Disseminated BCG infection resembling langerhans cell histiocytosis in an infant with severe combined immunodeficiency: a case report

We present a very rare congenital immunologic disease, severe combined immunodeficiency syndrome (SCID) in 6-months-old-boy with prolonged mucocutaneous candidiasis, severe anaemia, skin rash similar to the infiltrative eczema of Langerhans cell histiocytosis (LCH) and subcutaneous nodules with histiocytic infiltration. Laboratory findings show profound absence of humoral and cell-mediated immunity. Pathology specimens analysis of subcutaneous nodule revealed numerous S-100 protein and Cd1a negative histiocytes, occupied by BCG intracellular growth. Histopathology and immunohistochemistry confirmed the diagnosis of BCG dissemination. BCG vaccination in infants with SCID can lead to life threatening dissemination, resembling to the infiltrative eczema of LCH and may mislead the clinician.     

Brief report: Evaluation of an interactive intervention designed to reduce pediatric distress during radiation therapy procedures

OBJECTIVE: To evaluate the efficacy of an interactive intervention in reducing distress related to radiation therapy (RT) among pediatric cancer participants as measured by occurrence of sedation, observed behavioral distress (OBD), and heart rate (HR). METHODS: Seventy-nine children receiving RT simulation were assigned randomly to a STARBRIGHT Hospital Pals group (i.e., interactive intervention group; IG) or modified control group (MCG). The interactive intervention included filmed modeling, exposure to an interactive Barney character, and passive auditory distraction. RESULTS: Children in the IG experienced significantly lower HR when compared with MCG participants. No differences were found in terms of sedation or OBD. CONCLUSIONS: The interactive intervention was effective at reducing RT-related distress (as measured by HR) and would be a useful tool in pediatric radiation oncology settings.     

3Alpha,7alpha-dihydroxy-12-oxo-5beta-cholanate as blood-brain barrier permeator

The aim of the study was to test the efficacy of 3alpha,7alpha-dihydroxy-12-oxo-5beta-cholanate as a blood-brain barrier (BBB) permeator by examining its effect on quinine uptake into the central nervous system in rats, analgesic action of morphine, and on the sleeping time induced by pentobarbital. The obtained results indicate that sodium 3alpha,7alpha-dihydroxy-12-oxo-5beta-cholanate can be considered as modifier of BBB permeability, as it exhibited a promoting effect in all three tests. In the test of quinine uptake, methyl ester of 3alpha,7alpha-dihydroxy-12-oxo-5beta-cholanoic acid (included in the study for comparison) did not show a promoting effect, which can suggest its specific action.     

Urinary aquaporin-2 excretion in preterm and full-term neonates

The study was undertaken to define the role of aquaporin-2 (AQP2) in renal concentrating performance by measuring urinary AQP2 excretion and urine osmolality in healthy preterm and full-term neonates during early postnatal life. Random urine samples were obtained from 9 full-term newborn infants (mean birth weight 3,218 g, mean gestational age 39.2 weeks) at postnatal ages of 1, 3 and 5 days. Five premature infants with a mean birth weight of 1,570 g and mean gestational age of 30.6 weeks were studied at the end of the 1st week and then weekly up to the 6th week. Urine osmolality (Knauer osmometer), creatinine (modified Jaffé's method) and AQP2 concentrations (radioimmunoassay) were measured. In full-term neonates, urinary AQP2 excretion showed no consistent changes over the age period studied, while urine osmolality decreased significantly with advancing age. In premature infants, urinary AQP2 excretion remained practically unchanged during the first 4 weeks followed by an abrupt increase thereafter. Urine osmolality did not follow the developmental pattern of AQP2 excretion; its mean values varied only from 78 +/- 39 to 174 +/- 146 mosm/l during the experimental period. It is concluded that during the early postnatal period, urinary AQP2 excretion does not serve as a direct marker of the renal action of AVP and the renal capacity to concentrate urine.     

Expression of nerve growth factor in cerebrospinal fluid of congenital hydrocephalic and normal children

Cerebrospinal fluid (CSF) is secreted by the choroids plexuses and has the potential to act as a signaling pathway for physiological control as it has been demonstrated to contain molecules such as interleukins, leukoterins, neuropeptides, growth transforming factor-beta (TGF-beta) and nerve growth factor (NGF), which are present at specific times during development. In this study, CSF from hydrocephalic and normal children were analysed using SDS-PAGE followed by silver staining. In order to obtain semi-quantitative estimates of the relative amounts of 26 kDa protein, an image analyzer was used to determine the intensities of the band in the respective lanes in silver-stained gels. Quantification of the silver-stained gels from repeated experiments showed that the amount of 26 kDa protein was clearly increases in the hydrocephalic CSF when compared with the normal CSF. A Western blot analysis using anti-NGF antibody as a probe confirmed the presence of NGF. Using enzyme-linked immunosorbent assay (ELISA), it was shown that the level of NGF in the hydrocephalic CSF is higher than in normal CSF. It is concluded that NGF is not only a constant component of human CSF but could also be significantly involved in the pathophysiology of hydrocephalus.