A Case of Acquired Hemophilia A Diagnosed in Conjunction with Oral Bleeding.

Acquired hemophilia A is a rare disease in which autoantibodies to factor VIII are present. It is often manifested as a sudden onset of a critical bleeding episode, and its incidence is reported to be 1.48 cases per million persons per year. We report herein on a case of acquired hemophilia A associated with a submucosal hematoma of the oral floor, pharynx, and larynx. A 78-year-old male presented with fresh bleeding from his mouth, associated with hematoma of the oral floor, pharynx, and larynx. Laboratory test showed that the activated partial thromboplastin time was prolonged, and the platelet count and prothrombin time were normal. Coagulation tests revealed decreased factor VIII levels, and the presence of factor VIII inhibitor. A diagnosis of acquired hemophilia A was made, and immunotherapy with corticosteroids was initiated. After treatment, the bleeding tendency was controlled. On hospital day 29, the symptoms disappeared. Although acquired hemophilia A is a rare coagulopathic condition, it should be considered as one of the differential diagnoses in a case of sudden onset of severe hemorrhagic tendency of unknown origin.

Prediction of gastroesophageal reflux in otitis media with effusion in adults.

CONCLUSIONS: The measurement of pepsinogen I (PGI) in middle-ear effusions (MEEs) and a questionnaire on the frequency symptoms of gastroesophageal reflux (GER) disease are tools that can be used to screen for the existence of GER. OBJECTIVE: To seek methods that would be beneficial as a screen for the presence of GER among adult patients with OME. MATERIALS AND METHODS: Fifty-eight adult outpatients with OME were asked to answer a questionnaire of the frequency scale for symptoms of GER disease. Samples of MEEs were obtained from each subject and were measured for concentrations of PGI and PGII. Some patients were followed up after being treated with a proton pump inhibitor. RESULTS: The percentage of patients with high PGI concentrations in their MEEs was higher in those with GER-related symptoms than in those without GER-related symptoms. Moreover, OME was present bilaterally in a higher percentage of patients with GER-related symptoms. There were patients in whom PGI levels decreased after receiving treatment for GER.

Daytime polysomnography for early diagnosis and treatment of patients with suspected sleep-disordered breathing.

Excessive daytime sleepiness (EDS) is a common complaint among patients with sleep-disordered breathing (SDB). Population-based studies on traffic and industrial accidents suggest a relationship between EDS and life-threatening events, and adults with EDS have cognitive and memory problems. Nocturnal polysomnography (nPSG) is essential for diagnosing SDB but it is time and energy consuming. We examined the usefulness of daytime polysomnography (dPSG) for the early diagnosis and treatment of patients with suspected SDB. We studied 108 consecutive patients aged 51.9 +/- 13.5 years (mean+/-SD). All patients underwent dPSG and nPSG. The number of apnea/hypopnea episodes per hour (apnea/hypopnea index: AHI) and the number of 3% desaturation episodes per hour (desaturation index: DSI) were calculated. All patients were classified into two groups. The REM group consisted of subjects who had an AHI < or = 25/h, AHI(REM)/AHI(NREM) > 2, and AHI(NREM) < 15/h. Those who did not satisfy these criteria were placed in the NREM group. Continuous positive airway pressure (CPAP) titration was performed for patients whose AHI was > or =20/h on dPSG. Using the international classification of sleep disorders, 96 patients were diagnosed as obstructive sleep apnea [including five upper airway resistance syndrome (UARS) patients], six patients were snoring, four had idiopathic hypersomnia due to a medical condition, and two had circadian rhythm sleep disorders. The sensitivity of dPSG for AHI was 81.0%, specificity was 100%, and accuracy was 83.5%. The sensitivity and accuracy of dPSG for AHI in the REM group were considerably lower than in the NREM group. There was no significant difference for optimal CPAP between dPSG and nPSG. In the five patients with UARS, their AHI, DSI, and arousal index on dPSG were 0.92 +/- 1.2/h, 2.9 +/- 3.4/h, and 29.3 +/- 3.5/h, respectively, and their AHI and DSI on nPSG were 3.2 +/- 2.5/h and 2.8 +/- 2.4/h, respectively. However, their respiratory effort-related arousals were 37.9 +/- 7.4/h, and their arousal index was 33.2 +/- 6.3/h. The five patients with UARS were also treated with CPAP, and their daytime sleepiness was improved. Although dPSG has limitations, these results indicate that dPSG recording is clinically useful for the diagnosis of and determination of types of treatment in patients with suspected SDB.

Obstructive sleep apnoea associated with congenital choanal atresia.

We report here a very rare case of bilateral congenital choanal atresia untreated for the 27 years after birth. A 27-year-old Japanese man complaining of long-term nasal obstruction, snoring and daytime sleepiness visited our hospital. He exhibited the characteristic appearances of long face and bilateral hypoplastic cheeks. Examination by flexible fiberscopy and computed tomography led us to diagnose him with bilateral choanal atresia. Subsequent polysomnography revealed the number of apnoea or hypopnoea episodes per hour (apnoea-hypopnoea index, or AHI) to be over 10. On the basis of these observations, we modified the diagnosis to obstructive sleep apnoea syndrome (OSAS) secondary to congenital choanal atresia.

Nasal resistance for determinant factor of nasal surgery in CPAP failure patients with obstructive sleep apnea syndrome.

OBJECTIVES: Given that criteria for nasal surgery in individuals with obstructive sleep apnea syndrome (OSAS) have not been proposed, we investigated the effectiveness of nasal surgery for CPAP failure in patients with both severe OSAS and nasal obstruction. PATIENTS AND METHODS: Conventional nasal surgery was performed in 12 patients who were refractory to treatment by CPAP. The subject group consisted of 12 males (mean age, 54.2 +/- 9.2 years; range 39-66 years). The effect of nasal surgery was evaluated with data from preoperative and postoperative polysomunography. The nasal resistance value was first deduced to determine which OSAS patients with CPAP failure should undergo nasal surgery, compared to control values. RESULTS: Nasal surgery resulted in a significant decrease in nasal resistance, as measured by rhinomanometry, from 0.57 +/- 0.31 Pa/cm3 /sec to 0.16 +/- 0.03 Pa/cm3/sec and rendered all patients tolerant to CPAP. In addition, the lowest nocturnal oxygen saturation significantly increased from 68.3 +/- 12.1% to 75.3 +/- 7.1% after surgery. Subjectively, Epworth sleepiness scale (ESS) significantly decreased from 11.7 +/- 4.1 to 3.3 +/- 1.3 after surgery, but the number of apnea and hypopnea episodes per hour did not change significantly. In five patients, for whom it was possible to perform a CPAP titration before nasal surgery, the value decreased significantly from 16.8 +/- 1.1 to 12.0 +/- 1.9 cmH2O. The bilateral nasal resistance of the 410SAS patients with CPAP therapy (control group) was 0.24 +/- 0.11 Pa/cm3/sec. The cut off value for differentiation between CPAP failure patients and control group was determined as 0.38 Pa/cm3 /sec. CONCLUSION: Increased nasal resistance is a determinant of CPAP failure, and the surgical correction of severe nasal obstruction should thus be considered to facilitate treatment of OSAS patients with CPAP.