Small fiber neuropathy in children, adolescents, and young adults with chronic orthostatic intolerance and postural orthostatic tachycardia syndrome: A retrospective study.

PURPOSE: To determine in children, adolescent and young adult (CAYA) patients presenting with Orthostatic Intolerance (OI) or Postural Orthostatic Tachycardia Syndrome (POTS) associated with the additional symptoms of neuropathic discomfort (pain, paresthesia and/or allodynia): 1) the incidence of small fiber neuropathy, and 2) assess if there was serologic evidence for an underlying inflammatory or autoimmune state. METHODS: A cohort of 109 CAYA patients with the above symptoms underwent epidermal skin biopsy for nerve fiber density. Blood biomarkers for inflammation were tested (CRP, ESR, ANA, complement (C3), thyroid function testing with antibodies (thyroid peroxidase antibody and thyroglobulin antibody), and cytokine panel 13). Patients completed a Quality of Health questionnaire. Statistical analysis was performed using Wilcoxon rank sum tests. RESULTS: In CAYA patients with OI or POTS and neuropathic symptoms, skin biopsy for small fiber neuropathy was abnormal in 53 %. The sample population was predominantly female and Caucasian with moderately decreased perceived quality of health. OI /POTS patients with small fiber neuropathy had a 3-fold probability of having a positive ANA or anti-thyroid antibody, suggesting an underlying autoimmune or inflammatory process. CONCLUSION: Our data suggest a link between OI and POTS and small fiber neuropathy. Small fiber neuropathy was found by skin biopsy in over half of the patients tested. OI and Postural orthostatic tachycardia patients with small fiber neuropathy expressed multiple markers suggesting an underlying autoimmune or inflammatory process. Future research will be done to evaluate the symptomatic implication of SFN and whether immune or pharmacologic manipulation can alter patient symptoms.

In-phase and mixed-phase measure synchronization of camphor rotors.

The numerical, analytical, and experimental analyses are presented for synchronizing two rotors under the Yukawa interaction. We report that the rotors exhibit in-phase and mixed-phase measure synchronizations for a pair of coupled rotors. Here, the analytical condition for synchronization is derived, tested numerically, and confirmed experimentally using coupled camphor infused rotors as a test bed. Moreover, the concept of measure synchronization is discussed. We report that, in conservative systems, not only the critical coupling parameter but initial conditions also play an essential role for estimating the measure synchronization region.

Generation of aperiodic motion due to sporadic collisions of camphor ribbons.

We present numerical and experimental results for the generation of aperiodic motion in coupled active rotators. The numerical analysis is presented for two point particles constrained to move on a unit circle under the Yukawa-like interaction. Simulations exhibit that the collision among the rotors results in chaotic motion of the rotating point particles. Furthermore, the numerical model predicts a route to chaotic motion. Subsequently, we explore the effect of separation between the rotors on their chaotic dynamics. The numerically calculated fraction of initial conditions which led to chaotic motion shed light on the observed effects. We reproduce a subset of the numerical observations with two self-propelled ribbons rotating at the air-water interface. A pinned camphor rotor moves at the interface due to the Marangoni forces generated by surface tension imbalance around it. The camphor layer present at the common water surface acts as chemical coupling between two ribbons. The separation distance of ribbons (L) determines the nature of coupled dynamics. Below a critical distance (L_{T}), rotors can potentially, by virtue of collisions, exhibit aperiodic oscillations characterized via a mixture of co- and counterrotating oscillations. These aperiodic dynamics qualitatively matched the chaotic motion observed in the numerical model.

The Challenges of Identifying Fibromyalgia in Adolescents.

Aim: Fibromyalgia (FM) is a noninflammatory disorder of the nervous system characterized by widespread musculoskeletal pain and somatic complaints of at least 3 months duration. There are no current diagnostic criteria for fibromyalgia in children to guide clinicians in recognition, thus leading to many subspecialty referrals and extensive imaging and tests. The purpose of this retrospective review is to compare two diagnostic criteria for juvenile fibromyalgia. Methods: A retrospective chart review of 20 children diagnosed with juvenile fibromyalgia from a singular pain physician practice was performed. Both the Yunus diagnostic criteria and the 2016 American College of Rheumatology (ACR) diagnostic criteria were applied and compared. Results: 85% of patients met criteria for fibromyalgia under both criteria. 15% of patients met only ACR criteria as the Yunus criteria excluded those with underlying conditions. Of the children who fulfilled criteria with use of both diagnostic tools, this cohort reported a high somatic symptom burden as demonstrated by the ACR symptom severity scales of 12 and satisfaction of at least 4 Yunus and Masi minor criteria on average. Widespread pain was noted with an ACR Widespread Pain Index (WPI) of 7, and tender points were 4.8 on average across the cohort. Effective therapeutic regimens among patients varied widely from medical monotherapy to multimodal treatment. Patients presented with pain for 1.8 yrs on average prior to a diagnosis. All of the cohort had a normal laboratory evaluation; half the cohort received additional imaging and testing. Conclusion: This case series suggests the need for an updated diagnostic tool for pediatric fibromyalgia to facilitate recognition and treatment.

Genetic and Phenotypic Heterogeneity of the Nocardiopsis alba Strains of Seawater.

This study deals with the genetic and phenotypic heterogeneity of the marine Nocardiopsis alba strains isolated during pre-monsoon, monsoon and post-monsoon seasons. The isolates were characterized for their morphological and biochemical attributes, growth media preferences, antibiotic susceptibility and extracellular enzyme secretion. Nocardiopsis alba strains were assessed against 12 different antibiotics, and the responses were expressed in terms of the multiple antibiotic resistance (MAR) number. The majority of the strains produced multiple extracellular enzymes: proteases, amylases and lipases. Further, the strains were characterized on the basis of 16S rRNA gene sequencing and the majority were identified as Nocardiopsis alba along with few strains of Streptomyces lopnurensis, Nocardiopsis synnemataformans and Nocardiopsis dassonvillei. Neighbor-joining (NJ) phylogenetic tree suggested variation among the genetically similar Nocardiopsis alba species. The study establishes significant heterogeneity with respect to genetic and phenotypic characteristics of the strains of Nocardiopsis alba. Phylogenetic tree and phenogram-based comparison reflect the heterogeneity in terms of different clustering patterns of the strains. Further, the whole genome sequence data available in the literature also confirm the observed heterogeneity. Nocardiopsis alba strains displayed a relatively regressive pattern of dependence on the environmental factors based on the canonical correspondence analysis plot. The study represents cultivation, characterization, phylogenetic analysis and enzymatic potential of the Nocardiopsis alba species of seawater origin.

Two steps purification, biochemical characterization, thermodynamics and structure elucidation of thermostable alkaline serine protease from Nocardiopsis alba strain OM-5.

The Nocardiopsis alba strain OM-5 showed maximum protease production in submerged culture. The OM-5 protease was purified by hydrophobic interaction chromatography. The purified protease of 68 kDa showed maximum activity (3312 ± 1.64 U/mL) at 70 °C and was quite stable at 80 °C up to 4 M NaCl (w/v) at pH 9. The purified protease showed significant activity and stability in different cations, denaturing agents, metal ions, and osmolytes. The thermodynamic parameters including deactivation rate constant (Kd) and half lives (t1/2) at 50-80 °C were in the range of 2.50 × 10-3 to 5.50 × 10-3 and 277.25-111.25 min respectively at 0-4 M NaCl. The structural stability of the OM-5 protease under various harsh conditions was elucidated by circular dichroism (CD) spectroscopy followed by K2D3 analysis revealed that the native structure of OM-5 protease was stable even in sodium dodecyl sulfate and Tween 20 indicated by increased α-helices content assisted with decreased ß-sheets content.

Anti-Mullerian Hormone Levels in Indian Women Seeking Infertility Treatment: Are Indian Women Facing Early Ovarian Senescence?

BACKGROUND: Antimullerian hormone (AMH) is a key marker of ovarian reserve and predictor of response to fertility treatment. AIM: To understand the prevalence of low ovarian reserve in Indian women seeking infertility treatment, compare their AMH with age-matched fertile Indian controls and understand ethnic differences with Caucasian women. SETTING AND DESIGN: Retrospective observational study done as collaboration between our in vitro fertilization centre and a laboratory with Pan-India presence. MATERIALS AND METHODS: Women aged 20-44 years were selected as Group A (seeking infertility treatment n = 54,473), Group B (conceived naturally in the past; n = 283) and Group C (data of Caucasian women; n = 718). Serum AMH levels were measured and descriptive analysis done. STATISTICAL ANALYSIS: Descriptive statistics and Chi-square test. RESULTS: In Group A, 28.7%, 48.7% and 70.6% of women aged <30 years, 30-34 years and 35-39 years had serum AMH levels ≤2 ng/mL and the proportions were higher than Group B. The rate at which median AMH decreased was 1.1-2 times faster in Group B as compared to Group C. The decrease in median AMH across age groups in Group A was similar to Group B. CONCLUSIONS: Indian women in their late twenties and early thirties visiting fertility centers showed a worrisome trend of low AMH. Our study can be used as a reference for those women considering postponing pregnancy. It may be time to look at intangible cultural factors linked to social habits, ethnicity, diet, genetic predispositions, and environmental factors like endocrine disrupting chemicals contributing to premature ovarian senescence.

Slow speed resistance exercise training in children with polyarticular juvenile idiopathic arthritis.

Background: Juvenile idiopathic arthritis (JIA) is an inflammatory autoimmune disease that can cause severe impairment and disability. Exercise is recommended to preserve joint mobility and function. Our objectives were to assess the safety, feasibility, and effects of slow speed resistance exercise in children with polyarticular JIA. Methods: Patients were recruited from a pediatric rheumatology clinic at an urban hospital and randomized to exercise or control groups. In the intervention group, slow speed resistance exercise with individualized instruction by a certified trainer was performed 1-2 times per week for 12 weeks. The control group performed home-based aerobic exercise 3 days per week for 12 weeks. Pre and post-body composition measurements by dual-energy X-ray absorptiometry; aerobic fitness by peak oxygen uptake during cycle ergometry; isometric muscle strength; and quality of life measures were obtained. Results: In the exercise group, 9/17 (53%) completed any exercise training. Of these nine subjects, five (55%) completed all 12 weeks of the protocol. In the control group, 8/16 (50%) reported compliance with the recommended aerobic exercise training at least one time per week. Only 2 subjects (12%) reported exercising more than once per week. There was no significant difference between pre- and post-measurements in any category in the exercise group. There was also significantly elevated body fat in both groups with only 17% in the control group and 23% in the exercise group meeting recommended <30% total body fat levels. Conclusions: Children with JIA participated safely in this resistance exercise protocol. The exercise was well-tolerated with no serious adverse events noted. While individual subjects reported improvement in fatigue and improved energy, there was no statistical difference in pre- and post-exercise measures of body composition or quality of life. Identifying ways to improve adherence and encourage exercise in children with JIA is important.

Increased household exposure to respiratory pathogens in HIV exposed uninfected children due to maternal HIV.

Little is known about household exposures to respiratory pathogens in HIV-exposed uninfected children (HEU) in Indian families. This case series investigates the nasopharyngeal carriage of Streptococcus pneumoniae, Staphylococcus aureus, and respiratory viruses at multiple points in three mother child pairs: (1) an HIV-infected child and mother, (2) an HEU child and HIV-infected mother, and (3) an HIV-unexposed uninfected (HUU) child and mother. Nasopharyngeal carriage densities of Streptococcus pneumoniae and Staphylococcus aureus were higher in mothers and children living in HIV-affected households, regardless of the child's HIV status. Maternal HIV and ART status impact these household exposures.

Phylogeny, novel bacterial lineage and enzymatic potential of haloalkaliphilic bacteria from the saline coastal desert of Little Rann of Kutch, Gujarat, India.

This report describes cultivation-dependent diversity, phylogeny and enzymatic potential of the haloalkaliphilic bacteria isolated from the unvegetated desert soil of yet unexplored, saline desert of Little Rann of Kutch (LRK), India. The LRK is a unique ecosystem displaying a combination of Dry Rann and Wet Rann. A total of 25 bacteria were isolated and characterized on the basis of colony morphology, biochemical profile, sugar utilization, secretion of the extracellular enzymes and antibiotic sensitivity. Further, the identification and phylogenetic relatedness of 23 bacteria were established by the analysis of 16S rRNA gene sequences. The phylogenetic analysis indicated that the isolates belong to the phylum Firmicutes, comprising low G + C, Gram-positive bacteria, with different genera: Bacillus (~ 39%), Staphylococcus (~ 30%), Halobacillus (~ 13%), Virgibacillus (~ 13%), Oceanobacillus (~ 4%). Majority of the bacterial isolates produced proteases (30% isolates) followed by cellulases (24% isolates), CMCases (24% isolates) and amylases (20% isolates). Halobacillus, Virgibacillus and Bacillus predominantly produced hydrolases, while many produced multiple enzymes at high salinity and alkaline pH. Highest antibiotic resistance was observed against Ampicillin and Penicillin (32%) followed by Cefaclor (20%); Colistin, Cefoperazone and Cefotaxime (16%); Cefuroxime (12%); Gentamycin and Cefixime (8%); Erythromycin, Cefadroxil, Azithromycin, Co-trimoxazole, Amoxycillin, Norfloxacin, Cefpodoxime, Amikacin and Augmentin (4%). KJ1-10-99 and KJ1-10-93 representing < 97% of 16S rRNA gene sequence similarity belong to a novel lineage within the family Bacillaceae. Comparison of the phenogram and phylogram revealed the contradiction of the phenogram pattern and the phylogenetic placement of the isolates. The isolates belonging to same species have shown considerable phenotypic variation. The study on the cultivable haloalkaliphilic bacteria of an unexplored enigmatic niche reflects ecological and biotechnological significance.

Approach to Membranous Lupus Nephritis: A Survey of Pediatric Nephrologists and Pediatric Rheumatologists.

OBJECTIVE: To describe treatment practices for childhood pure membranous lupus nephritis (MLN). METHODS: Survey study of Childhood Arthritis and Rheumatology Research Alliance and American Society of Pediatric Nephrology members. RESULTS: There were 117 respondents who completed the survey (60 pediatric nephrologists, 57 pediatric rheumatologists). Steroids and nonsteroid immunosuppression (NSI) were routinely used by the majority for MLN. Mycophenolate mofetil was the favored initial NSI. Nephrologists used steroids (60% vs 93%) and NSI (53% vs 87%) less often than did rheumatologists for MLN without nephrotic syndrome (NS). CONCLUSION: Pediatric rheumatologists and nephrologists both recommend steroids and NSI for children with MLN, with or without NS.

Successful Thrombolysis of a Large Pulmonary Artery Thrombosis.

A 32 yrs old man presented with shortness of breath and syncopal episode with preceding history of DVT 15days above. Patient has tachycardia hypoxia and hypotension, on evaluation ECG Showed S1 Q3 T3 Pattern, bedside Echo Showed visible thrombus of 3cm in pulmonary artery, successfully thrombolysed with tenecteplase and streptokinase. This case study is presented to stress importance of urgent bedside echo in all sudden onset dysponea and hypoxia to rule out pulmonary Embolism which can be successfully thrombolysed without delay.

Purification strategies, characteristics and thermodynamic analysis of a highly thermostable alkaline protease from a salt-tolerant alkaliphilic actinomycete, Nocardiopsis alba OK-5.

An alkaline protease from salt tolerant alkaliphilic actinomycetes, Nocardiopsis alba strain OK-5 was purified to homogeneity by 27 and 13 fold with a yield of 35 and 13% using two-steps and one-step method, respectively. The purification methods involved hydrophobic interaction on phenyl sapharose matrix. The apparent molecular mass was 20 kDa. The temperature optimum shifted from 70 to 80°C in 4M NaCl and 30% Na-glutamate, with significant stability at 60-80°C in Na-glutamate. Deactivation rate constant (K(d)) increased and half life (t(1/2)) decreased with the increasing temperatures from 37 to 80°C. The order of stability was: 30% Na-glutamate>4M NaCl>2M NaCl>0M NaCl. The enzyme was stable even at 80°C in 30% Na-glutamate with K(d) 4.11 and t(1/2) 168.64 min. The activation energies (E), enthalpy (ΔH*) and entropy (ΔS*) for protease deactivation in with Na-glutamate were 31.97 kJ/mole, 29.23 kJ/mole and -211.83 J/mole, respectively. The change in free energy (ΔG*) for protease deactivation at 60°C in 30% Na-glutamate was 101.70 kJ/mole. Protease had the highest activity and stability at pH 10-11. While the enzyme was highly resistant against chemical denaturation, it had varied responses to metal ions. Complete inhibition by PMSF confirmed serine nature of the protease. Na-glutamate, H(2)O(2), ß-mercaptoethanol and different surfactants enhanced the activity.

Clinico-microbiological study of opportunistic infection in HIV seropositive patients.

OBJECTIVES: The present study was undertaken to determine the prevalence of respiratory, gastrointestinal and other pathogens in 100 Human Immunodeficiency Virus (HIV) seropositive patients. SETTINGS: This study was carried out on randomly selected 100 HIV seropositive patients from S. S. G. Hospital during the period from Jan 2006 to Jan 2007. MATERIALS AND METHODS: Sputum samples, stool samples and oral swabs were collected from all the patients and cerebrospinal fluid (CSF) was collected from symptomatic patients and processed as per the standard protocol. Sputum samples were examined by microscopy and cultured for bacterial respiratory pathogens. Stool samples were concentrated and examined by microscopy for enteric parasites. Oral swabs and CSF were also examined microscopically and cultured for fungal pathogens. CSF was also examined for bacterial pathogens. RESULTS: A total of 101 pathogens were detected in 60 patients. More than one pathogen was observed in 30 patients. Candida was the commonest isolate (32.67%), followed by Mycobacterium tuberculosis (22.71%) and Cryptosporidium parvum (19.8%). CONCLUSIONS: Since opportunistic infections are a major cause of mortality and morbidity in HIV seropositive patients, an early diagnosis and effective treatment are required to tackle them. The type of pathogens infecting HIV patients varies from region to region, and therefore such patients should be constantly screened for these pathogens.

Persistent low albumin and temporary vascular access in pediatric patients with SLE on hemodialysis.

Pediatric patients with systemic lupus erythematosus (SLE) often present with significant kidney disease. In a previous cross-sectional analysis, we showed that pediatric patients with ESRD secondary to SLE have lower serum albumin levels and less permanent vascular access for hemodialysis (HD) compared to pediatric patients on HD secondary to other causes. The goal of this longitudinal study was to determine if there was an improvement in these targets over time. To this end, we performed a longitudinal analysis of patients receiving HD in the ESRD Clinical Performance Measures Project 2000-2004 study years, comparing achievement of clinical targets between pediatric patients with SLE and pediatric patients with other causes of ESRD. In the longitudinal follow-up, pediatric patients with SLE were less likely to reach target albumin levels than other children with ESRD maintained on HD [odds ratio (OR) 0.18, 95% confidence interval (CI) 0.09, 0.35] and were less likely to have arteriovenous fistulas or grafts than other pediatric patients (OR 0.45, 95% CI 0.23, 0.89). Pediatric patients with SLE maintained on HD are at particularly high risk for failing to meet some clinical targets that have been associated with improved long-term outcomes in other populations. This is true even as they remain on dialysis over time.

Reduced albumin levels and utilization of arteriovenous access in pediatric patients with systemic lupus erythematosus (SLE).

Systemic lupus erythematosus (SLE) is an autoimmune disease that affects between five and ten thousand children in the USA. Kidney disease may progress to end-stage renal disease (ESRD) and subsequent need for dialysis therapy in a significant number of children with SLE. We performed a cross-sectional analysis comparing achievement of National Kidney Foundation/Kidney Disease Outcomes Quality Initiative clinical targets in pediatric patients with SLE maintained on hemodialysis (HD) to pediatric patients with other causes of ESRD. Ninety-seven unique SLE patients and two control groups-1,823 unique pediatric patients with other causes of ESRD and 694 pediatric patients with glomerulonephritis-were identified in the End Stage Renal Disease Clinical Performance Measures 2000-2004 Project Years. SLE patients were older, with a female and black race predominance compared with both control groups. Pediatric patients maintained on HD secondary to SLE were less likely to meet albumin targets and more likely to have vascular catheters than were pediatric patients on HD secondary to other causes. These findings may be associated with increased morbidity and mortality in pediatric patients with SLE maintained on HD and deserve further study.

Preoperative autologous blood donation by arthritis patients is associated with preoperative anemia and perioperative transfusion.

BACKGROUND: Preoperative autologous blood donation (PAD) became a common practice in the 1990s in an attempt to reduce the risk of transmission of infectious agents from allogeneic blood. However, a potential risk of PAD is the development of preoperative anemia that may lead, in turn, to an enhanced need for transfusion. Furthermore, the ready availability of autologous blood may predispose to more liberal transfusion peri-/postoperatively. OBJECTIVES: To examine these hypotheses, we retrospectively examined a cohort of knee and hip arthroplasty patients. METHODS: Charts of patients of 2 orthopedic surgeons from the mid 1980s and 1990s were reviewed for transfusions needed and hematocrits before and after arthroplasties. RESULTS: PAD proved to be a significant risk factor for the development of preoperative anemia and for peri-/postoperative blood transfusion even after adjusting for confounders. CONCLUSIONS: These results suggest that PAD may lead to a self-defeating cycle of blood donation followed by blood transfusion. With the improved safety of the allogeneic blood supply, rheumatologists may want to play a more active role in considering PAD in patients in whom elective arthroplasty is planned.

Laparoscopic tubal sterilization after two or more cesarean sections.

STUDY OBJECTIVE: To determine the safety of laparoscopic tubal sterilization in women who have had two or more cesarean sections. DESIGN: Retrospective study (Canadian Task Force classification III). SETTING: Private clinic and hospitals. PATIENTS: Two hundred ten consecutive women. INTERVENTION: Laparoscopic tubal sterilization. MEASUREMENTS AND MAIN RESULTS: The procedures were done with no difficulty in all but two women. In addition to the two failures, one woman experienced bladder trauma. CONCLUSION: Laparoscopic sterilization after several cesarean sections is possible and is associated with low morbidity. Scarring from cesarean sections should not be a contraindication if extra care is taken.