Multisystem Langerhans’ cell histiocytosis (Hand-Schüller-Christian disease) in an adult: a case report and review of the literature

Langerhans cell histiocytosis (LCH) is a rare and enigmatic clonal disorder that affects mainly children. It is characterized by single or multiple granulomatous mass lesions composed of cells with the Langerhans cell phenotype. Clinical presentation and behavior are heterogeneous and can range from a solitary lytic bone lesion (i.e., eosinophilic granuloma) with a favorable course to a fatal disseminated leukaemia-like form, with a wide spectrum of intermediate clinical presentations between these two extremes. Although LCH typically involves the bone, lesions can be found in almost all organs. We are reporting the case of a multisystem LCH in a 47-year-old patient who presented with a panhypopituitarism and diabetes insipidus, and who, 5 years later, developed mandibular, mastoid and femoral lesions. The final diagnosis of LCH was made on mandibular biopsy.     

surgical management of traumatic facial paralysis: a case review study

Objective To evaluate efficacy of surgical treatment in traumatic facial paralysis.Methods:Thirty-three cases were reviewed,including temporal bone fracture and iatrogenic facial nerve injury.All the p...     

Clinical effectiveness of palifermin in prevention and treatment of oral mucositis in children with acute lymphoblastic leukaemia: a case–control study

The aim of this study was to evaluate the efficacy of palifermin, an N-terminal truncated version of endogenous keratinocyte growth factor, in the control of oral mucositis during antiblastic therapy. Twenty patients undergoing allogeneic stem-cell transplantation for acute lymphoblastic leukaemia were treated with palifermin, and compared to a control group with the same number of subjects and similar inclusion criteria. Statistical analysis were performed to compare the outcomes in the treatment vs. control groups. In the treatment group, we found a statistically significant reduction in the duration of parenteral nutrition （P=0.002）, duration of mucositis （P= 0.003） and the average grade of mucositis （P= 0.03）. The statistical analysis showed that the drug was able to decrease the severity of mucositis. These data, although preliminary, suggest that palifermin could be a valid therapeutic adjuvant to improve the quality of life of patients suffering： from leukaemia.     

Nasal complaints and signs of disease in farmers – a methodological study

Conclusion. The methods used in this study are suitable for field studies that involve examinations of groups of workers. For individual examinations, there is no gold standard method that can discriminate work-related discomfort from other causes of rhinitis. Objectives. Studies of the effects of occupation on farmers’ health have mainly focused on lower airways; few studies have examined effects on upper airways. This study investigated nasal functions in three groups of farmers (swine, milk and grain producers) and a control group using different methods, suitable for field studies. Subjects and methods. Health-related complaints were examined and several functional tests, such as expirogram, olfactory threshold test, acoustic rhinometry, nasal lavage with biomarkers of inflammation (eosinophilic cationic proteins (ECP), myeloperoxidase (MPO), tryptase, albumin) and allergy tests were performed. The different tests were correlated to nasal complaints and to each other. Results. Nasal blockage complaints were more common among farmers; overall, nasal polyps were more frequent in grain producers. Objective parameters showed more pronounced mucosal swelling in farmers and higher concentrations of ECP in nasal lavage compared with controls. Lung function, olfactory threshold, atopy frequency and allergen-specific IgE to the storage mite Lepidoglyphus destructor did not differ between farmers and controls. Mucosal swelling measured with acoustic rhinometry was more pronounced in subjects with nasal complaints, hypersensitivity, nasal polyps and symptoms from lower airways. There was a correlation between biomarkers in nasal lavage (MPO, albumin and ECP).     

The efficacy of a voice training program: a case–control study in China

The objective of this study was to design a voice training program for Chinese speakers, and to evaluate its efficacy. It was a prospective, randomized, case–control study practiced in three middle schools in Beijing, China. Teachers in the treatment group received voice training for 4 weeks, whereas the control group subjects received no treatment. The voice training program, which was adapted for Chinese, contained vocal hygiene education and group voice training. The outcome was assessed by voice handicap index (VHI), maximum phonation time (MPT) and acoustic analysis parameters including, noise to harmonic ratio (NHR), jitter and shimmer. The results showed that at the onset of the study, no significant differences were found between the subjects in two groups for VHI, MPT and NHR. VHI of treatment group subjects who received voice training decreased significantly, whereas VHI of control group subjects showed no significant change. Treatment group MPT was significantly increased after training, whereas the control group one presented no significant change during the same period. NHR in treatment group decreased significantly after training, whereas the one in control group showed no significant change. There were no significant changes for jitter and shimmer in both groups before and after the study. So we conclude that the voice training program is suitable to treat voice diseases, particularly for middle school teachers. This result provided reliable evidence for carrying out further voice training in China.     

Varied manifestations of Rosai-Dorfman disease and its management — a case report and review of literature

Rosai Dorfman disease or sinus histiocytosis is a rare, idiopathic, benign and self-limiting histiocytic proliferative disorder, usually seen in younger patients. It most commonly involves the cervical lymph nodes, with a predominant infiltration of sinusoidal histiocytes and classically presents with massive cervical lymhadenopathy. Extranodal disease occurs in about 43% of cases and produces different signs and symptoms depending upon its location. We report a case of this disorder in 22-year-old male with initial isolated involvement of bilateral nasal cavity and paranasal sinuses with subsequent involvement of cervical lymph nodes and skin. The clinical presentation, histologic characterstics, radiographic findings and treatment of the disease are discussed.     

A case against spiral ligament atrophy as a cause of Ménière's disease

The etiology of Ménière's disease and endolymphatic hydrops has been very difficult to determine. In order to develop rational therapies for patients with Ménière's disease the etiology needs to be established. In the current study an examination was made of the spiral ligament in temporal bones from individuals with Ménière's and endolymphatic hydrops and normal control temporal bones. No evidence of atrophy in either the length or width or the fibrocyte cell density was seen in the temporal bones with Ménière's disease. The role of the spiral ligament in the development of endolymphatic hydrops is still unclear.     

Ménière's disease: a long-term follow-up study of bilateral hearing levels

CONCLUSION: It is suggested that a holistic factor - such as psychological stress--is involved in Menière's disease (MD) and that the pathological changes in MD may be a result not only of endolymphatic hydrops but also of disorders affecting the entire cochlea. PATIENTS AND METHODS: Changes in the hearing of 51 patients with unilateral MD were investigated to ascertain the correlation between changes in hearing loss (a) in the affected ear vs the contralateral ear and (b) at low frequencies vs high frequencies. RESULTS: About half of the MD patients showed a significant positive correlation between the hearing level in the affected ear and that in the contralateral ear and also between the average hearing level at lower frequencies and that at 8 kHz. These tendencies were more pronounced in patients with severe fluctuation of hearing and/or severe hearing loss.     

Stress as a trigger of attacks in Menière's disease. A case-crossover study

BACKGROUND: Menière's disease is defined as the presence of recurrent, spontaneous episodic vertigo, hearing loss (HL), aural fullness, and tinnitus. The occurrence of attacks is unpredictable. The etiology is still unknown, but the disease has a pathologic correlate in hydropic distension of the endolymphatic system. Earlier studies have shown increased incidence of stress on the same day as vertigo attacks, but it has not been determined whether stress occurring on the day of the vertiginous episode came before or after the onset of the vertigo. METHODS: A case-crossover study including 46 patients with active Menière's disease. MAIN OUTCOME MEASURE: Relative risks with 95% confidence intervals (CI). FINDINGS: During the study period, 153 Menière's attacks were reported. Twenty-four (52%) of the 46 patients reported attacks. Twelve of the 153 (8%) attacks occurred within 3 hours after exposure to emotional stress. The relative risk of having an attack was 5.10 (95% CI 2.37-10.98) during 3 hours after being exposed to emotional stress. Twenty-nine percent of the patients with attacks had at least one attack after exposure to emotional stress. For mental stress, the relative risk was 4.16 (95% CI 1.46-11.83) and the hazard period 1 hour, but only five attacks were exposed. No excess risk was found after physical stress. INTERPRETATION: Being exposed to emotional stress increases the risk of getting an attack of Menière's disease during the next hour, and the hazard period is possibly extended up to 3 hours.     

Castleman’s disease (giant lymph node hyperplasia) of the neck: a case report

Castleman’s disease (giant lymph node hyperplasia) is an uncommon cause of neck mass. Its cause and pathogenesis are still unknown. Giant lymph node hyperplasia (GLH) usually presents as an asymptomatic solitary mass and can occur anywhere in the head and neck. Diagnostic test results are always inconclusive. Excision and histopathological evaluation are the only ways to make a definitive diagnosis. The disease is curable by surgical excision. A case of GLH presenting as a solitary neck mass in a 68-year-old man is reported. There has been no recurrence during about 7 months. Received: 25 May 1999 / Accepted: 7 September 2000     

P-100 in the treatment of Ménière's disease: a clinical study

Patients suffering from Ménière's disease are particularly sensitive to negative pressure in the middle ear. For example, attacks of vertigo can be triggered by a descent in an aircraft when ventilation of the middle ear can become critical. Positive-pressure pulse treatment has been shown to have a beneficial effect on the symptomatology and is now a true alternative in the treatment of Ménière's disease. In this study, we compared two devices that produced positive-pressure pulses delivered to the inner ear via the external ear canal and after the insertion of a middle-ear ventilation tube. Both devices (Meniett and P-100) were equally successful and confirmed that positive-pressure pulse treatment is a true alternative to current treatment modalities. However, the P-100 is the preferred device, particularly for its convenience of use and its cost, which is considerably lower.     

Medical management of Ménière’s disease: a 10-year case series and review of literature

Ménière’s disease is frequent in our area. It is a disabling pathology that affects the patient’s quality of life. Its etiology and pathophysiology remain unclear and there are some therapeutic alternatives with controversial results. We present our series and treatment protocol for Ménière’s disease and discuss the current evidence regarding its medical treatment. In the last 10 years, 252 patients were diagnosed with Ménière’s disease based on their symptoms, and the results of tonal audiometry, videonystagmography and caloric stimulation tests. All cases received medical treatment according to our management protocol. Ménière’s disease is more frequent in women (54.76%); 83.73% of cases are unilateral. Among these, vertigo was observed in 93.7%, hearing loss in 88.1%, tinnitus in 86.1% and pathologic caloric stimulation tests in 71% (88.27% with a reduced vestibular response). A 5-year follow-up was performed in 89.6% of the patients. Subjective improvement as reported by patients was achieved in 94.4% of the cases. There is great controversy about the medical treatment of Ménière’s disease. We found no evidence for the most adequate medical treatment among the different alternatives described in literature. The patient’s acceptance and understanding of the disease is very important.     

Intratympanic steroid perfusion for the treatment of Ménière's disease: a retrospective study

We conducted a retrospective outcomes review of the charts of 22 patients with Ménière's disease who were treated with intratympanic perfusion of methylprednisolone and/or dexamethasone. Outcomes were determined by subjective assessment of vertigo control and by objective changes in audiometric pure-tone average (PTA) and speech discrimination score (SDS). These evaluations were made at the first postperfusion visit (short term) and at least 12 months later (long term). In the short term, 12 patients (54.5%) achieved vertigo control, 4 patients (18.2%) demonstrated a greater than 10-dB improvement in PTA, and 1 patient (4.5%) experienced an increase in SDS of at least 15%. In the long term, the corresponding numbers of patients were 4 (18.2%), 2 (9.1%), and 1 (4.5%). The level of hearing ultimately deteriorated in 9 patients (40.9%). These findings suggest that intratympanic steroid perfusion does not result in any long-term alleviation of vertigo or hearing loss. However, the short-term alleviation of vertigo seen in approximately half of these patients suggests that this treatment may be useful for the temporary relief of symptoms of Ménière's disease.     

When is a biopsy justified in a case of relapsing polychondritis?

Relapsing polychondritis (RP) is a relatively rare rheumatic condition of unknown aetiology. It is characterized by recurrent episodic inflammation of cartilaginous structures (nose, ear and trachea). The clinical diagnosis of polychondritis can frequently be made with confidence in the absence of histological confirmation. A 61-year-old diabetic man, with bilateral relapsing aural inflammation, left ear deafness with tinnitus and pain at the sternocostal junctions is reported. After clinical diagnosis of relapsing polychondritis steroid therapy was started. An ear cartilage biopsy was performed confirming the clinical diagnosis. Subsequently soft tissue infection occurred at the operation site. The abscess was drained and oral ciprofloxacin was given with complete resolution of the infection over 30 days. As the infection is the main cause of death in these patients, we analyse whether biopsy is absolutely necessary for the diagnosis of RP in some patients.