Reflectance confocal microscopy as a non-invasive diagnostic tool for Hailey-Hailey disease.

BACKGROUND/PURPOSE: Reflectance confocal microscopy (RCM) is a non-invasive method for high-resolution, in vivo imaging of the epidermis and upper dermis. The purpose of the study was to evaluate the potential usefulness of RCM as a non-invasive diagnostic tool for Hailey-Hailey disease (familial benign chronic pemphigus). METHODS: Four patients with Hailey-Hailey disease were examined by RCM. Subsequently, punch biopsies were taken to compare RCM images with corresponding histopathologic findings. RESULTS: On RCM images, the most sticking feature was acantholysis at the level of the granular and spinous layer, resembling a 'dilapidated brick wall'. We suggest the term 'dilapidated brick wall RCM sign' to describe this feature and to distinguish from the corresponding histopathology finding. Other RCM features included: epidermal disarray, intraepidermal clefts, inflammatory cells in the epidermis and in the superficial dermis. These RCM abnormalities correlated with analogous histopathology findings. CONCLUSION: Reflectance confocal microscopy is a promising non-invasive diagnostic tool for Hailey-Hailey disease. The method may also be considered useful for choosing the best site for biopsy, which may aid pathology evaluation and spare time needed to establish the diagnosis.

'Black dots' seen under trichoscopy are not specific for alopecia areata.

BACKGROUND: 'Black dots' are macrocomedo-like round structures localized to the follicular ostium, and are considered a specific trichoscopic feature of alopecia areata (AA). AIM: To characterize specific features of 'black dots', and assess their possible presence in common hair and scalp disorders. METHODS: In total, 107 patients with hair loss [30 with alopecia areata (AA), 37 with androgenetic alopecia (AGA), 17 with chronic telogen effluvium (TE), 23 with other hair and scalp diseases] and 93 healthy controls were examined, using a videodermoscope with 20-70 times magnification. RESULTS: There was a correlation between the black dots and the early acute phase of the various alopecia types with the presence of the black dots. Black dots were found in 11% (22/107) of patients with hair loss, including 53.3% (16/30) with AA; in 40% (2/5) of patients with severe chemotherapy-induced alopecia, and in 100% of patients with dissecting cellulitis of the scalp (n = 2), hypotrichosis simplex (n = 1), and congenital aplasia cutis (n = 1). No black dots were seen in patients with AGA or TE. CONCLUSIONS: Black dots are not specific for AA, and may be present in other hair and scalp diseases.

Increased serum fractalkine in systemic sclerosis. Down-regulation by prostaglandin E1.

OBJECTIVES: To evaluate serum levels of fractalkine (FKN), a mediator of leukocyte transmigration, C-reactive protein (CRP) and expression of integrins CD11a and CD49d on peripheral blood lymphocytes in systemic sclerosis (SSc) and to investigate whether they are modulated by intravenous prostaglandin E1 (PGE1). METHODS: Serum levels of fractalkine and C-reactive protein and expression of CD11a and CD49d on peripheral blood lymphocytes were assessed in 50 SSc patients and in 18 healthy controls. In 25 SSc patients studied parameters were evaluated also after 3 consecutive daily PGE1 infusions (20 microg-40 microg-60 microg) and after 4 weeks. RESULTS: In SSc fractalkine basal level was significantly higher than in controls (9.04+/-1.79 ng/ml vs. 1.17+/-0.1 ng/ml; p<0.0001) and decreased significantly after PGE1 (5.16+/-1.27 ng/ml, p<0.05). After four weeks fractalkine level was still significantly lower than baseline 7.70+/-2.19 ng/ml (p<0.05). Basal percentage of CD11a (+) nor CD49d (+) lymphocytes in SSc (82.38+/-1.60%, 70.74+/-1.68%, respectively) did not differ from controls (85.73+/-2.04%, 75.62+/-2.48%; respectively, p>0.05). PGE1 treatment resulted in decrease of both CD11a (+) (67.72+/-3.34%, p<0.0001) and CD49d (+) lymphocytes (65.32+/-1.62%, p<0.0001). After 4 weeks the percentage of CD11a (+) and CD49d (+) lymphocytes remained significantly lower than at baseline (77.80+/-2.47% and 65.32+/-1.62%, respectively, both p<0.001). In SSc CRP basal level was significantly higher than in controls (4.70+/-2.01 mg/dl vs. 1.40+/-1.79 mg/dl, p<0.005) and reduced significantly after PGE1 (3.39+/-2.06 mg/dl, p<0.05). After 4 weeks, CRP level (4.38+/-2.19 ng/ml) was significantly lower than baseline (p<0.05). CONCLUSION: Fractalkine may play an important role in the pathogenesis of vascular dysfunction in systemic sclerosis. Prostaglandin E1 down-regulates serum fractalkine level, as well as CD11a and CD49d expression on peripheral blood lymphocytes, which suggests additional mechanisms in which this vasodilatatory agent exerts its efficacy in systemic sclerosis.

[Results of the activity of Jose Carreras Memorial Cord Blood Bank in Warsaw]. / Podsumowanie dzialalnosci Banku Krwi Pepowinowej im. Jose Carrerasa w Warszawie.

Following the first successful cord blood transplantation in 1988, several hundreds of patients were treated using same protocol. The main limitation of the wide use of cord blood as a source of haematopoietic cells is the number of available units of this tissue. To make possible selection of HLA-matched cells for individual patient, several thousands of cord blood samples must be collected and stored in liquid nitrogen. The network of cooperating cord blood banks with join accessible database is necessary. In this paper the activity of Jose Carreras memorial Cord Blood Bank established in Warsaw was described. Since the middle of January the collection of cord blood units for clinical purposes was started. During first three months 80 samples of cord blood was collected. Collections were obtained from normal full-term deliveries after the third stage of labour. For the banking the collection over 60 ml or contain over 4 x 10(8) of mononuclear cells were qualified. Whole blood samples and plasma samples obtained following volume reduction were used for HLA and bacteriology tests. After volume reduction the number of nucleated cells (WBC), mononuclear cells (MNC) and hematopoietic cells (CD34+) were evaluated. After processing the cord blood samples were frozen using control freezer and were stored in liquid nitrogen storage tanks. According to results of cord blood transplantation hundred percent of banked samples are suitable for recipients weighing 10 kg and only 7 percent for these weighing 50 kg.

[Comparison of the selected methods of cord processing for transplantation purposes]. / Porównanie wybranych metod preparatyki krwi pepowinowej przeznaczonej dla celów transplantacyjnych.

Human umbilical cord blood (UCB) has been successfully used as a source of allogeneic hematopoietic cells for transplantation. Banking of the UCB requires its volume reduction to decrease storage space, costs and volume of infused DMSO. In order to select an optimal method for volume reduction we compared several methods of cord blood processing, namely buffy coat centrifugation, red cell lysis, hydroxyethyl starch (HES)-, methylcellulose- and gelatin-sedimentations. The viability of cells and the recoveries of total white blood cells, mononuclear cells and CD34+ cells was evaluated. We also compared the efficacy of red cells depletion from the original UCB sample. Buffy coat centrifugation, red cell lysis, HES, gelatin or methylcellulose resulted in high mononuclear cell recoveries, whereas high hematopoietic cell recovery was observed only after HES sedimentation and buffy coat processing. The HES sedimentation procedure compared to buffy coat processing is more time and labor consuming and resulted in higher red blood cell and platelets depletion. Both methods can be recommended as a method at choice for the umbilical cord blood processing before banking.

[Evaluation of the intrauterine fetus in hypertension--biochemical assessment of amniotic fluid. IV. Ions]. / Ocena stanu wewnatrzmacicznego plodu w nadcisnieniu tetniczym krwi--poprzez badania biochemiczne plynu owodniowego. IV. Jony.

Sixty two samples of amniotic fluid, collected by ultrasound guided amniocentesis, were submitted to biochemical investigation including 31 samples from women with pregnancy complicated by hypertension (studied group) and 31 samples deriving from healthy pregnant women (control group with). Both investigated and control groups consisted of pregnant women with the same gestational age of 37 +/- 2 weeks (34-40 weeks). The following ions were measured: NH4+, K+, Na+, Cl-, Mg++, total Ca, Ca++ and inorganic phosphorus (Pi). The ionic composition of amniotic fluid deriving from women with pregnancy complicated by hypertension was normal. The regular concentration of NH4+ ions in amniotic fluids of a studied group may suggest kidneys of the fetuses in pregnancy complicated by hypertension were as mature as in the normal pregnancy within the same gestational age.

[Calcium-phosphorus-magnesium homeostasis in women with threatened abortion]. / Homeostaza wapniowo-fosforanowo-magnezowa u kobiet z poronieniem zagrazajacym.

One hundred twenty eight women were submitted to research including: 38 healthy not pregnant women, 40 healthy women in the first trimester of uncomplicated pregnancy (6-15 weeks) and 50 pregnant women with symptoms of threatened abortion (6-15 weeks). The following parameters were measured in serum: total Ca, Ca++, ionised inorganic phosphorus (Pi), magnesium (Mg), total protein and albumin and also total alkaline phosphatase activity (APt). Micromethods generally accepted in clinical laboratories were used. The study showed symptoms of threatened abortion are related to decreased concentrations of Pi, Mg, total protein and albumin and reduced activity of APt. Women who did not underwent miscarriage showed significantly higher Pi concentration compared to those who lost pregnancy, what might be of prognostic value. Threatened abortion was not considered to alter calcium homeostasis.

[Calcium, phosphorus and magnesium in pregnant women with primary hypertension]. / Wapn, forsfor i magnez u kobeit ciezarnych z nadcisnienien tetniczym krwi pierwotnym.

Sixty five women were in the third trimester of pregnancy (29-40 weeks of gestation) submitted to the study including 35 with primary hypertension (the studied group) and 30 healthy (control group). The following parameters were measured in blood serum and urine from 24 hrs, collection: total Ca and Ca++, inorganic phosphorus (Pi) and magnesium. Generally accepted micromethods were used; Ca++ was measured using AVL type 9140 analyser. Women of the studied group presented mean blood pressure 164 +/- 14/106 +/- 9.7 mm Hg and did not have proteinuria and oedema. They presented decreased concentrations of total Ca (p < 0.004) and ionised Ca++ (p < 0.004), and an increase of Pi (p < 0.002) in blood serum. No differences in magnesium concentrations were found. Distinct decrease of calcium excretion in urine was found in hypertensive women (4.50 +/- 2.76 vs 6.60 +/- 3.4 mmol/24 hrs, p < 0.024). No alterations in phosphorus and magnesium urine excretion were observed in women with hypertension (women of both groups had the same volume of 24 urine). Our study concludes the main alterations in calcium-phosphorus-magnesium homeostasis in pregnant women with primary hypertension are the calcium homeostasis alterations. Phosphorus homeostasis is less affected while magnesium distribution does not change. Hypocalciuria might be related to impaired glomerular filtration in this pathology in pregnancy. Hypocalciuria and lowered serum concentrations of total Ca and ionised Ca++ might prove general deficiency of this element in pregnancy complicated by primary hypertension.

[Evaluation of fetal condition in pregnancy complicated by hypertension--biochemical assessment of amniotic fluid. I. Protein and lipid components and kidney maturation]. / Ocena stanu wewnatrzmacicznego plodu w nadcisnieniu tetniczym krwi--poprzez badania biochemiczne plynu owodniowego. I. Skladniki bialkowe, lipidowe i dojrzalosci nerek.

Sixty two samples of amniotic fluid were submitted to biochemical investigation including 31 samples from women with pregnancy complicated by hypertension (studied group with mean blood pressure 165 +/- 15/95 +/- 5 mmHg) and 31 samples deriving from healthy pregnant women (control group with mean blood pressure 118 +/- 10/74 +/- +/- 9 mmHg). In the studied group pregnancy induced hypertension, primary hypertension and nephrogenic hypertension scored 58%, 32% and 10% of cases respectively. Both investigated and control groups consisted of pregnant women with the same gestational age of 37 +/- 2 weeks (34-40 weeks). The following parameters of amniotic fluid were measured: 1) total protein, 2) albumin, 3) beta-lipoprotein, 4) cholesterol, 5) uric acid, 6) urea, 7) creatinine. Micromethods were used. Amniotic fluid deriving from women with pregnancy complicated by hypertension showed decreased concentrations of total protein, albumin and beta-lipoprotein. In pregnancy complicated by hypertension kidneys of the fetuses were estimated to be as mature as in the normal pregnancy within the same gestational age.

[Evaluation of fetal condition in pregnancy complicated by hypertension--biochemical assessment of amniotic fluid. II. Enzymes]. / Ocena stanu wewnatrzmacicznego plodu w nadcisnieniu tetniczym krwi--poprzez badania biochemiczne plynu owodniowego. II. Enzymy.

Sixty two samples of amniotic fluid were submitted to biochemical investigation including 31 samples from women with pregnancy complicated by hypertension (studied group with blood pressure -65 +/- 15/95 +/- 5 mm Hg) and 31 samples deriving from healthy pregnant women (control group with mean blood pressure 118 +/- 10/74 +/- +/- 9 mm Hg). The following parameters of amniotic fluid were measured: 1) aminotransferases: alanine AlAT and aspartate AspAT, 2) alkaline phosphatase (APt) and its thermostable isoenzyme (APh), 3) ceruloplasmin (Crlp), 4) alpha-amylase (alpha-Amy). The study showed pregnancy complicated by hypertension is related to fetal salivary gland's immaturity presenting decreased activity of alpha amylase in amniotic fluid. Amniotic fluids deriving from women with pregnancy complicated by hypertension showed normal activities of AlAT, AspAT, APt, APh and Crlp.

[Evaluation of fetal condition in pregnancy complicated by hypertension--biochemical assessment of amniotic fluid. III. Acid-base balance]. / Ocena stanu wewnatrzmacicznego plodu w nadcisnieniu tetniczym krwi--poprzez badania biochemiczne plynu owodniowego. III. Równowaga kwasowo-zasadowa.

Sixty two samples of amniotic fluid, collected by ultrasound guided amniocentesis, were submitted to biochemical investigation including 31 samples from women with pregnancy complicated by hypertension (studied group) and 31 samples deriving from healthy pregnant women (control group with). The following parameters of acid-base balance were measured in amniotic fluids of both groups: pH, pCO2, base deficiency, standard HCO3 and total CO2. Corning device type 168 was used. Distinct metabolic-respiratory acidosis was present in amniotic fluids of studied group showing the decrease of a pH, pO2, standard HCO3 values and an increase of CO2 values and base deficiency. Authors believe in pregnancy complicated by hypertension biochemical environment of intrauterine fetal development with regard to acid-base balance is highly unfavourable.

Predictor of transplanted kidney deterioration following pregnancy--daily urine protein loss or serum creatinine concentration?

Increasing number of female kidney recipients deciding to born a baby makes us to investigate the impact of pregnancy on graft survival. The aim of study was to find the parameter that would have the predictive value for graft function following delivery. Scr and P in 33 pregnant renal allograft recipients (mean age 27.1 +/- 6.1 yrs) treated with pred + aza + CsA were studied for 6 mo before, during and 6 mo following delivery. As measured only by Scr graft function was stable in all pts (1.4 +/- 0.05 mg/dl). Significant rise in Scr following pregnancy was found in 6 of 33 pts. This "unstable" group was compared with 27 patients with "stable" despite pregnancy graft function. Proteinuria, but not Scr differentiated groups prior to pregnancy. The estimation of P prior to conception seems to be more potent parameter to predict kidney graft deterioration following pregnancy than Scr alone. Increase in P during and following pregnancy in "unstable" pts may reflect the acceleration of subclinical (not yet manifested with rise of Scr) chronic graft rejection due to pregnancy-induced hiperfiltration.

[Fetal metabolism in post term pregnancy based on biochemical investigations of amniotic fluid. IV. Ion composition]. / Metabolizm plodu w ciazy przeterminowanej--na podstawie badan biochemicznych plynu owodniowego. IV. Sklad jonowy.

Concentrations of calcium, sodium, magnesium, calcium (total and ionised) and inorganic phosphorus were determined in amniotic fluid. Samples were taken from patients between 42-43 week of gestation (investigated group) and between 38 and 40 week (control group). Micro-methods, generally acknowledged in laboratories of clinical biochemistry were used. In the protracted pregnancies increase of magnesium levels and decrease of sodium levels were observed. No differences in concentrations of calcium, calcium (total and ionised) and inorganic phosphorus between investigated and control group were noted. Independently of the duration of pregnancy, near term or post term, ionised calcium constituted 90% of total calcium in amniotic fluid.

[Fetal metabolism in post-term pregnancy based on biochemical examinations of amniotic fluid. V. Glucose, bilirubin, H+ ions, hormones and tests of lung maturation]. / Metabolizm plodu w ciazy przeterminowanej--na podstawie badan biochemicznych plynu owodniowego. V. Glukoza, bilirubina, jony H+, hormony i testy dojrzalosci pluc.

Concentrations of glucose, bilirubin, H+ ions, estriol, HPL were determined in the samples of amniotic fluid from post term and near term pregnancies. Investigated group consisted of samples taken between 42-43 week of gestation, control group between 38-40 week. In both groups biochemical tests of pulmonary maturation--Lectin/Sphingomyelin ratio, foam test, 650 nm absorbance were carried out. In the diagnosis of post term pregnancy glucose concentrations, oestrogens, HPL, 650 nm adsorption, foam test and L/S ratio had proper diagnostic value. No differences were observed in bilirubin concentrations.

[Kidney function in pregnant women with primary arterial hypertension]. / Czynnosc nerek u kobiet ciezarnych z nadcisnieniem tetniczym krwi pierwotnym.

Investigations covered 64 women in the III trimester of pregnancy. In this group 34 were with diagnosed primary arterial hypertension (examined group), and 30 were apparently healthy (control group). In both groups the blood serum concentrations of creatinine, urea, uric acid and electrolytes were determined. Creatinine clearance and acid-base balance were determined in these cases also. In 24 hours urine samples the NH4+, H+, Na+ and K+ ions concentrations were established. Impaired kidney function was shown in the patients from the examined group.

[Kidney function in pregnant women with hypertension in the course of chronic kidney disease]. / Czynnosc nerek u kobiet ciezarnych z nadcisnieniem tetniczym krwi w przebiegu przewleklych chorób nerek.

In 53 pregnant patients in the III trimester of pregnancy kidney function investigations were carried. The group consisted of 23 patients with chronic kidney diseases with superimposed arterial hypertension (examined group) and of 30 healthy pregnant women (control group). In the examined group an increase of blood-serum urea, uric acid and creatinine concentrations were demonstrated. In these women the blood pH was decreased also. The urinary excretion of NH4+ and H+ ions was decreased, the excretion of Na+ and K+ was normal.

[Alpha-amylase in serum and urine of women with hypertension during the III trimester of pregnancy]. / Alfa-amylaza w surowicy krwi i w moczu u kobiet z nadcisnieniem tetniczym krwi w III trymestrze ciazy.

The urinary and blood serum activity of alpha-amylase was tested in 34 women with arterial hypertension in the III trimester of pregnancy (examined group). The results were compared with a control group of healthy women (35 urine samples and 108 blood-serum samples). In both groups other biochemical examinations were carried additionally (the serum creatinine, urea, uric acid, electrolytes and the acid-base balance levels were established). In the examined group an increased activity of the alpha-amylase in the blood serum was demonstrated.