Growth and Endocrine Function in Long-term Adult Survivors of Childhood Stem Cell Transplant.

The number of long-term surviving stem cell transplant (SCT) recipients has increased steadily, and attention has now extended to the late complications of this procedure. The objective of this study was to investigate relationship among growth and endocrine functions in long-term adult survivors of childhood SCT. The inclusion criteria of this study were survival at least 5 yr after SCT and achievement of adult height. Fifty-four patients (39 males) fulfilled these criteria and were included in this study. Growth was mainly evaluated by height standard deviation score (SDS) and individual longitudinal growth curves. Among the 54 patients, those that received SCT before 10 yr of age showed significantly greater reductions in changes in height SDS (mean -1.75, range -4.80 to -0.10) compared with those that received SCT at or after 10 yr of age (mean -0.50, range -1.74 to 1.20; P<0.001). The mean loss of height for all patients who received SCT during childhood was estimated to be approximately 1 SDS/6.5 yr (r=0.517). Individual longitudinal growth curves indicated that a significant growth spurt was absent in severe short stature patients during the pubertal period without severe endocrine dysfunctions including GH deficiency. The incidence of growth disorder in long-term adult survivors depends on the age at SCT and whether they received radiation therapy. Life-long follow-up is necessary for survivors to detect, prevent and treat the late endocrine complications in SCT survivors.

Spontaneous expectoration of bronchial foreign body: a case report.

A nine-year-old girl with mental retardation accidentally swallowed an axle of a toy car and was urgently hospitalized with the diagnosis of left bronchial foreign body. While various monitors were installed and removal of the foreign body by a ventilating rigid bronchoscope was in preparation, the patient suddenly coughed and vomited, with the foreign body found in the vomit. When examining infant cases of air way foreign body, attention tends to be focused on the diagnosis and treatment. However, patients are at risk of aggravating difficulty in breathing or suffocation as long as foreign bodies are present in the air way. It is important to monitor carefully for changes in the breathing and to prepare for unexpected events.

Hyperaldosteronemia and hypogammaglobulinemia secondary to atopic dermatitis-induced exudation in an infant presenting with growth failure.

The present case is a 5-month-old female with atopic dermatitis who was brought to hospital for growth failure noted upon regular health examination. Laboratory examinations revealed hyponatremia, hyperkalemia, hypoproteinemia, hypogammaglobulinemia, elevated plasma renin activity and hyperaldosteronemia. Immune function was normal. Composition of the exudate collected from the skin lesions of atopic dermatitis was similar to that of plasma. Application of a steroid ointment improved the lesions as well as all laboratory values. These findings indicate that voluminous exudation caused by extensive atopic dermatitis can lead to hypotonic dehydration, electrolyte abnormalities, hypoproteinemia, hypogammaglobulinemia and, finally, to growth failure in infants. We conclude that intensive treatment is important for severe atopic dermatitis in infants to prevent serious complications.

Long-term follow-up of thyroid function in patients who received bone marrow transplantation during childhood and adolescence.

An increasing number of long-term surviving bone marrow transplant (BMT) recipients have recovered from their primary disease but are at risk of developing failure of endocrine organs. We investigated 147 patients who underwent allogeneic BMT. Thyroid function was evaluated by serial measurement of basal TSH and free T4 levels as well as by TRH provocative test. Thyroid ultrasound examination was performed for evaluation of thyroid tumor after BMT. Five patients were found to have overt thyroid dysfunction (hypothyroidism in four patients and hyperthyroidism in one patient). Twenty-three patients in the under 9-yr-old group at BMT and 16 patients in the over 10-yr-old group at BMT had subclinical compensated hypothyroidism. Younger age at BMT was the strongest factor for developing thyroid dysfunction, compared with older age (P < 0.001). Only in patients with subclinical compensated hypothyroidism did median basal and peak TSH increase to the upper half of the normal range by 8 yr after BMT and then returned slightly to the middle of the normal range spontaneously. These results suggest that thyroid dysfunction in long-term BMT survivors depends on age at BMT, with a greater risk among younger patients, indicating the need for life-long surveillance.

Changes in thyroid function after bone marrow transplant in young patients.

BACKGROUND: Changes in thyroid function among young patients who received bone marrow transplantation (BMT) were evaluated. METHODS: The study included 91 patients (50 males) who underwent BMT from 1985 to 1995 at the age of 0.6-21 years. Sixty patients had neoplastic disease such as leukemia or lymphoma, and the remainder had non-neoplastic diseases. Preconditioning regimen for BMT included 12 Gy of fractionated-total body irradiation (TBI) for patients with neoplastic disease and 3-8 Gy of irradiation for the remaining patients, in addition to chemotherapy. Evaluation of thyroid function was performed by serial assessment of basal serum FT4, FT3, TSH concentrations as well as by TRH test. RESULTS: No patient had overt hypothyroidism or elevated basal TSH concentrations (>10 mU/L). However, 6 (7%) of patients experienced exaggerated peak TSH response to TRH stimulation several years after BMT. In 33 patients whose thyroid status was evaluated before, within 3 months, and 1 year after BMT, serum FT3 concentrations as well as peak TSH response to TRH stimulation significantly decreased immediately after BMT (<3 months) and normalized within 1 year. However, serum FT4 concentrations did not change significantly. One patient developed primary hypothyroidism and another developed follicular adenoma of the thyroid 5 and 12 years after BMT, respectively. CONCLUSION: Short-term changes in thyroid function after BMT can indicate euthyroid sick syndrome rather than tertiary hypothyroidism. It must be noted that overt hypothyroidism may occur several years after BMT, hence long-term follow-up of thyroid function is warranted.

Persistence of mild hyperthyrotropinemia after discontinuation of three-year course of low-dose L-thyroxine therapy in infants with borderline hypothyroidism.

Treatment plan for neonates with borderline hypothyroidism (persistent hyperthyrotropinemia with normothyroxinemia) has not been established. In this study, changes in thyroid function after discontinuation of low-dose L-thyroxine (L-T4) supplement in infants with the condition were evaluated. Fourteen infants with hyperthyrotropinemia at neonatal screening had repeated hyperthyrotropinemia (> 8 mU/L) with normothyroxinemia. TSH response was exaggerated at TRH testing. The subjects were treated with low-dose L-T4 (3 to 9 microg/kg/day) for 2.2 to 6 years, and euthyroid status was maintained. After discontinuation of therapy, mild hyperthyrotropinemia persisted up to 24 months, while serum FT4 remained within the lower half of the normal range. TSH response to TRH stimulation, which tended to be exaggerated 1 month after discontinuation, became lower 6 to 12 months later. RAIU and thyroid scintigraphy were normal in all subjects. No patient developed hypothyroxinemia, although mild elevation of TSH lasted rather long after discontinuation of low-dose L-T4 therapy. Administration of L-T4 was not resumed provided the subjects were followed at regular interval. Further long-term investigation is needed to define whether re-administration is necessary or not.