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PULMONARY-HILUM CLAMP
235 On. Nov. 25, 1954, further radiographs showed both 8/8 high up above the still unerupted 7/7 and corresponding to a dental age of 10 years. There was no change of calcification in the remaining teeth during the intervening eighteen months but an apical area had developed on the/2. From this dental evidence some factor appears to have supervened at about the age of 8 years or over, preventing the full development and calcification of the teeth. Progress and Treatment.-Idiopathic hypoparathyroidism was diagnosed in view of the low serum-calcium level, high serum-phosphorus level, normal radiological appearances of the bones, absence of renal insufficiency, and absence of steatorrhoea. Despite the patient’s suggestive facies and stature, the case was not considered to belong to the pseudohypoparathyroid variety (Albright et a]. 1942) in view of the absence of dyschondroplasia and subcutaneous calcification (MacGregor and Whitehead 1954, Robinson et a]. 1954). The macrocytic anaemia was noted but examination of the bone-marrow at this time was inconclusive. Anticonvulsant drugs were withdrawn for a week in June, 1954, and treatment was started with dihydrotachysterol 1 ml. (1-25 mg.) The former was t.d.s. and calcium lactate gr. 25 t.d.s. gradually increased to 20 ml. daily, but the serum-phosphorus The level remained higher than the serum-calcium level. Hb increased at first but later decreased again. In November, 1954, examination of the blood showed Hb 42% (6-2 g. per 100 ml.), colour-index 1-35, leucocytes 2700 per c.mm., mean corpuscular volume 110 c.[.L, reticulocytes 0-8%, and serumbilirubin level 1-4 mg. per 100 ml. Sternal puncture showed severe megaloblastic dysplasia of the bone-marrow. CyanoThe Hb cobalamin 50 jjt.g. was given daily by injection. increased rapidly and after five days there was a reticulocytosis of 8%. Calciferol 200,000 units daily was substituted for the dihydrotachysterol and the serum-calcium level considerably improved. No fit had been recorded since March, 1954, and the patient now felt very well, and the Further electrosigns of latent tetany had disappeared. encephalograms showed improvement. On Nov. 4, 1954, there was an alpha rhythm at 9 c.p.s. of moderate amplitude and fair symmetry. There was excess diffuse episodic theta activity at 3-6 c.p.s. No specific epileptic waves or focus seen. On Dec. 10, 1954, the electro-encephalogram could be considered within normal limits for the patient’s age. - FoHoM’-p.—When seen as an outpatient in February, 1955, the patient’s Hb was 100%, serum-calcium level 8-4 mg. per 100 ml., and serum-phosphorus level 11-4 mg. per 100 ml. Discussion
The nature of the anaemia is an interesting problem. Three main possibilities were considered : (1) a true pernicious anaemia ; (2) the result of long-continued dosage with phenytoin sodium ; and (3) the result o the hypoparathyroidism. True pernicious anaemia is rare under the age of 40. Murphy (1939), in 550 patients seen personally, found only 1 aged less than 20. Nevertheless the histaminefast achlorhydria, the typical peripheral blood picture, the frank megaloblastic marrow in October, 1954, and the rapid response to cyanocobalamin point strongly to true pernicious anaemia. As regards phenytoin sodium Hawkins and Meynell (1954) described a case in which a megaloblastic change was thought to have been induced by phenytoin sodium. Their patient, however, had free gastric acid. The hypoparathyroidism is considered to be the least likely cause. While the patient was receiving dihydrotachysterol 20 ml. daily, her Hb was decreasing rapidly. After a month’s treatment with calciferol there was much improvement in the electro-encephalograms, which could be considered normal for the first time (the first record having been taken in March, 1953). This suggests a definite relationship between the treatment, which produced a rise in the serum-calcium level, and the improvement noted electro-encephalographically. Robinson et al. (1954) suggest that the basic electrical rhythm is responsible for the abnormal pattern in the electro-encephalogram ; but this -case supports the hypothesis that the changes are largely due to a
biochemical
abnormality.
The appearance of the teeth indicates retarded development due to some factor supervening about the age of 8 years. The epilepsy in this case also began at 8 years, and one can assume the onset of hypoparathyroidism near that age. This dental finding fully agrees with the suggestions of Albright and Strock (1933). Calciferol was as efficacious as dihydrotachysterol in bringing the serum-calcium and serum-phosphorus levels back to normal and was less expensive. The serumphosphorus level was very high again in February, 1955, although the serum-calcium level was normal; this indicates a renal tubular defect with increased resorption of phosphorus.
Summary A with
of
idiopathic hypoparathyroidism presenting epilepsy from the age of 8 years and recently developing megaloblastic anaemia is described. The probable cause of the anaemia is discussed. case
I wish to thank Dr. H. Hilton Stewart for permission to case, which was under his care, and for his advice.
report this
REFERENCES
Albright, F., Burnett, C. H., Smith, P. H., Parson, W. (1942) Endocrinology, 30, 922. — Strock, M. S. (1933) J. clin. Invest. 12, 974. Hawkins, C. F., Meynell. M. J. (1954) Lancet, ii, 737. MacGregor, M. E., Whitehead, T. P. (1954) Arch. Dis. Childh. 29, 398. Murphy, W. P. (1939) Anemia in Practice. Philadelphia and London: p. 195.
Robinson, P. K., Carmichael, E. A., Cumings, J. N. (1954) Quart. J. Med. 23, 383.
New Inventions PULMONARY-HILUM CLAMP THE clamp illustrated here was devised about two years ago for use in the thoracic unit in Manchester and has been used by several surgeons in many pulmonary
resections. There are occasions
when, owing to inflammation or to neoplastic enlargement of glands, the distal ends of the pulmonary vessels are difficult to secure satisfactorily
either by ligature or by the commoner clamps-e.g., straight Spencer Wells, Roberts forceps, and cholecystectomy forceps. Sometimes a Roberts or a cholecystectomy forceps could be applied backwards to the distal ends of the vessels, or alternatively the vessels could be cut distally and allowed to "bleed back."
In such
a
situation
a
fairly strong clamp with an angled shank and a reversed curve on its blades, which are longitudinally serrated but no wider than a Roberts forceps, gives most satisfactory control without fear of slipping. This instrument has a strong box-joint so that it can also be used, if necessary, as a distal clamp on the bronchus without straining it. I am indebted to Mr. H. Woodall, of Allen & Hanburys, for having the clamp made to my specifications. All inquiries should be addressed
to him.
P. JEWSBURY Blackpool Victoria Hospital
F.R.C.S.
The nature of the anaemia is an interesting problem. Three main possibilities were considered : (1) a true pernicious anaemia ; (2) the result of long-continued dosage with phenytoin sodium ; and (3) the result o the hypoparathyroidism. True pernicious anaemia is rare under the age of 40. Murphy (1939), in 550 patients seen personally, found only 1 aged less than 20. Nevertheless the histaminefast achlorhydria, the typical peripheral blood picture, the frank megaloblastic marrow in October, 1954, and the rapid response to cyanocobalamin point strongly to true pernicious anaemia. As regards phenytoin sodium Hawkins and Meynell (1954) described a case in which a megaloblastic change was thought to have been induced by phenytoin sodium. Their patient, however, had free gastric acid. The hypoparathyroidism is considered to be the least likely cause. While the patient was receiving dihydrotachysterol 20 ml. daily, her Hb was decreasing rapidly. After a month’s treatment with calciferol there was much improvement in the electro-encephalograms, which could be considered normal for the first time (the first record having been taken in March, 1953). This suggests a definite relationship between the treatment, which produced a rise in the serum-calcium level, and the improvement noted electro-encephalographically. Robinson et al. (1954) suggest that the basic electrical rhythm is responsible for the abnormal pattern in the electro-encephalogram ; but this -case supports the hypothesis that the changes are largely due to a
biochemical
abnormality.
The appearance of the teeth indicates retarded development due to some factor supervening about the age of 8 years. The epilepsy in this case also began at 8 years, and one can assume the onset of hypoparathyroidism near that age. This dental finding fully agrees with the suggestions of Albright and Strock (1933). Calciferol was as efficacious as dihydrotachysterol in bringing the serum-calcium and serum-phosphorus levels back to normal and was less expensive. The serumphosphorus level was very high again in February, 1955, although the serum-calcium level was normal; this indicates a renal tubular defect with increased resorption of phosphorus.
Summary A with
of
idiopathic hypoparathyroidism presenting epilepsy from the age of 8 years and recently developing megaloblastic anaemia is described. The probable cause of the anaemia is discussed. case
I wish to thank Dr. H. Hilton Stewart for permission to case, which was under his care, and for his advice.
report this
REFERENCES
Albright, F., Burnett, C. H., Smith, P. H., Parson, W. (1942) Endocrinology, 30, 922. — Strock, M. S. (1933) J. clin. Invest. 12, 974. Hawkins, C. F., Meynell. M. J. (1954) Lancet, ii, 737. MacGregor, M. E., Whitehead, T. P. (1954) Arch. Dis. Childh. 29, 398. Murphy, W. P. (1939) Anemia in Practice. Philadelphia and London: p. 195.
Robinson, P. K., Carmichael, E. A., Cumings, J. N. (1954) Quart. J. Med. 23, 383.
New Inventions PULMONARY-HILUM CLAMP THE clamp illustrated here was devised about two years ago for use in the thoracic unit in Manchester and has been used by several surgeons in many pulmonary
resections. There are occasions
when, owing to inflammation or to neoplastic enlargement of glands, the distal ends of the pulmonary vessels are difficult to secure satisfactorily
either by ligature or by the commoner clamps-e.g., straight Spencer Wells, Roberts forceps, and cholecystectomy forceps. Sometimes a Roberts or a cholecystectomy forceps could be applied backwards to the distal ends of the vessels, or alternatively the vessels could be cut distally and allowed to "bleed back."
In such
a
situation
a
fairly strong clamp with an angled shank and a reversed curve on its blades, which are longitudinally serrated but no wider than a Roberts forceps, gives most satisfactory control without fear of slipping. This instrument has a strong box-joint so that it can also be used, if necessary, as a distal clamp on the bronchus without straining it. I am indebted to Mr. H. Woodall, of Allen & Hanburys, for having the clamp made to my specifications. All inquiries should be addressed
to him.
P. JEWSBURY Blackpool Victoria Hospital
F.R.C.S.