In 1932 Burger and Grutz described a previously unrecognized disorder characterized by triglyceride hyperlipaemia with variable clinical features, includ- ing attacks of abdominal pain, xanthomatous skin lesions, and hepatosplenomegaly.This condition is now usually called 'idiopathic hyperlipaemia' (Hsia, 1959).More recently, Gasser, Gautier, Steck, Sieben- mann, and Oechslin (1955) have defined the haemo- lytic-uraemic syndrome in young children.The features of this condition are renal damage with uraemia, a haemolytic anaemia with fragmentation of red blood cells, and thrombocytopenia.In this report we record the association of these two relatively rare conditions in monozygotic twins. Case ReportsTwin girls, the first children of young unrelated parents, were born in hospital on March 3, 1961.The mother was told the twins were identical; each weighed 4 lb.(1 -8 kg.).The neonatal period was uneventful and they were discharged from the maternity hospital when 51 lb.(2-5 kg.).Twin I. On August 30, 1961, she was admitted to the Royal Belfast Hospital for Sick Children.She had been ill for 12 hours with a cough, vomiting, and rapid breath- ing.On examination her temperature was 1020 F. (38. 9 C.) due to a chest infection; this was treated with a 5-day course of sulphadimidine.The haemoglobin on admis- sion was 8 g./100 ml.; 6 days later this had fallen to 6-2 g./ 100 ml.(Fig. 1).By September 7, 8 days after admission, she had be- come extremely pale and was passing blood-stained urine.There was no evidence of oedema.
FIG. 1.-Example of a partial thoracic stomach in a 12-month-old child.is referred to throughout as a 'partial thoracic stomach'.On the evidence available the use of a descriptive term has been preferred to one signifying a specific pathogenesis.
It is becoming recognised that weakness of the valve mechanism between oesophagus and stomach is a not infrequent cause of vomiting in infancy and early childhood (1, 2). This gastro-oesophageal incompetence allows abnormal reflux of stomach contents into the gullet, a process facilitated by the horizontal posture and fluid diet of infancy. In 1947, Neuhauser and Berenberg described in babies the condition of cardio-oesophageal relaxation or “chalasia,” in which the cardia is normally situated but wide and patulous, permitting easy reflux (3–5). More frequent than this condition, in our experience with children, is the so-called “short oesophagus,” with a small proportion of the stomach situated above the diaphragm. At The Children's Hospital in Birmingham (England), the annual incidence of hypertrophic pyloric stenosis is about 90 cases. In one year we see about 18 infants under twelve months of age, as well as a number in the older age groups, in whom vomiting is associated with a minor degree of partial thoracic stomach. Thus the condition is of considerable importance. The present paper is based on a study of 115 cases. Pathology The normal valve mechanism between the oesophagus and the stomach may be considered as a triple entity (6–10). One component is the “pinchcock” of the muscular right crus of the diaphragm, which largely constitutes the oesophageal hiatus. Possibly more important is the normal obliquity of insertion of the oesophagus into the stomach. This angle is maintained by the oblique fibres of the stomach wall, looped around the cardiac incisura, and by the sling-like action of the right crus, an action which Allison (10) compares to that of the pubo-rectalis muscle in controlling defaecation. The third and perhaps weakest element is the sphincteric action inherent in the lower end of the oesophagus itself; although there is no anatomical sphincter to be detected here, there is evidence that a functional sphincter exists. In the condition of “short oesophagus” and partial thoracic stomach, the oesophagus has a normal direct course but terminates above the diaphragm by entering the apex of a small loculus of the thoracic stomach. The proportion of the stomach that lies above the diaphragm is variable; in all of the present series of cases it has been estimated at under 15 per cent and often very much less. (More gross herniations are relatively uncommon; they are less apt to produce symptoms due to gastro-oesophageal incompetence and may conceivably have a different aetiology.) The diaphragmatic hiatus is wider than usual and, as a result, the “pinch-cock” action of the right crus is less effective than in the normal individual. Moreover, the direct entry of the oesophagus into the apex of the thoracic stomach means that the second valvular element, the obliquity of insertion, is lost. Only the weak sphincteric action of the oesophagus remains (Fig. 1).
Gastric emptying was measured using a modification of the double‐sampling dye dilution technique in 16 children with gastroesophageal reflux and partial thoracic stomach (hiatal hernia), 13 with reflux per se, and 12 controls with nonspecific vomiting. No differences could be demonstrated between the rate of emptying in these groups. Our study failed to provide a rational explanation for the copious projectile vomiting that is a frequent manifestation of these disorders.
The improved prognosis of acute renal failure has largely resulted from the use of conservative dietary regimes of treatment as suggested by Borst (1948), Pratt (1948) and Bull, Joekes and Lowe (1949, 1950).Few publications refer to children with acute tubular necrosis treated in this manner.In 1948 Pratt reported seven children with acute anuria of over '4 hours' duration treated conservatively with a limited fluid and liberal glucose diet: five recovered.Children with acute tubular necrosis successfully treated on similar lines have also been reported by Riddell (1951, two patients), Kaplan and Fomon (1953, one patient) and Wagner (1954, one patient).Other recent reports of acute tubular necrosis in children have been concerned primarily with the biochemical and pathological aspects (Zuelzer, Charles, Kurnetz, Newton and Fallon, 1951 Schwartz, Tomsovic and Schwartz, 1951).The purpose of this paper is to direct attention to the condition of acute tubular necrosis in infancy and early childhood, to describe problems en- countered in the management of three patients under 6 years of age and to discuss the biochemical disturbances observed.Possible aetiological factors are considered.The results include a review of each of these children two years or more after their initial illness.Case Reports Case 1. P.B., a girl aged 5 years 2 months was admitted at 3.50 a.m. on December 25, 1952, with a 24-hour history of dyspnoea and epigastric pain.She appeared an ill child with a temperature of 1028-8: signs of pneumonic consolidation were present over the left chest.Three-hourly intramuscular injections of crystalline penicillin were started immediately.Twelve hours later her condition had deteriorated.As additional therapy she was given 100 mg.aureomycin intravenously at 6.30 and again the following morning.The urine output was not recorded on the day of admission but she passed urine twice, the last occasion at 4.30 p.m.She vomited five timnes during the day.The blood pressure was 105 75 mm.Hg.The following day intravenous fluid therapy was begun because of vomiting and dehydration.Varying proportions of the following three solutions were given: normal saline, 500 dextrose in 1/5 isotonic saline, and 500 dextrose solution.No urne was passed.Antibiotic therapy was confined to penicillin.Next day she appeared improved and the fever had subsided.Abdominal distension and absent bowel sounds were noted.The blood pressure was 115 80 mm.Hg.Vomiting persisted and intravenous fluids were continued.By evening the sacral region and ankles were slightly oedematous.No calculus or renal calcification was demonstrated radiologically.One millilitre of urine was obtained on catheterization: this contained numerous red and white blood cells but no casts or crystals.Culture was sterile.On December 28 pulmonary oedema had developed and both ankle and sacral oedema had increased.She remained anuric.Intravenous fluids were discontinued (a total of 2,800 ml.having been given in 48 hours, equivalent in salt content to 1 litre of isotonic saline).Bull's regime was sub- stituted.She remained anuric until January 2, 1953, i.e., eight days.During the period of anuria vomiting decreased, oedema subsided, no hypertension was recorded and abdominal distension with absent bowel sounds persisted: she was at times mentally confused.Spectroscopic examination of the serum was negative.Infection of the mouth with Candida albicans was noted on December 31.A purpuric eruption on the neck appeared on January 1: no abnormalities were found in the bleeding, clotting or prothrombin times and the platelet count was 160,003 per c.mm. Serum biochemical findings are shown in Fig. 1.An E.C.G. on January 1 (serum potassium concentration 8 -5 mEq.litre) showed sharply peaked T waves in lead II and chest leads.In an attempt to lower the serum potassium, 5 g. of cation-exchange resin, 'zeocarb 225' in the hydrogen form, was given through the ga tric tube.In addition, vomit, with a potassium content of 3-9 mEq./litre, was replaced via the gastric drip by an equal quantity of an artificial mixture containing equivalent salt concentrations except for potassium i.e., 30 ml.N/10 HCI, 30 ml.N/ 10 NaCI and 40 ml.water.At 9-0 a.m. the following morning (January 2) bowel sounds were audible on auscultation: the serum potas- sium had fallen by 2-2 mEq./litre.Twelve hours later 40 ml. of urine were passed.After four days the urine 512 by copyright.
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