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Telangiectatic Focal Nodular Hyperplasia of the Liver: A Case Detected at Birth.

Han Seong KIM ; Young A KIM ; Chong Jai KIM ; Yeon Lim SUH ; Ja June JANG ; Je G CHI

Journal of Korean Medical Science.2003;18(5):746-750. doi:10.3346/jkms.2003.18.5.746

A case of telangiectatic focal nodular hyperplasia (FNH) was detected at birth and was surgically removed. Grossly, the lesion was a solitary nodule and showed vague nodularity, appearing as an adenoma-like mass with fine fibrous septa, but having no macroscopic scar. On microscopic scale, the mass typically had neither fibrous central scar nor hyperplastic nodules different from the usual FNHs. The hepatic plates were separated by sinusoidal dilatation, sometimes alternating with areas of marked ectasia. Instead of large fibrous scar, thin fibrous septa were often found, and contained abnormal tortuous large arteries. These high-pressure vessels were connected directly into the adjacent sinusoids and made marked dilation of sinusoids. Bile ductular proliferation was also noted in the thin fibrous septa. To our knowledge, this is considered to be the first reported case of telangiectatic FNH detected at birth.
Focal Nodular Hyperplasia/*congenital/*diagnosis/radiography ; Human ; Hyperplasia ; Infant ; Liver/pathology ; Liver Neoplasms/congenital/*diagnosis/radiography ; Male ; Tomography, X-Ray Computed

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A Case of Primary Pericardial Undifferentiated Sarcoma.

Nam Ho KIM ; Kyoung Hee KWEON ; Seok Kyu OH ; Moo Rim PARK ; Ki Jung YUN ; Seon Kwan JUHNG ; Jong Bum CHOI ; Jin Won JEONG

Journal of Korean Medical Science.2003;18(5):742-745. doi:10.3346/jkms.2003.18.5.742

Primary pericardial sarcomas are extremely rare. The authors report a case of a 46-yr-old woman in whom a large mediastinal mass was discovered. The patient presented with cough, dyspnea, and orthopnea. Diagnostic investigations, such as echocardiography, computed tomography, and exploratory thoracotomy provided the evidence of a large mass in the mediastinum, attached by a broad base to the superior portion of the pericardium. A excisional biopsy was performed, and histologic examination of a biopsy specimen showed undifferentiated sarcoma. However, the complete removal of the mass was impossible due to adhesion to the adjacent great vessels. After the completion of the chemotherapy the patient was completely asymptomatic. However, follow-up transesophageal echocardiography showed a residual 3x4 cm-sized mass. The patient received the radiotherapy with a total dose of 55 Gy over 6 weeks. At present, there is no evidence of disease progression.
Biopsy ; Cough ; Disease Progression ; Dyspnea ; Echocardiography ; Female ; Human ; Mediastinal Neoplasms/*diagnosis/radiography ; Middle Aged ; Prognosis ; Sarcoma/*diagnosis/radiography ; Tomography, X-Ray Computed

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A Case of Human Cyclosporiasis Causing Traveler's Diarrhea after Visiting Indonesia.

Jae Ran YU ; Woon Mok SOHN

Journal of Korean Medical Science.2003;18(5):738-741. doi:10.3346/jkms.2003.18.5.738

This is the first case of human cyclosporiasis reported in Korea. We detected the oocyst of Cyclospora cayetanensis from a 14-yr old girl who complained of persistent diarrhea after traveling to Indonesia. Round oocysts sized about 8 to 9 m with wrinkle on the wall were found in modified acid fast stained stool specimen. Stainability was variable from red to pale. Oocyst wall showed typical autofluorescence under ultraviolet illumination. The exact diagnosis for the cause of diarrhea and treatment for this patient were not provided at the right moment from the hospital since the diagnostic system for the Cyclospora infection was not ready in the clinical laboratory of the hospital. More attention should be paid on Cyclospora as a cause of diarrhea especially for those returning from a trip to the tropics and an adequate diagnostic system for the Cyclospora infection should be implemented in clinical laboratories as soon as possible.
Adolescent ; Animals ; Cyclospora/metabolism ; Cyclosporiasis/*diagnosis/*pathology ; Diarrhea/*diagnosis/pathology ; Female ; Human ; Indonesia ; Oocysts/metabolism ; Polymerase Chain Reaction ; Support, Non-U.S. Gov't ; Travel

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Initial Dose Cascade of TTS Fentanyl with Proper Adjuvant Medications in Cancer Pain.

Seong Wan BAIK ; Kyung Hoon KIM ; Yong Chul KIM ; Sang Chul LEE ; Dong Ki KIM ; Jae Eun JUNG

Journal of Korean Medical Science.2003;18(5):733-737. doi:10.3346/jkms.2003.18.5.733

According to the three step-ladder analgesics in patients with cancer pain, adjuvant drugs are required for pain relief according to the pain character and also to reduce side effects of opioids. Pain clinicians sometimes want to decide to jump directly from naive and mild opioid to transdermal therapeutic system (TTS) fentanyl with less side effects. We investigated the safety, efficacy, and satisfaction of the patients of TTS fentanyl converting from opioid-naive and mild-opioid with adjuvant drug medications in related to dose cascade of TTS fentanyl. Both opioid-naive (n=3) and opioid-using (n=34) patients started with TTS fentanyl in the lowest available delivery rate (25 microgram/hr) with rescue medication. A numeric rating scale (NRS, from 0=no pain to 10=worst pain imaginable), satisfaction of the patients with the transdermal therapy and side effects were recorded everyday during 29 days. Average reductions of NRS scores were 1.79 and 2.77, and the mean doses were 35.14 and 44.12 microgram/hr on the 15th and 29th day, respectively. Reported level of satisfaction with the transdermal patch and generalized pain management were 'completely satisfied' and 'satisfied'. Frequent side effects were nausea, vomiting, and constipation. In conclusion, initial application of TTS fentanyl with proper adjuvant medications is effective, safe, and well tolerated.
*Administration, Cutaneous ; Adult ; Aged ; Aged, 80 and over ; Analgesics, Opioid/*therapeutic use ; Dose-Response Relationship, Drug ; Female ; Fentanyl/*therapeutic use ; Human ; Logistic Models ; Male ; Middle Aged ; Neoplasms/*drug therapy ; Pain/*drug therapy ; *Pain Measurement ; Time Factors

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Rapid Detection of Duplication/Deletion of the PMP22 Gene in Patients with Charcot-Marie-Tooth Disease Type 1A and Hereditary Neuropathy with Liability to Pressure Palsy by Real-time Quantitative PCR using SYBR Green I Dye.

Sang Wun KIM ; Kwang Soo LEE ; Hyun Seok JIN ; Tae Mi LEE ; Soo Kyung KOO ; Yong Jun LEE ; Sung Chul JUNG

Journal of Korean Medical Science.2003;18(5):727-732. doi:10.3346/jkms.2003.18.5.727

Mutations and altered gene dosage of the peripheral myelin protein (PMP22) gene in chromosome 17p11.2-12 are the main causes for hereditary neuropathies, accounting for approximately 70% of all cases. Patients with duplication of the PMP22 develop Charcot-Marie-Tooth disease type 1A (CMT1A) and deletion of one PMP22 allele leads to hereditary neuropathy with liability to pressure palsy (HNPP). Twenty patients with CMT1A, 17 patients with HNPP, and 18 normal family members and 28 normal controls were studied by real-time quantitative PCR using SYBR Green I on the ABI 7700 Sequence Detection System. The copy number of the PMP22 gene was determined by the comparative threshold cycle method and the albumin was used as a reference gene. The PMP22 duplication ratio ranged from 1.45 to 2.06 and the PMP22 deletion ratio ranged from 0.42 to 0.64. The PMP22 ratio in normal controls, including normal family members, ranged from 0.85 to 1.26. No overlap was found between patients with CMT1A or patients with HNPP and normal controls. This method is fast, highly sensitive, specific, and reproducible in detecting PMP22 duplication and deletion in CMT1A and HNPP patients, respectively.
Charcot-Marie-Tooth Disease/*diagnosis/*genetics ; Chromosomes, Human, Pair 17 ; Family Health ; Female ; Fluorescent Dyes/*pharmacology ; Gene Deletion ; Gene Duplication ; Hereditary Motor and Sensory Neuropathies/*genetics ; Human ; Male ; Membrane Proteins/*biosynthesis ; Organic Chemicals/*pharmacology ; Paralysis/*genetics ; Peripheral Nervous System Diseases/*genetics ; Reverse Transcriptase Polymerase Chain Reaction

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Biochemical Characteristics of a Korean Patient with Mucolipidosis III (Pseudo-Hurler Polydystrophy).

Junghan SONG ; Dong Soon LEE ; Han Ik CHO ; Jin Q KIM ; Tae Joon CHO

Journal of Korean Medical Science.2003;18(5):722-726. doi:10.3346/jkms.2003.18.5.722

We performed a biochemical study on the patient with mucolipidosis III (ML-III, pseudo-Hurler polydystrophy) in Korea. Confluent fibroblasts from the patient and from normal controls were cultured for 4, 12, 24, 48, and 72 hr, respectively. Lysosomal enzyme activities in culture media after different incubation times and in plasma, leukocytes, and fibroblasts were determined. Most of the leukocyte lysosomal enzymes were within normal limits or slightly lowered; however, plasma lysosomal enzyme activities such as those of hexosaminidase and arylsulfatase A were markedly increased. Numerous phase-dense inclusions were present in the cytoplasm of cultured fibroblasts. Lysosomal enzyme activities of fibroblasts were markedly decreased except for beta-glucosidase. The rates of increase of the lysosomal enzyme activities with incubation time were greater in the culture medium of the patient than in normal control, whereas no difference in the beta-glucosidase activity of the culture media of the patient and the control was found. This study describes the first case of ML-III in Korea, with its typical biochemical characteristics, i.e., a problem with targeting and transporting of lysosomal enzymes which results in a marked increase in plasma lysosomal enzyme activities and a high ratio of extracellular to intracellular lysosomal enzyme activities in cultured fibroblasts.
Cerebroside-Sulfatase/blood ; Child, Preschool ; Culture Media/metabolism ; Cytoplasm/metabolism ; Female ; Fibroblasts/metabolism ; Human ; Korea ; Lysosomes/metabolism ; Microscopy, Phase-Contrast ; Mucolipidoses/*metabolism ; Support, Non-U.S. Gov't ; Time Factors ; beta-Glucosidase/metabolism ; beta-N-Acetylhexosaminidase/blood

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Distribution of Lengths of the Normal Femur and Tibia in Korean Children from Three to Sixteen Years of Age.

Jeong Hyun HA ; In Ho CHOI ; Chin Youb CHUNG ; Tae Joon CHO ; Sung Taek JUNG ; Ho Seung LEE ; Soo Sung PARK ; Han Yong LEE ; Chang Wug OH ; In One KIM

Journal of Korean Medical Science.2003;18(5):715-721. doi:10.3346/jkms.2003.18.5.715

To develop a standard growth curve of the lower extremity in Korean children from 3 to 16 yr of age, the lengths from a total of 2087 normal long bone segments (582 femurs and 645 tibias in boys, and 417 femurs and 443 tibias in girls) were measured. Children were grouped by years of bone age, which was determined by using the Korean specific bone age standard; TW2-20 method. The growth spurt occurred in girls from eight to eleven years by bone age, and in boys from eleven to thirteen years. The mean tibial length relative to the mean femoral length was 0.78 in boys and 0.79 in girls. The overall growth pattern was similar to that observed in American children in the 1960s. Korean children and adolescents appear to have a different tempo of skeletal maturation during pubertal growth from that of English and American children and adolescents. The Korean standard growth curve and the Korean bone age chart allow determination of the presence of any existent growth abnormalities and prediction of future remaining growth in lower extremities. These normative growth standards can be used for leg-length equalization purposes in children with anisomelia.
Adolescent ; Age Determination by Skeleton ; Bone Development ; Child ; Child, Preschool ; Female ; Femur/*anatomy & histology ; Growth ; Human ; Korea ; Male ; Reference Values ; Tibia/*anatomy & histology

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99mTc-HDP Pinhole SPECT Findings of Foot Reflex Sympathetic Dystrophy: Radiographic and MRI Correlation and a Speculation about Subperiosteal Bone Resorption.

Sung Hoon KIM ; Soo Kyo CHUNG ; Yong Whee BAHK ; Yong An CHUNG ; Kyung Sub SONG

Journal of Korean Medical Science.2003;18(5):707-714. doi:10.3346/jkms.2003.18.5.707

Reflex sympathetic dystrophy (RSD) is a common rheumatic disorder manifesting painful swelling, discoloration, stiffening and atrophy of the skin. Radiographic alterations include small, spotty subperiosteal bone resorption (SBR) and diffuse porosis, and MR imaging shows bone and soft-tissue edema. The purposes of current investigation were to assess 99mTc HDP pinhole SPECT (pSPECT) findings of RSD, to correlate them with those of radiography and MRI and to speculate about causative mechanism of SBR which characterizes RSD. pSPECT was performed in five patients with RSD of the foot. pSPECT showed small, discrete, spotty hot areas in the subperiosteal zones of ankle bones in all five patients. Diffusely increased tracer uptake was seen in the retrocalcaneal surface where the calcaneal tendon inserts in two patients with atrophic RSD. pSPECT and radiographic correlation showed spotty hot areas, that reflect focally activated bone turnover, to closely match with SBR. Further correlation with MRI showed both spotty hot areas and SBR to coincide in location with the insertions of ligaments and tendons, onto which pulling strain is constantly exerted. In contrast, the disuse osteoporosis in unstrained bones did not show any more significantly increased tracer uptake than normal cancellous bones.
Adult ; *Bone Resorption ; Bone and Bones/pathology ; Edema ; Female ; Foot Bones/pathology ; Foot Diseases/pathology ; Human ; Image Processing, Computer-Assisted ; Magnetic Resonance Imaging/*methods ; Male ; Middle Aged ; Osteoporosis/pathology ; Reflex Sympathetic Dystrophy/*pathology ; Technetium/*pharmacology ; Tomography, Emission-Computed, Single-Photon/*methods

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Inhibitory Effects of Potassium Channel Blockers on Carbachol-induced Contraction in Rat Detrusor Muscle.

Seung June OH ; Seung Cheol AHN

Journal of Korean Medical Science.2003;18(5):701-706. doi:10.3346/jkms.2003.18.5.701

We present accidental findings that potassium channel blockers, such as tetraethyl-ammonium (TEA) or 4-aminopyridine (4-AP), inhibit the sustained tonic contraction induced by carbachol in rat detrusor muscle strips. The relatively lower concentrations (<2 mM) of TEA and 4-AP inhibited phasic and tonic contractions induced by 5 micrometer carbachol, whilst the relatively higher concentrations of TEA and 4-AP (>5 mM) potentiated phasic contractions. The potentiation of phasic contraction was not observed in nicardipine pretreated condition. In nicardipine pretreated condition, the concentration-response curves for the negative inotropic effect of potassium channel blockers were shifted to the right by the increasing concentration of carbachol from 0.5 micrometer to 5 micrometer. IC50 was changed significantly from 0.19 to 0.64 mM (TEA) and from 0.21 to 0.96 (4-AP). Such inhibitory effects were also observed in Ca2+ depleted condition, where 0.1 mM EGTA and 1 micrometer thapsigargin were added into Ca2+ free solution. In conclusion, inhibitory effects of potasssium channel blockers on carbachol-induced contraction may be ascribed to the direct inhibition of receptor-agonist binding.
4-Aminopyridine/pharmacology ; Animals ; Bladder/metabolism ; Calcium/chemistry/metabolism ; Calcium Channel Blockers/pharmacology ; Carbachol/*pharmacology ; Dose-Response Relationship, Drug ; Female ; Guinea Pigs ; Inhibitory Concentration 50 ; Male ; Mice ; Muscle Contraction/*drug effects ; Muscles/drug effects/metabolism/*pathology ; Nicardipine/pharmacology ; Potassium Channel Blockers/*pharmacology ; Protein Binding ; Rabbits ; Rats ; Rats, Sprague-Dawley ; Support, Non-U.S. Gov't ; Tetraethylammonium/pharmacology ; Vasodilator Agents/pharmacology

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Serum Albumin Level Correlates with Disease Severity in Patients with Hemorrhagic Fever with Renal Syndrome.

Young Ok KIM ; Sun Ae YOON ; Young Mi KU ; Chul Woo YANG ; Yong Soo KIM ; Suk Young KIM ; Euy Jin CHOI ; Yoon Sik CHANG ; Byung Kee BANG

Journal of Korean Medical Science.2003;18(5):696-700. doi:10.3346/jkms.2003.18.5.696

Hypoalbuminemia frequently occurs in Hemorrhagic Fever with Renal Syndrome (HFRS), but clinical significance of hypoalbuminemia is not well known. This study was designed to evaluate hypoalbuminemia as a marker of severity of disease in patients with HFRS. We evaluated the relationship between the level of serum albumin and clinical parameters representing the severity of disease in 144 patients with HFRS. The patients were divided into three groups based on the level of serum albumin; Group I (normal serum albumin), Group II (serum albumin <3.5 g/dL and > or =3.0 g/dL), and Group III (serum albumin <3.0 g/dL). Of the total of 144 patients, 42 patients (29.2%) were categorized as Group I, 39 patients (27.1%) as Group II, and 63 patients (43.8%) as Group III. Group III had a higher rate of incidence in episode of hypotension, pulmonary edema than did Group I and Group II. The lowest level of serum albumin was positively correlated with platelet count (r=0.505, p<0.001) and was negatively correlated with leukocyte count (r=-0.329, p<0.001), BUN (r=-0.484, p<0.001), serum creatinine (r=-0.394, p<0.001), and AST (r=-0.251, p=0.002). Our data suggest that hypoalbuminemia frequently occurs in the acute stage of HFRS, and level of serum albumin is associated with the disease severity of HFRS.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Female ; Fluorescent Antibody Technique, Indirect ; Hemorrhagic Fevers, Viral/*blood/mortality ; Human ; Hypoalbuminemia/blood ; Kidney Diseases/*blood/mortality ; Male ; Middle Aged ; Retrospective Studies ; Serum Albumin/*biosynthesis ; Treatment Outcome

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