Series Of Clinical Manifestations Research Articles

Postoperative Total Hypopituitarism Complicated with Refractory Gout: A Case Report

Craniopharyngioma is a benign tumor of sellar epithelial tissue. The tumor is close to the important structures of the brain and is usually surgically removed. The main postoperative complication is impaired pituitary function, resulting in a series of clinical manifestations. Postoperative panpituitarism combined with refractory gout is rare in clinical practice. The report is as follows to provide reference for improving the clinical understanding of the disease.

Giant Renal Angiomyolipoma: A Case Report

Renal Angiomyolipoma (AML), moreover known as a renal hamartoma, is a solid tumor with no malignant characteristics. The inheritance pattern of renal AML is autosomal dominant. If the lesion grows to a large size, a series of clinical manifestations and important complications might also occur. This research paper presents a case of huge renal AML in a 47-year-old lady, who visited the Emergency Department of Bahrain Defence Forces Hospital with right-sided abdominal pain of sudden onset. The patient underwent a total right nephrectomy. The resected mass was sized 10.5 x 13 x 14 cm. Postoperative histopathological examination confirmed the lesion as a huge renal AML. Due to the huge size of the tumor, it is crucial to record similar cases, along with their diagnosis and treatment. Keywords: Abdominal pain, Angiomyolipoma, Hamartoma, Kidney Neoplasms, Nephrectomy

Reperfusion syndrome: state of the art

Reperfusion syndrome is a complex series of clinical manifestations resulting from restoration of blood flow to previously ischaemic tissues. It is accompanied by damage to cells, tissues and organs at various levels, followed by the development of multiple organ failure. This review deals with the main pathophysiological mechanisms of the development of reperfusion syndrome in lesions of cardiac, cerebral and lower-limb vessels. Oxidative stress is considered to be the most important marker of ischaemia-reperfusion injury irrespective of the type of tissues affected. Presented herein are the data on contemporary possibilities of influencing various stages and components of the development of reperfusion injury by means of drug therapy, demonstrating that due to the importance of oxidative stress as a key link of reperfusion injury, antioxidant therapy should be the main component of prevention and treatment of reperfusion injury.

Internal jugular vein stenosis associated with elongated styloid process: five case reports and literature review

BackgroundInternal jugular vein stenosis (IJVS), characterized by a series of clinical manifestations, such as head and neck symptoms, visual and ear symptoms, as well as sleep disorder, has been receiving attention in recent years. However, its’ etiologies are not fully understood.Case presentationWe report a cases series of IJVS induced by styloid oppression. We define it as the stylo-jugular type of Eagle syndrome (ES).ConclusionsOur study reveals that external oppression, especially by styloid process, is an important etiology of IJVS. The stylo-jugular ES diagnosis can be identified by Computed tomography venography. Whether stylo-jugular ES can be corrected by styloidectomy requires further investigation.

New Goals and Strategies of Chinese Medicine in Prevention and Treatment of Chronic Kidney Disease.

Chronic kidney disease (CKD) is a clinical syndrome with a series of clinical manifestations and metabolic disorders caused by many diseases, which are characterized by progressive deterioration or irreversible damage of renal structures and functions. With the progress of epidemiological research, CKD has brought about huge economic and psychological burdens. There is a considerable risk of cardiovascular events or death than progression to end-stage renal disease for patients. Particular attentions should be paid to the new goals of reducing cardiovascular events and all-cause mortality. It is important to analyze the etiology and pathogenesis according to patients' ages, regions, primary disease as well as different stages of disease, and choose the appropriate therapeutic strategies accordingly. In clinical practice, due to the uncertainty of therapeutic effects of modern medicine based on the risk factors, it is necessary to use Chinese medicine (CM) to delay the disease progression and reduce comorbidities. Turbid toxin and blood stasis are two critical pathological factors worthy of concerns in the theory of CM. In addition, appropriate use of CM may help improve the quality of life of patients with CKD.

Advancements in the pathogenesis of ossification of the posterior longitudinal ligament

Ossification of the posterior longitudinal ligament (OPLL) is a degenerative disease with a series of clinical manifestations caused by compression of the spinal cord and nerve roots. The disease is prevalent in East Asian countries, and the average prevalence rate of Chinese people is 3.08%. Disease progression can lead to paraplegia, but there is no effective preven-tion and cure. The pathogenesis of OPLL is still unclear. Most scholars suggest it may be related to genes, hormones, environment, lifestyle and other factors, but all stay in the stage of hypothesis and theory. In this paper, we searched Pubmed, Embase, CNKI and Wanfang database. According to the included literatures, the pathogenesis of OPLL was divided into the following parts: extra-cellular microenvironment, intracellular cytokines, genetic factors, biomechanical factors, and endocrine and living habits. We summarized and analyzed these literatures. These existing studies independently elucidated the possible mechanisms of OPLL from different angles. Different pathogenic factors promote mesenchymal stem cells to differentiate into osteoblasts through corre-sponding molecular pathways, initiate osteogenesis, and lead to the formation of ectopic ossification. However, none of these stud-ies have clarified the complete pathogenesis of OPLL. Most of the studies focused on the field of genetics. In recent years, a large number of susceptible genes or loci related to the pathogenesis of OPLL have been screened, but they have shortcomings such as insufficient sample size, ethnic regional differences, and poor reproducibility.

The Influence of Thymectomy Over Acetylcoline Antibody Anti-receptor Concentration and Over the Clinical Manifestations on Myasthenia Gravis Patients

Myasthenia gravis is an autoimmune disorder which presents a series of clinical manifestations which are generally motor. The hallmark of the disorder is muscle weakness, which typically worsens during physical exercise and improves upon rest. Approximatively 80% of the patients presenting this affection test positive for Acetylcholine antibody anti-receptor (AChR-ab).Treatment of patients with myasthenia gravis consists of:administration of oral medica-tion, immunomodulating treatment by removal of AChR-ab through plasmapheresis or surgical intervention (thymectomy).This article presents a retrospective study in which the authors attempted to identify factors which can influence the effect of thymectomy on AChR-ab titration and on the clinical presentation of the patients suffering with myasthenia gravis.

Rabbit Model of Corneal Endothelial Injury Established Using the Nd: YAG Laser.

To develop a new rabbit model of corneal endothelial injury using a neodymium-doped yttrium aluminum garnet (Nd:YAG) laser. The corneal endothelia of 2 groups of New Zealand white rabbits were treated with an Nd:YAG laser in a uniformly scattered fashion. Rabbits in group A underwent laser burns on the whole corneal endothelium, including the limbus area, whereas rabbits in group B were subjected to laser burns in the central 9-mm diameter zone of the endothelium. Slit-lamp biomicroscopy, optical coherence tomography, applanation tonometry, confocal microscopy, scanning electron microscopy, and histological examinations were performed during 4 weeks of follow-up. In both groups, dotted or focal corneal endothelium defects were directly observed. The stroma was intact. Right after laser application, a series of clinical manifestations appeared, including subepithelial and stromal edema, increased central corneal thickness, and corneal opacity. Laser burn had more notable effects in group A than in group B. In both groups, we observed no damage to the intraocular structures, and intraocular pressure was normal after laser treatment. Nd:YAG laser treatment in a 9-mm diameter zone of the endothelium can effectively induce bullous keratopathy in a rabbit, whereas treatment for the entire corneal endothelium maintains bullous keratopathy for a longer period. The procedure is simple and reproducible, and it retains normal intraocular structures. This study provided a promising model for future research into endothelial cell damage and for the development of new therapies.

Giant renal angiomyolipoma: A case report.

Renal angiomyolipoma (AML), also referred to as renal hamartoma, is a rare solid tumor without malignant characteristics. The inheritance pattern of renal AML is autosomal dominant. If the lesion grows to a large size, a series of clinical manifestations and serious complications may occur. We herein present a case of giant renal AML in a 34-year-old female patient, who presented with left-sided abdominal bloating for ~6 months. Following abdominal ultrasound and computed tomography examination, the patient underwent total left nephrectomy. The resected mass was sized 29×20x10 cm. Postoperative histopathological examination confirmed the lesion as a giant renal AML. There are only few cases of giant renal AML (>20 cm) reported in the literature to date. Due to the large size of the tumor, it is crucial to report similar cases, their diagnosis and treatment.

Clinical manifestation and molecular mechanism of FMR1 gene premutation

Fragile X syndrome (FXS) is one of the common causes of inherited intellectual disability.It is mainly caused by the mutation of FMR1 gene which lead to (CGG) n abnormal amplification(n>200). However, the FMR1 gene premutation (n=55-200) can cause a series of clinical manifestations through an entirely distinct molecular mechanism from FXS, which involved increased FMR1 mRNA toxicity.A few years after the discovery of the FMR1 gene in 1991, it was generally believed that the carriers of premutation were clinically unaffected, but female carriers of premutation had children with FXS.Thus, a few doctors pay attention to these disorders and generally lack of awareness the difference between the premutation and FXS.A review of the clinical manifestations, neuropathology and molecular mechanisms will show in the following paper. Key words: FMR1 gene premutation; Clinical manifestation; Neuropathology; Molecular mechanism

IgG4-related retroperitoneal fibrosis: a newly characterized disease.

Retroperitoneal fibrosis (RPF) is a rare disease characterized by chronic, nonspecific inflammatory and sclerotic or fibrotic tissue in the periaortic or periiliac retroperitoneum that encases adjacent structures. There will be a series of clinical manifestations once the proliferated fibrous tissues encase the abdominal aorta, iliac arteries and urinary duct. RPF is generally divided into two types: idiopathic retroperitoneal fibrosis (IRPF) without identified pathogenesis, making up about two-thirds of cases, and secondary retroperitoneal fibrosis. Recent studies on Immunoglobulin G4-related disease (IgG4-RD) reveal that abundant infiltration of IgG4 positive plasma cells is found in biopsies on the mass of RPF of some IRPF patients, which is identified as one spectrum of IgG4-RD and is named IgG4-related RPF. IgG4-related RPF is often misdiagnosed as retroperitoneal visceral malignancy and is treated with surgery. In addition, because of its good response to glucocorticoid, early detection and treatment is important. We review the definition, epidemiology, clinical features, diagnostic criteria, treatment and prognosis of IgG4-related RPF in this article to raise awareness of this newly characterized disease.

Neurolymphomatosis: A Case Series of Clinical Manifestations, Treatments, and Outcomes (P04.187)

Objective: Our goal was to ascertain common factors to render earlier diagnosis, and prognostic factors to predict response to therapy. Background Neurolymphomatosis involves infiltration of malignant lymphocytes into the peripheral nervous system, specifically cranial nerves, roots, plexuses, and/or peripheral nerves. It is distinct from more common neurological complications related to chemotherapy, irradiation, paraneoplastic disorders, and leptomeningeal carcinomatosis. Design/Methods: We review five patients who developed neurolymphomatosis at M.D. Anderson Cancer Center between 2010 and 2011. Clinical, radiologic, and laboratory data were extracted from their medical records. Results: The average age at onset was 48 (range 26 - 76) with primary diagnosis being non-Hodgkin9s lymphoma in three (Burkitt9s, large B-cell) and leukemia in two (AML, CML). Initial symptoms included weakness (5/5), pain (3/5), and sensory change (2/5) and localized to lower extremities (3/5), upper extremities (2/5), unilateral (4/5), bilateral (1/5), and cranial nerve (1/5). Physical exam findings included weakness (5/5), sensory disturbance (5/5), atrophy (4/5), hypotonia (2/5), and hyporeflexia (5/5). Three patients demonstrated MRI abnormalities with clinical correlation, affecting neural foramina (1/5), paraspinal (2/5), or subcutaneous (1/5) regions; no patients had imaging evidence of parenchymal, spinal cord, or leptomeningeal pathology. All patients had positive EMG/NCS and diagnoses included mononeuritis multiplex, multifocal plexopathy, demyelinating polyneuropathy, and chronic axonal polyradiculoneuropathy. One patient had positive CSF cytology; PET was performed and positive in four patients and no nerve biopsies or autopsies were performed. Therapies included systemic (5/5) and intrathecal chemotherapy (4/5), immunomodulators (2/5), and plasma exchange (1/5); no patients underwent radiotherapy. Mortality was 40% with average survival from neurologic presentation 6 months (range 1–12 months); the other patients progressed despite treatment. Neither age, gender, malignancy type, recurrence, positive CSF/imaging/EMG, nor intervention correlated with worse prognosis. Conclusions: Further studies are warranted to define risk for developing neurolymphomatosis and identify factors affecting outcome. Disclosure: Dr. Gildersleeve has nothing to disclose. Dr. Hormozdi has nothing to disclose. Dr. Kamiya Matsuoka has nothing to disclose. Dr. Woodman has nothing to disclose.

Physiotherapy interventions for the management of whiplash injuries

Age dependent decrease in androgen levels is the main and most important mechanism responsible for involutional osteoporosis in men. Decreased physical activity may contribute to the development of osteoporosis. Supplementation with DHEA and increased physical activity may counteract the development of male osteoporosis and decrease fracture risk. DHEA may increase bone formation after conversion to testosterone, and exercise — by activation of the canonical Wnt/β-catenin pathway. The aim of the present study was to examine the effect of DHEA and moderate exercise on mechanical properties of long bones in rats with androgen deficiency induced by orchidectomy. The experiments were carried out on 3-month-old Wistar rats, divided into following groups (n=8–10 rats per group): non-orchidectomized control rats, orchidectomized (ORX) control rats, ORX rats receiving DHEA, ORX rats submitted to exercise, ORX rats receiving DHEA submitted to exercise. The rats were submitted to the moderate exercise on the treadmill once daily (1 hour) for 8 weeks. DHEA was administered at a dose 7 mg/kg p.o. once daily for 8 weeks. Mechanical properties of tibial metaphysis and femoral diaphysis (in three-point bending tests) and femoral neck (in a compression test), bone turnover markers (serum osteocalcin and C-terminal telopeptide of type I collagen fragments), geometric parameters, mass, and mass of bone mineral in the tibia and femur were studied. Androgen deficiency inhibited bone formation and resorption. It weakened mechanical properties of tibial metaphysis as well as femoral neck and diaphysis, and decreased bone mass and mass of bone mineral. Exercise increased bone formation and counteracted unfavorable changes in mechanical properties of tibial metaphysis and femoral neck, induced by androgen deficiency. DHEA increased bone formation and significantly inhibited the effect of androgen deficiency on the mechanical properties of the tibial metaphysis. Administration of DHAE combined with exercise counteracted unfavorable changes in bone mechanical properties (especially in the tibial metaphysis) induced by androgen deficiency. This article is part of a Special Issue entitled ECTS 2011. Disclosure of interest: None declared. doi:10.1016/j.bone.2011.03.670 PP540-T Physiotherapy interventions for the management of whiplash injuries V. Barkatsa ⁎, C. Iliadis , V. Moraitis , N. Syrmos , M. Tzagarakis , K. Tsipras d a Physiotherapy, Greece b Neurosurgery, Venizeleion General Hospital, Heraklion, Greece c Pathology, General Hospital, Rethymno, Greece d Endocrinology, Greek Institute of Social Security, Athens, Greece Abstract: Introduction/background: Whiplash injuries are associated with motor Introduction/background: Whiplash injuries are associated with motor vehicle accidents or in sporting mishaps after an acceleration–deceleration mechanism of energy transfer to the neck. The injury results in a series of clinical manifestations (mainly cervical pain 88% and headaches) known as Whiplash Associated Disorder. The syndrome involves trauma to amultiplicity of tissues in the cervical spine and it may affect other areas of the vertebral column. Clinicians are challenged to use different treatments in order to relieve symptoms and to improve body function. Method: systematic review of the effectiveness of physiotherapy interventions in the management of whiplash injuries using MEDLINE, CINAHL, Cochrane database systematic reviews and published guidelines. Results: In the acute stage (0 to 2 weeks after injury) physiotherapy interventions recommended for pain reduction are active exercises, manual mobilisation, use of TENS, education and advice and relaxation techniques compared to electrotherapy, traction, and the use of soft collar. There is insufficient evidence to support the use of massage and acupuncture. In the sub acute stage (2–12 weeks after injury) and in the chronic stage( after 12 weeks) a multimodal programme including active exercises, postural training, manual techniques, physical agents and psychosocial support should be used to reduce pain and enable people to return to normal activities. Conclusion: Recovery is improved by early active management and early return to pre-accidental activities. This article is part of a Special Issue entitled ECTS 2011.

Anti-Ro antibody and clinical manifestations: lessons from systemic lupus erythematosus in the elderly

Dear Editor, As we know, previous studies have shown the association of systemic lupus erythematosus (SLE) and anti-Ro antibody with a series of clinical manifestations. However, available evidence suggests that there still exist a large number of inconsistencies. Recently, Paz et al. reported that there was no direct relationship between anti-Ro antibody and cutaneous photosensitivity in SLE patients [1]. Another study also found that anti-Ro antibody was not associated to photosensitivity. However, there was an association with anti-La antibody. Moreover, in multivariate analysis, patients with anti-Ro antibody has 1.63 (95% CI, 1.07–2.50) more risk to develop cutaneous vasculitis than patients without this antibody [2]. Interestingly, Shinjo et al. showed that SLE cutaneous vasculitis group was not associated to anti-Ro antibody, but the group had a higher frequency of photosensitivity. Additionally, the authors also found that had a higher frequency of Raynaud phenomenon in cutaneous vasculitis group [3]. Clinical manifestation in late onset SLE may provide a good reference for this disagreement. Rovenský et al. systematically reviewed clinical manifestation for SLE in the elderly and found that late onset SLE patients manifested higher rate of positive findings of anti-Ro and anti-La antibodies. In contrast, skin vasculitis, photosensitivity and Raynaud phenomenon occured rarely in the elderly patients [4]. The review may prove that anti-Ro antibody is associated to prevalence of anti-La antibody but not photosensitivity, cutaneous vasculitis and Raynaud phenomenon, which is in agreement with most findings from the reports discussed above [1–3]. It should be noted that further studies are required to clarify precise molecular mechanism. In spite of this, clinical manifestation in late onset will provide a better understanding for the role of anti-Ro antibody in SLE.

Anti-Ro antibody and cutaneous vasculitis in systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a complex systemic autoimmune disease with various clinical and serological manifestations. Previous studies have shown the association of SLE and anti-Ro antibody with a series of clinical manifestations. We investigated this association in Brazilian patients with SLE. Five hundred and nine consecutive patients who fulfilled the revised American College of Rheumatology criteria for the SLE were enrolled in the study from June to December 2007. All patients were from our Service of Rheumatology, School of Medicine, University of São Paulo, Brazil. Frequencies of a series of laboratorial and clinical manifestations were calculated. Anti-Ro antibody was associated to anti-La antibody, female, and cutaneous vasculitis. In multivariate analysis, patients with anti-Ro antibody has 1.63 (95% CI 1.07-2.50) more risk to develop cutaneous vasculitis than patients without this antibody. Our data have demonstrated that anti-Ro antibody is an independent useful serologic marker for cutaneous vasculitis.