A three-year old patient had ichthyosis and neutral lipid storage disease (Chanarin-Dorfman syndrome), characterized by congenital ichthyosiform erythroderma and leukocyte vacuoles. He did not show any of the internal system involvement that was found in previously described cases. Patients with this syndrome demonstrate a great variability of clinical involvement. The affliction may be very mild as in our case or it may lead to rapid death, as with the patient’s brother born a Harlequin baby, who only survived 5 days. Because of this wide spectrum of clinical variability mild cases might escape diagnosis. It is, therefore, suggested that every case of ichthyosis should have a peripheral blood smear evaluation with special attention to the morphology of the leukocytes. Our case clearly demonstrates the value of such a screening examination. Although the patient had been under treatment for almost 3 years, only screening examination of blood smears of all the patients with ichthyosis finally led to the correct diagnosis.

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Autoantibodies against bactericidal/permeability-increasing protein (BPI-ANCA) were found in patients with cystic fibrosis (CF). It is speculated that they represent a marker of the chronic endobronchial infection and sustained inflammatory response in CF. Our aim was to evaluate whether azithromycin (AZM), through its antiinflammatory effect, could affect the level of BPI-ANCA in CF patients. Eighteen patients with CF aged 5.5-36.3 years (median 15.1) were enrolled in a randomised, double-blind, placebo-controlled trial of AZM (250 mg twice a week to 10 patients) or placebo (8 patients) for 12 weeks. BPI-ANCA levels were recorded pre- and post-treatment and compared to a group of 18 matched healthy controls. Chi-square analysis, Kruskal-Wallis and Mann-Whitney tests were used to compare between the groups. Pre- and post-treatment values were compared using the Wilcoxon Signed-Ranked test. BPI-ANCA was found in 12 CF patients (67%) and four (22%) healthy subjects (P<0.001). The mean BPI-ANCA level was 3.94+/-6.15 U/ml (mean+/-SD) in healthy subjects and 38.11+/-42.34 U/ml in CF patients (P=0.023). The mean BPI-ANCA level was higher in patients with Pseudomonas aeruginosa compared to those without (64+/-35 U/ml and 25+/-41 U/ml respectively, P=0.032). No change in BPI-ANCA levels occurred in the AZM-treated patients [35 (0-127) U/ml (median (range) and 30 (0-120) U/ml, respectively] or in the placebo group [10 (0-66) U/ml and 13 (0-83) U/ml, respectively]. BPI-ANCA levels are significantly higher in patients with CF compared to healthy controls. BPIANCA levels are higher among patients colonised with P. aeruginosa. Twelve weeks of AZM therapy did not lower the BPI-ANCA level in patients with CF.

Vibrio vulnificus was isolated from blood and stool cultures from a 65-year-old man who had underlying alcoholic liver disease. The patient had eaten raw oysters the day before he became ill. To our knowledge, this is the first published report of isolation of the organism from stool in a patient with primary septicemia, and it provides support for epidemiologic studies suggesting that the infection is acquired through the gastrointestinal tract by eating raw seafood containing the organism. It was also possible, in this case, to demonstrate the presence of high antibody titers to the blood isolate by indirect immunofluorescence but not by agglutinating or vibriocidal tests.

ABSTRACT. Recent studies have shown 1,25(OH)zD~ receptor-mediated modulation of leukocyte proliferation, differentiation, and function. We examined the phagocytosis and killing of microorganisms by neutrophils and monocytes from five patients of three families with hereditary resistance to 1,25(OH)2D3. Phagocytosis of microorganisms by patients' neutrophils and monocytes was normal. However, defective neutrophil killing activity toward Candida albicans (30-40% of controls) was found in all patients. The killing of Staphylococcus aureus was normal. The neutrophil chemiluminescence, nitroblue tetrazolium (NBT) dye reduction, and the generation of superoxide ions and hydrogen peroxide by neutrophils and monocytes after induction by either soluble stimuli or zymozan particles, did not differ from those in controls. The neutrophil myeloperoxidase activity was also normal. Monocytes obtained from two patients of different families before long-term calcium infusion therapy and after they became normocalcemic, demonstrated a similar impaired fungicidal activity toward Saccharomyces cerevisiae, indicating that hypocalcemia itself was not the cause of the killing defect. However, the addition of the Ca+2 ionophore A23187 (1 p M ) to the test medium restored the monocyte fungicidal activity to normal. As patients' neutrophil cytosolic free calcium concentration was similar to that in controls, it is suggested that 1,25-(OH)2D3 exerts its effect on leukocyte function by a putative receptor-mediated regulation of subcellular calcium localization which may be important for fungicidal activity. (Pediatr Res 25276-279, 1989) Abbreviations 1,25(OH)zD3, 1,25-dehydroxyvitamin D3 TPA, 12-0-tetra-decanoyl-phorbol-13-acetate f-met-leu-phe, N-formyl-L-methionyl-L-phenylalanine NBT, nitroblue-tetrazolium Recently, it has been shown that receptors for 1,25-(OH)2D3, the hormonal form of vitamin D3, are present in cellular components of the immune system and that specific functional receptors for 1 ,25-(OH)2D3 mediate the effects of 1 ,25-(OH)2D3

Pediatricians in Israel, regardless of country of origin, medical school, or place of practice, are aware of the correct use of ORS but do not follow nutritional practices recommended recently by the AAP. These findings suggest that steps for implementing the guidelines are needed in Israel and most probably worldwide.

This study examined lipopolysaccharide (LPS) induced in vitro secretion of interleukin-1 (IL-1) by peripheral blood monocytes from pre-term infants with and without sepsis. Thirteen pre-term babies were tested; eight were completely healthy and five suffered from six episodes of sepsis. The latter group was tested both in the acute septic phase and in the convalescent period. IL-1 secretion by monocytes derived from septic pre-term infants was lower, but not significantly different from healthy pre-term infants (7.1 +/- 1.0 U/ml versus 8.1 +/- 0.9 U/ml, respectively). IL-1 secretion by monocytes of eight control full-term babies was in the same range (8.4 +/- 0.6 U/ml). In the convalescent period IL-1 secretion by monocytes from septic pre-term babies increased (9.0 +/- 0.3 U/ml) and was significantly higher than values measured during acute infection (p less than 0.05). Septic premature babies were also found to have higher absolute blood neutrophil concentration (p less than 0.001), but their body temperature did not increase along the infectious stage. The decreased secretion of IL-1 by monocytes from pre-term babies in the acute phase of infection compared to the convalescent period may have contributed to their inability to mount appropriate immunological as well as inflammatory responses. Sepsis promoting IL-1 production in vivo may have limited the monocytes' capacity for LPS stimulated IL-1 synthesis in vitro.