| 1. |
- Arvidsson, J, et al.
(författare)
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The first case of the Sanfilippo type C syndrome in Scandinavia
- 1983
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Ingår i: Acta Paediatrica Scandinavica. - : Wiley. - 0001-656X .- 0803-5253 .- 1651-2227. ; 72:2, s. 313-316
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Tidskriftsartikel (refereegranskat)abstract
- A Swedish patient with typical symptoms of the Sanfilippo Syndrome (Mucopolysaccharidosis III) is described. The early onset of the disease, the presence of hepatomegaly, early dementia and the absence of umbilical bernia are consistent with the subgroup Sanfilippo A. Enzyme studies indicate the diagnosis Sanfilippo C, and thus the patient represents a more severe form of this subgroup than any of the four other patients hitherto described in detail.
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| 2. |
- Donnér, M, et al.
(författare)
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Platelet surface-bound IgG and platelet-specific IgG in plasma in childhood thrombocytopenia
- 1990
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Ingår i: Acta Paediatrica Scandinavica. - : Wiley. - 0001-656X .- 0803-5253 .- 1651-2227. ; 79:3, s. 328-334
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Tidskriftsartikel (refereegranskat)abstract
- Quantification of platelet-bound immunoglobulin is widely used in the evaluation of thrombocytopenia. Several methods have been devised among which labelled ligand-binding assays seem to be most appropriate. In series of adult patients such assays have been shown to be superior in separating immune-thrombocytopenia from thrombocytopenia of non-immune causes. We studied 62 children with thrombocytopenia of various causes, using radiolabelled protein A as a ligand to measure platelet-surface bound IgG. The test was highly sensitive (93%) in detecting immune-thrombocytopenia. The specificity, however, was only 57%, which is less than in published studies of adults. In a number of cases presumed to be non-immune-thrombocytopenia, notably a few patients with leukaemia and bone marrow aplasia, we found increased amounts of platelet surface-bound IgG. The significance of this finding is not clear. An indirect assay measuring platelet-specific IgG in plasma was less sensitive (46%) but highly specific for immune-thrombocytopenia (89%). The measurements of platelet-surface-bound IgG and platelet-specific IgG in plasma are of limited diagnostic value in childhood thrombocytopenia but are useful in following the treatment in chronic ITP.
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| 3. |
- Greisen, G, et al.
(författare)
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EEG depression and germinal layer haemorrhage in the newborn
- 1987
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Ingår i: Acta Paediatrica Scandinavica. - : Wiley. - 0001-656X .- 0803-5253 .- 1651-2227. ; 76:3, s. 519-525
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Tidskriftsartikel (refereegranskat)abstract
- Amplitude integrated EEG (aEEG) recordings from 32 mechanically ventilated infants, gestational age 32 weeks or less, were analysed. All recordings were started within 24 h of birth and continued for at least 50 h. Germinal layer haemorrhage (GLH) was diagnosed by repeated ultrasonography. In six infants neither GLH nor hypocalcaemia were diagnosed; aEEG in these infants rapidly became more active after birth: at 30 h of age continuous background activity was present for more than 20% of the time, and a seizure-like pattern was exceptional. In seven infants without GLH but with hypocalcaemia and other signs of metabolic derangement, continuous background activity appeared later and seizure-like activity was frequent. In the infants with GLH, depression of the background activity was apparent. This finding was particularly distinct in the presence of severe haemorrhages. Four infants developed GLH after 30 h of age. All these infants had depressed aEEG before the development of GLH, with less than 20% continuous activity at 30 h of age. In ten infants an analysis of the aEEG during the occurrence of GLH was possible. In six of these, cortical electrical activity decreased. Due to the limitation of GLH timing, it was not possible to decide whether this decrease closely preceded or followed GLH. We suggest that GLH primarily occurs in brains with a preceding metabolic and neurophysiologic abnormality, and that further functional deterioration is caused by the most severe haemorrhages.
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| 4. |
- Hallberg, A, et al.
(författare)
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Natural killer cell activity and antibody-dependent cellular cytotoxicity in newborn infants
- 1982
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Ingår i: Acta Paediatrica Scandinavica. - : Wiley. - 0001-656X .- 0803-5253 .- 1651-2227. ; 71:3, s. 431-436
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Tidskriftsartikel (refereegranskat)abstract
- The ability of lymphocytes from the peripheral blood of preterm and term infants and adult women and men to mediate natural killing (NK) and K cell activity (antibody-dependent cellular cytotoxicity) was analysed in 4 hours 51Cr-release assays. K 562 cells were targets for NK activity. K cell activity was assayed on antibody-coated rat thymocytes. Lymphocytes from adult male donors were significantly more cytotoxic to K 562 cells than were lymphocytes from adult female donors. Lymphocytes from both preterm and term infants had significantly lower NK and K cell activity than lymphocytes from adult donors. During the first month of life no increase in NK activity or K cell activity occurred in 7 infants who were re-examined. It is concluded that neither NK nor K cell activities are fully developed during the first month of life.
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| 5. |
- Helin, I, et al.
(författare)
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Outbreak of coxsackievirus A-14 meningitis among newborns in a maternity hospital ward
- 1987
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Ingår i: Acta Paediatrica Scandinavica. - : Wiley. - 0001-656X .- 0803-5253 .- 1651-2227. ; 76:2, s. 234-238
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Tidskriftsartikel (refereegranskat)abstract
- During the late winter of 1983, 16 newborns with vague symptoms of failure to thrive, reluctance to feed and a slight rise in body temperature, were found to have meningitis caused by Coxsackievirus A-14. The cerebrospinal fluid showed pleocytosis with polymorphonuclear cells in excess but was otherwise normal. The clinical course was uneventful in all infants, but two of them demonstrated clinical signs of incipient cerebral oedema during the acute phase of the illness. An electroencephalogram (EEG) during the initial course of the disease and at nine months of age was normal in all. During a follow-up period of 2 1/2 years they all developed normally and no sequelae were noted. The presentation also demonstrates the usefulness of Vero cells for the propagation of the responsible virus.
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| 6. |
- Hellström-Westas, Lena, et al.
(författare)
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Lidocaine for treatment of severe seizures in newborn infants. I. Clinical effects and cerebral electrical activity monitoring
- 1988
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Ingår i: Acta Paediatrica Scandinavica. - : Wiley. - 0001-656X .- 0803-5253 .- 1651-2227. ; 77:1, s. 79-84
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Tidskriftsartikel (refereegranskat)abstract
- The anticonvulsive effect of lidocaine was evaluated in 46 newborn infants with severe, recurrent seizures. Before the lidocaine all infants were being given phenobarbital, and 22 infants were also treated with diazepam. Different dosages of lidocaine were tested. A loading dose of 2 mg/kg followed by i.v. infusion of 6 mg/kg/hour was the most effective dosage and had an immediate anticonvulsive effect in 18 of 25 infants; within 30 min the same effect was attained in another five of the infants, with an overall seizure control in 92% of the sample population. During the lidocaine treatment cerebral electrical activity was followed continuously with a cerebral function monitor (CFM), which also enabled evaluation of the treatment. No serious side effects on blood-pressure, heart-rate or cerebral electrical activity were registered. For newborn infants with severe recurrent seizures not responding to other drugs, lidocaine is an effective additional mode of treatment.
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| 7. |
- Hellström-Westas, Lena, et al.
(författare)
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Silent seizures in sick infants in early life. Diagnosis by continuous cerebral function monitoring
- 1985
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Ingår i: Acta Paediatrica Scandinavica. - : Wiley. - 0001-656X .- 0803-5253 .- 1651-2227. ; 74:5, s. 741-748
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Tidskriftsartikel (refereegranskat)abstract
- Cerebral electric activity was surveilled with a Cerebral Function Monitor (CFM) technique in 87 newborn infants under neonatal intensive care. A total of 26 infants had electrographical signs of repeated seizure activity. Among these infants 14 had periods of one hour or more of silent seizures activity. Among these infants 14 had periods of one hour or more of silent seizures, i.e. typical pattern of ictal epileptic activity on CFM without clinical symptoms or signs of convulsions. The occurrence of silent seizures and their pattern in relation to the clinical condition and management was unpredictable in most cases. Besides general limpness or flaccidity in an outward quiet baby these infants showed no clinical fits or clonic convulsions. The findings indicate that anticonvulsive therapy in small infants may be insufficient and need re-evaluation, since the long-term effect of silent seizures on cerebral function and activity is still uncertain.
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| 8. |
- Ljung, R., et al.
(författare)
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More than half the sporadic cases of Hemophilia A in Sweden are due to a recent mutation
- 1991
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Ingår i: Acta Paediatrica Scandinavica. - : Wiley. - 0001-656X .- 0803-5253 .- 1651-2227. ; 80:3, s. 343-348
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Tidskriftsartikel (refereegranskat)abstract
- The aim of this study was to ascertain how many of the sporadic cases of severe Haemophilia A in Sweden are due to recent mutation, and establish its origin. DNA analysis was performed in 18 randomly selected families with a sporadic case of severe haemophilia A. Restriction fragment length polymorphism (RFLP) patterns were investigated, two intragenic (Bc1 I, Xba I/Kpn I) and two extragenic (DX 13, St 14) RFLP being used. In 10/18 families a haemophilia-linked gene was found to have derived from the healthy maternal grandfather; on the basis of clotting and immunological assay results, the odds were high (>104:1) for maternal carriership in four of these 10 cases, and for maternal non-carriership in two, four being indeterminate. In 4/18 families a haemophilia-linked gene derived from the healthy maternal grandmother; according to clotting and immunological assay results, in two cases the odds were high for maternal non-carriership. In the remaining 4/18 families no conclusions could be drawn from the RFLP pattern as to the origin of mutation. We conclude that at least 55% of the sporadic cases of severe hemophilia A in Sweden are due to a recent mutation within the last two genrations.
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| 9. |
- Moëll, Christian, et al.
(författare)
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Bcg vaccination as a cause of multifocal osteomyelitis in a 12-year-old girl
- 1983
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Ingår i: Acta Paediatrica Scandinavica. - : Wiley. - 0001-656X .- 0803-5253 .- 1651-2227. ; 72:1, s. 153-155
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Tidskriftsartikel (refereegranskat)abstract
- A 12-year-old girl with multifocal skeletal sclerosis has been investigated. She was BCG vaccinated at birth and developed regional lymphadenitis in her left groin at two months of age. She was healthy until approximately 10 years of age, after which deficient height and weight gain occurred. BCG-itis was diagnosed in skeleton lesions. No evidence of immunodeficiency was found in the patient. After antituberculous treatment, the skeleton lesions, previously seen at bone scan, had disappeared. There has been a dramatic effect on her weight gain, growth and general well-being.
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| 10. |
- Mårdh, Per-Anders, et al.
(författare)
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Intrauterine lung infection with Chlamydia trachomatis in a premature infant
- 1984
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Ingår i: Acta Paediatrica Scandinavica. - : Wiley. - 0001-656X .- 0803-5253 .- 1651-2227. ; 73:4, s. 569-572
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Tidskriftsartikel (refereegranskat)abstract
- The present study shows the probable intrauterine infection with Chlamydia trachomatis in a premature infant born in the 29th week of gestation. Chlamydiae were isolated from lung tissue collected at sterile autopsy and also demonstrated in sections of such tissue by immunofluorescence tests using monoclonal antibodies.
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