Tourette syndrome associated with Page kidney

Clinical and Experimental Nephrology - Page kidney is a rare condition leading to secondary hypertension and encountered most frequently due to traumatic subcapsular hematoma. Here, we present a...     

Tension band plates have greater risks of complications in temporary epiphysiodesis

PurposeThis study aimed to compare the efficacy of decreasing leg-length discrepancy (LLD) and postoperative complications between tension band plates (TBP) and percutaneous transphyseal screws (PETS).MethodsThis retrospective study reviewed LLD patients who underwent temporary epiphysiodesis at the distal femur and/or proximal tibia from 2010 to 2017 (minimum two years follow-up). Efficacy of decreasing LLD was assessed one and two years postoperatively. Complications were classified with the modified Clavien-Dindo-Sink complication classification system. Knee deformities were assessed by percentile and zone of mechanical axis across the tibial plateau.ResultsIn total, 53 patients (25 boys, 28 girls) underwent temporary epiphysiodesis (mean age, 11.4 years). The efficacy of decreasing LLD at two years between the TBP (n = 38) and PETS (n = 15) groups was comparable. Seven grade III complications were recorded in six TBP patients and in one PETS patient who underwent revision surgeries for knee deformities and physis impingement. Four grade I and two grade II complications occurred in the TBP group. The mechanical axis of the leg shifted laterally in the PETS group and medially in the TBP groups (+7.1 percentile versus -4.2 percentile; p < 0.05). Shifting of the mechanical axis by two zones was noted medially in four TBP patients and laterally in two PETS patients.ConclusionMore implant-related complications and revision surgeries for angular deformities were associated with TBP. A tendency of varus and valgus deformity after epiphysiodesis using TBP and PETS was observed, respectively. Patients and families should be informed of the risks and regular postoperative follow-up is recommended.Level of evidenceLevel III     

SC phocomelia syndrome, premature centromere separation, and congenital cranial nerve paralysis in two sisters, one with malignant melanoma

Two middle age sisters had most manifestations of the SC phocomelia syndrome including postnatal growth retardation, symmetric limb deficiencies with radial aplasia and absent thumbs, facial anomalies with microcephaly, microphthalmia, hypoplastic nasal alae, and borderline to mild mental retardation. Unusual findings included congenital paralysis of some cranial nerves in both patients and malignant melanoma in the proposita. Cultured lymphocytes from both patients, and skin fibroblasts, Epstein Barr virus-transformed lymphocytes, and tumor cells from the proposita demonstrated premature separation of centromeric heterochromatin (PCS) of many chromosomes, a finding noted previously in the SC phocomelia syndrome and the similar but more severe Roberts syndrome. Extensive overlap of the phenotypes of the sisters and 15 other patients with either syndrome and PCS confirms that these are either allelic conditions or the same disease--designated Roberts-SC phocomelia syndrome. The role of PCS in the syndrome(s) remains uncertain since some patients with the characteristic clinical phenotypes are reported to lack it.     

Growth suppression of human colorectal carcinoma in nude mice by monoclonal antibody C27-abrin A chain conjugate

PURPOSE: The aim of this study was to assess an immunotoxin, monoclonal antibody C27-abrin A chain conjugate (MAAC), that might be effective in the treatment of colorectal carcinoma. METHODS: The immunotoxin was prepared by a specific monoclonal antibody against carcinoembryonic antigen (CEA), monoclonal antibody C27, linked toN-succinimidyl-3-(2-pyridyldithio)propionate and then coupled covalently to the toxic abrin-A chain to synthesize MAAC. The therapeutic role of this immunotoxin in suppressing thein vitro andin vivo growth of CEA-secreting human colorectal cancer cells (LS174T) was assayed by methods of protein biosynthesis inhibition, cell colony proliferation, and treatment of tumor cells before and after inoculation in nude mice. RESULTS: We found that MAAC effectively suppressed the growth of LS174T in culture medium and completely eradicated cells in inoculated nude mice. In contrast, irrelevant immunotoxin antiferritin-abrin A chain conjugate and isotype-matched monoclonal immunoglobin (MOPC21IgG1)-abrin A chain conjugate did not cause such effects. Thein vitro toxicity was highly specific because the conjugate (MAAC) inhibitedde novo protein biosynthesis, impeded growth, and caused death of cells possessing surface CEA determinants. The 50 percent inhibition dose values of the conjugate for colonogenic survival and for protein biosynthesis in LS174T cells were 0.09 g/ml and 0.06 g/ml, respectively. Colony survival was inhibited 96.3 percent after prolonged MAAC treatment. MAAC showed selective cytotoxicity; the inhibitory effect of MAAC to the CEA-secreting LS174T cells over the CEA-nonsecreting human embryonic kidney cells was 16-fold. CONCLUSION: These results indicate that MAAC may be of benefit in therapy during or soon after resection of colorectal carcinoma or in patients who have micrometastasis.Supported by a grant from the National Science Council and the Veterans General Hospital-Taipei, Taipei, Taiwan.     

Serum levels of interferon-α and-γ in acute and chronic hepatitis B virus infection

To evaluate the potential implication ofin vivo interferon production in the pathogenesis of different forms of acute and chronic hepatitis B virus infection, serum levels of interferon- and- were measured using immunoassay techniques in 20 patients with acute hepatitis B who subsequently cleared the virus (group Ia), 8 patients with acute hepatitis B who became HBsAg carriers (group Ib), 55 patients with chronic hepatitis B (group II), and 15 healthy controls. None of the controls had interferon- or- detectable in serum, while 15% and 100% of group Ia patients, 25% and 100% of group Ib patients, and 22% and 15% of group II patients, had raised serum levels of interferon- and-, respectively. Serum interferon- was detected significantly more frequently in group Ia and Ib patients than in controls and in group II patients. Among patients with acute hepatitis B, serum levels of interferon- and- showed no significant difference between group Ia and group Ib patients. Among patients with chronic hepatitis B, interferon- was detected significantly more frequently in patients with serum HBV-DNA (31.4% or 11/35) than in those without (5% or 1/20), whereas interferon- was detected significantly more frequently in patients with chronic active hepatitis (28% or 7/25) than in those with chronic persistent hepatitis (3.3% or 1/30). In conclusion, in acute hepatitis B, serum levels of interferon- and- did not show a significant difference between patients who subsequently cleared the virus and those who became HBsAg carriers. In chronic hepatitis B, the raised serum levels of interferon- correlated with the presence of viral replication, while the raised serum levels of interferon- correlated with the presence of histological evidence of active hepatitis.     

Differential regulation of EGF production, EGF receptor-binding, and cellular growth by sodium-butyrate in hep3b and plc/prf/5 human hepatoma-cells

Hep3B and PLC/PRF/5 human hepatoma cells express epidermal growth factor (ECF) mRNA and secret this polypeptide growth factor into the culture medium. The production of EGF was inhibited by sodium butyrate in a dose-dependent manner. EGF receptor numbers in both cell lines were increased after treatment with butyrate for 2 days, In addition, the binding affinity of EGF to its receptor was decreased in butyrate-treated PLC/PRF/5 cells while it did not change in Hep3B cells. EGF-stimulated cell growth in PLC/PRF/5 cells was attenuated by sodium butyrate whereas no significant inhibition df cell growth of Hep3B cells was found in the same condition. Our results suggest that EGF acts as an autocrine growth stimulator in human hepatoma cells and sodium butyrate can differentially regulate the responses of hepatoma cells to EGF by modulating the differentiation states of these cells.     

Response from lieberman and colleagues

Respond on comments on Lieberman's article: Cyclosiloxanes Produce Fatal Liver and Lung Damage in Mice. Environ Health Perspect 107:161-165     

Mortality for certain diseases in areas with high levels of arsenic in drinking water.

Blackfoot disease was prevalent in a limited area on the southwest coast of Taiwan, where artesian well water containing arsenic (median = 0.78 ppm arsenic) had been used for many years. Previous studies of arsenic exposure in the blackfoot disease endemic area have been focused on malignant tumors. We, therefore, conducted this study to analyze mortality of all death causes in blackfoot disease endemic areas and to determine other neglected cancers or noncancer diseases related to artesian well water containing high levels of arsenic. We calculated standardized mortality ratios for cancer and noncancer diseases, by sex, during the period from 1971 to 1994 and compared them to the local reference group (i.e, Chiayi-Tainan County) and the national reference group (i.e., Taiwan population). The results revealed marked standardized mortality ratio differences for the 2 reference groups. Greater mortality was found for males and females with bladder, kidney, skin, lung, nasal-cavity, bone, liver, larynx, colon, and stomach cancers, as well as lymphoma than in the local reference population. With respect to noncancer diseases, we found greater mortality for males and females who had vascular disease, ischemic heart disease, diabetes mellitus, and bronchitis than in the local reference group. Mortalities for other diseases--including rectal cancer, cerebrovascular disease, and other diseases--were higher among cases than the local reference group. Our results indicated that the hazardous effect of arsenic is systemic. Diseases related to arsenic exposure included those reported previously by other investigators, as well as diseases reported in the present study.     

Rectal cancer mortality and total hardness levels in Taiwan's drinking water.

The possible association between the risk of rectal cancer and hardness levels in drinking water from municipal supplies was investigated in a matched case-control study in Taiwan. All eligible rectal cancer deaths (986 cases) of Taiwan residents from 1990 through 1994 were compared with deaths from other causes (986 controls), and the hardness levels of the drinking water used by these residents were determined. Data on water hardness throughout Taiwan were collected from Taiwan Water Supply Corporation (TWSC). The control group consisted of people who died from other causes and the controls were pair matched to the cases by sex, year of birth, and year of death. The results show a significant negative relationship between drinking water hardness and rectal cancer mortality. Odds ratio and 95% confidence intervals were 1.24 (1.01-1. 55) and 1.38 (1.10-1.73), respectively, for exposure to moderately hard water and soft water compared with the use of hard water. Trend analyses showed an increasing odds ratio for rectal cancer with decreasing levels of hardness in drinking water. This is an important finding for the Taiwan water industry and human health.     

Fungal corneal ulcers of onion harvesters in southern Taiwan

Fungal corneal ulcers related to agriculture has been reported throughout the world, especially in tropical areas. Most of them were sporadic and had histories of ocular trauma or use of topical corticosteroids and topical antibiotics. Five onion harvesters had fungal corneal ulcers during the same harvest period in Southern Taiwan. The authors think that this is the first report of a group occurrence relating to agricultural workers. Although all of the patients improved after medical and surgical management, their vision was greatly decreased. It is suggested that the tropical climate, the harvest procedure, the characteristic monsoon, and lack of eye protection were involved. Therefore, the importance of the eye protection, hygiene education, and improving medical care to reduce the occurrence of fungal corneal ulcer in agriculture workers must be emphasised.