Recurrent Oral and Genital Ulcers in an Infant: Neonatal Presentation of Pediatric Behçet Disease

Behçet disease is a complex, multisystem disease characterized by recurrent oral and genital ulcerations. It rarely occurs in infants or children. Neonatal Behçet disease has been reported in infants whose ulcers resolve at or before 9 weeks of age. Few cases of neonatal Behçet disease persisting into childhood have previously been reported. We report the case of a 1‐month‐old infant who presented with severe recurrent genital ulcerations and at 6 months developed recurrent oral ulcerations. Her orogenital ulcerations continue to recur. Human leukocyte antigen testing revealed HLA‐B51 and B44 positivity. This is a case of pediatric Behçet disease in the neonatal period. Behçet disease should be considered in the differential diagnosis of recurrent genital and oral ulcerations in infants and children.     

Linear and angular measurements of computer-generated models: are they accurate, valid, and reliable?

INTRODUCTION: Traditional advanced imaging modalities such as CT and MRI are limited in their ability to perform accurate linear distance and angular measurements regardless of anatomical orientation. The construction of 3D models has been used to perform anthropometric analyses as well as in the reconstruction of rapid prototypes. We hypothesized that such measurements would be precise to within 2 mm or 2 degrees of measurements performed with a coordinate measurement machine (CMM). We also hypothesized that there would be a high degree of interobserver reliability with these measurements. MATERIALS AND METHODS: Multiple aluminum screws were implanted in various positions in three foam pelvises which were subsequently scanned by CT and rendered as 3D models using a commercially available software package (Mimics). Linear and angular measurements were performed using a CMM machine, the software package, and a dial caliper or goniometer. The deviation of the measurements from the CMM data was compared using ANOVA. The interobserver reliability of both the manual and computer-generated measurements was calculated. RESULTS: The mean difference between the CMM distances and those measured manually and with the software was 2.12 +/- 1.20 mm and 1.57 +/- 1.05 mm, respectively. The mean difference between the CMM angular measurements and the angular measurements performed manually and with the software was 4.07 +/- 4.70 degrees and 1.62 +/- 1.32 degrees, respectively. In all cases, the manual measurements were significantly less accurate (p < 0.0001) and there was a high degree of interobserver reliability. CONCLUSIONS: Computer-generated measurements taken from three-dimensionally reconstructed models are more accurate than manual measurements and are within 2 mm and 2 degrees of measurements performed with a CMM. These measurements have high interobserver reliability.     

Intratemporal facial nerve transfer with direct coaptation to the hypoglossal nerve.

OBJECTIVE: To evaluate functional recovery after facial-hypoglossal nerve transfer with direct coaptation of the intratemporal part of the facial nerve. STUDY DESIGN: Retrospective study. SETTING: University-based tertiary referral center. PATIENTS: Nine patients who underwent facial-hypoglossal transfer surgery between 2001 and 2006 to treat a unilateral complete facial nerve palsy. INTERVENTION: The facial nerve is mobilized in the temporal bone, transsected at the second genu, transferred and directly coaptated to a partially incised hypoglossal nerve. MAIN OUTCOME MEASURES: The House-Brackmann grading system was used to evaluate facial nerve reinnervation. Tongue atrophy and movements were documented. Quality of life related to facial function was assessed using the validated Facial Disability Index. RESULTS: A House-Brackmann Grade III (86%) was achieved in six patients, and Grade IV (14%) in one patient with an average follow-up of 22 months (range, 12-48 mo). Two patients had a follow-up of less than 12 months after surgery, and reinnervation was still in progress. In none of the patients who were operated on was tongue atrophy or impaired movement observed. Postoperative Facial Disability Index scores (mean, 71.8 +/- standard deviation [SD] 10.6) for physical functioning and social functioning (mean, 85.7 +/- SD 9.8) were increased for all patients when compared with preoperative scores (mean, 28.6 +/- SD 9.0; mean, 37.7 +/- SD 14.4, respectively). CONCLUSION: The facial-hypoglossal nerve transfer with direct coaptation of the intratemporal part of the facial nerve offers good functional results with low lingual morbidity and improved quality of life. The technique is straightforward, relatively simple, and should be considered as first option for reanimation of traumatic facial nerve lesions.     

Cancer of the maxillary sinus treated with surgery and/or radiation therapy

This is an analysis of 37 previously untreated patients with squamous cell carcinoma of the maxillary sinus treated with curative intent at the University of Florida from January 1966 through January 1984. All patients were followed for at least two years and 86 per cent (32/27) were followed for a minimum of five years. Patients were treated for cure with radiation therapy alone (25), surgery alone (1), or surgery and preoperative (6) or postoperative (5) radiation therapy. This study presents the results of treatment and the incidence of treatment-related complications in this group of patients.     

Alcohol consumption and impaired glycoregulation results in a population of 6665 salaried employees

Alcohol consumption and glycosuria were found to be associated (p < 0.001) in a population of 6571 salaried employees who underwent a systematic examination. The prevalence of glycosuria was found to range from 1.3% among 2609 non-drinkers to 5% among 816 heavy drinkers (six glasses or more of alcoholic beverage daily). This association was still significant after adjustement for age, sex and body mass index. Similarly, a positive association was observed between fasting glycemia and alcoholic intake in a subgroup of 998 subjects when such a result was available (p < 0.05).     

Methylmercury poisoning: Long-term clinical,radiological, toxicological,and pathological studies of an affected family

For 3 months in 1969 a family in the United States that included a pregnant mother consumed pork containing methylmercury. Children, aged 20, 13, and 8 years and a neonate, developed severe neurological signs. Twenty-two years later, the 2 oldest had cortical blindness or constricted visual fields, diminished hand proprioception, choreoathetosis, and atentional deficits. Magnetic resonance images showed tissue loss in the calcarine and parietal cortices and cerebellar folia. The youngest had quadriplegia, blindness, and severe mental retradation until their deaths. The brain of the 8-year-old who died at age 30 showed cortical atrophy, neuronal loss, and gliosis, most pronounced in the paracentral and parietooccipital regions. The total mercury level in formalin-fixed, left occipital cortex was 1,974 ng/gm as measured by atomic absorption. Regional brain mercury levels correlated with extent of brain damage. A control patient had 38.5 ng of mercury/gm in the occipital cortex. Systemic organs in the patient and a control subject had comparable mercury levels. In mercury-intoxicated rats, we found that only 5 to 10% of total brain mercury was lost by formalin fixation. Brain inorganic mercury in the patient ranged from 82 to 100%. Since inorganic mercury crosses the blood-brain barrier poorly, biotransformation of methyl to inorganic mercury may have occurred after methylmercury crossed the blood-brain barrier, accounting for its persistence in brain and causing part of the brain damage.