PubMed Health⌕ Search

Biomedical subjects

A C Moll

Publications and source records attributed to A C Moll.

31 records · Page 2Linked to original sources

Incidence and survival of retinoblastoma in The Netherlands: a register based study 1862-1995.

AIM: The aim of this study was to determine the (time trends in) incidence and survival of hereditary (familial and sporadic) and non-hereditary retinoblastoma for male and female patients born in the Netherlands between 1862 and 1995. METHOD: The national retinoblastoma register was updated and now consists of 955 patients. The missing dates of death were obtained from the municipal registers and the Central Bureau of Genealogy in The Hague. Mortality was compared with the Dutch vital statistics. RESULTS: From 1862 to 1995 no significant differences in incidence for retinoblastoma were found in the hereditary subgroups. Further, no significant differences between males and females were found, both overall and in the hereditary subgroups. The average incidence of retinoblastoma increased until 1944, probably due to incompleteness of the register, and stabilised after 1945 (1 per 17000 live births). From 1900 to 1995 the standardised mortality ratio increased for hereditary retinoblastoma patients from 2.9 to 9.0 and decreased for non-hereditary retinoblastoma patients from 1.9 to 1.0. CONCLUSION: Although survival for retinoblastoma was significantly better after 1945 than before, in comparison with the Dutch population the mortality between 1900 and 1990 increased for the hereditary and decreased for the non-hereditary retinoblastoma patients.

Eye Neoplasms↗

Second primary tumors in patients with retinoblastoma. A review of the literature.

PURPOSE: The aim of this survey was to review the different studies regarding the occurrence of second primary tumours (SPT) among survivors of retinoblastoma. METHODS: Ovid (Medline, Current contents life, Psychlit, Embase) was searched for the years 1966-1995 using the mesh headings: 'retinoblastoma', 'second primary neoplasms', and 'multiple primary neoplasms'. The inclusion criteria were: the study should involve 50 patients or more and should not be limited to one specific SPT. A checklist with criteria regarding the study design and the results was applied to each study. RESULTS: Eleven studies were identified which met the inclusion criteria. Thirty-five different types of SPT (Ntotal = 243) were reported. Most of them were osteosarcomas (37.0%), followed by melanomas (7.4%), soft-tissue sarcomas (6.9%), brain tumors (4.5%), fibrosarcomas (3.3%), chondrosarcomas (3.3%), and sarcomas (3.3%). Less frequently reported were leukemias (2:4%), sebaceous cell carcinomas (1.6%), and non-Hodgkin lymphomas (1.6%). Pineoblastoma, which in fact is a trilateral retinoblastoma and not an SPT, was found in 2.4%. Despite the differences, all 11 studies showed a higher incidence of SPT compared to the general population. Only 4 studies were judged to be free from selection bias, reporting a cumulative incidence of SPT of 8.4% 18 years after diagnosis, 15.7% at the age of 20 years, 19% at the age of 35 years, and a relative risk of 15.4 for SPT, respectively. CONCLUSION: SPT is a serious problem for the survivors of hereditary retinoblastoma and its importance should be recognized in (genetic) counseling of patients.

Adolescent↗

Second primary tumors in patients with hereditary retinoblastoma: a register-based follow-up study, 1945-1994.

The aim of this register-based follow-up study was to evaluate the long-term cumulative incidence of second primary tumors (SPT) among survivors of hereditary retinoblastoma, with special interest for the incidence of pineoblastoma in retinoblastoma patients born after 1970. The Dutch Retinoblastoma Register was completed and updated: in the period 1945-1994, 639 retinoblastoma patients were registered. The vital status of each patient was obtained from the municipal registries and the Central Office of Genealogy. SPT were traced and histopathologically confirmed. Survival curves and cumulative incidence of SPT were calculated by the Kaplan-Meier method. The survival of patients with hereditary retinoblastoma was significantly shorter than that of patients with non-hereditary retinoblastoma. The cumulative incidence of SPT in hereditary patients was 3.7 and 17.7% at the ages of 10 and 35 years, respectively. Long-term follow-up revealed a high proportion of melanomas (7 melanomas out of 28 SPT). In the sub-cohort of the hereditary-retinoblastoma patient group born after 1970, the cumulative incidence of pineoblastomas at the age of 5 years was 9.3%. Our results suggest that patients with hereditary retinoblastoma should have careful follow-up, and procedures for diagnosing SPT and pineoblastomas at an early and potentially treatable stage should be developed.

Adolescent↗

High parental age is associated with sporadic hereditary retinoblastoma: the Dutch retinoblastoma register 1862-1994.

We wished to determine the influence of parental age at the birth of a retinoblastoma patient on the risk of sporadic hereditary retinoblastoma. The parental age at birth of 941 patients of the Dutch retinoblastoma register (1862-1994) was identified and compared between sporadic hereditary and nonhereditary patients. In a subcohort (1936-1994), a comparison was made with parental age at birth in the general population, as obtained from the Central Bureau of Statistics. Missing birth dates of the parents of retinoblastoma patients were traced with the help of the municipal registries and the Central Bureau of Genealogy. The mean paternal age was 10.7 months higher and the mean maternal age was 11.0 months higher in the sporadic hereditary retinoblastoma patients than in parents of nonhereditary patients. In the subcohort, the mean paternal and maternal ages of sporadic hereditary patients were also higher (12.4 and 11.5 months, respectively) than those of the general population. All differences were statistically significant. This study shows that a high parental age is associated with an enhanced risk of sporadic hereditary retinoblastoma.

Adult↗

Quantification of orbital and mid-facial growth retardation after megavoltage external beam irradiation in children with retinoblastoma.

PURPOSE: The late side effects of external beam irradiation in patients with retinoblastoma such as orbital bony growth retardation, are a serious problem in adolescence. Therefore, a quantitative study was performed to investigate the late effects of irradiation on orbital growth in patients with retinoblastoma. METHODS: The orbits of 68 patients with retinoblastoma, 52 bilateral and 16 unilateral, were divided into two treatment groups: radiotherapy alone, 77 orbits; and radiotherapy + enucleation, 43 orbits. Follow-up time was 12 to 240 months (mean, 95 months) in group 1 and 27 to 216 months (mean, 97 months) in group 2. These groups were subdivided further into age groups at which radiotherapy was given. The morphometric measurements of these groups were compared. RESULTS: The authors showed that irradiation causes a significant growth retardation when compared with the growth of nonirradiated orbits (P<0.001). They also demonstrated that radiotherapy in children younger than 6 months of age is more damaging to the orbital growth than at an older age (P<0.01). Finally, the authors showed that secondary enucleation does not have an additive growth-retarding effect. CONCLUSION: Orbital growth retardation in patients with retinoblastoma after radiotherapy is influenced mainly by the age at which irradiation is given and is defined at 6 months. Theoretically, it would be desirable to postpone irradiation in children until they are older than 6 months of age if possible. The irradiation effect on these orbits is not enhanced by enucleation.

Bone Development↗

Three histopathological types of retinoblastoma and their relation to heredity and age of enucleation.

The histopathology of 61 eyes was studied with special attention to the morphology of the retina adjacent to the main tumour. Three retinal types were distinguished. Retina type 1 (RT-1, 28 specimens) contained a single tumour that was sharply demarcated from surrounding normal retina. In retina type 2 (RT-2, 29 specimens) large parts of the retina were affected and the main tumour mass gradually blended with the adjacent pathological retina. Retina type 3 (RT-3, four specimens) was characterised by a retina almost entirely affected by diffuse tumour growth. RT-1 correlated significantly with early enucleation (0-3 years) both in hereditary and non-hereditary cases. RT-2 was seen in eyes enucleated later (2-5 years). The progressing tumour may release growth factors in the intraocular space that stimulate the cells of the adjacent retina and lead to multiple new primary tumours in the adjacent retinal area. RT-3 was only present in non-hereditary cases with late enucleation (at 2-5 years). Hereditary retinoblastoma cases are usually detected early. Therefore in hereditary cases RT-1 is significantly more common than RT-2. In 25 eyes of the 44 patients with unilateral sporadic retinoblastoma, multifocal tumours of the retina were observed. Such cases should not mistakenly be classified as hereditary cases on the basis of the histological pattern of multifocality of the tumour process.

Age Factors↗

A giant congenital orbital tumor: an unusual presentation of retinoblastoma.

We report a case of an unusual giant congenital tumor presenting in a newborn infant as a large exophytic mass emerging from the left orbit. After enucleation orbital recurrence developed within 14 days. No anti-tumor treatment was given and the child died at the age of 4 weeks. The histopathological and cytogenetical analysis of the tumor is presented. The tumor was diagnosed as a retinoblastoma but we could not exclude the possibility of a neuroblastoma.

Eye Neoplasms↗

Prevalence of blindness and low vision of people over 30 years in the Wenchi district, Ghana, in relation to eye care programmes.

A population based survey on the prevalence of major blinding disorders was conducted in the Wenchi district in central Ghana between March and May 1991. In 10 villages, 1425 people of 30 years and older were screened, using the WHO eye examination record. The prevalence of bilateral blindness above 30 years proved to be 1.7% (best acuity < 3/60): the prevalence of low vision above 30 years was 2.0% (best visual acuity 6/18 to 3/60). The causes of blindness were determined as cataract (62.5%), onchocerciasis (12.5%), corneal opacity (non-trachomatous) (8.2%), refraction anomalies (4.2%), phthisis bulbi (4.2%), optic atrophy (4.2%), and vascular retinopathy (4.2%). In the Wenchi district, 1.0% of the population over the age of 30 years was found to need a cataract extraction because of blindness of both eyes. Another potential 1.0% needs a cataract extraction because of low vision. A minor second study (n = 149) was undertaken in the same district, but in a village in an area near the Black Volta river in which onchocerciasis is endemic. The prevalence of blindness (8.1%) and low vision (3.4%) caused by onchocerciasis and cataract both proved to be higher. The survey provided the basis for a preventive and curative eye care programme.

Adult↗

Effect of donor cornea preservation in tissue culture and in McCarey-Kaufmann medium on corneal graft rejection and visual acuity.

In a retrospective study the rate of rejection and clouding of the donor cornea for other reasons was investigated in 230 penetrating keratoplasties performed between 1984-1986. Donor corneas were stored in McCarey-Kaufmann Medium (MK) at 4 degrees C or in a modified Minimal Essential Medium (MEM) at 31 degrees C. No statistical differences in rejection rate, cloudiness due to other causes or visual acuity was found between MEM- and MK-stored donor corneas.

Adult↗

Cognitive function after spinal or general anesthesia for transurethral prostatectomy in elderly men.

Cognitive functions in 53 elderly men who underwent a transurethral prostatectomy were assessed pre-operatively and 4 days and 3 months post-operatively. Thirteen patients had a preference for one particular type of anesthesia, and the remaining 40 were randomly allocated to receive either spinal or general anesthesia. Cognitive function was not different between the groups receiving different types of anesthesia at either time point and did not decrease post-operatively. No pre- or perioperative variable could distinguish the subgroup of patients who had a post-operative decrease of 2 points or more on the Mini-Mental State Examination. No difference in post-operative performance was found in the patient groups with pre-operative Mini-Mental State Examination scores above or under their age-specific norm. It is concluded that neither hospitalization nor the two forms of anesthesia investigated cause a decrease in cognitive function in elderly men.

Aged↗

Age at onset in Huntington's disease: effect of line of inheritance and patient's sex.

The Leiden Roster for Huntington's disease (HD) contained data on 2617 cases up to July 1988. The age at onset (AO) was known in 1084 cases and in 1020 of these both their AO and the sex of the affected parent was known. The mean AO was higher for females than for males and higher for maternal than for paternal cases. However, in the group born before 1925 only females with maternal inheritance had a higher mean AO. Data on influence of sex and line of inheritance were present for the grandparents as well as for the great grandparents. Influence of the line of inheritance from the grandparents was particularly present for the grandmother-father (MP) lineage; regarding the great grandparents a significant difference was found between the MPM and PMP lineage. The results obtained for juvenile HD cases were comparable to those previously published. In late onset cases (over 50 years) no maternal preponderance in inheritance was found.

Adult↗

Anticoagulant therapy and cataract surgery.

A questionnaire was sent round to the 200 members of the Netherlands Intraocular Implant Club (NIOIC), in which they were asked about the policy followed in 1988 with regard to anticoagulant therapy (ACT) and the use of aspirin before and after cataract surgery. Ninety-nine formulas were returned, of which ninety-two were suitable for analysis. It appeared that 62% of the eye surgeons stopped ACT, whereas only 33% stopped the use of aspirin. After stopping ACT nine, in some cases serious, systemic complications arose. The continuation of ACT led to 3 ocular complications. It would appear that continuation of ACT and the use of aspirin is to be recommended.

Anticoagulants↗