An immunocompetent case of chronic pulmonary aspergillosis caused by Aspergillusviridinutans.

We encountered an extremely rare immunocompetent case of chronic pulmonary aspergillosis (CPA) caused by Aspergillus viridinutans. A 74-year-old woman was admitted with fever and hemoptysis. Chest computed tomography revealed a nodule in the left upper lobe. Bronchoscopy was performed, and the transbronchial biopsy specimen revealed Aspergillus fungi. Treatment of the nodule was initially ineffective with voriconazole but effective with liposomal amphotericin B. The causative organism was later identified as A. viridinutans based on the gene sequence of ß-tubulin. This is the first immunocompetent case of CPA caused by A. viridinutans.

Acute Hemorrhagic Rectal Ulcer Complicated by Cytomegalovirus Enteritis following Steroid Pulse Therapy for Acute Exacerbation of Interstitial Pneumonia.

We herein report a rare case of acute hemorrhagic rectal ulcer (AHRU) complicated by cytomegalovirus enteritis following steroid pulse therapy for interstitial pneumonia. An 86-year-old woman underwent steroid pulse therapy for interstitial pneumonia. She was bedridden with dyspnea and suddenly developed melena. Colonoscopy revealed AHRU, which did not improve with conservative treatment, but did improve with ganciclovir administration for cytomegalovirus enteritis. This gastrointestinal complication has not received much attention by pulmonologists who perform steroid pulse therapy for interstitial pneumonia. Delayed treatment of this complications can be fatal. Caution should be taken when administering steroid pulse therapy to bedridden patients with interstitial pneumonia.

Dramatic improvement in innominate artery compression syndrome with only postural changes in a bedridden elderly patient.

We present the case of a bedridden elderly patient who demonstrated dramatic improvement in innominate artery compression syndrome with postural changes alone. A 94-year-old woman presented with dyspnea. Physical findings revealed stridor in the right-sided decubitus position. Her symptoms did not improve with conventional asthma treatment. Plain computed tomography revealed tracheal compression by the bent innominate artery. Contrast-enhanced computed tomography with postural changes altered the compression sites of the organs, resolving the tracheal stenosis. The number of similar bedridden elderly patients will increase in an aging society. We report this case to aid physicians in managing such patients.

A case of nonmucinous pulmonary micropapillary adenocarcinoma mimicking pulmonary tuberculosis.

We report a rare case of nonmucinous pulmonary micropapillary adenocarcinoma mimicking pulmonary tuberculosis. A 68-year-old woman was hospitalized with hemoptysis. Her computed tomography revealed cavities and tree-in-bud appearance similar to the extensive form of pulmonary tuberculosis. However, histopathological findings of transbronchial biopsies of all lesions revealed adenocarcinoma and no pulmonary tuberculosis. Tree-in-bud appearance may relate to the floating micropapillary tufts in alveolar spaces. If pulmonary carcinoma is complicated by pulmonary tuberculosis, patients must be isolated and disadvantaged in cancer treatments. Therefore, recognizing this case may be therapeutically useful for respiratory physicians treating both diseases.

Dramatic Response to Alectinib in a Critically Ill Elderly Patient with Lung Adenocarcinoma Due to Trousseau Syndrome and Disseminated Intravascular Coagulation.

Lung cancer complicated with Trousseau syndrome (TS) or disseminated intravascular coagulation (DIC) has a severe prognosis. We herein report an elderly lung cancer patient who presented with a critically ill condition due to concomitant TS and DIC and responded dramatically to alectinib. There are no rules regarding treatment indications based on the age or severity of critically ill patients. If the patient's cancer cells are positive for anaplastic lymphoma kinase rearrangement, alectinib is worthwhile to administer, even in a critically ill condition. In our patient, anticoagulation failed to suppress the TS complications. We also report how to prevent the recurrence of TS.

A Fast-growing Benign Metastasizing Leiomyoma Mimicking Malignancy.

We herein report a rare case of fast-growing benign metastasizing leiomyoma. A 52-year-old woman was admitted to our hospital with abnormal chest shadows. Chest computed tomography showed well-circumscribed cystic tumors. Because malignancy could not be completely distinguished in fast-growing tumors, video-assisted thoracic surgery was performed. The pathological findings revealed many cysts and the proliferation of smooth muscle cells. According to the Stanford criteria, the tumor was diagnosed as benign metastasizing leiomyoma. One possible reason for the fast growth of the tumor was enlargement of the cysts. Malignant diseases characterized by cystic tumors are rare but occasionally reported. Therefore, differentiation by a pathological examination is essential.

A Novel Type of Aspiration Presenting With Acute Respiratory Failure Caused by Upper Airway Obstruction Due to Diaper Pica.

We herein report an extremely rare case of acute respiratory failure due to pica of the high-molecular-weight polymers in a diaper (HMWPD). An 81-year-old woman was admitted to our hospital because of dyspnea. Several hours earlier, the facility staff saw her tearing the diaper and eating it. And several hours later, the staff found her complaining of dyspnea. Her chest computed tomography revealed airway foreign bodies. Immediately, flexible bronchoscopy was performed. The trachea and the bilateral bronchi were filled with the HMWPD. We tried to grasp the HMWPD with various common instruments, but the HMWPD could not be grasped. Finally, we performed direct bronchoscopic suction. All the HMWPD were removed successfully. The HMWPD have hygroscopic property resulting in volume expansion and the fatal consequence of upper airway obstruction. Physicians working in an emergency department should be aware of this acute respiratory failure due to diaper pica and its effective treatment.

PaO2-guided Low-dose Oxygen Therapy for Pneumatosis Cystoides Intestinalis to Prevent Acute Exacerbation of Interstitial Pneumonia.

We herein report the first case of low-dose oxygen therapy for pneumatosis cystoides intestinalis (PCI) using PaO2 as a therapeutic index to prevent acute exacerbation of interstitial pneumonia. An 86-year-old man was admitted to our hospital with abdominal distension. PCI was diagnosed by abdominal computed tomography. Low-dose oxygen therapy was started to avoid acute exacerbation of interstitial pneumonia. The oxygen dose was adjusted so that the PaO2 value was approximately 100 mmHg. After seven days of treatment, the colon gas had disappeared, and no acute exacerbation of interstitial pneumonia was observed. A PaO2 value around 100 mmHg is effective for PCI without inducing acute exacerbation of interstitial pneumonia.

Successful Treatment of Antifungal Combination Therapy with Inhaled Liposomal Amphotericin B and Oral Voriconazole for Intractable Chronic Progressive Pulmonary Aspergillosis.

We experienced a case of the successful treatment of intractable pulmonary aspergillosis with inhaled liposomal amphotericin B (L-AMB) and oral voriconazole (VRCZ). A 52-year-old man was admitted to our hospital with a fever. Chest computed tomography (CT) revealed an infiltrative shadow. Two separate sputum cultures detected Aspergillus niger. Although we treated the patient with single and combined antifungal agents, the infiltrative shadow worsened. After obtaining sufficient informed consent from the patient, we switched him to an inhaled L-AMB. The infiltrative shadow subsequently improved. The patient has remained well for one year without exacerbation. We herein report the usefulness of inhaled L-AMB and oral VRCZ.

Interstitial Pneumonia Secondary to Hermansky-Pudlak Syndrome Type 4 Treated with Different Antifibrotic Agents.

Hermansky-Pudlak syndrome (HPS) is an autosomal recessive hereditary disease that may be complicated by progressive and potentially fatal interstitial pneumonia. We herein report a 64-year-old woman with interstitial pneumonia associated with HPS type 4 whom we treated with nintedanib after pirfenidone proved ineffective. To our knowledge, there have been no previous reports of nintedanib being used to treat a patient with HPS type 4. There is a need for clinical trials of antifibrotic agents, including nintedanib, pirfenidone, and new therapeutic agents with different mechanisms of action in these patients.

Relationship between tympanic membrane retraction and habitual sniffing in patients with cholesteatoma.

CONCLUSION: Habitual sniffing affects the pathogenicity and recurrence of cholesteatoma. Postoperative instructions requesting patients to cease sniffing may reduce the retraction and recurrence of cholesteatoma. OBJECTIVE: To examine the relationship between tympanic membrane retraction and habitual sniffing in patients with cholesteatoma. METHODS: We recruited 98 patients (102 ears) who were surgically treated for cholesteatoma by canal wall-down tympanoplasty (22 ears) or canal wall-down tympanoplasty with reconstruction methods (80 ears). We classified these patients into two groups on the basis of their preoperative habitual sniffing: habitual and non-habitual sniffers. The findings of the contralateral tympanic membrane were examined in each group and were classified according to the Tos classifications. Next, we evaluated the incidence of 1-year postoperative tympanic membrane retraction treated by the canal-down tympanoplasty with reconstruction method in the following three groups: non-habitual sniffing group, sniffing cessation group, and continual sniffing group. RESULTS: In habitual sniffers, the Tos classifications of contralateral tympanic membrane were normal in 7% (3/41). In contrast, for non-habitual sniffers, the findings were normal in 39% (21/54). These results indicate that sniffing causes tympanic membrane retraction. The tympanic membranes of patients in the sniffing cessation group were largely normal after surgery. However, more than 50% of the patients who continued to sniff after surgery showed retraction or recurrent cholesteatoma.

A Case of Hypocalcemia with Severe Vitamin D Deficiency following Treatment for Graves' Disease with Methimazole.

We herein report the case of a 41-year-old Japanese female office worker who developed symptomatic hypocalcemia with severe vitamin D deficiency following treatment for Graves' disease with methimazole. The patient's hypocalcemia was mainly caused by vitamin D deficiency due to unbalanced diets and inadequate exposure to sunlight in addition to the resolution of hyperthyroidism. Vitamin D deficiency is increasing worldwide, and it has been more recently shown to relate to the pathogenesis of Graves' disease. However, vitamin D deficiency as a cause of hypocalcemia has received little attention. Taken together, this case suggests that we should take more care in calcium kinetics and vitamin D status during treatment for Graves' disease with antithyroid drugs.

Clinical outcome in patients with leptomeningeal metastasis from non-small cell lung cancer: Okayama Lung Cancer Study Group.

OBJECTIVE: We examined the prognosis of patients with leptomeningeal metastasis (LM) from non-small cell lung cancer (NSCLC) and that stratified by epidermal growth factor receptor (EGFR) mutation status in LM patients receiving EGFR-tyrosine kinase inhibitors (TKIs). METHODS: We retrospectively analyzed a series of 91 consecutive NSCLC patients with LM between 2001 and 2010. RESULTS: Most of the LM patients had adenocarcinoma histology and a poor performance status (PS). The median survival time (MST) for all patients was 3.6 months. Adenocarcinoma and TKI treatment were associated with a better prognosis. Among the patients, 51 received EGFR-TKIs. Of these, the EGFR mutation status was assessed in 30 patients; 7 (23%) showed no mutation (group 1), 10 (33%) had a mutation in exon 21 (group 2), and 13 (43%) had deletions in exon 19 (group 3). Interestingly, PS was significantly improved in groups 2 and 3 but not in group 1. The MST in these subgroups was 1.4, 7.1, and 11.0 months in groups 1, 2, and 3, respectively (p<0.001). The median time to progression or symptom deterioration was 0.9, 2.0, and 7.8 months for groups 1, 2, and 3, respectively (p<0.001). A multivariate analysis showed that EGFR-mutant tumors were associated with a better prognosis in patients receiving EGFR-TKIs. CONCLUSIONS: The prognosis for patients with LM from NSCLC was still poor. Survival after the initiation of EGFR-TKI treatment differed according to the type of EGFR mutation, suggesting the potential benefit of TKIs for patients with EGFR mutations, even though they suffered from LM.

Two cases of myxoma of the external auditory canal.

Myxomas were found in the outer ear canal of a 66-year-old female and a 51-year-old male. Both tumors were soft and sessile and located at the antero-superior part of the external auditory canal near the eardrum. Profuse, clear mucus was seen when the tumor ruptured or was incised. Histology showed spindle-shaped and stellate cells in myxoid extracellular matrix. CT images revealed a small bone defect adjacent to the tumor in the meatal bone. This bony defect was confirmed during surgery. The bony defects seen in both cases may have something to do with the genesis of the tumor. We found only 5 sporadic cases, including ours, reported in the English literature. As myxoma of the outer ear canal is also reported in 18 patients as a symptom of Carney's complex, we must check for accompanying symptoms of the complex such as spotty skin pigmentation, endocrine tumors, or schwannoma. Total removal of the tumor is necessary to prevent recurrence.

Phase II study of irinotecan and amrubicin in patients with relapsed non-small cell lung cancer: Okayama Lung Cancer Study Group Trial 0402.

BACKGROUND: The survival advantage achieved by existing anti-cancer agents as second-line therapy for relapsed non-small cell lung cancer (NSCLC) is modest and further improvement of treatment outcome is desired. Combination chemotherapy with irinotecan and amrubicin for advanced NSCLC has not been fully evaluated. METHODS: The primary endpoint of this phase II clinical trial was objective response. Patients with NSCLC who had been treated previously with one or two chemotherapy agents were enrolled. Irinotecan and amrubicin were both administered on Days 1 and 8 of a 21-day cycle, at doses of 100 mg/m(2) and 40 mg/m(2), respectively. RESULTS: Between 2004 and 2006, 31 patients received a total of 101 courses; the median number of courses administered was three (range, one to six). Objective response was obtained in nine of the 31 patients (29.0% response rate; 95% confidence interval (CI), 12.1-46.0%). With a median follow-up time of 43.9 months, median survival time and the median progression-free survival time were 14.2 and 4.0 months, respectively. Myelosuppression was the most frequently observed adverse event, with grade 3/4 neutropenia in 51% of patients. Febrile neutropenia developed after nine courses (9%) and resulted in one treatment-related death. Cardiac toxicity and diarrhea, possibly specific for both agents, were infrequent and manageable. CONCLUSION: Combination chemotherapy with irinotecan and amrubicin is effective in patients with NSCLC but showed moderate toxicities in second- or third-line settings.

Drug discontinuation after treatment with minimum maintenance dose of an antithyroid drug in Graves' disease: a retrospective study on effects of treatment duration with minimum maintenance dose on lasting remission.

According to the guideline issued by the Japan Thyroid Association in 2006 for treatment of Graves' disease, discontinuing antithyroid drug (ATD) therapy is recommended when serum free thyroxine (FT4) and thyroid stimulating hormone (TSH) concentrations have been maintained within the reference range for a certain period after treatment with one ATD tablet every other day (minimum maintenance dose therapy, MMDT). In this retrospective study, the relationship between MMDT duration and remission rate was investigated. The participants were 107 consecutive patients with Graves' disease whose ATD therapy was stopped according to the guideline. Serum FT4, TSH, and TSH receptor antibody (TRAb) levels were measured when ATD was discontinued and every 3 months thereafter. The percentage of patients in remission was 86.9% at 6 months, 73.8% at 1 year, and 68.2% at 2 years after ATD discontinuation. The remission rate increased with MMDT duration, being significantly higher in patients with MMDT durations of 19 months or more than those with MMDT durations of 6 months or less. In patients with MMDT durations of 6 months or less, the remission rate was significantly lower in TRAb-positive patients than in TRAb-negative patients at the time of withdrawal of ATD; however, this was not observed in patients with MMDT durations of 7 months or more. These findings suggest that in patients who discontinue ATD after a certain MMDT duration, the remission rate increases as the MMDT duration increases, and ATD should not be discontinued in TRAb-positive patients with MMDT durations of 6 months or less.

Persistent high TRAb values during pregnancy predict increased risk of neonatal hyperthyroidism following radioiodine therapy for refractory hyperthyroidism.

Serum levels of TSH receptor antibody (TRAb) often increase after radioiodine treatment for Graves' disease, and high-serum levels of maternal TRAb in late pregnancy indicate a risk of neonatal hyperthyroidism. The aim of this retrospective study is to investigate the characteristics of Graves' women who had a history of radioiodine treatment for intractable Graves' disease, and whose neonates suffered from hyperthyroidism. The subjects of this study were 45 patients with Graves' disease who became pregnant during the period from 1988 to 1998 after receiving radioiodine treatment at Ito Hospital. 25 of the 45 subjects had had a relapse of hyperthyroidism after surgical treatment for Graves' disease. 19 pregnancies were excluded because of artificial or spontaneous abortion. In the remaining 44 pregnancies of 35 patients, neonatal hyperthyroidism developed in 5 (11.3%) pregnancies of 4 patients. Serum levels of TRAb at delivery were higher in patients whose neonates suffered from hyperthyroidism (NH mother) than those of patients who delivered normal infants (N mother). Furthermore, serum levels of TRAb in NH mother did not change during pregnancy, although those of 4 patients of N mother, in which serum levels of TRAb before radioiodine treatment were as high as in NH mother, decreased significantly during pregnancy. In conclusion, women who delivered neonates with hyperthyroidism following radioiodine treatment seem to have very severe and intractable Graves' disease. Persistent high TRAb values during pregnancy observed in those patients may be a cause of neonatal hyperthyroidism.