Transient scoliosis in a child with juvenile rheumatoid arthritis

An 8 year old girl was admitted to hospital complaining of arthralgia in a few large joints. According to the clinical course and serological tests, a diagnosis of a polyarticular type of juvenile rheumatoid arthritis was made. About 6 weeks after the onset, scoliosis was observed. The curvature regressed spontaneously over the following 6 months. Generally, scoliosis associated with juvenile rheumatoid arthritis has been noted a few years after the onset. A transient scoliosis in the early phase of this disease is rare.     

Idiopathic infantile hypercalcemia discovered in the newborn period

We report a male newborn with typical clinical signs of idiopathic infantile hypercalcemia (IIH); that is, hypercalcemia, hypercalciuria, an elfin face and nephrocalcinosis without giving Vitamin D₃ supplementation to the patient. He had been treated with a vitamin D-free, low calcium milk and rectal administration of exogenous calcitonin (elcatonin). The latter seemed to be more effective as a treatment for IIH. The serum calcium level came within the normal range and the serum 1,25-dihydroxyvitamin D₃ (1,25[OH]₂D₃) level decreased from 101.5 to 75.6 pg/mL with the treatments mentioned above. These results suggest that a high serum concentration of 1,25(OH)₂D₃ is part of the pathogenesis of IIH. However, we were not able to clarify the pathogenesis of the high serum concentration of 1,25(OH)₂D₃.     

Alloimmune Neonatal Neutropenia in Monozygous Twins: High-dose Intravenous Gammaglobulin Therapy

ABSTRACT. An indirect immunofluorescence test using a flow cytometer (FACS420) was useful for detection of antineutrophil antibodies (anti-NA2 antibodies) in a case of monozygous twins with alloimmune neonatal neutropenia. When high-dose intravenous gammaglobulin (300 mg/kg for five consecutive days) was given to one twin, the increase in neutrophils was not obvious. Although neutropenia persisted for about three months, no infections were observed in either of the twins.     

Inverse Relation of Body-Surface Activation-Recovery Interval and Recovery Time to Activation Time in Normal Subjects: Stronger Correlation and More Heterogeneous Distribution in Activation-Recovery Interval Than in Recovery Time

The activation-recovery interval (ARI), measured directly from the myocardium, has shown a good correlation with the action potential duration (APD) in experiments. APD has been reported to be inversely related to the activation time (AT). However, no studies have examined the correlation between the body-surface ARI and AT in normal subjects. Fifty normal subjects (25 men and 25 women) were studied to elucidate the relationship between the body-surface ARI and AT. The body-surface AT was defined as the duration between the QRS onset and the minimum dV/dt of the QRS wave, and ARI as the interval between the minimum dV/dt of the QRS wave and the maximum dV/dt of the T wave in each lead of an 87 unipolar lead system. We also measured the recovery time (RT) defined as the duration between the QRS onset and the maximum dV/dt of the T wave. ARI was inversely correlated with AT (r = -0.73). RT was also inversely correlated with AT (r = -0.61), however, RT had a less heterogeneous distribution than ARI (148 ms vs 159 ms). There were no differences between male and female subjects in the relation between ARI and RT or in the body-surface distribution of ARI and RT. These findings suggest that the body-surface ARI may reflect recovery properties over the cardiac surface and that APD may distribute inhomogsneously over the human cardiac surface with a longer RT over an area with a shorter AT. ARI calculated from body-surface ECG may be a useful noninvasive and repeatedly measurable estimate of APD.     

Five cases of necrotizing fasciitis: Lack of skin inflammatory signs as a clinical clue for the fulminant type

Herein, we describe five patients with necrotizing fasciitis (NF) who had variable outcomes and clinical manifestations. At the onset, all patients exhibited purpura with or without blister and ulceration accompanied by severe pain and tenderness in the affected skin. Out of five patients, three lacked inflammatory signs such as redness and heat, and two of the three patients showed fulminant progression and died despite intensive treatments including surgical debridement, antimicrobial therapy, close monitoring and physiological support. Tissue specimens from the patients without skin inflammatory signs showed mild neutrophil infiltration in addition to necrosis from the epidermis to subcutaneous fat, and variable amounts of thrombi. Furthermore, numerous bacteria were detected by Gram stain. By contrast, the remaining two patients with skin inflammatory signs revealed slower progression, and tissue specimens from both patients showed heavy neutrophil infiltration, but bacteria were hardly detected. Therefore, these cases suggest the possibility that the paucity of skin inflammatory signs, such as redness and heat, in NF may be a clinical clue to predict the fulminant type.     

Functional ablation of afferent nerves aggravates dextran sulphate sodium-induced colonic damage in rats

Dextran sulphate sodium (DSS) is an oral agent capable of inducing chronic diarrhoea and colonic inflammation and necrosis in rats. The role of the afferent nerves in this model of colonic mucosal damage is not known. The hypothesis that functional ablation of the capsaicin-sensitive afferent nerves will aggravate DSS-induced colonic damage in rats was tested. Capsaicin pretreatment was used to ablate afferent nerve function and DSS was administered in the drinking water. Control rats received vehicle pretreatment and water without DSS. There were significant correlations between diarrhoea score, mucosal neutrophil infiltration, mucosal necrosis, and anaemia. Capsaicin pretreatment increased diarrhoea score and colonic mucosal neutrophil infiltration in the rats with colonic damage after 2 or 14 days of DSS. In addition, it induced anaemia and mortality in rats after 14 days of DSS. The data supports the hypothesis that functional ablation of the capsaicin-sensitive afferent nerves aggravates the colonic damage induced by DSS.     

Ultrastructural study of Betz cells in the primary motor cortex of the human brain

The ultrastructure of Betz cells in the 5th layer of the primary motor cortex of 17 neurologically and psychiatrically normal control individuals was studied. Normal-appearing Betz cells showed a wide range of features including novel electron-dense inclusion bodies (Bunina-like bodies) resembling Bunina bodies characteristic of amyotrophic lateral sclerosis (ALS), accumulations of neurofilaments (10 nm in diameter), bundles of filaments (20–25 nm in diameter) thicker than neurofilaments, lamellar structures, lamellar bodies and structures similar to Hirano bodies. Among these 'abnormal' features, the presence of Bunina-like bodies may be an age-related nonspecific degenerative change, since they appeared more frequently in elderly individuals. The presence of these abnormal features—particularly the Bunina-like bodies—in the Betz cells of normal human brains must be considered in the assessment of the pathognomonic significance of such structures in ALS and other neurological diseases that affect the motor cortex.