Association of hiatus hernia with asplenia syndrome.
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Hernia, Hiatal↗
Biomedical subjects
M H Chang
Publications and source records attributed to M H Chang.
Resting tremor only: a variant of Parkinson's disease or of essential tremor.
Resting tremor is one of the characteristic features of Parkinson's disease. However, there are a number of patients who typically have resting tremor alone for at least 5 years without development of other parkinsonian signs or symptoms. The etiology of an isolated resting tremor is still obscure. Recently, positron emission tomography was used to study these patients with isolated resting tremor, and demonstrated a markedly decreased striatal uptake of fluoro-dopa to the range of Parkinson's disease. These findings suggested the existence of a separate subtype, namely, tremulous Parkinson's disease with a manifestation of resting tremor alone. In order to confirm the existence of this subgroup of tremulous Parkinson's disease and further investigate its morphological changes and the usefulness of magnetic resonance imaging, we collected 5 patients who typically have resting tremor for at least 8 years in the absence of other features of Parkinson's disease. MRI was performed and the results of the images showed typical findings of Parkinson's disease with smudging or decreased distance between substantia nigra and red nucleus. Quantitative analysis also demonstrated a significant decrease of the above-noted distance when the resting tremor group was compared to the essential tremor group. Therefore, patients with an isolated resting tremor can have morphological abnormalities in addition to functional disturbances shown by positron emission tomography. To our knowledge, this is the first paper to report that resting tremor is a variant of Parkinson's disease rather than essential tremor, by using a double-blind method, with magnetic resonance imaging to support.
Aged↗
A follow-up study of combined vaccination with plasma-derived and recombinant hepatitis B vaccines in infants.
This study was aimed to evaluate the efficacy and immunogenicity of combined hepatitis B vaccination with plasma-derived vaccine (PDV) and recombinant vaccine (RV). A total of 329 infants was recruited, including 224 high-risk infants born to hepatitis B e antigen-positive mothers and 105 low-risk infants born to hepatitis B e antigen-negative mothers. The high-risk infants received four doses of hepatitis B vaccine at 0, 1, 2 and 12 months of age with five different schedules. Group A1 and A2 infants were vaccinated with PDV as the first dose and RV (SB vaccine for group A1, MSD vaccine for group A2) as the remaining three doses. Group B1 and group B2 infants were vaccinated with PDV as the first two doses and RV (SB vaccine for group B1, MSD vaccine for group B2) as the remaining two doses. Group C infants received four doses of PDV. Low-risk infants were vaccinated with PDV at birth, and RV at 1 and 6 months of age (group D1, using SB vaccine; group D2, using MSD vaccine). At completion of vaccination schedules, 20 of 224 high-risk infants (9%) were positive for hepatitis B surface antigen. The overall protective efficacy was 90%. Hepatitis B surface antibody (anti-HBs)-positive rate ranged between 94 and 100% among the remaining infants. The protective efficacy and immunogenicity were similar among groups except that the mean level of anti-HBs in group C, D1 and D2 infants tended to be lower than that of the other four groups. To ensure an optimal immune response, four doses of vaccine are recommended in high-risk infants when two types of vaccine are to be used in combination.
Child, Preschool↗
Absence of infection in breast-fed infants born to hepatitis C virus-infected mothers.
The role of breast-feeding in perinatal transmission of hepatitis C virus (HCV) was explored in 15 HCV-infected mothers and their infants. The 15 carrier mothers had anti-HCV titers ranging from 1:80 to 1:40,000 and also had HCV-ribonucleic acid with concentrations ranging from 10(4) to 2.5 x 10(8) copies/ml. Both anti-HCV antibody and HCV-ribonucleic acid were present in colostral samples in much lower levels, but none of the 11 breast-fed infants had evidence of HCV infection for up to 1 year of age. Thus breast-feeding seems safe for these infants.
Breast Feeding↗
Long-term efficacy of recombinant hepatitis B vaccine and risk of natural infection in infants born to mothers with hepatitis B e antigen.
To evaluate the long-term protection afforded by the vaccine, recombinant hepatitis B (HB) vaccine was given to 171 infants born to hepatitis B e antigen-positive carrier mothers. Group A (53 infants) and group B (57 infants) received four doses of HB vaccine at birth and at 1, 2, and 12 months of age, with a dose of 20 micrograms in group A and 10 micrograms in group B. Group C (61 infants) received three 20 micrograms doses of HB vaccine at birth and at 1 and 6 months of age. These children were followed up annually up to 5 years of age. Six children (4%) became HB carriers before 1 year of age, and the carrier state persisted to the end of follow-up. The overall seropositive rate of HB surface antibody (anti-HBs) dropped from 99% at 1 year of age to 83% at 5 years of age. Among 548 serum pairs taken at 1-year intervals from children negative for HB surface antigen (HBsAg), a fourfold rise of anti-HBs titer was noted in 58 (11%) and a 10-fold rise of anti-HBs was noted in 17 (3%). Maternal HB core antibody disappeared in most children (151/152, 99%) before 2 years of age. Natural infections, as judged by persistence or reappearance of HB core antibody, occurred in 19 of 163 (12%) HBsAg-negative children. None of these episodes was associated with HBsAg positivity. We conclude that the long-term protection afforded by recombinant HB vaccine is satisfactory and that a further booster dose before 5 years of age is not necessary.
Biomarkers↗
Extrauterine twin-twin transfusion syndrome after birth in conjoined twins.
Cross-circulation of conjoined twins in utero has been demonstrated and the extent of its severity has varied. Significant 'extrauterine' twin-twin transfusion syndrome after birth, however, caused by an unbalanced shunting within the circulation has not been reported. In a case of omphalopagus conjoined twins complicated by hypovolemia in one twin and hypervolemia in the other, we demonstrated by color Doppler imaging an aberrant vessel from the hepatic artery of one twin to the hepatic vein of the other. Unbalanced shunting through this aberrant vessel was suspected and urgent separation of the conjoined twins was undertaken. Circulatory changes after birth leading to 'extrauterine twin-twin transfusion syndrome' are discussed. A possible explanation of the pathophysiology of intrauterine twin-twin transfusion syndrome is also given.
Adult↗
Glucose-6-phosphatase gene G327A mutation is common in Chinese patients with glycogen storage disease type Ia.
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Asian People↗
Precore mutant of hepatitis B virus in childhood fulminant hepatitis B: an infrequent association.
Serum HBV DNA from infants and children with fulminant hepatitis B (FHB) or acute self-limiting hepatitis B (AHB) and patients believed to be contamination sources was amplified by polymerase chain reaction (PCR) followed by direct sequencing of the precore region. A precore mutation from G to A at nucleotide 1896 was found in 5 of 14 FHB patients and in 3 of 10 AHB patients. Among the 10 infants who developed hepatitis during the first 6 months of age, the precore mutation was detected in only 2 of 7 with FHB and in 1 of 3 with AHB. In 1 infant with FHB, a shift from wild type to precore mutant predominance occurred in the serum virus population during the incubation period. Thus, the precore mutation is neither necessary nor sufficient to cause FHB in childhood, although its contributory role cannot be excluded. Factors other than precore mutations may be important in the pathogenesis of FHB.
Acute Disease↗
The association of syndromic paucity of the interlobular bile ducts and congenital mechanical obstruction of the small intestine.
Three patients with congenital mechanical intestinal obstruction and features of syndromic paucity of the interlobular bile ducts (SPILBD) are presented. Two of them have siblings with syndromic paucity of the interlobular bile ducts. Two patients had jejunoileal atresia, while the other had a meconium plug with intestinal perforation. All subjects underwent surgery for intestinal obstruction within the first 24 h of life. Progressive disappearance of the bile ductules was observed in one case. We believe that an insult such as vascular insufficiency to both bile ductal epithelium and the small intestine may be the cause of this congenital disorder.
Abnormalities, Multiple↗
Hydrosonography in the evaluation of colorectal polyps.
Prewarmed saline enemas and transabdominal ultrasound (hydrosonography) were used to evaluate 17 consecutive children with rectal bleeding before colonoscopy. Twelve patients with polyps were identified (10 by ultrasound, 10 by endoscopy): these included multiple hyperplastic polyps (1), multiple polyps (1), solitary polyps (9), and pseudopolyps (1). Ultrasound identified 11 polyps in 10 patients, missing two patients with small polyps less than 0.5 cm in diameter. The polyps were hyperechoic ovoid masses fixed to the colonic wall, with a stalk (7), submucosal infolding (5), and intraluminal floating (5). There was one false positive. Colonoscopy was refused by one patient and failed to reach beyond the distal sigmoid in another following previous surgery for malrotation. Colonoscopy was superior in identifying finer mucosal detail (colitis, ulcers, proctitis, anal fissure) and in detecting smaller polyps (sessile polyps, hyperplastic polyps). Hydrosonography of the colon is a simple, relatively non-invasive procedure that provides an alternative, radiation-free examination of the whole colon before colonoscopy. It is complementary to colonoscopy in the management of rectal bleeding in children.
Child↗
Studies on new catechol containing cephalosporins. I. Synthesis and structure-activity relationships of cephalosporins having a catechol moiety at the C-3 position.
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Catechols↗
Studies on new catechol containing cephalosporins. II. Synthesis and structure-activity relationships of cephalosporins having a catechol moiety at the C-7 position.
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Catechols↗
The significance of spontaneous hepatitis B e antigen seroconversion in childhood: with special emphasis on the clearance of hepatitis B e antigen before 3 years of age.
To investigate the significance of spontaneous hepatitis B e antigen (HBeAg) seroconversion during childhood, 415 hepatitis B surface antigen (HBsAg) carrier children (ages 0 to 15 years) were prospectively followed for 7.1 +/- 2.9 years. Hepatitis B virus (HBV) markers and liver function profiles of each child were tested at least once every 6 months. Among them, 50 were initially anti-HBe positive and 140 seroconverted from HBeAg to anti-HBe during follow-up. Before HBeAg seroconversion, jaundice occurred in 9 and alanine transaminase (ALT) activities elevated in 99 of the 140 seroconverters. Serum ALT returned to normal in all patients within 1 to 5 years of seroconversion. Six had reelevated ALT later after seroconversion. Only 7 (9.7%) of the 72 carrier infants seroconverted before 3 years of age. The peak ALT levels in five of them exceeded 100 IU/L, and two had jaundice before HBeAg seroconversion. One of the early seroconverters developed hepatocellular carcinoma (HCC) at 11 years of age, although his liver function profiles remained normal after HBeAg seroconversion. Liver biopsy specimens from 30 children during the anti-HBe-positive stag e showed inactive cirrhosis in 2 (including one with HCC), chronic hepatitis with marked fibrosis in 1, mild activity and moderate fibrosis in 2, mild activity and mild fibrosis in 9, and minimal histologic changes in the remaining 16. Although most will achieve a normalization of ALT and inactive liver histologic changes, the seroconversion of HBsAg carrier children from HBeAg to anti-HBe is not necessarily an indicator of favorable prognosis; a small proportion of children will develop cirrhosis or even HCC.
Adolescent↗
Percutaneous endoscopic gastrostomy in infants.
After a trial of 1 month of nasogastric tube feeding, three infants (aged 5-14 mo) underwent percutaneous endoscopic gastrostomy (PEG) to enable long-term enteral feeding. The Ponsky-pull technique was employed for PEG insertion. In two patients, the procedure was complicated by superficial wound infection at the cutaneous exists of the PEG tubes: one with Pseudomonas sp and the other staphylococcal colonization. Both responded well to antibiotic treatment. All patients obtained good nutritional support. The preliminary experience reported here suggests that, even in infants, PEG is a safe and easy nonsurgical method of enteral feeding.
Endoscopy↗
Analysis of integrated hepatitis B virus DNA and flanking cellular sequences in a childhood hepatocellular carcinoma.
The DNA of tumor tissue K1 obtained at autopsy from a case of hepatocellular carcinoma (HCC) in a 9-year-old boy contained integrated hepatitis B virus (HBV) DNA at a single site in the chromosome (case 2, Chang et al.: Hepatology 13:316-320, 1991). To characterize further the integrated viral DNA sequences, a genomic library of the K1 DNA was constructed in the lambda L47.1 vector. One phage clone, designated KTM-1, containing integrated HBV DNA and cellular flanking sequences was obtained from this library. The restriction map and DNA sequence of this clone showed that the integrated HBV DNA was partially deleted and rearranged. The most conserved viral DNA sequences were surface and X genes and arranged in the opposite orientation. The viral core gene was not present. Using chloramphenicol acetyltransferase (CAT) assay, the C-terminal truncated X open reading frame was demonstrated to retain its trans-activating ability. The result suggested that the functional integrated X gene may play a role in hepatocarcinogenesis. The study also showed that the right cellular flanking sequences were human alphoid repetitive sequences.
Base Sequence↗
Gastrointestinal bleeding and paraparesis in blue rubber bleb nevus syndrome.
Blue rubber bleb nevus syndrome (BRBNS), which consists of cutaneous and visceral hemangiomas, is a rare disease. Complications such as gastrointestinal (GI) bleeding, anemia and coagulopathy have been documented. We report a patient with BRBNS who presented with acute paraparesis in addition to GI bleeding and coagulopathy.
Blood Coagulation Disorders↗
Amniocentesis in mothers who are hepatitis B virus carriers does not expose the infant to an increased risk of hepatitis B virus infection.
Sixty-seven pairs of mothers with hepatitis B virus (HBV) surface antigen (HBsAg) and their infants were divided into two study groups to determine the effect of amniocentesis on intrauterine HBV infection. In the first study group (35 pairs), the infant's HBsAg status in cord blood was studied and the results were compared with those obtained in the cord blood from 65 infants born to HBsAg-positive women who did not have an amniocentesis. In the second study group (32 pairs), the HBV status of the infants was studied at the age of three months to five years and compared with the HBV status of 3,454 infants in the National HBV Prevention Program. In the first study group, one sample (2.9%) was weakly positive for HBsAg; while in the first control group, two (3.1%) were positive. In the second study group, three (10%) infants were positive for HBsAg. The failure rates of immunoprophylaxis in the second study and control groups were similar (9.4% vs 11% for HBsAg carrier mothers; 30% vs 14% for HBe antigen-positive carrier mothers). This suggested that genetic amniocentesis did not increase the risk of intrauterine HBV infection.
Adult↗
Response to supplementary vaccination with recombinant or plasma hepatitis B vaccine in healthy non-responding children.
Fifty-three children who failed to respond to four doses of plasma hepatitis B (HB) vaccine (anti-HBs titre < 10 IU l-1) were divided into two groups and received revaccination with either three doses of recombinant HB vaccine (10 micrograms/dose, by 0, 1, 6 month schedule; group A) or two additional doses of plasma HB vaccine (5 micrograms/dose, by 0, 1 month schedule; group B) respectively. Thirty-two vaccinees in group A had a response rate (with anti-HBs > 10 IU l-1) of 53.1% (17/32), 87.5% (28/32), and 100% (32/32) after first, second and third doses of the vaccine respectively. Twenty-one vaccinees in group B had a response rate of 61.9% (13/21) after two additional doses of plasma vaccine. High anti-HBs titres (> 1000 IU l-1) were noted in 50% of the vaccinees in group A after three doses of vaccine. Comparing anti-HBs response between group A and group B after two additional doses of HB vaccine, group A had a higher anti-HBs titre (geometric mean titre 104.7 IU l-1 versus 75.9 IU l-1) along with a better seroconversion rate (87.5 versus 61.9%). However, the differences in vaccine dose between the two groups may also be a contributory factor. Our findings indicate that three doses of recombinant HB vaccine were invariably effective in eliciting a good immune response in previous non-responders to the four doses of plasma HB vaccine. Therefore, it is speculated that these young vaccinees who did not respond to four doses of plasma HB vaccine may not be real non-responders, but hyporesponders.
Child↗