{"id":219,"date":"2019-11-28T12:11:58","date_gmt":"2019-11-28T12:11:58","guid":{"rendered":"http:\/\/www.rhesusnegative.net\/staynegative\/?p=219"},"modified":"2020-01-22T04:37:33","modified_gmt":"2020-01-22T04:37:33","slug":"rare-diseases-rh-deficiency-syndrome","status":"publish","type":"post","link":"https:\/\/www.rhesusnegative.net\/staynegative\/rare-diseases-rh-deficiency-syndrome\/","title":{"rendered":"Rare diseases: Rh deficiency syndrome"},"content":{"rendered":"<div style=\"padding-bottom:20px; padding-top:10px;\" class=\"hupso-share-buttons\"><!-- Hupso Share Buttons - https:\/\/www.hupso.com\/share\/ --><a class=\"hupso_toolbar\" href=\"https:\/\/www.hupso.com\/share\/\"><img data-recalc-dims=\"1\" decoding=\"async\" src=\"https:\/\/i0.wp.com\/static.hupso.com\/share\/buttons\/share-medium.png?w=640&#038;ssl=1\" style=\"border:0px; padding-top:5px; float:left;\" alt=\"Share Button\"\/><\/a><script type=\"text\/javascript\">var hupso_services_t=new Array(\"Twitter\",\"Facebook\",\"Pinterest\",\"Linkedin\");var hupso_background_t=\"#EAF4FF\";var hupso_border_t=\"#66CCFF\";var hupso_toolbar_size_t=\"medium\";var hupso_twitter_via = \"datebytype\";var hupso_image_folder_url = \"\";var hupso_twitter_via=\"datebytype\";var hupso_url_t=\"\";var hupso_title_t=\"Rare diseases: Rh deficiency syndrome\";<\/script><script type=\"text\/javascript\" src=\"https:\/\/static.hupso.com\/share\/js\/share_toolbar.js\"><\/script><!-- Hupso Share Buttons --><\/div>\n<div class=\"wp-block-image\"><figure class=\"aligncenter\"><img data-recalc-dims=\"1\" loading=\"lazy\" decoding=\"async\" width=\"401\" height=\"250\" src=\"https:\/\/i0.wp.com\/www.rhesusnegative.net\/staynegative\/wp-content\/uploads\/2020\/01\/rh-deficiency-syndrome.jpg?resize=401%2C250&#038;ssl=1\" alt=\"\" class=\"wp-image-6136\" srcset=\"https:\/\/i0.wp.com\/www.rhesusnegative.net\/staynegative\/wp-content\/uploads\/2020\/01\/rh-deficiency-syndrome.jpg?w=401&amp;ssl=1 401w, https:\/\/i0.wp.com\/www.rhesusnegative.net\/staynegative\/wp-content\/uploads\/2020\/01\/rh-deficiency-syndrome.jpg?resize=300%2C187&amp;ssl=1 300w\" sizes=\"auto, (max-width: 401px) 100vw, 401px\" \/><figcaption> The\u00a0<strong>Rh deficiency syndrome<\/strong>\u00a0, also known as\u00a0<strong>Rh<\/strong>-null\u00a0<strong>syndrome<\/strong>, is a blood\u00a0<strong>disorder<\/strong>\u00a0where people have red blood cells (RBCs) lacking all\u00a0<strong>Rh<\/strong>\u00a0antigens. The\u00a0<strong>Rh<\/strong>\u00a0antigens maintain the integrity of the RBC membrane and therefore, RBCs which lack\u00a0<strong>Rh<\/strong>\u00a0antigens have an abnormal shape. <\/figcaption><\/figure><\/div>\n\n\n<p>Rh deficiency syndrome designates rare individuals who have either absent (Rhnull) or markedly reduced (Rhmod) Rh antigen expression, mild to moderate hemolytic anemia associated with the presence of stomatocytes, and occasional spherocytes on the peripheral blood film. Hemolytic anemia is improved by splenectomy.<\/p>\n<p>Here is one example of a case:<\/p>\n<blockquote><p>The hematological aspects of the original case of Rhmod are reported. The subject, as in other reported cases, had a chronic hemolytic anemia characterized by stomatocytosis, reduced osmotic fragility, and abnormal autohemolysis correctable with the addition of glucose. The 51Cr red cell survival studies showed the spleen to be the preferential site of red cell destruction and splenectomy produced a dramatic improvement in red cell survival. The topic of Rh deficiency syndrome (Rhnull and Rhmod) is briefly reviewed with regard to the number of cases reported, to genetic aspects, to the hematological findings, and to the results of splenectomy.<\/p><\/blockquote>\n<p>A cause for Rh deficiency and Rh-null hemolytic anemia (RHN) is <strong><a href=\"http:\/\/www.ncbi.nlm.nih.gov\/gene?Db=gene&amp;Cmd=ShowDetailView&amp;TermToSearch=6005\" target=\"_blank\" rel=\"noopener noreferrer\">RHAG <\/a><\/strong> (Rh-associated glycoprotein).<br>\nThe protein encoded by this gene is erythrocyte-specific and is thought to be part of a membrane channel that transports ammonium and carbon dioxide across the blood cell membrane. The encoded protein appears to interact with Rh blood group antigens and Rh30 polypeptides. Defects in this gene are a cause of regulator type Rh-null hemolytic anemia (RHN), or Rh-deficiency syndrome.<\/p>\n<p>More details and information here:<\/p>\n<p><a href=\"http:\/\/www.rhesusnegative.net\/staynegative\/topics\/what-is-the-rh-deficiency-syndrome\/\" target=\"_blank\" rel=\"noopener noreferrer\">What is the Rh deficiency syndrome?<\/a><\/p><div style=\"padding-bottom:20px; padding-top:10px;\" class=\"hupso-share-buttons\"><!-- Hupso Share Buttons - https:\/\/www.hupso.com\/share\/ --><a class=\"hupso_toolbar\" href=\"https:\/\/www.hupso.com\/share\/\"><img data-recalc-dims=\"1\" decoding=\"async\" src=\"https:\/\/i0.wp.com\/static.hupso.com\/share\/buttons\/share-medium.png?w=640&#038;ssl=1\" style=\"border:0px; padding-top:5px; float:left;\" alt=\"Share Button\"\/><\/a><script type=\"text\/javascript\">var hupso_services_t=new Array(\"Twitter\",\"Facebook\",\"Pinterest\",\"Linkedin\");var hupso_background_t=\"#EAF4FF\";var hupso_border_t=\"#66CCFF\";var hupso_toolbar_size_t=\"medium\";var hupso_twitter_via = \"datebytype\";var hupso_image_folder_url = \"\";var hupso_twitter_via=\"datebytype\";var hupso_url_t=\"\";var hupso_title_t=\"Rare diseases: Rh deficiency syndrome\";<\/script><script type=\"text\/javascript\" src=\"https:\/\/static.hupso.com\/share\/js\/share_toolbar.js\"><\/script><!-- Hupso Share Buttons --><\/div>","protected":false},"excerpt":{"rendered":"<div style=\"padding-bottom:20px; padding-top:10px;\" class=\"hupso-share-buttons\"><!-- Hupso Share Buttons - https:\/\/www.hupso.com\/share\/ --><a class=\"hupso_toolbar\" href=\"https:\/\/www.hupso.com\/share\/\"><img src=\"https:\/\/static.hupso.com\/share\/buttons\/share-medium.png\" style=\"border:0px; padding-top:5px; float:left;\" alt=\"Share Button\"\/><\/a><script type=\"text\/javascript\">var hupso_services_t=new Array(\"Twitter\",\"Facebook\",\"Pinterest\",\"Linkedin\");var hupso_background_t=\"#EAF4FF\";var hupso_border_t=\"#66CCFF\";var hupso_toolbar_size_t=\"medium\";var hupso_twitter_via = \"datebytype\";var hupso_image_folder_url = \"\";var hupso_twitter_via=\"datebytype\";var hupso_url_t=\"\";var hupso_title_t=\"Rare diseases: Rh deficiency syndrome\";<\/script><script type=\"text\/javascript\" src=\"https:\/\/static.hupso.com\/share\/js\/share_toolbar.js\"><\/script><!-- Hupso Share Buttons --><\/div>Rh deficiency syndrome designates rare individuals who have either absent (Rhnull) or markedly reduced (Rhmod) Rh antigen expression, mild to moderate hemolytic anemia associated with the presence of stomatocytes, and occasional spherocytes on the peripheral blood film. Hemolytic anemia is improved by splenectomy. Here is one example of a case: The hematological aspects of the original case of Rhmod are reported. The subject, as in other reported cases, had a chronic hemolytic anemia characterized by stomatocytosis, reduced osmotic fragility, and abnormal autohemolysis correctable with the addition of glucose. The 51Cr red cell survival studies showed the spleen to be the preferential site of red cell destruction and splenectomy produced a dramatic improvement in red cell survival. The topic of Rh <div style=\"padding-bottom:20px; padding-top:10px;\" class=\"hupso-share-buttons\"><!-- Hupso Share Buttons - https:\/\/www.hupso.com\/share\/ --><a class=\"hupso_toolbar\" href=\"https:\/\/www.hupso.com\/share\/\"><img src=\"https:\/\/static.hupso.com\/share\/buttons\/share-medium.png\" style=\"border:0px; padding-top:5px; float:left;\" alt=\"Share Button\"\/><\/a><script type=\"text\/javascript\">var hupso_services_t=new Array(\"Twitter\",\"Facebook\",\"Pinterest\",\"Linkedin\");var hupso_background_t=\"#EAF4FF\";var hupso_border_t=\"#66CCFF\";var hupso_toolbar_size_t=\"medium\";var hupso_twitter_via = \"datebytype\";var hupso_image_folder_url = \"\";var hupso_twitter_via=\"datebytype\";var hupso_url_t=\"\";var hupso_title_t=\"Rare diseases: Rh deficiency syndrome\";<\/script><script type=\"text\/javascript\" src=\"https:\/\/static.hupso.com\/share\/js\/share_toolbar.js\"><\/script><!-- Hupso Share Buttons --><\/div>","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"jetpack_post_was_ever_published":false,"amp_status":"","_jetpack_newsletter_access":"","_jetpack_dont_email_post_to_subs":false,"_jetpack_newsletter_tier_id":0,"_jetpack_memberships_contains_paywalled_content":false,"_jetpack_memberships_contains_paid_content":false,"footnotes":"","jetpack_publicize_message":"","jetpack_publicize_feature_enabled":true,"jetpack_social_post_already_shared":true,"jetpack_social_options":{"image_generator_settings":{"template":"highway","enabled":false},"version":2}},"categories":[12,1],"tags":[],"class_list":["post-219","post","type-post","status-publish","format-standard","hentry","category-diseases","category-facts"],"jetpack_publicize_connections":[],"jetpack_featured_media_url":"","jetpack_sharing_enabled":true,"jetpack_shortlink":"https:\/\/wp.me\/p5DV68-3x","_links":{"self":[{"href":"https:\/\/www.rhesusnegative.net\/staynegative\/wp-json\/wp\/v2\/posts\/219","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.rhesusnegative.net\/staynegative\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.rhesusnegative.net\/staynegative\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.rhesusnegative.net\/staynegative\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/www.rhesusnegative.net\/staynegative\/wp-json\/wp\/v2\/comments?post=219"}],"version-history":[{"count":5,"href":"https:\/\/www.rhesusnegative.net\/staynegative\/wp-json\/wp\/v2\/posts\/219\/revisions"}],"predecessor-version":[{"id":6138,"href":"https:\/\/www.rhesusnegative.net\/staynegative\/wp-json\/wp\/v2\/posts\/219\/revisions\/6138"}],"wp:attachment":[{"href":"https:\/\/www.rhesusnegative.net\/staynegative\/wp-json\/wp\/v2\/media?parent=219"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.rhesusnegative.net\/staynegative\/wp-json\/wp\/v2\/categories?post=219"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.rhesusnegative.net\/staynegative\/wp-json\/wp\/v2\/tags?post=219"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}