IgA class antibody against human jejunum in sera of children with dermatitis herpetiformis.

Sera of 44 children with dermatitis herpetiformis with granular IgA deposits in the papillary dermis were investigated on cryostat sections of normal jejunum of three children aged 2 months, 1 year, and 10 years by indirect immunofluorescence. Eighteen of 25 patients on a normal diet had an IgA class antibody showing the following staining patterns on substrate jejunums: tubular positivity in the lamina propria--around the crypts, beneath the villous epithelial basement membrane, and in some instances in the middle of the villous also, following the capillary system of villi; coalescence of tubular positivity at the muscularis mucosae; and positive blood vessels and smooth muscle endomysium. Eleven of 18 children with positive sera were put on a gluten-free diet (GFD) and their sera became negative. One of these 11 patients was challenged with gluten and the antibody reappeared. Nineteen patients examined only on a GFD and 30 healthy blood donors did not have this antibody. There was no strict correlation between the titer of antibody and the severity of jejunal mucosal damage.

Immunoglobulin A deposition in jejunal mucosa of children with dermatitis herpetiformis.

Previously we have shown by indirect immunofluorescence (IF) technique that a special IgA antibody in the sera of patients with dermatitis herpetiformis (DH) binds to the structures of the normal jejunum. Now we show by direct IF that specific IgA deposits are present in the proximal jejunum of 11/12 DH and 2/2 celiac patients before a gluten-free diet (GFD). The IgA deposition was in a tubular pattern underlying the villous and crypt epithelial basement membranes and in the lamina propria. This IgA deposition diminished or was not detectable in DH patients under a GFD for a year, and became detectable under gluten challenge in three DH patients. One patient with celiac disease and IgA deficiency, four with other intestinal diseases, and four without jejunal damage had neither jejunal IgA deposition nor circulating IgA anti-jejunal antibody. The deposition of IgA in the jejunum seemed to be correlated with the presence of IgA anti-jejunal antibody in the serum and with the presence of jejunal damage, but the degree of jejunal atrophy, the titer of the anti-jejunal antibody, and the intensity of jejunal IgA deposition in DH patient were not clearly related. Deposition of IgA in the jejunum in DH did not clearly correlate with the activity of the skin symptoms and thus may not be directly related to the pathogenesis of the skin disease of DH.

Comparison of urapidil and atenolol in hypertension.

The antihypertensive efficacy of urapidil was compared with that of atenolol in a double-blind randomised parallel-group 10-week study. 44 patients with essential hypertension entered the study, and 43 with a supine DBP of 100 to 125 mm Hg after a 2-week placebo period were randomised either to urapidil (n = 22; 60 to 120 mg/day) or atenolol (n = 21; 50 to 100 mg/day) for 8 weeks. Blood pressure, heart rate, electrocardiogram (supine and standing) and side effects were recorded every week, and medical examinations and laboratory tests were done at the end of the placebo and active treatments. 36 patients completed the trial (17 on urapidil and 19 on atenolol). Patients' characteristics were similar in the two groups, and there were no differences between them in blood pressure and heart rate at the end of placebo period. Both drugs produced a significant fall in supine and standing blood pressure; urapidil decreased supine blood pressure from 164/109 to 150/96 (p less than 0.001), and atenolol decreased it from 167/111 to 146/94 mm Hg by week 8 (p less than 0.001). There were no differences in the blood pressure-reducing effects of the two drugs. There was no change in heart rate in the urapidil group, but a marked reduction occurred in the atenolol group, with significant differences from placebo and urapidil (p less than 0.001). Side effects were reported in 32% of the urapidil group and in 29% of the atenolol group; however, these were mild and transient in all but two patients (urapidil = 1, atenolol = 1) who were withdrawn.(ABSTRACT TRUNCATED AT 250 WORDS)

Antihypertensive effect of the calcium antagonist isradipine and lipid profile.

The hypothesis that plasma lipids may modulate the antihypertensive effect of the calcium antagonist isradipine was tested in 85 patients who had essential hypertension. Significant linear correlations were found between the antihypertensive effect of isradipine and plasma levels of total cholesterol and high-density lipoprotein (HDL2 or HDL3) in normotriglyceridemic (n = 63), but not in hypertriglyceridemic (n = 22), patients. From this, we conclude that normal levels of plasma lipids may modulate the function of calcium channels and their interaction with calcium antagonists.

Hungarian Isradipine Study (HIS): long-term (3-year) effects on blood pressure and plasma lipids.

These are the preliminary data of an open multicenter trial of antihypertensive treatment with isradipine as monotherapy (dose, 4.55 +/- 0.56 mg twice daily; n = 11) or isradipine (7.5 +/- 0.63 mg twice daily) in combination with bopindolol (1.16 +/- 0.12 mg once daily; n = 30) administered for 3 years to patients with essential hypertension (WHO classification I or II). Blood pressure was significantly decreased in both treatment groups and there was no indication of resistance to therapy. Plasma levels of total cholesterol and triglycerides were decreased by the end of the second year of treatment, and there was a tendency toward increase in plasma levels of high-density lipoprotein cholesterol (HDL2 or HDL3). The atherogenic index (ratio between total cholesterol and HDL2 plus HDL3) was also decreased. Blood glucose levels remained unchanged in both normoglycemic patients and those with non-insulin-dependent diabetes mellitus (NIDDM) during 3 years of therapy. It is concluded that isradipine is safe and effective when administered long-term in the treatment of hypertensive patients with either hyperlipidemia or NIDDM.

Gestational trophoblastic disease: treatment results at the Brewer Trophoblastic Disease Center.

Three hundred fifty-nine patients with gestational trophoblastic disease (choriocarcinoma and invasive mole) received complete treatment at the Brewer Trophoblastic Disease Center of Northwestern University Medical School from 1962 through 1978. Data were gathered as of December 31, 1978, to permit a minimum follow-up of 2 years. An overall remission rate of 92% was achieved: 100% (185/185) for nonmetastatic disease and 83% (144/174) for metastatic disease. All 200 patients with invasive mole and 129 of 159 patients (81%) with choriocarcinoma were cured. Chemotherapy was the main form of treatment, with adjuvant surgery and radiation therapy being used in selected patients. Five factors were determined to significantly influence response to treatment in patients with metastatic disease: 1) clinicopathologic diagnosis of choriocarcinoma versus invasive mole (71 versus 100%, P much less than .0005); 2) pretreatment human chorionic gonadotropin titer greater than 100,000 IU/liter and time greater than 4 months from pregnancy event to treatment (62 versus 93%, P much less than .0005); 3) metastases to sites other than lung and/or vagina (37 versus 92%, P much less than .0005); 4) antecedent term gestation compared with hydatidiform mole, abortion, and ectopic pregnancy (56 versus 79%, P less than .02); and 5) prior unsuccessful chemotherapy compared with no previous treatment (48 versus 83%, P much less than .0005). The value of secondary chemotherapy and adjuvant irradiation was evaluated. Relapse from remission was also studied.

Two-years' study with a combination of pindolol and clopamide ('Viskaldix') in patients with moderate hypertension.

A study was carried out to evaluate the long-term effects and side-effects of a combination product containing the beta-blocker pindolol (10 mg) and the diuretic clopamide (5 mg) in 15 patients with moderate hypertension. All patients completed the 2-years' study. The dose of the combination was increased until blood pressure normalized or a maximum dose of 3 tablets (equivalent to 30 mg pindolol and 15 mg clopamide) daily was reached. Blood pressure and heart rate were recorded monthly and detailed medical examinations were done regularly throughout the study. A mean dose of 2 tablets of the combination product (20 mg pindolol and 10 mg clopamide) produced a significant reduction in blood pressure. In all but 1 patient, blood pressure control was achieved and maintained. No tolerance developed. Heart volume showed a marked decrease. No side-effects of clinical importance were noted.

Antihypertensive effect of bopindolol: a multi-centre study.

The objective of the study was to investigate the efficacy of different dose levels of bopindolol monotherapy in hypertension. This potent nonselective beta-adrenergic receptor blocker has intrinsic sympathomimetic activity and long duration of action. Forty-four patients with essential hypertension of mild (n = 40) or moderate (n = 4) severity (90 less than DBP less than or equal to 115 mmHg at the end of the placebo period) entered and completed the single-blind, placebo-controlled trial. The study lasted 14 weeks: 2 weeks on placebo, and 12 weeks on active treatment during which the initial dose of bopindolol, 1 mg daily, was augmented up to 1.5 mg, then to 2 mg at four-week intervals until BP normalized or a maximum dose of 2 mg/day bopindolol was reached. The bopindolol was administered once a day in the morning. Patients were seen every other week in the morning before drug taking, when BP and heart rate, supine and standing, a twelve lead ECG and side-effects were recorded. Compared with placebo, supine BP was significantly reduced by bopindolol: from 169 +/- 2/103 +/- 1 mmHg to 148 +/- 3/92 +/- 1, 144 +/- 3/90 +/- 1 and 136 +/- 2/85 +/- 0.6 mmHg at the end of 4, 8 and 12 weeks of treatments, respectively (P less than 0.01 for each). BP changes during standing were similar. Bopindolol lowered the supine heart rate from 84 +/- 2 to 75 +/- 1, 74 +/- 1, 72 +/- 1 beats/min (P less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Costello syndrome with decreased glucose tolerance.

We report a case of Costello syndrome, which is an uncommon multisystemic condition with cutaneous manifestations on the palms and soles. In the literature there are 29 cases described, all the studies are published in the genetic literature with a few exceptions. We add a further case associated with impaired glucose tolerance. The diagnostic clinical signs are impressive, and highly characteristic. Cutaneous manifestations are: loose skin of the hands and feet "washer woman's hand", hyperkeratosis palmoplantaris, curly or sparse hair, acanthosis nigricans, papillomata nasi. Coarse, progeroid facial features with a bulbous nose, feeding difficulties in infancy, cardiac involvement with cardiomyopathy or conduction defect, and in our case impaired glucose tolerance also presented. Postnatal growth retardation, mental retardation, and a distinctive friendly personality is characteristic. Hyperextensible fingers with broad distal phalanges and joint contractures were observed, and peroneal hypertonicity required treatment by Achilles tendon lengthening. The decreased glucose tolerance is interesting in the view of the acanthosis nigricans. No storage disease and no chromosomal abnormality were observed. Only in one case is a balanced translocation described in the literature.

Gestational trophoblastic disease. The effect of duration of disease and hCG titer on response to therapy.

The height of the pretreatment hCG titer and the time interval from termination of the antecedent pregnancy to institution of treatment were determined in 352 patients with gestational trophoblastic disease in order to judge their effect, both individually and together, on response to therapy. When all patients in need of treatment for gestational trophoblastic disease, both metastatic and nonmetastatic, were considered as one group, examination of time alone, of hCG titer alone and of time and titer together each permitted the identification of patients at high risk with equal reliability (p less than 0.0005 for each). When patients with only metastatic gestational trophoblastic disease were evaluated, time and titer taken separately and together each identified those patients at high risk, but not in an equal manner (time alone, p = 0.02; titer alone, p less than 0.05; time and titer together, p less than 0.0005). Time and hCG titer, alone or in combination, did not have a statistically significant effect on outcome when patients with metastatic choriocarcinoma were considered separately. Other factors, such as metastatic site and antecedent pregnancy, seem to be more important in determining prognosis than duration of disease and hCG titer in this group of patients.

Discrete palmar and plantar symptoms in children with dermatitis herpetiformis Duhring.

Unusual, discrete palmar and plantar symptoms observed in thirty of forty-seven children with dermatitis herpetiformis are described. The diagnosis was verified in every case by the demonstration of granular IgA deposits in the skin. Forty-five of the children showed villous atrophy in jejunal biopsy specimens. In four cases extensive, exudative, bullous palmar symptoms, similar mild plantar changes, and healing with desquamation were observed. At least once during treatment we found very discrete, reddish-brown spots or small blisters on the flexor surface of the fingers and on the palms in thirty patients. Similar lesions occurred on the soles and plantar surface of the toes in only three patients. In asymptomatic patients and those treated with either a gluten-free diet or sulfone/sulfapyridine, the phenomenon was not manifest.

Ultrastructural features of canine kidneys after preservation with hyperthermic solutions and subsequent reperfusion.

An analysis of the ultrastructural features of 35 canine kidneys after hypothermic preservation in Rheomacrodex, Collins3 and Sacks II solutions, and subsequent autologous reperfusion for 2 to 3 hours is presented. Signs of irreversibility (intramitochondrial crista fragmentation, cell membrane rupture, detachment of the brush borders in the area of the proximal tubular system) were confined to the Rheomacrodex-preserved kidneys. Other abnormal features (cytoplasmic vacuolation, mitochondrial swelling in the proximal tubules, vacuolation of the visceral epithelial cells, heteromorphism in the glomerular system) found in the acute stage of revascularization may be regarded as reversible and account for the transitory functional disorders of renal transplants.

[Cefaclor therapy followed by serum sickness-like symptoms]. / Cefaclor kezelést követó szérumbetegség-szerú tünetek.

Cefaclor is an oral cefalosporin for a wide range of gram-positive and gram-negative infection. A retrospective study of cefaclor toxicoderma cases at the Department of Dermatology of Heim Pál Children's Hospital found 11 cases. After the administration of the drug, serum sickness like syndromes were observed. The characteristic symptoms were: erythema multiforme like urticaria, polyarticular swelling and oedema of joints and fever. Laboratory findings were: arised activity of the liver enzymes. After the acute symptoms urticaria factitia appeared.

[Organization of liver transplantation--role of the transplantation coordinator]. / A májtranszplantáció szervezése--a transzplantációs koordinátor feladatai.

The authors give a short overview about the background of the organisation of the liver transplantation and about the process of the "liver alarm". One of the most important work of the coordinator is to increase the number of organ donation. The other part of the preparation of liver transplantation is to create the recipient waiting list. The organisation of the "liver alarm" needs the work of two coordinators. The donor coordinator keeps contact with the donor hospital, harmonizes the travel of different transplant teams and organizes the organ explantation. In the same time, the recipient coordinator prepares the liver recipient for the liver transplantation and keeps contact with the donor coordinator in the donor hospital. If the donor liver macroscopically seems good for transplantation, the patient can be send to the operating room and the work of the anaesthesiologists and the surgeons starts.

[Comparative studies on freeze dryers of one and two chamber systems]. / Egy és kétkamrás liofilezö berendezés müködésének összehasonlító vizsgálata.

Authors carried out a comparative study on freeze--dryers of one--and two chamber-systems. Samples containing dextran and mannitol were used for the control of process-uniformity. Moisture content of the products were monitored during the sublimation process and after closing the ampules by colouring of the CoCl2 labelled samples. They put forward proposals for the combined measurement of barometric vapor pressure and product temperature in order to improve safety.

Factors associated with stable high blood pressure in adolescents.

Our aim was to study the relative importance of the factors associated with stable high blood pressure (BP) in adolescents. Out of 17 634 children born in 1964 and screened in 1977, two samples, an upper group [the upper 5% of the distribution curves for both systolic (SBP) and diastolic (DBP) blood pressures] and a lower group (a 10% random sample from the remainder), were chosen for yearly follow-up (1680 boys and 1143 girls) from 1978 to 1981. A multiple logistic function was used to analyse the factors associated with stable high SBP and DBP (with at least three different measurements of BP at or above the 95th percentile). Different sets of variables were studied. Of the boys with stable high SBP, 55% were found in the upper quintile of risk distribution when Quetelet's index was taken into consideration, 63% when Quetelet's index + age + heart rate+sexual maturation, 67% when Quetelet's index + age + heart rate + serum uric acid and 74% when Quetelet's index + age + heart rate + cardiothoracic index + heart volume index were taken into consideration. Of the girls with a stable high SBP, 68% were found in the upper quintile of risk distribution when any of the following variables was added to Quetelet's index + age + heart rate: R and S waves, serum cholesterol or post-load blood sugar. Similar but smaller effects of associated factors on DBP were observed. Our study shows that Quetelet's index and heart rate are the most important factors associated with stable high BP in adolescents.