Primary hyperparathyroicism is a state of hypersecretion of PTH by the parathyroid. The etiology has not been established. The three possible etiologies of piimary hyperparathyroidism and incidences are adencena(83%), hyperplasia(15%), and carcinoma(1~2%). Parathyroid carcinoma usually presents in the fourth decades. The hallmark preoperative signs are hypercalcemia(serum calcium 15mg/dl). Palpable neck mass and bane and renal disease. Patients may present with multiple signs and syrnptoms, including recurrent nephrolithiasis, peptic ulcers, mental change, less frequently, extensive bone resorption. However, with greater awareness of the disease and wider use of screening tests, including blood calcium determinations, the diagnosis is frequently made in patients who have no symptoms and minimal, if any, signs of the disease ather than hypercalcemia and elevated levels of parathyroid Hormone. An 38-years-old woman was admitted to the hospital due to pain on the left knee joint. We experienced full-brown symptom pertaining to hyperpara- thyroidism. Thus we report a case herein and also discuss clinical anifestation, histologic features and treatment.
Bone Resorption ; Calcium ; Diagnosis ; Female ; Humans ; Hypercalcemia ; Hyperparathyroidism ; Incidence ; Knee Joint ; Mass Screening ; Neck ; Nephrolithiasis ; Parathyroid Hormone ; Parathyroid Neoplasms ; Peptic Ulcer ; Thyroid Gland

BACKGROUND: Ebstein's anomaly is characterized by various degrees of apical displacement of the proximal attachment of the tricuspid valve. The disease has an extremely variable course in presenting itself. Therefore, we examined the morphologic and clinical features of Ebstein's anomaly as presented in the fetus to define the factors that determine its outcome. METHODS: We reviewed the history and echocardiographic studies of 10 fetuses (mean gestational age 28.0 weeks) diagnosed in utero with Ebstein's anomaly. The reason for referral was cardiomegaly on routine obstetric scanning in 5 cases, fetal arrhythmia in 3 cases, small main pulmonary artery in 3 cases, vessel view in 1, and twin pregnancy complicated by polyhydramniosis in 1. RESULTS: Of the 10 cases, pregnancy was terminated after diagnosis in 5, 2 cases were carried to term, and 1 case was at 34 weeks of gestation. We were unable to follow-up 2 cases. There were no intrauterine deaths and no misdiagnoses. Massive cardiomegaly and severe tricuspid regurgitation were detected in 7 cases; mild cases were detected in two, and one was nearly normal. Associated cardiac lesions included pulmonary artresia in 4 cases, pulmonary stenosis in 3, and pulmonary hypoplasia in 1. The cases with right ventricular outflow tract (RVOT) obstruction showed a tendency to develop more cardiomegaly and tricuspid regurgitation than with normal RVOT. There was no abnormality in other organ systems and karyotyping. CONCLUSION: Although increased cardiothoracic ratio and associated lesions of the right ventricular outflow tract contribute to the poor outcome in the prenatally detected cases, the absence of these features does not always indicate a good prognosis because progression of the disease can occur with advancing gestational age. No absolute measurement or single echocardiographic feature emerged as a consistent predictive factor for prognosis.
Arrhythmias, Cardiac ; Cardiomegaly ; Diagnosis ; Diagnostic Errors ; Ebstein Anomaly* ; Echocardiography* ; Fetus* ; Follow-Up Studies ; Gestational Age ; Karyotyping ; Pregnancy ; Pregnancy, Twin ; Prognosis ; Pulmonary Artery ; Pulmonary Valve Stenosis ; Referral and Consultation ; Tricuspid Valve ; Tricuspid Valve Insufficiency

PURPOSE: To evaluate the NT in the first trimester associated with fetal chromosomal abnormality, fetal structural abnormality and adverse pregnancy outcome. METHODS: From Mar. 1993 to Dec. 1997, 133 cases of increased NT were reviewed retrospectively. All these fetues were taken a karyotyping and/or a high resolution ultrasonography for associated anomaly. The NT was measured by transvaginal ultrasonography(TVS) and transabdominal ultrasonography(TAS) if difficulty was encountered with TVS in the period of the 10th-14th week of gestation. The increased NT is approved when it is over 3mm. RESULTS: Of these 133 cases, 102 cases(76.7%) revealed normal outcomes and 31 cases(23.3%) revealed abnormal outcomes such as chromosomal abnormality, structural abnormality and adverse pregnancy outcome, The mean value of NT was 3.44+/-0.68mm in the normal group and 4.93+/-2.70mm in the abnormal group, respectively. The chromosomal abnormalities were found in 16 of the 31 cases: nine Down SD, two Patau syndrome, one Tuner synrome, one triploidy, one 46, XX,i(18q), one 47,iso(Xp)/46,XX one confined placental mosaicism. In 12 cases of the 31 cases, the structural anomalies were detected. Of these 12 cases, 3 cases were eventually developed to cytsic hygroma, 3 cases were cardiac anomaly, 3 cases were renal anomaly, 1 case was congenital diaphragmatic hernia, 1 case was skeletal dysplasia and 1 case was suspected syndrome. The adverse pregnancy outcome such as intrauterine growth restriction, intrauterine fetal death and preterm labor was revealed in 3 cases. In the cases of NT over 7mm, the result was 100% abnormal outcome. CONCLUSION: Increased NT in the first trimester could be a highly efficient the predictor for detection of chromosomal abnormality, structural anomaly and adverse pregnancy outcome.
Chromosome Aberrations ; Female ; Fetal Death ; Hernia, Diaphragmatic ; Humans ; Karyotyping ; Lymphangioma, Cystic ; Mosaicism ; Nuchal Translucency Measurement ; Obstetric Labor, Premature ; Pregnancy ; Pregnancy Outcome* ; Pregnancy Trimester, First ; Pregnancy* ; Retrospective Studies ; Triploidy ; Ultrasonography

Microdeletion of 22q11 is responsible for DiGeorge syndrome, velocardiofacial syndrome, congenital conotruncal heart defects, and related disorders. We report our experiences on prenatal diagnosis by fluorescence in situ hybridization (FISH) for 22q11 deletion in two fetuses with tetralogy of fallot. Karyotyping and FISH of the parents revealed that one fetus inherited the disease from maternal microdeletion. These findings suggest the importance of performing FISH in pregnancies with prenatally detected tetralogy of Fallot.
Adult ; *Chromosome Deletion ; *Chromosomes, Human, Pair 22 ; Echocardiography ; Female ; Fetal Diseases/*diagnosis/genetics ; Humans ; In Situ Hybridization, Fluorescence/methods ; Pregnancy ; *Prenatal Diagnosis/methods ; Tetralogy of Fallot/*diagnosis/genetics

Scleredema is a rare connective tissue disease of unknown etiology and characterized by non-pitting thickening of subcutaneous tissue of the neck, face, head, upper trunk and arms. We present a 28 year-old man with extensive skin changes following upper respiratory infection, who have not any concurrent disorders such as diabetes mellitus. Histopathological findings of skin showed normal epidermis with a widening of spaces between collagen bundles by infiltration of acid mucopolysaccharide in the dermis.
Adult ; Arm ; Collagen ; Connective Tissue Diseases ; Dermis ; Diabetes Mellitus ; Epidermis ; Head ; Humans ; Neck ; Scleredema Adultorum* ; Skin ; Subcutaneous Tissue

Herein is described the successful treatment of complex regional pain syndrome type II with the combination treatment of spinal cord stimulation and radiofrequency thermocoagulation of the lumbar sympathetic ganglion. A 62 years old male patient, suffering from CRPS type II in his left lower extremity, visited our pain clinic. Medication and nerve blockade produced only slight improvement in his symptoms and signs. Therefore, a linear type spinal cord simulator was inserted into the thoracic epidural space, using a non-surgical percutaneous approach, with the cephalad lead located at the T11 level. Two months later, the repositioning of the electrode to the T12 level for more effective pain control, with radiofrequency thermocoagulation of lumbar sympathetic ganglion also performed at the left L2 and 3 levels for the control of trophic change. These resulted in significant pain relief and decreased trophic change, with no complications, after which the patient was able to resume a normal life.
Causalgia ; Electrocoagulation* ; Electrodes ; Epidural Space ; Ganglia, Sympathetic* ; Humans ; Lower Extremity* ; Male ; Middle Aged ; Nerve Block ; Pain Clinics ; Spinal Cord Stimulation* ; Spinal Cord*

Ginger, one of worldwide consumed dietary spice, is not only famous as food supplements, but also believed to exert a variety of remarkable pharmacological activity as herbal remedies. In this study, a ginger constituent, 12-dehydrogingerdione (DHGD) was proven that has comparable anti-inflammatory activity with positive control 6-shogaol in inhibiting LPS-induced interleukin (IL)-6, tumor necrosis factor (TNF)-α, prostaglandin (PG) E₂, nitric oxide (NO), inducible NO synthase (iNOS) and cyclooxygenase (COX)-2, without interfering with COX-1 in cultured microglial cells. Subsequent mechanistic studies indicate that 12-DHGD may inhibit neuro-inflammation through suppressing the LPS-activated Akt/IKK/NF-κB pathway. Furthermore, 12-DHGD markedly promoted the activation of NF-E2-related factor (Nrf)-2 and heme oxygenase (HO)-1, and we demonstrated that the involvement of HO-1 on the production of pro-inflammatory mediators such as NO and TNF-α by using a HO-1 inhibitor, Zinc protoporphyrin (Znpp). These results indicate that 12-DHGD may protect against neuro-inflammation by inhibiting Akt/IKK/IκB/NF-κB pathway and promoting Nrf-2/HO-1 pathway.
Dietary Supplements ; Ginger ; Heme Oxygenase (Decyclizing) ; Interleukins ; Microglia* ; Nitric Oxide ; Nitric Oxide Synthase ; Prostaglandin-Endoperoxide Synthases ; Spices ; Tumor Necrosis Factor-alpha ; Zinc

Eight distinct bacteria were isolated form diseased mycelia of the edible mushroom, Pleurotus eryngii. 16S rDNA sequence analysis showed that the isolates belonged to a variety of bacterial genera including Bacillus (LBS5), Enterobacter (LBS1), Sphingomonas (LBS8 and LBS10), Staphylococcus (LBS3, LBS4 and LBS9) and Moraxella (LBS6). Among them, 4 bacterial isolates including LBS1, LBS4, LBS5, and LBS9 evidenced growth inhibitory activity on the mushroom mycelia. The inhibitory activity on the growth of the mushroom fruiting bodies was evaluated by the treatment of the bacterial culture broth or the heat-treated cell-free supernatant of the broth. The treatment of the culture broths or the cell-free supernatants of LBS4 or LBS9 completely inhibited the formation of the fruiting body, thereby suggesting that the inhibitory agent is a heat-stable compound. In the case of LBS5, only the bacterial cell-containing culture broth was capable of inhibiting the formation of the fruiting body, whereas the cell-free supernatant did not, which suggests that an inhibitory agent generated by LBS5 is a protein or a heat-labile chemical compound, potentially a fungal cell wall-degrading enzyme. The culture broth of LBS1 was not inhibitory. However, its cell-free supernatant was capable of inhibiting the formation of fruiting bodies. This indicates that LBS1 may produce an inhibitory heat-stable chemical compound which is readily degraded by its own secreted enzyme.
Agaricales ; Bacillus ; Bacteria ; DNA, Ribosomal ; Enterobacter ; Fruit ; Moraxella ; Ostreidae ; Pleurotus ; Sequence Analysis ; Sphingomonas ; Staphylococcus

A NK/T cell lymphoma is highly aggressive lymphoma of a putative NK-cell origin with a predominant extranodal presentation and a rapidly fatal course. CD56 has been reported to be the most reliable marker for a NK/T cell lymphoma. A NK/T cell lymphoma is strongly associated with the Epstein-Barr virus. We report a case of an aggressive NK/T cell lymphoma presenting as a gastric ulcer. A 52-year-old female patient presented with hematochezia and fever. The endoscopic finding showed a gastric ulcer with a recent bleeding stigma. Histologically, the gastric biopsy showed a diffuse infiltration of small or medium sized lymphoma cells with an angiocentric growth pattern and necrosis. The lymphoma cells were CD56+, CD3+, CD20-, which are consistent with a NK/T cell lymphoma. Unfortunately, the patient was discharged without treatment. We report this case with a review of the relevant literature.
Biopsy ; Female ; Fever ; Gastrointestinal Hemorrhage ; Hemorrhage ; Herpesvirus 4, Human ; Humans ; Lymphoma* ; Middle Aged ; Necrosis ; Stomach ; Stomach Ulcer

Cap polyposis is a rare intestinal disease that is characterized by the presence of inflammatory polyps consisting of elongated, tortuous and distended crypts that are covered by a 'cap' of granulation tissue. The pathogenesis and proper treatment of cap polyposis are still unclear. We experienced a case of cap polyposis, that was treated successfully by conservative management without the need for a pharmacotherapy or resection.
Drug Therapy ; Granulation Tissue ; Intestinal Diseases ; Polyps