scholarly journals Pfeifer-Weber-Christian Disease and Benign Multiple Subcutaneous Noninfiltrative Angiolipomas: A Puzzling Case and Review of a Rare Entitety

2020 ◽  
Author(s):  
Goran Radunovic ◽  
Slavica Pavlov-Dolijanovic ◽  
Tanja Milicic ◽  
Martina Bosic ◽  
Nada Vujasinovic Stupar ◽  
...  
Keyword(s):  
Corticosteroid Therapy ◽  
De Novo ◽  
Specific Treatment ◽  
Case Presentation ◽  
Diagnostic Difficulties ◽  
High Degree ◽  
Fibrin Thrombi ◽  
Autoinflammatory Condition ◽  
Systemic Corticosteroid Therapy

Abstract Background: Pfeifer-Weber-Christian disease (PWCD) is one of many rare diseases that may be easily missed if there is not a high degree of suspicion. Angiolipomas are rare, benign subcutaneous tumors, composed of adipose tissue and blood vessels and often containing fibrin thrombi. The majority of angiolipomas occur sporadically; however, there is a minority of cases that have been associated with long-term corticosteroid use. Case presentation: We report here an unusual case of PWCD associated with benign multiple subcutaneous noninfiltrative angiolipomas confirmed by skin biopsy. Systemic corticosteroid therapy was not effective at reducing flares of panniculitis, and during this therapy angiolipomas gradually increased in size. In contrast, administration of oral Cyclosporine A (CyA) led to a rapid remission of the PWCD. Conclusions: As PWCD has no known aetiology and no specific treatment has been established, the successful therapy with the CyA supports the hypothesis that PWCD is a T cell mediated autoinflammatory condition. Also, this case represent very rare side effects of corticosteroid therapy, such as induction of de novo angiolipomas or increased growth of existing tumours. This case bring diagnostic difficulties in everyday clinical practice, especially in patients with panniculitides, but histopathological evaluation usually resolves the dilemma.

Long-term follow-up of a child with Klinefelter syndrome and achondroplasia from infancy to 16 years

2017 ◽  
Vol 30 (7) ◽  
Author(s):  
Jessica D. Arditi ◽  
Loretta Thomaidis ◽  
Helen Frysira ◽  
Artemis Doulgeraki ◽  
George P. Chrousos ◽  
...  
Keyword(s):  
Sex Chromosome ◽  
De Novo ◽  
Klinefelter Syndrome ◽  
Pubertal Development ◽  
Pediatric Endocrinology ◽  
Case Presentation ◽  
Long Term Follow Up ◽  
Chromosome Disorder

AbstractBackground:Achondroplasia (ACH), an autosomal dominant skeletal dysplasia, occurs in approximately 1:20,000 births. On the other hand, 47,XXY aneuploidy (Klinefelter syndrome [KS]) is the most common sex chromosome disorder, with a prevalence of approximately 1:600 males. To the best of our knowledge, only five cases of patients presenting both ACH and KS have been reported to date in the international literature. However, none of these cases has been longitudinally followed during the entire childhood.Case presentation:We report a male patient with ACH and KS, diagnosed in early infancy because of his typical phenotype of ACH. The diagnosis was confirmed by molecular analysis revealing a de novo heterozygous 1138 G-to-A mutation of theConclusions:This is the first reported case with both conditions that was diagnosed in infancy and was longitudinally followed by a pediatric endocrinology team regularly, from infancy to late adolescence. With a typical phenotype of ACH, it is striking and noteworthy that he did not develop the classical endocrine complications of a child with KS, neither did he necessitate testosterone supplementation during his pubertal development, due to his normal virilization and testosterone levels.

scholarly journals A Long-Term Control of Intramedullary Thoracic Spinal Cord Metastasis from Small Cell Lung Cancer

2018 ◽  
Vol 61 (2) ◽  
pp. 57-59 ◽  
Author(s):  
Hajime Osawa ◽  
Shinichiro Okauchi ◽  
Gen Ohara ◽  
Katsunori Kagohashi ◽  
Hiroaki Satoh
Keyword(s):  
Lung Cancer ◽  
Spinal Cord ◽  
Small Cell Lung Cancer ◽  
Corticosteroid Therapy ◽  
Cell Lung Cancer ◽  
Systemic Corticosteroid ◽  
Small Cell ◽  
Small Cell Lung ◽  
Systemic Corticosteroid Therapy

Radiotherapy with systemic corticosteroid therapy has been used to treat intramedullary spinal cord metastasis (ISCM), but recovery of function and long-term survival of these patients has been rarely observed. We report herein a small cell lung cancer (SCLC) patient with recurrent thoracic ISCM, who was successfully treated with radiotherapy and systemic corticosteroid therapy. A 70-year-old man, who was diagnosed as having SCLC seven months previously, developed thoracic ISCM. Soon after the detection of the lesion, the patient received radiotherapy with systemic corticosteroid therapy. Sensory disturbance in both extremities and neurogenic bladder and bowel dysfunction was recovered. The patient could walk after irradiation again. The patient received additional chemotherapy and survived 20 months after the diagnosis of ISCM recurrence. Prompt diagnosis and appropriate treatment for ISCM and effective chemotherapy for recurrent SCLC might be the favorable factors for such patients. Further studies will be required to define a favorable subset of patients most likely to benefit from a conventional approach.

scholarly journals Tubulointerstitial nephritis and uveitis syndrome in an adolescent female: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Tadej Petek ◽  
Maja Frelih ◽  
Nataša Marčun Varda
Keyword(s):  
Corticosteroid Therapy ◽  
Systemic Corticosteroid ◽  
Tubulointerstitial Nephritis ◽  
Early Recognition ◽  
Renal Involvement ◽  
Mixed Cell ◽  
Diagnostic Work ◽  
Noncaseating Granulomas ◽  
High Degree ◽  
Systemic Corticosteroid Therapy

Abstract Background Tubulointerstitial nephritis with uveitis syndrome is a rare disease affecting mainly children and young women. Tubulointerstitial nephritis with uveitis is a diagnosis of exclusion, requiring a high degree of clinical suspicion. Studies report recent infections or certain drugs as precipitating factors of a lymphocytic oculorenal immune response. The prognosis is usually favorable with topical and systemic corticosteroid therapy. Case presentation We report a literature review and the case of a 14-year-old white girl, who presented to the ophthalmology department with features of one-sided uveitis. Upon transfer of patient to nephrological care, diagnostic work-up revealed renal involvement. Renal biopsy showed a mixed-cell and granulomatous tubulointerstitial nephritis with some noncaseating granulomas, leading to a diagnosis of tubulointerstitial nephritis with uveitis syndrome. With topical ocular and systemic corticosteroid therapy, the patients’ condition improved over several weeks. Conclusions Our case highlights the importance of early recognition and treatment of this syndrome, where cross-specialty care typically leads to a favorable outcome.

IgE LEVELS IN SERA OF CHILDREN WITH ASTHMA

PEDIATRICS ◽  
1971 ◽  
Vol 47 (5) ◽  
pp. 848-856
Author(s):  
Lata Kumar ◽  
Richard W. Newcomb ◽  
Kimishige Ishizaka ◽  
Elliott Middleton ◽  
Margaret M. Hornbrook
Keyword(s):  
Atopic Dermatitis ◽  
Bronchial Asthma ◽  
Corticosteroid Therapy ◽  
Systemic Corticosteroid ◽  
Skin Tests ◽  
Positive Skin ◽  
Children With Asthma ◽  
Severity Of The Disease ◽  
Systemic Corticosteroid Therapy

Concentrations of the immunoglobulin that is associated with reaginic activity, IgE (γE), were measured in sera of 116 children with bronchial asthma. High γE (>700 ng/ml) were found in only 30. High γE levels were found in children with many positive skin tests (P < 0.01) and with active atopic dermatitis (P < 0.01). Long term, systemic corticosteroid therapy was found to be associated with normal γE values (P < .001) even when the characteristics of the disease would otherwise have indicated high values. As no correlation is found to be evident between severity of the disease and γE levels, it may be suggested that corticosteroids may decrease intravascular γE. The serum γE values failed to reveal two or more clinically different groups among our patients.

scholarly journals Acute Kidney Injury in Children

2021 ◽  
Vol 1 (2) ◽  
Author(s):  
Nilzete Liberato Bresolin
Keyword(s):  
Acute Kidney Injury ◽  
Specific Treatment ◽  
Kidney Injury ◽  
Critically Ill Children ◽  
Patients At Risk ◽  
Underlying Causes ◽  
Diagnosis And Classification ◽  
New Biomarkers ◽  
High Degree

Recent literature data has shown that acute kidney injury (AKI) is a common occurrence in critically ill children and that it is associated with high rates of morbidity and mortality, even after adjusting for other risk factors. In addition, it can result in long-term sequelae translated by the development of arterial hypertension, microalbuminuria and chronic renal dysfunction. High degree of suspicion, early diagnosis based on the recognition of patients at risk and the use of new criteria for the diagnosis and classification of AKI and, whenever possible, using the new biomarkers can positively alter the prognosis of these patients. Although there is still no specific treatment for AKI, it is recommended to focus on the immediate institution of preventive measures in order to maintain renal hemodynamics, early treatment of septic patients, elimination of exogenous nephrotoxins, recognition and management of patients with AKI secondary to high levels of endotoxins, control and normalization of volume after the initial phase of fluid resuscitation, sufficient nutritional support, and recognition and treatment of the underlying causes are necessary. Patients that fail with conservative care will need renal replacement therapy.

scholarly journals Assessment of glycemic status of COPD patients on long term corticosteroid therapy

2017 ◽  
Vol 5 (9) ◽  
pp. 3997
Author(s):  
Vivek Kumar Verma ◽  
Ranjit Kumar Nim ◽  
Manoj Kumar ◽  
Premshanker Singh ◽  
Geeta Singh ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Corticosteroid Therapy ◽  
Systemic Corticosteroid ◽  
Inhaled Corticosteroids ◽  
Beta Agonist ◽  
Airflow Limitation ◽  
Glycemic Status ◽  
Copd Patients ◽  
Systemic Corticosteroid Therapy

Background: Chronic obstructive pulmonary disease (COPD) is a preventable and treatable disease characterized by persistent airflow limitation that is usually progressive and associated with an enhanced chronic inflammatory response in the airways and the lung to noxious particles or gases. Among the pharmacological therapy, inhaled beta- agonist and anticholinergics are the mainstay therapy of COPD along with corticosteroid. Steroid therapy is associated with various potential adverse effects like steroid induced deranged glycemic status. So, we sought to examine the association between long term corticosteroid therapy and glycemic status in COPD patients.Methods: A cross sectional study was done to assess the glycemic status in COPD patients on long term corticosteroid therapy in a rural tertiary care centre on patients satisfying inclusion and exclusion criteria. Inclusion criteria includes COPD patients on steroid based therapy (inhaled/systemic or both) for at least 6 months. Known case of type 2 diabetes mellitus, bronchial asthma, interstitial lung disease, coronary artery disease, cardiomyopathies, connective tissue disorders, recipients of organ transplant or immunosuppressive therapy, patients having co morbidities like renal failure, liver failure, heart failure and patients on other drugs known to cause hyperglycemia were excluded. Random blood sugar, HbA1C etc, was done and data was analyzed by SPSS version 22.0.Results: Total 46 patients were included in study. Mean age was 63.22 years with minimum age 47 years and maximum age 80 years. 35 patients (76.09%) were male and 11 patients (23.91%) were female. Sex ratio was 3.18:1. 26 patients (56.52%) were taking both inhaled and systemic corticosteroids, 12 patients (26.09%) were taking only inhaled corticosteroids and 8 patients (17.39%) were taking systemic corticosteroid therapy. 14 patients (30.43%) were found to have impaired glucose tolerance, 7 patients (15.22%) were diagnosed as a case of diabetes mellitus and 25 patients (54.35%) were found to be euglycemic.Conclusions: We conclude that incidence of deranged glycemic status is more common among COPD patients receiving only systemic corticosteroid therapy or both systemic and inhaled corticosteroid therapy. Further, inhaled corticosteroids are better in terms of glycemic control among COPD patients on corticosteroid therapy. Hence, we recommend routine screening of glycemic status in COPD patients on corticosteroid therapy.

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