[Durable remission of Langerhans cell sarcoma attained by autologous hematopoietic stem cell transplantation following surgical resection].

Langerhans cell sarcoma (LCS) is a rare neoplastic proliferation of Langerhans cells with a poor prognosis. Owing to its rarity, standard treatment for LCS has not been established to date. Here, we report a case of LCS occurring in multiple lymph nodes in the right cervix in which remission is maintained by autologous hematopoietic stem cell transplantation (auto-HSCT) after surgical resection. A 58-year-old male presented with enlarged right submandibular lymph nodes. Positron-emission tomography/computed tomography (PET/CT) revealed multiple lymphadenopathies in his right cervix. We performed a lymph node biopsy, and he was diagnosed with LCS. We selected the CHOP regimen as the first-line chemotherapy; however, rapid disease progression was observed soon after the first cycle of the therapy. The neck dissection was performed on day 16 of the CHOP therapy. As the residual tumor was suspected, we started the second-line chemotherapy with a combination of etoposide, cisplatin, ifosfamide, and gemcitabine; complete remission was confirmed by PET/CT. Subsequently, the patient was administered high-dose chemotherapy with auto-HSCT. After 2 years of auto-HSCT, complete remission has been maintained. Although there is no report of auto-HSCT for LCS, it could be an effective therapeutic tool for the disease.

[Time course of changes in gustatory function test results and subjective symptoms, and predictive factors for response in patients with taste disorder receiving 24-week zinc replacement treatment].

In a taste disorder, an agreement between patients' complaints and gustatory function test results is not necessarily found both at the initial hospital visit and during the course of treatment; therefore, it is difficult to assess treatment responses and review treatment strategies based on the assessed treatment responses. The present study investigated the time course of changes in disc gustometry results and subjective symptom scores measured at 4-week intervals in 44 patients with a taste disorder who were considered eligible for zinc replacement treatment and who received polaprezinc at a dose of 150 mg/day (equivalent to a 34 mg/day dose of zinc) for up to 24 weeks. The study also examined the potential differences in treatment outcomes according to the predictive factors for response such as patient background and assessed disc gustometry results during the course of treatment. Results indicated that disc gustometry results and subjective symptom scores showed different time courses of changes. The response rate as measured by disc gustometry was 47.7% at week 12 of treatment, and showed a subsequent slow increase to 56.8% at week 24. On the other hand, subjective symptom scores showed a time-proportional improvement up to week 24. Among the patients included in the present study, a clear difference was found according to the presence or absence of an improving trend as determined by disc gustometry at week 12 of treatment, although there were no differences in ultimate treatment responses, including categories of taste disorder, according to patient background. Patients showing a trend toward improvement had significantly better treatment responses in terms of both ultimate response rates and subjective symptom scores, whereas patients showing no trend toward improvement were less likely to respond to the subsequent 12-week continued treatment.

[An endoscopic transnasal transsphenoidal biopsy for pituitary gland Langerhans cell histiocytosis in a child: a case report].

Endoscopic transnasal transsphenoidal surgery is gaining wide acceptance as the first-line treatment of pituitary tumors in adults, as this procedure is minimally invasive and associated with good outcomes. On the other hand, conventional transcranial and sublabial approaches are chosen for infants as their small anatomical structures pose some difficulty. This study reports on the case of a 5-year-old boy who had a Langerhans cell histiocytosis appearing in the sella. The biopsy was performed using the endoscopic transnasal transsphenoidal approach, and the patient has not exhibited any post-operative complications. Although the patient was a fairly young child, the endoscopic transnasal transsphenoidal approach resulted in fewer complications as compared to the transcranial and translabial approaches. Otolaryngologists, therefore, should view this approach more positively. The indication for pediatric skull base diseases can be expected to expand to include endoscopic transnasal surgery.

[A case of a large neonatal pharyngeal teratoma].

The congenital teratoma is a rare malformation. We present herein on the case of a large teratoma that arose from the epipharynx in a neonate. The obstructive mass caused maternal polyhydramnios and was identified prenatally by ultrasonography at another hospital. The mother was transferred to our hospital for delivery. We explained about the ex utero intrapartum treatment (EXIT) because of the risk of airway obstruction at birth, but EXIT was refused by the parents. Delivery was accomplished by cesarean section at the 35 th week. Since the newborn had a severe respiratory disorder due to a large intraoral tumor and intratracheal intubation was very difficult, a tracheostomy was done under local anesthesia for 7 min. The tumor was removed successfully on the 9th day and 44th day of age.

[The effect of zinc agent in 219 patients with zinc deficiency-inductive/ idiopathic taste disorder: a placebo controlled randomized study].

Diagnosis and treatment of taste disorders are challenging because the disorder can only be determined by the awareness of the patient. Hence, these disorders still require comprehensive evidence. We conducted a randomized, placebo-controlled double-blind study to investigate the effect of polaprezinc, a zinc-containing agent, in 219 patients with either zinc deficiency-inductive or an idiopathic taste, disorder. As a result, the zinc-treated arm experienced a statistically significant improvement against the placebo-treated arm in the perceptible threshold scores of the filter-paper disk method 8 weeks after the administration of the investigational drug. Moreover, the effect lasted for 4 weeks after discontinuation of the drug. However, the effective ratios based on the initial criteria were 55.6% in the treatment group and 43.2% in the placebo, where no statistical significance was recorded. Sex and degree of depression could be two of the potential factors to explain this discrepancy. Furthermore, the effect was not significant among male patients and patients with a high depression score based on the Self-rating Depression Scale (SDS) test. These results indicate that determining the symptom among such patients remains undisclosed. Whereas, in approximately 77%, or 168 patients with "normal" SDS scores and with completely impaired taste qualities, the ratio of effective cases reached 60.9% in the zinc-treated group, the ratio of the placebo-treated group reached 39.5%, resulting in a statistical significance. This may be partly because of a problem in the adaption of male subjects to the gustatory analyses, especially to the identification of saltiness and sourness. Care must also be taken regarding the depressive state of patients when diagnosing and treating taste disorders. Taste disorders caused by depression may not be cured by zinc supplementation due in part to the fact that the symptom is based on a mental issue, and due in part to the conservative responding bias generated by the depression itself, which may inhibit accurate and precise diagnosis of the disorder. In conclusion, administration of a zinc agent is effective for patients with taste disorders, provided selection of appropriate patients is performed, and that proper examination and evaluation are conducted. The present study also indicated that examining depressiveness based on the SDS scores and investigating disturbance of each taste quality using the filter-paper disk method are recommended for the diagnosis and determination of the treatment effect of a taste disorder.

[A case report on cervical neuroblastoma with ataxia].

We present a case of pediatric primary cervical neuroblastoma (NB), which is extremely rare. A 3-year-old girl with ataxia but no nasal obstruction, dysphagia, or stridor was diagnosed with cervical NB. Diagnostic results including clinical chemistry, full blood count, and serology, were normal. Ataxia worsened within a few days after onset and was not cured by steroids or intravenous immunoglobulin, delaying a definite diagnosis until a tumor was detected. Opsoclonus-myoclonus syndrome is typified by opsoclonus with myoclonus and ataxia, primarily associated with neuroblastoma. Brain stem cell and cerebellum dysfunction is thought to be due to an autoimmune mechanism. Following chemotherapy and selective neck dissection, the girl has had no recurrence or adverse sequelae. Ataxia disappeared during chemotherapy. We suggest that neuroblastoma should be considered in any child with unexpected by prolonged ataxia.

A zinc-containing compound, Polaprezinc, is effective for patients with taste disorders: randomized, double-blind, placebo-controlled, multi-center study.

CONCLUSIONS: The effect of a zinc-containing compound, Polaprezinc, was shown to clinically improve the disease conditions of idiopathic taste disorders with no serious side effects in a well controlled double-blinded clinical study. OBJECTIVES: The purpose of the present study was to evaluate the efficacy and safety of a zinc-containing compound in the treatment of patients with idiopathic taste disorders, including patients with low serum zinc, by a Good Clinical Practice (GCP)-compliant, randomized, placebo-controlled, double-blind, multi-center clinical study. SUBJECTS AND METHODS: A group of 109 patients suffering from taste disorders was assigned into placebo and three treatment groups. Each group of patients was given either placebo (n=28), or 17 mg (n=27), 34 mg (n=26) or 68 mg (n=28) of oral zinc, Polaprezinc preparations, daily for 12 weeks. RESULTS: The group of patients given 68 mg zinc showed a significant improvement in their gustatory sensitivity compared with the placebo group. The most common side effects observed were increase in serum triglyceride and serum alkaline phosphatase, decrease in serum iron, and some gastrointestinal incidents, although they were not serious.

[Tracheal cartilaginous sleeve in craniosynostosis].

Tracheal cartilaginous sleeve (TCS) is a congenital malformation involving fusion of the tracheal arches that may be isolated to a few tracheal arches, include the entire trachea, or extend beyond the carina into the bronchi. Tracheotomy was required in 9 of 23 craniosynostosis cases undergoing gradual distraction at Osaka City General Hospital from March 2002 to April 2006. TCS was diagnosed in 5 of 9 cases-four Pfeiffer patients and one Crouzon patient. Diagnosis was made intraoperatively during tracheotomy or at autopsy. 3D-CT was not useful in diagnosing TCS. Aggressive management of respiratory infection and pulmonary secretion, selection of appropriate tracheostomy tubes, and endoscopic evaluation are very important to care in managing TCS patients.

Gamma knife radiosurgery for intracanalicular acoustic neuromas.

In the present study we reviewed our long-term experience with radiosurgery for intracanalicular acoustic neuromas. The study involved 25 patients with unilateral intracanalicular acoustic neuromas. The treatment volume was 0.07 to 0.8 cm(3) (median: 0.27 cm(3)). The marginal radiation dose used for treatment was 12 Gy for all patients. The follow-up period ranged from 36 to 132 months (mean: 89 months). The actuarial rate of tumour growth control at 5 and 10 years after radiosurgery was 96%. Hearing preservation was achieved in 16 patients (64%) and improvement (>20 dB relative to presurgical values) was noted in one patient (4%). No patients experienced post-radiosurgery facial palsy or other cranial nerve deficits. Based on our study and long-term follow-up, radiosurgery can produce high rates of tumour growth control and hearing preservation for intracanalicular acoustic neuromas, and is an alternative to surgical resection given its low level of invasiveness. When determining treatment for intracanalicular acoustic neuromas, the condition's benign natural course and the likelihood of hearing preservation must be taken into account.

Taste disorders: a survey of the examination methods and treatments used in Japan.

CONCLUSIONS: In Japan, the number of patients seeking treatment for taste disorders from otolaryngologists is approximately 240 000/year, and this number has almost doubled in the last 13 years. Taste examinations are routinely conducted in almost all university hospitals; testing is not as common in private clinics. Administration of zinc is the main treatment for taste disorders in Japan. OBJECTIVES: To investigate the number of patients who visited otolaryngologists for treatment of taste disorders between 2000 and 2002. In addition to determining changes in the number of patients that had occurred in the 13 years since the last study in 1990, the survey also sought to find out what types of taste examination were used to diagnose taste disorders in Japan and what therapies were used to treat them. MATERIAL AND METHODS: A questionnaire was mailed to 1559 members of the Japan Society of Stomato-pharyngology. RESULTS: The average number of patients with taste disorders who visited otolaryngological clinics between 2000 and 2002 was 244 858/year. This number is 1.77-fold greater than the number of patients reported by the last study in 1990 (138 575). Electrogustometry was used in 99% of university hospitals and in 20% of private clinics. The filter-paper disk method was also used in 57% of university hospitals and in 27% of private clinics. The main treatment was zinc administration. Most doctors (70%) prescribed polaprezinc, a zinc-containing drug that is used to treat peptic ulcers. Of these doctors, 78% stated that it effectively treated taste disorders and 11% that it did not.

Further examination of infants referred from newborn hearing screening.

OBJECTIVES: Universal newborn hearing screening (UNHS) is considered beneficial and is accepted worldwide. However, some problems remain, and administrative systematization has yet to be established in many countries. This study assessed the hearing screening of referred newborn infants and discusses the problems that remain. MATERIALS AND METHODS: Over the two years from July 2001 to June 2003, 98 ears of 49 infants were judged as a "referral" from a newborn hearing screening program, and were subsequently referred to our hospital for further examination using conventional ABR and other audiological tests. The methodology used for hearing screening varied between practitioners and hospitals that utilized both different recording apparatus for AABR and/or automated DPOAEs and independent protocols. RESULTS: Conventional ABR identified 21 infants with bilateral normal hearing, 12 with unilateral hearing loss, and 16 with bilateral hearing loss, and a total correspondence rate of 40.8% (20 out of 49 infants). In a comparative analysis, 26 ears out of 98 (26.5%) were determined as false-positive, seven out of 98 as false-negative (7.1%), and there was a total correspondence rate of 66.3% (65 out of 98 ears). Five of the seven false-negative cases who were referred with unilateral hearing loss exhibited moderate to profound bilateral hearing loss (moderate; one infant, severe to profound; four infants). Of the 16 infants with bilateral hearing loss, nine with more than moderate loss had hearing aids fitted at our hospital or related educational institution before most were six months old. CONCLUSIONS: Our results suggest the accuracy of newborn hearing screening remains an issue, but may be improved by an experienced examiner and better protocols including a two-stage process and altered timing of screening. Other ongoing health care programs need to monitor for signs of hearing loss even in the "passed" infants because of possible false-negatives and delayed-onset hearing loss. Improvement is needed in both the intervention systems and diagnostic follow-up of hospitals. Early public support is also required for infants with either severe to profound or moderate hearing loss. From the viewpoint of test conditions and puerperal parental psychological problems, it is considered that the timing of screening needs further discussion. Here it is suggested that screening should be performed within the first three months of infant's life but not be limited to before hospital discharge, and incorporated into the routine health care program for one-month-old infants without reducing efficiency.

Magnetic resonance imaging for diagnosis of congenital anosmia.

Magnetic Resonance Imaging (MRI) was performed on 9 patients who lacked a sense of smell since birth. Seven of them, including two patients with Kallmann syndrome, exhibited abnormality of the olfactory bulb, olfactory tract, olfactory sulcus, or rectus gyrus, with some variation among patients in type and degree of abnormality. The other two patients exhibited normal olfactory pathway morphology, and for them the possibility of acquired sensorineural anosmia could not be ruled out. MRI is useful for determining whether patients with congenital anosmia have olfactory pathway anomalies. Many patients with congenital anosmia and hypoplasty or aplasty of the olfactory pathway nevertheless had no gonadal or endocrinological disorders.

[A device for tracheostomized children in home care].

Tracheomized children with deglutition disturbance require frequent suction--a procedure that may present problems for parents and other home-caregivers. We introduced a suction pump to drain the thoracic cavity continuously. This reduced the number of times of suction was required and raised no problems.