Huke B
Z Orthop Ihre Grenzgeb. 1978;116(2):203-13.
A long-term study conducted in a female patient who is now 21 years old and who showed a disease pattern similar to cranio-carpo-tarsal dystrophia, investigated thoroughly by Liersch and Hoevels when the girl was 6 years old. It must be stated as a result of this long-term study that in our case, the osteolyses at the wristbone (carpalia) and tarsal bone remained progressive up to the onset of puberty. The radiological changes came to a standstill after puberty had set in; in fact, there was even a certain amount of clinical compensation of the radiologically visible defects during the years that followed. It seems that the disease pattern described here, is limited to the growth period of the patient. It was not possible to classify this disease pattern as belonging to a particular category, and its cause remains largely unknown. The author describes the disease pattern as an "essential" one.
一项针对一名现年21岁女性患者的长期研究,该患者表现出与颅腕跗骨发育不良相似的疾病模式,在女孩6岁时由利尔施和赫费尔斯进行了全面调查。这项长期研究的结果必须说明,在我们的病例中,腕骨(腕骨)和跗骨的骨质溶解在青春期开始前一直呈进行性发展。青春期开始后,放射学变化停止;事实上,在随后的几年里,放射学可见缺陷甚至有一定程度的临床代偿。这里描述的疾病模式似乎仅限于患者的生长期。不可能将这种疾病模式归类到特定类别,其病因在很大程度上仍然未知。作者将这种疾病模式描述为一种“原发性”模式。