Clinical analysis for 15 patients with pulmonary Langerhans cell histiocytosis and literature review / 中南大学学报(医学版)

OBJECTIVES@#Pulmonary Langerhans cell histiocytosis (PLCH) is a clonal disease, characterized by proliferation of Langerhans cells that derived from bone marrow infiltrating the lungs and other organs. Due to the rarity of the disease, the current understanding of the disease is insufficient, often misdiagnosed or missed diagnosis. This study aims to raise clinicians' awareness for this disease via summarizing the clinical characteristics, imaging features, and treatment of PLCH.@*METHODS@#We retrospectively analyzed clinical and follow-up data of 15 hospitalized cases of PLCH from September 2012 to June 2021 in the Second Xiangya Hospital of Central South University.@*RESULTS@#The age of 15 patients (9 men and 6 women, with a sex ratio of 3 to 2) was 21-52 (median 33) years. Among them, 8 had a history of smoking and 5 suffered spontaneous pneumothorax during disease course. There were 3 patients with single system PLCH and 12 patients with multi-system PLCH, including 7 patients with pituitary involvement, 7 patients with lymph node involvement, 6 patients with bone involvement, 5 patients with liver involvement, 2 patients with skin involvement, 2 patients with thyroid involvement, and 1 patients with thymus involvement. The clinical manifestations were varied but non-specific. Respiratory symptoms mainly included dry cough, sputum expectoration, chest pain, etc. Constitutional symptoms included fever and weight loss. Patients with multi-system involvement experienced symptoms such as polyuria-polydipsia, bone pain, and skin rash. All patients were confirmed by pathology, including 6 by lung biopsy, 3 by bone biopsy, 2 by lymph node biopsy, and 4 by liver, skin, suprasternal fossa tumor, or pituitary stalk biopsy. The most common CT findings from this cohort of patients were nodules and/or cysts and nodular and cystic shadows were found in 7 patients. Three patients presented simple multiple cystic shadows, 3 patients presented multiple nodules, and 2 patients presented with single nodules and mass shadows. Pulmonary function tests were performed in 4 patients, ventilation dysfunction was showed in 2 patients at the first visit. Pulmonary diffusion function tests were performed in 4 patients and showed a decrease in 3 patients. Smoking cessation was recommended to PLCH patients with smoking history. Ten patients received chemotherapy while 2 patients received oral glucocorticoid therapy. Among the 11 patients with the long-term follow-up, 9 were in stable condition.@*CONCLUSIONS@#PLCH is a neoplastic disease closely related to smoking. The clinical manifestations and laboratory examination are not specific. Pneumothorax could be the first symptom which is very suggestive of the disease. Definitive diagnosis relies on histology. There is no unified treatment plan for PLCH, and individualized treatment should be carried out according to organ involvement. Early smoking cessation is essential. Chemotherapy is the main treatment for rapidly progressing PLCH involved multiple organs. All diagnosed patients can be considered for the detection of BRAFV600E gene and relevant targeted therapies have been implemented recently.

Good / 中南大学学报(医学版)

A patient with thymoma associated immunodeficiency syndrome (Good's syndrome) and bronchiectasis was retrospectively analyzed. Good's syndrome is a rare condition of immunodeficiency that is characterized by thymoma and hypogammaglobulinemia. It is important to bear in mind that Good's syndrome should be included in the differential diagnosis When patients repeatedly visited for bronchiectasis or infection, we should alert to their immune state and history of thymoma. Early screening of immunological status and aggressive correction of immune deficiency are beneficial to improving the prognosis to patients with Good's syndrome.

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.

Swine-spread severe influenza-associated pneumonia: A case report and literature review / 中南大学学报(医学版)

We report and analyze the clinical data of the first case of severe pneumonia caused by influenza B virus from swine. The patient, a 62 year-old male domestic pig breeder, was admitted to hospital because of fever and muscle pain for 5 days, and anhelation for 3 days. One week before the onset of disease, the patient kept close contact with pigs. CT scan of the chest showed diffuse infiltration in both lungs. Influenza B virus antigen detection (colloidal gold method) was repeatedly positive. These all confirmed influenza B virus infection. Poor appetite, weight loss, cough, poor spirit of pigs, positive influenza B virus antigen test occurred in the pig, while the patient had no history of exposure to influenza B-infected patients. It was likely that influenza B virus was transmitted from domestic pigs to the patient by droplets or close contact. Influenza B virus epidemics always occur every five or six years a time, and patients and carriers are the main source of infection. After searching the Pubmed, Web of science, Elsevier, Wanfang, and CNKI databases, it was found that although there were many studies on influenza B virus infecting seals, ferret, domestic pigs, guinea pigs, and other animals, there was no case report for animal-to-human infection. It is the first case report of type B influenza virus transmission from domestic pigs to people in the world, which provides a new direction for the research and prevention of influenza B virus.

Clinical characteristics of histoplamosis in 8 patients: case report and literature review / 中南大学学报(医学版)

To explore the clinical characteristics, imaging manifestation, diagnosis and treatment for histoplasmosis and to improve therapeutic level, we retrospectively analyzed the clinical data of 8 patients with biopsy-confirmed histoplasmosis from 2004 to 2014 in the Second Xiangya Hospital of Central South University and reviewed relevant literatures. The main clinical symptoms of histoplasmosis included fever, cough, expectoration, chest pain, blood-stained sputum, lymphadenectasis, etc. The major lung imaging features were mass, node or pneumonia-like performance. No case was diagnosed as histoplasimosis firstly. Four patients whose imaging manifestations were focal pulmonary lesion received lobectomy of lung lesions or wedge resection. Clinical and imaging manifestations in 3 patients, who treated with amphotericin B or its liposomal, itraconazole or fluconazole, were improved. The clinical symptoms and imaging findings of histoplasmosis are nonspecific. It is easy for the physicians to misdiagnose histoplasmosis as bacterial infection, lung cancer, tuberculosis lymphoma, etc. Therefore, it is significant and necessary to carry out multiple biopsies combined with multiple etiological examinations for patients with difficult diagnosis.

Diagnosis and treatment for 2 cases of tuberous sclerosis complex with pulmonary lymphangioleiomyomatosis / 中南大学学报(医学版)

To improve the diagnosis and treatment for tuberous sclerosis complex (TSC) with pulmonary lymphangioleiomyomatosis, a retrospective analysis was performed based on the clinical data of 2 patients with such disease. Both of them have typical thin-walled cystic lesion throughout the lung field, renal angioleiomyolipoma, and various degrees of skin lesions. Central nervous system is involved in one patient. Lesions in the lung and kidney in one patient were improved significantly after 5 months of rapamycin treatment. The clinical phenotypes were diverse in TSC patients. The CT imaging showed typical characteristics when the lung was invaded by the tumor. When a patient was diagnosed as pulmonary lymphangioleiomyomatosis, we should pay attention to the clinical screening of TSC. Rapamycin is an effective and safe treatment for this disease.

Clinical characteristics of primary pulmonary cryptococcosis in 28 patients / 中南大学学报(医学版)

OBJECTIVE@#To determine the clinical characteristics, causes of pre-operative misdiagnosis and therapy of pulmonary cryptococcosis.@*METHODS@#We retrospectively analyzed the clinical data of 28 patients suffering from pulmonary cryptococcosis from 2008 to 2013 in the Second Xiangya Hospital of Central South University. All patients were diagnosed pathologically.@*RESULTS@#Of the 28 patients, 19 had no clear host factors. No patient was exposed to pigeons recently. The imaging findings showed that most patients had solitary, multiple nodules, masses, and patches. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) showed abnormal accumulation of fluorodeoxyglucose. Seven patients demonstrated malignancy and 1 demonstrated tuberculosis. None was considered as pulmonary fungus diseases. Microscopically, cryptococcosis granuloma formation was found in all patients and cryptococcosis neoformans were detected by Periodic acid-schiff and Grocott methenamine silver staining methods in the histopathological examination, respectively. Twenty-seven patients underwent lobectomy, and 1 had the medical antifungal drugs treatment. During the follow-up, symptoms in only 1 patient were not controlled.@*CONCLUSION@#Most pulmonary cryptococcosis patients have no evident immunocompromise. Clinical presentation of pulmonary cryptococcosis varies and is often related to the immune status of patients. Radiological manifestation of pulmonary cryptococcosis is indistinguishable from malignant tumor, and even 18F-FDG-PET imaging does not help to get a clear diagnosis. After surgical resection of the lung, systemic antifungal treatment is still necessary for special population. Systemic therapy of both fluconazole and itraconazole is classic choice for pulmonary cryptococcosis.

Treatment of pulmonary and retroperitoneal lymphangioleiomyomatosis with rapamycin: a case presentation and literature review / 中南大学学报(医学版)

To improve the diagnosis and treatment of pulmonary lymphangiomyomatosis, clinical data for the first successfully treated case of pulmonary and retroperitoneal lymphangiomyomatosis in our hospital has been comprehensively analyzed, and the relevant literature has been reviewed. A 45-year-old Han Chinese woman initially presented six months ago with increasing shortness of breath on exertion and was admitted to our hospital after four days of chest pain. Admission examination revealed chylothorax, interstitial lung disease, and enlarged retroperitoneal lymph nodes. The patient was finally diagnosed with pulmonary and retroperitoneal lymphangiomyomatosis based on laparotomy examination and biopsy of the retroperitoneal lymph nodes. After six months of rapamycin treatment, the symptoms - lung function, arterial blood gas, and imaging of the patient- were improved significantly. Pulmonary lymphangiomyomatosis clinically manifests as progressive dyspnea, recurrent pneumothorax, and chylothorax, and can be diagnosed by its characteristic features in high-resolution computed tomographic images or pathological examination. The successful treatment of pulmonary lymphangiomyomatosis with rapamycin brings new hope to those afflicted with this disease.

Clinical analysis of critically ill patients with H1N1 influenza / 中南大学学报(医学版)

Objective To determine the clinical characteristic, main treatment, and prognosis for the sake of more effective treatments for critically ill patients with H1N1 influenza. Methods Eight critically ill patients with H1N1 influenza in intensive care unit were retrospectively studied, including clinical characteristics, indexex of correlation, and prognosis. Results The acute physiology and chronic health evaluationⅡ score was 19.0±7.8. Five patients died, 4 of whom were caused by respiratory failure. The number of platelets in dead patient was lower than that in healing and improved ones(χ2=8.000,P＜0.05).All the 4 patients treated with glucocorticoid died, 5 out of the 6 patients received invasive mechanical ventilation rather than noninvasive mechanical ventilation, and 3 of them who complicated barotraumas in the lung died at last. Conclusion Critically ill patients with H1N1 influenza have high mortality. Respiratory failure is the main cause of death. Critically ill patients with H1N1 influenza should not be treated with glucocorticoid. Patients who need mechanical ventilation should be treated with invasive mechanical ventilation with low tidal volume and low positive end-expiratory pressure.

Effect of nerve growth factor on secretion of Th1/Th2 cytokines in asthmatic rats / 基础医学与临床

Objective To study the effect of nerve growth factor(NGF) on secretion of Th1/Th2 cytokines. Methods (1)32 SD rats were divided into 4 groups randomly: asthma(the asthmatic models were established), control, NGF, anti-NGF-antibody groups. After 14 days the lung tissues were examined for pathologic changes by HE staining. NGF in the lungs was determined by enzyme-linked immunosorbent assay (ELISA) and immunohistochemistry assay。The level of interferon-?(IFN-?, one of the Th1 cytokines) and interleukin-4 (IL-4, one of the Th2 cytokines) was detected by ELISA. Results In the asthma group, more inflammatory cells were detected in the lungs. more NGF-immunoreactive infiltrating cells and higher levels of NGF(P

Study on the effect of nerve growth factor on Th1/Th2 cell immune imbalance in asthmatic rats / 中国实用内科杂志

Objective To study the effect of nerve growth factor on airway inflammation and Th1/Th2 cells immune imbalance.Methods(1)32 SD rats were divided into 4 groups randomly:asthma,control,NGF,anti-NGF-antibody groups.The asthmatic model were established by inhalation and injection of ovalbumin.(2)We investigated the expression of NGF mRNA in the lungs in asthma and control groups by reverse transcription-polymerase chain reaction(RT-PCR).(3)In the lungs of the four groups,the expression of interferon-?(IFN-?,one of the Th1 cytokines),interleukin-4(IL-4,one of the Th2 cytokines)mRNA was detected by RT-PCR too.Results(1)As compared with the control group,NGF mRNA was significantly enhanced in the asthma group[(90.4?7.6)% vs(51.8?12.3)%,P

Efficacy and Adverse Effects of Methotrexate in the Treatment of Steroid-dependent Asthma / 中国医师杂志

Objective To investigate the efficacy and adverse effects of methotrexate(MTX) in the treatment of steroid-dependent asthma.Methods By a randomized, double-blind scheme,50 patients with steroid dependent asthma were divided into two group:MTX group and placebo group:MTX(15mg?week -1 ) and placebo agent were given respectively for 12 weeks.The dose of oral steroid daily,symptom scores, peak flow rates, spirometry, ?-agonist frequency were monitored before and after treatment. During the treatment,frequency of asthma attack,adverse events of MTX were noted,and blood routine examination,liver function test,kidney function test were performed.Results 45 subjects completed the study. Compared with before treatment,the MTX significantly decreased daily steroid dosage(14 38?4 90mg?d -1 versus 10 63?5 77mg?d -1 ,P0 05).No serious adverse effects of MTX were found during the study.Conclusion We consider that low-dose MTX may be beneficial for steroid-dependent asthma and that this therapy is well tolerated by patients.