The findings in 40 children (24 female, 16 male) with osteoarticular complications of bruceliosis are presented. Raw milk was the main source of infection. Most patients had acute onset of fever, arthralgia, and myalgia. Arthritis was the presenting symptom in all patients, of whom two also had osteomyelitis. Arthritis was monoarticular in 28 (70%) of 40 cases with predilection for large weight-bearing joints. Spine and smali joints were spared.Brucella melitensis was detected in 23/31 (74%) cases. Mild anaemia, leucopenia, increased liver enzymes, positive acute phase reactants, and low titres of autoantibodies were prominent non-specific laboratory findings. Bone scintigraphy was more helpful than conventional radiography in detecting hip and sacroiliac joint disease. Treatment with a combination of antibiotics for six weeks or more resulted in a cure rate of 92-5%. Early recognition of infection, prolonged treatment, and long term follow up should improve the outcome of patients.

BACKGROUND AND OBJECTIVES:Ambiguous genitalia is a complex, medical and social emergency. The aim of this study is to present our experience over two decades, focusing on the pattern and clinical presentation.DESIGN AND SETTING:A retrospective study conducted in the pediatric endocrine clinic at a university hospital Saudi Arabia during the period 1989-2008.PATIENTS AND METHODS:Medical records of children with ambiguous genitalia were reviewed and the genitalia described.RESULTS:Of the 81 children with ambiguous genitalia, 53 (65.4%) patients were genetically females (46XX), with congenital adrenal hyperplasia being the common cause in 51 (96.5%) patients. Hyperpigmentation, variable degrees of salt wasting and a family history of a similar problem helped in diagnosis. Male genetic sex (46XY) was present in only 28 (34.6%) patients with a diversity of causes; multiple congenital anomalies in 9 (32.1%), local anorectal anomalies in 2 (7.1%), congenital adrenal hyperplasia (3-β-hydroxysteroid dehydrogenase deficiency) in 2 (7.14%), 5-α-reductase deficiency in 4 (14.28%), partial androgen insensitivity in 3 (10.7%), complete androgen insensitivity in 4 (14.28%), and hypogonadotrophin deficiency in 4 (14.3%).Twenty-five (47.2%) of females were wrongly assigned as males, where only two (7.1%) males were wrongly assigned as females.CONCLUSION:Ambiguous genitalia, currently termed disorders of sex development (DSD), is not uncommon in our community. Increased awareness, a detailed history, and a careful physical examination, coupled with appropriate laboratory and radiological investigations aid in early diagnosis and avoid serious sequelae.

The hand surgeon is frequently involved in the medicolegal assessment of birth palsy cases. Although brachial plexus injury has been reported in newborns delivered by Caesarean section, it is difficult to determine if these cases are due to excessive force when delivering the infant from the uterus or whether the palsy is related to other factors. We have studied our series of 16 cases of obstetrical brachial plexus palsy with special attention to the newborn baby delivered by Caesarean section. We have reviewed the English literature over the last decade and found that birth palsy in newborns delivered by Caesarean section is extremely rare (1% of all birth palsy cases). The differentiation between brachial plexus injury caused by forcible delivery and congenital upper limb palsy from other causes is discussed.

Twenty-eight children with ambiguous genitalia were seen at King Khalid University Hospital over a 6-year period. The incidence of this disorder was 0.4/1000 live births. Of the total, 21 (75%) were Saudis and seven (25%) were non-Saudis. The consanguinity rate was 67.9%. Twenty-four (85.7%) were born in hospital and four (14.3%) at home. In only three (10.7%) was the news first broken to the parents by a senior doctor, in 13 (46.4%) by a junior doctor, and in 11 (39.3%) by a nurse. Ambiguous genitalia were observed in 22 (78.6%) at birth and in six (21.4%) were picked up later. Owing to a lack of immediate investigative facilities and for some socio-cultural reasons, 19 of the latter groups were assigned sex without prior investigations. There was an obvious preference to assign male sex. On investigation, 13 (46.4%) had XX chromosomes, 11 (39%) XY and one (3.6%) XO: in three (10.7%), chromosomal results were not available. There were 14 cases (50%) of congenital adrenal hyperplasia, two of 5-alpha reductase deficiency (7.1%), and five of testicular feminization syndrome (17.9%), in addition to others. After investigation, five (17.9%) of the children needed sex reassignment. This was accepted by two and rejected for socio-cultural reasons by three. The opinion of the religious leaders was obtained. Some recommendations on management of these cases are made, based on our local experience.

The haematological manifestations were reviewed in 94 patients (55 males and 39 females) with visceral leishmaniasis. Their ages ranged from 4 months to 12 years (mean per cent 1.8 years). All patients had splenomegaly and were anaemic, while (73 per cent) were neutropenic and (56 per cent) thrombocytopenic. Coagulation abnormalities were encountered in 10 (11 per cent) patients; in four patients this was associated with disseminated intravascular coagulopathy. Bone marrow was hypercellular in (90 per cent), normocellular in (5 per cent), and hypocelluar in (4 per cent). Also variable degrees of erythrophagocytosis and leukophagocytosis were noted with preponderance of histiocytes (46 per cent) and granulomatous formation (25 per cent). Low haemosiderin content in the bone marrow was noted, which together with the finding of high serum ferritin is consistent with anaemia of chronic inflammation. Hypersplenism, haemophagocytosis and granulomatous lesions of the bone marrow, chronic inflammation, and dietary factors appear to be the most important factors in the causation of the haematological changes in visceral leishmaniasis.