Transtibial amputation with plantar flap for congenital deficiency of the tibia.

Disarticulation of the knee has been the preferred treatment for the severe type (Type Ia and Type Ib classification of Jones et al) of congenital deficiency of the tibia because of marked flexion contracture of the knee and loss of quadriceps function. In such cases, the disarticulated stump is often small and poorly covered by soft tissues because of dysplastic femoral condyles and calf muscles. Therefore, stump complications after disarticulation may prevent early aggressive walking exercises and delay independent ambulation. To overcome this problem, a greater weightbearing surface was created by a transtibial amputation with a short stump of the fibula using the flexed knee. By this method, the distal femoral condyle and the anterior surface of the fibula were used for weightbearing. In addition, coverage of the new weightbearing area by a neurovascular pedicled sensate plantar flap provided a more tolerable weightbearing site. The purpose of the current study was to report a 5-year-old boy with bilateral congenital total deficiency of both tibias, who was treated using this technique. The patient was ambulating independently 15 weeks after surgery. A transtibial amputation with a plantar flap is an alternative procedure to knee disarticulation for the severe type of congenital deficiency of the tibia.

Transient hypothyroxinemia of prematurity is associated with abnormal cranial ultrasound and illness severity.

Transient hypothyroxinemia without elevated thyroid-stimulating hormone (TSH) levels is common in prematurity, especially in very-low-birth-weight (VLBW) infants. The transient hypothyroxinemia of prematurity (THOP) has been seen as a "benign" condition not requiring medical treatment. However, some recent large observational studies have revealed a relationship between THOP and abnormal neurodevelopment. Furthermore, one study showed THOP had twice the risk of brain echolucency, which was the best predictable neurodevelopmental dysfunction, than the premature infants with normal or higher thyroxine levels. The relationships among THOP, illness severity, and neurodevelopmental dysfunction remain unclear. We propose a hypothesis that THOP is associated with abnormal ultrasound and illness severity. We studied 54 infants who were admitted more than 14 days at our neonatal intensive care unit (NICU) with a birth weight <2000 g from March 1999 to March 2000. The infants received serum thyroxine (T4), free-T4, and TSH measurement during the first weeks of life. Most of them had serum thyroxine levels measured at approximately 2 weeks of age. The infants enrolled in the study were examined by at least 1 of 3 cranial ultrasounds during the first weeks of life, illness severity evaluation according to the neonatal therapeutic intervention scoring system (NTISS) score, as well as NICU hospital stay period. Infant were classified as THOP by T4 value <5.3 microg/dL (68 nmol/L), which is 2.6 SD below the mean for term infants in Massachusetts, without elevated TSH value (<20 microIU/mL). After adjusting for some confounding factors, such as gestational age, birth weight, duration of mechanical ventilation, infants with THOP were associated with abnormal cranial ultrasound, illness severity, and lower 1-minute Apgar score. In our studies, THOP was related with brain ultrasound anomaly, neonatal illness, and lower Apgar score at 1 minute. Does early thyroxine intervention improve the prognosis and neurodevelopmental dysfunction? This question requires further investigation.

Prevalence of iodine deficiency and goitre during pregnancy in east Hungary.

OBJECTIVE: To assess the iodine nutritional status and the prevalence of goitre during pregnancy in a region of Hungary that appeared to be iodine sufficient in previous studies. DESIGN: A cross-sectional voluntary screening study was organized in which 313 pregnant women participated. METHODS: Urine iodine concentration and the volume of the thyroid gland were measured in every woman. In the presence of low urinary iodine concentrations, goitre, or both, thyroid function tests were performed. RESULTS: Iodine deficiency was found in 57.1% of the pregnant women, and was severe in 15.6%. The volume of the thyroid gland was enlarged in 19.2% of individuals. Nodular goitre was found in 17 women (5.4%). The frequency of goitre and the mean thyroid volume were increased in the group of iodine-deficient women. In the 89 cases of iodine deficiency or goitre, thyrotrophin concentrations were in the normal range; however, the free triiodothyronine:free throxine ratio was increased in 97% of them, indicating that the thyroid gland was in a stimulated state in these individuals. CONCLUSIONS: Iodine deficiency with high prevalence of goitre was recognized among pregnant women in an area that previously appeared to be iodine sufficient. An unexpected mild iodine deficiency was also noted in the non-pregnant control group. Reassessment and continuous monitoring of iodine nutritional status is warranted even in populations that are apparently considered to be 'at no risk' of iodine deficiency, especially in pregnant women. Regular administration of iodine, starting at preconception or in early pregnancy and continuing during the period of nursing, is recommended in these regions.

Sonomorphologic evaluation of goiter in an iodine deficiency area in the Ivory Coast.

OBJECTIVES: This study evaluated the extent of thyroid abnormalities in a remote iodine-deficient area of the Ivory Coast. METHODS: Ultrasonography was used in detecting the presence of goiter. RESULTS: The overall prevalence rates of goiter were 64.7% among females and 53.3% among males. In children aged 6 to 15 years (n = 314), the prevalence of goiter was 62% regardless of sex. Thyroid volume increased steadily with age, with significantly larger goiters in women 25 years and older. Frequency of cysts and calcifications did not correlate with sex. CONCLUSIONS: Especially in developing countries, prophylaxis of iodine deficiency disorders must be improved in iodine-deficient areas to prevent substantial morbidity, which is more severe in women and elderly persons.

[The skeletal changes in premature infants with a copper deficiency]. / Skelettveränderungen bei Frühgeborenen mit Kupfermangel.

We describe 5 preterm infants (25th to 30th week of gestation) suffering from alimentary copper deficiency. The diagnosis was confirmed by low serum copper and caeruloplasmin concentrations. Characteristic clinical findings were repeated apnoeic attacks, hypopigmentation of skin and hair, anaemia, neutropenia and leucopenia refractory to other therapy, as well as increasing serum alkaline phosphatase activity in the first month of life. Starting in the 3rd to 12th week of life the radiographic findings were general skeletal osteoporosis and retardation, metaphyseal radiodense lines, irregular metaphyses, cupping and spurring of the metaphyses, followed by multiple fractures and subperiosteal new bone formation and enlarged costochondral junctions. Copper was substituted orally resulting in complete healing of fractures and improvement in both clinical symptoms and laboratory parameters.

Renal osteodystrophy--a radiological review.

Renal osteodystrophy is an amalgam of a number of distinct pathological conditions, in particular, hyperparathyroidism and osteomalacia. In addition, there may be a change in the guantity of bone, i.e., osteopenia (osteoporosis) or osteosclerosis. While bone biopsy may be the most reliable method for detecting these lesions, it is not yet a routine procedure in many centers. Radiological assessment of the bones, therefore, is the most widely used method for assessing the type and severity of the bone lesions in patients with chronic renal failure. This article reviews the world literature and pays attention to conventional radiological techniques as well as macroradiography. In addition, studies in which radiological appearances are correlated with histological appearances are described. Mention is also made of the effects on radiological bone disease of dialysis and transplantation. Consideration is also given to the manifestations of soft-tissue calcification, both of the vascular and subcutaneous type, and to the effects of treatment.