Medical imaging in pediatric ophthalmology

The use of CT and MR imaging has improved the understanding of many congenital and acquired conditions in pediatric ophthalmology, resulting in more appropriate therapeutic intervention and giving insight into the pathogenesis of these conditions.     

Expression of the lipogenic enzyme fatty acid synthase (FAS) as a predictor of poor outcome in nephroblastoma: an interinstitutional study

BACKGROUND: Treatment of nephroblastoma (Wilms tumor) has presently achieved a greater than 80% cure rate. Pathologic stage and grade are considered the most reliable prognostic parameters, but other biologic factors are under study in order to improve patient stratification into risk groups. Correlation of elevated levels of the lipogenic enzyme fatty acid synthase (FAS) with aggressiveness of some cancers has drawn attention to this enzyme as a possible marker of poor prognosis. PROCEDURE: To determine the predictive strength of FAS expression in Wilms tumor (with particular emphasis on intermediate risk, i.e., non anaplastic tumors, the vast majority of nephroblastomas), we evaluated immunostaining expression in archival specimens from 94 neoplasms. The degree of expression was correlated with stage, grade, clinical course and administration of prenephrectomy chemotherapy. RESULTS: Expression of FAS increased in anaplastic tumors (P = 0.043) and higher stages (P = 0.029). FAS expression correlated with OS and DFS at both univariate and multivariate analysis. Comparable results were obtained when analyzing the intermediate risk population separately. Pretreatment resulted in an increased FAS expression, without reaching significance level (P = 0.059). CONCLUSIONS: Expression of FAS might be an independent prognostic factor, particularly for intermediate-risk patients. The blockade of fatty acid synthesis by inhibition of FAS enzymatic function by means of metabolic analogues might prove a novel target pathway for the treatment of nephroblastoma.     

Kawasaki disease in Oman--a clinical study

A total of 39 patients were diagnosed to have Kawasaki disease by the standard diagnostic criteria, at The Royal Hospital, Muscat, Oman, during the period January 1995 to August 2002. A retrospective analysis of the case records of the patients with the diagnosis of Kawasaki disease was done. The results of the clinical features and the laboratory manifestations of the patients, who are from the Middle East region, are presented. The peak age at presentation was 6-30 months, mean 29 months. Peak months of the occurrence of the disease were March and October. Oral lesions were found in all cases. The mean duration of hospital stay for these patients was 8.5 days, range 2-21 days. The total duration of fever ranged from 6 to 21 days, with a mean value of 9.4 days. An elevated erythrocytic sedimentation rate (ESR; 91.7 per cent) and a raised C-reactive protein (C-RP; 92.3 per cent) were the most significant laboratory findings. Echocardiographic abnormalities were found in 25 per cent of cases, the incidence of coronary artery involvement (dilatation with or without stenosis) being 12.5 per cent. High dose intravenous immunoglobulin (2 g/kg over a 12-h period) plus aspirin therapy was found to be safe and effective in patients with Kawasaki disease.     

Paget's disease of the oesophagus associated with mucous gland carcinoma of the lower oesophagus

AIM: To report a rare case of oesophageal Paget's disease and its rarer combination with submucosal gland carcinoma of the lower oesophagus. METHODS AND RESULTS: A 74-year-old Saudi female was admitted with the complaint of dysphagia. Endoscopic examination showed an ulcerated tumour at the gastro-oesophageal junction. Initial biopsy showed an undifferentiated carcinoma with pagetoid spread in the oesophageal stratified squamous epithelium. Oesophago-gastrectomy specimen showed a lobulated, poorly differentiated mucous gland carcinoma at the gastro-oesophageal junction. The tumour showed focal acinar differentiation and obvious cancerization of the submucosal glands, somewhat similar to the breast lobular carcinoma in situ. One of the isolated and cancerized submucosal glands also showed carcinoma in situ of its duct. Oesophageal surface epithelium showed extensive pagetoid spread, both over and away from the main tumour. The pagetoid tumour cells showed selective positivity for cytokeratin 7, cytokeratin Cam 5.2, BerEP4 and to a lesser extent for CEA. CONCLUSIONS: As far as we know, this is the fifth case report of oesophageal Paget's disease and the first report of its association with the underlying mucous gland carcinoma.     

Presence and significance of somatic cell apoptosis markers in human ejaculated spermatozoa

Ejaculated spermatozoa, particularly in infertile men, have been shown to display morphological and biochemical features that are typical of an apoptotic phenotype in somatic cells. Deregulation of apoptosis is known to play roles in a number of disease processes, but roles for apoptosis in ejaculated spermatozoa and male infertility are poorly defined or have not been studied. Preliminary data demonstrated that populations of ejaculated spermatozoa express: (i) various degrees of plasma membrane translocation of phosphatidylserine and DNA fragmentation; and (ii) active caspase-3, the main executor of apoptosis in somatic cells, with an apparent exclusive cellular location to the mid-piece. Tests are currently being carried out on the effects of well-known apoptosis agonists and caspase inhibitors on such markers using purified populations of leukocyte-free ejaculated human spermatozoa. The main objective is to determine if somatic cell apoptosis markers are relevant indicators and/or causative factors of male infertility.     

Current knowledge of risks and benefits of prophylactic oophorectomy at hysterectomy for benign disease in United Kingdom and Republic of Ireland

OBJECTIVE: To estimate current practice of prophylactic oophorectomy at hysterectomy for benign disease and also define the role of the variables considered prior to making the decision of prophylactic oophorectomy among consultant obstetricians and gynaecologists in UK and Republic of Ireland. DESIGN: A postal questionnaire was sent to all 1536 practising consultant gynaecologists in UK and Republic of Ireland. METHODS AND MAIN OUTCOMES MEASURED: Of the 809 replies received, 21% of the respondents routinely performed prophylactic oophorectomy. There was a wide regional variation; 40% consultants in Wales considered prophylactic oophorectomy compared to 16% in the Northern and Yorkshire regions. Consultants with a special interest with a more surgical bias were more likely to consider performing prophylactic oophorectomy. The majority of respondents estimated that prophylactic oophorectomy would reduce risk of ovarian cancer by up to 5%, and that following hysterectomy and ovarian conservation there was an increased risk of premature menopause. CONCLUSION: There is a large variation and uncertainty in the practice of prophylactic oophorectomy in UK and Ireland.     

Plexiform fibrohistiocytic tumor: a rare low-grade malignancy of children and young adults

Plexiform fibrohistiocytic tumor (PFT) is a low-grade, superficial, soft tissue neoplasm with a limited but significant ability to metastasize. This type of tumor only rarely presents in the skin of the head and neck. Clinicians first encountering young patients with facial neoplasia, such as a PFT, might be unaware of its exact oncologic potential and instead be primarily concerned with the cosmetic outcome. We treated a 17-year-old boy with a PFT on his cheek who was initially treated only by shave biopsy. The tumor subsequently recurred and metastasized to the cervical lymph nodes 3 years after the initial biopsy. Therefore, appropriate initial therapy for PFT requires complete excision with negative resection margins.     

Epidemiology and outcome of “Early-onset” vs “Late-onset” necrotizing enterocolitis

Objective: We hypothesized that among neonates with necrotizing entercolitis (NEC), important epidemiologic and outcome differences exist between those with early-onset vs. those with late-onset NEC.Methods : We reviewed all records of neonates cared for in the King Fahad University Hospital during the past ten years who had the diagnosis of NEC. We separated cases into two groups depending on age at diagnosis. Specifically, we termed “early-onset” those cases diagnosed during the first seven days of life, and “late-onset” those diagnosed thereafter. We compared, in the two groups, gestational age, clinical signs at onset, laboratory data, surgical findings, complications, and mortality.Results: From 1989 to 1999, 37 cases of stage lla (or higher) NEC were diagnosed; 25 “early-onset “ and 12 “late-onset”. Neonates with “earlyonset” NEC were more mature (35.4 ±2.5 weeks gestation) than those with “late-onset” (27.7 ±2.8 weeks, P = 0.0001), were more likely to have feedings begun in the first 48 hours of life (P = 0.0002), and more likely to have feeding increments of >25 ml/kg/day (P=0.03). Neonates with “late-onset” NEC were more likely to present with vomiting (P=0.003) and apnea (P=0.001), and were more likely to have ileal rather than colonic necrotic lesions, short bowel syndrome, and mortality (P = 0.03).Conclusion: During the past 10 years at the King Fahad University Hospital, cases of early and late-onset NEC have had distinct epidemiologic and outcome features. Recognizing these differences may be useful in prognostication and counseling