The Emergence of 5-Year-Olds' Behavioral Difficulties: Analyzing Risk and Protective Pathways in the United Kingdom and Germany.

This study aimed to advance our understanding of 5-year-olds' behavioral difficulties by modeling and testing both mediational protective and risk pathways simultaneously. Drawing on two national samples from different Western European countries-the United Kingdom (13,053) and Germany (2,022), the proposed model considered observed sensitive parental interactive behaviors and tested child vocabulary as protective pathways connecting parental education with children's behavioral outcomes; the risk pathways focused on negative parental disciplinary practices linking (low) parental education, parental distress, and children's difficult temperament to children's behavioral difficulties. Further, the tested model controlled for families' income as well as children's sex and formal child care attendance.

Review of antihemophilic factor injection for the routine prophylaxis of bleeding episodes and risk of joint damage in severe hemophilia A.

Individuals with severe factor VIII deficiency experience recurrent hemorrhages and develop progressive joint damage. Large retrospective, nonrandomized studies of patient cohorts followed over decades show that factor prophylaxis initiated at an early age before the onset of recurrent bleeding reduces the incidence of hemophilic arthropathy. Two recent prospective, multicenter, randomized trials conducted in Europe (the ESPRIT study) and the USA (the Joint Outcome Study) confirm the efficacy of prophylaxis in the prevention of hemarthroses and arthropathy.

Mutations in SLC29A3, encoding an equilibrative nucleoside transporter ENT3, cause a familial histiocytosis syndrome (Faisalabad histiocytosis) and familial Rosai-Dorfman disease.

The histiocytoses are a heterogeneous group of disorders characterised by an excessive number of histiocytes. In most cases the pathophysiology is unclear and treatment is nonspecific. Faisalabad histiocytosis (FHC) (MIM 602782) has been classed as an autosomal recessively inherited form of histiocytosis with similarities to Rosai-Dorfman disease (RDD) (also known as sinus histiocytosis with massive lymphadenopathy (SHML)).

Diagnostic pitfalls in acute leukemia.

In most children presenting with the common signs and symptoms of leukemia, the diagnosis is readily made. However, unusual features of the disease and the absence of abnormalities on the complete blood count may render the diagnosis problematic, especially if this malignancy is not suspected. The current report focuses on the unusual presentation of leukemia and the difficulties in the diagnosis of the malignancy.

Hereditary and familial syndromes of bone and blood. Genetic pathways, diagnostic pitfalls.

Numerous metabolic disorders, teratogenic agents, and in utero infections lead to congenital bone disease and malformation. This review focuses on hereditary and familial disorders of bone with particular emphasis on impaired hematopoiesis, myelofibrosis, pathologic fractures, and dysmorphology of the forearm and craniofacial structures. The severity of bone disease and marrow dysfunction of any given disorder may vary considerably from one affected individual to the next, and intrapersonal variability over time may be substantial as well.