Pediatric head and neck manifestations associated with multiple endocrine neoplasia syndromes.

INTRODUCTION: Multiple endocrine neoplasia (MEN) syndromes are a group of hereditary cancer syndromes that can predispose children to endocrine neoplasms developing within the head and neck. OBJECTIVE: To examine the neoplastic manifestations of MEN type 1 (MEN1) and MEN type 2 (MEN2) in the pediatric head and neck. METHODS: Single-institution, retrospective review of pediatric MEN between 2005 and 2022. RESULTS: Fifty-three children were genetically confirmed with MEN (15 MEN1, 34 MEN2A, and 4 MEN2B), while three patients received clinical diagnoses of MEN1. The male to female ratio was essentially equal (1.15:1), and a documented family history of cancer was present in 89% (50/56). After multidisciplinary evaluation, a familial MEN diagnosis was confirmed in 91% (51/56). The mean ages of initial presentation and surgical intervention were 8.9 years (SD 5) and 9.8 years (SD 4.8), respectively. Although patients with MEN2 received surgery earlier than patients with MEN1 (8.7 vs 12.7 years), surgical patients with MEN2 in this cohort were older relative to current American Thyroid Association (ATA) guidelines primarily due to late presentation. Thyroid malignancies were identified in 36% (9/25) of thyroidectomy specimens (21 MEN2A, 4 MEN2B), with medullary thyroid carcinoma (MTC) present in five MEN2A patients and three MEN2B patients (89%), and papillary thyroid carcinoma (PTC) present in one MEN2A patient (11%). Nearly 90% (8/9) of thyroid malignancies were occult, with some occurring earlier than predicted by current guidelines (ATA-MOD and ATA-H). Central neck dissections were performed in 24% (2 MEN1, 2 MEN2A, and 4 MEN2B), with two MEN2B (50%) demonstrating cervical lymph node (LN) metastases. Additional histopathologic findings included C-cell hyperplasia in 57% (12/21) of MEN2A thyroidectomy patients. Of the eight MEN1 parathyroidectomy patients, four demonstrated parathyroid hyperplasia and four presented with parathyroid adenoma. CONCLUSION: Nearly 60% required head and neck procedures. While MEN1 guidelines were appropriate for our cohort, we identified patients with MEN2 that developed MTC earlier than expected based on current ATA guidelines, including children in categories considered lower risk. In conjunction with a multidisciplinary approach, pediatric head and neck surgeons should be aware of the potential need for earlier surgical intervention in the pediatric MEN2 population.

[Ambulatory treatment of hypertension]. / Ambulante Behandlung der Hypertonie.

In an open study 30 hypertensive outpatients have been treated with Indapamide in a dosage of 2,5 mg daily in the morning for eight weeks. In the course of this treatment a highly significant decrease of blood pressure could be observed. The antihypertensive effect came up gently in the beginning. Indapamide has been well tolerated by all patients. The rate of adverse reactions was low. Syncopal episodes did not occur. The biochemical data monitored (fasting glucose level, creatinine, urea, potassium, sodium, calcium, chloride) did not show any significant deviation. According to the present data Indapamide may be used as basic medication in the treatment of genuine hypertension. The gentle effect in the beginning prevents syncopal episodes, however a sufficient period of treatment, at least eight weeks, is needed to assess the success of the therapy.

Effect of temperature and calcium on the diastolic length-tension relationship of rat heart muscle.

Experiments were performed on strips from the right ventricle of the rat in order to investigate the influence of temperature and Ca++-removal on the length-tension relationship. Temperature change (22-38 degrees) had no effect on the resting, non-stimulated muscle. The isometric minima curves of the electrically stimulated preparations at 18 degrees and 38 degrees (diastolic length-tension relationship), however, were shifted to the right compared to those at 28 degrees. When temperature decreased (at fixed muscle length), the diastolic tension surmounted a maximum at the optimal temperature (28 degrees) and declined with further cooling. Moreover the electrolyte exchange characteristics (Ca, K, Na), which follow upon a variation in temperature, were studied. The calcium content of the stimulated preparations showed a similar dependence on temperature as the diastolic tension, indicating that calcium might play a role with respect to the influence of temperature on the diastolic length-tension relationship. Indeed an addition of EDTA to the bath solution caused a parallel shift of the isometric minima curve to the right. These results show that an active mechanism might contribute to the increase of diastolic tension in response to stretch.

[Transfemoral embolus aspiration from both renal arteries]. / Transfemorale Embolusaspiration aus beiden Nierenarterien.

In a 50-year-old female patient who had suffered from coronary heart disease for about 3 years, a cerebral embolus resulted in left-sided hemiparesis. Four days later she became anuric. Acute thromboembolis occlusion of the renal arteries was assumed and renal angiography was performed. The angiography showed complete obstruction of the left renal artery and partial obstruction of the right renal artery. Due to the poor general condition of the patient it was not possible to carry out renovascular surgery or streptokinasetherapy. An attempt was therefore made to remove the emboli from the renal arteries by a new technique (transfemoral embolus aspiration). The subsequent angiography showed normal circulation in the right kidney and improved circulation in the left kidney. Ten days of dialysis treatment were required until urine production started again. Death then occurred from other causes. The autopsy confirmed free permeable renal vessels and absence of permanent sequelae in the parenchyma.

[Heart tumour in the right atrium: clinical recognition and therapeutical management (author's transl)]. / Herztumor im rechten Vorhof: Diagnostik und Therapie.

A 62 year old lady came to the hospital in cause of right heart failure. Right and left heart catheterization and cineangiocardiography revealed a tumour in the right atrium. Further angiographic investigations demonstrated a hypernephroma in the left kidney. The operation of both tumours was successful. Pathohistological examination confirmed the diagnosis of hypernephroma in the left kidney with metastasis into the right atrium.

Effect of beta-sympatholytic agents on vascular responses to noradrenaline and potassium chloride.

Vasoconstrictory responses to noradrenaline (NA) or high potassium chloride (130 mM) usually show a biphasic behaviour: an initial peak is followed by a lower steady state level. The influence of three beta-sympatholytic agents (propranolol, pindolol, practolol) and a "Ca-antagonist" (verapamil) on this particular behaviour of KCl-induced vasoconstrictions was compared with responses to NA. Studies were performed on the intact vascular bed of an isolated intestinal preparation of the rat. 1. Increases in the concentration of propranolol and pindolol from 10(-10) to 10(-6) M attenuated the vascular responses to high KCl. Practolol, however, distinctly enhanced the KCl-responses. NA (1.3 microgram/ml)-elicited vasoconstriction was found to be influenced in a similar manner by the beta-receptor antagonists used, though lower concentrations of pindolol had an enhancing, higher concentrations a reducing effect on the constrictory responses to NA. 2. The extent to which the biphasic response adjusted to the steady state level was concentration-dependently increased KCl-responses and significantly decreased to a monophasic response to NA, in the presence of the beta-sympatholytic agents. 3. By using a quotient of deltaPs (steady-state phase) to deltaPi (initial vasoconstriction) and by comparing the effect of the three beta-sympatholytic agents with that of verapamil on this quotient showed a concentration-dependent decrease in presence of the beta-sympatholytic as well as of the Ca-antagonistic agents. These myotropic actions, i.e. negative influences on the mechanism of vascular smooth muscle activation ranged in the following sequence: Verapamil greater than propranolol greater than pindolol greater than practolol. 4. The mechanism underlying the biphasic responses to high potassium chloride are concluded to be basically different from the NA-responses because they are inversely affected by beta-sympatholytic agents. The steady-state response to KCl was found to be very sensitive to non-specific pharmacological actions of beta-sympatholytic substances. The myotropic action of the beta-receptor antagonists is compared with that of the Ca-antagonist verapamil and the role of calcium in the mechanism of activation of vascular smooth muscle is discussed.

[Mitral valve replacement in large hearts (author's transl)]. / Der Miralklappenersatz bei grossen Herzen.

Of 61 patients with mitral valve defects operated on, 9 had a heart with an external volume of more than 1700 ml. Five of these patients died, four are still alive today. Three patients from the group with an external heart volume between 1247 ml and 1700 ml died, and in the group of patients with an external heart volume of less than 1247 ml no patient has died as yet. Under certain circumstances, the preoperative size of the heart can be a measure with which the limits of surgical mitral valve replacement can be defined.

Arteriosclerotic coronary arterial aneurysms in a 49-year-old man with crescendo angina: family history, natural course and prevalence.

In a 49-year-old man with crescendo angina, elevated serum cholesterol level and an old posterior myocardial infarction, selective coronary arteriography showed multiple arteriosclerotic aneurysms of the right coronary artery associated with extensive and severe arteriosclerotic disease of the left coronary artery. The patient's mother and brother have both died of a myocardial infarction. Another brother suffers from angina and has documented arteriosclerotic coronary artery disease. Two sisters suffer from angina as well. The possibility of embolization of distal vessels from a friable clot of the aneurysms as a cause of the patient's infarction is discussed. To the best of our knowledge, this is the tenth patient with nonfistulous arteriosclerotic coronary artery aneurysm diagnosed and documented angiographically ante mortem. Including the present case and reviewing the literature, the prevalence of this condition among nonfistulous coronary aneurysms diagnosed ante mortem is 35 per cent and henceforth cannot be regarded as an incidental autopsy finding in cardiac asymptomatic patients.

[Coronary arteriography in threatened myocardial infarction. Performance and results (author's transl)]. / Die Koronarographie bei drohendem Herzinfarkt. Durchführung und Ergebnisse

Within 12 months, 315 coronary arteriographies were performed by the Sones-technique. There were no deaths. 6 of these patients with impending infarction were investigated as out-patients. Six patients with impending reinfarction were hospitalized before coronary arteriography. Nine patients were transferred to the Department of Cardiovascular Surgery of the University Hospital of Erlangen within 4-24 hours after the beginning of coronary arteriography. For one patient there was a transfer delay of 48 hours and in a second case 2 months. No problems arose from the geographical separation of both departments and the ambulant catheterization in certain cases.

[Significance of risk factors of functional disorders of pacemakers (author's transl)]. / Die Bedeutung von Risikofaktoren in der Herzschrittmacher-Uberwachung

In spite of increasing reliability of pacemakers and expensive electronic monitoring systems unforeseen disorders of the regular stimulation of the heart cannot be prevented with certainty. Criteria are given by which the individual risk of the carrier of a pacemaker can be determined, so that the degree of danger can be foreseen. Individual methods of monitoring and in particular checking intervals adapted to the risk are suggested.

[Coronary angiography in the pre-infarct syndrome (author's transl)]. / Koronarographie beim Prä-Infarktsyndrom

During the first ten month of the past year (1974) we did 310 selective coronary arteriographies by the Sones technique at the Department of Cardiology of the Medical Clinic Würzburg. The incidence of complications in this comparatively small group was 0%. In seven cases we did an emergency coronary arteriography. Six of these patients were transferred immediately after examination to the Department of cardiac Surgery of the University of Erlangen for emergency aortocoronary vein bypass. One of these patients died within the next 6 hours postoperatively. Five patients in this group are in good condition. Three patients had a double aorto-coronary vein bypass.

[A new method of transseptal levocardiography]. / Uber eine neue Technik der transseptalen Lävokardiographie

Perforations of the left atrial or ventricular wall and extravasations of contrast medium during transseptal left heart catheterization or angiocardiography can be eliminated by replacing the routinely used transseptal catheters with Pigtail-catheters. With 2.8% minor complications without sequelas in 181 successful studies, transseptal angiocardiography of the left heart through Pigtail-catheters is not only less hazardous than injections through the transseptal catheters employed up to now, but bears even less risk than direct retrograde injection into the left ventricle. To show the left atrial cavity and the mitral valve, transseptal left atrial injection is the method of choice. For quantitative angiocardiography to evaluate left ventricular function, transseptal angiocardiography with injection into the left atrium is superior to retrograde direct ventriculography in our experience, as ventricular ectopic beats were absent and supraventricular ectopic beats as rare as 5% of the cases, local disturbances of wall motion during injection could be avoided and 1 or 2 more cycles could be evaluated before the depressant effect of contrast medium started.