Background: Kawasaki disease (KD) is an acute febrile illness of unknown etiology. The most common and alarming complication is coronary artery aneurysm. High dose immunoglobulin therapy (IVIG) is effective in resolving inflammation and reducing the incidence of aneurysm. However, 10-20% of KD children have persistent or recurrent fever after IVIG, remaining high risks of aneurysm. We evaluated which factor is most strongly associated with patient unresponsiveness to IVIG. Methods:We retrospectively reviewed clinical records of 53 KD patients who had been treated with IVIG. They were divided into two groups; 39 patients who responded to IVIG at a dose of 1 g/kg or 2 g/kg (responder group), and 14 who failed to respond to escalated doses, requiring additional treatments (non-responder group). We analyzed a total of 16 variables, including demographic, clinical, and laboratory data, as well as three scoring systems (Gunma, Kurume and Tenri scores). To determine how accurately each variable predicts patient unresponsiveness to IVIG, we calculated the standardized distance between the groups（Δ）, the areas under the ROC curve (AUC),and univariate logistic regression statistics (Wald P). The Tenri score was obtained using logistic stepwise selection based on the likelihood. We then divided the responder group into two subgroups, according to whether patients responded to 1 g/kg IVIG or required a further 2 g/kg dose. Each subgroup was analyzed using the same methods. Results: The Gunma model yielded values for Δand AUC, and a Wald P value, similar to those of the Tenri model, and achieving better results than the Kurume score or other variables. Since the Gunma model was developed and validated using different data sets, it appears to be the most reliable predictor of unresponsiveness to IVIG. However, whereas the Tenri model has four variables, the Gunma model has seven, including the duration of illness before treatment, which reflects severity of the disease (confounding variable). Ockham's razor suggests that a better model could be created using fewer variables. In regard to the distinction between the two subgroups of responders, the Tenri model attained statistical significance, but the Gunma model gave a non-significant result. Conclusion: The Gunma score was able to predict patient unresponsiveness to IVIG more accurately than the Kurume score or other variables, giving comparable results to those of the Tenri score. Nevertheless, the ideal prediction model, with an optimal combination of variables, awaits development using larger datasets.

Background: D-dimer has high sensitivity for pulmonary thrombo embolism (PTE), and is as reliable as applying the Wells criteria to rule out PTE, which is a serious disease and sometimes fatal. FDP is another widely used blood marker of fibrin deposition and fibrinolytic activation that is used to rule out PTE. It has been reported that D-dimer is superior to FDP in ruling out PTE, at least outside Japan. However, no published report compares D-dimer and FDP for ruling out PTE using the methods of measurement and cut-off values adopted in Japan, and the frequency of PTE in Japan differs from other countries. We therefore sought to compare and evaluate the sensitivities of FDP and D-dimer for PTE for the first step of constructing Japanese original algorithm for diagnosing PTE. Methods: We analyzed 59 patients who were hospitalized in Tenri hospital and were diagnosed with PTE during January 2005 and December 2010. All 59 patients underwent examination for both FDP and D-dimer. For 52 of the 59 PTE patients we were able to obtain Wells criteria scores. We also analyzed 178 patients who were not diagnosed with PTE and examined both their FDP and D-dimer during January 2011. Our main purpose was to compare the sensitivities of FDP and D-dimer for the 59 PTE patients. We also compared the sensitivities of FDP and D-dimer for PTE patients classified as the low-risk group by Wells criteria, and as the intermediate to high-risk group by Wells criteria. We also drew the ROC curves of FDP and D-dimer for PTE and compared the AUCs of the two ROC curves. Results: In 59 PTE patients, the sensitivities of FDP and D-dimer for PTE were respectively 78% and 95%. In patients classified into the low-risk group, D-dimer has a sensitivity of 100% (9/9 patients), but FDP has a sensitivity of 78% (7/9 patients). In patients classified into the intermediate to high-risk group, the sensitivities of FDP and D-dimer for PTE were respectively 74% and 93%. The AUCs of FDP and D-dimer were respectively 0.688 and 0.745. Conclusion: According to the present study, D-dimer is more sensitive than FDP at ruling out PTE. This study also suggests that D-dimer is more reliable than FDP in ruling out PTE.

A 60-year-old woman underwent cord blood transplantation (CBT) for a second remission of adult T-cell leukemia/lymphoma (ATLL).At about day +80 after CBT, the patient presented with clinical symptoms of thyrotoxicosis. Her level of free thyroxine was >7.7 ng/dL, free triiodothyronine was >32.5 pg/mL, thyroid stimulating hormone was <0.1 μU/mL, and the level of autoantibody against thyroglobulin was 546 IU/ml. A 99m-technetium scintigraphy revealed markedly decreased thyroid uptake, indicating the destruction of thyroid tissue that led to release of preformed hormone into the circulation. The clinical symptoms resolved within　14 days of treatment with propranolol and prednisolone, and levels of thyroid hormones declined rapidly, within 14 days. A combination of toxic and immune-mediate thyroid injuries associated with CBT was apparently involved in the development of thyrotoxicosis. We believe that this is the first report of thyrotoxicosis occurring after CBT for the treatment of ATLL.

We describe a 64-year-old woman who developed metachronous bilateral breast cancer. At 22 months after epirubicin-based chemo-radiotherapy for the second such cancer, she developed acute myeloid leukemia (AML). The leukemic cells were monoblastic and carried a t(9;11)(p22;q23) translocation involving the MLL gene, determined by conventional cytogenetic analysis and fluorescence in situ hybridization. These findings are consistent with the features of therapy-related AML induced by treatment with DNA topoisomerase II inhibitors; epirubicin was the most likely causative chemotherapeutic agent. The patient responded to induction and consolidation chemotherapy for AML, and attained complete remission. She exhibited various arrhythmias, however, including ventricular tachycardia, which may have been due to the effects of anthracyclines.

Sporadic gastric fundic gland polyp (FGP) without familial adenomatous polyposis is reported to develop in the stomach without Helicobacter pylori infection. We describe two cases of sporadic FGP which developed in the stomach infected with H. pylori. The FGPs developed in the mucosa without endoscopic atrophy. Sporadic FGP has been reported to disappear following acquisition of H. pylori, and to develop after eradication of H. pylori. Namely, sporadic FGP has not developed in the mucosa inflamed by H. pylori infection. H. pylori infection and histological inflammation in the mucosa around the FGP were present in one of the two patients, however. Our case reports show that sporadic FGP can develop in the mucosa without atrophy but with inflammation due to H. pylori infection.

Bullous pemphigoid (BP) is an acquired autoimmune blistering disorder that affects mainly the skin. Although the esophageal mucous membrane is sometimes involved in mucous mem brane pemphigoid (MMP), it is rarely involved in bullous pemphigoid. An 82-year-old Japanese man with BP developed blood-filled blisters and circumferential ulcers in the upper esophagus. Ulcer formation correlated with the titer of anti-BP180 antibody, suggesting that the esophageal lesions were due to BP itself. Only a few Japanese cases of BP have been reported in the esophagus involving anti-BP180 antibody. It might be necessary to examine esophageal lesions even when patients complain of mild symptoms such as dysphagia or are merely vomiting.

A 3-year-old girl with a left cervical mass was admitted to our hospital. A firm, immobile left cervical mass was palpable. Computed tomography and magnetic resonance imaging revealed a lesion extending mainly from the left carotid space to the left lobe of the thyroid gland. As malignancy could not be excluded, we performed an open biopsy. During the biopsy, a small amount of pus outflowed from the mass; Streptococcus parasanguis, a normal oral flora, was isolated by culturing the pus. A histopathological study revealed chronic inflammation, with scattered acute inflammatory processes. Since the abscess was on the left side and a normal oral flora was thought to be the pathogen responsible, pyriform sinus fistula was suspected. Although pharyngoesophagography is stated to be useful for diagnosing pyriform sinus fistulas, it was hard to perform in the present case because of the patient's fear and anxiety. Because grape juice was her favorite, we prepared a contrast material consisting of equal parts of grape juice and barium sulfate. A carton of this juice was employed as a container and a blinder for the contrast material. By drinking the contrast material with grape flavor through a straw inserted to the carton as usual, she might feel less fear and anxiety, resulting in successful pharyngoesophagographywith sufficient cooperation. Although pharyngoesophagography did not reveal a fistula tract, she was treated by incision with drainage and antibiotics and was then discharged. Pharyngoesophagography was repeated 3 months after discharge and revealed a left pyriform sinus fistula. We reviewed published pediatric cases; the relative ineffectiveness of pharyngoesophagography in preschool children was demonstrated. Their poor cooperation was thought to impair its usefulness. Our case suggests that devising a patient-friendly method can alleviate the difficulty in performing pharyngoesophagography and lead to successful diagnosis of pyriform sinus fistulas, even in preschool children.

A64-year-old woman was admitted to our hospital suffering from a rapidly progressive foot edema and a bloating sensation for six weeks. Based on these symptoms we suspected carcinomatous peritonitis and malnutrition, and performed abdominopelvic enhanced CT and aspiration of ascites. CT showed big folds of gastric mucosa, but histology of ascites was negative. We also performed esophagogastroduodenoscopy, but histology of gastric mucosa showed only foveolar hyperplasia with interstitial lymphocytic infiltration. We detected Helicobacter pylori infection with a positive urea breath test. Eradication therapy for Helicobacter pylori improved all of her symptoms about ten days after the eradication, and she was discharged. She was finally diagnosed as having Ménétrier's disease with Helicobacter pylori infection. We reported Ménétrier's disease as a rare case in the differential diagnosis of rapidly progressive anasarca.

We have introduced a new method of resection for two patients with a benign nasopharyngeal mass, using ultrasonic cutting and a coagulation system, and a rigid endoscope. These masses were hypervascular lesions arising from the posterior wall of the nasopharynx. They were removed surgically under general anesthesia, for diagnosis and treatment. For adequate hemostasis and resection of the lesion, we inserted cutting instruments and an endoscope through the oral cavity, lifting up the soft palate with a flexible catheter, and resected the mass. The procedure took less than five minutes in each case, being minimally invasive, and there was no postoperative bleeding or severe pain. One case proved to be pyogenic granuloma and the other was schwannoma, which are rare diseases in the nasopharyngeal region.

In cancer therapy, achievement of cure and delaying of death make enormous differences in patient survival benefit and quality of life. Unfortunately, conventional survival analyses such as the log-rank test and Cox regression have failed to distinguish between these two outcomes, occasionally misguiding clinicians in the evaluation of cancer therapies and prognostic factors. Cox himself has recently acknowledged the limitations of his model. These problems have spurred the present body of work which has included the development of computer programs for cancer survival analysis based on the Boag model and its extensions, and the distribution of CDROMs of the programs among clinical oncologists so that they can share relevant survival information with their patients. The present paper explains the Boag model and its extensions, and instructs how to run the programs. Boag assumed that, in a group of cancer patients, a fraction c are cured of the disease (cancer under study) while the remaining uncured patients (1-c) die from the disease at times whose logarithms follow a normal distribution with mean m and variance s ². The first task is to calculate the Boag three parameters for a given group of patients. The second task is to predict the mean survival time (life expectancy) for the whole group using the competing risk model, taking into consideration the risk of deaths from all causes. The third task is to evaluate the effects of prognostic factors (e.g., treatments, laboratory data, clinicopathological variables) on the Boag parameters using the Gamel three regressions, in which prognostic factors are predictor variables and c, m and s are dependent variables. The computer programs were first written in HTBasic for Windows (by S.M.) and translated into Visual Basic for Application (by H.B). It is hoped that this survival analysis will provide more meaningful survival information to clinical oncologists and patients.