Sibling Death Research Articles

HEPATIC STEATOSIS IN CYSTIC FIBROSIS. APROPOS OF A CASE

Cystic Fibrosis (CF) is a genetic disease characterized by dysfunction of the exocrine glands. Hepatic involvement is the leading non-pulmonary cause of death in CF patients. This paper aims to present the case of a schoolchild with hepatic alterations as the initial clinical manifestation of CF. 10-year-old male with a history of sibling death due to liver failure at three years of age, multiple respiratory symptoms in the first two years of life and hospitalization at 3 years of age due to bronchitis. The condition began one year before the assessment with growth arrest and in the last six months with two diarrheal episodes without mucus or blood. On physical examination, weight and height below p5 for age, icteric color, without hepatosplenomegaly, limbs with acropaquia (Image 1). Average bone age of 5-6 years, paraclinical tests with hypertransaminasemia, hypetriglyceridemia and decreased HDL (table 1), normal blood biometry, liver elastography with ARFI 2.1 m/s and liver biopsy with macro and microvesicular steatosis with moderate portoportal fibrosis (image 1-2). Negative approach for infectious hepatitis, Wilson´s disease. 1 alpha-antitrypsin deficiency and lysosomal acid lipase deficiency. Diagnosis of CF with sweat electrolytes of 105 mmol/L was made. Currently under follow-up by gastroenterology and pediatric pulmonology. CF is the most common autosomal recessive disease in the Caucasian population, with multi-organ involvement. Liver disease has a high incidence in the first 10 years of life with 2.5/100 patient-years. However, no CFTR gene mutation has been directly associated with the presence or severity of the liver disease. Clinical presentation varies from mild asymptomatic form to cirrhosis with the need for liver transplantation in these patients. The most common initial suspicion is hepatomegaly and transaminases alteration and laboratory studies and histology alteration. Hepatic fibroelastography represents an emerging method of study for diagnosis, as it represents one of the forms of confirmation of the criteria for liver disease associated with cystic fibrosis. Liver biopsy provides information on the predominant type of lesion (steatosis or focal cirrhosis) and the extent of portal fibrosis. However, it should be taken with caution because of the risk of underestimating the severity of the lesions. Although the liver disease in cystic fibrosis does not represent the initial manifestation, the evaluation and monitoring in these patients are important for prognosis and survival since it can progress to cirrhosis and liver failure. The authors declare that there is no conflict of interest.

How Gender Shapes the Effects of Immediate Family Members’ Deaths on Adults’ Psychological Well-Being

The deaths of family members constitute one of the most serious negative life events experienced in adulthood. The impact of these losses on psychological well-being may differ considerably by the structural relationship between the deceased and the survivors, and by the genders of both family members; however, few studies have been able to explore these variations by generation, gender, and time since death. In this paper, we use mixed-methods data to explore how depressive symptoms are affected differentially in adulthood by the deaths of mothers, fathers, and siblings, as well as by the gender of survivors. We address these questions using data collected from approximately 600 adult children nested within 250 later-life families, in which approximately 55% experienced the death of at least one parent and 15% experienced the death of a sibling in the previous decade. Preliminary multilevel regression analyses showed that deaths of siblings predicted sisters’ but not brothers’ depressive symptoms. In the case of parents, only mothers’ deaths were found to predict daughters’ depressive symptoms, whereas neither parents’ deaths predicted sons’ well-being. Further, these patterns differed little by time since death. Qualitative data revealed that women were more likely to report that both their mothers’ and siblings’ deaths had led to higher conflict within the sibling network, which previous research has shown predicts psychological well-being. Taken together, these findings demonstrate the salient role of gender in shaping well-being in the face of events of deaths of parents and siblings in adulthood.

Selective screening for inborn errors of metabolism by tandem mass spectrometry at Sohag University Hospital, Egypt

BackgroundInborn errors of metabolism (IEMs) comprise a group of inherited diseases that can have devastating consequences and cause irreversible damage to different body systems and even lead to death. Newborn screening helps in the presymptomatic diagnosis of many medical disorders including IEMs. Early diagnosis and management of IEMs helps reduce morbidity and mortality. ObjectiveThis study aimed to estimate the prevalence of IEMs among at-risk children and contribute toward early diagnosis and management in order to minimize morbidity and mortality. MethodsThis prospective study was conducted at the Pediatrics and Neonatology Department, Sohag University Hospital, Egypt. The study enrolled 308 participants suspected of having IEMs. Cases were included based on the presence of any of the following: unexplained convulsions, persistent metabolic acidosis, persistent hypoglycemia, disturbed consciousness, delayed milestones, or family history of previous sibling death with IEMs or sibling death with a history suggestive of IEMs. All participants in the study were subjected to metabolic screening by tandem mass spectrometry (MS/MS). ResultsOut of 308 neonates, 93 (30.2%) were diagnosed with IEMs. The most common diagnosis was phenylketonuria, followed by glutaric aciduria type 1 and maple syrup urine disease (43%, 19.4%, and 14%, respectively). Five patients had Canavan disease, four had medium-chain acyl CoA dehydrogenase deficiency, three had congenital lactic acidosis, two had methylmalonic acidemia, and two had primary carnitine deficiency. Propionic acidemia, isovaleric acidemia, homocystinuria, short-chain acyl CoA dehydrogenase deficiency, B-ketothiolase deficiency, and ketone body utilization defect were diagnosed in one patient each. Most patients improved (73.1%) following proper specific management. ConclusionWe recommend newborn screening for IEMs using MS/MS, which may help with the early diagnosis and management of this group of disorders.

Update: Primary immunodeficiency disorders among north Indian children

Primary immunodeficiency disorders (PIDs) are a group of genetic abnormalities characterized by defectin one or more constituents of the immune system.This group of disorders are largely undiagnosed and unreported worldwide due to lack of awareness among the medical practitioners,parents as well as lack of state of art diagnostic facilities. Earlier we had reported the distribution pattern of various categories of PID in children of north India; in this report we are appending the data with current findings.In this retrospective study we pooled data from PIDs workup of 706 children with suspected PIDs, below the age of 18Yrs, in the period of May 2017 October 2019. The clinical assessment and presentation of these children was suggestive of PID. The peripheral blood of these children was used for flow cytometry based immunophenotyping of immune cells. PIDs were classified according to the International Union of Immunological Societies’ (IUIS) criteria.A total of 133 (18.38%) children were diagnosed with one or other form of PID with overall median age was 3.25 years (male: 2.3 and female: 4.2Yrs). Chronic infection, persistent diarrhea and retarded growth were the common warning signsin these patients. Combined humoral and cellular immunodeficiency was observed in 32%, phagocytic defect in 23%, antibody defect in 17%, dysregulated innate immunity in 19% and other well defined syndromes in 9% of total diagnosed PID children. Around 15.78% of PID cases were seen in coupleswithconsanguineous marriage, past family history of PID in 20.30% and families with sibling death of unknown cause in 24.06%. The cause of death of the sibling was not known. PID diagnosed children received prophylactic antibiotics and/or antifungals in addition tospecific therapy for the underlying immune deficiency.The field of PID remainsunexplored worldwide. The awareness in the developed countries is more than that of developing countries like India. The developing countries face several challenges in the diagnosis of PIDs such as awareness among patients and medical practitioners, mostly in the rural settings, lack of sufficient number of tertiary care centres, lack of equipped immunological laboratory to diagnose the disease.

Early diagnosis of Wiskott-Aldrich syndrome in the neonatal period and successful haematopoietic stem cell transplant in infancy

Wiskott-Aldrich syndrome (WAS) is a rare X-linked inborn error of immunity characterised by triad of eczema, microthrombocytopenia and recurrent infections. We herein report a case, where a male new-born with thrombocytopenia and no other symptoms was suspected of WAS in view of the significant family history of two previous sibling deaths. The diagnosis was confirmed by flowcytometry for WAS protein expression. He was initially managed with intravenous immunoglobulin replacement, regular cotrimoxazole prophylaxis, emollients and topical steroids for eczema (which child developed from the third month). The child underwent haematopoietic stem cell transplant (HSCT) at 8 months of age successfully and has remained asymptomatic at 5 years of follow-up. Early diagnosis of inborn error of immunity is extremely important for the prevention of complications and successful treatment including HSCT.

Adverse Childhood Experiences and Subsequent Chronic Diseases Among Middle-aged or Older Adults in China and Associations With Demographic and Socioeconomic Characteristics

Associations between adverse childhood experiences (ACEs) and chronic diseases among middle-aged or older Chinese individuals have not been well documented. In addition, whether demographic and socioeconomic characteristics modify any such associations has been underexplored. To examine associations between ACEs and subsequent chronic diseases and to assess whether age, sex, educational level, annual per capita household expenditure level, and childhood economic hardship modify these associations. This population-based cross-sectional study used data from the China Health and Retirement Longitudinal Study (CHARLS), a survey of residents aged 45 years or older in 28 provinces across China; specifically, the study used data from the CHARLS life history survey conducted from June 1 to December 31, 2014, and a CHARLS follow-up health survey conducted from July 1 to September 30, 2015. The study population included 11 972 respondents aged 45 years or older who had data on at least 1 of 14 specified chronic diseases and information on all 12 of the ACE indicators included in this study. Data analysis was performed from December 1 to 30, 2020. Any of 12 ACEs (physical abuse, emotional neglect, household substance abuse, household mental illness, domestic violence, incarcerated household member, parental separation or divorce, unsafe neighborhood, bullying, parental death, sibling death, and parental disability), measured by indicators on a questionnaire. The number of ACEs per participant was summed and categorized into 1 of 5 cumulative-score groups: 0, 1, 2, 3, and 4 or more. Hypertension, dyslipidemia, diabetes, heart disease, stroke, chronic lung disease, asthma, liver disease, cancer, digestive disease, kidney disease, arthritis, psychiatric disease, and memory-related disease were defined by self-reported physician diagnoses or in combination with health assessment and medication data. Multimorbidity was defined as the presence of 2 or more of these 14 chronic diseases. Logistic regression models were used to assess associations of the 12 ACEs with the 14 chronic diseases and with multimorbidity. Modification of the associations by demographic and socioeconomic characteristics was assessed by stratified analyses and tests for interaction. Of the 11 972 individuals included (mean [SD] age, 59.85 [9.56] years; 6181 [51.6%] were females), 80.9% had been exposed to at least 1 ACE and 18.0% reported exposure to 4 or more ACEs. Compared with those without ACE exposure, participants who experienced 4 or more ACEs had increased risks of dyslipidemia, chronic lung disease, asthma, liver disease, digestive disease, kidney disease, arthritis, psychiatric disease, memory-related disease, and multimorbidity. The estimated odds ratios (ORs) ranged from 1.27 (95% CI, 1.02-1.59) for dyslipidemia to 2.59 (95% CI, 2.16-3.11) for digestive disease. A dose-response association was also observed between the number of ACEs and the risk of most of the chronic diseases (excluding hypertension, diabetes, and cancer) (eg, chronic lung disease for ≥4 ACEs vs none: OR, 2.01; 95% CI, 1.59-2.55; P < .001 for trend) and of multimorbidity (for individuals among the overall study population with ≥4 ACEs vs none: OR, 2.03; 95% CI, 1.70-2.41; P < .001 for trend). The demographic or socioeconomic characteristics of age, sex, educational level, annual per capita household expenditure level, or childhood economic hardship were not shown to significantly modify the associations between ACEs and multimorbidity. In this population-based, cross-sectional study of adults in China, exposure to ACEs was associated with higher risks of chronic diseases regardless of demographic and socioeconomic characteristics during childhood or adulthood. These findings suggest a need to prevent ACEs and a need for a universal life-course public health strategy to reduce potential adverse health outcomes later in life among individuals who experience them.

Sibling Deaths, Racial/Ethnic Disadvantage, and Dementia in Later Life.

Sibling loss is understudied in the bereavement and health literature. The present study considers whether experiencing the death of siblings in mid-to-late life is associated with subsequent dementia risk and how differential exposure to sibling losses by race/ethnicity may contribute to racial/ethnic disparities in dementia risk. We use discrete-time hazard regression models, a formal mediation test, and a counterfactual simulation to reveal how sibling loss in mid-to-late life affects dementia incidence and whether unequal exposures by race/ethnicity mediate the racial/ethnic disparities in dementia. We analyze data from the Health and Retirement Study (2000-2016). The sample includes 13,589 respondents (10,670 non-Hispanic White, 1,761 non-Hispanic Black, and 1,158 Hispanic adults) aged 65 years and older in 2000 who show no evidence of dementia at baseline. Discrete-time hazard regression results show that sibling loss in mid-to-late life is associated with up to 54% higher risk for dementia. Sibling loss contributes to Black-White disparities in dementia risk. In addition, a simulation analysis shows that dementia rates would be 14% lower for Black adults if they experienced the lower rates of sibling loss experienced by White adults. This pattern was not observed among Hispanic adults. The death of a sibling in mid-to-late life is a stressor that is associated with increased dementia risk. Black adults are disadvantaged in that they are more likely than Whites to experience the death of siblings, and such losses contribute to the already substantial racial/ethnic disadvantage in dementia.

Bereavement Among Adult Siblings: An Examination of Health Services Utilization and Mental Health Outcomes.

We aimed to examine the association of young and middle-aged sibling bereavement with use of health services and risk of developing mental health disorders over 24 months before and after sibling's death. All persons aged 18-56 years who died of illness/natural causes between 2009 and 2016 were identified in the Danish Register of Causes of Death. The study sample included 31,842 bereaved siblings and 31,842 age- and sex-matched controls. Overall, the largest effect was observed for increased use of psychological services: For women, the peak was observed 4-6 months after the death (odds ratio (OR)= 3.31, 95% confidence interval (CI): 2.85, 3.85) and for men in the first 3 months after the death (OR= 2.63, 95% CI: 2.06, 3.36). The peak of being diagnosed in a hospital setting with any mental disorder for women was observed in the period 13-15 months after sibling death (OR= 1.52, 95% CI: 1.11, 2.07) and for men in the first 3 months after the death (OR= 1.75, 95% CI: 1.32, 2.32). Young and middle-aged adults who experienced the death of a sibling are more likely to use health services and are at risk of poorer mental health outcomes. During the bereavement process, young and middle-aged siblings are especially in need of mental health support, such as professional psychological counseling.

Familial Atypical Hemolytic Uremic Syndrome with Positive p.S1191L (c.3572C>T) Mutation on the CFH Gene: A Single-center Experience.

The atypical hemolytic uremic syndrome (aHUS) is characterized by thrombocytopenia, microangiopathic hemolytic anemia and acute kidney injury (AKI), which can exhibit a poor prognosis. Complement factor H (CFH) gene mutations play a key role in this disease, which may be sporadic or familial. We studied 13 people from the same family, investigated for gene mutations of the familial aHUS after a family member presented to our emergency clinic with the aHUS and reported a family history of chronic renal failure. The p.S1191L mutation on the CFH gene was heterozygous in six people from the patient’s family with the aHUS. One of these family members is our patient with acute kidney injury, and the other two are followed at the Nephrology Clinic, Medeniyat University, Goztepe Training and Research Hospital, Istanbul, Turkey, due to chronic renal failure. The other three family members showed no evidence of renal failure. The index case had a history of six sibling deaths; three died of chronic renal failure. Plasmapheresis and fresh frozen plasma treatment were administered to our patient. When the patient showed no response to this treatment, eculizumab (ECZ) therapy was started. The study demonstrated that thorough family history should be taken in patients with the aHUS. These patients may have the familial type of the disease, and they should be screened genetically. Eculizumab should be the first choice in the treatment with plasmapheresis. It should be kept in mind that the use of ECZ as prophylaxis in posttransplant therapy is extremely important for preventing rejection.

Differential in infant, childhood and under-five death clustering among the empowered and non-empowered action group regions in India

BackgroundWith 8,82,000 deaths in the under-five period, India observed varied intra-state and inter-regional differences across infant and child mortality in 2018. However, scarce literature is present to capture this unusual concentration of mortality in certain families by examining the association of the mortality risks among the siblings of those families along with various unobserved characteristics of the mother. Looking towards the regional and age differential in mortality, this paper attempts to provide evidence for the differential in mortality clustering among infants (aged 0–11 months), children (12–59 months) and under-five (0–59 months) period among mothers from the Empowered Action Group (EAG) and non-EAG regions of India.MethodsThe study used data from the National Family Health Survey (2015–16) which includes all the birth histories of 475,457 women aged 15–49 years. Bivariate and multivariate analyses were used to fulfil the objectives of the study. A two-level random intercept Weibull regression model was used to account for the unexplained mother (family) level heterogeneity.ResultsAbout 3.3% and 5.9% of infant deaths and 0.8% and 1.6% of childhood deaths were observed in non-EAG and EAG regions respectively. Among them, a higher percentage of infant and child death was observed due to the death of a previous sibling. There were 1.67 times [95% CI: 1.55–1.80] and 1.46 times [CI: 1.37–1.56] higher odds of infant and under-five mortality of index child respectively when the previous sibling at the time of conception of the index child was dead in the non-EAG regions. In contrast, the odds of death scarring (death of previous sibling scars the survival of index child) were 1.38 times [CI: 1.32–1.44] and 1.24 times [CI: 1.20–1.29] higher for infant and under-five mortality respectively in the EAG regions.ConclusionThe extent of infant and child mortality clustering and unobserved heterogeneity was higher among mothers in the non-EAG regions in comparison to their EAG region counterparts. With the growing situation of under-five mortality clustering in non-EAG states, region-wise interventions are recommended. Additionally, proper care is needed to ameliorate the inter-family variation in mortality risk among the children of both EAG and non-EAG regions throughout their childhood.

Impact of End-of-Life Circumstances on the Adjustment of Bereaved Siblings of Children Who Died from Cancer.

The aim of this study was to examine the impact of end-of-life (EoL) circumstances on grief and internalizing symptoms among bereaved siblings. Bereaved families (N = 88) were recruited from three sites 3-12months (M = 11.57, SD = 3.48) after their child's death from cancer. One sibling per family aged 8-17years (M = 12.41, SD = 2.64) was randomly selected to participate. Families completed measures of siblings' grief and internalizing symptoms, as well as a structured interview about circumstances surrounding the death. Mother and sibling reports of EoL circumstances were generally concordant, except there was a discrepancy between mothers and children about whether or not children expected their sibling's death (t(75) = 1.52, p = .018). Mother reports of sibling internalizing symptoms were above the normative mean (t(83) = 4.44, p ≤ .001 (M = 56.01 ± 12.48), with 39% (n = 33) in the borderline/clinical range. Sibling opportunity to say goodbye was associated with greater grief-related growth (t(79) = - 1.95, p = .05). Presence at the death and wishing they had done something differently were both associated with greater grief (t(80) = - 2.08, p = .04 and t(80) = - 2.24, p = .028, respectively) and grief-related growth (t(80) = - 2.01, p = .048 and t(80) = - 2.31, p = .024, respectively). However, findings were primarily unique to sibling report, with few mother-reported effects. The adjustment of bereaved siblings may be affected by certain modifiable circumstances surrounding the death of their brother or sister. A proportion of bereaved siblings had elevated internalizing symptoms irrespective of circumstances at EoL. Further work is needed to understand predictors of adjustment among bereaved siblings to provide better support and optimize their outcomes.

Religious Coping in Children With Grief After a Sibling Death Because of Cancer.

Religious Coping in Children With Grief After a Sibling Death Because of Cancer.

Psychosocial well-being and quality of life in siblings of children with congenital heart disease: A systematic review.

Congenital heart disease (CHD) is a major global health problem. Until recently, the siblings of this group did not receive much attention. This review, conducted from November 2019 to October 2020, aims to summarize knowledge about psychosocial well-being and quality of life (QoL), associated factors, and interventions for siblings of children with CHD. Systematic searches were conducted in PubMed, PsycINFO, PsycARTICLES, Web of Science via EBSCOhost, and CENTRAL. Twelve articles were included. Results showed that psychosocial well-being was impaired in 14% to 40% of siblings. Negative impact of illness was highest for CHD siblings compared to siblings of children with cancer, cystic fibrosis, or diabetes. QoL was impaired in up to one-third. Siblings of children with CHD and cancer rated their QoL lower than those of siblings of children with cystic fibrosis or type-1 diabetes. Associated factors were sibling age, gender, socioeconomic status, miscarriage, previous sibling death, visibility of illness, and severity of condition. Only one of two interventions focused on siblings of CHD children. Although data are scarce and inhomogeneous, it indicates that siblings of CHD children suffer from lower psychosocial well-being and QoL than siblings of children with other chronic conditions. Interventions to improve their situation should be developed.

Congenital cytomegalovirus infection presenting as cerebral palsy

A 9 month old boy presented with history of delayed attainment of milestones. He was born to a primigravida mother, with an uneventful perinatal period, but had a low birth weight (2.1 kg) for gestational age. There was no history of seizures, abnormal movements, loss of previously gained milestones or prior sibling deaths. On examination, he had severe microcephaly, failure to thrive and hepatosplenomegaly. Neurological examination revealed severe axial hypotonia and spastic quadriplegia with brisk deep tendon reflexes and intermittent scissoring of lower limbs. Fundus examination and hearing evaluation were normal. His current developmental age was 4 months and developmental quotient was 30.

Spectrum of Inborn errors of immunity in a cohort of 90 patients presenting with complications to BCG vaccination in India.

World Health Organisation recommends the practice of BCG vaccination at birth in countries which have a high incidence of tuberculosis and/or high leprosy burden. The BCG vaccination is considered safe for a competent immune system. However, in children with weakened immune systems cause of which can be primary or secondary, the vaccine may lead to side effects which can be localized or disseminated. In this study, we report a spectrum of inborn errors of immunity (IEI) commonly referred to as primary immunodeficiency disorders (PIDs) diagnosed in a large cohort of patients presenting with complications to BCG vaccination from India. Retrospective data analysis of patients referred to ICMR- National Institute of Immunohematology (ICMR-NIIH) for IEI workup between 2007 and 2019 was done. IEI was identified in n=52/90 (57.7%) patients presenting with BCG complications. Of these, n=13(14.4%) patients were diagnosed with severe combined immune deficiency, n=15(16.7%) with chronic granulomatous disease, n=19(21.1%) with Inborn errors of IFN-γ immunity, n=4(4.4%) with Combined immunodeficiency and n=1(1.1%) with Leucocyte Adhesion Deficiency type1. Majority of cases with BCGosis (88%) had an underlying IEI. This study strongly highlights the need for evaluation of patients with BCG complications for underlying IEI. While disseminated BCGosis strongly predicts underlying IEI, even localized persistent adenitis may be a warning sign of underlying IEI. It is also strongly recommended to record a family history of previous sibling death prior to administration of this live vaccine and deferring live vaccine till the diagnosis of IEI is ruled out in cases with a positive family history.

Sibling deaths due to infections

Sibling deaths due to infections

Uzamış nöbet ve status epileptikus için risk faktörleri: tek merkez deneyimi

Objectives: To analyze the clinical features of prolonged seizures and status epilepticus and perform risk analysis on super refractory status epilepticus (SRSE) in pediatric intensive care unit (PICU) admissions. Method: Demographic features, underlying etiologies, treatment modalities, electroencephalographic and neuroimaging outcome of intensive care unit admissions between 2015 and 2019 were analyzed. Results: Seventy-one children were enrolled. The common etiology for prolonged seizure was fever in 45.1% of the children (central nervous system infection: 16.9%, infection other than central nervous system: 28.2%), withdrawal of the antiepileptic medication in 40.9%, intoxications in 12.7% of the children and intracranial hemorrhage due to arteriovenous malformation in one (1.4%) patient. Initial diagnostics exhibited 23.9% of hypoglycemia, 18.3% of hypocalcemia, 15.5% of hyponatremia, 35.2% of acidosis, and 25.4% of elevated lactate. Group categorizations were based on fever and new onset of seizure. Approximately 45.1% of the population acquired fever at the onset and consisted of younger children compared to their counterparts (p=0.023). Children with pre-existing epilepsy had frequent history of sibling death, while patients with new-onset of seizure possessed significant lactate elevations, acidosis, and required mechanical ventilation more often (p=0.002, p=0.008, p=0.017). Twelve (16.9%) patients developed SRSE. Low serum sodium and calcium levels were associated with developing SRSE (OR: 10.800, 95%CI: 2.518-46.318; OR: 4.554, 95%CI: 1.159-17.892); however PRISM-3 score has been identified the single independent risk factor in children proceeding to SRSE (OR: 1.174, 95% CI: 1.039-1.327). Three (4.2%) children died of secondary complications. Tracheostomy cannulation was performed in two (16.7%) SRSE patients due to neurological incapability to maintain a patent airway. Conclusion: PRISM-3 score is the independent risk factor of SRSE. Electrolyte abnormalities (hyponatremia and hypocalcemia) are associated with developing SRSE.

A child's grief after the death of a sibling – a review of selected theoretical and empirical aspects

The aim of the article is to review selected theoretical and empirical aspects concerning the death of a sibling as well as its psychological and social consequences for a living child. This topic has not been of interest to researchers so far. This fact may pose an obstacle in searching for effective forms of social and educational support for families who have been orphaned in this way, especially for children. The article illustrates the importance of selected factors that determine the grief experience of children in connection with the loss of a sibling, such as: gender and age, type and cause of sibling death, features of the family environment. Considering the practical implications, it may be emphasized that the ability of the living child to cope with the loss may depend on the willingness of parents to discuss the death of a sibling. Moreover, in order to plan the care and educational impact, gender, age, type and cause of sibling death should be taken into account.

A case study on various disorders and defects present in congenital myasthenia syndrome

The Congenital Myasthenia Syndromes (CMS) are a different gathering of problems that have a hidden deformity in the transmission of signs from nerve cells to muscles. These problems are described by muscle shortcoming, which is declined upon effort. The time of beginning, seriousness of introducing indications and dissemination of muscle shortcoming can shift starting with one patient then onto the next. The synapse, acetylcholine, or ACh for short that goes about as a compound 'courier' with guidelines for the muscles to contract. A three years old child female patient was brought to our department with the complaints of drooping of the left eyelid after one week she developed drooping of right eye. With scientific and laboratory discoveries, she is identified by congenital myasthenia and treatment was started. Evidence from case notes, history, review and accept. Muscle fatigue habits included limb, trunk, bulbar, respiratory, nasal, extraocular muscles and patients reacted with anticholinesterase and 3,4-diaminopyridine. Quick channel syndrome compared with AChR in serious respiratory emergencies in infancy or early childhood. Two children's fatalities, also in care and family history of sibling deaths, highlight the need for effective genetic diagnosis.

Sibling Death in the Family and Dementia Risk

Growing evidence points to the role of stress in contributing to dementia risk, and experiencing the death of a family member is a particularly stressful life event. Sibling relationships are typically life-long relationships and the death of a sibling is likely to be a stressful event in the life course; however, there is little research illuminating the possible consequences of sibling loss for dementia risk. This study considers whether experiencing the death of a sibling before midlife is associated with subsequent dementia risk and how such losses, which are more common for Black and Hispanic than for White populations, may add to racial/ethnic disparities in dementia risk. We use discrete-time event history models to predict dementia incidence among 9,590 non-Hispanic white, 1,669 non-Hispanic black, and 1,109 Hispanic respondents from the Health and Retirement Study, 2000-2014. Losing a sibling during the observation period is associated with increased risk for later dementia. The death of a sibling is robust to the inclusion of a variety of biosocial factors that contribute to subsequent dementia risk. The death of a sibling is a life course event with consequences that appear to increase dementia risk for Black and Hispanic older adults, and this increased risk is explained by biosocial processes likely activated by bereavement. However, Black and Hispanic Americans are further disadvantaged in that they are more likely than White Americans to experience the death of a sibling, and such losses add to the already substantial racial disadvantage in dementia risk.