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 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In order to study the long term outcome of hepatic glycogen storage diseases, a national retrospective inquiry gathered 76 patients older than 12 years. In adolescents and adults, hypoglycemia, failure to thrive, pubertal delay, hepatomegaly and metabolic disturbances are major in type I, intermediary in type III and mild in type "VI+IX". Spontaneous improvement of these symptoms is noted in older patients. Beside these classical signs, anemia, high blood pressure, renal failure and persistent hypercholesterolemia were reported in some type I glycogen storage disease and bad school and professional results in type III. The knowledge of these complications should lead to a better management of these patients.
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PMID:[Long-term course of hepatic glycogenosis. A retrospective study of 76 cases]. 306 69

The effects of pregnancy on acute metabolic complications of diabetes may have important consequences for both mother and fetus. The consequences of pregnancy for chronic complications of diabetes, including retinopathy, nephropathy, neuropathy, and hypertension, are not clear. Recent data are reviewed so that health care providers will be able to provide reasonable advice to insulin-dependent diabetic women contemplating pregnancy both for problems that may potentially arise during gestation and those that may affect long-term health and survival. Diabetic ketoacidosis is an uncommon problem that arises during gestation. Acute alterations in pH and electrolyte concentrations as well as hyperglycemia, however, may have important consequences for mother and fetus, including perinatal asphyxia and reduced fetal oxygen delivery. Hypoglycemia, on the other hand, may result in maternal coma or seizures and, when frequent, has been associated with infant respiratory distress syndrome. Background retinopathy often worsens during gestation, with regression common postpartum. Data suggest that progression of background disease is related to both glycemic control and the acute institution of intensive insulin therapy with those patients with poor control requiring more aggressive therapeutic intervention most adversely affected. The course of proliferative retinopathy is more variable, with both progression and regression reported. Preconception photocoagulation may prevent progression. Preconceptional ophthalmologic evaluation with frequent assessments during pregnancy is advised. Increases in 24-hour protein excretion are common during gestation in patients with preexisting renal disease and resolve in many patients postpartum. Serum creatinine and creatinine clearance increase during the first trimester and generally do not change during the remainder of pregnancy.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Impact of pregnancy on complications of insulin-dependent diabetes mellitus. 313 6

The pathophysiology of renal dysfunction in generalized sepsis remains unknown. In this study, 24 hours after surgical induction of peritonitis in 20 volume-loaded sheep, three patterns of renal function were seen. In group 1 (n = 8), glomerular filtration rate (GFR) decreased by 70%, urine volume by 85%, absolute sodium excretion by 95%, and fractional sodium excretion by 83%. Group 2 (n = 4) exhibited similar sodium retention but GFR did not fall. Group 3 (n = 8) showed no change in GFR or urine volume and only minimally reduced sodium excretion. Mean arterial pressure fell 17% in group 1 only; central venous pressure, pulmonary capillary wedge pressure, and plasma volume were maintained at or above presepsis values in all groups. Cardiac index was either increased or unchanged, and renal plasma flow was maintained in all groups; there was thus no hemodynamic evidence to suggest volume contraction. Histologic examination showed only minor changes with no consistent pattern. Renal functional changes correlated with other manifestations of severe sepsis--GFR and sodium retention correlated significantly with increased cardiac index, decreased systemic vascular resistance, pulmonary arterial hypertension, leukopenia, hypoproteinemia, and hypoglycemia. All of these changes were most marked in group 1. In groups 1 and 2, plasma renin activity (PRA) increased and urinary kallikrein excretion decreased. PRA correlated inversely with GFR, urine volume, and sodium excretion; urinary kallikrein excretion correlated positively with urine volume and sodium excretion. Urinary excretion of 6-keto-PGF1 alpha was increased in groups 1 and 2 and correlated inversely with mean arterial pressure in group 1 animals. During sepsis, urinary thromboxane B2 excretion continued at presepsis values in all groups. The results suggest that unusual reciprocal changes in activity of the renin-angiotensin and renal kallikrein-kinin systems may play a role in the renal response to sepsis. PGI2 synthesis is increased and may affect systemic hemodynamics and renal function; the role of thromboxane A2 in this context is unknown.
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PMID:Vasoactive hormones in the renal response to systemic sepsis. 327 70

Until drugs that will prevent metabolic derangements that cause the complications of diabetes have been developed, the best approach to their prevention is control: of hyperglycemia, of hypertension, of obesity, and of smoking. Intensive insulin therapy, although demonstrably effective, must be approached with caution because hypoglycemia is a potentially life-endangering threat. Conversely, a Danish study has demonstrated a decrease in hypoglycemic episodes with intensive insulin therapy (Parving HH. Personal communication, 1988). With this in mind, it may be essential to bring blood glucose levels into a reasonable range shortly after the diagnosis of diabetes mellitus has been made. Insulin therapy is required for type I diabetic patients, and it may also be an appropriate therapy for all type II patients who do not become rapidly normoglycemic following diet and oral sulfonylurea treatment. Some physicians believe that a frontal "attack" of a split-mixed program of insulin therapy when type II diabetes is diagnosed is of psychologic as well as physiologic benefit, impressing the patient with the importance of control and vigilance. Compliance to rigid dietary change is notoriously unsuccessful, and the "trial-and-failure" approach, often ending in insulin therapy in any case, may not be the most effective. The advent of easy-to-use blood glucose monitoring devices and convenient and discreet insulin delivery systems has made maintenance of glycemic control less difficult for the insulin-using patient. New antihypertensive agents, lipid-reducing drugs, and second-generation sulfonylureas that do not affect the quality of life are now available and should be used in the person with diabetes as necessary.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Prevention of the complications of diabetes. 329 Sep 19

Evoked responses of various modalities are being used for sensory and neurological clinical assessment. An additional application is their use in studying the functional activity of several sensory pathways and many brain regions during induced deviations from homeostasis in the intact animal. The auditory nerve-brain-stem evoked response (ABR) has been studied during hypoxia, hypercapnia, acidosis, hypoglycemia, systemic hypotension, intracranial hypertension and decreased cerebral perfusion pressure. In many such experiments the following were also recorded: EEG, cortical evoked potential (EP), somatosensory EP (including peripheral nerve, brain-stem and cortical components), visual and vestibular EP. Even though the EEG was already isoelectric, the ABR and many of the other EPs were not affected by relatively severe deviations in homeostasis, several of which have been shown to induce brain lesions and severe perturbations of energy metabolism. The ability of these brain pathways to produce electrical activity in such conditions may be due to the generation of such EP by oligosynaptic pathways and to compensatory mechanisms such as increased local cerebral blood flow. Clinically, these findings point out the usefulness of ABR recordings in the diagnosis of brain death and to the possibility that when ABR is continuously monitored in the intensive care patient, alterations in the ABR may indicate the need for immediate intervention.
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PMID:Auditory evoked potentials during deviations from homeostasis: theoretical and clinical implications. 330 16

This study concerns a series of 34 pregnant diabetic patients, insulin-dependent or requiring insulin, which were followed between January 1st 1980 and May 31st 1986. The blood level of glycolated hemoglobin (HbA1C) decreased constantly, demonstrating the improvement of the balance of the blood sugar, but its level, at the end of the pregnancy, could not be correlated with the presence of a macrosomia (27%) and/or of a neonatal hypoglycemia (24%). On the contrary, the infraclinical hypertrophy of the ventricular septum (HVS) diagnosed systematically by sonogram, and discovered in 6 newborns (nb) could represent a good marker of the harmful role of maternal hyperglycemia, even if moderate. Arterial hypertension, found in 8 cases, has a prognostic value as it is responsible for acute fetal distress (AFD), 2 cases, and for delivery by caesarean section (8 cases). A multidiscipline approach seems to improve the prognosis of pregnancy in diabetic patients, which nevertheless, should still be considered as a high-risk pregnancy.
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PMID:[Monitoring of the diabetic pregnancy. Apropos of 34 insulin-treated cases]. 331 52

The development of hyperglycemia in the elderly is often multifactorial in etiology, and its presentation is often confounded by the advanced age of the patient, the presence of coexisting diseases and altered mental states, the absence of symptoms, and physical conditions specific to the medical care of the geriatric patient. Manifestations of macro- and microvascular complications of non-insulin-dependent diabetes mellitus (NIDDM) often herald the disease in the elderly, yet there is incomplete knowledge of the natural history of the disease and poor guidelines for its effective management in the geriatric population. Once NIDDM is diagnosed in the older patient, the propensity for these patients to develop atherosclerotic vascular complications involving every organ system and the socioeconomic sequela of the disease make treatment prudent. Coexisting risk factors for atherosclerosis, such as dyshypoproteinemia, hypertension, obesity, and cigarette smoking, should be treated vigorously, and poor diet, physical inactivity, and medications affecting glucose tolerance modified. Hyperglycemia resistant to nonpharmacologic therapy should be treated with second-generation oral sulfonylureas, and the judicious use of insulin is advised because of a heightened risk for the hazards of hypoglycemia in the elderly. The treatment of NIDDM has important implications in the elderly because of its prevalence and its association with other age-related pathophysiologic processes. Such effective treatment may have the potential to reduce morbidity and mortality and improve the quality of life of older people.
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PMID:Non-insulin-dependent diabetes mellitus in the elderly. Influence of obesity and physical inactivity. 332 19

Infants of diabetic mothers are thought to be at risk for perinatal asphyxia. We hypothesized that the following are significant risk factors for perinatal asphyxia: poor third-trimester glycemic control, diabetic vascular disease (nephropathy, retinopathy) appearing in pregnancy, pregnancy-associated hypertension, smoking, prematurity, fetal macrosomia, and maternal hyperglycemia and hypoglycemia within 6 hours preceding delivery. We prospectively studied 162 infants born to 149 diabetic mothers (White classes B through R-T). Perinatal asphyxia was defined clinically as fetal distress during labor (late decelerations, persistent fetal bradycardia, or both), 1-minute Apgar score less than or equal to 6, or intrauterine fetal death. Forty-four infants (26.7%) had perinatal asphyxia. The presence of perinatal asphyxia did not correlate with third-trimester glycemic control, pregnancy-associated hypertension, smoking, fetal macrosomia, or maternal hypoglycemia before delivery, but it did correlate significantly with nephropathy appearing in pregnancy, maternal hyperglycemia before delivery, and prematurity. We speculate that (1) the appearance of diabetic vasculopathy (nephropathy) during pregnancy is accompanied by placental vascular disease and subsequently by fetal compromise and (2) in pregnancy complicated by diabetes, maternal and subsequently fetal hyperglycemia before delivery leads to fetal hypoxemia.
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PMID:Perinatal asphyxia in infants of insulin-dependent diabetic mothers. 339 99

Although kidney enlargement occurs in Type I glycogen storage disease, renal disease has not been considered a major problem. Death from renal failure in three patients known to us prompted a study of renal function in this disorder. Of the 38 patients with Type I glycogen storage disease under our care, the 18 children under 10 years old had normal renal function. Fourteen of the 20 older patients (13 to 47 years) had disturbed renal function, manifested by persistent proteinuria; many also had hypertension, hematuria, or altered creatinine clearance. Progressive renal insufficiency developed in 6 of these 14 patients, leading to three deaths from renal failure. At the onset of proteinuria, creatinine clearance was increased in seven patients (3.05 +/- 0.68 ml per second per 1.73 m2 of body-surface area; range, 2.47 to 4.13 [normal range, 1.33 to 2.33 ml per second per 1.73 m2]). Renal biopsies were performed in three patients after an average of 10 years of proteinuria. All three biopsies demonstrated focal segmental glomerulosclerosis in various stages of progression. Our data suggest that chronic renal disease is a frequent and potentially serious complication of Type I glycogen storage disease. In addition to treating hypoglycemia vigorously, physicians should monitor renal function carefully in patients with this disorder.
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PMID:Renal disease in type I glycogen storage disease. 342 4

A retrospective analysis was undertaken of 128 pregnancies (131 infants) complicated by diabetes; 66 (51 per cent) were Class A and 62 (49 per cent) Class B-D-F-R. 53.9 per cent of all infants were large for gestational age (LGA) and there were no differences between the classes of diabetics. LGA infants occurred with equal frequency in those diabetic patients with pregnancy-induced or chronic hypertension. Congenital anomalies occurred in 9.7 per cent with 11/12 in Class A, B, or C. Major neonatal morbidity included: 1) hypoglycemia: two (3 per cent) Class A and 21 (32.8 per cent) insulin-dependent mothers (P less than 0.01); and 2) respiratory distress syndrome: seven (5.3 per cent) and all were in classes B-F (P less than 0.05). Modern management of diabetes in pregnancy has, for unknown reasons, increased the incidence of LGA infants.
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PMID:Large for gestational age: dilemma of the infant of the diabetic mother. 350 63


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