25 research outputs found
Individual variation in levels of haptoglobin-related protein in children from Gabon
Background: Haptoglobin related protein (Hpr) is a key component of trypanosome lytic factors (TLF), a subset of highdensity lipoproteins (HDL) that form the first line of human defence against African trypanosomes. Hpr, like haptoglobin (Hp) can bind to hemoglobin (Hb) and it is the Hpr-Hb complexes which bind to these parasites allowing uptake of TLF. This unique form of innate immunity is primate-specific. To date, there have been no population studies of plasma levels of Hpr, particularly in relation to hemolysis and a high prevalence of ahaptoglobinemia as found in malaria endemic areas. Methods and Principal Findings: We developed a specific enzyme-linked immunosorbent assay to measure levels of plasma Hpr in Gabonese children sampled during a period of seasonal malaria transmission when acute phase responses (APR), malaria infection and associated hemolysis were prevalent. Median Hpr concentration was 0.28 mg/ml (range 0.03-1.1). This was 5-fold higher than that found in Caucasian children (0.049 mg/ml, range 0.002-0.26) with no evidence of an APR. A general linear model was used to investigate associations between Hpr levels, host polymorphisms, parasitological factors and the acute phase proteins, Hp, C-reactive protein (CRP) and albumin. Levels of Hpr were associated with Hp genotype, decreased with age and were higher in females. Hpr concentration was strongly correlated with that of Hp, but not CRP
Incomplete Kawasaki Disease with Recurrent Skin Peeling: A Case Report with the Review of Literature
Kawasaki disease (KD) is an acute systemic vasculitis of unknown
aetiology that has largely replaced rheumatic heart disease as a cause
of acquired heart disease in children of many developed countries. We
report a case of incomplete KD in a five-year-old girl. The diagnosis
of incomplete KD was made after exclusion of conditions with similar
presentation. She was treated with intravenous immunoglobulin following
which she made an uneventful recovery but demonstrated thrombocytosis
in the second week of convalescence. During the six-month follow up
period, she had two episodes of recurrent skin peeling a phenomenon,
which is recently reported with KD but not with atypical or incomplete
KD. It is important for the treating physicians to become aware of the
incomplete KD as prompt diagnosis and early treatment of these patients
with intravenous immunoglobulin is vital for the prevention of lethal
coronary complications. Physicians need to have a "high index of
suspicion" for KD and even, higher for IKD. (J Postgrad Med
2003;49:72-74
Nephritis and cerebellar ataxia: Rare presenting features of enteric fever
Enteric fever is a common infectious disease of the tropical world,
about 80% of these cases occur in Asian countries. Enteric fever
presenting with isolated cerebellar ataxia or nephritis is rare. We
report three cases of enteric fever that presented with these
complications. Isolated cerebellar ataxia usually occurs in the second
week, whereas in our cases it presented within first four days of
fever. The common complications of enteric fever related to the urinary
tract are cystitis, pyelitis, and pyelonephritis. Glomerulonephritis is
uncommon. Most patients with enteric glomerulonephritis present with
acute renal failure, hypertensive encephalopathy, or nephritic
syndrome. In comparison, our case had milder manifestations. All three
patients were treated with parenteral ceftriaxone and showed a prompt
recovery
Study of Takayasu's Arteritis in Children: Clinical Profile and Management
AIMS: To study clinical features, pattern of involvement and treatment
modalities of Takayasu's arteritis (TA) in children. MATERIAL AND
METHODs: Retrospective analysis of hospital records of children
admitted over a period of 4.5 years. Results: 17patients in the age
group of 5 to 11 years (M: F = 1.1: 1) were diagnosed to have TA on the
basis of Ishikawa's criteria. One child was diagnosed post-mortem. The
commonest presenting features were hypertension (64.7%), congestive
cardiac failure (47%), weak or absent peripheral pulses, cardiomyopathy
(41.1% each) and cardiac valvular affection (35.2%). Retinopathy,
hypertensive encephalopathy and abdominal bruits were uncommon. None
presented in the prepulseless phase. No patient had an active
tuberculous lesion, although Mantoux or BCG test was positive in 6
(35.2%). The predominant pattern of angiographic affection was Type II
(52.9%). Nephrotic syndrome and portal cavernoma seen in one patient
each were incidental associations. Anti-hypertensive drugs, oral
steroids and drugs to control congestive heart failure were the
mainstays of medical management. Antitubercular therapy was started in
six patients. Angioplasty was attempted in 15 cases and proved to be
partially beneficial in six. Three patients who failed to respond to
medical management had to undergo surgical procedures, either bypass,
nephrectomy or both. In-hospital mortality was 11.7%. Conclusions:of TA
in this study presented acutely in the pulseless phase, with
hypertension and its complications. Type II involvement was the
commonest pattern. Anti-hypertensive agents and steroids along with
angioplasty were partially successful in controlling symptoms in 35.2%.
Surgical procedures were reserved for a minority with poor response to
drugs and angioplasty
Ovarian Dysgenesis with Balanced Autosomal Translocation
Autosomal translocations are rare in the patients with ovarian
dysgenesis. An 18-year-old female who presented with primary
amenorrhoea had hypergonadotropic hypogonadism and streak ovaries with
hypoplastic uterus. Karyotype analysis revealed a balanced autosomal
translocation involving chromosomes 1 and 11. The probable role of
autosomal translocations in ovarian dysgenesis has been discussed
Neurofibromatosis-Noonan syndrome or LEOPARD syndrome? A clinical dilemma
Neurofibromatosis (NF), Noonan syndrome (NS), and LEOPARD syndrome are
all autosomal dominant conditions, each being a distinct clinical
entity by itself. Rarely, one encounters cases with features of NF and
NS and is termed as the 'Neurofibromatosis-Noonan syndrome' (NF-NS).
The authors report a clinical dilemma with major clinical features of
the NF-NS syndrome and LEOPARD syndrome co-existing in the same
patient. Also, features of Noonan syndrome and LEOPARD syndrome are
compared with the case reported