scholarly journals Rapidly progressive antineutrophil cytoplasm antibodies associated with pulmonary-renal syndrome in a 10-year-old girl

2001 ◽  
Vol 119 (1) ◽  
pp. 29-32 ◽  
Author(s):  
Fermin Blanco Filho ◽  
Luci Carla Ernesto ◽  
Mônica Assis Rosa ◽  
Luis Antônio Stuginski ◽  
Eliana Regina Zlochevsky ◽  
...  

CONTEXT: The term pulmonary-renal syndrome has been used frequently to describe the clinical manifestations of a great number of diseases in which pulmonary hemorrhage and glomerulonephritis coexist. The classic example of this type of vasculitis is Goodpasture´s syndrome, a term used to describe the association of pulmonary hemorrhage, glomerulonephritis and the presence of circulating antiglomerular basement membrane antibodies (anti-GBM). Among the several types of systemic vasculitides that can present clinical manifestations of the pulmonary-renal syndrome, we focus the discussion on two types more frequently associated with antineutrophil cytoplasm antibodies (ANCA), microscopic polyangiitis and Wegener´s granulomatosis, concerning a 10 year old girl with clinical signs and symptoms of pulmonary-renal syndrome, with positive ANCA and rapidly progressive evolution. CASE REPORT: We describe the case of a 10-year-old girl referred to our hospital for evaluation of profound anemia detected in a primary health center. Five days before entry she had experienced malaise, pallor and began to cough up blood-tinged sputum that was at first attributed to dental bleeding. She was admitted to the infirmary with hemoglobin = 4 mg/dL, hematocrit = 14%, platelets = 260,000, white blood cells = 8300, 74% segmented, 4% eosinophils, 19% lymphocytes and 3% monocytes. Radiographs of the chest revealed bilateral diffuse interstitial alveolar infiltrates. There was progressive worsening of cough and respiratory distress during the admission day, when she began to cough up large quantities of blood and hematuria was noted. There was rapid and progressive loss of renal function and massive lung hemorrhage. The antineutrophil cytoplasm antibody (ANCA) test with antigen specificity for myeloperoxidase (anti-MPO) was positive and the circulating anti-GBM showed an indeterminate result.

2018 ◽  
Vol 7 (3) ◽  
pp. 38-41 ◽  
Author(s):  
Aliya Hasan ◽  
Dapo Akintola ◽  
Aliya Hasan ◽  
Dapo Akintola

Gorlin-Goltz syndrome encompasses a variety of clinical signs and symptoms including important oral manifestations which general dental practitioners should be aware of. In light of the risk of malignancy it is important to be aware of this syndrome and recognise the need for early referral for multidisciplinary management. This paper aims to discuss Gorlin-Goltz syndrome, the pathophysiology of the condition and address the wide range of clinical manifestations. The author will pay particular attention to the oral manifestations of the condition and the management of such anomalies.


2020 ◽  
Vol 21 (15) ◽  
pp. 5338 ◽  
Author(s):  
Mauro Giorgi ◽  
Silvia Cardarelli ◽  
Federica Ragusa ◽  
Michele Saliola ◽  
Stefano Biagioni ◽  
...  

In March 2020, the World Health Organization declared the severe acute respiratory syndrome corona virus 2 (SARS-CoV2) infection to be a pandemic disease. SARS-CoV2 was first identified in China and, despite the restrictive measures adopted, the epidemic has spread globally, becoming a pandemic in a very short time. Though there is growing knowledge of the SARS-CoV2 infection and its clinical manifestations, an effective cure to limit its acute symptoms and its severe complications has not yet been found. Given the worldwide health and economic emergency issues accompanying this pandemic, there is an absolute urgency to identify effective treatments and reduce the post infection outcomes. In this context, phosphodiesterases (PDEs), evolutionarily conserved cyclic nucleotide (cAMP/cGMP) hydrolyzing enzymes, could emerge as new potential targets. Given their extended distribution and modulating role in nearly all organs and cellular environments, a large number of drugs (PDE inhibitors) have been developed to control the specific functions of each PDE family. These PDE inhibitors have already been used in the treatment of pathologies that show clinical signs and symptoms completely or partially overlapping with post-COVID-19 conditions (e.g., thrombosis, inflammation, fibrosis), while new PDE-selective or pan-selective inhibitors are currently under study. This review discusses the state of the art of the different pathologies currently treated with phosphodiesterase inhibitors, highlighting the numerous similarities with the disorders linked to SARS-CoV2 infection, to support the hypothesis that PDE inhibitors, alone or in combination with other drugs, could be beneficial for the treatment of COVID-19.


2020 ◽  
Author(s):  
Wenjun Du ◽  
Jinhong Yu ◽  
Hui Wang ◽  
Xiaoguo Zhang ◽  
Shouwei Zhang ◽  
...  

Abstract Aims & Background: The COVID-19 outbreak spread in China and is a threat to the world. We reported on the epidemiological, clinical, laboratory, and radiological characteristics of children cases to help health workers better understand and provide timely diagnosis and treatment.Methods: Retrospectively, two research centers’ case series of 67 consecutive hospitalized cases including 14 children cases with COVID-19 between 23 Jan 2020 to 15 Feb 2020 from Jinan and Rizhao were enrolled in this study. Epidemiological, clinical, laboratory, and radiological characteristics of children and adults were analyzed and compared.Results: Most cases in children were mild(21.4%) and conventional cases(78.6%), with mild clinical signs and symptoms, and all cases were of family clusters. Fever (35.7%) and dry cough(21.4%) were described as clinical manifestations in children cases. Dry cough and phlegm were not the most common symptoms in children compared with adults(p=0.03). In the early stages of the disease, lymphocyte counts did not significantly decline but neutrophils counts did in children compared with adults(p=0.00).There was an elevated level of LDH(p=0.01) and a lower level of CRP(p=0.00)and IL-6(p=0.01) in children compared with adults. There were 8 (57.1%)asymptomatic cases and 6 (42.9%)symptomatic cases among the 14 children cases. The age of asymptomatic patients was younger than that of symptomatic patients(p=0.03). Even among asymptomatic patients, 5(62.5%)cases had pneumonia including 3 (60%) cases with bilateral pneumonia, which was not different compared with that of asymptomatic cases(p=0.58, p=0.74).Conclusions: The clinical symptoms of children are mild, and the positive indicators of laboratory tests are rare, which may easily cause clinical misdiagnoses.


2005 ◽  
Vol 133 (7-8) ◽  
pp. 348-352
Author(s):  
Marija Mladenovic ◽  
Nedeljko Radlovic ◽  
Zoran Lekovic ◽  
Dragana Ristic ◽  
Dragana Zivanovic ◽  
...  

Introduction. The disorder of cow milk protein intolerance is characterized by a wide spectrum of clinical manifestations caused by hypersensitivity of type I, II, or IV, and occurs in 2-3% of children, mostly infants. Objective. The aim of this study was to present our experiences and observations of clinical signs and symptoms of cow milk protein intolerance in infants aged below 12 months. Method. The investigation was carried out on a sample of 55 infants, aged between 1.5-9 months (x=4.2l?1.25), who had cow milk protein intolerance. Diagnosis of illness was based on characteristic anamnestic, clinical, and laboratory parameters, as well as on an adequate patient's response to antigen elimination. Results. The clinical presentation of cow milk protein intolerance was dominated by cutaneous problems, found in 40/55 (72.73%) infants, followed by digestive disorders, found in 31 (56.36%), while respiratory tract disorders were observed least frequently (14.55%). None of the patients developed anaphylactic shock. Changes involving only one system were found in 35 (63.64%) patients; of these 20 (36.36%) were cutaneous and 15 (27.27%) digestive. Twenty (36.36%) infants displayed multisystemic changes; of these 12 were cutaneus with digestive, 4 were cutaneus with respiratory, while 4 infants had cutaneous, digestive, and respiratory disorders. Of the 55 infants with cow milk protein intolerance, 26 (47.27%) had urticaria, 22 (40.00%) perioral erythema, 21 (38.18%) diarrhoea (15 haemorrhagic, 6 non-haemorrhagic), 13 (23.64%) vomiting, 12 (21.82%) Quincke's oedema, 12 (21.82%) eczema, 5 (9.09%) obstructive bronchitis, while 3 (5.45%) infants had laryngitis. In 5 (9.09%) patients we found a significant body weight deficit and in 3 (5.45%), sideropenic anaemia, while longitudinal growth retardation was not registered in any of the patients. Conclusion. Our study showed that the disorder of cow milk protein intolerance predominantly involved cutaneous disorders as well as combinations of cutaneous and digestive disorders, while respiratory system disorders proved to be relatively rare.


2014 ◽  
Vol 60 (6) ◽  
pp. 282-284
Author(s):  
S.H. Morariu ◽  
M.A. Badea ◽  
M.D. Vartolomei ◽  
Iudita Maria Badea ◽  
O.S. Cotoi

Abstract Psoriasis is a common dermatosis, however the rupoid type is considered as an exceptional form of this disease. Rupoid scabs are very rare in dermatological daily practice, usually being seen as secondary to syphilis in immunosuppressed patients. Rupoid psoriasis is characterized by thick and multilayered crusts that are resistant to local therapy and present a sudden onset. Severe arthropathy is a common manifestation. We did not found in literature any association of rupoid psoriasis with intermittent fever. We present the case of a patient who exhibited a rush of rupoid boards with severe arthralgia accompanied by intermittent fever. The suspicion of malignant syphilis was raised considering the clinical signs and symptoms and the specific social context of STDs. This suspicion was unconfirmed by TPHA negative reaction and histopathological appearance that showed changes typical of psoriasis. Clinical manifestations were successfully controlled with methotrexate.


Author(s):  
Vaishnavi Tammishetty ◽  
Sravika Nagamalla ◽  
Pravalika Sakilam ◽  
Hyma Ponnaganti

As there is an outbreak of novel corona virus in 2019 it has spread globally that resulted in severe acute respiratory syndrome coronavirus 2 (SARS-COV-2) pandemic and mostly there is limited data provided on children. The main aim of this review is to provide a knowledge on introduction, epidemiology, pathogenesis, transmission, clinical manifestations, laboratory findings, treatment of COVID-19 in children. And it also includes latest statistical data of children prone to COVID-19. Besides respiratory and GI symptoms atypical features such as chilblains and multi-inflammatory system are also reported. pathophysiology gives information regarding the life cycle of virus in hostcell and epidemiology explains the different types of viruses affecting the respiratory system. The clinical signs and symptoms are almost similar to the adults but they are in mild, and most of the children affected with Covid-19 are asymptomatic. This review study makes a medical practitioner to have a quick, practical approach to the disease to use in different scopes, especially in pediatric medicine.


2021 ◽  
Vol In Press (In Press) ◽  
Author(s):  
Alireza Ghodsi ◽  
Elnaz Mahmoudabadi ◽  
Sara Ghahremani ◽  
Abdolreza Malek

Context: Multisystem inflammatory syndrome in children (MIS-C) is an emerging condition after the spread of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic, termed COVID-19. This study aimed to describe the cardiac manifestations of children diagnosed with MIS-C. Evidence Acquisition: This narrative review was conducted by searching the PubMed, Scopus, and Google Scholar databases to review MIS-C cardiac manifestations up to September 30, 2020. The demographic features, past medical history, clinical signs and symptoms, cardiac involvement, and the type of COVID-19 diagnosis confirmation were extracted. Results: In many children, MIS-C seems to be a post-infectious complication of the COVID-19 infection. This syndrome affects multiple organs and has various clinical manifestations mimicking Kawasaki disease. Patients frequently present with persistent fever, kidney injury, gastrointestinal (GI) problems, neurologic symptoms, mucosal changes, conjunctivitis, and cardiac involvement. Children with MIS are more likely to present with hypotension, shock, and cardiac dysfunction, rather than coronary artery abnormalities and arrhythmia. Children with MIS need close observation; some need to be hospitalized, and a few may need a Pediatric Intensive Care Unit (PICU) admission. Treatment currently includes anticoagulants, IV immunoglobulin, and anti-inflammatory drugs. Conclusions: As a novel syndrome associated with SARS-CoV-2 infection, MIS-C is potentially lethal. Cardiac manifestations, including coronary and myocardial involvement, are common and should be carefully identified. With prompt diagnosis and proper treatment, most children will survive, but the outcomes of the disease are unknown, so long-term follow-ups are required.


2011 ◽  
Vol 64 (3-4) ◽  
pp. 188-193 ◽  
Author(s):  
Vera Milenkovic ◽  
Biljana Lazovic

Gestational trophoblastic disease is characterized by abnormal proliferation of pregnancy-associated trophoblastic tissue with malignant potential. Gestational trophoblastic disease covers a spectrum of conditions including hydatidiform mole, invasive mole, choriocarcinoma and placental site trophoblastic tumour. It is very important to understand the pathophysiology and natural history of the disease in order to achieve faster recognition and effective treatment. The presence and course of the disease can be monitored with quantitative levels of human chorionic gonadotrophin in all cases. Clinical signs and symptoms are usually insufficient to diagnose and predict the extent of disease. Nowadays, gestational trophoblastic diseases are the best treated gynaecological malignancy thanks to modern technology. This review covers various aspects of gestational trophoblastic disease: its development, epidemiology, aetiology and pathogenesis, as well as its classification, clinical manifestations and diagnostic methods.


Primer on Multiple Sclerosis, second edition is designed to be a practical guide to the basic science and clinical manifestations of multiple sclerosis. It is intended primarily for neurologists and other health care professionals who treat persons with this disease. The book starts with a review of the history of multiple sclerosis and the basic genetics, immunology, electrophysiology, and pathophysiology that are central to the disease. It then reviews the common and uncommon clinical signs and symptoms of multiple sclerosis and the management of these conditions. The latest diagnostic strategies are presented. There is extensive coverage of approved and experimental disease-modifying therapies, including algorithms to assist clincians in choosing these therapies. Complementary and alternative therapies that are popular among persons with multiple sclerosis are examined. New additions to this edition include a chapter for nursing health care professionals, and updates on therapeutics. Unique to this book are the chapters on the legal, psychosocial, and vocational issues that often present challenges for person with multiple sclerosis, topics that typically are not covered in standard texts.


PEDIATRICS ◽  
1979 ◽  
Vol 63 (1) ◽  
pp. 88-93
Author(s):  
Donald T. Whelan ◽  
Robert Hill ◽  
Eamonn D. Ryan ◽  
Marilyn Spate

A 5-month-old infant had an unusual combination of clinical signs and symptoms. These consisted of irritability, dystonia, lack of head control, grimacing, opisthotonos, choreoathetoid movements, delayed development, and severe metabolic acidosis. Metabolic investigation by gas-liquid chromatography/mass spectrometry detected urinary organic acids. This confirmed the diagnosis of l-glutaric aciduria. The concentration of l-glutaric acid in the patient's plasma was 2.5 mg/dl (normal range, 0 to 0.1 mg/dl), and in the patient's urine was 4.6 mg/mg of creatinine (normal range, 0 to 0.05 mg/mg of creatinine),) but the concentration was not elevated in the plasma and urine of the infant's parents nor of two other family members. No glutaryl-CoA dehvdrogenase activity was found in leukocytes taken from the patient. Three of the four family members, including the parents, demonstrated 38%, 42%, and 42% activity, respectively, compared with the activity of normal controls. These findings are consistent with an autosomal recessive disorder involving the Metabolism of ghitaryl-CoA to crotonyl-CoA. Dietary restriction was instituted on two separate occasions. First, a low protein diet of 1.6 gm/kg of body weight per day was given, then a low lysine intake of 50 mg/kg/day. These dietary manipulations caused a decrease in the plasma and urine concentrations of L-glutaric acid and β-hydroxyglutaric acid. However, no effect on the clinical manifestations of the disease was noted.


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