[The 486th case: chronic diarrhea and orthostatic hypotension].

A 57-year-old man was admitted to hospital with diarrhea for 10 months and dizziness for 4 months. The patient had 1-2 liters watery stool per day, without pyogenic blood or abnormality in gastroenteroscopy examination. The level of hemoglobin and albumin was generally normal, and fasting test was positive. At the same time, he was accompanied with hyperalgesia of lower limbs and orthostatic hypotension. After the discussion of multiple disciplinary teams, the patient was diagnosed with amyloidosis by sural nerve biopsy, myocardial MRI, and the assays of urine immunoelectrophoresis and serum free light chain. Light chain amyloidosis was confirmed after excluded the diagnosis of familial amyloidosis. The patient was improved after courses of chemotherapy with melphalan and dexamethasone.

[The clinicopathologic features and prognostic analysis of pancreatic metastasis from clear cell renal cell carcinoma].

Objective: To investigate the clinicopathologic features and prognosis of the patients who had clear cell renal cell carcinoma (CCRCC) with metastasis to the pancreas. Methods: From Jan, 2000 to Dec, 2018, 18 patients with clear cell renal cell carcinoma (CCRCC) and had pathologically diagnosed metastasis to the pancreas were enrolled at Peking Union Medical College Hospital. The clinical and pathological data were retrospectively analyzed. Results: 11 out of 18 patients were male, and the other 7 were female. The average age of onset of CCRCC was 51.4 years. 8 cases (44.4%) occurred in the left kidney, and the other 10 cases (55.6%) with right kidney tumor. Three patients had synchronous pancreatic metastasis, and the other 15 patients had metachronous pancreatic metastasis. The median time from CCRCC onset to pancreas metastasis was 156 months. The main complaints of pancreas metastasis were abdominal pain, jaundice, gastrointestinal bleeding, nausea, weakness, loss of weight and so on. Seven patients (38.9%) had single lesion of pancreas, while 11 patients (66.1%) had multiple lesions of pancreas. Nine patients (50%) had other organs metastasis besides pancreatic metastasis at the same time. Five patients underwent pancreatic metastasis resection, while 15 patients received oral tyrosine kinase inhibitor(TKI). The mean follow-up was 171.7 months(1~361.5 months) and 5 patients died. The median overall survival (mOS) was 122 months, and the 5 year-survival rate was 81.4%. In univariate analysis, synchronous metastasis to the pancreas, relapse after 10 years, Memorial Sloan Kettering Cancer Center prognostic index, International Metastatic Renal Cell Carcinoma Database Consortium index were all significant parameters for patients'survival. Conclusions: Metastasis to the pancreas from clear cell renal cell carcinoma were rare. These patients had better survival outcomes, especially those relapsing after ten years. Pancreatic metastasis resection had no significant benefit on patient's survival.

Effects of growth hormone on pregnancy rates of patients with thin endometrium.

PURPOSE: To investigate whether growth hormone (GH) could improve pregnancy rates of patients with thin endometrium by clinical study and laboratory experiments. MATERIALS AND METHODS: Ninety-three patients were randomized to either the GH-received group (40) or the routine exogenous administration of estrogens control group (53) for clinical study. The human endometrial carcinoma cell line RL95-2 was used for testing the role of GH with Western blot and real-time PCR by exposure to various concentrations of GH (0.1 nM,1 nM,10 nM,100 nM). RESULTS: Patients treated with GH had a significantly (P < 0.05) greater endometrium thickness on day 3 (7.87±0.72 vs 6.34±0.86), higher implantation rates (24.4% vs 10.5%) and greater clinical pregnancy rates (42.5% vs 18.9%) compared with the control group. No adverse events were associated with the use of GH. Administration of GH significantly up-regulated the expression of VEGF, ItgB3 and IGF-I expression in RL95-2 cells at both mRNA and protein levels (P < 0.05). AG490, an inhibitor of JAK2, nearly completely inhibited the up-regulative effect of GH through the JAK2-STAT5 pathway, and GH-induced effects could be mediated through autocrine IGF-I together with its hepatic counterpart. IGF-I mRNA was detected in the RL95-2 cells. CONCLUSION: GH may improve pregnancy outcomes of patients with thin endometrium who undergo frozen embryo transfer by acting on human endometrial cells to promote proliferation and vascularization and to up-regulate receptivity-related molecular expression.

Correction to: Effects of growth hormone on pregnancy rates of patients with thin endometrium.

Unfortunately, there are errors that occurred in the name and manufacture of the growth hormone (GH) received by the patients in the GH group on page two, Table 1 and figure 1 on page three.

[The 465th case: intestinal obstruction, gastrointestinal hemorrhage and duodenal fistula].

This is a complicated and difficult case. The onset symptom of a 62-year-old male was recurrent intestinal obstruction. Ileocecal and ileocolic operation was done twice. Massive gastrointestinal bleeding occurred due to giant fistula of descending duodenum, which connected to ileocolic anastomosis. After consultation by multidisciplinary team, jejunal-feeding tube was placed to provide enteral nutrition. With general condition improving, duodenal fistula repair and involved bowel resection were performed. Postoperative pathology confirmed Crohn's disease. The patient was treated with thalidomide and recovered well during follow-up.

[The colonoscopic characteristics of colorectal endometriosis: a single-centered retrospective study].

Objective: To reinforce the awareness of colorectal endometriosis (EM) in colonoscopy examination. Methods: Patients diagnosed as colorectal EM at Peking Union Medical College Hospital between February 2002 and February 2017 were enrolled in this study. The clinical characteristics and endoscopic features of EM lesions were summarized and compared between pathologically positive group and negative group. Results: A total of 34 cases were included with average age of (38.3±8.9) years old. All EM lesions located within rectum and sigmoid colon. The endoscopic lesions manifested as protrusion in 21 cases (61.8%) and protrusion-depression in 13 cases (38.2%), local stenosis in 8 cases (23.5%); erosive surface in 33 cases (97.1%) with local spontaneous hemorrhage in 4 cases (11.8%); nodal surface in 23 cases (67.6%), and lymphangiectasis base in 9 cases (26.4%). Endoscopic biopsy specimens were obtained in all cases with average 3 (2, 4) pieces. Positive results were found only in 4 patients (11.8%) with 3 endometriosis and one (endometrial) adenosarcoma. Compared with negative group, spontaneous hemorrhage was more frequent in positive group (2/4 vs. 2/30, P=0.013). Mean biopsy sample number was significantly larger in positive group (5 vs. 3, P=0.004). Conclusions: Colorectal endometriosis is mostly located within rectosigmoid region. Endoscopic features mainly include protrusion or protrusion-depression lesions with erosive and nodular surface, or local stenosis. Spontaneous hemorrhage under colonoscopy yields higher positive rate for biopsy, thus increasing biopsy sample numbers may improve pathology results.

[An analysis of clinical characteristics of twelve cases of mesenteric panniculitis].

Objective: Mesenteric panniculitis is an idiopathic, uncommon disease involving the adipose tissue of mesentery. The etiology, diagnosis and treatment are still unnoticed. We thus reported a case series to improve the understanding of this rare disorder. Methods: We retrospectively analyzed the clinical data of 12 patients with mesenteric panniculitis including manifestation, diagnosis, treatment and prognosis. Results: We found a male predominance (M∶F 3∶1) with the median age of 58 years old at diagnosis. The most common symptom was abdominal pain (9/12), followed by abdominal distension (3/12) and weight loss (3/12). Physical examination was unremarkable in the majority of patients (8/12). C reactive protein (9/12) and erythrocyte sedimentation rate (10/12) were normal in majority of patients. CT findings were of much diagnostic value. All patients had small intestinal mesentery involvement and multi-nodular appearance with increased fat density. Pseudo-capsule sign (8/12) and fat halo sign (6/12) were common. Pathological diagnosis was obtained in 4 cases showing fat tissue inflammation with local necrosis and fibrosis. Six cases all received prednisone, 2 with combined cyclophosphamide, 1 with azathioprine, 1 with tripterygium wilfordii. Short-term clinical response was achieved in all cases, but two patients relapsed. Conclusions: Mesenteric panniculitis occurs predominantly in middle-aged and elderly. Abdominal pain is the leading symptom. Inflammatory markers are often normal while computed tomography is the most important diagnostic tool. Surgery combined with cortical steroid and immunosuppressant agents is effective.

[An analysis of clinical features of celiac disease patients in different ethnic].

OBJECTIVE: To summarize the clinical features of different racial patients with celiac disease (CD) and analyze the disease prevalence, diagnosis and treatment in Chinese population. METHODS: All the patients were diagnosed as CD and enrolled in Beijing United Family Hospital between January 2005 and July 2015.Clinical data including nationality, age, symptoms, endoscopic and pathological findings, outcome were collected and compared in patients from different countries. RESULTS: A total of 87 patients were enrolled including 63 Caucasians, 18 Asian patients and 6 Middle East patients.The peak age of disease onset was 40-60 years old.Patients with typical symptoms such as chronic diarrhea and weight loss only accounted for 20.7%(18/87) and 9.2%(8/87) respectively.Some patients presented with nonspecific symptoms such as abdominal pain and bloating [32.2%(28/87)], even constipation [5.7%(5/87)].13.8%(12/87) patients were previously diagnosed as irritable bowel syndrome.The incidence of abdominal pain, bloating, diarrhea and constipation between Asians and Caucasians had no statistical significance (P>0.05); but the proportions of weight loss, growth retardation, iron deficiency anemia and dermatitis herpetiformis in Asian group were significantly higher than that in Caucasian group (P<0.05). IgA type of anti-gliadin antibody (AGA), endomysium antibody (EMA) and tissue transglutaminase antibody (tTGA) were dominant autoimmune antibodies in patients with CD, which accounted for 58.6%(51/87), 44.8%(39/87) and 36.8%(32/87) respectively.The endoscopy showed that the lesion of CD was mainly located in small intestine, with reducing severity from the proximal to the distal small intestine.The lesions of duodenal bulb and descending duodenum appeared more significant in Asian group.Accordingly pathological intestinal atrophy and the degree of intraepithelial lymphocytosis were more severe in Asian patients.All 87 cases took the gluten-free diet (GFD). Eighty-one cases received serological follow up and 8 with endoscopic intestinal biopsy.The celiac disease antibodies in 47 patients turned negative from 6-9 months after GFD treatment, while 34 patients turned negative from 12-18 months after GFD.All patients reported disease remission to some extent.After 1 year GFD treatment, the pathology of endoscopic intestinal biopsy in 8 patients showed significant improvement of villous atrophy and lymphocyte infiltration. CONCLUSIONS: CD patients with typical clinical manifestations are not the majority.Serological celiac disease antibodies (AGA, EMA and tTGA) have a high diagnostic value.GFD treatment is effective on majority of celiac patients.Clinical manifestations, endoscopy, intestinal pathology, and response to GFD in Chinese patients are not the same as Caucasians.Clinicians need to pay attention to the differential diagnosis.

[A retrospective case-control study of immunoglobulin G4-related disease combined with malignancy].

IgG4-related disease (IgG4-RD) has been proved to be associated with malignancy.The incidence and risk factors of malignancy development in IgG4-RD were not clear. Nine IgG4-RD patients with malignancies and 27 IgG4-RD control cases were analyzed for risk factors and clinical features. The incidence of malignancy in IgG4-RD was 3.3%, higher than age-controlled general population. Smoking history was significantly more common in patients with malignancies than in the control group (9/9 vs 16/27, P<0.05). A total of 6/9 malignancies occurred within the first year after the diagnosis of IgG4-RD. Colorectal, biliary and thyroid cancers were the leading types. Smoking history is a risk factor for IgG4-RD associated malignancy. Careful vigilance to monitor malignancy needs to be paid during follow-up.

The effect of weight-bearing on the radiographic measurement of the position of the femoral head after total hip arthroplasty.

BACKGROUND: In most radiographic studies of polyethylene wear, investigators have used routine annual radiographs made with the patient in the supine position in order to measure penetration by the femoral head into the polyethylene liner. However, researchers have begun to question the effect of weight-bearing on the position of the head within the acetabular cup and, consequently, the effect of weight-bearing on measurements of penetration by the head. The purpose of the current study was to determine the effect of weight-bearing on the two-dimensional radiographic position of the femoral head within the acetabular cup. METHODS: Thirty-seven patients (forty-seven hips) who had had a total hip arthroplasty had radiographs made at one of two separate institutions. A set of anteroposterior radiographs was made for each patient: one radiograph was made with the patient supine and one was made with the patient upright bearing full weight on the replaced hip. At one of the institutions, a third anteroposterior radiograph was made with the patient in the same upright position but not bearing weight on the replaced hip. All measurements of the two-dimensional position of the head were performed by a single observer with use of a previously published computerized measurement system. RESULTS: Data from both institutions revealed that measurements of the position of the head on radiographs made with the patient supine were strongly and significantly correlated with measurements of the position of the head on radiographs made with the patient bearing weight (r2>0.93, p<0.001 for both regressions). Examination of the differences between the measurements revealed no bias for one set of measurements to consistently underestimate or overestimate the values derived with use of the other method. Moreover, we found a nearly perfect relationship between the measurements of the position of the head on radiographs made with the patient standing and bearing weight and those on radiographs made with the patient standing but not bearing weight (r2 = 0.97, p<0.001, slope = 0.99, intercept = 0.02 millimeter). CONCLUSIONS: On the basis of these findings, there is no evidence that radiographs must be made with the patient bearing weight in order to accurately measure the position of the femoral head within the polyethylene liner.

[Roentgenological and pathological findings in 22 cases of secondary fungus ball of pulmonary aspergillosis].

Twenty-two cases of secondary fungus ball of pulmonary aspergillosis defined by operation were reported. All cases were referred with incorrectly diagnosis to the authors and only 45.3% were correctly diagnosed before operation. The roentgenological and pathological findings were studied. The results show that not familiar with non-typical appearance is the most common cause for incorrect diagnosis.

Variceal bleeding and portal hypertension: still a therapeutic challenge?

In the primary prevention of variceal hemorrhage, beta-blockers continue to be the first-line treatment. Newer nonselective beta-blockers with anti-alpha1-adrenergic activity, such as carvedilol, appear to have a better impact on reducing the hepatic venous pressure gradient than propranolol. The addition of isosorbide mononitrate appears to improve the effectiveness of beta-blockers in primary prophylaxis, but not that of somatostatin in the treatment of acute variceal hemorrhage. The use of vasoactive drugs alone in acute variceal bleeding has not proved to be more effective than endoscopic treatment. The advent of endoscopic variceal ligation (EVL) has strengthened the role of endoscopy in the management of bleeding esophageal varices. EVL has improved the results, particularly in terms of lowering the treatment-related morbidity, compared with endoscopic variceal sclerotherapy (EVS). However, the variceal recurrence rate after initial eradication with EVL is relatively high. In contrast to synchronous combined therapy with EVL plus EVS, metachronous combination of EVL and low-dose EVS may improve the results of EVL alone. For bleeding fundic varices, obliteration using cyanoacrylate is currently the treatment of choice. Endosonography (EUS) is coming into more widespread use in the assessment of variceal eradication and in further attempts to improve the results of endoscopic injection therapy. According to two meta-analysis studies, transjugular intrahepatic portosystemic shunt (TIPS) is not yet capable of replacing endoscopic treatment in the secondary prevention of variceal bleeding.

Two-dimensional versus three-dimensional radiographic measurements of polyethylene wear.

Because polyethylene wear adversely affects the outcome of total hip arthroplasty, reliable techniques for in vivo radiographic evaluation of femoral head penetration into the polyethylene liner are needed. With the recent development of software to measure three-dimensional femoral head penetration, the adequacy of two-dimensional head penetration measurements to estimate a three-dimensional process has been questioned. Thus, the purpose of the current study was to compare directly two-dimensional and three-dimensional radiographic measurements of femoral head penetration. Using two computer assisted radiographic techniques, the authors of this study measured the two-dimensional and the three-dimensional penetration of the femoral head into the polyethylene liner in 202 hips implanted with porous coated acetabular components. A comparison of two-dimensional and three-dimensional measurements showed that, on average, the mean amount of three-dimensional head penetration was larger than the mean amount of two-dimensional head penetration. However, linear regression analysis showed that the two measurements were highly correlated (r2 = 0.87, slope = 0.99, intercept = 0.08 mm). For most patients (95%), two-dimensional and three-dimensional measurements of femoral head penetration were nearly equal. However, there was a small subset of patients (5%) for whom three-dimensional radiographic penetration measurements were three times greater than corresponding two-dimensional radiographic penetration measurements. No common risk factor among this small subset of patients was identified. The current study provides practical information for the orthopaedic surgeon trying to assess polyethylene wear in hip replacement cases. It shows that for most patients head penetration can be measured sufficiently from anteroposterior radiographs alone. However, in a small percentage of patients (5% in the current study) the femoral head also moves perpendicular to the plane of the anteroposterior radiograph. For these cases, accurate analysis of head penetration requires three-dimensional analysis using anteroposterior and lateral radiographs.