الملخص
Hypophosphatasia (HPP), also called Rathbun disease, is a rare genetic disorder that is caused by the loss-of-function mutation in the ALPL gene encoding tissue nonspecific alkaline phosphatase. Doctor Rathbun first described the case of a 3-week-old infant who presented with severe osteopenia, rickets, and multiple radio graphic fractures, and died shortly after of epileptic seizure and respiratory distress. The term “hypophosphatasia” was coined as the patients’ alkaline phosphatase levels were significantly low. Since then, our understanding of HPP has evolved, and now we appreciate causative genetic mutation and the broad spectrum of clinical presentation depending on the age of onset, severity, and skeletal involvement: perinatal, infantile, childhood, adult and odontohypophosphatasia. The new development of enzyme replacement with asfostase alfa has saved the lives of severe form of hypophosphatasia. However, it is still unclear and remains challenging how to manage adult HPP that often presents with mild and non-specific symptoms such as muscle pain, joint stiffness, fatigue, anxiety, or low bone mass, which are common in the general population and not necessarily attributed to HPP. In this review, we will present 3 unique cases of adult HPP and discuss the pathophysiology, clinical presentation particularly neuromuscular and neurocognitive symptoms and management of adult HPP.
الملخص
A nematode species belonging to the genus Cosmocephalus was collected from the stomach of 2 common gulls, Larus canus. The common gulls were found dead on the seaside of Gangneung City, the Republic of Korea. The worms were identified and classified by light (LM) and scanning electron microscopy (SEM) on the basis of important taxonomic characters. The nematodes were characterized by a body length 9.1-9.3 mm (males) and 15.5-15.9 mm (females) and cordons recurrent in anterior direction and anastomosing laterally at about the level of anterior quarter of the buccal cavity. The salient bicuspid deirids were located on the posterior to the cordons. Lateral alae were well-developed, extending from the level just posterior of deirids to the level about middle of the body. LM and SEM observations identified the worms as C. obvelatus. This is the first reported case of C. obvelatus infection in common gulls in Korea.
الموضوعات
Animals , Female , Male , Charadriiformes/parasitology , Microscopy , Republic of Korea , Spirurida/anatomy & histology , Spirurida Infections/parasitologyالملخص
A nematode species belonging to the genus Cosmocephalus was collected from the stomach of 2 common gulls, Larus canus. The common gulls were found dead on the seaside of Gangneung City, the Republic of Korea. The worms were identified and classified by light (LM) and scanning electron microscopy (SEM) on the basis of important taxonomic characters. The nematodes were characterized by a body length 9.1-9.3 mm (males) and 15.5-15.9 mm (females) and cordons recurrent in anterior direction and anastomosing laterally at about the level of anterior quarter of the buccal cavity. The salient bicuspid deirids were located on the posterior to the cordons. Lateral alae were well-developed, extending from the level just posterior of deirids to the level about middle of the body. LM and SEM observations identified the worms as C. obvelatus. This is the first reported case of C. obvelatus infection in common gulls in Korea.
الموضوعات
Animals , Female , Male , Charadriiformes/parasitology , Microscopy , Republic of Korea , Spirurida/anatomy & histology , Spirurida Infections/parasitologyالملخص
The aim of this study was to provide descriptive information on meal and snack patterns and to investigate snacks in relation to energy intake and food choice according to the meal patterns of employed people in Korea. 683 employed people (292 males, 391 females) were interviewed to collect one day dietary data by using 24-h dietary recall. A recorded day was divided into 3 meal and 3 snack periods by the respondent's criteria and the time of consumption. To analyze the eating pattern participants were divided as the more frequent snack eaters (MFSE) and the less frequent snack eaters (LFSE). They were also categorized into 6 groups according to the frequency of all eating occasions. The common meal pattern in nearly half of the subjects (47.6%) was composed of three meals plus one or two snacks per day. A trend of an increasing the number of snacks in between main meals emerges, although the conventional meal pattern is still retained in most employed Korean adults. Women, aged 30-39, and urban residents, had a higher number of being MFSE than LFSE. Increasing eating occasions was associated with higher energy, protein, and carbohydrate intakes, with the exception of fat intakes. 16.8% of the total daily energy intake came from snack consumption, while the 3 main meals contributed 83.2%. Energy and macronutrient intakes from snacks in the MFSE were significantly higher than the LFSE. Instant coffee was the most popular snack in the morning and afternoon, whereas heavy snacks and alcohol were more frequently consumed by both of the meal skipper groups (< or =2M+2,3S and < or =2M+0,1S) in the evening. In conclusion, meal pattern is changing to reflect an increase of more snacks between the three main meals. Meal and snack patterns may be markers for the energy and macronutrient intakes of employed people in Korea.
الموضوعات
Adult , Aged , Female , Humans , Male , Coffee , Eating , Energy Intake , Korea , Meals , Porphyrins , Snacksالملخص
The present study was undertaken to determine the viability and infectivity of oocysts of Cryptosporidium baileyi that had been stored from 1 to 40 months at 4 degrees C preserved in 2.5% potassium dichromate solution. Oocysts of C. baileyi were purified from the feces of experimentally infected chickens using discontinuous sucrose gradients. Subsequently, the purified oocysts were suspended in 2.5% potassium dichromate solution at a concentration of 1 x 10 (7) organism/ml, and their viabilities were assessed by nucleic acid staining, histologic examination, and infectivity to 2-day-old chickens. All chickens inoculated with oocysts that had been stored for 1-18 months developed patent infections, while chickens infected with older oocysts remained uninfected. Between 5.8% and 82.2% of the oocysts, stored at 4 degrees C in 2.5% potassium dichromate solution, were found to be viable, as determined by nucleic acid staining. Parasite colonization in the bursa of Fabricius was detected in the microvillus border of bursal epithelium. The finding that C. baileyi oocysts remain infective to chickens for at least 18 months offers important time-saving advantages to investigators who frequently require large numbers of oocysts.
الموضوعات
Animals , Bursa of Fabricius/parasitology , Chickens/parasitology , Coloring Agents , Cryptosporidiosis/parasitology , Cryptosporidium/drug effects , Feces/parasitology , Oocysts/drug effects , Organic Chemicals , Potassium Dichromate/pharmacology , Poultry Diseases/parasitology , Preservation, Biological/methods , Staining and Labelingالملخص
Studies on the correlation between proliferative activity of biopsied specimen and pathologic findings of resected specimen have been carried out to find the prognostic factors. To estimate the proliferative activity, 100 cases of biopsied specimen of gastric adenocarcinoma were tested for the PCNA (proliferating cell nuclear antigen) and the AgNOR (argyrophilic nucleolar organizer region) by the immunohistochemical and histochemical stainings, respectively. The resected tumors classified by histologic type, differentiation, depth of invasion, and nodal metastatic status were followed by cell cycle analysis using flow cytometry. The PCNA LI (labelling index) were higher in well or moderately differentiated tumors (P<0.01) than the poorly differentiated ones and the aneuploid tumors (P<0.05) more than in diploid ones. However, there were no correlations among histologic types, depth of invasion, nodal metastatic status and PCNA LI. The AgNOR counts were higher in advanced tumor than in the EGC (early gastric cancer) (P<0.01). In cases with nodal metastasis, most of them showed the AgNOR counts higher than those without nodal metastasis. There were no correlations between the AgNOR counts and the DNA ploidy, histologic type, or differentiation. High PCNA LI and high AgNOR counts were shown in cases with advanced tumors (P=0.000) and nodal metastasis (P<0.05). No correlation was shown with the histologic type or differentiation. The results show that proliferative activity of the biopsied specimen of gastric adenocarcinoma is correlated with the differentiation and the invasion depth of resected specimen. Especially, better correlation is obtained by analyzing both the PCNA LI and the AgNOR counts than by analyzing each.