Changes in characteristics and outcomes in Japanese patients with heart failure from the 2000s to the 2010s: The HIJ-HF cohorts.

Background: The number of hospitalized patients with heart failure (HF) is increasing as a result of the increase in the elderly population in Japan. We evaluated changes in the characteristics and outcomes of HF patients hospitalized in the 2000s to the 2010s and discharged alive based on left ventricular ejection fraction (LVEF).

Methods: Pooled patient data were obtained from The Heart Institute of Japan Heart Failure studies (HIJ-HF I: 2001-2 and HIJ-HF II: 2013-4).

Clinical implications of midventricular obstruction in patients with hypertrophic cardiomyopathy.

Objectives: We investigated the prevalence, clinical characteristics, and prognosis of hypertrophic cardiomyopathy (HCM) patients with midventricular obstruction (MVO).

Background: Previous descriptions of patients with MVO have been confined to case reports or small patient series, and this subgroup of HCM patients has therefore remained underrecognized.

Methods: The study population included 490 HCM patients.

Clinical characteristics and outcome of hospitalized patients with congestive heart failure: results of the HIJC-HF registry.

Background: Heart failure (HF) represents a major public health issue in an aging population. Although HF is a leading cause of morbidity and mortality in developed countries, the clinical features of HF in Japan remain unclear.

Methods And Results: This observational cohort study analyzed data from the Heart Institute of Japan--Department of Cardiology (HIJC)-HF Registry, which is based on a nationwide survey by the HIJC, Tokyo Women's Medical University and its affiliated hospitals.

Hearing loss in Turner syndrome.

Objective: To address the characteristics of hearing loss in patients with Turner syndrome (TS), we evaluated hearing levels of patients with TS and analyzed causative factors.

Study Design: Thirty-three patients with TS (8 to 40 years of age) were studied through the use of audiological measurements, and causative factors were explored.

Results: Twenty cases (35 of 66 ears tested) showed high-frequency (8 kHz) sensory neural hearing loss (HFQ-SNHL).

[Long-term outcome and tolerability of carvedilol therapy in Japanese patients with chronic heart failure].

Objectives: Randomized controlled clinical studies in western countries have shown that the beta-blockers improve the survival of patients with chronic heart failure and are highly tolerable. The tolerability of beta-blocker therapy and the long-term prognosis for patients with chronic heart failure receiving beta-blockers remain unclear in Japan. In the present study, carvedilol was employed for the treatment of mild to severe chronic heart failure and the long-term prognosis of the patients was monitored to evaluate the tolerability and chronic efficacy of beta-blocker therapy in Japanese patients.

[Congenital laryngeal stridor].

We reviewed the diagnosis, complications and treatment of congenital laryngeal stridor (CLS), in 97 patients who consulted our clinic between 1991 and 2001. The 97 patients were diagnosed with laryngeal malacia (32%), vocal cord paralysis and laryngeal stenosis (22%), a neoplastic disease like hemagioma and papilloma (11%), or cystic disease (7%). The cases with vocal cord paralysis, laryngeal stenosis or laryngeal cysts were usually diagnosed within 2 months of birth based on severe dyspnea.

Mondini dysplasia and recurrent bacterial meningitis in a girl with relapsing Langerhans cell histiocytosis.

We report a case of a girl with Langerhans cell histiocytosis (LCH) of multifocal bone disease, who developed recurrent bacterial meningitis and unilateral sensorineural hearing loss during the relapsing course of the disease. Mondini dysplasia, a congenital inner ear anomaly, was suspected by high resolution computed tomographic scan and the dysplasia with cerebrospinal fluid leakage was confirmed by surgery in the ipsilateral ear showing hearing loss. Although rare, congenital inner ear anomalies such as Mondini dysplasia should be kept in mind in pediatric patients with hearing impairment and/or recurrent bacterial meningitis during chemotherapy for various types of neoplasms including LCH.

[Congenital choanal atresia and nasal stenosis].

The most common form of nasal obstruction in neonates is soft tissue edema, but congenital bony nasal anomalies are recognized as an important cause of newborn airway obstruction. We reviewed 20 cases of congenital bony nasal abnormalties such as choanal atresia and nasal stenosis referred to National Children's Hospital between 1996 and 2001. Of the 20, 8 involved choanal atresia (6 bilateral and 2 unilateral) and 12 nasal stenosis (5 nasal cavity stenosis, 2 pyriform aperture stenosis and 5 unknown).

Radiographic diagnosis of congenital bony nasal stenosis.

Congenital bony nasal stenosis (CBNS) is a rare condition that causes respiratory distress in neonates. Between 1986 and 1996, we encountered 13 cases of CBNS. Recently, CT measurements have been used to evaluate the features of this type of stenosis, but no satisfactory investigation of the severity of bony nasal stenosis has been reported.

Lingual cyst in infancy: importance of palpation for diagnosis.

Two infants, 5 and 12 weeks of age, with lingual cysts were presented. Histological findings were compatible with a salivary retention cyst in one and a thyroglossal duct cyst in the other. Both infants were admitted to our hospital because of severe stridor that had developed from one to two weeks of age.

Delayed post-neonatal intensive care unit hearing disturbance.

Ten children, who developed hearing disturbance after discharge from the neonatal intensive care unit (NICU), are reported. All were born at full term, between November 1988 and November 1992, weighed at least 2500 g and had normal auditory brainstem response (ABR) at discharge from the NICU. All ten underwent thorough evaluation to determine the cause of the subsequent hearing loss.

[Diagnosis of sinusitis in children with allergic diseases--comparison of waters projection radiographs and CT scans].

Sinusitis is common in children with allergic diseases, and the relationship between sinusitis and reactive airway diseases involving asthma has been reported. Most pediatricians and physicians base their diagnosis of sinusitis on findings from plain radiographs of the sinuses, especially Waters projection radiographs. We compared the diagnoses made by 11 pediatric allergists using 56 Waters projection radiographs with transverse CT findings.

[Hearing loss following discharge from the NICU].

We experienced 10 pediatric cases born between November of 1988 and November of 1992, whose ABR were normal at the time of discharge from the NICU, who subsequently developed hearing impairment. These children underwent thorough evaluations and the causes of their hearing loss are discussed herein. All 10 were born at full term, birth weights ranged from 2500g to 3826g, and all had severe cardiovascular and/or pulmonary disorders.

Idiopathic deafness or hearing loss of unknown etiology following discharge from the NICU.

The clinical course, treatment and respiratory management of 6 infants who developed profound hearing loss (n = 5) or high tone hearing loss (n = 1) some months after discharge from the NICU are presented. All 6 infants had congenital and/or perinatal diseases causing hypoxia, and all were treated with gentamycin. All were nearly normal at discharge but later rose to 80-90 dB, or 60 dB in the case with high tone hearing loss.

Management of acute otitis media. Assessment of therapeutic strategy in Japan today.

Currently, myringotomy is widely used in the routine initial treatment of acute otitis media in Japan. In an attempt to evaluate the validity of this strategy, a number of issues that demand reassessment or clarification have been identified. These issues and factors contributing to the persisting uncertainty about myringotomy are examined.

Choanal atresia and congenital pharyngeal stenosis.

From 1980 to 1992, 8 patients with choanal atresia were treated in our facility. The female to male ratio was 5:3. One patient, a girl, had unilateral osseous obstruction with membranous closure of the opposite naris while the other 7 had bilateral osseous obstruction.

[Congenital nasal stenosis--new concept and its cases].

Congenital nasal stenosis is a new disease concept which was reported, for the first time, by Knegt-Junk et al in 1988. The authors experienced 10 cases of this disease from 1986 to 1990. Radiography revealed that transverse width of the bony nasal passage of these patients was smaller than that of normal infants.