First Symptom In Patients Research Articles

The Additional Disease Burden Accompanying a Delayed Diagnosis of Multiple Myeloma: Utilising a Large Real-World Evidence Base

Background Multiple myeloma (MM) is one of the most difficult cancers to diagnose, leading to one of the longest delays in diagnosis of all cancers. Delays in diagnosis of MM can reduce survival and lead to complications; however, the extent of disease burden patients experience has not been fully explored from the patient perspective. Our large-scale real-world surveys explore the symptom profiles, disease burden, and the downstream impact on the quality of life (QoL) of MM patients with a delayed versus timely diagnosis. Methods MM patients participated in two national surveys in the UK. Patients were asked to provide demographics, information on their symptoms at diagnosis, and whether they received a timely (<3 months from initial symptoms) or delayed diagnosis (3 months or more from their initial symptoms). Patients subsequently completed the PROMIS-29 v2.0 Health-Related QoL Questionnaire where they were asked to reflect on their current experiences; having completed at least 1 line of treatment. The questionnaire assessed seven health domains (i.e. physical function, fatigue, pain interference, depression, anxiety, social activity participation, and sleep disturbance) alongside a 10-item pain intensity scale. Results 586 MM patients completed Survey 1 and 617 patients completed Survey 2. Overall, patients reported a wide range of symptoms at diagnosis with ‘pain’ reported most frequently overall (74% of cases). Patients with a delayed diagnosis had significantly more symptoms ( M = 5, SD = 4), compared to those with a timely diagnosis ( M = 3, SD = 3), p < .001. MM patients with a delayed diagnosis also reported significantly more symptoms in 9 of the categories of symptoms (see Figure 1) compared to patients with a timely diagnosis, p < .001. Moreover, patients with a delayed diagnosis perceived more of their symptoms as having a ‘high impact’ on their daily lives at the time of diagnosis compared to those with a timely diagnosis, p < .001. The next analysis assessed the likely order that symptoms manifest at diagnosis depending on whether patients had a delayed or timely diagnosis. The most frequently experienced first symptom in patients with a timely or delayed diagnosis was ‘pain’. Decision tree analysis showed that patients with a timely diagnosis who experienced ‘pain’ as their first symptom, were 57% more likely to experience spinal fractures as their next symptom. Conversely, those with a delayed diagnosis were 83% more likely to experience mobility issues as their second symptom. The downstream impact of a delayed diagnosis revealed MM patients with a delayed diagnosis had significantly higher pain interference ( M = 57 vs 53), pain intensity ( M = 4 vs 3), sleep disturbance ( M = 53 vs 51), anxiety ( M = 56 vs 53), depression ( M = 53 vs 50) and fatigue ( M = 57 vs 55), compared to MM patients with a timely diagnosis. MM patients with a delayed diagnosis reported significantly lower physical functioning scores ( M = 43 vs 45) than patients with a timely diagnosis. Scores measuring the ability of patients to carry out social roles and activities were also significantly lower in patients with a timely than delayed diagnosis ( M = 42 vs 44). Conclusions Patients in these large-scale and real-world surveys experienced considerable disease burden at diagnosis when they had a delayed versus timely diagnosis, both in terms of frequency and range of symptoms. The results show the considerable impact of individual symptoms on the daily lives of patients at diagnosis, as well as the downstream impact delayed diagnosis has on the QoL of MM patients. The results offer a potential model for disease progression based on the staging of symptoms and a method for identifying MM patients in clinical practice.

Hoarseness as the first symptom in a patient with acute suppurative thyroiditis secondary to a pyriform sinus fistula: a case report

BackgroundPyriform sinus fistulas (PSFs) are rare congenital anomalies of the third or fourth brachial pouch. Dyspnea is reportedly secondary to compression by a neck mass. However, hoarseness, as the first symptom of PSF, has not yet been reported.Case presentationThis report describes an 11-year-old girl presenting with hoarseness as the first symptom of PSF. Hoarseness occurred 2 days prior to admission. On admission, she had fever, hoarseness, and an elastic soft mass on her left anterior neck. Contrast-enhanced computed tomography of the cervical region demonstrated an abscess partially infiltrating the thyroid gland and an air pocket near the pyriform sinus. Pharyngoscopy revealed swelling of the left arytenoid region, with purulent retention. The left vocal cord was swollen but not paralyzed. Additionally, the laboratory data indicated thyrotoxicosis. Suspecting a PSF infection, parenteral treatment with cefotaxime and dexamethasone was initiated. On the following day, the hoarseness disappeared, and the fever resolved. Four weeks after onset, the thyroid hormone levels returned to the normal range, and a barium esophagogram revealed residual contrast in the left pyriform sinus, leading to a diagnosis of PSF.ConclusionPSF presenting with hoarseness as the first symptom in patients should be considered.

Myotonia and myotonic discharges of dystrophic myotonia type 1 at the first decade onset: a literature review and data of the case series

Background. Dystrophic myotonia type 1 (DM1) is the most common muscular dystrophy in patients of any age. Myotonia “delayed relaxation of muscle” is the leading symptom in DM1 and can occur at any time after onset disease. Myotonia symptoms and electrical myotonia registration are delayed after onset in patients with congenital and infantile forms of DM1. This makes it difficult to diagnose and prevent fatal complications in these patients in a timely manner. Objective: presentation of the clinical data and results of needle electromyography in patients with DM1 onset in the first decade of the life; determination of the first symptoms of the disease, to estimate the age of myotonia and electrical myotonia manifestation for the optimization of the timely diagnostics of the disease.Materials and methods. 13 patients with DM1 aged from 2 months to 34 years were described. 10 patients underwent needle electromyography with analysis of spontaneous activity and needle EMG pattern. The diagnosis was made on the basis of clinical and paraclinical manifestations of the disease and identification of an increase in CTG repeats (>50) in the DMPK gene.Results. The onset with extramuscular signs of respiratory and/or feeding disturbances, dysarthria, school learning disorders, autism spectrum disturbance and “floppy infant syndrome” was noted as the first symptoms of the disease. Clinical myotonia symptoms and electrical manifestations of myotonia were absent in all patients for a long time after the disease onset. DM1 was confirmed in all mothers, however in 5 cases the onset of the disease was later than the first symptoms in patients with congenital and childhood onset forms of DM1.Conclusion. The first symptoms of the congenital and infantile forms of DM1 are not specific and occur in a wide range of diseases. Such discriminating signs of DM1 as clinical myotonia, distal muscle atrophy and electrical myotonia appear much later than the onset disease. In the group of patients before and after the formation of phrasal speech, the presented combinations of symptoms allow diagnostics of the congenital and infantile forms of DM1 at the onset of the disease. In its turn, it allows genetic counseling in burdened families and timely prevention of fatal complications.

Long-Term Subjective and Objective Assessment of Smell and Taste in COVID-19.

Among the first clinical symptoms of the SARS-CoV-2 infection is olfactory–gustatory deficit; this continues for weeks and, in some cases, can be persistent. We prospectively evaluated 162 patients affected by COVID-19 using a visual analogue scale (VAS) for nasal and olfactory–gustatory symptoms. Patients were checked after 7, 14, 21, 28, 90, and 180 days. A total of 118 patients (72.8%) reported an olfactory VAS < 7 at baseline (group B), and 44 (27.2%) reported anosmia (VAS ≥ 7) (group A) and underwent the Brief Smell Identification Test (B-SIT) and Burghart Taste Strips (BTS) to quantify the deficit objectively and repeated the tests to confirm the sense recovery. Group A patients showed B-SIT anosmia and hyposmia in 44.2% and 55.8% of cases, respectively. A total of 88.6% of group A patients reported ageusia with VAS ≥ 7, and BTS confirmed 81.8% of ageusia and 18.2% of hypogeusia. VAS smell recovery was recorded starting from 14 days, with normalization at 28 days. The 28-day B-SIT score showed normosmia in 90.6% of group A patients. The mean time for full recovery (VAS = 0) was shorter in group B (22.9 days) than in group A (31.9 days). Chemosensory deficit is frequently the first symptom in patients with COVID-19, and, in most cases, recovery occurs after four weeks.

Surgical Management of abnormal uterine bleeding with endometrial sampling during COVID-19

To investigate the delay in surgical management of abnormal uterine bleeding with endometrial sampling during the peak of the COVID-19 pandemic at a single, urban tertiary medical center. We conducted a retrospective chart review of 868 patients, 466 who received an endometrial biopsy in 2019 and 402 between January 1–March 23, 2020 (during the first peak of COVID-19) at a tertiary academic medical center in Philadelphia. We collected baseline patient characteristics including: age, self-identified race or ethnicity, and BMI. We assessed the time from an abnormal endometrial biopsy to surgical management, use of at least one telemedicine appointment between biopsy and surgical management, and resulting pathology results. Chi-squared test was used to compare proportions of populations and two-tailed student's T-test was used to compare days between biopsy and surgical management. P-value was set at 0.05. 466 and 402 patients underwent an endometrial biopsy in 2019 and between January 1 – March 23, 2020, respectively. In 2019, 4.94% were diagnosed with an endometrial malignancy and 95.1% had resulting benign pathology; while in 2020, 5.22% had a diagnosed endometrial malignancy and 94.8% had benign pathology (p-value = 0.84). Median age was 51.0 years (range, 19.0–89.0) in 2019 and 51.0 years (range 24–89) in 2020. Median BMI was 31.4 (range, 17.6–66.9) in 2019 and 31.1 (range, 16.3–74.2) in 2020. Median time between endometrial biopsy and surgical management was 66.5 days (range, 0–453 days) in 2019 and 94.0 days (range 13.0–335) in 2020 (p-value = 0.57). Median time from biopsy to surgery for patients with a resulting pathology of endometrial malignancy was 53 days (range, 0–441) in 2019 and 87.5 days (range 13.0–323) in 2020 (p-value = 0.50). Median time for patients with resulting benign pathology was 69.0 days (range, 9.00–453) in 2019 and 112 days (range, 33.0–335) in 2020 (p-value = 0.48). 57.4% of patients in 2020 had at least one telemedicine appointment with their physician between the initial encounter for abnormal uterine bleeding and surgical treatment, while no patients had a telemedicine appointment in 2019. During the COVID-19 pandemic, individual patients with abnormal uterine bleeding may have experienced delays between initial abnormal endometrial biopsy and surgical management. However, comparing the populations as a whole, there was not a statistical difference in time between biopsy and surgical management for abnormal uterine bleeding, reinforcing the quality of care given to our patients. However, further studies are needed to examine the effects of COVID-19 on possible delay in surgical treatment from first symptoms in patients with abnormal uterine bleeding to biopsy and to surgical management.

Wilson Hastalarının Nörolojik Bulguları

Objective: Wilson’s disease is an autosomal recessive disorder characterized by copper metabolism disorder. Prognosis depends on the severity of liver involvement and neurological findings. Neurological involvement can sometimes be the first symptom in patients, early diagnosis and treatment can control brain damage and symptoms. In this study, we wanted to emphasize the importance of neurological involvement of the disease. Material and Methods: This study included Wilson patients with neurological findings who applied to Pediatric Neurology Department between April 2008 and May 2018. The age, gender distribution, complaints of the patients, neurological symptoms, hepatological findings, treatments and treatment responses were analyzed retrospectively. A detailed neurological examination was performed on all 42 Wilson patients who were eligible. Fifteen patients with neurological findings were evaluated. Results: The mean age of the patients was 12.5±3.6 years (8-20 years), 53.3% male, 46.7% female. Hepatologic symptoms were present in 66.7% of the patients, neurological symptoms in 35.7% and Kayser-Fleischer ring in 26.7% of the patients. The most common symptoms in patients with neurological findings were ataxia and tremor (40%). Other symptoms were dystonia (13.4%), korea (6.6%), seizures (13.4%), behavioral disorders (13.4%), dysarthria and sialorrhea (13.4%). Radiologic findings were present in 10 (66.7%) of the patients with neurological findings. Ten patients received penicilamine treatment and five patients received trientine treatment. At the end of the first year of treatment, neurological symptoms improved in 80% of those receiving trientine treatment and in 50% of those receiving penicillamine treatment. Conclusion: Neurological involvement is common in Wilson’s disease. Since the findings may be overlooked at the beginning of the disease, neurological examination should be performed in all patients receiving the diagnosis. Wilson’s disease should also be kept in mind in etiology in patients with movement disorder.

UNUSUAL PARADOXICAL ARTERIAL EMBOLISM INVOLVING RIGHT UPPER EXTREMITY IN A PATIENT WITH PULMONARY EMBOLISM AND PATENT FORAMEN OVALE

Paradoxical arterial embolism (PDE) may cause the first symptoms in patients with a coexisting hypercoagulable state and PFO. Most pts with PFO are asymptomatic. However, in the younger population it could be the underlying etiology of recurrent arterial embolus events, such as cryptogenic stroke or

Central and peripheral vestibular signs simultaneously present in a patient with instability: A case report

Introduction. Cerebellar ataxia, neuropathy, vestibular areflexia syndrome is a rare neurodegenerative disease with instability as the main presenting symptom. Patients with this syndrome often present with central and peripheral vestibular signs. This slowly progressive disease usually starts after 60 years of age and it takes 11 years to diagnose it. Case Report. Here I present a 62-year-old woman with instability lasting for 7 years, but deteriorating in the last two years with two episodes of falls, diplopia when looking to the right, paresthesia in the extremities and clumsiness with hands. Clinical examination revealed dysarthria, positive Romberg test, left hand dysmetria, gaze evoked and downbeat nystagmus, positive head impulse test, absent vestibulo-ocular reflex at video head impulse test, no response to caloric stimulation, no smooth pursuit and dysmetric and prolonged saccades at videonystagmography, positive visually enhanced vestibulo ocular reflex test, normal head magnetic resonance imaging, subclinical signs of polyneuropathy at electroneurography and negative autoimmune and paraneoplastic cerebellar antibodies. Conclusion. Instability is the first symptom in patients with cerebellar ataxia, neuropathy, vestibular areflexia syndrome. Easy to perform, positive visually enhanced vestibulo-ocular reflex test points to a concomitant central and peripheral vestibular disorder. Negative autoimmune and paraneoplastic antibodies rule out other cerebellar diseases. However, normal head magnetic resonance imaging findings without expressed signs of peripheral sensory neuropathy are in concordance with a slowly progressive form of this syndrome.

First neuropsychiatric symptoms and neurocognitive correlates of behavioral variant frontotemporal dementia

Previous works highlight the neurocognitive differences between apathetic and disinhibited clinical presentations of the behavioral variant frontotemporal dementia (bvFTD). However, little is known regarding how the early presentation (i.e., first symptom) is associated to the neurocognitive correlates of the disease's clinical presentation at future stages of disease. We analyzed the neurocognitive correlates of patients with bvFTD who debuted with apathy or disinhibition as first symptom of disease. We evaluated the neuropsychological, clinical and neuroanatomical (3 T structural images) correlates in a group of healthy controls (n = 30) and two groups of bvFTD patients (presented with apathy [AbvFTD, n = 18] or disinhibition [DbvFTD, n = 16]). To differentiate groups according to first symptoms, we used multivariate analyses. The first symptom in patients described the evolution of the disease. AbvFTD and DbvFTD patients showed increased brain atrophy and increased levels of disinhibition and apathy, respectively. Whole brain analyzes in AbvFTD revealed atrophy in the frontal, insular and temporal areas. DbvFTD, in turn, presented atrophy in the prefrontal regions, temporoparietal junction, insula and temporoparietal region. Increased atrophy in DbvFTD patients (compared to AbvFTD) was observed in frontotemporal regions. Multivariate analyses confirmed that a set of brain areas including right orbitofrontal, right dorsolateral prefrontal and left caudate were enough to distinguish the patients’ subgroups. First symptom in bvFTD patients described the neurocognitive impairments after around three years of disease, playing an important role in the early detection, disease tracking, and neuroanatomical specification of bvFTD, as well as in future research on potential disease-modifying treatments.Disclosure of interestThe authors have not supplied their declaration of competing interest.

First Symptoms and Neurocognitive Correlates of Behavioral Variant Frontotemporal Dementia.

Previous works highlight the neurocognitive differences between apathetic and disinhibited clinical presentations of the behavioral variant frontotemporal dementia (bvFTD). However, little is known regarding how the early presentation (i.e., first symptom) is associated to the neurocognitive correlates of the disease's clinical presentation at future stages of disease. We analyzed the neurocognitive correlates of patients with bvFTD who debuted with apathy or disinhibition as first symptom of disease. We evaluated the neuropsychological, clinical, and neuroanatomical (3T structural images) correlates in a group of healthy controls (n = 30) and two groups of bvFTD patients (presented with apathy [AbvFTD, n = 18] or disinhibition [DbvFTD, n = 16]). To differentiate groups according to first symptoms, we used multivariate analyses. The first symptom in patients described the evolution of the disease. AbvFTD and DbvFTD patients showed increased brain atrophy and increased levels of disinhibition and apathy, respectively. Whole brain analyzes in AbvFTD revealed atrophy in the frontal, insular, and temporal areas. DbvFTD, in turn, presented atrophy in the prefrontal regions, temporoparietal junction, insula, and temporoparietal region. Increased atrophy in DbvFTD patients (compared to AbvFTD) was observed in frontotemporal regions. Multivariate analyses confirmed that a set of brain areas including right orbitofrontal, right dorsolateral prefrontal, and left caudate were enough to distinguish the patients' subgroups.∥Conclusion: First symptom in bvFTD patients described the neurocognitive impairments after around three years of disease, playing an important role in the early detection, disease tracking, and neuroanatomical specification of bvFTD, as well as in future research on potential disease-modifying treatments.

Alzheimer’s Disease and Memory Loss - A Review

Memory loss is one of the first symptoms in patients suffering from Alzheimer’s disease (AD). During the course of the disease patients experience problems with their language, mood swings, and behavioural issues. Due to these conditions patients often withdraw from family or society with the fear and embarrassment and gradually, bodily functions are lost leading to death. The pattern of memory loss correlates with structural and functional integrity of brain. In patients with AD the formation of memory will be affected at molecular level disturbing the framework of neural networks. Recently, many studies have focused on how the AD impacts episodic memory and the underlying neural processes. In this review the findings of these studies are discussed and the effects of current and promising treatments for AD on memory loss are summarized. The main aim of this review is creating awareness in understanding the intact and dysfunctional memory. Moreover the knowledge of a specific process of a particular memory loss due to AD helps the basic scientists in developing novel therapies and drugs.

Surgical management of intradural spinal cord tumors in children and young adults: A single-center experience with 50 patients.

Background:Intradural spinal cord tumors (IDSCTs) in children and young adults are rare diseases. This present study is aimed to demonstrate our experience with a large series of children and young adults with IDSCT.Methods:A total of 50 patients aged <20 years with IDSCT treated in our department between 1990 and 2010 were included in the study. Clinical, histological, and radiological findings, treatment strategies, and clinical outcome were retrospectively assessed. Depending on the relation to the spinal cord, IDSCT were dichotomized into intramedullary SCT (IMSCT) and extramedullary SCT (EMSCT). The functional outcome was evaluated with the Frankel score assessing the longest available follow-up period.Results:Mean age was 10.3 years (range 6 months–19 years). IDSCT surgery was performed in 44 patients (88%). A common first symptom in patients with EMSCT was neck and back pain (41%), whereas monoparesis of arms (43%) were often seen in patients with IMSCT. The main duration of the symptoms was longer in patients with IMSCT. The postoperative functional outcome was generally comparable to the preoperative functional condition, while better for EMSCT (P < 0.01). The functional outcome at last follow-up correlated significantly with the preoperative Frankel score (P < 0.002).Conclusion:Due to the mostly mild impact of the surgery on the functional outcome, the surgical treatment of IDSCT in children and young patients can be uniquely advocated.

First symptoms in patients with thrombotic thrombocytopenic purpura: what are they and when do they occur?

Idiopathic thrombotic thrombocytopenic purpura (TTP) is a rare cause of thrombocytopenia and hemolysis characterized by life-threatening end-organ ischemia due to platelet microthrombi. TTP arises from severe deficiency of ADAMTS13 (< 10% activity). Although neurologic symptoms are considered classical of TTP, they are not present in all patients. Previous studies have described other manifestations in a significant percentage of the patients, related or not to hemolysis and anemia.1 Therefore, physicians must have a high level of suspicion in order to diagnose TTP. Delay to refer a patient for therapeutic plasma exchange (TPE) is potentially fatal.2 Following Institutional Review Board’s approval, we reviewed the records of all adults diagnosed with a first episode of TTP at the University of Alabama at Birmingham (UAB) and the Oklahoma TTP-HUS Registry from January 1, 2007 to June 30, 2010 to answer a specific question: What are the first symptoms of TTP and when do they occur? TTP was defined as thrombocytopenia, microangiopathic hemolytic anemia, and ADAMTS13 activity of < 10% . Patients with underlying conditions such as: pregnancy/postpartum, stem cell transplantation, sepsis, systemic malignancy, autoimmune disease, or a bloody diarrhea prodrome, were excluded, even if their ADAMTS13 activity was <10%. Thirty-four patients met eligibility criteria (23 from UAB and 11 from the OK Registry). Their mean age was 46 years, and 76% were female. Their laboratory data are shown in Table 1. Table 2 displays the initial symptoms and the number of days until the first TPE. While neurologic complaints were the most common (44%), they varied from mild, such as headache or lightheadedness, to serious, such as seizures and focal deficits. The single most common symptom was abdominal pain (23.5%), with or without nausea/vomiting. Less than 10% of patients reported bleeding secondary to thrombocytopenia. The mean number of days prior to TPE was 12, the median was 5.5, and 62% were treated within 7 days. Table 1 Laboratory characteristics of patients with TTP: Table 2 Initial symptoms and time to first therapeutic plasma exchange (TPE): Our data confirm that patients with TTP present with a wide range of symptoms, not immediately suspicious for TTP, such as abdominal pain, nausea and vomiting. We postulate that some of these symptoms reflect an underlying disease that may have triggered TTP. Influenza, for example, has been shown to induce antibodies to ADAMTS13 and clinical TTP.3 Moreover, we suggest that abdominal pain, not considered classic of TTP, represents intra-abdominal ischemia, as it has been rarely described.4-6 In the past year, we have seen two patients with ADAMTS13 deficiency that died with massive gastrointestinal ischemia and hemorrhage, confirmed by autopsy. Unfortunately, the nonspecific nature of signs and symptoms of TTP are likely to hamper a physician’s ability to suspect it on clinical grounds alone. Unless a complete blood cell count is ordered and the combination of severe thrombocytopenia and microangiopathic hemolytic anemia is noted, it is concerning that some patients may experience a delay in diagnosis. We did not collect any information on how many clinic or emergency room visits our patients had prior to being diagnosed with TTP. Anecdotally, we know that many patients are seen by several physicians until the constellation of history, laboratory values and consultation with colleagues in hematology or apheresis medicine eventually leads to the correct diagnosis of TTP. Indeed, patients included in this study had one or more of the following diagnoses prior to being referred for TPE: meningitis, arthritis, gastroenteritis, systemic lupus erythematosus, immune thrombocytopenia, stroke, urosepsis, diabetic non-ketotic hyperosmolar acidosis, pernicious anemia, pancreatitis, and antiphospholipid antibody syndrome, confirming our concerns.

Factores predictores de esofagitis eosinofílica en impactación esofágica por bolo alimenticio

Food bolus esophageal impaction is often the first symptom in patients diagnosed with eosinophilic esophagitis, representing a change in the epidemiology and management of this urgency. To detect eosinophilic esophagitis predictive factors in patients with esophageal impaction due to food bolus. Patients seen for foreign body impaction were retrospectively analyzed. Epidemiologic characteristics, endoscopic findings, and impaction history were studied. The statistical analysis was carried out using the Student's t test and the chi square test and a logistic regression model. Of the 131 patients, 65% were men and the mean age was 56 years. The endoscopic suspicion of eosinophilic esophagitis was the most frequent finding in patients with food bolus impaction (n=89); those patients that did not have histologic confirmation were excluded (n=7). The remaining patients (n=82) were divided into two groups: confirmed eosinophilic esophagitis (Group A) (n=18) and other endoscopic findings (Group B) (n=64). Group A presented with a lower mean age (36.47 vs. 64.45, P=.001) and a more frequent past history of impaction (38% vs. 6%, OR=15.70, 95% CI (3.60-62.50), P=.001) than Group B. Age and impaction history acted as predictors for eosinophilic esophagitis with 82% sensitivity, 80% specificity, and 84% diagnostic accuracy (P<.001). Age and a history of impaction predict the presence of eosinophilic esophagitis in patients with food bolus impaction.

In vivo study of skin mechanical properties in Raynaud's phenomenon

Raynaud's phenomenon (RP) is usually the first symptom in patients with systemic sclerosis (SS) and may precede skin changes by several months or years. Non-invasive measurements of skin elasticity are very sensitive and appropriate for objective and quantitative evaluation of sclerodermatous skin. The aim of this study was to investigate and compare the mechanical properties of the skin in patients with primary, secondary, and suspected secondary RP. A total of 63 patients were studied. They were classified as having scleroderma-type nailfold capillary abnormalities--17 with indurative phase of scleroderma (group 1), nine with edematous phase of scleroderma (group 2), 18 with suspected secondary RP (group 3) and as having RP-type nailfold capillary abnormalities, 19 with primary RP (group 4). Thirty-nine sex- and age-matched healthy individuals with normal nailfold capillaroscopy pattern were also studied as controls. Mechanical properties of the skin were evaluated using a non-invasive suction device (Cutometer) equipped with a 2 mm probe. Measurements were performed over five anatomic regions: cheeks, volar forearms, wrists, hands, and proximal phalanx of the fingers. The skin mechanical parameters analyzed were distensibility (Uf), elasticity (Ua/Uf) and viscoelasticity (Uv/Ue). Most demonstrative changes were observed over volar forearms. Patients included in groups 1-3 were characterized by significantly lower Uf and higher Uv/Ue compared with patient group 4 and controls. Patient groups 1 and 2 showed significantly lower Ua/Uf, as well. There were no significant differences in skin mechanical parameters between patient group 4 and control group. Mechanical properties of the skin in patients with suspected secondary RP significantly differ from these in patients with primary RP and resemble those in patients with edematous phase of scleroderma. Our findings suggest that the non-invasive measurements of skin elasticity could be helpful in identifying patients with RP at risk of developing SS.

Perirenales Urinom durch Urethralklappen bei einem Säugling mit fluktuierender Flankenschwellung

Flank swelling and pseudotumors of the kidney are unusual manifestations of obstructive uropathies in small children. Our case illustrates typical problems and briefly reviews management options. A 5-week-old boy presented with a large, palpable urinoma due to posterior urethral valves. Sonography and voiding cystourethrogram led to the diagnosis and immediate suprapubic transcutaneous urinary diversion was performed. However, the urinoma did not resolve. Thus, in addition to suprapubic urinary diversion, indirect drainage - instead of percutaneous puncture - was performed by retrograde insertion of a double-J catheter. Urethral valves were resected 4 weeks later and follow-up demonstrated an uneventful further development with normal renal function as assessed by regular ultrasound studies, a repeat cystourethrogram and a renal scan. Perirenal urinomas may be the first symptom in patients with posterior urethral valves. Drainage via double-J stenting offers a promising alternative to percutaneous puncture. A renoprotective "pop-off" mechanism by which intrarenal pressure may be relieved is discussed.

Recurrent Laryngeal Nerve Palsy in Well‐differentiated Thyroid Carcinoma: Clinicopathologic Features and Outcome Study

Involvement of the recurrent laryngeal nerve (RLN) by well-differentiated thyroid carcinoma may not invariably lead to unilateral cord palsy, although the presence of RLN palsy is associated with locally advanced disease. The present study evaluates the clinicopathologic features and outcomes of patients surgically treated for well-differentiated thyroid carcinoma with documented nonfunctioning RLN at presentation. From 1970 to 2002, 20 of 709 patients undergoing surgical treatment for well-differentiated thyroid carcinoma were found to have ipsilateral unilateral cord palsy by routine preoperative laryngoscopy. There were 5 men and 15 women with a median age of 70 years. Nine patients (45%) did not have a clinically palpable thyroid mass, and hoarseness was the primary presenting symptom. All patients had histologically confirmed pT4 papillary thyroid carcinoma with a median size of 4 cm. Cervical nodal and pulmonary metastases were detected in 14 (70%) and 2 (10%) patients, respectively. The ipsilateral recurrent nerve was transected in all patients because of gross tumor involvement, and 19 patients underwent total or completion total thyroidectomy. Resection was incomplete in 15 patients, including 2 who underwent a debulking procedure and required reoperation for local control. Postoperative radioactive iodine ablation and external-beam irradiation were administered to 18 and 13 patients, respectively. Over a median follow-up of 4.5 years, 10 patients survived without evidence of recurrence, 5 died of disease recurrence, and 5 died of unrelated causes. The 5-year and 10-year cause-specific mortality was 17% and 42%, respectively. Patients developing distant metastasis at presentation or during follow-up had a significantly increased cause-specific mortality (p = 0.002). Preoperative RLN palsy can be the first symptom in patients with locally advanced papillary thyroid carcinoma. Despite the adoption of a relatively conservative surgical treatment, long-term survival can be achieved in selected patients.

Chest Pain as an Unusual First Symptom in Patients with Systemic Amyloidosis

Summaries for Patients7 December 1999Chest Pain as an Unusual First Symptom in Patients with Systemic AmyloidosisSearch for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-131-11-199912070-00029 SectionsAboutFull Text ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail What is the problem and what is known about it so far?Systemic amyloidosis is a rare disease caused by the buildup of an abnormal protein substance called amyloid in body tissues. Amyloidosis can affect many organs of the body, but involvement of the heart and blood vessels is the most common cause of illness and death in patients with this disease. In patients whose heart and blood vessels are affected by amyloidosis, shortness of breath, leg swelling, or abnormal heart rhythms are usually the first signs that they have this disease. Angina pectoris (chest pain) has not been thought ... Author, Article, and Disclosure InformationAffiliations: The summary below is from the full report titled “Systemic Amyloidosis Presenting with Angina Pectoris.” It is in the 7 December 1999 issue of Annals of Internal Medicine (volume 131, pages 838-841). The authors are J. Al Suwaidi, J.L. Velianou, M.A. Gertz, R.O. Cannon III, S.T. Higano, D.R. Holmes Jr., and A. Lerman.Summaries for Patients are a service provided by Annals to help patients better understand the complicated and often mystifying language of modern medicine.Summaries for Patients are presented for informational purposes only. These summaries are not a substitute for advice from your own medical provider. If you have questions about this material, or need medical advice about your own health or situation, please contact your physician. The summaries may be reproduced for not-for-profit educational purposes only. Any other uses must be approved by the American College of Physicians-American Society of Internal Medicine. Previousarticle Advertisement FiguresReferencesRelatedDetailsSee AlsoSystemic Amyloidosis Presenting with Angina Pectoris Jassim Al Suwaidi , James L. Velianou , Morie A. Gertz , Richard O. Cannon III , Stuart T. Higano , David R. Holmes Jr. , and Amir Lerman Metrics Cited byHeart involvement in AL-amyloidosis. Current state of the issue 7 December 1999Volume 131, Issue 11Page: 838KeywordsAmyloidosisAnginaBiopsyBlood vesselsCardiovascular therapyCatheterizationHeartHeart diseasesIschemiaMyocardial infarction ePublished: 15 August 2000 Issue Published: 7 December 1999 Copyright & PermissionsCopyright © 1999 by American College of Physicians. All Rights Reserved.Loading ...