Small intestinal mucosal abnormalities using video capsule endoscopy in intestinal lymphangiectasia.

BACKGROUND: Intestinal lymphangiectasia (IL) is a rare protein-losing enteropathy caused by disorders of the intestinal lymphatics. There are only a few case reports and case series concerning the VCE (video capsule endoscopy) findings of IL. This work aimed to evaluate the VCE characteristics of small intestinal mucosal abnormalities in patients with IL, and to investigate the relationship between clinical and VCE characteristics. METHODS: Consecutive patients with IL who underwent VCE were enrolled in this retrospective study. The cases were classified into the white villi group and non-white villi group according to mucosal abnormalities detected by VCE. Clinical and endoscopic characteristics were investigated and analyzed. RESULTS: A total of 98 patients with IL with a median onset age of 26.3 ± 19.2 years were included. VCE revealed the following small intestinal lesions: (i) white villi type (57/98, 58.2%), i.e.: white-tipped or granular villi, white nodular villi or plaques; (ii) non-white villi type (41/98, 41.8%), i.e.: diffused low and round villi; (iii) complications (46/98, 46.9%), i.e.: bleeding, ulcers, protruding or vesicular-shaped lesions, stenosis and lymphatic leakage. A total of 58.2% (57) and 41.8% (41) of the cases were classified into the white villi and non-white villi groups respectively. The percentage of chylothorax in the white villi group was significantly lower than that in the non-white villi group (12/57 vs. 19/41, p = 0.008). In VCE, there were no significant differences in the involved segments and total detected rate of complications between the white villi and non-white villi groups (p > 0.05), while the detected rate of lymphatic leakage in the white villi group was significantly higher than that in the non-white villi group (31.6% vs. 12.2%, p = 0.026). CONCLUSIONS: Our study evaluated the entire small intestinal mucosal abnormalities of IL by VCE, especially endoscopic complications. IL has specific VCE abnormalities in addition to classical endoscopic findings.

A retrospective analysis of Stewart-Treves syndrome in the context of chronic lymphedema

Abstract Background stewart-treves syndrome (STS) is an angiosarcoma associated with chronic lymphedema. Objectives This article analyses the characteristics of twenty-two patients and proposes active intervention in lymphedema and the early diagnosis of STS. Methods Twenty-two patients with STS were diagnosed at the centre over an 11-year period. Clinical manifestations, a series of conventional analyses, and histopathology were used to study these cases retrospectively. Results The age range of 22 patients with STS was 15 to 78 years. The main clinical manifestations included multiple skin and subcutaneous nodules and scattered red or purplish-red rashes in the lymphoedematous limbs. These patients often showed clinical symptoms such as lymphedema, weakness, emaciation, pain, mass, lymphadenopathy and so on. The positive rates of ultrasonography, MRI and radionuclide imaging were 66.7% (6/9), 92.3% (12/13) and 18.2% (2/11), respectively. The main points regarding active intervention in lymphedema and early diagnosis of STS were summarized. Study limitations Since this was a retrospective study, the main points summarized by the author need to be further quantified in clinical work to guide the diagnosis of this kind of disease more conveniently. In addition, further clinical trials are needed to evaluate the role of lymphedema in the occurrence and development of malignant tumors. Conclusions STS can appear in lymphoedematous tissue many years after lymphedema onset. To avoid delays in the diagnosis and therapy of STS, physicians should actively look for signs or symptoms of malignant lymphedema during the follow-up period and promptly manage patients developing problems.

A clinical study of liposuction followed by lymphovenous anastomosis for treatment of breast cancer-related lymphedema.

Objective: In this work, we studied the clinical effect of liposuction followed by lymphovenous anastomosis (LVAs) for the treatment of breast cancer-related lymphedema (BCRL). Methods: We analyzed 158 patients with unilateral upper limb BCRL who underwent liposuction followed by LVAs 2-4 months later. Arm circumferences before and 7 days after the combined treatments were prospectively recorded. Circumferences of different upper extremities were measured before the procedure, 7 days after LVAs, and during the follow-ups. Volumes were calculated with the frustum method. During the follow-ups, the conditions of patients' treated arms, i.e., the frequency of erysipelas episodes and dependence on compression garments, were recorded. Results: The mean circumference difference between two upper limbs decreased significantly from M (P25, P75) of 5.3 (4.1, 6.9) preoperatively to 0.5 (-0.8, 1.0) (P < 0.05) 7 days after treatments, while at follow-up 0.3 (-0.4, 1.0). The mean volume difference decreased significantly from M (P25, P75) of 838.3 (662.4, 1,129.0) preoperatively to 7.8 (-120.3, 151.4) (P < 0.05) 7 days after treatments, while at follow-up 43.7 (-59.4, 161.1). The incidence of erysipelas also significantly decreased (P < 0.05). 6.3% of patients were already independent of compression garments during the past six months or even more. Conclusion: Liposuction followed by LVAs is an effective method for the treatment of BCRL.

Phospholipase A2 (PLA2) injured lymphatic endothelial cells leading to progression of secondary lymphoedema.

Secondary lymphoedema is one of the common complications after lymph node dissection for gynecologic malignancies and breast cancer. In this study, the relationship between PLA2 and postoperative lymphoedema in cancer at the molecular level has been explored through transcriptomics and metabolomic assays. Transcriptome sequencing technology, as well as metabolomic assays, were utilized to explore the expression of PLA2 in lymphoedema patients, and search for potential pathways in the pathogenesis and exacerbation mechanism of lymphoedema. The effect of sPLA2 on human lymphatic endothelial cells was investigated by culturing human lymphatic endothelial cells. Secretory phospholipases A2 (sPLA2) showed high expression levels in lymphoedema tissues, however, cytoplasmic phospholipases A2 (cPLA2), showed low expression in lymphoedema, as demonstrated by RT-qPCR. By culturing human lymphatic vascular endothelial cells, the study found that sPLA2 causes HLEC vacuolization and has an inhibitory effect on HLEC proliferation and migration. By detecting sPLA2 in the serum of lymphoedema patients and analyzing clinical data, it was found that sPLA2 was positively correlated with the severity of lymphoedema. Secretory Phospholipase A2 (sPLA2) is highly expressed in lymphoedema tissue, damages lymphatic vessel endothelial cells, is strongly associated with disease severity, and can be used as a potential predictor of disease severity.Abbreviations: PLA2: Phospholipase A2; DEGs: differentially expressed genes; DMP: differential metabolic production.

A retrospective analysis of Stewart-Treves syndrome in the context of chronic lymphedema.

BACKGROUND: stewart-treves syndrome (STS) is an angiosarcoma associated with chronic lymphedema. OBJECTIVES: This article analyses the characteristics of twenty-two patients and proposes active intervention in lymphedema and the early diagnosis of STS. METHODS: Twenty-two patients with STS were diagnosed at the centre over an 11-year period. Clinical manifestations, a series of conventional analyses, and histopathology were used to study these cases retrospectively. RESULTS: The age range of 22 patients with STS was 15 to 78 years. The main clinical manifestations included multiple skin and subcutaneous nodules and scattered red or purplish-red rashes in the lymphoedematous limbs. These patients often showed clinical symptoms such as lymphedema, weakness, emaciation, pain, mass, lymphadenopathy and so on. The positive rates of ultrasonography, MRI and radionuclide imaging were 66.7% (6/9), 92.3% (12/13) and 18.2% (2/11), respectively. The main points regarding active intervention in lymphedema and early diagnosis of STS were summarized. STUDY LIMITATIONS: Since this was a retrospective study, the main points summarized by the author need to be further quantified in clinical work to guide the diagnosis of this kind of disease more conveniently. In addition, further clinical trials are needed to evaluate the role of lymphedema in the occurrence and development of malignant tumors. CONCLUSIONS: STS can appear in lymphoedematous tissue many years after lymphedema onset. To avoid delays in the diagnosis and therapy of STS, physicians should actively look for signs or symptoms of malignant lymphedema during the follow-up period and promptly manage patients developing problems.

Does a home-based exercise program play any role in the treatment of knee osteoarthritis? A meta-analysis.

BACKGROUND: Knee-osteoarthritis is a very common joint disorder, affecting about 85% of the population worldwide. The effectiveness of home-based exercises is still debatable, with many studies indicating positive outcomes with few side effects, while others find them of limited utility. OBJECTIVES: To assess the role of home-based exercise (HBE) programs in the treatment of knee osteoarthritis. MATERIAL AND METHODS: Randomized controlled trials were included as per the predefined Population, Intervention, Comparison, Outcomes and Study (PICOS) criteria. Demographic summaries and event data for osteoarthritis therapy in the exercise and control groups were assessed, and comparative efficacy was evaluated using clustered graphs. The RevMan software was used to calculate the odds ratio (OR) and risk ratio of the included studies. The risk of bias was also evaluated and heterogeneity analysis was performed. RESULTS: Fifteen clinical trials performed from 2000 to 2022, with a total of 2922 osteoarthritis patients, were included in the study, according to the chosen inclusion criteria. We observed a reduction in Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) scores but a more marked improvement in clinical symptoms in the exercise group. The Knee Injury and Osteoarthritis Outcome Score (KOOS) increased only in the exercise group and not in the control group. We obtained a pooled OR of 0.59 (95% confidence interval (95% CI): 0.36-0.98), T2 value of 0.88, χ2 value of 185.41, degrees of freedom (df) value of 14, I2 value of 92%, and p-value <0.00001. The overall Z effect was 2.04 with a p-value of 0.04. The pooled risk ratio was 0.81 (95% CI: 0.66-0.99) with a T2 value of 0.14, χ2 value of 191.53, df value of 14, I2 value of 93%, and p-value <0.00001. CONCLUSION: The data from the studies included in this meta-analysis are in favor of the use of HBEs for the treatment of knee osteoarthritis.

Liposuction in cancer-related lower extremity lymphedema: an investigative study on clinical applications.

BACKGROUND: Lymphedema is a progressive, noncurable condition consisting of increases in subcutaneous fat and interstitial fluid in the limbs and fibrosis during later stages. The disease most commonly affects the limbs following injury to or removal of the lymph nodes. The aim of this study was to investigate the therapeutic outcomes of liposuction for cancer-related lower extremity lymphedema. METHODS: Sixty-two patients with cancer-related lymphedema in the unilateral lower extremity were recruited for this study, and all patients underwent liposuction. The volume of hemorrhage and lipids, the operation time, and the volume changes of the affected extremity were compared by applying the t tests, and the subjective feelings of patients were compared with the chi-square tests. RESULTS: The total lipid volume was 2539 ± 1253.5 ml, and the hemorrhage volume was 828 ± 311.8 ml. For the comparison of objective indices, (1) the percent volume differences (PVDs) before surgery, intraoperatively, and at the 3-month follow-up were 5.5 ± 12.2 vs. 11.6 ± 18.4 vs. 43.2 ± 23.7, P < 0.05, respectively; (2) greater lipid volumes and higher liposuction rates were observed for female patients, as was a smaller volume of hemorrhage; (3) greater hemorrhage volumes were observed in patients with a history of recurrent erysipelas; and (4) greater lipid volumes and liposuction rates (LRs) and smaller hemorrhage volumes were observed for stage II than for stage III patients. CONCLUSIONS: Liposuction is an effective therapy for cancer-related lower extremity lymphedema. Sex, stage, and recurrent erysipelas history influence the course and effect of liposuction.

Lymphedema complicated by protein-losing enteropathy with a 22q13.3 deletion and the potential role of CELSR1: A case report.

INTRODUCTION: 22q13.3 deletion syndrome is a well-known syndrome characterized by typical clinical findings including neonatal hypotonia, absent or severely delayed speech, intellectual disability, and other various features, and detection of a heterozygous deletion of chromosome 22q13.3 with the involvement of at least part of SHANK3. It is reported that 10% to 29% of patients with 22q13.3 deletion syndrome present lymphedema. Protein-losing enteropathy (PLE) has never been reported in 22q13.3 deletion syndrome. PATIENT CONCERNS: The patient presented to our institution for refractory hypoalbuminemia and chronic lymphedema in both legs. DIAGNOSIS: The patient manifested intellectual disability, absent speech, tooth grinding, dysmorphic face, and abnormal hands and toenails. Copy-number variation sequencing confirmed the maternal deletion in 22q13.31-q13.33 (chr22:46285592-51244566, hg19). The patient was genetically diagnosed with 22q13.3 deletion syndrome. INTERVENTIONS: Low-fat diets and medium-chain triglycerides supplements were prescribed. The patient was recommended to wear compression garments and elevate legs. OUTCOMES: The symptom of diarrhea was resolved, but hypoalbuminemia persisted. Lower extremities lymphedema was gradually becoming severe. CONCLUSIONS: Primary lymphedema and PLE can occur simultaneously in a patient with 22q13.3 deletion syndrome. The 2 phenotypes could share the same genetic etiology of congenital lymphatic abnormalities. CELSR1 deletion may play a role in lymphatic dysplasia. The case also provides additional proof of the pathogenic effect of CELSR1 on hereditary lymphedema.

New application of direct lymphangiography in the diagnosis and treatment of chylothorax after lung cancer surgery: a case series.

BACKGROUND: Chylothorax is caused by accumulation of chylous fluid in the thoracic cavity following injury to the thoracic duct or its tributaries. It is a rare but relatively severe complication of lung cancer surgery, especially after lung resection and mediastinal lymph node dissection. If chylothorax is not treated promptly and effectively, it may result in relatively high mortality rate. Although the majority of patients with chylothorax secondary to lung cancer surgery can be treated with conservative measures, there are still some patients who fail to respond to conservative treatment or suffer from recurrent episodes. This study aims to explore the causes of postoperative chylothorax and how targeted treatment can help achieve better outcomes. METHODS: Four cases of chylothorax secondary to lung cancer surgery are reported in which patients underwent direct lymphangiography (DLG) after failed conservative treatment. RESULTS: Thoracic duct obstruction was found in all patients on DLG, as well as dilated intrathoracic part of the thoracic duct. Thoracic duct reconstruction was performed to successfully treat 3 of the 4 patients. CONCLUSIONS: DLG is of great value in the diagnosis of chylothorax secondary to lung cancer surgery, for which thoracic duct reconstruction proves to be an effective procedure. DLG provides a new objective basis for the diagnosis and treatment of chylothorax. It is our best wish to help more patients with chylothorax secondary to lung cancer surgery.

CT Lymphangiography (CTL) in Primary Intestinal Lymphangiectasia (PIL): A Comparative Study with Intraoperative Enteroscopy (IOE).

RATIONALE AND OBJECTIVES: To investigate the clinical feasibility of CT lymphangiography (CTL) in primary intestinal lymphangiectasia (PIL) by comparison with intraoperative enteroscopy (IOE) during exploratory laparotomy. MATERIALS AND METHODS: Eleven PIL patients (F/M, two/nine, age range 10-37 years) were recruited in this study, and they were performed IOE during exploratory laparotomy for suspected serious lymphatic-intestinal leakages. All the patients were performed CTL before surgery, and the imaging data were reviewed by two radiologists separately. CTL assessments included intestinal lesions, edematous lesions, intestinal and mesenteric lymphangiectasia, lymphaticabdominal leakages, lymph fluid reflux, lymphangioma and abnormal lymphatics in other area. The intestinal lymphangiectasia and lymphaticintestinal leakages were confirmed by histology and IOE. RESULTS: For CTL, (1) nine intestinal wall thickening; (2) eight ascites, complicated with four pleural effusions, (3) eight intestinal and mesenteric lymphangiectasia, (4) six lymph fluid reflux (5) one lymphatic-abdominal leakage, (6) two lymphangioma. While for IOE, intestinal lymphangiectasia has been confirmed in all patients, including five segemental and six diffusive lesions in intestinal mucosa. Besides, one lymphatic-intestinal fistula, one lymphatic-abdominal leakage was confirmed. Compared to IOE and histology, the accuracy of CTL was 72.7% in detecting intestinal lymphangiectasia. CONCLUSION: Compared to IOE, CTL demonstrates feasibility in detection of intestinal lymphangiectasia and other abnormalities in whole lymphatic circulation for PIL. Combination of CTL with IOE accommodates guidance for preoperative evaluation and therapeutic management for PIL.

[Application of direct lymphangiography in the diagnosis of primary chyluria].

OBJECTIVE: To discuss the role of direct lymphangiography in the diagnosis of primary chyluria. METHODS: Iodized oil was infused into lymphatic vessel of unilateral foot in 25 cases. The condition of lymphatic system imaging was dynamically observed via Innova 2100-IQ digital flat-panel angiography system. RESULTS: All of them underwent direct lymphangiography. The results revealed unilateral (n = 18) or bilateral (n = 7) renal reflux of contrast media. Among 22 stenotic cases, there were partial (n = 18) or whole (n = 4) cervical section of thoracic duct, no imaging in cervicothoracic section of thoracic duct (n = 1); thin imaging in cervical section and partial thoracic section (n = 2); retroperitoneal lymphangictasia & tortuous lymphatic vessels (n = 21); reflux of contrast medium to pelvic and/or contralateral sacroiliac part (n = 13). CONCLUSION: Capable of acquiring dynamic images of chyle (lymph) circulation, direct lymphangiography provides accurate estimation for the location, scope, extent of lesions of renal chylous reflux in primary chyluria so as to guide surgery. Stenotic cervical section of thoracic duct plays an important etiologic role in primary chyluria.

Developing classification indices for Chinese pulse diagnosis.

AIM: To develop classification criteria for Chinese pulse diagnosis and to objectify the ancient diagnostic technique. METHODS: Chinese pulse curves are treated as wave signals. Multidimensional variable analysis is performed to provide the best curve fit between the recorded Chinese pulse waveforms and the collective Gamma density functions. RESULTS: Chinese pulses can be recognized quantitatively by the newly-developed four classification indices, that is, the wave length, the relative phase difference, the rate parameter, and the peak ratio. The new quantitative classification not only reduces the dependency of pulse diagnosis on Chinese physician's experience, but also is able to interpret pathological wrist-pulse waveforms more precisely. CONCLUSIONS: Traditionally, Chinese physicians use fingertips to feel the wrist-pulses of patients in order to determine their health conditions. The qualitative theory of the Chinese pulse diagnosis is based on the experience of Chinese physicians for thousands of years. However, there are no quantitative theories to relate these different wrist-pulse waveforms to the health conditions of patients. In this paper, new quantified classification indices have been introduced to interpret the Chinese pulse waveform patterns objectively.

[The diagnosis and therapy of chylous ascites].

OBJECTIVE: To discuss the diagnosis and therapy of chylous ascites. METHODS: To diagnose 40 patients of chylous ascite with regular test and quantitative analysis of chyle, direct lymphangiography, CT (immediately after direct lymphangiography), lymphangioscintigraphy, MRI. Twenty-two patients received conservative therapy, 18 patients received retroperitoneal lymphangiectomy and (or) lymph-vein shunting. RESULTS: Lymphatic dysplasia and chylous reflux were found in almost every patient, total parenteral nutrition showed good results. Followed up from 1 month to 5 years, in conservative therapy group, 9 patients were controlled well clinically, the condition of 6 patients was improved better. Seven patients showed no effect. In operation group, 11 patients were controlled well clinically. Four patients got mitigated. Total 7 patients died, although 4 of them ameliorated temporarily. CONCLUSIONS: Direct lymphangiography, CT (immediately after direct lymphangiography) are the most important diagnosis methods. The influence of the therapy to the malformed lymphatic system of patients should be well considered. Lymph-vein shunting, such as thoracic duct-left external jugular vein anastomosis, gastroenteral or retroperitoneal lymphatics-testicular or ovarian vein anastomosis, could improve the circulation of lymph and chyle of patients. Lymphatic microsurgery will play more and more important roles in the treatment of chylous diseases.

Treating external chylous fistula with retroperitoneal lymphangiectomy plus lymph-vein shunting.

OBJECTIVE: To discuss the diagnosis and therapy of chylous reflux and external chylous fistula. METHODS: All of 6 patients were diagnosed with direct lymphagiography, lymphscintigraphy. Among them, 2 patients received CT after direct lymphagiography. Retroperitoneal lymphangiectomy plus lymph-vein shunting was performed in 5 patients, and retroperitoneal lymphangiectomy in 1 patient. RESULTS: The 6 patients were followed up from 6 months to 6 years. In 5 patients, chylous cysts disappeared and chylous fistula closed, and in 1 patient, chylous fistula didn't cicatrize, and chyle still leak out from the scrotum. CONCLUSIONS: Retroperitoneal lymphangiectomy plus lymph-vein shunting has curative effect on chylous external fistula of the lower extremity and genitalia, but it has the chance to not obstruct all paths of chylous reflex.