A rare case of Evans syndrome associated with sudden loss of vision: A case report.

PURPOSE: To report a case of Evans syndrome with a vascular occlusive event leading to severe loss of vision. CASE REPORT: A 12-year-old boy with Evans syndrome presented with painless acute loss of vision in the left eye during a period of remission from the disease. Examination showed visual acuity of hand motion in the left eye, left relative afferent pupillary defect, pale optic nerve head, and attenuated vessels. Optical coherence tomography showed thin macula in both eyes, and fundus fluorescein angiography revealed delayed filling time of the arterial phase in the left eye, with attenuation and sclerosis of the arterioles. An electrophysiological study showed an electronegative electroretinogram. Based on these findings, we reached a concurrent diagnosis of atypical retinitis pigmentosa in both eyes along with a major superimposed vascular occlusive event in the left eye leading to severe visual loss. CONCLUSION: This is a case describing a rare ocular complication of Evans syndrome, leading to severe loss of vision due to vascular occlusion of unknown mechanism.

Intrauterine upper limb ischemia associated with fetal thrombophilia: a case report and review of the literature.

Neonatal extremity gangrene is rare, even rarer are those born with evidence of intrauterine vascular occlusion. Intrauterine limb ischemia has been attributed to several etiological factors which include thromboembolic disease occluding the arteries of the affected limb or compression of the limb during intrauterine life. In this report, we present a case of brachioradial arterial thrombosis associated with mild homocysteinemia and double heterozygosity of methylenetetrahydrofolate reductase 677C-T and factor V Leiden gene mutations. We suggest investigating the neonates and their mothers for possible genetic prothrombotic risk factors when they present with intrauterine thrombosis as this issue is important for management and counseling.

Idiopathic hypertrophic pachymeningitis in a child with hydrocephalus.

Idiopathic hypertrophic pachymeningitis is a rare but increasingly recognized disorder characterized by diffuse thickening of the dura mater of unknown etiology. The inflammation usually involves the cranial or spinal dura mater, with resultant neurologic deficits. Although it is reported primarily in adults, there is one previous report describing the condition in a child. Described here is the case of a child who presented at the age of 3.5 years with idiopathic hypertrophic pachymeningitis involving the entire central nervous system, with poor response to steroids, cyclophosphamide, and intraventricular cytarabine.

Predictors of severe hemolysis in patients with glucose-6-phosphate dehydrogenase deficiency following exposure to oxidant stresses.

BACKGROUND AND OBJECTIVES: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a genetic enzymatic disorder that affects millions of people worldwide, and is a major health problem in Jordan. We studied factors that may predict severe hemolysis in children with G6PD deficiency. METHODS: We reviewed the records of patients with low G6PD activity admitted to a teaching hospital be- tween 1996 to 2007. We collected demographic data, details of sign and symptoms, history and type of fava bean ingestion, blood and Rh group, history of neonatal jaundice, history and type of drug use, abdominal pain at admission and the results of tests for hemoglobin, white blood cells (WBC), and hepatic function. We classified patients into mild and severe groups based on hemoglobin levels at admission. RESULTS: Of 428 children with G6PD deficiency, 79 (18%) were severe cases and 349 (82%) patients with mild disease. There were no statistically significant differences in most factors between the two groups. Factors that achieved statistical significance for severe hemolysis included younger age (P<.05), male gender (P<.05), higher alkaline phosphatase (ALP) (P<.05), presence of fever at admission (P<.01), presence of vomiting during the at- tack (P=.006), and a negative family history for G6PD deficiency (P=.005). CONCLUSIONS: Severe hemolysis can be predicted during hemolytic episodes in children with low G6PD by young age, male gender, a negative family history of G6PD deficiency, the presence of fever and vomiting and a high ALP.

Emergency peripartum hysterectomy in Northern Jordan: indications and obstetric outcome (an 8-year review).

OBJECTIVE: To review cases of emergency peripartum hysterectomy regarding their incidence, risk factors, indications and complications and their results were carefully analysed. MATERIALS AND METHODS: A retrospective study of cases of emergency peripartum hysterectomy which were performed in the period between February 1994 and February 2002 at the Princess Badeea Teaching Hospital in Northern Jordan. Demographic and clinical data were extracted and closely interpreted RESULTS: In the study period there were a 70,252 deliveries and 61 cases of emergency peripartum hysterectomies. The overall incidence was 0.87 peripartum hysterectomies per 1,000 deliveries. There were 50 cases (82%) delivered by caesarean section and 11 cases (18%) were delivered vaginally. Caesarean hysterectomy was performed in 50 cases and postpartum hysterectomy was performed in 11 cases. Total hysterectomy was performed in 39 cases (64%) and subtotal hysterectomy was performed in 22 cases (36%). The main indications for hysterectomy were morbidly adherent placenta (47.5%), ruptured uterus (27.9%) and uncontrollable haemorrhage from uterine atony (21.3%). There were two maternal deaths and 7 cases of stillbirths and 4 cases of early neonatal deaths. CONCLUSION: Peripartum hysterectomy is a dramatic with high risk but a life saving operation. It is usually associated with significant maternal and fetal morbidity and mortality. Obstetricians should identify patients at risk and anticipate the procedure and complications, as early intervention and proper management facilitate optimal outcome.