We observed that some patients had presented with different prenatal or perinatal forms of edema in some kindreds. Within weeks or months after birth, these exhibited a spontaneous, complete and definitive resorption. [ncbi.nlm.nih.gov]

We describe the case of a 16-year-old girl who presented neonatally with abnormal liver function tests and ascites. She was extensively investigated at that time. A liver biopsy showed hepatitis and fatty changes. [unboundmedicine.com]

Clinical description The clinical presentation of the disease is very heterogeneous. [rarediseases.info.nih.gov]

• Inflammation

  A mild portal inflammation was seen, which included a few eosinophils. [docplayer.net]

• Collapse

  We report a case of undiagnosed myasthenia gravis presenting with postpartum collapse. [dokumen.tips]

• Internal Bleeding

  Th ere was no sign of internal bleeding and vaginal bleeding was minimal. She lost consciousness later and was intubated by the anaesthetist. She was transferred to intensive care with an intravenous dopamine infusion. [dokumen.tips]

• Sputum

  Bronchoscopy revealed thick copious yellowish sputum throughout her right upper lobe and suction was performed. Her husband recalled that the patient had generalised weakness throughout the pregnancy. [dokumen.tips]

• Respiratory Distress

  Initial respiratory distress progressively disappeared and blood gases became normal with a CPAP for 13 days. [dokumen.tips]

• Recurrent Abdominal Pain

  Recurrent abdominal pain led to cholecystectomy at 15 years. Studies showed no evidence of preferential splenic sequestration or destruction, so splenectomy was not performed. [fn.bmj.com]

• Heart Murmur

  A heart murmur and mild heart failure was noted at day. On day 2, marked abdominal swelling was noted and shown to be due to ascites. One week later, she became jaundiced and the bilirubin was 109 lmol/l (100 lmol/l conjugated). [docplayer.net]

• Scleral Icterus

  The clinical presentation is heterogeneous, ranging from mild to moderate hemolytic anemia associated with scleral icterus, splenomegaly, and choletithiasis. Iron overload may develop later in life. [ncbi.nlm.nih.gov]

  Further history revealed intermittent jaundice and scleral icterus. His physical examination was notable for short stature, dental caries, bilateral short fourth fingers, and splenomegaly (spleen palpable 4 cm below the costal margin). [bloodjournal.org]

• Skin Edema

  No skin edema and, in particular, nonuchal edema, were detected. [docslide.fr]

• Cesarean Section

  Oneshould prepare for intensive care (prenatal andpostnatal draining of fluids, and Cesarean section).Treating anemia with intrauterine transfusions wouldnot be justified by the degree of anemia and would notreduce the edema (Entezami et al., 1996). [docslide.fr]

• Vaginal Bleeding

  Th ere was no sign of internal bleeding and vaginal bleeding was minimal. She lost consciousness later and was intubated by the anaesthetist. She was transferred to intensive care with an intravenous dopamine infusion. [dokumen.tips]

• Anisopoikilocytosis

  The peripheral smear showed anisocytosis and marked stomatocytosis (panel A: peripheral blood, original magnification 100; anisopoikilocytosis and increased stomatocytes [arrow]). [bloodjournal.org]

  Her blood film was re-examined at this stage and showed anisopoikilocytosis with stomatocytes, target cells and some cells with two areas of central pallor) (Fig 1). [docplayer.net]

  A blood film showed reticulocytosis (14%), anisopoikilocytosis, schistocytes, and target cells (fig 3). Her haemolytic anaemia was extensively investigated, a striking finding being decreased osmotic fragility. [fn.bmj.com]

• Anisocytosis

  The peripheral smear showed anisocytosis and marked stomatocytosis (panel A: peripheral blood, original magnification 100; anisopoikilocytosis and increased stomatocytes [arrow]). [bloodjournal.org]

  Haemoglobin was 159 g/l at birth, mean corpuscular volume 95–97 fl, with anisocytosis, occasional fragments, and target cells. White cells and platelets were normal. [fn.bmj.com]

  Of note was a disarray of liver cell plates with marked anisocytosis and widespread steatosis in predominantly small vacuoles (Fig 2B). There were a few shrunken, deeply eosinophilic hepatocytes and occasional giant hepatocytes. [docplayer.net]

Splenectomy is a highly deleterious treatment, because it favors, with virtually no exception, the occurrence of thromboembolic disease. We describe here the extreme case of a patient with DHS and an associated sickle cell trait. [kclpure.kcl.ac.uk]

Symptomatic treatment of the hydrops assists survival until spontaneous resorption occurs. [ncbi.nlm.nih.gov]

Management and treatment Treatment is mainly symptomatic. Occurrence of cholelithiasis should be regularly monitored. Folic acid supplementation should be proposed in case of anemia. Pregnancy should be closely monitored. [rarediseases.info.nih.gov]

It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. Our staff consists of biologists and biochemists that are not trained to give medical advice . [uniprot.org]

Prognosis Overall prognosis is favorable in well managed patients (not splenectomized and with regular monitoring of their iron status). Splenectomized patients are at risk of early or late thrombotic events. Last updated: 12/15/2015 [rarediseases.info.nih.gov]

Splenectomy is contraindicated in DHS due to an elevated risk of life threatening arterial and venous thrombotic events.PrognosisOverall prognosis is favorable in well managed patients (not splenectomized and with regular monitoring of their iron status [malacards.org]

DHS is a rare cause of ascites with a relatively benign prognosis (Ami et al. 2009). It is defi ned by mild anaemia, increased mean corpuscular volume and increased mean corpuscular haemoglobin concentration. [dokumen.tips]

The importance of this rare condition lies in its relatively benign prognosis compared with the more common and more devastating causes of non-immune fetal hydrops (parvovirus infection, cardiac malformation or dysfunction, and aneuploidy). [fn.bmj.com]

Prognosis Some clinical problems in Hereditary Stomatocytosis are mild enough to let the patient live a normal life. Although Gallstones can be a problem, that can be easily fixed with a surgery. [hereditarystomatocytosiskw.weebly.com]

Congenital/surgery Animals Female Humans Hydrops Fetalis/genetics* Hydrops Fetalis/surgery Intermediate-Conductance Calcium-Activated Potassium Channels/genetics* K562 Cells Male Mice Mutation, Missense* Splenectomy/adverse effects Thromboembolism/etiology [ncbi.nlm.nih.gov]

Etiology Most reported DHS cases are caused by gain-of-function mutations in the gene PIEZO1 (16q24.3) which encodes part of a mechanosensitive ion channel. [rarediseases.info.nih.gov]

Salomon, Jonathan Rosenblatt, Romain Favre, Véronique Houfflin‐Debarge, Bernard Broussin, Fabien Guimiot, Honorine Fenaux, Anne‐Lise Delezoide and Françoise Muller, Biochemical analysis of ascites fluid as an aid to etiological diagnosis: a series of [doi.org]

The etiology of these disorders of cation permeability is unknown. [medtextfree.wordpress.com]

Articles include: Epidemiology of Pulmonary Arterial Hypertension, Pathology of Pulmonary Hypertension, Genetics of Pulmonary Arterial Hypertension, Diagnosis of Pulmonary Arterial Hypertension, Pulmonary Hypertension Owing to Left Heart Disease, Pulmonary [books.google.com]

Epidemiology The prevalence of DHS is unknown but to date, about 20 families with DHS have been described in the literature. Clinical description The clinical presentation of the disease is very heterogeneous. [rarediseases.info.nih.gov]

Epidemiology, modes of transmission and prevention of infections and the need for routine use of current infection control measures as recommended by the U.S. centers for disease control and prevention. DHS 105.17(1n)(a)2.g. g. [docs.legis.wisconsin.gov]

Our findings also suggest a new role for mechanotransduction in red blood cell biology and pathophysiology. [ncbi.nlm.nih.gov]

Balances summaries of relevant pathophysiology with clear, practical clinical guidance to help you thoroughly understand the underlying science of diseases. [books.google.com]

The primary defect is in the anion transporter, band 3 (see section on “Pathophysiology”). [oncologynurseadvisor.com]

It is this protein which mediates the cation leaks which are characteristic of this disease. [1] Pathophysiology [ edit ] Osmosis leads to the red blood cell having a constant tendency to swell and burst. [en.wikipedia.org]

Koralkova P, van Solinge WW, van Wijk R: Rare hereditary red blood cell enzymopathies associated with hemolytic anemia - pathophysiology, clinical aspects, and laboratory diagnosis. Int J Lab Hematol 2014 Jun;36(3):388-397 4. [mayomedicallaboratories.com]

[…] to disasters As the number and scope of threats to U.S. interests from hackers have grown in recent years, cyberterrorism prevention has become a key aspect of DHS homeland security efforts. [searchcompliance.techtarget.com]

The Family Violence Prevention and Services Program is an initiative that supports the establishment, maintenance and expansion of programs and projects to prevent family violence. [dhs.dc.gov]

Get optimal test results with guidance on error detection, correction, and prevention as well as cost-effective test selection. [books.google.com]

Fertility Preferences - total fertility rate, desired family size, marriage and sexual activity Gender/Domestic Violence - history of domestic violence, frequency and consequences of violence HIV/AIDS Knowledge, Attitudes, and Behavior - knowledge of HIV prevention [dhsprogram.com]