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Biomedical subjects

L Callis

Publications and source records attributed to L Callis.

At least 19 recordsLinked to original sources
PubMed2000

Towards guidelines for dialysis in children with end-stage renal disease.

There are few data describing the current practices of treatment selection for children with end-stage renal disease (ESRD). In an effort to establish a consensus among Spanish pediatric nephrologists for inclusion and exclusion criteria for renal replacement therapy in children with ESRD, in 1995 we surveyed members of the Spanish Pediatric Nephrology Association. Although only 43% of members responded, pediatric nephrologists and bioethicists studied the results and compiled a list of ten guidelines for treatment of children with ESRD. The proposed guidelines are meant to be a starting point for further discussion. An emphasis on flexibility, individual case assessment, and consideration of the best interests of the patient must remain central to any treatment plan. Decision making should ideally be shared by parents, professionals, the child, when appropriate, and ethics committees, as necessary.

Child↗
PubMed1999

Autosomal recessive Alport syndrome: linkage analysis and clinical features in two families.

BACKGROUND: Genetic heterogeneity is a well-known feature of Alport syndrome (AS). Most families with AS show an X-linked dominant pattern of inheritance but about 15% of families show an autosomal inheritance of the disease. Autosomal recessive AS may account for 10% of the total number of cases and is caused by mutations in the COL4A3 and COL4A4 genes. The clinical spectrum of this rare disorder has not been well clarified. METHODS: We present two families with AS. Two affected members of these families have entered end-stage renal disease (ESRD) in their 30s, and the other three are older than 15 years and have normal serum creatinine. Four of the five patients have deafness but none have ocular abnormalities. Two have been transplanted and have not suffered from anti-GBM antibody nephritis. Men and women are equally affected. We have performed linkage analysis for chromosome 2 with the following markers: D2S279, COL4A3/4 DNTR, COL4A4 RFLP Hae III. RESULTS: We demonstrate that both families, one of them consanguineous, are linked to the COL4A3/4 locus. CONCLUSIONS: We can conclude that the only significant difference between the X-linked and the autosomal recessive forms of AS lies in the fact that in the latter females are as affected as males; thus the idea that autosomal recessive AS causes ESRD during childhood must be discarded. Other clinical features such as age of deafness or the presence of post-transplant anti-GBM antibody nephritis show no differences between the entities. Thus an accurate familial study is mandatory in patients with AS, as the identification of the different patterns of inheritance may cause a great difference in genetic counselling. Linkage analysis is the only effective molecular diagnosis that can be performed nowadays.

Adolescent↗
PubMed1997

Tertiary hyperparathyroidism after renal transplantation.

Tertiary hyperparathyroidism is considered as an autonomous proliferation state of the parathyroid glands with biological hyperfunction resistant to calcium/vitamin suppressor therapy. This phenomenon is thought to be secondary to monoclonal inactivation of tumoral growth suppression factor located on chromosome 11. Three patients, 13, 15, and 22 years of age, with chronic renal insufficiency of long evolution who presented with tertiary hyperparathyroidism following renal transplantation are described. The three patients underwent subtotal parathyroidectomy with consequent normalization of biochemical parameters of phospho-calcium metabolism in the first few weeks post surgery. Pathologic study showed adenoma in the affected glands with hyperplasia of the rest. We believe that in patients with long-term renal insufficiency an aggressive treatment, either medical or surgical, of secondary hyperparathyroidism which is continued after renal transplantation may be useful in preventing the development of tertiary hyperparathyroidism.

Adenoma↗
PubMed1993

Renal calcifications in patients with autosomal recessive polycystic kidney disease: prevalence and cause.

OBJECTIVE: We investigated the prevalence of renal calcifications in children with autosomal recessive polycystic kidney disease and studied the metabolic changes that could cause this complication. SUBJECTS AND METHODS: Nine patients with known autosomal recessive polycystic kidney disease were examined with sonography and CT and screened for biochemical evidence of metabolic causes of nephrocalcinosis. RESULTS: CT showed bilateral renal calcifications in seven of the nine patients. The two patients without renal calcifications were less than 1 year old. Four patients had only a few calcifications and three patients had many. The severity of the renal calcifications correlated with the degree of kidney failure. All patients with kidney failure were found to have urine acidification defects. Hypocitraturia was present in all patients. CONCLUSION: Our results show that renal calcifications are common in older children with autosomal recessive polycystic kidney disease. Hypocitraturia and the urine acidification defect resulting from kidney failure are the leading factors in the pathogenesis of the calcifications.

Adolescent↗
PubMed1993

[Berger's disease in children: its form of presentation, pathological anatomy and evolution in 22 cases].

We have reviewed 22 cases of Berger's disease in children (glomerular nephritis with mesangial IgA deposits), all of which were diagnosed by renal biopsy between 1976 and the present time. We describe the clinical and pathological findings in these patients. In addition, we put special emphasis on the evolution of the disease in relationship to some of the parameters that have been reported in the literature as being related to a bad prognosis of glomerular function such as, massive proteinuria at the onset of the disease, histological classification, presence of deposits of IgM or fibrinogen derivatives and glomerular sclerosis. All of the patients started with hematuria, 21 of which had gross hematuria (95%). Fourteen patients (63%) showed proteinuria (2 of which also had a temporary nephrotic syndrome). Five children showed some transient decrease in glomerular filtration rate and another patient rapidly developed renal failure and then end stage renal disease. We were able to follow 15 children for 3 years: 8 (53%) still showed outbreaks of gross hematuria, 5 (33%) only had microhematuria and 2 (14%) showed no signs of hematuria. Four children (27%) still had proteinuria. The glomerular filtration rate was still normal in all but two children (one with rapid evolution to end stage renal disease and another with a glomerular filtration decrease of 20%). Ten children were followed for 6 to 13 years. After 6 years, 2 (20%) still showed outbreaks of gross hematuria, 1 (10%) still had proteinuria.(ABSTRACT TRUNCATED AT 250 WORDS)

Age Factors↗
PubMed1986

Renal arterial hypertension in pediatric patients.

Three hundred twenty-three children with hypertension due to different kidney disorders were analysed. Acute transient hypertension was the most frequent form of renal hypertension and was usually due to acute post-infectious glomerulonephritis. Chronic hypertension was frequently associated with renal insufficiency. In glomerular disorders the appearance of hypertension was usually accompanied by renal failure.

Adolescent↗
PubMed1986

Long-term treatment with captopril in pediatric patients with severe hypertension and chronic renal failure.

Captopril was given to 42 hypertensive patients aged 1 to 17 years who were treated by regular haemodialysis because of terminal renal failure. Initially all patients were on antihypertensive treatment without sufficient control of blood pressure. Under captopril all patients presented a significant decrease of both systolic and diastolic blood pressures. The mean decrease of systolic blood pressure was from 162 to 114 and that of diastolic blood pressure from 106 to 86 mmHg. Plasma renin activity rose significantly in all cases whereas plasma aldosterone dropped and reached normal levels for age. The maximum effective dosage was 3 mg/kg/day. Total duration of treatment with captopril ranged from 1 1/2 to 6 1/12 (mean 3 2/12) years. At last observation the dosage of captopril varied between 0.3 and 3 mg/kg/day.

Adolescent↗
PubMed1986

Long-term treatment with captopril in paediatric patients with severe hypertension and chronic renal failure.

Forty two children with end stage renal failure and hypertension on chronic haemodialysis have been treated with captopril for from 18 to 78 months. Satisfactory control has been obtained in doses of 0.3 to 3.0 mg/kg given every 24 or 48 hours. Tolerance was good. The results of the present study suggest that captopril is a suitable drug for long-term use in paediatric patients.

Adolescent↗
PubMed1984

[Nephropathy in Schoenlein-Henoch purpura. Long-term developmental aspects in 55 cases].

Fifty five patients with Schöelein-Henoch nephropathy were reviewed. These patients were divided into five groups according to the clinical picture initially presented: 1. Hematuria with proteinuria. 2. Hematuria with nephrotic syndrome. 3. Isolated hematuria. 4. Hematuria, nephrotic syndrome and renal failure. 5. Proteinuria. From a histopathological point of view, they were classified into six groups according to ISKDC criteria. A strong correlation was found between clinical presentation and histological lesion. The worst prognosis corresponded to patients with nephrotic syndrome and renal failure, whose biopsy revealed more than 75% of glomeruli with epithelial crescents.

Adolescent↗
PubMed1983

Survival time in cystinosis. A collaborative study.

In a retrospective study the overall survival time of 205 cystinotic patients of six countries was determined. The median survival time was 8.5 years. The median time for 'renal death' (age at death due to uraemia or age at starting renal replacement therapy) was 9.2 years. The youngest patient dying of renal death was 5.2 years. No sex difference in survival time was noticed. Furthermore no difference in survival time was noted between the different countries. The analysis of the overall survival curve indicates no clear differences between the infantile and adolescent types of cystinosis.

Adolescent↗
PubMed1980

Chlorambucil treatment in minimal lesion nephrotic syndrome: a reappraisal of its gonadal toxicity.

Twenty-six preadolescent children ( 16 boys and 10 girls) with minimal lesion nephrotic syndrome, who were either unresponsive to or dependent on corticosteroid therapy, were treated exclusively with chlorambucil. Evaluation of chlorambucil toxicity on the reproductive system was based on penile and testicular size, serum testosterone values, and sperm count. Nine boys had azoospermia and one had oligospermia. The other six, who received a mean cumulative dose of 10.2 mg/kg of chlorambucil, had a normal sperm count. However, we consider that the total safe dose of chlorambucil should not exceed 8.2 mg/kg, equivalent to six weeks treatment at a daily dose of 0.2 mg/kg. In the ten girls, pubertal development was normal; axillary and pubic hair, breast development, age at onset of menarche, and length of the menstrual cycle were all normal; however, the possibility of abnormalities in ovarian functions cannot be excluded.

Adolescent↗
PubMed1977

Studies on the site of renal sodium loss in two patients with cystinosis. Effects of hydrochlorothiazide on tubular sodium reabsorptive mechanisms.

The purpose of this study was to examine the effect of hypotonic saline diuresis on two patients with cystinosis, trying to localize the site of renal sodium loss. At the same time, we have performed the same hypotonic saline infusion under hydrochlorothiazide administration. Results seem to prove the proximal tubular localization of the salt-wasting syndrome in cystinotic patients. On the other hand, these results suggest that hydrochlorothiazide effect can be related to the hypovolemia due to the distal tubular action of hydrochlorothiazide that could inhibit the 'third' natriuretic factor, thus increasing the proximal tubular sodium reabsorption.

Absorption↗
PubMedDate not supplied

Infantile idiopathic hypercalciuria, high congenital myopia, and atypical macular coloboma: a new oculo-renal syndrome?

Four cases of infantile idiopathic hypercalciuria with high congenital myopia and atypical macular coloboma are discussed; two of them are brother and sister. The eye examination shows bilateral congenital myopia of more than 10 diopters in every case. In the fundus examination, there is a bilateral macular colobomatous lesion in two of them and monocular lesion in the remaining ones. Hypercalciuria is considered to be due to renal tubulus malformation. The macular atypical coloboma etiopathogenesis is discussed, with comment on the malformative, dystrophic and inflammatory hypothesis, having discarded the usual inflammatory etiologies. We have only found two cases similar to these in the literature, Given the relative scarce occurrence of each of the three mentioned clinical features we suggest the possibility of a new oculo-renal syndrome rather than a coincidental association.

Adolescent↗