scholarly journals Long-term consequences of Lyell's syndrome suffered in childhood

2021 ◽  
pp. 79-82
Author(s):  
O.M. Okhotnikova ◽  
◽  
T.M. Tkachоva ◽  
A.S. Andriyko ◽  
I.O. Kurian ◽  
...  
Keyword(s):  
Clinical Case ◽  
Chronic Sinusitis ◽  
Treatment Strategies ◽  
Chronic Obstructive ◽  
Long Term Consequences ◽  
Lyell’S Syndrome ◽  
The Right ◽  
Chronic Conjunctivitis ◽  
Lyell's Syndrome

The article provides a review of literature on the long-term consequences of Lyell's syndrome (one of the forms of toxic epidermal necrolysis). Among them: chronic eczema with xerosis and itching, skin depigmentation, hypertrophic and keloid scars, nail lesions, eyes, including dry eye syndrome and chronic conjunctivitis with/without obliteration of the nasolacrimal canal, kidney disease, lung disease, Sjogren's syndrome, Hashimoto's thyroiditis and others. Clinical case. The authors present their clinical case of an 11-year-old girl who suffered from Lyell's syndrome at the age of 8 and has been observed for the past three years, share their clinical and laboratory-instrumental observations and treatment strategies. In particular, they report about the revision of the clinical diagnosis with which the girl was admitted to the OHMADYT hospital, from BA to chronic secondary obstructive bronchitis, the detection of chronic conjunctivitis with obliteration of the nasolacrimal canals, chronic sinusitis, secondary immune deficiency, keloid skin scars. Conclusions. Despite constant rehabilitation therapy and a significant improvement in the general condition of the girl, it was not possible to fully restore the function of mucociliary transport, as a result ventilation of small bronchi gradually deteriorates, bronchiectasis is observed in the basal segments of the right lung, a polyserial strain S. aureus is formed, which is constantly sown from the child's sputum; it was not possible to fully restore the patency of the nasolacrimal canals and achieve a stable remission of chronic sinusitis. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: Lyell's syndrome, children, Long-term consequences, chronic obstructive bronchitis, obesity, treatment.

scholarly journals Long-term consequences of Lyell's syndrome suffered in childhood

2021 ◽  
pp. 69-78
Author(s):  
O.M. Okhotnikova ◽  
◽  
T.M. Tkachоva 0000-0001-8951-7767 ◽  
A.S. Andriyko ◽  
I.O. Kurian ◽  
...  
Keyword(s):  
Clinical Case ◽  
Chronic Sinusitis ◽  
Treatment Strategies ◽  
Chronic Obstructive ◽  
Long Term Consequences ◽  
Lyell’S Syndrome ◽  
The Right ◽  
Chronic Conjunctivitis ◽  
Lyell's Syndrome

The article provides a review of literature on the long-term consequences of Lyell's syndrome (one of the forms of toxic epidermal necrolysis). Among them: chronic eczema with xerosis and itching, skin depigmentation, hypertrophic and keloid scars, nail lesions, eyes, including dry eye syndrome and chronic conjunctivitis with/without obliteration of the nasolacrimal canal, kidney disease, lung disease, Sjogren's syndrome, Hashimoto's thyroiditis and others. Clinical case. The authors present their clinical case of an 11-year-old girl who suffered from Lyell's syndrome at the age of 8 and has been observed for the past three years, share their clinical and laboratory-instrumental observations and treatment strategies. In particular, they report about the revision of the clinical diagnosis with which the girl was admitted to the OHMADYT hospital, from BA to chronic secondary obstructive bronchitis, the detection of chronic conjunctivitis with obliteration of the nasolacrimal canals, chronic sinusitis, secondary immune deficiency, keloid skin scars. Conclusions. Despite constant rehabilitation therapy and a significant improvement in the general condition of the girl, it was not possible to fully restore the function of mucociliary transport, as a result ventilation of small bronchi gradually deteriorates, bronchiectasis is observed in the basal segments of the right lung, a polyserial strain S. aureus is formed, which is constantly sown from the child's sputum; it was not possible to fully restore the patency of the nasolacrimal canals and achieve a stable remission of chronic sinusitis. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: Lyell's syndrome, children, Long-term consequences, chronic obstructive bronchitis, obesity, treatment.

scholarly journals Early Changes and Indicators Characterizing Lung Aging in Neonatal Chronic Lung Disease

2021 ◽  
Vol 8 ◽  
Author(s):  
Jennifer Sucre ◽  
Lena Haist ◽  
Charlotte E. Bolton ◽  
Anne Hilgendorff
Keyword(s):  
Lung Disease ◽  
Chronic Lung Disease ◽  
Health Care Professionals ◽  
Treatment Strategies ◽  
Oxygen Toxicity ◽  
Growth Factor Signaling ◽  
Inflammatory Processes ◽  
Long Term Consequences ◽  
Neonatal Chronic Lung Disease

Infants suffering from neonatal chronic lung disease, i.e., bronchopulmonary dysplasia, are facing long-term consequences determined by individual genetic background, presence of infections, and postnatal treatment strategies such as mechanical ventilation and oxygen toxicity. The adverse effects provoked by these measures include inflammatory processes, oxidative stress, altered growth factor signaling, and remodeling of the extracellular matrix. Both, acute and long-term consequences are determined by the capacity of the immature lung to respond to the challenges outlined above. The subsequent impairment of lung growth translates into an altered trajectory of lung function later in life. Here, knowledge about second and third hit events provoked through environmental insults are of specific importance when advocating lifestyle recommendations to this patient population. A profound exchange between the different health care professionals involved is urgently needed and needs to consider disease origin while future monitoring and treatment strategies are developed.

scholarly journals A clinical case of arrhythmogenic right ventricular dysplasia

2021 ◽  
Vol 2 (1) ◽  
pp. 109-114
Author(s):  
E. V. Solovyoval ◽  
N. A. Popova ◽  
T. V. Vlasoval ◽  
M. L. Gorbunova ◽  
L. N. Antsygina
Keyword(s):  
Right Ventricular ◽  
Clinical Case ◽  
Ventricular Myocardium ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Myocardial Diseases ◽  
Conduction Disorders ◽  
Right Ventricular Dysplasia ◽  
Common Causes ◽  
The Right

Arhythmogenic right ventricular dysplasia (ADP) refers to hereditary myocardial diseases, in which there are structural and functional disorders in the right ventricular myocardium, causing rhythm and conduction disorders, including fatal ventricular arrhythmias. ADP is considered one of the most common causes of sudden cardiac death in young people and people who are engaged in sports. However, in practice, there are cases of this disease in people of an older age category. Diagnosis of ADP is still difficult due to the possible long-term asymptomatic course of the disease. The article describes a clinical case of ADP in a 48-year-old man.

scholarly journals Bilateral polysegmentary pneumonia caused by SARS-CoV-2 in a transplanted liver recipient

2020 ◽  
Vol 11 (2) ◽  
pp. 101-106
Author(s):  
Roman V. Ishenko ◽  
Sergei V. Golovincki ◽  
Artur R. Akhmedianov ◽  
Sergei E. Voskanyan ◽  
Ilya Yu. Kolyshev
Keyword(s):  
Infectious Disease ◽  
Clinical Case ◽  
Organ Transplant ◽  
Immunosuppressive Drugs ◽  
Right Lobe ◽  
Coronavirus Infection ◽  
Disease Hospital ◽  
The Right ◽  
Liver Recipient

Background. In December 2019, the humanity met a previously unknown infectious disease (COVID-19) caused by a new coronavirus called SARS-CoV-2. An important role in the treatment of COVID-19 belongs to anti-inflammatory and immunosuppressive drugs. In this regard, the cases of the disease in patients undergoing long-term immunosuppressive therapy, for example, organ transplant recipients, are of particular interest. We present our clinical observation of COVID-19 in a liver recipient patient, which, apparently, is the first in the Russian Federation. Clinical case description A 54-year-old man, 10 years ago at the A.I. Burnazyan Center underwent transplantation of the right lobe of the liver after resection of hepatocellular carcinoma, T2N0M0, and due to liver cirrhosis as a result of НСV hepatitis. At the time of hospitalization, he had been constantly receiving immunosuppressive monotherapy with everolimus. The patient was transferred to an infectious disease hospital due to a positive PCR test for SARS-CoV-2 RNA. No signs of respiratory failure were found upon admission. Subsequently, a mild course of COVID-19 was observed, without signs of an acute inflammatory reaction, with normal CRP values and a slight increase of ferritin. 7 days after the treatment, the patient was discharged for outpatient observation. Conclusion. This clinical case is of interest not only by the success of the treatment of the new coronavirus infection COVID-19 in an immunocompromised patient a recipient of a liver transplant, but also by the fact that the disease manifested itself primarily as a transient increase in hepatic aminotransferases, which can be attributed to the gastrointestinal manifestations of COVID-19.

Clinical case of Alloplant biomaterial use in surgical treatment of ophthalmological complications in patient with Lyell's syndrome

2019 ◽  
Vol 17 (1) ◽  
pp. 141-144
Author(s):  
R. Z. Kadyrov ◽  
◽  
R. E. Primov ◽  
I. R. Karachurina ◽  
◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Clinical Case ◽  
Lyell’S Syndrome ◽  
Lyell's Syndrome

scholarly journals Pro/Con: A unified conclusion-Inhaled corticosteroids for chronic obstructive pulmonary disease

2019 ◽  
Vol 2 (1) ◽  
pp. 46-47
Author(s):  
Margaret F. Ragland ◽  
Donald A. Mahler ◽  
Barry J. Make
Keyword(s):  
Chronic Obstructive Pulmonary Disease ◽  
Pulmonary Disease ◽  
Inhaled Corticosteroids ◽  
Symptom Burden ◽  
Chronic Obstructive ◽  
Con A ◽  
Obstructive Pulmonary Disease ◽  
Long Acting ◽  
The Right

ICS are like any other drug: they have benefits and they have risks.6,7 In the right patient, often the patient with frequent exacerbations and severe uncontrolled symptoms, an inhaler regimen, including dual long-acting bronchodilators and an ICS may be the right choice. For the patient with infrequent exacerbations, low symptom burden, and recent pneumonia that caused hospitalization, an inhaler regimen that contains an ICS may bring more risk than benefit. In addition, analysis of recent data, including the WISDOM8 trial, has provided evidence that ICS may be most beneficial in the immediate postexacerbation period. Further, ICS may be safely discontinued in patients with few exacerbations and stable symptoms.9 As with any long-term medication, a nuanced risk-benefit calculation by the prescribing physician and informed discussion with the patient are ultimately the best ways to balance the use of ICS that have benefits in some patients and risks in others.

Long-term outcomes of endovascular treatment of isolated subclavian artery aneurysms

Vascular ◽  
2020 ◽  
pp. 170853812097524
Author(s):  
Gang Fang ◽  
Yuan Fang ◽  
Xin Xu ◽  
Jue Yang ◽  
Ting Zhu ◽  
...  
Keyword(s):  
Endovascular Treatment ◽  
Subclavian Artery ◽  
Treatment Strategies ◽  
Limb Ischemia ◽  
Surgical Trauma ◽  
Covered Stents ◽  
Long Term Outcomes ◽  
The Right

Objectives The aim of the present study was to review our institutional experience of endovascular treatment for isolated subclavian artery aneurysms and evaluate the long-term outcomes. Methods A retrospective review of all patients with isolated subclavian artery aneurysms who underwent endovascular treatment between March 2008 and March 2020 was performed. The demographics, aneurysmal characteristics, treatment strategies, and in-hospital and follow-up outcomes were recorded and then analyzed. Results From March 2008 to March 2020, 35 isolated subclavian artery aneurysms were endovascularly treated at our institution. Atherosclerosis was the most common cause of aneurysms in this series. Most aneurysms were intrathoracic (91.4%) and located at the right side (77.1%). There were 26 true aneurysms, seven pseudoaneurysms, and two ruptured isolated subclavian artery aneurysms. Five types of endovascular strategies were performed. Covered stent placement across the aneurysm was the most (54.3%) commonly used method. Technical success was achieved in all patients. The median postoperative in-hospital stay was 4.0 days (range, 1–15 days). One patient died after discharge but within 30 days of surgery due to myocardial infarction. The median follow-up time was 62.0 months (range, 3–132 months). No death, stroke, stent fractures, or severe upper limb ischemia developed during the follow-up period. The cumulative survival rate at five years was 97.1%. The overall complication rate was 25.7% and one-third of complications (8.6%) required reinterventions. Conclusions Endovascular treatment for isolated subclavian artery aneurysms is safe, effective and technically achievable in most patients. Short- and long-term outcomes are promising. Reasonable and flexible use of covered stents can also get satisfactory outcomes in some complicated lesions such as isolated subclavian artery aneurysms located at the origin of the right subclavian artery, avoiding the huge surgical trauma caused by conventional open repair.

P0327SUCCESSFUL ACHIEVMENT OF LONG-TERM COMPLETE REMISSION OF LUPUS NEPHRITIS IN A PATIENT WITH SEVERE SYSTEMIC LUPUS ERYTHEMATOSUS AND SECONDARY INFECTIOUS COMPLICATOINS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Anna Bespalova ◽  
Tatiana Bondarenko ◽  
Natalya Kozlovskaya
Keyword(s):  
Complete Remission ◽  
Immunosuppressive Therapy ◽  
Shoulder Joint ◽  
Clinical Case ◽  
Infectious Complications ◽  
High Dose ◽  
Laboratory Examination ◽  
Limited Range Of Motion ◽  
The Right

Abstract Background and Aims A significant contribution to the structure of mortality in SLE is made by infectious complications, which complicate the course of SLE and complicate the management of patients due to the impossibility of prescribing immunosuppressive therapy in full. Method A clinical case of successful achievement of LN remission in a patient with severe SLE and secondary APS is described, despite the development of secondary infectious complications and the inability to fully immunosuppressive therapy. Results A 42-year-old woman has been observed at the clinic from autumn 2013 to the present. Since the end of Aug.2013 she got a severe dyspnea, generalized edema, ulcers of the legs, blood pressure persistently increased to 180/110 mm Hg, diuresis decreased. Laboratory examination at admission in Oct.2013: (table 1), polyserositis, non-inclusive thrombosis of the posterior tibial vein on the left. An immunological examination: anti-dsDNA 101.6 U/L, ANA 5.2, C3 complement fragment 0.29 g/L, antibodies to beta-2-glycoprotein-1 55.7 U/ml, positive lupus anticoagulant. Intravenous methylprednisolone at a dosе of 1000 mg/day for 3 days plus high dose CYC at a dosе of 800 mg/day for 1 days was started, followed by prednisone 60 mg/day. This intravenous pulsewas repeated after 3 weeks. Renal biopsy in Nov.2013: diffuse proliferative glomerulonephritis with 7% cellular crescents, ISN/RPS class IV LN. Positive dynamics in Dec.2013 (table 1). By mid-Dec.2013, the patient had complaints of pain and limited range of motion in the right shoulder joint, the appearance of edema in this region. According to the puncture of the joint cavity, MRI, purulent arthritis of the right shoulder joint was diagnosed with the formation of intermuscular phlegmon of this area. Sepsis was diagnosed. The patient underwent emergency surgical drainage of phlegmon, followed by repeated revisions of this area. Prescribed meropenem at a dose of 3 g/day. Implementation of the complete immunosuppressive therapy protocol was impossible; a gradual decrease in the dose of prednisone per os was started. Due to the continued activity of SLE, in January 2014, a repeated intravenous pulse methylprednisolone without СYС was performed. Since Dec.2013, the patient has not received cystostatics. But despite it positive dynamics in Feb.2014 (table 1) . In Mar.2014 current treatment was hydroxychloroquine 400 mg/day, prednisone 15 mg/day, azathioprine 100 mg/twid. After 2 years of complete remission of LN in April 2016: prednisone 10 mg/day, hydroxychloroquine 400 mg/day, azathioprine 50 mg/twid. After another 2 years of complete remission of LN in 2018: prednisone 5 mg/day, hydroxychloroquine 400 mg/day. During the observation, a complete clinical and laboratory remission of SLE was noted. Laboratory examination at admission in Oct.2019: complete remission of SLE (table 1). The patient takes prednisone 5 mg/day, hydroxychloroquine 400 mg/day. The patient has limited mobility of the right shoulder joint, but complete loss of function did not occur. According to the control MRI there are the formation of aseptic necrosis of the head of the right humerus. Conclusion Infectious complications remain one of the leading causes of mortality in patients with SLE. The uniqueness of this clinical case in the successful achievement of long-term complete remission of SLE, despite the secondary infectious complications. The success of achieving long-term remission of SLE is associated with the onset of immunosuppressive therapy as soon as possible from the debut of the disease, with the conduct in the maximum allowable volumes. The morphological picture with a small % cellular crescents was also a favorable factor.

scholarly journals Current outcomes and treatment of tetralogy of Fallot

F1000Research ◽  
2019 ◽  
Vol 8 ◽  
pp. 1530 ◽  
Author(s):  
Jelle P.G. van der Ven ◽  
Eva van den Bosch ◽  
Ad J.C.C. Bogers ◽  
Willem A. Helbing
Keyword(s):  
Right Ventricle ◽  
Right Ventricular ◽  
Ventricular Dysfunction ◽  
Pulmonary Regurgitation ◽  
Treatment Strategies ◽  
Right Ventricular Dysfunction ◽  
Pulmonary Valve Replacement ◽  
Term Survival ◽  
The Right

Tetralogy of Fallot (ToF) is the most common type of cyanotic congenital heart disease. Since the first surgical repair in 1954, treatment has continuously improved. The treatment strategies currently used in the treatment of ToF result in excellent long-term survival (30 year survival ranges from 68.5% to 90.5%). However, residual problems such as right ventricular outflow tract obstruction, pulmonary regurgitation, and (ventricular) arrhythmia are common and often require re-interventions. Right ventricular dysfunction can be seen following longstanding pulmonary regurgitation and/or stenosis. Performing pulmonary valve replacement or relief of pulmonary stenosis before irreversible right ventricular dysfunction occurs is important, but determining the optimal timing of pulmonary valve replacement is challenging for several reasons. The biological mechanisms underlying dysfunction of the right ventricle as seen in longstanding pulmonary regurgitation are poorly understood. Different methods of assessing the right ventricle are used to predict impending dysfunction. The atrioventricular, ventriculo-arterial and interventricular interactions of the right ventricle play an important role in right ventricle performance, but are not fully elucidated. In this review we present a brief overview of the history of ToF, describe the treatment strategies currently used, and outline the long-term survival, residual lesions, and re-interventions following repair. We discuss important remaining challenges and present the current state of the art regarding these challenges.

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