Give me a break: Gerhard Küntscher and his nail.

The treatment of broken bones has long adhered to principles of tight juxtaposition of fracture ends and of strict rest. Splinting and casting with plaster of Paris helped considerably. Attempts at internal splinting with pegs, bolts, or stabilizing contraptions usually failed. Foreign materials were either not biocompatible or broke easily, and the insertion of these prostheses directly into the fracture created additional risks from infections. All this changed when Gerhard Küntscher (1900-1972) stabilized broken long bones by intramedullary insertion of stainless steel nails through a small incision, distant from the fracture. Küntscher employed his method from the late 1930s on, initially as a surgeon at the Kiel University in Germany. His early and encouraging results were rejected when presented in 1940, and he remained ostracized thenceforth. During WWII he was stationed in Finland, where he treated wounded soldiers from both sides of the front. POWs returning home propagated the new method by revealing these rods in roentgenograms of their legs; had he not been so successful in treating POWs, he might have had to stand trial at the Nuremberg Doctors' Trial (1947). He documented his experiments and experiences in more than 200 articles and three monographs. Today, his method is employed universally.

Recommendations for the diagnosis and treatment of the acute porphyrias.

The acute porphyrias, 4 inherited disorders of heme biosynthesis, cause life-threatening attacks of neurovisceral symptoms that mimic many other acute medical and psychiatric conditions. Lack of clinical recognition often delays effective treatment, and inappropriate diagnostic tests may lead to misdiagnosis and inappropriate treatment. We review the clinical manifestations, pathophysiology, and genetics of the acute porphyrias and provide recommendations for diagnosis and treatment on the basis of reviews of the literature and clinical experience. An acute porphyria should be considered in many patients with unexplained abdominal pain or other characteristic symptoms. The diagnosis can be rapidly confirmed by demonstration of a markedly increased urinary porphobilinogen level by using a single-void urine specimen. This specimen should also be saved for quantitative measurement of porphobilinogen, 5-aminolevulinic acid, and total porphyrin levels. Intravenous hemin therapy, started as soon as possible, is the most effective treatment. Intravenous glucose alone is appropriate only for mild attacks (mild pain, no paresis or hyponatremia) or until hemin is available. Precipitating factors should be eliminated, and appropriate supportive and symptomatic therapy should be initiated. Prompt diagnosis and treatment greatly improve prognosis and may prevent development of severe or chronic neuropathic symptoms. We recommend identification of at-risk relatives through enzymatic or gene studies.

Did Ulysses have porphyria?

Although the biosynthetic pathway to heme has been well elucidated and errors along that route have been identified and firmly connected to specific diseases, the porphyrias, slight but nonspecific abnormalities, are occasionally invoked as proof of porphyria or in support of other diagnoses. An errant patient with a conundrum of symptoms but without an explanation for them might have to take iatrogenic detours only to learn after what are at times ulyssean vagaries that the initial diagnosis of porphyria is in the end untenable. Thus the porphyrias are superb examples of the interface between laboratory and clinical medicine, in which the occurrence of the Ulysses syndrome can be curtailed through the careful ordering of tests and cogent interpretation of their results.