Codon 219 Lys allele of PRNP is not found in sporadic Creutzfeldt-Jakob disease

OBJECTIVE: To determine the prevalence of hypergastrinemia in cats with naturally developing chronic renal failure (CRF) and the correlation between gastrin concentration in plasma and severity of CRF. DESIGN: Cohort study. ANIMALS: 30 cats with naturally developing CRF and 12 clinically normal control cats. PROCEDURE: Gastrin concentrations in plasma were determined by double-antibody radioimmunoassay of blood samples obtained from cats after food was withheld 8 hours. Concentrations were compared, using a nonparametric Kruskal-Wallis ANOVA. RESULTS: 18 cats with CRF had high gastrin concentrations (median, 45 pg/ml; range, < 18 to > 1,333 pg/ml), compared with those for control cats (< 18 pg/ml). Prevalence of hypergastrinemia increased with severity of renal insufficiency. Three of 9 cats with mild CRF, 6 of 11 cats with moderate CRF, and 9 of 10 cats with severe CRF had high gastrin concentrations. Gastrin concentrations were significantly different between control cats and cats with CRF, regardless of disease severity. CLINICAL IMPLICATIONS: The potential role of high concentrations of gastrin on gastric hyperacidity, uremic gastritis, bleeding from the gastrointestinal tract, and associated clinical signs of hypergastrinemia (e.g., anorexia and vomiting) may justify use of histamine2-receptor antagonists or proton pump inhibitors to suppress gastric acid secretion in cats with CRF that have these clinical signs.     

Zollinger-Ellison syndrome. Improved treatment options for this complex disorder

Zollinger-Ellison syndrome is a rare disorder characterized by severe peptic ulcer disease, gastric acid hypersecretion, and non-beta islet cell tumors of the pancreas. Most gastrinomas are found within an anatomic area known as the gastrinoma triangle. However, they commonly occur in extrapancreatic sites in multiple endocrine neoplasia type 1 syndrome. In patients in whom Zollinger-Ellison syndrome is suspected, laboratory evidence of hypergastrinemia and hyperacidity establishes the diagnosis. Until the advent of proton pump inhibitors, total gastrectomy was the treatment of choice. Therapy with these agents (eg, omeprazole, lansoprazole) can prevent ulcer disease. However, surgical removal of gastrinomas offers a chance for cure and can improve longevity by preventing the malignant spread of the tumors.     

Implications of the DCCT. Looking beyond tight control

A burning sensation in the throat is often complained of by patients, but this symptom has been neglected in the literature. To emphasize its importance, should we give the name 'caloripharyngeus' to the symptom of burning in the throat? A preliminary study of 30 patients with the sensation of burning in the throat is presented in this study. Of the patients studied 73.33 per cent had hyperacidity detected for the first time. Reflux was presented in only one patient; four patients showed oesophagitis in the lower one-third of the oesophagus; 90 per cent of the patients responded to antacids. We suggest that one of the causes of burning in the throat is a referred sensation due to hyperacidity.     

Eosinophilia in newborn infants

To evaluate the clinical significance of eosinophilia in newborn infants, 261 admissions to the neonatal unit over a 12-month period were studied retrospectively; 33 babies with eosinophilia (> 1.0 x 10(9)/l) were studied. Clinical and laboratory data for the first month of life were compared, where available, between gestational age-matched pairs with and without eosinophilia. Of the 33 babies with eosinophilia, 23 were > 26 weeks' gestation and all had age-matched controls; 10 were < or = 26 weeks' gestation but had no appropriate gestational age-matched controls. Babies > 26 weeks' gestation with eosinophilia had a significantly higher number of septic episodes than controls: 20 of 23 versus 4 of 23. All 10 babies < or = 26 weeks' gestation with eosinophilia developed sepsis. Infections with gram-negative organisms and necrotizing enterocolitis occurred only in babies who developed eosinophilia. In 5 babies no cause for the eosinophilia was found. In conclusion, eosinophilia in the newborn is usually explainable and is most often associated with infection.     

Lansoprazole: a cause of secretory diarrhea

A case of severe diarrhea and hypergastrinemia after 6 wk of lansoprazole therapy is presented. This represents the only fully evaluated report of severe diarrhea due to lansoprazole and comes to the interesting conclusion that it was a secretory diarrhea likely due to lansoprazole and not a gastrinoma or another cause.     

High turnover osteopenia in preterm babies

Osteopenia is common in preterm babies, but its pathogenesis is uncertain. In this study bone density in babies was quantitated, postnatal bone mineralization compared to expected intrauterine bone mineralization and the pathogenesis of osteopenia investigated. Healthy babies (103 term, 76 preterm) were examined clinically, biochemically and radiologically the day after birth and at a time corresponding to expected full term gestation. Appendicular bone density was quantitated by magnification radiogrammetry, using the humeral cortical index (CI). The CI of preterm and term babies was similar the day after birth. In preterm babies elevated serum alkaline phosphatase and high urinary hydroxyproline indicated increased bone turnover. The CI of preterm babies at expected full term gestation was lower (p = 0.0001) than that of term babies at birth, implying that postnatal bone mineralization lagged behind expected intrauterine bone mineralization. Radiologic data suggested increased endosteal resorption rather than decreased bone formation. At expected full term gestation the preterm babies had higher serum alkaline phosphatase and urinary calcium, phosphate, c-AMP and hydroxyproline (p = 0.0001) than term babies at birth, and 15% had periosteal reactions. The biochemical as well as the radiologic data therefore indicated high turnover osteopenia in preterm babies. We conclude that postnatal bone mineralization in preterm babies lagged significantly behind expected intrauterine bone mineralization and that the osteopenia observed in preterm babies is caused by increased bone resorption and not by decreased bone formation. The cause(s) of this high turnover osteopenia, however, remains to be ascertained.     

Aggressive forms of gastric neuroendocrine tumors in multiple endocrine neoplasia type I

In recent classifications of gastric endocrine tumors, tumors arising in patients with multiple endocrine neoplasia type 1 (MEN-1) are regarded to be regulated by the concomitant hypergastrinemia resulting from to pancreatic or, most commonly, duodenal gastrinomas and to have a benign behavior. In this article, we report on two cases of MEN-1 gastric neuroendocrine tumors having a fatal course. Case 1 was a young male with hyperparathyroidism and Zollinger-Ellison syndrome and with florid development of multiple gastric carcinoids and multiple duodenal gastrinomas. Metastases occurred in the liver, of exclusive gastric origin, in periduodenal lymph nodes, of exclusive duodenal origin, and in perigastric lymph nodes, of mixed origin. The patient died 48 months after diagnosis. Case 2 was an adult female patient with hyperparathyroidism, adrenocortical disorders, and gastric tumors but no hypergastrinemia. The patient died 3 months after tumor diagnosis. At autopsy, the stomach showed multiple benign carcinoids and two independent neuroendocrine carcinomas not reported before in MEN-1 and massively metastatizing to lymph nodes, liver, and peritoneum. Multiple islet cell tumors mostly producing pancreatic polypeptide were found, whereas gastrinomas were seen in neither the pancreas nor the duodenum. Allelic losses at the MEN-1 gene locus in chromosome 11q13, the mechanism responsible for tumor development in MEN-1 syndrome, were demonstrated in the carcinoid tumors of case 1 and in the neuroendocrine carcinoma of case 2. We conclude that gastric neuroendocrine tumors in patients with MEN-1 may have a poor outcome, they have the same genetic mechanism as MEN-1 tumors in other organs, and they may be independent of the trophic effect of hypergastrinemia.     

Low fetal morbidity in pregnancy associated with acute and chronic idiopathic thrombocytopenic purpura

Forty-six mothers with immune thrombocytopenic purpura (ITP) gave birth to 72 babies. Sixty-two babies were delivered vaginally and 10 babies by cesarean section. There was no mortality among mothers or babies. Eighteen infants were born thrombocytopenic (PLT < 100 x 10(9)/l). Eleven infants had a platelet count of less than 50 x 10(9)/l. All the severely thrombocytopenic babies (except 1) were born to post splenectomy thrombocytopenic mothers, regardless of steroid treatment during pregnancy. Five babies had clinical manifestations of bleeding; 3 had mild purpura, 1 severe gastrointestinal bleeding, and 1 intracranial bleeding. The latter 2 babies were born prematurely to the same mother who was severely thrombocytopenic despite splenectomy in childhood. In view of very low morbidity in babies of ITP mothers, we suggest that they be delivered vaginally. Cesarean delivery should be performed in selected cases where the mother is severely thrombocytopenic despite splenectomy or where prematurity or obstetrical complications are encountered.     

稀酸固氟技术改进试验

文章介绍了针对制酸系统洗涤酸中氟浓度高,腐蚀设备,影响其使用周期的情况,通过试验,采用稀酸固氟办法,从而提高稀酸固氟效果和烟气脱氟效率,减少腐蚀,创造了更高的经济效益.     

Energy requirements for growth and development in infancy

Current international recommendations for energy requirements are based on >9000 measurements of energy intake in both breast- and formula-fed infants. The measurement of energy intake in babies is far from straightforward and the possibility of significant error is great. The opportunity now exists, however, to compare current recommendations with measurements of total energy expenditure (TEE) obtained via the doubly labeled water technique. Approximately 300 measurements of TEE in the first year of life have been made in normal, healthy babies. These data show that estimates of energy intake derived from the measurements of TEE are considerably below the current international recommendations. The same technique has also allowed the energy requirements of sick infants to be evaluated. Two examples are highlighted of infants born small for gestational age and infants born with cystic fibrosis. First, data collected from babies born small for gestational age suggest that such infants have a TEE and hence requirement approximately 20% above that found in infants born with a weight appropriate for their gestational age. This information will be relevant to those professionals attempting to supply optimum nutrition to babies born small for gestational age. Second, in cystic fibrosis it has been suggested that, concurrent with the basic features of the disease, there is an energy-wasting lesion that will increase TEE and hence energy requirement. Recent data collected from babies with cystic fibrosis strongly suggest that this is not the case, and previous data were confounded by subclinical disease status.     

Retinopathy of prematurity: incidence and risk factors

OBJECTIVE: To determine the incidence of Retinopathy of Prematurity (ROP) in high risk babies admitted to the neonatal unit and to study risk factors for it's development. DESIGN: Prospective cohort study. SETTING: Level II Neonatal Intensive Care Unit. SUBJECTS: 100 babies admitted to the neonatal unit during a 4 year period who were below 1500 g or whose gestation was < or = 34 weeks. METHODS: Examination of the eye was done in the neonatal unit or in the neonatal follow up clinic by an Ophthalmologist by indirect Ophthalmoscopy at 4-6 weeks postnatal age. RESULTS: The incidence of ROP was 46%. Of the 100 babies screened, 21 had stage I, 14 had stage II, 8 had stage III and 3 had stages IV and V. The incidence of ROP was 73.3% among < 1000 g babies and 47.3% among < 1500 g babies. The incidence of ROP among 28-29 weeks, 30-31 weeks and 32-33 weeks babies was 83%, 60% and 50%, respectively. The maximum stage of ROP developed between 37-42 weeks post conceptional age in 69% subjects. On univariate analysis, gestation < or = 32 weeks, anemia, Blood transfusions, apnea and exposure to oxygen significantly increased the risk of developing ROP. On multivariate logistic regression analysis, anemia and duration of oxygen therapy were the significant independent predictors of development of ROP. Nine of the 46 babies underwent cryotherapy for threshold ROP. CONCLUSION: The incidence of ROP among high risk babies is significant and duration of oxygen therapy and anemia are independent factors predicting the development of ROP. All high risk babies should be screened for ROP. Cryotherapy is a relatively simple procedure which can be done in the neonatal unit.     

Ethical issues in neonatal paediatrics--the Singapore perspective

In Singapore, formulating ethical guidelines for people who live in a multiracial, multilingual, multicultural and multi-religious community can be difficult. The "individualised prognostic" strategy in the management of critically ill infants has been followed. Our neonatal paediatricians encounter the following ethical problems: extremely premature babies whose viability is doubtful, babies born with severe congenital malformations, babies born with signs of life in legal or therapeutic termination of pregnancy, the asphyxiated babies or babies with severe or extensive brain damage, and babies who are chronically sick and have no chance of recovery or leaving the hospital. Good ethical decisions require medical facts. The infant's diagnosis and prognosis must be accurate. There should also be detailed information that continuation of any form of medical treatment for the infant is futile, will do more harm than good and is inhumane. Ethical decisions should be made in the best interests of the infant. Dating of the infant's gestational age should be accurate and reliable, and there should also be unanimous definitions such as fetal viability, abortions and lethal malformations. Ethical guidelines and the law must also keep pace with changes in medical practice.     

Depression in first-time mothers: Mother-infant interaction and depression chronicity.

Married, middle-class women who met diagnostic criteria for depression and a comparable group of nondepressed women were videotaped interacting with their infants at home at 2, 4, and 6 months. When depression was defined in terms of 2-month diagnosis, there were no differences between depressed and comparison mothers or babies in either positive or negative interaction during feeding, face-to-face interaction, or toy play. However, women whose depressions lasted through 6 months were less positive with their infants across these 3 contexts than women whose depressions were more short-lived, and their babies were less positive during face-to-face interaction. These data highlight the need to distinguish between transient and protracted depression in evaluating depression effects on the mother–infant relationship and infant outcome. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Necrotizing enterocolitis in babies (author's transl)

Newborns and babies weakened in their resistance will mainly be affected with necrotizing enterocolitis. A conservative therapy is principally applied. It is only by increasing signs of peritonitis or caused by perforation of the intestine that laparotomy is required. A report is presented on 11 babies with surgical therapy and the kind of operative procedure applied. 6 of these babies were cured.     

Bacteremia in private pediatric practice

The capillary white blood cell count and differential, a test easily done in office practice, was used in unexplained febrile illness of infants and toddlers to help distinguish those babies most likely to have bacteremia who would benefit from blood culture and early bacteriologic diagnosis. Four criteria were used as indicators suggestive of bacterial infection: white blood cell count greater than or equal to 15,000/cu mm, total segmented neutrophils greater than or equal to 10,000/cu mm, total band cells greater than or equal to 500/cu mm, and total polymorphonuclear leukocytes (segmented neutrophils plus band cells) greater than or equal to 10,500/cu mm. These measurements were found helpful in separating a small group of bacteremic babies at high risk of complications from a large group of babies who recovered without antibiotic treatment and without complications. There were 146 febrile illnesses recorded in babies 3 to 24 months of age; bacteremia was proven in eight of these. Three or four blood cell count criteria were fulfilled in seven of the eight bacteremic babies and in only ten (7.2%) of the remaining 138 febrile illnesses (P < .001 by chi 2 test).     

Decreased plasma cortisol level during alprazolam treatment of panic disorder: a case report

OBJECTIVE: Our goal was to evaluate the impact of fetal compromise on the outcome of borderline viable babies. STUDY DESIGN: All 142 babies born in our hospital from 1990 to 1995 with a gestational age of 23 to 25 weeks were included. Fetal compromise was considered present if one of the following was documented: a major anomaly, congenital sepsis, chronic intrauterine infection, intrauterine drug exposure, congenital anemia, severe growth restriction, fetal acidosis, or cardiorespiratory and neurologic depression in the delivery room. RESULTS: The 43 babies who had at least one cause of fetal compromise had a lower birth weight (p < 0.001), but there were no other differences in demographics or complications of prematurity. The survival rate was significantly better for babies free of fetal compromise (75% vs 33%, p < 0.001), particularly for babies born at 23 weeks of gestation (75% vs 6%, p < 0.001). For surviving babies free of fetal compromise, the outcome at 23 weeks was comparable to that at 24 to 25 weeks for major causes of long-term neurologic morbidity. CONCLUSIONS: Like advancing gestational age and increasing birth weight, the absence of fetal compromise has a major beneficial impact on the outcome of borderline viable babies that might be important when decisions are made about the appropriate level of support.     

Spatial distribution of corticospinal potentials following transcranial electric and magnetic stimulation in human spinal cord

To search for concomitant anomalies among babies with congenital hypothyroidism, 120 newborn babies with confirmed congenital hypothyroidism were studied at the Veterans General Hospital, Taipei. The incidence of concomitant anomalies was estimated to be 11.67% (14/120). Among these anomalies, cardiac and gastrointestinal systems were the most commonly involved, comprising 35.7% (5/14) and 28.6% (4/14) of all anomalies, respectively. The type (i.e. agenesis, ectopia or eutopic goiter) as well as the severity of hypothyroidism were analyzed between groups of babies with or without concomitant anomalies. No differences existed between the two groups of babies regarding these two aspects.     

Hydrocephalus in premature infants

In the light of personal observations from the years 1948-1974 the author discusses the incidence of hydrocephalus in premature babies. The material observed confirms the generally accepted view that the incidence of hydrocephalus is higher in premature babies than in full-term babies. The author analyzes the causes which may be responsible for this finding and concludes that noxious factors during early or late pregnancy are the main cause although perinatal injuries play also a significant role.     

Vertical transmission of hepatitis E virus

Little is known about vertical transmission of hepatitis E virus from infected mothers to their infants. We studied eight babies born to mothers infected with hepatitis E in third trimester. One baby was icteric at birth with elevated transaminases and four babies had anicteric hepatitis. Two babies were born with hypothermia and hypoglycaemia and died within 24 h; one had massive hepatic necrosis. Hepatitis E virus RNA was detected by PCR in cord or birth blood samples of five infants. Six infants had evidence of hepatitis E infection. We conclude that hepatitis E virus is commonly transmitted from infected mothers to their babies with significant perinatal morbidity and mortality.     

Sleep and brain malformation in the neonatal period

Polygraphic recordings of 20 neonates with brain malformations and/or chromosomal anomalies were compared with those obtained in 29 normal fullterm neonates. Seep assessment was made with different methods described in the literature. These methods of sleep scoring gave similar results in the normal newborn babies but discrepant results in many pathological neonates. Thus sleep was also studied by other approaches; assessment of cycling of the 5 parameters (EEG, REM, EMG, Motility, Respiration) and study of the discrepant parameters during a sustained period of Active sleep (AS) or Quiet Sleep (QS), defined by the most flexible method. Newborn babies with brain malformations appeared to be poor sleepers. Amount of wakefulness is higher than in normal neonates. Some babies were nearly insomniac. Correlations of sleep patterns and anatomical lesions were possible in five cases where brain anatomy was available. A good sleep organization has been seen in babies with normal brain-stems but a normal brain stem with a normal locus coeruleus has been found in a baby with absence of AS. Absence of sleep organization has been seen in babies with an abnormal brain stem; but this anomaly was not isolated as these babies also had lesions of the forebrain. The sleep of human neonates shows a greater liability in the coordination of the different parameters than the sleep of human adults or adult animals. It appears that sleep mechanisms at the pontine level present more plasticity than the rigid mono aminergic model would suggest.