Clinical characteristics of pulmonary alveolar proteinosis / 中南大学学报(医学版)

OBJECTIVES@#Pulmonary alveolar proteinosis (PAP) is a rare disease with non-specific and various clinical manifestations, often leading to misdiagnosis. This study aims to raise the awareness of this disease via summarizing the clinical characteristics, diagnosis, and therapy of PAP.@*METHODS@#We retrospectively analyzed clinical data of 25 hospitalized cases of PAP during 2008 and 2019 in the Department of Respiratory and Critical Care Medicine of the Second Xiangya Hospital of Central South University.@*RESULTS@#Cough with unkown reason and dyspnea were common clinical manifastations of PAP. Five patients had a history of occupational inhalational exposure. Sixteen patients had typical image features including ground-glass opacification of alveolar spaces and thickening of the interlobular and intralobular septa, in typical shapes called crazy-paving and geographic pattern. Fourteen patients underwent pulmonary function tests, revealing a reduction in the diffusing capacity for carbon monoxide. The positive rate of transbronchial biopsy was 95%. Five patients received the whole lung lavage and the symptoms and imaging fcauters significantly relieved after five-years follow-up.@*CONCLUSIONS@#PAP is characterized by radiographic pattern and pathology. Transbronchial lung biopsy is effective to make diagnosis of PAP. The whole lung lavage remains a efficient therapy.

A case report / 中南大学学报(医学版)

Langerhans cell histiocytosis (LCH) is a rare and unexplained disease that can involve in any organ or system in the body and displays a variety of clinical manifestations. A 31-year-old man, who had a more than 10-year smoke history, initially presented dry cough, polydipsia and diuresis, with recurrent spontaneous pneumothorax. Pulmonary high-resolution computed tomography showed diffuse cystic and nodular lesions. Langerhans cell histiocytosis was confirmed by a transbronchial cryobiopsy. The disease is involved in the lung, pituitary, thyroid, liver, lymph node, and skin. Glucocorticoid or systemic chemotherapy is commonly used in the treatment for this disease. BRAF gene mutation inhibitor is a new direction for the treatment.

Progress in the evaluation of cognitive function in with obstructive sleep apnea by resting functional magnetic resonance / 中南大学学报(医学版)

Obstructive sleep apnea is often accompanied by functional changes in attention, alertness, long-term vision and language memory, visual space/structural competence, and executive function due to long-term intermittent hypoxia, high blood carbonate, and sleep structure disorders. In recent years, some scholars have found that the changes of brain structure, metabolism and function revealed by static functional magnetic resonance imaging (MRI) are closely related to the changes of cognitive function reflected in the cognitive function evaluation scale. In recent years, static functional magnetic resonance, especially voxel-based morphometry, diffusion tensor imaging, magnetic resonance spectroscopy and functional magnetic resonance imaging have confirmed significant changes in brain structure, metabolism and function in OSA patients, and the changes are closely related to the changes of cognitive function reflected in the cognitive function evaluation scale. It is great significance to study the mechanism of cognitive function change in OSA patients, and helpful to the early diagnosis and treatment as well as the evaluation of clinical efficacy.

A case of severe obstructive sleep apnea hypopnea syndrome with urinary and anal incontinence / 中南大学学报(医学版)

A case of a young male patient,who came to the Second Xiangya Hospital,Central South University because of snoring for 10 years and nocturnal gatism for half month,was analyzed retrospectively.He was diagnosed as obstructive sleep apnea hypopnea syndrome (OSAHS) finally.The patient had been diagnosed and treated as stroke in the local hospital,while urinary and anal incontinence were not relieved.It was a dilemma for him to be properly diagnosed and treated.Polysomnography in our hospital revealed apnea hypopnea index (AHI) at 44.7 events/h,oxygen desaturation index (ODI) at 70.8 events/h and the longest apnea time at 185 seconds while the lowest blood oxygen saturation reduced to 31％.In addition,413 events of apnea accounted for 61.2％ of the sleep time and the minimal heart rate was 23 times/min.The patient was diagnosed as severe OSAHS with hypoxia metabolic brain disease,moderate pulmonary arterial hypertension,secondary polycythemia and obesity hypoventilation syndrome finally.He received the treatment of positive airway pressure non-invasive ventilator with an average pressure at 11.7 cmH2O with reduced AHI and increased blood oxygen saturation.The urinary and anal incontinence disappeared during the first night of treatment and it has been totally resolved so far.We considered that gatism was secondary to OSAHS with severe hypoxia resulted from attenuated regulation of primary defecation in the night.Physicians should pay attention to OSAHS when accepting obese patients with nocturnal incontinence,obvious daytime sleepiness and night snoring.Urinary and anal incontinence could be completely disappeared under therapy of positive airway pressure.

Obstructive sleep apnea hypopnea syndrome and alveolar hypoventilation syndrome in motor neuron disease: A case report and literature review / 中南大学学报(医学版)

Objective:To investigate the clinical characteristics of a patient with motor neuron disease,which caused sleep-disordered breathing (SDB) and alveolar hypoventilation syndrome,and to improve the diagnosis rate for this disease.Methods:Retrospectively analyze the diagnosis and treatment process for a 52 year-old male patient,who was accepted by the Second Xiangya Hospital,Central South University because of dyspnea,shortness of breath and malaise for 4 months,and eventually was diagnosed as motor neuron disease associated with obstructive sleep apnea hypopnea syndrome and alveolar hypoventilation syndrome.In addition,we searched CNKI,Wanfang and PubMed databases to review relevant literature with keywords (motor neuron disease or amyotrophic lateral sclerosis or progressive bulbar palsy or progressive muscular atrophy or primary lateral sclerosis) AND (sleep apnea or sleep disordered breathing) from January 1990 to May 2017.Results:The major clinical manifestation of motor neuron disease induded impaired upper and lower motor neuron displayed with proximal musde weakness,musde tremor,amyotrophy,bulbar symptoms and pyramidal sign.It was a chronic,progressive disease with worse prognosis,low survival and difficult in diagnosis.Electroneuromyography was a vital way for diagnosis.Furthermore,sleep disordered breathing was common in patients with motor neuron disease,which was featured as decreased rapid eye movement sleep,increased awaking time,apnea and hypopnea.The main mechanism for sleep disordered breathing in motor neuron disease might be due to the disturbed central nervous system and paralysis of diaphragm and respiratory muscle.Moreover,the patient suffered from restrictive ventilatory dysfunction,alveolar hypoventilation and subsequent partial pressure of carbon dioxide and hypoxernia.Therefore,respiratory failure was the most frequent cause of death for patients with motor neuron disease.Non-invasive positive pressure ventilation was suggested to apply to such patients,whose forced vital capability was less than 75 percent of predicted value.Conclusion:Sleep disordered breathing is common in patients with motor neuron disease.Hence,polysomnography is suggested as a routine examination to confirm the potential complications and give timely therapy.Treatment with non-invasive positive pressure ventilation is important for patients to improve life qualit,survival rate and prognosis.