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Case Reports 2015; 36(2): 119-123 PubMed PMID: 26071578 Citation  Keywords:  Adrenal Insufficiency:chemically induced, Adult, Female, Hashimoto Disease:chemically induced, Human Growth Hormone:adverse effects, Humans, Polyendocrinopathies, Autoimmune:chemically induced, Turner Syndrome:drug therapy, Young Adult,.   

: Autoimmune polyglandular syndrome is a constellation of signs and symptoms of simultaneous insufficiencies of several endocrine glands. Autoimmune polyglandular syndrome type 2 (APS 2) may be diagnosed when the adrenocortical insufficiency is associated with an autoimmune thyroid disease (Hashimoto's thyroiditis or Graves' disease), and/or insulin-dependent diabetes mellitus. Turner syndrome is the most common chromosomal disorder in females, caused by complete or partial X chromosome monosomy. We present the case of a 20-year-old woman with Turner syndrome, in whom APS 2 (Hashimoto's thyroiditis and adrenocortical insufficiency) has been diagnosed after introduction of recombinant human growth hormone (rhGH) therapy. In Turner syndrome, examination of the patient must regularly be conducted in order to diagnose a possible onset of autoimmune diseases; respective treatment must be applied as soon as the diagnosis is established. In particular, therapy of rhGH, used for short stature treatment, may be a trigger factor of adrenal insufficiency. The cortisol level in blood should be assessed before rhGH administration and carefully monitored during the therapy, especially in case of autoimmune thyroid disease coexistence....

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Case Reports 2015; 36(2): 112-114 PubMed PMID: 26071576 Citation  Keywords:  Aged, 80 and over, Autoimmune Diseases:blood, Disease Progression, Empty Sella Syndrome:blood, Humans, Immunoglobulin G:blood, Male, Pituitary Diseases:blood,.   

: An 87-year-old man was admitted with fatigue, anorexia, vomiting, urinary incontinence, and a depressive state. His consciousness was evaluated as a 13 on the Glasgow Coma Scale (E4V3M6), and he had a body temperature of 36.4°C, a blood pressure of 91/60 mmHg, and a heart rate of 88 beats/min. General laboratory data were unremarkable except for a mildly elevated serum creatinine level. The plasma levels of growth hormone, luteinizing hormone, and follicle stimulating hormone were depressed. On the other hand, the prolactin level was elevated, and the corticotropin, cortisol, and thyrotropin levels were within the reference ranges. Cranial magnetic resonance imaging (MRI) revealed the marked swelling of the pituitary gland and the infundibular stalk, and the serum immunoglobulin G4 (IgG4) level was elevated (2.85 g/L; reference range, 0.048-1.05 g/L). Accordingly, a diagnosis of IgG4-related autoimmune hypophysitis (AH) was made. The patient responded well to glucocorticoid therapy, but the presence of diabetes insipidus was revealed and was subsequently controlled using desamino-D-arginine vasopressin (DDAVP). To our surprise, an empty sella was apparent on an MRI examination performed on Day 12. The patient's serum IgG4 level had decreased in a log-linear manner with a half-life of 30 days, which was comparable to the half-life of IgG4 in control subjects (21 days). At a 16-month follow-up examination, no substantial changes in the morphology or function of the pituitary gland were noted. In conclusion, an empty sella developed within 12 days after the clinical onset of AH in the present case, suggesting that an empty sella may be the direct outcome of AH. The conversion of AH to an empty sella was associated with an immediate shutdown of IgG4 overproduction....

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Journal Article 2015; 36(1): 72-79 PubMed PMID: 25789587 Citation  Keywords:  Adolescent, Autonomic Nervous System:physiopathology, Colitis, Ulcerative:physiopathology, Crohn Disease:physiopathology, Female, Heart Rate:physiology, Humans, Inflammatory Bowel Diseases:physiopathology, Male,.   

OBJECTIVE: Heart rate variability (HRV) oscillations are used in the detection of autonomic instabilities in various clinical disorders.

METHODS: We compared the HRV as a possible marker of chronic distress in children with inflammatory bowel disease (IBD) with HRV frequencies in the healthy controls. Participants were 29 children with IBD (19 Crohn's disease and 10 ulcerative colitis), 25 children were in remission and 4 presented mild disease activity. They were compared with the control group of 35 healthy children of the same age (13-16 years-old).

RESULTS: In HRV assessment, adolescents with IBD had significantly lower levels of the spectral activity in an LF band in all three positions; lower levels of VLF in both supine positions; and the ratio of the spectral activity at LF/HF was significantly lower in the second post (standing).

CONCLUSION: These results indicate children with IBD have less adaptability to stress....

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Journal Article 2014; 35(7): 577-585 PubMed PMID: 25617880 Citation  Keywords:  Adult, Antioxidants:metabolism, Autoimmune Diseases:immunology, Bacterial Translocation:immunology, Epitopes:immunology, Fatigue Syndrome, Chronic:immunology, Female, Humans, Immunoglobulin M:immunology, Male, Middle Aged, Nitroso Compounds:immunology, Ox.   

OBJECTIVES: There is evidence that inflammatory, oxidative and nitrosative stress (IO&NS) pathways participate in the pathophysiology of a subgroup of patients with Myalgic Encephalomyelitis/chronic fatigue syndrome (ME/CFS). Increased IgM-related autoimmune responses to oxidative specific epitopes (OSEs), including malondialdehyde (MDA), oleic acid and phosphatidyl inositol (Pi), and nitroso-(NO)-adducts, including NO-tryptophan (NOW), NO-arginine and NO-cysteinyl, are frequently observed in ME/CFS. Autoimmune responses in ME/CFS may be driven by increased bacterial translocation as measured by IgM and IgA responses to LPS of gram negative bacteria.

METHODS: The aim of this study is to examine whether IgM responses to OSEs and NO-adducts are related to a better outcome as measured by the Fibromyalgia and Fatigue Rating Scale (FF). 76 ME/CFS patients with initially abnormal autoimmune responses were treated with care-as-usual, including nutraceuticals with anti-IO&NS effects (NAIOS), such as L-carnitine, coenzyme Q10, taurine + lipoic acid, with or without curcumine + quercitine or N-acetyl-cysteine, zinc + glutamine.

RESULTS: We found that use of these NAIOS was associated with highly significant reductions in initially increased IgM-mediated autoimmune responses to OSEs and NO-adducts. A greater reduction in autoimmune responses to OSEs during intake of these NAIOS was associated with a lower FF score. Reductions in IgM responses to oleic acid, MDA and Pi, but not in any of the NO-adducts, were associated with reductions in severity of illness. These associations remained significant after adjusting for possible effects of increased bacterial translocation (leaky gut).

CONCLUSIONS: Our results show that autoimmune responses to OSEs are involved in the pathophysiology of ME/CFS and that these pathways are a new drug target in a subgroup of ME/CFS patients. Although hypernitrosylation and nitrosative stress play a role in ME/CFS, reductions in these pathways are not associated with lowered severity of illness. Randomized controlled trials with NAIOS should be carried out in the subgroup of ME/CFS patients with initially increased autoimmune responses to OSEs....

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Case Reports 2014; 35(5): 335-341 PubMed PMID: 25275265 Citation  Keywords:  Adolescent, Antithyroid Agents:administration & dosage, Autoantibodies:immunology, Child, Child, Preschool, Female, Graves Disease:drug therapy, Hashimoto Disease:drug therapy, Humans, Hypothyroidism:chemically induced, Male, Methimazole:administration & .   

BACKGROUND: The main clinical manifestations of autoimmune thyroid diseases are Graves' disease (GD) and Hashimoto's thyroiditis (HT). Graves' disease is the cause of most cases of hyperthyroidism in childhood. Indications for radical therapy (surgery or 131I treatment) in children are still a matter of discussion, as sustained (sometimes very long) remission of GD is possible, while the radical therapy almost always leads to hypothyroidism. Spontaneous evolution from GD with hyperthyroidism to HT with hypothyroidism may also be observed.

OBJECTIVE: The aim of the study was to analyze the clinical course of 6 cases of hyperthyroid girls with GD in whom a normalization of previously increased autoantibodies against thyrotropin (TSH) receptor (anti-TSHR) was observed together with a significant increase in autoantibodies against thyroid peroxidase (anti-TPO) and thyroglobulin (anti-Tg), with concomitant hypo- or euthyroidism but no recurrence of hyperthyroidism.

SUBJECTS: Patients' age at diagnosis ranged from 5.0 to 16.5 years. Two (2) patients had Turner syndrome, another one (1), diabetic, was on insulin therapy.

RESULTS: In all the girls, antithyroid drugs were administered and euthyroid state was achieved during the first 2.0-3.5 months of the treatment. Mild side effects were observed in only one case. The therapy was continued up to 1.5-4.0 years. Relapses during the therapy were observed in 2 cases. Up to now, no relapses have been observed for 0.5-7.5 years since the therapy withdrawal in 5 patients (1 patient was lost to follow-up), 2 patients are currently treated with levothyroxine due to hypothyroidism.

CONCLUSIONS: It seems that the prolonged pharmacotherapy with antithyroid drugs, followed by observation after remission of hyperthyroidism, may be an appropriate therapeutic option at least in some children with GD as they can be cured without radical therapy and the potential risks of such treatment....

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Case Reports 2014; 35(3): 179-182 PubMed PMID: 24977964 Citation  Keywords:  Adult, Anti-Inflammatory Agents, Non-Steroidal:therapeutic use, Bromocriptine:therapeutic use, Female, Humans, Pituitary Neoplasms:drug therapy, Polyendocrinopathies, Autoimmune:drug therapy, Prolactinoma:drug therapy,.   

: Excessive prolactin release and/or receptor action may be implicated in the pathogenesis of many autoimmune disorders. We report here a case of a woman who developed Graves' disease and subclinical autoimmune adrenal failure, and was diagnosed as having autoimmune polyglandular syndrome type 2. Because of coexisting microprolactinoma she was treated with bromocriptine for 24 months. This treatment resulted in a normalization of thyroid and adrenal function tests (with the exception of 21-hydroxylase antibodies) and reduced monocyte cytokine release. Our study indicates that bromocriptine and probably also the remaining prolactin-lowering agents produce anti-inflammatory effects and may prevent or delay the progression of autoimmune disorders of endocrine glands....

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Case Reports 2014; 35(2): 95-97 PubMed PMID: 24878970 Citation  Keywords:  Adult, Autoantibodies:blood, Female, Humans, Limbic Encephalitis:blood, Magnetic Resonance Imaging, Proteins:immunology,.   

: We report a case of limbic encephalitis with LGI1 antibodies and cranial MRI abnormality. A 41-year-old woman who presented with confusional state that had started 1 month ago. Brain magnetic resonance imaging revealed hyperintense signal in right basal ganglia followed by hyperintense signals in left hippocampus and bilateral basal ganglia half a month later. This case expands the spectrum of limbic encephalitis to include LGI1 antibodies and cranial MRI changing process....

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Journal Article 2013; 34(7): 701-707 PubMed PMID: 24464014 Citation  Keywords:  Abortion, Habitual:epidemiology, Adult, Antibodies, Anticardiolipin:blood, Antibodies, Antinuclear:blood, Autoantibodies:blood, Female, Humans, Interferon-gamma:blood, Lupus Coagulation Inhibitor:blood, Middle Aged, Placenta:immunology, Pregnancy, Risk Fa.   

OBJECTIVE: The 155 patients suffering from primary RM who took part in this study were qualified after excluding known causes of abortions.

METHODS: The presence of the following immunological factors was examined in serum samples: autoantibodies such as anti-cardiolipin (ACA) IgG and IgM, lupus-anticoagulant (LA), anti-thyroid (ATA): anti-thyroglobulin (anti-TG) and anti-thyroid peroxydase (anti-TPO), anti-nuclear (ANA), anti-placental (APA) antibodies and alloimmunological disturbances following HLA-class I antibody test (LCT-lymphocytotoxic test), lack of blocking proliferative activity in mixed lymphocyte reaction test (MLR), anti-sperm (ASA) antibodies, levels of extracellular interferon γ (IFN γ) and tumour necrosis α (TNFα) produced by peripheral blood lymphocytes.

RESULTS: Immunological disturbances were found in 69.1% of the patients suffering from primary RM of unknown etiology. The number of RM correlated with the frequency of immunological disturbance. Among the samples from patients who underwent ≥5 RM, positive levels of TNFα were the most frequently observed risk factor (up to 27%) (p=0.05). Although the incidence of immunological factors was comparable between patients with early and late pregnancy losses, elevated ATA levels were frequently seen among late miscarriage (95% CI=36.0, p≤0.01).

CONCLUSION: The heterogeneity of immunological risk factors shown in this study indicates the usefulness of detecting alloimmune factors as well as autoantibodies in patients with RM of unknown etiology. This may be helpful to analyse the therapeutical effectivity of various treatment in a better characterized group of patients and to explain unsuccessful results of treatment in patients with RM of unknown etiology....

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