Necrotizing fasciitis following rapidly deteriorating neonatal omphalitis with good initial presentation.

Neonatal omphalitis is a postpartum infection of periumbilical superficial soft tissues that usually has a good prognosis in developed countries. In rare cases, it could progress to periumbilical necrotizing fasciitis (NF), which is an infection of the deep soft tissues, including muscle fascia, and has a high mortality rate. However, the signs and timing of developing NF secondary to omphalitis are unclear. We encountered a neonatal case of NF following omphalitis. In the initial days of the clinical course, general symptoms and condition of the patient were good, and abdominal physical findings were mild; however, the patient rapidly developed NF. The patient was successfully treated by emergent surgical debridement, broad-spectrum antibiotics, and intensive care. To determine the area of blood perfusion, we intravenously injected indocyanine green by intraoperative angiography, and then extensively removed necrotic and hypoperfused tissues. In neonatal omphalitis, the deterioration can suddenly occur despite good initial conditions; intensive monitoring should be required during the first few days of the clinical course.

Diaminodiphenyl Sulfone-Induced Hemolytic Anemia and Alopecia in a Case of Linear IgA Bullous Dermatosis.

Linear immunoglobulin A (IgA) bullous dermatosis (LABD) is an autoimmune mucocutaneous disease characterized by subepidermal blistering induced by IgA autoantibodies against several autoantigens in the basal membranous zone of the skin and mucosal tissue. Although diaminodiphenyl sulfone (DDS), also known as dapsone, is generally recognized as the first-line therapy for LABD, DDS can induce several severe side effects. We present a Japanese case of LABD with DDS-induced hemolytic anemia and alopecia. In the present case, the DDS-induced hemolytic anemia and hair loss made the DDS monotherapy difficult. When DDS is used in LABD patients with iron deficiency anemia (IDA), hemolytic anemia is concealed by IDA. It is thus necessary to carefully and frequently examine the laboratory data to find the signs of DDS-induced hemolytic anemia. Even though there is no literature on DDS-induced alopecia, alopecia was reported as one of the side effects of DDS in an FDA report, and, in our case, hair loss was improved after reducing its dosage. We have to recognize that alopecia is one of the side effects of DDS and that careful management is needed in order not to overlook the adverse side effects of DDS when treating LABD patients.

Spectra of functional gastrointestinal disorders diagnosed by Rome III integrative questionnaire in a Japanese outpatient office and the impact of overlapping.

BACKGROUND AND AIMS: The aim of this study was to establish the spectra of functional gastrointestinal disorders (FGID) in a Japanese outpatient office in Rome III. METHODS: The Rome III Diagnostic Questionnaire for Adult Functional GI Disorders was translated into Japanese and an automated analyzing program was made according to the scoring algorithm of the questionnaire. Among 1378 patients who visited the outpatient office of the Social Insurance Shiga Hospital between May 2007 and April 2009, 112 serial patients who had symptoms possibly originating from the gastrointestinal (GI) tract, but did not have evidence of organic disease, were recruited. The subjects answered the questionnaire, and the answers were analyzed with the automatic analyzer. RESULTS: During the study period, 94 of the 112 patients were diagnosed as having active FGID. Non-overlapping FGID was diagnosed in 41 (43.6%) of those. Of the 41 non-overlapping FGID patients, the most frequent diagnosis was irritable bowel syndrome (IBS) in 13 patients. Including overlapping cases, 165 FGID were diagnosed in 94 patients. The most frequent diagnosis was IBS in 33 patients (35.1%), the second was functional dyspepsia (FD) in 29 (30.9%) and the third was functional constipation in 21 (22.3%). The most frequent FGID overlapping with IBS was FD (36.4%), and the most frequent FGID overlapping with FD was IBS (41.4%). Of the 29 FD patients, 20 (69.0%) had functional bowel disorders. CONCLUSION: The most frequent FGID was IBS in both overlapping and non-overlapping FGID patients. IBS and FD were the most frequent combinations in overlapping FGID. Most cases of FD are possibly parts of functional bowel disorders.

Short-term effects of oral appliances with equal bite-raising distance but with varying protrusions on occlusal force, contact area and load center.

The purpose of this study was to demonstrate how short-term wearing of an oral appliance (OA) with equal bite-raising distance but with varying protrusions affects occlusal force, contact area and load center. Twelve young healthy volunteers participated. With the appliance, the mandible was protruded 0%, 45%, 60% and 75% of maximum protrusion capacity, with 10 mm bite-raising between the first molars. The occlusal force, contact area and load center at maximum voluntary clenching were measured before wearing the OA, at 1 h, 3 h and 6 h during wearing, and 1 h after removal. When compared to the values before wearing the OA, occlusal force was significantly lower at 1 h, 3 h and 6 h during wearing in the case of no mandibular protrusion, and at 3 h and 6 h after for 45%, 60% and 75% of maximum mandibular protrusion (P < 0.05). Occlusal contact area was significantly smaller at 1 h and 6 h during wearing in the case of no protrusion, and at 6 h during wearing in the case of 45% of maximum protrusion (P < 0.05). There was a tendency for anterior shift in the location of the occlusal load center at 3 h and 6 h during wearing of the OA with any level of maximum protrusion. No significant change in these three measurements was found at 1 h after removal of the OA. The present study demonstrated that wearing an OA had only a marginal and transient influence on oral functions when their changes were compared before and after wearing the OA.

Epistatic connections between microphthalmia-associated transcription factor and endothelin signaling in Waardenburg syndrome and other pigmentary disorders.

Waardenburg syndrome (WS) is an inherited sensorineural deafness condition in humans caused by melanocyte deficiencies in the inner ear and forelock. Mutation of microphthalmia-associated transcription factor (MITF) is known to produce WS type IIA whereas mutations of either endothelin (EDN) or its receptor endothelin receptor B (EDNRB) produce WS type IV. However, a link between MITF haploinsufficiency and EDN signaling has not yet been established. Here we demonstrate mechanistic connections between EDN and MITF and their functional importance in melanocytes. Addition of EDN to cultured human melanocytes stimulated the phosphorylation of MITF in an EDNRB-dependent manner, which was completely abolished by mitogen-activated protein kinase kinase inhibition. The expression of melanocyte-specific MITF mRNA transcripts was markedly augmented after incubation with EDN1 and was followed by increased expression of MITF protein. Up-regulated expression of MITF was found to be mediated via both the mitogen-activated protein kinase-p90 ribosomal S6 kinase-cAMP response element-binding protein (CREB) and cAMP-protein kinase A-CREB pathways. In addition, EDNRB expression itself was seen to be dependent on MITF. The functional importance of these connections is illustrated by the ability of EDN to stimulate expression of melanocytic pigmentation and proliferation markers in an MITF-dependent fashion. Collectively these data provide mechanistic and epistatic links between MITF and EDN/EDNRB, critical melanocytic survival factors and WS genes.