Characteristics and feasibility of ambulatory respiratory assessment of paediatric neuromuscular disease: an observational retrospective study.

PURPOSE: To investigate the characteristics of respiratory involvement in Chinese paediatric neuromuscular disease (NMD) at early stage and to explore convenient monitoring methods. MATERIALS AND METHODS: Children with NMD (age < 18) diagnosed at a multidisciplinary joint NMD clinic at Peking University First Hospital from January 2016 to April 2021 were included. Overnight polysomnography (PSG) and pulmonary function test (PFT) data were analysed, and the characteristics of four groups: congenital muscular dystrophy (CMD), congenital myopathy, spinal muscular atrophy, and Duchenne muscular dystrophy (DMD) were compared. RESULTS: A total of 83 children with NMD were referred for respiratory assessment, of who 80 children underwent PSG; 41 performed spirometry and 38, both. The duration of pulse oxygen saturation (SpO2) <90% over apnoea and hypopnoea index (AHI) was lowest in DMD and significantly different from CMD (p = 0.033). AHI was positively correlated with the oxygen desaturation index (ODI) (r = 0.929, p = 0.000). The peak expiratory flow (PEF) were positively correlated with forced vital capacity (FVC), both as actual values and percent pred, respectively (r = 0.820, 0.719, p = 0.000). ROC derived sensitivity and specificity of prediction of AHI > 15/h or duration of SpO2<90% ≥ 60 min from FVC <51% pred was 75.8% and 85.7%, respectively. CONCLUSIONS: AHI and hypoxia burden were independent factors in children with NMD in PSG and attention needed to be paid in both. FVC might be a daytime predictor for significant sleep-disordered breathing or hypoxia. Nocturnal consecutive oximetry with diurnal peak flow measurement may be convenient and effective for home monitoring at early stage of respiratory involvement.

Renal involvement in a silicosis patient - case report and literature review.

A 43-year-old Chinese man with a silicosis history was admitted to our hospital due to bilateral lower extremity edema for 1 year, exacerbating with hematuria for 2 months. He started working as a coal miner 30 years ago, and was diagnosed as silicosis 3 months ago. Lab tests revealed hematuria 3+, proteinuria 3+, and a serum creatinine value 2.47 mg/dl on routine check. He was diagnosed with focal proliferative IgA nephropathy (IgAN) and acute tubulo-interstitial nephritis by renal biopsy. He was treated with corticosteroids and got a remission 4 months later. Immunohistochemical staining showed the deposition of macrophage receptor with collagenous structure (MARCO), nod-like receptor pyrin domain-containing-3 (NLRP3), Caspase-1, apoptosis-associated speck (ASC), interleukin (IL)-1ß, and IL-18 in both glomerular and tubulo-interstitial areas. We proposed that the silicon exposure could be related to his kidney disease in the patient and NLRP3 mediated inflammation might be involved in its pathogenesis which needs further explorations.

Severe pneumonia caused by adenovirus 7 in pregnant woman: Case report and review of the literature.

We report a rare case of adenoviral pneumonia in a previously healthy pregnant woman at 26(+4) weeks' gestation. She presented with persistent high fever, cough for 5 days, and developed progressive dyspnea with hypoxemic respiratory failure and bilateral pulmonary infiltrates with pleural effusions. Aggressive supportive care and timely obstetrical management saved the mother and prevented preterm delivery and fetal anomaly.

Use of 6-minute walk test for assessing severity of interstitial lung disease: an observational study.

BACKGROUND: The 6-minute walk test (6MWT) is a potential tool for assessing the severity of interstitial lung disease (ILD). OBJECTIVES: To explore the relationship between 6MWT results and traditional measures including pulmonary function and chest computed tomography(CT) and to determine factors that might influence the 6-minute walk distance (6MWD). METHODS: Seventy-three patients with ILD were enrolled at Peking University First Hospital. All patients underwent 6MWT, pulmonary CT, and pulmonary function tests and their correlations were analyzed. Multivariate regression analysis was used to identify factors that might impact 6MWD.  Results: Thirty (41.4%) of the patients were female and the mean age was 66.1 ± 9.6 years. 6MWD was correlated with forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC), total lung capacity (TLC), diffusing capacity for carbon monoxide (DLCO) and DLCO%pred. The decrease in oxygen saturation (SpO2) after the test was correlated with FEV1%pred, FVC%pred, TLC, TLC%pred, DLCO, DLCO%pred and the percentage of normal lung calculated by quantitative CT. The increase in Borg dyspnea scale was correlated with FEV1, DLCO and the percentage of normal lung. The backward multivariate model (F = 15.257, P < 0.001, adjusted R2 = 0.498) indicated that 6MWD was predicted by age, height, body weight, increase in heart rate, and DLCO. CONCLUSIONS: The 6MWT results were closely correlated with pulmonary function and quantitative CT in patients with ILD. However, in addition to disease severity, 6MWD was also influenced by individual characteristics and the degree of patient effort, which should thus be considered by clinicians when interpreting 6WMT results.

[Retrospective analysis of relationship between radiological features and prognoses of pneumocystis pneumonia in non-AIDS immunocompromised patients].

OBJECTIVE: To elucidate the relationship between the radiological features and prognoses of pneumocystis pneumonia (PCP) in non-acquired immunodeficiency syndrome (non-AIDS) immunocompromised patients. METHODS: Retrospective analyses were performed for the radiological features and prognoses of 36 non-AIDS immunocompromised PCP patients hospitalized at Peking University First Hospital from April 2006 to April 2010. There were 24 males and 12 females with an average age of (51.7 ± 18.9) years. RESULTS: All patients underwent chest photographic examinations and chest computed tomography (CT) was performed on 29 of them. The overall case fatality rate (CFR) of 36 patients was 33.3% (12/36). At the start of anti-PCP treatment, there were normal chest radiography (n = 7), bilateral diffuse infiltration (n = 21), bilateral diffuse consolidation (n = 8), aerothorax, mediastinal emphysema or pneumohypoderma (n = 6) and numbers of deceased patients (n = 0, 8, 5 and 5 respectively). The CFRs of the patients with normal chest radiography and aerothorax, mediastinal emphysema or pneumohypoderma were both statistically significant as compared with the overall rate (P < 0.05). At the start of anti-PCP treatment, there were bilateral diffuse ground glass opacities (GGO) (n = 29), "mosaic sign" (n = 19), air bronchogram (n = 19), pulmonary consolidation (n = 17), bilateral pleural effusion (n = 7), thickened lobular septa (n = 6), cystic lesions (n = 5), aerothorax, mediastinal emphysema or pneumohypoderma (n = 4) and numbers of deceased patients (n = 8, 5, 5, 5, 2, 2, 2 and 4 respectively). The CFRs of the patients with aerothorax, mediastinal emphysema or pneumohypoderma were statistically significant as compared with the overall rate (P < 0.05). CONCLUSIONS: The major radiological features of PCP in non-AIDS immunocompromised patients are bilateral diffuse infiltration. And bilateral diffuse GGO on chest CT scans are usually accompanied with "mosaic" sign, air bronchogram or pulmonary consolidation. The prognoses are excellent for the patients with normal chest radiograph and poor for those with aerothorax, mediastinal emphysema or pneumohypoderma.

Characteristics of unilateral main bronchus obstruction and differentiation from chronic obstructive pulmonary disease by spirometry.

BACKGROUND: Pattern of flow-volume (F-V) loop in unilateral main bronchus obstruction (UMBO) is under-represented and sometimes misinterpreted as chronic obstructive pulmonary disease (COPD). METHODS: A cross-sectional study was performed among patients with UMBO and COPD confirmed by bronchoscopy, radiographic imaging and spirometry from 2006 to 2019. Data were extracted from electronic medical records. Spirometry data and flow-volume curves (F-V curves) were analyzed. Expiratory F-V curve was classified as monophasic or biphasic according to the absence or presence of breakpoint separating two distinct slopes. Propensity score method was used to reduce the selection bias, and logistic analysis in combination with decision tree approach was performed to explore the differences among groups. RESULTS: Fifty-six patients with UMBO, 121 individuals with COPD and 68 healthy subjects were included. Typical biphasic expiratory F-V curve was observed in 57.1% in UMBO group, especially of grade II (stenosis was 51-90%), and in 46.3% in COPD group, while biphasic inspiratory curve presented in 7.1% of UMBO, and none in COPD. In UMBO, breakpoints tended to appear gradually and smoothly between MEF75 and MEF50, whereas in COPD they often occurred abruptly and rigidly, ahead of MEF75. CONCLUSIONS: The characteristics of F-V curve, apart from biphasic pattern, the location and configuration of breakpoint in expiratory curve, seemed to be important features of UMBO, which might help to differentiate them from COPD. More data is needed to validate these findings.

[A case report of lymphomatoid granulomatosis and review of the literature].

To explore the clinical manifestation of lymphomatoid granulomatosis (LG) and the early diagnosis. Retrospective analyses of the clinical features of LG and pathology features by open lung biopsies were conducted. The patient was a 42-year-old female with irregular fever, and her chest X-ray and computed tomography showed nodules with cavity and pleural effusion. Open lung biopsy proved LG. LG is seldom seen in clinic. Open lung biopsy is very important for pathology diagnosis. Early diagnosis and treatment are the key to improving survival in these patients. The therapeutic effect is good.

[Idiopathic pulmonary alveolar proteinosis: report of three cases and literature review].

To describe the clinical, radiological and pathological characteristics of idiopathic pulmonary alveolar proteinosis (I-PAP) and to evaluate the methods of diagnosis and treatment. Three patients were successfully diagnosed and treated in our hospital and the literature on the subject was reviewed. Three patients, two males and one female (mean age 46 years), were diagnosed averagely in 4 months. Two severe patients presented with progressive dyspnea and type I respiratory failure, and one mild patient only with dry cough and hypoxemia. Chest X-ray radiographs all showed perihilar "butterfly" shadow and chest CT scans showed diffused ground-glass opacities (GGO), typically with "map" changes and "crazy paving" patterns. All the patients underwent bronchoscope, branchoalveolar lavage fluid (BALF) had grossly opaque and/or milky appearance and its sediment was periodic acid-Schiff stain positive. Trans-bronchoscopic lung biopsy (TBLB) specimens were obtained and under light microscopy alveoli and some of the small bronchioles were filled with eosinophilic proteinaceous material with needle-like clefts. By electron microscopy numerous cellular debris and extracellular multilamellated bodies were found. Two severe patients were successfully treated with sequential whole-lung lavage and one required repeated lavages. I-PAP is rare and prone to be misdiagnosed. The radiological features may indicate the diagnosis and examinations of TBLB and BALF can make the accurate diagnosis. Whole-lung lavage is the most effective therapy by now and granulocyte-macrophage colony-stimulating factor (GM-CSF) may be beneficial in some patients.

[H3N2 subtype of human influenza pneumonia with staphylococcal sepsis and staphylococcal pneumonia: report of one case].

To investigate the clinical characteristics, diagnosis and therapy of influenza pneumonia with staphylococcal infection. One patient in our hospital was diagnosed and the literatures on the subject were reviewed. The patient presented with high fever and dyspnea. Arterial gas analysis indicated type 1 respiratory failure. Chest X ray photographs showed bilateral infiltrations and bilateral encapsulated pleural effusions. Viral separation and culture of pharyngeal swab indicated H(3)N(2) subtype of human influenza virus. Blood, sputum and bronchoalveolar lavage fluid (BALF) cultures showed Staphylococcus aureus. Pleural effusion was complex parapneumonic pleural effusion. After the administration of anti-virus, anti-staphylococcal antibiotics and pleural cavity drainage, the patient was cured. The infection of staphylococcus aureus is a typical characteristic of influenza pneumonia, and anti-staphylococcal antibiotic therapy (with MRSA activity in MRSA endemic regions) should be initiated in hospitalized cases of influenza pneumonia. If complex parapneumonic pleural effusion or empyema complicated, we should perform pleural cavity drainage in time. The oral neuraminidase inhibitor (oseltamivir) could significantly improve prognosis.

[A case of marginal zone B-cell lymphoma of the pulmonary mucosa-associated lymphoid tissue type].

Marginal zone B-cell lymphoma of the pulmonary mucosa-associated lymphoid tissue type (pulmonary MALT-MZL), a common kind of primary pulmonary lymphoma, is rare in pulmonary malignant tumors. One patient in our hospital was diagnosed by bronchoscope and the literatures on the subject were reviewed. The patient presented with periodical fever, cough and chest pain, and antibiotic therapy had no use. Chest CT scan showed the consolidation of right middle lobe and left lower lobe with CT angiogram signs, air bronchograms and distended bronchi. Pleural effusion in the left thorax mainly consisted of monocytes. Monoclonal protein was found in the electrophoresis of serum protein. Bronchial stenosis and swollen mucosa were seen with bronchoscope. The tissue section of transbronchial lung biopsy (TBLB) specimens showed diffusedly infiltrated small lymphocytes and a lymphoepithelial lesion. CD20 was positive and CD3, CD5, CD10, CD21, CD23, bcl2, bcl6 were negative in immunohistochemical stain. The clinical manifestations of pulmonary MALT-MZL are nonspecific and misdiagnosis is common, Appropriate invasive biopsy procedures are necessary for early diagnosis. Presentations such as periodical fever, distended bronchi in pulmonary consolidation, monoclonal protein might indicate diagnosis. Treatment includes surgical resection, radiotherapy and chemotherapy. Pulmonary MALT-MZL belongs to inert lymphoma and prognosis is relatively good.

[Retrospective analysis of three cases of pulmonary mucormycosis].

OBJECTIVE: To describe the clinical characteristics of pulmonary mucormycosis and to evaluate the approaches to its diagnosis and treatment. METHODS: Three cases of pulmonary mucormycosis were successfully diagnosed by histopathologic examination of infected tissues obtained by fiberoptic bronchoscopy in this hospital. The clinical features of the cases were analyzed and the literature reviewed. RESULTS: Three patients were all farmers and two of them had had diabetic ketoacidosis. The first patient died from massive hemoptysis one day after the diagnosis was confirmed. The second patient was cured by large doses of intravenous amphotericin B (total dosage 2 g). The third patient presented with right middle bronchial stenosis after right upper lobectomy because of squamous carcinoma, and was cured by amphotericin B (1.5 g) combined with interventional therapy under fiberoptic bronchoscopy. The two patients were followed for 0.5 - 1 year without relapse. CONCLUSIONS: The mortality of pulmonary mucormycosis is high. Fiberoptic bronchoscopy is a useful diagnostic method for histopathologic examination. Successful treatment of pulmonary mucormycosis includes early diagnosis, control of underlying diseases and surgical intervention of isolated lesions combined with large dosage of amphotericin B.

[An analysis of the clinical and radiological features of bronchioloalveolar carcinoma].

OBJECTIVE: To describe the clinical and radiological features of bronchioloalveolar carcinoma. METHODS: Data of 1 050 inpatients of lung cancer, including 50 cases of pathology-proven bronchioloalveolar carcinoma, diagnosed in our hospital between 1993 and 2003, were retrospectively reviewed. The clinical information of all the patients and the HRCT findings of 31 cases of bronchioloalveolar carcinoma were analyzed. RESULTS: There was a female predominance (60%) in the 50 patients with bronchioloalveolar carcinoma. Cough was the most common presenting symptom (20/50). Twenty-four patients sought medical attention because of abnormal chest X-rays, most of which were of nodular type at the early stage. Twenty patients were completely asymptomatic at presentation. Out of the 31 cases with HRCT, eight were of the miliary type. "Bubble-like lucency" and calcifications inside nodules were present in 7 cases respectively out of the 11 cases of the nodular type. There were 9 cases each with low attenuation consolidation, pseudocavities, reticular shadowing and ground-glass opacities. The signs of "crazy paving" and "CT angiogram sign" (distinct vasculature at the background of low-attenuated consolidation at the peak of contrast scanning) were found in 2 cases. CONCLUSIONS: Bronchioloalveolar carcinoma accounted for 4.76% of lung carcinoma in our series, with a female predominance. High prevalence of asymptomatic patients at presentation and unusual long course should prompt regular chest X-ray examination. The characteristics of HRCT findings are very helpful in its diagnosis and differential diagnosis.

Phonospirometry for noninvasive measurement of ventilation: methodology and preliminary results.

We measured tracheal flow from tracheal sounds to estimate tidal volume, minute ventilation (VI), respiratory frequency, mean inspiratory flow (VT/TI), and duty cycle (TI/Ttot). In 11 normal subjects, 3 patients with unstable airway obstruction, and 3 stable asthmatic patients, we measured tracheal sounds and flow twice: first to derive flow-sound relationships and second to obtain flow-volume relationships from the sound signal. The flow-volume relationship was compared with pneumotach-derived volume. When subjects were seated, facing forward and with neck rotation, flexion, and standing, flow-volume relationship was within 15% of pneumotach-derived volume. Error increased with neck extension and while supine. We then measured ventilation without mouthpiece or nose clip from tracheal sounds during quiet breathing for up to 30 min. Normal results +/- SD revealed tidal volume = 0.37 +/- 0.065 liter, respiratory frequency = 19.3 +/- 3.5 breaths/min, VI = 6.9 +/- 1.2 l/min, VT/TI = 0.31 +/- 0.06 l/s, and TI/Ttot = 0.37 +/- 0.04. Unstable airway obstruction had large VI due to increased VT/TI. With the exception of TI/Ttot, variations in ventilatory parameters were closer to log normal than normal distributions and tended to be greater in patients. We conclude that phonospirometry measures ventilation reasonably accurately without mouthpiece, nose clip, or rigid postural constraints.