• The Korean Journal of Medicine
• /
• v.99 no.2
• /
• pp.116-121
• /
• 2024

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis predominantly involving medium- or small-sized arteries, typically of the kidneys and other internal organs. Given the rarity of PAN and the variable clinical presentation, diagnosis is challenging and, to date, no definitive diagnostic marker has been identified. A patient diagnosed with immunoglobulin A nephropathy was observed to exhibit deterioration in renal function. To determine whether new structural abnormalities had developed, computed tomography scans of the kidneys, ureters, and bladder were obtained. Both kidneys exhibited multiple cortical defects, and a renal angiogram was performed to determine the cause. Angiography revealed partial obliteration of the left distal renal artery branches and multifocal extensive infarctions in both kidneys, and the patient was diagnosed with renal-limited PAN. Following steroid monotherapy, an improvement in renal function was observed. We believe that this case report may be helpful to physicians who assess and treat patients with suspected renal-limited PAN.

• Journal of Cerebrovascular and Endovascular Neurosurgery
• /
• v.25 no.1
• /
• pp.87-92
• /
• 2023

Moyamoya disease (MMD) is a rare progressive steno-occlusive cerebrovascular disorder. Currently, revascularization surgery is used as optimal treatment to overcome MMD. However, revascularization for MMD has reported several complications. Also, iatrogenic complications such as pseudoaneurysms formation or dural arteriovenous fistulas (dAVFs) formation-has been identified in rare cases after the surgical intervention for revascularizations. We describe two cases. In first case, the patency of the anastomosis site was good and saccular type pseudoaneurysm formation was found at parietal branch of posterior middle meningeal artery (MMA) in transfemoral cerebral angiography (TFCA) performed on the twelfth day after surgery. We decided to treat pseudoaneurysm by endovascular embolization the next day, but the patient was shown unconsciousness and anisocoria during sleep at that day. Computed tomography showed massive subdural hemorrhage at the ipsilateral side, thus we performed decompressive craniectomy and hematoma evacuation. In second case, the patency of the anastomosis site was good and dAVF formation at right MMA was found in TFCA performed on the sixth day after surgery. We performed endovascular obliteration of the arteriovenous fistula under local anesthesia. Pseudoaneurysm formation or dAVF formation after revascularization surgery is an exceptional case. If patients have such complications, practioner should carefully screen the patients by implementing digital subtraction angiogram to identify anatomic features; as well as consider immediate treatment in any way, including embolization or other surgery

• Childhood Kidney Diseases
• /
• v.4 no.1
• /
• pp.48-56
• /
• 2000

Purpose: Alport SD., the most common herectitary rephriris, is a renal disease with rapid progression. Deafness, ocular abnormalities and a specific EM finding may be associated in addition to a family history. We have aralyged retrospectively. Methods: We observed 12 children with Alport syndrome who were diagnosed at Dept. of pediatrics in Kyunghee Univ., College of Medicine, from Apr. 1991 until Jun. 1999. We used four criteria for diagnosis: renal disease, family history, deafness or eye abnormalities, and a specific finding in electron microscopy Results: 2 of 12 patients had all features of the four diagnostic criteria. We could not trace an exact family history in 3 patients, and 6 patients did not exhibit deafness or eye abnormality. One could not have renal biopsy because offer chronic renal failure. Other three criteria were observed in her. The ratio of male to female observed was 1:2 respectively and the mean age of initial renal symptom was 5.6 years. 9 of 12 patients had a family history of renal disease. In the audiogram and ocular examination for 11 of 12 cases, sensorineural hearing loss was observed in 6 and ocular abnormality in 2 cases. In electron microscopic finding, irregular thickness of the capillary basement membranes with lamination of lamina densa and foot process obliteration was noted in 9 of 11 and thin basement membrane with splitting and foot process obliteration was noted in the other 2. The mean period of follow-up was 3 6/12 years. And one patient developed the chronic renal failure until now and had kidney transplantation. Conclusion: For the diagnosis of Alport syndrome, the following four diagnostic criteria are very important : renal disease, family history, deafness or eye abnormalities, and a specific finding on electron microscopy. We expect that more patients can be detected through the analysis of these characteristics.

• Proceedings of the KOR-BRONCHOESO Conference
• /
• 1979.05a
• /
• pp.3.2-3
• /
• 1979

Bone conduction loss is one of the most common complication in chronic otitis media, and is mostly high frequency loss. Of 233 tympanomastoidectomy ears, 187 ears were considered eligible for this study. A histopathological change was examined in the natural otitis media of guinea pigs. It is our intention to analyze the pattern of bone conduction loss in chronic otitis media, and to correlate this findings with clinical and pathological changes in human and animal otitis media. l) In unilateral cases, a significant difference in bone conduction threshold was observed between normal and diseased ears, and between each frequency with significant interaction between 2KHz and 4KHz (p 0.01). 2) Using one way analysis of variance, mean bone conduction was compared with the duration of disease. We observed a significant difference (p 0.05) between each group of duration, except between 11-15 and 15-20 years group. 3) A comparison of bone conduction between stapes loss group and intact stapes group revealed significant t ratio (p 0.01) at each frequency. The effect of stapes loss on each frequency was evaluated, using one way analysis of variance. there were significant difference(p 0.05) between 250Hz and 500Hz. and between 2KHz and 4KHz. 4) A comparison of bone conduction between round window obliteration and nonobliteration group revealed significant t ratio (p 0.01) at each frequency. Using one way analysis of variance. the effect of round window obliteration was evaluated in each frequency. We observed significant difference (p 0.05) between 250Hz and 500Hz. and between 2KHz and 4KHz. 5) A comparison of bone conduction between cholesteatoma and non -cholesteatoma group revealed significant t ratio (p 0.01) only in 2KHz and 4KHz. No significant differency was observed in mean bone conduction. 6) In a histopathological study of natural otitis media in guinea pig, we observed inflammatory infiltration of the round window membrane, serofibrinous precipitate in the scala tympani, and degeneration of the organ of Corti most significant near the basal turn. These changes would explain high tone bone conduction loss in the process of chronic otitis media.

• Journal of Chest Surgery
• /
• v.32 no.3
• /
• pp.215-223
• /
• 1999

Background: The replacement of the narrowed long-segment trachea with various prosthetic materials or tissue grafts remains a difficult and unsolved surgical problem. Homologous cryopreserved tracheal transplantation has been considered to treat the irreversibly-damaged organs, such as in the lung or heart transplantation and also to overcome the limited supply of donor organs. We examined the morphological changes and the immunosuppressive effects of the cryopreserved trachea after the heterotopic transplantation in the rats. Material and Method: Sixty tracheal segments harvested from 30 donor Wistar rats were heterotopically implanted into the peritoneal cavity of 20 recipient Wistar rats and 40 Sprague Dawley rats. The 60 recipient rats were divided into 6 groups(10 rats/ group). In groups I, II, and III, 30 tracheal segments were implanted immediately after the harvesting and in groups IV, V, and VI, the segments were implanted 28 days after the cryopreservation. Groups I and IV were Wistar syngeneic controls. Groups II and V were Sprague Dawley recipients receiving no immunosuppression and Groups III and VI, were Sprague Dawley recipients receiving immunosuppressive agents. At 28 days all rats were sacrificed and the tracheal segments were evaluated grossly and histologically. Result: Immunosuppression of the tracheal segments had a significant influence on the changes of the tracheal lumen and tracheal epithelial cells, irrespective of the cryopreservation of the trachea(p<0.001). In groups III and VI receiving immunosuppressive agents, the tracheal lumen was patent and the normal epithelial cells were observed, however in the other groups not receiving the immunosuppressive agents, there were almost luminal obliteration by the proliferation of the fibrous tissues and a loss of the epithelial cells, the findings were similar to those in the case of obliterative bronchiolitis after a lung and a heart-lung transplantation. Conclusion: With the appropriate immunosuppressive agents, the lumen and the respiratory epithelium of the transplanted tracheal segment were well preserved, even after the cryopreservation of the tracheal segment, which shows the possibility of the long-term preservation and homologous transplantation of the trachea. But fibroproliferative obliteration of the tracheal lumen and the loss of the normal respiratory epithelial cells, characteristic findings of obliterative bronchiolitis, were observed in the groups without the immunosuppression. This experiment using the rat trachea may be useful in studying the pathogenesis, treatment, and prevention of obliterative bronchiolitis after a lung and a heart-lung transplantation.

• Tuberculosis and Respiratory Diseases
• /
• v.42 no.4
• /
• pp.481-491
• /
• 1995

Background: Recently there has been a trend of an increasing incidence of mediastinal tuberculous lymphadenitis(MTL) in adults. MTL often cause bronchial stenosis or esophago-mediastinal fistula. In spite of effective treatment, it is difficult to cure. Moreover, relapse frequently occurs. Authors analyzed chest CT findings and clinical features of 29 cases with MTL Methods: 29 cases with MTL were retrospectively studied with the clinical and radiologic features from April 1990 to March 1995 Results: 1) A total of 29 cases were studied. 12 cases were male and 17 cases were female. The male to female ratio was 1:1.4 Mean age was 29 years old. The 3rd decade(45%) was the most prevalent age group 2) The most common presenting symptoms and signs were palpable neck masses(62%) followed by cough(59%) and sputum(38%) 3) Except in one case of MTL, all patients had coexisting pulmonary tuberculosis, cervical tuberculous lymphadenitis, endobronchial tuberculosis and tuberculous pleurisy. Among the coexisting tuberculous diseases, Pulmonary tuberculosis was the most common(76%) 4) On simple chest X-ray, mediastinal enlargement was noted in 21 cases(72%), but it was not noted in 8 cases(28%). The most frequently involving site was the paratracheal node in 16 cases(72%). Rt side predominence(73%) was noted 5) Patterns of node appearance on a postcontrast CT scan were classified into 3 types. There were 19 cases(30%) of the Homogenous type, 30 cases(47%) of the Central low density type and 15 cases(23%) of the Peripheral fat obliteration type. The most common type was the central low density type. The most common lymph node size was 1~2 cm(88%) 6) The most frequently involved site was the paratracheal node in 26 cases(89%) by chest CT. Rt side(63%) was predominant 7) 9 cases(43%) had complete therapy and most common treatment duration was 13 - 18 months. 12 cases(57%) had incomplete continuing antituberculous medication and half of the cases had been treated above 19 months. Conclusion: Chest CT findings of MTL showed central low density area and peripheral rim enhancement, so this characteristic findings could differentiate it from other mediastinal diseases and help a diagnosis of tuberculosis. In spite of effective antituberculous medication, it is difficult to cure. Moreover, relapse frequently occurs. Further studies will be needed of the clinical features and the treatment of MTL.

• Clinical and Experimental Pediatrics
• /
• v.48 no.3
• /
• pp.327-332
• /
• 2005

Interstitial lung disease refers to a group of pulmonary disorders characterized by inflammation of the interstitium, derangements and loss of alveolar capillary units leading to disruption of alveolar gas exchange, which induces symptoms of restrictive lung disease. Cases of interstitial pneumonia in children are uncommon and mostly have unknown causes. We have experienced an 8-year old boy who had symptoms of cyanosis, dry cough, dyspnea and abrupt weight loss. He had not been exposed to organic dusts, allergens or any other systemic disease infections. Chest radiology showed diffuse ground glass opacity in both lung fields. High resolution computed tomography(HRCT) showed multiple small patchy areas of consolidation with an underlying ground glass appearance in both lungs. The pathologic findings of lung biopsy tissue showed patchy areas of interstitial fibrosis, alveolar obliteration and nodular fibrotic areas, strongly suggesting interstitial pneumonia. No specific finding of viral inclusion or any other evidence of infection was found under electromicroscopy. We used peak flow meters to compare functional improvement. Forced expiratory volume in one second ($FEV_1$) was decreased to 25 percent of predicted value. The boy was given treatment with prednisone and showed improvements in HRCT findings after two months. He was able to tolerate easy exercise in school and showed clinical improvements after one year of follow up.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.27 no.1
• /
• pp.1-6
• /
• 2000

Dentinogenesis imperfecta is an inherited disorder of dentin formation, usually exhibiting an autosomal dominant mode of transmission. Type I dentinogenesis imperfecta occurs in patients afflicted with osteogenesis imperfecta. Type II dentinogenesis imperfecta is not associated with osteogenesis imperfecta. Type III dentinogenesis imperfecta (Brandywine type) occurs in a racial isolate area in the state of Maryland. In all three types, teeth of both dentitions are affected with variable clinical appearances. The teeth are opalescent with the color ranging from bluish-gray to brown to yellowish. The dentin is abnormally soft, providing inadequate functional support to the overlying enamel. Although the enamel is normal, it fractures or chips away easily, exposing the occlusal and incisal dentin. The exposed soft dentin often undergoes rapid and severe functional attrition. The teeth exhibit bulb-shaped crowns with constricted cementoenamel junctions and thin roots. The teeth will exhibit varying stages of obliteration of the coronal and root pulpal chambers. The cementum, periodontal ligament and supporting alveolar bone appear normal. The enamel is normal. The mantle dentin remains nearly normal, whereas the remaining dentin is severely dysplastic. The dentinal tubules are disoriented, irregular, widely spaced, and usually larger than normal.

• The Journal of Korean Academy of Prosthodontics
• /
• v.56 no.4
• /
• pp.317-322
• /
• 2018

The tongue, especially its lateral part, is the most common site of oral tumors. Patients who undergo glossectomy for the treatment of tongue cancer may experience difficulty in proper functioning for pronunciation, chewing, swallowing, and oral hygiene maintenance; therefore, a palatal augmentation prosthesis can be used to restore function of the tongue. In this case, an implant overdenture was used in a patient who had residual ridge resorption and obliteration of alveololingual sulcus after undergoing glossectomy for tongue cancer treatment. In addition, a palatal augmentation prosthesis with a metal framework, support, and retention part was fabricated. The palatal vault was reduced, so that even with limited tongue movement, adequate tongue-palate contact could be achieved. After placement of the definitive prostheses, the patient showed improvement in the functions of chewing, swallowing, and pronunciation.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.36 no.2
• /
• pp.293-297
• /
• 2009

Traumatic intrusion is a type of injury that involves axial displacement of a tooth toward the alveolar bone. Its occurance is relatively rare compared to other types of luxation in permanent dentition. It is more common in boys than in girls, and most common etiology of intrusion is fallen down. Various complication may occur following traumatic intrusion, such as pulp necrosis, root resorption, pulp obliteration and marginal bone loss. In addition, traumatic intrusion is commonly combined with hard or soft tissue injuries. Therefore, it is difficult to establish proper treatment plan. Choice of treatment for an intruded tooth by trauma include waiting for spontaneous re-eruption, orthodontic repositioning, and surgical repositioning. In this case, we repositioned the intruded central incisor using orthodontic traction, in a six-year old girl, which failed to re-erupt spontaneously.