From Relevant Capabilities to Relevant Indicators: Defining an Indicator System for Children’s Well-Being in Spain

In the last two decades, studies dealing with the measurement of children’s well-being have proliferated. These studies develop mainly from the need to address the topic from a multidimensional perspective, capable of integrating approaches into a more comprehensive view of reality. In this regard, key issues have been tackled and discussions are still open, such as those on the inclusion of boys and girls as active agents in the definition of their needs, or on the consideration of aspects that affect both present and future needs as part of well-being. The capability theory sets a very interesting theoretical framework within this context. This work will, first of all, try to approach the topic of children’s well-being from such perspective in conjunction with the Life Sustainability proposition. There is, however, no translation of these theoretical contributions to the development of indicators, even though the actual need for them is well acknowledged, given the political and social-action implications of bringing this progress to the realm of the tangible. There are two main reasons for this. First, the scarcity of data about children that could allow the empirical development of valid and reliable measurements in this field. Second, the methodological difficulty of appropriately defining this kind of factors, which are very often linked to subjective and/or intangible aspects, for quantification purposes. Keeping all this in mind, the second part of this work aims at making some progress in this direction and proposes a system of indicators to support what is first analyzed from a conceptual point of view.     

Recent advances in minimal change nephrotic syndrome

The etiology of MCNS remains an enigma. Increase in the permeability of the glomerular capillary filter to plasma proteins results in massive proteinuria and hypoalbuminemia which are the hallmarks of this disease. The cause of this increased permeability remains to be ascertained. Research in pursuit of this goal has led to the accumulation of fragmentary data on the morphological abnormalities, immunological dysfunction and metabolic changes that accompany this disease. In our experience, immunology dysfunctions, abnormalities of lipid profile and the deposition of IgM in the mesangium as detected by immunofluorescent microscopy are some of the unusual features of MCNS. The therapeutic implications of these findings and some of the other recent advances are discussed in this article.     

Shared dilemmas of research and practice in developmental and behavioral pediatrics: introduction to the special issue on methodology.

Research in developmental and behavioral pediatrics is characterized by special methodological problems involving measurement, design, and analysis related to research questions, populations, and nature of interventions in this field. To sensitize researchers and practitioners to these problems and to enhance the quality of research and critical consumption of research reports, this special issue provides a summary of basic methodological issues in developmental and behavioral pediatrics. Advances in this field will require the kind of critical observation and decision making that are shared by researchers and practitioners and are illustrated by contributions to this issue.     

Parenteral nutrition-associated cholestasis

Cholestasis is a major complication that occurs frequently in patients with the short bowel syndrome and accounts for the majority of morbidity and mortality in this group of patients. The exact cause of this condition is not known and the etiology is likely multifactorial. Many new mechanistic insights into this disease are discussed and have paved the way for future investigation. For now, prompt recognition, early initiation of enteral feeding, prevention of overfeeding with parenteral nutrition, and agents that induce bile flow may be useful to prevent this catastrophic morbidity.     

Sudden death caused by cardiac tamponade

Central venous catheters today can be inserted with a remarkable degree of ease due to advances in catheter design and cannulation techniques. This is partly responsible for the increase in the use of this form of venous access. Because of this increase, the occurrence of less well-known complications of central venous cannulation are predicted as illustrated by this report on three infants in whom centrally placed catheters penetrated the pericardial sac, causing tamponade. This complication was associated with the use of one particular type of cannula. The manner in which this described hazard was first detected is highlighted. The realisation that tamponade is not entirely preventable and that the clinical manifestations there of are variable makes the exclusion of a hydropericardium by diagnostic tap an essential step during the resuscitation of any patient who collapses unexpectedly with a central venous catheter in place. The recognition of this fact has altered the poor prognosis previously associated with this complication.     

Arrhythmogenic right ventricular dysplasia in a child with congenital enteropeptidase deficiency and hypogammaglobulinaemia

A case of arrhythmogenic right ventricular dysplasia in a 10 year old girl is described which provides some evidence for an inherited aetiology of this unusual form of heart disease. The parents of this child were first cousins, thus increasing the possibility of inherited disorders in their offspring. She had been known from infancy to have the rare disorder of congenital deficiency of intestinal enteropeptidase, and low serum immunoglobulins G and A. An untyped adenovirus was grown from a myocardial biopsy taken early in the course of her cardiac disease. However, it is unlikely that this virus was a major factor in the aetiology of her cardiac disease. Both the cardiac and intestinal diseases are now commonly believed to result from hereditary factors, and this report provides further support for this view.     

Cerebral venous sinus thrombosis: report of a case and review of the literature

Cerebral venous sinus thrombosis is a condition that is well documented as a complication of the latter stages of pregnancy and with an even greater incidence during the puerperium. More recently this entity had been associated with the use of oral contraceptives. A case is presented of a 15-year-old girl with this condition, in whom the occurrence as well as the resolution of a superior sagittal sinus thrombosis was demonstrated with serial computed tomography (CT) scans and radionuclide studies. The discussion of this case, coupled with a review of the literature, describes the clinical presentation, explores the possible etiology, offers a noninvasive technique for confirming the diagnosis, and presents the medical management of this condition. It is postulated that cerebral venous sinus thrombosis occurs with a greater frequency than is currently recognized. Health professionals involved with monitoring the health care of adolescents during pregnancy and in the puerperium should be aware of this potential complications. In addition, association of this condition with oral contraceptive use should be recognized.     

Infantile glaucoma associated with the Diamond-Blackfan syndrome.

Diamond-Blackfan syndrome is a rare congenital hematologic disorder characterized by isolated erythroid hypoplasia. Physical abnormalities such as short stature, thumb deformities, and ophthalmic findings including strabismus, hypertelorism, and microphthalmos have been reported to occur with this disease entity. We describe two patients with this blood dyscrasia and infantile glaucoma with trabeculodysgenesis. Both patients underwent multiple surgical procedures to control their glaucoma. The patients were on chronic systemic adrenal corticosteroid therapy for their anemia, which caused some confusion regarding the etiology of their glaucoma. To our knowledge, this is the first report of an association of infantile glaucoma with Diamond-Blackfan syndrome. We hope this report will encourage early recognition and treatment of glaucoma in patients with this disorder.     

Renal medullary carcinoma: prolonged remission with chemotherapy, immunohistochemical characterisation and evidence of bcr/abl rearrangement

BACKGROUND: Renal medullary carcinoma (RMC), an extremely rare tumour of the kidney, carries a dismal prognosis, with no reports to date of significant response to chemotherapy or radiotherapy. A case of this tumour in a male child, who showed a dramatic response to chemotherapy, is described. PROCEDURE: A detailed histological evaluation of the tumour and cytogenetic analysis using fluorescent in situ hybridisation (FISH) was carried out. The child was treated with multiagent chemotherapy, followed by abdominal radiotherapy. RESULTS: A detailed histopathological and immunohistochemical portrait of this tumour is described, and FISH studies confirmed the presence of a bcr/abl rearrangement. The child obtained complete radiological remission following chemotherapy, although he later relapsed and died of progressive disease despite further attempts at treatment with chemotherapy. CONCLUSIONS: Although there are no previous reports of response of this tumour to chemotherapy, this case illustrates that treatment of this disease is justified. The responses of other cases to similar drug regimens would be of interest to confirm whether the encouraging response described for this case could be reproduced. Cytogenetic analysis of other cases of RMC may clarify whether the abnormalities seen in this case are typical.     

Amnion degeneration over fetal placental surface vessels possibly resulting from focal hypoxia: a case report.

The monoamnionic placenta of this twin gestation had focal amnion necrosis, but this was present only over the fetal surface vessels of one twin; this twin also developed cerebral atrophy. We hypothesize that this degeneration is due to a more severely reduced oxygen tension in its vessels. The placental amnion epithelium may undergo several degenerative processes, including amnion nodosum and changes due to meconium staining. Sonography had disclosed what appeared to be a dividing membrane, but this was not found at birth when monoamnionic twins with entangled cords presented. The amnion degeneration was present only over the large surface fetal vessels of the placenta of that twin who also developed central nervous system degeneration, and macrophage infiltration was confined to the same lesions. Focal hypoxia from entangling cords may have caused this defect.     

Linear programming: a mathematical tool for analyzing and optimizing children's diets during the complementary feeding period

During the complementary feeding period, children require a nutrient-dense diet to meet their high nutritional requirements. International interest exists in the promotion of affordable, nutritionally adequate complementary feeding diets based on locally available foods. In this context, two questions are often asked: 1) is it possible to design a diet suitable for the complementary feeding period using locally available food? and 2) if this is possible, what is the lowest-cost, nutritionally adequate diet available? These questions are usually answered using a "trial and error" approach. However, a more efficient and rigorous technique, based on linear programming, is also available. It has become more readily accessible with the advent of powerful personal computers. The purpose of this review, therefore, is to inform pediatricians and public health professionals about this tool. In this review, the basic principles of linear programming are briefly examined and some practical applications for formulating sound food-based nutritional recommendations in different contexts are explained. This review should facilitate the adoption of this technique by international health professionals.     

Chemotherapy-associated eccrine hidradenitis: neutrophilic eccrine hidradenitis reevaluated: the role of neutrophilic infiltration

Two cases of chemotherapy-associated eccrine hidradenitis are presented. Previous cases have been labeled "neutrophilic eccrine hidradenitis" (NEH). Both cases reported herein involve neutropenic patients and are notable for the absence of neutrophils on histologic examination. Oncologists need to be aware of this newly described process, and this rash must be added to the differential diagnosis of the neutropenic oncology patient. The role of neutrophils in this disorder is discussed, and a new term, "chemotherapy-associated eccrine hidradenitis," is suggested for this unusual process.     

Unique diagnostic features and successful management of a patient with disseminated lymphangiomatosis and chylothorax

Disseminated lymphangiomatosis is a rare vascular tumor characterized by a proliferation of abnormal lymphatic channels that often involves multiple organ systems. One particularly morbid manifestation of this disorder is the presence of bony lytic lesions with associated chylothorax. Because of its unusual nature, this condition is often a diagnostic and therapeutic challenge. In this report, we present the diagnostic features, including a unique radiologic finding, and successful management of a 7-year-old girl with this condition using a combination of aggressive surgery and medical treatment with interferon and pamidronate.     

Multidimensional Adherence Classification System: initial development with adolescent transplant recipients

Abstract:  As transplantation has progressively become a more viable option for children with life-threatening illness, ensuring that adolescents do not lose their new organ secondary to medication non-adherence is paramount. The first step to addressing non-adherence is adequate assessment of this construct. In this investigation, we introduce the MACS. The MACS includes self-report and drug assay levels. Self-report is a subjective measure with a low false-positive rate, but is vulnerable to social desirability. Drug assays are an objective measure of drug ingestion, but values suggestive of non-adherence may be influenced by medical complications and timing. The MACS builds on the strengths of both methods and attempts to contain their weaknesses. The sample in this study consisted of 82 adolescent solid organ transplant recipients. The non-adherence rate using the MACS in this sample was 61%. Initial data to support this system are promising. The occurrence of rejection episodes and mortality were significantly related to membership in the Genuinely Non-adherent category. Beyond providing initial support for the MACS, we discuss the clinical implications of this adherence classification system.     

Paroxysmal Hisian tachycardia and familial sarcoidosis

We report a His-bundle tachycardia in a 6 year-old patient without evidence of heart disease. Diagnosis and follow-up were assessed by exercise stress testing and repeated long-term electrocardiographic recording. As sarcoidosis was present in the mother and the aunt, this condition was discussed in this child also. However, since there is no absolute proof for this disease, the hypothesis of an antenatal injury of the bundle of His was discussed: this could be secondary to the transplacental transfer of abnormal maternal antibodies, as frequently observed in women with clinical or biologic evidence of connective tissue disease.     

Intra-abdominal Kaposiform hemangioendothelioma and the benefits of laparoscopic surveillance

We report a case of a neonate presenting with the rare vascular tumor, Kaposiform hemangioendothelioma. She had a lesion arising from the left ovary with multiple intraperitoneal metastases causing small bowel obstruction. We managed this case with primary surgical resection followed by laparoscopic surveillance of the peritoneal cavity and metastectomy. The child is fit and well and free of tumor after 32 months. Neither this presentation of this tumor nor this management approach has been previously described in the literature.     

Necrotizing enterocolitis comes in different forms: Historical perspectives and defining the disease

The specific cause of what is commonly referred to as necrotizing enterocolitis (NEC) disease has been elusive largely because it is becoming clear that this entity represents more than one disease with multifactorial pathogenic mechanisms. Furthermore, finding clear and consistent diagnostic biomarkers will be difficult until the different subsets of what we are calling this disease are better delineated. In this introductory chapter, we discuss different disease entities that are frequently termed “NEC” in the newborn infant. We hope this will set the stage for more focused research and development of preventative measures for at least the most common forms of this disease.     

A Case of Thanatophoric Dwarfism With Renal Hypoplasia

An autopsy case of thanatophoric dwarfism with renal hypoplasia is described. Histologically, the characteristic findings were disorganization of endochondral ossification at the epiphyseal plate of each femu, and this findings were useful for different diagnosis. Hypogenesis of the right kidney is rare in this disorder, and is suggestive of the vaiety of this disorder.     

The management of meconium aspiration syndrome.

Meconium aspiration is one of the important causes of mortality and morbidity in the neonatal period. Recently, advances for preventing meconium aspiration syndrome by the use of fetal monitoring, mechanical ventilation and supportive treatment have decreased the incidences of morbidity and mortality in this syndrome. In this paper, the management of this syndrome is reviewed and recent advances noted.