Unilateral absence of pulmonary artery is a rare congenital anomaly that is frequently associated with other cardiovascular abnormalities. Most patients who have no associated cardiac anomalies have only minor or absent symptoms in childhood and survive into adulthood. We experienced a case of unilateral absence of pulmonary artery in a 5 year-old female patient with the chief complaint of abnormal chest X-ray finding. Unilateral absence of pulmonary artery was diagnosed by lung perfusion scan, cardiac catheterization with pulmonary and aortic angiography. So we report this case with brief review of related literatures.
Angiography ; Cardiac Catheterization ; Cardiac Catheters ; Cardiovascular Abnormalities ; Child, Preschool ; Female ; Humans ; Lung ; Perfusion ; Pulmonary Artery* ; Thorax

Hyponatremia is commonly seen in those patients with central nervous system injury associated with infection or trauma. And decreasing intracranial pressure through restriction of maintenance fluid and salt is practiced as a routine therapeutic measure in the early stages of meningitis to prevent or ameliorate the syndrome of inappropriate secretion of antidiuretic hormone(SIADH). However, lots of patient do not show the typical symptoms of SIADH, instead they are dehydrated, have low plasma volume, increased urine sodium concentration and increased net sodium loss, which are the symptoms of cerebral salt wasting syndrome(CSW). Recent reports have prompted a reconsideration of CSW distinct from SIADH and moreover CSW has more proportion of hyponatremia associated with acute brain insult. CSW involves renal salt loss leading to hyponatremia and volume loss, whereas SIADH is a euvolemic or hypervolemic condition. While fluid restriction is the treatment of choice in SIADH, the treatment of CSW consists of vigorous sodium and volume replacement. And by correcting hyponatremic state as soon as possible, we can reduce mortality rate and improve neurologic sequelae. We report a case of CSW which was treated by replacement of vigorous sodium and volume replacement.
Brain ; Central Nervous System ; Child* ; Humans ; Hyponatremia ; Inappropriate ADH Syndrome ; Intracranial Pressure ; Meningitis* ; Mortality ; Plasma Volume ; Sodium ; Wasting Syndrome*

Henoch-Schonlein purpura (HSP) is a vasculitis of the small vessels in skin, joints, gastrointestinal (GI) tract and kidney. GI symptoms occur in up to 85% of patients and may lead to severe problems such as intussusception, obstruction, and perforation. GI symptoms may not be easily controlled, showing refractoriness to the conventional corticosteroid therapy. Although GI involvements of HSP are acute, and self-limited in most instances, they may cause fatal results in some unusual cases. In such conditions all the possible therapeutic modalities should be considered. We report two cases of severe small bowel involvement of HSP. One case presented with severe abdominal pain showing refractoriness to corticosteroid, but improved with IV immunoglobulin therapy. In the second case, HSP with transmural infarction in the small bowel could be cured with surgical intervention.
Abdominal Pain ; Humans ; Immunization, Passive ; Immunoglobulins ; Infarction ; Intussusception ; Joints ; Kidney ; Purpura, Schoenlein-Henoch* ; Skin ; Vasculitis

PURPOSE: Radiation-induced autophagy has been shown to play two different roles, in malignant glioma (MG) cells, cytocidal or cytoprotective. However, neither the role of radiation-induced autophagy for cell death nor the existence of autophagy-induced apoptosis, a well-known cell-death pathway after irradiation, has been verified yet. MATERIALS AND METHODS: We observed both temporal and dose-dependent response patterns of autophagy and apoptosis to radiation in MG cell lines. Additionally, we investigated the role of autophagy in apoptosis through knockdown of autophagy-related proteins. RESULTS: Autophagic activity measured by staining of acidic vesicle organelles and Western blotting of LC-3 protein increased in proportion to radiation dose from day 1 to 5 after irradiation. Apoptosis measured by annexin-V staining and Western blotting of cleaved poly(ADP-ribose) polymerase demonstrated relatively late appearance 3 days after irradiation that increased for up to 7 days. Blocking of pan-caspase (Z-VAD-FMK) did not affect apoptosis after irradiation, but silencing of Atg5 effectively reduced radiation-induced autophagy, which decreased apoptosis significantly. Inhibition of autophagy in Atg5 knockdown cells was shown to be beneficial for cell survival. Stable transfection of GFP-LC3 cells was observed after irradiation. Annexin-V was localized in cells bearing GFP-LC3 punctuated spots, indicating autophagy in immunofluorescence. Some of these punctuated GFP-LC3 bearing cells formed conglomerated spots and died in final phase. CONCLUSION: These findings suggest that autophagy appears earlier than apoptosis after irradiation and that a portion of the apoptotic population that appears later is autophagy-dependent. Thus, autophagy is a pathway to cell death after irradiation of MG cells.
Apoptosis* ; Autophagy* ; Blotting, Western ; Cell Death* ; Cell Line ; Cell Survival ; Fluorescent Antibody Technique ; Glioma* ; Organelles ; Poly(ADP-ribose) Polymerases ; Transfection

Following reports of patients with unexplained pneumonia at the end of December 2019 in Wuhan, China, the causative agent was identified as coronavirus (SARS-CoV-2), and the 2019 novel coronavirus disease was named COVID-19 by the World Health Organization. Putative patients with COVID-19 have been identified in South Korea, and attempts have been made to isolate the pathogen from these patients.

PURPOSE: Not all premature infants have respiratory distress syndrome (RDS), although prematurity is the most crucial risk-factor. Since genetic factors are known to be an etiology of RDS, this dissertation examines if specific SP-A alleles/genotypes are associated with either increased or decreased risk of RDS. METHODS: Investigated for this research were 272 preterm Korean infants. Among them, 89 infants with RDS and 183 controlled infants were analyzed for SP-A genotypes by using the real- time PCR assay. RESULTS: The specific frequencies of the alleles of the SP-A1 gene among the preterm infants (n=544 alleles) turned out to be 47.6% for 6A3, 27.2% for 6A2, 23.7% for 6A4, and 1.5% for others. Those of the alleles of the SP-A2 gene were 46.9% for 1A12, 18.9% for 1A6 and 18.9% for 1A10 (n=544 alleles). Others include 3.9% each for 1A and 1.1% for 1A0. These results present great difference from previous studies. This research found new genotypes each of SP-A1 and SP-A2 genes. The 1A12/1A12 genotype has statistical relations with different gestational age under 32 weeks. The 1A12/1A12 was underrepresented (14.6% vs 26.8%) (P<0.05) among the preterm infants with RDS. In the preterm infants with RDS born at gestational age> or =32 wk, the 1A12/1A12 acts as the only significant protective factor from the development of RDS [odds ratio 0.156 (P=0.014, 95% confidence intervals 0.035-0.691)]. CONCLUSION: The SP-A gene polymorphism is the crucial factor to the predisposition to RDS when the gestational ages of preterm infants are higher.
Alleles ; Genotype ; Gestational Age ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Polymerase Chain Reaction ; Pulmonary Surfactant-Associated Protein A*