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The discovery of MODY (Maturity-Onset Diabetes of the Young) and the elucidation of its heritability enabled more precise clinical characteristics of different MODY subtypes and led to understanding that glucokinase MODY (GCK-MODY) is not associated with vascular complications in long term follow-up, whereas MODY of transcription factors (e.g. HNF1A-MODY) is in case of bad metabolic control connected with the acceleration of particularly microvascular complications. There is a strong evidence of the needlessness of any specific antidiabetic treatment in prognosticaly favourable GCK-MODY (except for the pregnancy). On the contrary, in MODY of transcription factors, including the most common one - HNF1A-MODY, the treatment, traditionally based on sulphonylurea derivatives (and after their failure on insulin) is required. Due to wider spectrum of available antidiabetic agents offering individualization of the treatment, the question of efficacy of other antidiabetic agents in MODY patients arises. This review article summarizes current knowledge of therapeutic options in patients with MODY.Key words: insulin - MODY - oral hypoglycemic drugs - treatment.

In spite of progress in medicine, studies from a number of countries indicate steadily increased risk of perinatal morbidity and mortality in the offspring of diabetic mothers. No data regarding the pregnancy outcome in women with diabetes mellitus type 1 and 2 (pregestational DM) have been published in the Czech Republic. The aim of the study was to evaluate the pregnancy course of women with pregestational DM and outcome of their offspring and to assess whether it has improved in ten years.A retrospective evaluation of pregnancy outcome of pregestational DM women followed up in the University Hospital Pilsen in years 2000-2009 (Group A, n = 107) and comparison with the period 1990-1997 (Group B, n = 39) were performed. Wilcoxon non-paired test, contingency tables, step-wise logistic regression and step-wise linear multiple regression methods were used for statistical analyses.Data is presented as median (interquartile range). Women from the Group A were older 28 (25, 31) vs 25 (22, 27) years, p = 0.01. Otherwise, the groups did not statistically significantly differ in diabetes duration, BMI, and representation of women with type 2 diabetes. A better glycemic control (HbA1c, mmol/mol) was achieved in the Group A in all trimesters - 1st trimester: 59 (47, 67) vs 66 (56, 76), 2nd trimester: 46 (40, 52) vs 54 (48, 59) and 3rd trimester: 46 (40, 51) vs 53 (47, 60), p = 0.01. The caesarean section rate decreased (65.2 % vs 87.5 %, p0.05). The incidence of the respiratory distress syndrome after adjustment for age and diabetes duration also decreased (8.9 % vs 18.2 %, p0.05). A decreasing trend in the rate of premature delivery before 34th week of gestation (1.1 % vs 6.3 %) and neonatal mortality (1.1 % vs 2.9 %) was observed, however, the differences were not statistically significant.The achieved improved glycemic control led to only a partial improvement in the course of pregnancy and outcome of the offspring of diabetic mothers.

The authors describe a case of a female patient with diabetes mellitus (DM) type 1 who faced a progress of diabetic retinopathy (DR) during her pregnancy with a development of diabetic macular oedema (DMO) and a deterioration of visual acuity (VA) in the right eye. The patient had been under observation for DM for 18 years, the last six years for the onset of the non-proliferative form of DR. During the 28th week of pregnancy, a significant reduction of visual acuity in the patient's right eye occurred as a result of a fast developing DMO. The patient was generally given corticosteroids for a gynaecological indication to accelerate the maturing of the foetus. Betamethasone (Diprophos) in dose 12 mg with intramuscular application was administered twice in total, i.e. in the 29th and the 32rd week of pregnancy. After each application, a good effect was observed on the improvement of the visual acuity accompanied by a DMO reduction. However, the effect of corticosteroids was only temporary and at the end of their application in each case, a fast reduction of VA and a progression of DMO were observed, even though retinal laser photocoagulation was initiated. Delivery via Caesarean section was indicated in the 35th week of pregnancy, after a consultation with a diabetologist, gynaecologist and ophthalmologist. The delivery went without complications; both the child and the mother were in a normal condition after the delivery. A gradual improvement in VA was observed during the post-delivery period, with a reduction of DMO until a level of VA that corresponded to the status before the pregnancy was reached.

The authors demonstrate two cases of caudal regression syndrome (CRS), a rare malformative syndrom, seen mainly in cases of maternal diabetes with poor metabolic control.Case report.Department of Obstetrics and Gynecology, Department of Medicine Regional Hospital Pardubice.The caudal regression syndrome (CRS) was revealed in two women with praegestational diabetes. The diagnosis was made at 18 and 20 weeks. The characteristic ultrasound findings include abrupt interruption of the spine and abnormal position of the lower limbs. The femur bones are fixed in a "V" pattern, giving a typical "Buddha's poise". A complete examination must be conducted for possible urinary and intestinal malformations. The mechanism leading to malformation is discussed in the article. To prevent pregnancy at the time of bad controlled diabetes is the only way to minimaze the risk of producing a congenitally malformed baby including caudal regression syndrom in the population of diabetic mothers. Family planning and supervision by the specialists is always advisable.Early diagnosis of CRS is possible using vaginal ultrasound. Emphasis is placed on the association of abrupt disruption of dorsal or lumbar spine and abnormal images of the lower limbs fixed in a,,V" formation, which is characteristic sign of CRS.

In recent years a very marked drop of complications in mothers with type 1 diabetes (DM 1) and type 2 diabetes (DM 2) was achieved. Nevertheless this group of patients is still considered at risk. The most serious possible complications are congenital malformations of the foetus and possible deterioration of long-term diabetic complications of the mother. The subsequent text is a brief review of contemporary views on pregnancy of diabetic patients.

In the course of pregnancy increasing insulin resistance develops due to deteriorated phosphorylation of the insulin receptor and its substrate (IRS-1). As a result of relations between secretion and the action of insulin the beta-cell reacts by insulin hypersecretion and thus the development of hyperinsulinaemia is influenced. Furthermore pregnancy is characterized by increasing differences between the anabolic and catabolic stage in the intermediary metabolism manifested in particular by a tendency towards ketosis and hypoglycaemia during fasting and also by hypoaminoacidaemia. In addition to these metabolic changes there are also other laboratory deviations, manifested e.g. by hyperfibrinogenaemia and an increased concentration of tPA inhibitor (PAI-1) without detectable endothelial dysfunction. The increasing albuminuria is not a manifestation of developing nephropathy but the sign of functional changes which after delivery disappear very quickly. The mentioned deviations are the consequence of altered hormonal regulations in the course of pregnancy, while after delivery the baseline state present before pregnancy is restored.

Diabetes of all types is a known complication of pregnancy and is a risk for mother and foetus. Despite significantly improved care of pregnancies complicated by diabetes the perinatological results are still somewhat worse than in the healthy population. Therefore in the further improvement of perinatological indicators preconception care participates as well as systematic compensation of insulin dependent diabetes, preventive admission on hospital and correct timing of termination of pregnancy in pregestational as well as in gestational diabetes. All care of these pregnancies is shared by the obstetrician and diabetologist.

High standard metabolic compensation of diabetes reduces in a significant way the risk of developmental defects and the perinatal morbidity of children of diabetic mothers and the maternal mortality. A considerable proportion of diabetic women is inadequately informed on the risks of pregnancy. As many as two thirds of pregnancies in diabetic women are unplanned and thus the risk of inadequate compensation of diabetes in early stages of pregnancy increases. Preparation of diabetic women for planned pregnancy is therefore very important. Ensuring long-term normal blood sugar levels in type 1 diabetics has many pitfalls and involves in particular optimal insulin treatment and systematic and high standard control of the blood sugar level and other parameters.

The objective of the presented work was to find possible predictive factors of pregnancy-induced hypertension (PIH) in women with the risk of gestational diabetes (GDM) during pregnancy. A group of women with the risk of development of gestational diabetes was selected because it is known that in women with GDM a hypertension is encountered 2-4 times more frequently than in women without disorders of glucose tolerance during pregnancy. The patients were divided into four groups with regard to the risk of development of hypertension. The examinations were made once during the 20th week of gestation. PIH is manifested in the great majority of patients only after the 20th week of pregnancy. All patients were normotensive at the time of examination. During the subsequent course of pregnancy in all patients the diagnosis of gestational diabetes and pregnancy-induced hypertension was investigated. The investigated parameters were fasting insulin levels during the 20th week of gestation, coagulation factors--antithrombin III (AT III), D-dimer, number of thrombocytes. The authors investigated also anamnestic data on the presence of type II diabetes and hypertension in close relatives of the patients.No differences were found in the insulin, antithrombin III and D-dimer levels nor in the number of thrombocytes. A significant relationship was revealed between the incidence of hypertension during pregnancy and the family history of the patient. In women where there was diabetes type 2 and hypertension in the family, there was a markedly more frequent incidence of pregnancy-induced hypertension after the 20th week of pregnancy (p0.0002) than in the other investigated groups.

The authors investigated whether vascular complications of insulin-dependent diabetic pregnant women can influence on a long-term basis their children in adult life. In a group of 169 children of diabetic mothers (CDM) 12 developed diabetes during childhood and adolescence; in the remaining 157, age 20.75 +/- 0.31 (SE) the following tests were made: oral glucose tolerance test (OGTT), insulin level, parameters of the lipid metabolism, and blood pressure was assessed. CDM were divided into three groups: 1. CDM whose mothers had diabetic complications; according to White's classification of diabetes during pregnancy they belonged into class D and F (n = 10). 2. CDM whose mothers were classified with regard to diabetes during pregnancy as class B and C with subsequent toxaemia of pregnancy (n = 27), 3. CDM of mothers class B and C without toxaemia of pregnancy (n = 117). All investigated groups of CDM had significantly higher sums of blood sugar and insulin levels during the OGTT (P0.01), as compared with the control group (n = 31). In the first group of CDM the values were significantly higher than in the third group of CDM. As to cholesterol and triacylglycerol levels, the differences were not significant, the non-esterified fatty acid levels were higher in the second group than in controls (P0.05). In the first two groups also a higher systolic pressure was recorded than in controls (P0.01). Two children of the first group, two children of the second group and eight children of the third group of CDM developed diabetes.(ABSTRACT TRUNCATED AT 250 WORDS)

The participation of essential fatty acids and their derivatives in the blood (serum) of physiological healthy neonates (physiological pregnancy, physiological delivery) is compared with the spectrum of essential fatty acids of risk newborns (prematurity, prematurity with the tocolytic therapy and hypotrophic newborns). In the following part the presence and participation of essential fatty acids in the human maternal milk were measured. The milk of healthy mothers, of mothers with preterm delivery (37 week of gestation), of diabetic mothers were mutually compared and finally the values of mentioned essential fatty acids in formula Feminar and in the milk from the bank were established. As a consequence of our observations, they point out the absence or serious deficit of some essential fatty acids in certain groups of risk newborns as well as in the milk of diabetic mothers, of mothers with preterm delivery and in Feminar and Milk-bank. The presented facts--after their opinion--could be well judged as a rational basis for adequate nutritional therapy in risk newborns.

The paper presents the first results obtained as a part of a screening programme of detection of congenital developmental defects of the fetus. Pregnancies with diabetes are one of the risk groups in this respect. We have divided our patients (n = 58) into two groups. In the first there were 28 diabetics in the 15th-16th week of pregnancy. They were examined for alpha-1-fetoprotein (MS AFP) and trophoblast-specific beta-1-glycoprotein (MS SP1): in chosen cases chorionic gonadotropine (MS HCG) was assessed at the same time. The second group were 35 insulin-dependent pregnant diabetics followed-up in Regional Ambulance of Central Bohemia and hospitalized at the First Clinic of Obstetrics and Gynaecology of the First Medical Faculty, whose MS SP1 levels were being assessed (usually in one-week intervals) during the second and third trimester of pregnancy. 21 patients were simultaneously examined for glycated serum proteins. Our study confirmed MS AFP levels of diabetics in the 15th-16th week of pregnancy to be range of low limit of normal values (0.5-1.0 MOM). MS HCG levels in sera of diabetics ranged from 5,000 to 60,000 IU/ml in the 15th-16th week. Comparing glycated serum proteins of women with physiological pregnancies with those of healthy non-pregnant controls we had found no differences. In diabetics the values found in the second and third trimester of pregnancy were increased.

The authors compare perinatal results in insulin treated diabetic women who were followed up in the out-patient department of the Second Gynaecological and Obstetric Clinic of the Masaryk University in Brno. In 1980-1984 148 pregnant diabetic women were followed up and in 1985-1991 when self-monitoring has become part of comprehensive treatment of pregnant diabetics in our department, 279 women were followed up. The patients were monitored during pregnancy by a diabetologist and gynaecologist. Other members of the team are a paediatrician, geneticist, psychologist, dietitian, and educational nurse. As to laboratory parameters, short-term and long-term compensation of diabetes were monitored. Systematic preconception preparation of diabetic patients, with an attempt to achieve normal blood sugar levels and genetic examination of both partners from part of comprehensive care. The results achieved in our department revealed a statistically significant decline of the incidence of foetopathies in neonates at the 5% level of significance and a decline of hypoglycaemia in foetuses at the 0.1% level.

Hundred and seventy five infants of diabetic mother born and followed up carefully in the Institute for the Care of Mother and Child were examined at a mean age of 20.75 +/- 4.08 years. The purpose of the investigation was to assess the relationship of hypertrophy of neonates of diabetic mothers to other deviations in neonates and their long-term prognosis. In neonates with a marked degree of hypertrophy the authors recorded significantly more frequently hypoglycaemia, early asphyctic syndrome, impaired respiration and neurological symptoms. Children born as hypertrophic developed equally frequently diabetes (8.7%) as children of diabetic mothers who did not develop hypertrophy (8.0%). Abnormal glucose tolerance was recorded in those with hypertrophy in 6.6%, in the group without hypertrophy in 5.0%. As compared with the offspring of healthy mothers, they suffered significantly more frequently from higher blood sugar levels and higher insulinaemia (sigma C ad sigma I) during the oGTT. The body weight expressed as BMI and systolic blood pressure were also higher than in the control group. None of the investigated indicators in adult age was associated with body weight and the degree of hypertrophy. The development of diabetes is determined more by genetic factors.

During the regular follow-up of risk pregnancies in the Central Bohemian region, concentrated at the First Obstetric Clinic in Prague, the authors detected 34 women with retinopathies. In nine they performed panretinal photocoagulation by means of an argon laser and recorded favourable results. The authors consider early laser therapy of retinopathies in pregnant diabetics useful and essential. Advanced forms of diabetic retinopathy in pregnant women still remain a contraindication of pregnancy. Pregnancy in diabetic women is always a risk and it would be a mistake not to take this fact into account.