Quantitative evaluation of maxillary bone deformation by computed tomography in patients with leprosy.

BACKGROUND: Facial deformation as a sequela of leprosy is caused not only by a saddle nose but also by regression of the maxilla, as well documented in paleopathological observations of excavated skeletal remains of patients with leprosy. However, maxillary changes in living patients have been evaluated only by the subjective visual grading. Here, we attempted to evaluate maxillary bone deformation in patients with leprosy using three-dimensional computed tomography (3D-CT). METHODS: Three-dimensional images centered on the maxilla were reconstructed using multiplanar reconstruction methods in former patients with leprosy (n = 10) and control subjects (n = 5); the anterior-posterior length of the maxilla (MA-P) was then measured. The difference between the MA-P of the patients and those of controls was evaluated after compensating for individual skull size. These findings were also compared with those from previous paleopathological studies. FINDINGS: Three former patients with lepromatous leprosy showed marked atrophy of the maxilla at the prosthion (-8.6, -11.1 and -17.9 mm) which corresponded with the visual appearance of the maxillary deformity, and these results were consistent with paleopathological findings of excavated skeletal remains. Additionally, the precise bone defects of the maxilla could be individually calculated for accurate reconstructive surgery. INTERPRETATION: We have successfully illustrated maxillary bone deformities in living patients with leprosy. This study also confirmed the maxillary regression described in paleopathological studies.

[Effectiveness of thalidomide for erythema nodosum leprosum (ENL): retrospective study of 20 Japanese cases in National Sanatrium Oku-Komyoen].

Thalidomide is a TNF-alpha inhibitor and has been administrated for erythema nodosum leprosum (ENL, Type II leprosy reaction) which is one of leprosy reactions and can cause serious illness to patients oflepromatous pole among the immune spectrum. Twenty live cases (at May, 2011) were identified to whom thalidomide had been administrated since 1978 for their ENL reactions. Data were collected from their clinical records in order to evaluate the usage and effectiveness of thalidomide in National Sanatorium Oku-Komyoen, Okayama, Setouchi-city, Japan. Individual data includes bacillary index (BI), total dose, average daily dose, maximum daily dose, minimum daily dose, methods of thalidomide administration and change of symptoms of ENL. Results: No adverse effect was found among 20 cases. Average daily dose of 20 cases was 19 mg. Regarding to the maximum daily dose, in 3 cases (15%) more than 100 mg, in 3 cases (15%) 50 mg, and in 14 cases (70%) less than 40 mg was administrated. Dose was gradually tapered in most cases. From clinical records, thalidomide was found effective for ENL in 19 cases and clinicians concerned were trying to adjust the proper dose of the drug carefully depending on the current symptoms, because there was no guideline of thalidomide administration for ENL. This data suggests that even less than 50-100 mg as the initial daily dose was still effective, though 50-100 mg daily dose is recommended in the current guideline of Japan (2011) and more dose had been administrated in USA and India.

[Hansen's disease-associated disabilities in sanatoria residents. A study of the actual situation in three sanatoria located in the Setouchi area].

PURPOSE: Clarification of details of Hansen's disease in Japan is important for obtaining an adequate understanding of the present circumstances regarding control and for formulation of specific suggestions for future measures. The average age of residents in national Hansen's disease sanatoria reached 74.9 in May 2002 and most of these suffer from serious disabilities caused by the disease. In order to implement measures facilitating their return to society or secure living in sanatoria, a proper understanding of the residents' physical situation is essential. Therefore, the present study was conducted of residents suffering from Hansen's disease in Japan. METHOD: A direct interview was conducted with all 1282 residents of three sanatoria, located in the Setouchi area: Nagashima-aiseien, Oku-komyoen, and Oshima-seishoen. An investigator visited the rooms of the residents, interviewed them using a questionnaire, which consisted of about 100 items including the OPCS scale, and filled out an answer form. The reply rate was 64%. RESULTS: The average age of the residents was found to be 72.8 years, their average age when they entered the sanatoria was 26.0 years, and the average period of residence was 52.4 years. The damage they suffer as a result of the isolation policy is physical, mental, economical and social. That is to say, their entire life is harmed as a direct result of the Leprosy Prevention Law or the Eugenic Protection Law with discrimination in society and negative effects on their families. Their physical condition features serious disabilities in the use of fingers, as well as in walking and seeing caused by peripheral nerve damage. Such disabilities are related to their age and period of residence, and in addition, these in use of their fingers are related to the work which residents were forced to do in sanatoria. The results indicate that patients' physical condition is related to their age, how long they have had the disease and their work in sanatoria. CONCLUSION: It is clear that previous Hansen's disease control in Japan has caused a great deal of harm, from which it is very difficult to recover so that residents have to live with serious problems. In order to implement measures allowing their return to society or more secure life at sanatoria, sufficient care is needed to treat progressive disabilities caused by ageing and prevent deterioration caused by peripheral nerve damage.