Hepatic artery resistance in children with obesity and fatty liver.

OBJECTIVE: The present study assessed whether there is a correlation between hepatic artery resistive index (HARI) and increase of body mass index and hepatosteatosis grading in children with non-alcoholic fatty liver disease (NAFLD) reflecting hemodynamic effects of hepatosteatosis. METHODS: Thirty three healthy children [body mass index(BMI): mean+/- standart deviation(SD), min-max: 20.1+/-1.14(18.5-23.7), 33 overweight [BMI:25.1+/-2.2 (18.5-23.7)] and 66 obese [BMI:31.1+/-2(25.6-40)] adolescents were enrolled into the study. To search the relation of HARI with fatty liver degree, study subjects subdivided into groups according to their degree of fatty liver at ultrasonography(US). RESULTS: Increase of HARI was correlated with increase in BMI (p<0.0001, r=0.533). Increase of HARI was well correlated with increase in degree of fatty liver (p<0.0001, r=0.630). CONCLUSION: The present study results suggest that there are positive correlations of HARI with BMI and hepatosteatosis grade in obese children with NAFLD. HARI may be a candidate parameter to determine early alarming hemodynamic changes in hepatic tissue of obese children with fatty liver before development of severe stages NAFLD.

Delayed gastric emptying in children with poor appetite.

BACKGROUND: In this study we aimed to evaluate gastric emptying time in children with poor appetite. METHODS: Anthropometrical measurements, daily energy intakes and gastric emptying times were determined in 36 children with poor appetite. The children were grouped as those with normal and delayed gastric emptying. These groups were then compared with respect to age and nutritional status. RESULTS: Malnutrition was found in 63.9% and gastric emptying was delayed in 58.3% of all cases. Children with delayed gastric emptying were significantly older and malnutrition was significantly higher in this group (7.2 +/- 3.1 vs 3.9 +/- 1.5 years, P: 0.001 and 81.0% vs 40.0%, P: 0.017, respectively). Daily mean energy intake was significantly lower than daily mean energy requirement in children with delayed and normal gastric emptying (P = 0.002 and 0.026 respectively). CONCLUSION: In children with poor appetite, probability of gastric motility disorders should be taken into consideration. Especially in children with failure to thrive at preschool and early school years gastric motility studies should be undertaken.

Prokinetic agents in childen with poor appetite.

BACKGROUND: Of the feeding disorders in children, poor appetite is probably one of the most common complaints notified by parents. Since gastric motility disorders may be the cause of this symptom, the aim of our study was to investigate the effect of prokinetics on children with poor appetite and delayed gastric emptying. METHODS: Poor appetite was graded by VAS at start and end of treatment. Delayed gastric emptying was assessed by 99mTechnetium tincolloid scintigraphy. Malnutrition was defined according to Waterlow criteria. After initial assessment behavioural feeding recommendations were provided and trimebutine given for 6 months. Scintigraphy was repeated during treatment. Anthropometrical measurements and daily energy intake calculations were performed monthly RESULTS: 21 children (M/F: 12/9) with a mean age of 7 +/- 3 years were studied. At the end of treatment, weight and height standard deviation scores improved significantly (-1.0 +/- 0.6 vs. -0.8 +/- 0.7, p = 0.008 and -1.1 +/- 1.0 vs. -0.8 +/- 0.9, p=0.003, respectively) and malnutrition rate decreased from 81% to 66.7%. Improvement of gastric emptying was shown in 11 out of 16 children but was not statiscally significant (P = 0.059). The visual analogue scale scores of appetite showed significant improvement with therapy (7.5 +/- 1.3 vs. 5.3 +/- 2.0, p = 0.012). CONCLUSION: Children with poor appetite who have delayed gastric emptying might benefit from prokinetic agent therapy combined with behavioural feeding recommendations.

The prevalence of liver function abnormalities in pediatric celiac disease patients and its relation with intestinal biopsy findings.

UNLABELLED: Abnormal liver function tests and liver damage are seen frequently with celiac disease. However, the pathogenesis of liver functions abnormality is not clearly understood. The aim of this study was to determine the frequency of abnormal liver functions in children with celiac disease and its relation with anthropometric measurements and severity of intestinal damage. PATIENTS AND METHODS: Twenty seven patients with celiac disease were included in the study. Anthropometric and laboratory examinations and intestinal biopsies were performed in all the patients. Mucosal lesions were classified according to the Marsh classification. Villous area, crypt height and mitotic count were measured morphometrically for all biopsy samples. RESULTS: The mean age of patients was 6 +/- 5 years on admission. Alanine aminotransferase levels were normal (group 1) or elevated (group 2) in 20 and 7 patients, respectively. The mean alanine aminotransferase levels were 22.0 +/- 7.2 in group 1 and 70.5 +/- 31.1 U/L in group 2 patients, (p < 0.001). Complaints, mean age, height for age, weight for height, serum albumin level, villous area, crypt height and mitotic count were not significantly different between the two groups. Similarly, the ratio of Marsh classification was not significantly different between the two groups. All patients were given a gluten-free diet. Serum aminotransferase values returned to normal after 7.4 +/- 2.7 months of a gluten-free diet. CONCLUSION: Abnormalities of liver functions are frequently seen in paediatric celiac disease patients. These abnormalities are not correlated with malnutrition and severity of intestinal mucosal lesions. Liver enzyme activities return to normal levels in a few months after gluten-free diet.

Assessment of protein-energy malnutrition in children with chronic arthritis.

Protein-energy malnutrition (PEM) has been estimated to occur in 10 to 50% of children with juvenile chronic arthritis (JCA). Thirty-eight children with JCA were evaluated and their nutritional status determined, and they were compared with 23 healthy sex and age-matched children as controls. A standardized, 9-parameter comprehensive nutritional assessment profile was used. The simple anthropometric measurements, height and weight for age, were abnormal in 30% and 27% of the patients, respectively. A detailed evaluation revealed that 71% had abnormal somatic protein stores, and that they also had significantly low levels of visceral protein stores, when compared to their healthy peers. The results were consistent with the fact that inflammation put the JCA patients at significant risk for developing complicated malnutrition and it might result in PEM without any obvious signs of malnutrition. A nutritional screening test would be very useful in detecting early PEM in children with chronic arthritis.

Helicobacter pylori infection in children with constitutional delay of growth and puberty.

Helicobacter pylori is a gastroduodenal pathogen strongly associated with chronic gastritis and duodenal ulceration. It is thought that H. pylori infection might be one of the causes of growth retardation in children. The aim of this study was to evaluate the seroprevalence of H. pylori in children with constitutional delay of growth and puberty (CDGP). H. pylori seropositivity was studied in 24 children with CDGP (22 M, 2 F) and 32 healthy age-matched children with normal pubertal development. Mean age of the children with CDGP was 14.53 +/- 1.12 yr and all of them had been diagnosed as CDGP after physical and laboratory assessment. H. pylori IgG positivity was detected in 16 of the 24 children with CDGP (66.6%) and 12 of 32 controls (37.5%) (p <0.05). This finding is consistent with the hypothesis that H. pylori infection could be one of the environmental factors causing CDGP.

The effect of vitamin A on the course of renal ablation nephropathy.

Renal scarring due to pyelonephritis was shown to improve in rats given vitamin A. We evaluated the effect of vitamin A in a renal ablation nephropathy model. Four groups, each including 7 rats with 5/6 nephrectomy, were formed: group I (no vitamin A), group II (60 kIU vitamin A), group III (120 kIU vitamin A), and group IV (180 kIU vitamin A). Four sham-operated rats comprised the control group. After 6 weeks of 5/6 nephrectomy, the rats were sacrificed and serum creatinine, vitamin A, and beta-carotene levels were determined in addition to histopathological evaluation of the remnant kidneys. The tubulointerstitial and glomerular changes were graded as "0-3" and "0-5" respectively, in accordance with the severity of the lesions. Tubulointerstitial score (TIS), mean glomerulosclerosis score (MGS, arithmetical mean of the sclerosis scores of 100 glomeruli), and severity of glomerulosclerosis index (SGI, ratio of the number of glomeruli with grade > or = 3 sclerosis to the total number of glomeruli examined) were calculated for each rat. Serum creatinine levels were higher in the study groups than the control rats (P < 0.05), but there was no significant difference between the study groups (although the levels increased as the dose of vitamin A increased). Serum vitamin A levels were significantly higher in the groups given vitamin A than the control rats and group I (P < 0.05). In addition, serum vitamin A levels increased significantly in parallel to increasing doses of vitamin A (P < 0.05). Serum beta-carotene levels did not differ between the groups, except for group II, which had lower levels than controls (P = 0.01). MGS and SGI were significantly higher in the study groups than control rats (P < 0.05), but did not differ between the study groups. Study and control rats were not different with respect to TIS, but there was a difference between the control group and group III (P = 0.04). Group II had the lowest MGS, SGI, and TIS scores among the study groups. When all the rats were considered together, vitamin A levels did not correlate with the MGS and SGI, but correlated positively with the TIS (r = 0.391, P = 0.027). beta-Carotene levels also did not correlate with the MGS, SGI, and TIS. In conclusion, vitamin A administration did not significantly affect the clinical and pathological course of renal ablation nephropathy in rats. Furthermore, higher doses of vitamin A might even damage renal tissue.

Chronic hypoxemia leads to reduced serum IGF-I levels in cyanotic congenital heart disease.

Cyanotic congenital heart disease in children commonly causes more pronounced growth retardation in comparison with acyanotic congenital heart disease. Chronic hypoxemia has been suggested as the cause of poor growth in these patients, but the relationship between serum IGF-I levels and chronic hypoxemia is unclear. Serum IGF-I concentrations, oxygen saturation and nutritional status were evaluated in 29 patients with cyanotic congenital heart disease, and serum IGF-I levels were compared with a group of 20 well-nourished, age-matched control children to assess the relationship between IGF-I levels and chronic hypoxemia. The nutritional status of each patient was determined by using anthropometric parameters and calorie and protein intake ratios. The patients were divided into malnourished and well-nourished groups (21 and 8 patients, respectively) according to their nutritional status. Serum IGF-I concentrations were measured in the two patient groups and the controls. The malnourished group had the lowest IGF-I levels (48.14 +/- 21.8 ng/ml, p<0.05). However, the well-nourished group's IGF-I levels were significantly lower than the control subjects' despite improved nutritional status (85.5 +/- 30.2 and 107 +/- 19.7 ng/ml, respectively, p<0.05). In addition, we found a positive correlation between serum IGF-I levels and oxygen saturation of the patients (r=0.402, p<0.05). These findings indicate that chronic hypoxemia has a direct or indirect effect to reduce serum IGF-I concentrations and this may be a cause of the increased growth failure in patients with cyanotic congenital heart disease.

Helicobacter pylori and Behçet's disease.

BACKGROUND: Recent investigation of the etiology of Behçet's disease (BD) has focused on heat shock proteins (HSP) which belong to the HSP 60 family. Both the gastric pathogen Helicobacter pylori (HP) and BD may cause ulcers in the gastrointestinal tract and, HP expresses HSP 60. OBJECTIVE: Whether HP is linked to the pathogenesis of BD or not, and to investigate the influence of HP eradication on clinical parameters of BD. METHODS: Patients with BD were divided into two groups. Group I comprised 49 patients and was investigated for HP seroprevalence and compared with age- and sex-matched controls. Group II comprised 20 patients with BD and HP infection diagnosed by serological and endoscopic examinations as well as the rapid urease test (RUT). A 1-week eradication therapy was administered for HP infection. Patients were examined for the course of BD at monthly intervals. Two months after the eradication therapy, patients underwent an endoscopic examination and RUT for eradication control. Seven patients were excluded because of eradication failure. Thirteen patients were evaluated for the influence of HP eradication on clinical manifestations of BD. The number and size of oral and genital ulcers before the eradication and at the end of the follow-up period were compared statistically. RESULTS: HP seroprevalence between patients with BD and controls did not show significant difference. In 13 patients with BD, the number and size of oral and genital ulcers diminished significantly and various clinical manifestations regressed after the eradication of HP. CONCLUSION: HP may be involved in the pathogenesis of BD.

Precocious puberty in a patient with indicanuria.

A 7-5/12 year-old girl, who was followed-up after diagnosis of indicanuria, presented with symptoms of bilateral breast enlargement. Her breast development was at Tanner stage II. No pubic or axillary hair was observed. Pelvic ultrasonography revealed multiple follicles on both ovaries. Basic endocrinological evaluation and cranial magnetic resonance imaging (MRI) were normal. The diagnosis of precocious puberty was established with respect to the pubertal response to GnRH stimulation test. Although precocious puberty has been reported associated with some metabolic diseases, this is the first description in a patient with indicanuria. The question of whether precocious puberty in our patient with indicanuria is a coincidence or whether it is related to metabolic changes activating the hypothalamo-pituitary-gonadal axis remains open.

The role of vitamin A in preventing renal scarring secondary to pyelonephritis.

OBJECTIVE: To evaluate the efficiency of exogenously administered vitamin A in preventing renal scarring caused by experimental pyelonephritis in rats. MATERIALS AND METHODS: Forty Wistar rats were injected with 0.1 mL of solution containing Escherichia coli (1010 /mL) into both renal medullae. Five equal groups were then formed: group 1 was treated only with ciprofloxacin (30 mg/kg per day, twice daily, intraperitoneally) for 5 days, starting 3 days after bacterial inoculation; in group 2, 60 kIU of vitamin A was injected intramuscularly with the bacterial inoculation; in group 3, 60 kIU of vitamin A was injected similarly, but 3 days after bacterial inoculation; in group 4, 60 kIU of vitamin A was given orally with the bacterial inoculation; and group 5 was treated with ciprofloxacin for 5 days and vitamin A intramuscularly from the third day after bacterial inoculation. All the rats were killed 6 weeks after bacterial injection; blood samples were obtained to determine serum vitamin A and beta-carotene levels, and both kidneys were examined pathologically for scarring, graded as 0 (none), 1 (mild), 2 (moderate) and 3 (severe). RESULTS: Serum vitamin A levels were higher in the rats given vitamin A (group 2-5) than in group 1, being highest in group 4, although only this group had significantly higher levels of vitamin A than group 1 (P<0.05). Histopathologically, the fibrosis was mildest in groups 2 and 4 (two of 16 kidneys grade 1), whereas it was most severe in group 1 (all 16 grade 2-3). Fibrosis was significantly less in groups 2-5 than in group 1 (P<0.05). There was a significant negative correlation between vitamin A levels and the sum of the fibrosis, inflammation and tubular atrophy scores of all rats (r=-0.391, P<0.02). beta-carotene levels were unrelated to renal scarring. CONCLUSION: The administration of vitamin A could have a role in preventing renal scar formation from pyelonephritis induced experimentally in rats.

Serum vitamin A and beta-carotene concentrations and renal scarring in urinary tract infections.

OBJECTIVE: To evaluate the role of vitamin A on renal scarring in recurrent urinary tract infections (UTIs). DESIGN: Twenty three children with UTIs and renal scarring (mean (SD) age 7.3 (3.9) years) and 91 children without renal scarring (6.4 (3.4) years) were studied. All the children had serum vitamin A and beta-carotene measurements and nutritional evaluation. Renal scarring was assessed by technetium-99m dimercaptosuccinic acid (99mTc DMSA) scanning. Nutritional status of all the patients was within normal limits and not different between the groups. RESULTS: Mean (SD) serum vitamin A and beta-carotene concentrations were not significantly different between the patients with and without renal scarring (vitamin A 53.2 (22.6)/46.8 (17.0) micrograms/dl and beta-carotene 232.3 (201.3)/272.4 (86.0) micrograms/dl respectively). However, when the patients with renal scarring and with greater than 10% difference among the DMSA uptakes of their kidneys (11 cases) were evaluated, a significant negative correlation was determined between the serum vitamin A concentrations and the magnitude of the difference in uptakes of each kidney. The same relation was not true for serum beta-carotene concentrations. CONCLUSION: This study demonstrated a relation between serum vitamin A concentrations and magnitude of hypoactivity in 99mTc DMSA scanning in kidneys with advanced scarring.

A case of juvenile ankylosing spondylitis and Crohn's disease.

Juvenile ankylosing spondylitis (JAS) is a chronic inflammatory arthritis of the peripheral and axial skeleton, frequently accompanied by enthesitis. About four percent of patients with JAS have ulcerative colitis or Crohn's disease. Crohn's disease is the more common of the two and is diagnosed in 26 percent of patients with chronic spondyloarthropathy. In this paper, a 14-year-old male patient is presented as a typical case of juvenile ankylosing spondylitis and Crohn's disease with low back pain, morning stiffness, limited motion in anterior and lateral flexion and extension, left sacroiliitis, ankylosis in the apophyseal joints of the lumbar vertebrae, abdominal pain, bloody diarrhea, characteristic histopathologic changes of colonic involvement such as lymphoid follicles, fissures, submucosal polymorphonuclear cell infiltration and definite ganglion cells. The current therapy with mesalazin, having fewer side effects than sulfosalazin, and its applicability in combination with naproxen sodium is also discussed.

Vitamin A and beta carotene levels in constitutional delay of growth and puberty.

The objective of this study was to investigate the vitamin A (vit A) status and beta carotene levels of children with constitutional delay of growth and puberty (CDGP). Serum vit A and beta-carotene levels of 26 children with CDGP were measured. 20 age-matched healthy children with normal pubertal development served as controls. Except for the height SDS, which was significantly lower in the CDGP group (p < 0.05), no significant differences were found between chronological ages, weight for height indices and beta-carotene levels of the two groups (p > 0.05). Although serum vit A levels of children in both groups were within normal limits according to WHO criteria, serum vit A levels were significantly lower in the CDGP group than in controls (44.13 +/- 12.25 and 59.60 +/- 19.75 micrograms/dl respectively, p < 0.05). It was concluded that vit A deficiency may play a role in CDGP in developing countries.

Combination of steroid with azathioprine in treatment of giant cell autoimmune hepatitis.

Giant cell hepatitis is a rare disorder after the newborn period. Drugs, autoimmunity, and viruses (lately, paramyxovirus infection) have been implicated in its etiology. Without treatment, liver dysfunction is progressive and fatal. Immunosuppression with steroids and azathioprine has been demonstrated to sustain improvement in the disease. In this report, a one-year-old boy who has giant cell hepatitis with Coombs' positive hemolytic anemia and anti-smooth muscle antibodies is presented, and the course of the disease and the patient's response to treatment with steroid and azathioprine is reviewed.

Single photon emission computed tomography in adrenoleukodystrophy. A case report.

Adrenoleukodystrophy (ALD) is a genetic disorder leading to progressive dysfunction of the adrenal cortex and nervous system white matter. Accumulation of fatty acids in cerebral white matter results in some anatomical and functional changes which are detectable by imaging studies. Lately, single photon emission computed tomography (SPECT) has been suggested for evaluation of cerebral perfusion changes. In this report, magnetic resonance imaging (MRI) and SPECT neurolmaging of an eight-year-old boy with ALD are presented. SPECT revealed more extensive involvement than that demonstrated by MRI. Its role in early prediction of the extention of disease is stressed.

Mcardle's disease. A case report.

McArdle's disease is a hereditary, metabolic myopathy characterized by weakness and muscle cramps after exercise, appearing mostly in the second or third decade of life. Due to myophosphorylase deficiency in skeletal muscle, glycogen cannot be used and deposited in the sarcolemmal spaces, leading to lack of endurance to sustained work. The ischemic exercise test is a screening procedure for muscle energy disorders, and the diagnosis is confirmed by reduced enzyme activity in muscle biopsy. In this report, a family with one child having enzyme assay-proven McArdle's disease and two other children demonstrating a positive ischemic exercise test is presented.

Anthropometric and nutritional evaluation of short statured children from low socio-economic class.

The objective of this study was to evaluate the anthropometric parameters of short statured children examined in our outpatient clinic. 367 children were evaluated. Body weight for age, weight for height, height for age and body mass index of subjects were expressed as percent of standards. Wasting and stunting were established according to Waterlow's criteria /4/. Of 367 children, 27.8% were wasted and stunted; 59.1% were stunted; 3.8% had constitutional delay of growth and 9.3% had growth hormone deficiency. Ninety-seven percent of growth hormone deficient children had deficiency in weight as well as height. This study indicates that most short statured children and some growth hormone deficient children present with anthropometric indices of chronic nutritional deficiency in a country where nutritional deprivation is frequent.