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.
Agammaglobulinemia/complications* ; Bronchiectasis/complications* ; Humans ; Retrospective Studies ; Thymoma/complications* ; Thymus Neoplasms/complications*

OBJECTIVETo investigate the etiology and clinical manifestation of hemoptysis in children.

METHODA retrospective analysis was performed for 106 cases of hemoptysis who were admitted to The Second Affiliated Hospital & Yuying Children's Hospital of Wenzhou Medical University from January 2005 to December 2014.The clinical information including laboratory tests and image data were collected and analyzed.

RESULTA total of 106 patients (50 males and 56 females) were identified. The median age was 9.1 years (range 2 months to 18 years). Pneumonia (35, 31.1%) was the most common etiology of hemoptysis, which included bacterial pneumonia (27 cases), mycoplasmal pneumonia(4 cases), chlamydial pneumonia (3 cases), and influenza pneumonia(1 case). Other causes included bronchitis(15, 14.2%), pulmonary tuberculosis (11, 10.4%), bronchiectasis (11, 10.4%), diffuse alveolar hemorrhage (8, 7.5%), idiopathic pulmonary hemosiderosis(6, 5.7%), cardiovascular dysplasia(6, 5.7%), pulmonary contusion (4, 3.8%), foreign body in bronchus (2, 1.9%), allergic bronchopulmonary aspergillosis (2, 1.9%). Eighty-six patients manifested mild hemoptysis; moderate and massive hemoptysis were found in nine and eleven patients, respectively. Pneumonia accounted for 33.7% of mild hemoptysis and 45.5% of massive hemoptysis were due to bronchiectasis; 80.2% were treated with antibiotics and 41.5% were given hemostatic agents; 8.5% received lobectomy. Ninety-six patients (90.6%) were cured and parents gave up treatment in 4 cases (3.8%). Six patients (5.7%) suffered from recurrent hemoptysis.

CONCLUSIONHemoptysis mainly occurred in children who were older than 6 years, the most common cause of hemoptysis was respiratory tract infection. In most cases, the amount of hemoptysis was small and the overall prognosis was good.

Adolescent ; Bronchiectasis ; complications ; Bronchitis ; complications ; Child ; Child, Preschool ; Female ; Foreign Bodies ; complications ; Hemoptysis ; diagnosis ; etiology ; therapy ; Hemosiderosis ; complications ; Humans ; Infant ; Influenza, Human ; complications ; Lung Diseases ; complications ; Lung Injury ; complications ; Male ; Pneumonia, Bacterial ; complications ; Prognosis ; Retrospective Studies ; Tuberculosis, Pulmonary ; complications

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

PURPOSE: Bronchiectasis is the main cause of hemoptysis. When patients with bronchiectasis develop hemoptysis, clinicians often perform bronchoscopy and bronchial washing to obtain samples for microbiological and cytological examinations. Bronchial washing fluids were analyzed from patients with bronchiectasis who developed hemoptysis, and the clinical impacts of these analyses were examined. MATERIALS AND METHODS: A retrospective observational study of patients who underwent fiberoptic bronchoscopy for hemoptysis in Seoul National University Bundang Hospital, a university affiliated tertiary referral hospital, between January 2006 and December 2010 were reviewed. Among them, patients who had bronchiectasis confirmed by computed tomography and had no definite cause of hemoptysis other than bronchiectasis were reviewed. The demographic characteristics, bronchoscopy findings, microbiological data, pathology results and clinical courses of these patients were retrospectively reviewed. RESULTS: A total of 130 patients were reviewed. Bacteria, non-tuberculous mycobacteria (NTM), and Mycobacterium tuberculosis were isolated from bronchial washing fluids of 29.5%, 21.3%, and 0.8% patients, respectively. Suspected causal bacteria were isolated only from bronchial washing fluid in 19 patients, but this analysis led to antibiotics change in only one patient. Of the 27 patients in whom NTM were isolated from bronchial washing fluid, none of these patients took anti-NTM medication during the median follow-up period of 505 days. Malignant cells were not identified in none of the patients. CONCLUSION: Bronchial washing is a useful method to identify microorganisms when patients with bronchiectasis develop hemoptysis. However, these results only minimally affect clinical decisions.
Adult ; Aged ; Aged, 80 and over ; Bronchiectasis/*complications/microbiology ; Bronchoscopy ; Female ; Hemoptysis/*diagnosis/etiology/microbiology ; Humans ; Male ; Middle Aged ; Retrospective Studies

OBJECTIVECystic fibrosis (CF) is rare in Chinese mainland. We present two cases of CF patients diagnosed by gene analysis. Their clinical manifestations and genetic mutation features are analyzed in this article. It will be of special interest to pediatricians in recognition of CF.

METHODThe clinical material of two CF patients who were diagnosed by gene analysis was retrospectively analyzed.

RESULTThe first patient is a 13-year-old girl. She had a complaint of recurrent fever and cough for 6 months, expectoration for 2 months and hemoptysis for 20 days. After 3 months of her birth, she was operated on for bullae of lung. She was susceptible to upper respiratory tract infection. There was no family history of recurrent wheeze and other special diseases. Aspergillus fumigatus specific IgE was at grade 3 and aspergillus fumigatus IgG was high. Pseudomonas aeruginosa was positive in sputum culture. Sweat testing was performed and Na+ was higher. Pulmonary CT indicated bronchiectasis. Nasal sinus CT showed optical density of soft tissue within maxillary sinus and chronic bilateral sinusitis. The electron microscopy of cilia suggested immobile cilia syndrome. A heterozygotic mutation (263T > G, 2909G > A) was found after CFTR genetic mutation analysis. Both her parents were carriers. She was treated with inhalation of nebulized hypertonic saline and postural drainage for a long time. And she got better during a follow up period of 1 year. The second patient was a 10-year-old girl who complained of recurrent expectoration for 3 years and shortness of breath for half a year. She had a history of sinusitis and steatorrhea. The family history was normal. Both the lipase and insulin level in blood serum was lower.Pseudomonas aerugino and Aspergillus fumigatus were both positive in sputum culture. Aspergillus fumigatus IgE was normal. Pulmonary CT indicated bronchiolitis and bronchiectasis. Nasal sinus CT showed bilateral maxillary sinusitis. CFTR genetic mutation analysis revealed a homozygous mutation (3196C > T). Her parents and relatives did not participate in this study. Unfortunately, this child died of respiratory failure 3 months after discharge.

CONCLUSIONCFTR gene mutation was a main cause of CF. Common symptoms are those of bronchiectasis, pancreatitis and sinusitis. The two Chinese patients were diagnosed by gene analysis. One had a heterozygous mutation (263T > G, 2909G > A) and the other had a homozygous mutation (3196C > T), not ΔF508 which is common in western countries.

Adolescent ; Asian Continental Ancestry Group ; genetics ; Bronchiectasis ; etiology ; genetics ; Child ; Cystic Fibrosis ; complications ; diagnosis ; genetics ; Cystic Fibrosis Transmembrane Conductance Regulator ; genetics ; DNA Mutational Analysis ; Female ; Heterozygote ; Homozygote ; Humans ; Mutation ; Retrospective Studies ; Sinusitis ; etiology ; genetics

T Hemoptysis is a common complication of bronchiectasis, but concurrent primary hyperfibrinolysis is very rare and easily gives rise to missed diagnosis. Patients with bronchiectasis complicated by primary hyperfibrinolysis do not respond to regular treatment for hemoptysis, which may potential cause fatal consequences. We report two cases of bronchiectasis combined with primary hyperfibrinolysis. Both of the patients had a history of bronchiectasis and were admitted for intermittent hemoptysis. Conventional treatment such as vasopressin failed to produce a favorable response. The eventual definite diagnosis of primary hyperfibrinolysis was established based on laboratory examinations. After diagnosis, the patients received fibrinogen and cryoglobulin to maintain a fibrinogen level over 1.5 g/L. Both of the patients subsequently showed improved conditions and were discharged.
Aged ; Bronchiectasis ; complications ; Hemorrhagic Disorders ; complications ; Humans ; Male

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

BACKGROUNDTranscatheter bronchial artery embolization (BAE) is widely used for the treatment of hemoptysis and the immediate success rate is high, but there are still some hemorrhage recurrences. One of the common reasons for failure of BAE is collateral branches as blood supply. The inferior phrenic artery (IPA) is one of the most common collateral branches that is scarcely reported. Our purpose was to observe manifestations of IPA supplying to hemoptysis and evaluate the efficacy and safety of IPA embolization.

METHODSAngiography during interventional treatment of 178 hemoptysis patients in the past 7 years confirmed that IPA hemorrhage resulted in hemoptysis in 25 patients (26 - 67 years old) who had: lung cancer (11 patients), bronchiectasis (11 patients), chronic lung inflammation (2 patients), and pulmonary tuberculosis (1 patient). Among the 25 patients, 7 patients had twice interventional operations within one week and 6 patients still experienced intraoperative hemoptysis after conventional embolization of the bronchial artery, the internal thoracic artery, and the intercostal artery, then had the second interventional operation immediately. The total number of cases were 191. Selective embolization of the IPA was performed using polyvinyl alcohol microspheres, gelatin sponge particles, and microcoil. The safety and clinical significance of IPA embolization were evaluated. The Pearson chi(2) test and Fisher's exact probability test were used in this study.

RESULTSSelective IPA angiography showed increased diameter of the IPA, disorganization of the branches, and varying degrees of angiogenesis. In 11 cases, contrast material was seen in vessels supplying the tumor and in the tumor. In 9 cases, contrast material had leaked into the area supplied by the IPA; in 8 cases, non-specific flake-like deposits of contrast material were seen; and in 14 cases, abnormal communication or shunt was visualized. Lesions were closely related to the pleura in 25 patients. Fifteen lesions were close to the diaphragmatic pleura, seven close to the mediastinal pleura, and three close to the lateral pleura of the lower lung. Eleven cases had inferior thoracic pleural thickening and adhesions. The IPA was embolized in 25 cases, and the success rate of hemostasis was 100%. The IPA was not embolized in the other 166 cases, and the success rate of hemostasis was 92.17 %. In the 25 cases with IPA embolization, the involvement of the IPA in the blood supply of the hemoptysis was correlated with the duration of the disease (P = 0.0344). The involvement of IPA in the blood supply of the hemoptysis was not correlated with the characteristic of the lung lesions (benign or malignant) (P = 1.0000). Duration of follow-up was 8 months to 5 years. Hemoptysis recurred in four patients 1, 2, 3, and 6 months after interventional operation, respectively, and was controlled by conservative treatment. Twenty-one patients had no recurrence of hemoptysis.

CONCLUSIONSBleeding from the IPA can result in hemoptysis and failure of BAE in the treatment of hemoptysis. If IPA hemorrhage contributes to hemoptysis, supplementary IPA embolization may be a safe and effective treatment.

Adult ; Aged ; Angiography ; Bronchiectasis ; complications ; diagnostic imaging ; Collateral Circulation ; Embolization, Therapeutic ; methods ; Female ; Hemoptysis ; diagnostic imaging ; etiology ; therapy ; Humans ; Lung Injury ; complications ; diagnostic imaging ; Lung Neoplasms ; complications ; diagnostic imaging ; Male ; Middle Aged ; Tuberculosis, Pulmonary ; complications ; diagnostic imaging

BACKGROUND: Thoracoplasty has become a rarity in current clinical practice, although it has been widely employed for well over a century as a procedure for reducing the capacity of the thoracic cavity. Yet we have perform tailoring thoracoplasty following or concomitant with pulmonary resection in 20 patients. The aim of this study is to evaluate the early and late clinical results and also the significance of tailoring thoracoplasty. MATERIAL AND METHOD: From March 1995 to June 2005, modified thoracoplasty following or concomitant with pulmonary resection was performed in 20 patients out of a total of 298 pulmonary resections for closing air leaks and for treating persistent pleural space following pulmonary resections, and to tailor the thoracic cavity to accept a diminished lung volume. Of the 20 patients, 14 patients had tailoring thoracoplasty performed concomitant with pulmonary resection, and the remaining 6 patients also had tailoring thoracoplasty performed following pulmonary resection. The subjects ages ranged from 24 to 77 (mean 59.1+/-6.4) and a male preponderance was noted (17:3); the number of left and right surgeries was equal. The preoperative primary underlying diseases were lung cancer in 7 patients, pneumothorax with giant bullous change in 6 patients, bronchiectasis in 2 patients, previous pulmonary tuberculosis associated with aspergilloma in 2 patients, empyema with fibrothorax in 2 patients and multiple lung abscesses & destruction due to previous trauma in 1 patient. The operative methods were apicolysis and subperiosteal removal of the 2nd, 3rd and 4th ribs (the costochondral junction to the posterior portions of the ribs) with preservation of the first rib and compression of the anterior chest via cotton bags and elastic bandages. RESULT: The mean duration of the air leaks after thoracoplasty was 1.6+/-0.2 days (range: 0~7 days) and the mean duration of an indwelling chest tube was 7 days (range: 5~11 days); the mean duration of hospitalization was 19.2+/-2.8 days (range: 8~47 days). The postoperative complications were wound infection (2) and pneumonia (2); reoperation was done due to bleeding (1) in one patient who underwent concomitant thoracoplasty and there was 1 case of wound infection (1) after postresection thoracoplasty. The mortality was 1 patient in the early phase and 4 patients in the late phase. CONCLUSION: We conclude that tailoring thoracoplasty may be performed to close anticipated persistent pleural spaces and to accommodate the diminished lung volume with acceptable cosmetic results when this procedure is combined with pulmonary resection in selected patients.
Bronchiectasis ; Chest Tubes ; Compression Bandages ; Empyema ; Hemorrhage ; Hospitalization ; Humans ; Lung ; Lung Abscess ; Lung Neoplasms ; Male ; Mortality ; Pneumonia ; Pneumothorax ; Postoperative Complications ; Reoperation ; Ribs ; Thoracic Cavity ; Thoracoplasty* ; Thorax ; Tuberculosis, Pulmonary ; Wound Infection

OBJECTIVETo evaluate the value of X-ray and spiral computed tomography (SCT) in the diagnosis of Swyer-James syndrome (SJS).

METHODSA total of 28 patients, 12 males and 16 females, were studied retrospectively. Ages ranged from 11 to 57 years, the mean age was 32 years. All patients underwent inspiratory chest X-ray films, 5 with expiratory chest films and 1 with bronchogram. Furthermore, inspiratory and expiratory SCT scans were performed. The SCT findings were analyzed and compared with X-ray films.

RESULTSSCT demonstrated 56 lobes with hyperlucency and diminished vascularity. The size of 51 lobes were smaller and 5 were normal. X-ray films showed that hyperlucency was only in 29 lobes, in which 19 lobes were small-sized and the other 10 lobes normal. There were 56 lobes with air-trapping on expiratory SCT scans, but only 5 lobes with air-trapping on expiratory X-ray films. Bronchogram in 1 case demonstrated bronchiectasis and bronchiolitis obliterans. SCT showed 24 patients with bronchiectasis, 9 patients with tuberculosis, 10 patients with bronchiolitis, and 2 with segmental collapse.

CONCLUSIONSCT scan is superior to chest radiography in the diagnosis and differential diagnosis of SJS.

Adolescent ; Adult ; Bronchiectasis ; complications ; diagnostic imaging ; Bronchiolitis ; complications ; diagnostic imaging ; Child ; Diagnosis, Differential ; Female ; Humans ; Lung, Hyperlucent ; complications ; diagnostic imaging ; Male ; Middle Aged ; Radiography, Thoracic ; Retrospective Studies ; Tomography, Spiral Computed ; methods ; Tuberculosis, Pulmonary ; complications ; diagnostic imaging