OBJECTIVETo describe the clinicopathologic features and immunophenotypes of carcinoid tumorlets in the lung with bronchiectasis, and to study the morphogenesis of these tiny tumors.

METHODSThe histopathologic characteristics of 3 bronchiectasis cases with carcinoid tumorlets, 11 bronchiectasis and 2 normal lungs were studied. Specific markers of the tiny tumors and the number of neuroendocrine cells (NECs) in the airway mucosa were immunohistochemically detected by EnVision method.

RESULTSThe tumorlets in the lungs presented as multi-focal nodules and most were displayed only under microscopy. These cells were arranged in clusters and foci of fascicles which were situated in the surrounding bronchial wall and bronchioles adjacent to bronchiectatic lesion, or in the scar tissues. The tiny tumors were consisted of short fusiform cells and small ovoid cells. Their nuclei were circular, oval or long fusiform and the cells were strongly argyrophilic on Grimelius staining. Intensive positive immunostaining for calcitonin, chromogranin A, NSE and gastrin were detected. Weak positive for CK, EMA, S-100 and focal positive for HC, ACTH, 5-HT were also observed. Proliferative NECs in airway mucosa adjacent to the tiny tumors increased significantly in number, compared with those in the airway mucosa of bronchiectasis without tumorlets and normal lungs (P < 0.001, respectively).

CONCLUSIONSThe clinicopathologic features and immunophenotypes of carcinoid tumorlets resemble carcinoid tumors. They are the early stage of carcinoid development. Their development may be related to the chronic pulmonary damage resulting in hypoxia and stimulating the proliferation of NECs. These pulmonary carcinoid tumorlets can be used as a model to study the tumorigenesis of carcinoid carcinoma of the lung.

Adult ; Aged ; Bronchiectasis ; pathology ; Carcinoid Tumor ; chemistry ; pathology ; Female ; Humans ; Immunohistochemistry ; Lung Neoplasms ; chemistry ; pathology ; Middle Aged ; Morphogenesis ; Neurosecretory Systems ; pathology

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

OBJECTIVE: To determine clinical features and diagnostic methods for primary tracheobronchial amyloidosis (TBA).
 METHODS: The clinical manifestations and diagnosis of a male patient who had been misdiagnosed for many years were described and analyzed.
 RESULTS: The patient was a 68-year-old male who complained of recurrent cough, expectoration, and progressive dyspnea for more than 30 years. He had been diagnosed with chronic bronchitis, bronchiectasis, and endobronchial tuberculosis in other hospitals and treated with antibiotics frequently and anti-tubercular agents for 3 months. Despite the treatments, the patient's symptoms were progressively worse. Finally, he came to Xiangya Hospital, Central South University, and was clearly diagnosed with primary TBA based on histopathological evidence after bronchoscopy.
 CONCLUSION TBA, a rare disease resulting from abnormal submucosal amyloid deposition in the trachea and bronchi, may display with many different symptoms. TBA is often misdiagnosed with other pulmonary diseases. The use of bronchoscopic techniques is essential for the diagnosis of TBA. Histopathology remains the gold standard for diagnosis of primary TBA. So, for patients with chronic cough of unknown etiology, bronchoscopy should be performed to obtain biopsy samples for the definitive diagnosis.
Aged ; Amyloidosis ; diagnosis ; Bronchi ; pathology ; Bronchial Diseases ; diagnosis ; Bronchiectasis ; Bronchitis, Chronic ; Bronchoscopy ; Delayed Diagnosis ; Humans ; Immunoglobulin Light-chain Amyloidosis ; Male ; Trachea ; pathology ; Tracheal Diseases ; diagnosis ; Tuberculosis

BACKGROUNDCommon variable immunodeficiency (CVID) is one of the most common symptomatic primary immunodeficiency syndromes. The purpose of this article was to broaden our knowledge about CVID for better diagnosis and treatment.

METHODSClinical and immunological features of 40 Chinese patients with CVID were analyzed retrospectively.

RESULTSThe median age at onset was 11-year-old (range 4-51 years). The median age at diagnosis was 14.5-year-old (range 5-66 years). The average time of delay in diagnosis was 5.3 years (range 1-41 years). The most common main complaint was fever due to infections (35 cases, 87.5%). Pneumonia (28 cases, 70%) was the most common type of infections. Bronchiectasis was present in 6 patients (15%). Autoimmune disease was detected in 6 cases of CVID, and malignancy in 2 cases. The median total serum levels of IgG, IgA, and IgM at diagnosis were 1.07 g/L, 0.07 g/L, and 0.28 g/L, respectively. The percentages of CD3- /CD19 + B-cells were 1%-3.14%.

CONCLUSIONSInfection is the most frequent presentation of CVID. Patients with unexplainable infections should receive further examination including serum immunoglobulin (Ig) and lymphocyte subset analysis. Regular and sufficient substitution with Ig is recommended.

Adolescent ; Adult ; Aged ; Bronchiectasis ; drug therapy ; immunology ; pathology ; Child ; Child, Preschool ; China ; Common Variable Immunodeficiency ; drug therapy ; immunology ; pathology ; Humans ; Immunoglobulins ; metabolism ; Immunoglobulins, Intravenous ; Middle Aged ; Young Adult

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

Recently, the numbers of lung transplantation (LT) has been increased in Korea. However, post-LT outcome has not been successful in all patients, which may be partially affected by the primary lung disease. Therefore comprehensive understanding in original pathological diagnosis of patients with LT would be needed for achieving better clinical outcome. To address this issue, we performed clinico-pathological analysis of the explanted lungs from 29 patients who underwent LT over a 9-yr period in Seoul National University Hospital. Among them, 26 patients received single (1/26) or double (25/26) LT, while heart-lung transplantation was performed in 3 patients. The final clinico-pathological diagnoses were idiopathic pulmonary fibrosis/usual interstitial pneumonia (UIP) (n = 6), acute interstitial pneumonia (AIP)/diffuse alveolar damage (DAD) (n = 4), AIP/non-specific interstitial pneumonia with DAD (n = 1), collagen vascular disease-related interstitial lung disease (CVD-ILD)/DAD (n = 3), CVD-ILD/UIP (n = 1), lymphangioleiomyomatosis (n = 1), bronchiectasis (n = 4), pulmonary arterial hypertension (n = 2), tuberculosis (n = 1), bronchiolitis obliterans (BO) (n = 1), and lung cancer (n = 1). Moreover, 4 patients who had chemotherapy and hematopoietic stem cell transplantation due to hematologic malignancy showed unclassifiable interstitial pneumonia with extensive fibrosis in the lungs. Our study demonstrates that pathology of the explanted lungs from Korean patients with LT is different from that of other countries except for interstitial lung disease and bronchiectasis, which may be helpful for optimization of selecting LT candidates for Korean patients.
Acinetobacter baumannii/isolation & purification ; Adolescent ; Adult ; Aged ; Bronchiectasis/*pathology ; Child ; Female ; Humans ; Idiopathic Pulmonary Fibrosis/*pathology ; Lung/microbiology/*pathology ; Lung Diseases, Interstitial/*pathology ; *Lung Transplantation ; Male ; Middle Aged ; Republic of Korea ; Treatment Outcome ; Vancomycin-Resistant Enterococci/isolation & purification ; Young Adult

Cystic fibrosis (CF) is inherited as an autosomal recessive trait, and the mutations in cystic fibrosis transmembrane conductance regulator (CFTR) gene contributes to the CF syndrome. Although CF is common in Caucasians, it is known to be rare in Asians. Recently, we experienced two cases of CF in Korean children. The patients were girls with chronic productive cough since early infancy. Chest computed tomography showed the diffuse bronchiectasis in both lungs, and their diagnosis was confirmed by the repeated analysis of a quantitative pilocarpine iontophoresis test (QPIT). The sweat chloride concentrations of the first patient were 108.1 mM/L and 96.7 mM/L. The genetic analysis revealed that she was the compound heterozygote of Q1291X and IVS8 T5 -M470V. In the second case, the sweat chloride concentrations were 95.0 mM/L and 77.5 mM/L. Although we performed a comprehensive search for the coding regions and exonintron splicing junctions of CFTR gene, no obvious disease-related mutations were detected in the second case. To our knowledge, this is the first report of CF in Korean children identified by a QPIT and genetic analysis. The possibility of CF should be suspected in those patients with chronic respiratory symptoms even in Korea.
Blood Pressure ; Bronchiectasis/diagnosis/pathology ; Child ; Cough ; Cystic Fibrosis/*diagnosis/*genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; DNA Mutational Analysis ; Exons ; Female ; Heterozygote ; Humans ; Introns ; Iontophoresis/*methods ; Korea ; Lung/pathology/radiography ; Muscarinic Agonists/*pharmacology ; Mutation ; Pancreas/pathology ; Pedigree ; Phenotype ; Pilocarpine/*pharmacology ; Polymorphism, Genetic ; Research Support, Non-U.S. Gov't ; Sinusitis/diagnosis/pathology ; Sweat ; Time Factors ; Tomography, X-Ray Computed

OBJECTIVETo study the clinical and pathological features of pulmonary neuroendocrine cell hyperplasia and tumorlets with bronchiectasis.

METHODSBoth the clinicopathologic changes and immunohistochemical findings were examined with microscopy and EnVision method in 22 cases of pulmonary neuroendocrine cell hyperplasia and tumorlets.

RESULTSThe average age of the 22 patients was 53 years, with a male to female ratio of 9:13. On macroscopic examination the lungs showed bronchiectasis; one case was accompanied by gray-white, soft nodules (diameter < 5 mm). Microscopy of the HE sections showed the basic pathologic change was bronchiectasis, accompanied by neuroendocrine cell hyperplasia and tumorlet formation in the pulmonary parenchyma surrounding the bronchioles, presenting as single nodule (10 patients), or multifocal nodules (12 patients), with average size of 1.6 mm in diameter. No tumor cells were identified in the lymph nodes. Sixteen of 22 patients were disease-free after an average follow-up period of 58 months (17 - 117 months); one patient died suddenly after surgery; and five were loss of follow up. Immunohistologically, the tumor cells were positive for CgA (18/18), Syn (16/16), AE1/AE3 (16/16) , TTF-1 (14/15), and CD56 (14/14), and Ki-67 index was < 2% in 12 cases.

CONCLUSIONSImmunohistological staining for CgA, Syn, CD56, TTF-1 and AE1/AE3 can confirm the diagnosis. Early detection, pulmonary resection and follow-up help prevent the progression of these diseases.

Adult ; Aged ; Bronchiectasis ; pathology ; Chromogranin A ; metabolism ; DNA-Binding Proteins ; metabolism ; Disease-Free Survival ; Female ; Follow-Up Studies ; Humans ; Hyperplasia ; Ki-67 Antigen ; metabolism ; Lung Neoplasms ; metabolism ; pathology ; surgery ; Male ; Middle Aged ; Neuroendocrine Cells ; pathology ; Neuroendocrine Tumors ; metabolism ; pathology ; surgery ; Pneumonectomy ; Synaptophysin ; metabolism ; Transcription Factors

Various forms of hypogammaglobulinemia can occur in patients with autoimmune diseases and vice versa. We report a 13-yr-old boy with membranous nephropathy and common variable immunodeficiency. He presented with the nephrotic syndrome, pneumonia with bronchiectasis, and profound hypogammaglobulinemia. Renal biopsy showed diffusely thickened glomerular capillary walls with 'spikes' suggesting a membranous nephropathy. Secondary causes were ruled out by laboratory studies; however, heavy proteinuria persisted with steroid therapy. Cyclosporine and intravenous immunoglobulin were added, and the patient was discharged with decreased proteinuria. Hypogammaglobulinemia may have a deleterious impact on the immune dysregulation in some patients with membranous nephropathy.
Adolescent ; Bronchiectasis/etiology ; Common Variable Immunodeficiency/complications/*diagnosis/drug therapy ; Cyclosporine/therapeutic use ; Drug Therapy, Combination ; Glomerulonephritis, Membranous/complications/*diagnosis/drug therapy ; Humans ; Immunoglobulins/therapeutic use ; Immunosuppressive Agents/therapeutic use ; Injections, Intravenous ; Kidney/pathology ; Male ; Pneumonia/etiology ; Proteinuria/etiology ; Steroids/therapeutic use