RESUMO
We describe three previously healthy children who developed acute extensive bruises about two weeks after a mild stomach bug. Coagulation tests revealed a shortened thromboplastin time (TT), long PT time, low level of coagulation factor II, and positive lupus anticoagulant among the antiphospholipid antibodies. In one patient the clinical symptoms disappeared during a one-week course of prednisolone, another one received a prothrombin complex preparation as substitution therapy. In the third patient the symptoms were milder and vanished without any specific treatment. The levels of coagulation factor increased in all patients and the lupus anticoagulant disappeared within a couple of months.
Assuntos
Anticorpos Antifosfolipídeos/sangue , Transtornos Hemorrágicos/sangue , Testes de Coagulação Sanguínea , Criança , Diagnóstico Diferencial , Glucocorticoides/uso terapêutico , Transtornos Hemorrágicos/tratamento farmacológico , Humanos , Prednisolona/uso terapêutico , Protrombina/uso terapêuticoRESUMO
Regular audit of training of specialist physicians by means of comparable methods is a recommended means to take care of immediate quality assessment of the training. An audit group consisting of the professor of pediatrics at the University of Helsinki and professor of pediatrics at the University of Tampere, two clinical lecturers and two representatives of physicians specializing in pediatrics at the Children's Hospital together with an outside expert evaluates the common trunk belonging to specialization in pediatrics in teaching hospitals. The group stated that the recommendation for degree associated with specialist training is being followed in the hospitals, but the follow-up of the objectives and their implementation is insufficient.
Assuntos
Educação de Pós-Graduação em Medicina/normas , Avaliação Educacional , Pediatria/educação , Finlândia , Hospitais de Ensino , HumanosRESUMO
BACKGROUND: We report a new mutation in the human DNAJC19 gene that causes early onset dilated cardiomyopathy syndrome (DCMA). METHODS: Two brothers of Finnish origin presented with an unusual combination of early onset dilated cardiomyopathy syndrome, a disease which was associated with cardiac noncompaction, microcytic anemia, ataxia, male genital anomalies and methylglutaconic aciduria type V. Suspicion of a DCMA syndrome prompted sequencing of the human DNAJC19 gene. RESULTS: Sequencing of the human DNAJC19 gene showed a homozygous single nucleotide (A) deletion in alanine 63 coding triplet in exon 6, which does not immediately cause amino acid change but leads 11 amino acids later to a stop codon and to premature termination of the peptide. This DNAJC19 protein is located in the inner mitochondrial membrane and has been shown to function as a mitochondrial chaperone. CONCLUSION: This is the first clinical report of DCMA syndrome, a human DNAJC19 deficiency, that is related to cases of severe dilated cardiomyopathy diagnosed in Europe. DNAJC19 deficiency causes a relatively specific finding in urinary organic acid analysis (methylglutaconic aciduria type V), which together with the clinical features of the ensuing cardiac disease, allows for effective screening before undertaking molecular genetic analysis.
Assuntos
Anormalidades Múltiplas/genética , Anemia/genética , Ataxia/genética , Cardiomiopatia Dilatada/genética , Proteínas de Transporte da Membrana Mitocondrial/genética , Mutação , Anormalidades Urogenitais/genética , Sequência de Aminoácidos , Anemia/terapia , Ataxia/terapia , Autopsia , Sequência de Bases , Cardiomiopatia Dilatada/terapia , Células Cultivadas , Pré-Escolar , Análise Mutacional de DNA , Evolução Fatal , Predisposição Genética para Doença , Humanos , Lactente , Masculino , Dados de Sequência Molecular , Fenótipo , Síndrome , Anormalidades Urogenitais/terapiaRESUMO
BACKGROUND: Corticosteroids have been shown not to prevent the development of Henoch-Schönlein nephritis. However, long-term follow-up data are scarce. METHODS: The long-term outcome of patients in a randomized placebo-controlled prednisone study was evaluated 8 years later with a health questionnaire completed by 160/171 (94%) patients and by urine and blood pressure screening (138/171, 81%). RESULTS: Twelve patients had hematuria and/or proteinuria and seven had hypertension. The patients with nephritis at onset of Henoch-Schönlein purpura (HSP) had an increased risk of hypertension and/or urine abnormalities (odds ratio 3.6, p = 0.022, 95% confidence interval 1.3-10.0). There were no differences between the prednisone and placebo groups. Recurrences of purpura were reported by 15 patients, with some recurrences continuing for 10 years. All five reported pregnancies were complicated by proteinuria. Four patients presented with hematuria and/or proteinuria at the control visit, and four had hypertension. Of these, two had a decreased estimated glomerular filtration rate. CONCLUSIONS: HSP has a good long-term prognosis in unselected patients, although skin relapses with/without late-onset nephritis may occur, even a decade after the initial disease. Urine and blood pressure abnormalities 8 years after HSP are associated with nephritis at its onset. Early prednisone treatment does not affect the outcome and should not be routinely used.
Assuntos
Glucocorticoides/uso terapêutico , Vasculite por IgA/tratamento farmacológico , Prednisona/uso terapêutico , Adolescente , Adulto , Pressão Sanguínea , Distribuição de Qui-Quadrado , Criança , Método Duplo-Cego , Feminino , Finlândia , Hematúria/etiologia , Hematúria/urina , Humanos , Hipertensão/etiologia , Hipertensão/fisiopatologia , Vasculite por IgA/complicações , Masculino , Nefrite/etiologia , Nefrite/urina , Razão de Chances , Placebos , Proteinúria/etiologia , Proteinúria/urina , Recidiva , Medição de Risco , Fatores de Risco , Inquéritos e Questionários , Fatores de Tempo , Resultado do Tratamento , Adulto JovemRESUMO
AIM: To describe the clinical course, morbidity and platelet recovery in an unselected Nordic cohort of children with chronic Immune Thrombocytopenic Purpura (ITP). METHODS: Prospective 5-year follow-up of 96 children with ITP lasting more than 6 months, with reporting of hospital admissions, severity of bleeding episodes and stabilization of platelet counts above 20, 50 and 150 × 10(9) /L. RESULTS: The estimated 5-year recovery rate was 52%; exclusion of 12 splenectomized children did not change the estimate. Events eliciting admission to hospital occurred in 39 (41%). Major haemorrhages occurred in eight children (8%), including a nonfatal intracranial haemorrhage in one child (1%). The overall admission rate was 0.4/year of thrombocytopenia, decreasing during follow-up as thrombocytopenia converted to milder degrees. Early recovery within 2 years of diagnosis occurred in 35%, was associated with low morbidity and was more likely in young children with abrupt onset of symptoms. CONCLUSION: In a Nordic cohort of children with chronic ITP, one half had recovered 5 years after diagnosis, more than half never required hospitalization and <10% experienced serious bleeding episodes, always with a platelet count <20 × 10(9) /L. Aggressive management can be restricted to the minority of children with continuing severe thrombocytopenia and frequent, clinically significant bleeding events.
Assuntos
Púrpura Trombocitopênica Idiopática , Adolescente , Criança , Pré-Escolar , Doença Crônica , Feminino , Finlândia/epidemiologia , Seguimentos , Hospitalização/estatística & dados numéricos , Humanos , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , Contagem de Plaquetas , Prognóstico , Estudos Prospectivos , Púrpura Trombocitopênica Idiopática/sangue , Púrpura Trombocitopênica Idiopática/epidemiologia , Púrpura Trombocitopênica Idiopática/terapia , Recuperação de Função Fisiológica , Remissão Espontânea , Países Escandinavos e Nórdicos/epidemiologia , Índice de Gravidade de Doença , Esplenectomia , Fatores de TempoRESUMO
Knowledge about how to treat severe Henoch-Schönlein nephritis (HSN) is scarce. The aim of our study is to compare cyclosporine A (CyA) and methylprednisolone pulses (MP) in the treatment of severe HSN. Out of 24 pediatric HSN patients with nephrotic-range proteinuria or crescentic HSN in kidney biopsy, seven were randomized to receive CyA for 12 months at an initial dose of 5 mg/kg and eight to receive 3 MP pulses of 30 mg/kg followed by prednisone for 4 months. The other nine patients received identical treatment without randomization. Kidney biopsies were performed at inclusion and after 2 years. The primary outcomes were the duration of proteinuria and hematuria, estimated glomerular filtration rate, and renal biopsy histology. All the 11 CyA-treated patients achieved resolution of nephrotic-range proteinuria within 3 months, while the MP-group response was slower, and in 6/13 was not achieved with the initial treatment. Additional immunosuppressive treatment was needed in none of the CyA-treated patients but in six patients treated with MP (difference in proportion 46%, p = 0.008). The 2-year control biopsies were similarly improved in both groups. After mean 6.1 years (2.2-10.4 years), 16 patients (eight CyA, eight MP) had no renal symptoms and six (three CyA, three MP) had persistent nephropathy but normal renal function. One MP-treated patient had reduced renal function and another had developed ESRD and received a renal transplant. CyA gave a 100% resolution of nephrotic-range proteinuria and a 100% renal survival rate without additional therapy after a mean follow-up of 6 years. Treatment of HSN with CyA is efficacious, safe and not inferior to MP.
Assuntos
Ciclosporina/uso terapêutico , Vasculite por IgA/tratamento farmacológico , Imunossupressores/uso terapêutico , Metilprednisolona/uso terapêutico , Nefrite/tratamento farmacológico , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Vasculite por IgA/complicações , Masculino , Nefrite/etiologiaRESUMO
Thrombocytopenia associated with ITP in children usually returns to normal within a couple of months. Drug therapies are required to treat hemorrhages in approximately one fifth of the patients. Intravenously infused immunoglobulin is effective more rapidly than corticosteroid therapy, but the treatment response is mostly temporary. Thrombocytopenia is alleviated by splenectomy in approx. 70% of chronic ITP patients. In the absence of primary disease causing thrombocytopenia, splenectomy has to be contemplated, if thrombocytopenia impairs the quality of life for more than one year. New drug therapies such as rituximab and growth factor products increasing thrombopoiesis may decrease the number of splenectomies.
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Púrpura Trombocitopênica Idiopática/terapia , Corticosteroides/uso terapêutico , Anticorpos Monoclonais Murinos/uso terapêutico , Criança , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Peptídeos e Proteínas de Sinalização Intercelular/uso terapêutico , Qualidade de Vida , Rituximab , EsplenectomiaRESUMO
Thalassemias are a group of autosomically recessively inherited diseases involving a decreased production of structurally normal hemoglobin chains. The clinical picture of thalassemias ranges from fatal to symptomless and is dependent on the extent of the gene defect. Hematological picture typically reveals a microcytic and hypochromic hemolytic anemia, which is mild with respect to microcytosis in heterozygotes and does not exhibit a lower-than-normal erythrocyte count. The recognition of thalassemia, even a mild one, is important with respect to genetic counseling, and its distinction from iron deficiency is essential. Special examinations are required only in special cases.
Assuntos
Talassemia/diagnóstico , Diagnóstico Diferencial , Contagem de Eritrócitos , Finlândia/epidemiologia , Aconselhamento Genético , Testes Hematológicos , Hemoglobinas , Humanos , Talassemia/sangue , Talassemia/epidemiologia , Talassemia/genéticaRESUMO
OBJECTIVE: To assess the risk factors for developing Henoch-Schönlein purpura nephritis (HSN) and to determine the time period when renal involvement is unlikely after the initial disease onset. DESIGN: A prospective study of 223 paediatric patients to examine renal manifestations of Henoch-Schönlein purpura (HSP). The patient's condition was monitored with five outpatient visits to the research centre and urine dipstick testing at home. RESULTS: HSN occurred in 102/223 (46%) patients, consisting of isolated haematuria in 14%, isolated proteinuria in 9%, both haematuria and proteinuria in 56%, nephrotic-range proteinuria in 20% and nephrotic-nephritic syndrome in 1%. The patients who developed HSN were significantly older than those who did not (8.2±3.8 vs 6.2±3.0 years, p<0.001, CI for the difference 1.1 to 2.9). Nephritis occurred a mean of 14 days after HSP diagnosis, and within 1 month in the majority of cases. The risk of developing HSN after 2 months was 2%. Prednisone prophylaxis did not affect the timing of the appearance of nephritis. The risk factors for developing nephritis were age over 8 years at onset (OR 2.7, p=0.002, CI 1.4 to 5.1), abdominal pain (OR 2.1, p=0.017, CI 1.1 to 3.7) and recurrence of HSP disease (OR 3.1, p=0.002, CI 1.5 to 6.3). Patients with two or three risk factors developed nephritis in 63% and 87% of cases, respectively. Laboratory tests or blood pressure measurement at onset did not predict the occurrence of nephritis. CONCLUSION: The authors recommend weekly home urine dipstick analyses for the first 2 months for patients with HSP. Patients with nephritis should be followed up for more than 6 months as well as the patients with HSP recurrence.
Assuntos
Vasculite por IgA/complicações , Nefrite/etiologia , Adolescente , Distribuição por Idade , Idade de Início , Criança , Pré-Escolar , Métodos Epidemiológicos , Glucocorticoides/uso terapêutico , Hematúria/etiologia , Humanos , Nefrite/diagnóstico , Nefrite/prevenção & controle , Prednisona/uso terapêutico , Prognóstico , Proteinúria/etiologia , Recidiva , Fatores de Tempo , Urinálise/métodosRESUMO
OBJECTIVE: To describe the extrarenal symptoms and clinical course of Henoch-Schönlein purpura (HSP). DESIGN: A prospective national multicentre trial with 6-month follow-up. Patients A total of 223 newly diagnosed paediatric HSP patients. RESULTS: Purpura was the initial symptom in 73% of the patients and was preceded by joint or gastrointestinal manifestations in the rest by a mean of 4 days. Joint symptoms, abdominal pain, melena, nephritis and recurrences occurred in 90%, 57%, 8%, 46% and 25% of the patients, respectively. Orchitis affected 17/122 (14%) of the boys. Seven patients developed protein-losing enteropathy characterised by abdominal pain, oedema and serum albumin under 30 g/l, and an additional 49 patients had subnormal albumin levels without any proteinuria. Positive fecal occult blood (26/117, 22%) and α1-antitrypsin (7/77, 9%) suggested mucosal injury even in the patients without gastrointestinal symptoms. HSP was often preceded by various bacterial, especially streptococcal (36%) and viral infections. Previous streptococcal infection did not induce changes in the level of complement component C3. Recurrences were more frequent in patients >8 years of age (OR 3.7, CI 2.0 to 7.0, p<0.001) and in patients with nephritis (OR 4.6, CI 2.3 to 8.9, p<0.001). Patients with severe HSP nephritis had more extrarenal symptoms up to 6 months. There was no difference in the clinical course between the prednisone-treated and non-treated patients during the 6-month follow-up. CONCLUSIONS: Serum albumin is often low in HSP patients without proteinuria, due to protein loss via the intestine. Although corticosteroids alleviate the symptoms, they seem not to alter the clinical course of HSP during 6 months of follow-up.
Assuntos
Glucocorticoides/uso terapêutico , Vasculite por IgA/tratamento farmacológico , Adolescente , Criança , Pré-Escolar , Ciclosporina/uso terapêutico , Métodos Epidemiológicos , Feminino , Gastroenteropatias/tratamento farmacológico , Gastroenteropatias/etiologia , Humanos , Vasculite por IgA/complicações , Vasculite por IgA/microbiologia , Lactente , Artropatias/tratamento farmacológico , Artropatias/etiologia , Masculino , Metilprednisolona/uso terapêutico , Orquite/tratamento farmacológico , Orquite/etiologia , Prednisona/uso terapêutico , Prognóstico , Recidiva , Albumina Sérica/metabolismo , Infecções Estreptocócicas/complicações , Resultado do Tratamento , Viroses/complicaçõesRESUMO
AIM: To explore whether early treatment of children with idiopathic thrombocytopenic purpura (ITP) with immunoglobulin and/or corticosteroids reduces subsequent morbidity. METHODS: Centres participating in a Nordic ITP study were divided according to whether they had treated more than 2/3, from 1/3 to 2/3, or less than 1/3 children within 14 days of diagnosis. The course of disease from 15 days to 6 months after diagnosis was compared for children managed at the three centre categories. The comparison was restricted to children in whom at least one platelet count <20x10(9)/l was measured, numbering 156, 143 and 84 in the three different categories, respectively. RESULTS: The three groups of children were clinically similar but were managed with initial treatment rates of 89%, 57% and 14%, respectively. By day 15, the platelet count had stabilised to >20x10(9)/l in 67%, 67% and 52% (p<0.05) and to >150x10(9)/l in 38%, 29% and 29% (p<0.20). At 1 month after diagnosis there was no difference in recovery rates. Chronic ITP developed in 27%, 22% and 25% in the three groups. During follow-up, one or more disease-related events occurred in 23%, 22% and 19%, with no difference in the average numbers of episodes with mucosal bleeding. Treatment courses were administered to 19%, 13% and 11%, respectively. CONCLUSION: Active treatment policies accelerated platelet recovery in children with short-lasting ITP but did not avert the development of chronic ITP and did not cause a reduction in morbidity during follow-up.
Assuntos
Corticosteroides/uso terapêutico , Imunoglobulinas Intravenosas/uso terapêutico , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Pré-Escolar , Feminino , Finlândia/epidemiologia , Hemorragia/etiologia , Humanos , Islândia/epidemiologia , Lactente , Recém-Nascido , Masculino , Contagem de Plaquetas , Estudos Prospectivos , Púrpura Trombocitopênica Idiopática/complicações , Púrpura Trombocitopênica Idiopática/epidemiologia , Países Escandinavos e Nórdicos/epidemiologia , Resultado do TratamentoRESUMO
The Nordic idiopathic thrombocytopenic purpura study data showed that morbidity occurred mainly in children with thrombocytopenia lasting >3 months, whereas, the risk period with platelet counts <20 x 10(9)/l was short and the number of bleeding events low in children with shorter disease duration. These brief, uneventful courses were predicted by developing a scoring system based on six clinical features: abrupt onset (weight 5), age <10 years (3), preceding infection (2), platelet count <5 x 10(9)/l, wet purpura (1) and male gender (1). The score was derived and validated in two different cohorts of children. High scores (10-14) clearly identified low-risk patients. The score provides valid prognostic information and may be useful in clinical decision-making.
Assuntos
Púrpura Trombocitopênica Idiopática/sangue , Fatores Etários , Criança , Feminino , Indicadores Básicos de Saúde , Humanos , Imunoglobulinas Intravenosas , Masculino , Razão de Chances , Contagem de Plaquetas , Prognóstico , Estudos Prospectivos , Medição de Risco/métodos , Fatores SexuaisRESUMO
AIM: To describe the management practices of newly diagnosed childhood idiopathic thrombocytopenic purpura (ITP) in the Nordic countries. METHODS: A prospective registration was done from 1998 to 2000, including all children with newly diagnosed ITP aged 0-14 years and at least one platelet count < 30 x 10(9)/L. RESULTS: 506 children from 98 departments were registered. A diagnostic bone marrow aspiration was obtained within 14 days in 33%. Platelet and/or red blood cell transfusion was given in 11%. 287 children (57%) received platelet-enhancing therapy with intravenous immune globulin (IVIG) or corticosteroids within 14 days of diagnosis, IVIG being the first line choice in over 90% of the cases. There were noticeable national differences in the management. The decision to start drug treatment within two days of diagnosis was influenced mainly by the platelet count. Neither early treatment nor response to treatment changed the risk of chronic disease. CONCLUSION: This study has shown a great variation in the management practices of children with newly diagnosed ITP. Prospective studies are required to produce evidence-based recommendations for this patient group.
Assuntos
Corticosteroides/uso terapêutico , Imunoglobulinas Intravenosas/uso terapêutico , Púrpura Trombocitopênica/tratamento farmacológico , Adolescente , Criança , Pré-Escolar , Dinamarca , Finlândia , Humanos , Islândia , Lactente , Noruega , Estudos Prospectivos , Púrpura Trombocitopênica/diagnóstico , SuéciaRESUMO
OBJECTIVE: To evaluate the efficacy of early prednisone therapy in preventing renal and treating extrarenal and renal symptoms in Henoch-Schönlein purpura (HSP) in a placebo-controlled trial. STUDY DESIGN: A total of 171 patients (84 treated with prednisone and 87 receiving placebo) were included and followed up for 6 months. The endpoints were renal involvement at 1, 3, and 6 months and healing of extrarenal symptoms. The analyses were performed on an intent-to-treat basis. RESULTS: Prednisone (1 mg/kg/day for 2 weeks, with weaning over the subsequent 2 weeks) was effective in reducing the intensity of abdominal pain (pain score, 2.5 vs 4.8; P = .029) and joint pain (4.6 vs 7.3; P = .030). Prednisone did not prevent the development of renal symptoms but was effective in treating them; renal symptoms resolved in 61% of the prednisone patients after treatment, compared with 34% of the placebo patients (difference = 27%; 95% confidence interval = 3% to 47%; P = .024). CONCLUSIONS: The general use of prednisone in HSP is not supported, but patients with disturbing symptoms may benefit from early treatment, because prednisone reduces extrarenal symptoms and is effective in altering (but not preventing) the course of renal involvement.
Assuntos
Anti-Inflamatórios/uso terapêutico , Vasculite por IgA/tratamento farmacológico , Prednisona/uso terapêutico , Dor Abdominal/diagnóstico , Dor Abdominal/epidemiologia , Adolescente , Anti-Inflamatórios/efeitos adversos , Artralgia/diagnóstico , Artralgia/epidemiologia , Criança , Método Duplo-Cego , Esquema de Medicação , Feminino , Humanos , Vasculite por IgA/epidemiologia , Nefropatias/diagnóstico , Nefropatias/epidemiologia , Masculino , Prednisona/efeitos adversos , Prevalência , Estudos Prospectivos , Fatores de Risco , Índice de Gravidade de Doença , Resultado do TratamentoRESUMO
AIM: To describe the epidemiology of idiopathic thrombocytopenic purpura (ITP) in the Nordic countries, to define clinical subgroups and to investigate factors predicting chronic disease. METHODS: A prospective registration was done from 1998 to 2000, including all children with newly diagnosed ITP aged 0-14 y and at least one platelet count <30 x 10(9)/l. RESULTS: 506 children were registered and 423 followed for 6 mo. The incidence was 4.8/10(5) per year. Most children were aged 0-7 y (78%), with a predominance of boys, while patients aged 8-14 y had equal representation of the two sexes. There were seasonal variations determined by variations in postinfectious cases with sudden onset. The platelet count was <10 x 10(9)/l in 58%, but bleeding manifestations were mild or moderate in 97%. The insidious form (symptoms for more than 2 wk) was more frequent in older children and girls, showed little seasonal variation, had milder manifestations and ran a chronic course in more than half the cases. Intracranial haemorrhages did not occur in the first 6 mo after diagnosis. Chronic ITP developed in 25%. The strongest predictor of chronic disease was insidious onset of symptoms (OR 5.97). CONCLUSION: In the Nordic countries, ITP mainly affects children aged 0-7 y, with a winter bulk of postinfectious cases superimposed on a steady occurrence of non-infectious cases. Clinically, it may be useful to distinguish between children with sudden versus insidious onset of symptoms rather than between different age groups.
Assuntos
Púrpura Trombocitopênica Idiopática/epidemiologia , Doença Aguda/epidemiologia , Adolescente , Criança , Pré-Escolar , Doença Crônica/epidemiologia , Feminino , Finlândia/epidemiologia , Hemorragia/epidemiologia , Hemorragia/etiologia , Humanos , Islândia/epidemiologia , Incidência , Lactente , Recém-Nascido , Masculino , Prognóstico , Estudos Prospectivos , Púrpura Trombocitopênica Idiopática/complicações , Púrpura Trombocitopênica Idiopática/diagnóstico , Fatores de Risco , Países Escandinavos e Nórdicos/epidemiologia , Estatísticas não ParamétricasRESUMO
OBJECTIVE: To determine the duration of the risk period with platelet counts <20 x 10(9)/L and the frequency of bleeding episodes in unselected children with idiopathic thrombocytopenic purpura (ITP). STUDY DESIGN: We established a registry for patients with newly diagnosed ITP in the five Nordic countries, enrolling children aged 0 to 14 years with platelet counts <30 x 10(9)/L. Treatment centers prospectively reported presenting features, management details, and disease-related events during the first six months after diagnosis. RESULTS: At presentation (n=501), more than half of the children had a platelet count <10 x 10(9)/L, but only 15 (3.0%) had a hemorrhage requiring blood transfusion. During follow-up of 409 patients, thrombocytopenia resolved uneventfully in 277. A risk period was present in 376 cases. Among 283 with self-limiting ITP, 26 were at risk >1 month and 25 had 30 events. Among 93 patients with chronic ITP, 73 were at risk >1 month and 44 had 111 events. Events occurred with an average frequency of 0.39 per month at risk. Life-threatening hemorrhages did not occur in the first six months after diagnosis. CONCLUSION: Most children with ITP are at risk for serious bleeding for less than one month. Continuing severe thrombocytopenia is associated with little morbidity, bleeding episodes being infrequent and very rarely serious.