Nutritional deficiency of vitamin D is common in developing countries as a result of both inadequate diet and exposure to ultraviolet light. The most striking biochemical finding in this illness is hypocalcemia. Reduction in serum calcium level may affect ventricular contraction. The purpose of this study was to evaluate prospectively left ventricular function in a group of 27 infants diagnosed as having rickets. Electrocardiograms and echocardiographic studies were undertaken in all patients. A group of ten healthy infants was used as a control for the echocardiographic examinations. Patients were divided into three groups according to the biochemical classification of rickets. There were eight patients in group I, nine in group II, and ten in group III. Abnormal electrocardiographic findings were noted in four infants in group I, three in group II, and six in group III before treatment of the rickets. These changes resolved following treatment. Echocardiographic studies revealed left ventricular dysfunction in the pretreatment stage. The most striking echocardiographic finding is the increase in the ratio of interventricular septal thickness to left ventricular posterior wall thickness in eight patients from group III. This returned to normal after treatment of the rickets. This study has demonstrated echocardiographic evidence of left ventricular dysfunction in children with rickets. These abnormalities were not, however, sufficiently severe to be associated with clinical signs of cardiac failure. Cardiomyopathy may develop in rickets, especially in the third stage of the disease, and this finding may return to normal following adequate treatment of the rickets.
Behçet’s disease (BD) is a multisystem inflammatory disorder of unknown etiology characterized by recurrent oral and genital ulcerations and uveitis, with varying other manifestations associated with vascular inflammation. A unifying feature of BD inflammation is the skin pathergy reaction (SPR), a nonspecific tissue hyperreactivity to minor trauma involving epithelial disruption. This study compared skin responses to needle prick in BD patients and normal healthy volunteers. Two study groups, each consisting of 10 BD patients with SPR+ and 6 controls, were evaluated using either immunohistochemistry or quantitative real-time PCR to measure inflammatory cell and cytokine levels in biopsy specimens obtained serially from independent sites at 0, 8, and 48 h after needle prick. We found similar cellular infiltration patterns in response to needle prick in BD patients and controls between 0 and 8 h. Further development of this immune response was limited in skin of normal control subjects, with stable or decreased inflammatory mediators observed at 48 h. In contrast, in BD-derived skin specimens, increased influxes of mature dendritic cells, monocytes, and lymphocytes, including T regulatory cells, were noted by 48 h. Similarly, increases in cytokines (IFN-γ, IL-12 p40, IL-15), chemokines (MIP3-α, IP-10, Mig, and iTac), and adhesion molecules (ICAM-1, VCAM-1) were noted at 48 h in the skin of BD patients with SPR+ but not in the skin of normal controls. These results suggest that, in contrast to the self-limited inflammation associated with epithelial disruption of normal skin, BD patients experience marked cellular influxes into the injury site, leading to an exaggerated lymphoid Th1-type response.
Guillain-Barre syndrome is an acquired demyelinating polyneuropathy that is presumed to be immune-mediated. On the basis of this assumption, intravenous immunoglobulin (IVIG) has been used in the treatment of Guillain-Barre syndrome in recent years and found to be effective. To test this we performed a randomized study in patients with Guillain-Barre syndrome by giving IVIG (1 g/kg body weight per day over 2 consecutive days) in 9 children who were compared with 9 patients who were observed but not given specific therapy. We concluded that intravenous immunoglobulin is a safe and effective treatment for childhood Guillain-Barre syndrome which shortens the time to recovery.
Background: The pustular skin lesions of Behçet's syndrome (BS) are clinically and histopathologically similar to ordinary acne, but BS patients get lesions at sites not commonly involved in acne, such as the legs and arms. The microbiology of these lesions has not been studied adequately. Objective: To make a detailed study of the microbiology of BS lesions. Methods: Subjects were patients with BS and acne vulgaris. Material was extracted from pustular lesions and directly plated to aerobic and anaerobic media by sterile swab. Anaerobic bacteria were identified using a commercial kit (API 20A). Aerobic bacteria were defined by standard procedures. Results: 58 BS patients and 37 acne patients were studied. Pustules were cultured from the following sites: BS patients (70 pustules): face (17), back (30), chest (2), arm (4), leg (17); acne patients (37 pustules): face (27), back (6), chest (1), arm (2), leg (1). At least one type of microorganism was grown from each pustule. Staphylococcus aureus (41/70, 58.6%, p = 0.008) and Prevotella spp (17/70, 24.3%, p = 0.002) were significantly more common in pustules from BS patients, and coagulase negative staphylococci (17/37, 45.9%, p = 0.007) in pustules from acne patients. Conclusions: The pustular lesions of BS are not usually sterile. The microbiology of these lesions is different from ordinary acne. It remains to be determined whether the infection is secondary or has any pathogenic implications.
Epileptic seizures during infancy have a wide variety of clinical presentations and the outcome differs according to the etiology. Among the benign and rare causes of infantile seizures, Vitamin B12 deficiency has been encountered. Common symptoms of Vitamin B12 deficiency in infants include megaloblastic anemia, feeding difficulties, developmental delay, microcephaly, failure to thrive, hypotonia, lethargy, irritability, involuntary movements, seizures and cerebral atrophy. Involuntary movements and seizures may rarely be the initial symptoms of Vitamin B12 deficiency. Involuntary movements have also been reported to appear after initiation of Vitamin B12 supplementation in isolated cases, whereas, no such information exits for seizures. In this paper, three infants with Vitamin B12 deficiency associated with motor and mental retardation are reported because of long-lasting focal/multifocal epileptic seizures following the initiation of intramuscular Vitamin B12 treatment. Antiepileptics were introduced in addition to Vitamin B12. Seizures disappeared within a few days or weeks; electroencephalographic findings were normalized in a few months. No relapses occurred during the follow-up period.
Low-dose depot corticosteroids did not have any beneficial effect on genital ulcers. However, it was useful in controlling erythema nodosum lesions, especially among the females.
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