ABSTRACT
OBJECTIVE: The treatment of Henoch-Schönlein purpura (HSP) with moderate proteinuria remains controversial. We retrospectively analyzed the efficacy of immune suppressants, with a particular emphasis on mycophenolate mofetil (MMF). METHODS: Ninety-five HSP patients with moderate proteinuria (1.0-3.5 g/24 h) after at least three months of therapy with angiotensin-converting enzyme inhibitor (ACEI) or angiotensin receptor blocker (ARB) were divided into three groups: an MMF group (n=33) that received MMF 1.0-1.5 g/d combined with prednisone (0.4-0.5 mg/(kg·d)), a corticosteroid (CS) group (n=31) that received full-dose prednisone (0.8-1.0 mg/(kg·d)), and a control group (n=31). Patients in the MMF and CS groups continued to take ACEI or ARB at the original dose. The patients in the control group continued to take ACEI or ARB but the dose was increased by (1.73±0.58)-fold. The patients were followed up for 6-78 months (median 28 months). RESULTS: The baseline proteinuria was higher in the MMF group ((2.1±0.9) g/24 h) than in the control group ((1.6±0.8) g/24 h) (P=0.039). The proteinuria decreased significantly in all groups during follow-up, but only in the MMF group did it decrease significantly after the first month. At the end of follow-up, the proteinuria was (0.4±0.7) g/24 h in the MMF group and (0.4±0.4) g/24 h in the CS group, significantly lower than that in the control group ((0.9±1.1) g/24 h). The remission rates in the MMF group, CS group, and control group were respectively 72.7%, 71.0%, and 48.4% at six months and 72.7%, 64.5%, and 45.2% at the end of follow-up. The overall number of reported adverse events was 17 in the MMF group, 30 in the CS group, and 6 in the control group (P<0.001). CONCLUSIONS: MMF with low-dose prednisone may be as effective as full-dose prednisone and tend to have fewer adverse events. Therefore, it is probably superior to conservative treatments of adult HSP patients with moderate proteinuria.
Subject(s)
IgA Vasculitis/complications , IgA Vasculitis/drug therapy , Mycophenolic Acid/analogs & derivatives , Nephritis/drug therapy , Nephritis/etiology , Prednisone/administration & dosage , Adult , Anti-Inflammatory Agents/administration & dosage , Dose-Response Relationship, Drug , Drug Therapy, Combination , Female , Humans , IgA Vasculitis/diagnosis , Male , Mycophenolic Acid/administration & dosage , Nephritis/diagnosis , Remission Induction/methods , Retrospective Studies , Treatment OutcomeABSTRACT
Conjoined twins (CT) are rare with possible serious malformations in which soft tissue, bone, or some organs are joined in utero. The extent of cardiac fusion and intracardiac anatomy of CT determine the viability, natural history, and outcome of potential surgical intervention. Early prenatal diagnosis and assessment may provide a window of opportunity to counsel the family for their informed decision on the pregnancy and to plan for prenatal and perinatal care. In this report, we describe a case of thoracopagus twins diagnosed by fetal echocardiography at 23-week gestation. The 2 hearts fused at the atrial and ventricular levels. The outcome and review of literature on fetal echocardiographic characteristics of this malformation are discussed.
Subject(s)
Fetal Heart/diagnostic imaging , Heart Defects, Congenital/diagnostic imaging , Twins, Conjoined , Ultrasonography, Prenatal/methods , Abortion, Eugenic , Adult , Female , Heart Atria/diagnostic imaging , Heart Ventricles/diagnostic imaging , Humans , PregnancyABSTRACT
OBJECTIVE: To explore the value of transthoracic and right heart contrast echocardiography in the diagnosis of coronary sinus septal defect (CSSD), also known as unroofed coronary sinus. METHODS: The echocardiographic characteristics of 20 patients with CSSD who underwent surgery in our hospital between October 1999 and June 2012 were reviewed retrospectively, including results of 9 cases studied by contrast echocardiography, and compared with surgical results. RESULTS: Of the coronary sinuses in these 20 patients, 40% were totally unroofed, 35% partially unroofed in mid-portion, 20% partially unroofed in terminal portion, and 5% mixed type (mid- and terminal portions). Of these 20 patients, 65% were associated with other types of atrial septal defects, and 65% had a persistent left superior vena cava. The diagnostic accuracy of echocardiography for the CSSD was 65%. In patients whose diagnoses were confirmed by echocardiography, the accuracy for the types of CSSD was 84.6%. All of the first 6 cases studied before 2002 were either undiagnosed or misdiagnosed, and 78.6% of the last 14 cases studied were diagnosed accurately. Of 9 patients who underwent contrast echocardiography, 8 were correctly diagnosed, including 5 type II and 3 Raghib syndromes. 1 case of type IIIa was misdiagnosed as Raghib syndrome. CONCLUSIONS: The combination of transthoracic and contrast echocardiography can diagnose and classify CSSD accurately in most cases and should be the first choice for diagnosing CSSD.
