JOURNAL OF CLINICAL AND EXPERIMENTAL HEPATOLOGY
ACUTE INTERMITTENT PORPHYRIA—A
DIAGNOSTIC DILEMMA
Murtaza Kamal
Safdarjung Hospital & VMMC, New Delhi, India
Porphyrias are metabolic diseases resulting from a
partial deficiency of an enzyme in the heme biosyn-
thetic pathway. AIP (deficiency of hydroxymethylbi-
lane synthase) causes acute attacks due to secondary
accumulation of heme precursors. It presents with
abdominal pain, tachycardia, hypertension, hypona-
tremia, seizures, motor neuropathy, etc. Screening is
done with qualitative urinary PBG and ALA. Urinary
and fecal fractionated porphyrins and DNA testing
provided the confirmation. Acute attacks are treated
with IV hemin and glucose. Prevention of acute
attacks is with smoking cessation and avoidance of
inciting agents. A 11-year-old undernourished girl, on
multiple antiepileptics, for 4 years (started for refrac-
tory seizures), presented with complaints of recurrent
generalized abdominal pain with weight loss since the
past 1 year, with constipation and poor oral accep-
tance since 10 days. Examination revealed tender
epigastrium, decreased air entry on left side of chest,
generalized muscle wasting, and paresthesias. Inves-
tigations revealed Hb 9 g/dl; TLC 8200/cu mm (77/
20/01/02); platelets 1,84,000/cu mm; PS-normocytic
normochromic; Na 131 meq/L; K 4 meq/L; Ca
8.9 mg/dl; PO4 3.3 md/dl; ALP 255 U/L; blood urea
49 mg/dl; serum creatinine 0.9 mg/dl; bilirubin
0.5 mg/dl; SGOT 54 U/L; SGPT 21 U/L; blood
culture—no growth; HIV-NR; urine examination—
normal; serum amylase 112U/L; serum lipase 71U/
L; lipid profile, carbamazepine level, and CXR—nor-
mal; PPD test –NR; GA for AFB—negative; USG abdo-
men, CECT abdomen, and brain—normal; CECT
thorax-opacities and necrotic patches on left lower
zone consistent with pulmonary tuberculosis; EEG-
partial epilepsy with focus at right temporal lobe;
serum lead level—normal; Urine for porphobilinogen,
5-ALA, and total porphyrins—positive; and NCV—
Severe axonal motor and sensory neuropathy in all
four limbs. A diagnosis of AIP with pulmonary tuber-
culosis with partial seizure with undernutrition was
made. Antiepileptics were tapered gradually and gaba-
pentine with modified ATT was started and the child
responded very well. AIP should be kept in the differ-
entials of chronic abdominal pain and patients with
psychological symptoms.
http://dx.doi.org/10.1016/j.jceh.2015.07.151
Journal of Clinical and Experimental Hepatology | June/July 2015 | Vol. 5 | No. S2 | S69–S81
EVALUATION OF STRONGER NEO-
MINOPHAGEN-C (SNMC) IN PATIENTS
WITH ACUTE HEPATITIS
Kiran Reddyvari, Avinash Balekuduru, Umesh Jalihal,
Satyaprakash Bonthala
M.S. Ramaiah medical college and hospital, Bangalore, India
Background: Stronger Neo-Minophagen-C (SNMC)
is an intravenous drug with glycyrrhizin as the prin-
cipal ingredient. There is increasing evidence to indi-
cate the therapeutic efficacy of SNMC in normalizing
liver function tests (LFT) in patients with acute
hepatitis.
Objectives: To evaluate the efficacy of SNMC in
patients with hepatitis of all etiologies without
underlying chronic liver disease.
Methods: In this study conducted at a tertiary care
centre, 11 consecutive patients with deranged liver
function tests, unresponsive to conventional treat-
ment with ursodeoxycholic acid for 5 days, were
selected and their response to SNMC was evaluated
based on the improvement in the LFT values. 60 ml
SNMC in 500 ml normal saline was administered as
Miscellaneous
an intravenous infusion over 2 h once a day for 3 days
and the LFT values were assessed on day 4.
Results: The study group comprised 4 women and 7
men. Vomiting and abdominal pain were the present-
ing features. The most common etiology was hepa-
titis B (5/11). Based on LFT and PT values, marked
improvement was observed in all 11 patients (100%).
A significant reduction in alanine transaminase
(ALT), aspartate aminotransferase (AST), prothrom-
bin time (PT), and International Normalised Ratio
(INR) was observed after 3 days of SNMC therapy
using paired T test and Wilcoxon signed ranks test.
There were no side effects noted during the infusion
or follow-up. The median values (25–75 percentile) of
AST, ALT, PT, and INR before SNMC administration
were 1132 IU/Ml (128–1367), 1040 (145–2084), and
14.1 (13–18.1), 1.2 (1.2–1.58) and after SNMC admin-
istration were 160 (75–499), 507 (49.3–810), 13.2
(12.1–17.7), and 1.1 (1.0–1.48), respectively. The
remaining values also showed decreasing trend but
were insignificant.
Conclusion: Administration of SNMC is useful in
the improvement of ALT, AST, PT, and INR levels in
patients with acute hepatitis. Larger studies are
needed to confirm this pilot study results.
Corresponding author: Kiran Reddyvari.
E-mail: kinnureddy@yahoo.com
http://dx.doi.org/10.1016/j.jceh.2015.07.152
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