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

A female child with Weaver syndrome is described. She did not show the excessive growth characteristic of this syndrome until after adequate caloric intake was achieved by feeding through a gastric feeding tube. Her inadequate nutrition was a result of feeding difficulties during infancy. In addition, she had recurrent pulmonary infections, apneic spells, and severe developmental delay. She died at 16 months of sepsis, congestive heart failure, and respiratory arrest. The autopsy revealed marked atrophy of the brain and cardiac ventricular hypertrophy. Most of the brain changes were thought to be from hypoxia.
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PMID:Weaver syndrome: a case without early overgrowth and review of the literature. 195 26

Fifty-eight patients with occipital encephalocoeles were retrospectively examined. These comprised about one-half of the cases seen and evaluated for treatment at the University College Hospital, Ibadan, Nigeria, between January 1973 and December 1987. There was a female-to-male preponderance of 2:1. Of the patients 91% were treated during infancy. Only one patient was precluded from surgery because his large ulcerated lesion was associated with severe microcephaly and neonatal sepsis, to which he succumbed. About four-fifths of the lesions exceeded 5 cm in diameter. The operative mortality was 6%, all deaths occurring in patients who were neonates at the time of surgery and whose hernia sacs contained brain substance. Clinically apparent hydrocephalus was more frequently encountered postoperatively, than preoperatively. Developmental delay was apparent in 5 of the 13 patients in whom developmental milestones were assessed during follow-up. For most patients, the follow-up period was short, possibly a reflection of the poor prognosis of the disease.
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PMID:Occipital encephalocoeles in 57 Nigerian children: a retrospective analysis. 234 May 37

Six infants with necrotizing enterocolitis were discharged after periods of prolonged hospitalization (two to nine months) with intact ileostomies. Their initial hospitalization was complicated by feeding difficulties, chronic diarrhea, sepsis, rickets, and developmental delay. All were rehospitalized within three months, with severe acidosis and dehydration after a presumed viral-type illness. Each had large-volume ileostomy output, which was rich in electrolytes and bicarbonate. A prolonged recovery phase (two to eight months) again was punctuated with episodes of diarrhea, problems in starting oral feeding, and sepsis. After reanastomosis of the remaining bowel, no infant has had a similar life-threatening episode. It is speculated that the infants' recurrent "salt-and-water-losing states" are secondary to either an anatomic or functional loss of the colon. This problem appears to be a poorly recognized sequela of bowel surgery and necrotizing enterocolitis, and early reanastomosis of discontinuous bowel should be of benefit.
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PMID:Importance of early ileostomy closure to prevent chronic salt and water losses after necrotizing enterocolitis. 709 91

The neurodevelopmental outcome of 78 infants with bronchopulmonary dysplasia (BPD) was compared with that of 78 control infants matched for birthweight. To determine the effect of the severity of BPD, 62 infants requiring oxygen at 36 weeks' postmenstrual age (sBPD) were compared with their matched controls. Infants were followed up to 2 years of age, corrected for prematurity, and were classified for neurological impairment, developmental delay, and neurodevelopmental disability. Seventy six (98%) BPD infants and 71 (91%) controls had follow up data available to two years. Neurological impairment, developmental delay, and neurodevelopmental disability occurred more frequently in infants with BPD than in controls but this was not significant. For infants with sBPD, the increased incidence of neurological impairment and definite developmental delay was not significant when compared with the controls, though neurodevelopmental disability occurred more frequently (odds ratio (OR) 3.6: 95% confidence intervals (CI) 1.1-11.8). Predictors of disability in infants with sBPD included periventricular haemorrhage (OR 19.4: 95% CI 4.3-86.6), ventricular dilatation (OR 12.8: 95% CI 2.9-57.3), and sepsis (OR 5.0: 95% CI 1.3-19.4). Adjusting for the presence of these factors, the association between BPD and disability was no longer apparent (OR 0.9: 95% CI 0.2-3.6). The findings suggest that BPD is not independently associated with adverse neurodevelopmental outcome.
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PMID:Neurodevelopmental outcome of preterm infants with bronchopulmonary dysplasia. 853 67

The detection of neurodegenerative and neurometabolic diseases in children relies on a high index of suspicion as most will present as common paediatric problems such as recurrent vomiting, feeding problem, failure to thrive, sepsis, or developmental delay. Alternatively, children may present with an acute encephalopathy or with a chronic progressive encephalopathy. Clinical clues suggestive of neurometabolic disorders include encephalopathic features such as microcephaly, macrocephaly, developmental regression, developmental arrest, change in sensorium, seizures, hypotonia, hypertonia, abnormal eye signs; also extrapyramidal or cerebellar signs and systemic features like abnormal respiration, hepatosplenomegaly, abnormal hair, liver dysfunction, renal tubular dysfunction, cardiomyopathy, and feeding difficulties or growth problems. Initial screening include tests for acidosis, ketosis, hyperlacticemia, and hyperammonemia. Further investigations should amino acid chromatography, assays of organic acids, specific enzyme assay of white cell or fibroblast culture, and histopatholgy of cell and tissue biopsy (white blood cell, skin, muscle, conjunctiva, bone marrow, liver, rectum, or brain). The correct diagnosis holds implications for targeted therapeutic intervention, genetic counselling, and possibly, prenatal diagnosis.
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PMID:Neurodegenerative diseases in children. 1184 61

We report a pair of brothers who have leukoencephalopathy, arthritis, colitis, and hypogammaglobulinemia. Both presented initially with seizures in the early postnatal period. They have significant developmental delay, and brain MRIs demonstrate leukoencephalopathy, characterized by profound hypomyelination. They have developed arthritis, which in one brother has required chronic treatment; and persistent intermittent diarrhea, necessitating treatment for inflammatory bowel disease. Finally, they have had multiple hospitalizations, including several for sepsis; an immunological analysis revealed that they have IgG1-subclass hypogammaglobulinemia and low B cell levels. There is no family history of similar problems, and these brothers have an unaffected brother. We believe this constellation of symptoms represents a novel syndrome: LACH syndrome (leukoencephalopathy, arthritis, colitis, and hypogammaglobulinemia). The etiology of this syndrome remains unknown despite extensive investigations.
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PMID:Leukoencephalopathy, arthritis, colitis, and hypogammaglobulinemia (LACH) in two brothers: a novel syndrome? 1521 57

Three hundred-forty-eight out of a regional population of 1272 newborn infants were randomly chosen and followed neurologically until age of two years to study the epidemiology of neurodevelopmental disorders, and to reveal the main factors influencing outcome. The most frequent neonatal pathologies were low Apgar scores - 45 (3.5%), neonatal sepsis - 28 (2.2%), neonatal seizures - 26 (2.0%), neonatal sepsis complicated with bacterial meningitis - 13 (1.0%), traumatic injury of peripheral nerves - 7 (0.6%), intracranial hemorrhages - 4 (0.3%) and CNS malformations - 3 (0.2%). At the age of 24 months abnormal development was identified in 29 cases (8.5%) of children, comprising global developmental delay in five (1.5%), unclassified motor problems (hypotonia without ataxia) in four (1.2%), cerebral palsy in three (0.9%), behavioral/sleep disorders in 12 (3.5%) and epilepsy in five (1.5%). The most significant single risk factors for abnormal neurodevelopmental outcome were maternal age, chorioamnionitis, gestational age <37 weeks, pathological delivery, and a low (<5) Apgar score at 5min after birth. Coexistence of several risk factors increased the probability of an adverse outcome.
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PMID:Epidemiology of neurodevelopmental disorders in 2 years old Georgian children. Pilot study - population based prospective study in a randomly chosen sample. 1968 48

Few cases of severe postnatally acquired cytomegalovirus (CMV) infection are reported in premature infants. We report on an extremely low birthweight (ELBW) preterm infant who presented with a sepsis-like syndrome and multiple organ involvement, notably pneumonitis and colitis. The course of infection was assessed by repeated analysis of urine, tracheal aspirates and blood. The patient was given intravenous ganciclovir. The clinical course was rapidly favorable. Development of neutropenia led to the discontinuation of the antiviral treatment after 28 days. Follow-up showed moderate white matter anomalies on cerebral MRI, a transient hypoacusis and a mild developmental delay at 18 months of corrected age. To the best of our knowledge, this is the first description of a severe combination of pneumonitis and colitis in postnatal CMV infection. Many issues remain controversial and are discussed. We propose that antiviral treatment should be considered in severe postnatal CMV infection in ELBW patients.
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PMID:Severe postnatally acquired cytomegalovirus infection presenting with colitis, pneumonitis and sepsis-like syndrome in an extremely low birthweight infant. 1994 May 17

Pica is characterized by the persistent eating of non-nutritive substances over some time that is inappropriate for the maturation stage of the individual and is not culturally sanctioned. A 9-year-old boy with Goldenhar syndrome, significant developmental delay and pica, collapsed and died after a short history of diarrhea and vomiting. Death was due to a sigmoid volvulus resulting from filling of the distal colon with feces containing dirt, stones, and rice with evidence of ischemic intestinal necrosis. Lethal complications of pica include intestinal obstruction and perforation with peritonitis and generalized sepsis. Other findings at autopsy may include airway obstruction, heavy metal poisoning, and parasitic infestation. Presenting symptoms and signs of such complications may be subtle or masked given the nature of underlying conditions, and so careful evaluation of the medical histories of individuals with pica may be necessary to provide pertinent details of associated medical and psychiatric conditions.
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PMID:A review of the forensic implications of pica. 2497 10

Bacterial infection is one of the main causes of preterm premature rupture of membranes (PPROM) leading to preterm delivery, pulmonary hypoplasia, sepsis and joint deformities. Expectant management, broad-spectrum antibiotics and antenatal corticosteroids are routinely used in this condition with very limited success to prevent bacteremia, chorioamnionitis, funisitis and intra-amniotic infection syndrome. Here, we report a case in which we attempted to treat PPROM at 26+3 weeks of gestation with anhydramnion colonized by multiresistant Klebsiella. A perinatal port system was implanted subcutaneously at 28+0 weeks of gestation, enabling long-term continuous lavage of the amniotic cavity with a hypotonic aqueous composition similar to human amniotic fluid combined with intra-amniotic antibiotic application. The patient gave birth to a preterm female infant at 31+1 weeks without any signs of infection. The girl was discharged with a weight of 2,730 g in very good condition. In the follow-up examinations at 5 months and 1 year of age, there was no apparent neurological disturbance, developmental delay or Klebsiella colonization.
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PMID:Treatment of Preterm Premature Rupture of Membranes with Oligo-/Anhydramnion Colonized by Multiresistant Bacteria with Continuous Amnioinfusion and Antibiotic Administrations through a Subcutaneously Implanted Intrauterine Port System: A Case Report. 2644 23


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