A community survey of children with severe intellectual disability and their families: psychological adjustment, carer distress and the effect of respite care.

The present paper describes a cross-sectional study of the psychosocial adjustment of 143 children with severe disability and their families identified from a regional case register for children with special needs. Thirty-eight per cent of the children had significant psychiatric morbidity. By contrast, the overall level of distress in carers was not that different from probable community prevalence figures. Nevertheless, distress among carers was consistently associated with increased disability in the child. Although respite care is a valuable resource for many carers, its use is indicative of underlying distress in the carer.

MRI of a silicone breast implant surrounded by an enlarging hemorrhagic collection.

MRI yields cross-sectional image acquisition in multiple planes with very high contrast resolution. Additionally, in MRI of the breast, evaluation of the entire implant is possible. Not only is MRI very sensitive to loss of implant integrity and rupture, but also MRI can evaluate and characterize adjacent fluid collections or soft-tissue masses. Anatomic relationships of abnormalities and normal structures are well seen. In some situations, MRI may offer clinically relevant information not adequately addressed by other imaging modalities.

A prospective study of early soft tissue coverage of grade IIIB tibial fractures.

Between 1987 and 1990, 45 consecutive patients with grade III tibial injuries were treated by an established protocol. There were 31 men and 14 women. The average age was 27 years (range, 17-68 years). The average follow-up was 16 months (range, 12-46 months). Early bony fixation consisted of an external fixator in 28 patients and a non-reamed intramedullary nail in 17 patients. No significant difference in complications was noted between the two types of fixation systems. Forty-three percent of the patients underwent early bone grafting. Free muscle flaps were employed in 78% of patients with a 97% success rate. Local muscle flaps were utilized in 22% of patients with an 84% success rate. Local infection occurred in three patients (6%). Osteomyelitis occurred in two patients (4%). Bony union was present in 98% of patients (44 of 45). Limb salvage was 98% (44 of 45). Early bone grafting (< or = 3 months) yielded earlier bony union (average, 40 weeks) than late bone grafting (average, 52 weeks). This study proves the efficacy of an established protocol of early muscle flap coverage in the management of grade IIIB tibial fractures in a consecutive series of patients. Early bone grafting appears to be beneficial to early bony union. The intramedullary rod fixation system offers an acceptable alternative to the external fixator system in severe acute open tibial fractures.

Case report: gastric angiolipoma with chronic hemorrhage and severe anemia.

Lipomas are benign tumors that are common in other sites but rare in the stomach. They are usually submucosal and, when symptomatic, are most often accompanied by gastrointestinal hemorrhage. Angiolipoma is a not uncommon benign lipomatous neoplasm with a characteristic vascular component that almost exclusively occurs in the subcutaneous tissue. A case of gastrointestinal angiolipoma, which appears to be only the second one reported, is discussed. The patient, a 69-year-old man, presented with signs and symptoms of chronic hemorrhage and severe anemia (hemoglobin 6, hematocrit 19.9). The patient's condition was found to have resulted from ulceration of the gastric mucosa overlying a submucosal angiolipoma, and the anemia resolved after local surgical resection of the tumor. Electron microscopic studies supported the light microscopic diagnosis of angiolipoma. The absence of fibrin thrombi in this and other nonsubcutaneous angiolipomas and the possible significance of the vascularity of these tumors is discussed. The literature regarding gastric lipoma and angiolipoma is reviewed.

The modification and standardisation of the Harter self-esteem questionnaire with Scottish school children.

This paper describes a study to modify an American questionnaire measuring self-esteem in children for use in the United Kingdom and also to obtain normative values for this questionnaire with Scottish school children aged 8-15 years. Five thousand children resident in the Lothian Region of Scotland, a 5% sample of the age group, were identified using a cluster sampling technique in order to provide a random sample of the general population for this age group. The main findings were as follows: children usually rated themselves higher than the midpoint on most subscales, indicating that they have a positive regard for themselves; boys tended to rate themselves higher than girls on most subscales except behaviour; scores tended to decline as children get older, especially for girls; global or overall self-esteem was highly correlated with the other subscales, especially physical appearance or attractiveness; self-esteem scores were not influenced by social class, school or religion. The modified Harter questionnaire can be used to measure self-esteem in several situations. These include comparisons between different groups of children, changes in self-esteem following treatment interventions or the effects of illness on children's psychological adjustment. Finally, the study has provided normative values for a Scottish population of school children aged 8-15 years.

Helping parents and children with epilepsy cope successfully: the outcome of a group programme for parents.

This paper describes a study to determine the effectiveness of a group programme for parents designed to reduce the psychosocial morbidity among children with epilepsy and their families. The participants were the parents of 108 children attending the seizure clinic at a children's hospital. The first part of the project had shown that half of the children and their families had considerable morbidity. Despite the high rate of psychosocial morbidity among these children and their families, only 35% of the parents expressed an interest in participation in the intervention programme, with only 12% attending any meetings. The latter were mainly the small number of parents whose children had severe intractable epilepsy as well as educational and family problems. Many parents did, however, request more information about epilepsy and for the provision of a counselling service. The latter is likely to be most beneficial in the months following diagnosis.

Boron neutron capture therapy for murine malignant gliomas.

Boron neutron capture therapy (BNCT) involves administration of a boron compound followed by neutron irradiation of the target organ. The boron atom captures a neutron, which results in the release of densely ionizing helium and lithium ions that are highly damaging and usually lethal to cells within their combined track length of approximately 12 microns. Prior to Phase I clinical trials for patients with malignant gliomas, mice with glioma 261 intracerebral tumors were fed D,L-3-(p-boronophenyl)alanine and irradiated with total tumor doses of 1000-5000 RBE-cGy of single fraction thermal neutrons to determine the maximum tolerated dose and effect on survival. These mice were compared to mice that received D,L-3-(p-boronophenyl)alanine alone, neutron irradiation alone, photon irradiation alone, or no treatment. Additional normal mice received escalating doses of neutron irradiation to determine its toxicity to normal brain. BNCT caused a dose-dependent, statistically significant prolongation in survival at 1000-5000 RBE-cGy. At 3000 RBE-cGy, median survival rates of the BNCT and untreated control groups were 68 and 22 days, respectively, with a long-term survival rate of 33%. At 4000 RBE-cGy, median survival was 72 and 21 days, respectively, with a long-term survival rate of 43%. At lower radiation doses, the extended survival was comparable between the BNCT and photon-irradiated mice; however, at 3000 and 4000 RBE-cGy the median survival of BNCT-treated mice was significantly greater than photon-irradiated mice. The maximum tolerated single fraction dose to normal brain was approximately 2000 RBE-cGy.

Giant cystosarcoma phyllodes of the prostate associated with adenocarcinoma.

Cystosarcoma phyllodes of the prostate is a rare neoplasm, occurring in adult men. It closely resembles the not uncommon tumor of the female breast and usually behaves in a similar manner. This case of benign cystosarcoma phyllodes of the prostate occurred in a 53-year-old man who presented with increasing abdominal girth and underwent exploratory laparotomy and removal of the 11.2-kg tumor. It was remarkable for its very large size and the presence of foci of well-differentiated adenocarcinoma, prostatic acinar type. The glandular epithelium of both the phyllodes tumor and the carcinoma were immunoreactive for cytokeratin, epithelial membrane antigen, prostate-specific antigen, and prostate-specific acid phosphatase. The presence of typical prostatic type adenocarcinoma and this immunoreactivity pattern strongly supports a prostatic origin for this rare neoplasm.

Multifocal malignant melanoma arising in vesicovaginal melanosis.

An 80-year-old white woman developed vulvar melanosis and malignant melanoma of the labia majora and clitoris and underwent a simple vulvectomy. She subsequently developed melanosis of the urinary bladder and presented 3 years later with multifocal malignant melanoma involving the vagina, urethra, and urinary bladder in a background of extensive melanosis with variable degrees of atypia. She underwent radical surgery but died 18 months later with liver metastases and liver failure. Malignant melanoma is uncommon in the vulva and vagina and is rare in the urinary bladder. This case illustrates the previously described association between melanosis and malignant melanoma. The unusual features are the widespread distribution of the melanosis (vagina and bladder) and the subsequent development of multifocal malignant melanoma.

Metastatic basal cell carcinoma: report of a case presenting with respiratory failure.

Metastasis from basal cell carcinoma (BCC) of skin is rare. A case of a 58-year-old male presenting with increasing shortness of breath and right pleural effusion is described. Open right pleural biopsy demonstrated metastatic carcinoma consistent with BCC. Review of medical records from another hospital revealed that four years previously a recurrent BCC had been excised from the left back. One and three years prior to this excision, a skin lesion at this same site had been treated with electrocautery. Review of the skin excision slides demonstrated infiltrating BCC histologically very similar to the metastatic pleural neoplasm. The patient died two months after the pleural biopsy. At autopsy, the cutaneous BCC had not recurred and metastatic BCC extensively infiltrated the pleura bilaterally, with focal involvement of underlying lung parenchyma, subcarinal lymph nodes, diaphragm, and pericardium.

Human papillomavirus and carcinoma of the urinary bladder.

The human papillomaviruses have been strongly associated with anogenital cancers. Sporadic reports have linked papillomavirus infection to bladder neoplasms. We analyzed 27 normal and malignant bladder tissue samples for the presence of human papillomavirus by in situ DNA hybridization and the polymerase chain reaction. Only one invasive, keratinizing squamous cell carcinoma was found to contain human papillomavirus DNA. This occurred in a 61-yr-old female who was immunocompetent and had no previous evidence of papillomavirus-associated disease. This case represents the first invasive carcinoma of the bladder associated with papillomavirus infection.

Psychosocial adjustment of children with chronic epilepsy and their families.

A cross-sectional study was undertaken of psychosocial morbidity among 108 children with chronic epilepsy and their families. Approximately half the children showed evidence of significant psychological disturbance. Neurotic or emotional disturbance was the most common diagnostic category, with similar rates of disturbance among boys and girls; findings not reported previously. Behavioural disturbance was strongly associated with epileptic variables, family factors, individual characteristics of the child and with maternal anxiety about epilepsy. Several factors are responsible for this increase, and further study is needed to elucidate these and to reduce the incidence of psychosocial morbidity among these children.

Chronic cavitary respiratory papillomatosis.

A 19-year-old white man with multiple recurrences of respiratory papillomatosis was admitted for recurrent left lower lobe pneumonia and lung abscesses. He was found to have a single large laryngeal papilloma, widespread bronchial papillomatosis, and large cavitary lesions of the left lower lobe. A lobectomy was performed. The smooth-walled, squamous-lined cavities contained large numbers of papillomas, which were strongly positive for human papillomavirus type 11 by in situ DNA hybridization. Findings of evaluation of the patient's humoral and cell-mediated immunity were within normal limits. Cavitation appears to have resulted from bronchial obstruction, postobstructive pneumonia, and liquefactive necrosis. We speculate that squamous metaplasia allowed the continued proliferation of papillomavirus within the cavities.

Auditory function in newborn intensive care unit patients revealed by auditory brain-stem potentials.

The relations between clinical illness and auditory response (as revealed by auditory brain-stem potentials) were prospectively studied in a neonatal intensive care unit. Forty-nine tests were performed on 29 infants with gestational age 24 to 43 weeks and birth weight 530 to 3,380 gm. Auditory test results were classified as pass or fail, depending on the presence or absence of wave V at a latency of 7 to 11 ms in response to clicks 60 dB above the normal adult threshold. Six patients failed and 23 patients passed. The failures were not correlated with excessive noise exposure or ototoxic medication. Five of the patients who failed had intracranial hemorrhage. Routine screening of infants in the NICU for auditory impairment is a clinically feasible and useful procedure.