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Biomedical subjects

I Zamora

Publications and source records attributed to I Zamora.

34 records · Page 2Linked to original sources

[Renal transplantation in children: preliminary report (author's transl)].

The preliminary results of a pediatric renal transplantation program started two years ago are presented. Fourteen children aged four to 13 years received a renal transplantation from April 1979 through March 1981. In eight patients renal graft was obtained from a living related donor (father-mother) and six from corpse donor. Recipients body weight ranged from 11 to 40 kg. (mean, 25 kg.). The previous time on hemodialysis was between three and 13 months (mean, seven months). In all cases a transfusion protocol prior to renal transplantation was followed (five transfusions minimum). Donor-recipient compatibility ranged zero-three HLA compatibilities. The patients were coordinately managed by pediatric specialists. The follow-up of the transplanted children ranges from one to 24 months (mean, 10 months). All of the patients are alive and the graft is functioning in all but one. A chronic rejection episode begun five months after renal-transplantation caused in one patients a graft loss. Plasma creatinine is lesser than 1.50 mg/100 ml. in 11 cases and inferior to 2 mg./100 ml. in the other two patients. The non-immunological complications presented have been unremarkable and no repercussions on the patients evolution have been detected. Even though the follow-up period is short, the results are highly encouraging, supporting that renal transplantation is the election treatment in children with terminal renal failure.

Adolescent↗

Lennert's lymphoma presenting with clusters of cutaneous infection.

We recently diagnosed and studied a case of Lennert's lymphoma in a young black man who presented with a chronic pyoderma and multiple cutaneous infections of viral origin. Lennert's lymphoma, or malignant lymphoma with a high content of epithelioid histiocytes, is an uncommon variant of non-Hodgkin's lymphoma. This report highlights the role of idiopathic immunosuppression in lymphoma patients.

Adult↗

[Incidence of renal involvement in Schönlein-Henoch syndrome (author's transl)].

To determine the incidence of renal involvement in Schönlein-Henoch syndrome, authors enrolled in a prospective study all children admitted with this diagnoses from 1971 through 1978. They followed-up 112 patients, which represented the 2 per one thousand of the total hospital pediatric admission in the same period; 22 patients (19.6%) developed renal involvement. After two years of follow-up they observe one patient with progressive renal failure, four with minor urinary abnormalities and 17 asymptomatics. The single patient with renal failure represents the 4.5% of the patients with Schönlein-Henoch nephropathy and the 0.9% of the total affected with this syndrome.

Child↗

Premature cataracts in a family with hidrotic ectodermal dysplasia.

In a family with hidrotic ectodermal dysplasia affecting five members in three generations, bilateral premature cataracts have developed in four of the five affected individuals. To our knowledge, this represents the first report of a family in which bilateral premature cataracts appear to be inherited with hidrotic ectodermal dysplasia.

Adult↗

The acidification defect in the syndrome of renal tubular acidosis with nerve deafness.

An 8-year-old boy with renal tubular acidosis and nerve deafness, has been followed for seven years. Repeated studies of his renal acidification defect showed that until the age of six years the tubular defect was mixed, proximal and distal (type 1,2 hybrid). After that age the defect of proximal acidification disappeared and the patient only presented a distal renal tubular acidosis type 1. When this is associated with nerve deafness, it is considered a distinct nosological entity.

Acid-Base Equilibrium↗

[Morbidity and mortality of acute renal failure in neonatal period (author's transl)].

A retrospective study of 35 newborn with acute renal failure is presented. The main causes of renal failure were neonatal hypoxia by asfixia or hemorrhagic shock (eight), congenital malformations (two) and hypertonic dehydration (25). Mortality rate was 22% including two neonates with severe congenital malformations. Sepsis was considered as the main complicating factor and often as inducer of renal failure. It was present on 55% of cases and on 75% of the deceased newborn. Cerebral injury was frequent but a follow-up study is necessary to establish the rate of neurologic sequelae. Early diagnosis and treatment of renal failure will decrease complications with improvement in prognosis. Etiological analysis of neonatal renal failure shows the need of a better health education of people and also medical control of pregnancy and perinatal period.

Acute Kidney Injury↗

[Long term renal function following acute failure in the newborn (author's transl)].

A long-term follow-up study of 27 survivors of acute renal failure in newborns was performed. A study of the evolution of renal function was possible in 20 cases, with follow-up periods ranging from 15 to 62 months (mean follow-up: 38 months). Renal function was determined by assessing glomerular filtration rate, free-water reabsorption, tubular reabsorption of phosphate and acidification ability. Results show a marked difference, according to the etiology of the acuete renal failure, between the two groups studied: 1) Most of those displaying renal failure following neonatal hipoxia, maintained persistent nephrological sequels. II) Parameters of renal function normalized between 6 and 12 months of age in those in which renal failure was related to hypovolemic shock, caused by hypertonic dehydration. Of the six patients with acute renal failure caused by neonatal hypoxia and shock (group 1), only one displayed normal renal function after 17 months. In the remaining fire patients, glomerular filtration rate continued to decrease even after three years. Three of them displayed urinary acidification disorders. An alteration in free-water reabsorption was found in the majority of the patients in both groups during the first year. This alteration persisted after three years in only four patients of group 1, although in relation to decreased glomerular filtration rate.

Acute Kidney Injury↗

Mannitol osmolar clearance in diabetes insipidus of children.

A modified technique of amnnitol-induced diuresis is described, in order to assess renal concentrating ability in infants and children. The infusion of 10% mannitol in 0.9% saline avoided the hypertonic saline overload and the fluid restriction period, both badly tolerated by infants and small children. In a control group of children aged from two months to seven years, the values of T(H2O) plotted against C(OSM) allowed to calculate the adjustment curve y=0.80x0.75, r=0.98 (p is less than 0.0001). In six patients with pituitary diabetes insipidus (PDI), the test was used in order to quantify the degree of ADH deficiency and evaluate the carbamazepine and clofibrate effect, in the renal concentrating mechanism. The test was tolerated perfectly in every case, obtaining qualitative and quantitative data and avoiding the hyponatremia and hypokalemia produced by the mannitol.

Carbamazepine↗

Selective effect of mineralocorticoid replacement therapy on renal acid excretion in congenital adrenal hyperplasia.

The renal acid excretion of eight children with salt-losing congenital adrenal hyperplasia, was studied in three different situations: before treatment (period I), under glucocorticoid therapy (period II) and when both glucocorticoid and mineralocorticoid were given as replacement treatment (period III). Although administration of glucocorticoid therapy alone allowed the correction of acidemia, normalization of urinary net acid excretion was achieved only after mineralocorticoid was added to the treatment.

Acid-Base Equilibrium↗

[Glomerulocystic kidney disease and hemolytic-uremic syndrome: clinicopathological case].

Glomerulocystic kidney is a heterogeneous group of conditions morphologically characterised by multiple cortical cysts apparently originated from a cystic dilation of the filtration space with atrophy of the glomerular tufts. We report a case of glomerulocystic kidney affecting a 13-year-old boy who underwent renal transplantation for end-stage renal disease following a haemolytic-uraemic syndrome diagnosed nine years ago. The absence of other stigmas (urinary obstruction, extrarenal congenital abnormalities and family history of cystic kidney disease) suggest that our observation is apparently a sporadic and acquired glomerulocystic kidney following a haemolytic-uraemic syndrome, an infrequent association previously reported only twice. Our histological and immunohistochemical findings suggest that the cysts in this rare condition are really of glomerular origin but the pathogenesis of cyst development remains unknown.

Adolescent↗

[Cytomegalovirus nephrotic syndrome].

The case of a 5 months old infant with a nephrotic syndrome after neonatal cytomegalovirus infection is reported. Genomic amplification nested-PCR for CMV was positive in renal biopsy. Treatment with gancyclovir was effective to maintain nephrotic syndrome remission. We stresses the importance to discharge an infections cause of the nephrotic syndrome of newborns and infants due to the possibility on curative treatment.

Antibodies, Viral↗

Congenital thymic cyst in the neck.

Cervical thymic cysts are rare. Thirty-four cases are reported in the English literature. Persistence of a part of thymopharyngeal duct may lead to the cyst formation in the neck. The majority of the cysts are seen in children under 10 years of age. There is marked variation in the size of the cysts. The majority of them are multilocular and the color of the fluid varies widely. The epithelial lining is variable; the majority of the cysts show stratified squamous epithelium. The characteristic histological features are Hassal's corpuscles, cholesterol crystals and aggregates of lymphocytes. Our patient was a four and a half year old female; she presented with a four month history of a painless swelling in the neck. The multilocular bluish-black cyst excised from the left side of the neck had all the histological features of a thymic cyst. A pre-operative diagnosis of such a cyst can be made only by bearing in mind the possibility of thymic remnants in the neck.

Child, Preschool↗