• Clinical and Experimental Pediatrics
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• 제50권6호
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• pp.524-530
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• 2007

The recent advances in the basic hematology and immunology have significantly enhanced the understanding of histiocytic disorders. The Histiocyte Society which was established in 1985 enabled the randomized trials for these diseases, and important knowledge regarding pathogenesis, clinical presentation, diagnosis, therapy and late consequences has been obtained. The treatment of Langerhans cell histiocytosis (LCH) has varied greatly over last decades, and is still controversial. Therapy can be reduced for low risk patients, and it is possible to discriminate early the non-responding patients with risk disease who might require more intensified treatment. Current therapy of LCH recommended by the Histiocyte Society (LCH-III protocol) is activated in 2001. Hemophaocytic histiocytosis (HLH) is fatal if diagnosis is delayed and appropriate therapy is not instituted rapidly. The diagnostic criteria for HLH is revised by the Histiocyte Society for the current treatment protocol (HLH-2004) which consists of dexamethasone, etoposide, and cyclosporin in combination with intathecal methotrexate. Hematopoietic stem cell transplantation is usually necessary for the primary HLH and recurrent secondary HLH.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제12권1호
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• pp.180-186
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• 1990

This is a case report of first branchial cleft cyst in 56 year old male patient, which was tentatively diagnosed as acute right submandibular abscess resulted from the periapical lesion of the lower right second molar. The results are as follows, 1. The accompanying ipsilateral inflammatory swelling resulted from the periapical lesion of lower right second molar tooth makes the diagnosis difficult. 2. The onset of this case was very late in comparison to the mean discovering age of branchial cleft cysts. 3. The plain radiography using contrast media is helpful for the diagnosis of cystic lesions within soft tissues. 4. This case in a first branchial cleft cyst(Type I) which occurs less than 1% of all branchial cleft anomalies.

• Journal of Korean Neurosurgical Society
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• 제63권3호
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• pp.314-320
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• 2020

Retained medullary cord (RMC) is a relatively recent term. Pang et al. newly defined the RMC as a late arrest of secondary neurulation leaving a non-functional vestigial portion at the tip of the conus medullaris. RMC, which belongs to the category of closed spinal dysraphism, is a cord-like structure that is elongated from the conus toward the cul-de-sac. Because intraoperative electrophysiological confirmation of a non-functional conus is essential for the diagnosis of RMC, only a tentative or an assumptive diagnosis is possible before surgery or in cases of limited surgical exposure. We suggest the term 'possible RMC' for these cases. An RMC may cause tethered cord syndrome and thus requires surgery. This article reviews the literature to elucidate the pathoembryogenesis, clinical significance and treatment of RMCs.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제19권4호
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• pp.276-280
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• 2016

Dieulafoy lesions, vascular anomalies typically found along the gastrointestinal tract, have been viewed as rare and obscure causes of sudden intestinal bleeding, especially in pediatric patients. Since their discovery in the late 19th century, the reported incidence has increased. This is due to an increased awareness of, and knowledge about, their presentation and to advanced endoscopic diagnosis and therapy. Our patient was a three-year-old male, without a complex medical history. He presented to the emergency department with acute hematemesis with blood clots and acute anemia requiring blood transfusion. Endoscopy revealed four isolated Dieulafoy lesions along the lesser curvature of the stomach, which were treated with an epinephrine injection. The Dieulafoy lesion, although thought to be rare, should be considered when investigating an acute gastrointestinal bleed. These lesions have been successfully treated endoscopically. Appropriate anticipation and preparation for diagnosis and therapy can lead to optimal outcomes for the pediatric patient.

• Journal of Yeungnam Medical Science
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• 제27권1호
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• pp.52-56
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• 2010

Idiopathic peripartum cardiomyopathy is an uncommon malady disease. Making the diagnosis is often difficult and it is always necessary to exclude other prior heart disease and other causes of left ventricular dysfunction in pregnant women. Heart failure in these women ensues when the cardiovascular demands of normal pregnancy are further amplified when the common complications of pregnancy complications superimposed upon these underlying conditions that cause compensated ventricular hypertrophy. This may be aggravated by making a late diagnosis and providing inappropriate treatment. We experienced a 38-year-primigravida who has diagnosed with idiopathic peripartum cardiomyopathy and underwent elective cesarean section with general anesthesia.

• 대한유전성대사질환학회지
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• 제13권1호
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• pp.20-29
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• 2013

Inborn error of metabolism usually presents with a constellation of clinical pictures involving multiorgan systems. Because of its rarity and clinical diversity, it is difficult to make diagnosis accurately and efficiently. Many inborn error of metabolism shows predominantly hepatic symptoms and signs. The onset of symptoms is also varying depending the disease. The onset might be even prenatal, either neonatal or infantile, and late childhood. The major manifestation patterns are jaundice or cholestasis, hepatomegaly with or without splenomegaly, hypoglycemia and acute or chronic hepatocellular dysfunction. Based on pronounced hepatic symptoms and onset of symptoms, differential diagnosis can be more easily made with subsequent further laboratory investigation. In this review paper, major inborn error of metabolism with hepatic symptoms are described from the perspective of mode of clinical presentations.

• Journal of Korean Neurosurgical Society
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• 제53권6호
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• pp.377-379
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• 2013

Perforation of the hypopharynx, which can occur after anterior cervical approach, is a very rare type of complication. If diagnosed late, it can lead to very fatal course, such as mediastinitis and hematosepsis. Therefore, a precise and prompt diagnosis is crucial. When conservative treatment alone is not expected to heal the perforated site or is likely to lead to serious complications, surgical treatment becomes necessary. This report demonstrates that surgical intervention performed immediately after an early diagnosis can lead to the successful treatment of a large perforation in the hypopharynx on a 58-year-old male patient.

• 대한치과의사협회지
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• 제52권3호
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• pp.132-138
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• 2014

The emergence of Cone Beam Computed Tomography(CBCT) in the late 1990s represented an innovative advancement in the field of dental and maxillofacial radiology because it greatly reduced the radiation exposure to patients and offered 3D images easily. The 3D information generated by this technique brings the potential of improved diagnosis and treatment planning for a wide range of clinical applications in dentistry. The use of CBCT includes diagnosis and surgical assessment of the orofacial hard tissue lesions, dental implant treatment planning and postoperative evaluation, TMJ assessment, diagnosis of craniofacial fracture, orthodontics, endodontics, and so on. All CBCT examinations should be justified on an individualized needs. The clinical benefits to the patient for each CBCT scan must outweigh the potential risks associated with exposure to ionizing radiation. CBCT scans should be taken with initially obtained medical and dental histories of patients and a close clinical examination. CBCT should be considered as an imaging alternative of other conventional radiography in cases where the anatomical structures of interest may not be seen. The smallest possible field of view(FOV) and the lowest setting of tube current and scan time should be chosen, and the entire images scanned should be interpreted by a qualified expert.

• Clinical and Experimental Pediatrics
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• 제54권1호
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• pp.22-28
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• 2011

Purpose: To investigate the clinical characteristics of late-onset epilepsy combined with autism spectrum disorder (ASD), and the relationship between certain types of electroencephalography (EEG) abnormalities in ASD and associated neuropsychological problems. Methods: Thirty patients diagnosed with ASD in early childhood and later developed clinical seizures were reviewed retrospectively. First, the clinical characteristics, language and behavioral regression, and EEG findings of these late-onset epilepsy patients with ASD were investigated. The patients were then classified into 2 groups according to the severity of the EEG abnormalities in the background rhythm and paroxysmal discharges. In the severe group, EEG showed persistent asymmetry, slow and disorganized background rhythms, and continuous sharp and slow waves during slow sleep (CSWS). Results: Between the two groups, there was no statistically significant difference in mean age (P=0.259), age of epilepsy diagnosis (P=0.237), associated family history (P=0.074), and positive abnormal magnetic resonance image (MRI) findings (P=0.084). The severe EEG group tended to have more neuropsychological problems (P=0.074). The severe group statistically showed more electrographic seizures in EEG (P=0.000). Rett syndrome was correlated with more severe EEG abnormalities (P=0.002). Although formal cognitive function tests were not performed, the parents reported an improvement in neuropsychological function on the follow up checkup according to a parent's questionnaire. Conclusion: Although some ASD patients with late-onset epilepsy showed severe EEG abnormalities, including CSWS, they generally showed an improvement in EEG and clinical symptoms in the longterm follow up. In addition, severe EEG abnormalities tended to be related to the neuropsychological function.

• Journal of Preventive Medicine and Public Health
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• 제46권5호
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• pp.249-260
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• 2013

Objectives: Previous observations propose that risk-taking behaviors such as cigarette smoking are prevailing among young people with chronic conditions including diabetes. The purpose of this study was to examine whether cigarette smoking is more prevalent among diabetics than non-diabetics and whether it differs by age at the time of diagnosis with diabetes from young adulthood (YAH) to adulthood (AH). Methods: We used US panel data from the National Longitudinal Study of Adolescent Health (Add Health Study) during the years 2001 to 2002 (Wave III, YAH) and 2007 to 2008 (Wave IV, AH). Multivariate logistic regression models were applied to estimate odds ratios (ORs) and 95% confidence intervals (CIs) of cigarette use behaviors according to age at the time of diagnosis with diabetes, after adjusting for demographic and selected behavioral factors. Results: Of 12 175 study participants, 2.6% reported having been diagnosed with diabetes up to AH. Early-onset diabetics (age at diagnosis <13 years) were more likely than non-diabetics to report frequent cigarette smoking (smoking on ${\geq}20$ days during the previous 30 days) in YAH (OR, 3.34; 95% CI, 1.27 to 8.79). On the other hand, late-onset diabetics (age at diagnosis ${\geq}13$ years) were more likely than non-diabetics to report heavy cigarette smoking (smoking ${\geq}10$ cigarettes per day during the previous 30 days) in AH (OR, 1.54; 95% CI, 1.03 to 2.30). Conclusions: The current study indicated that diabetics are more likely than non-diabetics to smoke cigarettes frequently and heavily in YAH and AH. Effective smoking prevention and cessation programs uniquely focused on diabetics need to be designed and implemented.