Der Internist
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A 26-year-old female presented to the emergency department due to abdominal pain in spite of painkillers. After extensive clinical diagnostics, no specific cause could be found. ⋯ Drugs that were known to be triggers were avoided and treatment with heme arginate and glucose was started. In addition, treatment of the delirium and infection led to a complete remission of symptoms.
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Porphyrias are caused by enzyme defects along the heme biosynthetic pathway. The first line diagnosis of porphyria is based on specific biochemical patterns of elevated porphyrins and porphyrin precursors in urine, feces, and blood. In clinically active disease accumulated porphyrin precursors and/or porphyrins lead to abdominal, neurologic, psychiatric, endocrine and cardiovascular symptoms, liver damage and/or skin photosensitivity. ⋯ Patients with symptomatic (clinically active) acute hepatic porphyria, e.g. acute intermittent porphyria, porphyria variegata, hereditary coproporphyria, and aminolevulinic acid dehydratase deficiency porphyria, display accumulation of porphyrin precursors, 5‑aminolevulinic acid and porphobilinogen due to regulation disorder. In the non-acute forms of porphyria, such as porphyria cutanea tarda, erythropoietic porphyria, X‑linked protoporphyria and congenital erythropoietic porphyria, accumulated porphyrins lead to skin photosensitivity and occasionally also to severe liver damage. Several different options for treatment, proven and innovative ones, are available for most porphyrias.
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Case Reports
[Deep lumbar back pain and neurological sensory deficits in a 56-year-old male patient].
Echinococcus infestations are rare in humans, infestation of bone occurs in less than 1% and a primary spinal infestation is extremely rare. This article reports on a clinical case of lumbar and sacral spinal infestation by Echinococcus multilocularis in a 56-year-old male Caucasian with neurological sensory deficits and deep lumbar back pain. ⋯ A radical surgical débridement was carried out and anthelminthic treatment was started. This article describes this unusual case in detail and gives a brief summary of the current literature on this disease.