Long-term results after Boston brace treatment in late-onset juvenile and adolescent idiopathic scoliosis

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Gunderson Ragnhild

2011-08-01

Full Text Available Abstract Background It is recommended that research in patients with idiopathic scoliosis should focus on short- and long-term patient-centred outcome. The aim of the present study was to evaluate outcome in patients with late-onset juvenile or adolescent idiopathic scoliosis 16 years or more after Boston brace treatment. Methods 272 (78% of 360 patients, 251 (92% women, responded to follow-up examination at a mean of 24.7 (range 16 - 32 years after Boston brace treatment. Fifty-eight (21% patients had late-onset juvenile and 214 had adolescent idiopathic scoliosis. All patients had clinical and radiological examination and answered a standardised questionnaire including work status, demographics, General Function Score (GFS (100 - worst possible and Oswestry Disability Index (ODI (100 - worst possible, EuroQol (EQ-5D (1 - best possible, EQ-VAS (100 - best possible, and Scoliosis Research Society - 22 (SRS - 22 (5 - best possible. Results The mean age at follow-up was 40.4 (31-48 years. The prebrace major curve was in average 33.2 (20 - 57°. At weaning and at the last follow-up the corresponding values were 28.3 (1 - 58° and 32.5 (7 - 80°, respectively. Curve development was similar in patients with late-onset juvenile and adolescent start. The prebrace curve increased > 5° in 31% and decreased > 5° in 26%. Twenty-five patients had surgery. Those who did not attend follow-up (n = 88 had a lower mean curve at weaning: 25.4 (6-53°. Work status was 76% full-time and 10% part-time. Eighty-seven percent had delivered a baby, 50% had pain in pregnancy. The mean (SD GFS was 7.4 (10.8, ODI 9.3 (11.0, EQ-5D 0.82 (0.2, EQ-VAS 77.6 (17.8, SRS-22: pain 4.1 (0.8, mental health 4.1 (0.6, self-image 3.7 (0.7, function 4.0 (0.6, satisfaction with treatment 3.7 (1.0. Surgical patients had significantly reduced scores for SRS-physical function and self-image, and patients with curves ≥ 45° had reduced self-image. Conclusion Long-term results were

Juvenile-onset Sporadic Amyotrophic Lateral Sclerosis with a Frameshift FUS Gene Mutation Presenting Unique Neuroradiological Findings and Cognitive Impairment.

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Hirayanagi, Kimitoshi; Sato, Masayuki; Furuta, Natsumi; Makioka, Kouki; Ikeda, Yoshio

2016-01-01

A 24-year-old Japanese woman developed anterocollis, weakness of the proximal arms, and subsequent cognitive impairment. A neurological examination revealed amyotrophic lateral sclerosis (ALS) without a family history. Systemic muscle atrophy progressed rapidly. Cerebral MRI clearly exhibited high signal intensities along the bilateral pyramidal tracts. An analysis of the FUS gene revealed a heterozygous two-base pair deletion, c.1507-1508delAG (p.G504WfsX515). A subset of juvenile-onset familial/sporadic ALS cases with FUS gene mutations reportedly demonstrates mental retardation or learning difficulty. Our study emphasizes the importance of conducting a FUS gene analysis in juvenile-onset ALS cases, even when no family occurrence is confirmed.

Hyperparathyroidism and new onset diabetes after renal transplantation.

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Ivarsson, K M; Clyne, N; Almquist, M; Akaberi, S

2014-01-01

Secondary hyperparathyroidism persists after renal transplantation in a substantial number of patients. Primary hyperparathyroidism and secondary hyperparathyroidism are both associated with abnormalities in glucose metabolism, such as insufficient insulin release and glucose intolerance. The association of hyperparathyroidism and diabetes after renal transplantation has, as far as we know, not been studied. Our aim was to investigate whether hyperparathyroidism is associated with new-onset diabetes mellitus after transplantation (NODAT) during the first year posttransplantation. In a retrospective study, we analyzed data on patient characteristics, treatment details, and parathyroid hormone (PTH) in 245 adult nondiabetic patients who underwent renal transplantation between January 2000 and June 2011. The first year cumulative incidence of NODAT was 15%. The first serum PTH value after transplantation was above normal range in 74% of the patients. In multiple logistic regression analysis, PTH levels above twice normal range (>13.80 pmol/L) were significantly associated with NODAT (odds ratio [OR], 4.25; 95% confidence interval [CI], 1.13-15.92; P = .03) compared with PTH within normal range (≤6.9 pmol/L). Age between 45 and 65 years (OR, 2.80; 95% CI, 1.07-7.36; P = .04) compared with age hyperparathyroidism and NODAT in the first year after renal transplantation. Both conditions are common and have a negative impact on graft and patient survivals. Our results should be confirmed in prospective studies. Copyright © 2014 Elsevier Inc. All rights reserved.

Is late-onset OCD a distinct phenotype? Findings from a comparative analysis of "age at onset" groups.

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Sharma, Eesha; Sundar, A Shyam; Thennarasu, Kandavel; Reddy, Y C Janardhan

2015-10-01

Significant differences in clinical profile and comorbidity patterns have been observed between "juvenile-onset" and "adult-onset" obsessive-compulsive disorder (OCD). There is little systematic research on onset of OCD after the fourth decade. The current study aims to compare the demographic, clinical, and comorbidity patterns of patients with "juvenile-onset" (OCD. Eight hundred two consecutive patients who consulted a specialty OCD clinic at a tertiary care hospital in India were evaluated with the Mini International Neuropsychiatric Interview, the Yale-Brown Obsessive-Compulsive Scale, and the Clinical Global Impression scale. 37.4%, 57.4%, and 5.2% of patients had juvenile-, adult-, and late-onset OCD, respectively. Late-onset OCD was associated with female gender (χ2=42, pOCD in first-degree relatives (χ2=20.4, pOCD was significantly associated with female gender, collecting compulsions, and less aggressive obsessions, in comparison with adult-onset OCD. In comparison with juvenile-onset, late-onset OCD was significantly associated with female gender, presence of precipitating factors, and less aggressive obsessions, sexual obsessions, and repeating compulsions. Late-onset OCD is characterized by female gender, lesser familial loading for OCD, and presence of precipitating factors, suggesting that it may have a distinct pathophysiology compared to juvenile- and adult-onset OCD. Systematic research is required to understand the family-genetic, neuropsychological, and neurobiological correlates of late-onset OCD.

Cardiovascular assessment of asymptomatic patients with juvenile-onset localized and systemic scleroderma: 10 years prospective observation.

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Borowiec, A; Dabrowski, R; Wozniak, J; Jasek, S; Chwyczko, T; Kowalik, I; Musiej-Nowakowska, E; Szwed, H

2012-02-01

The aim of the present study was non-invasive evaluation of the cardiovascular system in asymptomatic young adult patients with juvenile localized scleroderma (JLS) and juvenile systemic sclerosis (JSS). A group of 34 consecutive children with scleroderma were prospectively observed in the study. The control group (CG) consisted of 20 healthy subjects. In each subject 12-lead electrocardiographic, echocardiographic, ECG Holter, and ambulatory blood pressure monitoring examinations were performed at the baseline visit and after 10 years. Additionally, B-type natriuretic peptide (BNP) concentrations were measured after 10 years. Examinations were performed in 13 patients with JLS and 15 with JSS at the final visit. Two children had died (one from each group). Four patients were alive but refused the final visit. After 10 years, a higher prevalence of ventricular extrasystoles (p = 0.01) and an elevated pulmonary arterial pressure (JLS: p = 0.04, JSS: p = 0.03) were observed in both groups, but in comparison with the controls there was no significant difference at the final visit. In JLS patients more cases of left ventricle diastolic dysfunction, hypertension, and sinus tachycardia were diagnosed at the final visit (p ≤ 0.05). More atrioventricular block episodes in both groups of scleroderma patients were observed. Over the 10 years, arterial hypertension was diagnosed in three patients from the JLS group and in two with JSS. There were no significant differences in BNP concentrations at the final visit. The results of the present study show that juvenile scleroderma seems to be more benign than adult-onset disease. This observational study shows subclinical, not severe, cardiac abnormalities in adult patients with juvenile-onset disease.

Late-onset renal vein thrombosis: A case report and review of the literature.

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Hogan, Jessica L; Rosenthal, Stanton J; Yarlagadda, Sri G; Jones, Jill A; Schmitt, Timothy M; Kumer, Sean C; Kaplan, Bruce; Deas, Shenequa L; Nawabi, Atta M

2015-01-01

Renal vein thrombosis, a rare complication of renal transplantation, often causes graft loss. Diagnosis includes ultrasound with Doppler, and it is often treated with anticoagulation or mechanical thrombectomy. Success is improved with early diagnosis and institution of treatment. We report here the case of a 29 year-old female with sudden development of very late-onset renal vein thrombosis after simultaneous kidney pancreas transplant. This resolved initially with thrombectomy, stenting and anticoagulation, but thrombosis recurred, necessitating operative intervention. Intraoperatively the renal vein was discovered to be compressed by a large ovarian cyst. Compression of the renal vein by a lymphocele or hematoma is a known cause of thrombosis, but this is the first documented case of compression and thrombosis due to an ovarian cyst. Early detection and treatment of renal vein thrombosis is paramount to restoring renal allograft function. Any woman of childbearing age may have thrombosis due to compression by an ovarian cyst, and screening for this possibility may improve long-term graft function in this population. Published by Elsevier Ltd.

Consensus Treatment Plans for New-Onset Systemic Juvenile Idiopathic Arthritis

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DeWitt, Esi Morgan; Kimura, Yukiko; Beukelman, Timothy; Nigrovic, Peter A.; Onel, Karen; Prahalad, Sampath; Schneider, Rayfel; Stoll, Matthew L.; Angeles-Han, Sheila; Milojevic, Diana; Schikler, Kenneth N.; Vehe, Richard K.; Weiss, Jennifer E.; Weiss, Pamela; Ilowite, Norman T.; Wallace, Carol A.

2012-01-01

Objective There is wide variation in therapeutic approaches to systemic juvenile idiopathic arthritis (sJIA) among North American rheumatologists. Understanding the comparative effectiveness of the diverse therapeutic options available for treatment of sJIA can result in better health outcomes. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed consensus treatment plans and standardized assessment schedules for use in clinical practice to facilitate such studies. Methods Case-based surveys were administered to CARRA members to identify prevailing treatments for new-onset sJIA. A 2-day consensus conference in April 2010 employed modified nominal group technique to formulate preliminary treatment plans and determine important data elements for collection. Follow-up surveys were employed to refine the plans and assess clinical acceptability. Results The initial case-based survey identified significant variability among current treatment approaches for new onset sJIA, underscoring the utility of standardized plans to evaluate comparative effectiveness. We developed four consensus treatment plans for the first 9 months of therapy, as well as case definitions and clinical and laboratory monitoring schedules. The four treatment regimens included glucocorticoids only, or therapy with methotrexate, anakinra or tocilizumab, with or without glucocorticoids. This approach was approved by >78% of CARRA membership. Conclusion Four standardized treatment plans were developed for new-onset sJIA. Coupled with data collection at defined intervals, use of these treatment plans will create the opportunity to evaluate comparative effectiveness in an observational setting to optimize initial management of sJIA. PMID:22290637

Acute renal response to rapid onset respiratory acidosis.

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Ramadoss, Jayanth; Stewart, Randolph H; Cudd, Timothy A

2011-03-01

Renal strong ion compensation to chronic respiratory acidosis has been established, but the nature of the response to acute respiratory acidosis is not well defined. We hypothesized that the response to acute respiratory acidosis in sheep is a rapid increase in the difference in renal fractional excretions of chloride and sodium (Fe(Cl) - Fe(Na)). Inspired CO(2) concentrations were increased for 1 h to significantly alter P(a)CO(2) and pH(a) from 32 ± 1 mm Hg and 7.52 ± 0.02 to 74 ± 2 mm Hg and 7.22 ± 0.02, respectively. Fe(Cl) - Fe(Na) increased significantly from 0.372 ± 0.206 to 1.240 ± 0.217% and returned to baseline at 2 h when P(a)CO(2) and pH(a) were 37 ± 0.6 mm Hg and 7.49 ± 0.01, respectively. Arterial pH and Fe(Cl) - Fe(Na) were significantly correlated. We conclude that the kidney responds rapidly to acute respiratory acidosis, within 30 min of onset, by differential reabsorption of sodium and chloride.

Síndrome de ativação macrofágica em pacientes com artrite idiopática juvenil Macrophage activation syndrome in patients with juvenile idiopathic arthritis

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Rogério do Prado

2004-10-01

Full Text Available A síndrome de ativação macrofágica (SAM é uma complicação rara das doenças reumáticas crônicas, particularmente a artrite idiopática juvenil (AIJ de início sistêmico. Este processo pode ser desencadeado por agentes infecciosos virais e bacterianos, neoplásicos, drogas antiinflamatórias não esteroidais ou drogas modificadoras da doença, mudanças abruptas das medicações e doenças reumáticas. O quadro clínico inicia-se com irritação do sistema nervoso central, acompanhado de falências hepática e renal, além de pancitopenia. Relatamos três casos de pacientes com AIJ do nosso serviço que desenvolveram SAM com descrição das características clínicas, evolutivas e de tratamento.The macrophage activation syndrome (MAS is an uncommon complication of chronic rheumatic diseases, specially systemic onset juvenile idiopathic arthritis (JIA. It can be triggered by infectious (viral or bacterial or malignant diseases, non-steroidal anti-inflammatory or disease modified anti-rheumatic drugs, changes in the therapy and rheumatic diseases. The clinical features present at the onset are related mainly with central nervous system involvement, hepatic and renal failure and pancytopenia. We describe the clinical, evolutive features and treatment of three patients with JIA that developed MAS.

The Evolution of Juvenile Schönlein-Henoch Purpura

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O.E. Chernyshova

2016-10-01

Full Text Available Background. Hemorrhagic vasculitis, or Schönlein-Henoch purpura (SHP, is the most common type of systemic vasculitis in childhood, and peculiarities of the further evolution of the pathological process in adult patients remain unexplored. Objective: to study the evolution of juvenile SHP, comparing the nature of lesions of the skin, joints, heart and kidneys in patients in childhood and adulthood. Material and methods. The study included 92 patients (61 men and 31 women with the average age of 27 years, and the average age of disease onset — 11 years. I degree of the activity of the pathological process is determined in 40 % of cases, II — in 35 %, III — in 25 %. Seropositivity by hyperimmunoglobulinemia A occurred in 27 % of cases, by the presence of rheumatoid factor — in 21 %. At the time of the survey, cutaneous syndrome was diagnosed in 55 % of patients, joint one — in 45 %, kidney one — in 71 %. Renal biopsy was performed in 15 cases. Results. The cutaneous, cutaneous-joint-abdominal and cutaneous-abdominal-renal forms of the disease, lesions of the skin, gastrointestinal tract, wrist, ankle and knee joints become rarer in the process of juvenile SHP evolution, but exceptionally renal variant of the pathological process, changes of skeletal muscles, liver, spleen and the heart are revealed more often, chronic kidney disease with the renal failure progression is developed in 12 % of patients (in 17 % of cases of nephropathy, sacroiliitis, spondylopathy, tendovaginitis, enthesopathies, epiphyseal osteoporosis and meniscitis of the knee joints are arisen, II, III, VI and IV morphological classes of Henoch glomerulonephritis are formed in a ratio of 8 : 4 : 2 : 1 with tubulointerstitial component in all cases, and lymphohistiocytic infiltration of the vascular wall is the unfavorable sign for the prognosis of the disease. Conclusions. In cases of transition of juvenile SHP into the chronic adult form, the disease often obtain

Late Onset Juvenile Xanthogranuloma

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Punithwavathy K

1999-01-01

Full Text Available A 19 year old female was seen with multiple skin coloured and hyperpigmented macules, discrete as well as grouped papules and nodules of varying sizes distributed over the face, neck, extensor and flexor aspects of both upper and lower extremities including joints. The trunk was spared. Some of the lesions showed features of spontaneous regression. Investigations confirmed the diagnosis of juvenile xanthogranuloma. Lesions regressed satisfactorily with liquid nitrogen cryotherapy.

Relationship between histopathological changes in post partum renal biopsies and renal function tests of African women with early onset pre-eclampsia.

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Khedun, S M; Naicker, T; Moodley, J

2000-05-01

To improve the diagnostic accuracy of concurrent renal disease in hypertension of pregnancy, biopsy evaluation is essential. In addition, establishing underlying renal disease is important for prognosis on future pregnancies. We therefore designed a study to determine the diagnostic yield of postpartum renal biopsy and the nature and frequency of complications associated with this procedure. Also, to determine relationships, if any, between renal function tests and ultrastructural and histopathological findings. Fifty renal biopsies were performed in the immediate postpartum period in black African women with early onset pre-eclampsia. Each biopsy specimen was placed in a separate container and coded so that sampling was unknown to the electron microscopist. Each biopsy specimen was divided into three parts, and processed and stained for light, fluorescent and transmission electron microscopy using conventional techniques. Renal tissue biopsies were adequate for diagnostic purposes in all cases. There were no complications in any of the 50 patients studied. Ultrastructural examination confirmed the light microscopy findings. In addition the ultrastructural findings showed intramembranous deposits, foot process fusion and mesangial deposits. In 16 patients with normal renal function tests; the biopsies evaluation from these patients showed ultrastructural changes. In the remaining 34 patients with abnormal renal function tests of varying severity; biopsy evaluation from these patients showed both ultrastructural and histopathological changes. Renal biopsy procedure is safe, and ultrastructural and histological findings obtained from postpartum renal biopsies are more informative than the routine renal function tests.

Regulation of renal NaPi-2 expression and tubular phosphate reabsorption by growth hormone in the juvenile rat.

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Woda, Craig B; Halaihel, Nabil; Wilson, Paul V; Haramati, Aviad; Levi, Moshe; Mulroney, Susan E

2004-07-01

Growth hormone (GH) is an important factor in the developmental adaptation to enhance P(i) reabsorption; however, the nephron sites and mechanisms by which GH regulates renal P(i) uptake remain unclear and are the focus of the present study. Micropuncture experiments were performed after acute thyroparathyroidectomy in the presence and absence of parathyroid hormone (PTH) in adult (14- to 17-wk old), juvenile (4-wk old), and GH-suppressed juvenile male rats. While the phosphaturic effect of PTH was blunted in the juvenile rat compared with the adult, suppression of GH in the juvenile restored fractional P(i) excretion to adult levels. In the presence or absence of PTH, GH suppression in the juvenile rat caused a significant increase in the fractional P(i) delivery to the late proximal convoluted (PCT) and early distal tubule, so that delivery was not different from that in adults. These data were confirmed by P(i) uptake studies into brush-border membrane (BBM) vesicles. Immunofluorescence studies indicate increased BBM type IIa NaP(i) cotransporter (NaPi-2) expression in the juvenile compared with adult rat, and GH suppression reduced NaPi-2 expression to levels observed in the adult. GH replacement in the [N-acetyl-Tyr(1)-d-Arg(2)]-GRF-(1-29)-NH(2)-treated juveniles restored high NaPi-2 expression and P(i) uptake. Together, these novel results demonstrate that the presence of GH in the juvenile animal is crucial for the early developmental upregulation of BBM NaPi-2 and, most importantly, describe the enhanced P(i) reabsorption along the PCT and proximal straight nephron segments in the juvenile rat.

Mediterranean style diet is associated with low risk of new-onset diabetes after renal transplantation

NARCIS (Netherlands)

Osté, Maryse C J; Corpeleijn, Eva; Navis, Gerjan J; Keyzer, Charlotte A; Soedamah-Muthu, S.S.; Van Den Berg, Else; Postmus, Douwe; De Borst, Martin H; Kromhout, Daan; Bakker, Stephan J L

2017-01-01

Objective The incidence of new-onset diabetes after transplantation (NODAT) and premature mortality is high in renal transplant recipients (RTR). We hypothesized that a Mediterranean Style diet protects against NODAT and premature mortality in RTR. Research design and methods A prospective cohort

Mediterranean style diet is associated with low risk of new-onset diabetes after renal transplantation

NARCIS (Netherlands)

Osté, Maryse C J; Corpeleijn, Eva; Navis, Gerjan J; Keyzer, Charlotte A; Soedamah-Muthu, Sabita S; van den Berg, Else; Postmus, Douwe; de Borst, Martin H; Kromhout, Daan; Bakker, Stephan J L

2017-01-01

OBJECTIVE: The incidence of new-onset diabetes after transplantation (NODAT) and premature mortality is high in renal transplant recipients (RTR). We hypothesized that a Mediterranean Style diet protects against NODAT and premature mortality in RTR. RESEARCH DESIGN AND METHODS: A prospective cohort

Condyloma in pregnancy is strongly predictive of juvenile-onset recurrent respiratory papillomatosis

DEFF Research Database (Denmark)

Silverberg, Michael J.; Thorsen, Poul; Lindeberg, Henning

2003-01-01

OBJECTIVE: To assess the risk of juvenile-onset recurrent respiratory papillomatosis conferred by a maternal history of genital warts in pregnancy, and to identify additional cofactors such as the method of delivery (cesarean versus vaginal) and procedures or complications during pregnancy. METHODS......: A retrospective cohort design was used to evaluate maternal and infant characteristics associated with respiratory papillomatosis among Danish births between 1974 and 1993. Using data from Danish registries, we identified 3033 births with a maternal history of genital warts during pregnancy. Fifty......-seven respiratory papillomatosis cases were identified by review of medical records from ear, nose, and throat departments. RESULTS: Seven of every 1000 births with a maternal history of genital warts resulted in disease in the offspring, corresponding to a 231.4 (95% confidence interval 135.3, 395.9) times higher...

Clinical presentation of juvenile Huntington disease

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Ruocco Heloísa H.

2006-01-01

Full Text Available OBJECTIVE: To describe the clinical presentation a group of patients with juvenile onset of Huntington disease. METHOD: All patients were interviewed following a structured clinical questioner. Patients were genotyped for the trinucleotide cytosine-adenine-guanine (CAG repeat in the Huntington Disease gene. High resolution brain MRI was performed in all patients. RESULTS: We identified 4 patients with juvenile onset of disease among 50 patients with Huntington disease followed prospectively in our Neurogenetics clinic. Age at onset varied from 3 to 13 years, there were 2 boys, and 3 patients had a paternal inheritance of the disease. Expanded Huntington disease allele sizes varied from 41 to 69 trinucleotide repeats. The early onset patients presented with rigidity, bradykinesia, dystonia, dysarthria, seizures and ataxia. MRI showed severe volume loss of caudate and putamen nuclei (p=0.001 and reduced cerebral and cerebellum volumes (p=0.01. CONCLUSION: 8% of Huntington disease patients seen in our clinic had juvenile onset of the disease. They did not present with typical chorea as seen in adult onset Huntington disease. There was a predominance of rigidity and bradykinesia. Two other important clinical features were seizures and ataxia, which related with the imaging findings of early cortical atrophy and cerebellum volume loss.

Brief Report: Association of Myositis Autoantibodies, Clinical Features, and Environmental Exposures at Illness Onset With Disease Course in Juvenile Myositis.

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Habers, G Esther A; Huber, Adam M; Mamyrova, Gulnara; Targoff, Ira N; O'Hanlon, Terrance P; Adams, Sharon; Pandey, Janardan P; Boonacker, Chantal; van Brussel, Marco; Miller, Frederick W; van Royen-Kerkhof, Annet; Rider, Lisa G

2016-03-01

To identify early factors associated with disease course in patients with juvenile idiopathic inflammatory myopathies (IIMs). Univariable and multivariable multinomial logistic regression analyses were performed in a large juvenile IIM registry (n = 365) and included demographic characteristics, early clinical features, serum muscle enzyme levels, myositis autoantibodies, environmental exposures, and immunogenetic polymorphisms. Multivariable associations with chronic or polycyclic courses compared to a monocyclic course included myositis-specific autoantibodies (multinomial odds ratio [OR] 4.2 and 2.8, respectively), myositis-associated autoantibodies (multinomial OR 4.8 and 3.5), and a documented infection within 6 months of illness onset (multinomial OR 2.5 and 4.7). A higher overall clinical symptom score at diagnosis was associated with chronic or monocyclic courses compared to a polycyclic course. Furthermore, severe illness onset was associated with a chronic course compared to monocyclic or polycyclic courses (multinomial OR 2.1 and 2.6, respectively), while anti-p155/140 autoantibodies were associated with chronic or polycyclic courses compared to a monocyclic course (multinomial OR 3.9 and 2.3, respectively). Additional univariable associations of a chronic course compared to a monocyclic course included photosensitivity, V-sign or shawl sign rashes, and cuticular overgrowth (OR 2.2-3.2). The mean ultraviolet index and highest ultraviolet index in the month before diagnosis were associated with a chronic course compared to a polycyclic course in boys (OR 1.5 and 1.3), while residing in the Northwest was less frequently associated with a chronic course (OR 0.2). Our findings indicate that myositis autoantibodies, in particular anti-p155/140, and a number of early clinical features and environmental exposures are associated with a chronic course in patients with juvenile IIM. These findings suggest that early factors, which are associated with poorer

Integration of HPV6 and downregulation of AKR1C3 expression mark malignant transformation in a patient with juvenile-onset laryngeal papillomatosis.

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Christian Ulrich Huebbers

Full Text Available Juvenile-onset recurrent respiratory papillomatosis (RRP is associated with low risk human papillomavirus (HPV types 6 and 11. Malignant transformation has been reported solely for HPV11-associated RRP in 2-4% of all RRP-cases, but not for HPV6. The molecular mechanisms in the carcinogenesis of low risk HPV-associated cancers are to date unknown. We report of a female patient, who presented with a laryngeal carcinoma at the age of 24 years. She had a history of juvenile-onset RRP with an onset at the age of three and subsequently several hundred surgical interventions due to multiple recurrences of RRP. Polymerase chain reaction (PCR or bead-based hybridization followed by direct sequencing identified HPV6 in tissue sections of previous papilloma and the carcinoma. P16(INK4A, p53 and pRb immunostainings were negative in all lesions. HPV6 specific fluorescence in situ hybridization (FISH revealed nuclear staining suggesting episomal virus in the papilloma and a single integration site in the carcinoma. Integration-specific amplification of papillomavirus oncogene transcripts PCR (APOT-PCR showed integration in the aldo-keto reductase 1C3 gene (AKR1C3 on chromosome 10p15.1. ArrayCGH detected loss of the other gene copy as part of a deletion at 10p14-p15.2. Western blot analysis and immunohistochemistry of the protein AKR1C3 showed a marked reduction of its expression in the carcinoma. In conclusion, we identified a novel molecular mechanism underlying a first case of HPV6-associated laryngeal carcinoma in juvenile-onset RRP, i.e. that HPV6 integration in the AKR1C3 gene resulted in loss of its expression. Alterations of AKR1C gene expression have previously been implicated in the tumorigenesis of other (HPV-related malignancies.

The role of heme oxygenase-1 in systemic-onset juvenile idiopathic arthritis.

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Takahashi, Akitaka; Mori, Masaaki; Naruto, Takuya; Nakajima, Shoko; Miyamae, Takako; Imagawa, Tomoyuki; Yokota, Shumpei

2009-01-01

We have determined the serum levels of heme oxygenase-1 (HO-1) in 56 patients with systemic-onset juvenile idiopathic arthritis (s-JIA) and compared these with serum HO-1 levels in healthy controls and patients with other pediatric rheumatic diseases. Serum HO-1 levels were measured by the sandwich enzyme-linked immunosorbent assay. The mean serum HO-1 level in s-JIA patients during the active phase was 123.6 +/- 13.83 ng/ml, which was significantly higher than that in patients with polyarticular juvenile idiopathic arthritis (p-JIA), Kawasaki disease, systemic lupus erythematosus or mixed connective tissue disease (P < 0.0005). The serum levels of HO-1, cytokines and cytokine receptors in patients with s-JIA were also assessed at both the active and inactive phases. The serum HO-1 level in patients with s-JIA in the active phase was found to be significantly greater than that in patients with the disease in the inactive phase (P < 0.0001). An assessment of the relationships between serum HO-1 levels and other laboratory parameters or cytokines in patients with s-JIA did not reveal any strong correlations. These results suggest that the serum level of HO-1 may be a useful marker for the differential diagnosis of s-JIA. Further study will be necessary to elucidate the mechanism of HO-1 production and to clarify the role of HO-1 in the disease process.

Association of STAT4 gene single nucleotide polymorphisms with Iranian juvenile-onset systemic lupus erythematosus patients.

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Salmaninejad, Arash; Mahmoudi, Mahdi; Aslani, Saeed; Poursani, Shiva; Ziaee, Vahid; Rezaei, Nima

2017-01-01

Salmaninejad A, Mahmoudi M, Aslani S, Poursani S, Ziaee V, Rezaei N. Association of STAT4 gene single nucleotide polymorphisms with Iranian juvenile-onset systemic lupus erythematosus patients. Turk J Pediatr 2017; 59: 144-149. Juvenile-onset systemic lupus erythematosus (JSLE) is a complex autoimmune disease, characterized by multi-organ involvement. Single nucleotide polymorphisms (SNPs) of signal transducer and activator of transcription 4 (STAT4) gene have been reported to have relationship with the risk of several autoimmune diseases. Studies have provided evidence that STAT4 may participate in the pathogenesis of JSLE. Therefore, we aimed to evaluate the association of STAT4 SNPs with JSLE in Iranian population. In this case-control study, two SNPs of STAT4 gene, including rs7574865 and rs7601754 were genotyped in 50 Iranian JSLE patients and 281 matched healthy individuals using real-time PCR allelic discrimination approach. Our experiments demonstrated that G and T alleles of rs7574865 SNP had similar distribution between patients and controls (P = 0.16). Additionally, differences in frequency of GG, GT, and TT genotypes (P = 0.14, 0.29, and 0.54, respectively) were not significant. Likewise, A and G alleles, as well as genotypes of rs7601754 SNP did not show significant differences between JSLE patients and healthy individuals. Lack of association of rs7574865 and rs7601754 SNPs in STAT4 gene with susceptibility to JSLE in Iranian population, despite their association with the risk of adult SLE in the same population, implicates on difference of genetic background of JSLE and SLE.

Reversible posterior leukoencephalopathy syndrome secondary to systemic-onset juvenile idiopathic arthritis: A case report and review of the literature

OpenAIRE

ZHANG, PINGPING; LI, XIAOFENG; LI, YATING; WANG, JING; ZENG, HUASONG; ZENG, XIAOFENG

2014-01-01

Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinical syndrome based on changes in clinical imaging, and it has been reported to mainly occur in adults. However, it has been recently discovered that RPLS is also prevalent in infant patients, particularly in those using glucocorticoids, immunosuppressant medications and cytotoxic drugs. The current study presents a 5-year-old male with a previous diagnosis of systemic-onset juvenile idiopathic arthritis (SoJIA) and macrophage-...

Adult-onset offenders: Is a tailored theory warranted?

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Beckley, Amber L.; Caspi, Avshalom; Harrington, Honalee; Houts, Renate M.; Mcgee, Tara Renae; Morgan, Nick; Schroeder, Felix; Ramrakha, Sandhya; Poulton, Richie; Moffitt, Terrie E.

2016-01-01

Purpose To describe official adult-onset offenders, investigate their antisocial histories and test hypotheses about their origins. Methods We defined adult-onset offenders among 931 Dunedin Study members followed to age 38, using criminal-court conviction records. Results Official adult-onset offenders were 14% of men, and 32% of convicted men, but accounted for only 15% of convictions. As anticipated by developmental theories emphasizing early-life influences on crime, adult-onset offenders’ histories of antisocial behavior spanned back to childhood. Relative to juvenile-offenders, during adolescence they had fewer delinquent peers and were more socially inhibited, which may have protected them from conviction. As anticipated by theories emphasizing the importance of situational influences on offending, adult-onset offenders, relative to non-offenders, during adulthood more often had schizophrenia, bipolar disorder, and alcohol-dependence, had weaker social bonds, anticipated fewer informal sanctions, and self-reported more offenses. Contrary to some expectations, adult-onset offenders did not have high IQ or high socioeconomic-status families protecting them from juvenile conviction. Conclusions A tailored theory for adult-onset offenders is unwarranted because few people begin crime de novo as adults. Official adult-onset offenders fall on a continuum of crime and its correlates, between official non-offenders and official juvenile-onset offenders. Existing theories can accommodate adult-onset offenders. PMID:27134318

Syndrome of rapid onset end stage renal disease in incident Mayo Clinic chronic hemodialysis patient

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M. A. C. Onuigbo

2014-01-01

Full Text Available Despite decades of research, a full understanding of chronic kidney disease (CKD-end stage renal disease (ESRD progression remains elusive. The common consensus is a predictable, linear, progressive and time-dependent decline of CKD to ESRD. Acute kidney injury (AKI on CKD is usually assumed to be transient, with recovery as the expected outcome. AKI-ESRD association in current nephrology literature is blamed on the so-called "residual confounding." We had previously described a relationship between AKI events and rapid onset yet irreversible ESRD happening in a continuum in a high-risk CKD cohort. However, the contribution of the syndrome of rapid onset-ESRD (SORO-ESRD to incident United States ESRD population remained conjectural. In this retrospective analysis, we analyzed serum creatinine trajectories of the last 100 consecutive ESRD patients in 4 Mayo Clinic chronic hemodialysis units to determine the incidence of SORO-ESRD. Excluding 9 patients, 31 (34% patients, including two renal transplant recipients, had SORO-ESRD: 18 males and 13 females age 72 (range 50-92 years. Precipitating AKI followed pneumonia (8, acutely decompensated heart failure (7, pyelonephritis (4, post-operative (5, sepsis (3, contrast-induced nephropathy (2, and others (2. Time to dialysis was shortest following surgical procedures. Concurrent renin angiotensin aldosterone system blockade was higher with SORO-ESRD - 23% versus 5%, P = 0.0113. In conclusion, SORO-ESRD is not uncommon among the incident general US ESRD population. The implications for ESRD care planning, AV-fistula-first programs, general CKD care and any associations with renal ageing/senescence warrant further study.

Juvenile chronic arthritis into adulthood: a long-term follow-up study

DEFF Research Database (Denmark)

Zak, M; Pedersen, F K

2000-01-01

To evaluate a group of 65 adults with a history of or persistent juvenile chronic arthritis (JCA), on average, 26.4 yr after disease onset.......To evaluate a group of 65 adults with a history of or persistent juvenile chronic arthritis (JCA), on average, 26.4 yr after disease onset....

An unusual presentation of juvenile lupus nephritis

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Malleshwar Bottu

2016-01-01

Full Text Available The incidence of juvenile lupus varies widely ranging between 4 and 250 per 100,000 population. Most common organ involvement in juvenile lupus is kidney. Neurological, cutaneous and hematological involvements are also involved. Skeletal muscle involvement in the form of myositis is rare. Myositis as presenting manifestation in juvenile lupus is also unusual. Herein, we report one such case wherein myositis preceded the onset of lupus nephritis

An ARHGEF10 deletion is highly associated with a juvenile-onset inherited polyneuropathy in Leonberger and Saint Bernard dogs.

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Kari J Ekenstedt

2014-10-01

Full Text Available An inherited polyneuropathy (PN observed in Leonberger dogs has clinical similarities to a genetically heterogeneous group of peripheral neuropathies termed Charcot-Marie-Tooth (CMT disease in humans. The Leonberger disorder is a severe, juvenile-onset, chronic, progressive, and mixed PN, characterized by exercise intolerance, gait abnormalities and muscle atrophy of the pelvic limbs, as well as inspiratory stridor and dyspnea. We mapped a PN locus in Leonbergers to a 250 kb region on canine chromosome 16 (Praw = 1.16×10-10, Pgenome, corrected = 0.006 utilizing a high-density SNP array. Within this interval is the ARHGEF10 gene, a member of the rho family of GTPases known to be involved in neuronal growth and axonal migration, and implicated in human hypomyelination. ARHGEF10 sequencing identified a 10 bp deletion in affected dogs that removes four nucleotides from the 3'-end of exon 17 and six nucleotides from the 5'-end of intron 17 (c.1955_1958+6delCACGGTGAGC. This eliminates the 3'-splice junction of exon 17, creates an alternate splice site immediately downstream in which the processed mRNA contains a frame shift, and generates a premature stop codon predicted to truncate approximately 50% of the protein. Homozygosity for the deletion was highly associated with the severe juvenile-onset PN phenotype in both Leonberger and Saint Bernard dogs. The overall clinical picture of PN in these breeds, and the effects of sex and heterozygosity of the ARHGEF10 deletion, are less clear due to the likely presence of other forms of PN with variable ages of onset and severity of clinical signs. This is the first documented severe polyneuropathy associated with a mutation in ARHGEF10 in any species.

Increased Fas and Bcl-2 Expression on Peripheral Blood T and B Lymphocytes from Juvenile-Onset Systemic Lupus Erythematosus, but not from Juvenile Rheumatoid Arthritis and Juvenile Dermatomyositis

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Bernadete L. Liphaus

2006-01-01

Full Text Available Defective regulation of apoptosis may play a role in the development of autoimmune diseases. Fas and Bcl-2 proteins are involved in the control of apoptosis. The aims of this study were to determine the expression of Fas antigen and Bcl-2 protein on peripheral blood T and B lymphocytes from patients with juvenile-onset systemic lupus erythematosus (JSLE, juvenile rheumatoid arthritis (JRA and juvenile dermatomyositis (JDM. Thirty-eight patients with JSLE, 19 patients with JRA, 10 patients with JDM and 25 healthy controls entered the study. Freshly isolated peripheral blood mononuclear cells (PBMC were stained for lymphocyte markers CD3, CD4, CD8, CD19 and for Fas and Bcl-2 molecules. Expressions were measured by three-color flow cytometry. Statistical analysis was performed using Kruskal–Wallis test. Percentages of freshly isolated T lymphocytes positively stained for Fas protein from JSLE patients were significantly increased compared to healthy controls, patients with JRA and patients with JDM. Percentages of B lymphocytes positive for Fas from JSLE patients were higher than healthy controls and JRA patients. In addition, Fas expression on T cells from patients with JRA was increased compared to JDM patients. Otherwise, Fas expression on T and B cells from JRA and JDM patients were similar to healthy controls. MFI of Bcl-2 positive T lymphocytes from JSLE patients were significantly increased compared to healthy controls and JRA patients. MFI of Bcl-2 protein on B lymphocytes from JSLE patients was similar to healthy controls and patients with JRA and JDM. Bcl-2 expression did not differ between JRA and JDM patients and healthy controls. In conclusion, increased expression of Fas and Bcl-2 proteins observed in circulating T and B lymphocytes from patients with JSLE, but not from patients with JRA and JDM, suggests that abnormalities of apoptosis may be related to the pathogenesis of JSLE and probably are not a result of chronic inflammation.

Coronary artery abnormalities in children with systemic-onset juvenile idiopathic arthritis.

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Lefèvre-Utile, Alain; Galeotti, Caroline; Koné-Paut, Isabelle

2014-05-01

Still's disease (Systemic-onset Juvenile Idiopathic Arthritis: SoJIA) is characterised by high-spiking daily fevers, arthritis and evanescent rashes. Diagnosis of Still's disease is often challenging. Infectious diseases and other inflammatory conditions, especially in young children, Kawasaki disease may look similar. Clinicians often rely on echocardiographic evidence of coronary artery abnormalities to differentiate between Kawasaki disease and Still's disease. Coronary artery dilation would typically favour the diagnosis of Kawasaki disease. We present four children with Still's disease and coronary artery abnormalities who were initially misdiagnosed as Kawasaki disease. The first patient had pericarditis and an irregular wall of the left coronary artery, without dilation on echocardiography. The second patient had a left coronary artery dilatation and a pericarditis. The third patient had thickened left coronary artery walls, and the fourth patient had a hyperechogenicity of the left and right coronary arteries. They received IVIG without success. The diagnosis of Still's disease was made secondary with evidence of persistent arthritis. All but one patient finally needed biologic treatments. Coronary abnormalities may be observed during various febrile conditions and do not exclude the diagnosis of Still's disease. Copyright © 2013 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

A case of late-onset oligomeganephronia

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Rafael José Vargas Alves

2012-12-01

Full Text Available A 33-year old caucasian man was investigated for pain in the right flank, proteinuria, hemathuria and an elevated serum creatinine level. He also presented an abnormal ultrasonography, which revealed asymmetric kidneys. Through renal biopsy, the diagnosis of oligomeganephronia (OMN was confirmed. OMN is a very rare form of renal hypoplasia, and late-onset in adulthood is even rarer. In the pediatric population, OMN leads to end-stage-renal-failure(ESRF in a few years. This is the sixth case related in the literature of a late-onset OMN who have not yet developed ESRF.

Naevoxanthoendothelioma (Synonym: Juvenile Xanthogranuloma

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F Handa

1978-01-01

Full Text Available A case of naevoxanthoendothelioma juvenile xanthogranuloma is reported with rare features like late onset of the disease, involvement of liver and diffuse cutaneous lesions including cafe au lait spots and pigmented naevus. Final diagnosis could be achieved only on histopathology report.

JUVENILE RHEUMATOID ARTHRITIS

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I N Sartika

2012-11-01

Full Text Available Juvenile rheumatoid arthritis (JRA is the most common rheumatic condition in children. JRA is defined as persistent arthritis in 1 or more joints for at least 6 weeks, with the onset before age 16 years. The etiology of JRA is unknown. Antigen activated CD4+ T cell stimulate monocytes, macrophages, and synovial fibroblasts to produce the cytokines Interleukin-1 (IL-1, IL-6, and tumor necrosis factor ? (TNF-? and to secrete matrix metalloproteinases, which lead to chronic inflammation due to infiltration of inflammatory cell, angiogenesis, destruction of cartilage and bone with pannus formation. The 3 major subtypes of JRA are based on the symptoms at disease onset and are designated systemic onset, pauciarticular onset, and polyarticular onset. For all patients, the goals of therapy are to decrease chronic joint pain and suppress the inflammatory process. Poor prognostic have been observed in patients with polyarticular onset, rheumatoid factor, persistent morning stiffness, tenosynovitis, involvement of the small joints, rapid appearance of erosions, active late onset childhood, subcutaneous nodules, or antinuclear antibody.

Neuropathological Comparison of Adult Onset and Juvenile Huntington's Disease with Cerebellar Atrophy: A Report of a Father and Son.

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Latimer, Caitlin S; Flanagan, Margaret E; Cimino, Patrick J; Jayadev, Suman; Davis, Marie; Hoffer, Zachary S; Montine, Thomas J; Gonzalez-Cuyar, Luis F; Bird, Thomas D; Keene, C Dirk

2017-01-01

Huntington's disease (HD) is an autosomal dominant neurodegenerative disease caused by a trinucleotide (CAG) repeat expansion in huntingtin (HTT) on chromosome 4. Anticipation can cause longer repeat expansions in children of HD patients. Juvenile Huntington's disease (JHD), defined as HD arising before age 20, accounts for 5-10% of HD cases, with cases arising in the first decade accounting for approximately 1%. Clinically, JHD differs from the predominately choreiform adult onset Huntington's disease (AOHD) with variable presentations, including symptoms such as myoclonus, seizures, Parkinsonism, and cognitive decline. The neuropathologic changes of AOHD are well characterized, but there are fewer reports that describe the neuropathology of JHD. Here we report a case of a six-year-old boy with paternally-inherited JHD caused by 169 CAG trinucleotide repeats who presented at age four with developmental delay, dysarthria, and seizures before dying at age 6. The boy's clinical presentation and neuropathological findings are directly compared to those of his father, who presented with AOHD and 54 repeats. A full autopsy was performed for the JHD case and a brain-only autopsy was performed for the AOHD case. Histochemically- and immunohistochemically-stained slides were prepared from formalin-fixed, paraffin-embedded tissue sections. Both cases had neuropathology corresponding to Vonsattel grade 3. The boy also had cerebellar atrophy with huntingtin-positive inclusions in the cerebellum, findings not present in the father. Autopsies of father and son provide a unique opportunity to compare and contrast the neuropathologic findings of juvenile and adult onset HD while also providing the first immunohistochemical evidence of cerebellar involvement in JHD. Additionally this is the first known report to include findings from peripheral tissue in a case of JHD.

Reduced incidence of new-onset diabetes mellitus after renal transplantation with 3-hydroxy-3-methylglutaryl-coenzyme a reductase inhibitors (statins).

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Prasad, G V Ramesh; Kim, S Joseph; Huang, Michael; Nash, Michelle M; Zaltzman, Jeffrey S; Fenton, Stanley S A; Cattran, Daniel C; Cole, Edward H; Cardella, Carl J

2004-11-01

Statins have anti-inflammatory effects, modify endothelial function and improve peripheral insulin resistance. We hypothesized that statins influence the development of new-onset diabetes mellitus in renal transplant recipients. The records of all previously non-diabetic adults who received an allograft in Toronto between January 1, 1999 and December 31, 2001 were reviewed with follow-up through December 31, 2002. All patients receiving cyclosporine or tacrolimus, mycophenolate mofetil and prednisone were included. New-onset diabetes was diagnosed by the Canadian Diabetic Association criteria: fasting plasma glucose > or =7.0 mmol/L or 2-h postprandial glucose > or =11.1 mmol/L on more than two occasions. Statin use prior to diabetes development was recorded along with other variables. Cox proportional hazards models analyzing statin use as a time-dependent covariate were performed. Three hundred fourteen recipients met study criteria, of whom 129 received statins. New-onset diabetes incidence was 16% (n = 49). Statins (p = 0.0004, HR 0.238[0.109-0.524]) and ACE inhibitors/ARB (p = 0.01, HR 0.309[0.127-0.750]) were associated with decreased risk. Prednisone dose (p = 0.0001, HR 1.007[1.003-1.010] per 1 mg/d at 3 months), weight at transplant (p = 0.02, HR 1.022[1.003-1.042] per 1 kg), black ethnicity (p = 0.02, HR 1.230[1.023-1.480]) and age > or =45 years (p = 0.01, HR 2.226[1.162-4.261]) were associated with increased diabetes. Statin use is associated with reduced new-onset diabetes development after renal transplantation.

Chest Abnormalities in Juvenile-Onset Mixed Connective Tissue Disease: Assessment with High-Resolution Computed Tomography and Pulmonary Function Tests

International Nuclear Information System (INIS)

Aaloekken, T.M.; Mynarek, G.; Kolbenstvedt, A.; Lilleby, V.; Foerre, Oe.; Soeyseth, V.; Pripp, A.H.; Johansen, B.

2009-01-01

Background: Mixed connective tissue disease (MCTD) is associated with several chest manifestations. Only a few studies have focused on chest manifestations in juvenile-onset MCTD (jMCTD), and the true prevalence of pulmonary abnormalities on high-resolution computed tomography (HRCT) in these patients is unknown. Purpose: To investigate the occurrence of pulmonary abnormalities in jMCTD with particular reference to interstitial lung disease (ILD), and to evaluate a possible association between pulmonary findings and disease-related variables. Material and Methods: Twenty-four childhood-onset MCTD patients with median disease duration of 10.5 years (range 1-21 years) were investigated in a cross-sectional study by means of HRCT, pulmonary function tests (PFT), and clinical assessment. Results: Discrete ILD was identified in six patients (25%). Median extent of ILD was 2.0%, and all except one of the patients had very mild disease in which 5% or less of the parenchyma was affected. The CT features of fibrosis were mainly microcystic and fine intralobular. The most frequently abnormal PFT was carbon monoxide uptake from the lung, which was abnormal in 33% of the patients. PFT and disease duration were not significantly associated with HRCT findings of ILD. Conclusion: The prevalence of ILD in childhood-onset MCTD patients was lower than previously believed. In most of the patients with ILD, the findings were subtle and without clinical correlation. The results suggest a low extent of ILD in childhood-onset MCTD, even after long-term disease duration

The challenge of juvenile Huntington disease: to test or not to test.

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Koutsis, Georgios; Karadima, Georgia; Kladi, Athina; Panas, Marios

2013-03-12

In a cohort of patients with suspected juvenile-onset Huntington disease (HD), we compared HD expansion-positive and -negative cases in order to identify parameters that may allow differentiating between them and may act as a guide to clinicians contemplating genetic testing. We analyzed the clinical and genetic characteristics of 76 juvenile-onset patients referred consecutively for HD genetic testing over a 16-year period. In total, 24 patients were positive for the HD expansion (7.8% of our HD cohort). Mean age at onset of expanded cases was similar to unexpanded cases. All expanded cases had a family history of genetically confirmed HD compared to only 13.5% of unexpanded cases (p = 0.000). Clinical symptoms at onset or at presentation could not differentiate between expanded and unexpanded patients. Although criteria suggested by previous reports allowed statistical differentiation between the 2 groups, they were not sufficiently sensitive and specific to be used in clinical context and performed less satisfactorily than presence of a family history of HD alone. A diagnosis of juvenile HD should be primarily contemplated in symptomatic children with a family history of HD, although a proportion of these will test negative. With no family history of HD, juvenile HD is very unlikely and genetic testing should never delay searching for other causes. The specific nature of symptoms at onset or at presentation is of limited value in guiding the decision to test or not to test.

A Practical Approach to Juvenile Dermatomyositis and Juvenile Scleroderma.

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McCann, Liza J; Pain, Clare E

2016-02-01

Juvenile dermatomyositis and juvenile scleroderma are rare multisystem autoimmune disorders. Although they share some pathognomonic hallmarks with adult onset myositis or scleroderma, there are significant differences in presentation, characteristics and associated features when the diseases present in childhood. In view of this, and the rarity of the conditions, it is important for care to be led by teams with expertise in pediatric rheumatology conditions. Prognosis has improved significantly in the West; likely due to early diagnosis and aggressive treatment with immunosuppressive medications. However, this trend is not replicated in the developing world. Early recognition of these diseases is crucial to achieve rapid and sustained remission and prevent disease or medication associated complications. This article aims to provide a practical overview for recognition, diagnosis and treatment of these conditions.

Are Indian patients with juvenile-onset ankylosing spondylitis taller than reference population ?

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Pulukool Sandhya

2015-01-01

Full Text Available Background & objectives: Paucity of growth retardation has been observed by us in patients with juvenile-onset ankylosing spondylitis (JAS in a tertiary care health centre in south India. We, therefore, undertook this pilot study to assess and compare anthropometry of patients with JAS who were 15 yr and older with that of adult onset ankylosing spondylitis (AAS and matching Indian reference population. Methods: Consecutive male patients (December 2009- October 2012 with JAS and AAS fulfilling Modified New York Criteria were selected after applying inclusion and exclusion criteria. Demography and anthropometry were noted. Height of both patient groups as well as their parents and siblings were compared with that of the reference population. Mid-parental height and delta height were derived. Those with delta height of >8.5 cm were compared with the remaining. Multivariate logistic regression was done for variables that were found to be significant by chi-square in bivariate analysis. Similar analysis was done for BMI also. Results: There was no significant difference in anthropometric variables between JAS and AAS groups. Twenty eight of the 30 (93.33% JAS patients were taller as compared to the reference population. Twenty six (86.67% AAS patients were taller than the reference population. The mean heights of JAS (170.67 ± 6.94 cm and AAS (168.2 ± 5.94 cm patients were significantly higher than the reference value of 163.11 cm; both p0 <0.001. Logistic regression revealed that tallness in JAS was associated positively with hypermobility (OR=23.46,95%CI 1.2-447.2, p0 =0.036. No significant association was detected for height in AAS and for BMI in both JAS and AAS groups. Interpretation & conclusions: No growth retardation was seen in patients with JAS in our study. Majority of patients with JAS and AAS were taller than reference population. The difference between mean height of JAS and AAS was not significant. Larger studies involving different

The effect of low serum bicarbonate values on the onset of action of local anesthesia with vertical infraclavicular brachial plexus block in patients with End-stage renal failure

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Al-mustafa Mahmoud

2010-01-01

Full Text Available Vertical infraclavicular brachial plexus block is utilized in patients with chronic renal failure at the time of creation of an arterio-venous fistula (AVF. The aim of this study is to test the effect of impaired renal function, with the resulting deranged serum electrolytes and blood gases, on the success rate and the onset of action of the local anesthetics used. In this prospective clinical study, we investigated the effect of the serum levels of sodium, potassium, urea, crea-tinine, pH, and bicarbonate on the onset of action of a mixture of lidocaine and bupivacaine administered to create infraclavicular brachial plexus block. A total of 31 patients were studied. The success rate of the block was 93.5 % (29 patients. The mean onset time for impaired or re-duced sensation was found to be 8.9 ± 4.7 mins and for complete loss of sensation, was 21.2 ± 6.7 mins. There was no significant association with serum sodium, potassium, urea, creatinine or the blood pH level (P> 0.05. The bivariate correlation between serum bicarbonate level and the partial and complete sensory loss was -0.714 and -0.433 respectively, with significant correlation (P= 0.00, 0.019. Our study suggests that infraclavicular block in patients with chronic renal failure carries a high success rate; the onset of the block is delayed in patients with low serum bicarbonate levels.

Juvenile Idiopathic Arthritis Onset in a Neonate: A Rare Case Report

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Abdolreza Malek

2017-06-01

Full Text Available Background: A common type of chronic arthritis in children and adolescents is juvenile idiopathic arthritis (JIA.According to the International League of Associations for Rheumatology (ILAR classification, JIA diagnostic criteria include age under 16 years and disease duration of six-weeks. Based on the number of involved joints in the first sixmonths of disease onset, JIA is categorized into oligoarticular or polyarticular subtypes. Age is a characteristic factor in the diagnosis of disease subsets; it is worth mentioning that cases younger than six months of age are seldom found in any of the subtypes. Case report: In this report, we present a rare case of JIA in an infant, presenting at 20 days of age. Effusion of the right hip joint was one of the primary manifestations of the disease. During hospitalization, she went through sepsis workup and a four-week antibiotic therapy for management of lower limb pseudoparalysis. In spite of antibiotic therapy, she developed effusion of a second joint. According to the course and duration of symptoms and ILAR classification forJIA, oligoarticular JIA was diagnosed and treated.Conclusion: In this case, infectious diseases, such as tuberculosis and brucellosis, and malignancies were ruled out as a cause of inflammation through bone marrow aspiration, culture, and tests; ultrasound and magnetic resonance imaging showed no lytic and sclerotic lesions or a fracture. Our experience showed a rare case of JIA and suggested that JIA must be considered in children with joint inflammation at any age

Long term outcome of treatment of end stage renal failure.

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Henning, P; Tomlinson, L; Rigden, S P; Haycock, G B; Chantler, C

1988-01-01

The most common causes of end stage renal failure in 46 children (mean age 11 years, range 4-14) treated between January 1972 and June 1977 were: reflux nephropathy (n = 12), cystinosis (n = 7), focal and segmental glomerulosclerosis (n = 6), and Schönlein-Henoch disease (n = 5). The quality of life, degree of renal function, and height attainment of the 31 survivors were assessed in June 1985, when their mean age was 22 years (range 14-27), using hospital records and a questionnaire designed to highlight social and psychological problems. Twenty six patients had a functioning transplanted kidney. Average growth during treatment for all survivors was normal, but most were disappointed with their 'final height'. Though five patients had some form of disabling bone disease, all 31 could walk and 27 could run. Sixteen (67%) were in full or part time employment and nine were living independently. A group of 32 patients with juvenile onset diabetes treated at this hospital for at least five years were also asked to complete the questionnaire and of these, 17 responded. On average, their data could usefully be compared with those of cases of end stage renal failure. More of the diabetics had jobs, but most sexually mature patients with renal disease were concerned about their physical appearance and had not achieved any stable long term sexual relationships. We suggest that a poor body image resulting in low self esteem may be responsible for the deficiency and believe that further study in this group is warranted.

Uveíte na artrite idiopática juvenil Uveitis in juvenile idiopathic arthritis

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Adriana M. Roberto

2002-02-01

üente na população de pacientes com AIJ associada com uveíte (60% do que naqueles sem uveíte (12% (pObjective: to evaluate the frequency of chronic anterior uveitis in patients with juvenile idiopathic arthritis and its association with the presence of antinuclear antibodies. Patients and methods: we retrospectively studied 72 patients with juvenile idiopathic arthritis. All of them were submitted to slit-lamp examination of the anterior chamber at diagnosis. Both antinuclear antibodies and rheumatoid factor were determined. Patients with positive results for antinuclear antibodies were evaluated every three months and those with negative results were assessed every six months.Results: forty patients were male (55.5% and 36 were Caucasoid (50%. The mean age at the onset of juvenile idiopathic arthritis was 6.4 years (range = 1 to 14 years and the mean age at the beginning of the study was 10.4 years (1 to 19 years. According to the type of disease at onset, 32 were pauciarticular (44.4% (17 boys and 15 girls, 30 were polyarticular (41.6% (17 boys and 13 girls and 10 were systemic (14% (6 boys and 4 girls. We observed chronic anterior uveitis in five patients (6.5% (mean age = 11.4 years. Among them, four (80% had pauciarticular juvenile idiopathic arthritis at disease onset (three girls with type I juvenile idiopathic arthritis and positive antinuclear antibodies and one boy with type I juvenile idiopathic arthritis and negative antinuclear antibodies and one girl with polyarticular juvenile idiopathic arthritis (negative antinuclear antibodies and rheumatoid factor. In this group, the mean age at the onset of juvenile idiopathic arthritis was 5.1 years and the mean age of uveitis onset was 9 years. Antinuclear antibodies were positive in 3/5 patients (60% with uveitis. Antinuclear antibodies were positive in 12% of the patients without uveitis (n = 67. Among the patients with uveitis, three had only one flare and the other two had four flares with cataract. The

Early onset bilateral juvenile myasthenia gravis masquerading as simple congenital ptosis

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Alam, Md. Shahid

2017-03-01

Full Text Available Myasthenia gravis is an autoimmune disorder affecting the neuromuscular junction. Ocular myasthenia gravis presents as ptosis with extraocular motility restriction and is prone to be misdiagnosed as third nerve palsy or congenital or aponeurotic ptosis. Juvenile ocular myasthenia gravis in very young children is difficult to diagnose and can be easily labeled as a case of congenital ptosis, the more so when the condition is bilateral. We present a case of a two-year-old child who presented with bilateral ptosis and was diagnosed as a case of simple congenital ptosis elsewhere with the advice to undergo tarsofrontalis sling surgery. The child was diagnosed with juvenile myasthenia gravis on thorough history, examination, and systemic evaluation and was started on anti-myasthenic treatment.

Impact of Antiinflammatory Treatment on the Onset of Uveitis in Juvenile Idiopathic Arthritis: Longitudinal Analysis From a Nationwide Pediatric Rheumatology Database.

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Tappeiner, Christoph; Schenck, Sandra; Niewerth, Martina; Heiligenhaus, Arnd; Minden, Kirsten; Klotsche, Jens

2016-01-01

Based on a nationwide database, this study analyzed the influence of methotrexate (MTX), tumor necrosis factor (TNF) inhibitors, and a combination of the 2 medications on uveitis occurrence in juvenile idiopathic arthritis (JIA) patients. Data from the National Paediatric Rheumatological Database in Germany were used in this study. Between 2002 and 2013, data from JIA patients were annually documented at the participating pediatric rheumatologic sites. Patients with a JIA disease duration of treatment on the occurrence of uveitis was evaluated by discrete-time survival analysis. A total of 3,512 JIA patients (mean ± SD age 8.3 ± 4.8 years, 65.7% female, 53.2% antinuclear antibody positive, and mean ± SD age at arthritis onset 7.8 ± 4.8 years) fulfilled the inclusion criteria. Mean ± SD total followup time was 3.6 ± 2.4 years. Uveitis developed in a total of 180 patients (5.1%) within 1 year after arthritis onset. Uveitis onset after the first year was observed in another 251 patients (7.1%). Disease-modifying antirheumatic drug (DMARD) treatment in the year before uveitis onset significantly reduced the risk for uveitis as follows: MTX: hazard ratio (HR) 0.63, P = 0.022; TNF inhibitors: HR 0.56, P uveitis risk (HR 0.29, P uveitis onset. Early MTX use within the first year of disease and the combination of MTX with a TNF inhibitor had the highest protective effect. © 2016 The Authors. Arthritis Care & Research published by Wiley Periodicals, Inc. on behalf of the American College of Rheumatology.

Bayesian comparative effectiveness study of four consensus treatment plans for initial management of systemic juvenile idiopathic arthritis: FiRst-Line Options for Systemic juvenile idiopathic arthritis Treatment (FROST).

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Nigrovic, Peter A; Beukelman, Timothy; Tomlinson, George; Feldman, Brian M; Schanberg, Laura E; Kimura, Yukiko

2018-03-01

Systemic juvenile idiopathic arthritis is a rare febrile arthritis of childhood characterized by a potentially severe course, including prolonged glucocorticoid exposure, growth failure, destructive arthritis, and life-threatening macrophage activation syndrome. Early cytokine-blocking biologic therapy may improve long-term outcomes, although some systemic juvenile idiopathic arthritis patients respond well to non-biologic treatment, leaving optimal management undefined. Consequently, treatment of new-onset systemic juvenile idiopathic arthritis by expert clinicians varies widely. To describe a pragmatic, observational comparative effectiveness study that takes advantage of diversity in the management of a rare disease: FiRst-Line Options for Systemic juvenile idiopathic arthritis Treatment (FROST), comparing non-biologic and biologic consensus treatment plans for new-onset systemic juvenile idiopathic arthritis within the 60-center Childhood Arthritis and Rheumatology Research Alliance Registry (CARRA). FiRst-Line Options for Systemic juvenile idiopathic arthritis Treatment (FROST) is a multicenter, prospective, non-randomized study that compares four Childhood Arthritis and Rheumatology Research Alliance (CARRA) consensus treatment plans for new-onset systemic juvenile idiopathic arthritis: (1) glucocorticoids alone, (2) methotrexate, (3) interleukin-1 blockade, and (4) interleukin-6 blockade. Patients consenting to participation in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry are started on one of four Consensus Treatment Plans at the discretion of the treating physician. The outcome of primary interest is clinically inactive disease off glucocorticoids at 9 months, comparing non-biologic (Consensus Treatment Plans 1 + 2) versus biologic (Consensus Treatment Plans 3 + 4) strategies. Bayesian analytic methods will be employed to evaluate response rates, using propensity scoring to balance treatment groups for potential

Bilateral renal masses in a 10-year-old girl with renal failure and urinary tract infection: the importance of functional imaging

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Urbania, Thomas H. [University of California, San Francisco, Department of Radiology and Biomedical Imaging, 505 Parnassus Ave., Box 0628, San Francisco, CA (United States); Kammen, Bamidele F.; Nancarrow, Paul A. [Children' s Hospital and Research Center Oakland, Department of Diagnostic Imaging, Oakland, CA (United States); Morrell, Rose Ellen [Children' s Hospital and Research Center Oakland, Department of Nephrology, Oakland, CA (United States)

2009-02-15

Renal sonography is a routine step in the evaluation of new onset renal failure. When renal masses are discovered in this setting, functional imaging may be critical. We report a case of bilateral renal masses in a girl with urinary tract infection and renal insufficiency found to have vesicoureteral reflux. Renal scintigraphy revealed these masses to be the only remaining functional renal tissue, preventing potentially harmful resection. (orig.)

Bilateral renal masses in a 10-year-old girl with renal failure and urinary tract infection: the importance of functional imaging

International Nuclear Information System (INIS)

Urbania, Thomas H.; Kammen, Bamidele F.; Nancarrow, Paul A.; Morrell, Rose Ellen

2009-01-01

Renal sonography is a routine step in the evaluation of new onset renal failure. When renal masses are discovered in this setting, functional imaging may be critical. We report a case of bilateral renal masses in a girl with urinary tract infection and renal insufficiency found to have vesicoureteral reflux. Renal scintigraphy revealed these masses to be the only remaining functional renal tissue, preventing potentially harmful resection. (orig.)

Superior oblique tendon (Brown’s syndrome as the presenting finding in childhood onset HLA-B27-related enthesitis and juvenile idiopathic oligoarticular arthritis

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C. Pham

2014-11-01

Full Text Available We report two patients who presented with Brown’s syndrome. The first is a 7-year-old boy who at the time of his diagnosis was also found to have enthesitis and HLA-B27 positivity. The second patient was diagnosed with bilateral Brown’s syndrome at 13 months of age. At age 7 she developed a persistent oligoarticular arthritis and unilateral anterior iritis consistent with the oligoarticular Juvenile Idiopatic Arthritis (JIA phenotype. These cases highlight ophthalmologic findings and diagnostic considerations with respect to Brown’s syndrome and associated childhood onset rheumatologic disease.

Long-term outcome in patients with juvenile dermatomyositis

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Mathiesen, P; Hegaard, H; Herlin, Troels

2012-01-01

To evaluate a group of 53 patients with juvenile dermatomyositis (JDM), on average 13.9 years after disease onset, in order to describe the long-term disease outcome and to identify disease-related parameters associated with poor disease outcome....

Rapidly progressive renal disease as part of Wolfram syndrome in a large inbred Turkish family due to a novel WFS1 mutation (p.Leu511Pro)

DEFF Research Database (Denmark)

Yuca, Sevil Ari; Rendtorff, Nanna Dahl; Boulahbel, Houda

2012-01-01

Wolfram syndrome, also named "DIDMOAD" (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness), is an inherited association of juvenile-onset diabetes mellitus and optic atrophy as key diagnostic criteria. Renal tract abnormalities and neurodegenerative disorder may occur in the third...... and fourth decade. The wolframin gene, WFS1, associated with this syndrome, is located on chromosome 4p16.1. Many mutations have been described since the identification of WFS1 as the cause of Wolfram syndrome. We identified a new homozygous WFS1 mutation (c.1532T>C; p.Leu511Pro) causing Wolfram syndrome...

Renal cell carcinoma in India demonstrates early age of onset & a late stage of presentation

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Shalini Agnihotri

2014-01-01

Full Text Available Background & objectives: Clinical spectrum of most of the diseases in developing countries is different from the west. Similarly whether renal cell carcinomas (RCC in a developing country like India is seen in the same spectrum in relation to the age at presentation as in the west is not described in the literature. This study was carried out to investigate the spectrum of RCC in India with regards to age of onset, stage at presentation and survival. Methods: Patients with renal tumour, treated between January 2000 to December 2012 in a tertiary care hospital in north India, were analyzed for age at presentation, clinical features and histopathological characteristics. Clinical diagnosis was made by contrast enhanced computerized tomography (CECT scans and/or magnetic resonance imaging (MRI. Renal masses diagnosed as angiomyolipoma, infective masses and hydatid cysts were excluded from the analysis. Impact of various age groups on gender, tumour size, TNM stage, Fuhrman grade, histopathological subtypes, lymph node, inferior vena cava (IVC involvement and survival was analyzed. Patients were grouped in five age groups i.e. ≤39, 40-49, 50-59, 60-69 and more than 70 yr of age. Results: Of the total 617 patients with 617 renal tumours (2 patients had bilateral tumours but only the larger tumour was considered clinically suspected as RCC, 586 had epithelial cell tumour and the remaining 31 had non epithelial cell tumour. The mean tumour size was 8.08±3.5 cm (median 7, range 1-25 cm. Tumour of less than 4 cm size was present in only 10.4 per cent patients. The mean age at diagnosis was 55.15±13.34 (median 56, range 14-91 yr years. A total of 30.03 per cent of renal tumours presented in patients younger than 50 yr of age. Though there was no difference in stage, Fuhrman′s grade, IVC involvement and lymph nodal spread among various age groups, younger patients had higher proportion of non clear cell RCC and only 48.59 per cent of them presented

Uveitis and Juvenile Psoriatic Arthritis or Psoriasis.

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Salek, Sherveen S; Pradeep, Archana; Guly, Catherine; Ramanan, Athimalaipet V; Rosenbaum, James T

2018-01-01

To describe the phenotype of the uveitis that accompanies juvenile psoriatic arthritis or psoriasis. Observational case series. Setting: Two university-based referral clinics: 1 in England, 1 in the United States. Five children with uveitis and psoriatic arthritis and 1 with uveitis and psoriasis Observational Procedure: Retrospective chart review. Demographics of subjects such as age and sex; description of ocular and joint disease; surgical and other complications; medical treatment. Five of the 6 children in this series had the onset of disease at or before age 6 (P = .0008 compared to expected age of onset for psoriatic arthritis in childhood). All children in this series had an inadequate response to topical corticosteroids. Most of the children were treated with systemic corticosteroids for many months, yet all of them went on to require methotrexate. Therapy with systemic methotrexate did not suffice, as all the patients also required some form of biologic therapy. Five of 6 had surgeries such as vitrectomy, cataract extraction, or a procedure for glaucoma control. The observations suggest that the uveitis that accompanies juvenile psoriatic arthritis might be a distinct disease that is particularly severe when its onset affects children aged 6 years or younger. Copyright © 2017 Elsevier Inc. All rights reserved.

Rapidly progressive renal disease as part of Wolfram syndrome in a large inbred Turkish family due to a novel WFS1 mutation (p.Leu511Pro).

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Yuca, Sevil Ari; Rendtorff, Nanna Dahl; Boulahbel, Houda; Lodahl, Marianne; Tranebjærg, Lisbeth; Cesur, Yasar; Dogan, Murat; Yilmaz, Cahide; Akgun, Cihangir; Acikgoz, Mehmet

2012-01-01

Wolfram syndrome, also named "DIDMOAD" (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness), is an inherited association of juvenile-onset diabetes mellitus and optic atrophy as key diagnostic criteria. Renal tract abnormalities and neurodegenerative disorder may occur in the third and fourth decade. The wolframin gene, WFS1, associated with this syndrome, is located on chromosome 4p16.1. Many mutations have been described since the identification of WFS1 as the cause of Wolfram syndrome. We identified a new homozygous WFS1 mutation (c.1532T>C; p.Leu511Pro) causing Wolfram syndrome in a large inbred Turkish family. The patients showed early onset of IDDM, diabetes insipidus, optic atrophy, sensorineural hearing impairment and very rapid progression to renal failure before age 12 in three females. Ectopic expression of the wolframin mutant in HEK cells results in greatly reduced levels of protein expression compared to wild-type wolframin, strongly supporting that this mutation is disease-causing. The mutation showed perfect segregation with disease in the family, characterized by early and severe clinical manifestations. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

Identification a novel MYOC gene mutation in a Chinese family with juvenile-onset open angle glaucoma.

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Zhao, Xin; Yang, Chaoshan; Tong, Yi; Zhang, Xiaohui; Xu, Liang; Li, Yang

2010-08-25

To describe the clinical and genetic findings in one Chinese family with juvenile-onset open angle glaucoma (JOAG). One family was examined clinically and a follow-up took place 5 years later. After informed consent was obtained, genomic DNA was extracted from the venous blood of all participants. Linkage analysis was performed with three microsatellite markers around the MYOC gene (D1S196, D1S2815, and D1S218) in the family. Mutation screening of all coding exons of MYOC was performed by direct sequencing of PCR-amplified DNA fragments and restriction fragment length polymorphism (RFLP) analysis. Bioinformatics analysis by the Garnier-Osguthorpe-Robson (GOR) method predicted the effects of variants detected on secondary structures of the MYOC protein. Clinical examination and pedigree analysis revealed a three- generation family with seven members diagnosed with JOAG, three with ocular hypertension, and five normal individuals. Through genotyping, the pedigree showed a linkage to the MYOC on chromosome 1q24-25. Mutation screening of MYOC in this family revealed an A-->T transition at position 1348 (p. N450Y) of the cDNA sequence. This missense mutation co-segregated with the disease phenotype of the family, but was not found in 100 normal controls. Secondary structure prediction of the p.N450Y by the GOR method revealed the replacement of a coil with a beta sheet at the amino acid 447. Early onset JOAG, with incomplete penetrance, is consistent with a novel mutation in MYOC. The finding provides pre-symptomatic molecular diagnosis for the members of this family and is useful for further genetic consultation.

Síndrome de ativação macrofágica associada com artrite idiopática juvenil sistêmica Macrophage activation syndrome associated with systemic juvenile idiopathic arthritis

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Clovis Artur A. Silva

2004-12-01

Full Text Available OBJETIVO: Descrever as características da síndrome de ativação macrofágica associada a artrite idiopática juvenil. DESCRIÇÃO DOS CASOS: Foram analisados retrospectivamente os prontuários de 462 pacientes com artrite idiopática juvenil. Destes, sete (1,5% pacientes desenvolveram síndrome de ativação macrofágica; todos tinham a forma sistêmica da doença. A mediana de idade de início da artrite idiopática juvenil foi de 3 anos e 10 meses, e a mediana do tempo de duração da artrite idiopática juvenil antes da síndrome de ativação macrofágica foi de 8 anos e 4 meses. Todos os pacientes apresentaram febre, icterícia, hepatoesplenomegalia, sangramentos, pancitopenia e elevação das enzimas hepáticas e dos tempos de coagulação e bilirrubina direta. Três casos apresentaram infecções associadas e um caso desenvolveu a síndrome de ativação macrofágica 2 semanas após a introdução de sulfasalazina. Três pacientes morreram. Proliferação macrofágica e hemofagocitose foram evidenciadas em cinco. A terapêutica da síndrome de ativação macrofágica incluiu pulsoterapia com metilprednisolona em todos, ciclosporina em três, plasmaférese em dois e gamaglobulina endovenosa em dois. COMENTÁRIOS: A síndrome de ativação macrofágica é uma complicação da artrite idiopática juvenil sistêmica com alta morbidade e mortalidade.OBJECTIVE: To describe the characteristics of macrophage activation syndrome associated with juvenile idiopathic arthritis. DESCRIPTION: This is a retrospective study involving 462 patients with juvenile idiopathic arthritis. Seven (1.5% of those patients suffered from systemic onset juvenile idiopathic arthritis and developed macrophage activation syndrome. The median age of the juvenile idiopathic arthritis onset was 3 years and 10 months and the median duration of juvenile idiopathic arthritis before macrophage activation syndrome was 8 years and 4 months. All of them presented with fever

Methotrexate treatment may prevent uveitis onset in patients with juvenile idiopathic arthritis: experiences and subgroup analysis in a cohort with frequent methotrexate use.

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Kostik, Mikhail M; Gaidar, Ekaterina V; Hynnes, Alla Y; Dubko, Margarita F; Masalova, Vera V; Snegireva, Ludmil S; Chikova, Irina A; Isupova, Eugenia A; Nikitina, Tatiana N; Serogodskaya, Elena D; Kalashnikova, Olga V; Ravelli, Angelo; Chasnyk, Vyacheslav G

2016-01-01

To re-evaluate the ability of methotrexate (MTX) to prevent the onset of uveitis in Russian children with juvenile idiopathic arthritis (JIA). The clinical charts for all consecutive patients who received a stable management for at least 2 years with or without MTX were reviewed. Patients who were given systemic medications other than MTX (except NSAID) and patients with systemic arthritis, rheumatoid factor-positive arthritis, or enthesitis-related arthritis were excluded. Each patient was examined after at least a 2-year follow-up period after the first visit to establish whether uveitis had occurred. A total of 281 patients with a median disease duration of 3.8 years were included. 191 patients (68%) were treated with MTX. During the observation period, 64 patients (22.8%) developed uveitis, a median of 1.6 year after disease onset. The frequency of uveitis was lower in MTX-treated than in MTX-untreated patients (11.5% vs. 46.7%, respectively, OR=6.7 (95%CI:3.7-12.3), p=0.0000001). Survival analysis confirmed that patients treated with MTX had a lower probability of developing uveitis (HR=4.35, p=0.000001). In subgroup analysis it was shown that MTX was more preventive in boys than in girls, and in patients with JIA onset age of over 5 years compared to those with disease onset less than 5 years. The data of survival analysis of MTX prevention has shown that benefits do not depend on the number of active joints and ANA status. MTX therapy may prevent the onset of uveitis in children with JIA. Further randomised controlled trials are required to confirm our results.

Familial juvenile hyperuricemic nephropathy : report on a new mutation and a pregnancy

NARCIS (Netherlands)

Lhotta, Karl; Gehringer, A; Jennings, P; Kronenberg, F.; Brezinka, C; Andersone, I; Strazdins, V

BACKGROUND: Familial juvenile hyperuricemic nephropathy (FJHN) is a rare autosomal dominant disease caused by mutations in the uromodulin gene (UMOD) and leading to gout, tubulointerstitial nephropathy and end-stage renal disease. CASE REPORTS AND RESULTS: A Latvian family suffering from FJHN is

Abnormal muscle MRI in a patient with systemic juvenile arthritis

International Nuclear Information System (INIS)

Miller, M.L.; Levinson, L.; Pachman, L.M.; Poznanski, A.

1995-01-01

Although myositis has been described in children with systemic-onset juvenile arthritis (JA), its documentation by MRI has not been reported. We describe a 13-year-old boy with systemic-onset JA, severe myalgia, and elevated muscle enzymes, but normal muscle strength, who had an MRI consistent with myositis. Magnetic resonance imaging can identify the specific location of myositis, allowing more precise definition of a potential complication of systemic JA. (orig.)

Abnormal muscle MRI in a patient with systemic juvenile arthritis

Energy Technology Data Exchange (ETDEWEB)

Miller, M.L. [Dept. of Pediatrics, Northwestern Univ. Medical School, Chicago, IL (United States); Levinson, L. [Dept. of Pediatrics, Northwestern Univ. Medical School, Chicago, IL (United States); Pachman, L.M. [Dept. of Pediatrics, Northwestern Univ. Medical School, Chicago, IL (United States); Poznanski, A. [Dept. of Radiology, Northwestern Univ. Medical School, Chicago, IL (United States)

1995-11-01

Although myositis has been described in children with systemic-onset juvenile arthritis (JA), its documentation by MRI has not been reported. We describe a 13-year-old boy with systemic-onset JA, severe myalgia, and elevated muscle enzymes, but normal muscle strength, who had an MRI consistent with myositis. Magnetic resonance imaging can identify the specific location of myositis, allowing more precise definition of a potential complication of systemic JA. (orig.)

Conversion (dissociative) symptoms as a presenting feature in early onset bipolar disorder: a case series

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Ghosal, Malay Kumar; Guha, Prathama; Sinha, Mausumi; Majumdar, Debabrata; Sengupta, Payel

2009-01-01

We present three cases of early onset bipolar disorder where dissociative (conversion) symptoms preceded the onset of mania. This case series underscores the significance of dissociative/conversion symptoms as an early atypical presentation in juvenile bipolar disorder.

Learning and memory deficits in male adult mice treated with a benzodiazepine sleep-inducing drug during the juvenile period

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Yusuke Furukawa

2016-07-01

Full Text Available Gamma-aminobutyric acid (GABA, the major inhibitory neurotransmitter in the mammalian central nervous system, is also known to be important for brain development. Therefore, disturbances of GABA receptor (GABA-R mediated signaling (GABA-R signal during brain development may influence normal brain maturation and cause late-onset brain malfunctions. In this study, we examined whether the temporal stimulation of the GABA-R signal during brain development induces late-onset adverse effects on the brain in adult male mice. To stimulate the GABA-R signal, we used either the benzodiazepine sleep-inducing drug triazolam (TZ or the non-benzodiazepine drug zolpidem (ZP. We detected deficits in learning and memory in mice treated with TZ during the juvenile period, as seen in the fear conditioning test. On the other hand, ZP administration during the juvenile period had little effect. In addition, decreased protein expression of GluR1 and GluR4, which are excitatory neurotransmitter receptors, was detected in the hippocampi of mice treated with TZ during the juvenile period. We measured mRNA expression of the immediate early genes (IEGs, which are neuronal activity markers, in the hippocampus shortly after the administration of TZ or ZP to juvenile mice. Decreased IEG expression was detected in mice with juvenile TZ administration, but not in mice with juvenile ZP administration. Our findings demonstrate that TZ administration during the juvenile period can induce irreversible brain dysfunction in adult mice. It may need to take an extra care for the prescription of benzodiazepine sleep-inducing drugs to juveniles because it might cause late onset learning and memory defects.

Zinc transporter-8 autoantibodies can replace IA-2 autoantibodies as a serological marker for juvenile onset type 1 diabetes in India

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C Shivaprasad

2014-01-01

Full Text Available Introduction: Zinc transporter-8 (ZnT8 is an islet cell secretory granule membrane protein recently identified as an autoantigen in type 1 diabetes (T1D. The aim of this study was to estimate the prevalence of antibodies to ZnT8 (ZnT8A in juvenile onset T1D and to determine the utility of ZnT8A as an independent marker of autoimmunity either alone in antibody-negative subjects or in conjunction with glutamic acid decarboxylase (GAD and insulinoma-2 antigen antibodies (GADA and IA2A. Research Design: ZnT8A, GADA, and IA2A were measured in sera of consecutive T1D patients (n = 88, age range 2-18 years within 4 years of diagnosis and 88 sex-matched controls. Results: The prevalences of GADA, ZnT8a, and IA2A were 64.7%, 31.8% and 19.3%, respectively. In newly diagnosed patients, the frequency of ZnT8A was 45%. ZnT8A were positive in 26% of patients negative for both GADA and IA2A. IA2A were positive only in two patients who were negative for other two antibodies. Combined use of ZnT8A and GADA could detect 97% of antibody positive patients. In receiver operating characteristic (ROC analysis, the performances of GADA and ZnT8As were better than that of IA2A; and AUCs of GADA, ZnT8A, and IA2A for the prediction of T1D were 0.8, 0.65, and 0.59, respectively. Conclusions: ZnT8A complements GADA and increases the diagnostic sensitivity for detection of autoimmunity in juvenile-onset T1D. Inclusion of ZnT8A increases the proportion of patients with antibody positivity to nearly 80%. ZnT8A can replace IA2A as a serological marker for autoimmunity in Indian T1D patients without loss of sensitivity and specificity.

Subpopulations Within Juvenile Psoriatic Arthritis: A Review of the Literature

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Matthew L. Stoll

2006-01-01

Full Text Available The presentation of juvenile psoriatic arthritis (JPsA has long been recognized to be clinically heterogeneous. As the definition of JPsA expanded to accommodate atypical manifestations of psoriasis in young children, studies began to reflect an increasingly clear biphasic distribution of age of onset, with peaks in the first few years of life and again in early adolescence. These two subpopulations differ in gender ratio, pattern of joint involvement, laboratory findings and potentially response to therapy. Intriguingly, a similar distribution of age of onset has been observed in juvenile rheumatoid arthritis (JRA, and correlates with patterns of HLA association. While a secure classification of subpopulations within JPsA awaits improved pathophysiologic understanding, future research must consider the possibility that different disease mechanisms may be operative in distinct subsets of patients with this disorder.

Impact of Juvenile Idiopathic Arthritis Associated Uveitis in Early Adulthood

NARCIS (Netherlands)

Haasnoot, AJW; Vernie, Lenneke A; Rothova, Aniki; V D Doe, Patricia; Los, Leonoor I; Schalij-Delfos, Nicoline E; de Boer, Joke H

2016-01-01

BACKGROUND: Typically juvenile idiopathic arthritis (JIA)-associated uveitis (further referred as 'JIA-uveitis') has its onset in childhood, but some patients suffer its, sometimes visual threatening, complications or ongoing disease activity in adulthood. The objective of this study was to analyze

Impact of Juvenile Idiopathic Arthritis Associated Uveitis in Early Adulthood

NARCIS (Netherlands)

Haasnoot, Anne-Mieke J. W.; Vernie, Lenneke A.; Rothova, Aniki; van der Doe, Patricia; Los, Leonoor I.; Schalij-Delfos, Nicoline E.; de Boer, Joke H.

2016-01-01

Background Typically juvenile idiopathic arthritis (JIA)-associated uveitis (further referred as 'JIA-uveitis') has its onset in childhood, but some patients suffer its, sometimes visual threatening, complications or ongoing disease activity in adulthood. The objective of this study was to analyze

Impact of juvenile idiopathic arthritis associated uveitis in early adulthood

NARCIS (Netherlands)

Haasnoot, A.-M.J.W. (Anne-Mieke J. W.); Vernie, L.A. (Lenneke A.); A. Rothová (Aniki); Doe, P.V.D. (Patricia V. D.); L.I. Los (Leonoor I.); N.E. Schalij-Delfos (Nicoline); J.H. de Boer (Joke)

2016-01-01

textabstractBackground: Typically juvenile idiopathic arthritis (JIA)-associated uveitis (further referred as 'JIA-uveitis') has its onset in childhood, but some patients suffer its, sometimes visual threatening, complications or ongoing disease activity in adulthood. The objective of this study was

Sepsis patients' renal manifestation on contrast-enhanced CT

International Nuclear Information System (INIS)

Sasaguri, K.; Yamaguchi, K.; Nakazono, T.; Mizuguchi, M.; Irie, H.

2016-01-01

Aim: To evaluate renal volume and attenuation changes in patients with sepsis on contrast-enhanced computed tomography (CT) with respect to the severity of sepsis. Materials and methods: Forty-four patients with sepsis who underwent CT before and after the onset of sepsis were retrospectively analysed. Renal volume and CT attenuation value of the renal cortex on contrast-enhanced CT were measured for each patient and changes in renal volume and CT attenuation value from before to after the onset of sepsis were calculated. The changes were correlated with the severity of sepsis (Sepsis-related Organ Failure Assessment [SOFA] score). The time course of the renal volume and CT attenuation changes were also evaluated. Results: Renal volume increased by 17.6% and CT attenuation value decreased by 19% after the onset of sepsis with statistically significant differences (p<0.001 for both renal volume and CT attenuation changes). The renal volume and CT attenuation changes had significant correlations with the SOFA score (r=0.36, p=0.018 and −0.43, p=0.005, respectively). The time course of the renal volume and CT attenuation changes seemed to be gradual compared to that of the SOFA score and to lag behind the peak of the SOFA score. Conclusion: In patients with sepsis, the renal volume increases and the CT attenuation value decreases in proportion to the severity of sepsis. The changes may lag behind the peak of severity of sepsis and can be observed for a relatively long time after a patient's recovery from sepsis. - Highlights: • The renal volume increases and the renal enhancement on contrast-enhanced CT decreases in patients with sepsis. • The degrees of these changes are correlated with severity of sepsis. • These changes may lag behind the peak of severity of sepsis and last for a long time after a patient's recovery from sepsis.

Renal Impairment in Cirrhosis Unrelated to Hepatorenal Syndrome

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Gavin Low

2015-01-01

Full Text Available Renal impairment is common in liver disease and may occur as a consequence of the pathophysiological changes that underpin cirrhosis or secondary to a pre-existing unrelated insult. Nevertheless, the onset of renal impairment often portends a worsening prognosis. Hepatorenal syndrome remains one of the most recognized and reported causes of renal impairment in cirrhosis. However, other causes of renal impairment occur and can be classified into prerenal, intrinsic or postrenal, which are the subjects of the present review.

Increased frequency of HLA-DPw2 in pauciarticular onset juvenile chronic arthritis

DEFF Research Database (Denmark)

Ødum, Niels; Morling, Niels; Friis, J

1986-01-01

Thirty-six unrelated Danish patients with pauciarticular Juvenile Chronic Arthritis (PJCA) and 120 controls were typed for HLA-DPw1-w6 and the local specificity CDPHEI with bulk-expanded Primed Lymphocyte Typing (PLT) cells. The frequency of HLA-DPw2 was 52.8% in PJCA patients and 16.7% in controls...

Increased frequency of HLA-DPw2 in pauciarticular onset juvenile chronic arthritis

DEFF Research Database (Denmark)

Ødum, Niels; Morling, Niels; Friis, J

1986-01-01

Thirty-six unrelated Danish patients with pauciarticular Juvenile Chronic Arthritis (PJCA) and 120 controls were typed for HLA-DPw1-w6 and the local specificity CDPHEI with bulk-expanded Primed Lymphocyte Typing (PLT) cells. The frequency of HLA-DPw2 was 52.8% in PJCA patients and 16.7% in contro...

Renal paraneoplastic vasculitis complicating lung adenocarcinoma

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Besma Ben Dhaou

2014-01-01

Full Text Available Renal paraneoplastic vasculitis (RNPV is rare. It can be revealed by glomerulonephritis, microaneurysms or renal failure. RPNV may precede the onset of the primary tumor, and treatment and prognosis depend on the etiology (primary tumor. A 54-year-old man who had a primary lung adenocarcinoma was admitted for nephrotic syndrome. The investigations revealed RNPV. The patient was treated with corticosteroids at high dose and cyclophosphamide with improvement of the renal condition; however, the patient died from worsening of his pulmonary neoplasia.

Elevated levels of peripheral blood CD14(bright) CD16+ and CD14(dim) CD16+ monocytes may contribute to the development of retinopathy in patients with juvenile onset type 1 diabetes.

Science.gov (United States)

Ryba-Stanisławowska, Monika; Myśliwska, Jolanta; Juhas, Ulana; Myśliwiec, Małgorzata

2015-09-01

The study aimed to analyze the CD14(bright) CD16(+) and CD14(dim) CD16(+) monocyte subsets in juvenile-onset complication-free diabetes mellitus type 1 in the context of their association with microvascular complications. 61 children with type 1 diabetes and 30 healthy individuals were enrolled in a study. CD14(bright) CD16(+) and CD14(dim) CD16(+) monocytes were quantified in peripheral blood by means of flow cytometry. At the time of sampling blood glucose concentration was taken along with biochemical measurement of renal function, CRP and glycosylated hemoglobin. The Spearman's correlations were used to compare the relationship between CD16(+) monocyte subsets and the clinical parameters that can predict the development of microangiopathies. The flow cytometric analysis of monocyte subsets in peripheral blood of analyzed subjects revealed that the numbers of CD14(bright) CD16(+) and CD14(dim) CD16(+) monocytes were significantly higher in patients with type 1 diabetes than in the healthy individuals. As to the relationship between CD16(+) monocyte subsets and the clinical parameters that can predict development of microangiopathies, it was shown that both CD16(+) subsets were associated with increased risk of retinopathy development, defined as retinopathy development value. Elevated levels of intermediate CD14(bright) CD16(+) and non-classical CD14(dim) CD16(+) monocytes predict development of diabetic retinopathy in patients with type 1 diabetes. © 2015 APMIS. Published by John Wiley & Sons Ltd.

Associations of clinical features and prognosis with age at disease onset in patients with systemic lupus erythematosus.

Science.gov (United States)

Feng, X; Zou, Y; Pan, W; Wang, X; Wu, M; Zhang, M; Tao, J; Zhang, Y; Tan, K; Li, J; Chen, Z; Ding, X; Qian, X; Da, Z; Wang, M; Sun, L

2014-03-01

The objective of this study is to evaluate the association of clinical features and prognosis with age at disease onset in patients with systemic lupus erythematosus (SLE) in a large, multicenter Chinese cohort. Medical records of 1898 SLE inpatients from 15 hospitals were reviewed and classified into three groups according to their ages at disease presentation. Categorical data were analyzed by chi-square test and potentially associated factors were tested by multinomial logistic regression. Among the patients studied, 259 (13.6%) were juvenile onset (≤18 years), 1444 (76.1%) were early onset (>18 and ≤45 years) and 195 (10.3%) were late onset (>45 years). Whenever manifestations occurred, most patients (>80%) were diagnosed within two years. Juvenile-onset patients were more likely to be untreated before admission (p lupus. Interestingly, our data showed that more patients with late-onset disease had a SLEDAI score change of >7 at discharge. In conclusion, age at onset has an impact on SLE disease status, and infection is the main cause of death in those with late-onset lupus. Considering that the late-onset patients had simultaneously easily controllable diseases and high incidence of comorbidities, a different treatment strategy from younger patients should be considered.

Late onset neurodegeneration in the Cln3-/- mouse model of juvenile neuronal ceroid lipofuscinosis is preceded by low level glial activation.

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Pontikis, Charlie C; Cella, Claire V; Parihar, Nisha; Lim, Ming J; Chakrabarti, Shubhodeep; Mitchison, Hannah M; Mobley, William C; Rezaie, Payam; Pearce, David A; Cooper, Jonathan D

2004-10-15

Mouse models of neuronal ceroid lipofuscinosis (NCL) exhibit many features of the human disorder, with widespread regional atrophy and significant loss of GABAergic interneurons in the hippocampus and neocortex. Reactive gliosis is a characteristic of all forms of NCL, but it is unclear whether glial activation precedes or is triggered by neuronal loss. To explore this issue we undertook detailed morphological characterization of the Cln3 null mutant (Cln3(-/-)) mouse model of juvenile NCL (JNCL) that revealed a delayed onset neurodegenerative phenotype with no significant regional atrophy, but with widespread loss of hippocampal interneurons that was first evident at 14 months of age. Quantitative image analysis demonstrated upregulation of markers of astrocytic and microglial activation in presymptomatic Cln3(-/-) mice at 5 months of age, many months before significant neuronal loss occurs. These data provide evidence for subtle glial responses early in JNCL pathogenesis.

Obesity increases the risk of renal involvement in children with Henoch-Schönlein purpura.

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Zhao, Yong-Li; Liu, Zheng-Juan; Bai, Xue-Mei; Wang, Yu-Chuan; Li, Guo-Hua; Yan, Xue-Yan

2015-10-01

The main aim of this study was to evaluate the relationship between obesity and renal involvement in children with Henoch-Schönlein purpura (HSP). A retrospective study of 141 pediatric patients with HSP was conducted in our hospital. The clinical data of all patients were collected from the electronic medical record management system from January 2010 to June 2014. The possible risk factors of renal involvement, especially obesity, were analyzed using univariate and multivariate analyses. Renal involvement occurred in 45/141 of the patients. A univariate analysis showed that an age more than 7 years at onset, persistent purpura, obesity, time from symptoms onset to diagnosis more than 14 days, and decreased C3 all increased the risk of renal involvement in HSP. The forward stepwise logistic regression analysis indicated obesity (odds ratio (OR) 4.43, 95 % confidence interval (CI) 1.896 to 10.358), age more than 7 years at onset (OR 2.81, 95 % CI 1.142 to 6.907), and persistent purpura (OR 2.57, 95 % CI 1.119 to 5.909) were independent risk factors for renal involvement. Our results show that obesity can increase the hazard of renal involvement in children with HSP and reconfirm that older age at onset and persistent purpura are the independent risk factors for renal involvement. • There have been some reports that obesity was associated with the development of renal injury. • It is not clear whether obesity can increase the risk of renal involvement in children with HSP. What is New: • The main finding of this study is that obesity can increase the hazard of renal involvement in children with HSP.

Treatment of Juvenile Idiopathic Arthritis-Associated Uveitis

OpenAIRE

Oray, Merih; Tu?al-Tutkun, ?lknur

2016-01-01

Pediatric uveitis may be a serious health problem because of the lifetime burden of vision loss due to severe complications if the problem is not adequately treated. Juvenile idiopathic arthritis (JIA)-associated uveitis is characterized by insidious onset and potentially blinding chronic anterior uveitis. Periodic ophthalmologic screening is of utmost importance for early diagnosis of uveitis. Early diagnosis and proper immunomodulatory treatment are essential for good visual progno...

Liraglutide and Renal Outcomes in Type 2 Diabetes

DEFF Research Database (Denmark)

Mann, Johannes F E; Ørsted, David D; Brown-Frandsen, Kirstine

2017-01-01

BACKGROUND: In a randomized, controlled trial that compared liraglutide, a glucagon-like peptide 1 analogue, with placebo in patients with type 2 diabetes and high cardiovascular risk who were receiving usual care, we found that liraglutide resulted in lower risks of the primary end point (nonfatal...... patients were assigned to receive liraglutide or placebo. The secondary renal outcome was a composite of new-onset persistent macroalbuminuria, persistent doubling of the serum creatinine level, end-stage renal disease, or death due to renal disease. The risk of renal outcomes was determined with the use...

Adult-Onset Metachromatic Leukodystrophy: Two Cases

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Gaye Eryaşar

2011-12-01

Full Text Available Metachromatic Leukodystrophy(MLD is a lisosomal storage disorder which is characterized with arylsulphatase A deficiency. Enzyme deficiency results with demiyelination and storage of sulphatides in central nervous system.According to onset age;the disease has three major clinical forms as late infantile,juvenile and adult form. It is a rare disorder. For the patients who did not develop neurological findings bone marrow or hematopoietic stem cell transplantation may be effective as treatment.

Evaluation of the ACR and SLICC classification criteria in juvenile-onset systemic lupus erythematosus: a longitudinal analysis.

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Lythgoe, H; Morgan, T; Heaf, E; Lloyd, O; Al-Abadi, E; Armon, K; Bailey, K; Davidson, J; Friswell, M; Gardner-Medwin, J; Haslam, K; Ioannou, Y; Leahy, A; Leone, V; Pilkington, C; Rangaraj, S; Riley, P; Tizard, E J; Wilkinson, N; Beresford, M W

2017-10-01

Objectives The Systemic Lupus International Collaborating Clinics (SLICC) group proposed revised classification criteria for systemic lupus erythematosus (SLICC-2012 criteria). This study aimed to compare these criteria with the well-established American College of Rheumatology classification criteria (ACR-1997 criteria) in a national cohort of juvenile-onset systemic lupus erythematosus (JSLE) patients and evaluate how patients' classification criteria evolved over time. Methods Data from patients in the UK JSLE Cohort Study with a senior clinician diagnosis of probable evolving, or definite JSLE, were analyzed. Patients were assessed using both classification criteria within 1 year of diagnosis and at latest follow up (following a minimum 12-month follow-up period). Results A total of 226 patients were included. The SLICC-2012 was more sensitive than ACR-1997 at diagnosis (92.9% versus 84.1% p < 0.001) and after follow up (100% versus 92.0% p < 0.001). Most patients meeting the SLICC-2012 criteria and not the ACR-1997 met more than one additional criterion on the SLICC-2012. Conclusions The SLICC-2012 was better able to classify patients with JSLE than the ACR-1997 and did so at an earlier stage in their disease course. SLICC-2012 should be considered for classification of JSLE patients in observational studies and clinical trial eligibility.

Analysis of CD45- [CD34+/KDR+] endothelial progenitor cells as juvenile protective factors in a rat model of ischemic-hemorrhagic stroke.

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Julius L Decano

Full Text Available Identification of juvenile protective factors (JPFs which are altered with age and contribute to adult-onset diseases could identify novel pathways for reversing the effects of age, an accepted non-modifiable risk factor to adult-onset diseases. Since endothelial progenitor cells (EPCs have been observed to be altered in stroke, hypertension and hypercholesterolemia, said EPCs are candidate JPFs for adult-onset stroke. A priori, if EPC aging plays a 'master-switch JPF-role' in stroke pathogenesis, juvenile EPC therapy alone should delay stroke-onset. Using a hypertensive, transgenic-hyperlipidemic rat model of spontaneous ischemic-hemorrhagic stroke, spTg25, we tested the hypothesis that freshly isolated juvenile EPCs are JPFs that can attenuate stroke progression and delay stroke onset.FACS analysis revealed that CD45- [CD34+/KDR+] EPCs decrease with progression to stroke in spTg25 rats, exhibit differential expression of the dual endodthelin-1/VEGFsp receptor (DEspR and undergo differential DEspR-subtype specific changes in number and in vitro angiogenic tube-incorporation. In vivo EPC infusion of male, juvenile non-expanded cd45-[CD34+/KDR+] EPCs into female stroke-prone rats prior to stroke attenuated progression and delayed stroke onset (P<0.003. Detection of Y-chromosome DNA in brain microvessels of EPC-treated female spTg25 rats indicates integration of male EPCs into female rat brain microvessels. Gradient-echo MRI showed delay of ischemic-hemorrhagic lesions in EPC-treated rats. Real-time RT-PCR pathway-specific array-analysis revealed age-associated gene expression changes in CD45-[CD34+/KDR]EPC subtypes, which were accelerated in stroke-prone rats. Pro-angiogenic genes implicated in intimal hyperplasia were increased in stroke-prone rat EPCs (P<0.0001, suggesting a maladaptive endothelial repair system which acts like a double-edged sword repairing while predisposing to age-associated intimal hyperplasia.Altogether, the data

High risk of renal dysfunction in patients with fulminant type 1 diabetes

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Takahashi, Nobuyuki; Tsujimoto, Tetsuro; Chujo, Daisuke; Kajio, Hiroshi

2017-01-01

Abstract Aims/Introduction To compare the incidence rate of renal dysfunction between patients with fulminant type 1 diabetes and those with acute‐onset type 1 diabetes. Materials and Methods The present retrospective cohort study included patients with fulminant type 1 diabetes and acute‐onset type 1 diabetes diagnosed during April 1993 to March 2016 at a national center in Japan. Glycated hemoglobin levels, incidence rates of renal dysfunction defined as an estimated glomerular filtration r...

The dark side of the kidney in cardio-renal syndrome: renal venous hypertension and congestive kidney failure.

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Di Nicolò, Pierpaolo

2018-03-01

Renal involvement in some forms of acute or chronic diseases, such as heart failure or sepsis, presents with a complex pathophysiological basis that is not always clearly distinguishable. In these clinical settings, kidney failure is traditionally and almost exclusively attributed to renal hypoperfusion and it is commonly accepted that causal elements are pre-renal, such as a reduction in the ejection fraction or absolute or relative hypovolemia acting directly on oxygen transport mechanisms and renal autoregulation systems, causing a reduction of glomerular filtration rate. Nevertheless, the concept emerging from accumulating clinical and experimental evidence is that in complex clinical pictures, kidney failure is strongly linked to the hemodynamic alterations occurring in the renal venous micro and macrocirculation. Accordingly, the transmission of the increased venous pressure to the renal venous compartment and the consequent increasing renal afterload has a pivotal role in determining and sustaining the kidney damage. The aim of this review was to clarify the physiopathological aspects of the link between worsening renal function and renal venous hypertension, analyzing the prognostic and therapeutic implications of the so-called congestive kidney failure in cardio-renal syndrome and in other clinical contexts of its possible onset.

Expansion of CD14+CD16+ monocytes producing TNF-α in complication-free diabetes type 1 juvenile onset patients.

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Myśliwska, Jolanta; Smardzewski, Marcin; Marek-Trzonkowska, Natalia; Myśliwiec, Małgorzata; Raczyńska, Krystyna

2012-10-01

We concentrated on the complication-free phase of juvenile onset type 1 diabetes mellitus (T1DM) searching for associations between concentration of inflammatory factors TNF-α, CRP and VEGF and two monocyte subsets the CD14(++)CD16(-) and CD14(+)CD16(+). We analysed a randomly selected group of 150 patients without complications (disease duration 2.74 ± 2.51 years) at the start of the project and 5 years later. They were compared with 24 patients with retinopathy (6.53 ± 3.39 years of disease) and 30 healthy volunteers. Our results indicate that in the complication-free period the concentration of TNF-α significantly increased and continued to increase after retinopathy was established. After 5 years the percentage and absolute number of CD14(+)CD16(+) monocytes doubled in complication-free patients. Our study indicates that the size of CD14(+)CD16(+) monocyte subset may be used alternatively to CRP values as an indicator of inflammation grade. Our results imply the necessity of trials using anti-TNF-α therapy in the complication-free phase of the disease. Copyright © 2012 Elsevier Ltd. All rights reserved.

Working With Female Juvenile Delinquents: What Youth Practitioners Need to Know

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Joy D. Patton

2008-09-01

Full Text Available This article is organized in a way to help youth practitioners recognize the most pertinent issues faced by female juveniles and to provide help in guiding professional interactions, communication and decision-making. The guidelines discussed are suggestions for practice based on an empirical review of the literature. Recent research has identified ten characteristics of female juvenile offenders to consider when working with this population. These areas include: (a impaired cognitive functioning, (b low academic achievement, (c weak language skills, (d peer relationships, (e onset of menarche, (f early sexual experiences, (g mental illness, (h victimization (i low self-esteem and (j race.

Renal hemangioma

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Theodorico F. da Costa Neto

2004-06-01

Full Text Available INTRODUCTION: Renal hemangioma is a relatively rare benign tumor, seldom diagnosed as a cause of hematuria. CASE REPORT: A female 40-year old patient presented with continuous gross hematuria, anemia and episodic right lumbar pain, with onset about 3 months previously. The patient underwent multiple blood transfusions during her hospital stay and extensive imaging propedeutics was performed. Semi-rigid ureterorenoscopy evidenced a bleeding focus in the upper calix of the right kidney, with endoscopic treatment being unfeasible. The patient underwent right upper pole nephrectomy and presented a favorable outcome. Histopathological analysis of the surgical specimen showed that it was a renal hemangioma. COMMENTS: Imaging methods usually employed for diagnostic investigation of hematuria do not have good sensitivity for renal hemangioma. However, they are important to exclude the most frequent differential diagnoses. The ureterorenoscopy is the diagnostic method of choice and endoscopic treatment can be feasible when the lesion is accessible and electrocautery or laser are available. We emphasize the open surgical treatment as a therapeutic option upon failure of less invasive methods.

Analysis of the Juvenile Idiopathic Arthritis Immunization Schedule

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L. S. Namazova-Baranova

2016-01-01

Full Text Available Background: The connection between vaccination and autoimmune diseases (and rheumatic pathology in particular is still a subject of discussions. When discussing the possibility of vaccinating rheumatic patients we should take into account the ultra high dangers that infectious diseases pose for such patients, including those that can be prevented by vaccination. We should also take into account the experience of using various vaccine types in rheumatic patients, which illustrates of their high safety profile.Objective: Our aim was to study the immunization schedule in children with juvenile idiopathic arthritis.Methods: The evaluation of vaccine history and other anamnestic data in juvenile idiopathic arthritis patients was based on individual medical records (individual child’s card/preventive vaccination certificate, as well as questionnaires filled by mothers.Results: It has been determined that a significant proportion of children with vaccination schedule deviations are juvenile idiopathic arthritis patients. Almost one in four children with a confirmed rheumatic diagnosis has not been immunized against the major vaccine-preventable diseases. In one non-vaccinated group, there was a case of juvenile arthritis onset after recovering from measles. A small number of patient mothers connects the manifestation of rheumatic diseases with vaccination.Conclusion: Violations of vaccination status in JIA patients require corrections according to the results of clinical studies and the recommendations of international experts.

Nephrogenic systemic fibrosis symptoms alleviated by renal transplantation

DEFF Research Database (Denmark)

Hansen, Jesper Melchior

2011-01-01

are limited. Anecdotal reports have shown partial or complete resolution of NSF following successful renal transplantation early in the course of NSF. In this report, we describe alleviation of NSF symptoms in two women following successful renal transplantation more than 3 years after onset of NSF.......Nephrogenic systemic fibrosis (NSF) is a rare, serious, and life-threatening disease of patients with severe renal impairment. Gadolinium-containing contrast agents have been shown to be the crucial trigger. There is no proven medical cure for the disease, and symptomatic treatment options...

Renal vasculitis presenting with acute kidney injury.

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Villacorta, Javier; Diaz-Crespo, Francisco; Acevedo, Mercedes; Cavero, Teresa; Guerrero, Carmen; Praga, Manuel; Fernandez-Juarez, Gema

2017-06-01

Renal failure secondary to ANCA-associated vasculitis represents a clinical and therapeutic challenge. In this study, we aimed to assess the treatment response rates and long-term outcomes of vasculitis patients presenting with renal failure. This retrospective study included 151 patients with renal vasculitis from three hospitals who underwent a renal biopsy between 1997 and 2014. Patients with renal failure which required dialysis at the onset were compared to those presenting with more preserved renal function. The primary end point was treatment response and patient surivival. Patients with severe renal involvement had a lower response to treatment compared to those having preserved renal function (26.6 versus 93.4%; p renal recovery (41.6 versus 12.5%; p = 0.05). A higher incidence of severe infections was observed among patients with severe renal involvement (38.4 versus 18.1%, p = 0.01). The mortality rate was significantly higher among vasculitis patients presenting with renal failure (53.8 versus 22.2%, p = 0.001). Global survival at 1 and 5 years was 60 and 47% in patients requiring dialysis compared with 90 and 80% among those with more preserved renal function (p renal dysfunction represents an independent risk factor for patient survival in renal vasculitis. Patients requiring dialysis associate a lower response rate to immunosuppressive therapy and a higher incidence of severe infections.

Late-onset Bartter syndrome type II.

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Gollasch, Benjamin; Anistan, Yoland-Marie; Canaan-Kühl, Sima; Gollasch, Maik

2017-10-01

Mutations in the ROMK1 potassium channel gene ( KCNJ1 ) cause antenatal/neonatal Bartter syndrome type II (aBS II), a renal disorder that begins in utero , accounting for the polyhydramnios and premature delivery that is typical in affected infants, who develop massive renal salt wasting, hypokalaemic metabolic alkalosis, secondary hyperreninaemic hyperaldosteronism, hypercalciuria and nephrocalcinosis. This BS type is believed to represent a disorder of the infancy, but not in adulthood. We herein describe a female patient with a remarkably late-onset and mild clinical manifestation of BS II with compound heterozygous KCNJ1 missense mutations, consisting of a novel c.197T > A (p.I66N) and a previously reported c.875G > A (p.R292Q) KCNJ1 mutation. We implemented and evaluated the performance of two different bioinformatics-based approaches of targeted massively parallel sequencing [next generation sequencing (NGS)] in defining the molecular diagnosis. Our results demonstrate that aBS II may be suspected in patients with a late-onset phenotype. Our experimental approach of NGS-based mutation screening combined with Sanger sequencing proved to be a reliable molecular approach for defining the clinical diagnosis in our patient, and results in important differential diagnostic and therapeutic implications for patients with BS. Our results could have a significant impact on the diagnosis and methodological approaches of genetic testing in other patients with clinical unclassified phenotypes of nephrocalcinosis and congenital renal electrolyte abnormalities.

JUVENILE SCLERODERMA-what has changed in the meantime?

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Adrovic, Amra; Sahin, Sezgin; Barut, Kenan; Kasapcopur, Ozgur

2018-04-22

Juvenile scleroderma is a rarely seen chronic connective tissue disorder characterized by stiffening of the skin. The frequency of the disease was reported as one per million. According to organ involvement, the disease is divided into two main forms: systemic and localized scleroderma. Since it is uncommon in children, many aspects of the disease remain discussable. With this review, we aimed to revise recent findings and new developments in this rare condition. Skin manifestations are most prominent feature of the systemic form, followed by musculoskeletal and vascular involvement. Cardiovascular, gastrointestinal and renal disorders are rare in childhood. Combination of disease modifying antirheumatic drugs (methotrexate, mycophenolate-mofetil, cyclosporine) and steroid reprents the first line therapy. Bosentan is used for cases with pulmonary hypertension and for extensive digital ulcerations. Biological treatment emerges as a useful treatment option in most severe form of the disease. Localized scleroderma is characterized with sclerodermatosis of the skin. Internal organ involvement is not expected. Classification of the local scleroderma is made according to the size and localization of the skin changes. There are few different therapeutical options but there is no specific therapy for the localized scleroderma. Many data regarding disease features and treatment options in juvenile scleroderma are based on studies among adults. There is a striking need for multicentric, prospective studies among children with juvenile scleroderma.Emerging biological agents and new treatment options are showing promising results. Anyhow, juvenile scleroderma remains a mystery with many aspects of the disease waiting to be solved. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

Oromandibular Dyskinesia as the Initial Manifestation of Late-Onset Huntington Disease

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Dong-Seok Oh

2011-10-01

Full Text Available Huntington’s disease (HD is a neurodegenerative disorder characterized by a triad of choreoathetosis, dementia and dominant inheritance. The cause of HD is an expansion of CAG trinucleotide repeats in the HD gene. Typical age at onset of symptoms is in the 40s, but the disorder can manifest at any time. Late-onset (≥ 60 years HD is clinically different from other adult or juvenile onset HD and characterized by mild motor problem as the initial symptoms, shorter disease duration, frequent lack of family history, and relatively low CAG repeats expansion. We report a case of an 80-year-old female with oromandibular dyskinesia as an initial manifestation of HD and 40 CAG repeats.

Risk factors for aggressive recurrent respiratory papillomatosis in adults and juveniles.

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Turid Omland

Full Text Available In this cohort study we examined whether gender, age at onset, observation time or human papillomavirus (HPV genotype are risk factors for an aggressive clinical course in Recurrent Respiratory Papillomatosis (RRP. Clinical data from patient records comprised gender, age at onset, date of first endolaryngeal procedure with biopsy, date of last follow-up, total number of endolaryngeal procedures, and complications during the observation period. Disease was defined as juvenile (JoRRP or adult onset (AoRRP according to whether the disease was acquired before or after the age of 18. Aggressive disease was defined as distal spread, tracheostomy, four surgical operations annually or >10 surgeries in total. DNA was extracted from formalin-fixed paraffin-embedded tissue. HPV genotyping was performed by quantitative PCR assay identifying 15 HPV genotypes. The study included 224 patients. The majority were males (141/174 in AoRRPs and 31/50 in JoRRPs; p = 0.005. The median follow-up from initial diagnosis was 12.0 years (IQR 3.7-32.9 for JoRRPs and 4.0 years (IQR 0.8-11.7 for AoRRPs. The disease was more aggressive in juveniles than adults (p10 years (OR = 13.41, 95% CI [5.46, 32.99[, p10 years were risk factors for an aggressive disease course in AoRRPs.

Systemic onset juveniile chronic arthritis (JCA) in a Nigerian boy: a ...

African Journals Online (AJOL)

Background: Juvenile chronic arthritis (JCA) is a chronic arthritis affecting children below age of 16 years. The systemic onset subgroup is also known as Still's disease. There are several distinct subgroups. There is paucity of literature of this disease entity in our environment due to under diagnosis of the disease. Method: ...

Clinical and Biochemical Characteristics of Children with Juvenile Idiopathic Arthritis

International Nuclear Information System (INIS)

Ahmed, S.; Ali, S. R.; Ishaque, S.

2014-01-01

Objective: To determine the clinical and biochemical characteristics of children with Juvenile Idiopathic Arthritis (JIA) at a tertiary care centre in Karachi, Pakistan. Study Design: A descriptive study. Place and Duration of Study: Paediatric Rheumatology Clinic of The Aga Khan University Hospital (AKUH), Karachi, from January 2008 to December 2011. Methodology: Clinical and laboratory profile and outcome of children less than 15 years of age attending the Paediatric Rheumatology Clinic of the Aga Khan University, Karachi with the diagnosis of Juvenile Idiopathic Arthritis according to International League against Rheumatism were studied. These children were classified into different types of JIA; their clinical and laboratory characteristics, response to therapy and outcome was evaluated. Results: Sixty eight patients satisfying the criteria of International League against Rheumatism (ILAR) for Juvenile Idiopathic Arthritis were enrolled during the study period of four consecutive years, their age ranged from 9 months to 15 years. Mean age at onset was 6.45 +- 4.03 years while mean age at diagnosis was 7.60 +- 3.93 years. Polyarticular was the most predominant subtype with 37 (54%) patients, out of these, 9 (24%) were rheumatoid factor positive. An almost equal gender predisposition was observed. Fever and arthritis were the most common presenting symptoms, with only 2 patients presenting with uveitis. Conclusion: The clinico-biochemical characteristics of JIA at the study centre showed a pattern distinct with early onset of disease, high frequency of polyarticular type and a higher rheumatoid factor (QRA) and ANA positivity in girls. (author)

Adult-onset juvenile xanthogranuloma of the external auditory canal: A case report

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Hur, Joon Ho; Kim, Jae Kyun; Seo, Gi Young; Choi, Woo Sun; Byun, Jun Soo; Lee, Woong Jae; Lee, Tae Jin [Chung-Ang University College of Medicine, Chung-Ang University Hospital, Seoul (Korea, Republic of); Kim, Na Ra [Dept. of Radiology, Samsung Medical Center, Sungkyunkwan University College of Medicine, Seoul (Korea, Republic of)

2016-05-15

Juvenile xanthogranuloma (JXG) is a benign, spontaneously regressing lesion that usually occurs during the first year of life, but may also occur in adulthood. Although the most common presentation of JXG is the cutaneous lesion, it can also manifest in various visceral organs. JXG of the external auditory canal is extremely rare, and there have been only a few reports of those cases in the English literature. In this study, we present a case of pathologically proven JXG that occurred in the external auditory canal with a symptomatic clinical presentation.

Serial elongation-derotation-flexion casting for children with early-onset scoliosis.

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Canavese, Federico; Samba, Antoine; Dimeglio, Alain; Mansour, Mounira; Rousset, Marie

2015-12-18

Various early-onset spinal deformities, particularly infantile and juvenile scoliosis (JS), still pose challenges to pediatric orthopedic surgeons. The ideal treatment of these deformities has yet to emerge, as both clinicians and surgeons still face multiple challenges including preservation of thoracic motion, spine and cage, and protection of cardiac and lung growth and function. Elongation-derotation-flexion (EDF) casting is a technique that uses a custom-made thoracolumbar cast based on a three-dimensional correction concept. EDF can control progression of the deformity and - in some cases-coax the initially-curved spine to grow straighter by acting simultaneously in the frontal, sagittal and coronal planes. Here we provide a comprehensive review of how infantile and JS can affect normal spine and thorax and how serial EDF casting can be used to manage these spinal deformities. A fresh review of the literature helps fully understand the principles of the serial EDF casting technique and the effectiveness of conservative treatment in patients with early-onset spinal deformities, particularly infantile and juvenile scolisois.

Reversible posterior leukoencephalopathy syndrome secondary to systemic-onset juvenile idiopathic arthritis: A case report and review of the literature.

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Zhang, Pingping; Li, Xiaofeng; Li, Yating; Wang, Jing; Zeng, Huasong; Zeng, Xiaofeng

2015-01-01

Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinical syndrome based on changes in clinical imaging, and it has been reported to mainly occur in adults. However, it has been recently discovered that RPLS is also prevalent in infant patients, particularly in those using glucocorticoids, immunosuppressant medications and cytotoxic drugs. The current study presents a 5-year-old male with a previous diagnosis of systemic-onset juvenile idiopathic arthritis (SoJIA) and macrophage-activation syndrome who developed posterior reversible encephalopathy syndrome during treatment with glucocorticoids, disease-modifying antirheumatic drugs and biological agent (etanercept) therapy. After ~5 days of treatment, the patient made a complete clinical recovery; the magnetic resonance imaging reviewed 2 weeks later showed that the previous hyper-intensity signal had disappeared and the multiple lesions in the brain had been completely absorbed. The case report shows that, conforming to recent literature, SoJIA in infants should be considered a risk factor for developing RPLS. The clinical manifestations of the disease are multiple, but usually reversible, and the patients mostly have a good prognosis. Rapid diagnosis and treatment is essential as early treatment may prevent progression to irreversible brain damage. By increasing the awareness of RPLS, the patient care may improve and further insight may be gained.

Pre-transplantation glucose testing for predicting new-onset diabetes mellitus after renal transplantation.

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Ramesh Prasad, G V; Huang, M; Bandukwala, F; Nash, M M; Rapi, L; Montada-Atin, T; Meliton, G; Zaltzman, J S

2009-02-01

New-onset diabetes after renal transplantation (NODAT) adversely affects graft and patient survival. However, NODAT risk based on pre-transplant blood glucose (BG) levels has not been defined. Our goal was to identify the best pre-transplant testing method and cut-off values. We performed a case-control analysis of non-diabetic recipients who received a live donor allograft with at least 6 months post-transplant survival. Pre-transplant glucose abnormalities were excluded through 75 g oral glucose tolerance testing (OGTT) and random BG (RBG) measurement. NODAT was defined based on 2003 Canadian Diabetes Association criteria. Multivariate logistic and Cox regression analysis was performed to determine independent predictor variables for NODAT. Receiver-operating-characteristic (ROC) curves were constructed to determine threshold BG values for diabetes risk. 151 recipients met initial entry criteria. 12 had pre-transplant impaired fasting glucose and/or impaired glucose tolerance, among who 7 (58%) developed NODAT. In the remaining 139, 24 (17%) developed NODAT. NODAT risk exceeded 25% for those with pre-transplant RBG > 6.0 mmol/l and 50% if > 7.2 mmol/l. Pre-transplant RBG provided the highest AUC (0.69, p = 0.002) by ROC analysis. Increasing age (p = 0.025), acute rejection (p = 0.011), and RBG > 6.0 mmol/l (p = 0.001) were independent predictors of NODAT. Pre-transplant glucose testing is a specific marker for NODAT. Patients can be counseled of their incremental risk even within the normal BG range if the OGTT is normal.

Growth speed in patients with chronic renal failure undergoing to renal transplantation between 2000 and 2009 in the Hospital Nacional de Ninos: research protocol

International Nuclear Information System (INIS)

Arroyo Molina, Ana Victoria

2013-01-01

The growth speed was investigated in children with chronic renal failure after renal transplantation, in the Hospital Nacional de Ninos during the study period January 2000-December 2009. Factors that have influenced are analyzed: age of onset of renal disease, etiology of renal disease, metabolic acidosis, anemia, renal osteodystrophy, episodes of infection and rejection. Besides, on the growth rate and expected family size, to intervene or prevent them in future cases. Also, the use that has given in the hospital to growth hormone, before and after renal transplantation is determined to eventually use parallel therapies to the transplantation. An echocardiographic study is recommended to perform as part of the treatment of chronic renal failure to identify the existence of left ventricular hypertrophy and heart failure, which may occur as a result of complications of the failure [es

Juvenile angiofibroma

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Nasal tumor; Angiofibroma - juvenile; Benign nasal tumor; Juvenile nasal angiofibroma; JNA ... Juvenile angiofibroma is not very common. It is most often found in adolescent boys. The tumor contains many blood ...

Tocilizumab: The evidence for its place in the treatment of juvenile idiopathic arthritis

DEFF Research Database (Denmark)

Herlin, Troels

2010-01-01

INTRODUCTION: Juvenile idiopathic arthritis (JIA) is one of the most common chronic diseases with childhood onset. It comprises different subtypes of which the systemic onset subtype is often resistant to treatment. With the advent of biological treatment with tumor necrosis factor-alpha (TNFalpha......)-inhibitors, the clinical outcome of JIA has improved considerably, but only for subtypes other than systemic JIA. Substantial evidence shows that the proinflammatory cytokine interleukin-6 (IL-6) plays a pivotal role in systemic JIA. The blockage of IL-6 action by tocilizumab, a humanized anti-IL-6-receptor monoclonal...

Effects of obesity on health-related quality of life in juvenile-onset systemic lupus erythematosus.

Science.gov (United States)

Mina, R; Klein-Gitelman, M S; Nelson, S; Eberhard, B A; Higgins, G; Singer, N G; Onel, K; Tucker, L; O'Neil, K M; Punaro, M; Levy, D M; Haines, K; Ying, J; Brunner, H I

2015-02-01

This study evaluated the effects of obesity on health-related quality of life (HRQOL) measures in juvenile-onset systemic lupus erythematosus (jSLE). Obesity was defined as a body mass index (BMI) ≥ 95 th percentile according to the Sex-specific Center for Disease Control BMI-For-Age Charts and determined in a multicenter cohort of jSLE patients. In this secondary analysis, the domain and summary scores of the Pediatric Quality of Life (PedsQL) Inventory and the Child Health Questionnaire (CHQ) of obese jSLE patients were compared to those of non-obese jSLE patients as well as historical obese and non-obese healthy controls. Mixed-effects modeling was performed to evaluate the relationship between obesity and HRQOL measures. Among the 202 jSLE patients, 25% (n = 51) were obese. Obesity had a significant negative impact on HRQOL in jSLE, even after adjusting for differences in current corticosteroid use, disease activity, disease damage, gender and race between groups. Obese jSLE patients had lower physical functioning compared to non-obese jSLE patients, and to non-obese and obese healthy controls. Compared to their non-obese counterparts, obese jSLE patients also had worse school functioning, more pain, worse social functioning and emotional functioning. Parents of obese jSLE patients worry more. The CHQ scores for obese jSLE patients were also worse compared to non-obese jSLE patients in several other domains. Our study demonstrates the detrimental effects of obesity on patient-reported outcomes in jSLE. This supports the importance of weight management for the therapeutic plan of jSLE. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

Renal denervation decreases blood pressure and renal tyrosine hydroxylase but does not augment the effect of hypotensive drugs.

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Skrzypecki, Janusz; Gawlak, Maciej; Huc, Tomasz; Szulczyk, Paweł; Ufnal, Marcin

2017-01-01

The effect of renal denervation on the efficacy of antihypertensive drugs has not yet been elucidated. Twenty-week-old spontaneously hypertensive rats were treated with metoprolol, losartan, indapamide, or saline (controls) and assigned to renal denervation or a sham procedure. Acute hemodynamic measurements were performed ten days later. Series showing a significant interaction between renal denervation and the drugs were repeated with chronic telemetry measurements. In the saline series, denervated rats showed a significantly lower mean arterial blood pressure (blood pressure) than the sham-operated rats. In contrast, in the metoprolol series denervated rats showed a significantly higher blood pressure than sham rats. There were no differences in blood pressure between denervated and sham rats in the losartan and indapamide series. In chronic studies, a 4-week treatment with metoprolol caused a decrease in blood pressure. Renal denervation and sham denervation performed 10 days after the onset of metoprolol treatment did not affect blood pressure. Denervated rats showed markedly reduced renal nerve tyrosine hydroxylase levels. In conclusion, renal denervation decreases blood pressure in hypertensive rats. The hypotensive action of metoprolol, indapamide, and losartan is not augmented by renal denervation, suggesting the absence of synergy between renal denervation and the drugs investigated in this study.

[Acute renal failure: a rare presentation of Addison's disease].

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Salhi, Houda

2016-01-01

Addison's disease is a rare condition. Its onset of symptoms most often is nonspecific contributing to a diagnostic and therapeutic delay. Acute renal failure can be the first manifestation of this disease. We report the case of a patient with Addison's disease who was initially treated for acute renal failure due to multiple myeloma and whose diagnosis was adjusted thereafter. Patient's condition dramatically improved after treatment with intravenous rehydration; injectable hydrocortisone.

Dyslipidaemia among renal transplant recipients: cyclosporine versus tacrolimus.

Science.gov (United States)

Fazal, Muhammad Asim; Idrees, Muhammad Khalid; Akhtar, Syed Fazal

2014-05-01

To compare new onset dyslipidaemia in live-related renal transplant recipients taking cyclosporine versus tacrolimus after 3 months of therapy. The randomised controlled trial was conducted at the Sindh Institute of Urology and Transplantation (SIUT) Karachi, from September 2010 to April 2011, and included 182 End Stage Renal Disease patients on maintenance haemodialysis with pre-transplant normal lipid profile. The patients, who had live-related renal transplant, were randomly allocated to two equal groups using lottery. Group A received cyclosporine (3 mg/kg) and group B was treated with tacrolimus (0.1 mg/kg). All patients had pre-transplant fasting lipid profile checked when they were on maintenance haemodialysis and 3 months after renal transplantation. Serum fasting lipid profile was collected by taking 5 ml blood by venipuncture after an overnight fast of 9-12 hours. SPSS 10 was used for statistical analyses. Of the 182 patients, 144 (79.1%) were males and 38 (20.9%) were females. The overall mean age was 30.18 +/- 9.57 years, and the mean weight was 54.41 +/- 11.144 kg. Significant difference was not observed between the two groups regarding age and weight of the patients. Dyslipidaemia was found in 115(63.2%) subjects; 61(67%) in group A and 54 (59.3%) in group B. There was no statistical difference (p=0.28) when comparison was done after 3 months of therapy. The occurrence of new onset hyperlipidaemia is similar in renal transplant recipients receiving either cyclosporine or tacrolimus in first 3 months post-transplant, but there is room for more research in this field as dyslipidaemia following successful renal transplantation is a frequent and persistent complication.

Effects of natal departure and water level on survival of juvenile snail kites (Rostrhamus sociabilis) in Florida

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Dreitz, V.J.; Kitchens, W.M.; DeAngelis, D.L.

2004-01-01

Survival rate from fledging to breeding, or juvenile survival, is an important source of variation in lifetime reproductive success in birds. Therefore, determining the relationship between juvenile survival and environmental factors is essential to understanding fitness consequences of reproduction in many populations. With increases in density of individuals and depletion of food resources, quality of most habitats deteriorates during the breeding season. Individuals respond by dispersing in search of food resources. Therefore, to understand the influence of environmental factors on juvenile survival, it is also necessary to know how natal dispersal influences survival of juveniles. We examined effects of various environmental factors and natal dispersal behavior on juvenile survival of endangered Snail Kites (Rostrhamus sociabilis) in central and southern Florida, using a generalized estimating equations (GEEs) approach and model selection criteria. Our results suggested yearly effects and an influence of age and monthly minimum hydrologic levels on juvenile Snail Kite survival. Yearly variation in juvenile survival has been reported by other studies, and other reproductive components of Snail Kites also exhibit such variation. Age differences in juvenile survival have also been seen in other species during the juvenile period. Our results demonstrate a positive relationship between water levels and juvenile survival. We suggest that this is not a direct linear relationship, such that higher water means higher juvenile survival. The juvenile period is concurrent with onset of the wet season in the ecosystem we studied, and rainfall increases as juveniles age. For management purposes, we believe that inferences suggesting increasing water levels during the fledging period will increase juvenile survival may have short-term benefits but lead to long-term declines in prey abundance and possibly wetland vegetation structure.

Erythrocyte Sedimentation Rate as Baseline Predictor for the Development of Uveitis in Children With Juvenile Idiopathic Arthritis

NARCIS (Netherlands)

Haasnoot, Arenda J W; van Tent-Hoeve, Maretta; Wulffraat, Nico M; Schalij-Delfos, Nicoline E; Los, Leonoor I; Armbrust, Wineke; Zuithoff, Nicolaas P A; de Boer, Joke H

PURPOSE: To analyze inflammatory parameters as possible predictors for the development of uveitis in juvenile idiopathic arthritis (JIA) patients. Further, to analyze the predictive value of demographic and clinical factors at the onset of arthritis. DESIGN: Retrospective cohort study. METHODS: In

TREATMENT APPROACH FOR JUVENILE IDIOPATHIC ARTHRITIS-RELATED UVEITIS: 2012 UPDATE

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Francesco Zulian

2012-01-01

Full Text Available Chronic anterior uveitis is the most common extra-articular complication of juvenile idiopathic arthritis. It is more frequent in the early onset forms with a higher prevalence in the oligoarticular (40% than in other juvenile idiopathic arthritis subtypes (5–14%. The risk for severe visual impairment is still high due to the development of sight-threatening complications (synechiae, band keratopathy, cataract, glaucoma, cystoid macular oedema. Treatment is not standardized and requires a complex decision-making process, involving a close collaboration between paediatric ophthalmologist and rheumatologist. Topical therapy alone is often inadequate to control ocular inflammation and bulbar injections are too invasive to perform in children therefore immunosuppressive treatment is often advocated. Low dose methotrexate is the second-line agent mostly used although no controlled studies comparing effects of early to late methotrexate treatment have been reported. Mycophenolate mofetil is effective in controlling inflammation in methotrexate -refractory patients. Its efficacy, however, seems to be more relevant in intermediate or posterior uveitis, than in juvenile idiopathic arthritis uveitis and scleritis. Anti-TNFα agents, namely infliximab and adalimimab showed effectiveness in open-label studies but no wide controlled trials have been reported so far. Adalimimab is as effective as infliximab but has an easier way of administration and a better drug tolerance. Abatacept should be used in anti-TNF refractory patients with juvenile idiopathic arthritis uveitis.

Juvenile generalized pustular psoriasis is a chronic recalcitrant disease: an analysis of 27 patients seen in a tertiary hospital in Johor, Malaysia.

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Lau, Bi-Wen; Lim, Dee-Zhen; Capon, Francesca; Barker, Jonathan N; Choon, Siew-Eng

2017-04-01

Limited information exists regarding juvenile generalized pustular psoriasis (GPP). We aim to determine the clinical profile and outcome of Malaysians with juvenile GPP. Review of hospital case notes on patients with juvenile GPP. Twenty-seven patients with juvenile GPP were identified. Female to male ratio was 1.4:1. The median age at onset of GPP was 6.5 years. Ten patients had prior psoriasis with a median pre-pustular duration of 2.7 years. Onset of GPP was earlier in patients without prior psoriasis (5.1 years vs. 12.0 years, P = 0.002). Precipitating factors identified included stress, upper respiratory tract infection, systemic steroid use, vaccination, and pregnancy. A positive family history of psoriasis and GPP was present in six and one patient(s), respectively. Twenty-one patients had acute, five annular, and one localized variant of GPP. Arthritis was present in 22.2%. Fever, leukocytosis, and transaminitis were mainly seen in patients with acute GPP at 80.9, 72.2, and 11.1%, respectively. Among 20 patients screened, eight carry IL36RN variants and one has CARD14 mutation. IL36RN-positive patients have more severe disease characterized by early onset, low prevalence of prior plaque psoriasis, high prevalence of systemic inflammation, and need for continuous long-term systemic therapy. Acitretin and cyclosporine were effective in aborting acute GPP in 100% of 16 and 66.7% of six patients treated, respectively. However, relapses were common. Only three of the 17 patients whose initial acute GPP was controlled with systemic agents were successfully weaned off treatment. Juvenile GPP is a chronic recalcitrant disease. IL36RN-positive patients have more severe disease. © 2017 The International Society of Dermatology.

The DSM-5 Limited Prosocial Emotions subtype of Conduct Disorder in incarcerated male and female juvenile delinquents.

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Pechorro, Pedro; Jiménez, Lucía; Hidalgo, Victoria; Nunes, Cristina

2015-01-01

The aim of the present study was to analyze the relevance of the DSM-5's Conduct Disorder new Limited Prosocial Emotions (CD LPE) specifier in incarcerated juvenile delinquents. A sample of 201 males and 98 females from the Juvenile Detention Centers managed by the Portuguese Ministry of Justice diagnosed with Conduct Disorder (CD) was used. Results showed that male juvenile delinquents with the CD LPE specifier scored higher on callous-unemotional traits (CU), general psychopathic traits, psychopathy taxon membership, self-reported delinquency, and crime seriousness, and lower on prosocial behavior and social desirability, while female juvenile delinquents with the CD LPE specifier scored higher on callous-unemotional traits (CU) and general psychopathic traits, and lower on prosocial behavior. Significant associations for both genders were found between the CD LPE specifier and age of crime onset and first problems with the law. Copyright © 2015 Elsevier Ltd. All rights reserved.

[Morphea or juvenile localised scleroderma: Case report].

Science.gov (United States)

Strickler, Alexis; Gallo, Silvanna; Jaramillo, Pedro; de Toro, Gonzalo

2016-01-01

Morphea or juvenile localised scleroderma (JLS) is an autoimmune, inflammatory, chronic, slowly progressive connective tissue disease of unknown cause that preferably affects skin and underlying tissues. To report a case of Juvenil Localised scleroderma in an 8-year old girl, contributing to an early diagnosis and treatment. The case is presented of an 8 year-old girl who presented with indurated hypopigmented plaques, of linear distribution in the right upper extremity of two years onset, together with papery texture hyperpigmented indurated plaques with whitish areas of thinned skin in right lower extremity, and leg and ankle swelling. The clinical features and diagnostic tests, including histology were compatible with linear and pansclerotic JLS. She started with immunosuppressive therapy, physiotherapy, and occupational therapy. We report a case of linear and pansclerotic ELJ type, in which there was a 2 year delay in diagnosis, however the response to treatment was positive as expected. Copyright © 2016 Sociedad Chilena de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

Dyslipidaemia among renal transplant recipients: cyclosporine versus tacrolimus

International Nuclear Information System (INIS)

Fazal, M. A.; Idrees, M. K.; Akhtar, S. F.

2014-01-01

Objectives: To compare new onset dyslipidaemia in live-related renal transplant recipients taking cyclosporine versus tacrolimus after 3 months of therapy. Methods: The randomised controlled trial was conducted at the Sindh Institute of Urology and Transplantation (SIUT) Karachi, from September 2010 to April 2011, and included 182 End Stage Renal Disease patients on maintenance haemodialysis with pre-transplant normal lipid profile. The patients, who had live-related renal transplant, were randomly allocated to two equal groups using lottery. Group A received cyclosporine (3mg/kg) and group B was treated with tacrolimus (0.1mg/kg). All patients had pre-transplant fasting lipid profile checked when they were on maintenance haemodialysis and 3 months after renal transplantation. Serum fasting lipid profile was collected by taking 5ml blood by venipuncture after an overnight fast of 9-12 hours. SPSS 10 was used for statistical analyses. Results: Of the 182 patients, 144(79.1%) were males and 38(20.9%) were females. The overall mean age was 30.18+-9.57 years, and the mean weight was 54.41+- 11.144kg. Significant difference was not observed between the two groups regarding age and weight of the patients. Dyslipidaemia was found in 115(63.2%) subjects; 61(67%) in group A and 54(59.3%) in group B. There was no statistical difference (p=0.28) when comparison was done after 3 months of therapy. Conclusions: The occurrence of new onset hyperlipidaemia is similar in renal transplant recipients receiving either cyclosporine or tacrolimus in first 3 months post-transplant, but there is room for more research in this field as dyslipidaemia following successful renal transplantation is a frequent and persistent complication. (author)

Huntington disease: a case study of early onset presenting as depression.

Science.gov (United States)

Duesterhus, Pia; Schimmelmann, Benno Graf; Wittkugel, Oliver; Schulte-Markwort, Michael

2004-10-01

Huntington disease is a dominantly inherited, neurodegenerative disease characterized by choreiform movement disturbances and dementia, usually with adult onset. The rare juvenile-onset Huntington disease differs from the adult phenotype. A case presenting twice, at age 10 with all the signs of a major depression and age 14 with mutism and rigidity, is reported. Meanwhile, the father developed the adult variant of Huntington disease. The boy's diagnosis was confirmed by molecular genetic analysis and magnetic resonance imaging. It is important to be aware of hereditary conditions such as Huntington disease and to provide family counseling before genetic testing and after the diagnosis is confirmed.

A Case of Juvenile Huntington Disease in a 6-Year-Old Boy

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Jun-Sang Sunwoo

2010-10-01

Full Text Available Huntington disease is a neurodegenerative disorder distinguished by the triad of dominant inheritance, choreoathetosis and dementia, usually with onset in the fourth and fifth decades. It is caused by an unstable cytosine-adenine-guanine (CAG trinucleotide repeat expansion in the gene IT15 in locus 4p16.3. Juvenile HD that constitutes about 3% to 10% of all patients is clinically different from adult-onset form and characterized by a larger number of CAG repeats typically exceeding 60. We report a case of a 6-year-old boy with myoclonic seizure and 140 CAG repeats confirmed by molecular genetic analysis.

Adult Onset Still's Disease and Rocky Mountain Spotted Fever

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Paul Persad

2010-01-01

Full Text Available Adult Still's Disease was first described in 1971 by Bywaters in fourteen adult female patients who presented with symptoms indistinguishable from that of classic childhood Still's Disease (Bywaters, 1971. George Still in 1896 first recognized this triad of quotidian (daily fevers, evanescent rash, and arthritis in children with what later became known as juvenile inflammatory arthritis (Still, 1990. Adult Onset Still's Disease (AOSD is an inflammatory condition of unknown etiology characterized by an evanescent rash, quotidian fevers, and arthralgias. Numerous infectious agents have been associated with its presentation. This case is to our knowledge the first presentation of AOSD in the setting of Rocky Mountain Spotted Fever. Although numerous infectious agents have been suggested, the etiology of this disorder remains elusive. Nevertheless, infection may in fact play a role in triggering the onset of symptoms in those with this disorder. Our case presentation is, to our knowledge, the first case of Adult Onset Still's Disease associated with Rocky Mountain spotted fever (RMSF.

Prodromal Symptoms and Atypical Affectivity as Predictors of Major Depression in Juveniles: Implications for Prevention

Science.gov (United States)

Kovacs, Maria; Lopez-Duran, Nestor

2010-01-01

Background: Given the long-term morbidity of juvenile-onset major depressive disorder (MDD), it is timely to consider whether more effort should be dedicated to its primary and secondary prevention. Methods: We reviewed studies of prodromal symptoms that may herald a first episode pediatric MDD and considered whether that literature has made an…

Juvenile Fibromyalgia: Different from the Adult Chronic Pain Syndrome?

Science.gov (United States)

Kashikar-Zuck, Susmita; King, Christopher; Ting, Tracy V; Arnold, Lesley M

2016-04-01

While a majority of research has focused on adult fibromyalgia (FM), recent evidence has provided insights into the presence and impact of FM in children and adolescents. Commonly referred as juvenile fibromyalgia (JFM), youths, particularly adolescent girls, present with persistent widespread pain and cardinal symptoms observed in adult FM. A majority of youth with JFM continue to experience symptoms into adulthood, which highlights the importance of early recognition and intervention. Some differences are observed between adult and juvenile-onset FM syndrome with regard to comorbidities (e.g., joint hypermobility is common in JFM). Psychological comorbidities are common but less severe in JFM. Compared to adult FM, approved pharmacological treatments for JFM are lacking, but non-pharmacologic approaches (e.g., cognitive-behavioral therapy and exercise) show promise. A number of conceptual issues still remain including (1) directly comparing similarities and differences in symptoms and (2) identifying shared and unique mechanisms underlying FM in adults and youths.

A novel splice-site mutation in ALS2 establishes the diagnosis of juvenile amyotrophic lateral sclerosis in a family with early onset anarthria and generalized dystonias.

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Saima Siddiqi

Full Text Available The diagnosis of childhood neurological disorders remains challenging given the overlapping clinical presentation across subgroups and heterogeneous presentation within subgroups. To determine the underlying genetic cause of a severe neurological disorder in a large consanguineous Pakistani family presenting with severe scoliosis, anarthria and progressive neuromuscular degeneration, we performed genome-wide homozygosity mapping accompanied by whole-exome sequencing in two affected first cousins and their unaffected parents to find the causative mutation. We identified a novel homozygous splice-site mutation (c.3512+1G>A in the ALS2 gene (NM_020919.3 encoding alsin that segregated with the disease in this family. Homozygous loss-of-function mutations in ALS2 are known to cause juvenile-onset amyotrophic lateral sclerosis (ALS, one of the many neurological conditions having overlapping symptoms with many neurological phenotypes. RT-PCR validation revealed that the mutation resulted in exon-skipping as well as the use of an alternative donor splice, both of which are predicted to cause loss-of-function of the resulting proteins. By examining 216 known neurological disease genes in our exome sequencing data, we also identified 9 other rare nonsynonymous mutations in these genes, some of which lie in highly conserved regions. Sequencing of a single proband might have led to mis-identification of some of these as the causative variant. Our findings established a firm diagnosis of juvenile ALS in this family, thus demonstrating the use of whole exome sequencing combined with linkage analysis in families as a powerful tool for establishing a quick and precise genetic diagnosis of complex neurological phenotypes.

Circulating C3 levels predict renal and global outcome in patients with renal vasculitis.

Science.gov (United States)

Villacorta, Javier; Diaz-Crespo, Francisco; Acevedo, Mercedes; Cavero, Teresa; Guerrero, Carmen; Praga, Manuel; Fernandez-Juarez, Gema

2016-11-01

Several studies have demonstrated the crucial role of complement activation in the pathogenesis of ANCA-associated vasculitis. We aimed to assess the association between baseline serum C3 (sC3) levels and long-term outcomes in patients with renal vasculitis. This retrospective study included 111 patients with renal vasculitis from three hospitals who underwent a renal biopsy between 1997 and 2014. Serum levels of C3 were measured at the onset and the study population was divided into three tertiles according to sC3 concentrations (tertile 1 128 mg/dl). Patients with lower sC3 (tertile 1) were compared with those having higher levels of sC3 (tertile 2 and tertile 3). Histological, clinical, and laboratory data were recorded for analysis. The primary end point was the composite of end-stage renal disease (ESRD) and death from any cause. Lower sC3 levels were associated with a higher need for dialysis and lower response rate to treatment (p = 0.04 and p = 0.007, respectively). Renal and global survival at 1 and 5 years was 53 and 46 % in patients with lower sC3 (tertile 1) compared with 72 and 65 % in patients with higher sC3 (upper two tertiles) (p = 0.04). In a multivariate Cox-regression model, when adjusted by renal function and histopatholologic categories, lower sC3 remained as an independent predictor of ESRD and death (HR, 1.9; 95 % CI, 1.1 to 3.4; p = 0.02). Baseline serum C3 levels have an independent prognostic value in predicting long-term renal and global survival in patients with renal vasculitis.

A Novel Treatment of Aggressive Psammomatoid Juvenile Ossifying Fibroma: A Case Report

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Badri

2014-08-01

Full Text Available Introduction Juvenile ossifying fibroma is a benign, bone-forming neoplasm with aggressive local growth that is distinguished from other fibro-osseous lesions primarily by its age of onset, clinical presentation and aggressive behavior. Although a benign entity, juvenile ossifying fibroma is known to be locally aggressive and has a high tendency to recur. Two distinctive microscopic patterns have been described: a trabecular variant and a psammomatoid variant. This latter variant is predominantly a craniofacial lesion and occurs rarely in the jaws. Treatment plans include a range of enucleation to total resection. Case Presentation Reported here is a case of a huge psammomatoid juvenile ossifying fibroma of the maxilla in a 19-year-old man. Right maxillectomy was done while brain exposure in sphenoid and ethmoidal region was covered with fat and bone graft. After 1 year follow up, an endosseous dental implant was inserted in right lateral pterygoid plate. Conclusions Six months later an obturator was made over that osseointegrated implant to reconstruct the anatomic defect, achieving functional rehabilitation and satisfactory facial appearance.

Obstructive uropathy and acute renal failure due to ureteral calculus in renal graft: a case report.

Science.gov (United States)

Lusenti, T; Fiorini, F; Barozzi, L

2009-09-01

Obstructive uropathy caused by kidney stones is quite rare in transplant kidneys. The authors report the case of a patient, previously gastrectomized for gastric carcinoma. He underwent renal transplantation using uretero-ureterostomy, and presented an episode of acute renal failure 7 years after surgery. Ultrasound (US) examination showed no sign of rejection but allowed detection of moderate hydronephrosis in the transplant kidney. Subsequent computed tomography (CT) revealed a kidney stone in the middle ureter at the crossing of the iliac vessels. The patient therefore urgently underwent percutaneous nephrostomy of the graft and recovered diuresis and renal function. The patient was transferred to the Transplant Center where he underwent ureterotomy with removal of the stone and subsequent ureteropyelostomy. Also transureteral resection of the prostate (TURP) was performed due to urinary retention of prostatic origin. Histological examination showed prostate carcinoma, Gleason stage 3, which was treated conservatively using radiotherapy without suspension of the administered low dose of immunotherapy. Calculosis is one of the least common causes of obstructive uropathy in transplant kidneys. In the described case, US examination performed after onset of renal insufficiency led to subsequent radiological investigation and resulting interventional procedures (nephrostomy and surgical removal of the stone) with complete recovery of pre-existing renal function.

Hypothyroidism in late-onset Pompe disease.

Science.gov (United States)

Schneider, Joseph; Burmeister, Lynn A; Rudser, Kyle; Whitley, Chester B; Jarnes Utz, Jeanine

2016-09-01

In Pompe disease, a deficiency of acid α-glucosidase enzyme activity leads to pathologic accumulation of glycogen in tissues. Phenotype heterogeneity in Pompe includes an infantile form and late-onset forms (juvenile- and adult-onset forms). Symptoms common to all phenotypes include progressive muscle weakness and worsening respiratory function. Patients with late-onset forms of Pompe disease commonly complain of chronic fatigue and generalized muscle weakness prior to being diagnosed with Pompe disease, and this may lead to consideration of hypothyroidism in the differential diagnosis. This study aimed to evaluate the prevalence of hypothyroidism in the adult-onset form of Pompe disease. Electronic chart review was performed at the Advanced Therapies Clinic at the University of Minnesota Medical Center (UMMC) to identify patients with late-onset Pompe disease. The identified charts were reviewed for a co-diagnosis of hypothyroidism. A query was made to the clinical data repository at UMMC searching diagnosis ICD9 code 244.9 (hypothyroidism not otherwise specified) and/or presence of levothyroxine from 2011 to 2014 in patients 18 years of age and older. The clinical data repository found a prevalence of hypothyroidism of 3.15% (56,072 of 1,782,720 patients) in the adult patient population at UMMC. Ten adult patients with Pompe disease were identified, five with the diagnosis of hypothyroidism (50%, 95% CI: 23.7, 76.3, p Hypothyroidism was found at a higher prevalence in patients with late-onset Pompe disease compared to the general adult population at UMMC. Studies in larger populations of patients with Pompe disease would be needed to confirm an association of Pompe disease and hypothyroidism. Challenges include finding an adequate sample size, due the rarity of Pompe disease.

Juvenile rheumatoid arthritis

Science.gov (United States)

... joints. This form of JIA may turn into rheumatoid arthritis. It may involve 5 or more large and ... no known prevention for JIA. Alternative Names Juvenile rheumatoid arthritis (JRA); Juvenile chronic polyarthritis; Still disease; Juvenile spondyloarthritis ...

Enhanced Apoptosis of Monocytes from Complication-Free Juvenile-Onset Diabetes Mellitus Type 1 May Be Ameliorated by TNF-α Inhibitors

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Jolanta Myśliwska

2014-01-01

Full Text Available Diabetes mellitus type 1 is associated with an enhanced apoptosis of different cells and tissues, accelerating occurrence of diabetic microvascular complications. The aim of our study was to determine spontaneous apoptotic potential of the monocyte subsets in juvenile-onset complication-free diabetes mellitus type 1 and to compare them with the corresponding values of the healthy. Moreover, we wanted to assess effects of TNF-R1 blocking agents and those of general TNF-α blocker (Infliximab on spontaneous apoptosis of monocytes. Sixty randomly selected DM1 patients (14.5 ± 3.2 years and 30 healthy (13.5 ± 2.8 years volunteers were enrolled in the study. Our results indicate that three monocyte subsets are distinguishable in the groups of young diabetic patients and the healthy, similarly to in the blood of adults. DM1 patients were characterized by higher values of apoptotic monocytes than the healthy. The manipulation with drugs inhibiting TNF-R1 expression diminished the pool of CD16+ apoptotic monocytes. Infliximab reduced the apoptotic CD16− cells. In conclusion, diabetes mellitus type 1 is associated with greater apoptosis of three monocyte subsets which may contribute to the development of microvascular complications. TNF-α modifiers appear to ameliorate monocyte apoptosis. They may be useful for controlling excessive monocyte apoptosis in diabetic patients.

Opposite effect of phencyclidine on activity-regulated cytoskeleton-associated protein (Arc) in juvenile and adult limbic rat brain regions

DEFF Research Database (Denmark)

Thomsen, Morten S; Hansen, Henrik H; Mikkelsen, Jens D

2010-01-01

The psychotomimetic effect of NMDA antagonists such as phencyclidine (PCP) in humans spurred the hypoglutamatergic theory of schizophrenia. This theory is supported by animal studies demonstrating schizophrenia-like behavioral and molecular changes following PCP administration to adult or neonatal...... animals. However, schizophrenia is believed to develop in part due to neurodevelopmental dysfunction during adolescence. Therefore, the effects of PCP in juvenile animals may better reflect the pathophysiology of schizophrenia. Here, we compare the effect of PCP (5mg/kg/day for 5 days) on activity......RNA in juvenile rats corresponds best with the proposed "hypofrontality" in schizophrenia, suggesting the merits of using PCP in juvenile animals as a model for schizophrenia, as this would relate better to the typical onset and clinical features of schizophrenia....

Visual Disability Among Juvenile Open-angle Glaucoma Patients.

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Gupta, Viney; Ganesan, Vaitheeswaran L; Kumar, Sandip; Chaurasia, Abadh K; Malhotra, Sumit; Gupta, Shikha

2018-04-01

Juvenile onset primary open-angle glaucoma (JOAG) unlike adult onset primary open-angle glaucoma presents with high intraocular pressure and diffuse visual field loss, which if left untreated leads to severe visual disability. The study aimed to evaluate the extent of visual disability among JOAG patients presenting to a tertiary eye care facility. Visual acuity and perimetry records of unrelated JOAG patients presenting to our Glaucoma facility were analyzed. Low vision and blindness was categorized by the WHO criteria and percentage impairment was calculated as per the guidelines provided by the American Medical Association (AMA). Fifty-two (15%) of the 348 JOAG patients were bilaterally blind at presentation and 32 (9%) had low vision according to WHO criteria. Ninety JOAG patients (26%) had a visual impairment of 75% or more. Visual disability at presentation among JOAG patients is high. This entails a huge economic burden, given their young age and associated social responsibilities.

Renal Function Recovery with Total Artificial Heart Support.

Science.gov (United States)

Quader, Mohammed A; Goodreau, Adam M; Shah, Keyur B; Katlaps, Gundars; Cooke, Richard; Smallfield, Melissa C; Tchoukina, Inna F; Wolfe, Luke G; Kasirajan, Vigneshwar

2016-01-01

Heart failure patients requiring total artificial heart (TAH) support often have concomitant renal insufficiency (RI). We sought to quantify renal function recovery in patients supported with TAH at our institution. Renal function data at 30, 90, and 180 days after TAH implantation were analyzed for patients with RI, defined as hemodialysis supported or an estimated glomerular filtration rate (eGFR) less than 60 ml/min/1.73 m. Between January 2008 and December 2013, 20 of the 46 (43.5%) TAH recipients (age 51 ± 9 years, 85% men) had RI, mean preoperative eGFR of 48 ± 7 ml/min/1.73 m. Renal function recovery was noted at each follow-up interval: increment in eGFR (ml/min/1.73 m) at 30, 90, and 180 days was 21 ± 35 (p = 0.1), 16.5 ± 18 (p = 0.05), and 10 ± 9 (p = 0.1), respectively. Six patients (30%) required preoperative dialysis. Of these, four recovered renal function, one remained on dialysis, and one died. Six patients (30%) required new-onset dialysis. Of these, three recovered renal function and three died. Overall, 75% (15 of 20) of patients' renal function improved with TAH support. Total artificial heart support improved renal function in 75% of patients with pre-existing significant RI, including those who required preoperative dialysis.

Guidelines for imaging infants and children with recent-onset epilepsy

International Nuclear Information System (INIS)

Gaillard, W.D.; Chiron, C.; Cross, H.; Harvey, S.; Kuzniecky, R.; Hertz-Pannier, L.

2009-01-01

The International League Against Epilepsy (ILAE) Subcommittee for Pediatric Neuroimaging examined the usefulness of, and indications for, neuroimaging in the evaluation of children with newly diagnosed epilepsy. The retrospective and prospective published series with n ≥ 30 utilizing computed tomography (CT) and magnetic resonance imaging (MRI) (1.5 T) that evaluated children with new-onset seizure(s) were reviewed. Nearly 50% of individual imaging studies in children with localization-related new-onset seizure(s) were reported to be abnormal; 15-20% of imaging studies provided useful information on etiology or and seizure focus, and 2-4% provided information that potentially altered immediate medical management. A significant imaging abnormality in the absence of a history of a localization-related seizure, abnormal neurologic examination, or focal electro-encephalography (EEG) is rare. Imaging studies in childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy, and benign childhood epilepsy with centro-temporal spikes (BECTS) do not identify significant structural abnormalities. Imaging provides important contributions to establishing etiology, providing prognostic information, and directing treatment in children with recently diagnosed epilepsy. Imaging is recommended when localization-related epilepsy is known or suspected, when the epilepsy classification is in doubt, or when an epilepsy syndrome with remote symptomatic cause is suspected. When available, MRI is preferred to CT because of its superior resolution, versatility, and lack of radiation. (authors)

Guidelines for imaging infants and children with recent-onset epilepsy

Energy Technology Data Exchange (ETDEWEB)

Gaillard, W.D. [Department of Neuroscience, Children' s National Medical Center, George Washington University, Washington DC (United States); Chiron, C. [Inserm, Hopital Necker-Enfants Malades, Universite Rene Descartes, Paris (France); Cross, H. [Neurosciences Unit, Institute of Child Health, and GreatOrmondStreet Hospital for Children, London (United Kingdom); Harvey, S. [Department of Neurology, Royal Children' s Hospital, University of Melbourne, Melbourne (Australia); Kuzniecky, R. [Department of Neurology, New York University School of Medicine, New York, NY (US); Hertz-Pannier, L. [Department of Radiology, Hopital Necker-Enfants Malades, Universite Descartes, Paris (FR); CEA-DSV-I2BM-Neurospin, 91191 Gif sur Yvette (FR)

2009-07-01

The International League Against Epilepsy (ILAE) Subcommittee for Pediatric Neuroimaging examined the usefulness of, and indications for, neuroimaging in the evaluation of children with newly diagnosed epilepsy. The retrospective and prospective published series with n {>=} 30 utilizing computed tomography (CT) and magnetic resonance imaging (MRI) (1.5 T) that evaluated children with new-onset seizure(s) were reviewed. Nearly 50% of individual imaging studies in children with localization-related new-onset seizure(s) were reported to be abnormal; 15-20% of imaging studies provided useful information on etiology or and seizure focus, and 2-4% provided information that potentially altered immediate medical management. A significant imaging abnormality in the absence of a history of a localization-related seizure, abnormal neurologic examination, or focal electro-encephalography (EEG) is rare. Imaging studies in childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy, and benign childhood epilepsy with centro-temporal spikes (BECTS) do not identify significant structural abnormalities. Imaging provides important contributions to establishing etiology, providing prognostic information, and directing treatment in children with recently diagnosed epilepsy. Imaging is recommended when localization-related epilepsy is known or suspected, when the epilepsy classification is in doubt, or when an epilepsy syndrome with remote symptomatic cause is suspected. When available, MRI is preferred to CT because of its superior resolution, versatility, and lack of radiation. (authors)

Osteonecrosis or spontaneous fractures following renal transplantation

International Nuclear Information System (INIS)

Andresen, J.; Nielsen, H.E.; Aarhus Univ.

1981-01-01

31 renal transplant recipients with posttransplant development of osteonecrosis or spontaneous fractures were evaluated with regard to age, duration of dialysis before transplantation. Determination of metacarpal bone mass at the time of transplantation and registration of bone resorption and soft tissue calcification at the time of transplantation and at the time of onset of osteonecrosis and spontaneous fractures were made. Apart from the increased mean age in patients with spontaneous fractures no difference was seen between the groups. Osteonecrosis and spontaneous fractures occurred in areas of trabecular bone. It seems most likely that after renal transplantation the patients show bone complications of different localization. (orig.) [de

An aid to the diagnosis of genetic disorders underlying adult-onset renal failure : a literature review

NARCIS (Netherlands)

Joosten, H.; Strunk, A. L. M.; Meijer, S.; Boers, J. E.; Aries, M.J.H.; Abbes, A. P.; Engel, H.; Beukhof, J. R.

Several genetic disorders can present in adult patients with renal insufficiency. Genetic renal disease other than ADPKD accounts for ESRD in 3% of the adult Dutch population. Because of this low prevalence and their clinical heterogeneity most adult nephrologists are less familiar with these

Adalimumab (TNFα Inhibitor Therapy Exacerbates IgA Glomerulonephritis Acute Renal Injury and Induces Lupus Autoantibodies in a Psoriasis Patient

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S. S. Wei

2013-01-01

Full Text Available Adalimumab (Humira is a tumour necrosis factor α (TNFα inhibitor that is approved for the treatment of rheumatoid arthritis, psoriasis, psoriatic arthritis, Crohn's disease, ankylosing spondylitis, and juvenile idiopathic arthritis (Sullivan and Preda (2009, Klinkhoff (2004, and Medicare Australia. Use of TNFα inhibitors is associated with the induction of autoimmunity (systemic lupus erythematosus, vasculitis, and sarcoidosis or sarcoid-like granulomas (Ramos-Casals et al. (2010. We report a patient with extensive psoriasis presenting with renal failure and seropositive lupus markers without classical lupus nephritis after 18 months treatment with adalimumab. He has renal biopsy proven IgA nephritis instead. Renal biopsy is the key diagnostic tool in patients presenting with adalimumab induced nephritis and renal failure. He made a remarkable recovery after adalimumab cessation and steroid treatment. To our knowledge, this is a unique case of a psoriasis patient presenting with seropositive lupus markers without classical lupus nephritis renal failure and had renal biopsy proven IgA glomerulonephritis after receiving adalimumab.

Conceptualizing juvenile prostitution as child maltreatment: findings from the National Juvenile Prostitution Study.

Science.gov (United States)

Mitchell, Kimberly J; Finkelhor, David; Wolak, Janis

2010-02-01

Two studies were conducted to identify the incidence (Study 1) and characteristics (Study 2) of juvenile prostitution cases known to law enforcement agencies in the United States. Study 1 revealed a national estimate of 1,450 arrests or detentions (95% confidence interval [CI]: 1,287-1,614) in cases involving juvenile prostitution during a 1-year period. In Study 2, exploratory data were collected from a subsample of 138 cases from police records in 2005. The cases are broadly categorized into three main types: (a) third-party exploiters, (b) solo prostitution, and (c) conventional child sexual abuse (CSA) with payment. Cases were classified into three initial categories based on police orientation toward the juvenile: (a) juveniles as victims (53%), (b) juveniles as delinquents (31%), and (c) juvenile as both victims and delinquents (16%). When examining the status of the juveniles by case type, the authors found that all the juveniles in CSA with payment cases were treated as victims, 66% in third-party exploiters cases, and 11% in solo cases. Findings indicate law enforcement responses to juvenile prostitution are influential in determining whether such youth are viewed as victims of commercial sexual exploitation or as delinquents.

Adaptive changes in renal mitochondrial redox status in diabetic nephropathy

Energy Technology Data Exchange (ETDEWEB)

Putt, David A.; Zhong, Qing; Lash, Lawrence H., E-mail: l.h.lash@wayne.edu

2012-01-15

Nephropathy is a serious and common complication of diabetes. In the streptozotocin (STZ)-treated rat model of diabetes, nephropathy does not typically develop until 30 to 45 days post-injection, although hyperglycemia occurs within 24 h. We tested the hypothesis that chronic hyperglycemia results in a modest degree of oxidative stress that is accompanied by compensatory changes in certain antioxidants and mitochondrial redox status. We propose that as kidneys progress to a state of diabetic nephropathy, further adaptations occur in mitochondrial redox status. Basic parameters of renal function in vivo and several parameters of mitochondrial function and glutathione (GSH) and redox status in isolated renal cortical mitochondria from STZ-treated and age-matched control rats were examined at 30 days and 90 days post-injection. While there was no effect of diabetes on blood urea nitrogen, measurement of other, more sensitive parameters, such as urinary albumin and protein, and histopathology showed significant and progressive worsening in diabetic rats. Thus, renal function is compromised even prior to the onset of frank nephropathy. Changes in mitochondrial respiration and enzyme activities indicated existence of a hypermetabolic state. Higher mitochondrial GSH content and rates of GSH transport into mitochondria in kidneys from diabetic rats were only partially due to changes in expression of mitochondrial GSH carriers and were mostly due to higher substrate supply. Although there are few clear indicators of oxidative stress, there are several redox changes that occur early and change further as nephropathy progresses, highlighting the complexity of the disease. Highlights: ►Adaptive changes in renal mitochondrial and redox status in diabetic rats. ►Modest renal dysfunction even prior to onset of nephropathy. ►Elevated concentrations of mitochondrial GSH in diabetic kidneys. ►Change in GSH due partly to increased protein expression of transporter. �

Adaptive changes in renal mitochondrial redox status in diabetic nephropathy

International Nuclear Information System (INIS)

Putt, David A.; Zhong, Qing; Lash, Lawrence H.

2012-01-01

Nephropathy is a serious and common complication of diabetes. In the streptozotocin (STZ)-treated rat model of diabetes, nephropathy does not typically develop until 30 to 45 days post-injection, although hyperglycemia occurs within 24 h. We tested the hypothesis that chronic hyperglycemia results in a modest degree of oxidative stress that is accompanied by compensatory changes in certain antioxidants and mitochondrial redox status. We propose that as kidneys progress to a state of diabetic nephropathy, further adaptations occur in mitochondrial redox status. Basic parameters of renal function in vivo and several parameters of mitochondrial function and glutathione (GSH) and redox status in isolated renal cortical mitochondria from STZ-treated and age-matched control rats were examined at 30 days and 90 days post-injection. While there was no effect of diabetes on blood urea nitrogen, measurement of other, more sensitive parameters, such as urinary albumin and protein, and histopathology showed significant and progressive worsening in diabetic rats. Thus, renal function is compromised even prior to the onset of frank nephropathy. Changes in mitochondrial respiration and enzyme activities indicated existence of a hypermetabolic state. Higher mitochondrial GSH content and rates of GSH transport into mitochondria in kidneys from diabetic rats were only partially due to changes in expression of mitochondrial GSH carriers and were mostly due to higher substrate supply. Although there are few clear indicators of oxidative stress, there are several redox changes that occur early and change further as nephropathy progresses, highlighting the complexity of the disease. Highlights: ►Adaptive changes in renal mitochondrial and redox status in diabetic rats. ►Modest renal dysfunction even prior to onset of nephropathy. ►Elevated concentrations of mitochondrial GSH in diabetic kidneys. ►Change in GSH due partly to increased protein expression of transporter. �

Longitudinal change in renal function after nephroureterectomy in patients with upper tract urothelial carcinoma

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Chih-Yuan Chou

2015-06-01

Conclusion: In this study, it was found that the average renal function of the patients with upper tract urothelial carcinoma is not as good as the general population. More than half of the normal renal function patients have new onset chronic kidney disease after surgery. For preventing further deterioration of renal function, the implication of partial nephrectomy or segmental ureterectomy for selected patients with localized urothelial carcinoma should be re-examined. Besides, neoadjuvant chemotherapy should be considered for those who are not good candidates for local treatment.

Anti-chromatin antibodies in juvenile rheumatoid arthritis

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V. Gerloni

2011-09-01

Full Text Available Objective: to evaluate the prevalence and clinical significance of anti-chromatin antibodies (Abs in juvenile rheumatoid arthritis (JRA. Methods: IgG anti-chromatin Abs were detected by an enzyme-linked immunosorbent assay (ELISA, in sera of 94 children with JRA (10 children with systemic, 38 with polyarticular and 46 with oligoarticular disease onset. As control group, 33 age- and-sex-matched healthy children (HC were also examined. Results: Abs to chromatin were detected in 24/94 (25,5% of children suffering from JRA. Particularly, the higher prevalence of anti-chromatin Abs has been found in children with oligoarticular (30,4% and polyarticular (23,7% onset JRA. In these groups Abs titers were significantly higher compared to systemic JRA and HC (p=0.003. Anti-chromatin Abs were observed more frequently in patients with oligoarticular disease and chronic uveitis (21,7%. Furthermore, higher levels of anti-chromatin Abs has been found in all the patients treated with anti-TNFα therapy (p<0.0001. Conclusions: our results confirm previous data about the prevalence of anti-chromatin Abs in JRA. These Abs were significantly higher in the group of patients with oligoarticular onset with past or present hystory of ocular involvement and in the group with polyarticular JRA treated with biologic therapy. A long-term follow-up study could be useful to evaluate the potential utility of these autoantibodies.

Age of onset as a risk factor of renal involvement in Henoch-Schönlein purpura

Science.gov (United States)

Ledika, Masayu Amanda; Sapartini, Gartika; Setiabudiawan, Budi

2014-01-01

Background Henoch-Schönlein purpura (HSP) is the most common vasculitis in children, characterized by triad of symptoms; palpable purpura without thrombocytopenia, abdominal pain, and arthritis. Renal involvement often occur in children with HSP. No data on the renal involvement of children with HSP in Indonesia, especially West Java. Objective To evaluate renal involvement in children with HSP. Methods Retrospective study was conducted in children with HSP in Department of Child Health, Hasan Sadikin Hospital, from 2006 to 2011. Characteristics and clinical manifestations was reviewed from medical record. HSP was diagnosed by American College of Rheumatology 1990 criteria or European League Against Rheumatism/Pediatric Rheumatology International Trials Organization/Pediatric Rheumatology European Society 2008. Results There were 128 patients, consisting of 82 male (64.9%) and 46 female (35.1%) with ratio 1.8:1. Mean age was 7.9 ± 2.9 years old which range from 6 month to 15 years. Peak morbidity was between 5-10 years old. Prevalence of HSP in Hasan Sadikin Hospital tend to raise from 2.7/100,000 in 2008 to 5.2/100,000 in 2010. In most patients (71%) purpura was the first symptom. Seventy-one patients (44.5%) had arthritis and 89 patients (69.5%) had abdominal pain, while renal involvement was in 28 patients (21.8%). Gastrointestinal manifestations tend to manifest in patients less than 5 years old (p = 0.267), while renal involvement tend to manifest in age group 11-15 years old (p = 0.015) with odds ratio 3.1 (95% confidence interval, 1.2-8.1). Conclusion Renal involvement in children with HSP is more common in age group 11 to 15 years old. PMID:24527410

Juvenile Arthritis

Science.gov (United States)

Juvenile arthritis (JA) is arthritis that happens in children. It causes joint swelling, pain, stiffness, and loss of motion. It can affect any joint, but ... of JA that children get is juvenile idiopathic arthritis. There are several other forms of arthritis affecting ...

Juvenile rheumatoid arthritis

International Nuclear Information System (INIS)

Naz, S.; Mushtaq, A.; Bari, A.; Maqsud, A.; Khan, M. Z.; Ahmad, T. M.; Saira Rehman

2013-01-01

Objective: To determine the spectrum of clinical presentation, laboratory parameters and drug therapy in patients with Juvenile Rheumatoid Arthritis (JRA). Study Design: Case series. Place and Duration of Study: The Children's Hospital and The Institute of Child Health, Lahore, from October 2008 to October 2011. Methodology: All patients who fulfilled the American College of Rheumatology criteria for JRA were enrolled. Their clinical features, investigations done and treatment received for JRA were noted. Statistical analysis of data was done on SPSS version 16.0 for obtaining descriptive statistics. Results: Out of 185 patients, 50.3% (n = 93) were females; 54% (n = 100) were between 10 - 15 years of age. Polyarthritis was found in 71.9% (n = 133) followed by oligoarthritis (22.7%, n = 42) and systemic onset disease (5.4%, n = 10). Morning stiffness (78%) and fever (68%) were the most common clinical presentations. All patients with systemic onset disease had fever (n = 10) followed by skin rash, hepatosplenomegaly and lymphadenopathy. Uveitis was found in 2 patients, and both belonged to the oligoarticular group. Rheumatoid factor was found in 10.27% (n = 19) of all patients. All patients were given non-steroidal anti-inflammatory drugs (NSAIDs). Disease modifying agents (methotrexate) were given to 43.8% (n = 81). Steroids were used in 61% (n = 113) of patients either with NSAIDs alone or NSAIDs plus methotrexate. Conclusion: Disease profile of JRA at the study centre showed that polyarthritis is the commonest type. Recognition of subtypes will help in planning the management of these patients. (author)

What Is Juvenile Arthritis?

Science.gov (United States)

... Initiative Breadcrumb Home Health Topics English Español Juvenile Arthritis Basics In-Depth Download Download EPUB Download PDF What is it? Points To Remember About Juvenile Arthritis Juvenile arthritis is the term used to describe ...

Epidemic juvenile hypothyroidism among a population of Hanford 'downwinders'

International Nuclear Information System (INIS)

Grossman, C.M.; Nussbaum, R.H.; Nussbaum, F.D.; Kordysh, E.; Goldsmith, J.R.

2001-01-01

Self-defined 'downwinders' responded to questionnaires as to their health and their potential exposures to emissions from the Hanford Nuclear Facility in the U.S. The area considered was between the Cascades and the Rocky Mountains, primarily to the East and Northeast of the facility. Of 801 questionnaires, returned, 518 were from women. Of these, 210 reported that they had been diagnosed by their physicians as having hypothyroidism. 31 of the 283 men had reported being diagnosed as hypothyroid. The condition is generally found to be frequent among adult women, but is uncommon in children. 54 of the 210 cases among females were diagnosed among those less than 20 years of age, and 5 out of 31 males were found in this age range. Peak years for diagnosis among females were from 1945 to 1965, with only three juvenile cases occurring after 1970 among 80 female hypothyroid cases overall. Among males there were five juvenile cases in 1955-1960 out of 7 overall, but no juvenile cases among 24 cases at other times. The timing of the onset of this epidemic follows closely after the massive release of I 131 from the facility in 1945, so that a causal relationship is strongly suggested. The counties of residence of cases corresponds to the counties with high cumulative dose estimates in children's thyroid glands. Validation of the diagnostic criteria was checked in a sample of cases. Other types of thyroid abnormalities were also increased. One case of juvenile thyroid cancer was reported. While thyroid cancer in adults was unusually frequent, the first cases occurred in 1955 and the highest number (8) in 1985. Our findings are consistent with the clinical finding that hypothyroidism in children frequently follows radiation of the head and neck. Similarities with the Marshall Islanders exposed to the nuclear test, 'Bravo' and to those exposed near Chernobyl are discussed. (orig.)

Update on the Treatment of Uveitis in Patients with Juvenile Idiopathic Arthritis: A Review.

Science.gov (United States)

Asproudis, Ioannis; Katsanos, Andreas; Kozeis, Nikolaos; Tantou, Alexandra; Konstas, Anastasios G

2017-12-01

Chronic uveitis is a common extra-articular manifestation of juvenile idiopathic arthritis. The classic clinical picture is one of chronic anterior uveitis, which usually remains asymptomatic until ocular complications arise. The risk of uveitis is increased in girls with an early onset of oligoarthritis and positive antinuclear antibodies. Even though the inflammation in patients with juvenile idiopathic arthritis is initially limited in the anterior part of the eye, chronic active inflammation may eventually cause significant damage to the posterior pole. Complications may include band keratopathy, cataract, secondary glaucoma, posterior synechiae, cystoid macular edema, and hypotony. The cooperation of ophthalmologists with rheumatologists may help define the best treatment plan. The ophthalmic therapeutic regimen includes topical corticosteroids and mydriatics, while in severe cases immunosuppressive and biological agents are introduced. Surgical management of complications might be needed.

Wheel running from a juvenile age delays onset of specific motor deficits but does not alter protein aggregate density in a mouse model of Huntington's disease

Directory of Open Access Journals (Sweden)

Spires Tara L

2008-04-01

Full Text Available Abstract Background Huntington's disease (HD is a neurodegenerative disorder predominantly affecting the cerebral cortex and striatum. Transgenic mice (R6/1 line, expressing a CAG repeat encoding an expanded polyglutamine tract in the N-terminus of the huntingtin protein, closely model HD. We have previously shown that environmental enrichment of these HD mice delays the onset of motor deficits. Furthermore, wheel running initiated in adulthood ameliorates the rear-paw clasping motor sign, but not an accelerating rotarod deficit. Results We have now examined the effects of enhanced physical activity via wheel running, commenced at a juvenile age (4 weeks, with respect to the onset of various behavioral deficits and their neuropathological correlates in R6/1 HD mice. HD mice housed post-weaning with running wheels only, to enhance voluntary physical exercise, have delayed onset of a motor co-ordination deficit on the static horizontal rod, as well as rear-paw clasping, although the accelerating rotarod deficit remains unaffected. Both wheel running and environmental enrichment rescued HD-induced abnormal habituation of locomotor activity and exploratory behavior in the open field. We have found that neither environment enrichment nor wheel running ameliorates the shrinkage of the striatum and anterior cingulate cortex (ACC in HD mice, nor the overall decrease in brain weight, measured at 9 months of age. At this age, the density of ubiquitinated protein aggregates in the striatum and ACC is also not significantly ameliorated by environmental enrichment or wheel running. Conclusion These results indicate that enhanced voluntary physical activity, commenced at an early presymptomatic stage, contributes to the positive effects of environmental enrichment. However, sensory and cognitive stimulation, as well as motor stimulation not associated with running, may constitute major components of the therapeutic benefits associated with enrichment

Radiology of skeletal and soft tissue changes

International Nuclear Information System (INIS)

Walker, H.C. Jr.; Coleman, C.C.; Hunter, D.W.

1986-01-01

Skeletal complications are very common in renal transplant patients. Loss of bone mass in the posttransplant period places the skeletal system in jeopardy. Osteonecrosis, while not life threatening, often prevents rehabilitation. Spontaneous fractures are frequent but are usually not a major problem except in the diabetic transplant recipient. Septic arthritis and osteomyelitis are usually successfully managed by conservative measures, except when accompanied by severe occlusive vascular disease. Juvenile onset diabetic patients still may develop disabling neuropathic joint disease or occlusive vascular disease after renal transplantation. The authors hope that successful pancreas transplantation will avert these problems in the future

RENAL INVOLVEMENT IN SUBJECTS WITH PERIPHERAL ATHEROSCLEROSIS

International Nuclear Information System (INIS)

FAWZY, A.; IBRAHIM, S.

2008-01-01

Ischemic nephropathy is an important cause of renal failure.Sub-clinical renal function abnormalities may exist in patients with extra renal atherosclerosis and may precede the onset of overt ischemic nephropathy. To assess the impact of extrarenal atherosclerosis on the kidney, the study evaluated renal function in 50 subjects with differing degrees of peripheral atherosclerosis without manifest clinical or laboratory signs of ischemic nephropathy and renovascular hypertension.All laboratory testing including total LDL and HDL-cholesterol, triglycerides, ultrasonography with Doppler analysis for the localization of peripheral vascular disease (carotid and lower limb arteries), and non-invasive evaluation of renal function by radionuclide studies of renal plasma flow (MAG3 clearance) and glomerular filtration (DTPA clearance) were determined as well as smoking habit was recorded. By combining sonographic data on arterial tree stenosis (ATS), the subjects were grouped according to the atherosclerotic vascular damage (ATS involvement). The results showed no change in plasma creatinine while DTPA clearance was increased from 91.58±26.53 to 93.47±24.82 ml/min/1.73 m. MAG3 clearance was progressively declined with the severity of vascular damage from 244.86 ± 60.60 to 173.59±58.74 ml/min/1.73 m.Stepwise, multiple regression analysis indicated that MAG3 clearance was best explained by ATS involvement (standardized B coefficient -0.40; P< 0.001), smoking habit (-0.34;P=0.004) and serum LDL-cholesterol (-0.24; P<0.035).It could be concluded that the renal hemodynamic profile in atherosclerotic patients might constitute functional evidence of the silent phase of ischemic renal disease. The findings suggest that renal function should be carefully assessed in patients with extrarenal atherosclerosis, particularly in those with classic cardiovascular risk factors

Juvenile Firesetting.

Science.gov (United States)

Peters, Brittany; Freeman, Bradley

2016-01-01

Juvenile firesetting is a significant cause of morbidity and mortality in the United States. Male gender, substance use, history of maltreatment, interest in fire, and psychiatric illness are commonly reported risk factors. Interventions that have been shown to be effective in juveniles who set fires include cognitive behavior therapy and educational interventions, whereas satiation has not been shown to be an effective intervention. Forensic assessments can assist the legal community in adjudicating youth with effective interventions. Future studies should focus on consistent assessment and outcome measures to create more evidence for directing evaluation and treatment of juvenile firesetters. Copyright © 2016 Elsevier Inc. All rights reserved.

Growth speed in patients with chronic renal failure undergoing to renal transplantation between 2000 and 2009 in the Hospital Nacional de Ninos: research protocol; Velocidad de crecimiento en pacientes con insuficiencia renal cronica sometidos a trasplante renal entre el ano 2000 y el 2009 en el Hospital Nacional de Ninos: protocolo de investigacion

Energy Technology Data Exchange (ETDEWEB)

Arroyo Molina, Ana Victoria

2013-07-01

The growth speed was investigated in children with chronic renal failure after renal transplantation, in the Hospital Nacional de Ninos during the study period January 2000-December 2009. Factors that have influenced are analyzed: age of onset of renal disease, etiology of renal disease, metabolic acidosis, anemia, renal osteodystrophy, episodes of infection and rejection. Besides, on the growth rate and expected family size, to intervene or prevent them in future cases. Also, the use that has given in the hospital to growth hormone, before and after renal transplantation is determined to eventually use parallel therapies to the transplantation. An echocardiographic study is recommended to perform as part of the treatment of chronic renal failure to identify the existence of left ventricular hypertrophy and heart failure, which may occur as a result of complications of the failure [Spanish] La velocidad del crecimiento fue investigada en ninos con insuficiencia renal cronica despues del transplante renal, en el Hospital Nacional de Ninos durante el periodo de estudio enero 2000-diciembre 2009. Factores que han influido son analizados: edad de inicio de la enfermedad renal, etiologia de la enfermedad renal, la acidosis metabolica, la anemia, la osteodistrofia renal, los episodios de infecciones y rechazos. Ademas, sobre la velocidad de crecimiento y la talla familiar esperada, para intervenir en ellos o prevenirlos en casos futuros. Tambien, el uso que se ha dado en el hospital a la hormona de crecimiento, tanto antes como despues del transplante renal es determinado para eventualmente utilizar terapias paralelas al transplante, fueron determinadas. Un estudio ecocardiografico es recomendado realizar como parte del tratamiento de la insuficiencia renal cronica para identificar la existencia de hipertrofia ventricular izquierda e insuficiencia cardiaca, que pueden ocurrir como consecuencia de las complicaciones de la insuficiencia.

A rare case of renal infarction caused by infective endocarditis.

Science.gov (United States)

Zakaria, Rasheed; Forsyth, Vhari; Rosenbaum, Tomas

2009-10-01

A 29-year-old man presented to the emergency department of a general hospital complaining of sudden onset left loin pain, radiating to the groin, which had started 48 h previously. He described no urological symptoms and had no medical history of note. Physical examination, electrocardiography, dipstick testing of urine, radiography of the chest and abdomen, blood tests (white blood cell count and serum urea, creatinine, sodium, potassium and C-reactive protein levels), CT of the renal tract, blood and urine cultures, renal angiography, thromboembolic blood panel, urine and blood tests for illicit drugs, transthoracic echocardiography, transesophageal echocardiography, renal ultrasonography. Infective endocarditis resulting in thromboembolic unilateral renal infarction. The patient was started on anticoagulation therapy with low-molecular-weight heparin and treated with intravenous gentamicin and benzylpenicillin for 4 weeks. He was seen in an outpatient clinic 4 weeks after discharge, at which time serum urea and creatinine levels and repeat ultrasonography of the renal tract confirmed normal renal function. He will be followed up regularly by cardiologists and urologists, at 6 weeks initially, and every 6 months to 1 year thereafter by his family physician.

C19orf12 mutations in neurodegeneration with brain iron accumulation mimicking juvenile amyotrophic lateral sclerosis.

Science.gov (United States)

Deschauer, M; Gaul, C; Behrmann, C; Prokisch, H; Zierz, S; Haack, T B

2012-11-01

Mutations in C19orf12 have been recently identified as the molecular genetic cause of a subtype of neurodegeneration with brain iron accumulation (NBIA). Given the mitochondrial localization of the gene product the new NBIA subtype was designated mitochondrial membrane protein-associated neurodegeneration. Frequent features in the patients described so far included extrapyramidal signs and pyramidal tract involvement. Here, we report three C19orf12-mutant patients from two families presenting with predominant upper and lower motor neuron dysfunction mimicking amyotrophic lateral sclerosis with juvenile onset. While extrapyramidal signs were absent, all patients showed neuropsychological abnormalities with disinhibited or impulsive behavior. Optic atrophy was present in the simplex case. T2-weighted cranial MRI showed hypointensities suggestive of iron accumulation in the globi pallidi and the midbrain in all patients. Sequence analysis of C19orf12 revealed a novel mutation, p.Gly66del, compound heterozygous with known mutations in all patients. These patients highlight that C19orf12 defects should be considered as a differential diagnosis in patients with juvenile onset motor neuron diseases. Patients have to be examined carefully for neuropsychological abnormalities, optic neuropathy, and signs of brain iron accumulation in MRI.

[From gene to disease; mutations in the WFS1-gene as the cause of juvenile type I diabetes mellitus with optic atrophy (Wolfram syndrome)

NARCIS (Netherlands)

Pennings, R.J.E.; Dikkeschei, L.D.; Cremers, C.W.R.J.; Ouweland, J.M.W. van den

2002-01-01

Wolfram syndrome patients are mainly characterised by juvenile onset diabetes mellitus and optic atrophy. A synonym is the acronym DIDMOAD: diabetes insipidus, diabetes mellitus, optic atrophy, deafness. Diabetes insipidus and sensorineural high-frequency hearing impairment are important additional

A clinical study and the diagnosis in magnetic resonance imaging of renal scarring

International Nuclear Information System (INIS)

Tsugaya, Masayuki; Hirao, Noriaki; Ohtaguro, Kazuo; Kato, Jiro.

1989-01-01

Twenty-nine kidneys of seventeen patients (nine boys and eight girls) with vesicoureteral reflux and repeated urinary tract infection were studied by magnetic resonance imaging for diagnosis of renal scarring and correlation between clinical data and the degree renal scarring. Renal scarring is classified into three types according to findings in magnetic resonance imaging. The degree of renal scarring are classified into five grades according to traditional grading of intravenous pyelogram. If a fine deformity of calyx is shown on intravenous pyelogram, magnetic resonance imaging demonstrates renal scarring. Magnetic resonance imaging without irradiation is exceedingly valuable for the diagnosis of renal scarring. The appearances of magnetic resonance imaging were supported by X-ray computed tomography. There is a substantial correlation between serum creatinine and the grades of renal scarring by magnetic resonance imaging. There is a substantial correlation between fever attacks and the grade of renal scarring, and there is a significant reverse correlation between the age of the onset of upper urinary tract infection and the grade of renal scarring. It is suggested that upper urinary tract infection is the most significant factor in scar formation. (author)

Autoantibodies to IL-1 alpha in sera from umbilical cords, children, and adults, and from patients with juvenile chronic arthritis

DEFF Research Database (Denmark)

Müller, K; Hansen, M B; Zak, M

1996-01-01

umbilical cords (n = 11), children (n = 45), and adults (n = 20), as well as in 51 patients with juvenile chronic arthritis (JCA) of pauciarticular (n = 34), polyarticular (n = 8), or systemic onset type (n = 9). RESULTS. The frequency of positive sera was significantly lower in children than in cord blood...

Experimental Transmission of Frog Virus 3-Like Ranavirus in Juvenile Chelonians at Two Temperatures.

Science.gov (United States)

Allender, Matthew C; Barthel, Ashley C; Rayl, Jeremy M; Terio, Karen A

2018-06-07

The pathogenicity of frog virus 3 (FV3)-like ranavirus varies in adult chelonian species at different environmental temperatures, but differences in pathogenicity at different temperatures has yet to be determined in juveniles. Our objective was to determine the susceptibility to FV3-like ranavirus in four species of juvenile chelonians: red-eared sliders (RES; Trachemys scripta elegans), Mississippi map turtles ( Graptemys pseudogeographica kohnii), false map turtles (FMT; Graptemys pseudogeographica), and eastern river cooters ( Pseudemys concinna concinna) at two environmental temperatures. Two simultaneous trials ( n=8 treatment and n=4 controls of each species) were conducted in separate temperature-controlled rooms with animals maintained at 22 C or 27 C. All of the inoculated animals of each species at each temperature died, but no mortality was observed in control animals. Median survival times varied between 8 d and 11 d, based on species and temperature, with RES in the 27 C trial surviving the shortest time and the FMT in the 22 C trial surviving the longest. Combining all species, turtles in the 27 C trial survived for fewer days than those housed at 22 C, despite all turtles in both trials having similar viral copies detected in postmortem tissues. Lesions in inoculated turtles resembled those noted in natural and experimental FV3-like ranavirus infections and included vasculitis, thrombosis, hemorrhage in multiple organs, renal tubular necrosis, and hepatic necrosis. Myositis was not present in any juvenile, infected turtles in this study. This study confirmed that juvenile chelonians have a high susceptibility to ranaviral disease.

Subcutaneous blood flow during insulin-induced hypoglycaemia: studies in juvenile diabetics with and without autonomic neuropathy and in normal subjects

Energy Technology Data Exchange (ETDEWEB)

Hilsted, J; Madsbad, S; Sestoft, L

1982-08-01

Subcutaneous blood flow was measured preceding insulin-induced hypoglycaemia, at the onset of hypoglycaemic symptoms and 2 h later in juvenile diabetics with and without autonomic neuropathy and in normal males. In all groups subcutaneous blood flow decreased at the onset of hypoglycaemic symptoms compared with pre-hypoglycaemic flow. Two hours after onset of hypoglycaemic symptoms, subcutaneous blood flow was still significantly decreased compared with pre-hypoglycaemic flow. In normal subjects local nerve blockade had no effect on blood flow changes during hypoglycaemia, whereas local alpha-receptor blockade abolished the vasoconstrictor response. We suggest that circulating catecholamines stimulating vascular alpha-receptors are probably responsible for flow reduction in the subcutaneous tissue during hypoglycaemia.

Juvenile Court Statistics - 1972.

Science.gov (United States)

Office of Youth Development (DHEW), Washington, DC.

This report is a statistical study of juvenile court cases in 1972. The data demonstrates how the court is frequently utilized in dealing with juvenile delinquency by the police as well as by other community agencies and parents. Excluded from this report are the ordinary traffic cases handled by juvenile court. The data indicate that: (1) in…

Juvenile Court Statistics, 1974.

Science.gov (United States)

Corbett, Jacqueline; Vereb, Thomas S.

This report presents information on juvenile court processing of youth in the U.S. during 1974. It is based on data gathered under the National Juvenile Court Statistical Reporting System. Findings can be summarized as follows: (1) 1,252,700 juvenile delinquency cases, excluding traffic offenses, were handled by courts in the U.S. in 1974; (2) the…

The role of low levels of juvenile hormone Esterase in the metamorphosis of Manduca sexta

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M.H. Browder

2001-10-01

Full Text Available The activity of juvenile hormone esterase (JHE in feeding fifth instar larvae of Manduca sexta increases gradually with larval weight and rises to a peak after larvae pass the critical weight when juvenile hormone secretion ceases. Starvation of larvae of Manduca sexta (L. that had exceeded the critical weight inhibited peak levels of JHE, but did not delay entry into the wandering stage when larvae leave the plant in search of a pupation site. This suggests that peak levels of JHE may not be essential for the normal timing of metamorphosis. Starved larvae pupated normally, indicating the peak of JHE was not necessary for a morphologically normal pupation. Treatments of larvae with the selective JHE inhibitor O-ethyl-S-phenyl phosphoramidothiolate (EPPAT that began immediately after larvae achieved the critical weight (6.0 to 6.5 grams for our strain of Manduca delayed entry into the wandering stage. By contrast, EPPAT treatment of larvae at weights above 8.0g had no effect on the subsequent timing of the onset of wandering. Therefore, although the normal timing of the onset of wandering does not require peak levels of JHE, it requires low to moderate levels of JHE to be present until larvae reach a weight of about 8.0g.

PHARMACOKINETICS OF ROCURONIUM BROMIDE IN PATIENTS WITH AND WITHOUT RENAL-FAILURE

NARCIS (Netherlands)

COOPER, RA; MIRAKHUR, RK; WIERDA, JMKH; MADDINENI, VR

We studied the onset and duration of action and pharmacokinetics of rocuronium bromide during anaesthesia with nitrous oxide, fentanyl and isoflurane after a single bolus dose of rocuronium (0.6 mg kg(-1)) in nine patients with chronic renal failure requiring regular haemodialysis, and in nine

Diagnosis of myxedema coma complicated by renal failure: a case report.

Science.gov (United States)

Takamura, Akiteru; Sangen, Ryusho; Furumura, Yoshiki; Usuda, Daisuke; Kasamaki, Yuji; Kanda, Tsugiyasu

2017-04-01

Myxedema coma, caused by severe lack of thyroid hormone, is characterized by deterioration of mental status, hypothermia, hypotension, hyponatremia, and hypoventilation. We describe an 84-year-old woman who presented with renal failure and new onset severe hypothyroidism leading to challenges in the recognition of myxedema coma.

Early administration of tolvaptan preserves renal function in elderly patients with acute decompensated heart failure.

Science.gov (United States)

Kimura, Kazuhiro; Momose, Tomoyasu; Hasegawa, Tomoya; Morita, Takehiro; Misawa, Takuo; Motoki, Hirohiko; Izawa, Atsushi; Ikeda, Uichi

2016-05-01

Loop diuretics used in the treatment of heart failure often induce renal impairment. This study was conducted in order to evaluate the renal protective effect of adding tolvaptan (TLV), compared to increasing the furosemide (FRM) dose, for the treatment of acute decompensated heart failure (ADHF) in a real-world elderly patient population. This randomized controlled trial enrolled 52 consecutive hospitalized patients (age 83.4±9.6 years) with ADHF. The patients were assigned alternately to either the TLV group (TLV plus conventional treatment, n=26) or the FRM group (increasing the dose of FRM, n=26). TLV was administered within 24h from admission. The incidence of worsening renal function (WRF) within 7 days from admission was significantly lower in the TLV group (26.9% vs. 57.7%, p=0.025). Furthermore, the rates of occurrence of persistent and late-onset (≥5 days from admission) WRF were significantly lower in the TLV group. Persistent and late-onset WRF were significantly associated with a higher incidence of cardiac death or readmission for worsening heart failure in the 90 days following discharge, compared to transient and early-onset WRF, respectively. Early administration of TLV, compared to increased FRM dosage, reduces the incidence of WRF in real-world elderly ADHF patients. In addition, it reduces the occurrence of 'worse' WRF-persistent and late-onset WRF-which are associated with increased rates of cardiac death or readmission for worsening heart failure in the 90 days after discharge. Copyright © 2015 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

THE STUDY OF FEATURES OF GUILT OF JUVENILE OFFENDERS IN THE CONTEXT OF JUVENILE JUSTICE

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Natalija Vladimirovna Galkina

2015-08-01

Full Text Available The article is devoted to the results of empirical studies of the experiences of guilt of juvenile offenders in the context of juvenile justice where a minor appears as the subject of legal relations. Restorative approach of juvenile justice is based on an admission of guilt to the victim. In connection with it, the research of features of the guilt of minors who have committed an offence and the conditions for the development of the subjectivity will enhance understanding of the possibilities of restorative juvenile justice system in the prevention of juvenile delinquency.Thus, the results of empirical research presented in the article are important for determining of the psychological bases of realization of rehabilitation programs in the context of juvenile justice. In particular, the results are important for the organization and conduct of psychological work to overcome the psychological barriers in the behavior of juveniles having inherently maladaptive guilt and destructive psychological defense mechanisms.

Juvenile Justice in Mexico

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Martha Frías Armenta

2014-08-01

Full Text Available The first tribunal in Mexico was established in the central state of San Luis Potosi in 1926. The Law Regarding Social Prevention and Juvenile Delinquency for the Federal District and Mexican territories was promulgated in 1928. In 2005, Article 18 of the Mexican Constitution was modified to establish a comprehensive system (“Sistema Integral de justicia” in Spanish of justice for juveniles between 12 and 18 years old who had committed a crime punishable under criminal law. Its objective was to guarantee juveniles all the due process rights established for adults, in addition to the special ones recognized for minors. The constitutional reform also provides a framework that includes special tribunals as well as alternative justice options for juveniles. With these reforms, institutionalization of minors was to be considered an extreme measure applicable only to felonies and to juveniles older than 14. In 2006, all states within the Mexican federation enacted the “Law of justice for adolescents”. This system, at both the federal and state levels, formalizes a new global paradigm with regard to the triangular relationship between children, the State and the Law. It recognizes that children are also bearers of the inherent human rights recognized for all individuals, instead of simply objects in need of protection. However, despite formally aligning Mexican juvenile justice law with the Convention on the Rights of the Child (CRC, issues of actual substantive rights remained and new ones have appeared. For example, juveniles younger than 14 who have not committed a felony are released from institutions without any rehabilitation or treatment options, and alternative forms of justice were included without evaluating their possibilities of application or their conditions for success. In addition, the economic status of most juvenile detainees continues to be one of the most important determining factors in the administration of justice

Tacrolimus Versus Cyclosporine as Primary Immunosuppressant After Renal Transplantation: A Meta-Analysis and Economics Evaluation.

Science.gov (United States)

Liu, Jin-Yu; You, Ru-Xu; Guo, Min; Zeng, Lu; Zhou, Pu; Zhu, Lan; Xu, Gang; Li, Juan; Liu, Dong

2016-01-01

Tacrolimus and cyclosporine are the major immunosuppressants for renal transplantation. Several studies have compared these 2 drugs, but the outcomes were not consistent. The aim of this study was to evaluate the efficacy, safety, and pharmacoeconomics of cyclosporine and tacrolimus in the treatment of renal transplantation and provide evidence for the selection of essential drugs. Trials were identified through a computerized literature search of PubMed, EMBASE, Cochrane Controlled Trials Register, Cochrane Renal Group Specialized Register of randomized controlled trials, and Chinese Biomedical database. Two independent reviewers assessed trials for eligibility and quality and then extracted data. Data were extracted for patient and graft mortality, acute rejection, and adverse events. Dichotomous outcomes were reported as relative risk with 95% confidence intervals. A decision tree model was populated with data from a literature review and used to estimate costs and quality-adjusted life years gained and incremental cost-effectiveness. Altogether, 6137 patients from 27 randomized controlled trials were included. The results of our analysis were that tacrolimus reduced the risks after renal transplantation of patient mortality, graft loss, acute rejection, and hypercholesterolemia. Nevertheless, tacrolimus increased the risk of new-onset diabetes. Pharmacoeconomic analysis showed that tacrolimus represented a more cost-effective treatment than does cyclosporine for the prevention of adverse events following renal transplant. Tacrolimus is an effective and safe immunosuppressive agent and it may be more cost-effective than cyclosporine for the primary prevention of graft rejection in renal transplant recipients. However, new-onset diabetes should be closely monitored during the medication period.

Juvenile mammary papillomatosis; Papilomatosis juvenil mamaria

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Alvarez, M.; Jimenez, A. V. [Hospital Reina Sofia. Cordoba (Spain)

2001-07-01

Juvenile mammary papillomatosis is a benign proliferative disease of young patients, generally under 30 years of age. The most frequent clinical presentation is the existence of an elastic and mobile lymph node of the breast. Anatomopathologically, it is characterized because it presents ductal epithelial hyperplasia, sometimes with marked atypia, and there are numerous cysts having different sizes among the findings. It has been associated with an increase in the incidence of breast cancer, both in the patient herself as well as her family. We review the literature on the subject and present the mammographic and ultrasonographic findings of a 22 year old woman diagnosed of juvenile mammary papillomatosis. (Author) 12 refs.

Childhood- versus adolescent-onset antisocial youth with conduct disorder: psychiatric illness, neuropsychological and psychosocial function.

Science.gov (United States)

Johnson, Vicki A; Kemp, Andrew H; Heard, Robert; Lennings, Christopher J; Hickie, Ian B

2015-01-01

The present study investigates whether youths with childhood-onset antisocial behavior have higher rates of psychiatric illness, neuropsychological and psychosocial dysfunction than youths who engage in antisocial behavior for the first time in adolescence. Prior studies have generally focused on single domains of function in heterogeneous samples. The present study also examined the extent to which adolescent-onset antisocial behavior can be considered normative, an assumption of Moffitt's dual taxonomy model. Forty-three subjects (34 males, 9 females, mean age = 15.31, age range 12-21) with a diagnosis of conduct disorder (CD) were recruited through Headspace Services and the Juvenile Justice Community Centre. We compared childhood-onset antisocial youths (n = 23) with adolescent-onset antisocial youths (n = 20) with a conduct disorder, across a battery of psychiatric, neuropsychological and psychosocial measures. Neuropsychological function of both groups was also compared with normative scores from control samples. The childhood-onset group displayed deficits in verbal learning and memory, higher rates of psychosis, childhood maltreatment and more serious violent behavior, all effects associated with a large effect size. Both groups had impaired executive function, falling within the extremely low range (severely impaired). Childhood-onset CD displayed greater cognitive impairment, more psychiatric symptoms and committed more serious violent offences. The finding of severe executive impairment in both childhood- and adolescent-onset groupings challenges the assumption that adolescent-onset antisocial behavior is a normative process.

Possible hepatotoxic consequence of nevirapine use in juvenile albino rats

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Elias Adikwu

2017-08-01

Full Text Available Context: Nevirapine (NVP is used in human immunodeficiency virus exposed neonates. This could present safety concern due to decreased liver metabolizing enzymes activity and renal clearance in neonates. Aims: To determine the hepatotoxic effect of NVP in juvenile albino rats. Methods: Juvenile albino rats were weighed, divided into groups and treated orally with 4-32 mg/kg/day of NVP for 14 days including a recovery group. The control groups were treated with water (placebo and normal saline (solvent. At the end of NVP treatment, rats were weighed and sacrificed, blood was collected and serum extracted. Serum was analyzed for alanine aminotransferase (ALT, aspartate aminotransferase (AST, alkaline phosphatase (ALP, total bilirubin (TB and conjugated bilirubin (CB. The liver was harvested via dissection, weighed and evaluated for AST, ALT, ALP, superoxide dismutase (SOD, catalase (CAT, glutathione (GSH, malondialdehyde (MDA levels and histological damage. Results: The body, absolute and relative liver weights of rats in NVP treated groups were not significantly different (p>0.05 when compared to placebo. However, serum levels of AST, ALT, ALP, TB and CB were significantly increased (p<0.05 in a dose-dependent manner in NVP-treated groups. Furthermore, liver levels of ALT, ALP, AST and MDA were significantly increased (p<0.05 while SOD, CAT, and GSH were decreased in a dose dependent manner in NVP-treated groups. NVP-treated rats were characterized by varying degrees of hepatic morphological alterations. However, in the recovery group, the effects of NVP were reversed. Conclusions: This study observed dose-dependent and reversible hepatotoxicity in nevirapine- treated juvenile albino rats.

[Macrophage activation syndrome in a patient with systemic juvenile idiopathic arthritis].

Science.gov (United States)

Tavares, Anna Carolina Faria Moreira Gomes; Ferreira, Gilda Aparecida; Guimarães, Luciano Junqueira; Guimarães, Raquel Rosa; Santos, Flávia Patrícia Sena Teixeira

2015-01-01

Machrophage activation syndrome (MAS) is a rare and potentially fatal disease, commonly associated with chronic rheumatic diseases, mainly juvenile idiopathic arthritis. It is included in the group of secondary forms of haemophagocytic syndrome, and other causes are lymphoproliferative diseases and infections. Its most important clinical and laboratorial manifestations are non-remitting fever, splenomegaly, bleeding, impairment of liver function, cytopenias, hypoalbuminemia, hypertriglyceridemia, hypofibrinogenemia and hyperferritinemia. The treatment needs to be started quickly, and the majority of cases have a good response with corticosteroids and cyclosporine. The Epstein-Barr virus is described as a possible trigger for many cases of MAS, especially in these patients in treatment with tumor necrosis factor (TNF) blockers. In these refractory cases, etoposide (VP16) should be administered, associated with corticosteroids and cyclosporine. Our objective is to describe a rare case of MAS probably due to EBV infection in a subject with systemic-onset juvenile idiopathic arthritis, which achieved complete remission of the disease after therapy guided by 2004-HLH protocol. Copyright © 2014 Elsevier Editora Ltda. All rights reserved.

[Early diagnosis, variety of outcomes and prognosis of juvenile schizophrenia with over-value disorders of the "metaphysical intoxication" type onset (results of a late follow-up study)].

Science.gov (United States)

Tsutsul'kovskaia, M Ia; Izvol'skiĭ, S A; Bol'zho, A G; Kopeĭko, G I

1986-01-01

On the basis of follow-up findings about juvenile schizophrenia first expressed in superworship manifestations according to the type of metaphysical intoxication (98 observations), the authors established that most frequently these cases were characterized by a torpid course of the disease in the framework of slowly progressing juvenile schizophrenia. There was a high rate of favourable outcomes at the level of "clinical recovery" (in 42.5%). Favourable prognostic signs were as follows: the complete nature of the clinical picture of the syndrome, its similarity to pubertal crisis manifestations, the absence in its structure of other positive disturbances and a number of specific features of the premorbid picture. The authors determined the pattern of correlation between the typological characteristics of the state of juvenile metaphysical intoxication and the outcome of the disease, peculiarities of manifestations of this phenomenon at the initial stage of psychotic forms of juvenile schizophrenia, as well as distinctive features of superworship formations according to the type of juvenile metaphysical intoxication observed in the framework of pubertal decompensation in schizoid psychopathies.

Diagnosis of myxedema coma complicated by renal failure: a case report

OpenAIRE

Takamura, Akiteru; Sangen, Ryusho; Furumura, Yoshiki; Usuda, Daisuke; Kasamaki, Yuji; Kanda, Tsugiyasu

2017-01-01

Key Clinical Message Myxedema coma, caused by severe lack of thyroid hormone, is characterized by deterioration of mental status, hypothermia, hypotension, hyponatremia, and hypoventilation. We describe an 84?year?old woman who presented with renal failure and new onset severe hypothyroidism leading to challenges in the recognition of myxedema coma.

Juvenile giant fibroadenoma

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Vipul Yagnik

2011-07-01

Full Text Available Fibroadenomas are benign solid tumor associated with aberration of normal lobular development. Juvenile giant fibroadenoma is usually single and >5 cm in size /or >500 gms in weight. Important differential diagnoses are: phyllodes tumor and juvenile gigantomastia. Simple excision is the treatment of choice.

The influence of partial renal function impairment on thyroid hormones level in acute renal failure-ARF patients

International Nuclear Information System (INIS)

Ilic, S.; Vlajkovic, M.; Rajic, M.; Bogicevic, M.

2002-01-01

The aim of this study was to assess the relationship of thyroid hormone levels with glomerular and tubular renal function in ARF. In thirty one patients with different underlying diseases, glomerular filtration (GFR) was estimated by measuring 99mTc-DTPA clearance and tubular function by 131I-OIH clearance. The thyroid status was evaluated by radioimmunoassays of T4, FT4, T3, FT3, rT3 and TSH, while the cortisol level was used to indicate the stress role. All the measurements were performed within 7 days as well after 1,3,6 and 12 months from the ARF onset. The results obtained were compared with those of 30 healthy subjects. At the ARF beginning, the impairment of renal function was manifested by the fall of 99mTc-DTPA clearance to 21∫7ml/min and 131I-OIH clearance to 172±34ml/min, while end values were 77±19ml/min and 395±70ml/min, respectively. The hormone analysis within 7 days, showed T3 decrease to 1.21±0.16nmol/l and the increase rT3 to 38±7ng/1ooml, as well as cortisol to 350±71ng/ml, but at the end of the study these hormone levels were normalized. Other hormone levels didn't differ significantly from control values. Regression analysis established significant correlation of 99mTc-DTPA clearance with T3 (r=0.693, p 100, OIH>500), partial recovery (DTPA 51-100, OIH 250-500) and without any recovery (DTPA<50,OIH<250), showed hormone disturbances proportional to the degree of renal function damage. The most severe disorders were found in the third group, where both total and free T4 and T3 were lowered, while rT3 and cortisol elevated at 7th day from ARF onset. After 12 months all these values associated with that of TSH were observed as declined.This study suggests thyroid hormone levels to be dependent on the renal function in ARF. Greater impairment of glomerular related to tubular function appears to be more responsible for thyroid dysfunction, due to uremic toxins retention as well as stress induced by the extreme function failure

Temporary Efficacy of Pyrimethamine in Juvenile-Onset Tay-Sachs Disease Caused by 2 Unreported HEXA Mutations in the Indian Population.

Science.gov (United States)

Udwadia-Hegde, Anaita; Hajirnis, Omkar

2017-01-01

Juvenile Tay-Sachs disease is rarer than other forms of Tay-Sachs disease and is usually seen in children between the age of 2 and 10 years. Pyrimethamine as a pharmacological chaperone was used to increase β-hexosaminidase A activity in this patient. We describe a patient with Tay-Sachs disease from the Indian population, a juvenile case who presented with developmental regression starting at the age of three, initially with motor followed by language regression. She is currently incapacitated with severe behavioral issues. This brief communication gives an insight into the efficacy of pharmacological chaperones. It also describes two unreported mutations in hexosaminidase A gene from the Indian population. After commencing Pyrimethamine, though initial benefits with increase in levels corresponded with briefly halting the motor regression, the observed increase was only transient and not associated with discernible beneficial neurological or psychiatric effects.

Juvenile Idiopathic Arthritis

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Kenan Barut

2017-04-01

Full Text Available Juvenile idiopathic arthritis is the most common chronic rheumatic disease of unknown aetiology in childhood and predominantly presents with peripheral arthritis. The disease is divided into several subgroups, according to demographic characteristics, clinical features, treatment modalities and disease prognosis. Systemic juvenile idiopathic arthritis, which is one of the most frequent disease subtypes, is characterized by recurrent fever and rash. Oligoarticular juvenile idiopathic arthritis, common among young female patients, is usually accompanied by anti-nuclear antibodie positivity and anterior uveitis. Seropositive polyarticular juvenile idiopathic arthritis, an analogue of adult rheumatoid arthritis, is seen in less than 10% of paediatric patients. Seronegative polyarticular juvenile idiopathic arthritis, an entity more specific for childhood, appears with widespread large- and small-joint involvement. Enthesitis-related arthritis is a separate disease subtype, characterized by enthesitis and asymmetric lower-extremity arthritis. This disease subtype represents the childhood form of adult spondyloarthropathies, with human leukocyte antigen-B27 positivity and uveitis but commonly without axial skeleton involvement. Juvenile psoriatic arthritis is characterized by a psoriatic rash, accompanied by arthritis, nail pitting and dactylitis. Disease complications can vary from growth retardation and osteoporosis secondary to treatment and disease activity, to life-threatening macrophage activation syndrome with multi-organ insufficiency. With the advent of new therapeutics over the past 15 years, there has been a marked improvement in juvenile idiopathic arthritis treatment and long-term outcome, without any sequelae. The treatment of juvenile idiopathic arthritis patients involves teamwork, including an experienced paediatric rheumatologist, an ophthalmologist, an orthopaedist, a paediatric psychiatrist and a physiotherapist. The primary goals

Multiple carcinomas in the hemodialysis access induced ischemic hand of a renal transplant patient.

NARCIS (Netherlands)

Hoek, F. van; Tits, H.W.H.J.; Lijnschoten, I. Van; Haas, B.D. De; Scheltinga, M.R.M.

2010-01-01

Long term immunosuppression following organ transplantation promotes the onset of skin cancers. A renal transplant patient developed multiple hyperkeratotic nodi in the left hand and digital pain following prolonged immunosuppression. Several skin abnormalities were observed in an ischemic and

The Forgotten Complication of Recurrent Nephrolithiasis: “Squamous Cell Carcinoma of the Renal Pelvis”—A Case and Review of the Literature

OpenAIRE

Tarek Assi; Elie El Rassy; Samah Naderi; Tania Moussa; Maroun Moukarzel; Joseph Kattan

2015-01-01

Squamous cell carcinoma (SCC) of the renal pelvis is an aggressive tumor with insidious onset of unspecific symptoms and advanced stages at diagnosis. It is a rare entity, accounting for 0.5–8% of renal tumors. In this paper, we describe the case of a patient with a history of recurrent nephrolithiasis that presented with an aggressive form of SCC of the renal pelvis with rapid relapse after resection.

Steroid withdrawal in renal transplant patients: the Irish experience.

LENUS (Irish Health Repository)

Phelan, P J

2012-02-01

BACKGROUND: Steroid therapy is associated with significant morbidity in renal transplant recipients. However, there is concern that steroid withdrawal will adversely affect outcome. METHODS: We report on 241 renal transplant recipients on different doses of corticosteroids at 3 months (zero, <\\/= 5 mg\\/day, > 5 mg\\/day). Parameters analysed included blood pressure, lipid profile, weight change, new onset diabetes after transplantation (NODAT), allograft survival and acute rejection. RESULTS: Elimination of corticosteroids had no impact on allograft survival at 1 year. There were no cases of NODAT in the steroid withdrawal group compared with over 7% in each of the steroid groups. There were no significant improvements in weight gain, blood pressure control or total cholesterol with withdrawal of steroids before 3 months. CONCLUSIONS: In renal transplant patients treated with tacrolimus and mycophenolate, early withdrawal of steroids does not appear to adversely affect allograft outcome at 1 year. It may result in less NODAT.

Steroid withdrawal in renal transplant patients: the Irish experience.

LENUS (Irish Health Repository)

Phelan, P J

2010-10-29

BACKGROUND: Steroid therapy is associated with significant morbidity in renal transplant recipients. However, there is concern that steroid withdrawal will adversely affect outcome. METHODS: We report on 241 renal transplant recipients on different doses of corticosteroids at 3 months (zero, ≤5 mg\\/day, >5 mg\\/day). Parameters analysed included blood pressure, lipid profile, weight change, new onset diabetes after transplantation (NODAT), allograft survival and acute rejection. RESULTS: Elimination of corticosteroids had no impact on allograft survival at 1 year. There were no cases of NODAT in the steroid withdrawal group compared with over 7% in each of the steroid groups. There were no significant improvements in weight gain, blood pressure control or total cholesterol with withdrawal of steroids before 3 months. CONCLUSIONS: In renal transplant patients treated with tacrolimus and mycophenolate, early withdrawal of steroids does not appear to adversely affect allograft outcome at 1 year. It may result in less NODAT.

Imaging of renal metastases

International Nuclear Information System (INIS)

Bruneton, J.N.; Normand, F.; Balu-Maestro, C.; Rogopoulos, A.; Drouillard, J.; Laurent, F.

1988-01-01

Metastases are the most frequent malignant tumors of the kidney, but these lesions are of late onset in neoplastic disease. The 19 cases reported here were all investigated with various imaging techniques (CT 12 cases, ultrasonography 12 cases, urography 8 cases, angiography 2 cases, MRI 1 case). The most common primary malignancies were lung cancer, melanoma and cancer of the controlateral kidney. In this series, 8 of the lesions were solitary, and 9 were unilateral. Tumor vascularity was evaluated in 15 cases: 14 of these lesions were hypovascular. The differential diagnosis includes small cysts, lymphoma, bilateral renal cancer, multiple small abscesses and multiple small infarcts [fr

The frequency of pulmonary hypertension in patients with juvenile scleroderma

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Amra Adrovic

2015-08-01

Full Text Available Juvenile scleroderma (JS represents a rarely seen group of connective tissue diseases with multiple organ involvement. Cardiac involvement in JSS is well known and, although rare in children, it may be an important cause of mortality and morbidity. Therefore, an early determination of cardio-vascular and pulmonary involvement is of the most relevance to reduce the mortality in patients with juvenile scleroderma. The aim of the study was to explore the non-invasive methods (Doppler echocardiography, pulmonary function tests, Forced vital capacity (FVC and Carbon monoxide diffusion capacity (DLCO in the assessment of the cardiopulmonary involvement in patients with JS. The assessment of pulmonary arterial pressure (PAP and risk factors for pulmonary arterial hypertension (PAH were made by the measurement of maximum tricuspid insufficiency (TI, end-diastolic pulmonary insufficiency (PI, ratio of acceleration time (AT to ejection time (ET (AT/ET, right atrial pressure (RAP and contraction of vena cava inferior during inspiration. Thirty-five patients with confirmed JS were included in the study. The mean age of onset of the disease was 9.57 years (median 10 years, range 2-18 years. The mean disease duration and follow-up time was 2 years (median 1 year, range 0.5-8 years and 3.57 years (median 2 years, range 0.5-14.5 years, respectively.The values of all the analyzed parameters including TI, PI, AT/ET, PAP, FVC and DLCO were found to be within normal ranges in all the patients tested, confirming an uncommonness of cardiopulmonary involvement in patients with juvenile scleroderma.

The frequency of pulmonary hypertension in patients with juvenile scleroderma.

Science.gov (United States)

Adrovic, Amra; Oztunc, Funda; Barut, Kenan; Koka, Aida; Gojak, Refet; Sahin, Sezgin; Demir, Tuncalp; Kasapcopur, Ozgur

2015-08-22

Juvenile scleroderma (JS) represents a rarely seen group of connective tissue diseases with multiple organ involvement. Cardiac involvement in JSS is well known and, although rare in children, it may be an important cause of mortality and morbidity. Therefore, an early determination of cardio-vascular and pulmonary involvement is of the most relevance to reduce the mortality in patients with juvenile scleroderma. The aim of the study was to explore the non-invasive methods (Doppler echocardiography, pulmonary function tests), Forced vital capacity (FVC) and Carbon monoxide diffusion capacity (DLCO) in the assessment of the cardiopulmonary involvement in patients with JS. The assessment of pulmonary arterial pressure (PAP) and risk factors for pulmonary arterial hypertension (PAH) were made by the measurement of maximum tricuspid insufficiency (TI), end-diastolic pulmonary insufficiency (PI), ratio of acceleration time (AT) to ejection time (ET) (AT/ET), right atrial pressure (RAP) and contraction of vena cava inferior during inspiration. Thirty-five patients with confirmed JS were included in the study. The mean age of onset of the disease was 9.57 years (median 10 years, range 2-18 years). The mean disease duration and follow-up time was 2 years (median 1 year, range 0.5-8 years) and 3.57 years (median 2 years, range 0.5-14.5 years), respectively.The values of all the analyzed parameters including TI, PI, AT/ET, PAP, FVC and DLCO were found to be within normal ranges in all the patients tested, confirming an uncommonness of cardiopulmonary involvement in patients with juvenile scleroderma.

Evidence of small-fiber polyneuropathy in unexplained, juvenile-onset, widespread pain syndromes.

Science.gov (United States)

Oaklander, Anne Louise; Klein, Max M

2013-04-01

We tested the hypothesis that acquired small-fiber polyneuropathy (SFPN), previously uncharacterized in children, contributes to unexplained pediatric widespread pain syndromes. Forty-one consecutive patients evaluated for unexplained widespread pain beginning before age 21 had medical records comprehensively analyzed regarding objective diagnostic testing for SFPN (neurodiagnostic skin biopsy, nerve biopsy, and autonomic function testing), plus histories, symptoms, signs, other tests, and treatments. Healthy, demographically matched volunteers provided normal controls for SFPN tests. Age at illness onset averaged 12.3 ± 5.7 years; 73% among this poly-ethnic sample were female (P = .001). Sixty-eight percent were chronically disabled, and 68% had hospitalizations. Objective testing diagnosed definite SFPN in 59%, probable SFPN in 17%, and possible SFPN in 22%. Only 1 of 41 had entirely normal SFPN test results. Ninety-eight percent of patients had other somatic complaints consistent with SFPN dysautonomia (90% cardiovascular, 82% gastrointestinal, and 34% urologic), 83% reported chronic fatigue, and 63% had chronic headache. Neurologic examinations identified reduced sensation in 68% and vasomotor abnormalities in 55%, including 23% with erythromelalgia. Exhaustive investigations for SFPN causality identified only history of autoimmune illnesses in 33% and serologic markers of disordered immunity in 89%. Treatment with corticosteroids and/or intravenous immune globulin objectively and subjectively benefited 80% of patients (12/15). More than half among a large series of patients with childhood-onset, unexplained chronic widespread pain met rigorous, multitest, diagnostic criteria for SFPN, which extends the age range of acquired SFPN into early childhood. Some cases appeared immune-mediated and improved with immunomodulatory therapies.

Hypophosphatemia in a Malnourished Child: When Renal Fanconi Syndrome Does Not Stand for Refeeding Syndrome.

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Runde, Joseph; Rivera-Rivera, Edgardo; Pompeii-Wolfe, Cecelia; Clardy, Christopher; Sentongo, Timothy

2018-05-10

Refeeding syndrome is diagnosed based on the onset of multiple laboratory abnormalities (most commonly hypophosphatemia) and clinical signs in the setting of nutrition rehabilitation of malnourished patients. Because definitions are not uniform, a broad differential diagnosis should always include renal tubular dysfunction. Our report details a 3 year-old child with undiagnosed renal tubular dysfunction who presented with the clinical picture of refeeding syndrome with refractory electrolyte abnormalities. A diagnosis of renal Fanconi syndrome was made after urinalysis that revealed glucosuria and urine electrolyte losses. Thus, urinalysis can aid in making a positive diagnosis of refeeding syndrome. © 2018 American Society for Parenteral and Enteral Nutrition.

Childhood- versus adolescent-onset antisocial youth with conduct disorder: psychiatric illness, neuropsychological and psychosocial function.

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Vicki A Johnson

Full Text Available The present study investigates whether youths with childhood-onset antisocial behavior have higher rates of psychiatric illness, neuropsychological and psychosocial dysfunction than youths who engage in antisocial behavior for the first time in adolescence. Prior studies have generally focused on single domains of function in heterogeneous samples. The present study also examined the extent to which adolescent-onset antisocial behavior can be considered normative, an assumption of Moffitt's dual taxonomy model.Forty-three subjects (34 males, 9 females, mean age = 15.31, age range 12-21 with a diagnosis of conduct disorder (CD were recruited through Headspace Services and the Juvenile Justice Community Centre. We compared childhood-onset antisocial youths (n = 23 with adolescent-onset antisocial youths (n = 20 with a conduct disorder, across a battery of psychiatric, neuropsychological and psychosocial measures. Neuropsychological function of both groups was also compared with normative scores from control samples.The childhood-onset group displayed deficits in verbal learning and memory, higher rates of psychosis, childhood maltreatment and more serious violent behavior, all effects associated with a large effect size. Both groups had impaired executive function, falling within the extremely low range (severely impaired.Childhood-onset CD displayed greater cognitive impairment, more psychiatric symptoms and committed more serious violent offences. The finding of severe executive impairment in both childhood- and adolescent-onset groupings challenges the assumption that adolescent-onset antisocial behavior is a normative process.

Temporary Efficacy of Pyrimethamine in Juvenile-Onset Tay-Sachs Disease Caused by 2 Unreported HEXA Mutations in the Indian Population

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Anaita Udwadia-Hegde MD, DCH, MRCPCH

2017-01-01

Full Text Available Background: Juvenile Tay-Sachs disease is rarer than other forms of Tay-Sachs disease and is usually seen in children between the age of 2 and 10 years. Pyrimethamine as a pharmacological chaperone was used to increase β-hexosaminidase A activity in this patient. Patient: We describe a patient with Tay-Sachs disease from the Indian population, a juvenile case who presented with developmental regression starting at the age of three, initially with motor followed by language regression. She is currently incapacitated with severe behavioral issues. Conclusion: This brief communication gives an insight into the efficacy of pharmacological chaperones. It also describes two unreported mutations in hexosaminidase A gene from the Indian population. After commencing Pyrimethamine, though initial benefits with increase in levels corresponded with briefly halting the motor regression, the observed increase was only transient and not associated with discernible beneficial neurological or psychiatric effects.

The Natural History of Juvenile or Subacute GM2 Gangliosidosis: 21 New Cases and Literature Review of 134 Previously Reported

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Maegawa, Gustavo H. B.; Stockley, Tracy; Tropak, Michael; Banwell, Brenda; Blaser, Susan; Kok, Fernando; Giugliani, Roberto; Mahuran, Don; Clarke, Joe T. R.

2010-01-01

OBJECTIVE Juvenile GM2 gangliosidosis is a group of inherited neurodegenerative diseases caused by deficiency of lysosomal β-hexosaminidase resulting in GM2 ganglioside accumulation in brain. The purpose of this study was to delineate the natural history of the condition and identify genotype-phenotype correlations that might be helpful in predicting the course of the disease in individual patients. METHODS A cohort of 21 patients with juvenile GM2 gangliosidosis, 15 with the Tay-Sachs variant and 6 with the Sandhoff variant, was studied prospectively in 2 centers. Our experience was compared with previously published reports on 134 patients. Information about clinical features, β-hexosaminidase enzyme activity, and mutation analysis was collected. RESULTS In our cohort of patients, the mean (±SD) age of onset of symptoms was 5.3 ± 4.1 years, with a mean follow-up time of 8.4 years. The most common symptoms at onset were gait disturbances (66.7%), incoordination (52.4%), speech problems (28.6%), and developmental delay (28.6%). The age of onset of gait disturbances was 7.1 ± 5.6 years. The mean time for progression to becoming wheelchair-bound was 6.2 ± 5.5 years. The mean age of onset of speech problems was 7.0 ± 5.6 years, with a mean time of progression to anarthria of 5.6 ± 5.3 years. Muscle wasting (10.6 ± 7.4 years), proximal weakness (11.1 ± 7.7 years), and incontinence of sphincters (14.6 ± 9.7 years) appeared later in the course of the disease. Psychiatric disturbances and neuropathy were more prevalent in patients with the Sandhoff variant than in those with the Tay-Sachs variant. However, dysphagia, sphincter incontinence, and sleep problems occurred earlier in those with the Tay-Sachs variant. Cerebellar atrophy was the most common finding on brain MRI (52.9%). The median survival time among the studied and reviewed patients was 14.5 years. The genotype-phenotype correlation revealed that in patients with the Tay-Sachs variant, the presence

Temporomandibular joint involvement in juvenile idiopathic arthritis: clinical predictors of magnetic resonance imaging signs

International Nuclear Information System (INIS)

Argyropoulou, Maria I.; Margariti, Persefoni N.; Astrakas, Loukas; Kosta, Paraskevi; Karali, Aikaterini; Alfandaki, Sapfo; Siamopoulou, Antigoni

2009-01-01

The aim of the study was to define clinical predictors of magnetic resonance imaging (MRI) findings of temporomandibular joint (TMJ) involvement in juvenile idiopathic arthritis (JIA). Forty-six patients, aged 2.08-36.7 years, with JIA (oligoartitular 18, polyarticular 17, systemic type 11) were examined with standard plain and contrast-enhanced sequences. Of 88 TMJs examined, an abnormal condyle was observed in 32%, flattened articular eminence in 27%, flattened articular disk in 17%, intra-articular fluid in 10%, enhancing pannus in 45% and restricted condylar motion in 9%. Logistic regression analysis revealed that for abnormal condyle and flattened articular eminence, independent predictors were type of JIA (P 0.5 when the disease started before 4 years of age. In conclusion, the systemic type of JIA, young age at onset and long duration of activity are risk factors for TMJ damage. MRI of the TMJ should be performed in patients who are less than 4 years of age at the onset of JIA, and in those with the systemic type, whatever the age of onset. (orig.)

Farmacodinâmica do cisatracúrio no transplante renal Farmacodinámica del cisatracúrio en el transplante renal Cisatracurium pharmacodynamics in renal transplantation

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Ismar Lima Cavalcanti

2002-06-01

: Cisatracurium seems to be beneficial, especially for patients with organ dysfunction, due to organ-independent Hofmann elimination and a lower trend to histamine release. This study aimed at determining cisatracurium pharmacodynamic profile in renal transplantation. METHODS: Participated in this study 30 patients who were distributed in two groups: 15 healthy patients submitted to maxillofacial surgery, and 15 patients with chronic renal failure submitted to renal transplantation. All patients were anesthetized with etomidate, sufentanil and 0.5% to 1% sevoflurane . Intravenous cisatracurium was administrated after anesthetic induction and additional 0.05 mg.kg-1 was injected whenever T1 recovered 25%. Neuromuscular function was continuously monitored by acceleromyography using TOF stimulation, through supramaximal ulnar nerve stimulation. RESULTS: Onset time (4.1 and 4.9 min, clinical duration (68.9 and 75.4 min and recovery time (20.2 and 28 min were similar between normal and renal failure groups, respectively. Time spent until T4/T1 > 0.7 (34.3 and 51.4 min, and > 0.9 (49.7 and 68.6 min since the last 25% recovery of T1 were statistically different between groups, with the higher values observed in the renal failure group. Accumulation ratio was 1.08. CONCLUSIONS: Onset, clinical duration and recovery time were comparable between groups. Time to T4/T1 > 0.7 and > 0.9 was longer in the renal failure group as compared to the normal group and cisatracurium did not show cumulative effects in the renal failure group.

Impaired Glucose Metabolism Despite Decreased Insulin Resistance After Renal Transplantation

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Manfred Hecking

2012-06-01

Full Text Available The pathophysiology underlying new-onset diabetes after transplantation (NODAT is unresolved. We obtained demographics and laboratory data from all 1064 renal transplant recipients followed at our outpatient clinic in 2009/2010, randomly assigned 307 patients without previously diagnosed diabetes to a routine 2-hour oral glucose tolerance test (OGTT, and compared the metabolic results to a large, unrelated cross-sectional cohort of non-transplanted subjects. Among renal transplant recipients, 11% had a history of NODAT, and 12% had type 1 and type 2 diabetes. 42% of all OGTTs were abnormal (9% diabetic, predominantly in older patients who received tacrolimus. Compared to non-transplanted subjects, basal glucose was lower and HbA1c higher in renal transplant patients. Compared to non-transplanted subjects, insulin secretion was inferior, and insulin sensitivity improved at ≥6 months, as well as 3 months post-transplantation:(The Figure shows linear spline interpolation; all p for overall difference between non-Tx and Tx patients <0.02, using likelihood ratio testing. Our results indicate that impaired insulin secretion is the predominant problem after renal transplantation, suggesting benefit for therapeutic regimens that preserve beta cell function after renal transplantation. The mechanism of increased insulin sensitivity might be pathophysiologically similar to pancreatogenic diabetes.fx1

Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis.

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Weimer, Jill M; Benedict, Jared W; Getty, Amanda L; Pontikis, Charlie C; Lim, Ming J; Cooper, Jonathan D; Pearce, David A

2009-04-17

Juvenile neuronal ceroid lipofuscinosis (JNCL), or Batten disease, is a neurodegenerative disease resulting from a mutation in CLN3, which presents clinically with visual deterioration, seizures, motor impairments, cognitive decline, hallucinations, loss of circadian rhythm, and premature death in the late-twenties to early-thirties. Using a Cln3 null (Cln3(-/-)) mouse, we report here several deficits in the cerebellum in the absence of Cln3, including cell loss and early onset motor deficits. Surprisingly, early onset glial activation and selective neuronal loss within the mature fastigial pathway of the deep cerebellar nuclei (DCN), a region critical for balance and coordination, are seen in many regions of the Cln3(-/-) cerebellum. Additionally, there is a loss of Purkinje cells (PC) in regions of robust Bergmann glia activation in Cln3(-/-) mice and human JNCL post-mortem cerebellum. Moreover, the Cln3(-/-) cerebellum had a mis-regulation in granule cell proliferation and maintenance of PC dendritic arborization and spine density. Overall, this study defines a novel multi-faceted, early-onset cerebellar disruption in the Cln3 null brain, including glial activation, cell loss, and aberrant cell proliferation and differentiation. These early alterations in the maturation of the cerebellum could underlie some of the motor deficits and pathological changes seen in JNCL patients.

[Clinical picture of hemorrhagic fever with renal syndrome in Croatia].

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Kuzman, Ilija

2003-01-01

Among many viral hemorrhagic fevers, only hemorrhagic fever with renal syndrome (HFRS) occurs in Croatia. HFRS is a natural focus zoonosis with sudden onset, characterized by high fever and other clinical symptoms, renal insufficiency and hemorrhages. In Croatia, HFRS is caused by two types of hantaviruses--Puumala (PUU) and Dobrava (DOB). The basic pathologic and patophysiologic disorder in HFRS is capillary damage (vasculitis). Incubation of HFRS has not been precisely determined, it is most frequently around two weeks. The disease onset is usually abrupt. At the beginning, general symptoms include high fever and myalgias, especially in the lumbar region, and abdominal pain, as well as strong headaches, malaise and nausea, and often vomiting or diarrhea. In half of the patients respiratory symptoms occur. Later on, some patients may experience hypotension, oliguria and other signs of renal failure, and apart from petechial, severe hemorrhages may also occur in other organs. During typical clinical presentation of the disease, some characteristic symptoms are clearly distinguished in particular stages of the disease. Therefore, the course of HFRS is usually divided into five distinct stages (febrile, hypotensive, oliguric, polyuric and convalescent). Such a course of the disease is more commonly present in case of DOB virus than PUU virus infection. The febrile stage with sudden onset usually lasts from 3 to 7 days, when thrombocytopenia and hemoconcentration, as well as albuminuria and hematuria are almost always recorded. The hypotensive stage lasts from one to 2 days on an average and is characterized by lower blood pressure and signs of renal failure. The oliguric stage usually starts at the beginning of the second week of the disease, when extensive hemorrhage may occur and urea and creatinine reach their highest values. The oliguric stage is followed by the polyuric stage which can last for up to two weeks, and is characterized by excretion of a large

Pregnancy in renal transplant recipients.

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Bouattar, T; Hakim, H; Rhou, H; Benamar, L; Bayahia, R; Ouzeddoun, N

2009-06-01

Renal transplantation with a well-functioning graft leads to a rapid restoration of endocrine and sexual functions. The aim of this study was to examine our experience with pregnancies among renal transplant patients, particularly with regard to their impact on graft function. We analyzed 10 pregnancies in 7 renal transplant recipients for long-term graft outcomes in terms of clinical and biological data. The mean patient age was 28.5 +/- 4 years. They all received a living donor kidney. The time between transplantation and the onset of pregnancy was 33.4 +/- 23.2 months. Regarding the immunosuppressive therapy, all patients received steroids and cyclosporine; 4 patients received in addition azathioprine and 2 received mycophenolate mofetil that was changed at 1 month before conception to azathioprine. There was no significant difference between the serum creatinine before and during pregnancy. We did not observe any acute rejection episode. Pregnancy complications were preclampsia in 1 case, hypertension in 1 case, urinary tract infection in 2 cases, and anemia in 80% of patients during the third trimester. Premature rupture of membranes occurred in 1 case and preterm delivery in 2 cases. Two cases of neonatal death were registered. Cesarean section was performed in 50% of cases. The follow-up revealed 2 cases of chronic rejection. A multidisciplinary approach is necessary for pregnancy which generally occurs at 2 years after kidney transplantation.

Clinical features of children with enthesitis-related juvenile idiopathic arthritis / juvenile spondyloarthritis followed in a French tertiary care pediatric rheumatology centre.

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Goirand, Maxime; Breton, Sylvain; Chevallier, Frédéric; Duong, Ngoc-Phoi; Uettwiller, Florence; Melki, Isabelle; Mouy, Richard; Wouters, Carine; Bader-Meunier, Brigitte; Job-Deslandre, Chantal; Quartier, Pierre

2018-04-02

Childhood-onset spondyloarthropathies usually start with enthesitis and peripheral arthritis. However, axial disease may develop afterward. Patients are most often classified, following revised (Edmonton 2011) ILAR criteria, as enthesitis-related arthritis, psoriatic arthritis, or unclassified juvenile idiopathic arthritis, particularly in cases of psoriasis in the patient or a first-degree relative. In adults, peripheral spondyloarthritis is classified by ASAS criteria. We retrospectively studied patients with childhood-onset spondyloarthropathies followed for more than one year in our referral centre. We did not exclude patients with a personal or familial history of psoriasis. We included 114 patients followed between January 2008 and December 2015 for a median of 2.5 years (IQR = 2.3). Sixty-nine per-cent of patients fulfilled the revised ILAR classification criteria for enthesitis-related arthritis, and 92% the ASAS criteria for peripheral spondyolarthritis (p <  0.001). Axial disease and sacroiliitis were rare at disease onset. However, they appeared during follow-up in 63% and 47% of cases respectively, after a median disease duration of 2.6 (IC 95% [2.2-4.4]) and 5.3 years (IC 95% [4.1-7.7]), respectively. Multivariable analysis showed that familial history of spondyloarthritis was associated with the presence of sacroiliitis and active disease at the latest follow-up (OR = 3.61 [1.5-8.7], p <  0.01 and 2.98 [1.2-7.3], p = 0.02, respectively). Axial involvement developed in most patients within five years. Revised Edmonton criteria were less sensitive than ASAS criteria to classify patients as having childhood-onset spondyloarthropathies. The main risk factor for both sacroiliitis and persistent active disease was a familial history of spondyloarthritis.

Juvenile angiofibromer

DEFF Research Database (Denmark)

Thuesen, Anne Daugaard; Jakobsen, John; Nepper-Rasmussen, Jørgen

2005-01-01

Juvenile angiofibroma is a rare, benign, rich vascular tumor, and approximately one new case is diagnosed in Denmark each year. It sits in the foramen sphenopalatinum and occurs in boys from 14 to 25 years of age. The most frequent initial symptoms are nasal obstruction and epistaxis. Through...... the years, the treatment of juvenile angiofibroma has included many methods, including surgical excision, electrocoagulation, interstitial or external radiation therapy, cryosurgery, hormone administration and chemotherapy. Radiation, chemotherapy and surgery have proven to be the most effective treatments...

Hypoglycemic action of vitamin K1 protects against early-onset diabetic nephropathy in streptozotocin-induced rats.

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Sai Varsha, M K N; Raman, Thiagarajan; Manikandan, R; Dhanasekaran, G

2015-10-01

Vitamin K is a potent regulator of vascular dynamics and prevents vascular calcification. Vitamin K is increasingly being recognized for its antioxidant and antiinflammatory properties. Recently we demonstrated that vitamin K1 (5 mg/kg) protects against streptozotocin-induced type 1 diabetes and diabetic cataract. The aim of this study was to determine whether the hypoglycemic action of vitamin K1 could inhibit early-onset diabetic nephropathy in a streptozotocin-induced rat kidney. Male Wistar rats were administered with 35 mg/kg STZ and after 3 days were treated with vitamin K1 (5 mg/kg, twice a week) for 3 months. Blood glucose was monitored once a month. At the end of the study, animals were sacrificed and kidney was dissected out and analysed for free radicals, antioxidants, aldose reductase, membrane ATPases, histopathology evaluation and expression of pro- and anti-inflammatory cytokines. Urea, uric acid, creatinine, albumin and insulin levels were also estimated. Treatment of diabetic rats with vitamin K1 resulted in a decrease in blood glucose and prevented microalbuminuria. Vitamin K1 also reduced oxidative stress and protected renal physiology by modulating Ca(2+) and Na(+)/K(+)-ATPases. Vitamin K1 inhibited renal inflammation by reducing nuclear factor-κB and inducible nitric oxide synthase. Interleukin-10 levels were increased in renal tissues, suggesting the ability of vitamin K1 to trigger antiinflammatory state. The hypoglycemic action of vitamin K1 could have an indirect effect by inhibiting early-onset diabetic nephropathy triggered by high blood glucose. Vitamin K1 could be an important nutrient based interventional strategy for early onset diabetic nephropathy. Copyright © 2015 Elsevier Inc. All rights reserved.

Parenting and juvenile delinquency

NARCIS (Netherlands)

Hoeve, M.

2008-01-01

Juvenile delinquency is a noteworthy problem. This thesis addressed the association between parenting and juvenile delinquency by analyzing the concepts of parenting adopted in family research in relation to criminological concepts and measures of delinquent behavior. Four studies were conducted.

Juvenile Huntington's disease confirmed by genetic examination in twins Doença de Huntington juvenil confirmada por exame genético em gêmeas

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GILBERTO LEVY

1999-09-01

Full Text Available Early-onset Huntington's disease (HD occurs in approximately 10% of HD's cases. We report juvenile HD in phenotypically identical twins, evaluated by history, clinical and neurologic examination, mini-mental state examination, blood laboratory exams, cerebrospinal fluid examination, skull computed tomography, and genetic examination for HD. Patients had the akinetic-rigid variety (Westphal variant of the disease and paternal inheritance. The laboratory workup confirmed the clinical diagnosis of HD, which adds this report to the rare cases of HD in twins reported in the literature.Doença de Huntington (DH de início precoce ocorre em aproximadamente 10% dos casos de DH. Relatamos DH juvenil em gêmeas fenotipicamente idênticas, avaliadas por história, exames clínico e neurológico, mini-exame do estado mental, exames de sangue, exame do líquido cefalorraquidiano, tomografia computadorizada de crânio e exame genético para DH. As pacientes apresentavam a variedade rígido-acinética (variante de Westphal da doença e herança paterna. A avaliação laboratorial confirmou o diagnóstico clínico de DH, acrescentando-se este relato aos raros casos de DH em gêmeos relatados na literatura.

Juvenile delinquency and correctional treatment in Britain

OpenAIRE

堀尾, 良弘; ホリオ, ヨシヒロ; Yoshihiro, Horio

2006-01-01

Japanese modernistic culture is influenced not a little from Britain. In looking at the Juvenile Law and the history of correctional treatment in Britain, understanding of today's juvenile delinquency and treatment deepen. Moreover, the background and issue of juvenile delinquency in Britain are also discussed. As a feature of the juvenile delinquency in Britain, the common field with Japan and the field peculiar to Britain became clear in each. It is common to the world that the juvenile del...

Juvenile prison in parallel legislation

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Lutovac Mitar

2016-01-01

Full Text Available The need for punishment of juveniles occurred from the time when there was no clear line separating them from the adult criminal population. At the same time, the evolution of the juvenile punishment is not in itself involve substantial changes to their criminal status. On the contrary, the status of minors in society did not show serious differences regarding the status of young adults, as well as the adult elderly. On the other hand, on the ground of their punishment is recorded deviations that go in the direction of application of mild corporal punishment. Closing the minor was performed in a physically separate parts of the general penal institutions with the use of a lower degree of restrictions while serving juvenile prison. Due to the different treatment of minors during the evolution of their criminal status leads to their different treatment in comparative law. That is why we are witnessing the existence of numerous differences in the juvenile punishment in some countries in the world. On the European continent there is a wide range of different legal solutions when it comes to punishing juveniles. There are considerable differences in the procedure pronouncing juvenile prison and in particular penal treatment of juveniles in penitentiary institutions. For these reasons, the author has decided to show the basic statutory provisions in the part that relates to the issue of punishment of minors in the legislation of individual countries.

Bilateral, independent juvenile nasopharyngeal angiofibroma

DEFF Research Database (Denmark)

Mørkenborg, Marie-Louise; Frendø, M; Stavngaard, T

2015-01-01

BACKGROUND: Juvenile nasopharyngeal angiofibroma is a benign, vascular tumour that primarily occurs in adolescent males. Despite its benign nature, aggressive growth patterns can cause potential life-threatening complications. Juvenile nasopharyngeal angiofibroma is normally unilateral, originating...... from the sphenopalatine artery, but bilateral symptoms can occur if a large tumour extends to the contralateral side of the nasopharynx. This paper presents the first reported case of true bilateral extensive juvenile nasopharyngeal angiofibroma involving clinically challenging pre-surgical planning...... embolisation. Radical removal performed as one-step, computer-assisted functional endoscopic sinus surgery was performed. The follow-up period was uncomplicated. CONCLUSION: This case illustrates the importance of suspecting bilateral juvenile nasopharyngeal angiofibroma in patients presenting with bilateral...

Criminal Profiles of Violent Juvenile Sex and Violent Juvenile Non-Sex Offenders: An Explorative Longitudinal Study

Science.gov (United States)

van Wijk, Anton Ph.; Mali, Bas R. F.; Bullens, Ruud A. R.; Vermeiren, Robert R.

2007-01-01

Few studies have longitudinally investigated the criminal profiles of violent juvenile sex and violent juvenile non-sex offenders. To make up for this lack, this study used police records of juveniles to determine the nature of the criminal profiles of violent sex offenders (n = 226) and violent non-sex offenders (n = 4,130). All offenders…

Juvenile morphology in baleen whale phylogeny.

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Tsai, Cheng-Hsiu; Fordyce, R Ewan

2014-09-01

Phylogenetic reconstructions are sensitive to the influence of ontogeny on morphology. Here, we use foetal/neonatal specimens of known species of living baleen whales (Cetacea: Mysticeti) to show how juvenile morphology of extant species affects phylogenetic placement of the species. In one clade (sei whale, Balaenopteridae), the juvenile is distant from the usual phylogenetic position of adults, but in the other clade (pygmy right whale, Cetotheriidae), the juvenile is close to the adult. Different heterochronic processes at work in the studied species have different influences on juvenile morphology and on phylogenetic placement. This study helps to understand the relationship between evolutionary processes and phylogenetic patterns in baleen whale evolution and, more in general, between phylogeny and ontogeny; likewise, this study provides a proxy how to interpret the phylogeny when fossils that are immature individuals are included. Juvenile individuals in the peramorphic acceleration clades would produce misleading phylogenies, whereas juvenile individuals in the paedomorphic neoteny clades should still provide reliable phylogenetic signals.

Juvenile Sex Offending Through a Developmental Life Course Criminology Perspective.

Science.gov (United States)

Lussier, Patrick

2017-02-01

Current American policies and responses to juvenile sex offending have been criticized for being based on myths, misconceptions, and unsubstantiated claims. In spite of the criticism, no organizing framework has been proposed to guide policy development with respect to the prevention of juvenile sex offending. This article proposes a developmental life course (DLC) criminology perspective to investigate the origins, development, and termination of sex offending among youth. It also provides a review of the current state of knowledge regarding various parameters characterizing the development of sex offending (e.g., prevalence, age of onset, frequency, persistence, continuity in adulthood, and versatility). The review highlights some heterogeneity across these developmental parameters suggesting the presence of different sex offending patterns among youth. In fact, it is proposed that, based on the current knowledge, such heterogeneity can be accounted for by a dual taxonomy of adolescents involved in sexual offenses: (a) the adolescent-limited and (b) the high-rate/slow-desister. The DLC criminology approach and the dual taxonomy are proposed as organizing frameworks to conduct prospective longitudinal research to better understand the origins and development of sex offending and to guide policy development and responses to at-risk youth and those who have committed sexual offenses.

Larval, pre-juvenile and juvenile development of Diapterus peruvianus (Perciformes: Gerreidae

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Sylvia Patricia Adelheid Jiménez Rosenberg

2003-06-01

Full Text Available The development of Diapterus peruvianus (Sauvage 1879 is based on 60 larvae collected in superficial tows made in Bahía Concepción, and on 16 prejuvenile and juvenile organisms collected in Bahía de La Paz, B. C. S., México, using a standard plankton net and a rectangular epibenthonic net, respectively. Larvae of D. peruvianus show three large blotches on the dorsum of the gut that can fuse together and give the appearance of one large continuous blotch. There are two to three pre-anal pigments and 16 post-anal pigments in the ventral midline; cephalic pigments are present from the postflexion stage, as well as a serrated preoperculum. The prejuvenile and juvenile organisms are distinguished by their body depth, the analfin formula, the serrated preoperculum and the base pigments in the dorsal and anal fins.El desarrollo de Diapterus peruvianus se analizó con base en 60 larvas recolectadas en Bahía Concepción y 16 pre-juveniles y juveniles recolectados en la Ensenada de La Paz, B. C. S. México, usando respectivamente, una red estándar de plancton en arrastres superficiales y una red epibentónica para arrastres de plancton. Las larvas presentan desde la pre-flexión tres manchas alargadas sobre la superficie dorsal de la masa visceral, que pueden unirse y dar apariencia de pigmentación continua, observándose hasta 16 pigmentos post-anales en la línea media ventral y de dos a tres pigmentos pre-anales; la pigmentación cefálica así como la forma aserrada del pre-opérculo característica del género, aparecen a partir de la post-flexión. Los organismos pre-juveniles y juveniles se distinguen por la profundidad del cuerpo, la fórmula de la aleta anal, la fina forma aserrada del pre-opérculo y la pigmentación en la base de las aletas dorsal y anal.

Adult-onset hypophosphatemic osteomalacia associated with Sjogren syndrome

Science.gov (United States)

Shen, Guohua; Zhang, Yuwei; Hu, Shuang; Liu, Bin; Kuang, Anren

2017-01-01

Abstract Rationale: Hypophosphatemic osteomalacia (HO) is a metabolic bone disease, exhibiting different etiologies such as genetic mutation, tumor induction, dysimmunity, or renal disease. Sjogren's syndrome (SS) is a connective tissue disorder commonly involving exocrine glands; however kidney involvement is also encountered, leading to abnormal phosphorus metabolism, even HO. Patient concerns: A 47-year-old female patient presented progressively worsening pain in the chest wall, back and bilateral lower extremities as well as muscle weakness was referred to our department. Diagnoses, interventions and outcomes: Due to the laboratory test results, radiographic findings and pathologic results, she was diagnosed with adult-onset HO associated with SS. She was then treated with alkalinization, steroids, neutral phosphate, calcium supplements together with activated vitamin D. So far, she recovered uneventfully with relieved pain and increased serum phosphorus level. Lessons: HO may be secondary to renal tubular acidosis of SS patients, and it might be a diagnostic challenge when the kidney involvement in SS is latent and precede the typical sicca symptoms. PMID:28353596

Radiographic imaging study of pneumocystis carinii pneumonia in renal transplantation recipient

International Nuclear Information System (INIS)

Chen Chengshui; Li Yuping; Ye Min; Zhang Dongqing; Zheng Shaoling; Xing Lingling; Chen Shaoxian

2005-01-01

Objective: To improve the understanding of the imaging features of pneumocystis carinii pneumonia (PCP) in renal transplantation recipient. Methods: Twenty-four renal transplantation recipients suffered from PCP. There were 19 males and 5 females, the age ranged from 23 to 62 years (mean 39.2 years). The mean time duration from renal transplantation to onset of illness was 5.6 months, and the mean time from onset of illness to consultation was 5.5 days. All patients had fever and dyspnea. The chest radiographic imaging was reviewed and the dynamic imaging changes were followed up. Results: Pathology showed alveolar exudation, inflammation in the interstitium and alveolar lumen, fibrosis in lung interstitium, and erosion of alveolar epithelium. Initial chest X-ray demonstrated diffuse changes in only 10 patients. Of the 10 patients, 3 showed ground-glass changes, 2 showed ground-glass and reticular changes, and 4 showed consolidation. But all patients had abnormal ill-defined ground-glass findings on thoracic CT images, 9 of them showed reticulum among ground-glass changes, and 12 of them showed consolidation among ground-glass changes. Among patients with clinical deterioration, chest radiographs and CT showed progression of pulmonary infiltrations, and it reached the top level within 1 to 2 weeks. With successful response to therapy, chest radiographs and CT showed resolution of the lung opacities, but the resolution was retarded for about 1 week, complete resolution would need 4 weeks. Conclusion: The radiographic imaging features of PCP in renal transplantation recipient were diffuse interstitial alterations and consolidations, and with fast progression. With successful response to therapy, it showed resolution of the lung opacities, but the resolution was retarded for about 1 week, and complete resolution would need 4 weeks. Chest CT was more sensitive than radiographs. (authors)

Juvenile Residential Facility Census, 2010: Selected Findings. Juvenile Offenders and Victims: National Report Series. Bulletin NCJ 241134

Science.gov (United States)

Hockenberry, Sarah; Sickmund, Melissa; Sladky, Anthony

2013-01-01

This bulletin is part of the "Juvenile Offenders and Victims National Report Series." The "National Report" offers a comprehensive statistical overview of the problems of juvenile crime, violence, and victimization and the response of the juvenile justice system. During each interim year, the bulletins in the "National…

The Evidence-Based Approach to Adult-Onset Idiopathic Nephrotic Syndrome.

Science.gov (United States)

Canetta, Pietro A A; Radhakrishnan, Jai

2015-01-01

Adult-onset nephrotic syndrome (NS) differs from its pediatric counterpart in several important ways. Most importantly, NS in adults is more etiologically heterogeneous compared to children, and thus treatment approaches rely heavily on the histological diagnosis provided by renal biopsy. The evidence-based approach to treatment of adult NS has been critically examined by the Kidney Disease Improving Global Outcomes (KDIGO) guidelines in glomerulonephritis, published in 2012. Here, we examine the strengths and limits of those guidelines and review recent work that expands the evidence-based approach.

Juvenile Confinement in Context

Science.gov (United States)

Mendel, Richard A.

2012-01-01

For more than a century, the predominant strategy for the treatment and punishment of serious and sometimes not-so-serious juvenile offenders in the United States has been placement into large juvenile corrections institutions, alternatively known as training schools, reformatories, or youth corrections centers. America's heavy reliance on…

The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies.

Science.gov (United States)

Rider, Lisa G; Shah, Mona; Mamyrova, Gulnara; Huber, Adam M; Rice, Madeline Murguia; Targoff, Ira N; Miller, Frederick W

2013-07-01

frequencies of black race, severe onset, distal weakness, falling episodes, Raynaud phenomenon, cardiac involvement, high CK levels, chronic disease course, frequent hospitalization, and wheelchair use. Characteristic features of the anti-Mi-2 subgroup included Hispanic ethnicity, classic dermatomyositis and malar rashes, high CK levels, and very low mortality. Finally, the most common features of patients without any currently defined MSA or myositis-associated autoantibodies included linear extensor erythema, arthralgia, and a monocyclic disease course. Several demographic and clinical features were shared between juvenile and adult idiopathic inflammatory myopathy subgroups, but with several important differences. We conclude that juvenile myositis is a heterogeneous group of illnesses with distinct autoantibody phenotypes defined by varying clinical and demographic characteristics, laboratory features, and outcomes.

Juvenile Angiofibroma: Evolution of Management

Science.gov (United States)

Nicolai, Piero; Schreiber, Alberto; Bolzoni Villaret, Andrea

2012-01-01

Juvenile angiofibroma is a rare benign lesion originating from the pterygopalatine fossa with distinctive epidemiologic features and growth patterns. The typical patient is an adolescent male with a clinical history of recurrent epistaxis and nasal obstruction. Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal treatment for juvenile angiofibroma. Refinement in preoperative embolization has provided significant reduction of complications and intraoperative bleeding with minimal risk of residual disease. During the last decade, an endoscopic technique has been extensively adopted as a valid alternative to external approaches in the management of small-intermediate size juvenile angiofibromas. Herein, we review the evolution in the management of juvenile angiofibroma with particular reference to recent advances in diagnosis and treatment. PMID:22164185

Juvenile Angiofibroma: Evolution of Management

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Piero Nicolai

2012-01-01

Full Text Available Juvenile angiofibroma is a rare benign lesion originating from the pterygopalatine fossa with distinctive epidemiologic features and growth patterns. The typical patient is an adolescent male with a clinical history of recurrent epistaxis and nasal obstruction. Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal treatment for juvenile angiofibroma. Refinement in preoperative embolization has provided significant reduction of complications and intraoperative bleeding with minimal risk of residual disease. During the last decade, an endoscopic technique has been extensively adopted as a valid alternative to external approaches in the management of small-intermediate size juvenile angiofibromas. Herein, we review the evolution in the management of juvenile angiofibroma with particular reference to recent advances in diagnosis and treatment.

Diagnostic utility of magnetic resonance imaging and radiography in juvenile spondyloarthritis: evaluation of the sacroiliac joints in controls and affected subjects.

Science.gov (United States)

Jaremko, Jacob L; Liu, Lei; Winn, Naomi J; Ellsworth, Janet E; Lambert, Robert G W

2014-05-01

To compare the utility of radiography and magnetic resonance imaging (MRI) for the diagnosis of juvenile-onset spondyloarthritis in pediatric patients presenting with low back and/or sacroiliac (SI) pain of potentially inflammatory etiology. Radiographs and MRI studies of the SI joints in 26 patients with juvenile spondyloarthritis (JSpA) and 35 controls were assessed independently by 2 radiologists, with discrepancies arbitrated by a third. Radiographs and MRI were blinded and read in separate batches in random order. Erosion was common and was the most useful diagnostic feature on radiography [positive likelihood ratio (LR) = 3.5] and was especially diagnostic of SpA on MRI (LR = 6.7). Subchondral sclerosis was common but was the least specific feature for both modalities. Joint space narrowing had some utility on radiography (LR = 2.0) and MRI (LR = 2.7) but was uncommon and had poor reader reliability. Bone marrow edema (LR = 3.1) and subarticular fat infiltration (LR = 4.5), detectable only on MRI, were both useful features. Global diagnostic impression of MRI (LR = 9.4) had very high utility for the diagnosis of JSpA, exceeding radiography (LR = 4.4) because of superior specificity. In addition, global diagnosis of SpA is much more reliably made on MRI (κ = 0.80) compared to radiography (κ = 0.30). Specificity and reliability of MRI of the SI joints are superior to radiography for the diagnosis of juvenile-onset SpA and, where available, MRI should replace radiography as the first line of investigation.

Psoriasis and associated variables in classification and outcome of juvenile idiopathic arthritis - an eight-year follow-up study

DEFF Research Database (Denmark)

Ekelund, Maria; Aalto, Kristiina; Fasth, Anders

2017-01-01

BACKGROUND: To study the impact of psoriasis and features associated with psoriasis on classification and outcome in a population-based follow-up cohort of children with juvenile idiopathic arthritis (JIA). METHODS: In all, 440 children with JIA were followed for a median of 8 years...... in a prospective Nordic population-based cohort study. Data for remission was available for 427 of these children. The presence of psoriasis, psoriasis-like rash, dactylitis, nail pitting, enthesitis, tenosynovitis and heredity was assessed in relation to ILAR classification and remission. RESULTS: Clinical...... findings associated with psoriasis developed consecutively during the 8-year period. Six of 14 children with psoriasis were not classified as juvenile psoriatic arthritis according to the ILAR criteria at 8 year follow-up. Dactylitis was more common in children with early onset of JIA. After 8 years we...

Is basiliximab induction, a novel risk factor for new onset diabetes after transplantation for living donor renal allograft recipients?

Science.gov (United States)

Prasad, Narayan; Gurjer, Desraj; Bhadauria, Dharmender; Gupta, Amit; Srivastava, Aneesh; Kaul, Anupama; Jaiswal, Akhilesh; Yadav, Brijesh; Yadav, Subhash; Sharma, Raj K

2014-04-01

It was found that, by affecting populations of T lymphocytes and regulatory T cells, basiliximab also indirectly affects pancreatic β-cell function and glucose homeostasis. In this prospective observational study, we included all renal transplant recipients from 1 July 2007 to 31 July 2011. The overall incidence of hyperglycaemia (transient hyperglycaemia, impaired fasting glucose (IFG), impaired glucose tolerance (IGT) and new onset diabetes after transplantation (NODAT)) was compared between patients with and without basiliximab induction. Of the 439 eligible study patients, 105 patients received basiliximab induction and 334 patients did not. Overall hyperglycaemia (transient hyperglycaemia, IFG, IGT and NODAT) was detected in 102/334 (30.5%) patients without induction and 44/105 (41.9%) patients with induction (P = 0.03). Of the 102 patients with hyperglycaemia in patients without basiliximab, 46 (45.1%) patients improved, while only 10 (22.7%) of the 44 patients with basiliximab improved (P = 0.016) at the end of 3 months. Finally, NODAT was observed in 56/334 (16.7%) patients without induction and 102/334 (30.5%) patients with induction. Relative risk of NODAT with basiliximab was 2.3 (95% CI 1.4-3.9) compared to that of patients without induction. Basiliximab and hepatitis C virus infection were independent risk factors for NODAT. Risk of NODAT remained high with basiliximab despite adjusting the acute rejections episodes. Basiliximab induction prevents acute rejection; however, it is associated with increased risk of NODAT. © 2014 Asian Pacific Society of Nephrology.

Comparison of the Protective Effects of Erythropoietin and Melatonin on Renal Ischemia-Reperfusion Injury.

Science.gov (United States)

Banaei, Shokofeh; Ahmadiasl, Nasser; Alihemmati, Alireza

2016-07-01

Renal ischemia-reperfusion (IR) contributes to the development of acute renal failure (ARF). Oxygen free radicals are considered to be the principal components involved in the pathophysiological tissue alterations observed during renal IR. In this study, we compared the effects of melatonin (MEL) and erythropoietin (EPO), both known antioxidant and anti-inflammatory agents, on IR-induced renal injury in rats. Wistar albino rats were unilaterally nephrectomized and then subjected to 45 minutes of renal pedicle occlusion followed by 24 hours of reperfusion. MEL (10 mg/kg, i.p) and EPO (5000 U/kg, i.p) were administered prior to the onset of ischemia. After 24 hours of reperfusion and following decapitation, blood samples were collected for the determination of the hemoglobin (Hb) and hematocrit (Hct) levels. Additionally, renal samples were taken for histological evaluation. Ischemia-reperfusion significantly decreased the observed Hb and Hct values. The histopathological findings in the IR group confirmed that there was an increase in the hyaline cast and thickening of the Bowman capsule basement membrane. Treatment with EPO or MEL significantly increased the Hb and Hct values. In the MEL + IR group, the histopathological changes were lower than those found in the EPO + IR group. Treatment with EPO and MEL had a beneficial effect on renal IR injury. The results may also indicate that MEL protects against morphological damage better than EPO in renal IR injury.

Epidemic juvenile hypothyroidism among a population of Hanford 'downwinders'

Energy Technology Data Exchange (ETDEWEB)

Grossman, C.M.; Nussbaum, R.H.; Nussbaum, F.D. [Northwest Radiation Health Alliance, Portland, OR (United States); Kordysh, E.; Goldsmith, J.R. [Dept. of Epidemiology, Ben Gurion Univ. of the Negev, Beer Sheva (Israel)

2001-07-01

Self-defined 'downwinders' responded to questionnaires as to their health and their potential exposures to emissions from the Hanford Nuclear Facility in the U.S. The area considered was between the Cascades and the Rocky Mountains, primarily to the East and Northeast of the facility. Of 801 questionnaires, returned, 518 were from women. Of these, 210 reported that they had been diagnosed by their physicians as having hypothyroidism. 31 of the 283 men had reported being diagnosed as hypothyroid. The condition is generally found to be frequent among adult women, but is uncommon in children. 54 of the 210 cases among females were diagnosed among those less than 20 years of age, and 5 out of 31 males were found in this age range. Peak years for diagnosis among females were from 1945 to 1965, with only three juvenile cases occurring after 1970 among 80 female hypothyroid cases overall. Among males there were five juvenile cases in 1955-1960 out of 7 overall, but no juvenile cases among 24 cases at other times. The timing of the onset of this epidemic follows closely after the massive release of I{sup 131} from the facility in 1945, so that a causal relationship is strongly suggested. The counties of residence of cases corresponds to the counties with high cumulative dose estimates in children's thyroid glands. Validation of the diagnostic criteria was checked in a sample of cases. Other types of thyroid abnormalities were also increased. One case of juvenile thyroid cancer was reported. While thyroid cancer in adults was unusually frequent, the first cases occurred in 1955 and the highest number (8) in 1985. Our findings are consistent with the clinical finding that hypothyroidism in children frequently follows radiation of the head and neck. Similarities with the Marshall Islanders exposed to the nuclear test, 'Bravo' and to those exposed near Chernobyl are discussed. (orig.)

Renal artery stenosis after radiotherapy for Ewing's sarcoma

International Nuclear Information System (INIS)

Tacconi, S.; Bieri, S.

2008-01-01

Background: the fact that therapeutic irradiation can induce significant stenosis in the arteries of the head, neck, and chest, as welt as in the aorta and the iliac arteries, is familiar in daily practice and well documented in the literature. By contrast, radiation-induced renal artery stenosis seems to be a less widely known complication. Patients and methods: the sudden onset of medically refractory arterial hypertension and coma in a 27-year-old man is reported, who had been treated at age 20 with chemotherapy and radiotherapy for Ewing's sarcoma in the lumbar region. This treatment had been performed at the hospital of Sion, Switzerland in 2001. Also, the relevant literature from 1965 to 2007 is reviewed to underscore various aspects of this problem and to demonstrate the clinical relevance of renal artery stenosis as a potential long-term sequela of radiotherapy. Conclusion: radiation-induced renal artery stenosis has only rarely been described in the literature, but arterial hypertension due to radiation-induced renal artery stenosis is a serious long-term sequela that can appear at a latency of up to 20 years after treatment. The paucity of reports presumably reflects the lesser frequency of radiotherapy for retroperitoneal tumors as compared to head-and-neck cancers, as well as lower awareness of the problem due to diagnostic bias in the era before CT and MRI were in routine use: at that time, carotid artery stenosis was easy to diagnose by ultrasonography, while radiation-induced renal artery stenosis, whose real incidence may well be higher, probably often went undetected. Thus, when a patient with a history of abdominal or retroperitoneal radiotherapy unexpectedly develops intractable hypertension, radiation-induced renal artery stenosis must be included in the differential diagnosis. (orig.)

Juvenile polyposis syndrome

OpenAIRE

Hsiao, Yi-Han; Wei, Chin-Hung; Chang, Szu-Wen; Chang, Lung; Fu, Yu-Wei; Lee, Hung-Chang; Liu, Hsuan-Liang; Yeung, Chun-Yan

2016-01-01

Abstract Background: Juvenile polyposis syndrome, a rare disorder in children, is characterized with multiple hamartomatous polyps in alimentary tract. A variety of manifestations include bleeding, intussusception, or polyp prolapse. In this study, we present an 8-month-old male infant of juvenile polyposis syndrome initially presenting with chronic anemia. To the best of our knowledge, this is the youngest case reported in the literature. Methods: We report a rare case of an 8-month-old male...

The evidence-based approach to adult-onset idiopathic nephrotic syndrome

Directory of Open Access Journals (Sweden)

Pietro A. Canetta

2015-09-01

Full Text Available Adult-onset nephrotic syndrome differs from its pediatric counterpart in several important ways. Most importantly, nephrotic syndrome in adults is more etiologically heterogeneous compared to children, and thus treatment approaches rely heavily on the histologic diagnosis provided by renal biopsy. The evidence-based approach to treatment of adult nephrotic syndrome has been critically examined by the Kidney Disease Improving Global Outcomes (KDIGO guidelines in glomerulonephritis, published in 2012. Here, we examine the strengths and limits of those guidelines and review recent work that expands the evidence-based approach.

Novel exonic mutation inducing aberrant splicing in the IL10RA gene and resulting in infantile-onset inflammatory bowel disease: a case report.

Science.gov (United States)

Yanagi, Tadahiro; Mizuochi, Tatsuki; Takaki, Yugo; Eda, Keisuke; Mitsuyama, Keiichi; Ishimura, Masataka; Takada, Hidetoshi; Shouval, Dror S; Griffith, Alexandra E; Snapper, Scott B; Yamashita, Yushiro; Yamamoto, Ken

2016-01-28

Although deleterious mutations in interleukin-10 and its receptor molecules cause severe infantile-onset inflammatory bowel disease, there are no reports of mutations affecting this signaling pathway in Japanese patients. Here we report a novel exonic mutation in the IL10RA gene that caused unique splicing aberrations in a Japanese patient with infantile-onset of inflammatory bowel disease in association with immune thrombocytopenic purpura and a transient clinical syndrome mimicking juvenile myelomonocytic leukemia. A Japanese boy, who was the first child of non-consanguineous healthy parents, developed bloody diarrhea, perianal fistula, and folliculitis in early infancy and was diagnosed with inflammatory bowel disease. He also developed immune thrombocytopenic purpura and transient features mimicking juvenile myelomonocytic leukemia. The patient failed to respond to various treatments, including elemental diet, salazosulfapyridine, metronidazole, corticosteroid, infliximab, and adalimumab. We identified a novel mutation (c.537G > A, p.T179T) in exon 4 of the IL10RA gene causing unique splicing aberrations and resulting in lack of signaling through the interleukin-10 receptor. At 21 months of age, the patient underwent allogeneic hematopoietic stem cell transplantation and achieved clinical remission. We describe a novel exonic mutation in the IL10RA gene resulting in infantile-onset inflammatory bowel disease. This mutation might also be involved in his early-onset hematologic disorders. Physicians should be familiar with the clinical phenotype of IL-10 signaling defects in order to enable prompt diagnosis at an early age and referral for allogeneic hematopoietic stem cell transplantation.

sup(99m)Tc-DMSA renal scintigraphy in renal failure due to various renal diseases

Energy Technology Data Exchange (ETDEWEB)

Hosokawa, S; Daijo, K; Okabe, T; Kawamura, J; Hara, A [Kyoto Univ. (Japan). Hospital

1979-08-01

Renal contours in renal failure were studied by means of sup(99m)Tc-dimercaptosuccinic acid (DMSA) renoscintigraphy. Renal cortical images were obtained even in renal failure cases. Causes of renal failure were chronic glomerulonephritis in 7, bilateral renal tuberculosis in 2, chronic pyelonephritis in 3, bilateral renal calculi in 3, diabetic nephropathy in 2, polycystic kidney disease in 2 and stomach cancer in 1.

sup(99m)Tc-DMSA renal scintigraphy in renal failure due to various renal diseases

International Nuclear Information System (INIS)

Hosokawa, Shin-ichi; Daijo, Kazuyuki; Okabe, Tatsushiro; Kawamura, Juichi; Hara, Akira

1979-01-01

Renal contours in renal failure were studied by means of sup(99m)Tc-dimercaptosuccinic acid (DMSA) renoscintigraphy. Renal cortical images were obtained even in renal failure cases. Causes of renal failure were chronic glomerulonephritis in 7, bilateral renal tuberculosis in 2, chronic pyelonephritis in 3, bilateral renal calculi in 3, diabetic nephropathy in 2, polycystic kidney disease in 2 and stomach cancer in 1. (author)

IgG4-related disease: description of a case with pulmonary lesions, mediastinal lymphadenopathies and rapidly progressive renal failure

Directory of Open Access Journals (Sweden)

Loreto Fernández Lorente

2015-03-01

Full Text Available This is a case report of a 73-year-old man with new-onset acute renal failure while being investigated for pulmonary infiltrates and mediastinal lymphadenopathies. Urine tests showed tubular range proteinuria with no microhaematuria. Immunology tests showed elevated serum IgG and hypocomplementaemia (classical pathway activation. Renal biopsy and clinical-pathological correlation were crucial in this case, reinforcing their important role in the final diagnosis of acute kidney injury.

Social and psychological aspects of criminal juvenile justice in the world practice (Anglo-Saxon model of juvenile justice

Directory of Open Access Journals (Sweden)

D.S. Oshevsky

2013-10-01

Full Text Available The article is the final part of the review of existing foreign models of juvenile criminal justice system. We analyze the principles of juvenile justice in the criminal trial: protective orientation, personalization and social richness of the trial, the emphasis on educational influences. We present the foreign experience of incorporating social, psychological and clinical special knowledge into specialized justice concerning juvenile offenders. We analyze modern trends in the development of juvenile justice in the United States and Canada. We present material related to methods of risk assessment of re-offending among adolescents. We highlight approaches to complex long-term follow-up of juvenile offenders in Anglo-Saxon juvenile justice. We describe some aspects of the probation service using the method of case management. In the context of the accepted “National Strategy for Action for the Benefit of Children for 2012-2017”, the prospects for the development of specialized criminal justice for young offenders in the Russian Federation are discussed

Juvenile polyposis syndrome

Science.gov (United States)

Hsiao, Yi-Han; Wei, Chin-Hung; Chang, Szu-Wen; Chang, Lung; Fu, Yu-Wei; Lee, Hung-Chang; Liu, Hsuan-Liang; Yeung, Chun-Yan

2016-01-01

Abstract Background: Juvenile polyposis syndrome, a rare disorder in children, is characterized with multiple hamartomatous polyps in alimentary tract. A variety of manifestations include bleeding, intussusception, or polyp prolapse. In this study, we present an 8-month-old male infant of juvenile polyposis syndrome initially presenting with chronic anemia. To the best of our knowledge, this is the youngest case reported in the literature. Methods: We report a rare case of an 8-month-old male infant who presented with chronic anemia and gastrointestinal bleeding initially. Panendoscopy and abdominal computed tomography showed multiple polyposis throughout the entire alimentary tract leading to intussusception. Technetium-99m-labeled red blood cell (RBC) bleeding scan revealed the possibility of gastrointestinal tract bleeding in the jejunum. Histopathological examination on biopsy samples showed Peutz-Jeghers syndrome was excluded, whereas the diagnosis of juvenile polyposis syndrome was established. Results: Enteroscopic polypectomy is the mainstay of the treatment. However, polyps recurred and occupied the majority of the gastrointestinal tract in 6 months. Supportive management was given. The patient expired for severe sepsis at the age of 18 months. Conclusion: Juvenile polyposis syndrome is an inherited disease, so it is not possible to prevent it. Concerning of its poor outcome and high mortality rate, it is important that we should increase awareness and education of the parents at its earliest stages. PMID:27631205

Acupuntura em adolescentes com fibromialgia juvenil Acupuntura en adolescentes con fibromialgia juvenil Acupuncture in adolescents with juvenile fibromyalgia

Directory of Open Access Journals (Sweden)

Marialda Höfling P. Dias

2012-01-01

Full Text Available OBJETIVO: Descrever a utilização da acupuntura em adolescentes com fibromialgia juvenil. MÉTODOS: Estudo retrospectivo realizado em pacientes com fibromialgia juvenil (critérios do Colégio Americano de Reumatologia submetidos a, pelo menos, 11 sessões semanais de acupuntura. As avaliações antes e após acupuntura incluíram dados demográficos, características da dor musculoesquelética, número de pontos dolorosos (NPD, escala visual analógica (EVA de dor, algiometria e índice miálgico (IM. Durante o estudo, os pacientes puderam usar analgésicos, amitriptilina e foram orientados a praticar atividade física aeróbica. Os resultados antes e após acupuntura foram comparados pelo teste não paramétrico de Wilcoxon. RESULTADOS: Dos 38 pacientes com fibromialgia juvenil acompanhados em oito anos consecutivos, 13 tinham todas as informações nos prontuários e nas fichas de acupuntura e foram avaliados. Destes 13, sete obtiveram melhora nos três parâmetros analisados (número de pontos dolorosos, EVA de dor e IM. As medianas do número de pontos dolorosos e da EVA de dor foram significativamente maiores antes do tratamento quando comparados ao final do tratamento com as sessões de acupuntura [14 (11-18 versus 10 (0-15, p=0,005; 6 (2-10 versus 3 (0-10, p=0,045; respectivamente]. Em contraste, a mediana do IM foi significativamente menor antes do tratamento [3,4 (2,49-4,39 versus 4,2 (2,71-5,99, p=0,02]. Nenhum dos pacientes com fibromialgia juvenil apresentou eventos adversos associados à acupuntura. CONCLUSÕES: Acupuntura é uma modalidade de Medicina Tradicional Chinesa que pode ser utilizada nos pacientes pediátricos com fibromialgia. Futuros estudos controlados serão necessários.OBJETIVO: Describir el uso de acupuntura en adolescentes con fibromialgia juvenil. MÉTODOS: Estudio retrospectivo realizado en pacientes con fibromialgia juvenil (criterios del Colegio Americano de Reumatología sometidos a al menos 11 sesiones

Temporomandibular joint involvement in juvenile idiopathic arthritis: clinical predictors of magnetic resonance imaging signs

Energy Technology Data Exchange (ETDEWEB)

Argyropoulou, Maria I.; Margariti, Persefoni N.; Astrakas, Loukas; Kosta, Paraskevi [Medical School University of Ioannina, Department of Radiology, Ioannina (Greece); Karali, Aikaterini; Alfandaki, Sapfo; Siamopoulou, Antigoni [University of Ioannina, Department of Child Health, Medical School, Ioannina (Greece)

2009-03-15

The aim of the study was to define clinical predictors of magnetic resonance imaging (MRI) findings of temporomandibular joint (TMJ) involvement in juvenile idiopathic arthritis (JIA). Forty-six patients, aged 2.08-36.7 years, with JIA (oligoartitular 18, polyarticular 17, systemic type 11) were examined with standard plain and contrast-enhanced sequences. Of 88 TMJs examined, an abnormal condyle was observed in 32%, flattened articular eminence in 27%, flattened articular disk in 17%, intra-articular fluid in 10%, enhancing pannus in 45% and restricted condylar motion in 9%. Logistic regression analysis revealed that for abnormal condyle and flattened articular eminence, independent predictors were type of JIA (P < 0.015), age at onset (P < 0.038), and duration of disease activity (P < 0.001). Plots of the logistic regression models showed that TMJ involvement approached certainty for systemic sooner than for the other JIA types. Pannus was present with probability >0.5 when the disease started before 4 years of age. In conclusion, the systemic type of JIA, young age at onset and long duration of activity are risk factors for TMJ damage. MRI of the TMJ should be performed in patients who are less than 4 years of age at the onset of JIA, and in those with the systemic type, whatever the age of onset. (orig.)

Can preeclampsia be considered a renal compartment syndrome? A hypothesis and analysis of the literature.

Science.gov (United States)

Reuter, David G; Law, Yuk; Levy, Wayne C; Seslar, Stephen P; Zierler, R Eugene; Ferguson, Mark; Chattra, James; McQuinn, Tim; Liu, Lenna L; Terry, Mark; Coffey, Patricia S; Dimer, Jane A; Hanevold, Coral; Flynn, Joseph T; Stapleton, F Bruder

2016-11-01

The morbidity and mortality associated with preeclampsia is staggering. The physiology of the Page kidney, a condition in which increased intrarenal pressure causes hypertension, appears to provide a unifying framework to explain the complex pathophysiology. Page kidney hypertension is renin-mediated acutely and ischemia-mediated chronically. Renal venous outflow obstruction also causes a Page kidney phenomenon, providing a hypothesis for the increased vulnerability of a subset of women who have what we are hypothesizing is a "renal compartment syndrome" due to inadequate ipsilateral collateral renal venous circulation consistent with well-known variation in normal venous anatomy. Dynamic changes in renal venous anatomy and physiology in pregnancy appear to correlate with disease onset, severity, and recurrence. Since maternal recumbent position is well known to affect renal perfusion and since chronic outflow obstruction makes women vulnerable to the ischemic/inflammatory sequelae, heightened awareness of renal compartment syndrome physiology is critical. The anatomic and physiologic insights provide immediate strategies to predict and prevent preeclampsia with straightforward, low-cost interventions that make renewed global advocacy for pregnant women a realistic goal. Copyright © 2016 American Society of Hypertension. Published by Elsevier Inc. All rights reserved.

Angiographic Findings and Embolotherapy in Renal Arterial Trauma

International Nuclear Information System (INIS)

Sofocleous, Constantinos T.; Hinrichs, Clay; Hubbi, Basil; Brountzos, Elias; Kaul, Sanjeev; Kannarkat, George; Bahramipour, Philip; Barone, Alison; Contractor, Daniel G.; Shah, Tanmaya

2005-01-01

from 1 month to 7 years (mean 31 months). No procedure-related or delayed onset of renal insufficiency occurred.Conclusion In hemodynamically stable and controlled patients selective and superselective embolization is a safe and effective method for the management of renal vascular injury

The importance of an ophthalmologic examination in patients with juvenile idiopathic arthritis.

Science.gov (United States)

Rodríguez-García, Alejandro

2015-01-01

Uveitis occurs within the first year of arthritis onset in 73% of patients with juvenile idiopathic arthritis (JIA) considered at risk. The intraocular inflammation is characterized by an insidious onset and a silent and chronic clinical course capable of producing significant visual loss due to complications such as: cataract formation, secondary glaucoma, maculopathy and optic neuropathy. The absence of initial signs and symptoms, along with a deficient ophthalmic monitoring produce a delay in diagnosis with serious consequences. It has been estimated that 47% of JIA patients at risk for developing uveitis are legally blind (20/200 or worse) at least in one eye at the time of their first visit to the ophthalmologist. To reduce ocular complications and improve their visual outcome, it is necessary that rheumatologists refer all patients recently diagnosed (within the first month) with JIA for an ophthalmic evaluation, and maintain periodical follow-up visits based on classification and risk category of the disease. Copyright © 2014 Elsevier España, S.L.U. All rights reserved.

Renal computed angiography. Part I: Renal CT phlebography. Renal veins variants

International Nuclear Information System (INIS)

Al-Amin, M.; Krupev, M.; Hadjidekov, V.; Plachkov, I.

2012-01-01

The changing trend in renal surgery, transplantation and minimal invasive urology implies preprocedure evaluation of renal veins. Development of imaging methods offers new possibilities for venographic visualization. The goal of this study is to present authors experience in visualization of renal veins using 64 MDCT and to evaluate the utility in assessments of their variants. 128 patients (68 females and 60 males, mean age 53,3) with urological complaints underwent 64MDCT examination including CT angiography. Contrast enhancement includes 3-4ml/sec injection flow of 90 ml contrast medium followed by 20 ml saline at the same rate. In 23 out of 128 examined patients some of the common variants of the renal vein is found. 64 MDCT angiography visualize very well renal veins and becomes method of choice in preoperative assessment of renal vein anatomy. (authors)

Late-onset acute rejection after living donor liver transplantation

Institute of Scientific and Technical Information of China (English)

Nobuhisa Akamatsu; Yasuhiko Sugawara; Sumihito Tamura; Junichi Keneko; Yuichi Matsui; Kiyoshi Hasegawa; Masatoshi Makuuchi

2006-01-01

AIM: To investigate the incidence and risk factors of late-onset acute rejection (LAR) and to clarify the effectiveness of our immunosuppressive regime consisting of life-long administration of tacrolimus and steroids.METHODS: Adult living donor liver transplantation recipients (n = 204) who survived more than 6 mo after living donor liver transplantation were enrolled.Immunosuppression was achieved using tacrolimus and methylprednisolone. When adverse effects of tacrolimus were detected, the patient was switched to cyclosporine. Six months after transplantation,tacrolimus or cyclosporine was carefully maintained at a therapeutic level. The methylprednisolone dosage was maintained at 0.05 mg/kg per day by oral administration.Acute rejections that occurred more than 6 mo after the operation were defined as late-onset. The median followup period was 34 mo.RESULTS: LAR was observed in 15 cases (7%) and no chronic rejection was observed. The incidence of hyperlipidemia, chronic renal failure, new-onset posttransplantation diabetes, and deep fungal infection were 13%, 2%, 24%, and 17%, respectively. Conversion from tacrolimus to cyclosporine was required in 38 patients (19%). Multivariate analysis revealed that a cyclosporinebased regimen was significantly associated with LAR.CONCLUSION: Both LAR and drug-induced adverse events happen at a low incidence, supporting the safety and efficacy of the present immunosuppression regimen for living donor liver transplantation.

High-sensitive CRP as a predictive marker of long-term outcome in juvenile idiopathic arthritis

DEFF Research Database (Denmark)

Alberdi-Saugstrup, Mikel; Zak, Marek; Nielsen, Susan

2017-01-01

To evaluate whether C-reactive protein (CRP), including variation within the normal range, is predictive of long-term disease outcome in Juvenile Idiopathic Arthritis (JIA). Consecutive patients with newly diagnosed JIA were included prospectively from defined geographic areas of the Nordic...... countries from 1997 to 2000. Inclusion criteria were availability of a baseline serum sample within 12 months after disease onset and 8-year clinical assessment data. Systemic onset JIA was not included. CRP was measured by high-sensitive ELISA (detection limit of 0.2 mg/l). One hundred and thirty...... participants with a median follow-up time of 97 months (range 95–100) were included. At follow-up, 38% of the patients were in remission off medication. Absence of remission was associated with elevated level of CRP at baseline (odds ratio (OR) 1.33, confidence interval (CI) 1.08–1.63, p = 0.007). By applying...

Juvenile dispersal in Calomys venustus (Muridae: Sigmodontinae)

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Priotto, José; Steinmann, Andrea; Provensal, Cecilia; Polop, Jaime

2004-05-01

Both spacing behaviour and dispersal movement are viewed as hierarchical processes in which the effects may be expressed at spatial scale. This research was carried out to examine the hypothesis that the presence of parents promotes the dispersal of juveniles from their natal nest and their father or mother home-range, in Calomys venustus.The study was carried out in four 0.25 ha fences (two controls and two experimentals), in a natural pasture. This study had two periods: Father Removal (FR) (August and December 1997; year one) and Mother Removal (MR) (August 1998 and January 1999; year two). For the FR treatment fathers were removed after juveniles were born, but in the MR treatment mothers were removed after the juveniles were weaned. The effect of parents on the dispersal distance of juveniles was analysed with respect to their natal nest and their mother and father home-range. Dispersal distance from the nest of C. venustus was independent of either male or female parent. Juveniles were more dispersing in relation to the centre of activity of their mothers than to that of their fathers, and females were more dispersing than males. Female juveniles overlap their home-range with their parents less than male juveniles do. The differences observed between female and male juveniles would be related to their different sexual maturation times, as well as to the female territoriality.

Growth Abnormalities in Children with Type 1 Diabetes, Juvenile Chronic Arthritis, and Asthma

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Cosimo Giannini

2014-01-01

Full Text Available Children and adolescents with chronic diseases are commonly affected by a variable degree of growth failure, leading to an impaired final height. Of note, the peculiar onset during childhood and adolescence of some chronic diseases, such as type 1 diabetes, juvenile idiopathic arthritis, and asthma, underlines the relevant role of healthcare planners and providers in detecting and preventing growth abnormalities in these high risk populations. In this review article, the most relevant common and disease-specific mechanisms by which these major chronic diseases affect growth in youth are analyzed. In addition, the available and potential targeting strategies to restore the physiological, hormonal, and inflammatory pattern are described.

Alcohol consumption, new onset of diabetes after transplantation, and all-cause mortality in renal transplant recipients

NARCIS (Netherlands)

Zelle, Dorien M.; Agarwal, Pramod K.; Ramirez, Jessica L. Pinto; van der Heide, Jaap J. Homan; Corpeleijn, Eva; Gans, Reinold O. B.; Navis, Gerjan; Bakker, Stephan J. L.

2011-01-01

Background. Renal transplant recipients (RTR) are often advised to refrain from alcohol because of possible interaction with their immunosuppressive medication. Although moderate alcohol consumption is associated with reduced risk of diabetes and mortality in the general population, this is unknown

Renal blood flow and oxygenation drive nephron progenitor differentiation.

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Rymer, Christopher; Paredes, Jose; Halt, Kimmo; Schaefer, Caitlin; Wiersch, John; Zhang, Guangfeng; Potoka, Douglas; Vainio, Seppo; Gittes, George K; Bates, Carlton M; Sims-Lucas, Sunder

2014-08-01

During kidney development, the vasculature develops via both angiogenesis (branching from major vessels) and vasculogenesis (de novo vessel formation). The formation and perfusion of renal blood vessels are vastly understudied. In the present study, we investigated the regulatory role of renal blood flow and O2 concentration on nephron progenitor differentiation during ontogeny. To elucidate the presence of blood flow, ultrasound-guided intracardiac microinjection was performed, and FITC-tagged tomato lectin was perfused through the embryo. Kidneys were costained for the vasculature, ureteric epithelium, nephron progenitors, and nephron structures. We also analyzed nephron differentiation in normoxia compared with hypoxia. At embryonic day 13.5 (E13.5), the major vascular branches were perfused; however, smaller-caliber peripheral vessels remained unperfused. By E15.5, peripheral vessels started to be perfused as well as glomeruli. While the interior kidney vessels were perfused, the peripheral vessels (nephrogenic zone) remained unperfused. Directly adjacent and internal to the nephrogenic zone, we found differentiated nephron structures surrounded and infiltrated by perfused vessels. Furthermore, we determined that at low O2 concentration, little nephron progenitor differentiation was observed; at higher O2 concentrations, more differentiation of the nephron progenitors was induced. The formation of the developing renal vessels occurs before the onset of blood flow. Furthermore, renal blood flow and oxygenation are critical for nephron progenitor differentiation. Copyright © 2014 the American Physiological Society.

Essential habitat for sardine juveniles in Iberian waters

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Sílvia Rodríguez-Climent

2017-09-01

Full Text Available In a period when the Iberian sardine stock abundance is at its historical minimum, knowledge of the sardine juvenile’s distribution is crucial for the development of fishery management strategies. Generalized additive models were used to relate juvenile sardine presence with geographical variables and spawning grounds (egg abundance and to model juvenile abundance with the concurrent environmental conditions. Three core areas of juvenile distribution were identified: the Northern Portuguese shelf (centred off Aveiro, the coastal region in the vicinity of the Tagus estuary, and the eastern Gulf of Cadiz. Spatial differences in the relationship between juvenile presence and egg abundances suggest that essential juvenile habitat might partially differ from the prevailing spawning grounds. Models also depicted significant relationships between juvenile abundance, temperature and geographical variables in combination with salinity in the west and with zooplankton in the south. Results indicate that the sardine juvenile distribution along the Iberian Peninsula waters are an outcome of a combination of dynamic processes occurring early in life, such as egg and larva retention, reduced mortality and favourable feeding grounds for both larvae and juveniles.

Intraosseous Venography with Carbon Dioxide in Percutaneous Vertebroplasty: Carbon Dioxide Retention in Renal Veins

International Nuclear Information System (INIS)

Komemushi, Atsushi; Tanigawa, Noboru; Kariya, Shuji; Kojima, Hiroyuki; Shomura, Yuzo; Tokuda, Takanori; Nomura, Motoo; Terada, Jiro; Kamata, Minoru; Sawada, Satoshi

2008-01-01

The objective of the present study was to determine the frequency of gas retention in the renal vein following carbon dioxide intraosseous venography in the prone position and, while citing references, to examine its onset mechanisms. All percutaneous vertebroplasties performed at our hospital from January to December 2005 were registered and retrospectively analyzed. Of 43 registered procedures treating 79 vertebrae, 28 procedures treating 54 vertebrae were analyzed. Vertebral intraosseous venography was performed using carbon dioxide as a contrast agent in all percutaneous vertebroplasty procedures. In preoperative and postoperative vertebral CT, gas retention in the renal vein and other areas was assessed. Preoperative CT did not show gas retention (0/28 procedures; 0%). Postoperative CT confirmed gas retention in the renal vein in 10 of the 28 procedures (35.7%). Gas retention was seen in the right renal vein in 8 procedures (28.6%), in the left renal vein in 5 procedures (17.9%), in the left and right renal veins in 3 procedures (10.7%), in vertebrae in 22 procedures (78.6%), in the soft tissue around vertebrae in 14 procedures (50.0%), in the spinal canal in 12 procedures (42.9%), and in the subcutaneous tissue in 5 procedures (17.9%). In conclusion, in our study, carbon dioxide gas injected into the vertebra frequently reached and remained in the renal vein.

Diffuse Lymphomatous Infiltration of Kidney Presenting as Renal Tubular Acidosis and Hypokalemic Paralysis: Case Report

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Jhamb, Rajat; Gupta, Naresh; Garg, Sandeep; Kumar, Sachin; Gulati, Sameer; Mishra, Deepak; Beniwal, Pankaj

2007-01-01

We report the case of a 22-year-old woman who presented with acute onset flaccid quadriparesis. Physical examination showed mild pallor with cervical and axillary lymphadenopathy, hepatomegaly, and bilateral smooth enlarged kidneys. Neurological examination revealed lower motor neuron muscle weakness in all the four limbs with hyporeflexia and normal sensory examination. Laboratory investigations showed anemia, severe hypokalemia, and metabolic acidosis. Urinalysis showed a specific gravity of 1.010, pH of 7.0, with a positive urine anion gap. Ultrasound revealed hepatosplenomegaly with bilateral enlarged smooth kidneys. Renal biopsy was consistent with the diagnosis of non-Hodgkin lymphoma (B cell type). Metabolic acidosis, alkaline urine, and severe hypokalemia due to excessive urinary loss in our patient were suggestive of distal renal tubular acidosis. Renal involvement in lymphoma is usually subclinical and clinically overt renal disease is rare. Diffuse lymphomatous infiltration of the kidneys may cause tubular dysfunction and present with hypokalemic paralysis. PMID:18074421

[Decline in renal function in old age : Part of physiological aging versus age-related disease].

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Braun, F; Brinkkötter, P T

2016-08-01

The incidence and prevalence of chronic renal disease (CKD) in elderly patients are continuously increasing worldwide. Loss of renal function is not only considered to be part of the aging process itself but also reflects the multimorbidity of many geriatric patients. Calculating the glomerular filtration rate using specific algorithms validated for the elderly population and measuring the amount of proteinuria allow an estimation of renal function in elderly patients with high accuracy. Chronic renal failure has many clinical consequences and not only results in a delayed excretion of toxins cleared by the kidneys but also affects hematogenesis, water and electrolyte balance as well as mineral bone metabolism. Furthermore, CKD directly leads to and aggravates geriatric syndromes and in particular the onset of frailty. Therapeutic strategies to halt progression of CKD not only comprise treatment of the underlying disease but also efficient blood pressure and diabetic control and the avoidance of nephrotoxic medications.

Acute quadriplegia caused by necrotizing myopathy in a renal transplant recipient with severe pneumonia: acute onset and complete recovery.

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Tu, Guo-Wei; Song, Jie-Qiong; Ting, Simon Kang Seng; Ju, Min-Jie; He, Hong-Yu; Dong, Ji-Hong; Luo, Zhe

2015-02-03

Critical illness polyneuropathy and myopathy are multifaceted complications that follow severe illnesses involving the sensorimotor axons and proximal skeletal muscles. These syndromes have rarely been reported among renal transplant recipients. In this paper, we report a case of acute quadriplegia caused by necrotizing myopathy in a renal transplant recipient with severe pneumonia. The muscle strength in the patient's extremities improved gradually after four weeks of comprehensive treatment, and his daily life activities were normal a year after being discharged.

Juvenile offenders: competence to stand trial.

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Soulier, Matthew

2012-12-01

This article details the legal background and assists the reader in the preparation and practical conduct of evaluations regarding juvenile adjudicative competency. The material is presented to be useful as a guide to direct questions of competency and covers aspects of evaluation that include: legal standard for competency to stand trial, developmental immaturity, current practice in juvenile competency to stand trial, forensic evaluation of juvenile competency to stand trial, organizing the evaluation, collateral sources of information, psychiatric evaluation of juvenile adjudicative competency, assessment of mental disorder and intellectual disability, assessment of developmental status, assessment of functional abilities for adjudicative competence, and reaching the forensic opinion. Copyright © 2012 Elsevier Inc. All rights reserved.

O adulto com artrite idiopática juvenil poliarticular The adult patient with polyarticular juvenile idiopathic arthritis

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Liz Wallin

2009-08-01

Full Text Available Crianças com a forma poliarticular da artrite idiopática juvenil (AIJ, ao entrarem na idade adulta, têm um quadro similar ao de pacientes com artrite reumatoide (AR de início no adulto. No presente estudo comparam-se características clínicas e imunológicas desses dois grupos de pacientes. Para isso, foram estudados vinte adultos com AIJ poliarticular e cinquenta pacientes com AR (pareados para sexo e tempo de duração de doença, para presença de autoanticorpos, nódulos subcutâneos, síndrome de Sjögren secundária, hipotireoidismo e para a determinação de índices funcionais e antropométricos. Encontraram-se, nesses dois grupos, características similares, exceto pela presença de fator reumatoide (menor no grupo de AIJ poliarticular; P = 0,026 e menor IMC nos pacientes com AIJ poliarticular (P When children with polyarticular juvenile idiopathic arthritis (JIA reach adulthood, they have a condition similar to that of patients with adult onset rheumatoid arthritis (RA. In the present study, the clinical and immunological characteristics of these two groups of patients are compared. The presence of autoantibodies, subcutaneous nodules, secondary Sjögren syndrome, and hypothyroidism, was determined in 20 adult patients with polyarticular JIA and in 50 patients with RA (paired for gender and duration of the disease, as well as the determination of functional and anthropometric indexes. Both groups had similar characteristics, except for the presence of rheumatoid factor (lower in the polyarticular JIA group; P = 0.026 and lower BMI in patients with polyarticular JIA (P < 0.001.

Endothelin-A receptor blockade slows the progression of renal injury in experimental renovascular disease.

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Kelsen, Silvia; Hall, John E; Chade, Alejandro R

2011-07-01

Endothelin (ET)-1, a potent renal vasoconstrictor with mitogenic properties, is upregulated by ischemia and has been shown to induce renal injury via the ET-A receptor. The potential role of ET-A blockade in chronic renovascular disease (RVD) has not, to our knowledge, been previously reported. We hypothesized that chronic ET-A receptor blockade would preserve renal hemodynamics and slow the progression of injury of the stenotic kidney in experimental RVD. Renal artery stenosis, a major cause of chronic RVD, was induced in 14 pigs and observed for 6 wk. In half of the pigs, chronic ET-A blockade was initiated (RVD+ET-A, 0.75 mg·kg(-1)·day(-1)) at the onset of RVD. Single-kidney renal blood flow, glomerular filtration rate, and perfusion were quantified in vivo after 6 wk using multidetector computer tomography. Renal microvascular density was quantified ex vivo using three-dimensional microcomputer tomography, and growth factors, inflammation, apoptosis, and fibrosis were determined in renal tissue. The degree of stenosis and increase in blood pressure were similar in RVD and RVD+ET-A pigs. Renal hemodynamics, function, and microvascular density were decreased in the stenotic kidney but preserved by ET-A blockade, accompanied by increased renal expression of vascular endothelial growth factor, hepatocyte growth factor, and downstream mediators such as phosphorilated-Akt, angiopoietins, and endothelial nitric oxide synthase. ET-A blockade also reduced renal apoptosis, inflammation, and glomerulosclerosis. This study shows that ET-A blockade slows the progression of renal injury in experimental RVD and preserves renal hemodynamics, function, and microvascular density in the stenotic kidney. These results support a role for ET-1/ET-A as a potential therapeutic target in chronic RVD.

Impact of Juvenile Idiopathic Arthritis Associated Uveitis in Early Adulthood

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Vernie, Lenneke A.; Rothova, Aniki; v. d. Doe, Patricia; Los, Leonoor I.; Schalij-Delfos, Nicoline E.; de Boer, Joke H.

2016-01-01

Background Typically juvenile idiopathic arthritis (JIA)-associated uveitis (further referred as ‘JIA-uveitis’) has its onset in childhood, but some patients suffer its, sometimes visual threatening, complications or ongoing disease activity in adulthood. The objective of this study was to analyze uveitis activity, complications and visual prognosis in adulthood. Methods In this multicenter study, 67 adult patients (129 affected eyes) with JIA-uveitis were retrospectively studied for best corrected visual acuity, visual fields, uveitis activity, topical/systemic treatments, ocular complications, and ocular surgeries during their 18th, 22nd and 30th year of life. Because treatment strategies changed after the year 1990, outcomes were stratified for onset of uveitis before and after 1990. Results Sixty-two of all 67 included patients (93%) had bilateral uveitis. During their 18th life year, 4/52 patients (8%) had complete remission, 28/52 (54%) had uveitis activity and 37/51 patients (73%) were on systemic immunomodulatory treatment. Bilateral visual impairment or legal blindness occurred in 2/51 patients (4%); unilateral visual impairment or legal blindness occurred in 17/51 patients (33%) aged 18 years. The visual prognosis appeared to be slightly better for patients with uveitis onset after the year 1990 (for uveitis onset before 1990 (n = 7) four patients (58%) and for uveitis onset after 1990 (n = 44) 13 patients (30%) were either visual impaired or blind). At least one ocular surgery was performed in 10/24 patients (42%) between their 18th and 22nd year of life. Conclusions Bilateral visual outcome in early adulthood in patients with JIA-uveitis appears to be fairly good, although one third of the patients developed one visually impaired or blind eye. However, a fair amount of the patients suffered from ongoing uveitis activity and needed ongoing treatment as well as surgical interventions. Awareness of these findings is important for ophthalmologists and

[The temporomandibular joint in juvenile idiopathic arthritis: what radiologists need to look for on magnetic resonance imaging].

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De La Hoz Polo, M; Navallas, M

2014-01-01

The term "juvenile idiopathic arthritis" (JIA) encompasses a group of arthritis of unknown cause with onset before the age of 16 years that last for at least 6 weeks. The prevalence of temporomandibular joint involvement in published series ranges from 17% to 87%. Temporomandibular joint involvement is difficult to detect clinically, so imaging plays a key role in diagnosis and monitoring treatment. MRI is the technique of choice for the study of arthritis of the temporomandibular joint because it is the most sensitive technique for detecting acute synovitis and bone edema. Power Doppler ultrasonography can also detect active synovitis by showing the hypervascularization of the inflamed synovial membrane, but it cannot identify bone edema. This article describes the MRI technique for evaluating the temporomandibular joint in patients with juvenile idiopathic arthritis, defines the parameters to look for, and illustrates the main findings. Copyright © 2013 SERAM. Published by Elsevier Espana. All rights reserved.

Parenting and juvenile delinquency

OpenAIRE

Hoeve, M.

2008-01-01

Juvenile delinquency is a noteworthy problem. This thesis addressed the association between parenting and juvenile delinquency by analyzing the concepts of parenting adopted in family research in relation to criminological concepts and measures of delinquent behavior. Four studies were conducted. The first study addressed a meta-analysis on parenting characteristics and styles in relation to delinquency. In this meta-analysis, previous manuscripts were systematically analyzed, computing mean ...

PSYCHOSOCIAL PROFILE OF JUVENILE DIABETES

Science.gov (United States)

Dass, Jyoti; Dhavale, H.S.; Rathi, Anup

1999-01-01

A study of the complex relationships between the patient characteristics, family and environmental influences, physician's behaviour and the demands of the disease with its management in Juvenile Diabetics was taken up at a general hospital. 90 subjects were selected for the study and grouped into three. Group A consisted of 30 Juvenile Diabetics, Group B of 30 Adult Diabetics and Group C of 30 Normal healthy adolescents. The impact of the illness was measured on the Diabetes Impact Measurement Scale (DIMS), the behavioural deviations and the parental attitudes towards child rearing on the Fallstrom's Questionnaire (FQ) and the family environment on the Family Climate Scale (FCS). Psychiatric morbidity was assessed using DSM-IV criteria. Group A & B were compared on the DIMS and Group A & C on FQ & FCS. Adult diabetics had a greater impact of diabetes. Juvenile diabetics had significantly higher frequency of behavioural deviations as compared to controls. Also there was a higher number of responses on questions indicating an overprotecting attitude amongst parents of juvenile diabetics. There was an increased incidence of psychiatric morbidity in juvenile diabetics as compared to normal adolescents irrespective of the family environment. The results are discussed in relation to current literature. PMID:21430802

Interleukin 1 beta (IL1ß) rs16944 genetic variant as a genetic marker of severe renal manifestations and renal sequelae in Henoch-Schönlein purpura.

Science.gov (United States)

López-Mejías, Raquel; Genre, Fernanda; Remuzgo-Martínez, Sara; Sevilla Pérez, Belen; Castañeda, Santos; Llorca, Javier; Ortego-Centeno, Norberto; Ubilla, Begoña; Mijares, Verónica; Pina, Trinitario; Calvo-Río, Vanesa; Miranda-Filloy, Jose A; Navas Parejo, Antonio; Argila, Diego; Sánchez-Pérez, Javier; Rubio, Esteban; Luque, Manuel León; Blanco-Madrigal, Juan María; Galíndez-Aguirregoikoa, Eva; Martín, Javier; Blanco, Ricardo; González-Gay, Miguel A

2016-01-01

Data from a small series suggested that the Interleukin 1 beta (IL1ß) rs16944 polymorphism may be associated with severe renal involvement and persistent renal damage (renal sequelae) in Henoch-Schönlein purpura (HSP). To confirm this association, we assessed the largest cohort of Caucasian HSP patients ever considered for genetic studies. 338 Spanish HSP patients and 635 sex and ethnically matched controls were recruited in this study. All patients were required to have had at least 6 months' follow-up. Patients and controls were genotyped for IL1β rs16944 by TaqMan genotyping assay. No differences between IL1β rs16944 genotype or allele frequencies were found either in the case/control study or when HSP patients were stratified according to the age at disease onset, presence of nephritis or gastrointestinal manifestations. Nevertheless, 4 (25%) of the 16 HSP patients who developed severe renal manifestations carried the TT genotype versus 29 (9%) of 322 who did not develop this complication (p=0.01, OR=5.48, 95% CI: 1.01-28.10). Accordingly, patients carrying the mutant T allele had an increased risk of developing severe nephropathy (p=0.016, OR=2.35, 95% CI: 1.09-5.07). Additionally, a significant increase of the TT genotype was observed in patients with persistent renal damage when compared with those patients without this complication (25% versus 8.6%, respectively; p=0.0035, OR=4.90, 95% CI: 1.26- 18.51). Moreover, renal sequelae were more common in patients carrying the mutant T allele (p=0.0076, OR=2.20, 95% CI: 1.17-4.14). Our results support that the IL1ß rs16944 polymorphism may be a potential marker of severe renal manifestations and renal sequelae in HSP.

Juvenile Prostitution.

Science.gov (United States)

Csapo, Marg

1986-01-01

Recent research and Canadian government committee reports concerning juvenile prostitution are reviewed. Proposals are made in the realms of law and social policy; and existing programs are described. (DB)

Late-onset systemic lupus erythematosus in Latin Americans: a distinct subgroup?

Science.gov (United States)

Catoggio, L J; Soriano, E R; Imamura, P M; Wojdyla, D; Jacobelli, S; Massardo, L; Chacón Díaz, R; Guibert-Toledano, M; Alvarellos, A; Saurit, V; Manni, J A; Pascual-Ramos, V; Silva de Sauza, A W; Bonfa, E; Tavares Brenol, J C; Ramirez, L A; Barile-Fabris, L A; De La Torre, I Garcia; Alarcón, G S; Pons-Estel, B A

2015-07-01

To examine the characteristics of patients who developed late onset systemic lupus erythematosus (SLE) in the GLADEL (Grupo Latino Americano de Estudio del Lupus) cohort of patients with SLE. Patients with SLE of less than two years of disease duration, seen at 34 centers of nine Latin American countries, were included. Late-onset was defined as >50 years of age at time of first SLE-related symptom. Clinical and laboratory manifestations, activity index (SLEDAI), and damage index (SLICC/ACR- DI) were ascertained at time of entry and during the course (cumulative incidence). Features were compared between the two patient groups (lupus, adjusting for other variables. Of the 1480 patients included, 102 patients (6.9 %) had late-onset SLE, 87% of which were female. Patients with late-onset SLE had a shorter follow-up (3.6 vs. 4.4 years, p  0.05). In multivariable analysis, late onset was independently associated with higher odds of ocular (OR = 3.66, 95% CI = 2.15-6.23), pulmonary (OR = 2.04, 95% CI = 1.01-4.11), and cardiovascular (OR = 1.76, 95% CI = 1.04-2.98) involvement and lower odds of cutaneous involvement (OR = 0.41, 95% CI = 0.21-0.80), number of cumulative SLE criteria (OR = 0.79, 95% CI = 0.64-0.97), use of cyclophosphamide (OR = 0.47, 95% CI = 0.24-0.95), and anti-RNP antibodies (OR = 0.43, 95% CI = 0.20-0.91). A Cox regression model revealed a higher risk of dying in older onset than the younger-onset SLE (OR = 2.61, 95% CI = 1.2-5.6). Late-onset SLE in Latin Americans had a distinct disease expression compared to the younger-onset group. The disease seems to be mild with lower cumulative SLE criteria, reduced renal/mucocutaneous involvements, and less use of cyclophosphamide. Nevertheless, these patients have a higher risk of death and of ocular, pulmonary, and cardiovascular involvements. © The Author(s) 2014.

Daño renal cortical en niños con primera infección del tracto urinario alto Cortical renal damage in children with a first infection of the high urinary tract

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Tulio Antonio Amaya Sorto

2012-03-01

.Introduction: between the 5 and the 22 % of children suffering acute pyelonephritis will develop a renal scar. Objective: to describe the clinical-epidemiological features of the cortical renal damage in children with a first infection of high urinary tract. Methods: a longitudinal, prospective and observational study was conducted on the cicatricial renal damage admitted in the Nephrology services of the "William Soler" University Children Hospital from January 1, 2008 to December 31, 2009. Fifty patients were diagnosed and 38 fulfilled the inclusion criteria to study. Patients had a mean age of 18 months and underwent renal ultrasound during the acute phase of disease and static renal scintigraphy between 6 and 12 months after the acute picture, to specify exactly the cortical renal injury. In cases of renal scar, lack or decrease of the radioactive drug capture (99mTc-DMSA authors carried out miction uretrocystography to specify exactly the presence of vesicoureteral reflux. Results: twenty six patients (73.7 % are females, 17 (44.7 % aged under 6 months, 17 (44.7 % have between 6 and 36 months and 4 (10.6 % > 3 years old. The urinary infection was atypical in 23 (60.5 % and as a isolated germ the Escherichia coli in 33 (86.8 %. Ultrasound of acute phase demonstrated a renal pelvis dilation in 3 (7.9 % and renal asymmetry in 1 (2.6 %. In 2 patients (5.2 % there was renal scar and in 11 (28.4 % an decreased function of the renal cortex. The miction uretrocystography demonstrated the presence of grade III vesicoureteral reflux in a girl, who also had a renal scar. There was not relation between the onset of symptoms, the onset of therapeutics and the cortical injury. Conclusions: the risk factors to develop a post-pyelonephritis renal scar were: female sex, be aged under 3 and grade III vesicoureteral reflux.

Adult-onset hypophosphatemic osteomalacia associated with Sjogren syndrome: Clinical case report.

Science.gov (United States)

Shen, Guohua; Zhang, Yuwei; Hu, Shuang; Liu, Bin; Kuang, Anren

2017-03-01

Hypophosphatemic osteomalacia (HO) is a metabolic bone disease, exhibiting different etiologies such as genetic mutation, tumor induction, dysimmunity, or renal disease. Sjogren's syndrome (SS) is a connective tissue disorder commonly involving exocrine glands; however kidney involvement is also encountered, leading to abnormal phosphorus metabolism, even HO. A 47-year-old female patient presented progressively worsening pain in the chest wall, back and bilateral lower extremities as well as muscle weakness was referred to our department. Due to the laboratory test results, radiographic findings and pathologic results, she was diagnosed with adult-onset HO associated with SS. She was then treated with alkalinization, steroids, neutral phosphate, calcium supplements together with activated vitamin D. So far, she recovered uneventfully with relieved pain and increased serum phosphorus level. HO may be secondary to renal tubular acidosis of SS patients, and it might be a diagnostic challenge when the kidney involvement in SS is latent and precede the typical sicca symptoms.

End-Stage Renal Disease After Renal Surgery in Patients with Normal Preoperative Kidney Function: Balancing Surgical Strategy and Individual Disorders at Baseline.

Science.gov (United States)

Capitanio, Umberto; Larcher, Alessandro; Terrone, Carlo; Antonelli, Alessandro; Volpe, Alessandro; Fiori, Cristian; Furlan, Maria; Dehò, Federico; Minervini, Andrea; Serni, Sergio; Porpiglia, Francesco; Trevisani, Francesco; Salonia, Andrea; Carini, Marco; Simeone, Claudio; Montorsi, Francesco; Bertini, Roberto

2016-10-01

Although nephron-sparing surgery (NSS) has demonstrated benefit in terms of renal function preservation, it is unclear whether NSS might also decrease the risk of end-stage renal disease (ESRD) relative to radical nephrectomy (RN). In the current paper, we aimed to report the rate and the predictors of ESRD after surgery, accounting for detailed individual baseline characteristics and comorbidities. A multi-institutional collaboration among five European tertiary care centers allowed study of 2027 patients with normal preoperative renal function and a clinically localized T1abN0M0 renal mass. Cox regression analyses were used to predict the risk of ESRD (defined as the onset of a postoperative estimated glomerular filtration rate kidney disease. Univariable ESRD rates at 5 and 10 yr of follow-up were virtually equivalent for patients who underwent NSS (1.5% and 2.5%, respectively) versus RN (1.9% and 2.7%, respectively; hazard ratio [HR]: 0.8; 95% confidence interval [CI], 0.4-1.6). However, diabetes, smoking, uncontrolled hypertension, and other comorbidities were consistently more frequent in the NSS group relative to their RN counterparts. After adjusting for detailed baseline individual characteristics, NSS was shown to have an independent protective effect relative to RN (HR: 0.4; 95% CI, 0.2-0.8; p=0.02) at multivariable analyses. After accounting for individual baseline characteristics, such as age, diabetes, uncontrolled hypertension, or other comorbidities, partial nephrectomy independently protects against end-stage renal disease and the consequent need for dialysis relative to radical nephrectomy. Copyright © 2016 European Association of Urology. Published by Elsevier B.V. All rights reserved.

IgG4-related disease: description of a case with pulmonary lesions, mediastinal lymphadenopathies and rapidly progressive renal failure.

Science.gov (United States)

Fernández Lorente, Loreto; Álvarez, Dolores López; López, Virginia García; Kollros, Vesna Abujder; Ariza, Aurelio; Gálvez, Alejandro; Bonet, Josep

2015-01-01

This is a case report of a 73-year-old man with new-onset acute renal failure while being investigated for pulmonary infiltrates and mediastinal lymphadenopathies. Urine tests showed tubular range proteinuria with no microhaematuria. Immunology tests showed elevated serum IgG and hypocomplementaemia (classical pathway activation). Renal biopsy and clinical-pathological correlation were crucial in this case, reinforcing their important role in the final diagnosis of acute kidney injury. Copyright © 2015. Published by Elsevier España, S.L.U.

A Patient-Specific Foot Model for the Estimate of Ankle Joint Forces in Patients with Juvenile Idiopathic Arthritis.

Science.gov (United States)

Prinold, Joe A I; Mazzà, Claudia; Di Marco, Roberto; Hannah, Iain; Malattia, Clara; Magni-Manzoni, Silvia; Petrarca, Maurizio; Ronchetti, Anna B; Tanturri de Horatio, Laura; van Dijkhuizen, E H Pieter; Wesarg, Stefan; Viceconti, Marco

2016-01-01

Juvenile idiopathic arthritis (JIA) is the leading cause of childhood disability from a musculoskeletal disorder. It generally affects large joints such as the knee and the ankle, often causing structural damage. Different factors contribute to the damage onset, including altered joint loading and other mechanical factors, associated with pain and inflammation. The prediction of patients' joint loading can hence be a valuable tool in understanding the disease mechanisms involved in structural damage progression. A number of lower-limb musculoskeletal models have been proposed to analyse the hip and knee joints, but juvenile models of the foot are still lacking. This paper presents a modelling pipeline that allows the creation of juvenile patient-specific models starting from lower limb kinematics and foot and ankle MRI data. This pipeline has been applied to data from three children with JIA and the importance of patient-specific parameters and modelling assumptions has been tested in a sensitivity analysis focused on the variation of the joint reaction forces. This analysis highlighted the criticality of patient-specific definition of the ankle joint axes and location of the Achilles tendon insertions. Patient-specific detection of the Tibialis Anterior, Tibialis Posterior, and Peroneus Longus origins and insertions were also shown to be important.

Continuous renal replacement therapy improves renal recovery from acute renal failure.

Science.gov (United States)

Jacka, Michael J; Ivancinova, Xenia; Gibney, R T Noel

2005-03-01

Acute renal failure (ARF) occurs in up to 10% of critically ill patients, with significant associated morbidity and mortality. The optimal mode of renal replacement therapy (RRT) remains controversial. This retrospective study compared continuous renal replacement therapy (CRRT) and intermittent hemodialysis (IHD) for RRT in terms of intensive care unit (ICU) and hospital mortality, and renal recovery. We reviewed the records of all patients undergoing RRT for the treatment of ARF over a 12-month period. Patients were compared according to mode of RRT, demographics, physiologic characteristics, and outcomes of ICU and hospital mortality and renal recovery using the Chi square, Student's t test, and multiple logistic regression as appropriate. 116 patients with renal insufficiency underwent RRT during the study period. Of these, 93 had ARF. The severity of illness of CRRT patients was similar to that of IHD patients using APACHE II (25.1 vs 23.5, P = 0.37), but they required significantly more intensive nursing (therapeutic intervention scale 47.8 vs 37.6, P = 0.0001). Mortality was associated with lower pH at presentation (P = 0.003) and increasing age (P = 0.03). Renal recovery was significantly more frequent among patients initially treated with CRRT (21/24 vs 5/14, P = 0.0003). Further investigation to define optimal timing, dose, and duration of RRT may be beneficial. Although further study is needed, this study suggests that renal recovery may be better after CRRT than IHD for ARF. Mortality was not affected significantly by RRT mode.

A Case of Severe Hydroxychloroquine-Induced Retinal Toxicity in a Patient with Recent Onset of Renal Impairment: A Review of the Literature on the Use of Hydroxychloroquine in Renal Impairment

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Rajen Tailor

2012-01-01

Full Text Available We present a case of a 67-year-old female who presented with a twelve-month history of progressive blurred vision in both eyes. The patient was on hydroxychloroquine 200 mg twice a day for eight years for the treatment of scarring alopecia. Two years prior to presenting, the patient was found to have chronic kidney disease stage 3 secondary to hypertension. Examination revealed bilateral reduced visual acuities with attenuated arterioles and pigmentary changes on retinal assessment. Goldmann visual fields showed grossly constricted fields in both eyes. The patient was diagnosed with retinal toxicity secondary to hydroxychloroquine probably potentiated by renal impairment. Risk factors for retinal toxicity secondary to hydroxychloroquine can be broadly divided into dose-related and patient-related factors. Our patient developed severe retinal toxicity despite being on the recommended daily dose (400 mg per day. Although retinal toxicity at this dose has been documented, the development of renal impairment without dose adjustment or close monitoring of visual function is likely to have potentiated retinal toxicity. This case highlights the need to monitor renal function in patients on hydroxychloroquine. Should renal impairment develop, either the drug should be stopped or the dose reduced with close monitoring of visual function by an ophthalmologist.

Púrpura trombocitopênica trombótica na apresentação de pacientes com lúpus eritematoso sistêmico juvenil Thrombotic thrombocytopenic purpura at presentation of juvenile systemic lupus erythematosus patients

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Lucia M. A. Campos

2013-02-01

Full Text Available Púrpura trombocitopênica trombótica (PTT é uma alteração hematológica rara e com risco de morte, caracterizada por trombocitopenia, anemia hemolítica microangiopática e alterações neurológicas e/ou renais. A PTT foi descrita em raros pacientes com lúpus eritematoso sistêmico juvenil (LESJ e, até onde se sabe, a prevalência dessa manifestação em uma população de lúpus pediátrico ainda não foi estudada. Assim, entre janeiro de 1983 e dezembro de 2010, revisamos os prontuários de 5.508 pacientes acompanhados na Unidade de Reumatologia Pediátrica do nosso hospital universitário. Foram identificados 279 (5,1% casos de LESJ que preencheram os critérios de classificação do American College of Rheumatology. Dois destes (0,7% apresentavam PTT, ambos no início do LESJ, e foram aqui descritos. Os dois pacientes tinham febre, anemia hemolítica microangiopática (com esquizócitos no sangue periférico e trombocitopenia. O paciente do gênero masculino apresentava hemiparesia e proteinúria, e a paciente do gênero feminino tinha cefaleia persistente e hematúria. Ambos foram tratados com metilprednisolona endovenosa e plasmaferese quando do diagnóstico de PPT. Após tratamento, não houve recidiva da PTT, e hematócritos, contagens de plaquetas e níveis de desidrogenase lática permaneceram normais. Em conclusão, a PTT é uma rara e grave manifestação no início do LESJ. Os casos relatados reforçam a importância de um diagnóstico precoce e de uma terapia agressiva em pacientes com PTT, devido à sua alta morbidade.Thrombotic thrombocytopenic purpura (TTP is a rare and life-threatening hematological abnormality characterized by thrombocytopenia and microangiopathic hemolytic anemia, with neurological abnormalities and/or renal disease. TTP has been rarely reported in juvenile systemic lupus erythematosus (JSLE patients and, to our knowledge, its prevalence in a paediatric lupus population has not been studied. Therefore

Imágenes juveniles, medios y nuevos escenarios

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Oscar Aguilera Ruiz

2015-01-01

Full Text Available Este artículo nace del análisis de los discursos radiales producidos exclusivamente para jóvenes en Santiago de Chile. Aborda además las categorías comprensivas de la vida juvenil, la vida juvenil de los años 90, el imaginario juvenil des-simbolizado, hacia una comprensión de lo juvenil, dinámica social propuesta por los medios y estrategias comunicacionales.

Juvenile myasthenia gravis in Norway: Clinical characteristics, treatment, and long-term outcome in a nationwide population-based cohort.

Science.gov (United States)

Popperud, T H; Boldingh, M I; Rasmussen, M; Kerty, E

2017-09-01

This study aimed to characterize juvenile myasthenia gravis in a national population-based cohort in Norway, and to evaluate long-term outcome and potential differences correlated with prepubertal versus postpubertal disease onset. Patients with onset of myasthenia gravis aged ≤18 years were identified through multiple strategies. Retrospective clinical data were collected by means of medical charts. All patients had an updated clinical examination. Cases were divided into prepubertal and postpubertal onset using age 12 years as the cut off. In total, 75 patients were identified of whom 63 were included in the study: 21 in the prepubertal and 42 in the postpubertal onset group. There was a female preponderance in both groups. In total, 59% presented with ocular symptoms, but the great majority of patients in both groups generalized during the two first years of the disease. Myasthenic crisis was more frequent in the prepubertal onset group. All patients were initially treated with pyridostigmine, 26 with steroids, and 17 with other immunosuppressive treatment. The postpubertal cases were more often treated with immunosuppressive therapy. Fifty patients (79%) underwent thymectomy. The general outcome was favourable: 57% became asymptomatic and only four subjects failed to attain clinical improvement. One-third had at least one additional autoimmune disease. Despite frequent symptom generalization and a subgroup of prepubertal onset with severe disease, the long-term outcome was good, especially in the thymectomized prepubertal onset group. Polyautoimmunity occurred in both groups in one-third. Copyright © 2017 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

Renal cell carcinoma in patient with crossed fused renal ectopia

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Ozgur Cakmak

2016-01-01

Full Text Available Primary renal cell carcinomas have rarely been reported in patients with crossed fused renal ectopia. We presented a patient with right to left crossed fused kidney harbouring renal tumor. The most frequent tumor encountered in crossed fused renal ectopia is renal cell carcinoma. In this case, partial nephrectomy was performed which pave way to preservation of the uninvolved both renal units. Due to unpredictable anatomy, careful preoperative planning and meticulous delineation of renal vasculature is essential for preservation of the uninvolved renal units.

Early-Onset Acute Recurrent and Chronic Pancreatitis Is Associated with PRSS1 or CTRC Gene Mutations.

Science.gov (United States)

Giefer, Matthew J; Lowe, Mark E; Werlin, Steven L; Zimmerman, Bridget; Wilschanski, Michael; Troendle, David; Schwarzenberg, Sarah Jane; Pohl, John F; Palermo, Joseph; Ooi, Chee Y; Morinville, Veronique D; Lin, Tom K; Husain, Sohail Z; Himes, Ryan; Heyman, Melvin B; Gonska, Tanja; Gariepy, Cheryl E; Freedman, Steven D; Fishman, Douglas S; Bellin, Melena D; Barth, Bradley; Abu-El-Haija, Maisam; Uc, Aliye

2017-07-01

To assess whether the age of onset was associated with unique features or disease course in pediatric acute recurrent pancreatitis (ARP) or chronic pancreatitis (CP). Demographic and clinical information on children with ARP or CP was collected at INSPPIRE (INternational Study Group of Pediatric Pancreatitis: In Search for a CuRE) centers. The Cochran-Armitage trend test and Jonckheere-Terpstra test were used to examine for differences between pediatric age groups (pancreatitis, 111 (32%) were 6-11 years of age, and 102 (30%) were ≥12 years of age. Early-onset disease was associated with mutations in cationic trypsinogen (PRSS1) (P pancreatitis (P = .02), family history of CP (P chronic renal failure (P = .02). Later-onset disease was more commonly present with hypertriglyceridemia (P = .04), ulcerative colitis (P = .02), autoimmune diseases (P pancreatitis is associated strongly with PRSS1 or CTRC mutations and family history of pancreatitis. Children with later-onset disease are more likely to have nongenetic risk factors. Future studies are needed to investigate whether the disease course, response to therapy, or clinical outcomes differ relative to the timing of disease onset. Copyright © 2017 Elsevier Inc. All rights reserved.

European Veterinary Renal Pathology Service: A Survey Over a 7-Year Period (2008-2015).

Science.gov (United States)

Aresu, L; Martini, V; Benali, S L; Brovida, C; Cianciolo, R E; Dalla Riva, R; Trez, D; Van Der Lugt, J J; Van Dongen, A; Zini, E

2017-09-01

The European Veterinary Renal Pathology Service (EVRPS) is the first Web-based registry for canine renal biopsy specimens in Europe. The aim was to verify whether differences exist between the clinical and laboratory presentation of dogs with nephropathy according to renal pathological findings, as defined by light and electron microscopy of renal biopsy specimens submitted to EVRPS. Renal biopsy specimens of dogs were collected from the archive of the service (n = 254). Cases were included if both light and electron microscopy were available (n = 162). Renal biopsy specimens were classified based on the morphological diagnoses. Thereafter, they were grouped into 3 disease categories, including immune-complex-mediated glomerulonephritis (ICGN), non-immune-complex-mediated GN (non-ICGN), and renal lesions not otherwise specified (RL-NOS). Differences among morphological diagnoses and among disease categories were investigated for clinical and laboratory variables. Serum albumin concentration was lower in dogs with ICGN than in those with non-ICGN (P = 0.006) or RL-NOS (P = 0.000), and the urine protein-to-creatinine ratio (UPC) was significantly higher in ICGN than in the other 2 disease categories. Regarding morphological diagnoses, albumin was significantly lower in amyloidosis (AMY) and membranous (MGN), membranoproliferative (MPGN) or mixed glomerulonephritis (MixGN) than in minimal change disease, primary (FSGS I) or secondary (FSGS II) focal and segmental glomerulosclerosis and juvenile nephropathies (JN). The UPC was higher in MPGN than in FSGS I and FSGS II. Dogs with ICGN, in particular MPGN, had higher protein loss than those with non-ICGN or RL-NOS, leading to more severe hypoalbuminemia. Clinical and laboratory differentiation among dogs with the different morphological diagnoses and among dogs with different disease categories was difficult due to overlapping results. Copyright © 2017 The Authors. Journal of Veterinary Internal Medicine

Vegetative propagation of mature and juvenile northern red oak

Science.gov (United States)

James J. Zaczek; K. C. Steiner; C. W., Jr. Heuser

1993-01-01

Rooting trials were established to evaluate rooting success of cuttings from mature and juvenile, grafted and ungrafted northern red oak (NRO). Buds from 4 mature NRO ortets and juvenile seedlings were grafted onto juvenile and mature rootstock. Cuttings were collected from the grafts and from juvenile and mature shoots developed in situ and...

REFORMATIONS IN ZIMBABWE'S JUVENILE JUSTICE SYSTEM

African Journals Online (AJOL)

Mugumbate

1996-05-23

May 23, 1996 ... The article is based on a desk review of existing literature on juvenile crime in the country. ... that Zimbabwe's juvenile justice system is transforming from being ... recommendations include expanding the Pre-trial Diversion ...

Chronic renal failure due to unilateral renal agenesis and total renal dysplasia (=aplasia)

International Nuclear Information System (INIS)

Kroepelin, T.; Ziupa, J.; Wimmer, B.

1983-01-01

Three adult patients with unilateral renal agenesis/total dysplasia (= aplasia) and with an early chronic renal failure are presented. One patient had renal agenesis without ureter bud and ureteric ostium on one side, and reflux pyelonephritis on the other; one had small compact total renal dysplasia (= aplasia) on one side, while chronic uric acid nephropathy (chronic renal disease as a cause of gout) was diagnosed on the other; the third patient had a total large multicystic dysplasia on one side, and on the other a segmental large multicystic dysplasia. Radiological steps and radiodiagnostic criteria are discussed and the combination of urogenital and extraurogenital anomalies is referred to. (orig.)

Efficacy of ultrasonography-guided renal biopsy for the evaluation of renal dysfunction following renal transplantation

International Nuclear Information System (INIS)

Kim, Young Jae; Choi, Chul Soon; Min, Seon Jeong; Lee, Gyung Kyu; Lee, Eil Seong; Kang, Ik Won; Bae, Sang Hoon

2003-01-01

To evaluate the usefulness and complications of renal biopsy under ultrasonography-guidance in renal dysfunction after renal transplantation. Ultrasonography-guided renal biopsy was done in 47 patients with the transplanted kidney. The subjects consisted of 30 males and 17 females, age ranged from 16 to 66 years (average age=38 years). Biopsies were done once in 27 patients, twice in 17 patients, three times in 3 patients, a total of 70 biopsies. The success rate of renal biopsy for the accurate pathologic diagnosis and the incidence and types of complications following biopsy were evaluated. The success rate of renal biopsy for the accurate pathologic diagnosis was 96%(67/70). Pathologic diagnosis included 27 cases of acute rejection (39%), 8 cases of acute tubular necrosis (11%), 4 cases of acute rejection and acute tubular necrosis (6%), 4 cases of cyclosporin toxicity (6%), 4 cases of primary disease recurrence (6%), 4 cases of infection (6%) and others. Complications after renal biopsy included 15 cases of microscopic hematuria (21%), 1 case of gross hematuria with spontaneous cessation and 1 case of life threatening hemorrhage. Ultrasonography-guided renal biopsy is a safe and effective diagnostic method for the evaluation of renal dysfunction following renal transplantation.

Interactive effects of ATOH7 and RFTN1 in association with juvenile primary open-angle glaucoma

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De-Gui Wang

2017-03-01

Full Text Available AIM:To study ATOH7 and RFTN1 sequence variations in patients with juvenile primary open-angle glaucoma(JOAG. METHODS: In 298 controls(age≥60yand 52 JOAG(ageATOH7 was sequenced by direct sequencing. Additional single nucleotide polymorphisms the RFTN1 SNP(rs690037and at upstream ATOH7(rs1900004 and rs3858145were genotyped by Taqman assay. RESULTS: No any coding mutation was detected in JOAG. There were no significance in allele frequencies and haplotypes between JOAG and control group of rs7916697, rs61854782, rs1900004、rs3858145 and rs690037, so no SNP was associated with JOAG(P>0.05. CONCLUSION: Although preliminary study has showed combination of ATOH7 and RFTN1 SNPs could increase the risk of getting adult-onset primary open angle glaucoma, ATOH7 and RFTN1 are not associated with juvenile primary open-angle glaucoma in this study, so different types of open-angle glaucoma may be differences in genetic mechanism and be worthy of further study.

Methotrexate is an effective treatment for chronic uveitis associated with juvenile idiopathic arthritis.

Science.gov (United States)

Foeldvari, Ivan; Wierk, Angela

2005-02-01

To assess the effectiveness of methotrexate (MTX) in the treatment of juvenile idiopathic arthritis (JIA) associated uveitis, which is still one of the most common causes of visual impairment. A retrospective chart review of patients with the diagnosis of uveitis associated with JIA between July 1, 2002, and December 31, 2002. Four hundred sixty-seven patients with JIA were followed. Thirty-eight had uveitis: 31 associated with oligoarticular JIA and 7 with psoriatic JIA. Twenty-five of the 38 patients received MTX; in 23 patients uveitis was the indication for MTX therapy. In the MTX treated group 46/50 eyes had uveitis, the mean (range) age at onset of uveitis was 7.82 years (1.8-15.8), and the mean age at onset of arthritis was 7.25 years (1.25-15.7). MTX treatment was started an average of 11.4 months (0-72) after the onset of uveitis. The mean MTX dose was 15.6 mg/m2. Remission occurred after 4.25 months (1-12). Mean duration of remission was 10.3 months (3-27). The total duration of MTX therapy was 661 months and patients were in remission for 417/661 months. In 6 patients MTX was discontinued after 12 months of remission. Four patients were still in remission after 7.5 months (1-14). MTX seems to be an effective therapy for JIA associated uveitis.

Síndrome de ativação macrofágica em paciente com lúpus eritematoso sistêmico juvenil Macrophage activation syndrome in a patient with juvenile systemic lupus erythematosus

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Simone Manso de Carvalho

2008-08-01

Full Text Available A hemofagocitose reativa ou síndrome de ativação macrofágica (SAM é uma complicação das doenças inflamatórias sistêmicas, causada por expansão de células T e macrófagos, com produção maciça de citocinas pró-inflamatórias, ocorrendo mais freqüentemente na artrite idiopática juvenil sistêmica e raramente no lúpus eritematoso sistêmico juvenil (LESJ. OBJETIVO: Relatar um caso de LESJ que evoluiu com SAM precipitada por infecção e infarto esplênico, com desfecho fatal. RELATO DE CASO: Uma menina de 7 anos, com diagnóstico de LESJ desde os 5 anos, evoluiu com artrite em atividade, alopecia intensa, citopenias, cefaléia, infecções respiratórias recorrentes e elevação intermitente de transaminases. Os anticorpos anti-DNA e anticardiolipina IgG e IgM foram identificados e a biópsia renal evidenciou glomerulonefrite lúpica de classe III. A paciente foi tratada com pulso de metilprednisolona, prednisona, azatioprina e hidroxicloroquina. Após dois anos, na vigência de pneumonia apresentou abdome agudo e convulsões, evoluindo para o choque hemorrágico fatal após esplenectomia, que evidenciou infarto esplênico e infiltração maciça por macrófagos hemofagocíticos CD163+. CONCLUSÃO: A revisão do desfecho sugere a SAM precipitada por infecção e sobreposta a atividade inflamatória do lúpus com febre persistente, citopenias, disfunção hepática, hepatomegalia e esplenomegalia, como efeitos do excesso de produção de citocinas. Os anticorpos anticardiolipina podem ter tido papel precipitante na coagulopatia, que resultou infarto esplênico e choque hemorrágico.Reactive haemophagocytosis or macrophage activation syndrome (MAS is a complication of systemic inflammatory disorders, caused by expansion of T cells and haemophagocytic macrophages, with cytokine overproduction. It has been described most often in systemic juvenile idiopathic arthritis and rarely in juvenile systemic lupus erythematosus (JSLE

Protein-energy malnutrition during early gestation in sheep blunts fetal renal vascular and nephron development and compromises adult renal function.

Science.gov (United States)

Lloyd, Louise J; Foster, Thomas; Rhodes, Phillip; Rhind, Stewart M; Gardner, David S

2012-01-15

A nutritionally poor maternal diet can reduce nephron endowment and pre-empt premature expression of markers for chronic renal disease in the offspring. A mechanistic pathway from variation in maternal diet through altered fetal renal development to compromised adult kidney structure and function with adult-onset obesity has not been described. We show that maternal protein-energy malnutrition in sheep blunts nephrogenic potential in the 0.44 gestation (65 days gestation, term ∼147 days) fetus by increasing apoptosis and decreasing angiogenesis in the nephrogenic zone, effects that were more marked in male fetuses. As adults, the low-protein-exposed sheep had reduced glomerular number and microvascular rarefaction in their kidneys compensated for, respectively, by glomerular hypertrophy and increased angiogenic support. In this study, the long-term mild anatomical deficits in the kidney would have remained asymptomatic in the lean state, but when superimposed on the broad metabolic challenge that obesity represents then microalbuminuria and blunted bilateral renal function revealed a long-term physiological compromise, that is only predicted to worsen with age. In conclusion, maternal protein-energy malnutrition specifically impacts fetal kidney vascular development and prevents full functionality of the adult kidney being achieved; these residual deficits are predicted to significantly increase the expected incidence of chronic kidney disease in prenatally undernourished individuals especially when coupled with a Western obesogenic environment.

“Transcollateral” Renal Angioplasty for a Completely Occluded Renal Artery

International Nuclear Information System (INIS)

Chandra, Subash; Chadha, Davinder S.; Swamy, Ajay

2011-01-01

Percutaneous transluminal renal angioplasty with stenting has been effective in the control of hypertension, renal function, and pulmonary edema caused by atherosclerotic renal artery stenosis. However, the role of the procedure has not been fully established in the context of chronic total occlusion of renal artery. We report the successful use of this procedure in 57-year-old male patient who reported for evaluation of a recent episode of accelerated hypertension. A renal angiogram in this patient showed ostial stenosis of the right renal artery, which was filling by way of the collateral artery. Renal angioplasty for chronic total occlusion of right renal artery was successfully performed in a retrograde fashion through a collateral artery, thereby leading to improvement of renal function and blood pressure control.

Adolescent neglect, juvenile delinquency and the risk of recidivism.

Science.gov (United States)

Ryan, Joseph P; Williams, Abigail B; Courtney, Mark E

2013-03-01

Victims of child abuse and neglect are at an increased risk of involvement with the juvenile justice and adult correctional systems. Yet, little is known about the continuation and trajectories of offending beyond initial contact with law enforcement. Neglect likely plays a critical role in continued offending as parental monitoring, parental rejection and family relationships are instrumental in explaining juvenile conduct problems. This study sought to determine whether neglect is associated with recidivism for moderate and high risk juvenile offenders in Washington State. Statewide risk assessments and administrative records for child welfare, juvenile justice, and adult corrections were analyzed. The sample was diverse (24 % female, 13 % African American, 8 % Hispanic, 5 % Native American) and included all moderate and high risk juvenile offenders screened by juvenile probation between 2004 and 2007 (n = 19,833). Official records from child protection were used to identify juvenile offenders with a history of child neglect and to identify juvenile offenders with an ongoing case of neglect. Event history models were developed to estimate the risk of subsequent offending. Adolescents with an ongoing case neglect were significantly more likely to continue offending as compared with youth with no official history of neglect. These findings remain even after controlling for a wide range of family, peer, academic, mental health, and substance abuse covariates. Interrupting trajectories of offending is a primary focus of juvenile justice. The findings of the current study indicate that ongoing dependency issues play a critical role in explaining the outcomes achieved for adolescents in juvenile justice settings. The implications for improved collaboration between child welfare and juvenile justice are discussed.

Do juveniles bully more than young offenders?

Science.gov (United States)

Ireland, Jane L

2002-04-01

This study compares bullying behaviour among juvenile and young offenders and incorporates two different methods to measure bullying. Ninety-five male juvenile and 196 male young offenders completed two questionnaires, one that measured bullying directly and one that measured behaviours indicative of "being bullied" or of "bullying others". Juveniles perceived a higher extent of bullying than young offenders. Juveniles reported significantly more physical, psychological or verbal and overall direct forms of bullying behaviour than young offenders. A number of differences were found between juveniles and young offenders with regard to the types of prisoners likely to become victims, who they would advise a victim to speak to and how bullying could be prevented. The results are discussed in relation to developmental theories of aggression and how bullying behaviour can be defined and measured among prisoners. Copyright 2002 The Association for Professionals in Services for Adolescents. Published by Elsevier Science Ltd. All rights reserved.

Bacterial acquisition in juveniles of several broadcast spawning coral species.

Science.gov (United States)

Sharp, Koty H; Ritchie, Kim B; Schupp, Peter J; Ritson-Williams, Raphael; Paul, Valerie J

2010-05-28

Coral animals harbor diverse microorganisms in their tissues, including archaea, bacteria, viruses, and zooxanthellae. The extent to which coral-bacterial associations are specific and the mechanisms for their maintenance across generations in the environment are unknown. The high diversity of bacteria in adult coral colonies has made it challenging to identify species-specific patterns. Localization of bacteria in gametes and larvae of corals presents an opportunity for determining when bacterial-coral associations are initiated and whether they are dynamic throughout early development. This study focuses on the early onset of bacterial associations in the mass spawning corals Montastraea annularis, M. franksi, M. faveolata, Acropora palmata, A. cervicornis, Diploria strigosa, and A. humilis. The presence of bacteria and timing of bacterial colonization was evaluated in gametes, swimming planulae, and newly settled polyps by fluorescence in situ hybridization (FISH) using general eubacterial probes and laser-scanning confocal microscopy. The coral species investigated in this study do not appear to transmit bacteria via their gametes, and bacteria are not detectable in or on the corals until after settlement and metamorphosis. This study suggests that mass-spawning corals do not acquire, or are not colonized by, detectable numbers of bacteria until after larval settlement and development of the juvenile polyp. This timing lays the groundwork for developing and testing new hypotheses regarding general regulatory mechanisms that control bacterial colonization and infection of corals, and how interactions among bacteria and juvenile polyps influence the structure of bacterial assemblages in corals.

Analysis of adverse events of renal impairment related to platinum-based compounds using the Japanese Adverse Drug Event Report database.

Science.gov (United States)

Naganuma, Misa; Motooka, Yumi; Sasaoka, Sayaka; Hatahira, Haruna; Hasegawa, Shiori; Fukuda, Akiho; Nakao, Satoshi; Shimada, Kazuyo; Hirade, Koseki; Mori, Takayuki; Yoshimura, Tomoaki; Kato, Takeshi; Nakamura, Mitsuhiro

2018-01-01

Platinum compounds cause several adverse events, such as nephrotoxicity, gastrointestinal toxicity, myelosuppression, ototoxicity, and neurotoxicity. We evaluated the incidence of renal impairment as adverse events are related to the administration of platinum compounds using the Japanese Adverse Drug Event Report database. We analyzed adverse events associated with the use of platinum compounds reported from April 2004 to November 2016. The reporting odds ratio at 95% confidence interval was used to detect the signal for each renal impairment incidence. We evaluated the time-to-onset profile of renal impairment and assessed the hazard type using Weibull shape parameter and used the applied association rule mining technique to discover undetected relationships such as possible risk factor. In total, 430,587 reports in the Japanese Adverse Drug Event Report database were analyzed. The reporting odds ratios (95% confidence interval) for renal impairment resulting from the use of cisplatin, oxaliplatin, carboplatin, and nedaplatin were 2.7 (2.5-3.0), 0.6 (0.5-0.7), 0.8 (0.7-1.0), and 1.3 (0.8-2.1), respectively. The lower limit of the reporting odds ratio (95% confidence interval) for cisplatin was >1. The median (lower-upper quartile) onset time of renal impairment following the use of platinum-based compounds was 6.0-8.0 days. The Weibull shape parameter β and 95% confidence interval upper limit of oxaliplatin were impairment during cisplatin use in real-world setting. The present findings demonstrate that the incidence of renal impairment following cisplatin use should be closely monitored when patients are hypertensive or diabetic, or when they are co-administered furosemide, loxoprofen, or pemetrexed. In addition, healthcare professionals should closely assess a patient's background prior to treatment.

Brivaracetam: a novel antiepileptic drug for focal-onset seizures.

Science.gov (United States)

Stephen, Linda J; Brodie, Martin J

2018-01-01

Brivaracetam (BRV), the n -propyl analogue of levetiracetam (LEV), is the latest antiepileptic drug (AED) to be licensed in Europe and the USA for the adjunctive treatment of focal-onset seizures with or without secondary generalization in patients aged 16 years or older. Like LEV, BRV binds to synaptic vesicle protein 2A (SV2A), but BRV has more selective binding and a 15- to 30-fold higher binding affinity than LEV. BRV is more effective than LEV in slowing synaptic vesicle mobilization and the two AEDs may act at different binding sites or interact with different conformational states of the SV2A protein. In animal models, BRV provides protection against focal and secondary generalized seizures and has significant anticonvulsant effects in genetic models of epilepsy. The drug undergoes first-order pharmacokinetics with an elimination half-life of 7-8 h. Although BRV is metabolized extensively, the main circulating compound is unchanged BRV. Around 95% of metabolites undergo renal elimination. No dose reduction is required in renal impairment, but it is recommended that the daily dose is reduced by one-third in hepatic dysfunction that may prolong half-life. BRV has a low potential for drug interactions. The efficacy and tolerability of adjunctive BRV in adults with focal-onset seizures have been explored in six randomized, placebo-controlled studies. These showed significant efficacy outcomes for doses of 50-200 mg/day. The most common adverse events reported were headache, somnolence, dizziness, fatigue and nausea. Patients who develop psychiatric symptoms with LEV appear to be at risk of similar side effects with BRV, although preliminary data suggest that these issues are likely to be less frequent and perhaps less severe. As with all AEDs, a low starting dose and slow titration schedule help to minimize side effects and optimize seizure control and thereby quality of life.

Laser capture microdissection of gonads from juvenile zebrafish

DEFF Research Database (Denmark)

Jørgensen, Anne; Nielsen, John; Morthorst, Jane Ebsen

2009-01-01

was adjusted and optimised to isolate juvenile zebrafish gonads. Results: The juvenile zebrafish gonad is not morphologically distinguishable when using dehydrated cryosections on membrane slides and a specific staining method is necessary to identify the gonads. The protocol setup in this study allows......Background: Investigating gonadal gene expression is important in attempting to elucidate the molecular mechanism of sex determination and differentiation in the model species zebrafish. However, the small size of juvenile zebrafish and correspondingly their gonads complicates this type...... of investigation. Furthermore, the lack of a genetic sex marker in juvenile zebrafish prevents pooling gonads from several individuals. The aim of this study was to establish a method to isolate the gonads from individual juvenile zebrafish allowing future investigations of gonadal gene expression during sex...

Extending juvenility in grasses

Energy Technology Data Exchange (ETDEWEB)

Kaeppler, Shawn; de Leon Gatti, Natalia; Foerster, Jillian

2017-04-11

The present invention relates to compositions and methods for modulating the juvenile to adult developmental growth transition in plants, such as grasses (e.g. maize). In particular, the invention provides methods for enhancing agronomic properties in plants by modulating expression of GRMZM2G362718, GRMZM2G096016, or homologs thereof. Modulation of expression of one or more additional genes which affect juvenile to adult developmental growth transition such as Glossy15 or Cg1, in conjunction with such modulation of expression is also contemplated. Nucleic acid constructs for down-regulation of GRMZM2G362718 and/or GRMZM2G096016 are also contemplated, as are transgenic plants and products produced there from, that demonstrate altered, such as extended juvenile growth, and display associated phenotypes such as enhanced yield, improved digestibility, and increased disease resistance. Plants described herein may be used, for example, as improved forage or feed crops or in biofuel production.

Influences of renal stone surgeries on renal function; Evaluation of renal function with sup 99m Tc-DMSA renal scintigraphy

Energy Technology Data Exchange (ETDEWEB)

Katayama, Yasushi (Niigata Univ. (Japan). School of Medicine)

1991-10-01

From 1984 to 1990, {sup 99m}Tc-DMSA renal scintigraphy was performed before and after nephrolithotomy (15 cases), pyelolithotomy (15 cases), percutaneous nephrolithotripsy (PNL: 15 cases) and extracorporeal shock wave lithotripsy (ESWL: 16 cases, 17 kidneys) in order to evaluate of influences of renal stone surgeries on split renal function. DMSA renal uptake change ratio of treated kidneys of nephrolithotomy (-24.94{+-}5.60%) was significantly lower than that of PNL (-0.06{+-}3.92%), pyelolithotomy (-4.08{+-}4.79%) (p<0.01) and ESWL (-7.72{+-}3.87%) (p<0.05). The average change ratios of contralateral kidneys were as follows: PNL 4.80{+-}4.21% nephrolithotomy 4.67{+-}4.73%, pyelolithotomy -1.46{+-}5.39% and ESWL -2.02{+-}4.44%. One to 3 weeks after PNL, the cold area on the renal image was found in 10 (66.7%) of 15 cases. In cases of ESWL, DMSA renal uptake decreased even 4-10 weeks (mean 7 weeks) after treatment. In conclusion, possibility of deterioration of renal function after ESWL was suggested. (author).

Renal Osteodystrophy

Directory of Open Access Journals (Sweden)

Aynur Metin Terzibaşoğlu

2004-12-01

Full Text Available Chronic renal insufficiency is a functional definition which is characterized by irreversible and progressive decreasing in renal functions. This impairment is in collaboration with glomeruler filtration rate and serum creatinine levels. Besides this, different grades of bone metabolism disorders develop in chronic renal insufficiency. Pathologic changes in bone tissue due to loss of renal paranchyme is interrelated with calcium, phosphorus vitamine-D and parathyroid hormone. Clinically we can see high turnover bone disease, low turnover bone disease, osteomalacia, osteosclerosis and osteoporosis in renal osteodystropy. In this article we aimed to review pathology of bone metabolism disorders due to chronic renal insufficiency, clinic aspects and treatment approaches briefly.

Law & psychiatry: punishing juveniles who kill.

Science.gov (United States)

Appelbaum, Paul S

2012-10-01

Punishment of juvenile murderers forces policy makers to weigh the developmental immaturity of adolescents against the heinousness of their crimes. The U.S. Supreme Court has progressively limited the severity of punishments that can be imposed on juveniles, holding that their impulsivity, susceptibility to peer pressure, and more fluid character render them less culpable for their actions. Having eliminated the death penalty as a punishment, the Court recently struck down mandatory life sentences without prospect of parole. The decision is interesting for its emphasis on rehabilitation, opening the door to further restrictions on punitive sentences for juveniles-and perhaps for adults too.

CT differentiation of infiltrating renal cell carcinoma and renal urothelial tumor

International Nuclear Information System (INIS)

Choi, Hyo Kyeong; Goo, Dong Erk; Bang, Sun Woo; Lee, Moon Gyu; Cho, Kyoung Sik; Auh, Yong Ho

1994-01-01

It may be difficult to differentiate renal cell carcinoma involving collecting system from renal urothelial tumor invading into renal parenchyma. The purpose of this study was to assess the differences of CT findings between two conditions. CT findings of 5 cases of renal cell carcinoma involving the renal collecting systems and 10 cases of renal urothelial tumors invading the renal parenchyma were compared, and analyzed about the presence or absence of hydronephrosis, normal or abnormal CT nephrogram, renal contour changes due to mass and tentative diagnosis. The diagnoses were confirmed at surgery. Renal cell carcinoma showed hydronephrosis in only 20% and normal CT nephrogram and outward contour bulging in all cases. In contrast, renal urothelial tumor showed hydronephrosis(70%), abnormal CT nephrogram(60%), and preservation of reinform shape(100%). Renal contour changes and CT nephrogram may be useful in distinguishing both disease entities

Osteoporosis grave con aplastamientos vertebrales en dermatomiositis juvenil: Efecto del tratamiento con alendronato oral Severe osteoporosis with vertebral crushes in juvenile dermatomyositis: Effect of oral alendronate therapy

Directory of Open Access Journals (Sweden)

Cristina Tau

2007-02-01

Full Text Available Los glucocorticoides son usados comúnmente para el tratamiento de enfermedades inflamatorias, autoinmunes, enfermedades malignas, y en la prevención de rechazo de órganos trasplantados. Un efecto secundario frecuente del tratamiento prolongado es la pérdida de masa ósea que se produce por varios mecanismos y es causa de osteoporosis y fracturas vertebrales. El tratamiento con disfosfonatos ha sido propuesto para esta situación. Presentamos un caso clínico de osteoporosis grave en una niña con dermatomiositis juvenil, que respondió favorablemente al tratamiento con disfosfonatos orales.Glucocorticoids are used for the treatment of inflammatory and autoimmune diseases, cancer, and in prevention of organ rejects. A frequent secondary effect of longterm treatment with corticoids is the loss of bone mass, caused by several mechanisms: decrease in the intestinal calcium absorption, increase of the renal calcium excretion at the distal renal tubule, suppressive effect on the osteoblast and also in apoptosis of osteoclasts, inhibition in local production of IGF I (Insulin-like growth factor and IGFBPs (binding IGF I proteins necessary for bone metabolism, and decrease on osteocalcin production. Longterm treatment with corticoids is associated with osteoporosis and vertebral fractures. To improve this condition, treatment with bisphosphonates has been proposed. We present here a clinical case of a girl with dermatomyositis and severe osteoporosis with vertebral crushes, who responded well to oral bisphophonate treatment.

Development of consensus treatment plans for juvenile localized scleroderma: a roadmap toward comparative effectiveness studies in juvenile localized scleroderma.

Science.gov (United States)

Li, Suzanne C; Torok, Kathryn S; Pope, Elena; Dedeoglu, Fatma; Hong, Sandy; Jacobe, Heidi T; Rabinovich, C Egla; Laxer, Ronald M; Higgins, Gloria C; Ferguson, Polly J; Lasky, Andrew; Baszis, Kevin; Becker, Mara; Campillo, Sarah; Cartwright, Victoria; Cidon, Michael; Inman, Christi J; Jerath, Rita; O'Neil, Kathleen M; Vora, Sheetal; Zeft, Andrew; Wallace, Carol A; Ilowite, Norman T; Fuhlbrigge, Robert C

2012-08-01

Juvenile localized scleroderma (LS) is a chronic inflammatory skin disorder associated with substantial morbidity and disability. Although a wide range of therapeutic strategies has been reported in the literature, a lack of agreement on treatment specifics and accepted methods for clinical assessment has made it difficult to compare approaches and identify optimal therapy. Our objective was to develop standardized treatment plans, clinical assessments, and response criteria for active, moderate to high severity juvenile LS. A core group of pediatric rheumatologists, dermatologists, and a lay advisor was engaged by the Childhood Arthritis and Rheumatology Research Alliance (CARRA) to develop standardized treatment plans and assessment parameters for juvenile LS using consensus methods/nominal group techniques. Recommendations were validated in 2 face-to-face conferences with a larger group of practitioners with expertise in juvenile LS and with the full membership of CARRA, which encompasses the majority of pediatric rheumatologists in the US and Canada. Consensus was achieved on standardized treatment plans that reflect the prevailing treatment practices of CARRA members. Standardized clinical assessment methods and provisional treatment response criteria were also developed. Greater than 90% of pediatric rheumatologists responding to a survey (66% of CARRA membership) affirmed the final recommendations and agreed to utilize these consensus plans to treat patients with juvenile LS. Using consensus methodology, we have developed standardized treatment plans and assessment methods for juvenile LS. The high level of support among pediatric rheumatologists will support future comparative effectiveness studies and enable the development of evidence-based guidelines for the treatment of juvenile LS. Copyright © 2012 by the American College of Rheumatology.

The semantic sphere of juvenile offenders

Directory of Open Access Journals (Sweden)

Oshevsky D.S.

2017-01-01

Full Text Available The article presents the results of a preliminary empirical study aimed to identify features of the semantic sphere of adolescents who have committed illegal, including aggressive acts. The study included 50 male juveniles aged of 16 - 17 years. The first group consisted of adolescents convicted of aggressive and violent crimes; the second – of property socially dangerous acts (SDA. It is shown that evaluation of such adolescents is generally categorical and polar, the semantic field is subdifferentiable, less hierarchic, and has not enough realistic structure of meanings. Developed structure of motives and meanings is the basis of voluntary regulation of socially significant behavior. Thus, assessing the semantic sphere of juvenile offenders we can highlight its characteristics as risk factors of unlawful behavior, as well as the resource side, that will contribute to addressing issues of prevention and correction of unlawful behavior. Key words: juvenile offenders, semantic field of juvenile offenders, unlawful behavior.

The renal scan in pregnant renal transplant patients

International Nuclear Information System (INIS)

Goldstein, H.A.; Ziessman, H.A.; Fahey, F.H.; Collea, J.V.; Alijani, M.R.; Helfrich, G.B.

1985-01-01

With the greater frequency of renal transplant surgery, more female pts are becoming pregnant and carrying to term. In the renal allograft blood vessels and ureter may be compressed resulting in impaired renal function and/or, hypertension. Toxemia of pregnancy is seen more frequently than normal. Radionuclide renal scan monitoring may be of significant value in this high risk obstetrical pt. After being maintained during the pregnancy, renal function may also deteriorate in the post partum period. 5 pregnant renal transplant pts who delivered live babies had renal studies with Tc-99m DTPA to assess allograft perfusion and function. No transplanted kidney was lost during or after pregnancy as a result of pregnancy. No congenital anomalies were associated with transplant management. 7 studies were performed on these 5 pts. The 7 scans all showed the uterus/placenta. The bladder was always distorted. The transplanted kidney was rotated to a more vertical position in 3 pts. The radiation dose to the fetus is calculated at 0.024 rad/mCi administered. This study demonstrates the anatomic and physiologic alterations expected in the transplanted kidney during pregnancy when evaluated by renal scan and that the radiation burden may be acceptable in management of these pts

N-terminal pro-B-type Natriuretic Peptide in three different mechanisms of dysnatremia onset after a child's craniopharyngioma surgery.

Science.gov (United States)

Spatenkova, Vera; Hradil, Jan; Suchomel, Petr

2017-10-01

Craniopharyngioma, due to its sellar location, can be perioperatively complicated by different types of dysnatremia. We present a rare postoperative onset of a combination of three different mechanisms of dysnatremia with N-terminal pro-B-type Natriuretic Peptide (NT-proBNP) and renal function parameters in a boy with a good outcome after craniopharyngioma surgery: 1/ Central diabetes insipidus (CDI) onset immediately after the operation, hypernatremia with peak serum sodium (SNa) 158 mmol/l) caused by free water polyuria (electrolyte-free water clearance, EWC 0.104 ml/s), NT-proBNP 350 pg/ml; 2/ cerebral salt wasting (CSW) onset on day 7, hyponatremia (SNa 128 mmol/l) with hypoosmolality (measured serum osmolality, SOsm 265 mmol/kg) caused by natriuresis (sodium - daily output 605 mmol/day, fractional excretion 0.035), NT-proBNP 191 pg/ml; 3/ Polydypsia onset on day 11 caused hyponatremia (SNa 132 mmol/l), EWC 0.015, NT-proBNP 68 pg/ml.

Differential heritability of adult and juvenile antisocial traits.

Science.gov (United States)

Lyons, M J; True, W R; Eisen, S A; Goldberg, J; Meyer, J M; Faraone, S V; Eaves, L J; Tsuang, M T

1995-11-01

Studies of adult antisocial behavior or criminality usually find genetic factors to be more important than the family environment, whereas studies of delinquency find the family environment to be more important. We compared DSM-III-R antisocial personality disorder symptoms before vs after the age of 15 years within a sample of twins, rather than comparing across studies. We administered the Diagnostic Interview Schedule Version III-revised by telephone to 3226 pairs of male twins from the Vietnam Era Twin Registry. Biometrical modeling was applied to each symptom of antisocial personality disorder and summary measures of juvenile and adult symptoms. Five juvenile symptoms were significantly heritable, and five were significantly influenced by the shared environment. Eight adult symptoms were significantly heritable, and one was significantly influenced by the shared environment. The shared environment explained about six times more variance in juvenile anti-social traits than in adult traits. Shared environmental influences on adult antisocial traits overlapped entirely with those on juvenile traits. Additive genetic factors explained about six times more variance in adult vs juvenile traits. The juvenile genetic determinants overlapped completely with genetic influences on adult traits. The unique environment (plus measurement error) explained the largest proportion of variance in both juvenile and adult antisocial traits. Characteristics of the shared or family environment that promote antisocial behavior during childhood and early adolescence also promote later antisocial behavior, but to a much lesser extent. Genetic causal factors are much more prominent for adult than for juvenile antisocial traits.

Juveniles' Motivations for Remaining in Prostitution

Science.gov (United States)

Hwang, Shu-Ling; Bedford, Olwen

2004-01-01

Qualitative data from in-depth interviews were collected in 1990-1991, 1992, and 2000 with 49 prostituted juveniles remanded to two rehabilitation centers in Taiwan. These data are analyzed to explore Taiwanese prostituted juveniles' feelings about themselves and their work, their motivations for remaining in prostitution, and their difficulties…

DERMATOMIOSITIS JUVENIL Y EMBARAZO

OpenAIRE

Evans M,Gregorio; Poulsen R,Ronald; Blanco R,Romiely; Luna V,Viviana

2002-01-01

La dermatomiositis juvenil es un desorden inflamatorio crónico multisistémico del tejido conectivo. Tiene una incidencia de 2-3/100.000/año. Con la disminución en la mortalidad experimentada en los últimos decenios, la atención está cifrada en la morbilidad a largo plazo y en las alteraciones funcionales. Con un tratamiento agresivo los niños con dermatomiositis juvenil generalmente tienen un futuro promisorio, sin incapacidad o con incapacidad mínima. La mortalidad actualmente se estima cerc...

Importance of early audiologic assessment in distal renal tubular acidosis

Directory of Open Access Journals (Sweden)

Elizabeth Norgett

2010-12-01

Full Text Available Anand P Swayamprakasam1, Elizabeth Stover1, Elizabeth Norgett1, Katherine G Blake-Palmer1, Michael J Cunningham2, Fiona E Karet11Department of Medical Genetics, Cambridge Institute for Medical Research, Cambridge, UK; 2Department of Otolaryngology, Massachusetts Eye and Ear Infirmary, Boston, MA, USAAbstract: Autosomal recessive distal renal tubular acidosis is usually a severe disease of childhood, often presenting as failure to thrive in infancy. It is often, but not always, accompanied by sensorineural hearing loss, the clinical severity and age of onset of which may be different from the other clinical features. Mutations in either ATP6V1B1 or ATP6V0A4 are the chief causes of primary distal renal tubular acidosis with or without hearing loss, although the loss is often milder in the latter. We describe a kindred with compound heterozygous alterations in ATP6V0A4, where hearing loss was formally diagnosed late in both siblings such that they missed early opportunities for hearing support. This kindred highlights the importance of routine audiologic assessments of all children with distal renal tubular acidosis, irrespective either of age at diagnosis or of which gene is mutated. In addition, when diagnostic genetic testing is undertaken, both genes should be screened irrespective of current hearing status. A strategy for this is outlined.Keywords: sensorineural hearing loss, renal tubular acidosis, recessive, genetics, mutation

Primary Amyloidosis With Renal Involvement: Outcomes in 77 Consecutive Patients at a Single Center.

Science.gov (United States)

Wong, Sandy W; Toskic, Denis; Warner, Melissa; Varga, Cindy; Moreno-Koehler, Alejandro; Fein, Daniel; Fogaren, Teresa; Lee, Lisa; Oliver, Colin M; Guthrie, Spencer D; Comenzo, Raymond L

2017-11-01

Outcomes in primary amyloid renal patients are of interest as the era of monoclonal antibody therapies begins. We studied 77 consecutive primary amyloid renal patients (58% men) for renal progression (end stage renal disease [ESRD]), renal response (RR), and overall survival (OS). At diagnosis median age was 63 (range, 35-81) years, estimated glomerular filtration rate 70 mL/min (range, 5-114), difference between involved and uninvolved free light chains 127 mg/L (range, 1-9957), ESRD 4%, renal stage 2 and 3 78%, and cardiac stage 2 and 3 56%. Ninety-six percent received bortezomib and 44% stem cell transplantation as well as bortezomib, 68% achieved complete or very good partial hematologic response (CR/VGPR), 34% had ESRD, and 39% RR. Median times to ESRD and RR were 18 (range, 3-81) and 12 (range, 2-30) months, respectively. Median OS was not reached in this cohort and was not reached from onset of ESRD. More than two-thirds of patients with ESRD also achieved CR/VGPR. In those without ESRD at diagnosis, baseline creatinine and absent RR predicted progression to ESRD in multivariate Cox regression analysis, whereas CR/VGPR predicted RR. In multivariate Cox regression analysis, cardiac stage and achievement of CR/VGPR predicted OS, enabling construction of a prognostic model. Anti-plasma cell therapies provide a definite albeit limited benefit and new approaches to amyloid-related organ dysfunction are needed. Copyright © 2017 Elsevier Inc. All rights reserved.

Clinical characterization and proposed mechanism of juvenile glaucoma--a patient with a chromosome 4p deletion, Wolf-Hirschhorn Syndrome.

Science.gov (United States)

Curtin, Jeremy; Moloney, Greg; Grigg, John; Sharota Franzco, Dorian

2010-09-01

The case presented is that of a 22-year-old male with Wolf-Hirschhorn syndrome who was referred with glaucoma refractory to medical treatment. Six other patients have been described with Wolf-Hirschhorn syndrome (WHS) and glaucoma, most being congenital glaucoma with diagnosis in infancy. We describe the first case of juvenile onset glaucoma in this syndrome. Our patient had narrow angles on gonioscopy, with ultrasound biomicroscopy revealing ciliary body cysts. We alert others to the possibility of this mechanism of secondary narrow angle glaucoma associated with this chromosomal deletion syndrome.

Long-term renal toxicity in children following fractionated total-body irradiation (TBI) before allogeneic stem cell transplantation (SCT)

International Nuclear Information System (INIS)

Gerstein, Johanna; Meyer, Andreas; Fruehauf, Joerg; Karstens, Johann H.; Bremer, Michael; Sykora, Karl-Walter

2009-01-01

Purpose: to retrospectively assess the incidence and time course of renal dysfunction in children (≤ 16 years) following total-body irradiation (TBI) before allogeneic stem cell transplantation (SCT). Patients and methods: between 1986 and 2003, 92 children (median age, 11 years; range, 3-16 years) underwent TBI before allogeneic SCT. 43 of them had a minimum follow-up of 12 months (median, 51 months; range, 12-186 months) and were included into this analysis. Conditioning regimen included chemotherapy and fractionated TBI with 12 Gy (n = 26) or 11.1 Gy (n = 17). In one patient, renal dose was limited to 10 Gy by customized renal shielding due to known nephropathy prior to SCt. Renal dysfunction was defined as an increase of serum creatinine > 1.25 times the upper limit of age-dependent normal. Results: twelve children (28%) experienced an episode of renal dysfunction after a median of 2 months (range, 1-10 months) following SCT. In all but one patient renal dysfunction was transient and resolved after a median of 8 months (range, 3-16 months). One single patient developed persistent renal dysfunction with onset at 10 months after SCT. None of these patients required dialysis. The actuarial 3-year freedom from persistent renal toxicity for children surviving > 12 months after SCt was 97.3%. Conclusion: the incidence of persistent renal dysfunction after fractionated TBI with total doses ≤ 12 Gy was very low in this analysis. (orig.)

AFSC/ABL: Juvenile rockfish DNA species identification

Data.gov (United States)

National Oceanic and Atmospheric Administration, Department of Commerce — Many pelagic juvenile rockfish (Sebastes) were collected in juvenile salmonid surveys in the Gulf of Alaska (GOA) from 1998 to 2002. Often species identification of...

Renal perfusion scintiscan

Science.gov (United States)

... Radionuclide renal perfusion scan; Perfusion scintiscan - renal; Scintiscan - renal perfusion Images Kidney anatomy Kidney - blood and urine flow Intravenous pyelogram References Rottenberg G, Andi AC. Renal ...

Study of acute renal insufficiency and chronic renal insufficiency using radioisotopes

International Nuclear Information System (INIS)

Raynaud, C.

1976-01-01

Radioisotopic renal function tests are of assistance to diagnose and follow-up the course of renal insufficiency. The radioisotopic renogram is useful in assessing the response to therapy of child obstructive uropathies and evaluating renal transplant function. The renal scan is helpful, in an emergency service, to differenciate chronic renal insufficiency from acute renal insufficiency. Hg renal uptake test provides informations on physiopathological problems. Among them, the following problems are emphasized: evolution of a nonfunctioning kidney, control of the success of a reparative surgery and of bilateral obstructive uropathies with unilateral symptoms [fr

"Scared Straight" and other juvenile awareness programs for preventing juvenile delinquency.

Science.gov (United States)

Petrosino, A; Turpin-Petrosino, C; Buehler, J

2002-01-01

'Scared Straight' and other programmes involve organised visits to prison by juvenile delinquents or children at risk for criminal behavior. programmes are designed to deter participants from future offending through first-hand observation of prison life and interaction with adult inmates. These programmes remain in use world-wide despite studies and reviews questioning their effectiveness. To assess the effects of programmes comprising organised visits to prisons by juvenile delinquents (officially adjudicated or convicted by a juvenile court) or pre-delinquents (children in trouble but not officially adjudicated as delinquents), aimed at deterring them from criminal activity. Handsearching by the first author in identifying randomised field trials 1945-1993 relevant to criminology was augmented by structured searches of 16 electronic data bases, including the Campbell SPECTR database of trials and the Cochrane CCTR. Experts in the field were consulted and relevant citations were followed up. Studies that tested the effects of any program involving the organised visits of juvenile delinquents or children at-risk for delinquency to penal institutions were included. Studies that included overlapping samples of juvenile and young adults (e.g. ages 14-20) were included. We only considered studies that randomly or quasi-randomly (i.e. alternation) assigned participants to conditions. Each study had to have a no-treatment control condition with at least one outcome measure of "post-visit" criminal behavior. We report narratively on the nine eligible trials. We conducted one meta-analysis of post-intervention offending rates using official data. Information from other sources (e.g. self-report) was either missing from some studies or critical information was omitted (e.g. standard deviations). We examined the immediate post-treatment effects (i.e. "first-effects") by computing Odds Ratios (OR) for data on proportions of each group re-offending, and assumed both fixed and

Burden of childhood-onset arthritis

Directory of Open Access Journals (Sweden)

Hassett Afton L

2010-07-01

Full Text Available Abstract Juvenile arthritis comprises a variety of chronic inflammatory diseases causing erosive arthritis in children, often progressing to disability. These children experience functional impairment due to joint and back pain, heel pain, swelling of joints and morning stiffness, contractures, pain, and anterior uveitis leading to blindness. As children who have juvenile arthritis reach adulthood, they face possible continuing disease activity, medication-associated morbidity, and life-long disability and risk for emotional and social dysfunction. In this article we will review the burden of juvenile arthritis for the patient and society and focus on the following areas: patient disability; visual outcome; other medical complications; physical activity; impact on HRQOL; emotional impact; pain and coping; ambulatory visits, hospitalizations and mortality; economic impact; burden on caregivers; transition issues; educational occupational outcomes, and sexuality. The extent of impact on the various aspects of the patients', families' and society's functioning is clear from the existing literature. Juvenile arthritis imposes a significant burden on different spheres of the patients', caregivers' and family's life. In addition, it imposes a societal burden of significant health care costs and utilization. Juvenile arthritis affects health-related quality of life, physical function and visual outcome of children and impacts functioning in school and home. Effective, well-designed and appropriately tailored interventions are required to improve transitioning to adult care, encourage future vocation/occupation, enhance school function and minimize burden on costs.

Preventing Juvenile Delinquency

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Carolina dos Reis

2016-04-01

Full Text Available This article aims to problematize discourses about protection and care that have surrounded compulsory hospitalization by evidencing its use as a control and punishment mechanism that increases the social vulnerability of young drug users. For such, we analyze lawsuits involving juveniles who were consigned to psychiatric institutions for drug addiction treatment as a protection measure in the state of Rio Grande do Sul, in Brazil. The analysis of the materials has evidenced discourses that have circumscribed young drug users and constructed this population as potentially dangerous subjects as well as a population category at risk. In this sense, we point out how compulsory hospitalization has emerged out of the lawsuits as a tool for prevention of juvenile delinquency.

Exclusion of candidate genes from the chromosome 1q juvenile glaucoma region and mapping of the peripheral cannabis receptor gene (CNR2) to chromosome 1

Energy Technology Data Exchange (ETDEWEB)

Sunden, S.L.F.; Nichols, B.E.; Alward, W.L.M. [Univ. of Iowa, Iowa City, IA (United States)] [and others

1994-09-01

Juvenile onset primary open angle glaucoma has been mapped by linkage to 1q21-q31. Several candidate genes were evaluated in the same family used to identify the primary linkage. Atrionatriuretic peptide receptor A (NPR1) and laminin C1 (LAMC1) have been previously mapped to this region and could putatively play a role in the pathogenesis of glaucoma. A third gene, the peripheral cannabis receptor (CNR2) was not initially mapped in humans but was a candidate because of the relief that cannabis affords some patients with primary open angle glaucoma. Microsatellites associated with NPR1 and LAMC1 revealed multiple recombinations in affected members of this pedigree. CNR2 was shown to be on chromosome 1 by PCR amplification of a 150 bp fragment of the 3{prime} untranslated region in monochromosomal somatic cell hybrids (NIGMS panel No. 2). These primers also revealed a two allele single strand conformation polymorphism which showed multiple recombinants with juvenile onset primary open angle glaucoma in large pedigrees, segregating this disorder. The marker was then mapped to 1p34-p36 by linkage, with the most likely location between liver alkaline phosphatase (ALPL) and alpha-L-1 fucosidase (FUCA1).

Distal renal tubular acidosis in recurrent renal stone formers

DEFF Research Database (Denmark)

Osther, P J; Hansen, A B; Røhl, H F

1989-01-01

Renal acidification ability was examined in 90 recurrent renal stone formers, using fasting morning urinary pH levels followed by a short ammonium chloride loading test in subjects with pH levels above 6.0. Fifteen patients (16.6%) revealed a distal renal tubular acidification defect: one patient......, this has important therapeutic implications. The pathological sequence in renal stone formers with dRTA is discussed....

Exercise is More Effective at Altering Gut Microbial Composition and Producing Stable Changes in Lean Mass in Juvenile versus Adult Male F344 Rats.

Directory of Open Access Journals (Sweden)

Agnieszka Mika

Full Text Available The mammalian intestine harbors a complex microbial ecosystem that influences many aspects of host physiology. Exposure to specific microbes early in development affects host metabolism, immune function, and behavior across the lifespan. Just as the physiology of the developing organism undergoes a period of plasticity, the developing microbial ecosystem is characterized by instability and may also be more sensitive to change. Early life thus presents a window of opportunity for manipulations that produce adaptive changes in microbial composition. Recent insights have revealed that increasing physical activity can increase the abundance of beneficial microbial species. We therefore investigated whether six weeks of wheel running initiated in the juvenile period (postnatal day 24 would produce more robust and stable changes in microbial communities versus exercise initiated in adulthood (postnatal day 70 in male F344 rats. 16S rRNA gene sequencing was used to characterize the microbial composition of juvenile versus adult runners and their sedentary counterparts across multiple time points during exercise and following exercise cessation. Alpha diversity measures revealed that the microbial communities of young runners were less even and diverse, a community structure that reflects volatility and malleability. Juvenile onset exercise altered several phyla and, notably, increased Bacteroidetes and decreased Firmicutes, a configuration associated with leanness. At the genus level of taxonomy, exercise altered more genera in juveniles than in the adults and produced patterns associated with adaptive metabolic consequences. Given the potential of these changes to contribute to a lean phenotype, we examined body composition in juvenile versus adult runners. Interestingly, exercise produced persistent increases in lean body mass in juvenile but not adult runners. Taken together, these results indicate that the impact of exercise on gut microbiota

Effects of chronic fructose overload on renal dopaminergic system: alteration of urinary L-dopa/dopamine index correlates to hypertension and precedes kidney structural damage.

Science.gov (United States)

Rukavina Mikusic, Natalia L; Kouyoumdzian, Nicolás M; Del Mauro, Julieta S; Cao, Gabriel; Trida, Verónica; Gironacci, Mariela M; Puyó, Ana M; Toblli, Jorge E; Fernández, Belisario E; Choi, Marcelo R

2018-01-01

Insulin resistance induced by a high-fructose diet has been associated to hypertension and renal damage. The aim of this work was to assess alterations in the urinary L-dopa/dopamine ratio over three time periods in rats with insulin resistance induced by fructose overload and its correlation with blood pressure levels and the presence of microalbuminuria and reduced nephrin expression as markers of renal structural damage. Male Sprague-Dawley rats were randomly divided into six groups: control (C) (C4, C8 and C12) with tap water to drink and fructose-overloaded (FO) rats (FO4, FO8 and FO12) with a fructose solution (10% w/v) to drink for 4, 8 and 12 weeks. A significant increase of the urinary L-dopa/dopamine ratio was found in FO rats since week 4, which positively correlated to the development of hypertension and preceded in time the onset of microalbuminuria and reduced nephrin expression observed on week 12 of treatment. The alteration of this ratio was associated to an impairment of the renal dopaminergic system, evidenced by a reduction in renal dopamine transporters and dopamine D1 receptor expression, leading to an overexpression and overactivation of the enzyme Na + , K + -ATPase with sodium retention. In conclusion, urinary L-dopa/dopamine ratio alteration in rats with fructose overload positively correlated to the development of hypertension and preceded in time the onset of renal structural damage. This is the first study to propose the use of the urinary L-dopa/dopamine index as marker of renal dysfunction that temporarily precedes kidney structural damage induced by fructose overload. Copyright © 2017 Elsevier Inc. All rights reserved.

Imaging MS in Toxicology: An Investigation of Juvenile Rat Nephrotoxicity Associated with Dabrafenib Administration

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Groseclose, M. Reid; Laffan, Susan B.; Frazier, Kendall S.; Hughes-Earle, Angela; Castellino, Stephen

2015-06-01

As part of an investigative nephrotoxicity study, kidney tissues from juvenile rats orally administered dabrafenib at different age intervals between postnatal day (PND) 7 to 35 were investigated by MALDI and LDI imaging mass spectrometry (IMS) to determine the chemical composition of tubular deposits. In the youngest age group (PND 7-13), MALDI IMS demonstrated that a dabrafenib carboxylic acid metabolite was diffusely localized to the regions of tubular deposits (medulla and corticomedullary junction); however, no dabrafenib-related material was detected directly from the deposits. Rather, the LDI IMS analysis determined that the deposits were composed primarily of calcium phosphate. Based on these data, the dabrafenib associated nephrotoxicity, including the formation of tubular deposits, was determined to be age dependent. Furthermore, immature renal function was hypothesized to be responsible for the susceptibility of the youngest pups.

Screening renal stone formers for distal renal tubular acidosis

DEFF Research Database (Denmark)

Osther, P J; Hansen, A B; Røhl, H F

1989-01-01

A group of 110 consecutive renal stone formers were screened for distal renal tubular acidosis (RTA) using morning fasting urinary pH (mfUpH) levels followed by a short ammonium chloride loading test in patients with levels above 6.0. In 14 patients (12.7%) a renal acidification defect was noted...... RTA in renal stone formers. Regardless of whether the acidification defect is primary or secondary to stone formation, however, all renal stone formers with distal RTA can expect to benefit from prophylactic alkaline therapy and it is recommended that the screening procedure, which is easy to use...

Complications of percutaneous renal tumor biopsy: An analysis of 340 consecutive biopsies

DEFF Research Database (Denmark)

René Rasmussen, Lars; Loft, Martina; Høyer, Søren

Purpose Ultrasound Guided Percutaneous Kidney Biopsy (UGPKB) plays a major role in diagnosis of renal tumours. There seems to be little consensus regarding post-biopsy observation period. We aim to identify complications in UGPKB among outpatients with a suspected malignant renal lesion as well...... as the timing of onset of these complications, helping to clarify the optimal observation period. Many studies in this field suggest a lower complication risk for outpatients compared to hospitalized patients. In the latter group, an observation period of 24h after biopsy is often recommended. Material...... discrepancy. Results As for one third of the patients, analysed up until now, we find a total of one major complication and a few minor, all arisen within less than 6 hours after biopsy. Conclusions Rates of both major and minor complications in UGPKB are very low suggesting a shorter observation period...

Acute renal infarction Secondary to Atrial Fibrillation Mimicking Renal Stone Picture

International Nuclear Information System (INIS)

Salih, Salih Bin; Al-Durihim, H.; Al-Jizeeri, A.; Al-Maziad, G.

2006-01-01

Acute renal infarction presents in a similar clinical picture to that of a renal stone. We report a 55-year-old Saudi female, known to have atrial fibrillation secondary to mitral stenosis due to rheumatic heart disease. She presented with a two day history of right flank pain that was treated initially as renal stone. Further investigations confirmed her as a case of renal infarction. Renal infarction is under-diagnosed because the similarity of its presentation to renal stone. Renal infarction should be considered in the differential diagnosis of loin pain, particularly in a patient with atrial fibrillation. (author)

Tophaceous gout of the lumbar spine in a renal transplant patient: a case report and literature review

International Nuclear Information System (INIS)

Thornton, Frank J.; Torreggiani, William C.; Brennan, Paul

2000-01-01

Tophaceous gout of the spine is rare. We report the case of a 27-year-old male, renal transplant recipient, who presented with an acute onset of lower back pain. Radiological, biochemical and histo-pathological findings confirmed a diagnosis of tophaceous gout of the lumbar spine. We present the case history with the radiological findings and discuss the literature

[Neuroendocrine mechanisms of puberty onset].

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Teinturier, C

2002-10-01

An increase in pulsatile release of GnRH is essential for the onset of puberty. However, the mechanism controlling the pubertal increase in GnRH release is still unclear. The GnRH neurosecretory system is already active during the neonatal period but subsequently enters a dormant state by central inhibition in the juvenile period. When this central inhibition is removed or diminished, an increase in GnRH release occurs with increase in synthesis and release of gonadotropins and gonadal steroids, followed by the appearance of secondary sexual characteristics. Recent studies suggest that disinhibition of GnRH neurons from GABA (gamma-aminobutyric acid) appears to be a critical factor in female rhesus monkey. After central inhibition is removed, increases in stimulatory input from glutamatergic neurons as well as new stimulatory input from norepinephrine and NPY neurons and inhibitory input from beta endorphin neurons appear to control pulsatile GnRH release as well as gonadal steroids. Nonetheless, the most important question still remains: what determines the timing to remove central inhibition? Because many genes are turned on or turned off to establish a complex series of events occurring during puberty, the timing of puberty must be regulated by a master gene or genes, as a part of developmental events.

The World of Juvenile Justice According to the Numbers

Science.gov (United States)

Rozalski, Michael; Deignan, Marilyn; Engel, Suzanne

2008-01-01

Intended to be an instructive, yet sobering, introduction to the complex and disturbing nature of the juvenile justice system, this article details the "numbers," including selected percentages, ratios, and dollar amounts, that are relevant to developing a better understanding of the juvenile justice system. General statistics about juvenile and…

HIV testing among non-incarcerated substance-abusing juvenile offenders.

Science.gov (United States)

Tolou-Shams, Marina; Conrad, Selby; Louis, Alaina; Shuford, Sarah Hart; Brown, Larry K

2015-11-01

Juvenile offenders are a subgroup of adolescents at particular risk for HIV/STI infection. Although HIV prevalence among these youth is low (justice system, which is known to have an extremely high rate of HIV infection. US constitutional mandates provide HIV/STI testing for incarcerated juveniles, but close to 80% of juvenile arrestees are never detained. Moreover, although they engage in similar HIV risk behaviors as those detained, they have limited access to available HIV/STI testing services. Thus, our study examined rates of lifetime HIV testing among a pilot sample of 60 court-involved, substance-using juveniles monitored in the community to explore rates of testing and the reasons related to lifetime testing among a high-risk, yet understudied US juvenile population.

Bacterial acquisition in juveniles of several broadcast spawning coral species.

Directory of Open Access Journals (Sweden)

Koty H Sharp

Full Text Available Coral animals harbor diverse microorganisms in their tissues, including archaea, bacteria, viruses, and zooxanthellae. The extent to which coral-bacterial associations are specific and the mechanisms for their maintenance across generations in the environment are unknown. The high diversity of bacteria in adult coral colonies has made it challenging to identify species-specific patterns. Localization of bacteria in gametes and larvae of corals presents an opportunity for determining when bacterial-coral associations are initiated and whether they are dynamic throughout early development. This study focuses on the early onset of bacterial associations in the mass spawning corals Montastraea annularis, M. franksi, M. faveolata, Acropora palmata, A. cervicornis, Diploria strigosa, and A. humilis. The presence of bacteria and timing of bacterial colonization was evaluated in gametes, swimming planulae, and newly settled polyps by fluorescence in situ hybridization (FISH using general eubacterial probes and laser-scanning confocal microscopy. The coral species investigated in this study do not appear to transmit bacteria via their gametes, and bacteria are not detectable in or on the corals until after settlement and metamorphosis. This study suggests that mass-spawning corals do not acquire, or are not colonized by, detectable numbers of bacteria until after larval settlement and development of the juvenile polyp. This timing lays the groundwork for developing and testing new hypotheses regarding general regulatory mechanisms that control bacterial colonization and infection of corals, and how interactions among bacteria and juvenile polyps influence the structure of bacterial assemblages in corals.

A Giant Juvenile Nasopharyngeal Angiofibroma

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Yüce, Salim; Uysal, İsmail Önder; Doğan, Mansur; Polat, Kerem; Şalk, İsmail; Müderris, Suphi

2012-01-01

Juvenile nasopharyngeal angiofibroma (JNA) are locally growing highly vascular tumours. They are treated primarily by surgical excision ranging from open approach to endoscopic approach. We presented a 20-year-old male with a giant nasopharyngeal juvenile angiofibroma obliterating the pterygopalatine fossa bilaterally, invasing the sphenoid bone and extending to the left nasal passage. His complaints were epistaxis and nasal obstruction. After embolization, the patient was treated surgically with endoscopic approach and discharged as cured without any complication. PMID:23714961

Combination of Juvenile Papillomatosis, Juvenile Fibroadenoma and Intraductal Carcinoma of the Breast in a 15-Year-old Girl.

Science.gov (United States)

Sedloev, Theophil; Bassarova, Assia; Angelov, Kostadin; Vasileva, Mariela; Asenov, Yavor

2015-09-01

The association of juvenile papillomatosis with carcinoma is proven, but very rare, as there exist several reported cases. A 15-year-old girl with no family history of breast cancer presented with two masses in the left breast. The excisional biopsy on both lumps revealed juvenile fibroadenoma and juvenile papillomatosis epithelial proliferation multiple cystic expanded channels. In some of these channels, cytological features of intraductal carcinoma were observed. We performed a full immunohistochemical examination of the juvenile papillomatosis. The patient refused any further surgical or adjuvant treatment. There are no signs of recurrence in the 15 year follow-up. This case is a diagnostic and therapeutic challenge, taking into account the patient's age and the controversial treatment recommendations. Good collaboration between surgeons and pathologists is essential for an accurate diagnostic process and aims to avoid under- or overtreatment. Copyright© 2015 International Institute of Anticancer Research (Dr. John G. Delinassios), All rights reserved.

Angiopoietin-2 Is an Early Indicator of Acute Pancreatic-Renal Syndrome in Patients with Acute Pancreatitis

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Mateusz Sporek

2016-01-01

Full Text Available Within the first week of the disease, acute kidney injury (AKI is among the most common causes of mortality in acute pancreatitis (AP. Recently, serum angiopoietin-2 (Ang-2 has been associated with hyperdynamic state of the systemic circulation. The aim of this study was to examine the associations between Ang-2 and the clinical AP severity during the first 72 hours of the disease, and organ disfunction, including AKI. Methods. Study included patients admitted to the surgery ward, diagnosed with AP. AKI was diagnosed according to KDIGO guidelines and renal failure according to modified Marshall scoring system. Ang-2 was determined in serum with ELISA. Results. AP was classified as mild (MAP in 71% of patients, moderately severe (MSAP in 22%, and severe (SAP in 8%. During the first 72 hours of AP, 11 patients developed AKI and 6 developed renal failure. Ang-2 at 24, 48, and 72 hours following the onset of AP symptoms significantly predicted SAP and MSAP, as well as AKI and renal failure. Also, Ang-2 significantly correlated with acute phase proteins as well as with the indicators of renal disfunction. Conclusions. Serum Ang-2 may be a relevant predictor of AP severity, in particular of the development of AP-renal syndrome.

Challenges and prospects of the juvenile justice administration in ...

African Journals Online (AJOL)

Juvenile justice administration in Nigeria is weak and has been given very little priority, despite Nigeria being signatory to the major international instruments relevant to the administration of juvenile justice. This is attributable to the history of the penal system of Nigeria, with laws guiding juvenile justice administration having ...

The Effects of Renal Denervation on Renal Hemodynamics and Renal Vasculature in a Porcine Model.

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Willemien L Verloop

Full Text Available Recently, the efficacy of renal denervation (RDN has been debated. It is discussed whether RDN is able to adequately target the renal nerves.We aimed to investigate how effective RDN was by means of functional hemodynamic measurements and nerve damage on histology.We performed hemodynamic measurements in both renal arteries of healthy pigs using a Doppler flow and pressure wire. Subsequently unilateral denervation was performed, followed by repeated bilateral hemodynamic measurements. Pigs were terminated directly after RDN or were followed for 3 weeks or 3 months after the procedure. After termination, both treated and control arteries were prepared for histology to evaluate vascular damage and nerve damage. Directly after RDN, resting renal blood flow tended to increase by 29±67% (P = 0.01. In contrast, renal resistance reserve increased from 1.74 (1.28 to 1.88 (1.17 (P = 0.02 during follow-up. Vascular histopathology showed that most nerves around the treated arteries were located outside the lesion areas (8±7 out of 55±25 (14% nerves per pig were observed within a lesion area. Subsequently, a correlation was noted between a more impaired adventitia and a reduction in renal resistance reserve (β: -0.33; P = 0.05 at three weeks of follow-up.Only a small minority of renal nerves was targeted after RDN. Furthermore, more severe adventitial damage was related to a reduction in renal resistance in the treated arteries at follow-up. These hemodynamic and histological observations may indicate that RDN did not sufficiently target the renal nerves. Potentially, this may explain the significant spread in the response after RDN.

The Effects of Renal Denervation on Renal Hemodynamics and Renal Vasculature in a Porcine Model

Science.gov (United States)

Verloop, Willemien L.; Hubens, Lisette E. G.; Spiering, Wilko; Doevendans, Pieter A.; Goldschmeding, Roel; Bleys, Ronald L. A. W.; Voskuil, Michiel

2015-01-01

Rationale Recently, the efficacy of renal denervation (RDN) has been debated. It is discussed whether RDN is able to adequately target the renal nerves. Objective We aimed to investigate how effective RDN was by means of functional hemodynamic measurements and nerve damage on histology. Methods and Results We performed hemodynamic measurements in both renal arteries of healthy pigs using a Doppler flow and pressure wire. Subsequently unilateral denervation was performed, followed by repeated bilateral hemodynamic measurements. Pigs were terminated directly after RDN or were followed for 3 weeks or 3 months after the procedure. After termination, both treated and control arteries were prepared for histology to evaluate vascular damage and nerve damage. Directly after RDN, resting renal blood flow tended to increase by 29±67% (P = 0.01). In contrast, renal resistance reserve increased from 1.74 (1.28) to 1.88 (1.17) (P = 0.02) during follow-up. Vascular histopathology showed that most nerves around the treated arteries were located outside the lesion areas (8±7 out of 55±25 (14%) nerves per pig were observed within a lesion area). Subsequently, a correlation was noted between a more impaired adventitia and a reduction in renal resistance reserve (β: -0.33; P = 0.05) at three weeks of follow-up. Conclusion Only a small minority of renal nerves was targeted after RDN. Furthermore, more severe adventitial damage was related to a reduction in renal resistance in the treated arteries at follow-up. These hemodynamic and histological observations may indicate that RDN did not sufficiently target the renal nerves. Potentially, this may explain the significant spread in the response after RDN. PMID:26587981

The investigation of genetic and clinical features in Chinese patients with juvenile amyotrophic lateral sclerosis.

Science.gov (United States)

Liu, Z-J; Lin, H-X; Liu, G-L; Tao, Q-Q; Ni, W; Xiao, B-G; Wu, Z-Y

2017-09-01

Juvenile amyotrophic lateral sclerosis (JALS) occurs at an age of onset below 25 years with a heterogeneous disease onset location, variable progression and survival time. To investigate whether an ALS gene profile could resolve any aspects of clinical symptom heterogeneity, we have used targeted sequencing technology in a cohort of 12 JALS patients of Chinese descent. We detected 5 likely pathogenic mutations, 2 in familial probands and 3 in sporadic patients. One was a known TARDBP mutation (p.G348V) and 4 were FUS frameshift mutations including a known p.Gln519Ilefs*9 mutation and 3 novel mutations, p.Gly515Valfs*14, p.Gly486Profs*30, and p.Arg498Alafs*32. Of the 4 FUS mutations, 2 were able to be confirmed as de novo mutations. The TARDBP mutation carrier showed a classic ALS phenotype. All patients with FUS mutations experienced limb weakness at an early age and developed bulbar symptoms during the disease course. FUS mutations have previously been associated with increased JALS disease progression, however, we found a large range 12 to 84 months in disease survival (mean 58.2 months). Our results justify future screening for variants in FUS as it remains the most frequent genetic determinant of early onset, JALS (found in 30% of our patients). © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

The social competence and behavioral problem substrate of new- and recent-onset childhood epilepsy.

Science.gov (United States)

Almane, Dace; Jones, Jana E; Jackson, Daren C; Seidenberg, Michael; Hermann, Bruce P

2014-02-01

This study examined patterns of syndrome-specific problems in behavior and competence in children with new- or recent-onset epilepsy compared with healthy controls. Research participants consisted of 205 children aged 8-18, including youth with recent-onset epilepsy (n=125, 64 localization-related epilepsy [LRE] and 61 idiopathic generalized epilepsy [IGE]) and healthy first-degree cousin controls (n=80). Parents completed the Child Behavior Checklist for children aged 6-18 (CBCL/6-18) from the Achenbach System of Empirically Based Assessment (ASEBA). Dependent variables included Total Competence, Total Problems, Total Internalizing, Total Externalizing, and Other Problems scales. Comparisons of children with LRE and IGE with healthy controls were examined followed by comparisons of healthy controls with those having specific epilepsy syndromes of LRE (BECTS, Frontal/Temporal Lobe, and Focal NOS) and IGE (Absence, Juvenile Myoclonic, and IGE NOS). Children with LRE and/or IGE differed significantly (pcompetence (Total Competence including School and Social). Similarly, children with specific syndromes of LRE and IGE differed significantly (pcompetence (Total Competence including School). Only on the Thought Problems scale were there syndrome differences. In conclusion, children with recent-onset epilepsy present with significant behavioral problems and lower competence compared with controls, with little syndrome specificity whether defined broadly (LRE and IGE) or narrowly (specific syndromes of LRE and IGE). Copyright © 2013 Elsevier Inc. All rights reserved.

Renal pathological implications in type 2 diabetes mellitus patients with renal involvement.

Science.gov (United States)

Li, Li; Zhang, Xiuhui; Li, Zhicheng; Zhang, Rui; Guo, Ruikun; Yin, Qinghua; Yang, Lichuan; Yue, Rongzheng; Su, Baihai; Huang, Songmin; Xu, Huan; He, Cijiang; Liu, Fang

2017-01-01

To investigate the renal pathological implications in type 2 diabetes mellitus patients with renal involvement. A total of 328 type 2 diabetes mellitus (T2DM) patients with renal involvement who underwent a renal biopsy and received follow-up for at least one year were recruited in our study. The patients were divided into the diabetic nephropathy (DN), non-diabetic renal disease (NDRD), and NDRD superimposed on DN groups based on the pathological diagnosis. Renal outcomes were defined by the initiation of renal replacement therapy or doubling of the serum creatinine. Kaplan-Meier analysis was used to compare renal survival, and Cox proportional hazard analysis was used to determine the predictors of renal outcomes in the DN group. Renal biopsy findings revealed that 188 patients (57.32%) had pure DN, 121 patients (36.89%) had NDRD alone, and 19 patients (5.79%) had NDRD superimposed on DN. The most frequent subclassification of NDRD was membranous nephropathy (MN). Compared with the NDRD and NDRD superimposed on DN groups, patients with pure DN had poorer renal function and lower renal survival rates. In the DN group, the five-year renal survival rates of glomerular classes of I, IIa, IIb, III and IV were 100%, 84.62%, 60%, 47.5% and 33.33%, respectively. Multivariate Cox proportional hazard analysis showed that the glomerular lesions, proteinuria and serum creatinine were independent risk factors for renal outcomes, while interstitial fibrosis/inflammation and arteriolar hyalinosis were not independently associated with renal outcomes in the DN group. Making an accurate pathologic diagnosis by renal biopsy is crucial for diabetes mellitus (DM) patients with renal involvement. The findings of our present study indicated that patients with pure DN had poorer renal outcomes than patients with NDRD or NDRD superimposed on DN. The classification of glomerular lesions, proteinuria and serum creatinine were independent risk factors for renal outcomes in the DN group

A new mass mortality of juvenile Protoceratops and size-segregated aggregation behaviour in juvenile non-avian dinosaurs.

Directory of Open Access Journals (Sweden)

David W E Hone

Full Text Available BACKGROUND: Monodominant bonebeds are a relatively common occurrence for non-avian dinosaurs, and have been used to infer associative, and potentially genuinely social, behavior. Previously known assemblages are characterized as either mixed size-classes (juvenile and adult-sized specimens together or single size-classes of individuals (only juveniles or only adult-sized individuals within the assemblage. In the latter case, it is generally unknown if these kinds of size-segregated aggregations characterize only a particular size stage or represent aggregations that happened at all size stages. Ceratopsians ("horned dinosaurs" are known from both types of assemblages. METHODS/PRINCIPAL FINDINGS: Here we describe a new specimen of the ceratopsian dinosaur Protoceratops andrewsi, Granger and Gregory 1923 from Mongolia representing an aggregation of four mid-sized juvenile animals. In conjunction with existing specimens of groups of P. andrewsi that includes size-clustered aggregations of young juveniles and adult-sized specimens, this new material provides evidence for some degree of size-clustered aggregation behaviour in Protoceratops throughout ontogeny. This continuity of size-segregated (and presumably age-clustered aggregation is previously undocumented in non-avian dinosaurs. CONCLUSIONS: The juvenile group fills a key gap in the available information on aggregations in younger ceratopsians. Although we support the general hypothesis that many non-avian dinosaurs were gregarious and even social animals, we caution that evidence for sociality has been overstated and advocate a more conservative interpretation of some data of 'sociality' in dinosaurs.

A new mass mortality of juvenile Protoceratops and size-segregated aggregation behaviour in juvenile non-avian dinosaurs.

Science.gov (United States)

Hone, David W E; Farke, Andrew A; Watabe, Mahito; Shigeru, Suzuki; Tsogtbaatar, Khishigjav

2014-01-01

Monodominant bonebeds are a relatively common occurrence for non-avian dinosaurs, and have been used to infer associative, and potentially genuinely social, behavior. Previously known assemblages are characterized as either mixed size-classes (juvenile and adult-sized specimens together) or single size-classes of individuals (only juveniles or only adult-sized individuals within the assemblage). In the latter case, it is generally unknown if these kinds of size-segregated aggregations characterize only a particular size stage or represent aggregations that happened at all size stages. Ceratopsians ("horned dinosaurs") are known from both types of assemblages. Here we describe a new specimen of the ceratopsian dinosaur Protoceratops andrewsi, Granger and Gregory 1923 from Mongolia representing an aggregation of four mid-sized juvenile animals. In conjunction with existing specimens of groups of P. andrewsi that includes size-clustered aggregations of young juveniles and adult-sized specimens, this new material provides evidence for some degree of size-clustered aggregation behaviour in Protoceratops throughout ontogeny. This continuity of size-segregated (and presumably age-clustered) aggregation is previously undocumented in non-avian dinosaurs. The juvenile group fills a key gap in the available information on aggregations in younger ceratopsians. Although we support the general hypothesis that many non-avian dinosaurs were gregarious and even social animals, we caution that evidence for sociality has been overstated and advocate a more conservative interpretation of some data of 'sociality' in dinosaurs.

Detection of acute renal allograft rejection by analysis of renal tissue proteomics in rat models of renal transplantation

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Dai Yong

2008-01-01

Full Text Available At present, the diagnosis of renal allograft rejection requires a renal biopsy. Clinical management of renal transplant patients would be improved if rapid, noninvasive and reliable biomarkers of rejection were available. This study is designed to determine whether such protein biomarkers can be found in renal-graft tissue proteomic approach. Orthotopic kidney transplantations were performed using Fisher (F344 or Lewis rats as donors and Lewis rats as recipients. Hence, there were two groups of renal transplant models: one is allograft (from F344 to Lewis rats; another is syngrafts (from Lewis to Lewis rats serving as control. Renal tissues were collected 3, 7 and 14 days after transplantation. As many as 18 samples were analyzed by 2-D Electrophoresis and mass spectrometry (MALDI-TOF-TOF-MS. Eleven differentially expressed proteins were identified between groups. In conclusion, proteomic technology can detect renal tissue proteins associated with acute renal allograft rejection. Identification of these proteins as diagnostic markers for rejection in patients′ urine or sera may be useful and non-invasive, and these proteins might serve as novel therapeutic targets that also help to improve the understanding of mechanism of renal rejection.

Renal sympathetic denervation: MDCT evaluation of the renal arteries.

LENUS (Irish Health Repository)

Hutchinson, Barry D

2013-08-01

Percutaneous transluminal renal sympathetic denervation is a new treatment of refractory systemic hypertension. The purpose of this study was to assess the clinical utility of MDCT to evaluate the anatomic configuration of the renal arteries in the context of renal sympathetic denervation.

Sports Participation and Juvenile Delinquency: A Meta-Analytic Review.

Science.gov (United States)

Spruit, Anouk; van Vugt, Eveline; van der Put, Claudia; van der Stouwe, Trudy; Stams, Geert-Jan

2016-04-01

Participation in sports activities is very popular among adolescents, and is frequently encouraged among youth. Many psychosocial health benefits in youth are attributed to sports participation, but to what extent this positive influence holds for juvenile delinquency is still not clear on both the theoretical and empirical level. There is much controversy on whether sports participation should be perceived as a protective or a risk factor for the development of juvenile delinquency. A multilevel meta-analysis of 51 published and unpublished studies, with 48 independent samples containing 431 effect sizes and N = 132,366 adolescents, was conducted to examine the relationship between sports participation and juvenile delinquency and possible moderating factors of this association. The results showed that there is no overall significant association between sports participation and juvenile delinquency, indicating that adolescent athletes are neither more nor less delinquent than non-athletes. Some study, sample and sports characteristics significantly moderated the relationship between sports participation and juvenile delinquency. However, this moderating influence was modest. Implications for theory and practice concerning the use of sports to prevent juvenile delinquency are discussed.

Moral development of solo juvenile sex offenders

NARCIS (Netherlands)

van Vugt, E.; Stams, G.J.; Dekovic, M.; Brugman, D.; Rutten, E.; Hendriks, J.

2008-01-01

This study compared the moral development of solo juvenile male sex offenders (n = 20) and juvenile male non-offenders (n = 76), aged 13-19 years, from lower socioeconomic and educational backgrounds. The Moral Orientation Measure (MOM) was used to assess punishment- and victim-based moral

Recurrent reciprocal genomic rearrangements of 17q12 are associated with renal disease, diabetes, and epilepsy.

Science.gov (United States)

Mefford, Heather C; Clauin, Severine; Sharp, Andrew J; Moller, Rikke S; Ullmann, Reinhard; Kapur, Raj; Pinkel, Dan; Cooper, Gregory M; Ventura, Mario; Ropers, H Hilger; Tommerup, Niels; Eichler, Evan E; Bellanne-Chantelot, Christine

2007-11-01

Most studies of genomic disorders have focused on patients with cognitive disability and/or peripheral nervous system defects. In an effort to broaden the phenotypic spectrum of this disease model, we assessed 155 autopsy samples from fetuses with well-defined developmental pathologies in regions predisposed to recurrent rearrangement, by array-based comparative genomic hybridization. We found that 6% of fetal material showed evidence of microdeletion or microduplication, including three independent events that likely resulted from unequal crossing-over between segmental duplications. One of the microdeletions, identified in a fetus with multicystic dysplastic kidneys, encompasses the TCF2 gene on 17q12, previously shown to be mutated in maturity-onset diabetes, as well as in a subset of pediatric renal abnormalities. Fine-scale mapping of the breakpoints in different patient cohorts revealed a recurrent 1.5-Mb de novo deletion in individuals with phenotypes that ranged from congenital renal abnormalities to maturity-onset diabetes of the young type 5. We also identified the reciprocal duplication, which appears to be enriched in samples from patients with epilepsy. We describe the first example of a recurrent genomic disorder associated with diabetes.

Relationship between renal cortex and parenchyma thickness and renal function: study with CT measurement

International Nuclear Information System (INIS)

Xu Yufeng; Tang Guangjian; Jiang Xuexiang

2006-01-01

Objective: To study the relationship between renal morphology and renal function, and to assess the value of CT as a criterion to grade renal function. Methods: Enhancement CT were performed in 89 patients with no local renal disease whose split renal glomerular filtration rates (GFR) were measured by renal dynamic imaging with 99 Tc m -DTPA. The 178 kidneys were divided into normal renal function, mild and severe renal impairment groups according to renal function. Differences between three groups respect to the mean thickness of renal cortex and parenchyma were assessed by ANOVA. Using Pearson's correlation test, the correlation between the renal cortex, parenchyma thicknesses and renal GFR were examined. The value of CT in predicting renal function was assessed by using ROC analysis. Results: The renal cortex thicknesses of normal renal function, mild and severe renal impairment groups were (5.9±1.1), (4.6± 1.1), and (3.3±1.0) mm respectively, and the renal parenchyma thicknesses were (26.3±4.2), (21.3±4.6), (16.2±4.6) mm. There were significant differences of renal cortex, parenchyma thicknesses between 3 groups (cortex F=54.78, P<0.01; parenehyma F=43.90, P<0.01). The thicknesses of renal cortex (r=0.752, P<0.01), parenchyma (r=0.738, P<0.01) had positive linear correlation with renal function. ROC analysis of the renal cortex thicknesses measured by CT in predicting mild and severe renal impairment showed that the Az was 0.860 and 0.905 respectively, whereas that of parenchyma was 0.868 and 0.884. Conclusion: The thicknesses of renal cortex, parenchyma measured by CT can reflect renal function. CT was a supplementary method to assess renal function. (authors)

Glomerular Filtration Rate Estimation in Renal and Non-Renal Solid Organ Transplantation

DEFF Research Database (Denmark)

Hornum, Mads; Feldt-Rasmussen, Bo

2017-01-01

Following transplantation (TX) of both renal and non-renal organs, a large proportion of patients have renal dysfunction. There are multiple causes for this. Chronic nephrotoxicity and high doses of calcineurin inhibitors are important factors. Preoperative and perioperative factors like...... or estimates of renal function in these patients, in order to accurately and safely dose immunosuppressive medication and perform and adjust the treatment and prophylaxis of renal dysfunction. This is a short overview and discussion of relevant studies and possible caveats of estimated glomerular filtration...... rate methods for use in renal and non-renal TX....

Superselective transcatheter renal arterial embolization for acute renal bleeding in patients with renal insufficiency: its clinical efficacy and safety

International Nuclear Information System (INIS)

Hu Tingyang; Zhou Bing; Yu Wenqiang; Luo Zuyan; Mao Yingmin; Chen Fanghong; Li Bo; Yuan Jianhua

2010-01-01

Objective: To discuss the clinical efficacy and complications of super selective renal arterial embolization in treating acute renal arterial bleeding in patients with renal insufficiency, and to evaluate the influence of the treatment on the renal function. Methods: During the period of January 2000 December 2009, super selective renal arterial embolization was performed in our institution for acute renal bleeding in 13 patients with renal insufficiency. The complete clinical and imaging materials of all patients were properly collected. The clinical effectiveness, the renal function, the extent of embolization and the complications were observed and the relationship between each other was analyzed. Results: The embolization procedure was successfully completed in all patients with a technical success rate of 100%. The mean embolized territory was 22% of a single kidney. Three days after the procedure, the hemoglobin level, hematocrit, blood pressure and heart rate were considerably improved in all patients. Compared to the corresponding preoperative data, all the differences were statistically significant (P 0.05), while the blood urea nitrogen was markedly decreased (P=0.011). Post embolization syndrome occurred in 5 patients and progressive aggravation of the renal function was observed in one patient, who had to receive hemodialysis finally. The embolized territory in patients occurring complications was larger than that in patients without occurring complications (U=1.500, P=0.006). Conclusion: Super selective renal arterial embolization is an effective and safe treatment for acute renal arterial bleeding in patients with renal insufficiency, the therapy will not significantly worsen the renal function. Appropriate and reasonable extent of embolization, as small as possible, is the key point for reducing the complications. (authors)

Very early-onset schizophrenia with secondary onset tic disorder

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Shilpa A Telgote; Shreyas Shrikant Pendharkar; Amol D Kelkar; Sachin Bhojane

2017-01-01

Very early-onset schizophrenia (defined as an onset of psychosis before 13 years of age) is a rare and severe form of the disorder which is clinically and neurobiologically continuous with the adult-onset disorder. It is rarely reported

Metabolic factors in the development of retinopathy of juvenile-onset type I diabetes mellitus

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Khosla P

1994-01-01

Full Text Available Thirty-five patients of insulin-dependent diabetes mellitus (IDDM were investigated for the effect of various metabolic factors on retinopathy. The severity of retinopathy increased with duration and age of onset of IDDM. Degree of glycaemia (fasting blood sugar, FBS was similar in patients with or without retinopathy. All IDDM patients as a group showed severe carbohydrate intolerance with lower basal and post glucose serum immunoreactive insulin (IRI levels and serum C-peptide radioimmunoreactivity (CPR as compared to controls. The insulin secretory response was similar in no retinopathy, mild retinopathy and severe retinopathy groups. Patients with retinopathy had higher incidence of hyperlipidemia but mean serum levels of cholesterol and triglyceride were similar. This study does not suggest a direct relationship between the various metabolic factors studied and retinopathy due to IDDM

Very Early-onset Schizophrenia with Secondary Onset Tic Disorder.

Science.gov (United States)

Telgote, Shilpa A; Pendharkar, Shreyas Shrikant; Kelkar, Amol D; Bhojane, Sachin

2017-01-01

Very early-onset schizophrenia (defined as an onset of psychosis before 13 years of age) is a rare and severe form of the disorder which is clinically and neurobiologically continuous with the adult-onset disorder. It is rarely reported tic disorder.

Detection of acute renal allograft rejection by analysis of Renal TissueProteomics in rat models of renal transplantation

International Nuclear Information System (INIS)

Dai, Y.; Lv, T.; Wang, K.; Li, D.; Huang, Y.; Liu, J.

2008-01-01

At present, the diagnosis of renal allograft rejection requires a renalbiopsy. Clinical management of renal transplant patients would be improved ifrapid, noninvasive and reliable biomarkers of rejection were available. Thisstudy is designed to determine whether such protein biomarkers can be foundin renal graft tissue proteomic approach. Orthotopic kidney transplantationswere performed using Fisher (F344) or Lewis rats as donors and Lewis rats asrecipients. Hence, there were two groups of renal transplant models: one isallograft (from F344 to Lewis rats); another is syngrafts (from Lewis toLewis rats) serving as control. Renal tissues were collected 3, 7 and 14 daysafter transplantation. As many 18 samples were analyzed by 2-DElectrophoresis and mass spectrometry (MALDI-TOF-TOF-MS). Elevendifferentially expressed proteins were identified between groups. Inconclusion, proteomic technology can detect renal tissue proteins associatedwith acute renal allograft rejection. Identification of these proteins asdiagnostic markers for rejection in patient's urine or sera may be useful andnon-invasive, and these proteins might serve as novel therapeutic targetsthat also help to improve the understanding of mechanisms of renal rejection.(author)

Anatomic Patterns of Renal Arterial Sympathetic Innervation: New Aspects for Renal Denervation.

Science.gov (United States)

Imnadze, Guram; Balzer, Stefan; Meyer, Baerbel; Neumann, Joerg; Krech, Rainer Horst; Thale, Joachim; Franz, Norbert; Warnecke, Henning; Awad, Khaled; Hayek, Salim S; Devireddy, Chandan

2016-12-01

Initial studies of catheter-based renal arterial sympathetic denervation to lower blood pressure in resistant hypertensive patients renewed interest in the sympathetic nervous system's role in the pathogenesis of hypertension. However, the SYMPLICITY HTN-3 study failed to meet its prespecified blood pressure lowering efficacy endpoint. To date, only a limited number of studies have described the microanatomy of renal nerves, of which, only two involve humans. Renal arteries were harvested from 15 cadavers from the Klinikum Osnabruck and Schuchtermann Klinik, Bad Rothenfelde. Each artery was divided longitudinally in equal thirds (proximal, middle, and distal), with each section then divided into equal superior, inferior, anterior, and posterior quadrants, which were then stained. Segments containing no renal nerves were given a score value = 0, 1-2 nerves with diameter 4 nerves or nerve diameter ≥600 µm a score = 3. A total of 22 renal arteries (9 right-sided, 13 left-sided) were suitable for examination. Overall, 691 sections of 5 mm thickness were prepared. Right renal arteries had significantly higher mean innervation grade (1.56 ± 0.85) compared to left renal arteries (1.09 ± 0.87) (P renal artery has significantly higher innervation scores than the left. The anterior and superior quadrants of the renal arteries scored higher in innervation than the posterior and inferior quadrants did. The distal third of the renal arteries are more innervated than the more proximal segments. These findings warrant further evaluation of the spatial innervation patterns of the renal artery in order to understand how it may enhance catheter-based renal arterial denervation procedural strategy and outcomes. The SYMPLICITY HTN-3 study dealt a blow to the idea of the catheter-based renal arterial sympathetic denervation. We investigated the location and patterns of periarterial renal nerves in cadaveric human renal arteries. To quantify the density of the

Juvenil idiopatisk arthritis

DEFF Research Database (Denmark)

Herlin, Troels

2002-01-01

The new classification of juvenile idiopathic arthritis (JIA) is described in this review. Clinical characteristics divide JIA in to subtypes: systemic, oligoarticular (persistent and extended type), RF-positive and--negative polyarticular, enthesitis-related arthritis and psoriatic arthritis...

Orofacial pain, jaw function, and temporomandibular disorders in adult women with a history of juvenile chronic arthritis or persistent juvenile chronic arthritis

DEFF Research Database (Denmark)

Bakke, M.; Zak, M.; Jensen, B.L.

2001-01-01

Orofacial pain, jaw function, temporomandibular disorders, adult women persistent juvenil chronic arthritis......Orofacial pain, jaw function, temporomandibular disorders, adult women persistent juvenil chronic arthritis...

Renal Blood Flow, Glomerular Filtration Rate, and Renal Oxygenation in Early Clinical Septic Shock.

Science.gov (United States)

Skytte Larsson, Jenny; Krumbholz, Vitus; Enskog, Anders; Bragadottir, Gudrun; Redfors, Bengt; Ricksten, Sven-Erik

2018-06-01

Data on renal hemodynamics, function, and oxygenation in early clinical septic shock are lacking. We therefore measured renal blood flow, glomerular filtration rate, renal oxygen consumption, and oxygenation in patients with early septic shock. Prospective comparative study. General and cardiothoracic ICUs. Patients with norepinephrine-dependent early septic shock (n = 8) were studied within 24 hours after arrival in the ICU and compared with postcardiac surgery patients without acute kidney injury (comparator group, n = 58). None. Data on systemic hemodynamics and renal variables were obtained during two 30-minute periods. Renal blood flow was measured by the infusion clearance of para-aminohippuric acid, corrected for renal extraction of para-aminohippuric acid. Renal filtration fraction was measured by renal extraction of chromium-51 labeled EDTA. Renal oxygenation was estimated from renal oxygen extraction. Renal oxygen delivery (-24%; p = 0.037) and the renal blood flow-to-cardiac index ratio (-21%; p = 0.018) were lower, renal vascular resistance was higher (26%; p = 0.027), whereas renal blood flow tended to be lower (-19%; p = 0.068) in the septic group. Glomerular filtration rate (-32%; p = 0.006) and renal sodium reabsorption (-29%; p = 0.014) were both lower in the septic group. Neither renal filtration fraction nor renal oxygen consumption differed significantly between groups. Renal oxygen extraction was significantly higher in the septic group (28%; p = 0.022). In the septic group, markers of tubular injury were elevated. In early clinical septic shock, renal function was lower, which was accompanied by renal vasoconstriction, a lower renal oxygen delivery, impaired renal oxygenation, and tubular sodium reabsorption at a high oxygen cost compared with controls.

Juvenile myopia progression, risk factors and interventions.

Science.gov (United States)

Myrowitz, Elliott H

2012-07-01

The development and progression of early onset myopia is actively being investigated. While myopia is often considered a benign condition it should be considered a public health problem for its visual, quality of life, and economic consequences. Nearly half of the visually impaired population in the world has uncorrected refractive errors, with myopia a high percent of that group. Uncorrected visual acuity should be screened for and treated in order to improve academic performance, career opportunities and socio-economic status. Genetic and environmental factors contribute to the onset and progression of myopia. Twin studies have supported genetic factors and research continues to identify myopia genetic loci. While multiple myopia genetic loci have been identified establishing myopia as a common complex disorder, there is not yet a genetic model explaining myopia progression in populations. Environmental factors include near work, education levels, urban compared to rural location, and time spent outdoors. In this field of study where there continues to be etiology controversies, there is recent agreement that children who spend more time outdoors are less likely to become myopic. Worldwide population studies, some completed and some in progress, with a common protocol are gathering both genetic and environmental cohort data of great value. There have been rapid population changes in prevalence rates supporting an environmental influence. Interventions to prevent juvenile myopia progression include pharmacologic agents, glasses and contact lenses. Pharmacological interventions over 1-2 year trials have shown benefits. Peripheral vision defocus has been found to affect the emmetropization process and may be affected by wearing glasses or contacts. Accommodation accuracy also has been implicated in myopia progression. Further research will aim to assess both the role and interaction of environmental influences and genetic factors.

Juvenile Obesity, Physical Activity, and Lifestyle Changes.

Science.gov (United States)

Bar-Or, Oded

2000-01-01

Because many obese children become obese adults, the recent rapid increase in juvenile obesity poses a major public health challenge. Enhanced physical activity is a cornerstone in a multidisciplinary approach to preventing and treating juvenile obesity. Giving exercise recommendations focused for obese youth is critical. Cutting down on sedentary…

The 64-MSCT study of relationship between renal corticomedullary differentiation, contrast between renal cortex and medulla, renal cortex and medulla CT peak value with the single renal function in hydronephrotic kidney

International Nuclear Information System (INIS)

Wang Yunhua; Hou Weiwei; Liu Ruihong; He Jianjun; Zhi Ke

2009-01-01

Objective: To study 64-MSCT perfusion imaging features about renal corticomedullary differentiation, contrast between renal cortex and medulla (CMC), renal cortex and medulla CT peak value in normal and hydronephrotic kidneys, and to explore the relationship between them and the unilateral renal function. Methods: Thirty-six patients with obstructive nephrohydrosis underwent 64-MSCT perfusion scanning. The split renal glomerular filtration rates (GFR) of their kidneys were measured by SPECT renal dynamic imaging. The 72 kidneys were divided into groups of normal renal function group, mild and severe renal impairment groups according to GFR. Renal corticomedullary differentiation on CT images was graded as clear, obscure, part clear. The CT intensity of cortex and medulla was measured in order to calculate contrast between renal cortex and medulla (CMC). Using Pearson correlation test, the correlation between them and renal GFR were examined. Results: (1) In the 24 kidneys of normal group, all kidneys showed clear CMD. In the 21 kidneys of mild renal impairment group, 14 kidneys showed clear CMD, 2 showed obscure CMD and 5 showed part clear of CMD. In the 27 kidneys of severe renal impairment group, 7 kidneys showed clear CMD, 5 showed obscure CMD and 15 showed part clear of CMD. (2)The CMC of normal group was 0.62 ± 0.20, while it was 0.52 ± 0.14 and 0.37 ± 0.11 for mild renal impairment group and severe renal impairment group CMC respectively. The CMC had positive linear correlation with GFR (r=0.536,P<0.05). (3) The renal cortex and medulla CT peak value of normal group were (133 ± 22) and (104 ± 16) HU; The renal cortex and medulla CT peak value of mild renal impairment group were (91 ± 29) and (76 ± 25) HU; The renal cortex and medulla CT peak value of severe renal impairment group were (68 ± 24) and (57 ± 21) HU(F=42.76 and 32.68,P<0.05). The renal cortex and medulla CT peak value had positive linear correlation with GFR (r=0.672 and 0.623, P<0

Preliminary evidence of altered biomechanics in adolescents with juvenile fibromyalgia.

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Sil, Soumitri; Thomas, Staci; DiCesare, Christopher; Strotman, Daniel; Ting, Tracy V; Myer, Gregory; Kashikar-Zuck, Susmita

2015-01-01

Juvenile fibromyalgia (FM) is characterized by chronic musculoskeletal pain and marked reduction in physical activity. Despite recommendations for exercise to manage juvenile FM pain, exercise adherence is poor. Because of pain and activity avoidance, adolescents with juvenile FM are at risk for altered joint mechanics that may make them susceptible to increased pain and reduced tolerance for exercise. The primary aim of this study was to assess functional deficits in patients with juvenile FM compared to healthy controls using objective biomechanical assessment. Female adolescent patients with juvenile FM (n = 17) and healthy controls (n = 14) completed biomechanical assessments, including gait analysis and tests of lower extremity strength (isokinetic knee extension/flexion and hip abduction) and functional performance (drop vertical jump test) along with self-reported measures of disability (Functional Disability Inventory), pain intensity, depressive symptoms (Children's Depression Inventory), and fear of movement (Tampa Scale of Kinesiophobia). Patients with juvenile FM demonstrated mild deficiencies in walking gait and functional performance (P < 0.05 for both) and significantly lower left knee extension and flexion strength (18-22% deficit) and bilateral hip abduction strength (34-38%) compared with healthy controls (P < 0.008 for all). Patients with juvenile FM reported significantly higher functional disability, pain intensity, depressive symptoms, and fear of movement relative to controls (P < 0.01 for all). This study showed that adolescents with juvenile FM exhibited objective alterations in biomechanics and self-reported fear of movement that may have reinforced their activity avoidance. Interventions for juvenile FM should include a focus on correcting functional deficits and instilling greater confidence in adolescents with juvenile FM to engage in exercise to improve functional outcomes. Copyright © 2015 by the American College of Rheumatology.

Examination of the influence of juvenile Atlantic salmon on the feeding mode of juvenile steelhead in Lake Ontario tributaries

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Johnson, James H.; Waldt, Emily M.

2014-01-01

We examined diets of 1204 allopatric and sympatric juvenile Atlantic salmon (Salmo salar) and steelhead (Oncorhynchus mykiss) in three tributaries of Lake Ontario. The diet composition of both species consisted primarily of ephemeropterans, trichopterans, and chironomids, although juvenile steelhead consumed more terrestrial invertebrates, especially at the sympatric sites. Subyearlings of both species consumed small prey (i.e. chironomids) whereas large prey (i.e. perlids) made up a higher percentage of the diet of yearlings. The diet of juvenile steelhead at the allopatric sites was more closely associated with the composition of the benthos than with the drift, but was about equally associated with the benthos and drift at the sympatric sites. The diet of both subyearling and yearling Atlantic salmon was more closely associated with the benthos than the drift at the sympatric sites. The evidence suggests that juvenile steelhead may subtly alter their feeding behavior in sympatry with Atlantic salmon. This behavioral adaptation may reduce competitive interactions between these species.

Multiple Renal Artery Pseudoaneurysms in Patients Undergoing Renal Artery Embolization Following Partial Nephrectomy: Correlation with RENAL Nephrometry Scores

International Nuclear Information System (INIS)

Gupta, Nakul; Patel, Anish; Ensor, Joe; Ahrar, Kamran; Ahrar, Judy; Tam, Alda; Odisio, Bruno; Huang, Stephen; Murthy, Ravi; Mahvash, Armeen; Avritscher, Rony; McRae, Stephen; Sabir, Sharjeel; Wallace, Michael; Matin, Surena; Gupta, Sanjay

2017-01-01

PurposeTo describe the incidence of multiple renal artery pseudoaneurysms (PSA) in patients referred for renal artery embolization following partial nephrectomy and to study its relationship to RENAL nephrometry scores.Materials and MethodsThe medical records of 25 patients referred for renal artery embolization after partial nephrectomy were retrospectively reviewed for the following parameters: size and number of tumors, RENAL nephrometry scores, angiographic abnormalities, technical and clinical outcomes, and estimated glomerular filtration rates (eGFRs) after embolization.ResultsTwenty-four patients had primary renal tumors, while 1 patient had a pancreatic tumor invading the kidney. Multiple tumors were resected in 4 patients. Most patients (92 %) were symptomatic, presenting with gross hematuria, flank pain, or both. Angiography revealed PSA with (n = 5) or without (n = 20) AV fistulae. Sixteen patients (64 %) had multiple PSA involving multiple renal vessels. Higher RENAL nephrometry scores were associated with an increasing likelihood of multiple PSA. Multiple vessels were embolized in 14 patients (56 %). Clinical success was achieved after one (n = 22) or two (n = 3) embolization sessions in all patients. Post-embolization eGFR values at different time points after embolization were not significantly different from the post-operative eGFR.ConclusionA majority of patients requiring renal artery embolization following partial nephrectomy have multiple pseudoaneurysms, often requiring selective embolization of multiple vessels. Higher RENAL nephrometry score is associated with an increasing likelihood of multiple pseudoaneurysms. We found transarterial embolization to be a safe and effective treatment option with no long-term adverse effect on renal function in all but one patient with a solitary kidney.

Multiple Renal Artery Pseudoaneurysms in Patients Undergoing Renal Artery Embolization Following Partial Nephrectomy: Correlation with RENAL Nephrometry Scores

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Gupta, Nakul [Houston Methodist Hospital (United States); Patel, Anish [The University of Texas Southwestern Medical Center (United States); Ensor, Joe [Houston Methodist Research Institute, The Houston Methodist Cancer Center (United States); Ahrar, Kamran; Ahrar, Judy; Tam, Alda; Odisio, Bruno; Huang, Stephen; Murthy, Ravi; Mahvash, Armeen; Avritscher, Rony; McRae, Stephen; Sabir, Sharjeel; Wallace, Michael [The University of Texas MD Anderson Cancer Center, Department of Interventional Radiology (United States); Matin, Surena [The University of Texas MD Anderson Cancer Center, Department of Urology (United States); Gupta, Sanjay, E-mail: sgupta@mdanderson.org [The University of Texas MD Anderson Cancer Center, Department of Interventional Radiology (United States)

2017-02-15

PurposeTo describe the incidence of multiple renal artery pseudoaneurysms (PSA) in patients referred for renal artery embolization following partial nephrectomy and to study its relationship to RENAL nephrometry scores.Materials and MethodsThe medical records of 25 patients referred for renal artery embolization after partial nephrectomy were retrospectively reviewed for the following parameters: size and number of tumors, RENAL nephrometry scores, angiographic abnormalities, technical and clinical outcomes, and estimated glomerular filtration rates (eGFRs) after embolization.ResultsTwenty-four patients had primary renal tumors, while 1 patient had a pancreatic tumor invading the kidney. Multiple tumors were resected in 4 patients. Most patients (92 %) were symptomatic, presenting with gross hematuria, flank pain, or both. Angiography revealed PSA with (n = 5) or without (n = 20) AV fistulae. Sixteen patients (64 %) had multiple PSA involving multiple renal vessels. Higher RENAL nephrometry scores were associated with an increasing likelihood of multiple PSA. Multiple vessels were embolized in 14 patients (56 %). Clinical success was achieved after one (n = 22) or two (n = 3) embolization sessions in all patients. Post-embolization eGFR values at different time points after embolization were not significantly different from the post-operative eGFR.ConclusionA majority of patients requiring renal artery embolization following partial nephrectomy have multiple pseudoaneurysms, often requiring selective embolization of multiple vessels. Higher RENAL nephrometry score is associated with an increasing likelihood of multiple pseudoaneurysms. We found transarterial embolization to be a safe and effective treatment option with no long-term adverse effect on renal function in all but one patient with a solitary kidney.

Juvenile idiopathic arthritis in adulthood: fulfilment of classification criteria for adult rheumatic diseases, long-term outcomes and predictors of inactive disease, functional status and damage.

Science.gov (United States)

Oliveira-Ramos, Filipa; Eusébio, Mónica; M Martins, Fernando; Mourão, Ana Filipa; Furtado, Carolina; Campanilho-Marques, Raquel; Cordeiro, Inês; Ferreira, Joana; Cerqueira, Marcos; Figueira, Ricardo; Brito, Iva; Canhão, Helena; Santos, Maria José; Melo-Gomes, José A; Fonseca, João Eurico

2016-01-01

To determine how adult juvenile idiopathic arthritis (JIA) patients fulfil classification criteria for adult rheumatic diseases, evaluate their outcomes and determine clinical predictors of inactive disease, functional status and damage. Patients with JIA registered on the Rheumatic Diseases Portuguese Register (Reuma.pt) older than 18 years and with more than 5 years of disease duration were included. Data regarding sociodemographic features, fulfilment of adult classification criteria, Health Assessment Questionnaire, Juvenile Arthritis Damage Index-articular (JADI-A) and Juvenile Arthritis Damage Index-extra-articular (JADI-E) damage index and disease activity were analysed. 426 patients were included. Most of patients with systemic JIA fulfilled criteria for Adult Still's disease. 95.6% of the patients with rheumatoid factor (RF)-positive polyarthritis and 57.1% of the patients with RF-negative polyarthritis matched criteria for rheumatoid arthritis (RA). 38.9% of the patients with extended oligoarthritis were classified as RA while 34.8% of the patients with persistent oligoarthritis were classified as spondyloarthritis. Patients with enthesitis-related arthritis fulfilled criteria for spondyloarthritis in 94.7%. Patients with psoriatic arthritis maintained this classification. Patients with inactive disease had lower disease duration, lower diagnosis delay and corticosteroids exposure. Longer disease duration was associated with higher HAQ, JADI-A and JADI-E. Higher JADI-A was also associated with biological treatment and retirement due to JIA disability and higher JADI-E with corticosteroids exposure. Younger age at disease onset was predictive of higher HAQ, JADI-A and JADI-E and decreased the chance of inactive disease. Most of the included patients fulfilled classification criteria for adult rheumatic diseases, maintain active disease and have functional impairment. Younger age at disease onset was predictive of higher disability and decreased the

Factor analysis of symptom profile in early onset and late onset OCD.

Science.gov (United States)

Grover, Sandeep; Sarkar, Siddharth; Gupta, Gourav; Kate, Natasha; Ghosh, Abhishek; Chakrabarti, Subho; Avasthi, Ajit

2018-04-01

This study aimed to assess the factor structure of early and late onset OCD. Additionally, cluster analysis was conducted in the same sample to assess the applicability of the factors. 345 participants were assessed with Yale Brown Obsessive Compulsive Scale symptom checklist. Patients were classified as early onset (onset of symptoms at age ≤ 18 years) and late onset (onset at age > 18 years) OCD depending upon the age of onset of the symptoms. Factor analysis and cluster analysis of early-onset and late-onset OCD was conducted. The study sample comprised of 91 early onset and 245 late onset OCD subjects. Males were more common in the early onset group. Differences in the frequency of phenomenology related to contamination related, checking, repeating, counting and ordering/arranging compulsions were present across the early and late onset groups. Factor analysis of YBOCS revealed a 3 factor solution for both the groups, which largely concurred with each other. These factors were named as hoarding and symmetry (factor-1), contamination (factor-2) and aggressive, sexual and religious factor (factor-3). To conclude this study shows that factor structure of symptoms of OCD seems to be similar between early-onset and late-onset OCD. Copyright © 2017 Elsevier B.V. All rights reserved.

Intelligence Score Profiles of Female Juvenile Offenders

Science.gov (United States)

Werner, Shelby Spare; Hart, Kathleen J.; Ficke, Susan L.

2016-01-01

Previous studies have found that male juvenile offenders typically obtain low scores on measures of intelligence, often with a pattern of higher scores on measures of nonverbal relative to verbal tasks. The research on the intelligence performance of female juvenile offenders is limited. This study explored the Wechsler Intelligence Scale for…

Euthyroid sick syndrome in patients with acute renal failure

International Nuclear Information System (INIS)

Ilic Slobodan; Vlajkovic Marina; Rajic Milena; Bogicevic Momcilo

2004-01-01

Objectives: The purpose of this study was to evaluate serum thyroid hormone profile in acute renal failure (ARF) patients according to the initial 131I-OIH clearance value as a predictor of ARF outcome. Patients and methods: Radioimmuno-assays of T4, T3, FT4, FT3, rT3 and TSH were performed in 32 ARF patients within 7 days and 6 months after ARF onset. The patients were divided into three groups according to the kidney function recovery potential measured by 131I-OIH clearance as follows: Group I: high probability for kidney recovery (131I-OIH clearance >250 ml/min), Group II: intermediate probability for kidney recovery (131I-OIH clearance 151-250 ml/min) and Group III: low probability for kidney recovery (131I-OIH clearance <150 ml/min). The results were compared with those obtained in 20 healthy patients. Results: Total thyroid hormone and TSH values are displayed in the table, Values of total T4 and TSH were slightly declined in the Group I but without reaching the statistical significance, while total T3 value was significantly decreased seven days after ARF onset. In the groups with intermediate and low probability for kidney recovery both T3 and T4 values were significantly dropped, being most prominent in the III group. After six months, the most severe fall of thyroid hormone levels without reaching the normalization was found only in the Group III while in the Group I and II normalization of total thyroid hormone levels was achieved. At the end of the observation period ARF patients with low probability for kidney recovery were found significantly lower values of TSH. Conclusion: Acute renal failure affects thyroid function leading to euthyroid sick syndrome characterized by declined serum T3 and T4 without TSH elevation. Thyroid hormone a disturbance is in accordance with the impairment of renal function being the most pronounced in patient with low probability for kidney recovery. This pattern of altered thyroid hormone levels could be a result of

Penalty responsibility of juveniles in the Republic of Srpska

Directory of Open Access Journals (Sweden)

Grbić-Pavlović Nikolina

2011-01-01

Full Text Available The youngest members of organized society, more intensive than ever enter the circle of those whose behavior is deviant. Juvenile delinquency is a social problem, which recently experienced an expansion in all modern countries, including Bosnia and Herzegovina and the Republic of Srpska. Considering the fact that juvenile delinquency includes lighter criminal conducts, such as, for example misdemeanors, in this paper a position of juveniles when they are a perpetrators of misdemeanors will be analyzed. Also, the paper will statistically show the number of misdemeanors in the field of public peace and order that juveniles conducted in the Republic of Srpska in the period 2004-2009.

Delincuencia y responsabilidad penal juvenil en Colombia

Directory of Open Access Journals (Sweden)

Cristina Montalvo Velásquez

2011-01-01

Full Text Available ResumenEl término «delincuencia juvenil» fue acuñado en Inglaterra en el año 1815, “Se entiende por delincuencia juvenil el conjunto de delitos, contravenciones o comportamientos socialmente reprochables, que cometen las personas consideradas como jóvenes por la ley”1 . Cada Estado está sujeto a su propio sistema jurídico, para algunos es delincuente juvenil el adolescente que comete acciones sancionadas por la ley sin importar su gravedad, otros Estados sólo consideran como delincuente juvenil al joven que comete un acto delictivo grave.El fenómeno de la delincuencia juvenil es algo que se inscribe en los espacios de una sociedad en la cual su estructura material, y su formación social consecuente, se halla en una profunda crisis. Que jóvenes conformen bandas de delincuencia organizada nos está indicando que son el resultado de la misma criminalidad general que se ha apoderado de la sociedad en la perspectiva de lograr sobrevivir materialmente. El capitalismo no es sólo acumulación de riqueza sino concentración de la misma en muy pocas manos; y todo el sistema institucional y legal tiende a favorecer ese fenómeno porque éste constituye la supra estructura del modo de producción capitalista. Así como los adultos se organizan para delinquir, lo hacen los niños y los jóvenes a partir de una edad en la cual pueden percibir que la sociedad no es sana y no tienen porvenir humano en ella. Abandonados y sujetos a la violencia que engendra el sistema, ellos simplemente responden en una manifestación de reflejos condicionados que sostienen la sobrevivencia en forma instintiva; “los niños no saben de normas legales sino de formas de sobrevivir a semejante situación; el instinto de sobrevivencia no tiene edades ni la normatividad puede incidir en él”.Palabras ClavesDelincuencia juvenil, Jóvenes, Criminalidad, Familia, Factores, Acto delictivo, Responsabilidad Penal.AbstractThe term “juvenile delinquency” was coined in

The renal quantitative scintillation camera study for determination of renal function

International Nuclear Information System (INIS)

Thompson, I.M. Jr.; Boineau, F.G.; Evans, B.B.; Schlegel, J.U.

1983-01-01

The renal quantitative scintillation camera study assesses glomerular filtration rate and effective renal plasma flow based upon renal uptake of 99mtechnetium-iron ascorbate and 131iodine-hippuran, respectively. The method was compared to inulin, para-aminohippuric acid and creatinine clearance studies in 7 normal subjects and 9 patients with various degrees of reduced renal function. The reproducibility of the technique was determined in 15 randomly selected pediatric patients. The values of glomerular filtration rate and effective renal plasma flow were not significantly different from those of inulin and para-aminohippuric acid studies. The reproducibility of the technique was comparable to that of inulin and para-aminohippuric acid studies. Patient acceptance of the technique is excellent and the cost is minimal. Renal morphology and excretory dynamics also are demonstrated. The technique is advocated as a clinical measure of renal function

Evolution of Juvenile Ankylosing Spondylitis

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Ye.V. Prohorov

2013-02-01

Full Text Available Evolution of juvenile ankylosing spondylitis tend to follow a more frequent involvement in the pathological process of elbow and ankle joints, development of enthesiopathies, changes of intraarticular meniscal horns, forming of Baker’s cysts, cartilage flaps and systemic osteoporosis, and total value of all these signs 13 times exceeds thereof in patients with with the debut of disease in adulthood, but for juvenile ankylosing spondylitis vertebral lesion is less common. Age dimorphism of the use of certain groups of drugs and physiotherapy facilities is observed.

The prevalence of new onset diabetes mellitus after renal transplantation in patients with immediate posttransplant hyperglycemia in a tertiary care centre

Directory of Open Access Journals (Sweden)

Saba Samad Memon

2017-01-01

Full Text Available Objectives: This study aimed to determine the prevalence of immediate posttransplant hyperglycemia and new onset diabetes after renal transplantation (NODAT. It also aims at answering whether posttransplant hyperglycemia per se is a risk factor for future development of NODAT. Methods: A retrospective study was conducted among patients undergoing kidney transplantation under a single surgical unit in a tertiary care hospital in the past 5 years. All known patients with diabetes were excluded from the study. Immediate postoperative hyperglycemia was defined as random blood sugar (RBS ≥200 mg/dl or requirement of insulin. NODAT was defined as fasting plasma glucose ≥126 mg/dl or RBS ≥200 mg/dl or if the patient is receiving therapy for glycemic control at 6 weeks or 3 months posttransplantation. Results: The study population included 191 patients. The overall prevalence of posttransplant hyperglycemia and NODAT was 31.4% and 26.7%, respectively. NODAT developed in 28 patients (46.7% of those who had posttransplant hyperglycemia. Thus, posttransplant hyperglycemia was associated with a fourfold increased risk of NODAT (P = 0.000. Posttransplant hyperglycemia was associated with increased infections (P = 0.04 and prolonged hospital stay (P = 0.0001. Increased age was a significant risk factor for NODAT (P = 0.000, whereas gender, acute rejection episodes, cadaveric transplant, hepatitis C virus status, human leukocyte antigen mismatch, and high calcineurin levels were not significantly associated with the future development of NODAT. Conclusion: The significant risk of NODAT posed by posttransplant hyperglycemia makes it prudent to follow up these patients more diligently in a resource-limited setting wherein routine monitoring in all patients is cumbersome.

Inhibiting aerobic glycolysis suppresses renal interstitial fibroblast activation and renal fibrosis.

Science.gov (United States)

Ding, Hao; Jiang, Lei; Xu, Jing; Bai, Feng; Zhou, Yang; Yuan, Qi; Luo, Jing; Zen, Ke; Yang, Junwei

2017-09-01

Chronic kidney diseases generally lead to renal fibrosis. Despite great progress having been made in identifying molecular mediators of fibrosis, the mechanism that governs renal fibrosis remains unclear, and so far no effective therapeutic antifibrosis strategy is available. Here we demonstrated that a switch of metabolism from oxidative phosphorylation to aerobic glycolysis (Warburg effect) in renal fibroblasts was the primary feature of fibroblast activation during renal fibrosis and that suppressing renal fibroblast aerobic glycolysis could significantly reduce renal fibrosis. Both gene and protein assay showed that the expression of glycolysis enzymes was upregulated in mouse kidneys with unilateral ureter obstruction (UUO) surgery or in transforming growth factor-β1 (TGF-β1)-treated renal interstitial fibroblasts. Aerobic glycolysis flux, indicated by glucose uptake and lactate production, was increased in mouse kidney with UUO nephropathy or TGF-β1-treated renal interstitial fibroblasts and positively correlated with fibrosis process. In line with this, we found that increasing aerobic glycolysis can remarkably induce myofibroblast activation while aerobic glycolysis inhibitors shikonin and 2-deoxyglucose attenuate UUO-induced mouse renal fibrosis and TGF-β1-stimulated myofibroblast activation. Furthermore, mechanistic study indicated that shikonin inhibits renal aerobic glycolysis via reducing phosphorylation of pyruvate kinase type M2, a rate-limiting glycolytic enzyme associated with cell reliance on aerobic glycolysis. In conclusion, our findings demonstrate the critical role of aerobic glycolysis in renal fibrosis and support treatment with aerobic glycolysis inhibitors as a potential antifibrotic strategy. Copyright © 2017 the American Physiological Society.

RENAL CRYOABLATION

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A. V. Govorov

2012-01-01

Full Text Available Renal cryoablation is an alternative minimally-invasive method of treatment for localized renal cell carcinoma. The main advantages of this methodology include visualization of the tumor and the forming of "ice ball" in real time, fewer complications compared with other methods of treatment of renal cell carcinoma, as well as the possibility of conducting cryotherapy in patients with concomitant pathology. Compared with other ablative technologies cryoablation has a low rate of repeat sessions and good intermediate oncological results. The studies of long-term oncological and functional results of renal cryoablation are presently under way.

Renal artery pulsatility index and renal volume: Normal fetuses versus growth-retarded fetuses

International Nuclear Information System (INIS)

Lee, Kyung Soon; Woo, Bock Hi

2001-01-01

To evaluate the blood flow velocity waveform of the renal artery and renal volume of growth-retarded fetuses and to compare them with those of normal fetuses. Pulsatility index of the renal artery and renal volume measured by three-dimensional ultrasonography were obtained from seventy eight normal fetuses at the gestational age from twenty five to thirty nine weeks and eighteen intrauterine growth retarded fetuses whose weight was below ten percentile at birth. We studied changes of the pulsatility index of the renal artery and renal volume according to the gestational age and compared with those of growth-retarded fetuses. Pulsatility index (PI) of the fetal renal artery decreased throughout the gestational period (r=0.703, p<0.0001). In growth-retarded fetuses, despite of abnormal doppler velocity waveform of the middle cerebral artery, which was showing fetal hypoxia, the renal PI was not increased significantly. The fetal renal volume increased throughout the gestational period (r=0.834, p<0.0001) whereas in growth-retarded fetuses, all renal volume was below fifth percentile of normal fetuses. In growth-retarded fetuses, fetal renal volume was decreased significantly without change of the renal vascular flow. Therefore, the fetal renal volume measured by three-dimensional ultrasonography may be a helpful parameter in the diagnosis of growth-retarded fetuses.

Renal artery pulsatility index and renal volume: Normal fetuses versus growth-retarded fetuses

Energy Technology Data Exchange (ETDEWEB)

Lee, Kyung Soon; Woo, Bock Hi [Ewha Womans University College of Medicine, Seoul (Korea, Republic of)

2001-06-15

To evaluate the blood flow velocity waveform of the renal artery and renal volume of growth-retarded fetuses and to compare them with those of normal fetuses. Pulsatility index of the renal artery and renal volume measured by three-dimensional ultrasonography were obtained from seventy eight normal fetuses at the gestational age from twenty five to thirty nine weeks and eighteen intrauterine growth retarded fetuses whose weight was below ten percentile at birth. We studied changes of the pulsatility index of the renal artery and renal volume according to the gestational age and compared with those of growth-retarded fetuses. Pulsatility index (PI) of the fetal renal artery decreased throughout the gestational period (r=0.703, p<0.0001). In growth-retarded fetuses, despite of abnormal doppler velocity waveform of the middle cerebral artery, which was showing fetal hypoxia, the renal PI was not increased significantly. The fetal renal volume increased throughout the gestational period (r=0.834, p<0.0001) whereas in growth-retarded fetuses, all renal volume was below fifth percentile of normal fetuses. In growth-retarded fetuses, fetal renal volume was decreased significantly without change of the renal vascular flow. Therefore, the fetal renal volume measured by three-dimensional ultrasonography may be a helpful parameter in the diagnosis of growth-retarded fetuses.

Yolo Bypass Juvenile Salmon Utilization Study 2016—Summary of acoustically tagged juvenile salmon and study fish release, Sacramento River, California

Science.gov (United States)

Liedtke, Theresa L.; Hurst, William R.

2017-09-12

The Yolo Bypass is a flood control bypass in Sacramento Valley, California. Flood plain habitats may be used for juvenile salmon rearing, however, the potential value of such habitats can be difficult to evaluate because of the intermittent nature of inundation events. The Yolo Bypass Juvenile Salmon Utilization Study (YBUS) used acoustic telemetry to evaluate the movements and survival of juvenile salmon adjacent to and within the Yolo Bypass during the winter of 2016. This report presents numbers, size data, and release data (times, dates, and locations) for the 1,197 acoustically tagged juvenile salmon released for the YBUS from February 21 to March 18, 2016. Detailed descriptions of the surgical implantation of transmitters are also presented. These data are presented to support the collaborative, interagency analysis and reporting of the study findings.

Reduced ovulation rate, failure to be mated and fertilization failure/embryo loss are the underlying causes of poor reproductive performance in juvenile ewes.

Science.gov (United States)

Edwards, Sara J; Smaill, Bronwyn; O'Connell, Anne R; Johnstone, Peter D; Stevens, David R; Quirke, Laurel D; Farquhar, Philip A; Juengel, Jennifer L

2016-04-01

A ewe that is mated as a juvenile (producing a lamb at 1 year of age) will produce an average of only 0.6 lambs to weaning, compared to an average of 1.2 lambs in adult ewes. Understanding the underlying causes of this low reproductive efficiency and designing methods to improve or mitigate these effects could potentially increase adoption of mating juvenile ewes. In Experiment 1, 2 Cohorts of ewes, born a year apart, were mated in order to lamb at 1 and 2 years of age and the performance of the ewes at each age was compared. Onset of puberty, mating by the fertile ram, ovulation rate, early pregnancy (day 30-35) litter size, number of lambs born and number of lambs weaned were measured. In juvenile ewes, by day 35 of pregnancy, 43% of ova had failed to become a viable embryo and this early loss was the largest contributor to the poor reproductive performance observed. Compared with young adult ewes, ovulation rate was lower (pewes with a greater ovulation rate (pewes, failure to mate with the ram, lower ovulation rate and increased fertilisation failure/embryo loss underlie their poor reproductive performance. Copyright © 2016 Elsevier B.V. All rights reserved.

Obesity and renal hemodynamics

NARCIS (Netherlands)

Bosma, R. J.; Krikken, J. A.; van der Heide, J. J. Homan; de Jong, P. E.; Navis, G. J.

2006-01-01

Obesity is a risk factor for renal damage in native kidney disease and in renal transplant recipients. Obesity is associated with several renal risk factors such as hypertension and diabetes that may convey renal risk, but obesity is also associated with an unfavorable renal hemodynamic profile

Prevalence of traumatic brain injury in juvenile offenders: a meta-analysis.

Science.gov (United States)

Farrer, Thomas J; Frost, R Brock; Hedges, Dawson W

2013-01-01

Studies of traumatic brain injury (TBI) among adult populations demonstrate that such injuries can lead to aggressive behaviors. Related findings suggest that incarcerated individuals have high rates of brain injuries. Such studies suggest that traumatic brain injury may be related to the etiology and recidivism of criminal behavior. Relatively few studies have examined the prevalence of TBI using a delinquent juvenile sample. In order to assess the relationship between TBI and juvenile offender status, the current study used meta-analytic techniques to examine the odds of having a TBI among juvenile offenders. Across 9 studies, we found that approximately 30% of juvenile offenders have sustained a previous brain injury. Across 5 studies that used a control group, a calculated summary odds ratio of 3.37 suggests that juvenile offenders are significantly more likely to have a TBI compared to controls. Results suggest that the rate of TBIs within the juvenile offender population is significant and that there may be a relationship between TBIs and juvenile criminal behavior.

Urinary C-type natriuretic peptide excretion: a potential novel biomarker for renal fibrosis during aging.

Science.gov (United States)

Sangaralingham, S Jeson; Heublein, Denise M; Grande, Joseph P; Cataliotti, Alessandro; Rule, Andrew D; McKie, Paul M; Martin, Fernando L; Burnett, John C

2011-11-01

Renal aging is characterized by structural changes in the kidney including fibrosis, which contributes to the increased risk of kidney and cardiac failure in the elderly. Studies involving healthy kidney donors demonstrated subclinical age-related nephropathy on renal biopsy that was not detected by standard diagnostic tests. Thus there is a high-priority need for novel noninvasive biomarkers to detect the presence of preclinical age-associated renal structural and functional changes. C-type natriuretic peptide (CNP) possesses renoprotective properties and is present in the kidney; however, its modulation during aging remains undefined. We assessed circulating and urinary CNP in a Fischer rat model of experimental aging and also determined renal structural and functional adaptations to the aging process. Histological and electron microscopic analysis demonstrated significant renal fibrosis, glomerular basement membrane thickening, and mesangial matrix expansion with aging. While plasma CNP levels progressively declined with aging, urinary CNP excretion increased, along with the ratio of urinary to plasma CNP, which preceded significant elevations in proteinuria and blood pressure. Also, CNP immunoreactivity was increased in the distal and proximal tubules in both the aging rat and aging human kidneys. Our findings provide evidence that urinary CNP and its ratio to plasma CNP may represent a novel biomarker for early age-mediated renal structural alterations, particularly fibrosis. Thus urinary CNP could potentially aid in identifying subjects with preclinical structural changes before the onset of symptoms and disease, allowing for the initiation of strategies designed to prevent the progression of chronic kidney disease particularly in the aging population.

Does the presence of accessory renal arteries affect the efficacy of renal denervation?

Science.gov (United States)

Id, Dani; Kaltenbach, Benjamin; Bertog, Stefan C; Hornung, Marius; Hofmann, Ilona; Vaskelyte, Laura; Sievert, Horst

2013-10-01

This study sought to assess the efficacy of catheter-based renal sympathetic denervation in patients with accessory renal arteries and to compare the blood pressure (BP)-lowering effect with that observed in patients with bilateral single renal arteries after renal denervation. Catheter-based renal sympathetic denervation causes significant BP reductions in patients with resistant hypertension. Seventy-four patients were included in this study. Patients were assigned to 2 main groups: a bilateral single renal arteries group I (n = 54) and an accessory renal arteries group II (n = 20). Group II consisted of 9 patients whose accessory renal arteries were all denervated (group IIa), and 11 patients whose accessory renal arteries were not, or only incompletely, denervated (group IIb). The primary endpoint was the change in office systolic BP after 6 months. The procedure was successful in all patients. Group I: mean BP at baseline was 166.2/89.4 ± 20.5/14.6 mm Hg and decreased by -16.6 (p renal denervation in patients with accessory renal arteries is less pronounced than in patients with bilateral single renal arteries. Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

Radiopharmaceuticals for renal studies

International Nuclear Information System (INIS)

Verdera, Silvia

1994-01-01

Between the diagnostic techniques using radiopharmaceuticals in nuclear medicine it find renal studies.A brief description about renal glomerular filtration(GFR) and reliability renal plasma flux (ERPF),renal blood flux measurement agents (RBF),renal scintillation agents and radiation dose estimates by organ physiology was given in this study.tabs

Glomerular Filtration Rate Estimation in Renal and Non-Renal Solid Organ Transplantation

DEFF Research Database (Denmark)

Hornum, Mads; Feldt-Rasmussen, Bo

2017-01-01

Following transplantation (TX) of both renal and non-renal organs, a large proportion of patients have renal dysfunction. There are multiple causes for this. Chronic nephrotoxicity and high doses of calcineurin inhibitors are important factors. Preoperative and perioperative factors like hyperten......Following transplantation (TX) of both renal and non-renal organs, a large proportion of patients have renal dysfunction. There are multiple causes for this. Chronic nephrotoxicity and high doses of calcineurin inhibitors are important factors. Preoperative and perioperative factors like...... hypertension, hypotension, drugs and infections may play a causative role as well. Organ-specific causes include hepatorenal syndrome, cirrhosis, low cardiac function, low respiratory function and diabetes developed both before and after TX. It is important to be able to perform precise and valid measurements...... rate methods for use in renal and non-renal TX....

Treatment of non-neoplastic renal hemorrhage with segmental embolization of renal artery

International Nuclear Information System (INIS)

Zhu Bing

2007-01-01

Objective: To explore the value of segmental embolization of renal artery in dealing with non- neoplastic renal hemorrhage. Methods: Four cases of non-neoplastic hemorrhage, including 2 with bleeding after renal acupuncture biopsy, 2 with bleeding after nephrolithotomy and 1 with congenital renal arteriovenous malformation, were treated with superselective segmental embolization of renal artery. 2 were embolized with coil, 1 with alcohol plus coil and 1 with PVA parcels. Results: Hematuria disappeared in 1-3 days. There was no recurrence in 7-45 months follow up and no complications induced by embolization. Conclusion: It is a safe and reliable therapy to treat non-neoplastic renal hemorrhage with segmental embolization of renal artery. (authors)

Acute renal failure by ingestion of Euphorbia paralias

Directory of Open Access Journals (Sweden)

Karima Boubaker

2013-01-01

Full Text Available Euphorbia paralias is known in traditional medicine as an anti-inflammatory agent, a purgative and for its local anesthetic property. To the best our knowledge, renal toxicity of this substance has not been previously reported. In this paper, we report the case of a 29-year-old male who developed renal damage following ingestion of Euphorbia paralias. He had been on follow-up for nephrotic syndrome since 1986, although irregularly, with several relapses but each responding well to steroid therapy. A kidney biopsy had not been performed earlier due to refusal by the patient. He was off steroids since April 2008 because the patient developed osteoporosis. He was admitted with general malaise and oliguria to our department in May 2009, following repeated vomiting and watery diarrhea for three days. On examination, he was edematous but had normal vital signs except for a pulse rate of 120/min. Hemoglobin was only 5.5 g/dL but with normal white cell and platelet counts. Blood biochemistry showed evidence of advanced renal failure with a serum creatinine level of 1835 μmol/L and urea at 44.6 mmol/L, sodium of 132 μmol/L and potassium at 4.3 mmol/L. He had features of nephrotic syndrome with severe hypoproteinamia and 24-h urinary protein of 10.45 g. Ultrasonography revealed enlarged kidneys with a reduced echogenecity of the medulla and the papillae. Subsequently, after hemodialysis with blood transfusion, a kidney biopsy was performed that showed focal segmental glomerulosclerosis associated with an acute tubular injury. On intensive interrogation, the patient gave a history of ingesting boiled Euphorbia paralias as a native treatment for edema, ten days prior to the onset of the current illness. A diagnosis of acute renal failure (ARF resulting from the possible nephrotoxic effect of Euphorbia paralias poisoning was made. He was treated with intermittent hemodialysis and corticosteroids. Serum creatinine values improved after 48 days. At six

Renal scar formation and kidney function following antibiotic-treated murine pyelonephritis

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Patrick D. Olson

2017-11-01

Full Text Available We present a new preclinical model to study treatment, resolution and sequelae of severe ascending pyelonephritis. Urinary tract infection (UTI, primarily caused by uropathogenic Escherichia coli (UPEC, is a common disease in children. Severe pyelonephritis is the primary cause of acquired renal scarring in childhood, which may eventually lead to hypertension and chronic kidney disease in a small but important fraction of patients. Preclinical modeling of UTI utilizes almost exclusively females, which (in most mouse strains exhibit inherent resistance to severe ascending kidney infection; consequently, no existing preclinical model has assessed the consequences of recovery from pyelonephritis following antibiotic treatment. We recently published a novel mini-surgical bladder inoculation technique, with which male C3H/HeN mice develop robust ascending pyelonephritis, highly prevalent renal abscesses and evidence of fibrosis. Here, we devised and optimized an antibiotic treatment strategy within this male model to more closely reflect the clinical course of pyelonephritis. A 5-day ceftriaxone regimen initiated at the onset of abscess development achieved resolution of bladder and kidney infection. A minority of treated mice displayed persistent histological abscess at the end of treatment, despite microbiological cure of pyelonephritis; a matching fraction of mice 1 month later exhibited renal scars featuring fibrosis and ongoing inflammatory infiltrates. Successful antibiotic treatment preserved renal function in almost all infected mice, as assessed by biochemical markers 1 and 5 months post-treatment; hydronephrosis was observed as a late effect of treated pyelonephritis. An occasional mouse developed chronic kidney disease, generally reflecting the incidence of this late sequela in humans. In total, this model offers a platform to study the molecular pathogenesis of pyelonephritis, response to antibiotic therapy and emergence of sequelae

Miastenia gravis juvenil Juvenile myasthenia gravis

OpenAIRE

Oscar Papazian; Israel Alfonso; Nayle Araguez

2009-01-01

La miastenia gravis juvenil (MGJ) es un trastorno crónico auto inmune en el cual existen anticuerpos séricos que al unirse a los receptores de acetilcolin nicotínicos de la membrana muscular de la placa motora alteran la transmisión neuromuscular. El resultado es fatiga muscular precoz con progresión a la parálisis durante estados de contracción muscular iterativos (movimientos) o sostenidos (posturas) y más raramente parálisis permanente durante el reposo. Los músculos inervados por los nerv...

Bilateral renal artery variation

OpenAIRE

Üçerler, Hülya; Üzüm, Yusuf; İkiz, Z. Aslı Aktan

2014-01-01

Each kidney is supplied by a single renal artery, although renal artery variations are common. Variations of the renal arteryhave become important with the increasing number of renal transplantations. Numerous studies describe variations in renalartery anatomy. Especially the left renal artery is among the most critical arterial variations, because it is the referred side forresecting the donor kidney. During routine dissection in a formalin fixed male cadaver, we have found a bilateral renal...

Juvenile xanthogranuloma of the corneoscleral limbus.

Science.gov (United States)

Yanoff, M; Perry, H D

1995-07-01

Juvenile xanthogranuloma is a rare and usually benign skin disease of unknown cause that occurs in infants and young children. We studied a case of juvenile xanthogranuloma of the corneoscleral limbus in a 17-year-old black boy, who presented with a 5-month history of a lump in the right eye. The lesion extended from the superior limbus, fanning out as it proceeded posteriorly for 6 mm with a width of 9 mm and a height of 2 to 3 mm. This yellow-orange mass was vascular and firmly fixed to the underlying tissue. The lesion was diagnosed as a dermoid and observed for 7 months without documented growth before an uneventful excisional biopsy was performed. The pathologic diagnosis showed the characteristic picture of juvenile xanthogranuloma with numerous Touton giant cells. Lipid stains provided further confirmation.

Modelling population effects of juvenile offshore fish displacement towards adult habitat

DEFF Research Database (Denmark)

van de Wolfshaar, K.E.; Tulp, I.; Wennhage, H.

2015-01-01

consequences on population dynamics through changes in resource use and competition. To explore this, a conceptual stage-structured model was developed with 3 stages and 2 resources and allowing a move of large juveniles from the shallow to the deep habitat. Large juveniles compete with small juveniles...... in shallow waters and with adults in deeper waters. Alternative stable states occur, with one state dominated by small juvenile biomass and the other dominated by adult biomass. The model results show for both states that while large juvenile biomass responds to a change in time spent in the deep habitat...

Imaging of juvenile spondyloarthritis. Part I: Classifications and radiographs

Directory of Open Access Journals (Sweden)

Iwona Sudoł-Szopińska

2017-09-01

Full Text Available Juvenile spondyloarthropathies are manifested mainly by symptoms of peripheral arthritis and enthesitis. By contrast with adults, children rarely present with sacroiliitis and spondylitis. Imaging and laboratory tests allow early diagnosis and treatment. Conventional radiographs visualize late inflammatory lesions and post-inflammatory complications. Early diagnosis is possible with the use of ultrasonography and magnetic resonance imaging. The first part of the article presents classifications of juvenile spondyloarthropathies and discusses their radiographic presentation. Typical radiographic features of individual types of juvenile spondyloarthritis are listed (including ankylosing spondylitis, juvenile psoriatic arthritis, reactive arthritis and arthritis in the course of inflammatory bowel diseases. The second part will describe changes visible on ultrasonography and magnetic resonance imaging. In patients with juvenile spondyloarthropathies, these examinations are conducted to diagnose inflammatory lesions in peripheral joints, tendon sheaths, tendons and bursae. Moreover, magnetic resonance imaging also visualizes early inflammatory changes in the axial skeleton and subchondral bone marrow edema, which is considered an early sign of inflammation.

Síndrome CINCA: um diagnóstico diferencial da artrite idiopática juvenil CINCA syndrome: a differential diagnosis of the juvenile idiopathic arthritis

Directory of Open Access Journals (Sweden)

Erica Naomi Naka

2007-08-01

Full Text Available A síndrome CINCA (crônico-infantil-neurológica-cutâneaarticular é uma enfermidade inflamatória multissistêmica rara, de início no período neonatal e caracterizada por febre, exantema cutâneo, envolvimento articular e do sistema nervoso central. É também conhecida pela literatura médica norte-americana como NOMID (doença multissistêmica inflamatória de início neonatal. Relatamos o caso de uma criança de 3 anos de idade admitida em nosso serviço com história de febre e exantema cutâneo desde o período neonatal. Apresentou crises convulsivas no sexto mês de vida e artrite simétrica de joelhos desde o nono mês. Na admissão, mostrava-se toxemiada, pálida, com um exantema maculopapular generalizado e artrite de joelhos e tornozelos. Apresentava ainda retardo de crescimento e desenvolvimento. Achados laboratoriais incluíram anemia, leucocitose, trombocitose, níveis elevados de proteína C reativa e meningite asséptica no exame do liquor. Os outros exames foram negativos. Os achados radiográficos dos joelhos, quadris e tornozelos foram anormais. A criança recebeu tratamento com antiinflamatório não hormonal, corticosteróide e metotrexato, com melhora apenas da dor e da febre. A etiologia da síndrome CINCA permanece desconhecida e nenhum tratamento tem se mostrado eficaz. Essa doença deve ser distinguida da forma sistêmica da artrite idiopática juvenil (AIJ, o principal diagnóstico diferencial.CINCA syndrome (chronic-infantile-neurological-cutaneousarticular is a rare multisystemic inflammatory disease with neonatal onset characterized by fever, skin rash, articular, and central nervous system involvement. This syndrome is known in the North American medical literature as infantile onset multisystem inflammatory disease (NOMID. We describe the case of a 3-yearold child admitted in our service with fever and skin rash since the neonatal period. She presented seizures at 6 months-old and bilateral arthritis of the

Spatial dynamics of juvenile anchovy in the Bay of Biscay

KAUST Repository

Boyra, Guillermo

2016-07-08

In autumn 2009, the implementation of two successive acoustic surveys targeting juvenile anchovy (Engraulis encrasicolus) in the Bay of Biscay allowed us to monitor the changes in the spatial distribution and aggregation patterns of juveniles of this species during 45 days under fairly stable meteorological conditions. Juvenile anchovy changed its biological condition and behavior in a different manner in two distinct areas. In the Spanish sector, the juveniles migrated 20 nautical miles (n.mi.) towards the coast, but they remained on the shelf and near the surface during the whole surveyed period. As the advance towards the shelf break progressed, their area of distribution decreased, their density increased and the juveniles spread in fewer but heavier shoals. In the French sector, the juveniles also migrated from slope waters towards the coast at a similar velocity, but they crossed the shelf break into the continental shelf, where they increased their mean depth significantly until gradually adopting the typical nyctemeral migrations of adult anchovy. The mean length of the juveniles that adopted the nyctemeral migrations was significantly higher than that of the juveniles remaining at the surface, suggesting that body size is relevant to accomplish this change. Besides, the stronger temperature gradients between the shelf and oceanic waters in the Spanish sector, favored by a narrow shelf, may have acted as a barrier influencing the distinct observed spatial patterns in the two areas. © 2016 John Wiley & Sons Ltd

Spatial dynamics of juvenile anchovy in the Bay of Biscay

KAUST Repository

Boyra, Guillermo; Peñ a, Marian; Cotano, Unai; Irigoien, Xabier; Rubio, Anna; Nogueira, Enrique

2016-01-01

In autumn 2009, the implementation of two successive acoustic surveys targeting juvenile anchovy (Engraulis encrasicolus) in the Bay of Biscay allowed us to monitor the changes in the spatial distribution and aggregation patterns of juveniles of this species during 45 days under fairly stable meteorological conditions. Juvenile anchovy changed its biological condition and behavior in a different manner in two distinct areas. In the Spanish sector, the juveniles migrated 20 nautical miles (n.mi.) towards the coast, but they remained on the shelf and near the surface during the whole surveyed period. As the advance towards the shelf break progressed, their area of distribution decreased, their density increased and the juveniles spread in fewer but heavier shoals. In the French sector, the juveniles also migrated from slope waters towards the coast at a similar velocity, but they crossed the shelf break into the continental shelf, where they increased their mean depth significantly until gradually adopting the typical nyctemeral migrations of adult anchovy. The mean length of the juveniles that adopted the nyctemeral migrations was significantly higher than that of the juveniles remaining at the surface, suggesting that body size is relevant to accomplish this change. Besides, the stronger temperature gradients between the shelf and oceanic waters in the Spanish sector, favored by a narrow shelf, may have acted as a barrier influencing the distinct observed spatial patterns in the two areas. © 2016 John Wiley & Sons Ltd

Using the Juvenile Justice Poster. Teaching Strategy.

Science.gov (United States)

Update on Law-Related Education, 2000

2000-01-01

Presents a lesson that can help students review and summarize what they have learned about the juvenile justice system. Explains that the students discuss how the juvenile justice system can be improved and conduct a survey on how it might be changed in the future. Provides a copy of the survey and directions. (CMK)

Reformations in Zimbabwe's juvenile justice system | Ruparanganda ...

African Journals Online (AJOL)

Children in conflict with the law are often stigmatized and shunned by society as they are perceived as a threat to society. Historically, Zimbabwe's juvenile justice system has been retributive and focused on punishing the juvenile offender. As a result, it has been criticised from a number of viewpoints, including the need to ...

Juvenile Delinquency and Teenage Pregnancy: A Comparison of Ecological Risk Profiles among Midwestern White and Black Female Juvenile Offenders

Science.gov (United States)

Khurana, Atika; Cooksey, Elizabeth C.; Gavazzi, Stephen M.

2011-01-01

The authors examined ecological risk factors associated with teen pregnancy with a sample of 1,190 court-involved female juvenile offenders between 11 and 18 years of age. Data were obtained from five Midwestern juvenile county courts using a recently developed youth risk assessment instrument called the global risk assessment device (GRAD). In…

[Aortic dissection spread to the renal arteries: role of renal volumetry after angioplasty].

Science.gov (United States)

Vautrin, E; Thony, F; Chavanon, O; Hannachi, I; Barone-Rochette, G; Pierre, H; Baguet, J-P

2012-06-01

Type A or B aortic dissection can extend to renal arteries, causing a renal ischemia which treatment is usually endovascular. The aim of our study is to show the interest of the renal volumetry in the follow-up of these patients. Twenty-two patients (16 men, mean age 63.4±11.8years, BMI 25.2±3.4kg/m(2)) with a type A or B aortic dissection spread to one or to both renal arteries and followed at Grenoble university hospital were consecutively included. All patients underwent renal angiography with aorto-renal pressure gradients measurements and follow-up by renal volumetry (scanner Siemens(®)). A renal ischemia was defined by a decrease of 20% or more of the volumetry. Sixteen patients (73%) were hypertensive before the aortic dissection among which ten (62%) were treated. Eight patients (36%) have a significant renal pressure gradient among which five (62%) underwent renal endovascular therapy. The renal volumetry of these five patients remained unchanged while six of 17 patients (36%) without angioplasty have a decreasing volumetry. Renal volumetry appeared an effective and attractive option for the follow-up of the patients with aortic dissection spread to the renal arteries. These results should be taken into account to put the indication of an endovascular treatment. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

BILATERAL DUPLICATION OF RENAL ARTERIES

OpenAIRE

Prajkta A Thete; Mehera Bhoir; M.V.Ambiye

2014-01-01

Routine dissection of a male cadaver revealed the presence of bilateral double renal arteries. On the right side the accessory renal artery originated from the abdominal aorta just above the main renal artery. On the left side the accessory renal artery originated from the abdominal aorta about 1 cm above the main renal artery. Knowledge of the variations of renal vascular anatomy has importance in exploration and treatment of renal trauma, renal transplantation, renal artery embolization, su...

83 CHALLENGES AND PROSPECTS OF THE JUVENILE JUSTICE ...

African Journals Online (AJOL)

Fr. Ikenga

Juvenile justice administration in Nigeria is weak and has been given very .... The Nigerian criminal justice system, of which the juvenile justice system is an integral part, ... as instruments of security and justice but as weapons of oppression8.

Bilateral, independent juvenile nasopharyngeal angiofibroma: case report.

Science.gov (United States)

Mørkenborg, M-L; Frendø, M; Stavngaard, T; Von Buchwald, C

2015-10-01

Juvenile nasopharyngeal angiofibroma is a benign, vascular tumour that primarily occurs in adolescent males. Despite its benign nature, aggressive growth patterns can cause potential life-threatening complications. Juvenile nasopharyngeal angiofibroma is normally unilateral, originating from the sphenopalatine artery, but bilateral symptoms can occur if a large tumour extends to the contralateral side of the nasopharynx. This paper presents the first reported case of true bilateral extensive juvenile nasopharyngeal angiofibroma involving clinically challenging pre-surgical planning and surgical strategy. A 21-year-old male presented with increasing bilateral nasal obstruction and discharge. Examination revealed tumours bilaterally and imaging demonstrated non-contiguous tumours. Pre-operative angiography showed strictly ipsilateral vascular supplies requiring bilateral embolisation. Radical removal performed as one-step, computer-assisted functional endoscopic sinus surgery was performed. The follow-up period was uncomplicated. This case illustrates the importance of suspecting bilateral juvenile nasopharyngeal angiofibroma in patients presenting with bilateral symptoms. Our management, including successful pre-operative planning, enabled one-step total removal of both tumours and rapid patient recovery.

Radium rentention and dosimetry in juvenile beagles

International Nuclear Information System (INIS)

Lloyd, R.D.; Jones, C.W.; Bruenger, F.W.; Atherton, D.R.; Mays, C.W.

1983-01-01

Retention of administered 226 Ra was substantially greater in beagles injected as 3-month-old juveniles than as 1.4-year-old adults, but the measured 222 Rn/ 226 Ra ratio in bone was significantly less in juveniles for about the first 600 days after injection. An equation that describes the total-body biological retention R in beagles injected with 226 Ra at 3 months of age at any time t (in days) after injection during the first 6.6 years is R = 0.331e/sup -0.206t/ + 0.245e/sup -0.00374t/ + 0.424e/sup -0.000114t/. The rate constant of the final term in the equation for juveniles is similar to that for young adults, suggesting that this component reflects the net turnover rate in the slowly remodeling component of adult bone. Compared to young adult beagles, animals injected as juveniles had a greater fraction of their retained 226 Ra in parts of the skeleton containing much cortical bone, such as paws, and a smaller fraction in those parts containing much trabecular bone

Association analysis of cigarette smoking with onset of primary open-angle glaucoma and glaucoma-related biometric parameters

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Wang Degui

2012-11-01

Full Text Available Abstract Background To date, studies on the role played by cigarette smoking in primary open-angle glaucoma (POAG remains controversial. The current study evaluated cigarette smoking as a risk factor of POAG and its relationships with vertical cup-to-disc ratio (VCDR, central corneal thickness (CCT and intraocular pressure (IOP in a Chinese cohort. Methods In a total of 248 unrelated individuals including 30 juvenile-onset POAG (JOAG, 92 adult-onset POAG (AOAG and 126 sex-matched senile cataract controls, underwent comprehensive ophthalmic examination. Their smoking was obtained and documented by questionnaire. Association of cigarette smoking with POAG was performed using logistic regression controlled for age and sex. Effects of cigarette smoking on VCDR, IOP and CCT were analyzed with multiple linear regression. Results In either JOAG or AOAG, no association of cigarette smoking was found with disease onset (P = 0.692 and 0.925 respectively. In controls and JOAG, no significant effects of smoking were found on VCDR, IOP or CCT (all P > 0.05. Smoking was found to be correlated with decreased CCT in AOAG and combined POAG (JOAG + AOAG (P = 0.009 and 0.003, but no association with VCDR or IOP was observed (P > 0.05. Conclusions Although cigarette smoking was not found to be risk factor for onset of POAG, it was correlated with CCT in AOAG, and thus might still play a role in the disease course, especially for AOAG.

Race and the fragility of the legal distinction between juveniles and adults.

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Aneeta Rattan

Full Text Available Legal precedent establishes juvenile offenders as inherently less culpable than adult offenders and thus protects juveniles from the most severe of punishments. But how fragile might these protections be? In the present study, simply bringing to mind a Black (vs. White juvenile offender led participants to view juveniles in general as significantly more similar to adults in their inherent culpability and to express more support for severe sentencing. Indeed, these differences in participants' perceptions of this foundational legal precedent distinguishing between juveniles and adults accounted for their greater support for severe punishment. These results highlight the fragility of protections for juveniles when race is in play. Furthermore, we suggest that this fragility may have broad implications for how juveniles are seen and treated in the criminal justice system.

Recurrent new-onset uveitis in a patient with rheumatoid arthritis during anti-TNFα treatment

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C. Leonetti

2011-09-01

Full Text Available Inflammation involving the uveal tract of the eye, termed uveitis, is frequently associated with various rheumatic disease, including seronegative spondylarthropathies, juvenile rheumatoid arthritis, Crohn’s disease and Behçet’s disease. Scleritis and keratitis may be associated with rheumatoid arthritis and systemic vasculitides such as Wegener’s granulomatosis. Immune-mediated uveitis can have a chronic relapsing course and produce numerous possible complications, many of which can result in permanent vision loss. Treatment typically includes topical or systemic corticosteroids with cycloplegic-mydriatic drugs and/or noncorticosteroid immunosuppressants, but often there is an insufficient clinical effectiveness. Anti-TNFα therapy is promising in the treatment of sight threatening uveitis, particularly in patients with Behçet’s disease. However, there have been also reports of new-onset uveitis during treatment of joint disease with TNFα inhibitors. We describe a case of new-onset uveitis in a patient with rheumatoid arthritis during therapy with etanercept at first and infliximab at last. Although we cannot exclude uveitis as linked to rheumatoid arthritis, it is unlike that the uveitis arises when the joint disease is well controlled. The hypothetical paradoxical effect of anti-TNF is here discussed.

Computed tomography of renal cell carcinoma in patients with terminal renal impairment

International Nuclear Information System (INIS)

Ferda, Jiri; Hora, Milan; Hes, Ondrej; Reischig, Tomas; Kreuzberg, Boris; Mirka, Hynek; Ferdova, Eva; Ohlidalova, Kristyna; Baxa, Jan; Urge, Tomas

2007-01-01

Purpose: An increased incidence of renal tumors has been observed in patients with end-stage-renal-disease (ESRD). The very strong association with acquired renal cystic disease (ACRD) and increased incidence of the renal tumors (conventional renal cell carcinoma (CRCC), papillary renal cell carcinoma (PRCC) or papillary renal cell adenoma (PRCA)) was reported. This study discusses the role of computed tomography (CT) in detecting renal tumors in patients with renal impairment: pre-dialysis, those receiving dialysis or with renal allograft transplants. Materials and methods: Ten patients (nine male, one female) with renal cell tumors were enrolled into a retrospective study; two were new dialysis patients, three on long-term dialysis, and five were renal transplant recipients with history of dialysis. All patients underwent helical CT, a total of 11 procedures were performed. Sixteen-row detector system was used five times, and a 64-row detector system for the six examinations. All patients underwent nephrectomy of kidney with suspected tumor, 15 nephrectomies were performed, and 1 kidney was assessed during autopsy. CT findings were compared with macroscopic and microscopic assessments of the kidney specimen in 16 cases. Results: Very advanced renal parenchyma atrophy with small cysts corresponding to ESRD was found in nine patients, chronic pyelonephritis in remained one. A spontaneously ruptured tumor was detected incidentally in one case, patient died 2 years later. In the present study, 6.25% (1/16) were multiple PRCA, 12.5% (2/16) were solitary PRCC, 12.5% tumors (2/16) were solitary conventional renal cell carcinomas (CRCC's), 12.5% tumors (2/16) were multiple conventional renal cell carcinomas (CRCC's), 25% (4/16) were CRCC's combined with multiple papillary renal cell carcinomas with adenomas (PRCC's and PRCA's), and 25% (4/16) of the tumors were multiple PRCC's combined with PRCA's without coexisting CRCC's. Bilateral renal tumors were found in our study

Traumatic renal infarction

International Nuclear Information System (INIS)

Yashiro, Naobumi; Ohtomo, Kuni; Kokubo, Takashi; Itai, Yuji; Iio, Masahiro

1986-01-01

Four cases of traumatic renal artery occlusion were described and illustrated. In two cases, direct blows to the abdomen compressed the renal artery against the vertebral column. Clinically, they were severely injured with macroscopic hematuria. Aortograms showed abrupt truncation of renal arteries. In the other two, rapid deceleration caused sudden displacement of the kidney producing an intimal tear with resultant thrombosis. Although they showed little injury without macrohematuria, aortograms revealed tapered occlusion of renal arteries. One of them developed hypertension. ''Rim sign'' of post-contrast CT and hypertension resulted from traumatic renal artery occlusion were reviewed. (author)

Role of Calcium Sensing Receptor in Streptozotocin-Induced Diabetic Rats Exposed to Renal Ischemia Reperfusion Injury

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Bo Hu

2018-02-01

Full Text Available Background/Aims: Renal ischemia/reperfusion (I/R injury (RI/RI is a common complication of diabetes, and it may be involved in altering intracellular calcium concentrations at its onset, which can result in inflammation, abnormal lipid metabolism, the production of reactive oxygen species (ROS, and nitroso-redox imbalance. The calcium-sensing receptor (CaSR is a G-protein coupled receptor, however, the functional involvement of CaSR in diabetic RI/ RI remains unclear. The present study was intended to investigate the role of CaSR on RI/RI in diabetes mellitus (DM. Methods: The bilateral renal arteries and veins of streptozotocin (STZ-induced diabetic rats were subjected to 45-min ischemia followed by 2-h reperfusion with or without R-568 (agonist of CaSR and NPS-2143 (antagonist of CaSR at the beginning of I/R procedure. DM without renal I/R rats served as control group. The expressions of CaSR, calmodulin (CaM, and p47phox in the renal tissue were analyzed by qRT-PCR and Western blot. The renal pathomorphology, renal function, oxidative stress, inflammatory response, and calcium disorder were evaluated by detection of a series of indices by hematoxylin-eosin (HE staining, transmission electron microscope (TEM, commercial kits, enzyme-linked immunosorbent assay (ELISA, and spectrophotofluorometry, respectively. Results: Results showed that the expressions of CaSR, CaM, and p47phox in I/R group were significantly up-regulated as compared with those in DM group, which were accompanied by renal tissue injury, increased calcium, oxidative stress, inflammation, and nitroso-redox imbalance. Conclusion: These results suggest that activation of CaSR is involved in the induction of damage of renal tubular epithelial cell during diabetic RI/RI, resulting in lipid peroxidation, inflammatory response, nitroso-redox imbalance, and apoptosis.

Criminal history and future offending of juveniles convicted of the possession of child pornography.

Science.gov (United States)

Aebi, Marcel; Plattner, Belinda; Ernest, Melanie; Kaszynski, Katie; Bessler, Cornelia

2014-08-01

Most child pornography is distributed online. It is estimated that 3% to 15% of child pornography consumers are juveniles. The present study analyzed a consecutive sample of 54 male juveniles convicted of the possession of child pornography. Demographic characteristics, criminal history, and subsequent offending were assessed from criminal files and official reports. Juvenile possessors of child pornography were compared to three different groups of juveniles: Juvenile possessors of other illegal pornography (n = 42), juveniles who committed a sexual contact offense against a child (n = 64), and juveniles who committed a sexual contact offense against a peer or adult (n = 104). Juvenile possessors of child pornography were found to have downloaded the illegal material more frequently and over a longer time period than juvenile possessors of other illegal pornography. Furthermore, juvenile possessors of child pornography differed from juveniles who had committed a sexual contact offense in terms of demographics and showed fewer previous and subsequent offending than juveniles who sexually offended against a peer or adult. We conclude that juvenile possessors of child pornography need a specific target intervention focusing on dysfunctional Internet use and sexually deviant arousal. © The Author(s) 2013.

Imaging chronic renal disease and renal transplant in children

International Nuclear Information System (INIS)

Carmichael, Jim; Easty, Marina

2010-01-01

At Great Ormond Street Hospital we have the highest number of paediatric renal transplant patients in Europe, taking cases from across the United Kingdom and abroad. Our caseload includes many children with rare complicating medical problems and chronic renal failure related morbidity. This review aims to provide an overview of our experience of imaging children with chronic renal failure and transplants. (orig.)

Cerebellar pathology in childhood-onset vs. adult-onset essential tremor.

Science.gov (United States)

Louis, Elan D; Kuo, Sheng-Han; Tate, William J; Kelly, Geoffrey C; Faust, Phyllis L

2017-10-17

Although the incidence of ET increases with advancing age, the disease may begin at any age, including childhood. The question arises as to whether childhood-onset ET cases manifest the same sets of pathological changes in the cerebellum as those whose onset is during adult life. We quantified a broad range of postmortem features (Purkinje cell [PC] counts, PC axonal torpedoes, a host of associated axonal changes [PC axonal recurrent collateral count, PC thickened axonal profile count, PC axonal branching count], heterotopic PCs, and basket cell rating) in 60 ET cases (11 childhood-onset and 49 adult-onset) and 30 controls. Compared to controls, childhood-onset ET cases had lower PC counts, higher torpedo counts, higher heterotopic PC counts, higher basket cell plexus rating, and marginally higher PC axonal recurrent collateral counts. The median PC thickened axonal profile count and median PC axonal branching count were two to five times higher in childhood-onset ET than controls, but the differences did not reach statistical significance. Childhood-onset and adult-onset ET had similar PC counts, torpedo counts, heterotopic PC counts, basket cell plexus rating, PC axonal recurrent collateral counts, PC thickened axonal profile count and PC axonal branching count. In conclusion, we found that childhood-onset and adult-onset ET shared similar pathological changes in the cerebellum. The data suggest that pathological changes we have observed in the cerebellum in ET are a part of the pathophysiological cascade of events in both forms of the disease and that both groups seem to reach the same pathological endpoints at a similar age of death. Copyright © 2017 Elsevier B.V. All rights reserved.

Absorption fever characteristics due to percutaneous renal biopsy-related hematoma.

Science.gov (United States)

Hu, Tingyang; Liu, Qingquan; Xu, Qin; Liu, Hui; Feng, Yan; Qiu, Wenhui; Huang, Fei; Lv, Yongman

2016-09-01

This study aims to describe the unique characteristics of absorption fever in patients with a hematoma after percutaneous renal biopsy (PRB) and distinguish it from secondary infection of hematoma.We retrospectively studied 2639 percutaneous renal biopsies of native kidneys. We compared the clinical characteristics between 2 groups: complication group (gross hematuria and/or perirenal hematoma) and no complication group. The axillary temperature of patients with a hematoma who presented with fever was measured at 06:00, 10:00, 14:00, and 18:00. The onset and duration of fever and the highest body temperature were recorded. Thereafter, we described the time distribution of absorption fever and obtained the curve of fever pattern.Of 2639 patients, PRB complications were observed in 154 (5.8%) patients. Perirenal hematoma was the most common complication, which occurred in 118 (4.5%) of biopsies, including 74 small hematoma cases (thickness ≤3 cm) and 44 large hematoma cases (thickness >3 cm). Major complications were observed in only 6 (0.2%) cases resulting from a large hematoma. Of 118 patients with a perirenal hematoma, absorption fever was observed in 48 cases. Furthermore, large hematomas had a 5.23-fold higher risk for absorption fever than the small ones.Blood pressure, renal insufficiency, and prothrombin time could be risk factors for complications. Fever is common in patients with hematoma because of renal biopsy and is usually noninfectious. Evaluation of patients with post-biopsy fever is necessary to identify any obvious infection sources. If no focus is identified, empiric antibiotic therapy should not be initiated nor should prophylactic antibiotics be extended for prolonged durations. Absorption fevers will resolve in time without specific therapeutic interventions.

Large cell non-Hodgkin's lymphoma masquerading as renal carcinoma with inferior vena cava thrombosis: a case report

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Weissman Alan

2011-06-01

Full Text Available Abstract Introduction Many cancers are associated with inferior vena cava (IVC obstruction, but very few cancers have the ability to propagate within the lumen of the renal vein or the IVC. Renal cell carcinoma is the most common of these cancers. Renal cancer with IVC extension has a high rate of recurrence and a low five year survival rate. Case presentation A 62-year-old Caucasian woman previously in good health developed the sudden onset of severe reflux symptoms and right-sided abdominal pain that radiated around the right flank. A subsequent ultrasound and CT scan revealed a right upper pole renal mass with invasion of the right adrenal gland, liver, left renal vein and IVC. This appeared to be consistent with stage III renal cancer with IVC extension. Metastatic nodules were believed to be present in the right pericardial region; the superficial anterior abdominal wall; the left perirenal, abdominal and pelvic regions; and the left adrenal gland. The pattern of these metastases, as well as the invasion of the liver by the tumor, was thought to be atypical of renal cancer. A needle biopsy of a superficial abdominal wall mass revealed a surprising finding: The malignant cells were diagnostic of large-cell, B-cell non-Hodgkin's lymphoma. The lymphoma responded dramatically to systemic chemotherapy, which avoided the need for nephrectomy. Conclusion Lymphomas only rarely progress via intraluminal vascular extension. We have been able to identify only one other case report of renal lymphoma with renal vein and IVC extension. While renal cancer would have been treated with radical nephrectomy and tumor embolectomy, large-cell B-cell lymphomas are treated primarily with chemotherapy, and nephrectomy would have been detrimental. It is important to remember that, rarely, other types of cancer arise from the kidney which are not derived from the renal tubular epithelium. These may be suspected if an atypical pattern of metastases or unusual

Attention capture by contour onsets and offsets: no special role for onsets.

Science.gov (United States)

Watson, D G; Humphreys, G W

1995-07-01

In five experiments, we investigated the power of targets defined by the onset or offset of one of an object's parts (contour onsets and offsets) either to guide or to capture visual attention. In Experiment 1, search for a single contour onset target was compared with search for a single contour offset target against a static background of distractors; no difference was found between the efficiency with which each could be detected. In Experiment 2, onsets and offsets were compared for automatic attention capture, when both occurred simultaneously. Unlike in previous studies, the effects of overall luminance change, new-object creation, and number of onset and offset items were controlled. It was found that contour onset and offset items captured attention equally well. However, display size effects on both target types were also apparent. Such effects may have been due to competition for selection between multiple onset and offset stimuli. In Experiments 3 and 4, single onset and offset stimuli were presented simultaneously and pitted directly against one another among a background of static distractors. In Experiment 3, we examined "guided search," for a target that was formed either from an onset or from an offset among static items. In Experiment 4, the onsets and offsets were uncorrelated with the target location. Similar results occurred in both experiments: target onsets and offsets were detected more efficiently than static stimuli which needed serial search; there remained effects of display size on performance; but there was still no advantage for onsets. In Experiment 5, we examined automatic attention capture by single onset and offset stimuli presented individually among static distractors. Again, there was no advantage for onset over offset targets and a display size effect was also present. These results suggest that, both in isolation and in competition, onsets that do not form new objects neither guide nor gain automatic attention more efficiently

Growth of juvenile shrimp Metapenaeus monoceros fed with squid and mussel

Digital Repository Service at National Institute of Oceanography (India)

Achuthankutty, C.T.; Nair, S.R.S.; Krishnakumari, L.

Small juveniles of both sexes and females of large juveniles of Metapenaeus monoceros attained faster growth with squid diet. Males of large juveniles registered better growth with mussel diet. No significant difference was observed in moult weights...

Radionuclide evaluation of renal transplants

International Nuclear Information System (INIS)

Yang Hong; Zhao Deshan

2000-01-01

Radionuclide renal imaging and plasma clearance methods can quickly quantitate renal blood flow and function in renal transplants. They can diagnose acute tubular necrosis and rejection, renal scar, surgical complications such as urine leaks, obstruction and renal artery stenosis after renal transplants. At the same time they can assess the therapy effect of renal transplant complications and can also predict renal transplant survival from early post-operative function studies

IL-1 inhibition in systemic juvenile idiopathic arthritis

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Gabriella Giancane

2016-12-01

Full Text Available Systemic juvenile idiopathic arthritis (sJIA is the form of childhood arthritis whose treatment is most challenging. The demonstration of the prominent involvement of interleukin (IL-1 in disease pathogenesis has provided the rationale for the treatment with biologic medications that antagonize this cytokine. The three IL-1 blockers that have been tested so far (anakinra, canakinumab and rilonacept have all been proven effective and safe, although only canakinumab is currently approved for use in sJIA. The studies on IL-1 inhibition in sJIA published in the past few years suggest that children with fewer affected joints, higher neutrophil count, younger age at disease onset, shorter disease duration, or, possibly, higher ferritin level may respond better to anti-IL-1 treatment. In addition, it has been postulated that use of IL-1 blockade as first-line therapy may take advantage of a window of opportunity, in which disease pathophysiology can be altered to prevent the occurrence of chronic arthritis. In this review, we analyze the published literature on IL-1 inhibitors in sJIA and discuss the rationale underlying the use of these medications, the results of therapeutic studies, and the controversial issues.

Contrast-enhanced MRI features in the early diagnosis of Juvenile Idiopathic Arthritis

International Nuclear Information System (INIS)

Hemke, Robert; Maas, Mario; Kuijpers, Taco W.; Schonenberg-Meinema, Dieneke; Nusman, Charlotte M.; Rossum, Marion A.J. van; Berg, J.M. van den; Dolman, Koert M.

2015-01-01

To determine whether clinical, laboratory or Magnetic Resonance Imaging (MRI) measures differentiate Juvenile Idiopathic Arthritis (JIA) from other forms of active childhood arthritis. We prospectively collected data of 80 treatment-naive patients clinically suspected of JIA with active non-infectious arthritis of (at least) one knee for <12 months duration. Upon presentation patients underwent clinical and laboratory assessments and contrast-enhanced MRI. MRI was not used as a diagnostic criterion. Forty-four (55 %) patients were clinically diagnosed with JIA, whereas in 36 (45 %) patients the diagnosis of JIA was discarded on clinical or laboratory findings. MRI-based synovitis was present in 27 (61.4 %) JIA patients and in 7 (19.4 %) non-JIA patients (P < 0.001). Five factors (male gender, physician's global assessment of overall disease activity, joints with limited range of motion, HLA-B27, MRI-based synovitis) were associated with the onset of JIA. In multivariate analysis MRI-based synovitis proved to be independently associated with JIA (OR 6.58, 95 % CI 2.36-18.33). In patients with MRI-based synovitis, the RR of having JIA was 3.16 (95 % CI 1.6-6.4). The presence of MRI-based synovitis is associated with the clinical onset of JIA. Physical examination could be supported by MRI, particularly to contribute in the early differentiation of different forms of non-infectious childhood arthritis. (orig.)

Contrast-enhanced MRI features in the early diagnosis of Juvenile Idiopathic Arthritis

Energy Technology Data Exchange (ETDEWEB)

Hemke, Robert; Maas, Mario [University of Amsterdam, Department of Radiology Academic Medical Center, Amsterdam (Netherlands); Kuijpers, Taco W.; Schonenberg-Meinema, Dieneke [University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Nusman, Charlotte M. [University of Amsterdam, Department of Radiology Academic Medical Center, Amsterdam (Netherlands); University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Rossum, Marion A.J. van; Berg, J.M. van den [University of Amsterdam, Department of Pediatric Hematology, Immunology, Rheumatology and Infectious Disease, Emma Children' s Hospital AMC, Amsterdam (Netherlands); Department of Pediatric Rheumatology, Reade, Amsterdam (Netherlands); Dolman, Koert M. [Department of Pediatric Rheumatology, Reade, Amsterdam (Netherlands); St. Lucas Andreas Hospital, Department of Pediatrics, Amsterdam (Netherlands)

2015-11-15

To determine whether clinical, laboratory or Magnetic Resonance Imaging (MRI) measures differentiate Juvenile Idiopathic Arthritis (JIA) from other forms of active childhood arthritis. We prospectively collected data of 80 treatment-naive patients clinically suspected of JIA with active non-infectious arthritis of (at least) one knee for <12 months duration. Upon presentation patients underwent clinical and laboratory assessments and contrast-enhanced MRI. MRI was not used as a diagnostic criterion. Forty-four (55 %) patients were clinically diagnosed with JIA, whereas in 36 (45 %) patients the diagnosis of JIA was discarded on clinical or laboratory findings. MRI-based synovitis was present in 27 (61.4 %) JIA patients and in 7 (19.4 %) non-JIA patients (P < 0.001). Five factors (male gender, physician's global assessment of overall disease activity, joints with limited range of motion, HLA-B27, MRI-based synovitis) were associated with the onset of JIA. In multivariate analysis MRI-based synovitis proved to be independently associated with JIA (OR 6.58, 95 % CI 2.36-18.33). In patients with MRI-based synovitis, the RR of having JIA was 3.16 (95 % CI 1.6-6.4). The presence of MRI-based synovitis is associated with the clinical onset of JIA. Physical examination could be supported by MRI, particularly to contribute in the early differentiation of different forms of non-infectious childhood arthritis. (orig.)

Juvenile technologies in foreign publications

Directory of Open Access Journals (Sweden)

Shpagina E.M.

2012-09-01

Full Text Available The article provides the review of foreign publications, concerning the juvenile technologies used in France, Canada, Germany and Switzerland. The paper presents legal, social and psychotherapeutic aspects of juvenile judiciary in foreign countries. The authors paid special attention to the complexity of approaches to young children and teenagers who found themselves in complicated life circumstances or got into trouble with the law. The article gives examples of using the following techniques: cognitive-behavioral intervention, mediation, family therapy (including family background and family history, relations theory, narrative practices, utilization of «emotional intelligence» resources.

Development of Acute Pericarditis Associated with New-onset Rheumatoid Arthritis in a Diabetic Patient with Renal Impairment: The Elusive Nature of Uremia.

Science.gov (United States)

Kohara, Marina; Takeda, Shin-ichi; Miki, Takuya; Ohara, Ken; Yamanaka, Yuko; Kawamata, Mutsumi; Hishida, Erika; Wakabayashi, Natsuko; Yamazaki, Tomoyuki; Ichida, Masaru; Akimoto, Tetsu; Muto, Shigeaki; Nagata, Daisuke

2016-01-01

Uremic patients may have a variety of organ involvement, however, the precise causality may be impossible to determine in some cases because the symptoms of uremia are also associated with other diseases. With an emphasis on the elusive nature of uremia, we herein describe a 53-year-old man with preexisting renal impairment who developed acute pericarditis with deterioration of his renal function. Hemodialysis was immediately initiated on the presumption of uremia, however, articular symptoms emerged approximately a month later and led to a final diagnosis of rheumatoid arthritis, followed by successful withdrawal of hemodialysis.

Corporal and capital punishment of juveniles.

Science.gov (United States)

Frazier, H C

1990-01-01

There is a previously unobserved connection between corporal punishment of public school children and capital punishment of juveniles. Both are barometers of acceptable levels of violent punishment and their elimination is a hallmark of a maturing and decent society. Within a majority of the eighteen states where school authorities most frequently strike children are housed 25 of the nation's 28 juvenile death row inmates. On average, the homicide rates of these jurisdictions are two and a half times greater than those that have abolished both state-sanctioned corporal and capital punishment or limit death sentences to those age eighteen and older at the time of their crime(s). Most of the eighteen state abolitions of corporal punishment occurred in the 1980's. The US Supreme Court has ruled both corporal and capital punishment of juveniles constitutional. Additional state legislative abolition of both is anticipated in the 1990s.

Renal Replacement Therapy Modality in the ICU and Renal Recovery at Hospital Discharge.

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Bonnassieux, Martin; Duclos, Antoine; Schneider, Antoine G; Schmidt, Aurélie; Bénard, Stève; Cancalon, Charlotte; Joannes-Boyau, Olivier; Ichai, Carole; Constantin, Jean-Michel; Lefrant, Jean-Yves; Kellum, John A; Rimmelé, Thomas

2018-02-01

Acute kidney injury requiring renal replacement therapy is a major concern in ICUs. Initial renal replacement therapy modality, continuous renal replacement therapy or intermittent hemodialysis, may impact renal recovery. The aim of this study was to assess the influence of initial renal replacement therapy modality on renal recovery at hospital discharge. Retrospective cohort study of all ICU stays from January 1, 2010, to December 31, 2013, with a "renal replacement therapy for acute kidney injury" code using the French hospital discharge database. Two hundred ninety-one ICUs in France. A total of 1,031,120 stays: 58,635 with renal replacement therapy for acute kidney injury and 25,750 included in the main analysis. None. PPatients alive at hospital discharge were grouped according to initial modality (continuous renal replacement therapy or intermittent hemodialysis) and included in the main analysis to identify predictors of renal recovery. Renal recovery was defined as greater than 3 days without renal replacement therapy before hospital discharge. The main analysis was a hierarchical logistic regression analysis including patient demographics, comorbidities, and severity variables, as well as center characteristics. Three sensitivity analyses were performed. Overall mortality was 56.1%, and overall renal recovery was 86.2%. Intermittent hemodialysis was associated with a lower likelihood of recovery at hospital discharge; odds ratio, 0.910 (95% CI, 0.834-0.992) p value equals to 0.0327. Results were consistent across all sensitivity analyses with odds/hazards ratios ranging from 0.883 to 0.958. In this large retrospective study, intermittent hemodialysis as an initial modality was associated with lower renal recovery at hospital discharge among patients with acute kidney injury, although the difference seems somewhat clinically limited.

Technical aspects of renal denervation in end-stage renal disease patients with challenging anatomy.

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Spinelli, Alessio; Da Ros, Valerio; Morosetti, Daniele; Onofrio, Silvia D; Rovella, Valentina; Di Daniele, Nicola; Simonetti, Giovanni

2014-01-01

We describe our preliminary experience with percutaneous renal denervation in end-stage renal disease patients with resistant hypertension and challenging anatomy, in terms of the feasibility, safety, and efficacy of this procedure. Four patients with end-stage renal disease patients with resistant hypertension (mean hemodialysis time, 2.3 years) who had been taking at least four antihypertensive medications underwent percutaneous renal denervation. Renal artery eligibility included the absence of prior renal artery interventions, vessel stenosis renal denervation is a feasible approach for end-stage renal disease patients with resistant hypertension with encouraging short-term preliminary results in terms of procedural efficacy and safety.

Renal rescue of dopamine D2 receptor function reverses renal injury and high blood pressure

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Konkalmatt, Prasad R.; Asico, Laureano D.; Zhang, Yanrong; Yang, Yu; Drachenberg, Cinthia; Zheng, Xiaoxu; Han, Fei; Jose, Pedro A.; Armando, Ines

2016-01-01

Dopamine D2 receptor (DRD2) deficiency increases renal inflammation and blood pressure in mice. We show here that long-term renal-selective silencing of Drd2 using siRNA increases renal expression of proinflammatory and profibrotic factors and blood pressure in mice. To determine the effects of renal-selective rescue of Drd2 expression in mice, the renal expression of DRD2 was first silenced using siRNA and 14 days later rescued by retrograde renal infusion of adeno-associated virus (AAV) vector with DRD2. Renal Drd2 siRNA treatment decreased the renal expression of DRD2 protein by 55%, and DRD2 AAV treatment increased the renal expression of DRD2 protein by 7.5- to 10-fold. Renal-selective DRD2 rescue reduced the expression of proinflammatory factors and kidney injury, preserved renal function, and normalized systolic and diastolic blood pressure. These results demonstrate that the deleterious effects of renal-selective Drd2 silencing on renal function and blood pressure were rescued by renal-selective overexpression of DRD2. Moreover, the deleterious effects of 45-minute bilateral ischemia/reperfusion on renal function and blood pressure in mice were ameliorated by a renal-selective increase in DRD2 expression by the retrograde ureteral infusion of DRD2 AAV immediately after the induction of ischemia/reperfusion injury. Thus, 14 days after ischemia/reperfusion injury, the renal expression of profibrotic factors, serum creatinine, and blood pressure were lower in mice infused with DRD2 AAV than in those infused with control AAV. These results indicate an important role of renal DRD2 in limiting renal injury and preserving normal renal function and blood pressure. PMID:27358912

Tc-99m-DMSA renal uptake rate and renal volume of elderly persons

International Nuclear Information System (INIS)

Ohishi, Yukihiko; Machida, Toyohei; Kido, Akira

1987-01-01

Renal function of erderly persons was evaluated by the radionuclide renal function test based on the renal uptake rate and the renal volume determined by Tc-99m-DMSA transectional tomographic images using single photon emission computed tomography (SPECT). Forty-three erderly cases (13 healthy persons and 30 patients with various types of renal disorders) aged between 60 and 87 on an average of 70 were studied and compared with results obtained from 20 healthy adults (18 - 45 years old). Renal volume was calculated from the summation of voxels in the region districted by equi-count threshold level (percentage to maximum count) on each section of the SPECT image. Attenuation correction was made by GE-STAR protocol utilizing Sorrenson's precorrection method. The renal uptake rate was expressed as a percentage of the total radioactivity detected within the renal volume, against an amount of dose injected. In the 26 kidneys of 13 healthy elderly persons, Tc-99m-DMSA renal uptake was 23 ± 5 %, which was significantly lower (p < 0.01) than that of healthy adults being 27 ± 2 %. A correlation coefficient between renal volume and uptake of 79 kidneys of 43 elderly persons was 0.5081 (p < 0.01). Creatinine clearance (Ccr) was better correlated with the total renal uptake (r = 0.6471, p < 0.01) than with the total renal volume (r = 0.3592, p < 0.01). This method is considered to be useful for clinical purpose as a test of renal function for elderly persons since it requires neither blood nor urine samples. (author)

The efficacy of hemodialysis in interventional therapy in coronary artery disease patients with chronic renal insufficiency.

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Zhai, Hongxia; Li, Liang; Yin, Yaxin; Zhang, Jinjin; Chen, Haiwei; Liu, Runmei; Xia, Yun-feng

2016-01-01

The aim of this study was to explore the efficacy and safety of hemodialysis in interventional therapy for patients with coronary artery disease combined with chronic renal insufficiency. With the aging and social development, the number of coronary artery disease patients with chronic renal insufficiency gradually increased. Total 58 coronary heart disease patients with chronic renal dysfunction were selected. These patients were characterized with typical angina symptoms and typical electrocardiogram (ECG) changes of onset angina. Continuous oral administration of sodium bicarbonate tablets 1 g 3/day × 3 days and slow intravenous input sodium chloride 1000 ∼1500 mL 3-12 h before operation were given. By this way, all patients were treated by hydration and alkalization. After percutaneous coronary intervention (PCI) treatment, patients were immediately transferred to undergo 4 h of dialysis treatment without removing indwelling of femoral artery puncture sheath tube to protect renal function. Changes in renal function including serum creatinine, glomerular filtration rate, and urine were observed and recorded. All patients were successfully underwent PCI treatment. Within one month after PCI, there were no obvious complication and no stent thrombosis occurred. Among of 58 patients, 56 cases showed no significant increase in serum creatinine levels compared with those before operation. However, serum creatinine level of one patient increased to 251 umol/L and one patient still required permanent dialysis. Using hemodialysis in interventional therapy in coronary artery disease patients with chronic renal insufficiency could significantly improve the prognosis of the patients.

Early renin-angiotensin system intervention is more beneficial than late intervention in delaying end-stage renal disease in patients with type 2 diabetes

DEFF Research Database (Denmark)

Schievink, B; Kröpelin, T; Mulder, S

2016-01-01

AIMS: To develop and validate a model to simulate progression of diabetic kidney disease (DKD) from early onset until end-stage renal disease (ESRD), and to assess the effect of renin-angiotensin system (RAS) intervention in early, intermediate and advanced stages of DKD. METHODS: We used data from...

28 CFR 0.94 - Office of Juvenile Justice and Delinquency Prevention.

Science.gov (United States)

2010-07-01

... Delinquency Prevention. 0.94 Section 0.94 Judicial Administration DEPARTMENT OF JUSTICE ORGANIZATION OF THE... Delinquency Prevention. The Office of Juvenile Justice and Delinquency Prevention is headed by an...., relating to juvenile delinquency, the improvement of juvenile justice systems and missing children. ...

Online feedback-controlled renal constant infusion clearances in rats.

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Schock-Kusch, Daniel; Shulhevich, Yury; Xie, Qing; Hesser, Juergen; Stsepankou, Dzmitry; Neudecker, Sabine; Friedemann, Jochen; Koenig, Stefan; Heinrich, Ralf; Hoecklin, Friederike; Pill, Johannes; Gretz, Norbert

2012-08-01

Constant infusion clearance techniques using exogenous renal markers are considered the gold standard for assessing the glomerular filtration rate. Here we describe a constant infusion clearance method in rats allowing the real-time monitoring of steady-state conditions using an automated closed-loop approach based on the transcutaneous measurement of the renal marker FITC-sinistrin. In order to optimize parameters to reach steady-state conditions as fast as possible, a Matlab-based simulation tool was established. Based on this, a real-time feedback-regulated approach for constant infusion clearance monitoring was developed. This was validated by determining hourly FITC-sinistrin plasma concentrations and the glomerular filtration rate in healthy and unilaterally nephrectomized rats. The transcutaneously assessed FITC-sinistrin fluorescence signal was found to reflect the plasma concentration. Our method allows the precise determination of the onset of steady-state marker concentration. Moreover, the steady state can be monitored and controlled in real time for several hours. This procedure is simple to perform since no urine samples and only one blood sample are required. Thus, we developed a real-time feedback-based system for optimal regulation and monitoring of a constant infusion clearance technique.

Parental Low Self-Control, Family Environments, and Juvenile Delinquency.

Science.gov (United States)

Meldrum, Ryan C; Connolly, George M; Flexon, Jamie; Guerette, Rob T

2016-10-01

Research consistently finds that low self-control is significantly correlated with delinquency. Only recently, however, have researchers started to examine associations between parental low self-control, family environments, and child antisocial behavior. Adding to this emerging area of research, the current study examines associations between parental low self-control, aspects of the family environment, and officially recoded juvenile delinquency among a sample (N = 101) of juveniles processed through a juvenile justice assessment facility located in the Southeastern United States. Furthermore, it considers whether aspects of family environments, particularly family cohesion, family conflict, and parental efficacy, mediate the influence of parental low self-control on delinquency. The results of a series of analyses indicate that parental low self-control is correlated with various aspects of family environments and juvenile delinquency, and that the association between parental low self-control and juvenile delinquency is mediated by family environments. Supplementary analyses also suggest that the association between parental low self-control and the family environment may be reciprocal. © The Author(s) 2015.

Temperament of juvenile delinquents with history of substance abuse.

Science.gov (United States)

Chang, Hsueh-Ling; Chen, Sue-Huei; Huang, Chien

2007-01-01

The etiological factors and interrelations of juvenile delinquents, with psychiatric morbidity and substance abuse have been continuously debated. Cloninger's Tridimensional Theory of Temperament has been reported to predict patterns of substance abuse and comorbidity. In the current study, we aimed to examine the usability of the theory in predicting juvenile delinquency and substance abuse. Sixty consecutive and newly incarcerated male delinquents with history of substance abuse were recruited from a juvenile correctional facility in northwestern Taiwan from January 2002 through December 2003. All subjects were assessed of their temperament, behavioral problems, and psychiatric disorders on an individual base. The juvenile delinquent subjects with childhood history of attention deficit and hyperactivity disorder (ADHD) were significantly younger, consumed less betel nuts, and had more siblings with history of drug abuse. Consistent with the results of Cloninger's studies, novelty seeking positively correlated to the amount of substance abuse, while harm avoidance inversely correlated in juvenile delinquents. Endemic trend of choice of substance abuse needs to be taken into consideration in future research projects.

Factors Associated with the Incidence and Severity of New-Onset Atrial Fibrillation in Adult Critically Ill Patients

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Péricles A. D. Duarte

2017-01-01

Full Text Available Background. Acute Atrial Fibrillation (AF is common in critically ill patients, with significant morbidity and mortality; however, its incidence and severity in Intensive Care Units (ICUs from low-income countries are poorly studied. Additionally, impact of vasoactive drugs on its incidence and severity is still not understood. This study aimed to assess epidemiology and risk factors for acute new-onset AF in critically ill adult patients and the role of vasoactive drugs. Method. Cohort performed in seven general ICUs (including cardiac surgery in three cities in Paraná State (southern Brazil for 45 days. Patients were followed until hospital discharge. Results. Among 430 patients evaluated, the incidence of acute new-onset AF was 11.2%. Patients with AF had higher ICU and hospital mortality. Vasoactive drugs use (norepinephrine and dobutamine was correlated with higher incidence of AF and higher mortality in patients with AF; vasopressin (though used in few patients had no effect on development of AF. Conclusions. In general ICU patients, incidence of new-onset AF was 11.2% with a high impact on morbidity and mortality, particularly associated with the presence of Acute Renal Failure. The use of vasoactive drugs (norepinephrine and dobutamine could lead to a higher incidence of new-onset AF-associated morbidity and mortality.

Blood Pressure Response to Main Renal Artery and Combined Main Renal Artery Plus Branch Renal Denervation in Patients With Resistant Hypertension.

Science.gov (United States)

Fengler, Karl; Ewen, Sebastian; Höllriegel, Robert; Rommel, Karl-Philipp; Kulenthiran, Saaraaken; Lauder, Lucas; Cremers, Bodo; Schuler, Gerhard; Linke, Axel; Böhm, Michael; Mahfoud, Felix; Lurz, Philipp

2017-08-10

Single-electrode ablation of the main renal artery for renal sympathetic denervation showed mixed blood pressure (BP)-lowering effects. Further improvement of the technique seems crucial to optimize effectiveness of the procedure. Because sympathetic nerve fibers are closer to the lumen in the distal part of the renal artery, treatment of the distal main artery and its branches has been shown to reduce variability in treatment effects in preclinical studies and a recent randomized trial. Whether this optimized technique improves clinical outcomes remains uncertain. We report a 2-center experience of main renal artery and combined main renal artery plus branches renal denervation in patients with resistant hypertension using a multielectrode catheter. Twenty-five patients with therapy-resistant hypertension underwent renal sympathetic denervation with combined main renal artery and renal branch ablation and were compared to matched controls undergoing an ablation of the main renal artery only. BP change was assessed by ambulatory measurement at baseline and after 3 months. At baseline, BP was balanced between the groups. After 3 months, BP changed significantly in the combined ablation group (systolic/diastolic 24-hour mean and daytime mean BP -8.5±9.8/-7.0±10.7 and -9.4±9.8/-7.1±13.5 mm Hg, P renal artery and branches appears to improve BP-lowering efficacy and should be further investigated. © 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

Renal angiographic and computed tomographic evaluation of local extension of renal cell carcinoma

International Nuclear Information System (INIS)

Masuda, Fujio; Onishi, Tetsuro; Sasaki, Tadamasa; Arai, Yoshikazu; Shoji, Ryo

1981-01-01

In 23 cases of renal cell carcinoma, the degree of local invasion of carcinoma was diagnosed using renal angiography and CT, and compared with the findings obtained by operation or autopsy. Among 5 cases in which the tumor was confined to the renal capsule, accurate diagnosis could be established with renal angiography in 4 cases and with CT in all of 5 cases. Both renal angiography and CT provided correct diagnosis in 7 of 8 cases in which the tumor showed infiltration extending to the perinephric fat. Out of 5 cases with tumor invasion of renal vein or inferior vena cava, diagnosis could be established correctly by renal angiography and CT in 3 cases. Among the remaining 2 cases the diagnosis could be established by renal angiography and CT in one each case. Among 5 cases with metastases to the regional lymph nodes, diagnosis could be established by renal angiography in only 2 cases, while all of 5 cases could be diagnosed by CT. In 3 cases where the tumor invaded an ajacent organ beyond Gerota's fastia, renal angiography could diagnose in none of the 3 cases while with CT all of 3 cases could successfully be diagnosed. The consistency of degree of local invasion as revealed by renal angiography and CT was seen in 15 of 23 cases (65%) for renal agniography and 20 of 23 cases (87%) for CT, indicating superiority of CT in this respect. In particular, CT appears to be more superior to renal angiography for determining whether the tumor confined to Gerota's fastia or it infiltrated over it. Both combined use of renal angiography and CT, the degree of infiltration of tumor could be diagnosed correctly in 22 of 23 cases (96%). (author)

Prevention and Firesetting: Juvenile Justice and Intervention Strategies.

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Slavkin, Michael L.

2003-01-01

Examines the literature on preventing firesetting behavior in preadolescents and adolescents, suggesting the need for policies and programs designed to help juveniles by providing community support and stability. Alternatives to juvenile justice interventions include making changes in the home environment, acquiring a greater sense of self, and…

Dental and facial characteristics of patients with juvenile idiopathic arthritis Características dentárias e faciais de pacientes com artrite idiopática juvenil

Directory of Open Access Journals (Sweden)

Cynthia Savioli

2004-01-01

Full Text Available OBJECTIVE: It has been shown that the temporomandibular joint is frequently affected by juvenile idiopathic arthritis, and this degenerative disease, which may occur during facial growth, results in severe mandibular dysfunction. However, there are no studies that correlate oral health (tooth decay and gingival diseases and temporomandibular joint dysfunction in patients with juvenile idiopathic arthritis. The aim of this study is to evaluate the oral and facial characteristics of the patients with juvenile idiopathic arthritis treated in a large teaching hospital. METHOD: Thirty-six patients with juvenile idiopathic arthritis (26 female and 10 male underwent a systematic clinical evaluation of their dental, oral, and facial structures (DMFT index, plaque and gingival bleeding index, dental relationship, facial profile, and Helkimo's index. The control group was composed of 13 healthy children. RESULTS: The mean age of the patients with juvenile idiopathic arthritis was 10.8 years; convex facial profile was present in 12 juvenile idiopathic arthritis patients, and class II molar relation was present in 12 (P = .032. The indexes of plaque and gingival bleeding were significant in juvenile idiopathic arthritis patients with a higher number of superior limbs joints involved (P = .055. Anterior open bite (5 and temporomandibular joint noise (8 were present in the juvenile idiopathic arthritis group. Of the group in this sample, 94% (P = .017 had temporomandibular joint dysfunction, 80% had decreased mandibular opening (P = 0.0002, and mandibular mobility was severely impaired in 33% (P = .015. CONCLUSION: This study confirms that patients with juvenile idiopathic arthritis a have a high incidence of mandibular dysfunction that can be attributed to the direct effect of the disease in the temporomandibular joint and b have a higher incidence of gingival disease that can be considered a secondary effect of juvenile idiopathic arthritis on oral health

Axillary brachial plexus block duration with mepivacaine in patients with chronic renal failure. Case-control study.

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Mojica, V; Nieuwveld, D; Herrera, A E; Mestres, G; López, A M; Sala-Blanch, X

2017-04-01

Regional anaesthesia is commonly preferred for arteriovenous fistula (AVF) creation. Previous studies suggest a shorter block duration in patients with chronic renal failure, maybe because of the changes in regional blood flow. The aim of our study was to evaluate the duration of the axillary block with 1.5% mepivacaine in patients with chronic renal failure scheduled for AVF compared with healthy controls. Patients scheduled for AVF creation for the first time (GIRC) were included. They were compared with patients without renal failure (GC), with similar anthropometric characteristics. Ultrasound-guided axillary blocks with 20mL of 1.5% mepivacaine were performed on all patients. We evaluated onset time, humeral artery diameter and blood flow before and after the block, as well as the block duration. Twenty-three patients (GIRC: 12 and GC: 11) were included. No differences between groups were observed in block duration (GIRC: 227±43min vs GC: 229±27min; P=.781), or in onset time (GIRC: 13±5min vs GC: 12.2±3min; P=.477). The humeral blood flow before and after block was significantly lower in the GIRC (pre-block: GIRC: 52±21ml/min GC: 100±62ml/min; P=.034 and p ost block: GIRC: 130±57ml/min and GC: 274±182ml/min; P=.010). There was no significant correlation between the duration of the block and the preblock humeral blood flow (Spearman Rho: 0.106; P=.657) or the postblock humeral blood flow (Spearman Rho: 0.267; P=.254). The duration of the axillary block with 1.5% mepivacaine in patients with chronic renal failure was similar to that of the control patients. The duration of axillary brachial plexus block seems not to be related to changes in regional blood flow. Copyright © 2016 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.

Vascular toxicity of urea, a new "old player" in the pathogenesis of chronic renal failure induced cardiovascular diseases.

Science.gov (United States)

Giardino, Ida; D'Apolito, Maria; Brownlee, Michael; Maffione, Angela Bruna; Colia, Anna Laura; Sacco, Michele; Ferrara, Pietro; Pettoello-Mantovani, Massimo

2017-12-01

Chronic kidney disease in children is an irreversible process that may lead to end-stage renal disease. The mortality rate in children with end-stage renal disease who receive dialysis increased dramatically in the last decade, and it is significantly higher compared with the general pediatric population. Furthermore, dialysis and transplant patients, who have developed end-stage renal disease during childhood, live respectively far less as compared with age/race-matched populations. Different reports show that cardiovascular disease is the leading cause of death in children with end-stage renal disease and in adults with childhood-onset chronic kidney disease, and that children with chronic kidney disease are in the highest risk group for the development of cardiovascular disease. Urea, which is generated in the liver during catabolism of amino acids and other nitrogenous metabolites, is normally excreted into the urine by the kidneys as rapidly as it is produced. When renal function is impaired, increasing concentrations of blood urea will steadily accumulate. For a long time, urea has been considered to have negligible toxicity. However, the finding that plasma urea is the only significant predictor of aortic plaque area fraction in an animal model of chronic renal failure -accelerated atherosclerosis, suggests that the high levels of urea found in chronic dialysis patients might play an important role in accelerated atherosclerosis in this group of patients. The aim of this review was to provide novel insights into the role played by urea in the pathogenesis of accelerated cardiovascular disease in renal failure.

Neurofibromatosis Type 1 Diagnosed in a Child Based on Multiple Juvenile Xanthogranulomas and Juvenile Myelomonocytic Leukemia

DEFF Research Database (Denmark)

Jans, Sune R R; Schomerus, Eckhard; Bygum, Anette

2015-01-01

An association between juvenile xanthogranuloma (JXG), neurofibromatosis type 1 (NF1), and juvenile myelomonocytic leukemia (JMML) has been described in the literature but has only been documented in approximately 20 cases. We diagnosed a patient with NF1 at 25 months of age, before any cutaneous...... with chemotherapy and allogenic bone marrow transplantation. With increased awareness, patients with JXG and NF1 who develop symptoms possibly related to JMML, such as paleness, skin bleeding, cough, unexplained fever, and hepatosplenomegaly, should be further evaluated. We also emphasize that multiple JXG lesions...

Clinical application of calculated split renal volume using computed tomography-based renal volumetry after partial nephrectomy: Correlation with technetium-99m dimercaptosuccinic acid renal scan data.

Science.gov (United States)

Lee, Chan Ho; Park, Young Joo; Ku, Ja Yoon; Ha, Hong Koo

2017-06-01

To evaluate the clinical application of computed tomography-based measurement of renal cortical volume and split renal volume as a single tool to assess the anatomy and renal function in patients with renal tumors before and after partial nephrectomy, and to compare the findings with technetium-99m dimercaptosuccinic acid renal scan. The data of 51 patients with a unilateral renal tumor managed by partial nephrectomy were retrospectively analyzed. The renal cortical volume of tumor-bearing and contralateral kidneys was measured using ImageJ software. Split estimated glomerular filtration rate and split renal volume calculated using this renal cortical volume were compared with the split renal function measured with technetium-99m dimercaptosuccinic acid renal scan. A strong correlation between split renal function and split renal volume of the tumor-bearing kidney was observed before and after surgery (r = 0.89, P volumetry had a strong correlation with the split renal function measured using technetium-99m dimercaptosuccinic acid renal scan. Computed tomography-based split renal volume measurement before and after partial nephrectomy can be used as a single modality for anatomical and functional assessment of the tumor-bearing kidney. © 2017 The Japanese Urological Association.

Assessment of relative individual renal function based on DMSA uptake corrected for renal size

International Nuclear Information System (INIS)

Estorch, M.; Camacho, V.; Tembl, A.; Mena, I.; Hernandez, A.; Flotats, A.; Carrio, I.; Torres, G.; Prat, L.

2002-01-01

Decreased relative renal DMSA uptake can be a consequence of abnormal kidney size, associated with normal or impaired renal function. The quantification of relative renal function based on DMSA uptake in both kidneys is an established method for the assessment of individual renal function. Aim: To assess relative renal function by means of quantification of renal DMSA uptake corrected for kidney size. Results were compared with relative renal DMSA uptake without size correction, and were validated against the absolute renal DMSA uptake. Material and Methods: Four-hundred-forty-four consecutive patients (147 adults, mean age 14 years) underwent a DMSA study for several renal diseases. The relative renal function, based on the relative DMSA uptake uncorrected and corrected for renal size, and the absolute renal DMSA uptake were calculated. In order to relate the relative DMSA uptake uncorrected and corrected for renal size with the absolute DMSA uptake, subtraction of uncorrected (SU) and corrected (SC) relative uptake percentages of each pair of kidneys was obtained, and these values were correlated to the matched subtraction percentages of absolute uptake (SA). If the individual relative renal function is normal (45%-55%), the subtraction value is less or equal to 10%. Results: In 227 patients (51%) the relative renal DMSA uptake value was normal either uncorrected or corrected for renal size (A), and in 149 patients (34%) it was abnormal by both quantification methods (B). Seventy-seven patients (15%) had the relative renal DMSA uptake abnormal only by the uncorrected method (C). Subtraction value of absolute DMSA uptake percentages was not significantly different of subtraction value of relative DMSA uptake percentages corrected for renal size when relative uncorrected uptake was abnormal and corrected normal. where * p<0.0001, and p=NS. Conclusion: When uncorrected and corrected relative DMSA uptake are abnormal, the absolute uptake is also impaired, while when