Revised listings for growth disorders and weight loss in children. Final rule.

This rule adopts, with one change, the rule for evaluating growth disorders in children we proposed in a notice of proposed rulemaking (NPRM) published in the Federal Register on May 22, 2013. Several body systems in the Listing of Impairments (listings) contain listings for children based on impairment of linear growth or weight loss. We are replacing those listings with new listings for low birth weight (LBW) and failure to thrive; a new listing for genitourinary impairments; and revised listings for growth failure in combination with a respiratory, cardiovascular, digestive, or immune system disorder. These revisions reflect our program experience, advances in medical knowledge, and comments we received from medical experts and the public.

A practical approach to classifying and managing feeding difficulties.

Many young children are thought by their parents to eat poorly. Although the majority of these children are mildly affected, a small percentage have a serious feeding disorder. Nevertheless, even mildly affected children whose anxious parents adopt inappropriate feeding practices may experience consequences. Therefore, pediatricians must take all parental concerns seriously and offer appropriate guidance. This requires a workable classification of feeding problems and a systematic approach. The classification and approach we describe incorporate more recent considerations by specialists, both medical and psychological. In our model, children are categorized under the 3 principal eating behaviors that concern parents: limited appetite, selective intake, and fear of feeding. Each category includes a range from normal (misperceived) to severe (behavioral and organic). The feeding styles of caregivers (responsive, controlling, indulgent, and neglectful) are also incorporated. The objective is to allow the physician to efficiently sort out the wide variety of conditions, categorize them for therapy, and where necessary refer to specialists in the field.

Talla baja idiopática. Revisión y puesta al día / Idiopathic short stature. A literature review and update

Con la denominación de talla baja idiopática (TBI) se agrupan una serie de entidades clínicas de etiología desconocida que tienen en común un retraso crónico de crecimiento con talla inferior a −2 desviaciones estándar (DE), con preservación de la armonía entre los segmentos corporales y en las que, en su evolución espontánea, las expectativas de talla adulta son inferiores a −2 DE. Es un diagnóstico de exclusión que exige una evaluación clínica, bioquímica, hormonal y molecular minuciosa con el objetivo de descartar cualquier etiología conocida del retraso de crecimiento, especialmente el retraso constitucional del crecimiento y desarrollo (RCCD). La TBI es un diagnóstico frecuente entre los pacientes que consultan por retraso de crecimiento, existiendo lagunas y controversias sobre su abordaje diagnóstico y terapéutico. Este documento de consenso recoge información actualizada sobre la definición, diagnóstico y tratamiento de la TBI, y aporta datos y recomendaciones que no han sido contemplados en documentos anteriores (AU)

Idiopathic short stature (ISS) refers to all clinical conditions involving an alteration of growth (height <−2 SD) of unknown cause, with preservation of proportionality among body segments, with the expectation of adult height < −2 SDS, and in which a diagnosis of constitutional delay of growth and development has been previously ruled out. ISS is an exclusion diagnostic which requires clinical, biochemical, hormonal and molecular studies in order to rule out all known causes of growth retardation and short stature.ISS is a frequent diagnosis among children with short stature. Despite its frequency, there is still controversy on the best diagnostic and therapeutic approach when treating patients with ISS. This consensus document contains updated information on the definition, diagnosis and treatment of ISS, and provides new data and recommendations that have not been addressed in previous documents (AU)

Diagnosis on a positive fashion of nonorganic failure to thrive.

AIMS: To study the predictive value of predefined symptoms and signs for allocating children into one of two groups: nonorganic and organic failure to thrive. PATIENTS AND METHODS: Two hundred eight outpatients (6 months-14 years old) suffering from failure to thrive (FTT) were included in the study. Predefined symptoms and signs were considered as potential predictors of organic/nonorganic failure to thrive. All patients underwent an established diagnostic work up in order to exclude organic causes of FTT. RESULTS: The percentage of patients without any organic symptom (negative predictive value), who were diagnosed as NOFTT was 92%; the percentage of patients having nonorganic symptoms only (positive predictive value), who were diagnosed as NOFTT was 96%, while their absence does not exclude a NOFTT diagnosis as well (negative predictive value = 41%). CONCLUSION: The detection of at least one nonorganic symptom or sign, with the exclusion of any organic symptom, can support a diagnosis of nonorganic FTT and therefore only few laboratory investigations seem to be warranted.

Failure to thrive: still a problem of definition.

The term 'failure to thrive' (FTT) is widely used to describe inadequate growth in early childhood. However, no consensus exists concerning the specific anthropometrical criteria to define this description. The aim of this study was to make an updated assessment concerning the use of FTT definitions and describe possible trends regarding the use of specific criteria. A cross-sectional review was done covering English-language articles published from January 2003 until June 2004, and recent textbooks of general pediatrics. Most of the reviewed literature broadly defined FTT as inadequate growth and total agreement existed to define FTT based solely on anthropometrical parameters. Large differences, however, were seen regarding which growth parameters to use and whether to use attained values or velocities. Weight was the most predominant choice, but many included more than one anthropometrical parameter. Failure to thrive in children is currently described solely based on anthropometrical indicators, with weight gain as the predominant choice of indicator and cut off around the 5th percentile. Discussion is needed as to whether the term 'failure to thrive' is still a useful common term for pediatric undernutrition of different types.

Optimal timing for living-related liver transplantation in children.

BACKGROUND: The pediatric end stage liver disease (PELD) score has been used widely to prioritize children awaiting cadaveric liver transplantation (LTx). To establish the objective parameter for optimal timing of living-related LTx (LRLTx), we have assessed our cases using the PELD score. METHODS: From 1997 to 2002, 24 children were evaluated 28 times for the indication of LRLTx. Among them, 15 were for jaundice and nine for growth failure, hepatopulmonary syndrome, and variceal bleeding. Nine of 24 children underwent LRLTx. They were divided into several groups according to their clinical course. The PELD score consisted of age, albumin, total bilirubin, prothrombine time-international ratio (INR) and growth failure. A cut-off value was obtained by the highest positive and negative predictive value. RESULTS: The PELD score in cases whose indication for LRLTx was approved was significantly higher compared with the cases who were not, and a cut-off value of 4 was obtained. The PELD score in cases who were alive after LRLTx was significantly lower compared with the cases who died after LRLTx or evaluation of the indication, and a cut-off value of 22 was established. CONCLUSION: LRLTx may be considered when the PELD score exceeds 4, and LRLTx may be required immediately when the PELD score exceeds 22.

Diagnóstico del retraso del crecimiento / Diagnosis of growth retardation

El crecimiento es un proceso biológico complejo, producto de la interacción entre múltiples factores endógenos (genéticos, hormonales, metabólicos, receptividad de los tejidos diana) y factores exógenos (nutrición, actividad física e influencias psicosociales).Es uno de los indicadores más sensibles del estado de salud del niño, de su nutrición y de sus antecedentes genéticos. Las desviaciones de la normalidad pueden ser la primera manifestación de una patología subyacente congénita o adquirida, por lo que se necesita disponer de una correcta comprensión del proceso del crecimiento y de los diferentes trastornos que pueden alterarlo. Entendemos por talla baja aquella en la que un individuo se encuentra por debajo de - 2 desviaciones estándar, para una determinada edad y sexo, en relación con la media poblacional. Actualmente, la clasificamos en talla baja idiopática y talla baja patológica. El diagnóstico continúa basándose en la correcta interpretación de la anamnesis y de la exploración física, aun cuando la historia reciente de la endocrinología se ha caracterizado por un emergente desarrollo de los métodos diagnósticos. Entre ellos destacan los avances en genética molecular, que han permitido la localización y caracterización en el ser humano de los genes que codifican proteínas implicadas en la regulación hormonal del crecimiento, como GH, POU1F1, PROP 1, Hex1, rGHRH, rGH, IGF-1, LHX3, LHX4, etc. Esta área constituye una de nuestras principales líneas de investigación y en esta revisión aportaremos algunos resultados, entre ellos el hallazgo de una nueva mutación (MiL) en un paciente con síndrome de Laron (AU)

Selection of growth parameters to define failure to thrive.

Failure to thrive (FTT) is a syndrome of growth failure due to undernutrition. Determining whether an infant has FTT is based on the use of an anthropometric indicator and a selected cutoff value for that indicator. These anthropometric indicators include weight for age, weight for length, and length for age, and the cutoff values include the 10th, 5th, and 3rd percentiles. Each indicator and selected cutoff value provide unique information about an infant's growth. However, these parameters are often used interchangeably to explain the same growth phenomenon. The sensitivity and specificity of each anthropometric indicator are a function of the cutoff value selected and dictate which infants will be classified as having FTT and which infants will be classified as healthy. Depending on the sensitivity and specificity of the indicator, some infants with FTT will be classified as healthy, and some healthy infants will be classified as having FTT. A clear rationale for the selection of an anthropometric indicator and a cutoff value for defining FTT are important for increasing the generalizability of research findings and thereby expanding the current knowledge base related to FTT.

Feeding disorders in infants and toddlers: diagnosis and treatment.

Each of the six feeding disorders described presents with specific symptoms, has a different origin, and responds to different interventions. Consequently, it is important to establish an accurate diagnosis and use the appropriate intervention for the specific feeding disorder. As the study by Benoit et al [7] demonstrates, an intervention that may be helpful for one feeding disorder can be completely ineffective for another.

Comparison of methods to categorize undernutrition in children.

We compared the results of three methods of categorizing undernutrition in a group of 258 children referred for evaluation of "failure to thrive." There was broad variation between the number of children classified as malnourished (75% to 98%) and the degree of undernutrition, depending on the method used. We recommend that categorization systems be used only to define a child as having a risk of possible adverse effects of undernutrition, not as being malnourished.

Infantile depression, nonorganic failure to thrive, and DSM-III-R: a different perspective.

A controversy exists regarding the classification of nonorganic failure to thrive within the psychiatric nomenclature. There are a number of DSM-III-R diagnoses that may be applied to NOFTT, including Reactive Attachment Disorder of Infancy (RADI) and Major Depressive Disorder (MDD). The behaviors characteristic of NOFTT are symptomatic of depression, and are similar to those exhibited by infants with anaclitic depression as well as those of the adult with depression. The correspondence of the behaviors of NOFTT and the DSM-III-R criteria for Major Depression are reviewed, as are the conceptual and therapeutic reasons to view NOFTT infants as suffering from Depression.

Feeding disorders in infancy: classification and treatment considerations.

This paper critically discusses conceptualizations of failure to thrive as an organic and nonorganic dichotomy. Research findings are used to refute this type of categorization. Instead, an approach which recognizes both organic and nonorganic influences and consequences of the syndrome are suggested. Physical, psychological and interpersonal systems and their interactions are explored. Potential contributions of the psychiatrist in the context of a multidisciplinary team approach to treatment are described.

Estudio clínico endocrinológico del retardo del crecimiento / Clinical endocrinologic study in growth retardation

El retardo en el crecimiento es uno de los primeros motivos de consulta en la unidad de endocrinología del Instituto Nacional de Salud del Niño, donde el diagnóstico se dificulta debido a la multiplicidad de factores que lo acasionan. Se resumen los conocimientos básicos relativos a la fisiología del crecimiento, medidas de evaluación y clasificación del retardo de crecimiento. Se presenta el estudio realizado en 100 pacientes con tallas menores del 3er percentil que acudieron al INSN a partir de 1982 y tuvieron un seguimiento mínimo de 6 meses y un máximo de 4 años. Fueron evaluados mediante historia clínica completa y ficha de seguimiento para registro de datos antropométricos y exámenes auxiliares. Los pacientes fueron traidos a la consulta a una edad promedio de 7 años y sólo 13 pudieron brindar información sobre controles previos de crecimiento y desarrollo. Las tallas de los progenitores estuvieron entre el 3º y 10º percentil de la población adulta como referencia. Se encontró que el 38 por ciento correspondió a variantes normales, 27 con retardo constitucional y 9 con talla corta familiar. El mayor número de causas patológicas estuvo dado por la desnutrición (20 por ciento) en el grupo de alteraciones extrínsecas; endocrinas (14 por ciento), digestivas o parasitarias (13 por ciento) y respiratorias (7 por ciento). Entre las endocrinas predominó el hipotiroidismo. En cuanto a alteraciones intrínsecas, predominaron las alteraciones cromosómicas (6 sindromes de Turner y un sindrome de Down). Se evaluó el resultado del tratamiento en pacientes con desnutrición, parasitosis e hipotiroidismo que demostró diferencias altamente significativas luego de su instalación. Se discuten los resultados resaltando las características fundamentales en relación al pronóstico y dificultades para el diagnóstico y tratamiento. Se concluye que los pacientes son traídos tardíamente a la consulta y que es notoria la escasez de antecedentes sobre crecimiento y desarrollo lo cual retarda el diagnóstico en varios meses y aun años

Non-organic failure to thrive: a reappraisal.

Non-organic failure to thrive has traditionally been regarded as due primarily to maternal rejection and neglect. A critical reappraisal of the evidence suggests a more balanced view of the mother-child relationship should be taken. A classification of the condition, founded on facts not concepts, is urgently required. Non-organic failure to thrive should be viewed in a multidimensional context, in which potential influences upon the symptomatic infant are considered. Inadequacy of nutrition is caused by both a failure of adequate provision of food and by inadequate intake. A vicious circle of maladaptive behavioural interaction between caregiver and infant is often present, sustained by high emotional tensions. Clinical intervention should aim to clarify the contributions made by both caregiver and infant to that interaction and thus break the cycle. The basis on which intervention is made should be direct observation of the parent and child relationship in as many different environmental contexts as feasible, especially during feeding. The multidisciplinary team has an important role to play in management. An emphasis on parental culpability in the aetiology of non-organic failure to thrive, in the absence of direct evidence of neglect, is wrong.