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Query: UMLS:C0025362 (mental retardation)
 15,878 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Deficiency of cystathionine beta-synthase (CBS) is the commonest cause of primary homocystinuria. Homocysteine metabolism is intimately linked with the metabolism of folate, vitamin B12 (cobalamin) and pyridoxine. It is hypothesised that the pathogenesis of neuropsychiatric manifestations in homocystinuria, folate and cobalamin deficiencies are related to imbalance neurotransmitters in the CNS through disturbances in the pathways linking the metabolism of homocysteine and these vitamins. Although neuropsychiatric disorders are relatively common among patients with homocystinuria, it is not well recognised as the causative factor among patients presenting with neuropsychiatric disorders. A 31 year old woman presented with a three week history of delirium and inappropriate and labile affect. There was no history suggestive of drug or alcohol abuse, nutritional deficiency or organic disorders. EEG, cerebral CT, MRI and microbiological investigations did not reveal any organic causes. Because of a diagnosis of pyridoxine-responsive homocystinuria seven years previously, the possibility of homocystinuria was considered and investigated. Laboratory tests revealed macrocytosis and a high concentration of urinary total homocystine. Commencement of pyridoxine at 400 mg/day resulted in disappearance of homocystine in urine within four days with remarkable clinical improvement. Homocystinuria should be considered in the differential diagnosis of unexplained neuropsychiatric disorders in patients who have past or family history of homocystinuria, mental retardation, thromboembolic episodes, vascular diseases or clinical and laboratory features resembling folate and/or vitamin B12 deficiencies. Homocystinuria-associated neuropsychiatric disturbances can easily be treated with pyridoxine in 50% of cases.
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PMID:Homocystinuria and psychiatric disorder: a case report. 1050 67

Percutaneous endoscopic gastrostomy (PEG) has gained great popularity for children with malnutrition and eating disorders secondary to chronic illness. However, the procedure is not without risks. We report on 62 infants and children, median age 4 years (1 month-20 years), who underwent PEG placement. Cerebral palsy with or without mental retardation was the most common diagnosis (50%). No complications related to the PEG procedure itself occurred, but postoperative pneumonia was seen in 10%. Late complications were few: intraperitoneal migration of the button in one child and prolapse of the stoma in another. At the time of button placement, after median 14 weeks, mean weight had increased from a standard deviation score of -2.7 to -2.2 (P < 0.001). We consider PEG to be a safe procedure for children with malnutrition requiring enteral feeding. Due to potential risks and complications related to this method, a multidisciplinary approach, as found in a "nutritional support team", is recommended.
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PMID:[Help to children and adolescents with malnutrition or eating disorders. Percutaneous endoscopic gastrostomy with button: simple, safe and cost-effective]. 1081 20

This study describes the frequency of and indications used for total feeding assistance and tube feeding in a national representative sample of Belgian hospital patients (n = 421 314). Data from the 1990 national minimum nursing data registration was used. Orem's self-care model was used to describe and categorize types of nursing care and related indications. The scope of wholly compensatory nursing care was limited to total feeding assistance and tube feeding. This type of nursing care is indicated when self-care agency is undeveloped or cannot be used and when self-care demands are significantly increased. Based on review of the literature, hypotheses to indicate nursing care were formulated and tested. Wholly compensatory nursing care related to enteral food intake is given to 14.4% of the total patient population in Belgian hospitals. If self-care demand increases due to malnutrition, then this is an indication for total feeding assistance. If self-care agency decreases due to blindness, mental retardation, disorientation or upper extremity dysfunction, then this is also an indication for total feeding assistance. If self-care agency decreases due to impaired chewing, then this is an indication for tube feeding. This study has generated the first representative national nursing statistics about total feeding assistance and tube feeding in hospitals.
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PMID:Frequency of and indications for wholly compensatory nursing care related to enteral food intake: a secondary analysis of the Belgium National Nursing Minimum Data Set. 1088 51

To study the phenotypic spectrum and management of holoprosencephaly (HPE), we reviewed the findings of eight children with HPE from 3 to 10 years of age, who underwent intervention programs and rehabilitation at our center. One patient had alobar HPE, three semilobar HPE, and four lobar HPE. All patients had postnatal growth retardation, and seven showed a decreased BMI (< 25% tile). All patients had severe developmental delay and mental retardation (DQ < 40), showing no obvious correlation between their severity and the type of HPE. Neurologically seven patients had spasticity (3 spastic quadriplegia, 2 spastic diplegia, 2 mixed-type), except one patient with a 7q deletion [46,XY,del(7) (q35)] who had generalized hypotonia. Seven had variable types of seizures. All patients had feeding difficulties and were assessed by speech-language therapists. Four patients required tube feeding, four had gastroesophageal reflux disease. Recurrent respiratory tract infection was common. Three patients had abnormal serum sodium concentration (1 diabetes insipidus, 1 idiopathic hypernatremia, 1 hyponatremia). No family history of HPE was elicited. In conclusion, patients with HPE should be followed up closely for complications such as feeding difficulty, malnutrition, seizures, spasticity, infection, and osmoreceptor-hypothalamus-hypophyseal axis abnormalities.
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PMID:[Clinical spectrum and management of holoprosencephaly]. 1091 68

We tested whether dimensional measures of empathic ability, theory of mind, and intelligence would differentiate autism spectrum disorders from each other and from non-spectrum disorders. Tests were administered to children with a diagnosis of Autistic Disorder (AutD; n = 20), Asperger's Disorder (AspD; n = 28), Attention Deficit/Hyperactivity Disorder (Inattentive Type) (ADHD; n = 35), Mental Retardation (Mild) (MR; n = 34), Anxiety Disorder (AnxD; n = 14), or No Psychological Disorder (NPD; n = 36). Results showed that empathic ability discriminated among groups on the autism spectrum (AutD < AspD < NPD). Because empathic ability is not independent of intelligence (AutD < AspD < NPD on intelligence; MR < ADHD < NPD on empathic ability), both dimensions are necessary to discriminate autism spectrum from non-spectrum disorders. When intelligence is covaried, empathic ability discriminated AutD, but not AspD, from other disorders (AutD < MR < ADHD < NPD = AnxD = AspD).
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PMID:Do autism spectrum disorders differ from each other and from non-spectrum disorders on emotion recognition tests? 1146 82

Profound hypothermia (core temperature of less than 28 degrees C) is a life threatening state and a medical emergency associated with a high mortality rate. The prognosis depends on underlying diseases, advanced or very early age, the duration prior to treatment, the degree of hemodynamic deterioration, and especially, the methods of treatment, including active external or internal rewarming. This is a case study of an 80-year-old female patient with severe accidental hypothermia (core temperature 27 degrees C). She was found in her home lying immobile on the cold floor after a fall. The patient was in a profound coma with cardiocirculatory collapse, and the medical staff treating her was inclined to pronounce her deceased. On her arrival at the hospital, she was resuscitated, put on a respirator and actively warmed. Very severe metabolic disorders were found, including a marked metabolic acidosis composed of diabetic ketoacidosis (she had suffered from insulin treated type 2 diabetes mellitus) and lactic acidosis with a very high anion gap (42) and a hyperosmotic state (blood glucose 1202 mg/dl). There were pathognomonic electrocardiographic abnormalities, J-wave of Osborn and prolonged repolarization. Slow atrial fibrillation with a ventricular response of 30 bpm followed by a nodal rhythm of 12 bpm and reversible cardiac arrest were recorded. The pulse and blood pressure were unobtainable. Despite the successful resuscitation and hemodynamic and cognitive improvement, rhabdomyolysis (CKP 6580 u/L), renal failure and hepatic damage developed. She was extubated and treated with intravenous fluids containing dopamine, bicarbonate, insulin and antibiotics. Her medical condition gradually improved, and she was discharged clear minded, functioning very well and independent. Renal and liver tests returned eventually to normal limits. Progressive bradycardia, hypotension and death due to ventricular fibrillation or asystole commonly occur during severe hypothermia. Respiratory and metabolic, sometimes lactic, acidosis, lethargy and coma, hypercoagulopathy, hyperosmolar state, acute pancreatitis and renal and hepatic failure are frequent complications of hypothermia. Underlying predisposing causes of hypothermia are diabetic ketoacidosis, cerebrovascular disease, mental retardation, hypothyroidism, pituitary and adrenal insufficiency, malnutrition, acute alcoholism, liver damage, hypoglycemia, sepsis, hypothalamic dysfunction, sepsis and polypharmacy, and especially, the use of sedative and narcotic drugs. Our case demonstrates once again that CPR once begun should continue until the successful rewarming because "no one is dead until warm and dead".
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PMID:[Severe accidental hypothermia in an elderly woman]. 1175 73

Altered dendritic spines are characteristic of traumatized or diseased brain. Two general categories of spine pathology can be distinguished: pathologies of distribution and pathologies of ultrastructure. Pathologies of spine distribution affect many spines along the dendrites of a neuron and include altered spine numbers, distorted spine shapes, and abnormal loci of spine origin on the neuron. Pathologies of spine ultrastructure involve distortion of subcellular organelles within dendritic spines. Spine distributions are altered on mature neurons following traumatic lesions, and in progressive neurodegeneration involving substantial neuronal loss such as in Alzheimer's disease and in Creutzfeldt-Jakob disease. Similarly, spine distributions are altered in the developing brain following malnutrition, alcohol or toxin exposure, infection, and in a large number of genetic disorders that result in mental retardation, such as Down's and fragile-X syndromes. An important question is whether altered dendritic spines are the intrinsic cause of the accompanying neurological disturbances. The data suggest that many categories of spine pathology may result not from intrinsic pathologies of the spiny neurons, but from a compensatory response of these neurons to the loss of excitatory input to dendritic spines. More detailed studies are needed to determine the cause of spine pathology in most disorders and relationship between spine pathology and cognitive deficits.
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PMID:Dendritic spine pathology: cause or consequence of neurological disorders? 1208 7

In India the health services are unable to meet the real needs of the poor. The colonial policy of exploitation, expropriation and plunder created widespread disruption in the way of life of the Indian people. The colonialism destroyed the health culture of India and created a Western bias in the health services which continues to this day. The bias has extend to research - for example, by postulating a correlation between malnutrition and mental retardation. The commitment of the current political leadership to bypass the medical establishment and go directly to the people has created a favorable setting to challenge the basic scientific, sociological and economic premises of the earlier approach to development of the health services in India, and to formulate a new approach to development of the health services in India, and to formulate a new approach to take its place. The main premise of Another Development in the health services is to begin with the people - a framework is designed for a health service system particularly tailored to meet the needs of the people. Efforts to relieve the suffering caused by health problems can contribute to the initiation of political, economic and social action. In this context, the formulation and implementation of Another Development in Health acquires much significance: 1) the very alleviation of suffering has political significance, because it narrows the gap between the ruling classes and the masses; 2) Another Development in Health would provide an entry point for change agents, who could use the opportunity to work with the people to initiate changes in other social and economic fields; and 3) by generating social awareness, Another Development in Health may work as a lever for promoting similar developments in other social and economic fields.
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PMID:Health as a lever for another development. 1226 Jun 75

This paper focuses on the influence of maternal nutrition on infant survival and growth and on subsequent fertility. During pregnancy, modifications in the maternal hormonal system help maintain availability of nutrients to the fetus relatively independently of maternal nutrition. When maternal dietary deficiencies reach critical levels, the effectiveness of these mechanisms in maintaining fetal nutrition decreases. Studies have confirmed the correlation of severe maternal malnutrition and lowered birth weight, neurological disorders, impaired physical growth, mental retardation, and poor school performance. Marginal nutritional status affects milk production capacity. The basis of most recommendations for lactating mothers is that the average mother produces 850 ml of milk each day. Depending on the anticipatory reserves of fat from pregnancy still available and the level of physical activity of the mother, from 2750 to 3300 Kcal/day may be needed. When the mother's energy intake is good, milk fat resembles that of her dietary fat, but when there is a shortage of food energy, the milk fatty acid pattern resembles the mother's subcutaneous fat stores. The lactose content and overall protein content of milk seem stable despite changes in the maternal diet, but the vitamin content, particularly water soluble vitamins, is very sensitive to dietary intake. Birth weight has consistently been found to be associated with infant mortality through 2 main mechanisms: maternal malnutrition may lead to a smaller placental size and decreased nutrient supply to the fetus, resulting in developmental retardation during intrauterine life, or maternal malnutrition may result in suboptimal lactation performance which will contribute to malnutrition and growth retardation of the child. Maternal nutritional status has an independent effect on the duration of postpartum amenorrhea. Improving maternal diet without concurrently introducing contraception may shorten the birth interval and consequently elevate the birthrate.
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PMID:Maternal nutrition, infant health, and subsequent fertility. 1226 49

This comprehensive review includes results of studies on the relationships maternal and child health and fertility and hypotheses on their interactions from a programmatic viewpoint. Child mortality is high for 1st born, low for the 2nd and 3rd child, and increases with parity after the 4th. Infant survival is lowest when the birth interval is 1 year or less. The frequency of low intelligence, mental retardation, malnutrition, and infections increase with family size. Maternal mortality and morbidity increase with parity. Rapid population growth and density is correlated with infections, such as tuberculosis and social pathology, such as schizophrenia. The relationship between health and fertility is complex, since in the absence of family planning, improved health decreases mortality and morbidity, and increase fertile life span. Amenorrhea in general and during lactation lasts longer during malnutrition, but lactation is likely to be more successful in healthy women. It is probably more efficient in personnel, facilities and transportation to combine maternal and child health and family planning services. The work would be more stimulating, and the clients more likely to trust a familiar person. In the prenatal, postpartum, and postabortion periods women are motivated to accept both services. It is said that education and services in infant health will convince people to start planning their families: a few epidemiologic and longitudinal studies support this hypothesis. Probably incentive programs are less cost-effective than combining family health and planning services.
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PMID:[Interactions between health and population]. 1233 84


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