ABSTRACT
OBJECTIVE@#To investigate the effect of hyperoxia on intestinal metabolomics in mice.@*METHODS@#Sixteen 8-week-old male C57BL/6 mice were randomly divided into hyperoxia group and control group, with 8 mice in each group. The hyperoxia group was exposed to 80% oxygen for 14 days. Mice were anesthetized and euthanized, and cecal contents were collected for untargeted metabolomics analysis by liquid chromatography-mass spectrometry (LC-MS) combined detection. Orthogonal partial least square discriminant analysis (OPLS-DA), volcano plot analysis, heat map analysis, and Kyoto Encyclopedia of Genes and Genomes (KEGG) were used to analyze the effects of hyperoxia on metabolism.@*RESULTS@#(1) OPLS-DA analysis showed that R2Y was 0.967 and Q2 was 0.796, indicating that the model was reliable. (2) Volcano plot and heat map analysis showed significant statistical differences in the expression levels of metabolites between the two groups, with 541 up-regulated metabolites, 64 down-regulated metabolites, and 907 no differences, while the elevated 5-hydroxy-L-lysine was the most significant differential metabolite induced by high oxygen. (3) KEGG pathway enrichment analysis showed that porphyrin and chlorophyll metabolism (P = 0.005), lysine degradation (P = 0.047), and aromatic compound degradation (P = 0.024) were the targets affected by hyperoxia. (4) Differential analysis of metabolic products through KEGG enrichment pathway showed that hyperoxia had a significant impact on the metabolism of porphyrin and chlorophyll, lysine, and aromatic compounds such as benzene and o-cresol.@*CONCLUSIONS@#Hyperoxia significantly induces intestinal metabolic disorders. Hyperoxia enhances the metabolism of porphyrins and chlorophyll, inhibits the degradation of lysine, and delays the degradation of aromatic compounds such as benzene and o-cresol.
Subject(s)
Mice , Male , Animals , Lysine , Hyperoxia , Benzene , Mice, Inbred C57BL , Metabolic Diseases , Oxygen , Chlorophyll , Porphyrins , Biomarkers/metabolismABSTRACT
A novel heterometallic metal-porphyrinic framework (MPFs) built from Y and K ions as nods and meso-tetra(4-carboxyphenyl)porphyrin as linkers has been successfully synthesized and characterized. The single crystal X-ray diffraction indicated that this complex 1 exhibited a bilayered architecture of the porphyrins, which is seldom seen in MPFs. In addition, in vitro anticancer activity of complex 1 on three human breast cancer cells (BT474, SKBr-3 and ZR-75-30) was further determined.
Subject(s)
Humans , Porphyrins/chemistry , Breast Neoplasms/drug therapy , Metal-Organic Frameworks/pharmacology , Metal-Organic Frameworks/chemistry , Antineoplastic Agents/pharmacology , Antineoplastic Agents/chemistry , Reference Values , Tetrazolium Salts , Reproducibility of Results , Crystallography, X-Ray , Cell Line, Tumor , Coordination Complexes/pharmacology , Coordination Complexes/chemistry , FormazansABSTRACT
BACKGROUND: The aim of the present study was to investigate the potential effects of the 5,10,15,20-tetrakis (1-methylpyridinium-4-yl) porphyrin (TMPyP4) on the proliferation and apoptosis of human cervical cancer cells and the underlying mechanisms by which TMPyP4 exerted its actions. RESULTS: After human cervical cancer cells were treated with different doses of TMPyP4, cell viability was determined by 3-(4,5-dimethyl-2-thiazolyl)-2,5-diphenyl-2-H-tetrazolium bromide (MTT) method, the apoptosis was observed by flow cytometry (FCM), and the expression of p38 mitogen-activated protein kinase (MAPK), phosphated p38 MAPK (p-p38 MAPK), capase-3, MAPKAPK2 (MK-2) and poly ADP-ribose polymerase (PARP) was measured by Western blot analysis. The analysis revealed that TMPyP4 potently suppressed cell viability and induced the apoptosis of human cervical cancer cells in a dose-dependent manner. In addition, the up-regulation of p-p38 MAPK expression levels was detected in TMPyP4-treated human cervical cancer cells. However, followed by the block of p38 MAPK signaling pathway using the inhibitor SB203580, the effects of TMPyP4 on proliferation and apoptosis of human cervical cancer cells were significantly changed. CONCLUSIONS: It was indicated that TMPyP4-inhibited proliferation and -induced apoptosis in human cervical cancer cells was accompanied by activating the p38 MAPK signaling pathway. Taken together, our study demonstrates that TMPyP4 may represent a potential therapeutic method for the treatment of cervical carcinoma.
Subject(s)
Humans , Female , Porphyrins/pharmacology , Uterine Cervical Neoplasms/drug therapy , p38 Mitogen-Activated Protein Kinases/metabolism , Antineoplastic Agents/pharmacology , Tetrazolium Salts , HeLa Cells/drug effects , Cell Survival/drug effects , Cells, Cultured , Blotting, Western , Reproducibility of Results , Apoptosis/drug effects , p38 Mitogen-Activated Protein Kinases/antagonists & inhibitors , Cell Proliferation/drug effects , Enzyme Activation/drug effects , Caspase 3/analysis , FormazansABSTRACT
Las porfirias son enfermedades metabólicas consecuencia de fallas en la biosíntesis del hemo, caracterizadas por un patrón específico de acumulación y excreción de intermediarios, responsables de su patofisiología. En las porfirias agudas el exceso de ácido d-aminolevúlico (ALA) produce una sintomatología neuroabdominal asociada al daño oxidativo por formación de especies reactivas de oxígeno (ROS), originadas por autooxidaxión del ALA. En las cutáneas, la sintomatología es producto de la acumulación de porfirinas, que como el ALA, inducen la formación de ROS. Su desencadenamiento se precipita por factores endógenos (ayuno, estrés, hormonas) y/o exógenos (fármacos), en particular algunos anestésicos. Se presenta una revisión de los estudios bioquímicos y genéticos en pacientes con diferentes porfirias obtenidos en el Centro de Investigaciones de Porfirias y Porfirinas (CIPYP), durante los últimos 38 años, que permitieron ampliar el conocimiento sobre las bases moleculares sobre estas patologías. Se describen los logros resultantes del empleo de modelos experimentales de porfiria, inducida farmacológica o genéticamente, que contribuyeron a la clasificación de algunas drogas como prohibidas para pacientes con porfiria. Finalmente, las porfirinas generadoras de ROS, y por ende inductoras de muerte celular, tienen su aplicación para combatir infecciones por organismos hemo-deficientes como Trypanosoma cruzi y también para ser utilizadas como fotosensibilizadores en la terapia fotodinámica (TFD).
Porphyrias comprise a group of metabolic disorders of the heme biosynthesis pathway resulting in a specific accumulation and excretion of intermediates which are responsible for their pathophysiology. Acute porphyrias are characterized by acute neurovisceral symptoms due to the overproduction and accumulation of d-aminolevulinic acid (ALA) which leads to an oxidative damage resulting from the formation of reactive oxygen species (ROS). In cutaneous porphyrias, the symptomatology is a result of porphyrin accumulation which also induces ROS moulding. In both cases, their clinical signs are precipitated by endogenous factors (stress, hormones, low calories intake) and/or exogenous drugs, in particular some anaesthetics. A review of the biochemical and genetic results obtained from patients with different porphyrias, diagnosed at the CIPYP during the last 38 years is presented here, aimed at obtaining additional evidence about the molecular nature of these disorders. The achievements obtained from experimental porphyria models -pharmacologically or genetically induced- are also described, which contributed to the classification of some drugs as prohibited for their use in porphyric patients. Finally, as porphyrins produce ROS and therefore cellular death, they can be used to treat infections by heme-deficient organisms like Trypanosoma cruzi and also as photosensitizers in photodynamic therapy (TFD).
As Porfirias são doenças metabólicas decorrentes de falhas na biossíntese do Hemo, caracterizadas por um padrão específico de acumulação e excreção de intermediários responsáveis de sua patofisiologia. Nas Porfirias Agudas, o excesso de ácido δ-aminolevulínico (ALA) produz uma sintomatologia neuroabdominal associada ao dano oxidativo por formação de espécies reativas de oxigênio (ROS), decorrentes da auto-oxidação do ALA. Nas Cutâneas a sintomatologia é produto da acumulação de porfirinas, que como o ALA, induzem a formação de ROS. Seu desencadeamento precipita-se por fatores endógenos (jejum, estresse, hormônios) e/ou exógenos (fármacos), especialmente alguns anestésicos. Apresenta-se uma revisão dos estudos bioquímicos e genéticos em pacientes com diferentes Porfirias obtidos no Centro de Investigações de Porfirias e Porfirinas (CIPYP), durante os últimos 38 anos, que permitiram ampliar o conhecimento sobre as bases moleculares destas patologias. Descrevem-se as conquistas resultantes do uso de modelos experimentais de Porfiria, induzida farmacológica ou geneticamente, que contribuíram à classificação de algumas drogas como proibidas para pacientes com Porfiria. Afinal, as porfirinas geradoras de ROS e, por conseguinte, indutoras de morte celular têm sua aplicação para combater infecções por organismos hemo-deficientes como Trypanosoma cruzi e também ser utilizadas como fotossensibilizadores na terapia fotodinâmica (TFD).
Subject(s)
Humans , Anesthetics , Photochemotherapy , Porphyrias , Porphyrias/metabolism , Porphyrins , Trypanosoma cruzi , Porphyria, Erythropoietic , Protoporphyria, ErythropoieticABSTRACT
Due to interesting therapeutic properties of 177Lu and tumor avidity of tetraphenyl porphyrins (TPPs), 177Lu-tetraphenyl porphyrin was developed as a possible therapeutic compound. 177Lu of 2.6-3 GBq/mg specific activity was obtained by irradiation of natural Lu2O3sample with thermal neutron flux of 4 × 1013 n.cm-2.s-1. Tetraphenyl porphyrin was synthetized and labeled with 177Lu. Radiochemical purity of the complex was studied using Instant thin layer chromatography (ITLC) method. Stability of the complex was checked in final formulation and human serum for 48 h. The biodistribution of the labeled compound in vital organs of wild-type rats was studied up to 7 d. The absorbed dose of each human organ was calculated by medical internal radiation dose (MIRD) method. A detailed comparative pharmacokinetic study was performed for 177Lu cation and [177Lu]-TPP. The complex was prepared with a radiochemical purity: >97±1% and specific activity: 970-1000 MBq/mmol. Biodistribution data and dosimetric results showed that all tissues receive approximately an insignificant absorbed dose due to rapid excretion of the complex through the urinary tract. [177Lu]-TPP can be an interesting tumor targeting agent due to low liver uptake and very low absorbed dose of approximately 0.036 to the total body of human...
Devido às propriedades interessantes do 177Lu e da avidez tumoral das tetrafenil porfirinas (TPP), desenvolveu-se a 177Lu-tetrafenil porfirina como composto terapêutico potencial. 177Lu de atividade específica de 2,6-3 GBq/mg foi obtido por irradiação de amostra de Lu2O3 com fluxo térmico de nêutrons de 4 × 1013 n.cm-2.s-1. Sintetizou-se a tetrafenil porfirina e marcou-se com 177Lu. A pureza radioquímica do complexo foi estudada usando método de Cromatografia Instantânea de Camada Delgada ( ITLC). A estabilidade do complexo foi checada na formulação final e no ser humano por 48 h. A biodistribuição do composto marcado em órgãos vitais de ratos do tipo selvagem foi estudada por mais de 7 dias. A dose absorvida para cada órgão humano foi calculada pelo método da Dose Médica de Radiação Interna (MIRD). Estudo farmacocinético comparativo detalhado foi efetuado para o cátion 177Lu e para o [177Lu]-TPP. O complexo foi preparado com pureza radioquímica >97±1% e atividade específica de 970-1000 MBq/mmol. Os dados de biodistribuição e os resultados dosimétricos mostraram que todos os tecidos receberam uma dose absorvida aproximadamente insignificante devido à rápida excreção do complexo pelo trato urinário. O [177Lu]-TPP pode ser um agente interessante de direcionamento do tumor devido à baixa captação pelo fígado e pela dose bem baixa absorvida, de, aproximadamente, 0,036 do corpo humano total...
Subject(s)
Humans , Lutetium , Lutetium/administration & dosage , Lutetium/therapeutic use , Radioisotopes , Radioisotopes/administration & dosage , Radioisotopes/therapeutic use , Radiopharmaceuticals/administration & dosage , Radiopharmaceuticals/therapeutic use , Porphyrins/administration & dosage , Porphyrins/therapeutic use , Uses of RadiationABSTRACT
Las porfirias constituyen un grupo de trastornos causados por defectos en la vía de síntesis del grupo hemo. En la porfiria eritropoyética congénita existe deficiencia de la uroporfirinógeno sintetasa, lo que a su vez provoca acumulación de grandes cantidades de uroporfirina I en todos los tejidos; dando lugar a fotosensibilidad con lesiones mutilantes de la piel, eritrodoncia,anemia hemolítica, esplenomegalia, y fragilidad ósea. El diagnóstico definitivo se fundamenta en la demostración de la actividad deficiente de la uroporfirinógeno sintetasa, o la determinación de mutaciones específicas en el gen respectivo. El rasgo histopatológico característico muestra una ampolla subepidérmica y su tratamiento requiere colaboración multidisciplinaria. En el presente artículo se describe un caso de porfiria eritropoyética congénita con la presentación clínica dermatológica clásica...
Subject(s)
Child , Hydroxymethylbilane Synthase , Porphyrins , Blister , Porphyria, Erythropoietic , SkinABSTRACT
Photodynamic therapy (PDT), because of its good targeting, minimal invasion, and safety, is becoming a very active area in cancer prevention and treatment, in which the photosensitizers have proved to be the core element for PDT. We developed a new HPLC method for analyzing porphyrin photosensitizers using Shiseido Capcell PAK C18 (150 mm x 4.6 mm, 5 µm) as the column at 30 °C, methanol-1% aqueous solution of acetic acid as the mobile phase in a flow rate of 1.0 mL · min(-1) in a gradient elution mode, and the detection wavelength at 380 nm. This method, showing good specificity, precision, accuracy and robusty via methodology validations, can be applied to the purity test and assay of porphyrin photosensitizers, and has played a key guide role in the R&D of the new porphyrin photosensitizer--sinoporphyrin sodium.
Subject(s)
Chromatography, High Pressure Liquid , Photochemotherapy , Photosensitizing Agents , Chemistry , Porphyrins , ChemistryABSTRACT
PURPOSE: To study the retinal pigment epithelium (RPE) and retinal alterations in chronic central serous chorioretinopathy treated with photodynamic therapy, and its correlation with functional parameters such as best-corrected visual acuity (BCVA) and contrast sensitivity (CS). METHODS: Retrospective, noncomparative, consecutive evaluation by optical coherence tomography and its correlation with BCVA and CS in 31 eyes of 26 patients. RESULTS: In all affected patients, 88.5% were male with a mean age of 42.9 years. The right eye was involved in 64.5% of cases, bilateral in 19% and 73.9% were hyperopic (spherical refraction between 0 and +5.0 diopters). Of these cases, 51.5% had peri-RPE abnormalities, 17.3% hyperreflective substances at RPE, 19.4% RPE atrophy, 55.3% foveolar atrophy, 3.1% pigment epithelial detachment, 5.2% subretinal fluid persistence, 8.3% fibrin deposits, 68.4% photoreceptor inner and outer segment line interruption and 31.1% external limiting membrane interruption. CONCLUSIONS: Time evolution and number of outbreaks were related to the decrease in foveal and chorodial thickness and in those with worse BCVA and CS. RPE abnormalities and atrophy were related to the age of onset of symptoms. Photoreceptor elongation has been correlated with poor BCVA and inner and outer segment line destructuring and interruption with poor CS.
Subject(s)
Adult , Female , Humans , Male , Middle Aged , Central Serous Chorioretinopathy/diagnosis , Chronic Disease , Fluorescein Angiography , Follow-Up Studies , Fundus Oculi , Photochemotherapy/methods , Photosensitizing Agents/administration & dosage , Porphyrins/administration & dosage , Retina/diagnostic imaging , Retrospective Studies , Tomography, Optical Coherence , Treatment Outcome , Visual AcuityABSTRACT
La pseudoporfiria es una dermatosis ampollar poco frecuente con similares caracter¡sticas cl¡nicas e histopatol¢gicas a las de la porfiria cut nea tarda pero con cifras normales de porfirinas. Se presenta el caso de una paciente con pseudoporfiria asociada a hemodi lisis que fue tratada con N-acetilciste¡na.
The pseudoporphyria is a bullous dermatosis rare with similar clinical and histopathologic features to the ofporphyria cutanea tarda but with normal numbers of porphyrins. The case of a patient is presented with pseudoporphtyria associated with hemodialysis who was treated with N-acetylcysteine.
Subject(s)
Humans , Adult , Female , Acetylcysteine/therapeutic use , Hand Dermatoses , Renal Dialysis , Skin Diseases , Porphyrins , Blister , Medical IllustrationABSTRACT
El presente trabajo de investigación trata de determinar el contenido de hierro, saponinas y porfirinas en Cassia grandis L. procedente de Masaya, Chinandega y Jalapa¸ asi como, la mejor relación droga solvente y concentración de solvente para el proceso extractivo de las muestras de Cassia grandis L. , señala también, la cuantificación del contenido de saponinas y porfirinas mediante espectrofotometría UV/Visible y la valoración del contenido de hierro, por espectrofotometría de absorción atómica en muestras de Cassia grandis L.
Subject(s)
Humans , Iron , Plants, Medicinal/chemistry , Porphyrins , Saponins , NicaraguaABSTRACT
To report transient increased exudation after photodynamic therapy [PDT] of three different intraocular tumors [retinal hemangioblastoma, retinal astrocytoma, amelanotic choroidal melanoma]. PDT with verteporfin [6 mg/m[2] body surface area] was delivered at a dose of 50 J/cm[2] and intensity of 600 mW/cm[2] over 83 s. All patients experienced decreased vision within a few days following PDT. Optical coherence tomography showed development of subfoveal fluid in all cases and noncystoid intraretinal edema in the eye with juxtapapillary retinal hemangioblastoma. There was complete absorption of retinal/subretinal fluid with improvement of visual acuity to 20/20 in all cases between 3 weeks to 4 months after PDT
Subject(s)
Humans , Female , Eye Neoplasms , Hemangioblastoma , Retinal Neoplasms , Astrocytoma/drug therapy , Photosensitizing Agents , Melanoma, Amelanotic/drug therapy , Choroid Neoplasms/drug therapy , Porphyrins/analogs & derivatives , Tomography, Optical Coherence , Subretinal FluidABSTRACT
The aim of the present study was to evaluate the effect of photodynamic therapy in the treatment of experimental corneal neovascularization [NV] with benzoporphyrin derivative [BPD]. One group was considered as control [n=6 eyes] then, corneal NV was induced in 30 New Zealand male rabbits [n=60 eyes] by placing 7.0 silk sutures at midstromal depth approximately 1mm from the limbus. Fifteen rabbits with corneal NV were left without any treatment, and 15 rabbits were subjected to photodynamic therapy [PDT] by intravenous injection with Verteporfin at a dose of 1.5 mg /Kg. Diode laser [660 nm] was applied after 15 minutes for 5 minutes with a power of 50 mW/cm[2]. All rabbits were successively followed up by slit lamp examination for periods of 1 day, 1, 2, 3 and 4 weeks. Three rabbits were selected and sacrificed weekly [n=6 eyes each] and the corneas were isolated for histopathological examination. The results of slit lamp examination indicated the gradual regression of the cornea neovascularization 4 weeks of PDT. Furthermore, regression of corneal neovascularization was documented clinically by decrease number and length of blood vessels and by histopathological examination. PDT with Verteporfin can provide efficacious treatment of corneal neovascularization
Subject(s)
Animals, Laboratory , Photochemotherapy , Thrombosis , Porphyrins , Lasers, Semiconductor , RabbitsABSTRACT
PURPOSE: To report a case of Pseudomonas fluorescens infection following endoscopic dacryocystorhinostomy and silicone tube intubation in a healthy patient who was using steroid nasal spray. In addition, a literature review is conducted. CASE SUMMARY: A 72-year-old female patient came to our clinic with tearing and hyperemia in the right eye. Ten months prior, she had undergone endoscopic dacryocystorhinostomy and silicone tube intubation due to nasolacrimal duct obstruction in the right eye. Six months after the first operation, dacryocystorhinostomy revision with silicone tube exchange was performed due to obstruction of the nasal bony orifice. In addition, the patient was using a steroid nasal spray. On slit lamp examination, conjunctival injection, marked inflammation and punctal edema around the tube were observed. The silicone tube was removed and the tube cultured. Pseudomonas fluorescens was isolated from the tube contents. The patients was treated with topical 0.3% gatifloxacin 4 times a day, methylol cephalexin lysinate 1000 mg 3 times a day and the nasal spray was discontinued. Two weeks later, all symptoms were resolved after treatment with antibiotic treatment. CONCLUSIONS: A case of Pseudomonas fluorescens canaliculitis which occurred in healthy patient who was using steroid nasal spray is presented with a literature review. Pseudomonas fluorescens canaliculitis can be treated by using proper antibiotics.
Subject(s)
Female , Humans , Anti-Bacterial Agents , Cephalexin , Corneal Ulcer , Dacryocystitis , Dacryocystorhinostomy , Edema , Eye , Fluoroquinolones , Hyperemia , Inflammation , Intubation , Nasolacrimal Duct , Porphyrins , Pseudomonas , Pseudomonas fluorescens , Silicones , CanaliculitisABSTRACT
PURPOSE: We designed our study to evaluate the hypothesis that gastric cancer is correlated with iodine deficiency or thyroid dysfunction. MATERIALS AND METHODS: We investigated the total body iodine reserve, thyroid function status and autoimmune disorder in 40 recently diagnosed gastric adenocarcinoma cases versus 80 healthy controls. The participants came from a region with high gastric cancer rate but sufficient iodine supply due to salt iodination. The investigation included urine iodine level, thyroid gland clinical and ultrasonographic examination, and thyroid function tests. RESULTS: Goiter was detected more frequently in the case group (P=0.001); such a finding, however, was not true for lower than normal urine iodine levels. The free T3 mean level was significantly lower in the case group compared to the control group (P=0.005). CONCLUSIONS: The higher prevalence of goiter rather than low levels of urinary iodine in gastric adenocarcinoma cases suggests that goiter, perhaps due to protracted but currently adjusted iodine deficiency, is more likely to be associated with gastric adenocarcinoma compared to the existing iodine deficiency itself.
Subject(s)
Adenocarcinoma , Autoimmune Diseases , Goiter , Halogenation , Iodine , Porphyrins , Prevalence , Stomach Neoplasms , Thyroid Function Tests , Thyroid GlandABSTRACT
Pulmonary artery stenosis that causes pulmonary hypertension is usually observed in congenital anomalies and is seldom observed in adults. A 47-year-old female patient came to the hospital for aggravated dyspnea and peripheral edema. Severe pulmonary hypertension was found on an echocardiography examination, along with dilation of the main pulmonary artery and alias flow at the bifurcation sites of both pulmonary arteries. Under coronary angio-computed tomography, 70% high grade stenosis was observed on both sides of the proximal pulmonary arteries without showing atherosclerotic plaque or a thrombus. The patient's symptoms improved after performing pulmonary artery angiography and angioplasty. Thus, we report our experience of a patient with Behcet's disease and severe pulmonary hypertension caused by pulmonary artery stenosis, which was treated with percutaneous pulmonary angioplasty.
Subject(s)
Adult , Female , Humans , Angiography , Angioplasty , Angioplasty, Balloon , Constriction, Pathologic , Dyspnea , Echocardiography , Edema , Hypertension, Pulmonary , Plaque, Atherosclerotic , Porphyrins , Pulmonary Artery , ThrombosisABSTRACT
Hemangioma is one of the most common benign neoplasm involved on head and neck. However, cavernous hemangiomas are relatively rare in preauricular sinus space and has rarely been reported around the world. Recently, a 44-year-old female patient came to our clinic with cavernous hemangioma involving the preauricular sinus. This case was surgically managed via excision. Preoperatively, it was difficult to differentiate the mass of hemangioma clinically and diagnostically from a preauricular fistula, a salivary gland tumor, or an enchondroma. This case report describes the subcutaneous hemangioma on a preauricular sinus, which is rarely seen in Korea. The clinical presentation and management are discussed with a review of the literature.
Subject(s)
Female , Humans , Chondroma , Fistula , Head , Hemangioma , Hemangioma, Cavernous , Korea , Neck , Porphyrins , Salivary GlandsABSTRACT
Autosomal dominant hereditary spastic paraplegia (AD-HSP) is due to mutations in the "spastin" gene (SPAST gene) encoding the AAA protein. The main clinical features of "pure" HSP are progressive lower-limb spasticity with corticospinal tracts and dorsal column degeneration without peripheral neuropathy. Here we report the case of HSP with novel SPAST gene mutation that misdiagnosed with subacute combined degeneration initially. A 58-year-old man with gait disturbance came to our hospital. He was unable to regulate his steps by himself. The impaired gait began 3 years after he had undergone subtotal gastrectomy and chemotherapy for 6 months. Thereafter, he started feeling tingling sensations in the hands and feet and acquired gait difficulties. He denied having a family history of abnormal gait or developmental problem. We diagnosed him with subacute combined degeneration on the evidence of history of gastrectomy, lower normal limit of vitamin B12 (363 pg/ml), apparent absence of vibration sensations and paresthesia in the feet. He was intramuscularly administered cyanocobalamin regularly. However, there was no improvement in his condition. We reconsidered his symptoms and signs, decided to examine the SPAST gene, which is the most common mutation in HSP. The SPAST gene, c.870+1delG, heterozygote, splicing mutation is detected from the gene sample. There was no previous information of this polymorphism or mutation at this locus. We examined his two children, and the same mutation was founded in his son. We report a patient of novel SPAST gene mutation with AD-HSP which is misdiagnosed with SCD.
Subject(s)
Child , Humans , Foot , Gait , Gastrectomy , Hand , Heterozygote , Muscle Spasticity , Paresthesia , Peripheral Nervous System Diseases , Porphyrins , Pyramidal Tracts , Sensation , Spastic Paraplegia, Hereditary , Subacute Combined Degeneration , Vibration , Vitamin B 12ABSTRACT
Potamotrygon motoro, also known as the Marble motoro, is a potamodromous freshwater ray native to the basins of the Amazon River. Marble motoros were introduced to South Korea in the 2000s, and, because they are easy to raise, were sold as aquarium fish. The aim of this report is to illustrate a new case involving envenomation by a Marble motoro. A 35-year-old commercial aquarium assistant came to the hospital after being pricked by a Marble motoro. The clinical picture in this case showed acute local pain with minimal systemic manifestations. This patient recovered after receiving symptomatic treatment and wound care. This case of envenomation accentuates the potential for injury among people coming in contact with a venomous Marble motoro.
Subject(s)
Adult , Humans , Calcium Carbonate , Fresh Water , Porphyrins , Republic of Korea , Rivers , Spine , VenomsABSTRACT
The brief history of photodynamic therapy (PDT) research has been focused on photosensitizers (PSs) and light delivery was introduced recently. The appropriate PSs were developed from the first generation PS Photofrin (QLT) to the second (chlorins or bacteriochlorins derivatives) and third (conjugated PSs on carrier) generations PSs to overcome undesired disadvantages, and to increase selective tumor accumulation and excellent targeting. For the synthesis of new chlorin PSs chlorophyll a is isolated from natural plants or algae, and converted to methyl pheophorbide a (MPa) as an important starting material for further synthesis. MPa has various active functional groups easily modified for the preparation of different kinds of PSs, such as methyl pyropheophorbide a, purpurin-18, purpurinimide, and chlorin e6 derivatives. Combination therapy, such as chemotherapy and photothermal therapy with PDT, is shortly described here. Advanced light delivery system is shown to establish successful clinical applications of PDT. Phtodynamic efficiency of the PSs with light delivery was investigated in vitro and/or in vivo.