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Biomedical subjects

S J Lin

Publications and source records attributed to S J Lin.

At least 199 records · Page 11Linked to original sources
PubMedDate not supplied

Hepatobiliary involvement of Henoch-Schönlein purpura in children.

Hepatobiliary manifestation and its evolution in children with Henoch-Schönlein purpura (HSP) had been scarcely reported. Over a ten-year period between June 1988 and November 1998, medical charts of 225 children with the diagnosis of HSP encountered at Chang Gung Children's Hospital were reviewed. Those with the evidence of hepatobiliary involvement were enrolled in the study. The patients with hepatobiliary involvement were defined by having an elevated serum alanine transaminase (ALT) or gamma-glutamyl transferase (GGT), and the presence of abnormal sonographic findings involving liver, biliary system, and gallbladder. The patients included 9 boys and 11 girls with range of age from 3 to 11 years. The mean age of these patients was 6.5 +/- 3.7 years. Preceding upper respiratory tract infection was common in these patients (35%). The main clinical manifestation were distinct from typical abdominal symptoms of HSP, and included right upper quadrant pain (80%), nausea (45%), lethargy (20%), and vomiting (15%). Elevated serum ALT was noted in 15 cases (75%) and GGT in 6 cases (30%). Abdominal ultrasonography revealed hepatomegaly (15/20, 75%), and gallbladder wall thickening (5/20, 25%). No specific laboratory parameters were correlated with the hepatobiliary manifestation of HSP. Fourteen children (70%) received steroid therapy and disappearance of symptoms and sonographic resolution of hepatobiliary lesion was noted within 3-7 days of steroid therapy. All patients recovered completely except for one patient with hepatobiliary recurrence manifested as purpuric rash, abdominal pain, elevated GGT, and gallbladder wall thickening during 2-year follow-up.

Adrenal Cortex Hormones↗
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Papillary cystic neoplasm of the pancreas in children: report of three cases.

Papillary cystic neoplasm of the pancreas is very rare in children. There were only 35 pediatric cases reported in the literature. We herein three children who had papillary cystic neoplasm of pancreas. They were female teenagers, and were pathologically diagnosed. The major presenting symptoms were abdominal pain and abdominal mass. Serum tumor markers of these patients showed normal results. A CT scan of these patients showed that this tumor was of pancreatic origin. These 3 tumors were localized to head, body, and tail, respectively. The mean maximal diameter of these tumors was 11.3 +/- 3 cm. Sonography and CT examination showed that the tumor was a heterogeneous mass with solid and cystic components. Angiography of this tumor showed a hypervascular mass with blood supply mainly from pancreatic branch of splenic artery. They all underwent tumor resection. All tumors contained some degree of internal hemorrhage or cystic degeneration and all were well encapsulated. Histologically, tumor cells generally showed solid and pseudopapillary growth around the fibrovascular stalks. No metastasis, mortality or recurrence was noted during follow-ups. In conclusion, CT scan helps to make a prospective diagnosis of papillary cystic neoplasm of pancreas. Our study confirmed that a papillary neoplasm of the pancreas is a low-grade malignant tumor. Surgical resection of the tumor is the mainstay of effective management.

Adolescent↗
PubMedDate not supplied

Analysis of anthropometric growth trends and prevalence of abnormal body status in Tainan elementary-school children.

Objectives of this study were to examine the prevalence of over/underweight or obesity in Chinese children and investigate the trend of anthropometric change through the years. Cross-sectional measurements on randomly selected 6,373 Tainan elementary-school children were conducted in 1997. Anthropometric parameters such as height, weight, body mass index (BMI), triceps skinfold thickness (TSF), mid-arm circumference (MAC) and body-fat percentage (%FAT) were measured. Girls and boys had an average of 4.8 cm and 5.1 cm, or 3.7% and 3.9% increase in height, and 5.5 kg, 6.2 kg, or 19.9% and 22.2% increase in weight when compared to data of nationwide survey in 1986-88. If the height and weight were compared to the data of nationwide survey in 1993-1996, Tainan girls and boys would have similar height but have averaged 3.4% and 4.7% heavier weight. Mean BMI reached 19.4 kg/m2 for girls and 19.6 kg/m2 for boys of age 12 to 13. Mean %FAT of girls was 24.3% and of boys was 23.7%. When using weight-for-length index (WLI) larger than 1.2, mean weight exceeding 120% of age-and-sex specific mean weight, %FAT exceeding 30% to evaluate prevalence of obesity, results would be 42.1%, 17% and 22.0% respectively. In conclusion, both sexes had faster growth in weight than in height in recent 10 years. Boys had significantly higher weight, WLI, BMI, MAC than girls, while girls had significantly higher TSF and %FAT than boys. Prevalence of obesity is highly method-dependent. Appropriate index and cutoff values need to be developed.

Body Height↗
PubMedDate not supplied

Alstrom syndrome with hepatic dysfunction: report of one case.

Alstrom syndrome is a rare autosomal recessive disorder associated with early childhood retinopathy, progressive sensorineural hearing loss, truncal obesity, and acanthosis nigricans. We report a 10-year-old boy with Alstrom syndrome presenting with general malaise and abnormal liver function for 1 year. In addition to the above mentioned features, he also had hyperglycemia and hyperinsulinemia. The mechanism responsible for the persistent elevation of liver enzymes could not be identified. To the best of our knowledge, this is the first-reported case of Alstrom syndrome with hepatic dysfunction in Taiwan.

Acanthosis Nigricans↗
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Menetrier's disease associated with cytomegalovirus infection in a child.

Menetrier's disease is a rare disease with unknown etiology characterized by protein-losing hypertrophic gastropathy and hypoproteinemia. We report on a 4-year-old boy who presented with a 2-week history of vomiting and periorbital edema. Upper gastrointestinal endoscopy revealed prominent gastric rugae, while pathological examination showed hyperplastic mucosa with proliferation, elongation, and basal cystic dilatation of the gastric glands replaced by mucous-secreting epithelium, confirming the diagnosis of Menetrier's disease. Virus isolation from urine revealed cytomegalovirus (CMV); and CMV immunoglobulin G and immunoglobulin M antibodies were detected in the serum. His condition gradually improved under omeprazole, with complete histological recovery after 3 months.

Anti-Ulcer Agents↗
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[Anthropometric study on Down syndrome in Taiwan].

Anthropometric studies of Down syndrome in foreign children had been reported. To have similar information for Chinese children with Down syndrome, data based on 1624 measurements of height and 1208 measurements of weight were done on 496 children who took part in a collaborative study done via several hospitals and institutions in Taiwan. Height and weight were measured using standard methods. Additional data about these children were also solicited from hospital and school records to obtain semilongitudinal data which were then analyzed with SAS PC software. Descriptive statistics and percentiles were estimated using flexible mathematical functions. Results showed that ethnic Chinese children with Down syndrome were significantly shorter than normal children. Mean weight, however, was not significantly different from normal children. Centile charts for assessment of stature and weight are also presented.

Adolescent↗
PubMedDate not supplied

Unusual cardiac malformations in Holt-Oram syndrome: report of two cases.

The Holt-Oram syndrome is a hereditary disease which associated with upper limbs anomalies and cardiac defects such as secundum type atrial septal defect. Two cases of this syndrome with unusual cardiac findings are reported: One has an unroofing coronary sinus type atrial septal defect and preexcitation syndrome, the other has pentalogy of Fallot.

Abnormalities, Multiple↗
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A follow-up study of genetic counseling in Down syndrome.

To ascertain the effects of genetic counseling on the education of counselees, a questionnaire study on the acceptance of genetic knowledge was carried out among 126 parents of children with Down syndrome. Those parents from the "Down Syndrome Association" had best scores for knowledge, followed by those who had received counseling according to a designed format (programmed counseling) in this Hospital. Parental perceptions of the information needed in genetic counseling were also studied and it was found that most parents thought that the topics of education and medical care were more important than genetic information. Realization of such difference in primary concern between counselors and counselees may improve the effectiveness of genetic counseling.

Analysis of Variance↗
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Congenital isolated absence of pulmonary valve in a neonate with partial trisomy 13q.

A term female newborn with partial trisomy 13q [46,XX, -18, +der(18) t(13;18) (13qter-->13q22:: 18q23--> 18pter) pat], is described with unbalanced translocation and isolated absence of the pulmonary valve. The clinical manifestations included hypertelorism, corneal opacification, high arch palate, rocker-bottom feet, polydactyly (both hands and feet), respiratory distress and intractable congestive heart failure. Echocardiogram showed isolated absent pulmonary valve. This consents report such a combination in partial chromosome 13 trisomy.

Abnormalities, Multiple↗
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Common atrium with Ebstein's anomaly in a neonate with Ellis-van Creveld syndrome.

A female newborn with characteristic multiple anomalies of Ellis-van Creveld syndrome is reported. Common atrium with Ebstein's malformation of the tricuspid valve was diagnosed by serial echocardiographic examinations. Such a combination of cardiac anomalies have not previously been reported in this syndrome.

Ebstein Anomaly↗
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Experience of intravenous immunoglobulin and acyclovir in neonates at risk for severe varicella infection--report of five cases.

Five cases of at-risk neonates for severe varicella infection were reported. Intravenous immunoglobulin (IVIG) was given soon after birth in different ways to four of them (Case 1 received 400 mg/kg/day for the first three days of life; Case 2 received a single dose of 200 mg/kg on day 2, and another dose of 160 mg/kg on day 7; Cases 3 and 4 received 400 mg/kg on day 2). All five patients were given acyclovir therapy, 40 to 45 mg/kg/day for five to nine days. (Case 1 for prophylaxis, Cases 2, 3, 4, 5 for treatment). Case 2, 3, 4, 5 developed vesicular rash, mild to moderate in degree, with rapid resolution without any constitutional symptoms. Case 1, who received both IVIG and acyclovir for prophylaxis, had no vesicle occurrence. IVIG, in combination with acyclovir, is a safe and probably effective regimen for preventing severe varicella in at-risk neonates. A multi-centered, collaborative study is mandatory to study the efficacy of prophylactic acyclovir with IVIG in these neonates.

Acyclovir↗
PubMedDate not supplied

Kabuki make-up syndrome associated with congenital heart disease.

The Kabuki make-up syndrome, reported in 1981 by Niikawa et al. and Kuroki et al. independently, is also known as "Niikawa-Kuroki syndrome". Here we report two cases of this syndrome, both of them were boys, showing facial dysmorphism, hand abnormalities and congenital heart disease. The first case had developmental retardation noticed since 3 months old and frequent upper respiratory tract infections in the following years, and the cardiac echo revealed mitral regurgitation and tricuspid regurgitation. The second case had patent ductus arteriosus and ventricular septal defect diagnosed at 17 days old. He underwent corrective surgery for his heart problems, he also couldn't complete a sentence at two and half years old. It was estimated that the incidence of this syndrome was 1/32,000 in Japanese Children, and Chinese are similar to Japanese as yellow-skinned oriental, there should be more cases in our country, and a detailed cardiac study may be necessary for them.

Abnormalities, Multiple↗
PubMedDate not supplied

Bilateral internal carotid artery aneurysms with diffuse intracranial calcification: report of one case.

A 15-year-old girl with voice hoarseness and neck soreness for 4 months had a pulsating mass, sized 3 x 5 cm, adjacent to the right mastoid process. Physical examination revealed normal blood pressure recordings in 4 limbs and no stigmata of Marfan syndrome. Examination of the oral cavity showed bulging of the right pharyngeal wall medially, and the tongue deviated to the right at protrusion. Laboratory workups including complete blood counts, biochemistry, electrolytes, serological and immunological surveys revealed nothing particular. Echocardiography disclosed mild aortic dilatation. Computed tomography of the head and neck revealed bilateral carotid aneurysms with extensive intracranial calcification. The saccular aneurysm over the left internal carotid artery was resected, and end-to-end anastomosis of the left internal carotid artery was performed. The giant occluded right carotid aneurysm was left unmanaged. The combination of bilateral carotid aneurysms and intracranial calcification has not been reported. The etiologies are discussed and a causal relationship between these two conditions is postulated.

Adolescent↗
PubMedDate not supplied

[Insulinoma in childhood--report of a case].

A 14-year-old boy was having recurrent hypoglycemia over the past 6 months, so he was brought to our hospital for further evaluation. During the episodes of hypoglycemia, his serum insulin level was inappropriately high and the symptoms of hypoglycemia were dramatically ameliorated by intravenously administering glucose bolus. Computed tomography of the abdomen revealed a 1 x 1 cm mass located at the tail of the pancreas. Under the impression of an insulinoma, enucleation of the tumor was performed smoothly, and the pathological finding confirmed the diagnosis. The postoperative course was smooth and no episodes of hypoglycemia were noted after treatment. In children with hypoglycemic attacks, adequate blood sampling before correction to determine plasma insulin levels is essential.

Adolescent↗
PubMedDate not supplied

Rigid spine syndrome presenting with respiratory failure--report of one case.

A case of rigid spine syndrome presenting with respiratory failure was reported. A seven-year-old girl had had dyspnea and orthopnea for one month. Symptoms had aggravated gradually and she was in a state of respiratory failure on arrival at our hospital. There was no evidence of active lung lesions and response to mechanical ventilation was good. Physical examination revealed an extremely thin girl with marked flexion limitation of neck and severe wasting of sternocleidomastoid and intercostal muscles. Serum creatine phosphokinase was moderately elevated (801 IU/L). Muscle biopsy specimen obtained from the right quadriceps femoris revealed increased perimyseal connective tissue and marked Type II fiber atrophy. Electromyography of left paraspinal muscles demonstrated small amplitude, short duration motor unit potentials. She received tracheostomy and a home-care ventilator use during sleep. Ventilatory insufficiency, though rare, should be anticipated in patients with rigid spine syndrome, and timely ventilatory support should be given.

Child↗
PubMedDate not supplied

Suprasellar embryonal carcinoma: report of one case.

Intracranial embryonal carcinoma is a rare germ cell tumor found predominantly in the pineal region and, to a lesser extent, in the suprasellar region. The case of a 12-year-old female with a history of secondary amenorrhea for 6 months is reported; her symptoms included decreased visual acuity, dizziness and postprandial vomiting over a 1-month period. A huge suprasellar mass was found by computed tomography. Serum alpha-fetoprotein (AFP) and beta-human chorionic gonadotropin (beta-HCG) levels were elevated. The tumor was subtotally resected; pathologic and immunocytochemical findings were compatible with embryonal carcinoma. The patient died three weeks after operation. The case is described and pertinent literature is reviewed.

Brain Neoplasms↗