Outcome of lower L-thyroxine dose for treatment of congenital hypothyroidism

The appropriateness of the recommended L-thyroxine dose (10-15 micrograms/kg/day) for the treatment of congenital hypothyroidism has been questioned because of the risk of iatrogenic hyperthyroidism. We report the outcome of 23 newborns with congenital primary hypothyroidism treated with 25 micrograms L-thyroxine per day (5.3-9.2 micrograms/kg/day) and followed for an average of 59 months. Serum thyroxine (T4) values increased (X = 11.4 +/- 2.7 micrograms/dL) within 4 weeks posttherapy; eight infants had T4 levels > or = 13 micrograms/dL on only half the currently recommended dose. Thyroid-stimulating hormone (TSH) values remained elevated in 18 of 21 patients for 2-21 months despite a high-normal T4. Psychometric tests were performed in 19 of the 23 patients. The mean Full Scale IQ for the congenital hypothyroid group (n = 16) was 101.4 +/- 13.2 with comparable Verbal and Performance IQ scores. Patients with a bone age (BA) of < or = 32 weeks or T4 < 2 micrograms/dL at initial evaluation had significantly Lower Verbal IQ scores. A standardized parent-report assessment of behavioral and emotional functioning revealed subgroup scale scores that were indistinguishable from nonclinical norms. We conclude that (1) average range IQ scores and positive behavioral adaptation are observed in congenitally hypothyroid children treated with L-thyroxine doses lower than currently recommended; (2) the L-thyroxine dose should be individualized to prevent iatrogenic hyperthyroidism; (3) TSH normalization should not be a primary objective of treatment, and (4) a prospective study comparing the advantages and risks of different doses of L-thyroxine is needed.     

Prenatal detection of congenital idiopathic cataracts

We present a case of fentanyl overdose via mucous membrane absorption. A 31-year-old man presented to the emergency department in respiratory arrest. At intubation, a Duragesic transdermal patch (75 micrograms/h) was recovered from the buccal cavity. A second fentanyl transdermal patch (75 micrograms/h) was noted on the right lateral aspect of the thigh. Postmortem blood evaluation returned a venous fentanyl level of 17.2 micrograms/L. The therapeutic range for analgesic use is 1 microgram/L to 3 micrograms/L. Drug screens were positive for benzodiazepines and cocaine. Mass spectrophotometry/gas chromatography was used to determine fentanyl levels and to confirm drug screen results. Case history, findings at intubation, and high fentanyl blood concentration suggest the cause of respiratory arrest and death was fentanyl overdose.     

Lensectomy in treatment of traumatic cataracts

Results of pars plana lensectomy (36 cases) and ab externo lensectomy (14 cases) were presented. Intra- and postoperative complications, and the advantages of these procedures in traumatic cataracts were discussed. It was established that both procedures improved the visual acuity in the majority of patients although pars plana lensectomy had slightly lower rate of complications.     

Outcome of nonsurgical treatment for plantar fasciitis

Four hundred eleven patients with a clinical diagnosis of plantar fasciitis were assessed for predisposing factors. Each patient completed an outcomes assessment survey instrument that ranked effectiveness of various nonsurgical treatment modalities. Listed in descending order of effectiveness, the treatment modalities assessed were short leg walking cast, steroid injection, rest, ice, runner's shoe, crepe-soled shoe, aspirin or nonsteroidal anti-inflammatory drug, heel cushion, low-profile plastic heel cup, heat, and Tuli's heel cup. Treatment with a cast ranked the best. The Tuli's heel cup ranked the poorest. Most of the treatments were found to be unpredictable or minimally effective. The ineffectiveness of nonsurgical treatments noted in this outcomes study is at variance with most published clinical studies in which generally favorable results are reported after nonsurgical treatment for plantar fasciitis.     

Outcome after temporal lobectomy in bilateral temporal lobe epilepsy

We determined how noninvasive presurgical data relate to prognosis after temporal lobectomy in patients with independent bilateral temporal lobe (IBTL) complex partial seizures on the intracranial electroencephalogram (EEG). Between 1986 and 1994, 28 patients had IBTL seizures on intracranial EEG. Fifteen of these 28 patients underwent temporal lobectomy and 13 were not offered surgery. Of the 15 patients who had surgery, 10 patients became seizure-free. Magnetic resonance imaging (MRI) and the Wada test were the only variables associated with a seizure-free outcome. Seven of 10 seizure-free patients had a lateralized Wada result or the presence of unilateral hippocampal sclerosis, whereas none of the patients with persistent seizures had either of these findings. Variables not found to be predictive of a seizure-free outcome included location of scalp interictal spikes, degree of seizure-onset laterality, presence of early epilepsy risk factor, duration of epilepsy, and full-scale intelligence quotient. We conclude that MRI and the Wada test provide information of prognostic value in patients with bilateral temporal seizures independent of intracranial EEG data.     

Two cases of bilateral congenital cholesteatoma--usual and unusual presentations

We generated mice harboring germline mutations in which the enhancer element located 9 kb 3' of the immunoglobulin kappa light chain gene (3'E kappa) was replaced either by a single loxP site (3'E kappa delta) or by a neomycin resistance gene (3'E kappa N). Mice homozygous for the 3'E(kappa delta) mutation had substantially reduced numbers of kappa-expressing B cells and increased numbers of lambda-expressing B cells accompanied by decreased kappa versus lambda gene rearrangement. In these mutant mice, kappa expression was reduced in resting B cells, but was normal in activated B cells. The homozygous 3'E(kappa)N mutation resulted in a similar but more pronounced phenotype. Both mutations acted in cis. These studies show that the 3'E(kappa) is critical for establishing the normal kappa/lambda ratio, but is not absolutely essential for kappa gene rearrangement or, surprisingly, for normal kappa expression in activated B cells. These studies also imply the existence of additional regulatory elements that have overlapping function with the 3'E(kappa) element.     

Outcome from treatment for spinal arteriovenous malformation

BACKGROUND: prospective studies have demonstrated that a predominance of small, dense LDL particles (pattern B) precedes the clinical onset of coronary heart disease. Prevalence and characteristics of subjects with this LDL size abnormality were studied in young, nonobese, Japanese normolipidemic men. METHODS AND RESULTS: LDL peak particle diameter (PPD) was measured by continuous disc polyacrylamide gel electrophoresis in 223 nonobese normolipidemic men aged 18-20 years (mean+/-S.D. body mass index: 21.9+/-3.7 kg/m2, total cholesterol: 180+/-29 mg/dl, triglyceride: 62+/-34 mg/dl, HDL cholesterol: 58+/-12 mg/dl). Men with small LDL (PPD < 25.8 nm) were found in only 5.4% (n=12) whereas 197 men (88.3%) had a preponderance of large LDL (PPD 26.3 nm). As compared with men in a top tertile (PPD 27.5 nm) those in a low tertile (PPD < 26.9 nm) had higher serum levels of LDL cholesterol (120+/-31 vs 104+/-24 mg/dl), triglyceride (72+/-39 vs 49+/-16 mg/dl) and apolipoprotein (apo) B (84+/-21 vs 68+/-14 mg/dl), and lower HDL cholesterol (54+/-10 vs 60+/-12 mg/dl). They also had greater body mass index (23.2+/-4.6 vs 20.9+/-3.1 kg/m2) and percent body fat (21.5+/-7.7 vs 17.5+/-4.9%). LDL-PPD was positively correlated with HDL cholesterol (R=0.20, P=0.002) and was negatively correlated with apoB (R=0.34, P < 0.001), triglyceride (R=0.32, P < 0.001). percent body fat (R=0.26, P < 0.001), body mass index (R=0.24, P < 0.001), fat mass (R=0.23, P=0.001), total cholesterol (R=0.20, P=0.002). In multiple regression analysis, apoB, triglyceride, HDL cholesterol, apoAI and percent body fat explained 18% of LDLPPD variability. CONCLUSION: even in young, nonobese, normolipidemic men, LDL size appears to be associated with triglyceride-rich lipoprotein metabolism and body fat.     

Outcome of treatment of rectal cancer

To demonstrate the trend and treatment outcome of rectal cancer after the advent of adjuvant therapy, all case notes of rectal cancer patients admitted to Chulalongkorn Hospital from 1985-1994 were reviewed and cases were followed until 1996. Mean follow-up period was 685.3 days (8-3, 193 days). Most rectal tumors were Dukes' C (43.8%), well-differentiated (54.1%) and at the distal third (53.4%). AP resection remained the most common procedure before and after the advent of adjuvant therapeutic options (62.3%). Of 146 patients treated by curative operations, 60 had adjuvant therapies of which radical radiotherapy with or without chemotherapy was the most common. However, chemotherapy was increasingly employed as the neoadjuvant and as combined chemoradiotherapy. There was a preferential selection of less well-differentiated, more distal, more Dukes' C disease and younger patients for the adjuvant therapy (p < 0.05). Recurrence rate in the adjuvant group was not different from the surgery group despite significant poorer prognostic indicators (17.4% & 21.7%, p = 0.53). Mortality was higher in Dukes' B + C patients in adjuvant group (17.3% & 3.4%, p = 0.02). The outcomes were not different among Dukes' A patients. The complications; i.e. wound problems, gut obstruction; did not increase with the adjuvant treatment. No adverse effect was observed on the healing of colorectal or coloanal anastomoses in the adjuvant group.     

Morphology of untreated bilateral congenital dislocation of the hips in a seventy-four-year-old man

Two untreated completely dislocated hips were examined pathologically in a 74-year-old male dissecting room cadaver. The femoral heads bilaterally were 9 cm above the acetabular fossae. They lay adjacent to each ilium with no secondary acetabulum formation. A thickened and markedly elongated fibrous capsule appeared to be the major structure that had checked further migration of the femoral heads during weight-bearing. The femoral heads, although slightly flattened medially and somewhat smaller in size than in the normal adult, showed no degenerative arthritis. Dysplastic acetabular fossae were filled with soft tissue. Bone-to-bone contact with secondary degenerative changes was present only where the femurs, at the level of the lesser trochanters, rubbed against the overhanging superior acetabular rims.     

Outcome of ICU treatment in invasive aspergillosis

OBJECTIVE: To assess the outcome of intensive care treatment in invasive aspergillosis. DESIGN: Retrospective study. SETTING: University Hospital, Medical Intensive Care Unit (ICU). PATIENTS: Twenty-five patients with invasive aspergillosis who were admitted to the medical ICU in a 5 1/2 year period. Twenty-two had received high-dose chemotherapy for (mainly hematologic) malignancies, one had been treated with cyclosporine and prednisolone for systemic lupus erythematosus, one with high-dose methylprednisolone for polyarteritis nodosa and one had an ARDS after near-drowning. MEASUREMENTS AND RESULTS: The medical records were reviewed for patient and disease characteristics, outcome, reasons for admission to the ICU, supportive care and antifungal therapy as well as for the results of cultures and autopsy. Out of 25 patients, a definite ante mortem diagnosis could be established in seven. When autopsied patients were included, a total of 15 suffered from proven invasive aspergillosis. Although standard antifungal treatment and maximal available supportive care were given, 23 of 25 patients (92%) died after a mean of 15 (1-51) days in the ICU. Both patients who recovered had received high-dose chemotherapy for hematologic malignancy and showed bone marrow recovery and/or had a localized pulmonary infection. CONCLUSIONS: In patients with highly suspected or proven invasive aspergillosis, admission to an ICU and mechanical ventilation should be considered in cases of localized infection and obvious signs of hematologic recovery. In most other circumstances ICU admission for mechanical ventilation does not seem to improve survival.     

Outcome of trigger finger treatment in diabetes

Trigger finger is an underdiagnosed hand disorder causing disability in longstanding diabetic patients. Sixty diabetic patients [39 insulin-dependent diabetes mellitus (IDDM) and 21 non-insulin-dependent diabetes mellitus (NIDDM)] and 60 nondiabetic patients were examined. All were initially treated by steroid injections: failure to alleviate symptoms was the indication for surgery. The incidence of multiple digit involvement was higher in IDDM patients as compared with the control group (p < 0.001). The diffuse type was 1.45 times more frequent in IDDM and NIDDM than in nondiabetic patients (p < 0.008). The diabetic patients had a relatively longer duration of symptoms (p < 0.003). Significantly, a higher recovery rate upon steroid injection was achieved in control patients as compared with the diabetic ones (p < 0.001). IDDM patients required more surgery compared with NIDDMs and, in 13.3% of diabetic patients, the surgical outcome was not successful. Diabetic patients should be diagnosed early for multiple and diffuse types of trigger digits. Steroid injection as the first mode of therapy is highly recommended although not always successful. Surgery is the definitive treatment but requires a long course of physiotherapy and may be associated with some complications.     

Randomised double-masked placebo-controlled trial of a treatment for congenital nystagmus

Several therapies have been developed for congenital nystagmus (CN) but without placebo-controlled trials. We investigated a treatment which combined two therapies that had been advocated by several authors and were reported to improve visual acuity (VA). A placebo treatment was designed to mimic the time, attention, 'high tech' apparatus, and the explanation used in the experimental treatment. To each group, 38 subjects with CN were randomly allocated. Their VA and contrast sensitivity (CS) were assessed three times before undergoing treatment for 6 weeks and then once more. An improvement in VA occurred, however, this was not significantly different in the two groups. The improvement in CS was greater in the experimental than in the control group, but the difference failed to reach significance in most statistical tests. We conclude that putative therapies for CN should be assumed to be placebos until proven otherwise with randomized controlled trials.     

Catheter treatment of congenital heart disease

Over the last 10 years, several advances have been made in paediatric cardiology and cardiac surgery. However, the major developments have been in non-surgical attempts at transcatheter treatment of congenital heart disease. Initially these concerned some simple defects such as pulmonary valve stenosis but lately much more high-risk and complex defects have been treated.     

Outcome of pregnancy after radioactive iodine treatment for well differentiated thyroid carcinomas

This study sought to determine the outcome of pregnancy in female patients with differentiated thyroid carcinoma who became pregnant after radioactive iodide treatment. A total of 779 female thyroid cancer patients were treated at Chang Gung Medical Center in Linkou between January 1977 and December 1995. The medical records of these patients were reviewed retrospectively. Thirty-seven of these patients had well differentiated thyroid carcinoma receiving 131I treatment and conceived at a mean age of 27.97 +/- 3.49 year-old. A total of 58 pregnancy episodes were recorded during this study period. Among these 37 patients, 3 episodes of artificial abortion, 8 episodes of spontaneous abortion and 2 threatened abortions were observed. These patients delivered a total of 47 babies including 3 premature babies. Seven of these patients conceived within 6 months after the last administration of 131I, including 2 cases within 1 month, 4 cases within 4 months, and 1 patient within 5 months. Of these 7 patients, only one patient who conceived within 6 months after the last administration of 131I (14.3%) had a spontaneous abortion. The present results suggest that previous administration of 131I in female patients with well differentiated thyroid cancer does not result in demonstrable adverse effects in subsequent pregnancies. However, further studies involving long-term follow-up of children delivered by mothers who became pregnant within 6 months after the last administration of 131I is needed to further elucidate the possible chronic effects and sequelae of 131I therapy on subsequent pregnancies.     

Diagnosis and treatment of congenital thumb hypoplasia

The hypoplastic thumb has a variety of presentations, which have been generalized into a formal classification system that has treatment implications. In most cases, the spectrum of hypoplasia has predictable deficits in terms of tendon and bone absence. The ultimate goal is always the same--to enhance usage of the radial digit. In this article, a straightforward, reliable, and reproducible surgical technique is discussed and illustrated. Pitfalls, indications, and treatment regimes also are discussed to help the physician successfully treat the child with a hypoplastic thumb.     

Outcome research on the psychosocial treatment of personality disorders

This brief overview of outcome research on psychosocial treatment of personality disorders first examines difficulties in using existing classifications of abnormal personality. A summary is offered of uncontrolled investigations of the treatment of personality disorders, a few controlled studies, and literature on the impact of comorbid personality disorder on treatment of Axis I symptomatology. The conclusions take into account the particular challenges of these disorders in the current health care context.     

Shaving: a simple and effective treatment for moderate congenital hypertrichosis

We describe a 60-year-old man with stage IIA myeloma, who despite achieving a systemic remission after C-VAMP, progressed with isolated meningeal myeloma. Meningeal involvement in myeloma is a rare complication with a poor prognosis.     

Modified Van Nes operation for treatment of congenital proximal femoral focal deficiency

In this study, experimental hyperthyroidism was established and used to investigate possible alterations in the calcium (Ca), magnesium (Mg), and zinc (Zn) homeostasis by assessing their concentrations in plasma and erythrocytes. In the L-thyroxine-induced hyperthyroidism condition, the experimental animals show a significant decrease in erythrocyte Ca, Mg, and Zn concentrations, and a significant decrease in plasma Mg concentration. Significant positive correlations were found for Mg and Zn both in plasma and in erythrocytes. The results suggest that the homeostasis of Ca, Mg, and Zn is altered during experimental hyperthyroidism.