ABSTRACT
In 16 children with multisystem Langerhans cell histiocytosis (mean age 22 months, range 5 to 36 months) severe symptomatic skin involvement was treated with topical nitrogen mustard (mechlorethamine hydrochloride). In each case, rapid clinical improvement occurred within 10 days; subsequent complete healing was observed in 14 children, and partial healing in 2 others in whom treatment was a component of palliative care. Mean duration of treatment was 3.5 months (range 2 to 6 months). Systemic treatment was averted in 11 patients because response to topical therapy was so favorable, but bone marrow or respiratory failure led to a fatal outcome in 5 other patients. Adverse effects were minimal. One patient developed contact allergy to topical nitrogen mustard after 2 years of intermittent therapy, but was successfully desensitized and was then able to continue treatment. We conclude that the topical application of nitrogen mustard is an effective treatment for cutaneous Langerhans cell histiocytosis. Although adverse effects were minimal in the short term, there remains concern about the possibility of long-term cutaneous carcinogenicity.
Subject(s)
Histiocytosis, Langerhans-Cell/drug therapy , Mechlorethamine/administration & dosage , Skin Diseases/drug therapy , Administration, Topical , Child, Preschool , Drug Evaluation , Female , Humans , Infant , Male , Mechlorethamine/adverse effects , Powders , Remission Induction , SolutionsABSTRACT
Nineteen children with hypomelanosis of Ito are described. Fourteen were developmentally delayed and nine had a history of seizures. Hemihypertrophy was present in four patients, syndactyly in three, and scoliosis in one. Twelve of the children had abnormal electroencephalograms and nine had abnormal brain scans, four with appearances suggestive of abnormal neuronal migration. There is very little evidence, either from the literature or from our patients, that the disease is inherited. The pattern of the cutaneous lesions suggests that the condition may result from the presence of two different cell populations as a result of mosaicism.
Subject(s)
Mosaicism , Pigmentation Disorders/genetics , Brain/diagnostic imaging , Child , Electroencephalography , Female , Genetic Linkage , Humans , Infant , Infant, Newborn , Male , Pigmentation Disorders/diagnostic imaging , Pigmentation Disorders/physiopathology , Radiography , X ChromosomeSubject(s)
Kidney/physiology , Nephrectomy , Caseins , Child , Child, Preschool , Dietary Proteins , Female , Humans , Kidney Function Tests , Kidney Neoplasms/surgery , Male , Postoperative Period , Wilms Tumor/surgerySubject(s)
Child, Preschool , Child , Humans , Male , Female , beta 2-Microglobulin , Urinary Tract InfectionsABSTRACT
Se estudio la influencia de la edad y el sexo en la excrecion de leucocitos y eritrocitos en la orina de 177 ninos sanos, los que se dividieron en tres grupos segun la edad: de dos a cinco, de seis a 11 y de 12 a 16 anos. El grupo de 12 a 16 anos excreto mayor cantidad de leucocitos y eritrocitos que los grupos de menor edad. La excrecion de leucocitos en las minas fue ligeramente mayor que en los ninos.Las cifras de excrecion de leucocitos obtenidos en la percentila 97 fueron de 500, 600 y 1000 por minuto en ninos; de eritrocitos de 1000, 850 y 1100 por minuto en ninas;de eritrocitos de 800, 800 y 950 por minuto en ninos y de 700, 800 y 850 por minuto en ninas, respectivamente por grupo de edad
Subject(s)
Child, Preschool , Child , Adolescent , Erythrocyte Count , Leukocyte Count , UrineABSTRACT
The association between urinary tract infection and diarrhea was investigated in 35 infants with acute gastroenteritis; one case (3%) showed this association, which is less than that observed in previous reports. The clinical picture and laboratory data were not useful for the differentiation of the positive case; the urinalysis was abnormal in the 35 patients. The urine sample for culture taken with the micturition technique, is frequently contamined. The bladder suprapubic puncture for the correct diagnosis of urinary infection is reliable and safe.
Subject(s)
Bacteria/isolation & purification , Diarrhea, Infantile/microbiology , Gastroenteritis/microbiology , Urinary Tract Infections/microbiology , Acute Disease , Diagnosis, Differential , Diarrhea, Infantile/urine , Female , Gastroenteritis/urine , Humans , Infant , Infant, Newborn , Male , Urinary Tract Infections/urineABSTRACT
Seventy-eight A-V fistulas were constructed in 45 children whoses age were between 4 to 16 years, in order to use it for chronic hemodialysis. Twenty-four of them were external A-V shunts which were occluded by thrombosis in 13 instances and deinserted in 3 patients. Thirty-six A-V fistulas were performed with saphenous wein autograft between an artery and vein at the forearm. The main complication in this group was thormbosis in 10 instances and dilations of the graft in 5. In 9 patients, latero-lateral arteriovenous anasthomosis was performed. In these, adequate blood flow developed slowly or was poor in most patients. Six patients with saphenous-femoral anasthomosis developed thrombosis in 3 instances and venous puncture was frequently difficult. In three other patients, bovine carotid artery graft was very efficient to give a good access to circulation for hemodialysis. There was no evidence of local or cardiovascular complications associated with fistulas.
Subject(s)
Acute Kidney Injury/therapy , Arteriovenous Shunt, Surgical/methods , Renal Dialysis , Adolescent , Animals , Carotid Arteries/transplantation , Cattle , Child , Child, Preschool , Female , Femoral Vein/transplantation , Humans , Male , Saphenous Vein/transplantation , Transplantation, Autologous , Transplantation, HeterologousABSTRACT
Six uremic dhildren in periodic hemodialysis with protein-calorie malnutrition were studied. Three of them were given diet supplementation with a compound constituted by carbohydrates and essential amino acids. Evaluation at ,3 and 6 months with somatometry, rutine laboratory analysis, intravenous glucose tolerance test and plasma amino acid determinations, showed that patients with diet supplementation had a slight increase in height and body weight, improved glucose in tolerance, that was initialy detected, and an abnormal pattern of plasma amino acids not modified during the study. Patients without diet supplementation showed no changes in height, body weight, glucose tolerance and plasma amino acids. These results suggest that diet supplementation with carbohydrates and amino acids is useful to improve nutrition in uremic children on hemodialysis, but it is neccesary to study more patients.
Subject(s)
Acute Kidney Injury/therapy , Amino Acids, Essential/therapeutic use , Dietary Carbohydrates/therapeutic use , Protein-Energy Malnutrition/diet therapy , Adolescent , Body Height , Body Weight , Evaluation Studies as Topic , Glucose Tolerance Test , Humans , Renal DialysisABSTRACT
In three hypertensive uremic children in whom hypertension was not controlled by sodium-free diet, ultrafiltration and hypotensive drugs, a total of twenty low sodium concentration hemodialyses were carried out. In all patients there was insignificant sodium excretion in urine and diastolic blood pressure diminished 10 to 20 mm Hg; two patients showed also reduction of systolic blood pressure. No important changes were seen in the serum concentrations of sodium, calcium and glucose. In one patient improvement of his retin lesions, as well as radiological reduction of heart size were observed. In other two cases, cardio-respiratory symptoms associated with hypertension disappeared. In all but two of them hypotensive drugs were withdrawn; in the other one dose was reduced. With low sodium concentration hemodialysis, sodium extraction was higher than that obtained when using the conventional dialysis solution. Interchangeable sodium decreased in all patients.
Subject(s)
Acute Kidney Injury/complications , Hypertension/complications , Renal Dialysis/methods , Acute Kidney Injury/therapy , Adolescent , Child , Female , Humans , Hypertension/therapy , Hypotonic Solutions , Male , Sodium Chloride/therapeutic useABSTRACT
Nine infants without evidence of nephropathy were studied. After a control period, furosemide, 1.0 mg/kg. of body weight, was administered intravenously and urine was collected. In all patients, urinary volume increased from a mean of 0.15 ml/min. during the control period, to 0.69 ml/min. after furosemide and urinary sodium excretion rose from 1.77 micro Eq/min. in the first period, to 50.13 micro Eq/min. in the second. There was no significant change in urinary osmolarity and in serum electrolytes. Three hours after furosemide, all children showed dehydration from light to moderate and saline infusion was necessary. No correlation was found between age and weight of the infants with the response to furosemide. Because this diuretic has an energetic action in infants as in children and adults, it must be used carefully and it is proposed that the initial dose of furosemide in infants be 0.5 mg/kg.
Subject(s)
Diuresis/drug effects , Furosemide/administration & dosage , Natriuresis/drug effects , Age Factors , Birth Weight , Drug Evaluation , Female , Furosemide/pharmacology , Humans , Infant , Infant, Newborn , Injections, Intravenous , Kidney/drug effects , Male , Osmolar Concentration , Sodium/urineABSTRACT
Two patients with shunt nephritis are reported. One of the patients had a positive blood culture to Staphylococcus albus; the kidney biopsy showed an immune complex disease. In the second patient, Klebsiella sp., was cultured in the blood, and this is the first report with such bacteria as the cause of nephritis. The finding of fibrin in the glomeruli by light, immunofluorescence and electron microsocopy studies, suggests a pathogenesis different from intravascular coagulation. In both patients, nephritis became inactive when the shunt was removed.
Subject(s)
Endocarditis, Bacterial/complications , Glomerulonephritis/etiology , Hydrocephalus/surgery , Klebsiella Infections/complications , Staphylococcal Infections/complications , Cerebrospinal Fluid Shunts/adverse effects , Child , Child, Preschool , Glomerulonephritis/microbiology , Humans , MaleABSTRACT
There is a high correlation index (0.97) in the measurement of urine osmolality and specific gravity in healthy children and adults and in children with renal disease without heavy proteinuria or glucosuria. This is a useful tool, because osmometers are not used in many laboratories, but specific gravity is of general use, so that urine concentration in mOsm/kg. can be derived from the specific gravity. The same parameters in urines with heavy proteinuria or glucosuria were also measured and the correlation index and the modifications per gram of abnormal solute that should be applied in order to obtain the correct urine concentration figures, were established.