Brain MR Imaging Findings in Woodhouse-Sakati Syndrome.

BACKGROUND AND PURPOSE: Woodhouse-Sakati syndrome is a rare autosomal recessive disorder characterized by hypogonadism, alopecia, diabetes mellitus, and progressive extrapyramidal signs. The disease is caused by biallelic pathogenic variants in the DCAF17 gene. The purpose of this study was to describe the spectrum of brain MR imaging abnormalities in Woodhouse-Sakati syndrome. MATERIALS AND METHODS: We reviewed brain MR images of 26 patients with a clinical and genetic diagnosis of Woodhouse-Sakati syndrome (12 males, 14 females; age range, 16-45 years; mean age, 26.6 years). Follow-up studies were conducted for 6 patients. RESULTS: All patients had abnormal MR imaging findings. The most common abnormalities were a small pituitary gland (76.9%), pronounced basal ganglia iron deposition (73%), and white matter lesions in 69.2%. White matter lesions showed frontoparietal and periventricular predominance. All white matter lesions spared subcortical U-fibers and were nonenhanced. Prominent perivascular spaces (15.3%) and restricted diffusion in the splenium of the corpus callosum (7.6%) were less frequent findings. Follow-up studies showed expansion of white matter lesions with iron deposition further involving the red nucleus and substantia nigra. Older age was associated with a more severe degree of white matter lesions (P < .001). CONCLUSIONS: Small pituitary gland, accentuated iron deposition in the globus pallidus, and nonenhancing frontoparietal/periventricular white matter lesions were the most noted abnormalities seen in our cohort. The pattern and extent of these findings were observed to correlate with older age, reflecting a possible progressive myelin destruction and/or axonal loss. The presence of pituitary hypoplasia and white matter lesions can further distinguish Woodhouse-Sakati syndrome from other neurodegenerative diseases with brain iron accumulation subtypes.

Definitive repair of a stab wound to the right ventricle with skin staples in emergency.

Penetrating cardiac injuries represent a challenge to surgeons. Several surgical techniques for repair of these injuries have been described. The authors report on the use of skin staples for a right ventricular stab wound in a patient in extremis.

Tract microflora in Saudi patients with cholelithiasis.

OBJECTIVES: To identify the microflora in the gallbladder of patients undergoing laparoscopic cholecystectomy for gallstones, and the antibiotic susceptibility pattern of the isolates, as well as the usefulness of Gram staining of bile at the time of operation. METHODS: Bile samples were obtained from 112 patients undergoing elective laparoscopic cholecystectomy for gallstones and inoculated directly into aerobic and anaerobic blood culture bottles in the operating theatre. Samples were also collected in sterile universal containers for Gram staining of a centrifuged deposit. Isolates were identified and their in-vitro susceptibilities determined by Kirby Bauer technique. RESULTS: Of 112 bile samples examined, 28 (25%) were culture positive, four of which contained more than one organism. The most common organisms isolated were Escherichia coli 9 (28.1%), Enterococcus faecalis 5 (15.6%) and Pseudomonas aeruginosa 3 (9.4%). In one sample we found Aeromonas hydrophilia and Enterobacter cloacae. No anaerobes were detected but Candida albicans was isolated in one case. In 19 bile samples (67.8%) organisms were identified on Gram stain. Positive bile cultures were found statistically significant (P < 0.05) in patients over the age of 50 (13/32), in patients who developed post-operative fever (6/12) and patients who developed leucocytosis (5/6). CONCLUSION: Age over 50 years was the only significant pre-operative factor associated with positive bile cultures (P < 0.05). In view of the microflora of the gallbladder and the susceptibility pattern of our isolates we would suggest that antibiotic prophylaxis recommended for laparoscopic cholecystectomy for gallstones needs to be reviewed and the role of bacteribilia in the surgical management of cholelithiasis requires further study.

An outbreak of food poisoning associated with restaurant-made mayonnaise in Abha, Saudi Arabia.

In May 1996, an outbreak of gastroenteritis occurred among customers who bought dinner from a restaurant that specialised in fried chicken in Abha city, south-west Saudi Arabia. The median incubation period was 10 hours (range: 3 to 27 hours). Of the 10 food items served, only mayonnaise (RR 2.52; 95% CI 1.71-3.73) and minced garlic (RR 1.20; 95% CI 1.02-1.41) were associated with cases. Salmonella enterica was isolated from 124 (84%) of the 159 persons with symptoms of food poisoning, and 91 (73%) were serogroup Enteritidis, phage type B 14. Mayonnaise was prepared in the restaurant using a regular blender. Minced garlic was prepared with the same blender immediately after making the mayonnaise. Unsafe storage of the mayonnaise at room temperature for a median of 6 hours could have resulted in overgrowth of bacteria and a high infective dose of bacteria per serving.