Sclerosing of recurrent lymphangioma using OK-432.

The authors present two cases of lymphangioma of the cervicofacial region, treated with a new investigational drug in North America, OK-432 (picibanil), a sclerosing agent intralesionally injected. Both patients had been treated surgically and had recurrence of the tumor. Intralesional injection of OK-432 without aspiration was employed for the first patient, and after aspiration in the second patient.

Maximum and average urine flow rates in normal children--the Miskolc nomograms.

Objective: To establish normal reference ranges of urinary flow rates in children.

Subjects And Methods: From a screening programme, 200 healthy children (96 girls and 104 boys, between 3 and 18 years old) with no renal, urological, psychological or neurological disorders were selected, and they provided 433 micturitions for analysis. The children were divided into those with body surface areas of < 0.

A further report of Brachmann-de Lange syndrome in two sibs with normal parents.

We report on a family in which a girl and a boy in the same sibship show variable manifestations of a less severe type of Brachmann-de Lange syndrome without significant prenatal growth deficiency and reduction deformities of the forearms. Both parents are healthy and phenotypically normal, and no other family members are affected. All the affected sibs except one described so far with normal parents presented the severe type of Brachmann-de Lange syndrome (now sometimes classified as type I: "classic" or "full" Brachmann-de Lange syndrome), with major upper limb anomalies, severe growth and mental retardation and, frequently, early death.

Development conditions and principles for identification of cow's milk protein hypersensitivity in infants.

The authors studied a group of 153 infants presenting pneumonia and/or bronchitis suspected to be immunized to cow's milk proteins (cow's milk allergy). The diagnosis was confirmed in 96 children (62.7%), which is 20.

Blood pressure measurement using oscillometric finger cuffs in children and young adults. A comparison with arm cuffs during general anaesthesia.

Arterial blood pressure measurements (y) obtained from forefinger cuffs were compared with standard arm cuff readings (x) in 41 anaesthetised children and young adults. Mean (SD) differences between cuff measurements were -0.21 (9.

Speech perception training can facilitate sound production learning.

This study examined the role of speech perception training in the correction of phonological errors. Twenty-seven preschoolers with phonological impairment who misarticulated /integral of/ were randomly assigned to one of three groups: Group 1 children listened to a variety of correctly and incorrectly produced versions of the word "shoe"; Group 2 children listened to the words "shoe" and "moo"; Group 3 children listened to the words "cat" and "Pete." A computer game was used to provide reinforcement for correct identification of the words.

Serum pseudocholinesterase and very-low-density lipoprotein metabolism.

Serum pseudocholinesterase (PChE) was discovered in 1932. Since this protein mimics many of the catalytic properties of acetylcholinesterase, it has traditionally been referred to as PChE, even though its true biological function is unknown. Serum PChE is synthesized in the liver and secreted into the circulation as a sialated glycoprotein.

Aminopterin Syndrome Sine Aminopterin (ASSA) syndrome in two siblings: further delineation of the syndrome and review of the literature.

In this report we describe two sibs with an unusual and complex pattern of severe malformations resembling Aminopterin Syndrome Sine Aminopterin (ASSA) syndrome, the phenotypic features of which are similar to those of aminopterin embryopathy. Six nonfamilial cases of ASSAS have been published in the literature to date. Our family with two affected sibs of different sexes supports the previous suggestion of autosomal recessive inheritance.

Idiopathic acute portal vein thrombosis: a case report.

There are few reported cases of acute portal vein thrombosis presenting as an acute abdomen in adolescent age group. Most published series concern chronic extrahepatic portal vein thrombosis. Acute portal vein thrombosis is rare, but can develop into serious complications.

The impact of spina bifida on the medical services of Newfoundland and Labrador.

This institution receives all live spina bifida patients from Newfoundland and Labrador. The aim of this study is to determine the outcome of these patients and the impact on the medical system. On a retrospective chart review, 274 patients born between 1967 and 1990 were studied.

Nonrenal cystic masses in neonates and children.

With the increasing use of ultrasonography (US) in children, abdominal cystic masses, many of them nonrenal, are being detected more frequently both before and after birth. Between 1986 and 1991, 20 predominantly cystic abdominal or pelvic masses of nonrenal origin were detected by US in patients less than 10 years of age. Ten of the 20 patients were neonates; in 5 of these the masses were detected antenatally.

Miller postaxial acrofacial dysostosis. The phenotypic changes with age.

We report data on the clinical and psychological follow-up of a boy with Miller postaxial acrofacial dysostosis syndrome between the ages of 1 and 7 years. After an initial psychomotor retardation intellectual and social function improved significantly after the use of hearing aids (50-70 db bilateral hearing loss). The facial appearance changed with age: the face became asymmetric and triangular with more pronounced ectropion and fine, thin lips.

Subendocardial abscess as a complication of prolonged central venous access for parenteral nutrition.

Patients with chronic gastrointestinal diseases may require long-term parenteral nutrition. The authors describe a case in which a subendocardial abscess developed in the right atrium in association with staphylococcal septicemia. The patient, a 15-year-old boy, had a malpositioned Silastic catheter, the tip of which was in his right atrium.

Growth rate in children with vitamin-D-dependent rickets in relation to 1-alpha-hydroxyvitamin D3 dosage.

Growth rate of five children with vitamin D-dependent rickets was analyzed during the long-term treatment with an active analog of vitamin D3. Considerable increase in growth rate together with the improvement of biochemical values and radiological pattern took place during the initial phase of administration of 1-hydroxyvitamin D3. During the maintenance treatment of long duration with 1-hydroxyvitamin D3 both the acceleration of growth and catch-up growth persisted.

Palliative home care program for terminally ill children.

A study conducted for the Dr. Charles A. Janeway Child Health Centre, St.

Procollagen type III peptide in the assessment of liver fibrosis in children with congenital alpha-1-antitrypsin deficiency.

In 31 children with congenital alpha 1-antitrypsin deficiency the concentration of procollagen type III peptide was determined in a trial establishing liver fibrosis degree and monitoring of fibrosis progression. No correlation was found between the degree liver fibrosis determined by histological examination and the serum concentration of procollagen type III peptide. The concentration of procollagen type III peptide was higher with coexistent cholestasis and in case of inflammatory processes outside the liver (pneumonia).

Evaluation of the liver blood flow in the children with portal hypertension.

The aim of the study was to evaluate the liver blood flow at different stages of hepatitis B as compared with prehepatic block. The examination was performed on 79 children aged 1-17 years who were divided in two groups. Group 1 consisted of 20 children with HBV chronic active hepatitis B: 10 children with liver efficiency (Gr.

A case of organic aciduria--suspected 3-hydroxy-3-methylglutaric aciduria.

In a girl from a family with muscular hypotonia, hypoglycaemia, lactic acidosis and delayed development the analysis of organic acids in urine suggested a defect in leucine metabolism--3-hydroxy-3-methylglutaric aciduria. A good therapeutic effect was obtained with low-protein diet.