The male patient was referred to the hospital at 44 days old due to dyspnea after birth and inability to wean off oxygen. His brother died three days after birth due to respiratory failure. The main symptoms observed were respiratory failure, dyspnea, and hypoxemia. A chest CT scan revealed characteristic reduced opacity in both lungs with a "crazy-paving" appearance. The bronchoalveolar lavage fluid (BALF) showed periodic acid-Schiff positive proteinaceous deposits. Genetic testing indicated a compound heterozygous mutation in the ABCA3 gene. The diagnosis for the infant was congenital pulmonary alveolar proteinosis (PAP). Congenital PAP is a significant cause of challenging-to-treat respiratory failure in full-term infants. Therefore, congenital PAP should be considered in infants experiencing persistently difficult-to-treat dyspnea shortly after birth. Early utilization of chest CT scans, BALF pathological examination, and genetic testing may aid in early diagnosis.
Infant ; Infant, Newborn ; Humans ; Male ; Bronchoalveolar Lavage/adverse effects* ; Pulmonary Alveolar Proteinosis/pathology* ; Dyspnea/etiology* ; Respiratory Insufficiency

Objective: To analyze the clinical characteristics and prognosis of 6 children with idiopathic interstitial pneumonia (IIP). Methods: This retrospective study analyzed the clinical manifestations, examinations, treatment and prognosis of 6 children with IIP who were hospitalized in Children's Hospital of Nanjing Medical University from January 2015 to March 2020. Results: Of the 6 children, 2 were males and 4 were females, aged 4.8 to10.6 years. All children had a subacute onset, and presented with cough, shortness of breath and cyanosis. The lung high-resolution CT (HRCT) showed diffuse patchiness in bilateral lung fields in all the children and reticular pattern in 2 cases. Pulmonary function test found moderate to severe mixed defect in 5 children. Lung biopsy was performed in 4 children. All of the 6 children were treated with systemic glucocorticoids, of whom 2 cases had additional inhaled glucocorticoids. Four children were finally diagnosed as cryptogenic organizing pneumonia (COP), whose lung HRCT return to normal in 1-11 months. Two children were finally diagnosed as nonspecific interstitial pneumonia (NSIP), and had long-term residual fibrosis on lung HRCT. The 6 children were followed up for 1 year to 6 years and 5 months after discontinuation of systemic glucocorticoids, and all had no recurrence. Conclusions: The clinical characteristics of IIP in children are subacute onset presented with cough, shortness of breath, cyanosis and diffuse patchiness in bilateral lungs on HRCT. The common subtypes of IIP in children are COP and NSIP. Systemic glucocorticoid is effective for IIP in children and there is a good prognosis overall.
Child ; Cough/etiology* ; Cryptogenic Organizing Pneumonia ; Cyanosis/pathology* ; Dyspnea/pathology* ; Female ; Glucocorticoids/therapeutic use* ; Humans ; Idiopathic Interstitial Pneumonias/pathology* ; Lung/pathology* ; Male ; Retrospective Studies

dyspnea. Pathology following a lung biopsy revealed typical HDLI finding which was determined to be due to humidifier disinfectant exposure. Patient of case 2 used humidifier disinfectant from 2001 to 2008 for about 3 months each winter. The patient's cough and sputum production symptoms began in December of 2007. The patient was admitted to the respiratory medicine department due to worsening dyspnea. Pathology following a lung biopsy revealed typical HDLI finding. This was determined to have been caused by humidifier disinfectant exposure.CONCLUSIONS: Because the typical radiologic findings associated with HDLI can improve over time, it is necessary to consider the revision of current diagnostic criteria that the presence of radiologic findings is important.]]>
Adult ; Biopsy ; Cough ; Disinfectants ; Dyspnea ; Humans ; Humidifiers ; Intensive Care Units ; Judgment ; Korea ; Lung Diseases ; Lung Injury ; Lung ; Pathology ; Pulmonary Medicine ; Sputum ; Thorax

We present the case of a 48-year-old woman who complained of sustained dyspnea and newly developed dyspnea, who then suddenly and unexpectedly expired during bronchoscopy. On postmortem examination, the deceased had advanced gastric cancer as a primary tumor. Frequent lymphatic tumor emboli were observed with some pulmonary lymphangitic carcinomatosis (PLC), and pulmonary tumor thrombotic microangiopathy (PTMA). PLC and PTMA are lethal forms of pulmonary metastasis, and PTMA can lead to sudden death. The characteristic findings of PLC and PTMA in the deceased were not predominant, however, and the clinical manifestation was not acutely deteriorating. These findings are, therefore, insufficient to explain the deceased's sudden death. Clinically, the deceased manifested hypoxemia, bradycardia and cardiac arrest during bronchoscopy and then soon expired, suggesting the possibility of cardiovascular complication related to bronchoscopy. Despite several limitations, we assumed that the sudden unexpected death might have been induced by cardiovascular complications related to bronchoscopy and due to the underlying pathologic condition by PLC and PTMA.
Anoxia ; Autopsy ; Bradycardia ; Bronchoscopy ; Carcinoma ; Death, Sudden ; Dyspnea ; Female ; Forensic Pathology ; Heart Arrest ; Humans ; Middle Aged ; Neoplasm Metastasis ; Stomach Neoplasms ; Thrombotic Microangiopathies

Idiopathic nonspecific interstitial pneumonia (NSIP) is one of the varieties of idiopathic interstitial pneumonias. Diagnosis of idiopathic NSIP can be done via multidisciplinary approach in which the clinical, radiologic, and pathologic findings were discussed together and exclude other causes. Clinical manifestations include subacute or chronic dyspnea and cough that last an average of 6 months, most of which occur in non-smoking, middle-aged women. The common findings in thoracic high-resolution computed tomography in NSIP are bilateral reticular opacities, traction bronchiectasis, reduced volume of the lobes, and ground-glass opacity in the lower lungs. These lesions can involve diffuse bilateral lungs or subpleural area. Unlike usual interstitial pneumonia, honeycombing is sparse or absent. Pathology shows diffuse interstitial inflammation and fibrosis which are temporally homogeneous, namely NSIP pattern. Idiopathic NSIP is usually treated with steroid only or combination with immunosuppressive agents such as azathioprine, cyclophosphamide, cyclosporine, and mycophenolate mofetil. Prognosis of idiopathic NSIP is better than idiopathic pulmonary fibrosis. Many studies have reported a 5-year survival rate of more than 70%.
Azathioprine ; Bronchiectasis ; Cough ; Cyclophosphamide ; Cyclosporine ; Diagnosis ; Dyspnea ; Female ; Fibrosis ; Humans ; Idiopathic Interstitial Pneumonias ; Idiopathic Pulmonary Fibrosis ; Immunosuppressive Agents ; Inflammation ; Lung ; Lung Diseases, Interstitial ; Pathology ; Prognosis ; Survival Rate ; Traction

OBJECTIVES: A saccular cyst is defined as a dilated saccule of the larynx, filled with mucus, and is located between the false vocal cords and the thyroid cartilage. Although this uncommon laryngeal condition is benign in nature, it could lead to dyspnea, stridor, and airway obstruction, depending on its size and location. Furthermore, some saccular cysts have been associated with laryngeal carcinoma. This study aimed to characterize this rather uncommon laryngeal condition to aid in determining the proper management of this pathology. METHODS: Medical records were retrospectively reviewed of all patients with saccular cysts diagnosed and treated between 2006 and 2017 at a tertiary otolaryngologic care center. RESULTS: Seven patients with saccular cysts were identified (male:female=2:5; mean age, 34.1 years); two were pediatric patients. Surgical intervention was performed in all patients by laryngo-microsurgery using CO2 laser. There was no recurrence after the initial surgical treatment. CONCLUSION: Saccular cysts can be managed endoscopically using CO2 laser, without requiring an external approach. Therefore, an endoscopic approach should be actively considered for an optimal treatment outcome.
Airway Obstruction ; Dyspnea ; Humans ; Larynx ; Lasers, Gas ; Medical Records ; Mucus ; Pathology ; Recurrence ; Respiratory Sounds ; Retrospective Studies ; Saccule and Utricle ; Thyroid Cartilage ; Treatment Outcome ; Vocal Cords

A 23-year-old Indian man presented with shortness of breath and new-onset confusion along with a rash on his chest on Postoperative Day 2, following internal fixation of his femur fracture. Although computed tomography pulmonary angiography was negative for filling defects in the pulmonary vasculature, it showed mosaic attenuation changes with some interlobular septal thickening. Magnetic resonance imaging of the brain showed patchy signal abnormalities, predominantly in the grey-white matter junction region with extensive susceptibility artefacts, consistent with petechial haemorrhages. The laboratory work-up showed thrombocytopenia and anaemia. A diagnosis of fat embolism syndrome was established, based on the clinical presentation combined with laboratory and imaging findings. The clinical and imaging features of fat embolism syndrome are discussed.
Brain ; pathology ; Dyspnea ; Embolism, Fat ; diagnostic imaging ; Femoral Fractures ; diagnostic imaging ; Humans ; Hypoxia ; India ; Magnetic Resonance Imaging ; Male ; Pulmonary Embolism ; Thrombocytopenia ; Tomography, X-Ray Computed ; Young Adult

A 12-year-old girl presented with a history of cervical mass, and one week of throat discomfort and dyspnea. Five years ago, the patient was diagnosed as Hashimoto's thyroiditis and hyperthyroidism; she received antithyroid drug treatment, but the result was not satisfactory. B-ultrasonic showed that the size of thyroid gland was 8.1 cm×3.2 cm in the left and 8.2 cm×4.8 cm in the right. After iodine 131 combined with radiofrequency ablation (RFA) treatment, throat discomfort and recumbent breathing difficulties disappeared, and B-ultrasonic showed that the size of thyroid reduced to 2.3 cm×1.7 cm (left) and 2.8 cm×2.0 cm (right). No recurrence was observed during the two and a half years of follow-up.
Ablation Techniques ; methods ; Child ; Dyspnea ; etiology ; therapy ; Female ; Goiter ; complications ; diagnostic imaging ; pathology ; therapy ; Hashimoto Disease ; therapy ; Humans ; Hyperthyroidism ; therapy ; Iodine Radioisotopes ; therapeutic use ; Radio Waves ; therapeutic use ; Ultrasonography

Hookworm infections as well as other intestinal nematodiases are endemic in China. In this case, a 70-year-old male showed symptoms of chest tightness, shortness of breath, and both lower extremities edema. The diagnostic result was chronic renal insufficiency, chronic kidney disease (5th stage), and renal anemia at first. Then, he received treatment with traditional drugs. However, this treatment did not help to alleviate the symptoms of the patient significantly. The results of gastroendoscopy showed hookworms in the duodenum, also confirmed by pathology examination. Anemia was markedly ameliorated after eliminating the parasites. The results mentioned above suggested that ancylostomiasis was the leading causes of anemia in this patient, and the etiology of anemia in uremic patients should be systematically considered. Especially when anemia could not be cured by regular treatments, rare diseases should be investigated.
Aged ; Ancylostomatoidea* ; Ancylostomiasis ; Anemia* ; China* ; Duodenum ; Dyspnea ; Edema ; Hookworm Infections ; Humans ; Lower Extremity ; Male ; Parasites ; Pathology ; Peritoneal Dialysis* ; Rare Diseases ; Renal Insufficiency, Chronic ; Thorax

Aged ; Bronchiectasis ; etiology ; pathology ; Comorbidity ; Dyspnea ; etiology ; pathology ; Female ; Humans ; Male ; Middle Aged ; Pulmonary Disease, Chronic Obstructive ; complications ; pathology ; Smoking ; adverse effects