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Biomedical subjects

A Benderly

Publications and source records attributed to A Benderly.

At least 19 recordsLinked to original sources
PubMed1995

Dopa reaction of fetal melanocytes before and after skin transplantation on to nude mice.

We have previously demonstrated that human fetal epidermal melanocytes are dopa-negative. The present study was conducted to test the hypothesis that human fetal melanocytes can be activated to produce melanin under conditions differing from their natural in utero environment. To address this question, dopa staining activity of fetal epidermal sheets, obtained from seven aborted fetuses with estimated gestational ages of 13-20 weeks, was evaluated before and after engraftment on to nude mice. Dopa staining became positive 7 days post-engraftment. The intensity of the dopa reaction and the mean number of melanocytes increased by day 14 post-engraftment, and these changes were even greater by day 30. These observations indicate that human fetal melanocytes, potentially capable of synthesizing melanin under conditions differing from their normal in utero environment, are either inhibited, or not stimulated to do so.

Adult↗
PubMed1995

Use of saliva in home monitoring of carbamazepine levels.

Total carbamazepine (CBZ) levels in serum of 61 epileptic children were compared with saliva levels. Both resting and stimulated saliva was analyzed. The salivary levels were 38.6% of serum CBZ levels. A highly significant correlation was noted (r = 0.89, p < 0.001). Stimulation had no effect on saliva CBZ levels (r = 0.97). Salivary and serum CBZ levels were not affected by storing the samples for 7 days at room temperature. The data indicate that salivary CBZ may provide a reliable alternative monitoring method to Tegretol therapy, especially in children, in whom blood sampling is difficult. Furthermore, the samples may be collected at home and delivered to the laboratory by mail.

Adolescent↗
PubMed1994

Cholestatic pruritus: effect of phototherapy on pruritus and excretion of bile acids in urine.

Pruritus associated with hepatic cholestasis may cause significant morbidity and its correlation to retention of bile acids in skin is inconsistent. Available treatment modalities are only partially effective and can have several adverse effects. Phototherapy has recently been reported to improve cholestatic pruritus, but has not been evaluated previously in children, and its mechanism is still unclear. We report the outcome of multiple Daylite phototherapy treatments over two years in a seven-year-old child with chronic hepatic cholestasis that was resistant to other therapeutic modalities. Bile acid levels in urine were used as markers of effectiveness in parallel with clinical response. Night phototherapy alone increased the bile acids/creatinine ratio in urine from 1.54 +/- 0.04 mumol/mg at baseline to 2.07 +/- 0.29 mumol/mg. Continuous phototherapy combined with night diuresis raised the ratio further to 2.28 +/- 0.55 mumol/mg. Night diuresis alone had no effect. Continuous phototherapy combined with night diuresis raised the bile acids/creatinine ratio by 44% on the first day and by 61% on the second day, but declined to baseline on the third day of treatment. A marked clinical improvement was noted for one week following two days of phototherapy. This schedule has been repeatedly effective in improving pruritus for approximately one year and may be due to the ability of phototherapy to enhance excretion of bile acids and other possible pruritogens into urine.

Bile Acids and Salts↗
PubMed1991

High dose intravenous gamma-globulin in intractable epilepsy of childhood.

Eight children aged between 1.3 and 13 years suffering from epilepsy refractory to conventional anticonvulsive therapy were treated with high dose intravenous gamma globulin (200 mg/kg, 3 times per week, repeated after 3 weeks). Immunological studies after therapy showed normal results. In four children, clinical and EEG findings markedly improved. In one other case a partial response was noted. No improvement was observed in the remaining three cases. We confirm that although the mechanism is still obscure, high doses of i.v. gammaglobulin may have a beneficial effect in a significant number of children with intractable epilepsy.

Adolescent↗
PubMed1991

Dopa reaction test in hair bulbs of fetuses and its application to the prenatal diagnosis of albinism.

No information is available on the amount of tyrosinase normally present in fetuses. A dopa reaction test in hair bulbs from the scalp of normal fetuses obtained after abortion showed that tyrosinase is present in fetuses as early as 17 weeks. Only faint activity was detected in skin specimens other than from the scalp. This assay can serve as a quick and reliable method for the prenatal diagnosis of tyrosinase-negative albinism.

Albinism, Oculocutaneous↗
PubMed1991

Effect of urine and urine components on the chemiluminescent response of bacteria-stimulated polymorphonuclear leukocytes.

The role of polymorphonuclear leukocytes (PMN) found in urine during infectious episodes is still unknown. Opsonophagocytosis of Escherichia coli by normal blood PMN in the presence of urine was measured using a chemiluminescence (CL) assay. PMN were challenged by a type I fimbriated E. coli strain shown to elicit a CL response through attachment to the mannose-containing receptors on the leukocytes. In the presence of urine the CL response decreased significantly. Urine osmolality due to inorganic salts partially caused this decrease. A higher inhibitory effect was elicited by urea. Under otherwise similar conditions, the presence of an additional CL-inhibiting factor, most probably a protein, was detected in urine; however, its identity has not yet been defined. In vitro and in vivo urine dilution improved PMN function. No difference in effect on CL response was found between urine obtained from 25 children with recurrent urinary tract infections and urine from 15 age-matched controls.

Adolescent↗
PubMed1990

Serum lipoprotein profile in children with celiac disease.

Jejunal mucosa is responsible for the absorption of triglycerides and the production of lipoproteins [chylomicrons, very-low-density lipoprotein (VLDL), high-density lipoprotein (HDL)] and apolipoproteins (B-48, A-I, A-II, A-IV, C-II). Mucosal damage is known to cause fat malabsorption and probably also affects the serum lipid profile. To determine lipoprotein production in states of enterocyte dysfunction, we compared the serum lipid profiles in a group of 12 children with untreated celiac disease (flat jejunal mucosa) with the profiles in a control group of 10 children suffering from other intestinal diseases. Statistically significant differences were found in the following parameters (celiac versus control): plasma levels of triglycerides (70 versus 119 mg/dl), cholesterol content in LDL (107 versus 67.7 mg/dl), protein content in VLDL (6 versus 10 mg/dl), and level of apoprotein A-I (112 versus 140 mg/dl). No significant differences were found between the two groups in the serum levels of total cholesterol, the cholesterol content in VLDL and HDL, the protein content in LDL and HDL, and the level of apoprotein B. Following institution of a gluten-free diet, the lipoprotein profile reverted to normal. These data suggest that the changes in the serum lipoprotein profile in celiac disease are secondary to alterations in enterocyte function and not only a reflection of fat malabsorption.

Celiac Disease↗
PubMed1990

Effect of an intravenous gammaglobulin preparation on the opsonophagocytic activity of preterm serum against coagulase-negative staphylococci.

Recent reports have described cases of septicaemia caused by coagulase-negative staphylococci in preterm neonates, mainly due to the use of artificial intravenous devices. It was of interest to examine if intravenous immunoglobulin therapy, known to be effective in group B streptococcal infections of neonates, had a similar beneficial effect in coagulase-negative staphylococcal infections. Opsonophagocytosis of coagulase-negative staphylococci by normal polymorphonuclear leukocytes in the presence of cord blood serum supplemented with the commercial IgG preparation 'Sandoglobulin' was investigated, using luminol-dependent chemiluminescence. It was found that with two different coagulase-negative staphylococcal strains, Sandoglobulin had a concentration-dependent enhancing effect on the chemiluminescent response. This effect was demonstrated in the presence of native as well as inactivated cord blood serum and in the presence of sera from preterm infants (28-33 weeks). It is concluded that intravenous Sandoglobulin therapy may be effective in the treatment of preterm infants with severe coagulase-negative staphylococcal infections.

Drug Evaluation↗
PubMed1990

Saethre-Chotzen syndrome associated with defective neutrophil chemotaxis.

An 11-month-old male infant with Saethre-Chotzen syndrome and recurrent respiratory infections is described. Persistent extremely high leukocytosis warranted evaluation of neutrophil functions. It was found that the opsonophagocytic activity was normal, but neutrophil chemotaxis was markedly decreased. Further studies pointed to an intracellular neutrophil defect causing this motility dysfunction.

Acrocephalosyndactylia↗
PubMed1989

Defective leukocyte fungicidal activity in end-organ resistance to 1,25-dihydroxyvitamin D.

Recent studies have shown 1,25(OH)2D3 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 microM) 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.

Calcitriol↗
PubMed1989

Humoral and cellular immune dysfunction in a patient with Bloom's syndrome and recurrent infections.

Immunological evaluation of a patient with Bloom's syndrome (BS) who suffered from recurrent bacterial and fungal infections, revealed low serum levels of IgG and high levels of IgM accompanied by an elevated proportion of surface membrane IgM positive B-lymphocytes and a decreased proportion of IgG positive B-cells. In vitro IgG secretion was also reduced whereas IgM production was normal. Although proportions of T-cell subsets were normal and proliferative responses to T-cell mitogens were adequate, a defective regulatory T-cell function for the generation of IgG was observed. Natural killer (NK) cell activity against K562 tumor cells was also decreased in this patient. The findings in this patient may suggest a maturation arrest of lymphocytes at an early developmental stage, and this may explain in part the increased susceptibility to infections.

Adolescent↗
PubMed1989

[Kawasaki disease associated with Epstein-Barr virus].

We describe a 14-month-old child who for 3 weeks had high fever and developed lesions of the mucous membranes, enlarged lymph nodes, and an erythematous rash. Kawasaki disease was diagnosed and there was clear evidence for infection with Epstein-Barr virus. Thus it is possible that this virus is one of the etiological factors in the development of Kawasaki disease.

Herpesviridae Infections↗