An unusual malformation of the forebrain and concomitant destructive encephalopathy with calcifications.

A 9-year old boy with profound mental retardation and severe neurologic deficit presented an unusual malformation of the forebrain distinguished by the following features: 1) microtelencephalon, alobulation, afissuration, and abnormal convolutional pattern; 2) persistence of hippocampal formation at its embryonic site in the dorsomedial wall of the telencephalon; 3) hypoplasia and abnormal configuration of ventricles; 4) agenesis of cerebral commissures; and 5) abnormal location and orientation of gray structures. Important concomitant findings characterized by severe destructive lesions, massive calcification, granular ependymitis, and low grade inflammation were suggestive of late sequelae of an infectious process, possibly congenital. Transplacental transmission of an unidentified pathogen with teratogenic properties was hypothesized as the probable cause. The teratogenic insult started in an early embryonic period and affected primarily the development of the neopallium in the telencephalic wall. The arrested development of the neopallium disrupted the chain of interdependent developmental events; consequently characteristic morphological modifications normally induced by the continuous growth and differentiation of the neopallium failed to occur or took an abnormal course. The name architelencephalon (Greek: arche, beginning; telencephalon, cerebral hemispheres) proposed for this particular malformation of the forebrain indicates its resemblance both to the human brain at early stages of development and also to mammalian brains on a lower level of phylogeny. Associated anomalies included an unilateral microphthalmia with cataract, severe stenosis of the aqueduct of Sylvius and macrocephaly. The microphthalmia was either a developmental anomaly or was infectious in origin. The stenosis of the aqueduct was attributed to granular ependymitis. Tearing of the thin dorsal diencephalic plate and arachnoid membrane and escape of the cerebrospinal fluid into the subdural space probably accounted for the macrocephaly.

Long survival of primary cerebral lymphoma with progressive radiation necrosis.

A 56-year-old man lived 8 years after excision and irradiation of a primary cerebral lymphoma. Delayed radiation necrosis caused progressive neurologic deterioration and probably his steroid-responsive episodes of obtundation. Vasogenic edema induced by radiation may account for the latter. An incidental extraneural lymphoma was found postmortem without evidence of CNS lymphoma.

Basilar artery occlusion in an adolescent girl: a risk of oral contraceptives?

An 18-year-old adolescent girl died of an extensive pontine infarction. At postmortem examination she was found to have a thrombosis of the basilar and intracranial vertebral arteries with associated focal hyperplasia of the intima. Her ovaries were grossly small and inactive, with absent corpora lutea. Based on these findings, we suspect she was taking oral contraceptives. In cases of unexplained fatal strokes in young females, careful pathologic examination of the ovaries may provide etiological clues.

Referral patterns, lesion prevalence, and patient care parameters in a clinical oral pathology practice.

OBJECTIVE: The purpose of this study was to determine the following parameters in a referral-based private practice oral and maxillofacial pathology clinic: (1) sources of clinical referrals; (2) types of problems referred; and (3) clinical effectiveness of treatment. STUDY DESIGN: Clinical charts were reviewed for a cohort of 362 patients seen over a 2 1/2-year period (1993-1995). From these charts, we determined the source of referral and the final diagnosis for each patient. In addition, 50 patients were randomly selected and surveyed by telephone; each was asked a series of questions to determine the following: (1) the number of health care practitioners previously seen with regard to the patient's condition; (2) the length of time that the condition had been present before the patient came to the oral and maxillofacial pathology clinic; (3) the costs associated with medications and office visits that had been incurred before the patient came to the oral and maxillofacial pathology clinic; (4) the costs associated with medications and office visits that were incurred at the oral and maxillofacial pathology clinic; and (5) the patient's level of satisfaction with the oral and maxillofacial pathology clinic. RESULTS: Fifty-five percent of the referrals came from dentists, and 45% came from physicians. The 3 problems most commonly seen were candidiasis (12%), burning mouth syndrome (10%), and lichen planus (8%). For the 50 patients who were interviewed, the mean number of health care practitioners seen previously was 2.2 (range, 1-9). The mean time from initial symptoms to evaluation by an oral pathologist was 15 months. The mean approximate cost of medications and office visits before evaluation by an oral pathologist was $350 (range, $30-$4,000; median, $100); this compared with a cost of $94 (range, $50-$300; median, $70) for the patient visit and medications associated with the oral pathology appointment. The difference was statistically significant (P < or = .001). CONCLUSIONS: This preliminary study suggests that the clinical evaluation of oral lesions by an oral pathologist appears to be cost-effective and should be an integral part of a comprehensive health management system. These results should be corroborated by similar multicenter studies.

The biocompatibility of dense and porous Nickel-Titanium produced by selective laser melting.

Nickel-Titanium shape memory alloys (NiTi-SMA) are of biomedical interest due to their unusual range of pure elastic deformability and their elastic modulus, which is closer to that of bone than any other metallic or ceramic material. Newly developed porous NiTi, produced by Selective Laser Melting (SLM), is currently under investigation as a potential carrier material for human mesenchymal stem cells (hMSC). SLM enables the production of highly complex and tailor-made implants for patients on the basis of CT data. Such implants could be used for the reconstruction of the skull, face, or pelvis. hMSC are a promising cell type for regenerative medicine and tissue engineering due to their ability to support the regeneration of critical size bone defects. Loading porous SLM-NiTi implants with autologous hMSC may enhance bone growth and healing for critical bone defects. The purpose of this study was to assess whether porous SLM-NiTi is a suitable carrier for hMSC. Specimens of varying porosity and surface structure were fabricated via SLM. hMSC were cultured for 8 days on NiTi specimens, and cell viability was analyzed using two-color fluorescence staining. Viable cells were detected on all specimens after 8 days of cell culture. Cell morphology and surface topography were analyzed by scanning electron microscopy (SEM). Cell morphology and surface topology were dependent on the orientation of the specimens during SLM production. The Nickel ion release can be reduced significantly by aligned laser processing conditions. The presented results clearly attest that both dense SLM-NiTi and porous SLM-NiTi are suitable carriers for hMSC. Nevertheless, before carrying out in vivo studies, some work on optimization of the manufacturing process and post-processing is required.

Living at high altitude and risk of hospitalisation for atopic asthma in children: results from a large prospective birth-cohort study.

BACKGROUND: Asthma is among the most common chronic diseases in childhood and is steadily increasing in prevalence. Better characterisation of factors that determine the risk of hospitalisation for atopic asthma in childhood may help design prevention programmes and improve our understanding of disease pathobiology. This study will focus on the altitude of residence. METHODS: This is an ongoing prospective birth-cohort study that enrolled all live-born infants in the Tyrol. Between 1994 and 1999, baseline data were collected for 33 808 infants. From 2000 to 2005, all children hospitalised for atopic asthma at the age of > or =6 years (n = 305) were identified by a careful search of hospital databases. Disease status was ascertained from the typical medical history, a thorough examination and proof of atopy. RESULTS: Living at higher altitude was associated with an enhanced risk of hospitalisation for atopic asthma (multivariate RRs (95% confidence interval 2.08 (1.45 to 2.98) and 1.49 (1.05 to 2.11) for a comparison between altitude categories > or =1200 m and 900-1199 m versus <900 m; p<0.001). This finding applied equally to hospital admissions in spring, summer, autumn and winter. When altitude of residence was analysed as a continuous variable, the risk for asthma hospitalisation increased by 7% for each 100-m increase in altitude (p = 0.013). CONCLUSIONS: This large prospective study shows a significant association between the risk of hospitalisation for atopic asthma and altitude of residence between 450 and 1800 m. The underlying mechanisms remain to be elucidated, but it is tempting to speculate about a role for altitude characteristics such as the decline in outdoor temperature and air humidity and increase in ozone levels, which may trigger airway hyper-responsiveness and attenuate lung function.

Novel Galloylhamameloses from Hamamelis virginiana1.

The range of natural galloylhamameloses is extended by the recognition of the highly unstable novel analogues, the 1- O-(4-hydroxybenzoyl)-2',5-di- O-galloyl-alpha- D-hamamelofuranose and its 1- O-beta-anomer. The observed facile elimination of the C-1 acyl moiety is discussed and a mechanism proposed. These compounds are accompanied in the bark of HAMAMELIS VIRGINIANA by the "conventional" hamamelitannin, the related new 1,2',5-tri- O-galloyl-alpha- D-hamamelofuranose, and a tentatively characterized di- O-galloyl- D-hamamelopyranose, the first pyranose analogue, shown to be also present in commercially available hamamelitannin. The structures of these compounds were established from spectroscopic evidence of their acetate derivatives.

Effects of H2O2 on membrane potential and [Ca2+]i of cultured rat arterial smooth muscle cells.

The effect of 1 mmol/l H2O2 was studied on the membrane potential and [Ca2+]i with microelectrodes and the fura-2 technique, respectively. H2O2 induced a biphasic increase in [Ca2+]i with a fast transient peak and a subsequent plateau. H2O2 also led to a biphasic hyperpolarization of the cells with a similar time course. This was followed by a slight depolarization after wash-out of H2O2. External Ca2+ free solutions and treatment with the Ca2+ ionophore A23187 (1 mumol/l) abolished the effect of H2O2 on [Ca2+]i and almost entirely reduced the effect on the membrane potential. Phenylephrine (10 mumol/l) or A23187 also induced very similar biphasic hyperpolarizations of the membrane as H2O2 which were fully reversible after wash-out. It is concluded that H2O2 hyperpolarizes the membrane by opening of Ca2+ dependent K+ channels.

Fulminant leukemic polyradiculoneuropathy in a case of B-cell prolymphocytic leukemia. A clinicopathologic report.

A 52 year old man with a 10-month history of B-cell prolymphocytic leukemia (PLL) died of an apparent acute fulminant polyradiculoneuropathy, a condition generally attributed to paraneoplastic complication. The pathologic examination disclosed diffuse leukemic infiltrations of the peripheral nervous system. It is suggested that this particularly aggressive form of B-cell chronic prolymphocytic leukemia presented a constellation of features that promoted the invasion of the peripheral nervous system by way of the bloodstream and may explain the unusual clinical presentation.

[AgNOR analysis of astrocytoma in childhood]. / AgNOR-Analyse bei Astrozytomen des Kindesalters.

To evaluate the malignant potential of a tumor, data about its proliferative rate are essential. In this study clinical data, histological grading and immunohistochemical marker PCNA were applied on astrocytomas in childhood and were correlated to number, size and distribution of AgNORs. Tumor material from 90 patients, aged 1 to 15 years, was available. Histological grading, immunohistochemistry and AgNOR staining were performed on paraffin sections. PCNA-positivity was evaluated semiquantitatively. The AgNORs were quantified on 100 nuclei/case with an image analysis system. With aggravating malignancy we could show a shorter anamnesis, a lower survival and a higher PCNA-reactivity. We found a strong correlation between several AgNOR-features and the grade of malignancy of the astrocytomas, especially for the number of the AgNORs (from grade I: 1.73 AgNORs/nucleus to grade IV: 4.56 AgNORs/nucleus), the size of the AgNORs and its area amount of the nucleus. There were correlations between AgNOR-number and PCNA-reactivity as well as AgNOR-number and survival. Analysis of AgNORs, consequently, should provide prognostically relevant information helpful in more effective grading of astrocytomas.