[Seizures revealing phosphocalcic metabolism abnormalities]. / Crises épileptiques révélant des anomalies du métabolisme phosphocalcique.

Hypocalcemia due to hypoparathyroidism produces a broad spectrum of clinical manifestations, but overt symptoms may be sparse. One unusual presentation is onset or aggravation of epilepsy in adolescence revealing hypoparathyroidism. This situation can lead to delayed diagnosis, with inefficacity of the antiepileptic drugs. We report five cases of adolescence-onset epilepsy with unsuccessful antiepileptic therapy, even with gradually increasing dose. Physical examination revealed signs of hypocalcemia, confirmed biologically. Full testing disclosed the origin of the seizures: hypoparathyroidism in three patients and pseudohypoparathyroidism in the other two. In four of five patients, computed tomography showed calcification of the basal ganglia, defining Fahr's syndrome. The patients were treated with oral calcium and active vitamin D (1-alphahydroxy vitamin D3). Seizure frequency progressively decreased and serum calcium levels returned to normal. These cases illustrate the importance of the physical examination and of routine serum calcium assay in patients with new-onset epileptic seizures in order to detect hypocalcemia secondary to hypoparathyroidism.

[Infantile herpes zoster]. / Zona chez un nourrisson.

Herpes zoster occurs seldom in infants, especially in the absence of exposure to maternal varicella either intrauterine or postnatal. We report on a case in a 3-month-old infant admitted for herpes zoster in the sciatic nerve territory. No cutaneous eruption was found in the mother or in people who were in contact with the patient. This rare clinical situation is here reviewed, showing that the absence of antenatal or postnatal exposure to herpes viruses does not preclude the occurrence of herpes zoster infection in early infancy.

[Severe poisoning by traditional medication in the newborn]. / Intoxications graves par médication traditionnelle chez le nouveau-né.

The use of traditional products for therapeutic purposes in the newborn cause many cases of severe poisoning, especially in newborns. The aim of this study was to identify the main medicinal plants causing poisoning and emphasize the seriousness of poisonous plants. We report a series of eight newborns admitted for intake of poisonous medicinal plants admitted to the neonatal unit and neonatal intensive care unit at the Fez (Morocco) Hassan II University Hospital in 2014. The reasons for use were dominated by the infant's refusing to suckle. Clinical signs at admission were neurological signs and respiratory distress. The decision was spontaneously declared by the family for only two patients. A particular smell of the urine and unusual lesion associations were the main signs drawing the attention of physicians in other cases. The amounts and rates differed. Laboratory tests revealed renal failure in two cases. Transaminases (SGOT and SGPT) were greater than three times normal and the TP was below 50% in two newborns. Hemoglobin less than 9g/dL in three cases and thrombocytopenia less than 50,000/mm3 in two newborns. The main poisonous plants were identified as cade oil and henna. Medicinal plants were associated in all cases. Toxicological analysis was negative in three cases and was not done in the other five cases. The management was symptomatic and etiological. The outcome was favorable in six newborns. Death occurred in two neonates despite resuscitation. Medicinal plants must, like drugs, obey strict rules that only the standard herbal medicine specialist can answer. This necessarily involves the regulation of the profession in our country.

[Neonatal mucolipidosis type II]. / Révélation néonatale d'une mucolipidose de type II.

Mucolipidosis type II (ML II, OMIM 252,500) is an autosomal recessive disorder clinically characterized by facial dysmorphia similar to Hurler syndrome and pronounced gingival hypertrophy. The disorder is caused by a defect in targeting acid hydrolases on the surface of lysosomes, which impede their entry and lead to accumulation of undigested substrates in lysosomes. The onset of the symptoms is usually in infancy, beginning in the 6th month of life. Early onset, at birth or even in utero, is a sign of severity and involves the specific dysmorphia as well as skeletal dysplasia related to hyperparathyroidism. We report on a severe neonatal form of this disorder revealed by respiratory distress with severe chest deformity. The dysmorphic syndrome, combining coarse features, pronounced gingival hypertrophy, with diffuse bone demineralization and secondary hyperparathyroidism associating significant elevation of parathyroid hormone and alkaline phosphatase with normal levels of vitamin D and calcium were characteristics of mucolipidosis type II. Recognizing this specific association of anomalies helps eliminate the differential diagnosis and establish appropriate diagnosis and care.

[Neonatal purpura fulminans without sepsis due to a severe congenital protein C deficiency]. / Purpura fulminans néonatal sans sepsis par déficit congénital sévère en protéine C.

Severe congenital protein C deficiency is a rare life-threatening coagulopathy. In the early hours of life, the neonate presents with extensive purpura fulminans and substantial skin necrosis contrasting with a preserved general state and a negative infectious exam. Disseminated intravascular coagulation sets in secondarily. Prenatal outset of thrombotic events is a rare situation that worsens the prognosis, especially protein C replacement in utero is not available. We report a case of a male newborn of consanguineous parents who were asymptomatic carriers of heterozygous protein C deficiency. This infant presented prenatal ventricular hemorrhage with hydrocephalus and rapidly extensive postnatal skin necrosis that was not regressive in spite of fresh frozen plasma administrated after 24h of life. Prenatal diagnosis, early recognition, and urgent therapy with protein C replacement and anticoagulant treatment are crucial to improve the prognosis, avoid further damage after delivery, and prevent the devastating consequences of severe protein C deficiency.

[Hypercalcemia complicating BCG lymphadenitis: case report]. / Hypercalcémie compliquant une bécégite locorégionale : à propos d'un cas.

One of the side effects of the BCG vaccine is a local infection that may spread to the regional lymph nodes causing lymphadenitis, which can resolve spontaneously without treatment. We report the case of an immunocompetent infant who developed lymphadenitis after administration of the BCG vaccine, complicated with persistent symptomatic hypercalcemia in spite of the usual treatment including corticotherapy. Antituberculous treatment was necessary to reduce this hypercalcemia.

[Subcutaneous emphysema, pneumomediastinum, pneumopericardium, pneumorachis, and pneumoretroperitoneum revealing an unknown foreign body aspiration]. / Emphysème sous-cutané massif, pneumomédiastin, pneumopéricarde, pneumorachis et pneumorétropéritoine révélant une inhalation méconnue d'un corps étranger.

Subcutaneous emphysema develops from the spread of air essentially from the mediastinum into the subcutaneous tissue causing progressive distension and infiltration. Diagnostic and therapeutic delay expose the patient to massive air effusion and risk of compression of cervical and mediastinal structures. The initial mechanism is a breach in the tracheobronchial tree with air diffusion into the interstitial space and along the perivascular spaces toward the mediastinum, and then spread and dissection in the subcutaneous tissue. A tracheobronchial foreign body is a very rare cause of emphysema and is often localized. An association with a combination of epidural emphysema, pneumopericardium, or pneumoretroperitoneum is exceptional. Here, we present a unique case associating massive subcutaneous emphysema, pneumomediastinum, pneumopericardium, pneumorrhachis, and pneumoretroperitoneum in a 3.5-year-old child complicating an unrecognized aspirated foreign body. The extraction of the foreign body resulted in gradual regression of the symptoms and the disappearance of these emphysematous locations.

[The contribution of transfontanellar ultrasound in the early diagnosis of neonatal cerebral venous thrombosis: report on three cases]. / Apport de l'échographie transfontanellaire dans le diagnostic précoce des thromboses veineuses cérébrales néonatales : à propos de trois cas.

The clinical aspects of neonatal cerebral venous thrombosis are polymorphic in their mode of onset, making diagnosis difficult. Transfontanellar ultrasound allows for the morphological and vascular exploration of intracranial contents. We report three cases of neonatal cerebral venous thrombosis diagnosed early by transfontanellar ultrasound. The patients were treated with low-molecular-weight heparin, and the outcome was favorable in all three newborns. Transfontanellar ultrasound has a variable sensitivity for the detection of thrombus, but it remains the first-line examination before any additional secondary exploration. The early diagnosis of neonatal cerebral venous thrombosis with transfontanellar ultrasound requires the implementation of emergency treatment.

[Overdose or hypersensitivity to vitamin D?]. / Surdosage ou hypersensibilité à la vitamine D ?

Vitamin D intoxication with severe hypercalcemia is rare in the neonatal and infancy period. Through nine cases of hypercalcemia, secondary to taking 600,000 units of vitamin D (Sterogyl(®)), a review of vitamin D requirements and possible mechanisms of toxicity including hypersensitivity to this vitamin will be discussed. We report nine cases of babies admitted to our department between the ages of 25 and 105 days for treatment of severe dehydration. The pregnancies were normal, with no incidents at delivery. Clinical signs were dominated by weight loss, vomiting, and fever. Examination on admission revealed dehydration whose degree ranged from 8 to 15% with preserved diuresis and loss weight between 100 and 1100 g. Laboratory tests objectified hypercalcemia between 113 and 235mg/L, hypercalciuria (urinary calcium/creatinine mmol/mmol >0.5), and a low-level of parathyroid hormone. The vitamin D values in nine patients were toxic (344-749 nmol/L; normal >50 nmol/L; toxicity if >250 nmol/L). Abdominal ultrasound objectified renal nephrocalcinosis in seven patients. The DNA study, performed in eight patients, did not reveal a mutation of the vitamin D 24-hydroxylase gene (CYP24A1). The treatment consisted of intravenous rehydration with treatment of hypercalcemia (diuretics and corticosteroids). Serum calcium returned to the normal range within 4-50 days, with weight gain progressively over the following weeks. The follow-up (2 years for the oldest case) showed the persistence of images of nephrocalcinosis. Genetic susceptibility and metabolic differences appear to modulate the threshold of vitamin D toxicity. However, respect for recommended doses, recognized as safe in a large study population, reduces the risk of toxicity.

[Congenital chylothorax: rapid and complete response to polyvidone iodine]. / Chylothorax congénital : une réponse rapide et totale à la polyvidone iodée.

Idiopathic congenital chylothorax refers to abnormal accumulation of lymphatic fluid within the pleural space due the disruption of the thoracic duct or its lymphatic tributaries. It is a relatively rare disease and a cause of neonatal respiratory distress with major nutritional and immunological consequences. Therefore, it is critical to decrease pleural effusion promptly. Conservative treatment is always started as soon as the diagnosis is made, and refractory cases require chemical pleurodesis or surgery. However, the choice and timing of therapeutic escalation is not clear in the neonatal period and long waiting periods may have adverse consequences for the baby. We report a case of congenital idiopathic chylothorax who did not respond to conservative treatment after 18 days in whom one intrapleural injection of 5 mL of 4% concentrated Betadine stopped the pleural effusion promptly, effectively, and definitively, with no side effects observed, thus challenging such delayed administration.

[Neonatal group A streptococcal meningitis and portal vein thrombosis: a casual association?]. / Méningite néonatale à streptocoque A et thrombose porte : une association fortuite ?

Invasive group A streptococcal infections are potentially serious. The occurrence in the neonatal period and meningeal location are two unusual situations. The complications reported in the literature vary; we add the risk of thromboembolic events. We report the case of a newborn, admitted to our department at 22 days of life for late neonatal group A streptococcal meningitis and diffuse cerebral infarction lesions. Ultrasound and abdominal CT scan objectified the presence of portal vein thrombosis and cavernoma. Echocardiography, electrocardiogram, as well as coagulation and thrombophilia tests were normal. Progression was marked by the installation of cerebral atrophy and ventricular dilation without the appearance of signs of portal hypertension over 18 months. We therefore concluded in neonatal group A streptococcal meningitis complicated by multiple thrombosis that can be explained by the invasive properties and hypercoagulability characterizing group A beta-hemolytic streptococcus. However, the characteristics of the fetal circulation may explain the possibility of paradoxical cerebral embolism from portal thrombosis.

[Jaundice and urinary tract infection in neonates: simple coincidence or real consequence?]. / L'ictère au cours de l'infection urinaire chez le nouveau-né : simple coïncidence ou véritable conséquence ?

UNLABELLED: In neonates, jaundice may be one of the initial symptoms related to urinary tract infection (UTI). The routine testing of the urine in jaundiced neonates is controversial. This study aimed to evaluate the related factors of neonatal infants with the initial presentation of hyperbilirubinemia and the final diagnosis of UTI by evaluating data that help diagnose UTI early in apparently healthy newborns with jaundice. PATIENTS AND METHODS: We retrospectively investigated the medical records of neonates who had been admitted for management of jaundice (n=26) and compared with neonates with jaundice but without UTI (n=26). RESULTS: There was a significant difference between the two groups in male gender and maternal conditions (prolonged rupture of membranes, maternal UTI). There was also a significant difference between the two groups in their age at the time jaundice started (4 ± 3 days vs 2 ± 1 days) in the UTI and non-UTI groups, respectively (P>0.05). The cases in the UTI group had significantly lower total bilirubin levels (183 ± 71 mg/l) vs (227 ± 40 mg/l) in the non-UTI group, but a higher indirect bilirubin rate than the non-UTI group (P<0.05). Type B blood group was more common in neonates with UTI (P<0.01). In the cases presented herein, none of the jaundiced infants with UTI presented conjugated hyperbilirubinemia. Therefore, urinary tests for UTI should not be absolutely excluded or neglected in neonates in the early stage with unconjugated hyperbilirubinemia. Performing urinary tests to exclude the possibility of coincidental UTI may be necessary for admitted jaundiced infants younger than if they have a high level of indirect bilirubin, especially in male newborns with group B blood and in the presence of maternal urinary infection.

Epidemiological and clinical characteristics of scorpion stings in children in Fez, Morocco

Scorpion stings are a public health problem in Morocco, especially among children, who experience the most severe cases. Epidemiological and clinical findings on scorpion stings in Fez, Morocco, were evaluated in this investigation. Of 163 cases that required medical attention, 62.6 percent were male children. The mean age of patients was 4.8 ± 3.4 years. The mean time between stings and first medical attention was 3.36 ± 2.5 hours. Almost all cases occurred in the summer (94 percent) and extremities represented the most frequent sting sites (86.5 percent). Local pain, hyperemia, scarification, vomiting, sweating, restlessness, tachycardia and tachypnea were the observed clinical symptoms. Regarding severity, 55.2 percent of patients belonged to class III, followed by class II (26.4 percent) and class I (18.4 percent). None of our patients received antivenom; however, all of them were treated symptomatically depending on clinical manifestations.(AU)