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Biomedical subjects

S Mir

Publications and source records attributed to S Mir.

At least 19 recordsLinked to original sources

Clinicopathologic correlations of Henoch-Schönlein nephritis in Turkish children.

BACKGROUND: In this study, 66 patients with Henoch-Schönlein nephritis (HSN) were investigated clinicopathologically. METHODS: The patients were classified according to their initial presentation, histologic findings, recurrences of purpura, type of treatment and clinical outcome. Logistic regression analysis was performed. RESULTS: Sixty-eight percent of patients were hospitalized with mild renal disease. Most patients were evaluated as class I and II according to light microscopy. In addition to IgA deposition alone, 33% of patients showed IgA + C3 and 27% had IgA + IgG + C3 depositions. After the follow-up period of 3.3 years, 15 patients had minor urinary findings, 4 had active renal disease and 1 had renal insufficiency. Recurrences occurred in 37.9% of patients and 37.1% of patients with recurrences had persistent pathologic findings. Symptomatic treatment was given to 51.5% of patients, while 27.2% were given corticotherapy. CONCLUSIONS: Clinical presentation was found to be correlated with outcome. Recurrence of the disease and the type of the treatment also affected the outcome. It was also thought that mesangial IgG and C3 depositions may have a role in the pathogenesis of renal damage in HSN.

Adolescent

Effect of enalapril on proteinuria, phosphaturia, and calciuria in insulin-dependent diabetes.

Elevated urinary calcium and phosphate excretion have been observed in children with insulin-dependent diabetes mellitus (IDDM). This may be related to a defect in tubular reabsorption. It is well known that converting enzyme inhibition decreases microalbuminuria and may prevent or retard diabetic nephropathy. We investigated whether enalapril also improves the defect in calcium and phosphate reabsorption. We studied 16 children and young adults (age 12-21 years) with IDDM and persistent microalbuminuria before and during 12 weeks of enalapril treatment. Before treatment microalbuminuria, urinary calcium excretion, and fractional tubular phosphorus reabsorption (TPR) were 153+/-53 microg/min, 5.5+/-0.9 mg/kg per day, and 71.4+/-3.6%, respectively. At the end of the 12th week, microalbuminuria had decreased to 20.3+/-7.9 microg/min and calcium excretion to 3.3+/-0.4 mg/kg per day (P<0.01), while the TPR increased to 80.1+/-3.8% (NS). The renal threshold phosphate concentration increased from 1.8+/-0.15 to 2.92+/-0.23 mg/dl (P<0.01). The fasting serum glucose and hemoglobin Alc levels did not change significantly during the study. Systolic and diastolic blood pressures were 120.4+/-2.2 / 79.3+/-1.4 mm Hg and 110.5+/-1.8 / 71.3+/-0.9 mm Hg before and after 12 weeks, respectively. We conclude that enalapril treatment improves not only microalbuminuria but also abnormal calcium and phosphate excretion in microalbuminuric children with IDDM.

Adolescent

Plasma and urinary platelet activating factor concentrations and leukotriene releasing activity of leukocytes in steroid sensitive nephrotic syndrome of childhood.

Platelet activating factor (PAF) is synthesized and secreted by glomerular mesangial and endothelial cells. It increases glomerular basement membrane permeability and induces proteinuria. Leukotrienes (LT) are mediators released by either leukocytes or glomerular cells under the PAF effect. The possible role of PAF in steroid sensitive nephrotic syndrome (SSNS) of childhood was studied in 8 children with SSNS in the acute stage, 5 children in remission and 8 healthy controls. The PAF concentrations in urine and plasma were determined. Leukocytes were stimulated in vitro and the LT release in response to stimulation was determined. The urinary and plasma concentrations of PAF were significantly higher in the acute phase than in remission and in control patients. Children with SSNS were found to have peripheral leukocytes with increased LT releasing activity in vitro. These results are in accordance with clinical and experimental observations indicating that PAF originates in the kidney and plays a role in normal kidney physiology. Urinary PAF concentrations may be related to proteinuria because they were strongly correlated in the present study. Elevated plasma PAF concentrations in the acute stage of SSNS could result from either its secretion from the circulating leukocytes or decreased acetyl hidrolase activity needed for its hydrolysis in plasma. The increased LT release in vitro suggests that these cells might have been activated by PAF secreted from glomeruli. It is proposed that PAF and different LT in systemic and glomerular circulation are important mediators in childhood SSNS.

Adolescent

Pindolol augmentation of antidepressant therapy.

BACKGROUND: Antidepressant therapy is not always effective and is slow to take effect. In theory, these shortfalls may be caused by induction of neuronal negative feedback via pre-synaptic 5-HTIA receptors. Pindolol, an antagonist at somatodentritic pre-synaptic 5-HTIA receptors has been investigated as a potential accelerator and augmentor of antidepressant response. METHOD: A Medline search was conducted in November 1997. RESULTS: Six open-label studies and six controlled studies were identified for review. CONCLUSIONS: Open-label studies strongly suggest that pindolol may accelerate and augment antidepressant response, but controlled studies do not wholly support these findings: only three of six studies clearly demonstrate benefit. Larger, well-designed, controlled trials are needed to determine definitively the effectiveness of pindolol in this context.

Adrenergic beta-Antagonists

Does immunologic status predict peritonitis in children treated with CAPD?

The purpose of this study was to evaluate whether immunologic status could predict the development of peritonitis in children on continuous ambulatory peritoneal dialysis (CAPD). Twenty-one patients (12 boys, 9 girls) aged 10.1 +/- 4.32 years (range: 23 months to 14 years) were studied. The mean duration of CAPD treatment was 12.88 +/- 6.69 months (range: 2-22 months). Twelve healthy children (mean age 11.5 years) were selected as a control group. Lymphocyte subpopulations (CD3, CD4, CD8, CD19, NK, and IL-2R) were determined by double-color flow cytometry (Becton-Dickinson). Statistical evaluation was made by Student's t-test. CD4 (38.4 +/- 7.1% vs. 28.0 +/- 5.4%), CD4/CD8 ratio (1.30 +/- 0.4 vs. 0.96 +/- 0.3), and B lymphocyte (19.9 +/- 8.9% vs. 12.0 +/- 3.2%) levels were significantly higher in CAPD patients compared with controls. CAPD patients showed significantly lower natural killer (NK) cell values than controls (12.7 +/- 7.0% vs. 27.3 +/- 8.3%). Apart from CD19 values (21.9 +/- 10.4% vs. 12.0 +/- 3.2%) there were no significant differences between CAPD patients without infection and the control group in the laboratory parameters studied. On the other hand, CD3 (66.3 +/- 7.9% vs. 55.9 +/- 9.7%), CD4 (38.4 +/- 7.1% vs. 28.0 +/- 5.4%), and CD19 (6.3 +/- 3.3% vs. 12.0 +/- 3.2%) levels were significantly higher in CAPD patients with infection compared with the controls. CAPD patients with infection showed significantly lower NK activity (12.7 +/- 7.1% vs. 27.3 +/- 8.3%) than those in the control group. In conclusion, these results can explain the increased vulnerability to peritonitis in CAPD patients compared with healthy subjects. Additionally, immunologic status can predict the development of peritonitis in children treated with CAPD.

Adolescent

A comparative histologic study of the fibrillin microfibrillar system in the lens capsule of normal subjects and subjects with Marfan syndrome.

PURPOSE: To gain a better understanding of the pathogenesis of ectopia lentis and myopia in Marfan syndrome, studies were performed to determine the distribution and structure of fibrillin microfibrils in the lens capsule of normal subjects and of subjects with Marfan syndrome. METHODS: Frozen sections and/or flat mounts of lens capsules were prepared from six autopsy eyes, nine surgical capsulotomy specimens obtained at the time of cataract extraction, and five capsules from patients with Marfan syndrome obtained at intracapsular lens extraction. Avidin-biotin-peroxidase complex (ABC) immunoperoxidase or immunofluorescence staining with monoclonal antifibrillin antibody was used to localize fibrillin in lens capsules. Image analysis was also performed to compare the amount of fibrillin expression in normal and Marfan syndrome capsules. RESULTS: Based on fibrillin staining patterns, we identified three distinct zones in the equatorial and periequatorial regions of the normal lens capsule. Zone I, a 0.75-mm-wide peripheral ring of the anterior capsule, contained radial bundles of fibrillin fibers. In Zone II, a 1-mm-wide meshwork of fibrillin-rich fibers encircled the equator and served as an insertion platform for zonular fibers. Zone III was composed of radial, 0.1-mm-wide bands arranged in a periodic fashion in the most peripheral part of the posterior capsule. Fibrillin fibers were abnormal and disrupted in all three zones in patients with Marfan syndrome. The amount of fibrillin staining per unit area was significantly reduced in Marfan capsules compared with normal capsules (16-26% versus 49-56% per unit area, respectively; P < 0.001). CONCLUSIONS: Fibrillin was a major constituent of the peripheral and equatorial areas of the lens capsule. Zonular fibers, also rich in fibrillin, insert into the equatorial region, primarily in Zone II. Possibly, fibrillin played a role in the ability of the lens to change its configuration during accommodation. The observed qualitative and quantitative abnormalities in fibrillin expression in the lens capsule of patients with Marfan syndrome supported a causal relationship to lens abnormalities in these patients.

Actin Cytoskeleton

The adjustment of post-dialysis dry weight based on non-invasive measurements in children.

BACKGROUND: The clinical criteria to assess hydration status are not always reliable. Hence, the development of techniques to estimate more accurately post-dialysis dry weight (DW) remains a major challenge. The present study evaluates the value of the inferior vena cava (IVC) diameter, plasma concentration of atrial natriuretic peptide (ANP), and plasma renin activity (PRA) in determining the DW in chronic haemodialysis children. METHODS: Twelve overhydrated haemodialysis patients (4 girls, 8 boys) with a mean age of 12.8 were admitted to the study. Clinical, electrocardiographic, telecardiographic and echocardiographic findings, IVC and collapsibility indices and plasma concentrations of ANP and PRA were investigated before and after ultrafiltration (UF) therapy. Twelve age-matched normal children were studied as controls. Analysis of variance and Dunnett's test were applied for comparisons between patients and controls. RESULTS: Following UF therapy the patients' mean IVC collapsibility index was increased from 42.3 to 53.6% and IVC index was decreased from 1.08 to 0.81 cm/m2, both statistically significant. The pre-UF therapy collapsibility and IVC indices of the patient group were significantly different from those of the control group (56.9% and 0.70 cm/m2 respectively). The patients' mean plasma concentrations of ANP were 171 +/- 47.4 pg/ml before UF, 129 +/- 51.3 pg/ml after UF and 102 +/- 38.7 pg/ml in the control group. The ANP levels of the patients showed a significant decrease following the UF therapy. PRA was measured as 0.82 ng/ml/h before UF and 1.08 ng/ml/h after UF, but the increase was not statistically significant. CONCLUSIONS: Our findings revealed increased diameter of the IVC and plasma ANP concentrations and decreased collapsibility due to overhydration. Echography of IVC may be a promising non-invasive tool to estimate the DW in haemodialysis children. Further studies providing normative values for the IVC indices in both haemodialysis and normal children are required.

Adolescent

Image-directed and color Doppler ultrasonography in the diagnosis of postbiopsy arteriovenous fistulas of native kidneys.

Using image-directed and color Doppler ultrasonography (ICDUS), we examined 65 patients with single kidney biopsy and diagnosed one arteriovenous fistula (AVF) in each of 8 kidneys. Three of them were associated with pseudoaneurysms. Three of the patients with AVF who presented with macrohematuria underwent angiography. Therapeutic percutaneous embolization was performed in 2 of them. The remaining 6 patients were followed up with ICDUS. All the lesions had disappeared at the end of a 6-month period. We conclude that ICDUS is an easy and noninvasive imaging technique in the diagnosis of postbiopsy native renal AVFs.

Adolescent

Lymphocyte subpopulations in children with vitamin D deficient rickets.

Recent studies have shown 1,25(OH)2D3-mediated modulation of the immune system. We examined lymphocyte subpopulations of 16 children with nutritional rickets. Most of the patients suffered more frequent infection episodes than the control group of 15 healthy children and low serum levels of 25OHD and 1,25(OH)2D, such as 38.2 +/- 8.6 ng/mL and 15.7 +/- 2.6 pg/mL respectively. This decrease correlated with a significant decrease in total T lymphocytes and an increase in B lymphocytes expressing surface IgA, IgM, IgG molecules. These results suggest that vitamin D plays an important role in the impaired functions of T lymphocytes which may lead to frequent infection episodes in nutritional rickets.

Child, Preschool

Major histocompatibility complex antigens and immune mechanisms in steroid-responsive nephrotic syndrome.

Although the pathogenesis of steroid-responsive nephrotic syndrome (SRNS) is obscure, involvement of an immune mechanism is often suggested. Further evidence of an immune basis for this disorder is an increased frequency of specific major histocompatibility complex (MHC) antigens. In the first part of this study, the phenotypic frequency of HLA-A, -B, -C, -DR antigens were investigated in 30 children with SRNS and in 630 controls. In the second part, total T (CD3+ cells) and B lymphocytes (CD19+ cells) and the lymphocyte subsets (CD4+, CD8+ cells and their ratio) were studied in the same patients and in 30 healthy children. The investigations of all patients were performed during the acute stage and 14 of 30 during remission stage. Human leukocyte antigens (HLA) were determined by standard microlymphocytotoxicity assay and lymphocytes were analyzed by flow cytometry. Human leukocyte antigens A3, DR4, DR7 and the haplotype HLA-A2/B12 showed the strongest association with SRNS. In the studies for cellular immune disorder, CD3+ and CD8+ cells were found to be decreased significantly in the acute stage before beginning steroid therapy. No significant difference in lymphocyte subsets was observed in the remission stage without steroid or immunosuppressive therapy.

B-Lymphocytes

Why do UK-born Pakistani babies have high perinatal and neonatal mortality rates?

A prospective study of 4934 babies of different ethnic groups has confirmed the high perinatal mortality rate for Pakistanis and has shown that this was not due to a reluctance to terminate a fetus who is known to be seriously malformed. The major cause of early mortality was a high rate of lethal malformations, which occurred in about 1 in 100 Pakistani babies and which accounted for about half of their perinatal mortality. Many of these were autosomal recessive and occurred only in the offspring of consanguineous parents. However, there was also an excess of lethal cardiac malformations which were not associated with parental consanguinity. The remainder of the excess perinatal mortality was probably due to socio-economic factors. These causes are partly amenable to preventive measures, such as the referral of Pakistani women for expert ultrasonography at 18 to 20 weeks of pregnancy.

Cause of Death

Race, consanguinity and social features in Birmingham babies: a basis for prospective study.

STUDY OBJECTIVE: The aim of the study was to investigate the influence of consanguinity on children's health. DESIGN: The study is a prospective survey from birth to five years of a cohort of babies born in a multiracial community. This report details the initial findings on consanguinity. SETTING: Participating families live predominantly in three health districts of Birmingham, and were recruited in three local maternity hospitals. PARTICIPANTS: Babies of 2432 European mothers, 509 Afro-Caribbean mothers, 625 Indian mothers, 956 Pakistani mothers, and 216 Bangladeshi mothers have been enrolled in the study. Eighty mothers refused to participate. MEASUREMENTS AND RESULTS: Sociodemographic information was obtained using a structured questionnaire administered at interview. Interview data were supplemented with obstetric information from the medical records. The highest prevalence of parental consanguinity was in Pakistani Muslims (69%), whereas in Muslims from other countries it was 23%, and it was less than 1% in non-Muslims. In the majority of consanguineous Muslim pedigrees the degree of inbreeding was greater than that for first cousin parents. CONCLUSIONS: This prospective study will allow an assessment to be made about any ill health in childhood arising from parental consanguinity, about whether screening programmes are indicated for particular autosomal recessive diseases, and about whether premarital health education might be beneficial. The study has also documented parental ages in different races and this, together with the levels of parental consanguinity in all races, will be useful in genetic methods for assessing the frequency of recessive genes, the possibility of genetic heterogeneity, and whether or not parental age effect exists for new mutations of specific genetic disorders.

Adolescent

An experimental evaluation of microcapsules for arterial chemoembolization.

Microcapsules, 106 micron (range, 50-350 micron), of different capsular materials (monoglyceride, monodiglyceride, natural wax, cellulose polymer, or lactic acid polymer) with and without floxuridine (2'-deoxy-5-fluorouridine, FUDR) were intraarterially injected into dog kidneys. The drug-release characteristics of the microcapsules, as determined by analysis of renal and systemic venous blood samples over a 6-hour period, were uniphasic or multiphasic depending on the capsular material. Histologic changes of varying degrees were noted in all kidneys embolized except for those subjected to capsules of the cellulose polymer. The most striking changes were produced by the lactide polymer capsules. The potential applications of microencapsuled chemotherapeutic agents in intraarterial transcatheter treatment of cancer are discussed.

Animals

Transcatheter thrombolysis in cancer patients.

Arterial thrombolysis was successfully accomplished in 15 of 18 (83%) cancer patients by the continuous intraarterial transcatheter administration of streptokinase (70 units/kg/h or 5000 u/h and/or urokinase (400-500 u/kg/h) over 24-96 h. In 16 of these patients, the arterial thrombosis was a complication of transcatheter infusion of chemotherapeutic agents or embolization therapy. A combination of streptokinase, urokinase and heparin (10,000-25,000 U/day) was utilized without significant side effect and was effective in all 8 patients receiving treatment. Creating a tunnel through the clot, daily monitoring of the catheter with repositioning when necessary, and adjusting the type and dose of thrombolytic agent aided the therapeutic management.

Adolescent