Panniculitis in a patient with mixed connective tissue disease

A 51-year-old woman who had been suffering from mixed connective tissue disease (MCTD) for 8 years developed an erythematous rash with pain and tenderness on her left leg. A skin biopsy revealed septal panniculitis with multiple lymphangiectasis. No vasculitis was observed. An increase in her prednisolone dose from 5mg to 20mg/day led to an improvement in these lesions. Panniculitis is very rare in MCTD. The clinical significance of panniculitis in MCTD is also discussed.     

Usefulness of a selective neutrophil elastase inhibitor (sivelestat) in septic ARDS patients after gastrointestinal surgery

BACKGROUND/AIMS: The usefulness of a neutrophil elastase inhibitor (sivelestat) was evaluated for treating septic acute respiratory distress syndrome (ARDS) after gastrointestinal surgery. METHODOLOGY: The subjects were 36 patients with septic ARDS after gastrointestinal surgery. ARDS was defined as acute lung injury associated with systemic inflammatory response syndrome. Sivelestat was intravenously administered at a dose of 0.2 mg/kg/hr continuously for 3 days or more. The effectiveness of sivelestat was evaluated based on the lung injury score, P/F ratio, and ventilator free days (VFD). RESULTS: Marked responses were observed in 12 patients (33.3%), responses in 17 (47.2%), and no response in 7 (19.4%). In the patients with marked responses or responses (responders), the P/F ratio was significantly improved on day 3 of drug administration and at the end of administration compared with the pre-administration ratio. Comparison between the responders and non-responders showed significant differences on day 3 and at the end of drug administration. VFD significantly differed between the responders (18.8 days) and the non-responders (11.0 days). CONCLUSION: In conclusion, sivelestat may be effective against septic ARDS. The effectiveness of the drug could be determined based on improvement in oxygenation ability on day 3 of drug administration.     

Appropriate cut-off value of 13C-urea breath test after eradication of Helicobacter pylori infection in Japan

BACKGROUND AND AIM: A cut-off value of 2.5 per thousand for the 13C-urea breath test (UBT) is recommended in Japanese persons, based on the result of a multicenter trial in patients prior to treatment for eradication of Helicobacter pylori. The cut-off value of 2.5 per thousand has also been used in the assessment of eradication after treatment. The 6-8-week evaluation after treatment is recommended in the guidelines of the Japanese Society of Gastroenterology. The present study aimed to prospectively re-assess the cut-off value of the 13C-UBT at 6 weeks after treatment by using the results obtained at 6 months as an indication of true positive or true negative H. pylori infection status. METHODS: One hundred and ninety patients who were positive for H. pylori underwent eradication treatment, and 177 patients of these patients who were assessed as having true positive or true negative H. pylori status at 6 months after treatment were evaluated in this study. Eradication was assessed by 13C-UBT, culture, and histology at 6 weeks and at 6 months after treatment, and the cut-off value of 13C-UBT at 6 weeks was re-assessed. RESULTS: A cut-off value of 3.5 per thousand. at 6 weeks after treatment showed 97.2% diagnostic accuracy, while a cut-off value of 2.5 per thousand at 6 weeks showed 96.0% diagnostic accuracy. For a 3.5 per thousand cut-off value, only five patients were positive by 13C-UBT and were negative by culture and histology at 6 weeks, and three patients were true positive and two were false positive by the 13C-UBT at 6 months. CONCLUSION: A cut-off value of 3.5 per thousand for the 13C-UBT is recommended at 6 weeks after eradication treatment in Japanese persons.     

Development of severe aplastic anemia in a girl with Hashimoto's thyroiditis and papillary thyroid carcinoma

A 14-year-old girl was admitted because of general fatigue and cervical lymphadenopathy. She showed bilateral struma (IInd degree) and enlargement of her left cervical lymph nodes. Laboratory data revealed neutropenia (219/microliter) and thrombocytopenia (Plt 5.1 x 10(4)/microliter) with mild anemia (Hb 11.1 g/dl), and the bone marrow aspirate and biopsy specimens showed hypocellularity. In addition, auto-antibodies against thyroid peroxidase (TPO) and thyroglobulin (TG) were highly elevated. Computed tomography of the neck showed a nodule in the left thyroid lobe with marked lymphadenopathy, and fine needle aspiration biopsy demonstrated papillary thyroid carcinoma with Hashimoto's thyroiditis and metastasis to the lymph nodes. One month after left thyroid lobectomy and cervical lymphadenectomy, the patient's condition progressed to very severe aplastic anemia, and she received immunosuppressive therapy consisting of cyclosporin A and anti-thymocyte globulin. Hematologically, partial and complete responses were obtained three and six months later, respectively. Of interest, anti-TPO and TG antibody titers remarkably decreased after immunosuppressive therapy. The patient had HLA-DR 2(DRB 1*1501) and DR 8(DRB 1*0802). The former is frequently found in patients with cyclosporin A-dependent aplastic anemia, and the latter is frequently found in Asian patients with Hashimoto's thyroiditis, suggesting an underlying autoimmune background for the simultaneous outbreak of aplastic anemia and Hashimoto's thyroiditis complicated by thyroid carcinoma.     

Condylar surface CT value in sagittal plane before and after sagittal split ramus osteotomy

Purpose

The purpose of this study was to evaluate the relationship between computed tomography (CT) values of the condylar surface and temporomandibular joint (TMJ) disc position in the sagittal plane before and after sagittal split ramus osteotomy (SSRO) setback surgery, retrospectively.

Materials and methods

The subjects were 75 patients (150 condyles) who underwent bilateral SSRO setback surgery. They were divided into two groups (42 symmetric patients and 33 asymmetric patients). Maximum CT values (pixel values) of five points of the condylar surface and condylar height, length, fossa height, fossa length, and ramus angle in the sagittal plane were measured preoperatively and 1 year postoperatively. Disc position was classified as anterior disc displacement, anterior type, fully covered type, and posterior type, both pre- and postoperatively, using magnetic resonance imaging (MRI).

Results

Postoperative value was significantly higher than preoperative one in CT value of 135° (P = 0.0199) and 180° (0.0363), in the non-deviation side in the asymmetry group. The anterior disc displacement group was significantly larger than those of some other areas pre- and postoperatively in the CT value of 0° point (P < 0.05).

Conclusions

This study suggested that CT value of the posterior site of the condylar surface could change in the non-deviation side in the asymmetry group after 1 year SSRO, and the condyle with anterior displacement showed high CT value at the anterior site of the condyle before and after surgery.

     

Regression of retinal capillaries following N‐methyl‐D‐aspartate‐induced neurotoxicity in the neonatal rat retina

Degeneration of retinal capillaries occurs following N‐methyl‐D ‐aspartate (NMDA)‐induced retinal neurotoxicity, and the degree of capillary degeneration decreases in an age‐dependent manner. To determine the role of vascular endothelial growth factor (VEGF) in the high susceptibility of capillaries to neuronal damage during the early postnatal stage, this study compares the vascular regression patterns between NMDA‐treated retinas and retinas treated with N‐[2‐chloro‐4‐{(6,7‐dimethoxy‐4‐quinazolinyl)oxy}phenyl]‐N′‐propylurea (KRN633), a VEGF receptor tyrosine kinase inhibitor, in neonatal rats. Two days after a single intravitreal injection of NMDA (200 nmol/eye) on postnatal day (P) 7, substantial retinal neuron loss and delayed expansion of the retinal vascular bed were observed. The reduction in the capillary density in the central retina reached statistical significance 4 days after NMDA treatment. In retinas of rats injected subcutaneously with KRN633 (10 mg/kg) on P7 and P8, simplified vasculature attributable to capillary regression and prevention of endothelial cell growth were seen on P9, whereas no visible changes in the morphology of the retinal layers were observed. The degree of capillary degeneration in NMDA‐treated retinas was less than that in KRN633‐treated retinas. No apparent changes in immunoreactivities for VEGF were found 2 days after NMDA treatment. These results indicate that neuronal cell loss in the retina precedes retinal capillary degeneration following NMDA treatment, and VEGF‐dependent immature capillaries might be more susceptible to NMDA‐induced neuronal damage. © 2014 Wiley Periodicals, Inc.     

Branchial Cleft-Like Cysts Involving 3 Different Organs: Thyroid Gland,Thymus, and Parotid Gland

Branchial cleft cysts (BCCs) are also named lateral cervical cysts and widely acknowledged as being derived from embryonic remnants. Lymphoepithelial cysts (LECs) generally show microscopic features that are identical to those of BCCs, and rarely occur at unusual sites or organs.A case of multiple cysts arising in both lobes of the thyroid gland, thymus, and right parotid gland in a 41-year-old man is reported. Clinically, the patient presented with Hashimoto''s thyroiditis for about 20 years and had past histories of idiopathic thrombocytopenic purpura and severe respiratory infection.This case is unusual in that multiple cysts arose synchronously and/or heterochronously and grew, increasing their sizes in these different organs. Microscopic examinations revealed that all of the cysts were composed of squamous epithelium, dense lymphoid tissue with germinal centers, and a fibrous capsule. These findings corresponded to those of BCCs or LECs. It is notable that the histopathological features were nearly the same in the individual organs. A review of the literature disclosed no previous such reported cases.The etiology is unknown. However, based upon the similar histopathological features of all the excised specimens, common immune and/or hematopoietic disorders may have contributed to their occurrence and development in association with putative genetic abnormalities.