Case presentation An 18 year old female patient presented with acute symptoms of abdominal and flank pain, nausea, dark red urine, fevers and chills. [bmchematol.biomedcentral.com]

Hemoglobinuria results in the production of classically dark urine during the night and in the morning (about 25 % of patients), and patients may present with renal insufficiency. Jaundice may be present. [orpha.net]

• Tachypnea

  Affected cows also may have fever, diarrhea, and tachypnea. Cows that survive the hemolytic crisis may take several months to recover completely. [msdvetmanual.com]

• Abdominal Tenderness

  On physical exam, her skin is jaundiced, her liver edge is palpable, and she has abdominal tenderness to palpation. Her physicians are suspicious of Budd-Chiari syndrome. [step1.medbullets.com]

• Red Urine

  With clinical disease, rapid intravascular hemolysis leads to severe anemia, tachycardia, weakness, hemoglobinuria with dark brown or red urine, and pallor over several days. Milk production drops rapidly. [msdvetmanual.com]

  The clinical features of PNH are shown in Table 1 .[ 3, 4 ] The most common are fatigue (typically out of proportion to the degree of anemia), shortness of breath, and hemoglobinuria (red urine), especially in the first morning sample ( Figure 1 ). [bcmj.org]

• Nocturia

  10-CM D59.6 Hemoglobinuria due to hemolysis from other external causes Or: 2015/16 ICD-10-CM D59.8 Other acquired hemolytic anemias Approximate Synonyms Extrinsic hemoglobinuria Hemoglobinuria - external causation Hemoglobinuria, extrinsic Paroxsymal nocturia [icd9data.com]

  "Eine neue form intermittierender haemoglobinurie (Haemoglobinuria paroxysmalis nocturia)". Klin Wochenschr (in German). 7 (43): 2045–2047. doi : 10.1007/BF01846778. External links [ edit ] [en.wikipedia.org]

  […] neoplasms ( C00-D49 ) symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified ( R00 - R94 ) Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism Approximate Synonyms Paroxsymal nocturia [icd10data.com]

A subsequent workup failed to reveal any cardiac or pulmonary cause for her symptoms, and abdominal imaging was also unremarkable. [hematologyandoncology.net]

The laboratory workup revealed a white blood cell count 4.6 k/uL, RBC count of 3.8 k/uL, hemoglobin of 11.8 g/dL, hematocrit 35%, MCV 91 fl, platelets 327 k/uL, reticulocytes 24.5%, PTT 27.8, INR 1.7, D dimer 2.5 ug/mL, bilirubin 1.3 mg/dL, bilirubin [f1000research.com]

These differentials were kept in mind prior to ordering the workup. His laboratory data at admission are outlined in table 1. A previous renal function test was done in 2007 which showed 97.24 µmol/l serum creatinine and 4.99 mmol/l BUN. [karger.com]

• Albuminuria

  Urobilinogen Bilirubin Creatinine RBC WBC Urinary casts Chemical properties Urine specific gravity Urine osmolality Urine pH Urine anion gap Abnormal findings Red blood cells Hematuria ( Microscopic hematuria ) White blood cells Eosinophiluria Proteinuria Albuminuria [en.wikipedia.org]

  Proteinuria 1.3 g/day with albuminuria 170 mg/day and non-selective aminoaciduria, thus indicating PTD. The acid-base status, phosphate and uric acid were normal. [revistanefrologia.com]

• Microalbuminuria

  Bilirubin Creatinine RBC WBC Urinary casts Chemical properties Urine specific gravity Urine osmolality Urine pH Urine anion gap Abnormal findings Red blood cells Hematuria ( Microscopic hematuria ) White blood cells Eosinophiluria Proteinuria Albuminuria / Microalbuminuria [en.wikipedia.org]

• Ketonuria

  […] gravity Urine osmolality Urine pH Urine anion gap Abnormal findings Red blood cells Hematuria ( Microscopic hematuria ) White blood cells Eosinophiluria Proteinuria Albuminuria / Microalbuminuria Myoglobinuria Hemoglobinuria Small molecules Glycosuria Ketonuria [en.wikipedia.org]

• Hyperuricosuria

  Urine pH Urine anion gap Abnormal findings Red blood cells Hematuria ( Microscopic hematuria ) White blood cells Eosinophiluria Proteinuria Albuminuria / Microalbuminuria Myoglobinuria Hemoglobinuria Small molecules Glycosuria Ketonuria Bilirubinuria Hyperuricosuria [en.wikipedia.org]

• Glycosuria

  […] specific gravity Urine osmolality Urine pH Urine anion gap Abnormal findings Red blood cells Hematuria ( Microscopic hematuria ) White blood cells Eosinophiluria Proteinuria Albuminuria / Microalbuminuria Myoglobinuria Hemoglobinuria Small molecules Glycosuria [en.wikipedia.org]

• Hemoglobin Increased

  Hemoglobin increased to 9.8 g/dl; reticulocyte count normalized, LDH was normal, centrifuged sample of serum returned to normal color [Figure 4]. Jaundice subsided, and renal parameters also improved. Urine color returned to normal [Figure 5]. [atmph.org]

an increase in serum creatinine by 25% or more compared to baseline or nadir during Soliris treatment; or, an increase in serum LDH by 25% or more over baseline or nadir during Soliris treatment. [soliris.net]

Prognosis after immunosuppressive therapy (IST) in the good prognosis and the poor prognosis group. (A) Overall survival. (B) Failure-free survival. (C) Transplantation-free survival. [haematologica.org]

Prevention, and Complications Prognosis improved with use of eculizumab Complications renal insufficiency thromboembolism 5% develop myelodysplasia 2.5% develop acute leukemias Please rate topic. [medbullets.com]

Treatment and prognosis blood transfusions anticoagulation bone marrow transplant 1 eculizumab 1 Without therapy approximately 50% of patients die as a direct result of the disease. Many others are transfusion dependent for decades 3. [radiopaedia.org]

Artesunate was stopped suspecting it to be the etiological agent of hemolysis and hemoglobinuria. [atmph.org]

Pathogenesis Etiology Erythrocyte lysis within urine Hematuria Hematuria. Intravascular hemolysis Hemoglobin filtered by glomeruli. Intravascular hemolysis is more likely to result in hemoglobinuria than is extravascular hemolysis. Idiopathic. [vetstream.com]

Etiology PNH is caused by somatic mutations in the PIGA gene (Xp22.1), encoding a protein involved in the biosynthesis of the glycosylphosphatidylinositol (GPI) anchor. [orpha.net]

Summary Epidemiology Although PNH has been described worldwide, exact prevalence data are not available. It is estimated at 1/80,000 in France. [orpha.net]

[…] granulocytes have thrombosis within 10 years after diagnosis Flow cytometry testing – monitor response to monoclonal antibody therapy directed against C5 Therapy success associated with increased ratios of erythrocyte clones to granulocyte clones Background Epidemiology [arupconsult.com]

He then did Masters in Science from the Department of Epidemiology at the Medical College of Wisconsin. [karger.com]

Over the last 20 years, we have seen a dramatic unraveling of the pathophysiology of this rare and complex disorder. [dovepress.com]

PNH is rarely investigated in stroke patients and its contribution to the pathophysiology of ESUS is unknown. [clinicaltrials.gov]

Pathophysiology Hemoglobinuria may result from either of two main sources: True hemoglobinuria results when hemoglobin is filtered by the glomeruli. Hemoglobinuria also results from hematuria when erythrocytes are lysed within the urine. [vetstream.com]

Correction of mineral deficiencies and elimination of plant toxins from the diet may help prevent recurrence. 2018 Merck Sharp & Dohme Corp., a subsidiary of Merck & Co., Inc., Kenilworth, NJ, USA) [msdvetmanual.com]

Prevention There is no known way to prevent this disorder. References Brodsky RA. Paroxysmal nocturnal hemoglobinuria. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 7th ed. [mountsinai.org]

Blood thinners may also be needed to prevent clots from forming. Soliris (eculizumab) is a drug used to treat PNH. It blocks the breakdown of red blood cells. Bone marrow transplantation can cure this disease. [nlm.nih.gov]

Absence of PIG-A function in a cell prevents synthesis of the glycosylphosphatidylinositol (GPI) moiety, which anchors many different types of proteins to the cell membrane. [clinicaltrials.gov]