[{"Title":"病態生理","UniqueID":"v970288_ja","Index":1.0,"Summary":"

ヘモグロビン分子はポリペプチド鎖で構成され，ポリペプチド鎖の化学構造は遺伝的に制御されている。正常な成人型ヘモグロビン分子（ヘモグロビンA）は，α鎖およびβ鎖と命名された2組の分子鎖から構成される。正常な成人の血液には，2.5％以下の割合でヘモグロビンA2（α鎖およびδ鎖から成る）も，また1.4％未満の割合でヘモグロビンF（胎児型ヘモグロビン，β鎖の部位をγ鎖が占めている）も含まれている（妊娠中の異常ヘモグロビン症妊娠中の異常ヘモグロビン症正常では，妊娠中には骨髄の赤芽球過形成が起こり，赤血球量は増加する。しかしながら，血漿量の不均衡な増加により血液希釈（妊娠水血症）が生じる：ヘマトクリット（Hct）は妊娠していない健康な女性の場合38～45％であるが，単胎妊娠後期では約34％，多胎妊娠後期では30％に減少する。以下のヘモグロビン（Hb）値およびHct値は貧血として分類され... さらに読むも参照）。在胎中はヘモグロビンFが多数を占め，出生後徐々に減少していき，特に生後数カ月で大きく減少するが，特定のヘモグロビン合成障害や再生不良性貧血再生不良性貧血 $\"再生不良性貧血\"$ ，その他の骨髄不全疾患，および骨髄増殖性腫瘍骨髄増殖性腫瘍の概要では，その濃度が上昇する。

","HeadType":"F_Head","SchemeItemProp":"","Children":[{"Title":"急性増悪","UniqueID":"v28580787_ja","Index":1.0,"Summary":"

","HeadType":"G_Head","SchemeItemProp":"","Children":[]},{"Title":"合併症","UniqueID":"v28580804_ja","Index":2.0,"Summary":"

","HeadType":"G_Head","SchemeItemProp":"","Children":[]},{"Title":"ヘテロ接合体","UniqueID":"v28580810_ja","Index":3.0,"Summary":"

","HeadType":"G_Head","SchemeItemProp":"","Children":[]}]},{"Title":"症状と徴候","UniqueID":"v970305_ja","Index":2.0,"Summary":"

大半の症状は，ホモ接合体の患者のみにみられ，以下に起因して生じる：

","HeadType":"F_Head","SchemeItemProp":"","Children":[]},{"Title":"診断","UniqueID":"v970320_ja","Index":3.0,"Summary":"

DNA検査（出生前診断）

","HeadType":"F_Head","SchemeItemProp":"","Children":[{"Title":"出生前スクリーニング","UniqueID":"v970333_ja","Index":1.0,"Summary":"

DNA検査（出生前診断）

","HeadType":"G_Head","SchemeItemProp":"","Children":[]},{"Title":"新生児スクリーニング","UniqueID":"v970336_ja","Index":2.0,"Summary":"

DNA検査（出生前診断）

","HeadType":"G_Head","SchemeItemProp":"","Children":[]},{"Title":"小児および成人のスクリーニングと診断","UniqueID":"v970339_ja","Index":3.0,"Summary":"

DNA検査（出生前診断）

","HeadType":"G_Head","SchemeItemProp":"","Children":[]},{"Title":"増悪の評価","UniqueID":"v970350_ja","Index":4.0,"Summary":"

DNA検査（出生前診断）

","HeadType":"G_Head","SchemeItemProp":"","Children":[]}]},{"Title":"予後","UniqueID":"v970356_ja","Index":4.0,"Summary":"

ホモ接合体患者の寿命は着実に延びており，50歳を超えている。一般的な死因は，急性胸部症候群，併発感染症，肺塞栓症，重要臓器の梗塞，肺高血圧症，および慢性腎臓病である。

","HeadType":"F_Head","SchemeItemProp":"","Children":[]},{"Title":"治療","UniqueID":"v970359_ja","Index":5.0,"Summary":"

広域抗菌薬（感染症に対して）

","HeadType":"F_Head","SchemeItemProp":"itemprop=\"possibleTreatment\" itemscope itemtype=\"http://schema.org/MedicalTherapy\"","Children":[{"Title":"抗菌薬","UniqueID":"v970374_ja","Index":1.0,"Summary":"

広域抗菌薬（感染症に対して）

","HeadType":"G_Head","SchemeItemProp":"itemprop=\"possibleTreatment\" itemscope itemtype=\"http://schema.org/MedicalTherapy\"","Children":[]},{"Title":"鎮痛薬","UniqueID":"v970377_ja","Index":2.0,"Summary":"

広域抗菌薬（感染症に対して）

","HeadType":"G_Head","SchemeItemProp":"itemprop=\"possibleTreatment\" itemscope itemtype=\"http://schema.org/MedicalTherapy\"","Children":[]},{"Title":"輸液","UniqueID":"v28580827_ja","Index":3.0,"Summary":"

広域抗菌薬（感染症に対して）

","HeadType":"G_Head","SchemeItemProp":"itemprop=\"possibleTreatment\" itemscope itemtype=\"http://schema.org/MedicalTherapy\"","Children":[]},{"Title":"酸素","UniqueID":"v67165677_ja","Index":4.0,"Summary":"

広域抗菌薬（感染症に対して）

","HeadType":"G_Head","SchemeItemProp":"itemprop=\"possibleTreatment\" itemscope itemtype=\"http://schema.org/MedicalTherapy\"","Children":[]},{"Title":"輸血","UniqueID":"v970380_ja","Index":5.0,"Summary":"

広域抗菌薬（感染症に対して）

","HeadType":"G_Head","SchemeItemProp":"itemprop=\"possibleTreatment\" itemscope itemtype=\"http://schema.org/MedicalTherapy\"","Children":[]},{"Title":"根治的治療","UniqueID":"v49749683_ja","Index":6.0,"Summary":"

広域抗菌薬（感染症に対して）

","HeadType":"G_Head","SchemeItemProp":"itemprop=\"possibleTreatment\" itemscope itemtype=\"http://schema.org/MedicalTherapy\"","Children":[]},{"Title":"健康維持","UniqueID":"v970385_ja","Index":7.0,"Summary":"

広域抗菌薬（感染症に対して）

","HeadType":"G_Head","SchemeItemProp":"itemprop=\"possibleTreatment\" itemscope itemtype=\"http://schema.org/MedicalTherapy\"","Children":[]},{"Title":"治療に関する参考文献","UniqueID":"v67165683_ja","Index":8.0,"Summary":"

広域抗菌薬（感染症に対して）

","HeadType":"G_Head","SchemeItemProp":"itemprop=\"possibleTreatment\" itemscope itemtype=\"http://schema.org/MedicalTherapy\"","Children":[]}]},{"Title":"要点","UniqueID":"v9017663_ja","Index":6.0,"Summary":"

ヘモグロビンSのホモ接合体の患者は異常β鎖を有するため，赤血球が脆弱で相対的に変形しにくいことから，毛細血管を詰まらせて組織の梗塞を引き起こすことがあるほか，溶血を生じて貧血を起こしやすい。

","HeadType":"F_Head","SchemeItemProp":"","Children":[]},{"Title":"より詳細な情報","UniqueID":"v49749689_ja","Index":7.0,"Summary":"

有用となりうる英語の資料を以下に示す。ただし，本マニュアルはこの資料の内容について責任を負わないことに留意されたい。

","HeadType":"F_Head","SchemeItemProp":"","Children":[]}]