Polycythemia vera as a rare cause of hypertension in a young man

2021 ◽  
Vol 14 (6) ◽  
pp. e242008
Author(s):  
Maria Vetluzhskaya ◽  
Antonina Abramova ◽  
Karine Oganesyan ◽  
Valery Podzolkov

Polycythemia vera (PV) is an orphan haematological disease and one of the most common myeloproliferative diseases, with the incidence rate of about 0.4–2.8 cases per 100 000 population per year. In patients, proliferation of all three haematopoietic lineages is observed, typically with the development of erythrocytosis. As a rule, PV occurs in patients aged 60–70 years, slightly more often in men. The main clinical signs of PV are weakness, significant burning sensation in fingers and palms due to the increased blood viscosity and microcirculation disorders, discomfort in the left hypochondrium due to splenomegaly at the background of extramedullary haematopoietic sites development, as well as gross vascular complications (thrombosis) of various localisation. Our clinical case represents a rare cardiac manifestation of the PV in a young man.

2020 ◽  
pp. 97-99
Author(s):  
U. V. Kukhtenko ◽  
O. A. Kosivtsov ◽  
L. A. Ryaskov ◽  
E. I. Abramian

A clinical case of successful surgical treatment of a patient with a giant cervical retrosternal nontoxic goiter with severe cardiac pathology is presented. Thyroidectomy from cervical access without sternotomy was performed. At the follow-up examination 5 months after the operation, instrumental and clinical signs of disease relapse were not detected.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1827.3-1827
Author(s):  
O. Alekseeva ◽  
N. Yudkina ◽  
A. Demina ◽  
A. Volkov ◽  
E. Nasonov

Background:Systemic sclerosis (SSc) can lead to vascular complications such as digital ulcers or pitting scars (DU/PS). These changes develop in most patients with SSc and exacerbate their condition. However, there are no methods for dynamic assessment of the vascular involvement. The dynamics of capillaroscopic changes is very slow.Objectives:The aim of the study was to compare blood flow parameters of digital arteries in SSc patients and healthy individuals and to compare with nailfold capillaroscopy and clinical signs of ischemia (DU/PS).Methods:32 SSc patients, mean age 49,5 [42,0; 59,0] yrs and 26 ‘healthy’, mean age 43,5 [33,0; 57,0], were included. Groups of patients differed by gender and age. The exclusion criterion was the presence of obliterating vascular disease of the upper extremities. An Esaote MyLab Twice US system with 22 MHz linear probe was used. A total of SSc patients and controls underwent Color Doppler ultrasonography (CDUS) of 376 (256 + 208) digital arteries to compare blood flow velocity, resistive indices (RIs) and presence of occlusion. Nailfold capillaroscopy, clinical and laboratory data were also evaluated.Results:In digital arteries, pulsatility index (PI), peak systolic velocity (PSV) and end-diastolic velocity (EDV) were significantly lower and RI higher in SSc patients compared with controls (PSV: 13,28 [9,88; 16,7] vs17,45 [12,65; 22,5] cm/s, p=0,008; EDV: 2,68 [1,78, 4,05] vs 6,37 [4,75; 8,5] cm/s, p=0,000; RI: 0,78 [0,69; 0,81] vs 0,68 [0,59; 0,74], p=0,005; PI: 1,73 [1,32; 2,19] vs 1,22 [0,99; 1,55], p=0,002).We did not find any correlation between two methods. Also, we did not reveal any correlation between DU/PS, clinical, laboratory data and CDUS, but we found relationship between DU/PS and avascular areas or capillaroscopic findings (r= 0,37, p=0,045 and r= 0,40, p=0,03 correspondingly).Conclusion:Blood flow is significantly decreased in digital arteries in SSc, but clinical features of vasculopathy depend on microcirculatory disorders. It is important to continue research to find methods for dynamic evaluation of microcirculatory changes.References:noDisclosure of Interests:None declared


2017 ◽  
Vol 62 (3) ◽  
pp. 122-125 ◽  
Author(s):  
Enrico M Zardi ◽  
Silvia Spoto ◽  
Luciana Locorriere ◽  
Giulio Cacioli ◽  
Silvia Mazzaroppi ◽  
...  

Introduction Platypnea-orthodeoxia syndrome is a combination of positional dyspnoea and hypoxemia; it is caused by several cardiac, pulmonary and hepatic diseases. Case presentation In this study, we describe a 77-year-old female affected by unexplained dizziness and hypoxia that exacerbated in upright position. After diagnosing platypnea-orthodeoxia syndrome and excluding all possible causes (liver cirrhosis, acute and chronic pulmonary diseases and arteriovenous malformations), the origin of the syndrome was individuated in the presence of a patent foramen ovale with right-to-left shunt. Endovascular patent foramen ovale closure permitted the resolution of symptoms and disappearance of platypnea-orthodeoxia syndrome. Conclusion Although patent foramen ovale may be present since birth without giving clinical signs, it may represent a common enough cause of platypnea-orthodeoxia syndrome and other vascular complications in the elderly.


Doctor Ru ◽  
2020 ◽  
Vol 19 (10) ◽  
pp. 57-60
Author(s):  
A.V. Vitebskaya ◽  
◽  
Chernova E.V. Chernova ◽  

Objective of the Paper: to describe a clinical case of paediatric hypophosphatasia (HPP) and identify clinical signs, most characteristic of the paediatric HPP form. Key Points. HPP is an congenital rickets-like disease caused by reduced activity of tissue-nonspecific alkaline phosphatase (ALP). According to the time of manifestation, there are perinatal, infant, paediatric, and adult HPPs. The article describes a clinical case of paediatric HPP in a 3.5-year old boy. HPP was diagnosed due to reduced ALP and characteristic X-ray findings. The diagnosis was confirmed with DNA testing: compound heterozygous mutation in с.571 G>A/с.144_148dup of ALPL was found. Conclusion. Typical findings in paediatric HPP are growth retardation and muscular hypotonia, motor retardation; gait disturbances, myalgia, marked fatigue causing limited period of walking; rachitic deformations, premature loss of milk teeth with unchanged roots, respiratory disturbances, and frequent bronchopulmonary disorders. Keywords: hypophosphatasia, children, alkaline phosphatase.


2015 ◽  
Vol 125 (2) ◽  
pp. 65-71
Author(s):  
Dorota Mrozek-Budzyn ◽  
Renata Majewska ◽  
Agnieszka Kiełtyka ◽  
Małgorzata Augustyniak

Abstract Introduction. Poland is a member of the WHO European Region where a complete eradication of measles and rubella is planned to be finished by 2015. Poland accounted for 99% of all reported rubella cases in 27 EU/EEA countries in 2013. It is a good time to evaluate whether the established Polish vaccination strategy was sufficient to reach the goal of rubella elimination in the near future. Aim. The aim of this study was to analyze the epidemiology of rubella in Poland when the disease outbreak took place in 2013, to determine the reasons of that situation and to find the solution for future rubella elimination strategies. Material and methods. To analyze the epidemiology of rubella in Poland during the disease outbreak in 2013 the authors used rubella surveillance data collected by the Provincial and National Notifiable Disease Reporting System in 2004-2013. The information at the provincial level derived from one of the 16 provinces (Malopolska). The data on MMR vaccination coverage in 2003-2012 derived from the National Surveillance System. The percentages of rubella cases and vaccine coverage between Poland with Malopolska province were compared. Results. The outbreak started in late 2012 and continued through 2013, when 38548 rubella cases (incidence rate 100.1/ 100 000) were notified. Geographically, rubella cases were reported from the entire country, with the highest incidence rate in Malopolska province (254.9/100 000). Only 5 cases from Malopolska and 120 in whole country were laboratory confirmed, the remaining 99.7% were reported solely on the basis of clinical signs. The vaccination coverage was not sufficient to protect the population against rubella outbreak in Poland, especially among adolescents and young adult males. Conclusions. The strengthening of routine immunization program and implementation of some additional vaccination campaigns in young adults as well as laboratory confirmation of all suspected cases are the challenges that will have to be met to eliminate rubella in Poland


Author(s):  
R.A. Burya ◽  
◽  
A.A. Fil ◽  
E.L. Sorokin ◽  
◽  
...  

The article describes a clinical case of diagnosis and detection of primary open-angle glaucoma (POAG) in a patient after previously performed anterior dosed radial keratotomy (ADRK). The reasons that hampered the primary diagnosis of POAG in the presented patient at an outpatient appointment with an ophthalmologist were: inadequate assessment of the ratio of the state of the lenses and low vision; a falsely underestimated level of tonometric intraocular pressure (IOP), as well as the absence of an in-depth ophthalmoscopic assessment of the condition of the optic nerve head, failure to comply with the mandatory perimetric examination in such cases. It should be especially remembered that there is a high likelihood of underestimation of applanation tonometry indices after performing ADRK, therefore, the assessment of its results should be treated with caution, identifying other clinical signs characteristic of glaucoma. This will make it possible to diagnose glaucoma in time and prescribe pathogenetic treatment in a timely manner. Key words: anterior dosed radial keratotomy, primary open-angle glaucoma, intraocular pressure.


Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
C Njeru ◽  
A Migowa

Abstract Background Kawasaki disease (kDa) is a childhood vasculitides that affects small and medium-sized arteries. It is self-limiting but when left untreated can cause coronary artery aneurysms in about 25% of children1. The diagnosis is clinical and is made with the criteria of fever for at least five days, and at least four out of five other clinical signs: bilateral non-exudative conjunctivitis, oral mucous membrane changes, peripheral extremity changes, polymorphous rash, and cervical lymphadenopathy. Incomplete kDa is diagnosed with fever for at least five days and at least 2–3 of the principal signs, with suggestive lab investigations and lack of an alternative diagnosis1. kDa is named after Dr Tomisaku Kawasaki who first described it in 1967 in Japan2. The highest incidence of kDa continues to be reported among Asian children, with an incidence rate of 264.8 per 100 000 population aged 0–4 years as per Japan’s latest nationwide survey3. In Africa, the true incidence is unknown but several case reports have been published. A 2016 paper by Gorrab et al.4 found that the annualized incidence rate of kDa in the Maghreb children living in Quebec (18.49/year/100 000 children under 5 years of age) was 4–12 times higher than reported in their countries of origin- Tunisia, Morocco, and Algeria (0.95, 4.52, and 3.15, respectively) suggesting a likelihood of under-diagnosis. This case series sought to highlight the characteristics, presentation, and management of patients diagnosed with kDa in a tertiary hospital in Kenya. Methods This was a retrospective cross-sectional study carried out by reviewing the charts of all the patients with a discharge diagnosis of kDa from January 2013 to December 2017 at the Aga Khan University Hospital, Nairobi. Their demographics, presentation, diagnostic work-up, and management are reported. Analysis was done by descriptive statistics using the Microsoft Excel 2016 Application. Results A total of 15 cases were identified and the patient characteristics and presentation are as tabulated below: In addition to elevated inflammatory markers (C reactive protein and/or Erythrocyte Sedimentation Rate), a significant number of the patients also had sterile pyuria (9/9), hypoalbuminemia (8/10), thrombocytosis (8/15), and anaemia (11/15). Nine out of eleven had negative blood cultures. Fourteen out of the fifteen patients had echocardiograms done during admission. Only one patient was found to have abnormal findings of bilaterally dilated coronaries arteries. Five patients had at least one documented repeat echocardiogram. Fourteen patients received Intravenous Immunoglobulin (IVIG), with 13 of them responding to treatment. No adverse effects were reported after treatment. One patient did not improve and needed a second dose of IVIG and intravenous methylprednisone (30 mg/kg). Fourteen patients received aspirin but dosing varied from high (80–90 mg/kg/day) to moderate (30–50 mg/kg/day) to low dose (3 mg/kg/day). One patient on high-dose aspirin was noted to have developed symptoms consistent with aspirin-induced bronchospasm and was changed to low dose. Conclusion This case series highlights the presence of kDa in the Kenyan pediatric population with patient characteristics similar to what is reported globally. Diagnosis was made after a mean of about 7 days, possibly due to low awareness of the disease among healthcare professionals. Management with IVIG in most cases was successful but more guidance is needed around the use of steroids and the dosing of aspirin.


Blood ◽  
1961 ◽  
Vol 18 (6) ◽  
pp. 717-726 ◽  
Author(s):  
CHARLES A. HALL ◽  
Alexander E. Finkler ◽  
Edward S. Allen ◽  
Booker T. Moore

Abstract 1. The plasma disappearance of a small intravenous dose of radioactive vitamin B12 was determined in control subjects and in patients with various blood disorders. 2. A delayed, sometimes irregular, disappearance was observed in the majority of patients with acute and chronic myelogenous leukemia, myeloid metaplasia, and polycythemia vera. 3. Disappearance was normal in the lymphogenous leukemias, secondary polycythemia and relative polycythemia. 4. The abnormalities observed are believed to indicate an abnormality of vitamin B12 metabolism common to the diseases of the myeloproliferative group and are further evidence of the close relationship between these diseases.


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