Status of selenium and antioxidant enzymes of goitrous children is lower than healthy controls and nongoitrous children with high iodine deficiency.

In order to investigate the relations of iodine deficiency and/or goiter with selenium (Se) and antioxidant enzyme (AOE) status, we determined the relevant parameters of goitrous high school children living in an endemic goiter area of Turkey. Subjects were selected by a simple random sampling technique after screening the whole population of the high schools of two towns by neck palpation. The results of the goitrous group (n = 48, aged 15-18 yr) were compared with those of nongoitrous control children (n = 49) from the same populations, and with an outside control group (n = 24) from a lower-goiter-prevalence area. The overall prevalence of goiter was 39.6% in the high school population of the area. Activities of erythrocyte AOE (glutathion peroxidase, catalase, and superoxide dismutase) and concentrations of plasma and erythrocyte Se and urinary iodine were found to be significantly lower in goitrous children than both in-region and out-region of the control groups. When the whole study group was reclassified according to the severity of iodine deficiency, it was found that the AOE and Se status of those control children without goiter but with high iodine deficiency was significantly higher than goitrous children, although they did not differ from nondeficient control group. This might be the result of the possibility that goitrous children are exposed of oxidative stress, which may introduce alterations to the antioxidant defense system and/or the antioxidant status is relatively lower in goitrous children than those children who are highly iodine-deficient but did not develop goiter. The results of this study seem to support the view that the risk of goiter development may be higher in highly iodine-deficient children with lower enzymatic antioxidant and Se status.

Successful treatment of visceral leishmaniasis with allopurinol plus ketoconazole in an infant who developed pancreatitis caused by meglumine antimoniate.

Pentavalent antimony is still the drug of choice in the treatment of visceral leishmaniasis. Pantavalent antimony can cause a wide range of adverse effects, the most serious of which are cardiotoxicity and hepatotoxicity. Acute pancreatitis is a rarely reported adverse effect. An infant who developed pancreatitis during meglumine antimoniate treatment for visceral leishmaniasis and who was successfully treated with a combination of allopurinol and ketoconazole is reported.

A new case of Balci's syndrome (corneal opacity, microphthalmia, microcephaly, mental retardation, and generalized muscular spasticity associated with congenital heart disease).

The association of corneal opacity, microphthalmia, microcephaly, mental retardation, and generalized muscular spasticity with hyperglycinemia was presented for the first time by Balci and colleagues in 1974. After this report, some similar cases in the literature were referred to as Balci's syndrome. In this paper we describe a new case of Balci's syndrome, a 2.5-month-old female patient with corneal opacity, microphthalmia, microcephaly, mental retardation, and generalized muscular spacticity. All of these findings are acceptable as Balci's syndrome, and in addition she had congenital heart disease (ventricular septal defect) and renal anomalies. In this paper other syndromes associated with corneal opacity and mental retardation are discussed.

Parvovirus B19 infection reminiscent of myelodysplastic syndrome in three children with chronic hemolytic anemia.

The authors have seen transient pancytopenia with erythroid hypoplasia and striking trilineage myelodysplasia reminiscent of true myelodysplastic syndrome (MDS) in 3 children, 1 with thalassemia intermedia and the other 2 with previously undiagnosed hereditary spherocytosis. In these 3 children transient pancytopenia and myelodysplasia coincided with serological evidence of acute parvovirus-B19 (PV-B19) infection, strongly suggesting their relevance. It is of interest that these 3 cases were encountered within a period of 6 months. This might be an incidental event, but it might also be concluded that acute PV-B19 infection associated transient pancytopenia with morphological appearance of MDS may occur more frequently than reported in the literature. So, PV-B19-associated nonclonal MDS should be considered in the differential diagnosis of true clonal MDS.

Can zinc deficiency be used as a marker for the diagnosis of celiac disease in Turkish children with short stature?

BACKGROUND: It is generally accepted that celiac disease (CD) must always be considered when dealing with growth failure in children. Therefore, it is important to develop screening tests for detecting patients that need an intestinal biopsy. The aim of the present study was to investigate the value of plasma zinc levels for the diagnosis of monosymptomatic CD in short-statured children. METHODS: Fourty-nine children with a short stature and 34 healthy controls were investigated. Plasma zinc levels were assayed by atomic absorption spectrophotometry in short-statured children and controls. All patients with short stature underwent endoscopic small intestinal biopsy. RESULTS: Duodenal mucosal histopathology was normal in 25 children. Low plasma zinc values were observed in 54.2% of patients with CD, 32.0% of patients with idiopathic short stature and 14.8% of controls. The mean values of plasma zinc levels were not significantly different among the three groups. Sensitivity, specificity and the positive and negative predictive values for plasma zinc were 45.8, 76.0, 64.7 and 59.4%, respectively. CONCLUSIONS: These results indicate that zinc deficiency is an important problem in CD children with short stature; however, plasma zinc levels are not useful as a screening test for selecting patients for jejunal biopsy.

Nasal midazolam effects on childhood acute seizures.

Sixteen children, aged from 2 months to 14 years, with a diagnosis of acute seizures and seen at Dr. Sami Ulus Child Health and Disease Center, were included in this study. Midazolam (5 mg/mL) 0.2 mg/kg was administered intranasally in 30 seconds by an injector. The heart rate, respiratory rate, blood pressure, and oxygen saturation were recorded at 0, 5, and 10 minutes after administration. The seizures of three (18.7%) patients terminated within 1 minute, of seven (43.7%) patients in 1 to 2 minutes, and of three (18.7%) patients in 2 to 5 minutes. However, three (18.7%) patients did not respond to treatment. As a result, it was concluded that intranasal midazolam administration is easy and effective. The half-life of midazolam is shorter than diazepam, and midazolam has fewer complications when compared with diazepam. It is easier to use in nasal drop and spray forms.

Adenosine deaminase in childhood pulmonary tuberculosis: diagnostic value in serum.

The diagnostic value of serum adenosine deaminase (ADA) activity was evaluated in childhood pulmonary tuberculosis. Serum ADA levels were measured in 20 children diagnosed with pulmonary tuberculosis (group 1) and 150 children (group 2) including 128 with tuberculosis infection (Mantoux test positive) and 22 healthy children. In group 1, the mean serum ADA activity was 74.06 +/- 18.5 U/l, which was significantly (p < 0.001) higher than that of group 2 (40.36 +/- 12.0 U/l). A serum ADA level of > or = 53.76 U/l had a sensitivity of 100 per cent, specificity of 90.7 per cent, positive predictive value of 58.8 per cent, and a negative predictive value of 100 per cent in children with tuberculosis disease. To conclude, measurement of serum ADA activity was a useful diagnostic tool in childhood pulmonary tuberculosis.

Congenital malaria: a case report.

Congenital malaria is an uncommon disease even in endemic areas. A 19-day-old female infant with congenital malaria is presented. The mother of the patient was diagnosed to have malaria at the seventh month of gestation and was treated with chloroquine orally for three days. No malarial prophylaxis was given. The infant developed fever, hyperbilirubinemia, anemia and hepatosplenomegaly postnatally. Thin blood smears revealed many Plasmodium vivax parasites. She was treated with oral chloroquine for three days. We emphasize the importance of adequate antenatal medical therapy and prophylaxis during pregnancy.

Fatal outcome of infantile oxalosis: case reports from three families and a review of literature.

Infantile oxalosis is a rare, autosomal recessive disorder. We present three unrelated cases of infantile oxalosis and their families, emphasizing its place as a cause of acute renal failure in infancy, and showing the clinical heterogeneity of the disease within the same family. The affected infants (two males, one female) were 2.5, 3.5, and five months old. Two families had first degree parental consanguinity; two revealed a history of nephrolithiasis; and one of these two had a member who received liver and kidney transplants because of primary hyperoxaluria type I. All the patients presented with the symptoms and findings of acute renal failure. Their hemoglobin levels were between 6.8-9.6 g/dl, urinalysis revealed (+) to ( +) proteinuria and microscopic hematuria. All had metabolic acidosis with BUN levels 67-113 mg/dl and creatinine 3.5-7.7 mg/dl. The abdominal ultrasonographies revealed normal sized hyperechogenic kidneys with the loss of corticomedullary junctions. Calcium oxalate crystals were demonstrated in retina and bone marrow of two patients, and in renal parenchyma of all the patients. The patients were treated with peritoneal dialysis. Renal functions continued to be abnormal (BUN: 47-168 mg/dl, creatinine: 2.8-11 mg/dl) after dialysis, and the outcome was fatal in all. In the presented families, because of the variation of the clinical presentation and the fatal outcome, presence of the multiple genetic loci appeared to be most likely. Further molecular studies will clarify the heterogeneity of this disorder.

Hodgkin's disease in 82 Turkish children diagnosed over a 10-year period: epidemiological, clinical, and histopathologic features and prognosis with prolonged chemotherapy.

In this study, 82 Turkish children with Hodgkin's disease (HD) between 1 and 14 years of age and diagnosed over a 10-year period were evaluated retrospectively. More than half of the patients (54%) presented with advanced stages of HD. Mixed cellularity (MC) was the most frequent (56.1%) histopathologic type, which was followed by nodular sclerosing (NS, 18.3%) in frequency. None of the patients received radiotherapy as initial treatment. In 67 children the COPP regimen alone and in 15 the ABVD regimen alternating with COPP were started, to be given as a total of 12 courses. In the patients who presented with stage I-II HD the overall survival (OAS) rate and 5-year event free survival (EFS) rate were 92.3% and 77.8%, respectively. In the patients with advanced disease (stage III-IV) OAS and 5-year EFS were estimated to be 89.5% and 67.4%, respectively. No serious toxicity of chemotherapy was detected during the follow-up. In this group, clinical, epidemiological and histopathologic features of the disease showed a special pattern close to the type I pattern of HD. Regarding the survival rules and occurrence of low toxicity in our patients, results of prolonged chemotherapy alone seem to be encouraging in most of the children with HD. However, the follow-up duration is not yet sufficient to declare a clear conclusion related to the late complications.

Budd-Chiari syndrome in a child secondary to membranous obstruction of the hepatic vein treated by percutaneous transluminal angioplasty. Report of a case.

Budd-Chiari syndrome (BCS) due to membranous obstruction of the hepatic vein and the inferior vena cava is rare in children. We report a child with BCS that had a membranous obstruction at the level of the hepatic veins. The web was successfully dilated percutaneously by balloon catheters. Symptoms and signs of obstruction improved without any complication. As percutaneous catheterization is an effective, safe and repatable procedure, we recommend this technique for treatment of children and adults with BCS due to membranous obstruction of the hepatic veins.

Primary hyperparathyroidism in infancy: a case report.

Primary hyperparathyroidism is a rare disease of childhood. The condition is even rarer in the neonatal and infant stages. The disease, with its main manifestation-hypercalcemia-often is fatal. The authors successfully treated a 2.5-month-old boy who had primary parathyroid hyperplasia. The patient had recurrent pneumonia and failure to thrive. Blood test results showed an abnormally high level of calcium, which was resistant to medical therapy. Further investigations showed high levels of parathyroid hormone. The patient underwent neck exploration, which showed hyperplasia of the all four parathyroid glands. Total parathyroidectomy was performed, with one gland being autotransplanted to the deltoid muscle. The patient had an immediate hypocalcemic period, followed by normocalcemia. In light of the present case and others in the Literature, the authors recommended total parathyroidectomy followed by autotransplantation of a gland to an accessible muscle.

Burkitt's lymphoma in 63 Turkish children diagnosed over a 10 year period.

Sixty-three Turkish children with Burkitt's lymphoma (BL) diagnosed over a 10-year period in a single institution were retrospectively analyzed. Burkitt's lymphoma included 41.7% of non-Hodgkin's lymphomas and 17.2% of all childhood malignant solid tumors diagnosed in our department in this duration. The patients studied with BL were aged between 3 and 14 years (mean 5.9 years), with a male of female ratio of 2:1. While the age distribution in our patients was similar to that in African BL (endemic), the predominance of abdominal involvement and the frequency of bone marrow infiltration and pleural effusion were reminiscent of American BL (sporadic). The incidence of jaw involvement (15.9%) in our group was higher than in American BL, however, and was not a high as in African BL. Most of the patients were of a lower socioeconomic status. Significant growth retardation was found in the children with BL compared with 40 age-matched children without malignancy, nor chronic or endocrinologic disorders, who were of a similar socioeconomic status. A serological study for Epstein-Barr virus (EBV) was performed in 18 children, and the IgG-type antibody to the viral capsid antigen of EBV was found to be positive in all of them. As a result, BL seems to include a considerable proportion of all childhood malignant solid tumors in Turkey. The epidemiological and clinical presentation and course indicate that BL appears in Turkish children in a form that is between the African and American types of the disease. Further molecular and chromosomal studies in Turkish children with BL are needed.

Henoch-Schonlein nephritis associated with subacute thyroiditis.

We present a 12-year-old boy who developed subacute thyroiditis during the course of rapidly progressive glomerulonephritis due to Henoch-Schonlein purpura (HSP) proven by clinical findings and percutaneous renal needle biopsy. The thyroid gland of the patient suddenly enlarged with mild tenderness while he was on steroid and dipyridamole therapy. Thyroid hormone levels revealed T3 0.31 ng/ml (nl: 0.52-1.75 ng ml), T4 2.53 ug/dl (nl: 4.8-12.8 ug/dl), free T3 0.80 pg/ml (nl: 2.14-5.34 pg/ml), free T4 0.2 ng/dl (nl: 0.73-1.95 ng/dl) and TSH 1.02 U/ml (nl: 0.36-3.25 U/ml). Antimicrosomal antibody was negative while antithyroglobulin antibody was slightly positive (1/80+). Hypoactivity with a spotty pattern was demonstrated by thyroid scanning. Serologically proven mumps infection was detected and may have been a triggering factor in the development of both HSP and subacute thyroiditis.

Screening for congenital hypothyroidism in Turkey.

UNLABELLED: A pilot study was performed to determine the incidence of congenital hypothyroidism (CH) in Turkey and to build a model for nationwide screening. From December 1991 to December 1992, 30,097 newborns were screened for CH using a primary measurement of thyroid stimulating hormone in capillary blood on days 3-5 of life. Samples were obtained in collaboration with the ongoing nationwide phenylketonuria screening programme. Eleven cases of primary CH were detected giving the incidence of 1:2736. Recall rate was 2.3%. Replacement therapy with L-thyroxine was started after the confirmation of diagnosis. The median age at the initiation of replacement therapy was 23 days (range 7-35 days). CONCLUSION: The incidence of CH is notably higher in Turkey than reported in most other countries. Iodine deficiency and/or dyshormonogenesis might contribute to this high incidence. This result emphasizes the necessity of a nationwide screening programme.