Bidirectional casual effects between periodontitis and Sjögren's syndrome: a Mendelian randomization study / 口腔疾病防治

Objective@#To evaluate the bidirectional association between periodontitis and Sjögren's syndrome using the Mendelian randomization (MR) method.@*Methods@#Genome-wide association study (GWAS) data of periodontitis (N = 45 563) and Sjögren's syndrome (N = 214 435) were selected to meet the requirements of the same ethnicity and different regions. Inverse variance-weighted (IVW), MR-Egger, and weighted median (WM) tests were used to evaluate the causal effect. Cochran's Q statistics, MR-Egger intercept, MR-PRESSO and leave-one-out analysis were used as sensitivity analyses to assess the stability and reliability of the results.@*Results@#After screening, the GWAS data of Sjögren's syndrome were based on the Finnish region, and the periodontitis GWAS data were based on the UK region, both of which originated from European ancestry. Using IVW (OR = 1.017, 95% CI = 0.956-1.082), MR-Egger (OR = 0.985, 95% CI= 0.956-1.082), and WM (OR =1.021, 95% CI = 0.948-1.099), no causal effect of Sjögren's syndrome on periodontitis was found using any of the three methods. Conversely, no causal effect of periodontitis on Sjögren's syndrome was found (IVW, OR = 1.024, 95% CI = 0.852-1.230; MR-Egger, OR = 0.978, 95% CI = 0.789-1.212; WM, OR = 1.024, 95% CI = 0.846-1.260). The sensitivity analyses indicated that the results were stable and reliable. Cochran's Q test and MR-PRESSO revealed that there was no significant heterogeneity among the instrumental variables, which included single nucleotide polymorphisms (SNPs). The intercept of MR-Egger regression indicated no pleiotropy in the included SNPs. No individual SNP was found that significantly affected the results using the leave-one-out method.@*Conclusion@#This study does not support a bidirectional causal effect between periodontitis and Sjögren's syndrome.

A rare case of primary ciliary dyskinesia with Kartagener抯 syndrome - A case report

Primary ciliary dyskinesia (PCD) is an autosomal recessive hereditary disease that includes various forms of ciliary ultrastructural defects. The most serious form is Kartagener syndrome (KS), which accounts for 50% of all cases of PCD. Kartagener?s syndrome is a rare disorder and the prevalence is about 1 in 30,000. It is autosomal recessive ciliary disorder comprising the triad of situs inversus totalis, chronic sinusitis, and bronchiectasis. The defective movement of cilia leads to recurrent respiratory infections, and ear/ nose/ throat infections, and infertility. The diagnosis is made clinically and confirmed through electron microscopy, which reveals abnormalities of structural organization of the axoneme in cilia from respiratory epithelia and in spermatozoa. Underlying structural defects include 1) absent inner and/or outer dynein arms, 2) tubular defects, and 3) radial spoke defects. We hereby report a rare case of Kartagener?s syndrome, in an infertile male with immotile sperms. The clinician should have a high index of suspicion, so as to make an early diagnosis. An early diagnosis helps in making the options for timely treatment of infertility may be offered and unnecessary evaluation is avoided.

Diagnosis and treatment strategies for patients with refractory recurrent aphthous ulcer and systemic diseases / 口腔疾病防治

@#Recurrent aphthous ulcer (RAU) is one of the most common diseases of the oral mucosa. At present, no effective method is available for RAU treatment, especially for refractory RAU, which significantly affects patients’ oral health and quality of life. Research shows that combination with systemic diseases greatly increases the difficulty of curing refractory RAU, making conventional oral ulcer treatment harder to perform effectively. This is probably because dentists commonly only focus on handling oral ulcers but neglect to think about the etiology of oral ulcers from a holistic perspective. Thus, we summarized some conditions of refractory RAU accompanied by systemic diseases, including inflammatory bowel disease, iron deficiency anemia, diabetes mellitus, Behçet’s disease, Reiter’s syndrome, sprue syndrome, Sutton syndrome, and acquired immunodeficiency syndrome. We also outlined the treatment principles of these patients. To be specific, on the one hand, dentists should cooperate with the relevant specialists to treat the systemic diseases, while on the other hand they should take measures including topical/general use of medicine, local physical therapy, Traditional Chinese medicine treatment, and psychotherapy for RAU management. This paper aims to provide clinicians with a more comprehensive understanding of the diagnosis and treatment of refractory RAU, in order to make personalized treatment plans for patients and improve the clinical efficacy of refractory RAU.

Expert Consensus on Bilateral Inferior Petrosal Sinus Sampling in the Diagnosis of Cushing′s Syndrome (2023) / 罕见病研究

@#Cushing′s syndrome(CS)is a clinical syndrome caused by a variety of causes, with main manifestations exhibited by central obesity, purple skin striae, hypertension, and diabetes. In patients with adrenocorticotropic hormone(ACTH)-dependent CS, the result of bilateral inferior petrosal sinus sampling(BIPSS)is the gold standard for determining the source of ACTH in the absence of routine imaging findings.However, the indications, contraindications, operating procedures, precautions and outcome judgments of BIPSS differ from one medical center to another, and there are currently no international and domestic clinical guidelines and expert consensus on BIPSS. In order to further improve the operation specifications of BIPSS in the diagnosis process of difficult CS, the Hypothalamic and Pituitary Disease Group of the China Alliance for Rare Diseases and the Innovation Center of Pituitary Diseases of Peking Union Medical College Hospital organized experts in endocrinology, interventional radiology, neurosurgery and laboratory in China to formulate this consensus. This consensus aims to provide standardized methodological guidance for the use of BIPSS in the differential diagnostic process of CS in clinical practice.

Sjogren’s Syndrome with Interstitial Lung Disease

@#Sjogren’s syndrome (SS) is an autoimmune disease affecting exocrine glands with known properties to cause chronic systemic multi-organ disease involvement. It produced the typical features of sicca syndrome, but due to insidious onset of the disease, patient may present with complications of the condition at the initial presentation. We present a case of primary Sjogren’s syndrome (SS) who first presented with pulmonary manifestations and was subsequently diagnosed as Lymphocytic Interstitial Pneumonia (LIP). The patient was extensively investigated and received appropriate treatment modalities.

Presentación inusual del síndrome de Lutembacher / Unusual presentation of Lutembacher’s syndrome

Resumen Introducción: El síndrome de Lutembacher corresponde a la asociación de un defecto interauricular (congénito o iatrogénico) e insuficiencia o estenosis mitral (congénita o adquirida). La etiología reumática es la causa más frecuente del compromiso mitral. Tiene una prevalencia reportada de 0.001 por cada 1.000.000 habitantes. Caso clínico: Paciente femenina de 6 años con cuadro de 10 meses de palpitaciones asociadas a dolor torácico. Se realizó un electrogardiograma en el que se evidenció bloqueo incompleto de la rama derecha del haz de His y prolongación del intervalo PR, además de un ecocardiograma en el que se visualizó una comunicación interauricular tipo ostium secundum no restrictiva de 28 mm, con cortocircuito de izquierda a derecha, dilatación del ventrículo derecho, prolapso de válvula mitral, valvas engrosadas e insuficiencia mitral moderada a grave. Se realizó plastia de válvula mitral y cierre quirúrgico de la comunicación interauricular, sin complicaciones. Durante el seguimiento se encuentra asintomática desde el punto de vista cardiovascular, en manejo farmacológico. Conclusiones: La asociación Lutembacher tiene una prevalencia de 0.001/1.000.000 habitantes; la mayoría tiene etiología reumática. La paciente no tiene historia de fiebre reumática y sería la paciente más joven reportada en la literatura con síndrome de Lutembacher.

Abstract Introduction: Lutembacher’s syndrome corresponds to the association of an atrial septal defect (congenital or iatrogenic) and mitral regurgitation or stenosis (congenital or acquired), with rheumatic etiology being the most-frequent cause of mitral regurgitation. It has a reported prevalence of 0.001 for every 1,000,000 inhabitants. Clinical case: Female patient six years of age with a 10-month condition of palpitations associated with chest pain. An electrocardiogram was performed with evidence of incomplete right His bundle branch block and PR interval prolongation; additionally, an echocardiogram showed 28-mm non-restrictive ostium secundum atrial septal defect, with left-to-right shunt, right ventricular dilation, mitral valve prolapse, thickened valves, and moderate-to-severe mitral regurgitation. Mitral valve plasty and surgical closure of the atrial septal defect were performed, without complications. During follow-up, she was asymptomatic from the cardiovascular point of view, under pharmacological management. Conclusions: Lutembacher’s association has a prevalence of 0.001/1´000.000 inhabitants; the majority with rheumatic etiology. Our patient has no history of rheumatic fever and would be the youngest patient reported in the literature with Lutembacher’s syndrome.

A rare case of Kartagener’s syndrome - A case report

Kartagener?s syndrome is a rare disorder. The estimated prevalence of Kartagener?s syndrome is about 1 in 30,000. It is autosomal recessive ciliary disorder comprising the triad of situs inversus totalis, chronic sinusitis, and bronchiectasis. There is a defective movement of cilia, which leads to recurrent respiratory infections, and ear/ nose/ throat infections, and infertility. We hereby report a rare case of Kartagener?s syndrome, an infertile male with azoospermia. The clinician should have a high index of suspicion, so as to make an early diagnosis. An early diagnosis helps in such patients so that the options for timely treatment of infertility may be offered and unnecessary evaluation is avoided.

Outcomes after laparoscopic adrenalectomy for unilateral primary aldosteronism / Resultados luego de adrenalectomía laparoscópica en hiperaldosteronismo primario unilateral

Abstract Unilateral primary aldosteronism (PA) is the most common surgically correctable cause of hypertension. Determination of success after laparoscopic adrenalectomy (LA) is limited by the lack of standardized criteria. We sought to evaluate the surgical recurrence and functional outcomes of LA in patients with Conn's syndrome applying the primary aldosteronism surgical outcome (PASO) Criteria. Descriptive obser vational analysis of patients treated with LA due to confirmed u nilateral Conn's syndrome between May 2007 and August 2020: Twenty patients were included in the cohort; 16 patients had TLA and other four PLA [58% male, median age 47 (IQR: 44-59.5) years and median follow-up of 64 (IQR: 2-156) ] months. Median tumor size was 1.2 (0.8-1.8) cm. No conversions to open surgery were recorded and the overall morbidity of the series was 1/20. No surgical or biochemical recurrence was observed. Five patients were excluded from the analysis of functional results due to lack of follow-up. According to the PASO criteria, complete, partial, and no success were observed in 8/15, 6/15, and 1/15, respectively. The surgical treatment of the disease is supported by the literature, and we were able to reproduce the results of other series. The use of standardized and reproducible criteria to assess its functional results would be essential for a more complete and integrated evaluation of adrenal surgery.

Resumen El hiperaldosteronismo primario es la causa más frecuente de hipertensión secundaria pasible de tratamiento quirúrgico. La determinación del éxito de la adrenalectomía laparoscópica (AL), actualmente, está limitada por la falta de criterios estandarizados. Buscamos evaluar la tasa de recurrencia quirúrgica y los resultados funcionales de la AL en pacientes con Síndrome de Conn aplicando los criterios PASO (primary aldosteronism surgical outcome). Análisis descriptivo y observacional de pacientes tratados con AL en contexto de síndrome de Conn unilateral confirmado, entre Mayo-2007 y Agosto-2020. Se incluyeron 20 pacientes en el estudio; 16 pacientes tratados mediante AL total y 4 con AL parcial (55% hombres, edad mediana de 47 (IQR: 44-59.5) años y mediana de seguimiento 64 (IQR: 2-156) meses. La mediana de tamaño tumoral fue de 1.2 (0.8-1.8) cm. No se registraron conversiones a cirugía abierta y la morbilidad global de la serie: 1/20. No se observó recurrencia quirúrgica o bioquímica. Se excluyeron 5 pacientes en el análisis de resultados funcionales por falta de seguimiento. Según los criterios PASO, se observó un éxito completo, parcial y ausente en 8/15, 6/15 y 1/15, respectivamente. El tratamiento quirúrgico de la enfermedad es avalado por la literatura y pudimos reproducir los resultados de otras series. El uso estandarizado y reproducible de criterios para valorar sus resul tados funcionales sería fundamental para una evaluación más completa e integrada de la cirugía suprarrenal.

Ossification of bilateral stylohyoid ligament with Bell facial paralysis: a case report and literature review / 口腔疾病防治

Objective @# investigate the correlation between the ossification of the styloid hyoid ligament and Bell’s facial paralysis and provide a reference for clinical diagnosis and treatment.@* Methods@# A case of ossification of the bilateral stylohyoid ligament with Bell's facial palsy caused by ossification of the bilateral stylohyoid ligament was diagnosed by clinical manifestations, differential diagnosis and imaging examination. The surgical plan was determined, and combined surgical resection of the ossified area of the styloid hyoid ligament and the greater horn of the hyoid was performed. Postoperative cefoxitin sodium anti-inflammatory treatment, methylprednisolone hormone treatment, acyclovir antiviral treatment, mecobalamin nutritional neurotherapy, and the relevant literature were analyzed. @* Results@# The patient experienced pain when swallowing before surgery, disappearance of right frontal ridges, incomplete eyelid closure, and ptosis of mouth corners. An MRI scan of the brain excluded intracranial space-occupying lesions and resulted in the diagnosis of Bell’s facial paralysis. High-resolution CT of the styloid process confirmed ossification of the styloid hyoid ligament. Styloid process shortening and partial hyoid resection were performed under general anesthesia. Half a month after discharge, the symptoms of sore throat and pain in swallowing disappeared, facial nerve function recovered well, right eyelid closure function recovered well, and right mouth droop improved. The facial nerve function basically returned to normal after 1 month of follow-up. A review of the relevant literature showed that ossification of the stylohyoid ligament to form pseudojoint dilation can locally stimulate the peripheral facial nerve and lead to facial paralysis symptoms. @*Conclusion@# Ossification of the styloid hyoid ligament is usually characterized by pharyngeal pain, which can be confirmed by imaging examination. Ossification of the styloid hyoid ligament with facial paralysis is rare in the clinic, so it is necessary to make a clear diagnosis and treat the symptoms.

Rotor’s Syndrome: A family study

@#A family was studied in which three members in the sibship belonging to the fourth generation were found to have Rotor’s syndrome. More detailed examinations including blood studies, liver profiles, oral cholecystograms, and liver biopsies where performed on the affected siblings. The results were related to what is at present known about the features and mechanisms of Rotor’s syndrome, pari passu the current concept of bilirubin metabolism. It is suggested that the constant finding, and possibly the only characteristic one in Rotor’s syndrome, is the absence of abnormal hepatic cell pigmentation. Pedigree analysis of the present family shows that the transmission of this disorder may be conditioned by an autosomal recessive gene.

Autoimmune Haemolytic Anaemia: A cross sectional study in a Tertiary Haematological Centre (Anemia Hemolitik Autoimun: Kajian Keratan Lintang di Pusat Hemotologi Tertier) / Jurnal Sains Kesihatan Malaysia

@#Autoimmune haemolytic anaemia (AIHA) is a group of disorders wherein autoantibody causes decompensated acquired haemolysis. There has been no epidemiological study of autoimmune haemolytic anaemia (AIHA) in Malaysia. This study retrospectively analysed the epidemiology of AIHA including Evan’s Syndrome in a Tertiary Haematology Centre in Malaysia. Patients diagnosed with AIHA and Evan’s Syndrome at 18 years old and above between 1 January 1994 to 1 October 2020 at the out-patient Haematology Clinic of Hospital Raja Permaisuri Bainun, Ipoh were selected. Patients’ information was retrieved from the outpatient clinic records. A total of 71 patients were included of which predominantly female. The mean age for both genders were comparable. Ethnic stratification revealed AIHA was higher in Malays followed by Chinese and Indian. Warm AIHA was most prevalent at 40.8%, compared to cold AIHA and Evan’s Syndrome (both 23.9%), and mixed AIHA (11.3%). Primary was more common than secondary AIHA followed by Evan’s Syndrome. Approximately half of the secondary AIHA and secondary Evan’s Syndrome were due to SLE. Overall, 67.6% of patients received corticosteroid only and 28.2% combined with immunosuppressant. Individuals at higher age and females have higher risk of developing AIHA and Evan’s Syndrome. The highest prevalence was seen among the Malay ethnic. Primary warm AIHA is the most common type and majority of Evan’s syndrome are secondary to autoimmune diseases.

Case series and literature review of Opalski syndrome: A rare brainstem stroke

@#A rare type of lateral medullary infarction, the Opalski Syndrome is weakness ipsilateral to the lesion of the infarct. The lesion involves the ipsilateral corticospinal tract below the pyramidal decussation. We report the first two known documented case in the Philippines, admitted on the same tertiary hospital almost one year apart.

Adrenocortical Carcinoma with Cushing’s Syndrome and Extensive Tumor Thrombosis of the Inferior Vena Cava in a 30-year-old Filipino female / Journal of the ASEAN Federation of Endocrine Societies

@#Adrenocortical carcinoma (ACC) is a rare and aggressive neoplasm with poor prognosis. We report a case of a 30-year-old female who presented with profound classic features of an adrenocorticotrophic hormone (ACTH)-independent Cushing’s syndrome (CS) and a large adrenal mass with massive venous tumor thrombosis of the entire inferior vena cava (IVC), left renal and adrenal veins confirmed by imaging. Adrenal biopsy histopathology and immunohistochemistry confirmed ACC. Systemic palliative chemotherapy was administered. This rare case presents a unique and atypical presentation of an extensive tumor thrombosis of IVC. With the advanced stage at diagnosis, aggressive nature and poor prognosis of the disease, there is still a need to determine viable therapeutic options for metastatic ACC associated with venous invasion.

Síndrome de Lermoyez, presentación de caso clínico / Lermoyez´s syndrome, a case report presentation

Lermoyez´ syndrome is an unusual clinical variant of Ménière's disease, because in both pathologies there is a peripheral vascular disorder, what It consists in the dilation of the labyrinthine membranes associated with an increase in the volume of the endolymph. Clinically, they have severe frequency fluctuations of hearing loss, tinnitus and vertigo attacks. FinaIly, it evolves to the deterioration of hearing in all frequencies. Lermoyez´ síndrome, or labyrinthine angiospasm, has an unknown etiopathogeny, although several theories have been proposed, but none explains its nature, nor its clinical course, being one of them the allergy. Their diagnosis is clinical, they present the typical triad (peripheral vertigo crisis with neurosensory hearing loss and tinnitus and/or otic fullness), but they characterised by the improvement in hearing just after a sudden vertigo attack. With the development of the disease, dizziness attacks and hearing recovery become more and more rare, and disappear. Treatment aims to relieve symptoms during crises and improve disease progression. It is present the clinical case of Lermoyez syndrome, in a male patient with allergic pathology, who was diagnosed with Ménière's disease at the beginning. It is a rare syndrome described described in classical ENT (ear-nose-throat)

El síndrome de Lermoyez es una variante clínica inusual de la enfermedad de Ménière. En ambas afecciones existe un trastorno vascular periférico, que consiste en la dilatación de las membranas laberínticas, asociadas con aumento del volumen de la endolinfa. Clínicamente, el individuo presenta fluctuaciones en la audición (hipoacusia neurosensorial en las frecuencias graves), acúfenos y ataques de vértigo. En su evolución final se deteriora la audición en todas las frecuencias. El síndrome de Lermoyez, o angioespasmo laberíntico, tiene una etiopatogenia desconocida, aunque se han propuesto varias teorías, pero ninguna explica su naturaleza ni su curso clínico, y una de ellas es la alergia. Su diagnóstico es clínico, presentan la triada típica (crisis de vértigo periférico con hipoacusia neurosensorial y acúfenos o plenitud ótica), pero el síndrome de Lermoyez se caracteriza por la mejora de la audición tras un ataque brusco de vértigo. Con el avance de la enfermedad, los ataques de vértigo y la recuperación de la audición llegan a ser cada vez más raros, hasta desaparecer. El tratamiento tiene como finalidad aliviar los síntomas durante las crisis y mejorar la evolución de la enfermedad. Se presenta un caso clínico de síndrome de Lermoyez, en un paciente varón con afección alérgica, cuyo diagnóstico de inicio fue de enfermedad de Ménière. Es un síndrome infrecuente, descrito en la otorrinolaringología clásica

Effect of CAG repeats on the age at onset of patients with spinocerebellar ataxia type 2 in China / 中南大学学报(医学版)

Objective: Spinocerebellar ataxia type 2 (SCA2) is one of the most common autosomal dominant ataxias in the world. Several reports revealed that CAG repeats in some polyQ-containing genes may affect the age at onset (AAO) of patients with SCA2, however, little studies were conducted among Chinese patients with SCA2. Thus, the aim of this study is to evaluate the effect of CAG repeats on the AAO of patients with SCA2 in China.Methods:A total of 119 patients with SCA2 were enrolled and were divided into 2 groups according to their major phenotype:17 patients from 9 families with Parkinson ' s syndrome were grouped as the Parkinson ' s disease-SCA2 (PD-SAC2); 91 patients from 66 SCA2 families and 11 sporadic SCA2 patients were grouped as the ataxia-SCA2 (A-SCA2). Blood samples were obtained from the subjects, and the CAG repeat length in ATXN2 and other (CAG)n-containing genes was screened using fluorescent PCR. The Spearman ' s rank correlation between the CAG repeat length in (CAG)n-containing genes and AAO was analyzed. Regression analysis was performed to investigate whether the CAG repeat length could explain the variant of AAO. A t-test was used to compare the difference of CAG repeat length in (CAG)n-containing genes between the PD-SAC2 and A-SCA2 groups. Results:The CAG repeat length in the longer allele of ATXN2 was negatively correlated with AAO of SCA2 (R=?0.251, P<0.05), and the CAG repeat length could explain 41.7％of the variation of AAO. AAO negatively correlated with the CAG repeat length in the shorter allele of ATXN7 (R=?0.251, P=0.006) or in the longer allele of TBP gene (R=?0.197, P=0.034). A tendency of delay in the AAO was also observed in patients with SCA2 carrying the CAG repeat within the ATXN3, CACNA1A, ATXN7, TBP, and RAI1. In addition, we found that the CAG repeat length in ATXN7 and ATXN2 between the A-SCA2 and the PD-SCA2 groups was significantly different (both P<0.05).Conclusion:The CAG repeat in ATXN2 is a major genetic factor for the AAO of patients with SCA2 in China. The CAG repeat length in ATXN3, CACNA1A, ATXN7, TBP, and RAI1 genes might be a potential factor associated with the AAO of SCA2. The CAG repeat in ATXN7 might be a potential factor affecting the Parkinson??s syndrome in SCA2.

Evaluation of the efficacy of cevimeline in improving the clinical symptoms of patients with primary Sjögren′s syndrome / 口腔疾病防治

Objective@# To investigate the clinical efficacy of cevimeline as a pharmacotherapeutic approach to stimulating gland activity in improving the symptoms and signs of primary Sjögren syndrome (pSS).@*Methods@#Sixty-three patients diagnosed with pSS who attended the Affiliated Huai'an Hospital of Xuzhou Medical University from January 2018 to September 2019 were included in this trial. They were randomly assigned to the therapeutic group and control group. All patients were recalled at baseline and after 2 weeks, 3 months and 6 months. Measurement of salivary and lacrimal flow as well as evaluation of subjective symptoms was performed at the follow-up. @*Results@# Fifty-eight patients completed the trial and were included in the statistical analysis. There was a significant difference between the two groups in the measurement of salivary and lacrimal flow at the second week and third month (P < 0.05). Improvement in subjective symptoms of oral, ocular and gland was detected at the third month (P < 0.05). At the sixth month, compared with the control group, only the salivary gland symptom score of the treatment group was statistically significant (P < 0.05). @*Conclusion@#Cevimeline has good specificity and safety and can increase salivary and lacrimal flow and improve subjective symptoms of pSS in a short time.

Paeoniflorin besylate relieves mice with antigen-induced experimental Sjogren's syndrome by regulating GRK2-JAK1-STAT1/2 signaling pathway / 中国药理学通报

Aim To observe the effect of CP-25 on the ESS mouse model and establish whether its effect is through regulating the binding of GRK2 to JAK1 and inhibiting the JAK1-STAT1/2-CXCL13 signaling pathway. Method We established ESS mouse model induced by SG protein, established into normal group, model group, CP-25 group with concentration of 35 mg • kg"1, 70 mg • kg"1, and HCQ group with concentration of 80 mg • kg"1. Mouse saliva flow was measured. The infiltration of lymphocyte in SG was observed by HE staining. The expression of p-JAKl, p- STAT1 and p-STAT2 in submandibular gland tissue was detected by Western blot. The level of CXCL13 in SG of mice was tested by IHC. GRK2 and JAK1 binding was determined by immunofluorescence and CO- IP. Results Compared with normal group, the saliva flow rate of ESS mice was low and lymphocytes were significantly infiltrated in the submandibular gland pathological sections. The CXCL13 protein level was highly expressed, which activated the JAK1-STAT1/2 signal. CP-25 significantly increased the salivary flow rate in ESS mice, reduced lymphocyte infiltration, improved pathological abnormalities, and inhibited the expression of JAK1-STAT 1/2 signaling and CXCL13. CP-25 significantly promoted the binding of GRK2 to JAK1. Conclusions CP-25 may inhibit the binding of GRK2 to JAK1, and then inhibit the activation of JAK1-STAT1/2-CXCL13 signaling pathway, improve the abnormal pathological manifestations of lymphocyte infiltration in submandibular gland, and improve the rate of saliva flow. CP-25 plays a therapeutic role in ESS mice.

A rare occurrence of Premature Birth and Recurrent Acute Pulmonary Oedema in the mother due to Cushing’s Syndrome: A case report / Journal of the ASEAN Federation of Endocrine Societies

@#Presentation of Cushing’s syndrome during pregnancy is extremely rare. We report a 21-year-old female with Cushing’s syndrome diagnosed at 23 weeks of gestation and had recurrent acute pulmonary oedema during the antepartum and postpartum period. She delivered prematurely via emergency caesarean section at 28 weeks of gestation. This case highlights the rare occurrence of recurrent acute pulmonary oedema during pregnancy and consequential premature birth in a patient with adrenal Cushing’s. She was diagnosed with adrenal Cushing’s during the postpartum period based on unsuppressed serum cortisol after overnight and low-dose dexamethasone suppression test with a suppressed ACTH. CT scan of the adrenal glands revealed a right adrenal cortical adenoma. The risk of complications in infants and mothers who suffer from Cushing’s syndrome needs to be handled carefully. The diagnosis of Cushing’s syndrome in pregnant women often overlaps and is difficult to establish in early pregnancy.

Changes of corneal nerve fibers in patients with Sjögren's syndrome treated with Fluorometholone combined with sodium hyaluronate eye drops / 国际眼科杂志(Guoji Yanke Zazhi)

@#AIM: To observe the nerve morphological changes in cornea of fluorometholone eye drops combined with sodium hyaluronate eye drops in the treatment of dry eye of Sjögren's syndrome by <i>in vivo</i> confocal microscopy(IVCM).<p>METHODS: From January 2017 to July 2020, totally 52 dry eye patients(104 eyes)of Sjögren's syndrome in our hospital were enrolled and were randomly divided into two groups: 26 patients(52 eyes)in the experimental group were treated with 0.1% fluorometholone eye drops and sodium hyaluronate eye drops, while 26 patients(52 eyes)in the control group were treated with sodium hyaluronate eye drops alone. The diameter of central corneal nerve fibers, the number of nerve beads, the score of nerve tortuosity, tear film break-up time(BUT), Schirmer I test(S I t)and non-contact intraocular pressure were measured before treatment and 2, 4 and 12wk after treatment.<p>RESULTS: There were no significant differences in the results of the diameter of central corneal nerve fibers, the number of nerve beads, the score of nerve tortuosity, BUT, S I t and non-contact intraocular pressure between the two groups before treatment(<i>P</i>>0.05). At 2, 4 and 12wk after treatment, the BUT prolonged in the experimental group compared with the control group(<i>P</i><0.05); At 12wk after treatment, the diameter of corneal nerve fibers increased, the number of nerve beads and the nerve tortuosity score decreased, the S I t increased in the experimental group compared with the control group(all <i>P</i><0.05), and there were no significant difference in non-contact intraocular pressure(<i>P</i>>0.05).<p>CONCLUSION: Fluorometholone eye drops combined with sodium hyaluronate eye drops is safe and effective in the treatment of Sjögren's syndrome related dry eye, and contribute to corneal nerve recovery.

Severe haemoptysis in a 5 year old child with Kartagener's syndrome: case report

Kartagener`s syndrome, a rare autosomal recessive disorder is a type of Primary Ciliary Dyskinesia (PCD) associated situs inversus, bronchiectasis, sinusitis and male infertility. We present a case of a 5-year-old girl who came with features of bilateral glue ear, recurrent sinusitis, recurrent hemoptysis and dextrocardia. She was diagnosed to have Kartagener`s syndrome and was evaluated for recurrent hemoptysis.