Late spontaneous recovery of chronic thrombocytopenia.

Five cases are reported of spontaneous remission of chronic childhood thrombocytopenia four or more years after diagnosis. Other than typical features of chronic idiopathic thrombocytopenic purpura there were no obvious markers predictive of late remission, although a slow progressive recovery was common to four of the patients. In light of this experience splenectomy is not recommended in clinically mild thrombocytopenia.     

Late spontaneous recovery of chronic thrombocytopenia

Five cases are reported of spontaneous remission of chronic childhood thrombocytopenia four or more years after diagnosis. Other than typical features of chronic idiopathic thrombocytopenic purpura there were no obvious markers predictive of late remission, although a slow progressive recovery was common to four of the patients. In light of this experience splenectomy is not recommended in clinically mild thrombocytopenia.     

¿Cuál es el pronóstico de la trombopenia inmunitaria crónica?

ObjectivesTo analyse the characteristics of children with chronic ITP (chronic immune thrombocytopenia) in the Hospital Infantil Universitario Niño Jesús (HIUNJ) between 2003 and 2008. To also evaluate whether clinical variables as age, gender, initial platelet count, and treatment have any prognostic significance on the outcome of ITP.Patients and methodsData were retrospectively collected from 288 patients diagnosed with «Purpura and other haemorrhagic illnesses». Forty-two out of these 288 satisfied the criteria for «chronic ITP».ResultsTen patients out of 42 (23.8%) achieved remission with splenectomy, and 25 (almost 60%) achieved it without splenectomy (14 were complete remissions and 11 were partial remissions). Eight patients (almost 20% of patients with chronic ITP) had spontaneous remissions between 6 and 12 months from initial diagnosis. None of the clinical variables analysed were related to the outcome of the disease and the prognosis of the disease.ConclusionsAlmost 60% of children with chronic ITP achieve remission without treatment regardless of age, gender, initial treatment or platelet count. Splenectomy is one of the treatments with best results; however the high spontaneous recovery rate in children with cITP, the low percentage of bleeding, and the generally benign outcome should encourage delaying this as long as possible. As it is possible to have a remission between 6 and 12 months from the initial diagnosis, the term «chronic» should be reserved for patients with ITP lasting more than 1 year.     

Comments on problems of the antibiotic therapy of urinary tract infections in children (author's transl)

Basic principles of the antibiotic therapy of urinary tract infections in children are discussed with reference to the literature and own recent publications. In the field of pediatrics there seems to be no justification for a short-term therapy of so-called uncomplicated urinary tract infections as for example cystourethritis as has been propagated for adults because of the lack of reliable criteria to differentiate between infections of the upper or lower urinary tract. Only exception to this is the long-known cystourethritis of the young girl without previous renal disease. For some patients there is no doubt the chance of a spontaneous recovery from an acute pyelonephritis; nevertheless, each urinary tract infection in children should be treated properly since there is always the chance that an acute infections becomes chronic due to a therapy which was unspecific, too short, and without later check-ups. There is no alternative to the individually planned prophylaxis of reinfection in chronic pyelonephritis, including regular check-ups over a long period.     

Idiopathic thrombocytopenic purpura of childhood

Idiopathic thrombocytopenic purpura (ITP) is a benign hemorrhagic disorder characterised by peripheral thrombocytopenia and increased megakaryocytes in the bone marrow. The exact pathogenesis of ITP is not well understood. The adherence of viral induced immune complexes to the platelet membrane is thought to trigger the phagocytosis of damaged platelets by macrophages in the reticuloendothelial system. The role of platelet associated IgG in the pathogenesis of ITP is under investigation. Although spontaneous recovery is observed in 80–90% of patients, a short course of steroid therapy is recommended to reduce the duration of thrombocytopenia. The steroids however, have no influence on the course or outcome of the disease, and their possible role in reducing the incidence of intracranial hemorrhage (ICH) is unknown. Emergency management of patients presenting with signs and symptoms suggestive of ICH is essential to prevent the fatal outcome. Approximately 10–20% of patients develop chronic ITP. Splenectomy, considered the treatment of choice in these patients, is not always curative. The post-splenectomy sepsis also imposes a great risk for these individuals. Recent experience with intravenous immunoglobulin (IV IgG) treatment indicates that the splenectomy could safely be deferred, or even avoided in chronic ITP. The use of IV IgG in acute ITP is being investigated.     

Idiopathic thrombocytopenic purpura in children. Apropos of 98 cases

The authors report a retrospective study of 98 cases of idiopathic thrombocytopenic purpura in children. The sex ratio was 1/1 and the mean age 5 years. A history of viral infection preceded the purpura in 45% of the cases. Sudden onset was observed in 81%. The platelet count was less than 20,000 mm3 in 78.5%. A follow-up was obtained in 79 patients: 76% had an acute disease. A spontaneous remission occurred in 88.6% of the cases, most of them in the first six months (49 cases/68), and in 10 cases/68 between 7 and 11 months after onset. Six patients presented one or two relapses prior to recovery. The illness became chronic in 11% of the patients despite prednisone therapy. Four patients were treated by splenectomy, in 3 cases with success. Two children died, one girl from cerebral haemorrhage and one boy from septicemia.     

Long-term outcome of chronic idiopathic thrombocytopenic purpura in children

OBJECTIVES: Children with chronic idiopathic thrombocytopenic purpura (ITP) generally have a favorable outcome, but it is not known whether there are any prognostic factors to predict outcome. The objectives of this study were to assess the spontaneous remission rate and the prognostic significance of age, gender, initial platelet count, initial treatment, and response to treatment. METHODS: In this retrospective review of 62 consecutive children with chronic ITP, 37 were girls and 27 were 10 years of age or older (median age 9 years; range, 0.75-19). RESULTS: Thirty-five patients (56%) achieved spontaneous remission (remission without splenectomy), 30 of them (48%) within 4 years from diagnosis. Twenty-eight (45%) were complete remissions (platelet counts of >/=100,000) and 7 (11%) were partial remissions (50,000-99,000). There was no significant difference in the spontaneous remission rate between the younger (<10 years) and older children (55.8% vs. 57.1%, P = 0.95) or between boys and girls (56% vs. 56.7%, P = 0.98). Similarly, platelet count at initial diagnosis, initial therapy, or response to initial therapy did not have any prognostic significance. All 14 patients who underwent splenectomy achieved complete remission. CONCLUSIONS: More than 50% of children with chronic ITP achieve spontaneous remission. Age, gender, platelet count at initial diagnosis, initial treatment, and response to initial treatment do not have any prognostic significance toward the outcome of chronic ITP.     

Spontaneous dural sinus thrombosis in children

The clinical course of spontaneous dural sinus thrombosis in children varies from indolent to fulminant. Although many different etiologies for the development of dural sinus thrombosis have been described, a full recovery can be anticipated in most children following rehydration and the administration of systemic antibiotics. Steroids, systemic anticoagulation and intrasinus thrombolysis may be beneficial in selected patients, although the efficacy of these therapies has not been established prospectively in children. We reviewed 12 pediatric patients with spontaneous dural sinus thrombosis (1978-1998) to determine the etiology, clinical course and best treatment options. In the absence of a hypercoagulable state, pediatric patients generally recover well with rehydration and antibiotics and do not require anticoagulation.     

Is medical therapy effective for regional lymphadenitis following BCG vaccination?

We describe 120 patients with regional lymphadenitis following intradermal BCG vaccination. Seventy-eight of the patients were given medical therapy to prevent drainage and suppuration, and 42 patients were followed up without such treatment. The medical therapy group is divided into three subgroups: 36 were given erythromycin stearate, 21 isoniazid, and 21 isoniazid plus rifampin. No statistical difference in the incidence of spontaneous drainage and suppuration was found between the "no therapy" and the "medical therapy" groups. No significant superiority of any specific therapy was shown. If lymphadenitis develops rapidly (in two months), the incidence of spontaneous drainage and suppuration is significantly higher than in patients with slowly developing lesions. Total surgical excision is recommended to prevent spontaneous drainage and chronic suppuration in these rapidly evolving instances.     

Idiopathic thrombocytopenic purpura in children. The case for management without corticosteroids

Acute ITP in children under 13 years of age is generally a benign, self-limited condition with spontaneous recovery occurring within a matter of days or weeks. Our analysis of platelet data indicate no advantage in terms of rate of recovery when steroids are used. In fact, the median of 3 weeks and mean of 3 1/2 weeks from onset to recovery in the nonsteroid-treated children were significantly better than the corresponding figures in the steroid-treated group. In addition, while reducing the risk of intracranial hemorrhage is generally given as the chief therapeutic rationale for using steroids, we have not seen a single case of ICH among 465 consecutive cases of acute ITP in children, the majority (93%) of whom did not receive steroids. On the other hand, adolescents, as adults, with ITP often have the autoimmune (chronic) form of the disease. In this group, corticosteroids may be of at least transient benefit and should be used.     

Recurrent urticaria and angioedema

Urticaria is common in childhood, affecting up to 15% of British children. It is characterised by the sudden onset of wheals, angioedema, or both. Episodes are usually acute, often triggered by viral infections ± antibiotics, with approximately a third progressing to chronic or recurrent urticaria. This review covers urticaria subtypes, diagnosis and treatment options for children. The diagnosis is usually made clinically, and a focused history is vital. Further investigations are usually unnecessary. Chronic urticaria is divided into chronic spontaneous urticaria and the inducible urticarias. Chronic spontaneous urticaria is autoimmune in origin, in approximately 40% of older children. Cold urticaria and dermographism are the most common inducible urticarias. Isolated angioedema should prompt consideration of hereditary angioedema. The mainstay of treatment is non-sedating antihistamines, often with higher doses than standard and avoidance of triggers. Tranexamic acid may provide symptomatic relief for isolated angioedema. Treatment options include leukotriene receptor antagonists, anti-IgE therapy and systemic immunosuppression in unresponsive patients. Short courses of oral steroids may be used in acute episodes or highly symptomatic patients with chronic urticaria. Urticaria usually resolves; the vast majority of patients are disease free after 7 years.     

Parvovirus B19 infection associated with severe aplastic anemia in an immunocompetent patient

Human parvovirus B19 (PVB19) infection may cause mild pancytopenia characterized by transient and spontaneous recovery in healthy subjects. Severe aplastic anemia associated with PVB19 infection in patients without an underlying disease has been described in a number of reports. Here, a previous healthy, 10-year-old girl with severe aplastic anemia associated with PVB19 infection is described. The patient underwent bone marrow transplantation from her HLA-identical sibling resulting in complete recovery. PVB19 infection should be considered as one of the causes of aplastic anemia in patients without an underlying disease.     

High-Dose Intravenous Immunoglobulin as Single Therapy in a Child with Autoimmune Hemolytic Anemia

Between 1975 and 1992 450 children with idiopathic thrombocytopenic purpura (ITP) were diagnosed, and of those 100 (22%) developed the chronic form of the disease. Approximately half the patients with chronic ITP presented with mild to moderate hemorrhagic manifestations at the onset of purpura (30 cases) andlor later during the course of the disease (25 cases). The incidence of intracranial hemorrhage was 1 %, and the mortality rate due to overwhelming septicemia after splenectomy was also 1%. Overall one-third of the patients received no therapy; two-thirds of them went into spontaneous remission within 8 months to 8 years from the onset of ITP. Steroids given in conventional or high doses (51 cases) achieved a transient (if any) rise in platelet count, but in no case were steroids curative. Remission related to intravenous immune globulin (IVIG) therapy was noticed in 38.5% of the children (10 of 26) after variable courses. The response rate to splenectomy was 95.0%. Ultimately the long-term outcome in children with chronic ITP was as follows: remission, 58 cases (spontaneous, 30; after IVIG therapy, 10; after splenectomy, 18); hemostatic platelet values, 22 cases (spontaneous, 16; after IVIG, 5; after splenectomy, I). Thirteen children were lost in follow-up, and 7 remain thrombocytopenic but asymptomatic. These data indicate that chronic ITP in childhood runs a benign course in most cases and may remit with or without therapy euen several years from onset. Therefore, therapeutic intervention has to be individvalized, and splenectomy, which is not always safe, should be reserved for problematic cases that fail to respond to conventional therapeutic modalities.     

Relation of serum erythropoietin levels to renal excretory function: evidence for lowered set point for erythropoietin production in chronic renal failure

The relationship of serum erythropoietin (Ep) levels to hematocrit and glomerular (GFR) filtration rate was evaluated in patients with chronic renal disease. The Ep level was measured by radioimmunoassay in 119 blood samples from 48 patients obtained over a period of up to 5 years. Hematocrit values correlated significantly with the GFR, but serum Ep levels did not change with a decline in the GFR. Significant anemia was noted only when the GFR fell below 20 ml/min/1.73 m2. Episodes of spontaneous acute hypoxic stress were observed in six patients with chronic renal failure. Serum Ep levels obtained during these episodes (mean +/- SEM: 273 +/- 76 mU/ml) were tenfold higher than Ep levels during stable steady-state chronic renal failure (26 +/- 6 mU/ml), even though Ep levels were inappropriately low for the degree of anemia in the stable state. Our findings suggest that the tissue oxygenation-Ep-hematocrit feedback mechanism operates at a lower set point in patients with chronic renal failure in comparison with normal subjects.     

Autoimmune neutropenia of infancy

Neutrophil antibodies were demonstrated in 119 of 121 infants and young children with chronic neutropenia, establishing the diagnosis of autoimmune neutropenia of infancy. The median age at diagnosis was 8 months (range 3 to 30 months), and the female/male ratio was 6:4. Autoimmune neutropenia of infancy was manifested by recurrent fever and infection. All patients had selective neutropenia (absolute neutrophil count 0 to 500), and many had monocytosis. Fifteen of 16 patients tested failed to respond to epinephrine and hydrocortisone stimulation. Bone marrow had myeloid hyperplasia and reduced mature neutrophils. Recovery occurred in all 81 patients who passed the age of 5 years, except for one patient who is recovering at 6 1/2 years. The median age at recovery was 30 months; 95% recovered before 4 years. The estimated median duration of disease was 20 months. Neutrophil antibodies were detected early in the neutropenic phase by a combination of immunofluorescence and agglutination tests. Ten percent of these antibodies had specificity for NA1 or NA2. Ten of the 12 serum samples with a strong reaction in the flow cytometer reacted only with neutrophils. Two also reacted with an unidentified subpopulation (30%) of lymphocytes. Lymphocyte subsets were normal in 10 patients investigated, and abnormal levels of circulating immune complexes were detected in sera from 11 of 25 (44%) patients tested. Temporary remission was induced in all of eight patients who received intravenous IgG therapy. Autoimmune neutropenia of infancy is probably the most common cause of chronic neutropenia in infancy and early childhood, can be diagnosed by immunologic techniques, and requires only conservative management; spontaneous cure appears to be the rule.     

Presence of platelet antibodies in idiopathic thrombocytopenic purpura may discriminate acute from chronic disease

This study estimated the prevalence of serum antibodies against thrombocyte glycoproteins, at disease onset (54 patients) and later on during the course of the disease (71 patients), in sera from children with idiopathic thrombocytopenic purpura (ITP). Only a minority had serum antibodies at disease onset, with a significantly higher frequency in those who developed the acute form of the disease than in those who developed the chronic form. Serum antibodies may persist after spontaneous cure of acute disease. There was no switch from immunoglobulin M (IgM) to IgG antibodies over time. CONCLUSION: The pathogenesis of the acute and chronic forms of ITP may be different.     

Transient erythroblastopenia of childhood presenting with papilledema

A 4-year-old boy with transient erythroblastopenia of childhood (TEC) presented with papilledema and transient hemiparesis. Upon spontaneous hematologic recovery, the papilledema resolved. It is concluded that TEC, like other forms of anemia, may present with papilledema and even focal neurologic deficit, from which spontaneous recovery may be anticipated.     

Anaphylactic shock during elimination diets for severe atopic eczema.

Eighty patients with atopic eczema were treated with various elimination diets. Some or all foods were withdrawn then later reintroduced singly to the diet. In four patients reintroduction of a single food (soya, chicken, corn, cows'' milk) caused anaphylactic shock. In two patients spontaneous recovery occurred but in two resuscitation and intensive care were required. Anaphylaxis is a definite hazard of these elimination diets. Other than warning the parents, practical precautions are difficult because of the unpredictability of violent reactions and uncertainty about the life saving efficacy of injected adrenaline.     

Guidelines for therapy of idiopathic thrombocytopenic purpura (ITP) in childhood

The term "idiopathic thrombocytopenic purpura (ITP)" comprises a rather heterogeneous group of diseases with different etiology and pathogenesis. 80% of the cases are acute forms following viral infections. Within the first 6 months the spontaneous remission rate is higher than 80%. The remaining patients develop an intermittent or chronic form of the disease. Lethal complications, commonly cerebral hemorrhage, are rare (less than 2%). Following a review of the established and the recent experimental therapeutic approaches, the authors try to give comprehensive therapeutical guide-lines for the management of the various clinical forms of the disease. If there is only a minor bleeding diathesis, it is recommended to withhold therapy for 2 to 3 weeks, irrespective of the platelet count. If no spontaneous remission occurs, we suggest therapy with corticosteroids. In case of failure of this therapy, infusions of high-dose immunoglobulins are advisable. Short-lasting successes may even be prolonged with further infusions (once a week or at longer intervals)--thus postponing splenectomy. In addition, a combination of high-dose immunoglobulins and corticosteroids may be effective. Following pneumococcal vaccination and penicillin prophylaxis, splenectomy should be performed not earlier than one year after diagnosis of ITP and only in children older than 5 years. If splenectomy does not lead to recovery of the platelet count, immunoglobulin infusions--possibly combined with corticosteroids--may be repeated. Finally, if all the above proposed therapeutic actions have failed, immunosuppressive drugs (azathioprime or vincristine) may be necessary.(ABSTRACT TRUNCATED AT 250 WORDS)     

Spasticity of the lower limbs and the Weaver-Smith syndrome

The diagnosis of Weaver-Smith syndrome has been carried out on two patients with facial dysmorphic features, excessive growth and accelerated bone maturation. A marked spasticity of the lower limbs with joint contractures in one patient, a spastic quadriplegia with delayed milestones in the second patient were the most prominent clinical features. In both cases a spontaneous improvement of muscle tone with complete recovery was observed at the end of the first year of life.