Acute pulmonary manifestation in ulcerative colitis / 대한내과학회지

The ulcerative colitis is a chronic inflammatory bowel disease of unknown etiology. The major symptoms of ulcerative colitis are diarrhea, hematochezia, tenesmus, and abdominal pain. However, occasionally the arthritis, skin disorders, hepatobiliary inflammation, and uveitis are recognized systemic complications or extracolonic manifestations of ulcerative colitis. Though there have been rare reports of coexistent pulmonary and inflammatory bowel disease, the lung is not generally considered a target organ in ulcerative colitis. Since the first observation of pulmonary involvement in ulcerative colitis by Kraft in 1976, a few cases have been reported. We describe of a patient with ulcerative colitis and extracolonic manifestations in whom pulmonary involvement developed that was responsive to corticosteroid therapy.

A case of capsule endoscopic finding on Churg-Strauss syndrome with gastrointestinal involvement / 대한내과학회지

Churg-Strauss syndrome is a disorder of hypereosinophilia and systemic vasculitis in subjects with asthma and allergic rhinitis. Clinically, this syndrome can be involved with various manifestations of disease of lung, heart, skin, musculoskeletal system, nerve system, gastrointestinal and hepatobiliary tract. Gastrointestinal manifestations often occur in patients. However, endoscopic finding is rare because of risk on intestinal perforation and hemorrhage in vasculitis-phase. We experienced a case of Churg-Strauss syndrome in a 27-year-old male patient with severe abdominal pain and diarrhea. He also showed leukocytosis with peripheral eosinophilia, bronchial asthma, and chronic paranasal sinusitis. Based on findings, we suggested Churg-Strauss syndrome with gastrointestinal involvement and he received a capsules endoscopy and gastroduodenal endoscopy. Capsules endoscopy showed diffuse erythema and mucosal edema on proximal jejunum to ileum, which meant the Churg-Strauss syndrome with gastrointestinal involvement. We report this case with a review of the relevant literatures.

A Case of Systemic Sclerosis Developed after Autologous Hematopoietic Stem Cell Transplantation in the Patient with Multiple Sclerosis

Herein, the case of a 43-year-old woman, with a relapsing-remitting variant of multiple sclerosis (MS), which began when she was 34 years of age, and gave rise to severe neurological complications, including progressive paralysis in both legs and visual deterioration, is reported. Despite heavy immunomodulatory treatment, her condition relapsed and became aggravated. At this point, the decision was made to perform autologous hematopoietic stem cell transplantation (HSCT). The enrichment of CD34+ cells was followed by depletion of the peripheral T cells. The post-transplantation course was uneventful, and autoimmune thrombocytopenia developed within 7 months of the HSCT. The patient was treated with cyclosporine (CsA) and oral prednisolone, but subsequently developed systemic sclerosis (SSc). The administration of CsA following the syngeneic/autologous HSCT caused a T lymphocyte-dependent autoimmune disease, which resembled graft-versus-host disease (GVHD). It is quite probable the auto-reactive lymphocytes, which were paradoxically elicited by the CsA during the reconstitution of the immune system, partly contributed to the occurrence of the other autoimmune disease, SSc. To our knowledge, this is the first description of a MS patient, having undergone CD34+-selected autologous HSCT followed by the administration of CsA, who subsequently developed SSc.

Treatment of Pulmonary Hypertension with Percutaneous Stenting of Pulmonary Artery in Fibrosing Mediastinitis

Fibrosing mediastinitis is a rare benign disorder caused by excessive proliferation of fibrous tissue within the mediastinum. Pulmonary artery stenosis is uncommon complication of fibrosing mediastinitis. We present a case of percutaneous stent deployment in a patient with severe pulmonary artery stenosis causing pulmonary hypertension secondary to fibrosing mediastinitis.

Two Cases of Spongy Myocardium Detected in Adult

Isolated noncompaction of the left ventricular myocardium is a rare cardiac disorder due to an arrest in myocardial morphogenesis. It is characterized by prominent and excessive trabeculation in a ventricular wall segment, with deep intertrabecular spaces perfused from the ventricular cavity. Echocardiographic findings are important clues for the diagnosis. Clinical symptoms include signs of left ventricular systolic dysfunction even to the point of heart failure, ventricular arrhythmias, and embolic events. We describe two cases of isolated noncompaction of the myocardium, with ventricular tachycardia in one, and chest pain due to microvascular dysfunction in the other.

Epicanthoplasty with three-dimensional Z-Plasty

The presence of epicanthal folds in Asian eyelids is one of their unique features, in combination with the lack of supratarsal folds. Western culture has influenced many people to prefer to eliminate the prominent epicanthal fold. A number of surgical techniques have been suggested for their correction. However, difficulty with design, excessive and prominent scarring of medial canthal and nasal area, recurrence, and rigidity of application are potential problems associated with many procedures. This paper describes a epicanthoplasty with three-dimensional Z-plasty. Through epicanthal fold incision, the medial canthal tecdon is medially advanced and sutured to the periosteum of nasal bone. After the transposition of the flaps, trimming of the flap is usually required. Three-dimensional Z-plasty crates the attactive eyes. From March, 1995 to March, 1997, the technique was applied to 37 patients with epicanthal fold and was performed with or without double-fold operation. There were 34 females and 3 males with ages ranging from 17 to 30 years. Through 2 years follow-up, this techniques has delivered esthetically good results with minimal postoperative scar and could made the reduction of ICD from 40 +/- 2.13mm to 34 +/- 1.98mm. The advantages of epicanthoplasty procedure using three-dimensional Z-plasty(Yoon's method) are as follows; 1) simple in design 2) minimal postoperative scar in the medial canthal area 3) versatile in its application 4) no recurrence 5) no hypertrophic scar 6) preserving ethnic identity.