scholarly journals A Toddler Presenting with Pulmonary Renal Syndrome

2017 ◽  
Vol 7 (2) ◽  
pp. 73-80 ◽  
Author(s):  
Florence A. Aeschlimann ◽  
Rae S.M. Yeung ◽  
Ronald M. Laxer ◽  
Diane Hebert ◽  
Ashley Cooper ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Treatment Options ◽  
Renal Involvement ◽  
Pulmonary Hemorrhage ◽  
Disease Systemic Lupus Erythematosus ◽  
Threatening Condition ◽  
Life Threatening ◽  
History Of ◽  
Pulmonary Renal Syndrome ◽  
Clinically Significant

Pulmonary renal syndrome refers to an association of pulmonary and glomerular disease and includes disorders, such as the ANCA-associated vasculitides, anti-glomerular basement membrane antibody disease, systemic lupus erythematosus, and IgA vasculitis (Henoch-Schönlein purpura). We present the medical history of a 26-month-old boy with an extensive purpuric rash, involving the limbs, trunk, and face, who developed clinically significant pulmonary hemorrhage and renal involvement. Rapid recognition of this rare but potentially life-threatening condition is crucial. In this report, we discuss the differential diagnosis, diagnostic studies, and treatment options to consider when facing a young child presenting with a pulmonary renal syndrome.

scholarly journals An uncommon cause of diffuse alveolar hemorrhage in a young male

2019 ◽  
Vol 89 (2) ◽  
Author(s):  
Anshul Mittal ◽  
Jagdish Chander Suri ◽  
Shibdas Chakrabarti ◽  
Pranav Ish
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Renal Involvement ◽  
Young Male ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Systemic Lupus ◽  
Threatening Condition ◽  
Life Threatening

It is uncommon for Systemic lupus erythematosus (SLE) to present with diffuse alveolar hemorrhage (DAH) as the initial presentation. To diagnose this in a young male with no renal involvement is further uncommon. We report a case of a 16-year-old boy, who presented with hemoptysis and was eventually diagnosed as DAH with underlying SLE. Treatment with steroids and immunosuppressant helped in rapid recovery from this potentially life-threatening condition. This case highlights the need of defining diagnostic criteria for SLE in patients presenting as DAH and formulating guidelines for treatment of the same, especially in absence of co-existing lupus nephritis.

scholarly journals Hydralazine Induces Myeloperoxidase and Proteinase 3 Anti-Neutrophil Cytoplasmic Antibody Vasculitis and Leads to Pulmonary Renal Syndrome

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Gaurav Agarwal ◽  
Ghayyath Sultan ◽  
Sherry L. Werner ◽  
Claudia Hura
Keyword(s):  
Systemic Vasculitis ◽  
High Titer ◽  
Pulmonary Hemorrhage ◽  
Kidney Injury ◽  
Necrotizing Glomerulonephritis ◽  
Threatening Condition ◽  
Life Threatening ◽  
Pulmonary Renal Syndrome ◽  
One Year

We report a case of hydralazine-induced ANCA-associated glomerulonephritis with pulmonary hemorrhage. A 62-year-old Hispanic man with hypertension, who was being treated with hydralazine 100 mg three times a day for four and half years, presented to the hospital with severe anemia. He had acute kidney injury and urinalysis showed proteinuria, dysmorphic RBCs, and rare RBC cast. CT scan of the chest revealed bilateral pulmonary ground-glass infiltrates. Transbronchial biopsy was consistent with pulmonary hemorrhage. Serologic tests showed high titer PR3 ANCA and, to a lesser extent, MPO ANCA. Kidney biopsy revealed focal segmental necrotizing glomerulonephritis with crescents, without evidence of immune complex deposits. Hydralazine was discontinued and the patient was treated with corticosteroids and intravenous cyclophosphamide. At one-year follow-up, he had no symptoms and anemia had resolved. Kidney function improved dramatically. Serology showed undetectable PR3 ANCA and minimally elevated MPO ANCA. To our knowledge, hydralazine-associated PR3 ANCA has not been previously reported. The possibility of ANCA systemic vasculitis should be included in the differential diagnosis of any patient with hydralazine use and pulmonary renal syndrome. This is a potentially life threatening condition requiring prompt cessation of the drug and treatment with glucocorticoids and immunosuppression.

scholarly journals Myelodysplastic Syndrome Clinically Presenting with the “Classic TTP Pentad”

2017 ◽  
Vol 2017 ◽  
pp. 1-6 ◽  
Author(s):  
Santiago Fabián Moscoso Martínez ◽  
Evelyn Carolina Polanco Jácome ◽  
Elizabeth Guevara ◽  
Vijay Mattoo
Keyword(s):  
Myelodysplastic Syndrome ◽  
Clinical Presentation ◽  
Renal Involvement ◽  
Medical Emergency ◽  
Complete Blood Cell Count ◽  
Blood Cell Count ◽  
Thrombocytopenic Purpura ◽  
Life Saving ◽  
Threatening Condition ◽  
Life Threatening

The clinical presentation of myelodysplastic syndrome (MDS) is not specific. Many patients can be asymptomatic and can be detected only due to an abnormal complete blood cell count (CBC) on routine exam or for other reasons while others can be symptomatic as a consequence of underlying cytopenias. Thrombotic thrombocytopenic purpura (TTP) usually is suspected under the evidence of microangiopathic hemolytic anemia (MAHA) and thrombocytopenia and because it is a life-threatening condition (medical emergency) immediate initiation of plasmapheresis could be life-saving. The following case illustrates an unusual presentation of MDS in a patient who came in to the emergency room with the classic TTP “pentad” of fever, renal involvement, MAHA, mental status changes, and thrombocytopenia. We will focus our discussion in the clinical presentation of this case.

Bradyarrhythmias

2016 ◽  
Author(s):  
Inna Leybell ◽  
Liliya Abrukin
Keyword(s):  
Atrioventricular Block ◽  
Sinus Bradycardia ◽  
Sinus Node ◽  
Treatment Options ◽  
Presenting Symptoms ◽  
Threatening Condition ◽  
Node Disease ◽  
Life Threatening ◽  
Sinus Node Disease ◽  
Sinoatrial Block

Bradyarrhythmias can present as an incidental electrocardiographic (ECG) finding or a life-threatening condition requiring immediate intervention. They are caused by sinus node disease or atrioventricular block. This review covers pathophysiology, stabilization and assessment, diagnosis and treatment options, and disposition and outcomes for patients with bradycardia. Figures in the review demonstrate characteristic ECG tracings. Tables list classifications of sinus node dysfunction as well as of sinoatrial and atrioventricular blocks, presenting symptoms, important information to elicit on history taking, various causes of bradycardia, and specific interventions for toxicologic etiologies. Key words: atrioventricular block, atropine, bradyarrhythmia, bradycardia, first-degree atrioventricular block, second-degree atrioventricular block, sinoatrial block, sinus bradycardia, sinus node disease, tachycardia-bradycardia syndrome, third-degree atrioventricular block This review contains 9 highly rendered figures, 7 tables, and 92 references.

scholarly journals Rare Treatments for Rare Dyslipidemias: New Perspectives in the Treatment of Homozygous Familial Hypercholesterolemia (HoFH) and Familial Chylomicronemia Syndrome (FCS)

2021 ◽  
Vol 23 (11) ◽  
Author(s):  
Laura D’Erasmo ◽  
Simone Bini ◽  
Marcello Arca
Keyword(s):  
Familial Hypercholesterolemia ◽  
Treatment Options ◽  
New Drugs ◽  
Lipid Lowering ◽  
Homozygous Familial Hypercholesterolemia ◽  
Published Evidence ◽  
Novel Drugs ◽  
Life Threatening ◽  
History Of ◽  
Definition Of

Abstract Purpose of Review This review aims to summarize the most recent published literature concerning lomitapide and volanesorsen that are approved for the use in HoFH and FCS patients, respectively. Moreover, it will briefly revise the published evidence on novel, non-approved treatments that are under evaluation for the management of these rare forms of dyslipidemias Recent Findings The definition of rare dyslipidemias identifies a large number of severe disorders of lipid metabolism of genetic origin. Among them were homozygous familial hypercholesterolemia (HoFH) (OMIM #143890) and familial chylomicronemia syndrome (FCS) (OMIM #238600), which are characterized by a markedly impaired cholesterol- and triglyceride-containing lipoproteins metabolism. They are being particularly associated with poor health outcomes and quality of life. Considering the severity of these diseases, common lipid-lowering drugs are often ineffective or do not allow to achieve the recommended lipid targets to prevent the development of complications. Nowadays, several new drugs have been found to effectively treat HoFH and FCS with an acceptable safety profile. Summary Treating patients with HoFH and FCS remains very challenging. However, novel treatment options are emerging and might be considered in addition to conventional therapy for managing these diseases. These novel drugs will possibly change the natural history of these two rare and life-threatening diseases.

scholarly journals Haemophilic arthropathy

2014 ◽  
Vol 2 (4) ◽  
pp. 225-227
Author(s):  
Sarath Chandra Mouli V ◽  
Pradeep H
Keyword(s):  
Factor Viii ◽  
Lupus Erythematosus ◽  
Treatment Options ◽  
Left Groin ◽  
Left Hand ◽  
Acquired Haemophilia ◽  
Haemophilic Arthropathy ◽  
Fatal Bleeding ◽  
Coagulant Activity ◽  
History Of

Acquired haemophilia (AH) is extremely rare and potentially fatal bleeding disorder and it is associated with significant morbidity and mortality rate between 7.9% to 22%. Upto 90% of people with AH experience bleeding. It is mainly due to the spontaneous development of auto antibodies that inhibit the coagulant activity of factor VIII (FVIII). Autoimmune disorders (16.6%) like Rheumatoid arthritis, Systemic lupus erythematosus (SLE), Multiple Sclerosis etc., may lead to trigger acquired inhibitors to Factor VIII. In the case report we described a 70-year-old male patient who has history of migratory joint pains and swellings for five months and on physical examination there was an echymotic rash over the left hand elbow and left groin extending into the left thigh and we reemphasize that even though a series of effective treatment options available, a through workup, early diagnosis and early treatment are important.

Haemophagocytic lymphohistiocytosis secondary to brucellosis in a young child

2021 ◽  
Vol 14 (3) ◽  
pp. e240759
Author(s):  
Jashan Mittal ◽  
Prawin Kumar ◽  
Jagdish Prasad Goyal ◽  
Abhishek Purohit
Keyword(s):  
Haemophagocytic Lymphohistiocytosis ◽  
Appetite Loss ◽  
Specific Therapy ◽  
Adequate Treatment ◽  
Underlying Condition ◽  
Persistent Fever ◽  
Threatening Condition ◽  
Life Threatening ◽  
History Of ◽  
Life Threatening Complication

Brucellosis is a common zoonotic disease worldwide. It has protean clinical manifestation and sometimes may has a life-threatening complication. A 4-year-old boy presented with a history of fever, myalgia and appetite loss for 3 weeks. On examination, he had hepatosplenomegaly. The initial working diagnosis was an infection, autoimmune disease and malignancy. Investigations showed positive Brucella serology, and he was started on rifampicin and cotrimoxazole. He was further investigated because of persistent fever, which revealed evidence of haemophagocytic lymphohistiocytosis (HLH). He continued treatment for brucellosis, except rifampicin which was replaced with doxycyclin due to a worsening liver function. The child showed complete clinical and biochemical improvement after 6 weeks of therapy. HLH is a life-threatening condition and should be suspected in children with brucellosis, who did not respond to appropriate antibiotics treatment. Secondary HLH does not always require specific therapy; it may improve with adequate treatment of the underlying condition.

scholarly journals Non-occlusive mesenteric ischaemia associated with anorexia nervosa

2019 ◽  
Vol 12 (5) ◽  
pp. e229703
Author(s):  
Takashi Sakamoto ◽  
Alan Kawarai Lefor ◽  
Tadao Kubota
Keyword(s):  
Anorexia Nervosa ◽  
Distal Ileum ◽  
Interdisciplinary Management ◽  
Emergency Laparotomy ◽  
Diffuse Peritonitis ◽  
Chronic Haemodialysis ◽  
Mesenteric Ischaemia ◽  
Threatening Condition ◽  
Life Threatening ◽  
History Of

Non-occlusive mesenteric ischaemia (NOMI) is a life-threatening condition that requires emergent intervention and anorexia nervosa is a chronic eating disorder that requires careful medical and nutritional management. A 54-year-old woman with a history of anorexia nervosa and undergoing chronic haemodialysis developed abdominal pain and called an ambulance. On arrival, she was in shock and abdominal examination was consistent with diffuse peritonitis. Computed tomography scan suggested ischaemia from the distal ileum to the ascending colon. Emergency laparotomy revealed NOMI from the distal ileum to the transverse colon. The treatment strategy included staged operations and careful medical management to optimise nutritional support and electrolyte management with survival of the patient. NOMI and anorexia nervosa are both difficult to manage. Meticulous interdisciplinary management can result in a good outcome.

scholarly journals Aortic Dissection in a Pregnant Patient without Other Risk Factors

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Priya V. Patel ◽  
Raanan Alter ◽  
Recia Frenn ◽  
Thaddeus P. Waters
Keyword(s):  
Risk Factors ◽  
Aortic Dissection ◽  
Aortic Rupture ◽  
Treatment Options ◽  
Pregnant Patient ◽  
Physical Exam ◽  
Vascular Events ◽  
Increased Risk ◽  
Threatening Condition ◽  
Life Threatening

Background. An aortic dissection is a life-threatening condition in which the intima of the artery tears causing separation of the intima and media. Pregnancy places women at a significantly increased risk of common vascular events including venous thromboembolism, myocardial infarction, and stroke, while also increasing the risk of rarer vascular events such as aortic dissection and aortic rupture. Case. A 30-year-old previously healthy multiparous woman presenting at 36 weeks of pregnancy with a Type A aortic dissection. She underwent a combined emergent cesarean delivery followed by repair of her aortic root. Conclusions. Aortic dissection should be high on the differential for pregnant patients presenting with the characteristic complaints and physical exam findings given the high mortality rate associated with this vascular event. Teaching Points. (1) This report reviews the characteristic presentation, risk factors, and physical exam findings in a patient with an aortic dissection. (2) The report includes treatment options for pregnant patients based on the classification of the dissection.

Salicylate Intoxication

1997 ◽  
Vol 12 (2) ◽  
pp. 66-78 ◽  
Author(s):  
Luke Yip ◽  
Michael S. Jastremski ◽  
Richard C. Dart
Keyword(s):  
Life Support ◽  
Care Facility ◽  
Health Care Facility ◽  
Poison Control ◽  
Electrolyte Balance ◽  
Threatening Condition ◽  
Life Threatening ◽  
History Of ◽  
Salicylate Intoxication ◽  
Base Management

Aspirin (acetylsalicylic acid) is one of the most widely used over-the-counter medications. Because of its availability and widespread use, aspirin has a long history of human toxicity from accidental or intentional overdosing. According to the American Association of Poison Control Centers aspirin was implicated in 19083 exposures in 1995, with 11800 cases treated in a health care facility, and 52 associated deaths. Aspirin toxicity may be a life-threatening condition that produces multiple system organ failure requiring treatment in an intensive care unit. Managing a patient with salicylism will challenge the skills of the critical care team, especially in the areas of life support, fluid and electrolyte balance, and acid-base management. This article reviews the physiology, pathophysiology, acute and chronic salicylism in children and adults, and management of salicylate intoxication.

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