Fibrous dysplasia of the jaws: Integrating molecular pathogenesis with clinical,radiological, and histopathological features

Fibrous dysplasia is a non‐neoplastic developmental process that affects the craniofacial bones, characterized by painless enlargement as a result of bone substitution by abnormal fibrous tissue. Postzygotic somatic activating mutations in the GNAS1 gene cause fibrous dysplasia and have been extensively investigated, as well as being helpful in the differential diagnosis of the disease. Fibrous dysplasia may involve one (monostotic) or multiple bones (polyostotic), sporadically or in association with McCune‐Albright syndrome, Jeffe‐Lichenstein syndrome, or Mazabreud syndrome. This review summarizes the current knowledge on fibrous dysplasia, emphasizing the value of integrating the understanding of its molecular pathogenesis with the clinical, radiological, and histopathological features. In addition, we address important aspects related to the differential diagnosis and patient management.     

Alcohol consumption patterns and predictors of use following liver transplantation for alcoholic liver disease.

For patients who receive a liver transplant (LTX) for alcoholic liver disease (ALD), investigators are focusing beyond survival to determine specific alcohol use outcomes. Studies suggest the use of alcohol ranges from 8 to 22% for the first post-transplant year with cumulative rates reaching 30 to 40% by 5 years following transplantation. Yet while investigators are interested in determining specific rates of alcohol use and predictors of use, only three studies since 1990 have been prospective. In 1998, we began a prospective study of post-LTX alcohol consumption in ALD recipients using multiple repeated measures of alcohol use. After 5 years of follow-up, we found that 22% had used any alcohol by the first year and 42% had a drink by 5 years. By 5 years, 26% drank at a heavier use (binge) pattern and 20% drank in a frequent pattern. In a univariate model, predictors of alcohol use included pre-transplant length of sobriety, a diagnosis of alcohol dependence, a history of other substance use, and prior alcohol rehabilitation.     

Isolated right atrial appendage (RAA) rupture in blunt trauma – a case report and an anatomic study comparing RAA and right atrium (RA) wall thickness

Background  

Heart chambers rupture in blunt trauma is uncommon and is associated with a high mortality. The determinant factors, and the incidence of isolated heart chambers rupture remains undetermined. Isolated rupture of the right atrium appendage (RAA) is very rare, with 8 cases reported in the reviewed literature. The thin wall of the RAA has been presumed to render this chamber more prone to rupture in blunt trauma.     

Characterization of the human jumonji gene

While constructing a cDNA library of human embryos, we have isolated a clone homologous to jumonji, a mouse gene required for neural tube formation. We have determined the complete coding sequence of the human homologue (JMJ) and deduced the amino acid sequence of the putative protein. We show here that human and mouse jumonji putative proteins are homologous and present 90% identity. During human embryogenesis, JMJ mRNAs are predominantly expressed in neurons and particularly in dorsal root ganglion cells. They are also expressed in neurons of human adult cerebral cortex. In view of these observations, we propose JMJ as a candidate gene for developmental defects of the central nervous system in the human. The human JMJ gene maps at position 6p24-6p23.      

Die Lipoide der weißen Hirnsubstanz während der Autolyse und bei der anämischen Erweichung

Zusammenfassung Während der sterilenAutolyse der weißen Hirnsubstanz bis zu 24 Tagen verändern sich die Lipoide nur geringfügig. Die erst vom 9. Tage an verstärkte Abnahme des Lipoid-P beruht auf der Spaltung von Lecithin und Plasmalogen und wird von einer entsprechenden Zunahme freier Fettsäuren (FFS), geringerer der Aldehyde begleitet. FFS sind in der frischen weißen Substanz nur in Spuren nachweisbar. Lysophosphatide treten—im Gegensatz zu anderen Organen—in papierchromatographisch nachweisbarer Menge nicht auf. Cholesterin und seine Ester bleiben unverändert. Kephaline, Inositphosphatide, Sphingomyelin, Cerebroside und Sulfatide verhalten sich chromatographisch weitgehend konstant. Die postmortale Autolyse hat bis mehrere Tage nach dem Tode keinen nennenswerten Einfluß auf die Lipoidzusammensetzung.Bei der Untersuchung von 20 verschiedenenErweichungsherden des Gehirns fand sich eine vom Alter der Herde nicht unbedingt abhängige Zunahme der FFS, Aldehyde, Triglyceride und Cholesterinester. Freie wie auch glycerid- und cholesteringebundene Fettsäuren und freie höhere Aldehyde stammen offensichtlich aus den strukturbildenden Lipoiden des Myelin. In verschiedenen Herden treten spurenweise Lysophosphatide auf.

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Summary Lipides change slightly during sterile autolysis of human white matter up to the 24 th day. From the 9 th day on, there is a decrease of lipid-phosphorus caused by a breakdown of lecithin and plasmalogen accompanied by a comparable increase of free fatty acids (FFA) and aldehydes. In fresh white matter there are only traces of FFA. In the autolysing brain, contrary to several other autolysing organs, there are no lysophosphatides detectable by means of paper chromatography. Free and esterified cholesterol, cephalins, phosphoinositides, sphingomyelin, cerebrosides and sulfatides remain chromatographically constant during sterile autolysis. According to the above findings it can be concluded that postmortem autolysis up to several days duration has only neglectable effects on the lipid-composition of the white matter.After analysis of 20 different foci of anaemic softening of human brain we found an increase of FFA, aldehydes, triglycerides and of esterified cholesterol which is independent on the age of the lesion. FFA as well as those fatty acids which are bound to glycerol and cholesterol and free higher aldehydes derive from the myelin lipides. Also in contrast to autolysis there were traces of lysophosphatides found in several foci.

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Herrn Prof. Dr.E. Letterer zum Geburtstag am 30. Juni 1966 gewidmet.

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Mit Unterstützung durch die Deutsche Forschungsgemeinschaft.     

Present status of bancroftian filariasis in Maceió, State of Alagoas, Brazil

Epidemiological and entomological surveys were carried out in the human and mosquito populations in Maceió, Alagoas, in order to assess the present status of bancroftian lymphatic filariasis. Examination of thick blood smears of 10,450 students from different areas of the city revealed 0.66% Wuchereria bancrofti microfilaria carriers. The distribution of filariasis is focal in the city, 80% of the individuals with patent infection living in two neighboring areas with 1.24% and 5.25% prevalence. Parallel studies performed with samples of all age groups in the human population showed similar microfilaria prevalence rates observed previously in the student survey. However, thick blood smears taken from members of families with at least one subject with patent infection gave a prevalence six times greater suggesting, increased transmission in households. The percentage of carriers was higher in the youngest age group (< 20 years). Culex quinquefasciatus mosquitos caught at the locations where the autochthonous cases were found presented natural infection rates ranging from 0.28% to 4.62%. The combination of all these findings indicates occurrence of active transmission of W. bancrofti in the urban area of Maceió, Alagoas State. Based on these data, measures for the potential control of filariasis were planned.     

Nonpuerperal breast infection

Objective: We undertook a microbiological study of purulent specimens from women with symptomatic breast abscesses.Methods: Fifty-one purulent samples were collected in 2 periods (December 1991-April 1992 and January 1994-June 1994) from nonpuerperal breast abscesses in 44 patients attending our hospital.Results: One of the most frequently isolated microorganisms was Proteus mirabilis (9 patients, 20.4%), present as a pure culture in all but 1 specimen (isolated together with Peptostreptococcus spp.). Staphylococcus aureus was isolated in 10 specimens, 6 of which were post-tumorectomy abscesses. Polymicrobial anaerobic flora were isolated in 11 specimens (21.5%); Staphylococcus epidermidis in 4 (8%); and Streptococcus milleri,Alcaligenes sp., and mixed aerobic-anaerobic flora in 1 specimen each. The 7 remaining samples (13.7%) were negative bacteriological cultures.Conclusions: We draw attention to the frequent isolation of P. mirabilis in recurrent and torpid breast abscesses in 4 women in whom surgery was necessary in addition to antibiotic treatment.     

Gut ischemia/reperfusion activates lung macrophages for tumor necrosis factor and hydrogen peroxide production

BACKGROUND: Gut ischemia followed by reperfusion (I/R) is implicated as a prime initiating event in the mechanism of multiple organ failure after trauma and hemorrhagic shock. Several lines of evidence indicate that macrophages are involved in this prime event. Our purpose was to evaluate hydrogen peroxide (H2O2) and tumor necrosis factor (TNF) production and phagocytosis by lung macrophages in a gut I/R model of multiple organ failure in rats. METHODS: In the experimental group (I/R), Wistar rats (n = 35) were anesthetized and subjected to a median laparotomy, and the superior mesenteric artery was clamped for 45 minutes followed by 60 minutes of reperfusion. In the control group (LAP) (n = 37), animals underwent sham laparotomy. After the period of reperfusion, bronchoalveolar lavage (BAL) was performed and the resulting BAL cells were assayed for H2O2 production using the horseradish peroxidase-mediated red phenol oxidation method. TNF release was determined using the L929 cells bioassay. Zymosan phagocytosis by BAL macrophages was quantitated using phase microscopy. RESULTS: H2O2 release in BAL cells of I/R rats (19.90 +/- 7.98 nmol/L/2 x 10(5) cells) is statistically higher than in the LAP group (10.92 +/- 5.01 nmol/L per 2 x 10(5) cells) (p = 0.0155), and the TNF production by BAL cells of the I/R group (38.09 +/- 20.79 units per 10(6) cells) was significantly higher than that of LAP rats (17.16 +/- 13.35 units per 10(6) cells) (p = 0.0281). Phagocytic activity of BAL mac. Macrophages of I/R rats was not statistically different from LAP animals. CONCLUSION: These results suggest that BAL macrophage play a role in the mechanism of acute lung injury after trauma and hemorrhagic shock.     

Tracheostomy in children: there is a place for acceptable risk

BACKGROUND: Tracheostomy in children remains controversial regarding the risk of complications. METHODS: Forty-six trauma patients (35 male and 11 female, mean age = 6.8 years) were admitted to the intensive care unit between 1987 and 1991 with severe head injury plus coma. Tracheostomy was performed with standard technique after 5.9 days (range, 2-12 days) of intubation. RESULTS: There were no deaths from tracheostomy, but six deaths resulted from severe head injury. One child was discharged with tracheostomy. The 39 survivors remained with tracheostomy 16.14 days (range, 4-71 days) in the intensive care unit. After cannula removal, 31 remained asymptomatic; 8 had respiratory distress: 2 were normal, 5 had endoscopic treatment for subglottic granulomas/stenosis from intubation, and 1 had tracheomalacia from tracheostomy. In 1997, the 18 patients located for follow-up were asymptomatic. At endoscopy, 8 were normal, 9 had subglottal granulomas from intubation, and 1 had 20% tracheal stenosis from tracheostomy. CONCLUSION: Most complications after tracheostomy result from intubation. Tracheostomy has an acceptable risk in children with severe head injury who need prolonged ventilatory support.