Clinical Investigation and Experience with Distal phalanx Replantation

Objective： To explore the measures and methods for improving distal phalanx replantation. Methods： One hundred and forty-eight cases with distal phalanx replantation from Sep 2004 to Sep 2007 were investigated. The indication for surgery and the operative program was determined by the trauma type and the degree of injury. As many microcirculation pathways as possible were reconstructed during the operation, and postoperative tissue decompression was also performed. Results： The survival rate of distal phalanx replantation was 89.9% （169/148）. Conclusion： To improve the survival rate of distal phalanx replantation it is essential to reconstruct an effective local blood circulation, determine the degree of injury in cases with ecchymosis, and employ comprehensive postoperative measures.

A three-year clinical investigation of a Chinese child with craniometaphyseal dysplasia caused by a mutated ANKH gene

BACKGROUND Craniometaphyseal dysplasia(CMD)is a rare genetic disorder.Autosomal dominant CMD(AD-CMD)is caused by mutations in the ANKH gene.Affected individuals typically have distinctive facial features including progressive thickening of the craniofacial bones.Treatment for AD-CMD primarily consists of surgical intervention to release compression of the cranial nerves and the brain stem/spinal cord.To alleviate progression of the clinical course and improve the quality of life in children waiting to undergo the necessary surgery,we investigated clinical changes in a diagnosed patient with AD-CMD over three years.CASE SUMMARY A 17-mo-old boy presented with progressive nasal obstruction,snoring and hearing loss symptoms.Physical examination showed enlargement of the head circumference and clinical features such as wide nasal bridge,paranasal bossing,widely spaced eyes with an increased bizygomatic width,and a prominent mandible.The patient underwent otolaryngological examination,endoscopy,hearing test,laboratory examination of phosphorus and bone metabolism,cranial and femoral computed tomography,X-ray and next-generation sequencing.The patient was diagnosed with AD-CMD due to p.Phe377 deletion(c.1129_1131del)on exon 9 of the ANKH gene.After adherence to a prescribed low-calcium diet,the boy’s alkaline phosphatase(ALP)levels continuously decreased to within the normal range.However,after 14 mo of dietary intervention,his parents altered his diet to an intermittent low-calcium diet to include milk and eggs.The patient’s ALP was slightly higher than normal after the dietary change but remained close to the normal range.His serum osteocalcin changed to within normal levels after dietary regulation for 33 mo.His serum combined beta C-terminal telopeptide of type I collagen also continuously decreased after the nutritional intervention,although still slightly higher than normal levels.Despite fluctuating blood test results,the boy’s nasal symptoms were markedly relieved and steadily improved after dietary intervention.No significant changes were found in the craniofacial bones by cranial radiography.Close monitoring of clinical features is still ongoing.Calcitriol treatment is currently under consideration and a surgical procedure is planned as necessary in the future.CONCLUSION We herein report the first Chinese case of AD-CMD with heterozygous mutation of p.Phe377 deletion(c.1129_1131del)on the ANKH gene.Biochemical alterations were significantly improved after dietary intervention indicating that a lowcalcium diet may be applied in pediatric AD-CMD patients with ANKH mutations to help alleviate phenotypic manifestations and improve the quality of life before surgical intervention.Further large scale studies are needed to replicate these findings and to establish the appropriate timing for nutritional and surgical interventions。

Clinical Analysis of 84 Cases of Erythrodermic Psoriasis and 121 Cases of Other Types of Erythroderma from 2010–2015

Erythroderma with complicated etiology is one of the severe skin diseases and has high mortality, of which the incidence was 0.5‰–1.5‰ in skin diseases. Erythrodermic psoriasis（EP） is the commonest type of erythroderma. In addition, there are drug-induced erythroderma, erythroderma secondary to preexisting dermatoses, malignancy-related erythroderma, and idiopathic erythroderma of unknown etiology. Erythroderma of different etiologies has various clinical manifestations, resulting in relevant curative effects and outcomes. In this article, we retrospectively investigated 205 erythroderma patients about clinical symptoms, auxiliary examination and treatments, and evaluated the efficacy and prognosis. There were 84 cases of EP among 205 patients, 10 cases of erythroderma caused by specific drugs, 77 cases of erythroderma secondary to preexisting dermatoses（excluding psoriasis）, 7 cases of erythroderma patients suffering from malignancy and 27 cases with unknown causes. We concluded that the etiology of male patients in different age groups had significant difference. The incidence of EP was the highest among all types. The EP was commonly accompanied with hypoproteinemia, and changed into psoriasis vulgaris after treatment. Drug-induced erythroderma was commonly accompanied with fever, and mostly cured by systematic steroid therapy. For erythroderma secondary to preexisting dermatoses, the original dermatoses must be actively treated to achieve a satisfying prognosis. Erythroderma with malignancy or unknown causes had long-term duration, poor response to the treatment, and high potential to relapse. Therefore, clarifying the etiology, providing an appropiate and individual regimen, and regular follow-up are crucial for the successful treatment of erythroderma with unknown causes.