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Sequential magnetic resonance spectroscopic changes in a patient with nonketotic hyperglycinemia

  • Shin, Ji-Hun;Ahn, So-Yoon;Shin, Jeong-Hee;Sung, Se-In;Jung, Ji-Mi;Kim, Jin-Kyu;Kim, Eun-Sun;Park, Hyung-Doo;Kim, Ji-Hye;Chang, Yun-Sil;Park, Won-Soon
    • Clinical and Experimental Pediatrics
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    • v.55 no.8
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    • pp.301-305
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    • 2012
  • Nonketotic hyperglycinemia (NKH) is a rare inborn error of amino acid metabolism. A defect in the glycine cleavage enzyme system results in highly elevated concentrations of glycine in the plasma, urine, cerebrospinal fluid, and brain, resulting in glycine-induced encephalopathy and neuropathy. The prevalence of NKH in Korea is very low, and no reports of surviving patients are available, given the scarcity and poor prognosis of this disease. In the current study, we present a patient with NKH diagnosed on the basis of clinical features, biochemical profiles, and genetic analysis. Magnetic resonance spectroscopy (MRS) allowed the measurement of absolute glycine concentrations in different parts of the brain that showed a significantly increased glycine peak, consolidating the diagnosis of NKH. In additional, serial MRS follow-up showed changes in the glycine/creatinine ratios in different parts of the brain. In conclusion, MRS is an effective, noninvasive diagnostic tool for NKH that can be used to distinguish this disease from other glycine metabolism disorders. It may also be useful for monitoring NKH treatment.

Endobronchial ALK-Positive Anaplastic Large Cell Lymphoma Presenting Massive Hemoptysis

  • Kim, Hee Kyung;Kim, Bo Hye;Kim, Sae Ahm;Shin, Jae Kyoung;Song, Ji-Hyun;Kwon, Ah-Young;Kim, Jung-Hyun;Kim, Eun-Kyung;Lee, Ji-Hyun;Kim, Gwaung-Il;Jeong, Hye Cheol
    • Tuberculosis and Respiratory Diseases
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    • v.78 no.4
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    • pp.390-395
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    • 2015
  • Primary anaplastic large cell lymphoma (ALCL) of the lung is highly aggressive and quite rare. We report here a case of anaplastic lymphoma kinase-positive endobronchial ALCL, that was initially thought to be primary lung cancer. A 68-year-old woman presented with hemoptysis, dyspnea, and upper respiratory symptoms persisting since 1 month. The hemoptysis and and bronchial obstruction lead to respiratory failure, prompting emergency radiotherapy and steroid treatment based on the probable diagnosis of lung cancer, although a biopsy did not confirm malignancy. Following treatment, her symptoms resolved completely. Chest computed tomography scan performed 8 months later showed increased and enlarged intra-abdominal lymph nodes, suggesting lymphoma. At that time, a lymph node biopsy was recommended, but the patient refused and was lost to follow up. Sixteen months later, the patient revisited the emergency department, complaining of persistent abdominal pain since several months. A laparoscopic intra-abdominal lymph node biopsy confirmed a diagnosis of ALCL.

Effects of a low-FODMAP enteral formula on diarrhea on patients in the intensive care unit

  • Bae, Eunjoo;Kim, Jiyoon;Jang, Jinyoung;Kim, Junghyun;Kim, Suyeon;Chang, Youngeun;KIM, MI YEON;Jeon, Mira;Kang, Seongsuk;Lee, Jung Keun;Kim, Tae Gon
    • Nutrition Research and Practice
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    • v.15 no.6
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    • pp.703-714
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    • 2021
  • BACKGROUND/OBJECTIVES: A dietary restriction on the intake of fermentable oligosaccharides, disaccharides, monosaccharides, and polyols (FODMAPs) has been reported to be effective in the treatment of gastrointestinal (GI) tract complications. Enteral nutrition (EN) is widely used for patients who cannot obtain their nutritional requirements orally, but many studies have reported EN complications, especially diarrhea, in up to 50% of patients. SUBJECTS/METHODS: We performed a single-center, non-randomized, controlled trial to determine the effects of a low-FODMAP enteral formula on GI complications in patients in intensive care units (ICUs). Patients in the ICU who needed EN (n = 66) were alternately assigned to the low-FODMAP group (n = 33) or the high-FODMAP group (n = 33). RESULTS: Anthropometric and biochemical parameters were measured, and stool assessment was performed using King's Stool Chart. We excluded patients who received laxatives, GI motility agents, proton pump inhibitors, antifungal agents, and antibiotics other than β-lactams. There were no differences in GI symptoms during 7 days of intervention, including bowel sound, abdominal distension, and vomiting between the 2 groups. However, diarrhea was more frequent in the high-FODMAP group (7/33 patients) than the low-FODMAP group (1/33 patients) (P = 0.044). CONCLUSIONS: Our results suggest that a low-FODMAP enteral formula may be a practical therapeutic approach for patients who exhibit enteral formula complications. Our study warrants further randomized clinical trials and multicenter trials.

Current Status of the Diagnosis and Management of Pancreatic Neuroendocrine Tumors in Japan

  • Tetsuhide Ito;Masami Miki;Keijiro Ueda;Lingaku Lee;Ken Kawabe;Hisato Igarashi;Nao Fujimori;Kazuhiko Nakamura;Kohei Yasunaga;Robert T. Jensen;Takao Ohtsuka;Yoshihiro Ogawa
    • Journal of Digestive Cancer Research
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    • v.4 no.2
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    • pp.51-57
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    • 2016
  • The epidemiology of pancreatic neuroendocrine neoplasms (PNENs) in Asia has been clarified through epidemiological studies, including one conducted in Japan, and subsequently another in South Korea. As endoscopic ultrasonography (EUS) has become more widely accessible, endoscopic ultrasound-fine needle aspiration (EUS-FNA) has been performed in pancreatic tumors for which the clinical course was only monitored previously. This has enabled accurate diagnosis of pancreatic tumors based on the 2010 WHO classification; as a result, the number of patients with an accurate diagnosis has increased. Although surgery has been the standard therapy for PNENs, new treatment options have become available in Japan for the treatment of advanced or inoperable PNENs; of particular note is the recent introduction of molecular target drugs (such as everolimus and sunitinib) and streptozocin. Treatment for progressive PNENs needs to be selected for each patient with consideration of the performance status, degree of tumor differentiation, tumor mass, and proliferation rate. Somatostatin receptor (SSTR)-2 is expressed in many patients with neuroendocrine tumor. Somatostatin receptor scintigraphy (SRS), which can visualize SSTR-2 expression, has been approved in Japan. The SRS will be a useful diagnostic tool for locating neuroendocrine neoplasms, detecting distant metastasis, and evaluating therapy outcomes. In this manuscript, we review the latest diagnostic methods and treatments for PNENs.

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Hemothorax Due to Diaphragm Laceration Induced Osteochondroma of Rib - A case report- (늑골연골증이 횡격막 열상을 일으킨 혈흉 -치험 1예 -)

  • Kim Yong In;Lim Yong Su;Kim Jae Kwang;Jin Wook;Lee Chi Hoon;Lee Suk Ki;Hyun Sung Youl
    • Journal of Chest Surgery
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    • v.38 no.1 s.246
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    • pp.84-87
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    • 2005
  • Spontaneous hemothorax vary in cause and are rare for hemothorax induced osteochondroma. Sometimes hemothroax is reported due to osteochondroma induced injury of diaphragm, lung, pericardium, heart, or pleura. We report a patient with diaphragm laceration due to osteochondroma.

Ghrelin is Present in Teeth

  • Aydin, Suleyman;Ozercan, I brahim Hanefi;Geckil, Hikmet;Dagli, Ferda;Aydin, Suna;Kumru, Sinem;Kilic, Nermin;Sahin, I brahim;Ozercan, Mehmet Resat
    • BMB Reports
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    • v.40 no.3
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    • pp.368-372
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    • 2007
  • Ghrelin belongs to the family of a gut-brain hormone that promotes food intake and controls energy balance. Recently, it has also been shown to regulate bone formation directly. Dental tissue shares several functional, developmental and anatomical similarities with bone, and in the present study we have investigated the presence of ghrelin in 44 human teeth using immunocytochemistry and radioimmunoassay. Both methods showed that the hormone is present in canines and molars, mainly in the odontoblasts but also in the pulp. Ghrelin could potentially play interesting physiological roles in teeth.

CLINICAL STUDIES OF LAG -SCREW OSTEOSYNTHESIS IN CONDYLAR FRACTURES OF THE MANDIBLE (하악 과두 골절 환자에서 Lag-screw를 이용한 관혈적 정복 후 임상적 연구)

  • Jeong, Jong-Cheol;Song, Min-Seok;Choi, Jae-Uk;Kim, Seong-Beom;Seo, Ji-Hun;Lee, Gye-Hyeok
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.27 no.5
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    • pp.442-446
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    • 2001
  • Purpose : This study evaluated clinical and radiographic results after open reduction and lag-screw fixation of mandibular condyle fractures. Patients and Methods : 26 patients had been treated by lag-screw fixation for madibular condyle fracures via submandibular approach and follow up for over 6 months. The lag-screw used in this study was designed by Eckelt. Results : Radiographically, resorption of the condyle head found in 4 cases (15%) and minimal displacement of the condyle head was found in 3 cases (12%). Post-operative infection were not found in all cases. Clinically, All patiens had a stable occlusion and normal mouth opening (over 40mm). TMD problems with crepitus or some discomfort encountered in 4 patients but tolerable to the patients. Conclusion : Lag screw fixation can be a good option especially high level condylar fractures, however this procedure must be used in cautiously because of slipping of the fragments and possible to resorption of the fragments.

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