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A possible case of Langerhans-cell histiocytosis? Differential diagnosis in a rare case from the Late Antiquity Bavaria (Germany)
International Journal of Paleopathology ( IF 1.2 ) Pub Date : 2021-05-26 , DOI: 10.1016/j.ijpp.2021.05.005
Michael Schultz 1 , Martina Weber 2 , Tyede H Schmidt-Schultz 2 , Juliane Dorn 3 , Albert Zink 4 , Andreas Nerlich 5
Affiliation  

Objective

To outline the importance of accurate diagnosis in ancient rare diseases by presenting a possible case of Langerhans-cell histiocytosis.

Materials

Skeletal elements from a well-preserved skeleton of a nine to eleven-year-old, probably female child who lived around 300–400 AD Late Roman Neuburg / Donau (Germany).

Methods

Macroscopic, radiologic, light and scanning-electron microscopic and physical techniques were used.

Results

Resorptive defects, particularly in the cranium, but also in the left hip bone and the right femur, suggest the presence of Langerhans-cell histiocytosis macroscopically and radiologically. The presence of morphological changes along the edges of osteolytic lesions and in the diploic spaces appear to be post-mortem artifacts based on microscopic investigation and elemental analysis.

Conclusions

Re-evaluation of morphological structures and elemental constitution of lesions is critical to differential diagnosis. In the case examined here, the identification of post-mortem structures rules out the former diagnosis of Langerhans-cell histiocytosis. Re-evaluation of cases of rare diseases require applying a range of methods during the analysis, as every single case makes a difference in the numbers of this very small group of diseases.

Significance

This study emphasizes the importance of utilizing different analytical techniques to avoid false diagnoses.

Limitations

Not all morphological features can reliably be diagnosed using microscopic and elemental techniques.

Suggestions for Further Research

In the case of rare diseases that are difficult to diagnose, the widest possible spectrum of techniques should always be used, particularly microscopy.



中文翻译:

朗格汉斯细胞组织细胞增生症的可能病例?古代巴伐利亚晚期(德国)罕见病例的鉴别诊断

客观的

通过介绍朗格汉斯细胞组织细胞增生症的一个可能病例,概述准确诊断古代罕见病的重要性。

材料

一具保存完好的 9 至 11 岁儿童的骨骼元素,可能是生活在公元 300 至 400 年左右的女童,晚期罗马诺伊堡/多瑙(德国)。

方法

使用了宏观、放射、光和扫描电子显微镜和物理技术。

结果

吸收缺陷,特别是在颅骨,但也在左髋骨和右股骨,在肉眼和放射学上表明存在朗格汉斯细胞组织细胞增生症。沿溶骨性病变边缘和双倍空间的形态学变化似乎是基于显微镜调查和元素分析的验尸伪影。

结论

重新评估病变的形态结构和基本构成对于鉴别诊断至关重要。在这里检查的病例中,验尸结构的鉴定排除了先前诊断的朗格汉斯细胞组织细胞增生症。对罕见疾病病例的重新评估需要在分析过程中应用一系列方法,因为每个病例都会对这一极少数疾病的数量产生影响。

意义

这项研究强调了利用不同的分析技术来避免错误诊断的重要性。

限制

并非所有形态特征都可以使用显微和元素技术进行可靠诊断。

进一步研究的建议

对于难以诊断的罕见疾病,应始终使用尽可能广泛的技术,尤其是显微镜检查。

更新日期:2021-05-26
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