Long-term health-related quality of life and psychological adjustment in children after haemolytic-uraemic syndrome.

BACKGROUND: In children after haemolytic-uraemic syndrome (HUS), little is known about long-term health-related quality of life (HRQoL) and psychological adjustment as defined by behavioural problems, depressive symptoms and post-traumatic stress symptoms. METHODS: Sixty-two paediatric patients with a history of HUS were included in this study. Medical data of the acute HUS episode were retrieved retrospectively from hospital records. Data on the clinical course at study investigation were assessed by clinical examination and laboratory evaluation. HRQoL and psychological adjustment data were measured by standardised, parent- and self-reported questionnaires. RESULTS: Haemolytic-uraemic syndrome was diagnosed at a mean of 6.5 years before the initiation of the study (standard deviation 2.9, range 0.1-15.7) years. Among the preschool children, parents reported that their child was less lively and energetic (HRQoL emotional dimension), while no increased behavioural problems were reported. In the school-age children, self- and proxy-reported HRQoL was well within or even above the norms, while increased total behavioural problems were found. The school-age children reported no increased depression scores. Also none of the children met the criteria for full or partial HUS-associated posttraumatic stress disorder. CONCLUSIONS: Healthcare providers should be particularly alert to behavioural problems in school-age children with a history of HUS and to lower HRQoL in preschool children.

Health-related quality of life and mental health in parents of children with hemolytic uremic syndrome.

BACKGROUND: Little is known about health-related quality of life (HRQoL) and mental health of parents having children with a history of hemolytic uremic syndrome (HUS). METHODS: This study included 63 mothers and 58 fathers of a cohort of 63 HUS-affected children. At assessment, the mean time since a child experienced an acute episode of HUS was 6.4 years. Parental HRQoL, mental health and posttraumatic stress disorder (PTSD) were assessed with standardized self-report questionnaires. Medical data were extracted from patients' hospital records. RESULTS: The HRQoL and mental health of both the mothers and fathers were not impaired compared to normative data. However, a shorter time since a child's acute HUS episode was a significant predictor of lower HRQoL among the mothers, while no such effect was found among the fathers. Two fathers (3%), but no mothers, met the criteria for a diagnosis of HUS-related full PTSD; one father (2%) and four mothers (6%) met the criteria for a diagnosis of HUS-related partial PTSD. CONCLUSIONS: Our study shows that most parents of our study sample were doing well in terms of HRQoL and mental health, although a small number met the criteria for full or partial PTSD diagnosis due to their child's HUS. We therefore recommend that healthcare providers pay special attention to parents regarding PTSD symptoms during the clinical follow-up of a HUS-affected child since some parents may benefit from psychological support.

Psychological outcome, fatigue, and quality of life after infection with shiga toxin-producing Escherichia coli O104.

BACKGROUND & AIMS: From May through July 2011 in northern Germany, there was a large outbreak of hemolytic uremic syndrome and bloody diarrhea, which was related to infections from Shiga toxin-producing Escherichia coli O104 (STEC). We investigated the depression, posttraumatic symptoms, fatigue, and health-related quality of life among patients within the first 6 months after STEC infection and aimed to identify factors associated with poor outcome. METHODS: In a cohort study, we performed baseline assessments of 389 patients (69% female) 3 months after STEC infection (82 ± 36 days) and follow-up assessments of 308 of the patients 6 months afterward (199 ± 17 days). Data were collected at 13 hospitals in northern Germany. Patients completed validated self-report scales and a diagnostic interview. RESULTS: At baseline, hemolytic uremic syndrome was diagnosed in 31% of the patients. Six months after the infection, mean self-reported severity of depression and posttraumatic symptoms and fatigue were significantly greater than in the general population, and the mean score from the mental component of health-related quality of life survey was significantly lower than average. Posttraumatic stress disorder had recently developed in 3% of patients (95% confidence interval, 1%-5%), and 43% of patients had clinically relevant fatigue (95% confidence interval, 41%-45%). The most important baseline factors associated with poor psychological health 6 months after STEC infection were previous traumatic events, neuroticism, and low social support (all P < .05). CONCLUSIONS: Six months after the major outbreak of STEC infection in northern Germany, a substantial number of patients had poor psychological health, persistent fatigue, and impaired quality of life. For future outbreaks, patients' premorbid risk factors should be considered, which might minimize the long-term effects of infections on mental health.

Long-term outcomes of Shiga toxin hemolytic uremic syndrome.

Shiga toxin-producing Escherichia coli (STEC) hemolytic uremic syndrome (HUS) is an important cause of acute kidney injury (AKI). The outcomes of STEC HUS have improved, and the acute mortality rate in children is 1-4%. About 70% of patients recover completely from the acute episode and the remainder have varying degrees of sequelae. Only a few retrospective studies have reviewed these patients over long periods. Methodological flaws include a lack of strict definitions, changing modes of treatment, ascertainment bias and loss of subjects to follow-up. The kidneys bear the brunt of the long-term damage: proteinuria (15-30% of cases); hypertension (5-15%); chronic kidney disease (CKD; 9-18%); and end-stage kidney disease (ESKD; 3%). A smaller number have extra-renal sequelae: colonic strictures, cholelithiasis, diabetes mellitus or brain injury. Most renal sequelae are minor abnormalities, such as treatable hypertension and/or variable proteinuria. Most of the patients who progress to ESKD do not recover normal renal function after the acute episode. Length of anuria (more than 10 days) and prolonged dialysis are the most important risk factors for a poor acute and long-term renal outcome. After the acute episode all patients must be followed for at least 5 years, and severely affected patients should be followed indefinitely if there is proteinuria, hypertension or a reduced glomerular filtration rate (GFR).

Emotional and behavioral changes in parents of children affected by hemolytic-uremic syndrome associated with verocytotoxin-producing Escherichia coli: a qualitative analysis.

BACKGROUND: The long-term clinical outcome for children affected by hemolytic uremic syndrome associated with verocytotoxin-producing Escherichia coli (VTEC-HUS) is well documented, but the parental experience is not. OBJECTIVE: The authors investigated the effects of the critical-care hospitalization for this condition on well-being of patients' families. METHOD: A group of 30 parents completed a free-response format survey when their child presented to the hospital; 19 of this cohort completed a 1-year follow-up. RESULTS: Content analysis demonstrated that this cohort of parents experienced long-term emotional distress and substantive disruption to family and daily life. DISCUSSION: These results corroborate anecdotal clinical observations. The authors suggest future research initiatives and best practices to reduce parental distress.

[Life-experience workshops with hemolytic uremic syndrome patients relatives. Towards an integral approach for HUS treatment]. / Talleres vivenciales con familiares de pacientes de síndrome urémico hemolítico. Hacia un abordaje integral de su tratamiento.

This article describes a group technique designed to assist (affective and contention needs from) relatives of children who have suffered HUS. It consists on group meetings, called Life-Experience Workshops, where central topics considered important by the participants are discussed. These group meetings with HUS patient's relatives have no reported precedents and permit analyzing the traumatic value of life-experiences during and after the disease acute phase and its impact on the family involved. As a conclusion, it is considered that this first step opens possibilities to continue working and searching in this field, unexplored so far. A contribution to an integral approach for the child and his or her family is pursued, which complements medical treatment with a psychological and social perspective.

Tallheres vivenciales con familiares de pacientes de Síndrome Urémico Hemolítico: hacia un abordaje integral de su tratamiento / Life-experience workshops with hemolytic uremic syndrome patients relatives. Towards an integral approach for HUS treatment

This article describes a group technique designed to assist (affective and contention needs from) relatives of children who have suffered HUS. It consists on group meetings, called Life-Experience Workshops, where central topics considered important by the participants are discussed. These group meetings with HUS patients relatives have no reported precedents and permit analyzing the traumatic value of life-experiences during and after the disease acute phase and its impact on the family involved. As a conclusion, it is considered that this first step opens possibilities to continue working and searching in this field, unexplored so far. A contribution to an integral approach for the child and his or her family is pursued, which complements medical treatment with a psychological and social perspective.(AU)

Tallheres vivenciales con familiares de pacientes de Síndrome Urémico Hemolítico: hacia un abordaje integral de su tratamiento / Life-experience workshops with hemolytic uremic syndrome patients relatives. Towards an integral approach for HUS treatment

This article describes a group technique designed to assist (affective and contention needs from) relatives of children who have suffered HUS. It consists on group meetings, called Life-Experience Workshops, where central topics considered important by the participants are discussed. These group meetings with HUS patient's relatives have no reported precedents and permit analyzing the traumatic value of life-experiences during and after the disease acute phase and its impact on the family involved. As a conclusion, it is considered that this first step opens possibilities to continue working and searching in this field, unexplored so far. A contribution to an integral approach for the child and his or her family is pursued, which complements medical treatment with a psychological and social perspective.

A support group for patients who have recovered from thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS): The six-year experience of the Oklahoma TTP-HUS Study Group.

A support group for patients who have recovered from thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS), named The Oklahoma TTP-HUS Study Group, has been a successful program for 6 years. This group has met 3 times each year with an average attendance of 16 former patients; in addition, an average of 14 family members and friends have attended each meeting. Eighty-four percent of patients who attended a meeting were women as compared to 68% women among those who did not attend (P = 0.016). Twenty-three percent of patients who attended a meeting have relapsed as compared to 8% among those who did not attend (P = 0.006). There was also a difference in the rates of attendance among the different clinical categories of patients (P < 0.001). A survey of former patients, as well as their families and friends, documented that interest in learning more about TTP-HUS and talking with other people who have had TTP-HUS were principal motivations for attending the meetings. The meetings of The Oklahoma TTP-HUS Study Group have been successful not only for support of former patients but also for research. Long-term patient follow-up has been facilitated and the group discussions have revealed previously unreported persistent problems with cognitive ability and endurance. A survey of member centers of the American Association of Blood Banks and America's Blood Centers revealed no similar programs for patients who have recovered from TTP-HUS. The absence of a support group for TTP-HUS in the national survey contrasts to the 274 patient support groups and related wellness/education classes, including some for rare disorders, currently active in the Oklahoma City metropolitan area. The experience of The Oklahoma TTP-HUS Study Group suggests that it serves a previously unmet need.

Thrombotic stroke in a child with diarrhea-associated hemolytic-uremic syndrome with a good recovery.

A boy aged 3.5 years with post-diarrheal hemolytic-uremic syndrome (HUS) was referred to our hospital because of convulsion and stupor. He had been admitted to a regional hospital with a 3-day history of bloody diarrhea, colic abdominal pain and fever. Two days later, he had complained of generalized seizures and oliguria. On admission, he developed anuria, and serum blood nitrogen and creatinine increased to 56 mg/100 ml and 2.8 mg/100 ml, respectively. Platelets decreased to 42,000/microl. Under the diagnosis of HUS, a continuous hemodiafiltration treatment had to be instituted. Computed tomography of his head at hospital day 5 revealed abnormal low density area of infarction with edema in both the basal ganglia involving with the posterior limb of internal capsule. Serum titer of IgM antibody to Escherichia coli O157 showed positive value. Although his anuria and stupor persisted over 10 days, he recovered without serious complications. These clinical observations may indicate that patients with similar lesions do not necessarily have serious morbidity.

Neuropsychological sequelae of haemolytic uraemic syndrome. Investigators of the HUS Cognitive Study.

BACKGROUND: Severe haemolytic uraemic syndrome (HUS) in childhood can cause stroke, hemiplegia, cortical blindness, and psychomotor retardation. These outcomes are evident at the time of discharge immediately after the acute illness. Less is known about the neuropsychological outcomes of less severely affected children who recover from acute HUS. AIMS: This multicentre case control study investigated the hypothesis that children who survive an acute episode of HUS without recognizable neurological injuries have greater impairment of cognitive, academic, and behavioural functions than controls. DESIGN: Children with HUS were eligible if they had no evidence of severe neurological dysfunction when discharged from one of six Canadian hospitals. Controls had been admitted to hospital for a non-HUS illness and were matched by age, sex, first language, and socioeconomic status. All subjects underwent evaluation of behaviour, academic achievement, cognitive function, and verbal abilities using standardised tests administered by a psychometrist blinded to the case or control status. RESULTS: Ninety-one case control pairs were enrolled. No important differences between patients with HUS and paired controls were evident on tests of IQ, behaviour, verbal abilities, or academic achievement. There was no increased risk of attention deficit disorder among patients with HUS. There was no correlation between the severity of acute renal failure and neuropsychological measures, although scores on some verbal ability tests were lower in those with the highest serum creatinine concentrations during illness. CONCLUSIONS: Children discharged from hospital without apparent neurological injury after an episode of acute HUS do not have an increased risk of subclinical problems with learning, behaviour, or attention.

Sequelae of haemolytic uraemic syndrome.

Twenty two patients with previous episodes of haemolytic uraemic syndrome (HUS) were investigated for evidence of deficits in cognitive, behavioural, and academic function. Patients were pair matched with 22 controls for age (+/- 1 year), gender, and socioeconomic status. HUS patients had numerically lower cognitive and achievement scores and higher behavioural problem ratings than their controls on every measure. None of the group differences was significant at the 0.01 level. Significance values between 0.10 and 0.01 were obtained for the Wechsler full scale and verbal intelligence quotient scores and for several of the achievement measures and behaviour ratings. These results were conservatively interpreted as trends and are considered to provide preliminary indications of a post-HUS deficit in behaviour, verbal intelligence, and the verbally based skills of reading comprehension and vocabulary. The findings provide interim guidelines for follow up care but require confirmation and elaboration in a larger study.