Cranial fasciitis of childhood with reactive periostitis

We report a rare case of cranial fasciitis in an 11-year-old girl. The patient was found to have an ossifying soft tissue mass over the occiput that was arising from the cranial periosteum and the deep fascial layer. An associated florid periosteal reaction was found in the occipital bone adjacent to the scalp mass. The computed tomography and histologic pattern of this unusual lesion are discussed. A brief review of the literature is included emphasizing the need to investigate further this completely benign lesion that frequently is confused with a malignant neoplasm.     

Seven DNA polymorphisms in the LDL receptor gene: application to the study of familial hypercholesterolemia in Spain

We have performed restriction fragment length polymorphism (RFLP) analysis at the low density lipoprotein receptor (LDLR) locus in order to investigate the molecular genetics of familial hypercholesterolemia (FH) in Spain. Firstly, a sample of 50 unrelated patients with a clinical diagnosis of FH was screened for the presence of major rearrangements at this locus by Southern blot analysis of Bgt II digested genomic DNA. Four different mutations were detected, accounting for 8% of the mutant alleles in the Spanish FH sample. Then, we determined the relative allele frequency and estimated linkage disequilibrium between seven RFLPs of the LDLR gene in the remaining 46 FH patients and in 61 normolipidemic controls. Hindi, Avail, Pvu II, Msp I, and Nco I are the most polymorphic sites with individual PIC values higher than 0.28, whereas the Taq I and Stu I sites display low levels of polymorphism. The usefulness of the seven RFLPs to confirm a clinical diagnosis of FH was investigated in 15 FH-families, consisting of 118 individuals, in whom the presence of Familial Defective Apolipoprotein B-100 (FDB) due to the apoB₃₅₀₀ mutation was excluded. Independent haplotypes were constructed for 71 chromosomes: 15 FH and 56 control haplotypes. A total of 14 different haplotypes was found. In 12 families, clinical diagnosis of FH was confirmed by cosegregation analysis, which makes these RFLPs useful for studying the inheritance of the LDLR gene in 80% of Spanish families with FH. Comparison of haplotypes found in the Spanish sample with those found in Swiss and Norwegians suggests heterogeneity of haplotypes among European populations.     

Comparative study of roles of the lobus parolfactorius and intermediate medial hyperstriatum ventrale in memory formation in the chick brain.

Two discrete areas of the chick brain, the intermediate medial hyperstriatum ventrale (IMHV) and lobus parolfactorius (LPO), were found to have different functions during the formation of memory for a 1-trial peck-avoidance paradigm. Glutamate, ouabain, and emetine, known to disrupt short-, intermediate-, and long-term memory when injected into the IMHV, were injected into the cerebellum and LPO. All amnestic agents investigated produced amnesia when injected into the IMHV; only one of these agents produced amnesia when injected into the LPO, and none of the agents produced amnesia when injected into the cerebellum. The chick brain was also found to exhibit hemispheric asymmetries: The left IMHV and LPO were more sensitive to the amnestic agents than their corresponding right structure. From these data, hypotheses for the roles of these structures during memory are proposed.     

Liver Transplant Recipients Older Than 60 Years Have Lower Survival and Higher Incidence of Malignancy

Older age is not considered a contraindication for liver transplantation, but age-related morbidity may be a cause of mortality. Survival and the incidence of the main post-transplant complications were assessed in 111 adult liver transplant recipients. They were divided in two groups according to their age (patients younger than 60 years, n=54; patients older than 60 years, n=57) and both groups were compared. Older patients were more frequently transplanted for hepatitis C (p= 0.03) and hepatocellular carcinoma (p= 0.05) and their liver disease was less advanced (Child-Pugh and MELD scores were significantly lower; p=0.004 and p=0.05, respectively). After transplantation, older patients had a significantly lower survival (p=0.02). Higher age was independently associated with mortality (hazard ratio for each 10-year increase: 2.1; 95% confidence interval: 1.1- 4.0; p=0.02). The incidence of de novo neoplasia and nonskin neoplasia were higher in older patients (p=0.02 and p =0.007, respectively). Malignancy was the cause of death in one patient younger than 60 years and in 12 patients older than 60 years (p =0.002). In multivariate analysis, a higher age and smoking were independently associated with a higher risk of dying of de novo neoplasia. In conclusion, older liver transplant recipients have a significantly lower survival than younger patients. Malignancy is responsible for this decreased survival.     

Effects of postoperative pain on lung volume in patients undergoing aortic surgery

To evaluate the potential advantages of the administration of extradural morphine to control postoperative pain and its effects on respiratory function, 39 patients were randomly assigned to one of two groups before aortic surgery. The first group (20 patients) received intravenous analgesia as required (control group). The second group (19 patients) received extradural morphine in a programmed fashion. During the immediate postoperative period the following parameters were measured in both groups: respiratory rate, vital capacity, peak expiratory volume in the first second, PaO2, PaCO2, arterial pH, heart rate, and systolic and diastolic blood pressure. In the group treated with morphine the postoperative increase in heart and respiratory rate was significantly smaller than in the control group (p less than 0.01). Postoperative forced pulmonary volumes were higher in the morphine group (p less than 0.01). However, there were no differences in time of hospitalization between both groups. There were more complications in the control group, but the difference did not reach statistical significance.     

Numerical method for weights adjustment in minimax multi‐model LQ‐control

The minimax linear quadratic problem, where ‘max’ is taken over a finite set of indices (models) and ‘min’ is taken over the set of admissible controls, is considered. The solution is obtained by the robust optimal control application. The control turns out to be a linear combination of the controls optimal for each individual model. This paper develops a numerical method for the optimal weights adjustment. An example shows a quick convergence of the proposed procedure. Copyright © 2007 John Wiley & Sons, Ltd.     

Galea and subgalea graft for lip augmentation revision

In this paper we will review the results obtained during the last 2 years with the aponeurotic galea and subgalea for vermillion lip augmentation. The survey was carried out on 42 patients who displayed either an absence of or reduction in the vermillion of one or both lips or a senile lip. In all cases the surgical procedure introduced aponeurotic galea and subgalea in the space found between the orbicular lip muscle and the vestibular mucus, just behind the vermillion. All the operations were performed under local anaesthetic. The size of the aponeurotic galea fragment removed varied in length between 10 and 12 cms. and in width between 1 and 2 cms., using the following parameters for its dimensions: the previous volume of the lips, and the distance between the two buccal commissures when in the “smile position.”     

Portal hypertension in Williams syndrome: report of two patients

Williams or Williams-Beuren syndrome (WBS) is a developmental disorder with multisystemic manifestations characterized by distinctive facial features, mental disability with unique cognitive and personality profiles, vascular stenoses, growth retardation, and occasional infantile hypercalcemia, caused by haploinsufficiency for genes deleted in chromosome band 7q11.23. However, with the exception of arterial stenoses caused by haploinsufficiency for the elastin gene (ELN), no specific implication of any other gene in the phenotype has been established. We present two patients with portal hypertension leading to splenomegaly and pancytopenia carrying the common 1.5 Mb WBS deletion. We propose this is an additional severe vascular complication of ELN deficiency and discuss the specific characteristics of the portal venous tract that could explain the impact of ELN deficiency in that venous territory. This complication is potentially lethal and should thus be considered in any patient with WBS and splenomegaly.     

Intestinal microvillous atrophy in a patient with Down syndrome and aganglionic megacolon

Intestinal microvillous disorders are an uncommon cause of severe diarrhea, with very poor prognosis. The authors report the case of a female infant with Down syndrome, aganglionic megacolon, severe diarrhea, and jejunal biopsy with ultrastructural changes consistent with microvillous atrophy. The patient condition improved after a colostomy performed in the setting of the treatment of Hirschprung disease.     

Infection of rats with Taenia taeniformis metacestodes increases hepatic CYP450, induces the activity of CYP1A1, CYP2B1 and COH isoforms and increases the genotoxicity of the procarcinogens benzo[a]pyrene,cyclophosphamide and aflatoxin B(1)

Infection of rat liver by Taenia taeniformis metacestodes produced an increase in total CYP450 content and induced activity of the CYP1A1, CYP2B1 and COH isoforms. Variations in activity and p450 total content were found with increasing time of infection. During increased activity of p450 isoforms, rats were challenged with carcinogens metabolized by the mentioned isozymes and an increased amount of genotoxic damage was found when benzo[a] pyrene, cyclophosphamide and aflatoxin B(1) were used. No change was seen in CYP2E1 activity. These results support previous findings regarding an increased susceptibility to genotoxic damage of infected organisms.