• Journal of Korean Neurosurgical Society
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• v.49 no.5
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• pp.284-286
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• 2011

Glossopharyngeal neuralgia is a relatively rare condition characterized by severe, paroxysmal episodes of lancinating pain in the tongue, throat, ear, and tonsil. This disorder is assumed to be due to compression of the glossopharyngeal nerve by vascular structures. A 47-year-old woman complaining of sharp and lancinating pain in the right periauricular and submandibular areas visited our hospital. Swallowing, chewing, and lying on her right side triggered the pain. Her neurologic examination revealed no specific abnormalities. The results of routine hematologic and blood chemistry studies were all within normal limits. Carbamazepine and gabapentin were given, but her symptoms persisted. Her pain was temporarily relieved only by narcotic pain medication. MRI showed an arachnoid cyst located in the right cerebellomedullary cistern extending to the cerebellopontine cistern. Cyst removal was performed via a right retrosigmoid approach. Lateral suboccipital craniotomy was performed using the right park-bench position. After opening the dura and cerebellopontine angle, the arachnoid cyst was exposed. The arachnoid cyst was compressing the flattened lower cranial nerves at the right jugular fossa. Her symptoms resolved postoperatively. Two months after the operation, she was completely free from her previous symptoms.

• Archives of Plastic Surgery
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• v.34 no.4
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• pp.516-519
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• 2007

Purpose: Hemophilia is one of the most common bleeding disorder, and circulating levels of factor VIII or IX are closely related to the severity and frequency of the bleeding. The severity of hemophilia is classified to be severe, moderate, and mild when the factor level is less than 1%, between 1% and 5%, and between 5% and 25%, respectively. Hemophilic pseudotumor is a rare complication occurring in 1 - 2% of patients with factor VIII or IX deficiency. If the treatment is delayed, it would cause disabling and life threatening results. Methods: We experienced hemophilic pseudotumor developed at the fracture site of the proximal phalanx of the hand in a hemophilic B patient. Hemophilic pseudotumor was successfully treated with perioperative factor replacement and surgical intervention included excision and autologous bone graft. Results: Hemophilic pseudotumor was healed with complete regression, and no specific complication was developed. Conclusion: When we accounter hematoma like lesion after surgery unpredictably, we must consider hemophilic pseudotumor and make a accurate diagnosis with preoperative hematologic screening and various imaging study. Subsequently, adequate perioperative supplement of concentrate and surgical intervention brings to the satisfactory result without recurrence.

• Journal of Chest Surgery
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• v.22 no.3
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• pp.402-415
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• 1989

Extracorporeal circulation leads to functional disorder and structural damage of organs, especially hematologic and pulmonary system, mainly by sequestration of neutrophils and deposition of macrophages at lung. Then, proteases are secreted, which insult vascular basement membrane of pulmonary capillary and alveolar septa of the lung. Among these, the most important protease at lung is elastase, because major component of lung is elastin. For prevention of lung injury, inactivators or antidotes to elastase should be necessary and Alpha 1-Proteinase Inhibitor is the elastase inactivator. Clinical experimental study was carried out to investigate the immediate postoperative change of serum Alpha 1-PI level following cardiopulmonary bypass for 20 heart cases [congenital 16 cases, acquired 4 cases] and 10 control [subtotal gastrectomy] cases. Also preliminary study was performed for 31 cases of open heart patients. The results were as follows: l. Immediate postoperative serum levels of Alpha 1-PI was significantly decreased at open heart surgery group [P< 0.005], but not decreased at control group. 2. There were no significant difference in change of serum Alpha 1-PI level between and membrane and bubble oxygenator group.Z 3. There were no significant difference in changes of serum Alpha 1-PI level between CHD and AHD. Alpha 1-PI is consumed at lung during cardiopulmonary bypass and increase after operation compensatedly and protect multiple organic damage especially lung. Therefore, Alpha 1-PI can be indicator for evaluation of prevention and treatment of pump-lung syndrome.

• Journal of Sleep Medicine
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• v.15 no.2
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• pp.62-67
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• 2018

Objectives: Restless leg syndrome (RLS) is a common sensorimotor disorder and is frequently associated with periodic limb movement in sleep (PLMS). Also about one third of patient with RLS have periodic limb movement during wakefulness (PLMW). However there is little research on the correlation between PLMW and RLS. We aimed to evaluate difference in sleep characteristics between patients with RLS with PLMW and those without PLMW. Methods: Our study included twenty eight RLS patients. Subjects underwent suggested immobilization test (SIT) prior to one full-night polysomnography study. Patients were classified into two groups according to the presence of PLMW based on SIT-PLMW index. Polysomnographic findings, subjective sleep quality, and hematologic results were analyzed and compared between the two groups. Results: Mean age of patient with frequent PLMW (SIT-PLMW ${\geq}40/hr$) was significantly higher. RLS patients with frequent PLMW were also significantly related to insomnia severity. The PLMS index was higher in patients with PLMW and showed a significant correlation with the PLMW index. Conclusions: PLMW influence sleep quality such as insomnia and is correlated with movement during sleep.

• Clinical and Experimental Pediatrics
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• v.63 no.3
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• pp.79-87
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• 2020

Inherited platelet disorders (IPDs), which manifest as primary hemostasis defects, often underlie abnormal bleeding and a family history of thrombocytopenia, bone marrow failure, hematologic malignancies, undefined mucocutaneous bleeding disorder, or congenital bony defects. Wide heterogeneity in IPD types with regard to the presence or absence of thrombocytopenia, platelet dysfunction, bone marrow failure, and dysmegakaryopoiesis is observed in patients. The individual processes involved in platelet production and hemostasis are genetically controlled; to date, mutations of more than 50 genes involved in various platelet biogenesis steps have been implicated in IPDs. Representative IPDs resulting from defects in specific pathways, such as thrombopoietin/MPL signaling; transcriptional regulation; granule formation, trafficking, and secretion; proplatelet formation; cytoskeleton regulation; and transmembrane glycoprotein signaling are reviewed, and the underlying gene mutations are discussed based on the National Center for Biotechnology Information database and Online Mendelian Inheritance in Man accession number. Further, the status and prevalence of genetically confirmed IPDs in Korea are explored based on searches of the PubMed and KoreaMed databases. IPDs are congenital bleeding disorders that can be dangerous due to unexpected bleeding and require genetic counseling for family members and descendants. Therefore, the pediatrician should be suspicious and aware of IPDs and perform the appropriate tests if the patient has unexpected bleeding. However, all IPDs are extremely rare; thus, the domestic incidences of IPDs are unclear and their diagnosis is difficult. Diagnostic confirmation or differential diagnoses of IPDs are challenging, time-consuming, and expensive, and patients are frequently misdiagnosed. Comprehensive molecular characterization and classification of these disorders should enable accurate and precise diagnosis and facilitate improved patient management.

• Korean Journal of Clinical Pharmacy
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• v.25 no.3
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• pp.166-170
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• 2015

Background: Primary warm autoimmune hemolytic anemia (AIHA) is a relatively rare hematologic disorder resulting from autoantibody production against red blood cells. There has been very few studies about primary warm AIHA in South Korea because of its low incidence. We retrospectively analyzed the treatment outcome of primary warm AIHA. Method: We reviewed retrospectively the medical records of 9 primary warm AIHA patients from December 2002 to January 2015. We analyzed the causes and clinical characteristics of primary warm AIHA patients. We retrospectively analyzed the clinical data in electronic medical records for 9 Korean patients with AIHA patients who were diagnosed during the period from December 2002 to January 2015 at the Regional University Hospital in Korea. The study protocol was approved by the Institutional Review Board (IRB #2015-08-007, Chosun University Hospital IRB). Results: The mean age was 52 years (range 27~78), the mean hemoglobin level was 5.0 g/dL (range 2.5~6.4 g/dL). All patients received steroids at therapeutic dosages (corticosteroid 1 mg/Kg) as first line treatment. Eight of them showed complete response (5/8, 62.5%) and partial response (3/8, 37.5%), one patient required second-line treatment with rituximab. Two patients who responded first line treatment were relapsed at 86 weeks and 24 weeks after response, respectively. Only one patient of them was retreated with corticosteroid because of anemic symptoms. Conclusion: This study indicates that oral corticosteroid is an effective therapy for primary warm AIHA.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.14 no.1
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• pp.86-90
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• 2011

Wilson's disease is an autosomal recessive disorder of copper metabolism consequence of which leads to accumulation of copper in the liver, brain, cornea and other tissues. The manifestations are more likely to be hepatic in the early childhood and neurological in the adolescents. In addition, the abnormalities that develop during disease progression may result in other manifestations such as hematologic, endocrine, or renal findings. We report a thirteen year-old girl who manifested tetany shortly after the initial diagnosis of Wilson's disease. Despite aggressive calcium, magnesium and vitamin D replacement, the hypocalcemia and hypomagnesemia did not respond to the therapy promptly. It took more than three weeks for blood levels of the minerals to be normal. We concluded that tetany occurred in our patient because of hypoparathyroidism as a rare complication of Wilson disease, vitamin D deficiency resulting from various conditions, and inconclusive hypomagnesemia.

• Childhood Kidney Diseases
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• v.12 no.2
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• pp.221-226
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• 2008

Paroxysmal nocturnal hemoglobinuria(PNH) is an acquired hematologic disorder characterized by intravascular hemolysis, nocturnal hemoglobinuria, thrombotic events and bone marrow failure. It rarely occurs in children and can be complicated by acute renal failure(ARF). Here, we present two cases of ARF complicating PNH which has not been reported yet in Korean children. We suggest that PNH should be considered in differential diagnosis of ARF in children associated with intravascular hemolysis.

• Tuberculosis and Respiratory Diseases
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• v.44 no.6
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• pp.1419-1425
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• 1997

Primary mediastinal nonseminomatous germ cell tumor associated with Klinefelter's syndrome is a rare disorder. We experienced a case of recurred primary mediastinal nonseminomatous germ cell tumor developed in a 24-year-old patient with Klinefelter's syndrome. The patient had been treated with surgery and combination chemotherapy under the diagnosis of primary mediastinal nonseminomatous germ cell tumor before. A round mass was found on the right lower lung field in the chest X-ray during follow up. The patient was diagnosed as recurred primary nonseminomatous genu cell tumor and Klinefelter's syndrome through tumor markers, peripheral blood karyotyping, and other tests including hormonal assay and was treated with combination chemotherapy and surgery again. When the patient is diagnosed as primary mediastinal nonseminomatous germ cell tumor, Klinefelter's syndrome and hematologic malignancies should be considered to be associated diseases and vice versa.

• Tuberculosis and Respiratory Diseases
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• v.47 no.5
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• pp.609-617
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• 1999

Background: The aims of this study were to assess the etiologies, survival and prognostic factors of patients with chronic cor pulmonale visited Pusan National University Hospital. Methods : This study included 103 patients with chronic cor pulmonale. There were 67 men and 36 women. The diagnosis of chronic cor pulmonale was primarily based on the presence of underlying lung disorder and echocardiographic finding of enlarged or hypertrophied right ventricle. Other clinical data including patients' symptoms and signs, findings of arterial blood gas analysis, hematologic and biochemical laboratory and pulmonary function test were assessed. Results: The most common underlying lung disorder was pulmonary tuberculosis(59.2%) and chronic obstructive pulmonary disease was the next(28.2%). The survival rate was 57% in one year, 45% in two years, and 34% in three years. The prognostic factors were maximal voluntary ventilation(MVV), forced vital capaoity(FVC), $FEV_1$ serum Na, vital capacity(VC), serum albumin and peak expiratory flow(PEF) in univariate analysis. And in multivariate analysis, serum albumin(p=0.0144) and VC(p=0.0078) were statistically significant. Conclusion: Pulmonary tuberculosis was the most important underlying lung disorder in chronic cor pulmonale. The survival rate was 57% in one year, 45% in two years, and 34% in three years. Serum albumin (p=0.0144) and VC(p=0.0078) were statistically significant prognostic factors.