No abstract available.
Child* ; Humans ; Rhinitis*

Few cases of ichthyosis bullosa of Siemens(IBS) have been reported since 1939, as a distinct entity from bullous congenital ichthyosiform erythroderma(BCIE). IBS can be differentiated from BCIE by the absence of congenital erythroderma and a different distribution of involved skin area. It's characteristic features include blistering, superficial erosion or moulting of the outer skin. Histological features are tonofilaments aggregation confined to the granular and upper spinous layer of the epidermis. However, in BCIE these findings are present in the whole suprabasal compartment. The original reports of Siemens and cases from other authors showed an autosomal dominant inheritance. Our patient developed IBS sporadically without a familial background.
Blister ; Dermatitis, Exfoliative ; Epidermis ; Humans ; Ichthyosis Bullosa of Siemens* ; Ichthyosis* ; Intermediate Filaments ; Molting ; Skin ; Wills

No abstract available.
Pulmonary Edema*

Although thrombotic thrombocytopenic purpura (TTP) is a rare disease, when it develops in a post-cardiac surgery patient, it may have a fatal outcome. Since the frequency of early-onset thrombocytopenia in post-cardiac surgery patients is high, platelet concentrates are commonly transfused during postoperative management. However, when TTP is the likely diagnosis, platelet transfusion is not recommended. We experienced a postoperative TTP in a cardiac surgery patient and discovered the importance of identifying the etiology of postoperative thrombocytopenia. Here, we report the case with a brief review of the literature.
Blood Platelets ; Fatal Outcome ; Humans ; Platelet Transfusion ; Purpura, Thrombotic Thrombocytopenic ; Rare Diseases ; Thoracic Surgery ; Thrombocytopenia ; Thymine Nucleotides

Although thrombotic thrombocytopenic purpura (TTP) is a rare disease, when it develops in a post-cardiac surgery patient, it may have a fatal outcome. Since the frequency of early-onset thrombocytopenia in post-cardiac surgery patients is high, platelet concentrates are commonly transfused during postoperative management. However, when TTP is the likely diagnosis, platelet transfusion is not recommended. We experienced a postoperative TTP in a cardiac surgery patient and discovered the importance of identifying the etiology of postoperative thrombocytopenia. Here, we report the case with a brief review of the literature.
Blood Platelets ; Fatal Outcome ; Humans ; Platelet Transfusion ; Purpura, Thrombotic Thrombocytopenic ; Rare Diseases ; Thoracic Surgery ; Thrombocytopenia ; Thymine Nucleotides

No abstract available.
Arteriovenous Fistula*

Chylothorax is a rare complication following cardiac surgery for congenital heart diseases. Although conservative management is successful in the majority of cases, surgical intervention is required in a refractory one. Recently, subcutaneous or intravenous infusion of octreotide has been used as a safe treatment that helps avoiding surgical intervention. Herein, we report two cases of postoperative chylothorax treated with parenteral octreotide and conservative therapy.
Chylothorax* ; Heart Diseases ; Infusions, Intravenous ; Octreotide* ; Postoperative Complications ; Thoracic Surgery

Forty cases of infiltrating duct carcinoma of the breast were examined immunohistochemically for expression of TGF-beta and angiogenesis in order to analyze significant correlation with prognostic parameters including tumor size, axillary lymph node metastasis, clinical stage, histologic grade, estrogen receptor and progesterone receptor status. The TGF-beta expression was observed in tumors center and advancing edges of tumors. To determine microvessel density for angiogenesis, we stained endothelial cells for Factor VIII related antigen and counted microvessel within tumor. The results were as follows: 1) The strong immunohistochemical expression of TGF-beta and higher counts of microvessels were observed in advancing edges of tumors (p<0.05). 2) The TGF-beta expression in the advancing edges of tumors was closely related to clinical stage and presence of axillary lymph node metastasis (p<0.05). 3) The mean microvessel counts were significantly higher in tumors from patients with axillary lymph node metastasis and increased with increasing clinical stage (p<0.05). 4) The TGF-beta expression was not related to histologic grade, estrogen receptor and progesterone receptor status(p>0.05). Therefore, the results suggested that the TGF-beta expression and angiogenesis in infiltrating duct carcinoma of the breast may play an important part in prognostic factors, closely related to the lymph node metastasis and clinical stage.
Neoplasm Metastasis

OBJECTIVE: The purpose of this study was to investigate the influence of hyperactive stretch reflexes and contralateral adductor spread on the prognosis of gait in cerebral palsy with spastic diplegia and to investigate the reflex characteristics of spastic patients according to motor development. METHOD: Thirty three children with spastic diplegia were examined. Compound muscle action potentials elicited by electrical hammer were used for quantification of stretch reflexes. Clinical spasticity was evaluated with the modified Ashworth scale and re-evaluation was done at least for 18 months. The patients were classified as 3 groups according to the stage of motor development: Group I consisted of children who were unable to sit; Group II children were able to pull to stand but unable to walk independently; Group III children could walk independently more than ten steps. Reflex irradiation, amplitudes of compound muscle action potentials, amplitude ratios, and Ashworth scores were analyzed and compared between the groups. Twenty four normal infants and 18 children were examined as control. RESULTS: Contralateral adductor responses were elicited with patellar tendon tap stimulation in all spastic diplegic children. The amplitudes of contralateral adductor were 2.41 mV in group I, 1.75 mV in group II, and 1.21 mV in group III. The amplitude ratio of contralateral adductor to rectus femoris were 0.53 in group I, 0.40 in group II, and 0.26 in group III, respectively, and correlated with the degree of spasticity. CONCLUSION: The stretch reflex responses in children with cerebral palsy with spastic diplegia were highly exaggerated, and correlated with motor development. Spread of reflexes to contralateral adductor muscle would be in clinical and electrophysiological value.
Action Potentials ; Cerebral Palsy* ; Child* ; Gait ; Humans ; Infant ; Muscle Spasticity* ; Patellar Ligament ; Prognosis ; Quadriceps Muscle ; Reflex ; Reflex, Stretch* ; Walking*

OBJECTIVE: To investigate the reliability of distoproximal latency ratio of median sensory nerve as a diagnostic criterion of carpal tunnel syndrome (CTS) in patients with diabetic polyneuropathy. SUBJECT: Electrophysiologic study was performed in 264 hands of 208 patients with diabetes. Forty eight hands (24 subjects) without diabetes mellitus or CTS were included as a normal control group. Another 48 hands having CTS without diabetes mellitus were also included as a CTS control group. METHOD: Clinical and electrophysiologic findings were included to detect carpal tunnel syndrome in patients with diabetic neuropathy. Sensitivity and specificity of various electrodiagnostic parameters to confirm clinical CTS were obtained. RESULTS: Diabetic neuropathy was diagnosed in 66.3%, and median neuropathy was diagnosed in 52.7%. CTS was found in 32.2% as determined by the distoproximal latency ratio. The sensitivity of distoproximal latency ratio as a diagnostic tool for CTS was the highest (95.1%) and the specificity was the second highest (51.3%) among 5 different electrodiagnostic criteria of CTS. CONCLUSION: The results suggest that distoproximal latency ratio is an important parameter with high sensitivity in determining CTS in the patients with diabetic polyneuropathy.
Carpal Tunnel Syndrome* ; Diabetes Mellitus ; Diabetic Neuropathies* ; Electrodiagnosis* ; Hand ; Humans ; Median Neuropathy ; Sensitivity and Specificity