• Parasites, Hosts and Diseases
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• v.53 no.3
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• pp.355-359
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• 2015

Hydatid disease imposing serious threat on human health and great loss in live-stock pastoralism remains a major public health problem in western China. To assess and monitor the effect of control program on transmission dynamics, we used the prevalence of cystic echinococcosis in slaughtered sheep at slaughterhouse as an indicator during the period of 2007 to 2013 in Emin County, Xinjiang Uygur Autonomous Region, China. The results showed a significant decline trend of prevalence in all age groups during the 7 years when the control program was implemented; particularly, the rate was reduced by 72% after first 3 years. Among the sheep slaughtered, the age distribution evidenced that the prevalence increased significantly as the sheep grew older. The baseline data indicated that the rate was 4.5% at the age <1, 6.7% at age 2~, and reached to the highest 17.9% at age ${\geq}4$ years. Earlier response to the intervention pressure was seen in the sheep at the younger age. Significant decline started from 2008 at the age <1, from 2009 at age of 1~, 2010 at 2~ to 3~, and the latest, in 2012 at age ${\geq}4$. This study demonstrated that the prevalence of cystic echinococcosis in slaughtered sheep may be used as an indicator to assess and monitor the transmission status during and after control program providing information for betterment of performance to sustain control strength.

• Tuberculosis and Respiratory Diseases
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• v.43 no.5
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• pp.805-811
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• 1996

Congenital cystic adenomatoid malformation of the Lung(CCAM) is characterized by anomalous fetal development of terminal respiratory structures, resulting in an adenomatoid proliferation of bronchiolar elements and cystic formation. CCAM was first described and differentiated from other cystic lung disease in the English literature by Ch'in and Tang in 1949. CCAN is a rare, potentially lethal form of congenital pulmonary cystic disease and the salient features of lesion are an irregular network of terminal respiratory bronchiole-like structures and macrocysts variably lined by pseudostratified ciliated columnar epithelium and simple cuboidal epithelium. Adult presentation of CCAM of the lung is so rare that only 9 cases have been reported in the literature of date. The pathogenesis of CCAM remains disputed and reseachers have variously proposed that the lesion represents a developmental anomaly, hamartoma, or a fonn of pulmonary dysplasia. Van Dijk and Wagenvoort divided CCAM into three subtypes : cystic, intermediated, and solid. These correspond to types I, II, and III of Stocker. In adults, the evaluation of cystic or multi cystic lung disease requires consideration of a differential diagnosis including the acquired lesions of lung abscess, cavitary neoplasm or inflammatory mass, bullous disease, bronchiectasis, and postionflammatory pneumatocele. Congenital lesions such as sequestration, bronchopulmonary-foregut anomalies, and bronchogenic cyst are also encounted. The definitive treatment for CCAM is complele removal of the involved lobe. Panial lobectomy leads to multiple complications, including severe post-operative infection. We report a case of CCAM in a 14-year-old female presentated with a pneumothorax and large bullae, who was treated by surgical remove of the involved lobe.

• Clinical and Experimental Pediatrics
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• v.56 no.10
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• pp.456-458
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• 2013

Cystic fibrosis (CF) is a genetic disease with autosomal recessive inheritance and is common in Caucasian people. The prevalence of this disease is between 1/2,000 and 1/3,500 live births, and the incidence varies between populations. Although the CF transmembrane conductance regulator gene is expressed in the kidneys, renal involvement is rare. With advances in the treatment of CF, life expectancy has increased, and some previously unobserved disease associations are now seen in patients with CF. It is important to follow patients with CF for possible abnormalities that may accompany CF. In this paper, we present two rare cases of CF accompanied by nephrotic syndrome.

• Parasites, Hosts and Diseases
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• v.48 no.3
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• pp.263-265
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• 2010

Echinococcosis is a multisystem disease and has propensity to involve any organ, an unusual anatomical site, and can mimic any disease process. Primary peritoneal echinococcosis is known to occur secondary to hepatic involvement but occasional cases of primary peritoneal hydatid disease including pelvic involvement have also been reported. We report here 1 such case of primary pelvic hydatidosis mimicking a malignant multicystic ovarian tumor where there was no evidence of involvement of the liver or spleen. Our patient, a 27-year-old female, was detected to have a large right cystic adnexal mass on per vaginal examination which was confirmed by ultrasonography. Her biochemical parameters were normal and CA-125 levels, though mildly raised, were below the cut off point. She underwent surgery and on exploratory laparotomy, another cystic mass was found attached to the mesentery of the small gut. The resected cysts were processed histopathologically. On cut sections both large cysts revealed numerous daughter cysts. Microscopic examination of fluid from the cysts revealed free scolices with hooklets and the cyst wall had a typical laminated membrane with inner germinal layer containing degenerated protoplasmic mass. The diagnosis of pelvic hydatid disease was confirmed and patient was managed accordingly. Hydatid disease must be considered while making the differential diagnosis of pelvic cystic masses, especially in endemic areas.

• Journal of Chest Surgery
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• v.21 no.6
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• pp.1095-1098
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• 1988

Congenital cystic adenomatoid malformation[CCAM] is a rare disease that cause respiratory distress in the newborn and infants, but is one of the two causes along with lobar emphysema. This malformation has the pathologic characteristics which can be differentiated from other forms of diffuse cystic disease, i.e. CCAM is marked proliferation of the terminal bronchioles and that can enlarge rapidly by air trapping in cystic areas. The CCAM has a clinical importance because of rapid worsening respiratory distress, with tachypnea, subcostal retraction and cyanosis. This is a strict surgical condition and after operation[lobar, segmental resection or pneumonectomy] the symptoms relieved obviously. We experienced 6 cases of CCAM from July, 1980 to September, 1987 at the Department of Cardiovascular and Thoracic Surgery, Severance Hospital, College of Medicine, Yonsei University. The male patients were two and female patients were four. The age distribution was from premature to 10 year old. One of them was 27 weeks gestational premature female who was borne dead. The other 5 patients were performed on thoractomy[1 case pneumonectomy and 5 cases lobectomy]. The postoperative courses were good and no complications were seen.

• Journal of Chest Surgery
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• v.26 no.6
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• pp.501-504
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• 1993

Adenoid cystic carcinoma of the tracea is rare, but is a very serious critical life-threatening disease.Nearly all the lesions of the trachea are presented as obstructive lesions. Bronchoscopic examination including chest CT, tomogram and air tracheogram are essential for the further definition of these lesions. This is a case report of adenoid cystic carcinoma in a 47 years old male patient. The tumor was located in cervical trachea with wide base and obstructing the lumen almost completely. The patient`s symptom was productive cough and hoarseness for 4 months. The tumor was resected and End-to-End anastomosed. The tumor was confirmed to be adenoid cystic carcinoma histopathologically. The need for removal of tracheal tumor whether complete or incomplete, is clear enough regardless of the histology of the tumor. This patient was treated post-operatively with radiation.

• Molecules and Cells
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• v.46 no.1
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• pp.10-20
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• 2023

Antisense oligonucleotide (ASO) technology has become an attractive therapeutic modality for various diseases, including Mendelian disorders. ASOs can modulate the expression of a target gene by promoting mRNA degradation or changing pre-mRNA splicing, nonsense-mediated mRNA decay, or translation. Advances in medicinal chemistry and a deeper understanding of post-transcriptional mechanisms have led to the approval of several ASO drugs for diseases that had long lacked therapeutic options. For instance, an ASO drug called nusinersen became the first approved drug for spinal muscular atrophy, improving survival and the overall disease course. Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause cystic fibrosis (CF). Although Trikafta and other CFTR-modulation therapies benefit most CF patients, there is a significant unmet therapeutic need for a subset of CF patients. In this review, we introduce ASO therapies and their mechanisms of action, describe the opportunities and challenges for ASO therapeutics for CF, and discuss the current state and prospects of ASO therapies for CF.

• Parasites, Hosts and Diseases
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• v.53 no.6
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• pp.699-703
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• 2015

Cystic echinococcosis (CE) is one of the most widespread zoonotic helminthiases, which can last an asymptomatic infection for several years. The purpose of this study was to demonstrate serum antibody prevalence of CE among asymptomatic people in Uzbekistan using ELISA. A total of 2,547 serum samples were collected, 66 from confirmed CE patients and 2,481 of patients with other diseases than CE at a hospital in Tashkent, Uzbekistan. The serum samples were screened for CE specific IgG antibodies by ELISA using cystic fluid antigen obtained from sheep. The serum antibody positive rate was 89.4% (59/66) in CE and 3.6% (89/2,481) in other disease patients. The present ELISA recognized 89.4% sensitivity and 96.4% specificity. The ELISA absorbance of positive samples was distributed 0.271-0.971 for CE and 0.273-0.887 for other disease patients. The other disease patients with high absorbance over 0.3 were 50 (2.0%) who were presumed to be active CE patients. The patients in their 40s showed the highest positive rate of 5.2% (P=0.181), and women were 4.4% while men were 3.1% positive (P=0.136). The data confirmed that there are many asymptomatic patients of CE in Tashkent. It is indicated that CE is an endemic disease of public health importance in Uzbekistan.

• Childhood Kidney Diseases
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• v.3 no.2
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• pp.221-226
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• 1999

Glomerulocystic kidney disease(GCKD) is a rare form of renal cystic disease defined histopathologically by containing dilated Bowman's space with variable atrophy of glomerular tufts, which may occur as sporadically or as familial cases and can be presented as a major component of heritable syndromes. It has not been recognized in Korean children but only one report of adult case has been reported having GCKD. We experienced a case of GCKD in a 10-year-10-month-old boy, who was admitted for hypertension. Abdominal ultrasonography and computed tomography revealed clustered numerous small cysts in left kidney and renal biopsy findings was consistent with the GCKD showing cystic dilatation of Bowman's space with intact glomerular structure.

• Journal of Yeungnam Medical Science
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• v.29 no.2
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• pp.136-140
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• 2012

A 61-year-old male who complained of right upper quadrant pain was referred to the authors for evaluation after his computed tomography suggested biliary adenocarcinoma. The lesion consisted of multiple cysts with papillary mass and peri-ampullay mass. The patient underwent an operation due to a clinical suspicion of biliary cystadenocarcinoma, but the pathology confirmed biliary papillomatosis (BP) after diagnosing intrahepatic papillary neoplasm with high-grade dysplasia and invasive adenocarcinoma with papillary neoplasm from the distal common bile duct to the duodenum. BP is a disease characterized by multiple papillary masses. Its cause has yet to be discovered. It commonly manifests as bile duct dilation but rarely as a ductal cystic change. Under computed tomography or magnetic resonance imaging, both the BP and the cystic neoplasm can show bile duct dilation and a papillary mass, which makes their differential diagnosis difficult. A confirmative diagnosis can be made through a pathologic examination. BP is classified as a benign disease that can become malignant and may recur, though rarely. Its treatment of choice is surgical resection. Laser ablation or photodynamic therapy can be used for unresectable lesions. In the case featured in this paper, biliary papillomatosis was difficult to differentiate from cystic adenocarcinoma due to diffusely scattered multiple large cystic lesions in the liver, and it was histologically confirmed to have become malignant with cystic duct dilation after the operation. This case is reported herein with a literature review.