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Query: UMLS:C0011849 (diabetes)
 277,896 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty-seven children with early-onset (less than 4 yr) diabetes (EOD), 24 children with late-onset (greater than 4 yr) diabetes (LOD), and 30 sibling controls were compared in their performance on tests of intellectual functioning and school achievement. The results indicated that children with EOD, particularly girls, scored lower than the other groups of diabetic children and siblings on tests of visuospatial (P less than .05) but not verbal ability. Many of the girls with EOD were also having difficulty at school, and several were receiving special education. Children with EOD had more hypoglycemic convulsions than those with LOD. Both convulsions and age of onset were associated with poorer performance on spatial tasks. Girls with EOD had lower spatial test scores regardless of convulsion history, whereas boys with EOD scored lower only if they had had a convulsion.
Diabetes Care
PMID:Intellectual deficits associated with early onset of insulin-dependent diabetes mellitus in children. 362 9

Replacement of destroyed B-cells with new "healthy" ones appears to be the most physiological approach to treatment of insulin-dependent (type 1) diabetes. This could be achieved either by transplantation of isolated islets or pancreatic segments or by stimulation of the replicatory activity in the islet cells surviving the acute toxic or infectious insult. The results of our experimental investigations indicate that adult, mature insulin-producing cells can replicate very actively when challenged with a proper stimulus, such as when implanted into obese-hyperglycaemic mice. The growth of transplanted islets is, however, dependent on the site of implantation. Thus, the subcapsular space of the kidney is more favourable than the spleen. Furthermore, immunosuppressive therapy may impair islet cell replicatory activity, as demonstrated in this study for Cyclosporin A. In a search for factors stimulating islet cell replication, we found that the hepatotoxic agent galactosamine enhanced this process as observed autoradiographically one week after the injection. This fits in with previous case reports demonstrating increased mitotic activity in islets of patients suffering from various acute liver diseases. Altogether this series of experiments demonstrates that islet cell replication is a more dynamic process than has hitherto been believed. Obviously many conditions influence this process so significantly that an impact on the diabetic state of the patients can well be expected.
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PMID:Experimental studies of transplantation and regeneration of endocrine pancreatic cells. 393 14

The etiology, epidemiology, pathophysiology, and complications, therapy, and prognosis of hypernatremic (hypertonic) dehydration in infants are briefly discussed. The most likely causal condition for hypernatremic states in infants is enteric disease, because the symptoms of diarrhea and vomiting result in water loss and inability to take in water for replenishment. Other causes include dubious feeding practices, diabetes mellitus, diabetes insipidus, and maladroit diagnostic and therapeutic maneuvers, including administration of radiologic contrast medium or hypertonic sodium bicarbonate or mannitol infusions, or the use of salt solutions as an emetic. Epidemiologically, 2 factors are apparent: high saline diet and winter season. The clinical hallmarks of hypernatremic disturbance are relative preservation of circulation and early presence of neurologic symptomatology. Renal tube necrosis is also occasionally encountered. Therapy is rehydration, but the bone of contention is the technique for replacing water in the face of the fact that water administered without electrolyte causes the brain to swell and frequently results in convulsions. The management of hypernatremic dehydration begins with a replenishment phase if neither shock nor apparent anuria is present. The principle is to replenish the body slowly, and 48 hours has been chosen as the target, so that for volume the deficit plus 2 days of ongoing losses should be allocated. However, the sodium and other ion contents are derived solely from the deficit, without factoring the 2-day maintenance period. A recipe for rehydration fluid is presented.
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PMID:Hypernatremic (hypertonic) dehydration in infants. 457 33

The anaesthetic management of four paediatric patients with the Prader-Willi syndrome is reported. The syndrome is characterized by obesity, mental retardation, genital hypoplasia, hypotonia, and diabetes mellitus. All patients were anaesthetized with halothane. Succinylcholine or pancuronium were used for muscle relaxation, without evidence of abnormal response. Common anaesthetic difficulties in this syndrome are obesity, hypotonia, disturbance in thermoregulation, arrhythmias, diabetes mellitus and convulsions.
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PMID:Anaesthetic considerations in the Prader-Willi syndrome: report of four cases. 613 37

We report five children who required total pancreatectomy in the neonatal period for life threatening hypoglycaemia. Subsequent diabetes was managed with once daily subcutaneous insulin, oral pancreatic extract, and a controlled carbohydrate diet. Daily home blood glucose profiles in four of these children were different from those of 14 C-peptide negative children with type 1 insulin dependent diabetes--in the pancreatectomy group there was less variation in blood glucose throughout the day with a fall after breakfast as against a rise after breakfast in the type 1 group. Hyperglycaemia (greater than 10 mmol/l) with or without ketonaemia was extremely rare in the pancreatectomy group. Despite neonatal convulsions, physical growth and development have been normal. Hypoglycaemia, usually after food refusal, has been a major problem. In addition, there have been considerable emotional and family disturbances despite the excellent metabolic control, which presumably reflect the cumulative difficulties in managing such young children with diabetes.
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PMID:Management of secondary diabetes mellitus after total pancreatectomy in infancy. 637 5

The clinical and EEG findings were reviewed for 270 juvenile children from the Montreal Children's Hospital Diabetic Clinic in an attempt to correlate the EEG findings at the onset of diabetes mellitus with the future risk of having a convulsion with a hypoglycemic reaction. Compared to a non-diabetic control population, the number of epileptiform EEG abnormalities was significantly increased in the initial EEG of diabetic patients who later had a hypoglycemic convulsion. The initial epileptiform EEG did not help to differentiate those diabetics with recurrent hypoglycemic induced convulsions from diabetics who would have only a single convulsion.
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PMID:EEG abnormalities and convulsions in juvenile diabetes mellitus. 661 50

Three children with the femoral hypoplasia-unusual facies syndrome are described. Two had the characteristic facial pattern of upslanted palpebral fissures, long philtrum with thin upper lip, micrognathia, and hypoplastic alae nasi. The other, an infant girl who died within 24 hours after birth, had a cleft lip, which distorted some of the other features. She also had a cleft palate, as did one of the two older boys. All three children had ear defects, upper limb involvement, and rib, vertebral, lower extremity, and genitourinary tract abnormalities. The infant girl died of lung hypoplasia associated with dysplastic kidneys and widely patent ductus arteriosus. All three were infants of diabetic mothers, one mother having developed overt diabetes in the first trimester of pregnancy. A literature review of 36 reported cases of FH/UFS revealed 12 individuals who were IDMs, establishing a strong relationship of the syndrome with maternal diabetes. A multifactorial inheritance model fits with the reported patients, with the relationship to diabetes, and with the similarity of FH/UFS to caudal regression, another condition related to maternal diabetes.
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PMID:Femoral hypoplasia-unusual facies syndrome in infants of diabetic mothers. 685 50

Several vitamins have been demonstrated to interfere with the pathogenesis of some metabolic diseases, mainly by three different mechanisms: 1) vitamin malabsorption, 2) errors in vitamin metabolism, 3) vitamin dependent syndromes. The latter is due to a deficiency of the apoenzyme whose coenzyme is the vitamin itself. In this case pharmacological, instead of nutritional doses of the vitamin may be needed. The vitamins which interfere with inborn metabolic errors are reviewed; for each vitamin the corresponding diseases which may be treated are indicated. The vitamins are: 1) thiamine (leucinosis); b) nicotinic acid (hyperlipoproteinemia); c) biotin (beta-methyl-crotonyl-glycinuria, propionic aciduria); d) pyridoxine (infantile convulsions, familial pyridoxine responsive anemia, homocystinuria, cystathioninuria, xanthurenicaciduria); e) cobalamins (congenital intrinsic factor deficiency, cobalamin malabsorption, transcobalamin deficiency, methylmalonic aciduria) f) folic acid (congenital folic acid malabsorption, formimino-transferase deficiency, methylenetetrahydrofolic reductase deficiency, Lesch-Nyhan syndrome); g) vitamin D (phosphatic diabetes, Prader's type rickets, Albright's syndrome; essential hereditary hypophosphatemia, etc). It is noteworthy that the vitamin therapy of these diseases, not only corrects the metabolic errors, but can also promote the healing or the amelioration of the psycho-physical growth, of central nervous system alterations and of other lesions.
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PMID:[Vitamins in metabolic diseases]. 702 68

Family history data were collected in 1203 consecutive admissions of insulin-dependent diabetes mellitus cases to the Children's Hospital of Pittsburgh between Dec. 31, 1964 and Jan. 1, 1981. This report deals with two issues: the closeness in time of dates of onset of diabetes in multiple sibling case families and the similarity of ages of onset within these families. The age-specific incidence rates among siblings were 6-18 times higher than in the general population. Contrary to other reports, this study does not find an increased risk to siblings during the first year or two after the onset of the index diabetic case. The mean duration between cases is 6.1 years. Fitting a log-normal distribution to the periods between cases verified the long median incubation period (4.35 years) with a high degree of variability (dispersion factor = 3.38). However, the log-normal was not a good fit to the data (p less than 0.005). There is a significant correlation for age of onset for pairs of affected siblings within families (0.25). However, it is shown that the similarity in age of onset within families may be a function of the greater similarity to ages of siblings within a family. It is noted that secondary cases are more often younger children in the family, particularly so if the index child is of school age at the time of onset.
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PMID:Pittsburgh diabetes mellitus study. II. Secondary attack rates in families with insulin-dependent diabetes mellitus. 704 31

There is an increasing interest on one of the smallest human chromosomes as it is shown by the First International Symposium on the Human Chromosome 20 and by the genetic map prepared by EUROGEN. The conserved part of the long arm of human chromosome 20 is synthenic with the distal part of the mouse chromosome 2 allowing for some analogies between them. Human chromosome 20 contains several important genes for the human pathology. Mutations of one of them, the vasopressin-neurophysin II gene, are responsible for hereditary neurohypophyseal diabetes insipidus. Severe combined immunodeficiency due to adenosin deaminase deficiency is the first human disorder successfully treated by somatic gene therapy. Spongiform encephalopathies are related to mutation and/or polymorphisms of the PRNP amyloid gene. One form of benign familiar neonatal convulsions is mapped to a specific locus on chromosome 20. In some families, maturity onset diabetes of the young (MODY) is caused by alterations of a hypothetical gene closely linked to the ADA locus. Allegile syndrome is often associated with deletions and microdeletions of the short arm of the chromosome. Finally, deletions of the long arm of the chromosome is a frequent finding in several hematologic malignities, specifically in myeloproliferative disorders and myelodysplastic syndromes.
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PMID:[The human genome--chromosome 20]. 748 83


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