Clinical and immunological overlap between autoimmune lymphoproliferative syndrome and common variable immunodeficiency.

Autoimmune lymphoproliferative syndrome (ALPS) is mainly caused by defects in the CD95 pathway. Raised CD3+TCRαß+CD4-CD8- double negative T cells and impaired T cell apoptosis are hallmarks of the disease. In contrast, the B cell compartment has been less well studied. We found an altered distribution of B cell subsets with raised transitional B cells and reduced marginal zone B cells, switched memory B cells and plasma blasts in most of 22 analyzed ALPS patients. Moreover, 5 out of 66 ALPS patients presented with low IgG and susceptibility to infection revealing a significant overlap between ALPS and common variable immunodeficiency (CVID). In patients presenting with lymphoproliferation, cytopenia, hypogammaglobulinemia and impaired B cell differentiation, serum biomarkers were helpful in addition to apoptosis tests for the identification of ALPS patients. Our observations may indicate a role for apoptosis defects in some diseases currently classified as CVID.

Invasive infections caused by Neisseria meningitidis, Haemophilus influenzae and Streptococcus pneumoniae among children in St Petersburg, Russia.

This study investigated the causes of invasive bacterial infections in children aged <15 years in St Petersburg, Russia, during 2001-2003, using culture and antigen detection methods (rapid antigen latex agglutination (RAL)) for normally sterile body fluids. A pathogen was detected in 90 cases (culture 50, RAL 40). Neisseria meningitidis was the most common pathogen (66%), followed by Haemophilus influenzae (19%) and Streptococcus pneumoniae (16%). Meningitis was the main clinical diagnosis (68/90, 76%), with N. meningitidis serogroup B, H. influenzae type b (Hib), and S. pneumoniae serogroup 1 being the most common isolates. Hib was less prevalent in St Petersburg than it was in industrialised countries before the introduction of Hib vaccinations.

Pain medication during terminal care of children with cancer.

The purpose of this study was to evaluate the need for pain medication and the adequacy or inadequacy of the analgesia achieved, in children with cancer who died while in terminal care. Of the 100 pediatric patients with cancer treated at the Children's Hospital, University of Helsinki, Finland, who died during 1987-1992, 70 died while in terminal care. The underlying diseases were leukemia (N = 25), solid tumors (N =24), and brain tumors (N = 21). Of these children, 60% were treated at home, 29% at hospital, and 11% at both. The assessment of pain during terminal care was retrospective and included analysis of the patients' records and a structured interview of the two parents separately. In total 62 children (89%) received regular pain medication, with a mean duration of 17 days in children with leukemia, 58 days in those with solid tumors, and 66 days in those with brain tumors. Medication was usually started with anti-inflammatory drugs, then changed to oral opioids when deemed necessary, and finally to parenteral opioids. Parenteral morphine was administered to 40 children, to 30 as a continuous infusion through a central venous line. The dose of morphine was 0.8 mg/kg/day at the start and was increased to 4.9 (range, 0.2-55) mg/kg/day. Of the 62 children who received regular pain medication, the majority (81%) had adequate analgesia. In 19%, analgesia had been suboptimal. In conclusion, the vast majority of children with cancer need regular pain medication while in terminal care. This can be administered adequately at home, even if continuous intravenous infusions are required.

Terminal care of the child with cancer at home.

One hundred paediatric patients with either leukaemia (36%), solid tumours (34%) or brain tumours (30%), treated at the Children's Hospital, University of Helsinki, Finland, died during 1987-92; 70 of them died while in organized terminal care. They were treated at home (60%), in hospital (29%), and partly at both (11%). One or both parents stayed at home to take care of their child. Personnel of the oncologic ward coordinated home care. The purpose of this study was to evaluate the advantages and disadvantages of a terminal care program, with special reference to terminal care at home. Evaluation included retrospective analysis of patients' records, as well as a structured interview with the two parents separately. The quality of life of the children during the terminal period was greatly influenced by their happiness at being at home. Relief of symptoms, particularly pain, was in most instances adequate. Most parents had no complaints to make afterwards. Only some of them complained of having received too little information, too little supervision and support, and insufficient preparation for the death of the child. Thus, the system of terminal care at home proved satisfactory for the child and the whole family in many different respects. For successful home care, the parents need continuous supervision, help and support by well-trained personnel.

Autopsy in children with cancer who die while in terminal care.

BACKGROUND: The purpose of this study was to find out whether autopsy of children with cancer should be recommended after terminal care, or whether in those circumstances it could be abandoned. PATIENTS AND METHODS: One hundred pediatric patients with cancer treated at the Children's Hospital, University of Helsinki, Finland, died during 1987-92. Seventy children died while in organized terminal care. The underlying diagnoses were brain tumors (21), other solid tumors (24), and leukemias (25). The method was a retrospective analysis of patients' records and autopsy reports, in addition to a structured interview of the two parents separately. RESULTS: Autopsy was performed in 40 (57%) of these 70 cases. It was more often performed on children dying in hospital (69%) than in those dying at home (39%). The autopsy rate also varied with the underlying disease: 68% of patients with leukemia, 50% of those with solid tumors, and 52% of those with brain tumors were autopsied. Autopsy afforded totally new medical information in 20% of cases, and important additional information in 55%. Nothing unexpected was found in 25%. Almost all the parents (94%) who agreed to autopsy felt that it was appropriate. Of both mothers and fathers, 50% felt that knowing the findings at autopsy was helpful for them, and all the parents except one mother thought that the autopsy of their child would at least be helpful to other patients. CONCLUSIONS: Autopsy often provides important and even unexpected information in those dying after terminal care. The majority of our parents felt that autopsy was an acceptable and appropriate practice. We recommend that autopsies should be performed, with the parents' consent, even after terminal care.