Congenital cerebellar malignant rhabdoid tumor in an infant with junctional epidermolysis bullosa.

Epidermolysis bullosa (EB), a hereditary blistering condition of the skin, is divided into simplex, hemidesmosomal, junctional, and dystrophic types. It may be complicated by the development of squamous cell carcinoma of the skin, but other neoplasms, especially those separate from involved skin, are distinctly rare. We report a male infant with junctional EB who died of Pseudomonas sepsis and was found at autopsy to have a clinically unrecognized cerebellar malignant rhabdoid tumor (MRT).

Sudden death in toddlers with viral meningitis, massive cerebral edema, and neurogenic pulmonary edema and hemorrhage: report of two cases.

Viral (lymphocytic) meningitis typically does not cause sudden death, especially in the absence of severe inflammation in the brain or other organs. We report 2 toddlers with clinical evidence of a viral infection who died unexpectedly and were found at autopsy to have lymphocytic meningitis associated with severe brain edema, transtentorial herniation, neurogenic pulmonary edema and hemorrhage, and cardiomegaly. Influenza A virus, demonstrated in tracheal epithelium by immunocytochemistry, is the presumed cause of the mild meningitis in 1 case; adenovirus was cultured from swabs of the brain and anus in the 2nd case.

Delayed death in sudden infant death syndrome: a San Diego SIDS/SUDC Research Project 15-year population-based report.

A fraction of SIDS cases have death delayed by successful CPR, yet they have not been compared to SIDS cases which were found dead or not successfully resuscitated. Our aims were to: (1) determine the percent of SIDS cases in the San Diego SIDS Research Project database for whom death was delayed by CPR and subsequent life support; (2) compare demographics, circumstances of death and autopsy findings of delayed death SIDS cases (delayed SIDS) with those whose deaths were not delayed (non-delayed SIDS); (3) examine the evolution of pathologic changes in delayed SIDS as a function of survival interval. A retrospective 15-year population-based study of 454 infant deaths attributed to SIDS revealed 29 delayed SIDS cases (Group I) and 425 non-delayed SIDS cases (Group II).

Unclassified sudden infant death associated with pulmonary intra-alveolar hemosiderosis and hemorrhage.

The significance of severe pulmonary intra-alveolar hemosiderosis in sudden infant death is controversial in forensic pathology. We report a previously healthy 9-month-old female infant who died suddenly and unexpectedly after being placed and then found prone in her crib. Her gestation and delivery were uncomplicated, and she had no history of anemia, hemoptysis, chest trauma, or chronic lung disease.

Hemoglobin F in sudden infant death syndrome: a San Diego SIDS/SUDC Research Project report.

Whether levels of fetal hemoglobin (HbF), a possible marker of antecedent hypoxemia, are increased in sudden infant death syndrome (SIDS) compared to controls is unresolved. Our aims are to: (1) Compare percent fetal hemoglobin (%HbF) levels in SIDS and control cases, and (2) compare our findings with those reported in previous studies. Using Triton-acid-urea gel electrophoresis and quantitative densitometry, %HbF was determined in whole blood specimens obtained at autopsy from SIDS and control cases accessioned into the San Diego SIDS/SUDC Research Project database.

Nephrogenic systemic fibrosis with multiorgan involvement in a teenage male after lymphoma, Ewing's sarcoma, end-stage renal disease, and hemodialysis.

Nephrogenic systemic fibrosis (NSF) is a rare condition that always occurs after acute or chronic renal failure with or without dialysis. The vast majority of cases in the literature are adults, and postmortem findings have been reported in only 5 cases. We report a 15-year-old male who developed NSF with multiorgan involvement after successful treatment of renal lymphoma and a subsequent sacral Ewing's sarcoma, and end-stage renal disease treated with hemodialysis.

Use of isolated hepatocyte preparations for cytochrome P450 inhibition studies: comparison with microsomes for Ki determination.

Predicting drug-drug interactions requires an assessment of the drug concentration available to the enzyme active site, both in vivo, and within an in vitro incubation. These predictions are confounded when the inhibitor accumulates within the liver, either as a result of active transport processes or intracellular binding (including lysosomal trapping). In theory, hepatocytes should provide a more accurate estimation of inhibitory potency compared with microsomes for those compounds that undergo hepatic accumulation.

Structure/function characterization of micro-conotoxin KIIIA, an analgesic, nearly irreversible blocker of mammalian neuronal sodium channels.

Peptide neurotoxins from cone snails continue to supply compounds with therapeutic potential. Although several analgesic conotoxins have already reached human clinical trials, a continuing need exists for the discovery and development of novel non-opioid analgesics, such as subtype-selective sodium channel blockers. Micro-conotoxin KIIIA is representative of micro-conopeptides previously characterized as inhibitors of tetrodotoxin (TTX)-resistant sodium channels in amphibian dorsal root ganglion neurons.

A (27)Al MAS NMR study of a sol-gel produced alumina: Identification of the NMR parameters of the theta-Al(2)O(3) transition alumina phase.

(27)Al MAS NMR has been used to study a sol-gel prepared alumina annealed at various temperatures. Two-field simulation of the sample heated to 1200 degrees C confirmed the presence of corundum, as suggested by XRD, and also the presence of nanocrystalline theta-Al(2)O(3). (27)Al MAS NMR chemical shifts, quadrupolar coupling constants and asymmetry parameters are reported for the tetrahedral and octahedral aluminium sites within theta-Al(2)O(3).

Characterisation of nanocrystalline magnesium oxide by X-ray absorption spectroscopy.

There are numerous methods of preparing nanocrystalline materials. Magnesium oxide is an ideal model system on which to probe the relation of the preparative route and the microstructure. Using X-ray absorption spectroscopy (XAS) we show that the sol-gel route can be used to prepare highly crystalline material provided there is careful control of the calcination conditions.

Sudden unexpected death in childhood associated with cardiac rhabdomyoma, involuting adrenal ganglioneuroma, and megalencephaly: another expression of tuberous sclerosis?

We report a 9-year-old, previously healthy girl who died suddenly and unexpectedly and was found at postmortem examination to have a cardiac rhabdomyoma, megalencephaly, and an involuting adrenal ganglioneuroma. Her death was possibly caused by a fatal cardiac arrhythmia resulting from interference of the ventricular septal rhabdomyoma with the cardiac conduction fibers. Her extended family history included a variety of disorders, including cleft lip and palate and ill-defined cardiac and neurologic diseases.

A 27Al, 29Si, 25Mg and 17O NMR investigation of alumina and silica Zener pinned, sol-gel prepared nanocrystalline ZrO2 and MgO.

Alumina and silica Zener pinning particles in sol-gel prepared nanocrystalline ZrO2 and MgO have been characterised using 27Al and 29Si MAS NMR after annealing at various temperatures up to 1200 degrees C. The structures of the pinning phases were found to differ not just between the two metal oxide systems but also depending on the exact method of manufacture. Three distinct transitional alumina phases have been observed in different alumina-pinned samples annealed at 1200 degrees C, one in particular identified by a peak at a shift of 95 ppm in the 27Al NMR spectrum.

Aspiration of gastric contents in sudden infant death syndrome without cardiopulmonary resuscitation.

Objectives: (1) To compare demographic profiles among sudden infant death syndrome (SIDS) infants with or without gastric aspiration, for whom cardiopulmonary resuscitation (CPR) had not been attempted; (2) to review the severity and potential significance of aspiration in those SIDS cases; and (3) to assess the risk of supine sleep position with regard to gastric aspiration.

Study Design: Retrospective review of records and microscopic slides for all postneonatal SIDS cases (29 to 365 days of age) accessioned by the San Diego County Medical Examiner from 1991 to 2004.

Results: Ten (14%) of 69 cases of SIDS infants who had not undergone CPR before autopsy revealed microscopic evidence of gastric aspiration into the distal lung; this group was not otherwise clinically or pathologically different from cases of SIDS infants without aspiration.

Pulmonary intra-alveolar hemorrhage in SIDS and suffocation.

The differentiation of SIDS from accidental or inflicted suffocation may be impossible in some cases. Severe pulmonary intra-alveolar hemorrhage has been suggested as a potential marker for such differentiation. Our aims are to: (1) Compare pulmonary hemorrhage in SIDS and a control group comprised of infants whose deaths were attributed to accidental or inflicted suffocation.

A comparison of pulmonary intra-alveolar hemorrhage in cases of sudden infant death due to SIDS in a safe sleep environment or to suffocation.

The differentiation of SIDS from accidental or inflicted suffocation may be impossible without corroborating findings from the death scene or autopsy or in the absence of a confession from a perpetrator. Pulmonary intra-alveolar hemorrhage (PH) has been proposed as a potential clue to suffocation, but none of the previous studies on this topic have limited SIDS cases to those who were in a safe sleep environment, in which all were found supine and alone on a firm surface with their heads uncovered. Our aims are to: (1) compare PH in SIDS cases found in a safe sleep environment to a control group comprised of infants whose deaths were attributed to accidental or inflicted suffocation and (2) assess the effect of age, CPR, and postmortem interval (PMI), with regard to the severity of PH in this subset of safe-sleeping SIDS cases.

Pneumocystis is not a direct cause of sudden infant death syndrome.

We compared the frequency of Pneumocystis in 126 sudden infant death syndrome (SIDS) cases with a control group of 24 infants from the San Diego SIDS/SUDC Research Project who died of accidental or inflicted injuries. Cysts were identified in 33% of SIDS cases and 29% of controls. We conclude that Pneumocystis is not a direct cause of SIDS.

Multiple serotonergic brainstem abnormalities in sudden infant death syndrome.

Context: The serotonergic (5-hydroxytryptamine [5-HT]) neurons in the medulla oblongata project extensively to autonomic and respiratory nuclei in the brainstem and spinal cord and help regulate homeostatic function. Previously, abnormalities in 5-HT receptor binding in the medullae of infants dying from sudden infant death syndrome (SIDS) were identified, suggesting that medullary 5-HT dysfunction may be responsible for a subset of SIDS cases.

Objective: To investigate cellular defects associated with altered 5-HT receptor binding in the 5-HT pathways of the medulla in SIDS cases.

Internet information on rheumatoid arthritis: an evaluation.

Objective: To evaluate the quality of internet information, readily accessible by the public, relating to rheumatoid arthritis and to investigate the relationship between financial interests of websites and the quality of information provided.

Methods: Five well-known search engines on the internet were investigated in an attempt to replicate a search undertaken by a typical patient. The phrase 'rheumatoid arthritis' was used for each search and the first 20 results were evaluated.

A review of the history of hand exercises in rheumatoid arthritis.

The management of the hand in the patient with rheumatoid arthritis (RA) is a challenge to all therapists and evidence for optimum management historically scarce. Given that it is estimated that hands and wrists are affected in 80-90% of RA patients (Maini and Feldmann, 1998), it is apparent that this is an important area for both the patient and practitioner. While acknowledging the importance of hand function to the patient with RA (Jones et al.

Local atomic and electronic structure in nanocrystalline Sn-doped anatase TiO2.

Tin-doped anatase TiO(2) nanopowders and nanoceramics with particle sizes between 12 and 30 nm are investigated by X-ray absorption fine-structure (EXAFS) and Mössbauer spectroscopies. Furthermore, ab initio calculations based on the density functional theory are performed to analyze changes in the electronic structure due to Sn doping. The three approaches consistently show that Sn is dissolved on substitutional bulk sites with a slight increase of the bond lengths of the inner coordination shells.

Synthesis of ordered mesoporous Fe3O4 and gamma-Fe2O3 with crystalline walls using post-template reduction/oxidation.

Ordered mesoporous Fe(3)O(4) with crystalline walls (inverse spinel structure) has been synthesized for the first time, representing to the best of our knowledge, the first synthesis of a reduced mesoporous iron oxide. Synthesis was achieved by reducing ordered mesoporous alpha-Fe(2)O(3) (corundum structure) to Fe(3)O(4) spinel then to gamma-Fe(2)O(3) by oxidation, while preserving the ordered mesostructure and crystalline walls throughout. Such solid/solid transformations demonstrate the stability of the mesostructure to structural phase transitions from the hexagonal close packed oxide subarray of alpha-Fe(2)O(3) (corundum structure) to the cubic close packed subarray of Fe(3)O(4) spinel and gamma-Fe(2)O(3).

The oxygen vacancy in crystal phases of WO(3).

The oxygen vacancy in WO(3) has previously been implicated in the electrochromism mechanism in this material. Previous theoretical calculations on the oxygen vacancy in WO(3) have not considered the full range of crystal structures adopted by the material. Here we report studies of the oxygen vacancy in seven crystal phases.

Child Protective Services referrals in cases of sudden infant death: a 10-year, population-based analysis in San Diego County, California.

The potential diagnostic significance of prior family referral to Child Protective Services (CPS) in cases of sudden infant death is unknown. Therefore, the authors retrospectively searched for CPS data for the 5-year referral history on all 533 families whose infants died suddenly from Sudden Infant Death Syndrome (SIDS), other natural diseases, accidents, or inflicted injuries and underwent postmortem examination by the medical examiner during a 10-year period. No family had more than one infant death.

Pulmonary intra-alveolar siderophages in SIDS and suffocation: a San Diego SIDS/SUDC Research Project report.

Pulmonary intra-alveolar siderophages (PS) have been suggested as a marker of previous attempts at imposed suffocation in infants dying suddenly and unexpectedly. The aims of this study were to (1) compare PS counts between cases of sudden infant death syndrome (SIDS) and a control group comprised of infants whose deaths were attributed to accidental or inflicted suffocation, (2) compare clinical variables in SIDS and control suffocation cases, and (3) review individual cases irrespective of the cause and manner of death with an average PS count greater than 200 per 20 high-power fields (hpf) per lung lobe. Retrospective assessment of siderophages in available iron-stained lung sections was undertaken in 91 SIDS cases and 29 cases of death due to suffocation (27 accidents and 2 homicides) from the San Diego SIDS and Sudden Unexplained Death in Childhood (SUDC) Research Project (SDSSRP) database.