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Frontiers in Medicine 2022We present the case of a female patient with a heterozygous somatic BLNK mutation, a T-cell LGL (large granular lymphocyte) leukemia, and multiple autoimmune diseases....
We present the case of a female patient with a heterozygous somatic BLNK mutation, a T-cell LGL (large granular lymphocyte) leukemia, and multiple autoimmune diseases. Although this mutation seems uncommon especially in this kind of clinical observation, it could represent a new mechanism for autoimmune diseases associated with LGL leukemia. The patient developed several autoimmune diseases: pure red blood cell apalsia, thyroiditis, oophoritis, and alopecia areata. She also presented a T-cell LGL leukemia which required treatment with corticosteroids and cyclophosphamide, with good efficacy. Interestingly, she had no notable infectious history. The erythroblastopenia also resolved, the alopecia evolves by flare-ups, and the patient is still under hormonal supplementation for thyroiditis and oophoritis. We wanted to try to understand the unusual clinical picture presented by this patient. We therefore performed whole-genome sequencing, identifying a heterozygous somatic BLNK mutation. Her total gamma globulin level was slightly decreased. Regarding the lymphocyte subpopulations, she presented a B-cell deficiency with increased autoreactive B-cells and a CD4+ and Treg deficiency. This B-cell deficiency persisted after complete remission of erythroblastopenia and LGL leukemia. We propose that the persistent B-cell deficiency linked to the BLNK mutation can explain her clinical phenotype.
PubMed: 36465938
DOI: 10.3389/fmed.2022.997161 -
Cureus Feb 2024Xanthogranulomatous salpingo-oophoritis is an infrequent and challenging inflammatory condition of the female genital tract. It involves the destruction of the fallopian...
Xanthogranulomatous salpingo-oophoritis is an infrequent and challenging inflammatory condition of the female genital tract. It involves the destruction of the fallopian tube and ovarian tissue by infiltrating inflammatory cells comprising lipid-laden macrophages, lymphocytes, plasma cells, and multinucleated giant cells. While more commonly found in other organs like the gallbladder and kidney, its occurrence in the female genital tract is rare. We present a case of xanthogranulomatous salpingo-oophoritis in a 45-year-old woman, shedding light on its diagnostic and clinical complexities. Notably, this case features a rare histopathological finding of coexisting salpingitis isthmic nodosa (SIN) with xanthogranulomatous inflammation, adding to its uniqueness.
PubMed: 38455808
DOI: 10.7759/cureus.53693 -
Journal of Virology Jan 2020Mumps virus (MuV), an enveloped negative-strand RNA virus belonging to the family , enters the host cell through membrane fusion mediated by two viral envelope proteins,...
Disruption of the Dimer-Dimer Interaction of the Mumps Virus Attachment Protein Head Domain, Aided by an Anion Located at the Interface, Compromises Membrane Fusion Triggering.
Mumps virus (MuV), an enveloped negative-strand RNA virus belonging to the family , enters the host cell through membrane fusion mediated by two viral envelope proteins, an attachment protein hemagglutinin-neuraminidase (MuV-HN) and a fusion (F) protein. However, how the binding of MuV-HN to glycan receptors triggers membrane fusion is not well understood. The crystal structure of the MuV-HN head domain forms a tetramer (dimer of dimers) like other paramyxovirus attachment proteins. In the structure, a sulfate ion (SO) was found at the interface between two dimers, which may be replaced by a hydrogen phosphate ion (HPO) under physiological conditions. The anion is captured by the side chain of a positively charged arginine residue at position 139 of one monomer each from both dimers. Substitution of alanine or lysine for arginine at this position compromised the fusion support activity of MuV-HN without affecting its cell surface expression, glycan-receptor binding, and interaction with the F protein. Furthermore, the substitution appeared to affect the tetramer formation of the head domain as revealed by blue native-PAGE analysis. These results, together with our previous similar findings with the measles virus attachment protein head domain, suggest that the dimer-dimer interaction within the tetramer may play an important role in triggering membrane fusion during paramyxovirus entry. Despite the use of effective live vaccines, mumps outbreaks still occur worldwide. Mumps virus (MuV) infection typically causes flu-like symptoms and parotid gland swelling but sometimes leads to orchitis, oophoritis, and neurological complications, such as meningitis, encephalitis, and deafness. MuV enters the host cell through membrane fusion mediated by two viral proteins, a receptor-binding attachment protein, and a fusion protein, but its detailed mechanism is not fully understood. In this study, we show that the tetramer (dimer of dimers) formation of the MuV attachment protein head domain is supported by an anion located at the interface between two dimers and that the dimer-dimer interaction plays an important role in triggering the activation of the fusion protein and causing membrane fusion. These results not only further our understanding of MuV entry but provide useful information about a possible target for antiviral drugs.
Topics: Amino Acid Substitution; HEK293 Cells; Humans; Membrane Fusion; Mumps virus; Mutation, Missense; Phosphates; Protein Domains; Protein Multimerization; Sulfates; Viral Fusion Proteins; Virus Attachment; Virus Internalization
PubMed: 31619562
DOI: 10.1128/JVI.01732-19 -
Journal of Epidemiology Aug 2021Mumps vaccination coverage is low in Japan, partly because of its voluntary nature. Although pediatric cases of mumps virus infection are captured by the National...
BACKGROUND
Mumps vaccination coverage is low in Japan, partly because of its voluntary nature. Although pediatric cases of mumps virus infection are captured by the National Epidemiological Surveillance of Infectious Diseases program under the Infectious Disease Law, there are currently no data regarding the occurrence of mumps and its complications in adults.
METHODS
We investigated the annual incidence rates of mumps and its complications based on health insurance reimbursement data for 5,209,660 individuals aged 0-64 years for 2005-2017, obtained from JMDC Inc., to estimate the mumps-related disease burden during this period.
RESULTS
There were three mumps outbreaks (2006, 2010, and 2016) during 2005-2017. The annual incidence of mumps was highest in individuals aged 0-5 years (808-3,792 per 100,000 persons), followed by those aged 6-15 years (658-2,141 per 100,000 persons). The incidence of mumps was higher in females than in males (male/female ratio, 0.90). Among mumps-related complications, the overall incidence (per 1,000 mumps cases) was highest for orchitis (6.6), followed by meningitis (5.8), deafness (1.3), pancreatitis (0.5), and encephalitis (0.3). No cases of oophoritis were noted. The overall incidence of mumps-related complications was 2.5 times higher in males than in females.
CONCLUSIONS
This study revealed the disease burden due to mumps and its complications in Japan during 2005-2017. These data suggest the need for mumps-prevention measures in adolescents and adults, as well as in children.
Topics: Adolescent; Adult; Child; Child, Preschool; Cost of Illness; Disease Outbreaks; Female; Humans; Incidence; Infant; Infant, Newborn; Insurance, Health, Reimbursement; Japan; Male; Middle Aged; Mumps; Young Adult
PubMed: 32684528
DOI: 10.2188/jea.JE20200048 -
Health Science Reports Jul 2023Human papillomavirus (HPV) infection has been considered an important involved factor for infertility. Since one of the causes of decreased ovarian reserve is oophoritis...
BACKGROUND
Human papillomavirus (HPV) infection has been considered an important involved factor for infertility. Since one of the causes of decreased ovarian reserve is oophoritis due to viral infections, this study aimed to evaluated the association between HPV infection and ovarian reserve.
METHODS
This case-control study was performed on 219 women aged 25-35 years who were referred to the gynecologic oncology clinic during 2019-2020. The positive or negative HPV infection was confirmed by cervical biopsy and polymerase chain reaction (PCR) test. Cervical lesions or abnormalities in the cervix were assessed by colposcopy and histopathological analysis. Serum anti-Mullerian hormone (AMH) levels were measured for all participants to assess ovarian reserve.
RESULTS
The results of this study showed that in patients who were HPV positive, decreased ovarian reserve was more common than in the HPV negative group ( = 0.0001). Also, there was a significant difference between Cervical intraepithelial neoplasia (CIN) I and CIN III sub-groups in AMH level ( = 0.0001).
CONCLUSIONS
Traces of HPV have been observed in various aspects of infertility, but no study has been performed on its association with ovarian reserve. According to the results of this study, decreased ovarian reserve was more common in patients who were HPV positive.
PubMed: 37484059
DOI: 10.1002/hsr2.1343 -
Effect of GnRH agonist (deslorelin) on reproductive activity in captive female veiled chameleons ().Veterinarni Medicina Jul 2023Eighteen 5 months old veiled chameleon females () were used in the study. Seven females received subcutaneous implants with gonadotropin-releasing hormone agonist...
Eighteen 5 months old veiled chameleon females () were used in the study. Seven females received subcutaneous implants with gonadotropin-releasing hormone agonist (GnRH) deslorelin acetate whereas eleven females were used as control animals without any implants. Females were kept in five terraria, in groups of four females (in 3 terraria) and groups of three females (in 2 terraria), respectively. A minimum of one female with GnRH implants was present in each terrarium. They were kept under standard husbandry conditions. Females of both groups (females with GnRH and controls, respectively) were monitored for three years. No differences between females with GnRH implants and females without GnRH implants were observed with respect to the presence of large ovarian follicles, number of eggs/female, or number of clutches/female. GnRH implants did not prevent spontaneous reproductive activity in any of the 7 females with implants. Ovariectomy was performed in 8 females (4 females with GnRH implants and 4 control females). Ten females (3 females with GnRH implants and 7 control females) had to be euthanised. In 17 of 18 female veiled chameleons of this study histologic examination revealed heterophilic granulomatous oophoritis. The use of GnRH agonist implants did not prove to be an appropriate method for the control of reproductive function in captive female veiled chameleons.
PubMed: 37982053
DOI: 10.17221/31/2023-VETMED -
International Journal of Surgery Case... Jun 2024Xanthomatous inflammation is a rare chronic inflammatory condition typically affecting organs such as the kidney and gallbladder. Its occurrence in the female genital...
INTRODUCTION AND IMPORTANCE
Xanthomatous inflammation is a rare chronic inflammatory condition typically affecting organs such as the kidney and gallbladder. Its occurrence in the female genital tract, particularly in the ovaries and fallopian tubes, is exceptionally rare and sparsely documented.
CASE PRESENTATION
We report a unique case of xanthomatous inflammation involving the fallopian tube and ovary, characterized by the presence of hobnail cells and apocrine metaplasia. This represents the first documented instance in medical literature. A 55-year-old woman presented with pelvic masses, initially raising suspicion of more common conditions such as ovarian neoplasms or tuberculosis.
CLINICAL DISCUSSION
Xanthomatous salpingo-oophoritis (XSO) often presents with symptoms resembling ovarian tumors or infectious diseases, posing challenges in diagnosis. Accurate preoperative identification is essential to avoid unnecessary radical surgeries and optimize patient management.
CONCLUSION
This case highlights the importance of considering xanthomatous inflammation in the differential diagnosis of ovarian and tubal lesions, especially when typical symptoms of pelvic masses are present. Recognizing this rare inflammatory condition can prevent overtreatment and guide appropriate therapeutic strategies.
PubMed: 38908163
DOI: 10.1016/j.ijscr.2024.109930 -
Children (Basel, Switzerland) Jan 2024Mumps is an acute contagious viral disease caused by paramyxovirus characterized by complications that include orchitis, oophoritis, aseptic meningitis, and spontaneous...
The High Seropositivity of Mumps Virus IgG Antibodies among School-Aged Children in Rural Areas of the Mbarali District in the Mbeya Region, Tanzania: It Is High Time for Consideration in the National Immunization Program.
Mumps is an acute contagious viral disease caused by paramyxovirus characterized by complications that include orchitis, oophoritis, aseptic meningitis, and spontaneous abortion among many others. This study reports high mumps IgG seropositivity among school-aged children in rural areas of the Mbeya region, information that might be useful in understanding the epidemiology of mumps and instituting appropriate control measures including vaccination. Between May and July 2023, a cross-sectional study involving 196 enrolled children aged 5-13 years was conducted. Sociodemographic information and other relevant information were collected using a structured data collection tool. Blood samples were collected and used to detect mumps immunoglobulin G antibodies using indirect enzyme-linked immunosorbent assay (ELISA). A descriptive analysis was performed using STATA version 15. The median age of the enrolled children was 13 (interquartile range (IQR): 8-13) years. The seropositivity of mumps IgG antibodies was 88.8% (174/196, 95% CI: 83.5-92.5). By multivariable logistic regression analysis, history of fever (OR: 5.36, 95% CI: 1.02-28.22, = 0.047) and sharing utensils (OR: 8.05, 95% CI: 1.99-32.65, = 0.003) independently predicted mumps IgG seropositivity. More than three-quarters of school-aged children in rural areas of the Mbeya region are mumps IgG-seropositive, which is significantly associated with the sharing of utensils and history of fever. This suggests that the virus is endemic in this region, which calls for further studies across the country so as to institute evidence-based, appropriate control measures including a vaccination program.
PubMed: 38255386
DOI: 10.3390/children11010073 -
Cureus Oct 2023Background and objective The period spanning 1923-1927 was a turbulent period in Greek history following the catastrophic defeat of the Greek army in September 1922,...
Morbidity During the Early Interwar Period (1923-27): A Historical-Epidemiological Study of 15,146 Cases Treated at the "Agios Dimitrios" Hospital in Thessaloniki, Greece.
Background and objective The period spanning 1923-1927 was a turbulent period in Greek history following the catastrophic defeat of the Greek army in September 1922, known as the "Asia Minor Catastrophe". The massive settlement of refugees in Thessaloniki, Greece, entailed massive economic, public health, and social challenges. The present historical-epidemiological study aims to evaluate the diseases of individuals hospitalized at the "Agios Dimitrios" Hospital in Thessaloniki during the aforementioned period. Materials and methods This study involved 15,146 consecutive patients (January 1923-March 1927) treated at the hospital. Data were collected from the General Hospital of Thessaloniki "Agios Dimitrios" and were manually entered into a pre-coded database. Descriptive statistics were calculated. In addition, the case fatality rates (CFR) were calculated; the respective 95% confidence intervals (CI) were estimated. Results The most frequent causes for admission to the hospital were as follows: normal delivery/delivery without disclosed sequalae (n=1915, 12.7%), followed by tuberculosis (n=1514, 10.0%), malaria (n=1438, 9.5%), injuries/falls/fractures (n=1394, 9.2%), pneumonia/pleuritis (n=1010, 6.7%), appendicitis (n=623, 4.1%), dysentery/enterocolitis/typhoid (n=489, 3.2%), salpingitis/salpingo-oophoritis (n=358, 2.4%), soft tissue abscesses (n=309, 2.0%), hernias (n=295, 2.0%), rabies (n=239, 1.6%), metrorrhagia/menorrhagia (n=233, 1.5%), ocular cataract (n=225, 1.5%), postpartum infections/endometritis (n=181, 1.2%), uterine discomfort/uterine pain (n=162, 1.1%), nephritis/uremia (n=157, 1.0%), miscarriage (n=155, 1.0%), skin infections/inflammations excluding abscesses (n=152, 1.0%), otitis/mastoiditis/labyrinthitis (n=96, 0.6%), and peptic ulcer (n=93, 0.6%). Tuberculosis was particularly associated with high CFR (49.5%, 95% CI: 47.2-52.3%), followed by nephritis/uremia (CFR: 37.6%), dysentery/infectious enterocolitis/typhoid (CFR: 24.3%), peptic ulcer (CFR: 22.6%), pneumonia/pleuritis (CFR: 16.1%), postpartum infections/endometritis (CFR: 15.5%). Conclusions Infections predominated in the disease spectrum of the hospitalized population. The documented fatality rates were high; poor outcomes may reflect the socioeconomic adversities and limited medical means and resources available at that time.
PubMed: 37916245
DOI: 10.7759/cureus.48004 -
BMJ Case Reports Jan 2023Xanthogranulomatous endometritis (XGE) is a rare pathological entity which is characterised by sheets of foamy histiocytes and lymphoplasmacytic infiltrates. This...
Xanthogranulomatous endometritis (XGE) is a rare pathological entity which is characterised by sheets of foamy histiocytes and lymphoplasmacytic infiltrates. This condition can mimic endometrial carcinoma. We report a case, clinically suspected as carcinoma of the endometrium/ovary, which was diagnosed as XGE with left salpingo-oophoritis on histopathology.
Topics: Female; Humans; Oophoritis; Endometritis; Postmenopause; Salpingitis; Granuloma; Xanthomatosis; Endometrial Neoplasms
PubMed: 36657821
DOI: 10.1136/bcr-2021-247341