Epidemiology of limb reduction defects as registered in the Medical Birth Registry of Norway, 1970-2016: Population based study

Abstract

Background Following the Thalidomide disaster, the Medical Birth Registry of Norway (MBRN) was established in 1967, with epidemiological surveillance of congenital anomalies as one main aim. Limb reduction defects (LRD) constitute a rare and heterogeneous anomaly group, where correct registration and classification is important for surveillance and research. We aimed at reviewing and recoding LRD cases in the MBRN using the same classification system for all years, and evaluate time trends, characteristics and risk factors, 1970–2016. Methods After reviewing and recoding LRD cases using International Classification of Diseases (ICD), 10 th version, for all years, time trends, association with major anomalies, risk factors and infant outcomes were calculated. Generalized linear models for the binomial family with log link gave relative risks (RR) with 95% confidence intervals (CI). Classification of LRD as suggested by European surveillance of congenital anomalies (EUROCAT) was attempted. Results Overall LRD prevalence, 1970–2016, was 4.4 per 10 000, slightly increasing during 1970–1981, followed by relatively stable rates. There were more defects in upper than lower limbs. Defects in hands/fingers were most common, but unspecific descriptions prevented classification of LRD according to EUROCAT. A majority of cases had associated anomalies, the most common being other limb defects, followed by cardiac defects and anomalies in the nervous and digestive systems. From 1999, 26% of LRD cases were terminated, more than 90% of these had associated major anomalies. Stillbirth, neonatal and infant mortality were higher among infants with LRD, also related to associated anomalies. Pre-gestational diabetes was associated with a more than three times increased risk of offspring total LRD, while no association with maternal epilepsy was found. Taking folate/multivitamin supplements before and/or during pregnancy was associated with lower risk of offspring LRD (adjusted RR 0.7; 95% CI 0.6–0.9), while daily smoking did not significantly increase the risk. Conclusion The MBRN now has information on LRD coded by ICD-10 from 1970, but information is not specific enough to use other recommended classification systems. Collecting radiographic descriptions and/or more details from hospital records would improve the quality of the registry data. Taking folate supplements before/during pregnancy may reduce the risk of offspring LRD.

Congenital limb reduction defects in 1.6 million births in Argentina

“…This is higher than the perinatal mortality of 20% reported by Källén et al (). Our stillbirth rate of 6.6% was within the range reported in the literature (3–10.5%) (Evans et al, ; Gold et al, ; Klungsøyr et al, ), which excludes ETOPFA. The proportion of patients who died before hospital discharge in our study (19.3%) was higher than the proportion of patients that died within the first year of life reported by Evans et al (), Klungsøyr et al (), Gold et al, and Froster‐Iskenius and Baird () of 5, 5.5, 6.2, and 12.9%, respectively.…”

“…There are other reports in the literature detailing associated CA in patients with LRD (Bedard, Lowry, Sibbald, & Kiefer, ; Calzolari et al, ; Evans et al, ; Klungsøyr et al, ; Rosano et al, ; Stoll et al, ; Vasluian et al, ). There are methodological differences that impede an adequate comparison, particularly regarding the exclusion of syndromic cases from the analysis.…”

“…These factors could explain at least part of the differences in prevalence. Studies that have included cases with a definition similar to ours are Klungsøyr et al (), EUROCAT, , Vasluian et al (), Gold et al (), Riaño et al (), Yang et al (), and Evans et al (). Other differences between the different registries are the period of ascertainment and whether the surveillance systems are active or passive, and the inclusion of single or multiple sources.…”

Consequences of Hydrogel Granules Swallowing by Children: Case Study

“…Распространенность нарушений, вследствие которых возникает потребность в протезировании конечностей, в разных странах значительно варьирует -от 4,4 на 10 000 новорожденных в Норвегии [1] до 21,1 -в Голландии [2]. При этом частота врожденных особенностей строения конечностей в 2 раза выше, чем приобретенных [3,4]. Распространенность нарушений, требующих протезирования конечностей, в России остается неизученной.…”

“…2 The rehabilitation was timely if a) first prosthetic care performed at the age of 6 months, b) rehabilitation treatment initiated since birth. 3 Complex approach in rehabilitation was: a) training how to use prosthetics during rehabilitation, b) participation of interdisciplinary team: at least exercise physiologist (or rehabilitation therapist), ergotherapist, clinical psychologist, c) involvement of modern technologies like virtual reality. 4 Individual approach in rehabilitation: a) availability of individual rehabilitation and abilitation programs, b) individual goals and aims setting with a child, c) working in natural environment -school/kindergarten/home, г) individual exercises.…”