Factors other than sheer numbers of blood cells in circulation may be responsible for clinical presentations in chronic myeloid leukaemia.

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e19527-e19527
Author(s):  
Nicolas Anthony Othieno-Abinya ◽  
Andrew Oluoch Odhiambo ◽  
Mohammed Shabbir Ezzi ◽  
Peter Omondi Oyiro
Keyword(s):  
Heart Failure ◽  
Abdominal Pain ◽  
Blood Cells ◽  
Visual Loss ◽  
Clinical Manifestations ◽  
Bleeding Tendency ◽  
Demographic Profiles ◽  
Clinical Presentations ◽  
Patient Assistance ◽  
Biologic Factors

e19527 Background: CML commonly presents with abdominal pain and swelling, and in up to about 50% of cases it is discovered incidentally due to abnormally elevated white blood cell (wbc) counts in circulation. Some present with bleeding tendency, others nusual presentations including hearing and visual loss whose pathogenesis is poorly understood also occur. Priapism presents rarely, attributed to hyperviscosity complicating wbc counts ≥ 250x109/litre. Nonspecific symptoms also present. We wanted to interrogate the link between baseline myeloid elements in circulation, bone marrow blasts, and BCR-ABL values with clinical manifestations. Methods: Patients attending the Glivec International Patient Assistance Clinic at the Nairobi Hospital had records prospectively collected, and analyzed. These included demographic profiles, physical examination, and laboratory values. Absolute, range, median and mean counts for wbcs, absolute neutrophil counts (ANC), platelets, haemoglobin and BCR-ABL values, were tested against each of the clinical presentations. Results: There were 583 patients, males 322 (55.2%) and females 261 (44.8); mean age 39.8 years. Commonest symptom was abdominal swelling in 235 cases (40.3%); 70 (12%) experienced abdominal pain. Priapism occurred in 4 males (1.2%), blindness and deafness in 8 patients (1.4%). Splenomegaly was more common among males than females; [Pearson Chi2 (1) = 5.9545, ( Pr = 0.015)]. Patients aged ≤ 20 years were more likely to be in heart failure, [Pearson Chi2 (3) = 8.8255,( Pr = 0.032)], leg swelling more in those with high platelet counts; [Ranksum test, (p = 0.023)]. Unclear presentation was more common in those with high wbc counts; [Ranksum test, (p = 0.015)]. Total wbc counts and BCR-ABL levels were higher among younger patients; [Kruskal-Wallis test (p = 0.036, and 0.025 respectively)]. Patients with leg swelling had lower BCR-ABL levels; [Ranksum test (p = 0.005)], those in heart failure had significantly higher BCR-ABL levels at baseline compared with the rest; [Ranksum test (p = 0.037)]. Patients with lymphadenopathy were more likely to have lower ANCs; [Ranksum test (p = 0.035)]. Conclusions: Clinical manifestations of CML cannot be explained merely by haematologic values. Complex biologic factors should be interrogated further.

scholarly journals Atypical Clinical Manifestations of Graves' Disease: An Analysis in Depth

2012 ◽  
Vol 2012 ◽  
pp. 1-8 ◽  
Author(s):  
Mohamed Osama Hegazi ◽  
Sherif Ahmed
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Clinical Manifestations ◽  
Right Heart Failure ◽  
Deficiency Anemia ◽  
Graves Disease ◽  
Anemia Of Chronic Disease ◽  
Delay In Diagnosis ◽  
Clinical Presentations ◽  
Atypical Manifestations

Over the past few decades, there has been an increase in the number of reports about newly recognized (atypical or unusual) manifestations of Graves' disease (GD), that are related to various body systems. One of these manifestations is sometimes the main presenting feature of GD. Some of the atypical manifestations are specifically related to GD, while others are also similarly seen in patients with other forms of hyperthyroidism. Lack of knowledge of the association between these findings and GD may lead to delay in diagnosis, misdiagnosis, or unnecessary investigations. The atypical clinical presentations of GD include anemia, vomiting, jaundice, and right heart failure. There is one type of anemia that is not explained by any of the known etiological factors and responds well to hyperthyroidism treatment. This type of anemia resembles anemia of chronic disease and may be termed GD anemia. Other forms of anemia that are associated with GD include pernicious anemia, iron deficiency anemia of celiac disease, and autoimmune hemolytic anemia. Vomiting has been reported as a presenting feature of Graves' disease. Some cases had the typical findings of hyperthyroidism initially masked, and the vomiting did not improve until hyperthyroidism has been detected and treated. Hyperthyroidism may present with jaundice, and on the other hand, deep jaundice may develop with the onset of overt hyperthyroidism in previously compensated chronic liver disease patients. Pulmonary hypertension is reported to be associated with GD and to respond to its treatment. GD-related pulmonary hypertension may be so severe to produce isolated right-sided heart failure that is occasionally found as the presenting manifestation of GD.

Schönlein-Henoch Syndrome: Observations on Some Atypical Clinical Presentations

PEDIATRICS ◽  
1972 ◽  
Vol 49 (4) ◽  
pp. 614-616
Author(s):  
David J. Sahn ◽  
Allen D. Schwartz
Keyword(s):  
Differential Diagnosis ◽  
Abdominal Pain ◽  
Clinical Manifestations ◽  
Joint Involvement ◽  
Long Duration ◽  
Clinical Presentations

Case records of 20 children with Schönlein-Henoch syndrome are reviewed. The clinical manifestations are similar to those described in previous reports except for a 38% incidence of testicular involvement in males. In several cases a long duration of abdominal pain and joint involvement was noted prior to the onset of rash. We believe that this syndrome should be considered in the differential diagnosis of children with abdominal pain, edema, or hematuria prior to the onset of rash. Occasionally the disease may occur sine eruption.

Disseminated intravascular coagulation syndrome

2020 ◽  
pp. 22-40
Author(s):  
A. Kulikov
Keyword(s):  
Clinical Practice ◽  
Disseminated Intravascular Coagulation ◽  
Blood Cells ◽  
Physical State ◽  
Clinical Manifestations ◽  
Vascular Wall ◽  
Stages Of Development ◽  
Intravascular Coagulation ◽  
Hemostatic Disorder

Presented material reveals main links in the pathogenesis of hemostatic disorder. In particular, attention is paid to the role of the lungs, liver and other organs in the development of this process. Role of vascular wall and blood cells in regulation of the physical state of blood is described in detail. The most frequent factors leading to hypercoagulation are indicated. Difference between hypercoagulation and thrombophilia is shown. The latter is found in clinical practice quite often, but at the same time, it is poorly diagnosed. Such a terrible complication of hemostatic disorder as disseminated intravascular coagulation is described. Its classification, stages of development, clinical manifestations are offered to the readers.

scholarly journals Non-Celiac Gluten Sensitivity: A Challenging Diagnosis in Children with Abdominal Pain

2018 ◽  
Vol 73 (Suppl. 4) ◽  
pp. 39-46 ◽  
Author(s):  
Frank M. Ruemmele
Keyword(s):  
Abdominal Pain ◽  
Celiac Disease ◽  
Clinical Symptoms ◽  
Gluten Sensitivity ◽  
Gluten Free ◽  
Clear Cut ◽  
Clinical Presentations ◽  
New Findings ◽  
Three Phase ◽  
Gi Symptoms

Several disorders related to the ingestion of gluten are well recognized despite overlapping clinical presentations: celiac disease, an autoimmune enteropathy triggered by gluten ingestions in susceptible individuals, allergy to wheat, and more recently non-celiac gluten sensitivity (NCGS). While celiac disease and wheat allergy are well-known disorders with a clear-cut diagnosis based on clinical tests and biological parameters, NCGS is a more difficult diagnosis, especially in children with functional gastrointestinal (GI) complaints. NCGS is considered a syndrome of intestinal but also extraintestinal symptoms occurring within hours, but sometimes even after several days of gluten ingestion. In children, the leading symptoms of NCGS are abdominal pain and diarrhea, while extraintestinal symptoms are rare, in contrast to adult patients. No precise diagnostic test nor specific biomarkers exist, except a rather cumbersome three-phase gluten-exposure, gluten-free diet, followed by a blinded placebo-controlled gluten challenge with crossover to provoke symptoms elicited by gluten in a reproducible manner that disappear on gluten-free alimentation. Recent data indicate that the peptide part of wheat proteins is not necessarily the sole trigger of clinical symptoms. Mono- or oligosaccharides, such as fructan and other constituents of wheat, were able to provoke GI symptoms in clinical trials. These new findings indicate that the term gluten sensitivity is probably too restrictive. The incidence of NCGS was reported in the range of 1–10% in the general population and to increase steadily; however, most data are based on patients’ self-reported gluten intolerance or avoidance without a medically confirmed diagnosis. Treatment consists of gluten avoidance for at least several weeks or months. Patients with NCGS require regular reassessment for gluten tolerance allowing with time the reintroduction of increasing amounts of gluten.

scholarly journals Interleukin-1 receptor-induced PGE2 production controls acetylcholine-mediated cardiac dysfunction and mortality during scorpion envenomation

2020 ◽  
Vol 11 (1) ◽  
Author(s):  
Mouzarllem B. Reis ◽  
Fernanda L. Rodrigues ◽  
Natalia Lautherbach ◽  
Alexandre Kanashiro ◽  
Carlos A. Sorgi ◽  
...  
Keyword(s):  
Heart Failure ◽  
Cardiac Dysfunction ◽  
Acetylcholine Release ◽  
Interleukin 1 ◽  
Clinical Manifestations ◽  
Prostaglandin E ◽  
High Dose ◽  
Scorpion Envenomation ◽  
Risk Of Death ◽  
Include Heart Failure

Abstract Scorpion envenomation is a leading cause of morbidity and mortality among accidents caused by venomous animals. Major clinical manifestations that precede death after scorpion envenomation include heart failure and pulmonary edema. Here, we demonstrate that cardiac dysfunction and fatal outcomes caused by lethal scorpion envenomation in mice are mediated by a neuro-immune interaction linking IL-1 receptor signaling, prostaglandin E2, and acetylcholine release. IL-1R deficiency, the treatment with a high dose of dexamethasone or blockage of parasympathetic signaling using atropine or vagotomy, abolished heart failure and mortality of envenomed mice. Therefore, we propose the use of dexamethasone administration very early after envenomation, even before antiserum, to inhibit the production of inflammatory mediators and acetylcholine release, and to reduce the risk of death.

scholarly journals Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Jeong-Seon Lee ◽  
Joong-Gon Kim ◽  
Soyoung Lee
Keyword(s):  
Polyarteritis Nodosa ◽  
Clinical Manifestations ◽  
Long Term Outcomes ◽  
Childhood Onset ◽  
Laboratory Findings ◽  
Clinical Presentations ◽  
Long Term Prognosis ◽  
Digital Amputation

AbstractChildhood-onset polyarteritis nodosa (PAN) is a rare and systemic necrotising vasculitis in children affecting small- to medium-sized arteries. To date, there have been only a few reports because of its rarity. Thus, we aimed to investigate the clinical manifestations, laboratory findings, treatment, and long-term outcomes in patients with childhood-onset PAN and to evaluate the usefulness of the paediatric vasculitis activity score (PVAS). We retrospectively analysed the data of nine patients with childhood-onset PAN from March 2003 to February 2020. The median ages at symptom onset, diagnosis, and follow-up duration were 7.6 (3–17.5), 7.7 (3.5–17.6), and 7.0 (1.6–16.3) years, respectively. All patients had constitutional symptoms and skin manifestations, while five exhibited Raynaud’s phenomenon. Organ involvement was observed in one patient. The median PVAS at diagnosis was 7 (range: 2–32). Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived, but three patients with high PVAS at diagnosis experienced irreversible sequelae, including intracranial haemorrhage and digital amputation. In conclusion, early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. This study suggests that high PVAS score at diagnosis may be associated with poor prognosis.

scholarly journals Autoimmune encephalitis in a South Asian population

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Nilanka Wickramasinghe ◽  
Dhanushka Dasanayake ◽  
Neelika Malavige ◽  
Rajiva de Silva ◽  
Thashi Chang
Keyword(s):  
Sri Lanka ◽  
Psychiatric Symptoms ◽  
South Asians ◽  
Laboratory Data ◽  
Clinical Manifestations ◽  
Autoimmune Encephalitis ◽  
Asian Population ◽  
South Asian Population ◽  
Clinical Presentations

Abstract Background Autoimmune encephalitis (AE) is now considered a main, potentially curable cause of encephalitis, but remains conspicuously underreported from South Asia. We studied the clinical characteristics in relation to their antibody status and outcomes of patients presenting with AE in Sri Lanka. Methods Patients admitting to government hospitals who were clinically suspected of AE by an on-site neurologist were prospectively recruited over a period of 12 months. Sera and cerebrospinal fluid were tested for NMDAR, AMPAR1, AMPAR2, LGI1, CASPR2, GABARB1/B2 antibodies (Ab) using commercial cell-based assays. Demographic, clinical and laboratory data were compiled into an investigator-administered proforma. Patients were reviewed at 1 year follow up either in person or via telephone. Results One-hundred and forty-two patients from 21 of 25 districts in Sri Lanka (median age = 20.5 years; range 1–86 years; females = 61.3%) were recruited. Of them, 65 (45.8%; median age = 19 years; range 1–86 years; females = 64.6%) fulfilled diagnostic criteria for probable NMDAR-antibody encephalitis (NMDARE) and 6 (4.2%; median age = 44 years; range 28–71 years; females = 83.3%) limbic encephalitis (LE). Abnormal behaviour (95.3%), seizures (81.5%) and movement disorders (69.2%) were the most frequent clinical manifestations of probable NMDARE. NMDAR-antibodies were detectable in 29 (44.6%) and not detectable in 36 in CSF of probable-NMDARE patients. Abnormal EEG was more frequent (p = 0.003) while a worse outcome (OR = 2.78; 95% CI = 0.88–9.09) and deaths (OR = 2.38; 95% CI = 0.67–8.33) were more likely in antibody-negative than antibody-positive probable-NMDARE. Most patients with LE had amnesia (50%) and/or confusion (100%) with agitation (83.3%) and seizures (100%) but none had detectable antibodies to any of the antigens tested. Conclusions NMDARE is the commonest type of AE among South Asians as is the case worldwide. Clinical presentations of NMDARAb-positive and NMDARAb-negative AE patients do not significantly differ but EEG may be a useful marker of an autoimmune basis for psychiatric symptoms.

scholarly journals AB0249 POSITIVE EFFECT OF ANTIRHEUMATIC THERAPY ON THE COURSE OF CHRONIC HEART FAILURE IN PATIENTS WITH EARLY RHEUMATOID ARTHRITIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1424.3-1425
Author(s):  
I. Kirillova ◽  
D. Novikova ◽  
T. Popkova ◽  
Y. Gorbunova ◽  
E. Markelova ◽  
...  
Keyword(s):  
Heart Failure ◽  
Chronic Heart Failure ◽  
Left Ventricle ◽  
Disease Activity ◽  
Diastolic Function ◽  
Clinical Course ◽  
Clinical Manifestations ◽  
Antirheumatic Therapy ◽  
Early Ra ◽  
Lv Diastolic Function

Background:Objectives:to evaluate the effect of antirheumatic therapy according to the “treat to target” strategy on the course of chronic heart failure (CHF) in patients with early RA.Methods:The study included 22 patients CHF with valid diagnosis of RA (criteria ACR / EULAR, 2010), 17 (77%) of women, median (Me) age - 60 years, Me disease duration - 7 months; IgM seropositive for rheumatoid factor 10 (45%) and / or antibodies to the cyclic citrulline peptide 22 (100%), DAS28-5.6 [4,8;6,5]. CHF verified in accordance the recommendations for the diagnosis and treatment of CHF Society of Specialists in Heart Failure (2013). The concentration of NT-proBNP was determined by electrochemiluminescence. For all patients was started methotrexate (MT) therapy with a rapid increase in the dose to 30 mg per week subcutaneously. If the MT was not effective enough, after 3 months a biological Disease-Modifying Anti-Rheumatic Drug (bDMARDs) was added to the therapy, predominantly TNF-alpha inhibitors. After 18 months, 10 (45%) patients were in remission and low disease activity, 6 (60%) of patients underwent MT therapy in combination with bDMARDs.Results:In baseline CHF with preserved EF was revealed in 21 (95%) patients, in 1 patient - CHF with reduced EF. After 18 months there was a positive dynamics of improvement of clinical symptoms, echocardiographic indicators (decrease the size of the left atrium (LА) and the index of end-systolic volume of LА, IVRT, E’ LV), diastolic function of the left ventricle (LV). There was no decompensation of CHF. LV diastolic function normalized in 7 (32%) patients who reached the target level of blood pressure, remission (n = 5) and low (n = 2) disease activity, mainly in the treatment of MT and bDMARDs. In patients with RA and CHF, the level of NT-proBNP decreased from 192.2 [151.4; 266.4] to 114.0 [90.4; 163.4] pg / ml (p <0.001), normalized in 16 of 22 (73%) patients (p <0.001) with remission or low RA activity. In 5 (22%) patients, the clinical manifestations of CHF regressed, LV diastolic function and NT-proBNP level normalized.Conclusion:In patients with early RA and CHF anti-rheumatic therapy improves the clinical course of CHF. There were an improvement in the clinical course of CHF, diastolic function of the left ventricle and a decrease in NT-proBNP.Disclosure of Interests:None declared

scholarly journals New biohumoral markers of differential diagnosis in patients with suspected heart failure

2010 ◽  
Vol 63 (5-6) ◽  
pp. 387-392
Author(s):  
Radomir Matunovic ◽  
Zdravko Mijailovic ◽  
Milorad Rabrenovic ◽  
Violeta Rabrenovic
Keyword(s):  
Heart Failure ◽  
Congestive Heart Failure ◽  
Natriuretic Peptide ◽  
Correct Diagnosis ◽  
Clinical Manifestations ◽  
High Sensitivity ◽  
Renal Diseases ◽  
Significant Implication ◽  
Acute Patient ◽  
Degree Of Certainty

Introduction. In the regular clinic practice, the assessment of the cause of dispnea is a dilemma which has a significant implication in both the estimation of prognosis and treatment of the patient. In emergency cases, when most necessary, it is often very difficult to determine whether dispnea was caused by a heart or lung disease. Role of natriuretic peptide in patients with dispnea. An acute patient with dispnea might suffer serious consequences of inadequately established diagnosis so congestive heart failure (CHF) has to be diagnosed quickly and precisely in the ER. Unfortunately, symptoms and signs of CHF are unspecific, it is sometimes impossible to obtain an adequate anamnesis and diagnosic procedures currently applied are either insufficiently precise or provide scarce information or can not always be performed under appropriate conditions. On the basis of previous findings, it has been proved that brain natriuretic peptide (BNP) can considerably contribute to the establishment of correct diagnosis as well as to the possibility of introducing an adequate therapy for those patients. However, the real value of those peptides should be estimated in relation to other clinical manifestations and indicators and the specifics of examined patients including the age, gender and the presence or absence of pulmonary or renal diseases. Natriuretic peptide in accessement gradient of heart failure. Determination of natriuretic peptide has represented most probably the greatest progress in diagnosing the heart failure since the introduction of echocardiography into practice. Its high sensitivity and negative predicted value makes it a valid test for excluding congestive heart failure with a very high degree of certainty.

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