ABSTRACT
OBJECTIVE: Having previously demonstrated release of histamine from mast-cell-deficient rat aorta, the objective of this study was to determine and localize histamine synthesis capability in the aorta by detecting histidine decarboxylase (HDC), the enzyme that catalyzes histamine formation. MATERIALS AND METHODS: Experiments were conducted with nested reverse transcription-polymerase chain reaction (nRT-PCR) to detect HDC mRNA and with immunofluorescence and western blot analysis to detect HDC protein in rat aorta, cultured rat aortic smooth muscle (RASMC) and endothelial cells (RAEC). RESULTS: Gel electrophoresis of nRT-PCR products indicated HDC mRNA in liver, aorta and RASMC but not in RAEC or kidney. Sequence analysis confirmed that the band observed in RASMC was the target HDC amplicon. Immunofluorescence indicated the presence of HDC protein in RASMC and not in RAEC. Western Blot analysis revealed HDC protein (55 kDa) in liver, aorta, RASMC but not in RAEC or kidney. CONCLUSIONS: The results of this study are the first to demonstrate the presence of HDC mRNA and protein in rat aorta and more specifically in RASMC, indicative of their capability to synthesize histamine.
Subject(s)
Aorta/cytology , Aorta/enzymology , Histidine Decarboxylase/genetics , Histidine Decarboxylase/metabolism , Muscle, Smooth, Vascular/enzymology , Myocytes, Smooth Muscle/enzymology , Animals , Blotting, Western , Cells, Cultured , Escherichia coli/genetics , Fluorescent Antibody Technique , Muscle, Smooth, Vascular/cytology , RNA, Messenger/genetics , RNA, Messenger/metabolism , Rats , Rats, Inbred F344 , Reverse Transcriptase Polymerase Chain ReactionABSTRACT
Erythema nodosum leprosum (ENL) classically presents as tender, erythematous nodules over the face, arms and legs. Severe ENL can become vesicular or bullous and break-down and is termed erythema necroticans (Jopling & McDougall, 1996) and is treated with corticosteroids. The causes of death in a majority of leprosy patients are the same as in the general population, with the exception of renal damage in lepromatous leprosy. There is possible increased mortality from side-effects of antileprosy drugs, steroids, or other drugs used in reactions, from toxaemia in severe reactions, and from asphyxia due to glottic oedema (Jopling & McDougall, 1996). We report here a case of erythema necroticans, the cause of death being septicaemia, secondary to skin ulcers and urinary tract infection, precipitated by corticosteroids.
Subject(s)
Adrenal Cortex Hormones/adverse effects , Erythema Nodosum/pathology , Leprosy, Lepromatous/pathology , Skin Diseases, Vesiculobullous/pathology , Adrenal Cortex Hormones/therapeutic use , Anti-Infective Agents/therapeutic use , Ciprofloxacin/therapeutic use , Erythema Nodosum/drug therapy , Fatal Outcome , Humans , Leprosy, Lepromatous/drug therapy , Male , Middle Aged , Skin Diseases, Vesiculobullous/drug therapyABSTRACT
Parents of 42 patients with Lesch-Nyhan disease completed a questionnaire systematizing caregiver observations of the subject's behavior during a wide variety of daily events. Responses were grouped in nine categories reflecting different aspects of cognitive skills. Only 1 boy appears to have any significant generalized cognitive impairment. The patients' memory for both recent and past events is excellent, their emotional life has a normal range of reactions and is appropriate; they have good concentration, are capable of abstract reasoning, have good self-awareness, and are highly social. However, they are behind in academic ability, with only 15% at grade level for math and reading. Implications for designing educational activities, parenting or caregiver strategies, and research methodology are discussed.
Subject(s)
Achievement , Intelligence , Lesch-Nyhan Syndrome/diagnosis , Neuropsychological Tests , Adolescent , Adult , Awareness , Behavior Therapy , Child , Child, Preschool , Combined Modality Therapy , Female , Humans , Intellectual Disability/diagnosis , Intellectual Disability/psychology , Intellectual Disability/therapy , Language Development Disorders/diagnosis , Language Development Disorders/psychology , Language Development Disorders/therapy , Lesch-Nyhan Syndrome/psychology , Lesch-Nyhan Syndrome/therapy , Male , Self-Injurious Behavior/diagnosis , Self-Injurious Behavior/psychology , Self-Injurious Behavior/therapy , Social Adjustment , Social Behavior , Social Environment , ThinkingABSTRACT
Studies of myocardial function in patients with hypophosphatemia have yielded conflicting results. Systolic time intervals were performed in 19 patients during and after the correction of hypophosphatemia; 11 had severe (0.9 +/- 0.15 mg/dL) and eight had moderate (1.4 +/- 0.11 mg/dL) hypophosphatemia. Controls were 14 patients with normal serum phosphorus levels. No patient with hypophosphatemia had clinical congestive heart failure. When hypophosphatemia was corrected, improvement in left ventricular performance was seen only in patients with severe hypophosphatemia (p less than 0.001); in eight patients left ventricular performance was normal during hypophosphatemia but showed significant improvement with its correction (p less than 0.01). Patients with moderate hypophosphatemia showed no significant change. Our results confirm the findings of O'Conner et al, whose study is the only previous one to demonstrate hypophosphatemia-induced myocardial depression in humans. Contradictory results from other studies may be explained by the inclusion of patients with moderate hypophosphatemia and failure to repeat measurements after the correction of hypophosphatemia. We conclude that reversible depression of myocardial performance is seen in hypophosphatemia only when it is severe. In some cases, normal left ventricular performance improves when hypophosphatemia is corrected.
