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Eleven cases of true hermaphroditism, 6 raised as female and 5 as male, were seen in the 20-year period from 1965 to 1985. External genitalia varied from Prader types II to IV. The ovotestis (11/22) was the most common gonad, and a marked variation in macroscopic and microscopic appearances with five patterns of distribution of gonadal tissue was noted. Ovulation was observed in ovarian tissue, and spermatogonia, but no spermatozoa, in testicular tubules. A hypothesis is developed suggesting abnormal testicular differentiation, and a model is presented to explain observed patterns of gonadal tissue distribution. A uterus was present in all cases, but a hemiuterus or lateralized uterus was noted in the 5 cases in which only testicular or predominantly testicular tissue was observed on the contralateral side. Karyotype analysis showed 46 XX in 5 cases (1 with an H-Y antigen) and a mosaic involving 46 XY in the remaining 5. Spontaneous puberty was achieved in 3 of the females, and of the 4 males with residual testicular tissue, all are prepubertal.

The diagnostic value of 73 bronchoalveolar lavages was assessed in 67 immunocompromised children (aged 3 months to 16 years) with pulmonary infiltrates. Thirty one children had primary and 19 secondary immune deficiency, 14 acquired immunodeficiency syndrome (AIDS), and three AIDS related complex. Bronchoalveolar lavage was performed during fibreoptic bronchoscopy, under local anaesthesia in all but two. One or more infective agents was found in eight of 11 patients with severe acute pneumonia and in 26 of 62 patients with interstitial pneumonitis. In interstitial pneumonitis, the most frequently encountered agents were Pneumocystis carinii (12), cytomegalovirus (8), and Aspergillus fumigatus (3). The yield was related to the severity of interstitial pneumonitis. The mean cellular count and cytological profile in lavage returns from patients with varying infective agents or underlying pathological conditions showed no significant difference, except in those children with AIDS and AIDS related complex who had appreciable lymphocytosis (mean percentage of lymphocytes 28 (SD 17]. In children with AIDS and chronic interstitial pneumonitis lymphocytosis without pneumocystis infection was observed in eight of nine bronchoalveolar lavage returns and was suggestive of pulmonary lymphoid hyperplasia. Finally, bronchoalveolar lavage produced a specific diagnosis from the microbiological or cytological findings in 44 instances (60%). Transient exacerbation of tachypnoea was observed in the most severely ill children but there was no case of respiratory decompensation attributable to the bronchoscopy. Bronchoalveolar lavage is a safe and rapid examination for the investigation of pulmonary infiltrates in immunocompromised children. It should be performed as a first line investigation and should reduce the use of open lung biopsy techniques.

A case of obstructive jaundice caused by a solitary myofibroma in an 18-week-old infant is presented. Preoperative investigations demonstrated total biliary and partial pancreatic obstruction, although no obstructing lesion was identified radiologically. At operation a 3-cm solitary myofibroma was found in the head of the pancreas. Because of the morbidity associated with resection at this site, a bypass procedure was performed in the hope that the lesion will regress.

Most congenital lateral cervical cysts, fistulae, and skin tags are considered to be from the branchial apparatus. This is a 13-year review of 58 patients (with 66 branchial lesions) who were operated on. There were eight simple cysts and six cysts with a fistula; 43 external fistulae with or without an internal opening, and nine skin tags. Eighty-seven percent (39/45) of patients with skin tags and external fistulae were less than 5 years of age at the time of operation. On the other hand, all eight patients with cysts but no fistula were greater than 9 years of age. Eight lesions were considered to be the first branchial remnants, and 44 lesions were suspected to be from the second branchial cleft. One external fistula with an internal communication to the pharynx at the level of thyrohyoid membrane was considered to be a third (or fourth) branchial remnant. The other branchial cyst with thyroid tissue in its wall was suggested to be a fourth branchial remnant. Pathology of the excised lesions showed columnar, squamous, or a mixed epithelium. Lymphoid aggregates were documented in 31 patients. Duration of hospital stay was short, except for four patients with first cleft defects who stayed more than five days. Three of the four recurrent cases were first branchial remnants, including one case with the first operation performed at another hospital. In view of these findings, first branchial remnants must be excised with extra care.

Pharyngeal pouches present far more commonly on the left side of the neck than the right. Sixty one patients with a history of pharyngeal pouch were questioned about their handedness and about whether they had had symptoms or signs predominantly on one side of the neck before receiving treatment. There was a highly significant association between handedness and pharyngeal pouches on the opposite side of the neck. It is suggested that this is the reason for the rarity of right sided pouches.

Cysticercosis of the central nervous system is seen infrequently in Australia. Most cases occur in adults, but two imported cases of neurocysticercosis in children are described. A 10-year-old Anglo-Indian girl presented with aseptic meningitis, raised intracranial pressure and a block to the flow of cerebrospinal fluid in the lumbar region. Laminectomy showed two cysticerci in the spinal subdural space. A three-year-old boy from Zimbabwe suffered a focal seizure. A granuloma that contained a degenerate cysticercus was found in the left parietal cortex. Serological examination of blood for cysticercosis was performed only in the second case and gave negative results. Examination of stools in both children gave negative results for the presence of parasites, cysts and ova.

An epidemiological study of Q fever infection in a Brisbane meatworks was undertaken. A retrospective 10-year study (1968 to 1977) revealed an average annual incidence of 1% for the nine years excluding 1969, while an outbreak with a 7.9% incidence occurred in 1969. No seasonal distribution was found. Results indicated that cattle form the major source of Coxiella burneti (infectious agent of Q fever) in the meatworks and that the highest risk of infection exists on the cattle slaughter floor. The 1969 outbreak showed a departure from the average pattern of infection in that the highest incidences were recorded in small stock (sheep and swine) workers and non-meat handlers rather than in cattle slaughtermen. This suggested the importance of sheep and/or swine forming a source of the infection. Investigations into the factors contributing to the 1969 outbreak in this meatworks failed to reveal an; conclusive data. Inhalation of infectious dust and aerosols proved to be the major channel of transmission of infection rather than direct inoculation. A serological survey of 139 employees revealed 15.8% prevalence of complement-fixing antibodies to C. burneti, 0% to brucellosis, 0.7% to leptospirosis, and 0.7% to Toxoplasma gondii. Of those with positive titres for Q fever, nine of the 22 subjects did not recall any clinical illness.

Severe derangement of the protective function of the larynx results in intractable aspiration with secondary life-threatening pneumonia. The new surgical technique of epiglottopexy is described, which in addition to achieving protection of the laryngeal inlet, allows preservation of speech and in certain cases an adequate laryngeal airway. The advantages over current alternative treatment methods is discussed, and the results of five cases of gross laryngeal incompetence managed by epiglottopexy are presented.

To test the hypothesis of a defect in GH-receptor interaction, which could explain the growth failure of thalassemic children, the binding of [125I]human (h) GH to membrane fractions prepared from liver biopsies was studied. Small amounts of liver were obtained from 6 girls and 11 boys with homozygous beta-thalassemia, aged 3-15 yr, all prepubertal, at the time of splenectomy. Specific binding of [125I]hGH ranged from 0.37-5.11% of the added radioactivity/100 micrograms liver membrane protein, with variations in both receptor number and binding affinity. This 14-fold variation in hGH binding to liver membranes of thalassemic children was comparable to that in membrane fractions of livers obtained from normal donors at the time of liver transplant. The binding of insulin to liver membranes from the thalassemic patients ranged from 9.8-17.9% of the added radioactivity/100 micrograms membrane protein and from 2.8-15.0%/100 micrograms membrane protein in the normal donors. Insulin and GH binding to liver membranes did not vary in a consistent way. A 3-fold difference was found in 5'-nucleotidase activity of the membrane fractions. Histological hepatic modifications were assessed with respect to siderosis and fibrosis. No correlation was found between these parameters and GH binding. These results suggest that possible membrane alterations are not the only reason for the variations in hGH binding. All patients had retarded growth, and all but 2 had low plasma insulin-like growth factor I levels. No relationship was found between the level of GH binding to liver membranes and the growth failure. Thus, a defect in GH binding to liver membranes is probably not the cause of the growth retardation of thalassemic children.