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PubMedDate not supplied

[The epithelial proliferative activity of the mucosa of the large intestine in dysentery patients at the acute period of the disease depending on the expression of morphological changes].

The dependence of proliferative activity on the degree of histomorphological changes in colonic mucosa of patients with dysentery was studied histoautoradiographically in colonic biopsies. Morphological changes were evaluated according to N. B. Shalygina's schema (catarrhal, exudative, erosive or ulcerous inflammation) A 2.5-3-fold increase of the labelled nuclei index (LNI) and label intensity (LI) was found in the catarrhal inflammation. LNI was increased 1.2-fold and LI 1.5-fold in cases of other types of inflammation; they were lower than in catarrhal inflammation.

Acute Disease↗
PubMedDate not supplied

[Thymidine kinase activity in leukocytes from patients with chronic myeloid leukemia at various periods in the disease].

The level of thymidine kinase activity in the premature leukocytes of patients with chronic myeloleukemia during the stable phase was shown to serve as a measure of the disease development. Considerable variations in thymidine kinase activity in blast cells in myeloid and lymphoid blast crises demonstrated that analysis of the enzyme activity might be used in the biochemical diagnosis of blast crisis in chronic myeloleukemia simultaneously with the enzymes of purine metabolism--ADA and PNP. During cell differentiation, the activity of thymidine kinase was decreased and in the myeloid cells the enzymatic activity was much higher of that in lymphoid cells as shown by investigations using blast cells of patients with blast crisis in chronic myeloleukemia, cells K-562, thymocytes, spleen and peripheric blood lymphocytes. Isozyme thymidine kinase I was mainly responsible for the rate of enzymatic activity in premature leukocytes of patients with chronic myeloleukemia regardless of the disease stage.

Blast Crisis↗
PubMed1991

[Clinical manifestations of ischemic heart disease in the subacute period of myocardial infarction and post-infarct cardiosclerosis depending on the status of coronary arteries].

Multifactor discriminant analysis was used on a computer to examine 100 patients with myocardial infarction in the subacute period (at week 4 of the disease onset) and 76 in the postinfarction period (at months 2-120 of postinfarction). A complex of clinical and coronary signs was defined, which differentiated patients with subacute myocardial infarction and postinfarct cardiosclerosis, as well as those with postinfarct stable and progressive angina pectoris.

Acute Disease↗
PubMed1996

Tumor recurrence after oLTX.

Although early survival following transplantation for primary hepatic cancer is excellent, previously reported high recurrence rates have generally discouraged liver replacement for this condition. The aim of this retrospective analysis was to examine the influence of risk factors on the development of early tumor recurrence. Between December 1982 and June 1995, 480 liver transplantations were performed at a single institution. Out of these, 103 patients had unresectable primary hepatic cancer (88 hepatocellular cancer; HCCA; 20%) and 15 had cholangiocellular cancer (CHCA; 4%). The influence of the following tumor-associated risk factors was assessed: tumor size, tumor distribution within the liver, grading, pseudocapsular formation, vascular invasion, lymph node metastasis, and cirrhotic alteration. The diagnosis of tumor recurrence was made using various radiological imaging techniques, reelevation of serum alphafetoprotein, or autopsy. For patient survival and disease-free period, data analysis was performed by the method of Kaplan-Meier. The Cox model was used for multivariate analysis; a P-value of less than 0.05 was considered to be significant. The mean age of the 103 patients was 54 years (range 15-63a). There were 22 female and 81 male patients. The follow-up period ranged between 4 and 108 months. Twenty-nine patients (50%) died during the follow-up period due to recurrence of disease. The survival rates of the 88 patients with HCCA were 57%, 34%, and 26% at 1, 3, and 5 years, respectively, after orthotopic liver transplantation (oLTX; follow-up 36 month). Of the 15 pts with CHCA the rates were 53%, 33%, and 33%, respectively, with a median follow-up of 60 months. The influence of the risk factors studied showed a significantly longer disease-free period for the following tumor characteristics: grading below or equal 2 (P = 0.009) and absence of vascular invasion (P = 0.04). Regarding a median survival rate of 2-4 months for patients with unresectable malignant liver tumors, these results confirmed the indication for oLTX, especially if the patient does not compete with someone on the waiting list for benign liver disease.

Adolescent↗
PubMed2000

The barium enema in constipation: comparison with rectal manometry and biopsy to exclude Hirschsprung's disease after the neonatal period.

BACKGROUND: The diagnosis of Hirschsprung's disease is usually made in neonates but often considered in older infants and children with constipation: these children may be referred for barium enema. Since it is widely accepted that a normal barium enema does not exclude Hirschsprung's disease, some children, after a normal enema, undergo more invasive procedures such as rectal manometry or biopsy. Our study asked how frequently a diagnosis of Hirschsprung's disease was made by biopsy or manometry in children who had normal barium enema. MATERIALS AND METHODS: We reviewed the medical records and barium enemas of 54 patients older than 28 days with constipation or difficulty passing stool who had a barium enema followed by manometry and/or biopsy. RESULTS: Forty-eight patients had normal enemas: 24 of those patients had biopsies, 16 had manometry, and 8 both manometry and biopsy. Only 1 had manometry suggestive of Hirschsprung's disease, confirmed by biopsy. Six patients had abnormal enemas. Five had biopsy and manometry compatible with Hirschsprung's disease; one had a normal biopsy and manometry study. CONCLUSION: The barium enema is a good initial screening test for Hirschsprung's disease in severely constipated children since it correlates well with manometry and biopsy. The enema is particularly useful in centers without easy access to pediatric gastroenterology services, and a normal enema in this setting allows the continuation of medical therapy with further evaluation only if there is a lack of response. An abnormal enema, however, requires referral to a facility equipped to perform confirmatory manometry or biopsy.

Adolescent↗
PubMed1994

Electromyographic silent period after transcranial brain stimulation in Huntington's disease.

The silent period evoked by transcranial (TCS) and nerve stimulation was studied in the hand muscles in 13 patients with Huntington's disease and in 11 normal subjects. The duration of the silent period after TCS was longer in patients and correlated significantly with the severity of chorea; in contrast, the duration of the silent period after nerve stimulation was similar in patients and controls. The prolongation of the cortical silent period suggests that the duration of the silent period is a functional correlate reflecting basal ganglia influence over the motor cortex.

Adult↗
PubMedDate not supplied

[The pathogenetic substantiation of the periods of traumatic brain disease].

Advances in the study of the molecular mechanisms of the pathogenesis of closed craniocerebral trauma (CCCT) which was conducted on an experimental model, a clinical equivalent of CCCT, allow one to form a new view of the course of traumatic disease and its division into periods. The data obtained provide evidence that increase of the pathological process in the posttraumatic period is of a skipping and cascade character. The existence of such breaks makes it possible to distinguish periods with consideration for the pathogenetic essence of the pathological changes. 1. The period of intensified metabolic processes, "conflagration of metabolism". 2. The period of development of energy deficit in the nerve tissue. 3. The period of development of cell intoxication processes and secondary structural changes. 4. The period of formation of posttraumatic homeostasis (a) in the regimen of stable homeostasis, (b) in the regimen of stress and subsequent exhaustion of the activity of adaptation systems with the formation of late-term progressive sequelae.

Brain↗
PubMed2002

[Functional cardiovascular status in patients with high blood pressure and coronary heart disease in a remote period of uranium intoxication]].

High blood pressure and coronary heart disease in far-off period of chronic uranium intoxication result in re-modelling of left ventricle with predominant concentric re-modelling. Lower myocardial mass of left ventricle and more marked changes in diastolic function occurred in the main group if compared to the reference one having no contact with occupational hazards.

Aged↗
PubMed2000

[Von Hippel-Lindau disease: protocols for diagnosis and periodical clinical monitoring. National Von Hippel-Lindau Disease Working Group].

Von Hippel-Lindau disease (VHL) is an autosomal dominantly inherited syndrome with high penetrance, characterised by tumours in various organs. The Dutch VHL working group presents guidelines for DNA testing and clinical monitoring, to enhance early detection and treatment of VHL patients in the Netherlands. Diagnosis of VHL is justified in patients presenting with a typical VHL tumour with a positive family history, but patients with a VHL tumour and a negative family history may also have VHL. Diagnosis of VHL can be confirmed by molecular genetic analysis of the VHL gene which is informative in virtually all VHL families. In a patient with (suspicion for) VHL there is an indication for genetic counselling. A protocol for clinical monitoring of VHL is presented and is recommended for: carriers of a VHL germline mutation; members of VHL families with an unknown familial mutation; members of VHL families who decline testing of the familial mutation; patients suspected for VHL, but without a detectable VHL gene mutation.

Clinical Protocols↗
PubMed2007

Short femurs detected at 25 and 31 weeks of gestation diagnosed as Leroy I-cell disease in the postnatal period: a report of two cases.

Leroy I-cell disease is a rare autosomal recessive lysosomal storage disorder characterized by marked psychomotor and growth retardation, skeletal anomalies, and typical facial features. There is a biochemical defect in uridine diphospho-N-acetylglucosamine-1-phosphotransferase, which is the enzyme responsible for addition of a mannose phosphate residue for lysosomal trafficking. Prenatal diagnosis is possible by analysis of enzyme activity in chorionic villi or cultured amniocytes, but this is offered to families only known to be at increased risk. We describe two cases that had bilateral shortness of the femurs at 25 and 31 weeks of gestation in the ultrasound scan and were diagnosed as Leroy I-cell disease by plasma enzyme analysis in the postnatal period. There was also bowing of the femurs in one case. None of the two families had a history of Leroy I-cell disease. The parents of one case were second-degree cousins. In view of these two cases that are presented, we propose that Leroy I-cell disease should be included in the differential diagnosis of short femurs even when there is no evident family history.

Adult↗
PubMed2006

Diagnostic scoring system of Hirschsprung's disease in the neonatal period.

OBJECTIVE: Eighty to ninety percent of Hirschsprung's disease (HD) patients present in newborns. However, the diagnosis of HD in the neonatal period remains difficult. Our present study aims to propose a diagnostic scoring system and hope this will increase early diagnosis of HD and avoid unnecessary rectal biopsy. METHODS: In the first study period, 57 suspected HD patients (0-3 months) completed our predetermined study protocol in which barium enema (BE), rectal manometry (RM) and full-thickness rectal biopsy were performed. Symptoms, signs and investigations were analysed for their correlation with HD diagnosis. A HD diagnostic scoring system was developed according to the statistical results and was assessed in 74 patients in the second study period. RESULTS: Forty-five patients were diagnosed with HD in the first study period. A HD scoring system was developed in which delayed meconium, tight anus, BE and RM were diagnostic factors. A cut-off point of 3 provided 84% of HD patients score >3, whereas 75% non-HD patients score <or=3 (p < 0.05). In the second study group, patients with score <or=3 were selected for rectal biopsy. CONCLUSION: In the neonatal period, using a HD scoring system may help to select patients for further invasive investigation so that unnecessary biopsy can be avoided.

Early Diagnosis↗
PubMedDate not supplied

[Thyrotoxic periodic paralysis and Graves' disease. Description of an HLA-CW7-positive case].

Thyrotoxic periodic paralysis is an uncommon complication of hyperthyroidism, more frequent in Oriental populations and in male than in female subjects. Electron microscopic investigations have shown characteristic degenerative changes of muscle fiber. In this study the sequence of pathophysiologic events proposed by the literature is illustrated and a case of thyrotoxic periodic paralysis in a HLA-CW7 positive patient affected by Graves' disease is reported.

Adult↗
PubMed1990

Statistical problems in epidemiologic studies of the natural history of disease.

The development of effective disease prevention and treatment programs depends on an understanding of the natural history of disease. A conceptual framework is presented for disease natural history and consists of an asymptomatic period of disease followed by a period of symptomatic disease. The focus is on epidemiologic studies for identifying risk factors of the onset of asymptomatic disease, for identifying cofactors of progression to symptomatic disease, and for estimating the duration of the asymptomatic period. The strengths and limitations of various epidemiologic study designs and sources of epidemiologic data are considered for characterizing disease natural history. Issues in the interpretation and analysis of natural history parameters of disease estimated from cross-sectional, prevalent cohort, cohort, and matched case-control studies are considered. The issues and analytic methods are illustrated with studies of the acquired immunodeficiency syndrome (AIDS) and cervical cancer. Based on these analytic methods, an estimate of the incubation period distribution of AIDS is given.

Acquired Immunodeficiency Syndrome↗