Publications by authors named "Bourrahouat A"

9 Publications

  • Page 1 of 1

Atypical Presentation of Celiac Disease: Recurrent Acute Small Bowel Obstruction.

Clin Med Insights Case Rep 2021 13;14:1179547620986152. Epub 2021 Jan 13.

Pediatric B Department, Mother-Child Pole, Mohammed VI University Hospital, Marrakesh, Morocco.

Context: Intussusception is the most common cause of small bowel obstruction in children under 4 years of age. Intussusception is not a widely recognized complication of celiac disease.

Case Report: We present a clinical case of a 23-month-old boy with a 1-month history of watery diarrhea complicated by 2 episodes of intestinal obstruction, both had required surgery. He presented with acute and severe abdominal distention with bilious vomiting, and an appearance of intussusception on abdominal ultrasound. Upon further investigation, the diarrhea was found to be malabsorptive. The diagnosis of celiac disease was confirmed by the presence of specific serum autoantibodies (IgA Tissue transglutaminase and endomysium Antibodies >200 UI/ml with normal serum IgA level). He started a gluten-free diet and his symptoms were almost completely resolved.

Conclusion: Recurrent intussusception may be associated with celiac disease, so celiac serology is recommended in children with recurrent intussusceptions. However, intestinal tuberculosis and lymphoma associated with enteropathy should be considered in the differential diagnosis. Intussusception in celiac disease is usually transient and should be managed expectantly rather than early surgical reduction.
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http://dx.doi.org/10.1177/1179547620986152DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7809297PMC
January 2021

A New Presentation: Aphallia, Vesicoureteral Reflux, Rectovesical Fistula, and Adrenal Insufficiency.

Case Rep Pediatr 2020 23;2020:8826520. Epub 2020 Nov 23.

Pediatric B Department-Mother-Child Pole, Mohammed VI University Hospital, Marrakesh, Morocco.

Aphallia or penile agenesis is a rare congenital malformation with an estimated incidence rate of 1 in 10 to 30 million births. More than half of aphallia cases have associated anomalies including caudal axis, cardiovascular, genitourinary, and gastrointestinal anomalies. The penile agenesis associated with adrenal insufficiency has never been reported in an infant. We report a rare case of a newborn that was diagnosed as a case of aphallia with vesicorectal fistula and vesicoureteral reflux, complicated by adrenal insufficiency with salt-wasting crisis.
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http://dx.doi.org/10.1155/2020/8826520DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7704127PMC
November 2020

Update of a colorimetric method for quantitative determination of galactose in blood samples: A simple and rapid method for the early detection of inherited metabolic diseases.

Carbohydr Res 2020 Dec 17;498:108179. Epub 2020 Oct 17.

Metabolic Platform, Biochemistry Laboratory, Team for Childhood, Health and Development Faculty of Medicine, Cadi Ayad University, Marrakech, Morocco. Electronic address:

Background: A colorimetric microassay for the quantitative determination of galactose in the blood was taken and updated. This method helps in diagnosis and follow-up of several inherited metabolic diseases connected to galactose metabolism deficiency such as galactosemia, glycogenosis, glycosylation, tyrosinemia and citrin deficiency. Galactose assay in the blood presents difficulties due to interference with glucose. In this study, we update a method to get around these difficulties.

Method: This procedure was based on the incubation of whole blood with orcinol in a strongly acidic solution to form a galactose and glucose complexes able to absorb at two different wavelengths.

Results: The standard curve analysis for the individual solutions of these two sugars showed a wide range of linearity from 0 to 200 mg / l. Under optimal experimental conditions, the stirring time of the orcinol is 3 minutes, the heating time of the reaction is 20 minutes at 56 ° C, and the duration of the incubation in the dark is 40 minutes. The analysis is carried out on fresh blood. The maximum absorbance of galactose and glucose is respectively 569 nm and 421 nm. An adapted diagnosis algorithm was developed based on our results.

Conclusion: this method could help in screening and identifying patients with hypergalactosemia that need further investigations. It could represent a promising method for neonatal screening in countries with limited resources.
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http://dx.doi.org/10.1016/j.carres.2020.108179DOI Listing
December 2020

Laboratory abnormalities in children with novel Coronavirus Disease 2019.

Clin Med Insights Pediatr 2020 11;14:1179556520955177. Epub 2020 Sep 11.

Department of Paediatrics, University hospital of Mohammed VI, Marrakesh, Morocco.

The novel Coronavirus disease 2019 continues to be a worldwide pandemic. Yet, little is still known about the biological features of this emergent infection in children. In this prospective study, we collected 68 children infected with SARS-COV-2 from March 2020 to May 2020, in Marrakesh, Morocco. No severe cases were observed in this cohort, and 66% of the patients were asymptomatic. The main laboratory abnormalities were hematological, as we found Leucopoenia in 4.4% of the cases, hyperleukocytosis in 1.6%. Neutropenia was found in 5 patients (7%) and only 2 cases (3%) had Lymphopenia. The inflammation and coagulation biomarkers were normal in the majority of the cases, as for liver and kidney function. Lactate dehydrogenase (LDH) serum levels were elevated in 8 cases (11.67%). The COVID-19 in children seems to have mild course and better outcome than in adults, which impacts the laboratory findings in this category. More studies must be conducted to learn more about the laboratory abnormalities in pediatric COVID-19.
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http://dx.doi.org/10.1177/1179556520955177DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7488170PMC
September 2020

Implementation of an Affordable Method for MPS Diagnosis from Urine Screening to Enzymatic Confirmation: Results of a Pilot Study in Morocco.

Clin Lab 2020 03;66(3)

Background: Rapid and accurate diagnosis of mucopolysaccharidoses (MPS) is still a challenge due to poor access to screening and diagnostic methods and to their extensive clinical heterogeneity. The aim of this work is to perform laboratory biochemical testing for confirming the diagnosis of mucopolysaccharidosis (MPS) for the first time in Morocco.

Methods: Over a period of twelve months, 88 patients suspected of having Mucopolysaccharidosis (MPS) were referred to our laboratory. Quantitative and qualitative urine glycosaminoglycan (GAG) analyses were performed, and enzyme activity was assayed on dried blood spots (DBS) using fluorogenic substrates. Enzyme activity was measured as normal, low, or undetectable.

Results: Of the 88 patients studied, 26 were confirmed to have MPS; 19 MPS I (Hurler syndrome; OMIM #607014/Hurler-Scheie syndrome; OMIM #607015), 2 MPS II (Hunter syndrome; OMIM #309900), 2 MPS IIIA (Sanfilippo syndrome; OMIM #252900), 1 MPS IIIB (Sanfilippo syndrome; OMIM #252920) and 2 MPS VI (Maroteaux-Lamy syndrome; OMIM #253200). Parental consanguinity was present in 80.76% of cases. Qualitative urinary glycosaminoglycan (uGAGs) assays showed abnormal profiles in 31 cases, and further quantitative urinary GAG evaluation and Thin Layer Chromatography (TLC) provided important additional information about the likely MPS diagnosis. The final diagnosis was confirmed by specific enzyme activity analysis in the DBS samples.

Conclusions: The present study shows that the adoption of combined urinary substrate analysis and enzyme assays using dried blood spots can facilitate such diagnosis, offer an important tool for an appropriate supporting care, and a specific therapy, when available.
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http://dx.doi.org/10.7754/Clin.Lab.2019.190720DOI Listing
March 2020

Hepatitis A Virus Infection Associated with Cryoglobulinemic Vasculitis.

Indian Pediatr 2020 01;57(1):71-72

Department of Pediatrics, Child and Mother Hospital, Mohammed VI University Hospital Center, Marrakesh Medical and Pharmacy Faculty, Caddy Ayad University, City of Marrakesh, Morocco.

Atypical symptoms, especially immune complex disorders, are uncommonly reported with hepatitis A virus (HAV) infection. We report an 8-year-old child who contracted HAV infection complicated by cryoglobulinemic vasculitis, and responded well to oral steroids. HAV infection may be considered in the etiology of cryoglobulinemia in children.
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January 2020

46th Medical Maghrebian Congress. November 9-10, 2018. Tunis.

Authors:
A Alami Aroussi A Fouad A Omrane A Razzak A Aissa A Akkad A Amraoui A Aouam A Arfaoui A Belkouchi A Ben Chaaben A Ben Cheikh A Ben Khélifa A Ben Mabrouk A Benhima A Bezza A Bezzine A Bourrahouat A Chaieb A Chakib A Chetoui A Daoudi A Ech-Chenbouli A Gaaliche A Hassani A Kassimi A Khachane A Labidi A Lalaoui A Masrar A McHachi A Nakhli A Ouakaa A Siati A Toumi A Zaouali A Y Condé Abdeddayem Haggui Abdelaziz Belaguid Abdelkader Jalil El Hangouche Abdelkarim Gharbi Abdelmajid Mahfoudh Abderrazek Bouzouita Abir Aissaoui Abir Ben Hamouda Abir Hedhli Adel Ammous Afef Bahlous Afef Ben Halima Ahlem Belhadj Ahlem Bezzine Ahlem Blel Ahlem Brahem Ahmed Banasr Ahmed Meherzi Ahmed Saadi Ahmed Sellami Ahmed Turki Aicha Ben Miled Aida Ben Slama Aida Daib Alaa Zommiti Ali Chadly Ali Jmaa Ali Mtiraoui Alia Ksentini Alia Methnani Alya Zehani Amal Kessantini Amani Farah Amani Mankai Amani Mellouli Amani Zaouali Amany Touil Ameni Hssine Amine Ben Safta Amine Derouiche Amine Jmal Amir Ferjani Amira Djobbi Amira Dridi Amna Aridhi Anis Bahdoudi Anis Ben Amara Anis Benzarti Anouar Youssef Ben Slama Anouer Oueslati Aouatef Soltani Aschraf Chadli Asma Aloui Asma Belghuith Sriha Asma Bouden Asma Laabidi Asma Mensi Asma Ouakaa Asma Sabbek Asma Zribi Assia Green Atef Ben Nasr Aymen Azaiez Aza Yeades Azza Belhaj Azza Mediouni Azza Sammoud Azza Slim B Amine B Chelly B Jatik B Lmimouni Bachir Daouahi Badereddine Ben Khelifa Bechir Louzir Ali Dorra Besma Dhahri C Ben Nasrallah C Chefchaouni C Konzi C Loussaief C Makni Chadli Dziri Chaker Bouguerra Chaker Kays Chekib Zedini Cherif Dhouha Cherif Mohamed Chiraz Aichaouia Chiraz Dhieb D Fofana Dalila Gargouri Dhekra Chebil Dhilel Issaoui Donia Gouiaa Dorra Brahim Dorra Essid Dorra Jarraya Dorra Trad E Ben Hmida E Sboui Ehsen Ben Brahim Elyes Baati Emna Talbi Eya Chaari Eya Hammami Ezzeddine Ghazouani F Ayari F Ben Hariz F Bennaoui F Chebbi F Chigr F Guemira F Harrar F Z Benmoula F Z Ouali F M R Maoulainine Faten Bouden Faten Fdhila Fatiha Améziani Fatma Bouhaouala Fatma Charfi Fatma Chermiti Ben Abdallah Fatma Hammemi Fatma Jarraya Fatma Khanchel Fatma Ourda Fatma Sellami Fatma Trabelsi Ferdaous Yangui Feten Fekih Romdhane Fethi Mellouli Fethi Nacef Jomli Fethia Mghaieth G Draiss G Elamine G Kablouti G Touzani G B Manzeki Ghada Garali Ghassen Drissi Ghazi Besbes H Abaza H Azzouz H Said Latiri H Rejeb H Ben Ammar H Ben Brahim H Ben Jeddi H Ben Mahjouba H Besbes H Dabbebi H Douik H El Haoury H Elannaz H Elloumi H Hachim H Iraqi H Kalboussi H Khadhraoui H Khouni H Mamad H Metjaouel H Naoui H Zargouni H O Elmalki Habib Feki Habib Haouala Habib Jaafoura Habiba Drissa Habiba Mizouni Hager Kamoun Haifa Ouerda Haifa Zaibi Haithem Chiha Hajer Kamoun Hajer Saibi Hajer Skhiri Hamza Boussaffa Hamza Majed Hana Blibech Hana Daami Hana Harzallah Hanan Rkain Hanen Ben Massoud Hanene Jaziri Hanène Ben Said Haroun Ayed Hassen Harrabi Hatem Chaabouni Hayet Ladida Debbache Hayett Harbi Haythem Yacoub Hela Abroug Hela Ghali Hela Kchir Hela Msaad Héla Ghali Héla Manai Hend Riahi Hiba Bousselmi Hiba Limem Hichem Aouina Hichem Jerraya Houda Ben Ayed Houda Chahed Houda Snéne I Lahlou Amine I Nouiser I Ait Sab I Chelly I Elboukhani I Ghanmi I Kallala I Kooli Ibtissem Bouasker Ilhem Fetni Imen Bachouch Imen Bouguecha Imen Chaabani Imen Gazzeh Imen Samaali Imen Youssef Imen Zemni Imene Bachouche Imène Youssef Ines Bouannene Ines Kasraoui Ines Laouini Ines Mahjoubi Ines Maoudoud Ines Riahi Ines Selmi Ines Tka Islem Hadj Khalifa Islem Mejri Ismail Béjia J Bellagha Jallel Boubaker Jalloul Daghfous Jamel Dammak Jamila Hleli Jihen Ben Amar Jihen Jedidi Jihen Marrakchi K Kaoutar K Arjouni K Ben Helel K Benouhoud K Rjeb Kacem Imene Kais Samoud Kaouther El Jeri Karem Abid Kays Chaker Khadija Abid Khadija Bouzghaîa Khaled Kamoun Khaled Zitouna Khalil Oughlani Khaoula Lassoued Khaoula Letaif Khaouther Hakim L Cherif Alami L Benhmidoune L Boumhil L Bouzgarrou L Dhidah L Ifrine Lamia Kallel Latifa Merzougui Leila Errguig Leila Mouelhi Lina Sahli M Maoua M Rejeb M Ben Rejeb M Bouchrik M Bouhoula M Bourrous M Bouskraoui M El Belhadji M El Belhadji M Essakhi M Essid M Gharbaoui M Haboub M Iken M Krifa M Lagrine M Leboyer M Najimi M Rahoui M Sabbah M Sbihi M Zouine M C Chefchaouni M H Gharbi M M El Fakiri M R Tagajdid Maha Shimi Maha Touaibia Mahbouba Jguirim Maher Barsaoui Maissa Belghith Maissa Ben Jmaa Makram Koubaa Makram Tbini Malak Boughdir Mamia Ben Salah Manel Ben Fraj Manel Ben Halima Manel Ben Khalifa Manel Bousleh Manel Limam Manel Mabrouk Manel Mallouli Manel Rebeii Mariem Ayari Mariem Belhadj Mariem Ben Hmida Mariem Boughattas Mariem Drissa Mariem El Ghardallou Mariem Fejjeri Mariem Hamza Mariem Jaidane Mariem Jrad Mariem Kacem Mariem Mersni Mariem Mjid Mariem Sabbah Mariem Serghini Mariem Triki Maroua Ben Abbes Maroua Boussaid Maroua Gharbi Maroua Hafi Maroua Slama Maroua Trigui Marouen Taoueb Marouene Chakroun Marwa Ben Cheikh Marwa Chebbi Marwa Hadj Taieb Marwa Kacem Mehdi Ben Khelil Mehdi Hammami Mehdi Khalfallah Mehdi Ksiaa Mehdi Mechri Mehdi Mrad Mehdi Sboui Mejda Bani Melek Hajri Menel Mellouli Mohamed Allouche Mohamed Amine Mesrati Mohamed Amine Mseddi Mohamed Amri Mohamed Bejaoui Mohamed Bellali Mohamed Ben Amor Mohamed Ben Dhieb Mohamed Ben Moussa Mohamed Chebil Mohamed Cherif Mohamed Fourati Mohamed Kahloul Mohamed Khaled Mohamed Machghoul Mohamed Mansour Mohamed Morched Abdesslem Mohamed Ali Ben Chehida Mohamed Ali Chaouch Mohamed Ali Essid Mohamed Ali Meddeb Mohamed Chawki Gharbi Mohamed Habib Elleuch Mohamed Hédi Loueslati Mohamed Mehdi Sboui Mohamed Nabil Mhiri Mohamed Osman Kilani Mohamed Riadh Ben Slama Mohamed Ridha Charfi Mohamed Said Nakhli Mohamed Sami Mourali Mohamed Selim El Asli Mohamed Taher Lamouchi Mohammed Cherti Mohsen Khadhraoui Mokhtar Bibi Moncef Hamdoun Mondher Kassis Mongi Touzi Monia Ben Khaled Monia Fekih Monia Khemiri Monia Ouederni Monjia Hchicha Monther Kassis Mossadak Ben Attia Moufida Yahyaoui Mouna Ben Azaiez Mouna Bousnina Mounir Ben Jemaa Mounir Ben Yahia Mounir Daghfous Mourad Haj Slimen Msadak Assidi N Belhadj N Ben Mustapha N El Idrissislitine N Hikki N Kchir N Mars N Meddeb N Ouni N Rada N Rezg N Trabelsi Nabiha Bouafia Nabil Haloui Nacéra Benfenatki Naceur Bergaoui Naceur Yomn Nadia Ben Mustapha Nadia Maamouri Nadia Mehiri Nadia Siala Najeh Beltaief Najet Aridhi Najet Sidaoui Najia Walid Najla Mechergui Najla Mnif Nariméne Ben Chekaya Nawel Bellil Nawel Dhouib Nazek Achour Nejib Kaabar Nejib Mrizak Nejla Mnif Nesrine Chaouech Nesrine Hasni Nesrine Issaoui Nidhal Ati Nidhal Balloumi Nidhal Haj Salem Nizar Ladhari Nora Akif Nora Liani Nouha Hajji Nouha Trad Nour Elleuch Nour El Houda Marzouki Noureddine Larbi Nourelhouda M'barek Nouri Rebai Noursaf Bibani Nozha Ben Salah O Belmaachi O Elmaalel O Jlassi O Mihoub Olfa Ben Zaid Olfa Bouallègue Olfa Bousnina Olfa Bouyahia Olfa El Maalel Omar Fendri Ons Azzabi Ons Borgi Ons Ghdes Oussama Ben Rejeb R Rachid R Abi R Bahiri R Boulma R Elkhayat R Habbal R Rachid R Tamouza Rabaa Jomli Rabiaa Ben Abdallah Racha Smaoui Radhouene Debbeche Radhouene Fakhfakh Rafik El Kamel Rahma Gargouri Raja Jouini Ramzi Nouira Rana Fessi Rania Bannour Rania Ben Rabeh Rania Kacem Rania Khmakhem Raouf Ben Younes Raouf Karray Rezaik Cheikh Riadh Ben Malek Ridha Ben Slama Ridha Kouki Rim Baati Rim Bechraoui Rim Fakhfakh Rim Fradi Rim Lahiani Rim Ridha Rim Zainine Roua Kallel S Rostom S Ben Abdallah S Ben Hammamia S Benchérifa S Benkirane S Chatti S El Guedri S El Oussaoui S Elkochri S Elmoussaoui S Enbili S Gara S Haouet S Khammeri S Khefecha S Khtrouche S Macheghoul S Mallouli S Rharrit S Skouri Saan Helali Sabrine Boulehmi Sahar Abid Sahbi Naouar Saida Zelfani Salah Ben Amar Salem Ajmi Salem Braiek Salem Yahiaoui Salima Ghezaiel Salma Ben Toumia Salma Thabeti Salsabil Daboussi Salwa Ben Abderahman Samar Rhaiem Sami Ben Rhouma Samia Rekaya Samir Haddad Samir Kammoun Samira Merai Samira Mhamdi Rabaa Ben Ali Sana Gaaloul Sana Ouali Sana Taleb Saoussen Zrour Sarah Hamdi Sarah Zaghdoudi Sarra Ammari Sarra Ben Abderrahim Sarra Karaa Sarra Maazaoui Sarra Saidani Sarra Stambouli Seif Mokadem Selim Boudiche Selim Zaghbib Shema Ayedi Sinda Jardek Siwar Bouselmi Sonda Chtourou Sondos Manoubi Sonia Bahri Sonia Halioui Sonia Jrad Sonia Mazigh Sonia Ouerghi Sonia Toujani Soraya Fenniche Souad Aboudrar Souad Meriem Amari Souhaiel Karouia Soumaya Bourgou Soumaya Halayem Soumaya Rammeh Sourour Yaïch Syrine Ben Nasrallah Syrine Chouchane Syrine Ftini Syrine Makni Syrine Manoubi Syrine Miri Syrine Saadi Syrine Azza Manoubi T Khalfallah Takwa Mechergui Taoufiq Dakka Tarek Barhoumi Tej El Baha M'rad Thouraya Ajmi Trad Dorra Uta Ouali W Hannachi Wael Ferjaoui Wafa Aissi Wafa Dahmani Wafa Dhouib Wafa Koubaa Wafa Zhir Wafe Gheriani Wajdi Arfa Wajih Dougaz Wajih Sahnoun Walid Naija Y Sami Y Bouteraa Y Elhamdaoui Y Hama Yacine Ouahchi Yassine Guebsi Yassine Nouira Yosr Daly Yosra Mahjoubi Yosra Mejdoub Yosra Mosbahi Yosra Said Yosra Zaimi Yosra Zgueb Yousr Dridi Yousra Mesbahi Youssef Gharbi Youssef Hellal Z Hechmi Z Zid Z Elmouatassim Z Ghorbel Z Habbadi Zahra Marrakchi Zaki Hidouri Zeineb Abbes Zeineb Ouhachi Zied Khessairi Zied Khlayfia Zied Mahjoubi Zied Moatemri

Tunis Med 2019 Jan;97(1):177-258

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January 2019

Systemic Lupus Erythematosus-Related Pancreatitis in Children: Severe and Lethal Form.

Case Rep Pediatr 2018 31;2018:4612754. Epub 2018 Dec 31.

Pediatric ICU Department, Mother-Child Pole, Mohammed VI University Hospital, Marrakesh, Morocco.

Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease of unknown cause, characterized by multisystemic involvement. Its occurrence in children is rare, and acute pancreatitis is exceptional in this matter. Its diagnosis is clinical, biological, and radiological. Its treatment is based on corticosteroid therapy, and its progress is generally lethal. We report two cases of acute pancreatitis in the course of SLE, highlighting its life-threatening severity despite well-conducted treatment. : 14-year-old patient, admitted to the pediatric ICU for altered state of consciousness. This child, an outpatient since 2009 for chronic arthralgia, was hospitalized five days previously in the pediatric ward for suspicion of severe SLE, before presenting abdominal pain and vomiting. Hyperlipasemia was found, and an abdominal CT scan confirmed the diagnosis of acute pancreatitis. The patient was put under immunosuppressive therapy composed of high-dosage of corticosteroid and cyclophosphamide cures. She died 20 days after her hospitalization by severe lupus flare with multiorgan failure. : 14-year-old child, admitted to the Pediatric ward for prolonged fever associated with polyarthralgia (nondeforming, immovable, and additive) that had been progressing since 6 months with altered general state; his symptoms got worst 15 days before his hospitalization by having behavioral disorders and epigastralgia with vomiting. Pancreatitis was strongly suspected in the absence of improvement on symptomatic treatment and confirmed by hyperlipasemia 6 times the normal value and a swollen pancreas on the abdominal CT scan. The child was treated with Solumedrol and cyclophosphamide without improvement and then died after one month of hospitalization by a septic shock.
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http://dx.doi.org/10.1155/2018/4612754DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6332963PMC
December 2018

[Cyclophosphamide in idiopathic nephrotic syndrome: Outcome and outlook].

Nephrol Ther 2018 Apr 1;14(2):85-90. Epub 2018 Mar 1.

Service de pédiatrie B, CHU Mohamed VI, avenue Ibn Sina Amerchich, BP 2360, Marrakech-principal, Maroc.

Introduction: Cyclophosphamide (CYP) has been used for over 40 years in patients with steroid-sensitive nephrotic syndrome (NSSS) presenting frequent relapses (NSRF) or steroid dependence (NSSD). However, the long-term success of treatment with cyclophosphamide is difficult to predict. The objectives of this study are to determine long-term outcomes of cyclophosphamide and identify the factors associated with sustained remission.

Methods: We retrospectively studied the data from 50 patients with idiopathic nephrotic syndrome, treated by oral cyclophosphamide and followed at service of pediatric for more than 8 years for idiopathic nephrotic syndrome and related factors for survival without relapse were evaluated by univariate analysis.

Results: The median age at the time of diagnosis was 4.3 years, and median follow-up time was 1.7 years with the median of 8 years at the first use of CYC. Patients had received a median cumulative dose of 168mg/kg. At the end of follow-up, 38% of patients entered into remission after using CYC while 62% failed to respond and further relapses then occur. The median time of stopping corticosteroid therapy was three month. The survival without relapse was respectively 56% (28 patients), 52% (26 patients), 48% (24 patients), and 38% (19 patients), at 6 months, one year, two years and more than two years. In univariate analysis, the survival without relapse was related to the age at the moment of starting the therapy par CYC (the median was 5 months for an age < 8 years and 41 months for an age≥8 years; P=0.049), the type of nephrotic syndrome [36 months for SNRF, 4 months for NSSD and nephrothic syndrome steroid resistant (NSSR); P=0.068], and the histological lesion (6 months for diffuse mesangial proliferation, 2 months for segmental glomerulosclerosis; P=0.009). The age at the moment of diagnosis, the sex and the cumulative dose of CYC did not have significant influence.

Conclusion: The results presented in this study suggest the use of oral cyclophosphamide for short period remain second line effective therapy. Further well-designed trials are required to evaluate the efficacy of other steroid-sparing agents.
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http://dx.doi.org/10.1016/j.nephro.2017.03.004DOI Listing
April 2018