The clinical signs of acquired hypothyroidism are usually manifested insidiously over several months to years. The incidence increases after 6 years of age and peaks at 11 to 18 years of age. The clinical symptoms and signs are fatigue, constipation, decreased growth velocity and delayed bone age, compromised intellectual performance, obesity, myxedema, hyperlipidemia, peripheral neuropathy and delayed or precocious puberty. Two children were referred to our hospital for the evaluation of severe obesity and short stature. During the evaluation we found they also had hyperlipidemia, cardiomegaly with or without pericardial effusion. Thyroid function test revealed decreased serum thyroid hormone levels with positive anti- microsome and anti-thyroglobulin antibodies consistent with long-standing acquired hypothyroidism. After the supplement of L-thyroxine, both of them showed rapid improvement of above symptoms, except for the incomplete catch-up growth. We herein report two cases of acquired hypothyroidism with severe obesity, short stature, hyperlipidemia and cardiomegaly with review of literatures.
Antibodies ; Cardiomegaly* ; Child ; Constipation ; Fatigue ; Humans ; Hyperlipidemias ; Hypothyroidism* ; Incidence ; Microsomes ; Myxedema ; Obesity ; Obesity, Morbid* ; Pericardial Effusion ; Peripheral Nervous System Diseases ; Puberty, Precocious ; Thyroid Function Tests ; Thyroid Gland ; Thyroxine

The clinical signs of acquired hypothyroidism are usually manifested insidiously over several months to years. The incidence increases after 6 years of age and peaks at 11 to 18 years of age. The clinical symptoms and signs are fatigue, constipation, decreased growth velocity and delayed bone age, compromised intellectual performance, obesity, myxedema, hyperlipidemia, peripheral neuropathy and delayed or precocious puberty. Two children were referred to our hospital for the evaluation of severe obesity and short stature. During the evaluation we found they also had hyperlipidemia, cardiomegaly with or without pericardial effusion. Thyroid function test revealed decreased serum thyroid hormone levels with positive anti- microsome and anti-thyroglobulin antibodies consistent with long-standing acquired hypothyroidism. After the supplement of L-thyroxine, both of them showed rapid improvement of above symptoms, except for the incomplete catch-up growth. We herein report two cases of acquired hypothyroidism with severe obesity, short stature, hyperlipidemia and cardiomegaly with review of literatures.
Antibodies ; Cardiomegaly* ; Child ; Constipation ; Fatigue ; Humans ; Hyperlipidemias ; Hypothyroidism* ; Incidence ; Microsomes ; Myxedema ; Obesity ; Obesity, Morbid* ; Pericardial Effusion ; Peripheral Nervous System Diseases ; Puberty, Precocious ; Thyroid Function Tests ; Thyroid Gland ; Thyroxine

No abstract available.
Extracorporeal Membrane Oxygenation* ; Thrombosis*

Distal hereditary motor neuropathy (dHMN) is a heterogeneous group of disorders characterized by weakness and wasting of distal limb muscles without overt sensory abnormalities. Recently, autosomal dominant dHMN has been mapped to chromosome 12q24 and 7q11-q21. We present a family with autosomal dominant adult onset dHMN type II consisting of fi ve affected individuals spanning three generations. They developed mild symmetrical distal lower limb weakness, muscle wasting, and severe foot deformity after the third decade. Genetic analysis showed no support for linkage to chromosome 12q24 and 7q11-q21 in our family. These fi ndings further demonstrate a genetic heterogeneity within dHMN type II.

No abstract available.
Syphilis, Cutaneous

PURPOSE: Either gated myocardial perfusion SPECT or attenuation corrected SPECT can be used to improve specificity in the diagnosis of coronary artery disease. We investigated in this study whether gating or attenuation correction improved diagnostic performance of rest/stress perfusion SPECT in patients having intermediate pre-test likelihood of coronary artery disease. MATERIALS AND METHODS: Sixty-eight patients underwent rest attenuation-corrected T1-20l/dipyridamole stress gated attenuation-corrected Tc-99m-MIBI SPECT using an ADAC vertex camera (M:F=29:39, aged 59+/-12 years, coronary artery stenosis> or =70%. one vessel: 13, two vessel: 18, three vessel: 8, normal: 29). Using a five-point scale, three physicians graded the post-test likelihood of coronary artery disease for each arterial territory (1 normal, 2: possibly normal 3:equivocal, 4: possibly abnormal, 5: abnormal). Sensitivity, specificity and area under receiver-operating-characteristic curves were compared for each operator between three METHODS: (A) non-attenuation-corrected SPECT; (B) gated SPECT added to (A); and (C) attenuation-corrected SPECT added to (B). RESULTS: When grade 3 was used as the criteria for coronary artery disease, no differences in sensitivity and specificity were found between the three methods for each operator Areas under receiver- operating-characteristic curves for diagnosis of coronary artery disease revealed no differences between each modality (p>005). CONCLUSION: In patients at intermediate risk of coronary artery disease, gated SPECT and attenuation-corrected SPECT did not improve diagnostic performance.
Coronary Artery Disease* ; Coronary Vessels* ; Diagnosis ; Humans ; Perfusion* ; ROC Curve ; Sensitivity and Specificity ; Tomography, Emission-Computed, Single-Photon*

We retrospectively reviewed the sex distribution, age at oepration, chief complaints, peroperative and postoperative refractive errors, and corrected visual acuity in 160 previously operated patients to evaluate the clinical manifestations, reractive error, frequency of amblyopia, age at operation, and the postoperative factors affected by the operation of epiblepharon and congenital entropion itself that influence visual acuity and refractive error. The average age at operation was 7.9 years. The chief complaint was ocular discomfort, followed by visual disturbance, photophobia, eyelid rubbing and epiphora. One hundred thirty-three wyws(41.6%) whose preoperative corrected visual acuity was below 0.6 had a mean visual acuity of 0.7. Ninety-five eyes (29.7%) were preoperative myopes above -1.0D, 77 eyes(24%) were hyperopes above +1.0D, and 163 eyes were astigmatic above -1.0D. Twenty-two out of 76 eyes who were followed up for more than one year had a corrected visual acuity of below 0.6. The difference between the older and less than 7 years groups was significantly not significant. Astigmatic changes were not statistically different at any age group. However, the mean significantly visual acuity at postoperative one year was 0.73, which was significantly different from the preoperative value(P=0.006). In conclusion, concurrent postoperative glasses correction and amblyyopia therapy is indicated because the incidence of refractive errors and amblyopia is higher in epiblepharon and congenital entropion.
Amblyopia ; Entropion* ; Eyeglasses ; Eyelids ; Glass ; Humans ; Incidence ; Lacrimal Apparatus Diseases ; Photophobia ; Refractive Errors ; Retrospective Studies ; Sex Distribution ; Visual Acuity

A 13-year old boy was seen for progressive enlargement of a right scrotal mass that had been present since early childhood. Scrotal mass was excised and consisted of a well demarcated multinodular cystic soft mass. Pathology was characteristic of cavernous lymphangioma. Lymphangiomas are benign tumors of congenital origin, occurring about 95% of the lesion at neck and axilla. Their scrotal location is quite uncommon. Treatment consists of surgical excision. Unless completely removed, recurrences are common.
Adolescent ; Axilla ; Humans ; Lymphangioma* ; Male ; Neck ; Pathology ; Recurrence ; Scrotum*

Polydipsia in schizophrenic patients is not uncommon, but a frequently underdiagnosed condition. The etiology of polydipsia remains unclear, and its complications can be life-threatening, while often being difficult to manage it. We report a case of a successfully treated chronic schizophrenic patient with polydipsia. The patient was male, 47-year-old, suffering 27-years of residual schizophrenia who had been consuming more than 10 L of water per day, and is complicated by hyponatremia. He was treated with irbesarten 300 mg and naltrexone 50 mg in the setting of closed ward. He consumed less than 3.5 L of water per day and serum sodium levels seemed to be stable following discharge from the closed ward. We suggest that irbesartan and naltrexone may have beneficial effects for treating polydipsia, and future prospective and well-controlled studies are to be performed.
Humans ; Hyponatremia ; Male ; Middle Aged ; Naltrexone* ; Polydipsia* ; Prospective Studies ; Schizophrenia* ; Sodium ; Water

Henoch-Sch nlein purpura(HSP) frequently follows upper respiratory infection, and one of the causes of this disease is inferred to beta-hemolytic streptococcal infection, but the relationship is still unclear. Familial tendency of this disease is unclear, too. Also genetic relationship of this disease has been in a controversy yet. We experienced two cases of HSP in brother and sister at similar period, and report this case with review of related literatures.
Humans ; Purpura, Schoenlein-Henoch* ; Siblings* ; Streptococcal Infections