Epidemiology, clinical features, and mortality rate of Wilson disease in Moroccan children: A pediatric case series.

BACKGROUND AND STUDY AIMS: Wilson's disease is an autosomal recessive disorder, that affects copper metabolism, leading to copper accumulation in the liver, nervous system, and cornea. Data are lacking on the epidemiology, the clinical and laboratory characteristics, treatment, and survival of Wilson's disease in Morocco. The aim of this study was to examine these features and the cause of death in a Moroccan pediatric population. PATIENTS AND METHODS: The study was carried out at the University Hospital Center of Marrakesh, Morocco; 46 children were diagnosed with Wilson's disease from 2008 to 2019. The diagnosis was based on low serum ceruloplasmin, increased urinary copper concentrations, the presence of Kayser-Fleischer rings, a family history of Wilson's disease, and a Leipzig score of ≥ 4. RESULTS: A total of 42 patients were referred to the center for hepatic or neurological manifestations; four patients were asymptomatic. Consanguineous marriage was found in 67.4% of the cases. The mean duration of illness (42 patients) was 4.9 ± 3.9 years. Kayser-Fleischer rings were found in 60.9% of 46 patients. Of the 42 symptomatic patients: 28 of 30 (93.3%) patients had low serum ceruloplasmin (<0.2 g/L), and 24 h urinary copper >100 µg/day was found in 34 of 35 (97.1%) cases. The treatment was established with D-penicillamine for 43 of the 46 patients, with zinc acetate for one patient and with zinc sulfate in for one patient, while one patient was not treated. D-penicillamine was discontinued in nine patients because of adverse effects such as thrombocytopenia, neurological deterioration, pancytopenia, severe vomiting and severe hypersensitivity. In total 28 patients were clinically and biologically stabilized, two patients experienced vision loss, and 16 patients died (38%). The main cause of death was diagnosis made at an advanced stage of disease and stopping treatment. CONCLUSION: Wilson's disease is a rare condition associated with treatement efficacy, but late diagnosis and stopping treatment can lead to a high mortality rate.

[Cerebral revascularisation in Takayasu's arteritis]. / Revascularisation cérébrale dans la maladie de Takayasu.

SUBJECT: Supraaortic angioplasty is often not feasible in patients with Takayasu's arteritis because of involvement of long segment of arteries. Consequently, the role of surgical treatment in the management of cerebral ischemia is important in this disease. The objective of this work is to specify the indications and surgical techniques in lesions of arteries to the head in this disease and to report our experience. METHODS: Seven patients with cervical arterial lesions due to Takayasu's arteritis were treated by bypass surgery in the department of vascular surgery, Ibn-Sina hospital on one period of 11 years. RESULTS: It is about 6 women and one man of middle age at the time of the diagnosis of 33,8 years. The revealing signs were essentially of neurological and ocular order. Six of our patients were in inflammatory thrust at the time of the diagnosis, and required a medical treatment first to basis of corticosteroids. Bypasses from the ascending aorta to the carotid artery were performed in six cases. In one case, the bypass was performed between the brachiocephalic artery and common carotid artery. A death in relation with a cerebral hemorrhage occurred 2 days after the revascularisation. A clean improvement of the functional signs was noted among 3 patients, whereas the improvement was partial at two other. A secondary thrombosis of the bypass surgery occurred in 3 cases. CONCLUSION: The natural history of Takayasu's arteritis and its evolution is badly known. The operative indications must not rest solely on the only anatomical balance, but based on a bundle of arguments in which, the assessment of the cerebral blood flow would be useful. Cerebral hyperperfusion syndrom constitutes a major risk that can be reduced by staged revascularisations.

[High cord blood TSH in Morocco: iatrogenic hypothyroidism?]. / TSH au cordon élevées au Maroc: hypothyroïdie iatrogène?

A short prospective study was conducted to assess thyroid status in healthy full term newborns (n=90) of a large maternity of Marrakech (Morocco), as part of the validation of a national salt iodisation program. High TSH (>5mU/l) was detected in 89% of infants tested; urinary iodine excretion was measured in 35 of the mothers, and was found to be normal (100-200microg/l) or high (>200microg/l)(n=27) in all of them. Milk iodine concentration was measured in 315 lactating women from the same area. Low values (<41microg/l) were found in 60% of them. The common use of iodinated disinfectants during delivery could be responsible for the high urine iodine values of mothers; however iodine deficiency seems to remain a widespread problem in this population and justifies a large scale survey of iodine status.

Iron availability and consumption of tea, vervain and mint during weaning in Morocco.

BACKGROUND/AIMS: Iron deficiency impairs growth and psychomotor development of infants. In Morocco, infusions are introduced very early in infant diet, and could contribute to iron deficiency, due to their high polyphenol content. METHODS: The availability of tea, mint and vervain infusions was assessed using an in vitro model of digestion and dialysis. Two gastric pHs were used: pH 4 as in the first week life, and pH 2.5 as in older infants. Six repetitions of each experiment were made. The total polyphenol content of infusions was measured. RESULTS: At pH 4 and at pH 2.5, iron availability was decreased by tea and vervain, and increased by mint and ascorbic acid. At both pHs it was increased by addition of ascorbic acid to tea and vervain. In addition, at pH 2.5 it was increased by addition of ascorbic acid to mint. The highest value was observed in the presence of both ascorbic acid and mint (33.1 +/- 4.1%). In any case, iron availability was higher at pH 2.5 than at pH 4 (with single compounds or combinations with ascorbic acid). The polyphenol contents (mg/l) of tea, vervain and mint infusions were 2,236.1, 771.1, and 16.5. CONCLUSIONS: Tea and vervain infusions inhibited iron availability. In contrast, mint improved it; vitamin C helped in preventing these inhibiting properties. It could be proposed to discourage tea and vervain drinking at early weaning and to replace them by mint infusion, or at least to promote the consumption of vitamin C-rich fruit juice to counteract these inhibiting effects.

Toxic and essential trace metals in muscle, liver and kidney of bovines from a polluted area of Morocco.

Toxic and essential trace metals were measured in muscle, bone, liver and kidney of bovine grazing on the municipal wastewater spreading field of Marrakech City (Morocco). Bovines were found to be seriously contaminated by toxic metals, for metal bioaccumulation. The high cadmium content seemed to contribute to a reduction in zinc and copper levels. The arithmetic mean concentrations of zinc, especially cadmium, and levels were higher in liver and kidney, specific target organs copper and cadmium in liver and kidney, were respectively: 126, 112 and 5.1 microg/g in liver; 89, 33 and 10.3 microg/g in kidney.

Lead and traditional Moroccan pharmacopoeia.

The use of traditional cosmetics and remedies such as kohl and henna is very common in Morocco, especially among women, children and babies. Kohl is a dangerous eye cosmetic. It is usually mixed with other harmful substances, then applied on women's eyebrows and used in skin treatments for infants. Henna is another traditional product, with religious associations, which has been widely used over the centuries for cosmetic and medical purposes. Many people add various herbs or other substances to the henna in order to strengthen it or to give it a stronger colour. Our results were reassuring in that the concentrations of lead found in non-elaborate (henna only) samples of henna were low. However, when henna was mixed with other products (elaborate henna), these concentrations increased. Lead concentrations in kohl were very high however, unlike henna, were lower in mixed kohl as mixing with other products diluted the concentration of lead. Nevertheless, in both types of kohl, lead concentrations were very high and consequently constitute a risk for public health, particularly for children.

Trace elements in children's hair, as related exposure in wastewater spreading field of Marrakesh (Morocco).

Lead and cadmium concentration was determined in the hair of 327 school children living in a wastewater spreading field of Marrakesh (Morocco). The influence of age, sex, food habits and family occupation on the children's hair Pb and Cd concentration was also evaluated. Girls had more metal in their hair than boys (16.5 +/- 5.4 micrograms/g and 12.5 +/- 3.5 micrograms/g, respectively). However, for Cd the boys had more metal (2.9 +/- 0.6 and 2.2 +/- 0.4, respectively) but the difference was not statistically significant and metal levels decreased with age. Family occupation, direct contact with wastewater, customs and food habits were the most significant factors influencing the metal content of children's hair. The average Pb and Cd content were higher in the exposed children (14.8 +/- 4.5 micrograms/g and 2.5 +/- 0.5 micrograms/g, respectively) than in the non-exposed children (4.6 +/- 2.2 micrograms/g and 0.6 +/- 0.2, respectively), but the difference was not statistically significant. This study shows that in this area all the population (especially children) was extremely exposed to the danger caused by potentially toxic metals.