Recurrent abdominal pain and headache--psychopathology, life events and family functioning.

We assessed the psychopathology of children and adolescents with Recurrent Abdominal Pain (RAP) and tension-type headaches (TTH), the psychopathology and Expressed Emotion (EE) of their mothers and family functioning. Additionally, we assessed the relationship of negative Life Events (LE) to RAP and headaches. Sixty-nine children and adolescents with either RAP or headaches, and their mothers were examined and compared to controls. Of the children with RAP or headache, 81.6% and 83.9% respectively carried a psychiatric diagnosis, primarily anxiety or depressive disorder, in contrast to 15% of controls. Mothers of patients with RAP showed more symptoms of anger and hostility than controls. Index mothers had higher EE than control mothers. More problems were reported in the families of patients with RAP. Families of patients with headache were similar to those with RAP but differed from controls in terms of behaviour control and general functioning. More negative LE were experienced by both index groups. If psychological intervention is decided for certain children with RAP or TTH, it should address their depression, anxiety, the impact of negative LE and family functioning.

Iron overload and kidney lysosomes.

Iron overload has been associated with damage of the liver and other organs of patients with primary or secondary increased iron load. In order to study the effect of iron overload on the pathophysiology of kidney lysosomes, experimentally induced iron overload models were employed. Iron overload was achieved through intraperitoneal injections of Fe-dextran (Imferon) in male rats, at different final iron concentrations (825 and 1650 mg/kg, single and double dose groups respectively). Controls were injected with dextran following a similar protocol. The animals were killed at different time points after the last injection. Subcellular fractionation studies of kidney homogenates were carried out by differential centrifugation and density gradient centrifugation. The kidney iron load was increased with both doses. Iron appeared to accumulate mainly in the lysosomes, bringing about distinct changes in the behaviour of the organelles as judged by subcellular fractionation studies. Lysosomes became more fragile and showed increased density. The extent of the above changes seemed to correlate with the extent and duration of iron accumulation and could be reversed when the iron load was reduced.

In vivo effect of sodium valproate on mouse liver.

The in vivo effect of sodium valproate (SV) on the activity of uridine diphosphate glucuronosyltransferase (UDP-GT) and hepatotoxicity in the mouse liver was studied. Mice were injected intraperitoneally (IP) with SV at doses varying from 50 to 800 mg/kg per day, for six consecutive days (dose-response group) or at a standard dose of 300 mg/g per day for 2-10 days (time-response group), whereas the controls were injected with normal saline. Valproic acid levels had a positive correlation to the dose (P < 0.001) and duration of drug administration (P = 0.006). A gradual increase in UDP-GT activity was observed in doses of up to approximately 400 mg/kg per day, whereas in higher doses the enzyme activity gradually decreased. The time course of UDP-GT activity at the standard dose of 300 mg/kg per day increased progressively, with a maximum up to the sixth day and then had a gradual reduction. Hepatic necrosis (which was unrelated to the dose or the duration of drug administration) was found in 13% of the SV-treated animals and in none of the controls. We conclude that at an optimal dose (300-400 mg/kg per day) and at a time course of 6 days, SV causes liver UDP-GT induction, whereas in higher doses and longer duration of administration, UDP-GT activity is gradually reduced. SV also causes hepatotoxicity unrelated to dose and time course.

Primary malignant melanoma of the conjunctiva of the upper eyelid. A case report.

BACKGROUND: Malignant melanoma of the conjunctiva is rare. The nomenclature and clinical and pathologic features of cutaneous and conjunctival melanomas are different. CASE: A 62-year-old male presented with a history of slight bleeding of the upper conjunctiva for the previous six months. On clinical examination the ophthalmologist observed a smooth, partly nodular, pigmented lesion on the conjunctiva under the left eyelid, 1.5 cm in diameter. Fine needle aspiration (FNA) biopsy of the mass showed tumor cells dispersed as single cells with eccentric, round nuclei; coarsely granular chromatin; prominent nucleoli; and dense cytoplasm with occasional brownish pigmentation as well as small aggregates of spindle-shaped neoplastic cells with hyperchromatic nuclei and no cytoplasmic pigment. CONCLUSION: FNA cytology is a simple and efficient method of making the diagnosis of malignant melanoma in conjunctival masses. Careful correlation with the clinical history and histologic findings is often necessary for confirmation of the diagnosis.

Action of intermediate doses of methotrexate on dihydrofolate reductase in malignant diseases.

This report describes the effect of intermediate methotrexate (MTX) doses on dihydrofolate reductase (DHFR) activity in vivo in the leukocytes of 16 children with malignant diseases. The authors used a cytochemical technique, and the enzyme was studied in intact cells. The treatment protocols included MTX 500 mg-2 g/m2 weekly with leucovorin rescue. The above doses of MTX partially inhibit DHFR. The reduction of enzyme activity was observed in leukocytes within 24 h after MTX infusion, and it was more obvious in the polymorphonucleas and the monocytes. Complete inhibition of enzyme activity was not observed. These results do not agree with those of previous reports using biochemical techniques, which showed that small amounts of MTX inhibit DHFR activity. Even the large doses of MTX used in this study do not completely inhibit enzyme activity. It would be worthwhile to test the effect of even larger doses of MTX to find out if DHFR activity is inhibited.

Familial hypobetalipoproteinaemia.

Familial hypobetalipoproteinaemia is a rare condition and is usually asymptomatic in heterozygotes. We report a case of hypobetalipoproteinaemia in an 8-month-old boy presenting with diarrhoea and failure to thrive since birth, who has been successfully treated with dietary restriction and large oral doses of vitamin E. This disease is one of the causes of chronic diarrhoea in childhood and its early treatment probably prevents irreversible retinal and nervous system lesions in later life.

Multilocular cyst of the kidney.

The case of a 17-month-old girl with a multilocular renal cyst is presented. Clinically and radiologically the cyst simulated a Wilm's tumor and was treated by nephrectomy. Simple excision may be equally effective in a few cases provided a correct preoperative diagnosis of the nature and extent of the lesion can be made.

Granulosa-theca-cell tumour in a 17-month-old girl.

A granulosa-theca-cell tumour in a 17-month-old girl with isosexual precocity and hirsutism is described. Following removal of the tumour, pubertal signs and hirsutism gradually subsided. Serum alpha-fetoprotein was very high (1800 ng/ml) before, but not any longer detectable after the removal of the tumour and on repeated measurements for the subsequent 34 months, at which time the clinical condition of the child has been very good.

Fryns syndrome without deletion 16q.

Fryns and his colleagues described two children with a remarkably similar phenotype, and suggested their condition was due to a partial or total deletion of the long arm of chromosome 16 distal to band q21. We report the same phenotype in a boy whose blood karyotype was normal.

Bilateral nephroblastoma in a horseshoe kidney.

A case of bilateral nephroblastoma in a horseshoe kidney is presented. It is the second to be reported in the international literature.

Decreased fidelity of DNA polymerase activity isolated from aging human fibroblasts.

DNA polymerase (deoxynucleosidetriphosphate: DNA nucleotidyltransferase, EC 2.7.7.7 or DNA nucleotidyltransferase) activity, isolated from late and early passage cells of the diploid human fibroblast line, MRC-5, was compared. The level of activity dropped with increasing passage. In addition, when the fidelity of polymerization was monitored with four synthetic templates under a variety of conditions, it was observed that the enzyme from late passage cells was more error-prone. The possible relation of these observations to "senescence" of the fibroblasts is discussed.

A DNA polymerase from Ustilago maydis. 1. Purification and properties of the polymerase activity.

A DNA polymerase from Ustilago maydis has been purified to apparent homogeneity. The native enzyme possesses a subunit structure consisting of 50000 and 55000-dalton monomers. The apparent sedimentation coefficient of the polymerase activity in the absence of salt is 8.4 S (Mr=180000-200000), that in its presence (0.6 M NaCl or 0.12 M KCl) being 6.3 S (Mr=80000-100000). Low concentrations of EDTA also converted the 8.4-S to a 6.3-S form, whereas magnesium ions catalysed the reverse association. The enzyme has an absolute requirement for both a DNA or RNA template and a DNA primer. For homopolymer templates the primer requirement was satisified by a short complementary oligodeoxynucleotide, but oligoribonucleotides were extremely inefficient primers. With the template-primer poly(dA) X (dT)12, the enzyme added an average of 50 dTMP nucleotides on to each primer molecule, whereas with poly(rA) X (dT)12, this figure was 300. The enzyme also possesses an associated deoxyribonuclease activity. No other DNA polymerase activity was detected in cell-free extracts of U. maydis.