We report a case of acute intermittent porphyria presenting with variable symptoms and signs such as hypertension, polyneuropathy, syndrome of inappropriate secretion of antidiuretic hormone and cerebral infarction. A 47 year-old female patient entered hospital with abdominal pain followed by generalized seizure. She was diagnosed to have acute intermittent porphyria in consequence of Watson-Schwartz test and δ-ALA in 24 hours urine. She was managed with conservative treatment but died of respiratory failure.
Abdominal Pain ; Cerebral Infarction ; Female ; Humans ; Hypertension ; Polyneuropathies ; Porphyria, Acute Intermittent* ; Respiratory Insufficiency ; Seizures

BACKGROUND: Polyarteritis nodosa(PAN) is a disease of necrotzing vasculitis which has a clinical spectrum encompassing those cases of multisystem involveme it and skin-limited variant. The clinical course of cutaneous PAN has been considered to be a benign one, however there is sorne controversy regarding its nosological entity. OBJECTIVE: To characten the clinical course of the patients with cutaneous PAN and determine whether or not it is a benign disease or something more sever. METHODS: Clinical and laboratory findings in 8 patients with Logy-proven cutaneous PAN but without any detectable visceral involvement were observed periochelly during a follow-up period over 18 months. Meticulous physical and pertinent laboratory earinations were performed each time during their visits. RESULTS: Livedo reticulari, on the lower legs was the mostorenon cutaneous findings with these patients. During the follow-up periods there were no suspected abnormal physical and laboratory findings to have other organ involvement in all 8 patients Serologic examinations for antineutrophil cytoplasmic antibody and hepatitis-B surface antigen revealed negative in all patients tested. CONCLUSION: The follow-up periods of 2 years or so does not seem to be long enough in characterizing the clinical features of PAN. However, the cutaneous: on of PAN can be regarded as a benign cutaneous variant of the disease having no visceral lesion of arteritis, even though mild degrees of constitutional symptoms and a few laboratory abnormlites could be seen.
Antibodies, Antineutrophil Cytoplasmic ; Antigens, Surface ; Arteritis ; Follow-Up Studies ; Humans ; Leg ; Polyarteritis Nodosa* ; Vasculitis

A retrospective study was made of 10 consecutive patients who underwent mandibular reconstruction with PCBM from December 1994 to July 1996. Free autogenous iliac bone in the from of particulate cancellous bone and marrow was densely packed into the crib that was adapted to bridge the mandibular discontinuity defect. Frozen-treated autogenous mandibular bone, splitted autogenous rib, and titanium mesh(Dumbach, Leibinger) were used as cribs carrying the PCBM. All ten cases underwent successful healing with the formation of a continuous bony union with the remaining mandible. The rate of resorption was assessed by sequential panoramic radiographs. The mean horizontal dimension of the madibular defects was 44mm and the mean vertical dimension of the reconstructed segments was 23mm. The bony height of the reconstructed segments retained about 90% of the bony height of over a 1-year period. We confirmed that PCBM grafts were the most successful and predictable grafts in mandibular discontinuity reconstruction.
Bone Marrow* ; Humans ; Infant Equipment ; Mandible ; Mandibular Reconstruction* ; Retrospective Studies ; Ribs ; Titanium ; Transplants ; Vertical Dimension

A 40-year-old woman had a pruritic, vesiculobullous eruption of her face for 6 weeks with no evidence of systemic disease. A biopsy showed subepidermal blisters and dermal infiltrates of neutrophilic inflammatory cells. Direct immunofluorescence demonstrated thick linear deposits of IgG and C3 along the basement membrane zone. The cleavage plane was identified to be just beneath the lamina densa. Using Western immunoblots, the patient's IgG autoantibodies were found to recognize type VII proeollagen. Moderate starting doses of systemic prednisolone gave a good response for this patient with an early inflammatory form of epidermolysis bullosa acquisita.
Adult ; Autoantibodies ; Basement Membrane ; Biopsy ; Blister ; Blotting, Western ; Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Female ; Fluorescent Antibody Technique, Direct ; Humans ; Immunoglobulin G ; Neutrophils ; Prednisolone

BACKGROUND: To evaluate the effect of preoperative fatty degeneration of deltoid and teres minor muscles on the clinical outcome in patient with reverse total shoulder arthroplasty (RTSA). METHODS: Nineteen patients with RTSA were enrolled. The mean follow-up period was 16.1 months. The fatty degeneration of three distinct parts in each deltoid and the teres minor muscle was measured using a preoperative magnetic resonance imaging. Postoperatively, the muscle strengths for forward elevation (FE), abduction (Abd), and external rotation (ER) were measured using a myometer at the last follow-up. The parameters for clinical outcome were Constant Score (CS) and Korean Shoulder Score (KSS). RESULTS: The number of cases was 10 in group 1 and 9 in group 2. The strength of FE and Abd were significantly higher in group 1 (p<0.001 and p<0.001, respectively), and the strength of ER was not different significantly between two groups (p=0.065). For the clinical outcome, both CS and KSS were higher in group 1 (p=0.002 and p=0.002, respectively). The number of patients in group A was 11, and group B was 8. Although there was not a significant difference in terms of FE and Abd between group A and B (p=0.091, p=0.238), ER was significantly higher in group A (p=0.012). We did not find a significant difference in the clinical scores (CS, p=0.177 and KSS, p=0.238). CONCLUSIONS: These findings suggest the importance of a preoperative evaluation of the fatty degeneration of deltoid and teres minor muscles for predicting postoperative strength and clinical outcome.
Arthroplasty* ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Muscle Strength ; Muscles* ; Shoulder*

Fat Embolism is a rare complication of multiple long bone fracture or extensive soft tissue injury. The pathogenesis of fat embolism has been poorly understood and still its definite pathogenesis, diagnosis and treatment were not fully established. Recently fat embolism considered as a post traumatic respiratory failure. Monitoring of blood gas is required for early diagnosis and respiratory supportive treatment with continued minitoring is necessary until resolution. Fifteen cases of fat embolism treated at from September 1979 to October 1981 Eul Ji General Hospital were clinically analized. Among the fifteen cases, fourteen were recovered without sequalae and one was expired ten days after trauma.
Diagnosis ; Early Diagnosis ; Embolism, Fat ; Fractures, Bone ; Hospitals, General ; Respiratory Insufficiency ; Soft Tissue Injuries

Azithromycin* ; Doxycycline* ; Scrub Typhus*

No abstract available.

No abstract available.
Encephalitis, Arbovirus*

No abstract available.
Child* ; Humans