Introduction and importance Protein C deficiency is a rare disorder associated with an increased risk of developing abnormal blood clots. Mild and heterozygous cases are usually asymptomatic and may present with recurrent thrombosis. These recurrent thrombi are usually associated with ischemic stroke or concomitant thrombosis during pregnancy and recurrent miscarriage, but arterial thrombosis is relatively uncommon. Case presentation In this case report, we introduce an interesting 21-year-old female patient with a protein C deficiency, which presented with a set of symptoms related to ischemia and thrombosis in several different systems, including the colon, brain, and lower extremities. Clinical discussion With the diagnosis of ischemic colitis, she underwent medical treatment with hydration, antibiotics, anticoagulant, and GI rest. She was discharged with a suitable response to medical treatment and good general condition. One month later, the patient presented with right upper limb paresis and speech disorder, and at the same time, he had swelling of the left lower limb. The patient was diagnosed with DVT of the common femoral vein and protein c deficiency and treated appropriately with anticoagulant (heparin 1000 lU/h) and was discharged with oral rivaroxaban after symptoms improved. Conclusion In young patients with ischemic colitis without a history of previous surgery, increased coagulation should be considered, including impaired fibrinolysis and impaired microcirculation and inflammatory processes. Protein C deficiency is one of several reasons for thrombotic disorders that should be considered in these people, especially if they have a history of DVT or other vascular thromboses.
Aim Systemic sclerosis (SSc) is a rare autoimmune disorder characterized by vascular and fibrosing involvement of the skin and internal organs. In this study we determined the prevalence and characteristics of radiological hands and feet involvements in Iranian SSc patients identified disease–phenotype associations. Methods 43 SSc patients (41 women and 2 men), with a median age of 44.79 years (ranges 26 to 70 years) and a mean disease duration of 11.78 years (ranges 2 to 28 years) were studied in this cross-sectional study. Results 42 patients had radiological changes both in hands and feet. Only one patient had changes just in hand. The most frequent changes that we found in hand was Juxta-articular Osteoporosis (93%), Acro-osteolysis (58.2%), Joint Space Narrowing (55.8%). The prevalence of joint space narrowing or acro-osteolysis were higher in subjects with active skin involvement (modified Rodnan skin score (mRSS) > 14) (16/21 v 4/16 for patients with inactive skin involvement (mRSS < 14); p = 0.002). The most frequent changes that we found in foot were Juxta-articular Osteoporosis (93%), Acro-osteolysis (46.5%), Joint Space Narrowing (58.1%), subluxation (44.2%). The presence of anti-ccp antibody was detected in 4 (9.3%), while positive rheumatoid factor was found in 13 (30.2%) of SSc patients. Conclusion This study corroborates that an arthropathy is common in SSc patients. The introduction of the specific radiological involvements of SSc needs to be confirmed by further studies. in order to define the appropriate prognosis and treatment of patients.
Aim Systemic sclerosis (SSc) is a rare autoimmune disorder characterized by vascular and fibrosing involvement of the skin and internal organs. In this study we determined the prevalence and characteristics of radiological hands and feet involvements in Iranian SSc patients identified disease–phenotype associations. Methods 43 SSc patients (41 women and 2 men), with a median age of 44.79 years (ranges 26 to 70 years) and a mean disease duration of 11.78 years (ranges 2 to 28 years) were studied in this cross-sectional study. Results 42 patients had radiological changes both in hands and feet. Only one patient had changes just in hand. The most frequent changes that we found in hand was Juxta-articular Osteoporosis (93%), Acro-osteolysis (58.2%), Joint Space Narrowing (55.8%). The prevalence of joint space narrowing or acro-osteolysis were higher in subjects with active skin involvement (modified Rodnan skin score (mRSS)>14) (16/21 v 4/16 for patients with inactive skin involvement (mRSS<14); p = 0.002). The most frequent changes that we found in foot were Juxta-articular Osteoporosis (93%), Acro-osteolysis (46.5%), Joint Space Narrowing (58.1%), subluxation (44.2%). The presence of anti-ccp antibody was detected in 4 (9.3%), while positive rheumatoid factor was found in 13 (30.2%) of SSc patients. Conclusion This study corroborates that an arthropathy is common in SSc patients. The introduction of the specific radiological involvements of SSc needs to be confirmed by further studies. in order to define the appropriate prognosis and treatment of patients.
Background and aim: SSc (Systemic sclerosis) is a systemic inflammatory heterogeneous disease, characterized by fibrosis of skin and internal organs particularly the gastrointestinal tract by affecting any part of it. Calprotectin is a calcium- and zinc-binding protein and its level increases during an inflammatory process in the presence of neutrophils, measurable in plasma, urine, saliva, and feces. FC (Fecal calprotectin) is a non-invasive and repeatable marker for the diagnosis of intestinal inflammation and unlike other inflammatory markers, the FC level is not affected by other sources of inflammation other than intestines. The quality of life in SSc patients is severely affected by gastrointestinal disorders. So, we decided to examine FC as a simple and accessible method with no complication to diagnose gastrointestinal disorders and the extent of disease activity.Materials and methods: Totally 46 patients with SSc were invited to include in our study. Seven patients did not enter our study because of having the items in exclusion criteria. For the remaining 39 patients, a questionnaire about gastrointestinal disorders symptoms (UCLA SCTS 2.0) was filled in and then their skin was examined and the skin score was determined. Stool samples were collected to measure the calprotectin level. Barium Swallow and CT Enterography was done to determine gastrointestinal disorders. FC level wasn’t less than 50µgr/gr in any of our cases, therefore the patients was divided into two groups: the one with FC level less than 200 µgr/gr and the one with FC level as 200 µgr/gr or more than 200 µgr/gr and the latter was considered as positive. Chi-Square test was used and to determine the correlation between FC level and age, disease type, qualitative findings in radiography and quantitative variables like skin score.Results: The correlation between FC level and sex was not verifiable cause all patients were female. Increased levels of FC didn’t have any correlation with age (P Value=0.79), disease type (P Value=0.59), skin score (P Value=0.25), final GI score (P Value=0.30), esophageal dilatation (P Value=0.18), small intestine wall thickening (P Value=0.10), and none of serum tests. Also, no signs of micronutrient deficiencies related to FC level was found due to vitamin and mineral supplements consumption in most of our patients.Discussion and Conclusion: Although there was no statistical correlation between FC level and variables, but the results are in favor of FC specificity to assess intestinal wall thickness, which due to the small sample size requires to be assessed in future researchers’ study with a larger sample size.The main cause of the lack of statistical significance in the relationships of our study can be the low sample size. Therefore, it is recommended that in future researchers’ study with a larger sample size.
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