous Pakistani family had fatal diarrhoea with an onset at 24 and 48 hours after birth. A diagnosis of microvillous inclusion disease (MVID) was established by showing characteristic light and electron microscopic features in the small intestinal biopsy specimen on day 6 of life in case 1. The typical abnormalities of MVID were also demonstrated retrospectively in case 2 by examining archival appendicular tissue from 10 years previously. These cases are consistent with an autosomal recessive inheritance for MVID. Retrospective diagnosis of MVID is possible by examining appropriate archival material, which may aid genetic counselling and future research.

Appendix↗
PubMed1993

Carcinoid tumours of the appendix in children 1957-1986: incidence, treatment and outcome.

Forty carcinoid tumours of the appendix were identified in children under 15 years of age between 1957 and 1986 from the records of the West Midlands Regional Children's Tumour Research Group. A significant excess of girls was observed (P = 0.02). All tumours were discovered incidentally and two patients underwent further surgery. Twenty-two of the 29 confirmed locations (76 per cent) were at the tip of the appendix and 34 of the 40 tumours (85 per cent) had invaded beyond the appendiceal submucosa. The reported incidence rate of 1.14 per million children per year remained constant throughout the study period. No recurrences, metastases or deaths from tumour were detected. Appendicectomy alone is confirmed as usually curative, although right hemicolectomy is occasionally necessary. Caution during routine appendicectomy is recommended to avoid possible tumour damage, as is consistent histological examination of all appendices in the paediatric age group. The importance of long-term follow-up is emphasized.

Adolescent↗
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↗