102例维持性腹膜透析营养不良患者中医证型分布特点

目的：分析维持性腹膜透析患者营养不良的中医证型特点,并探讨其中医发病机制。方法：对102例维持性腹膜透析营养不良患者进行中医辨证分型,对其分型特点进行分析。结果：根据营养不良程度：本虚证轻度营养不良患者中脾肾气虚证最多,随着营养不良程度加重,脾肾阳虚证和阴阳两虚证比例明显增加。标实证轻度营养不良患者中湿浊证最多,其次为瘀血证,随着营养不良程度加重,湿热、瘀血、风动证比例逐渐增加。透析充分性与中医证型：本虚证中,Kt/V﹥1．7,脾肾气虚证出现率最高,随着Kt/V值降低,脾肾气虚证比例明显减少,肝肾阴虚证、气阴两虚证和阴阳两虚证比例明显增加。标实证中,Kt/V﹥1．7,湿浊证出现率最高,其次为瘀血证、湿热证,随着Kt/V值降低,湿浊证、瘀血证患者比例明显减少,湿热证、风动证比例明显增加。结论：PD营养不良患者中医证型分布与透析充分性密切相关,其发病机制可概括为“本虚标实”,本虚以脾肾亏虚为主,标实以湿浊瘀血为主。     

MITOMYCIN-C INDUCED HEMOLYTIC UREMIC SYNDROME: A CASE REPORT

Mitomycin-C is used widely in the treatment of malignancies and is associated with serious dose related adverse effects including the occurrence of hemolytic uremic syndrome. We report a patient with a colonic adenocarcinoma who developed hemolytic uremic syndrome after receiving 85 mg/m² of the drug. The patient was left untreated and died 5 months after this diagnosis was made, of a second malignancy. Differential diagnosis and treatment of this condition are discussed.     

克氏综合征的诊断与治疗(附9例报告)

目的：探讨克氏综合征即先天性睾丸发育不全的临床特点及诊治方法。方法：回顾性分析我院收治的9例克氏综合征患者的临床资料。结果：9例患者均获得正确的诊断及相应的治疗,第二性征或(和)性功能得到不同程度的改善和维持。结论：正确诊断、及早合理治疗对改善患者精神状态,提高生活适应能力．解决婚育问题等,具有重要的临床意义。     

A Young Female with Spitzer-Weinstein Syndrome Diagnosed by Thiazide Test

Spitzer-Weinstein syndrome is a rare disorder characterized by thiazide responsive hyperkalemia and normal anion gap metabolic acidosis, similar to Gordon syndrome. The hyperfunction of thiazide-sensitive Na⁺-Cl^? cotransporter (TSC) is the main pathophysiological mechanism. We presented a 19-year-old female with short stature, normal blood pressure, persistently elevated serum potassium, and metabolic acidosis. The diagnosis of Spitzer-Weinstein Syndrome was made by clinical pictures and thiazide test. After taking 4 mg hydrochlorothiazide, her daily urine potassium excretion increased from 18.1 mmol to 41.8 mmol, urine pH decreased from 7.32 to 6.50, and urinary net charge decreased from 65.7 to 54.4. This patient then took hydrochlorothiazide 4 mg daily. The persistent hyperkalemia and metabolic acidosis were corrected. Thiazide, a powerful inhibitor of TSC, proved to be a useful tool for the diagnosis and treatment of Spitzer-Weinstein syndrome.     

慢性肾炎CKD 3期风湿内扰证的临床病理特点

目的：为进一步了解慢性肾炎风湿内扰证的特点,以及在慢性肾脏病（CKD）3期中的作用地位。方法：根据有无风湿内扰证将56例慢性肾炎CKD 3期患者分成两组,分析比较两组的临床与肾病理学资料。结果：56例患者辨证风湿内扰证43例,无风湿内扰证13例,两组兼夹虚、瘀、湿热证例数及肾病理分布虽差异无统计学意义,但风湿内扰证患者兼夹肾络瘀痹严重,与无风湿内扰证患者比较,肾络瘀痹症状总积分以及尿血、舌下脉络瘀滞积分显著升高;风湿内扰证患者除尿RBC2、4 h尿蛋白定量等较无风湿内扰证患者差异有统计学意义外,平均动脉压（MBP）也显著升高;采用katafuchi评分发现,风湿内扰证患者肾小管间质积分显著高于无风湿内扰证患者;Andreoli评分发现,风湿内扰证患者活动性指数总积分、慢性化指数总积分显著高于无风湿内扰证患者,其中间质炎细胞浸润、纤维/纤维细胞新月体也以风湿内扰证患者明显（P〈0.05）。结论：风湿内扰证是慢性肾炎CKD 3期患者中主要证候,风湿内扰加重肾络瘀痹,有致瘀致虚的演变趋势。风湿内扰与肾病理具有一定相关性,是慢性肾炎病情加重的主要病机。     

Anaesthesia and the Kearns-Sayre syndrome

A six-month-old infant presented for anaesthesia with, unbeknown to us, some of the manifestations of the Kearns-Sayre Syndrome. This syndrome is one of the clinical presentations in patients with mitochondral myopathy. The anaesthetic related events and aspects of mitochondral myopathy are described and caveats are suggested for the management of anaesthesia for patients with this type of myopathy.     

Anaesthetic management of an obstetrical patient with arthrogryposis multiplex congenita

Arthrogryposis Multiplex Congenita (AMC) describes a rare multifactorial syndrome caused by a variety of neurogenic and myopathic disorders first diagnosed at birth and often progressing to a state of significant disability. It includes abnormalities of the cardiovascular, respiratory, nervous and genitourinary systems. The syndrome is seen primarily in the paediatric population, and orthopedic procedures are common. There remains much confusion regarding diagnosis, aetiology, genetic probabilities, natural history and management.^1-2 Occasionally an adult with this sydrome presents for an anaesthetic. The following is a case report of a patient with the manifestations of this syndrome presenting for a primary Caesarean section.     

The Larsen Syndrome with multiple congenital dislocations and a normal facies

Summary Details of four children with congenital skeletal abnormalities are reported. In one, typical of the Larsen Syndrome, an autosomal dominant pattern of inheritance is demonstrated. The three others show marked skeletal abnormalities similar to those seen in the Larsen Syndrome but without the usual characteristic facial features.

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Résumé Observations détaillées de quatre enfants porteurs d'anomalies squelettiques congénitales. Chez l'un d'entre eux, présentant un syndrome de Larsen typique, on a pu faire la preuve d'une transmission héréditaire autosomique dominante. Les trois autres étaient atteints d'anomalies squelettiques semblables à celles du syndrome de Larsen, mais sans les habituelles modifications caractéristiques du faciès.

     

Murray-Puretic syndrome: a report of two cases

The Murray-Puretic Syndrome is also known as juvenile hyaline fibromatosis. It is a congenital disorder characterized by skin lesions, joint contractures, gingival hypertrophy and bone lesions. Two siblings with this syndrome are presented. Received: 28 June 1996 / Accepted: 23 March 1998     

Intraosseous Phlebography,Intraosseous Pressure Measurements and 99mTc-Polyphosphate Scintigraphy in Patients with Various Painful Conditions in the hip and Knee

Twenty-five patients with pain in the knee or hip were examined by means of bilateral intraosseous phlebography, intraosseous pressure measurements and ^99mTechnetium polyphosphate scintigraphy. All patients with typical rest pain - either due to osteoarthritis or to the intraosseous engorgement-pain syndrome - showed venous stasis and increased pressure in the bone marrow near the painful joint and abnormally high uptake of the radiotracer. in patients with other types of pain this correlation was absent. the results indicate that ^99m Technetium polyphosphate scintigraphy can be used as a screening method in the diagnosis of the intraosseous engorgement-pain syndrome in patients with a typical history. However, increased isotope uptake in a joint region may be due to a variety of other causes. the identical findings with all three methods of investigation in patients with the intraosseous engorgement-pain syndrome and osteoarthritis suggest a common pathomechanism.     

Intra abdominal hypertension and the abdominal compartment syndrome

The patho-physiological significance of raised intra-abdominal pressure, known as Intra Abdominal Hypertension, with subsequent organ dysfunction and failure, known as Abdominal Compartment Syndrome, has recently been demonstrated to occur relatively frequently in mixed populations of critically ill patients. Clinical diagnosis is unreliable, so routine measurement of intra abdominal pressure should be undertaken, particularly in specific groups of patients known to be at high risk. Whilst definitive therapy requires surgical abdominal decompression, less invasive therapies have been investigated and, if initiated early, may help to minimise progression of the condition. Clearly defined indications for surgical intervention remain elusive however and require prospective investigation. This review summarises the patho-physiology of the syndrome, its diagnosis and surveillance, and current management strategies, both medical and surgical.     

Understanding patellofemoral pain with maltracking in the presence of joint laxity: Complete 3D in vivo patellofemoral and tibiofemoral kinematics

Patellofemoral pain is widely accepted as one of the most common pathologies involving the knee, yet the etiology of this pain is still an open debate. Generalized joint laxity has been associated with patellofemoral pain, but is not often discussed as a potential source of patellar maltracking. Thus, the objective of this study was to compare the complete 6 degree of freedom patellofemoral and tibiofemoral kinematics from a group of patients diagnosed with patellofemoral pain syndrome and maltracking to those from an asymptomatic population. The following null hypotheses were tested: kinematic alterations in patellofemoral maltracking are limited to the axial plane; knee joint kinematics are the same in maltrackers with and without generalized joint laxity (defined by a clinical diagnosis of Ehlers Danlos Syndrome); and no correlations exist between tibiofemoral and patellofemoral kinematics or within patellofemoral kinematics. This study demonstrated that alterations in patellofemoral kinematics, associated with patellofemoral pain, are not limited to the axial plane, minimal correlations exist between patellofemoral and tibiofemoral kinematics, and distinct subgroups likely exist within the general population of maltrackers. Being able to identify subgroups correctly within the omnibus diagnosis of patellar maltracking is a crucial step in correctly defining the pathophysiology and the eventual treatment of these patients. © 2008 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 27: 561–570, 2009     

18F-FDG PET用于急性呼吸窘迫综合征研究进展

急性呼吸窘迫综合征(ARDS)是常见呼吸危重症,早期诊断、早期治疗有利于改善患者预后。~(18)F-FDG PET可于分子水平对ARDS进行示踪,有助于量化肺组织炎症细胞募集及激活状态、监测肺组织区域代谢及炎症活动、揭示ARDS异质性病理改变,对ARDS早期诊断、病情监测、疗效评价等方面具有重要应用价值。本文对~(18)F-FDG PET用于ARDS的进展进行综述。     

Muscle atrophy at diagnosis of carpal and cubital tunnel syndrome

PURPOSE: This study was designed to test the hypothesis that patients with an initial diagnosis of cubital tunnel syndrome are more likely to present with muscle atrophy than patients with an initial diagnosis of carpal tunnel syndrome. METHODS: A list of patients presenting to the office of a single hand surgeon from January 2000 to June 2005 with an initial diagnosis of isolated, idiopathic carpal tunnel syndrome or cubital tunnel syndrome was generated from billing records. The medical records of 58 patients with cubital tunnel syndrome and 370 patients with carpal tunnel syndrome were reviewed for age, gender, diabetes, and presence of atrophy. RESULTS: Twenty-three of 58 patients with an initial diagnosis of cubital tunnel syndrome had atrophy compared with only 62 out 370 patients with an initial diagnosis of carpal tunnel syndrome. Multiple logistic regression revealed that age (odds ratio, 1.06; 95% CI, 1.04-1.08) and diagnosis (cubital tunnel patients were more likely than carpal tunnel patients to present with atrophy; odds ratio, 4.5; 95% CI, 2.7-8.6) were factors significantly associated with atrophy at presentation. CONCLUSIONS: Patients with carpal tunnel syndrome present earlier in the course of their disease than patients with cubital tunnel syndrome. Patients with cubital tunnel syndrome are more likely to present with muscle atrophy, reflecting advanced nerve damage that may not respond to surgery.     

Results of the Pregnancies with HELLP Syndrome

In this study, clinical features, developing complications, and results of thirty-six patients, which were followed up in our Obstetrics and Gynecology and Nephrology departments between 1997 and 2001, with the diagnosis of HELLP syndrome were searched retrospectively. The mean age of the cases followed up with diagnosis of HELLP syndrome were 30.2 ± 5.9 (17–46) years. HELLP syndrome was diagnosed on average in the 32.6 ± 4.8th (23–41) week of gestations. Seventy percent of the cases were with severe preeclampsia and 30% of the cases were with mild preeclampsia. Eleven cases (30%) were nullipara and twenty-five cases (70%) multipara. The average of arterial systolic blood pressure of the cases were 161.6 ± 26 mmHg, and that of diastolic blood pressure was 98.5 ± 16.8 mmHg. In thirteen cases (36%) acute renal failure (ARF), six cases (17%) placenta detachment, two cases disseminate intravascular coagulation (DIC), one case Adult Respiratory Distress Syndrome (ARDS) were developed. In seven cases (19%) intrauterine dead fetuses were detected. In twenty-three cases by cesarian section (64%), in thirteen cases by induction (36%) the pregnancies were terminated in 72 h after diagnosing HELLP syndrome. Birth weights of eleven babies (30%) were below 1500 g. Five of the eleven babies were dead in the neonatal period. Six of the thirteen patients who had ARF were given hemodialysis. Two patients died because of the development of ARF + DIC and ARDS. No predicting factors for the development of HELLP syndrome could be detected, but severe preeclampsia. Therefore we think that preeclamptic pregnancies must be followed up very closely and if HELLP syndrome develops, termination of the pregnancy would be proper as soon as possible.     

Cowden Syndrome: Serendipitous Diagnosis in Patients with Significant Breast Disease. Case Series and Literature Review

Cowden syndrome (CS) is a multi‐system disease that carries an increased lifetime risk of developing certain cancers as well as benign neoplasms. The presence of features of CS in the general unaffected population results in difficulties in the recognition and diagnosis of this condition. Early diagnosis is essential to prevent the development of malignant neoplasms, yet despite the introduction of diagnostic criteria and risk calculators, accurate diagnosis remains a challenge. We identified three patients who presented to the symptomatic breast unit of a University Teaching Hospital over a period of 12 weeks who subsequently were diagnosed with CS. In this article, we discuss their clinical presentations as well as their path to diagnosis. The short timeframe between the presentations of these patients undoubtedly expedited their diagnosis. Upon application of internationally recognized diagnostic criteria, only two out of our three patients were accurately diagnosed. The risk of breast cancer in CS is comparable with that found in Hereditary Breast and Ovarian Cancer Syndrome and while a protocol for breast screening in these patients exists in most centres, no such protocol exists for patients with CS in our institution. The recommended cancer surveillance programs for patients with CS have not been found to prolong survival, however. CS consists of a vast array of diseases that span the various specialties and the subsequent varied phenotypic presentation poses diagnostic difficulties for clinicians as emphasized in our series. Continued research is required to improve recognition and diagnosis and will hopefully result in the emergence of life prolonging strategies.     

Cauda equina syndrome without mechanical cauda compression: a report of 2 cases

Study design Case report. Objective To highlight the concept of Cauda Equina Syndrome without cauda compression. Summary of background data Cauda equina syndrome (CES) is a rare disorder. CES occurs usually due to direct physical compression of the cauda nerve roots, most often from the extruded nucleus pulposus; other rare causes reported in the literature include compression by tumour, infection, fracture, penetrating trauma, chiropractic manipulation, free epidural fat graft, postoperative haematoma, ankylosing spondylitis, metastatic invasion, schwnnoma, Paget’s disease and laminar hook compression. All these causes of CES physically compress the cauda. Chemical irritation of the root can cause radicular pain but this has not been noted to cause CES before. We present two cases of Cauda Equina Syndrome was brought on by non-physical compression of the cauda. Annular tears can induce CES by chemical irritation of the cauda by inflammatory mediators. Conclusion The idea of presenting this paper is to highlight that cauda equina syndrome can be caused by non-mechanical cauda compression; in our cases it was due to chemical irritation and caudal epidural steroid injection has produced satisfactory outcome. Awareness of non-physical cauda equina syndrome is essential to identify and to initiate appropriate treatment, and the outcome seems to be satisfactory.     

Infected palmar pits: a rare presentation of Gorlin's syndrome

This paper presents a rare hand presentation of Gorlin's Syndrome, also known as Naevoid Basal Cell Carcinoma Syndrome, which has not been reported previously. This condition is an autosomal dominant, multisystem condition whose diagnosis is important to ensure further surveillance and treatment of features of the condition not routinely dealt with by hand surgeons. However, hand surgeons should be aware of this condition and those parts of the syndrome which may present to them.     

Case report of the first Severe Acute Respiratory Syndrome patient in China: successful application of extracorporeal liver support MARS therapy in multiorgan failure possibly induced by Severe Acute Respiratory Syndrome

A previously healthy patient was transferred to our infectious department with a 9-day-history of continued fever. The patient was placed on assisted respiration support in addition to anti-viral medication. The diagnosis of SARS (Severe Acute Respiratory Syndrome) was made in view of the severe hypoxemia and the characteristic symptoms exhibited by the patient. Despite the best intensive therapy, he clinically deteriorated into multiorgan dysfunction syndrome (MODS) including additional dysfunction of kidney, liver, and heart. We initiated MARS therapy (extracorporeal liver support utilizing albumin dialysis) with intention to positively influence the organ functions in his MODS on the basis of recently published studies which suggested a positive impact of MARS in multiorgan failure secondary to respiratory illnesses and the possible influence on inflammatory mediators and cytokines. The application of 4 intermittent MARS treatments (8 h each, mean blood flow rate 180 ml/min) on 4 consecutive days resulted in an immediate improvement of clinical conditions within the treatment days. The further improvement of organ functions allowed withdrawing the patient from ventilatory support 13 days after start of MARS, and 44 days after admission he was discharged home with completely resolved organ functions and laboratory abnormalities. SARS is a severe form of the epidemic outbreak of atypical pneumonia which remains poorly defined regarding etiology and special therapy recommendations. However, the development and aggravation of this ARDS-like severe acute respiratory syndrome is pathologically associated with the systemic inflammatory response syndrome (SIRS) which may then mediate or cause MODS. To our knowledge, this is the first report of an application of MARS therapy in MODS which was probably induced by SARS in a patient in China which improved the clinical condition of the patient in multi-organ failure secondary to respiratory failure indicating that MARS might be an additional therapeutic option in multiorgan failure induced by SARS.     

Young woman with nutcracker syndrome without main clinic manifestation: Hematuria—Case report

IntroductionThe nutcracker syndrome occurs due to the compression of the left renal vein between the aorta and the superior mesenteric artery. The diagnosis of the syndrome is often difficult and under diagnosed. The main clinical manifestations are hematuria and pelvic or back pain.Presentation of caseA 25 years old female patient with severe abdominal pain in the lower abdomen without hematuria. Diagnosis with nutcracker syndrome after performing computed tomography. Presented the first episode of pain with 17 years old and was diagnosed at age 25.DiscussionIn the nutcracker syndrome anatomical changes do not generate specific symptoms, causing the disease to be underdiagnosed. In this syndrome, there is increased pressure on the left renal vein, generating several signs and symptoms, and hematuria is one of present. Our patient did not have hematuria. Because of common symptoms, it makes nutcracker syndrome difficult diagnosis, confusing with other diseases, especially with nephrolithiasis. It is usually diagnosed after exclusion of other diseases.ConclusionThe diagnosis of nutcracker syndrome is done after exclusion of other causes of chronic pelvic pain. In most cases, macroscopic and microscopic hematuria are present but was unobserved in this case. Making it more difficult diagnosis.