To explore the risk factors for Guillain-barre syndrome. Methods Case-control study design was used in 51 cases of Guillain-barre syndrome,and 51 matched controls.All of the 51 cases in this study had been examined ...To explore the risk factors for Guillain-barre syndrome. Methods Case-control study design was used in 51 cases of Guillain-barre syndrome,and 51 matched controls.All of the 51 cases in this study had been examined by electrophysiology. Serum IgG antibodies specific for C. jejuni were determined in all the subjects by ELISA. Each case and control were interviewed using an ad hoc questionnaire, including his/her demographic information,onset of the illness, their personal hygiene and so on. Results The study showed that Guillain-barre syndrome was associated with a few factors, such as polio vaccine immunization before onset of illness (OR=7.27), no hand washing after defecation and before meals (OR=6.15). Infection of C. jejuni was strongly associated with the illness (OR=9.5,P<0.001). Conclusion It is suggested that occurrence of Guillain-barre syndrome may correlate to infection of C. jejuni and poor personal hygiene in children.展开更多
BACKGROUND: Human tumor necrosis factor-like molecule 1A (hTL1A) is a strong T helper cell type 1 (Thl) co-stimulator. Guillain-Barre syndrome (GBS) is an autoimmune disorder of the nervous system, which is med...BACKGROUND: Human tumor necrosis factor-like molecule 1A (hTL1A) is a strong T helper cell type 1 (Thl) co-stimulator. Guillain-Barre syndrome (GBS) is an autoimmune disorder of the nervous system, which is mediated by Thl cells. OBJECTIVE: To determine hTL1A expression in peripheral blood T lymphocytes of acute GBS children and the effects of hTL1A on secretion of interferon-γ. DESIGN, TIME AND SETTING: A randomized, controlled, neuroimmunological in vitro study was performed at the Central Laboratory of First Hospital of Jilin University, China from November 2005 to November 2007. MATERIALS: Venous blood samples were obtained from 6 healthy donors, aged 6-12 years (all routine blood examination items were normal), and 6 additional children with acute GBS, aged 6-12 years. The GBS children fell ill within 1 week and were not treated with hormones or immunoglobulin Purified recombinant human soluble tumor necrosis factor-like molecule 1A (rhsTL1A, 1 mg/mL, relative molecular mass 22 000, 6× His tag, soluble form) was supplied by the Central Laboratory of First Hospital of Jilin University, China. METHODS: Peripheral blood mononuclear cells were isolated from healthy donors using the standard Ficoll gradient centrifugation and were incubated in 96-well culture plates. The cells were assigned to the following groups: control (2 μg/mL phytohemagglutinin), 2μg/mL phytohemagglutinin + 25, 100 and 400 ng/mL rhsTL1A. T cell proliferation was quantified using the tritiated thymidine (3H-TdR) method. Serum interferon-γ levels in acute GBS children were detected by enzyme-linked immunosorbent assay (ELISA). The ratio of hTL1A-positive T cells to CD3-positive T cells in peripheral blood of acute GBS children was determined using flow cytometry. Following in vitro pre-activation of peripheral blood mononuclear cells by 2 μg/mL phytohemagglutinin, the peripheral blood mononuclear cells were treated with 400 ng/mL exogenous rhsTLIA. Finally, peripheral blood mononuclear cell-secreted interferon-γlevels were measured by ELISA. MAIN OUTCOME MEASURES: The following parameters were measured: rhsTLIA stimulation index to stimulate proliferation of T cells; the serum interferon-γ levels in acute GBS children; the ratio of hTL1A-positive cells to CD3-positive cells; the levels of interferon-γ secreted by peripheral blood mononuclear cells in acute GBS children, as well as rhsTL1A-stimulated interferon-γ levels. RESULTS: T cell proliferation assay revealed that the stimulation index in each rhsTL1A group was greater than the control group. The stimulation index of the 400 ng/mL rhsTL1A group was the greatest. Serum interferon-γ levels in acute GBS children were significantly greater than the control group (P 〈 0.05). The ratio of hTLIA+ CD3+ T cells to CD3+ T cells in acute GBS children was significantly greater than the control group (P 〈 0.01 ). Phytohemagglutinin stimulated peripheral blood mononuclear cells to a greater extent than 400 ng/mL rhsTL1A in the acute GBS group, and the secreted interferon-γ levels were significantly increased (P 〈 0.05). CONCLUSION: In T cells pre-activated with 2 μg/mL phytohemagglutinin, proliferation was effectively increased with 400 ng/mL rhsTL1A treatment. Expression of hTLIA was increased in activated T cells from peripheral blood of acute GBS children, followed by increased interferon-γ secretion. These mechanisms are considered to be part of the pathological process that induces the secretion of inflammatory cytokines in GBS syndrome.展开更多
Rationale:To report a case of cervicobrachial variant of acute inflammatory demyelinating polyneuropathy presenting with papilledema and GQ1b positivity.Patient concern:A 35-year-old female,68 days postpartum,presente...Rationale:To report a case of cervicobrachial variant of acute inflammatory demyelinating polyneuropathy presenting with papilledema and GQ1b positivity.Patient concern:A 35-year-old female,68 days postpartum,presented with headache,vomiting,and gait difficulty in swallowing with bilateral upper limb weakness and difficulty in walking,13 days after ChAdOx1 nCoV-19 vaccination.Diagnosis:Guillain-Barre syndrome with GQ1b positivity.Intervention:Five cycles of plasmapheresis were given.Outcome:The patient’s clinical condition improved.Palatal weakness improved and she could walk without support.There were mild sensory symptoms involving upper limbs which gradually improved.Lessons:AIDP should be considered in case of weakness following ChAdOx1 nCoV-19 vaccination.Albumino-cytological dissociation and anti-GQ1b positivity are needed to confirmed the diagnosis.展开更多
BACKGROUND: Guillain-Barre syndrome (GBS) is an autoimmune disease which is characterized by demyelination of peripheral nerve and nerve root, and inflammatory reaction of lymphocyte and macrophage. Neuroelectrophy...BACKGROUND: Guillain-Barre syndrome (GBS) is an autoimmune disease which is characterized by demyelination of peripheral nerve and nerve root, and inflammatory reaction of lymphocyte and macrophage. Neuroelectrophysiological examination and cerebrospinal fluid (CSF) analysis are of significance for its diagnosis. OBJECTIVE: To study the association of neuroelectrophysiology and cerebrospinal fluid immunoglobulin (CSF-lg) with pathogenetic conditions of patients with GBS. DESIGN: Case control study SETTING: Department of Neurology, Shenzhen Municipal Shekou Group Hospital; Department of Neuroelectrophysiology, People's Hospital of Guangdong Province. PARTICIPANTS: A total of 32 GBS patients including 18 males and 14 females who aged from 17 to 72 years were selected as experimental group from the Department of Neurology, People's Hospital of Guang- dong Province from January 2004 to December 2005. All cases conformed with GBS diagnostic criteria established by Asbury in 1990 and they were divided into three types according to neurological criteria established by Chinese Neurology and Psychology Journal in 1993: mild, moderate and severe types. Another 30 patients with vascular headache were selected as control group from the same hospital including 14 males and 16 females who aged from 17 to 79 years. METHODS: ① Neuroelectrophysiological examination: Multiple-functional electromyography device provided by Nicolet Company, USA was used to measure nerve conduction velocity (NCV), including motor nerve conduction velocity (MCV) and sensory nerve conduction velocity (SCV); meanwhile, electromyologram (EMG), somatosensory evoked potential (SEP) and electroencephalogram (EEG) were also measured. ② Detection of CSF-lg: Concentrations of IgG, IgA and IgM were measured with immunofixation electrophoresis. ③Follow-up: Among 32 GBS patients, 14 cases received follow-up after treatment and the longest fol- low-up time was 1 year after onset. Among them, 8 cases were reexaminined with neuroelectrophysiological and CSF examinations. MAIN OUTCOME MEASURES: Results of NCV, EMG, SEP and EEG; comparison of CSF-lg content; results of follow-up examinations. RESULTS: All 32 GBS cases and 30 patients with vascular headache were involved in the final analysis. ① Abnormal rate of neuroelectrophysiological test: 75% of NCV, 88% of F-wave, 53% of MCV, 25% of SEP, 47% of EMG and 31% of EEG. There were no significant differences among various types (P 〉 0.05). ② Results of CSF-lg test: There were no significant differences among various types (P 〉 0.05); however, abnormalities in experimental group was higher than those in control group (P 〈 0.01). CONCLUSION : Results of follow-up study suggest that improvement of clinical symptom is earlier than neuroelectrophysiological recovery; MCV and EMG recoveries are faster than that of NCV; the earlier the abnormality of EMG, the poorer the recovery. CSF4g recovers normally along improvement of clinical symptoms. It is of significance for neuroelectrophysiology and abnormality of CSF-Ig to determine degree of peripheral nerve demyelination and prognosis.展开更多
The severe acute respiratory syndrome coronavirus 2 infection typically presents with respiratory symptoms. Additionally, there are a number of less frequent neurological manifestations of infection with the coronavir...The severe acute respiratory syndrome coronavirus 2 infection typically presents with respiratory symptoms. Additionally, there are a number of less frequent neurological manifestations of infection with the coronavirus disease 2019 (COVID-19) with case reports suggesting an association with the Guillain-Barre syndrome. Most patients present with the typical upper respiratory symptoms in association with these neurological symptoms. We present a case of an unvaccinated gentleman with none of the typical respiratory symptoms of COVID-19 who presented with the Guillain Barre syndrome and myalgia. His symptoms settled following treatment with intravenous immunoglobulins. This case highlights the importance of testing for COVID-19 in patients without typical symptoms but who present with neurological illness and supports the use of intravenous immunoglobulin therapy.展开更多
BACKGROUND Guillain-Barrésyndrome(GBS)is an autoimmune-mediated peripheral neuropathy characterized by symmetric weakness.Asymmetric weakness in GBS is uncommon and may be easily confused with other differential ...BACKGROUND Guillain-Barrésyndrome(GBS)is an autoimmune-mediated peripheral neuropathy characterized by symmetric weakness.Asymmetric weakness in GBS is uncommon and may be easily confused with other differential diagnoses.We herein present three cases of asymmetric GBS and review the literature on this atypical subtype of GBS in order to describe the characteristics of asymmetric GBS and to provide experience for clinicians.CASE SUMMARY Different from patients in the previous reports,our patients showed persistent asymmetric limb weakness from the onset to recovery phase.All three patients were serologically positive for antecedent infections.Two of the three cases had IgG antibodies against ganglioside GM1.Two patients received immunotherapy including intravenous immunoglobulin and plasma exchange,while one patient received only supportive treatment.Autoantibodies against gangliosides,asymmetry of congenital development of blood-nerve barrier and limb use may contribute to the development of asymmetric limb weakness in GBS.CONCLUSION Asymmetric GBS may be a rare clinical variant and should be considered when a patient develops acute and progressive asymmetric limb weakness.The differences in clinical features and prognosis between asymmetric GBS and classic GBS deserve further investigation in a large study.展开更多
The recommended therapy for chronic hepatitis C (CHC) infection is the combination of a Pegylated interferon and Ribavirin. Almost all such patients on combination therapy experience one or more adverse events during ...The recommended therapy for chronic hepatitis C (CHC) infection is the combination of a Pegylated interferon and Ribavirin. Almost all such patients on combination therapy experience one or more adverse events during the course of treatment. Significant neurological side effects are rare. A few cases of Bell's Palsy, chronic inflammatory demyelinating polyneuropathy and even one case of acute demyelinating polyneuropathy with atypical features for Guillain-Barre syndrome (GBS) associated with Interferon therapy have been reported but no report of GBS with typical features has been published. We present a case report of typical GBS associated with Peginterferon alfa-2a and Ribavirin used for treatment of CHC infection.展开更多
Background: Guillain-Barre syndrome (GBS) is an autoimmune disorder characterized by a heterogeneous group of pathological and clinical entities. It is associated with ascending areflexic paralysis, some autonomic dys...Background: Guillain-Barre syndrome (GBS) is an autoimmune disorder characterized by a heterogeneous group of pathological and clinical entities. It is associated with ascending areflexic paralysis, some autonomic dysfunction and respiratory failure in severe cases and ultimately death if not promptly diagnosed and treated. It may be preceded by an antecedent event in about two-third of cases. This could be an upper respiratory tract infection, viral illness, recent history of vaccination, pregnancy, cancer or even trauma. The condition is exceedingly rare in pregnancy and only few cases have been reported in literature. Case Report: This is a case of a 28-year-old Gravida 3, Para 1+1 and Estimated Gestational Age of 30 weeks and 4 days. There was a history of upper respiratory tract infection eight weeks prior to presentation which spontaneously resolved. On examination, she was a young woman, anxious, weak, afebrile, not pale, the neck could not hold the head upright and there was bilateral non tender pitting pedal oedema extending to her mid-shin. There were no cranial nerve deficits and no sign of meningeal irritation. There were normal muscle bulk with global hypotonia and flaccid quadriparesis, Power was 3/5. The proximal groups of muscles were more affected than the distal parts. Reflexes were diminished globally with plantar flexor response. She had immunoglobulin as treatment. Conclusion: In a low resource setting like ours it is important to have a high index of suspicion of GBS when an apparently healthy gravid woman presents with progressive weakness of the limbs.展开更多
Background: The association of Guillain-Barre syndrome and cerebral sinus thrombosis is uncommon. Case Presentation: We report a 37-year-old patient hospitalized in medical ICU for respiratory distress following a Gui...Background: The association of Guillain-Barre syndrome and cerebral sinus thrombosis is uncommon. Case Presentation: We report a 37-year-old patient hospitalized in medical ICU for respiratory distress following a Guillain-Barre syndrome. He had symptomatic treatment in addition to plasma exchange. In the presence of clonic movements, a brain venography magnetic resonance showed a thrombophlebitis of the left lateral sinus, and hence a low-molecular-weight heparin treatment was begun. Immunological, thrombophilia and serological tests were negative. After a favorable evolution, he was transferred to the neurology department. Conclusion: The combination of a Guillain-Barre syndrome and a cerebral sinus thrombosis would suggest a common process. A rigorous investigation, including the use of imaging, is necessary in front of any unusual clinical sign during a GBS.展开更多
Guillain-Barre syndrome(GBS)-associated achalasia is a very rare disease of uncertain cause. We report the case of a patient diagnosed with GBS-associated type Ⅰ achalasia who was successfully treated with peroral en...Guillain-Barre syndrome(GBS)-associated achalasia is a very rare disease of uncertain cause. We report the case of a patient diagnosed with GBS-associated type Ⅰ achalasia who was successfully treated with peroral endoscopic myotomy(POEM). A 30-year-old man who was diagnosed with GBS 3 mo before was referred to our department with dysphagia and mealrelated regurgitation. The results of esophagography, endoscopy, and high-resolution manometry(HRM) revealed type Ⅰ achalasia. POEM that utilized a submucosal tunneling technique was performed to treat the GBS-associated type Ⅰ achalasia. After POEM, smooth passage of a contrast agent into the stomach was shown in follow-up esophagography, and followup HRM revealed a decrease in the mean integrated relaxation pressure 22.9 mm Hg to 9.6 mm Hg. The patient remained without dysphagia for 7 mo, even though the patient's neurological problems were not fully resolved. POEM may be a safe and effective treatment for GBS-associated type Ⅰ achalasia.展开更多
Guillain⁃Barré syndrome (GBS) is an immune-mediated peripheral neuropathy with acute or subacute onset of flaccid paralysis of the limbs with symmetrical hypesthesia and autonomic nerve involvement [1]. The clini...Guillain⁃Barré syndrome (GBS) is an immune-mediated peripheral neuropathy with acute or subacute onset of flaccid paralysis of the limbs with symmetrical hypesthesia and autonomic nerve involvement [1]. The clinical manifestations of autonomic nerve damage are complex and varied, which may involve extensive or limited autonomic function damage, including abnormalities of the skin, pupil, urinary tract, gastrointestinal tract, cardiovascular system, body temperature, lacrimal and salivary glands, and sexual function, etc. [2], and some patients may even have autonomic nerve damage as the only symptom, which is a variant of GBS and is prone to misdiagnosis or underdiagnosis. Recurrence of GBS is rare, and the manifestations of recurrence are often similar to those of the first symptoms [3], but the patient admitted to our hospital had syncope as the main clinical manifestation of recurrence, which was completely different from that of the first incidence, and syncope is not a common and typical clinical manifestation of GBS, so misdiagnosis is highly likely.展开更多
BACKGROUND The role of diverse antibodies in mediating peripheral nerve injury in Guillain-Barrésyndrome(GBS)is becoming clearer,but positivity for multiple antibodies in one case is uncommon.To our knowledge,thi...BACKGROUND The role of diverse antibodies in mediating peripheral nerve injury in Guillain-Barrésyndrome(GBS)is becoming clearer,but positivity for multiple antibodies in one case is uncommon.To our knowledge,this is the first case involving GBS with positive anti-sulfatide,anti-GT1a,and anti-GT1b antibodies.CASE SUMMARY A 20-year-old female patient was admitted to the hospital due to weakness of limbs for 5 d,and deterioration of the weakness and muscle aches for 1 d.The patient's limbs were weak,but the tendon reflexes in the part of the limbs were normal.There was no comorbid peripheral nociception or deep sensory dysfunction.She was diagnosed with GBS and was discharged after receiving intravenous human immunoglobulin pulse therapy.CONCLUSION In this article,the clinical manifestations,neurophysiological examination,and auxiliary examination findings of a GBS patient positive for multiple antibodies were analyzed to improve the identification of the disease by clinical physicians at an early stage.展开更多
Forty cases of Guillain-Barre Syndrome were reported, (GBS) which came from the regions where 'Chinese Paralytic Syndrome' and 'Acute Motor Axonal Neuropathy (AMAN)' were presented before. A study on t...Forty cases of Guillain-Barre Syndrome were reported, (GBS) which came from the regions where 'Chinese Paralytic Syndrome' and 'Acute Motor Axonal Neuropathy (AMAN)' were presented before. A study on their clinical, electrophysiological and pathological features was conducted, records on 3 733 GBS cases reported in northern China from 1978 to 1992 were reviewed and compared with cases of 'acute moter axonal nouropathy' . The results showed that the clinical, electrophysiological and pathological features of GBS in north China are similar to those of typical GBS cases in western countries, though there seemed to be some special epidemiological features in age, seasonal and regional distribution. It was concluded that the GBS in northern China is de-myelinating GBS dominantly, not a new entity.展开更多
Familial amyloid cardiomyopathy is a challenging condition that mimics many other diseases, particularly in patients with pronounced neurological presentations and unexplained or equivocal cardiac abnormalities. In th...Familial amyloid cardiomyopathy is a challenging condition that mimics many other diseases, particularly in patients with pronounced neurological presentations and unexplained or equivocal cardiac abnormalities. In this case, a 57-year-old man was admitted for outpatient cardiological evaluation of progressive right heart failure and limb paraesthesias. The patient presented with hypertension, chronic Guillain-Barre syndrome, and sick sinus syndrome. Transthoracic echocardiograms showed a thickened ventricular wall and enlarged atrium. Tissue Doppler showed a restrictive filling pattern. Transthyretin (TTR)-associated amyloidosis, which was revealed by abdominal fat-pad biopsy and DNA analysis, explained the concurrence of independent pathological features, including neuropathy and cardiac involvement. Genetic testing identified a G 〉 T mutation in exon 4 of the transthyretin (TTR) gene. This mutation resulted in the alanine-to-serine substitution at amino acid position 117. Moreover, genetic testing confirmed that the patient's asymptomatic son carried the same amyloidogenic TTR mutation. Given these findings, the diagnosis of familial amyloid cardiomyopathy, which was misdiagnosed as chronic Guillain-Barre syndrome, was proposed.展开更多
Aim:The aim was to investigate the electro-physiological characteristics in disease progression of Guillain-Barre syndrome(GBS)and observe the effect of conduction block(CB)in classification and severity of the diseas...Aim:The aim was to investigate the electro-physiological characteristics in disease progression of Guillain-Barre syndrome(GBS)and observe the effect of conduction block(CB)in classification and severity of the disease.Methods:Two hundred and ninety-four patients with GBS were divided into acute inflammatory demyelinating poly-neuropathy(AIDP)group,acute motor axonal neuropathy(AMAN)group and equivocal group according to their electro-physiological results and then reclassificated after electro-physiological review.All of the patients were followed for 6 months since their attacks.Results:Bad prognosis is more pronounced in AMAN group than in AIDP group(P<0.05).Most of the patients classificated as AIDP transformed into AMAN when CB occurred in the early phase of the disease.There is a positive relationship between CB in the early phase of the disease and severity of illness(P<0.05),but CB showed no correlation with prognosis of the patients(P>0.05).Conclusion:CB in the early phase of GBS indicates the probability of AIDP transforming into AMAN;it suggests that patients with CB in the early phase of the disease might be in serious conditions in a certain extent.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
Fecal culture of Campylobacter jejuni was prepared by the method of Skirrow, and serum class specific antibodies (IgG.IgM and IgA)to Campylobacter jejuni and class specific antibodies(IgG IgM)to GMI were prepared wit...Fecal culture of Campylobacter jejuni was prepared by the method of Skirrow, and serum class specific antibodies (IgG.IgM and IgA)to Campylobacter jejuni and class specific antibodies(IgG IgM)to GMI were prepared with solid phase enzyme linked immunasorbent assay in 16 cases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
基金the Rockefeller Foundation in the United States (Grant no. HS 9430).
文摘To explore the risk factors for Guillain-barre syndrome. Methods Case-control study design was used in 51 cases of Guillain-barre syndrome,and 51 matched controls.All of the 51 cases in this study had been examined by electrophysiology. Serum IgG antibodies specific for C. jejuni were determined in all the subjects by ELISA. Each case and control were interviewed using an ad hoc questionnaire, including his/her demographic information,onset of the illness, their personal hygiene and so on. Results The study showed that Guillain-barre syndrome was associated with a few factors, such as polio vaccine immunization before onset of illness (OR=7.27), no hand washing after defecation and before meals (OR=6.15). Infection of C. jejuni was strongly associated with the illness (OR=9.5,P<0.001). Conclusion It is suggested that occurrence of Guillain-barre syndrome may correlate to infection of C. jejuni and poor personal hygiene in children.
基金Supported by:the Program of the Key Laboratory of Health Department of Jilin Province, No.2006079the Fortieth National Post-Doctoral Scientific Foundation,No. 20060400893
文摘BACKGROUND: Human tumor necrosis factor-like molecule 1A (hTL1A) is a strong T helper cell type 1 (Thl) co-stimulator. Guillain-Barre syndrome (GBS) is an autoimmune disorder of the nervous system, which is mediated by Thl cells. OBJECTIVE: To determine hTL1A expression in peripheral blood T lymphocytes of acute GBS children and the effects of hTL1A on secretion of interferon-γ. DESIGN, TIME AND SETTING: A randomized, controlled, neuroimmunological in vitro study was performed at the Central Laboratory of First Hospital of Jilin University, China from November 2005 to November 2007. MATERIALS: Venous blood samples were obtained from 6 healthy donors, aged 6-12 years (all routine blood examination items were normal), and 6 additional children with acute GBS, aged 6-12 years. The GBS children fell ill within 1 week and were not treated with hormones or immunoglobulin Purified recombinant human soluble tumor necrosis factor-like molecule 1A (rhsTL1A, 1 mg/mL, relative molecular mass 22 000, 6× His tag, soluble form) was supplied by the Central Laboratory of First Hospital of Jilin University, China. METHODS: Peripheral blood mononuclear cells were isolated from healthy donors using the standard Ficoll gradient centrifugation and were incubated in 96-well culture plates. The cells were assigned to the following groups: control (2 μg/mL phytohemagglutinin), 2μg/mL phytohemagglutinin + 25, 100 and 400 ng/mL rhsTL1A. T cell proliferation was quantified using the tritiated thymidine (3H-TdR) method. Serum interferon-γ levels in acute GBS children were detected by enzyme-linked immunosorbent assay (ELISA). The ratio of hTL1A-positive T cells to CD3-positive T cells in peripheral blood of acute GBS children was determined using flow cytometry. Following in vitro pre-activation of peripheral blood mononuclear cells by 2 μg/mL phytohemagglutinin, the peripheral blood mononuclear cells were treated with 400 ng/mL exogenous rhsTLIA. Finally, peripheral blood mononuclear cell-secreted interferon-γlevels were measured by ELISA. MAIN OUTCOME MEASURES: The following parameters were measured: rhsTLIA stimulation index to stimulate proliferation of T cells; the serum interferon-γ levels in acute GBS children; the ratio of hTL1A-positive cells to CD3-positive cells; the levels of interferon-γ secreted by peripheral blood mononuclear cells in acute GBS children, as well as rhsTL1A-stimulated interferon-γ levels. RESULTS: T cell proliferation assay revealed that the stimulation index in each rhsTL1A group was greater than the control group. The stimulation index of the 400 ng/mL rhsTL1A group was the greatest. Serum interferon-γ levels in acute GBS children were significantly greater than the control group (P 〈 0.05). The ratio of hTLIA+ CD3+ T cells to CD3+ T cells in acute GBS children was significantly greater than the control group (P 〈 0.01 ). Phytohemagglutinin stimulated peripheral blood mononuclear cells to a greater extent than 400 ng/mL rhsTL1A in the acute GBS group, and the secreted interferon-γ levels were significantly increased (P 〈 0.05). CONCLUSION: In T cells pre-activated with 2 μg/mL phytohemagglutinin, proliferation was effectively increased with 400 ng/mL rhsTL1A treatment. Expression of hTLIA was increased in activated T cells from peripheral blood of acute GBS children, followed by increased interferon-γ secretion. These mechanisms are considered to be part of the pathological process that induces the secretion of inflammatory cytokines in GBS syndrome.
文摘Rationale:To report a case of cervicobrachial variant of acute inflammatory demyelinating polyneuropathy presenting with papilledema and GQ1b positivity.Patient concern:A 35-year-old female,68 days postpartum,presented with headache,vomiting,and gait difficulty in swallowing with bilateral upper limb weakness and difficulty in walking,13 days after ChAdOx1 nCoV-19 vaccination.Diagnosis:Guillain-Barre syndrome with GQ1b positivity.Intervention:Five cycles of plasmapheresis were given.Outcome:The patient’s clinical condition improved.Palatal weakness improved and she could walk without support.There were mild sensory symptoms involving upper limbs which gradually improved.Lessons:AIDP should be considered in case of weakness following ChAdOx1 nCoV-19 vaccination.Albumino-cytological dissociation and anti-GQ1b positivity are needed to confirmed the diagnosis.
文摘BACKGROUND: Guillain-Barre syndrome (GBS) is an autoimmune disease which is characterized by demyelination of peripheral nerve and nerve root, and inflammatory reaction of lymphocyte and macrophage. Neuroelectrophysiological examination and cerebrospinal fluid (CSF) analysis are of significance for its diagnosis. OBJECTIVE: To study the association of neuroelectrophysiology and cerebrospinal fluid immunoglobulin (CSF-lg) with pathogenetic conditions of patients with GBS. DESIGN: Case control study SETTING: Department of Neurology, Shenzhen Municipal Shekou Group Hospital; Department of Neuroelectrophysiology, People's Hospital of Guangdong Province. PARTICIPANTS: A total of 32 GBS patients including 18 males and 14 females who aged from 17 to 72 years were selected as experimental group from the Department of Neurology, People's Hospital of Guang- dong Province from January 2004 to December 2005. All cases conformed with GBS diagnostic criteria established by Asbury in 1990 and they were divided into three types according to neurological criteria established by Chinese Neurology and Psychology Journal in 1993: mild, moderate and severe types. Another 30 patients with vascular headache were selected as control group from the same hospital including 14 males and 16 females who aged from 17 to 79 years. METHODS: ① Neuroelectrophysiological examination: Multiple-functional electromyography device provided by Nicolet Company, USA was used to measure nerve conduction velocity (NCV), including motor nerve conduction velocity (MCV) and sensory nerve conduction velocity (SCV); meanwhile, electromyologram (EMG), somatosensory evoked potential (SEP) and electroencephalogram (EEG) were also measured. ② Detection of CSF-lg: Concentrations of IgG, IgA and IgM were measured with immunofixation electrophoresis. ③Follow-up: Among 32 GBS patients, 14 cases received follow-up after treatment and the longest fol- low-up time was 1 year after onset. Among them, 8 cases were reexaminined with neuroelectrophysiological and CSF examinations. MAIN OUTCOME MEASURES: Results of NCV, EMG, SEP and EEG; comparison of CSF-lg content; results of follow-up examinations. RESULTS: All 32 GBS cases and 30 patients with vascular headache were involved in the final analysis. ① Abnormal rate of neuroelectrophysiological test: 75% of NCV, 88% of F-wave, 53% of MCV, 25% of SEP, 47% of EMG and 31% of EEG. There were no significant differences among various types (P 〉 0.05). ② Results of CSF-lg test: There were no significant differences among various types (P 〉 0.05); however, abnormalities in experimental group was higher than those in control group (P 〈 0.01). CONCLUSION : Results of follow-up study suggest that improvement of clinical symptom is earlier than neuroelectrophysiological recovery; MCV and EMG recoveries are faster than that of NCV; the earlier the abnormality of EMG, the poorer the recovery. CSF4g recovers normally along improvement of clinical symptoms. It is of significance for neuroelectrophysiology and abnormality of CSF-Ig to determine degree of peripheral nerve demyelination and prognosis.
文摘The severe acute respiratory syndrome coronavirus 2 infection typically presents with respiratory symptoms. Additionally, there are a number of less frequent neurological manifestations of infection with the coronavirus disease 2019 (COVID-19) with case reports suggesting an association with the Guillain-Barre syndrome. Most patients present with the typical upper respiratory symptoms in association with these neurological symptoms. We present a case of an unvaccinated gentleman with none of the typical respiratory symptoms of COVID-19 who presented with the Guillain Barre syndrome and myalgia. His symptoms settled following treatment with intravenous immunoglobulins. This case highlights the importance of testing for COVID-19 in patients without typical symptoms but who present with neurological illness and supports the use of intravenous immunoglobulin therapy.
基金Supported by Teacher Research Support Foundation of Jining Medical University,No.JYFC2018FKJ140Natural Science Foundation Youth Project of Shandong Province,No.ZR2020QH110.
文摘BACKGROUND Guillain-Barrésyndrome(GBS)is an autoimmune-mediated peripheral neuropathy characterized by symmetric weakness.Asymmetric weakness in GBS is uncommon and may be easily confused with other differential diagnoses.We herein present three cases of asymmetric GBS and review the literature on this atypical subtype of GBS in order to describe the characteristics of asymmetric GBS and to provide experience for clinicians.CASE SUMMARY Different from patients in the previous reports,our patients showed persistent asymmetric limb weakness from the onset to recovery phase.All three patients were serologically positive for antecedent infections.Two of the three cases had IgG antibodies against ganglioside GM1.Two patients received immunotherapy including intravenous immunoglobulin and plasma exchange,while one patient received only supportive treatment.Autoantibodies against gangliosides,asymmetry of congenital development of blood-nerve barrier and limb use may contribute to the development of asymmetric limb weakness in GBS.CONCLUSION Asymmetric GBS may be a rare clinical variant and should be considered when a patient develops acute and progressive asymmetric limb weakness.The differences in clinical features and prognosis between asymmetric GBS and classic GBS deserve further investigation in a large study.
文摘The recommended therapy for chronic hepatitis C (CHC) infection is the combination of a Pegylated interferon and Ribavirin. Almost all such patients on combination therapy experience one or more adverse events during the course of treatment. Significant neurological side effects are rare. A few cases of Bell's Palsy, chronic inflammatory demyelinating polyneuropathy and even one case of acute demyelinating polyneuropathy with atypical features for Guillain-Barre syndrome (GBS) associated with Interferon therapy have been reported but no report of GBS with typical features has been published. We present a case report of typical GBS associated with Peginterferon alfa-2a and Ribavirin used for treatment of CHC infection.
文摘Background: Guillain-Barre syndrome (GBS) is an autoimmune disorder characterized by a heterogeneous group of pathological and clinical entities. It is associated with ascending areflexic paralysis, some autonomic dysfunction and respiratory failure in severe cases and ultimately death if not promptly diagnosed and treated. It may be preceded by an antecedent event in about two-third of cases. This could be an upper respiratory tract infection, viral illness, recent history of vaccination, pregnancy, cancer or even trauma. The condition is exceedingly rare in pregnancy and only few cases have been reported in literature. Case Report: This is a case of a 28-year-old Gravida 3, Para 1+1 and Estimated Gestational Age of 30 weeks and 4 days. There was a history of upper respiratory tract infection eight weeks prior to presentation which spontaneously resolved. On examination, she was a young woman, anxious, weak, afebrile, not pale, the neck could not hold the head upright and there was bilateral non tender pitting pedal oedema extending to her mid-shin. There were no cranial nerve deficits and no sign of meningeal irritation. There were normal muscle bulk with global hypotonia and flaccid quadriparesis, Power was 3/5. The proximal groups of muscles were more affected than the distal parts. Reflexes were diminished globally with plantar flexor response. She had immunoglobulin as treatment. Conclusion: In a low resource setting like ours it is important to have a high index of suspicion of GBS when an apparently healthy gravid woman presents with progressive weakness of the limbs.
文摘Background: The association of Guillain-Barre syndrome and cerebral sinus thrombosis is uncommon. Case Presentation: We report a 37-year-old patient hospitalized in medical ICU for respiratory distress following a Guillain-Barre syndrome. He had symptomatic treatment in addition to plasma exchange. In the presence of clonic movements, a brain venography magnetic resonance showed a thrombophlebitis of the left lateral sinus, and hence a low-molecular-weight heparin treatment was begun. Immunological, thrombophilia and serological tests were negative. After a favorable evolution, he was transferred to the neurology department. Conclusion: The combination of a Guillain-Barre syndrome and a cerebral sinus thrombosis would suggest a common process. A rigorous investigation, including the use of imaging, is necessary in front of any unusual clinical sign during a GBS.
文摘Guillain-Barre syndrome(GBS)-associated achalasia is a very rare disease of uncertain cause. We report the case of a patient diagnosed with GBS-associated type Ⅰ achalasia who was successfully treated with peroral endoscopic myotomy(POEM). A 30-year-old man who was diagnosed with GBS 3 mo before was referred to our department with dysphagia and mealrelated regurgitation. The results of esophagography, endoscopy, and high-resolution manometry(HRM) revealed type Ⅰ achalasia. POEM that utilized a submucosal tunneling technique was performed to treat the GBS-associated type Ⅰ achalasia. After POEM, smooth passage of a contrast agent into the stomach was shown in follow-up esophagography, and followup HRM revealed a decrease in the mean integrated relaxation pressure 22.9 mm Hg to 9.6 mm Hg. The patient remained without dysphagia for 7 mo, even though the patient's neurological problems were not fully resolved. POEM may be a safe and effective treatment for GBS-associated type Ⅰ achalasia.
文摘Guillain⁃Barré syndrome (GBS) is an immune-mediated peripheral neuropathy with acute or subacute onset of flaccid paralysis of the limbs with symmetrical hypesthesia and autonomic nerve involvement [1]. The clinical manifestations of autonomic nerve damage are complex and varied, which may involve extensive or limited autonomic function damage, including abnormalities of the skin, pupil, urinary tract, gastrointestinal tract, cardiovascular system, body temperature, lacrimal and salivary glands, and sexual function, etc. [2], and some patients may even have autonomic nerve damage as the only symptom, which is a variant of GBS and is prone to misdiagnosis or underdiagnosis. Recurrence of GBS is rare, and the manifestations of recurrence are often similar to those of the first symptoms [3], but the patient admitted to our hospital had syncope as the main clinical manifestation of recurrence, which was completely different from that of the first incidence, and syncope is not a common and typical clinical manifestation of GBS, so misdiagnosis is highly likely.
基金The Scientific and Technological Innovation Talent Team Project of Zunyi City,No.[2022]2Guizhou Maotai Hospital Research and Talent Cultivation Funding Project,No.MTYK 2022-06.
文摘BACKGROUND The role of diverse antibodies in mediating peripheral nerve injury in Guillain-Barrésyndrome(GBS)is becoming clearer,but positivity for multiple antibodies in one case is uncommon.To our knowledge,this is the first case involving GBS with positive anti-sulfatide,anti-GT1a,and anti-GT1b antibodies.CASE SUMMARY A 20-year-old female patient was admitted to the hospital due to weakness of limbs for 5 d,and deterioration of the weakness and muscle aches for 1 d.The patient's limbs were weak,but the tendon reflexes in the part of the limbs were normal.There was no comorbid peripheral nociception or deep sensory dysfunction.She was diagnosed with GBS and was discharged after receiving intravenous human immunoglobulin pulse therapy.CONCLUSION In this article,the clinical manifestations,neurophysiological examination,and auxiliary examination findings of a GBS patient positive for multiple antibodies were analyzed to improve the identification of the disease by clinical physicians at an early stage.
文摘Forty cases of Guillain-Barre Syndrome were reported, (GBS) which came from the regions where 'Chinese Paralytic Syndrome' and 'Acute Motor Axonal Neuropathy (AMAN)' were presented before. A study on their clinical, electrophysiological and pathological features was conducted, records on 3 733 GBS cases reported in northern China from 1978 to 1992 were reviewed and compared with cases of 'acute moter axonal nouropathy' . The results showed that the clinical, electrophysiological and pathological features of GBS in north China are similar to those of typical GBS cases in western countries, though there seemed to be some special epidemiological features in age, seasonal and regional distribution. It was concluded that the GBS in northern China is de-myelinating GBS dominantly, not a new entity.
文摘Familial amyloid cardiomyopathy is a challenging condition that mimics many other diseases, particularly in patients with pronounced neurological presentations and unexplained or equivocal cardiac abnormalities. In this case, a 57-year-old man was admitted for outpatient cardiological evaluation of progressive right heart failure and limb paraesthesias. The patient presented with hypertension, chronic Guillain-Barre syndrome, and sick sinus syndrome. Transthoracic echocardiograms showed a thickened ventricular wall and enlarged atrium. Tissue Doppler showed a restrictive filling pattern. Transthyretin (TTR)-associated amyloidosis, which was revealed by abdominal fat-pad biopsy and DNA analysis, explained the concurrence of independent pathological features, including neuropathy and cardiac involvement. Genetic testing identified a G 〉 T mutation in exon 4 of the transthyretin (TTR) gene. This mutation resulted in the alanine-to-serine substitution at amino acid position 117. Moreover, genetic testing confirmed that the patient's asymptomatic son carried the same amyloidogenic TTR mutation. Given these findings, the diagnosis of familial amyloid cardiomyopathy, which was misdiagnosed as chronic Guillain-Barre syndrome, was proposed.
文摘Aim:The aim was to investigate the electro-physiological characteristics in disease progression of Guillain-Barre syndrome(GBS)and observe the effect of conduction block(CB)in classification and severity of the disease.Methods:Two hundred and ninety-four patients with GBS were divided into acute inflammatory demyelinating poly-neuropathy(AIDP)group,acute motor axonal neuropathy(AMAN)group and equivocal group according to their electro-physiological results and then reclassificated after electro-physiological review.All of the patients were followed for 6 months since their attacks.Results:Bad prognosis is more pronounced in AMAN group than in AIDP group(P<0.05).Most of the patients classificated as AIDP transformed into AMAN when CB occurred in the early phase of the disease.There is a positive relationship between CB in the early phase of the disease and severity of illness(P<0.05),but CB showed no correlation with prognosis of the patients(P>0.05).Conclusion:CB in the early phase of GBS indicates the probability of AIDP transforming into AMAN;it suggests that patients with CB in the early phase of the disease might be in serious conditions in a certain extent.
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Fecal culture of Campylobacter jejuni was prepared by the method of Skirrow, and serum class specific antibodies (IgG.IgM and IgA)to Campylobacter jejuni and class specific antibodies(IgG IgM)to GMI were prepared with solid phase enzyme linked immunasorbent assay in 16 cases
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.