Coincidence of giant uterine myomatosis and detection of two advanced malignancies in 77-year-old female patient.

OBJECTIVE: Our aim is to emphasize the importance of patient participation in screening programs available in the Czech Republic and in preventive gynaecological examinations. As an example, we present a case report of a 77-year-old female patient with the accumulation of serious gynaecological diseases (extreme uterine myomatosis, breast cancer) and non-Hodgkin´s B-lymphoma. DESIGN: Case report. SETTING: Department of Obstetrics and Gynaecology, Faculty Hospital in Pilsen, Charles University Medical Faculty in Pilsen. CASE REPORT: Our case report of a 77-year-old patient is a rare case of the simultaneous detection of giant uterine myomatosis causing voluminous ventral hernia, invasive ductal breast cancer, and non-Hodgkin´s B-lymphoma. The patient was not gynecologically examined for last 20 years and did not undergo any screening examinations. In the postoperative period, the patient was diagnosed with invasive ductal breast cancer and follicular lymphoma. All the diseases were diagnosed in advanced stage. CONCLUSION: The occurrence of bulky fibroids is very rare in postmenopause. Long-term neglect of clinical symptoms and adverse health signals allows their gradual progression. Especially in older patients, other serious illnesses may complicate the situation.

[Severe immune thrombocytopenia diagnosed in pregnancy]. / Závazná imunitní trombocytopenie diagnostikovaná v gravidite.

OBJECTIVE: The aim of this article is present a patient with severe immune thrombocytopenia in pregnancy. DESIGN: Case report. SETTING: Department of Obstetrics and Gynecology, University Hospital Olomouc, Faculty of Medicine, Palacký Univerzity Olomouc. CASE REPORT: Presents a patient with immune thrombocytopenia diagnosed in pregnancy. Severe thrombocytopenia was diagnosed incidentally. CONCLUSION: Severe immune thrombocytopenia is rare and substantial complication in pregnancy. In pregnancy is important cooperation between obstetritians and hematologists, in time of delivery neonatologists and anesthesiologists.

Intestinal microbiota in exclusively breast-fed infants with blood-streaked stools.

Intestinal microbiota in exclusively breast-fed infants with blood-streaked stools and in healthy exclusively breast-fed babies was compared. Total anaerobes, bifidobacteria, lactobacilli, coliform bacteria, enterococci and clostridia were quantified by cultivation methods in feces of 17 full-term exclusively breastfed patients (aged 16.3 +/- 7.4 weeks) with blood-streaked stools and in the control group of 22 healthy fullterm exclusively breast-fed infants (13.7 +/- 6.4 weeks). Specific fluorescence in situ hybridization kits for Bifidobacterium spp. were used for the quantitative detection of bifidobacteria in samples. Control samples had significantly (p < 0.05) higher counts of total anaerobes. Bifidobacteria were not detected in patients' samples in 65 % and in controls in 36 % (p < 0.01). Bifidobacteria counts were also significantly higher in the control group (p < 0.01). Furthermore, clostridia strains were detected only in feces from bifidobacteria-negative infants reaching counts >8 log CFU/g. Lactobacilli were not detected in 65 % patients and in 45 % control samples. However, this difference was not significant as well as the difference in lactobacilli counts. Eosinophilia was observed in 35 % of patients, low IgA concentration in 71 % and also low IgG concentration in 71 %. pANCA positivity was found in 53 % of patients. In conclusion a significant low proportion of bifidobacterial microbiota in patients with blood-streaked stools was shown in comparison with controls.

[Delivery of a healthy child from pregnancy after IVF complicated hyperstimulation syndrome, phlebothrombosis and resection of uterine horn. Case report]. / Porod zdravého plodu v gravidite po IVF komplikované hyperstimulacním syndromem, flebotrombózou a resekcí rohu delozního. Kazuistika.

OBJECTIVE: To evaluate the negative effect of uterine horn resection for heterotopic pregnancy in the uterine horn in the first trimester on the course of pregnancy and labor. To point out the increased incidence of other complications in pregnancy after IVF+ET (ovarian hyperstimulation syndrome, phlebothrombosis of the pelvic veins). DESIGN: Case study. SETTING: Department of Obstetrics and Gynecology, 1st Faculty of Medicine, Charles University and General Faculty Hospital, Prague. METHODS: In this study, the authors analyze their experience with the course of pregnancy of a patient who had uterine horn resection for a heterotopic uterine horn pregnancy after IVF+ET. The course of pregnancy was associated with further complications such as ovarian hyperstimulation syndrome and the resulting occurence of thrombosis in the 2nd trimester of pregnancy. The pregnancy was terminated by caesarean section for the indication of prior uterine surgery-resection of the uterine horn in the first trimester. A healthy infant was delivered. CONCLUSION: This case study demonstrates the multiple occurrence of serious complications in pregnancy after IVF+ET, which are associated with ovarian hyperstimulation and the transfer of multiple embryos. The pregnancy was terminated with the delivery of a healthy term infant.

Renal concentrating capacity is linked to blood pressure in children with autosomal dominant polycystic kidney disease.

Impaired glomerular filtration rate (GFR) is a risk factor for the development of hypertension in patients with autosomal dominant polycystic kidney disease (ADPKD). However, markers of tubular function were not tested whether they are linked to hypertension or blood pressure (BP) level. The aim of our study was to investigate the relationship between renal concentrating capacity and BP in children with ADPKD. Fifty-three children (mean age 11.8+/-4.4 years) were investigated. Standardized renal concentrating capacity test was performed after nasal drop application of desmopressin, BP was measured by ambulatory BP monitoring (ABPM). Renal concentrating capacity was decreased in 58 % of children. The prevalence of hypertension was significantly higher in children with decreased renal concentrating capacity (35 %) than in children with normal renal concentrating capacity (5 %) (p<0.05). Significant negative correlations were found between renal concentrating capacity, ambulatory BP and number of renal cysts (r = -0.29 to -0.39, p<0.05 to p<0.01). In conclusion, the concentrating capacity is decreased in about half of the patients and is linked to BP. Decreased renal concentrating capacity should be considered.

[Hemolytic-uremic syndrome]. / Hemolyticko-uremický syndrom.

Hemolytic-uremic syndrome (HUS) is the most common cause of acute renal failure in children below 3 years of age. It is defined by a triad of symptoms which associates hemolytic anemia with fragmented erythrocytes, thrombocytopenia and acute renal failure. Three types of HUS can be distinguished: typical HUS, also called diarrhoea-associated (D+HUS), very rare atypical HUS (D-HUS) and secondary HUS (drug induced, C+HUS, in patients receiving marrow transplantation, etc.). The common event among these entities appears to be vascular endothelial cell injury, which induces mechanical destruction of erythrocytes, activation of platelet aggregation and local intravascular coagulation, especially in the renal microvasculature. D+HUS represents 90% of HUS in children. Evidence of exposure to verotoxin (VT), shiga toxin (ST) producing Escherichia coli (VTEC or STEC) has been demonstrated in many countries in about 85% of cases. Serotype O157:H7 is the most frequent. Early and accurate supportive treatment and early start of dialysis is the major importance and allows a current mortality rate below 5%-10%. Vital prognosis is compromized in cases with multivisceral involvement. After 15 years or more of apparent recovery, 20 to 60% of patients have residual renal symptoms, with up to 20% having chronic renal insufficiency (CRI) or end-stage renal disease (ESRD). Atypical HUS represents less than 10% of HUS in children. Some of these cases (familial) are associated with low C3 levels, hereditary deficiency of factor H or with mutations in factor H gene. The deficiency of von Willebrand factor cleaving protease, as reported in adults with thrombotic thrombocytopenic purpura (TTP), is not present in D+HUS.

Mitochondrial DNA depletion in Alpers syndrome.

Mitochondrial dysfunction of the energy generating system was suggested in two infants with progressive infantile poliodystrophy characterised by hypotonia, refractory epilepsy, visual impairment, psychomotor retardation, profound brain atrophy, hepatopathy, and increased levels of lactate in blood and cerebrospinal fluid. Histochemical and electron microscopic analyses of liver biopsies revealed cytochrome c oxidase deficiency, microvesicular steatosis, and enormous multiplication of mitochondria of various sizes. In the first patient, the quantitative Southern blot analyses in tissues obtained at autopsy demonstrated reduced content of mtDNA in the liver, brain, and fibroblasts (11 %, 15 %, and 25 % of the mean values in controls) while a normal content of mtDNA was found in muscle and heart. In the second patient, a reduced content of mtDNA was found in the muscle, liver, and brain (15 %, 10 %, and 30 %, respectively, of the mean values in controls). Biochemical studies in the first patient revealed decreased activities of all respiratory chain complexes except complex II in isolated liver mitochondria and decreased amounts of respiratory chain complexes I, III, IV and ATP synthase in liver and frontal cortex, but not in muscle, heart, and fibroblasts. In conclusions, mtDNA depletion associated with Alpers syndrome may be tissue specific.

[Pregnancy in myelodysplastic syndrome]. / Gravidita pri myelodysplastickém syndromeu.

OBJECTIVE: Evaluation of the influence of myelodysplastic syndromee (MDS) on the course of pregnancy and delivery. DESIGN: Case report. SETTING: Gynecological and Obstetric Department 1st Medical Faculty Charles University and General Faculty Hospital, Prague. SUBJECTS AND METHODS: The authors analyze their experience with the course of pregnancy and delivery in a patient with MDS and refractory anaemia who was treated on account of the disease for several years before pregnancy in the Institute of Haematology and Blood Transfusion. Pregnancy was not associated with progression of the basic disease and complications which developed during pregnancy were not associated with MDS. Pregnancy was terminated in this patient on account of preeclampsia gravis per sectionem caesarean by delivery of a healthy foetus. CONCLUSION: The example draws attention to a serious haematological disease while during pregnancy and delivery deterioration of the basic disease was not observed.

Ambulatory blood pressure monitoring in children with unilateral multicystic dysplastic kidney.

UNLABELLED: Multicystic dysplastic kidney (MCDK) is one of the most common congenital renal anomalies. Arterial hypertension is a potential complication of MCDK. Blood pressure (BP) has so far been measured only casually and the frequency of hypertension has been estimated to be between 0%-8%. Ambulatory blood pressure monitoring (ABPM) provides more precise information on BP than the casual BP measurement. The aim of this study was to investigate the BP profile in children with MCDK using ABPM. A group of 25 children (16 girls), with a mean age of 7.8 years (range 3.8-17.7 years) were investigated. ABPM was performed using the oscillometric SpaceLabs 90207 device. Hypertension was defined as mean systolic and/or diastolic BP during the day and/or in the night exceeding 95th percentile for ABPM. Five (20%) children showed hypertension, two of them had combined daytime and night-time hypertension and three had isolated nocturnal hypertension, although daytime BP was between the 90th-95th percentile in two of them. Children with ultrasonographical and/or laboratory signs of contralateral kidney abnormalities showed a higher incidence of hypertension than those without abnormalities (two of four versus 3 of 21). The mean night-time systolic and diastolic BP of children with MCDK was significantly higher than in healthy children (+ 0.50 and + 0.54 SDS, respectively, P = 0.012 and 0.03, respectively). Three of the hypertensive children were already nephrectomised. All five hypertensive children showed ultrasonographical and/or laboratory signs of contralateral kidney abnormalities. Hypertensive children had significantly higher microalbuminuria than normotensive children (6.9 +/- 3.2 mg/mmol creatinine versus 1.8 +/- 0.7, P = 0.03). The nocturnal BP fall (dip) was attenuated in five children, only one of whom was hypertensive. CONCLUSION: Arterial hypertension in children with multicystic dysplastic kidney is seen more often if based on ambulatory blood pressure monitoring than on casual blood pressure recordings. The main risk factor for developing hypertension is contralateral kidney damage. Ambulatory blood pressure monitoring should be performed in children with multicystic dysplastic kidney, especially in those with contralateral kidney abnormalities.

Cattle can be a reservoir of sorbitol-fermenting shiga toxin-producing Escherichia coli O157:H(-) strains and a source of human diseases.

Using the immunomagnetic separation procedure, we isolated sorbitol-fermenting (SF) Shiga toxin-producing Escherichia coli (STEC) O157:H(-) strains from two patients, one with hemolytic-uremic syndrome and the other with diarrhea, and from a dairy cow epidemiologically associated with the patients. The phenotypic and genotypic characteristics of all isolates were identical or closely related. Moreover, the bovine isolate showed a clonal relatedness to SF STEC O157:H(-) strains isolated from patients in Germany and the Czech Republic from 1988 to 1998. This is the first evidence that cattle can be a reservoir of SF STEC O157:H(-) and a source of human diseases.

Isolation and characterization of sorbitol-fermenting Shiga toxin (Verocytotoxin)-producing Escherichia coli O157:H- strains in the Czech Republic.

Two sorbitol-fermenting (SF) Shiga toxin-producing Escherichia coli (STEC) O157:H- strains were isolated from patients with hemolytic-uremic syndrome in the Czech Republic in 1995. Their phenotypic and genotypic characteristics and genomic DNA fingerprints were identical or closely related to those of SF STEC O157:H- strains isolated in Germany in 1988 to 1997. This indicates that the Czech isolates belong to the SF STEC O157 clone which is widespread in Germany. It is the first finding of the clone outside Germany.

Human Escherichia coli O157:H7 infection associated with the consumption of unpasteurized goat's milk.

A cluster of four cases of haemolytic uraemic syndrome in children occurred in Northern Bohemia, Czech Republic, between 15 June and 7 July, 1995. All the cases had significantly elevated titres of anti-O157 lipopolysaccharide (LPS) antibodies as detected by the indirect haemagglutination assay. All but one of them had drunk unpasteurized goat's milk from the same farm within the week before the disease. Evidence of E. coli O157 infection was subsequently found in 5 of 15 regular drinkers of the farm's raw goat's milk; four of them were asymptomatic, 1 had mild diarrhoea at the end of June. Verocytotoxin 2-producing E. coli O157:H7 strains of phage type 2 and of identical pulsed-field gel electrophoresis patterns were isolated from 1 of 2 farm goats and from 1 of the asymptomatic goat's milk drinkers. The frequency of anti-O157 LPS antibodies found among regular drinkers of the farm's raw goat's milk (33%; 5 of 15) was significantly higher than that found in control population (0%; none of 45) (P = 0.0005; Fisher's exact test). Our findings indicate that goats may be a reservoir of E. coli O157:H7 and a source of the infection for humans; raw goat's milk may serve as a vehicle of the pathogen transmission.

Verocytotoxin-producing Escherichia coli in children with hemolytic uremic syndrome in the Czech Republic.

Since October 1988 till July 1995, 35 children (mean age 16 +/- 15.5 months) with classical hemolytic uremic syndrome (HUS) were examined for the presence of verocytotoxin (VT)-producing Escherichia coli (VTEC) infection. Stool samples from all patients were cultured for VTEC strains and tested for free fecal neutralizable VT. Serum samples from 18 patients taken on admission were also tested for antibodies to the lipopolysaccharide (LPS) from E. coli O157, O26, O55, O111, and O128 using the passive hemagglutination assay (PHA). Diagnosis of VTEC infection was established in 28 (80%) patients by the combined use of microbiological and serological techniques. VTEC were isolated from 16 (46%) patients, 5 of them had infection with 2 different VTEC serotypes; 11 (52%) VTEC isolates belonged to the serotypes O26: H11 (5), and O157: H7/NM (6). Free fecal VT was found in 21 (60%) patients. PHA antibodies to one or more LPS were detected in 14 (78%) of 18 patients; 9 had antibodies to O157 LPS and 6 to O26 LPS. We conclude from this study that VTEC are the important cause of pediatric HUS in the Czech Republic and the strains belonging to the serogroups O157 and O26 are the most prevalent.

[The Camurati-Engelmann syndrome (progressive diaphyseal dysplasia)]. / Morbus Camurati-Engelmann (progresivní diafyzární dysplazie).

The presented case-history demonstrates an infant with a rare bone disease with a very serious prognosis. Based on X-ray findings on the skeleton (sclerosis of the diaphyses of long bones, ribs, vertebrae and in particular the base and vault of the skull), the diagnosis of morbus Camurati-Engelmann--progressive diaphyseal dysplasia was established. With regard to the striking progression of the process in the cranial area and the progressing stigmatization of the patient's face, the authors consider also a more recent diagnostic unit--craniodiaphyseal dysplazia. With advancing sclerotization of the skull the most serious complications are compression of the cranial nerves which can be resolved in the final stage only by surgery. From a brief review of the literature: the aetiology of the disease is not known, the therapeutic effect of prednisone or calcitonin is small.

[Infantile cortical hyperostosis (the Caffey-Silvermann syndrome)]. / Infantilní kortikální hyperostóza (syndrom Caffey-Silvermann).

In 1984-1989 at the First Paediatric Clinic of the Motol Faculty Hospital six patients with the diagnosis of infantile cortical hyperostosis (Caffey-Silvermann syndrome) were hospitalized. Non-familial concurrence of the disease was involved. All children developed the disease before the age of 4.5 months, four before the age of three months. Among the six patients was only one girl. As to pathological findings there was a markedly elevated FW in 5/6, leucocytosis in 5/6, thrombocytosis in 3/4, eosinophilia in 3/6, elevated alkaline phosphatases in 2/6. The affection was four times on the mandible, once in the area of the hip joints and in one instance it was multifocal. The authors present one detailed case-history with extreme affection of the clavicle which was originally taken for a bone tumour.

[Severe Vicedrin poisoning in a 17-month-old girl]. / Tezká intoxikace Vicedrinem u 17 mesícního devcátka.

The child ingested 7 tabl. of VICEDRIN (a combination of phenacetin, ephedrin, chinin, acid. ascorbicum), the total dose of phenacetin was 140 mg/kg of b.w. Lethal doses of phenacetin vary between 100-200 mg/kg, the sensitivity to phenacetin being increased in infants. Toxicological examination in this case revealed a high concentration of phenacetin in urine. The clinical signs of intoxication were vomiting (hematemesis), methemoglobinemia and somnolence. 2 hemoperfusions were performed lasting 6 hrs and 5 hrs resp. (HEMASORB 400 A 4), the second one were combined with hemodialysis because after the first perfusion a high concentration of metabolic products of phenacetin was detected in urine. After the second perfusion the status of the child rapidly improved and we could discharge the patient of the 10th day after admission. Hemoperfusion is recommended in severe intoxication with phenacetin, the combination with, hemodialysis is possible to remove its metabolic product.

Verotoxigenic (enterohaemorrhagic) Escherichia coli in infants and toddlers in Czechoslovakia.

The results of the investigation indicate that verotoxigenic Escherichia coli (VTEC) belonging to enteropathogenic and other serogroups including Escherichia coli O157:H7 or H- are important enteropathogens in infants and toddlers in Czechoslovakia. As to enteropathogenic serotypes, verotoxin (VT) production was proved most frequently in strains of serogroup O26, and also O111 and O128. Diseases caused by them were as a rule manifested by febrile watery diarrhoea with mucus in the stool. In two of five infants with Escherichia coli O26 :H11 with VT1 production in titres of greater than or equal to 1:512 (blood was present) in the stool and one suffered from marked abdominal pain. In one infant haemorrhagic colitis due to Escherichia coli O157:H- was found. Haemolytic uraemic syndrome associated with VTEC of serogroups O157, O26, O18, O5 and O1 with VT1 and/or VT2 was observed in six children including five who contracted the disease during an outbreak in a small town, and the source of infection was probably contaminated water. Five children recovered and one died; the postmortem examination revealed haemorrhagic colitis and necrosis of the renal cortex. Haemorrhagic colitis caused by Escherichia coli O157 in infants and toddlers differed from the course hitherto described in older subjects by fever and the presence of mucus in the stools.

Vero cytotoxin-producing strains of Escherichia coli in children with haemolytic uraemic syndrome and diarrhoea in Czechoslovakia.

The presence of verotoxin-producing strains of Escherichia coli (VTEC) was examined in six children with haemolytic uraemic syndrome and one child with haemorrhagic colitis. Stools were screened for strains of serogroup O157 on sorbitol-MacConkey agar for VTEC of other serogroups by serotyping. Verotoxin (VT) was tested on Vero cell monolayers: the antigenic variant of VT was assessed by neutralization experiments. Strains producing verotoxin 1 or verotoxin 2 or both were detected in the stools of all seven children. Three strains belonged to serogroup O157 (two of them to serotype O157:H7, one was non-motile) and another five belonged to serogroups O26 (two strains), O1, O5 and O18. The faeces of five children available for testing contained free VT. Production of VT was also examined retrospectively in 32 E. coli strains of serotype O26:H11 isolated from children with diarrhoea; eight (25%) of them produced moderate to high levels of verotoxin 1 despite several years storage in vitro. In conclusion, VTEC including strains of serogroup O157 seem to be an important cause of haemolytic uraemic syndrome, haemorrhagic colitis and diarrhoea in children in Czechoslovakia.