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Biomedical subjects

H H Ho

Publications and source records attributed to H H Ho.

At least 19 recordsLinked to original sources

Limited knee joint range of motion due to invisible gouty tophi.

OBJECTIVES: Tophi deposits are a well-known cause of joint destruction, gouty nephropathy and spinal cord compression. This study reports another major complication of gout, namely tophi deposition causing limited knee joint excursion. METHODS: Seven gout patients with limited knee joint excursion owing to tophi deposition were studied to reveal clinical features and magnetic resonance imaging (MRI) findings. None of the patients were able to assume a full squatting posture. RESULTS: No patients had visible subcutaneous tophi over the knee joints, except for one case in which a pea-sized subcutaneous tophus was noted. Additionally, two patients even lacked visible tophi elsewhere. All knee problems in the study group were initially regarded as being due to degenerative or other internal derangements, but MRI unexpectedly revealed multiple tophaceous depositions within and around the joint. CONCLUSIONS: Intra-articular and periarticular tophi limiting knee joint range of motion are a rare but important cause of walking disability in gout patients. Although most patients do not display visible subcutaneous tophi over the knee on physical examination, the differential diagnosis should consider intra-articular tophi and MRI is valuable in this clinical setting.

Adult↗

Concomitant septic and gouty arthritis--an analysis of 30 cases.

OBJECTIVES: To analyse the clinical features and outcomes of gouty patients with concomitant septic arthritis in a medical centre. METHODS: From the hospital database, we collected 30 hospitalized cases with concomitant septic arthritis and gouty arthritis from 1987 to 2001. All patients had positive bacterial culture and monosodium urate crystals in the affected joints. Medical records of the patients were analysed in detail. RESULTS: The mean age of patients was 52.8+/-12.5 yr. One-third of patients were afebrile at presentation, 30% had a normal blood leucocyte count and 10% had a synovial fluid leucocyte count less than 6000/mm3. The knee joint was the most common site of involvement, followed by the ankle, shoulder and wrist joints. Most patients had long-standing disease and subcutaneous tophi. Subcutaneous tophi rupture with secondary wound infection is the most common route of infection. Causative micro-organisms were Staphylococcus aureus (16 cases, 7 of whom were oxacillin-resistant), Streptococcus sp. (5 cases), Pediococcus sp. (1 case), and Gram-negative bacilli (9 cases). Fourteen patients received surgical debridement, among them two patients had an arthrodesis owing to severe joint destruction and one received above-knee amputation. Two patients died. One died of septic complications and the other died of acute myocardial infarction. CONCLUSIONS: Septic arthritis coexistent with gout presented a diagnostic difficulty. An early diagnosis requires a high level of suspicion. Prompt aspiration and analysis of the synovial fluid is imperative, regardless of the absence of fever or leucocytosis. Culture of the aspirated synovial fluid is warranted in gouty attack, even when it has a low white cell count or the Gram stain reveals no organisms.

Adult↗

Charged residues at the intracellular boundary of transmembrane helices 2 and 3 independently affect constitutive activity of Kaposi's sarcoma-associated herpesvirus G protein-coupled receptor.

Because charged residues at the intracellular ends of transmembrane helix (TMH) 2 and TMH3 of G protein-coupled receptors (GPCRs) affect signaling, we performed mutational analysis of these residues in the constitutively signaling Kaposi's sarcoma-associated herpesvirus GPCR (KSHV-GPCR). KSHV-GPCR contains the amino acid sequence Val-Arg-Tyr rather than the Asp/Glu-Arg-Tyr ((D/E)RY) motif at the intracellular end of TMH3. Mutation of Arg-143 to Ala (R143A) or Gln (R143Q) abolished constitutive signaling whereas R143K exhibited 50% of the basal activity of KSHV-GPCR. R143A was not stimulated by agonist, whereas R143Q was stimulated by growth-related oncogene-alpha, and R143K, similar to KSHV-GPCR, was stimulated further. These findings show that Arg-143 is critical for signal generation in KSHV-GPCR. In other GPCRs, Arg in this position may act as a signaling switch by movement of its sidechain from a hydrophilic pocket in the TMH bundle to a position outside the bundle. In rhodopsin, the Arg of Glu-Arg-Tyr interacts with the adjacent Asp to constrain Arg outside the TMH bundle. V142D was 70% more active than KSHV-GPCR, suggesting that an Arg residue, which is constrained outside the bundle by interacting with Asp-142, leads to a receptor that signals more actively. Because the usually conserved Asp in the middle of TMH2 is not present in KSHV-GPCR, we tested whether Asp-83 at the intracellular end of TMH2 was involved in signaling. D83N and D83A were 110 and 190% more active than KSHV-GPCR, respectively. The double mutant D83A/V142D was 510% more active than KSHV-GPCR. That is, cosubstitutions of Asp-83 by Ala and Val-142 by Asp act synergistically to increase basal signaling. A model of KSHV-GPCR predicts that Arg-143 interacts with residues in the TMH bundle and that the sidechain of Asp-83 does not interact with Arg-143. These data are consistent with the hypothesis that Arg-143 and Asp-83 independently affect the signaling activity of KSHV-GPCR.

Amino Acid Sequence↗

Lead chelation therapy and urate excretion in patients with chronic renal diseases and gout.

BACKGROUND: It is known that chronic renal insufficiency (CRI) patients with gout may have subtle lead poisoning. In addition, gout episodes frequently aggravate progressive renal insufficiency because of the use of nephrotoxic drugs and urate deposition. Our study was arranged to evaluate the causal effect of environmental lead exposure on urate excretion in CRI patients. METHODS: A cross-section study and a randomized, controlled trial were performed. Initially, 101 patients with CRI and without a history of previous lead exposure received ethylenediaminetetraacetic acid mobilization tests to assess body lead stores (BLS). Then, a clinical trial was performed; 30 CRI patients with gout and high-normal BLS and the changes of urate excretion in these patients were compared before and after lead chelating therapy. The treated group received four-week chelating therapy, and the control group received a placebo therapy. RESULTS: The BLS of patients with CRI and gout was higher than that of patients with CRI only, and none had subtle lead poisoning. The BLS, not the blood lead level (BLL), significantly correlated to indices of urate excretion in all CRI patients after related factors were adjusted. In addition, after lead chelating therapy, urate clearance markedly improved after a reduction of the BLS of patients with CRI and gout (study group 67.9 +/- 80.0% vs. control group 1.2 +/- 34.0%, P = 0.0056). CONCLUSION: Our findings suggest that the chronic low-level environmental lead exposure may interfere with urate excretion of CRI patients. Importantly, the inhibition of urate excretion can be markedly improved by lead chelating therapies. These data shed light on additional treatment of CRI patients with gout; however, more studies are needed to confirm our findings.

Adult↗

Clinical characteristics of juvenile rheumatoid arthritis in Taiwan.

This study aimed to investigate the clinical features of juvenile rheumatoid arthritis among Taiwan children. The medical records of 228 children who had juvenile rheumatoid arthritis treated in the Chang Gung Medical Center in Taiwan from 1978 through 1998 were retrospectively reviewed. A total of 146 boys and 82 girls (M:F ratio, 1.8:1) were included in this study. Clinical and laboratory data of these patients were collected from medical charts. Pauciarticular onset (56%) was the most common type of juvenile rheumatoid arthritis, followed by polyarticular (36%) and systemic (8%) type. The positive rate for rheumatoid factor, human leukocyte antigen B27, and antinuclear antibody were 9.2%, 55.2%, and 16.2%, respectively. Uveitis was observed in 5.7% of patients. Compared with previous reports in other regions and populations, remarkably different features of juvenile rheumatoid arthritis were found in this study, which included a higher prevalence among boys than girls, a high positive rate of human leukocyte antigen B27, and a low rate of uveitis.

Adolescent↗

Correlation of acoustic velocity of synovial fluid with markers of inflammation in arthritic patients.

Analysis of synovial fluid is important in the evaluation and treatment of arthritic conditions. This study measured the acoustic velocity of synovial fluids in patients with degenerative joint diseases, crystal arthropathy, and other inflammatory arthropathies using the ultrasonic pulse-echo method. The measured acoustic velocities of these bio-fluids were then correlated with clinical parameters including the synovial white blood cell count (WBC), clarity, viscosity, string test, erythrocyte sedimentation rate (ESR), and serum C-reactive protein (CRP). Results showed that the acoustic velocities were correlated with ESR (p = 0.0016) and CRP (p = 0.0001). The mean acoustic velocity of inflammatory synovial fluids, defined as synovial fluids with WBCs of more than 2000/mm3, was greater than that of synovial fluids with WBCs of less than 2000/mm3 (1550 +/- 4.5 m/s vs 1544 +/- 1.5 m/s, p = 0.007). This study demonstrated that the acoustic velocity of synovial fluid correlates well with severity of inflammation. These findings suggest that measurement of acoustic velocity may be useful in the clinical evaluation and management of arthritic conditions.

Arthritis↗

Effect of secretin on children with autism: a randomized controlled trial.

To determine the effect of intravenous porcine secretin on autistic behaviours in children aged 2 to 7 years, the effects of secretin on (1) performance on a standardized language measure, and (2) autistic behaviours, as rated by parents and child development professionals was examined. Employing a randomized, double-blind, placebo-controlled design, 95 participants were assigned to one of two groups and administered a single dose of either secretin or placebo. A follow-up assessment was conducted 3 weeks after the injection. No significant differences in language or autistic behaviour measures were observed at the 3-week follow-up between the groups. Also, there was no significant difference in the proportion of individuals who improved by > or = 6 points on the language measure at follow-up. This study showed no significant effects of secretin on children with autism. Our results are consistent with a systematic review of randomized controlled trials evaluating the effect of secretin in children with autism.

Animals↗

Intermittent pulse therapy with itraconazole for onychomycosis. Experience in Hong Kong Chinese.

Forty-four Hong Kong Chinese adults comprising 10 cases of fingernail and 42 cases of toenail onychomycosis were recruited for a 3-weekly itraconazole pulse therapy. Each pulse consisted of seven consecutive daily 400 mg doses with a 3-week interval between treatments. All patients in the fingernail group and 37 in the toenail group completed the study. The clinical cure, clinical response and mycological cure rates at week 36 were 70, 90 and 90% for fingernail and 35, 81 and 68% for toenail groups, respectively. Side-effects including biochemical abnormalities were minimal, and returned to normal upon cessation of treatment. We conclude that itraconazole pulse therapy is very effective, safe and well-tolerated for fingernail and toenail onychomycosis.

Adult↗

The clinical features and prognosis of lupus with disease onset at age 65 and older.

Systemic lupus erythematosus (SLE) in the elderly is uncommon and rarely reported with disease onset at age 65 and older. The aim of this study is to retrospectively analyze the influence of age at disease onset on the clinical features and prognosis of SLE. From 1988 to 1998, we encountered 21 lupus patients with disease onset at age 65 and older (all are included in group A). For comparison, 21 lupus patients with disease onset between 50-64 years of age (group B) and 152 lupus patients with disease onset before 50 years of age (group C) were obtained by a simple random sampling method from the hospital registry. Clinical features as included in the 1982 ARA revised criteria for classification of SLE and survival rate were analyzed and compared among these three groups. Group A had a smaller female to male ratio, longer duration from disease onset to diagnosis, less malar rash, more discoid lupus, and shorter survival rate that group C. There was no statistically significant difference in clinical features and survival between groups A and B, as well as between female and male patients of these two groups. The main cause of death in group A was septic shock. In conclusion, the clinical features and prognosis of SLE were influenced by the age at disease onset. However, clinical features and prognosis of SLE were similar in both late-onset lupus groups.

Adult↗

Crusted (Norwegian) scabies in two old-age home residents.

Scabies is commonly seen in hospitals, where it frequently affects geriatric and convalescent patients. The clinical features of the classic form of scabies are well recognised. Crusted (Norwegian) scabies, which is a hyperinfestation variant of scabies, is very contagious and can present as other dermatoses, thus delaying the correct diagnosis and management. Two residents of different old-age homes presented with hyperkeratotic skin eruptions, which later proved to be crusted scabies. In both cases, the scabies was initially misdiagnosed as eczema. The delay in making a correct diagnosis led to an outbreak of scabies in the old-age home in which one of the patients was residing.

Aged↗

The N terminus of Kaposi's sarcoma-associated herpesvirus G protein-coupled receptor is necessary for high affinity chemokine binding but not for constitutive activity.

Kaposi's sarcoma-associated herpesvirus (KSHV) contains a gene encoding a G protein-coupled receptor (KSHV-GPCR) that is homologous to mammalian chemokine receptors. KSHV-GPCR signals constitutively (in an agonist-independent manner) via the phosphoinositide-inositol 1,4,5-trisphosphate pathway. Because it has been proposed that the N terminus (N-TERM) of other GPCRs may act as tethered agonists, we determined whether the N-TERM of KSHV-GPCR is necessary for constitutive signaling activity or ligand binding, or both. We show that replacement of the entire N-TERM of KSHV-GPCR with those of two other GPCRs, deletion of residues within the N-TERM, and disruption of a putative disulfide bond that may hold the N-TERM in close proximity to extracellular loop 3 do not affect constitutive signaling activity but decrease chemokine binding. There were differences in the effects of mutation of the N-TERM on binding of the chemokines growth-related oncogene alpha, which is an agonist, and interferon-gamma-inducible protein-10, which is an inverse agonist. The effects on chemokine binding were accompanied by changes in chemokine regulation of KSHV-GPCR signaling. We conclude that the N-TERM is not necessary for constitutive KSHV-GPCR signaling, i.e. the N-TERM is not a tethered agonist, but plays a crucial role in binding of chemokine ligands and of chemokine regulation of KSHV-GPCR signaling.

Animals↗

Glycosylation is important for binding to human calcitonin receptors.

Human calcitonin receptor (hCTR) subtypes contain three or four potential Asn-linked glycosylation sites in their extracellular amino termini. The role of glycosylation in hCTR function has not been identified, but it has been suggested that inhibition of glycosylation does not affect binding or signaling. To determine the role of glycosylation in hCTR biology, we studied the effects of inhibition of glycosylation and of substitution of Asn residues that are potential glycosylation sites. Native and mutated hCTRs were studied after transient expression in monkey kidney COS-1 cells. Tunicamycin, administered as part of a treatment protocol that inhibited glycosylation of all expressed receptors, decreased salmon calcitonin (sCT) binding affinities and signaling potencies at hCTRs with three or four potential glycosylation sites. In hCTR3, which contains three potential glycosylation sites at positions 26, 78, and 83, site-specific substitution of Asn-26 by Ala had no effect on sCT binding affinity or potency, whereas substitution of Asn-78 or Asn-83 lowered sCT affinity and potency. A mutant hCTR3 in which all three Asn residues were substituted with Ala exhibited no high-affinity sCT binding and potencies of several calcitonin analogues that were more than 100-fold lower than that of native hCTR3. Our data show that glycosylation is important for high-affinity binding and potency of calcitonin analogues at hCTRs.

Alanine↗

Chelation therapy for patients with elevated body lead burden and progressive renal insufficiency. A randomized, controlled trial.

BACKGROUND: Nephropathy is known to occur in persons exposed to high levels of lead, but the question of whether long-term exposure to low levels of environmental lead is associated with impaired renal function remains controversial. OBJECTIVE: To examine whether chelation therapy slows the progression of renal insufficiency in patients with mildly elevated body lead burden. DESIGN: Randomized, controlled trial. SETTING: Academic medical center in Taiwan. PATIENTS: 32 patients with chronic renal insufficiency (serum creatinine level > 132.6 micromol/L [1.5 mg/dL] and < 353.8 micromol/L [4.0 mg/dL]), mildly elevated body lead burden (> 0.72 micromol [150 microg] of lead per 72-hour urine collection and < 2.90 micromol [600 microg] of lead per 72-hour urine collection [EDTA mobilization tests]), and no history of heavy lead exposure. INTERVENTION: The treatment group received 2 months of chelation therapy; the control group received no therapy. MEASUREMENTS: The reciprocal of serum creatinine (1/Cr) was used as an index of progressive renal insufficiency. RESULTS: Rates of progression of renal insufficiency were similar in the treatment group and the control group during a 12-month baseline observation period (1/Cr, 0.000054 L/micromol per month compared with 0.000046 L/micromol per month; P > 0.2). After the 2-month treatment period, improvement in renal function was greater in the treatment group than in the control group. In the 12 months after the treatment period, renal insufficiency progressed more slowly in the treatment group than in the control group (1/Cr, 0.000033 +/- 0.00038 L/micromol per month compared with 0.000045 +/- 0.000038 L/micromol per month; P = 0.0030). CONCLUSION: Chelation therapy seems to slow the progression of renal insufficiency in patients with mildly elevated body lead burden. This implies that long-term exposure to low levels of environmental lead may be associated with impaired renal function in patients with chronic renal disease.

Adult↗

The correlates of depressed mood in adolescents in Hong Kong.

PURPOSE: Most studies of depressed mood and its correlates in adolescents have been conducted in Western countries. This study examined the relationship between a broad range of stressors and depressed mood in a community sample of Hong Kong adolescents. METHODS: Secondary school students (n = 996) completed the Chinese Beck Depression Inventory (C-BDI), provided demographic information, and indicated their perceptions of family and peer relationships, school function and pressures, and subjective health, and some measures salient to the Hong Kong environment: triad gang pressure, religiosity, and intent to emigrate. The correlation between C-BDI and these variables was assessed in bivariate and multivariate analyses. RESULTS: Hong Kong adolescents reported higher levels of depressive symptoms than a comparison group of Western teenagers. Girls showed more symptoms than boys. All stressors correlated in bivariate analyses with C-BDI, indicating similar influences on depressed mood in Western and Hong Kong teenagers. In multivariate analyses, the stressors contributed cumulatively to the C-BDI score. Perceptions of a lack of parental understanding and peer acceptance appeared as the strongest variables in predicting depressed mood. CONCLUSIONS: Depressed mood is highly prevalent among Hong Kong teenagers. Stressors play a cumulative role in their relationship to mood. Our findings point to the importance of broad screening of this vulnerable population.

Adolescent↗

Ankylosing spondylitis with severe chondrocalcinosis: case report.

The term chondrocalcinosis is often used to describe the radiological or pathological features of calcified joint cartilage, and it usually indicates the deposition in cartilage of calcium pyrophosphate dihydrate. The prevalence of chondrocalcinosis increases with age, with dramatic increases occurring in the decades past age 60. In younger patients with chondrocalcinosis, either clinical evidence of associated metabolic diseases leading to the chondrocalcinosis or familial disease occurrence usually can be detected. We report a 42-year-old Chinese woman with ankylosing spondylitis and arthritis involving multiple peripheral joints. Severe chondrocalcinosis was detected incidentally in this patient, however, subsequent studies revealed no associated metabolic disease or familial susceptibility and the clinical features of this patient were different from those of ankylosing chondrocalcinosis (pseudoankylosing spondylitis). The cause of chondrocalcinosis in this patient remains unknown, but joint damage and repair could have been initiating or aggravating factors of the chondrocalcinosis.

Adult↗

Salmonella septic arthritis in systemic lupus erythematosus and other systemic diseases.

Salmonella infection is an important problem in immunocompromised patients. The synovium is a particular metastatic focus of Salmonella infection and can result in many disabilities of life. Systemic lupus erythematosus (SLE) patients were highly susceptible to Salmonella infection. In the past 6 years, 41 patients with Salmonella septic arthritis have been treated in our hospital. Eleven patients had an underlying systemic disease of SLE which presented with a distinctive clinical course. Alcoholic liver disease (six cases) was another common underlying systemic disease. The most frequent predisposing articular factor was avascular necrosis (16 cases). The hip joint was the most commonly involved site. Salmonella group B was the most common serotype (30/41). Seventy-three per cent (8/11) of the SLE group had involvement of two or more joints compared with only three out of 30 patients in the non-SLE group. The sex differentiation shows a predominance of young females (10/11) in the SLE group and middle-aged males in the non-SLE group. Moreover, in the SLE group, all 11 patients shared the risk of lupus nephritis and steroid use. Ten patients had Salmonella group B bacteraemia and five had urinary tract infections simultaneously. In the non-SLE group, there were 10 patients with a history of steroid use, three with antecedent enteritis, 12 with bacteraemia, and three with necrotising fasciitis. Seven patients in each of the groups had a recurrent course. However, three patients in the non-SLE group had died during the episode of septic arthritis.

Adolescent↗

Kabuki make-up (Niikawa-Kuroki) syndrome: cognitive abilities and autistic features.

Kabuki make-up syndrome (KMS), also known as Niikawa-Kuroki syndrome, is a rare malformation complex characterized by a peculiar facies with long palpebral fissures and partially everted lower eyelid, mental retardation, dwarfism, and skeletal and dermatoglyphic abnormalities. Four children from different ethnic groups presented with phenotypic manifestation of KMS. They showed variable degrees of learning disabilities, mental retardation, and autistic behavior. Two boys declined in IQ in early adolescence. In addition to being aware of the variable presentation of cognitive and behavioral characteristics in those already diagnosed, it is important to consider KMS when assessing dysmorphic children with learning disabilities and/or autism.

Abnormalities, Multiple↗