Hospital Care and Surgical Treatment of Children With Congenital Upper Limb Defects.

BACKGROUND AND AIMS: To evaluate hospital care of children with congenital upper limb defects. MATERIALS AND METHODS: Three hundred and sixty-two children with an upper limb defect, born 1993-2005, and alive after birth admission were identified in the Finnish Register of Congenital Malformations. The data on hospital care, with focus on operative treatment, were collected from the National Hospital Discharge Register, until 31 December 2009. Mean follow-up was 10.2 years (range: 4-17 years). The results were compared with data on the whole children population (1.1 million) in Finland. RESULTS: Most children (321, 87%) with upper limb defects had hospital admissions: on average, one admission/year (range: 0-36), and they were treated in hospital 5 days/year (range: 0-150), which is 11-fold compared with an average child in Finland. Four surgical procedures/child were done (range: 0-45), including one hand surgical procedure. The most common procedures were orthopedic (513); gastrointestinal (263); ear, nose, and throat (143); dental (118); thoracic (48); and urologic (44). Of the 513 orthopedic procedures, 326 were directed to upper limbs, 107 to the lower limbs, and 10 to the spine. Median operation age was 2 years 7 months. Altogether, 60% of hospital admissions were non-surgical. Leading causes of non-operative hospital admissions were congenital anomalies (32%), gastroenterological problems (20%), respiratory tract conditions (13%), neurological problems (7%), perinatal conditions (5%), and infectious diseases (5%). CONCLUSION: Treatment of children with upper limb defects is teamwork between pediatric and surgical subspecialties. Burden of hospital care is 11-fold as compared with an average child.

Hospital admissions and surgical treatment of children with lower-limb deficiency in Finland.

BACKGROUND AND AIMS: There are no population-based studies about hospital admissions and need for surgical treatment of congenital lower-limb deficiencies. The aim is to assess the impact children with lower-limb deficiencies pose to national hospital level health-care system. MATERIALS AND METHODS: A population-based study was conducted using the national Register of Congenital Malformations and Care Register for Health Care. All 185 live births with lower-limb deficiency (1993-2008) were included. Data on hospital care were collected until 31 December 2009 and compared to data on the whole pediatric population (0.9 million) live born in 1993-2008. RESULTS: The whole pediatric population had annually on average 0.10 hospital admissions and the mean length of in-patient care of 0.3 days per child. The respective figures were 1.5 and 5.6 in terminal lower-limb amputations (n = 7), 1.1 and 3.9 in long-bone deficiencies (n = 53), 0.6 and 1.9 in foot deficiencies (n = 26) and 0.4 and 2.6 in toe deficiencies (n = 101). Orthopedic surgery was performed in 72% (5/7) of patients with terminal amputations, in 62% (33/53) of patients with long bone, in 58% (14/24) of patients with foot and in 25% (25/101) of patients in toe deficiencies. Half (54%) of all procedures were orthopedic operations. CONCLUSION: In congenital lower-limb deficiencies the need of hospital care and the number of orthopedic procedures is multiple-fold compared to whole pediatric population. The burden to the patient and to the families is markedly increased, especially in children with terminal amputations and long-bone deficiencies of lower limbs.

Calcitonin gene-related peptide (CGRP) and its effects on protein release in vitro in the developing submandibular gland of the rat.

Indirect immunohistochemical methods were used to study presence and number of CGRP-immunoreactive (CGRP-IR) nerve fibers in the submandibular gland and ganglion cells of the superior cervical, submandibular and trigeminal ganglia of the developing rat. The effect of CGRP on peroxidase and total protein release was also studied in the developing postnatal submandibular glands of 1, 5, 12 and 30-day-old, as well as adult rats by in vitro methods. The possible costimulation of CGRP with SP, NKA or carbachol on 5-day-old and adult rats was also tested. The stimulatory effects of these compounds were compared to the basic release of peroxidase and total amount of proteins from submandibular gland fragments in incubation solution. CGRP-IR nerve fibers were found in relatively high number during post-natal development, mainly around blood vessels and ducts. Some CGRP-IR nerve fibers were also detected around acini. The number of these fibers was quite low and remained constant during the post-natal development. The number of CGRP-IR trigeminal ganglion cells was higher on the 5th and 12th post-natal day than later in development and in adult animals. At the same time, superior cervical- and submandibular ganglion cells were non-reactive for CGRP, suggesting trigeminal origin of CGRP-IR nerve fibers during the development in the submandibular gland. In the secretory studies, CGRP per se stimulated both peroxidase and total protein release in the submandibular gland most effectively on 5th and 12th post-natal days, while there was no clear secretory response in the adult glands. In the 5-day-old submandibular gland CGRP in combination with SP, NKA or carbachol clearly enhanced the total protein secretory response when compared with the release caused by these substances alone. However, in the adult submandibular gland, the combinations did not enhance total protein release more than any of the substances alone. Furthermore, it can be concluded that the presence of a more dense CGRP-IR innervation during the early postnatal period in the developing submandibular gland is accompanied with an increased responsiveness of the secretory elements to CGRP.

Substance P (SP) and neurokinin A (NKA) in developing submandibular glands of the rat.

The effect of isoprenaline, carbachol, substance P (SP) and neurokinin A (NKA) on peroxidase and total protein secretion was studied in the developing postnatal submandibular glands of the rat using in vitro methods. Submandibular glands of 1, 5, 12 and 30 day-old rats were stimulated by 10(-5) M isoprenaline and carbachol, and 10(-6) M SP and NKA. The stimulatory effects of these compounds were compared to the basic release of peroxidase and total amount of protein from submandibular gland fragments in incubation solution with no added transmitter substances. Indirect immunohistochemical methods were used to study these developing glands from SP- and NKA-immunoreactive (IR) nerve fibers. The distributions of SP-IR and NKA-IR nerve fibers closely resembled each other, being most abundantly spread around the developing acini and ducts. The number of these fibers was high on the 1st, 5th and 12th days, but was decreased on the 30th day. On peroxidase release, isoprenaline was the most effective, causing a maximal response of 47 times the basic release on the first postnatal day, after which it gradually decreased. The effects of carbachol, SP and NKA on peroxidase release were clearly weaker and, unlike isoprenaline, their strongest response was on the 5th postnatal day (carbachol, 4.3; SP 5.2; NKA, 4.5). The total protein secretion effect patterns of the studied substances resembled each other more, showing their strongest response on the 5th day (isoprenaline, 5.0; carbachol, 4.5; SP, 4.2; NKA, 3.4) and decreasing thereafter.(ABSTRACT TRUNCATED AT 250 WORDS)

Calcitonin gene-related peptide immunoreactive nerve fibers in the developing salivary glands of the rat.

The appearance and distribution of calcitonin gene-related peptide immunoreactive (CGRP-IR) nerve fibers was studied by indirect immunofluorescence microscopy in the developing salivary glands of the rat at daily age intervals from the 15th day in utero (i.u.) until birth, and subsequently on the 2nd, 5th, 7th, 16th, 30th postnatal (p.n.) days. The findings were compared to samples of adult rats. CGRP-IR fibers appeared on day 20 i.u. both in parotid and submandibular glands. In submandibular glands, fibers were first seen surrounding the developing ductal branches, in the mesenchyme and also in association with developing ducts and blood vessels. In the parotid gland CGRP-IR fibers first appeared around ductal branches and blood vessels on day 20 i.u. and on day 21 i.u. CGRP-IR nerve fibers were found in the mesenchyme and around developing ducts. The density of CGRP-IR fibers was highest between the 2nd and 16th postnatal days, after which the amount of CGRP immunoreactivity slowly decreased to the levels of adult glands. The increase in a number of CGRP-IR nerve fibers especially around the ductal and vascular elements in the developing salivary glands indicates that they may play a role in the functional maturation of salivary glands.

Development of substance P and neurokinin A immunoreactivity in ganglia supplying nerves to the submandibular glands of the rat.

Developing submandibular, trigeminal and superior cervical ganglia, which provide innervation to the submandibular glands, were studied for substance P (SP)- and neurokinin A (NKA)-immunoreactive (IR) ganglion cells and nerve fibres in rat. These ganglia were examined by using an indirect immunofluorescence technique at daily intervals from the 16th day in utero (i.u.) until birth, and subsequently on the 2nd, 5th, 7th, 12th, 16th, 30th, 42nd postnatal day and in the adult (3 months). In the submandibular ganglion SP- and NKA-IR cells and fibres first appeared in considerable numbers on the 19th day i.u. (in one sample out of five on the 18th day i.u.), when more than 90% of the ganglion cells were immunoreactive to SP and NKA. The number stayed at more than 90% to the 7th postnatal day and then slowly decreased to the levels of adult animals (18% SP, 17% NKA). The first SP- and NKA-IR ganglion cells and fibres appeared in the trigeminal ganglion on the 18th day i.u. when they represented 7% (SP) and 4% (NKA) of the ganglion cells. The number of SP- and NKA-IR cells increased steadily, reaching a maximum at the time of birth when 68% (SP) and 74% (NKA) of the ganglion cells were immunoreactive. Thereafter they began to decrease toward the level of an adult rat (10% SP, 11% NKA). In the superior cervical ganglion only a few SP- and NKA-IR ganglion cells were detected from the 19th day i.u. to the fifth postnatal day. Positive ganglion cells were also occasionally found in the nerve trunks outside the superior cervical ganglion.(ABSTRACT TRUNCATED AT 250 WORDS)

Substance P and neurokinin A immunoreactive nerve fibres in the developing salivary glands of the rat.

The time of appearance and distribution of substance P (SP) and neurokinin A (NKA) immunoreactive nerve fibres in developing salivary glands of the rat were studied by the use of indirect immunohistochemical methods. The glands were examined at daily intervals from the 15th day in utero (i.u.) until birth, and subsequently on the 2nd, 5th, 7th, 12th, 16th and 30th postnatal day. The findings were compared to samples from adult. The first SP- and NKA-immunoreactive (IR) nerve fibres appeared on the 19th day i.u. in the parotid and submandibular glands and were abundantly distributed around developing ductal branches. In the mesenchyme around the developing ductal branches of the parotid gland the fibres appeared on the 20th day i.u. In the submandibular gland NKA-IR fibres appeared in the mesenchyme surrounding the developing ductal branches on the 19th day i.u. and SP-IR fibres on the 21st day i.u. Around blood vessels of both glands, SP- and NKA-IR fibres made their appearance only much later, on the second postnatal day. The number of SP- and NKA-IR nerve fibres in the developing salivary glands was already high on the 19th day i.u. when they were first detected. From this point up to the 16th postnatal day the glands were richly innervated by the fibres, but later the numbers slowly decreased to adult levels. The abundance of SP- and NKA-IR nerve fibres especially around the ductal branches and secretory structures in the developing salivary glands suggests a role in the functional maturation of salivary glands.

Neurokinin A in the parotid and submandibular glands of the rat: immunohistochemical localization and effect on protein and peroxidase secretion.

An indirect immunofluorescence technique was used to study the distribution of neurokinin A immunoreactive (NKA-IR) nerve fibres in submandibular and parotid glands of the rat. The functional role of neurokinin A on protein and peroxidase secretion in these glands was evaluated by using in vitro methods. In the parotid gland neurokinin A immunoreactive fibres were mainly distributed around the secretory acini, but some were also in evidence around the stromal blood vessels and ducts. The number of the neurokinin A immunoreactive nerve fibres was lower in the submandibular gland than in the parotid gland. They were mainly distributed around the secretory acini and stromal blood vessels and ducts. In vitro, neurokinin A significantly stimulated the release of total amount of released proteins and peroxidase from parotid gland fragments, while in the submandibular gland only the release of peroxidase was increased. By using SDS polyacrylamide gel electrophoresis (SDS-PAGE) specific changes were found in the release of proteins after neurokinin A stimulation. The results of the present study demonstrate that neurokinin A immunoreactive nerve fibres are present in the rat parotid and submandibular glands. Their localization around the secretory elements of the glands and the effect of neurokinin A in vitro experiments indicates that neurokinin A might have a significant role in the regulation of salivary secretion.