1.Management of Hyperphagia and Obesity in Prader–Willi Syndrome
The Ewha Medical Journal 2023;46(S1):e32-
Prader-Willi syndrome (PWS) is a neurodevelopmental disorder caused by the absence of paternally expressed imprinted genes on chromosome 15q11–13. Individuals with PWS typically experience feeding difficulties and a lack of appetite in infancy, followed by weight gain, uncontrolled appetite, and a lack of satiety. Hyperphagia in PWS is exacerbated by impaired satiety, low energy expenditure, and intellectual difficulties, including obsessive-compulsive disorder and/or autistic behaviors.Without rigorous external management of their eating behaviors, patients with PWS become severely obese and are at a higher risk of obesity-related morbidities, such as type 2 diabetes, obstructive sleep apnea, and hypertension. Moreover, the main causes of death for PWS are obesity-related comorbidities, such as renal failure, pulmonary embolism, and respiratory and heart failure. Clinical experiences with different supplements, diets, and other methods have not been encouraging.However, therapeutic options for patients with PWS may be improving, based on recent clinical trials for a number of medications. This report reviews the causes and management of hyperphagia, as well as previous and recent clinical trials aimed at treating hyperplasia in PWS. We are optimistic that the novel treatments currently in development will help alleviate the complex metabolic issues associated with PWS.
2.Two Cases of Tinea Faciei Mimicking Eczema Herpeticum in Siblings
JiHoon HWANG ; Ji-Man KANG ; Jong Gyun AHNC
Pediatric Infection & Vaccine 2021;28(1):49-56
Tinea faciei is a rare dermatophyte infection of the face that most often appears as a facial rash, followed by patches of small and raised bumps. Since it is uncommon in children and has similar rash patterns with various skin diseases, it is likely to be misdiagnosed as herpes simplex infection, contact dermatitis, disc-shaped lupus erythematosus, acne, and atopic dermatitis. In this case, siblings aged 3 and 4 were hospitalized due to skin rashes that occurred after traveling to Vietnam, and were administered antiviral drugs and systemic steroids under suspicion of herpes simplex infection with atopic dermatitis. Despite administration of these drugs, skin lesions did not show improvement. Serum beta-Dglucan assays were elevated in both patients, and after approximately 2 weeks, Trichophyton interdigitale was cultured in the older sister's skin fungal culture test. Both patients recovered after local and systemic antifungal therapy, without relapse or side effects. Skin lesions on the face, which do not respond to the existing treatment in children, should be checked for the possibility of tinea faciei through repeated fungal tests, and the beta-D-glucan assay can be a useful tool in diagnosing tinea faciei.
3.Two Cases of Tinea Faciei Mimicking Eczema Herpeticum in Siblings
JiHoon HWANG ; Ji-Man KANG ; Jong Gyun AHNC
Pediatric Infection & Vaccine 2021;28(1):49-56
Tinea faciei is a rare dermatophyte infection of the face that most often appears as a facial rash, followed by patches of small and raised bumps. Since it is uncommon in children and has similar rash patterns with various skin diseases, it is likely to be misdiagnosed as herpes simplex infection, contact dermatitis, disc-shaped lupus erythematosus, acne, and atopic dermatitis. In this case, siblings aged 3 and 4 were hospitalized due to skin rashes that occurred after traveling to Vietnam, and were administered antiviral drugs and systemic steroids under suspicion of herpes simplex infection with atopic dermatitis. Despite administration of these drugs, skin lesions did not show improvement. Serum beta-Dglucan assays were elevated in both patients, and after approximately 2 weeks, Trichophyton interdigitale was cultured in the older sister's skin fungal culture test. Both patients recovered after local and systemic antifungal therapy, without relapse or side effects. Skin lesions on the face, which do not respond to the existing treatment in children, should be checked for the possibility of tinea faciei through repeated fungal tests, and the beta-D-glucan assay can be a useful tool in diagnosing tinea faciei.
4.Treatment of Ulnar Olecranon Fracture Using Acutrak Screw.
Hyungchun KIM ; Kwangryul KIM ; Moonsup LIM ; Youngil PARK ; Inhwan HWANG ; Jihoon LEE
Journal of the Korean Fracture Society 2009;22(4):270-275
PURPOSE: To evaluate the clinical results of Acutrak screw fixation for ulnar olecranon fractures. MATERIALS AND METHODS: We reviewed 15 cases of ulnar olecranon fractures which were treated with Acutrak screws from February 2003 to September 2007. Follow-up period is from 12 months to 42 months. We used Mayo classification. Radiologic results were analyzed according to step-off, gap, reduction loss, and functional results were analyzed according to pain and ROM. We analyzed union time, operation time, incision size and complications. RESULTS: In functional results, there were 3 good cases out of 3 Mayo type IA, 8 good cases and 2 fair cases out of 10 type IIA, 1 fair case and 1 poor case out of 2 type IIB. In radiologic results, there was 1 case of reduction loss. Average union time was 9.4 weeks, average operation time was 24 minutes and average incision size was 1.8 cm. CONCLUSION: We conclude that Acutrak screw fixation can be a treatment option for olecranon fracture of Mayo type IA and IIA.
Follow-Up Studies
;
Olecranon Process
;
Ulna
5.Review of Inhalation Health Risks Involving Chloromethylisothiazolinone (CMIT) and Methylisothiazolinone (MIT) Used as Disinfectants in Household Humidifiers
Jiwon KIM ; Jiwon KIM ; Soyoung PARK ; Soyoung PARK ; Kyung Ehi ZOH ; Kyung Ehi ZOH ; Jihoon PARK ; Jihoon PARK ; Sangjun CHOI ; Sangjun CHOI ; Sung Ho HWANG ; Sung Ho HWANG ; So-Yeon LEE ; So-Yeon LEE ; Dong-Uk PARK ; Dong-Uk PARK
Journal of Korean Medical Science 2022;37(13):e101-
The association between lung injury and exposure to humidifier disinfectant (HD) containing a mixture of chloromethylisothiazolinone (CMIT) and methylisothiazolinone (MIT) has been controversial in South Korea. This study conducts a literature review in order to evaluate the likelihood of CMIT/MIT reaching the lower part of the respiratory tract and causing lung injury. A literature review focused on the inhalation risk of HD containing a mixture of CMIT and MIT. The major contents included the physicochemical properties of CMIT and MIT contained in HDs and methodological reviews on substance analysis, toxicity tests and clinical cases. HD products marketed in South Korea have been reported to contain approximately 1–2% CMIT and 0.2–0.6% MIT along with magnesium nitrate (20–25%), magnesium chloride (0.2–1.0%), and water (70–75%). The types of CMIT and MIT dispersed into the air and deposited in the respiratory tract are assumed to be either gaseous substances or nanoparticles mixed with magnesium salts. The result of the literature review including clinical cases of lung injury among CMIT/MIT HD product users, demonstrated that these chemicals likely reach the lower respiratory tract and accordingly cause lung injury.A number of humidifier disinfectant-associated lung injury cases with clinical evidence should be prioritized in risk assessment of HD containing CMIT and MIT, even though there might be insufficient evidence in all related areas, including inhalation exposure assessment studies, animal testing, and epidemiological studies.
6.Isolated Posteroinferior Cerebellar Artery Dissection Diagnosed by High-Resolution Vessel Wall MRI.
Hea Ree PARK ; Jaechun HWANG ; Ye Sel KIM ; Juhyeon KIM ; Hyunjin JO ; Young Hee JUNG ; Jihoon CHA ; Sung Tae KIM ; Gyeong Moon KIM
Journal of the Korean Neurological Association 2016;34(3):209-212
Arterial dissection is an important cause of stroke. We report two cases of isolated posterior inferior cerebellar artery (PICA) dissection diagnosed by high-resolution vessel-wall MRI (HRVW-MRI). One subject complained of abrupt-onset vertigo and headache, and the other subject had headache, vertigo, and Horner syndrome. Conventional MRA showed only focal dilatation of the PICA, but HRVW-MRI revealed intramural hematoma and double-lumen contour in the PICA, suggesting arterial dissection. We suggest that the use of HRVW-MRI should be considered when diagnosing isolated PICA dissection in a PICA infarct with an unknown cause.
Arteries*
;
Dilatation
;
Headache
;
Hematoma
;
Horner Syndrome
;
Magnetic Resonance Imaging*
;
Pica
;
Stroke
;
Vertigo
7.Early Experience With a Partial Stapled Hemorrhoidopexy for Treating Patients With Grades III–IV Prolapsing Hemorrhoids.
Hyeonseok JEONG ; Sunghwan HWANG ; Kil O RYU ; Jiyong LIM ; Hyun Tae KIM ; Hye Mi YU ; Jihoon YOON ; Ju Young LEE ; Hyoung Rae KIM ; Young Gil CHOI
Annals of Coloproctology 2017;33(1):28-34
PURPOSE: Circular stapled hemorrhoidopexy (CSH) is widely used to treat patients with grades III–IV hemorrhoids because of less pain and short hospital stay. However, this procedure is associated with some complications, such as urge to defecate, anal stenosis, staple line dehiscence, abscess and sepsis. To avoid these complications, surgeons perform a partial stapled hemorrhoidopexy (PSH). The aim of this study is to present our early experience with the PSH. METHODS: We retrospectively reviewed the medical records of 58 patients with hemorrhoids who were treated with a PSH at Busan Hang-Un Hospital from January 2016 to June 2016. A specially designed tri-window anoscope was used, and a purse string suture was made at the mucosae of the protruding hemorrhoids through the window of the anoscope. The hemorrhoidopexy was done by using a circular stapler. RESULTS: Of the 58 patients included in this study, 34 were male and 24 were female patients (mean age, 50.4 years). The mean operation time was 12.4 minutes, and the mean postoperative hospital stay was 3.8 days. Three patients experienced bleeding (5.1%) 5 urinary retention (8.6%) and 5 skin tags (8.6%). Urge to defecate, tenesmus, abscess, rectovaginal fistula, anal stricture, incontinence, and recurrence did not occur. CONCLUSION: PSH is a minimally invasive, feasible, and safe technique for treating patients with grades III–IV hemorrhoids. A PSH, instead of a CSH, can be used to treat certain patients with hemorrhoids.
Abscess
;
Busan
;
Constriction, Pathologic
;
Female
;
Hemorrhage
;
Hemorrhoids*
;
Humans
;
Length of Stay
;
Male
;
Medical Records
;
Mucous Membrane
;
Rectovaginal Fistula
;
Recurrence
;
Retrospective Studies
;
Sepsis
;
Skin
;
Surgeons
;
Sutures
;
Urinary Retention
8.A case of food-induced acute pancreatitis in a child with egg white allergy
Yeseul LEE ; JiHoon HWANG ; Su Kyung KIM ; Mi Jin KIM ; Tae Yeon JEON ; Jihyun KIM ; Kangmo AHN ; Jiwon KIM
Allergy, Asthma & Respiratory Disease 2022;10(2):123-126
Acute pancreatitis (AP) rarely occurs in association with food allergy (FA), and the mechanism is not clearly explored. We herein report a rare case of egg protein-induced AP. A 5-year-old girl was brought to the hospital because of tongue pruritus and an increased level of egg white-specific immunoglobulin E. During an oral food challenge, she developed hives, abdominal pain and vomiting after ingestion of a cumulative of 610 mg of boiled egg white (70 mg of protein). After management with intramuscular injection of epinephrine and orally administered prednisolone and levocetirizine, her skin symptoms improved. The next day, blood tests showed elevated levels of amylase and lipase, and computed tomography revealed a swollen pancreas with a low-attenuated necrotic portion. She was diagnosed with AP and treated with food restriction, intravenous methylprednisolone and gabexate mesilate. The serum levels of amylase and lipase were reduced to normal levels on day 5 and her symptoms completely resolved on day 10. A suspicion of food-induced AP is required for correct diagnosis and proper management in children with FA when they manifest severe abdominal pain.