Data concerning the transcription of growth hormone and the various interactions between growth hormone/insulin-like growth factor (IGF) axis in uremia, acidosis and nutrition are presented. The recent evidence of tissue resistance to growth hormone in uremia provided the medical rationale for the use of growth hormone in chronic renal failure. The growth hormone receptor resistance in uremia and the decreased IGF-I by acidosis are additional rationale for the use of growth hormone. New findings of how acidosis causes the reduction of IGF-I expression at the growth plate of the long bone and the significant proteolysis after even small changes in serum bicarbonate content are presented to provide the pediatrician with an overview of these recent advances.
Pemphigus vulgaris is an autoimmune vesiculobullous disease of the skin and the mucous membrane. It most commonly involves the population of the fifth and sixth decades, and is extremely rare in children. We report a case of this disease in a 13-year-old boy, who was documented to have pemphigus vulgaris with historical, histopathological, and immunological criterion. He received corticosteroid and immunosuppressive drugs and a good response was achieved. We concluded that pemphigus vulgaris is a rare disease in children, but it should be kept in mind as a differential diagnosis of oral ulceration along with skin manifestation in children.
Liver tumors are relatively rare in childhood, but may be associated with a range of diagnostic, genetic, therapeutic, and surgical challenges sufficient to tax even the most experienced clinician. This article outlines the epidemiology, etiology, pathologic condition, initial workup, and management of hepatocellular carcinoma in children and adolescents.
Institute of Pediatric Gastroenterology is superspecialised referral institute for all Pediatric Gastroenterological diseases from all over the country and for adjoining countries. We have our data and experience on 10,500 cases of proven Hepatitis E (HEV) in Pediatric population. HEV is non-enveloped 27-30 nm diameter RNA virus, prototype for alpha-like supergroup of positive stranded RNA virus. Indian HEV strain has 97% nucleotide and 98% amino acid sequence identity with Chinese strains but much diversity with Mexican strain. More than 70% acute hepatitis occurring in Pediatric population in this subcontinent are caused by HEV and 80% of these are sporadic. 90% cases were enterically transmitted, spread primarily by fecally contaminated drinking water (70%) and by food (20%), in 9.5% case spread probably was because of person to person and household contact. We could demonstrate HEV in urine, respiratory secretions. Interestingly we found HEV in insects like Flies, Cockroaches, and also in engorged Bedbugs and in Mosquitoes, apart from briefly boiled Mussels, and partially cooked cockles. Maternal-neonatal transmission could be seen if mother had HEV infection in third trimester of pregnancy. In 5 cases we could demonstrate HEV in breast milk. By studying on 10 volunteers, 40% have anicteric form only accompanied by anorexia, epigastric pain. HEV appeared in serum before the icteric phase. Shedding of virus in stool starts before the icteric phase and continued during the high levels of abnormal ALT. Hepatitis IgG anti-HEV persist up to 4 years. In 5 cases we could establish Transfusion associated Hepatitis (TAH). No chronicity could be documented. 5% cases had fulminant viral Hepatitis (FVH)/Sub fulminant viral Hepatitis (SVH), alpha-interferon (IFN) has been proved beneficial in these cases, further use of intravenous PGEl could also be beneficial. Inadequate chlorination of drinking water was an important additional factor for causing epidemics. A free residual chlorine concentration of at least 0.5 mg/l for minimum of 30 minutes is considered adequate as quality of drinking water.
Encouraging results have been reported from several large trials of tetravalent rhesus rotavirus vaccine, with efficacy of 70-80% against severe disease. A recent Venezuelan study showed similar results to trials in USA and Europe. The vaccine may soon be licensed in USA. It provides the exciting prospect of a strategy to prevent one of the world's major child killers. Other candidate vaccines are under development including human-bovine reassortants, neonatal strains, non-replicating rotaviruses, vector vaccines and other genetically engineered products. Second and third generation rotavirus vaccines are on the horizon. The need for a rotavirus vaccine is well accepted by paediatricians, but public health authorities need to be lobbied. Other issues which need to be addressed include relative importance of non-group A rotaviruses, possible administration with OPV, the influence of breast feeding, and most importantly, cost. It is essential that rotavirus vaccine is somehow made available to all of the world's children, not just those in developed countries.
We present a term, 2950 gm female neonate who was delivered by vacuum extraction and subsequently developed convulsions 23 hours after birth. Acute subduralhematoma was rapidly diagnosed by computed tomographic brain scan and successfully removed. The patient had a smooth postoperative recovery but cerebral infarction was found during follow up imaging studies. Magnetic resonance angiography revealed poor visualization of bilateral posterior cerebral arteries. After a follow-up period of 4 months, the infant had normal neurological development except for mild microcephalus. We report the occurrence of cerebral infarction in an infant following evacuation of a subdural hematoma. Onset of the late complication suggests the importance of serial neuroradiological and clinical evaluations.
A comparison was made of the outcome of 73 neonates born with their birth weight of 500-999 gm in National Taiwan University Hospital during the period between January 1, 1993 and December 31, 1996 (Period II), with the outcome of 21 such neonates born between April 1, 1988 and October 31, 1992 (Period I). Exclusion criteria included parental refusal for resuscitation, and major anomalies. Data were collected via a predetermined record sheet. The incidences of the extremely-low-birth-weight infants were 23/10,173 (0.23%) and 81/13,835 (0.59%) in Periods I and II, respectively. Early neonatal mortality rate was significant decreased in Period II (43% versus 14%). The limit of viability was improved from gestational age of 26 weeks or 700 gm to gestational age of 24 weeks or 600 gm. The incidence of neonatal morbidity (80% versus 50%) and total survival rate (48% versus 60%) have not changed significantly as seen in this limited number of cases. This study concluded that, with the introduction of exogenous surfactant and modern neonatal care, early neonatal survival rate and the limit of viability were improved.
A previous healthy 5 year-old girl developed a right hyperlucent lung following Mycoplasma pneumoniae pneumonia 14 months before admission. Serial chest radiographs revealed a persistent right upper lobe atelectasis and gradual development of the right hyperlucent lung associated with frequent bouts of wheezing and exertional dyspnea. Physical examination showed markedly decreased breathing sounds in the right hemithorax with fine inspiratory crackles and expiratory wheezes. A diagnosis of Swyer James syndrome was confirmed by the exclusion of other causes of unilateral hyperlucent lung using computed tomographic scans of chest, fiberoptic bronchoscopy and lung perfusion scintigraphy. She has been followed up at our hospital using anti-asthmatic medication.
Necrotizing enterocolitis (NEC) is the most common gastrointestinal emergency in premature infants. A major component of the pathophysiology of NEC is the nature of the interaction of bacteria with the premature gut. Intestine microflora are important to the host in resistance to bacterial infections. Diet and environmental conditions can influence this ecosystem. A breast-fed full-term infant has a preferred intestine microbiota in which bifidobacteria predominate over the potentially harmful bacteria, whereas in formula-fed infants coliforms, enterococci and bacteroides predominate. The pattern of bacterial colonization in the premature neonate gut is quite different from that in the gut of the healthy full-term infant. Those infants requiring intensive care acquire intestinal organisms slowly, and the establishment of bifidobacterial flora is retarded. A delayed bacterial colonization of the gut with a limited number of bacterial species tends to be virulent. Bacterial overgrowth is one of major factors promoting bacterial translocation. The aberrant colonization of the premature infant may contribute to the development of NEC. Breast feeding protects infants against NEC. Oligosaccharides and glycoconjugates, natural components in human milk, may prevent intestinal attachment of enteropathogens by acting as receptor homologues. Probiotics and prebiotics modulate the composition of human intestine microflora to the benefit of the host. The beneficial effects may result in the suppression of colonization of harmful microoganisms and/or the stimulation of bifidobacterial growth. In the future, control and manipulation of bacterial colonization in the neonate gut may be a new approach to the prevention and treatment of bacterial intestinal disease of various etiologies.
Between January 1996 and June 1998, 24 children with symptomatic hypomagnesemia were enrolled for analysis of their symptomatology, causes, and treatment outcome. Hypocalcemia and hyperphosphatemia suggesting impaired parathyroid function were the most common electrolyte disorders. Hypokalemia was also frequently noted. The related symptoms including seizure, tetany, and weakness were common. Drug-induced renal magnesium wasting was the most common cause of symptomatic hypomagnesemia, and tended to occur in older children using aminoglycoside, furosemide, and amphotericin-B. The associated gastrointestinal causes might add a minor contribution to the development of hypomagnesemia. Analyses of PTH levels in 13 children suggested that inhibition of PTH synthesis or secretion was responsible for hypomagnesemic hypocalcemia in most patients. However, peripheral PTH resistance might also account for the mechanism in a few patients. In most patients, symptomatic hypomagnesemia was transient, and improved after magnesium provision. Only one child with congenital renal magnesium wasting and two with primary hypomagnesemia needed long-term magnesium treatment.
We studied 30 atopic children who suspected of milk allergy by past history (age ranging from 1 yr 4 mo to 9 yr 6 mo, mean age: 5.03 yr.) diagnosed as having asthma, atopic dermatitis and/or allergic rhinitis. These 30 atopic children had been screened from the patients at our outpatient clinic by the Pharmacia CAP system RAST FEIA. All of them showed the presence of at least Class II (greater than 0.7 ku/l) IgE specific to proteins in cow's milk. Further analysis found IgE specific to alpha-lactoalbumin (alpha-LA) elevated in 1 patient (3.3%), 1 patient (3.3%) to beta-lactoglobulin (beta-LG), 4 patients (13.3%) to alpha-LA and beta-LG, 5 patients (16.7%) to casein, 8 patients (26.7%) to casein and alpha-LA, 11 patients (36.7%) to casein, alpha-LA and beta-LG. After 3 weeks' cow-milk-free diet, the patient's milk challenge test was performed at our outpatient clinic. According to the test result, none of these 30 atopic children showed clinical evidence of significant allergic reaction to cow's milk in the skin, the gastrointestinal tract or the respiratory tract either within two hours after the challenge test or within 3 days after they went home. We therefore conclude that: (1) No single major allergen is apparent in cow's milk: casein, alpha-LA and beta-LG all show a high proportion of positive reaction. (2) Many atopic children fully tolerate cow's milk, although they have high titer of IgE antibodies specific to cow's milk. The RAST test is only the first step to screen patients with suspected IgE-mediated allergies. To make sure, any positive reaction must be confirmed by the "golden standard" for diagnosis, i.e., the double-blind placebo-controlled food challenge.
We established reference ranges of serum gonadotropins, testosterone, 17-hydroxyprogesterone, aldosterone and renin in order to evaluate the causes of abnormal genitalia in Taiwanese newborns. Random serum samples were obtained from 60 full term newborns aged 3 to 5 days and the mean birth weight was 3.35 +/- 0.25 kg. The concentrations of follicle-stimulating hormone (FSH), luteinizing hormone (LH), total testosterone, 17-hydroxyprogesterone, aldosterone and renin were measured by radioimmunoassay. Mean FSH, LH, testosterone, 17-hydroxyprogesterone, aldosterone and renin were 0.61 +/- 1.13 IU/L, 2.95 +/- 2.72 IU/L, 0.70 +/- 0.47 ng/mL, 3.32 +/- 2.13 ng/mL, 795.73 +/- 511.36 pg/mL and 31.43 +/- 27.30 pg/mL, respectively. Significantly higher levels of testosterone (p < 0.05) were found in male than in female infants. Serum levels of FSH were significantly higher in female newborns (p < 0.05). There was no significant difference in the serum levels of LH between male and female infants. This study provides reference ranges of serum gonadotropins and some of the adrenocortical hormones in newborns.
To investigate the effect of erythromycin on feeding intolerance in very low birth weight infants, from February 1997 to December 1997 twenty infants weighing less than 1500 g, with prolonged intolerance of enteral feeding, were enrolled in this study. The protocol for erythromycin treatment was: a loading dose of 30 mg/kg/day, divided into three portions given every eight hours intravenously for 1 hour over a three day period; then a maintenance dose of 3-5 mg/kg intravenously for one hour once a day was given until full feeding was well established. The assessment of erythromycin effect was the daily net orogastric balance (volume of orogastric tube feeding minus volume of orogastric aspirates). The mean gestational age was 27.1 +/- 2.0 weeks (mean +/- SD) and the mean birth weight was 1025 +/- 196 g. The mean age when erythromycin started was 19.5 +/- 14 days; the mean days after the initiation of erythromycin when orogastric tube feeding could be started and full feeding established were 2.4 +/- 1.1 days and 15.1 +/- 2.2 days, respectively. At the beginning of erythromycin treatment, the net balance of tube aspirates was -4.8 +/- 4.1 ml. The net balance rose significantly to 30.6 +/- 15.3 ml, 92.6 +/- 25.4 ml and 125.3 +/- 18.1 ml at 7, 14 and 21 days after erythromycin treatment, respectively. In conclusion, erythromycin treatment is a safe method to improve intolerance of enteral feeding in very low birth weight infants. It is suggested that the effect of erythromycin on gastrointestinal motility in these infants should be further investigated in the context of a randomized, controlled trial before widespread clinical implementation of this treatment.
Prolonged hospitalization in infants suffering from chronic lung disease who require continuous oxygen therapy can be avoided by oxygen administration at home. In the period from August 1995 to September 1996, 155 very low-birth-weight (VLBW) infants were consecutively admitted to the neonatal intensive care unit of Mackay Memorial Hospital. Of the 155 infants, 72% (111/155) survived to discharge. However, 34% (38/111) of the survivors developed chronic lung disease. Twenty-three infants with chronic lung disease underwent home oxygen therapy after 107.0 +/- 43.6 days of hospitalization. The mean duration of home oxygen therapy was 4.3 +/- 3.4 months. In the first year after discharge, 91% of the patients required rehospitalization. One patient died during the fourth hospitalization. Follow-up information on growth and development at one year of corrected age was available for 19 patients. Five of the 19 patients had a body weight below the 5th percentile. Five of the 19 infants were mentally retarded and 12 of the 19 patients had significantly delayed motor development. In conclusion, carefully supervised home oxygen therapy permits safe early discharge of selected VLBW infants with chronic lung disease. Their somatic and psychomotor development should be carefully followed up.
To understand the difference in incidence and distribution of germ cell tumor (GCT) between cases seen in this institute and Western studies, 98 cases of GCTs collected from 1979 to 1996 were included 50 with gonadal GCTs, 9 with mediastinal GCTs; 9 with retroperitoneal GCTs, 10 with sacrococcygeal GCTs and 20 with intracranial GCTs; all were analyzed retrospectively by chart review. The incidences of testicular and intracranial GCT in all GCTs were higher in this study, i.e. 26.5%(26/98) and 20.4%(20/98) compared to 7% and 6% of American reports. But the incidence of sacrococcygeal GCT in all GCTs was lower in this study, i.e. 10.2%(10/98) compared to 41% of American reports. The incidences of testicular and ovarian GCT in all GCTs were about the same in this study, i.e. 26.5%(26/98) and 24.5%(24/98) compared to 7% and 29% of American reports; 51%(50/98) of all GCTs were gonadal in this study compared to 36% in the American reports.
Williams-Beuren syndrome (WBS) is a rare neurodevelopmental disorder, characterized by distinct facial changes, growth deficiency, mental retardation, supravalvular aortic stenosis (SVAS)/peripheral pulmonary stenosis, and associated at times with infantile hypercalcemia. A pilot study has been carried out to assess the reliability of the detection of hemizygosity at the elastin locus by fluorescence in situ hybridization (FISH) analysis as a diagnostic test in both classical and atypical WBS. Eight subjects with classical WBS and four others in whom a diagnosis could not be confirmed on clinical criteria alone were enrolled. In the classical WBS group, five (5/8) had a visible interstitial 7q11.22-11.23 deletion detected by high-resolution banding, and all (8/8) had a submicroscopic deletion of the elastin locus on chromosome 7 by FISH analysis. In the atypical WBS group, only one (1/4) had elastin deletion. The other three, with isolated SVAS, had normal development and minimal signs of WBS. Furthermore, the patients with microscopic 7q11.22-11.23 deletion have more associated features of WBS than those without visible interstitial deletions by high-resolution banding. These results, therefore, emphasize the importance of a combined high-resolution and molecular cytogenetic (i.e., FISH) approach to diagnosis and suggest that the degree to which microscopic/submicroscopic deletions of chromosome 7 extending in beyond the elastin locus may explain some of the phenotypical variability found in WBS.
From February to June in 1996, there were 47,800 students for health examination in Taichung City, Taiwan. The population consisted of the first and fourth graders of primary schools and the first grader of junior high schools. We selected 3,452 healthy students for further study by two stage sampling. In this report, 52.5% of the students were boys, and 47.5% of the students were girls. The mean age of students was 9.9 +/- 2.4 years. Girls at age 7 and age 10 had higher activity of alkaline phosphatase than boys at the same age. The peak of alkaline phosphatase activity in girls occurred at age 10. Alkaline phosphatase activity was significantly related to weight-length index by multiple regression analysis (p < 0.05). Although clinical application as an obesity factor still needs further investigation, in the future it may be well to routinely check this enzyme when assessing childhood obesity.
Simultaneous blood and saliva samples were collected for determination of the relationship between serum and saliva erythropoietin (EPO) concentrations in 12 adults (Group I), 15 full-term neonates (Group II), and 11 premature infants (Group III). Saliva was collected with a modified sputum-collecting tube combined with a vacuum suction pump. Serum and saliva EPO concentration was measured by enzyme-linked immunosorbent assay. The relationship between serum and saliva concentrations was explored using (1) regression analysis and (2) serum-to-saliva ratio. Salivary concentrations approximated 15 to 30% of the serum concentrations based on the serum-to-saliva ratios. Significant correlation was observed between serum and salivary concentrations in each group (p < 0.05). The regression analyses produced formulas for predicting serum EPO concentrations from saliva EPO concentrations which seemed to fit the data well. The resulting formula is surprisingly consistent with that derived from Group I. The ratio models seem to fit the data well as regression models. From the results it is concluded that the use of salivary samplings for serum EPO in adults, full term and premature infants may be a possible alternative method to blood samplings.
