Diagnostic performance of anti-citrullinated protein/peptide antibodies in juvenile idiopathic arthritis.

The prevalence rates of anti-citrullinated protein/peptide antibodies (ACPAs) were investigated in a cohort of juvenile idiopathic arthritis (JIA) patients, and their diagnostic performances were compared. ACPAs, including anti-cyclic citrullinated peptide IgG (anti-CCP), anti-CCP IgG/IgA (anti-CCP3.1), citrullinated recombinant rat filaggrin antibodies (CPA), anti-mutated citrullinated vimentin (anti-MCV), and antibodies to citrullinated human IgG-derived peptides (RA/CP), were measured in the sera from 81 JIA patients. Serum samples from 55 children with other joint diseases or viral infections and 49 healthy donors were tested as controls. Of the 81 JIA patients, 7 (8.6%), 8 (9.9%), 17 (21.0%), 23 (28.4%), and 18 (22.2%) were found to be positive for anti-CCP, anti-CCP3.1, CPA, anti-MCV, and RA/CP, respectively, with specificities of 98.1, 95.1, 93.3, 84.6, and 86.5%. Analysis by subtype revealed that 7/7 (100%) of RF-positive polyarticular JIA patients tested positive at high serum levels for anti-MCV or RA/CP, and 5/7 (71.4%) were positive for anti-CCP, anti- CCP3.1, or CPA (P < 0.001, compared with controls). Eighteen of 81 JIA patients demonstrated joint erosions on radiographs and erosive arthritis occurred more often in ACPAs positive patients (P < 0.01). Our findings indicate that although ACPAs are not satisfactory screening biomarkers for JIA due to low sensitivity, ACPA measurement can aid in diagnosing RF-positive polyarticular JIA and identifying JIA patients with severe bone involvement. The diagnostic performance of each ACPA in JIA is different, and the careful selection of assays is necessary.

Whole exome sequencing identifies a novel mutation in the transglutaminase 6 gene for spinocerebellar ataxia in a Chinese family.

Autosomal dominant spinocerebellar ataxias (SCA) constitute a heterogeneous group of inherited disorders. The transglutaminase 6 (TGM6) gene was recently suggested as a SCA causative gene in Chinese SCA families. In this study, two affected members of a three-generation Chinese family with SCA characterized by progressive cerebellar ataxia and lower limb pyramidal signs were subjected to whole exome sequencing. Through bioinformatics analysis of the sequence variants in these two individuals, we identified a novel mutation in the TGM6 gene (c.1528G>C) which showed perfect co-segregation with disease phenotype in all nine members of this family. This finding confirms that mutations in TGM6 gene represent an important cause of SCA in Chinese. This study also shows that whole exome sequencing of a small number of affected individuals, leveraged on bioinformatics analysis, can be an efficient strategy for identifying causative mutations in rare Mendelian disorders.

Composition and sources of aliphatic lipids and sterols in sediments of a tropical island, southern South China Sea: preliminary assessment.

Near-shore surface sediment was collected from five stations off Redang Island located on the eastern coast of Peninsular Malaysia. Freeze-dried sediments were Soxhlet extracted and then fractionated using column chromatography into aliphatic and polar fractions. Determination of these fractions was carried out using gas chromatography mass spectrometry. The concentration of total resolved aliphatic hydrocarbons in sediments ranged from 157 to 308 ng/g. The distribution of aliphatic fraction showed the presence of n-alkanes ranging from nC15 to nC33 with a minor odd-to-even predominance exhibiting carbon maximum, depending on station, at nC17, nC26, nC29 or nC31. Calculation of Carbon Preference Index (CPI) for CPI(15-33) gave values ranging from 1.09 to 1.46. n-Alkanol in all sediment exhibits even-to-odd carbon predominance ranging from nC16 to nC28 and maximising at nC22. n-Fatty acids distribution ranged from nC14 to nC24 with a dominant maximum at nC16 and exhibiting high values of short chain fatty acids (≤nC20) to long chain fatty acids (>nC20) ratios. Unsaturated fatty acids, particularly nC16:1 and nC18:1 is also ubiquitous in all samples. Cholesterol is the most abundant compound amongst the sterol group ranging from 42.8 to 62.6% of the total sterols. ß-Sitosterol, brassicasterol and stigmasterol, are also present but of relatively lower amount. These observations suggest that the aliphatic lipids and sterols in the study area originate, mainly, from biogenic sources of marine microbial with minor contribution from epiticular waxes of terrestrial plants.

[Clinical analysis of laryngeal non-Hodgkin's lymphoma].

Objective:To analyze the clinical characteristics, pathology and treatment of laryngeal non-Hodgkin's lymphoma.Method:Clinical information of 31 patients with laryngeal non-Hodgkin's lymphoma treated in our hospital was reviewed retrospectively. Result:T cell lymphoma, B cell lymphoma, NK/T cell lymphoma, the mixed pattern and unspecified lymphoma accounted for 16.1%(5/31),32.3%(10/31),35.5%(11/31)and 16.1%(5/31) of the patients, respectively.Twenty-seven cases were located in supraglottic region, 1 case was located in glottic region, and 3 cases were located in glottic and subglottic region. Conclusion:The laryngeal non-Hodgkin's lymphoma was a rare malignant neoplasm and the most localized in the supraglottic region.Its diagnosis depended on comprehensive immunohistochemistry examination.Chemotherapy and radiotherapy were the main therapeutic methods.

Distribution and sources of lipid compound series in sediment cores of the southern South China Sea.

Three short sediment cores from inner continental shelf of the southern South China Sea (5-50 km) off Terengganu were analyzed for lipid contents (i.e., homologous aliphatic compounds and sterols) using gas chromatography-mass spectrometry. The concentrations of the total aliphatic hydrocarbons (TAHs) ranged from 0.152 to 6.91 µg/g dry weight. The n-alkane distribution was from nC13 to nC36, with a carbon preference index (CPI13₋35) from 1.08 to 4.28 and a carbon number maximum (Cmax), depending on a sample, at 31 or 18. In addition, a strong odd-to-even carbon number predominance was observed in nC25-nC35 range. The distribution of the n-alkanoic acids and n-alkanols in all samples exhibited an even-to-odd carbon number predominance and ranged from C10 to C26 and from C12 to C34, respectively. The n-alkanols were dominated by the long-chain homologs with Cmax at 22; on the other hand, the n-alkanoic acid distributions showed a predominance of short-chain homologs with a Cmax at 16. The total sterol concentrations ranged from 0.41 to 3.57 µg/g dry weight. Cholesterol was most abundant at the offshore stations, whereas sitosterol was dominant at near-shore station. Pentacyclic triterpenoids such as friedelin and taraxerol α- and ß-amyrins, which are known biomarkers for higher plants, were detected at all stations with a dilution trend offshore. In conclusion, the marine sediments off southern Terengganu can still be considered uncontaminated, where the compound sources are biogenic from terrestrial plants superimposed with a marine productivity input.

Clinical outcome of generalized myasthenia gravis in Hong Kong Chinese.

BACKGROUND: Myasthenia gravis (MG) is an organ-specific autoimmune disease characterized by autoantibody-mediated impairment of skeletal muscle neuromuscular transmission. MG causes significant morbidity and even mortality. We studied the long-term clinical outcome of generalized MG (gMG) patients. METHODS: Records of Chinese gMG patients managed in Queen Mary Hospital from 1997 to 2012 were reviewed. Clinical, serological and radiological characteristics were studied for independent predictors of good long-term clinical outcome. RESULTS: A total of 123 Chinese gMG patients were studied. Their mean onset age was 44.8 years (range 7-83 years), 87 (70.7%) were female, and median follow-up duration was 114 months (interquartile range 67-188 months). Thymoma were detected in 45 patients (36.6%). Acetylcholine receptor autoantibodies were detected in 99 patients (87.6%). Ninety-three patients (75.6%) received immunosuppressant therapy (corticosteroid 75.6%, azathioprine 58.5%, mycophenolate mofetil 5.7%, cyclosporin 5.7%) and 77 (62.6%) received thymectomy. Thirty-five (28.5%) patients experienced MG crisis and two died. Ninety-six (78.0%) patients had good outcome defined by Myasthenia Gravis Foundation of America (MGFA) post-intervention status (PIS) of complete stable remission (CSR), pharmacological remission (PR) or minimal manifestation (MM) at latest follow-up, whereas 24 patients (19.5%) had intermediate outcome defined by MGFA PIS of Improved (I); 3 patients (2.4%) had poor prognosis defined by MGFA PIS of unchanged (U), worse (W), exacerbation (E) or died of MG (D). Azathioprine therapy was the only independent predictor of good outcome (OR 3.57, 95% CI 1.05-12.10, p=0.042). CONCLUSION: 78.0% of gMG patients had good long-term clinical outcome. Azathioprine therapy independently predicted good clinical outcome.

Transient expression of mouse hair keratins in transfected HeLa cells: interactions between "hard" and "soft" keratins.

Although it has been shown previously that an acidic (type I) "soft" keratin can interact with many basic (type II) "soft" keratins to form 10-nm intermediate filaments, it has been unclear whether "soft" keratins are compatible with the "hard" keratins typically found in hair and nail. To address this issue and to generate more structural information about hard keratins, we have isolated and sequenced a cDNA clone that encodes a mouse hair basic keratin (b4). Our sequence data revealed new information regarding the structural conservation of hard keratins as a group, being significantly different from soft keratins. Using expression vectors containing appropriate cDNA inserts, we studied the expression of this basic (b4) as well as an acidic (a1) mouse hair keratin in HeLa cells. The expression of these alien hair keratins in the transfected cells was surveyed using a panel of monoclonal and polyclonal antibodies. Our results indicated that the basic and acidic hair keratin readily incorporated into the existing endogenous soft keratin network of HeLa cells. Overproduction of hair keratin, however, occasionally led to the formation of cytoplasmic aggregates containing both hard and soft keratins. These data suggest that although small amounts of newly synthesized hair keratins can incorporate into the "scaffolding" of the preformed soft keratin filament network, possibly through dynamic subunit exchange, overproduction of hard keratins can lead to the partial collapse of the soft keratin network. These observations, along with the deduced amino acid sequence data, support and extend the concept that hard and soft keratins, although closely related, are divergent enough to justify their being divided into two separate subgroups.

Morning salivary 17-hydroxyprogesterone is a useful screening test for nonclassical 21-hydroxylase deficiency.

A screening test for nonclassical 21-hydroxylase deficiency (NC 21-OHD) has been established based on early morning salivary 17-hydroxyprogesterone (17-OHP) measurements. Saliva and serum samples were collected simultaneously between 0700 and 1100 h from 57 normal subjects (37 women and 20 men) and from 15 untreated patients (6 women and 9 men) with NC 21-OHD. The salivary (mean +/- SD, 524 +/- 508 pg/mL) and serum (10,548 +/- 5,998 pg/mL) 17-OHP concentrations in all 15 patients were unequivocally higher than the levels in normal subjects (saliva, 51 +/- 24 pg/mL; serum, 1,564 +/- 787 pg/mL). Salivary 17-OHP levels in patients with NC 21-OHD were significantly higher at 0700-0900 h (828 +/- 653 pg/mL) than at 0900-1100 h (314 +/- 227 pg/mL), while no such change was found in normal subjects. The salivary 17-OHP concentration was 1.3-6.9% of its serum concentration, and there was an excellent correlation (r = 0.93) between salivary and serum concentration in both normal subjects and patients with NC 21-OHD. In conclusion, early morning salivary 17-OHP measurement is an excellent screening test for the diagnosis of NC 21-OHD, since it accurately reflects serum 17-OHP levels, and sample collection is easy and noninvasive. We propose that salivary 17-OHP determination be used in population screening programs for NC 21-OHD to establish the true frequency of this disease.

Genetic defects of steroidogenesis in premature pubarche.

Twenty three patients (19 girls, 4 boys) presented with typical features of premature pubarche between the ages of 2-7 yr. The patients were studied for the presence of an adrenal steroidogenic defect by ACTH stimulation testing (Cortrosyn, 0.25 mg iv bolus dose). Based on published nomogram standards for serum 17-hydroxyprogesterone (17-OHP), seven patients (30%) were diagnosed as having the nonclassical symptomatic form of 21-hydroxylase deficiency [mean post ACTH 4244 +/- 1113 (SD) ng/dl]. Three patients (13%) were diagnosed to have a mild form of 3 beta-hydroxysteroid dehydrogenase deficiency based upon the response of serum delta 5-17-hydroxypregnenolone (delta 5-17P) and dehydroepiandrosterone, and the ratio of delta 5-17P/17-OHP to ACTH stimulation (delta 5-17P: 1543 +/- 272 ng/dl vs. Tanner stage I control subjects, 350 +/- 197 ng/dl; dehydroepiandrosterone: 675 +/- 190 ng/dl vs. Tanner stage I control subjects, 82 +/- 79 ng/dl; delta 5-17P/17-OHP: 8.1 +/- 2.6 vs. Tanner stage I control subjects, 1.4 +/- 0.6). No enzyme defect could be identified in the remaining 13 patients (57%). Eleven patients with premature pubarche, with and without an adrenal enzymatic defect, underwent dexamethasone suppression. In all patients the measured steroid levels were suppressed. Thus, the dexamethasone suppression test alone did not distinguish the pathogenesis of premature pubarche. In conclusion, premature pubarche is more commonly due to a partial enzyme defect in adrenal steroidogenesis than has been previously recognized.

Adrenal androgen response to metyrapone, adrenocorticotropin, and corticotropin-releasing hormone stimulation in children with hypopituitarism.

We determined the adrenal steroid responses to metyrapone, ACTH, and CRH in 12 ACTH-intact and 5 ACTH-deficient hypopituitary children to determine the mechanisms that control adrenal androgen secretion. Serum adrenal androgen concentrations [dehydroepiandrosterone (DHEA) and delta 4-androstenedione (delta 4-A)] rose in response to oral administration of metyrapone (450 mg/m2 X dose, every h for 7 doses) in ACTH-intact hypopituitary children with multiple or isolated pituitary hormone deficiencies [mean postmaryrapone level: DHEA, 225 ng/dL (range, 27-566); delta 4-A, 313 ng/dL (range, 105-651)], except in 2 young children in whom DHEA did not rise. These adrenal androgens did not rise in all ACTH-deficient hypopituitary children [mean postmetyrapone level: DHEA, 11.0 ng/dL (range, 3-16); delta 4-A, 6.2 ng/dL (range, 3-10)]. The increases in both serum cortisol and adrenal androgens, including DHEA sulfate, in response to short term ACTH infusion (40 U in 6 h) in ACTH-intact hypopituitary children were normal or above normal, while these steroid responses were significantly (P less than 0.05-0.01) lower in ACTH-deficient hypopituitary children compared to normal values. However, prolonged administration of ACTH (40 U/day, or im) for 6 days to 2 ACTH-deficient hypopituitary children resulted in normal DHEA responses to the 6-h ACTH stimulation test (DHEA levels after the first test, 14 and 30 ng/dL, after priming, 80 and 50 ng/dL). Furthermore, CRH administration to 4 ACTH-deficient patients caused a rise in serum DHEA and cortisol in patients with a normal ACTH response, while those with a poor ACTH response had a lesser rise in DHEA and cortisol. These data suggest that ACTH is the major tropic hormone for adrenal androgen secretion.

Late-onset adrenal steroid 3 beta-hydroxysteroid dehydrogenase deficiency. I. A cause of hirsutism in pubertal and postpubertal women.

To investigate the adrenal cause of hyperandrogenism in peri- and postpubertal hirsute women, baseline and ACTH-stimulated serum concentrations of delta 5-17-hydroxypregnenolone (delta 5-17P), dehydroepiandrosterone (DHEA) and its sulfate, 17-hydroxyprogesterone (17-OHP), cortisol, delta 4-androstenedione, and testosterone were determined in 116 women with hirsutism or acne of peri- and postpubertal onset with or without menstrual abnormalities. The results were compared with the same steroid concentrations in 30 normal age-matched women. Sixteen of the 116 women with hirsutism whose ACTH-stimulated 17-OHP levels (mean +/- SD, 5404 +/- 3234 ng/dl; normal, 334 +/- 194) were markedly elevated while their ratios of delta 5-17P to 17-OHP (0.4 +/- 0.2; normal, 3.4 +/- 1.5) were low were diagnosed as having nonclassical symptomatic 21-hydroxylase deficiency. Seventeen other hirsute women, including 3 siblings, had very high responses of delta 5-17P (2276 +/- 669 ng/dl; normal, 985 +/- 327) and DHEA (2787 +/- 386 ng/dl; normal, 1050 +/- 384) to ACTH stimulation, with significantly elevated ratios of delta 5-17P to 17-OHP (11 +/- 2.0; normal, 3.4 +/- 1.5) and DHEA to delta 4-androstenedione (7.5 +/- 2.3; normal, 4.6 +/- 1.5). In these hirsute women, the morning serum delta 5-17P and DHEA concentrations were elevated, had a diurnal variation, and were suppressed with dexamethasone administration. We propose that partial adrenal 3 beta-hydroxysteroid dehydrogenase deficiency is the cause of hirsutism in these women. This may represent an allelic variant at the genetic locus for 3 beta-hydroxysteroid dehydrogenase deficiency similar to that reported for symptomatic nonclassical 21-hydroxylase deficiency producing peripubertal excess androgen syndrome.

3 alpha-Androstanediol glucuronide in premature and normal pubarche.

To investigate the role of adrenal androgens in 3 alpha-androstanediol glucuronide (3AG) production in childhood, we compared serum 3AG and androgen levels [dehydroepiandrosterone (DHEA), DHEA sulfate (DS), androstenedione (delta 4-A), and testosterone (T)] in 32 children with premature pubarche due to idiopathic premature adrenarche (IPA; n = 26), partial 21-hydroxylase deficiency (n = 2), or 3 beta-hydroxysteroid dehydrogenase deficiency (n = 4) with those in 36 normal prepubertal (18 males and 18 females) and 22 normal pubertal Tanner II-III subjects (10 males and 12 females). Serum 3AG (2.7 +/- 2.0 nmol/L) and all androgen concentrations in children with IPA were significantly higher (P less than 0.05-0.001) than those in normal prepubertal children (3AG, 0.8 +/- 0.5 nmol/L). Serum 3AG and androgen levels, except T, in all children with premature pubarche due to 21-hydroxylase deficiency or 3 beta-hydroxysteroid dehydrogenase deficiency were higher than those in the normal prepubertal children. Serum 3AG and all androgen levels in normal Tanner II-III male (3AG, 3.8 +/- 1.7 nmol/L) or female (3AG, 1.74 +/- 0.52 nmol/L) subjects were also significantly higher (P less than 0.05-0.001) than those in prepubertal children. Serum 3AG, DHEA, DS, and delta 4-A levels in children with IPA were similar to those in normal Tanner II-III females or males, but serum T in children with IPA (0.37 +/- 0.2 nmol/L) was significantly lower (P less than 0.05-0.001) than that in normal pubertal females (0.71 +/- 0.37 nmol/L) or males (4.5 +/- 2.6 nmol/L). In the combined group (n = 88), 3AG levels correlated better with serum DS (r = 0.7), DHEA (r = 0.6), and delta 4-A (r = 0.52), than with T (r = 0.31) levels. These data suggest that the weak adrenal androgens DS, DHEA, and delta 4-A contribute substantially to 3AG production in premature and normal pubarche.

Worldwide experience in newborn screening for classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

The need for a reliable screening test for classical congenital adrenal hyperplasia prompted development of newborn screening programs. Worldwide incidence of classical congenital adrenal hyperplasia in this report was taken from newborn screening programs in France, Italy, Japan, New Zealand, Scotland, and the United States. Two populations in which the occurrence of congenital adrenal hyperplasia among live births has been reported with greater than usual frequency are the Yupik Eskimos of southwestern Alaska (1:282) and the people of La Reunion, France (1:2,141). Aside from these populations, 1,093,310 newborns were screened between 1980 and 1988, of whom 77 had congenital adrenal hyperplasia. Thus, worldwide incidence of this disorder was estimated at 1:14,199 live births for homozygous patients, 1:60 for heterozygous subjects, with a gene frequency of 0.0083. Incidence of congenital adrenal hyperplasia among whites was estimated to be 1:11,909 (41:488,279) for homozygous patients, 1:55 for heterozygous subjects with a gene frequency of 0.0091. Incidence for the salt-wasting form of congenital adrenal hyperplasia was 1:18,850 (58:1,093,310) compared with 1:57,543 (19:1,093,310) for congenital adrenal hyperplasia in the simple virilizing form. Thus, salt-wasting congenital adrenal hyperplasia was three times more common than simple virilizing congenital adrenal hyperplasia. Estimated incidence of congenital adrenal hyperplasia in white populations in Italy and France (1:10,866) was higher than in Scotland (1:17,098), New Zealand (1:14,500). The incidence in an Asian population (Japan) (1:15,800) did not differ significantly from that of the white population. In four of five populations, overall incidence was higher than previously reported, as was the frequency of the salt-wasting form (75% v 50% to 66%), suggesting improved case detection by newborn screening.(ABSTRACT TRUNCATED AT 250 WORDS)

Diet-hormone interactions: protein/carbohydrate ratio alters reciprocally the plasma levels of testosterone and cortisol and their respective binding globulins in man.

The aim of this study was to determine if a change in protein/carbohydrate ratio influences plasma steroid hormone concentrations. There is little information about the effects of specific dietary components on steroid hormone metabolism in humans. Testosterone concentrations in seven normal men were consistently higher after ten days on a high carbohydrate diet (468 +/- 34 ng/dl, mean +/- S.E.) than during a high protein diet (371 +/- 23 ng/dl, p less than 0.05) and were accompanied by parallel changes in sex hormone binding globulin (32.5 +/- 2.8 nmol/l vs. 23.4 +/- 1.6 nmol/l respectively, p less than 0.01). By contrast, cortisol concentrations were consistently lower during the high carbohydrate diet than during the high protein diet (7.74 +/- 0.71 micrograms/dl vs. 10.6 +/- 0.4 micrograms/dl respectively, p less than 0.05), and there were parallel changes in corticosteroid binding globulin concentrations (635 +/- 60 nmol/l vs. 754 +/- 31 nmol/l respectively, p less than 0.05). The diets were equal in total calories and fat. These consistent and reciprocal changes suggest that the ratio of protein to carbohydrate in the human diet is an important regulatory factor for steroid hormone plasma levels and for liver-derived hormone binding proteins.

ROC curve analysis of lesion detectability on phantoms: comparison of digital spot mammography with conventional spot mammography.

Although conventional screen--film mammography has excellent spatial resolution and is commonly used as a screening tool, certain inherent limitations prevent its further improvement. New digital mammography techniques, despite lower spatial resolution than screen--film mammography, may overcome these limitations. This study compared lesion detectability between charge coupled device-based digital spot mammography and conventional spot mammography. A total of 100 sets of images of specially designed breast phantoms was acquired, with variable background achieved by overlapping several layers of grapefruit fibre on a 4 cm thick lucite slab, using both modalities. 75 sets were "normal" images and 25 sets were images with simulated lesions. Four radiologists assessed the images according to a five-point confidence scale. The results were used to construct receiver operating characteristic curves. No statistical difference was observed between the two sets of curves for individual radiologists as well as pooled data. The lower spatial resolution of digital mammography was compensated for by its higher contrast sensitivity relative to conventional spot mammography.

Pulmonary embolism presenting as disseminated intravascular coagulation.

We report an unusual case of disseminated intravascular coagulation. Occult pulmonary embolism is a recognised cause of disseminated intravascular coagulation. Unexplained shock should prompt the physician to search for a thrombotic cause such as pulmonary thromboembolism.

Central nervous system inflammatory demyelinating disorders among Hong Kong Chinese.

Classical multiple sclerosis (CMS) and neuromyelitis optica spectrum disorders (NMOSD) are distinct central nervous system inflammatory demyelinating disorders (CNS IDD). Early diagnosis of CNS IDD is important as appropriate immunotherapies to optimize prognosis. We studied the diagnoses of CNS IDD among Hong Kong Chinese in a hospital-based setting. Consecutive Chinese patients who presented to our hospital with clinically isolated syndrome and subsequently diagnosed to have CNS IDD from 1980 to 2010 were reviewed. Patients with known diagnosis of CNS IDD referred for further care were excluded. Serial sera were assayed for aquaporin-4 autoantibodies (AQP4 Ab), at least 3 assays within 2-5years. A total of 210 patients diagnosed to have CNS IDD with disease duration of at least 2years were studied. Among 198 patients with serial sera available, 40 (20.2%, 20 had NMO and 20 other NMOSD) were AQP4 Ab-positive. Four patients who were AQP4 Ab-negative on the initial assay converted to AQP4 Ab-positive on repeated assays. The diagnoses of 210 patients were CMS in 88 (41.9%), NMOSD 47 (22.4%, 27 NMO, 20 other NMOSD), single attack of myelitis 23 (11.0%), single attack of optic neuritis 21 (10.0%), relapsing myelitis 10 (4.8%), acute disseminated encephalomyelitis (ADEM) 9 (4.3%), relapsing optic neuritis in 6 (2.9%), opticospinal multiple sclerosis 3 (1.4%) and single attack of brainstem encephalitis 3 (1.4%). Compared to CMS, NMOSD patients had older onset age, lower frequencies of brain MRI abnormalities and CSF OCB, higher frequency of LETM, higher CNS inflammation attack frequency in the first 2years, worse clinical outcome with higher EDSS score and mortality rate. This hospital-based study suggests that CMS (41.9%) and NMOSD (22.4%) are the most common CNS IDD among Hong Kong Chinese. NMOSD has worse clinical outcome than CMS. Detection of AQP4 Ab facilitates early diagnosis and prompts immunotherapies of NMOSD.

Inflammatory state of type II diabetic patients with chronic ulcers in response to herbal treatment.

BACKGROUND: Type II diabetic patients easily develop ulcers over their feet which heal with great difficulties and not infrequently, end up in amputations. In the quest for innovative means to avoid amputation, herbal medicine has been used in China to heal ulcers. METHOD: A randomized placebo controlled clinical trial involving 80 patients was conducted to test whether a herbal formula taken orally could help to preserve the ulcerated leg. Other parameters measured included granulation maturation time, skin temperature and circulation, and tumor necrosis factor alpha (TNF-α). RESULTS: showed a 85% limb rescue with the herbal treatment group showing superiority over placebo group. TNF-α decline was observed with gradual ulcer healing and the herbal supplement group showed a more impressive decline (p=0.037).