Fractional Flow Reserve: The Ideal Parameter for Evaluation of Coronary, Myocardial, and Collateral Blood Flow by Pressure Measurements at PTCA

To overcome the fundamental limitations of coronary arteriography to assess the functional significance of coronary artery disease, it is necessary to obtain direct information about coronary blood flow. Recently we validated three pressure flow equations, which enable calculation of maximum coronary, myocardial, and collateral flow by merely measuring aortic, central venous, and distal coronary pressures under the condition of maximum vasodilation and using an ultra thin pressure monitoring guide wire for distal coronary pressure recording. In this paper, the first clinical experiences of this method are described. For that purpose, the concept of fractional flow reserve (FFR) is important. Fractional coronary flow reserve (FFR_cor) is defined as the maximum achievable blood flow in a stenotic artery, divided by normal maximum flow in that same artery, i.e. maximum flow in that artery in the case that it would be completely normal. Fractional myocardial flow reserve (FFR_myo) is defined in a similar way, and recruitable collateral blood flow is expressed as a fraction of normal maximum myocardial flow. Fractional flow reserve, defined in this way, is easy to obtain at percutaneous transluminal coronary angioplasty (PTCA) by the pressure-flow equations, is independent of pressure changes, applicable to three vessel disease, and enables calculation of the separate contribution of coronary and collateral flow to total myocardial perfusion. In 18 patients a very close correlation was demonstrated between FFR_myo, calculated by pressure recordings at PTCA by the first pressure flow equation, and FFR_myo obtained by positron emission tomography, which is considered the gold standard for myocardial perfusion. In 60 other patients, maximum recruitable collateral blood flow at balloon inflation (Q_c/Q^N) was calculated according to the third pressure-flow equation and correlated to the presence or absence of ischemia. It could be demonstrated that Q_C/Q^N exceeds 22% in all 23 patients without ischemia, whereas Q_c/Q^N was less than 22% in 34 out of 37 patients who experienced ischemia during balloon inflation. This margin value of 22% is very close to the theoretically expected value of 20%. based upon a coronary flow reserve of 5 under standard physiologic conditions. It can be concluded that the concept of fractional flow reserve provides a rapid, accurate, and elegant way for quantitative assessment of maximum coronary and myocardial blood flow before and after PTCA. Moreover, this is the first method that enables quantitative calculation of collateral blood flow in clinical practice. (J Interven Cardiol 1993; 6:331–344)     

Episodic erythema gyratum repens with ichthyosis and palmoplantar hyperkeratosis without signs of internal malignancy

Two patients with typical lesions of erythema gyratum repens, peripheral ichthyosis, palmoplantar hyperkeratosis and nail changes are described. A non-specific erythrodermic eruption of several weeks' duration had preceded the typical lesions. No signs of internal malignancy were found and the typical gyrate lesions disappeared within some weeks with full restitution of all skin lesions within 6-8 months.     

The Fanconi-Bickel syndrome: a case of neonatal onset.

A male newborn infant was recognized having Fanconi-Bickel syndrome (FBS) in the neonatal period. The presenting clinical findings were hyperglycemia and polyuria detected during an episode of acute enteritis. Physical examination was normal, biochemical analyses were suggestive of FBS: glycosuria, proteinuria, phosphaturia, generalized aminoaciduria, and increased levels of urinary beta 2-microglobulin, serum glucose and serum alkaline phosphatase. The molecular genetic analysis showed homozygosity for mutations within the gene of the glucose transporter 2 (Glut 2), 1213 C>T. The patient demonstrated improved clinical and metabolic status following institution of diet with frequent small meals and galactose-free-milk as well as pharmacological treatment with phosphate and vitamin alpha-OH-D3. In conclusion, infants showing hyperglycemia and polyuria may be considered having FBS also in the neonatal period. Early institution of adequate caloric intake and replacement of electrolytes and vitamin D may avoid or reduce metabolic complications.     

Cryosurgery in cutaneous leishmaniasis

Thirty patients with cutaneous leishmaniasis were treated with cryotherapy using a CO₂ cryomachine and all were cured without noticeable scarring within 4–5 weeks, with no relapse. Histopathological examination showed that cryotherapy eradicated all parasites in less than 1 hour. Leishmania tropica, L. ethiopica and L. brasiliensis are all markedly thermosensitive and thus cryotherapy seems parasiticidal to all types. Adequate cryotreatment of cutaneous leishmaniasis will preclude the development of mucocutaneous extension, and its use in mucocutaneous disease is also recommended.