ABSTRACT
Objective: This study describes the clinical and haematological features of childhood Visceral Leishmaniasis from Pakistan
Materials and Methods: A total of 32 children, all below twelve years of age, with a confirmed diagnosis of Visceral Leishmaniasis [VL] were included in this study. All cases presented to the Children Hospital, Pakistan Institute of Medical Sciences [PIMS] Islamabad, from January 2014 to December 2016. The diagnosis of VL was established by the demonstration of Leishmania Parasites in bone marrow aspiration. Demographic information, physical signs at presentation, results of complete blood count and findings on bone marrow aspiration were recorded. The relationship between lymphadenopathy and histiocyte prominence with peripheral blood counts was also determined. The data was analyzed on SPSS version 23
Results: It was found that fever, pallor and abdominal distention were present in 100% of cases observed. Among the hematological features, pancytopenia in the peripheral blood was present in 20[62.5%] cases, histiocyte prominence in 19 [59.3%] cases and extracellular distribution of LD bodies in the bone marrow in 23[71.8%] cases. The values of peripheral blood counts were not significantly associated with the presence or absence of lymphadenopathy or increased Histiocytes in the bone marrow
Conclusion: Children from endemic areas coming with features of pallor, fever, abdominal distension, and reduced peripheral counts should prompt the clinician to suspect Visceral Leishmaniasis and the pathologist to carefully search for the presence of LD bodies in the bone marrow, in order to aid in the early identification and treatment of this infection
ABSTRACT
Persistent Mullerian Duct Syndrome [PMDS], a rare form of male pseudohermaphroditism, is characterized by the persistence of Mullerian duct structures [uterus, fallopian tubes and upper two-thirds of vagina] in otherwise normally-virilized males [Karyotype 46XY]. Patients suffering from PMDS present with cryptorchidism, inguinal hernia and infertility. Diagnosis is established when Mullerian duct structures are discovered either during ultrasonography for localization of undescended testis[s], during surgical exploration for cryptorchidism or herniorrhaphy [hernii uteri inguinalis]. Presence of both testes on one side of the scrotum is known as Transverse Testicular Ectopia [TTE]. Co-existence of PMDS and transverse testicular ectopia in a patient of mosaic Klinefelter's syndrome [Karyotype 46XY/47XXY] is a unique genetic association
Subject(s)
Humans , Male , Cryptorchidism , Klinefelter Syndrome , Mosaicism , Infertility , Hernia, Inguinal , Testis/abnormalities , Disorders of Sex DevelopmentABSTRACT
In Beta Thalassaemia Major repeated blood transfusions, ineffective erythropoiesis and increased gastrointestinal iron absorption lead to iron overload in the body. The management of the iron overload in these patients requires the administration of iron chelators continuously and evaluation of serum ferritin levels at regular intervals. In the present study serum ferritin levels, of the patients with beta thalassaemia major registered at two different centers of Rawalpindi and Islamabad, were measured. Majority of the patients revealed very high ferritin levels, with a mean of 3390 ng/ml. 21.34% patients had serum ferritin between 1000 to 2500 ng/ml, while 76% patients had values above 2500 ng/ml. These levels reflect inadequate chelation and vulnerability to develop iron overload related complications. There is a dire need to rationalize the chelation therapy, as at present no chelation, inadequate chelation, improper methods of chelators administration, non availability of infusion pumps, non affordability of patients to purchase pumps and chelators, inappropriate evaluation of iron overload and high levels of serum ferritin gives an overall bleak view