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Biomedical subjects

R J Teng

Publications and source records attributed to R J Teng.

At least 37 records · Page 2Linked to original sources

Tricho-rhino-phalangeal syndrome type II associated with epiglottic aplasia and congenital nephrotic syndrome.

A male neonate with tricho-rhino-phalangeal syndrome type II (Langer-Giedion syndrome) and interstitial deletion of chromosome 8 with karyotype 46, XY, del (8) (q24.11-->q24.13) is reported. In addition to hypotrichosis of the scalp hair, abnormally bulbous nose and redundant skin, which are usually found in this syndrome, aplasia of the epiglottis and non-Finnish type congenital nephrotic syndrome were also present. He died of renal failure and respiratory failure at 11 days of age. Electron microscopy of renal necropsy showed foot process loss, focal thickening and splitting of the glomerular basement membrane and mesangial expansion. These have never been reported and could be new associations in this disease that may serve to support the concept of contiguous gene syndrome in patients with tricho-rhino-phalangeal syndrome.

Epiglottis↗

Comparison of cryotherapy and laser photocoagulation in stage III retinopathy of prematurity.

Both cryotherapy and laser photocoagulation have been advocated for the treatment of stage III retinopathy of prematurity (ROP). In this retrospective study, we looked at which treatment was more effective and caused fewer complications. The outcomes of 23 patients (45 eyes) with stage III ROP treated with transconjunctival cryotherapy or indirect diode laser photocoagulation were reviewed. Group 1 (10 patients, 20 eyes) received cryotherapy and group 2 (13 patients, 25 eyes) received photocoagulation. In group 1, all had progressive ROP and one patient had bilateral neovascularization involving zone I. After treatment, 17 eyes had regression of ROP with good anatomic results, one eye had regression of the disease with mild to moderate macular ectopia and vessel traction, and two eyes (one patient) with zone I involvement advanced to stage V in 2 weeks. In group 2, 12 patients had threshold ROP, two had neovascularization involving zone I, and one patient had prethreshold zone I ROP. After treatment, 24 eyes had regression of ROP with good anatomic results and one eye had mild ectopic macula. Two eyes received two sessions of treatment. The results suggest that photocoagulation is at least as effective as cryotherapy and may be a favorable alternative for zone I ROP.

Cryotherapy↗

Management of severe pulmonary hypertension in an infant with obstructed total anomalous pulmonary venous return using magnesium sulfate.

Severe hypoxemia and pulmonary hypertension occurred in an infant with total anomalous pulmonary venous return. Though under hyperventilation with high fractional inspired oxygen and prostaglandin E1, hypoxemia remained intractable. Magnesium sulfate was used in view of its vasodilating effect. Significant clinical improvement was observed without causing systemic hypotension and the mechanism is discussed.

Alprostadil↗

Reliability of the Neonatal Neurobehavioral Examination--Chinese version.

A Chinese version of the Neonatal Neurobehavioral Examination (NNE-C) was applied to 15 high-risk infants and five normal term infants for investigation of reliability. The infants were assessed by three physical therapists to examine inter-rater reliability and reassessed by one of the therapists within 2 days to examine test-retest reproducibility. The internal consistency of the NNE-C scale was high, with an alpha coefficient of 0.84. The inter-rater reliability was high for item scores (kappa coefficients > 0.75 for 81% of the items) and for section and total scores (all intraclass correlation coefficients > 0.80). The test-retest reproducibility was moderate for item scores (kappa coefficients > 0.40 for 85% of the items) and was high for section and total scores (all intraclass correlation coefficients > 0.80). We conclude that the NNE-C scale is clinically feasible and reliable for the evaluation of neurobehavioral functions of high-risk and normal term infants in Chinese-speaking societies.

Behavior↗

Necrotizing bowel lesions complicated by Pseudomonas septicaemia in previously healthy infants.

UNLABELLED: Two previously healthy infants with Pseudomonas septicaemia presented with necrotizing bowel lesions. Necrotizing bowel lesions should be suspected when infants presenting with a history of diarrhoea, develop abdominal distension and toxic signs. Pseudomonas aeruginosa should be regarded as one of the important aetiologies in such disorders, especially if there is associated neutropenia and ecthyma gangrenosum-like lesions. Antibiotics must be able to cover this pathogen to avert a catastrophic outcome. CONCLUSION: The intestine should be considered a possible site of involvement in Pseudomonas sepsis and special attention should be paid to examination of the abdomen.

Enterocolitis, Pseudomembranous↗

Transfusion-related acute lung injury treated with surfactant in a neonate.

UNLABELLED: A term, male neonate suddenly developed respiratory distress and severe cyanosis while undergoing exchange transfusion for hyperbilirubinaemia. Transfusion-related acute lung injury was diagnosed. Because of persistent hypoxaemia despite aggressive treatment, two doses of surfactant were administered, resulting in marked improvement. CONCLUSION: Transfusion-related acute lung injury may occur in neonates, and may be successfully treated by surfactant replacement.

Exchange Transfusion, Whole Blood↗

Nonprogressive congenital unilateral ventriculomegaly.

Congenital unilateral ventriculomegaly is a rare condition, usually caused by obstruction of the foramen of Monro. In the past, this condition required surgical intervention. We present a female newborn with nonprogressive unilateral ventriculomegaly which was initially detected by prenatal sonography. No surgical intervention was performed, and during the 9 months of follow-up, she had normal head growth and reached appropriate developmental milestones.

Cephalometry↗

Decreased urinary epidermal growth factor in children with acute renal failure: epidermal growth factor/creatinine ratio not a reliable parameter for urinary epidermal growth factor excretion.

To verify some animal experimental results in humans, we have studied urinary epidermal growth factor (EGF) excretion in normal children as well as children with acute renal failure (ARF). Urinary EGF excretion was expressed as a ratio of urinary EGF to urinary creatinine concentration (EGF/Cr) for random and 24-h urine, and a daily total urinary EGF for 24-h urine. The highest urinary EGF/Cr in children was found at 1 mo to 3 y of age. There was a highly significant correlation between random urine EGF/Cr and 24-h urine EGF/Cr (r = 0.92, p < 0.001), whereas no correlation of urinary EGF/Cr with daily total urine EGF was found. During the course of ARF, a decline in urinary EGF/Cr from the period before peak serum creatinine to the period after the declination of serum creatinine was noted (p = 0.013, n = 13, by repeated measure analysis), with a constant low daily total urine EGF (p value not significant). However, a rise in both urinary EGF/Cr and daily total urine EGF was found between the period of serum creatinine decline and the period of completely normal serum creatinine (p < 0.001). Serum EGF remained unchanged throughout the course of ARF. These results suggest 1) the possible role of EGF in renal growth or maturation during the first 2 or 3 y of life, 2) the possible renal origin of human urinary EGF, and 3) decreased urinary EGF excretion in children with ARF. In particular, EGF/Cr is not a reliable indicator for the expression of actual urinary EGF excretion in ARF. Instead of urinary EGF/Cr, urinary EGF concentration may be used to predict the daily total urinary EGF excretion during ARF. These results provide the pattern of urinary EGF excretion during ARF in children and may be of help for further clinical studies.

Acute Kidney Injury↗

Hemolytic disease of the newborn caused by anti-M antibody.

An unusual case of hemolytic disease of the newborn caused by anti-M antibody is presented. Hyperbilirubinemia was noted in a full-term baby boy at 4 days of age. A total of 160 mL of M-positive packed red blood cells from the baby's father were transfused during the next 9 days and the hemolytic process became aggravated. The baby was referred to our hospital at 14 days of age. Maternal anti-M was detected and the baby was transfused with 50 mL of M-negative packed red blood cells. The baby's condition stabilized and he was discharged uneventfully at 18 days of age.

Erythroblastosis, Fetal↗

Rigid bronchoscopy and jet ventilation in an extremely low birthweight infant.

Until recently, rigid bronchoscopy was considered too risky for premature infants. We report a 658 g infant with life-threatening mediastinal shift due to right pulmonary atelectasis. Rigid bronchoscopy revealed tenacious mucous plugs in the right main bronchus. The collapsed lung was successfully reinflated after bronchial lavage with a rigid bronchoscope, with the help of intermittent high-frequency jet ventilation (HFJV). The infant's mediastinal shift was reversed and the vital signs became stable. In our experience, rigid bronchoscopy combined with HFJV is a valuable diagnostic and therapeutic procedure for very small premature infants.

Bronchoalveolar Lavage↗

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↗

Persistent pulmonary hypertension of the newborn treated with magnesium sulfate in premature neonates.

OBJECTIVE: To evaluate the clinical effects of magnesium sulfate (MgSO4) in the treatment of persistent pulmonary hypertension of the newborn (PPHN) in premature infants. METHODS: This was a prospective, nonrandomized, clinical study. Seven premature neonates with PPHN were treated with MgSO4 as soon as documentation of an interatrial right-to-left shunt was made. A loading dose of 200 mg/kg was infused over 30 minutes, followed by a maintenance dose of 20 to 50 mg/kg/h. Alveolar-arterial oxygen tension difference (AaDO2) and oxygenation index were followed up sequentially as the primary outcome measures. Blood pressures and serum electrolytes were also monitored. RESULTS: Six cases responded clinically. The decrease of AaDO2 reached significance at 36 hours, but the decrease of oxygenation index was not significant over 72 hours. Four infants survived. No significant side effects were encountered. CONCLUSION: Our results suggest that MgSO4 may be considered as an alternative treatment of PPHN in premature infants.

Female↗

Diffuse neonatal haemangiomatosis with intra-uterine haemorrhage and hydrops fetalis: a case report.

A case of diffuse neonatal haemangiomatosis involving the skin, liver, lungs, adrenals, gums, diaphragm, skull, and testes is reported. Intra-uterine onset of bleeding led to bloody amniotic fluid, severe anaemia, congestive heart failure, and hydrops fetalis. Intractable coagulopathy and renal failure resulted in persistent bleeding, anuria, metabolic acidosis, and hyperkalaemia, leading to a fatal outcome.

Anemia, Neonatal↗

Doppler sonographic detection of reverse twin-twin transfusion after intrauterine death of the donor.

We report a "stuck" twin, in which a reverse blood shunt from the recipient to the donor was detected by Doppler sonography after death of the donor. This acute reverse transfusion caused intrauterine fetal distress and severe neonatal anemia of the surviving recipient. We believe that acute hemodynamic change after death of one fetus, in addition to causing a derangement in coagulation, causes immediate danger or subsequent organ damage for the surviving co-twin.

Adult↗

Retinopathy of prematurity: an analysis of risk factors.

In order to investigate the possible clinical factors related to the occurrence of retinopathy of prematurity (ROP), 37 very-low-birth-weight infants documented to have ROP were compared to a group of 50 controls during the period from July 1988 to December 1991. The results revealed that: 1) the overall occurrence rate of ROP was 42.5% (37/87), with a higher occurrence rate in the less mature weight-stratified subgroup; 2) patients in the lower stages of ROP generally regressed during the follow-up, while nearly all of those in the higher stages eventually progressed and received surgical intervention; and 3) after multiple logistic regression analysis, the consistently significant factors were birth weight, duration of PaO2 > or = 80 mmHg, ventilation time > or = five days and a blood exchange transfusion. It is suggested that regular examination for ROP in high-risk premature infants is important in combination with other monitoring modalities in neonatal intensive care units to reduce the possible complications and sequelae of ROP.

Female↗

Congenital short bowel syndrome: report of a case treated with home central parenteral nutrition.

Congenital short bowel is a rare anomaly. Among the 21 cases reviewed in the literature, only four (19%) survived beyond infancy. We present a female infant with short small bowel, malrotation and floating colon, who was successfully treated with home central parenteral nutrition. Improved long-term survival in this group of patients is expected with prolonged parenteral nutritional support.

Female↗

An autopsy case of Reye's syndrome associated with acute pancreatitis, acute renal failure and disseminated intravascular coagulopathy.

A three-and-a-half-year-old boy was transferred to our hospital under the impression of Reye's syndrome. The laboratory data showed hypoglycemia, hyperammonemia and elevated serum transaminases. A remarkable high serum amylase level of 2,223 IU/L and CAm/CCr of 36% was noted. A blood urea nitrogen level of 143 mg/dl was found on the third day and a creatinine level of 8.7 mg/dl on the fourth day. Disseminated intravascular coagulopathy (DIC) and systemic candidal infection complicated his final course. He died after intensive treatment for eleven days. Hemorrhagic pancreatitis and fatty change of the liver were noted at autopsy. Disseminated candidal invasion was noted within the kidneys, cerebrum, and lungs. Tonsillar herniation, systemic candidiasis and bronchopneumonia were believed to be the causes of his death. It is extremely rare for all three complications, acute pancreatitis, acute renal failure and DIC, to occur in Reye's syndrome at the e time.

Acute Disease↗