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1.
Pediatric Emergency Medicine Journal ; : 72-75, 2023.
Article in Korean | WPRIM | ID: wpr-968477

ABSTRACT

Listeria monocytogenes is a rare cause of bacterial meningitis, particularly beyond the neonatal period. This article describes a 16-month-old girl who presented with a new-onset febrile status epilepticus, and subsequently developed altered mentality and lethargy. L. monocytogenes was detected on a point-of-care, multiplex polymerase chain reaction using the cerebrospinal fluid. On day 3, she developed a rapidly progressive hydrocephalus. Her consciousness improved after placement of an external ventricular drain. After 3-week antibiotic therapy, she was successfully discharged without residual complications.

2.
Journal of Korean Medical Science ; : e91-2023.
Article in English | WPRIM | ID: wpr-967478

ABSTRACT

Background@#The value of tiotropium bromide (TIO) in neutrophilic asthma was meaningful in previous study. We hypothesized that TIO’s mechanism of action is associated with histone deacetylase 2 (HDAC2) activity, which is key for controlling the transcription of inflammatory cytokines and usually downregulated in neutrophilic asthma. @*Methods@#The effects of TIO and dexamethasone (DEX) on HDAC2 activity, nuclear factor kappa B (NF-κB), and C-X-C motif chemokine ligand 1 (CXCL1) were evaluated in neutrophilic asthma mouse model (C57BL, 6-week-old). An in-vitro study was conducted using primary human bronchial/tracheal epithelial (HBE) cells from asthma patients.Western blot analyses were performed for phospho-phospholipase Cγ-1 (PLCγ-1) and inositol trisphosphate (IP3 ) receptors (IP3 R) with treating lipopolysaccharide (LPS) and TIO. @*Results@#Ovalbumin was used to induce eosinophilic inflammation in this study. After neutrophilic asthma was induced by LPS (O+L group), HDAC2 activity was diminished with increased NF-κB activity and CXCL1 compared to the control group. TIO significantly improved NF-κB activity, CXCL1, and HDAC2 activity compared with the O+L group in in-vivo study (P < 0.05, each). Western blot analyses showed that LPS treated HBE cells from asthma patients increased PLCγ-1 and diminished IP3 receptor levels. After TIO treatment, recovery of IP3 R and improved PLCγ-1 levels were observed. @*Conclusion@#These results support the hypothesis that TIO modulates inflammation by recovering HDAC2 activity from the acetylcholine-stimulated inflammation cascade in neutrophilic asthma. The detailed inflammation cascade of recovering HDAC2 activity by TIO might be associated with PLCγ-1-IP3-IP3R mediated intracellular calcium ion pathway.

3.
Allergy, Asthma & Respiratory Disease ; : 26-33, 2023.
Article in English | WPRIM | ID: wpr-966199

ABSTRACT

Purpose@#We aimed to evaluate the usefulness and safety of high-flow nasal cannula (HFNC) oxygen therapy in children with complex chronic diseases (CCD) with impending respiratory failure in the general ward. @*Methods@#Medical records of subjects with HFNC oxygen use in the general ward at a Korean tertiary children’s hospital were reviewed. Children with CCD and impending respiratory failure were included. treatment success was defined as successful weaning from HFNC oxygen support and treatment failure as weaning failure that led to higher level of respiratory support such as invasive ventilation or noninvasive positive pressure ventilation. @*Results@#Fifty cases were included. Thirty-five cases (70%) were weaned off HFNC oxygen successfully, 15 cases (30%) failed. At the time of HFNC oxygen administration, the treatment failure group showed higher heart rate (P = 0.043), carbon dioxide partial pressure (P = 0.002), and initial inspired oxygen fraction (P = 0.007). Within 72 hours of initial treatment, 20% of patients in the treatmentsuccess group were weaned off the HFNC oxygen and half in the treatment-failure group required invasive ventilation. One case experienced complication. @*Conclusion@#HFNC oxygen is safe and effective for respiratory support in the general ward to avoid invasive mechanical ventilation in children with CCD and impending respiratory failure.

4.
Journal of the Korean Ophthalmological Society ; : 734-742, 2023.
Article in Korean | WPRIM | ID: wpr-1001762

ABSTRACT

Purpose@#This study introduces a new machine learning-based auto-merge program (HydraVersion) that automatically combines multiple ocular photographs into single nine-directional ocular photographs. We compared the accuracy and time required to generate ocular photographs between HydraVersion and PowerPoint. @*Methods@#This was a retrospective study of 2,524 sets of 250 nine-directional ocular photographs (134 patients) between March 2016 and June 2022. The test dataset comprised 74 sets of 728 photographs (38 patients). We measured the time taken to generate nine-directional ocular photographs using HydraVersion and PowerPoint, and compared their accuracy. @*Results@#HydraVersion correctly combined 71 (95.95%) of the 74 sets of nine-directional ocular photographs. The average working time for HydraVersion and PowerPoint was 2.40 ± 0.43 and 255.9 ± 26.7 seconds, respectively; HydraVersion was significantly faster than PowerPoint (p < 0.001). @*Conclusions@#Strabismus and neuro-ophthalmology centers are often unable to combine and store photographs, except those of clinically significant cases, because of a lack of time and manpower. This study demonstrated that HydraVersion may facilitate treatment and research because it can quickly and conveniently generate nine-directional ocular photographs.

5.
Journal of Korean Medical Science ; : e358-2023.
Article in English | WPRIM | ID: wpr-1001181

ABSTRACT

Background@#In pediatric patients, the common cold coronavirus (ccCoV) usually causes mild respiratory illness. There are reports of coronavirus causing central nervous system (CNS) infection in experimental animal models. Some immunocompromised patients have also been reported to have fatal CNS infections with ccCoV. The aim of this study was to investigate the clinical characteristics of CNS complications related to ccCoV infection. @*Methods@#From January 2014 to December 2019, a retrospective analysis was performed of medical records from hospitalized patients under 19 years of age whose ccCoV was detected through polymerase chain reaction in respiratory specimens. The CNS complications were defined as clinically diagnosed seizure, meningitis, encephalopathy, and encephalitis. @*Results@#A total of 436 samples from 420 patients were detected as ccCoV. Among the 420 patients, 269 patients were immunocompetent and 151 patients were immunocompromised.The most common type of ccCoV was OC43 (52% in immunocompetent, 37% in immunocompromised). CNS complications were observed in 9.4% (41/436). The most common type of CNS complication was the fever-provoked seizure under pre-existing neurologic disease (42% in immunocompetent and 60% in immunocompromised patients).Among patients with CNS complications, two immunocompetent patients required intensive care unit admission due to encephalitis. Three patients without underlying neurological disease started anti-seizure medications for the first time at this admission. There was no death related to ccCoV infection. @*Conclusion@#ccCoV infection may cause severe clinical manifestations such as CNS complications or neurologic sequelae, even in previously healthy children.

6.
Journal of the Korean Ophthalmological Society ; : 423-430, 2023.
Article in Korean | WPRIM | ID: wpr-977095

ABSTRACT

Purpose@#To investigate the long-term outcomes of anterior chamber angle parameters in patients with primary angle closure glaucoma (PACG) after peripheral laser iridotomy (LI) combined with iridoplasty (PI) using a dual Scheimpflug analyzer. @*Methods@#This retrospective study included 32 eyes in 32 patients diagnosed with PACG who underwent LI plus PI. Patients with an acute angle closure crisis were excluded. Dual Scheimpflug analyzer images were acquired before and 1 week after LI plus PI, and yearly afterwards. Anterior chamber depth (ACD) and volume (ACV), mean anterior chamber angle (ACA), and intraocular pressure (IOP) were also measured. @*Results@#The mean follow-up was 32.28 ± 13.34 months. Baseline demographics were age 63 ± 7.9 years, 62.5% female, IOP 15.48 ± 4.79 mmHg, ACD 2.09 ± 0.19 mm, and visual field mean deviation -7.97 ± 8.49 dB. ACD increased from baseline to 2.15 ± 0.32 mm, but it was not significant (p = 0.136). ACV increased significantly from 78.32 ± 11.49 mm at baseline to 83.04 ± 11.16 mm at the last visit after LI (p = 0.011). The mean ACA increased significantly from 26.86 ± 2.53° at baseline to 28.82 ± 4.64° at the last visit (p = 0.022). IOP decreased significantly from baseline to 13.06 ± 2.21 mmHg at the last visit (p = 0.001). @*Conclusions@#The ACA parameters improved after LI combined with PI in patients with PACG and remained so on long-term follow-up. IOP was also significantly reduced for more than 2.5 years after LI plus PI.

7.
Korean Journal of Radiology ; : 271-279, 2022.
Article in English | WPRIM | ID: wpr-918220

ABSTRACT

Objective@#To compare the clinical and radiologic findings between perforated and non-perforated choledochal cysts in children. @*Materials and Methods@#Fourteen patients (mean age ± standard deviation, 1.7 ± 1.2 years) with perforated choledochal cysts (perforated group) and 204 patients (3.6 ± 3.8 years) with non-perforated choledochal cysts (non-perforated group) were included between 2000 and 2019. All patients underwent choledochal cyst excision after ultrasound, CT, or MR cholangiopancreatography. Relevant data including demographics, clinical symptoms, laboratory findings, imaging findings, and outcomes were analyzed. Statistical differences were compared using the Mann-Whitney U test and Fisher’s exact test. @*Results@#Choledochal cyst perforation occurred only in children under the age of 4 years. Acute symptoms, including fever (p < 0.001), were more common in the perforated group than in the non-perforated group. High levels of white blood cells (p = 0.004), C-reactive protein (p < 0.001), and serum amylase (p = 0.002), and low levels of albumin (p < 0.001) were significantly associated with the perforated group. All 14 patients with perforated choledochal cysts had ascites, whereas only 16% (33/204) of patients in the non-perforated group had ascites (p < 0.001). In the subgroup of patients who had ascites, a large amount of ascites (p = 0.001), increase in the amount of ascites in a short time (p < 0.001), complex ascites (p < 0.001), and perihepatic pseudocysts (p < 0.001) were more common in the perforated group than in the non-perforated group. @*Conclusion@#Children with perforated choledochal cysts have characteristic clinical and radiologic findings compared to those with non-perforated choledochal cysts. In young children with choledochal cysts, perforation should be differentiated in cases with acute symptoms, laboratory abnormalities, and characteristic ascites findings.

8.
Pediatric Infection & Vaccine ; : 118-123, 2022.
Article in English | WPRIM | ID: wpr-968518

ABSTRACT

A dermatophyte is a zoonotic infection that causes infection on the skin, hair, and nails and is transmitted through contact. The species of Microsporum, Trichophyton, and Epidermophyton are the major dermatophytes that infect humans. Tinea capitis is a dermatophyte infection in the scalp, and it may progress to Kerion celsi, including severe redness, swelling, and pus formation. Kerion celsi is sometimes misdiagnosed as a bacterial infection or a tumor. Trichophyton verrucosum is a zoophilic dermatophyte that mainly causes infection in cattle. It can be spread to dairy farmers or ranchers who have frequent contact with infected cattle. We report a pediatric case who received scalp tumor excision and a split-thickness skin graft for extensive and severe inflammatory scalp tumors that occurred after contact with cattle with ringworm. Finally, the patient was diagnosed with Kerion celsi caused by T. verrucosum infection. This was based on the patient’s medical history, clinical manifestations, and histopathologic findings.

9.
Korean Journal of Dermatology ; : 666-674, 2022.
Article in English | WPRIM | ID: wpr-968047

ABSTRACT

Background@#Prurigo nodularis (PN) is a chronic pruritic skin disorder with a large number of hyperkeratotic nodules. The precise mechanisms of its pathogenesis remain unknown. PN has been linked to atopic dermatitis (AD), but its association remains unclear. @*Objective@#We aimed to investigate the clinical, histological, and immunohistochemical characteristics of patients with PN and PN underlying AD (PN-AD). @*Methods@#Eight patients were recruited for PN, PN-AD, and eight normal subjects, respectively. Skin tissues were obtained from patients and healthy subjects for histological and immunohistochemical analyses. @*Results@#Histological examination showed increased epidermal thickness and dermal inflammatory cell counts in the PN-AD and PN groups compared to normal subjects. Immunohistochemical analyses revealed that the expression of interleukin (IL)-4, IL-13, IL-18, IL-31, IL-33, interferon (IFN)-γ, stromal-derived factor (SDF) 1-α and thymic stromal lymphopoietin (TSLP) was increased in the tissues of PN-AD and PN groups, in which the staining intensities of IL-4, IL-13, SDF1-α and TSLP in the PN-AD group were higher than those in the PN group, but the differences were not statistically significant. Conversely, the staining intensities of IL-18, IL-33 and IFN-γ were significantly higher in the PN group than those in the PN-AD group. @*Conclusion@#The pathogenesis of PN may differ from that of PN-AD, in which IL-18, IL-33 and IFN-γ may be associated, implying that epidermal injury is the initial cause of IL-18 and IL-33 induction, which then increases IFN-γ, resulting in the inflammatory process of PN.

10.
Journal of Acute Care Surgery ; (2): 43-46, 2022.
Article in English | WPRIM | ID: wpr-925135

ABSTRACT

Oral care is easily neglected in patients in an intensive care unit (ICU) because they are often intubated or have altered mental status. Although care workers pay careful attention to the mouth, tooth loss often occurs in the ICU. Here we report 3 cases of dental bridge loss undetected by the ICU staff. One patient was under mechanical ventilation via an endotracheal tube after emergency intubation, whilst 2 patients were drowsy but not intubated. Consecutive chest X-rays revealed dental bridge loss in all 3 cases, but this was not identified immediately. Along with other critical management approaches, these cases demonstrate how an initial evaluation of the oral cavity, with special attention to the number of teeth, and the existence of dental prosthetics is essential to preventing potential deleterious complications. The number of teeth and the existence of dental prosthetics must be documented in ICU patients.

11.
Tuberculosis and Respiratory Diseases ; : 18-24, 2022.
Article in English | WPRIM | ID: wpr-919476

ABSTRACT

Background@#Neutrophilic asthma (NeuA) is usually resistant to corticosteroids. Tiotropium bromide (TIO) is a bronchodilator that is used as an add-on therapy to inhaled corticosteroid and long-acting β2 agonist in asthma treatment. However, the role of TIO in NeuA is not fully known. Thus, the aim of this study was to evaluate the effect of TIO on NeuA compared to that of corticosteroids. @*Methods@#C57BL/6 female mice were sensitized with ovalbumin and lipopolysaccharide to induce neutrophilic inflammation. Dexamethasone (DEX) was administered on days 14, 17, 20, and 23. TIO was inhaled on days 21, 21, and 23. On day 24, mice were sacrificed. Airway hyper-responsiveness, levels of cytokines in bronchoalveolar lavage (BAL) and lung homogenates, and lung tissue histopathology were compared between the two groups. @*Results@#Neutrophil counts, T helper 2 cells (TH2)/TH17 cytokines, and pro-inflammatory cytokine in BAL fluids were elevated in the NeuA group. TIO group showed lower total cells, neutrophil counts, and eosinophil counts in BAL fluids than the DEX group (p<0.001, p<0.05, and p<0.001, respectively). Airway resistance was attenuated in the TIO group but elevated in the NeuA group (p<0.001). Total protein, interleukin (IL)-5, and IL-17A levels in BAL fluids were lower in the TIO group than in the NeuA group (all p<0.05). @*Conclusion@#TIO showed more potent effects than DEX in improving airway inflammation and attenuating airway resistance in NeuA.

12.
Journal of Korean Neurosurgical Society ; : 397-407, 2022.
Article in English | WPRIM | ID: wpr-926031

ABSTRACT

Abusive head trauma (AHT) is the most common and serious form of child abuse and a leading cause of traumatic death in infants and young children. The biomechanics of head injuries include violent shaking, blunt impact, or a combination of both. Neuroimaging plays an important role in recognizing and distinguishing abusive injuries from lesions from accidental trauma or other causes, because clinical presentation and medical history are often nonspecific and ambiguous in this age group. Understanding common imaging features of AHT can increase recognition with high specificity for AHT. In this review, we discuss the biomechanics of AHT, imaging features of AHT, and other conditions that mimic AHT.

13.
The Korean Journal of Internal Medicine ; : 830-840, 2022.
Article in English | WPRIM | ID: wpr-939105

ABSTRACT

Background/Aims@#Membranous nephropathy (MN) is a major cause of nephrotic syndrome in adults. This study aimed to evaluate the effect of rituximab (RTX) in patients with idiopathic MN (iMN) who have a high risk of progression. @*Methods@#We retrospectively analyzed data of 13 patients with iMN, who received RTX treatments from January 2014 to July 2020. RTX was indicated in patients with iMN with severe proteinuria and decreasing estimated glomerular filtration rate (eGFR) in the previous 6 months despite other immunosuppressive therapies. @*Results@#The patients were predominantly males (n = 11) and with a mean age of 55.3 years; median eGFR, 37.0 mL/min/1.73 m2 (interquartile range [IQR], 26.3 to 66.5); serum albumin level, 2.6 g/dL (IQR, 1.9 to 3.1); and spot urine protein-to-creatinine ratio at baseline, 6.6 g/g (IQR, 5.7 to 12.9). In a median follow-up of 22 months, eight patients (61.5%) achieved complete or partial remission. In responder group (n = 8), median eGFR increased from 31.5 to 61.5 mL/min/1.73 m2 (p = 0.049) and serum albumin level increased from 2.3 to 4.2 g/dL (p = 0.017) from RTX initiation to last follow-up. Antiphospholipase A2 receptor antibody (anti-PLA2R-Ab) was positive in six among seven tested patients, which markedly decreased in the responder group. There were no adverse events after RTX. @*Conclusions@#This study suggests that RTX is a safe and effective treatment option for patients with iMN who have a high risk of progression. Individualized therapy based on anti-PLA2R-Ab titer would be needed for better outcomes.

14.
Journal of Korean Diabetes ; : 144-152, 2022.
Article in Korean | WPRIM | ID: wpr-939042

ABSTRACT

Aceruloplasminemia (ACP) is a rare genetic disorder characterized by the triad of diabetes mellitus, retinal degeneration, and neurological symptoms. The patient’s clinical and biochemical features highlight substantial phenotype heterogeneity, contributing to the difficulties and delay in diagnosing ACP. We described a patient presenting with diabetes and abnormal iron studies due to ACP with typical neuroradiologic abnormality. A 56-year-old female patient visited our hospital with complaints of weight loss and anxiety. The leading cause of unintentional weight loss was uncontrolled diabetes. She was treated with oral hypoglycemic agents. Initial blood tests revealed unexplained microcytic anemia and high ferritin levels. We performed magnetic resonance imaging (MRI) of the brain to alleviate her excessive concerns about normal memory loss. We suspected that she might have ACP, based on the results of cortical pencil lining sign of the brain MRI and microcytic anemia with decreased ceruloplasmin (CP) and increased ferritin levels. Sequence analysis of the CP gene revealed homozygosity for c.2630 G>A, confirming the clinical diagnosis of ACP. The patient was started on deferasirox with progressive normalization of ferritin. In conclusion, unexplained anemia, often with microcytosis, diabetes, and typical neuroradiologic findings, is the best clue for early diagnosis of ACP.

15.
Journal of the Korean Society of Emergency Medicine ; : 314-321, 2022.
Article in Korean | WPRIM | ID: wpr-938353

ABSTRACT

Objective@#This study compared the treatment results of emergency department (ED) patients presenting with gingival bleeding, who were given dental consultations with those who were not. Also, the study compared patients presenting with gingival bleeding based on the period of availability of the dental consultation in the ED. @*Methods@#This was a retrospective study of patients with gingival bleeding visiting the ED from January 2011 to April 2020. The demographic data of patients, past medical history, etiology of bleeding, vital signs, lab findings, treatment methods, and disposition were analyzed. @*Results@#The mean level of hemoglobin was 13.3 g/dL in the group to whom dental consultation was made available, and 11.7 g/dL in the group not given dental consultation, which was a significant difference (P=0.010). Vitamin K injections were given to 1.9% of the former group and 6.2% of the latter group (P=0.049). Suturing was done in 14% of the former group and 1.2% of the latter (P<0.001). There were no significant differences between the two groups regarding admission to the ward or revisits to the ED. @*Conclusion@#Emergency physicians carried out conservative treatments for patients with gingival bleeding when dental consultation was unavailable. No differences in the treatment results were observed based on the length of time the dental consultation was made available.

16.
Journal of Korean Diabetes ; : 153-159, 2021.
Article in Korean | WPRIM | ID: wpr-918907

ABSTRACT

The present article demonstrates an unusual case of type 1 autoimmune pancreatitis (AIP), focusing on the cause of deterioration in glycemic control and weight loss in a patient with pre-existing type 2 diabetes mellitus (DM). A 67-year-old man was diagnosed with type 2 DM 23 years prior and presented with weight loss of approximately 6 kg over a period of 3 months and hemoglobin A1c (HbA1c) of 9.1%. His carbohydrate antigen 19-9 (CA 19-9) level was elevated to 158.54 U/mL (normal, 0~37 U/mL). Considering these findings, we needed to rule out hidden malignancy. Computed tomography of the abdomen showed diffuse swelling of the pancreas uncinated process. The serum immunoglobulin G (IgG) level was elevated to 2,418 mg/dL (normal, 700~1,600 mg/dL), and IgG4 level was elevated to 1,115.0 mg/dL (normal range, 3.9~86.4 mg/dL). This case highlights that AIP should be considered as a cause of significant weight loss and a deterioration in glycemic control in patients with DM. Furthermore, a pancreatic imaging study should be considered in clinical practice to differentiate pancreatic cancer and AIP.

17.
Journal of Korean Diabetes ; : 225-237, 2021.
Article in Korean | WPRIM | ID: wpr-918905

ABSTRACT

The accuracy and convenience of continuous glucose monitoring (CGM), which efficiently evaluates glycemic variability and hypoglycemia, are improving. There are two types of CGM: professional CGM and personal CGM. Personal CGM is subdivided into real-time CGM (rt-CGM) and intermittently scanned CGM (isCGM). CGM is being emphasized in both domestic and foreign diabetes management guidelines. Regardless of age or type of diabetes, CGM is useful for diabetic patients undergoing multiple insulin injection therapy or using an insulin pump. rt-CGM is recommended for all adults with type 1 diabetes (T1D), and can also be used in type 2 diabetes (T2D) treatments using multiple insulin injections. In some cases, short-term or intermittent use of CGM may be helpful for patients with T2D who use insulin therapy other than multiple insulin injections and/or oral hypoglycemic agents. CGM can help to achieve A1C targets in diabetes patients during pregnancy. CGM is a safe and cost-effective alternative to self-monitoring blood glucose in T1D and some T2D patients. CGM used in diabetes management works optimally with proper education, training, and follow up. To achieve the activation of CGM and its associated benefits, it is necessary to secure sufficient repetitive training and time for data analysis, management, and education. Various supports such as compensation, insurance coverage expansion, and reimbursement are required to increase the effectiveness of CGM while considering the scale of benefit recipients, policy priorities, and financial requirements.

18.
Journal of the Korean Ophthalmological Society ; : 1490-1501, 2021.
Article in Korean | WPRIM | ID: wpr-916404

ABSTRACT

Purpose@#To evaluate the repeatability of retinal nerve fiber layer (RNFL) thickness and Bruch’s membrane opening-minimum rim width (BMO-MRW) measurements by spectral-domain optical coherence tomography (SD-OCT) in wet age-related macular degeneration (wAMD) and diabetic macular edema (DME). @*Methods@#This was a prospective study. The RNFL thickness and BMO-MRW parameters for each sector and global average were measured twice by SD-OCT. Repeatability was evaluated using the intraclass correlation coefficient (ICC) and coefficient of variation (CV). If the optic disc membrane was confirmed, it was analyzed by dividing it into three groups based on severity. @*Results@#A total of 99 eyes (48 with wAMD, 51 with DME) were included in the analysis. The ICCs of the global RNFL thickness and global BMO-MRW measurements were 0.996 and 0.997, respectively, in wAMD and 0.994 and 0.996, respectively, in DME eyes. The CV values of global RNFL thickness and BMO-MRW were 0.60% and 0.73%, respectively, in wAMD eyes and 1.10% and 1.21%, respectively, in DME eyes. The disc membrane on the optic nerve head significantly affected global BMO-MRW repeatability (B = 0.814, p < 0.001). @*Conclusions@#Both RNFL thickness and BMO-MRW measurements showed good repeatability in eyes with wAMD and DME. The severity of the optic disc membrane significantly affected the repeatability of BMO-MRW measurements in eyes with wAMD and DME. Therefore, physicians should examine the BMO-MRW in eyes with severe optic disc membrane.

19.
Allergy, Asthma & Respiratory Disease ; : 216-224, 2021.
Article in English | WPRIM | ID: wpr-913323

ABSTRACT

Purpose@#Sleep-disordered breathing is one of the complicating characteristics in patients with Prader-Willi syndrome (PWS). No detailed description and risk factors are suggested on breathing problems during sleep in Korean children with PWS. @*Methods@#We reviewed clinical and sleep-study data in patients with PWS who underwent polysomnography before they took the growth hormone therapy. @*Results@#Of the 27 patients with PWS, 25 (92.6%) had sleep-disordered breathing, of whom 14 showed moderate to severe sleep apnea. Obstructive dominance was prevalent (64%), followed by central dominance (24%). The apnea-hypopnea index (AHI) increased with increasing weight-for-height z-score (WHZ) (r = 0.50, P = 0.009), but did not differ by age. Apnea duration of over 12 months was longer in the patient group than in the infant group (15.1 ± 4.3 seconds vs. 9.4 ± 1.7 seconds, P = 0.001) and in the obese than nonobese groups (16.8 ± 4.3 seconds vs. 10.0 ± 2.0 seconds, P = 0.003). Desaturation below 70% was more common in the obese than nonobese subjects (3/9 vs. 0/18, P = 0.029). Age was not different between the central and obstructive apnea groups, but patients with central apnea tended to be younger than patients with obstructive apnea (median [range]: 8.0 months [6.0–12.0 months] vs.16.5 months [8.5–79.5 months], P = 0.092). In addition, patients with obstructive apnea showed higher AHI (12.8 [5.9–19.2] vs. 3.9 [3.4–4.5], P = 0.045). @*Conclusion@#Sleep-disordered breathing is common in PWS children with different intensity and patterns according to age and BMI. Close monitoring of breathing problems during sleep is required in PWS patients.

20.
Journal of the Korean Radiological Society ; : 267-273, 2021.
Article in English | WPRIM | ID: wpr-875114

ABSTRACT

Lymphomatoid granulomatosis (LG) is a rare B-cell type angiocentric lymphoproliferative disease that can progress to extranodal lymphoma with high mortality. It most commonly affects the lungs, although extrapulmonary systems, including the brain and skin, can also be involved. LG in pediatric patients has been very rarely reported in the literature with limited imaging features. Herein, we report a pediatric case of LG involving the lung and brain with characteristic imaging findings.

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