Bullous Variant of Sweet's Syndrome after Herpes Zoster Virus Infection

Aim: Cutaneous manifestations of Sweet’s syndrome (SS) are typically painful plaque-forming erythematous papules, while bullae are quite uncommon. We present a case of bullous variant of SS in acute myeloid leukaemia. In this case, herpes infection of the left mandible had preceded the development of SS. Case Report: A 75-year-old male with myelodysplastic syndrome first presented with herpes zoster virus infection-like bullae and erosive plaques on the left side of the face and neck. Treatment with valacyclovir and antibiotics was effective only for the initial lesions, whereas the other bullae kept developing predominantly on the left side. Histopathological study revealed epidermal bulla formation, pandermal neutrophilic infiltration, erythrocyte extravasation and subepidermal oedema, but no vasculitis. The findings suggested the diagnosis of bullous variant of SS. Discussion: Our case was unique in that bullous SS symptoms developed predominantly on one side of the cheek and neck where the herpes zoster infection occurred prior to SS. The tendency may explain the possible association between viral infection and development of SS

Bronchogenic cyst of the interatrial septum

Although bronchogenic cysts are the most common primary mediastinal cysts, intracardiac bronchogenic cysts are extremely rare. We report a case of a bronchogenic cyst of the interatrial septum in a 42-year-old woman who presented with recent onset of dyspnea on exertion. Cardiac investigations including transthoracic echocardiography and computed tomography revealed a cystic homogeneous mass in the interatrial septum. The patient underwent surgical resection, and the resultant atrial septal defect was repaired using an autologous pericardial patch. Histopathological examination of the resected specimen revealed findings consistent with a benign bronchogenic cyst. Although bronchogenic cysts are extremely rare, they should be considered in the differential diagnoses of intracardiac tumors. Complete resection of bronchogenic cysts is recommended primarily for diagnostic and potentially therapeutic purposes

Two-Directional Arthrographic Assessment for Treating Bilateral Development Dislocation of the Hips in Children after Walking Age

We reviewed the treatment outcome in 14 hips of 7 patients who were diagnosed as having bilateral developmental dislocation of the hip (DDH) after walking age and could be followed up until they were at least 14 years of age. Based on the results of two-directional arthrography of the hip, closed reduction was performed in 2 hips, and open reduction was performed without osteotomy in 12 hips. The final radiographic evaluations were made according to the Kalamchi and MacEwen classification and Severin classification. The mean age at the initial visit was 1 year and 9 months (range, 1 year and 5 months to 3 years). The outcome was satisfactory for one hip in Group Ⅰ and 2 hips in Group Ⅱ according to the Kalamchi and MacEwen classification, and in 83% of the Severin Class Ⅰ and Ⅱ hips. Arthrography was useful for identifying asymmetry, demonstrating the usefulness of a treatment strategy based on arthrography of the hip

Myeloperoxidase-Antineutrophil Cytoplasmic Antibody-Negative Microscopic Polyangiitis with Pulmonary Haemorrhage and IgA Nephropathy

Aim: To report a case of a patient with myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA)-negative microscopic polyangiitis (MPA) and IgA nephropathy associated with severe pulmonary haemorrhage. Case Report: A 59-year-old man presented with ANCA-negative systemic vasculitis accompanied by purpura, nephritis and pulmonary haemorrhage. A skin biopsy specimen revealed pandermal leucocytoclastic vasculitis without IgA deposition and a kidney biopsy showed mesangial nephritis with IgA deposition. Considering these findings, the patient was diagnosed as having MPA with IgA nephropathy. Discussion: In most cases, MPA presents with rapidly progressive necrotizing glomerulonephritis and sometimes lung haemorrhage, while IgA nephropathy is less common among MPA cases. As recent research suggested that in MPA immunoglobulin deposition in the kidney may be an exacerbating factor for renal dysfunction and poor prognosis, close observation is required in these cases

Venous Thromboembolism after Total Hip Arthroplasty Diagnosed by Enhanced Computed Tomography : Comparison of Selective Thromboprophylaxis and No Thromboprophylaxis

Total hip arthroplasty (THA) is the most effective treatment for advanced or end-stage hip osteoarthritis. However, venous thromboembolism (VTE) remains one of its unresolved complications. We reviewed the records of 322 patients undergoing primary THA and investigated the efficacy of anticoagulant prophylaxis for VTE. Our study cohort consisted of 60 patients who received no anticoagulants, 100 patients who received a factor Xa inhibitor (fondaparinux), 100 patients who received low molecular weight heparin (enoxaparin), and 62 patients who selectively received no anticoagulant prophylaxis due to perioperative bleeding, weight, and/or hemoglobin concentration. Enhanced 64-slice multidetector row computed tomography was performed postoperatively for 7 days in all cases. The incidence of VTE in the four groups was 15ｵ, 9.0ｵ, 6.0ｵ, and 6.4ｵ, respectively. The incidence of VTE was significantly lower in the groups receiving anticoagulant prophylaxis and the group selectively receiving no anticoagulant prophylaxis than in the group receiving no anticoagulants. Complications of fondaparinux therapy included hepatic dysfunction in 4 cases (4.0ｵ), minor bleeding in 2 cases (2.0ｵ), persistent wound drainage in 3 cases (3.0ｵ), and eruption in 1 case (1.0ｵ). The complications of enoxaparin therapy were persistent wound drainage in 1 case (1.0ｵ) and progression of anemia in 1 case (1.0ｵ). The incidence of VTE was low in patients who selectively received no anticoagulant prophylaxis, so we conclude that anticoagulant prophylaxis should be used selectively in THA cases

For Vol.69, No.4, pp205-212 Venous Thromboembolism after Total Hip Arthroplasty Diagnosed by Enhanced Computed Tomography : Comparison of Selective Thromboprophylaxis and No Thromboprophylaxis

Total hip arthroplasty (THA) is the most effective treatment for advanced or end-stage hip osteoarthritis. However, venous thromboembolism (VTE) remains one of its unresolved complications. We reviewed the records of 322 patients undergoing primary THA and investigated the efficacy of anticoagulant prophylaxis for VTE. Our study cohort consisted of 60 patients who received no anticoagulants, 100 patients who received a factor Xa inhibitor (fondaparinux), 100 patients who received low molecular weight heparin (enoxaparin), and 62 patients who selectively received no anticoagulant prophylaxis due to perioperative bleeding, weight, and/or hemoglobin concentration. Enhanced 64-slice multidetector row computed tomography was performed postoperatively for 7 days in all cases. The incidence of VTE in the four groups was 15%, 9.0%, 6.0%, and 6.4%, respectively. The incidence of VTE was significantly lower in the groups receiving anticoagulant prophylaxis and the group selectively receiving no anticoagulant prophylaxis than in the group receiving no anticoagulants. Complications of fondaparinux therapy included hepatic dysfunction in 4 cases (4.0%), minor bleeding in 2 cases (2.0%), persistent wound drainage in 3 cases (3.0%), and eruption in 1 case (1.0%). The complications of enoxaparin therapy were persistent wound drainage in 1 case (1.0%) and progression of anemia in 1 case (1.0%). The incidence of VTE was low in patients who selectively received no anticoagulant prophylaxis, so we conclude that anticoagulant prophylaxis should be used selectively in THA cases

Cerebral hemorrhage in Fabry's disease

Fabry's disease is an X-linked lysosomal storage disorder resulting from alpha-galactosidase A deficiency. Although ischemic stroke is recognized as an important manifestation of Fabry's disease, hemorrhagic stroke is considered to be rare. Here, we report our recent clinical experience with three hemizygous male patients with Fabry's disease who developed cerebral hemorrhage. One patient had classic type Fabry's disease with p.Ala37Val mutation and others had cerebrovascular variant with p.Glu66Gln mutation. Degeneration of the cerebral small arteries secondary to deposition of glycosphingolipids and aging, in addition to hypertension and antiplatelet/anticoagulant agents, are considered to be contributing factors for hemorrhage. Fabry's disease is frequently associated with not only ischemic but also hemorrhagic stroke, especially in elderly patients. Journal of Human Genetics ( 2010) 55, 259-261; doi:10.1038/jhg.2010.18; published online 19 March 2010ArticleJOURNAL OF HUMAN GENETICS. 55(4):259-261 (2010)journal articl

HBD1 protein with a tandem repeat of two HMG-box domains is a DNA clip to organize chloroplast nucleoids in Chlamydomonas reinhardtii

葉緑体核様体をコンパクトに折りたたむ「DNAクリップ」の発見 --ミトコンドリアとも共通する普遍的なしくみの解明--. 京都大学プレスリリース. 2021-05-12.Compaction of bulky DNA is a universal issue for all DNA-based life forms. Chloroplast nucleoids (chloroplast DNA–protein complexes) are critical for chloroplast DNA maintenance and transcription, thereby supporting photosynthesis, but their detailed structure remains enigmatic. Our proteomic analysis of chloroplast nucleoids of the green alga Chlamydomonas reinhardtii identified a protein (HBD1) with a tandem repeat of two DNA-binding high mobility group box (HMG-box) domains, which is structurally similar to major mitochondrial nucleoid proteins transcription factor A, mitochondrial (TFAM), and ARS binding factor 2 protein (Abf2p). Disruption of the HBD1 gene by CRISPR-Cas9–mediated genome editing resulted in the scattering of chloroplast nucleoids. This phenotype was complemented when intact HBD1 was reintroduced, whereas a truncated HBD1 with a single HMG-box domain failed to complement the phenotype. Furthermore, ectopic expression of HBD1 in the mitochondria of yeast Δabf2 mutant successfully complemented the defects, suggesting functional similarity between HBD1 and Abf2p. Furthermore, in vitro assays of HBD1, including the electrophoretic mobility shift assay and DNA origami/atomic force microscopy, showed that HBD1 is capable of introducing U-turns and cross-strand bridges, indicating that proteins with two HMG-box domains would function as DNA clips to compact DNA in both chloroplast and mitochondrial nucleoids