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Sudden death in a 21-year-old man caused by thrombosed coronary aneurysm: late sequelae or a very late onset of Kawasaki disease?

Kawasaki disease (KD; mucocutaneous lymph node syndrome) is an infantile febrile illness of unknown origin that occurs in worldwide epidemics. The main clinical features of the disease, such as fever, mucositis, cervical lymphoadenopathy and skin rash, associated with coronary aneurysms or arteritis are diagnostic. Late sequelae of coronary arteritis are very rare in the second decade of life; we describe the case of a 21-year-old male who died suddenly. The autopsy revealed a large thrombotic coronary aneurysm, coronary arteritis and myocarditis, supporting the diagnosis of a late fatal sequela of KD. The hypothesis of a very late onset of the disease is also considered and discussed.

Adult↗

Preliminary evidence of behavioral and cognitive sequelae of obstructive sleep apnea in children.

OBJECTIVES: To characterize the daytime sequelae of obstructive sleep apnea (OSA) in children. BACKGROUND: OSA syndrome is a common disorder in children with estimates of prevalence ranging from 1.1 to 2.9%. Numerous studies have documented neuropsychological deficits in adults with OSA, although only a few preliminary studies have described these problems in children with OSA. METHODS: In the present study, otherwise healthy children with OSA (n=28), and a healthy age-matched comparison group (n=10) were assessed with standard measures of sleep, behavior, and cognitive function. RESULTS: Children with OSA had significantly more behavior problems than the healthy comparison group based on parents' reports (F=7.29, P<0.005). Children diagnosed with moderate to severe OSA had significantly lower scores on a timed cancellation task that assesses sustained attention (F=10.0, P<0.01). A significant association was found between OSA severity (rho=-86, P<0.01) and measures of verbal ability. CONCLUSIONS: These initial findings suggest that there are identifiable daytime sequelae of childhood OSA and that it is important to evaluate these daytime disturbances in making determinations about intervention.

Journal Article↗

Late neurocognitive sequelae in survivors of brain tumours in childhood.

As survival among children treated for cancer continues to improve, more attention is being focussed on the late effects of cancer treatment. In children treated for brain tumours, chronic neurocognitive effects are especially challenging. Deficits in cognitive development have been described most thoroughly among children treated for posterior-fossa tumours, specifically medulloblastomas and ependymomas, which account for about 30% of all newly diagnosed cases of brain tumours in children. Most children who have survived brain tumours have required surgical resection and focal or craniospinal radiotherapy (irradiation of the entire subarachnoid volume of the brain and spine), with or without systemic chemotherapy. Historically, intelligence quotient (IQ) scores have provided a benchmark against which to measure changes in cognitive development after treatment. Observed declines in IQ are most likely a result of failure to learn at a rate that is appropriate for the age of the child, rather than from a loss of previously acquired knowledge. The rate of IQ decline is associated with a several risk factors, including younger age at time of treatment, longer time since treatment, female sex, as well as clinical variables such as hydrocephalus, use of radiotherapy and radiotherapy dose, and the volume of the brain that received treatment. Loss of cerebral white matter and failure to develop white matter at a rate appropriate to the developmental stage of the child could partly account for changes in IQ score. Technical advances in radiotherapy hold promise for lowering the frequency of neurocognitive sequelae. Further efforts to limit neurocognitive sequelae have included design of clinical trials to test the effectiveness of cognitive, behavioural, and pharmacological interventions.

Antineoplastic Agents↗

Severe sequelae after stonefish envenomation.

More tourists are visiting the idyllic waters of the South Pacific each year. They share the shallow waters with the native wildlife, including the venomous stonefish. Although the injection of its venom has been reported to have occasional fatal neuromuscular and cardiovascular sequelae, severe tissue necrosis at the injection site has not been described. We present a case of stonefish injury that describes serious localised sequelae of stonefish envenomation

Amputation, Surgical↗

Acute viral bronchiolitis and its sequelae in developing countries.

Acute viral bronchiolitis (AVB) is a common disease found throughout the world. Various aspects of it are being studied: its epidemiology, diagnosis, prognosis and treatment. Most of these studies are being conducted in developed countries, with only a few taking place in developing countries. Risk factors such as poor nutrition, an adverse environment and early weaning should be studied where these features are common. Treatment aspects such as cost-effectiveness in low income settings need further study. Use of ribavirin and respiratory syncytial virus (RSV)-immunoglobulin are good examples. Post-bronchiolitic sequelae also need to be studied in low income countries. There is evidence that bronchiolitis obliterans is unusually frequent in some Latin-American countries such as Argentina and Brazil. It will be helpful to undertake combined studies in countries with the same socio-economics, investigating the preventive and management aspects of AVB and its sequelae to reduce the morbidity and mortality.

Acute Disease↗

Management of the late ocular sequelae of Stevens-Johnson syndrome.

The acute conjunctivitis seen initially in Stevens-Johnson syndrome is followed by a cicatricial phase, which often leads to severe ocular surface disease and visual morbidity. Manifestations include keratinization of the conjunctiva, lid margins, and lacrimal and meibomian ducts, resulting in an unstable tear film and mechanical trauma to the conjunctiva and cornea with blinking. Limbal stem cell deficiency is the most vision-threatening sequela of Stevens-Johnson syndrome, as it causes corneal neovascularization, chronic corneal inflammation, and an irregular corneal epithelium. Management of late sequelae often requires a multipronged approach, including strategies for ocular surface protection, ocular surface support, and ocular surface reconstruction. In this review, established therapies, as well as new experimental therapies, are discussed.

Journal Article↗

[Severe hypernatremia: survival without neurologic sequelae].

Hypernatremia is an electrolyte disturbance most frequently caused by excess water loss and less frequently by increased sodium intake. The few reported cases of severe hypernatremia (> 190 mEq/l) had an adverse outcome with high mortality and/or severe neurologic sequelae. The first case was a 7-year-old girl with renal failure undergoing continuous venovenous hemodiafiltration therapy who presented hypernatremia (216 mEq/l) after incorrect preparation of dialysis fluid. The patient was treated with hemodiafiltration and hypernatremia was resolved in 48 hours. She had a convulsive crisis without subsequent neurologic impairment. The second patient, a 3-year-old girl with pseudohypoaldosteronism type I and encephalopathy, had hypernatremia (203 mEq/l) due to erroneous sodium administration, which was corrected in 36 hours with intravenous fluid therapy. Her neurologic status was unchanged by treatment. We conclude that children with extreme hypernatremia survive without neurologic sequelae if treatment achieves a progressive decrease of natremia.

Child↗

Health-related quality of life and sequelae in patients treated with brachytherapy and external beam irradiation for localized prostate cancer.

PURPOSE: To evaluate late physical and psychosocial sequelae in patients treated with an association of external beam irradiation (EBI) and brachytherapy (BT) for localized prostate cancer. PATIENTS AND METHODS: Seventy-one patients free of disease, treated at the Centre François Baclesse from 1988 to 1992, were enrolled in a case-control study. Seventy-one healthy controls, matched on age and residence, were selected at random from electoral rolls. Two self-administered questionnaires were mailed in January 1996. The French translation of the Nottingham Health Profile questionnaire and the European Organization for Research and Treatment of Cancer (EORTC) QLQ-C30 core questionnaire were used to evaluate physical, role, emotional, cognitive and social functioning, global health status as well as energy and sleep disturbance. Specific problems related to prostate cancer were explored using the prostate specific module developed by the EORTC Genito-Urinary Tract Cancer Cooperative Group. Concordance between clinical complications reported by patients and those reported by physicians was also analyzed. RESULTS: General health quality of life scale scores did not significantly differ between patients and controls, nor did general symptom scale scores. Furthermore, no more late psychosocial sequelae were reported by patients than by controls. No major digestive complications were observed among patients. However, statistical differences were observed concerning interest in sex (P = 0.016) and sexual activity (P < 0.001), urinary incontinence (P < 0.001) and cystitis (P = 0.01). Late subjective morbidity (dysuria, nocturia, urinary incontinence, pelvic pain) appraisal differed slightly between patients and physicians who generally underestimate its severity. While nocturia was reported more often by physicians than by patients (P = 0.0016), patients reported urinary incontinence and pelvic pain more often than physicians (P < 0.001 and P < 0.001, respectively). CONCLUSIONS: The study demonstrates that survivors from localized prostate cancer treated with an association of BT and EBI have good global health status. Major problems that persist are sexual disorders, urinary incontinence and cystitis while digestive disorders were rare. This association could be an alternative to standard EBI in patients with localized prostate cancer. Whatever the treatment choice, patients should be involved in the therapeutic decision which should consider not only expected survival rate but also quality of life.

Aged↗

[Motor, cognitive and socio-emotional development of 11-year-olds with early childhood risk factors: late sequelae].

OBJECTIVES: The investigation of the impact of early childhood risk factors on developmental outcome at the age of 11 years. METHODS: The onset and course of developmental and behavioral disturbances were examined in a prospective longitudinal study of a sample of 362 children born with different risks. Organic (obstetric complications) and psychosocial risks (family adversity) were varied in a two-factorial design. Measures of motor, cognitive and social-emotional outcome were obtained from 341 children aged 11 years (168 boys, 173 girls, corresponding to 94.2% of the initial sample). Previous assessments had been conducted at the ages of 3 months, and again at the ages of 2, 4 1/2 and 8 years. RESULTS: The negative impact of early risk factors persisted into late childhood. Rates of developmental and behavioral disturbances in high-risk children were up to three times higher than in non-risk children. Both organic and psychosocial risks contributed to adverse outcomes. While organic complications were related to disturbances in motor and cognitive development, the detrimental effects of psychosocial adversity pertained to social-emotional functioning. Late sequelae of pre- and perinatal complications were found especially in cognitive outcome and school performance. The cumulative effect of early risks was best explained by summing up the single risk effects. CONCLUSIONS: Early risk factors have specific and long-term sequelae resulting in adverse school outcomes at later ages.

Affective Symptoms↗

The role of bacteriolysis in the pathophysiology of inflammation, infection and post-infectious sequelae.

The literature dealing with the biochemical basis of bacteriolysis and its role in inflammation, infection and in post-infectious sequelae is reviewed and discussed. Bacteriolysis is an event that may occur when normal microbial multiplication is altered due to an uncontrolled activation of a series of autolytic cell-wall breaking enzymes (muramidases). While a low-level bacteriolysis sometimes occurs physiologically, due to "mistakes" in cell separation, a pronounced cell wall breakdown may occur following bacteriolysis induced either by beta-lactam antibiotics or by a large variety of bacteriolysis-inducing cationic peptides. These include spermine, spermidine, bactericidal peptides defensins, bacterial permeability increasing peptides from neutrophils, cationic proteins from eosinophils, lysozyme, myeloperoxidase, lactoferrin, the highly cationic proteinases elastase and cathepsins, PLA2, and certain synthetic polyamino acids. The cationic agents probably function by deregulating lipoteichoic acid (LTA) in Gram-positive bacteria and phospholipids in Gram-negative bacteria, the presumed regulators of the autolytic enzyme systems (muramidases). When bacteriolysis occurs in vivo, cell-wall- and -membrane-associated lipopolysaccharide (LPS (endotoxin)), lipoteichoic acid (LTA) and peptidoglycan (PPG), are released. These highly phlogistic agents can act on macrophages, either individually or in synergy, to induce the generation and release of reactive oxygen and nitrogen species, cytotoxic cytokines, hydrolases, proteinases, and also to activate the coagulation and complement cascades. All these agents and processes are involved in the pathophysiology of septic shock and multiple organ failure resulting from severe microbial infections. Bacteriolysis induced in in vitro models, either by polycations or by beta-lactams, could be effectively inhibited by sulfated polysaccharides, by D-amino acids as well as by certain anti-bacteriolytic antibiotics. However, within phagocytic cells in inflammatory sites, bacteriolysis tends to be strongly inhibited presumably due to the inactivation by oxidants and proteinases of the bacterial muramidases. This might results in a long persistence of non-biodegradable cell-wall components causing granulomatous inflammation. However, persistence of microbial cell walls in vivo may also boost innate immunity against infections and against tumor-cell proliferation. Therapeutic strategies to cope with the deleterious effects of bacteriolysis in vivo include combinations of autolysin inhibitors with combinations of certain anti-inflammatory agents. These might inhibit the synergistic tissue- and- organ-damaging "cross talks" which lead to septic shock and to additional post-infectious sequelae.

Animals↗

Attachment theory as a framework for understanding sequelae of severe adolescent psychopathology: an 11-year follow-up study.

This study examined long-term sequelae of severe adolescent psychopathology from the perspective of adult attachment theory. The study compared 66 upper-middle-class adolescents who were psychiatrically hospitalized at age 14 for problems other than thought or organic disorders, to 76 sociodemographically similar high school students. When reinterviewed at age 25, virtually all of the previously hospitalized adolescents displayed insecure attachment organizations, in contrast to a more typical mixture of security and insecurity in the former high school sample. Lack of resolution of previous trauma with attachment figures accounted for much of this insecurity. Insecurity in adult attachment organization at age 25 was also linked to self-reported criminal behavior and use of hard drugs in young adulthood. These findings are discussed as reflecting a substantial and enduring connection between attachment organization and severe adolescent psychopathology and a possible role of attachment organization in mediating some of the long-term sequelae of such psychopathology.

Adolescent↗

Ocular sequelae of pneumatic drills.

PURPOSE: To determine the ocular sequelae of rock-drilling. METHODS: Nineteen rock-drillers and 20 age-, sex- and race-matched control subjects underwent ocular examination in a prospective manner. RESULTS: Subepithelial corneal opacities were found in 12 drillers (63%) and none of the controls; pigmented trabecular meshwork was noted in 13 drillers (68%) and 1 control (5%); low-tension glaucoma was diagnosed in 1 driller (5%); vitreous liquefaction was present in 17 of 17 drillers (100%) examined by a retina specialist and 9 of 20 controls (45%). CONCLUSIONS: The sequelae of drilling include corneal scars from projectile pieces of drilled stone as well as vibration-induced pigment deposition in the trabecular meshwork and vitreous liquefaction. Wearing of safety glasses is recommended.

Adult↗

Absence of renal sequelae after childhood Escherichia coli O157:H7 gastroenteritis.

Although a quarter of children who survive diarrhea-associated hemolytic uremic syndrome develop long-term renal sequelae, the prognosis of acute, self-limited Escherichia coli O157:H7 gastroenteritis has never been previously studied. Four years after a drinking water outbreak of E. coli O157:H7, we examined the risk of high blood pressure (>95th percentile expected for age, sex, and height), reduced kidney function, and microalbuminuria among previously healthy children and adolescents. Of the 951 participants, 313 were asymptomatic during the outbreak, 305 had moderate symptoms of acute gastroenteritis, and 333 had severe symptoms that necessitated medical attention. An additional 23 children who developed hemolytic uremic syndrome during the outbreak were excluded from this analysis. There were no differences in mean systolic blood pressure between those who had no, moderate, or severe symptoms of acute gastroenteritis during the outbreak (109, 110, and 107 mm Hg). Similarly, there were no group differences in diastolic blood pressure, estimated glomerular filtration rate, or random urine albumin to creatinine ratio (P ranged from 0.14 to 0.52), or in the adjusted relative risk of high blood pressure, a glomerular filtration rate <80 ml/min per 1.73 m(2), or microalbuminuria (P ranged from 0.23 to 0.89). Patients who presented to medical attention with gastroenteritis during this E. coli O157:H7 outbreak had an absence of renal sequelae 4 years later. With no existing data to support screening after self-limited E. coli O157:H7 gastroenteritis, we recommend that only those children who develop recognized features of hemolytic uremic syndrome be followed for long-term renal health.

Albuminuria↗

Torture and its neurological sequelae.

BACKGROUND: Refugees and asylum seekers continue to enter the United States and the European Union in record numbers. Some have estimated that between 5-35% of all refugees have suffered torture in their countries of origin. Although general practitioners and specialized physicians are likely to encounter victims of torture as patients, few providers are familiar with the health problems that may affect this patient population. PURPOSE: :To provide neurologists, neurosurgeons, and rehabilitation medicine physicians with basic knowledge about survivors of torture that can help in the diagnosis, treatment, and referral of such patients. METHODS: A MEDLINE (1966-October 2001) search using keywords torture and sequelae (nervous system diseases and brain injuries) was conducted. Other data sources included books, reference lists, online resources and expert opinion. FINDINGS: :Forms of torture that may affect the nervous system include beatings, gunshot wounds, stab wounds, asphyxiation, prolonged suspension and electrocution. Victims of torture commonly experience neurological symptoms such as headaches, vertigo, loss of consciousness and dizziness during and after torture. A successful and meaningful clinical interaction with a survivor of torture includes avoiding retraumatization, building trust, spelling out any limits on confidentiality, and above anything else, establishing empathy with the patient. CONCLUSIONS: Neurological sequelae of torture can be devastating physically and psychologically. The treatment of these neurological conditions does not differ from other patient populations. However, the clinical approach is unique and must focus on avoiding retraumatization and helping the victim reintegrate into society as quickly as possible.

Comorbidity↗

Neurological long-term sequelae after spinal anaesthesia in a tropical setting: a case control study.

Spinal anaesthesia (SA) is an important form of anaesthesia in tropical countries. It is considered to have few long-term complications or sequelae, although this hypothesis has not been proven in a rural tropical setting. In a case control study we found SA to he a significant risk factor for lower back pain, reflex abnormalities and muscular atrophy and mild impairment of muscle power in patients examined between 3 months and 4 years after SA. These long-term sequelae need to be confirmed in a larger prospective study employing all possible neurophysiological and neuroimaging techniques.

Adult↗

Sources and sequelae of bacterial contamination of hematopoietic stem cell components: implications for the safety of hematotherapy and graft engineering.

BACKGROUND: It is important to compare the incidence of bacterial contamination of components collected from the peripheral blood or bone marrow (BM), as well as of components processed with or without cell selection or depletion, and to evaluate the sequelae of such contamination. STUDY DESIGN AND METHODS: Bacterial contamination rates were compared in 1380 untreated autologous peripheral blood progenitor cells (PBPCs), 291 untreated autologous BM samples, 916 monoclonal antibody (MoAb)-treated autologous and allogeneic BM samples, and in 45 autologous PBPC components from which the CD34+ cells were selected. Bacterial cultures were performed at sequential time points during the processing of MoAb-treated BM. RESULTS: Bacterial contamination was documented in 44 of 2632 components from 1593 patients (1.67% of components, 2.76% of patients) before cryopreservation. Although only 0.65% of untreated PBPCs were contaminated before cryopreservation, each patient was more likely to have given a contaminated PBPC component than a contaminated BM component (2.41% vs. 0%, p < 0.01). Bacterial contamination of MoAb-treated BM was greater during or after manipulation than it was before (2.33% vs. 0.77%, p < 0.05). At thawing, contamination was documented in 42 (1.97%) of 2136 components cultured. Ten (13.7%) of 73 patients who received hematopoietic progenitor cells that were contaminated before cryopreservation or at thawing developed fever or positive blood cultures within 48 hours of transfusion. Fever was associated with bacteremia in two cases, but no irreversible clinical sequelae were noted. CONCLUSION: These studies suggest that, despite careful attention to sterile procedures, low-level contamination of hematopoietic stem cell components can be introduced before or during manipulation as well as at thawing, and that standards for monitoring of the procedures for collection, processing, cryopreservation, thawing, and transfusion of hematopoietic progenitor cells are necessary.

Antibodies, Monoclonal↗

Neurocognitive sequelae in the treatment of low-grade gliomas.

Patients with low-grade gliomas may live without disease progression for many years after initial diagnosis and treatment, but long-term cognitive deficits, which affect up to 90% of patients, can have a sustained negative effect on their daily functioning and quality of life. Cognitive deficits in patients with low-grade gliomas may be caused by the tumor itself or by various other factors including tumor-related epilepsy, tumor treatment (surgery, radiotherapy [RT], medical therapy [including chemotherapy]), psychological stress, or a combination of these. Although some studies suggest a strong link between RT and neurocognitive sequelae, other studies have found little evidence that RT is a major risk factor for cognitive deficits and suggest that other factors may play a major role. The results of a recent cross-sectional study involving 195 patients with low-grade gliomas (104 of whom received RT) strongly suggest that standard focal RT with fractional doses less than 2 Gy is not generally associated with an increased risk of cognitive deficits, but that higher fractional doses are likely to result in cognitive disability. Notably, the presence and severity of epileptic seizures and/or the use of antiepileptic drugs were more strongly associated with cognitive deficits than was RT. This and other studies suggest that disease and treatment factors other than standard focal RT may be more important with respect to increasing the risk of neurocognitive sequelae in patients with low-grade gliomas.

Brain Neoplasms↗

Identifying hypnotic sequelae: the problem of attribution.

A case of apparent hypnotic sequelae occurring in a non-clinical research context (involving a college student) is reported. Following his participation in a group session in which a standard hypnotic susceptibility scale was administered, 19 year-old Michael reported a number of reactions common to the hypnotic sequelae literature including dizziness, a mild headache, trembling, and slight nausea. An interview with Michael revealed several alternative sources of his reaction, highlighting the ever-present problem of attribution.

Adult↗