scholarly journals Clinical case of preiser disease, bilateral femoral head and humeral head osteonecrosis associated with chronic corticosteroid therapy

2021 ◽  
Vol 2 (4) ◽  
Author(s):  
Cunha R ◽  
◽  
Verraest X ◽  
Martins P ◽  
Madureira R ◽  
...  
Keyword(s):  
Femoral Head ◽  
Corticosteroid Therapy ◽  
Lymphoblastic Leukemia ◽  
Weight Bearing ◽  
Clinical Manifestations ◽  
Scaphoid Bone ◽  
Unknown Etiology ◽  
Early Stage Disease ◽  
Asymptomatic Patients ◽  
Chronic Corticosteroid

Osteonecrosis is a pathologic process that is associated with numerous conditions and therapeutic interventions. Most commonly the hip is involved but almost any bone can develop osteonecrosis. It can occur in the femoral head, but also affect the femoral condyles, humeral heads, proximal tibia, vertebrae, and small bones of the hand and foot. The most frequent etiological factors are trauma, alcoholism, and chronic corticosteroid therapy; the latter causing the most devastating form of osteonecrosis. Glucocorticoid-induced osteonecrosis is a known toxicity in pediatric and young adult patients treated for Acute Lymphoblastic Leukemia (ALL) and Non-Hodgkin Lymphoma (NHL). Osteonecrosis of the shoulders and weight-bearing joints of the lower extremities has emerged as a major adverse effect of modern ALL therapy. Pain in the absence of activity (ie, rest pain) occurs in approximately two-thirds of patients, and nocturnal pain occurs in one-third. MRI continues to be the gold standard for diagnosis in symptomatic and asymptomatic patients, especially in early-stage disease. Respectively to Preiser disease, this condition is a rare disease of unknown etiology characterized by Avascular Necrosis (AVN), also known as osteonecrosis, of the scaphoid bone and the clinical manifestations include insidious and progressive pain lasting from months to years, edema, loss of strength and reduced range of motion in the wrist. The treatment of non-traumatic osteonecrosis remains one of the most controversial subjects in the orthopedic literature. The purpose of this case report is to bring to attention osteonecrosis as a potential side effect of corticosteroid use in the pediatric population, as a delayed or missed diagnosis can lead to several joints destruction.

COVID 19: REALS AND CHALLENGES AGAINST DOCTORS

2020 ◽  
Vol 2 (1) ◽  
pp. 65-87
Author(s):  
Zen Ahmad
Keyword(s):  
Virus Disease ◽  
Clinical Manifestations ◽  
World Health ◽  
Unknown Etiology ◽  
Wuhan City ◽  
Traditional Markets ◽  
Pneumonia Case ◽  
The World ◽  
New Type ◽  
Health Organization

Corona Virus Disease (Covid-19) is a contagious disease caused by Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) which was discovered in December 2019 in China. This disease can cause clinical manifestations in the airway, lung and systemic. The World Health Organization (WHO) representative of China reported a pneumonia case with unknown etiology in Wuhan City, Hubei Province, China on December 31, 2019. The cause was identified as a new type of coronavirus on January 7, 2020 with an estimated source of the virus from traditional markets (seafood market). ) Wuhan city

KEJANG SEBAGAI MANIFESTASI NEUROLOGI DARI LEUKOSTASIS

2020 ◽  
Vol 3 (3) ◽  
pp. 127-131
Author(s):  
Eric Hartono Tedyanto ◽  
Ni Komang Sri Dewi Untari
Keyword(s):  
Lymphoblastic Leukemia ◽  
Clinical Manifestations ◽  
Emergency Situation ◽  
Early Mortality ◽  
Brain Cells ◽  
Trigger Factors ◽  
Medicine Department ◽  
Adult Age ◽  
Patient Morbidity ◽  
Laboratory Results

ABSTRAK Latar Belakang: Secara klinis, leukostasis didiagnosa pada pasien leukemia dengan hasil laboratorium hiperleukositosis (>100.000 u/L) disertai manifestasi respiratorik, neurologis, atau renal. Insidensi hiperleukositosis pada Leukemia Limfoblastik Akut (LLA) usia dewasa 10-30%, jarang pada wanita, dan jarang menyebabkan leukostasis. Laporan Kasus: Seorang wanita berusia 20 tahun dikonsulkan dari bagian penyakit dalam dengan kejang umum tonik-klonik. Hasil laboratorium menunjukkan leukosit 134.500 u/L, hasil EKG menunjukkan iskemik miokardium. Diskusi: Leukostasis jarang terjadi pada pasien leukemia. Leukostasis menyebabkan aliran oksigen dalam darah menuju sel menjadi inadekuat, termasuk salah satunya aliran darah yang menuju sel otak. Hipoksia jaringan otak merupakan salah satu faktor pemicu terjadinya kejang. Kesimpulan: Leukostasis merupakan suatu keadaan emergensi yang dapat meningkatkan morbiditas dan mortalitas pasien. Tujuan tatalaksana penyakit adalah mengurangi mortalitas dini, termasuk tatalaksana kejang, yang merupakan salah satu menifestasi klinis leukostasis. Kata kunci: kejang, leukostasis, leukemia.   ABSTRACT Background: Clinically, leukostasis is diagnosed in patients with leukemia with laboratory results of hyperleukocytosis (> 100,000 u / L) followed by respiratory, neurological, or renal manifestations. The incidence of hyperleukocytosis in Acute Lymphoblastic Leukemia (LLA) is 10-30% of adult age, rare in women, and rarely causes leukostasis. Case Report: A 20th-years-old woman was consulted from an Internal Medicine Department with a tonic-clonic general seizure. Laboratory results showed that leukocytes were 134,500 U / L and ECG results showed an ischemic myocardium. Discussion: Leukostasis rarely occurs in leukemic patients. Leukostasis causes the flow of oxygen in the blood to the cells to be inadequate, including the blood flow to brain cells. Brain tissue hypoxia is one of the trigger factors for seizures. Conclussion: Leukostasis is an emergency situation that can increase patient morbidity and mortality. The aim of disease management is to reduce early mortality, including management of seizures, which is one of the clinical manifestations of leukostasis. Keywords: seizure, leukostasis, leukemia.

Lymphomatoid Granulomatosis in a Child with Acute Lymphatic Leukemia in Remission

1978 ◽  
Vol 87 (5_suppl) ◽  
pp. 5-10 ◽  
Author(s):  
Seymour R. Cohen ◽  
Stuart Siegel ◽  
Eva Heuser ◽  
Benjamin H. Landing ◽  
Susan Shen ◽  
...  
Keyword(s):  
Survival Rate ◽  
Poor Prognosis ◽  
Lymphoblastic Leukemia ◽  
Lymphomatoid Granulomatosis ◽  
Unknown Etiology ◽  
Acute Lymphatic Leukemia ◽  
Clinical Onset ◽  
Lymphatic Leukemia ◽  
Trachea And Bronchi ◽  
Other Neoplasms

Lymphomatoid granulomatosis, a tumor-like process of unknown etiology, produced progressively destructive disease of the larynx, trachea and bronchi in an eight-year-old girl with acute lymphoblastic leukemia of five years duration. The leukemia had been in remission for 4½ years at the clinical onset of the lymphomatoid granulomatosis. Whether this occurrence suggests that lymphomatoid granulomatosis is a type of neoplasm, or is associated with immunologic depression, cannot be stated. Fortunately rare, and of poor prognosis, the disorder may become more frequent with improved survival rate of patients with leukemia and other neoplasms.

scholarly journals 2021 Update on the Clinical Management and Diagnosis of Kawasaki Disease

2021 ◽  
Vol 23 (3) ◽  
Author(s):  
Frank Zhu ◽  
Jocelyn Y. Ang
Keyword(s):  
High Risk ◽  
Kawasaki Disease ◽  
Corticosteroid Therapy ◽  
Clinical Management ◽  
Treatment Guidelines ◽  
Day Treatment ◽  
Significant Proportion ◽  
Clinical Manifestations ◽  
Heart Association ◽  
Ivig Resistance

Abstract Purpose of Review Provide an updated review of the clinical management and diagnosis of Kawasaki disease with inclusion of potential diagnostic difficulties with multisystem inflammatory syndrome in children (MIS-C) given the ongoing COVID-19 pandemic. Recent Findings Adjunctive corticosteroid therapy has been shown to reduce the rate of coronary artery dilation in children at high risk for IVIG resistance in multiple Japanese clinical studies (most notably RAISE study group). Additional adjunctive therapies (etanercept, infliximab, cyclosporin) may also provide limited benefit, but data is limited to single studies and subgroups of patients with cardiac abnormalities. The efficacy of other agents (atorvastatin, doxycycline) is currently being investigated. MIS-C is a clinically distinct entity from KD with broad clinical manifestations and multiorgan involvement (cardiac, GI, hematologic, dermatologic, respiratory, renal). MIS-C with Kawasaki manifestations is more commonly seen in children < 5 years of age. Summary The 2017 American Heart Association (AHA) treatment guidelines have included changes in aspirin dosing (including both 80–100 mg/kg/day and 30–50 mg/kg/day treatment options), consideration of the use of adjuvant corticosteroid therapy in patients at high risk of IVIG resistance, and the change in steroid regimen for refractory KD to include both pulse-dose IVMP and longer course of prednisolone with an oral taper. A significant proportion of children diagnosed with MIS-C, a post-infectious syndrome of SARS-CoV-2 infection, meet criteria for Kawasaki disease. Further investigation is warranted to further delineate these conditions and optimize treatment of these conditions given the ongoing COVID-19 pandemic.

scholarly journals Animal Models of Autosomal Recessive Parkinsonism

Biomedicines ◽  
2021 ◽  
Vol 9 (7) ◽  
pp. 812
Author(s):  
Guendalina Bastioli ◽  
Maria Regoni ◽  
Federico Cazzaniga ◽  
Chiara Maria Giulia De Luca ◽  
Edoardo Bistaffa ◽  
...  
Keyword(s):  
Animal Models ◽  
Sleep Disturbances ◽  
Autosomal Recessive ◽  
Clinical Manifestations ◽  
Cognitive Disorders ◽  
Dopamine Neurons ◽  
Substantia Nigra Pars Compacta ◽  
Current Status ◽  
Unknown Etiology ◽  
Neuron Death

Parkinson’s disease (PD) is the most common neurodegenerative movement disorder. The neuropathological hallmark of the disease is the loss of dopamine neurons of the substantia nigra pars compacta. The clinical manifestations of PD are bradykinesia, rigidity, resting tremors and postural instability. PD patients often display non-motor symptoms such as depression, anxiety, weakness, sleep disturbances and cognitive disorders. Although, in 90% of cases, PD has a sporadic onset of unknown etiology, highly penetrant rare genetic mutations in many genes have been linked with typical familial PD. Understanding the mechanisms behind the DA neuron death in these Mendelian forms may help to illuminate the pathogenesis of DA neuron degeneration in the more common forms of PD. A key step in the identification of the molecular pathways underlying DA neuron death, and in the development of therapeutic strategies, is the creation and characterization of animal models that faithfully recapitulate the human disease. In this review, we outline the current status of PD modeling using mouse, rat and non-mammalian models, focusing on animal models for autosomal recessive PD.

Body Weight Alterations Under Early Corticosteroid Withdrawal and Chronic Corticosteroid Therapy with Modern Immunosuppression

2005 ◽  
Vol 80 (1) ◽  
pp. 26-33 ◽  
Author(s):  
Christin C. Rogers ◽  
Rita R. Alloway ◽  
Joseph F. Buell ◽  
Robyn Boardman ◽  
J Wesley Alexander ◽  
...  
Keyword(s):  
Body Weight ◽  
Corticosteroid Therapy ◽  
Corticosteroid Withdrawal ◽  
Chronic Corticosteroid

Osteonecrosis of the Femoral Head of Laboratory Animals: The Lessons Learned from a Comparative Study of Osteonecrosis in Man and Experimental Animals

2003 ◽  
Vol 40 (4) ◽  
pp. 345-354 ◽  
Author(s):  
J. H. Boss ◽  
I. Misselevich
Keyword(s):  
Blood Flow ◽  
Femoral Head ◽  
Weight Bearing ◽  
Arterial Occlusion ◽  
Lessons Learned ◽  
Laboratory Animals ◽  
Treatment Modalities ◽  
Venous Stasis ◽  
Spontaneously Hypertensive ◽  
Bearing Loads

Animal models of osteonecrosis of the femoral head are indispensable to the understanding of successful treatment modalities for avascular necrosis of the femoral head in adults and in children with Legg-Calvé-Perthes disease. Many of these models adequately reflect the current “vascular deprivation” theory regarding the etiology of the disease. In addition to spontaneous occurrence, surgical- and corticosteroid-induced models are suitable, common experimental ones. Osteonecrosis of spontaneously hypertensive rats appears to be due to defective bone formation and compression of the arteries entering the femoral head at its lateral facets by daily weight-bearing loads. Successful modeling of surgical-induced femoral capital necrosis can be a challenge in animals with a dual epiphyseal blood supply. High doses of corticosteroids are a pivotal risk factor in the development of osteonecrosis. The pathogenesis of corticosteroid-induced osteonecrosis likely resides in reduced blood flow. Steroids may reduce blood flow by numerous mechanisms, including marrow adipocytic hypertrophy leading to sinusoidal compression, venous stasis and, eventually, obstruction of the arteries, and arterial occlusion by fat emboli and lipid-loaded fibrin-platelet thrombi. Other, less common varieties of osteonecrosis include those secondary to endotoxin-induced disseminated intravascular coagulation, immune reactions, immoderately low or high temperatures, and high-impact-related injuries. Common to these diverse forms of osteonecrosis are fibrin thrombi clogging arterioles and small arteries.

scholarly journals Adjuvant corticosteroid therapy in hepatosplenic candidiasis-related IRIS

2012 ◽  
Vol 4 (1) ◽  
pp. e2012018 ◽  
Author(s):  
Cengiz Bayram ◽  
Ali Fettah ◽  
Nese Yarali ◽  
Abdurrahman Kara ◽  
Fatih Mehmet Azik ◽  
...  
Keyword(s):  
Corticosteroid Therapy ◽  
Immune Reconstitution ◽  
Inflammatory Responses ◽  
Clinical Symptoms ◽  
Lymphoblastic Leukemia ◽  
Laboratory Findings ◽  
Hepatosplenic Candidiasis ◽  
Cell Acute Lymphoblastic Leukemia ◽  
Inflammatory Syndrome ◽  
Standard Risk

Hepatosplenic candidiasis (HSC) is a form of invasive fungal infection that occurs most commonly in patients with acute leukemia treated with chemotherapy and requires protracted antifungal therapy. Immune reconstitution inflammatory syndrome (IRIS) is best characterized as a dysregulated inflammatory responses triggered by rapid resolution of immunosuppression.We present a child diagnosed with standard-risk precursor B cell-acute lymphoblastic leukemia who developed HSC and Candida-related IRIS during recovery of neutropenia associated with induction chemotherapy. Addition of corticosteroid therapy to antifungal treatment is associated with the resolution of the clinical symptoms and laboratory findings

Urine Cultures among Hospitalized Veterans: Casting Too Broad a Net?

2014 ◽  
Vol 35 (5) ◽  
pp. 574-576 ◽  
Author(s):  
Dimitri M. Drekonja ◽  
Christina Gnadt ◽  
Michael A. Kuskowski ◽  
James R. Johnson
Keyword(s):  
Asymptomatic Bacteriuria ◽  
Clinical Manifestations ◽  
Antimicrobial Treatment ◽  
Antimicrobial Use ◽  
High Count ◽  
Asymptomatic Patients

Since detection of asymptomatic bacteriuria among inpatients often leads to inappropriate antimicrobial treatment, we studied why urine cultures were ordered and correlates of treatment. Most cultures were obtained from patients without urinary complaints and a minority from asymptomatic patients. High-count bacteriuria, not clinical manifestations, appeared to trigger most antimicrobial use.

Legg-Calvé-Perthes Disease

1986 ◽  
Vol 7 (10) ◽  
pp. 299-304
Author(s):  
William P. Bunnell
Keyword(s):  
Birth Weight ◽  
Femoral Head ◽  
Vascular Supply ◽  
Skeletal Age ◽  
Perthes Disease ◽  
Unknown Etiology ◽  
Necrotic Bone

Perthes disease is a condition of unknown etiology in which the vascular supply of the developing femoral head is temporarily interrupted, causing necrosis and collapse of the bony femoral head, followed by spontaneous revascularization and healing of the necrotic bone. It was independently described by four different authors (Legg, Calvé, Perthes, and Waldenstrom) in 1909 and 1910. They identified the condition as an affliction of the hip distinct from trauma and infection. The term "coxa plana" was applied to the characteristic flattening of the femoral head seen in Perthes disease. It was not until 10 years later that the actual pathology of avascular necrotic bone was described. EPIDEMIOLOGY The condition has its onset in children ranging in age from 2 to 12 years, with the majority of patients presenting between the ages of 4 and 8 years. Boys are affected four times more frequently than girls, and the condition is bilateral in approximately 15% of affected children. Certain constitutional factors are frequently found in children with Perthes disease. Skeletal age is delayed in nearly 90% of children affected with it. Affected boys have been found to be an average of 1 in shorter and affected girls 3 in shorter in height than their unaffected peers. Birth weight of affected children is frequently lower than average.

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