Significance of tubulointerstitial fibrosis in paediatric IgM nephropathy

Summary: The significance of tubulointerstitial fibrosis in paediatric patients with primary diffuse mesangial proliferative glomerulonephritis and IgM deposition (IgM nephropathy) has not been well documented. the clinical course, therapeutic response and final outcome of 35 patients in whom renal biopsies showed IgM nephropathy are reported. They have been subdivided into two groups according to the absence (19 patients: group I) or presence (16 patients: group II) of superimposed lesion of tubulointerstitial fibrosis. Clinical presentation was similar in both groups but the patients in group I were male predominant (2.8:1 vs 1: 1). 6/19 (31.6%) of the patients in group I and 1/16 (6.3%) in group II responded to corticosteroid, 11/19 (57.9%) in group I and 8/16 (50.0%) in group II had steroid dependent, whereas 2/19 (10.5%) in group I and 7/16 (43.7%) in group II had steroid resistant. About 42% of the patients in group I and 94% in group II required cyclophosphamide therapy by which similar response in both groups (approximately 75%) were observed. the steroid non-responders and cyclophosphosphamide therapy among patients in group II were significantly higher ( P <0.05) than the group I. At the latest assessment, 5/16 (31.3%) in group I and 7/14 (50%) in group II had impaired renal function. the follow-up period in both groups were 3.1 ± 2.8 and 3.4 ± 2.9 years, respectively. In conclusion, the finding of tubulointerstitial fibrosis in a paediatric IgM nephropathy indicates an unfavourable therapeutic response.     

Factors related to in-hospital heart failure are very different for unstable angina and non-ST elevation myocardial infarction

Non-ST-elevation myocardial infarction (NSTEMI) and unstable angina (UA) resulted in different degrees of damage to the heart muscle, and yet, when factors related to in-hospital outcomes were examined, these two subsets were often lumped together as non-STelevation acute coronary syndrome. Therefore, we investigated predictors of in-hospital heart failure (HF) in UA and NSTEMI separately. Factors related to HF (Killip ≥ 2) were analyzed for NSTEMI and UA in a Thai Acute Coronary Syndrome (ACS) registry conducted in 17 institutions between 2002 and 2005. The registry comprised of 9373 single admissions age 65.1 ± 12.3 years, 40.2% women, and 45.1% with HF. There were 3548 NSTEMI and 1989 UA with HF prevalence of 56.2% and 27.4%, respectively. Heart failure patients were older, more were women, sicker (as shown by more of those with shock, postcardiac arrest, and breathless on admission), more with diabetes mellitus (DM), received less intervention and medication, and showed higher total death (19.3% vs 5.3% for NSTEMI with and without HF; and correspondingly, 5.9% and 1.9% for UA). Independent predictors (at presentation) for the development of HF following NSTEMI or UA were age (not sex), breathlessness, and less prevalence of chest pain. However, shock and DM were risks only for NSTEMI but not UA. Heart failure was found to be a factor for in-hospital death for NSTEMI only, with odds ratio of 2.84 (confidence interval 2.11–3.82) and 3.23 (2.25–4.64) for total and cardiac deaths, respectively. Non-ST-elevation myocardial infarction and UA showed substantial differences in factors related to predictors for in-hospital outcome such that these should be examined separately.     

Diagnostic study of urinary frequency in children

A urologic study of 28 patients (11 males and 17 females, 4 to 14 years old) revealed that 23 patients (83%) had organic disease, 20 of whom had lower urinary tract abnormalities. Of 22 patients who were assessed psychologically, 14 had difficulty in adaptation, 20 had special birth order problems, and in 14 onset of symptoms occurred before school age. After five years of follow-up, 18 of 20 patients who were re-evaluated had no recurrent urinary frequency symptoms.     

Can a community‐based multidisciplinary intervention effectively restore renal function? A non‐randomized clinical trial

There is insufficient evidence in the literature to indicate whether the promotion of lifestyle changes to prevent renal dysfunction and activate regeneration is effective. We examined the effectiveness of a community‐based intervention program on renal restoration using a non‐randomized clinical trial design with a follow‐up period of six months. The training focused on lifestyle changes, including health education and personal consultation with a multidisciplinary health team. Our study included 120 patients with fractional excretion of magnesium levels of > 2%, who were assigned to experimental or control groups. Fractional excretion of magnesium levels were significantly decreased within six months in the experimental group; however, changes in the estimated glomerular filtration rate were not identified in either of the groups. These results suggest that screening of the fractional excretion of magnesium level and the “Healthy Kidney Program” had an effect in preventing chronic kidney disease or restoring kidney function.     

Cutaneous Manifestations of Childhood Systemic Lupus Erythematosus

Abstract: We assessed the mucocutaneous signs in 57 children with classical systemic lupus erythematosus seen during a 6 year period. The female:male ratio was 4.2:1. Ages ranged from 4 to 15 years (mean 11.9 years) at the time of diagnosis. Cutaneous manifestations (77%) were the second most common finding, next to renal involvement (84%). The skin changes noted were malar rash (74%), oral ulcer (46%), vasculitis (42%), photosensitivity (40%), alopecia (32%), and discoid lupus erythematosus (LE) (19%). All 11 discoid LE patients were girls. Periungual erythema, Raynaud's phenomenon, periungual gangrene, nail involvement, and subacute LE were rare. Antinuclear antibody reaction was positive in 93% and anti-dsDNA was positive in 46%. Eight patients died, six from severe infection and two from renal failure.     

Real prevalence of neural tube defects in Japan: How many of such pregnancies have been terminated?

The vital role of folic acid is to reduce the risk of having a neonate afflicted with neural tube defects. The prevalence of neural tube defects (myelomeningocele and anencephaly) has been reported in an incomplete form over the last 40 years in Japan. We aimed to evaluate the total number of neural tube defects including those delivered or terminated, to clarify the proportion of those terminated, and to internationally compare their prevalence. Through information on >311 000 deliveries obtained from 262 hospitals/clinics for 2 years of 2014 and 2015, we identified that the rate of total neural tube defects (termination of pregnancy, live births and stillbirths) was 8.29 per 10 000 deliveries for the year 2014 and was 8.72 for 2015, which were 1.5 and 1.6 times higher than the respective values (live births and stillbirths) reported. It is also observed that the ratio of the total number of myelomeningocele (termination of pregnancy, live births, and stillbirths) to that of anencephaly was approximately 1:1.2, that a half of pregnancies afflicted with neural tube defects were terminated, and that the proportion of termination of pregnancy due to myelomeningocele and due to anencephaly was 20% and 80%, respectively. Internationally, the real prevalence of neural tube defects in Japan was comparatively high, ranking fifth among the seven developed countries. In conclusion, the real prevalence of total neural tube defects was approximately 1.5 times higher than that currently reported by the Japan Association of Obstetricians and Gynecologists.     

Flow cytometric detection of recombinant human granulocyte-colony stimulating factor binding to leukemic cells

To examine binding of recombinant human granulocyte-colony stimulating factor to myeloid cells, the factor was labeled with fluorescein isothiocyanate, and incubated with blood specimens, which were then analyzed by flow cytometry. Neutrophils demonstrated an increased fluorescence, while lymphocytes were negative. These cell fractions were used as controls for cytometric binding assays of leukemic cells. Six patients with lymphocytic leukemia were negative in this assay. Ten of 15 patients with myelocytic leukemia were positive. All patients (n = 5) in myeloblastic crisis of chronic myelogenous leukemia were also positive. The flow cytometry results correlated well with the results of colony formation in response to granulocyte-colony stimulating factor. The results indicate that our method is useful in predicting the susceptibility of leukemic cells to recombinant growth factors. © 1993 Wiley-Liss, Inc.