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Hands & Feet
Published in Richard Ashton, Barbara Leppard, Differential Diagnosis in Dermatology, 2021
Richard Ashton, Barbara Leppard
This is an acquired porphyria and the one most likely to be seen in clinical practice. It is due to reduced levels of uroporphyrinogen decarboxylase in the liver resulting in increased levels of urophorphyrins in the urine and plasma. It is caused by excessive alcohol intake (2% of alcoholics develop PCT), hepatitis C, subclinical haemochromatosis or the use of oestrogens or HRT. The patient is usually a middle aged or elderly male (less common in females), who presents with blisters, scars and milia on sun exposed skin – dorsum of hands and forearms, face and bald scalp. There may also be hypertrichosis on the face. The patient rarely associates the development of skin lesions with sunlight. The diagnosis is made by finding increased porphyrins in the urine. A routine sample must be sent in a container which should be protected from light with aluminium foil. The urine can be screened using a Wood's (ultra-violet) lamp where a coral pink fluorescence is seen. Acidifying the urine or adding talc to it makes the fluorescence more obvious. Detailed analysis of the various porphyrins by a specialist laboratory will confirm the type of porphyria. Check also the liver enzymes, hepatitis C antibodies, serum ferritin and look for the haemochromatosis gene mutation.
Application of Bioresponsive Polymers in Drug Delivery
Published in Deepa H. Patel, Bioresponsive Polymers, 2020
Manisha Lalan, Deepti Jani, Pratiksha Trivedi, Deepa H. Patel
Photodynamic therapy (PDT) by porphyrins and related tetrapyr-role derivatives is treatment modality of age-related macular degeneration (AMD). This therapy combines the administration of porphyrins or porphyrin precursors and use of red light at the diseased sites.
Medicine
Published in Seema Khan, Get Through, 2020
Synthesis of heme, an important constituent of haemoglobin, occurs in the liver. The process is complex in which a large number of enzymes are needed at each step to convert haem precursors (known as porphyrins) into haem itself. Enzyme deficiency can occur at any step of the process leading to accumulation of porphyrins and to symptoms of porphyria. Porphyria is in fact a group of disorders which includes acute intermittent porphyria, cutaneous porphyria and mixed porphyria. Drugs, particularly those containing hormones (e.g. the pill or HRT), infections, smoking and alcohol are known precipitants.
Porphyromonas gingivalis diffusible signaling molecules enhance Fusobacterium nucleatum biofilm formation via gene expression modulation
Published in Journal of Oral Microbiology, 2023
Yukiko Yamaguchi-Kuroda, Yuichiro Kikuchi, Eitoyo Kokubu, Kazuyuki Ishihara
The expression of fap2 increased significantly in cocultured F. nucleatum. Fap2 is a galactose inhibitable adherence factor involved in coaggregation between F. nucleatum and P. gingivalis, hemagglutination by F. nucleatum [17], and localization of F. nucleatum to colon tumors [36]. The results indicate that the sensing of diffusible signaling molecules from P. gingivalis by F. nucleatum induces an increase in fap2. The increase in Fap 2 may contribute to binding between the species and enhance these intimate interactions, although further analysis is required to confirm the magnitude of contribution. Fap2 is also involved in hemagglutination by F. nucleatum [17]. P. gingivalis has hemagglutination activity, which is involved in heme acquisition [37]. In the cocultured F. nucleatum, cobK and cbiJ expression increased. These genes are involved in porphyrin metabolism. It is possible that hemagglutination is involved in iron acquisition in F. nucleatum. Therefore, the increase in Fap2 expression may be one of the adaptations for establishing a synergistic community of F. nucleatum and P. gingivalis.
Antiperspirant effects and mechanism investigation of Mulisan decoction in rats based on plasma metabolomics
Published in Pharmaceutical Biology, 2022
Shan-Peng Ma, Wei-Ping Ma, Shi-Ning Yin, Xiang-Yue Chen, Xiao-Qing Ma, Bao-Hong Wei, Jing-Guang Lu, Hong-Bing Liu
Protoporphyrin IX is a critical metabolite in porphyrin metabolism, whose level is vital to the health of the body. It can promote cell tissue respiration and improve protein and glucose metabolism (Zhu et al. 2020). Protoporphyrin IX is the intermediate product in the process of haemachrome and a precursor of haemoglobin (Immenschuh et al. 2017). Abnormal porphyrin metabolism can lead to the accumulation of porphyrins in vivo, and then damage the function of mitochondria. Mitochondria can convert food and oxygen into adenosine triphosphate (ATP) through the respiration effect to meet the large energy needs of the body (Paes et al. 2020). In this study, MLS could significantly increase the level of protoporphyrin IX in RP rats, indicating that MLS could improve porphyrin metabolism and reduce porphyrin accumulation in RP rats to restore cell mitochondrial function and promote energy production. This is beneficial to improve sweating symptoms in RP rats.
Organophosphate insecticide poisoning with monocrotophos-induced fabricated illness in a 7-year-old girl with refractory seizures over a 4-year period
Published in Paediatrics and International Child Health, 2022
Chisambo Mwaba, Chalilwe Chungu, Ronald Chola, Kafula Lisa Nkole, Somwe Wa Somwe, Evans Mpabalwani
Because the patient presented with recurrent episodes of non-specific abdominal pain, vomiting, seizures, tremulous movements and passage of red urine, a diagnosis of AIP was considered. AIP is a rare disease involving the hepatic haeme synthesis pathway. It has an estimated prevalence of 1/20,000 and is inherited as an autosomal dominant disease with variable penetrance [15]. There are various clinical presentations and laboratory findings [15]. Patients may present with many of the symptoms which this patient had, including numerous episodes of non-specific abdominal pain, vomiting, negative urine porphyrin results, seizures and confusion. Medical teams who are presented with CFIC patients have often been thrown off course by pursuing the diagnosis of a rare disease, thus delaying the diagnosis of CFIC [16]. Current recommendations are that organic disease does not necessarily have to be ruled out in order to consider CFIC in the differential diagnosis [2].