- Evans syndrome: clinical perspectives, biological insights and treatment modalities. [Review]
- JBJ Blood Med 2018; 9:171-184
- Evans syndrome (ES) is a rare and chronic autoimmune disease characterized by autoimmune hemolytic anemia and immune thrombocytopenic purpura with a positive direct anti-human globulin test. It is cl...
Evans syndrome (ES) is a rare and chronic autoimmune disease characterized by autoimmune hemolytic anemia and immune thrombocytopenic purpura with a positive direct anti-human globulin test. It is classified as primary and secondary, with the frequency in patients with autoimmune hemolytic anemia being 37%-73%. It predominates in children, mainly due to primary immunodeficiencies or autoimmune lymphoproliferative syndrome. ES during pregnancy is associated with high fetal morbidity, including severe hemolysis and intracranial bleeding with neurological sequelae and death. The clinical presentation can include fatigue, pallor, jaundice and mucosal bleeding, with remissions and exacerbations during the person's lifetime, and acute manifestations as catastrophic bleeding and massive hemolysis. Recent molecular theories explaining the physiopathology of ES include deficiencies of CTLA-4, LRBA, TPP2 and a decreased CD4/CD8 ratio. As in other autoimmune cytopenias, there is no established evidence-based treatment and steroids are the first-line therapy, with intravenous immunoglobulin administered as a life-saving resource in cases of severe immune thrombocytopenic purpura manifestations. Second-line treatment for refractory ES includes rituximab, mofetil mycophenolate, cyclosporine, vincristine, azathioprine, sirolimus and thrombopoietin receptor agonists. In cases unresponsive to immunosuppressive agents, hematopoietic stem cell transplantation has been successful, although it is necessary to consider its potential serious adverse effects. In conclusion, ES is a disease with a heterogeneous course that remains challenging to patients and physicians, with prospective clinical trials needed to explore potential targeted therapy to achieve an improved long-term response or even a cure.
- Maternal risk factors for neonatal jaundice: a hospital-based cross-sectional study in Tehran. [Journal Article]
- EJEur J Transl Myol 2018 Jul 10; 28(3):7618
- Diagnosis and timely treatment of neonatal jaundice is critical to preventing its dangerous side effects. Knowing the predisposing factors of neonatal jaundice is still a serious debate, which can be...
Diagnosis and timely treatment of neonatal jaundice is critical to preventing its dangerous side effects. Knowing the predisposing factors of neonatal jaundice is still a serious debate, which can be effective in controlling jaundice and the primary problem. The aim of this study was to evaluate maternal risk factors that contribute to the Hyperbilirubinemia among newborns admitted to Imam Khomeini and Ziaeean hospitals during 2015. We collected random samplings for the current study. Medical records for all newborns with jaundice were examined for risk factors associated with Hyperbilirubinemia. All variables were analyzed by SPSS software, version 19. Chi-square test and T-test were applied to evaluate qualitative and quantitative data, respectively. Our findings revealed that maternal age, weight, BMI, WBC, Hb, PLT, birth in the first pregnancy, numbers of pregnancies and prolonged delivery were significantly associated with bilirubin levels. Preventing the risk correlated with maternal factors or identifying neonates with these risk factors is important in effective management of infants. Therefore, the evaluation of neonatal jaundice in health care services should always be considered as a fundamental policy.
- A Low-Cost, Community Knowledge Approach to Estimate Maternal and Jaundice-Associated Mortality in Rural Bangladesh. [Journal Article]
- AJAm J Trop Med Hyg 2018 Oct 08
- In the absence of a civil registration system, a house-to-house survey is often used to estimate cause-specific mortality in low- and middle-income countries. However, house-to-house surveys are reso...
In the absence of a civil registration system, a house-to-house survey is often used to estimate cause-specific mortality in low- and middle-income countries. However, house-to-house surveys are resource and time intensive. We applied a low-cost community knowledge approach to identify maternal deaths from any cause and jaundice-associated deaths among persons aged ≥ 14 years, and stillbirths and neonatal deaths in mothers with jaundice during pregnancy in five rural communities in Bangladesh. We estimated the method's sensitivity and cost savings compared with a house-to-house survey. In the five communities with a total of 125,570 population, we identified 13 maternal deaths, 60 deaths among persons aged ≥ 14 years associated with jaundice, five neonatal deaths, and four stillbirths born to a mother with jaundice during pregnancy over the 3-year period before the survey using the community knowledge approach. The sensitivity of community knowledge method in identifying target deaths ranged from 80% for neonatal deaths to 100% for stillbirths and maternal deaths. The community knowledge approach required 36% of the staff time to undertake compared with the house-to-house survey. The community knowledge approach was less expensive but highly sensitive in identifying maternal and jaundice-associated mortality, as well as all-cause adult mortality in rural settings in Bangladesh. This method can be applied in rural settings of other low- and middle-income countries and, in conjunction with hospital-based hepatitis diagnoses, used to monitor the impact of programs to reduce the burden of cause-specific hepatitis mortality, a current World Health Organization priority.
- Intramuscular versus intravenous prophylactic oxytocin for the third stage of labour. [Review]
- CDCochrane Database Syst Rev 2018 09 22; 9:CD009332
- CONCLUSIONS: Very low-quality evidence indicates no clear difference between the comparative benefits and risks of intramuscular and intravenous oxytocin when given to prevent excessive blood loss after vaginal birth. Appropriately designed randomised trials with adequate sample sizes are needed to assess whether the route of prophylactic oxytocin after vaginal birth affects maternal or infant outcomes. Such studies could be large enough to detect clinically important differences in major side effects that have been reported in observational studies and should also consider the acceptability of the intervention to mothers and providers as important outcomes.
- Recurrent Abdominal Pain in Children. [Journal Article]
- AFAm Fam Physician 2018 Jun 15; 97(12):785-793
- Recurrent abdominal pain (RAP) in children is defined as at least three episodes of pain that occur over at least three months and affect the child's ability to perform normal activities. RAP is most...
Recurrent abdominal pain (RAP) in children is defined as at least three episodes of pain that occur over at least three months and affect the child's ability to perform normal activities. RAP is most often considered functional (nonorganic) abdominal pain, but an organic cause is found in 5% to 10% of cases. Further workup is warranted in children who have RAP and fever, vomiting, blood in the stool, more than three alarm symptoms, or a history of urinary tract infections. Physical examination findings that should prompt further workup include weight loss or failure to grow; jaundice; costovertebral tenderness or back pain with lower extremity neurologic symptoms; liver, spleen, or kidney enlargement; an abdominal mass; or localized tenderness on abdominal examination. Workup may include complete blood count, erythrocyte sedimentation rate, C-reactive protein level, fecal guaiac testing, fecal ova and parasite testing, or urinalysis. Pregnancy testing and screening for sexually transmitted infections should be considered in adolescents or if there are concerns about sexual abuse. Abdominal radiography can be helpful for diagnosing obstruction or constipation. Abdominal ultrasonography identifies an abnormality in up to 10% of children with RAP who meet criteria for further workup, compared with 1% of those who do not meet these criteria. Functional abdominal pain is a clinical diagnosis and no workup is needed. Management of functional abdominal pain focuses on improving quality of life, reducing parent and child concerns about the seriousness of the condition, and reducing the disability associated with pain rather than complete resolution of pain. Although evidence is lacking for most pharmacologic treatments of functional abdominal pain, psychological therapies such as cognitive behavior therapy and hypnotherapy have been shown to be beneficial.
- Graves' disease in children. [Journal Article]
- AEAnn Endocrinol (Paris) 2018 Aug 16
- R1 The diagnosis of Graves' disease in children is based on detecting a suppression of serum TSH concentrations and the presence of anti-TSH receptor antibodies. 1/+++. R2 Thyroid ultrasound is unnec...
R1 The diagnosis of Graves' disease in children is based on detecting a suppression of serum TSH concentrations and the presence of anti-TSH receptor antibodies. 1/+++. R2 Thyroid ultrasound is unnecessary for diagnosis, but can be useful for assessing the size and homogeneity of the goiter. 2/+. R3. Thyroid scintigraphy is not required for the diagnosis of Graves' disease. 1/+++. R4. The measurement of T4L and T3L levels is not necessary for the diagnosis of Graves' disease in children but can be useful for the management and assessment of prognosis. 1/++. R5. In the absence of TSH receptor autoantibodies, the possibility of genetically inherited hyperthyroidism must be considered. 1/++. R6. Drug therapy is the primary line of treatment for children and consists of imidazole, carbimazole or thiamazole, with an initial dosage of 0.4 to 0.8mg/kg/day (0.3 to 0.6mg/kg/day for thiamazole) depending on the initial severity, up to maximum of 30mg. 1/++. R7. Propylthiouracil is contraindicated for children with Grave's disease. 1/+++. R8. Before starting treatment, it may be useful to perform a CBC in order to assess the degree of neutropenia caused by hyperthyroidism. It is not necessary to perform systematic CBCs during follow-up. 2/+. R9. An emergency CBC should be performed if symptoms include fever or angina. If neutrophil counts are <1000/mm3, synthetic antithyroid therapy should be discontinued or decreased and may be permanently contraindicated in severe (<500) and persistent neutropenia. Otherwise treatment may be resumed. 1/++. R10. Transaminases levels should be measured before initiating treatment. Systematic monitoring of liver function is not consensually validated. 2/+. R11. In cases of jaundice, digestive disorders or pruritus, measuring liver enzymes (AST, ALT), total and conjugated bilirubin and alkaline phosphatases is indicated. 1/++. R12. Patients and parents should be informed of the possible side effects of antithyroid agents. 1/+. R13. Therapeutic education of parents and children is important in ensuring the best possible treatment compliance. 2/++. R14. Given the specificities involved in the treatment of Graves' disease in children, medical care should be provided by a specialist accustomed to treating endocrinopathies in pediatric patients. 2/+. R15. Depending on patient age, the severity of the disease at diagnosis and the persistence of anti-TSH receptor antibodies, the initial course of treatment must take place over an extended period of 3 to 6 years. R16.The anticipated success rates of medical treatment (50% of patients in remission following several years of treatment) should be explained to the family and the child. The possibility that radical treatment may be required in case of failure or intolerance of medical treatment should also be discussed. 1/++. R17.In females with Graves' disease, it is important to explain that they must undergo an assessment by an endocrinologist before planning future pregnancies, from the start of pregnancy and during the course of pregnancy. This is true in all female patients, even those in remission after medical treatment, or those who have undergone radical treatment. R18.Indications for a radical treatment can arise in cases of: 1/+: contraindication to antithyroid agents; poorly controlled hyperthyroidism due to lack of compliance; relapse despite prolonged medical treatment; a request made by the family and child for personal reasons. R19.Surgery is the radical method of treatment used in children under 5 years of age, or in cases of very large, nodular, or compressive goiters. 2/++. R20. The surgeon's experience in dealing with thyroidectomies in children is likely to be the most significant determining factor in limiting the morbidity of the procedure (alongside any collaboration between a pediatric surgeon and an adult surgeon). 1/++. R21 When radical treatment is indicated, I-131 treatment may be discussed after 5 years (but more often after puberty), if the goiter is not too large. Experience from monitoring Graves' disease in North American children is reassuring. 1/++.
- Leptospirosis in pregnancy: A lesson in subtlety. [Journal Article]
- MJMalays J Pathol 2018; 40(2):169-173
- CONCLUSIONS: Leptospirosis may have a subtle presentation and a high index of suspicion for this infection is required for early identification of the disease.
- New Insights in Genetic Cholestasis: From Molecular Mechanisms to Clinical Implications. [Review]
- CJCan J Gastroenterol Hepatol 2018; 2018:2313675
- Cholestasis is characterised by impaired bile secretion and accumulation of bile salts in the organism. Hereditary cholestasis is a heterogeneous group of rare autosomal recessive liver disorders, wh...
Cholestasis is characterised by impaired bile secretion and accumulation of bile salts in the organism. Hereditary cholestasis is a heterogeneous group of rare autosomal recessive liver disorders, which are characterised by intrahepatic cholestasis, pruritus, and jaundice and caused by defects in genes related to the secretion and transport of bile salts and lipids. Phenotypic manifestation is highly variable, ranging from progressive familial intrahepatic cholestasis (PFIC)-with onset in early infancy and progression to end-stage liver disease-to a milder intermittent mostly nonprogressive form known as benign recurrent intrahepatic cholestasis (BRIC). Cases have been reported of initially benign episodic cholestasis that subsequently transitions to a persistent progressive form of the disease. Therefore, BRIC and PFIC seem to represent two extremes of a continuous spectrum of phenotypes that comprise one disease. Thus far, five representatives of PFIC (named PFIC1-5) caused by pathogenic mutations present in both alleles of ATP8B1, ABCB11, ABCB4, TJP2, and NR1H4 have been described. In addition to familial intrahepatic cholestasis, partial defects in ATP8B1, ABCB11, and ABCB4 predispose patients to drug-induced cholestasis and intrahepatic cholestasis in pregnancy. This review summarises the current knowledge of the clinical manifestations, genetics, and molecular mechanisms of these diseases and briefly outlines the therapeutic options, both conservative and invasive, with an outlook for future personalised therapeutic strategies.
- Efficacy and Safety of Intravaginal Misoprostol for Mid-trimester Medical Termination of Pregnancy. [Journal Article]
- MMMymensingh Med J 2018; 27(3):544-549
- Misoprostol has been widely used in Obstetrics and Gynecology for cervical priming, medical abortion and induction of labour. The purpose of the present study was to evaluate the efficacy and safety ...
Misoprostol has been widely used in Obstetrics and Gynecology for cervical priming, medical abortion and induction of labour. The purpose of the present study was to evaluate the efficacy and safety of intravaginal misoprostol in mid-trimester medical termination of pregnancy. This non-randomized clinical trial was carried out in the Department of Obstetrics and Gynecology at Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh from July 2007 to June 2008 for a period of one year. Primi- or multigravid women with 13 to 28 weeks of singleton pregnancy, fetal congenital malformation and missed abortion who were selected for medical termination of pregnancy were included in this study. Women with spontaneous labour, low laying placenta, ruptured membrane with or without chorioamnionitis, acute bronchial asthma, glaucoma, history of hypersensitivity of PGs, uncontrolled hypertension, cardiac disease, jaundice, renal disease were excluded from this study. The selected patients received 200μg of misoprostol 6 hourly pervaginally upto 48 hours. The outcome variables were induction of delivery time, number of doses within 48 hours, result of induction, surgical evacuation, bleeding after induction, requirement of blood transfusion and Dinoprostone gel; side effect & complication of misoprostol were recorded. A total number of 30 women were recruited for this study. Delivery within 24 and 48hours were 43.3% cases and 38.3% cases respectively. Surgical evacuation was needed in 23.33% cases. Cramping (26.7%), had nausea (10.0%), vomiting (10.0%) and fever (26.7%) were reported. Out of all respondents 90.0% had average and 10.0% had more than average bleeding. For termination of pregnancy 200μg of intravasinat misoprostol are safe and effective in mid-trimester.
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- The impact of extremes of maternal age on maternal and neonatal pregnancy outcomes in women with pregestational diabetes mellitus. [Journal Article]
- JMJ Matern Fetal Neonatal Med 2018 Aug 13; :1-5
- CONCLUSIONS: Pregnancy outcomes in women with pregestational diabetes differ depending on maternal age category. Teens are at higher risk for preeclampsia and LGA neonates, but at lower risk for cesarean. Women aged 35-39 years are at higher risk for cesarean delivery, are less likely to have LGA neonates, and more likely to experience IUFD. Understanding the etiologies behind these differences may lead to improvements in these clinical outcomes.