Pediatric transplantation of the kidney, liver and heart: summary report

The following is a summary report of an extensive review of the literature from 1966 to 2001 on growth and development in children receiving kidney, liver and heart transplants. The literature was assessed for relevancy to current clinical practice and for reliability and generalizability of the inferences based on the study design, controls, sample size, age distribution, confounding factors, use of standardized instruments, and consistency with other findings. While studies on growth are included in the review, the main emphasis is on research in cognitive and psychosocial development since these areas have been far less thoroughly studied and contain various methodological deficiencies. On the basis of the literature review both general methodological recommendations and specific recommendations for future research studies are made. Access to the full is provided on the World Wide Web at http://light.emmes.com/pedstransplantation/.     

Traitement psychomoteur de la carence en milieu institutionnel. Soutenir le développement du jeune enfant dans le cadre d’une pouponnière de l’Aide Sociale à L’Enfance

When a young child (from 0 to 3 years old) is taken away from his family due to sanitary, emotional, or educational deficiencies, the Children's Social Aid generally places him in a nursery. It is in this antechamber that he will wait until justice and social services find an “educational solution” more favorable to his development. In this context of community, it is not uncommon for the child to freeze, an anxious observer of an environment in perpetual movement, thus putting development and acquisitions on hold. The care of the psychomotricist care then has two successive objectives: firstly, to support the emergence of self-confidence, and secondly, to allow physical experiences likely to remobilize the child in the pursuit of his development.     

Rational use of antibiotics in children with diabetic ketoacidosis needs attention

Diabetic ketoacidosis (DKA) in children may lead to acute kidney injury (AKI). Among 45 children with DKA in our center, eight cases had AKI on admission, and in one child, his kidney function did not recover until 3 mo after discharge. This child was treated with antibiotics (cephalosporin), and we cannot rule out delayed AKI recovery due to the combined effects of the drug and the disease. Pediatricians should be concerned about the impact of nephrotoxic drug and disease interactions on children's kidney function, and need to follow up children with DKA and AKI to determine the development of AKI.     

MicroRNA-21在肾脏疾病中的研究进展

MicroRNA是一类非编码小分子RNA,通过与靶mRNA的互补配对,在转录后水平上对基因的表达进行负调控,导致mRNA的降解或翻译抑制,从而对许多病理生理过程产生影响.MicroRNA-21是近年来发现的典型的microRNA,与急性肾损伤、IgA肾病、肾细胞癌等肾脏疾病的发生、发展及预后密切相关.因此,microRNA-21有可能是未来针对这些疾病诊断和治疗的新靶点.该文就microRNA-21在肾脏疾病中的研究进展进行综述.     

ADAM–A SEVERELY-DEPRIVED COLOMBIAN ORPHAN: A CASE REPORT

The case study of Adam, a severely-deprived Colombian orphan, is presented. At 16 months of age the child was suffering from severe marasmic malnutrition, his development was about the level of a 3-month old, he had been physically neglected and was emotionally withdrawn. Adam was adopted at 34 months of age. At 8 years of age his Full-Scale WISC-R-IQ was 113 and at 10 years of age he scored 100% on the Michigan Assessment Test. Now at 13 years 10 months he is functioning at the 8th grade school level, which is appropriate for his age, and his weight and height are in the 50th centile range.     

Successful living‐related kidney transplantation in a boy with inherited dysfibrinogenemia

In kidney transplantation, it is essential to avoid acute vascular complications, such as hemorrhage and renal vascular thrombosis, which may often lead to allograft loss. Inherited dysfibrinogenemia is a rare coagulation disorder with a wide spectrum of clinical manifestations, such as excessive bleeding and thrombosis. A 12‐yr‐old boy, previously diagnosed with renal hypodysplasia, was found to have reduced fibrinogen concentrations. Coagulation tests assessing surgical risk during kidney transplantation showed a discrepancy between functional and immunologic fibrinogen concentrations. Gene analysis confirmed inherited dysfibrinogenemia, with a heterozygous mutation in FGA (Aα Arg16His) in the patient and his mother. Based on the molecular and functional properties of the mutation, and a familial phenotype, in which his aunt had experienced a previous bleeding episode, the patient was considered at greater risk of bleeding than of thrombosis. The patient was administered fibrinogen concentrate before surgery, and kidney transplantation was performed with his father as the organ donor. The patient received additional prophylactic infusions of fibrinogen concentrate postoperatively, and his postoperative course was uneventful. Accurate diagnosis of dysfibrinogenemia, including gene analysis, is important for correctly managing patients with this coagulation disorder who are undergoing kidney transplantation.     

A validation study of the Norwegian version of the Ages and Stages Questionnaires

Aim: To explore the construct validity of the ASQ in relation to premature birth; maternal age, completeness of the family and level of education; and the child's gender.
Methods: Norwegian mothers' evaluations of 1172 children who completed age appropriate ASQs twice were statistically analysed by means of univariate and multivariate methods.
Results: The developmental level of girls is on average higher than that of boys at both assessments and there is a substantial increase between follow-up assessments in all ASQ scores except for gross motor skills. The mother's level of education; whether the child was born prematurely or at full-term; and the interaction between the child's gender and his/her mother's level of education are important factors relating to the child's developmental status in both assessments.
Conclusion: The results that were derived by means of the Ages and Stages Questionnaires concerning the impact of maternal socio-demographical factors, the child's gender, and premature birth upon early childhood development largely confirm the findings in the literature supporting the construct validity of the ASQ. The ASQ was found to be an effective diagnostic tool of developmental delay and/or disturbances. The development of gender dependent norms is recommended.     

Failure of isolated kidney transplantation in a pediatric patient with primary hyperoxaluria type 2

PH type 2 is caused by decreased activity of GRHPR enzyme that eventually leads to ESRD and systemic oxalosis. Here, we describe an Iranian pediatric patient with PH2 and early ESRD development who received recommended treatment by undergoing isolated kidney transplantation. Diagnosis criteria included a history of reoccurring calcium oxalate renal stones and elevated oxalate levels combined with liver biopsy and decreased enzymatic activity at age five. ESRD prompted transplantation and was performed at age nine. On Day 12 post‐op, his serum creatinine level increased. A graft biopsy showed calcium oxalate crystal deposits in renal tubes with no evidence of acute rejection, which resolved with intensive hydration and administration of a potassium citrate solution. Subsequent biopsies confirmed results found in first biopsy. Despite the immunosuppressive therapy, his serum creatinine level increased again after 11 months. Renal tubular obstruction then led to graft nephrectomy. Pathological analysis of tissue confirmed findings of past biopsies. This was a very rare case of early ESRD in PH2 resulting in a failed isolated kidney transplant. As the GRHPR enzyme is predominantly expressed in liver, we suggest a combined liver‐kidney transplant may be beneficial in patients with PH2.     

Growth and development of the human fetal kidney

Growth and development of human fetal kidney was studied with respect to anthropometric measurements, histological appearance and certain functional parameters. Upto 30 weeks of gestational age there was a proportional growth of the kidneys in respect of gestational age. The formation of the glomerular capsule was by a process of sequential development of the endo and epithelial cells rather than by an invaginative process. There comes a time in the development of the kidney when the developing nephrons appear to lie in pools of blood. Glomerular filtration appears earlier than tubular reabsorption. The process of tubular secretion was found to be associated with induction of specific proteins.     

Gene targeting in kidney development

Cancer and development are conceptually related because tumor formation in many cases results from the aberrant expression of a developmental program. This is certainly true of Wilms' tumors, which display a range of phenotypes resembling various stages of kidney development. WT1 has been identified as a tumor suppressor gene involved in a subset of Wilms' tumors. Gene targeting of the WT1 gene demonstrated the requirement for this gene product during early urogenital development. Several other genes, including Wnt-4, c-ret, Id and lim 1, have been shown by gene targeting to also be involved in early kidney development. This review discusses gene targeting as an approach to the study of development and reviews the phenotypes of these and other genes involved in kidney organogenesis. © 1996 Wiley-Liss, Inc.     

Invasive disease caused by Streptococcus pyogenes. Report of a case with cutaneous and kidney metastasis

Few cases of invasive disease due to Streptococcus pyogenes in children have been described. Greater knowledge of this disease is needed due to its increasing incidence and seriousness. New serotypes have been discovered with greater virulence due to exotoxin production and to the development of resistance to phagocytosis. We present a case of invasive disease due to S. pyogenes with bacteremia and cutaneous and kidney metastasis, in a non-immunodeficient child. The case is of interest because kidney dissemination has not been described in the literature and because evolution was satisfactory with endovenous beta-lactamica treatment during 15 days for only 48 hours. The importance of ruling out multi-organ metastatic foci, including use of urine cultures to rule out kidney involvement are highlighted. The epidemiological, pathogenic, clinical and diagnostic aspects of this entity, as reported in recent literature, are described.     

The early development of a thinking mind: a one-year infant observation

The author uses the one-year infant observation to illustrate the development of his mind and imagination, and tracks early signs of the development of the capacity to think. This is related to many good experiences with his mother, the primary carer and father, which he internalises over time, in identification with them. Bion's Theory of Thinking and Klein's work on the epistemophilic instinct both link with the capacity to tolerate being a separate person and to make use of internalised experiences and objects when carers are not present. The baby boy's mental development over one year is impressive; this is linked to his mother's capacity to think about him which is linked with an evident experience of feeling understood as well as cared-for.     

Synovial sarcoma in a premature newborn

Synovial sarcoma is rarely detected in infants, with an annual incidence of 0.5 per million. Synovial sarcoma occurs more frequently in adolescents and young adults, with the majority of patients presenting between 15 and 40 years of age. It is extremely rare, however, in pediatric patients under 2 years of age. In the present study we examined a 3‐day‐old male infant born at 32 weeks who had a mass on his left arm. Synovial sarcoma was identified on histopathological and immunohistochemical analysis of biopsy material acquired from the mass. On whole body magnetic resonance imaging, diffuse metastases were detected in the bilateral lungs in the retroperitoneal zone, in bilateral suprarenal glands, the right liver lobe, the right kidney, and the brain. To our knowledge this is the youngest patient to be diagnosed with synovial sarcoma in the literature.     

托莫西汀在注意力缺陷多动障碍患儿中的精准药学研究：CYP2D6基因检测和治疗药物监测

约3%的孕妇患有慢性肾脏病（chronic kidney disease,CKD）。该文复习了关于CKD母亲（包括透析和肾移植患者）的新生儿结局的文献。文献显示：妊娠合并CKD会增加新生儿发生早产、低出生体重及小于胎龄儿的风险,但不增加发生先天结构畸形的风险;从远期结局来看,对子代体格发育、免疫功能无显著影响;子代的神经发育结局与早产、低出生体重相关,与宫内药物暴露无关。仍需更进一步的研究及随访以探讨CKD母亲的新生儿结局。     

慢性肾脏病母亲的新生儿结局

约3%的孕妇患有慢性肾脏病（chronic kidney disease,CKD）。该文复习了关于CKD母亲（包括透析和肾移植患者）的新生儿结局的文献。文献显示：妊娠合并CKD会增加新生儿发生早产、低出生体重及小于胎龄儿的风险,但不增加发生先天结构畸形的风险;从远期结局来看,对子代体格发育、免疫功能无显著影响;子代的神经发育结局与早产、低出生体重相关,与宫内药物暴露无关。仍需更进一步的研究及随访以探讨CKD母亲的新生儿结局。     

Malignant hypertension in a child with solitary functioning hydronephrotic kidney

A child with solitary functioning hydronephrotic kidney and hypertensive encephalopathy is described. Removal of the contralateral dysplastic kidney failed to normalize his blood pressure.     

Molecular Mechanisms of Human Embryogenesis: Developmental Pathogenesis of Renal Tract Malformations

The focus of this review is the normal and abnormal development of the kidney and lower urinary tract; for convenience, we will refer to the whole system as the renal tract. The content represents a convergence among the clinical disciplines of histopathology, nephrology, and urology as well the basic sciences of developmental biology and molecular genetics. The story has considerable clinical relevance since diverse renal tract malformations are increasingly detected on fetal ultrasound screening and constitute major causes of chronic renal failure necessitating dialysis and kidney transplantation in children. Evidence is emerging that at least some of these disorders have a defined genetic basis; in others, an abnormal embryonic, or even maternal, environment may contribute to the pathogenesis. This field of study is frequently updated, with new discoveries being made almost every week. Hence this review can not be exhaustive or definitive, but instead highlights some specific areas of interest. Received July 1, 2001; accepted September 1, 2001.