Biomedical subjects

Hong-yan Bi

Publications and source records attributed to Hong-yan Bi.

3 recordsLinked to original sources

PubMed2006

[Immunopathological changes of micro-vessels in dermatomyositis].

OBJECTIVE: To investigate the immunopathological changes of micro-vessels in dermatomyositis. METHODS: Twelve patients with dermatomyositis, 5 males and 7 females, aged 40.8 (6 - 72), underwent muscle biopsy of biceps muscle of arm (n = 11) or deltoid muscle (n = 1). The specimens underwent routine histological, enzyme histochemical, and immunohistochemical staining and microscopy. Ten biopsy specimens from patients with other diseases were used as controls. RESULTS: Perifascicular atrophy of muscular fibers and inflammatory infiltration in perimysium were seen in all patients with dermatomyositis. Non-specific esterase staining showed deep staining of capillaries and micro vascular endothelium among the muscular fibers. Immunohistochemistry showed remarkable reduction of capillaries positive in von Willebrand factor (vWF), thrombomodulin (TM), and endothelial cell nitric oxide synthase (eNOS) in the perifascicular region, and low expression of .eNOS and TM in the microvascular endothelium in the perimysium. CONCLUSION: The vascular lesions of dermatomyositis are located not only in capillaries, but also in other microvessels. Lower expression of TM and eNOS in vascular endothelium suggests the reduction of anticoagulation and vasodilation functions of vascular endothelium. Dermatomyositis is an inflammatory vascular endothelial disease.

Adolescent↗

PubMed2006

[Two novel mutations in palmitoyl-protein thioesterase gene in two Chinese babies with infantile neuronal ceroid lipofuscinosis].

OBJECTIVE: To search for possible novel mutations in palmitoyl-protein thioesterase 1 (PPT1) gene in two Chinese babies with infantile neuronal ceroid lipofuscinosis (INCL). METHODS: Two probands with INCL, confirmed clinically and pathologically, were used for mutation search in PPT1 gene. Onset of the disease occurred before the age of 1 year and they mainly showed progressive mental and motor retardation. The 9 coding exons and their flanking intron sequences of palmitoyl-protein thioesterase 1 (PPT1) gene were amplified by using PCR and sequenced. The parents of proband 1 were also examined. RESULTS: One splicing mutation and two missense mutations were identified in the two probands: the proband 1 carrying a compound heterozygous mutation of a IVS1 + 1G-->A mutation in intron 1 and a c550G-->A mutation in exon 6 leading to the amino acid substitution of E184K. Additionally, the parents of the proband 1 also harbored one of the mutations of the patient, respectively. The proband 2 carrying a homozygous mutation of c272A-->C in exon 3, which resulted in the amino acid substitutions of Q91P. CONCLUSIONS: The IVS1 + 1G-->A mutation and Q91P mutation are novel mutations, which lead to INCL. The genetic abnormalities of PPT1 in Chinese patients may not be completely the same as those in the patients of other regions of the world.

Age of Onset↗

PubMed2005

[Neurolymphomatosis, a case report].

Neurolymphomatosis (NL) is characterized by lymphomatous infiltration of the peripheral nervous system. We report a case of neurolymphomatosis (NL) which was confirmed by sural nerve biopsy. Sural nerve specimen from a 49-year-old female patient with weakness of limbs was examined with routine histochemical and immunohistochemistry staining, in which the first antibodies against CD3, CD20, CD45, CD45RO and CD68 were used. Numerous T-lymphoma cells invaded in the adipose tissue of epineurium of sural nerve. The nerve biopsy showed marked axonal degeneration of myelinated fibers. The clinical and histopathologic findings confirmed the diagnosis of neurolymphomatosis.

Female↗