[Carnitine deficiency myopathy: a case of late diagnosis]. / Miopatía por déficit de carnitina: un caso de diagnóstico tardío.

Myopathies caused by lipidic metabolism alterations are very infrequent. Carnitine deficiency-associated myopathies are included in this group. Two main types of carnitine deficiency syndromes have been delineated: a predominantly myopathic form, with normal serum and low muscle carnitine levels, and a systemic form, with encephalopathy, hepatic dysfunction, muscle weakness and low muscle, liver and serum carnitine levels. Both types have typical lipid stores in muscle biopsy. We describe the case of a myopathic form of carnitine deficiency. Due to the age of the patient, this is an unusual case, with an unfavourable evolution. Therapeutic measures used in these patients have included prednisone, carnitine replacement and a low-fat with medium chain tryglycerides and high-carbohydrate diet. However, in none of the patients responding to therapy, a significative increase in muscle carnitine has been demonstrated.

Miopatía por déficit de carnitina: un caso de diagnóstico tardío / Carnitine deficiency myopathy: a case of late diagnosis

Las miopatías causadas por trastornos del metabolismo lipídico son un grupo patológico poco frecuente, entre las que destacan las asociadas a déficit de carnitina. En este grupo se distinguen dos formas clínicas: la forma miopática, que suele cursar con niveles séricos normales de carnitina y concentraciones descendidas en músculo, y la forma sistémica, que cursa con crisis de encefalopatía y disfunción hepática además de la clínica miopática, y en la que suelen estar descendidas las concentraciones de carnitina en suero, músculo e hígado. Ambas formas muestran en la biopsia acúmulos lipídicos característicos. Presentamos el caso de una forma miopática de déficit de carnitina, excepcional por la edad de la paciente en el momento del diagnóstico, cuya evolución fue desfavorable. Se ha descrito que ciertas modificaciones dietéticas, como el uso de triglicéridos de cadena media, la restricción de grasas o el incremento de carbohidratos de la dieta, así como la prednisona y los suplementos orales de carnitina, pueden producir mejorías clínicas, sin modificar significativamente las concentraciones musculares de carnitina (AU)

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[Comparative study of two lipid emulsions in the parenteral nutrition of the septic patient]. / Estudio comparativo de dos emulsiones lipídicas en la nutrición parenteral del enfermo séptico.

The nutritional support is essential in the treatment of the septic patient, but the optimal substrates, have not been well defined, although several studies advise the use of solutions with a high proportion of branched amino acids. The use of lipids in the Parenteral Nutrition (PN) of these patients is presently accepted by all authors, although there is no consensus of the composition of the mix to be used. We have done a prospective, randomized study in a group of critical septic patients who received PN. All cases were used a solution with a high proportion of branched amino acids and glucose. 40% of the non-protein calories were administered as lipids. Based on this, two groups were formed. Group 1 medium chain triglycerides and long chain triglycerides (MCT/LCT) in a ratio of 1:1, and Group II LCT. After ten days, both groups showed an increase in hepatic cholestasis enzymes and an improvement of the parameters of protein anabolism and catabolism. Group 1 showed a significantly higher increase in the retinol birdring protein and the improvement of the nitrogen balance. Besides this, the global analysis of all the parameters of protein anabolism and catabolism showed a more favorable evolution, with statistical significance, in those who received MCT/LCT. Therefore, the MCT/LCT in the PN of the septic patient who is given a solution rich in branched amino acids, achieves a greater protein saving and a faster recevesy of the markers of protein synthesis, than those with LCT. Both groups show an increase in cholestasis enzymes.

[Nutritional status in uremic patients treated with continuous ambulatory peritoneal dialysis. Relation with 1 year's evolution]. / Estado de nutrición en los pacientes urémicos tratados con diálisis peritoneal continua ambulatoria. Relación con la evolución en un año.

BACKGROUND: Signs of malnutrition have been detected in patients with chronic renal failure and a possible relation with complications of the disease has been speculated. The aim of the present study was to analyze the nutritional status of a group of patients with the aforementioned diagnosis on a substitutive treatment program with outpatient continuous peritoneal dialysis (CAPD) and its relation with complications over one year. METHODS: The nutritional status of 29 of these patients and 22 individuals with normal renal function (controls) with global parameters of body fat, muscular protein and visceral protein being evaluated. The study was repeated at 6 and 12 months after the initial evaluation. The incidence of mortality, need for hospital admission because of complication and episodes of clinical peritonitis were analyzed. RESULTS: More than 70% of the patients presented protein-caloric malnutrition to a greater or lesser degree. This percentage dit not modify throughout the study and was produced at the expense of visceral protein, additionally affecting the fat and muscular protein of the males. Mortality, need for admission and the number of cases of peritonitis were greater in those presenting the lowest fat parameters with these complications beings foreseen from determined fat values. CONCLUSIONS: These patients have malnutrition which is related with the complications in the course of substitutive therapy making surveillance and nutritional support necessary.

[Nocardiosis in a patient with AIDS]. / Nocardiosis en paciente con SIDA.

A young drug addict with positive anti-HIV antibodies, OKT4/OKT8 ratio below 1, oropharingeal candidiasis and pulmonary Pneumocystis carinii infection, dies due to a cerebral abscess with a septic status. The postmortem microbiologic study shows pulmonary abscesses and Nocardia asteroides is isolated from lung tissue and spinal fluid. We note out the rareness of this pathology and its relation to AIDS.

[Evolution of exocrine function of the pancreas after acute pancreatitis. Prognosis factors]. / Evolución de la función exocrina del páncreas tras la pancreatitis aguda. Factores pronósticos.

Using the NBT-PBA test, we studied pancreatic exocrine function in two groups of patients: one group with acute pancreatitis of lithiasic origin that underwent cholecystectomy and other with pancreatitis of alcoholic origin. The test was performed at the time of the acute picture and later at one month, three months, six months and a year. Regardless of etiology, exocrine pancreatic function was always impaired after acute pancreatitis, no case showing recuperation at one month of the picture but some did at three months (64% of the lithiasis group and 25% of the alcoholic group). We also attempted to relate the recuperation of the exocrine pancreas with the amylase levels, leukocytosis and calcemia encountered in the acute phase. The only correlation found was a poorer and later recuperation in the patients with the lowest calcemia levels during the acute picture.

[Possible effect of bromocriptine on the insulin receptor]. / Posible efecto de la bromocriptina sobre el receptor de insulina.

The administration of 0.00011 mg/g weight/day of bromocriptine (CB154) for 7 days to Wistar rats, improved the peripheral glucose uptake without significant changes in plasma insulin level, during the intravenous glucose tolerance test (0.33 g/kg). The mode of the bromocriptine action on binding of 125I insulin to erythrocyte insulin receptors has been evaluated. The total number of sites was greater with bromocriptine (513.1 +/- 124.1 pM/1,CB154 815.6 +/- 107.9 pM/l) (p less than 0.01). The high affinity/low capacity compound of insulin receptor, in CB154 rats (51.8 +/- 16.8 pM/l) was higher than in normal rats (18.3 +/- 8.9 pM/l) (p less than 0.005). Additional studies indicated that CB154 had no effect on the rate of association and dissociation of 125I insulin from rats erythrocyte insulin receptors. The degradation of insulin or the erythrocyte receptor sites do not change, after the treatment with CB154.