Pyoderma gangrenosum, acne, and suppurative hidradenitis syndrome in end-stage renal disease successfully treated with adalimumab.

PASH syndrome (pyoderma gangrenosum, acne, and suppurative hidradenitis) forms part of the spectrum of autoinflammatory diseases. We report an unusual case of PASH syndrome in a patient with end-stagerenal disease (ESRD) who was successfully treated with the tumor necrosis factor inhibitor, adalimumab. The case underscores the challenges associatedwith the treatment of PASH syndrome as well as the ongoing search to establish a genetic basis for the syndrome. Renal impairment has been reported in association with pyoderma gangrenosum but has notbeen described in PASH syndrome. We believe this to be the first reported case of a patient who developed PASH syndrome in the setting of ESRD.

STRENGTHENING INFRASTRUCTURE FOR STRENGTHENING THE HEALTHCARE / Шинэ санаа Шинэ нээлт

Монгол улсад хоёрдогч буюу өмнө нь өөр улсад ашиглагдаж байсан, мөн олон жил ашигласан эмнэлгийн тоног төхөөрөмжүүд одоог хүртэл хэрэглэгдсээр байна. ЭМСЯ-ны төсөл хөтөлбөрүүд, Азийн Хөгжлийн Банк, ДЭМБ-ын Монгол дахь яаралтай үеийн тусламж үйлчилгээ, эмнэлгийн эрүүл ахуйн төсөл зэрэг нь орчин үеийн эрүүл мэндийн технологийг нэвтрүүлэх, эрүүл мэндийн салбарт тулгамдаж буй асуудлуудыгшийдвэрлэхийг зорьж байгаа билээ. Гэсэн хэдий ч орчин үеийн тоног төхөөрөмжүүдийг тухайлбал компьютерт томографи, соронзон резонанст томографийг ашиглах туршлагадутуу байх, үйл ажиллагааны горимыг зөрчих, тоног төхөөрөмжийн эвдрэл гэмтлүүд Улаанбаатар хот, аймгуудад түгээмэл байна. Эмнэлгийн тоног төхөөрөмжийн 20 жилийн хөгжлийн чиг хандлагаас харахад орчны болон дэд бүтцийн өөрчлөлтөнд тэсвэртэй программ хангамжтай,туйлын мэдрэг чанар өндөртэй, амархан гэмтдэг эд анги, бүрэлдэхүүн хэсгээс тогтсон эмнэлгийн тоног төхөөрөмжүүд ихээхэн ашиглагдаж байна. Эмнэлгийн технологийн гэмтлийг илрүүлж чадахгүй байх, ашиглалтын горимын шаардлагыг хангаж ажиллахгүй байх зэрэг нь орчин үеийн эмнэлгийн тоног төхөөрмжийн хөрөнгө оруулалт нь мөнгө хөрөнгө дэмий үрэгдсэн ажил болгон хувиргаж байна. Цахилгаан хангамж, усны шугамын найдвартай хэмжилт нь эмнэлгийн тоног төхөөрөмжийн найдвартай, аюулгүй ажиллагаа, чанарыг дээшлүүлэх болон дэд бүтцийг тогтворжуулах гол хүчин зүйл юм. Өндөр хүчдэл, гүйдлээс шалтгаалсан тоног төхөөрөмжийн гэмтлээс гадна үйл ажиллагаа, аюулгүй байдлаас хамаарсангэмтлүүдийг засварлах асуудал тулгарч байна. Чанарын болон эрсдэлийн удирдлага нь тоног төхөөрөмжийн үйл ажиллагаа, тохиргоог хангах учир эрүүл мэндийн үйлчилгээг бэхжүүлж чадна.

Toxic epidermal necrolysis and Stevens-Johnson syndrome in South Africa: a 3-year prospective study.

BACKGROUND: Toxic Epidermal Necrolysis (TEN) and Stevens-Johnson syndrome (SJS) remain feared medication-related reactions. HIV infection and tuberculosis predispose to drug eruptions, yet there is a paucity of data on TEN/SJS in populations with high prevalences of both diseases. AIM: The aim of this prospective observational study was to describe the features and outcomes of patients admitted with TEN/SJS at a large academic hospital in South Africa. We aimed to identify poor prognostic indicators and to validate the use of the TEN-specific severity-of-illness score (SCORTEN) in this population. METHODS: All patients admitted with TEN/SJS over a 3-year period were enrolled. Disease severity was graded according to percentage skin involved and SCORTEN. Co-morbid diagnoses, clinical features, investigations, complications and outcomes were noted. RESULTS: 75 patients (39.9 ± 10.6 years, 16 males, 59 HIV positive) were classified as TEN (n = 42), TEN/SJS overlap (n = 11) and SJS (n = 22). Twenty-four percent died, most from refractory septic shock. Non-survivors had a higher mean SCORTEN on Days 1 and 3 (1.89 vs. 1.04, P = 0.006 and 2.27 vs. 0.90, P < 0.001). A SCORTEN ≥2 on Days 1 and 3 predicted non-survival (OR = 2.94, P = 0.047; OR = 7.45, P < 0.001). Other predictors of non-survival included HIV infection (OR = 6.01, P = 0.058), HIV-tuberculosis co-infection (OR = 8.5, P < 0.001), ≥40% skin involvement (OR = 20.27, P < 0.001), anaemia (OR = 4.68, P = 0.005), hypoalbuminemia (OR = 8.5, P = 0.001) and severe sepsis (OR = 71.09, P < 0.001). CONCLUSION: Most patients with TEN/SJS were HIV positive and female. We validated the use of SCORTEN and identified several prognostic indicators, most significant being HIV-tuberculosis co-infection, ≥40% skin involvement and severe sepsis.

Computer-assisted pre- and postoperative evaluation of surgically assisted rapid maxillary expansion.

OBJECTIVE: Computer-assisted methods were used to evaluate different variants of surgically assisted rapid maxillary expansion (SARME) in terms of bone repositioning, new bone formation in the osteotomy gap, and bone quality before and after surgery. MATERIALS AND METHODS: Twenty-nine patients (18 male, 11 female) with a mean age of 29 years (16 to 44 years) were included in the study. Surgically assisted rapid maxillary expansion with Le Fort I osteotomy was performed in all patients studied. High-resolution computed tomography (CT) was carried out directly before and 6 to 8 weeks after surgery. After registration of the preoperative CT data on the postoperative data, 3D models were constructed and superimposed. New bone formation in the osteotomy gap was visualized by means of a visualization procedure developed specifically for this purpose. Bone quality was analyzed by dividing the models into different anatomical segments. A qualitative comparison of the data was accomplished using a direct volume rendering procedure with a special transfer function. A quantitative comparison was carried out based on the pre- and postoperative histograms of each region. RESULTS: Maxillary widening was confirmed in all patients by computer-assisted analysis. Four patients exhibited significant maxillary asymmetry after surgery. New bone formation within the osteotomy gap was irregular along the osteotomy lines but often symmetrical on both sides. The more symmetrical the osteotomy, the more symmetrical the new bone formation proved to be. In all but two cases, the postoperative qualitative and quantitative analyses showed a significant decrease in Hounsfield units, particularly in the vestibular bone. CONCLUSION: The differences in new bone formation in the osteotomy gap suggest that the type of surgical technique and distractor used influence the outcome. Our results indicate that SAME results in a decrease in bone quality, particularly in the vestibular bone. Computer-assisted analysis clearly results in an information gain.