Myasthenia gravis in young children and adolescents: clinical signs and treatment options

2012 ◽  
Vol 43 (02) ◽  
Author(s):  
A Della Marina ◽  
H Trippe ◽  
S Lutz ◽  
G Shamdeen ◽  
U Schara
2018 ◽  
Vol 02 (01) ◽  
pp. E6-E9
Author(s):  
Adela Marina ◽  
Ulrike Schara

AbstractJuvenile myasthenia gravis (JMG) is an autoimmune disorder of neuromuscular transmission caused by production of antibodies against the postsynaptic membrane of the neuromuscular junction. Clinical signs in young children and adolescents range from isolated ocular symptoms to general muscular weakness and respiratory insufficiency. Clinical presentation of JMG in young children and adolescents shows distinct features compared to adults. Young children may show generalized muscular weakness already during the first two years of life, and in this group specific antibodies can be only slightly increased. Because of existing therapeutic options, an early diagnosis is important. In case of negative specific antibodies and onset of the first symptoms during infancy or early childhood, the diagnosis of a congenital myasthenic syndrome (CMS) must be considered and is not always clear to differentiate. Clinical symptoms, diagnostic procedures and therapeutic strategies with consideration of specificities of this age group are discussed.


2012 ◽  
Vol 22 (9-10) ◽  
pp. 856
Author(s):  
A. Della Marina ◽  
H. Trippe ◽  
S. Lutz ◽  
G.M. Shamdeen ◽  
U. Schara

Nutrients ◽  
2021 ◽  
Vol 13 (6) ◽  
pp. 1782
Author(s):  
Monika Grabia ◽  
Renata Markiewicz-Żukowska ◽  
Katarzyna Socha

Overweight and obesity are an increasingly common problem, not only among the healthy population, but also in adolescents with type 1 diabetes (T1DM). Excess body weight is related to many cardiometabolic complications as well as a high risk of metabolic syndrome (MetS). The purpose of this systematic review is to provide a concise and critical overview of the prevalence of MetS in children and adolescents with T1DM and, ultimately, to discuss prevention and treatment options. The study was conducted in accordance with PRISMA guidelines. This review shows that, apart from the growing percentage of overweight and obese children and adolescents with T1DM (on average 20.1% and 9.5%, respectively), the problem of the increasing incidence of MetS (range from 3.2 to 29.9%, depending on the criteria used) is one of the most important phenomena of our time. One of the methods of prevention and treatment is a combined approach: changing eating habits and lifestyle, but there are also reports about the beneficial effects of the gut microflora.


2021 ◽  
pp. 101269022110054
Author(s):  
Nicola Hague ◽  
Graeme Law

The world of football arguably brings together and unites people in support of their teams and countries, while inspiring young children and adolescents to dream of a professional career. Existing research in the field has sought to begin to understand what professional footballers experience on their journey through the game. However, much of this UK-based research has focused on first team players and their professional experiences, including transitions from youth team to first team and to retirement. This study, therefore, aimed to examine players during their youth academy scholarship at one English Championship club. This study focused on the transitional experiences of youth players from school to the academy and their resulting embodying of a footballer’s identity. Twelve semi-structured interviews with players aged 17–19, were conducted and then analysed by thematic analysis using figurational sociology concepts. Three different types of transition were identified. Among other reasons, early specialisation in football was a prevalent factor that partly influenced the way the players experienced their transition. The transition into the academy coincided with the transition from youth to adulthood that was arguably anything but linear as players managed the dominant sub-cultures present in the club.


1997 ◽  
Vol 87 (2) ◽  
pp. 66-69 ◽  
Author(s):  
A Glockenberg ◽  
E Sobel ◽  
JF Noël

Nonossifying fibroma is a benign, lytic lesion that occurs in young children and adolescents. Radiographically, the lesion is multilocular and sharply demarcated. It often occurs at the metaphyseal region of long bones of the lower extremity and is usually eccentrically located. Four cases of nonossifying fibroma occurring during the past 7 years are presented with a review of the literature.


2020 ◽  
Vol 24 (2) ◽  
pp. 244-249
Author(s):  
V.Yu. Pasik

Annotation. Respiratory diseases are relevant in pediatric practice, which is associated with its widespread and frequent complications, especially in young children. The aim of the study was to assess the diagnostic value of clinical symptoms, laboratory and ultra-sonographic parameters in pneumonia in children of the first 3 years of life. A retrospective study of medical records of 218 children who were hospitalized in the department for young children diagnosed with pneumonia for the period from 2016 to 2018. The average age of children was 11.67±9.97 months and it was within the range from 1 month to 3 years. The ration of boys and girls was practically identical (51.8% and 48.2% accordingly). The first group included children aged under one year (the average age is 4.57±0.84 months; n=88). The second group included children aged from 1 to 3 years (the average age is 18.2±4.25 months; n=130). To characterize the information content of clinical and laboratory symptoms the study has used objective parameters defined as the operational characteristics of tests. The most important operational characteristics of diagnostic methods included: sensitivity (Se, sensitivity) and specificity (Sp, specificity). To check the statistical hypothesis on differences of absolute and relative frequencies, fractions, and ratios in two independent samples, the criteria of хі-square (χ2) was used. While detailing an anamnesis, the disease was more often related to untimely treatment and outpatient care. Various data were obtained on the absolute and relative risk, as well as the sensitivity and specificity of the localization of pneumonia depending on age. Therefore, the incidence of bilateral pneumonia was considered an indicator of risk. On admission to hospital, the body temperature of patients was 38.2±0.66°С. Most of the complaints were on the unproductive or productive cough. Besides, in some cases, shortness of breath and runny nose were mentioned. Thus, in young children with pneumonia, a diagnostically significant clinical symptom is a bilateral lung impression (82.6%), compared with right-handed (15.1%) and left-handed (2.3%), which is significantly more common in children under 1-th year of life compared with patients 1–3 years; laboratory features are probably higher levels of liver-specific enzymes – ALT and AST in children under 1 year; ultrasonographic indicators associated with the presence of pneumonia in young children include increased liver size, gallbladder deformity, the presence of sediment in the gallbladder, dyskinesia of the biliary tract, thickening of the gallbladder wall; children under 1 year of age have a risk of liver enlargement and biliary dyskinesia.


2018 ◽  
Vol 91 (1) ◽  
pp. 37-41
Author(s):  
Horváth Adrienne ◽  
Papp Zsuzsanna Erzsébet

Abstract A broad spectrum of chemotherapy is being used in the therapy of childhood cancers, which may induce liver injury, impairing quality of life and efficacy of the treatment. History of, especially viral, liver diseases may increase toxicity. The aim of the paper is to assess the incidence, type and grade, predisposing factors and treatment options of drug-induced liver injury in children with malignant diseases under cytostatic therapy at the Hemato-Oncology Department of the Pediatric Clinic 2 from Targu-Mures, over a time period spanning from 2012 to 2017. The results of the study may serve as a foundation for such treatment strategies which would enable optimal outcomes with fewer cases of liver toxicity. During this period, we treated 26 patients with acute lymphoblastic leukemia (ALL), two patients with acute myeloblastic leukemia (AML), one patient with lymphoma and seven with solid tumors. We found liver toxicity in 77% of the patients treated for ALL, mainly during the maintenance therapy (65%) with oral 6-mercaptopurine and methotrexate. The most common clinical signs were anorexia, nausea, vomiting, abdominal pain and faltering weight gain. Cholestasis developed in two patients, while hepatocytolysis was the most common observed event (n = 24). Liver fibrosis, hypersplenism, portal hypertension and esophageal varices were found in two patients. One patient required endoscopic ligation of esophageal varices. Elevation of serum bilirubin appeared in two patients, while hypoproteinemia was observed in nine patients. None of the patients developed acute liver failure. We treated liver toxicity with hydration, alkalinization, i.v. Aspatofort, Aminosteril-N Hepa 8%, oral acetylcysteine, silymarin, ursodeoxycholic acid, Liv-52, Sargenor, and Essentiale forte. We found hepatotoxicity in 77% of the ALL patients undergoing chemotherapy, similar results have been published by other authors. Hepatotoxicity may develop through direct hepatic effects of cytostatics, or a preexisting liver disease impairs the metabolism and excretion of the drug, increasing its toxic effects. In our patients hepatotoxicity can be explained mainly by direct liver-injury, previous infections with hepatotropic viruses, such as cytomegalovirus, were detected only in three patients. Liver injury appeared in 77% of our ALL patients; 65% occurred during maintenance therapy with oral 6-mercaptopurine and methotrexate. Close followup of liver function during chemotherapy is mandatory for optimal results.


2016 ◽  
Vol 147 (1) ◽  
pp. 10-18.e8 ◽  
Author(s):  
Cristhiani Giane da Silva ◽  
Camila Pachêco-Pereira ◽  
André Luís Porporatti ◽  
Maria Gorete Savi ◽  
Marco A. Peres ◽  
...  

2019 ◽  
pp. 1-22
Author(s):  
Janet R. Gilsdorf

Before the advent of antibiotics, meningitis was a dreadful infection by any standard; many of its victims were young children, and almost all died, succumbing to the disease from days to six weeks, or sometimes longer, after the onset of their illness. Incredibly, patients occasionally survived but were often left with varying degrees of neurologic damage. Before the first spinal tap, the diagnosis of meningitis was based on clinical signs and symptoms and could be confirmed only by pathologic examination of the brains of deceased patients during an autopsy. Since the advent of the spinal tap, the diagnosis of meningitis rests on examination of the cerebrospinal fluid. Treatment of meningitis depends on which bacteria cause the infection. Helen Keller may be a famous surviving victim of this disease.


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