Hepatitis B complicated focal segmental glomerulosclerosis.

After the initial report of membranous glomerulopathy due to hepatitis B virus infection by Combes et al, other glomerular diseases - but rarely focal segmental glomerulosclerosis (FSGS) association with HBV infection - have been reported. Herein we present an 8-year-old boy with chronic HBV infection complicated FSGS. The patient was initially regarded as idiopathic FSGS and started on an immunosuppressive schedule. The elevation of liver transaminases in the course of the therapy revealed the immunotolerated perinatal HBV infection. It was considered that immunosuppressive agents have induced viral replication. The treatment was changed to lamivudine alone. The nephrotic syndrome has already been improved with the seroconversion of anti-HBeAg and reduced liver functional tests by the tenth month of the treatment. This case is peculiar for the seldom association of FSGS with chronic HBV infection and treatment modality particularly for the countries where this viral infection is endemic.

Nutritional effects on auditory brainstem maturation in healthy term infants.

OBJECTIVE: To assess the effects of dietary long chain polyunsaturated fatty acid (LCPUFA) supplementation on auditory brainstem maturation of healthy term newborns during the first 16 weeks of life by measuring brainstem auditory evoked potentials (BAEPs). DESIGN: Throughout the 16 week study period, infants in the formula A group (n = 28) were assigned to be fed exclusively with the same formula supplemented with DHA, and infants in the formula B group (n = 26) were assigned to receive only a DHA unsupplemented but otherwise similar formula. During the study period, the first 26 consecutive infants to be fed exclusively on their mother's milk for at least the first 16 weeks of life were chosen as the control group. BAEP measurements were performed twice: at the first and 16th week of age. RESULTS: There were no significant differences among the study and control groups in the BAEP measurements performed at the study entry. At 16 weeks of age, all absolute wave and interpeak latencies in the study and control groups had significantly decreased. The decreases were significantly greater in the formula A and control groups than in the formula B group. CONCLUSIONS: Infants fed on human milk or a formula supplemented with LCPUFAs during the first 16 weeks of life show more rapid BAEP maturation than infants fed on a standard formula. Although the clinical importance and long term effects of these findings remain to be determined, routine supplementation of formulas with LCPUFAs should be considered.

Adams-Oliver syndrome: further evidence for autosomal recessive inheritance.

We report a 7-year-old girl with Adams-Oliver syndrome who presented with extremely rare central nervous system anomalies including microcephaly, epilepsy, mental retardation and intracranial calcifications in addition to the classical scalp and limb defects.

The effect of iron supplementation on visual-evoked potentials in infants with iron-deficiency anemia.

Flash visual-evoked potentials were studied in 20 infants with iron-deficiency anemia to determine the effect of iron deficiency on visual function by using visual-evoked potentials in this type of anemia. After iron therapy for 12 weeks, visual-evoked potentials were retested in these otherwise healthy infants. All infants showed an excellent hematological response to iron therapy. Post-treatment visual-evoked potential N2 latencies (negative deflections) decreased significantly compared to the pre-treatment values (p < 0.05). These results suggest that iron-deficiency anemia causes subclinical visual impairment, and visual-evoked potentials may be a useful non-invasive means of detecting subtle effects of nutritional deficiencies and monitoring the nutritional status of infants.

Brainstem auditory-evoked potentials in iron-deficiency anemia.

Slight-to-moderate impairments may be observed in mental and motor developments of infants with iron- deficiency anemia. Brainstem auditory-evoked potentials provide a noninvasive means of examining the auditory aspect of the central nervous system functions. In this study the effect of iron-deficiency anemia on auditory functions was investigated by using brainstem auditory-evoked potentials. Brainstem auditory-evoked potentials of the 20 iron-deficient infants were not significantly different from those of the control group that included 20 healthy age-matched infants. Furthermore, there was not a statistically significant difference between the brainstem auditory-evoked potentials of the study group performed before and 3 months after oral iron therapy. Although we could not demonstrate a hearing loss in infants with moderate iron-deficiency anemia in this study, the relationship between severe iron-deficiency anemia and hearing loss or auditory dysfunction remains to be determined.

Fiberoptic phototherapy versus conventional daylight phototherapy for hyperbilirubinemia of term newborns.

The efficacy and wavelengths of fiberoptic phototherapy and conventional daylight phototherapy were compared in a relatively larger series of term newborns with nonhemolytic and significant hyperbilirubinemia than reported in previous studies. One hundred and nine term newborns were randomly assigned to receive either fiberoptic phototherapy on a fiberoptic phototherapy pad or overhead conventional phototherapy consisting of five daylight fluorescent lamps. Although the average spectral irradiance measured during the study period was significantly greater in the fiberoptic phototherapy group (9.2+/-1.2 microW/cm2/nm vs 7.1+/-1.1 microW/cm2/mm, p < 0.05), conventional phototherapy was significantly more effective in decreasing bilirubin levels: the duration of exposure to phototherapy was significantly shorter (49.4+/-14.4 hours vs 61+/-13.1 hours, p < 0.05), and overall bilirubin decline rate as mg/dl/h and percent/h was significantly greater in the conventional phototherapy group (0.15+/-0.06 mg/dl/h vs 0.11+/-0.05 mg/dl/h, and 0.81+/-0.34 percent/h vs 0.60+/-0.28 percent/h, p < 0.05). There were four failures of phototherapy in the fiberoptic phototherapy group whereas no phototherapy failure was observed in the conventional phototherapy group (p < 0.05). The emission spectrum of the daylight fluorescent lamp revealed a broad emission between the violet and red spectra with tiny narrow peak emission bands in 405 nm, 436 nm, 546 nm and 577 nm, while a broad emission through the blue and green wavelengths (mainly in the green spectrum) without any peak emissions was detected in the tungsten-halogen lamp of the fiberoptic phototherapy system. Conventional phototherapy with daylight fluorescent lamps should be preferred to fiberoptic phototherapy administered with fiberoptic phototherapy and in the treatment of term newborns with nonhemolytic hyperbilirubinemia.

Efficacy and safety of rectal thiopental sedation in outpatient echocardiographic examination of children.

UNLABELLED: The efficacy and safety of rectal thiopental administration in sedation for paediatric echocardiographic examination were prospectively investigated in infants with known or suspected congenital heart disease in an outpatient manner. A total of 1150 patients (546F, 604M) were studied; 264 were 7 d to 6 mo old (group I), 572 were 6 mo to 2-y-old (group II), and 314 were 2 to 6-y-old (group III). Thiopental sodium dissolved in 10 ml of water in a syringe to which a 6-F feeding catheter was attached was administered prior to echocardiographic examination to patients in groups I, II and III with doses of 50, 35 and 25 mg/kg, respectively in an emergency care environment. Length of time to achieve sedation (induction time), duration of sedation, length of time to return to normal activity (recovery time), whether sedation was successful and side effects were recorded. In the overall study population, sedation was successful in 1094 (95.1%) of the patients, the induction time was 16.34 +/- 3.69 min, the duration of sedation was 35.07 +/- 7.04 min, the recovery time was 63.25 +/- 10.17 min and the overall side-effect prevalence was 2%. Sedation was significantly more successful, the induction time was significantly shorter, the recovery time was significantly longer and side effects significantly more prominent in groups I and II compared to group III. CONCLUSION: Rectally administered thiopental is a safe and efficacious agent for sedation of infants and young children with known or suspected congenital heart disease who are undergoing echocardiography in an outpatient cardiology clinic, provided that it is used in an emergency care setting considering the risk of respiratory depression even though the prevalence of this side effect is significantly low.

Thoracic ectopic kidney in a child: a case report.

Congenital thoracic ectopic kidney is a very rare developmental anomaly and the rarest form of all ectopic kidneys. It is usually asymptomatic and discovered incidentally on a routine chest radiography. We report a thoracic ectopic kidney in a 19-month-old boy, which initially presented as a well demarcated mass at the base of the right lung on chest x-ray. Intravenous pyelography (IVP) and thoraco-abdominal computed tomography (CT) demonstrated a normal functioning transdiaphragmatic thoracic ectopic right kidney, but technetium-99m DTPA and DMSA scintigraphy demonstrated pelvic stasis. We hereby discuss the features of congenital thoracic ectopic kidney and review the literature. Although it is extremely rare, thoracic ectopic kidney should be considered in differential diagnosis of a mass with a well demarcated superior margin in the lower part of the thorax, and renal scintigraphy must be performed even if CT and IVP results are normal.

The value of first-day bilirubin measurement in predicting the development of significant hyperbilirubinemia in healthy term newborns.

OBJECTIVE: The recognition, follow-up, and early treatment of neonatal jaundice has become more difficult, since the earlier discharge of newborns from hospitals has become common practice. This prospective study was undertaken to identify the newborns at risk for developing significant hyperbilirubinemia later during the first days of life by measuring the serum bilirubin levels of the first 5 days of life to determine the critical predictive serum bilirubin value on the first day of life. METHODOLOGY: A total of 498 healthy term newborns were followed with daily serum total bilirubin measurements for the first 5 days of life, and cases with serum bilirubin levels of >/=17 mg/dL after 24 hours of life were defined to have significant hyperbilirubinemia. RESULTS: No newborns had a serum total bilirubin level of >/=17 mg/dL in the first 72 hours of life. Sixty of 498 cases (12.05%) had significant hyperbilirubinemia after 72 hours of life, and these cases had significantly higher bilirubin levels than those who did not develop significant hyperbilirubinemia on each of the first 5 days' measurements. Of the 206 newborns who had a serum bilirubin level of >/=6 mg/dL in the first 24 hours, 54 (26.21%) developed significant hyperbilirubinemia, whereas only 6 of the 292 newborns (2.05%) who had a serum bilirubin level of <6 mg/dL on the first day developed significant hyperbilirubinemia. A mean serum bilirubin level of >/=6 mg/dL on the first day had the highest sensitivity (90%). At this critical serum bilirubin value, the negative predictive value was very high (97.9%) and the positive predictive value was fairly low (26.2%). Furthermore, because no cases with a serum bilirubin level of <6 mg/dL in the first 24 hours of life required a subsequent phototherapy treatment and because all of those infants requiring a phototherapy treatment with serum bilirubin levels of >/=20 mg/dL were just among the cases whose first-day bilirubin levels were >/=6 mg/dL, the critical bilirubin level of 6 mg/dL on the first day made it possible, with the highest (100%) sensitivity and negative predictive value, to definitely predict all of the infants who would have a bilirubin level of >20 mg/dL, requiring a phototherapy treatment later during the first days of life. CONCLUSIONS: A serum bilirubin measurement and the use of the critical bilirubin level of 6 mg/dL in the first 24 hours of life will predict nearly all of the term newborns who will have significant hyperbilirubinemia and will determine all those who will require a phototherapy treatment later during the first days of life.

Is a standard protocol necessary for oscillometric blood pressure measurement in term newborns?

OBJECTIVE: The necessity of taking only one randomized blood pressure measurement or averaging three repeated measurements and, the effects of various stages of the restful state and body position on blood pressure measurements obtained with the oscillometric technique were investigated in 138 healthy term newborns. METHODS: The Athena oscillometer was used to measure blood pressure. Three successive measurements with a 5-min interval were made in each of two positions, prone and supine, in random order 30 min after the last feeding if newborns were in very quiet or quiet sleep. During routine recording of vital signs, another (single) measurement was obtained before feeding the infant regardless of the body position of the newborn, provided that they were not struggling, crying or moving. RESULTS: For all systolic, diastolic and mean blood pressures measured, there were no significant differences among either prone, supine and single measurements or among three successive measurements in each position. CONCLUSIONS: We conclude that, in the routine care of term newborns, blood pressure measurements with the oscillometric technique may be made without the need of a special position or sleep state, provided that the measurements are made with an appropriate sized cuff in the absence of struggling, crying and movement of the newborn. Taking only one randomized measurement under these conditions would be enough and practical in daily newborn care practice instead of repeating and averaging many measurements.

Double versus single phototherapy in term newborns with significant hyperbilirubinemia.

The efficacy of double phototherapy, in the form of conventional phototherapy with special blue light plus fiberoptic phototherapy, was compared with conventional phototherapy consisting of special blue lamps alone in a relatively larger series of term newborns with significant hyperbilirubinemia. During the study period the sum of the average spectral irradiances in the double phototherapy group was significantly higher than that of the single phototherapy group (p < 0.05). Phototherapy was effective in decreasing bilirubin levels in both groups, but the response was greater in the double phototherapy group; the duration of exposure to phototherapy was significantly shorter (31.2 +/- 8.5 vs. 38.98 +/- 14.7 h, p < 0.05), and the overall bilirubin decline rate as mumol/l/h and per cent/h was significantly greater in the double phototherapy group (4.1 +/- 1.37 vs. 3.3 +/- 0.86 mumol/l/h, and 1.29 +/- 0.38 vs. 1.02 +/- 0.44 per cent/h, p < 0.05). In phototherapy treatment of term newborns with significant hyperbilirubinemia, double phototherapy provided more rapid and effective bilirubin reduction than conventional phototherapy alone due to higher spectral irradiance and larger body surface area exposed to phototherapy. The value of double phototherapy in the treatment of newborns with hemolytic hyperbilirubinemia remains to be determined.

Comparison of the efficacy of conventional special blue light phototherapy and fiberoptic phototherapy in the management of neonatal hyperbilirubinaemia.

The efficacy and usefulness of two types of phototherapy differing in the source, wavelength and irradiance of the light, conventional phototherapy consisting of special blue light and fiberoptic phototherapy, were compared in a relatively larger series of term newborns with non-haemolytic and more significant hyperbilirubinaemia than those in previous studies. In total, 108 newborns were allocated sequentially to receive either conventional phototherapy consisting of five special blue lamps or fiberoptic phototherapy. The average spectral irradiance measured at the skin surface level of newborns during the study period was significantly greater in the conventional phototherapy group. The special blue lamp of the conventional phototherapy unit had an emission spectrum almost identical to the bilirubin absorption spectrum, whereas the tungsten-halogen lamp of the fiberoptic phototherapy had a broad emission through the blue and green wavelengths (mainly in the green spectrum). Phototherapy was more effective in the conventional phototherapy group; the duration of exposure to phototherapy (h) was significantly shorter, and the overall bilirubin decline rate (as micromol/l/h and %/h) was significantly greater in the conventional phototherapy group. According to the nursing personnel, fiberoptic phototherapy was more comfortable than the conventional phototherapy frame because of the easier accessibility and handling of the infants during phototherapy. They complained of giddiness, nausea, glare, temporary blurring of vision and difficulty in detecting the skin colour changes of newborns with the blue light of the conventional phototherapy unit. Conventional phototherapy consisting of special blue fluorescent lamps with approximately twofold higher irradiance and an emission spectrum almost identical to the bilirubin absorption spectrum is preferable to fiberoptic phototherapy in the standard treatment of term newborns with non-haemolytic hyperbilirubinaemia.

High-dose intravenous immunoglobulin therapy in neonatal immune haemolytic jaundice.

A controlled study was conducted to assess the role of high-dose i.v. immunoglobulin (HDIVIG) therapy in neonatal immune haemolytic jaundice. Patients with ABO and/or Rh incompatibilities proved by significant hyperbilirubinaemia (>204 mmol l(-1)), positive direct antiglobulin test and high reticulocyte count (> or =10%) were randomly assigned to receive either conventional phototherapy alone or phototherapy with high-dose i.v. immunoglobulin (1 g kg(-1), over 4 h) as soon as the diagnosis was established. Exchange transfusions were performed if serum bilirubin concentrations exceeded 290 mmol l(-1) and increased by more than 17 mmol l(-1) per h despite both treatment manoeuvres. Eight of 58 patients in the HDIVIG group required exchange transfusions, whereas it became necessary in 22 of 58 patients in the control group (p<0.001). The durations of phototherapy and hospitalization in terms of hours were significantly shorter in the HDIVIG group (p<0.05). No side effects of HDIVIG therapy were observed. In conclusion, HDIVIG therapy in newborns with ABO or Rh haemolytic diseases reduces haemolysis, serum bilirubin levels and the need for blood exchange transfusion, a procedure which has potential complications and carries a risk of mortality.

Auditory brainstem responses in children with congenital heart disease.

BACKGROUND: Cyanotic congenital heart diseases usually lead to growth and developmental delay in children due to chronic hypoxemia and undernourishment that may affect the central nervous system. The auditory brainstem responses are determined to assess the maturation and function of the brainstem. Therefore, we used the auditory brainstem responses to investigate the effect of cyanotic congenital heart diseases on brainstem maturation. METHODS: The auditory brainstem responses were investigated in 45 children (23 cyanotic, 22 acyanotic) with congenital heart diseases and compared with the results of 30 healthy counterparts (all children were aged between 2 months and 15 years). RESULTS: The results of auditory brainstem responses were similar in acyanotic patients and in normal children. The cyanotic patients under 1 year of age had more prolonged I-V interpeak latencies than those of control and acyanotic patients (P < 0.05). There was no difference between all groups older than 1 year of age. In cyanotic children, I-V interpeak latencies showed significant negative correlation with arterial oxygen saturation and partial oxygen pressure (P < 0.05). CONCLUSIONS: Cyanotic congenital heart diseases may cause significant retardation on brainstem maturation due to chronic hypoxemia, especially in infants under 1 year of age, whereas acyanotic congenital heart diseases have no effect on auditory brainstem responses.

Detection of early anthracycline-induced cardiotoxicity in childhood cancer with dobutamine stress echocardiography.

Asymptomatic long-term survivors of childhood cancer treated with anthracyclines may have latent cardiac dysfunction which is undetected by commonly used echocardiographic methods. A more sensitive echocardiographic screening test, dobutamine stress echocardiography, was performed on 22 patients (mean age 9.10 +/- 3.79 years) treated with 75 to 450 mg/m2 of anthracyclines (mean 210.45 +/- 127.34) and results were compared with 22 healthy age-matched control subjects. Echocardiographic Doppler studies were performed after each dobutamine infusion of 0.5, 2.5, 5 and 10 micrograms/kg/min. Although left ventricular mass was decreased and end-systolic walls stress increased in the patient group when compared with the control subjects (p < 0.01 and p < 0.05, respectively), no differences were found between shortening fraction and ejection force in control subjects and patients, at rest and during each dobutamine infusion. A decreased mitral E/A ratio (ratio of early-to-late peak filling velocity) was demonstrated in anthracycline-treated patients only during dobutamine infusion (p < 0.01). Our data showed left ventricular diastolic dysfunction during intropic stimulation with dobutamine, and suggest that dobutamine stress echocardiography is a useful technique for evaluating the cardiac status of anthracycline-treated patients on a long-term basis.

Evaluation of bone mineral density in children receiving antiepileptic drugs.

The effects of the valproic acid and carbamazepine monotherapies on bone mineral density were evaluated. Bone mineral density was measured in 53 children with primary epilepsy taking either valproic acid (n = 25) or carbamazepine (n = 28) for longer than 1 year and in a healthy control group (n = 26) by the dual-energy x-ray absorptiometry method at L2-L4 levels of lumbar vertebrae. The mean serum levels of valproic acid and carbamazepine were 66 +/- 2.2 microg/mL and 7.0 +/- 9.3 microg/mL, respectively, and the mean duration of treatment for each drug was 2.4 +/- 0.2 years and 2.6 +/- 0.5 years, respectively. Calcium intakes in diet were similar in both the control and study groups. The serum levels of calcium and phosphorus in all groups were normal. Bone mineral density values of both valproic acid and carbamazepine groups were not statistically different from that of the control group (P > 0.05).

Measurement of bone mineral density by dual energy X-ray absorptiometry in preterm infants fed human milk or formula.

UNLABELLED: Bone mineralization of healthy preterm infants fed human milk were compared with that of similar fed preterm formula. Bone mineralization was studied by dual energy X-ray absorptiometry in 43 preterm infants divided into two groups; 21 preterm infants were fed with maternal breast milk and 22 preterm infants with a preterm formula containing 70 mg calcium and 35 mg phosphorus per decilitre. CONCLUSION: Preterm infants fed breast milk had lower bone mineral density than the preterm formula-fed group. Fortifying preterm human milk with calcium and phosphorus will improve bone mineralization in preterm infants.