BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare,noninherited disease characterized by gastrointestinal polyposis with diarrhea and ectodermal abnormalities.CCS polyps are distributed through the whole digestive tra...BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare,noninherited disease characterized by gastrointestinal polyposis with diarrhea and ectodermal abnormalities.CCS polyps are distributed through the whole digestive tract,and they are common in the stomach and colon but very uncommon in the esophagus.CASE SUMMARY Here,we present a case of a 63-year-old man with skin hyperpigmentation accompanied by diarrhea,alopecia,and loss of his fingernails.Laboratory data indicated anemia,hypoalbuminemia,hypocalcemia,hypokalemia,and positive fecal occult blood.Endoscopy showed numerous polyps scattered throughout the digestive tract,including the esophagus.He was treated with nutritional support and glucocorticoids with remission of his symptoms.CONCLUSION Comprehensive treatment led by hormonal therapy can result in partial or full remission of clinical symptoms.Treatment should be individualized for each patient according to their therapy response.Surveillance endoscopy is necessary for assessing mucosal disease activity and detecting malignant transformation.展开更多
BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare disease of unknown etiology.The optimal treatment for CCS remains unknown.Treatment with corticosteroids is considered the mainstay treatment because of its high effi...BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare disease of unknown etiology.The optimal treatment for CCS remains unknown.Treatment with corticosteroids is considered the mainstay treatment because of its high efficacy,but the therapeutic strategy for steroid-resistant CCS is not yet established.CASE SUMMARY This is the case of an 81-year-old woman who was diagnosed with CCS.Given her severe diarrhea,nausea,vomiting,and hypoproteinemia,hormone therapy(40 mg/d)was administered,and the symptoms improved within 1 wk.After 3 mo,the patient had no obvious symptoms.The polyps were significantly reduced on review gastroscopy and colonoscopy,thus hormone reduction gradually began.The hormone level was maintained at 10 mg/d after 6 mo.Despite the age of the patient and the side effects of hormones,the patient had no obvious discomfort.However,hormone drugs were discontinued,and mesalazine was administered orally at 3 g/d.The patient's symptoms continued to improve after a follow-up of 5 years.CONCLUSION Corticosteroids and mesalazine are potential treatment options for CCS.展开更多
BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare disease,that causes gastrointestinal polyps,ectodermal abnormalities,and gastrointestinal symptoms.CCS is prone to thromboembolism,but clinical workers have not yet e...BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare disease,that causes gastrointestinal polyps,ectodermal abnormalities,and gastrointestinal symptoms.CCS is prone to thromboembolism,but clinical workers have not yet established a clinical consciousness of preventing thromboembolism.The present case illustrates pulmonary embolism(PE)complicated by CCS.CASE SUMMARY A 46-year-old male patient presented with mucus,purulent,and bloody stool.Ectodermal changes included skin pigmentation,alopecia,and nail dystrophy.Colonoscopy revealed the presence of multiple polyps.After a comprehensive evaluation,the patient was diagnosed with CCS.During the disease,he was also diagnosed with pulmonary embolism,Riehl's melanosis,and intestinal flora imbalance.After symptomatic treatment with omeprazole,mesalazine,rivaroxaban,nutritional support,and regulation of intestinal flora,the patient’s symptoms were significantly relieved.CONCLUSION CCS complicated with PE was reported for the first time in China in this study.Despite the fact that CCS is extremely rare,patients with CCS should be classified as a high-risk venous thromboembolism(VTE)population,and emphasis should be placed on venous thromboembolism risk assessment and stratification,deep venous thromboembolism screening,prevention of VTE,and careful long-term follow-up.展开更多
BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare,non-genetic disorder characterized by multiple gastrointestinal polyps,and ectodermal lesions such as alopecia,fingernail atrophy,and skin mucosal pigmentation.Unfort...BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare,non-genetic disorder characterized by multiple gastrointestinal polyps,and ectodermal lesions such as alopecia,fingernail atrophy,and skin mucosal pigmentation.Unfortunately,the pathogenesis of CCS is currently unknown.CASE SUMMARY Here,we describe the case of an elderly female with diarrhea,fatigue,and hair loss,who experienced abdominal pain for over half a year and was found to have multiple gastrointestinal polyps.She was diagnosed with CCS and was treated with albumin supplementation and prednisone,and her electrolyte imbalance was corrected.Following treatment,her symptoms significantly improved.To elucidate the role of potential genetic events in the pathogenesis of CCS,we performed exome sequencing using an extract of her colorectal adenoma.CONCLUSION Our data revealed multiple somatic mutations and copy number variations.Our findings provide a novel insight into the potential mechanisms of CCS etiology.展开更多
BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare nonhereditary disease characterized by chronic diarrhoea,diffuse gastrointestinal polyposis and ectodermal manifestations.The lethality of CCS can be up to 50%if it i...BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare nonhereditary disease characterized by chronic diarrhoea,diffuse gastrointestinal polyposis and ectodermal manifestations.The lethality of CCS can be up to 50%if it is untreated or if treatment is delayed or inadequate.More than 35%of the patients do not achieve long-term clinical remission after corticosteroid administration,with relapse occurring during or after the cessation of glucocorticoid use.The optimal strategy of maintenance therapy of this disease is controversial.CASE SUMMARY A 47-year-old man presented to the hospital with a 3-mo history of frequent watery diarrhoea,accompanied by macular skin pigmentation that included the palms and soles,and onychodystrophy of the fingernails and toenails.Gastroscopy and colonoscopy revealed numerous polyps in the stomach and colon.After other possibilities were ruled out by a series of examinations,CCS was diagnosed and treated with prednisone.The patient took prednisone for more than 1 year before achieving complete resolution of his symptoms and endoscopic findings.The patient was then given prednisone 5 mg/d for 6 mo of maintenance therapy.With clinical improvement and polyp regression,prednisone was discontinued.Eight mo after the discontinuation of prednisone,the diarrhoea and gastrointestinal polyps relapsed.Therefore,the patient was given the same dose of prednisone,and complete remission was achieved again.CONCLUSION It is necessary to extend the duration of prednisone maintenance therapy for CCS.Prednisone is still effective when readministered after relapse.Surveillance endoscopy at intervals of 1 year or less is recommended to assess mucosal disease activity.展开更多
BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare non-hereditary disease with a poor prognosis and a mortality rate of up to 55%.Currently,there is no standard treatment for CCS.The department of gastroenterology of ...BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare non-hereditary disease with a poor prognosis and a mortality rate of up to 55%.Currently,there is no standard treatment for CCS.The department of gastroenterology of our hospital admitted a patient with CCS whose symptoms improved significantly after treatment with thalidomide combined with endoscopy,and there was no obvious adverse reaction during the 2-year follow-up.CASE SUMMARY A 47-year-old Chinese man presented with diarrhea for more than 4 mo,accompanied by loss of taste,fatigue,and weight loss.Physical examination demonstrated that the patient’s skin and hands were hyperpigmented,the front edges of the nails of both hands were notably thickened and yellow,and the nails were partially atrophied.Gastrointestinal endoscopy identified a diffuse polypoid bulge,and the patient bore an albumin level of 27.3 g/L.The level of the calcium correction amount was(2.164 m M)which allowed for a comprehensive diagnosis of Cronkhite-Canada syndrome,combined with hypoalbuminemia and hypocalcemia.Thalidomide of 150 mg per day was administered to regulate immunity,and the symptoms were relieved after 1 wk.During the follow-up period,polyps were still found that had not been resolved by thalidomide treatment,and endoscopic therapy was performed.This resulted in further improvement of his condition and no particular discomfort during the 2 years of follow-up.CONCLUSION The patient’s symptoms were significantly relieved by thalidomide 2 years after treatment,proposing it as a potential treatment for CCS.展开更多
BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare nonhereditary disease with a syndrome of multiple gastrointestinal polyps,skin pigmentation,hair loss,and fingernail/toenail dystrophy.Intussusception is a serious co...BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare nonhereditary disease with a syndrome of multiple gastrointestinal polyps,skin pigmentation,hair loss,and fingernail/toenail dystrophy.Intussusception is a serious condition with an occurrence rate of 5%in adults,which is mainly caused by intestinal tumors or other intestinal occupations.CASE SUMMARY A 57-year-old woman was admitted to our hospital due to abdominal distension and pain for the past year.Her nausea and vomiting symptoms had been aggravated for the past month.Previous transoral enteroscopy results one year prior showed chronic erosive gastritis protuberans,duodenitis,and jejunitis.She had sparse body hair and brown pigmentation on the skin of her hands and bilateral anterior tibias.The nails of both hands were pale and lacked luster,and the fingernail of her ring finger was longitudinally cracked.Gastroscopy showed extensive diffuse polypoid lump changes in the gastric body and antrum,of 0.5-3 cm in size.Colonoscopy showed multiple polypoid mucosal bulges in the terminal ileum and multiple polyps(0.3-5 cm)throughout the colon.The patient was diagnosed with CCS and underwent partial excision of the polyps,but she refused hormone therapy.One month later,the patient complained of nausea and vomiting,accompanied by abdominal pain and inability to pass gas or stool.Contrast-enhanced computed tomography of the abdomen showed gastrointestinal polyposis and ileocecal intussusception.She underwent stomach and bowel surgery.CONCLUSION CCS,as a rare disease with poor prognosis,should be treated aggressively.Systematic steroids,immunosuppressive agents,and biological agents were not applied;thus,the patient’s symptoms quickly progressed,and intussusception occurred.She had to undergo surgery.Improved compliance may lead to a better prognosis.展开更多
BACKGROUND Gastrointestinal(GI)polyposis is a rare condition in GI diseases.To date about 500 cases of Cronkhite-Canada syndrome(CCS)have been reported worldwide.CASE SUMMARY We report a 60-year-old female patient who...BACKGROUND Gastrointestinal(GI)polyposis is a rare condition in GI diseases.To date about 500 cases of Cronkhite-Canada syndrome(CCS)have been reported worldwide.CASE SUMMARY We report a 60-year-old female patient who presented with dyspepsia,abdominal pain,and weight loss of 1-year duration.Her physical examination showed alopecia and onychodystrophy.Upper endoscopy revealed diffuse markedly thickened gastric mucosa involving the whole stomach with thickened gastric rugae and numerous polypoidal lesions.Histopathological examination showed marked hyperplasia of the foveolar glands with inflammatory cell infiltration.Endoscopic ultrasound showed a significantly hypertrophic mucosa and muscularis mucosa,while the submucosa and the muscularis propria were spared,favouring its benign nature.Colonoscopy showed multiple sessile polyps scattered at different parts of the colon.Histopathological examination revealed tubular adenomatous polyps with low-grade dysplasia.Differential diagnoses included CCS,Menterier disease(MD),other polyposis syndromes,lymphoma,amyloidosis,and gastric malignancies.The presence of alopecia,nail dystrophy,GI polyposis,markedly thickened gastric mucosa and folds,abdominal pain,weight loss,and marked foveolar gland hyperplasia;all was in favour of CCS.Lymphoma was excluded due to sparing of the muscularis propria.The presence of colonic polyps and antral and duodenal infiltration,and the absence of hypoproteinaemia decreased the possibility for MD.CONCLUSION The patient was diagnosed as having CCS.展开更多
Cronkhite-Canada syndrome(CCS)is a rare nonfamilial polyposis syndrome characterized by epithelial disturbances in the gastrointestinal tract and skin.The aim of this study was to investigate the clinical features and...Cronkhite-Canada syndrome(CCS)is a rare nonfamilial polyposis syndrome characterized by epithelial disturbances in the gastrointestinal tract and skin.The aim of this study was to investigate the clinical features and potential therapies for CCS.Six patients with CCS admitted from December 1992 to July 2008 to Peking Union Medical College Hospital were evaluated.All patients had clinical manifestation of nonhereditary gastrointestinal polyposis with diarrhea,skin hyperpigmentation,alopecia,and nail dystrophy.Fecal occult blood was positive in all six cases.Serum hemoglobin,potassium,calcium and protein were below the normal range in two cases.Anti-Saccharomyces cerevisiae and antinuclear antibodies were present in three cases.Multiple polyps were found in all patients by gastroscopy and colonoscopy,with only one in the esophagus.Histologically,there were hyperplastic polyps in five cases,tubular adenoma in three,and juvenile polyp in one with chronic inflammation and mucosal edema.Comprehensive treatment led by corticosteroids can result in partial remission of clinical symptoms,and longterm follow-up is necessary.展开更多
Cronkhite-Canada syndrome(CCS) is a rare but serious protein-losing enteropathy, but little is known about the mechanism. Further more, misdiagnosis is common due to non-familiarity of its clinical manifestation. A 40...Cronkhite-Canada syndrome(CCS) is a rare but serious protein-losing enteropathy, but little is known about the mechanism. Further more, misdiagnosis is common due to non-familiarity of its clinical manifestation. A 40-year-old male patient was admitted to our hospital because of diarrhea and hypogeusia associated with weight loss for 4 mo. On physical examination, skin pigmentation, dystrophic nail changes and alopecia were noted. He had no alike family history. Laboratory results revealed low levels of serum albumin(30.1 g/L, range: 35.0-55.0 g/L), serum potassium(2.61 mmol/L, range: 3.5-5.5 mmol/L) and blood glucose(2.6 mmol/L, range: 3.9-6.1 mmol/L). The erythrocyte sedimentation rate was elevated to 17 mm/h(range: 0-15 mm/h). X-ray of chest and mandible was normal. The endoscopic examination showed multiple sessile polyps in the stomach, small bowel and colorectum. Histopathologic examination of biopsies obtained from those polyps showed hyperplastic change, cystic dilatation and distortion of glands with inflammatory infiltration, eosinophilic predominance and stromal edema. Immune staining for IgG 4 plasma cells was positive in polyps of stomach and colon. The patient was diagnosed of CCS and treated with steroid, he had a good response to steroid. Both histologic findings and treatment response to steroid suggested an autoimmune mechanism underling CCS.展开更多
Cronkhite-Canada syndrome (CCS) is a rare, non-inherited polyposis syndrome, characterized by diffuse gastrointestinal (GI) hamartomatous polyposis with unique dermatologic changes including alopecia, skin hyperpigmen...Cronkhite-Canada syndrome (CCS) is a rare, non-inherited polyposis syndrome, characterized by diffuse gastrointestinal (GI) hamartomatous polyposis with unique dermatologic changes including alopecia, skin hyperpigmentation, and nail dystrophy. Patients can typically present with diarrhea, weight loss, protein-losing enteropathy, and nutritional deficiency. However, it can demonstrate diverse other clinical features, usually with poor prognosis. Currently, there are no specific diagnostic criteria established yet. The etiology of CCS is still obscure, but an autoimmune process has been suggested. Here we present an 81-year-old Caucasian female who had clinical presentations, physical exam, imaging, endoscopy and pathology findings that were all consistent with the diagnosis of CCS. We also include a detailed literature review of the other gastrointestinal polyposis syndromes (hamartomatous, adenomatous, hyperplastic and inflammatory polyposis). A high index of suspicion and recognition of the characteristic clinical, endoscopic as well as histopathological findings of CCS, as well as different gastrointestinal polyposis conditions, can help clinicians with more timely and correct diagnosis.展开更多
We report a case of Cronkhite-Canada syndrome(CCS)associated with myelodysplastic syndrome(MDS).A 54-year-old woman,diagnosed as MDS the prior year after evaluation of anemia,visited our hospital with the chief compla...We report a case of Cronkhite-Canada syndrome(CCS)associated with myelodysplastic syndrome(MDS).A 54-year-old woman,diagnosed as MDS the prior year after evaluation of anemia,visited our hospital with the chief complaint of epigastric discomfort.She also had dysgeusia,alopecia,atrophic nail change,and pigmentation of the palm,all of which began several months ago.Blood tests revealed severe hypoalbuminemia.Colonoscopy(CS)showed numerous,dense,red polyps throughout the colon and rectum.Biopsy specimens showed stromal edema,infi ltration of lymphocytes,and cystic dilatation of the crypt.Her clinical manifestations and histology were consistent with CCS.We prescribed corticosteroids,which dramatically improved her physical findings,laboratory data,and endoscopic fi ndings.This is the first report of CCS in a patient with MDS.展开更多
BACKGROUND In recent years,the prevalence of obesity and metabolic syndrome in type 1 diabetes(T1DM)patients has gradually increased.Insulin resistance in T1DM deserves attention.It is necessary to clarify the relatio...BACKGROUND In recent years,the prevalence of obesity and metabolic syndrome in type 1 diabetes(T1DM)patients has gradually increased.Insulin resistance in T1DM deserves attention.It is necessary to clarify the relationship between body composition,metabolic syndrome and insulin resistance in T1DM to guide clinical treatment and intervention.AIM To assess body composition(BC)in T1DM patients and evaluate the relationship between BC,metabolic syndrome(MS),and insulin resistance in these indi-viduals.METHODS A total of 101 subjects with T1DM,aged 10 years or older,and with a disease duration of over 1 year were included.Bioelectrical impedance analysis using the Tsinghua-Tongfang BC Analyzer BCA-1B was employed to measure various BC parameters.Clinical and laboratory data were collected,and insulin resistance was calculated using the estimated glucose disposal rate(eGDR).RESULTS MS was diagnosed in 16/101 patients(15.84%),overweight in 16/101 patients(15.84%),obesity in 4/101(3.96%),hypertension in 34/101(33.66%%)and dyslip-idemia in 16/101 patients(15.84%).Visceral fat index(VFI)and trunk fat mass were significantly and negatively correlated with eGDR(both P<0.001).Female patients exhibited higher body fat percentage and visceral fat ratio compared to male patients.Binary logistic regression analysis revealed that significant factors for MS included eGDR[P=0.017,odds ratio(OR)=0.109],VFI(P=0.030,OR=3.529),and a family history of diabetes(P=0.004,OR=0.228).Significant factors for hypertension included eGDR(P<0.001,OR=0.488)and skeletal muscle mass(P=0.003,OR=1.111).Significant factors for dyslipidemia included trunk fat mass(P=0.033,OR=1.202)and eGDR(P=0.037,OR=0.708).CONCLUSION Visceral fat was found to be a superior predictor of MS compared to conventional measures such as body mass index and waist-to-hip ratio in Chinese individuals with T1DM.BC analysis,specifically identifying visceral fat(trunk fat),may play an important role in identifying the increased risk of MS in non-obese patients with T1DM.展开更多
BACKGROUND Superior mesenteric artery(SMA)syndrome is a rare cause of duodenal obstruction by extrinsic compression between the SMA and the aorta(SMA-Ao).Although the left lateral recumbent position is considered effe...BACKGROUND Superior mesenteric artery(SMA)syndrome is a rare cause of duodenal obstruction by extrinsic compression between the SMA and the aorta(SMA-Ao).Although the left lateral recumbent position is considered effective in the treatment of SMA syndrome,individual variations in the optimal patient position have been noted.In this report,we present two elderly cases of SMA syndrome that exhibited rapid recovery due to ultrasonographic dynamic evaluation of the optimal position for each patient.CASE SUMMARY Case 1:A 90-year-old man with nausea and vomiting.Following diagnosis of SMA syndrome by computed tomography(CT),ultrasonography(US)revealed the SMA-Ao distance in the supine position(4 mm),which slightly improved in the lateral position(5.7–7.0 mm)without the passage of duodenal contents.However,in the sitting position,the SMA-Ao distance was increased to 15 mm accompanied by improved content passage.Additionally,US indicated enhanced passage upon abdominal massage on the right side.By day 2,the patient could eat comfortably with the optimal position and massage.Case 2:An 87-year-old woman with vomiting.After the diagnosis of SMA syndrome and aspiration pneumonia by CT,dynamic US confirmed the optimal position(SMA-Ao distance was improved to 7 mm in forward-bent position,whereas it remained at 5 mm in the supine position).By day 7 when her pneumonia recovered,she could eat with the optimal position.CONCLUSION The optimal position for SMA syndrome varies among individuals.Dynamic US appears to be a valuable tool in improving patient outcomes.展开更多
Emerging evidence and perspectives have pointed towards the heart playing an important role in hepatorenal syndrome(HRS),outside of conventional understanding that liver cirrhosis is traditionally considered the sole ...Emerging evidence and perspectives have pointed towards the heart playing an important role in hepatorenal syndrome(HRS),outside of conventional understanding that liver cirrhosis is traditionally considered the sole origin of a cascade of pathophysiological mechanisms directly affecting the kidneys in this context.In the absence of established heart disease,cirrhotic cardiomyopathy may occur more frequently in those with liver cirrhosis and kidney disease.It is a specific form of cardiac dysfunction characterized by blunted contractile responsiveness to stress stimuli and altered diastolic relaxation with electrophysiological abnormalities.Despite the clinical description of these potential cardiac-related complications of the liver,the role of the heart has traditionally been an overlooked aspect of circulatory dysfunction in HRS.Yet from a physiological sense,temporality(prior onset)of cardiorenal interactions in HRS and positive effects stemming from portosystemic shunting demonstrated an important role of the heart in the development and progression of kidney dysfunction in cirrhotic patients.In this review,we discuss current concepts surrounding how the heart may influence the development and progression of HRS,and the role of systemic inflammation and endothelial dysfunction causing circulatory dysfunction within this setting.The temporality of heart and kidney dysfunction in HRS will be discussed.For a subgroup of patients who receive portosystemic shunting,the dynamics of cardiorenal interactions following treatment is reviewed.Continued research to determine the unknowns in this topic is anticipated,hopefully to further clarify the intricacies surrounding the liver-heart-kidney connection and improve strategies for management.展开更多
Fraser syndrome is a rare malformative genetic syndrome whose main manifestations are cryptophthalmia, syndactyly, laryngeal atresia and urogenital malformations. We report the observation of a newborn from a non-cons...Fraser syndrome is a rare malformative genetic syndrome whose main manifestations are cryptophthalmia, syndactyly, laryngeal atresia and urogenital malformations. We report the observation of a newborn from a non-consanguineous marriage, admitted to the neonatology and neonatal intensive care unit at Day 1 of life for a poly-malformative syndrome. Clinically, the newborn presented with bilateral anophthalmia, cleft palate, dysmorphic facies with a rounded forehead, hypertelorism, micrognathia, low-set ears and a short neck, syndactility and bilateral cryptorchidism. Trans fontanellar ultrasound revealed tri ventricular hydrocephalus. Cerebral MRI angiography showed malformative tri-ventricular hydrocephalus, hypoplasia of the brainstem and cerebellum, and poly-microgyria. Transthoracic and renal ultrasonography were unremarkable, and the chest X-ray was normal. The authors discuss the malformative clinical and para-clinical aspects of this syndrome, multidisciplinary management and the importance of prenatal diagnosis.展开更多
Lynch syndrome is the fourth most common cancer in the United States, with an early age of onset and poor prognosis. Here, we present a unique case of a patient with progressive colon cancer due to a late diagnosis of...Lynch syndrome is the fourth most common cancer in the United States, with an early age of onset and poor prognosis. Here, we present a unique case of a patient with progressive colon cancer due to a late diagnosis of Lynch syndrome showing excellent response to immunotherapy. A 59-year-old male with a history of rectal cancer 30 years ago came to the hospital due to a fever and further found a large necrotic colon mass. Biopsy was positive for colorectal cancer;however, due to the size of the tumor, the patient was deemed not a surgical candidate and offered hospice with palliative chemotherapy. Based on further workup, the patient was diagnosed with Lynch syndrome, with colon cancer determined to be responsive to Immunotherapy. He was started on JEMPERLI (Dosterlimab-gxly), and after three cycles of therapy, imaging and PET scan were repeated, showing decreased activity and extent of the tumor—a tremendous success.展开更多
Background: Adherence to medications is dependent upon a variety of factors, including individual characteristics of the patient, interactions with health care providers, and medication complexity. Even though several...Background: Adherence to medications is dependent upon a variety of factors, including individual characteristics of the patient, interactions with health care providers, and medication complexity. Even though several studies were conducted to test intervention strategies, results are uncertain. Aim: The aim of the study is to assess if a tailored combined intervention strategy improves medication adherence in a large population of post-menopausal women affected by hypertension or metabolic syndrome. Methods: We enrolled 6833 patients aged 50 to 69 years, 85.7% with hypertension, and 14.3% with metabolic syndrome. A network between patients, general practitioners, and cardiologists was established. Interventions included education, adequate information to patients, a simplified scheme of treatment, and periodic adherence assessment. These were either delivered as healthcare provider supports or using modern technology. Medication adherence was estimated by the proportion of days covered for all classes of drugs after the index date. Results: Non-adherent hypertensive women were 297 (5%), and those with metabolic syndrome were 73 (7.4%) (p Conclusions: The rate of non-adherence in both settings of postmenopausal women was 7.7%, much lower than that described in the literature. This rate was increased in patients with metabolic syndrome;probably it is related to the complexity of the therapeutic scheme or to a poor consciousness of the disease. Therefore, implementing a tailored combined intervention can improve significantly patients’ adherence to medical therapy.展开更多
Introduction: Cardiac catheterisation plays a fundamental role in the management of acute coronary syndrome. These explorations require heavy, complex and costly equipment and a large team of doctors, nurses and techn...Introduction: Cardiac catheterisation plays a fundamental role in the management of acute coronary syndrome. These explorations require heavy, complex and costly equipment and a large team of doctors, nurses and technicians with highly specialized training. Aims: To describe epidemiological, clinical and coronary angiography aspects of patients with acute coronary syndrome. Patients and Methods: Descriptive study from September 2019 to December 2023 in the Cardiology Department of the Hôpital Mère-Enfant of Bamako. Inclusion criteria were patients admitted for coronary angiography with the diagnosis of acute coronary syndrome. Results: During the study period, 1253 patients underwent coronary angiography, 596 of whom had acute coronary syndrome as an indication, representing a hospital frequency of 47%. Sex-ratio was 2.10. Mean age of patients was 58.5 ± 11.39 years. ST elevation acute coronary syndrome was the most common indication with 63.92% of cases. High blood pressure was the main cardiovascular risk factor with 58.7% of cases, and radial access approach was used in 98% of cases. Coronary angiography was pathological in 91.70% of cases (n = 548). Patients with lesions of anterior interventricular artery were 73.73% of cases. Tritruncal lesions accounted for 40.63% of cases. Conclusion: ST elevation acute coronary syndrome is the most frequent manifestation of acute coronary syndrome. Anterior interventricular artery is most often the culprit lesion for our patients.展开更多
BACKGROUND This study presents a case of rapidly developing respiratory failure due to antisynthetase syndrome(AS)following coronavirus disease 2019(COVID-19)in a 33-year-old man diagnosed with Klinefelter syndrome(KS...BACKGROUND This study presents a case of rapidly developing respiratory failure due to antisynthetase syndrome(AS)following coronavirus disease 2019(COVID-19)in a 33-year-old man diagnosed with Klinefelter syndrome(KS).CASE SUMMARY A 33-year-old man with a diagnosis of KS was admitted to the Department of Pulmonary and Critical Care Medicine of a tertiary hospital in China for fever and shortness of breath 2 wk after the onset of COVID-19.Computed tomography of both lungs revealed diffuse multiple patchy heightened shadows in both lungs,accompanied by signs of partial bronchial inflation.Metagenomic next-generation sequencing of the bronchoalveolar lavage fluid suggested absence of pathogen.A biopsy specimen revealed organizing pneumonia with alveolar septal thickening.Additionally,extensive auto-antibody tests showed strong positivity for anti-SSA,anti-SSB,anti-Jo-1,and anti-Ro-52.Following multidisciplinary discussions,the patient received a final diagnosis of AS,leading to rapidly progressing respiratory failure.CONCLUSION This study underscores the clinical progression of AS-associated interstitial lung disease subsequent to viral infections such as COVID-19 in patients diagnosed with KS.展开更多
基金the Medical Health Science and Technology Project of Zhejiang Province,No.2022KY1109the Natural Science Foundation of Ningbo,No.2022J204Ningbo Key Clinical Specialty(Endocrinology),No.2022-B07.
文摘BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare,noninherited disease characterized by gastrointestinal polyposis with diarrhea and ectodermal abnormalities.CCS polyps are distributed through the whole digestive tract,and they are common in the stomach and colon but very uncommon in the esophagus.CASE SUMMARY Here,we present a case of a 63-year-old man with skin hyperpigmentation accompanied by diarrhea,alopecia,and loss of his fingernails.Laboratory data indicated anemia,hypoalbuminemia,hypocalcemia,hypokalemia,and positive fecal occult blood.Endoscopy showed numerous polyps scattered throughout the digestive tract,including the esophagus.He was treated with nutritional support and glucocorticoids with remission of his symptoms.CONCLUSION Comprehensive treatment led by hormonal therapy can result in partial or full remission of clinical symptoms.Treatment should be individualized for each patient according to their therapy response.Surveillance endoscopy is necessary for assessing mucosal disease activity and detecting malignant transformation.
文摘BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare disease of unknown etiology.The optimal treatment for CCS remains unknown.Treatment with corticosteroids is considered the mainstay treatment because of its high efficacy,but the therapeutic strategy for steroid-resistant CCS is not yet established.CASE SUMMARY This is the case of an 81-year-old woman who was diagnosed with CCS.Given her severe diarrhea,nausea,vomiting,and hypoproteinemia,hormone therapy(40 mg/d)was administered,and the symptoms improved within 1 wk.After 3 mo,the patient had no obvious symptoms.The polyps were significantly reduced on review gastroscopy and colonoscopy,thus hormone reduction gradually began.The hormone level was maintained at 10 mg/d after 6 mo.Despite the age of the patient and the side effects of hormones,the patient had no obvious discomfort.However,hormone drugs were discontinued,and mesalazine was administered orally at 3 g/d.The patient's symptoms continued to improve after a follow-up of 5 years.CONCLUSION Corticosteroids and mesalazine are potential treatment options for CCS.
文摘BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare disease,that causes gastrointestinal polyps,ectodermal abnormalities,and gastrointestinal symptoms.CCS is prone to thromboembolism,but clinical workers have not yet established a clinical consciousness of preventing thromboembolism.The present case illustrates pulmonary embolism(PE)complicated by CCS.CASE SUMMARY A 46-year-old male patient presented with mucus,purulent,and bloody stool.Ectodermal changes included skin pigmentation,alopecia,and nail dystrophy.Colonoscopy revealed the presence of multiple polyps.After a comprehensive evaluation,the patient was diagnosed with CCS.During the disease,he was also diagnosed with pulmonary embolism,Riehl's melanosis,and intestinal flora imbalance.After symptomatic treatment with omeprazole,mesalazine,rivaroxaban,nutritional support,and regulation of intestinal flora,the patient’s symptoms were significantly relieved.CONCLUSION CCS complicated with PE was reported for the first time in China in this study.Despite the fact that CCS is extremely rare,patients with CCS should be classified as a high-risk venous thromboembolism(VTE)population,and emphasis should be placed on venous thromboembolism risk assessment and stratification,deep venous thromboembolism screening,prevention of VTE,and careful long-term follow-up.
文摘BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare,non-genetic disorder characterized by multiple gastrointestinal polyps,and ectodermal lesions such as alopecia,fingernail atrophy,and skin mucosal pigmentation.Unfortunately,the pathogenesis of CCS is currently unknown.CASE SUMMARY Here,we describe the case of an elderly female with diarrhea,fatigue,and hair loss,who experienced abdominal pain for over half a year and was found to have multiple gastrointestinal polyps.She was diagnosed with CCS and was treated with albumin supplementation and prednisone,and her electrolyte imbalance was corrected.Following treatment,her symptoms significantly improved.To elucidate the role of potential genetic events in the pathogenesis of CCS,we performed exome sequencing using an extract of her colorectal adenoma.CONCLUSION Our data revealed multiple somatic mutations and copy number variations.Our findings provide a novel insight into the potential mechanisms of CCS etiology.
文摘BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare nonhereditary disease characterized by chronic diarrhoea,diffuse gastrointestinal polyposis and ectodermal manifestations.The lethality of CCS can be up to 50%if it is untreated or if treatment is delayed or inadequate.More than 35%of the patients do not achieve long-term clinical remission after corticosteroid administration,with relapse occurring during or after the cessation of glucocorticoid use.The optimal strategy of maintenance therapy of this disease is controversial.CASE SUMMARY A 47-year-old man presented to the hospital with a 3-mo history of frequent watery diarrhoea,accompanied by macular skin pigmentation that included the palms and soles,and onychodystrophy of the fingernails and toenails.Gastroscopy and colonoscopy revealed numerous polyps in the stomach and colon.After other possibilities were ruled out by a series of examinations,CCS was diagnosed and treated with prednisone.The patient took prednisone for more than 1 year before achieving complete resolution of his symptoms and endoscopic findings.The patient was then given prednisone 5 mg/d for 6 mo of maintenance therapy.With clinical improvement and polyp regression,prednisone was discontinued.Eight mo after the discontinuation of prednisone,the diarrhoea and gastrointestinal polyps relapsed.Therefore,the patient was given the same dose of prednisone,and complete remission was achieved again.CONCLUSION It is necessary to extend the duration of prednisone maintenance therapy for CCS.Prednisone is still effective when readministered after relapse.Surveillance endoscopy at intervals of 1 year or less is recommended to assess mucosal disease activity.
基金Supported by National Natural Science Foundation of China,No.82170550。
文摘BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare non-hereditary disease with a poor prognosis and a mortality rate of up to 55%.Currently,there is no standard treatment for CCS.The department of gastroenterology of our hospital admitted a patient with CCS whose symptoms improved significantly after treatment with thalidomide combined with endoscopy,and there was no obvious adverse reaction during the 2-year follow-up.CASE SUMMARY A 47-year-old Chinese man presented with diarrhea for more than 4 mo,accompanied by loss of taste,fatigue,and weight loss.Physical examination demonstrated that the patient’s skin and hands were hyperpigmented,the front edges of the nails of both hands were notably thickened and yellow,and the nails were partially atrophied.Gastrointestinal endoscopy identified a diffuse polypoid bulge,and the patient bore an albumin level of 27.3 g/L.The level of the calcium correction amount was(2.164 m M)which allowed for a comprehensive diagnosis of Cronkhite-Canada syndrome,combined with hypoalbuminemia and hypocalcemia.Thalidomide of 150 mg per day was administered to regulate immunity,and the symptoms were relieved after 1 wk.During the follow-up period,polyps were still found that had not been resolved by thalidomide treatment,and endoscopic therapy was performed.This resulted in further improvement of his condition and no particular discomfort during the 2 years of follow-up.CONCLUSION The patient’s symptoms were significantly relieved by thalidomide 2 years after treatment,proposing it as a potential treatment for CCS.
基金The Medical Health Science and Technology Project of Zhejiang Provincial Health Commission,No.2021436506General Scientific Research Project of Zhejiang Science and Technology Department,No.Y202044280.
文摘BACKGROUND Cronkhite-Canada syndrome(CCS)is a rare nonhereditary disease with a syndrome of multiple gastrointestinal polyps,skin pigmentation,hair loss,and fingernail/toenail dystrophy.Intussusception is a serious condition with an occurrence rate of 5%in adults,which is mainly caused by intestinal tumors or other intestinal occupations.CASE SUMMARY A 57-year-old woman was admitted to our hospital due to abdominal distension and pain for the past year.Her nausea and vomiting symptoms had been aggravated for the past month.Previous transoral enteroscopy results one year prior showed chronic erosive gastritis protuberans,duodenitis,and jejunitis.She had sparse body hair and brown pigmentation on the skin of her hands and bilateral anterior tibias.The nails of both hands were pale and lacked luster,and the fingernail of her ring finger was longitudinally cracked.Gastroscopy showed extensive diffuse polypoid lump changes in the gastric body and antrum,of 0.5-3 cm in size.Colonoscopy showed multiple polypoid mucosal bulges in the terminal ileum and multiple polyps(0.3-5 cm)throughout the colon.The patient was diagnosed with CCS and underwent partial excision of the polyps,but she refused hormone therapy.One month later,the patient complained of nausea and vomiting,accompanied by abdominal pain and inability to pass gas or stool.Contrast-enhanced computed tomography of the abdomen showed gastrointestinal polyposis and ileocecal intussusception.She underwent stomach and bowel surgery.CONCLUSION CCS,as a rare disease with poor prognosis,should be treated aggressively.Systematic steroids,immunosuppressive agents,and biological agents were not applied;thus,the patient’s symptoms quickly progressed,and intussusception occurred.She had to undergo surgery.Improved compliance may lead to a better prognosis.
文摘BACKGROUND Gastrointestinal(GI)polyposis is a rare condition in GI diseases.To date about 500 cases of Cronkhite-Canada syndrome(CCS)have been reported worldwide.CASE SUMMARY We report a 60-year-old female patient who presented with dyspepsia,abdominal pain,and weight loss of 1-year duration.Her physical examination showed alopecia and onychodystrophy.Upper endoscopy revealed diffuse markedly thickened gastric mucosa involving the whole stomach with thickened gastric rugae and numerous polypoidal lesions.Histopathological examination showed marked hyperplasia of the foveolar glands with inflammatory cell infiltration.Endoscopic ultrasound showed a significantly hypertrophic mucosa and muscularis mucosa,while the submucosa and the muscularis propria were spared,favouring its benign nature.Colonoscopy showed multiple sessile polyps scattered at different parts of the colon.Histopathological examination revealed tubular adenomatous polyps with low-grade dysplasia.Differential diagnoses included CCS,Menterier disease(MD),other polyposis syndromes,lymphoma,amyloidosis,and gastric malignancies.The presence of alopecia,nail dystrophy,GI polyposis,markedly thickened gastric mucosa and folds,abdominal pain,weight loss,and marked foveolar gland hyperplasia;all was in favour of CCS.Lymphoma was excluded due to sparing of the muscularis propria.The presence of colonic polyps and antral and duodenal infiltration,and the absence of hypoproteinaemia decreased the possibility for MD.CONCLUSION The patient was diagnosed as having CCS.
文摘Cronkhite-Canada syndrome(CCS)is a rare nonfamilial polyposis syndrome characterized by epithelial disturbances in the gastrointestinal tract and skin.The aim of this study was to investigate the clinical features and potential therapies for CCS.Six patients with CCS admitted from December 1992 to July 2008 to Peking Union Medical College Hospital were evaluated.All patients had clinical manifestation of nonhereditary gastrointestinal polyposis with diarrhea,skin hyperpigmentation,alopecia,and nail dystrophy.Fecal occult blood was positive in all six cases.Serum hemoglobin,potassium,calcium and protein were below the normal range in two cases.Anti-Saccharomyces cerevisiae and antinuclear antibodies were present in three cases.Multiple polyps were found in all patients by gastroscopy and colonoscopy,with only one in the esophagus.Histologically,there were hyperplastic polyps in five cases,tubular adenoma in three,and juvenile polyp in one with chronic inflammation and mucosal edema.Comprehensive treatment led by corticosteroids can result in partial remission of clinical symptoms,and longterm follow-up is necessary.
文摘Cronkhite-Canada syndrome(CCS) is a rare but serious protein-losing enteropathy, but little is known about the mechanism. Further more, misdiagnosis is common due to non-familiarity of its clinical manifestation. A 40-year-old male patient was admitted to our hospital because of diarrhea and hypogeusia associated with weight loss for 4 mo. On physical examination, skin pigmentation, dystrophic nail changes and alopecia were noted. He had no alike family history. Laboratory results revealed low levels of serum albumin(30.1 g/L, range: 35.0-55.0 g/L), serum potassium(2.61 mmol/L, range: 3.5-5.5 mmol/L) and blood glucose(2.6 mmol/L, range: 3.9-6.1 mmol/L). The erythrocyte sedimentation rate was elevated to 17 mm/h(range: 0-15 mm/h). X-ray of chest and mandible was normal. The endoscopic examination showed multiple sessile polyps in the stomach, small bowel and colorectum. Histopathologic examination of biopsies obtained from those polyps showed hyperplastic change, cystic dilatation and distortion of glands with inflammatory infiltration, eosinophilic predominance and stromal edema. Immune staining for IgG 4 plasma cells was positive in polyps of stomach and colon. The patient was diagnosed of CCS and treated with steroid, he had a good response to steroid. Both histologic findings and treatment response to steroid suggested an autoimmune mechanism underling CCS.
文摘Cronkhite-Canada syndrome (CCS) is a rare, non-inherited polyposis syndrome, characterized by diffuse gastrointestinal (GI) hamartomatous polyposis with unique dermatologic changes including alopecia, skin hyperpigmentation, and nail dystrophy. Patients can typically present with diarrhea, weight loss, protein-losing enteropathy, and nutritional deficiency. However, it can demonstrate diverse other clinical features, usually with poor prognosis. Currently, there are no specific diagnostic criteria established yet. The etiology of CCS is still obscure, but an autoimmune process has been suggested. Here we present an 81-year-old Caucasian female who had clinical presentations, physical exam, imaging, endoscopy and pathology findings that were all consistent with the diagnosis of CCS. We also include a detailed literature review of the other gastrointestinal polyposis syndromes (hamartomatous, adenomatous, hyperplastic and inflammatory polyposis). A high index of suspicion and recognition of the characteristic clinical, endoscopic as well as histopathological findings of CCS, as well as different gastrointestinal polyposis conditions, can help clinicians with more timely and correct diagnosis.
文摘We report a case of Cronkhite-Canada syndrome(CCS)associated with myelodysplastic syndrome(MDS).A 54-year-old woman,diagnosed as MDS the prior year after evaluation of anemia,visited our hospital with the chief complaint of epigastric discomfort.She also had dysgeusia,alopecia,atrophic nail change,and pigmentation of the palm,all of which began several months ago.Blood tests revealed severe hypoalbuminemia.Colonoscopy(CS)showed numerous,dense,red polyps throughout the colon and rectum.Biopsy specimens showed stromal edema,infi ltration of lymphocytes,and cystic dilatation of the crypt.Her clinical manifestations and histology were consistent with CCS.We prescribed corticosteroids,which dramatically improved her physical findings,laboratory data,and endoscopic fi ndings.This is the first report of CCS in a patient with MDS.
基金Supported by the“SDF-sweet doctor cultivation”Project of Sinocare Diabetes Foundation,No.2022SD11 and No.2021SD09.
文摘BACKGROUND In recent years,the prevalence of obesity and metabolic syndrome in type 1 diabetes(T1DM)patients has gradually increased.Insulin resistance in T1DM deserves attention.It is necessary to clarify the relationship between body composition,metabolic syndrome and insulin resistance in T1DM to guide clinical treatment and intervention.AIM To assess body composition(BC)in T1DM patients and evaluate the relationship between BC,metabolic syndrome(MS),and insulin resistance in these indi-viduals.METHODS A total of 101 subjects with T1DM,aged 10 years or older,and with a disease duration of over 1 year were included.Bioelectrical impedance analysis using the Tsinghua-Tongfang BC Analyzer BCA-1B was employed to measure various BC parameters.Clinical and laboratory data were collected,and insulin resistance was calculated using the estimated glucose disposal rate(eGDR).RESULTS MS was diagnosed in 16/101 patients(15.84%),overweight in 16/101 patients(15.84%),obesity in 4/101(3.96%),hypertension in 34/101(33.66%%)and dyslip-idemia in 16/101 patients(15.84%).Visceral fat index(VFI)and trunk fat mass were significantly and negatively correlated with eGDR(both P<0.001).Female patients exhibited higher body fat percentage and visceral fat ratio compared to male patients.Binary logistic regression analysis revealed that significant factors for MS included eGDR[P=0.017,odds ratio(OR)=0.109],VFI(P=0.030,OR=3.529),and a family history of diabetes(P=0.004,OR=0.228).Significant factors for hypertension included eGDR(P<0.001,OR=0.488)and skeletal muscle mass(P=0.003,OR=1.111).Significant factors for dyslipidemia included trunk fat mass(P=0.033,OR=1.202)and eGDR(P=0.037,OR=0.708).CONCLUSION Visceral fat was found to be a superior predictor of MS compared to conventional measures such as body mass index and waist-to-hip ratio in Chinese individuals with T1DM.BC analysis,specifically identifying visceral fat(trunk fat),may play an important role in identifying the increased risk of MS in non-obese patients with T1DM.
文摘BACKGROUND Superior mesenteric artery(SMA)syndrome is a rare cause of duodenal obstruction by extrinsic compression between the SMA and the aorta(SMA-Ao).Although the left lateral recumbent position is considered effective in the treatment of SMA syndrome,individual variations in the optimal patient position have been noted.In this report,we present two elderly cases of SMA syndrome that exhibited rapid recovery due to ultrasonographic dynamic evaluation of the optimal position for each patient.CASE SUMMARY Case 1:A 90-year-old man with nausea and vomiting.Following diagnosis of SMA syndrome by computed tomography(CT),ultrasonography(US)revealed the SMA-Ao distance in the supine position(4 mm),which slightly improved in the lateral position(5.7–7.0 mm)without the passage of duodenal contents.However,in the sitting position,the SMA-Ao distance was increased to 15 mm accompanied by improved content passage.Additionally,US indicated enhanced passage upon abdominal massage on the right side.By day 2,the patient could eat comfortably with the optimal position and massage.Case 2:An 87-year-old woman with vomiting.After the diagnosis of SMA syndrome and aspiration pneumonia by CT,dynamic US confirmed the optimal position(SMA-Ao distance was improved to 7 mm in forward-bent position,whereas it remained at 5 mm in the supine position).By day 7 when her pneumonia recovered,she could eat with the optimal position.CONCLUSION The optimal position for SMA syndrome varies among individuals.Dynamic US appears to be a valuable tool in improving patient outcomes.
文摘Emerging evidence and perspectives have pointed towards the heart playing an important role in hepatorenal syndrome(HRS),outside of conventional understanding that liver cirrhosis is traditionally considered the sole origin of a cascade of pathophysiological mechanisms directly affecting the kidneys in this context.In the absence of established heart disease,cirrhotic cardiomyopathy may occur more frequently in those with liver cirrhosis and kidney disease.It is a specific form of cardiac dysfunction characterized by blunted contractile responsiveness to stress stimuli and altered diastolic relaxation with electrophysiological abnormalities.Despite the clinical description of these potential cardiac-related complications of the liver,the role of the heart has traditionally been an overlooked aspect of circulatory dysfunction in HRS.Yet from a physiological sense,temporality(prior onset)of cardiorenal interactions in HRS and positive effects stemming from portosystemic shunting demonstrated an important role of the heart in the development and progression of kidney dysfunction in cirrhotic patients.In this review,we discuss current concepts surrounding how the heart may influence the development and progression of HRS,and the role of systemic inflammation and endothelial dysfunction causing circulatory dysfunction within this setting.The temporality of heart and kidney dysfunction in HRS will be discussed.For a subgroup of patients who receive portosystemic shunting,the dynamics of cardiorenal interactions following treatment is reviewed.Continued research to determine the unknowns in this topic is anticipated,hopefully to further clarify the intricacies surrounding the liver-heart-kidney connection and improve strategies for management.
文摘Fraser syndrome is a rare malformative genetic syndrome whose main manifestations are cryptophthalmia, syndactyly, laryngeal atresia and urogenital malformations. We report the observation of a newborn from a non-consanguineous marriage, admitted to the neonatology and neonatal intensive care unit at Day 1 of life for a poly-malformative syndrome. Clinically, the newborn presented with bilateral anophthalmia, cleft palate, dysmorphic facies with a rounded forehead, hypertelorism, micrognathia, low-set ears and a short neck, syndactility and bilateral cryptorchidism. Trans fontanellar ultrasound revealed tri ventricular hydrocephalus. Cerebral MRI angiography showed malformative tri-ventricular hydrocephalus, hypoplasia of the brainstem and cerebellum, and poly-microgyria. Transthoracic and renal ultrasonography were unremarkable, and the chest X-ray was normal. The authors discuss the malformative clinical and para-clinical aspects of this syndrome, multidisciplinary management and the importance of prenatal diagnosis.
文摘Lynch syndrome is the fourth most common cancer in the United States, with an early age of onset and poor prognosis. Here, we present a unique case of a patient with progressive colon cancer due to a late diagnosis of Lynch syndrome showing excellent response to immunotherapy. A 59-year-old male with a history of rectal cancer 30 years ago came to the hospital due to a fever and further found a large necrotic colon mass. Biopsy was positive for colorectal cancer;however, due to the size of the tumor, the patient was deemed not a surgical candidate and offered hospice with palliative chemotherapy. Based on further workup, the patient was diagnosed with Lynch syndrome, with colon cancer determined to be responsive to Immunotherapy. He was started on JEMPERLI (Dosterlimab-gxly), and after three cycles of therapy, imaging and PET scan were repeated, showing decreased activity and extent of the tumor—a tremendous success.
文摘Background: Adherence to medications is dependent upon a variety of factors, including individual characteristics of the patient, interactions with health care providers, and medication complexity. Even though several studies were conducted to test intervention strategies, results are uncertain. Aim: The aim of the study is to assess if a tailored combined intervention strategy improves medication adherence in a large population of post-menopausal women affected by hypertension or metabolic syndrome. Methods: We enrolled 6833 patients aged 50 to 69 years, 85.7% with hypertension, and 14.3% with metabolic syndrome. A network between patients, general practitioners, and cardiologists was established. Interventions included education, adequate information to patients, a simplified scheme of treatment, and periodic adherence assessment. These were either delivered as healthcare provider supports or using modern technology. Medication adherence was estimated by the proportion of days covered for all classes of drugs after the index date. Results: Non-adherent hypertensive women were 297 (5%), and those with metabolic syndrome were 73 (7.4%) (p Conclusions: The rate of non-adherence in both settings of postmenopausal women was 7.7%, much lower than that described in the literature. This rate was increased in patients with metabolic syndrome;probably it is related to the complexity of the therapeutic scheme or to a poor consciousness of the disease. Therefore, implementing a tailored combined intervention can improve significantly patients’ adherence to medical therapy.
文摘Introduction: Cardiac catheterisation plays a fundamental role in the management of acute coronary syndrome. These explorations require heavy, complex and costly equipment and a large team of doctors, nurses and technicians with highly specialized training. Aims: To describe epidemiological, clinical and coronary angiography aspects of patients with acute coronary syndrome. Patients and Methods: Descriptive study from September 2019 to December 2023 in the Cardiology Department of the Hôpital Mère-Enfant of Bamako. Inclusion criteria were patients admitted for coronary angiography with the diagnosis of acute coronary syndrome. Results: During the study period, 1253 patients underwent coronary angiography, 596 of whom had acute coronary syndrome as an indication, representing a hospital frequency of 47%. Sex-ratio was 2.10. Mean age of patients was 58.5 ± 11.39 years. ST elevation acute coronary syndrome was the most common indication with 63.92% of cases. High blood pressure was the main cardiovascular risk factor with 58.7% of cases, and radial access approach was used in 98% of cases. Coronary angiography was pathological in 91.70% of cases (n = 548). Patients with lesions of anterior interventricular artery were 73.73% of cases. Tritruncal lesions accounted for 40.63% of cases. Conclusion: ST elevation acute coronary syndrome is the most frequent manifestation of acute coronary syndrome. Anterior interventricular artery is most often the culprit lesion for our patients.
基金Supported by the Natural Science Foundation of Jiangxi Province,No.20202BAB206002 and No.20224BAB216084.
文摘BACKGROUND This study presents a case of rapidly developing respiratory failure due to antisynthetase syndrome(AS)following coronavirus disease 2019(COVID-19)in a 33-year-old man diagnosed with Klinefelter syndrome(KS).CASE SUMMARY A 33-year-old man with a diagnosis of KS was admitted to the Department of Pulmonary and Critical Care Medicine of a tertiary hospital in China for fever and shortness of breath 2 wk after the onset of COVID-19.Computed tomography of both lungs revealed diffuse multiple patchy heightened shadows in both lungs,accompanied by signs of partial bronchial inflation.Metagenomic next-generation sequencing of the bronchoalveolar lavage fluid suggested absence of pathogen.A biopsy specimen revealed organizing pneumonia with alveolar septal thickening.Additionally,extensive auto-antibody tests showed strong positivity for anti-SSA,anti-SSB,anti-Jo-1,and anti-Ro-52.Following multidisciplinary discussions,the patient received a final diagnosis of AS,leading to rapidly progressing respiratory failure.CONCLUSION This study underscores the clinical progression of AS-associated interstitial lung disease subsequent to viral infections such as COVID-19 in patients diagnosed with KS.