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ERYTHROCYTE ENZYME DEFICIENCY IN UNEXPLAINED KERNICTERUS
1093
So far we have used the without any toxic manifestation.
dangerous.
Department of Clinical Medicine, Medical School of Ribeirão Preto, São
Paulo,
drug
in 62
patients ’
CÁSSIO BOTTURA.
Brazil.
TREATMENT OF IDIOPATHIC HYPERCALCÆMIA OF INFANTS articles have now
appeared about the SiR,-Many treatment of idiopathic hypercalcsemia of infants. A low calcium and vitamin-D intake, a high phytate intake, and the administration of cortisone have been recommended. To my knowledge mention has not been made of the possible beneficial effect of total exclusion from ultraviolet rays. The antirachitic effect of sunlight is
thought to be due to the
activation of 7-dehydrocholesterol in skin. This activation may result in the formation of substances with vitamin-D activity
equivalent to 1000 or more units of vitamin D per day in infants who are exposed to ultraviolet radiation. Possibly this disease
-has not been reported in Negroes because of the lesser effect of ultraviolet rays on deeply pigmented skin. It seems probable that infants with idiopathic hypercalcaemia are sensitive to very small amounts of vitamin D and it would seem advisable that patients with this disease be excluded from sunlight during those seasons when rays between 280 and 320 [L are present. Possibly someone with knowledge of the metabolism of vitamin D might render judgment whether infants with idiopathic hypercalcaemia should be excluded from sunlight as part of their treatment. Department of Pediatrics, Jefferson Medical College, Ptdladelphia.
HANS G. KEITEL.
TREATMENT OF OBESITY BY THE HIGH-FAT DIET
SIR,-Bearing in mind case 5, and the large group of patients and friends she appears to represent, can Prof. Yudkin and Miss Carey (Oct. 29) offer a 1500-calorie diet that is permanently acceptable both socially and financially to an adult woman of any age ? An overweight woman is conspicuous in a crowd: she does not wish to draw further attention to herself by always differing from the group when she is not eating alone. We would be most grateful. DAPHNE SASIENI. ERYTHROCYTE ENZYME DEFICIENCY IN UNEXPLAINED KERNICTERUS
SIR,-I was very interested by the contributions from Smith and Vella1 and Doxiadis et al.2 They confirm some observations I had the opportunity to report 3-6 last year. I have
-
so far studied 19 jaundiced newborn children (11 females) with unstable G.s.H. and/or G.-6-P.D. defect in their erythrocytes. In 10 cases exchange transfusion was performed: 7 of the transfused children and none of the untreated survived without damage. Precipitating factors, such as dangerous drugs, were recognised in only a few cases. Severe anasmia was rarely present; on the other hand, jaundice was often associated with severe clotting disturbances, indicating perhaps hepatic impairment, which may be due to the enzyme abnormality itself. In fact, as I was able to demonstrate by liver biopsy, G.-6-P.D. activity is decreased in the liver of sensitive individuals as well as in their erythrocytes.8
males,
8
1. Smith, G., Vella, F. Lancet, 1960, i, 1133. 2. Doxiadis, S. A., Fessas, P., Valaes, T. ibid. July 2, 1960, p. 44. 3. Studi Sassaresi, 1959, 37, 19. 4. Acta pædiat. latina, 1959, 12, 904. 5. XVII Congrès des Pédiatres de Langue Française; Oct. 1959, vol. IV.
Montpelier.
6. Boll. Soc. Ital. Biolo.
Sper. 1960, 36, 106.
The
observations of Smith and Vella and Doxiadis et al. emphasise again the importance of this particular type of neonatal jaundice. They also confirm the high incidence of the syndrome in a few families bearing the defect; and this fact suggests that there is a genetic factor in addition to the G.-6-P.D. defect. Clinica Pediatrica, Università di Sassari,
FRANCO PANIZON.
Italy.
A SEVERE ANAPHYLACTOID REACTION TO HYDROCORTISONE
SiR,-Occasional allergic reactions following the local injection of hydrocortisone have been recorded, and in many cases the possibility that associated use of hyaluronidase or procaine was to blame has not been firmly excluded. The following case illustrates an allergic response of alarming severity to the injection of ’Hydrocortisyl’ (Roussel) 5 ml. in a patient who had never had either local or general steroid medication. No other solution was injected and it was possible to exclude any previous injections (within 2 months) and any tablets or medicines (except 2 tab. codein. co. B.P., to which the patient was accustomed, taken a week previously). Similarly no dietary explanation could be discovered. A woman of 50 had, on April 8, 1960, a cup arthroplasty for osteoarthritis of the right hip. She made a normal recovery, and was discharged to outpatients on June 17. On Sept. 15, during follow-up examination, she complained of pain in the right shoulder. A typical moderately severe supraspinatus tendinitis, with early pericapsulitis, was found, and at about 3.3Q P.M. hydrocortisyl 5 ml. was injected into the rotator cuff. She had no immediate ill effects, but at 7.30 P.M., after supper, she suddenly felt her " face funny-blotchy with lumps, and burning.". Red patches appeared on her arms, and she felt sick. She " passed out " and was carried to bed. An hour later, when her doctor arrived, she had regained consciousness, but she was vomiting profusely, had pain in the stomach and loins, and had urticaria, especially on the arms and face. Conglomerate petechim and bruises appeared over the arms and face. She was in a state of shock, with tachycardia, extrasystoles, and a weak pulse. There was much abdominal tenderness, especially in the liver area. She was given anti-histamines (’ Piriton ’), and she felt a bit better next day, but for 2 days she vomited repeatedly and had diarrhoea and rectal incontinence. The stools were fluid and black, and the vomit contained dark blood. She was readmitted to hospital on the 3rd day. Laboratory investigations showed: haemoglobin 92%, platelets 300,000 per c.mm., bleeding-time 2 min., clotting-time 6 min., clot retraction after 1 hour normal, prothrombin content 100% of normal, and prothrombin consumption normal. The vomit contained dark blood, and occult blood was present in the stools. The urine was normal. Vomiting and diarrhoea ceased on the day of admission, and the abdominal pain and tenderness cleared up within 48 hours. She returned home after 5 days. Although intradermal skin tests were unlikely to be helpful so soon after such a severe reaction, they were attempted on Sept. 7 using: (1) control solution; (2) a minute dose of penicillin (to exclude penicillin contamination of the syringe used for injection); and (3) hydrocortisyl 0-01 ml. An erytbematous plaque 0’75 cm. in diameter formed in 24 hours around the site of hydrocortisyl injection, and persisted for 2 weeks, while the control sites showed no reaction. Thus it appears almost certain that this alarming reaction was due to the hydrocortisyl injection. Some 1.
Kendall, P. H.
Ann.
phys. Med. 1958, 4,
170.
So far we have used the without any toxic manifestation.
dangerous.
Department of Clinical Medicine, Medical School of Ribeirão Preto, São
Paulo,
drug
in 62
patients ’
CÁSSIO BOTTURA.
Brazil.
TREATMENT OF IDIOPATHIC HYPERCALCÆMIA OF INFANTS articles have now
appeared about the SiR,-Many treatment of idiopathic hypercalcsemia of infants. A low calcium and vitamin-D intake, a high phytate intake, and the administration of cortisone have been recommended. To my knowledge mention has not been made of the possible beneficial effect of total exclusion from ultraviolet rays. The antirachitic effect of sunlight is
thought to be due to the
activation of 7-dehydrocholesterol in skin. This activation may result in the formation of substances with vitamin-D activity
equivalent to 1000 or more units of vitamin D per day in infants who are exposed to ultraviolet radiation. Possibly this disease
-has not been reported in Negroes because of the lesser effect of ultraviolet rays on deeply pigmented skin. It seems probable that infants with idiopathic hypercalcaemia are sensitive to very small amounts of vitamin D and it would seem advisable that patients with this disease be excluded from sunlight during those seasons when rays between 280 and 320 [L are present. Possibly someone with knowledge of the metabolism of vitamin D might render judgment whether infants with idiopathic hypercalcaemia should be excluded from sunlight as part of their treatment. Department of Pediatrics, Jefferson Medical College, Ptdladelphia.
HANS G. KEITEL.
TREATMENT OF OBESITY BY THE HIGH-FAT DIET
SIR,-Bearing in mind case 5, and the large group of patients and friends she appears to represent, can Prof. Yudkin and Miss Carey (Oct. 29) offer a 1500-calorie diet that is permanently acceptable both socially and financially to an adult woman of any age ? An overweight woman is conspicuous in a crowd: she does not wish to draw further attention to herself by always differing from the group when she is not eating alone. We would be most grateful. DAPHNE SASIENI. ERYTHROCYTE ENZYME DEFICIENCY IN UNEXPLAINED KERNICTERUS
SIR,-I was very interested by the contributions from Smith and Vella1 and Doxiadis et al.2 They confirm some observations I had the opportunity to report 3-6 last year. I have
-
so far studied 19 jaundiced newborn children (11 females) with unstable G.s.H. and/or G.-6-P.D. defect in their erythrocytes. In 10 cases exchange transfusion was performed: 7 of the transfused children and none of the untreated survived without damage. Precipitating factors, such as dangerous drugs, were recognised in only a few cases. Severe anasmia was rarely present; on the other hand, jaundice was often associated with severe clotting disturbances, indicating perhaps hepatic impairment, which may be due to the enzyme abnormality itself. In fact, as I was able to demonstrate by liver biopsy, G.-6-P.D. activity is decreased in the liver of sensitive individuals as well as in their erythrocytes.8
males,
8
1. Smith, G., Vella, F. Lancet, 1960, i, 1133. 2. Doxiadis, S. A., Fessas, P., Valaes, T. ibid. July 2, 1960, p. 44. 3. Studi Sassaresi, 1959, 37, 19. 4. Acta pædiat. latina, 1959, 12, 904. 5. XVII Congrès des Pédiatres de Langue Française; Oct. 1959, vol. IV.
Montpelier.
6. Boll. Soc. Ital. Biolo.
Sper. 1960, 36, 106.
The
observations of Smith and Vella and Doxiadis et al. emphasise again the importance of this particular type of neonatal jaundice. They also confirm the high incidence of the syndrome in a few families bearing the defect; and this fact suggests that there is a genetic factor in addition to the G.-6-P.D. defect. Clinica Pediatrica, Università di Sassari,
FRANCO PANIZON.
Italy.
A SEVERE ANAPHYLACTOID REACTION TO HYDROCORTISONE
SiR,-Occasional allergic reactions following the local injection of hydrocortisone have been recorded, and in many cases the possibility that associated use of hyaluronidase or procaine was to blame has not been firmly excluded. The following case illustrates an allergic response of alarming severity to the injection of ’Hydrocortisyl’ (Roussel) 5 ml. in a patient who had never had either local or general steroid medication. No other solution was injected and it was possible to exclude any previous injections (within 2 months) and any tablets or medicines (except 2 tab. codein. co. B.P., to which the patient was accustomed, taken a week previously). Similarly no dietary explanation could be discovered. A woman of 50 had, on April 8, 1960, a cup arthroplasty for osteoarthritis of the right hip. She made a normal recovery, and was discharged to outpatients on June 17. On Sept. 15, during follow-up examination, she complained of pain in the right shoulder. A typical moderately severe supraspinatus tendinitis, with early pericapsulitis, was found, and at about 3.3Q P.M. hydrocortisyl 5 ml. was injected into the rotator cuff. She had no immediate ill effects, but at 7.30 P.M., after supper, she suddenly felt her " face funny-blotchy with lumps, and burning.". Red patches appeared on her arms, and she felt sick. She " passed out " and was carried to bed. An hour later, when her doctor arrived, she had regained consciousness, but she was vomiting profusely, had pain in the stomach and loins, and had urticaria, especially on the arms and face. Conglomerate petechim and bruises appeared over the arms and face. She was in a state of shock, with tachycardia, extrasystoles, and a weak pulse. There was much abdominal tenderness, especially in the liver area. She was given anti-histamines (’ Piriton ’), and she felt a bit better next day, but for 2 days she vomited repeatedly and had diarrhoea and rectal incontinence. The stools were fluid and black, and the vomit contained dark blood. She was readmitted to hospital on the 3rd day. Laboratory investigations showed: haemoglobin 92%, platelets 300,000 per c.mm., bleeding-time 2 min., clotting-time 6 min., clot retraction after 1 hour normal, prothrombin content 100% of normal, and prothrombin consumption normal. The vomit contained dark blood, and occult blood was present in the stools. The urine was normal. Vomiting and diarrhoea ceased on the day of admission, and the abdominal pain and tenderness cleared up within 48 hours. She returned home after 5 days. Although intradermal skin tests were unlikely to be helpful so soon after such a severe reaction, they were attempted on Sept. 7 using: (1) control solution; (2) a minute dose of penicillin (to exclude penicillin contamination of the syringe used for injection); and (3) hydrocortisyl 0-01 ml. An erytbematous plaque 0’75 cm. in diameter formed in 24 hours around the site of hydrocortisyl injection, and persisted for 2 weeks, while the control sites showed no reaction. Thus it appears almost certain that this alarming reaction was due to the hydrocortisyl injection. Some 1.
Kendall, P. H.
Ann.
phys. Med. 1958, 4,
170.