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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Six selected cases with neonatal and fetal cochlear pathology are presented. Those denoting cochlear sepsis are: H. influenza purulent labyrinthitis, cytomegalovirus endolabyrinthitis, and aseptic meningitis and labyrinthitis. Those denoting cochlear neural aplasia are Goldenhar's syndrome, left-sided cardiac hypoplasia with partial basal turn neural aplasia, and cerebral cortical and ventricular hemorrhage with modiolar extension. These findings are compared to the fluorocitrate ototoxicity model for neural deafness in the guinea pig. This study suggests hypotheses for viral trophism in the inner ear and neural degeneration of the cochlea as mechanisms for sensorineural deafness.
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PMID:Histopathologic and experimental models for sensory and neural deafness. 99 43

Group B Streptococcus is a common cause of neonatal sepsis and meningitis. The purpose of this study was to evaluate the neurologic, psychologic, and academic status of children who had group B streptococcal meningitis and to compare these children with their siblings. Seventy-four children who acquired group B streptococcal meningitis between one day and 6 months of life formed the study population. Survivors were 3 to 18 years old at the time of their follow-up evaluations. Twenty children (27%) died, two were institutionalized, one severely affected child died at age 2 years, 15 were assessed by phone interview, and two were lost to follow-up. Thirty-four children and 21 siblings were comprehensively evaluated with physical and neurologic examinations, hearing tests, and tests of intellectual, perceptual-motor, and behavioral-adaptive functions. Of the total population, nine children (12%) had major neurologic sequelae (spastic quadraplegia, profound mental retardation, hemiparesis, deafness, or blindness). Six children had acute hydrocephalus; two were doing well after shunt placement. In general, those children surviving group B streptococcal meningitis without major sequelae appeared to be functioning normally or comparably to their sibling in intellectual, social, and academic matters.
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PMID:Long-term outcome of group B streptococcal meningitis. 351 45

Life-threatening human sepsis is often treated using bolus intravenous aminoglycoside injections with transient deafness as a reported side effect. Young adult cats were given various high dosages of gentamicin in bolus injections (1 ml in 30 s). Scala vestibuli calcium and gentamicin, blood gentamicin and compound action potentials of the VIII nerve were measured shortly after the injections and 45 min later. An obvious relationship could be demonstrated between levels of gentamicin, decreased calcium content and acoustic thresholds elevation. Even after a high dose of 175 mg/kg that abolished action potentials at t = 2 min, recovery invariably occurred. It is believed that the reported transient hearing loss in humans may be partly attributable to a temporary blockage of calcium by gentamicin.
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PMID:Perilymphatic calcium and VIII nerve action potentials during gentamicin bolus i.v. injections. A preliminary study. 361 67

A retrospective study of oculovisual assessment records of the population of three Ontario schools for deaf children assessed the differential effects of various causes of deafness on the prevalence of vision anomalies. Inherited deafness (ID) appears to be associated with the fewest visual anomalies. Congenital rubella (CR) had the greatest prevalence and was associated with the broadest spectrum of ocular and visual problems. It appears to have a significant effect on corneal curvature as well as being associated with anomalies of other organ systems. Children who had had CR, neonatal sepsis (NS), and Rh incompatibility (Rh) all showed higher rates of strabismus and amblyopia. Those who were deaf from meningitis (MEN) or NS showed a tendency to be more hyperopic, whereas the sample with Rh showed a trend toward myopia. The CR children had the broadest range of spherical refractive errors of all the causes of deafness. Children in schools for the deaf are generally those with substantial or profound hearing loss and thus are more likely to have an accompanying vision impairment. Knowledge of the effect on vision of the causes of deafness should aid practitioners in identifying and detecting similar effects on vision when mild or moderate deafness does not require a child's attendance at schools for the deaf.
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PMID:Differential effects of various causes of deafness on the eyes, refractive errors, and vision of children. 376 97

Clinical records of 181 children, aged between one month and seven years, admitted in a four year period, from 1978 through 1982, with the diagnosis of bacterial meningitis are revised. Peak incidence occurred in the age group between six months and three years, and during the months of January to May. N. meningitidis (35%), pneumococcus (4.9%) and H. influenzae (2.7%) were the most frequently isolated bacteria. CSF culture was negative in 56% of the children. All of them had previously taken antibiotics. Complications were present in 6.4%, with highest incidence in the known-agent group, on the following order: septic shock, 11%, seizures, 6.6%, and subdural effusion, 2.2%. Permanent sequelae were present in 3.8%, being deafness predominant. Twelve (6.3%) out of the 181 died, and death was result of fulminant meningococcal sepsis with endotoxic shock in ten of these patients. Clinical and psychological followed-up of twenty-nine children with isolated causal agent, were compared with a control group, finding no statistically-significant difference.
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PMID:[Bacterial meningitis in children. Analysis of 181 cases]. 650 29

The 6-year follow-up of a patient affected by Wolfram's syndrome, a rare disease characterized by diabetes insipidus (DI), diabetes mellitus (DM), optic atrophy (OA), neurosensory deafness (D), atony of the urinary tract and other abnormalities (DIDMOAD or Wolfram's syndrome), is described. Our patient has diabetes insipidus, diabetes mellitus, abnormal audiograms, without subjective evidence of hearing loss, and dilatation of the urinary tract. Diagnosis was suspected at the age of 8 years. Diabetes mellitus was the first manifestation and treatment with insulin was necessary. Desmopressin therapy decreased dramatically the daily urinary output. In view of the significant morbidity and mortality from renal failure associated with recurrent urinary infections, we have drawn special attention to the urological manifestations of the syndrome. During the follow-up, the patients underwent some investigations, such as renal ultrasound and echotomography and cystourethroscopy. Outstanding results of these studies are severe bilateral hydronephrosis with dilatated ureters and loss of renal tissue. The particular finding is the presence of posterior urethral valves with obstructed bladder. The anatomical outlet obstruction are variable and may be disastrous. There may be failure to thrive, sepsis, anemia be disanal failure. In such instances corrective surgery could improve bladder and ureteral functions.
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PMID:[Wolfram syndrome. Peculiar urologic aspects]. 779 16

Forty-two children who sustained a serum bilirubin (SBR) level above 339 mumol/L as newborn infants were assessed at our Growth and Development Clinic to determine presence of sequelae. Only one child (2.3%) had mild sensorineural deafness and one child (2.3%) performed below age-matched standards on psychological testing. As the SBR level rose the psychological scores were lower. Three infants had sepsis associated with the hyperbilirubinaemia. Two (maximum SBR levels of 371 and 366 mumol/L) children were normal (General Cognitive Index (GCI) 117 and 119, respectively) and one child (maximum SBR level 556 mumol/L) was borderline abnormal (GCI 74) on psychological testing; he also suffered from Rhesus erythroblastosis. Premature infants recorded a mean GCI of 109.9 (+/- 33.4) and for term infants mean GCI was 110.3 (+/- 17.3; NS); however, the youngest premature infant was 32 weeks' gestation. When maximum SBR level was correlated with GCI and Motor Index (MI) the only significant correlation (r = -0.7445; P = 0.03) occurred in infants with Rhesus erythroblastosis and GCI. Since exchange transfusion has a mortality of between 0.3 and 5.3% and an associated morbidity incidence of 5.2% we suggest that the standard indication for its use (SBR level of 342 mumol/L) should only apply to infants with Rhesus erythroblastosis. The actual SBR level which places a newborn infant at significant risk of bilirubin encephalopathy, where the cause of jaundice is other than Rhesus erythroblastosis, cannot be determined by this study.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Perhaps vigintiphobia should only apply to infants with Rhesus erythroblastosis. 794 48

With increased emphasis on early detection of hearing impairment, more babies are likely to be referred at younger ages to otolaryngologists for evaluation. With a diminution in the number of infants who have hearing impairment as a result of such factors as maternal infection, neonatal sepsis, or ototoxicity, the relative importance of detecting a genetic cause of newborn hearing impairment is likely to increase. Therefore, the otolaryngologist must become familiar with common causes of hereditary hearing impairment and the ways in which the newborn should be evaluated for hereditary hearing impairment. Advancements are rapidly being made in the ability to detect genes that cause hearing impairment, and we are now on the threshold of discovering ways to use gene therapy to prevent or treat hereditary deafness.
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PMID:Genetics and molecular biology of deafness. 1052 53

In July 1995, a 43-year-old Japanese man presented with deafness in the right ear. On hospital admission, he had deafness in both ears and right facial palsy. Variously sized lymphoid cells with convoluted nuclei were observed in the cerebrospinal fluid. Surface marker analysis revealed monoclonality of T lymphocytes in the spinal fluid. Similar abnormal cells were observed in peripheral blood and bone marrow. Biopsy specimens of the stomach and prostate showed tight proliferation of large lymphoid cells in the interstitium and epithelium. Antibody against human T-lymphotrophic virus type 1 (HTLV-1) was present. The diagnosis of non-Hodgkin's lymphoma, diffuse type, was made. Seven months later, the patient died of sepsis. Autopsy revealed multiple lymphadenopathy in the abdomen and the infiltration of atypical lymphocytes to the pancreas, kidneys, and other organs. A monoclonal band of HTLV-1 provirus was detected by Southern blot analysis. To our knowledge, this is the first report of adult T-cell leukemia/lymphoma with auditory nerve abnormalities as the initial symptom.
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PMID:Adult T-cell leukemia/lymphoma with initial deafness. 1072 96

Families with 3 different syndromes characterized by autosomal dominant inheritance of low platelet count and giant platelets were studied. Fechtner syndrome is an autosomal-dominant variant of Alport syndrome manifested by nephritis, sensorineural hearing loss, and cataract formation in addition to macrothrombocytopenia and polymorphonuclear inclusion bodies. Sebastian platelet syndrome is an autosomal-dominant macrothrombocytopenia combined with neutrophil inclusions that differ from those found in May-Hegglin syndrome or Chediak-Higashi syndrome or the Dohle bodies described in patients with sepsis. These inclusions are, however, similar to those described in Fechtner syndrome. Other features of Alport syndrome, though, including deafness, cataracts, and nephritis, are absent in Sebastian platelet syndrome. Epstein syndrome is characterized by macrothrombocytopenia without neutrophil inclusions, in addition to the classical Alport manifestations-deafness, cataracts, and nephritis-and it is also inherited in an autosomal-dominant mode. We mapped the disease-causing gene to the long arm of chromosome 22 in an Italian family with Fechtner syndrome, 2 German families with the Sebastian platelet syndrome, and an American family with the Epstein syndrome. Four markers on chromosome 22q yielded an LOD score greater than 2.76. A maximal 2-point LOD score of 3.41 was obtained with the marker D22S683 at a recombination fraction of 0.00. Recombination analysis placed the disease-causing gene in a 3.37-Mb interval between the markers D22S284 and D22S693. The disease-causing gene interval in these 3 syndromes is similar to the interval described recently in an Israeli family with a slightly different Fechtner syndrome than the one described here. Recombination analysis of these 3 syndromes refines the interval containing the disease-causing gene from 5.5 Mb to 3.37 Mb. The clinical likeness and the similar interval containing the disease-causing gene suggest that the 3 different syndromes may arise from a similar genetic defect.
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PMID:Autosomal-dominant giant platelet syndromes: a hint of the same genetic defect as in Fechtner syndrome owing to a similar genetic linkage to chromosome 22q11-13. 1107 40


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