Section ofPa?diatrics
19
.~~
..5
..... .._ .. up r .t ,,
..
hernt
345
negative pressure was applied. On the right side the drain bubbled for three days after insertion, but on the left no air came out after the initial aspiration. Aged 6 months her respiratory rate is slightly raised at 50/min and her X-rays show some increase in lung markings, but she is thriving, feeds well, and has survived several upper respiratory tract infections without developing bronchiolitis. She is neurologically normal. Previous reports of infants with similar cystic changes in their lungs have suggested that they shouldbe removed at open thoracotomy (Fletcher et at. 1974, Magilner et al. 1974). However, the ease of cyst aspiration in this case suggests that this should be the technique of choice in infants with severe generalized cystic ventilator-induced lung disease following IRDS. REFERENCES Avery M E & Fletcher B D (1974) The Lung and its Disorders in the Newborn Infant. W B Saunders, London, &c.; p 144 Fletcher B D, Outerbridge E W, Youssef S & Bolande R P (1974) Pediatrics 54, 808-811 Magilner A D, Capitanio M A, Wertheimer I & Burko H (1974) Radiology 111, 379-384 Roberton N R C (1976) In: Recent Advances in Respiratory Medicine. Ed. T Stretton. Churchill, London
Sarcoidosis in a Young Child W J Appleyard BM MRCP (Kent and Canterbury Hospital, Canterbury, CTJ 3NG) insertion of chest drain
Discussion There was no evidence of congenital structural abnormality of her lungs, or of infection with staphylococci or E. coi which can cause cystic changes in neonatal lungs (Avery & Fletcher 1974). The cysts in this infant were therefore attributed to an extension of the severe interstitial emphysema which developed during the first few days of IPPV, and represent an extremely severe form of the neonatal lung disease known as bronchopulmonary dysplasia. Although oxygen has been implicated in the etiology of this condition it is only found in those survivors of IRDS who have received IPPV at pressures in excess of 30 cm of water (Roberton 1976). The technique of 'pulmocentesis' proved surprisingly simple. The child was X-rayed with ski'n markers to give the surface markings of the cysts, and an Argyle FG 12 thoracocentesis drain was pushed into them. The drain was then connected to an underwater seal and three to five cm of
E M, girl, aged 2i History: Born 9.2.72. At age 11 months she developed a generalized* skin rash, consisting of tiny infiltrated follicular papules. The rash persisted apart from a few weeks following an attack of measles. A skin biopsy revealed noncaseating granulomata in the dermis. Mantoux 1:1000 negative. Subsequently, puffiness and swelling of her wrists and ankles occurred, and in December 1973 symptoms of polyuria and polydipsia, precipitated her admission to hospital where hypercalcaemia was confirmed. A strict low calcium diet caused a fall in the calcium level to 2.15 mmol/l and on a modified regime with Locasol milk and no added vitamin D, the polyuria settled as the calcium values returned to
normal. With increasing constitutional upset, the appearance of a painful right eye and hepatomegaly, a liver biopsy was performed (Dr Mark Rake). This confirmed the picture of multiple granulomata composed of epithelial cells and poorly formed giant cells (Fig 1).
346
Proc. roy. Soc. Med. Volume 69 May 1976
20 Table I Clinical features of sarcoidosis in young children
Skin rash Arthritis . Uveitis Salivary gland Constitutional symptoms Hypercaic;mia (Edema Hilar lymphadenopathy
Fig 1 Liver biopsy showing a non-caseous granuloma
Prednisolone 2 mg/kg was started and within a week the skin changes had improved. The ankle joint swelling settled but the cedema of the ankles required control with diuretics for several weeks. At present on an alternate day dose of 10 mg prednisolone, the skin rash has faded but there remain some old signs of uveitis and a little nonpainful puffiness of the wrists. Growth has been along normal centiles. Investigartions: Hb 11.2 g/100 ml. ESR 12 mm in first hour (Westergren). Bone marrow normal. Calcium 2.95 mmol/l. Phosphate 1.75 mmol/l. Alkaline phosphatase 5.3 K-A units. Total protein 70 g/l with slight increase in gamma globulin. IgG 17.6, IgA 0.69, IgM 1.9 g/l. RA test negative. ASOT under 200 iu. Serum parathormone 180 ng/l b.PTH (normal for age). X-rays of chest, skull and wrists normal. Discussion The clinical features of sarcoidosis in the infant and young child are quite distinct from those in the older child and adult where pulmonary manifestations and lymphadenopathy are characteristic (Gluck et at. 1972). The triad of a follicular skin rash, arthropathy, and uveitis occurs in ndarly all the young children so far described (North et at. 1970, Schweizer & Kanaar 1967, Kendig 1974, Holden & Heller 1975) (Table 1). The diagnosis should be confirmed by biopsy of the skin, synovia or liver. The hypercalc2emia in our patient did respond to a low calcium diet and was controlled by alternate day 10mg of prednisolone. It seems likely that the incidence of hyper-
14 reported cases 12 14 12 5
3 2 0 0
Present case + + + + + + -
calcwmia in the young child will after all prove to be as high as the 13 % in adults with sarcoidosis (Siltzbach et al. 1974). Steroids are the therapy of choice and prednisolone 2 mg/kg resulted in a dramatic response. The only guide to therapy has been the clinical progress. The ESR has remained essentially normal throughout. The most serious complication is blindness from uveitis (North et al. 1970). The patient has been on steroids for 2i years. An attempt to reduce the dose below 0.5 mg/kg resulted in a recurrence of her skin rash and it seems likely that treatment will be needed for several years (Gluck et al. 1972). REFERENCES Gluck J, Miller J J & Summerlin W T (1972) Journal ofPediatrics 81, 354 Holden K R & Heller R M (1975) American Journal of Diseases ofChildren 129, 103 Kendig E L (1974) Pediatrics 54, 289 North A F et al. (1970) American Journal of Medicine 48, 449 Schweizer A T & Kanaar P (1967) Archives of Disease in Childhood 42, 671 Siltzbach L E et al. (1974) American Journal ofMedicine 57, 847
The following cases were also presented:
Hypobetalipoproteintemia Associated with Cirrhosis Dr P J Milla (for Dr J T Harries) (Hospitalfor Sick Children, Great Ormond Street, London WCJ) Growth Failure and Features of Watery Diarrhea Hypokalemia Achlorhydria
(WDHA) Syndrome Dr P J Aggett (for Dr J T Harries) (Hospitalfor Sick Children, Great Ormond Street, London WCJ) Peritoneal Dialysis in Maple Syrup Urine Disease Dr A M Butterfill (for Professor 0 H Wolff) (Hospitalfor Sick Children, Great Ormond Street, London WCJ) Toxocariasis in Triplets Dr J Stanley-Jones (for Dr C G H Newman) (Queen Mary's Hospital. London SWJ5) CongenitAl Suprabulbar Paresis Dr M Connolly (for Dr J R Harper) (Northampton General Hospital, Northampton)
19
.~~
..5
..... .._ .. up r .t ,,
..
hernt
345
negative pressure was applied. On the right side the drain bubbled for three days after insertion, but on the left no air came out after the initial aspiration. Aged 6 months her respiratory rate is slightly raised at 50/min and her X-rays show some increase in lung markings, but she is thriving, feeds well, and has survived several upper respiratory tract infections without developing bronchiolitis. She is neurologically normal. Previous reports of infants with similar cystic changes in their lungs have suggested that they shouldbe removed at open thoracotomy (Fletcher et at. 1974, Magilner et al. 1974). However, the ease of cyst aspiration in this case suggests that this should be the technique of choice in infants with severe generalized cystic ventilator-induced lung disease following IRDS. REFERENCES Avery M E & Fletcher B D (1974) The Lung and its Disorders in the Newborn Infant. W B Saunders, London, &c.; p 144 Fletcher B D, Outerbridge E W, Youssef S & Bolande R P (1974) Pediatrics 54, 808-811 Magilner A D, Capitanio M A, Wertheimer I & Burko H (1974) Radiology 111, 379-384 Roberton N R C (1976) In: Recent Advances in Respiratory Medicine. Ed. T Stretton. Churchill, London
Sarcoidosis in a Young Child W J Appleyard BM MRCP (Kent and Canterbury Hospital, Canterbury, CTJ 3NG) insertion of chest drain
Discussion There was no evidence of congenital structural abnormality of her lungs, or of infection with staphylococci or E. coi which can cause cystic changes in neonatal lungs (Avery & Fletcher 1974). The cysts in this infant were therefore attributed to an extension of the severe interstitial emphysema which developed during the first few days of IPPV, and represent an extremely severe form of the neonatal lung disease known as bronchopulmonary dysplasia. Although oxygen has been implicated in the etiology of this condition it is only found in those survivors of IRDS who have received IPPV at pressures in excess of 30 cm of water (Roberton 1976). The technique of 'pulmocentesis' proved surprisingly simple. The child was X-rayed with ski'n markers to give the surface markings of the cysts, and an Argyle FG 12 thoracocentesis drain was pushed into them. The drain was then connected to an underwater seal and three to five cm of
E M, girl, aged 2i History: Born 9.2.72. At age 11 months she developed a generalized* skin rash, consisting of tiny infiltrated follicular papules. The rash persisted apart from a few weeks following an attack of measles. A skin biopsy revealed noncaseating granulomata in the dermis. Mantoux 1:1000 negative. Subsequently, puffiness and swelling of her wrists and ankles occurred, and in December 1973 symptoms of polyuria and polydipsia, precipitated her admission to hospital where hypercalcaemia was confirmed. A strict low calcium diet caused a fall in the calcium level to 2.15 mmol/l and on a modified regime with Locasol milk and no added vitamin D, the polyuria settled as the calcium values returned to
normal. With increasing constitutional upset, the appearance of a painful right eye and hepatomegaly, a liver biopsy was performed (Dr Mark Rake). This confirmed the picture of multiple granulomata composed of epithelial cells and poorly formed giant cells (Fig 1).
346
Proc. roy. Soc. Med. Volume 69 May 1976
20 Table I Clinical features of sarcoidosis in young children
Skin rash Arthritis . Uveitis Salivary gland Constitutional symptoms Hypercaic;mia (Edema Hilar lymphadenopathy
Fig 1 Liver biopsy showing a non-caseous granuloma
Prednisolone 2 mg/kg was started and within a week the skin changes had improved. The ankle joint swelling settled but the cedema of the ankles required control with diuretics for several weeks. At present on an alternate day dose of 10 mg prednisolone, the skin rash has faded but there remain some old signs of uveitis and a little nonpainful puffiness of the wrists. Growth has been along normal centiles. Investigartions: Hb 11.2 g/100 ml. ESR 12 mm in first hour (Westergren). Bone marrow normal. Calcium 2.95 mmol/l. Phosphate 1.75 mmol/l. Alkaline phosphatase 5.3 K-A units. Total protein 70 g/l with slight increase in gamma globulin. IgG 17.6, IgA 0.69, IgM 1.9 g/l. RA test negative. ASOT under 200 iu. Serum parathormone 180 ng/l b.PTH (normal for age). X-rays of chest, skull and wrists normal. Discussion The clinical features of sarcoidosis in the infant and young child are quite distinct from those in the older child and adult where pulmonary manifestations and lymphadenopathy are characteristic (Gluck et at. 1972). The triad of a follicular skin rash, arthropathy, and uveitis occurs in ndarly all the young children so far described (North et at. 1970, Schweizer & Kanaar 1967, Kendig 1974, Holden & Heller 1975) (Table 1). The diagnosis should be confirmed by biopsy of the skin, synovia or liver. The hypercalc2emia in our patient did respond to a low calcium diet and was controlled by alternate day 10mg of prednisolone. It seems likely that the incidence of hyper-
14 reported cases 12 14 12 5
3 2 0 0
Present case + + + + + + -
calcwmia in the young child will after all prove to be as high as the 13 % in adults with sarcoidosis (Siltzbach et al. 1974). Steroids are the therapy of choice and prednisolone 2 mg/kg resulted in a dramatic response. The only guide to therapy has been the clinical progress. The ESR has remained essentially normal throughout. The most serious complication is blindness from uveitis (North et al. 1970). The patient has been on steroids for 2i years. An attempt to reduce the dose below 0.5 mg/kg resulted in a recurrence of her skin rash and it seems likely that treatment will be needed for several years (Gluck et al. 1972). REFERENCES Gluck J, Miller J J & Summerlin W T (1972) Journal ofPediatrics 81, 354 Holden K R & Heller R M (1975) American Journal of Diseases ofChildren 129, 103 Kendig E L (1974) Pediatrics 54, 289 North A F et al. (1970) American Journal of Medicine 48, 449 Schweizer A T & Kanaar P (1967) Archives of Disease in Childhood 42, 671 Siltzbach L E et al. (1974) American Journal ofMedicine 57, 847
The following cases were also presented:
Hypobetalipoproteintemia Associated with Cirrhosis Dr P J Milla (for Dr J T Harries) (Hospitalfor Sick Children, Great Ormond Street, London WCJ) Growth Failure and Features of Watery Diarrhea Hypokalemia Achlorhydria
(WDHA) Syndrome Dr P J Aggett (for Dr J T Harries) (Hospitalfor Sick Children, Great Ormond Street, London WCJ) Peritoneal Dialysis in Maple Syrup Urine Disease Dr A M Butterfill (for Professor 0 H Wolff) (Hospitalfor Sick Children, Great Ormond Street, London WCJ) Toxocariasis in Triplets Dr J Stanley-Jones (for Dr C G H Newman) (Queen Mary's Hospital. London SWJ5) CongenitAl Suprabulbar Paresis Dr M Connolly (for Dr J R Harper) (Northampton General Hospital, Northampton)
