Symptomatic hypogammaglobulinemia in infancy and childhood – clinical outcome and in vitro immune responses

BACKGROUND: Symptomatic hypogammaglobulinemia in infancy and childhood (SHIC), may be an early manifestation of a primary immunodeficiency or a maturational delay in the normal production of immunoglobulins (Ig). We aimed to evaluate the natural course of SHIC and correlate in vitro lymphoproliferative and secretory responses with recovery of immunoglobulin values and clinical resolution. METHODS: Children, older than 1 year of age, referred to our specialist clinic because of recurrent infections and serum immunoglobulin (Ig) levels 2 SD below the mean for age, were followed for a period of 8 years. Patient with any known familial, clinical or laboratory evidence of cellular immunodeficiency or other immunodeficiency syndromes were excluded from this cohort. Evaluation at 6- to 12-months intervals continued up to 1 year after resolution of symptoms. In a subgroup of patients, in vitro lymphocyte proliferation and Ig secretion in response to mitogens was performed. RESULTS: 32 children, 24 (75%) males, 8 (25%) females, mean age 3.4 years fulfilled the inclusion criteria. Clinical presentation: ENT infections 69%, respiratory 81%, diarrhea 12.5%. During follow-up, 17 (53%) normalized serum Ig levels and were diagnosed as transient hypogammaglobulinemia of infancy (THGI). THGI patients did not differ clinically or demographically from non-transient patients, both having a benign clinical outcome. In vitro Ig secretory responses, were lower in hypogammaglobulinemic, compared to normal children and did not normalize concomitantly with serum Ig's in THGI patients. CONCLUSIONS: The majority of children with SHIC in the first decade of life have THGI. Resolution of symptoms as well as normalization of Ig values may be delayed, but overall the clinical outcome is good and the clinical course benign

The Evolution of Compact Binary Star Systems

We review the formation and evolution of compact binary stars consisting of white dwarfs (WDs), neutron stars (NSs), and black holes (BHs). Binary NSs and BHs are thought to be the primary astrophysical sources of gravitational waves (GWs) within the frequency band of ground-based detectors, while compact binaries of WDs are important sources of GWs at lower frequencies to be covered by space interferometers (LISA). Major uncertainties in the current understanding of properties of NSs and BHs most relevant to the GW studies are discussed, including the treatment of the natal kicks which compact stellar remnants acquire during the core collapse of massive stars and the common envelope phase of binary evolution. We discuss the coalescence rates of binary NSs and BHs and prospects for their detections, the formation and evolution of binary WDs and their observational manifestations. Special attention is given to AM CVn-stars -- compact binaries in which the Roche lobe is filled by another WD or a low-mass partially degenerate helium-star, as these stars are thought to be the best LISA verification binary GW sources.Comment: 105 pages, 18 figure

Autocrine PDGF stimulation in malignancies

Platelet-derived growth factor (PDGF) isoforms are important mitogens for different types of mesenchymal cells, which have important functions during the embryonal development and in the adult during wound healing and tissue homeostasis. In tumors, PDGF isoforms are often over-expressed and contribute to the growth of both normal and malignant cells. This review focuses on tumors expressing PDGF isoforms together with their tyrosine kinase receptors, thus resulting in autocrine stimulation of growth and survival. Patients with such tumors could benefit from treatment with inhibitors of either PDGF or PDGF receptors

ICAR: endoscopic skull‐base surgery

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Infants

Objective: Scintigraphy with Tc-99m dimercaptosuccinic acid (DMSA) is considered a reference method for the assessment of renal parenchymal lesions and estimation of differential renal function (DRF). Compared with Tc-99m mercaptoacetyltriglycin (MAG3), the disadvantages of DMSA are relatively higher radiation exposure to the kidney and longer procedural time. The aim of this study was to evaluate the performance of Tc-99m MAG3 dynamic renal scintigraphy in the detection of renal parenchymal defects and in the estimation of DRF in comparison with Tc-99m DMSA scintigraphy. Material and Methods: A retrospective review of the records indicated that 29 infants (20 boys and 9 girls) aged less than I year underwent both DMSA and MAG3 scintigraphies. The parenchymal phases of MAG3 scintigraphy were compared to DMSA images. Differential function was calculated based on the MAG3 and DMSA methods and the results were compared. Results: The findings of the two methods corresponded completely in 90% of patients. There was no significant difference between calculated DRF from DMSA and MAG3 images. There was a high correlation between the DRF obtained using the two methods (r = 0.91 and r = 0.90 for the left and right kidney, p < 0.01). The sensitivity and specificity of the MAG3 cortical images were calculated as 92% and 78%. Conclusion: We suggest that either a MAG3 or a DMSA scan can be used for the calculation of DRF. However, because of the low specificity of MAG3 cortical analysis, DMSA scintigraphy is required for the definitive diagnosis and management of renal cortical lesions

Infants

Objective: Scintigraphy with Tc-99m dimercaptosuccinic acid (DMSA) is considered a reference method for the assessment of renal parenchymal lesions and estimation of differential renal function (DRF). Compared with Tc-99m mercaptoacetyltriglycin (MAG3), the disadvantages of DMSA are relatively higher radiation exposure to the kidney and longer procedural time. The aim of this study was to evaluate the performance of Tc-99m MAG3 dynamic renal scintigraphy in the detection of renal parenchymal defects and in the estimation of DRF in comparison with Tc-99m DMSA scintigraphy. Material and Methods: A retrospective review of the records indicated that 29 infants (20 boys and 9 girls) aged less than I year underwent both DMSA and MAG3 scintigraphies. The parenchymal phases of MAG3 scintigraphy were compared to DMSA images. Differential function was calculated based on the MAG3 and DMSA methods and the results were compared. Results: The findings of the two methods corresponded completely in 90% of patients. There was no significant difference between calculated DRF from DMSA and MAG3 images. There was a high correlation between the DRF obtained using the two methods (r = 0.91 and r = 0.90 for the left and right kidney, p < 0.01). The sensitivity and specificity of the MAG3 cortical images were calculated as 92% and 78%. Conclusion: We suggest that either a MAG3 or a DMSA scan can be used for the calculation of DRF. However, because of the low specificity of MAG3 cortical analysis, DMSA scintigraphy is required for the definitive diagnosis and management of renal cortical lesions

Transfusion: Case Report

The principal indications for exchange transfusion are hemolytic diseases of the newborn with hyperbilirubinemia. However, there are some potential complications of exchange transfusion such as infection, coagulopathies (i.e., thrombocytopenia), electrolyte abnormalities (i.e., hypocalcemia), metabolic acidosis, hypoglycemia, and necrotizing enterocolitis. Stored blood develops some platelet-white cell microaggregates. These microaggregates or blood debris have been found to produce changes in pulmonary hemodynamics in animals and have been implicated in post-traumatic pulmonary insufficiency in man. Authors suggested that pulmonary gas exchange alterations following blood transfusion were primarily due to increased dead-space ventilation secondary to vasoconstriction and occlusion of the pulmonary microvasculature because of microaggregates. In this article, a newborn with transient pulmonary perfusion abnormality who had massive exchange transfusion for Rh isoimmunization and hyperbilirubinemia was reported