Relapsing polychondritis (RP) is a rare autoimmune disorder of unknown etiology characterized by recurrent episodes of inflammation and the destruction of cartilaginous tissues, primarily involving the ear, nose, and the respiratory tract. Nasal chondritis is present in 24% of patients at the time of diagnosis and develops subsequently in 53% throughout the diseases progress. Progressive destruction of nasal cartilage leads to the characteristic flattening of the nasal bridge, resulting in the saddle nose deformity. In patients with RP, surgical management for saddle nose is carefully decided due to the disease relapsing characteristics. We present a RP patient with a saddle nose deformity who underwent reconstruction rhinoplasty with autologous costal cartilage grafting. At 6-month follow-up, the patient retained good esthetic results and showed neither complication nor relapse of RP.
Congenital Abnormalities ; Costal Cartilage ; Diagnosis ; Ear ; Follow-Up Studies ; Humans ; Inflammation ; Nasal Cartilages ; Nose ; Polychondritis, Relapsing ; Recurrence ; Respiratory System ; Rhinoplasty ; Transplants

A 42-year-old man was diagnosed with cancer of the right lower lung lobe with a posteparterial type of tracheal bronchus, in which the posterior segmental bronchus of the right upper lobe arose from the distal bronchus intermedius. A mass involved the distal bronchus intermedius, requiring a right lower bilobectomy with an additional posterior segmental resection of the right upper lung lobe. Thus, we performed a right lower bilobectomy and sleeve anastomosis of the posterior segmental bronchus of the right upper lobe to the proximal bronchus intermedius, sparing the pulmonary parenchyma of the same lobe.
Adult ; Bronchi* ; Humans ; Lung Neoplasms* ; Lung* ; Respiratory System Abnormalities ; Trachea

Pulmonary systemic arterialization to normal basal lung without sequestration is a rare congenital anomaly. In this rare abnormality, arterialization of the left lower lobe is the most common type. In general, surgical treatments have been performed. Recently, for reducing the complications and risks of surgery, embolization is mainly attempted by using coils. We report a case of 22-year-old male patient with a 10 mm anomalous arterial supply to his normal lung, which is being successfully treated by transcatheter embolization when using the Amplatzer Vascular Plug that has been adapted for the treatment of high-flows and large artery occlusions.
Arteries ; Embolization, Therapeutic ; Humans ; Lung ; Male ; Pulmonary Artery ; Respiratory System Abnormalities ; Young Adult

PURPOSE: Previous studies suggest that the concentration of 25-hydroxyvitamin D [25(OH)D] in cord blood may show an inverse association with respiratory tract infections (RTI) during childhood. The aim of the present study was to examine the influence of 25(OH)D concentrations in cord blood on infant RTI in a Korean birth cohort. METHODS: The levels of 25(OH)D in cord blood obtained from 525 Korean newborns in the prospective COhort for Childhood Origin of Asthma and allergic diseases were examined. The primary outcome variable of interest was the prevalence of RTI at 6-month follow-up, as diagnosed by pediatricians and pediatric allergy and pulmonology specialists. RTI included acute nasopharyngitis, rhinosinusitis, otitis media, croup, tracheobronchitis, bronchiolitis, and pneumonia. RESULTS: The median concentration of 25(OH)D in cord blood was 32.0 nmol/L (interquartile range, 21.4 to 53.2). One hundred and eighty neonates (34.3%) showed 25(OH)D concentrations less than 25.0 nmol/L, 292 (55.6%) showed 25(OH)D concentrations of 25.0-74.9 nmol/L, and 53 (10.1%) showed concentrations of > or =75.0 nmol/L. Adjusting for the season of birth, multivitamin intake during pregnancy, and exposure to passive smoking during pregnancy, 25(OH)D concentrations showed an inverse association with the risk of acquiring acute nasopharyngitis by 6 months of age (P for trend=0.0004). CONCLUSION: The results show that 89.9% of healthy newborns in Korea are born with vitamin D insufficiency or deficiency (55.6% and 34.3%, respectively). Cord blood vitamin D insufficiency or deficiency in healthy neonates is associated with an increased risk of acute nasopharyngitis by 6 months of age. More time spent outdoors and more intensified vitamin D supplementation for pregnant women may be needed to prevent the onset of acute nasopharyngitis in infants.
Asthma ; Bronchiolitis ; Cohort Studies* ; Croup ; Fetal Blood* ; Follow-Up Studies ; Humans ; Hypersensitivity ; Infant ; Infant, Newborn ; Korea ; Nasopharyngitis ; Otitis Media ; Parturition ; Pregnancy ; Pregnant Women ; Prevalence ; Prospective Studies ; Respiratory System Abnormalities* ; Respiratory Tract Infections* ; Seasons ; Specialization ; Tobacco Smoke Pollution ; Vitamin D

OBJECTIVETo investigate and compare the diagnostic values of bronchoscopy and multi-slice spiral computed tomography (CT) for congenital dysplasia of the respiratory system in infants.

METHODSAnalysis was performed on the clinical data, bronchoscopic findings and multi-slice spiral CT findings of 319 infants (≤1 years old) who underwent bronchoscopy and/or multi-slice spiral CT and were diagnosed with congenital dysplasia of the respiratory system.

RESULTSA total of 476 cases of congenital dysplasia of the respiratory system were found in the 319 infants, including primary dysplasia of the respiratory system (392 cases) and compressive dysplasia of the respiratory system (84 cases). Of the 392 cases of primary dysplasia of the respiratory system, 225 (57.4%) were diagnosed by bronchoscopy versus 167 (42.6%) by multi-slice spiral CT. There were significant differences in etiological diagnosis between bronchoscopy and multi-slice spiral CT in infants with congenital dysplasia of the respiratory system (P<0.05). All 76 cases of primary dysplasia of the respiratory system caused by tracheobronchomalacia were diagnosed by bronchoscopy and all 17 cases of primary dysplasia of the respiratory system caused by lung tissue dysplasia were diagnosed by multi-slice spiral CT. Of the 84 cases of compressive dysplasia of the respiratory system, 74 cases were diagnosed by multi-slice spiral CT and only 10 cases were diagnosed by bronchoscopy.

CONCLUSIONSCompared with multi-slice spiral CT, bronchoscopy can detect primary dysplasia of the respiratory system more directly. Bronchoscopy is valuable in the confirmed diagnosis of tracheobronchomalacia. Multi-slice spiral CT has a higher diagnostic value for lung tissue dysplasia than bronchoscopy.

Bronchoscopy ; methods ; Humans ; Infant ; Multidetector Computed Tomography ; methods ; Respiratory System Abnormalities ; diagnosis ; Tracheobronchomalacia ; diagnosis

Female ; Humans ; Infant ; Infant, Newborn ; Larynx ; abnormalities ; surgery ; Male ; Respiratory System Abnormalities ; surgery ; Vocal Cords ; abnormalities ; surgery

Bronchoscopy ; Child ; Congenital Abnormalities ; pathology ; Humans ; Respiratory System ; pathology

Intralobar pulmonary sequestration is a rare congenital lung anomaly. It is defined as a portion of nonfunctioning lung parenchyma that receives its blood supply from an anomalous systemic artery. Patients often present with chronic or recurrent pneumonia. A chest radiograph may show a cystic lesion with air-fluid levels in the lung base. A high index of suspicion is needed for a diagnosis. Surgical removal of a symptomatic intralobar pulmonary sequestration is generally the treatment of choice. Identifying the aberrant artery is a difficult problem when resecting a pulmonary sequestration. The thoracic and abdominal aortas are the most common origins for the abnormal blood supply. However, arterial supply from the celiac artery is quite rare. We present a case of intralobar pulmonary sequestration with the blood supply originating from the celiac artery.
Aorta, Abdominal ; Arteries ; Bronchopulmonary Sequestration ; Celiac Artery ; Humans ; Lung ; Pneumonia ; Respiratory System Abnormalities ; Thorax

OBJECTIVETo explore clinical characteristics, radiographic findings and diagnostic methods of patients with congenital malformations of respiratory system for enhancing the diagnosis of congenital malformations of respiratory system in children.

METHODTotally 234 patients with congenital malformations of respiratory system were chosen from the inpatient department of Yuying Children's Hospital Affiliated to Wenzhou Medical College from July 2003 to June 2008. The clinical presentations and radiographic findings of these children were analyzed.

RESULTOf the 234 patients with congenital malformations of respiratory system, the age at diagnosis was between the first day and 14 years of age, mean age was 1.12 years. The main symptoms were persistent laryngeal stridor, recurrent wheezing, recurrent respiratory tract infections and dyspnea. Through the use of chest X-ray, spiral CT 3D reconstructions, fiberoptic bronchoscopy and other laboratory techniques, 213 cases were diagnosed as having single malformation and 21 cases were found to have multiple malformations. Of the 213 cases with single malformation, 97 cases had laryngeal malformation (congenital laryngeal stridor in 90 cases, congenital laryngeal webs in 5 cases and congenital laryngeal cyst in 2 cases), 35 cases had tracheal-bronchial malformation (congenital tracheobronchial stenosis in 17 cases, congenital abnormal bronchial origin in 7 cases, tracheobronchomalacia in 10 cases and tracheoesophageal fistula in 1 case), 43 cases had lung malformation (pulmonary sequestration in 5 cases, congenital lung cysts in 22 cases, congenital lobar emphysema in 1 case, agenesis of lung and hypoplasia of lung in 8 cases and congenital cystic adenomatoid malformation in 7 cases), 38 cases had diaphragm malformation, 28 cases had congenital tracheal-bronchial stenosis as confirmed by spiral CT 3D reconstructions and fiberoptic bronchoscopy. Ten cases with congenital abnormal bronchial origin were diagnosed with spiral CT 3D reconstructions. Laryngeal stridor and tracheobronchomalacia were diagnosed by fiberoptic laryngoscope and fiberoptic bronchoscopy. The accuracy rates of preoperative diagnosis through clinical and radiographic examinations of 37 cases with lung malformation and 36 cases with diaphragm malformation were 83.78% and 91.67%.

CONCLUSIONCongenital malformations of respiratory system are a group of diseases that are important for pediatric respiratory clinicians. Congenital malformations of respiratory system should be considered in children with persistent laryngeal stridor, recurrent wheezing, recurrent respiratory tract infections and dyspnea. The radiographic examination and respiratory endoscope play important roles in the diagnosis of congenital malformations of respiratory system.

Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Infant, Newborn ; Male ; Respiratory System Abnormalities ; diagnosis ; Retrospective Studies

Esophageal Atresia ; complications ; Humans ; Infant, Newborn ; Male ; Respiratory System Abnormalities ; complications ; Trachea ; abnormalities ; Tracheoesophageal Fistula ; complications