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繰り返す輸血で壊血病/明日から遅い夏休み

・壊血病=ビタミンC欠乏症については以前もかきましたが、復習。『医学書院医学大辞典第2版』によると↓


ビタミンC欠乏症 vitamin C deficiency
[同義語]アスコルビン酸欠乏症 ascorbic acid deficiency,壊血病 scorbutus(scurvy)

ビタミンCの欠乏によって起こる病気で,15世紀から17世紀にかけて遠洋航海に行く乗組員の60%以上が壊血病で死亡した。1753年にイギリスのリンド(Lind J)が新鮮なオレンジとレモンで予防できることを示した。症状は歯齦〈しぎん〉炎,下肢,特に膝のすぐ上部に皮下出血がみられる。小児の場合は,上記の症状に骨病変が加わりメラー-バーロー病,小児壊血病と呼ばれている。治療はビタミンCを50~2000mg/日投与する。

上にはかかれていませんが、その他様々な症状が出るそうです。(実際観たことないので、伝聞形)↓のようなレポートがありました。


Old Era Continues in Modern World: A Case Report of Scurvy Induced Myopathy in Patient with Chronic Alcoholism

Girish Singhania, Namrata Singhania, and Neha Chawla


Case Report |Volume 2020 |Article ID 4798941

https://www.hindawi.com/journals/crim/2020/4798941/

Abstract

We report a case of myopathy in a chronic alcoholic patient with scurvy who presented with generalized weakness, myalgias, and arthralgia. Our case raises awareness regarding rare interaction between vitamin C deficiency and myopathy which is seen more commonly in patients with history of chronic alcoholism and low socioeconomic status. Early treatment with vitamin C replacement is helpful in treatment of the disease and its complications.

・ここで取り上げられているのは、アルコール依存症の患者さんです。わたしはアルコール依存ときくとついビタミンB1欠乏を考えてしまうのですが、ビタミンC欠乏もあたまにいれておかないといけませんね。

・今回学んだのは食事性のビタミンC欠乏のみでなく、繰り返す輸血のようなことで鉄過剰になってもビタミンC欠乏がおこるということです。本文に↓にように書かれていました。

It is also seen in patients with iron overload due to frequent blood transfusions in diseases like sickle cell disease or thalassemia, or a history of bone marrow transplantation [9].

上の引用文献[9]が↓

Pediatr Radiol. 2017 Feb;47(2):214-220.
doi: 10.1007/s00247-016-3726-4. Epub 2016 Oct 24.

Modern American scurvy - experience with vitamin C deficiency at a large children's hospital

F. Golriz,et al.

Abstract

Background: Until recently scurvy has been viewed in developed countries as a disease of the past. More recently there have been reports of case series of children with scurvy who have had a delayed diagnosis after an extensive diagnostic workup that included imaging. Most of these children have had underlying neurologic conditions such as autism.

Objective: To review the medical records of children diagnosed with vitamin C (ascorbic acid) deficiency based on serum ascorbic acid levels at a large pediatric health care system, to determine imaging findings and utility of imaging in management, and to identify at-risk pediatric populations.

Materials and methods: We retrospectively identified cases of vitamin C deficiency in children tested for serum ascorbic acid levels during the last 5 years. We used the criteria of normal ascorbic acid >23 μmol/L and included children with ascorbic acid levels <23 μmol/L. We evaluated their clinical history, underlying medical condition, imaging studies obtained and imaging findings.

Results: We identified 32 children with vitamin C deficiency. All of these children had underlying medical conditions, most commonly iron overload from multiple transfusions related to sickle cell anemia or thalassemia (20), neurologic disorders (4) and bone marrow transplant/chemotherapy (3). No cases of scurvy from dietary deficiency in otherwise normal children were identified. All except two children had multiple imaging studies, primarily related to their underlying conditions. Three of these children had extensive imaging workups related to diffuse musculoskeletal pain. Imaging findings included ill-defined sclerotic and lucent metaphyseal bands (mainly at the knee) on radiography and MRI studies that showed diffuse increased T2-weighted signal in the bilateral lower-extremity long-bone metaphyses, periosteal reaction and adjacent soft-tissue edema.

Conclusion: Vitamin C deficiency is not uncommon in large pediatric health care facilities, and it is frequently missed on clinical evaluation and diagnostic imaging. At-risk populations include those with iron overload, neurologic conditions and history of chemotherapy. Scurvy related to dietary deficiency in otherwise normal children was not encountered. When characteristic MRI findings are seen, particularly in children with a predisposing condition for vitamin C deficiency, scurvy should be considered and a serum ascorbic acid level checked to potentially confirm a diagnosis prior to further invasive tests.

↑の論文は残念ながら有料ですが、そのsiteからいろいろ引用文献がわかります。その一つが↓
Scurvy Revealed by Difficulty Walking: Three Cases in Young Children
Kitcharoensakkul, Maleewan, et al.
JCR: Journal of Clinical Rheumatology: June 2014, Vol. 20 Issue 4
doi: 10.1097/RHU.0000000000000101
【Abstract】

Scurvy is rare in developed countries but is known to cause lower-extremity pain and refusal to ambulate in children. Since the discovery of the link between scurvy and dietary deficiency of ascorbic acid, there has been a substantial decrease in its prevalence and recognition. Here we describe 3 cases of scurvy in young children presenting with difficulty walking. Only 1 of 3 patients had gingival lesions at the initial presentation. Two cases underwent an extensive evaluation for hematologic and rheumatologic diseases before the diagnosis of scurvy was made. Dietary histories eventually revealed that all 3 patients had sharply limited intake of fruits and vegetables secondary to oral aversion, and 1 patient had autism. Radiographic changes of long bones were observed in all patients. Interestingly, all patients had concomitant vitamin D deficiency. After replacement with vitamin C, all patients recovered and started to walk again with improved leg pain. These clinical manifestations and radiologic findings highlight the importance for rheumatologists to have a higher index of suspicion for scurvy in nonambulatory children.

・これまた有料ですが、抄録だけ見ても興味深いですね。

・今回そもそも最初の論文の焦点、筋肉の障害について論じませんでしたが、筋痛や筋力低下をみたときビタミンC欠乏症も考えないといけないですね。あとは、鉄過剰症でもビタミンCがおこるというのが本日の教訓です。

以下日記

・本日10/13(火)は、5時17分起床。6時15分に家を出て6時40分に病院について宿直の交代。早朝回診後午前外来でした。幸い大きな事無し。14時頃病院出てお金の支払いや買い物して15時過ぎかえったでしょうか。ちょっと事務的な作業して16時から17時までZOOMで大学院のCritical appraisal.その後隣の義父の畑の雑木を鋸でセッセときりました。そして入浴、夕食。アルコールは飲まずちょっと勉強してこのブログを書いております。あすから遅い「夏休み」。ここ数日ブログのアップはできないと思いますが、facebookやinstagramに写真をアップすると思いますので、お暇な方はご覧ください↓

https://www.facebook.com/tatsuya.michibata.9

 


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