Robinow syndrome is a rare inherited disorder initially reported by Robinow et al. in 1969 as a new dwarfing syndrome. It is characterized by the presence of fetal face, forearm shortening, genital hypoplasia, and hemivertebrae. The autosomal recessive form of Robinow syndrome shows a more severe clinical phenotype which is expressed by the mutation of homozygous ROR2(Receptor tyrosine kinase like Orphans) gene, mapped to the chromosome 9q22, a region that overlaps the locus for autosomal dominant inherited brachydactyly type B(BDB). ROR2 encodes the receptor tyrosine kinase, which is important for the development of mesomelic long bones. We experienced a boy diagnosed as a Robinow syndrome with fetal face(frontal bossing, hypertelorism, small up-turned nose, triangular mouth with down turned angles, micrognathia), large anterior fontanelle, cranium bifidum, mesomelic shortening, cryptorchidism. We present this case with a brief review of related literature.
Brachydactyly ; Cranial Fontanelles ; Cryptorchidism ; Encephalocele* ; Forearm ; Humans ; Hypertelorism ; Male ; Mouth ; Nose ; Phenotype ; Protein-Tyrosine Kinases ; Skull*

Anthracyclines have been widely used in cancer therapy because of their efficacy in the treatment of various solid tumors and hem -atologic malignancy. Cumulative dose-related cardiotoxicity was a well-known toxicity of anthracyclines. Particularly, at total doses of more than 550 mg/m2, therapy with anthracyclines could produce irreversible cardiac injury. Anthracycline-induced cardiac toxicity was usually manifested by congestive heart failure or arrhythmia. In co- ntrast, acute myocardial infarction is a rare event of anthracycline-induced heart diseases. A 31-year-old man with non-Hodgkin lymphoma(NHL) and single cardiac risk factor, including smoking, was presented with chest pain after receiving 2nd CEOP-BLAM chemo-therapy. An electrocardiogram revealed ST segment elevation in inferior leads consistent with acute myocardial infarction. An echocardiogram revealed an ejection fraction of 60% and severe hypokinesia in inferior and anteroseptal wall. Three days later, coronary angiography revealed 50% of luminal stenosis of right coronary artery(RCA) and near total occlusion with large thrombi in m-RCA. After balloon angioplasty and stent insertion, the patient was transferred to coronary care unit and continuous intravenous heparin infusion was started. On the 10th days, the patient was discharged in good condition. Six months later, follow-up coronary angiography showed no significant lesion in right coronary artery. In a young man with NHL, we report an acute myocardial infarction after 2nd course of CEOP-BLAM chemotherapy with a review of relevant literatures.
Adult ; Angioplasty, Balloon ; Anthracyclines ; Arrhythmias, Cardiac ; Chest Pain ; Constriction, Pathologic ; Coronary Angiography ; Coronary Care Units ; Coronary Vessels ; Doxorubicin* ; Drug Therapy* ; Electrocardiography ; Follow-Up Studies ; Heart Diseases ; Heart Failure ; Heparin ; Humans ; Hypokinesia ; Lymphoma, Non-Hodgkin* ; Myocardial Infarction* ; Phenobarbital ; Risk Factors ; Smoke ; Smoking ; Stents

Anthracyclines have been widely used in cancer therapy because of their efficacy in the treatment of various solid tumors and hem -atologic malignancy. Cumulative dose-related cardiotoxicity was a well-known toxicity of anthracyclines. Particularly, at total doses of more than 550 mg/m2, therapy with anthracyclines could produce irreversible cardiac injury. Anthracycline-induced cardiac toxicity was usually manifested by congestive heart failure or arrhythmia. In co- ntrast, acute myocardial infarction is a rare event of anthracycline-induced heart diseases. A 31-year-old man with non-Hodgkin lymphoma(NHL) and single cardiac risk factor, including smoking, was presented with chest pain after receiving 2nd CEOP-BLAM chemo-therapy. An electrocardiogram revealed ST segment elevation in inferior leads consistent with acute myocardial infarction. An echocardiogram revealed an ejection fraction of 60% and severe hypokinesia in inferior and anteroseptal wall. Three days later, coronary angiography revealed 50% of luminal stenosis of right coronary artery(RCA) and near total occlusion with large thrombi in m-RCA. After balloon angioplasty and stent insertion, the patient was transferred to coronary care unit and continuous intravenous heparin infusion was started. On the 10th days, the patient was discharged in good condition. Six months later, follow-up coronary angiography showed no significant lesion in right coronary artery. In a young man with NHL, we report an acute myocardial infarction after 2nd course of CEOP-BLAM chemotherapy with a review of relevant literatures.
Adult ; Angioplasty, Balloon ; Anthracyclines ; Arrhythmias, Cardiac ; Chest Pain ; Constriction, Pathologic ; Coronary Angiography ; Coronary Care Units ; Coronary Vessels ; Doxorubicin* ; Drug Therapy* ; Electrocardiography ; Follow-Up Studies ; Heart Diseases ; Heart Failure ; Heparin ; Humans ; Hypokinesia ; Lymphoma, Non-Hodgkin* ; Myocardial Infarction* ; Phenobarbital ; Risk Factors ; Smoke ; Smoking ; Stents

BACKGROUND: Radioiodine treatment is effective for the removal of remnant thyroid tissues after thyroidectomy in patients with differentiated thyroid carcinoma. To induce the elevation of serum TSH level which facilitates the uptake of radioiodine into remnants, a 4 to 6 week interval between thyroidectomy and radioiodine administration has been established. During the period of preparation, most patients have experienced overt symptoms of hypothyroidism which have led to the development of alternative strategies. Some reports have suggested that the interval could be reduced to about 3 weeks with less symptoms. We reevaluated the adequate time needed for the elevation of serum TSH level above 30microU/mL after thyroidectomy. METHODS: Forty five patients who had undergone total thyroidectomy for differentiated thyroid carcinoma were investigated. Serum TSH and free T4 levels were measured one or more times within 3 weeks after operation(total 97 blood samples). Eighty nine blood samples were obtained within 15 days. RESULTS: In 41 patients (91.1%) serum TSH levels increased to 30 microU/mL until 15 days after operation. Until postoperative 21 days, serum TSH levels in all the other patients reached 30microU/mL. In linear equation, the daily increment of serum TSH levels was 2.62microU/mL for the first 8 days after operation and 5.34micorU/mL for the next 7 days. The half-life of serum free T4 levels showed marked individual variations. CONCLUSION: Measurement of serum TSH level at about 15 days after total thyroidectomy for differentiated thyroid carcinoma may be useful in determining the time of radioiodine administration.
Half-Life ; Humans ; Hypothyroidism ; Thyroid Gland* ; Thyroid Neoplasms* ; Thyroidectomy ; Thyrotropin

Severe hypoglycemia induces neuroglycopenic symptoms, including mental alteration, as glucose is the exclusive fuel for the central nervous system. However, some reports have shown that non-glucose fuels, like lactates and ketones, could be utilized by the brain during severe hypoglycemia. Herein, a case of extreme hypoglycemia in a 44-year old woman, subsequently diagnosed as congestive heart failure accompanied by ischemic hepatitis and lactic acidosis, is presented. In two episodes of extreme hypoglycemia, she was fully alert without obvious neurological deficits. In this unusual case, an increased supply of lactate might have maintained the cerebral function and prevented cerebral injury during the hypoglycemia that was induced as a result of starvation and hepatic and cardiac dysfunctions
Acidosis, Lactic* ; Adult ; Brain ; Central Nervous System ; Female ; Glucose ; Heart Failure ; Hepatitis ; Humans ; Hypoglycemia* ; Ketones ; Lactates ; Lactic Acid ; Masks* ; Starvation

Severe hypoglycemia induces neuroglycopenic symptoms, including mental alteration, as glucose is the exclusive fuel for the central nervous system. However, some reports have shown that non-glucose fuels, like lactates and ketones, could be utilized by the brain during severe hypoglycemia. Herein, a case of extreme hypoglycemia in a 44-year old woman, subsequently diagnosed as congestive heart failure accompanied by ischemic hepatitis and lactic acidosis, is presented. In two episodes of extreme hypoglycemia, she was fully alert without obvious neurological deficits. In this unusual case, an increased supply of lactate might have maintained the cerebral function and prevented cerebral injury during the hypoglycemia that was induced as a result of starvation and hepatic and cardiac dysfunctions
Acidosis, Lactic* ; Adult ; Brain ; Central Nervous System ; Female ; Glucose ; Heart Failure ; Hepatitis ; Humans ; Hypoglycemia* ; Ketones ; Lactates ; Lactic Acid ; Masks* ; Starvation

PURPOSE: Cytomegalovirus (CMV) infection is mostly asymptomatic but can be detrimental to certain hosts. We investigated changes of CMV seroprevalence in Koreans before and after the year 2000. METHODS: We reviewed laboratory values of patients who were tested for CMV immunoglobulin G (IgG) at Samsung Medical Center, Seoul, Korea, from January 1995 to December 2015. Changes in seroprevalence were analyzed by gender, age, region, and tested year period (period 1, 1995–2005 vs. period 2, 2006–2015). RESULTS: Overall CMV seropositivity was 94.1% (10,900/11,584). There was no significant difference for CMV seropositivity among the two periods (94.2% vs. 94.1%) (P=0.862). CMV seropositivity in the 11 to 20-year age group in period 2 (78.8%) was significantly lower than that of period 1 (89.9%) (P=0.001). The seropositivity of individuals aged 31–40 years (97.4%) was significantly higher than that of younger age groups (P < 0.001) and lower than that of older age groups (P < 0.001). Of 2,441 females of reproductive age (from 15 to 49), CMV seropositivity was 97% (2,467/2,441). The seropositivity in women aged 20–24-years was higher than that of men in the same age group (97.6% vs. 85.6%, P=0.003). No significant difference was observed among different regions. CONCLUSIONS: Overall CMV seropositivity of Koreans was estimated to be 94% and the average seropositivity of reproductive women was 97%. Monitoring of the changes in seroprevalence including the reproductive age group is needed in the future.
Cytomegalovirus ; Female ; Humans ; Immunoglobulin G ; Korea ; Male ; Seoul ; Seroepidemiologic Studies