儿童反射性无尿7例报告

目的 探讨儿童反射性无尿的诊断与治疗。方法对1995年1月42002年12月收治的7例患儿的临床及随访资料进行回顾性分析。结果7例患儿，男5例，女2例，年龄13d～6岁，均有先天性上尿路梗阻。3例为。肾盂和输尿管术后的患儿，余4例分别发生于下腹部外伤、腹泻、呕吐以及新生儿等状况下。初期1例患儿接受。肾盂成形术，1例行双侧输尿管膀胱再植术；后期2例予肾造瘘，3例予输尿管逆行插管引流，肾功能均恢复。结论上尿路梗阻的患儿易发生反射性无尿，其发生与输尿管痉挛相关，行上尿路引流是简单有效的治疗方法。     

Renal tubular dysgenesis with atypical histology and in-utero exposure to naproxen sodium

Renal tubular dysgenesis (RTD), a rare, lethal, autosomal recessive disorder, is characterised by short and poorly differentiated proximal convoluted tubules associated with oligohydramnios, Potter sequence and neonatal death from respiratory failure. We report an unusual case of neonatal anuria owing to RTD with normally formed lungs, in-utero exposure to naproxen sodium and atypical histology in that the glomeruli were not as crowded as usually seen in RTD. When there is anuria in an infant following birth in the context of a normal renal ultrasound and an absence of objective evidence of perinatal hypoxia, RTD should be considered.     

Adverse effects of drugs on the immature kidney

The immature kidney may be adversely affected by a variety of vasoactive or diuretic drugs, either administered to the mother during pregnancy, or to the neonate. Inhibitors of the angiotensin-converting enzyme administered to the hypertensive pregnant woman can severely and sometimes definitely impair renal function in the fetus, leading to postnatal anuria. Pathogenesis involves interference with the renin-angiotensin system and the prostaglandins. Beta-adrenergic agents administered during labor depress glomerular filtration rate transiently. Tolazoline, an alpha-adrenergic blocking agent useful in the treatment of persistent pulmonary hypertension of the neonate induces intense renal vasoconstriction with consequent hypoperfusion. Indomethacin, a prostaglandin synthetase inhibitor used for the pharmacological closure of a patent ductus arteriosus, also increases renal vascular resistance, and decreases urine output. Furosemide, the drug most often used in oliguric neonates, may also adversely affect the newborn infant. Its use has been associated with an increase in the incidence of patent ductus arteriosus, hypercalciuria, nephrocalcinosis and secondary hyperparathyroidism. These observations demonstrate that the proper use of drugs requires that the therapeutic endpoint be clearly defined and the predictable side effects be anticipated.     

Syndrome of Inappropriate Secretion of Antidiuretic Hormone Associated With Angiotensin-Converting Enzyme Inhibitor Administration

Angiotensin-converting enzyme inhibitors (ACEI’s) are an important medication in the treatment of congestive heart failure. However, ACEIs may cause harmful side effects, such as the syndrome of inappropriate secretion of antidiuretic hormone (SIADH), which is a rare but important side effect. We describe here a case of SIADH associated with ACEI administration in a 6-year-old boy with restrictive cardiomyopathy. After recovery from acute exacerbation of congestive heart failure by tolvaptan administration, an ACEI (cilazapril) was started to decrease the production of angiotensin II, which upregulates serum antidiuretic hormone secretion. The patient’s heart failure symptoms worsened, including accumulation of right pleural effusion and ascites, after the initiation of ACEI administration. Cessation of ACEI administration dramatically improved his symptoms. Because it is difficult to distinguish SIADH associated with ACEI from worsening congestive heart failure, the possibility of fluid retention due to ACEI administration should always be considered when this agent is administered to patients with heart failure.     

Efficacy of low-dose dopamine infusion

Urine output following administration of a low-dose dopamine infusion was assessed in 20 very immature infants (median gestational age 27 weeks). Prior to the infusion, all infants had had a period of anuria. Urine output improved significantly during the second 24 h after commencing the infusion but, at that time period, urine output was greater than 2 ml/kg/h (designated a good response) in only 13 infants. There was no significant difference in gestational age, birth weight, period of anuria or fluid input of infants who had a good or a poor response to dopamine. Although the baseline blood pressure did not differ significantly between these two groups, the increase in blood pressure resulting from dopamine administration was significantly greater in those infants with a good response in urine output (p<0.02). We conclude that low-dose dopamine infusion can improve urine output in very immature infants. Our results suggest that there may be inter-individual variation in the sensitivity to dopamine.     

Anuria in childhood due to bilateral urolithiasis. A report of four cases.

Four children with anuria due to occlusion of both ureters by calculi are presented. A plain film of the abdomen revealed faintly radiopaque urinary calculi bilaterally in three patients. In the fourth case, one calculus was visualized in the right ureter, but further contralateral exploration showed an obstructing ureteral calculus in the left ureter as well. It is concluded that in children with acute anuria, the possibility of bilateral ureteral obstruction due to calculi should be the physician's first consideration.     

Prognosis of hemolytic uremic syndrome in children. Importance of extrarenal involvement

The prognosis of hemolytic uremic syndrome was studied in 37 children hospitalized between January 1980 and January 1985. 75% of affected children were less than 3 years of age. Twenty-two (60%) presented with anuria or oliguria (mean duration of anuria: 10.7 days). In this last group, severe extrarenal manifestations occurred: 12 cases of neurological involvement (6 involving hypervolemia), gastrointestinal involvement in 5 cases, including a case of ileal necrosis; pancreatic involvement in another. Three children died during the first month of the disease (overall death-rate: 8%). Thirty-two children were followed with a mean 24 months follow-up (6 months to 60 months). Six (19%) presented with sequellae, of which 3 were severe: one severe arterial hypertension and two with chronic renal failure of which one terminal with severe neurologic sequellae. The existence of extrarenal manifestations implies a poor prognosis. Among the 22 children with anuria or oliguria, 8 had no extra-renal manifestations. All of these recovered. Three of 11 children with one extrarenal manifestation had an unfavorable outcome. The course was unfavorable in the 3 cases with multiple extrarenal involvement.     

Long-term outcome and prognostic indicators in the hemolytic-uremic syndrome

We examined 61 patients an average of 9.6 years (range 5 to 18 years) after an episode of childhood hemolytic-uremic syndrome. Twenty-four (39%) had one or more abnormalities. Seven (11%) had proteinuria and six (10%) had low creatinine clearance as solitary abnormalities. Eight (13%) had both proteinuria and reduced creatinine clearance; three (5%) had a combination of hypertension, proteinuria, and low creatinine clearance. Abnormalities sometimes appeared after an interval of apparent recovery. Logistic regression analysis showed that duration of anuria was the best predictor of disease at follow-up. No patients who had anuria lasting longer than 8 days or oliguria exceeding 15 days escaped chronic disease. However, 45% of those with disease had no anuria, and a third had no oliguria. Physicians should therefore be cautious in assuming recovery from HUS on the basis of a single evaluation and should periodically evaluate patients for an extended period.     

Prevalence, prevention, and treatment of microalbuminuria and proteinuria in children with sickle cell disease

Microalbuminuria (MA) and proteinuria (P) are believed to be precursors of sickle cell nephropathy. We analyzed our longitudinal data on MA/P in children with sickle cell disease (SS) to define the age of onset, association with age, sex, and hemoglobin, and to explore the safety and efficacy of hydroxyurea and angiotensin converting enzyme inhibitor (ACEI) therapy. Data on 191 patients with SS (ages 3 to 20 y) with a mean follow up of 2.19 years+/-2.05 were available. Urine MA was measured yearly with follow-up testing if abnormal. Prevalence of MA/P was 19.4%. Increasing age and lower hemoglobin levels were related to MA/P but sex was not. Microalbumin excretion normalized in 44% of patients treated with hydroxyurea and 56% of patients treated with ACEI. Hyperkalemia developed in 4 ACEI patients resulting in discontinuation of treatment in 3 children. In summary, MA/P often develops in childhood and preventive and treatment strategies for sickle cell nephropathy should be a focus of pediatric programs. Our preliminary data suggest that although both hydroxyurea and ACEI therapy may be beneficial for MA/P, hyperkalemia may limit the utility of ACEI.     

COMBINED PLASMAPHERESIS AND HEMODIALYSIS TREATMENT FOR SEVERE HEMOLYTIC-UREMIC SYNDROME FOLLOWING CAMPYLOBACTER COLITIS

ABSTRACT. A case of severe hemolytic-uremic syndrome (HUS) with long lasting anuria in a 7 month old patient is described. The patient failed to improve until treated with combined plasmapheresis and hemodialysis. Declining titres against Campylobacter jejuni/coli suggest that this was the causal agent. We feel that plasmapheresis should be tried in severe HUS with prolonged anuria, which has a poor prognosis with conventional treatment.     

Normal Outcome Following Administration of Gonadotropin-Releasing Hormone (GnRH) Agonist during Early Pregnancy

We observed an unusual case of a woman who had received a gonadotropinreleasing hormone (GnRH) agonist (GnRH-a), leuprolide acetate, during the first trimester and early in the second trimester of pregnancy. Nevertheless, the pregnancy was uncomplicated and a healthy male infant (2,670 g) was born after spontaneous labor at the 38th week of gestation. The infant suffered no malformations or respiratory complication and he achieved normal growth. Although GnRH-a is known to induce pregnancy loss, the drug does not appear to be teratogenic. Accordingly, a pregnancy that occurs during GnRH-a therapy and progresses normally should not be terminated for fear of its teratogenic effects.     

Renal tubular dysgenesis-a case presentation

Renal tubular dysgenesis (RTD) is a lethal, developmental anomaly of the fetal kidney characterized by a defect in differentiation of the proximal and distal convoluted tubules. It is usually associated with oligohydramnios in later pregnancy and Potter's syndrome. A neonate with typical features who presented with mild respiratory distress, dysmorphic appearance and anuria is described. At the age of seven days, peritoneal dialysis was started and was continued until the death of the baby at the age of three months. The diagnosis was made on the bases of clinical and ultrasonographic findings confirmed by renal biopsy. A review of the literature showed that this is the first case of RTD reported in Turkey.     

Maternal cigarette smoking during pregnancy, low birth weight and subsequent blood pressure in early childhood

Given the widely acknowledged inverse relationship between birth weight and blood pressure, a raised blood pressure in the offspring of smoking mothers as compared to those whose mothers did not smoke, would be anticipated by virtue of the reduction in birth weight associated with smoking during pregnancy. The objective of the present study was to test the hypothesis that maternal cigarette smoking during pregnancy has an effect on blood pressure in childhood independent of its effect on birth weight. Data was obtained from a prospective cohort study of 1708 pregnant women and their singleton offspring, delivered live at term, in Perth, Western Australia, commenced at 16 weeks gestation with serial blood pressure measurements through early childhood. Statistically significant associations were found between maternal smoking during pregnancy and systolic blood pressure at age six, between birth weight and systolic blood pressure at ages three and six, and between maternal smoking during pregnancy and birth weight. The relationship between birth weight and blood pressure in early childhood differed significantly on the basis of maternal cigarette smoking or not during pregnancy. This differential relationship persisted after adjustment for the child's current weight and socio-economic status. We concluded that intra-uterine exposure to maternal cigarette smoking increased children's blood pressure at age one through to age six. This was not wholly attributable to an effect on birth weight or confounding of the association between birth weight and subsequent blood pressure by the child's current weight or socio-economic factors. Furthermore, maternal smoking during pregnancy does not account for the acknowledged elevation in blood pressure associated with low birth weight. The present study is an exploration of a possible causal pathway underlying the birth weight/blood pressure association rather than simply a confirmation of such an association which has been detailed in many other papers.     

Generalized oedema of newborn associated with the administration of dipyrone

In fourteen infants, aged 9–60 days, with generalized oedema seen during a one year period the common denominator was the administration of dipyrone one to two days prior to the development of oedema. None of the other causes of oedema in early life could be incriminated in any of these babies. Pediatricians should be aware of this iatrogenic cause whenever they encounter a young infant with generalized oedema. Oedema disappeared in all the cases following discontinuation of dipyrone but anuria lasted for more than four days in one case. This study re-emphasizes the need to withold this potentially dangerous drug, especially during the neonatal period.     

Maternal-fetal relationships in the parathyroidectomized rat. Intestinal calcium transport, serum calcium, immunoreactive parathyroid hormone and calcitonin

We studied the role of the parathyroids in the adaptation of intestinal Ca transport that occurs during pregnancy, and whether maternal hypoparathyroidism causes fetal hyperparathyroidism. Serum Ca of pregnant parathyroidectomized (PTX) rats was significantly greater than nonpregnant, PTX animals. Intestinal active Ca transport was increased 2.1- and 2.2-fold by pregnancy in intact and PTX rats, respectively. Serum levels of immunoreactive parathyroid hormone (PTH) were nondetectable in PTX-pregnant rats. Fetuses from PTX rats appeared grossly normal. The serum PTH was not different in fetuses from PTX compared to fetuses from intact mothers and serum Ca, Mg, and P were normal. Thus, alleviation of maternal hypocalcemia during pregnancy in PTX rats may be due to an adaptive increase in intestinal Ca transport, which does not require the parathyroids. Fetuses from PTX mothers were euparathyroid and were protected from Ca deficiency during pregnancy.     

Labeled Lectin Studies of Renal Tubular Dysgenesis and Renal Tubular Atrophy of Postnatal Renal Ischemia and end-Stage Kidney Disease

Renal tubular dysgenesis (RTD), with hypoplasia especially of renal proximal convoluted tubules and clinical neonatal anuria or oliguria, has been reported as a congenital familial (autosomal recessive) disease, variably with features of oligohydramnios, Potter syndrome, or pulmonary hypoplasia. A similar tubular lesion due to antenatal tubular atrophy has been reported for conjoined twins with twin-twin transfusion syndrome or acardia and in infants of mothers given antihypertensive agents, including angiotensin-converting enzyme (ACE) inhibitors, during pregnancy, and it has been seen as a unilateral lesion in young infants with renal artery stenosis due to arteritis or medial arterial calcinosis. The renal tubular changes in RTD are very like those of the “endocrine kidney” in experimental animals and resemble those of the renal tubular atrophy of end-stage kidney diseases such as glomerulonephritis, tubulointerstitial kidney disease, obstructive uropathy/pyelonephritis, graft rejection of transplanted kidneys, or the renal parenchymal changes seen with protracted dialysis therapy. Labeled lectins that differentially mark proximal convoluted, distal convoluted and connecting, and collecting tubules showed no distinctive differences in stainingpatterns of the hypoplastic renal tubules of infants and children with RTD, postnatal renal artery obstruction, or the various types of end-stage renal disease with the lectins used (PNA, GSL1, UEA, and LTA). The findings suggest that the renal tubular changes in some if not all the conditions studied are the result of renal ischemia. The reported familial RTD with hypernephronic nephromegaly may be a specific disorder, but other forms could reflect renal ischemia acquired in utero or in early or later postnatal life.     

Exanthematic diseases during pregnancy and attention-deficit/hyperactivity disorder (ADHD).

Rash causing viral diseases may be transmitted during pregnancy, causing severe congenital disease. Although neurological and psychiatric disorders are common consequences of congenital rubella, children born to women who developed a viral rash during pregnancy do not appear to be at increased risk of these disorders if they were asymptomatic at birth. In a case-control study conducted to evaluate risk factors for ADHD, we found an increased risk of this disorder among children born to women experiencing a viral rash during pregnancy. The viral rash (i.e. measles, varicella, or rubella) was reported by 4 of 71 mothers of children with ADHD and none of the 118 controls' mothers (P<0.01). The difference remained statistically significant after adjusting for potential confounders (i.e. other factors found associated with ADHD, such as gender and familiarity). Although, the viral disease reported by the mothers, in accordance with their physician's diagnosis, did not represent a homogeneous nosological group, the unexpectedly high rate found among ADHD cases' mothers suggest a role for viral diseases occurring during pregnancy in the development of ADHD.     

Moderate alcohol consumption during pregnancy and child development

On the basis of prospectively collected data from 7,525 pregnancies it was examined whether moderate but daily alcohol consumption during pregnancy has consequences for child development during the first 3 years. The women were taken into the study during the first trimester of pregnancy and were seen every 4 weeks. Some 4.7% (353) of the total stated that they drank moderate quantities of alcohol every day; 275 of the 353 pregnant women stopped drinking during the first trimester of pregnancy or for a time, but 78 did not change their drinking habits in general. About 75% of the children were followed up to their 3rd birthday with examinations at birth, 6 weeks, and 9, 18 and 36 months. It was found that in growth and psychomotor development the exposed children did not differ from the the control group. The number of minor and major congenital anomalies was not increased.In general, moderate alcohol consumption during pregnancy does not seem to have any markedly adverse influence on the later development of children. However, an upper limit for apparently safe alcohol consumption cannot be given.Dedicated to Prof. Dr. H.-R. Wiedemann on the occasion of his 65 th birthday     

Drugs controlling proteinuria of patients with Alport syndrome

Background

Proteinuria is one of the risk factors for the progression of renal diseases including Alport syndrome (AS), a hereditary glomerular renal disease. This study aimed to evaluate the efficacy of angiotensin converting enzyme inhibitors (ACEIs) and/or tripterygium, a Chinese herbal medicine widely used in Chinese patients with hematuria and proteinuria, on proteinuria in patients with AS.

Methods

Twenty-nine children were enrolled into this retrospective study. Patients were divided into 3 therapy groups: ACEI group, tripterygium group, and ACEI plus tripterygium group.

Results

In the 29 children, 23 were male and 6 female. In the ACEI group and the tripterygium group, the effective rate was 87.5% and 25.0%, respectively and in the ACEI plus tripterygium group was 42.9%.

Conclusions

ACEI is effective in controlling proteinuria of AS patients. Tripterygium should be carefully administered in controlling proteinuria of AS patients.