OBJECTIVE: To evaluate the abnormality of protein S in patients with recurrent spontaneous abortion due to antiphospholipid syndrome. MATERIALS AND METHOD: Antigen and activity of protein S were analyzed by enzyme immunoassay and clotting method, respectively. RESULTS: Of 18 patients with antiphospholipid syndrome, 4 patients were found to have no abnormality of protein S. There were 14 cases of protein S abnormality. Among them, there were 8 cases of type 1, 1 case of type 2, and 5 cases of type 3 protein S deficiency. CONCLUSION: So in the workup of patients with recurrent spontaneous abortion due to antiphospholipid syndrome, the evaluation for protein S is required.
Abortion, Spontaneous ; Antiphospholipid Syndrome* ; Female ; Humans ; Immunoenzyme Techniques ; Pregnancy ; Protein S Deficiency* ; Protein S*

Introduction: Gitelman Syndrome (GS), a rare autosomal recessive inherited disorder, is frequently unrecognized in the clinical setting. GS typically manifests with severe hypokalemia with debilitating and potentially fatal consequences if untreated. As of writing, confirmatory genetic assays are currently unavailable in the country, and the diagnosis of GS is primarily based on several biochemical laboratory tests. This results in the difficulty with prompt diagnosis of GS in the locality. Case: We present a 52-year-old male who came in with chronic, intermittent paraparesis associated with persistent hypokalemia. A diagnosis of GS was made biochemically based on renal wasting of potassium and magnesium, hypocalciuria, and metabolic alkalosis. Electrolyte correction with lifelong supplementation, and administration of Spironolactone resulted in the resolution of bilateral leg weakness. Electrolyte levels were maintained within normal limits in the outpatient setting. Conclusion GS is an uncommon potentially debilitating disorder that may lead to problematic, potentially fatal consequences to electrolyte abnormalities if left untreated. The lack of awareness and consequent delay in the diagnosis, and the unavailability of confirmatory genetic testing remains a clinical challenge. Timely recognition and initiation of treatment leads to early control of electrolyte levels, and better prognosis.
Gitelman&rsquo ; s Syndrome ; Paraparesis ; Hypokalemia ; Hypomagnesemia ; Spironolactone ; Case Report

Protein S is found in two forms in plasma; as free and functionally active protein S, and complexed to C4b-binding protein. Patients with nephrotic syndrome are at risk for arterial and venous thrombosis at various localizations, and acquired protein S deficiency due to the selective urinary loss of the free form may be a risk factor for the development of thromboembolic complications. We report a case of cerebral arterial thrombosis associated with decreased level of free protein S antigen (44%) in a 39-year-old female patient with nephrotic syndrome.
Adult ; Case Report ; Female ; Human ; Intracranial Embolism and Thrombosis/*etiology ; Nephrotic Syndrome/*complications ; Protein S Deficiency/*complications

Sheehan's syndrome is a rare complication of post-partum hemorrhage that has decreased in incidence in the past decade due to better obstetrical practices, although still seen in developing countries. This is a case of a 31-year-old Gravida 1 Para 1 (1-0-0-0) with a 1-year history of enlarging maxillary sinus mass, where an incidental finding of an empty sella in an MRI with contrast was noted. The patient had amenorrhea of 15 years and received no medications for her undiagnosed Sheehan's syndrome incurred during her first and only pregnancy. The patient's cardiomyopathy and organizing hematoma may be rare complications of Sheehan's syndrome. Patients, laymen, health practitioners, and traditional birth attendants should be informed of these complications. Treatment should be individualized and administered after diagnosing a patient with Sheehan's syndrome to prevent complications such as adrenal insufficiency, hypothyroidism, infertility, and seen in this case, acute heart failure and possibly organizing hematoma.

Mesenteric venous thrombosis (MVT) is uncommon, but it is often aggravated to a lethal form of intestinal ischemia. Multiple risk factors are associated with mesenteric venous thrombosis, including hypercoagulable or inflammatory states, previous abdominal surgery, abdominal trauma, portal hypertension and oral contraceptives. The use of estrogen-containing oral contraceptives accounts for 4% to 5% of all the cases of MVT. Oral contraceptives have been reported to decrease antithrombin III, increase the levels of prothrombin complex factor and factor VIII, and to inhibit the fibrinolytic system. We describe here a case of 42-year-old woman who had taken oral contraceptives for 7 years and she was diagnosed to have Protein C and Protein S deficiency, and superior mesenteric venous (SMV) and portal vein thrombosis. She underwent segmental resection of the small bowel. She was later discharged without suffering with short bowel syndrome or any other complications.
Adult ; Antithrombin III ; Contraceptives, Oral ; Factor VIII ; Female ; Humans ; Hypertension, Portal ; Ischemia ; Protein C* ; Protein S Deficiency* ; Protein S* ; Prothrombin ; Risk Factors ; Short Bowel Syndrome ; Thrombosis* ; Venous Thrombosis

In this case, a 39 year-old man was admitted with Budd-Chiari syndrome associated with complete superior vena cava(SVC)obstruction causing general edema and hepatic failure. Conservative medical therapy was failed. And after the radiologist failed to invasive procedure of balloon dilatation, we attempted the inferior vena cava to right atrium bypass graft. Operation was done through median sternotomy and extended vertical oblique abdominal incision. A 24 mm Dacron tube was placed from the inferior vena cava just below the left renal vein to the right atrium without using the cardiopulmonary bypass pump. The patient's postoperative course was uneventful without signs of bleeding or anyother complications. We used anticoagulants at the postoperative first day. At the postoperative 26th day, we performed abdominal Doppler sonography and we confirmed that the graft patency was good. The patient was discharged with SVC obstructive symptoms but we noticed relief of SVC obstructive symptoms in the course of follow-up.
Adult ; Anticoagulants ; Budd-Chiari Syndrome* ; Cardiopulmonary Bypass ; Dilatation ; Edema ; Follow-Up Studies ; Heart Atria ; Hemorrhage ; Humans ; Liver Failure ; Polyethylene Terephthalates ; Protein S Deficiency* ; Protein S* ; Renal Veins ; Sternotomy ; Superior Vena Cava Syndrome ; Transplants ; Vena Cava, Inferior ; Vena Cava, Superior

OBJECTIVE: To investigate the clinical significance of thrombophilia in patients admitted with ovarian hyperstimulation syndrome (OHSS). METHODS: Twenty-five infertile women who were admitted into university hospital due to OHSS after ovarian hyperstimulation for intrauterine insemination or in vitro fertilization. Blood samples were drawn at the time of admission and three thrombophilic factors were assayed; antithrombin III, protein C and protein S. Subjects were divided into severe (n=18) and mild-to-moderate (n=7) OHSS, and laboratory parameters including three thrombophilic factors were compared. RESULTS: Antithrombin III level was abnormal in 40% of subjects, protein C in 12%, and protein S in 72%. There was no significant difference in the laboratory parameters between the patients with normal (n=15) and abnormal antithrombin III levels (n=10). However, the patients with abnormal antithrombin III levels had significantly more severe OHSS than those with normal value (100% vs 55.6%, P=0.013). The patients with at least one abnormal thrombophilic factor had significantly more severe OHSS than those with all normal value (94.4% vs 42.9%, P=0.012). CONCLUSIONS: Thrombophilic factors, particularly antithrombin III, may be associated with disease severity in patients with OHSS.
Antithrombin III ; Female ; Fertilization in Vitro ; Humans ; Insemination ; Ovarian Hyperstimulation Syndrome ; Protein C ; Protein S ; Reference Values ; Thrombophilia

Budd-Chiari syndrome (BCS) represents a spectrum of disease states resulting in hepatic venous outflow occlusion. Prothrombotic disorders, such as protein S deficiency may cause thrombosis of the portal and hepatic veins. We report the management of a 30-year-old BCS primigravida with protein S deficiency and destroyed lung by the pulmonary tuberculosis scheduled for Cesarean section. Moreover, patient's lungs were destroyed by the pulmonary tuberculosis. Spinal anesthesia was selected for the anesthetic management. The patient recovered without any complication and discharged from hospital on the fifth postoperative day.
Adult ; Anesthesia, Spinal ; Budd-Chiari Syndrome ; Cesarean Section ; Female ; Hepatic Veins ; Humans ; Lung ; Pregnancy ; Protein S Deficiency ; Thrombosis ; Tuberculosis, Pulmonary

Hypercoagulable states (HS) are well recognized as a cause of arterial and venous thromboembolism. These coditions are usually associated with abnormalities in natural anticoagulants, the fibrolytic system, or platelet aggregation. Primary arterial thrombosis is unusual in the young population. Hypercoagulable states are usually acquired, but they may be congenital. The congenital states occur in response to deficiency of protein C, protein S, AT-III. And the acquired states occur in response to disease, in response to tissue injury, or in response to therapy. But in this country, we have no any data of prevalence ratio of HS, which developed symptome of arterial occlusion, cases of bypass and theapeutic modality. We experienced two cases of acquired HS that is antiphospholipid syndrome and polycythemia. Based on our experience with this patients and a review of the literature on the previously reported. And we suggest that there will be made for the purpose of studying non-atherosclerotic lower extremity occlusion registry which developed in our country through the small study group.
Anticoagulants ; Antiphospholipid Syndrome ; Humans ; Lower Extremity ; Platelet Aggregation ; Polycythemia* ; Prevalence ; Protein C ; Protein S ; Thrombosis ; Venous Thromboembolism

Pulmonary nodular lymphoid hyperplasia (NLH) is a term first suggested by Kradin and Mark to describe one or more pulmonary nodules or localized lung infiltrates consisting of reactive lymphoid proliferation. To date, there have been only a few cases of pulmonary NLH reported associated with autoimmune disorders. There is no case of NLH associated with Sj?gren's syndrome from Korea in the medical literature. A 56-year-old woman was referred to our hospital with cough productive of sputum and chest tightness. The Computed tomography scans of the chest revealed multiple and well-defined peribronchiolar nodular opacities. A video assisted thoracoscopic surgery (VATS) biopsy was performed and the nodular opacity in the lung parenchyma was pathologically confirmed as NLH. Through meticulous review of patient's record, we found that she had been suffering from dry eye and dry mouth. The symptoms suggested Sj?gren's syndrome, which was confirmed by specific laboratory tests including the Schirmer test, anti-nuclear antibody and anti-Ro/La antibody. The patient is followed regularly and has no further progression of symptoms.
Biopsy ; Female ; Humans ; Immunohistochemistry ; Middle Aged ; Pseudolymphoma/complications/*pathology/*radiography ; Sjogren' ; s Syndrome/*complications ; Thoracic Surgery, Video-Assisted ; Tomography, X-Ray Computed