Congenital cystic disease of the lung represents pulmonary sequestration, congenital lobar emphysema, bronchogenic cyst and congenital cystic adenomatoid malformation. It has a variety of clinical manifestations from immediate postnatal respiratory distress to heart failure. Pulmonary sequestration sometimes causes heart failure in neonates through a shunt between an anomalous systemic feeding artery and the pulmonary venous system. We hereby report a case with both pulmonary sequestration and congenital lobar emphysema presenting with congestive heart failure and pulmonary hypertension, which improved after lobectomy.
Arteries ; Bronchogenic Cyst ; Bronchopulmonary Sequestration ; Cystic Adenomatoid Malformation of Lung, Congenital ; Emphysema ; Estrogens, Conjugated (USP) ; Heart Failure ; Humans ; Hypertension, Pulmonary ; Infant, Newborn ; Lung ; Pulmonary Emphysema

We experienced tracheobronchial stenosis caused by malrotation of the heart in a 3-year-old girl. Malrotation of the heart is induced by the decreased right lung volume, which was the result of right lung hypoplasia and herniation of the left thoracic cavity. We corrected the right lung volume and location of the heart to treat tracheobronchial stenosis.
Child, Preschool ; Constriction, Pathologic* ; Female ; Heart* ; Humans ; Lung ; Thoracic Cavity ; Tracheal Stenosis

PURPOSE: Early detection of Mycoplasma pneumoniae is important for appropriate antimicrobial therapy in children with pneumonia. This study aimed to evaluate the diagnostic value of a rapid antigen test kit in detecting M. pneumoniae from respiratory specimens in children with lower respiratory tract infection (LRTI). METHODS: A total of 215 nasopharyngeal aspirates (NPAs) were selected from a pool of NPAs that had been obtained from children admitted for LRTI from August 2010 to August 2018. The specimens had been tested for M. pneumoniae by culture and stored at −70°C until use. Tests with Ribotest Mycoplasma® were performed and interpreted independently by two investigators who were blinded to the culture results. RESULTS: Among the 215 NPAs, 119 were culture positive for M. pneumoniae and 96 were culture negative. Of the culture-positive specimens, 74 (62.2%) were positive for M. pneumoniae by Ribotest Mycoplasma®, and 92 of the 96 (95.8%) culture-negative specimens were negative for M. pneumoniae by Ribotest Mycoplasma®. When culture was used as the standard test, the sensitivity and specificity of Ribotest Mycoplasma® were 62.2% and 95.8%, respectively. Additionally, the positive predictive value, negative predictive value, and overall agreement rates with Ribotest Mycoplasma® were 94.9%, 67.2%, and 77.2%, respectively. CONCLUSIONS: A positive test result of Ribotest Mycoplasma® suggests a high likelihood of culture-positive M. pneumoniae infection. However, a negative test result should be interpreted with caution because nearly one-third of negative test results reveal culture-positive M. pneumoniae infections.
Child ; Diagnosis ; Humans ; Immunochromatography ; Mycoplasma pneumoniae ; Mycoplasma ; Pneumonia ; Pneumonia, Mycoplasma ; Point-of-Care Systems ; Research Personnel ; Respiratory Tract Infections ; Sensitivity and Specificity

BACKGROUND: Measurement of the plasma adrenocorticotropic hormone (ACTH) level has been recommended as the first diagnostic test for differentiating between ACTH-independent Cushing syndrome (CS) and ACTH-dependent CS. When plasma ACTH values are inconclusive, a differential diagnosis of CS can be made based upon measurement of the serum dehydroepiandrosterone sulfate (DHEA-S) level and results of the high-dose dexamethasone suppression test (HDST). The aim of this study was to assess the utility of plasma ACTH to differentiate adrenal CS from Cushing' disease (CD) and compare it with that of the HDST results and serum DHEA-S level. METHODS: We performed a retrospective, multicenter study from January 2000 to May 2012 involving 92 patients with endogenous CS. The levels of plasma ACTH, serum cortisol, 24-hour urine free cortisol (UFC) after the HDST, and serum DHEA-S were measured. RESULTS: Fifty-seven patients had adrenal CS and 35 patients had CD. The area under the curve of plasma ACTH, serum DHEA-S, percentage suppression of serum cortisol, and UFC after HDST were 0.954, 0.841, 0.950, and 0.997, respectively (all P<0.001). The cut-off values for plasma ACTH, percentage suppression of serum cortisol, and UFC after HDST were 5.3 pmol/L, 33.3%, and 61.6%, respectively. The sensitivity and specificity of plasma ACTH measurement were 84.2% and 94.3%, those of serum cortisol were 95.8% and 90.6%, and those of UFC after the HDST were 97.9% and 96.7%, respectively. CONCLUSION: Significant overlap in plasma ACTH levels was seen between patients with adrenal CS and those with CD. The HDST may be useful in differentiating between these forms of the disease, especially when the plasma ACTH level alone is not conclusive.
Adrenocorticotropic Hormone* ; Cushing Syndrome* ; Dehydroepiandrosterone Sulfate ; Dexamethasone ; Diagnosis, Differential ; Diagnostic Tests, Routine ; Humans ; Hydrocortisone ; Pituitary ACTH Hypersecretion* ; Plasma* ; Retrospective Studies ; Sensitivity and Specificity

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) involves dysregulation of the complement system, but whether this also occurs in thrombotic thrombocytopenic purpura (TTP) remains unclear. Although these conditions are difficult to differentiate clinically, TTP can be distinguished by low (<10%) ADAMTS13 activity. The aim was to identify the differences in complement activation products between TTP and aHUS and investigate ADAMTS13 activity as a prognostic factor in aHUS. METHODS: We analyzed patients with thrombotic microangiopathy diagnosed as TTP (N=48) or aHUS (N=50), selected from a Korean registry (N=551). Complement activation products in the plasma samples collected from the patients prior to treatment and in 40 healthy controls were measured by ELISA. RESULTS: The levels of generalized (C3a), alternate (factor Bb), and terminal (C5a and C5b-9) markers were significantly higher (all P<0.01) in the patients than in the healthy controls. Only the factor Bb levels significantly differed (P=0.008) between the two disease groups. In aHUS patients, high normal ADAMTS13 activity (≥77%) was associated with improved treatment response (OR, 6.769; 95% CI, 1.605–28.542; P=0.005), remission (OR, 6.000; 95% CI, 1.693–21.262; P=0.004), exacerbation (OR, 0.242; 95% CI, 0.064–0.916; P=0.031), and disease-associated mortality rates (OR, 0.155; 95% CI, 0.029–0.813; P=0.017). CONCLUSION: These data suggest that complement biomarkers, except factor Bb, are similarly activated in TTP and aHUS patients, and ADAMTS13 activity can predict the treatment response and outcome in aHUS patients.
Atypical Hemolytic Uremic Syndrome ; Biomarkers ; Complement Activation ; Complement System Proteins ; Enzyme-Linked Immunosorbent Assay ; Humans ; Mortality ; Plasma ; Purpura, Thrombotic Thrombocytopenic ; Thrombotic Microangiopathies

Eikenella corrodens rarely causes invasive head and neck infections in immunocompetent children. We report a case of epidural abscess caused by E. corrodens in a previously healthy 13-year-old boy who presented with fever, headache, and vomiting. On physical examination upon admission, there was no neck stiffness, but discharge from the right ear was observed. Brain magnetic resonance imaging (MRI) revealed approximately 4.5-cm-sized epidural empyema on the right temporal lobe as well as bilateral ethmoid and sphenoid sinusitis, right mastoiditis, and right otitis media. During treatment with vancomycin and cefotaxime, purulent ear discharge aggravated, and on follow-up brain MRI, the empyema size increased to 5.6×3.4 cm with interval development of an abscess at the right sphenoid sinus. Burr hole trephination was performed, and foul-smelling pus was aspirated from the epidural abscess near the right temporal lobe. Pus culture yielded E. corrodens. Endoscopic sphenoidotomy was also performed with massive pus drainage, and the same organism was grown. The patient was treated with intravenous cefotaxime for 3 weeks and recovered well with no other complications. Therefore, E. corrodens can cause serious complications in children with untreated sinusitis.
Abscess ; Adolescent ; Brain ; Cefotaxime ; Child ; Drainage ; Ear ; Eikenella corrodens ; Eikenella ; Empyema ; Epidural Abscess ; Fever ; Follow-Up Studies ; Head ; Headache ; Humans ; Magnetic Resonance Imaging ; Male ; Mastoid ; Mastoiditis ; Neck ; Otitis Media ; Physical Examination ; Sinusitis ; Sphenoid Sinus ; Sphenoid Sinusitis ; Suppuration ; Temporal Lobe ; Trephining ; Vancomycin ; Vomiting

The bleeding pseudoaneurysm is a rare condition but one of the lethal complications in patients with chronic pancreatitis. Early diagnosis and emergent intervention can reduce mortality and promise better outcomes. We experienced a case of a bleeding pseudo- aneurysm in a chronic pancreatitis patient. The 44-year-old male was suffering from con- tinuous, vague epigastric pain and black tarry stool. An abdominal CT scan revealed a homogeneously enhancing round mass in the pancreatic pseudocyst, and celiac angi- ography found that a pseudoaneurysm had originated from the pancreaticoduodenal artery. The patient was treated with Whipple's operation and was later discharged, having improved in his condition.
Adult ; Aneurysm ; Aneurysm, False* ; Arteries ; Early Diagnosis ; Hemorrhage ; Humans ; Male ; Mortality ; Pancreatic Pseudocyst ; Pancreatitis, Chronic* ; Tomography, X-Ray Computed

A 78-year-old man was diagnosed with renal cell carcinoma, and left nephrectomy was performed. He started pazopanib. One month later, he visited our hospital because of general weakness and dyspnea. His oxygen saturation was low. A chest X-ray showed pulmonary edema and bilateral pleural effusion. An echocardiogram showed a larger left ventricle and lower ejection fraction than observed at the previous examination. The patient discontinued pazopanib and started diuretics and digoxin. His symptoms improved and a follow-up X-ray showed improvement in the pulmonary edema with bilateral pleural effusion.
Aged ; Carcinoma, Renal Cell* ; Digoxin ; Diuretics ; Dyspnea ; Follow-Up Studies ; Heart Failure* ; Heart Ventricles ; Heart* ; Humans ; Nephrectomy ; Oxygen ; Pleural Effusion ; Pulmonary Edema ; Thorax

PURPOSE: We have assessed the clinical usefulness of solid maker transit technique and Barr-score in 31 patients with idiopathic constipation (male:female; 17:14, mean age; 6.7 years, range 1.4-12 years). METHODS: All patients underwent full history taking and physical examination including rectal examination. On first visit to gastroenterology clinic a plain abdominal film was taken for Barr-score which evaluated by two observers without the information of the patients. Kappa score was calculated for inter or intra observer variability. Sitz marker were given orally at 9 am on days 1, 2 and 3, and another plain abdominal film was taken on day 5. Total remained solid marker were counted for the degree of retention. We calulated the residual sitz marker. Each film was divided into right colon, left colon and rectosigmoid areas, using bony landmarks, and the marker content of each area counted. RESULTS: 2 patients were excluded because they resisted to take sitz maker. Transit times were normal, mild, moderate and severe delay in 12 patients, 2 patients, 3 patients and 12 patients respectively on day 5 film. The correlation coefficiency between the degree of transit delay and clinical severity was 0.89. Among 14 patients with transit delay, 12 patients has outlet obstruction, 1 patient right colonic delay and 1 patients left colonic delay. The Kappa for inter and intra-personal variability were 4.13 and 4.18 respectively (moderate consistency each). The correlation coefficiency between Barr-score and solid marker colonic transit time was 0.603 (P=0.0008). CONCLUSIONS: This results showed that the solid marker colonic transit time and Barr score were useful in evaluation of patients with constipation.
Colon ; Constipation* ; Gastroenterology ; Humans ; Observer Variation ; Patient Rights ; Physical Examination