To evaluate the vaccination coverage rate of patients with JIA followed at a paediatric tertiary care centre and to determine the coverage rate for individual vaccines required as per the Quebec ...Immunization Protocol.
Consecutive JIA patients coming for their scheduled visit were included if they were between 2 and 18 years of age and if they had an available written immunization record. Descriptive statistics were used to evaluate the proportion of children with complete vaccination status according to the Quebec Immunization Protocol at 2.5, 10.5 years and at their last clinic visit.
A total of 200 patients were included. Complete vaccination according to schedule was identified in only 52% of patients at 2.5 years, 68% at 10.5 years and 61% at their last clinic visit. The vaccination coverage rate for individual vaccines was good overall with the exception of low measles, mumps and rubella vaccine coverage at 2.5 years (58%).
Despite overall good vaccination coverage rate for individual vaccines, only 61% of our cohort had a complete vaccination status at their last clinic visit. Measures to optimize vaccination coverage, such as catch-up vaccination, should be implemented when possible.
Secretion of cytolytic granules content at the immunological synapse is a highly regulated process essential for lymphocyte cytotoxicity. This process requires the rapid transfer of perforin ...containing lytic granules to the target cell interface, followed by their docking and fusion with the plasma membrane. Defective cytotoxicity characterizes a genetically heterogeneous condition named familial hemophagocytic lymphohistiocytosis (FHL), which can be associated with perforin deficiency. The locus of a perforin (+) FHL subtype (FHL3), observed in 10 patients, was mapped to 17q25. This region contains
hMunc13-4, a member of the Munc13 family of proteins involved in vesicle priming function.
HMunc13-4 mutations were shown to cause FHL3. HMunc13-4 deficiency results in defective cytolytic granule exocytosis, despite polarization of the secretory granules and docking with the plasma membrane. Expressed tagged hMunc13-4 localizes with cytotoxic granules at the immunological synapse. HMunc13-4 is therefore essential for the priming step of cytolytic granules secretion preceding vesicle membrane fusion.
Décrire les caractéristiques de la population décédée dans un hôpital pédiatrique, les maladies en cause et les modalités de décès.
Il s'agit d'une étude épidémiologique rétrospective, descriptive, ...réalisée sur six ans. Ont été inclus les enfants décédés dans notre hôpital entre le 1
er janvier 1990 et le 31 décembre 1995. Ont été exclus les enfants arrivés morts à l'hôpital (morts subites du nourrisson, enfants décédés pendant le transport, corps déposés) et ceux pour lesquels le dossier n'a pu être retrouvé.
La population étudiée comportait 375 enfants décédés dont 195 nouveau-nés. Le sexratio était de 1,3. Au total, 91 % des décès ont eu lieu dans trois principaux services : en réanimation, en neurochirurgie-neurologie et en oncologie. La médiane de la durée d'hospitalisation était de trois jours. Les maladies les plus fréquemment en cause étaient accidentelles, neurologiques (notamment les nouveau-nés) et tumorales. L'analyse des modalités de décès a montré que 41,1 % d'entre eux faisaient suite à un échec des manœuvres de réanimation, 38,9 % à un arrêt des soins curatifs ou à une décision de ne pas réanimer et 21,6 % à un coma dépassé. L'évolution des modes de décès sur six ans a montré une diminution des échecs de réanimation, une augmentation des décisions de ne pas réanimer et des arrêts de soins curatifs, un nombre stable de comas dépassés. Douze prélèvements d'organes ont pu être effectués parmi les 81 enfants en coma dépassé.
La maladie d'origine accidentelle reste la première cause de décès. Les décès consécutifs à un arrêt des soins curatifs sont aujourd'hui plus fréquents qu'il y a quelques années.
To define the characteristics of patients dying in a pediatric hospital, including causes and modes of death.
This retrospective, descriptive, epidemiologic study was performed between 1 January 1990 and 31 December 1995. All patients who died in the hospital between these dates were included. Patients already dead on arrival (sudden infant death syndrome, children deceased during their transport), and those whose hospital records could not be found, were excluded.
A total of 375 children were studied, including 195 neonates. The sex ratio was 1.3. Ninety-one percent of deaths took place in three departments: intensive care, neurosurgeryneurology and oncology. Median duration of hospitalization was three days. The most common causes of deaths were accidents, neurologic diseases (particularly among neonates) and tumours. Analysis of modes of death revealed that 41.1% occurred following unsuccessful resuscitation, 38.8% were the result of withdrawal of life-support or a ‘do not resuscitate’ order and 21.6% resulted from brain death. Evolution of modes of death over the six years showed a reduction of cases with unsuccessful resuscitation, an increase in decisions of 'do not resuscitate'orders and withdrawal of life-support and no change in rates of brain death. Organs were made available for transplantation from 12 of the 81 children with brain death (14.8%).
Accidents were the most common cause of death. The distribution of deaths showed a clear increase in withdrawal or withholding of life-support care, relying on ethical decisions, which are more frequent than some years ago.
Allogeneic haemopoietic stem-cell transplantation (HSCT) is the treatment of choice for many haemological malignancies and inherited disorders. When stem cells for transplantation come from a human ...leucocyte antigen matched unrelated donor, or from a partly mismatched related donor, exvivo T-cell depletion of the graft can prevent development of graft-versus-host disease, but lead in turn to a delay in immune reconstitution and a concordant increase in incidence of opportunistic infections and leukaemic relapses. We aimed to infuse T cells selectively depleted in allogeneic T cells that cause graft-versus-host disease using an ex-vivo procedure designed to eliminate alloactivated donor T cells, with an immunotoxin that reacts with a cell surface activation antigen, CD25.
We did a phase 1/2 study, in which 1–8 × 105 allodepleted T cells/kg were infused between days 15 and 47 into 15 paediatric patients who had acquired or congenital haemopoietic disorders and who received HSCT on day 0. Occurrence of graft-versus-host disease and time to immune reconstitution were assessed. No treatment for graft-versus-host disease was given.
Less than 1% residual anti-host alloreactivity was recorded in 12 of 16 procedures. Other immune responses were preserved by the allodepletion procedure in 12 cases. No cases of severe (greater than grade II) graft-versus-host disease arose. Evidence for early T-cell expansion was shown in three patients with continuing viral infections. Specific antiviral responses, such as strong cytolytic activity, were noted.
Our results show that ex-vivo selective depletion of T cells that cause graft-versus-host disease is efficient and feasible, even in haploidentical settings.
Kaposi's sarcoma (KS) is rare in childhood. It may be favored by acquired immune deficiencies, but the predisposing factors to KS in other children are unclear.
KS has been reported in only two ...children and one adult with primary immunodeficiency. We report here a Tunisian child with a Wiskott-Aldrich syndrome who developed KS at the age of 14 months.
This observation expands the spectrum of primary immunodeficiencies associated with KS in childhood.
To define the characteristics of patients dying in a pediatric hospital, including causes and modes of death.
This retrospective, descriptive, epidemiologic study was performed between 1 January 1990 ...and 31 December 1995. All patients who died in the hospital between these dates were included. Patients already dead on arrival (sudden infant death syndrome, children deceased during their transport), and those whose hospital records could not be found, were excluded.
A total of 375 children were studied, including 195 neonates. The sex ratio was 1.3. Ninety-one percent of deaths took place in three departments: intensive care, neurosurgery-neurology and oncology. Median duration of hospitalization was three days. The most common causes of deaths were accidents, neurologic diseases (particularly among neonates) and tumours. Analysis of modes of death revealed that 41.1% occurred following unsuccessful resuscitation, 38.8% were the result of withdrawal of life-support or a 'do not resuscitate' order and 21.6% resulted from brain death. Evolution of modes of death over the six years showed a reduction of cases with unsuccessful resuscitation, an increase in decisions of 'do not resuscitate' orders and withdrawal of life-support and no change in rates of brain death. Organs were made available for transplantation from 12 of the 81 children with brain death (14.8%).
Accidents were the most common cause of death. The distribution of deaths showed a clear increase in withdrawal or withholding of life-support care, relying on ethical decisions, which are more frequent than some years ago.
Objective. –
To assess changes in growth parameters associated with the response to methotrexate (MTX) therapy in pre-pubertal children with juvenile idiopathic arthritis (JIA) and who had not been ...treated with steroids.
Methods. –
We enrolled 27 pre-pubertal children with JIA who had been treated with MTX but not with steroids. The children were considered to have responded to treatment if the number of joints with active disease decreased by at least 50% 1 year after treatment initiation. We compared growth parameters (height, growth rate, weight and body mass index (BMI)) in responders and non-responders.
Results. –
Twenty-one children (77.8%) responded to MTX therapy. The growth parameters were similar in the responders and non-responders before the onset of treatment. After 1 year, height (
P
=
0.025), growth rate (
P
=
0.03), weight (
P
=
0.007) and BMI (
P
=
0.05) increased significantly in the responder group, but not in the non-responder group. This increase was maintained for growth rate and weight after 2 and 3 years of treatment. After 1 year, height (
P
=
0.023) and growth rate (
P
=
0.0009) were significantly higher in the responders than in the non-responders, and these differences were still significant after 3 years (
P
=
0.01 and
P
=
0.033, respectively).
Conclusion. –
In pre-pubertal children with JIA, a clinical response to MTX therapy is associated with a significant increase in growth parameters.
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