Histopathology in pediatric recipients of living related liver transplantation.
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Biomedical subjects
Publications and source records attributed to C B Huang.
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Artificial larynges provide a means of verbal communication for people who have either lost or are otherwise unable to use their larynges. Although they enable adequate communication, the resulting speech has an unnatural quality and is significantly less intelligible than normal speech. One of the major problems with the widely used Transcutaneous Artificial Larynx (TAL) is the presence of a steady background noise caused by the leakage of acoustic energy from the TAL, its interface with the neck, and the surrounding neck tissue. The severity of the problem varies from speaker to speaker, partly depending upon the characteristics of the individual's neck tissue. The present study tests the hypothesis that TAL speech is enhanced in quality (as assessed through listener preference judgments) and intelligibility by removal of the inherent, directly radiated background signal. In particular, the focus is on the improvement of speech over the telephone or through some other electronic communication medium. A novel adaptive filtering architecture was designed and implemented to remove the background noise. Perceptual tests were conducted to assess speech, from two individuals with a laryngectomy and two normal speakers using the Servox TAL, before and after processing by the adaptive filter. A spectral analysis of the adaptively filtered TAL speech revealed a significant reduction in the amount of background source radiation yet preserved the acoustic characteristics of the vocal output. Results from the perceptual tests indicate a clear preference for the processed speech. In general, there was no significant improvement or degradation in intelligibility. However, the processing did improve the intelligibility of word-initial non-nasal consonants.
A case of bilateral cystic neuroblastoma with liver metastases in a newborn is reported. CT showed a 10 cm right suprarenal multicystic mass and numerous hepatic cystic masses with intracystic fluid-fluid levels. Multiple smaller cystic lesions were also present in the left adrenal gland. To our knowledge, the CT findings of neonatal bilateral cystic neuroblastoma with liver metastasis and massive acute intracystic haemorrhage has not been previously documented.
OBJECTIVE: To localize cytochrome P450 enzymes 4A and 2C in central nervous cells of normal male rats. METHODS: Eight drug/alcohol untreated normal male rats (150-200 g of body weight) were treated by the optimized perfusion technique, then brain tissues were postfixed, paraffin-embedded and cut into series sections, which were labeled by the improved strept-avidin-biotin complex DAB-nickel enhancer (SABC-DAB-Ni) immunohistochemistry and hematoxylin & eosin (H & E) stain techniques. RESULTS: The immunohistochemical results indicated that P450 2C-11 enzyme was localized in diverse numbers of neurons as well as some neuroglial cells, with focal or defuse distribution in many brain regions such as cerebrum, thalamus, olfactory bulb, hypothalamus, brain-stem, hippocampus, cerebellum, interpositus nucleus, caudate-putamen, and globus pallidus. In contrast, no positive findings of P450 4A-2, 3 and 8 enzymes were obtained in the same animals. With high magnification, 2C-11 protein was able to be roughly observed on the endoplasmic reticulum of the rat neurons. CONCLUSIONS: P450 2C-11 protein, rather than P450 4A-2, 3 and 8, may be a candidate of brain P450 enzymes in the normal male rats.
Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a rare congenital disorder characterized by a massively enlarged urinary bladder without mechanical outlet obstruction and microcolon, as well as a hypoperistaltic bowel with normal ganglion cell distribution. We report one such case to discuss the findings of antenatal ultrasound and the radiologic and pathologic features of this condition.
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BACKGROUND: Necrotizing enterocolitis (NEC) is the most significant acquired gastrointestinal (GI) emergency in the neonatal intensive care unit. METHODS: We sought to gain a clinical perspective on NEC by reviewing the records of NEC patients over a 9-year period. The case histories of 22 infants with NEC treated from September 1, 1986 to September 1, 1995 were reviewed. RESULTS: Mean gestational age was 32 weeks and mean birth weight was 1774 grams. Eighteen percent were full term babies and 82% were premature. Average age at the onset of NEC was 11 days. The most common clinical manifestations were abdominal distension (100%), gastric retention (64%), unstable vital signs (59%) and Guaiac-positive vomitus or stool (36%). Sixteen cases (73%) were classified as stage III NEC, which has the highest mortality and/or morbidity. CONCLUSION: Early identification and management are critical to improve the outcome of NEC.
We report a very rare case of systemic sclerosis in a 6-year-old girl. She presented with diffuse scleroderma, Raynaud's phenomenon, pulmonary interstitial fibrosis, pulmonary hypertension, and right ventricular failure. The diagnosis was confirmed by skin manifestations, high resolution computed tomography, cardiac catheterization, and anti-nuclear antibodies. Nifedipine, prednisolone, digoxin, and furosemide were given. There was remission of the right ventricular failure and dyspnea, and the skin showed partial improvement. The patient remained asymptomatic for a year. The symptoms of respiratory and right heart failure developed again after an episode of lower respiratory tract infection and she eventually died. We discuss the clinical manifestations, treatment, and outcome.
A 2-day-old newborn was treated with tolazoline for persistent pulmonary hypertension due to meconium aspiration syndrome. Hypoxemia improved during continuous tolazoline infusion, but gastrointestinal bleeding occurred. After treatment with cimetidine, there was a rapid deterioration with decreased oxygen saturation and arterial PO2 values. Tolazoline induces a dilatation of the pulmonary vascular system by stimulating H2 receptors. Cimetidine, an H2 blocker, may partially abolish the effect of tolazoline. However, given the clinically significant result to the interaction between tolazoline and cimetidine we report, the use of cimetidine in tolazoline induced upper gastrointestinal hemorrhage should deserve more attention.
Hydrometrocolpos is a rare congenital anomaly and serious life threatening condition in the newborn infant due to its long-term compression sequelae and associated congenital anomalies. Prenatal diagnosis of hydrometrocolpos by sonogram allows appropriate management during the prenatal and neonatal period. The combination of hydrometrocolpos and polydactyly is the cardinal hallmark feature of McKusick-Kaufman Syndrome. We present a case of congenital hydrometrocolpos due to vaginal atresia combined with polydactyly of both feet, mild atrial septum defect, bilateral hydronephrosis, fetal ascites and polyhydramnios. Pathogenesis and treatment of hydrometrocolpos and its associated congenital anomalies are discussed.
Candida infection contracted in utero and manifested at birth is a rare event. Two newborn infants are presented with generalized maculopapular rashes at birth. The eruption soon became vesicles or pustules; no other manifestations were found. Candida albicans was isolated from the skin lesion. After topical antifungal application, the lesions became fine and desquamated in the recuperative period. No complication was noted during the course. The diagnosis of congenital cutaneous candidiasis relies on its clinical course and positive culture result of the skin lesion. No systemic antifungal therapy is recommended except for the high risk infants.
Small-intestinal morphology, particularly the amount of goblet cells, was studied in Syrian golden hamsters fed either a low-fibre (0.5% fibre) diet or the same diet supplemented with oat bran, rye bran, or soybean hull (12% fibre) during 6 weeks. All fibre diets increased the density of goblet cell volume to epithelial volume and to villus volume in the ileum and to some extent in the jejunum and also the number of goblet cells related to surface traces of villi both in the proximal and distal small intestine. The weight of the small intestine and the circumference and the volume of the intestinal wall in the ileum were greater in the rye bran diet group as compared with all the other groups, suggesting hyperplasia. These results suggest that oat bran, rye bran, and soybean hull supplementation might result in higher goblet cell secretory activity in both the proximal and distal small intestine of hamsters. The various fibre sources influenced the small-intestinal wall differently.
A case of primary hyperparathyroidism in an 11-year-old boy who presented with bowlegs is reported and the literature is reviewed. From the literature, it appears that the pathologic findings, clinical manifestations and sex distribution of primary hyperparathyroidism is different in adults than in children and young infants. In adults, primary hyperparathyroidism is commonly caused by adenoma and is often found unexpectedly on a biochemical screen of the serum. Elderly women are most at risk. Renal stones or other renal complications are more frequently seen in adults than in children. Neonatal hyperparathyroidism is often genetically transmitted and is fatal unless recognized and treated early. The characteristic pathologic change is chief cell hyperplasia of the parathyroid glands. On the other hand, adenoma is the most common pathologic finding in older children. No case of childhood parathyroid carcinoma was found in our review of the literature. In children, primary hyperparathyroidism is more frequent in boys than in girls and osseous lesions are more common than in adults. Prognosis is good if the disease is recognized early and appropriate surgical treatment is received.
On the basis of clinical and laboratory data of 50 patients with adult onset Still's disease (AOSD), we strictly examined 4 diagnostic criteria adopted by different authors: criteria of Calabro, ARA, Medsger and Liu Gui-xin. Our result shows that Calabro's criteria has the best specificity (100%) and a higher diagnosis index (0.90) among the 4 criteria. Liu's criteria provides the best sensitivity (98%), but a lower specificity (86.9%) with a misdiagnosis rate of 14%. The rate of missed diagnosis with the ARA criteria is 35.7%. According to the present study, we recommend Liu's criteria for preliminary screen diagnosis, while Calabro's criteria for confirmation and differential diagnosis. Of special interest is that serum ferritin (SF) determination showed significantly higher level in 20 patients with AOSD (average 1194.5mg/L) than that in 19 patients with other rheumatic diseases (average 94mg/L, P < 0.001). AOSD patients with active disease have higher SF levels than patients with inactive disease (average 2742.9mg/L & 291.25mg/L, P < 0.001). So this test might be useful in diagnosis in AOSD and assessment of disease activity.
We present a case of lethal short rib-polydactyly syndrome (SRPS) that cannot be categorized into the existing classification. A nosologic discussion is presented. To our knowledge, situs inversus totalis, as in our case, has not been described before in any SRPS.
Edwards syndrome is the second most common autosomal trisomy syndrome, resulting from an extra chromosome in chromosome 18. Recently we encountered two cases with the following representative features: intrauterine growth retardation, polyhydramnios, low-set ears, micrognathia and clenched hands. Both cases were female newborns and had an abnormal karyotype 47, XX, 18.
Neonatal adrenal hemorrhage is frequently associated with large fetal size, birth trauma owing to difficult labor or delivery, perinatal asphyxia, fetal hypoxia, septicemia or coagulation defect. A case of giant baby with perinatal asphyxia and birth injury complicated with adrenal hemorrhage is presented. The baby was admitted at five days of life due to profound jaundice. Right adrenal hematoma was found by abdominal sonography. The hematoma resolved gradually under supportive management by three months of age as documented by serial sonography. No complication was found. We conclude that abdominal sonography is the modality of choice for initial diagnosis and follow-up of neonatal adrenal hemorrhage, and conservative management is the mainstay of treatment.
Congenital cystic adenomatoid malformation of the lung is a rare but potentially lethal variety of congenital pulmonary defect. This is a multicystic mass of dysplastic pulmonary tissue in which there is proliferation of bronchial structure at the expense of alveoli. We report a newborn infant with respiratory distress. The chest X-ray showed multicystic lesions with air-fluid level and shift of mediastinum. The patient was successfully treated with segmentectomy. The clinical manifestations, radiographic and pathologic features were summarized and the literature reviewed.