Peyrí Rey, E; Riverola Manzanilla, A; Cañas Tello, M A
A rare case of asymtomatic synchronous bilateral granulomatous orchitis idiopathic is decribed. In the scrotal ultrasonography are multiple hypoecoic areas, differential diagnosis between testicular tumor and granulomatous orchitis is very difficult in any examination by histological findings.
Alfonso, E.; Arrellanes, L.; Boruchoff, S. A.; Ormerod, L. D.; Albert, D. M.
A 52-year-old Mexican man presented with asymptomatic, bilaterally symmetrical lipid infiltrates of the cornea and adjacent limbus. No evidence of previous ocular disease or systemic disorder of lipid metabolism could be detected. Penetrating keratoplasty of the right eye was required. The cornea was rigid and thick, with posterior bulging into the anterior chamber. Light microscopy revealed deep corneal lipid granules, foamy histiocytes, vascularisation, and chronic non-granulomatous inflammation. Transmission electron microscopy showed extracellular lipid spaces and numerous intracytoplasmic lipid vacuoles in histiocytes, keratocytes, conjunctival epithelium, and the endothelium of blood vessels in the corneal stroma and adjacent limbal conjunctiva. Histochemical analysis revealed the presence of neutral fats, free fatty acids, cholesterol, and phospholipids. Images PMID:3395592
Grace, Mary; Balachandran, Venu; Menon, Sooraj
Idiopathic central diabetes insipidus (CDI) is a rare disorder characterized clinically by polyuria and polydipsia, and an abnormal urinary concentration without any identified etiology. We report a case of central diabetes insipidus in a 60-year-old lady in the absence of secondary causes like trauma, infection, and infiltrative disorders of brain.
Amsallem, L; Serane, J; Zbili, D; Marion, B; Boyer, P
We report on the case of bilateral avascular osteonecrosis (AVN) of the lunate and triquetrum in a 45-year-old woman without any relevant medical history for which the cause could not be established. She was treated conservatively with splinting and analgesics. The patient was able to pursue her work and hobbies with subnormal range of motion at 1-year follow-up. AVN of the triquetrum is very rare, as it is a richly vascularized bone. The presence of AVN in multiple carpal bones has only been described in patients receiving high doses of corticosteroids. To our knowledge, there are no other cases of idiopathic AVN of multiple carpal bones in the literature.
Borie, Eduardo; Watanabe, Plauto C.A.; Orsi, Iara A.; Fuentes, Ramón
INTRODUCTION Exostoses in paranasal sinuses have been reported in the otolaryngology literature, but they have not been described in the dental literature to our knowledge. The aim of this article is to describe an idiopathic and rare case of bilateral exostosis obtained by cone-beam computed tomography. PRESENTATION OF CASE The case shows a healthy and asymptomatic patient with a different size and form of exostoses in both maxillary sinuses. DISCUSSION It is difficult to clinically diagnose the antral exostosis due the asymptomatic nature of this condition, unless the approach would be through endoscope. Sometimes this condition is related with nasal irrigants, however in this case the patient asserted not having used nasal irrigation ever; thus, it is impossible to relate this kind of treatment as a principal cause. CONCLUSION The published data of exostoses in maxillary sinus seem to be limited in the dental literature, and this condition is important to consider in an implant treatment planning. Also, it is important to perform a follow-up of the cases in trying to find the possible causes of exostosis. PMID:25128728
Idiopathic hypersomnia of the central nervous system is a cause of excessive diurnal somnolence which affects 5-10% of the patients who attend sleep clinics for this reason. We describe three male patients who consulted for excessive diurnal somnolence. Nocturnal polysomnographic studies followed by tests for multiple latencies of sleep were done. In all cases there was confirmation of lengthening of the time of nocturnal sleep with normal phases of sleep and an increase in the number of sleep spindles in phase II. Similarly there was an average latency of sleep of less than 10 minutes and fewer than two phases of REM in the multiple latencies test. All patients improved with drugs stimulating vigil, two of them with centramine and the third with methilphenidate. We consider the clinical data the polysomnographic criteria which help to establish the diagnosis.
Agrawal, Amit; Shrestha, Rabin
Tic disorders are commonly considered to be childhood syndromes. Newly presenting tic disorders during adulthood are uncommon and mostly described in relation to an acquired brain lesion or as incidental tics, particularly in context with other neurological or psychiatric diseases. Tic disorder involving the ears is extremely uncommon with only few studies in English literature. In the present case, we describe an adult patient with new-onset idiopathic tics disorder involving both ears, causing social embarrassment. In addition, our patient had recent onset of the tics without any childhood or family history of tic disorders. The single most important component of management is an accurate diagnosis. At the same time, tics should be differentiated from other movement disorders such as chorea, stereotypy, and dystonias.
Ruane, Laurence E; Lau, Kenneth K; Low, Kathy; Crossett, Marcus; Vallance, Neil; Bardin, Philip G
Idiopathic bilateral vocal cord paralysis (VCP) is a rare and difficult condition often undiagnosed and frequently confused with asthma and other respiratory conditions. Accurate diagnosis is crucial since 80% of cases patients require surgical intervention, such as tracheostomy or laser surgery, to relieve symptoms. The "gold standard" for diagnosing VCP has been laryngoscopy. In this case study, we demonstrate for the first time that idiopathic bilateral VCP can be accurately diagnosed by means of a novel noninvasive methodology: dynamic volume 320-slice computed tomography larynx. Three-dimensional reconstruction of laryngeal motion during the breathing cycle permitted functional assessment of the larynx showing absence of vocal cord movements. The new methodology may be valuable for noninvasive diagnosis of vocal cord movement disorders before and for follow-up after surgery.
Ruane, Laurence E; Lau, Kenneth K; Low, Kathy; Crossett, Marcus; Vallance, Neil; Bardin, Philip G
Idiopathic bilateral vocal cord paralysis (VCP) is a rare and difficult condition often undiagnosed and frequently confused with asthma and other respiratory conditions. Accurate diagnosis is crucial since 80% of cases patients require surgical intervention, such as tracheostomy or laser surgery, to relieve symptoms. The “gold standard” for diagnosing VCP has been laryngoscopy. In this case study, we demonstrate for the first time that idiopathic bilateral VCP can be accurately diagnosed by means of a novel noninvasive methodology: dynamic volume 320-slice computed tomography larynx. Three-dimensional reconstruction of laryngeal motion during the breathing cycle permitted functional assessment of the larynx showing absence of vocal cord movements. The new methodology may be valuable for noninvasive diagnosis of vocal cord movement disorders before and for follow-up after surgery. PMID:25473555
Mugalur, Aakash; Pathak, Aditya C; Shahane, Sunil M; Samant, Ashwin
Introduction: Bilateral Flexion Deformity commonly results secondary to cerebral palsy, poliomyelitis, haemophilia etc. It is accompanied by valgus deformity and external rotation at knee in long standing cases secondary to the contracture of the iliotibial tract. Flexion deformity at knees is an impediment to the normal ambulation and is difficult to address. Case Report: A 34 year old male presented with bilateral knee stiffness. He had multifocal tuberculosis and was bed ridden for almost a year and consequently developed bilateral knee flexion deformity of 60o with further flexion upto 120o. Patient was treated with gradual distraction using a modified external fixator and achieved full correction at the end of 6 weeks. At final followup patient was walking comfortably and was able to squat and sit crossed legged. Conclusion: Idiopathic isolated bilateral flexion deformity of knees is very rare and is an impediment to normal ambulation. Arthrodiastasis with indigenously designed fixator using the Ilizarov principle and modified fixator is a simple, efficient and cost effective treatment for flexion deformity of knee. PMID:27299020
Mizrahi, Cezar J; Cohen, José E; Gomori, J M; Shoshan, Yigal; Spektor, Sergey; Moscovici, Samuel
We review our experience with four patients who presented to our Medical Center from 2005-2015 with adult idiopathic occlusion of the foramen of Monro (FM). All patients underwent CT scanning and MRI. Standard MRI was performed in each patient to rule out a secondary cause of obstruction (T1-weighted without- and with gadolinium, T2-weighted, fluid-attenuated inversion recovery [FLAIR] and diffusion-weighted imaging [DWI] protocols). When occlusion of the FM appeared to be idiopathic, further high-resolution MRI with multiplanar reconstructions for evaluation of stenosis or an occluding membrane at the level of the FM was performed (T1-weighted without- and with gadolinium, T2-weighted 3D turbo spin-echo). Occlusion of the FM was due to unilateral stenosis and septum pellucidum deviation in two patients, to an occluding membrane in one, and to bilateral stenosis in one patient. Urgent surgical intervention is mandatory when there are signs of increased intracranial pressure while asymptomatic patients may be managed conservatively. In this patient series, truly bilateral stenotic obstruction of the FM was best managed with ventriculoperitoneal shunt and patients with membranous obstruction or unilateral stenosis with septum deviation were treated endoscopically.
Heilmann, R M; Thieman-Mankin, K M; Cook, A K
A 2-year-old male castrated German Shepherd dog mix was presented with chronic macroscopic haematuria. Further diagnostics included abdominal ultrasound and urethrocystoscopy and led to a diagnosis of severe bilateral idiopathic renal haematuria (IRH). Medical treatment with Yunnan Baiyao was unsuccessful. Bilateral renal-sparing sclerotherapy was performed and, despite distal migration of both ureteral stents within 12 days, permanently resolved the macroscopic haematuria.
Wan-Wei, Loo; Tengku-Norina, Tuan-Jaffar; Azma-Azalina, Ahmad-Alwi; Zulkifli, Abdul-Ghani; Zunaina, Embong
A 45-year-old female with underlying idiopathic thrombocytopenic purpura (ITP) complained of acute onset of reduced vision and floaters, in both eyes, for 3 weeks. Visual acuity was 6/36 and 6/60 in the right eye and left eye, respectively. Ophthalmoscopy showed bilateral peripapillary, subhyaloid and vitreous hemorrhage. Hematological evaluation revealed moderate anemia (hemoglobin: 93 g/L) and mild thrombocytopenia (platelets: 120×10(9)/L). She was co-managed by a hematologist and ophthalmologists; she was treated medically. Follow-up care during the next 6 weeks revealed spontaneous, partially resolving hemorrhage, with improvement of visual acuity. The purpose of this case report is to highlight ophthalmic involvement of ITP in this patient, despite her only-mild thrombocytopenia, and her spontaneous recovery, despite her receiving only medical treatment.
STER, Anda-Maria; STAN, Cristina; GHERVAN, Mihaela
Central Retinal Vein Occlusion (CRVO) is a severe retinal pathology, which causes visual impairment usually after the age of 40. Mostly unilateral, less than 10% of cases are bilateral. Affected young adults (under the age of 40), usually exhibit a hidden, underlying systemic disease. Thorough testing has to be done in order to spot the pathogenic agent. We present the case of a 25 years old woman with bilateral CRVO caused by Diaphragmatic Eventration and Chronic Respiratory Failure. PMID:25705293
Yao, Peng; Hong, Tao; Wang, Zhi-bin; Ma, Jia-ming; Zhu, Yong-qiang; Li, Hong-xi; Ding, Yuan-yuan; Jiang, Chang-lin; Pan, Shi-nong
Abstract Radiofrequency thermocoagulation (RFT) is an effective treatment for trigeminal neuralgia, but consensus regarding an optimal treatment temperature is lacking. While treatment temperatures ranging from 60°C to 95°C have been reported, RFT at too high a temperature is often followed by serious complications, and comparative evaluations of RFT at different temperatures in a single study are rare. This current prospective cohort study was to compare immediate and long-term outcomes of RFT at varying temperatures in patients with bilateral idiopathic trigeminal neuralgia (ITN) of maxillary division of trigeminal nerve (V2), mandibular division of trigeminal nerve (V3), and V2+V3, including pain relief, complications, recurrence rate, and patient satisfaction. From May 2011 to April 2016, 62 consecutive patients with bilateral ITN of V2, V3, and V2+V3 were enrolled in the study. These patients underwent bilateral RFT at 68°C and 75°C, respectively, using the same RF parameters. Side-to-side results, including pain relief, complications, and patient satisfaction, were compared during a 5-year follow-up period. Overall pain relief was satisfactory after RFT. The rate of pain relief after treatment at 75°C was slightly higher than at 68°C (P > 0.05). The pain-free rate was 95.1% at 75°C and 93.5% at 68°C at 1 year, 84.3% and 78.1% at 3 years, and 80.7% and 74.4% at 5 years. There were 10 and 13 cases of recurrence, respectively, and 6 cases of bilateral recurrence. The incidence and severity of complications were greater at 75°C (P < 0.05) than at 68°C, and therefore the patient satisfaction at the higher temperature was lower (P < 0.05). Patients with bilateral ITN who underwent RFT at different temperatures had consistent pain relief after RFT at both 75°C and 68°C, but there were fewer and less severe complications at 68°C, which was accompanied by greater patient satisfaction. This suggests that RFT at lower temperatures may be
Shin, Eun-Ho; Kang, Joon-Soon
Idiopathic chondrolysis of the hip usually develops in adolescents and is a disease characterized by gradual degenerative changes of the hyaline cartilage surrounding the head of the femur. It eventually decreases the hip joint space and causes limitations in the hip joint range of motion due to pain. The authors had experienced an unusual case of bilateral idiopathic chondrolysis of the hip in an 54 year-old male; thus, we report the treatment results and literature reviews in this case report. PMID:28097114
Moon, Hoseok; Lee, Dae Yeong; Nam, Dong Heun
AIM To evaluate the axial length (AXL) in unilateral idiopathic central serous chorioretinopathy (CSC). METHODS This retrospective case-control study was comprised of a consecutive case series of 35 patients with acute unilateral idiopathic CSC, and age- and sex-matched 50 control eyes. AXL of both eyes of unilateral CSC patients and the control eyes were investigated. AXL was measured by ultrasonic biometry, and the adjusted AXL was calculated for CSC eyes as measured AXL plus differences of foveal thickness between CSC and normal fellow eyes in millimeters. The main outcome measures were comparison of AXL between CSC, fellow and control eyes. RESULTS The mean age of 35 CSC patients was 45.5y, and 31 males were included. The adjusted AXL of CSC eyes was 23.52 mm, and the AXL of fellow eyes was 23.46 mm, and of control eyes 23.94 mm. The AXL of both CSC and fellow eyes were significantly shorter than control eyes (CSC vs control, P=0.044; fellow vs control, P=0.026). There was no statistically significant difference in AXL between CSC and fellow eyes. CONCLUSION In unilateral idiopathic CSC, the AXL of CSC and fellow eyes are shorter than that of control eyes. Short AXL may be related with choroidal circulation abnormality in CSC. PMID:27275428
Bodukam, Vijay Kumar; Thakur, Kshitij; Singh, Amandeep; Jenkins, Donald; Bahl, Jaya
Adrenal hemorrhage is a largely uncommon condition typically caused by a number of factors including infection, MI, CHF, anticoagulants, trauma, surgery, and antiphospholipid syndrome. Yet, idiopathic bilateral hemorrhage is rare. The authors present a case of a 63-year-old male who presented with abdominal pain that was eventually diagnosed as bilateral adrenal hemorrhages due to an unknown origin. Abdominal CT revealed normal adrenal glands without enlargement, but an MRI displayed enlargement due to hemorrhage in both adrenals. There was no known cause; the patient had not suffered from an acute infection and was not on anticoagulants, and the patient's history did not reveal any of the other known causative factors. The case underscores the importance of keeping bilateral adrenal hemorrhages on the list of differentials even when a cause is not immediately clear. It also raises the question of whether CT is the most sensitive test in the diagnosis of adrenal hemorrhage and whether the diagnostic approach should place greater weight on MRI. The case highlights the need for prompt therapy with steroids once bilateral hemorrhage is suspected to avert the development or progression of adrenal insufficiency. PMID:25973281
Tylus, Magdalena; Święch-Zubilewicz, Anna; Dolar-Szczasny, Joanna; Mackiewicz, Jerzy
Central serous chorioretinopathy is a common retinopathy, which is manifested by the idiopathic detachment of the neurosensory retina in the posterior pole, secondary to fluid leakage from choroidal vessels at the level of retinal pigment epithelium. The disease is typically unilateral and affects young men. We present a case of a 48-year old man, admitted to the Department of Vitreo-Retinal Surgery, Medical University in Lublin, reporting vision impairment in his right eye. The bilateral ocular exam followed by optical coherence tomography, ultra-widefield fluorescein angiography and microperimetry revealed bilateral central serous chorioretinopathy. This case presents a bilateral manifestation of central serous chorioretinopathy and emphasizes the role of advanced diagnostic imaging techniques in analyzing retinal function and disease management.
Wang, Xun; Zheng, Cong; Liu, Wen
To identify the cause of a sudden binocular vision decrease in patients with retinitis pigmentosa and bilateral idiopathic demyelinating optic neuritis is difficult, but early diagnosis and treatment significantly improve the prognosis. Here, we report a 6-year-old boy with a progressive binocular vision decrease in 38 days. The patient had a history of night blindness, a mottled retina without pigmentation, extinguished electroretinographic response, tritanopia, and an absent ellipsoid zone outside the macula fovea by optical coherence tomography in both eyes. His condition was diagnosed as retinitis pigmentosa (RP) with idiopathic demyelinating optic neuritis (IDON). After corticosteroid therapy, visual acuity recovered to OD: 0.5 and OS: 0.4. Genetic analysis revealed a G985S variant in the oral-facial-digital syndrome 1 gene. Ophthalmologists should pay attention to the existence of other complications in patients with RP who suffer a sudden decrease in vision. A gene survey can help clarify this diagnosis. To our knowledge, this is the first report of a patient with RP and ON, as well as genetic testing results. Nevertheless, the pathogenicity of the variant needs further confirmation. PMID:28191358
Burwell, R G; Freeman, B J C; Dangerfield, P H; Aujla, R K; Cole, A A; Kirby, A S; Pratt, R K; Webb, J K; Moulton, A
The detection of anomalous extra-spinal left-right skeletal length asymmetries in the upper limbs, periapical ribs, ilia and lower limbs of subjects with adolescent idiopathic scoliosis (AIS) raises questions about skeletal bilateral symmetry of vertebrates in health and disorder, its origin and control. The vertebrate body plan externally has mirror-image bilateral symmetries that are highly conserved culminating in the adult form. The normal human body can be viewed as containing paired skeletal structures in the axial and appendicular skeleton as 1) separate left and right paired forms (eg long limb bones, ribs, ilia), and 2) united in paired forms (eg vertebrae, sternum, skull, mandible). Each of these separate and united pairs are mirror-image forms--enantiomorphs. Left-right asymmetries of growth plates (physes) may cause (1) in long bones length asymmetries, (2) within one or more vertebral physes putative growth conflict with distortion as deformity, and (3) between ribs and vertebrae putative growth conflict that triggers thoracic AIS suggesting preventive surgery on spine and ribs. There is evidence of a possible role for environmental factors in AIS development. Genes and the environment (nature/nurture) may interact pre- and/or post-natally to explain both the deformity of AIS and its association with widespread anomalous skeletal length asymmetries. If substantiated there may ultimately be a place for the prevention of AIS in some subjects.
Tevaraj, Jessica Mani Penny; Mohd-Noor, Raja-Azmi; Thavaratnam, Lakana Kumar; Salmah, Win Mar
A previously healthy 27-year-old Malay male presented with acute onset of painless, severe blurring of vision in his right eye. It was associated with headache and vomiting for the past week. Relative afferent pupillary defect was present in the right eye, with reduced optic nerve function. Patient also had bilateral generalised optic disc swelling, splinter haemorrhages, and tortuous vessels. Initial examination was suggestive of either optic neuritis or raised intracranial pressure. Typical features of bilateral optic perineuritis (OPN) such as tram track and doughnut sign were observed on magnetic resonance imaging. Connective tissue and infective screening were negative. He was diagnosed with bilateral optic perineuritis and treated with high dose intravenous corticosteroids followed by a three-month course of oral steroids. His vision and optic nerve function recovered to baseline levels. PMID:28078151
Herlin, C; Chaput, B; Rivier, F; Doucet, J C; Bigorre, M; Captier, G
The authors present the management of a young female patient who presented with longstanding bilateral calf muscle hypertrophy, with no known cause. Taking into account the patient's wishes and the fact that the hypertrophy was mainly located in the posteromedial compartment, we chose to carry out a subtotal bilateral resection of medial gastrocnemius muscles. This procedure was performed with an harmonic scalpel, permitting a excellent cosmetic result while avoiding complications or functional impairment. After a reviewing of the commonly used techniques, the authors discuss the chosen surgical approach taking into account its clinical particularity.
Chung, Eun Joo; Cho, Gu-no; Kim, Sang Jin
Paroxysmal kinesigenic dyskinesia (PKD) is presented as a short paroxysmal attack of focal or generalized involuntary movement. Bilateral striopallidodentate calcinosis (BSPDC) is referred to as Fahr's disease and is characterized by the calcification of the basal ganglia, cerebellar nuclei and thalamus. Most common presentation of BSPDC was a parkinsonism, and PKD has also been reported to few of the cases with sporadic BSPDC. Here, we report the case of a 35-year-old-man with PKD for 19 years, and we describe the pathogenesis of PKD in the BSPDC.
Battaglia, Patrick J.; Gliedt, Jordan; McDaniel, Courtney; Kettner, Norman
Objective The purpose of this case report is to describe a patient with bilateral idiopathic osteonecrosis of the femoral head (ONFH), provide a discussion of differential diagnosis for anterior hip pain, imaging, and treatment recommendations for ONFH. Clinical features A 34-year-old man was initially treated by a chiropractic physician for low back pain. At the end of a three week trial of care, the patient’s low back pain resolved. However, he reported a new complaint of mild left anterior hip stiffness. After re-examination, a homecare exercise program was prescribed. The patient returned 1 month later with substantial left anterior hip pain and walked with a noticeable limp. Radiography of the left hip demonstrated advanced ONFH. Magnetic resonance imaging of both hips demonstrated the extent of involvement of the left hip while incidentally revealing ONFH on the right. Intervention and Outcome A total hip arthroplasty was performed on the left hip and the right hip is being monitored without intervention. Conclusion Osteonecrosis of the femoral head is a challenging clinical problem with non-specific and wide-ranging signs and symptoms requiring clinicians to engage a cautious and comprehensive differential diagnosis. Prompt recognition ensures that appropriate treatment can be initiated in a timely manner and optimal patient outcomes achieved. PMID:25225469
Reynolds, W. Stuart; Dmochowski, Roger; Wein, Alan; Bruehl, Stephen
The pathophysiological mechanisms underlying overactive bladder syndrome (OAB) can include dysfunction of sensory pathways of the peripheral and central nervous systems, resulting in bladder hypersensitivity. Central sensitization describes an induced state of spinal hypersensitivity that is associated with a variety of chronic pain disorders that share many attributes with OAB, albeit without the presence of pain. As such, the concept of central sensitization might be relevant to understanding the mechanisms and clinical manifestations of OAB syndrome. An understanding of the pathophysiology and clinical manifestations of central sensitization, and the evidence that supports a role of central sensitization in OAB, including the potential implications of mechanisms of central sensitization for the treatment of patients with OAB could provide a novel approach to the treatment of patients with this disease. Such an approach would be especially relevant to those patients with central sensitization-related comorbidities, and has the potential to improve the outcomes of these patients in particular. PMID:27245505
Mbekeani, Joyce N; Abdel Fattah, Maaly; Al Nounou, Randa M; Chebbo, Wahiba; Dogar, Mohammed Asif
Bilateral, simultaneous optic nerve sheath infiltration as a manifestation of leukemia relapse is very rare. A 45-year-old woman with chronic myelogenous leukemia was successfully treated to cytogenetic bone marrow remission 1 year previously and maintained on imatinib. She developed total bilateral blindness with marked, bilateral optic disc edema and evidence of bilateral optic nerve infiltration on magnetic resonance imaging. Cerebrospinal fluid cytology confirmed central nervous system (CNS) blast crisis. She recovered visual acuity of 20/20 in the right eye, and 20/25 in the left eye with salvage systemic and intrathecal chemotherapy before radiation therapy. Our report underscores the importance of timely and aggressive intervention of blast crisis of the CNS and the need for CNS penetrating induction and maintenance therapy.
Portela, Maristela Barbosa; Sanchez, Ana Lúcia Freire; Gleiser, Rogério
A 7-year-old female patient presented with a rare case of bilateral distal ectopic eruption of the mandibular permanent central incisors. Treatment instituted was extraction of the retained primary teeth and placement of a fixed appliance for alignment and repositioning of the mandibular permanent incisors.
Pollithy, S; Ach, T; Schaal, K B; Dithmar, S
This article presents a case of acute bilateral impaired vision and central scotoma in an 11-year-old boy. Looking directly into a laser beam of a laser pointer for only a few seconds can cause retinal damage in the form of lesions of the retinal pigment epithelium and the photoreceptor layer, up to retinal hemorrhage. Patients often complain about impaired vision and a central scotoma of the affected eye.
Fernández-de-las-Peñas, César; de la Llave-Rincón, Ana Isabel; Fernández-Carnero, Josué; Cuadrado, María Luz; Arendt-Nielsen, Lars; Pareja, Juan A
The aim of this study was to investigate whether bilateral widespread pressure hypersensitivity exists in patients with unilateral carpal tunnel syndrome. A total of 20 females with carpal tunnel syndrome (aged 22-60 years), and 20 healthy matched females (aged 21-60 years old) were recruited. Pressure pain thresholds were assessed bilaterally over median, ulnar, and radial nerve trunks, the C5-C6 zygapophyseal joint, the carpal tunnel and the tibialis anterior muscle in a blinded design. The results showed that pressure pain threshold levels were significantly decreased bilaterally over the median, ulnar, and radial nerve trunks, the carpal tunnel, the C5-C6 zygapophyseal joint, and the tibialis anterior muscle in patients with unilateral carpal tunnel syndrome as compared to healthy controls (all, P < 0.001). Pressure pain threshold was negatively correlated to both hand pain intensity and duration of symptoms (all, P < 0.001). Our findings revealed bilateral widespread pressure hypersensitivity in subjects with carpal tunnel syndrome, which suggest that widespread central sensitization is involved in patients with unilateral carpal tunnel syndrome. The generalized decrease in pressure pain thresholds associated with pain intensity and duration of symptoms supports a role of the peripheral drive to initiate and maintain central sensitization. Nevertheless, both central and peripheral sensitization mechanisms are probably involved at the same time in carpal tunnel syndrome.
Hanta, Deniz; Törer, Birgin; Temiz, Fatih; Kılıçdağ, Hasan; Gökçe, Mahmut; Erdoğan, Özlem
Neonatal central diabetes insipidus (DI) is an extremely rare disorder that can cause severe morbidity and mortality. We have reported a very low birth weight infant with idiopathic central DI presenting in the first month of life who was successfully treated with sublingual desmopressin therapy. In this report, we emphasize that central DI should be kept in mind in an infant with unexplained hypernatremia and polyuria. Timely diagnosis and treatment with lyophilized desmopressin may prevent severe morbidity and mortality.
Bartoli, Andrea; Ghinda, Cristina Diana; Radovanovic, Ivan; Momjian, Shahan
A 39-year old man came to our outpatient clinic with long history of unspecific symptoms and signs. Cerebral MRI showed herniation of the cerebellar tonsils of more than 1 cm below the foramen magnum and a triventricular hydrocephalus. A diagnosis of Chiari I malformation was retained. After an osteo-dural decompression of the posterior fossa, post-operative MRI revealed an aqueductal stenosis with triventricular hydrocephalus. An endoscopic-third- ventriculostomy showed an idiopathic stenosis of the right foramen of Monro. Residual symptoms and persistence of biventricular hydrocephalus justified a ventriculo-peritoneal shunt. Aqueductal and foramina of Monro stenosis can mimick a Chiari I malformation.
Ossification of the subscapularis tendon is an extremely uncommon, poorly described lesion with little known about its etiopathogenesis and clinical significance. To the best of our knowledge, only three cases of this entity have been reported till now, which were all unilateral. The authors present first case of ossification of bilateral subscapularis tendons in a 57-year-old male and hope that with increase in the number of reported cases, proper guidelines for management of such cases can be formulated.
Ossification of the subscapularis tendon is an extremely uncommon, poorly described lesion with little known about its etiopathogenesis and clinical significance. To the best of our knowledge, only three cases of this entity have been reported till now, which were all unilateral. The authors present first case of ossification of bilateral subscapularis tendons in a 57-year-old male and hope that with increase in the number of reported cases, proper guidelines for management of such cases can be formulated. PMID:27709080
Background Widely used rod rotation and translation techniques for idiopathic scoliosis (IS) are effective in correcting spinal coronal deformity. Bilateral apical vertebral derotation technique by vertebral column manipulation (VCM) and vertebral coplanar alignment (VCA) technique are two strategies for three-dimensional (3D) correction for IS. The purpose of this study is to compare the post-surgical results and technical features of the bilateral apical vertebral derotation technique by VCM against the VCA technique in patients with Lenke type 1 IS. Methods Forty-eight patients with Lenke type 1 IS were enrolled in the present prospective clinical assay. They were divided into groups A (bilateral apical vertebral derotation technique by VCM, n=24) and B (VCA technique, n=24). Radiographic parameters measured before and after surgery included the Cobb angle, thoracic kyphosis, and apical vertebral rotation. Scoliosis Research Society (SRS)-22 scores were evaluated during the final follow-up. The differences in the demographics, surgical details, and radiographic measurements between the two groups were determined using a T test. The Mann–Whitney U test was used to evaluate the differences in the SRS-22 scores. A value of P<0.05 was considered statistically significant. Results In the coronal plane, a significant difference was found in the correction rate of the major curve (group A: 84.8%, group B: 78.4%; P=0.045) and in the Cincinnati Correction Index between two groups (group A: 2.21, group B: 1.98; P=0.047). In the sagittal plane, no difference was found in the postoperative thoracic kyphosis between the two groups (P=0.328). In the transverse plane, no difference was found between the two groups in the correction rates of the rotation angle sagittal (P=0.298), rib hump (P=0.934), apical vertebral body-to-rib ratio (P=0.988), or apical rib spread difference (P=0.184). Patients underwent follow up for an average of 21.9 and 22.2 months in groups A and B
Kumar, Mahesh; Yadav, Krishan; Verma, Saurabh Kumar; Maheshwari, Vikas
Spontaneous chronic subdural hematoma (CSH) in infants is extremely rare. A very limited number of cases are known and reported in literature. The clinical presentation can be myriad varying from asymptomatic cases to gross neurological deficits. We report a case of a 3-month-old child who presented to us with repeated episodes of focal seizures of the left upper and lower limb of 1 month duration. Subsequent imaging revealed bilateral CSH (right > left) with left internal carotid artery infarct and midline shift to left by 8 mm. The child improved following burr hole evacuation of the right-sided CSH. The management of such case and a brief review of literature are discussed. PMID:28217168
Khan, Zeeshan; Trotti, Lynn Marie
The central disorders of hypersomnolence are characterized by severe daytime sleepiness, which is present despite normal quality and timing of nocturnal sleep. Recent reclassification distinguishes three main subtypes: narcolepsy type 1, narcolepsy type 2, and idiopathic hypersomnia (IH), which are the focus of this review. Narcolepsy type 1 results from loss of hypothalamic hypocretin neurons, while the pathophysiology underlying narcolepsy type 2 and IH remains to be fully elucidated. Treatment of all three disorders focuses on the management of sleepiness, with additional treatment of cataplexy in those patients with narcolepsy type 1. Sleepiness can be treated with modafinil/armodafinil or sympathomimetic CNS stimulants, which have been shown to be beneficial in randomized controlled trials of narcolepsy and, quite recently, IH. In those patients with narcolepsy type 1, sodium oxybate is effective for the treatment of both sleepiness and cataplexy. Despite these treatments, there remains a subset of hypersomnolent patients with persistent sleepiness, in whom alternate therapies are needed. Emerging treatments for sleepiness include histamine H3 antagonists (eg, pitolisant) and possibly negative allosteric modulators of the gamma-aminobutyric acid-A receptor (eg, clarithromycin and flumazenil).
González-Bonet, Luis Germán; García-Boyero, Raimundo; Gaona-Morales, John
We describe a 71-year-old-patient receiving antiplatelet therapy and being attended by emergency medical services for psychomotor retardation and gait disturbance. An emergency computed tomographic scan showed a bilateral subacute hematoma. The patient reported a fall 2 weeks earlier. We performed bilateral drills and saw a solid mass that was biopsied. The patient had a history of mantle cell lymphoma (MCL) in complete remission (results of bone marrow biopsy and whole-body positron emission tomography-computed tomography scans were normal 6 months earlier). We diagnosed an intracranial MCL by immunohistochemistry, flow cytometry, and fluorescence in situ hybridization. We performed magnetic resonance imaging. The results of a new bone marrow biopsy were positive for recurrence of MCL. MCL constitutes approximately 5%-6% of non-Hodgkin lymphoma. The incidence of central nervous system (CNS) involvement between MCLs is 4.1%. After a review of the literatures we found small series comprising 3-5 cases and a multicenter study with 57 cases. Until now, the median survival was 3.7 months. Ibrutinib, an oral Bruton tyrosine kinase inhibitor, has demonstrated efficacy and CNS penetration in relapsed or refractory MCL with rapid and complete response even after 1 year of follow-up. Our patient received ibrutinib and had a complete response at 3 months, which was maintained to the present (6 months). After a review of the literature, we found different pathologies that can mimic subdural hematomas. However, this is the first report of a lymphoma with CNS involvement mimicking bilateral subdural hematomas. This report contributes to the knowledge of lymphomas with CNS involvement. Its strange radiographic appearance and histologic type make it unique.
Male rats received either ibotenic acid (IBO) or sham lesions bilaterally into the central or lateral amygdala or were assigned to an unoperated control group. After the postoperation recovery period all lesioned and unoperated animals were tested for open field behaviour and for the ability to master a free operant successive discrimination. Retention of the discrimination learning was evaluated 48 h later for the original and reversal problem. After the reversal learning retention test the unoperated animals were assigned at random to one unoperated control and two IBO amygdaloid lesioned groups (central and lateral) and these, unoperated and lesioned animals, received additional free operant successive discrimination retraining after the surgery recovery period. Significant lesion effects were found in the emotional indices in the open field test. The lesions significantly impaired the postoperative acquisition of a free operant successive discrimination and its reversal and diminished its retention but did not impair the retention of such a discrimination task acquired before the lesion. The contribution of central and lateral amygdala in open field behaviour and in the major components of a free operant successive discrimination is discussed. In order to know how the amygdala is involved in association of sensorial stimuli with reinforcement we suggest experimental designs controlling the detailed components of such an association.
Raman, Rajiv; Damkondwar, Deepali; Neriyanuri, Srividya; Sharma, Tarun
Microperimetry-1 (MP-1) evaluation and MP-1 biofeedback training were done in a case of bilateral myopic macular degeneration with a central scotoma. Fixation behavior, location and stability of preferred retinal locus, eye movement speed, and mean sensitivity were assessed. The mean retinal sensitivities before, after and at 1-year after training in the right eye were 2.9 dB, 2.9 dB and 3.7 dB and in the left eye were 3.5 dB, 3.7 dB and 1.8 dB. The fixation point in the 2° gravitation circle, improved from 40% to 50% in the right eye and from 43% to 67% in the left eye. The average eye speed before, after and at 1-year after training in right eye were 0.19°/s, 0.26°/s and 0.25°/s and in left eye were 0.36°/s, 0.25°/s and 0.27°/s. Thus, biofeedback training using MP-1 can improve the visual function in patients with macular diseases and central scotoma. PMID:26265646
Abacı, Ayhan; Çatlı, Gönül; Anık, Ahmet; Küme, Tuncay; Çalan, Özlem Gürsoy; Dündar, Bumin Nuri; Böber, Ece
The aim of the present study was to investigate the diagnostic role of serum neurokinin B level and its relationship with kisspeptin and leptin, which are known to be involved in the initiation of pubertal process. Girls who presented with breast development (<8 years) were included in the study. All patients underwent bone age assessment. Basal levels of serum follicle stimulating hormone and luteinizing hormone were measured and gonadotropin releasing hormone stimulation test was performed. Patients with a bone age/chronological age ratio >1 and a peak luteinizing hormone response in gonadotropin releasing hormone stimulation test >5mIU/L were included in the central precocious puberty group, while patients who did not meet these criteria were included in the premature thelarche group. Patients with organic pathologies were excluded. Healthy prepubertal girls with similar age were included as the control group. Leptin, kisspeptin and neurokinin B levels were measured by ELISA method. The study included 20 girls with idiopathic central precocious puberty 22 girls with premature thelarche and 24 prepubertal controls. While serum kisspeptin, leptin and neurokinin B levels were significantly higher in central precocious puberty and premature thelarche groups compared to controls, no significant difference was found between central precocious puberty and premature thelarche groups. Increased serum levels of leptin, kisspeptin and neurokinin B in patients with premature thelarche and central precocious puberty suggest that they take part during the initiation of pubertal process, however, these markers are not able to differentiate patients with central precocious puberty from premature thelarche.
Dahan, Albert; Sarton, Elise; Teppema, Luc
We investigated the effect of bilateral carotid body resection (bCBR) in a patient with bilateral carotid body tumors on central CO2 sensitivity. We applied multiple square-wave changes in end-tidal CO2 and measured ventilation before the first surgery and at regular intervals for 3 years after surgery. The data were analyzed using a two-compartment model of the ventilatory control system. bCBR resulted in the loss of the fast response to CO2, and a sharp reduction in the magnitude of central CO2 sensitivity (a reduction of about 80% within 3 months after bCBR). Central CO2 sensitivity gradually increased to pre-operative values within 2 years after surgery. These observations are a strong indication for (1) the existence of a tonic influence from the peripheral chemoreceptors of the carotid bodies on central CO2 drive; (2) absence of any recovery of the peripheral drive after bCBR; and (3) neural plasticity causing the regeneration of central drive after bCBR.
Khan, Muhammad Saim; Sameen, Murtaza; Lodhi, Arshad Ali; Ahmed, Munawar; Ahmed, Noman; Kamal, Mustafa; Junejo, Sameen Afzal
Objectives: To evaluate the effect of half adult dose of oral Rifampicin on mean change in best corrected visual acuity and central macular thickness in patients with central serous chorioretinopathy. Methods: Thirty-eight eyes of 31 patients with idiopathic central serous chorioretinopathy (CSCR) were registered. Unaided Visual acuity, best corrected visual acuity was documented and detailed slit lamp examination along with dilated ophthalmoscopy was performed. All subjects were treated with oral Rifampicin 300 mg (half adult dose) daily for 03 months. Patients underwent a complete ocular and systemic examination as well as central macular thickness (CMT) measurement by optical coherence tomography (OCT) every month after starting treatment until four months. Fundus fluorescein angiography (FFA) was performed in recurrent cases. Liver function tests were carried out prior to the treatment and during follow up period. Results: A total of 38 eyes of 31 patients (24 males, 07 females) were included in the study. Mean age of patients was 36.16±3.19 years (range 30-44). Mean best corrected visual acuity (BCVA) before treatment was 0.56±0.11 and improved to 0.47±0.14 at 04 weeks (P<0.001) of treatment. The mean CMT at the time of presentation was 494.39±96.29 um and was decreased to 306.90±50.71 um after 04 weeks of treatment (P<0.001). The mean induced reduction in CMT was 187.48±122 um (P<0.001) while that in BCVA 0.41±0.16 at 04 weeks of treatment (P<0.001). Liver function tests were within normal range before and after the treatment. Conclusion: Half adult dose rifampicin (300mg) is effective and safe in treatment of central serous chorioretinopathy without causing any systemic imbalance. PMID:27882013
Chithra, R; Sundar, R Ajai; Velladuraichi, B; Sritharan, N; Amalorpavanathan, J; Vidyasagaran, T
Aneurysms are rare in children. Isolated iliac artery aneurysms are very rare, especially bilateral aneurysms. Pediatric aneurysms are usually secondary to connective tissue disorders, arteritis, or mycotic causes. We present a case of a 3-year-old child with bilateral idiopathic common iliac aneurysms that were successfully repaired with autogenous vein grafts.
Tavallali, Ali; Yannuzzi, Lawrence A.
Idiopathic multifocal choroiditis (MFC) and/or punctate inner choroidopathy (PIC) describe a chronic progressive bilateral inflammatory chorioretinopathy that predominantly affect healthy myopic white women with no known associated systemic or ocular diseases. The principal sites of involvement are the retinal pigment epithelium (RPE) and outer retinal spaces; the choroid is not affected during the active phase of the disease. Idiopathic MFC with atrophy is a recently described variant. Although there is no generally accepted standard treatment, anti-inflammatory and anti-VEGF (vascular endothelial growth factor) agents are necessary in the acute stage to control the inflammation and choroidal neovascularization (CNV). PMID:27994812
Leka-Emiri, Sofia; Chrousos, George P; Kanaka-Gantenbein, Christina
Puberty is a major developmental stage. Damaging mutations, considered as "mistakes of nature", have contributed to the unraveling of the networks implicated in the normal initiation of puberty. Genes involved in the abnormal hypothalamic-pituitary-gonadal (HPG) axis development, in the normosmic idiopathic hypogonadotropic hypogonadism (nIHH), in the X-linked or autosomal forms of Kallmann syndrome and in precocious puberty have been identified (GNRH1, GNRHR, KISS1, GPR54, FGFR1, FGF8, PROK2, PROKR2, TAC3, TACR3, KAL1, PROK2, PROKR2, CHD7, LEP, LEPR, PC1, DAX1, SF-1, HESX-1, LHX3, PROP-1). Most of them were found to play critical roles in HPG axis development and regulation, the embryonic GnRH neuronal migration and secretion, the regulation and action of the hypothalamic GnRH. However, the specific neural and molecular mechanisms triggering GnRH secretion remain one of the scientific enigmas. Although GnRH neurons are probably capable of autonomously generating oscillations, many gonadal steroid-dependent and -independent mechanisms have also been proposed. It is now well proven that the secretion of GnRH is regulated by kisspeptin as well as by permissive or opposing signals mediated by neurokinin B and dynorphin. These three supra-GnRH regulators compose the kisspeptin-neurokinin B-dynorphin neuronal (KNDy) system, a key player in pubertal onset and progression. Moreover, an ongoing increasing number of inhibitory, stimulatory and permissive networks acting upstream on GnRH neurons, such as GABA, NPY, LIN28B, MKRN3 and others integrate diverse hormonal and peripheral signals and have been proposed as the "gate-keepers" of puberty, while epigenetic modifications play also an important role in puberty initiation.
Novák, P; Brabec, M; Novák, I; Manasová, M
The authors present a case review of a 30-year old patient, who developed central pleural effusion, a complication related to central venous catheterization and the consequent use of the central venous line. The authors aim to highlight potential complications of the method. The interesting feature of this case is the fact, that no apparent mistakes in the venous line introduction or its later use have been recorded.
Ambulkar, Prafulla S.; Sigh, Ramji; Reddy, MVR; Varma, Poonam S.; Gupta, Dilip O.; Shende, Moreshwar R; Pal, Asoke K
Background: Genetic factors cause about 15% of male infertility. Azoospermia factors (AZFa, AZFb, and AZFc) present on Yq are most important for spermatogenesis. We have made an attempt to evaluate the frequencies of microdeletions of AZFa, AZFb, AZFc in idiopathic cases of azoospermia and oligozoospermia from central Indian population. Materials and Methods: We have analyzed a total of 156 subjects (95 oligozoospermia and 61 azoospermia) & 50 control subjects. DNA samples were analyzed for microdeletions of Y chromosome by PCR-screening of 18 sequences-tagged-site (STS) markers from different region of the AZF on Yq and SRY on Yp. Results: Out of 156 cases analyzed, 13 (8.33%) subjects (8 azoospermia and 5 oligozoospermia) showed partial deletion of AZF regions, of which deletion in AZFc region was the most common (84.6%) followed by AZFb (15.4%) and AZFa (15.4%). The sites and sizes of deletions varied among patients. Histological study of the testicular tissue of the available subjects, who showed microdeletions of Y chromosome, showed spermatogenic arrest at different stages. The frequency of Y chromosome microdeletion in our subjects was 8.33%. Conclusion: Some Indian studies reported low frequencies of microdeletions than that of our result. We suggest that the frequency of deletions may be affected by the involvement of different genetic factors, ethnic population and different geographical regions. PCR based Y chromosome screening for microdeletions will be useful and great help to infertility clinics for genetic counselling and assisted reproduction. PMID:24783090
Cisternino, Mariangela; Della Mina, Erika; Losa, Laura; Madè, Alexandra; Rossetti, Giulia; Bassi, Lorenzo Andrea; Pieri, Giovanni; Bayindir, Baran; Messa, Jole; Zuffardi, Orsetta; Ciccone, Roberto
We report a girl with a de novo distal deletion of 9p affected by idiopathic central precocious puberty and intellectual disability. Genome-wide array-CGH revealed a terminal deletion of about 11 Mb, allowing to define her karyotype as 46; XX, del(9)(p23-pter). To our knowledge, this is the second reported case of precocious puberty associated with 9p distal deletion. A third case associates precocious puberty with a more proximal 9p deletion del(9)(p12p13,3). In our case, more than 40 genes were encompassed in the deleted region, among which, DMRT1 which is gonad-specific and has a sexually dimorphic expression pattern and ERMP1 which is required in rats for the organization of somatic cells and oocytes into discrete follicular structures. Although we cannot exclude that precocious puberty in our del(9p) patient is a coincidental finding, the report of the other two patients with 9p deletions and precocious puberty indeed suggests a causative relationship. PMID:23984122
Prospective mutation screening of three common deafness genes in a large Taiwanese Cohort with idiopathic bilateral sensorineural hearing impairment reveals a difference in the results between families from hospitals and those from rehabilitation facilities.
Wu, Chen-Chi; Chen, Pei-Jer; Chiu, Yu-Hsun; Lu, Ying-Chang; Wu, Ming-Chueh; Hsu, Chuan-Jen
Accurate epidemiological data on common deafness genes are essential to improve the efficiency and to reduce the cost of molecular diagnosis. They may depend on several factors, including a clear delineation of the source of patients being studied. In the present study, we hypothesize that patients with idiopathic sensorineural hearing loss recruited from different sources might reveal discrepancies in the epidemiological results of genetic screening, because patients from different sources might demonstrate distinct clinical or audiologic features and thus result in biased selection of subjects. To elucidate the relative importance of common deafness genes in Taiwanese and to verify our hypothesis, we conducted a prospective project screening mutations in GJB2, SLC26A4 and mitochondrial 12S rRNA gene in a total of 420 Taiwanese families with idiopathic bilateral sensorineural hearing loss, of which 325 families were recruited from hospitals and 95 from hearing rehabilitation facilities. Allele frequencies of common mutations in these three genes and distributions of the corresponding genotypes were then compared between the two groups. The allele frequencies of mutations in SLC26A4, GJB2 and mitochondrial 12S rRNA in the probands of the 420 families were 14.4, 21.7 and 3.8%, respectively. The allele frequency of SLC26A4 mutations in the hospital group was significantly higher than that in the rehabilitation facility group (16.2 vs. 8.4%, chi(2)-test, p < 0.05), whereas no difference in the frequencies of GJB2 mutations and mitochondrial 12S rRNA mutations was found between the two groups. Distributions of probands classified by SLC26A4 genotypes were also different between the two groups (chi(2)-test, p < 0.05). Accordingly, a discrepancy in the genetic screening results might exist between different sources of idiopathic hearing-impaired patients. Further analysis of audiological results and construction of a logistic regression model showed that different
YaDeau, Jacques T.; Urban, Michael K.
Bilateral total knee replacement (BTKR) has been associated with a higher incidence of fat embolism (FES) compared to single knee replacement. Consequently, intraoperative monitoring with a pulmonary artery catheter (PAC) has been recommended. This study compares clinical outcome in BTKR patients monitored with central venous pressure versus PAC. A retrospective chart review of 249 consecutive patients undergoing BTKR, 132 of whom had PAC insertion versus 117 who had central line insertion, over a 1-year period were included in the study. Their medical records were reviewed for co-morbidities, baseline characteristics, and type of intraoperative monitoring. Need and duration for postoperative monitoring in the postoperative care, length of hospital stay (LOHS), signs of fat embolism, development of arrhythmias, and respiratory failure were all outcome measures. A total of four patients (1.6%) had FES as per Schonfeld criteria. One of these patients died within 48 h of surgery. They all had PAC monitoring intraoperatively. Pulmonary artery pressure (PAP) remained unchanged during surgery which raises doubt as to the clinical utility and advisability of the use of PAC’s in this setting. There was no statistically significant difference in cardiac or pulmonary complications, or LOHS between the two groups. Central venous pressure monitoring appears to be sufficient in patients undergoing BTKR. PMID:19002531
Papais-Alvarenga, Regina Maria; Vasconcelos, Claudia Cristina Ferreira; Carra, Adriana; de Castillo, Ibis Soto; Florentin, Sara; Diaz de Bedoya, Fernando Hamuy; Mandler, Raul; de Siervi, Luiza Campanella; Pimentel, Maria Lúcia Vellutini; Alvarenga, Marina Papais; Papais Alvarenga, Marcos; Grzesiuk, Anderson Kuntz; Gama Pereira, Ana Beatriz Calmon; Gomes Neto, Antonio Pereira; Velasquez, Carolina; Soublette, Carlos; Fleitas, Cynthia Veronica; Diniz, Denise Sisteroli; Armas, Elizabeth; Batista, Elizabeth; Hernandez, Freda; Pereira, Fernanda Ferreira Chaves da Costa; Siqueira, Heloise Helena; Cabeça, Hideraldo; Sanchez, Jose; Brooks, Joseph Bruno Bidin; Gonçalves, Marcus Vinicius; Barroso, Maria Cristina Del Negro; Ravelo, Maria Elena; Castillo, Maria Carlota; Ferreira, Maria Lúcia Brito; Rocha, Maria Sheila Guimarães; Parolin, Monica Koncke Fiuza; Molina, Omaira; Marinho, Patricia Beatriz Christino; Christo, Paulo Pereira; Brant de Souza, Renata; Pessanha Neto, Silvio; Camargo, Solange Maria das Graças; Machado, Suzana Costa; Neri, Vanderson Carvalho; Fragoso, Yara Dadalti; Alvarenga, Helcio; Thuler, Luiz Claudio Santos
The idiopathic inflammatory demyelinating disease (IIDD) spectrum has been investigated among different populations, and the results have indicated a low relative frequency of neuromyelitis optica (NMO) among multiple sclerosis (MS) cases in whites (1.2%-1.5%), increasing in Mestizos (8%) and Africans (15.4%-27.5%) living in areas of low MS prevalence. South America (SA) was colonized by Europeans from the Iberian Peninsula, and their miscegenation with natives and Africans slaves resulted in significant racial mixing. The current study analyzed the IIDD spectrum in SA after accounting for the ethnic heterogeneity of its population. A cross-sectional multicenter study was performed. Only individuals followed in 2011 with a confirmed diagnosis of IIDD using new diagnostic criteria were considered eligible. Patients’ demographic, clinical and laboratory data were collected. In all, 1,917 individuals from 22 MS centers were included (73.7% female, 63.0% white, 28.0% African, 7.0% Mestizo, and 0.2% Asian). The main disease categories and their associated frequencies were MS (76.9%), NMO (11.8%), other NMO syndromes (6.5%), CIS (3.5%), ADEM (1.0%), and acute encephalopathy (0.4%). Females predominated in all main categories. The white ethnicity also predominated, except in NMO. Except in ADEM, the disease onset occurred between 20 and 39 years old, early onset in 8.2% of all cases, and late onset occurred in 8.9%. The long-term morbidity after a mean disease time of 9.28±7.7 years was characterized by mild disability in all categories except in NMO, which was scored as moderate. Disease time among those with MS was positively correlated with the expanded disability status scale (EDSS) score (r=0.374; p=<0.001). This correlation was not observed in people with NMO or those with other NMO spectrum disorders (NMOSDs). Among patients with NMO, 83.2% showed a relapsing-remitting course, and 16.8% showed a monophasic course. The NMO-IgG antibody tested using indirect
Papais-Alvarenga, Regina Maria; Vasconcelos, Claudia Cristina Ferreira; Carra, Adriana; de Castillo, Ibis Soto; Florentin, Sara; Diaz de Bedoya, Fernando Hamuy; Mandler, Raul; de Siervi, Luiza Campanella; Pimentel, Maria Lúcia Vellutini; Alvarenga, Marina Papais; Alvarenga, Marcos Papais; Grzesiuk, Anderson Kuntz; Gama Pereira, Ana Beatriz Calmon; Gomes Neto, Antonio Pereira; Velasquez, Carolina; Soublette, Carlos; Fleitas, Cynthia Veronica; Diniz, Denise Sisteroli; Armas, Elizabeth; Batista, Elizabeth; Hernandez, Freda; Pereira, Fernanda Ferreira Chaves da Costa; Siqueira, Heloise Helena; Cabeça, Hideraldo; Sanchez, Jose; Brooks, Joseph Bruno Bidin; Gonçalves, Marcus Vinicius; Barroso, Maria Cristina Del Negro; Ravelo, Maria Elena; Castillo, Maria Carlota; Ferreira, Maria Lúcia Brito; Rocha, Maria Sheila Guimarães; Parolin, Monica Koncke Fiuza; Molina, Omaira; Marinho, Patricia Beatriz Christino; Christo, Paulo Pereira; Brant de Souza, Renata; Pessanha Neto, Silvio; Camargo, Solange Maria das Graças; Machado, Suzana Costa; Neri, Vanderson Carvalho; Fragoso, Yara Dadalti; Alvarenga, Helcio; Thuler, Luiz Claudio Santos
The idiopathic inflammatory demyelinating disease (IIDD) spectrum has been investigated among different populations, and the results have indicated a low relative frequency of neuromyelitis optica (NMO) among multiple sclerosis (MS) cases in whites (1.2%-1.5%), increasing in Mestizos (8%) and Africans (15.4%-27.5%) living in areas of low MS prevalence. South America (SA) was colonized by Europeans from the Iberian Peninsula, and their miscegenation with natives and Africans slaves resulted in significant racial mixing. The current study analyzed the IIDD spectrum in SA after accounting for the ethnic heterogeneity of its population. A cross-sectional multicenter study was performed. Only individuals followed in 2011 with a confirmed diagnosis of IIDD using new diagnostic criteria were considered eligible. Patients' demographic, clinical and laboratory data were collected. In all, 1,917 individuals from 22 MS centers were included (73.7% female, 63.0% white, 28.0% African, 7.0% Mestizo, and 0.2% Asian). The main disease categories and their associated frequencies were MS (76.9%), NMO (11.8%), other NMO syndromes (6.5%), CIS (3.5%), ADEM (1.0%), and acute encephalopathy (0.4%). Females predominated in all main categories. The white ethnicity also predominated, except in NMO. Except in ADEM, the disease onset occurred between 20 and 39 years old, early onset in 8.2% of all cases, and late onset occurred in 8.9%. The long-term morbidity after a mean disease time of 9.28±7.7 years was characterized by mild disability in all categories except in NMO, which was scored as moderate. Disease time among those with MS was positively correlated with the expanded disability status scale (EDSS) score (r=0.374; p=<0.001). This correlation was not observed in people with NMO or those with other NMO spectrum disorders (NMOSDs). Among patients with NMO, 83.2% showed a relapsing-remitting course, and 16.8% showed a monophasic course. The NMO-IgG antibody tested using indirect
Background Central post-stroke pain is a neuropathic pain condition caused by a vascular lesion, of either ischemic or hemorrhagic origin, in the central nervous system and more precisely involving the spinothalamocortical pathway responsible for the transmission of painful sensations. Few animal models have been developed to study this problem. The objectives of this study were to evaluate different modalities of pain in a central neuropathic pain rat model and to assess the effects of ketamine administered at different doses. Animals were evaluated on the rotarod, Hargreaves, Von Frey and acetone tests. A very small hemorrhage was created by injecting a collagenase solution in the right ventral posterolateral thalamic nucleus. Following the establishment of the neuropathy, ketamine was evaluated as a therapeutic drug for this condition. Results Histopathological observations showed a well localized lesion with neuronal necrosis and astrocytosis following the collagenase injection that was localized within the VPL. No significant change in motor coordination was observed following surgery in either the saline or collagensae groups. In the collagenase group, a significant decrease in mechanical allodynia threshold was observed. A sporadic and transient cold allodynia was also noted. No thermal hyperalgesia was seen following the collagenase injection. Ketamine was then tested as a potential therapeutic drug. A significant decrease in motor coordination was seen only following the administration of 25 mg/kg of ketamine in both groups. An alleviation of mechanical allodynia was achieved only with the high ketamine dose. The minimal effective ketamine serum concentration (150 ng/mL) was only achieved in animals that received 25 mg/kg. Conclusions An intrathalamic hemorrhage induced a bilateral mechanical allodynia in rats. Cold hyperalgesia was observed in 60% of these animals. Mechanical allodynia was alleviated with high doses of ketamine which corresponded
Wojniusz, Slawomir; Callens, Nina; Sütterlin, Stefan; Andersson, Stein; De Schepper, Jean; Gies, Inge; Vanbesien, Jesse; De Waele, Kathleen; Van Aken, Sara; Craen, Margarita; Vögele, Claus; Cools, Martine; Haraldsen, Ira R.
Central precocious puberty (CPP) develops due to premature activation of the hypothalamic-pituitary-gonadal (HPG) axis, resulting in early pubertal changes and rapid bone maturation. CPP is associated with lower adult height and increased risk for development of psychological problems. Standard treatment of CPP is based on postponement of pubertal development by blockade of the HPG axis with gonadotropin releasing hormone analogs (GnRHa) leading to abolition of gonadal sex hormones synthesis. Whereas the hormonal and auxological effects of GnRHa are well-researched, there is a lack of knowledge whether GnRHa treatment influences psychological functioning of treated children, despite the fact that prevention of psychological problems is used as one of the main reasons for treatment initiation. In the present study we seek to address this issue by exploring differences in cognitive function, behavior, emotional reactivity, and psychosocial problems between GnRHa treated CPP girls and age-matched controls. Fifteen girls with idiopathic CPP; median age 10.4 years, treated with slow-release GnRHa (triptorelin acetate—Decapeptyl SR® 11.25) and 15 age-matched controls, were assessed with a comprehensive test battery consisting of paper and pencil tests, computerized tasks, behavioral paradigms, heart rate variability, and questionnaires filled in by the children's parents. Both groups showed very similar scores with regard to cognitive performance, behavioral and psychosocial problems. Compared to controls, treated girls displayed significantly higher emotional reactivity (p = 0.016; Cohen's d = 1.04) on one of the two emotional reactivity task conditions. Unexpectedly, the CPP group showed significantly lower resting heart rates than the controls (p = 0.004; Cohen's d = 1.03); lower heart rate was associated with longer treatment duration (r = −0.582, p = 0.037). The results suggest that GnRHa treated CPP girls do not differ in their cognitive or psychosocial
Wojniusz, Slawomir; Callens, Nina; Sütterlin, Stefan; Andersson, Stein; De Schepper, Jean; Gies, Inge; Vanbesien, Jesse; De Waele, Kathleen; Van Aken, Sara; Craen, Margarita; Vögele, Claus; Cools, Martine; Haraldsen, Ira R
Central precocious puberty (CPP) develops due to premature activation of the hypothalamic-pituitary-gonadal (HPG) axis, resulting in early pubertal changes and rapid bone maturation. CPP is associated with lower adult height and increased risk for development of psychological problems. Standard treatment of CPP is based on postponement of pubertal development by blockade of the HPG axis with gonadotropin releasing hormone analogs (GnRHa) leading to abolition of gonadal sex hormones synthesis. Whereas the hormonal and auxological effects of GnRHa are well-researched, there is a lack of knowledge whether GnRHa treatment influences psychological functioning of treated children, despite the fact that prevention of psychological problems is used as one of the main reasons for treatment initiation. In the present study we seek to address this issue by exploring differences in cognitive function, behavior, emotional reactivity, and psychosocial problems between GnRHa treated CPP girls and age-matched controls. Fifteen girls with idiopathic CPP; median age 10.4 years, treated with slow-release GnRHa (triptorelin acetate-Decapeptyl SR® 11.25) and 15 age-matched controls, were assessed with a comprehensive test battery consisting of paper and pencil tests, computerized tasks, behavioral paradigms, heart rate variability, and questionnaires filled in by the children's parents. Both groups showed very similar scores with regard to cognitive performance, behavioral and psychosocial problems. Compared to controls, treated girls displayed significantly higher emotional reactivity (p = 0.016; Cohen's d = 1.04) on one of the two emotional reactivity task conditions. Unexpectedly, the CPP group showed significantly lower resting heart rates than the controls (p = 0.004; Cohen's d = 1.03); lower heart rate was associated with longer treatment duration (r = -0.582, p = 0.037). The results suggest that GnRHa treated CPP girls do not differ in their cognitive or psychosocial
Billiard, M; Merle, C; Carlander, B; Ondze, B; Alvarez, D; Besset, A
Identification of idiopathic hypersomnia dates back 20 years only. It typically consists of prolonged nocturnal sleep, great difficulty waking up in the morning or at the end of a nap, and constant or recurrent excessive daytime sleepiness. Complete and incomplete forms are encountered. Twenty-three subjects fulfilling ICSD criteria are reported with clinical, polysomnographic and immunogenetic data. Considering differential diagnosis is an important step in the diagnosis of idiopathic hypersomnia. Idiopathic hypersomnia is much less frequent than narcolepsy. A strong genetic component is suggested by the high proportion of familial cases. No association with HLA has been evidenced to date.
Idiopathic hypersomnia is not as well delineated as narcolepsy and its history is much more recent. There are at least two forms of the disorder: (1) a polysymptomatic form, characterized by excessive daytime sleepiness, nocturnal sleep of abnormally long duration, and signs of sleep drunkenness on awakening, and (2) a monosymptomatic form that manifests only by excessive daytime sleepiness. The most widely used laboratory procedures are nocturnal polysomnographic recording following by an MSLT demonstrating a mean sleep latency of less than 10 minutes. At least in the polysymptomatic form, however, continuous polysomnography on an ad lib protocol deserves to be performed to catch the abnormally long major sleep episode and the long unrefreshing naps. Idiopathic hypersomnia is probably one of the most overdiagnosed sleep disorders. Several other disorders must be excluded before the diagnosis can be considered conclusive. Treatment of idiopathic hypersomnia relies on stimulants, which are frequently less effective and less well tolerated than in narcolepsy.
Bialasiewicz, Alexander Arthur; Abdelhamid, Mahmoud; Shenoy, Radha; Barman, Manish
A 55-year-old male presented with serous retinal detachment over 3 months in his right eye. His left eye was blind due to retinal pigment epithelium detachment since 1997 with atrophy of the neurosensory retina. Fluorescein angiography had previously shown bilateral polypoidal choroidal vasculopathy (PCV). Optical coherence tomography (OCT) confirmed PCV with central involvement. The patient underwent intravitreal injections of 6x Lucentis, 4x Avastin and one injection of aflibercept. PCV recurred from 1 to 4 months after each treatment. The patient had history of stroke, hypertension, and atrial fibrillation and was started on oral eplerenone 25 mg/day in October 2014, which resulted in a long-term ongoing complete retinal reattachment. OCT ganglion cell and inner plexiform layers showed full recovery of the fovea in the right eye and irreversible in the left eye. Low-dose eplerenone may resolve recalcitrant PCV with central involvement. The duration of treatment remains uncertain.
Bialasiewicz, Alexander Arthur; Abdelhamid, Mahmoud; Shenoy, Radha; Barman, Manish
A 55-year-old male presented with serous retinal detachment over 3 months in his right eye. His left eye was blind due to retinal pigment epithelium detachment since 1997 with atrophy of the neurosensory retina. Fluorescein angiography had previously shown bilateral polypoidal choroidal vasculopathy (PCV). Optical coherence tomography (OCT) confirmed PCV with central involvement. The patient underwent intravitreal injections of 6x Lucentis, 4x Avastin and one injection of aflibercept. PCV recurred from 1 to 4 months after each treatment. The patient had history of stroke, hypertension, and atrial fibrillation and was started on oral eplerenone 25 mg/day in October 2014, which resulted in a long-term ongoing complete retinal reattachment. OCT ganglion cell and inner plexiform layers showed full recovery of the fovea in the right eye and irreversible in the left eye. Low-dose eplerenone may resolve recalcitrant PCV with central involvement. The duration of treatment remains uncertain. PMID:27162459
Billiard, Michel; Sonka, Karel
Idiopathic hypersomnia continues to evolve from the concept of "sleep drunkenness" introduced by Bedrich Roth in Prague in 1956 and the description of idiopathic hypersomnia with two forms, polysymptomatic and monosymptomatic, by the same Bedrich Roth in 1976. The diagnostic criteria of idiopathic hypersomnia have varied with the successive revisions of the International classifications of sleep disorders, including the recent 3rd edition. No epidemiological studies have been conducted so far. Disease onset occurs most often during adolescence or young adulthood. A familial background is often present but rigorous studies are still lacking. The key manifestation is hypersomnolence. It is often accompanied by sleep of long duration and debilitating sleep inertia. Polysomnography (PSG) followed by a multiple sleep latency test (MSLT) is mandatory, as well as a 24 h PSG or a 2-wk actigraphy in association with a sleep log to ensure a total 24-h sleep time longer than or equal to 66O minutes, when the mean sleep latency on the MSLT is longer than 8 min. Yet, MSLT is neither sensitive nor specific and the polysomnographic diagnostic criteria require continuous readjustment and biologic markers are still lacking. Idiopathic hypersomnia is most often a chronic condition though spontaneous remission may occur. The condition is disabling, sometimes even more so than narcolepsy type 1 or 2. Based on neurochemical, genetic and immunological analyses as well as on exploration of the homeostatic and circadian processes of sleep, various pathophysiological hypotheses have been proposed. Differential diagnosis involves a number of diseases and it is not yet clear whether idiopathic hypersomnia and narcolepsy type 2 are not the same condition. Until now, the treatment of idiopathic hypersomnia has mirrored that of the sleepiness of narcolepsy type 1 or 2. The first randomized, double-blind, placebo-controlled trials of modafinil have just been published, as well as a double
Sharma, Aman; Ohri, Shivani; Bambery, Pradeep; Singh, Surjit
Lipoid pneumonia is a rare pulmonary disorder having no classical radiological appearance. We report a 33-year-old male, ex-smoker who was referred to us with history of cough, mild mucoid expectoration and progressively increasing dyspnoea since one year. He was investigated at local hospital and was treated with 30 mg prednisolone per day for 6 months for sarcoidosis without any response. On examination, he was normal except for fine basal crepitations in chest. Pulmonary function test (PFT) revealed mild airway obstruction. High resolution computerised tomographic scan (HRCT scan) revealed bilateral reticulonodular shadows and bronchiectasis in lower zones. Open lung biopsy revealed lipoid pneumonia. As there was no history of nasal distillation of oils, it was diagnosed to be idiopathic. The relevant literature is reviewed.
... Loss Surgery? A Week of Healthy Breakfasts Shyness Juvenile Idiopathic Arthritis (JIA) KidsHealth > For Teens > Juvenile Idiopathic ... can affect people under age 17. What Is Juvenile Idiopathic Arthritis? Arthritis doesn't affect young people ...
Batra, Vivek; Baras, Alexander
We present a case of bilateral lesions in a 50-year-old man, which were on first impression mistaken for and initially treated as bilateral cellulitis. We propose that bilateral cellulitis, as opposed to unilateral, is rare and that other aetiologies should be considered in evaluating a patient with bilateral lesions. The differential diagnosis includes stasis-dermatitis, lipodermatosclerosis, lymphoedema and vascular lesions such as Kaposi sarcoma, as was identified in this case. Early consultation with dermatology and biopsy in unclear cases mitigates the unnecessary use of prolonged antibiotics, antibiotic resistance and Clostridium difficile infections. HIV testing is an essential screening test in all adults who present with non-specific viral symptoms and rash.
Ji, Chengcheng; Wu, Liang; Zhao, Wenchan; Wang, Sishuo; Lv, Jianhao
Echinoderms take many forms of symmetry. Pentameral symmetry is the major form and the other forms are derived from it. However, the ancestors of echinoderms, which originated from Cambrian period, were believed to be bilaterians. Echinoderm larvae are bilateral during their early development. During embryonic development of starfish and sea urchins, the position and the developmental sequence of each arm are fixed, implying an auxological anterior/posterior axis. Starfish also possess the Hox gene cluster, which controls symmetrical development. Overall, echinoderms are thought to have a bilateral developmental mechanism and process. In this article, we focused on adult starfish behaviors to corroborate its bilateral tendency. We weighed their central disk and each arm to measure the position of the center of gravity. We then studied their turning-over behavior, crawling behavior and fleeing behavior statistically to obtain the center of frequency of each behavior. By joining the center of gravity and each center of frequency, we obtained three behavioral symmetric planes. These behavioral bilateral tendencies might be related to the A/P axis during the embryonic development of the starfish. It is very likely that the adult starfish is, to some extent, bilaterian because it displays some bilateral propensity and has a definite behavioral symmetric plane. The remainder of bilateral symmetry may have benefited echinoderms during their evolution from the Cambrian period to the present.
Zhao, Wenchan; Wang, Sishuo; Lv, Jianhao
Echinoderms take many forms of symmetry. Pentameral symmetry is the major form and the other forms are derived from it. However, the ancestors of echinoderms, which originated from Cambrian period, were believed to be bilaterians. Echinoderm larvae are bilateral during their early development. During embryonic development of starfish and sea urchins, the position and the developmental sequence of each arm are fixed, implying an auxological anterior/posterior axis. Starfish also possess the Hox gene cluster, which controls symmetrical development. Overall, echinoderms are thought to have a bilateral developmental mechanism and process. In this article, we focused on adult starfish behaviors to corroborate its bilateral tendency. We weighed their central disk and each arm to measure the position of the center of gravity. We then studied their turning-over behavior, crawling behavior and fleeing behavior statistically to obtain the center of frequency of each behavior. By joining the center of gravity and each center of frequency, we obtained three behavioral symmetric planes. These behavioral bilateral tendencies might be related to the A/P axis during the embryonic development of the starfish. It is very likely that the adult starfish is, to some extent, bilaterian because it displays some bilateral propensity and has a definite behavioral symmetric plane. The remainder of bilateral symmetry may have benefited echinoderms during their evolution from the Cambrian period to the present. PMID:22247765
Vincent, Stephen J; Lee, Graham A
Acquired limbal stem cell deficiency (LSCD) describes a condition in which the corneal limbal stem cells are altered or destroyed, typically due to ocular trauma, chronic allergy or inflammation. Idiopathic LSCD is a term used to describe limbal stem cell failure in the absence of any identifiable causative factor. While several cases of adult-onset LSCD have been identified previously, this case report describes a rare presentation of bilateral asymmetric idiopathic paediatric limbal stem cell deficiency in a sixteen-year-old male with an otherwise unremarkable ocular history.
Moreno Prieto, M; Carbonero Celis, M J; Cuadrado Caballero, M C
The coexistence of autoimmune hepatitis and juvenile idiopathic arthritis is very rare. This is the case of an 18 month old female patient whose first sign of disease was torticollis due to an underlying atlanto-axial subluxation. Three months later, bilateral knee arthritis developed and she was diagnosed with Juvenile Idiopathic Arthritis. Throughout the disease a persistent elevation of liver enzymes was noted, combined with positive antinuclear antibodies and hypergammaglobulinemia, reaching the diagnosis of concomitant autoimmune hepatitis.
Ryu, Jay H; Moua, Teng; Daniels, Craig E; Hartman, Thomas E; Yi, Eunhee S; Utz, James P; Limper, Andrew H
Idiopathic pulmonary fibrosis (IPF) occurs predominantly in middle-aged and older adults and accounts for 20% to 30% of interstitial lung diseases. It is usually progressive, resulting in respiratory failure and death. Diagnostic criteria for IPF have evolved over the years, and IPF is currently defined as a disease characterized by the histopathologic pattern of usual interstitial pneumonia occurring in the absence of an identifiable cause of lung injury. Understanding of the pathogenesis of IPF has shifted away from chronic inflammation and toward dysregulated fibroproliferative repair in response to alveolar epithelial injury. Idiopathic pulmonary fibrosis is likely a heterogeneous disorder caused by various interactions between genetic components and environmental exposures. High-resolution computed tomography can be diagnostic in the presence of typical findings such as bilateral reticular opacities associated with traction bronchiectasis/bronchiolectasis in a predominantly basal and subpleural distribution, along with subpleural honeycombing. In other circumstances, a surgical lung biopsy may be needed. The clinical course of IPF can be unpredictable and may be punctuated by acute deteriorations (acute exacerbation). Although progress continues in unraveling the mechanisms of IPF, effective therapy has remained elusive. Thus, clinicians and patients need to reach informed decisions regarding management options including lung transplant. The findings in this review were based on a literature search of PubMed using the search terms idiopathic pulmonary fibrosis and usual interstitial pneumonia, limited to human studies in the English language published from January 1, 2000, through December 31, 2013, and supplemented by key references published before the year 2000.
Strupp, M; Feil, K; Dieterich, M; Brandt, T
The leading symptoms of bilateral vestibulopathy (BVP) are postural imbalance and unsteadiness of gait that worsens in darkness and on uneven ground. There are typically no symptoms while sitting or lying under static conditions. A minority of patients also have movement-induced oscillopsia, in particular while walking. The diagnosis of BVP is based on a bilaterally reduced or absent function of the vestibulo-ocular reflex (VOR). This deficit is diagnosed for the high-frequency range of the angular VOR by a bilaterally pathologic bedside head impulse test (HIT) and for the low-frequency range by a bilaterally reduced or absent caloric response. If the results of the bedside HIT are unclear, angular VOR function should be quantified by a video-oculography system (vHIT). An additional test supporting the diagnosis is dynamic visual acuity. Cervical and ocular vestibular-evoked myogenic potentials (c/oVEMP) may also be reduced or absent, indicating impaired otolith function. There are different subtypes of BVP depending on the affected anatomic structure and frequency range of the VOR deficit: impaired canal function in the low- and/or high-frequency VOR range only and/or otolith function only; the latter is very rare. The etiology of BVP remains unclear in more than 50% of patients: in these cases neurodegeneration is assumed. Frequent known causes are ototoxicity mainly due to gentamicin, bilateral Menière's disease, autoimmune diseases, meningitis and bilateral vestibular schwannoma, as well as an association with cerebellar degeneration (cerebellar ataxia, neuropathy, vestibular areflexia syndrome=CANVAS). In general, in the long term there is no improvement of vestibular function. There are four treatment options: first, detailed patient counseling to explain the cause, etiology, and consequences, as well as the course of the disease; second, daily vestibular exercises and balance training; third, if possible, treatment of the underlying cause, as in bilateral
A case of central carcinoma of the mandible arising from a recurrent odontogenic keratocyst: delineation of surgical margins and reconstruction with bilateral rectus abdominis myocutaneous free flaps.
Ota, Y; Karakida, K; Watanabe, D; Miyasaka, M; Tsukinoki, K
A case of central carcinoma of the mandible arising from a recurrent odontogenic keratocyst is reported. A 38-year-old man was admitted to the Tokai University Hospital due to postoperative infection of a recurrent odontogenic keratocyst of the left mandible. He had had a cystectomy for an odontogenic keratocyst 4 years ago. The lesion revealed bony destruction of the mandible with worm-eating shaped margins with extension to the facial skin. A biopsy specimen revealed squamous cell carcinoma. The mandible was resected with facial skin and the sublingual space was dissected to preserve the lingual nerve. The oral and the facial resections were reconstructed with a titanium plate and bilateral rectus abdominis myocutaneous free flaps. The plate was removed due to infection around the margins and readjustment of the flaps was conducted 5 months after the surgery. He has not had a local relapse, metastasis, or incisional hernia for 8 months following surgery. Good occlusion has been attained by the residual mandible, and he is able to eat without any problems.
... Is Juvenile Idiopathic Arthritis the same as Juvenile Rheumatoid Arthritis? Yes, Juvenile Idiopathic Arthritis (JIA) is a new ... of chronic inflammatory diseases that affect children. Juvenile Rheumatoid Arthritis (JRA) is the older term that was used ...
Cardiomyopathies are certain heart diseases of unknown etiology and pathogenesis, occurring mostly in tropical and subtropical areas, where they constitute a major clinical problem and sometimes a public health problem. The need for international co-operation in the study of such forms of heart disease has long been recognized and WHO convened informal meetings of investigators on various aspects of the subject in 1964, 1965 and 1966. Out of these have arisen co-operative studies co-ordinated by WHO. In November 1967 a fourth informal meeting was held in Kingston, Jamaica, to review the following topics: the progress reports from all co-operating laboratories; the different types of cardiomyopathies; past experience with cardiac registries, and the diagnostic importance of coronary angiography. Steps were taken towards the formulation of a standard terminology, since too many confusing names are currently employed to mean “cardiomegaly of unknown origin”. A common name, “idiopathic cardiomegaly”, was therefore suggested for future use. The account presented here was prepared by Dr Z. Fejfar, Chief Medical Officer, Cardiovascular Diseases, World Health Organization, Geneva, on behalf of the other participants and is a précis of some of the information that was exchanged, some of the views that were expressed and of the suggestions that were made. PMID:4235740
Movahedi, Masoud; Tavakol, Marzieh; Hirbod-Mobarakeh, Armin; Gharagozlou, Mohammad; Aghamohammadi, Asghar; Tavakol, Zahra; Momenzadeh, Kaveh; Nabavi, Mohammad; Dabbaghzade, Abbas; Mosallanejad, Asieh; Rezaei, Nima
Chronic urticaria is the most common skin diseases, characterized by chronic cutaneous lesions which severely debilitates patients in several aspects of their everyday life. Vitamin D is known to exert several actions in the immune system and to influence function and differentiation of mast cells, central role players in the pathogenesis of chronic idiopathic urticaria. This study was performed to evaluate the relationship between vitamin D levels and susceptibility to chronic idiopathic urticaria. One hundred and fourteen patients with chronic idiopathic urticaria were recruited in this study along with one hundred and eighty seven sex-matched and age-matched healthy volunteers as the control group. For each patient, urticaria activity score was calculated and autologous serum skin test was done. Vitamin D metabolic statue was measured in serum as 25 hydroxyvitamin D using enzyme immunoassay method. Patients with chronic idiopathic urticaria significantly showed lower levels of vitamin D. Vitamin D deficiency was significantly associated with increased susceptibility to chronic idiopathic urticaria. There was a significant positive correlation between vitamin D levels and urticaria activity score. This study showed that patients with chronic idiopathic urticaria had reduced levels of vitamin D, while vitamin D deficiency could increase susceptibility to chronic idiopathic urticaria.
Krause, Thomas; Asseyer, Susanna; Geisler, Frederik; Fiebach, Jochen B; Oeltjenbruns, Jochen; Kopf, Andreas; Villringer, Kersten; Villringer, Arno; Jungehulsing, Gerhard J
Approximately 20% of patients suffering from stroke with pure or predominant sensory symptoms (referred to as sensory stroke patients) develop central poststroke pain (CPSP). It is largely unknown what distinguishes these patients from those who remain pain free. Using quantitative sensory testing (QST), we analyzed the somatosensory profiles of 50 patients with chronic sensory stroke, of which 25 suffered from CPSP. As compared with reference data from healthy controls, patients with CPSP showed alterations of thermal and mechanical thresholds on the body area contralateral to their stroke (P < 0.01). Patients with sensory stroke but without CPSP (non-pain sensory stroke [NPSS] patients) exhibited similar albeit less pronounced contralesional changes. Paradoxical heat sensation (PHS) and dynamic mechanical allodynia (DMA) showed higher values in CPSP, and an elevated cold detection threshold (CDT) was seen more often in CPSP than in patients with NPSS (P < 0.05). In patients with CPSP, changes in CDT, PHS, dynamic mechanical allodynia, and temporal pain summation (wind-up ratio) each correlated with the presence of pain (P < 0.05). On the homologous ipsilesional body area, both patient groups showed additional significant abnormalities as compared with the reference data, which strongly resembled the contralesional changes. In summary, our analysis reveals that CPSP is associated with impaired temperature perception and positive sensory signs, but differences between patients with CPSP and NPSS are subtle. Both patients with CPSP and NPSS show considerable QST changes on the ipsilesional body side. These results are in part paralleled by recent findings of bilaterally spread cortical atrophy in CPSP and might reflect chronic maladaptive cortical plasticity, particularly in patients with CPSP.
He, Qing-Qing; Zhu, Jian; Zhuang, Da-Yong; Fan, Zi-Yi; Zheng, Lu-Ming; Zhou, Peng; Hou, Lei; Yu, Fang; Li, Yan-Ning; Xiao, Lei; Dong, Xue-Feng; Ni, Gao-Feng
Background: A large proportion of the patients with papillary thyroid microcarcinoma are young women. Therefore, minimally invasive endoscopic thyroidectomy with central neck dissection (CND) emerged and showed well-accepted results with improved cosmetic outcome, accelerated healing, and comforting the patients. This study aimed to evaluate the safety and effectiveness of robotic total thyroidectomy with CND via bilateral axillo-breast approach (BABA), compared with conventional open procedure in papillary thyroid microcarcinoma. Methods: One-hundred patients with papillary thyroid microcarcinoma from March 2014 to January 2015 in Jinan Military General Hospital of People's Liberation Army (PLA) were randomly assigned to robotic group or conventional open approach group (n = 50 in each group). The total operative time, estimated intraoperative blood loss, numbers of lymph node removed, visual analog scale (VAS), postoperative hospital stay time, complications, and numerical scoring system (NSS, used to assess cosmetic effect) were analyzed. Results: The robotic total thyroidectomy with CND via BABA was successfully performed in robotic group. There were no conversion from the robotic surgeries to open or endoscopic surgery. The subclinical central lymph node metastasis rate was 35%. The mean operative time of the robotic group was longer than that of the conventional open approach group (118.8 ± 16.5 min vs. 90.7 ± 10.3 min, P < 0.05). The study showed significant differences between the two groups in terms of the VASs (2.1 ± 1.0 vs. 3.8 ± 1.2, P < 0.05) and NSS (8.9 ± 0.8 vs. 4.8 ± 1.7, P < 0.05). The differences between the two groups in the estimated intraoperative blood loss, postoperative hospital stay time, numbers of lymph node removed, postoperative thyroglobulin levels, and complications were not statistically significant (all P > 0.05). Neither iatrogenic implantation nor metastasis occurred in punctured porous channel or chest wall in both groups
Elhensheri, Mohamed; Linke, Reinhold P; Blankenburg, Anja; Beineke, Andreas
This report describes a case of systemic amyloidosis in a captive striped skunk. At necropsy, bilateral alopecia, as well as reno-, hepato-, and splenomegaly were present. Congo red staining and immunohistochemistry revealed depositions of AA-amyloid in different organs. The lack of a predisposing disease is suggestive of idiopathic systemic AA-amyloidosis.
Kar, Jugal Kishore; Kar, Manoranjan; Maiti, Saswati; Sen, Eva
Isolated idiopathic bilateral temporalis muscle hypertrophy is a very rare clinical entity. It can change the facial appearance that manifests itself as a morphopsychological conflict for the subject, which is termed as 'Minotaur syndrome' in the medical literature. Here one such case is reported. The second patient sought medical attention for fear of malignancy.
Lööf, Elin; Andriesse, Hanneke; André, Marie; Böhm, Stephanie; Broström, Eva W
Background and purpose Idiopathic clubfoot can be bilateral or unilateral; however, most studies of gait have assessed clubfoot cases as one uniform group. The contralateral foot in children with unilateral clubfoot has shown deviations in pedobarographic measurements, but it is seldom included in studies of gait. We evaluated gait in children with idiopathic clubfoot, concentrating on foot involvement. Patients and methods Three-dimensional gait analyses of 59 children, mean age 5.4 years, with bilateral (n = 30) or unilateral (n = 29) idiopathic clubfoot were stratified into groups of bilateral, unilateral, or contralateral feet. Age-matched controls (n = 28) were evaluated for comparison. Gait assessment included: (1) discrete kinematic and kinetic parameters, and (2) gait deviation index for kinematics (GDI) and kinetics (GDI-k). Results No differences in gait were found between bilateral and unilateral idiopathic clubfoot, but both groups deviated when compared to controls. Compared to control feet, contralateral feet showed no deviations in discrete gait parameters, but discrepancies were evident in relation to unilateral clubfoot, causing gait asymmetries in children with unilateral involvement. However, all groups deviated significantly from control feet according to GDI and GDI-k. Interpretation Bilateral and unilateral idiopathic clubfoot cases show the same persistent deviations in gait, mainly regarding reduced plantarflexion. Nevertheless, knowledge of foot involvement is important as children with unilateral clubfoot show gait asymmetries, which might give an impression of poorer deviations. The results of GDI/GDI-k indicate global gait adaptations of the contralateral foot, so the foot should preferably not be used as a reference for gait. PMID:27331243
Gangadharan, Geethu; Criton, Sebastian; Surendran, Divya
Acquired idiopathic generalized anhidrosis is a rare condition, where the exact pathomechanism is unknown. We report a case of acquired idiopathic generalized anhidrosis in a patient who later developed lichen planus. Here an autoimmune-mediated destruction of sweat glands may be the probable pathomechanism.
I report two patient presented to me with bilateral symmetrical ankle edema and bilateral acute iritis. A 42-year-old female of Indian origin and 30-year-old female from Somalia both presented with bilateral acute iritis. In the first patient, bilateral ankle edema preceded the onset of bilateral acute iritis. Bilateral ankle edema developed during the course of disease after onset of ocular symptoms in the second patient. Both patients did not suffer any significant ocular problem in the past, and on systemic examination, all clinical parameters were within normal limit. Lacrimal gland and conjunctival nodule biopsy established the final diagnosis of sarcoidosis in both cases, although the chest x-rays were normal.
... rule out other conditions or infections, such as Lyme disease , that may cause similar symptoms or occur along ... ESR) Bones, Muscles, and Joints Evaluate Your Child's Lyme Disease Risk Word! Arthritis Arthritis Lupus Juvenile Idiopathic Arthritis ( ...
Lee, Jason Kihyuk; Enns, Robert
Recent advances in understanding of pancreatitis and advances in technology have uncovered the veils of idiopathic pancreatitis to a point where a thorough history and judicious use of diagnostic techniques elucidate the cause in over 80% of cases. This review examines the multitude of etiologies of what were once labeled idiopathic pancreatitis and provides the current evidence on each. This review begins with a background review of the current epidemiology of idiopathic pancreatitis prior to discussion of various etiologies. Etiologies of medications, infections, toxins, autoimmune disorders, vascular causes, and anatomic and functional causes are explored in detail. We conclude with management of true idiopathic pancreatitis and a summary of the various etiologic agents. Throughout this review, areas of controversies are highlighted. PMID:18081217
... the NHLBI on Twitter. What Is Idiopathic Pulmonary Fibrosis? Pulmonary fibrosis (PULL-mun-ary fi-BRO-sis) is a ... time. The formation of scar tissue is called fibrosis. As the lung tissue thickens, your lungs can' ...
Hamad, Muddathir H; Alwadei, Ali H; Bashiri, Fahad A; Hassan, Hamdy H; Idris, Hiyam; Hassan, Saeed; Muayqil, Taim; Altweijri, Ikhlass; Salih, Mustafa A
Idiopathic intracranial hypertension (IIH) is a rare neurological disorder in children. It is characterized by raised intracranial pressure (ICP) in the absence of brain parenchymal lesion, vascular malformations, hydrocephalus, or central nervous system (CNS) infection. The diagnosis is usually confirmed by high opening pressure of cerebrospinal fluid (CSF) with exclusion of secondary causes of intracranial hypertension. If not treated properly, it may lead to severe visual dysfunction. Here we review the etiology, clinical presentation, diagnostic criteria and management of IIH in children through illustration of the clinical and radiological presentation of a 13-year-old overweight girl who presented with severe headache, diplopia and bilateral papilledema. Otherwise, she had unremarkable neurological and systemic examinations. Lumbar puncture showed a high CSF opening pressure (360–540 mmH2O). Her investigations showed normal complete blood count (CBC), normal renal, liver, and thyroid function tests. Cerebrospinal fluid (CSF) and blood chemistry were unremarkable. Magnetic resonant image (MRI) of the brain demonstrated empty sella turcica, tortuous optic nerves, and flattening of the posterior sclera. Magnetic resonant venography (MRV) showed focal narrowing of the distal transverse sinuses and absence of venous sinus thrombosis. She required treatment with acetazolamide and prednisolone. With medical treatment, weight reduction, and exercise, our patient had a remarkable improvement in her symptoms with resolution of papilledema in two months. This review highlights the importance of early recognition and management of IIH to prevent permanent visual loss. PMID:28096561
Bellis, Teri James; Billiet, Cassie; Ross, Jody
Two experiments were conducted to examine the performance of normal adults, normal children, and children diagnosed with central auditory dysfunction presumed to involve the interhemispheric pathways on a dichotic digits test in common clinical use for the diagnosis of central auditory processing disorder (CAPD) and its corresponding visual…
Mullaney, J; Mooney, D; O'Connor, M; McDonald, G S
A case of bilateral uveal melanoma in a 60-year-old woman in association with primary bilateral ovarian carcinoma is described. This is the first case in which ultrastructural studies have been performed on the ocular tumours. Seven previously described cases are summarised, and the extreme rarity of such reports would suggest that this may indeed be a new syndrome. Images PMID:6704361
Khan, Arif O; Aldahmesh, Mohammad
Duane retraction syndrome has been reported in association with structural abnormalities of the eye, including epibulbar dermoid, keratoconus, iris dysplasia, heterochromia iridis, persistent fetal vasculature, cataract, choroidal coloboma, microphthalmia, and optic nerve dysplasia. A novel association, that of bilateral Duane syndrome with bilateral aniridia, is the subject of this report.
Effects of pomegranate extract in supplementing gonadotropin-releasing hormone therapy on idiopathic central precocious puberty in Chinese girls: a randomized, placebo-controlled, double-blind clinical trial.
Liu, Jinsheng; Tang, Jiulai
Central precocious puberty (CPP) without organic abnormality is called idiopathic CPP (ICPP). The objective of this trial was to evaluate the effects of pomegranate extract in supplementing gonadotropin-releasing hormone (GnRH) analog therapy on ICPP-affected girls in the Chinese population. 286 girls, diagnosed with ICPP were initially enrolled into this trial, and among them 225 eligible patients were randomized to receive a combinational GnRH analog treatment supplemented with either a placebo or pomegranate extract on a daily basis for a period of 3 months. Their demographics, secondary sexual characteristics and hormone profiles were analyzed at baseline and end of trial. After 3 months of treatment, demographic profiles including bone age, growth velocity and height standard deviation score for bone age, and secondary sexual characteristics including uterus and ovary volume, as well as serum hormone profiles including estradiol, peak luteinizing hormone and insulin-like growth factor 1 were all significantly improved in girls receiving a combinational treatment of both GnRH analog and pomegranate extract. Daily consumption of pomegranate extract was able to supplement and improve the treatment outcomes of the GnRH analog therapy for ICPP in Chinese girls.
Kumar, Rakesh; Mittal, Radhey Shyam
Bilateral facial nerve palsy [FNP] is a rare condition. Mostly it is idiopathic. Post traumatic bilateral FNP is even more rare and having unique neurosurgical considerations. Post traumatic delayed presentation of bilateral FNP is socially debilitating and also having diagnostic challenge. Due to lack of facial asymmetry as present in unilateral facial paralysis, it is difficult to recognize. We are presenting a case of delayed onset bilateral FNP who developed FNP after 12 days of head injury with a brief discussion of its diagnostic dilemma and management along with literature review.
Joseph, Rona; Soman, Lali V.
Extramedullary plasmacytoma (EMP) is usually seen in the head and neck regions and in the upper respiratory, gastrointestinal, and central nervous systems. Testis is a rare site for EMP, and bilateral synchronous testicular plasmacytoma occurring as an isolated event at initial presentation has been reported only once previously. We present herein the second such report in a 70-year-old man who underwent bilateral orchidectomy. PMID:27034568
Kotwica, Tomasz; Szumarska, Joanna; Staniszewska-Marszalek, Edyta; Mazurek, Walentyna; Kosmala, Wojciech
Pulmonary artery aneurysm (PAA) is an uncommon lesion, which may be associated with different etiologies including congenital cardiovascular diseases, systemic vasculitis, connective tissue diseases, infections, and trauma. Idiopathic PAA is sporadically diagnosed by exclusion of concomitant major pathology. We report a case of a 56-year-old female with an idiopathic pulmonary artery dilatation identified fortuitously by echocardiography and confirmed by contrast-enhanced computed tomography. Neither significant pulmonary valve dysfunction nor pulmonary hypertension and other cardiac abnormalities which might contribute to the PAA development were found. Here, we describe echocardiographic and computed tomography findings and review the literature on PAA management.
Bäuerle, J; Egger, K; Harloff, A
This review describes the clinical findings as well as thes diagnostic and therapeutic options for idiopathic intracranial hypertension (pseudotumor cerebri). Furthermore, the pathophysiological concepts are discussed. Idiopathic intracranial hypertension is characterized by signs and symptoms of raised intracranial pressure with no established pathogenesis. Common symptoms include headaches, visual loss and pulsatile tinnitus. Treatment has two major goals: the alleviation of headaches and the preservation of vision. Weight loss and acetazolamide are the cornerstones in the treatment of the disorder. Drainage of cerebrospinal fluid, optic nerve sheath fenestration and stent angioplasty of a sinus stenosis can be employed in severe cases.
Bellis, Teri James; Billiet, Cassie; Ross, Jody
Two experiments were conducted to examine the performance of normal adults, normal children, and children diagnosed with central auditory dysfunction presumed to involve the interhemispheric pathways on a dichotic digits test in common clinical use for the diagnosis of central auditory processing disorder (CAPD) and its corresponding visual analog. Results of the first experiment revealed a significant right ear advantage (REA) for the dichotic listening task and a left-visual-field advantage (LVFA) for the corresponding visual analog in normal adults and children. In the second experiment, results revealed a significantly larger REA in the children with CAPD as compared to the normal children. Results also revealed a reversed cerebral asymmetry (RVFA) for the children with CAPD on the visual task. Significant cross-modal correlations suggest that the two tasks may reflect, at least in part, similar interhemispheric processing mechanisms in children. Findings are discussed in relation to differential diagnosis and modality-specificity of CAPD.
Bianco, Suzy D. C.
The major determinants of the variability in pubertal maturation are reported to be genetic and inherited. Nonetheless, nutritional status contributes significantly to this variability. Malnutrition delays puberty whereas obesity has been associated to a rise in Idiopathic Central Precocious Puberty (ICPP) in girls. However, epidemiology data indicate that contribution of obesity to early puberty varies significantly among ethnic groups, and that obesity-independent inheritable genetic factors are the strongest predictors of early puberty in any ethnic group. In fact, two human mutations with confirmed association to ICPP have been identified in children with no history of obesity. These mutations are in kisspeptin and kisspeptin receptor, a ligand/receptor pair with a major role on the onset of puberty and female cyclicity after puberty. Progressive increases in kisspeptin expression in hypothalamic nuclei known to regulate reproductive function has been associated to the onset of puberty, and hypothalamic expression of kisspeptin is reported to be sexually dimorphic in many species, which include humans. The hypothalamus of females is programmed to express significantly higher levels of kisspeptin than their male counterparts. Interestingly, incidence of ICPP and delayed puberty in children is markedly sexually dimorphic, such that ICPP is at least 10-fold more frequent in females, whereas prevalence of delayed puberty is about 5-fold higher in males. These observations are consistent with a possible involvement of sexually dimorphic kisspeptin signaling in the sexual dimorphism of normal puberty and of pubertal disorders in children of all ethnicities. This review discusses the likelihood of such associations, as well as a potential role of kisspeptin as the converging target of environmental, metabolic, and hormonal signals, which would be integrated in order to optimize reproductive function. PMID:23248615
Tuncer, Oğuz; Melek, Mehmet; Kaba, Sultan; Bulan, Keziban; Peker, Erdal
Though the perforation of the colon in neonates is rare, it is associated with more than 50% mortality in high-risk patients. We report a case of idiopathic neonatal perforation of the sigmoid colon in an 8-day-old, healthy, male neonate without any demonstrable cause. PMID:26023477
Brannan, Paul A; Kersten, Robert C; Kulwin, Dwight R
A 5-year-old girl referred for orbital cellulitis was found to have a right orbital mass. Computed tomography revealed a mass occupying the inferotemporal orbit, extending into the maxillary sinus. Biopsy yielded a diagnosis of sclerosing idiopathic orbital inflammation. She was successfully treated with prednisone.
Hornberger, Brad J; Elmore, James M; Roehrborn, Claus G
Scrotal lymphedema (scrotal elephantiasis) is a condition that has historically been described in areas endemic to filariasis. We present a unique case of a 22-year-old man with idiopathic lymphedema isolated to the scrotum. After acquired causes of lymphedema were ruled out, the patient was treated with scrotectomy and scrotal reconstruction.
Bolaji, I I; Meehan, F P
Case Report--A 33-year-old woman was examined because of primary infertility. Hysterosalpingography plus laparoscopy led to a diagnosis of the extremely rare condition of pigmentosis of the fallopian tube, with complete tubal occlusion as the cause of the infertility. The condition appeared to be idiopathic.
Bennani, S; Ait Bolbarod, A; el Mrini, M; Kadiri, R; Benjelloun, S
The authors report a case of idiopathic renal arteriovenous fistula. The diagnosis was established angiographically in a 24 year old man presenting gross hematuria. Embolization of the fistula was performed. Efficiency of this treatment was appreciated clinically and by duplex renal ultrasonography. The characteristics of renal arteriovenous fistulas are reviewed.
Collard, Harold R.; Moore, Bethany B.; Flaherty, Kevin R.; Brown, Kevin K.; Kaner, Robert J.; King, Talmadge E.; Lasky, Joseph A.; Loyd, James E.; Noth, Imre; Olman, Mitchell A.; Raghu, Ganesh; Roman, Jesse; Ryu, Jay H.; Zisman, David A.; Hunninghake, Gary W.; Colby, Thomas V.; Egan, Jim J.; Hansell, David M.; Johkoh, Takeshi; Kaminski, Naftali; Kim, Dong Soon; Kondoh, Yasuhiro; Lynch, David A.; Müller-Quernheim, Joachim; Myers, Jeffrey L.; Nicholson, Andrew G.; Selman, Moisés; Toews, Galen B.; Wells, Athol U.; Martinez, Fernando J.
The natural history of idiopathic pulmonary fibrosis (IPF) has been characterized as a steady, predictable decline in lung function over time. Recent evidence suggests that some patients may experience a more precipitous course, with periods of relative stability followed by acute deteriorations in respiratory status. Many of these acute deteriorations are of unknown etiology and have been termed acute exacerbations of IPF. This perspective is the result of an international effort to summarize the current state of knowledge regarding acute exacerbations of IPF. Acute exacerbations of IPF are defined as acute, clinically significant deteriorations of unidentifiable cause in patients with underlying IPF. Proposed diagnostic criteria include subjective worsening over 30 days or less, new bilateral radiographic opacities, and the absence of infection or another identifiable etiology. The potential pathobiological roles of infection, disordered cell biology, coagulation, and genetics are discussed, and future research directions are proposed. PMID:17585107
Do, Young Woo; Jung, Hee Suk; Lee, Chang Young; Lee, Jin Gu; Youn, Young-Nam; Paik, Hyo Chae
Coronary artery disease has historically been a contraindication to lung transplantation. We report a successful combined bilateral lung transplantation and off-pump coronary artery bypass in a 62-year-old man. The patient had a progressive decline in lung function due to idiopathic pulmonary fibrosis and a history of severe occlusive coronary artery disease. PMID:27965924
Wani, Nazir A; Parray, Fazl Q
The therapeutic effects of splenectomy in 15 patients with idiopathic hypersplenism were studied. The mean age was 43 years (range, 5-72 years). The male to female ratio was 1:1.14. The response to splenectomy was: in thrombocytopenia, complete response (CR) in 71%, partial response (PR) and no response (NR) in 14% each of patients; in anemia, CR in 83%, PR in 0%, and NR in 16% of patients; and in leukopenia, CR in 78% and PR and NR in 11% each of patients. However, the morbidity (27%) and mortality (20%) observed was quite high. Thus, we conclude that splenectomy is an excellent treatment to improve the hematological parameters in patients with idiopathic hypersplenism, but elderly patients and patients with multisystem disease should not have this surgery.
Adolescent idiopathic scoliosis is a 3D spinal deformity in frontal, sagittal and axial planes, with high relevance in the pediatric population especially in adolescents and females between 10 years of age and the end of growth spurt and skeletal maturity. The radiographic manifestation is a curve greater than 10° measured by Cobb method associated with vertebral rotation. "Idiopathic" diagnosis has to be done after neuroanatomical anomalies of the posterior cerebral fosa and spinal canal have been ruled out. The physical finding of a thoracic or lumbar hump is the clinical manifestation of vertebral rotation seen in a forward bending test (Adam's Test). It is recommended that all curves with a magnitude greater than 20° have to be controlled and treated by a spinal surgeon being observation, bracing and surgery the different treatment options based on the extent, progression of deformity and basically the clinical condition of the patient.
Xaubet, Antoni; Ancochea, Julio; Molina-Molina, María
Idiopathic pulmonary fibrosis is a fibrosing interstitial pneumonia associated with the radiological and/or histological pattern of usual interstitial pneumonia. Its aetiology is unknown, but probably comprises the action of endogenous and exogenous micro-environmental factors in subjects with genetic predisposition. Its diagnosis is based on the presence of characteristic findings of high-resolution computed tomography scans and pulmonary biopsies in absence of interstitial lung diseases of other aetiologies. Its clinical evolution is variable, although the mean survival rate is 2-5 years as of its clinical presentation. Patients with idiopathic pulmonary fibrosis may present complications and comorbidities which modify the disease's clinical course and prognosis. In the mild-moderate disease, the treatment consists of the administration of anti-fibrotic drugs. In severe disease, the best therapeutic option is pulmonary transplantation. In this paper we review the diagnostic and therapeutic aspects of the disease.
von Knorring, L; Ekselius, L
This report summarizes research on the hypothesis that idiopathic chronic pain syndromes and depressive disorders share certain common pathogenetic mechanisms. There is increasing evidence that this may be partly true. Not only do chronic pain syndromes respond to treatment with antidepressants, but there are also striking clinical similarities between these syndromes and depressive syndromes. However, important differences do exist (e.g., the courses of these disorders are usually dissimilar). Family studies show that affective disorders are common in first-degree relatives of patients with idiopathic pain syndromes, but it is impossible to conclude from this that clear-cut genetic factors are of importance. Factors common to both syndromes include common personality traits, shortened rapid eye movements in sleep EEG, hypercortisolaemia and pathological dexamethasone suppression tests, low levels of melatonin in serum and urine and high levels of endorphins and Fraction I in cerebro-spinal fluid. One important common pathogenetic mechanism seems to be disturbances in the serotoninergic system.
Cho, Min Jeng; Choi, Hyeon-Gon; Kim, Wan Seop; Yu, Yeong-Beom; Park, Kyoung Sik
Gigantomastia is a rare condition characterized by excessive breast growth. It has been reported that the majority of gigantomastia cases occur during either pregnancy or puberty. We were presented with a rare case of gigantomastia associated with neither pregnancy nor puberty, and successfully treated it with reduction mammaplasty and free nipple graft. This idiopathic gigantomastia is the very first case in Korea, and adds to the worldwide total of 9 reported cases. PMID:25741497
... the NHLBI on Twitter. What Causes Idiopathic Pulmonary Fibrosis? Sometimes doctors can find out what is causing pulmonary fibrosis (lung scarring). For example, exposure to environmental pollutants ...
Tschen, Jaime A.
Background: Calcinosis cutis—the deposition of insoluble calcium salts in the skin and the soft tissue—occurs in the following five settings: calciphylaxis, dystrophic, iatrogenic, idiopathic, and metastatic. Idiopathic calcinosis cutis of the penis is rare. Purpose: This paper describes a man with idiopathic calcinosis cutis of the penis, summarizes the clinical features of previously reported men with this condition, and also reviews dystrophic, iatrogenic, and metastatic penile calcinosis. Methods: A 27-year-old Pakistan man presented with concurrent, asymptomatic, individual nodules on the right mid-ventral penile shaft and left side of scrotum and two additional papules on the right side of the scrotum. Evaluation and treatment included the excision of all lesions. Reports of patients with penile calcinosis were identified using a medical search engine (PubMed Central) and referenced citations from the published papers on this subject. Results: Microscopic examination of the patient's nodules showed idiopathic and dystrophic calcinosis cutis of the penis and scrotum, respectively; the scrotal papules were fibroepithelial polyps. Including this individual, idiopathic calcinosis cutis of the penis has only been reported in 11 men. It presents as either an asymptomatic nodule (5 patients) or multiple lesions (6 patients) of less than one-year duration, on either the penile shaft (distal in 4 patients, mid in 2 patients, both in 1 patient, and site unspecified in 1 patient) or the prepuce (3 patients) of uncircumcised men less than 30 years of age. Concurrent scrotal calcification was noted in two patients. Dermal deposits of calcium are found in the dermis—often with surrounding histiocytes and multinucleated giant cells; concurrent features of dystrophic penile shaft calcification, such as calcium within syringomas or transepidermal elimination of calcium through eccrine sweat ducts, was only noted in two men. The nodules do not recur following excision
Wali, Aisha Syed; Khan, Rozilla Sadia
With the increase in incidence of ectopic pregnancy over the decades, bilateral ectopic pregnancy is also increasing. It is usually associated with assisted reproductive techniques (ART) but in recent years few cases of spontaneous bilateral ectopic pregnancy have been reported. Gynaecologists should be aware of this and that ultrasonography has limitations in diagnosis. In cases of ectopic pregnancy where contralateral adnexa is not clearly identified on ultrasound and fertility needs to be conserved, patient should be managed by experts in well equipped centres. A case of spontaneous bilateral tubal pregnancy that remained undiagnosed till laparotomy, is described.
Sozzo, S; Carratù, P; Damiani, M F; Falcone, V A; Palumbo, A; Dragonieri, S; Resta, O
A 57-year-old woman underwent an enucleoresection of her right kidney angiomyolipoma. Two weeks later she was admitted to our hospital because of dyspnea at rest with orthopnea. The chest x-ray showed the elevation of both hemidiaphragms and the measurement of the sniff transdiaphragmatic pressure confirmed the diagnosis of bilateral diaphragmatic paralysis. A diaphragm paralysis can be ascribed to several causes, i.e. trauma, compressive events, inflammations, neuropathies, or it can be idiopathic. In this case, it was very likely that the patient suffered from post-surgery neuralgic amyotrophy. To our knowledge, there are only a few reported cases of neuralgic amyotrophy, also known as Parsonage-Turner Syndrome, which affects only the phrenic nerve as a consequence of a surgery in an anatomically distant site.
Govil, Somya; Gupta, Vishesh; Misra, Neeta; Misra, Pradyumna
The bilateral lateral periodontal cyst is a rare nasological entity, which despite clinical and radiological presentation is being diagnosed by histological characteristics. It is asymptomatic in nature and is observed in routine radiography. The aim and objective of this article is to present a rare case of bilateral lateral periodontal cyst in a 14-year-old child. The clinical and radiographical findings, along with its management have been discussed. Enucleation of bilateral cyst without extraction of the adjacent tooth was performed. Lesion samples were sent for histopathological analysis. The histopathological analysis revealed a thin, non keratinised stratified squamous epithelium resembling reduced enamel epithelium. Epithelial plaques were also seen. A clinicopathological correlation incorporating the surgical, radiographical and gold standard histopathological findings was obtained to suggest the final diagnosis of the bilateral lateral periodontal cyst.
Valldeperas, Xavier; Angi, Martina; Romano, Vito; Romano, Mario R.
We report the clinical findings of a patient with severe bilateral keratectasia 34 years after a penetrating keratoplasty (PK) in both eyes. An otherwise healthy 67-year-old man complained of deterioration of the eyesight in both eyes over the last 6 months. The patient was diagnosed with bilateral keratoconus at the age of 32 years, and he underwent a bilateral PK. At presentation, visual acuity was 20/200 in the right eye and light perception in the left eye. A Pentacam pachymetric map revealed a central pachymetry of 720 μm in the right eye and of 710 μm in the left eye, as well as an average paracentral pachymetry of 436 and 270 μm in the 9-mm zone in the right and the left eye, respectively. Corneal topography revealed bilateral irregular and asymmetric bowing with generalized steepening and high corneal power. We describe a case of bilateral keratectasia 34 years after PK in a patient who was originally diagnosed with bilateral keratoconus. PMID:20737056
Purpose. To report unusual presentation of type 1A idiopathic macular telangiectasia (IMT). Methods. Two middle-aged women with bilateral IMT were examined. Results. Both patients presented with a gradual vision loss in both eyes. Fundus examination was unremarkable in one case and showed small macular telangiectasia in both eyes in the other case. Fluorescein angiography (FA) revealed early bilateral macular punctuated hyperfluorescence corresponding to the dilated capillaries in both cases. FA and fundus examination confirmed also the absence of vascular abnormalities in the middle or anterior fundus periphery in one case. Spectral-domain optical coherence tomography (SD-OCT) showed cystoid macular edema in both cases. No signs of retinal vein occlusions were detected in both cases and other differential diagnoses were excluded. Based on these findings, the patients were diagnosed with bilateral type 1A IMT according to Gass and Blodi classification and were treated with intravitreal antivascular endothelial growth factor (anti-VEGF) injections and focal laser photocoagulation. Twelve months later, SD-OCT revealed partial regression of the exudative signs and significant VA improvement. Conclusion. We described two patients with an unusual presentation of type 1A IMT with bilateral presentation, affecting two middle-aged women, with occult and without peripheral involvement in one case. The description of more cases of bilateral type 1 IMT should be helpful to more precisely define the pathophysiologic mechanism that could be different from a localized Coats' disease of the macula area. PMID:28203470
Roy, Somak; Hooda, Shveta; Parwani, Anil V
Idiopathic granulomatous orchitis is a rare inflammatory process of the testis of unknown etiology. It is characterized by presence of non-specific granulomatous inflammation and admixed multinucleated giant cells. It usually presents as a testicular mass which is highly suspicious of malignancy. Histologically, there is extensive destruction of seminiferous tubules with tubular or interstitial pattern of granulomatous inflammation and prominent collagen fibrosis. Trauma and possible auto-antibodies against sperms have been postulated to be the underlying mechanism. Differential diagnoses include intratubular germ-cell neoplasia, malignant lymphomas, and malakoplakia. Orchiectomy is currently the most appropriate therapy for this condition.
Dani, Nitin Hemchandra; Khanna, Dinkar Parveen; Bhatt, Vaibhavi Hitesh; Joshi, Chaitanya Pradeep
Idiopathic gingival fibromatosis (IGF) is a rare hereditary condition characterized by slowly progressive, nonhemorrhagic, fibrous enlargement of maxillary and mandibular keratinized gingiva caused by increase in submucosal connective tissue elements, mostly associated with some syndrome. This case report describes a case of nonsyndromic generalized IGF in an 18-year-old male patient who presented with generalized gingival enlargement. The enlarged tissue was surgically removed by internal bevel gingivectomy and ledge and wedge procedure. The patient was regularly monitored clinically for improvement in his periodontal condition as well as for any recurrence of gingival overgrowth. PMID:26941525
Choudhry, Muhammad Naghman; Ahmad, Zafar; Verma, Rajat
Background: Scoliosis refers to deviation of spine greater than 10 degrees in the coronal plane. Idiopathic Scoliosis is the most common spinal deformity that develops in otherwise healthy children. The sub types of scoliosis are based on the age of the child at presentation. Adolescent idiopathic scoliosis (AIS) by definition occurs in children over the age of 10 years until skeletal maturity. Objective: The objective of this review is to outline the features of AIS to allow the physician to recognise this condition and commence early treatment, thereby optimizing patient outcome. Method: A thorough literature search was performed using available databases, including Pubmed and Embase, to cover important research published covering AIS. Conclusion: AIS results in higher incidence of back pain and discontent with body image. Curves greater than 50 degrees in thoracic region and greater than 30 degrees in lumbar region progress at a rate of 0.5 to 1 degree per year into adulthood. Curves greater than 60 degrees can lead to pulmonary functional deficit. Therefore once the disease is recognized, effective treatment should be instituted to address the deformity and prevention of its long-term sequelae. PMID:27347243
Perez, Jesus; Scherle, Claudio; Machado, Calixto
Simultaneous or subsequent bilateral thalamic haemorrhage is rare, and most reported cases are from Asian countries. An 80-year-old white Cuban man, with a history of arterial hypertension, suffered sudden onset of right hemiparesis. Computed tomography (CT) scan showed a left posteromedial thalamic haemorrhage. Two days later his condition suddenly deteriorated: blood pressure was 220/105 mm Hg, he was stuporous and tetraplegic, respiration was ataxic, and his gaze was fixed and deviated downward and inward. CT scan showed haemorrhages in both thalami, extending to the ventricles. 32 h later the patient died. There are few previous publications of simultaneous or subsequent bilateral thalamic haemorrhages and this is the first report involving a Hispanic patient. Prognosis in patients with bilateral thalamic haemorrhage is poor, and the mechanism underlying the development of subsequent and symmetrical bleeding is not clear. PMID:21709830
Objectives This report describes the case of a patient with chronic idiopathic meralgia paresthetica associated with bilateral sacroiliac joint dysfunction who was managed with chiropractic care. Clinical Features A 35-year-old white woman presented to a private chiropractic clinic with a complaint of numbness in the right anterolateral thigh region. Neurological assessment revealed a diminution of sensibility and discrimination on the right lateral femoral cutaneous nerve territory. Pain was rated as 8.5 on a numeric pain scale of 0 to 10. Musculoskeletal examination of the pelvic region disclosed bilateral sacroiliac joint dysfunction. Intervention and Outcomes Chiropractic management included pelvic mobilizations, myofascial therapy, transverse friction massage, and stretching exercises. After 3 visits (2 weeks later), result of neurological evaluation was normal, with no residual numbness over the lateral thigh. Conclusion In the present case, chiropractic management with standard and applied kinesiology techniques resulted in recovery of meralgia paresthetica symptoms for this patient. PMID:22942840
Nassar, Basant R.; Lippa, Carol F.
Idiopathic normal pressure hydrocephalus (iNPH) is a potentially reversible neurodegenerative disease commonly characterized by a triad of dementia, gait, and urinary disturbance. Advancements in diagnosis and treatment have aided in properly identifying and improving symptoms in patients. However, a large proportion of iNPH patients remain either undiagnosed or misdiagnosed. Using PubMed search engine of keywords “normal pressure hydrocephalus,” “diagnosis,” “shunt treatment,” “biomarkers,” “gait disturbances,” “cognitive function,” “neuropsychology,” “imaging,” and “pathogenesis,” articles were obtained for this review. The majority of the articles were retrieved from the past 10 years. The purpose of this review article is to aid general practitioners in further understanding current findings on the pathogenesis, diagnosis, and treatment of iNPH. PMID:28138494
Alfaham, M A; Ferguson, S D; Sihra, B; Davies, J
A 14 year old girl with idiopathic hypereosinophilic syndrome is described. In addition to weight loss, anaemia, amenorrhoea, general lethargy, anorexia, mouth ulcers, blisters of hands and feet, and petechial skin rash, she had features of involvement of the cardiovascular system as the major complication. She responded well to treatment. After a comprehensive search of the published reports 18 cases of this syndrome were identified in children under 16 years. Fifteen of these children had involvement of the cardiovascular system as the major source of their morbidity and mortality. Summary of the clinical details and laboratory, biopsy, and necropsy findings of the involvement of the various organ systems of the 18 children is presented. PMID:3619478
Cottin, Vincent; Cordier, Jean-François
Idiopathic pulmonary fibrosis is a chronic disorder characterized histopathologically by a pattern of usual interstitial pneumonia, with heterogeneous and mutilating interstitial fibrosis with foci of proliferating fibroblasts, honeycomb lung, and little if any inflammation. The diagnosis is based on a pluridisciplinary analysis of the clinical symptoms, the chest high-resolution computerized tomography features, and pathology on video-thoracoscopic lung biopsy when indicated. In half of the cases, the typical tomodensitometric pattern allows to make a confident diagnosis without a lung biopsy. The median survival is only about 3 years and is presently not improved by any treatment. Treatment with N-acetylcysteine (antioxydant) in association with corticosteroids and azathioprine may slightly reduce the rate of functional worsening. Clinical trials are in progress to improve the treatment of this still incurable disease.
Schreiber, Doreen; Kottkamp, Hans
Idiopathic ventricular arrhythmias occur in patients without structural heart disease. They can arise from a variety of specific areas within both ventricles and in the supravalvular regions of the great arteries. Two main groups need to be differentiated: arrhythmias from the outflow tract (OT) region and idiopathic left ventricular, so-called fascicular, tachycardias (ILVTs). OT tachycardia typically originates in the right ventricular OT, but may also occur in the left ventricular OT, particularly in the sinuses of Valsalva or the anterior epicardium or the great cardiac vein. Activation mapping or pace mapping for the OT regions and mapping of diastolic potentials in ILVTs are the mapping techniques that are typically used. The ablation of idiopathic ventricular arrhythmias is highly successful, associated with only rare complications. Newly recognized entities of idiopathic ventricular tachycardias are those originating in the papillary muscles and in the atrioventricular annular regions.
Wolf, S R
Although acute idiopathic facial paresis is often labelled "Bell's palsy", historical studies show that Nicolaus Anton Friedreich (1761-1836) from Würzburg was the first physician to describe the typical symptoms of the disorder in 1797, approximately 24 years prior to the paper published by Sir Charles Bell. Diagnostics has now improved to the extent that acute idiopathic facial palsy can more frequently be assigned to etiologies caused by inflammatory disorders. Herpes simplex virus type I and Borrelia burgdorferi are particularly relevant. Underestimation of the degree of paresis is, particularly in children, a drawback of the clinical examination. "Incomplete eyelid closure" is not a reliable indicator of remaining nerve function. For this reason complete electromyography (EMG) is recommended in all cases of severe facial paresis. Since electroneurography does not reliably reflect the degree of denervation present, needle EMG is preferred. The therapy of the facial palsy of unclear etiology is still not well defined. Nevertheless, we recommend that a combined treatment should be used early, at least in patients with disfiguring pareses. Combinations may consist of cortisone, virostatic agents and hemorrheologic substances and possibly antibiotics. Surgical decompression of the facial nerve remains controversial, since positive surgical results lack statistical support. Individual instructions for facial exercises, massage and muscle relaxation can support rehabilitation and possibly reduce the production of pathological synkinesia. Electrical stimulation should not be used. There are a number of possibilities available to reduce the effects of misdirected reinnervation, especially the use of botulinum-A-toxin. However, intensive diagnosis and therapy in the early phase of paresis are decisive in obtaining a favorable outcome. Further refinements in rehabilitation and comparative multicenter controlled studies are still required for future improvements in
Fogelman, Joshua P.; Ashinoff, Robin; Soter, Nicholas A.
Objective: The purpose of this study was to analyze the efficacy and safety of the 585nm pulsed dye laser for the treatment of idiopathic flushing with dysesthesia. Design: This was a retrospective study of patients treated with a 585nm pulsed dye laser with fluences ranging from 3.5 to 7.5J/cm2 (purpura threshold fluences), a pulse duration of 450μsec, and a spot size of 5 or 10mm. Setting: The Ronald 0. Perelman Department of Dermatology at New York University Medical Center. Participants: Ten adult subjects who presented with flushing with dysesthesia. Measurements: Participants subjectively evaluated the decrease in dysesthesia and the number of flushing episodes. The objective response to treatment was evaluated by a single physician using pre- and postoperative photographs. The severity of postoperative erythema was compared with baseline using an ordinal scale ranging from zero (resolution of erythema) to four (76-100% of baseline erythema). Results: The mean number of treatments received by the subjects was seven. The mean fluence was 6.66J/cm2. Subjectively, 100 percent of subjects reported a decrease in dysethesia and the number of flushing episodes. Objectively, subjects demonstrated at least a 62.5-percent reduction in erythema. Conclusion: Laser surgery provided subjective relief of dysesthesia and decreased the number of flushing episodes with a greater than 62-percent objective reduction in the severity of erythema. The 585nm pulsed dye laser is a safe, efficacious treatment for the signs and symptoms of idiopathic flushing with dysesthesia. PMID:26345489
Suganthy, J; Rassau, Marina; Koshi, Rachel; Battacharjee, Suranjan
Congenital absence of tibia is a rare anomaly. We report a case of bilateral tibial hemimelia born to phenotypically normal parents. The two amputated legs with tibial dysplasia obtained from a 3-year-old boy were studied by radiography and anatomical dissection. The radiological evaluation revealed a normal hip joint. The lower end of femur was normal without any bifurcation, shortening or bowing. Fibula was present on both legs and there was no sign of bowing or doubling. Both right and left tibiae were absent. In addition, on the right side, five tarsal bones, two metatarsals and the corresponding digital rays were absent. On the left side, three tarsal bones were absent. Dissection of the amputated segments showed the presence of extensor digitorum longus, peroneus tertius, peroneus longus and brevis, gastrocnemius, and soleus. Following bilateral knee disarticulation the patient was fitted with prosthesis and is doing well.
Mulliken, John B
The surgeon's objectives are normal nasolabial appearance and normal speech. The principles for synchronous repair of bilateral cleft lip have been established, and the techniques continue to evolve. Primary repair impairs maxillary growth, but little can be done at this time except to practice gentle craftsmanship and to minimize tension on the lower labial closure. The cutaneous lip should never be reopened for revision, and the number of secondary procedures involving the nasal cartilages should be kept to a minimum. Many adolescents with repaired bilateral cleft lip need maxillary advancement to improve projection of the nasal tip, to protrude the upper lip, and to attain normal sagittal skeletal harmony. With expected improvements in the technology of distraction osteogenesis, maxillary advancement may someday become as acceptable as orthodontic treatment.
Malcolm, A.W.; Jaffe, N.; Folkman, M.J.; Cassady, J.R.
Twenty children with bilateral Wilms' tumor were presented to the Children's Hospital Medical Center and Children's Cancer Research Foundation, Sidney Farber Cancer Institute, and Joint Center for Radiation Therapy (CHMC-CCRF, SFCI, JCRT) from January 1, 1956 to December 31, 1976. Of these 20, 16 had simultaneous and 4 had metachronous disease on presentation. All patients were treated with surgery, radiation and chemotherapy. Of the 16 patients with simultaneous disease, 10 (63%) are alive and free of disease 12+ to 175+ months post diagnosis and treatment, with median follow-up of 121 months. There were no long-term survivors in the metachronous group; all were dead of disease within 21 months from initial presentation of original tumor. With these data we relate prognosis to extent of disease and discuss a general approach to the management of bilateral Wilms' tumor.
Suqati, Abrar A.; Alherabi, Ameen Z.; Marglani, Osama A.; Alaidarous, Tariq O.
Laryngocele is an uncommon condition that represents a benign dilatation of the laryngeal saccule with air and/or fluid, arising in the region of the laryngeal ventricle. Laryngoceles, or laryngomucocele can be classified as internal, or combined. The aim of presenting this rare case of a bilateral combined laryngocele, are to emphasize the importance of diagnostic laryngoscopy in upper airway pathologies evaluation, increase awareness in the general otolaryngologist community, and to highlight the external surgical method. PMID:27464869
Leonhardt, L; Geldszus, R; Molitor, S J
In a 39-year-old patient with chronic progressive idiopathic pulmonary fibrosis, the genetic aspects, course and therapeutic possibilities of the disease are discussed. In February, 1987, the English-born patient, Anthony V., attended for initial examination on account of progressive dyspnoea, on which occasion radiology and pulmonary function analysis revealed advanced pulmonary fibrosis. The patient's family history revealed a familial genesis, since both his father (?) and his sister had died of this disease. A comparative of the patient's chest films with original chest films of his sister revealed almost identical findings. Within the previous twelve months, follow-up examinations done on A.V. revealed an increase in the restrictive component (reduction of vital capacity from 2,400 ml to 1,500 ml), development of partial respiratory failure at rest, and global respiratory failure in response to mild ergometric exercise despite intermittent high-dose steroid administrations superimposed on long-term, low-dose steroid therapy. The unfavourable evolution observed over the past 12 months is underscored by an increase in mean pulmonary arterial pressure from 18 mmHg initially to a present 34 mmHg at rest, and 46 mmHg under submaximal ergometric loading. The only option still left to the patient is the possibility of a lung transplantation, which - probably initially unilateral - is scheduled to be carried out in the near future at the Chest Surgery Department of the Medical University at Hannover.
Rinsky, Lawrence A.; Gamble, James G.
Adolescent idiopathic scoliosis is the single most common form of spinal deformity seen in orthopedic practice. Our knowledge about the epidemiology, etiology, natural history, and treatment has recently increased dramatically. The incidence of small curves is rather high (2% of the population), whereas severe curves are much less common (<0.1%), but we cannot always predict which curve will progress. Abnormalities of the neuromuscular system and of calcium metabolism, and certain growth, genetic, and mechanical factors may all play roles in the pathogenesis of the disorder. The physiologic secondary effects of severe scoliosis relate to restrictive lung disease, but most patients do not have a deformity great enough to affect their cardiorespiratory function. The psychological and social effects of scoliosis are significant for patients but difficult to quantitate. For most patients with moderate scoliosis—that is, more than 25 to 30 degrees—treatment with an underarm brace or electrical stimulation is adequate to “control” progression of the curve. Surgical fusion allows actual correction of the curve but is indicated in only a small percentage of patients—usually those with more than 50 degrees of deformity. Images PMID:3279708
Francis, Johnson; Venugopal, K; Khadar, S A; Sudhayakumar, N; Gupta, Anoop K
Idiopathic fascicular ventricular tachycardia is an important cardiac arrhythmia with specific electrocardiographic features and therapeutic options. It is characterized by relatively narrow QRS complex and right bundle branch block pattern. The QRS axis depends on which fascicle is involved in the re-entry. Left axis deviation is noted with left posterior fascicular tachycardia and right axis deviation with left anterior fascicular tachycardia. A left septal fascicular tachycardia with normal axis has also been described. Fascicular tachycardia is usually seen in individuals without structural heart disease. Response to verapamil is an important feature of fascicular tachycardia. Rare instances of termination with intravenous adenosine have also been noted. A presystolic or diastolic potential preceding the QRS, presumed to originate from the Purkinje fibers can be recorded during sinus rhythm and ventricular tachycardia in many patients with fascicular tachycardia. This potential (P potential) has been used as a guide to catheter ablation. Prompt recognition of fascicular tachycardia especially in the emergency department is very important. It is one of the eminently ablatable ventricular tachycardias. Primary ablation has been reported to have a higher success, lesser procedure time and fluoroscopy time.
Chang, Tung-Ming; Yang, Kuender D; Yong, Su-Boon
Juvenile idiopathic arthritis is the most common rheumatic disease in childhood. It is a chronic inflammatory disease associated with arthritis of unknown etiology that begins before the age of 16 and persists for longer than 6 weeks. In this report, the case of a child who suffered recurrent alternative hip arthritis with bilateral hip arthritis is examined, in which he was finally diagnosed as suffering from Juvenile idiopathic arthritis. A 14-year-old boy of Taiwanese origin presented with a normal birth and developmental history. At the age of 10, right-side hip joint pain was experienced, which later migrated to the left side. On further inspection, synovium hypertrophy, cartilage erosion and hip turbid fluid accumulation were found and aseptic arthritis was presumed to be the primary cause. However, after re-examining both his clinical history and presentation, Juvenile idiopathic arthritis was the final diagnosis. Any child presenting with repeat joint swelling are at risk of Juvenile idiopathic arthritis. This is still to be the case if symptoms recede or heal and no initial diagnosis is made. Therefore, a better understanding of the risk of recurrent arthritis is needed. It cannot be emphasized strongly enough that Juvenile idiopathic arthritis should be suspected at all times when a child suffers from recurrent aseptic arthritis of the hip joint.
Idiopathic oligoasthenoteratozoospermia (iOAT) affects approximately 30% of all infertile men. This mini-review discussed recent data in this field. Age, non-inflammatory functional alterations in post-testicular organs, infective agents (Chlamydia trachomatis, herpes virus and adeno-associated viruses), alterations in gamete genome, mitochondrial alterations, environmental pollutants and "subtle" hormonal alterations are all considered possible causes of iOAT. Increase of reactive oxygen species in tubules and in seminal plasma and of apoptosis are reputed to affect sperm concentration, motility and morphology. iOAT is commonly diagnosed by exclusion, nevertheless spectral traces of the main testicular artery may be used as a diagnostic tool for iOAT. The following can be considered therapies for iOAT: 1) tamoxifen citrate (20 mg/d) + testosterone undecanoate (120 mg/d) (pregnancy rate per couple/month [prcm]: 3.8%); 2) folic acid (66 mg/d) + zinc sulfate (5 mg/d); 3) L-carnitine (2 g/d) alone or in combination with acetyl-L-carnitine (1 g/d) (prcm: 2.3%); and 4) both carnitines = one 30 mg cinnoxicam suppository every 4 days (prcm: 8.5%). Alpha-blocking drugs improved sperm concentration but not morphology, motility or pregnancy rate. Tranilast (300 mg/d) increased sperm parameters and pregnancy rates in an initial uncontrolled study. Its efficacy on sperm concentration (but not on sperm motility, morphology or prcm) was confirmed in subsequent published reports. The efficacy of tamoxifen + testosterone undecanoate, tamoxifen alone, and recombinant follicle-stimulating hormone is still a matter for discussion.
Flaherty, Kevin R.; Andrei, Adin-Cristian; King, Talmadge E.; Raghu, Ganesh; Colby, Thomas V.; Wells, Athol; Bassily, Nadir; Brown, Kevin; du Bois, Roland; Flint, Andrew; Gay, Steven E.; Gross, Barry H.; Kazerooni, Ella A.; Knapp, Robert; Louvar, Edmund; Lynch, David; Nicholson, Andrew G.; Quick, John; Thannickal, Victor J.; Travis, William D.; Vyskocil, James; Wadenstorer, Frazer A.; Wilt, Jeffrey; Toews, Galen B.; Murray, Susan; Martinez, Fernando J.
Rationale: Treatment and prognoses of diffuse parenchymal lung diseases (DPLDs) varies by diagnosis. Obtaining a uniform diagnosis among observers is difficult. Objectives: Evaluate diagnostic agreement between academic and community-based physicians for patients with DPLDs, and determine if an interactive approach between clinicians, radiologists, and pathologists improved diagnostic agreement in community and academic centers. Methods: Retrospective review of 39 patients with DPLD. A total of 19 participants reviewed cases at 2 community locations and 1 academic location. Information from the history, physical examination, pulmonary function testing, high-resolution computed tomography, and surgical lung biopsy was collected. Data were presented in the same sequential fashion to three groups of physicians on separate days. Measurements and Main Results: Each observer's diagnosis was coded into one of eight categories. A κ statistic allowing for multiple raters was used to assess agreement in diagnosis. Interactions between clinicians, radiologists, and pathologists improved interobserver agreement at both community and academic sites; however, final agreement was better within academic centers (κ = 0.55–0.71) than within community centers (κ = 0.32–0.44). Clinically significant disagreement was present between academic and community-based physicians (κ = 0.11–0.56). Community physicians were more likely to assign a final diagnosis of idiopathic pulmonary fibrosis compared with academic physicians. Conclusions: Significant disagreement exists in the diagnosis of DPLD between physicians based in communities compared with those in academic centers. Wherever possible, patients should be referred to centers with expertise in diffuse parenchymal lung disorders to help clarify the diagnosis and provide suggestions regarding treatment options. PMID:17255566
Milanlioglu, Aysel; Aydın, Mehmet Nuri; Gökgül, Alper; Hamamcı, Mehmet; Erkuzu, Mehmet Atilla; Tombul, Temel
Opercular syndrome, also known as Foix-Chavany-Marie syndrome, is a paralysis of the facial, pharyngeal, masticatory, tongue, laryngeal, and brachial muscles. It is a rare cortical form of pseudobulbar palsies caused by vascular insults to bilateral operculum. Its clinical presentations include anarthria, weakness of voluntary muscles involving face, tongue, pharynx, larynx, and masticatory muscles. However, autonomic reflexes and emotional activities of these structures are preserved. In the present case, an 81-year-old male presented with acute onset of anarthria with difficulties in chewing, speaking, and swallowing that was diagnosed with opercular syndrome. PMID:23476665
Milanlioglu, Aysel; Aydın, Mehmet Nuri; Gökgül, Alper; Hamamcı, Mehmet; Erkuzu, Mehmet Atilla; Tombul, Temel
Opercular syndrome, also known as Foix-Chavany-Marie syndrome, is a paralysis of the facial, pharyngeal, masticatory, tongue, laryngeal, and brachial muscles. It is a rare cortical form of pseudobulbar palsies caused by vascular insults to bilateral operculum. Its clinical presentations include anarthria, weakness of voluntary muscles involving face, tongue, pharynx, larynx, and masticatory muscles. However, autonomic reflexes and emotional activities of these structures are preserved. In the present case, an 81-year-old male presented with acute onset of anarthria with difficulties in chewing, speaking, and swallowing that was diagnosed with opercular syndrome.
Onychomadesis is a clinical sign of nail plate separation due to transient or permanent arrest of nail matrix activities. Onychomadesis can be considered as a severe form of Beau's line. This condition usually occurs after trauma, causal diseases, or medications, yet it rarely occurs as an idiopathic condition. We report a case of a 38-year-old Thai female who developed recurrence onychomadesis in several toenails in the absence of predisposing factors or associated conditions. To the best of our knowledge, our patient is the first reported case of idiopathic onychomadesis limited to toenails. PMID:27437152
Ho, Karen; Melanson, Michel; Desai, Jamsheed A
Lyme disease caused by the spirochete Borrelia burgdorferi is a multisystem disorder characterized by three clinical stages: dermatologic, neurologic, and rheumatologic. The number of known Lyme disease-endemic areas in Canada is increasing as the range of the vector Ixodes scapularis expands into the eastern and central provinces. Southern Ontario, Nova Scotia, southern Manitoba, New Brunswick, and southern Quebec are now considered Lyme disease-endemic regions in Canada. The use of field surveillance to map risk and endemic regions suggests that these geographic areas are growing, in part due to the effects of climate warming. Peripheral facial nerve palsy is the most common neurologic abnormality in the second stage of Lyme borreliosis, with up to 25% of Bell palsy (idiopathic peripheral facial nerve palsy) occurring due to Lyme disease. Here we present a case of occult bilateral facial nerve palsy due to Lyme disease initially diagnosed as Bell palsy. In Lyme disease-endemic regions of Canada, patients presenting with unilateral or bilateral peripheral facial nerve palsy should be evaluated for Lyme disease with serologic testing to avoid misdiagnosis. Serologic testing should not delay initiation of appropriate treatment for presumed Bell palsy.
Régent, Alexis; Autran, Brigitte; Carcelain, Guislaine; Cheynier, Rémi; Terrier, Benjamin; Charmeteau-De Muylder, Bénédicte; Krivitzky, Alain; Oksenhendler, Eric; Costedoat-Chalumeau, Nathalie; Hubert, Pascale; Lortholary, Olivier; Dupin, Nicolas; Debré, Patrice; Guillevin, Loïc; Mouthon, Luc
Abstract Idiopathic CD4 T lymphocytopenia (ICL) is a rare and severe condition with limited available data. We conducted a French multicenter study to analyze the clinical and immunologic characteristics of a cohort of patients with ICL according to the Centers for Disease Control criteria. We recruited 40 patients (24 female) of mean age 44.2 ± 12.2 (19–70) years. Patients underwent T-lymphocyte phenotyping and lymphoproliferation assay at diagnosis, and experiments related to thymic function and interferon (IFN)-γ release by natural killer (NK) cell were performed. Mean follow-up was 6.9 ± 6.7 (0.14–24.3) years. Infectious, autoimmune, and neoplastic events were recorded, as were outcomes of interleukin 2 therapy. In all, 25 patients had opportunistic infections (12 with human papillomavirus infection), 14 had autoimmune symptoms, 5 had malignancies, and 8 had mild or no symptoms. At the time of diagnosis, the mean cell counts were as follows: mean CD4 cell count: 127/mm3 (range, 4–294); mean CD8: 236/mm3 (range, 1–1293); mean CD19: 113/mm3 (range, 3–547); and mean NK cell count: 122/mm3 (range, 5–416). Most patients had deficiency in CD8, CD19, and/or NK cells. Cytotoxic function of NK cells was normal, and patients with infections had a significantly lower NK cell count than those without (p = 0.01). Patients with autoimmune manifestations had increased CD8 T-cell count. Proliferation of thymic precursors, as assessed by T-cell rearrangement excision circles, was increased. Six patients died (15%). CD4 T-cell count <150/mm3 and NK cell count <100/mm3 were predictors of death. In conclusion, ICL is a heterogeneous disorder often associated with deficiencies in CD8, CD19, and/or NK cells. Long-term prognosis may be related to initial CD4 and NK cell deficiency. PMID:24646462
Amato, Lauretta; Chiarini, Caterina; Berti, Samantha; Massi, Daniela; Fabbri, Paolo
clinical, serologic, histopathologic, and immunopathologic findings, a diagnosis of idiopathic atrophie blanche was made. The patient was treated with dapsone (50 mg p.o. q.d.) and pentoxifylline (400 mg p.o. t.i.d.) with pain relief and complete resolution of the ulcerations after 6 weeks of therapy.
Defining the precise nosological limits of narcolepsy and idiopathic hypersomnia is an ongoing process dating back to the first description of the two conditions. The most recent step forward has been done within the preparation of the second edition of the "International classification of sleep disorders" published in June 2005. Appointed by Dr Emmanuel Mignot, the Task Force on "Hypersomnias of central origin, not due to a circadian rhythm sleep disorder, sleep related breathing disorder, or other causes of disturbed nocturnal sleep" thoroughly revisited the nosology of narcolepsy and of idiopathic hypersomnia. Narcolepsy is now distinguished into three different entities, narcolepsy with cataplexy, narcolepsy without cataplexy and narcolepsy due to medical condition, and idiopathic hypersomnia into two entities, idiopathic hypersomnia with long sleep time and idiopathic hypersomnia without long sleep time. Nevertheless there are still a number of pending issues. What are the limits of narcolepsy without cataplexy? Is there a continuum in the pathophysiology of narcolepsy with and without cataplexy? Should sporadic and familial forms of narcolepsy with cataplexy appear as subgroups in the classification? Are idiopathic hypersomnia with long sleep time and idiopathic hypersomnia without long sleep time, two forms of the same condition or two different conditions? Is there a pathophysiological relationship between narcolepsy without cataplexy and idiopathic hypersomnia without long sleep time?
Kronenberg, Jona; Migirov, Lela; Taitelbaum-Swead, Rikey; Hildesheimer, Minka
Cochlear implant surgery became the standard of care in hearing rehabilitation of patients with severe to profound sensorineural hearing loss. This procedure may alter the lives of children and adults enabling them to integrate with the hearing population. In the past, implantation was performed only in one ear, despite the fact that binaural hearing is superior to unilateral, especially in noisy conditions. Cochlear implantation may be performed sequentially or simultaneously. The "sensitive period" of time between hearing loss and implantation and between the two implantations, when performed sequentially, significantly influences the results. Shorter time spans between implantations improve the hearing results after implantation. Hearing success after implantation is highly dependent on the rehabilitation process which includes mapping, implant adjustments and hearing training. Bilateral cochlear implantation in children is recommended as the proposed procedure in spite of the additional financial burden.
Sako, Wataru; Murakami, Nagahisa; Izumi, Yuishin; Kaji, Ryuji
The Val66Met (G196A; rs6265) single nucleotide polymorphism of brain-derived neurotrophic factor (BDNF) affects morphology and neuronal activity, and is expected to be associated with central nervous system disorders. However, it remains controversial whether Val66Met polymorphism is a risk factor for idiopathic dystonia. We aimed to clarify the impact of BDNF polymorphism on idiopathic dystonia. A literature search of PubMed was carried out. A random-effects model was employed for the meta-analysis. A pooled odds ratio (OR) was calculated along with 95% confidence intervals (CI) to reflect the risk of idiopathic dystonia in each genotype (GG, AG, AA) or minor allele. The proportion of variation due to heterogeneity was computed and expressed as I(2). Five case-control studies, comprising a total sample size of 1804 subjects (784 idiopathic dystonia patients, 1020 normal controls), were included in this meta-analysis. AA genotype was significantly more frequent in patients with idiopathic dystonia (OR=1.47, 95% CI 1.09-1.99, p=0.01, four studies, n=1716). This finding was derived from homogeneous studies (p=0.97, I(2)=0%). Our meta-analysis has revealed a significant overall effect of the AA genotype on the development of idiopathic dystonia.
Satoh, Fumiko; Fujita, Masaki Q; Seto, Yoshihisa; Tsuboi, Akio; Takeichi, Sanae
We report an autopsy case of sudden death in a 36-year-old craftsman with idiopathic scoliosis. The doctor identified his scoliosis at the age of thirteen, and he was under medical care for three years until he stopped consulting the doctor. He collapsed while walking at the station and was sent to an emergency room in cardiopulmonary arrest state, where he was declared dead in spite of more than an hour of CPR. Numbers of petechiae were seen on the bilateral palpebral conjunctivae and the lips were cyanotic. There were no particular injuries except for small abrasions observed on the face. The back showed right rib hump owing to midthoracic scoliosis (with 73 degrees of Cobb's angle) and right hemithorax was deformed showing an appearance of pectus excavatum in the front. The volume of the right thoracic cavity was significantly decreased. In the right lung, there was extensive stromal fibrosis, leaving almost no normal alveolar structures, and medial hypertrophy of pulmonary arteriolar walls. Hypertrophy of the right heart ventricle due to these pulmonary changes and the congestion of other organs suggested that the cause of death in this case was cor pulmonale due to pulmonary hypertension. This was a rare case of fatal outcome of advanced idiopathic scoliosis without medical care in spite of early detection through mass screening.
Debray, M-P; Borie, R; Danel, C; Khalil, A; Majlath, M; Crestani, B
Idiopathic interstitial pneumonias comprise 8 clinicopathological entities, most of them with a chronic course and various prognosis. Idiopathic pulmonary fibrosis is the most frequent and most severe of these. Computed tomography has an important role for its diagnosis. It can identify the corresponding pathological pattern of usual interstitial pneumonia in about 50 percent of cases. It can suggest differential diagnosis in other cases, most frequently fibrosing nonspecific interstitial pneumonia and chronic hypersensitivity pneumonitis. Imaging features should be integrated to clinical and available pathologic data during multidisciplinary team meetings involving physicians with a good knowledge of interstitial diseases. Some cases may be unclassifiable, but these could later be reclassified as new data may occur or imaging features may change. Surgical lung biopsy is being less frequently performed and an emerging less invasive technique, lung cryobiopsy, is under evaluation. Pleuroparenchymal fibroelastosis is a distinct entity only recently described, with uncertain prevalence and prognosis that seems being quite often associated to another pattern of interstitial pneumonia.
Noonan, Kenneth J; Richards, B Stephens
Because nonsurgical management was thought not to yield adequate correction and a durable result, most children with idiopathic clubfoot have undergone surgery with extensive posteromedial and lateral release. However, surgical management caused residual deformity, stiffness, and pain in some children; thus, the favorable long-term results with the Ponseti and French methods of nonsurgical management have garnered interest. The Ponseti method consists of manipulation and casting of idiopathic clubfeet; the French method consists of physiotherapy, taping, and continuous passive motion. Careful evaluation of the techniques and results of these two approaches may increase their use and decrease or minimize the use of surgical management and thus the associated morbidity resulting from extensile releases.
Lööf, Elin; Andriesse, Hanneke; André, Marie; Böhm, Stephanie; Broström, Eva W
Background and purpose - Idiopathic clubfoot can be bilateral or unilateral; however, most studies of gait have assessed clubfoot cases as one uniform group. The contralateral foot in children with unilateral clubfoot has shown deviations in pedobarographic measurements, but it is seldom included in studies of gait. We evaluated gait in children with idiopathic clubfoot, concentrating on foot involvement. Patients and methods - Three-dimensional gait analyses of 59 children, mean age 5.4 years, with bilateral (n = 30) or unilateral (n = 29) idiopathic clubfoot were stratified into groups of bilateral, unilateral, or contralateral feet. Age-matched controls (n = 28) were evaluated for comparison. Gait assessment included: (1) discrete kinematic and kinetic parameters, and (2) gait deviation index for kinematics (GDI) and kinetics (GDI-k). Results - No differences in gait were found between bilateral and unilateral idiopathic clubfoot, but both groups deviated when compared to controls. Compared to control feet, contralateral feet showed no deviations in discrete gait parameters, but discrepancies were evident in relation to unilateral clubfoot, causing gait asymmetries in children with unilateral involvement. However, all groups deviated significantly from control feet according to GDI and GDI-k. Interpretation - Bilateral and unilateral idiopathic clubfoot cases show the same persistent deviations in gait, mainly regarding reduced plantarflexion. Nevertheless, knowledge of foot involvement is important as children with unilateral clubfoot show gait asymmetries, which might give an impression of poorer deviations. The results of GDI/GDI-k indicate global gait adaptations of the contralateral foot, so the foot should preferably not be used as a reference for gait.
Huertas, Andres; Watson, Robert; Villalpando, Carlos; Goldberg, Steven
its position in the window as well as the pixel value for the central pixel of the window. The absolute difference between these two pixel values is calculated and used as an address in a lookup table. Each processing element has a lookup table, unique for its position in the window, containing the weight coefficients for the Gaussian function for that position. The pixel value is multiplied by the weight, and the outputs of the processing element are the weight and pixel-value weight product. The products and weights are fed to the adder tree. The sum of the products and the sum of the weights are fed to the divider, which computes the sum of products the sum of weights. The output of the divider is denoted the bilateral smoothed image. The smoothing function is a simple weighted average computed over a 3 3 subwindow centered in the 9 9 window. After smoothing, the image is delayed by an additional amount of time needed to match the processing time for computing the bilateral smoothed image. The bilateral smoothed image is then subtracted from the 3 3 smoothed image to produce the final output. The prototype filter as implemented in a commercially available FPGA processes one pixel per clock cycle. Operation at a clock speed of 66 MHz has been demonstrated, and results of a static timing analysis have been interpreted as suggesting that the clock speed could be increased to as much as 100 MHz.
Austin, Howard A.
Exciting progress recently has been made in our understanding of idiopathic membranous nephropathy, as well as treatment of this disease. Here, we review important advances regarding the pathogenesis of membranous nephropathy. We will also review the current approach to treatment and its limitations and will highlight new therapies that are currently being explored for this disease including Rituximab, mycophenolate mofetil, and adrenocorticotropic hormone, with an emphasis on results of the most recent clinical trials. PMID:22859855
Srivastava, G N; Prasad, Rajniti; Meena, Manoj; Hussain, Moosa
We present a case of acute silicosis with bilateral pneumothorax of a 28-year-old man working at a stone crusher factory for 1 year. He presented to the emergency department with cough, respiratory distress and diffuse chest pain. The patient was managed with bilateral intercostal tube drainage under water seal, oxygen inhalation and conservative therapy. On follow-up he showed improvement of resting dyspnoea and was doing well. This case is being reported because of the rare complications of acute silicosis as bilateral pneumothorax.
Udoji, Timothy N; Force, Seth D; Pelaez, Andres
Abstract A 33-year-old female patient with advanced idiopathic pulmonary artery hypertension underwent bilateral lung transplantation. The postsurgical course was complicated by prolonged mechanical ventilation and acute hypoxemia with recurrent episodes of pulmonary edema. An echocardiogram revealed improved right-sided pressures along with a dilated left atrium, a structurally normal mitral valve, and a new posterior-oriented severe mitral regurgitation. The patient's condition improved after treatment with arterial vasodilators and diuretics, and she has remained in World Health Organization functional class I after almost 36 months of follow-up. We hypothesize that cardiac ventricle remodeling and a geometric change in mitral valve apparatus after transplantation led to the hemodynamic changes and recurrent pulmonary edema seen in our patient. Our case is, to our knowledge, the second report of severe valvular regurgitation in a structurally normal mitral valve apparatus in the postoperative period and the first of a patient to be treated without valve replacement.
Kau, Chung How; Bejemir, Morvarid Poorsattar
This case report describes the successful treatment of an adult patient with idiopathic condylar resorption and Class II skeletal open bite malocclusion and temporomandibular joint disorder. A segmental Le Fort I bilateral osteotomy, ramus increasing length inverted L–osteotomy, and genioplasty combined with orthodontic treatment were performed. The treatment plan and surgery was aided by three-dimensional medical modeling, and we managed to resolve functional, esthetic, and pain concerns to a satisfactory level. PMID:26981482
Leu-Semenescu, S; Quera-Salva, M-A; Dauvilliers, Y
Idiopathic hypersomnia is a rare, central hypersomnia, recently identified and to date of unknown physiopathology. It is characterised by a more or less permanent, excessive daytime sleepiness, associated with long and unrefreshing naps. Night-time sleep is of good quality, excessive in quantity, associated with sleep inertia in the subtype previously described as "with long sleep time". Diagnosis of idiopathic hypersomnia is complex due to the absence of a quantifiable biomarker, the heterogeneous symptoms, which overlap with the clinical picture of type 2 narcolepsy, and its variable evolution over time. Detailed evaluation enables other frequent causes of somnolence, such as depression or sleep deprivation, to be eliminated. Polysomnography and multiple sleep latency tests (MSLT) are essential to rule out other sleep pathologies and to objectify excessive daytime sleepiness. Sometimes the MSLT do not show excessive sleepiness, hence a continued sleep recording of at least 24hours is necessary to show prolonged sleep (>11h/24h). In this article, we propose recommendations for the work-up to be carried out during diagnosis and follow-up for patients suffering from idiopathic hypersomnia.
Yilmaz, Murat; Kavak, Ayse; Yamaner, Nalan Jale
Tinea corporis has rarely been reported in some locations such as on the breast skin as unilaterally. Herein, we present a case of bilateral tinea mammae, which has not been reported before in English language literature to our knowledge.
Özdemir, Güzelali; Andıç, Kemal; Erdem Yaşar, Niyazi
In this case, we present a patient with the diagnosis of bilateral olecranon tophaceous gout. After the surgical treatment, there was no limitation of range of motion or wound problem at 6th month control. PMID:28326103
Brantley, Elise I; Mutasim, Diya F; Heaton, Charles
We report a case of acquired idiopathic generalized anhidrosis (AIGA) in a 56-year-old white woman. Acquired idiopathic generalized anhidrosis is an exceedingly rare group of heterogeneous disorders that has been almost exclusively reported in young Japanese males. Our case is unique in that AlGA may be underrecognized in this patient population.
Pal, Deb K; Ferrie, Colin; Addis, Laura; Akiyama, Tomoyuki; Capovilla, Giuseppe; Caraballo, Roberto; de Saint-Martin, Anne; Fejerman, Natalio; Guerrini, Renzo; Hamandi, Khalid; Helbig, Ingo; Ioannides, Andreas A; Kobayashi, Katsuhiro; Lal, Dennis; Lesca, Gaetan; Muhle, Hiltrud; Neubauer, Bernd A; Pisano, Tiziana; Rudolf, Gabrielle; Seegmuller, Caroline; Shibata, Takashi; Smith, Anna; Striano, Pasquale; Strug, Lisa J; Szepetowski, Pierre; Valeta, Thalia; Yoshinaga, Harumi; Koutroumanidis, Michalis
The term idiopathic focal epilepsies of childhood (IFE) is not formally recognised by the ILAE in its 2010 revision (Berg et al., 2010), nor are its members and boundaries precisely delineated. The IFEs are amongst the most commonly encountered epilepsy syndromes affecting children. They are fascinating disorders that hold many "treats" for both clinicians and researchers. For example, the IFEs pose many of the most interesting questions central to epileptology: how are functional brain networks involved in the manifestation of epilepsy? What are the shared mechanisms of comorbidity between epilepsy and neurodevelopmental disorders? How do focal EEG discharges impact cognitive functioning? What explains the age-related expression of these syndromes? Why are EEG discharges and seizures so tightly locked to slow-wave sleep? In the last few decades, the clinical symptomatology and the respective courses of many IFEs have been described, although they are still not widely appreciated beyond the specialist community. Most neurologists would recognise the core syndromes of IFE to comprise: benign epilepsy of childhood with centro-temporal spikes or Rolandic epilepsy (BECTS/RE); Panayiotopoulos syndrome; and the idiopathic occipital epilepsies (Gastaut and photosensitive types). The Landau-Kleffner syndrome and the related (idiopathic) epilepsy with continuous spikes and waves in sleep (CSWS or ESES) are also often included, both as a consequence of the shared morphology of the interictal discharges and their potential evolution from core syndromes, for example, CSWS from BECTS. Atypical benign focal epilepsy of childhood also has shared electro-clinical features warranting inclusion. In addition, a number of less well-defined syndromes of IFE have been proposed, including benign childhood seizures with affective symptoms, benign childhood epilepsy with parietal spikes, benign childhood seizures with frontal or midline spikes, and benign focal seizures of adolescence. The
Mayes, Jonathan; Niranjan, Gunaratnam; Dark, John; Clark, Stephen
This case describes the technique of using dual Novalungs (a pumpless extracorporeal system) to bridge a patient with idiopathic pulmonary hypertension to bilateral lung transplantation. A 41-year old lady with idiopathic pulmonary hypertension (with a possible veno-occlusive element) presented with symptoms of end-stage heart and lung failure. This was refractory to medical management with iloprost, sildenafil and bosentan. The patient was placed on the urgent waiting list for lung transplantation and central pulmonary artery to left atrial Novalung insertion was performed. Local anaesthetic was given before performing peripheral cardiopulmonary bypass due to the high risk of cardiac arrest. Two days later, donor organs became available and the patient was taken for double-lung transplantation. The pulmonary artery cannula was removed leaving a large defect. This was then closed using a bovine pericardial patch. Due to the damaged right superior pulmonary vein from Novalung cannulation, cardioplegia was given to facilitate an open atrial anastomosis. After 13 days in the intensive therapy unit, she was transferred to the ward. There were no further complications and she has been discharged home.
Ching, Teresa Y C; Massie, Robyn; Van Wanrooy, Emma; Rushbrooke, Emma; Psarros, Colleen
This paper summarises findings from studies that evaluated the benefits of bimodal fitting (combining a hearing aid and a cochlear implant in opposite ears) or bilateral cochlear implantation, relative to unilateral implantation, for children (Ching et al., 2007). On average, the size of binaural speech intelligibility advantages due to redundancy and head shadow was similar for the two bilateral conditions. An added advantage of bimodal fitting was that the low-frequency cues provided by acoustic hearing complemented the high-frequency cues conveyed by electric hearing in perception of voice and music. Some children with bilateral cochlear implants were able to use spatial separation between speech and noise to improve speech perception in noise. This is possibly a combined effect of the directional microphones in their implant systems and their ability to use spatial cues. The evidence to date supports the provision of hearing in two ears as the standard of care.
Shiode, Yusuke; Morizane, Yuki; Toshima, Shinji; Kimura, Shuhei; Kumase, Fumiaki; Hosokawa, Mio; Hirano, Masayuki; Doi, Shinichiro; Takahashi, Kosuke; Hosogi, Mika; Fujiwara, Atsushi; Shiraga, Fumio
Objective To investigate the occurrence ratio, localization, and surgical outcomes of intraretinal cystic spaces in idiopathic epiretinal membranes (ERMs). Methods We retrospectively reviewed the charts of 432 eyes of 398 consecutive patients with idiopathic ERM who underwent vitrectomy and ERM peeling from January 2012 to September 2015. We selected cases with intraretinal cystic space prior to surgery, detected by spectral-domain optical coherence tomography. We then evaluated the effects of ERM peeling on intraretinal cystic spaces, best corrected visual acuity, and central retinal thickness at 6 months after surgery. Results Twenty-four eyes (5.5%) showed intraretinal cystic spaces before surgery, present in the inner retinal layer (the inner group) in 9 eyes, in the outer retinal layer (the outer group) in 6 eyes, and in both the inner and the outer retinal layers (the combined group) in 9 eyes. Additionally, 30 eyes with ERM but without any presence of intraretinal cystic space were selected randomly and classified as the no cyst group. At 6 months after surgery, the disappearance rate of cystic spaces was significantly greater for the outer group than for the inner group (83.3% and 11.1%, respectively, P = 0.011). The mean best corrected visual acuity improved significantly after surgery in the inner group, the outer group, and the no cyst group (P < 0.05 for all three groups) but did not improve in the combined group (P = 0.58). The mean central retinal thickness decreased significantly after surgery in the inner group, the combined group, and the no cyst group (P < 0.05). Conclusions Intraretinal cystic spaces were observed in 5.5% of preoperative idiopathic ERM cases. Following surgery, the cystic spaces in the outer retinal layer disappeared at higher rates than those in the inner retinal layer, suggesting that the pathophysiologies of these cystic spaces are different. PMID:27992520
Moura, Diogo Lino; Marques, José Pedro; Lucas, Francisco Manuel; Fonseca, Fernando Pereira
Bilateral patellar tendon rupture is a rare entity, often associated with systemic diseases and patellar tendinopathy. The authors report a rare case of a 34-year-old man with simultaneous bilateral rupture of the patellar tendon caused by minor trauma. The patient is a retired basketball player with no past complaints of chronic knee pain and a history of steroid use. Surgical management consisted in primary end-to-end tendon repair protected temporarily with cerclage wiring, followed by a short immobilization period and intensive rehabilitation program. Five months after surgery, the patient was able to fully participate in sport activities.
Yalcinkaya, C; Sarioglu, A; Boltshauser, E
We report a personal series of 28 patients with neurofibromatosis 2 (NF-2), emphasizing the differences from classical NF-1. The hallmark of NF-2 is bilateral acoustic neuromas with initial symptoms usually occurring in the second or third decade. The natural history may lead to bilateral deafness, but hearing loss may also be a complication of surgery. NF-2 is frequently accompanied by additional intracranial tumors (particularly multiple meningiomas). Half of our patients had a spinal space-occupying lesion. NF-2 is inherited as an autosomal dominant trait, and many patients appear to represent new mutations.
Page, T L; Caldarola, P C; Pittendrigh, C S
The interactions between the bilaterally distributed components of the circadin system that controls the locomotor activity rhythm of the cockroach Leucophaea maderae were investigated in a series of surgical lesion experiments. Complete excision of one optic lobe (either right or left) or its surgical isolation from the central nervous system had no effect on the animals' ability to free-run in constant darkness nor was there any indication, as judged by postoperative pi values of any difference between left and right lobe pacemakers. However, these surgical procedures consistently resulted in a significant increase in tau over preoperative value while optic nerve section had no effect on tau. The propostion is developed that the left and right pacemakers in the two optic lobes are mutally coupled and that the compound pacemaker's period is shorter than either of its constituent pacemakers. It was also found that the integrity of either compound eye is sufficient to assure entrainment of both left and right pacemakers.
Ley, Brett; Collard, Harold R
Idiopathic pulmonary fibrosis is a chronic fibrotic lung disease of unknown cause that occurs in adults and has a poor prognosis. Its epidemiology has been difficult to study because of its rarity and evolution in diagnostic and coding practices. Though uncommon, it is likely underappreciated both in terms of its occurrence (ie, incidence, prevalence) and public health impact (ie, health care costs and resource utilization). Incidence and mortality appear to be on the rise, and prevalence is expected to increase with the aging population. Potential risk factors include occupational and environmental exposures, tobacco smoking, gastroesophageal reflux, and genetic factors. An accurate understanding of its epidemiology is important, especially as novel therapies are emerging. PMID:24348069
Yang, Ivana V
Idiopathic pulmonary fibrosis (IPF) is a complex lung disease of unknown etiology. Development of IPF is influenced by both genetic and environmental factors. Gene-expression profiling studies have taught us quite a bit about the biology of this fatal disease, but epigenetic marks may be the missing link that connects the environmental exposure in genetically predisposed individuals to transcriptome changes associated with the development of IPF. This review will begin with an introduction to the disease, followed by brief summaries of studies of gene expression in IPF and epigenetic marks associated with exposures relevant to IPF. The majority of the discussion will focus on epigenetic studies conducted so far in IPF, the limitations, challenges nd future directions in this field.
Oh, Chad K.; Murray, Lynne A.; Molfino, Nestor A.
Idiopathic pulmonary fibrosis (IPF) is a disease of unknown etiology with considerable morbidity and mortality. Cigarette smoking is one of the most recognized risk factors for development of IPF. Furthermore, recent work suggests that smoking may have a detrimental effect on survival of patients with IPF. The mechanism by which smoking may contribute to the pathogenesis of IPF is largely unknown. However, accumulating evidence suggests that increased oxidative stress might promote disease progression in IPF patients who are current and former smokers. In this review, potential mechanisms by which cigarette smoking affects IPF, the effects of cigarette smoking on accelerated loss of lung function in patients with IPF, key genetic studies evaluating the potential candidate genes and gene-environment (smoking) interaction, diagnosis, and treatment with emphasis on recently closed and ongoing clinical trials are presented. PMID:22448328
Hashisako, Mikiko; Fukuoka, Junya
The updated classification of idiopathic interstitial pneumonias (IIPs) in 2013 by American Thoracic Society/European Respiratory Society included several important revisions to the categories described in the 2002 classification. In the updated classification, lymphoid interstitial pneumonia (LIP) was moved from major to rare IIPs, pleuroparenchymal fibroelastosis (PPFE) was newly included in the rare IIPs, acute fibrinous and organizing pneumonia (AFOP) and interstitial pneumonias with a bronchiolocentric distribution are recognized as rare histologic patterns, and unclassifiable IIP (UCIP) was classified as an IIP. However, recent reports indicate the areas of concern that may require further evaluation. Here, we describe the histopathologic features of the updated IIPs and their rare histologic patterns and also point out some of the issues to be considered in this context. PMID:26949346
Arimura, T; Hayashi, T; Kimura, A
Summary Idiopathic cardiomyopathy (ICM) is a primary cardiac disorder associated with abnormalities of ventricular wall thickness, size of ventricular cavity, contraction, relaxation, conduction and rhythm. Over the past two decades, molecular genetic analyses have revealed that mutations in the various genes cause ICM and such information concerning the genetic basis of ICM enables us to speculate the pathogenesis of this heterogeous cardiac disease. This review focuses on the molecular pathogenesis, i.e., genetic abnormalities and functional alterations due to the mutations especially in sarcomere/cytoskeletal components, in three characteristic features of ICM, hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM) and restrictive cardiomyopathy (RCM). Understanding the functional abnormalities of the sarcomere/cytoskeletal components, in ICM, has unraveled the function of these components not only as a contractile unit but also as a pivot for transduction of biochemical signals. PMID:18646564
Lisanti, Francesco; Scarano, Enrico
Renal infarction is a rare cause of referral to the emergency department, with very low estimated incidence (0.004%–0.007%). Usually, it manifests in patients aged 60–70 with risk factors for thromboembolism, mostly related to heart disease, atrial fibrillation in particular. We report a case of idiopathic segmental renal infarction in a 38-year-old patient, presenting with acute abdominal pain with no previous known history or risk factors for thromboembolic diseases. Because of its aspecific clinical presentation, this condition can mimic more frequent pathologies including pyelonephritis, nephrolithiasis, or as in our case appendicitis. Here we highlight the extremely ambiguous presentation of renal infarct and the importance for clinicians to be aware of this condition, particularly in patients without clear risk factors, as it usually has a good prognosis after appropriate anticoagulant therapy. PMID:28203466
Spamer, M; Georgi, M; Häfner, R; Händel, H; König, M; Haas, J-P
Control of disease activity and recovery of function are major issues in the treatment of children and adolescents suffering from juvenile idiopathic arthritis (JIA). Functional therapies including physiotherapy are important components in the multidisciplinary teamwork and each phase of the disease requires different strategies. While in the active phase of the disease pain alleviation is the main focus, the inactive phase requires strategies for improving motility and function. During remission the aim is to regain general fitness by sports activities. These phase adapted strategies must be individually designed and usually require a combination of different measures including physiotherapy, occupational therapy, massage as well as other physical procedures and sport therapy. There are only few controlled studies investigating the effectiveness of physical therapies in JIA and many strategies are derived from long-standing experience. New results from physiology and sport sciences have contributed to the development in recent years. This report summarizes the basics and main strategies of physical therapy in JIA.
Kim, Ju Young; Lee, Moon Souk; Kim, Seung Yeon; Kim, Hyun Jung; Lee, Soo Jin; You, Chur Woo; Kim, Jon Soo
Harlequin syndrome, which is a rare disorder caused by dysfunction of the autonomic system, manifests as asymmetric facial flushing and sweating in response to heat, exercise, or emotional factors. The syndrome may be primary (idiopathic) with a benign course, or can occur secondary to structural abnormalities or iatrogenic factors. The precise mechanism underlying idiopathic harlequin syndrome remains unclear. Here, we describe a case of a 6-year-old boy who reported left hemifacial flushing and sweating after exercise. He had an unremarkable birth history and no significant medical history. Complete ophthalmological and neurological examinations were performed, and no other abnormalities were identified. Magnetic resonance imaging was performed to exclude lesions of the cerebrum and cervicothoracic spinal cord, and no abnormalities were noted. His final diagnosis was classic idiopathic harlequin syndrome. Herein, we report the first pediatric case of idiopathic harlequin syndrome in Korea. PMID:28018464
Murphy, W. G.; Allan, N. C.; Perry, D. J.; Stockdill, G.
A case of Hodgkin's disease presenting as idiopathic thrombocytopenic purpura in a 23-year-old male is reported. This is a rare presentation of Hodgkin's disease having been previously described in only two cases. PMID:6541338
Faussone-Pellegrini, Maria Simonetta; Grover, Madhusudan; Pasricha, Pankaj J; Bernard, Cheryl E; Lurken, Matthew S; Smyrk, Thomas C; Parkman, Henry P; Abell, Thomas L; Snape, William J; Hasler, William L; Ünalp-Arida, Aynur; Nguyen, Linda; Koch, Kenneth L; Calles, Jorges; Lee, Linda; Tonascia, James; Hamilton, Frank A; Farrugia, Gianrico
Abstract The ultrastructural changes in diabetic and idiopathic gastroparesis are not well studied and it is not known whether there are different defects in the two disorders. As part of the Gastroparesis Clinical Research Consortium, full thickness gastric body biopsies from 20 diabetic and 20 idiopathic gastroparetics were studied by light microscopy. Abnormalities were found in many (83%) but not all patients. Among the common defects were loss of interstitial cells of Cajal (ICC) and neural abnormalities. No distinguishing features were seen between diabetic and idiopathic gastroparesis. Our aim was to provide a detailed description of the ultrastructural abnormalities, compare findings between diabetic and idiopathic gastroparesis and determine if patients with apparently normal immunohistological features have ultrastructural abnormalities. Tissues from 40 gastroparetic patients and 24 age- and sex-matched controls were examined by transmission electron microscopy (TEM). Interstitial cells of Cajal showing changes suggestive of injury, large and empty nerve endings, presence of lipofuscin and lamellar bodies in the smooth muscle cells were found in all patients. However, the ultrastructural changes in ICC and nerves differed between diabetic and idiopathic gastroparesis and were more severe in idiopathic gastroparesis. A thickened basal lamina around smooth muscle cells and nerves was characteristic of diabetic gastroparesis whereas idiopathic gastroparetics had fibrosis, especially around the nerves. In conclusion, in all the patients TEM showed abnormalities in ICC, nerves and smooth muscle consistent with the delay in gastric emptying. The significant differences found between diabetic and idiopathic gastroparesis offers insight into pathophysiology as well as into potential targeted therapies. PMID:21914127
Alsaif, Fahad; Abduljabbar, Amr M.
Calcinosis cutis is a rare disorder characterized by the deposition of calcium in the skin and subcutaneous tissue. Unilateral idiopathic calcinosis cutis has only rarely been reported in the literature. Here, we report the case of a 7-year-old healthy girl who presented with multiple asymptomatic hard nodules on the right side of her body. Histopathological, radiological, and extensive blood investigations confirmed the diagnosis of unilateral idiopathic calcinosis cutis. PMID:28203159
Waller, Sandy McCombe; Whitall, Jill
Bilateral arm training has emerged as an approach that leads to positive outcomes in addressing upper extremity paresis after stroke. However, studies have not demonstrated improvements in all patients using current outcome measures. Furthermore, the rationale for using this type of training has been incompletely explained. The purpose of this article was to first review the theoretical justifications for the use of bilateral arm training by examining motor control and neural mechanisms underlying arm function and neural recovery, and second, to discuss examples of clinical studies using a variety of bilateral training strategies to identify who may benefit most from this approach. We argue that bilateral arm training is a necessary adjunct to unilateral training because bilateral re-training is important and best served through bilateral not unilateral training, and also, that bilateral training may help unilateral skill recovery through alternative putative mechanisms. Our review of the empirical evidence suggests that individuals at all levels of severity can benefit in some manner from bilateral training, but that not all approaches are effective for all severity levels. In addition to requesting more randomized controlled trials and studies of neurophysiological mechanisms we conclude the following: 1) Bilateral training can improve unilateral paretic limb functions of the upper extremity after stroke, however, specific training approaches need to be matched to baseline characteristics of the patients; 2) Given the importance of bilateral activities in daily life, there is a need to recognize, train and assess the important contribution of supportive role functions of the paretic arm used on its own and as part of complementary bilateral functional skills; 3) An assessment of bilateral and unilateral functioning which includes bilateral task analysis, as well as, evaluations of interlimb coordination should be included in all studies that include bilateral
McCombe Waller, Sandy; Whitall, Jill
Bilateral arm training has emerged as an approach that leads to positive outcomes in addressing upper extremity paresis after stroke. However, studies have not demonstrated improvements in all patients using current outcome measures. Furthermore, the rationale for using this type of training has been incompletely explained. The purpose of this article was to first review the theoretical justifications for the use of bilateral arm training by examining motor control and neural mechanisms underlying arm function and neural recovery, and second, to discuss examples of clinical studies using a variety of bilateral training strategies to identify who may benefit most from this approach. We argue that bilateral arm training is a necessary adjunct to unilateral training because bilateral re-training is important and best served through bilateral not unilateral training, and also, that bilateral training may help unilateral skill recovery through alternative putative mechanisms. Our review of the empirical evidence suggests that individuals at all levels of severity can benefit in some manner from bilateral training, but that not all approaches are effective for all severity levels. In addition to requesting more randomized controlled trials and studies of neurophysiological mechanisms we conclude the following: 1) Bilateral training can improve unilateral paretic limb functions of the upper extremity after stroke, however, specific training approaches need to be matched to baseline characteristics of the patients; 2) Given the importance of bilateral activities in daily life, there is a need to recognize, train and assess the important contribution of supportive role functions of the paretic arm used on its own and as part of complementary bilateral functional skills; 3) An assessment of bilateral and unilateral functioning which includes bilateral task analysis, as well as, evaluations of interlimb coordination should be included in all studies that include bilateral
Liao, B S; Byl, F M; Adour, K K
A recently published prospective study on acute sensorineural deafness in Lassa fever among a West African population showed the audiometric pattern of a known virally induced hearing loss. Using the audiometric data from the patients with Lassa fever in that study, we analyzed and classified the initial hearing loss and final recovery into three groups by pure-tone average values and then did the same for 222 patients with idiopathic sudden hearing loss (SHL) in our study. Statistical analyses of the severity of initial hearing loss and the hearing recovery pattern indicate that the clinical course of our 222 patients with idiopathic SHL showed no statistically significant differences from the clinical course of the patients with Lassa fever. We found a marked difference in age, however, and a clinically significant difference in the incidence of bilateral hearing loss. In reviewing the literature on sudden sensorineural hearing loss, we found no apparent relation between severity of viral illness and initial hearing loss or subsequent recovery. Cummins et al. suggest that virally induced hearing loss in Lassa fever is linked to the host's immune response and not to the viremia. We thus propose a virally induced immune response mechanism for idiopathic sensorineural SHL. Further prospective studies are needed for verification.
Toupet, Michel; van Nechel, Christian; Duquesne, Ulla; Hautefort, Charlotte; Lopez, Christophe
Recent findings suggest that vestibular information plays a significant role in anchoring the self to the body. Out-of-body experiences of neurological origin are frequently associated with vestibular sensations, and galvanic vestibular stimulation in healthy participants anchors the self to the body. Here, we provide the first objective measures of anchoring the self to the body in chronic bilateral vestibular failure (BVF). We compared 23 patients with idiopathic BVF to 23 healthy participants in a series of experiments addressing several aspects of visuo-spatial perspective taking and embodiment. In Experiment 1, participants were involved in a virtual “dot-counting task” from their own perspective or the perspective of a distant avatar, to measure implicit and explicit perspective taking, respectively. In both groups, response times increased similarly when the avatar’s and participant’s viewpoint differed, for both implicit and explicit perspective taking. In Experiment 2, participants named ambiguous letters (such as “b” or “q”) traced on their forehead that could be perceived from an internal or external perspective. The frequency of perceiving ambiguous letters from an internal perspective was similar in both groups. In Experiment 3, participants completed a questionnaire measuring the experienced self/body and self/environment “closeness”. Both groups reported a similar embodied experience. Altogether, our data show that idiopathic BVF does not change implicit and explicit perspective taking nor subjective anchoring of the self to the body. Our negative findings offer insight into the multisensory mechanisms of embodiment. Only acute peripheral vestibular disorders and neurological disorders in vestibular brain areas (characterized by strong multisensory conflicts) may evoke disembodied experiences. PMID:28107424
these bilateral relationships, while they are not quite “building blocks” of a possible regional “security architecture ,” are certainly a variable in...nationshave long influenced each other in the fields of culture, art, architecture , and language, especially during the 1526-1757 period, when the Mughals...countries are close to forming a single economic market, and on security issues there is an official commitment to harmonize policies and processes to the
Ozcan, Emel; Ural, Ahmet; Oktemer, Tuğba Koçak; Alpaslan, Gökhan
Although tonsilloliths are reported to be fairly common, florid cases casting distinct radiopaque shadows on panoramic radiographs are not often reported. This report illustrates such a case as an incidental finding in an asymptomatic 38-year-old female dental patient. Panoramic radiography revealed distinct radiopaque shadows over the ascending rami of the mandible bilaterally. These radiopacities were localized to the palatine tonsils by computerized tomographic inspection. Tonsillectomy was performed and tonsillolithiasis was confirmed by histopathologic examination.
Grande, M.; Lisi, G.; Bianchi, D.; Bove, P.; Miano, R.; Esser, A.; De Sanctis, F.; Neri, A.; Grande, S.; Villa, M.
Acute renal failure due to bilateral ureteral obstruction is a rare complication after appendectomy in children. We report a case of bilateral ureteric obstruction in a 14-year-old boy nine days after surgery for an acute appendicitis. After saline-filling of the urinary bladder, transabdominal ultrasound demonstrated bilateral hydronephrosis of moderate degree. No abscess was found with CT but presence of millimetric stones on both distal ureters was shown, with bilateral calyceal dilatation. Cystoscopy revealed inflammatory changes in the bladder base. Following introduction of bilateral ureteric stents, there was rapid normalisation of urinary output and serum creatinine. PMID:26295001
Zerwekh, Joseph E.
Observational and epidemiological studies alike have demonstrated that idiopathic hypercalciuric (IH) stone-forming patients typically demonstrate bone mineral density scores significantly less than those observed for age- and gender-matched normal subjects or those for non-hypercalciuric stone-forming patients. Most of these studies have relied on changes in bone mineral density (BMD) and have not explored the mechanism(s) involved. There have been a small number of studies that have relied on dynamic bone histomorphometry to ascertain the nature of the bone defect in IH patients. When performed, these studies have clearly demonstrated increased bone resorption and high bone turnover in patients with fasting hypercalciuria while suppressed bone formation indices are the most consistent finding in patients with the absorptive variant of IH. The causes of this apparent difference in bone remodeling between the two variants of IH is still uncertain. Available evidence suggests that potential mechanisms may be dependent in large part to genetic, metabolic, and nutritional causes of hypercalciuria and bone loss in patients with IH. PMID:18359394
Yang, Ivana V.; Schwartz, David A.
Idiopathic pulmonary fibrosis (IPF) is a complex lung disease of unknown etiology. Development of IPF is influenced by both genetic and environmental factors. Recent work by our and other groups has identified strong genetic predisposition factors for the development of pulmonary fibrosis while cigarette smoke remains the most strongly associated environmental exposure risk factor. Gene expression profiling studies of IPF lung have taught us quite a bit about the biology of this fatal disease and those in peripheral blood have provided important biomarkers. However, epigenetic marks may be the missing link that connects the environmental exposure in genetically predisposed individuals to transcriptional changes associated with disease development. Moreover, epigenetic marks represent a promising therapeutic target for IPF. In this review, we will introduce the disease, summarize genetic and gene expression studies in IPF, discuss exposures relevant to IPF and known epigenetic changes associated with cigarette smoke exposure, and summarize epigenetic studies conducted so far in IPF. We will end by discussing limitations, challenges and future opportunities in this field. PMID:24746870
Maestri, Michelangelo; Monzani, Fabio; Bonanni, Enrica; Di Coscio, Elisa; Cignoni, Fabio; Dardano, Angela; Iudice, Alfonso; Murri, Luigi
We report the case of a 32-year-old woman with a history of increased sleep need and difficulty waking up; the diagnosis of idiopathic hypersomnia was hypothesized. During ambulatory polysomnography (PSG), the patient presented an episode characterized by loss of consciousness and jerking of the four limbs. A video-PSG monitoring was performed and the patient showed unresponsiveness and drowsiness at 7 a.m. During the episode, EEG showed theta-delta diffuse activity, and blood glucose level was 32 mg dl(-1). The diagnosis of insulinoma was then assumed; CT scan showed a hypodense mass into the pancreatic tail, and a partial pancreasectomy was performed. The described symptoms disappeared, and 5 years later the findings of a complete clinical and neurophysiological examination were negative. The clinical picture of insulinoma presenting with paroxysmal disorders has been previously described; however, whereas hypersomnia is uncommon, in the current case it represents the main symptom. Clinicians should keep in mind that neuroglycopenia should be considered in the differential diagnosis of patients with hypersomnia, particularly if the clinical scenario does not conform to standard criteria.
Dubey, Suparna; Sharma, Rajeev; Maheshwari, Veena
Scrotal calcinosis is a rare benign local process characterized by multiple, painless, hard scrotal nodules in the absence of any systemic metabolic disorder. Histological examination reveals extensive deposition of calcium in the dermis, which may be surrounded by histiocytes and an inflammatory giant cell reaction. Numerous theories have been propounded to explain the pathogenesis of this condition, but the principal debate revolves around whether the calcium is deposited at the site of previous epithelial cysts or the calcified nodules are purely idiopathic. This is the largest study of scrotal calcinosis to date with 100 cases, on which clinical, biochemical, radiological, cytopathological, and histopathological examinations were conducted. The histological picture shows a continuous spectrum of changes ranging from intact epithelial cysts (41.0%) - both normal and inflamed; through inflamed cysts containing calcific material in the lumen but with intact cyst wall (53.0%); calcified inflamed cysts with partial epithelial lining (11.0%); to 'naked' calcium deposits lying in the dermis (100%), sometimes compressing surrounding collagen fibres to form a pseudocyst (56.0%). The presence of normal values of calcium and phosphorus along with this spectrum of changes in histology both support the theory that these form by dystrophic calcification of epithelial cysts in a progression that involves inflammation, rupture, calcification and obliteration of the cyst wall.
Li, Yi; Bao, Fang Jun
The purpose of this study was to evaluate the interocular symmetry of several biometric parameters between both eyes. The symmetry between the right and left eye of 397 subjects in 14 biometric parameters, spherical equivalent of refractive error (SE), Jackson crossed cylinder power of refractive error astigmatism with axes at 90° and 180° (RJ0) and at 45° and 135° (RJ45), best-corrected visual acuity (BCVA), average corneal curvature (CC), Jackson crossed cylinder power of corneal astigmatism (CJ0 and CJ45), corneal asphericity coefficient (Q), intraocular pressure (IOP), central corneal thickness (CCT), axial length (AL), anterior chamber depth (ACD), lens thickness (LT) and vitreous chamber depth (VCD), was assessed by comparative data analysis. Aside from RJ0 (p = 0.00), RJ45 (p = 0.02) and Q (p = 0.00), the overall interocular differences of other biometric parameters between fellow eyes were not significant (p > 0.05). The interocular correlation and Bland-Altman plots showed a good agreement between fellow eyes in 14 biometric parameters. Correlations between interocular differences in SE and that in RJ0 (p = 0.03), CC (p = 0.00), AL (p = 0.00) and VCD (p = 0.00) were statistically significant. There were similar strong linear relationships between refractive error astigmatism vectors and corneal astigmatism vectors in bilateral eyes. There were negative correlations of RJ45 and CJ45 between bilateral eyes. A potentially clinically important interocular symmetry in SE, BCVA, CC, CJ0, CJ45, IOP, CCT, AL, ACD, LT and VCD is found in this research, while the differences of RJ0, RJ45 and Q between left and right eyes seem a bit large. The negative interocular relationships of RJ45 and CJ45 demonstrate moderate mirror symmetry exists among fellow eyes. High interocular symmetry in bilateral eyes may be helpful in intraocular lens power calculation, intraocular pressure evaluation, post-operative visual acuity and refraction
Morrison, S; Kavanagh, J; Obst, S J; Irwin, J; Haseler, L J
finger tremor amplitude and EMG activity, coupled with the lack of any changes in tremor for the more proximal arm segments, indicate that these bilateral effects were mediated by an increase in the central neural drive to both limbs. Together this set of results challenges the general assumption of bilateral independence of tremor production, and further illustrate the task dependent nature of exercise-induced fatigue.
Lok, Julie Y C; Yip, Nelson K F; Chong, Kelvin K L; Li, C L; Young, Alvin L
Idiopathic hypertrophic pachymeningitis is a rare inflammatory condition with diffuse thickening of the dura mater, which may cause a compressive effect or vascular compromise. We report on a 28-year-old Chinese woman with a history of granulomatous mastitis 7 years previously and oligomenorrhoea, headache, blurred vision, and raised prolactin level 2 years previously, that was diagnosed as prolactinoma and treated conservatively with bromocriptine. However, she had recurrent bilateral vision loss when the bromocriptine was stopped. Her symptoms were resolved by high-dose steroid injection but remained steroid-dependent. Serial magnetic resonance imaging scan showed progressive diffuse thickening of the pachymeningitis with disappearance of pituitary apoplexy. Lumbar puncture showed lymphocytosis with no organisms. Open biopsy of the meninges was performed and histology showed features of inflammatory infiltrates and vasculitis. This is an unusual presentation of a rare condition in this age-group, with co-existing granulomatous mastitis and chronic otitis media, and is a diagnostic challenge mimicking pituitary macroadenoma and meningioma in initial magnetic resonance imaging scans.
Milman, N; Pedersen, F M
Idiopathic pulmonary haemosiderosis (IPH) is a rare clinical entity characterized by recurrent episodes of diffuse alveolar haemorrhage, often presenting with haemoptysis. Many patients have iron deficiency anaemia due to deposition of haemosiderin iron in the alveoli, and eventually develop moderate pulmonary fibrosis. Typically, intensive search for an aetiology ends up negative. There is no evidence of pulmonary vasculitis or capillaritis. The aetiology is obscure, but may be an immunological or toxic mechanism causing a defect in the basement membrane of the pulmonary capillary. IPH affects both children and adults. During an acute episode, a chest X-ray demonstrates bilateral, alveolar infiltrates. Sputum examination discloses haemosiderin-laden alveolar macrophages. Diagnosis is established by lung biopsy (fiber-optic or thoracoscopic), showing large numbers of haemosiderin-laden macrophages in the alveoli and without evidence of capillaritis or deposition of immunoglobulins. Corticosteroids and/or immunosuppressive drugs may be effective during an acute bleeding episode, and may in some patients improve symptoms and prognosis on the long-term, but the response to treatment displays great interindividual variation.
van de Graaf, R C; IJpma, F F A; Nicolai, J-P A; Werker, P M N
Bell's palsy is the eponym for idiopathic peripheral facial paralysis. It is named after Sir Charles Bell (1774-1842), who, in the first half of the nineteenth century, discovered the function of the facial nerve and attracted the attention of the medical world to facial paralysis. Our knowledge of this condition before Bell's landmark publications is very limited and is based on just a few documents. In 1804 and 1805, Evert Jan Thomassen à Thuessink (1762-1832) published what appears to be the first known extensive study on idiopathic peripheral facial paralysis. His description of this condition was quite accurate. He located several other early descriptions and concluded from this literature that, previously, the condition had usually been confused with other afflictions (such as 'spasmus cynicus', central facial paralysis and trigeminal neuralgia). According to Thomassen à Thuessink, idiopathic peripheral facial paralysis and trigeminal neuralgia were related, being different expressions of the same condition. Thomassen à Thuessink believed that idiopathic peripheral facial paralysis was caused by 'rheumatism' or exposure to cold. Many aetiological theories have since been proposed. Despite this, the cold hypothesis persists even today.
van Eijndhoven, P; Mulders, P; Kwekkeboom, L; van Oostrom, I; van Beek, M; Janzing, J; Schene, A; Tendolkar, I
Electroconvulsive therapy (ECT) is the most effective treatment for patients suffering from severe or treatment-resistant major depressive disorder (MDD). Unfortunately its underlying neurobiological mechanisms are still unclear. One line of evidence indicates that the seizures produced by ECT induce or stimulate neuroplasticity effects. Although these seizures also affect the cortex, the effect of ECT on cortical thickness is not investigated until now. We acquired structural magnetic resonance imaging data in 19 treatment-resistant MDD patients before and after a bilateral ECT course, and 16 healthy controls at 2 time points, and compared changes in cortical thickness between the groups. Our results reveal that ECT induces significant, bilateral increases in cortical thickness, including the temporal pole, inferior and middle temporal cortex and the insula. The pattern of increased cortical thickness was predominant in regions that are associated with seizure onset in ECT. Post hoc analyses showed that the increase in thickness of the insular cortex was larger in responders than in non-responders, which may point to a specific relationship of this region with treatment effects of ECT. PMID:27552587
Martin, Tristan; Moussay, Sébastien; Bulla, Ingo; Bulla, Jan; Toupet, Michel; Etard, Olivier; Denise, Pierre; Davenne, Damien; Coquerel, Antoine; Quarck, Gaëlle
Background New insights have expanded the influence of the vestibular system to the regulation of circadian rhythmicity. Indeed, hypergravity or bilateral vestibular loss (BVL) in rodents causes a disruption in their daily rhythmicity for several days. The vestibular system thus influences hypothalamic regulation of circadian rhythms on Earth, which raises the question of whether daily rhythms might be altered due to vestibular pathology in humans. The aim of this study was to evaluate human circadian rhythmicity in people presenting a total bilateral vestibular loss (BVL) in comparison with control participants. Methodology and Principal Findings Nine patients presenting a total idiopathic BVL and 8 healthy participants were compared. Their rest-activity cycle was recorded by actigraphy at home over 2 weeks. The daily rhythm of temperature was continuously recorded using a telemetric device and salivary cortisol was recorded every 3 hours from 6:00AM to 9:00PM over 24 hours. BVL patients displayed a similar rest activity cycle during the day to control participants but had higher nocturnal actigraphy, mainly during weekdays. Sleep efficiency was reduced in patients compared to control participants. Patients had a marked temperature rhythm but with a significant phase advance (73 min) and a higher variability of the acrophase (from 2:24 PM to 9:25 PM) with no correlation to rest-activity cycle, contrary to healthy participants. Salivary cortisol levels were higher in patients compared to healthy people at any time of day. Conclusion We observed a marked circadian rhythmicity of temperature in patients with BVL, probably due to the influence of the light dark cycle. However, the lack of synchronization between the temperature and rest-activity cycle supports the hypothesis that the vestibular inputs are salient input to the circadian clock that enhance the stabilization and precision of both external and internal entrainment. PMID:27341473
Brignole, Michele; Deharo, Jean-Claude; Guieu, Regis
Syncope due to idiopathic AV block is characterized by: 1) ECG documentation (usually by means of prolonged ECG monitoring) of paroxysmal complete AV block with one or multiple consecutive pauses, without P-P cycle lengthening or PR interval prolongation, not triggered by atrial or ventricular premature beats nor by rate variations; 2) long history of recurrent syncope without prodromes; 3) absence of cardiac and ECG abnormalities; 4) absence of progression to persistent forms of AV block; 5) efficacy of cardiac pacing therapy. The patients affected by idiopathic AV block have low baseline adenosine plasma level values and show an increased susceptibility to exogenous adenosine. The APL value of the patients with idiopathic AV block is much lower than patients affected by vasovagal syncope who have high adenosine values.
Singh, Arun Kumar; Nandini, R.
Bilateral cleft lip nose deformity is a multi-factorial and complex deformity which tends to aggravate with growth of the child, if not attended surgically. The goals of primary bilateral cleft lip nose surgery are, closure of the nasal floor and sill, lengthening of the columella, repositioning of the alar base, achieving nasal tip projection, repositioning of the lower lateral cartilages, and reorienting the nares from horizontal to oblique position. The multiplicity of procedures in the literature for correction of this deformity alludes to the fact that no single procedure is entirely effective. The timing for surgical intervention and its extent varies considerably. Early surgery on cartilage may adversely affect growth and development; at the same time, allowing the cartilage to grow in an abnormal position and contributing to aggravation of deformity. Some surgeons advocate correction of deformity at an early age. However, others like the cartilages to grow and mature before going in for surgery. With peer pressure also becoming an important consideration during the teens, the current trend is towards early intervention. There is no unanimity in the extent of nasal dissection to be done at the time of primary lip repair. While many perform limited nasal dissection for the fear of growth retardation, others opt for full cartilage correction at the time of primary surgery itself. The value of naso-alveolar moulding (NAM) too is not universally accepted and has now more opponents than proponents. Also most centres in the developing world have neither the personnel nor the facilities for the same. The secondary cleft nasal deformity is variable and is affected by the extent of the original abnormality, any prior surgeries performed and alteration due to nasal growth. This article reviews the currently popular methods for correction of nasal deformity associated with bilateral cleft lip, it's management both at the time of cleft lip repair and also secondarily
Vernie, Lenneke A.; Rothova, Aniki; v. d. Doe, Patricia; Los, Leonoor I.; Schalij-Delfos, Nicoline E.; de Boer, Joke H.
Background Typically juvenile idiopathic arthritis (JIA)-associated uveitis (further referred as ‘JIA-uveitis’) has its onset in childhood, but some patients suffer its, sometimes visual threatening, complications or ongoing disease activity in adulthood. The objective of this study was to analyze uveitis activity, complications and visual prognosis in adulthood. Methods In this multicenter study, 67 adult patients (129 affected eyes) with JIA-uveitis were retrospectively studied for best corrected visual acuity, visual fields, uveitis activity, topical/systemic treatments, ocular complications, and ocular surgeries during their 18th, 22nd and 30th year of life. Because treatment strategies changed after the year 1990, outcomes were stratified for onset of uveitis before and after 1990. Results Sixty-two of all 67 included patients (93%) had bilateral uveitis. During their 18th life year, 4/52 patients (8%) had complete remission, 28/52 (54%) had uveitis activity and 37/51 patients (73%) were on systemic immunomodulatory treatment. Bilateral visual impairment or legal blindness occurred in 2/51 patients (4%); unilateral visual impairment or legal blindness occurred in 17/51 patients (33%) aged 18 years. The visual prognosis appeared to be slightly better for patients with uveitis onset after the year 1990 (for uveitis onset before 1990 (n = 7) four patients (58%) and for uveitis onset after 1990 (n = 44) 13 patients (30%) were either visual impaired or blind). At least one ocular surgery was performed in 10/24 patients (42%) between their 18th and 22nd year of life. Conclusions Bilateral visual outcome in early adulthood in patients with JIA-uveitis appears to be fairly good, although one third of the patients developed one visually impaired or blind eye. However, a fair amount of the patients suffered from ongoing uveitis activity and needed ongoing treatment as well as surgical interventions. Awareness of these findings is important for ophthalmologists and
Cheikhrouhou, Héla; Khiari, Karima; Chérif, Lotfi; Ben Abdallah, Néjib; Ben Maïz, Hédi
The authors report a case of a 49-year-old woman presenting a Cushing's syndrome (January 1997). The Magnetic Resonance Image of the pituitary gland revealed a microadenoma without extension in the cavernous sinus and a partial empty sella. The computed tomography scan showed a discreet bilateral adrenal hyperplasia with a left nodule (23 mm in diameter) a second nodule was noted. These data suggested the eventuality of maconodular adrenocortical hyperplasia in long-standing Cushing's disease. We discuss the implications of this finding for diagnosis, treatment and follow-up.
Basrur, Gurudutt Bhaskar
Inguinal hernias are rare in females. The authors report a case of bilateral inguinal hernias in a 10-year-old female. On exploration, the patient was found to be having a sliding hernia containing incarcerated ovary as contents on both sides. Peroperatively the contents were reduced, the sac was transfixed at its base and the redundant sac was excised. The repair of this form of hernias is more difficult because of adhesions between the contents and the wall of the sac and risk of damage during dissection. A description of this clinical presentation in the pre operative assessment and operative management are discussed in this report. PMID:25918632
Moore, Christopher L.
Telemanipulator system includes master robot manipulated by human operator, and slave robot performing tasks at remote location. Two robots electronically coupled so slave robot moves in response to commands from master robot. Teleoperation greatly enhanced if forces acting on slave robot fed back to operator, giving operator feeling he or she manipulates remote environment directly. Main advantage of bilateral impedance control: enables arbitrary specification of desired performance characteristics for telemanipulator system. Relationship between force and position modulated at both ends of system to suit requirements of task.
Marasinghe, Jeevan P; Condous, George; Amarasinghe, W I
A 28-year-old woman presented at eight weeks and four days of gestation, according to her menstrual dates, complaining of painless vaginal bleeding for three days. Her urinary pregnancy test was positive. Initial transvaginal ultrasound demonstrated an irregular complex structure with a fluid filled centre in the right adnexum. Despite the diagnosis of a possible underlying unruptured right tubal ectopic pregnancy, she declined surgical intervention and was managed expectantly as an inpatient. When she complained of increasing abdominal pain with haemodynamic instability, an emergency laparotomy was performed and a diagnosis of bilateral tubal ectopic pregnancy was made.
Poornima, P; Katkade, Shashikant; Mohamed, Roshan Noor; Mallikarjuna, Rachappa
A 12-year-old patient presented with a severe delay of eruption in permanent maxillary and mandibular incisors. On examination, there was over-retained primary teeth and delayed eruption of permanent teeth. Retained primary teeth showed light yellow discolouration whereas permanent teeth were distinct yellow with thin or little enamel. Subsequent imaging revealed all the premolars except maxillary left first premolar showed signs of intra-alveolar coronal resorption, nephrocalcinosis with bilateral multiple calculi and small papillary tip calcifications, marked increase in alkaline phosphatase. Subsequent dental treatment for restoring the functional and aesthetic requirement followed by appropriate treatment for renal problem was undertaken.
Belloli, Elizabeth A; Beckford, Rosemarie; Hadley, Ryan; Flaherty, Kevin R
Non-specific interstitial pneumonia (NSIP) is an interstitial lung disease that may be idiopathic or secondary to connective tissue disease, toxins or numerous other causes. Idiopathic NSIP is a rare diagnosis and requires exclusion of these other possible causes. Patients typically present in mid-adulthood with dyspnoea, cough and often constitutional symptoms including fever and fatigue. The disease has a female predominance, and more than 50% of patients have never smoked. Physical exam features mild hypoxaemia and inspiratory rales. Pulmonary function tests demonstrate restriction and a low diffusing capacity for carbon monoxide. High-resolution computed tomography abnormalities include predominantly lower lobe subpleural reticular changes, traction bronchiectasis and ground-glass opacities; honeycombing is rarely seen. An evaluation of the underlying pathology is necessary for a firm diagnosis. Histologically, alveolar and interstitial mononuclear cell inflammation and fibrosis are seen in a temporally uniform pattern with preserved underlying alveolar architecture. NSIP must be differentiated from other parenchymal lung diseases including idiopathic pulmonary fibrosis and hypersensitivity pneumonitis. A thorough exposure history and assessment for underlying connective tissue diseases are highly important, as positive findings in these categories would likely denote a case of secondary NSIP. A multi-disciplinary discussion that includes pulmonologist(s), radiologist(s) and pathologist(s) assists in reaching a consensus diagnosis and improves diagnostic accuracy. Treatment of idiopathic NSIP, although not well proven, is generally instituted in the form of immunosuppression. Prognosis is favourable compared with idiopathic pulmonary fibrosis, although the diagnosis still carries an attributable mortality. Herein we will summarize the clinical characteristics and management of idiopathic NSIP.
Mercuri, Louis G
The term "osteoarthritis" has classically been defined as a low-inflammatory arthritic condition. The term "osteoarthrosis," a synonym for osteoarthritis in the medical orthopedic literature, has recently come to be identified in the dental/temporomandibular joint (TMJ) disorders literature with any noninflammatory arthritic condition that results in similar degenerative changes as in osteoarthritis. The term "idiopathic condylar resorption," also known as "progressive condylar resorption," is described as a dysfunctional remodeling of the TMJ manifested by morphologic change, decreased ramal height, progressive mandibular retrusion in the adult, or decreased mandibular growth in the juvenile. This article discusses the diagnosis and management of osteoarthritic TMJ disorders and idiopathic condylar resorption.
Kumari M.K., Kalpana; Mysorekar, Vijaya V.; S., Praveen
Giant-cell myocarditis is a disease of relatively young, predominantly healthy adults. The patients usually die of heart failure and ventricular arrhythmia unless a cardiac transplantation is performed. We are reporting here an autopsy case of idiopathic giant cell myocarditis with no symptoms in a 27-year old -worker who died suddenly. The purpose of this report was to emphasize that idiopathic giant cell myocarditis was a rare disease and that it could exist in the absence of any symptomatic heart disease. PMID:23205365
Ichida, Tatsuya; Kajita, Yoshihiro
The first case of idiopathic thyrotropin (TSH) deficiency in an old woman with thyroid functioning adenoma was reported. She got subtotal thyroidectomy before about four years of her admission to our hospital because of fatigability, puffy face and leg edema. At that time, she had low TSH and free T4 levels despite replacement therapy with desiccated thyroid. No response of only serum TSH after adminstration of combined stimulant containing TRH and repeated TRH suggested the failure of TSH secretion. CT MRI did not show any abnormality. These results indicated that her hypothyroidism was due to acquired idiopathic TSH deficiency. PMID:9159047
Brigante, C; Motta, G; Fusi, F; Coletta, M P; Busacca, M
Eighteen subfertile men, with idiopathic normogonadotropic oligozoospermia were treated with an antiestrogenic compound, tamoxifen (Nolvadex), at the dose of 20 mg/day for four months. Hormonal parameters (LH, FSH, Testosterone, Prolactin) were evaluated before treatment and after 45 and 90 days of therapy. Serum LH, FSH and Testosterone increased significantly after 45 days of tamoxifen treatment. Seminal analyses, performed before and after three months of therapy showed improvements in sperm motility and in sperm density. By our clinical findings, tamoxifen can be considered a useful approach for an empiric treatment of idiopathic oligozoospermia.
Ben-Shlomo, Y; Sieradzan, K
Since the introduction of levodopa therapy for idiopathic Parkinson's disease over 20 years ago, there has been an awakening of research interest in this chronic neuro-degenerative disorder. This paper describes current understanding of the role of genetic and environmental factors in the aetiology of idiopathic Parkinson's disease and problems associated with both diagnosis and management. It briefly outlines both pharmacological and non-pharmacological options for treatment. Despite an increasing armoury of available treatments, the optimum management for this condition remains controversial. PMID:7619574
Jeldres, Alejandro; Labarca, Gonzalo
Idiopathic pulmonary fibrosis has an ominous prognosis and there are virtually no effective therapies. It has been suggested that pirfenidone, an antifibrotic agent, could change its course. Searching in Epistemonikos database, which is maintained by screening multiple databases, we identified 13 systematic reviews comprising nine trials addressing the question of this article, seven of which are randomized and whose results were analyzed in this summary. We combined the evidence using meta-analysis and generated a summary of findings following the GRADE approach. We concluded pirfenidone decreases disease progression and mortality in idiopathic pulmonary fibrosis. Although it is associated with frequent gastrointestinal and cutaneous adverse effects, these are generally not severe.
Moghe, Swapnil; Pillai, Ajay; Guru, Kanishka Navin; Nair, Preeti P
Sickle cell disease is a common inherited autosomal disease that is characterised by abnormally shaped (sickle-shaped) red blood cells (RBCs). It can involve virtually any organ system. The clinical manifestations of sickle cell disease vary and are classified as vaso-occlusion, chronic anaemia and infection. The imaging appearances of central nervous system and musculoskeletal involvement by sickle cell disease have been well documented; however, involvement of the head and neck region is often unreported, although it is not uncommon. In the head and neck, sickle cell disease can involve the inner ears, orbits, paranasal sinuses, bones, lymph nodes and vessels. This paper describes a case of idiopathic facial swelling associated with sickle cell disease in a young patient.
1. Small animals (rat and guinea pig) vagotomized in the neck die within a period of hours, the lungs showing extensive congestion and edema. 2. Tracheotomy permits appreciably longer survival with minimal lung changes approximating those seen in the control animals. 3. Intrathoracic vagotomy (sparing the recurrent laryngeal nerve) on one side, and cervical vagotomy on the other, permits almost indefinite survival (guinea pig and rabbit), unless laryngeal paralysis from the unilateral denervation produces respiratory obstruction (rat, guinea pig, and rabbit). 4. Pulmonary edema following bilateral vagotomy probably results primarily from respiratory obstruction. It is suggested that circulatory failure may also be a factor of some importance. The rôle of vagotomy itself is considered in relationship to these two phenomena. 5. The reaction of smaller animals to bilateral vagotomy, with regard to lung changes, apparently differs in no way from that of the larger animals, but is less readily demonstrated because of the smaller diameters of the air passages. PMID:19870894
Balocco, Simone; Gatta, Carlo; Pujol, Oriol; Mauri, Josepa; Radeva, Petia
Speckle noise negatively affects medical ultrasound image shape interpretation and boundary detection. Speckle removal filters are widely used to selectively remove speckle noise without destroying important image features to enhance object boundaries. In this article, a fully automatic bilateral filter tailored to ultrasound images is proposed. The edge preservation property is obtained by embedding noise statistics in the filter framework. Consequently, the filter is able to tackle the multiplicative behavior modulating the smoothing strength with respect to local statistics. The in silico experiments clearly showed that the speckle reducing bilateral filter (SRBF) has superior performances to most of the state of the art filtering methods. The filter is tested on 50 in vivo US images and its influence on a segmentation task is quantified. The results using SRBF filtered data sets show a superior performance to using oriented anisotropic diffusion filtered images. This improvement is due to the adaptive support of SRBF and the embedded noise statistics, yielding a more homogeneous smoothing. SRBF results in a fully automatic, fast and flexible algorithm potentially suitable in wide ranges of speckle noise sizes, for different medical applications (IVUS, B-mode, 3-D matrix array US).
The effectiveness of the left internal mammary artery graft to the anterior descending coronary artery as a surgical strategy has been shown to improve the survival rate and decrease the risk of adverse cardiac events in patients undergoing coronary bypass surgery. These clinical benefits appear to be related to the superior short and long-term patency rates of the internal thoracic artery graft. Although the advantages of using of both internal thoracic arteries (ITA) for bypass grafting have taken longer to prove, recent results from multiple data sets now support these findings. The major advantage of bilateral ITA grafting appears to be improved survival rate, while the disadvantages of complex ITA grafting include the increased complexity of operation, and an increased risk of wound complications. While these short-term disadvantages have been mitigated in contemporary surgical practice, they have not eliminated. Bilateral ITA grafting should be considered the procedure of choice for patients undergoing coronary bypass surgery that have a predicted survival rate of longer than ten years. PMID:23977627
Kumar, Piyush; Jha, Abhijeet Kumar; Mallik, Sambeet Kumar; Raihan, Mohammed
A 35-year-old man presented with asymptomatic eruption on both forearms and lower aspects of the legs for 6 months. The lesions first appeared on his inner aspects of the wrist, the dorsal surface of the hands, and legs and progressed to involve proximal aspects of the extremities. There was no significant past history. On examination, multiple pearly white papules and depigmented atrophic plaques were found bilaterally on the flexors of the arms and the extensors of the legs. The lesions were arranged in a linear manner, following the lines of Blaschko (Figures 1 and 2). The surface of the atrophic plaques was notable for prominent telangiectasia, giving an erythematous appearance. The genitalia, oral cavity, palms, and soles were spared. Systemic examination was noncontributory. Lichen striatus and extragenital lichen sclerosus (ELS) were considered the differential diagnosis. Clinically, the age of the patient, the absence of scaling, and the presence of atrophic plaques and telangiectasia were in favor of ELS. A punch biopsy from an atrophic plaque was performed, and it revealed hyperkeratosis, atrophic epidermis, basal layer vacuolar degeneration, mild lymphocytic infiltration in the dermis, edema, and homogenization of collagen of the upper portion of the dermis (Figures 3 and Figure 4). Histopathologic findings were consistent with lichen sclerosus. A diagnosis of bilateral zosteriform ELS was made.
Franceschi, Francesco; Barnaba, Simona Angela; Rojas, Mario; Gualdi, Giancarlo; Rizzello, Giacomo; Papalia, Rocco; Denaro, Vincenzo
Knee injuries in young athletes include not only the typical adult bone injuries, ligament and cartilage, but also the growth plate lesions. Osteochondroses are idiopathic, self-limited disturbance of enchondral ossification in which a rapid growth spurt is present. The patella could be affected by two different kinds of osteochondroses: Kohler syndrome and Sinding-Larsen-Johansson. Here we are reporting the first case of simultaneous location of osteochondroses of the two ossification centers of both patella. A 9-year-old boy, competitive skater, presented a history of anterior knee pain involving both knees. Standard X-rays, axial patellar view, MRI and arthro-MR were performed. In order to follow the natural history of the pathology and the evolution of the healing, examinations at 2 years were repeated. We proposed the young skater a medical and a physiotherapeutic treatment based on unloading, isometric exercises, NSAID. As the symptoms improve a gradual return to competitive sports activity was allowed. The case mentioned above can be considered an atypical case because the patient suffered for a bilateral knee osteochondroses, involving simultaneously the primary ossification centre (Kohler syndrome) and the secondary ossification centre (Larsen syndrome) of the patella.
Jeong, Chang Wook; Park, Yong Hyun; Shin, Chan Soo; Kim, Hyeon Hoe
Abstract We present the first report of simultaneous laparoendoscopic single-site surgery (LESS) for bilateral primary aldosterone-producing adrenal adenomas. A 40-year-old man with hypertension that was resistant to pharmacotherapy was shown to have bilateral aldosterone-producing adrenal adenomas. A right partial and a left total adrenalectomy were performed at the same time via a single umbilical incision. The surgery and recovery were uncomplicated. This is the first report of synchronous bilateral LESS.
Babu, Manohar K V; Lee, Peter; Mahadev, Arjandas; Lee, Eng Hin
Unilateral sternocleidomastoid muscle contracture causing torticollis and other secondary deformities such as facial scoliosis, plagiocephaly and scoliosis of cervical spine are well known. The aetiology and pathogenesis is still intriguing. Although unilateral contracture of sternocleidomastoid is seen quite often, bilateral sternocleidomastoid contracture is almost unheard of. A review of the English literature revealed no cases of bilateral congenital sternocleidomastoid contracture being reported. We present a case report of a 19-year-old girl with congenital bilateral sternocleidomastoid contracture.
Diaz, Gloria E; Wirrell, Elaine C; Matsumoto, Joseph Y; Krecke, Karl N
Bilateral striopallidodentate calcinosis is characterized by calcification of the basal ganglia and other gray matter structures. We describe a 16-year-old boy with paroxysmal kinesigenic dyskinesia. He exhibited mineralization in the basal ganglia, posterior thalami, and dentate nuclei bilaterally, and was diagnosed with sporadic bilateral striopallidodentate calcinosis. The paroxysmal kinesigenic dyskinesia responded to low-dose treatment with carbamazepine (200 mg/day).
Carmel, Eldar; Taitelbaum-Swead, Ricky; Migirov, Lela; Hildesheimer, Minka; Kronenberg, Jona
Cochlear implantation is a standard method of hearing rehabilitation among patients with severe to profound bilateral sensorineural hearing loss. In recent years there have been an increasing number of studies showing superior hearing with bilateral cochlear implantation in comparison with a unilateral procedure. In this study we present our experience with 15 patients, children and adults, who had bilateral cochlear implant surgery. Speech perception test results demonstrated a hearing benefit in bilateral cochlear implantation in comparison with a unilateral device, mainly by improvement in the identification of speech in noise tests.
Singh, Aminder; Kaur, Pavneet; Sood, Neena; Puri, Harpreet; Garg, Bhavna
We are reporting a case of bilateral eosinophilic mastitis which is rare and hardly heard. It is a mimicker of carcinoma breast both clinically & radiologically. A 30 years old non diabetic female presented with bilateral breast lumps with history of rhinitis off & on and peripheral eosinophilia. Mammography was suspicious while ultrasonography was diagnostic of bilateral mastitis. Aspiration cytology exhibited inflammatory lesion rich in eosinophils. Histopathology revealed the diagnosis of eosinophilic mastitis. Eosinophilic infiltration of the breast is a rare manifestation of tissue involvement in peripheral eosinophilia and bilateralism is even rarer.
Lancer, Hannah R.
According to the National Hip Fracture Database, over 64,000 patients were admitted with a hip fracture across England, Wales, and Northern Ireland in 2013, but very few are bilateral, and there are no current cases in the literature of bilateral neck of femur fractures in a patient with bilateral below-knee amputations. We present a case of a 69-year-old bilateral below-knee amputee male admitted to the emergency department with bilateral hip pain and radiological evidence of bilateral displaced neck of femur fractures. The patient subsequently underwent synchronous bilateral total hip replacements under general anaesthetic and an epidural and then went on to make a full recovery. He was discharged 27 days after arrival in hospital. Outpatient follow-up at 3 months has shown that the patient has returned to a similar level of preinjury function and is still able to carry out his daily activities with walking aids and bilateral leg prostheses. PMID:26881162
Serrano-Munuera, C; Rojas-García, R; Gallardo, E; De Luna, N; Buenaventura, I; Ferrero, M; García, T; García-Merino, J A; González-Rodríguez, C; Guerriero, A; Marco, M; Márquez, C; Grau, J M; Graus, F; Illa, I
Antidisialosyl antibodies were found in two out of 13 patients with chronic idiopathic ataxic neuropathy (CIAN) and not in 32 patients with different sensory neuropathies of known cause. This finding confirms the association of antidisialosyl antibodies and CIAN regardless of the absence of the M band. These antibodies may have pathogenic relevance; however, larger series are needed to establish their clinical significance.
Attia, Tarek Hamed; Abd Alhamed, Mohamed Maisara; Selim, Mohamed Fouad; Haggag, Mohamed Salah; Fathalla, Diaa
Idiopathic arterial calcification of infancy is a rare autosomal recessive disease, characterized by deposition of calcium along the internal elastic membrane of arteries, accompanied by fibrous thickening of the intima which causes luminal narrowing. Here we are reporting a case of idiopathic arterial calcification of infancy in a Saudi female newborn of non-consanguineous pregnant woman who had polyhydramnios. The newborn baby had severe respiratory distress, systemic hypertension and persistent pulmonary hypertension of newborn. She was admitted to Neonatal Intensive Care Unit, where she was ventilated and proper treatment was provided. Molecular genetic testing was positive for mutations of ectonucleotide pyrophosphatase/phosphodiesterase1 gene which is reported in 80% of cases of Idiopathic arterial calcification of infancy. The baby died at about 5 month of age because of myocardial ischemia and cardiorespiratory arrest. Idiopathic Arterial Calcification of Infancy should be considered in any newborn who presented with persistent pulmonary hypertension of newborn, severe systemic hypertension and echogenic vessels on any radiological study. Calcifications of large and medium-sized arteries are important diagnostic finding.
Barrera, Ana Madeleine; Vargas, Leslie
Pulmonary ossification is a rare and usually asymptomatic finding reported as incidental in lung biopsies. Similarly, idiopathic pulmonary hemosiderosis is a rare cause of pulmonary infiltrates. We report the case of a 64-year old man with chronic respiratory symptoms in whom these two histopathological findings converged.
Gordon Smith, A; Robinson Singleton, J
Peripheral neuropathy is a common problem encountered by neurologists and primary care physicians. While there are many causes for peripheral neuropathy, none can be identified in a large percentage of patients ("idiopathic neuropathy"). Despite its high prevalence, idiopathic neuropathy is poorly studied and understood. There is evolving evidence that impaired glucose tolerance (prediabetes) is associated with idiopathic neuropathy. Preliminary data from a multicenter study of diet and exercise in prediabetes (the Impaired Glucose Tolerance Neuropathy Study) suggests a diet and exercise counseling regimen based on the Diabetes Prevention Program results in improved metabolic measures and small fiber function. Prediabetes is part of the Metabolic Syndrome, which also includes hypertension, hyperlipidemia and obesity. Individual aspects of the Metabolic Syndrome influence risk and progression of diabetic neuropathy and may play a causative role in neuropathy both for those with prediabetes, and those with otherwise idiopathic neuropathy. Thus, a multifactorial treatment approach to individual components of Metabolic Syndrome may slow prediabetic neuropathy progression or result in improvement.
Rihane, B; Le Borgne, J M; Bélair, C
We report a case of idiopathic brachial nevralgia of the right shoulder in a 30-year-old female, after caesarean section, under spinal anaesthesia. Two days after surgery, intense cervical pain appeared on the second day, associated with rapid collapse of muscular shoulder belt. Full recovery occurred in four months.
Bassetti, C; Aldrich, M S
The features of idiopathic hypersomnia are not well defined. We reviewed clinical and laboratory information on 42 subjects with idiopathic hypersomnia and obtained detailed follow-up evaluations on 28 of them. Only 29% of subjects had 'classic' idiopathic hypersomnia with non-imperative sleepiness, long unrefreshing naps, prolonged night-time sleep, difficult awakening with sleep drunkenness and prominent mood disturbances. Thirty-two percent had clinical features similar to narcolepsy, i.e. irresistible sleepiness, short and refreshing naps, few problems with awakening and good response to stimulants, without cataplexy or any indication of abnormal REM (rapid eye movement) sleep. The other 39% had intermediate clinical characteristics. We found no increase in the frequency of the human leucocyte antigens associated with narcolepsy. Overall, response to stimulants was good in three-quarters of the patients and spontaneous improvement of sleepiness occurred in one-quarter. Possible aetiologies identified in 10 patients included viral illness, head trauma and primary mood disorder. Idiopathic hypersomnia is a rare syndrome in which clinical heterogeneity suggests a variable or multifactoral pathogenesis. Only a minority of cases correspond to classical descriptions. Stimulants are often beneficial and spontaneous improvement appears to be more common than in narcolepsy.
Attia, Tarek Hamed; Abd Alhamed, Mohamed Maisara; Selim, Mohamed Fouad; Haggag, Mohamed Salah; Fathalla, Diaa
Idiopathic arterial calcification of infancy is a rare autosomal recessive disease, characterized by deposition of calcium along the internal elastic membrane of arteries, accompanied by fibrous thickening of the intima which causes luminal narrowing. Here we are reporting a case of idiopathic arterial calcification of infancy in a Saudi female newborn of non-consanguineous pregnant woman who had polyhydramnios. The newborn baby had severe respiratory distress, systemic hypertension and persistent pulmonary hypertension of newborn. She was admitted to Neonatal Intensive Care Unit, where she was ventilated and proper treatment was provided. Molecular genetic testing was positive for mutations of ectonucleotide pyrophosphatase/phosphodiesterase1 gene which is reported in 80% of cases of Idiopathic arterial calcification of infancy. The baby died at about 5 month of age because of myocardial ischemia and cardiorespiratory arrest. Idiopathic Arterial Calcification of Infancy should be considered in any newborn who presented with persistent pulmonary hypertension of newborn, severe systemic hypertension and echogenic vessels on any radiological study. Calcifications of large and medium-sized arteries are important diagnostic finding. PMID:27252793
Mahesh, E; Madhyastha, P R; Kalashetty, M; Gurudev, K C; Bande, S; John, M M
We report a case of idiopathic erythrocytosis in a 31-year-old male who was incidentally detected to have hypertension during his preemployment checkup. Urine routine showed proteinuria and hematuria. Biochemical parameters revealed raised serum creatinine, and histological findings of the renal biopsy showed IgAN.
Mahesh, E.; Madhyastha, P. R.; Kalashetty, M.; Gurudev, K. C.; Bande, S.; John, M. M.
We report a case of idiopathic erythrocytosis in a 31-year-old male who was incidentally detected to have hypertension during his preemployment checkup. Urine routine showed proteinuria and hematuria. Biochemical parameters revealed raised serum creatinine, and histological findings of the renal biopsy showed IgAN. PMID:28182057
Naik, Keyur; Mandel, Louis
Sialosis is observed in relation to diabetes, alcoholism, and malnutrition. An assumed relation between gout and sialosis is probably based on confusion that originated from the therapeutic use of phenylbutazone for gout and the sialadenitis that the medication caused. This report describes a case of sialosis in a patient with a longstanding history of gout that was idiopathic in origin.
Hawasli, Ammar H.; Hullar, Timothy E.; Dorward, Ian G.
Purpose Despite its high prevalence, the etiology underlying idiopathic scoliosis remains unclear. Although initial scrutiny has focused on genetic, biochemical, biomechanical, nutritional and congenital causes, there is growing evidence that aberrations in the vestibular system may play a role in the etiology of scoliosis. In this article, we discuss putative mechanisms for adolescent idiopathic scoliosis and review the current evidence supporting a role for the vestibular system in adolescent idiopathic scoliosis. Methods A comprehensive search of the English literature was performed using PubMed (http://www.ncbi.nlm.nih.gov/pubmed). Research articles studying interactions between adolescent idiopathic scoliosis and the vestibular system were selected and evaluated for inclusion in a literature review. Results Eighteen manuscripts of level 3-4 clinical evidence to support an association between AIS and dysfunction of the vestibular system. These studies include data from physiologic and morphologic studies in humans. Clinical data are supported by animal model studies to suggest a causative link between the vestibular system and AIS. Conclusions Clinical data and a limited number of animal model studies suggest a causative role of the vestibular system in AIS, although this association has not been reproduced in all studies. PMID:25430569
Page, T L; Caldarola, P C; Pittendrigh, C S
The interactions between the bilaterally distributed components of the circadin system that controls the locomotor activity rhythm of the cockroach Leucophaea maderae were investigated in a series of surgical lesion experiments. Complete excision of one optic lobe (either right or left) or its surgical isolation from the central nervous system had no effect on the animals' ability to free-run in constant darkness nor was there any indication, as judged by postoperative pi values of any difference between left and right lobe pacemakers. However, these surgical procedures consistently resulted in a significant increase in tau over preoperative value while optic nerve section had no effect on tau. The propostion is developed that the left and right pacemakers in the two optic lobes are mutally coupled and that the compound pacemaker's period is shorter than either of its constituent pacemakers. It was also found that the integrity of either compound eye is sufficient to assure entrainment of both left and right pacemakers. Images PMID:265571
Gulbahar, Gultekin; Kaplan, Tevfik; Turker, Hasan Bozkurt; Gundogdu, Ahmet Gokhan; Han, Serdar
First rib fractures are scarce due to their well-protected anatomic locations. Bilateral first rib fractures accompanying bilateral scapular fractures are very rare, although they may be together with scapular and clavicular fractures. According to our knowledge, no case of bilateral first rib fractures accompanying bilateral scapular fractures has been reported, so we herein discussed the diagnosis, treatment, and complications of bone fractures due to thoracic trauma in bias of this rare entity. PMID:26175916
Huebner, Stephen; Ali, Sayed
Intravascular migration of bullets and other foreign bodies is a rare but known complication of penetrating trauma. Missile embolization can represent a diagnostic challenge because it may present in various and unexpected ways. We present the case of a 54-year-old female who sustained shotgun pellet emboli to the pulmonary arteries following a left upper extremity gunshot wound and related vascular surgery. The case illustrates bilateral embolization, and the embolic events occurred following surgery. Embolization should be considered in evaluating patients with gunshot wounds, particularly if there are anomalous symptoms or the projectile is not found in the original, or expected, location. Close attention to the location of the foreign bodies on serial radiographs may reveal the diagnosis of intravascular embolization. PMID:22690290
Kyrtsos, Christina Rose; Stahl, Mark C.; Eslinger, Paul; Subramanian, Thyagarajan; Lucassen, Elisabeth B.
Capgras syndrome is a delusional misidentification syndrome (DMS) which can be seen in neurodegenerative diseases such as Lewy body dementia and, to a lesser extent, in Parkinson's disease (PD). Here, we report the case of a 78-year-old man with a history of idiopathic PD who developed Capgras syndrome following bilateral subthalamic nucleus deep brain stimulation (DBS) implantation. As the risk of DMS has been related to deficits in executive, memory, and visuospatial function preoperatively, this case highlights the importance of continuing to improve patient selection for DBS surgery. Capgras syndrome is a rare potential complication of DBS surgery in PD patients with preexisting cognitive decline. PMID:26078747
Cho, Deog Gon; Lee, Seok In; Chang, Yong Jin; Cho, Kyu Do; Cho, Suk Kyu
Background Simultaneously developed bilateral primary spontaneous pneumothorax (BPSP) is an indication for thoracic surgery of both sides. Recently, we have reported a new technique for BPSP, which is ipsilateral apicoposterior transmediastinal (TM) bullectomy of both sides using video-assisted thoracoscopic surgery (VATS), and we compared this TM VATS with bilateral sequential (BS) VATS for BPSP. Materials and Methods From June 2003 to May 2014, 11 and 14 patients were performed VATS TM and BS bullectomy for BPSP, respectively. We reviewed the medical records and compared the clinical data between the two groups. For TM group, we first performed the right VATS bullectomy and approached through the apicoposterior mediastinal region for contralateral VATS. In the other group, conventional BS VATS bullectomy was performed in the lateral decubitus position change. Results The mean follow-up was 62.0 ± 32.6 months. No mortality and major complications were observed. The operative time (68.18 ± 24.93 vs. 96.07 ± 37.73, p = 0.046), duration of left pleural drainage (1.00 ± 0.45 vs. 3.21 ± 1.37, p = 0.000), and length of hospital stay (3.82 ± 1.54 vs. 4.93 ± 1.07, p = 0.044) were significantly shorter in the TM group than in the BS group. No significant differences were seen in duration of general anesthesia, total number of wedge resections and endostaplers used in both lungs, duration of right drainage, and postoperative recurrence. Conclusion The TM VATS approach may be a safe and feasible modality for BPSP. It may decrease the operative time, patients inconvenience such as bilateral multiple wounds and longstanding placement of chest tubes, and decrease the hospital stay compared with the BS VATS approach.
Cosentino, Carlos; Velez, Miriam; Nuñez, Yesenia; Palomino, Henry; Quispe, Darko; Flores, Martha; Torres, Luis
Background Lesions in the Guillain–Mollaret triangle or dentate-rubro-olivary pathway may lead to hypertrophic olivary degeneration (HOD), a secondary trans-synaptic degeneration of the inferior olivary nucleus. HOD is usually associated with palatal tremor and rarely with Holmes tremor. Bilateral HOD is a very unusual condition and very few cases are reported. Case Report We report here two cases of bilateral HOD after two different vascular lesions located at the decussation of superior cerebellar peduncles, thus impairing both central tegmental tracts and interrupting bilaterally the dentate-rubral-olivary pathway. Interestingly, both developed bilateral Holmes tremor but not palatal tremor. Discussion Lesions in some of the components in the Guillain–Mollaret triangle may develop Holmes tremor with HOD and without palatal tremor. Magnetic resonance imaging is an invaluable tool in these cases. Better understanding of the pathways in this loop is needed. PMID:27536461
Inoue, Yasuteru; Miyashita, Fumio; Koga, Masatoshi; Yamada, Naoaki; Toyoda, Kazunori; Minematsu, Kazuo
Bilateral medial medullary infarction (MMI) is a rare type of stroke with poor outcomes. Inferior olivary nucleus hypertrophy results from a pathologic lesion in the Guillain-Mollaret triangle. The relationship between inferior olivary nucleus hypertrophy and the medullary lesion is obscure. To the best of our knowledge, only 1 autopsy case with unilateral medial medullary infarction that was associated with ipsilateral inferior olivary nucleus hypertrophy has been reported. We describe a rare case with acute infarction in the bilateral medial medulla oblongata accompanied by subacute bilateral inferior olivary nucleus hypertrophy and panmedullary edema. The hypertrophy appeared to have been caused by local ischemic damage to the termination of the central tegmental tract at the bilateral inferior olivary nucleus.
Dahan, Meryl; Lim, Chetana; Salloum, Chady
Postoperative bilateral adrenal hemorrhage is a rare but potentially life-threatening complication. This diagnosis is often missed because the symptoms and laboratory results are usually nonspecific. We report a case of bilateral adrenal hemorrhage associated with acute primary adrenal insufficiency following laparoscopic cholecystectomy. The knowledge of this uncommon complication following any abdominal surgery allows timey diagnosis and rapid treatment. PMID:27275469
Ling, D.; Korobkin, M.; Silverman, P.M.; Dunnick, N.R.
Bilateral adrenal hemorrhage with subsequent adrenal insufficiency is a recognized complication of anticoagulant therapy. Because the clinical manifestations are often nonspecific, the antemortem diagnosis of adrenal hemorrhage has been a difficult clinical problem. Computed tomography (CT) provides detailed images of the adrenal glands that are not possible with conventional imaging methods. The CT findings of bilateral adrenal hemorrhage in an anticoagulated patient are reported.
Montiani-Ferreira, Fabiano; Lima, Leandro; Bacellar, Marianna; D'Otaviano Vilani, Ricardo G; Fedullo, José Daniel; Lange, Rogério R
A 14-year-old, female, captive-born orangutan (Pongo pygmaeus) developed bilateral cataracts. Ultrasonography, electroretinography and cataract correction using phacoemulsification were performed bilaterally. This case report aims to describe the ophthalmic procedures performed in this animal critically endangered of extinction. The surgery successfully restored vision and normal activity to the patient.
Gadodia, A; Bhalla, A S; Sharma, R; Thakar, A; Parshad, R
Bilateral parotid swelling is not an uncommon occurrence and may pose a challenge for clinicians and radiologists. Numerous causes of bilateral parotid swellings have been identified. The purpose of this pictorial review is to display this wide array with a focus on multimodality approach. PMID:21960397
A 4-week-old male Pomeranian was presented with eyes that had remained closed since birth. Clinical examination of the orbits revealed that globes were bilaterally absent, suggesting clinical anophthalmos. Following ultrasound imaging of the orbits, a diagnosis of severe bilateral microphthalmos was made, a condition of rare occurrence with an etiology that is not fully understood.
Dahan, Meryl; Lim, Chetana; Salloum, Chady; Azoulay, Daniel
Postoperative bilateral adrenal hemorrhage is a rare but potentially life-threatening complication. This diagnosis is often missed because the symptoms and laboratory results are usually nonspecific. We report a case of bilateral adrenal hemorrhage associated with acute primary adrenal insufficiency following laparoscopic cholecystectomy. The knowledge of this uncommon complication following any abdominal surgery allows timey diagnosis and rapid treatment.
Cho, Sung Ik; Lee, Jung Hwan
Lumbar intraspinal synovial cysts are included in the difference diagnosis of lumbar radiculopathy. Developing imaging modalities has result in increased reporting about these lesions. However, the case of bilateral new lumbar intraspinal synovial cysts after laminectomy has been rarely reported. We report of a rare case with bilateral lumbar intraspinal synovial cysts after laminectomy, requiring surgical excision. PMID:27799997
Werler, Martha M; Yazdy, Mahsa M; Mitchell, Allen A; Meyer, Robert E; Druschel, Charlotte M; Anderka, Marlene; Kasser, James R; Mahan, Susan T
Clubfoot is a common structural malformation, occurring in approximately 1/1,000 live births. Previous studies of sociodemographic and pregnancy-related risk factors have been inconsistent, with the exception of the strong male preponderance and association with primiparity. Hypotheses for clubfoot pathogenesis include fetal constraint, Mendelian-inheritance, and vascular disruption, but its etiology remains elusive. We conducted a population-based case-control study of clubfoot in North Carolina, Massachusetts, and New York from 2007 to 2011. Mothers of 677 clubfoot cases and 2,037 non-malformed controls were interviewed within 1 year of delivery about socio-demographic and reproductive factors. Cases and controls were compared for child's sex, maternal age, education, cohabitation status, race/ethnicity, state, gravidity, parity, body mass index (BMI), and these pregnancy-related conditions: oligohydramnios, breech delivery, bicornuate uterus, plural birth, early amniocentesis (<16 weeks), chorionic villous sampling (CVS), and plural gestation with fetal loss. Odds ratios (ORs) and 95% confidence intervals (CIs) were adjusted for state. Cases were more likely to be male (OR: 2.7; 2.2-3.3) and born to primiparous mothers (1.4; 1.2-1.7) and mothers with BMI ≥30 kg/m(2) (1.4; 1.1-1.8). These associations were greatest in isolated and bilateral cases. ORs for the pregnancy-related conditions ranged from 1.3 (breech delivery) to 5.6 (early amniocentesis). Positive associations with high BMI were confined to cases with a marker of fetal constraint (oligohydramnios, breech delivery, bicornuate uterus, plural birth), inheritance (family history in 1st degree relative), or vascular disruption (early amniocentesis, CVS, plural gestation with fetal loss). Pathogenetic factors associated with obesity may be in the causal pathway for clubfoot.
Werler, Martha M.; Yazdy, Mahsa M.; Mitchell, Allen A.; Meyer, Robert E.; Druschel, Charlotte M.; Anderka, Marlene; Kasser, James R.; Mahan, Susan T.
Clubfoot is a common structural malformation, occurring in approximately 1/1000 live births. Previous studies of sociodemographic and pregnancy-related risk factors have been inconsistent, with the exception of the strong male preponderance and association with primiparity. Hypotheses for clubfoot pathogenesis include fetal constraint, Mendelian-inheritance, and vascular disruption, but its etiology remains elusive. We conducted a population-based case-control study of clubfoot in North Carolina, Massachusetts, and New York from 2007 to 2011. Mothers of 677 clubfoot cases and 2,037 non-malformed controls were interviewed within one year of delivery about socio-demographic and reproductive factors. Cases and controls were compared for child’s sex, maternal age, education, cohabitation status, race/ethnicity, state, gravidity, parity, body mass index (BMI), and these pregnancy-related conditions: oligohydramnios, breech delivery, bicornuate uterus, plural birth, early amniocentesis (<16 weeks), chorionic villous sampling (CVS), and plural gestation with fetal loss. Odds ratios (ORs) and 95% confidence intervals (CIs) were adjusted for state. Cases were more likely to be male (OR: 2.7; 2.2–3.3) and born to primiparous mothers (1.4; 1.2–1.7) and mothers with BMI ≥30 kg/m2 (1.4; 1.1–1.8). These associations were greatest in isolated and bilateral cases. ORs for the pregnancy-related conditions ranged from 1.3 (breech delivery) to 5.6 (early amniocentesis). Positive associations with high BMI were confined to cases with a marker of fetal constraint (oligohydramnios, breech delivery, bicornuate uterus, plural birth), inheritance (family history in 1st degree relative), or vascular disruption (early amniocentesis, CVS, plural gestation with fetal loss). Pathogenetic factors associated with obesity may be in the causal pathway for clubfoot. PMID:23686911
Val-Bernal, J Fernando; Argueta, Liza; Fernández-Flórez, Alejandro; Correas, Miguel Angel; Val, Daniel; Garijo, M Francisca; López-Rasines, Gerardo
Xanthogranulomatous orchitis (XGO) is a rare chronic inflammatory process characterized by destruction of tissue that is replaced by an outstanding cellular infiltrate of lipid-laden macrophages. To date, 20 cases of this process have been reported previously. We present herein the case of a 55-year-old man who had sustained complete tetraplegia at C-6 level and neuropathic bladder for 21 years. After repeated episodes of urinary tract infection, the patient developed a bilateral XGO and a right xanthogranulomatous epididymitis (XGE) that were treated with bilateral orchiepididymectomy. To our knowledge, a bilateral XGO has not yet been reported. Repeated episodes of high-pressure urinary reflux along the vas deferens during dyssynergic voiding possibly led to retrograde extension from the urinary tract by common urinary pathogens and development of bilateral XGO and right XGE. Since tissue destruction is a feature of this process, curative treatment required antibiotic therapy followed by bilateral excision of testes and epididymes.
Kurian, Jujju Jacob; Ninan, Pradeep Joseph
Childhood cystic partially differentiated nephroblastoma (CPDN) is an uncommon renal neoplasm. Bilateral CPDN or CPDN co-existing with a cystic nephroma/Wilms tumour is extremely rare. Treatment of CPDN is by complete surgical excision. Although local recurrences are uncommon, distant metastases have not been described. We present a case of bilateral CPDN that, after complete excision, recurred as bilateral cystic Wilms tumour. To the best of our knowledge, this is the first reported case in the literature where a bilateral CPDN has recurred as bilateral Wilms tumour.
Sun, Tong; Liu, Jing; Zhao, De Wei
Abstract There are a number of conflicting reports describing the clinical outcomes of using N-acetylcysteine for the treatment of idiopathic pulmonary fibrosis. We have, therefore, performed a meta-analysis to evaluate the efficacy of N-acetylcysteine, compared with control, for the treatment of idiopathic pulmonary fibrosis. Original controlled clinical trials evaluating the efficacy of N-acetylcysteine for the treatment of idiopathic pulmonary fibrosis were included in the analysis. Searches for relevant articles were carried out in July 2014 by 2 independent researchers using PubMed, Embase, Cochrane Central, and Google Scholar. Change in forced vital capacity, change in percentage of predicted vital capacity, change in percentage of predicted carbon monoxide diffusing capacity, changes in 6 minutes walking test distance, rate of adverse events, and rate of death were expressed as outcomes using RevMan 5.0.1. Five trials, with a total of 564 patients, were included in this meta-analysis. The meta-analysis showed that the control group had significant decreases in percentage of predicted vital capacity (standardized mean difference [SMD] = 0.37; 95% confidence interval [CI]: 0.13 to −0.62; P = 0.003) and 6 minutes walking test distance (SMD = 0.25; 95% CI: 0.02–0.48; P = 0.04). There were no statistically significant differences in forced vital capacity (SMD = 0.07; 95% CI: −0.13–0.27; P = 0.52), percentage of predicted carbon monoxide diffusing capacity (SMD = 0.12; 95% CI: −0.06–0.30; P = 0.18), rates of adverse events (odd ratio = 4.50; 95% CI: 0.19–106.41; P = 0.35), or death rates (odd ratio = 1.79; 95% CI: 0.3–5.12; P = 0.28) between the N-acetylcysteine group and the control group. N-Acetylcysteine was found to have a significant effect only on decreases in percentage of predicted vital capacity and 6 minutes walking test distance. N-acetylcysteine showed no beneficial effect on changes
Kotwicki, Tomasz; Chowanska, Joanna; Kinel, Edyta; Czaprowski, Dariusz; Tomaszewski, Marek; Janusz, Piotr
Idiopathic scoliosis is a three-dimensional deformity of the growing spine, affecting 2%–3% of adolescents. Although benign in the majority of patients, the natural course of the disease may result in significant disturbance of body morphology, reduced thoracic volume, impaired respiration, increased rates of back pain, and serious esthetic concerns. Risk of deterioration is highest during the pubertal growth spurt and increases the risk of pathologic spinal curvature, increasing angular value, trunk imbalance, and thoracic deformity. Early clinical detection of scoliosis relies on careful examination of trunk shape and is subject to screening programs in some regions. Treatment options are physiotherapy, corrective bracing, or surgery for mild, moderate, or severe scoliosis, respectively, with both the actual degree of deformity and prognosis being taken into account. Physiotherapy used in mild idiopathic scoliosis comprises general training of the trunk musculature and physical capacity, while specific physiotherapeutic techniques aim to address the spinal curvature itself, attempting to achieve self-correction with active trunk movements developed in a three-dimensional space by an instructed adolescent under visual and proprioceptive control. Moderate but progressive idiopathic scoliosis in skeletally immature adolescents can be successfully halted using a corrective brace which has to be worn full time for several months or until skeletal maturity, and is able to prevent more severe deformity and avoid the need for surgical treatment. Surgery is the treatment of choice for severe idiopathic scoliosis which is rapidly progressive, with early onset, late diagnosis, and neglected or failed conservative treatment. The psychologic impact of idiopathic scoliosis, a chronic disease occurring in the psychologically fragile period of adolescence, is important because of its body distorting character and the onerous treatment required, either conservative or surgical
Zapater Latorre, E; Castillo Ruiz, A; Alba García, J R; Armengot Carceller, M; Sancho Rieger, J; Basterra Alegría, J
Simultaneous bilateral facial paralisis (SBFP) occurs in 0.3-2% of all facial paralisis. We report a case of SBFP in association with Lyme disease. A review of literature about SBFP is made, studing specially the one caused by Borrelia burgdorferi. We present a diagnostic guideline of SBFP. Suspect diagnosis of Lyme disease is based on clinical and epidemiological criteria. Culture isolation of this bacteria is difficult, therefore serologic testing is required. Neuroborreliosis treatment is intravenous Ceftriaxone or Cefotaxime. Oral Doxycycline is useful in the treatment of neuritis without central nervous system involvement.
Sherani, Khalid M.; Upadhyay, Hinesh N.; Sherani, Farha K.; Vakil, Abhay P.; Sarkar, Samir S.
Diffuse alveolar hemorrhage (DAH) is characterized by the presence of hemoptysis, anemia, and the presence of diffuse parenchymal infiltrates on imaging studies. Idiopathic pulmonary hemosiderosis (IPH) is an uncommon cause of diffuse alveolar hemorrhage (DAH) and is classically known to present in childhood. Adult-onset IPH is extremely rare. We report the case of a 48-year-old female patient who presented with hemoptysis and acute hypoxic respiratory failure, requiring intubation and mechanical ventilation. Imaging studies showed diffuse bilateral patchy infiltrates. Bronchoalveolar lavage (BAL) confirmed the diagnosis of DAH. Extensive workup including video-assisted thoracoscopic surgical lung biopsy (VATS) failed to reveal any vasculitis, infectious, immunological or connective tissue disorder, as the underlying cause for DAH. The patient was successfully treated with high-dose steroid therapy. PMID:26180395
Arima, Hiroshi; Azuma, Yoshinori; Morishita, Yoshiaki; Hagiwara, Daisuke
ABSTRACT Central diabetes insipidus (CDI), characterized by polyuria and polydipsia, is caused by deficiency of arginine vasopressin (AVP), an antidiuretic hormone which acts on V2 receptors in kidney to promote reabsorption of free water. CDI is classified into three subtypes; idiopathic, secondary and familial. A previous study suggests that infundibulo-neurohypophysitis might be an underlying cause of idiopathic CDI. Among secondary CDI, the tumors in the central nervous system such as craniopharyngioma and germ cell tumors are the most frequent causes. Familial CDI is inherited mostly in an autosomal dominant mode, and the number of causal mutations in the AVP gene locus reported so far exceeds 80. CDI is treated with desmopressin, an analogue of vasopressin, and the tablet is preferred to the nasal form because it is easier to administer. It is also shown that the oral disintegrating tablet formula increases QOL and decreases the incidence of hyponatremia in CDI patients. In some CDI patients, the osmoreceptors in the hypothalamus do not function and patients do not sense thirst. These adipsic CDI patients are treated with desmopressin and adjusting the amount of daily water intake based on body weight measurement; but controlling the water balance is extremely difficult, and morbidity and mortality are shown to be high in these patients. PMID:28008190
Yamada, Takumi; Kay, G Neal
Idiopathic ventricular arrhythmias (VAs) are ventricular tachycardias (VTs) or premature ventricular contractions (PVCs) with a mechanism that is not related to myocardial scar. The sites of successful catheter ablation of idiopathic VA origins have been progressively elucidated and include both the endocardium and, less commonly, the epicardium. Idiopathic VAs usually originate from specific anatomical structures such as the ventricular outflow tracts, aortic root, atrioventricular (AV) annuli, papillary muscles, Purkinje network and so on, and exhibit characteristic electrocardiograms based on their anatomical background. Catheter ablation of idiopathic VAs is usually safe and highly successful, but can sometimes be challenging because of the anatomical obstacles such as the coronary arteries, epicardial fat pads, intramural and epicardial origins, AV conduction system and so on. Therefore, understanding the relevant anatomy is important to achieve a safe and successful catheter ablation of idiopathic VAs. This review describes the anatomical consideration in the catheter ablation of idiopathic VAs.
Kay, G Neal
Idiopathic ventricular arrhythmias (VAs) are ventricular tachycardias (VTs) or premature ventricular contractions (PVCs) with a mechanism that is not related to myocardial scar. The sites of successful catheter ablation of idiopathic VA origins have been progressively elucidated and include both the endocardium and, less commonly, the epicardium. Idiopathic VAs usually originate from specific anatomical structures such as the ventricular outflow tracts, aortic root, atrioventricular (AV) annuli, papillary muscles, Purkinje network and so on, and exhibit characteristic electrocardiograms based on their anatomical background. Catheter ablation of idiopathic VAs is usually safe and highly successful, but can sometimes be challenging because of the anatomical obstacles such as the coronary arteries, epicardial fat pads, intramural and epicardial origins, AV conduction system and so on. Therefore, understanding the relevant anatomy is important to achieve a safe and successful catheter ablation of idiopathic VAs. This review describes the anatomical consideration in the catheter ablation of idiopathic VAs. PMID:28116086
Elsaman, Ahmed Mohammed Mahrous Yousif; Thabit, Mohamed Nasreldin; Radwan, Ahmed Roshdy Al-Agamy; Ohrndorf, Sarah
The objective of the work described here was to evaluate the depth of the carpal tunnel (DCT) in patients with idiopathic carpal tunnel syndrome (CTS) and healthy volunteers by ultrasonography (US), through measurement of the distance from the flexor retinaculum to the surface of the capitate bone at the carpal tunnel outlet, and compare it with other ultrasonographic and electrophysiologic parameters in CTS. The study was conducted in 60 non-diabetic patients with idiopathic carpal tunnel syndrome (unilateral n = 37, bilateral n = 23) evidenced by electrophysiologic diagnosis according to the criteria of the American Association of Electrodiagnostic Medicine (AAEM). Furthermore, 40 hands from 20 healthy volunteers were examined. Median nerve cross-sectional area (CSA); flattening ratio (FR), the ratio of the length to the width of the median nerve; and DCT at the canal outlet were measured for all participants. The mean age was 35.6 ± 9.48 y. The female-to-male ratio was 47:13 in the CTS patients. The sensitivity and specificity were 82% and 95% for CSA, 75% and 60% for FR and 75% and 87.5% for DCT, respectively. Differences between patients and healthy controls were significant for all three parameters, greatest for DCT, followed by CSA and then FR. We conclude that DCT increased in CTS and this new parameter is comparable in sensitivity and specificity to CSA and FR. DCT increased independently of the cause of the CTS (decrease in size of canal or increase in contents).
Sato, Suguru; Tanino, Yoshinori; Misa, Kenichi; Fukuhara, Naoko; Nikaido, Takefumi; Uematsu, Manabu; Fukuhara, Atsuro; Wang, Xintao; Ishida, Takashi; Munakata, Mitsuru
Objective Since the term "combined pulmonary fibrosis and emphysema" (CPFE) was first proposed, the co-existence of pulmonary fibrosis and pulmonary emphysema (PE) has drawn considerable attention. However, conflicting results on the clinical characteristics of patients with both pulmonary fibrosis and PE have been published because of the lack of an exact definition of CPFE. The goal of this study was thus to clarify the clinical characteristics and phenotypes of idiopathic interstitial pneumonia (IIP) with PE. Methods We retrospectively analyzed IIP patients who had been admitted to our hospital. Their chest high-resolution computed tomography images were classified into two groups according to the presence of PE. We then performed a cluster analysis to identify the phenotypes of IIP patients with PE. Results Forty-four (53.7%) out of 82 patients had at least mild emphysema in their bilateral lungs. The cluster analysis separated the IIP patients with PE into three clusters. The overall survival rate of one cluster that consisted of mainly idiopathic pulmonary fibrosis (IPF) patients was significantly worse than those of the other clusters. Conclusion Three different phenotypes can be identified in IIP patients with PE, and IPF with PE is a distinct clinical phenotype with a poor prognosis.
Crumpler, L. S.; Head, J. W.; Campbell, D. B.
There are three main highland areas on Venus: Beta Regio, Ishtar Terra and Aphrodite Terra. The latter is least known and the least mapped, yet existing analyses of Aphrodite Terra based on available Pioneer-Venus orbiter data suggest that it may be the site of extensive rifting. Some of the highest resolution (30 km) PV data (SAR) included most of the western half of Aphrodite Terra. Recent analysis of the SAR data together with Arecibo range-doppler topographic profiling (10 X 100 km horizontal and 10 m vertical resolution) across parts of Aphrodite, further characterized the nature of possible tectonic processes in the equatorial highlands. The existence of distinct topographic and radar morphologic linear discontinuities across the nearly east-west strike of Aphrodite Terra is indicated. Another prominent set of linear features is distinctly parallel to and orthogonal to the ground tracks of the PV spacecraft and are not included because of the possibility that they are artifacts. Study of the northwest trending cross-strike discontinuities (CSD's) and the nature of topographic and morphologic features along their strike suggest the presence of bilateral topographic and morphologic symmetry about the long axis of Aphrodite Terra.
Zampetti, Benedetta; Grossrubatscher, Erika; Dalino Ciaramella, Paolo; Boccardi, Edoardo; Loli, Paola
Simultaneous bilateral inferior petrosal sinus sampling (BIPSS) plays a crucial role in the diagnostic work-up of Cushing's syndrome. It is the most accurate procedure in the differential diagnosis of hypercortisolism of pituitary or ectopic origin, as compared with clinical, biochemical and imaging analyses, with a sensitivity and specificity of 88-100% and 67-100%, respectively. In the setting of hypercortisolemia, ACTH levels obtained from venous drainage of the pituitary are expected to be higher than the levels of peripheral blood, thus suggesting pituitary ACTH excess as the cause of hypercortisolism. Direct stimulation of the pituitary corticotroph with corticotrophin-releasing hormone enhances the sensitivity of the procedure. The procedure must be undertaken in the presence of hypercortisolemia, which suppresses both the basal and stimulated secretory activity of normal corticotrophic cells: ACTH measured in the sinus is, therefore, the result of the secretory activity of the tumor tissue. The poor accuracy in lateralization of BIPSS (positive predictive value of 50-70%) makes interpetrosal ACTH gradient alone not sufficient for the localization of the tumor. An accurate exploration of the gland is recommended if a tumor is not found in the predicted area. Despite the fact that BIPSS is an invasive procedure, the occurrence of adverse events is extremely rare, particularly if it is performed by experienced operators in referral centres.
Grossrubatscher, Erika; Dalino Ciaramella, Paolo; Boccardi, Edoardo
Simultaneous bilateral inferior petrosal sinus sampling (BIPSS) plays a crucial role in the diagnostic work-up of Cushing’s syndrome. It is the most accurate procedure in the differential diagnosis of hypercortisolism of pituitary or ectopic origin, as compared with clinical, biochemical and imaging analyses, with a sensitivity and specificity of 88–100% and 67–100%, respectively. In the setting of hypercortisolemia, ACTH levels obtained from venous drainage of the pituitary are expected to be higher than the levels of peripheral blood, thus suggesting pituitary ACTH excess as the cause of hypercortisolism. Direct stimulation of the pituitary corticotroph with corticotrophin-releasing hormone enhances the sensitivity of the procedure. The procedure must be undertaken in the presence of hypercortisolemia, which suppresses both the basal and stimulated secretory activity of normal corticotrophic cells: ACTH measured in the sinus is, therefore, the result of the secretory activity of the tumor tissue. The poor accuracy in lateralization of BIPSS (positive predictive value of 50–70%) makes interpetrosal ACTH gradient alone not sufficient for the localization of the tumor. An accurate exploration of the gland is recommended if a tumor is not found in the predicted area. Despite the fact that BIPSS is an invasive procedure, the occurrence of adverse events is extremely rare, particularly if it is performed by experienced operators in referral centres. PMID:27352844
Suvirya, Swastika; Agrawal, Avinash; Parihar, Anit
A 42-year-old woman being treated with 5-fluorouracil for carcinoma of the sigmoid colon developed persistent serpentine supravenous hyperpigmented eruption (PSSHE), bilateral mottling of the palms and diffuse hyperpigmentation of the soles. To the best of our knowledge, such a combination of findings has not been reported earlier. Recognition and knowledge of this side effect are important as the dose of the drug need not be altered nor is there a need to replace the drug on worries about a serious adverse effect. However, changing the peripheral venous route to the central line can be useful in management of PSSHE.
Riggio, Oliviero; Gioia, Stefania; Pentassuglio, Ilaria; Nicoletti, Valeria; Valente, Michele; d'Amati, Giulia
The term idiopathic noncirrhotic portal hypertension (INCPH) has been recently proposed to replace terms, such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative hyperplasia, used to describe patients with a hepatic presinusoidal cause of portal hypertension of unknown etiology, characterized by features of portal hypertension (esophageal varices, nonmalignant ascites, porto-venous collaterals), splenomegaly, patent portal, and hepatic veins and no clinical and histological signs of cirrhosis. Physicians should learn to look for this condition in a number of clinical settings, including cryptogenic cirrhosis, a disease known to be associated with INCPH, drug administration, and even chronic alterations in liver function tests. Once INCPH is clinically suspected, liver histology becomes mandatory for the correct diagnosis. However, pathologists should be familiar with the histological features of INCPH, especially in cases in which histology is not only requested to exclude liver cirrhosis.
Selva-O'Callaghan, Albert; Ramos Casals, Manel; Grau Junyent, Josep M
The aim of this article is to study the evidence-based knowledge related to the use of biological therapies in patients diagnosed with idiopathic inflammatory myopathy (dermatomyositis, polymyositis and inclusion body myositis). In this review the leading published studies related to the use of biological therapy in patients with myositis are analysed; mainly those with high methodological standards, that means randomized and controlled studies. Methodological drawbacks due to the rarity and heterogeneity of these complex diseases are also addressed. Up to now is not possible to ascertain the biologics as a recommended therapy in patients with myositis, at least based in the current evidence-based knowledge, although it can not be neglected as a therapeutic option in some clinical situations, taking into account the scarce of effective treatments in those patients, especially in refractory myositis. Future studies probably will help to better define the role of biological therapies in patients with idiopathic inflammatory myopathy.
Schwier, Nicholas C; Coons, James C; Rao, Shivdev K
Idiopathic (viral) pericarditis is the most common form of pericardial disease in the Western world. Despite the combination of colchicine and nonsteroidal antiinflammatory drugs (NSAIDs) plus aspirin (ASA), considered first-line therapy, the incidence of recurrent pericarditis is ~20-30%. In addition, secondary recurrence without optimal first-line therapy is ~50%. This is due to the many clinical challenges, such as inappropriate NSAID/ASA duration of therapy, the use of corticosteroid therapy, contraindications or intolerances to therapy, adverse effects, and issues related to adherence. This review describes contemporary pharmacotherapeutic management of idiopathic (viral) pericarditis, with a particular emphasis on the role of colchicine. Emerging therapies and management strategies, such as high-sensitivity C-reactive protein-guided therapy and novel immunotherapies, are also reviewed. Ultimately, understanding appropriate treatment will assist the clinician in helping decrease the risk of recurrent, incessant, and refractory pericarditis.
Dianzani, C; Gaspardini, F; Persichetti, P; Brunetti, B; Pizzuti, A; Margiotti, K; Degener, A M
Scrotal elephantiasis is very rare disease in industrialized countries, where it is mainly due to surgery, irradiation or malignancies. It can be defined as idiopathic only when the possible congenital, infectious and compressive causes are excluded. We report a case of massive scrotal lymphoedema in an adult Caucasian patient, in Italy. He presented an extremely voluminous scrotal mass measuring 50 x 47 x 13 cm (weight 18 kg), which extended below his knees, invalidating all his daily activities. The patient was hospitalized in order to undergo to surgical treatment. Although genetic causes were searched and the possible role of infectious agents and compressive factors was evaluated, no etiology was ascertained. Histopathologic examination showed non-specific chronic inflammation, confirming the diagnosis of idiopathic elephantiasis. One year after surgical treatment, the patient is healthy without recurrence signs.
Riggio, Oliviero; Gioia, Stefania; Pentassuglio, Ilaria; Nicoletti, Valeria; Valente, Michele; d’Amati, Giulia
The term idiopathic noncirrhotic portal hypertension (INCPH) has been recently proposed to replace terms, such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative hyperplasia, used to describe patients with a hepatic presinusoidal cause of portal hypertension of unknown etiology, characterized by features of portal hypertension (esophageal varices, nonmalignant ascites, porto-venous collaterals), splenomegaly, patent portal, and hepatic veins and no clinical and histological signs of cirrhosis. Physicians should learn to look for this condition in a number of clinical settings, including cryptogenic cirrhosis, a disease known to be associated with INCPH, drug administration, and even chronic alterations in liver function tests. Once INCPH is clinically suspected, liver histology becomes mandatory for the correct diagnosis. However, pathologists should be familiar with the histological features of INCPH, especially in cases in which histology is not only requested to exclude liver cirrhosis. PMID:27555800
Parker, W D; Swerdlow, R H
Disordered mitochondrial metabolism may play an important role in a number of idiopathic neurodegenerative disorders. The question of mitochondrial dysfunction is particularly attractive in the case of idiopathic Parkinson disease (PD), since Vyas et al. recognized in the 1980s that the parkinsonism-inducing compound N-methyl-4-phenyl-1,2,3,6-tetrahydropyridine is a mitochondrial toxin. The unique genetic properties of mitochondria also make them worthy of consideration for a pathogenic role in PD, as well as in other late-onset, sporadic neurodegenerative disorders. Although affected persons occasionally do provide family histories that suggest Mendelian inheritance, the vast majority of the time these diseases appear sporadically. Because of unique features such as heteroplasmy, replicative segregation, and threshold effects, mitochondrial inheritance can allow for the apparent sporadic nature of these diseases.
Oldham, Justin M.; Noth, Imre
Summary Idiopathic pulmonary fibrosis (IPF), a devastating progressive interstitial lung disease (ILD) with no known cause or cure, is the most common and deadly of the idiopathic interstitial pneumonias. With a median survival of 3–5 years following diagnosis, IPF is characterized by a progressive decline in lung function and quality of life in most patients. Vigilance among clinicians in recognizing IPF early in the disease course remains critical to properly caring for these patients, as this provides the widest range of management options. When IPF is suspected, a multidisciplinary evaluation (MDE) by a clinician, radiologist and pathologist with ILD expertise should occur, as this improves diagnostic agreement in both community and academic settings. When community MDE is not possible, or diagnostic doubt exists, referral to an ILD center should be considered. ILD center referral may also provide access specialized care, including clinical trials and lung transplantation, and should be considered for any patient with an established diagnosis of IPF. PMID:24746629
Zhu, Bo; Xu, Gu-feng; Liu, Yi-feng; Qu, Fan; Yao, Wei-miao; Zhu, Yi-min; Gao, Hui-juan; Zhang, Dan
Ectopic pregnancy is identified with the widely-applied assisted reproductive technology (ART). Bilateral ectopic pregnancy is a rare form of ectopic pregnancy which is difficult to be diagnosed at the pre-operation stage. In this paper, we presented an unusual case of heterochronic bilateral ectopic pregnancy after stimulated intrauterine insemination (IUI), where there has been a delay of 22 d between the diagnoses of the two ectopic pregnancies. Literature was reviewed on the occurrence of bilateral ectopic pregnancy during the past four years in the MEDLINE database. We found 16 cases of bilateral ectopic pregnancy reported since 2008, and analyzed the characteristics of those cases of bilateral ectopic pregnancy. We emphasize that ovulation induction and other ARTs may increase the risk of bilateral ectopic pregnancy. Because of the difficulty in identification of bilateral ectopic pregnancy by ultrasonography, the clinician should be aware that the treatment of one ectopic pregnancy does not preclude the occurrence of a second ectopic pregnancy in the same patient and should pay attention to the intra-operation inspection of both side fallopian tubes in any ectopic pregnancy case.
Lorente-Muñoz, Asís; Cortés-Franco, Severiano; Moles-Herbera, Jesús; Casado-Pellejero, Juan; Rivero-Celada, David; Alberdi-Viñas, Juan
Idiopathic spinal cord herniation is a rare cause of thoracic myelopathy and its recurrence is even more infrequent. Cord herniation is through an anterior dural defect in thoracic spine with unknown causes. Symptomatic cases must be surgically treated to reduce the hernia and seal the defect to prevent recurrences. We report a patient presenting a Brown-Séquard syndrome secondary to a D5 spinal cord herniation treated successfully and its posterior traumatic recurrence.
Desy, Nicholas M; Bernstein, Mitchell; Harvey, Edward J; Hazel, Hazel
Kienbock's disease or osteonecrosis of the lunate is an uncommon cause of wrist pain. . Though there have been several reports of cases in patients with various rheumatologic diseases, the precise etiology has currently not been established. We report a case of Kienbock's disease that occurred in a patient with juvenile idiopathic arthritis. To our knowledge, this is the first case report with an association between these two conditions.
A 76-year-old man with a longstanding history of cold sensitivity developed wheals after the application of an ice cube. Cold urticaria is a type of physical urticaria that is characterized urticaria and angioedema after exposure to cold. It may be idiopathic or secondary to hematologic or infectious diseases. Treatment of primary cold urticaria includes antihistamines; however, ketotifen, doxantrazole, zafirlukast, cyclosporine, and cold-tolerance induction may be tried in refractory cases.
Background Cellular changes associated with diabetic and idiopathic gastroparesis are not well described. Aim Describe histologic abnormalities in gastroparesis and compare findings in idiopathic versus diabetic gastroparesis. Methods Full thickness gastric body biopsies were obtained from 40 gastroparetics (20 diabetic) and matched controls. Sections were stained for H&E and trichrome, and immunolabeled with antibodies against PGP 9.5, nNOS, VIP, substance P and tyrosine hydroxylase to quantify nerves, S100β for glia, Kit for interstitial cells of Cajal (ICC), CD45 and CD68, for immune cells and smoothelin for smooth muscle cells. Tissue was also examined by transmission electron microscopy (TEM). Results Histological abnormalities were found in 83% of patients. Most common defects were loss of ICC with remaining ICC showing injury, an abnormal immune infiltrate containing macrophages, and decreased nerve fibers. On light microscopy, no significant differences were found between diabetic and idiopathic gastroparesis with the exception of nNOS expression which was decreased in more idiopathic gastroparetics (40%) compared to diabetic (20%) patients by visual grading. On electron microscopy, a markedly increased connective tissue stroma was present in both disorders. Conclusion This study suggests that on full thickness biopsies, cellular abnormalities are found in the majority of patients with gastroparesis. Most common findings were loss of Kit expression suggesting loss of ICC and an increase in CD45 and CD68 immunoreactivity. These findings suggest that examination of tissue can lead to valuable insights into the pathophysiology of these disorders and offers hope that new therapeutic targets can be found. PMID:21300066
Doody, O.; Adam, W. R.; Foley, P. T.; Lyon, S. M.
Fibromuscular dysplasia (FMD) describes a group of conditions which cause nonatheromatous arterial stenoses, most commonly of the renal and carotid arteries, typically in young women. We report a rare case of bilateral segmental renal infarction secondary to FMD in a young male patient. His initial presentation with loin pain and pyrexia resulted in a delay in the definitive diagnosis of FMD. He was successfully treated with bilateral balloon angioplasty. The delayed diagnosis in this patient until the condition had progressed to bilateral renal infarcts highlights the need for prompt investigation and diagnosis of suspected cases of FMD.
Shetty, J P; Shetty, Bhaskar; Makkanavar, J H; Chandrika
Diagnosis of ectopic pregnancy continues to be an important challenge. Here a case of incidental diagnosis of bilateral tubal ectopic pregnancy is reported. There was a ruptured tubal ectopic pregnancy on the left side and chronic ruptured tubal ectopic pregnancy on the right side. Leading cause of first trimester maternal deaths is due to complications of ectopic pregnancy. Here an unusual case of ectopic pregnancy is reported in which the patient had spontaneous bilateral tubal ectopic pregnancy which presented with left tubal rupture and subsequently emergency exploratory laparotomy revealed bilateral tubal mass, which on histopathological examination confirmed tubal pregnancy.
Kim, So-Yeon; Jang, Han-Bin; Kim, Jinu; Yoon, Sang Pil
The sternocleidomastoid muscle shows a wide range of variations including supernumerary muscular heads. We found a rare variation in the sternocleidomastoid muscle with bilateral supernumerary heads in a 67-year-old Korean male cadaver. Bilateral four muscle bellies were recorded: two sternomastoids, one cleido-occipital and one cleido-mastoid occipital on the right side, and one sternomastoid, one cleido-occipital and two cleido-mastoids on the left side. The variation of bilateral four heads on sternocleidomastoid muscle is important to surgeons and anesthetists for clinical using.
Huppke, Peter; Heise, Alexander; Rostasy, Kevin; Huppke, Brenda; Gärtner, Jutta
Idiopathic hypothalamic dysfunction is a rare disorder presenting at age 3-7 years. Severe hypothalamic and brainstem dysfunction leads to death in 25% of patients. The disease is presumed to be autoimmune, or in some cases paraneoplastic. No successful treatment has been reported. Patient V. developed hyperphagia, hypersomnia, and extreme aggression at age 7 years, accompanied by episodes of hyperthermia, hypothermia, sinus bradycardia, hypernatremia, hyponatremia, persistent hyperprolactinemia, hypothyroidism, and growth-hormone deficiency. At age 9 years, a diagnosis of idiopathic hypothalamic dysfunction was rendered, and immunoglobulin therapy was commenced. Nine courses of immunoglobulins, at a dose of 2 g/kg every 4 weeks, were administered. Reproducible improvements in behavior and no further episodes of hyponatremia or hypernatremia and sinus bradycardia were evident. The endocrinologic abnormalities and poor thermoregulation remained. Administration of immunoglobulins during late stages of idiopathic hypothalamic dysfunction led to improvement in some but not all signs. Assuming an autoimmune basis for this disorder, treatment during early stages of disease should be more effective. To facilitate such early treatment, increased awareness of this disorder is necessary, to allow for early diagnosis.
Hirota, Shozo; Ichikawa, Satoshi; Matsumoto, Shinichi; Motohara, Tomofumi; Fukuda, Tetsuya; Yoshikawa, Takeshi
Purpose: To evaluate the usefulness of interventional radiological treatment for idiopathic portal hypertension. Methods: Between 1995 and 1998, we performed an interventional radiological treatment in five patients with idiopathic portal hypertension, four of whom had refused surgery and one of whom had undergone surgery. Three patients with gastroesophageal varices (GEV) were treated by partial splenic embolization (PSE), one patient with esophageal varices (EV) and massive ascites by transjugular intrahepatic portosytemic shunt (TIPS) and PSE, and one patient with GEV by percutaneous transhepatic obliteration (PTO). Midterm results were analyzed in terms of the effect on esophageal and/or gastric varices. Results: In one woman with severe GEV who underwent three sessions of PSE, there was endoscopic confirmation that the GEV had disappeared. In one man his EV shrunk markedly after two sessions of PSE. In two patients slight reduction of the EV was obtained with one application of PSE combined with endoscopic variceal ligation therapy. PTO for GV in one patient resulted in good control of the varices. All patients have survived for 16-42 months since the first interventional treatment, and varices are well controlled. Conclusion: Interventional radiological treatment is effective for patients with idiopathic portal hypertension, whether or not they have undergone surgery.
Poletti, Venerino; Ravaglia, Claudia; Gurioli, Carlo; Piciucchi, Sara; Dubini, Alessandra; Cavazza, Alberto; Chilosi, Marco; Rossi, Andrea; Tomassetti, Sara
Fibrosing interstitial lung diseases (f-ILDs) represent a heterogeneous group of disorders in which the aetiology may be identified or, not infrequently, remain unknown. Establishing a correct diagnosis of a distinct f-ILD requires a multidisciplinary approach, integrating clinical profile, physiological and laboratory data, radiological appearance and, when appropriate, histological findings. Surgical lung biopsy is still considered the most important diagnostic tool as it is able to provide lung samples large enough for identification of complex patterns such as usual interstitial pneumonitis (UIP) and nonspecific interstitial pneumonitis. However, this procedure is accompanied by significant morbidity and mortality. Bronchoalveolar lavage is still a popular diagnostic tool allowing identification of alternative diagnoses in patients with suspected idiopathic pulmonary fibrosis (IPF) when an increase in lymphocytes is detected. Conventional transbronchial lung biopsy has a very low sensitivity in detecting the UIP pattern and its role in this clinical-radiological context is marginal. The introduction of less invasive methods such as transbronchial cryobiopsy show great promise to clinical practice as they can be used to obtain samples large enough to morphologically support a diagnosis of IPF or other idiopathic interstitial pneumonias, along with fewer complications. Recent advances in the field suggest that less invasive methods of lung sampling, without significant side effects, in combination with other diagnostic methods could replace the need for surgical lung biopsy in the future. Indeed, these new multidisciplinary procedures may become the main diagnostic work-up method for patients with suspected idiopathic interstitial pneumonia.
Gellér, László; Szilágyi, Szabolcs; Solymossy, Katalin; Srej, Marianna; Zima, Endre; Tahin, Tamás; Merkely, Béla
Idiopathic fascicular ventricular tachycardia is an important and not very rare cardiac arrhythmia with specific electrocardiographic features and therapeutic options. Ventricular tachycardia is characterized by relatively narrow QRS complex and right bundle branch block pattern. The QRS axis depends on which fascicle is involved in the re-entry. Left axis deviation is noted with left posterior fascicular tachycardia and right axis deviation with left anterior fascicular tachycardia. A left septal fascicular tachycardia with normal QRS axis is also possible. Idiopathic fascicular tachycardia is usually seen in individuals without structural heart disease. Response to verapamil is an important feature of fascicular tachycardia. In some cases intravenous adenosine may also terminate the arrhythmia. During electrophysiology study, presystolic or diastolic potentials precede the QRS, presumed to originate from the Purkinje fibers. The potentials can be recorded during sinus rhythm and ventricular tachycardia in many patients with fascicular tachycardia. This potential (so-called Purkinje potential) has been used as a guide to catheter ablation. Correct diagnosis of fascicular tachycardia is very important because catheter ablation is very effective in the treatment of this type of ventricular tachycardia. In this review, we describe three patients with idiopathic ventricular tachycardia and their successful catheter ablation, and summarize the actual knowledge of the diagnosis and management of this special ventricular tachycardia.
Caraffa, Laurent; Tarel, Jean-Philippe; Charbonnier, Pierre
The bilateral filter and its variants, such as the joint/cross bilateral filter, are well-known edge-preserving image smoothing tools used in many applications. The reason of this success is its simple definition and the possibility of many adaptations. The bilateral filter is known to be related to robust estimation. This link is lost by the ad hoc introduction of the guide image in the joint/cross bilateral filter. We here propose a new way to derive the joint/cross bilateral filter as a particular case of a more generic filter, which we name the guided bilateral filter. This new filter is iterative, generic, inherits the robustness properties of the robust bilateral filter, and uses a guide image. The link with robust estimation allows us to relate the filter parameters with the statistics of input images. A scheme based on graduated nonconvexity is proposed, which allows converging to an interesting local minimum even when the cost function is nonconvex. With this scheme, the guided bilateral filter can handle non-Gaussian noise on the image to be filtered. A complementary scheme is also proposed to handle non-Gaussian noise on the guide image even if both are strongly correlated. This allows the guided bilateral filter to handle situations with more noise than the joint/cross bilateral filter can work with and leads to high peak signal-to-noise ratio values as shown experimentally.
Sarma, Nilendu; Chakraborty, Sayantani; Bhattacharya, Sneha Ranjan
Background: Acquired, non-nevoid, apparently idiopathic facial pigmentation are distributed over some specific locations like periorbital area, zygomatic area, malar area, root of nose, perioral and mandibular area. Periorbital pigmentation is the most well known entity in this group. These are bilaterally distributed homogenously diffuse gray to dark gray or slate-gray colored patches showing progressive intensification of pigmentation. These are often considered as physiologic or constitutional pigmentation. Some portions of the margins of these patches were described previously as pigmentary demarcation line (PDL- F, G, H). Aim: To analyze the distributional patterns of acquired, apparently idiopathic facial pigmentations and to evaluate the etiologic aspects of these conditions. Materials and Methods: Spatial patterns, distribution, and orientation were analyzed among 187 individuals with idiopathic non-nevoid, facial pigmentation. Observed patterns were compared with various pigmentary nevi and Blaschko's lines on face. Results: It was found that most of the idiopathic facial pigmentary alterations including periorbital pigmentation and PDL on face had specific patterned distribution that had high similarity to that of the pigmentary nevi and Blaschko's lines on face. Conclusion: It is hypothesized here that phenotypic expression of acquired patterned pigmentation (AIFPFP) is due to genetically determined increased pigmentary functional activity to various known and unknown yet natural factors like UV rays and aging. Mosaicism was a definite possibility. We also consider that the patterns actually reflected the normal patterns of embryological human pigmentation on face. PMID:24470659
Biyani, C S; Torella, F; Cornford, P A; Brough, S J
This case report describes a patient with bilateral nephrocutaneous fistulae and xanthogranulomatous pyelonephritis. Contralateral involvement of the psoas muscle is a rare occurrence and has not been previously documented.
Central hypersomnias are diseases manifested in excessive daytime sleepiness (EDS) not caused by disturbed nocturnal sleep or misaligned circadian rhythms. Central hypersomnias includes narcolepsy with and without cataplexy, recurrent hypersomnia, idiopathic hypersomnia, with and without long sleep time, behaviorally induced insufficient sleep syndrome, hypersomnia and narcolepsy due to medical conditions, and finally hypersomnia induced by substance intake. The Epworth Sleepiness Scale is a subjective tool mostly used for EDS assessment, while the Multiple Sleep Latency Test serves as an objective diagnostic method for narcolepsy and idiopathic hypersomnias. As for symptomatic therapy of EDS, the central nervous system stimulants modafinil and methylphenidate seem to work well in most cases and in narcolepsy and Parkinson’s disease; sodium oxybate also has notable therapeutic value. PMID:22973425
Sharma, Anand; Sharma, Achal; Mittal, Radhey S; Gandhi, Ashok
Bilateral cerebellopontine angle (CPA) arachnoid cysts (ACs) are very rare: only one case is reported in literature. Pathogenesis of those cysts is unknown; they are thought to be congenital. The presenting symptoms of CPA AC are frequently nonspecific or otological. The management of ACs of the CPA is controversial. We are reporting two cases of bilateral CPA AC with their pathophysiology and review of literature.
Neto, Francisco Laitano; Zerwes, Felipe Pereira
Fibroadenoma is the most common benign tumor of the breast but giant juvenile fibroadenoma represent only 0.5% of all fibroadenomas and when bilateral are much more rare. We describe the case of a 25 years old girl that presented with bilateral giant juvenile fibroadenomas and was treated by bilateral skin reducing mastectomy using the inferior dermal flap, implant, and free nipple graft. PMID:27563567
Nehete, Rajendra; Nehete, Anita; Singla, Sandeep; Adhav, Harshad
In bilateral thumb amputations, the functional impairment is serious and every attempt should be made to reconstruct the thumb. We report a case of bilateral post traumatic thumb amputation, reconstructed with bilateral second toe transfer. Only two such cases have been reported in literature so far. Though there are various modalities for the reconstruction of thumb, microvascular toe transfer has its own merits. The convalescent period is minimal with excellent function. It is bilaterally symmetric and aesthetically superior to the osteoplastic reconstruction. The technical details are discussed, and the long term functional and aesthetic results are presented.
Nguyen, Mai P; Lawler, Ericka A; Morcuende, Jose A
We report a rare case of a patient with bilateral mirror clubfeet and bilateral hand polydactyly. The patient presented to our orthopaedic clinic with bilateral mirror clubfeet, each with eight toes, and bilateral hands with six fingers and a hypoplastic thumb. The pattern does not fit any described syndrome such as Martin or Laurin-Sandrow syndrome. Treatments by an orthopaedic pediatric surgeon and an orthopaedic pediatric hand surgeon are described. The patient achieved excellent functional and cosmetic outcomes at four year follow-up.
Nehete, Rajendra; Nehete, Anita; Singla, Sandeep; Adhav, Harshad
In bilateral thumb amputations, the functional impairment is serious and every attempt should be made to reconstruct the thumb. We report a case of bilateral post traumatic thumb amputation, reconstructed with bilateral second toe transfer. Only two such cases have been reported in literature so far. Though there are various modalities for the reconstruction of thumb, microvascular toe transfer has its own merits. The convalescent period is minimal with excellent function. It is bilaterally symmetric and aesthetically superior to the osteoplastic reconstruction. The technical details are discussed, and the long term functional and aesthetic results are presented. PMID:22754176
Fillet, G.; Beguin, Y.; Baldelli, L. )
Iron transport in the reticuloendothelial (RE) system plays a central role in iron metabolism, but its regulation has not been characterized physiologically in vivo in humans. In particular, why serum iron is elevated and RE cells are much less iron-loaded than parenchymal cells in idiopathic hemochromatosis is not known. The processing of erythrocyte iron by the RE system was studied after intravenous (IV) injection of 59Fe heat-damaged RBCs (HDRBCs) and 55Fe transferrin in normal subjects and in patients with iron deficiency, idiopathic hemochromatosis, inflammation, marrow aplasia, or hyperplastic erythropoiesis. Early release of 59Fe by the RE system was calculated from the plasma iron turnover and the 59Fe plasma reappearance curve. Late release was calculated from the ratio of 59Fe/55Fe RBC utilization in 2 weeks. The partitioning of iron between the early (release from heme catabolism) and late (release from RE stores) phases depended on the size of RE iron stores, as illustrated by the inverse relationship observed between early release and plasma ferritin (P less than .001). There was a strong correlation between early release and the rate of change of serum iron levels during the first three hours in normal subjects (r = .85, P less than .001). Inflammation produced a blockade of the early release phase, whereas in idiopathic hemochromatosis early release was considerably increased as compared with subjects with similar iron stores. Based on these results, we describe a model of RE iron metabolism in humans. We conclude that the RE system appears to determine the diurnal fluctuations in serum iron levels through variations in the immediate output of heme iron. In idiopathic hemochromatosis, a defect of the RE cell in withholding iron freed from hemoglobin could be responsible for the high serum iron levels and low RE iron stores.
Karamanou, M; Antoniou, C; Stratigos, A J; Saridaki, Z; Androutsos, G
In 1872, the Hungarian born dermatologist Moriz Kaposi that was practicing in Vienna first described a rare endemic disease that bears his name, among elderly persons of Central European or Mediterranean origin named "idiopathic multiple pigmented sarcoma of the skin". Ten years later the Italian dermatologist Tommaso de Amicis confirms Kaposi's findings. For more than a century the disease was known as a rare low grade malignancy till the 1980s AIDS epidemic.
Bedoya, Maria A; McGraw, Michael H; Wells, Lawrence; Jaramillo, Diego
Bilateral agenesis of the anterior cruciate ligament (ACL) is extremely rare. We describe a 13-year-old girl who presented with bilateral knee pain without history of trauma; she has two family members with knee instability. Magnetic resonance imaging showed bilateral absence of the ACL, and medial posterior horn meniscal tears. Bilateral arthroscopic partial meniscectomy and anterior cruciate ligament reconstruction was performed.
Dauvilliers, Yves; Lopez, Régis
Central hypersomnias include narcolepsy type 1, type 2 and idiopathic hypersomnia with daytime sleepiness excessive in the foreground of the clinical symptoms. Despite major advances in our understanding of the mechanisms of the narcolepsy type 1 with a low level of hypocretin-1 in cerebrospinal fluid, its current management is only symptomatic. The current management is also only symptomatic for type 2 narcolepsy and idiopathic hypersomnia with an unknown pathophysiology. Treatment options may vary from a single drug targeting several symptoms or several drugs treating a specific symptom. The treatment of daytime sleepiness is based on modafinil in first intention. Other psychostimulants such as methylphenidate, pitolisant and exceptionally dextro-amfetamine may be considered. In narcolepsy type 1, antidepressants such as inhibitors of the reuptake of serotonin and noradrenaline will be considered to improve cataplexy. Sodium oxybate is an effective treatment on sleepiness, cataplexy and bad night sleep in narcolepsy. The management for other symptoms or comorbidities should be considered it necessary such as hallucinations, sleep paralysis, the disturbed nighttime sleep, unpleasant dreams, parasomnias, depressive symptoms, overweight/obesity, cardiovascular disease and obstructive sleep apnea syndrome. Important therapeutic perspectives are to be expected concerning new psychostimulant and anticataplectiques, but mainly on immune-based therapies administered as early as possible after disease onset and on hypocretin replacement therapy for patients with severe symptoms.
Wiegand, Samantha L; Beamon, Carmen J; Chescheir, Nancy C; Stamilio, David
Objective To estimate the association between the severity of idiopathic polyhydramnios and adverse outcomes. Study Design Retrospective cohort study of deliveries at one hospital from 2000 to 2012 with an amniotic fluid index (AFI) measurement ≥24 + 0 weeks' gestation. Pregnancies complicated by diabetes, multiples, or fetal anomalies were excluded. Exposure was the degree of polyhydramnios: normal (AFI 5-24 cm), mild (≥ 24-30 cm), and moderate-severe (> 30 cm). Primary outcomes were perinatal mortality, neonatal intensive care unit (NICU) admission, and postpartum hemorrhage. Results There were 10,536 pregnancies: 10,188 with a normal AFI, 274 mild (78.74%), and 74 moderate-severe polyhydramnios (21.26%). Adverse outcomes were increased with idiopathic polyhydramnios: NICU admission (adjusted odds ratio [AOR] 3.71, 95% confidence interval [CI] 2.77-4.99), postpartum hemorrhage (AOR 15.81, 95% CI 7.82-31.96), macrosomia (AOR 3.41, 95% CI 2.61-4.47), low 5-minute Apgar score (AOR 2.60, 95% CI 1.57-4.30), and cesarean (AOR 2.16, 95% CI 1.74-2.69). There were increasing odds of macrosomia (mild: AOR 3.19, 95% CI 2.36-4.32; moderate-severe: AOR 4.44, 95% CI 2.53-7.79) and low 5-minute Apgar score (mild: AOR 2.24, 95% CI 1.23-4.08; moderate-severe: AOR 3.93, 95% CI 1.62-9.55) with increasing severity of polyhydramnios. Conclusion Idiopathic polyhydramnios is independently associated with increased risks of morbidity. There appears to be a dose-response relationship for neonatal macrosomia and low 5-minute Apgar score risks.
Lee, C-S; Chen, T-L; Tzen, C-Y; Lin, F-J; Peng, M-J; Wu, C-L; Chen, P-J
Interstitial lung disease (ILD) in patients with myositis is defined by the presence of interstitial changes on radiographic examination. The reported prevalence of ILD varies from 0% to nearly 50%. However, only rarely has the pathological pattern of diffuse alveolar damage (DAD) associated with idiopathic inflammatory myopathy (IIM) been reported. We report five patients with IIM (one with dermatomyositis, one with polymyositis, and three with amyopathic dermatomyositis) and respiratory failure. Four underwent open lung biopsy with pathological proof of diffuse alveolar damage (DAD). Despite intensive immunosuppressive therapy, all of them died. In addition to the case reports, we discuss DAD in patients with IIM.
Zacharakis, Evangelos; Dudderidge, Tim; Zacharakis, Emmanouil; Ioannidis, Evangelos
Scrotal lymphedema (scrotal elephantiasis) is uncommon outside of filariasis endemic regions. We present a case of a 65-year-old with idiopathic lymphedema of the scrotum and functional impairment of the penis. The patient underwent surgical excision of the edematous subcutaneous tissues and plastic reconstruction of his penis and scrotum. Three years later, the patient showed no signs of local recurrence, had complete restoration of urinary and sexual function and was extremely satisfied with the result. Surgical management was an effective strategy in the management of scrotal lymphedema in this case.
Izzo, Anthony; McSweeney, Julia; Kulik, Thomas; Khatwa, Umakanth; Kothare, Sanjeev V
The usual differential diagnoses of nocturnal events in children include parasomnias, nocturnal seizures, nocturnal reflux (Sandifer syndrome), hypnic jerks, periodic limb movements of sleep, and sleep disordered breathing. We report a previously healthy young girl who presented to the sleep clinic for evaluation of nocturnal events which were diagnosed as medically refractory nocturnal seizures. It was not until a syncopal event occurred in the daytime, which prompted referral for cardiac evaluation, the diagnosis of idiopathic pulmonary arterial hyper-tension (IPAH) was made. Sleep physicians should consider IPAH in the differential diagnosis of nocturnal events in children.
Mauler, Daniela A; Van Soens, Iris; Bhatti, Sofie F; Cornelis, Ine; Martlé, Valentine A; Van Ham, Luc M
Two male neutered domestic shorthair cats were evaluated for generalised tremors. On neurological examination both cats showed whole-body tremors, worsening with stress. A mainly cerebellar disorder was suspected. Blood examination, cerebrospinal fluid analysis and electrophysiological examination of both cats and magnetic resonance imaging of the brain in one cat were normal. Idiopathic generalised tremor syndrome (IGTS) was suspected owing to the exclusion of underlying causes and the clinical similarities with the syndrome in dogs. Treatment as recommended for dogs was initiated and resulted in improvement. This report describes the first cases of IGTS in cats.
Sabato, Alessandro Fabrizio
Breakthrough pain (BTP) in treated patients with chronic pain states is neither well defined nor well understood. BTP is generally defined as a transient exacerbation of pain experienced by a patient with relatively stable and adequately controlled baseline pain. It is usually categorized as spontaneous, with no known cause, or incident, when initiated by voluntary or involuntary movements, or therapeutic procedures. Since pain is related to survival, it possibly cannot be completely and permanently controlled. It is hypothesized that glia are at least partially responsible for inducing pain spikes by attempting to reactivate unresponsive neurons. Therefore, compounds that modulate microglia may offer potential alternative therapeutic options in the control of idiopathic BTP.
Idiopathic hirsutism (IH) is defined as hirsutism in conjunction with normal ovulatory function and normal serum androgen levels. The pathogenesis of IH is still not clear. Increased peripheral 5alpha-reductase enzyme activity and abnormalities of androgen receptor gene polymorphisms have been postulated to explain the pathogenesis of this disorder. It is diagnosed in women who have hirsutism, normal ovulatory function, and normal levels of serum total or free testosterone. Combination treatment of IH, including androgen suppression, peripheral androgen blockade and cosmetic methods is most effective.
Marín-Manzano, E; González-de-Olano, D; Haurie-Girelli, J; Herráiz-Sarachaga, J I; Bermúdez-Cañete, R; Tamariz-Martel, A; Cuesta-Gimeno, C; Pérez-de-León, J
A 6-year-old-boy presented with epigastric pain and vomiting over 1 year. Chest X-ray and esophagogastric transit showed a mediastinal mass. A chest computerized tomography angiogram demonstrated a descending thoracic aortic aneurysm. Analytical determinations carried out were all negative. The aneurysm was surgically repaired using a Dacron patch. The anatomopathological study described atherosclerotic lesions with calcifications, compatible with an atherosclerotic aneurysm wall. Aneurysms are uncommon in the pediatric population. Usually, no pathogenesis can be determined, and thus, such cases are grouped as idiopathic. Direct repair with or without patch is a therapeutic alternative in pediatric aneurysms and can allow the growth of the aortic circumference.
Nakamura, Yutaro; Suda, Takafumi
Idiopathic pulmonary fibrosis (IPF) is a parenchymal lung disease characterized by progressive interstitial fibrosis. The clinical course of IPF can be unpredictable and may be punctuated by acute exacerbations. Although much progress is being made in unraveling the mechanisms underlying IPF, effective therapy for improving survival remains elusive. Longitudinal disease profiling, especially in terms of clinical manifestations in a large cohort of patients, should lead to proper management of the patients and development of new treatments for IPF. Appropriate multidisciplinary assessment in ongoing registries is required to achieve this. This review summarizes the current status of the diagnosis and clinical manifestations of IPF. PMID:27625576
Singh, Amit Pal; Kumar, Virad; Narula, Vineet; Meher, Ravi; Raj, Anoop
Branchial sinuses are one of the most common congenital anomalies present. They are usually unilateral; bilateral cases are present but are rare. The presentation of bilateral branchial sinus anomalies along with bilateral first arch anomalies is very rare. Here, we present a case of bilateral first arch anomalies co-existing with bilateral second arch anomalies in a patient with no related family history and no associated syndrome.
Wang, Emily; Verhagen Metman, Leo; Bakay, Roy; Arzbaecher, Jean; Bernard, Bryan
Previously, it was found that 16 right-handed patients with idiopathic Parkinsons disease who underwent unilateral implantation of deep brain stimulator in subthalamic nucleus (STN) showed significant improvement in their nonspeech motor functions. Eight of the 16 patients had stimulator in the left STN and eight in the right STN. In contrast, their speech function showed very mild improvement that was limited to the respiratory/phonotory subsystems. Further, there seemed a trend that the patients with right STN stimulation did better than those with left STN stimulation. It was speculated that the difference might be due to a micro lesion caused by the surgical procedure to the corticobulbar fibers run in the left internal capsule. This paper reports speech changes associated with bilateral DBS in STN in four of the 16 subjects who elected to have deep brain stimulator implanted in STN on the opposite side of the brain at a later time. Results show negative changes in speech after bilateral DBS in STN. The changes were not limited to the micro lesion effect due to the surgery itself, but also related to the active stimulation on the dominant hemisphere for speech processing. [Work supported by NIH.
Wang, Emily; Verhagen Metman, Leo; Bakay, Roy; Arzbaecher, Jean; Bernard, Bryan
Previously, it was found that 16 right-handed patients with idiopathic Parkinsons disease who underwent unilateral implantation of deep brain stimulator in subthalamic nucleus (STN) showed significant improvement in their nonspeech motor functions. Eight of the 16 patients had stimulator in the left STN and eight in the right STN. In contrast, their speech function showed very mild improvement that was limited to the respiratory/phonotory subsystems. Further, there seemed a trend that the patients with right STN stimulation did better than those with left STN stimulation. It was speculated that the difference might be due to a micro lesion caused by the surgical procedure to the corticobulbar fibers run in the left internal capsule. This paper reports speech changes associated with bilateral DBS in STN in four of the 16 subjects who elected to have deep brain stimulator implanted in STN on the opposite side of the brain at a later time. Results show negative changes in speech after bilateral DBS in STN. The changes were not limited to the micro lesion effect due to the surgery itself, but also related to the active stimulation on the dominant hemisphere for speech processing. [Work supported by NIH.
Fullagar, Bronwyn A; Rajala-Schultz, Päivi; Hettlich, Bianca F
This retrospective study compared complication rates in 93 client-owned dogs (119 stifles) undergoing single-session bilateral, staged bilateral, or unilateral surgery for bilateral medial patellar luxation. Clinical characteristics and complication rates were compared and risk factors for major complications were explored. Sixty-five dogs had unilateral, 16 staged bilateral and 11 single-session bilateral surgery. Complications occurred in 28/119 stifles (24%), 11 (9%) of which required revision surgery. Patellar reluxation occurred in 7/119 (6%) stifles, with no revision required. There was no significant association between timing of surgery and incidence of complications. In dogs < 10 kg with bilateral medial patellar luxation, single-session bilateral surgery is a feasible treatment option with a complication rate comparable to staged bilateral or unilateral medial patellar luxation surgery.
Fanous, Andrew A.; Olszewski, Nathan P.; Lipinski, Lindsay J.; Qiu, Jingxin
The differential diagnoses for spinal cord lesions include spinal tumors and inflammatory processes. The distinction between these pathologies can be difficult if solely based on imaging. We report for the first time to our knowledge a case of idiopathic transverse myelitis (ITM) mimicking a discrete cervical spinal lesion in a 66-year-old man who presented with gait instability and neck pain. The patient's symptoms failed to resolve after an initial course of steroid therapy. Surgical biopsy confirmed the diagnosis of ITM. Subsequent treatment with dexamethasone resulted in complete resolution of the symptoms as well as the intramedullary enhancement. ITM is most common in the cervical and thoracic spine, spanning 3-4 spinal segments. It usually occupies more than 50% of the cross-sectional area of the spinal cord and tends to be central, uniform, and symmetric. It exhibits patchy and peripheral contrast enhancement. These criteria are useful guidelines that help distinguish ITM from neoplastic spinal lesions. A decision to perform biopsy must take into consideration the patient's clinical symptoms, the rate of progression of neurological deficits, and the imaging characteristics of the lesion. Surgical biopsy for questionable lesions should be reserved for patients with progressive neurological deficits refractory to empirical medical therapy. PMID:27672469
Schmidt, E E; MacDonald, I C; Groom, A C
The spleen plays a central role in the pathogenesis of chronic idiopathic thrombocytopenic purpura (ITP); it produces massive quantities of antiplatelet antibodies, leading to accelerated phagocytosis of platelets. Lymphoid hyperplasia typically occurs in the spleen, characterized by large numbers of lymphatic nodules with active germinal centers. Whether changes in splenic microcirculatory pathways also occur is not known. We have studied this question by scanning electron microscopy of corrosion casts, comparing spleens removed from patients with ITP with normal spleens obtained from organ transplant donors. The casts demonstrate two major changes in microcirculatory pathways in ITP. Firstly, a striking proliferation of arterioles and capillaries is found in the white pulp and marginal zone (MZ), seen as extensive vascularization in 92.3% of lymphatic nodules (n = 191) versus 0.6% (n = 224) in normal spleens. Secondly, the marginal sinus, a series of flattened, anastomosing vascular spaces between the white pulp and MZ, is absent in 89.4% of lymphatic nodules versus 4.9% in normal spleens. The cause of these microcirculatory changes, which may not be exclusive to ITP, is presently unknown. Absence of the marginal sinus may affect distribution of blood flow through the MZ such that platelets spend increased amounts of time in the proximity of macrophages. In the presence of antiplatelet antibodies found in ITP spleens, this delayed transit would lead to greatly increased platelet destruction.
Soo, E; Adamali, H; Edey, A J
Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and is increasingly recognised. Prior to the advent of effective therapies, achieving an early diagnosis was arguably of little prognostic consequence given IPF was considered an untreatable and uniformly fatal disease. The advent of new drug treatments has given hope for the future and raised the profile of IPF. International management guidelines highlight the critical role of radiology as part of an interstitial lung disease multidisciplinary team approach in reaching an accurate and early diagnosis of IPF. The diagnostic criteria and levels of diagnostic confidence for the radio-pathological pattern associated with the clinical syndrome of IPF, usual interstitial pneumonia (UIP), appear seemingly straightforward; however, with increasing research and recognition of radiopathological interobserver variability, limitations of this classification model are becoming increasingly apparent. This review describes ancillary radiological features, comorbidities, and emerging new entities that potentially co-exist with IPF. Beyond diagnosis radiology is developing as a key prognostic tool to inform longitudinal patient evaluation. These diagnostic and prognostic clinical challenges and the future role of radiology in IPF are discussed.
Makker, Sudesh P.; Tramontano, Alfonso
Summary For more than 50 years researchers have debated the evidence for an autoimmune basis of human idiopathic membranous nephritis (MN). Work published in the past 2 years has substantially strengthened the belief that MN is indeed an autoimmune disease of the kidney. Autoantibodies of the IgG4 subclass to at least three podocyte membrane proteins including phospholipase A2-receptor, aldose reductase, and manganese superoxide dismutase have been detected by immunoblotting in sera as well as in acid eluates prepared from renal biopsy tissue of patients with this disease, using either whole tissue or microdissected glomeruli from frozen sections. In each case the podocyte antigen has been shown to co-localize with the subepithelial glomerular immune deposits in renal tissue of the same patients. It is not certain if any of these podocyte proteins is an inciting/primary autoantigen or whether they are secondary antigens recruited by intermolecular epitope-spreading, initiating from a yet-to-be-discovered autoantigen. Although it is clear that autoantibodies to podocyte membrane proteins are elicited in idiopathic MN and contribute to the formation of the subepithelial deposits, many questions remain concerning the triggers for their development and their contribution toward proteinuria and progression of the disease. PMID:21839366
Makker, Sudesh P; Tramontano, Alfonso
For more than 50 years researchers have debated the evidence for an autoimmune basis of human idiopathic membranous nephritis (MN). Work published in the past 2 years has substantially strengthened the belief that MN is indeed an autoimmune disease of the kidney. Autoantibodies of the IgG4 subclass to at least three podocyte membrane proteins including phospholipase A(2)-receptor, aldose reductase, and manganese superoxide dismutase have been detected by immunoblotting in sera as well as in acid eluates prepared from renal biopsy tissue of patients with this disease, using either whole tissue or microdissected glomeruli from frozen sections. In each case the podocyte antigen has been shown to co-localize with the subepithelial glomerular immune deposits in renal tissue of the same patients. It is not certain if any of these podocyte proteins is an inciting/primary autoantigen or whether they are secondary antigens recruited by intermolecular epitope-spreading, initiating from a yet-to-be-discovered autoantigen. Although it is clear that autoantibodies to podocyte membrane proteins are elicited in idiopathic MN and contribute to the formation of the subepithelial deposits, many questions remain concerning the triggers for their development and their contribution toward proteinuria and progression of the disease.
Taniguchi, Hiroyuki; Kondoh, Yasuhiro
Idiopathic interstitial pneumonias (IIPs) may have an acute or subacute presentation, or acute exacerbation may occur in a previously subclinical or unrecognized chronic IIP. Acute or subacute IIPs include acute interstitial pneumonia (AIP), cryptogenic organizing pneumonia (COP), nonspecific interstitial pneumonia (NSIP), acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) and AE-NSIP. Interstitial lung diseases (ILDs) including connective tissue disease (CTD) associated ILD, hypersensitivity pneumonitis, acute eosinophilic pneumonia, drug-induced lung disease and diffuse alveolar haemorrhage need to be differentiated from acute and subacute IIPs. Despite the severe lack of randomized controlled trials for the treatment of acute and subacute IIPs, the mainstream treatment remains corticosteroid therapy. Other potential therapies reported in the literature include corticosteroids and immunosuppression, antibiotics, anticoagulants, neutrophil elastase inhibitor, autoantibody-targeted treatment, antifibrotics and hemoperfusion therapy. With regard to mechanical ventilation, patients in recent studies with acute and subacute IIPs have shown better survival than those in previous studies. Therefore, a careful value-laden decision about the indications for endotracheal intubation should be made for each patient. Noninvasive ventilation may be beneficial to reduce ventilator associated pneumonia.
Lam, J; Castillo, O; Bravo, J; Henríquez, R; Tagle, F
Laparoscopic adrenalectomy, if done by skilled surgeons, is now the first choice for treating most adrenal tumors, including bilateral pheochromocytoma. We report two women, aged 35 and 34 years old, with bilateral adrenal pheochromocytoma successfully excised by laparoscopic surgery. Both had severe hypertension, high urinary catecholamine values (epinephrine + norepinephrine: 528 and 1083 ug/24 h) and bilateral adrenal tumors at CT scan. After 4 weeks of doxazosin treatment, a laparoscopic transperitoneal adrenalectomy was done (Gugner's technique), with surgical times of 7 and 5 hours respectively. Both patients received hydrocortisone and only the second one required one unit of packed cells. Postoperative evolution was uneventful and both patients were discharged at the fifth postoperative day. At two months of follow up, both patients are asymptomatic and normotensive.
Stott, M. A.; Sutton, R.; Royle, G. T.
Two hundred and forty four patients underwent either simultaneous bilateral inguinal hernia repair (n = 122) or unilateral inguinal hernia (n = 122) repair at a general hospital between January 1971 and December 1981. The two groups of patients were matched for age and sex. Both groups had a similar overall incidence of post-operative complications and in both groups the duration of post-operative stay and duration of operating time were similar. Chest infections developed in 12 patients after bilateral repair and in 3 patients after unilateral repair (P less than 0.02). All patients were assessed prospectively from 4 to 15 years after operation, when no significant difference in the number of recurrent hernias was found. Our results suggest that simultaneous bilateral inguinal herniorrhaphy is economical in terms of both operating time and duration of hospital stay, and that this economy is not bought at a cost of increased short term morbidity or long-term recurrence rate. PMID:3200778
McPhee, Kelly C; Denk, Christian; Al-Rekabi, Zeinab; Rauscher, Alexander
High-pass filtering is required for the removal of background field inhomogeneities in magnetic resonance phase images. This high-pass filtering smooths across boundaries between areas with large differences in phase. The most prominent boundary is the surface of the brain where areas with large phase values inside the brain are located close to areas outside the brain where the phase is, on average, zero. Cortical areas, which are of great interest in brain MRI, are therefore often degraded by high-pass filtering. Here, we propose the use of the bilateral filter for the high-pass filtering step. The bilateral filter is essentially a Gaussian filter that stops smoothing at boundaries. We show that the bilateral filter improves image quality at the brain's surface, without sacrificing contrast within the brain.
Klauss, V; Riedel, K
We report on 2 infants, one with a bilateral and the other with a unilateral corneal metaplasia. The first case with bilateral corneal metaplasia showed shortening of both upper and lower lids with formation of symblephara. By ultrasonography the right eye presented with microphthalmos, aphakia, and persistent hyaloid, whereas the inner parts of the left eye appeared to be normal. The question remains to be answered whether this is an abortive cryptophthalmos leading to bilateral corneal metaplasia or a primary corneal metaplasia inhibiting the lid growth. No suggestions concerning the aetiology are made. The second case presented with a unilateral corneal metaplasia, normal eye lids, aphakia, and microphthalmos. This aberration was probably caused by an amniotic band, as it is associated with malformation of the nose on the same side. In case 2 the dermoid was excised and a lamellar corneal graft performed. The histology is reported. Images PMID:6838805
Fujimoto, Yasunori; Ohnishi, Yu-ichiro; Wakayama, Akatsuki; Yoshimine, Toshiki
Locked-in syndrome (LIS) usually occurs as a result of pontine lesions and has been classified into various categories on the basis of neurologic conditions, of which transient total mesencephalic LIS is extremely rare. A 53-year-old man presented with bilateral ptosis followed by a total locked-in state. In the clinical course, the patient successfully recovered with only left slight hemiparesis and skew deviation remaining. Magnetic resonance imaging revealed multiple ischemic lesions caused by thrombosis at the top of basilar artery, including the bilateral cerebral peduncles, tegmentum of the midbrain, and the right cerebellar hemisphere. Antecedent bilateral ptosis before the locked-in state may be related to ischemia in the central caudal nucleus of the oculomotor nuclei. We should pay attention to this easily missed condition during the treatment of ischemic stroke involving the basilar artery.
King, Talmadge E.
The idiopathic interstitial pneumonias are a heterogeneous group of poorly understood diseases with often devastating consequences for those afflicted. Subclassification of the idiopathic interstitial pneumonia based on clinical-radiological-pathological criteria has highlighted important pathogenic, therapeutic and prognostic implications. The most critical distinction is the presence of usual interstitial pneumonia, the histopathological pattern seen in idiopathic pulmonary fibrosis. Idiopathic pulmonary fibrosis has a worse response to therapy and prognosis. New insight into the pathophysiology of usual interstitial pneumonia suggests a distinctly fibroproliferative process, and antifibrotic therapies show promise. While the clinical and radiographic diagnosis of idiopathic interstitial pneumonias can be made confidently in some cases, many patients require surgical lung biopsy to determine their underlying histopathology. A structured, clinical-radiological-pathological approach to the diagnosis of the idiopathic interstitial pneumonias, with particular attention to the identification of idiopathic pulmonary fibrosis, insures proper therapy, enhances prognostication, and allows for further investigation of therapies aimed at distinct pathophysiology. Images Fig. 2 Fig. 4 Fig. 5 Fig. 6 PMID:17060957
Sony, Parul; Venkatesh, Pradeep; Tewari, Hem Kumar; Garg, Sat Pal
A 28-year-old man presented with bilateral acute loss of vision following a high-voltage injury. The visual acuity was 6/36 in the right eye and 6/24 in the left eye. Fundus examination revealed a well-defined round lesion simulating a full thickness macular hole in the right eye and yellow deposits in the macular area in the left eye. Optical coherence tomography (OCT) showed bilateral macular cysts, with intact outer and inner retinal layers. At 1 month follow up, OCT showed a persistent macular cyst in the right eye with spontaneous resolution of the macular cyst along with visual improvement in the left eye.
Pandey, Sharad; Sharma, Vivek; Shinde, Neeraj; Sharma, Mukesh
Extradural hematoma (EDH) occurs in approximately 2% of all patients with head injuries. Bilateral EDHs account for 2–10% of all acute EDHs in adults but are exceedingly rare in children. Posterior fossa EDHs occurs in 5% of all cases of EDHs. EDHs in children are more frequently venous (from tears of a dural sinus or diploic veins) and consequently have a better prognosis than EDHs in adults. Once the diagnosis of BEH is confirmed, urgent surgical treatment should be considered. We are reporting such rare form of injury as bilateral occipital EDH with supratentorial extension in 12 years child following road traffic accident. PMID:26557174
Burghardt, Rolf D.; Yoshino, Koichi; Kashiwagi, Naoya; Yoshino, Shigeo; Bhave, Anil; Paley, Dror; Herzenberg, John E.
Purpose Outcome assessment after double level tibial lengthening in patients with dwarfism. Methods Fourteen patients with dwarfism were analyzed after bilateral simultaneous double level tibial lengthening. Results Average age was 15.1 years. Average lengthening was 13.5 cm. The two levels were lengthened by an average of 7.5 cm proximally and 6.0 cm distally. Concomitant deformities were also addressed during lengthening. External fixation treatment time averaged 8.8 months. Healing index averaged 0.7 months/cm. Conclusion Bilateral tibial lengthening for dwarfism is difficult, but the results are usually quite gratifying. PMID:26566326
Gültaşli, N; van den Hauwe, L; Bruneau, M; D'Haene, N; Delpierre, I; Balériaux, D
Primary solitary amyloidoma of Meckel's cave is rare, and a bilateral location is even more rare. To the best of our knowledge, only 12 cases in the literature have described such a primary lesion, including one case of bilateral involvement of Meckel's cave. We report here on the case of a 57-year-old woman presenting with pseudotumor masses involving both Meckel's caves and responsible for trigeminal neuropathy. The final diagnosis of amyloidoma was made on the basis of histological examination of surgical biopsy specimens.
Mahaudens, P; Banse, X; Mousny, M; Detrembleur, C
Adolescent idiopathic scoliosis (AIS) is a progressive growth disease that affects spinal anatomy, mobility, and left-right trunk symmetry. Consequently, AIS can modify human locomotion. Very few studies have investigated a simple activity like walking in a cohort of well-defined untreated patients with scoliosis. The first goal of this study is to evaluate the effects of scoliosis and scoliosis severity on kinematic and electromyographic (EMG) gait variables compared to an able-bodied population. The second goal is to look for any asymmetry in these parameters during walking. Thirteen healthy girls and 41 females with untreated AIS, with left thoracolumbar or lumbar primary structural curves were assessed. AIS patients were divided into three clinical subgroups (group 1 < 20 degrees, group 2 between 20 and 40 degrees, and group 3 > 40 degrees). Gait analysis included synchronous bilateral kinematic and EMG measurements. The subjects walked on a treadmill at 4 km/h (comfortable speed). The tridimensional (3D) shoulder, pelvis, and lower limb motions were measured using 22 reflective markers tracked by four infrared cameras. The EMG timing activity was measured using bipolar surface electrodes on quadratus lumborum, erector spinae, gluteus medius, rectus femoris, semitendinosus, tibialis anterior, and gastrocnemius muscles. Statistical comparisons (ANOVA) were performed across groups and sides for kinematic and EMG parameters. The step length was reduced in AIS compared to normal subjects (7% less). Frontal shoulder, pelvis, and hip motion and transversal hip motion were reduced in scoliosis patients (respectively, 21, 27, 28, and 22% less). The EMG recording during walking showed that the quadratus lumborum, erector spinae, gluteus medius, and semitendinosus muscles contracted during a longer part of the stride in scoliotic patients (46% of the stride) compared with normal subjects (35% of the stride). There was no significant difference between scoliosis groups 1
Banse, X.; Mousny, M.; Detrembleur, C.
Adolescent idiopathic scoliosis (AIS) is a progressive growth disease that affects spinal anatomy, mobility, and left-right trunk symmetry. Consequently, AIS can modify human locomotion. Very few studies have investigated a simple activity like walking in a cohort of well-defined untreated patients with scoliosis. The first goal of this study is to evaluate the effects of scoliosis and scoliosis severity on kinematic and electromyographic (EMG) gait variables compared to an able-bodied population. The second goal is to look for any asymmetry in these parameters during walking. Thirteen healthy girls and 41 females with untreated AIS, with left thoracolumbar or lumbar primary structural curves were assessed. AIS patients were divided into three clinical subgroups (group 1 < 20°, group 2 between 20 and 40°, and group 3 > 40°). Gait analysis included synchronous bilateral kinematic and EMG measurements. The subjects walked on a treadmill at 4 km/h (comfortable speed). The tridimensional (3D) shoulder, pelvis, and lower limb motions were measured using 22 reflective markers tracked by four infrared cameras. The EMG timing activity was measured using bipolar surface electrodes on quadratus lumborum, erector spinae, gluteus medius, rectus femoris, semitendinosus, tibialis anterior, and gastrocnemius muscles. Statistical comparisons (ANOVA) were performed across groups and sides for kinematic and EMG parameters. The step length was reduced in AIS compared to normal subjects (7% less). Frontal shoulder, pelvis, and hip motion and transversal hip motion were reduced in scoliosis patients (respectively, 21, 27, 28, and 22% less). The EMG recording during walking showed that the quadratus lumborum, erector spinae, gluteus medius, and semitendinosus muscles contracted during a longer part of the stride in scoliotic patients (46% of the stride) compared with normal subjects (35% of the stride). There was no significant difference between scoliosis groups 1, 2, and 3 for
Yoritaka, Asako; Hishima, Tsunekazu; Akagi, Kumiko; Kishida, Shuji
The pathogenesis of idiopathic-acquired hypohidrosis remains unknown, and no specific causes have yet been established. We report a 34-year-old man with acquired idiopathic hypohidrosis successfully treated with prednisolone. The patient noticed heat intolerance and hypohidrosis of the pectoral and back during the summer. No systemic disease or neurological findings were identified. Eccrine sweat glands displayed infiltration by inflammatory cells, with immunoglobulin G and C3 deposition in the basement membrane. Steroid therapy improved the hypohidrosis. An immunological pathogenesis could be a major factor in idiopathic-acquired hypohidrosis.
Ryu, Jay H.; Moua, Teng; Azadeh, Natalya; Baqir, Misbah; Yi, Eunhee S.
Idiopathic interstitial pneumonias comprise approximately one-third of interstitial lung diseases (also called diffuse parenchymal infiltrative lung diseases). The classification of idiopathic interstitial pneumonias has undergone several revisions since the initial description of 40 years ago, and the most recent version was published in 2013. Although some aspects have been clarified, this group of heterogeneous disorders continues to be a source of confusion and misunderstanding in clinical applications. In this article, we explore several topical themes in the evaluation and management of patients with idiopathic interstitial pneumonias. PMID:27853529
Gambaro, Giovanni; Abaterusso, Cataldo
Idiopathic calcium nephrolithiasis and hypercalciuria are multifactorial disease conditions, the pathogenesis of which involves the interaction of environmental and individual factors. Data support a strong role of genes in the pathogenesis of these two conditions. Findings obtained in monogenic disorders characterized by renal calcium stones, and/or hypercalciuria, and/or nephrocalcinosis have proposed a number of genes as candidate genes in the pathogenesis of the common idiopathic calcium nephrolithiasis and hypercalciuria. The physiological role of these genes, and findings in monogenic disorders and idiopathic, multifactorial disorders will be presented.
Ijaz, Mohsin; Sakam, Sailaja; Ashraf, Umair; Marquez, Jose Gomez
Patient: Male, 47 Final Diagnosis: Bilateral psoas abscess • acute lower extremity deep vein thrombosis • bilateral pulmonary embolism Symptoms: Progressive left leg swelling • productive cough with whitish sputum • right flank pain Medication: Antibiotics and anticoagulation Clinical Procedure: CT-guided percutaneous drain placement Specialty: Internal Medicine/Critical Care Objective: Unusual presentation Background: Psoas abscesses are a known cause of back pain, but they have not been reported as a cause of acute lower extremity thromboses and bilateral pulmonary emboli. We report a patient with bilateral psoas abscesses causing extensive pulmonary emboli through compression of the iliac vein. Case Report: A 47-year-old man presented with bilateral leg swelling over 4 weeks. Physical examination revealed a thin male with bilateral leg swelling, extending to the thigh on his left side. He had hemoglobin of 10.5 g/dl, leukocytosis of 16 000/ml, and an elevated D-dimer. A computed tomography (CT) angiogram of his chest showed extensive bilateral pulmonary emboli and infarcts. He remained febrile with vague flank pain, prompting a CT of his abdomen and pelvis that showed large, multiloculated, septated, bilateral psoas abscesses with compression of the left femoral vein by the left psoas abscess and a thrombus distal to the occlusion. Two liters of pus was drained from the left psoas abscess by CT-guidance, and although the Gram staining showed Gram-positive cocci in clusters, cultures from the abscess and blood were negative. A repeat CT showed resolution of the abscesses, and the drain was removed. He was discharged to a nursing home to complete a course of intravenous antibiotics and anticoagulation. Conclusions: Although the infectious complications of psoas abscesses have been described in the literature, the mechanical complications of bilateral psoas abscesses are lacking. It is important to assess for complete resolution of psoas abscesses through
Wall, Michael; Kupersmith, Mark J.; Kieburtz, Karl D.; Corbett, James J.; Feldon, Steven E.; Friedman, Deborah I.; Katz, David M.; Keltner, John L.; Schron, Eleanor B.; McDermott, Michael P.
IMPORTANCE To our knowledge, there are no large prospective cohorts of untreated patients with idiopathic intracranial hypertension (IIH) to characterize the disease. OBJECTIVE To report the baseline clinical and laboratory features of patients enrolled in the Idiopathic Intracranial Hypertension Treatment Trial. DESIGN, SETTING, AND PARTICIPANTS We collected data at baseline from questionnaires, examinations, automated perimetry, and fundus photography grading. Patients (n = 165) were enrolled from March 17, 2010, to November 27, 2012, at 38 academic and private practice sites in North America. All participants met the modified Dandy criteria for IIH and had a perimetric mean deviation between −2 dB and −7 dB. All but 4 participants were women. MAIN OUTCOMES AND MEASURES Baseline and laboratory characteristics. RESULTS The mean (SD) age of our patients was 29.0 (7.4) years and 4 (2.4%) were men. The average (SD) body mass index (calculated as weight in kilograms divided by height in meters squared) was 39.9 (8.3). Headache was the most common symptom (84%). Transient visual obscurations occurred in 68% of patients, back pain in 53%, and pulse synchronous tinnitus in 52%. Only 32% reported visual loss. The average (SD) perimetric mean deviation in the worst eye was −3.5 (1.1) dB, (range, −2.0 to −6.4 dB) and in the best eye was −2.3 (1.1) dB (range, −5.2 to 0.8 dB). A partial arcuate visual field defect with an enlarged blind spot was the most common perimetric finding. Visual acuity was 85 letters or better (20/20) in 71% of the worst eyes and 77% of the best eyes. Quality of life measures, including the National Eye Institute Visual Function Questionnaire–25 and the Short Form–36 physical and mental health summary scales, were lower compared with population norms. CONCLUSIONS AND RELEVANCE The Idiopathic Intracranial Hypertension Treatment Trial represents the largest prospectively analyzed cohort of untreated patients with IIH. Our data show
Lee, Young Sil; Moon, Seong Su
Hypothyroidism can cause a variety of signs and symptoms of the neuromuscular system. However, ptosis in a patient with hypothyroidism is very rare. We report here on a case of central hypothyroidism that was due to Sheehan's syndrome and it manifested as bilateral ptosis in a 51-yr-old woman. She complained of exertional dyspnea and weakness. About 25-yr ago, she had a history of severe postpartum vaginal bleeding. The laboratory studies demonstrated hypopituitarism with secondary hypothyroidism. The ptosis was improved by replacement of thyroid hormone. Hypothyroidism should be considered in the differential diagnosis of patients who manifest with ptosis and that prompt replacement of hormone can lead to a complete recovery.
Lang, C; Lehrl, S; Huk, W
A 76-year-old man with bilateral temporal lobe agenesis producing clinical features resembling the Robinson syndrome is described. The malformation was discovered during a routine CT examination after the appearance of a homonymous visual field defect. The patient was examined by (neuro) psychological testing. The findings are compared with other reported cases and discussed with regard to cerebral localisation. Images PMID:7288451
Jadhav, Ashutosh P.; Ducruet, Andrew F.; Jankowitz, Brian T.; Jovin, Tudor G.
Background Symptomatic bilateral internal carotid occlusive disease is a rare but potentially devastating entity. Medical therapy alone is associated with high rates of mortality and recurrent stroke. The optimal management of this disease remains poorly understood. Methods A retrospective review of a prospectively maintained database was conducted for patients who presented with an acute stroke in the setting of bilateral carotid occlusive disease between May and October 2013. Results We identified 3 patients. The admission National Institutes of Health Stroke Scale score ranged from 4 to 7. All patients had small- to moderate-sized infarcts in the anterior circulation on presentation. Angiography confirmed bilateral internal carotid occlusions with collateral filling via the posterior communicating artery and retrograde filling via external carotid artery supply to the ophthalmic artery. All patients were initially managed with permissive hypertension and anticoagulation followed by carotid angioplasty and stenting. At 1-year follow-up, all patients demonstrated a modified Rankin scale score of 0-1. Conclusions Carotid stenting may be a safe and effective therapy for patients presenting with symptomatic bilateral carotid occlusions. PMID:27051405
Xie, Jun; Ann Heng, Pheng
In this paper, we propose an approach to diffuse color images based on the bilateral filter. Real image data has a level of uncertainty that is manifested in the variability of measures assigned to pixels. This uncertainty is usually interpreted as noise and considered an undesirable component of the image data. Image diffusion can smooth away small-scale structures and noise while retaining important features, thus improving the performances for many image processing algorithms such as image compression, segmentation and recognition. The bilateral filter is noniterative, simple and fast. It has been shown to give similar and possibly better filtering results than iterative approaches. However, the performance of this filter is greatly affected by the choose of the parameters of filtering kernels. In order to remove noise and maintain the significant features on images, we extend the bilateral filter by introducing an adaptive domain spread into the nonlinear diffusion scheme. For color images, we employ the CIE-Lab color system to describe input images and the filtering process is operated using three channels together. Our analysis shows that the proposed method is more suitable for preserving strong edges on noisy images than the original bilateral filter. Empirical results on both nature images and color medical images confirm the novel method's advantages, and show it can diffuse various kinds of color images correctly and efficiently.
Raj, K Mohan; Reddy, P Arun Subhash; Kumar, Vikram Chella
Angle closure glaucoma is one of the ophthalmic emergencies and treatment has to be given at the earliest. It is a rare complication of general anesthesia. A female patient underwent Hysterectomy under general anesthesia. Following this, patient developed bilateral angle closure glaucoma. This patient was treated with antiglaucoma medications followed by YAG laser iridotomy and patient regained vision.
Milla, E; Zografos, L; Piguet, B
Mycoplasma pneumoniae is an atypical bacterium that can cause a great variety of respiratory infections and be responsible for ocular involvement such as conjunctivitis, anterior uveitis and very rarely optic neuropathy. We report herein an additional case of bilateral optic disc swelling with profound visual loss following Mycoplasma pneumoniae pneumonia and review the world literature on the ocular manifestations associated with this pathogen.
Murayama, K; Adachi-Usami, E
Two siblings with Leber's congenital amaurosis had bilateral macular colobomas, nystagmus, extinguished ERGs, and degenerative salt and pepper like changes in the fundus. They had non-recordable or non-meaningful visually evoked cortical potentials in response to both flash and pattern stimuli. The ophthalmic conditions were thought to be inherited as an autosomal recessive trait.
... disabilities of the right and left sides will be combined as usual, and 10 percent of this value will be added... extremities as a whole. Thus with a compensable disability of the right thigh, for example, amputation, and one of the left foot, for example, pes planus, the bilateral factor applies, and similarly...
... disabilities of the right and left sides will be combined as usual, and 10 percent of this value will be added... extremities as a whole. Thus with a compensable disability of the right thigh, for example, amputation, and one of the left foot, for example, pes planus, the bilateral factor applies, and similarly...
... or injury of both arms, or of both legs, or of paired skeletal muscles, the ratings for the... arm, forearm and hand, or the thigh, leg, and foot, but relates to the upper extremities and lower... one of the left foot, for example, pes planus, the bilateral factor applies, and similarly...
... or injury of both arms, or of both legs, or of paired skeletal muscles, the ratings for the... arm, forearm and hand, or the thigh, leg, and foot, but relates to the upper extremities and lower... one of the left foot, for example, pes planus, the bilateral factor applies, and similarly...
Rubio Hernández, María Caridad; Díaz Prado, Yenia Ivet; Pérez, Suanly Rodríguez; Díaz, Ronald Rodríguez; Aleaga, Zaili Gutiérrez
Male breast cancer, which represents only 1% of all breast cancers, is occasionally associated with a family history of breast cancer. Sporadic male breast cancers presenting with another primary breast cancer are extremely rare. In this article, we report on a 70-year-old male patient with bilateral multifocal and synchronous breast cancer and without a family history of breast cancer. PMID:24319497
Yamanaka, Toshiaki; Sawai, Yachiyo; Hosoi, Hiroshi
Subclavian steal syndrome (SSS) is usually caused by unilateral subclavian artery (SA) occlusion, and bilateral SSS is very rare. Takayasu's arteritis (TA) is a chronic granulomatous form of vasculitis that affects the SA, most commonly in women aged 15-40 years. We report a rare case of bilateral SSS due to TA in a 52-year-old woman, who exhibited severe vertigo. Although her blood pressure was within the normal range and did not differ between her arms, Doppler ultrasonography revealed low antegrade blood flow in the right SA and retrograde flow in the left SA. Computed tomography angiography demonstrated complete obstruction of the bilateral SA proximal to the vertebral artery origin. The more marked decrease in the blood flow of the vertebrobasilar artery experienced in bilateral SSS compared with unilateral SSS is considered to have caused the severe rotatory vertigo in the present patient. Since the vascular conditions of TA progressively deteriorate, delayed diagnosis and treatment could result in poor outcomes and unfavorable prognosis. We suggest that SSS with TA might require an early detection and treatment as well as careful follow-up for preventing vertigo and other neurological deficits in the vertebrobasilar arterial region.
García, Elena; Sánchez, Raquel; Martínez, Guillermo; Bernal, Carmen; Calatayud, M; Partida, M; Hawkins, Federico
Many problems may arise when defining whether adrenal lesions are primary to the adrenal glands or represent other tissue, whether they are benign or malignant and whether they are functioning or nonfunctioning. Adrenal imaging complements the clinical and hormonal evaluation of these patients. We present a patient with lumbar pain and bilateral adrenal masses.
Yakubov, Yakov; Mandel, Louis
Polycystic ovarian syndrome (PCOS) is recognized by the presence of polycystic ovaries, irregular menstruation, and increased androgen levels. Many patients have insulin resistance or impaired glucose tolerance and an associated development of type 2 diabetes mellitus. A patient with PCOS is presented whose cosmetic concerns centered on the prolonged existence of substantial bilateral parotid swelling. The pathophysiology, diagnosis, and therapy of sialosis are discussed.
Wilms, G.E.; Baert, A.L.; Kint, E.J.; Pringot, J.H.; Goddeeris, P.G.
The computed tomographic (CT) features of bilateral adrenal tuberculosis are reported in two cases that demonstrate two typical different clinical and morphological manifestations of the disease. The incidence and CT appearance of adrenal tuberculosis are discussed, with emphasis on differential diagnosis.
Hyman, Charles H; Cohen, Philip R
Knuckle pads are a rare, frequently overlooked, thickening of the skin usually overlying the extensor surface of the proximal interphalangeal joints. They are well- circumscribed, benign lesions that generally do not require treatment. Idiopathic knuckle pads must be differentiated from similar appearing lesions or trauma-induced pseudo-knuckle pads. Knuckle pads have been observed in association with autosomal dominant conditions such as Bart-Pumphrey syndrome, Dupuytren's contracture, Ledderhose disease, and Peyronie's disease. To the best of our knowledge, idiopathic familial knuckle pads have not previously been described in the English language literature. We describe a sister and brother with familial idiopathic knuckle pads with no associated conditions.
Barut, Kenan; Sahin, Sezgin; Adrovic, Amra
Macrophage activation syndrome, a severe complication of systemic juvenile idiopathic arthritis and other inflammatory diseases, represents one of the most important rheumatological emergencies. Delayed diagnosis could lead to life-threatening complications. Pulmonary hemosiderosis has been classically characterized by a triad of anemia, hemoptysis, and lung infiltrates on chest radiogram. Although the majority of patients of pulmonary hemosiderosis are considered idiopathic, secondary hemosiderosis associated with known diseases could be seen. In this case report, we aimed to present gradually increased pulmonary manifestations due to pulmonary hemosiderosis with recurrent macrophage activation syndrome attacks in a child with systemic juvenile idiopathic arthritis. PMID:28251009
Rivera, Cathleen M.; Grossardt, Brandon R.; Rhodes, Deborah J.; Brown, Robert D.; Roger, Véronique L.; Melton, L. Joseph; Rocca, Walter A.
Objective To investigate the mortality associated with cardiovascular diseases and the effect of estrogen treatment in women who underwent unilateral or bilateral oophorectomy before menopause. Design We conducted a cohort study with long-term follow-up of women in Olmsted County, MN, who underwent either unilateral or bilateral oophorectomy before the onset of menopause from 1950 through 1987. Each member of the oophorectomy cohort was matched by age to a referent woman from the same population who had not undergone any oophorectomy. We studied the mortality associated with cardiovascular disease in a total of 1,274 women with unilateral oophorectomy, 1,091 women with bilateral oophorectomy, and 2,383 referent women. Results Women who underwent unilateral oophorectomy experienced a reduced mortality associated with cardiovascular disease compared with referent women (hazard ratio [HR], 0.82; 95% confidence interval [CI], 0.67–0.99; P = 0.04). By contrast, women who underwent bilateral oophorectomy before age 45 years experienced an increased mortality associated with cardiovascular disease compared with referent women (HR, 1.44; 95% CI, 1.01–2.05; P = 0.04). Within this age stratum, the HR for mortality was significantly elevated in women who were not treated with estrogen through age 45 years or longer (HR, 1.84; 95% CI, 1.27–2.68; P = 0.001) but not in women treated (HR, 0.65; 95% CI, 0.30–1.41; P = 0.28; test of interaction, P = 0.01). Mortality was further increased after excluding deaths associated with cerebrovascular causes. Conclusions Bilateral oophorectomy performed before age 45 years is associated with increased cardiovascular mortality, especially with cardiac mortality. However, estrogen treatment may reduce this risk. PMID:19034050
Idiopathic pulmonary hemosiderosis is a rare disorder that can occur at any age and is characterized by the triad of hemoptysis, iron deficiency anemia and diffuse pulmonary infiltrates. The clinical course is exceedingly variable especially in children and a substantial proportion of this age group is undiagnosed. It is probably due to the fact that iron deficiency anemia may be the first and the only manifestation of IPH, preceding other symptoms and signs by several months and IPH is not considered as a rare cause of anemia, unless the typical triad is present. We present a case of IPH in a 13-year-old girl, treated for several months with persistent iron deficiency anemia, without response to therapy. PMID:24708654
Abraham, Bincy P; Sellin, Joseph H
The aetiology of diarrhoea can often be simple to identify, but in some cases may pose a challenge. The diagnosis of drug-induced diarrhoea can easily be sorted based on timing of the symptom with onset of a new drug. Treatment can vary from simply monitoring and eventual resolution with continuation of the drug, to discontinuation of the offending agent. In cases where a drug cannot always be stopped, additional medications can help control the symptom. Factitious diarrhoea can present a diagnostic challenge if the evaluating physician does not suspect its possibility. Typically a careful history, and in some cases, stool testing can provide clues. The diagnosis of idiopathic diarrhoea is often made when exhaustive testing provides no definite aetiology and the goal of management is supportive care and symptomatic treatment.
Johansson, H; Thorén, L; Werner, I; Grimelius, L
Eighty-four patients with recurrent kidney stones, serum calcium levels in the upper normal quartile, and most of whom with hypercalciuria had their parathyroids surgically explored. Parathyroid adenomata were found in 19 patients, hyperplasia in 39, and normal parathyroids in 26. Postoperatively there was a significant fall in serum calcium and urinary calcium excretion in all three groups. At clinical follow-up 2 to 5 years postoperatively there was no case of kidney stone recurrence among the adenoma patients. In the hyerplasia group there were recurrences tn 25 percent. The corresponding figure for the patients with normal parathyroids was 48 percent. The concept of normocalcemic primary hyperparathyroidism and the relationship between this syndrome and idiopathic hypercalciuria are discussed. Some prinicpal therapeutic measures are recommended.
Park, S. H.; Whang, C. J.; Sohn, M.; Oh, Y. C.; Lee, C. H.; Whang, Y. J.
Idiopathic hypertrophic pachymeningitis (IHP) is a rare, chronic nonspecific and granulomatous inflammatory disorder of the dura with unknown etiology. The diagnosis can be established by open biopsy and exclusion of all other specific granulomatous and infectious diseases. We report a typical case of spinal IHP occurring in a long segment of cervical and thoracic dura from C6 to T8. The patient was 56-yr-old female, who had been suffered from pain on her upper back and both arms for 3 months and recent onset motor weakness of both legs. During the 9 months of follow-up period, she experienced the improvement of her neurologic symptoms with combined therapy of partial excision and corticosteroid medication. Since early surgical intervention and subsequent pulse steroid therapy are mandatory for this disease to avoid irreversible damage of nervous system, the identification of this unique disease entity is essential on frozen diagnosis. A few cases have been reported in Korean literature. PMID:11641545
Arellano-Rodrigo, Eduardo; Esteve, Jordi; Giné, Eva; Panés, Julián; Cervantes, Francisco
A patient with ulcerative colitis (UC) who developed idiopathic myelofibrosis (IM) is reported. The initial diagnosis of UC was established by colonoscopy and large bowel biopsy, performed after a one-month history of abdominal pain and bloody diarrhea. The patient showed a favorable response to prednisone and mesalamine treatment and six months later he developed a new episode of UC, which was successfully controlled with treatment. However, two years later splenomegaly and anemia were observed, with aniso-poikilocytosis, tear-drop cells, immature myeloid precursors in the peripheral blood, and increased serum LDH, arising the suspicion of IM, a diagnosis that was confirmed by bone marrow biopsy. The present case represents a new association of IM with an autoimmune disease and gives support to the hypothesis of a possible immune basis of some IM cases.
Hernández-Boluda, J C; Jiménez, M; Rosiñol, L; Cervantes, F
A patient with primary biliary cirrhosis (PBC) who developed idiopathic myelofibrosis (IM) is reported. The initial diagnosis of PBC was established by liver biopsy, performed after a 2-month history of constitutional symptoms associated with abnormalities of the serum liver enzymes, with typical serum immunological markers being found. Although a favorable response of PBC to prednisone was observed, one and a half year later the patient developed anemia with anisocytosis and poikilocytosis, tear-drop cells, and leukoerythroblastic picture, and IM was diagnosed by bone marrow biopsy. A few months later, a rapid worsening of the patient's clinical condition was noted, with an increase in the constitutional symptoms and need for frequent packed RBC transfusions, and she finally died from an infectious complication. This case represents a new association of IM with an autoimmune disease, supporting the hypothesis of a possible immune basis of IM in some cases.
Tampakoudis, P; Billi, H; Tantanassis, T; Kalachanis, I; Garipidou, B; Sinakos, Z; Mantalenakis, S
Clinical data from eight pregnant women with idiopathic thrombocytopenic purpura (ITP) were retrospectively analyses. The mean age of the women was 28.2 years. Five women underwent splenectomy during childhood. The lowest maternal platelet count observed ranged from 8000 to 88000/mm3. Genital bleeding occurred in only one case. Treatment was based on administration of corticosteroids with or without human-pooled immunoglobulins. Caesarian section was performed in all cases. Six newborns were healthy and had a successful subsequent course. Two infants died, one in utero because of abruptio placentae and the other one 1 month post partum because of a cerebral haematoma. After a mean follow-up of eighteen months, thrombocytopenia is still present in two women, despite the continuous treatment. In conclusion, ITP rather rarely coincides with pregnancy. Treatment is usually successful for the mother but the risk for the fetus remains considerably high.
Shenoi, Susan; Wallace, Carol A
Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory arthritic disease affecting children. Despite the availability of potent disease-modifying antirheumatic medications, most children still experience a chronic course with long periods of active disease. Goals of treatment should include achievement of disease remission with optimal physical functioning that allows children to lead normal lives with no structural joint damage. The term remission implies a complete lack of disease activity. This article focuses on recently developed preliminary criteria for inactive disease and remission in JIA. Recent studies using these new definitions demonstrate only modest rates of achievement of remission favoring children with persistent oligoarticular JIA. Children with rheumatoid factor-positive polyarticular JIA are least likely to achieve remission. Therapeutic strategies to achieve remission are also discussed.
Hlavenková, Lucia; Gábor, Olivér; Benus, Radoslav; Smrcka, Václav; Jambor, Jaroslav; Hajdu, Tamás
This paper deals with cases of diffuse idiopathic skeletal hyperostosis (DISH) found at the Late Roman Age necropolis in Pécs, Hungary (4th century AD). The skeletal remains of two male individuals, aged between 60-70 years and 45-55 years, displayed right-sided ossification of the anterior longitudinal ligament with extra-spinal manifestations typical in DISH cases. It is presumed that both male individuals were middle-class citizens. Their social status was supplemented with trace element analysis in order to reconstruct the dietary habits of the urban population. Concentrations of Sr and Zn indicated a predominantly vegetal diet. Potential DISH risk factors and associations were subsequently discussed and compared with our findings.
Fujimoto, Hajime; Kobayashi, Tetsu; Azuma, Arata
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive lung disease with a prognosis that can be worse than for many cancers. The initial stages of the condition were thought to mainly involve chronic inflammation; therefore, corticosteroids and other drugs that have anti-inflammatory and immunosuppressive actions were used. However, recently, agents targeting persistent fibrosis resulting from aberrant repair of alveolar epithelial injury have been in the spotlight. There has also been an increase in the number of available antifibrotic treatment options, starting with pirfenidone and nintedanib. These drugs prevent deterioration but do not improve IPF. Therefore, nonpharmacologic approaches such as long-term oxygen therapy, pulmonary rehabilitation, and lung transplantation must be considered as additional treatment modalities. PMID:27980445
Verma, Shikha; Thakur, Binod Kumar
We present a case of an otherwise healthy 10-year-old girl who presented with asymptomatic brown macules over the trunk and proximal extremities, of three months’ duration. The cutaneous examination revealed multiple, dark brown, discrete, round to oval macules and a few mildly elevated lesions over the trunk and proximal limbs. The individual lesion was 1-3 cm in diameter and a few showed velvety appearance over the surface. Darier's sign was negative. The histopathological study from the velvety lesion showed acanthosis, papillomatosis and increased melanin in the basal layer. The upper dermis showed sparse perivascular infiltrate of lymphocytes without any dermal melanophages. It fulfilled the criteria for idiopathic eruptive macular pigmentation with additional histological finding of papillomatosis. PMID:23130237
Lerner, Alicja; Bagic, Anto
Idiopathic Parkinson disease (PD) is traditionally considered a movement disorder with hallmark lesions located in the substantia nigra pars compacta (SNpc). However, recent histopathological studies of some PD cases suggest the possibility of a multisystem disorder which progresses in a predictable sequence as described in Braak's staging criteria. The disease process starts in the dorsal motor nucleus of the vagus (dmX) and anterior olfactory nucleus and bulb, and from there, spreads through the brainstem nuclei to ultimately reach the SNpc, which then presents as symptomatic PD. In this article, we would like to revisit the olfactory pathogenesis of PD based on Braak's staging system and review anatomical pathways supporting such a possibility. We also suggest some biomarkers for early stages of PD. Additionally, we present and discuss the possibility that a prion-like process underlies the neurodegenerative changes in PD.
Mathew, Mariam; Salahuddin, Ayesha; Mathew, Namitha R.; Nandhagopal, Ramachandiran
Postpartum headache is described as headache and neck or shoulder pain during the first 6 weeks after delivery. Common causes of headache in the puerperium are migraine headache and tension headache; other causes include pre-eclampsia/eclampsia, post-dural puncture headache, cortical vein thrombosis, subarachnoid hemorrhage, posterior reversible leukoencephalopathy syndrome, brain tumor, cerebral ischemia, meningitis, and so forth. Idiopathic intracranial hypertension (IIH) is a rare cause of postpartum headache. It is usually associated with papilledema, headache, and elevated intracranial pressure without any focal neurologic abnormality in an otherwise healthy person. It is more commonly seen in obese women of reproductive age group, but rare during pregnancy and postpartum. We present a case of IIH who presented to us 18 days after cesarean section with severe headache and was successfully managed. PMID:26818168
Nascimento, Fábio A.; Gatto, Luana Antunes Maranha; Lages, Roberto Oliver; Neto, Heraldo Mello; Demartini, Zeferino; Koppe, Gelson Luis
Background: Diffuse idiopathic skeletal hyperostosis (DISH) is a systemic noninflammatory disease characterized by ossification of the entheses. Methods: This paper reviews the etiopathogenesis, epidemiology, clinical features, differential diagnosis, and treatment of DISH, based on current available literature. Results: Exact prevalence and incidence of DISH remains undetermined. Many external and genetic factors have been reported as being contributors to the pathogenesis of DISH. Current theories focus on the pathologic calcification of the anterior longitudinal ligament of the spine as the main physiopathological mechanism of disease. Clinical features are variable from monoarticular sinovitis to airway obstruction, and can be associated to systemic conditions. Comorbidities include obesity, hypertension, diabetes mellitus, hyperinsulinemia, dyslipidemia, and hyperuricemia according to a number of reports. Conclusions: DISH is a disease which involves the calcification of the anterior longitudinal ligament of the spine and can be associated with numerous clinical presentations and comorbidities. PMID:24843807
Martin, N D; Snodgrass, G J; Cohen, R D; Porteous, C E; Coldwell, R D; Trafford, D J; Makin, H L
Metabolites of vitamin D were measured in plasma from 83 patients with idiopathic infantile hypercalcaemia syndrome who were mentally handicapped but had normal calcium values at the time of the study. No significant difference was detected in the mean plasma concentrations of 25-hydroxyvitamin D2, 1,25-dihydroxyvitamin D, 24,25-dihydroxyvitamin D3, or 25,26-dihydroxyvitamin D3 between patients and age matched controls. The mean plasma concentration of 25-hydroxyvitamin D3 was significantly lower in patients than controls but this may be a secondary phenomenon related to less sunlight exposure. In addition, two hypercalcaemic patients with this syndrome were studied during the first year of life, and were found to have normal concentrations of vitamin D metabolites. These findings do not support a role for abnormal vitamin D metabolism in the pathogenesis of this syndrome. PMID:3879160
... in regulating phosphate levels within the body (phosphate homeostasis) by transporting phosphate across cell membranes. The SLC20A2 ... link familial idiopathic basal ganglia calcification with phosphate homeostasis. Nat Genet. 2012 Feb 12;44(3):254- ...
Hernandez, Antonio; And Others
Laboratory tests including blood count serum electrolyte measures, and electroencephalograms were performed on seven children ages 1 day to 18 years with recurrent attacks of rapid heart action known as idiopathic paroxysmal ventricular tachycardia. (CL)
Schuller, Simone; Le Garrérès, Alain; Remy, Isabelle; Peeters, Dominique
Idiopathic chylothorax and limb edema was diagnosed in two 2-year-old male whippet siblings. The fact that the 2 related animals developed similar clinical signs at a young age may suggest a congenital or hereditary etiology. PMID:22547848
Schuller, Simone; Le Garrérès, Alain; Remy, Isabelle; Peeters, Dominique
Idiopathic chylothorax and limb edema was diagnosed in two 2-year-old male whippet siblings. The fact that the 2 related animals developed similar clinical signs at a young age may suggest a congenital or hereditary etiology.
Wong, A R; Noor, A S Siti; Rasool, A H G; Quah, B S; Roberton, D
A rare case of childhood pulmonary haemosiderosis with juvenile idiopathic arthritis is discussed, with particular reference to treatment with hydroxychloroquine and sildenafil for pulmonary hypertension which occurs secondary to this disease.
Haut, Marc W.; Hogg, Jeffery P.; Marshalek, Patrick J.; Suter, Blair C.; Miller, Liv E.
We report a case of a 55-year-old man with ischemic lesions of the bilateral hippocampus and bilateral basal ganglia following a myocardial infarction during an episode of multiple drug use with subsequent anoxia requiring resuscitation. He presented for a neuropsychological evaluation with an anterograde amnesia for both explicit and procedural memory. There are two main points to this case, the unique aspects of the bilateral multifocal lesions and the functional, cognitive impact of these lesions. We hypothesize that his rare focal bilateral lesions of both the hippocampus and basal ganglia are a result of anoxia acting in synergy with his stimulant drug use (cocaine and/or 3,4-methylenedioxy-methamphetamine). Second, his unique lesions produced an explicit and implicit/procedural anterograde amnesia. PMID:28228745
Maniar, S; Azzi, K; Iraqi, H; El Hassan Garbi, M; Chraibi, A; Gaouzi, A
The hemihypertrophy or hemihyperplasy is a rare congenital abnormality, characterized by an asymmetric growth of the limbs, the trunk, and the face or half of the entire body. It may be isolated or be part of several syndromes including Beckwith-Wiedemann syndrome, Klippel-Trenaunay-Weber syndrome, Silver-Russell syndrome and Proteus syndrome. In its isolated form, it is called idiopathic. The latter may be associated with several anomalies including dermatological and urogenital abnormalities with increased risk of developing embryonal tumors. We report the case of a 22-month-old infant, who was referred by his pediatrician at the age of 15 months for a corporeal hemihypertrophy associated with hemihypertrichosis. In his medical history, a second degree parental consanguinity and a hypospadias in the father and a paternal uncle were found. Clinical examination found a weight and a size greater than chronological age (3 standard deviations), a hemihypertrophy of entire left side with a difference of length and diameter between the left and right limbs of 2 cm. The hemihypertrichosis was widespread in the left body and the genital examination found a hypospadias. Biological and radiological assessments did not show any abnormality, with the exception of an initially high plasma testosterone level, which gradually normalized. Thus, the diagnosis of idiopathic hemihypertrophy with congenital hemihypertrichosis was retained. This is the fourth case reported in the literature. Its management is similar to all hemihypertrophies, which consists of an initial assessment to detect an embryonic tumor, followed by a monitoring protocol including an abdominal and renal ultrasound every 6 months until the age of 8, determination of alpha-feto-protein every 6 to 12 weeks until the age of 4 years to track the development of the two most frequent tumors: Wilms tumor and hepatoblastoma. The hemihypertrophy associated with hemihypertrichosis has been exceptionally reported and the
Leppaenen, Martti; Seppaenen, Seppo; Laranne, Jussi; Kuoppala, Katriina
Purpose: To assess the efficacy and safety of microcatheter embolization in the treatment of intractable idiopathic epistaxis. Methods: Thirty-seven patients underwent microcatheter embolization in 1991-1998. We evaluated retrospectively the technical and clinical outcome, the number of complications, the duration of embolization in each case, and the number of blood transfusions needed. All embolizations were done with biplane digital subtraction angiography (DSA) equipment. The procedure was carried out under local anesthesia using transfemoral catheterization, except in one case where the translumbar route was used. Tracker 18 or 10 microcatheters were advanced as far as possible to the distal branches of the sphenopalatine artery. Polyvinyl alcohol (PVA) particles were used for embolization in most cases, while platinum coils or a combination of these two materials were occasionally used. The primary outcome was always assessed immediately by angiography. Follow-up data were obtained from patient records, by interviewing patients on the telephone or by postal questionnaires when necessary. The mean follow-up time was 21 months. Results: The embolization was technically successful in all 37 cases. A curative outcome was achieved in 33 cases (89%). The mean duration of the procedure was 110 min. Four patients (8%) had mild transient complications, but no severe or persistent complications were encountered. Twenty-three patients needed a blood transfusion. Slight rebleeding occurred in three patients during the follow-up; all responded to conservative treatment. One patient suffered two episodes of rebleeding within 2 months after primary embolization. Re-embolizations successfully stopped the bleeding. Conclusion: Embolization is the primary invasive modality for treating intractable idiopathic epistaxis. It proved both safe and effective over a relatively long follow-up.
Arnulf, Isabelle; Neutel, Dulce; Herlin, Bastien; Golmard, Jean-Louis; Leu-Semenescu, Smaranda; Cochen de Cock, Valérie; Vidailhet, Marie
Objective: To determine whether patients with idiopathic and symptomatic RBD were sleepier than controls, and if sleepiness in idiopathic RBD predicted earlier conversion to Parkinson disease. Methods: The Epworth Sleepiness Scale (ESS) and its determinants were compared at the time of a video-polysomnography for an RBD diagnosis in patients with idiopathic RBD, in patients with Parkinson disease, and in controls. Whether sleepiness at time of RBD diagnosis predicted an earlier conversion to neurodegenerative diseases was retrospectively analyzed in the followed-up patients. Results: The 75 patients with idiopathic RBD were sleepier (ESS: 7.8 ± 4.6) at the time of RBD diagnosis than 74 age- and sex-matched controls (ESS: 5.0 ± 3.6, P < 0.0001). They reached the levels of 114 patients with Parkinson disease (ESS: 8.7 ± 4.8), whether they had (n = 78) or did not have (n = 36) concomitant RBD. The severity of sleepiness in idiopathic RBD correlated with younger age, but not with sleep measures. Among the 69 patients with idiopathic RBD who were followed up for a median 3 years (1–15 years), 16 (23.2%) developed parkinsonism (n = 6), dementia (n = 6), dementia plus parkinsonism (n = 2), and multiple system atrophy (n = 2). An ESS greater than 8 at time of RBD diagnosis predicted a shorter time to phenoconversion to parkinsonism and dementia, from RBD onset, and from RBD diagnosis (when adjusted for age and time between RBD onset and diagnosis). Conclusions: Sleepiness is associated with idiopathic REM sleep behavior disorder and predicts more rapid conversion to parkinsonism and dementia, suggesting it is an early marker of neuronal loss in brainstem arousal systems. Citation: Arnulf I, Neutel D, Herlin B, Golmard JL, Leu-Semenescu S, Cochen de Cock V, Vidailhet M. Sleepiness in idiopathic REM sleep behavior disorder and Parkinson disease. SLEEP 2015;38(10):1529–1535. PMID:26085299
Krysiak, Robert; Marek, Bogdan; Okopień, Bogusław
Central precocious puberty, defined as the onset of puberty before the age 8 years in girls and 9 years in boys, results from a premature activation of gonadotropin-releasing hormone neurons in the hypothalamus. This condition is characterised by early pubertal changes, acceleration of growth velocity, and rapid bone maturation that often result in reduced adult height. It may be either idiopathic or associated with hypothalamic hamartoma, brain neoplasms, numerous non-cancerous disorders of the central nervous system and treatment of peripheral precocious puberty. The goal of the initial assessment of children is to exclude the presence of all these organic disorders. The diagnosis should include detailed anamnesis and clinical examination, measurement of pituitary and sex hormones, assessment of bone age, and imaging of the hypothalamus, pituitary gland, abdomen, pelvis and gonads. The treatment of choice are gonadotropin-releasing hormone agonists. In this paper, we review the current views on the etiopathogenesis, clinical presentation, diagnosis and management of central precocious puberty.
Volonte, M; Perani, D; Lanzi, R; Poggi, A; Anchisi, D; Balini, A; Comi, G; Fazio, F
A [18F]-FDG PET study was performed in a 44 year old man with proximal kinesigenic choreoathetosis (PKC) secondary to idiopathic primary hypoparathyroidism (IPH) before and 1 year after calcium/calcitriol therapy. The [18F]-FDG PET performed before the start of the therapy disclosed a significant bilateral hypometabolism in the ventral striatum. One year later, with the patient still under calcium/calcitriol therapy and free of any occurrence of PKC episodes, the [18F]-FDG PET did not show the previously detected hypometabolism. The hypometabolism of the ventral striatum secondary to hypocalcaemia seems to play a crucial part in the pathogenesis of paroxysmal kinesigenic choreoathetosis associated with IPH. PMID:11606688
Goyal, R W; Ng, A B Y; Bale, R S
Gas gangrene is a rare and rapidly progressive process with the propensity for devastating consequences. We report the first case of bilateral gas gangrene of the hand following bilateral middle phalangectomy of the middle fingers.
Kodama-Takahashi, K; Ohshima, K; Yamamoto, K; Iwata, T
We describe an elderly case of idiopathic dilatation of the right atrium in which right-sided heart failure was exacerbated by drug-induced bradyarrhythmia. An 84-year-old man, who had a 10-year history of episodic edema, was treated with proscillaridin and verapamil hydrochloride at another hospital. He had experienced a poor appetite and general malaise 2 months previously, and exertional dyspnea 10 days previously. On admission, he had jugular venous dilatation, systemic edema, and hepatomegaly. On auscultation, a third heart sound originating from the right ventricle and systolic murmur of tricuspid regurgitation were heard. An admission electrocardiogram showed an atrial standstill and junctional escape rhythm with a QRS rate of 31 beats/minute. Chest roentgenogram revealed a bilateral pleural effusion and cardiomegaly with a cardiothoracic ratio of 76%, but no pulmonary congestion. Echocardiogram disclosed idiopathic dilatation of the right atrium and secondary tricuspid regurgitation. He was given a diagnosis of right-sided heart failure due to idiopathic dilatation of the right atrium exacerbated by bradyarrhythmia, which was suspected to derive from the side effects of proscillaridin and verapamil hydrochloride. Thus, these agents were withheld. In addition, the patient reduced sodium intake and was treated with diuretics and beta-adrenergic agonist. Thereafter, right-sided heart failure markedly improved. At the time of the last follow-up 16 months after discharge, he felt well.
Gordon, Sarah; Kerns, Tamie; Londeree, William; Ching, Brian
Thrombosis of the inferior vena cava is less common than deep venous thrombosis of the lower extremities, particularly in the absence of an obvious congenital caval abnormality or hypercoagulable state. We present a case of IVC thrombosis in an otherwise healthy and active 28-year-old male soldier secondary to dehydration and venous webbing. IVC thrombosis is an uncommon and underrecognized condition; in this case, the patient's caval thrombosis was initially mistaken for acute back strain. Prompt recognition is necessary to minimize long-term sequelae. PMID:24187556
Singla, Komal; Preet Malhotra, Kiran; Rathore, Ruchi; Arora, Deepshikha; Sharma, Sonal
Leiomyosarcoma of the scrotum is a rare tumor. We report the case of a 60-year-old man who presented with bilateral testicular enlargement that was clinically misdiagnosed as a testicular tumor. The tumor was excised by bilateral inguinal orchiectomy. Histopathologic examination revealed a scrotal wall leiomyosarcoma with bilateral epididymo-orchitis.
Mauler, Flavien; Rahm, Stefan; Schweizer, Andreas; Nagy, Ladislav
Background Accessory carpal ossicles may be the cause of atraumatic wrist pain or may be misinterpreted as a fracture after a trauma. Case Description We report the case of a patient suffering with chronic, bilateral wrist pain without history of trauma. Sonographic examination showed a bilateral symptomatic os epilunatum, both of which were surgically resected, resulting in pain relief. Literature Review Os epilunatum is a rare entity that has been reported only in few cadaveric specimen. Clinical Relevance Os epilunatum is a rare accessory ossicle of the carpus that can cause this aggravating pain. Sonography enabled accurate diagnosis of this anomaly. Because of possible associated tear of the scapholunate ligament, we recommend intraoperative testing of the stability of the scapholunate joint. PMID:25709883
Garft, Kyla; Burt, Peter; Burt, Benjamin
We report a case of bilateral orbital hemorrhage as a complication of peribulbar anesthesia in a 78 year old man. Initially, unilateral orbital hemorrhage occurred but quickly spread to the contralateral side. Neuroophthalmological assessment revealed a proptosed tense globe with normal retinovascular findings. Visual acuity was adversely affected and this was conservatively managed with no lasting ophthalmic sequela. This patient’s case was reported as it illustrates an unusual complication of bilateral spread of orbital hemorrhage secondary to peribulbar anesthesia. It highlights how early ophthalmic assessment can ensure a good visual outcome in the setting of appropriate ophthalmic monitoring. The mechanisms of orbital hemorrhage spread and appropriate management options are discussed. PMID:27013899
Dundar, Mehmet Akif; Derin, Serhan; Aricigil, Mitat; Eryilmaz, Mehmet Akif
Sudden bilateral hearing loss are seen rarely and the toxic substance exposure constitutes a small part of etiology. A Fifty-eight-year-old woman admitted to our clinic with sudden bilateral hearing loss shortly after chlorpyrifos-ethyl exposure. Otolaryngologic examination findings were normal. The patient had 40 dB sensorineural hearing loss (SNHL) on the right ear and 48 dB SNHL on the left ear. Additional diagnostic tests were normal. The conventional treatment for sudden hearing loss was performed. On the second week following organophosphate (OP) exposure the patient's hearing loss almost completely resolved. OP's are heavily used in agriculture and should be taken into consideration as an etiologic factor in sudden hearing loss.
Lee, Song Eun; Jung, Chaeyong; Ahn, Kyu Youn
Anatomical variations of the biceps brachii have been described by various authors, but the occurrence of bilateral asymmetric supernumerary heads is rare and has not been reported. We found three accessory heads of the biceps brachii muscle on right arm and an anomalous third head of biceps brachii on left arm. The third, fourth, and fifth heads of right arm originated from the body of humerus at the insertion site of coracobrachialis and inserted into the distal part of biceps brachii short head in order. The third head of left arm originated from humerus at the insertion site of coracobrachialis and combined with the distal part of biceps brachii and continued to the proximal part of common biceps tendon. Understanding the existence of bilateral asymmetric supernumerary heads of biceps brachii may influence preoperative diagnosis and surgery on the upper limbs. PMID:22025976
Sulak, Osman; Albay, Soner; Tagil, Suleyman M; Malas, Mehmet A
Bilateral unusual coursed ovarian arteries were found in a 45-year-old female cadaver. The ovarian arteries arose from the abdominal aorta at a level inferior to the corpus of L1 vertebra bilaterally. The right ovarian artery was coursing behind the inferior vena cava. The left one was coursing between the left renal artery and vein upward to the level of the inferior part of the body of Th12 vertebra. At the level of Th12 vertebra, it turned caudally and proceeded downward between the anterior-inferior and posterior segmental renal veins. On both sides, ovarian arteries were on their normal routes below the L2 vertebra accompanying the ovarian veins. The variation determined by this study would reduce possible complications of nephrectomy operations that urologists, gynecologists and other related surgeons may encounter.
Turner, K; Reid, W; Homewood, J; Cook, R
Methods: 17 patients with Parkinson's disease were evaluated with a neuropsychological battery before and six months after bilateral pallidotomy. A comparison group (n = 8) was also assessed at six month intervals. Outcome variables were tests of memory, language, visuospatial function, attention, executive skills, and depression. Results: Despite a large number of variables studied, a significant postsurgical change was found only in performance of the tower of London task, a measure of planning abilities. The effect size of this change was larger than that of the comparison group, and a reliable change index score established that 5 of 13 surgical patients had statistically reliable reductions in planning performance. Conclusions: Patients with a young age of onset and long duration of Parkinson's disease who underwent bilateral pallidotomy had a relatively circumscribed reduction in neuropsychological functioning, being limited to motor planning efficiency. These data suggest that the cognitive role of the posteroventral globus pallidus is limited, at least in people with Parkinson's disease. PMID:12235317
Geidel, K; Schob, S; Unterlauft, J D; Wiedemann, P; Meier, P
A 49-year-old female victim of violent crime with an acute bilateral loss of vision was referred to our hospital. The ophthalmological evaluation showed complete subconjunctival hemorrhage of both eyes, bilateral hemophthalmos and hypotonia of the left eye. These raised the suspicion of an occult scleral rupture. We immediately performed exploratory surgery and found a perforating scleral lesion of the left eye and a penetrating scleral lesion of the right eye. Furthermore, a small, cruciform wound was detected on the left temple. In cooperation with the department of radiology, the extraordinary injury pattern was reconstructed: a horizontal stab wound with perforation of the left eye and penetration of the right eye caused by a screwdriver. Visual rehabilitation necessitated further surgical interventions. Besides the intraoperative approach, immediate primary wound management within 100 h of trauma plays a pivotal role for long-term outcome.
Undabeitia, Jose; Liu, Brian; Pendleton, Courtney; Nogues, Pere; Noboa, Roberto; Undabeitia, Jose Ignacio
Although traumatic injury of the facial nerve is a relatively common condition in neurosurgical practice, bilateral lesions related to fracture of temporal bones are seldom seen. We report the case of a 38-year-old patient admitted to Intensive Care Unit after severe head trauma requiring ventilatory support (Glasgow Coma Scale of 7 on admission). A computed tomography (CT) scan confirmed a longitudinal fracture of the right temporal bone and a transversal fracture of the left. After successful weaning from respirator, bilateral facial paralysis was observed. The possible aetiologies for facial diplegia differ from those of unilateral injury. Due to the lack of facial asymmetry, it can be easily missed in critically ill patients, and both the high resolution CT scan and electromyographic studies can be helpful for correct diagnosis.
Zhang, Miao; Wang, Heng; Pan, Xue-Feng; Wu, Wen-Bin; Zhang, Hui
The aim of the present study was to investigate the feasibility and efficacy of staged bilateral single-port thoracoscopic lung volume reduction surgery (LVRS) for patients with chronic obstructive pulmonary emphysema (COPE). Eleven male patients with a mean age of 60.27±12.11 years with bilateral COPE and bullae were admitted to the Department of Thoracic Surgery, Xuzhou Central Hospital from January 2013 to June 2014. The patients underwent staged bilateral single-port thoracoscopic LVRS. The hyperinflated bullae were resected using endoscopic staplers (Endo-GIA), followed by continuous suture and biological glue for reinforcement of the margin. In addition, pulmonary function, blood gas assay, 6-min walk distance (6MWD) and life quality evaluated by a short form 36-item health survey questionnaire (SF-36) were recorded before and after LVRS, respectively. All the patients survived after surgery. The chest tube drainage time was 9.09±1.31 days and postoperative hospital stay was 15.73±2.75 days, with 5 cases of persistent air leakage and 7 cases of pulmonary infection which were finally cured. The patients were followed up for 3 to 12 months, and the pulmonary function, partial pressure of oxygen (pO2), 6MWD and life quality after unilateral or bilateral LVRS were improved compared to these parameters before surgery. However, there was no significant difference between unilateral and bilateral LVRS in terms of life quality. In conclusion, staged bilateral single-port thoracoscopic LVRS may improve the short-term life quality of patients with COPE. PMID:27882084
Land, I; Knolle, H
It is reported on the rare case of a 46-year-old female patient with a bilateral mesothelioma of the pleura without contact to asbest. Although the female was suspected in a malignant tumor and many diagnostic investigations were performed, diagnosis could be ensured morphologically only a short time before her death. Causes and development of mesothelioma, histological types, clinical symptoms and diagnostic procedures are described.
Weng, Kedi; Zhang, Xingen; Bi, Qing; Zhao, Chen
Abstract Objective: A meta-analysis was performed to investigate the effectiveness and safety of tranexamic acid (TXA) for the treatment of blood loss after a bilateral total knee arthroplasty (TKA). Methods: Patients prepared for bilateral TKA and intervention including TXA versus placebo were comprehensively retrieved from MEDLINE (PubMed), Embase, Cochrane Central Register of Controlled Trials (CENTRAL), and Web of Science from the time of the establishment of these databases to January 2016. The outcomes were all calculated by Stata 12.0 software. The continuous endpoints (total blood loss and blood loss in drainage) were calculated as mean difference (MD) and 95% confidence intervals (CIs). Binary variables (the need for transfusion, and the occurrence of deep venous thrombosis [DVT]) were calculated as relative risk (RR) with 95% CIs. Results: Pooled results revealed that treatment with TXA associated with less need for transfusion (P = 0.000) and the value of Hb drop postoperatively (P = 0.290) after bilateral TKA. The results also indicated that TXA can decrease the total blood loss and blood loss in drainage after bilateral TKA (P < 0.05). Meanwhile, TXA can decrease the blood units transfused per patient by 1.23 U (P = 0.001). There is no statistically significant difference in terms of the occurrence of DVT between the 2 groups (P = 0.461). Conclusion: Based on the current evidence, TXA can decrease the need for transfusion and the total blood loss without increasing the occurrence of DVT, and its administration is recommended routinely in bilateral TKA. PMID:27684841
de Andrade, Joao; Schwarz, Marvin; Collard, Harold R.; Gentry-Bumpass, Tedryl; Colby, Thomas; Lynch, David; Schwarz, M.; Zisman, D. A.; Hunninghake, G.; Chapman, J.; Olman, M.; Lubell, S.; Morrison, L. D.; Steele, M. P.; Haram, T.; Roman, J.; Perez, R.; Perez, T.; Ryu, J. H.; Utz, J. P.; Limper, A. H.; Daniels, C. E.; Meiras, K.; Walsh, S.; Brown, K. K.; Schwarz, M.; Bair, C.; Kervitsky, D.; Lasky, J. A.; Ditta, S.; deAndrade, J.; Thannickal, V. J.; Stewart, M.; Zisman, D. A.; Lynch, J.; Calahan, E.; Lopez, P.; King, T. E.; Collard, H. R.; Golden, J. A.; Wolters, P. J.; Jeffrey, R.; Noth, I.; Hogarth, D. K.; Sandbo, N.; Strek, M. E.; White, S. R.; Brown, C.; Garic, I.; Maleckar, S.; Martinez, F. J.; Flaherty, K. R.; Han, M.; Moore, B.; Toews, G. B.; Dahlgren, D.; Raghu, G.; Hayes, J.; Snyder, M.; Loyd, J. E.; Lancaster, L.; Lawson, W.; Greer, R.; Mason, W.; Kaner, R. J.; Monroy, V.; Wang, M.; Lynch, D. A.; Colby, T.; Anstrom, K. J.; Becker, R. C.; Eisenstein, E. L.; MacIntyre, N. R.; Morrison, L. D; Rochon, J.; Steele, M. P.; Sundy, J. S.; Davidson-Ray, L.; Dignacco, P.; Edwards, R.; Anderson, R.; Beci, R.; Calvert, S.; Cain, K.; Gentry-Bumpass, T.; Hill, D.; Ingham, M.; Kagan, E.; Kaur, J.; Matti, C.; McClelland, J.; Meredith, A.; Nguyen, T.; Pesarchick, J.; Roberts, R. S.; Tate, W.; Thomas, T.; Walker, J.; Whelan, D.; Winsor, J.; Yang, Q.; Yow, E.; Reynolds, H. Y.; Tian, X.; Kiley, J.; Noth, I.; Olman, M.; Schwarz, M.; Toews, G. B.; Hunninghake, G.; Culver, D. A.; Chapman, J.; Olman, M.; Lubell, S.; Wehrmann, R.; Morrison, L. D.; Steele, M. P.; Haram, T.; Kidd, R.; Kallay, M.; Lyda, E.; Ryu, J. H.; Utz, J. P.; Limper, A. H.; Daniels, C. E.; Meiras, K.; Walsh, S.; Sahn, S.; O’Banner, N.; Stokes, F.; Brown, K. K.; Bair, C.; Kervitsky, D.; Ettinger, N. A.; Merli, S.; de Andrade, J.; Thannickal, V. J.; Stewart, M.; Belperio, J.; Lynch, J. P.; Calahan, E.; Lopez, P.; King, T. E.; Collard, H. R.; Golden, J.; Wolters, P.; Eller, A.; Noth, I.; Hogarth, D. K.; Sandbo, N.; Strek, M. E.; Maleckar, S.; Rahimova, G.; Sardin, L.; Roman, J.; Perez, R.; Perez, T.; Glassberg, M.; Simonet, E.; Martinez, F. J.; Baumann, K.; Chan, K.; Chughtai, A.; Gross, B.; Flaherty, K. R.; Han, M. L.; Hyzy, R.; Kazerooni, E.; Moore, B.; Myers, J.; Toews, G. B.; White, E.; Dahlgren, D.; Rossman, M.; Kreider, M.; Le, K.; Fitzgerald, J.; Glazer, C.; Scholand, M. B.; Brewster, L.; Johnson, A.; Raghu, G.; Berry-Bell, P.; Snydsman, A.; Loyd, J. E.; Lancaster, L.; Lawson, W.; Greer, R.; Kinser, K.; Richardson, R.; Mason, W.; Kaner, R. J.; Bandong, K.; Antin-Ozerkis, D.; Holm, C.; Estrom, J.; Lynch, D. A.; Colby, T.; Anstrom, K. J.; Eisenstein, E. L.; Sundy, J. S.; Davidson-Ray, L.; Dignacco, P.; Edwards, R.; Beci, R.; Calvert, S.; Gentry-Bumpass, T.; Hill, D.; Hofmann, P. V.; Hwang, K.; Kaur, J.; Matti, C.; Meredith, A.; Pesarchick, J.; Ramey, S.; Roberts, R. S.; Sharlow, A.; Winsor, J.; Yang, Q.; Yow, E.; Weinmann, G. G.; Reynolds, H.; Schmetter, B.; Tian, X.; Kiley, J.; Martinez, F. J.; Raghu, G.; Schwarz, M.; Toews, G. B.; Zibrak, J.; Demersky, A.; Vey, M.; Rosas, I. O.; Debrosse, P.; Culver, D. A.; Chapman, J.; Olman, M.; Lubell, S.; Wehrmann, R.; Morrison, L. D.; Steele, M. P.; Haram, T.; Kidd, R.; Kallay, M.; Lyda, E.; Ryu, J. H.; Utz, J. P.; Limper, A. H.; Daniels, C. E.; Meiras, K.; Walsh, S.; Sahn, S.; O’Banner, N.; Stokes, F.; Padilla, M.; Berhanu, G.; Brown, K. K.; Bair, C.; Kervitsky, D.; Ettinger, N. A.; Merli, S.; Criner, G. J.; Swift, I. Q.; Satti, A.; Cordova, F.; Patel, N.; West, K.; Jones, G.; Lasky, J. A.; Ditta, S.; de Andrade, J.; Thannickal, V. J.; Stewart, M.; Belperio, J.; Lynch, J. P.; Calahan, E.; Lopez, P.; King, T. E.; Collard, H. R.; Golden, J.; Wolters, P.; Eller, A.; Noth, I.; Hogarth, D. K.; Sandbo, N.; Strek, M. E.; Maleckar, S.; Rahimova, G.; Sardin, L.; Roman, J.; Perez, R.; Perez, T.; Glassberg, M.; Simonet, E.; Martinez, F. J.; Baumann, K.; Chan, K.; Chughtai, A.; Gross, B.; Flaherty, K. R.; Han, M. L.; Hyzy, R.; Kazerooni, E.; Moore, B.; Myers, J.; Toews, G. B.; White, E.; Dahlgren, D.; Rossman, M.; Kreider, M.; Le, K.; Fitzgerald, J.; Glazer, C.; Scholand, M. B.; Brewster, L.; Johnson, A.; Raghu, G.; Berry-Bell, P.; Snydsman, A.; Loyd, J. E.; Lancaster, L.; Lawson, W.; Greer, R.; Mason, W.; Kaner, R. J.; Bandong, K.; Antin-Ozerkis, D.; Holm, C.; Estrom, J.; Lynch, D. A.; Colby, T.; Anstrom, K. J.; Becker, R. C.; Eisenstein, E. L.; Sundy, J. S.; Davidson-Ray, L.; Dignacco, P.; Edwards, R.; Beci, R.; Calvert, S.; Cain, K.; Gentry-Bumpass, T.; Hill, D.; Huang, K.; Kaur, J.; Matti, C.; Meredith, A.; Pesarchick, J.; Ramey, S.; Roberts, R. S.; Sharlow, A.; Winsor, J.; Yow, E.; Weinmann, G. G.; Reynolds, H.; Schmetter, B.; Tian, X.; Kiley, J.
BACKGROUND: The National Heart, Lung, and Blood Institute-sponsored IPF Clinical Research Network (IPFnet) studies enrolled subjects with idiopathic pulmonary fibrosis (IPF) to evaluate drug therapies in treatment trials. An adjudication committee (AC) provided a structured review of cases in which there was uncertainty or disagreement regarding diagnosis or clinical event classification. This article describes the diagnosis and adjudication processes. METHODS: The diagnostic process was based on review of clinical data and high-resolution CT scans with central review of lung biopsies when available. The AC worked closely with the data coordinating center to obtain clinical, radiologic, and histologic data and to communicate with the clinical centers. The AC used a multidisciplinary discussion model with four clinicians, one radiologist, and one pathologist to adjudicate diagnosis and outcome measures. RESULTS: The IPFnet trials screened 1,015 subjects; of these, 23 cases required review by the AC to establish eligibility. The most common diagnosis for exclusion was suspected chronic hypersensitivity pneumonitis. The AC reviewed 88 suspected acute exacerbations (AExs), 93 nonelective hospitalizations, and 16 cases of bleeding. Determination of AEx presented practical challenges to adjudicators, as necessary clinical data were often not collected, particularly when subjects were evaluated outside of the primary study site. CONCLUSIONS: The IPFnet diagnostic process was generally efficient, but a multidisciplinary adjudication committee was critical to assure correct phenotype for study enrollment. The AC was key in adjudicating all adverse outcomes in two IPFnet studies terminated early because of safety issues. Future clinical trials in IPF should consider logistical and cost issues as they incorporate AExs and hospitalizations as outcome measures. TRIAL REGISTRY: ClinicalTrials.gov; No.: NCT00517933, NCT00650091, NCT00957242; URL: www.clinicaltrials.gov PMID
Jung, Cecilia S.; Payne, John F.; Bergstrom, Chris S.; Cribbs, Blaine E.; Yan, Jiong; Hubbard, G. Baker; Olsen, Timothy W.; Yeh, Steven
Objective To describe the clinical features and imaging characteristics in unilateral acute idiopathic maculopathy (UAIM). Methods This is a retrospective review of four patients diagnosed with UAIM. Clinical characteristics (age, symptoms, Snellen visual acuity (VA), and funduscopic features) and images from spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF), fluorescein (FA), and indocyanine green (ICG) angiography were analyzed. Results The median age at presentation was 31 years (range 27–52 years). The median interval between symptom onset and presentation was four weeks (range 1–20 weeks). Associated systemic findings included a viral prodrome (50%), orchitis (50%), hand-foot-mouth disease (25%), and positive Coxsackie virus titers (50%). The median presenting VA was 20/400 (range 20/70–1/400), which improved to 20/30 (range 20/20–20/60) at final follow-up. The median follow-up time was 6 weeks (range 0–8 weeks). Early in the disease course, the central macula developed irregular, circular areas of white-grey discoloration. Following recovery, the macula had a stippled retinal pigment epithelium characterized by rarefaction and hyperplasia. FA demonstrated irregular early hyperfluorescence and late subretinal hyperfluorescence. SD-OCT showed a partially reversible disruption of the outer photoreceptor layer. FAF initially revealed stippled autofluorescence that eventually became more hypoautofluorescent. ICG showed “moth-eaten” appearing choroidal vasculature, suggestive of choroidal inflammation. Conclusions The imaging characteristics highlight the structural changes during the active and resolution phases of UAIM. The visual recovery correlates with structural changes and suggests that the pathogenesis involves inflammation of the inner choroid, retinal pigment epithelium, and outer photoreceptor complex that is partially reversible. PMID:22232475
Leal-Arcas, Rafael; Grasso, Costantino; Rios, Juan Alemany )
The current international energy trade governance system is fragmented and multi-layered. Streamlining it for greater legal cohesiveness and international political and economic cooperation would promote global energy security. The current article explores three levels of energy trade governance: multilateral, regional and bilateral. Most energy-rich countries are part of the multilateral trading system, which is institutionalized by the World Trade Organization (WTO). The article analyzes the multilateral energy trade governance system by focusing on the WTO and energy transportation issues. Regionally, the article focuses on five major regional agreements and their energy-related aspects and examines the various causes that explain the proliferation of regional trade agreements, their compatibility with WTO law, and then provides several examples of regional energy trade governance throughout the world. When it comes to bilateral energy trade governance, this article only addresses the European Union’s (EU) bilateral energy trade relations. The article explores ways in which gaps could be filled and overlaps eliminated whilst remaining true to the high-level normative framework, concentrating on those measures that would enhance EU energy security.
Belaïd, Asma; Mghirbi, Fahmi; Béhi, Khalil; Doghri, Raoudha; Benna, Farouk
Breast cancer is the most common malignancy in women. The most frequent metastatic sites are lung, bone, liver and brain. On the other hand, gastric metastases are rare. Synchronous bilateral breast cancer (SBBC) occurs rarely. Lobular carcinoma is the histological type most often associated with bilateral breast carcinomas and gastric metastases. We made a retrospective study including four patients followed in the Salah Azaiez Institute, for a bilateral breast cancer with gastric metastases. We analyzed the epidemiological, anatomoclinical and therapeutic particularities of this rare entity. Symptoms were unspecific. The diagnosis of gastric metastasis of the SBBC was confirmed by a histopathological examination of an endoscopic biopsy. The median age was 46.2 years (range, 36–51 years) and the median time until the gastric involvement was 19 months (range, 0–41 months). None of patients had a surgical treatment for the gastric location. All Patients received at least one line of chemotherapy and radiotherapy. Median survival following the detection of gastric involvement was 22 months (range, 1–56 months). Gastric metastases from breast cancer are rare and frequently associated with other distant metastasis. Symptoms are unspecific and endoscopy may not be contributive. Therefore, gastric involvement is underestimated. Lobular infiltrating carcinoma (LIC) is the most histological type incriminated in its occurrence. The supply of immunohistochemistry is crucial to distinguish between primary or metastatic gastric cancer. PMID:28280631
Bethel, E. Wes
Image smoothing is a fundamental operation in computer vision and image processing. This work has two main thrusts: (1) implementation of a bilateral filter suitable for use in smoothing, or denoising, 3D volumetric data; (2) implementation of the 3D bilateral filter in three different parallelization models, along with parallel performance studies on two modern HPC architectures. Our bilateral filter formulation is based upon the work of Tomasi , but extended to 3D for use on volumetric data. Our three parallel implementations use POSIX threads, the Message Passing Interface (MPI), and Unified Parallel C (UPC), a Partitioned Global Address Space (PGAS) language. Our parallel performance studies, which were conducted on a Cray XT4 supercomputer and aquad-socket, quad-core Opteron workstation, show our algorithm to have near-perfect scalability up to 120 processors. Parallel algorithms, such as the one we present here, will have an increasingly important role for use in production visual analysis systems as the underlying computational platforms transition from single- to multi-core architectures in the future.
Imerci, Ahmet; Incesu, Mustafa; Bozoglan, Muhammet; Canbek, Umut; Ursavas, Hüseyin Tamer
The majority of plantar heel pain is diagnosed as plantar fasciitis or heel spur syndrome. When history or physical findings are unusual or when routine treatment proves ineffective, one should consider an atypical cause of heel pain. Stress fractures of the calcaneus are a frequently unrecognized source of heel pain. In a normal populatıon, the possibility of calcaneal stress fractures must be borne in mind with patients who have bilateral heel pain. When a stress fracture is considered, clinicans have different imaging options. First of all, x-rays must be used to evaluate for any visible osseous pathology. If plain films are inconclusive, the clinician can proceed with a bone scan or Magnetic Resonance Imaging. In the literature, calcaneal stress fractures are mostly reported in soldiers or athletes, but our case is one of a 44-year-old housewife with bilateral heel pain treated as Achilles tendinitis and plantar faciitis for a long time. Her final diagnosis was bilateral calcaneal fracture by Magnetic Resonance Imaging.
Selmic, Laura E; Ryan, Stewart D; Ehrhart, Nicole P; Withrow, Stephen J
Bilateral synchronous appendicular bone tumors, occurring in the same bone and same anatomic site within the bone are very rare. This report describes the clinical presentation and oncologic outcome for four dogs with this rare presentation. All cases presented to the authors following a history of unilateral lameness for several weeks. On presentation, case 1 had pain elicited in the contralateral proximal humerus but all the other cases had no abnormalities detectable on physical examination of the contralateral limb. All dogs had technetium 99m ((99m)Tc) nuclear scintigraphy performed that identified bilateral lesions of the distal radii in two dogs, proximal humeri and distal tibiae in one dog each. Thoracic radiographs performed on all dogs showed no evidence of pulmonary metastases. Three dogs were treated with palliative radiation therapy (two dogs received concurrent bisphosphonates) resulting in survival times from initial presentation of 50 days, 193 days, and 523 days, respectively. One dog had stereotactic radiation therapy (SRT) and a surgical limb-salvage performed followed by carboplatin chemotherapy, resulting in a survival time of 926 days from initial presentation. Palliative and curative-intent treatments for the bilateral synchronous appendicular bone tumors resulted in survival times similar to those reported for treatment of a single primary appendicular bone tumor.
Paša, L; Veselý, R; Kelbl, M
The authors present a rare case of bilateral asymmetric traumatic dislocation of hip joints, where the left joint was treated conservatively after the reduction, while the right joint, with an acetabular fragment interposition, was treated surgically - by arthroscopically assisted reduction and fixation of an osteochondral fragment of posterior wall of the acetabulum. The female patient healed with no complications, showing an excellent clinical outcome with no signs of instability or limited mobility of hip joints, and also with no signs of para-articular calcification or necrosis of the hip at 1 year after the injury and treatment. Bilateral asymmetric dislocation of hip joint is a rare injury with the total incidence of 150 cases as reported by the literature. Recently, its incidence is higher due to the increased traffic and the associated accident rate. A precise and prompt reduction of the injured hip joint is always necessary, if possible under general anesthesia. Also, it is always necessary to carry out a complete examination of the patient since this type of injury is always caused by a strong force and is often accompanied by injuries of other parts of the body. Key words: bilateral asymmetric dislocation of hip joints, hip arthroscopy, acetabular fracture.
Zhou, Xu-Jie; Liu, Li-Jun; Chen, Min; Zhou, Fu-De
Renal infarction (RI) is frequently misdiagnosed or diagnosed late because of its rarity and nonspecific clinical presentation, which may result in irreversible damage to the renal parenchyma or increase the risk of other embolic events affecting additional organs. Multiple causal mechanisms and cases of idiopathic RI have been reported, but the causal factors are not clear in most cases.Here, we report the case of a patient with heterochronic bilateral RI caused by thrombophilia. Although he had several risk factors for hypercoagulation disorders, two gene mutations-MTHFR 677 C>T and PLG 1858G>A-were identified by genome sequencing of the entire exome. The findings suggest the possibility of a synergistic relationship between the two gene mutations.Thus, screening for gene mutations may provide additional clues for clarifying the cause of RI and thrombophilia.
Seyer, J M; Hutcheson, E T; Kang, A H
Collagens in normal human lung and in idiopathic chronic fibrosis were investigated in terms of their covalent structure and compared for possible alterations in the diseased state. Collagens were solubilized by limited digestion with pepsin under nondenaturing conditions, and after purification they, were fractionated into types I and III. Carboxymethylcellulose and agarose chromatography of both types I and III collagens, and amino acid and carbohydrate analyses of the resulting alpha-chains indicated that the alpha 1 (I), alpha 2, and alpha 1 (III) chains of normal human lung were identical with the human skin alpha-chains in all respects examined except that the normal lung chains contained higher levels of hydroxylysine. Examination of collagens obtained from the diseased lung revealed that the content of hydroxylysine of the alpha 1 (I) and the alpha 1 (III) chains appeared to be diminished as compared to the normal lung chains. The values, expressed as residues per 1,000 residues, are 7.1 and 8.3 for the alpha 1 (I) and the alpha 1 (III) chains, respectively, as compared to 10.0 and 11.1 for the alpha-chains from the normal tissue. The chromatographic properties and amino acid and carbohydrate composition of the alpha-chains from the diseased tissue were otherwise indistinguishable from those of normal lung. In addition, isolation and characterization of the CNBr peptides of alpha 1 (I), alpha 2 and alpha 1 (III) from the diseased lung revealed no significant differences from the CNBr peptides from other human tissues reported previously. Normal and diseased lungs were also digested with CNBr, and the resultant alpha 1 (I) and alpha 1 (III) peptides were separated chromatographically. The relative quantities of these peptides indicate that type III collagen constitutes 33% of the total collagen in normal human lung, with the remainder being type I, whereas in idiopathic chronic pulmonary fibrosis, the relative content of type III collagen is markedly
Xu, Xi-Ming; Wang, Fei; Zhou, Xiao-Yi; Liu, Zi-Xuan; Wei, Xian-Zhao; Bai, Yu-Shu; Li, Ming
Abstract The relationship between spinal sagittal alignment and pelvic parameters is well known in adolescent idiopathic scoliosis. However, few studies have reported the sagittal spinopelvic relationship after selective posterior fusion of thoracolumbar/lumbar (TL/L) curves. We evaluated the relationship between spinal sagittal alignment and the pelvis, and analyzed how the pelvic sagittal state is adjusted in Lenke type 5C patients. We conducted a retrospective study of 36 patients with Lenke type 5C curves who received selective posterior TL/L curve fusion. Coronal and spinopelvic sagittal parameters were pre and postoperatively compared. Pearson coefficients were used to analyze the correlation between all spinopelvic sagittal parameters before and after surgery. We also evaluated 3 pelvic morphologies (anteverted, normal, and retroverted) before and after surgery. Preoperatively, the mean pelvic incidence was 46.0°, with a pelvic tilt and sacral slope (SS) of 8.2° and 37.8°, respectively, and 25% (9/36) of patients had an anteverted pelvis, whereas the other 75% had a normal pelvis. Postoperatively, 42% (15/36) of patients had a retroverted pelvis, 53% (19/36) had a normal pelvis, and 2 patients had an anteverted pelvis. Logistic regression analyses yielded 2 factors that were significantly associated with the risk for a postoperative unrecovered anteverted pelvis, including increased lumbar lordosis (LL) (odds ratio [OR] 4.8, P = 0.029) and increased SS (OR 5.6, P = 0.018). Four factors were significantly associated with the risk of a postoperative newly anteverted pelvis, including LL at the final follow-up (OR 6.9, P = 0.009), increased LL (OR 8.9, P = 0.003), LL below fusion (OR 9.4, P = 0.002), and increased SS (OR 11.5, P = 0.001). The pelvic state may be adjusted after selective posterior TL/L curve fusion in Lenke 5C adolescent idiopathic scoliosis patients. It is difficult to improve an anteverted pelvis in patients who have
Hughes, A W
A case of idiopathic chondrolysis of the hip in an adolescent Indian girl is reported. The association between idiopathic chondrolysis of the hip and primary protrusio acetabuli (Otto's pelvis) is discussed. Images PMID:3985694
Sun, Zihan; Dai, Hong
Purpose. To assess the morphology of idiopathic choroidal neovascularization (ICNV) by optical coherence tomography angiography (OCTA) and determine the therapeutic effects of intravitreal antivascular endothelial growth factor (anti-VEGF). Method. Patients with naive ICNV were assessed by spectral domain optical coherence tomography (SD-OCT) and OCTA in this observational study. The timing of observation was before treatment, 1 day after treatment with intravitreal anti-VEGF injection, and 1 month after the treatment. The central retina thickness (CRT) on SD-OCT, selected CNV area, and flow area on OCTA were measured. Results. A total of 17 eyes from 17 patients with ICNV were included in this study. OCTA showed visible irregular choroidal neovascularization with “tree-in-bud” form on outer retinal layer. After treatment, as well as in the 1-day follow-up, CNV decreased in size from the periphery, and the vessel density was reduced. As shown on OCTA, the selected CNV area and flow area were significantly reduced compared to pretreatment. The rate of CNV vessel area changes was higher on OCTA than the changes in CRT on SD-OCT at 1-day and 1-month follow-up. Conclusion. Intravitreal injection of anti-VEGF is effective for idiopathic choroidal neovascularization, and the treatment outcomes are observable after 1 day. OCTA provides a useful approach for monitoring and evaluating the treatment of intravitreal anti-VEGF for CNV. PMID:27471600
Verwoerd, Anouk; Ter Haar, Nienke M; de Roock, Sytze; Vastert, Sebastiaan J; Bogaert, Debby
Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood. The pathogenesis of JIA is thought to be the result of a combination of host genetic and environmental triggers. However, the precise factors that determine one's susceptibility to JIA remain to be unravelled. The microbiome has received increasing attention as a potential contributing factor to the development of a wide array of immune-mediated diseases, including inflammatory bowel disease, type 1 diabetes and rheumatoid arthritis. Also in JIA, there is accumulating evidence that the composition of the microbiome is different from healthy individuals. A growing body of evidence indeed suggests that, among others, the microbiome may influence the development of the immune system, the integrity of the intestinal mucosal barrier, and the differentiation of T cell subsets. In turn, this might lead to dysregulation of the immune system, thereby possibly playing a role in the development of JIA. The potential to manipulate the microbiome, for example by faecal microbial transplantation, might then offer perspectives for future therapeutic interventions. Before we can think of such interventions, we need to first obtain a deeper understanding of the cause and effect relationship between JIA and the microbiome. In this review, we discuss the existing evidence for the involvement of the microbiome in JIA pathogenesis and explore the potential mechanisms through which the microbiome may influence the development of autoimmunity in general and JIA specifically.
Finkelstein, J.S.; Klibanski, A.; Neer, R.M.; Greenspan, S.L.; Rosenthal, D.I.; Crowley, W.F. Jr.
To assess the effect of testosterone deficiency on skeletal integrity in men, we determined bone density in 23 hypogonadal men with isolated gonadotropin-releasing hormone deficiency and compared those values with ones from controls. Cortical bone density, as assessed by single-photon absorptiometry of the nondominant radius, ranged from 0.57 to 0.86 g/cm2 (mean +/- SE, 0.71 +/- 0.02) in patients with fused epiphyses and from 0.57 to 0.67 g/cm2 (mean, 0.61 +/- 0.01) in patients with open epiphyses, both of which were significantly (p less than 0.001) lower than normal. Spinal trabecular bone density, as assessed by computed tomography, was similarly decreased (p less than 0.0001) and ranged from 42 to 177 mg K2HPO4/cm3 (mean, 112 +/- 7). Cortical bone density was at least 2 SD below normal in 16 of 23 men, and 8 men had spinal bone densities below the fracture threshold of 80 to 100 mg K2HPO4/cm3. Osteopenia was equally severe in men with immature and mature bone ages, suggesting that abnormal bone development plays an important role in the osteopenia of men with idiopathic hypogonadotropic hypogonadism.
Gan, Ye; Herzog, Erica L; Gomer, Richard H
Idiopathic pulmonary fibrosis (IPF) is a discrete clinicopathologic entity defined by the presence of usual interstitial pneumonia on high-resolution CT scan and/or open lung biopsy and the absence of an alternate diagnosis or exposure explaining these findings. There are currently no FDA-approved therapies available to treat this disease, and the 5-year mortality is ∼80%. The pyridone derivative pirfenidone has been studied extensively as a possible therapeutic agent for use in this deadly disease. This review will present the unique clinical features and management issues encountered by physicians caring for IPF patients, including the poor response to conventional therapy. The biochemistry and preclinical efficacy of pirfenidone will be discussed along with a comprehensive review of the clinical efficacy, safety, and side effects and patient-centered foci such as quality of life and tolerability. It is hoped that this information will lend insight into the complex issues surrounding the use of pirfenidone in IPF and lead to further investigation of this agent as a possible therapy in this devastating disease. PMID:21339942
Veenhoven, W A; Van der Schans, G S; Nieweg, H O
An immunofluorescence (IF) technique for the detection of antibodies was applied to idiopathic thrombocytopenic purpura (ITP). Serum platelet antibodies were found in thirteen out of twenty-two patients (59 percent) with active disease, but in only four out of fifteen patients (27 percent) who had attained remission. Direct tests for platelet-associated IgG were positive in 36 and 44 percent of these patients respectively. In two cases IgM was observed on the patients' platelet membranes. C3 was not detedted on patients' platelets. Platelet-associated IgG was also found in several other disorders and its occurrence is not therefore diagnostic of ITP. In addition, serum platelet antibodies do not indicate specifically ITP as they may also be due to previous isoimmunization. Antibodies in the sera of patients with ITP generally did not fix Clq and in most cases bound to platelets only in the presence of EDTA. In contrast, isoantibodies often fixed Clq and they had equal affinity for platelets suspended in ACD or EDTA plasma. This was confirmed by quantitative data on IgG binding by platelets obtained by measuring 125-I-labelled protein A uptake. The simplicity of the IF technique permits its routine application and the technique may give useful information with respect to the nature of the antibodies. It must, however, be considered of limited value in the diagnosis of ITP. PMID:6991171
Ruggeri, R M; Imbesi, S; Saitta, S; Campennì, A; Cannavò, S; Trimarchi, F; Gangemi, S
Chronic urticaria is a common condition characterized by recurrent episodes of mast cell-driven wheal and flare-type skin reactions lasting for more than 6 weeks. In about 75% of cases, the underlying causes remain unknown, and the term chronic idiopathic urticaria (CIU) is used to emphasize that wheals develop independently of identified external stimuli. Although CIU affects about 1.0% of the general population, its etiopathogenesis is not yet well understood. It is now widely accepted that in many cases CIU should be regarded as an autoimmune disorder caused by circulating and functionally active IgG autoantibodies specific for the IgE receptor (FceRI) present on mast cells and basophils or for IgE itself. The well-known association of CIU with other autoimmune processes/diseases represents further indirect evidence of its autoimmune origin. Autoimmune thyroid diseases, especially autoimmune thyroiditis, represent the most frequently investigated diseases in association with CIU. Here we review this topic with particular regard to the association between Graves' disease and CIU. The possible pathogenetic mechanisms and the clinical implications of such an association are discussed.
Hara, Hisako; Mihara, Makoto; Seki, Yukio; Koshima, Isao
Primary lymphoedema includes some diseases whose genetic anomaly is detected and others whose pathology is unknown. In this article, we report a lymphatic thrombus found in a limb with lymphoedema during lymphatico-venous anastomosis (LVA). A 32-year-old man was aware of oedema in his left calcar pedis 3 years previously, which appeared without any trigger. Indocyanine green lymphography indicated lymphatic stasis in the left calf and thigh region, and we performed LVA for the patient. During the operation, we found yellow vessels, which were thought to be lymphatic vessels filled with a yellow solid substance, just beneath the superficial fascia at the left ankle. Pathological examination of the thrombi revealed hyaline material mixed with cell components. The cells were categorised as lymphatic endothelial cells, as they were positive for podoplanin. There was no evidence of malignancy. Causes of idiopathic lymphatic thrombus such as this may be one of the causes of so-called primary lymphoedema, and evaluation of such cases may be the first step towards elucidating the mechanisms involved in the development of primary lymphoedema.
Camós, Mireia; Arellano-Rodrigo, Eduardo; Abelló, Deborah; Muntañola, Ana; Ferrer, Ana; Grau, Josep Maria; Cervantes, Francisco
A woman with scleroderma and classic polyarteritis nodosa (PAN) who developed idiopathic myelofibrosis (IM) is reported. The patient presented with a one-year history of weakness, polyarthritis, Raynaud phenomenon, dry cough, and epigastralgia. The diagnosis of scleroderma with visceral involvement was made and treatment with prednisone subsequently started, with good clinical response. Six years later, fever, weight loss, livedo reticularis, and dysesthesias developed. Electromyographic studies were consistent with sensory neuropathy and a sural nerve biopsy yielded the diagnosis of PAN. The patient received cyclophosphamide plus prednisone with a favorable response, but 11 years later she was admitted because of weakness, constitutional symptoms, and abdominal pain due to spleen infarcts. Marked anemia, with aniso-poikilocytosis, tear-drop cells, immature myeloid precursors in the peripheral blood, and an increased serum LDH, was observed and the diagnosis of IM established by bone marrow biopsy. This case represents a new association between IM and an autoimmune disease and supports the hypothesis of an immune basis of IM in some patients.
Clarke, Sarah L N; Sen, Ethan S; Ramanan, Athimalaipet V
Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease of childhood, with JIA-associated uveitis its most common extra-articular manifestation. JIA-associated uveitis is a potentially sight-threatening condition and thus carries a considerable risk of morbidity. The aetiology of the condition is autoimmune in nature with the predominant involvement of CD4(+) T cells. However, the underlying pathogenic mechanisms remain unclear, particularly regarding interplay between genetic and environmental factors. JIA-associated uveitis comes in several forms, but the most common presentation is of the chronic anterior uveitis type. This condition is usually asymptomatic and thus screening for JIA-associated uveitis in at-risk patients is paramount. Early detection and treatment aims to stop inflammation and prevent the development of complications leading to visual loss, which can occur due to both active disease and burden of disease treatment. Visually disabling complications of JIA-associated uveitis include cataracts, glaucoma, band keratopathy and macular oedema. There is a growing body of evidence for the early introduction of systemic immunosuppressive therapies in order to reduce topical and systemic glucocorticoid use. This includes more traditional treatments, such as methotrexate, as well as newer biological therapies. This review highlights the epidemiology of JIA-associated uveitis, the underlying pathogenesis and how affected patients may present. The current guidelines and criteria for screening, diagnosis and monitoring are discussed along with approaches to management.
Wong, Hee-Kit; Tan, Ken-Jin
There have been great advances in the conservative and surgical treatment for adolescent idiopathic scoliosis in the last few decades. The challenge for the physician is the decision for the optimal time to institute therapy for the individual child. This makes an understanding of the natural history and risk factors for curve progression of significant importance. Reported rates of curve progression vary from 1.6% for skeletally mature children with a small curve magnitude to 68% for skeletally immature children with larger curve magnitudes. Although the patient's age at presentation, the Risser sign, the patient's menarchal status and the magnitude of the curve have been described as risk factors for curve progression, there is evidence that the absolute curve magnitude at presentation may be most predictive of progression in the long term. A curve magnitude of 25° at presentation may be predictive of a greater risk of curve progression. Advances in research may unlock novel predictive factors, which are based on the underlying pathogenesis of this disorder. PMID:20165671
Whyte Ferguson, Lucy
This article reports on an observational and treatment study using three case histories to describe common patterns of muscle and fascial asymmetry in adults with idiopathic scoliosis (IS) who have significant scoliotic curvatures that were not surgically corrected and who have chronic pain. Rather than being located in the paraspinal muscles, the myofascial trigger points (TrPs) apparently responsible for the pain were located at some distance from the spine, yet referred pain to locations throughout the thoracolumbar spine. Asymmetries in these muscles appear to tether the spine in such a way that they contribute to scoliotic curvatures. Evaluation also showed that each of these individuals had major ligamentous laxity and this may also have contributed to development of scoliotic curvatures. Treatment focused on release of TrPs found to refer pain into the spine, release of related fascia, and correction of related joint dysfunction. Treatment resulted in substantial relief of longstanding chronic pain. Treatment thus validated the diagnostic hypothesis that myofascial and fascial asymmetries were to some extent responsible for pain in adults with significant scoliotic curvatures. Treatment of these patterns of TrPs and muscle and fascial asymmetries and related joint dysfunction was also effective in relieving pain in each of these individuals after they were injured in auto accidents. Treatment of myofascial TrPs and asymmetrical fascial tension along with treatment of accompanying joint dysfunction is proposed as an effective approach to treating both chronic and acute pain in adults with scoliosis that has not been surgically corrected.
Cornegliani, Luisa; Vercelli, Antonella; Abramo, Francesca
A new papillomatous clinical entity is described affecting the penile mucosa of dogs. The animals, 11 male dogs of different breeds, ageing from 6 to 13 years, were presented for genital mass and occasional haematuria. Surgical incision of the prepuce skin of the anaesthetized dogs showed the presence of single pedunculated, soft, pink-red, cauliflower-like masses arising from the penile mucosa, with diameter ranging from 2 to 8 cm. In all cases, histopathological examination of the excised masses showed normal epithelial differentiation with digitiform expansion of all the layers and elongated rete ridges slanted towards the periphery of the lesion. Evidence of ballooning degeneration or basophilic intranuclear inclusion bodies was not found. Both immunohistochemistry and polymerase chain reaction techniques failed to reveal papillomavirus. According to the histological World Health Organization classification of papillomatous lesions and due to the lack of evidence of a viral origin the masses were identified as idiopathic mucosal penile squamous papillomas. Urinary problems resolved after surgical excision, haematuria was therefore considered secondary to ulceration of the papillated masses.
Ferrari-Marinho, Taissa; Macedo, Eugenia Fialho; Costa Neves, Rafael Scarpa; Costa, Lívia Vianez; Tudesco, Ivanda S S; Carvalho, Kelly C; Carrete, Henrique; Caboclo, Luis Otavio; Yacubian, Elza Marcia; Hamad, Ana Paula
Gastaut type idiopathic childhood occipital epilepsy is an uncommon epileptic syndrome characterised by frequent seizures, most commonly presenting as elementary visual hallucinations or blindness. Other occipital (non-visual) symptoms may also occur. Interictal EEG typically shows occipital paroxysms, often with fixation-off sensitivity. Ictal EEG is usually characterised by interruption by paroxysms and sudden appearance of low-voltage, occipital, fast rhythm and/or spikes. Despite well described clinical and EEG patterns, to our knowledge, there are very few reports in the literature with video-EEG recording of either seizure semiology or fixation-off phenomena. We present a video-EEG recording of a 12-year-old girl with Gastaut type epilepsy, illustrating the interictal and ictal aspects of this syndrome. Our aim was to demonstrate the clinical and neurophysiological pattern of a typical seizure of Gastaut type epilepsy, as well as the fixation-off phenomena, in order to further clarify the typical presentation of this syndrome. [Published with video sequences].
Living with idiopathic short stature (ISS) may entail significant risks to psychological functioning and quality of life. Apparent inconsistency among study findings can be resolved if methodological differences among study designs are taken into account (i.e., definition of particular endpoints, sample selection from clinic or population, source of report, specific or generic assessment instruments, statistical control of confounders). Some individuals fail and others succeed in mastering the challenges of ISS. The principles of multifinality and equifinality may explain the emergence of a broad variation of individuals with ISS as a result of an interaction of the individual medical and auxological features on the one side, and psychosocial risk and protective factors on the other. As a result, patients may show heterogeneous developmental outcomes ranging from clinical psychopathology to development of resilience. A taxonomy of four distinct pathways of adaptation to ISS is delineated as a basis for case formulation and treatment planning. Psychological intervention in ISS includes counseling, cognitive-behavioral therapy and assertiveness training to improve psychological functioning via enhancement of target coping behaviors for critical situations.
Malik, Asma; Hayat, Ghazala; Kalia, Junaid S.; Guzman, Miguel A.
Idiopathic inflammatory myopathies (IIM) are a group of chronic, autoimmune conditions affecting primarily the proximal muscles. The most common types are dermatomyositis (DM), polymyositis (PM), necrotizing autoimmune myopathy (NAM), and sporadic inclusion body myositis (sIBM). Patients typically present with sub-acute to chronic onset of proximal weakness manifested by difficulty with rising from a chair, climbing stairs, lifting objects, and combing hair. They are uniquely identified by their clinical presentation consisting of muscular and extramuscular manifestations. Laboratory investigations, including increased serum creatine kinase (CK) and myositis specific antibodies (MSA) may help in differentiating clinical phenotype and to confirm the diagnosis. However, muscle biopsy remains the gold standard for diagnosis. These disorders are potentially treatable with proper diagnosis and initiation of therapy. Goals of treatment are to eliminate inflammation, restore muscle performance, reduce morbidity, and improve quality of life. This review aims to provide a basic diagnostic approach to patients with suspected IIM, summarize current therapeutic strategies, and provide an insight into future prospective therapies. PMID:27242652
Withers, B G; Loube, D I; Husak, J P
In aviation, it is essential that all aircrew members remain alert and contribute, by their observations and actions, to flight safety. Especially in helicopter operations, crewmembers riding in the rear of the aircraft play an integral role in many aspects of flight, such as take-offs, landings, turns, formation flights, hazard avoidance, situational awareness, military operations, and crew coordination. We present the case of a helicopter crew chief with idiopathic hypersomnia, briefly review the disorder, and give the recent U.S. military aviation experience with sleep disorders. Flight surgeons and aeromedical examiners should be active in considering and diagnosing sleep-related disorders as the aviator or crewmember may not be aware of the disease or may not volunteer the history. A directed history is important in making the diagnosis, as are reports from family and other aircrew members. Referral to a sleep specialist is required in performing objective sleep studies, establishing the diagnosis, recommending treatment, and providing a prognosis. Many sleep disorders are treatable and aeromedically waiverable.
Kobus, Agnieszka; Kierklo, Anna; Sielicka, Danuta; Szajda, Sławomir Dariusz
Juvenile idiopathic arthritis (JIA) is the most common autoimmune inflammatory disease of connective tissue in children. It is characterized by progressive joint destruction which causes preserved changes in the musculoskeletal system. The literature describes fully clinical symptoms and radiological images in different subtypes of JIA. However, there is still a limited number of studies reporting on the medical condition of the oral cavity of ill children. JIA can affect hard and soft tissues of the oral cavity by: the general condition of the child's health, arthritis of the upper limbs, as the result of the pharmacotherapy, changes in secretion and composition of saliva, inflammation of the temporomandibular joint and facial deformity. The study summarizes the available literature on the condition of the teeth and periodontal and oral hygiene in the course of JIA. The presence of diverse factors that modify the oral cavity, such as facial growth, functioning of salivary glands, or the supervision and care provided by adults, prevents clear identification if JIA leads to severe dental caries and periodontal disease. Despite conflicting results in studies concerning the clinical oral status, individuals with JIA require special attention regarding disease prevention and maintenance of oral health.
Consolaro, Alessandro; Giancane, Gabriella; Schiappapietra, Benedetta; Davì, Sergio; Calandra, Serena; Lanni, Stefano; Ravelli, Angelo
Juvenile idiopathic arthritis (JIA), as a chronic condition, is associated with significant disease- and treatment-related morbidity, thus impacting children's quality of life. In order to optimize JIA management, the paediatric rheumatologist has begun to regularly use measurements of disease activity developed, validated and endorsed by international paediatric rheumatology professional societies in an effort to monitor the disease course over time and assess the efficacy of therapeutic interventions in JIA patients.A literature review was performed to describe the main outcome measures currently used in JIA patients to determine disease activity status.The Juvenile Disease Activity Score (JADAS), in its different versions (classic JADAS, JADAS-CRP and cJADAS) and the validated definitions of disease activity and response to treatment represent an important tool for the assessment of clinically relevant changes in disease activity, leading more and more to a treat-to-target strategy, based on a tight and thorough control of the patient condition. Moreover, in recent years, increasing attention on the incorporation of patient-reported or parent-reported outcomes (PRCOs), when measuring the health state of patients with paediatric rheumatic diseases has emerged.We think that the care of JIA patients cannot be possible without taking into account clinical outcome measures and, in this regard, further work is required.
Lourenço, Daniela M R; Buscatti, Izabel M; Lourenço, Benito; Monti, Fernanda C; Paz, José Albino; Silva, Clovis A
Optic neuritis (ON) was rarely reported in juvenile idiopathic arthritis (JIA) patients, particularly in those under anti-tumor necrosis factor alpha blockage. However, to our knowledge, the prevalence of ON in JIA population has not been studied. Therefore, 5,793 patients were followed up at our University Hospital and 630 (11%) had JIA. One patient (0.15%) had ON and was reported herein. A 6-year-old male was diagnosed with extended oligoarticular JIA, and received naproxen and methotrexate subsequently replaced by leflunomide. At 11 years old, he was diagnosed with aseptic meningitis, followed by a partial motor seizure with secondary generalization. Brain magnetic resonance imaging (MRI) and electroencephalogram showed diffuse disorganization of the brain electric activity and leflunomide was suspended. Seven days later, the patient presented acute ocular pain, loss of acuity for color, blurred vision, photophobia, redness and short progressive visual loss in the right eye. A fundoscopic exam detected unilateral papilledema without retinal exudates. Orbital MRI suggested right ON. The anti-aquaporin 4 (anti-AQP4) antibody was negative. Pulse therapy with methylprednisolone was administered for five days, and subsequently with prednisone, he had clinical and laboratory improvement. In conclusion, a low prevalence of ON was observed in our JIA population. The absence of anti-AQP4 antibody and the normal brain MRI do not exclude the possibility of demyelinating disease associated with chronic arthritis. Therefore, rigorous follow up is required.
Glasgow, Connie G.; El-Chemaly, Souheil; Moss, Joel
The primary function of the lymphatic system is absorbing and transporting macromolecules and immune cells to the general circulation, thereby regulating fluid, nutrient absorption and immune cell trafficking. Lymphangiogenesis plays an important role in tissue inflammation and tumour cell dissemination. Lymphatic involvement is seen in lymphangioleiomyomatosis (LAM) and idiopathic pulmonary fibrosis (IPF). LAM, a disease primarily affecting females, involves the lung (cystic destruction), kidney (angiomyolipoma) and axial lymphatics (adenopathy and lymphangioleiomyoma). LAM occurs sporadically or in association with tuberous sclerosis complex (TSC). Cystic lung destruction results from proliferation of LAM cells, which are abnormal smooth muscle-like cells with mutations in the TSC1 or TSC2 gene. Lymphatic abnormalities arise from infiltration of LAM cells into the lymphatic wall, leading to damage or obstruction of lymphatic vessels. Benign appearing LAM cells possess metastatic properties and are found in the blood and other body fluids. IPF is a progressive lung disease resulting from fibroblast proliferation and collagen deposition. Lymphangiogenesis is associated with pulmonary destruction and disease severity. A macrophage subset isolated from IPF bronchoalveolar lavage fluid (BALF) express lymphatic endothelial cell markers in vitro, in contrast to the same macrophage subset from normal BALF. Herein, we review lymphatic involvement in LAM and IPF. PMID:22941884
Sherry, David D; Malleson, Peter N
Idiopathic musculoskeletal pain syndromes in children have a variety of manifestations; they can be diffuse or well localized, constant or intermittent, with or without autonomic symptoms and signs, completely incapacitating or not limiting activities, and they can tax the physician's diagnostic skill. A careful history and examination is usually all that is needed to make a diagnosis, although the differential diagnosis is large and might require laboratory and radiographic investigation. Pain and functional assessment help track the progress with therapy. Intense exercise therapy is associated with the best outcome. Psychologic issues should be evaluated to determine if further psychologic intervention is indicated. The medium-term outcome is probably good for most of these children, but the long-term prognosis is unknown. One must be aware that other manifestations of psychologic problems might emerge. By the time these children and their families see the rheumatologist they are desperate and can be frustrating to work with due to their difficulty in accepting any kind of psychologic element to the pain and its associated disability. Nevertheless, it is rewarding to help the children understand and work through their pain so they can resume normal lives.
Thannickal, Victor J; Flaherty, Kevin R; Martinez, Fernando J; Lynch, Joseph P
Idiopathic pulmonary fibrosis (IPF) is a progressive, fibrosing disease of the distal air spaces of the lung of unknown aetiology. IPF is usually fatal with a median survival of < 3 years. There are currently no effective pharmacotherapeutic agents for the treatment of IPF. In this review, unifying concepts on the pathogenesis of IPF based on understanding of host responses to tissue injury are presented. These host responses involve tightly regulated and contextually orchestrated inflammatory and repair processes. Dysregulation of either of these processes can lead to pathological outcomes. Fibrosis results from an exaggerated or dysregulated repair process that proceeds 'uncontrolled' even after inflammatory responses have subsided. Disease heterogeneity may arise when inflammation and repair are in different (dys)regulatory phases, thus accounting for regional disparity. Usual interstitial pneumonia (UIP), the histopathological correlate of clinical IPF, represents a more fibrotic tissue reaction pattern and for which anti-inflammatory agents are ineffective. Emerging 'antifibrotic' drugs and strategies for UIP/IPF are discussed. The importance of accurately phenotyping a highly heterogeneous disease process that may require individualised and 'combined' therapies is emphasised.
Aldrich, M S
To better define the clinical spectra of narcolepsy and idiopathic hypersomnia, we retrospectively compared clinical and polygraphic findings and questionnaire results in groups of subjects with narcolepsy with or without cataplexy, idiopathic hypersomnia, insufficient sleep syndrome, mild sleep apnea, and excessive daytime sleepiness not otherwise specified. Sleep paralysis and sleep-related hallucinations were most frequent in narcolepsy-cataplexy, but their frequency did not differ between narcolepsy without cataplexy and idiopathic hypersomnia. Mean durations of nocturnal sleep, daytime naps, and morning grogginess were not increased in idiopathic hypersomnia compared with other groups. Among subjects without cataplexy, symptoms of sleep paralysis and sleep-related hallucinations were equally common in subjects with and without frequent sleep-onset REM periods. These findings suggest that the occurrence of these symptoms in subjects without classical narcolepsy-cataplexy is a function of factors other than a propensity for early onset of REM sleep and indicate a need to reevaluate diagnostic criteria for narcolepsy and idiopathic hypersomnia.
Choi, Jung-Kyu; Cha, Eun-Jong; Kim, Kyung-Ah; Won, Yonggwan; Kim, Jung-Ja
From a subject group of pes cavus, the purpose of this study was to evaluate the biomechanical characteristics of lower limbs, based on plantar foot pressure and electromyography (EMG) activities, by the effects on two kind of custom-made insoles. Ten individuals among thirty females with a clinical diagnosis of idiopathic pes cavus (mean age (SD): 22.3 (0.08) years) were selected for the study. The plantar foot pressure data and EMG activities of four lower limb muscles were collected, when subjects walked on a treadmill, under three different experimental conditions. The plantar foot pressure data was analyzed, after the bilateral foot was divided into three areas of masks and into four sections of stance phase, to compare plantar foot pressure. The EMG activities were analyzed for integrated EMG (IEMG) value. The results show that plantar foot pressure concentrated in particular parts is decreased by custom-made insoles. In the case of EMG, all the muscle activities decreased significantly. The custom-made insoles dispersed pressure concentrated by the higher medial longitudinal arch and improved the efficient use of muscles. In particular, the extension structure in the forefoot of custom-made insoles was more efficient for pes cavus. Therefore, it could help patients to walk, by offering support to prevent the disease of pes cavus deformity, and to relieve the burden and fatigue in the lower limbs on gait.
Igarashi, M; Rose, D F; Storgion, S A
Two infants, 18 months and 4 months of age, who were born with bilateral VI and VII nerve palsy (Moebius syndrome) experienced central respiratory failure requiring persistent ventilation. The computed tomography scan showed small brainstem tegmental calcifications. These findings suggest intrauterine brainstem necrosis including the respiratory center and poor prognosis for independent respiration.
Saint-Cyr, J A; Trépanier, L L; Kumar, R; Lozano, A M; Lang, A E
The aim of this study was to examine possible neuropsychological changes in patients with advanced idiopathic Parkinson's disease treated with bilateral deep brain stimulation (DBS) of the subthalamic nucleus (STN). Eleven patients (age = 67 +/- 8 years, years with Parkinson's disease = 15 +/- 3, verbal IQ = 114 +/- 12) were evaluated (in their best 'on state') with tests assessing processes reliant on the functional integrity of frontal striatal circuitry, prior to the procedure (n = 11), at 3-6 months (n = 11) and at 9-12 months (n =10) post-operatively. Six of these patients were older than 69 years. Despite clinical motor benefits at 3-6 months post-operative, significant declines were noted in working memory, speed of mental processing, bimanual motor speed and co-ordination, set switching, phonemic fluency, long-term consolidation of verbal material and the encoding of visuospatial material. Declines were more consistently observed in patients who were older than 69 years, leading to a mental state comparable with progressive supranuclear palsy. 'Frontal' behavioural dyscontrol without the benefit of insight was also reported by half (three of six) of the caregivers of the elderly subgroup. At 9-12 months postoperative, only learning based on multiple trials had recovered. Tasks reliant on the integrity of frontal striatal circuitry either did not recover or gradually worsened over time. Bilateral STN DBS can have a negative impact on various aspects of frontal executive functioning, especially in patients older than 69 years. Future studies will evaluate a larger group of patients and examine the possible reversibility of these effects by turning the DBS off.
Wang, Y.; Toprani, S.; Tang, Y.; Vrabec, T.; Durand, D.M.
In vivo studies of epileptiform discharges in the hippocampi of rodents have shown that bilateral seizure activity can sometimes be synchronized with very small delays (< 2 ms). This observed small time delay of epileptiform activity between the left and right CA3 regions is unexpected given the physiological propagation time across the hemispheres (> 6 ms). The goal of this study is to determine the mechanisms of this tight synchronization with in-vitro electrophysiology techniques and computer simulations. The hypothesis of a common source was first eliminated by using an in-vitro preparation containing both hippocampi with a functional ventral hippocampal commissure (VHC) and no other tissue. Next, the hypothesis that a noisy baseline could mask the underlying synchronous activity between the two hemispheres was ruled out by low noise in-vivo recordings and computer simulation of the noisy environment. Then we built a novel bilateral CA3 model to test the hypothesis that the phenomenon of very small left-to-right propagation delay of seizure activity is a product of epileptic cell network dynamics. We found that the commissural tract connectivity could decrease the delay between seizure events recorded from two sides while the activity propagated longitudinally along the CA3 layer thereby yielding delays much smaller than the propagation time between the two sides. The modeling results indicate that both recurrent and feedforward inhibition were required for shortening the bilateral propagation delay and depended critically on the length of the commissural fiber tract as well as the number of cells involved in seizure generation. These combined modeling/experimental studies indicate that it is possible to explain near perfect synchronization between the two hemispheres by taking into account the structure of the hippocampal network. PMID:24262205
Laberge, J.M.; Nguyen, L.T.; Homsy, Y.L.; Doody, D.P.
Bilaterality is uncommon in Wilms' tumor, being present in 4% to 8% of the cases. We report the combined experience of two children's hospitals in one city over a 20-year period. We encountered nine cases of synchronous bilateral nephroblastoma (National Wilms' Tumor Study 3, stage V). Age at diagnosis ranged from 9 to 41 months (mean 23 months). There were five girls and four boys. Associated findings include nephroblastomatosis in three cases (33%), one of which also had a familial history; undescended testis in two cases; and minor anomalies in two other cases. Surgical treatment consisted of unilateral nephrectomy with contralateral partial nephrectomy or tumorectomy in six cases, nephrectomy with contralateral biopsy only in two cases, and the other patient had bilateral biopsies initially, followed at a later date by partial nephrectomy on one side. All patients received chemotherapy; actinomycin D (AMD) only was used in the oldest case, vincristine and AMD in five cases, to which was added cyclophosphamide in one case and adriamycin in two. Seven patients received radiation therapy. Seven out of the nine patients survived more than 2 years (77%); five are well, off chemotherapy, with no evidence of disease from 4 to 11 years after diagnosis. Two patients suffered from chronic renal failure and one died from complications after renal transplantation more than 19 years after diagnosis. The two patients who died from their disease presented with more advanced tumor. Therefore, the agressiveness of multimodal therapy can be tailored according to stage and histology, and effective chemotherapy allows maximal preservation of renal parenchyma in patients with stage I and II tumors.
Nguyen, Theresa M; Roy, Neil K; Zlupko, George R
Acupuncture is becoming increasingly popular in the United States for a wide variety of uses, ranging from the treatment of chronic back pain to aiding in addiction therapy. As this form of complementary and alternative medicine becomes more prevalent in certain areas of the country, it is of paramount importance that the emergency physician be familiar with its methods and potential complications. In general, acupuncture is perceived as fairly safe. However, it is not without risks or side effects. In this case report, we discuss the history, methods, and common complications of acupuncture in the context of a patient who presented to the Emergency Department (ED) with bilateral pneumothoraces secondary to acupuncture therapy.
Mishra, Anupam; Mishra, Subhash Chandra
This report describes the third case of a true bilateral Juvenile nasopharyngeal angiofibroma (JNA), i.e. two separate JNA arising from both sides simultaneously. The associated multiple recurrences in such a case have not yet been reported. A 21-year-man underwent transpalatal excision and recurred twice. The last 'neo-occurrence' encountered after 2 years was at a different site and was subsequently managed by post-embolization endoscopic resection. A complete report of its clinico-radiological features and management outcome is discussed.
Yildizoglu, Uzeyir; Polat, Bahtiyar; Durmaz, Abdullah
Nasoalveolar cysts, which originate from epithelial remnants of nasolacrimal duct, are nonodontogenic soft tissue lesions of the upper jaw. These cysts are thought to be developmental and are presented with fullness in the upper lip and nose, swelling on the palate, and sometimes nasal obstruction. Because of cosmetic problems, they are often diagnosed at an early stage. These lesions are mostly revealed unilaterally but also can be seen on both sides. In this case report, a patient who complained of nasal obstruction and then diagnosed with bilateral nasoalveolar cysts and treated by sublabial excision is presented and clinical features and treatment approaches are discussed with the review of literature. PMID:27980871
Jabeen, Sadaqat; Raees, Mehnaz
Primary amenorrhea is a common problem. Diagnosis is usually by going through systematic approach of history, examination and investigations. This case had bilateral large endometriotic cysts in the adnexal region. Uterus was normal sized with well-formed endometrium. She underwent laparotomy followed by drainage of endometriotic cysts, stripping and reconstruction of ovaries was performed. Patient was given a trial of combined oral contraceptive pills for two consecutive cycles to observe withdrawal bleeding, but it failed. Till now we are unable to find out such case in literature. Exact case of primary amenorrhea could not be found.
Tan C C K Ho, G H; Bahadzor, B; Praveen, S; Goh, E H; Syahril, A S; Zulkifli, M Z
Gas-forming infection of the kidneys can affect either the parenchyma or the collecting system. They are known as emphysematous pyelonephritis (EPN) and emphysematous pyelitis (EP) respectively. Bilateral EPN is a fairly established entity and numerous articles about this condition have been published. However, much less is known about bilateral EP. We report a rare case of bilateral EP and a literature review of this disease. A 66-year-old woman with long-standing bilateral staghorn calculi presented with bilateral EP and severe sepsis. She was treated with antibiotics and bilateral double-J stents to drain the upper urinary tracts. She recovered after 1 month of intensive care and medical therapy. Percutaneous nephrolithotomy (PCNL) had been scheduled to treat her staghorn calculi. A literature search on MEDLINE and Google Scholar with the terms "bilateral emphysematous pyelitis" only found 1 case report in English and another 2 reports in the Korean language that discussed bilateral EP. The collective experience of these few cases, including the present case, suggests that bilateral EP runs a more benign course than bilateral EPN. It should be diagnosed as soon as possible with computed tomography (CT) scans of the renal system. Current evidence shows that can be treated successfully with timely antibiotics. Drainage of the collecting system either percutaneously or with placement of double-J stents might facilitate recovery.
M Md PhD, Jose C Pachon; Lobo Md, Tasso J; M Md, Enrique I Pachon; P Md, Tomas G Santillana; Tc Pachon Md, Carlos; M Md, Juan C Pachon; V Md, Remy N Albornoz; A Md, Juan C Zerpa; Ortencio Md, Felipe A
Idiopathic Ventricular Premature Contraction (VPC) is currently more routinely referred for electrophysiology evaluation. Usually it carries a good prognosis but, when symptomatic or suspected to produce ventricular dysfunction, will require treatment. Nowadays, RF ablation has great advantages over antiarrhythmic drugs. Classically the outflow tract (right or left), with the typical inferior axis with left (eventually right) bundle brunch block like ECG morphology, is considered the most frequent site of origin for idiopathic VPC, but with the widespread of EP procedures and advancement of technology making possible to map and ablate difficult locations, it is possible to see a growing and changing population referred for idiopathic VPC ablation, displaying that, almost any region of the heart may be source of this kind of arrhythmia that can be successfully treated. A well-planned procedure, with the presumed region of origin settled and employing the current technology and knowledge (tips), will have a high chance of cure.
Margaritopoulos, George A; Harari, Sergio; Caminati, Antonella; Antoniou, Katerina M
For many years, cigarette smoking has been considered as the leading cause of chronic obstructive pulmonary disease and lung cancer. Recently, however, it has also been associated with the development of diffuse interstitial lung diseases. In the latest classification of the major idiopathic interstitial pneumonias (IIP), the term smoking-related IIP has been introduced, including two entities, namely desquamative interstitial pneumonia (DIP) and respiratory bronchiolitis-interstitial lung disease (RB-ILD). Other entities in which smoking has a definite or suggested role include pulmonary Langerhan's cell histiocytosis, smoking-related interstitial fibrosis, combined pulmonary fibrosis and emphysema syndrome and idiopathic pulmonary fibrosis. In this review, we will focus on the mechanisms of smoking-related lung damage and on the clinical aspects of these disorders with the exception of idiopathic pulmonary fibrosis, which will be reviewed elsewhere in this review series.
Saha, Abhijeet; Chopra, Yogiraj; Theis, Jason D; Vrana, Julie A; Sethi, Sanjeev
We report a 12-year-old boy with nephrotic syndrome due to renal AA amyloidosis. The AA amyloidosis was associated with a 3-year history of systemic-onset juvenile idiopathic arthritis. The presence of serum amyloid A protein was confirmed by laser microdissection of Congo Red-positive glomeruli and vessels followed by liquid chromatography and tandem mass spectrometry; this analysis excluded hereditary and familial amyloidosis. Aggressive management of the systemic-onset juvenile idiopathic arthritis resulted in improvement in clinical and laboratory parameters. The case represents an unusual cause of nephrotic syndrome in children. Early diagnosis of renal amyloidosis and management of systemic-onset juvenile idiopathic arthritis is paramount to preventing progression of kidney disease.
Lumachi, Franco; Zulian, Elisa; Scaroni, Carla
Hirsutism affects 10% of women. Hirsute women with normal circulating androgen levels and normal ovarian function (i.e., regular and ovulatory menstrual cycles) are defined as having idiopathic hirsutism, which may affect more than 20% of all hirsute women. In the treatment of idiopathic hirsutism, different medical therapies, alone or in combination, have been reported. The drugs currently available are oral contraceptives, cyproterone acetate androgen receptors blockers (i.e., spironolactone and flutamide), 5alpha-reductase inhibitors (e.g., finasteride [Proscar, Aventis]) and gonadotrophin-releasing hormone analogs. After 1 year of treatment, each drug may improve hirsutism and reduce the Ferriman-Gallwey score by 35-40%. This review analyses the causes of hirsutism and provides information on each therapy and the cost-effective results in patients with idiopathic hirsutism.
Gomes, Samirah Abreu; dos Reis, Luciene Machado; Noronha, Irene Lourdes; Jorgetti, Vanda; Heilberg, Ita Pfeferman
Background and objectives: This study aimed to determine the expression of osteoprotegerin, receptor activator of nuclear factor κB ligand, interleukin-1α, transforming growth factor-β, and basic fibroblast growth factor in stone-forming patients with idiopathic hypercalciuria. Design, setting, participants, & measurements: Immunohistochemical analysis was performed in undecalcified bone samples previously obtained from 36 transiliac bone biopsies of patients who had idiopathic hypercalciuria and whose histomorphometry had shown lower bone volume, increased bone resorption, and prolonged mineralization lag time. Results: Bone expression of receptor activator of nuclear factor κB ligand and osteoprotegerin was significantly higher in patients with idiopathic hypercalciuria versus control subjects. Transforming growth factor-β immunostaining was lower in patients with idiopathic hypercalciuria than in control subjects and correlated directly with mineralization surface. Interleukin-1α and basic fibroblast growth factor staining did not differ between groups. Receptor activator of nuclear factor κB ligand bone expression was significantly higher in patients who had idiopathic hypercalciuria and exhibited higher versus normal bone resorption. Conclusion: A higher expression of receptor activator of nuclear factor κB ligand in bone tissue suggests that increased bone resorption in patients with idiopathic hypercalciuria is mediated by receptor activator of nuclear factor κB ligand. Osteoprotegerin bone expression might have been secondarily increased in an attempt to counteract the actions of receptor activator of nuclear factor κB ligand. The low bone expression of transforming growth factor-β could contribute to the delayed mineralization found in such patients. PMID:18480302
Idiopathic Pulmonary Fibrosis (IPF) is relentless progressive interstitial lung disease (ILD) of unknown etiology. Main pathogenesis is aberrant recovery of epithelial injury and collagen deposition. Majority of IPF patients have been elderly men with smokers. However, there are important differential diagnosis such as fibrotic non-specific interstitial pneumonia (NSIP), Connective Tissue Disease (CTD) associated ILD, chronic hypersensitivity pneumonia (CHP). Clinical point of view, non-productive cough and progressive exertional dyspnea are main symptoms. In addition, scalene muscle hypertrophy, fine crackles and finger clubbing are key findings. Serum marker such as lactate deydrogenase (LDH), Krebs von den Lungeng-6 (KL-6) are sensitive for ILD detection and activity. Pulmonary function test and 6 minute walk test (6MWT) are quite meaningful physiological examination. Serial change of forced vital capacity 6MWT distance predict mortality of IPF. International IPF guideline published recently and highlighted on the importance of high resolution computed tomography (HRCT) findings. Key findings of IPF are honeycombing, traction bronchiectasis and subpleural reticular opacity. IPF is chronic progressive disease. Therefore, tracing disease behavior is crucial and unifying clinical, physiological, imaging information over time provide useful information for physicians.In management, many candidate agent failed to have positive result. Pirfenidone which is anti-fibrotic agent showed to slow the decline of vital capacity and prevent of acute exacerbation. Molecular agent such as nintedanib is promising agent for prevention of progression of IPF. In this review, we review the clinical information of IPF and IPF guideline. Lastly, we show the clinical algorithm of this devastated disease.
Kuperus, Jonneke S; Westerveld, L Anneloes; Rutges, Joost P H J; Alblas, Jacqueline; van Rijen, Mattie H P; Bleys, Ronald L A W; Öner, F Cumhur; Verlaan, Jorrit-Jan
Diffuse idiopathic skeletal hyperostosis (DISH) is a predominantly radiographic diagnosis and histological knowledge of DISH is limited. The aim of this study was to describe the histological characteristics of DISH in the spinal column and to study the relation between DISH and intervertebral disc (IVD) degeneration. Therefore, 10 human cadaveric spines with fluoroscopic evidence of DISH were compared with 10 controls. Plain radiographs and computed tomography (CT) scans were obtained and tissue blocks were resected from three predefined levels of all specimens. The microscopic sections were scored by two blinded observers using a newly developed scoring system specific for characteristics of DISH and a validated scoring system for IVD degeneration. Maximum IVD height was measured on the CT scans. Analyses were performed using Fisher's exact test and Student's t-test. When compared to controls, the right sided sections from DISH specimens showed partial or complete bone bridges, consisting of cortical woven bone, accompanied by morphological changes in the adjoining part of the IVD. Using the histological scoring system for DISH, all parameters were significantly different between the DISH and control group (p < 0.01). The contralateral location did not show differences between the groups. The overall degree of IVD degeneration and height of IVD was comparable for the two groups. The histopathological changes observed in spines with DISH corresponded to the fluoroscopic images and CT scans. The degree of IVD degeneration and IVD height was comparable for both groups, suggesting a limited role for IVD degeneration in the pathogenesis of DISH. © 2016 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 35:140-146, 2017.
Salehi, Marzieh; Salehi, Maryam; Kalbasi, Nader; Hakamifard, Atousa; Salehi, Hassan; Salehi, Mohammad Mahdi; Sharifian, Jalil
Background: Mastitis is an inflammatory disorder in breast tissues due to bacterial factors, mycobacterial infections or autoimmune diseases. Idiopathic granulomatous mastitis (IGM) is a form of mastitis which may be affected by systematic diseases such as sarcoidosis, and infectious causes such as mycobacterium and fungus. This study evaluates the efficacy of medical therapy with a combination of corticosteroid and Azithromycin in patients with IGM. Materials and Methods: This study is a clinical trial research carried out in Alzahra Hospital (Isfahan, Iran) in 2013 on granulomatous mastitis patients. It was administered 250 mg of Azithromycin per 12 hour and 60 mg of Prednisolone per day within 2 weeks. Next, they took 40 mg/day within 8 weeks, and this dosage was tapered during 6 months and the patients clinically and radiologically followed up. The studied patients were examined within 1 week, 2 weeks, 1 month, 3 months, and 6 months, from the beginning of treatment. Results: This study investigated granulomatous mastitis patients in Alzahra hospital in 2013. The mean age of these patients was 33.6 ± 8.9, and their age range was 18–56 years old. Among 26 studied patients, 24 persons (92.3%) according to follow-up the patients by physical examination and sonography responded to treatment of corticosteroid and Azithromycin. The remaining (7.7%) underwent surgery. Treatment periods in case of drug use were respectively, 8.5 ± 0.71 months. Conclusion: Treatment with corticosteroid and Azithromycin is an effective and appropriate treatment for IGM. PMID:28217653
Moore, Terry L.
Juvenile idiopathic arthritis (JIA) reflects a group of clinically heterogeneous, autoimmune disorders in children characterized by chronic arthritis and hallmarked by elevated levels of circulating immune complexes (CICs) and associated complement activation by-products in their sera. Immune complexes (ICs) have been detected in patients’ sera with JIA utilizing a variety of methods, including the anti-human IgM affinity column, C1q solid-phase assay, polyethylene glycol precipitation, Staphylococcal Protein A separation method, anti-C1q/C3 affinity columns, and FcγRIII affinity method. As many as 75% of JIA patients have had IC detected in their sera. The CIC proteome in JIA patients has been examined to elucidate disease-associated proteins that are expressed in active disease. Evaluation of these ICs has shown the presence of multiple peptide fragments by SDS-PAGE and 2-DE. Subsequently, all isotypes of rheumatoid factor (RF), isotypes of anti-cyclic citrullinated peptide (CCP) antibodies, IgG, C1q, C4, C3, and the membrane attack complex (MAC) were detected in these IC. Complement activation and levels of IC correlate with disease activity in JIA, indicating their role in the pathophysiology of the disease. This review will summarize the existing literature and discuss the role of possible protein modification that participates in the generation of the immune response. We will address the possible role of these events in the development of ectopic germinal centers that become the secondary site of plasma cell development in JIA. We will further address possible therapeutic modalities that could be instituted as a result of the information gathered by the presence of ICs in JIA. PMID:27242784
Fiorentino, Marco; Tondolo, Francesco; Bruno, Francesca; Infante, Barbara; Grandaliano, Giuseppe; Gesualdo, Loreto
Background Rituximab represents a valid therapeutic option to induce remission in patients with primary glomerulonephritis. Despite several studies proving its efficacy in improving outcomes in patients with membranous nephropathy (MN), its role in therapeutic protocols is not yet defined. Methods We studied 38 patients with idiopathic MN treated with rituximab (in 13 patients as first-line therapy, in the remaining 25 after conventional immunosuppressive therapy). The patients were analyzed for a 15-month median (interquartile range 7.7–30.2) follow-up, with serial monitoring of 24-h proteinuria, renal function and circulating CD19+ B cells. Results The percentages of patients who achieved complete remission, partial remission and the composite endpoint (complete or partial remission) were 39.5% (15 patients), 36.8% (14 patients) and 76.3% (29 patients), respectively. The 24-h proteinuria was reduced significantly during the entire period of follow-up (from a baseline value of 6.1 to 0.9 g/day in the last visit; P < 0.01), while albuminemia increased constantly (from a baseline value of 2.6 to 3.5 g/dL in the last observation; P < 0.01). Renal function did not significantly change during the observation period. Circulating CD19+ B cells were reduced significantly from the baseline value to the 24-month value (P < 0.01); data about anti-phospholipase A2 receptor antibodies were available in 14 patients, 10 of which experienced a decreasing trend after treatment. No significant adverse events were described during and after infusions. Conclusions The present study confirmed that treatment with rituximab was remarkably safe and allowed for a large percentage of complete or partial remissions in patients with MN. PMID:27994855
Cucchiari, David; Angelini, Claudio
Renal involvement in idiopathic inflammatory myopathies is not as uncommon as was previously thought, as it develops in about one fifth of patients. Clinical presentation includes either acute kidney injury or chronic glomerulonephritis. The former usually develops abruptly during acute phases of rhabdomyolysis: in this case, kidney injury is caused by the toxic effects that myoglobinuria has on the kidney tubules, including cast formation and iron-induced oxidative stress and the development of a third space into the injured muscles. The latter instead has an autoimmune nature, a pleomorphic histological picture, and a more indolent course, with the exception of crescentic glomerulonephritis. Accurate diagnosis and management is crucial for these patients, as timely evaluation and treatment can prevent most of the complications. In the setting of rhabdomyolysis-induced acute kidney injury, the necessity of dialysis can be avoided through aggressive hydration and alkalinization, in order to force diuresis and avoid acidosis and hyperkalemia. In immune-mediated glomerulonephritis, renal biopsy is of undoubtedly value in the diagnostic process and can add prognostic and therapeutic information. In these forms, the development of chronic kidney disease can be prevented or at least delayed by the institution or modification of immunosuppressive treatment. Moreover, the use of drugs that inhibit the renin-angiotensin-aldosterone system and some lifestyle modifications, such as smoking cessation, weight loss, and salt restriction have also value in reducing proteinuria and the progression of kidney damage. In this review, we have summarized the currently available evidence and the different case series in an attempt to provide the readers with the most complete and practical notions that are needed to handle these delicate patients.
Madi, Haifa A; Masri, Ibrahim; Steel, David H
This review evaluates the current surgical options for the management of idiopathic macular holes (IMHs), including vitrectomy, ocriplasmin (OCP), and expansile gas use, and discusses key background information to inform the choice of treatment. An evidence-based approach to selecting the best treatment option for the individual patient based on IMH characteristics and patient-specific factors is suggested. For holes without vitreomacular attachment (VMA), vitrectomy is the only option with three key surgical variables: whether to peel the inner limiting membrane (ILM), the type of tamponade agent to be used, and the requirement for postoperative face-down posturing. There is a general consensus that ILM peeling improves primary anatomical hole closure rate; however, in small holes (<250 µm), it is uncertain whether peeling is always required. It has been increasingly recognized that long-acting gas and face-down positioning are not always necessary in patients with small- and medium-sized holes, but large (>400 µm) and chronic holes (>1-year history) are usually treated with long-acting gas and posturing. Several studies on posturing and gas choice were carried out in combination with ILM peeling, which may also influence the gas and posturing requirement. Combined phacovitrectomy appears to offer more rapid visual recovery without affecting the long-term outcomes of vitrectomy for IMH. OCP is licensed for use in patients with small- or medium-sized holes and VMA. A greater success rate in using OCP has been reported in smaller holes, but further predictive factors for its success are needed to refine its use. It is important to counsel patients realistically regarding the rates of success with intravitreal OCP and its potential complications. Expansile gas can be considered as a further option in small holes with VMA; however, larger studies are required to provide guidance on its use. PMID:26834454
Daccord, Cécile; Maher, Toby M.
Despite major research efforts leading to the recent approval of pirfenidone and nintedanib, the dismal prognosis of idiopathic pulmonary fibrosis (IPF) remains unchanged. The elaboration of international diagnostic criteria and disease stratification models based on clinical, physiological, radiological, and histopathological features has improved the accuracy of IPF diagnosis and prediction of mortality risk. Nevertheless, given the marked heterogeneity in clinical phenotype and the considerable overlap of IPF with other fibrotic interstitial lung diseases (ILDs), about 10% of cases of pulmonary fibrosis remain unclassifiable. Moreover, currently available tools fail to detect early IPF, predict the highly variable course of the disease, and assess response to antifibrotic drugs. Recent advances in understanding the multiple interrelated pathogenic pathways underlying IPF have identified various molecular phenotypes resulting from complex interactions among genetic, epigenetic, transcriptional, post-transcriptional, metabolic, and environmental factors. These different disease endotypes appear to confer variable susceptibility to the condition, differing risks of rapid progression, and, possibly, altered responses to therapy. The development and validation of diagnostic and prognostic biomarkers are necessary to enable a more precise and earlier diagnosis of IPF and to improve prediction of future disease behaviour. The availability of approved antifibrotic therapies together with potential new drugs currently under evaluation also highlights the need for biomarkers able to predict and assess treatment responsiveness, thereby allowing individualised treatment based on risk of progression and drug response. This approach of disease stratification and personalised medicine is already used in the routine management of many cancers and provides a potential road map for guiding clinical care in IPF. PMID:27303645
Yapp, Liam Z; McClymont, Liusaidh; Beggs, Ian; Gaston, Paul; Salter, Donald M
We report the case of a 23-year-old man diagnosed with adult-onset idiopathic chondrolysis of the hip. Chondrolysis of the hip is a disorder most frequently seen in children who have suffered with slipped capital femoral epiphyses. Idiopathic chondrolysis of the hip is extremely rare and to our knowledge, its onset has never been documented in adults aged over 20. With reference to the available medical literature, we summarise the current clinical management of this unusual but important cause of young adult hip pain.
Barone, Patrizia; Pignataro, Rossana; Garozzo, Maria Teresa; Leonardi, Salvatore
IL-6 has a key role in the pathogenesis, clinical manifestations and activity of Systemic Onset Idiopathic Arthritis (sJIA). Tocilizumab (TCZ), the first humanized antihuman IL-6 receptor antibody, inhibits the activity of IL-6. In this review, we summarize the main studies performed, to date, about the use of TCZ in children affected by sJIA refractory to conventional treatment. Nowadays TCZ can be used, alone or in association with Metotrexate, in children older than 2 years. Its use in children younger than 2 years is being investigated. Further study about its use in sJIA and other type of idiopathic arthritis should be done.
Spies, C K; Schwarz-Furlan, S; Hahn, P; Oppermann, J; Unglaub, F
Idiopathic thrombosis of palmar finger veins is rare and women suffer from it almost exclusively. Synovial cysts, epidermoid inclusion cysts, giant cell tumours and haemangiomatous lesions should be considered in the process of diagnosis. We present a 56-year-old woman with idiopathic and symptomatic thrombosis of palmar finger veins. Using the palmar approach the painful veins were identified and excised completely. An uncomplicated wound healing has followed with completely unrestricted and painless range of motion. Surgical excision of the finger vein thrombosis should be considered if there is continuing pain.
Freedman, R R; Sabharal, S C; Desai, N; Wenig, P; Mayes, M
In our study of 28 patients with idiopathic Raynaud's disease, the patients had significantly greater digital blood flow responses to intraarterial phenylephrine and clonidine than did normal control subjects. There were no group differences in finger blood flow responses to body heating, reflex cooling, digital ischemia, or to intraarterial tyramine or isoproterenol. There were also no group differences in blood pressure or heart rate during any procedure. These results suggest that patients with idiopathic Raynaud's disease have increased peripheral vascular alpha-adrenergic receptor sensitivity and/or density compared with normal persons.
Acquired idiopathic generalized anhidrosis (AIGA) is a sweating disorder characterized by inadequate sweating in response to heat stimuli such as high temperature, humidity, and physical exercise. Patients exhibit widespread nonsegmental hypohidrosis/anhidrosis without any apparent cause, but the palms, soles, and axillae are rarely affected. Heat stroke readily develops due to increased body temperature. AIGA commonly affects young males. Approximately 30-60% of patients show complications of cholinergic urticaria, also known as idiopathic pure sudomotor failure or hypohidrotic cholinergic urticaria. Systemic corticosteroids are the most effective therapy, although recurrence is not uncommon.
Bansal, Parul; Nikhil, Vineeta; Kapur, Sonali
Multiple idiopathic external apical root resorption (MIEARR) is a relatively rare condition affecting multiple teeth in a dentition. As the condition is nonsymptomatic, a case is usually detected as an incidental radiographic finding. However, it may cause pain and mobility in severe cases. It is sometimes self-limiting or sometimes may progress to tooth loss. This paper presents a case of external apical root resorption involving multiple teeth in which etiology was not identified, so idiopathic root resorption was considered as a diagnosis of exclusion. PMID:25657532
Makuc, Jana; Sekavčnik, Gregor
Kounis syndrome represents the concurrence of acute coronary syndromes or anginal pain with allergic, hypersensitivity, and anaphylactic reactions. It can be associated with normal coronary angiogram or preexistent coronary pathology. Idiopathic anaphylaxis is defined as anaphylaxis without any identifiable precipitating agent or event. We present a case of male who experienced attacks of dyspnoea, hypoxemia, hypotension, purple-red skin, and chest pain over several years. He was diagnosed with idiopathic anaphylaxis. Based on the pattern of chest pain of ischemic origin during the attacks he was retrospectively diagnosed with Kounis syndrome. PMID:28255467
Bussone, G; Grazzi, L
The aim of this paper is to review hypotheses regarding pain mechanisms in headache and relationships between headache pain and the brain's emotional network. There is evidence that chronic pain in idiopathic headaches is, in part, an emotional response induced by alterations in the homeostasis of the interoceptive system--a system that integrates nociceptive information with the emotional network (mediating emotional awareness). These findings suggest that idiopathic headaches are probably due to both an altered pain matrix on the one hand, and an altered affective-cognitive state on the other.
Shin, Sung Hyun; Kim, Young Mi; Kim, Hye Young; Lee, Yoon Jin; Nam, Sang Ook
Idiopathic intracranial hypertension is defined as increased intracranial pressure of unknown origin. Idiopathic intracranial hypertension associated with polycystic ovarian syndrome (PCOS) is a rare condition in adolescence. We report the case of a 14-year-old girl with sudden onset of decreased visual acuity, headache and menstrual irregularity. Clinical neurological examination and magnetic resonance imaging of the brain and orbit were normal. Lumbar puncture demonstrated an increased opening pressure of 31 cm H2O. Gynecologic investigation indicated PCOS. Her symptoms improved with medical and surgical treatment for the underlying PCOS.
Baddini-Martinez, José; Baldi, Bruno Guedes; da Costa, Cláudia Henrique; Jezler, Sérgio; Lima, Mariana Silva; Rufino, Rogério
Idiopathic pulmonary fibrosis is a type of chronic fibrosing interstitial pneumonia, of unknown etiology, which is associated with a progressive decrease in pulmonary function and with high mortality rates. Interest in and knowledge of this disorder have grown substantially in recent years. In this review article, we broadly discuss distinct aspects related to the diagnosis and treatment of idiopathic pulmonary fibrosis. We list the current diagnostic criteria and describe the therapeutic approaches currently available, symptomatic treatments, the action of new drugs that are effective in slowing the decline in pulmonary function, and indications for lung transplantation. PMID:26578138
Vijay, Vipul; Sud, Alok; Mehtani, Anil
Tuberculosis, or phthisis (consumption) as it was popularly known in the Greek era, has been endemic in Southeast Asia and Sub-Saharan Africa; however, the human immunodeficiency virus epidemic has seen the re-emergence of this disease in the areas in which it was not very commonly reported. With this, the need for understanding and treatment of rare presentations of tuberculosis has become of paramount importance to achieve the World Health Organization millennium goal of a "reversal of incidence by 2015." Foot involvement has been reported in 0.1% to 0.3% of extrapulmonary cases. Multifocal lesions have an incidence of <10% in osteoarticular tuberculosis. Bilateral feet involvement in multifocal tuberculosis has not yet been reported in either children or adults in published studies. We report a case of tuberculosis with lesions in the bilateral metatarsals, the occurrence of which is very rare. The diagnosis was mainly histopathologic owing to the paucibacillary nature of the disease. Early identification and treatment with antitubercular drugs will normally result in a good cosmetic and functional result.
Stefanek, M E
At present, the care of women at increased risk of developing breast cancer poses a clinical dilemma and remains an area of controversy. A number of investigators have addressed the pros and cons of prophylactic mastectomy versus close follow-up, utilizing annual mammography, semiannual or even more frequent physical examinations of the breast, and proficient monthly breast self-examinations. Recent efforts to isolate a gene (BRCA1) on chromosome 17q12-21 raise additional concerns about the management of women testing positive for BRCA1 mutations. These women are estimated to have an 85% lifetime risk of developing breast cancer. Testing for BRCA1 mutation carriers may soon be available for population screening. This article describes preliminary studies investigating health care provider and patient perceptions of bilateral prophylactic mastectomy. In addition, a number of research questions remain regarding the efficacy and utilization of bilateral prophylactic mastectomy as a treatment option for women at increased risk of developing breast cancer. These women include those testing positive for BRCA1 mutations. In addition, women with a strong family history opting against testing for BRCA1 mutations may express interest in surgery.
Couto, Cristóbal; Hurtado, Erika; Faingold, Dana; Demetrio, Carmen; Schlaen, Ariel; Zas, Marcelo; Zarate, Jorge; Rosetti, Silvia; de Lima, Andrea Paes; Croxatto, Juan Oscar; Chiaradía, Pablo; Burnier, Miguel N.
Bilateral Fuchs uveitis associated with vitreous infiltration and posterior segment involvement requires a thorough diagnostic evaluation. The lack of well-defined diagnostic criteria makes identification of this entity difficult. The aim of this case report was to present the characteristics of a patient with atypical Fuchs uveitis and the procedures needed to rule out the differential diagnosis with specific attention to the utility of in vivo confocal microscopy (IVCM). Case Report One case of chronic bilateral uveitis with severe vitreous opacities is presented. After extensive systemic workup, including vitrectomy, the case had no identifiable systemic etiology. IVCM of the cornea revealed the presence of dendritiform keratic precipitates. Conclusion The diagnosis of Fuchs uveitis is based on clinical findings as no confirmatory laboratory tests are available. A high index of suspicion is key to an early diagnosis, especially in the cases with vitreous opacities and posterior segment manifestations. Auxiliary tests such as IVCM may aid the clinician in the diagnosis of Fuchs uveitis. PMID:26483668
Parkash, Vivak; Mudhar, Hardeep Singh; Wagner, Bart E.; Raoult, Didier; Batty, Ruth; Lepidi, Hubert; Burke, John; Collini, Paul; de Silva, Thushan
Purpose To describe the clinical features of a Caucasian female patient with a history of treated gastrointestinal Whipple's disease (WD) who developed new-onset diplopia, with a description of the histopathological features of the extraocular muscle biopsies. Methods A previously fit 38-year-old Caucasian female presented with acute-onset diplopia after being on a sustained medication regime for biopsy-proven gastrointestinal WD. A magnetic resonance imaging scan of her orbits with gadolinium revealed diffuse enhancement of the bellies of the extraocular muscles bilaterally, particularly the medial rectus, superior rectus, and superior oblique muscles, consistent with an infiltrative myositis. She underwent unilateral extraocular muscle biopsies. Results The extraocular muscle biopsies contained macrophages between the muscle fibres. These contained periodic acid-Schiff-positive cytoplasmic granules. Immunohistochemistry with an antibody raised to Tropheryma whipplei showed positive staining of the same macrophages. Transmission electron microscopy confirmed the presence of effete T. whipplei cell membranes in lysosomes. Conclusion This case describes bilateral WD-associated extraocular muscle myositis. The exact mechanism for this unusual presentation is unclear, but both a WD-associated immune reconstitution inflammatory syndrome and treatment failure are possibilities, with a good response observed to antibiotic therapy and adjunctive corticosteroids.
Pellicer Costa, Juan José; Dusza, Jacek J.
The paper presents the results of gait parameters as a function of unilateral weight. The object of the research was a woman walking on a stationary surface and carrying in his hand weights from 0 to 15 kg. Her movement was recorded by 6 cameras recording the location of 34 markers placed at appropriate points in the body. 3D reconstruction was performed for each of the reflecting markers. Tested signals were changes in the value the joint angles of ankle, knee and hip. On the basis of about 6 cycles of movement of each load, a model for the average gait cycle was developed. The result of the experiments are graphs of changes the joint angles as a function of time, bilateral cyclograms, synchronized bilateral cyclograms and regression lines. The conclusion of the study is to determine how one-sided load affects gait asymmetry. Simple and easy to interpret method of presentation of results were also shown. Studies were conducted using VICON system.
García-Mejido, José Antonio; Delgado-Jiménez, Carmen; Gutiérrez-Palomino, Laura; Sánchez-Sevilla, Miguel; Iglesias-Bravo, Eva; Caballero-Fernández, Virginia
antecedentes: el cáncer de mama en el hombre es una enfermedad con baja incidencia, que se reduce aún más cuando es bilateral sincrónica. Existen pocas publicaciones en los últimos años. Objetivo: establecer pautas para el tratamiento de este cáncer, aunque sea infrecuente. Caso clínico: paciente masculino de 75 años de edad, con tumores en ambas mamas, que se le resecaron completamente con exéresis de ganglios palpables. El estudio histopatológico informó que se trataba de un carcinoma ductal infiltrante no especificado. Se indicó tratamiento adyuvante con tamoxifeno y radioterapia; en la actualidad está libre de enfermedad. Conclusiones: el carcinoma mamario bilateral sincrónico en el varón es una enfermedad poco frecuente. Su tratamiento principal es la cirugía, de ahí la importancia del diagnóstico temprano. En la mayoría de los casos se requiere quimioterapia y radioterapia adyuvante porque suelen diagnosticarse en un estadio avanzado.
Bilateral bifid mandibular condyle is a rarely seen malformation. The aetiology of bifid condyle is not completely understood, although developmental anomaly, traumas, condylar fracture, teratogenic embryopathy and surgical condylectomy may all be causative factors. Although a few studies on human dried skulls tried to shed light on this entity it remains obscure. As most bifid condyle subjects have no complaint related to temporamandibular joint(TMJ), the cases are generally diagnosed through incidental radiographic findings. The case of a 54-year-old female is presented. In a panoramic radiograph obtained after a clinical examination, bilateral bifid mandibular condyle was observed. The open-closed lateral radiograph of the TMJ (obtained using the TMJ-specific program of the panoramic device) demonstrated duplication of the right and left condyles. In order to better evaluate the TMJ morphology and to eliminate pathologies such as fractures that might be missed with conventional radiographs, a computed tomography scan was also obtained. The joint head orientation was observed in the mediolateral direction. The case is discussed in the context of the relevant literature. Until large population-based studies are undertaken and further experimental studies are performed, bifid condyle will remain an incidental finding of anatomic variation rather than a clinically informative observation.
Webster, D. R.; Rahman, S.; Dasi, L. P.
Motivation is drawn from the need to determine the sensory cues that animals such as blue crabs and lobsters use to track chemical odor plumes to locate food or mates. Major steps forward with this difficult problem can only be achieved through an appreciation of the spatial and temporal variation of concentration fields and the information content available to a forager in the plume. Here we discuss the usefulness of bilateral comparison to an animal tracking a turbulent plume. Instantaneous concentration fields of a chemical plume diffusing in a fully-developed turbulent open channel flow are measured using planar laser-induced fluorescence (PLIF). The plume is released iso-kinetically 25 mm above the smooth bed (z+ = 90), thus transport is mainly due to advection and ambient turbulence. A spatial correlation function in the spanwise direction is a dramatic indicator of the relative position of the centerline and distance from the source. The relative direction of the plume centerline can be estimated from an instantaneous bilateral comparison provided the sensors are separated by a distance that is relatively large compared to the spanwise integral length scale based on the spatial correlation function.
Frejo, Lidia; Soto-Varela, Andres; Santos-Perez, Sofía; Aran, Ismael; Batuecas-Caletrio, Angel; Perez-Guillen, Vanesa; Perez-Garrigues, Herminio; Fraile, Jesus; Martin-Sanz, Eduardo; Tapia, Maria C; Trinidad, Gabriel; García-Arumi, Ana María; González-Aguado, Rocío; Espinosa-Sanchez, Juan M; Marques, Pedro; Perez, Paz; Benitez, Jesus; Lopez-Escamez, Jose A
Meniere disease (MD) is a heterogeneous clinical condition characterized by sensorineural hearing loss, episodic vestibular symptoms, and tinnitus associated with several comorbidities, such as migraine or autoimmune disorders (AD). The frequency of bilateral involvement may range from 5 to 50%, and it depends on the duration of the disease. We have performed a two-step cluster analysis in 398 patients with bilateral MD (BMD) to identify the best predictors to define clinical subgroups with a potential different etiology to improve the phenotyping of BMD and to develop new treatments. We have defined five clinical variants in BMD. Group 1 is the most frequently found, includes 46% of patients, and is defined by metachronic hearing loss without migraine and without AD. Group 2 is found in 17% of patients, and it is defined by synchronic hearing loss without migraine or AD. Group 3, with 13% of patients, is characterized by familial MD, while group 4, that includes 12% of patients, is associated by the presence of migraine in all cases. Group 5 is found in 11% of patients and is defined by AD. This approach can be helpful in selecting patients for genetic and clinical research. However, further studies will be required to improve the phenotyping in these clinical variants for a better understanding of the diverse etiological factors contributing to BMD.
Frejo, Lidia; Soto-Varela, Andres; Santos-Perez, Sofía; Aran, Ismael; Batuecas-Caletrio, Angel; Perez-Guillen, Vanesa; Perez-Garrigues, Herminio; Fraile, Jesus; Martin-Sanz, Eduardo; Tapia, Maria C.; Trinidad, Gabriel; García-Arumi, Ana María; González-Aguado, Rocío; Espinosa-Sanchez, Juan M.; Marques, Pedro; Perez, Paz; Benitez, Jesus; Lopez-Escamez, Jose A.
Meniere disease (MD) is a heterogeneous clinical condition characterized by sensorineural hearing loss, episodic vestibular symptoms, and tinnitus associated with several comorbidities, such as migraine or autoimmune disorders (AD). The frequency of bilateral involvement may range from 5 to 50%, and it depends on the duration of the disease. We have performed a two-step cluster analysis in 398 patients with bilateral MD (BMD) to identify the best predictors to define clinical subgroups with a potential different etiology to improve the phenotyping of BMD and to develop new treatments. We have defined five clinical variants in BMD. Group 1 is the most frequently found, includes 46% of patients, and is defined by metachronic hearing loss without migraine and without AD. Group 2 is found in 17% of patients, and it is defined by synchronic hearing loss without migraine or AD. Group 3, with 13% of patients, is characterized by familial MD, while group 4, that includes 12% of patients, is associated by the presence of migraine in all cases. Group 5 is found in 11% of patients and is defined by AD. This approach can be helpful in selecting patients for genetic and clinical research. However, further studies will be required to improve the phenotyping in these clinical variants for a better understanding of the diverse etiological factors contributing to BMD. PMID:27822199
Emblom-Callahan, Margaret C; Chhina, Mantej K; Shlobin, Oksana A; Ahmad, Shahzad; Reese, Erika S; Iyer, Eswar P R; Cox, Daniel N; Brenner, Renee; Burton, Nelson A; Grant, Geraldine M; Nathan, Steven D
Activated fibroblasts are the central effector cells of the progressive fibrotic process in idiopathic pulmonary fibrosis (IPF). Characterizing the genomic phenotype of isolated fibroblasts is essential to understanding IPF pathogenesis. Comparing the genomic phenotype of non-cultured pulmonary fibroblasts from advanced IPF patients' and normal lungs revealed novel genes, biological processes and concomitant pathways previously unreported in IPF fibroblasts. We demonstrate altered expression in proteasomal constituents, ubiquitination-mediators, Wnt, apoptosis and vitamin metabolic pathways and cell cycle regulators, suggestive of loss of cellular homeostasis. Specifically, FBXO32, CXCL14, BDKRB1 and NMNAT1 were up-regulated, while RARA and CDKN2D were down-regulated. Paradoxically, pro-apoptotic inducers TNFSF10, BAX and CASP6 were also found to be increased. This comprehensive description of altered gene expression in isolated IPF fibroblasts underscores the complex biological processes characteristic of IPF and may provide a foundation for future research into this devastating disease.
Ninet, J.; Annat, G.; Boisson, D.; Holzhapfel, L.; Vincent, M.; Peyrin, L.; Michel, D.; Schott, B.; Devic, M.; Levrat, R.
Eight cases of Shy-Drager syndrome and three of Bradbury-Eggleston idiopathic orthostatic hypotension were examined. In all cases, examination of circulatory reflexes showed major dysfunction of the sympathetic vasoconstrictor system. Anomalies in the vagal cardiomoderator system were less constant. Normal urinary elimination of catecholamines was recorded daily. Characteristically, no elevation of blood or urine norepinephrine levels were found in orthostatism. Insulin hypoglycemia normally raised urinary adrenalin elimination in three of ten patients. Plasma dopa-beta-hydroxylase activity was normal. Renin-angiotensin-aldosterone system showed variable activity at basal state but usually rose during orthostatism. On the average, very low homovanillic acid levels were found in cerebrospinal fluid before and after probenecid; hydroxyindolacetic acid was normal. Cerebral autoregulation had deteriorated in two of four cases. Physiopathologically the two clinical types are indistinguishable with or without central neurological signs.
Pérez-de-Heredia-Torres, Marta; Martínez-Piédrola, Rosa M; Cigarán-Méndez, Margarita; Ortega-Santiago, Ricardo; Fernández-de-Las-Peñas, César
The aim of the current study was to investigate fine motor control ability and manual dexterity women with fibromyalgia syndrome (FMS) without symptoms in the upper extremity compared to healthy women. Subtests of the Purdue Pegboard Test (one-hand, bilateral and assembly) and of the Jebsen-Taylor hand-function test (writing, turning cards, picking up small, light and large heavy objects, simulated feeding and stacking checkers) were evaluated bilaterally in 20 women with FMS (aged 35-55 years) without symptoms in the upper limb and 20 age- and hand dominance-matched healthy women. Differences between sides and groups were analysed with several analysis of variance (ANOVA). The ANOVA revealed significant differences between groups (P < 0.001) and sides (P = 0.007) for one-hand pin placement subtest: women with FMS showed bilateral worse scores than controls. Patients also exhibited significantly lower scores in bilateral pin placement and assembly subtests when compared to healthy controls (P < 0.001). The ANOVA also revealed significant differences between groups for writing, turning over cards, picking up small objects, stacking checkers, picking up large light objects and picking up large heavy objects (all, P < 0.001): women with FMS needed more time for these subtests than healthy women with both hands. No difference for simulated feeding was found between groups. Our findings revealed bilateral deficits in fine motor control ability and manual dexterity in patients with FMS without symptoms in the upper extremity. These deficits are not related to the clinical features of the symptoms supporting an underlying central mechanism of altered motor control.
de la Llave-Rincón, Ana Isabel; Fernández-de-las-Peñas, César; Fernández-Carnero, Josué; Padua, Luca; Arendt-Nielsen, Lars; Pareja, Juan A
The aim of the current study was to evaluate bilaterally warm/cold detection and heat/cold pain thresholds over the hand/wrist in patients with carpal tunnel syndrome (CTS). A total of 25 women with strictly unilateral CTS (mean 42 +/- 10 years), and 20 healthy matched women (mean 41 +/- 8 years) were recruited. Warm/cold detection and heat/cold pain thresholds were assessed bilaterally over the carpal tunnel and the thenar eminence in a blinded design. Self-reported measures included both clinical pain history (intensity, location and area) and Boston Carpal Tunnel Questionnaire. No significant differences between groups for both warm and cold detection thresholds in either carpal tunnel or thenar eminence (P > 0.5) were found. Further, significant differences between groups, but not between sides, for both heat and cold pain thresholds in both the carpal tunnel and thenar eminence were found (all P < 0.001). Heat pain thresholds (P < 0.01) were negatively correlated, whereas cold pain thresholds (P < 0.001) were positively correlated with hand pain intensity and duration of symptoms. Our findings revealed bilateral thermal hyperalgesia (lower heat pain and reduced cold pain thresholds) but not hypoesthesia (normal warm/cold detection thresholds) in patients with strictly unilateral CTS when compared to controls. We suggest that bilateral heat and cold hyperalgesia may reflect impairments in central nociceptive processing in patients with unilateral CTS. The bilateral thermal hyperalgesia associated with pain intensity and duration of pain history supports a role of generalized sensitization mechanisms in the initiation, maintenance and spread of pain in CTS.
Kim, Ji Hwa; Roh, Kyung Jin; Suh, Sang Hyun; Lee, Kyung-Yul
Bilateral deafness is a rare but possible symptom of vertebrobasilar ischemia. We report a case of sudden bilateral sensorineural hearing loss caused by bilateral vertebral artery (VA) occlusion which dramatically improved after stenting. A 54-year-old man was admitted with sudden onset of bilateral deafness, vertigo, and drowsy mental status. Brain diffusion-weighted MRI showed acute infarction involving both the posterior inferior cerebellar artery and left posterior cerebral artery territory. Cerebral angiography showed bilateral distal VA occlusion, and emergency intracranial stenting was performed in the left VA. After reperfusion therapy his symptoms gradually improved, including hearing impairment. Endovascular stenting may be helpful in a patient with sudden deafness caused by bilateral VA occlusion. PMID:25697296
Simon, Helen J
This article is concerned with the evolution and pros and cons of bilateral amplification. Determining whether a bilateral hearing aid fitting is superior to that of a monaural hearing aid is a long-standing question; for this reason, the trend toward bilateral amplification has been slow. However, it is now assumed that bilateral amplification has significant advantages over monaural amplification in most cases, a view that is supported by our localization results. In this article, we will address the advantages of bilateral hearing aids and reveal some new localization data that show that most listeners with bilateral amplification, when tested unaided, as well as normal-hearing listeners manifested very high degrees of symmetry in their judgments of perceived angle while listeners who routinely use monaural amplification and those with asymmetric hearing loss had relatively large asymmetries. These data show that asymmetry in localization judgments is a much more sensitive indicator of abnormal localization ability than the magnitude of localization errors.
Karampatsas, Konstantinos; Patel, Himanshu; Basheer, Sheikh N; Prendergast, Andrew J
A previously well 12-year-old boy presented with a 2-week history of headache, nausea, vomiting and left-sided weakness. He subsequently developed meningism, right abducens nerve palsy, persistent papilloedema and reduced visual acuity in association with a bilateral macular star, consistent with neuroretinitis. Cerebrospinal fluid (CSF) examination indicated chronic meningitis and serological testing confirmed recent Mycoplasma pneumoniae infection, although PCR in CSF was negative. He was treated for aseptic meningitis with ceftriaxone, aciclovir, azithromycin and acetazolamide for intracranial hypertension, with gradual improvement in clinical condition and visual acuity over several weeks. This is the first report of M. pneumoniae chronic meningitis further complicated with bilateral neuroretinitis and intracranial hypertension. Evidence of central nervous system inflammation in the absence of direct infection suggests an immune-mediated pathophysiology. Although the use of macrolides with antibiotic and immunomodulatory activity might be beneficial, it was not possible to ascertain whether it influenced clinical recovery in this case.
Kanemoto, Nobuko; Kanemoto, Katsuyoshi; Kamoda, Tomohiro; Hasegawa, Makoto; Arinami, Tadao
We describe a female infant with bilateral facial paralysis and abducens palsy. To the best of our knowledge, this is the first report of Moebius syndrome presenting with congenital bilateral vocal cord paralysis (CBVCP). Although CBVCP can be part of a recognizable syndrome, i.e. Down syndrome, 22q deletion syndrome, Robinow's syndrome and cerebro-oculo-facio-skeletal syndrome, no reports of Moebius syndrome with CBVCP were found in the literature. CBVCP is often associated with central nervous system abnormalities. However, our patient had no detectable brain abnormalities. The etiology of Moebius syndrome remains unknown. It is interesting that the clinical manifestations of Moebius syndrome can include CBVCP. However, the pathophysiology of CBVCP is unknown and further investigations into the etiology of Moebius syndrome are required.
Arora, Nikhil; Juneja, Ruchika; Meher, Ravi; Bhargava, Eishaan K
Stridor in paediatric age group is not an uncommon presentation to the ENT emergency. The range of differential diagnosis is vast. The presentation may vary from noisy breathing to severe respiratory distress and apnea. Early and meticulous diagnosis is crucial for the management as the condition may be life threatening. We report a rare case series of 3 infants with Arnold Chiari Malformation who presented to the hospital with stridor and were diagnosed with bilateral vocal cord palsy. These 3 infants had similar underlying neurological condition with hydrocephalus and raised intracranial pressure. Chiari malformation is the one of the most common congenital central nervous system anomaly associated with bilateral vocal cord paralysis. However, the presentation is rare. This article, thus, emphasizes the significance of early diagnosis and immediate management of this condition.
Arora, Nikhil; Meher, Ravi; Bhargava, Eishaan K.
Stridor in paediatric age group is not an uncommon presentation to the ENT emergency. The range of differential diagnosis is vast. The presentation may vary from noisy breathing to severe respiratory distress and apnea. Early and meticulous diagnosis is crucial for the management as the condition may be life threatening. We report a rare case series of 3 infants with Arnold Chiari Malformation who presented to the hospital with stridor and were diagnosed with bilateral vocal cord palsy. These 3 infants had similar underlying neurological condition with hydrocephalus and raised intracranial pressure. Chiari malformation is the one of the most common congenital central nervous system anomaly associated with bilateral vocal cord paralysis. However, the presentation is rare. This article, thus, emphasizes the significance of early diagnosis and immediate management of this condition. PMID:27790480
HAO, XIAOSHENG; WANG, JIANGTAO; LIU, SONGYAN; CHEN, YINBO; ZHANG, YAN; HAO, YUNPENG
3-Hydroxy-3-methylglutaric aciduria (3-HMG, OMIN 246450) is a rare autosomal recessive metabolic disorder caused by a deficiency of 3-hydroxy-3-methylglutaryl-CoA lyase, a key enzyme in leucine metabolism and ketone body synthesis. Acute episodes of 3-HMG may be triggered by fasting or infection, and symptoms include vomiting, diarrhea, lethargy and hypotonia. If left untreated, prolonged hypoglycemia and metabolic acidosis may cause breathing problems, seizures, and coma. In addition, 3-HMG is associated with damage to the central nervous system, and there are several reports of white matter abnormality or cerebral atrophy. The presence of bilateral basal ganglia damage in 3-HMG has been rarely reported. Here, we present a case report of a 20-month old male with severe 3-HMG and prominent bilateral lesions in the basal ganglia. PMID:27284350
Poureisa, Masoud; Daghighi, Mohammad Hossein; Mazaheri-Khameneh, Ramin; Ghadirpour, Ali
We present 16 years old girl with primary breast lymphoma involving the both breasts simultaneously and co-incidence of central nervous system (CNS) involvement which originated from the breast, in addition to evidence of lymphoma in multi-organ such as skin, mediastinum and some abdominal viscera. Bilaterality has been observed in some series, but reports are few and series are often very small. Radiologic features may be indistinguishable from those of the other breast malignancies, but it can be concluded that primary involvement of the breast with lymphoma should be considered when breast mass or inflammatory changes occur in a patient without any previously diagnosed lymphoma. The clinical behavior, imaging and therapeutic procedures are discussed. The findings of this report may have clinical impact on oncologists or that may alter the disease concept of bilateral breast lymphoma.
Toupet, M; Pialoux, P
Vertical nystagmus provoked by simultaneous bilateral caloric stimulation has been known since 1907 (Bàràny). However, if a controlled and calibrated injection os water at 44 degrees C or 30 degrees C for 30 seconds at a flow rate of 250 ml/minute is given in normal subjects, this stimulus is insufficient to provoke a response, whilst in subjects with a central vestibular disorder there appear upward vertical movements with hot stimulation and downward movements with cold stimulation. The degree of this response is proportional to the degree of horizontal nystagmic responses to classical unilateral caloric tests. The authors feel that vertical nystagmic responses to simultaneous bilateral caloric stimulation reflect a loss of control of the vestibular reflex activity of the superior semicircular canals.
Galois, L; Meuley, E; Pfeffer, F; Mainard, D; Delagoutte, J P
We report a rare injury in an 18-year-old woman who sustained posterior bilateral hip dislocation with sacro-iliac dislocation after a high energy motor vehicle accident. She was treated by closed reduction and skeletal traction. Bilateral traumatic hip dislocation is an uncommon occurrence. Rarer still is bilateral traumatic hip dislocation associated with sacro-iliac dislocation because it combines two different mechanisms of trauma. (Hip International 2002; 1: 47-9).
Agarwal, Ashok; Sharma, Rakesh; Durairajanayagam, Damayanthi; Cui, Zhihong; Ayaz, Ahmet; Gupta, Sajal; Willard, Belinda; Gopalan, Banu; Sabanegh, Edmund
Among infertile men, a diagnosis of unilateral varicocele is made in 90% of varicocele cases and bilateral in the remaining varicocele cases. However, there are reports of under-diagnosis of bilateral varicocele among infertile men and that its prevalence is greater than 10%. In this prospective study, we aimed to examine the differentially expressed proteins (DEP) extracted from spermatozoa cells of patients with bilateral varicocele and fertile donors. Subjects consisted of 17 men diagnosed with bilateral varicocele and 10 proven fertile men as healthy controls. Using the LTQ-orbitrap elite hybrid mass spectrometry system, proteomic analysis was done on pooled samples from 3 patients with bilateral varicocele and 5 fertile men. From these samples, 73 DEP were identified of which 58 proteins were differentially expressed, with 7 proteins unique to the bilateral varicocele group and 8 proteins to the fertile control group. Majority of the DEPs were observed to be associated with metabolic processes, stress responses, oxidoreductase activity, enzyme regulation, and immune system processes. Seven DEP were involved in sperm function such as capacitation, motility, and sperm-zona binding. Proteins TEKT3 and TCP11 were validated by Western blot analysis and may serve as potential biomarkers for bilateral varicocele. In this study, we have demonstrated for the first time the presence of DEP and identified proteins with distinct reproductive functions which are altered in infertile men with bilateral varicocele. Functional proteomic profiling provides insight into the mechanistic implications of bilateral varicocele-associated male infertility. PMID:25999357
Kilic, Rahmi; Ozdek, Ali; Felek, Sevim; Safak, M Asim; Samim, Erdal
Bilateral simultaneous facial paralysis is an extremely rare clinical entity. Unlike the unilateral form, bilateral facial paralysis seldom falls into Bell's category. It is most often a special finding in a symptom complex of a systemic disease; many of them are potentially life-threatening, and therefore the condition warrants urgent medical intervention. Lyme disease, Guillian-Barre syndrome, Bell's palsy, leukemia, sarcoidosis, bacterial meningitis, syphilis, leprosy, Moebius syndrome, infectious mononucleosis, and skull fracture are the most common cause of bilateral facial paralysis. Here we present a 16-year-old patient with bilateral simultaneous Bell's palsy.
Kim, Dong-Wook; Hah, J. Hun; An, Soo-Youn; Chang, Hak
Mandibular metastasis of thyroid carcinoma is extremely rare. We present the case of a 46-year-old woman who had bilateral huge cheek masses that had grown rapidly over several years. Intra-oral mucosal tissue biopsy and imaging work-up including computed tomography scan and magnetic resonance imaging were performed and the initial diagnosis was presumed to be central giant cell granuloma. Incidentally detected thyroid lesions were studied with ultra-sonography guided fine needle aspiration and diagnosed as simple benign nodules. Due to continuous oral bleeding and the locally destructive feature of the lesions, we decided to excise the mass surgically. To avoid functional deficit, a stepwise approach was performed: Firstly, the larger left mass was excised and the mandible was reconstructed with a fibular free flap. The final pathologic diagnosis was follicular thyroid cancer. Postoperative I-131 thyroid scan and whole body positron-emissions-tomography were performed. Right side mass was revealed as a thyroid malignancy. Multiple bony metastases were detected. Since further radioactive iodine therapy was required, additional total thyroidectomy and right side mandibulectomy with fibular free flap reconstruction was performed. The patient also underwent high dose radioactive iodine therapy and palliative extra-beam radiotherapy for the metastatic lumbar lesion. Follicular thyroid carcinoma should be considered as a differential diagnosis for mandibular mass lesions. PMID:23526730
Kim, Dong-Wook; Hah, J Hun; An, Soo-Youn; Chang, Hak; Kim, Kwang Hyun
Mandibular metastasis of thyroid carcinoma is extremely rare. We present the case of a 46-year-old woman who had bilateral huge cheek masses that had grown rapidly over several years. Intra-oral mucosal tissue biopsy and imaging work-up including computed tomography scan and magnetic resonance imaging were performed and the initial diagnosis was presumed to be central giant cell granuloma. Incidentally detected thyroid lesions were studied with ultra-sonography guided fine needle aspiration and diagnosed as simple benign nodules. Due to continuous oral bleeding and the locally destructive feature of the lesions, we decided to excise the mass surgically. To avoid functional deficit, a stepwise approach was performed: Firstly, the larger left mass was excised and the mandible was reconstructed with a fibular free flap. The final pathologic diagnosis was follicular thyroid cancer. Postoperative I-131 thyroid scan and whole body positron-emissions-tomography were performed. Right side mass was revealed as a thyroid malignancy. Multiple bony metastases were detected. Since further radioactive iodine therapy was required, additional total thyroidectomy and right side mandibulectomy with fibular free flap reconstruction was performed. The patient also underwent high dose radioactive iodine therapy and palliative extra-beam radiotherapy for the metastatic lumbar lesion. Follicular thyroid carcinoma should be considered as a differential diagnosis for mandibular mass lesions.
Sanges, Sébastien; Yelnik, Cécile M.; Sitbon, Olivier; Benveniste, Olivier; Mariampillai, Kuberaka; Phillips-Houlbracq, Mathilde; Pison, Christophe; Deligny, Christophe; Inamo, Jocelyn; Cottin, Vincent; Mouthon, Luc; Launay, David; Lambert, Marc; Hatron, Pierre-Yves; Rottat, Laurence; Humbert, Marc; Hachulla, Eric
Abstract Occurrence of pulmonary arterial hypertension (PAH) in idiopathic inflammatory myopathies (IIMs) without extensive interstitial lung disease (ILD) has rarely been described in the medical literature. This study aimed to report all cases with association of PAH and IIM in the French Pulmonary Hypertension (PH) Registry, to identify IIM features associated with the presence of PAH, and to describe treatment modalities of these patients. All cases of IIM-PAH were retrieved from the French PH Registry, which gathers PH patients prospectively enrolled by 27 referral hospital centers across France. Patients were excluded if they had an extensive ILD or overlap syndrome. Characteristics of IIM-PAH patients were compared with a control group of IIM patients without PH. Among the 5223 PH patients in the Registry, 34 had a diagnosis of IIM. Among them, 3 IIM-PAH patients (2 females and 1 male) had no evidence of extensive ILD or overlap syndrome, and were included in this study. In these 3 patients, dermatomyositis (DM) was the only identified IIM. One patient had autoantibodies classically associated with IIM (anti-Ku). PAH had always developed after IIM onset, was severe in all cases, and led to a marked functional impairment. By pooling our cases with 6 patients previously reported in the literature, and comparing them with a control cohort of 35 IIM patients without PH, we identify several IIM characteristics possibly associated with PAH occurrence, including DM subtype (78% vs 46%; P = 0.02), skin involvement (P = 0.04), anti-SSA antibodies (P = 0.05), and peripheral microangiopathy (P = 0.06). Overall, IIM-PAH patients were managed by corticosteroids and/or immunosuppressants, either alone or combined with PAH therapy. Patients did not seem to respond to IIM treatment alone. Our study reports for the first time the rare but possible association of PAH and IIM in a large prospective PH Registry. In that setting, PAH seems associated with DM, skin involvement
Zhao, Yunfei; Qi, Lin; Yang, Jun; Zhu, Xiaodong; Yang, Changwei; Li, Ming
Abstract Pelvic rotation (PR) is commonly seen in patients with idiopathic scoliosis (IS), but factors contributing to this phenomenon and its relationship with the surgical outcome are not well established. This retrospective study included 85 IS patients in 2 groups: thoracic curve dominance group (group A) and lumbar curve dominance group (group B). Pre- and postoperative PR was measured on standing posteroanterior radiographs by the left/right ratio (L/R ratio) of horizontal distance between the anterior superior iliac spine (ASIS) and the inferior ilium (SI) at the sacroiliac joint on the same side in both groups. Other radiographic data, age, sex, and Risser sign of each patient were recorded to analyze their correlations with PR before and after operation. The patients ranged in age from 10 to 35 years with a mean of 17.0 ± 5.2 years. The mean L/R ratio of PR before operation was 0.99 (0.73–1.40) versus 0.98 (0.87–1.26) after operation. The L/R ration was beyond the range of 1 ± 0.1 (indicating the presence of PR) in 17 (20%) patients before operation and in 14 (16.5%) patients after operation. There was no significant difference in PR between the 2 groups of patients either before (P = 0.468) or after (P = 0.944) surgery. The preoperative PR showed a very low correlation with Risser sign (r = 0.220, P = 0.043), apex vertebral rotation (AVR) in the proximal thoracic curve (r = 0.242, P = 0.026), and AVR in the lumbar curve (r = 0.213, P = 0.049), while the postoperative PR showed a very low correlation with Risser sign (r = −0.341, P = 0.001) and postoperative trunk shift (TS) (r = −0.282, P = 0.009). Multiple stepwise regression analysis showed that preoperative PR was affected by proximal thoracic curve AVR and lumbar curve AVR. There was no significant difference between PR before operation and 2 years after operation. Preoperative PR was mainly correlated with Risser sign and the rotation
Nuzzo, Alexandre; Zappa, Magaly; Cazals-Hatem, Dominique; Bouhnik, Yoram
Abstract Background: Mesenteric panniculitis (MP) is mostly an associated sign of an intra-abdominal or systemic inflammatory primary disease. Nevertheless, etiological and differential diagnosis of idiopathic MP can be challenging when an associate primary cause is not in the foreground. Methods: We report here the case of an isolated small bowel Crohn's disease, long time considered as idiopathic MP. Results: This patient presented to our department with a 10-year history of acute abdominal symptoms evolving with flare-up and remission. A diagnosis of idiopathic MP was made based on compatible CT-scan features along with normal laboratory tests and upper and lower bowel endoscopies. As symptoms recurred, a steroid course was proposed which dramatically improved his condition for years. Finally, an explorative laparoscopy was performed because of concern of malignancy when he returned to our unit with a steroid refractory flare-up and weight loss, along with MP nodes growing up to 10 mm. Crohn's disease was eventually diagnosed, based on histopathological middle-gut bowel resection and numerous granulomas in mesenteric nodes without necrosis. Conclusion: This case emphasizes the importance of excluding inflammatory intestinal lesions before making the diagnosis of idiopathic MP (fecal calprotectin, magnetic resonance enterography, wireless capsule endoscopy). PMID:27684882
de Carvalho, Adriana Castro; Hovnanian, André Luiz; Fernandes, Caio Julio César dos Santos; Lapa, Mônica; Jardim, Carlos; Souza, Rogério
Phosphodiesterase inhibitors like sildenafil have already been shown to improve functional capacity and hemodynamics in the treatment of pulmonary arterial hypertension. Few studies address the effects of new phosphodiesterase inhibitors as tadalafil. We report a case of a patient with idiopathic pulmonary arterial hypertension in functional class IV (New York Heart Association) with significant response to treatment with tadalafil.
Ciarelli, A; Ciarelli, F; Mascia, D; Tentarelli, M
The authors report their findings in the study of idiopathic rectal incontinence using a defecatory balloon proctogram. The study provides a detailed anatomico-functional analysis of the sphincteric tract and, together with manometric and electromyographic studies, is useful in assessing the need for reconstructive surgery and for monitoring the results of ileoanal and coloanal anastomoses, sphincteric plastic surgery and posterior anal plastic surgery.
Pehlivan, Sultan; Akçan, Ramazan; Heybet, Eyup Ruşen; Cavlak, Mehmet; Pehlivan, Ali
Idiopathic giant cell myocarditis is a rare entity of unknown origin, which causes sudden death in more than half of the affected patients. It is rarely seen in childhood, and might result in death due to heart failure and ventricular arrhythmias. Idiopathic giant cell myocarditis is mostly diagnosed at autopsy incidentally. Here we present a rare case of childhood idiopathic giant cell myocarditis. A 10-year old boy found dead in his bed in the morning. Interview with family members revealed death the boy was in good health conditions apart from being overweight. At autopsy, external examination was completely normal. Internal examination revealed normal findings; the heart was 297g and macroscopically normal. No traces of any toxic agents detected in complete toxicological analyses. Areas characterized with granulomatous lesions, lymphocytes, histiocytes, and multinucleated giant cells were observed in myocardium at histopathological examination. No necrosis was observed in granulomatous areas. Tuberculosis was negative in the PCR assays. There were no signs indicative of fungal infection, and clinical status of the case was not compatible with the sarcoidosis. In this respect death was attributed to idiopathic giant cell myocarditis.
Brindle, R.S.; Fernandez, P.M.; Sattenberg, R.J.; Flynn, M.B.; Heidenreich, J.O.
Summary We describe a 65-year-old woman with an asymptomatic idiopathic lingual artery aneurysm which is suspected to be congenital. We review the literature on external carotid artery branch aneurysms, diagnostic evaluation and discuss treatment options for the various types and the specific chosen in the case presented. PMID:20377988
Peter, Jayanthi; Andrew, Nicholas H; Smith, Caroline; Figueira, Edwin; Selva, Dinesh
We present a first case of 58-year-old man with vision loss in a biopsy-proven idiopathic inflammatory orbital tendon sparing myositis. Tests for thyroid autoantibodies were negative at the initial presentation and at 10-month follow-up period. The diagnosis was confirmed on histopathological examination and was also supported by avid sarcolemmal staining for MHC-1 and MHC-2.
Wen, Yu-Hsuan; Chen, Peir-Rong; Wu, Hung-Pin
Profound idiopathic sudden sensorineural hearing loss is thought to have a poor prognosis, but few studies have focused on this condition. We aimed to assess the impact of patient factors, audiologic parameters, and salvage intratympanic steroid injection therapy on the prognosis of profound idiopathic sudden sensorineural hearing loss. The demographic, clinical, and audiologic data, degree of hearing recovery, and efficacy of intratympanic steroid injection therapy in 576 patients with profound idiopathic sudden sensorineural hearing loss (mean age 56.2 ± 14.9 years) who had been admitted at four tertiary referral centers between 2000 and 2011 were retrospectively reviewed. The mean hearing level at the initial presentation was 108.1 ± 9.5 dB. Many patients experienced vertigo (52.1%) and tinnitus (77.4%). At the 2-month follow-up, 172 (29.8%) patients showed some degree of hearing recovery, but only 21 (3.6%) patients recovered normal hearing. Further, the 116 patients who had received salvage intratympanic steroid injections showed a better audiologic outcome (improvement, 26.1 ± 24.3 vs. 15.7 ± 22.1 dB; P = 0.000) than those who had not (n = 429). In conclusion, a higher degree of hearing loss at the initial presentation indicates a poorer prognosis. Salvage intratympanic steroid injection therapy may improve the hearing of patients with profound idiopathic sudden sensorineural hearing loss after the failure of systemic steroid therapy.
Shuyuan, Liu; Changjun, Zhang; Haiying, Peng; Xiaoqin, Huang; Hao, Sun; Keqin, Lin; Kai, Huang; Jiayou, Chu; Zhaoqing, Yang
Telomeres are evolutionary conserved, multifunctional DNA-protein complexes located at the ends of eukaryotic chromosomes. Telomeres maintain chromosome stability and genome integrity and also play an important role in meiosis which aid in synapsis, homologous recombination, and segregation. Sperm telomere has been reported to play an important role in fertilization and embryo development. Nowadays, the association between telomere and reproduction is one of the major areas of interest, however whether sperm telomere associated with male infertility is not clear. In this study, in order to find out the association between Chinese idiopathic infertility and sperm telomere length, we analyzed the difference of sperm telomere length between idiopathic infertile men and normal fertile men, as well as the correlations between sperm telomere length and human semen characteristics. We analyzed 126 Chinese idiopathic infertile men and 138 normal fertile men for sperm telomere length by using quantitative PCR. We found that the relative sperm mean telomere length of infertile men was significantly shorter than that of fertile men (2.894 ± 0.115 vs. 4.016 ± 0.603, P=5.097 x 10⁻⁵). Both sperm count and semen progressive motility are related with telomere length. Our results suggest that sperm telomere length is associated with idiopathic male infertility of China and we proposed the possibility that shorter telomeres in sperm chromosome will reduce spermatogenesis and sperm functions, which finally affected the fertility of male.
Grover, Sanjiv; Basu, Atoshi
Idiopathic eruptive macular pigmentation (IEMP) is a rather under-reported condition of unknown etiology. Clinically consisting of benign hyperpigmented macules, the condition is characterized histopathologically by dermal melanization. It must be differentiated from lichen planus pigmentosus, erythema dyschromicum perstans, fixed drug eruption and mastocytosis.
Grover, Sanjiv; Basu, Atoshi
Idiopathic eruptive macular pigmentation (IEMP) is a rather under-reported condition of unknown etiology. Clinically consisting of benign hyperpigmented macules, the condition is characterized histopathologically by dermal melanization. It must be differentiated from lichen planus pigmentosus, erythema dyschromicum perstans, fixed drug eruption and mastocytosis. PMID:21063525