Juvenile Type Research Articles

Differences in the pattern of cognitive impairments between juvenile and adult onset myotonic dystrophy type 1

ObjectiveTo understand the different patterns of neuropsychological dysfunction observed between juvenile onset (jDM1) and adult onset (aDM1) myotonic dystrophy type 1. MethodWe enrolled 19 genetically confirmed DM1 patients, and neuropsychological tests—Wechsler Adult Intelligence Scale-Revised short form; Rey–Kim memory test; and Executive Intelligence Test for evaluating intelligence, memory, and executive function—were performed. Clinical parameters including cytosine-thymine-guanine (CTG) repeats, creatinine kinase level, pulmonary function test, six-minute walk test, motor scales, and cardiac function were evaluated. ResultsVerbal intelligence was significantly lower in the jDM1 than the aDM1 group (7.50 ± 1.64 vs. 11.00 ± 2.54, respectively; p = 0.009), while no difference was observed in performance intelligence. There was significant differences between the two groups (p = 0.022) with respect to memory function, as specifically revealed by the pattern of lower function in the verbal memory of the jDM1 group. However, the executive function test showed no intergroup differences. ConclusionVerbal memory impairment significantly deteriorated in the jDM1 group as compared to the aDM1 group, reflecting a more profound neurodevelopmental change in the juvenile type.

Hemobilia Induced by Gall Bladder Polyps

Hemobilia is an aberrant connection of hepatic biliary and vascular systems. It usually occurs post traumatic, blunt trauma or iatrogenic. Non traumatic hemobilia is a rare occurrence which there are few case reports in children which were due to pancreatic pseudocyst, choledochal cyst, hepatic abscess, hepatic fascioliasis and Meta chromatic leukodystrophy. All reported cases are female except one with von Willebrand’s disease. In few patients Meta chromatic leukodystrophy (MLD) was the cause of hemobilia. MLD is a rare neurological disease which is caused by Sulfatids deposition in nervous tissue and it has three subtypes, late infantile, juvenile and adult type. The gall bladder may be involved in the disease course and presents with different symptoms like abdominal pain or hemobilia. We present an eight-year-old girl with late infantile MLD who presented with upper GI bleeding and in evaluation the diagnosis of hemobilia and gall bladder polyps was confirmed.

The caldera-forming eruption of the quaternary Payún Matrú volcano, Andean back-arc of the southern volcanic zone

The Payún Matrú caldera is a Quaternary Andean back-arc caldera (36° 25′ S, 69° 12′ 30” W), and represents the main volcanic edifice of the homonym Payún Matrú volcanic field, located within the Payenia Basaltic province. The Payún Matrú volcanic field is also characterized by the Payún Liso volcano, and 220 monogenetic basaltic cones and associated lava flows. The Pleistocene – Holocene Payún Matrú caldera presents a long lived pre-caldera stage, which built a multi-vent and shield-like volcanic edifice, mainly trachytic in composition. The syn-caldera stage is represented by the trachytic Portezuelo Ignimbrite (the caldera-forming eruption deposits) constrained between 148 and 82 ka, and the resulting caldera depression of 8.5 km in diameter. Afterwards the caldera event, volcanism continued mostly along the caldera rim.Stratigraphic sections of the Portezuelo Ignimbrite and its correlations are presented, in addition to mineral compositions and previous whole-rock chemical analyses. Only the extra-caldera deposits can be observed, while the intra-caldera ignimbrite is hidden by post-caldera volcanic products and unconsolidated modern sediments. Different facies within the ignimbrite were recognized, being most of them massive or eutaxitic and massive lapilli tuff facies, deposited by dense pyroclastic density currents. Fall deposits are found only proximally and restrained to the caldera margin. The absence of a widespread fall deposit suggests the lack of a sustained eruptive column at the onset of eruption. Massive tuff facies at the topmost deposits indicates the development of a co-ignimbritic plume as the pyroclastic density currents wanned.On the basis of color, size and shape, vesicularity and crystallinity, four distinct juvenile clasts types were defined: gray fiamme, black juvenile, dark gray and light gray pumice. Available chemical analysis of gray fiamme and black juvenile clasts indicate a similar trachytic composition, although their mineralogical composition presents some differences.Several eruptive units for the northern and southern outer slopes of the caldera were defined, on the basis of the juvenile types present and discontinuity surfaces. The onset of caldera collapse is not marked by the presence of a lithic breccia, but it is suggested by the appearance of dark gray and light gray pumice along with black juveniles in the topmost eruptive units, together with an increase in lithic content relative to previous units. The sequential appearance of different juvenile clasts types suggests a zoned magma chamber, in terms of crystal content and composition.

Using the PAI-A to Classify Juvenile Offenders by Adjudicated Offenses.

To improve understanding and treatment of criminal behavior, researchers have developed typologies of juvenile offenders, primarily focusing on personality traits and criminal history to classify according to type of offense committed. Existing literature has examined underlying personality characteristics found in different subcategories of criminal offenses in juveniles; however, few studies have employed the Personality Assessment Inventory-Adolescent (PAI-A), instead choosing the MMPI-A. A typical classification model of juvenile offenses categorizes offenses into: Interpersonal, Property, and Drug/Alcohol-related charges, to further study within-group differences. The current study examines how personality profiles, examined by the PAI-A, can classify offenders into these offense-type groups. Personality profiles of participants were obtained through pre-sentencing psychological evaluations of 142 juvenile offenders ages 14 to 17. Binary logistic regressions were conducted using PAI-A Clinical, Treatment Consideration, and Interpersonal scales to predict offense-type group classifications. Results yielded statistically significant full models for all offense-type groups, with an average overall accuracy rate of 76.3%. Overall, results suggest that the PAI-A has good predictive power to classify juvenile offender types, and may be more effective in classifying certain types of offenders than the MMPI-A. Notably, Interpersonal and Treatment consideration scales were stronger predictors of offense-type than Clinical scales. This model of juvenile offender classification holds promise for more effective treatment, management, and prediction of behavior for juvenile offenders.

Characterization Of Exercise Blood Pressure Responses In Adolescents With A Chronic Inflammatory Condition

Exaggerated blood pressure (BP) responses to exercise are prognostic of cardiovascular disease (CVD) in adults. There are no criteria to define exaggerated BP responses in children, yet higher exercise BPs have been shown to be associated with CVD risk factors. Therefore, exercise BPs may provide important prognostic information in children, especially those at higher risk for CVD. PURPOSE: The study sought to characterize the exercise BP responses in an “at-risk” sample of adolescents with a chronic inflammatory condition. METHODS: Twenty-one adolescents (7 males; 14.7 ± 1.8 years) with a confirmed single diagnosis of chronic kidney disease, cystic fibrosis, inflammatory bowel disease, juvenile idiopathic arthritis or type 1 diabetes mellitus participated in this study. Brachial BP was assessed using a motion-tolerant automated auscultatory device every 2 minutes during the McMaster All-Out Progressive Continuous cycling test, using pediatric-specific criteria for test termination at “maximal” effort. Maximal BPs were compared to (1) sex-specific reference values for healthy adolescents, and (2) exaggerated BP criteria for adults. RESULTS: Maximal systolic BPs for males and females were 184 ± 35 and 172 ± 19 mmHg, respectively. Maximal diastolic BPs for males and females were 71 ± 11 and 74 ± 9 mmHg, respectively. Two males (29%) had a maximal BP above reference values with one having a higher systolic BP and the other having a higher diastolic BP. Nine females (64%) had a maximal BP above reference values with five exceeding the systolic BP reference, one exceeding the diastolic BP reference, and three exceeding both BP references. An exaggerated BP response occurred in two males and two females. CONCLUSION: Approximately half of our sample of adolescents with a chronic inflammatory condition reached higher exercise BPs compared to healthy adolescent reference values. Elevated exercise BPs were also more common in females compared to males, and 19% of our sample met the adult-criteria for exaggerated BP responses. Collectively our observations of elevated exercise BPs in an “at-risk” population highlight the importance of examining BP responses in children, including research on its potential prognostic value. Funding: Canadian Institutes of Health; Heart and Stroke Foundation

Adult-type granulosa cell tumor of the testicle: case report.

Granulosa cell tumor (GCT) is a sex-cord neoplasm of the gonads classified into either juvenile (jGrCT) or adult type (aGrCT). It is commonly arising in ovaries but is much rarer in men, with only around 50 male cases previously reported in the literature. We report on a 54-year-old male patient with a right testicular GCT measuring 10.0×8.0×6.0 cm. The tumor was treated successfully with radical orchiectomy followed by computed tomography to assess lymph node involvement, and no further treatment was done. Pathological reports showed diffuse positivity for immunohistochemical stains, inhibin, vimentin, calretinin, and CD99. The clinical and histopathological features, treatment, and prognosis of aGrCT arising in the testicle of an adult male are also reviewed in this manuscript.

Autoimmune Polyendocrinopathy.

This mini-review offers an update on the rare autoimmune polyendocrinopathy (AP) syndrome with a synopsis of recent developments. Systematic search for studies related to pathogenesis, immunogenetics, screening, diagnosis, clinical spectrum, and epidemiology of AP. AP (orphan code ORPHA 282196) is defined as the autoimmune-induced failure of at least two glands. AP is divided into the rare juvenile type I and the adult types II to IV. The prevalence is 1:100,000 and 1:20,000 for types I and types II to IV, respectively. Whereas type I (ORPHA 3453) is a monogenetic syndrome with an autosomal recessive transmission related to mutations in the autoimmune regulator (AIRE) gene, types II to IV are genetically complex multifactorial syndromes that are strongly associated with certain alleles of HLA genes within the major histocompatibility complex located on chromosome 6, as well as the cytotoxic T lymphocyte antigen 4 and the protein tyrosine phosphatase nonreceptor type 22 genes. Addison disease is the major endocrine component of type II (ORPHA 3143), whereas the coexistence of type 1 diabetes and autoimmune thyroid disease is characteristic for type III (ORPHA 227982). Genetic screening for the AIRE gene is useful in patients with suspected type I, whereas serological screening (i.e., diabetes/adrenal antibodies) is required in patients with monoglandular autoimmunity and suspected AP. If positive, functional endocrine testing of the antibody-positive patients as well as serological screening of their first-degree relatives is recommended. Timely diagnosis, genetic counseling, and optimal long-term management of AP is best offered in specialized centers.

OR26-3 ERK1/2-RSK2 Maintains Estrogen Homeostasis in the Adult Mammary Gland

In response to estrogens, estrogen receptor alpha (ERα) is degraded by the 26S proteasome; however, despite the continued presence of estrogen prior to menopause, ERα protein levels are maintained by an unidentified mechanism. We find that the Ser/Thr kinase, RSK2, negatively regulates ERK1/2-dependent ERα degradation in the mammary gland to maintain ERα protein levels in cycling adult female mice. In RSK2 knockout (RSK2-KO) mice, ERα protein levels are decreased ~70% in the adult mammary gland compared to the control. Mammary gland regeneration experiments demonstrated that regulation of ERα protein levels by RSK2 is intrinsic to the mammary epithelium. In juvenile animals the ERK1/2-RSK2 pathway is not active and consistent with that observation the ERα protein levels are similar in juvenile wild type and RSK2-KO mice. In adults, the levels of active ERK1/2 increase ~25-fold relative to juvenile mammary glands. Activation of ERK1/2 in the adult is restricted to the estrus phase of the estrous cycle and activation of ERK1/2 is estrogen-dependent. Oophorectomy or inhibition of the 26S proteasome restored ERα protein levels in RSK2-KO mice. Loss of RSK2 results in activation of ERK1/2 throughout the estrous cycle and results in an enrichment for estrogen-responsive genes in the RSK2-KO ER+ mammary epithelial cells relative to wild type. These data support a model in which the unrestricted activation of ERK1/2 in RSK2-KO mice drives inappropriate transcription-coupled proteolysis of ERα which leads to an increase in DNA-double stranded breaks. Bioinformatic analysis demonstrated a correlation between decreased RSK2 expression and increased expression of estrogen-regulated gene in normal breast tissue from women taking oral contraceptives. Furthermore, the RSK2-KO transcriptome signature identified a subset of ER+ breast cancer patients with low RSK2 expression. These findings establish RSK2 as a regulator of estrogen homeostasis in the pre-menopausal adult and suggest a possible mechanism for the increased cancer risk in women taking exogenous estrogens. Funding source: NIH

Theory of Bowstring Disease: Diagnosis and Treatment Bowstring Disease.

Bowstring disease (BSD) is a new classification of spine disease caused by axial stretched lesion on nerve roots and the spinal cord, which is differentiated from disc herniation and canal stenosis in that it is caused by nerve compression lesions. BSD could be caused by mismatched growth rates between the spine and nerve roots (the juvenile type), or by imbalanced degenerative rates between the spine column and nerve roots (degenerative type). Here, we propose that there are several self‐adjust mechanisms to relieve axial nerve tension: (i) nerve growth; (ii) posture adjustment and low back pain; (iii) autogenous degeneration of intervertebral disc; and (iv) idiopathic and degenerative scoliosis. Iatrogenic lesions could also result in BSD, which could be presented as adjacent segment degeneration, leading to adding‐on effects and other neurological symptoms. The diagnosis criteria are proposed based on symptoms, physical examination, and radiological presentations. To remove axial tension on nerve roots, lumbar surgery should aim to restore the coordination of spine and cord units. Capsule surgery, shortening the spine column, could decompress cord and nerve roots 3‐dimensionally.

Sex cord-stromal tumors of the ovary: granulosa-stromal cell tumors. Case report and literature review

Background. Sex cord-stromal tumors of the ovary (SCSTO) are a rare pathology of the ovary, representing less than 2% from the total primary ovarian cancer cases. Materials and method. This paper describes a case of granulosa tumor cell, juvenile type, in a young woman with secondary amenorrhea and primary infertility. Results. Granulosa tumor cell, juvenile type, was suspicioned after clinical and paraclinical tests had been done, and MRI de­scribed the ovarian tumor. This case emphasizes the importance of multidisciplinary team approach, involving gynecologist, fertility specialist, oncologist, and radiologist. We have reviewed the available literature in this report. Although most cases are treated with surgery alone fol­lowing oncological safety principles as in epithelial ovarian cancer approach, it is important to discuss the follow-up ap­proach, taking into consideration that this patient par­ti­cularly wished to procreate, and fertility sparing surgery was performed. Chemotherapy and radiotherapy were com­plementary treatments that were discussed in the multidisciplinary meeting, but as no adjuvant therapy was found to be effective, these adjuvant therapies are kept for recurrence and palliative care for advanced disease. Close follow-up was arranged for the patient as clinical and pelvic/abdominal MRI surveillance. Conclusions. Given the rarity of this disease worldwide, it is important to raise awareness amongst medical staff and educate the general population to seek medical attention early.

Juvenile Onset Niemann-Pick Type C Disease with Refractory Seizures.

Juvenile Onset Niemann-Pick Type C Disease with Refractory Seizures.

Atypical juvenile type of the Devergie disease

Atypical juvenile type of the Devergie disease

Juvenile Type Granulosa Cell Tumor

Abstract Granulosa cell tumor is a type of neoplasm, which represents 2-5% of all ovarian cancers. About 5% of these tumors are juvenile- type and usually occur to girls before puberty and to women younger than thirty years of age. There are signs premature puberty or premature emergence of secondary sexual characteristics with irregular vaginal bleeding that occur to these kind of patients. To the rare cases, like this, the occurrence of granulosa cell tumors can cause the appearance of hyperandrogenism with high levels of plasma testosterone, leading to virilization which happened to this female patient. We will present the female patient who was 35 years old and which was originally hospitalized to the Clinic for Haematology Clinical Center Kragujevac, because of extreme fatigue accompanied by dizziness. During diagnostics the patient underwent to the complete gynecological examination. After gynecological examinations and necessary diagnostic procedures, it was decided continuing the treatment at the Clinic of Gynecology and Obstetrics Clinical Center Kragujevac, where she underwent a total abdominal hysterectomy with bilateral salpingo- oophorectomy for suspected uterine neoplasm. Histopathological analysis of the obtained material confirmed the presence of follicular cysts of both ovaries and juvenile type granulosa cell tumor on the right ovary; the uterus was enlarged with multiple fibroid tumors. Granulosa cell tumor should be suspected in the cases of girls and young females if there is present an ovarian cyst paired with signs of preterm puberty or hyperestrogenism. In this case, the presence of granulosa cell tumor was masked by signs of hyperandrogenism, which is not so typical, as well as the presence of uterine fibroids who have actually been the main cause for surgical treatment.

Clinical and molecular characteristics of 11 Chinese probands with GM1 gangliosidosis.

GM1 gangliosidosis is an autosomal recessive lysosomal storage disease caused by the deficiency of β-galactosidase activity, precisely due to mutations in the GLB1 gene. To explore the clinical and molecular characteristics of GM1 gangliosidosis patients from China, GLB1 gene were analyzed in 11 probands with GM1 gangliosidosis by exploiting direct Sanger-sequencing. Among them, five patients were classified as the infantile type and the remaining six as the late-infantile or juvenile type. In these probands, eight novel mutations p.Y50N, p.Y237C, p.S267F, p.G453R, p.K578N, c.618delC, c.475_478delGACA and c.1979_1980insG have been identified. Among them, three novel missense mutations p.Y50N, p.S267F and p.G453R were transiently transfected in COS-7 cells by plasmid system for functional verification. In vitro GLB1 activities carrying the aforesaid missense mutants p.Y50N, p.S267F and p.G453R were 0.11%, 0 and 0.55% of wild-type, respectively. Mutation c.495_497delTCT and p.S149F accounted for 22.7 and 13.6% of the mutant alleles, respectively. Our results expand the spectrum of GLB1 gene, provide new insights into the clinical and molecular characteristics of GM1 gangliosidosis in China.

CONGENITAL MYOPATHIES: NEMALINE AND TITINOPATHIES: P.231The clinical, genetic, and pathological findings in a Chinese cohort of patients with hereditary nemaline myopathy

We aimed to identify the causative mutations of hereditary nemaline myopathy and to investigate the clinical, and pathological features in various genotypes. Methods: We recruited 48 patients with hereditary nemaline myopathy who had been diagnosed by muscle biopsies in our neuromuscular pathology lab from 2007 to 2017. Their clinical data and myopathological findings were retrospectively collected and analyzed. Next generation sequencing was used to identify the causative gene mutations in 40 patients. The onset age of these 48 patients ranged from 0 to 60 years old and the disease course ranged from 2 months to 38 years. Clinically, 23, 14 and 11 patients were typical congenital type, childhood or juvenile onset type and adult onset type, respectively. Molecular genetic analysis in 40 patients revealed possible pathogenic variants in 32 patients (80.00%), including 8 known mutation and 42 novel variants. NEB gene was the most frequent (23 patients, 57.50%), followed by ACTA1 (4 patients, 10.00%), RYR1 (2 patients, 5.00%), KBTBD13 genes (1 patient, 2.50%), TNNT1 (1 patient, 2.50%), and MYO18B (1 patient, 2.50%). Pathologically, thirteen patients showed coexistence of other myopathological features in addition to the presence of nemaline bodies in the muscle fibers, i.e., minicores in 4, cores in 3, central nuclei in 3 and fiber type disproportion in 3 patients. Nemaline bodies were present subsarcolemmally or in the cytoplasm of muscle fibers in patients with NEB mutation. In patients with ACTA1 mutation, nemaline bodies appeared in cluster or as thin filament in the region of disrupted myofibril. In patients with RYR1 mutation, there were rod-like structures formed by distorted Z-discs in the region of disrupted myofibrils. Hereditary nemaline myopathy showed wide clinical and genetic spectrum. Myopathological changes also revealed some difference depending on different genotypes. NEB is the most frequent causative gene in our patient cohort.

Clinical characteristics and prognosis of children with ventricular noncompaction

Objective To investigate the clinical features and prognosis in ventricular noncompaction children. Methods Thirty-four cases who diagnosed with ventricular noncompaction were included in this study in Shengjing Hospital of China Medical University from January 2012 to May 2018.According to age, the children were divided into infantile type(age <1 year old) and juvenile type(age ≥1 year old). We analyzed the clinical features, laboratory tests and prognosis. Results The average age at diagnosis was 3 years and 2 months.The sex ratio was 2.4∶1.Of these, 32 cases were left ventricular noncompaction, 1 was right ventricular noncompaction, and 1 was biventricular noncompaction.There were no statistically significant differences in family history, arrhythmia, and thrombotic events between infantile type and juvenile type.Heart failure was the first reason for most children, while older children often presented with fatigue when at diagnosis.More than half of them showed significant left ventricular ejection fraction(LVEF) decreasing, and non-compacted layer to compacted layer(N/C) ratio showed negative correlation with LVEF(r=-0.74, P<0.001). Sixty-five percent(22/34) of the patients presented abnormal electrocardiogram.During the follow-up, one patient died of pulmonary embolism.No significant LVEF improvement was found in 35%(12/34) of the patient.The COX proportional hazards model showed that N/C ratio was an independent risk factor for poor prognosis of ventricular noncompaction(OR=14.46, 95%CI 1.712-120.234, P<0.05). Conclusion Children with ventricular noncompaction showed different clinical features and prognosis.Early diagnosis, treatment and long term follow up are key issues for the prognosis. Key words: Ventricular noncompaction; Heart failure; Prognosis

Identification of Novel ARSA Mutations in Chinese Patients with Metachromatic Leukodystrophy.

Objective Metachromatic leukodystrophy (MLD) is an inherited disease caused by a deficiency of the enzyme arylsulfatase A (ARSA) that leads to severe physiologic and developmental problems. Our study is aimed at elucidating the clinical and genetic characteristics of Chinese MLD patients. Methods Clinical data of 21 MLD patients was collected. All coding exons of ARSA and their flanking intronic sequences were amplified by polymerase chain reaction and subjected to direct sequencing. Results All 21 patients were diagnosed with MLD clinically and genetically, out of which 17 patients were late infantile and 4 were juvenile types. A total of 34 ARSA mutations, including 28 novel mutations (22 missense, 1 splicing, 1 nonsense, 3 small insertions, and 1 small deletion mutation) and 6 known mutations (5 missense and 1 small insertion mutation), were identified. Prenatal diagnosis was performed for four pedigrees. One fetus was a patient, two fetuses were carriers, and two were wild type. Conclusions The present study discovered 28 novel ARSA mutations and widely expanded the mutation spectrum of ARSA. Four successful prenatal diagnoses provided critical information for MLD families.

A Review on Diabetes Mellitus Current Update on Management and Treatment

Diabetes mellitus (DM), or simply diabetes, is a group of metabolic diseases in which a person has high blood sugar, either because the body does not produce enough insulin, or because cells do not respond to the insulin that is produced. This high blood sugar produces the classical symptoms of polyuria (frequent urination), polydipsia (increased thirst) and polyphagia (increased hunger). Conventionally, diabetes has been divided into three types namely: Type 1 DM or insulin-dependent diabetes mellitus (IDDM) in which body fails to produce insulin, and presently requires the person to inject insulin or wear an insulin pump. This is also termed as juvenile Type 2 DM or non-insulin-dependent diabetes mellitus (NIDDM), results from insulin resistance, a condition in which cells fail to use insulin properly, with or without an absolute insulin deficiency. This type was previouslyreferred to as or adult-onset The third main type is gestational diabetes which occurs when women without a previous history of diabetes develop a high blood glucose level during her pregnancy. It may precede development of type 2 DM. Currently available pharmacotherapy for the treatment of diabetes mellitus includes insulin and oral hypoglycemic agents. Such drugs acts by either increasing the secretion of insulin from pancreas or reducing plasma glucose concentrations by increasing glucose uptake and decreasing gluconeogenesis. However these current drugs do not restore normal glucose homeostasis for longer period and they are not free from side effects such as hypoglycemia, kidney diseases, GIT problems, hepatotoxicity, heart risk problems, insulinoma and they have to take rest of life. Various herbal drugs have been also proved effective due to their beneficial contents in treatment of diabetes. The present review therefore is an attempt to focus on the physiological aspects of diabetes, its complications, goals of management, and synthetic and herbal treatment of diabetes.

CLINICAL CASE OG GRANULAR CELL OVARIAN TUMOR

Ivestigation of the diagnostics issues, treatment of granulosa-cell tumors of the ovary (GCTO), especially granulosa-cell tumors of the juvenile type (GCTJT), showed insufficient study of the problem, and many questions are quite contradictory. This is due to their relatively rare occurrence and a small number of analyzed observations, which does not allow us to draw conclusions on the main issues of problems with the required degree of reliability. The articlepresents a clinical case of GCTJT in a girl aged 17 years, which occurred in the practice of the doctors at the gynecological department of a hospital. In the case of ovaries tumor formation presence with a solid and cystic component GCTJT should be suspected. Tumor-like formations of applications with a solid component require the determination of antimulhuric hormone as a marker of GCTO. In case of GCTJT suspicion, it is useful to supplement the diagnostic stage by determining of the serum inhibin B level as a marker of the disease. The oncomarkers СА-125 and НЕ-4 are not specific markers GCTO. In the case, described below, the level of tumor markers of ovaries of CA-125 and NE4 was in the normal range. At the same time, the level of antimuler hormone, whose value was 15 times higher than the maximal reference value, was more indicative. The main treatment of GCTO is surgical. The operation allows to obtain the maximum diagnostic information for the planning of the further tactics. Introduction of on-line communication with oncologists in case of suspicion of malignancy of the process, improves the quality of diagnosis and provision of surgical care, since GCTO are rarely encountered in the practice of operating gynecologists and the most important is the intraoperative choice tactics. The familiarization of specialists with the clinical case of granulosic-cell tumor of the juvenile type ovary, described below - took place in our practice and we believe it will be useful to gynecologists.

INTEGRATED SIGMA QUANTA THERAPY AS A POSSIBLE ADJUNCT FOR TYPE 1 DIABETES: A CASE STUDY

BackgroundRegarding juvenile onset type 1 diabetes [JIDDM], although there have been advancements in the monitoring of blood glucose [BG] levels, as well as in the delivery of insulin, the end result has essentially remained the same, in that BG levels are measured in some manner, in order to determine the dose of insulin required to attempt to maintain BG and A1C levels within normal values. The present study examined a possible methodology by which chemical, physical, and psychoneurological factors are also considered in an approach designed to diminish the dependency on insulin alone to control blood glucose in JIDDM.MethodThis study was comprised of one newly diagnosed JIDDM female (age 8 years at onset of IDDM). The participant continued her usual pre‐diabetic lifestyle, but followed a standard routine of blood glucose monitoring and appropriate administration of insulin for blood glucose management for 45 weeks. Following 45 weeks of a conventional JIDDM management routine, blood work was obtained to establish a baseline A1C. At this point, 15 weeks of additional procedures were implemented, consisting of chemical (dietary), physical (exercise/treatment modalities), and psychoneurological (anti‐stress) protocols for comparison, with blood work repeated after an additional 7.5 and 15 weeks of observation.ResultsAfter the initial 45 week period, allowing the participant to adjust to the life and routine of having IDDM, her A1C value (normal range of 4.0–5.7%) was noted to be reduced from 9.0% at the onset of JIDDM to 7.1% with conventional management of BG levels through frequent BG measurement and insulin use alone. Upon implementing the additional chemical, physical, and psychoneurological protocols for 15 weeks, the A1C value was reduced to 7.0% at the halfway point, and was further reduced to 6.8% after the 15 week period.ConclusionAn evidence‐based understanding of JIDDM, classical management through BG monitoring and insulin administration, as well as, integration of chemical, physical, and psychoneurological methods of treatment can be used to leverage improved health in JIDDM, where higher mean BG levels, as indicative with higher A1C measurements, are linked to an increased risk for various health conditions. The typical management routine over the initial 45 week period indicated a need for more frequent BG monitoring, as well as the need for more insulin when compared to the 15 week modified routine. Additionally, there was an increase in the difficulty of management of normal BG levels with the traditional JIDDM management routine. It was apparent that monitoring BG and administering insulin alone may not be the most effective approach to managing BG levels; incorporating a modified routine, with additional focus on diet, physical input, and stress reduction, improved clinical management of BG levels, and following the modified routine appeared to increase the sensitivity to insulin, whereas less insulin was required to control the BG levels in JIDDM when compared to a conventional management style.Support or Funding InformationSupported by Institutional Resources of USAT Montserrat and th Einstein Medical Institute, NPB, FL.This abstract is from the Experimental Biology 2018 Meeting. There is no full text article associated with this abstract published in The FASEB Journal.