Reversible binocular visual loss in temporal association with artesunate-amodiaquine treatment in a child on mefloquine chemoprophylaxis.

A case of an acute reversible visual loss in a 10-year-old child who was on mefloquine prophylaxis, and was treated with artesunate-amodiaquine for an acute febrile illness diagnosed clinically as uncomplicated malaria, is reported. On admission the patient could not perceive light and had bilateral papilloedema. She was treated with dexamethasone and recovered her sight gradually over a 21-day period. There has been no previous report to our knowledge, of an association between acute visual loss and mefloquine, amodiaquine, or artesunate in the published literature, even though mefloquine is associated with blurring of vision, and antimalarials of the quinoline class have been associated with retinopathy (during long term use). While causality is difficult to ascribe in this case, it may be prudent to avoid the use of quinoline-based antimalarials for treating acute malaria in travelers taking mefloquine prophylaxis, because information on the safety of concurrent use of artemisinin combination therapies and mefloquine, or other recommended prophylactic regimens, is limited.

Hydrocephalus, ventriculo-peritoneal shunt and cerebrospinal fluid ascites.

A small number of patients with congenital hydrocephalus, who have been treated effectively with ventriculoperitoneal shunts, develop progressive increase in abdominal girth, due to cerebrospinal fluid ascites. This abdominal distension can produce respiratory difficulties that require endotracheal intubation and ventilator support. The respiratory difficulties and the abdominal distension were eliminated when the ventriculoperitoneal shunt was converted to a ventriculoatrial shunt in each of the three cases presented in this paper for discussion.

Indian childhood-like cirrhosis in three Saudi Arabian siblings.

Three Saudi siblings, two girls and one boy, presented at the ages of 7, 6 and 2.5 years, respectively, and were diagnosed as having features of Indian childhood cirrhosis (ICC). The two girls presented at a late stage of the disease and the boy was diagnosed during routine examination of the family. The initial presenting complaint was abdominal distention and pruritus. All three had a rapid and fatal course. There was no evidence of increased copper ingestion by the families, supporting the suggestion of a hereditary metabolic role in the aetiology of ICC. As far as we are aware, this is the first report of ICC in Saudi Arabian children.

Ultrastructural changes in animal fat associated lipoid pneumonia: report of two cases.

Traditional or cultural practices in different parts of the world where oils are used continue to produce lipoid pneumonia. We report the ultrastructural findings and observations in animal fat associated lipoid pneumonia from two children following a cultural practice of forced feeding with animal fat (ghee). Clinical findings showed an acute or chronic chest infection which failed to respond to anti-microbial therapy. X-ray and chest CT scan revealed collapse/consolidations of the right middle and left lower lobes. Histology and electron microscopy revealed thickening and destruction of the alveolar septa, filling of alveolar spaces with red blood cells and macrophage which contained both primary and secondary lysosomes. The alveolar walls contained mostly type II pneumocyte with most of them surrounded by thickened basement membranes with only a small portion of their surfaces exposed directly to the alveolar space where it showed numerous microvilli.

Hypereosinophilia associated with intrahepatic cholestasis in early infancy.

A male infant presented with intrahepatic cholestasis due to idiopathic eosinophilia damaging the biliary epithelium. No other etiological agent or cause could be identified. He responded well to prednisone therapy.

Renal reabsorption of phosphate in children with sickle cell anemia.

It has previously been reported that in adult patients with sickle-cell anemia the serum phosphate value and the maximum tubular reabsorption of phosphate per liter of glomerular filtrate (TmP/GFR) were significantly higher than in normal controls. This does not appear to have been studied in children with sickle cell anemia (young sicklers) and this prompted us to assess renal phosphate reabsorption in this group of patients. We looked at serum phosphate level and calculated renal phosphate reabsorption (TP/GFR) in children taking random urine and blood samples at the same time and using the formula TP/GFR = Sp - Up x SCr: UCr, in 30 young sicklers all of whom had normal renal function (mean age 7.3 years) and 40 normal matching controls (mean age 6.5 years). The mean serum phosphate value in young sicklers was significantly lower than in controls (4.3 against 5.3 mg/dl) while the mean value of TP/GFR was 4.09 +/- 0.74 mg/dl in young sicklers compared to 4.65 +/- 0.75 mg/dl in the control group (p = 0.0026). Therefore, the TP/GFR in young sicklers was also significantly lower (p = 0.0026) than in the control group. This may be explained by the high serum level of parathyroid hormone reported previously in patients with sickle cell anemia which is expected to lower phosphate reabsorption (TmP/GFR and TP/GFR are identical in children). The lower serum phosphate value and TP/GFR in younger sicklers seems to be in contrast with the relatively high serum phosphate value and TP/GFR previously reported in adults with sickle cell anemia.

Olive oil aspiration pneumonia (lipoid) in children.

In the Asir region of south-western Saudi Arabia, nasal instillation of olive oil to infants and children in the recumbent position is practised to relieve nasal congestion. Aspiration of olive oil results in lipoid pneumonia resistant to antimicrobial treatment. A series of 5 children, aged 4-72 months, with olive oil-induced lipoid pneumonia is presented. Clinical presentation included persistent coughing, tachypnoea, recurrent febrile illness and chest infections. The pulmonary radiological picture was mainly right middle lobar and perihilar infiltrates. Bronchial lavage and microscopic examination of the aspirate confirmed the presence of fat globules. The pneumonia resolved on treatment with steroids and physiotherapy in the form of clapping and vibrations. For infants and children in this area who present with persistent pulmonary infiltrates which are not responsive to antimicrobials, the differential diagnosis of not only animal fat (ghee, clarified butter) but also of olive oil lipoid pneumonia must be considered.

Bronchiectasis due to lipid aspiration in childhood: clinical and pathological correlates.

We report the clinical and pathological features in six Arab children with bronchiectasis caused by ghee lipid aspiration. They all had a history of ghee administration followed by a history of chronic cough dating from early childhood. Chest radiographs showed consolidation/collapse of the right middle and left lower lobes in the majority, and bronchography and chest CT scan confirmed bronchiectasis. The children were treated medically, without any improvement, and five required surgery. The histology of the lung revealed dilated bronchi filled with vacuolated granular eosinophilic material and the peripheral lung tissue was mostly atelectatic with patchy lymphocytic bronchiolitis. Frozen sections of the lung tissue showed scattered lipogranulomas with fat-laden macrophages and fat droplets within peripheral and perivascular lymphatics. In communities where the traditional practice of force-feeding infants and children with ghee exists, it may be an important predisposing cause of bronchiectasis.

Reaction of human lungs to aspirated animal fat (ghee): a clinicopathological study.

We report the clinical findings and pathological lung changes in four children following a cultural practice of forced feeding with animal fat (ghee) during infancy. The clinical presentation was of acute or chronic chest infection which failed to respond to antimicrobial therapy. The radiographic features ranged from extensive bronchopneumonia to collapse/consolidation and bronchiectasis. The light microscopy findings included diffuse mononuclear interstitial pneumonia, intraalveolar desquamation of pneumocytes, lipid granuloma formation, lung atelectasis and bronchiectasis. In the two children with longstanding reactions, the striking feature was the minimal lipid engulfment by the macrophages, the continuation of the mononuclear interstitial pneumonia, bronchiectasis and minimal lung fibrosis. In these two older children, the lung lymphatics were probably the main channels for drainage of the aspirated ghee.

Malaria in children - experience from Asir region, Saudi Arabia.

During a five year period, 233 cases of malaria (2.4%) were diagnosed among 9259 children with fever and hepatosplenomegaly seen in Asir Central Hospital, Abha, Saudi Arabia. The majority of these were below four years of age and came from Tihama, a hot, humid valley area in the Asir region. The infection was seasonal and occurred between December and May. Apart from fever, vomiting and hepatosplenomegaly, anemia was a common clinical finding; this was partly due to iron deficiency anemia, probably nutritional. Most of the cases responded to chloroquine therapy; however, three required intravenous quinine and two received Fansidar to effect eradication of the parasitemia. During the study, two patients died, one from cerebral malaria and the other from severe hemolytic anemia and hemoglobinuria. For prevention of malaria in this endemic area, an integrated program is advocated that includes the use of bednets impregnated with permethin, adequate treatment of proven cases and intensive health education on malaria control and nutrition.

Clinical and laboratory features of childhood visceral leishmaniasis in southwestern Saudi Arabia.

The clinical and laboratory features of visceral leishmaniasis (VL) were studied in 51 children at Abha in southwestern Saudi Arabia, an area endemic for the disease. The majority of patients came from the coastal Tihama Valley or had visited the Valley from the hills. There was a predilection for infants and young children (median age 18 months). A double quotidian fever pattern was observed in half the patients. Splenomegaly was noted to be more common and more severe than hepatomegaly. Important hematological features were anemia, aften severe, neutropenia and thrombocytopenia. Common biochemical abnormalities inclueded hepatic dysfunction, hypoalbuminemia and hyperglobulinemia. The hematological and biochemical changes noted in VL are nonspecific. Our findings are compared with those from other endemic areas. Saudi Arabian VL resembles the Mediterranean form of the disease.

Diagnostic and management problems in childhood visceral leishmaniasis in south-western Saudi Arabia.

The diagnosis and management of childhood visceral leishmaniasis were studied in 51 parasitologically proven cases from Abha, Saudi Arabia. Bone marrow aspiration was positive in 40 of 47 cases (85%). Splenic aspiration, though rarely used because of perceived dangers, was not associated with complications and revealed the parasite in all 12 cases in which it was used. There was prompt response to sodium stibogluconate, with defervescence in 93% and decrease of hepatosplenomegaly in 67% of patients within 1 week of commencing chemotherapy. A dose of 20 mg/kg/day for at least 3 weeks was generally safe and effective in achieving cure and preventing relapse. Two children with persistent massive splenomegaly after the first course responded to prolonged chemotherapy. Bronchopneumonia and severe cytopenia were common complications. Disseminated intravascular coagulation and hepatitis were associated with a poor outcome. The four patients who died had a progressive course with multiple complications. Early detection and appropriate management of complications may help to reduce morbidity and mortality in childhood visceral leishmaniasis.

Chest radiographic findings in childhood lipoid pneumonia following aspiration of animal fat.

The pulmonary radiological manifestations of lipoid pneumonia following a cultural practice of forced feeding of rendered animal fat (ghee) in 24 Saudi children are described. The age range is 15 days to 11 years with 68% of cases being in infancy. The chest radiographic appearances encountered could be grouped into four broad patterns, viz, bilateral multilobar consolidations (BMLC) in 50% (12 cases), bilateral perihilar infiltrates (BPHI) with or without associated lobar consolidation in 21% (five cases), right perihilar infiltrates (RPHI) in 21% (five cases) and unilateral right multilobar consolidation (RMLC) 8% (two cases). The pneumoniae were non-resolving acute or chronic lung consolidations despite the usual antimicrobial chemotherapy. Associated nodular dense opacities (granulomas) were seen in the lower lobes of four of the 12 cases in the first group. Complications, seen also in this first group, included chronic collapse consolidation, fibrosis and death in three cases. In communities where the traditional practice of infant feeding with ghee exists, these pulmonary radiological patterns, although in some cases indistinguishable from those of bacterial pneumoniae, should raise the suspicion of ghee administration with consequent early diagnosis and energetic management to avert prolonged morbidity and potential death.

Complications of Echis colorata snake bites in the Asir region of Saudi Arabia.

Seven Saudi children bitten by Echis colorata, a species of carpet viper encountered mainly in the Middle East, were studied in south-western Saudi Arabia. They all showed severe local signs of swelling, ecchymosis and blisters, and one also had local necrosis. Four children had systemic envenoming with vomiting and hypotension, and one child had acute renal failure. Three of them had prolonged prothrombin and partial thromboplastin times and hypofibrinogenaemia resulting from disseminated intravascular coagulopathy. They were initially managed in the intensive care unit and received intravenous fluids and polyvalent antivenom. Apart from one child who required skin grafting for local necrosis, complete recovery with no sequelae occurred in all cases.

Bronchoscopy in lipoid pneumonia.

Forcible administration of rendered animal fat to infants is a tradition in south western Saudi Arabia. Accidental inhalation may result in a resistant form of lipoid pneumonia. A series of 24 cases of lipoid pneumonia, 22 of which were diagnosed by bronchoscopy with bronchial lavage and microscopic examination of the aspirate, are reported. The technique is described briefly and the results analysed. A high index of suspicion together with bronchoscopy and bronchial lavage of all cases of resistant or recurrent pneumonia is essential in areas such as ours for diagnosis of this condition.

Osteoarticular complications of childhood brucellosis: a study of 57 cases in Saudi Arabia.

Fifty-seven (36%) of a cohort of 157 children with brucellosis from Saudi Arabia had arthritis. Most gave a history of contact with farm animals or drinking unpasteurized milk. Associated features included pyrexia, arthralgia, hepatosplenomegaly, and lymphadenopathy. A subacute presentation with peripheral oligoarthritis predominantly affecting hips or knees was common. Specific chemotherapy resulted in rapid defervescence followed by slower resolution of the arthritis. Children with osteoarticular brucellosis had a higher relapse rate and a longer hospital stay. Supervised combination chemotherapy for at least 6 weeks was effective in preventing relapse. A brucellar etiology should be considered in any child from an endemic area who has osteoarticular manifestations.

Childhood brucellosis in southwestern Saudi Arabia: a 5-year experience.

One-hundred-and-fifty-seven children admitted with brucellosis at Abha, Saudi Arabia, were studied prospectively. Ninety-two per cent gave a history of animal contact, usually with sheep or goats, or ingesting raw milk, milk products, or raw liver. Three-quarters of the patients had an acute or subacute presentation with diverse symptomatology: fever (100 per cent), malaise (91 per cent), anorexia (68 per cent), cough (20 per cent), abdominal symptoms (20 per cent), arthralgia (25 per cent). Hepatomegaly (31 per cent), splenomegaly (55 per cent), and lymphadenopathy (18 per cent) were common findings. Organ complications were rare except for arthritis (36 per cent) which usually presented as a peripheral oligoarthritis involving the hips and knees. All patients had significant agglutination titres; B. melitensis was grown from the blood in 7 of 16 (44 per cent) patients. Haematological variations were common, but non-specific: anaemia (64 per cent), thrombocytopenia (28 per cent), leucopenia (38 per cent), leucocytosis (12 per cent), and elevated erythrocyte sedimentation rate (81 per cent). Varying combinations of rifampicin, co-trimoxazole, tetracycline, and streptomycin resulted in a prompt pyrexial response (mean: 3.8 days), and a slower response in the arthropathy and hepatosplenomegaly. Relapses were related to poor compliance, use of a single drug or a shorter duration of chemotherapy. Brucellosis is a common childhood problem in southwestern Saudi Arabia as in other parts of the country and the Middle East. It should be considered in every child from an endemic area presenting with a febrile illness and a history of animal contact.

Fatal lipoid pneumonia in an infant complicated by Mycobacterium fortuitum infection.

We report a fatal case of lipoid pneumonia in a two and one half month old Saudi female caused by aspiration of animal fat, ghee and complicated by Mycobacterium fortuitum infection. It highlights the need to look for M. fortuitum in lipoid pneumonia and suggests the prevention of the condition through health education.

Urinary hydroxyproline and serum alkaline phosphatase in sickle cell disease.

Serum alkaline phosphatase, alkaline phosphatase isoenzymes, and urinary hydroxyproline excretion were studied in 20 young adult sickle cell patients and 58 matching normal controls. Total alkaline phosphatase was significantly higher in the sickle cell patients than in controls. Heat inactivation test and isoenzyme electrophoresis indicated that bone is the predominant isoenzyme in patients. Hydroxyproline excretion was significantly higher in the sickle cell patients than in controls. Serum total alkaline phosphatase correlated well with urinary hydroxyproline excretion in sickle cell patients (r = 0.73). Both alkaline phosphatase and hydroxyproline increased with age in the sickle cell patients. This study suggests that delayed growth and/or bone destruction may contribute to the elevated levels of alkaline phosphatase and urinary hydroxyproline.