bluuebunny/crossref_metadata_2025 huggingface dataset:
Molecular epidemiology of hepatitis A virus in Korea1
Authors: Byun Kwan Soo , Kim Jong Hun , Song Ki‐Joon , Baek Luck Ju , Song Jin‐Won , Park Sang Hoon , Kwon Oh Sang , Yeon Jong Eun , Kim Jae Seon , Bak Young Tae , Lee Chang Hong
Published: March 2003

Background: The prevalence of antibodies for hepatitis A virus (anti‐HAV) in adolescents and young adults has decreased remarkably following the economic growth in Korea. As a result, this age group has a high risk for HAV infection paradoxically, and over 1500 cases of clinically overt hepatitis A occurred in 1998. Human isolates of hepatitis A virus (HAV) are categorized within four genotypes (I, II, III, and VII). In some geographic regions, closely related isolates cluster, suggesting endemic spread of the virus, while in other regions multiple genotypes circulate. Virtually no data are available with regard to the genetic relatedness of Korean strains of HAV.

Methods and Results: A 168 base pair segment encompassing the putative VP1/2A junction of the HAV genome was amplified by RT‐PCR and sequenced in sera of 18 Korean patients with a sporadic form of acute hepatitis A. Pairwise comparisons of the nucleic acid and amino acid sequences of 18 Korean isolates with one another revealed that the Korean isolates showed > 94.6% and > 96.4% identity, respectively. All of the 18 Korean isolates clustered within genotype IA, irrespective of the geographic locations and the time that hepatitis occurred. Unique amino acid sequence changes that had never been reported in genotype IA were found in nine of the 18 isolates. These changes were Gln→Ser and Lys→Arg in 2A‐19 and 2A‐10 amino acid positions.

Conclusion: The presence of single genotype and unique mutations may be related with the circulation of endemic HAV over a long period of time in Korea.
Epinephrine irrigation for the prevention of pancreatic damage after endoscopic balloon sphincteroplasty
Authors: Ohashi Akira , Tamada Kiichi , Tomiyama Takeshi , Wada Shinichi , Higashizawa Toshihiko , Gotoh Yasuhiko , Satoh Yukihiro , Miyata Takamitsu , Tano Shigeo , Ido Kenichi , Sugano Kentaro
Published: March 2003

Background and Aim: Endoscopic balloon sphincteroplasty (EBS) is an alternative to sphincterotomy for the treatment of bile duct stones. The purpose of this study was to determine if epinephrine irrigation of the papilla can prevent the pancreatic damage associated with EBS.

Methods: A total of 173 patients with bile duct stones were treated by EBS. After conventional endoscopic retrograde cholangiography, EBS was performed by using a biliary dilatation catheter (balloon diameter: 8 mm). The duct was then cleared by using Dormia baskets or retrieval balloon catheters. Mechanical lithotripsy was performed before extraction when the stones were greater than 8 mm in diameter. In 81 patients, the dilated orifice was irrigated with 40–120 mL (50 ± 37 mL) of 1:1 000 000 epinephrine (epinephrine group). In the remaining 92 patients, epinephrine irrigation was not performed (control group). Acute pancreatitis was defined by a serum amylase concentration fivefold greater than the upper limits of normal in association with abdominal pain.

Results: After EBS, serum amylase concentrations were significantly increased in both groups. However, the degree of hyperamylasemia was less in the epinephrine group than in the control group (617 ± 611 vs 1037 ± 1491 IU/L, P < 0.05). The incidence of pancreatitis was lower in the epinephrine group than in the control group (1.2 vs 7.6%, P < 0.05).

Conclusions: Epinephrine irrigation is a simple and useful method to prevent post‐EBS pancreatic damage and pancreatitis.
Role of UGT1A1 mutation in fasting hyperbilirubinemia
Authors: Ishihara Tomoaki , Kaito Masahiko , Takeuchi Keisuke , Gabazza Esteban C , Tanaka Yuji , Higuchi Kunihiro , Ikoma Jiro , Watanabe Shozo , Sato Hiroshi , Adachi Yukihiko
Published: March 2003

Background and Aim:

Low‐grade fasting hyperbilirubinemia is a common observation in healthy subjects (HS), whereas high‐grade fasting hyperbilirubinemia is believed to be a characteristic finding of Gilbert’s syndrome. This study was undertaken to assess the role of mutation in bilirubin UDP‐ glycosyltransferase gene (UGT1A1) on fasting hyperbilirubinemia.

Methods:

Analysis of UGT1A1 and a caloric restriction test (400 kcal for 24 h) were performed in 56 healthy subjects (25 males, 31 females), and 28 patients with Gilbert’s syndrome (18 males, 10 females). There were 29 healthy subjects with no mutation in UGT1A1, and 27 healthy subjects and 26 Gilbert’s syndrome patients with mutations in the coding and/or promoter (TATA box) regions of UGT1A1.

Results:

The mean increment of serum bilirubin (ΔSB) was 9.6 μmol/L (males) and 4.1 μmol/L (females) in subjects with no UGT1A1 mutation. Subjects with mutation in UGT1A1 showed higher levels of ΔSB than individuals without mutation. Among healthy subjects, gender difference in ΔSB values was observed only in individuals with the wild type of UGT1A1, but not in those with mutations in this gene.

Conclusion:

The results of the present study suggest that UGT1A1 mutation has a role in the development of high‐grade fasting hyperbilirubinemia after caloric restriction.
Multiple hepatic peribiliary cysts discovered incidentally at a medical examination
Authors: Okada Shun‐Ichi , Kojima Yuichiro , Morozumi Atsuro , Ainota Takao , Miyazaki Yoshiki , Akahane Yoshihiro
Published: March 2003

Abstract We report a living patient with multiple hepatic peribiliary cysts. It was discovered incidentally during an ultrasonographic screening at a medical examination. Peribiliary cysts are multiple retention cysts of peribiliary glands. Although many autopsy cases of peribiliary cysts have been reported, there are few clinical cases of it in living patients. A CT performed immediately after drip‐infusion cholangiography (DIC) was most useful for diagnosis in various imaging tests we performed.
Evaluation of intestinal mucosal function by measuring expired 14CO2 after oral administration of 14C‐putrescine
Authors: Sasaki Akira , Tsujikawa Tomoyuki , Fujiyama Yoshihide , Bamba Tadao
Published: March 2003

Background: Diamine oxidase (DAO) is the enzyme that degrades putrescine, the key main product of polyamine metabolism, and reflects enterocytic maturity of absorption because diamine oxidase activity is highest in the small intestine. We have already shown that expired 14CO2 after oral administration of 14C‐putrescine correlated with intestinal DAO activity. However, the influence of food composition and the mucosal adaptation after intestinal resection have not been elucidated.

Methods: Male Wistar rats were fed normal chow or an elemental diet (ED) for 2 weeks. Resected rats underwent 50% jejunectomy or 50% ilectomy. Expired 14CO2 levels, following oral administration of 14C‐putrescine were measured in all rats, and compared with the intestinal DAO activity and other mucosal parameters.

Results: In the ED group, the 14CO2 levels reached a peak earlier, and values were 2.9‐fold higher than in the group fed with normal chow. Mucosal alkaline phosphatase (ALP) and DAO activity in the ED group were also higher than in the group fed normal chow, although the mucosal wet weight was significantly lower in the ED group. In the resection groups, all expired 14CO2 values increased during measurement. The peak 14CO2 values in the jejunectomy group shifted earlier in the postoperative period. The mucosal DAO activity in both the resection groups was higher than it was in the control group at the fifth and 10th postoperative day. However, there were no differences among the three groups at the 15th postoperative day.

Conclusions: Our studies suggested that expired 14CO2 after oral administration of 14C‐putrescine correlates with mucosal DAO activity, and that it also reflects intestinal function.
Anti‐oxidant ebselen causes the resolution of experimentally induced hepatic fibrosis in rats
Authors: Wasser Shanthi , Lim Ganymede Y , Ong Choon‐Nam , Tan Carolyn E L
Published: March 2003

Background:

Hepatic fibrosis occurs because of injury to the liver parenchyma and biliary system. We have investigated the effect of an organic selenium anti‐oxidant, ebselen, in the resolution of experimentally induced hepatic fibrosis, and evaluated its effect on various paradigms involved in hepatic fibrosis.

Methods:

Following pretreatment with phenobarbitone, liver fibrosis was induced in male Fischer 344 rats by using carbon tetrachloride treatment for 10 weeks. Carbon tetrachloride‐treated rats were randomly assigned into two groups: (i) no ebselen; and (ii) ebselen administered for 3 weeks following a 10‐week carbon tetrachloride treatment period. Normal controls were: (i) neither carbon tetrachloride nor ebselen treated; or (ii) ebselen treated for 13 weeks. Liver sections were stained with hematoxylin and eosin, Masson trichrome and stained for reticulin by using silver impregnation. Reverse transcription–polymerase chain reaction was used to analyze the steady‐state levels of gene(s) involved in: (i) hepatic fibrosis, namely, transforming growth factor‐β1, procollagen I and III, tissue inhibitor of metalloproteinase‐1 and matrix metalloproteinase‐13; (ii) oxidative stress, namely, cytochrome P4502E1; and (iii) preneoplastic liver foci, namely, the placental form of glutathione‐S‐transferase.

Results:

Histological staining showed that ebselen resolves carbon tetrachloride‐induced hepatic fibrosis. Treatment with ebselen reduced steady‐state levels of transforming growth factor‐β1, procollagen I and III, tissue inhibitor of metalloproteinase‐1, cytochrome P4502E1 and placental form glutathione‐S‐transferase transcripts, and increased transcripts of matrix metalloproteinase‐13.

Conclusion:

These findings provide evidence that ebselen significantly causes the resolution of carbon tetrachloride‐induced hepatic fibrosis in rats.
Role of the spleen in liver fibrosis in rats may be mediated by transforming growth factor β‐1
Authors: AKAHOSHI TOMOHIKO , HASHIZUME MAKOTO , TANOUE KAZUO , SHIMABUKURO RINSHYUN , GOTOH NORIKAZU , TOMIKAWA MORIMASA , SUGIMACHI KEIZO
Published: March 2003

Background: The effect of the spleen on the cirrhotic liver is unknown.  Transforming growth factor‐β1 (TGF‐β1), which plays a crucial role in the matrix production during liver fibrosis, is an inhibitory factor regarding the regeneration of hepatocytes. In this study, we investigated the TGF‐β1 production in the spleen of cirrhotic rats and the effects of a splenectomy on the healing process from liver fibrosis.

Methods: Thirty‐six Wister male rats were used. Thioacetamide (TAA) was administered intraperitoneally for 24 weeks. The rats underwent either a sham operation (TAA + Sham) or a splenectomy (TAA + SPL). The improvements in liver fibrosis and liver regeneration were investigated 10, 30 and 60 days after the operations in each group. The effect of a splenectomy on the plasma concentration of TGF‐β1 in the portal vein was investigated by ELISA. The TGF‐β1 expressions in the spleen were measured using immunohistochemical staining and the degree of such expression was measured using RT‐PCR. The activity of TGF‐β1 in the portal vein of TAA + Sham and TAA + SPL was assessed by the inhibiting effect of rat parenchymal hepatocyte proliferation in primary culture.

Results: Liver regeneration (PCNA‐labeling index) in the TAA + SPL rats was stimulated more

at 10 and 30 days after the operation (P < 0.05) than in the TAA + Sham

rats, and the improvement of liver fibrosis (fibrosis rate) in the TAA + SPL rats

was higher at 60 days (P < 0.05) than in the TAA + Sham rats. The plasma concentration of TGF‐β1 of the portal vein in TAA + SPL rats was significantly lower than in the TAA + Sham rats for each period. Immunohistochemically, TGF‐β1‐positive stained cells were recognized in the spleen macrophages in the red pulp of cirrhotic rats. The plasma of the TAA + Sham rats at 10 and 30 days after the operation was significantly stronger than that of the TAA + SPL rats in inhibiting the proliferation of rat hepatocytes of primary culture. Inhibitory effects were then dose‐dependently neutralized by monoclonal TGF‐β1 antibody.

Conclusion: Spleen‐derived TGF‐β1 may thus play an inhibitory role in the healing of liver cirrhosis by inhibiting the regeneration of the damaged liver.
Helicobacter pylori infection promotes gastric carcinogenesis in a mice model
Authors: HAN SANG‐UK , KIM YOUNG‐BAE , JOO HEE‐JAE , HAHM KI‐BAIK , LEE WON‐HEUNG , CHO YONG‐KWAN , KIM DAE‐YONG , KIM MYUNG‐WOOK
Published: March 2003

Background and Aim:

Debate that Helicobacter pylori might play a causative role in gastric carcinogenesis still exists in spite of the World
Health Organization’s definition of H. pylori as a class I carcinogen.
In order to define the exact role of H. pylori infection in gastric carcinogenesis,
we established a mice model of H. pylori infection.

Methods:

One hundred and forty‐four female C57BL/6 mice were divided into nine groups according to N‐methyl‐N‐nitrosourea (MNU) treatment
and H. pylori infection. All mice were killed at the 50th or 80th week after treatment, and their histopathological changes were evaluated according to group.

Results:

The incidence of gastric adenocarcinoma at the 50th week was 80% in mice treated with both MNU 240 μg/L and H. pylori infection,
whereas the incidence was only 27% in mice treated with only MNU 240 μg/L.
Although H. pylori caused marked expansion of the proliferative zone at the
surface epithelium, H. pylori infection alone caused only chronic atrophic
gastritis without any evidence of carcinomas until 80 weeks. The combination of MNU
and H. pylori infection also resulted in a significantly higher incidence
of gastric adenoma and adenocarcinoma. Significantly higher expressions of proliferating
cell nuclear antigen were noted in the gastric mucosa infected with H. pylori compared to controls.

Conclusions:

These results clearly demonstrated the role of H. pylori infection, rather than direct carcinogens, in promoting gastric carcinogenesis in a mice model.

© 2002 Blackwell Science Asia Pty Ltd
Epidemiology of Helicobacter pylori and peptic ulcer in India
Authors: SINGH VIRENDRA , TRIKHA BANDANA , NAIN CHANDER KANWAL , SINGH KARTAR , VAIPHEI KIM
Published: March 2003

Background

There is a wide variation in the prevalence of peptic ulcer in India both before and since the use of endoscopy. We studied the prevalence of peptic ulcer disease in a community in northern India and its relationship with Helicobacter pylori infection.

Methods

A house‐to‐house survey of residents aged 15 years or above in a sub‐sector of Chandigarh was performed as part of a pilot survey. Subsequently, the study randomly covered all sectors of Chandigarh and we screened 2649 persons. A questionnaire was administered to each subject by trained staff. All individuals with history of peptic ulcer/dyspepsia and an equal number of asymptomatic individuals were asked to attend the outpatient department of the Institute. Diagnosis of peptic ulcer was based on endoscopy or history of previous ulcer surgery.

Results

Two hundred and fifty‐four individuals attended the outpatient department at the Institute and 147 underwent endoscopy, biopsy for histology and rapid urease test, and blood was collected for H. pylori serology. There were 80 symptomatic and 67 asymptomatic individuals. Helicobacter pylori was positive in 38 (56.7%) asymptomatic and 49 (61.3%) symptomatic individuals (P > 0.05). The point prevalence of active peptic ulcer was 3.4% and the lifetime prevalence was 8.8%. The duodenal‐to‐gastric ulcer ratio was 12:1. Helicobacter pylori was present in 11/13 (84.6%) subjects with peptic ulcer. Peptic ulcer was more common in elderly and dyspeptic individuals and there was no effect of sex or socioeconomic status. Helicobacter pylori was associated with age only and did not depend on sex, socioeconomic status or dyspepsia. Of the 38 asymptomatic persons having H. pylori infection, none had active peptic ulcer.

Conclusions

This study demonstrates frequent occurrence of peptic ulcer and H. pylori in this part of the country. Peptic ulcer was more prevalent in the elderly and dyspeptic subjects. Helicobacter pylori was not associated with dyspepsia, and was more prevalent in elderly subjects. There was a low prevalence of peptic ulcer in asymptomatic H. pylori‐infected persons in this community.
Increase in colorectal epithelial apoptotic cells in patients with ulcerative colitis ultimately requiring surgery
Authors: HAGIWARA CHIKARA , TANAKA MASANORI , KUDO HAJIME
Published: March 2003

Abstract
Background and Aims
: Up to one‐third of patients with ulcerative colitis (UC) need to undergo surgery, but the factors that exacerbate inflammation remain unclear. The authors hypothesize that excessive apoptosis reported in active UC may disrupt epithelial defenses and exacerbate the disease. The aim of the present study was to clarify whether apoptotic epithelial cells and histiocytes engulfing them increased in patients with active UC who ultimately require surgery (UC‐S) rather than those receiving medication alone (UC‐M).

Methods:
The study included 29 patients with UC‐S, 35 with UC‐M, 18 with infectious colitis, and 16 healthy controls. Apoptotic cells were detected by terminal deoxynucleotidyl transferase‐mediated dUTP nick end‐labeling (TUNEL). Using biopsy specimens taken from the most severely inflamed rectosigmoid mucosa as determined endoscopically, the apoptotic index (apoptotic cells/epithelial cells,%) and density (per mm2) of lamina propria histiocytes positive for CD68 were then evaluated. Statistical differences were tested with the Mann–Whitney U‐test.

Results
: The apoptotic indices in UC‐M patients were significantly higher than those in controls (P < 0.05) but almost equal to those in infectious colitis patients. In the upper and lower halves of the mucosa, both apoptotic indices and histiocyte densities were significantly higher for UC‐S than in UC‐M (P < 0.01). Ratios of the mean apoptotic index for UC‐S to that for UC‐M exceeded 3.4, while ratios of the mean histiocyte density were limited to approximately 1.6.

Conclusions
: The results suggest that epithelial apoptosis is a non‐specific phenomenon and that an increased number of apoptotic cells exceeding histiocyte phagocytic capacity may play a part in the disruption of epithelial defenses and further accelerate mucosal inflammation.

© 2002 Blackwell Publishing Asia Pty Ltd
Efficacy of Stronger Neo‐Minophagen C compared between two doses administered three times a week on patients with chronic viral hepatitis
Authors: Miyake Kazuhiko , Tango Toshiro , Ota Yasuhiko , Mitamura Keiji , Yoshiba Makoto , Kako Makoto , Hayashi Shigeki , Ikeda Yusei , Hayashida Norimasa , Iwabuchi Shogo , Sato Yuzuru , Tomi Toshiaki , Funaki Naoya , Hashimoto Naoaki , Umeda Tomoyuki , Miyazaki Jun , Tanaka Katsuaki , Endo Yasuo , Suzuki Hiroshi
Published: March 2003

Background:
A daily injection of glycyrrhizin (Stronger Neo‐Minophagen C (SNMC) containing 40 mg glycyrrhizin in a 20 mL ampoule) lowers alanine aminotransferase (ALT) levels in patients with chronic viral hepatitis.

Methods:
The therapeutic effects of intermittent administration of SNMC three times a week for 12 weeks were evaluated and compared between two doses (40 and 100 mL) in a randomized clinical trial.

Results:
Overall, the therapeutic response was better in the 53 patients allocated 100 mL than the 59 who were allocated to have 40 mL SNMC (P = 0.0243). At the completion of SNMC treatment, ALT levels decreased more extensively in the patients on 100 mL than those on 40 mL SNMC (−29 vs−50% in comparison with the baseline value, P = 0.0002). Minor side‐effects occurred in both the patients on 100 mL (20%) and those on 40 mL (12%), but they did not require any therapies.

Conclusions:
Intermittent SNMC would be efficient in suppressing ALT levels in patients with chronic viral hepatitis in a dose‐dependent manner. Taken along with infrequent and very mild side‐effects, long‐term intermittent SNMC would benefit patients with chronic hepatitis by maintaining their quality of life with easier compliance.

© 2002 Blackwell Publishing Asia Pty Ltd
Skip lymph node metastases from a small hepatocellular carcinoma with difficulty in preoperative diagnosis
Authors: UEHARA KEISUKE , HASEGAWA HIROSHI , OGISO SEIJI , SAKAMOTO EIJI , OHIRA SYUSAKU , IGAMI TSUYOSHI , MORI TOSHIHARU
Published: March 2003

Abstract  Recent autopsy studies have clarified the frequency of lymph node (LN) metastases from hepatocellular carcinoma (HCC). However, LN metastases commonly occur in advanced and poorly differentiated HCC and are very rare in small HCC. We encountered a patient with skip LN metastases from a small HCC, 10 mm in diameter. An intra‐abdominal tumor adjoining the duodenum was detected by follow‐up ultrasonography for viral hepatitis C. Computed tomography showed, in addition to the tumor bordering the duodenum, a small low‐density area of the liver (S6), 2 cm in diameter, and a swelling of LN adjacent to the common hepatic artery. Upper gastrointestinal rentogenography revealed a compression of the duodenal second portion without irregularity of the mucosa. Our pre‐operative diagnosis was duodenal gastrointestinal stromal tumor with LN metastasis and HCC or liver metastasis. However, laparotomy proved them to be LN metastases from a small HCC and partial hepatectomy and LN dissection were performed. The patient is doing well 22 months after surgery with no signs of recurrence. In the cases of HCC with LN metastases, the prognosis is generally very poor. However, in small HCC, the clinical characteristics are not fully evaluated. In treatment, we have to keep LN metastases, particularly skip LN metastases, in mind, even in cases of small HCC.
Prediction of relapse after interferon therapy in hepatitis C virus‐infected patients by the use of triple assay
Authors: EL‐AWADY MOSTAFA K , ABDEL RAHMAN MOHAMED M , ISMAIL SOMAYIA M , AMR KHALDA S , OMRAN MOATAZA , MOHAMED SABRY A
Published: March 2003

Background and Methods: In recent years, several tests have been used as predictive factors for relapse after the end of interferon therapy of chronic hepatitis C carriers. Serum hepatitis C virus (HCV)‐RNA has proved insensitive for predicting relapse because more than 50% of patients with undetectable reverse transcription–polymerase chain reaction (RT‐PCR) levels of HCV at the end of therapy become positive for viral RNA after interferon withdrawal. The detection of a minus RNA strand of HCV in liver also failed to efficiently predict relapses in chronic hepatitis patients. Furthermore, the use of a whole blood assay of HCV‐RNA has been controversial. We used a triple assay comprised of RT‐PCR tests for the detection of HCV‐RNA plus strand in serum and peripheral blood mononuclear cells (PBMC), together with testing for the minus strand in PBMC for prediction of relapse after interferon + ribavirin combination therapy in 45 patients with chronic hepatitis C.

Results: The only four patients with a negative triple assay had no relapse 1 year after the end of therapy. In contrast, two‐thirds of the 12 patients who tested negative for viral RNA in serum at the end of therapy relapsed 1 year later.

Conclusion: We concluded that the absence of both minus and plus strands in patients who tested negative for serum PCR may indicate the total eradication of HCV.

© 2003 Blackwell Publishing Asia Pty Ltd
Young people with spina bifida: Transfer from paediatric to adult health care
Authors: SAWYER SM , COLLINS N , BRYAN D , BROWN D , HOPE MA , BOWES G
Published: March 2003

An expectation of health care for young people with disability is that quality coordinated care continues to be available as they pass from the paediatric to the adult health care system. While individual clinicians provide this service well, the widespread absence of coordinated multidisciplinary care for young people with spina bifida in the adult health care system is a major deficiency. This paper describes the planning and implementation that underpinned the transfer of 10 young people with spina bifida from a paediatric to an adult service. The range of structural, financial and ‘cultural’ barriers that need to be overcome before patients can be successfully transferred is highlighted; lessons learned from this model may serve to facilitate the development of other transfer services.
The musculoskeletal complications of cystic fibrosis
Authors: MASSIE RJH , TOWNS SJ , BERNARD E , CHAITOW J , HOWMAN‐GILES R , ASPEREN PP VAN
Published: March 2003

Objective:

To determine the spectrum of musculoskeletal complications of cystic fibrosis (CF) in a paediatric population in Australia.

Method:

Clinical assessment followed by serology and bone scan on patients attending a specialized CF clinic.

Results:

Of 125 patients studied, 21 had musculoskeletal complications, 17 attributable to CF. Eleven had joint involvement (six hypertrophic pulmonary osteoarthropathy (HPOA)), one CF arthropathy, two ciprofloxacin induced arthralgia, one joint contracture following long‐line placement, one chest infection associated arthralgia), four kyphosis (two also with HPOA) and two thoracic deformity. HPOA was associated with older age, lower average pulmonary function and lower average Shwachman score. Three patients with HPOA died within 12 months of reporting symptoms. Kyphosis was also associated with older age and lower pulmonary function.

Conclusion:

Increasing age with deteriorating clinical and pulmonary function were associated with a higher incidence of musculoskeletal involvement. The development of symptomatic HPOA is a marker of poor prognosis.
The athlete’s heart: Is this an entity in paediatrics?
Authors: QUEK SC , NG KS , TI TY , LOW PS
Published: March 2003

A case report of a boy who demonstrates features of an athlete’s heart, associated with dilatation of the coronary artery, is presented. The importance of distinguishing this benign condition from pathologic causes such as cardiomyopathy, and risk of sudden death in these athletes is discussed.
Surfactant protein B deficiency: Clinical, histological and molecular evaluation
Authors: Williams GD , Christodoulou J , Stack J , Symons P , Wert SE , Murrell MJ , Nogee And LM
Published: March 2003

Abstract: Congenital alveolar proteinosis due to surfactant protein B deficiency is an inherited disease which results in severe respiratory failure in term infants soon after birth. The pathophysiologic basis of this disease is now known to be an inability to synthesise adequate quantities of normally functioning surfactant protein B. We report a male infant with fatal respiratory failure of neonatal onset, and histopathological features typical of those seen in congenital alveolar proteinosis. Molecular analysis of genomic DNA revealed two mutations, the ‘common’ 121ins2 mutation in exon 4, and a novel 2bp frameshift mutation in exon 5. We believe this is the first Australian case of surfactant protein B deficiency confirmed by molecular analysis.
Methylxanthines and sensorineural outcome at 14 years in children < 1501 g birthweight
Authors: Davis PG , Doyle LW , Rickards AL , Kelly EA , Ford GW , Davis NM , Callanan C
Published: March 2003

Objectives: Methylxanthines, including theophylline, have been used extensively and successfully to treat apnoea in preterm infants. However, long‐term consequences of such therapy are largely unknown. The aim of this study was to determine the relationship between theophylline therapy and outcome at 14 years of age in surviving preterm children of birthweight < 1501 g.

Methodology: The subjects of this study were 154 consecutive survivors with birthweights < 1501 g born from 1 October 1980 to 31 March 1982; 130 (84.4%) were assessed at 14 years of age. Outcomes included motor function, psychological test scores, and growth.

Results: Of the 130 children assessed, 69 (53.1%) had been exposed to theophylline; 13.0% had cerebral palsy, significantly higher than 1.6% in the 61 children not exposed to theophylline (P < 0.02). This difference remained statistically significant after adjusting for potential confounding variables including the presence of cerebroventricular haemorrhage. In contrast, after adjusting for known confounding variables, children who had received theophylline achieved higher psychological test scores. There was no association between theophylline therapy and growth.

Conclusions: Theophylline therapy in the newborn period is associated with some evidence of harmful, but also helpful sensorineural effects at 14 years of age.
Blunt abdominal trauma in children
Authors: Rance CH , Singh SJ , Kimble R
Published: March 2003

Abstract: Blunt abdominal trauma is the commonest cause of intra‐abdominal injuries in children. The use of computerized axial tomography and non‐operative management of haemoperitoneum are two significant developments in the last two decades in the management of blunt abdominal trauma in children. The concept of non‐operative management was introduced in late 1979 and wherever possible remains the optimum treatment. Computerized tomography scan for paediatric abdominal trauma was first described in 1980 and remains the investigation of choice. There is no substitute, however, for a good history, astute physical examination, and strict adherence to the principles of primary and secondary survey, prompt resuscitation, vigilant monitoring and repeated evaluation.
Early motor development of blind children
Authors: Levtzion‐Korach O , Tennenbaum A , Schnitzer R , Ornoy A
Published: March 2003

Objectives: The purpose of this study was to assess the characteristic motor developmental pattern in blind children in Israel.

Methodology: The study compared the developmental data concerning 10 motor skills of 40 blind children to a control group of sighted children and to the motor developmental milestones of the Bayley Developmental Scale and the Revised Denver Developmental Screening Test.

Results: The motor development of blind children was delayed, the delay being significant in all 10 motor skills that were examined. This delay emphasizes the major importance of vision as a sensory input modality for the process of sensory– motor development.

Conclusion: An adequate stimulating environment and proper parental handling could potentially shorten the motor developmental delay but probably not eliminate it entirely.
Early cranial ultrasound changes as predictors of outcome during first year of life in term infants with perinatal asphyxia
Authors: Boo Ny , Chandran V , Zulfiqar Ma , Zamratol Sm , Nyein Mk , Haliza Ms , Lye Ms
Published: March 2003

Objectives: To identify the types of early cranial ultrasound changes that were significant predictors of adverse outcome during the first year of life in asphyxiated term infants.

Methodology: This was a prospective cohort study. Shortly after birth, cranial ultrasonography was carried out via the anterior fontanelles of 70 normal control infants and 104 asphyxiated infants with a history of fetal distress and Apgar scores of less than 6 at 1 and 5 min of life, or requiring endotracheal intubation and manual intermittent positive pressure ventilation for at least 5 min after birth. Neurodevelopmental assessment was carried out on the survivors at 1 year of age.

Results: Abnormal cranial ultrasound changes were detected in a significantly higher proportion (79.8%, or n = 83) of asphyxiated infants than controls (39.5%, or n = 30) (P < 0.0001). However, logistic regression analysis showed that only three factors were significantly associated with adverse outcome at 1 year of life among the asphyxiated infants. These were: (i) decreasing birthweight (for every additional gram of increase in birthweight, adjusted odds ratio (OR) = 0.999, 95% confidence interval (CI) 0.998, 1.000; P = 0.047); (ii) a history of receiving ventilatory support during the neonatal period (adjusted OR = 8.3; 95%CI 2.4, 28.9; P = 0.0009); and (iii) hypoxic‐ischaemic encephalopathy stage 2 or 3 (adjusted OR = 5.8; 95%CI 1.8, 18.6; P = 0.003). None of the early cranial ultrasound changes was a significant predictor.

Conclusions: Early cranial ultrasound findings, although common in asphyxiated infants, were not significant predictors of adverse outcome during the first year of life in asphyxiated term infants.
Hepatic glycogenosis: Reversible hepatomegaly in type 1 diabetes
Authors: Munns Cfj , McCrossin Rb , Thomsett Mj , Batch J
Published: March 2003

Objective: To describe the aetiology, clinical features and appropriate treatment for hepatic glycogenosis in poorly controlled type 1 diabetes.

Methods: A review of three adolescents with poor diabetes control, hepatomegaly and elevated serum liver transaminase concentrations.

Results: Symptoms included abdominal pain, anorexia, nausea and vomiting. All had tender hepatomegaly; two had splenomegaly. Liver biopsy was performed on two patients. Histology revealed hepatic glycogenosis in both; one also demonstrated macrovesicular steatosis. With improved glycaemic control, all three showed resolution of their symptoms, organomegaly and elevated serum liver transaminase concentrations.

Conclusions: Insulin‐reversible hepatic glycogenosis is the most common cause of hepatomegaly and raised serum liver transaminase concentrations in children and adolescents with type 1 diabetes. Having excluded other causes of hepatic dysfunction, a 4 week therapeutic trial of improved glycaemic control is recommended prior to more invasive investigations.
Quality of life of mothers and families caring for preterm infants requiring home oxygen therapy: A brief report
Authors: McLean A , Townsend A , Clark J , Sawyer Mg , Baghurst P , Haslam R , Whaites L
Published: March 2003

Objective: To investigate the impact on mothers and families of caring for a premature infant receiving home oxygen therapy (HOT).

Methods: Standard questionnaires were used to compare the functioning of mothers and families of 10 premature infants receiving HOT, 10 premature infants who were discharged from hospital on HOT but who no longer required it and 20 premature infants who had never required treatment with HOT.

Results: After adjustment for gestational age, chronological age, birthweight and place of residence (urban/rural), the care required by premature infants receiving HOT had a significantly greater impact on their families than the care of infants not receiving HOT. Mothers of premature infants receiving HOT reported significantly less vitality and more mental health problems than mothers of infants not receiving HOT.

Conclusions: The use of HOT for premature infants may have a significant adverse impact on their mothers and families.
Use of intravenous hydrocortisone in Henoch–Schonlein purpura
Authors: Leung SP
Published: March 2003

Abstract: Henoch–Schonlein purpura should be considered in children with severe abdominal pain that is unresponsive to conventional treatment when no apparent cause is found on investigation. Such children may respond dramatically to a trial of intravenous corticosteroid and, hence, may avoid unnecessary investigations and laparotomy.
Participation in research: Informed consent, motivation and influence
Authors: Hayman RM , Taylor BJ , Peart NS , Galland BC , Sayers RM
Published: March 2003

Objective: To investigate the process and quality of informed consent, motivation and influence in parents who were invited to enrol their baby in a research project.

Methodology: A mixed quantitative/qualitative questionnaire was sent to a cohort invited to participate in a physiological research project on sudden infant death syndrome (SIDS) at the Dunedin Public Hospital, Dunedin, New Zealand. Separate questionnaires were used for parents who participated (94) and those who declined to participate (103). Response rates were 69% and 47%, respectively.

Results: All consenting parents felt they understood the purpose and procedure of the study. The majority (90%) thought the information about the study was very good; 6.5% felt more detail was required. Eighty‐five per cent found the verbal explanation the most useful source of information. All participated for altruistic reasons such as to aid SIDS research. Although 27% had concerns about safety of the tests, after the tests all responders felt happy with the safety of the tests. Inconvenience was the main reason (53%) for declining to participate. Twenty‐eight per cent of declining parents were concerned about the safety of the tests.

Conclusion: Of those who responded to the questionnaire, the process for obtaining informed consent in the SIDS studies was satisfactory. Parents’ motives for participating were mostly altruistic. The role of recall bias and selection bias may make the implications of this study unclear.
Perforation complications of percutaneous central venous catheters in very low birthweight infants
Authors: Leipälä JA , Petäjä J , Fellman V
Published: March 2003

Objective: To prospectively survey perforation complications of consecutively inserted percutaneous central venous catheters (PCVC) in very low birthweight (VLBW) infants over a 2 year period.

Methodology and Results: Three serious perforation complications were encountered in a series of 100 consecutive PCVC. One infant (birthweight 685 g) developed pericardial effusion and fatal cardiac tamponade during the use of a polyurethane PCVC. At autopsy, the pericardial sac contained 8 mL fluid with a glucose concentration of 109 mmol/L and the catheter tip was embedded in the right ventricular wall. The second infant (birthweight 1380 g) showed pleural effusion and transient immobility of the right diaphragmatic leaf after perforation of a similar PCVC into the right pleural cavity. The third perforation, causing subcutaneous oedema, occurred in a 655 g infant who had a silastic PCVC.

Conclusions: The data suggest a 3% incidence for PCVC‐associated symptomatic perforation complications and a 1% incidence for fatal perforations, despite a policy of careful placement. The data also indicate that perforation complications occur regardless of the size or material of the PCVC. Proper visualization of the PCVC and vigilant attention to its location is required to prevent these rare but potentially fatal complications.
Leukaemia presenting as respiratory distress in a child with asthma
Authors: Heath JA
Published: March 2003

Abstract: Asthma is the most common reason for children to present to an emergency department complaining of shortness of breath. The diagnosis, based on one or more of a positive history of atopy, physical examination findings consistent with reactive airways disease, and a clinical response to bronchodilator therapy is usually straightforward. It is important however, to consider other diagnoses, particularly when patients present differently from that which has previously been documented and/or have unexplained physical findings.
Intracranial venous thrombosis and pulmonary embolism with antiphospholipid syndrome in systemic lupus erythematosus
Authors: Yuen SF , Lau KF , Steinberg AW , Grattan‐Smith PJ , Hodson EM
Published: March 2003

Abstract: The presence of antiphospholipid antibodies is associated with arterial and venous thrombosis. A 14‐year‐old girl, with systemic lupus erythematosus (SLE), developed headache and cough and was found to have intracranial venous sinus thrombosis with secondary pulmonary embolism associated with antiphospholipid syndrome. Clinical and radiological improvement occurred with anticoagulation therapy. Because SLE is commonly associated with antiphospholipid antibodies, thromboembolic events should be considered in the differential diagnosis of both cough and headache in children with SLE.
Risk factors for small‐for‐gestational‐age babies: The Auckland Birthweight Collaborative Study
Authors: Thompson JMD , Clark PM , Robinson E , Becroft DMO , Pattison NS , Glavish N , Pryor JE , Rees K , Mitchell EA
Published: March 2003

Objective: This case‐control study determined whether internationally recognized risk factors for small‐for‐gestational‐age (SGA) term babies were applicable in New Zealand.

Methodology: All babies were born at 37 or more completed weeks of gestation in one of three hospitals in Auckland. Cases weighed less than the sex specific 10th percentile for gestational age at birth, and controls (appropriate‐for‐gestational‐age (AGA)) were a random selection of heavier babies. Information was collected by maternal interview and from obstetric databases.

Results: Information from 1714 completed interviews (844 SGA and 870 AGA) was available for analysis. Computerized obstetric records were available for 1691 of the 1701 women who consented to such access. In a multivariate analysis allowing for sex, gestational age at birth, social class and other potential confounders, mothers who smoked had a significantly increased risk of an SGA baby (adjusted OR 2.41; 95% CI 1.78–;3.28), as did primiparous mothers (adjusted OR 1.34; 95% CI 1.03–;1.73), mothers of Indian ethnicity (adjusted OR 3.22; 95% CI 1.95–;5.30), women with pre‐eclamptic toxaemia (adjusted OR 2.42; 95% CI 1.08–;5.40) and those with pre‐existing hypertension toxaemia (adjusted OR 5.49; 95% CI 1.81–;16.71). Mothers of SGA infants were shorter (P < 0.001) and reported lower prepregnancy body weights (P < 0.001) than mothers of AGA infants. The population attributable fraction for smoking suggests that up to 18% of SGA infants born in the ABC Study could be related to maternal smoking.

Conclusions: Risk factors associated with SGA births in other countries are also important in New Zealand. Smoking in pregnancy is an important and potentially modifiable behaviour, and efforts to decrease the number of women who smoke during pregnancy should be encouraged.
Transcatheter patent ductus arteriosus occlusion: Evolution of techniques and results from the 1990s
Authors: O'Donnell C , Neutze JM , Skinner JR , Wilson NJ
Published: March 2003

Objective: To review the evolution of transcatheter patent ductus arteriosus (PDA) occlusion techniques and results.

Methods: A single institution, retrospective review including all patients with intention to close a PDA from 1991 to 1998, with no exclusions.

Results: Rashkind occluder (n = 65), sideris double‐button (n = 6), Cook detachable coil (n = 28) and Amplatzer ductal occluder (n = 4) were used. Successful implantation occurred in 99 of 103 patients. There was a need for a second transcatheter procedure to close residual ductal shunting in 12% of patients: Rashkind umbrellas (n = 8), double‐button (n = 1), coils (n = 3). Eight patients (8%) required surgery, including 4 of 6 patients with the double‐button occluder.

Conclusions: The Rashkind occluder and the Sideris double‐button device both had an unacceptably high rate of residual shunts requiring a second transcatheter procedure or surgical closure. Detachable coils and the Amplatzer ductal occluder have become the current technology of choice for transcatheter PDA closure with high success rates.
Use of a behavioural programme in the first 3 months to prevent infant crying and sleeping problems
Authors: St James‐Roberts I , Gillham P
Published: March 2003

Objective: To assess the effectiveness of a behavioural programme introduced in the first 3 months of age in preventing infant crying and sleeping problems. Two issues were addressed: (i) which elements of the behavioural programme would parents implement; and (ii) whether the behavioural programme was more effective in reducing infant crying and encouraging night‐time sleeping than an educational intervention or the routine services.

Methodology: Mothers and newborns were assigned at random to the behavioural programme (n = 205), educational intervention (n = 202), or control (n = 203) group. Behaviour diaries kept before randomization and at 3, 6, 9 and 12 weeks of age were used to measure implementation of the interventions and infant behaviour, including crying and sleeping. Crying and sleeping problems were followed up using questionnaire measures at 9 months of age.

Results: The educational intervention did not change parental care behaviour. One element of the behavioural programme, a focal feed between 10 PM and midnight, was not implemented. A second element, stretching of interfeed intervals after 3 weeks of age, was implemented initially, but not maintained at older ages. The third element, which asked parents to emphasise day and night differences in the environment, and to settle their babies in the cot and minimise interaction at night, was carried out by more parents in the behavioural group than in the other groups. This led to an increase of around 10% in the number of babies who slept for 5 or more hours at night (a definition of sleeping through the night) at 12 weeks of age. Fewer behavioural programme parents sought help for crying and sleeping problems between 3 and 9 months of age.

Conclusion: The behavioural programme produced a modest increase in the number of infants who slept through the night by 12 weeks of age. The results are discussed in relation to other findings, which bear on the programme's adoption for routine health‐care policy and practice.
Pyridoxine‐dependent seizures: A case report and a critical review of the literature
Authors: Gupta VK , Mishra D , Mathur I , Singh KK
Published: March 2003

Abstract: Pyridoxine‐dependent seizures are a recognized, although rare, cause of intractable seizures in neonates. Patients with this autosomal recessive disorder have recurrent seizures that are resistant to conventional anticonvulsants but respond dramatically to intravenous administration of pyridoxine. Life‐long supplementation with pyridoxine is required to prevent seizure recurrence. In the absence of a biological marker for the disease, clinical diagnosis is often delayed and severe neurological sequelae are common. Herein, we report on the clinical course of a neonate with pyridoxine‐dependent seizures. Delayed normalization of the electroencephalogram and a normal developmental outcome (at 15 months of age) on a dose of 10 mg/kg pyridoxine are distinctive features of the present case. We also review recent clinical observations and neurochemical studies that have added to our knowledge of this disorder.
Use of complementary and alternative therapies and probiotic agents by children attending gastroenterology outpatient clinics
Authors: Day AS
Published: March 2003

Objectives:  Complementary and alternative medicines (CAM) and probiotic therapies appear to be increasingly accepted and used. This questionnaire‐based study aimed to ascertain the frequency of use and the acceptance of these therapies by children attending outpatient gastroenterology clinics.

Methods:  Parents accompanying children to their appointments were asked to complete a questionnaire in order to determine usage of probiotic and alternative therapies. Questions also ascertained relevant background information and parental acceptance of alternative therapies.

Results:  Ninety‐two questionnaires were completed. The ages of the children varied from 6 months to 16 years (mean ± SD; 6.5 ± 4.3 years) and they had been prescribed an average of 1.7 ± 1.3 (range 0−6) conventional medications. Thirty‐three children (35.9%) were taking CAM and 98.6% of parents answered that they would be prepared to administer CAM to their child. Symptomatic improvements were attributed to CAM by the parents of 24 out of 33 children given these therapies. In addition, probiotic therapies were utilized by 23.8% of children, and 93.0% of parents would administer probiotic agents if recommended for their child's condition.

Conclusion:  Complementary and alternative medicines and probiotic therapies are used frequently by children attending gastroenterology clinics and are accepted widely by their parents.