Autoimmune polyendocrine syndrome type 1: case report and review of literature

Autoimmune polyendocrine syndrome type 1 (APECED) is a rare autosomal recessive disorder characterized by autoimmune multiorgan attack. The disease is caused by mutations in the autoimmune regulator gene (AIRE), resulting in defective AIRE protein, which is essential for selftolerance. Clinical manifestations are widely variable. Although the classic triad is composed by mucocutaneous candidiasis, hypoparathyroidism and adrenal failure, many other components may develop. Treatment is based on supplementation of the various deficiencies, and patients require regular follow-up throughout their lifespan. This article describes the case of a patient with the disease, and reviews literature data on the epidemiology, clinical course, immunogenetic aspects, diagnosis and treatment of the syndrome.     

Fundamentals of randomized clinical trials in wound care: Reporting standards

In wound care research, available high‐level evidence according to the evidence pyramid is rare, and is threatened by a poor study design and reporting. Without comprehensive and transparent reporting, readers will not be able to assess the strengths and limitations of the research performed. Randomized clinical trials (RCTs) are universally acknowledged as the study design of choice for comparing treatment effects. To give high‐level evidence the appreciation it deserves in wound care, we propose a step‐by‐step reporting standard for comprehensive and transparent reporting of RCTs in wound care. Critical reporting issues (e.g., wound care terminology, blinding, predefined outcome measures, and a priori sample size calculation) and wound‐specific barriers (e.g., large diversity of etiologies and comorbidities of patients with wounds) that may prevent uniform implementation of reporting standards in wound care research are addressed in this article. The proposed reporting standards can be used as guidance for authors who write their RCT, as well as for peer reviewers of journals. Endorsement and application of these reporting standards may help achieve a higher standard of evidence and allow meta‐analysis of reported wound care data. The ultimate goal is to help wound care professionals make better decisions for their patients in clinical practice.     

Deep sternal wound infections following open heart surgery in Iceland. A population-based study

Objective. The aim of this nationwide case-control study was to study the epidemiology and identify risk factors of deep sternal wound infections (DSWI) in Iceland. Patients and methods. Between 1997–2004, 1 650 adults underwent open cardiac surgery in Iceland. For every infected patient four control subjects were chosen (n =163), matched for time of operation. The groups were compared by multivariable logistic regression analysis. Results. Forty one patients (2.5%) developed DSWI, most often following CABG (76%). The most common pathogens were Staphylococcus aureus (39%) and coagulase-negative staphylococci (24%). All except two patients underwent debridement and rewiring of the sternum. Length of hospital stay was significantly longer in the DSWI group with a trend for increased hospital mortality and significantly greater 1-year mortality (17% vs. 5%, p =0.02). History of stroke (OR 5.12), peripheral arterial disease (OR 5), corticosteroid use (OR 4.25), smoking (OR 3.66) and re-operation for bleeding (OR 4.66) were the strongest independent predictors for DSWI. Conclusion. Incidence of DSWI in Iceland (2.5%) is comparable to other recently published studies, with similar risk factors and significantly reduced survival at one year following the infection.     

Delayed Surgery Does Not Reduce The Interface Strength Between The Surface Of A Bone Fracture And A Self-Curing Injectable Hydroxyapatite (Norian SRS)

The shear strength of the interface between bone and an injectable calcium phosphate bone substitute that cures to form a carbonated apatite (Norian SRS) was measured and related to the time that elapsed between injury and surgery. Eleven rabbits had a 3 mm drill hole made in the retropatellar aspect of the distal femur. After one week, the rabbits were reoperated on and the hole filled with Norian SRS after it had been cleaned gently with a sponge. During the same procedure a similar hole was burred in the opposite femur and filled with Norian SRS after a few minutes ("immediate injection"). Four other rabbits had only one side operated on with immediate injection of Norian SRS to compare with the opposite untreated side. The rabbits were killed four days after the injection of Norian SRS. The femurs were prepared and sawed perpendicularly to the burr channels to produce discs 3.5 mm thick. A push out test of the Norian SRS plug within the bone disc was done to measure the force at failure. In the immediately injected specimens the failure occurred at a mean of 28 N (range 5-57) compared with 42 N (range 25-65) in the specimens injected after a one week delay. There was no significant difference between delayed and immediate treatment (95% confidence interval -5 to 133). The histological examination showed that 9 of the 12 specimens had Norian SRS still adherent to half or more of the circumference of the hole. This indicates that the failure occurred in the Norian SRS rather than in the bone or at the interface.     

von Langenbeck procedures at 8 months or Wardill at 18 months for primary repair of cleft palate in adult Swedish patients with unilateral complete cleft lip and palate: A study of facial growth

The purpose of this study was to compare facial growth in patients with unilateral complete cleft lip and palate (UCLP) in whom the palate was repaired with the von Langenbeck procedure at 8 months (L-8), or the Wardill procedure at 18 months (W-18). A total of 151 unaffected subjects were used as a reference series. Sixty-one adult patients, 34 in the L-8 group and 27 in the W-18 group, were investigated clinically and with lateral skull radiography and also filled out a questionnaire about their dental condition. Multiple linear regression was used to analyse the effects of the surgical strategy, sex, and the presence of a velopharyngeal flap on several dependent variables indicating sagittal jaw relations, vertical jaw relations, and inclination of incisors. The UCLP group was characterised by a short (n-sp) and retrusive maxilla (s-n-ss) and a retrusive mandible for women (s-n-sm), larger maxillary inclination (NSL/NL), more retroclined lower incisors (IL_I/ML), shorter upper facial heights (n-sp) and normal total face height (n-gn) than the reference group. In the UCLP group there were significant differences between men and women in mandibular prognathism (s-n-sm), maxillary/mandibular relations (ss-n-sm), maxillary inclination (NSL/NL), total anterior facial height (n-gn), and upper anterior facial height (n-sp). Lateral crossbite was found in 20% of the UCLP group. The prevalence was similar in the L-8 and W-18 groups. Almost 80% of the patients in the UCLP group considered that their dental condition was good. The choice of surgical strategy had no significant influence on the variables measured on facial morphology.     

Subarachnoid blood infusion versus raised intracranial pressure: Effects on the amino acid pattern in the extracellular fluid of the rabbit hippocampus

Abstract

In order to evaluate the role of a hemorrhage versus that of a transient increase in intracranial pressure in subarachnoid hemorrhage, the two components were induced separately in rabbits. Extracellular glutamate, sampled from the hippocampus with microdialysis, was used to evaluate the degree of CNS tissue damage. In four rabbits, autologous arterial blood was infused in the cisterna magna in a volume that would not affect the intracranial pressure. The other group of animals was infused with saline to elevate the intracranial pressure from 10 to > 100 mmHg. The increase of intracranial pressure per se did not induce significant changes in extracellular glutamate. However, 20-60 min after infusion of blood, a significant glutamate increase was recorded. Furthermore, aspartate, alanine, glycine and serine were also raised. The results indicate that blood in the subarachnoid space damages the brain primarily by inducing ischemia. Furthermore, the parameters employed gave no indication that an increase in intracranial pressure had a deleterious effect on CNS tissue. [Neurol Res 1999; 21: 404-408]