A clinical comparison between dedifferentiated low-grade osteosarcoma and conventional osteosarcoma.

AIMS: The purpose of this study was to clarify the clinical behaviour, prognosis, and optimum treatment of dedifferentiated low-grade osteosarcoma (DLOS) diagnosed based on molecular pathology. PATIENTS AND METHODS: We retrospectively reviewed 13 DLOS patients (six men, seven women; median age 32 years (interquartile range (IQR) 27 to 38)) diagnosed using the following criteria: the histological coexistence of low-grade and high-grade osteosarcoma components in the lesion, and positive immunohistochemistry of mouse double minute 2 homolog (MDM2) and cyclin-dependent kinase 4 (CDK4) associated with MDM2 amplification. These patients were then compared with 51 age-matched consecutive conventional osteosarcoma (COS) patients (33 men, 18 women; median age 25 years (IQR 20 to 38)) regarding their clinicopathological features. RESULTS: The five-year overall survival (OAS) rates in the DLOS and COS patients were 85.7% and 77.1% (p = 0.728), respectively, and the five-year progression-free survival (PFS) rates were 57.7% and 44.9% (p = 0.368), respectively. A total of 12 DLOS patients received chemotherapy largely according to regimens for COS. Among the nine cases with a histological evaluation after chemotherapy, eight showed a poor response, and seven of these had a necrosis rate of < 50%. One DLOS patient developed local recurrence and five developed distant metastases. CONCLUSION: Based on our study of 13 DLOS cases that were strictly defined by histological and molecular means, DLOS showed a poorer response to a standard chemotherapy regimen than COS, while the clinical outcomes were not markedly different. Cite this article: Bone Joint J 2019;101-B:745-752.

Recurrent Membranous Nephropathy After Kidney Transplantation Associated With Phospholipase A2 Receptor and Successfully Treated With Rituximab: A Case Report.

Primary membranous nephropathy (MN) is an organ-specific autoimmune disease mainly caused by autoantibodies acting against the podocyte antigen M-type phospholipase A2 receptor 1 (PLA2R). Herein we present the clinical and histologic findings, including PLA2R staining, of early recurrent MN after kidney transplantation that was successfully treated with rituximab. A 60-year-old Japanese man had end-stage renal failure due to steroid-resistant primary MN and underwent ABO-incompatible living donor kidney transplantation. At 1 month after transplantation, a protocol biopsy revealed positive granular staining of IgG, C4d, and PLA2R on glomerular capillaries (GCs) without any abnormalities on light microscopy (LM). Although the patient had low-level proteinuria, recurrent MN was suspected based on the positive PLA2R staining; he was treated with an angiotensin receptor blocker and a single dose of 200 mg rituximab. However, proteinuria gradually increased to 877 mg/d. At 21 months after transplantation, a graft biopsy revealed spikes along the outer aspects of GC on LM, with stronger staining for PLA2R than that at 1 month after transplantation. A single dose of 500 mg rituximab was added, which effectively reduced proteinuria, and clinical remission continued until 3 years after transplantation. The latest graft biopsy showed reduced staining of PLA2R. The disease activity and therapeutic effect were well-reflected in the intensity of PLA2R staining. An approach intending an early diagnosis by protocol biopsy using PLA2R immunostaining is made and early treatment with rituximab will help reduce the risk of kidney graft loss due to recurrent primary MN.

Living Kidney Donation From a Donor With Pulmonary Sarcoidosis: A Case Report and Review of the Literature.

BACKGROUND: Sarcoidosis is a chronic systemic disease that is characterized by the formation of noncaseating granuloma and whose etiology is unclear. It is unclear whether patients with sarcoidosis are suitable organ donors. CASE: We treated a 56-year-old woman with pulmonary sarcoidosis who donated her kidney. She was previously in good health and was diagnosed with pulmonary sarcoidosis during her preoperative examination. Because she presented with no symptoms and was otherwise in good condition, donor nephrectomy was performed. RESULTS: Baseline biopsy examination showed no evidence of sarcoidosis. One year after transplantation, both the donor and the recipient had not developed kidney dysfunction or recurrence of sarcoidosis. CONCLUSION: This is a rare case in which a patient with pulmonary sarcoidosis donated a kidney for transplantation, and both the recipient and the donor were clinically healthy. A patient with sarcoidosis and no kidney lesion can donate a living kidney, because transplantation appears to be safe for both the recipient and the donor.

Utility of the Japanese Glomerular Filtration Rate Equation in Estimating Glomerular Filtration Rate of Donor Kidney.

BACKGROUND: An equation for the estimated glomerular filtration rate (eGFR) is generally used for evaluating renal function in Japan. OBJECTIVE: To assess the accuracy of the preoperative eGFR for estimating kidney donors' measured glomerular filtration rate (mGFR). METHODS: Between April 2009 and August 2014, 91 Japanese living kidney donors were included in this study. The eGFR was calculated as follows: eGFR = 194 × serum creatinine(-1.094) × Age(-0.287) (and × 0.739 for women), and the mGFR was evaluated using inulin clearance. The preoperative eGFR was then compared with the mGFR. RESULTS: Patients included 27 men and 64 women with a mean age of 56.8 ± 9.5 years (range, 36-79 years), mean body surface area of 1.56 ± 0.14 m(2) (range 1.27-1.92 m(2)), mean body mass index of 22.3 ± 2.3 kg/m(2) (range 14.0-27.0 kg/m(2)), and mean serum creatinine level of 0.66 ± 0.14 mg/dL (range 0.39-0.97 mg/dL). The mean eGFR was 81.3 ± 14.2 mL/min/1.73 m(2) (range 45.5-125.9 mL/min/1.73 m(2)), and the mean mGFR was 89.0 ± 15.5 mL/min/1.73 m(2) (range 45.4-130.7 mL/min/1.73 m(2)). The eGFR was significantly lower than the mGFR (P < .001). The correlation coefficient for the relationship between the eGFR and mGFR values was 0.503, and the mean difference between the 2 values was -7.8 (8.7%). CONCLUSIONS: Although the eGFR correlated with the mGFR, the eGFR values did not accurately estimate the mGFR in living kidney donors. Therefore, it is necessary to evaluate the mGFR, especially in marginal kidney donors.

Preemptive Living Donor Kidney Transplantation and Kidney Function at the Initial Hospital Visit: A Single-Center Case-Control Study.

BACKGROUND: Studies have revealed that patients who undergo preemptive kidney transplantation (PKT) have favorable prognoses compared with those who undergo kidney transplantation after the initiation of dialysis. The number of PKT cases performed worldwide has been increasing. The goal of this study was to determine the clinical characteristics of patients who may successfully receive PKT. METHODS: A single-center, case-control study was conducted to determine the clinical factors that lead to referral for PKT. RESULTS: Between April 1, 2009, and August 1, 2015, a total of 118 patients underwent living donor kidney transplantation. Thirty of these patients had not undergone dialysis before their initial visit to the study hospital. Of these, 20 received kidney transplantation before and after dialysis initiation, respectively (group PKT+, successful PKT; group PKT-, failed PKT). The baseline characteristics at the primary visit were compared between groups. The median duration from the first visit to the study institution to PKT was 5.6 ± 0.7 months. Serum creatinine (Cr) levels differed significantly between groups (PKT+ vs PKT-, 6.0 ± 0.3 mg/dL vs 7.5 ± 0.5 mg/dL; P = .03). The receiver-operating characteristic curves revealed that a serum Cr level >5.7 mg/dL at the initial visit to the unit was a cutoff point for predicting the success of PKT (area under the curve, 0.721; P = .02). CONCLUSIONS: Our results indicate that PKT should be performed within ∼6 months of the initial visit to the transplant center. Serum Cr levels <5.7 mg/dL predict successful PKT.

Immunoglobulin A Nephropathy in a Living Kidney Donor Diagnosed and Treated After Transplantation: A Case Report.

OBJECTIVE: We report the clinical course and pathologic findings of a kidney transplant donor who was diagnosed with immunoglobulin A (IgA) nephropathy by means of preimplantation biopsy and was later treated with methylprednisolone and tonsillectomy. CASE REPORT: The patient was a 57-year-old woman who met the criteria for kidney donation and was accepted as a donor. Donor nephrectomy was performed, and the preimplantation biopsy revealed that the donor had IgA nephropathy. One month after the nephrectomy, the donor's laboratory findings indicated proteinuria and hematuria. Because these findings indicated active IgA nephropathy, we decided to perform tonsillectomy and methylprednisolone pulse therapy. Soon after these treatments, the patient's proteinuria and hematuria were no longer observed. CONCLUSIONS: Subclinical IgA nephropathy can be incidentally found on preimplantation biopsies of living kidney donors. As demonstrated in this case, IgA nephropathy can become exacerbated and requires therapeutic intervention after kidney donation. Informed consent and careful observation should be used before and after transplantation, even for donors who have been determined to be eligible for kidney donation.

Plasma Cell-Rich Rejection After Kidney Transplantation and the Role of Donor-Specific Antibodies: A Case Report and Review of the Literature.

OBJECTIVE: We report a case of the clinical course and pathologic findings for a kidney transplant recipient with plasma cell-rich rejection (PCRR) accompanied by antibody-mediated rejection (ABMR). METHODS: A 29-year-old man with end-stage renal disease caused by lupus nephritis received an ABO-compatible living kidney transplant. RESULTS: Eighteen months after transplantation, the patient presented with proteinuria and increased serum creatinine. An episode biopsy revealed severe tubulointerstitial infiltration with plasma cells accompanied by peritubular capillaritis and positive findings on immunofluorescent C4d staining. Donor-specific antibodies were positive for DR52, and the patient was subsequently diagnosed with PCRR accompanied by ABMR. Treatment was initiated with high-dose steroids, intravenous immunoglobulin, gusperimus hydrochloride, muronmonab antibody CD3, and rituximab. However, ABMR persisted and allograft failure developed 20 months after onset. CONCLUSIONS: We argue that PCRR accompanied by ABMR is a subtype of PCRR that can progress to allograft failure owing to persistent ABMR.

Psychotropic medication adherence in correctional facilities: a review of the literature.

Despite the high percentage of incarcerated persons with mental illness, surprisingly little is known about effective ways to increase psychotropic medication adherence in prison and upon release. Currently, there are limited definitive data regarding psychotropic medication adherence patterns among incarcerated persons, and the data that exist often miss specific information on changes in behaviour, symptom management and adherence patterns over the length of a person's sentence, which has implications for post-release ability to live in the community. This paper presents the current literature regarding psychotropic medication adherence among mentally ill persons who are incarcerated. The factors identified in support of medication adherence, future clinical research and care strategies are provided.

Deletions involving both KCNQ2 and CHRNA4 present with benign familial neonatal seizures.

OBJECTIVE: Mutations of the genes encoding subunits of potassium voltage-gated channel, KCNQ2 and KCNQ3, have been identified in patients with benign familial neonatal seizures (BFNS). This study set out to determine the frequency of microchromosomal deletions of KCNQ2 or KCNQ3 associated with BFNS. METHODS: The study subjects were patients with BFNS (n = 22). Microdeletions were sought by multiplex ligation-dependent probe amplification and then confirmed by fluorescence in situ hybridization and characterized by array-based comparative genomic hybridization. RESULTS: Heterozygous multiple exonic deletions of KCNQ2 were identified in 4 of 22 patients with BFNS. Concomitant deletions of adjacent genes, including nicotinic cholinergic receptor alpha4 (CHRNA4), were detected in 2 of the 4 cases. The clinical courses of patients with deletions of both KCNQ2 and CHRNA4 were those of typical BFNS, and none presented with the phenotype of autosomal dominant nocturnal frontal lobe epilepsy, some of which are caused by mutations of CHRNA4. CONCLUSIONS: Our findings indicate that the clinical courses of patients with deletions of both KCNQ2 and CHRNA4 are indistinguishable from those of patients with deletions of KCNQ2 only.

Professional healthcare use for life-threatening obstetric conditions.

Obstetric complications are the major causes of death and disability in women of reproductive age. Our study aimed at investigating the use of professional healthcare when women in communities recognised possible life-threatening obstetric conditions (PLTCs). We conducted a survey in a Cambodian district with a population of 130,000. The subjects were women of reproductive age who had delivered babies during a 3-month period prior to the survey. We interviewed 980 women, and 141 (14.4%) of these had PLTCs. The utilisation rates of professional healthcare were 47% for prolonged labour; 42% for bleeding during the delivery and puerperal period; 33% for antenatal bleeding; 25% for convulsion; and 23% for postpartum high fever. A logistic regression analysis revealed that education, geographic accessibility and parity were significant determinants of seeking healthcare. Two additional determinants, namely, economic affluence and antenatal care attendance, were identified in the socioeconomic status (SES) and obstetric models.

High titre of anti-Ascaris immunoglobulin E associated with bronchial asthma symptoms in 5-year-old rural Bangladeshi children.

BACKGROUND: Increasing interest has arisen whether helminthic infections protect against asthma and allergy. The prevalence of wheezing among Bangladeshi children is higher in rural areas where helminthic infectious burden is greater, which is contrary to the general assumption. OBJECTIVE: We therefore examined the association between Ascaris infection, serum level of anti-Ascaris IgE, which should be investigated differently from the infection, and wheezing in 5-year-old children from rural Bangladesh. METHODS: A total of 219 children who reported wheezing during the previous 12 months and 122 randomly selected age-matched individuals who had never experienced wheezing were tested for serum levels of total and specific Ascaris, Dermatophagoides pteronyssinus, alternaria and cockroach IgEs, and for intestinal helminth infection as well. RESULTS: Anti-Ascaris IgE levels were significantly and independently associated with current wheezing during the previous 12 months [odds ratio (OR) per log(e) increment is 1.31 (95% confidence interval (CI) 1.08-1.60), P=0.007], a history of at least four episodes of wheezing [OR per log(e) increment is 1.52 (95% CI 1.18-1.96), P=0.001], wheezing with sleep disturbances [OR per log(e) increment is 1.35 (95% CI 1.10-1.64), P=0.011] and wheezing with speech disturbances [OR per log(e) increment is 1.57 (95% CI 1.19-2.08), P=0.001]. These were adjusted for gender, pneumonia history, parental asthma, Trichuris infection, use of dry leaves as fuel and other specific IgE levels. The prevalence of Ascaris infection by the presence of wheezing was not significantly different (76% vs. 72%, respectively). CONCLUSION: We conclude that a high titre of anti-Ascaris IgE is associated with an increased risk of asthma symptoms among 5-year-old rural Bangladeshi children with a high helminthic infectious load.

Magazine information on safety belt use for pregnant women and young children.

To assess the information on safety belt use for pregnant women and infants in maternity and baby magazines, we reviewed 2003 issues of the three top-selling monthly baby and maternity magazines. Articles on safety belt use during pregnancy and child safety seat (CSS) use for infants, and advertisements of the CSS, automobiles, or CSS-related products were examined for their informational content. Of the 9418 pages in the maternity magazines, 46 pages were on infants, 16 pages on pregnant women and 162 pages displayed advertisements. Of the 9750 pages in the baby magazines, 34 pages of articles addressed CSS use; no articles addressed the issue of pregnant women; 83 pages were advertisements. Although the magazines showed accurate information in general; its amount was not sufficient, and little specific information on appropriate use and selection of a CSS was seen. Some information was misleading and even incorrect: specifically related to when to start using a forward-facing seat, what products are attachable to the CSS, and the reuse of a CSS. Greater amounts and accuracy of safety information in these magazines would help pregnant women and parents acquire important safety knowledge that healthcare providers are falling short of providing in Japan.

Unintentional injuries among school adolescents in Kathmandu, Nepal: a descriptive study.

OBJECTIVE: To describe detailed mechanisms and activities at the time of unintentional injuries among adolescents in Kathmandu, Nepal. METHODS: A school-based study was conducted on 1557 students in grades 6-8 across 14 schools, using a self-administered questionnaire during August-September 2003. Mechanisms and activities were coded using the Nordic Medico Statistical Committee's classification. FINDINGS: Falls (65%) and cuts (63%) were the most common injuries, followed by transport-related injuries (36%) and burns (22%). In urban areas, falls (59%) were the most common injuries, followed by transport-related injuries (50%); in semi-urban areas, falls (66%) and cuts (65%) were the most common injuries, followed by transport-related injuries (34%). At the time of falls, boys were generally engaged in sports while girls were engaged in walking on streets or on stairs in houses, in both areas. Falls from trees among boys and falls from roofs among girls were also common in semi-urban areas. Cut injuries while processing food were common among boys and girls in both areas, whereas agricultural work was also a source of injury in semi-urban areas. Pedestrian injuries were common during transport among boys and girls in both areas; boys in semi-urban areas were almost equally likely to be injured during cycling. Burn injuries while cooking and serving food were more common among girls in semi-urban areas. CONCLUSION: Falls, cuts, transport-related injuries, and burn injuries were quite prevalent among adolescents in Kathmandu. The reported injury mechanisms and activities posing injury risks have implications for future interventions.

Oral health status of vulnerable groups in a village of the Central Highlands, southern Vietnam.

OBJECTIVES: Oral health status of vulnerable people in developing countries tends to be given lower priority than other health issues. Consequently, few studies have examined the oral health status of the poor and minorities in developing countries. We aim to examine the dental caries and periodontal status, and explore the risk indicators of dental caries between two ethnic groups in rural villages in southern Vietnam. METHODS: We examined the caries status and its risk indicators of 150 participants (Co-Ho minority and Kinh majority) living in a hamlet of Dangphuong village in Vietnam. We also assessed periodontal status of the participants aged 14 and over by Community Periodontal Index. RESULTS: We first found that dental caries were highly prevalent among both the Co-Ho minority and Kinh majority groups. Second, the higher numbers of dental caries among children with primary teeth were associated with a higher frequency of consuming sweets. Third, most people (87%) aged 14 and over had periodontal problems. Finally, the Kinh majority tended to have more dental caries than Co-Ho among people aged less than 30. CONCLUSION: Oral health promotion should be considered as a part of the development programmes for vulnerable groups in Vietnam and other developing countries.

Nutritional status and associated factors in children aged 0-23 months in Granada, Nicaragua.

OBJECTIVES: Nicaragua is one of two low-income countries in the Latin Americas. The objectives of this study were to clarify the nutritional status of children aged 0-23 months and to identify the associated factors in rural Nicaragua. STUDY DESIGN AND METHODS: This was a cross-sectional study; a questionnaire survey was performed and anthropometric measurements were taken in Granada province, Nicaragua. RESULTS: Seven hundred and fifty-six mothers with at least one child aged 0-23 months took part in this study. The proportion of underweight children [weight-for-age Z-score <-2 standard deviations (SD)] was 10.3%, while 30.1% of children exhibited stunted growth (height-for-age Z-score <-2 SD) and 5.0% were wasted (weight-for-height Z-score <-2 SD). Mothers' illiteracy or lack of formal education [odds ratio (OR)=3.476, P<0.01], a history of respiratory infection (OR=1.821, P<0.05) and a birth weight below the median (<3000 g; OR=1.704, P<0.05) were identified as risk factors for stunted growth. Breastfeeding for more than 12 months (OR=2.031, P<0.01), absence of participation in child growth monitoring (OR=1.956, P<0.05) and female gender of the child (OR=1.884, P<0.05) were identified as risk factors for underweight. DISCUSSION: Despite the steady economic development in Nicaragua since 1990, child nutritional status has not improved as much as might be expected. The present study revealed that a mother's favourable sociodemographic characteristics and her active participation in health activities, such as child growth monitoring, can protect against child malnutrition, particularly against underweight. More attractive health education or antenatal care programmes might lead to substantial improvements in maternal and child health in this small, poor community.

Use of rapid diagnostic tests for malaria in an emergency situation after the flood disaster in Mozambique.

OBJECTIVES: To determine how diagnosis of malaria may be improved by combining the use of rapid diagnostic tests (RDTs) for Plasmodium falciparum malaria with clinical diagnosis by the presence or history of fever compared with clinical diagnosis alone in emergency situations with flood-affected displaced populations in Mozambique. STUDY DESIGN: A cross-sectional study conducted at the emergency outpatient clinic in a village in the Chòkwè district of Gaza Province, 3 weeks after Cyclone Eline hit Mozambique in February 2000. METHODS: A hundred and thirty children aged less than 15 years with clinical malaria were selected for examination by RDT and fluorescent microscopy using acridine orange as a reference test. The diagnosis of clinical malaria was made by a history of fever in the last three days or axillary temperature above 37.0 degrees C at the time of attending the emergency outpatient clinic. Two positive predictive values were calculated; RDTs combined with clinical diagnosis and clinical diagnosis alone. RESULTS: Positive predictive values of RDTs combined with clinical diagnosis by the presence of fever or history of fever were 87.6% (92/105) (95% confidence interval (CI) 80.8-92.8) compared with 74.6% (97/130) (95% CI 66.2-81.8) for clinical diagnosis alone. Five patients were diagnosed false negative. CONCLUSION: RDTs combined with clinical diagnosis has sufficient positive predictive value to be used in emergency situations, while RDTs could result in increasing failure to treat when they are used for decisions of treatment compared with clinical diagnosis alone.

Traditional healers' roles on eye care services in Nepal.

AIM: To evaluate the traditional healer (TH) training programme carried out by Nepal Netra Jyoti Sangh, a non-governmental organisation in Nepal, by measuring the changes in knowledge and practices of trained THs in providing primary eye care services. METHODS: 103 trained THs practising in six districts of Nepal were interviewed with a semistructured questionnaire. Their knowledge about various illnesses and eye care practices were compared before and after the training. RESULTS: A significant change in the number of THs with accurate perceived knowledge about trachoma (28.2% v 70.9%, p<0.0001) and cataract (54.4% v 94.2%, p<0.0001) was found after the training. In total, 98 (95%) THs stopped using traditional eye medicines after receiving the training (p<0.0001). The referral practices of THs improved significantly after the training (15% v 100%, p<0.0001). After the training, 95% of the THs used an eye care kit to treat patients with red eyes and simple ocular trauma. CONCLUSION: The findings show that a TH training programme on primary eye care services convinced traditional healers to stop the use of traditional eye medicines and improve referral practices in Nepal.

Geographical characterization of the triatomine infestations in north-central Guatemala.

In an entomological study in 2002, the degree of domestic and peridomestic infestation with triatomine bugs and the geographical distribution of such infestations were investigated in north-central Guatemala. The survey team searched for triatomines in houses constructed with mud walls or thatched roofs, in villages suspected of being infested. The level of infestation observed was lower than that seen in the same area and in eastern Guatemala, in a preliminary survey, 3 years earlier. Most of the infestations detected were of Triatoma dimidiata but even this species was found in <7% of the houses investigated. Infestations with Rhodnius prolixus or other potential vectors of Trypanosoma cruzi were much rarer. The generally low levels of infestation make the elimination of R. prolixus and the reduction of the domestic population of Tri. dimidiata feasible in the study area. The southern part of the study area had higher levels of domestic infestation and colonization than the north, and peridomestic infestation was highest in the south-west. Given such geographical variation in the pattern of infestation, it would seem wise to stratify the study region into areas of high, moderate and low-risk of human-triatomine contact, so that appropriate vector-control strategies can be targeted at the worst-affected areas. Regular entomological surveillance, ideally with community participation, is recommended. Analysis of the relationship between the geographical patterns of infestation and factors such as vegetation, altitude and vector migration would be useful.

Herbal medicine use and quality of life among people living with HIV/AIDS in northeastern Thailand.

Many people living with HIV/AIDS (PHA) use herbal medicine as one of alternative therapies, where curative options are limited. This study aimed to examine the association between the herbal medicine use and quality of life (QOL) among PHA in northeastern Thailand. Participants were 132 HIV-positive Thai adults who attended the PHA's self-help group meetings from June to July 2002. Health-related QOL scores were measured by self-administered questionnaire from the Medical Outcomes Study-HIV Health Survey. Dimensions of physical function (PF) and mental health (MH) in QOL were assessed. Additional data were collected on herbal medicine use, socio-demographic, psychosocial and HIV-related characteristics. The herbal medicine users had significantly better MH scores than the non-users, while the herbal medicine use was not statistically associated with PF scores. When stratified, herbal medicine users with the following characteristics had significantly better MH scores than the non-users: female, widowed, having no income, reporting any HIV-related symptom, having no instrumental support or receiving subsidies. In conclusion, herbal medicine use was associated with better MH especially among socially vulnerable PHA. This study suggests that herbal medicine has a potential to improve the MH aspect of QOL among socially vulnerable PHA who cannot easily receive antiretroviral therapy in Thailand.