Gastrointestinal complications of gastrocystoplasty.

The cases are reported of five children with chronic renal failure who underwent gastrocystoplasty for a variety of urological disorders. Gastrocystoplasty comprises the transplantation of a vascularised segment of stomach to the bladder to form an augmented neobladder. The patients had gastrointestinal complications after the operation, including considerable weight loss in all five patients, accompanied by marked failure to thrive in four of the five patients, and food aversion, feeding intolerance, dumping syndrome, delayed gastric emptying, and oesophagitis in two patients. Three of the five patients developed severe abdominal pain and haemorrhagic cystitis secondary to gastric acid secretion in the neobladder from the transplanted gastric pedicle. Nutritional and pharmacological interventions were used to manage the gastrointestinal problems. Explanations are offered for the pathophysiology of the observed complications of gastrocystoplasty. It is believed that the use of this procedure in infants and children, particularly those with chronic renal failure and uraemia, warrants caution until successful long term follow up and experience with this procedure have been reported.

Nutritional manipulation in the management of dumping syndrome.

Two children with Nissen's fundoplication and either gastrocystoplasty or pyloroplasty developed dumping syndrome. Correction of their blood glucose abnormalities, resolution of symptoms, and weight gain were effectively achieved by addition of fats and uncooked corn starch (50 g/l) to their feeds.

Glycogen storage disease, Fanconi nephropathy, abnormal galactose metabolism and mitochondrial myopathy.

We present a 4-year-old male suffering from profound muscular weakness, enzymatically undefined glycogen storage disease. Fanconi nephropathy and impaired galactose utilization. Distorted mitochondria, intramitochondrial fat droplets and partial deficiencies of pyruvate dehydrogenase complex, succinate: cytochrome c oxidoreductase, and cytochrome c oxidase have been found in muscle tissue. The causal relationship between mitochondrial myopathy, glycogen storage disease, Fanconi nephropathy and impaired utilization of galactose is discussed.

Myocardial damage after a scorpion sting: long-term echocardiographic follow-up.

A seven-year-old girl, stung by a scorpion, was hospitalized in a confused state with signs of myocarditis and pulmonary edema. In spite of clinical improvement within 24 h, 14 serial echocardiograms and electrocardiograms performed during a four-month period showed severe changes. There have been no previously published reports of echocardiographic studies showing myocardial changes after a scorpion sting.

Transient hyperphosphatasemia of infancy.

Very high serum levels of alkaline phosphatase were found in four children aged 13 to 24 months. No other abnormalities nor explanation for the raised enzyme levels were found. The origins of the elevated enzyme levels were shown by isoenzyme studies to be the liver in one case, the bone in two cases, and undetermined in one case. Serum alkaline phosphatase levels returned to normal after periods of 5 to 20 weeks. Awareness of these benign forms of hyperphosphatasemia will aid the physician in the differential diagnosis of elevated alkaline phosphatase levels.

Partial deletion of the short arm of chromosome 3: further delineation of the 3p25-3pter syndrome.

A male newborn with partial deletion of the short arm of chromosome 3 is described. The patient shares most of the features with the previously reported cases. In addition, cardiac, skeletal and gastrointestinal anomalies not previously reported are described. These characteristics may help in further delineation of the syndrome.

Giardia lamblia: identification of different strains from man.

Four axenically cultured human Giardia lamblia isolates from Jerusalem (KC-1, 2, 3 and 4) and one from Bethesda (WB) were compared. Three distinct groups were defined by agglutination response to rabbit anti-G. lamblia sera viz. WB; KC-3; and KC-1, 2 and 4. The same major groups were identified by isoenzyme analysis using thin-layer starch-gel electrophoresis, each group differing from the others in three or more of five enzymes studied. In addition, a single enzyme difference distinguished KC-2 from KC-1 and 4. These findings reveal significant heterogeneity in G. lamblia isolates both from widely separated areas and within a single region. Immunoassays for diagnosis of giardiasis should take into account the differences between strains. Heterogeneity among G. lamblia strains may explain the variable clinical manifestations, host response and treatment efficacy characteristic of human giardiasis.