Atypical hemolytic uremic syndrome (aHUS) is a rare, progressive, life-threatening condition of thrombotic microangiopathy characterized by thrombocytopenia, microangiopathic hemolytic anemia, and renal impairment. The mechanisms underlying aHUS remain unclear. Herein, we present the first case in the literature of aHUS after a traumatic injury. A 55-year-old male visited the emergency department after a traumatic injury caused by a tree limb. Abdominal computed tomography revealed a rectal wall defect with significant air density in the perirectal space and preperitoneum, implying rectal perforation. Due to the absence of intraperitoneal intestinal perforation, we performed diverting sigmoid loop colostomy. An additional intermittent simple repair was performed due to perianal and anal injuries. One day postoperatively, his urine output abruptly decreased and serum creatinine level increased. His platelet level decreased, and a spiking fever occurred after 2 days. The patient was diagnosed with acute renal failure secondary to aHUS and was treated with fresh frozen plasma replacement. Continuous renal replacement therapy (CRRT) was also started for oliguria and uremic symptoms. The patient received CRRT for 3 days and intermittent hemodialysis thereafter. After hemodialysis and subsequent supportive treatment, his urine output and renal function improved. The hemolytic anemia and thrombocytopenia also gradually improved. Dialysis was terminated on day 22 of admission and the patient was discharged after recovery. This case suggests that that a traumatic event can trigger aHUS, which should be considered in patients who have thrombocytopenia and acute renal failure with microangiopathic hemolytic anemia. Early diagnosis and appropriate management are critical for favorable outcomes.
In this study the effect of vitamin C administration on pregnancy rates during summer heat stress in dairy cows was examined. A total of 80 Holstein-Friesian cows were divided into control and treatment groups (n = 40 each). Control group animals were given 10 mL isotonic normal saline, and treatment group, Vitamin C (4 mg/kg) on artificial insemination day (day 0) and 4th, 8th and 12th day post insemination. Pregnancy diagnosis was performed on 30th day post insemination by ultrasonography. Blood samples were randomly taken from 11 animals from each group. Serum P4, GSH, MDA and plasma 8-OHdG levels were determined by using ELISA method. Results showed that 8-OHdG levels were lower in treatment group on day 4, 8 and 12 (p < 0.05) compared with the control group. Similarly, pregnancy rate was higher in treatment group (32.5%) than control (22.5%), respectively. However, MDA, P4 and GSH levels were similar in both groups at 4th, 8th and 12th day. A gradual increase in P4, and MDA levels, and a strong positive correlation between 0, 4th (r = 0.54), 4, 8th (r = 0.59) and 8, 12th (r = 0.51) day was found. Similarly, GSH levels also showed positive correlation at days 0, 4th (r = 0.47) and 4, 8th (r = 0.56). However, a strong negative correlation (r = -0.56) between MDA day 0, and GSH day 8 was found. In conclusion, vitamin C application during insemination period in postpartum cows increases pregnancy rate, and reduces oxidative stress metabolite 8-OHdG levels.
[Purpose] Epidemiological evidence has shown that leisure-time physical activity and structured exercise before and after breast cancer diagnosis contribute to reducing the risk of breast cancer recurrence and mortality. Thus, in this review, we aimed to summarize the physical activity-dependent regulation of systemic factors to understand the biological and molecular mechanisms involved in the initiation, progression, and survival of breast cancer. [Methods] We systematically reviewed the studies on 1) the relationship between physical activity and the risk of breast cancer, and 2) various systemic factors induced by physical activity and exercise that are potentially linked to breast cancer outcomes. To perform this literature review, PubMed database was searched using the terms "Physical activity OR exercise" and "breast cancer", until August 5th, 2020; then, we reviewed those articles related to biological mechanisms after examining the resulting search list. [Results] There is strong evidence that physical activity reduces the risk of breast cancer, and the protective effect of physical activity on breast cancer has been achieved by long-term regulation of various circulatory factors, such as sex hormones, metabolic hormones, inflammatory factors, adipokines, and myokines. In addition, physical activity substantially alters wholebody homeostasis by affecting numerous other factors, including plasma metabolites, reactive oxygen species, and microRNAs as well as exosomes and gut microbiota profile, and thereby every cell and organ in the whole body might be ultimately affected by the biological perturbation induced by physical activity and exercise. [Conclusion] The understanding of integrative mechanisms will enhance how physical activity can ultimately influence the risk and prognosis of various cancers, including breast cancer. Furthermore, physical activity could be considered an efficacious non-pharmacological therapy, and the promotion of physical activity is probably an effective strategy in primary cancer prevention.
We report a case of successful treatment of congenital syphilis in an extremely preterm baby. A 1,395 g female infant was born by emergency Caesarean section due to preterm labor and breech presentation at gestational age at 29 weeks and 3 days with an Apgar score of 2 and 4 at 1 minute and 5 minutes, respectively. The mother of the newborn, an illegal immigrant who did not receive any antenatal care, was diagnosed as active syphilis infection by reactive rapid plasma regain (RPR) (titer 1:128) just before the delivery. Upon birth, the newborn presented with various clinical manifestations, including severe respiratory distress syndrome, persistent pulmonary hypertension of the newborn, disseminated intravascular coagulopathy, desquamation and scaling of the whole body, and osteolytic changes of long bone ends. Results of laboratory tests showed signs of early congenital syphilis, including positive syphilis reagin test (12.7 R.U.), reactive with RPR titer of 1:64, and positive for immunoglobulin (Ig) M and IgG fluorescent treponemal antibody absorption test. However, after completion of penicillin G treatment for two weeks, laboratory results dramatically improved, showing a negative syphilis reagin test (0.5 R.U.) and non-reactive in RPR. In conclusion, the incidence of congenital syphilis is prone to be resurgent in South Korea, neonatologists should be fully aware of the clinical features of congenital syphilis because early diagnosis and prompt treatment are essential in order to reduce the social and economic burden due to congenital syphilis.
Eosinophilic gastrointestinal disease (EGID), a chronic allergic condition characterized by dense infiltration of eosinophils in the digestive tract, is classified into two types, eosinophilic esophagitis (EoE), which features dense infiltration of eosinophils in the esophageal epithelial layer, and eosinophilic gastroenteritis (EGE), in which the entire digestive tract including the esophagus may be involved. Patients with EoE only have esophageal symptoms, since the other parts of the digestive tract are not involved. On the other hand, 80% of EGE patients have lesions in the small intestine. The esophageal epithelial layer in healthy individuals has no or negligible infiltration by eosinophils, while the small intestinal mucosal layer, especially the distal small intestinal mucosa, can show dense eosinophil infiltration even in the absence of disease. Therefore, histological changes observed in cases of EGE are not qualitative but rather quantitative, as compared to EoE, which has qualitative histopathological changes, indicating important pathogenetic differences between the types. Comparisons of clinical, laboratory, and morphological characteristics between EoE and EGE have revealed several interesting differences. Both EoE and EGE patients are frequently affected by atopic diseases, such as bronchial asthma and allergic rhinitis, and elevated plasma levels of Th2 type cytokines and chemokines are also similarly seen in both. On the other hand, age at diagnosis differs, as the former is generally found in individuals from 30 to 50 years old, while the latter appears in all age groups. Additionally, 80% of patients with EoE are male as compared to only 50% of those with EGE. Furthermore, approximately 60% of patients with EoE respond favorably to proton pump inhibitor (PPI) administration, whereas EGE patients rarely show a response to PPIs. Nevertheless, both diseases show a similarly favorable response to a six foods elimination diet and glucocorticoid administration. These similarities and differences of EoE and EGE provide important clues for understanding the pathogenesis of these EGID types.
Da-young An;Chae-eun Kim;Mi-kyung Kim;Seung-ho Sun
The Journal of Internal Korean Medicine
/
v.44
no.6
/
pp.1279-1293
/
2023
A 67-year-old female patient diagnosed with acute transverse myelitis visited our hospital in February 2023. Consultation papers indicated that she was diagnosed with acute transverse myelitis and that she complained of quadriparesis and numbness in both hands and feet. However, her spine magnetic resonance imaging (MRI) did not clearly indicate that she suffered from acute transverse myelitis. She was then sent to a high-level hospital and diagnosed with Guillain-Barre Syndrome through electromyography, cerebrospinal fluid test, and spine MRI. On March 2023, she visited our hospital again, still complaining about weakness in both hands and inability to walk. She was treated with Korean medicine, including acupuncture, electroacupuncture, pharmacopuncture, and herbal medicine such as Yanggyeoksanhwatang and Sipimijihwang-tanggami. After three months, she was discharged on cane walking. The manual muscle test (MMT) grade of her upper limbs increased from 3-/3-/3-/3- (shoulder/elbow/wrist/fingers) to 4/4/4/3+, and the MMT grade of her lower limbs increased from 2/2/2/2 (pelvis/knee/ankle/feet) to 4/4/4-/4-. Her numbness in both feet disappeared, and her numbness in both hands decreased from a numeric rating scale of 7 to 2. No adverse events were reported during treatment. This case implies the therapeutic potential of Korean medicine for Guillain-Barre Syndrome, especially for those who had delayed diagnosis and missed the opportunity to get plasma exchange.
Inflammatory myofibroblastic tumors (IMTs) are rare. They are characterized by myofibroblastic spindle cell proliferation with a varying degree of inflammatory cell infiltration. IMT can occur in any anatomic location but has been reported in the lung, mesentery, and omentum, mainly in children or young adults. It rarely occurs in the pancreas and is often difficult to distinguish from other tumors, including some malignant ones. Therefore, it can be challenging to make a radiological diagnosis of IMT. Here, we present a case of IMT that occurred in the pancreas head of a middle-aged female. The patient's ultrasonography, computed tomography, and magnetic resonance imaging findings are presented along with a review of the literature.
Kim, Jinsup;Yang, Misun;Yang, Aram;Cho, Eun Hye;Park, Hyung-Doo;Sohn, Young Bae;Cho, Sung Yoon;Jin, Dong-Kyu
Journal of The Korean Society of Inherited Metabolic disease
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v.17
no.3
/
pp.85-91
/
2017
Purpose: The aim of this study was to describe the clinical and biochemical features as well as the molecular analysis of a newly diagnosed illustrative case with ML II and to analyze the clinical features of 11 Korean patients with ML II/III. Method: Including a newly diagnosed patient, total 11 patients in 10 families were diagnosed as ML II (n=7) or ML III (n=4) were enrolled in the study. A diagnosis of ML II or III was made by demonstrating increased lysosomal enzyme activities in the plasma and sequence analysis of GNPTAB with characteristic clinical features. Result: A illustrative case of ML II patient was a 17 month-old boy showing characteristic facial appearance, multiple joint contractures with cardiac involvements. The enzyme assay showed increased lysosomal enzyme activities in the plasma. We identified compound heterozygous mutations in GNPTAB sequence analysis, including a frameshift (c.3428dupA [pAsn1143Lysfs*3]) and a nonsense variant c.673C>T (p.Gln225*). In total 11 patients with ML II/III, the patients with ML II showed severe growth retardation (height standard deviation score -3.2 [${\pm}1.5$]), compare to patients with ML III. Furthermore, patients with ML II patients had serious cardiac problem (n=4), hepatomegaly (n=3) and underwent tracheostomy (n=3) with further respiratory support due to respiratory distress. To improve osteoporosis and bone pain, all patients with ML III and four of 7 patients with ML II treated with intravenous pamidronate. Conclusion: Here we showed a newly diagnosed case of ML II and clinical features of 11 Korean patients with ML II or III. These data could be helpful for further diagnosis of mucolipidosis, a rare inherited metabolic disease, in Korea.
Park Jee-Min;Shin Jae-Il;Kim Pyung-Kil;Lee Jae-Seung
Childhood Kidney Diseases
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v.6
no.2
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pp.155-168
/
2002
Purpose; Systemic lupus erythematosus(SLE) is an autoimmune disease with multi-system involvement and renal damage is a major cause of morbidity and mortality in children. Renal involvement is more common and severe in children than in adults. Therefore, renal biopsy plays a crucial role in planning effective therapy. In this study, we investigated the clinical and pathological findings of lupus nephritis in children to aid clinical care of the disease. Methods: The clinical and pathological data of 40 patients who were diagnosed as SLE with renal involvement in Shinchon Severance Hospital from Jan. 1990 to Sep. 2002 were analyzed retrospectively. Results: The ratio of male to female patients was 1:3 and the median age at diagnosis was 12.1(2-18) years old. FANA(95.0%), anti-ds DNA antibody(87.5%), malar rash(80.0%) were the most common findings among the classification criteria by ARA. Microscopic hematuria with proteinuria(75.0%), nephrotic syndrome(55.0%), and microscopic hematuria alone(15.0%) were the most common renal presentations in the respective order at diagnosis. There were 27 cases with WHO class IV lupus nephritis confirmed by renal biopsy and 3 cases with pathological changes of WHO class type. Different treatment modalities were carried out : prednisolone only in 5 cases, prednisol-one+azat-hioprine in 9 cases, prednisolone+azathioprine+intravenous cyclophosphamide in 14 cases, prednisolone+cyclosporine A+intravenous cyclophosphamide in 12 cases, plasma exchange in 9 cases and intravenous gamma-globulin in 2 cases. The average follow-up period was $51.8{\pm}40.5$ months. During $51.8{\pm}40.5$ months. During follow-up, 4 patients expired. The risk factors associated with mortality were male, WHO class IV and acute renal failure at diagnosis. Conclusion: Renal involvement was noted in 63.5% of childhood SLE, and 67.5% of renal lesion was WHO class IV lupus nephritis which is known to be associated with a poor prognosis. Therefore aggressive treatment employing immunosuppressant during the early stages of disease could be helpful in improving long-term prognosis. But careful attention should be given to optimize the treatment due to unique problems associated with growth, psychosocial development and gonadal toxicity, especially in children.
Yoon, Jae Ho;Yeo, Chang Dong;Shin, Eun Joong;Song, So Hyang;Kim, Chi Hong;Moon, Hwa Sik;Song, Jeong Sup;Park, Sung Hak
Tuberculosis and Respiratory Diseases
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v.61
no.3
/
pp.294-298
/
2006
Lymphocytic interstitial pneumonia(LIP) is an uncommon condition in which the alveolar septa and extra-alveolar interstitial space are markedly expanded by small lymphocytes, plasma cells and histiocytes. Chest radiographs generally show nonspecific patterns with the most common pattern showing bibasilar reticular or reticulonodular infiltrates. Hilar or mediastinal lymphadenopathy and pleural effusions are usually absent. We encountered a 42-year-old female patient who was admitted to hospital because of exertional dyspnea and palpitation. The chest X-ray showed an enlarged bilateral hilar shadow and diffusely increased bronchovascular markings in both lung fields. The chest CT showed diffuse nodular infiltrations with mild septal thickening and combined patchy ground glass opacity in both lungs, and conglomerated mediastinal and bilateral hilar lymphadenopathy. A diagnosis of LIP was made from the tissue pathology taken by a thoracoscopic lung biopsy. The patient showed clinical and radiographic improvement after 3 months of treatment with prednisolone. We report a case of LIP presenting as diffuse nodular interstitial infiltrations with multiple mediastinal and bilateral hilar lymphadenopathy.
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