Biomedical subjects
Publications and source records attributed to F Raafat.
Two years after developing colonic Crohn's disease, a 17 year old boy presented with focal pulmonary consolidation. A lung biopsy specimen showed areas of non-caseating epithelioid granuloma. Although some respiratory abnormalities appear to be associated with inflammatory bowel disease, granulomatous disease affecting the lung has not previously been reported in a child.
A 2-year-old girl presented with isosexual precocity, and a juvenile granulosa cell tumor of the left ovary was removed. The tumor was unusual in that striated muscle differentiation of the tumor cells was demonstrated. This suggests that the mesenchymal cells retain the potential for divergent differentiation.
Serial sections of renal biopsies obtained from 44 nephrotic children with focal segmental glomerulosclerosis (FSGS) were reviewed in order to determine the glomerular location of segmental lesions and relate the findings to the outcome of illness. There were 23 boys and 21 girls aged 0.9-14.2 years at onset. FSGS was classified as "hilar" in biopsies containing at least one lesion contiguous with or involving the hilum, regardless of the location of other lesions, and as "peripheral" in the absence of hilar lesions. Of the 44 initial biopsies, 33 were designated hilar and 6 peripheral; the remaining 5 were unclassifiable as it was not possible to determine the location of 1-3 lesions in each biopsy. Twenty-eight of the 33 hilar biopsies also contained peripheral lesions, including paratubular (glomerular "tip") lesions in 15 instances. Paratubular lesions as the predominant abnormality were observed in only four biopsies. Repeat biopsies showed that transition occurred from one type to another, and only 4 biopsies remained with a final designation of peripheral FSGS. After a follow-up period of 1.6-24.9 years (mean 9.3), there was no significant difference in outcome between hilar and peripheral FSGS, whether diagnosed on the initial or repeat biopsy. The division into separate categories is not clearcut, and the use of this as a prognostic aid does not justify the additional cost of preparing and examining numerous serial sections.
The United Kingdom Children's Cancer Study Group's malignant germ cell tumor studies were undertaken to establish standard protocols for investigating, staging, and treating children, and to study the efficacy of new drug combinations and the value of serial measurement of serum alphafetoprotein (AFP) and human chorionic gonadotrophin (HCG). Boys with Stage I testicular tumors were treated by orchidectomy alone, whereas, after appropriate surgery, chemotherapy was recommended for children with more advanced testicular tumors or with tumors at other sites. From 1979 to 1987, 126 children aged 0 to younger than 16 years with malignant germ cell tumors were registered. They were similar to patients in other large pediatric series with respect to sites of origin, age at presentation in relationship to primary site, histology, female predominance for sacrococcygeal site, and presence of associated malformations (present in 17%). Serum AFP was measured in 123 patients and was elevated in 115, whereas HCG was raised in 19 of 77. Monitoring by serial AFP measurement proved valuable in assessing response to therapy and in early detection of tumor recurrence. When treatment results were assessed in February 1988, 101 of 122 patients were alive (four who received nonprotocol chemotherapy were excluded). Forty-four patients had been cured by surgery alone (41 with testicular tumors, two with ovarian tumors, and one with sacrococcygeal tumor). All of the remaining 78 children received chemotherapy. The initial low dose vincristine, actinomycin, and cyclophosphamide (LDVAC) regimen proved ineffective, actuarial survival at 5 years followup being 8% (12 patients), and a regimen of cisplatin, vinblastine, and bleomycin (PVB) caused unacceptable toxicity, with actuarial survival at 5 years follow-up being 67% (nine patients). Five-year actuarial survival was 87% for 17 children given high dose VAC with or without doxorubicin and 84% for 33 given bleomycin, etoposide, and cisplatin (BEP). All 7 children given various combinations of these regimens survived. Excluding the 12 LDVAC cases, patient survival by site was as follows: testis (59 patients, 100%); vagina, uterus, and prostate (four patients, 100%); ovary (25 patients, 88%); thorax (five patients, 40%), and other (four patients, 67%). Similarly, patient survival by stage was Stage I (62,97%), Stage II (14,86%); Stage III (18,83%); and Stage IV (16,72%). Survival by histology was analysed only in cases for which histologic review had been done the LDVAC cases were excluded.(ABSTRACT TRUNCATED AT 400 WORDS)
Benign tracheal tumours are uncommon in adults and rare in children. According to several extensive reviews, fibromas are the second most common benign tumour to occur in the trachea. (Gilbert et al., 1949, 1953). In our review of the literature, 197 adult cases of fibromas have been reported and 23 in children. However only one previous report of a congenital subglottic fibroma exists. (Tsui and Lore 1975). No previous case with a late onset vocal cord palsy has been reported.
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Salivary disease in childhood is uncommon. Clinical features are much the same as in adult disease, however, congenital, inflammation, and functional disease is more common. Management is governed by the same general principles in all age groups. Congenital abnormalities are rare. Inflammation, either bacterial or viral, is the most common disease. Chronic sialadenitis usually resolves at puberty. Neoplasia is rare, and the majority of tumors are benign and occur in the parotid gland. Malignancy is suggested by rapid growth, pain, skin fixation, and lymphadenopathy. Surgical treatment of sialorrhea should only be considered after careful assessment. Relocation of the submandibular ducts is the treatment of choice.
Measurements of glomerular basement membrane (GBM) thickness in children with minimal change nephrotic syndrome were made on electron micrographs of 43 biopsy specimens obtained from 35 children aged 1-13 years, of whom 13 were in remission at the time of biopsy. Measurements were also made in 2 children with non-glomerular disorders. A mean of 189 measurements per biopsy were made, using prints of x6,400-12,000 magnification. Quadratic curves were fitted by regression analysis to the mean GBM thickness for each of the 35 initial nephrotic biopsies, plotted against age for the whole group and separately for sex, race and the presence or absence of proteinuria at the time of biopsy. There were no significant differences in race or proteinuria, but there was a trend towards a thicker GBM in young males which disappeared by 9 years of age. From the mean +/- 2 SD of GBM thickness for each of the 35 initial nephrotic biopsies the calculated thickness ranged from 100-340 nm at 1 year of age to 190-440 nm at 9 years of age and older. The growth curve for GBM thickness increases steeply initially, reaching a plateau at 9 years of age. Because there was no significant difference between proteinuric patients and those in remission, while measurements in the nephrotic syndrome and non-glomerular disorders closely agreed, we believe that our results demonstrate the normal GBM thickness and growth pattern.
Forty-one confirmed cases of childhood neuroblastoma diagnosed over a 13-year period were reviewed and reclassified. Most of the tumors were stained using a peroxidase antiperoxidase method for neuron specific enolase (NSE), protein gene product (PGP) 9.5, and S100 protein, all of which have previously been reported to be positive in some neuroblastomas. The relation to prognosis of the histology and immunohistochemistry was studied. There was a significant trend toward improved survival with increasing degree of differentiation, and with decreasing mitosiskaryorrhexis index (MKI) in the stroma-poor group. There was no significant correlation between immunohistochemical staining and survival, although the presence and amount of staining for all three markers tended to increase with tumor differentiation. This study concludes that histologic classification in neuroblastoma is helpful in assessing prognosis but that the clinical features are generally more reliable as indicators of prognosis. The immunohistochemistry of markers used did not contribute towards assessment of prognosis.
A generalized ichthyosiform eruption developed in a 13-year-old boy three weeks before presentation with a paravertebral rhabdomyosarcoma. His skin returned to normal shortly after the onset of chemotherapy and remission of the tumor. This case is unusual because the ichthyosis developed acutely, following a generalized urticarial eruption, and also involved the palms and soles.
A dual sugar (mannitol, lactulose) absorption test was evaluated using an iso-osmolar oral dose in two groups of children: a study group of 43 children divided into five subgroups, based on severity of mucosal damage, and a control group of 53 children with histologically normal jejunal biopsy specimens. After an oral dose, the three hour urinary mannitol: lactulose ratios in the control group showed a highly significant positive correlation with body surface area. After correction for the body surface area relationship, a control lower limit was defined by the mean -2SD of the log10 transformed control mannitol: lactulose ratios. Specificity and sensitivity for severe villous atrophy was 98% and 95% respectively but the sensitivity declined rapidly with decreasing degrees of mucosal damage, and the test would not therefore be an adequate screening procedure for all enteropathies. In sequential studies in 18 children, the changes in the mannitol: lactulose ratio were consistent with the changes in mucosal structure induced by gluten challenge or gluten withdrawal. The test may therefore have a role in any sequential study of lesions of the mucosa of the small intestine.
A silver colloid technique to identify nucleolar organiser region associated protein (AgNOR) was applied to 16 fibrous proliferations of childhood and six low grade fibrosarcomas. The fibrous proliferations comprised five cases of infantile digital fibromatosis, seven of infantile desmoid type fibromatosis, and four of infantile myofibromatosis. The AgNORs were visualised as dots within the nuclei of the cells, and on the basis of their relative mean numbers of AgNORs fibrous proliferations of childhood could be easily differentiated from low grade infantile fibrosarcoma. The differences observed were significant (0.01 greater than p greater than 0.001). This technique, previously the province of the cytogeneticist, may be of use to the pathologist in differentiating infantile fibrous proliferations.
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Twenty confirmed cases of childhood neuroblastoma diagnosed over six years were reviewed and classified according to the subtyping proposed by Shimada et al. The tumours were stained using a silver colloid method for nucleolar organiser regions (NORs), and the mean number of NORs for every 200 cells was calculated. The correlation between the mean number of NORs and histology and survival was studied. There was a significant correlation between the mean numbers of NORs and differentiation, and with the mitosis-karyorrhexis index (MKI) in the stroma poor group (p = 0.01-0.001). A trend to increased survival with decreased numbers of NORs was observed in the study group as a whole (rank order of correlation = -0.57, p = 0.05-0.02). It is suggested that mean number of NORs is of prognostic value in neuroblastomas.
An immunological study of B cells in patients with colitis that was associated with Hirschsprung's disease was undertaken and compared with that in patients with ulcerative colitis and in normal controls. There was an appreciable increase in IgA and a decrease in IgG in both disease groups. Humoral reaction in colitis associated with Hirschsprung's disease seems to be indistinguishable from that in ulcerative colitis and possibly in other inflammatory bowel diseases.
Benign cystic mesothelioma of the peritoneum is a well-defined but rare entity. A total of 35 instances have been reported, almost all in adults, the majority females. Cystic mesotheliomas have, until recently, been labeled cystic lymphangiomas or lymphohemangiomas. We report the case of a 14-year-old boy who, despite only subtotal resection of his abdominal mass, is well and recurrence free 9 months after surgery. The role of immunohistochemistry and electron microscopy in diagnosis is emphasized.
We present a case of diffuse malignant peritoneal mesothelioma, initially misdiagnosed as benign. Electron microscopy and immunocytochemistry proved helpful diagnostically. Using monoclonal antibodies against cytokeratin and vimentin, we compared neoplastic with normal and reactive mesothelia and we found coexpression of these two intermediate filaments in the reactive and neoplastic mesothelial but not in the normal mesothelia, supporting the suggestion that surface mesothelial cells are derived from multipotential submesothelial cells.