Follow-up of serum KL-6 lung fibrosis biomarker levels in 173 patients with systemic sclerosis.

OBJECTIVES: A single-centre retrospective longitudinal study to investigate the predictive value of KL-6 serum levels for the outcome of interstitial lung fibrosis in a large systemic sclerosis (SSc) patient cohort. METHODS: ELISA tests for the mucin like glycoprotein KL-6 were performed in sera of 173 SSc patients. The clinical and laboratory data were evaluated by a standardised protocol of chest x-ray, lung function tests, echocardiography and high-resolution computed tomography. 158 patients were 29 ± 22 months later reinvestigated, 9 patients (2 lcSSc, 7 dcSSc) died from SSc-related causes, and 6 patients were lost to follow-up. RESULTS: Serum titer of KL-6 was negatively correlated with lung function parameters, independent of the time of investigation. There was a significantly higher probability of death among patients with high level of baseline KL-6. There was no statistically significant difference in the deterioration and improvement rates between groups with normal and elevated KL-6 level at study entry, even in patients in early phase of disease (disease duration <3 years). Serum levels of KL-6 significantly decreased in patients receiving cyclophosphamide treatment in spite of the fact that the spirometry results (FVC and DLCO) did not show a significant change. CONCLUSIONS: KL-6 can be used as a lung fibrosis severity marker, but its role as a marker for disease activity is questionable. Furthermore, following cyclophosphamide treatment serum KL-6 levels may decrease independently of the lung function parameters.

The relationship between pacifier use, bottle feeding and breast feeding.

OBJECTIVE: To investigate the influence of pacifier use and the introduction of formula milk on breast feeding. METHODS: The mothers of 356 healthy newborn infants who initiated breast feeding while in-patients were interviewed within 3 days of birth and later at 2 and 6 months postpartum. Information regarding previous birth, breast-feeding frequency, pacifier use and the adoption of formula milk were obtained. The data were analyzed using chi2 and Kruskal-Wallis tests. RESULTS: At the end of the second month, 264 (74%) of the mothers were still breast feeding; by the end of the sixth month this had fallen to 236 (66%). Among the 356 mothers, 152 had at least one previous infant, and 132 (86.8%) of them had breast fed a previous infant. Of the women who breast fed their previous infant for more than 2 months, 112 (84.8%) breast fed the current child until the end of the second month and 104 (78.7%) until the end of the sixth month after delivery. Of all investigated patients, 204 were primiparous (57.3%). Of these, 152 (74.5%) breast fed until the end of the second month, and 132 (64.7%) until the end of the sixth month after delivery. Amongthe 204 primiparae, 92 (45%) gave formula to their newborn infants. Forty-four of these 92 mothers (47.8%) had discontinued breast feeding by the end of the second month. Among the 356 newborn infants, 220 used pacifiers at the end of the second month. Within this group, 72 (33%) of the infants did not breast feed, while 148 (67%) did breast feed. Of all investigated mothers, 136 did not give pacifiers to their infants and only eight of these mothers (6%) had stopped breast feeding at the end of the second month. CONCLUSIONS: We found an inverse correlation between pacifier usage and breast feeding. Breast feeding was most common among multiparous mothers.

[Effectiveness of high-frequency oscillatory ventilation in the treatment of lung hypoplasia in connection with intrauterine growth retardation and maternal pre-eclampsia]. / Magas frekvenciás lélegeztetés alkalmazása anyai praeeclampsiához és intrauterin retardációhoz társuló tüdó-hypoplasia kezelésében.

The authors examine in a retrospective study the effectivity of high frequency oscillatory ventilation in group of intrauterine retardated babies with clinical signs of lung hypoplasia. During one year period they treated 12 intrauterine retarded patients with high frequency oscillatory ventilation because of failure of conventional ventilation. The indications were in seven cases the high pCO2 level and in five ones low saturation in spite of surfactant administration. The birth weight of babies were between 770 grams and 2150 grams, and theirs gestational age between 29 and 35 weeks. Four patients died. The causes of death were in two cases severe cardiomyopathy and at two patients intraventricular haemorrhage. Two survivors are suffering of bronchopulmonary dysplasia. They suggest that immediate high frequency ventilation reduces the period of mechanical ventilation and the number of residual picture. They suggest, that lung hypoplasia is an underestimated diagnosis in intrauterine retardated babies. If the cause of respiratory failure in cases complicated with maternal preeclampsia is not only hyaline membrane disease it is necessary to look for signs of lung hypoplasia and to follow the treatment with high frequency oscillatory ventilation.

[Atypical process of acute disturbance of liver function with severe thrombocytopenia in the third trimester]. / Atypischer Verlauf einer akuten Leberfunktionsstörung mit schwerer Thrombozytopenie im dritten Trimester.

The authors diagnosed disturbance of liver-function associated with severe thrombopenia in a pregnant woman in the third trimester. Principally, acute fatty liver of pregnancy can be characterized by existing symptoms, e.g. nausea, vomiting, epigastric pain, jaundice, hyperbilirubinemia, moderately elevated SGOT and SGPT levels, thrombopenia, leukocytosis, low fibrinogen level and disseminated intravascular coagulopathy, but hepatomegaly, purpura and petechia on lower and upper extremities, and high ALP and GGT levels during postpartum period do not confirm suspicion of this diagnosis. The present report draws attention to the difficulties of differential diagnosis of pregnancy-induced elevated liver enzymes diseases associated with low platelets, as there are several identical pathophysiological processes. Although causes and exact pathophysiology of disorders are unknown, similar symptoms during the process of diseases leave the question open whether they are different diseases or whether they are different manifestations of the same disease, and what kind of relationship exists between these diseases and preeclampsia. This case suggests careful evaluation of the whole clinical picture, moreover it is emphasized that prompt, aggressive treatment of hemostatic disturbance and the expeditious delivery can save maternal life.

["Maternal floor infarct", simultaneous manifestation of intrauterine fetal retardation and high maternal AFP level]. / "Maternal floor infarct", gleichzeitiges Auftreten von intrauteriner Fetusretardierung und hohem mütterlichen AFP-Spiegel.

High AFP level (386.9 ng/ml) at the 16th gestational week in a 23-year old pregnant woman was observed. Fetal malformations or maternal causes could not be detected. Monitoring of fetal development and that of the fetal heart rate showed a worsening intrauterine growth retardation (IUGR). Due to the chronic hypoxia and IUGR cesarean section was performed in the 32nd gestational week and a 960 g female newborn was delivered. Histological examination of the placenta showed signs of maternal floor infarct (MFI): intervillous fibrin netlike deposition with the increase of extravillous trophoblast (X cells) and septal cystic formation. High unexplained AFP level and IUGR can draw attention to the possibility of intrauterine fetal demise, which indicates intensive intrauterine fetal monitoring.

[Management of hemodynamically significant fetal arrhythmias]. / Hemodinamikailag jelentös magzati ritmuszavarok kezelése.

Between January 1, 1993, and April 30, 1996, authors treated 23 fetuses with severe rhythm disturbances in their Department. The correct diagnosis was made by fetal echocardiography. They had 15 tachyarrhythmic and 8 bradyarrhythmic patients. They found hydrops fetus at 7 patients because of atrial flutter (2 fetuses), supraventricular tachycardia (4 fetuses) and severe bradycardia (1 fetus). They treated successfully 13 patients with antiarrhythmic therapy given to the mother. They had 1 intrauterine death (treated because of bradycardia) and 1 neonatal death (hydropic because of supraventricular tachycardia). The causes of severe bradycardia were maternal antibody (3 fetuses), cardiac malformation (3 fetuses) and large number of blocked atrial extrasystoles. The prognosis of fetal tachycardia is good even in cases of fetal hydrops. The prognosis of bradycardia due to heart abnormalities is poor.

[The effect of the delivery method on the mortality of very low birth weight infants in case of breech presentation]. / A szülésvezetés módjának hatása az igen kis súlyú újszülöttek halálozására medencevégü fekvés esetén.

The authors have investigated the data of 1009 neonates born from breech presentation in the I. Department of Obstetrics and Gynecology of Semmelweis Medical School between January 1., 1990 and December 31., 1995. The frequency of caesarean section in this group was 75.5%. In the subgroup of very small neonates (those under the birthweight of 1500 grams) caesarean section was performed in 71.4%. In this group 73.8% of the neonates were alive on the 28th postpartum day, as opposed to 37.7% in the group spontaneously delivered. The difference proved to be significant in the group weighing between 750 and 1249 grams. Intraventricular haemorrhage was also more frequent in the group spontaneously delivered. The difference was not significant in the groups under 750 grams and between 1250 and 1500 grams birthweight. Based on their investigation the authors conclude, that in the group of fetuses whose estimated birthweight is predicted to be between 750 and 1250 grams by ultrasound examination, perinatal mortality and morbidity rates in breech presentation can be improved by performing caesarean section.

Very-low-birthweight breech infants: short-term outcome by method of delivery.

The purpose of this study was to determine the incidence of mortality and morbidity of the very-low-birthweight infant (< 1,500 g) in breech presentation based on mode of delivery and birthweight. A retrospective chart review of 1,009 infants who were in breech presentation at the time of delivery between January 1, 1990 and December 31, 1995 at the First Department of Obstetrics and Gynecology of Semmelweis Medical School in Budapest, Hungary. Data collected included birthweight, mode of delivery, pregnancy complications and neonatal mortality and morbidity. Comparison of groups was made based on mode of delivery, and data were analyzed using Fisher's exact test and chi-square analysis. For those infants weighing less than 1,500 g at birth, vaginal delivery was associated with higher mortality than for those delivered abdominally (73.8% vs. 37.7%, P < 0.001). There was no significant difference in survival for those infants weighing 1,500 g or more. Regarding morbidity, in those infants weighing less than 1,500 g, vaginal delivery was associated with a higher incidence of 1 min Apgar below 4 (21.7% vs. 5.2%, P < 0.001), a higher incidence of 5-min Apgar scores below 4 (11.6% vs. 1.2%, P < 0.001), a higher incidence of grade III or grade IV IVH (18.8% vs. 3.5%, P < 0.001) and a higher incidence of necrotizing enterocolitis (5.8% vs. 0.6%, P < 0.05). There is an increased incidence of mortality and morbidity for the VLBW breech infant delivered vaginally. Cesarean delivery may improve outcome for these infants.

[Correlation between neonatal mortality and hyperinsulinema]. / A neonatális morbiditás és a hyperinsulinaemia kapcsolata.

The authors investigated the correlation among the severity of maternal diabetes, the tight control of the maternal blood glucose concentration and the neonatal morbidity. Umbilical cord insulin level were measured in 115 newborns, 40 of them have diabetic mother. There were significant different in the serum insulin level between the low and high morbidity group. The authors suggest the importance of the tight blood glucose control during pregnancy, the diagnostic value of cardiological ultrasound to decrease the neonatal morbidity.

[Insulin concentration in amniotic fluid in mid-term pregnancy]. / A magzatvíz inzulintartalma középidös terhességben.

Insulin concentrations of 284 amniotic fluid samples were assessed, obtained from pregnant women by genetic amniocentesis between the 16-18 gestational weeks. The indication of amniocentesis was either advanced maternal age (> 35 years) or high risk genetic history. The amniotic fluid insulin concentration (mean +/- SD) was found 3.8 +/- 3.0 microIU/ml. The insulin concentration in 4.3% of all pregnant women was higher than the mean value +2 SD (9.8 microIU/ml) which represents individual pathological value, characteristic for gestational diabetes. Our data suggest that one part of gestational diabetes might be detected between the 16-18 gestational weeks, therefore we propose an earlier screening as the usual one. At institutes where the possibility of genetic amniocentesis is given the determination of insulin level in amniotic fluid may be reasonable. We could analyze the outcome of 230 pregnancies analysed. A close correlation was found between the amniotic fluid insulin concentration and birthweights of female newborns. It could not found such correlation at male newborn. A more careful screening test for gestational diabetes should be performed at mothers with female fetuses.

[A successfully treated case of congenital hydrops by Kell-alloimmunization]. / Kell-alloimmunizáció okozta congenitalis hydrops gyógyult esete.

The authors report a successfully treated case of congenital hydrops caused by Kell alloimmunization. It is very important the early proper therapy and to make difference between the non immune hydrops fetus and the hydrops caused by alloimmunization. It seems to be indicated--also in Hungary, as the other European countries--the introduction of the screening of irregular antibodies during the pregnancy.

Congenital osteogenesis imperfecta associated with arteriopathia calcificans infantum.

A 25-year-old mother during pregnancy was treated by accident with clomifen (Clostilbegyt). Ultrasound scan, performed in the 32nd gestational week, showed polyhydramnion and abnormally short, deformed fetal limbs. The newborn male infant died 20 minutes after caesarean section. Autopsy revealed osteogenesis imperfecta accompanied by a rare vascular alteration i.e. arteriopathia calcificans infantum. The possible relationship between the two diseases and estrogen therapy is discussed.