The adrenocorticotrophic hormone (4-9) analog ORG 2766 benefits autistic children: report on a second controlled clinical trial.

In a second controlled crossover trial, 20 autistic children received 40 mg/day of the neuropeptide ORG 2766, a synthetic analog of ACTH (4-9), for 8 weeks. Parents' checklist ratings (ABC) as well as clinicians' ratings (CGI) pointed to significant improvements after the course of treatment; improvements were clearest on the ABC social withdrawal subscale. The analysis of individual target symptoms and the parents' treatment preferences substantiated the beneficial effects of ORG 2766. In an ethologically analyzed playroom session, ORG 2766 treatment was associated with an improvement in the children's play behavior and a significant increase in the social interaction between child and experimenter. Gaze coordination between child and experimenter also was improved.     

Acute intermittent porphyria and primary liver-cell carcinoma.

A patient known with acute intermittent porphyria who developed primary liver-cell carcinoma is described. No other risk factors were found. A possible association of acute intermittent porphyria with the development of primary liver-cell carcinoma has been suggested in recent, mainly Scandinavian literature. So far this association has never been described in The Netherlands.     

Epithelioid sarcoma in a dog.

A tumour in an 11-year-old male crossbred Fox Terrier, showing the clinical and pathological features of epithelioid sarcoma in man is reported. Clinical aspects, location, peculiar pattern of spread and evolution, together with histopathological and immunohistological findings of this rare tumour simulating a granuloma are described. Controversial histogenesis is discussed.     

Acute fatal myeloencephalopathy after combined intrathecal chemotherapy in a child with acute lymphoblastic leukemia

We report a case of a fatal toxic encephalomyelopathy in a 12-year-old girl due to prophylactic intrathecal injection of methotrexate and cytosine arabinoside, with a characteristic progressive symptomatology leading to death after 28 days. The location and type of neuropathological changes support the hypothesis of a direct toxic effect of methotrexate and/or cytosine arabinoside on structures directly exposed to the cerebrospinal fluid.     

Intraoperative radiotherapy in the multidisciplinary treatment of bone sarcomas in children and adolescents

From September 1984 to December 1989, 38 patients of pediatric age with localized bone sarcomas received intraoperative radiotherapy (IORT) as part of a multidisci plinary treatment program. The age ranged from 6 to 21 years. The tumor histologies were 22 osteosarcomas and 16 Ewing's sarcomas. Thirty-four had initial primary disease (90%) and 4 were treated for local recurrence (10%). IORT was used on 32 untreated patients and in 6 previously treated with external beam radiotherapy (EBR). The IORT field included the surgically exposed tumor bed area. Single radiation doses ranging from 10 to 20 Gy were delivered, using 6–20 MeV electron beams. The median follow-up time for the entire group is 25 months (2–65+ months). The projected 5-year disease-free and overall survival rates are 65% and 69%, respectively. One patient developed a local recurrence in each histological group: one chondroblastic osteosarcoma and one cervical Ewing's sarcoma. Six patients died from metastatic progression: 3 initially recurrent tumors and three primary disease cases. Severe neuropathy and soft tissue necrosis were seen in some patients as IORT related complications. IORT is a feasible technique to be integrated in multidisciplinary programs that may promote local control in pediatric and adolescent patients with bone sarcomas. Peripheral nerves are dose-limiting tissue structures for IORT.     

Experimental and numerical models of acute intracranial hypertension and basilar artery blood flow velocity.

A numerical model based on Navier-Stokes equations was used in conjunction with an experimental model in rabbits to study the effects of acute intracranial hypertension on basilar artery blood flow velocity. The hypertension was induced by pressure transmission via an epidural pressure sensor inserted into a parietal intracranial opening. A critical value of half of the diastolic arterial pressure for the intracranial cerebral pressure was determined by both numerical and experimental models. At this intracranial cerebral pressure level, the total input resistance and total input compliance, determined by the numerical model, exhibited an increase of 27% and 10%, respectively, and the tissular compliance a decrease of 25% from their physiologic baseline values. When the intracranial cerebral pressure reaches the level of the diastolic arterial pressure, a zero diastolic flow is observed into the cerebral vascular system. This study validates the theoretical model, which could be used in assessing intracranial cerebral pressure noninvasively in humans when O2 pressure can be stabilized.     

The spectrum of opportunistic filamentous fungi present in the CBS culture collection

The mould collection of the Centraalbureau voor Schimmelcultures, Baarn, The Netherlands, was screened for isolates originating from warm-blooded animals. The range of species indicates that distribution of clinically relevant, pathogenic or opportunistic strains over the fungal kingdom is non-random. Some opportunistic fungi possess adaptations to life under hostile environmental conditions, enabling them to survive inside the human body. Presence of melanin or carotene seems to be an important virulence factor. Opportunistic fungi which sporulate in submersion are able to disseminate or cause severe local mycoses when the aspecific immune system of the host is impaired. Mycoses caused by a few dimorphic fungi, mostly in their natural ecological niche living in association with vertebrates, are promoted by specific immune deficiencies.