No abstract available.
Amniotic Fluid* ; Corticotropin-Releasing Hormone* ; Female ; Obstetric Labor, Premature* ; Pregnancy

Pseudomyxoma peritonei is a poorly understood condition characterized by mucinous ascites and multifocal peritoneal mucinous tumors. Pseudomyxoma peritonei is most commonly associated with mucinous tumors of the ovary and appendix. When two lesions are morphologically similar, it is difficult to determine whether one reflects a metastasis or whether the tumors are independent. Many studies concluded that most cases in which both the appendix and ovary were involved were primary appendiceal tumor with secondary involvement of the ovary, but we have experienced one case of pseudomyxoma peritonei originated from independent primary borderline mucinous tumors of the ovary and appendix. So we report this case with the brief review of literatures
Appendix* ; Ascites ; Female ; Mucins* ; Neoplasm Metastasis ; Ovary* ; Pseudomyxoma Peritonei*

PURPOSE: The purpose of this study was to evaluate the short- and long-term effectivensess of intracavitary urokinase with percutaneous catheter drainage in Ioculated thoracic empyemas. MATERIALS AND METHODS: 15 patients were identified as second stage of Ioculated thoracic empyema by estimating nature of pleural fluid, chest PA, lateral decubitus view and CT scan. Under the guidance of fluoroscopy or ultrasound, catheter was inserted percutaneously. Instillation of urokinase was started when amount of drained fluid became less than 30ml per day with 100,000U of urokinase mixed with 100ml of normal saline. Trial of urokinase was repeated until complete drainage of empyema was demonstrated on plain chest film obtained after 48 hours. RESULTS: Successful complete drainage was achieved in 14 of 15 patients. In long-term study, complete resorption was demonstrated in 11 of 12 patients. Average dosage of used urokinase was 330,000U and mean duration of catherter insertion was 35 days. CONCLUSION: lntracavitary urokinase with percutaneous catheter drainage is a safe and effective method to facilitate drainage of Ioculated empyema and to prevent recurrence.
Catheters ; Drainage ; Empyema ; Empyema, Pleural* ; Fluoroscopy ; Humans ; Recurrence ; Thorax ; Tomography, X-Ray Computed ; Ultrasonography ; Urokinase-Type Plasminogen Activator*

PURPOSE: The purpose of this study was to evaluate the short- and long-term effectivensess of intracavitary urokinase with percutaneous catheter drainage in Ioculated thoracic empyemas. MATERIALS AND METHODS: 15 patients were identified as second stage of Ioculated thoracic empyema by estimating nature of pleural fluid, chest PA, lateral decubitus view and CT scan. Under the guidance of fluoroscopy or ultrasound, catheter was inserted percutaneously. Instillation of urokinase was started when amount of drained fluid became less than 30ml per day with 100,000U of urokinase mixed with 100ml of normal saline. Trial of urokinase was repeated until complete drainage of empyema was demonstrated on plain chest film obtained after 48 hours. RESULTS: Successful complete drainage was achieved in 14 of 15 patients. In long-term study, complete resorption was demonstrated in 11 of 12 patients. Average dosage of used urokinase was 330,000U and mean duration of catherter insertion was 35 days. CONCLUSION: lntracavitary urokinase with percutaneous catheter drainage is a safe and effective method to facilitate drainage of Ioculated empyema and to prevent recurrence.
Catheters ; Drainage ; Empyema ; Empyema, Pleural* ; Fluoroscopy ; Humans ; Recurrence ; Thorax ; Tomography, X-Ray Computed ; Ultrasonography ; Urokinase-Type Plasminogen Activator*

No abstract available.

No abstract available.
Cisterna Magna*

Nephrocalcinosis often occurs in infants and is caused by excessive calcium or vitamin D supplementation, neonatal primary hyperparathyroidism, and genetic disorders. Idiopathic infantile hypercalcemia (IIH), a rare cause of nephrocalcinosis, results from genetic defects in CYP24A1 or SLC34A1. Mutations in CYP24A1, which encodes 25-hydroxyvitamin D 24-hydroxylase, disrupt active vitamin D degradation. IIH clinically manifests as failure to thrive and hypercalcemia within the first year of life and usually remits spontaneously. Herein, we present a case of IIH wih CYP24A1 mutations.An 11-month-old girl visited our hospital with incidental hypercalcemia. She showed failure to thrive, and her oral intake had decreased over time since the age of 6 months. Her initial serum parathyroid hormone level was low, 25-OH vitamin D and 1,25-OH vitamin D levels were normal, and renal ultrasonography showed bilateral nephrocalcinosis. Whole-exome sequencing revealed compound heterozygous variants in CYP24A1 (NM_000782.4:c.376C>T [p.Pro126Ser] and c.1310C>A [p.Pro437His]). Although her hypercalcemia and poor oral intake spontaneously resolved in approximately 8 months, we suggested that her nephrocalcinosis and renal function be regularly checked in consideration of potential asymptomatic renal damage. Hypercalcemia caused by IIH should be suspected in infants with severe nephrocalcinosis, especially when presenting with failure to thrive.

Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury without any association with preceding diarrhea. Dysregulation of the complement system is the most common cause of aHUS, and monoclonal humanized anti-C5 antibodies are now recommended as the first-line treatment for aHUS. However, if the complement pathway is not the cause of aHUS, C5 inhibitors are ineffective. In this study, we report the second reported case of aHUS caused by DGKE mutations in Republic of Korea. The patient was an 11-month-old infant who presented with prodromal diarrhea similar to typical HUS, self-remitted with conservative management unlike complement-mediated aHUS but recurred with fever. While infantile aHUS often implies genetic dysregulation of the complement system, other rare genetic causes, such as DGKE mutation, need to be considered before deciding long-term treatment with C5 inhibitors.

Purpose: Hematuria and proteinuria have various causes and consequential outcomes in children. Immunosuppressants are needed in some children with biopsy-proven glomerulonephropathy but have many adverse effects. Since the clinical practice patterns of Korean pediatric nephrologists are diverse, we surveyed their opinions. Methods: Using a clinical vignette, the survey was emailed to all Korean Society of Pediatric Nephrology members. The questionnaires included diagnosis, examination, medications, and dietary recommendations for patients with hematuria and proteinuria. Results: A total of 32 clinicians (5.48%, 22 pediatric certificated nephrologists) responded to the survey. Most responders (87.5%) suspected immunoglobulin A nephropathy, and 68.8% replied that kidney biopsies were a diagnostic tool. Renin-angiotensin system inhibition (62.5%) or steroids (18.8%) were selected as the treatment. Salt and protein intakes were usually encouraged as dietary reference intakes (34.4% and 65.6%, respectively). Conclusions Children with abnormal urinalysis have various causes, treatments, and prognoses. As treatments such as immunosuppressants can have many adverse effects, it is necessary to confirm an accurate diagnosis and indications of treatments before starting the treatment. Recommendations for a diet should not hinder growth.

Seborrheic keratosis is one of the most common benign growths of the skin, arising from keratinocytes within the epidermis. Unlike actinic keratosis, which frequently progresses to squamous cell carcinoma, seborrheic keratosis generally has a low risk of malignancy. The reasons for this low risk are not fully understood, but some studies suggest it may be due to the lack of mutations in tumor suppressor genes. This case report describes a rare instance of squamous cell carcinoma arising from a long-standing seborrheic keratosis located in a sun-protected area. We performed immunohistochemical staining for tumor suppressor proteins p53 and p16, as well as the proliferation marker Ki-67. Based on this case, we speculate that cumulative mutations in tumor suppressor genes might play a role in the progression of seborrheic keratosis to squamous cell carcinoma. Further studies involving a larger sample size and combining immunohistochemical staining with genomic analysis are necessary to elucidate the mechanisms underlying malignant transformation in seborrheic keratosis.