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I Melki

Publications and source records attributed to I Melki.

15 recordsLinked to original sources
PubMed2003

Right congenital diaphragmatic hernia a well-known pathology?

In right congenital diaphragmatic hernia (RCDH), several clinical diagnostic pitfalls are possible and should be known to those caring for infants and children with this disorder. The records of the 18 patients at Hotel Dieu de France Hospital with a history of CDH between 1990 and 1999 were collected; those of the ten who had a RCDH were reviewed retrospectively. The mean age at diagnosis was 6 months; the male-to-female ratio was 2:3. The delay between the first symptom and the diagnosis ranged between 0 and 10.5 months (mean 4.5 months). An acute presentation was observed in four cases, consisting of respiratory distress in three; the 4th presented with gastric volvulus and intestinal obstruction. The presenting symptoms were mild in four cases; recurrent respiratory infections in three and failure to thrive in one. The diagnosis was incidental in two cases during the evaluation of respiratory symptoms attributed to an atrial septal defect. The radiologic findings provided by a chest radiograph (CxR) were sufficient to make an accurate diagnosis in eight cases and peritoneography was useful in one. In six cases, the presenting CxR had been misinterpreted as normal or acute lobar pneumonia. Pathologic findings at surgery consisted of lateral and posterior right diaphragmatic defects in nine cases; the defect was lateral and anterior in one. A hernia sac was found in seven cases; malrotation was present in three. Surgical correction was done by an abdominal approach in nine cases and a thoracic approach in one. The diaphragmatic defect was repaired by transverse closure in six cases, diaphragm plication in three and prosthetic closure in one. The postoperative outcome was uneventful in eight cases. Two patients died. Thus, RCDH seems to cause less severe symptoms than left-sided LCDH. It usually manifests beyond the neonatal period as respiratory or gastrointestinal symptoms. The diagnosis should be made easily by a CxR. The presence of a hernia sac correlated with a mild presentation. An abdominal surgical approach is preferred.

Female↗
PubMed2001

Infantile spasms in Down syndrome: good response to a short course of vigabatrin.

PURPOSE: To evaluate the efficacy of vigabatrin (VGB) in the treatment of infantile spasms (ISs) associated with Down syndrome (DS) and to assess the feasibility of early discontinuation to reduce the possible retinal toxicity. METHODS: Five children with ISs with DS were treated with vigabatrin as first-line monotherapy in an open prospective study. The short-term response was evaluated, and VGB was continued in responders. The treatment was stopped after 6 months in children who were still spasm free. RESULTS: Four children of five became spasm free with VGB, three of them responding within 1 week. This response was maintained during the 6 months of VGB treatment. After VGB discontinuation, and with a follow-up ranging from 2 to 4 years, none of the responders experienced spasm recurrence or other types of seizures. CONCLUSIONS: This study confirms the efficacy of VGB in ISs associated with DS. Moreover, it shows that the duration of VGB treatment can be reduced to 6 months without relapse of ISs. This short treatment might reduce the risk of developing visual field constriction.

Adolescent↗
PubMed2000

Overlap between Baller-Gerold and Rothmund-Thomson syndrome.

We report a male patient with craniosysnostosis, bilateral radial and ulnar hypoplasia, absent thumbs, poikiloderma, and short stature. His parents are first cousins. Although this patient was originally diagnosed as having Baller-Gerold syndrome it is more likely that he has Rothmund-Thomson syndrome or a similar disorder. This report confirms the overlap between these two syndromes, and that Baller-Gerold syndrome is essentially a diagnosis of exclusion.

Abnormalities, Multiple↗
PubMed1996

Congenital cyst of the pancreas. Antenatal diagnosis.

We hereby report the third case of antenatal diagnosis of congenital cyst of the pancreas. It is a very rare lesion and could present as a differential diagnosis of any intra-abdominal cystic mass. Early treatment is easy and prevents complications such as ascites and pancreatitis in case of intra-pancreatic enteric duplication.

Diagnosis, Differential↗
PubMed1996

Neurenteric cyst: antenatal diagnosis and therapeutic approach.

We hereby report the second case of antenatally diagnosed "neurenteric cyst" in the literature. The patient we describe presented as a neonatal respiratory distress syndrome (NRDS) which did not respond to a vigorous resuscitation. Anomaly of the antenatal ultrasonography and of the initial chest x-ray added to the non-response to therapy, led us to an urgent thoracic scan and to a lifesaving thoracotomy. We think that every posterior mediastinal cystic mass, with or without vertebral malformations, diagnosed antenatally, should raise the possibility of neurenteric cyst. The perinatal management of the newborn to be, will thus be simplified. This will lead to a better outcome.

Humans↗
PubMed1993

[Percutaneous central venous catheters in neonatal intensive care. Personal experience].

The authors describe their own experience about using a siliconed percutaneous central venous catheter in the newborn, experience performed in their neonatal intensive care unit in Beirut. The technique consists in introducing this sort of device centrally by using a peripheral vein. The authors performed a prospective study on thirty-two newborns: Their results seem to indicate, in accordance to the literature review, that this technique offers many advantages such as sparing the newborn veins, prolonged hyperalimentation and treatment, and less incidents than the other perfusion techniques (peripheral and central). Few inconveniences are encountered: limitation of the amount of daily perfusion, impossibility of transfusing blood or derivatives across the silicone catheter.

Birth Weight↗
PubMed1993

[Right-sided endocarditis caused by Staphylococcus aureus during the neonatal period. (A case report)].

Right-sided infectious endocarditis in the neonate, due to staphylococcus aureus, is a rare entity. A high index of suspicion should be used when dealing with a very sick neonate, especially with aggressive reanimation. Although the diagnosis is clinical, echocardiography is essential to confirm the diagnosis and also is an excellent mean to follow the evolution of the disease. The pathogenesis of the infection is due to bacteremia with implantation of the staphylococcus on a normal endothelium. The prognosis is poor with a high mortality rate and possibility of sequelae (essentially cardiac and neurologic). The prognosis depends essentially on early diagnosis and treatment which should last for at least 4-6 weeks. The authors present the case of a 2-week-old girl who developed a right-sided staphylococcal endocarditis following treatment of neonatal jaundice with i.v. perfusion of albumin. The patient survived with cardiac and central nervous system sequelae.

Anti-Bacterial Agents↗
PubMed1988

[Hyperexplexia: the startle disease].

A case of hyperexplexia is reported in a newborn. Hyperexplexia or "startle disease" is very uncommon and is of autosomal dominant transmission. Clinical features consist in particular physical features during the neonatal period and subsequently in an abnormal startle reaction; the electromyogram shows particular patterns.

Electromyography↗
PubMed1987

Perinatal serum bone Gla-protein and vitamin D metabolites in preterm and fullterm neonates.

Whether the hypocalcemia often found in premature neonates results from an adaptation to extrauterine life or an expression of imbalanced mineral homeostasis has yet to be established. We compared serum levels of 25-hydroxyvitamin D, 1,25-dihydroxyvitamin D [1,25-(OH)2D], and bone Gla-protein (BGP), a specific marker of bone formation during the first month of life in fullterm and preterm neonates. Measurements were performed in cord blood and on days 1, 5, and 30 of life. In maternal blood, mean serum 1,25-(OH)2D concentrations were higher in the mothers of premature than in those of fullterm neonates, and serum BGP concentrations were lower than those in nonpregnant women. In cord blood mean serum BGP levels were 2-3 times higher than in adults. Serum BGP increased significantly on days 5 and 30 in fullterm infants. In preterm infants, and increase was found only on day 30. Mean serum 25-hydroxyvitamin D and 1,25-(OH)2D concentrations were lower in neonates than in mothers, but not different in fullterm and preterm neonates. In fullterm infants serum 1,25-(OH)2D increased rapidly from birth to day 5 and decreased on day 30. The pattern was similar in preterm infants, but 1,25-(OH)2D was higher than in fullterm infants on day 30. No sustained correlation between serum BGP and 1,25-(OH)2D levels was found. These data support the contention that changes in 1,25-(OH)2D reflect the perinatal equilibration of calcium homeostasis. Serum BGP may be a potential marker of bone growth in premature neonates.

Calcifediol↗
PubMed1987

[Gastric rupture and resuscitation maneuvers].

Near drowning in a bathtub occurred to a 3 years 7 months old boy. He was resuscitated at home by the firemen before admission in hospital, where he presented coma, convulsions and a massive distension of the abdomen. Surgical procedure revealed a 3 cm long perforation on the smaller curvature, which was repaired. This aspect and the localisation of the rupture resemble those related to resuscitation procedures: oxygenotherapy by nasal catheter, external cardiac massage, mouth-to-mouth ventilation. The stomach is filled with air because inadequate position of the catheter. It also may be related to relaxation of crico-pharyngeal sphincter during anesthesia or coma.

Child, Preschool↗
PubMed1987

[A single daily injection of ceftriaxone for treating suppurated meningitis in infants and children. Apropos of 31 cases].

Thirty-one infants and children aged 1 month to 15 years 3 months were treated with ceftriaxone once a day for the treatment of a meningitis related to Neisseria meningitidis (19 cases), haemophilus influenzae (7 cases), streptococcus pneumoniae (1 case), not identified bacteria (4 cases). All identified bacteria were sensitive to ceftriaxone. Twenty children were treated with 100 mg/kg/day, 11 with 50 mg/kg/day. CSF was sterile at the first control-generally performed 30 h after the onset of treatment-in all cases. Despite a great number of severe forms (fulminans purpura and septic shock; 11 cases; severe neurologic disturbances: 6 cases), all patients survived and recovered after a treatment of 9 to 22 days. Two infants exhibited neurologic sequelae: deafness, delayed development and hydrocephalus. Tolerance to ceftriaxone appeared to be good. With a 100 mg/kg/day dosage, mean CSF level at 6 h was 3.3 mg/l (0.8-7.7), on the first day of treatment. At the end of treatment, mean CSF level at 24h was 0.47 (0.15-2.5). With a 50 mg/kg/day dosage, mean CSF level at 6 h was 2,1 mg/l (1.1-3.9) in the first day of treatment. At the end of the treatment, mean CSF level at 24h was 0.22 mg/l (0.08-0.5). Once a day administration of ceftriaxone is adequate for the treatment of meningitis in infants and children. Though a 50 mg/kg/day dosage is probably sufficient in most cases, it seems to be more secure to use a 100 mg/kg/day dosage.

Adolescent↗
PubMedDate not supplied

[Familial lymphohistiocytosis. Evolution of management apropos of 3 cases].

Familial lymphohistiocytosis is a rare rapidly lethal genetic disease. It is characterized by an uncontrolled activation of T lymphocytes and macrophages, with multiple organ infiltration, beginning with fever and unexplained coagulopathy. Recently, one of the genes implicated in 50% of families at risk was identified (locus FHL1, chromosome 10, region q21-22). Based on data suggesting an essential role of T lymphocytes in the genesis of familial lymphohistiocytosis, the treatment has recently evolved from a chemotherapy including Etoposide (VP16) and corticosteroids, sometimes efficient but toxic, to an almost always efficient and slightly toxic immunosuppressive treatment. These two treatments achieved a remission somewhat lasting with no definite cure. In fact, all patients relapsed in the central nervous system and died. Bone marrow transplantation (BMT) is the only curative treatment. However only 20% of patients benefit from an HLA identical BMT. Recent improvements in HLA non-identical BMT offer an acceptable alternative to the other 80% of patients. In this review, we present three cases illustrating the evolution and optimization in the management of infants with familial lymphohistiocytosis.

Chromosome Aberrations↗