Objective: Bronchopulmonary dysplasia (BPD) is a significant respiratory disorder in premature infants, and its prevalence remains high at 35% to 40% of very low gestational age (<28 weeks gestation). The aim of this study is to analyze the respiratory outcomes of BPD in neonatal intensive care unit as well as respiratory outcomes over a corrected age of 18 to 24 months using 3 different definitions of BPD (National Institutes of Health [NIH] 2001, National Institute of Child Health and Human Development [NICHD] 2018 and Jensen 2019). Methods: We conducted a study on infants under 32 weeks of gestation between 2012 and 2021 at Ajou university hospital. First, we compared the incidence of BPD, mortality, and length of hospital stays. To evaluate long-term respiratory outcomes, the number of re-admissions and prescriptions due to respiratory problems were analyzed. Results: NIH 2001 showed an incidence of 281 (40.0%), NICHD 2018 showed 139 (19.7%), and Jensen 2019 showed 137 (19.5%). In grade III of Jensen 2019, it demonstrated the highest severity with mortality rate of 29.4% and an average length of hospital stay of 42.5 weeks. Also, it was confirmed that the period of use of invasive ventilator was the longest at 87.8±60.3 days. In the analysis of readmission and prescription counts, grade III showed statistically significant higher occurrences in both NICHD 2018 and Jensen 2019 than NIH 2001. Conclusion The latest definitions of BPD have demonstrated to better represent both short-term and long-term respiratory severity in premature infants less than 32 weeks.

Objective: Bronchopulmonary dysplasia (BPD) is a significant respiratory disorder in premature infants, and its prevalence remains high at 35% to 40% of very low gestational age (<28 weeks gestation). The aim of this study is to analyze the respiratory outcomes of BPD in neonatal intensive care unit as well as respiratory outcomes over a corrected age of 18 to 24 months using 3 different definitions of BPD (National Institutes of Health [NIH] 2001, National Institute of Child Health and Human Development [NICHD] 2018 and Jensen 2019). Methods: We conducted a study on infants under 32 weeks of gestation between 2012 and 2021 at Ajou university hospital. First, we compared the incidence of BPD, mortality, and length of hospital stays. To evaluate long-term respiratory outcomes, the number of re-admissions and prescriptions due to respiratory problems were analyzed. Results: NIH 2001 showed an incidence of 281 (40.0%), NICHD 2018 showed 139 (19.7%), and Jensen 2019 showed 137 (19.5%). In grade III of Jensen 2019, it demonstrated the highest severity with mortality rate of 29.4% and an average length of hospital stay of 42.5 weeks. Also, it was confirmed that the period of use of invasive ventilator was the longest at 87.8±60.3 days. In the analysis of readmission and prescription counts, grade III showed statistically significant higher occurrences in both NICHD 2018 and Jensen 2019 than NIH 2001. Conclusion The latest definitions of BPD have demonstrated to better represent both short-term and long-term respiratory severity in premature infants less than 32 weeks.

Objective: Bronchopulmonary dysplasia (BPD) is a significant respiratory disorder in premature infants, and its prevalence remains high at 35% to 40% of very low gestational age (<28 weeks gestation). The aim of this study is to analyze the respiratory outcomes of BPD in neonatal intensive care unit as well as respiratory outcomes over a corrected age of 18 to 24 months using 3 different definitions of BPD (National Institutes of Health [NIH] 2001, National Institute of Child Health and Human Development [NICHD] 2018 and Jensen 2019). Methods: We conducted a study on infants under 32 weeks of gestation between 2012 and 2021 at Ajou university hospital. First, we compared the incidence of BPD, mortality, and length of hospital stays. To evaluate long-term respiratory outcomes, the number of re-admissions and prescriptions due to respiratory problems were analyzed. Results: NIH 2001 showed an incidence of 281 (40.0%), NICHD 2018 showed 139 (19.7%), and Jensen 2019 showed 137 (19.5%). In grade III of Jensen 2019, it demonstrated the highest severity with mortality rate of 29.4% and an average length of hospital stay of 42.5 weeks. Also, it was confirmed that the period of use of invasive ventilator was the longest at 87.8±60.3 days. In the analysis of readmission and prescription counts, grade III showed statistically significant higher occurrences in both NICHD 2018 and Jensen 2019 than NIH 2001. Conclusion The latest definitions of BPD have demonstrated to better represent both short-term and long-term respiratory severity in premature infants less than 32 weeks.

Objective: Bronchopulmonary dysplasia (BPD) is a significant respiratory disorder in premature infants, and its prevalence remains high at 35% to 40% of very low gestational age (<28 weeks gestation). The aim of this study is to analyze the respiratory outcomes of BPD in neonatal intensive care unit as well as respiratory outcomes over a corrected age of 18 to 24 months using 3 different definitions of BPD (National Institutes of Health [NIH] 2001, National Institute of Child Health and Human Development [NICHD] 2018 and Jensen 2019). Methods: We conducted a study on infants under 32 weeks of gestation between 2012 and 2021 at Ajou university hospital. First, we compared the incidence of BPD, mortality, and length of hospital stays. To evaluate long-term respiratory outcomes, the number of re-admissions and prescriptions due to respiratory problems were analyzed. Results: NIH 2001 showed an incidence of 281 (40.0%), NICHD 2018 showed 139 (19.7%), and Jensen 2019 showed 137 (19.5%). In grade III of Jensen 2019, it demonstrated the highest severity with mortality rate of 29.4% and an average length of hospital stay of 42.5 weeks. Also, it was confirmed that the period of use of invasive ventilator was the longest at 87.8±60.3 days. In the analysis of readmission and prescription counts, grade III showed statistically significant higher occurrences in both NICHD 2018 and Jensen 2019 than NIH 2001. Conclusion The latest definitions of BPD have demonstrated to better represent both short-term and long-term respiratory severity in premature infants less than 32 weeks.

Objective: Bronchopulmonary dysplasia (BPD) is a significant respiratory disorder in premature infants, and its prevalence remains high at 35% to 40% of very low gestational age (<28 weeks gestation). The aim of this study is to analyze the respiratory outcomes of BPD in neonatal intensive care unit as well as respiratory outcomes over a corrected age of 18 to 24 months using 3 different definitions of BPD (National Institutes of Health [NIH] 2001, National Institute of Child Health and Human Development [NICHD] 2018 and Jensen 2019). Methods: We conducted a study on infants under 32 weeks of gestation between 2012 and 2021 at Ajou university hospital. First, we compared the incidence of BPD, mortality, and length of hospital stays. To evaluate long-term respiratory outcomes, the number of re-admissions and prescriptions due to respiratory problems were analyzed. Results: NIH 2001 showed an incidence of 281 (40.0%), NICHD 2018 showed 139 (19.7%), and Jensen 2019 showed 137 (19.5%). In grade III of Jensen 2019, it demonstrated the highest severity with mortality rate of 29.4% and an average length of hospital stay of 42.5 weeks. Also, it was confirmed that the period of use of invasive ventilator was the longest at 87.8±60.3 days. In the analysis of readmission and prescription counts, grade III showed statistically significant higher occurrences in both NICHD 2018 and Jensen 2019 than NIH 2001. Conclusion The latest definitions of BPD have demonstrated to better represent both short-term and long-term respiratory severity in premature infants less than 32 weeks.

Periventricular nodular heterotopia (PNH) is a neuronal migration disorder that occurs during early brain development. Patients with PNH may be asymptomatic and have normal intelligence; however, PNH is also known to cause various symptoms such as seizures, dyslexia, and cardiovascular anomalies. PNH is not commonly diagnosed during early infancy because of the lack of clinical manifestations during this period. We present the case of a female infant diagnosed with PNH based on brain magnetic resonance imaging, who had symptomatic patent ductus arteriosus that had to be ligated surgically and had prolonged feeding cyanosis with frequent apneic spells.

Nemaline myopathy is a genetically heterogeneous neuromuscular disorder and one of the most common congenital myopathies. The clinical manifestations usually vary depending on the age of onset. Neonatal nemaline myopathy has the worst prognosis, primarily due to respiratory failure. Several genes associated with nemaline myopathy have been identified, including NEB, ACTA1, TPM3, TPM2, TNNT1, CFL2, KBTBD13, KLHL40, KLHL41, LMOD3, and KBTBD13. Here, we report a neonatal Korean female patient with nemaline myopathy carrying compound heterozygous mutations in the gene KLHL40 as revealed using next generation sequencing (NGS). The patient presented with postnatal cyanosis, respiratory failure, dysphagia, and hypotonia just after birth. To identify the genetic cause underlying the neonatal myopathy, NGS-based gene panel sequencing was performed. Compound heterozygous pathogenic variants were detected in KLHL40: c.[1405G>T];[1582G>A] (p. [Gly469cys];[Glu528Lys]). NGS allows quick and accurate diagnosis at a lower cost compared to traditional serial single gene sequencing, which is greatly advantageous in genetically heterogeneous disorders such as myopathies. Rapid diagnosis will facilitate efficient and timely genetic counseling, prediction of disease prognosis, and establishment of treatments.

Nemaline myopathy is a genetically heterogeneous neuromuscular disorder and one of the most common congenital myopathies. The clinical manifestations usually vary depending on the age of onset. Neonatal nemaline myopathy has the worst prognosis, primarily due to respiratory failure. Several genes associated with nemaline myopathy have been identified, including NEB, ACTA1, TPM3, TPM2, TNNT1, CFL2, KBTBD13, KLHL40, KLHL41, LMOD3, and KBTBD13. Here, we report a neonatal Korean female patient with nemaline myopathy carrying compound heterozygous mutations in the gene KLHL40 as revealed using next generation sequencing (NGS). The patient presented with postnatal cyanosis, respiratory failure, dysphagia, and hypotonia just after birth. To identify the genetic cause underlying the neonatal myopathy, NGS-based gene panel sequencing was performed. Compound heterozygous pathogenic variants were detected in KLHL40: c.[1405G>T];[1582G>A] (p. [Gly469cys];[Glu528Lys]). NGS allows quick and accurate diagnosis at a lower cost compared to traditional serial single gene sequencing, which is greatly advantageous in genetically heterogeneous disorders such as myopathies. Rapid diagnosis will facilitate efficient and timely genetic counseling, prediction of disease prognosis, and establishment of treatments.

PURPOSE: Lymphoma originated from mucosa associated lymphoid tissue(MALT) is most common in gastrointestinal system, and rarely found in salivary gland, thyroid, bronchus or orbit. We experienced a case of MALT lymphoma which was originated from conjunctiva and involving lower eyelid without metastasis. METHODS: A 40-year-old man suffered palpable mass on right lower eyelid without pain. Orbital computed tomographic and ultrasonographic findings showed a conical mass(1.9x1.2x0.9cm size) inside lower eyelid. The mass was completely excised under local anesthesia and histopathological examination was followed. RESULTS: Microscopic finding showed a multiple follicular colonization. In the follicle, small lymphocytes and plasma cells differentiated to centrocyte-like cell, monocyte B cell, plasma cell were diffusely infiltrated. Immunophenotyping was preformed on fixed section. The majority of the small cells were immunoreactive for the B cell marker CD20. Based on the typical histological findings supported by immunostaining, the mass was defined as MALT lymphoma. After excision, SPECT, abdominal CT was carried out and there were no evidence of extraorbital disease. CONCLUSION: Biopsy and pathological examination should be performed in patients who complain palpable mass on lower eyelid because of possibility of MALT lymphoma. Although MALT lymphoma is rarely metastasized, it is necessary to evaluate the extraorbital involvement using SPECT or other radiologic exams. For detecting extraorbital involvement, periodic follow-up examination is need.
Adult ; Anesthesia, Local ; Biopsy ; Bronchi ; Colon ; Conjunctiva ; Eyelids ; Follow-Up Studies ; Humans ; Immunophenotyping ; Lymphocytes ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone ; Monocytes ; Mucous Membrane ; Orbit ; Plasma Cells ; Salivary Glands ; Thyroid Gland ; Tomography, Emission-Computed, Single-Photon

Xanthogranulomatous cholecystitis (XGC) is a rare inflammatory disease of the gallbladder. Not only does XGC occasionally present as a mass formation with adjacent organ invasion like a malignant neoplasm, it can also infrequently be associated with gallbladder cancer. In the situation, it is difficult to make a differential diagnosis between the diseases. Here, we describe a case of a simultaneous XGC and a carcinoma of the gallbladder in a 61-year-old woman. To the best of our knowledge, there are only a small number of reports on this combination of diseases.
Adenocarcinoma/complications/diagnosis/*pathology/surgery ; Biopsy, Needle ; Cholangiopancreatography, Endoscopic Retrograde ; Cholecystitis/complications/diagnosis/*pathology/surgery ; Endosonography ; Female ; Gallbladder Neoplasms/complications/diagnosis/*pathology/surgery ; Granuloma/complications/diagnosis/*pathology ; Humans ; Immunohistochemistry ; Middle Aged ; Neoplasm Staging ; Prognosis ; Risk Assessment ; Tomography, X-Ray Computed ; Xanthomatosis/complications/diagnosis/*pathology